Conditions in Occupational Therapy

CONDITIONS
IN OCCUPATIONAL
THERAPY
EFFECT ON OCCUPATIONAL
PERFORMANCE
CONDITIONS IN OCCUPATIONAL
THERAPY
EFFECT ON OCCUPATIONAL
PERFORMANCE

Ben J. Atchison, PhD, OTR/L, FAOTA
Professor and Chair
Department of Occupational Therapy
Western Michigan University
Kalamazoo, Michigan
Diane Powers Dirette, PhD, OTL, FAOTA

Professor
Kalamazoo, Michigan
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Names: Atchison, Ben, editor. | Dirette, Diane Powers, editor.
Title: Conditions in occupational therapy: effect on occupational performance / [edited by] Ben J. Atchison,
Diane Powers Dirette.
Description: Fifth edition. | Philadelphia : Wolters Kluwer, [2017] | Includes bibliographical references and
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Subjects: | MESH: Occupational Therapy | Case Reports
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To my wife, Marcia, my best friend
—Ben J. Atchison
To the Mexican Monday Crew; Claire, Madeleine, Jayce, Jordan, and Dave.
Thank you for the love, laughter, and support that make Mondays the best day of
the week!
—Diane Powers Dirette

Contributors
Ben J. Atchison, PhD, OTR/L, FAOTA Professor and Chair

Kalamazoo, Michigan
Shirley Blanchard, PhD, OTR/L, ABDA, FAOTA, FHDR
Associate Professor
Creighton University
Omaha, Nebraska
Ann Chapleau, DHS, OTRL

Associate Professor
Kalamazoo, Michigan
Gerry E. Conti, PhD, OTRL, FAOTA

Assistant Professor
Occupational Therapy Program
Wayne State University
Detroit, Michigan
Joan Ziegler Delahunt, OTD, MS, OTR/L

Rockhurst University
Kansas City, Missouri
Diane Powers Dirette, PhD, OTL, FAOTA

Professor
Kalamazoo, Michigan
Rosanne DiZazzo-Miller, PhD, DrOT, OTRL, CDP
Assistant Professor Occupational Therapy Program
Wayne State University
Detrotit, Michigan
Kathryn Ellsworth, MA, CCC-SLP

Speech-Language Pathologist
St. Paul, Minnesota
Joanne Phillips Estes, PhD, OTR/L

Assistant Professor
Xavier University
Cincinnati, Ohio
Jennifer L. Forgach, MS, OTR/L

Forgach-Smith
Team Rehabilitation
Bingham Farms, Michigan
Joyce Fraker, MS, OTR/L

Occupational Therapist
Physical Medicine and Rehabilitation Service
VA Ann Arbor Healthcare System
Ann Arbor, Michigan
Cynthia A. Grapczynski, EdD, OTR, FAOTA

Professor and Chair
Occupational Science and Therapy Department
Grand Valley State University
Allendale, Michigan
Jennifer Harrison, PhD, MSW

Faculty Specialist II
School of Social Work
Kalamazoo, Michigan
Nancy Hock, OTR/L, CHT
Faculty Specialist II and Site Coordinator
Grand Rapids, Michigan
Heather Javaherian-Dysinger, OTD, OTR/L

Associate Professor and MOT Program Director
Loma Linda University
Loma Linda, California
Reese Martin, MS, OTR/L

Instructor
Kalamazoo, Michigan
Laura V. Miller, MS, OTR/L, CDI, CDRS

Private Practitioner, Occupational Therapy
Certified Driver Rehabilitation Specialist, Driver’s Rehabilitation Center of
Michigan
Livonia, Michigan
Brandon G. Morkut, MS, OTR/L

Adjunct Faculty
Kalamazoo, Michigan
Timothy M. Mullen, PhD, OTR/L, CHT

Director of Clinical Operations
Orthopedic Physicians of Alaska
Anchorage, Alaska
Shelley Mulligan, PhD, OTR/L, FAOTA

Associate Professor
University of New Hampshire
Durham, New Hampshire
Linda M. Olson, PhD, OTRL

Chair and Assistant Professor
College of Health Sciences
Rush University
Chicago, Illinois
Rebecca Ozelie, DHS, OTR/L, BCPR

Assistant Professor and Academic Fieldwork Coordinator
Rush University
Chicago, Illinois
Sharon L. Pavlovich, MAM, COTA/L

Assistant Professor
Loma Linda University
Loma Linda, California
Emily Raphael-Greenfield, EdD, OTRL, FAOTA Assistant Professor

Programs in Occupational Therapy
Columbia University Medical Center
New York, New York
Mylene Schriner, PhD, OTR/L
Chair and Associate Professor of Occupational Therapy Education
Rockhurst University
Kansas City, Missouri
Michelle A. Suarez, PhD, OTR/L

Associate Professor
Kalamazoo, Michigan
Suzänne Fleming Taylor, PhD, MBA/HCM, OTR/L

Cancer Rehabilitation, Clinician IV
Adjunct Instructor, Department of Occupational Therapy
Virginia Commonwealth University Medical Center
Richmond, Virginia
Michael J. Urban, MS, OTR/L, MBA, CEAS, CWCS

Department of Veterans Affairs
Healthcare System of Connecticut
West Haven, Connecticut
Christine K. Urish, PhD, OTR/L, BCMH, FAOTA Professor

St. Ambrose University
Davenport, Iowa
Amy Wagenfeld, PhD, OTR/L, SCEM, FAOTA
Assistant Professor
Kalamazoo, Michigan
Andrea L. Washington, BS, OTR/L

Clinical Occupational Therapy Specialist
Inpatient Rehabilitation
Children’s Hospital of Michigan
Detroit, Michigan
Mary Steichen Yamamoto, MS, OTL

Occupational Therapist
Private Practice
Ann Arbor, Michigan
Jayne Yatczak, PhD, OTRL

Assistant Professor and Director, Occupational Therapy Program
Eastern Michigan University
Ypsilanti, Michigan
Preface
In this fifth edition of Conditions in Occupational Therapy: Effect on

Occupational Performance, we continue with the approach used in previous
editions which includes common conditions seen by occupational therapists as
published in the executive summary of the most recent practice analysis
published by the National Board for Certification of Occupational Therapists
(NBCOT, 2012). The book is designed to facilitate the teaching and learning of
conditions from an occupational therapy perspective. We honor Dr. Ruth
Hansen, Professor Emeritus, Eastern Michigan University, for her significant
contributions as the founding coeditor of this textbook in which she created the
design for analysis of conditions using an “occupational therapy way of
thinking.” Dr. Hansen’s original idea continues to hold forth in Chapter 1,
Thinking Like an OT, which begins with an overview and relevance of the
philosophy and values of occupational therapy in relation to understanding a
given condition and its impact on occupational performance.
In this new edition, we organized the conditions into units including
pediatric, mental, and physical conditions. Each chapter is consistent in its
structure to include an opening case, definition and descriptions, incidence and
prevalence, signs and symptoms, course and prognosis, medical/surgical
management, and impact on occupational performance, followed by two case
illustrations. We continue to incorporate the language of the Occupational
Therapy Practice Framework, third edition (OTPF) where relevant, as this is the
most current “language of the profession.”
Following a national review of faculty who have used the fourth edition of
our textbook and their helpful input, new content has been included. We are
pleased to announce new chapters in this edition. These include Obsessive
Compulsive and Related Disorders, Somatic Symptoms and Related Disorders,
Feeding and Eating Disorders, Sensory Processing Disorders, Substance Related
and Addictive Disorders, Personality Disorders, Cancer, and Obesity. Where
relevant, chapters have included updates published in the Diagnostic and
Statistical Manual of Mental Disorders, fifth edition (American Psychiatric
Association, 2013). As always, each chapter has incorporated the most recent
information available. The notion that occupational therapists treat diagnoses is
on ongoing point of discussion and debate. As emphasized in Chapter 1, we are
mindful of the importance of “person first” philosophy that a person is more than
a sum of his or her diagnosis. Simultaneously, it is essential that occupational
therapists understand the distinct client factors of a given condition that impact a
person’s ability to regain function necessary to resume occupational roles.
Each chapter in this edition provides the authors’ interpretation of the effects
of the condition on occupational performance and is not necessarily all inclusive.
Our goal is that the information will motivate occupational therapy students to
expand their knowledge and understanding of the given condition. We expect
that each chapter is a starting point for discussion and analysis of the condition
which then will lead to the development of effective intervention planning.
Ben J. Atchison
Diane Powers Dirette
American Psychiatric Association. (2013). Diagnostic and statistical manual of
mental disorders (5th ed.). Washington, DC: Author.
National Board for Certification of Occupational Therapists. (2012).
Practice analysis of the occupational therapist registered: Executive summary.
Gaithersburg, MD: Author.

To review key terms and their definitions, visit

http://thePoint.lww.com/Atchison5e, and use the access code on the inside front
cover of this book. Resources for instructors include a test generator, PowerPoint
presentations, and an image bank.
Contents
Contributors
Preface

1 Thinking Like an OT
UNIT 1 • PEDIATRIC CONDITIONS

2 Cerebral Palsy
3 Autism Spectrum Disorders
4 Intellectual Disability
5 Muscular Dystrophy
6 Attention Deficit Disorder
7 Sensory Processing Disorder
UNIT 2 • MENTAL CONDITIONS

8 Mood Disorders
9 Schizophrenia and Other Psychotic Disorders
10 Anxiety Disorders
11 Neurocognitive Disorders
12 Complex Trauma
13 Obsessive-Compulsive and Related Disorders
14 Somatic Symptoms and Related Disorders
15 Feeding and Eating Disorders
16 Substance-Related and Addictive Disorders
UNIT 3 • PHYSICAL CONDITIONS

17 Cerebrovascular Accident
18 Cardiopulmonary Disorders
19 Diabetes
20 Acquired Brain Injury
21 Burns
22 Progressive Neurodegenerative Disorders
23 Rheumatic Diseases
24 Spinal Cord Injury
25 Orthopedics
26 Low Vision Disorders
27 Cancer
28 Obesity

Index
Glossary
C H A P T E R
1 Thinking Like an OT
Diane Powers Dirette Ben J. Atchison
KEY TERMS
Altruism
Client factors
Context and environment
Core values
Equality
Evidence-based practice
Freedom
Justice
Occupations
Performance patterns
Performance skills
Personalized medicine
Person-first language
Philosophical assumptions
Practice Framework
Prudence
Truth
It is more important to know what kind of person has the disease than what kind of disease the person
has.
—Sir William Osler (Address at Johns Hopkins University, February 1905)
Lindsey is finishing her course work in occupational therapy and is now

beginning her first level II fieldwork experience. Throughout her education, she
has learned the importance of evidence-based practice to guide her treatment
decisions. Her challenge now is to develop her clinical reasoning skills to merge
the science she has learned with the art of practice. To achieve this, she must
understand the person’s diagnosis, analyze the person’s unique set of problems
based on the person’s individual characteristics, and determine the impact on
occupational performance. The first step of this process is the referrals she
receives. Each referral gives her some basic information about the person
including the person’s diagnosis. Her job is to decide what to do next.
How does a student learn to correlate general information about a diagnosis
with the needs of a particular person and to identify the problems that require
occupational therapy intervention? How does a staff therapist set priorities for
problems and decide which require immediate attention? How much problem
identification can be done before the therapist actually sees the patient? How
does a supervisor know when a student or therapist is doing a “good job” of
screening referrals and anticipating the dysfunction that the patient might be
experiencing? These are precursors to the actual intervention process and are
essential to effective and efficient clinical reasoning (Benamy, 1996).
The clinical reasoning procedure used by each health care professional is
somewhat different. The information that is the main focus of intervention for a
speech therapist will differ from that of a psychologist or a nurse. The basic tenet
of the occupational therapy profession is that practitioners gather and use
information to help people function in their daily activities. Such data gathering
and analysis provide the therapist with the foundation for a treatment plan
through a prioritized list of anticipated problems or dysfunctions for an
individual.
To comprehend the unique aspects of occupational therapy requires an
understanding of the core values, philosophical assumptions, and domain of
concern of the profession, as well as the language that is used to communicate
information clearly and precisely.
Core Values of Occupational Therapy
The core values of occupational therapy are set forth in the document “Core
Values and Attitudes of Occupational Therapy Practice” (Kanny, 1993). Seven
have been identified: altruism, dignity, equality, freedom, justice, truth, and
prudence.
1. Altruism is the unselfish concern for the welfare of others. This concept is
reflected in actions and attitudes of commitment, caring, dedication,
responsiveness, and understanding.
2. Dignity emphasizes the importance of valuing the inherent worth and
uniqueness of each person. This value is demonstrated by an attitude of
empathy and respect for self and others.
3. Equality requires that all individuals be perceived as having the same
fundamental human rights and opportunities. This value is demonstrated
by an attitude of fairness and impartiality.
4. Freedom allows the individual to exercise choice and to demonstrate
independence, initiative, and self-direction.
5. Justice places value on the upholding of such moral and legal principles as
fairness, equity, truthfulness, and objectivity.
6. Truth requires that we be faithful to facts and reality. Truthfulness or
veracity is demonstrated by being accountable, honest, forthright,
accurate, and authentic in our attitudes and actions.
7. Prudence is the ability to govern and discipline oneself through the use of
reason. To be prudent is to value judiciousness, discretion, vigilance,
moderation, care, and circumspection in the management of one’s affairs,
to temper extremes, make judgments, and respond on the basis of
intelligent reflection and rational thought (Kanny, 1993).
These values are the foundation of the belief system that occupational therapists
(OTs) use as a moral guide when making clinical decisions.
Philosophical Assumptions
The philosophical assumptions of the profession guide OTs in providing client-
centered therapy that meets the needs of the client and society. These
assumptions express our basic beliefs about the client and the context in which
the client functions (Mosey, 1996). These assumptions are as follows:
Each individual has a right to a meaningful existence: the right to live in

surroundings that are safe, supportive, comfortable, and over which he or
she has some control; to make decisions for himself or herself; to be
productive; to experience pleasure and joy; and to love and be loved.
Each individual is influenced by the biological and social nature of the
species.
Each individual can only be understood within the context of his or her
family, friends, community, and membership in various cultural groups.
Each individual has the need to participate in a variety of social roles and to
have periodic relief from participation.
Each individual has the right to seek his or her potential through personal
choice, within the context of accepted social constraints.
Each individual is able to reach his or her potential through purposeful
interaction with the human and nonhuman environment.
Occupational therapy is concerned with promoting functional
interdependence through interactions directed toward facilitating
participation in major social roles (occupational performance) and
development of biological, cognitive, psychological, and social components
(client factors) fundamental to such roles.
The extent to which intervention is focused on the context, on the areas of
occupational performance, or on the client factors depends on the needs of
the particular individual at any given time.
Personalized Medicine
The core values and philosophical assumption of the profession of OT lead
practitioners of OT to a focus on personalized medicine. According to Burke,
Trinidad, and Press (2014), “personalized medicine is best understood as a
comprehensive process to determine the best health care options for a particular
patient, deriving from a partnership between patient and clinician. This approach
offers the opportunity to weigh personal values and preferences as well as
clinical findings” (p. 196). In addition, Topol (2014) defines personalized
medicine as the tailoring of medical treatments to the individual characteristics
of each patient with a focus on the individual as the source of medical data and
as the driver of health care.
The core values, especially dignity, equality, and freedom, are the
profession’s moral guide to personalized medicine. They guide us to value
differences, to treat people equally despite those differences, and to allow
individuals to make their own choices based on differing perspectives and
preferences.
The philosophical assumptions summarize the OT profession’s basic beliefs
about focusing on the rights and preferences of individuals relative to their
biological and social environments. In addition, the philosophical assumptions
help guide practitioners to form a partnership with each individual to determine
the focus of the intervention. Each of these concepts form a practice in which
personalized medicine is an essential element.
Whereas the primary purpose of this book is to describe the potential impact
of a condition on occupational performance, the descriptions should not be
considered prescriptive or exhaustive. It is necessary to understand common
facts of these conditions, including etiology, basic pathogenesis, commonly
observed signs and symptoms, and precautions. However, it is equally important
to recognize that the effects of a condition on occupational well-being will also
be dependent on contextual factors such as age, developmental stage, health
status, and the physical, social, and cultural environment (Dunn, Brown, &
McGuigan, 1994). Rather than viewing an individual as a diagnostic entity, as a
condition, or as the sum of biological cells, the treatment must be personalized.
Language
Although many language systems and mechanisms are available, we will discuss
language from two perspectives. First is a philosophical discussion of using
person-first language. Second is the use of the Occupational Therapy Practice
Framework: Domain and Process, Third Edition (AOTA, 2014) that presents the
professional language and the occupational therapy domain of concern.
Person-First Language
In many cases, the literature and the media, both popular and professional,
describe a person with a given condition as the condition—the arthritic, the C.P.
kid, the schizophrenic, the alcoholic, the burn victim, the mentally disabled. All
of these terms label people as members of a large group rather than as a unique
individual. The use of person-first language requires that the person be identified
first and the disease used as a secondary descriptor. For example, a woman, who
is a physicist, is active in her church and has arthritis; the fourth grade boy, who
is a good speller, loves baseball and has cerebral palsy. The condition does not
and should not be the primary identity of any person.
Consider the following: a father is introducing his son to his coworkers.
Which of the following is the best introduction?
“Hey, everyone, this is my disabled son, John.”
“Hey, everyone, this is my son, John, who is disabled and loves soccer and
video games.”
“Hey, everyone, this is my son, John. He loves soccer and video games.”
Of course, the third statement is the best choice. Yet it is common when
describing a person who has a disability to emphasize the disability first. The
consequence is a labeling process. “Although such shorthand language is
commonplace in clinics and medical records, it negates the individuality of the
person. Each of us is a person, with a variety of traits that can be used to
describe aspects of our personality, behavior, and function. To use a disease or
condition as the adjective preceding the identifying noun negates the multiple
dimensions that make the person a unique individual” (Hansen, 1998).
The Occupational Therapy Practice
Framework
The official language for the profession of occupational therapy was revised in
2014 and presented in a document titled the Occupational Therapy Practice
Framework: Domain and Process, Third Edition (AOTA, 2014). The Practice
Framework outlines the language and constructs that describe the occupational
therapy profession’s domain of concern. The domain defines the area of human
activity to which the occupational therapy process is applied. The process
facilitates engagement in occupation to support participation in life. The specific
aspects of the domain are outlined in the language of the Practice Framework.
The Framework is organized into five aspects—occupations, client factors,
performance skills, performance patterns, and context and environment.
Occupations are various kinds of life activities in which individuals, groups, or
populations engage. Occupations include activities of daily living, instrumental
activities of daily living, rest and sleep, education, work, and play, leisure, and
social participation. Client factors are values, beliefs, and spirituality, the body
functions, and the body structures that reside within the person. These client
factors influence the person’s participation on occupations. Performance skills
are observable elements of action that have an implicit functional purpose. These
skills are separated into the categories of motor skills, process skills, and social
interaction skills. Performance patterns are the habits, routines, roles, and
rituals used by the person in the process of engaging in occupations or activities.
These patterns may enhance or hinder occupational performance. Context and
environment refers to a variety of interrelated conditions that are within and
surrounding the person. Those conditions include cultural, personal, temporal,
and virtual contexts. Environments are the physical and social conditions that
surround the person (Table 1.1).
TABLE 1.1 Occupational Therapy Practice Domains

Each of these aspects has a relationship and influence on the others. The
outcome is, of course, the ability to function and engage in occupations.
Although at a given time you may focus on an occupation or client factors, the
ultimate concern is whether the individual is able to function in daily life. For
example, a therapist may evaluate a person’s attention span, but not in isolation.
Attention span is evaluated within the realm of the performance patterns and
context of the person— the attention span required to work on an assembly line,
to drive a car, to learn a card game, or to conduct a business meeting.
Once a therapist knows the diagnosis and age of the person, he or she can
use this Practice Framework to examine systematically the deficits that occur in
the client factors, as well as how these particular deficits can and do alter the
person’s ability to complete functional activities relevant to occupations. In other
instances, the therapist may focus primarily on the occupation or the context,
without paying much attention to the underlying client factors that influence the
occupational performance. Definitions of all terms are provided in the Glossary
at the back of the book.
Evidence-Based Practice
There has been a call to action in the health professions to practice health care
based on evidence of the effectiveness of each treatment approach (Gutman,
2010). High levels of evidence are based on studies that compare groups of
people, usually with similar conditions. Evidence, especially high levels of
evidence, on which to base one’s practice, however, might be limited (Dirette,
Rozich, & Viau, 2009). First, it is limited by an insufficient number of resources
to support specific treatment approaches for specific conditions. Second, it might
be limited by the fact that groups of people with “average” results do not always
represent the unique situation of the person with whom the therapist is working.
Therefore, while we support the idea of evidence-based practice in general,
there is clearly a need for therapists to develop clinical reasoning skills that will
not only help them decide which evidence to use with people who have
particular conditions but also help them decide what to do with the unique
individual with whom they are working. Understanding the condition with which
the individual presents is often the first step in the clinical reasoning process.
This textbook provides information about common conditions seen by OTs and
provides the first steps in the clinical reasoning process by providing ideas about
the potential impact on occupational performance.
Framework of This Textbook
As an instructional tool, this book provides an opportunity to examine each
condition closely. The reader is urged to use the information as a springboard for
further study of the conditions included here and the many other conditions that
OTs encounter in practice. The analysis of the impact on occupational
performance for a particular condition is dynamic, and the identification of the
most important areas of dysfunction and, therefore, treatment will vary from
practitioner to practitioner. In addition, factors such as secondary health
problems, age, gender, family background, and culture contribute greatly to the
development of a unique occupational performance profile for each individual
served.
The occupational performance approach to the identification of dysfunction
described in this book can be used to examine the effects of any condition on a
person’s daily life. This process will enable the therapist to identify and set a
priority for problems in occupational performance, which, in turn, will serve as
the foundation for creating an effective intervention plan.
REFERENCES
American Occupational Therapy Association. (2014). Occupational therapy
practice framework: Domain and process, 3rd edition. American Journal of
Occupational Therapy, 68(Suppl. 1), S1–S51. Retrieved from
http://dx.doi.org/10.5014/ajot.2014.682006
Benamy, B. C. (1996). Developing clinical reasoning skills. San Antonio, TX:
Therapy Skill Builders.
Burke, W., Trinidad, S. B., & Press, N. A. (2014). Essential elements of
personalized medicine. Urologic Oncology: Seminars and Original
Investigations, 32, 193–197. Retrieved from
http://dx.doi.org/10.1016/j.urolonc.2013.09.002
Dirette, D., Rozich, A., & Viau, S. (2009). Is there enough evidence for
evidence-based practice in occupational therapy? American Journal of
Occupational Therapy, 63, 782–786. Retrieved from
http://dx.doi.org/10.5014/ajot.63.6.782
Dunn, W., Brown, C., & McGuigan, A. (1994). Ecology of human performance:
A framework for considering the effect of context. American Journal of
Occupational Therapy, 48(7), 595–607.
Gutman, S. A. (2010). From the desk of the editor: AJOT publication priorities.
American Journal of Occupational Therapy, 64, 679–681. Retrieved from
Hansen, R. A. (1998). Ethical implications. In J. Hinojosa & P. Kramer (Eds.),
Evaluation: Obtaining and interpreting data. Bethesda, MD: AOTA.
Kanny, E. (1993). Core values and attitudes of occupational therapy practice.
American Journal of Occupational Therapy, 47, 1085–1086.
Mosey, A. C. (1996). Applied scientific inquiry in the health professions: An
epistemological orientation (2nd ed.). Bethesda, MD: American Occupational
Therapy Association.
Topol, E. J. (2014). Individualized medicine from prewomb to tomb. Cell, 157,
241–253. Retrieved from http://dx.doi.org/10.1016/j.cell.2014.02.012
UNIT
1
Pediatric Conditions
The Pediatric Conditions Unit includes the most common conditions that
children have who are treated by occupational therapists as determined by the
National Board of Certification in Occupational Therapy. These chapters focus
on conditions that are typically diagnosed in childhood, but many of them affect
people throughout their lifespan. Each chapter provides information about the
etiology, incidence and prevalence, signs and symptoms, course and prognosis,
diagnosis, medical/surgical management, and impact on occupational
performance of these conditions. Case illustrations are used to provide examples
of lives affected by the condition. The conditions included in this unit are the
following:
Chapter 2: Cerebral Palsy
Chapter 3: Autism Spectrum Disorders
Chapter 4: Intellectual Disability
Chapter 5: Muscular Dystrophy
Chapter 6: Attention Deficit Disorder/ADHD
Chapter 7: Sensory Processing Disorder
C H A P T E R
2 Cerebral Palsy
Mary Steichen Yamamoto
KEY TERMS
Ataxia Athetoid (dyskinetic) Clonus Contracture Diplegia Dysarthria
Equinovarus Equinovalgus Gastroesophageal reflux Hemiplegia Homonymous
hemianopsia Hydrocephalus Hypertonicity (spasticity) Hyperreflexia
Hypotonicity Kyphosis Lordosis Nystagmus Primitive reflexes Quadriplegia
Scoliosis Strabismus Stretch reflex
A couple who had been trying to conceive a child for several years were thrilled
when a family friend asked if they would be interested in adopting a baby girl
that had just been born to a young unmarried woman in her church. The baby
was born 6 weeks early and her weight was 4 lb, but she appeared to be healthy.
After initiating the paperwork for a private adoption, they brought the baby
home and named her Jill. By the time of Jill’s 6-month well-baby visit, her
parents had become concerned. She appeared to be a bright baby who smiled and
cooed and enjoyed reaching for and playing with toys, but her legs seemed stiff
and she was not yet rolling over. They spoke with their family doctor about their
concerns, but he assured them that Jill was developing normally and they had
nothing to be concerned about. By the time of Jill’s 9-month well-baby visit, her
parents’ concerns were only growing. Jill was still not sitting up and had not yet
learned to roll over or crawl. Her doctor decided to refer Jill to the county early
intervention program for a developmental assessment. Jill was assessed by the
early intervention team consisting of an occupational therapist, physical
therapist, and speech and language pathologist. The occupational therapist noted
some mildly increased tone and incoordination in her upper extremities and a 2-
to 3-month delay in fine motor and self-help skills. The physical therapist noted
that Jill had hypertonicity and retained primitive reflexes in her lower
extremities, which was causing significant delay in the acquisition of gross
motor skills. The speech and language therapist found Jill’s cognitive, language,
and social skills to be at age level. The team suggested to the parents that they
have a pediatric neurologist assess Jill, as she was demonstrating some of the
signs and symptoms of cerebral palsy. Both the occupational and physical
therapist recommended that therapy services begin as soon as possible. An IFSP
(Individualized Family Service Plan) was developed at a subsequent meeting,
and Jill began receiving weekly physical and occupational therapy services.Jill’s
parents took her to a pediatric neurologist who diagnosed her with spastic
diplegia, a type of cerebral palsy. Her parents were initially overwhelmed and
devastated by the diagnosis. The next year was very difficult as they grieved the
loss of so many dreams that they had for Jill and faced so much uncertainty
about her future. They waded through an array of possible therapy approaches
and medical and surgical interventions that were recommended trying to decide
which would be right for Jill and their family. They struggled to find time to
work on home exercises that had been prescribed for Jill. The strain became so
great that they even separated for a period but eventually reconciled. By the time
Jill turned 3 years old, she was walking with a walker and able to sit in a chair
independently, although she needed assistance with changing positions. She was
feeding herself but not yet dressing herself. They enrolled her in a preschool
special education classroom where she received therapy services. By
kindergarten, Jill was in a regular education classroom with a paraeducator for
safety and support. Jill was a happy child, who had many friends and did well
academically. Jill most likely will continue to need some type of additional
support in order to be an independent adult, but all involved were optimistic
about her future.
Description and Definition
Sigmund Freud, in his monograph entitled “Infantile Cerebral Paralysis,” points
out that a well-known painting by Spanish painter Jusepe Ribera (1588–1656),
which depicts a child with infantile hemiplegia, proves that cerebral paralysis
existed long before medical investigators began paying attention to it in the mid-
1800s (Freud, 1968). Freud’s work as a neurologist is not generally well known,
and at the time that his monograph was published in 1897, he was already deep
into his work in the area of psychotherapy. However, he was recognized at the
time as the prominent authority on the paralyses of children. Today, cerebral
paralysis is known as cerebral palsy.
Cerebral palsy is not one specific condition but rather a grouping of clinical
syndromes that affect movement, muscle tone, and coordination as a result of an
injury or lesion of the immature brain. It is not considered a disease, but is
classified as a developmental disability since it occurs early in life and interferes
with the development of motor and sometimes cognitive skills. Historically,
cerebral palsy has been classified as and is still sometimes diagnostically
referred to as a static encephalopathy (Brooke, 2010). This is now considered
inaccurate due to recognition of the fact that the neurological manifestations of
cerebral palsy often change or progress over time. Static encephalopathy is
permanent and unchanging damage to the brain and includes other
developmental problems such as fetal alcohol syndrome, cognitive impairments,
and learning disabilities. Many individuals with cerebral palsy perform well
academically and vocationally without any signs of cognitive dysfunction
associated with the term encephalopathy (Johnson, 2004).
A child is considered to have cerebral palsy if all of the following
characteristics apply: The injury or insult occurs when the brain is still
developing. It can occur anytime during the prenatal, perinatal, or postnatal
periods. There is some disagreement about the upper age limit for a diagnosis of
cerebral palsy during the postnatal period. An upper age limit ranging from 2 to
8 years of age is applied to postneonatally acquired brain injury (Smithers-
Sheedy et al., 2009).
1. It is not progressive. Once the initial insult to the brain has occurred, there
is no further worsening of the child’s condition or further damage to the
central nervous system. However, the characteristics of the disabilities
affecting an individual often change over time.
2. It always involves a disorder in sensorimotor development that is
manifested by abnormal muscle tone and stereotypical patterns of
movement. The severity of the impairment ranges from mild to severe.
3. The sensorimotor disorder originates specifically in the brain. The muscles
themselves and the nerves connecting them with the spinal cord are
normal. Although some cardiac or orthopedic problems can result in
similar postural and movement abnormalities, they are not classified as
cerebral palsy.
4. It is a lifelong disability. Some premature babies demonstrate temporary
posture and movement abnormalities that look similar to patterns seen in
cerebral palsy but resolve typically by 1 year of age. For children with
cerebral palsy, these difficulties persist.
Etiology
Historically, birth asphyxia was considered the major cause of cerebral palsy.
When the British surgeon William Little first identified cerebral palsy in 1860,
he suggested that a major cause was a lack of oxygen during the birth process
(Little, 1862). In 1897, Sigmund Freud disagreed, suggesting that the disorder
might have roots earlier in life. Freud wrote, “Difficult birth, in certain cases is
merely a symptom of deeper effects that influence the development of the fetus”
(Freud, 1968). Although Freud made these observations in the late 1800s, it was
not until the 1980s that research supported his views (Freeman & Nelson, 1988;
Illingsworth, 1985). Only a small percentage of cases of cerebral palsy are a
result of birth complications. The majority of children (70% to 80%) who are
diagnosed with cerebral palsy have congenital cerebral palsy, that is, the injury
to the brain occurred prior to their birth (Johnson, 2004).
There are a large number of risk factors that can result in cerebral palsy, and
the interplay between these factors is often complex, making it difficult to
identify the specific cause (Blickstein, 2003). The presence of risk factors does
not always result in a subsequent diagnosis of cerebral palsy. The presence of
one risk factor may not result in cerebral palsy unless it is present to an
overwhelming degree. Current thought is that often two or more risk factors may
interact in such a way as to overwhelm natural defenses, resulting in damage to
the developing brain. The strongest risk factors are prematurity and low birth
weight (Lawson & Badawi, 2003). During the postpartum period, premature and
low birth weight infants are at greater risk for developing complications,
especially in the circulatory and pulmonary systems. These complications can
lead to brain hypoxia and result in cerebral palsy.
Additional risk factors include intrauterine exposure to infection and
disorders of coagulation (National Institute of Neurological Disorders and
Stroke, 2015). Maternal infection is a critical risk factor for cerebral palsy, both
during prenatal development and at the time of delivery. The infection does not
necessarily produce signs of illness in the mother, which can make it difficult to
detect. In a study conducted in the mid-1990s, it was determined that mothers
with infections at the time of birth had a higher risk of having a child with
cerebral palsy (Grether & Nelson, 1997). Table 2.1 lists specific risk factors
related to both congenital and acquired types of cerebral palsy.
TABLE 2.1 Cerebral Palsy: Contributing Risk Factors and Causes
Adapted from UCP Research and Educational Foundation. Factsheet: Cerebral Palsy: Contributing Factors
and Causes. September, 1995.
There are four types of injuries to the brain that often result in cerebral palsy
(National Institute of Neurological Disorders and Stroke, 2015):
1. Periventricular leukomalacia (PVL) involves damage to the white matter in
the brain adjacent to the lateral ventricles due to ischemia, or restriction in
blood supply to the brain tissue. Necrosis occurs resulting in empty areas or
cysts that fill up with fluid. This is most often associated with premature
birth before 32 weeks of gestation. Sixty to one hundred percent of
premature infants with PVL later show signs of cerebral palsy (Zach, 2010).
2. Hypoxic-ischemic encephalopathy (HIE), commonly known as perinatal
asphyxia, occurs when there is a loss of oxygen resulting in damage to brain
tissue. It most often occurs in the full-term infant in the perinatal period and
can be caused by many factors related to birth and delivery as well as fetal
stroke.
3. Intraventricular hemorrhage (IVH) involved bleeding into the brain’s
ventricular system. This most often occurs in infants born more than 10
weeks prematurely due to the blood vessels in the brain not being fully
developed at this gestational period. It is rarely present at birth, but
develops during the first several days of life. There are four types or grades
of IVF based upon the amount of bleeding that occurs. Grades 1 and 2
involve a smaller amount of bleeding and typically do not result in any
long-term developmental problems. Grades 3 and 4 involve more severe
bleeding. Hydrocephalus can occur when blood presses on or leaks into
the brain tissue resulting in blood clots blocking the flow of cerebral spinal
fluid. Grades 3 and 4 bleeds are most often associated with cerebral palsy.
4. Cerebral dysgenesis is a brain malformation. It differs from the other three
causes of cerebral palsy that are due to lesions or brain injuries. With
cerebral dysgenesis, the brain did not grow properly or fully develop. The
first 20 weeks of gestation are most critical for brain development, and
factors such as maternal infections or trauma can result in a brain
malformation. The severity of brain malformations can vary greatly with a
more severe type resulting in cerebral palsy.
In a small percentage of children with cerebral palsy, the condition was acquired
after birth. Causes in the postnatal and early childhood periods include
cerebrovascular accidents (CVA’s; infections such as meningitis or encephalitis;
poisoning; trauma such as near-drowning and strangulation; child abuse; and
illnesses such as endocrine disorders; Grether & Nelson, 1997; Marmer, 1997).
Closed-head injury that occurs during this period is now classified as traumatic
brain injury, even though the resulting impairments are very similar to cerebral
palsy.
Incidence and Prevalence
Cerebral palsy is the most common motor disability in childhood (Jones,
Morgan, Shelton, & Thorogood, 2007). The Autism and Developmental
Disabilities Monitoring Network has been monitoring the incidence of cerebral
palsy in children in four areas of the United States. Estimates of the incidence of
cerebral palsy in the United States range from 3.1 to 3.6 per 1,000 live births
(Christensen et al., 2014). They also found that it was significantly more
common among boys than among girls at a ratio of 1.4:1. It is also more
common among black children than among white or Hispanic children. There
was a 70% increase in prevalence in middle- and low-income areas as compared
with upper class areas (Centers for Disease Control and Prevention, 2004;
Cerebral Palsy International Research Foundation, 2009). The United Cerebral
Palsy Association estimated as of 2012 that 764,000 children and adults in the
United States show one or more symptoms of cerebral palsy. They estimated that
each year, 10,000 infants and 1,200 to 1,500 preschool-aged children are
diagnosed with cerebral palsy (United Cerebral Palsy Research and Education
Foundation Factsheet, 2012).
There has been considerable advancement in obstetric and neonatal care
during the past three to four decades. Many hoped these advancements would
reduce the incidence of cerebral palsy. Unfortunately, the rate has remained
relatively stable over this period. This is probably a result of increased survival
rates of very low birth weight and premature infants. Another factor may be the
use of fertility treatments by older women that have resulted in an increase in the
number of multiple births. Multiple births tend to result in infants who are
smaller and premature and are at greater risk for health problems. On the
average, they are half the weight of other babies at birth and arrive 7 weeks
earlier. There is a 400% increase in the probability of cerebral palsy in twin
births than in a single birth (United Cerebral Palsy Research and Educational
Foundation, 1997).
Signs and Symptoms
The early signs and symptoms common to all types of cerebral palsy are
abnormal muscle tone, reflex and postural abnormalities, delayed motor
development, and atypical motor performance.
Tone Abnormalities
Tone abnormalities include hypertonicity, hypotonicity, and fluctuating tone.
Fluctuating tone shifts in varying degrees from hypotonic to hypertonic. Muscle
tone can be characterized as the degree of resistance when a muscle is stretched.
For instance, when there is hypotonicity and the elbow is passively extended,
there will be little to no resistance to the movement and hypermobility in the
elbow joint. With hypertonicity, there will be increased resistance, and it may be
difficult to pull the elbow into full extension if the tone is strong. Most infants
with cerebral palsy initially demonstrate hypotonia. Later, the infant may
develop hypertonicity and fluctuating tone or continue to demonstrate hypotonia,
depending on the type of cerebral palsy.
Reflex Abnormalities
With hypertonicity, reflex abnormalities such as hyperreflexia, clonus,
overflow, enhanced stretch reflex, and other signs of upper motor neuron
lesions are present. Retained primitive infantile reflexes and a delay in the
acquisition of righting and equilibrium reactions occur in conjunction with all
types of abnormal tone. When hypotonia is present, there may be areflexia or an
absence of primitive reflexes. These reflexes should be present during the first
several months of life, and it is of concern when they are not.
Atypical Posture
The presence of primitive reflexes and muscle tone abnormalities causes the
child to have atypical positions at rest and to demonstrate stereotypical and
uncontrollable postural changes during movement. For instance, in an infant
lying supine with hypertonicity in the lower extremities, the hips are typically
internally rotated and adducted and the ankles plantar flexed. This posture is
caused by a combination of hypertonicity in the affected muscles and the
presence of the crossed extension reflex (Fig. 2.1). In contrast, when an infant
with hypotonicity in the lower extremities is lying supine, the hips are typically
abducted, flexed, and externally rotated because of low muscle tone, weakness in
the affected muscles, and the influence of gravity.
Figure 2.1 Child with spasticity in upper and lower extremities. Note hip
adduction and scissoring in his legs, internal rotation at shoulders, fisted hand
position, and overflow movements in his mouth. (From Hatfield, N. T. (2013).
Introductory maternity and pediatric nursing. Philadelphia, PA: Lippincott
Williams & Wilkins.)
Delayed Motor Development
Cerebral palsy is always accompanied by a delay in the attainment of motor
milestones. One of the signs that often alerts the pediatrician to the problem is a
delay in the child’s ability to sit independently or to crawl. While cerebral palsy
is often present at birth, it is often not recognized until the child fails to achieve
these early motor milestones.
Atypical Motor Performance
The way in which a child moves when performing skilled motor acts is also
affected. Depending on the type of cerebral palsy, the child may demonstrate a
variety of motor abnormalities such as asymmetrical hand use, unusual crawling
method or gait, uncoordinated reach, or difficulty sucking, chewing, and
swallowing.
Types of Cerebral Palsy
Types of cerebral palsy are classified neurophysiologically into three major
types: spastic, athetoid, and ataxia. Each results from damage in different parts
of the brain (Fig. 2.2).
Figure 2.2 Cerebral palsy. Shown are the major parts of the brain involved in
each of the three major types of cerebral palsy: spastic, athetoid, and ataxic.
(From Wilkins, E. M. (2012). Clinical practice of the dental hygienist (11th ed.).
Philadelphia, PA: Wolters Kluwer Health.)
1. Spastic is characterized by hypertonicity. Deep tendon reflexes are present

in affected limbs, and motor control is affected by the hypertonicity. This
type is the most common and accounts for approximately 80% of people
with cerebral palsy (Centers for Disease Control and Prevention, 2015).
The spasticity is a result of upper motor neuron involvement (Porter &
Kaplan, 2009). Within this category, types are further subdivided
anatomically according to the parts of the body that are affected.
2. Athetoid also known as dyskinetic type is characterized by involuntary
and uncontrolled movements. These movements are typically slow and
writhing. This type is much less common and results from basal ganglia
involvement (Porter & Kaplan, 2009).
3. Ataxia is characterized by unsteadiness and difficulties with balance,
particularly when ambulating. It is the least common type of cerebral palsy
and results from involvement of the cerebellum or its pathways (Porter &
Kaplan, 2009).
It is common for there to be mixed forms where two of the types occur together
as a result of diffuse brain damage. The most common is spastic with athetoid.
Persons with this type have signs of athetosis, and postural tone that fluctuates
from hypertonicity to hypotonia. Athetoid combined with ataxia is less common
(Porter & Kaplan, 2009).
Spastic
Spastic cerebral palsy is characterized by hypertonicity, retained primitive
reflexes in affected areas of the body, and slow, restricted movement. The impact
on motor function can range from a mild impairment that does not interfere with
functional skills, such as not having isolated finger movement, to a severe
impairment, where there is an inability to reach and grasp. Contractures, where
there is permanent shortening of a muscle or joint and deformities, are common
(Fig. 2.3). Spastic cerebral palsy is categorized anatomically according to the
area of the body that is affected. Spastic hemiplegia, spastic diplegia, and spastic
quadriplegia are the most common types. Spastic triplegia has similar features to
spastic quadriplegia with three limbs involved. Typically, it is both lower
extremities and one upper extremity. There are often mild coordination
difficulties in the noninvolved upper extremity. Monoplegia, where one limb is
affected, is rare and, when it does occur, is typically mild (Molnar & Alexander,
1998).
Figure 2.3 Due to spasticity and limited controlled movement and range of
motion in upper extremities and hands, this person is using a mouth stick to
activate a computer. Note wrist flexion contracture. (From Carter, P. J. (2011).
Lippincott textbook for nursing assistants. Philadelphia, PA: Lippincott Williams
& Wilkins.)
Spastic Hemiplegia
Spastic hemiplegia involves one entire side of the body, including the head,
neck, and trunk. Usually, the upper extremity is most affected. Early signs
include asymmetrical hand use during the first year or dragging one side of the
body when crawling or walking. The child learns to walk later than is typical,
and when walking, the child typically hyperextends the knee, and the ankle is in
equinovarus or equinovalgus position on the involved side. The child often
lacks righting and equilibrium reactions on the involved side and will avoid
bearing weight on this side. The shoulder is held in adduction, internal rotation;
the elbow is flexed; the forearm is pronated; the wrist is flexed and ulnar
deviated; the thumb is adducted, and the fingers are flexed (Fig. 2.4). Spasticity
increases during physical activities and emotional excitement. Arm and hand use
is limited on the involved side, depending on the severity. The child may use
more primitive patterns of grasping and lacks precise and coordinated
movement. In more severe cases, the child may totally neglect the involved side
or use it only as an assist during bilateral activities.
Figure 2.4 Abnormal hand position due to spasticity. Note thumb adduction and
flexion in (A). When wrist is flexed, thumb is released and tone reduced but still
not in a position for a functional grasp. (B) (From Salter, R. B. (1998). Textbook
of disorders and injuries of the musculoskeletal system. Philadelphia, PA:
Lippincott Williams & Wilkins.)
Spastic Diplegia
Spastic diplegia involves both lower extremities, with mild incoordination,
tremors, or less severe spasticity in the upper extremities. It is most often
attributed to premature birth and low birth weight and is, therefore, on the rise as
more infants born prematurely survive as a result of medical advances. The
ability to sit independently can be delayed up to 3 years of age or older because
of inadequate hip flexion and extensor and adductor hypertonicity in the legs
(Bobath, 1980). Frequently, the child will rely on the arms for support. The
young child will move forward on the floor by pulling along with flexed arms
while the legs are stiffly extended. Getting up to a creeping position is difficult
because of spasticity in the lower extremities. Similarly, standing posture and
gait are affected to varying degrees, depending on severity. Because of a lack of
lower extremity equilibrium reactions, excessive trunk and upper extremity
compensatory movements are used when walking. Lumbar lordosis, hip flexion
and internal rotation (scissoring), plantar flexion of the ankles, and difficulty
shifting weight when walking are common (Fig. 2.5). Many of these problems
result in contractures and deformities, including dorsal spine kyphosis, lumbar
spine lordosis, hip subluxation or dislocation, flexor deformities of hips and
knees, and equinovarus or equinovalgus deformity of the feet (Bobath, 1980).
Approximately 80% to 90% of children with diplegia will walk independently,
some requiring assistive devices such as crutches or a walker to do so (Sola &
Grant, 1995). Walking will be slower and more labored with a crouched gait
sometimes developing.
Figure 2.5 Atypical posture and structural deformities seen in spastic diplegia.
Note crouched posture due to abnormal muscle tone and strength: hip flexion
and internal rotation, knee flexion, and equinovalgus positioning of the feet.
(From Liebenson, C. (2014). Functional training handbook. Philadelphia, PA:
Wolters Kluwer Health.)
Spastic Quadriplegia
With spastic quadriplegia, the entire body is involved. The arms typically
demonstrate spasticity in the flexor muscles, with spasticity in the extensor
muscles in the lower extremities. Because of the influence of the tonic
labyrinthine reflex (TLR), shoulder retraction and neck hyperextension are
common, particularly in the supine position. This results in difficulty with
transitional movements such as rolling or coming up to sitting. In the prone
position, there is increased flexor tone, also a result of TLR influence, causing
difficulty with head raising and bearing weight on the arms. Independent sitting
and standing are difficult for the child because of hypertonicity, the presence of
primitive reflex involvement, and a lack of righting and equilibrium reactions.
Only a small percentage of children with quadriplegia are able to walk
independently (Sola & Grant, 1995). Oral musculature is usually affected, with
resulting dysarthria, eating difficulties, and drooling (Molnar & Alexander,
1998). Individuals are susceptible to contractures and deformities, particularly
hip dislocation and scoliosis, and must be closely monitored (Fig. 2.6).
Figure 2.6 Severe scoliosis with pelvic obliquity in person with spastic
quadriplegia. Scoliosis with this severity can compromise respiratory function.
(From Flynn, J. M., & Wiesel, S. W. (2010). Operative techniques in pediatric
orthopaedics. Philadelphia, PA: Lippincott Williams & Wilkins.)
Athetosis
Athetosis is the most common type of dyskinesia or dystonia, characterized by
slow, writhing involuntary movements of the face and extremities or the
proximal parts of the limbs and trunk. Abrupt, jerky distal movements
(choreiform) may also appear. The movements increase with emotional tension
and are not present during sleep. Head and trunk control is often affected, as is
the oral musculature, resulting in drooling, dysarthria, and eating difficulties.
Whereas spasticity is characterized by hypertonicity in the affected muscle
groups and restricted movement, fluctuating tone and excessive movement
characterize athetosis. Contractures are rare, but hypermobility may be present
because of fluctuating hypotonicity.
Ataxia
Ataxia is characterized by a wide-based, staggering, and unsteady gait. Children
with ataxia often walk quickly to compensate for their lack of stability and
control. Controlled movements are clumsy. Intention tremors may be present.
The ability to perform refined movements such as handwriting is affected.
Hypotonicity is often present (Low, 2010).
Associated Disorders
In addition to the motor impairments, there are a number of disorders and
difficulties associated with cerebral palsy that can significantly affect functional
abilities. In some cases, associated disorders can have a more significant impact
on function than the motoric aspects of cerebral palsy.
Cognitive Impairment
Of all the associated disorders with cerebral palsy, cognitive impairment has the
most significant impact upon functional outcomes. Estimates of the incidence of
cognitive or intellectual impairment with cerebral palsy range from 30% to 50%
(Molnar & Alexander, 1998). In about one-third of these instances, the cognitive
impairment is mild. The most significant impairments most often occur with
mixed types and severe spastic quadriplegia. Athetoid–type cerebral palsy has
the least occurrence of cognitive impairment. Many children with spastic
hemiplegia and diplegia have average intelligence (Molnar & Alexander, 1998;
Porter & Kaplan, 2009).
Seizure Disorder
Reports of the incidence of seizures in people with cerebral palsy range from
25% to 60% (Porter & Kaplan, 2009). The incidence varies across the diagnostic
categories. It is most common in spastic hemiplegia and quadriplegia and rare
with spastic diplegia and athetosis (Centers for Disease Control and Prevention,
2004; Johnson, 2004; Molnar & Alexander, 1998; Porter & Kaplan, 2009). A
population-based study published in 2003 found that the frequency of epilepsy in
children with cerebral palsy was 38%. Partial seizures were the most common
type, and children with cognitive impairments had a higher frequency of a
seizure disorder (Carlsson, Hagberg, & Olsson, 2003).
Visual and Hearing Impairments
Visual and hearing impairments occur at a higher rate with cerebral palsy than in
the general population. Common visual defects include strabismus, nystagmus,
and difficulties with visual fixation and tracking (Molnar & Alexander, 1998).
Some other visual defects are known to occur related to specific types of
cerebral palsy. Homonymous hemianopsia can occur in children with spastic
hemiplegia, paralysis of upward gaze in children with athetosis, and nystagmus
and lack of depth perception can occur with ataxia (Dufresne, Dagenais, Shevell,
& REPACQ Consortium, 2014). Hearing impairments most often include
sensorineural hearing loss, due to congenital nervous system infections
(Johnson, 2004). Conductive hearing losses, caused by persistent fluid in the ears
and middle ear infections, occur when there is severe motor involvement in
children who spend a lot of time in a prone or supine position (Blackman, 1997).
Oral Motor
If the oral musculature is affected, the individual with cerebral palsy may have
significant difficulty with speaking and eating. Dysarthria, if it is severe, may
affect functional communication resulting in the need for alternative forms of
communication. Eating difficulties can result in increased risk of aspiration,
limited amount of foods consumed, and difficulty with chewing and swallowing.
Drooling is a significant problem in about 10% of the cases of cerebral palsy
(Stanley & Blair, 1994). It can be due to many factors including oral motor
muscular control, impaired oral sensation, inefficient and infrequent swallowing,
poor lip closure, decreased jaw control, and poor head control.
Dental problems occur frequently in individuals with cerebral palsy. Motor
problems and oral sensitivity can make tooth brushing more difficult. The
combination of enamel dysplasia, mouth breathing, and poor hygiene leads to
increased tooth decay and periodontal diseases (Molnar & Alexander, 1998).
Gastrointestinal
Gastrointestinal difficulties occur frequently in cerebral palsy.
Gastroesophageal reflux can create much discomfort and can result in refusal
to eat or difficulty transitioning to solid foods. It requires medical intervention
such as medication or in more serious cases surgery. Constipation is common
due to decreased mobility and exercise as well as inadequate intake of water or
unusual diets due to difficulties with oral motor control (Molnar & Alexander,
1998).
Pulmonary
Individuals with more severe motor impairments such as spastic quadriplegia
often develop scoliosis or other spinal deformities that can impact respiration.
The respiratory muscles themselves may be affected, which results in poor
respiration. These individuals are prone to frequent upper respiratory infections
that can significantly impact their health. When difficulty chewing and
swallowing is associated with poor breathing and inadequate or decreased ability
to cough, this can result in increased aspiration pneumonia. A barium swallow
study can be conducted to rule out aspiration as a cause of frequent pneumonia.
In cases where there is aspiration, a gastric feeding tube may need to be inserted.
Premature infants who had bronchopulmonary dysplasia also have
compromised pulmonary systems (Molnar & Alexander, 1998).
Diagnosis
No definitive test will diagnose cerebral palsy. Several factors must be
considered. Physical evidence includes a history of delayed achievement of
motor milestones; however, delayed motor development can occur with a host of
other developmental disabilities and genetic syndromes. The quality of
movement is the factor that helps provide a differential diagnosis. The findings
of atypical or stereotypical movement patterns, the presence of infantile reflexes,
and abnormal muscle tone all point toward a diagnosis of cerebral palsy.
However, other causes must be ruled out, such as progressive neurological
disorders, mucopolysaccharidosis, muscular dystrophy, or a spinal cord tumor.
Many of these disorders can be ruled out by laboratory tests, although some must
be differentiated by clinical or pathological criteria. A magnetic resonance
imaging (MRI) or computed tomography (CT) scan may provide evidence of
hydrocephalus, help determine the location and extent of structural lesions, and
help to rule out other conditions. However, these scans are not definitive as far as
making a diagnosis of cerebral palsy and are not predictive as far as the child’s
functioning (Miller & Bachrach, 2006). The yield of finding an abnormal CT
scan in a child with cerebral palsy is 77% and for an MRI it is about 89%
(Ashwal et al., 2004). An electroencephalogram (EEG) should not be used to
determine the cause of cerebral palsy but should be obtained if there is an
indication that the child may be having seizures. Because of the high occurrence
of associated conditions, children with cerebral palsy should be screened for
cognitive, visual, and hearing impairments, as well as speech and language
disorders.
Cerebral palsy often is not evident during the first few months of life and is
rarely diagnosed that early. Most cases, however, are detected by 12 months, and
nearly all can be diagnosed by 18 months (Miller & Bachrach, 2006). In some
cases, early postural and tonal abnormalities in premature infants can resemble
cerebral palsy, but the signs are transient with normal subsequent development.
Course and Prognosis
The course of cerebral palsy varies depending on type, severity, and the presence
of associated problems. With mild motor involvement, the child will continue to
make motor gains and compensate for motor difficulties. With more severe
forms, little progress may be made in attaining developmental milestones and
performing functional tasks. As the child grows older, secondary problems such
as contractures and deformities will become more common, especially with
spasticity. Adults with cerebral palsy experience musculoskeletal difficulties and
loss of function at an earlier age than do their nondisabled peers. One study
found that 75% of individuals with cerebral palsy had stopped walking by age 25
due to fatigue and walking insufficiency (Murphy, Molnar, & Lankasky, 1995).
Another study of young adults found clinical evidence of arthritis in 27% of the
subjects with cerebral palsy as compared to 4% in the general population
(Cathels & Reddihough, 1993).
The survival rate for adults with cerebral palsy is good but lower than that of
the general population. A study in Great Britain found that there was an 86%
survival rate at age 50 for adults with cerebral palsy compared to 96% for the
general population. After age 50, the relative risk of death was only slightly
higher in women with cerebral palsy as compared to the general population. The
risk for men with cerebral palsy was the same as the general population. Adults
with cerebral palsy were more likely to die of respiratory disease than the
general population but less likely to die from an accident or injury (Hemming,
Hutton, & Pharoah, 2006).
Medical/Surgical Management
Because of the complexity and diversity of difficulties affecting the individual
with cerebral palsy, medical management requires a team approach using the
skills of many professionals. Depending on the type of cerebral palsy and the
presence of associated problems, team members typically include an
occupational therapist, physical therapist, speech pathologist, educational
psychologist, nurse, and social worker. The emphasis of intervention is on
helping the child gain as much motor control as possible, positioning the child to
minimize the effects of abnormal muscle tone, instructing the parents and
caregivers on handling techniques and ways to accomplish various activities of
daily living, recommending adaptive equipment and assistive technology to
increase the child’s ability to perform desired activities, providing methods to
improve feeding and speech if difficulties are present, and helping parents
manage behavioral concerns and family stresses.
The primary physician treats the usual childhood disorders and helps with
prevention of many health problems. Physicians with various medical specialties
may also be involved. These include a neurologist to assess neurological status
and help control seizures, if present; an orthopedist to prescribe orthotic devices
and any necessary surgeries; and an ophthalmologist to assess and treat any
visual difficulties.
Medical management includes both surgical and nonsurgical approaches,
with much of the focus on techniques to decrease spasticity. Oral medications
such as diazepam (Valium), dantrolene (Dantrium), and baclofen have been used
to reduce spasticity in severe cases with mixed results (Johnson, 2004).
Intrathecal baclofen infusion (ITB) administered through a pump implanted in
the abdominal wall to the spinal cord fluid has shown to be more effective than
oral medications in reducing severe spasticity and dystonia in cerebral palsy
(Albright, 1996). There is however the potential for serious side effects, and the
long-term consequences are not yet known (Stempien & Tsai, 2000). Another
treatment more widely used in recent years is the injection of botulinum toxin
(Botox) into muscles. Spasticity is reduced for a period of 3 to 6 months after
injection. Botox is injected into specific muscles, which, in addition to reducing
tone, increases range of motion and reduces deformities as well as provides an
opportunity to work on muscle strengthening. Minimal side effects have been
reported; however, its long-term effectiveness on function has not been
demonstrated (Steinbok, 2006).
Orthotics and splinting are used to improve function and prevent
contractures and deformities. Upper extremity resting or night splints are used to
maintain range of motion. Soft splints, dynamic splints, and those allowing
movement of the fingers and thumb are used during waking hours and functional
activities to reduce tone and promote more typical patterns of movement. Ankle-
foot orthosis (AFO) and variants are often used to control spastic equinus,
promote alignment of the hind foot, and control midfoot and excessive knee
extension when standing (Molnar & Alexander, 1998) (Fig. 2.7). Inhibitory and
progressive casting has gained acceptance as an alternative to bracing in recent
years. A molded footplate is constructed that inhibits the primitive reflexes, thus
reducing spasticity. The footplate is surrounded by a snug, bivalve below the
knee cast. Inhibitory and progressive casting also is used with the upper
extremities.
Figure 2.7 Child wearing knee-ankle-foot orthosis (KAFO). (From Hatfield, N.
T. (2013). Introductory maternity and pediatric nursing. Philadelphia, PA:
Surgical approaches are used to improve the function and appearance of affected
areas of the body and to prevent or correct deformities. Common surgical
procedures include tendon lengthening to increase range of motion and tendon
transfers to decrease spastic muscle imbalances. These procedures, commonly
used on the lower extremities, are performed more selectively in the upper
extremities (Steinbok, 2006). Selective dorsal rhizotomy (SDR) is a
neurosurgical technique that is used to reduce spasticity and improve function in
carefully selected individuals (Berman, Vaughan, & Peacock, 1990; The
Cleveland Clinic, 2015; Feger, Lunsford, Sauer, Novicoff, & Abel, 2015;
Kinghorn, 1992; Peacock & Staudt, 1991). The procedure involves dividing the
lumbosacral posterior nerve root into four to seven rootlets. Each rootlet is
stimulated electrically. The dorsal rootlets causing spasticity are cut, leaving the
normal rootlets intact. This approach is highly successful for individuals who
meet the selection criteria (Berman et al., 1990; Kinghorn, 1992). The most
likely candidates are children with diplegia or severe quadriplegia (Berman et
al., 1990; Kinghorn, 1992; Peacock & Staudt, 1991). For children with diplegia,
the goal is to improve gait and leg function. For children with spastic
quadriplegia who have very limited movement, the goal is to increase their
independence by allowing them to sit for longer periods of time enabling them to
use a wheelchair or potty chair as well as making daily care easier for their
caregivers by reducing spasticity, which makes dressing and other daily living
tasks more manageable. An essential part of this treatment approach includes
intensive postsurgical physical and occupational therapy for a period of several
weeks.
Impact on Occupational Performance
Virtually all of the body function categories can be affected in the individual that
has cerebral palsy. Which of the categories are affected depends on the type of
cerebral palsy, the severity of the condition, and the presence of associated
disorders. Milder forms of cerebral palsy may have limited impact upon
occupational performance. Some individuals will require physical assistance,
additional training, or assistive technology to participate fully in occupational
performance areas, while individuals with severe forms of cerebral palsy will be
limited in their performance of all areas of occupation. The body function
category that is always affected in individuals with cerebral palsy is
neuromusculoskeletal and movement-related function. If spasticity is present, it
affects joint mobility and results in limited active or passive range of motion or
both. Joint stability is affected in all types of cerebral palsy. With spastic type,
uneven muscle pull affects stability and joint cocontraction; fluctuating
movement and tone affects stability in athetoid type; and ataxic type is
characterized by a lack of joint stability. Underlying the tone abnormalities is
decreased muscle power. Tone abnormalities affect muscle endurance. It requires
much more effort to complete motor tasks with both hypotonia and hypertonia,
and endurance is often diminished. Endurance is also decreased if respiratory
muscles are affected. In movement functions, primitive motor reflexes such as
the stretch reflex, asymmetrical tonic neck reflex (ATNR), or grasp reflex are
retained in the child with spasticity and continue to influence movement
throughout life. Individuals with all types of cerebral palsy have impaired
involuntary movement reactions such as decreased righting and equilibrium
reactions. Involuntary movements impact individuals with athetoid cerebral
palsy. Some individuals with ataxia have intention tremors. Gait patterns are
affected in all types of cerebral palsy; however, in milder cases, they may have
little effect on occupational performance.
All sensory functions can be affected. All types of cerebral palsy have
associated visual difficulties such as strabismus, visual tracking and fixation
difficulties, or hearing impairments. Some individuals with cerebral palsy
demonstrate sensory processing difficulties with either hypersensitivity to
sensory input or their ability to discriminate sensory input.
Mental functions, both global and specific, can be affected, particularly if
there is an associated learning disability, attention deficit hyperactive disorder, or
cognitive impairment. Voice and speech functions can be affected if the oral
motor and respiratory muscles have tonal abnormalities. Functions of the
cardiovascular and respiratory system can be affected in a variety of ways, such
as associated spinal deformities that can compromise respiration or decreased
physical endurance or stamina as a result of the amount of effort that it takes to
move. The digestive system can be affected by a number of different medical
and physical factors such as reflux and feeding difficulties. Urinary functions are
impacted by decreased control of muscles used in urination and in some cases
cognitive factors.
It is important for the occupational therapist to be aware of all the client
factors that can be affected in individuals with cerebral palsy but to not make
any assumptions based upon the type of cerebral palsy and known associated
disorders but to directly assess each factor and its impact on occupational
performance. Each individual is unique and will have their own set of strengths
and challenges.
CASE-STUDY 1
A.K. is a 2-year-old girl who lives with her parents and older brother. She
was born at 37 weeks of gestation at a birth weight of 5 lb 10 oz. Pregnancy
and birth were unremarkable. She was healthy at birth, but by her well-baby
visit at 9 months of age, she was not yet rolling, crawling, or sitting
independently. Her pediatrician referred her to a pediatric neurologist who
diagnosed her with spastic diplegia at 11 months of age. The neurologist
referred her to a physical medicine and rehabilitation (PM&R) physician at
the local children’s hospital as well as the local school district for early
intervention services. The PM&R physician signed a referral for her to the
orthotics department to fit her with AFO’s (ankle-foot orthosis) to help her
with standing and walking. He has also recommended botulinum toxin
(Botox) injections and selective posterior rhizotomy for consideration as
future treatments. Through the early intervention program, she received a
multidisciplinary team assessment, which included physical, occupational,
and speech and language assessments. Delays were noted in gross motor,
fine motor, and self-help skills. Speech and language, social, and cognitive
skills were all determined to be at age level. Weekly occupational and
physical therapy home-based services were recommended.
Affected performance skills are in the motor area, which includes
posture, mobility, coordination, and strength/effort. She demonstrates
spasticity in all lower extremities and her trunk. Her movement patterns
reflect significant spasticity in her legs. Her joint range of motion is
significantly limited in her hamstrings and hip adductors with mild
limitations in her heel cords bilaterally. A.K. can sit independently;
however, it is difficult for her to sit on the floor with her legs extended in
front of her as a result of hamstring and hip adductor tightness. She requires
assistance moving in and out of sitting. In prone, she can push up to hands
and knees and can crawl for short distances. She bears weight on her legs in
supported standing with knees in a slightly flexed position, hips adducted,
and feet plantar flexed and pronated. She has begun ambulating with a
walker for short distances.
In her upper extremities, there is mildly increased muscle tone
bilaterally as well as some incoordination. A.K. grasps pegs and small
blocks and releases objects into a container. She can place a peg in a
pegboard but is not yet able to stack objects or complete a shape sorter.
Areas of occupation that are affected include activities of daily living
(ADLs) and play. Because she is only 2 years old, instrumental activities of
daily living, student, work, and leisure areas of occupational are not yet
relevant areas for her. In ADLs, because of her age, she would not yet be
expected to be independent. Given this, A.K.’s affected activities of daily
living include bathing, dressing, feeding, and functional mobility. A.K.
needs assistance with dressing skills such as undressing and removing shoes
and socks. She is independent in feeding with some adaptations. She
requires assistance with maintaining a stable sitting position in the bathtub
and requires assistance getting in and out of the bath tub. She needs
assistance with functional mobility, such as getting in and out of chairs and
moving from one place to another.
Exploratory play skills are affected by A.K.’s difficulty with moving
about her environment to obtain toys she wants to play with. Participation is
affected by the need for a stable position in which to free up her upper
extremities to manipulate toys. She uses a bench with a pelvis stabilizer and
tray for refined fine motor tasks.
CASE-STUDY 2
L.N. is a 54-year-old woman with cerebral palsy, spastic quadriplegic type.
She has lived alone in an apartment complex for the elderly and disabled
for the past 15 years. She supports herself on supplemental security income
(SSI) and disability payments from the state. A personal care attendant
provided by the Department of Social Services comes in each morning and
evening to assist her with activities of daily living, such as meal
preparation, bathing, and dressing. L.N. has never been employed but has
done volunteer work. She writes articles for a newsletter on her computer
and has worked in her church’s Sunday school. She has no family support
but has many friends. She enjoys learning and taking classes through
continuing education.
Spasticity, fluctuating tone, and retained primitive reflexes severely
restrict L.N.’s purposeful movement. She has limited range of motion in her
left upper extremity and both lower extremities. When reaching with the
left arm, she cannot bring it to shoulder height or behind her back. She has
a gross grasp in her right upper extremity and can grasp a joystick to
operate her electric wheelchair. She cannot write or perform other activities
requiring fine motor dexterity. Her left upper extremity is used as an assist
for bilateral activities, with no grasping ability present. She can maintain an
upright position in sitting, but her weight is shifted to the left (with resulting
scoliosis). She can bring her head to an upright position, but neck flexion
increases with activities requiring effort. Oral motor muscles are affected,
resulting in severe dysarthria, drooling, and difficulty eating. Endurance is a
problem, and L.N. becomes easily fatigued.
Communication/interaction skills are also affected. Articulation and
modulation when speaking are affected by L.N.’s oral motor control.
Limited dexterity and restrictions in movement limit her ability to use
gestures and to orient her body in relation to others when engaged in social
interactions.
All areas of occupation are affected. In activities of daily living, L.N.
needs assistance with bathing, personal hygiene and grooming, toilet
hygiene, and dressing. She brushes her teeth and performs light hygiene,
such as washing her face, independently. She can transfer herself between
her wheelchair and her bed. She needs assistance transferring to the shower
seat she uses for bathing. She can transfer on and off the toilet in her
apartment with grab bars and the toilet seat at the proper height and
position, although it takes her a while to do this. In eating, L.N. can feed
herself with adaptations if the food is set up for her, but the process is slow
and messy. She drinks from a straw. She takes her own medications if they
are set out for her.
In instrumental activities of daily living, L.N. needs assistance in
clothing care, cleaning her apartment, household maintenance, and meal
preparation. She can use a handheld portable vacuum cleaner for small
cleanups. She has a cat that she cares for. She shops independently but
needs assistance getting money out of her wallet at the cash register. All
areas of activities of daily living are affected except socialization. L.N. uses
a computer for written communication. She uses a speaker phone for
telephone communication. If she falls or is in danger at home, she has an
emergency alert system that she can activate. Because her speech is difficult
to understand, she has an augmented output device for communication but
uses it infrequently. She uses a motorized wheelchair for mobility. In the
community, L.N. uses public transportation with no difficulty. She has some
difficulty transferring herself to and from the toilet when using public
restrooms, which sometimes results in incontinence.
In work activities, L.N. has never been employed but has worked as a
volunteer for the past several years in the religious education program at her
church. She enjoys the interaction with the children who are in the classes.
In leisure activities, L.N. has varied interests. She is an avid reader and
enjoys computer games. Social activities include getting together with
friends frequently and going out into the community, either alone or with
friends. L.N. participates in church retreats as well as community-based
trips through an independent living center.
RECOMMENDED LEARNING
RESOURCES
Bowers, E. (2008). Finnie’s handling the young child with cerebral palsy at
home (4th ed.). Waltham, MA: Butterworth-Heinemann. This book was written
with the aim of helping parents assist their child with cerebral palsy achieve
most comfortable independence in all activities and assists professionals new to
the field in understanding, supporting, and encouraging young children with
cerebral palsy and their families.
Levitt, S. (2003). Treatment of cerebral palsy and motor delay (4th ed.).
Oxford, UK: Blackwell Science LTP. This book was written for occupational
and physical therapists working with children with cerebral palsy. Good
discussion of treatment approaches, principles of treatment, and description of
procedures.
Miller, F., & Bachrach, S. (2006). Cerebral palsy: A complete guide for
caregivers (2nd ed.). Baltimore, MD: John Hopkins University Press. Written by
a team of experts, the book is organized into three parts: explanation of the
condition, practical advice regarding caregiving, and an extensive encyclopedia
of medical terms, procedures, and devices related to cerebral palsy.
Morris, S., & Klein, M. (2000). Pre-feeding skills (2nd ed.). Tucson, AZ:
Psychological Corp. Excellent reference for oral-motor and feeding therapy for
children. Very thorough and good overall approach to feeding issues including
those occurring in children with cerebral palsy.
United Cerebral Palsy National Office 1660 L Street, NW Suite 700
Washington, DC 20036
Tel: (202) 776-0406; toll free: (800) 872-5827
ucp.org
Leading source of information on cerebral palsy and national advocacy
group.
Children’s Hemiplegia and Stroke Association (CHASA) 4101 West Green
Oaks Blvd.
Suite 305-149
Arlington, TX 76016
Tel: (817) 492-4325
www.chasa.org
A comprehensive, practical resource related to children with hemiplegia–
type cerebral palsy.
National Institute of Neurological Disorders and Stroke (NINDS). Cerebral
Palsy Information Page. Retrieved from
www.ninds.nih.gov/disorders/cerebral_palsy.htm. Lists resources including
organizations, publications, links, and general information about cerebral palsy.
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C H A P T E R
3 Autism Spectrum Disorders
Michelle A. Suarez Kathryn Ellsworth
KEY TERMS
Ambiguous hand preference Amygdala
Auditory processing Autism spectrum disorder (ASD) Casein
Cerebellum
Echolalia
Food selectivity Gastrointestinal (GI) disorder Gene
Gluten
Hippocampus
Hyperlexia
Inferior olive Joint attention Limbic system
Neurons
Purkinje cells Restrictive and repetitive behavior Rote memory
Sensory processing disorder Theory of mind
Rachel was a beautiful firstborn daughter, and during her first year of life, she
appeared to be a typically developing child. She responded to people and objects
in her environment with interest and enjoyment. Though slightly delayed in
learning to walk, she mastered this skill between 14 to 16 months of age. In
contrast, many of her language skills developed strongly during her first year.
Rachel was exposed to two different languages in her home and learned several
vocabulary words in each language by the time she was 14 months old.When
Rachel was approximately 14 months old, her parents noticed a startling change
in her behavior. Eye contact became rare, and she no longer turned in response
when her mother called her name. As a result of this decreased responsiveness,
her parents worried she was losing her hearing. However, an audiologic test
indicated normal hearing abilities. Dressing this little girl became a challenge.
Suddenly, she did not easily tolerate the sensation of clothes against her skin.
She became intensely distressed when her mother tried to brush her hair or clip
her nails. The vocabulary of approximately 30 words that she had previously
developed was replaced by silence or babbling. Upon reflection, her mother
realized Rachel had never used specific communicative gestures such as waving
to greet others or pointing. Engaging Rachel in daily activities became
increasingly difficult for she appeared frustrated, unable to express her needs and
wants, and unable to leave familiar, preferred activities without becoming highly
agitated. During these episodes of tantrums, Rachel would hit herself or bang her
head repeatedly against a wall while crying. Her mother described that overall,
“she no longer seemed happy.” When Rachel was 2 years and 6 months old, her
parents brought their concerns to the attention of early intervention specialists.
As a result, Rachel began receiving occupational therapy, speech-language
therapy, and special education services. During her preschool class, these
specialists observed the same concerning characteristics her parents had
described. Additionally, they noted she did not appear interested in the other
children in her preschool class. Transitioning from room to room consistently
distressed her, causing “meltdowns” during which she flung herself on the floor
and cried inconsolably. She used only a few real words or echoed words of
others but did not appear to understand what they meant. Many times, the
classroom environment seemed to provide her with far too much sensory input.
As a result, she would close her eyes or seek out places away from others. Yet
Rachel still showed moments of attachment and joy, such as a strong, loving
connection toward her parents and grandparents. Additionally, she smiled,
laughed, and shared eye contact during specific activities, such as swinging,
playing peekaboo, or singing.Despite these moments of engagement, the changes
and gaps in her development caused her parents and therapists to seek further
neurodevelopmental testing. Rachel was therefore assessed by a team of
specialists including a developmental pediatrician, a psychologist, a speech and
language pathologist, and an occupational therapist who confirmed the
suspicions of her family and therapists: Rachel presented with autism spectrum
disorder.
Description and Definitions
Autism spectrum disorder (ASD) is characterized by impairments in social
interaction and social communication and by the presence of restricted and
repetitive behaviors (American Psychiatric Association, 2013b). Individuals with
this developmental disorder often have difficulty with things like participating in
conversation or may misread nonverbal social cues from others. This can make it
difficult to make friends and interact with peers. In addition, people who have
ASD can have unusual responses to sensory information (e.g., tactile, auditory,
visual stimulus) or be highly sensitive to changes in their environment or overly
dependent on routines.
The Diagnostic and Statistical Manual of Mental Disorders, by the
American Psychiatric Association, provides differential diagnosis criteria for
autism. In 2013, the Diagnostic and Statistical Manual of Mental Disorders,
Fifth Edition (DSM-5) was published and provided major changes to the way
individuals with autism are diagnosed. In the earlier manual, the Diagnostic and
Statistical Manual of Mental Disorders, Fourth Edition (DSM-IV), individuals
could be diagnosed with one of five main disorders under the autism spectrum
umbrella. These disorders included autistic disorder, Asperger’s disorder (also
known as Asperger’s syndrome), pervasive developmental disorder not
otherwise specified (PDD-NOS), childhood disintegrative disorder, and Rett’s
disorder (American Psychiatric Association, 2000).
In a major departure from the presence of multiple categories of autism, the
DSM-5 provides for one universal ASD diagnosis. The rational for this change
was the inconsistent and inaccurate use of the DSM-IV criteria (American
Psychiatric Association, 2013a, DSM-5 ASD Fact Sheet). Researchers found
that across clinics and professionals, there were few reliability data to support
this continued separation of five separate disorders. In the DSM-5, anyone with a
DSM-IV diagnosis should still meet the criteria for ASD. In addition, the DSM-5
provides new specifiers to more accurately describe the variants and severity of
ASD, theoretically making it of more practical use for defining a treatment plan
for an individual based on their specific presentation of this condition. For
example, an individual who was previously diagnosed with Asperger’s disorder
(a previous variant of autism where intelligence is normal, language skills are
intact, but social skills are impaired) might now be diagnosed with ASD with or
without intellectual impairment or with or without language impairment.
Children with ASD share the following diagnostic characteristics (American
Psychiatric Association, 2013b):
1. Impairment in social communication and social interaction that occurs over
several contexts 2. Restricted, repetitive behavior, interests, and/or
activities
A child with ASD is deviant from, as well as delayed in, typical patterns of
development. Some children evidence problems from birth, such as appearing
aloof and making far less eye contact than other infants. In other cases, the
characteristics of autism noticeably emerge between 12 to 36 months of age.
Recognition of symptoms at this age is often due to one of two reasons. During
the 12- to 36-month period, there is typically an explosion of language skills.
Children with ASD may have been developing more slowly overall, and then
this language burst fails to occur. Alternatively, 12- to 36-month-old children
with ASD may lose language skills that they had demonstrated during earlier
months in their development (Strock, 2004).
ASD is considered a “lifelong” disability, and there is no cure. However,
intervention to improve skills, particularly in challenging areas that significant
limit participation in meaningful occupation, can have a positive and powerful
impact on quality of life for individuals with this disorder.
Etiology
Though researchers believe that certain factors (e.g., genetics) are more likely
than others to cause ASD, no one etiologic factor has clearly emerged.
Therefore, ASD is diagnosed based on observed behaviors using diagnostic
criteria. Autism was first described in literature in 1943 by Dr. Leo Kanner
(Kanner, 1943), and his initial hypotheses was that autism was caused by “cold”
or unresponsive parents (Bettelheim, 1967). This hypothesis has been proven
false and summarily rejected. Rather, autism is a neurologic disability (Lord &
McGee, 2001) that likely originates in utero due to errors in genes causing
abnormalities in brain structure and function (Autism Society of America, 2004).
Abnormalities in Brain Structure/Function
Children with ASD develop, process, and react differently to their world than
typically developing children. This diversion from normal development may be
due to the possibility that children with autism begin life with brains that are
physically different from those of typically developing children. Additionally,
the way in which life experiences are mapped onto the brain is altered as a result
of these physical differences (Siegel, 2003). The precise distinctions are not yet
entirely clear, since autism is not caused by a single obvious lesion in the brain.
In fact, studies have indicated subtle differences in several areas of the brain
with researchers unsure which area, or combination of areas, results in the
manifestation of autism (United Kingdom Medical Research Council, 2001).
Through the study of postmortem brain tissue and imaging studies such as
positron emission tomography (PET) scan and magnetic resonance imaging
(MRI), consistencies have emerged in the research of brain abnormalities.
1. The brain is made up of about 100 billion neurons, or single cells, that
interconnect and communicate information among the various brain
regions and from other areas of the body. The behaviors of autism may
result from abnormalities in the neural networking between the multiple
areas of the brain, rather than in one area, as the brain processes complex
information (Piven, Saliba, Bailey, & Arndt, 1997).
2. Though children with autism are born with normal head circumferences,
increased brain volume and head circumference become evident in
children with autism when they are 3 to 4 years old (United Kingdom
Medical Research Council, 2001).
3. There is a growing body of research that suggests that dysfunction of the
cerebellum is a likely contributor to autism. This area of the brain has been
traditionally known for its role in the coordination of movement. But the
cerebellum, which sits over the brainstem, may also play a role in sensory
discrimination, attention, emotions, mental imagery, problem solving, and
some aspects of language processing; the speed, consistency, and
appropriateness of mental and cognitive processes; and visual-spatial
orientation, spatial orientation, and visuomotor function (Bauman, 2004;
Kemper & Bauman, 1998). Neurons in the cerebellum known as Purkinje
cells form a layer near the surface of the cerebellum and convey signals
away from the cerebellum. In autistic individuals, an important deviation
consistently noted is a decrease in the Purkinje cell number.
4. In the brainstem, the neurons in the inferior olive of individuals with
autism also showed deviations depending on age. Cells were initially
larger than normal but typical in appearance in young children; in
adulthood, these cells were unusually small and pale (Kemper & Bauman,
1998; United Kingdom Medical Research Council, 2001).
5. Inferior olives are connected to Purkinje cells by climbing fibers, and this
bond from the inferior olive to the Purkinje cell is made at 28 to 30 weeks
of gestation. Once this union occurs, if a Purkinje cell dies, then the
inferior olive will also deplete. As previously noted, Purkinje cells are
reduced in number in children with autism, but the cells of the inferior
olive are normal in quantity. The implication is that damage to the
Purkinje cells in children with ASD would have likely occurred in utero
before the bond was established, prior to 30 weeks of gestation (Bauman,
2004; Kemper & Bauman, 1998).
6. Imaging studies have indicated reduced activation of the frontal lobe in
individuals diagnosed with autism (United Kingdom Medical Research
Council, 2001). Additionally, some areas of the frontal lobe appear
markedly larger in children with autism compared with typically
developing children (Carper & Courchesne, 2005). The frontal lobe’s
function is voluntary control of the body’s movements. Specific regions of
this lobe are also responsible for social behavior, spontaneous production
of language, initiation of motor activity, processing sensory stimuli, and
then planning reaction as a result of the input, abstract thinking, problem
solving, and judgment (Kemper & Bauman, 1998). How the abnormalities
affect the frontal lobe’s ability to conduct these roles remains unclear,
though many symptoms of autism appear related to problems in frontal
lobe functioning (Fig. 3.1).
7. Within the limbic system, a network of structures that regulates emotion,
structures known as the amygdala and hippocampus, appear abnormal in
the brains of individuals with ASD.
Figure 3.1 Autism: Connectivity in the brain. The behaviors of autism may
result from reduced connectivity among the different regions of the brain. This
figure illustrates reliably lower functional connectivity for autism participants
between pairs of key areas during sentence comprehension tasks. Darker end of
scale denotes lower connectivity (slide courtesy of Marcel Just, Carnegie Mellon
University).
“Memories, the desire to produce language, feelings, and the emotional coloring of thought are all
mediated by the limbic system. Anatomical systems necessary for cognitive functions, such as
language, spatial concepts, understanding of meaning in life, and so forth are all intimately linked to
the limbic system” (Helm-Estabrooks & Albert, 2003, p. 15).
Since the functioning of these abilities is impacted in individuals with autism, it

has been a logical step to focus research in this area. Thus far, anomalies include
decreased size of the neurons that make up the structures of the limbic system,
with a higher number of these neuronal cells packed into their respective spaces
(Kemper & Bauman, 1998) (Fig. 3.2).
Figure 3.2 Cerebral cortex. Left lateral view of the brain, including the four
lobes of the cerebrum and the cerebellum. (From Bickley, L. S., & Szilagyi, P.
(2003). Bates’ guide to physical examination and history taking (8th ed.).
Philadelphia, PA: Lippincott Williams & Wilkins.)
Scientists are persistently closing in on the differences in the brain structure and
function of this population. In the meantime, the question arises: What has
happened in a child’s system to cause these deviations in the brain to occur?
Parents of children with ASD, upon hearing their child’s diagnosis, typically
search for reasons why this condition occurred in their child. The professionals
who work with these parents must act as guides to discuss the most recent and
accurate information in the autism field. Parents are likely to question
professionals about many of the factors that will be discussed in the following
sections. It is important to share with parents those theories that are likely and
those that seem implausible based on current research.
Genetics
The differences in the neurobiology of children with autism are most likely
accounted for by genetics. Genes are composed of DNA and, through heredity,
determine the particular characteristics that distinguish one human from another.
Genes are encoded instructions—the brain’s written guidelines for function. If
any abnormalities lie in a brain’s genes, the brain deviates from a course of
typical development (Siegel, 2003). No single gene has been found to cause
ASD; therefore, current research is focused on finding a combination of genes
that may be responsible for an individual’s susceptibility to autism (United
Kingdom Medical Research Council, 2001; Veenstra-VanDerWeele, Cook, &
Lombroso, 2003).
Support for Genetic Etiology

In sibling studies, where one monozygotic (identical) twin was diagnosed
with autism, the other was also diagnosed somewhere on the spectrum in
90% to 95% of cases. For dizygotic (fraternal) twins, both children received
an ASD diagnosis in only 10% of cases studied. These statistics imply that
shared genetic codes significantly increase the likelihood of autism
occurrence (Rapin & Katzman, 1998; Veenstra-VanDerWeele et al., 2003).
In the general population, an individual has a 0.2% chance of having autism
(Veenstra-VanDerWeele et al., 2003). Siblings of children with autism,
however, are at a higher risk for presenting somewhere on the autism
spectrum. For parents, the probability of having a second child with autism
is 3% to 7% if the first child is male and has autism. If the first child is
female with autism, the likelihood of a second child with autism increases
to approximately 7% to 14% (Rapin & Katzman, 1998). A higher rate of
speech-language disorders has also been found among these families
(Rutter, Silberg, O’Connor, & Simonoff, 1999), suggesting that these
siblings received some, but not all, of the genes responsible for autism
(Siegel, 2003).
Relatives of these children more commonly displayed traits of autism (e.g.,
anxiety, aloofness) than did relatives of other children. The presence of
these traits in family members may be genetically linked to the
manifestation of autism (Jick & Kaye, 2003).
Additional weight is given to the genetic link since a disproportionately
higher number of males are diagnosed with autism, with three to four males
diagnosed for every one female (Rapin & Katzman, 1998).
Because specific genes have not yet been identified as the cause of autism,
current research is exploring other factors as possible etiologies or covariables.
Researchers are also considering that autism may be caused by a different
combination of variables for each child on the spectrum.
Environmental Factors
Environmental factors are external influences that may cause damage to people’s
systems if they are overexposed or exposed during a period of critical
development. The United Kingdom Medical Research Council (2001) reviewed
the following environmental risk factors as possible causes of autism: prenatal or
postnatal exposure to viruses, infections, drugs/alcohol, endocrine factors, and
carbon monoxide. However, these factors are thus far not significantly linked to
ASDs. In terms of obstetric complications, most mothers reported entirely
normal prenatal or perinatal experiences. Researchers have paid particular
attention to the measles-mumps-rubella (MMR) vaccination (which protects
against those diseases) in recent years, with some researchers speculating that
the mercury in this vaccination is responsible for causing changes in a person’s
system that lead to the symptoms of autism. When further investigated, a valid
association between the MMR vaccine and autism was not found. Though
exposure to mercury may lead to impairments that look similar to those found in
autism, children with autism have not consistently evidenced elevated levels of
mercury in their systems. In contrast, children with behavioral and/or
developmental difficulties were discovered to have a higher level of lead in their
systems, though it is unclear if exposure to lead results in autism (United
Kingdom Medical Research Council, 2001).
Physiologic Abnormalities
Children with autism need to be closely monitored for additional medical
concerns, since many of these children are not able to clearly express a medical
problem. For those children who are nonverbal, acting out with inappropriate
behaviors may be their only form of expressing discomfort. If a child
demonstrates sudden aggressive, severe, or unusual behaviors, or awakens
frequently from sleep, then medical attention by an experienced physician is
necessary (Horvath, Papadimitriou, Rabsztyn, Drachenburg, & Tildon, 1999).
One medical condition a child with autism may experience is a
gastrointestinal (GI) disorder, which is a disorder of the digestive tract.
Unusually high rates of GI disorders in children with autism have been
documented in recent years, including GI reflux, gastritis, persistent gas,
diarrhea, and constipation (Horvath et al., 1999; Levy, Mandell, Merhar,
Ittenbach, & Pinto-Martin, 2003, p. 418; McQueen & Heck, 2002). These
problems should be treated by a gastroenterologist, a physician who specializes
in GI disorders. A common, though unproven, theory hypothesizes that children
with autism experience abnormal digestion of gluten, a mixture of proteins that
may be found in wheat and other products (e.g., some snack foods or
delicatessen meats), and casein, a protein found in cow’s milk. This theory
suggests that children with autism lack an enzyme that efficiently digests these
substances; as a result, opioid or morphine-like substances accumulate and may
be the reason these children socially withdraw or engage in repetitive behaviors
(Levy & Hyman, 2003; McCandless, 2003).
It has been widely debated that children with autism have increased
vulnerability to immune system disorders. While this idea has gathered interest,
similar to theories of food allergies, there is a current lack of research to prove or
disprove this factor.
Combination of Factors
It is plausible that the interaction of gene susceptibility and environmental
factors gives rise to autism. In other words, an individual may be genetically at
risk for this disorder, but in order for characteristics to appear, an individual
would also need to be exposed to a yet-to-be-identified environmental factor
(United Kingdom Medical Research Council, 2001).
ASD impacts approximately 1% of the world’s population (CDC, 2014), and in
the United States, 3.5 million Americans live with this disability (Ostrow, 2014).
For reasons that are not entirely clear, the prevalence of autism has been rising
steadily and increased by 119.4% from the year 2000, where 1 in 150 were
diagnosed with autism, to the year 2010 where 1 in 68 children were diagnosed
(CDC, 2014). This makes autism the fastest growing developmental disability.
Though this increase has raised speculation of a possible “epidemic,” the rise in
prevalence likely results from better identification and availability of services,
along with changes in diagnostic criteria that allow more children to fit the
diagnosis of autism (Fombonne, 1999; Jick & Kaye, 2003; Rapin, 2002).
The likelihood of an autism diagnosis can vary by community and by
demographic characteristics (CDC, 2014). For example, only 1 in 175 children
were diagnosed in some areas of Alabama whereas 1 in 45 were diagnosed in
New Jersey. In addition, white children are much more likely to be given an
autism diagnosis than black or Hispanic children. This may be a reflection of
availability of services in rural versus urban areas and availability of resources to
some underserved populations. Finally, boys are just about five times more likely
to be identified with ASD than girls. This difference may be due to differences in
the biological structures of the male and female brain. The concept of autism
being caused by “extreme maleness” has been explored (Baron-Cohen,
Knickmeyer, & Belmonte, 2005). This theory relates to the population generality
that the male brain is more geared toward “systemizing” and the female brain
generally more geared toward empathizing. Overall, prevalence information can
be useful to inform policy and education of health care professionals so that all
children on the spectrum have access to services that can impact quality of life.
Incidence, or the measure of new cases per year, is best studied in disorders
that have a clear onset. Because the age of onset in autism is usually unclear,
accurate incident rates are difficult to measure. Estimates range from 2.1 to 8.3
per 10,000 for all children diagnosed with an ASD (United Kingdom Medical
Research Council, 2001).
Signs and Symptoms
Children with ASD all have difficulty with social communication and interaction
as well as restricted and repetitive behaviors. However, the severity and
functional presentation of these symptoms vary widely from one individual to
the next. In addition, these core behavior deficits do not encompass the entire
picture for these complex human beings. Motor abnormalities, sensory
processing disorders, feeding disorders, and co-occurring medical disorders
(e.g., seizures, sleep disturbances, GI problems) are only some of the additional
concerns that arise in this population. Every child with autism displays a
separate matrix of strengths and challenges with individual differences resulting
from personality and experiences (United Kingdom Medical Research Council,
2001). Core symptoms, described below, are drawn from the DSM-5 diagnostic
criteria for ASD (American Psychiatric Association, 2013b).
Core Symptoms
The first of the core symptoms, difficulty in social interaction, includes limited
use of eye contact, facial expressions, and social gestures (e.g., pointing,
shrugging, reaching arms up to be lifted). Additionally, children on the autism
spectrum are usually challenged to accurately interpret the body language and
facial expressions of other people. A child with ASD may not seek out others to
share enjoyment, share interest in the same objects with peers, or look for
approval or reassurance from parents. Moreover, individuals experience
challenges in developing friendships with same-age peers. These obstacles range
from showing a lack of interest in others to desiring friends but having difficulty
understanding how to relate appropriately to peers.
Problems with interaction may be partially due to the fact that children with
autism are hypothesized to have limitations in theory of mind, which is the
ability to understand another person’s thoughts, feelings, or intentions. It is how
an individual “reads” someone’s thoughts, understands another person’s
perspective on an issue, and predicts another’s feelings (Leslie & Frith, 1987).
Without this ability, a person is not able to predict or understand the actions of
others. Other social disturbances that manifest in children with autism include a
preference for playing alone, limited or repetitive play routines, and limited or
absent pretend play skills (National Research Council, 2001; Rapin, 1991).
Social communication and language impairments in the autism population
include delays in language development as well as abnormalities in language
use. Typically, developing children progress through several communication
milestones in their first few years (Strock, 2004). During a child’s first year of
life, a toddler gazes at others with interest, begins babbling, and reacts to sounds
and voices. By 12 months, gestures and first words emerge and the child shows
recognition of his or her name and some words and phrases. By 2 years of age,
the child has built a substantial vocabulary and combines words into two- to
three-word phrases. However, children with autism frequently show delays in
learning to speak, with some children unable to use words until their school-age
years (Strock, 2004). In severe cases of autism, spoken language may not be
acquired at all. A child’s ability to respond to voices or comprehend verbal
language (auditory processing) may also be impacted. Many parents report that
their child with autism does not respond when spoken to or called by name.
Social impairments further affect these children’s communication because they
may not be motivated to communicate with others, are typically challenged to
use or understand social cues such as intonation and facial expressions to
interpret meaning, and have difficulty maintaining the “give and take” of
conversations (e.g., a child may discuss only his or her topic of interest without
allowing others to take a turn).
The language of verbal children often appears even more peculiar because of
the presence of echolalia, pronoun reversal, and out-of-context words. Echolalia
is speech in which the child echoes back what he or she has previously heard.
Though all young children use echolalia as they are learning language, children
with autism retain this characteristic and often apply it in abnormal ways. It
results from poor auditory processing while auditory memory remains intact
(Siegel, 1996). Echolalia may be immediate, meaning that it occurs just after
another speaker’s utterance, or delayed, occurring significantly after hearing a
speaker (e.g., reciting an entire children’s book from memory an hour or day
later). Though it may appear unusual in conversation, children with autism
frequently use this inappropriate device in meaningful ways (e.g., reciting lines
from his favorite movie as a self-calming strategy when he feels anxious).
Because children with autism often fail to understand language, pronouns (e.g.,
“you” and “me”) are also often used incorrectly. Other times, these individuals
may use language in a context that seems out of place; for instance, saying “ball”
when actually requesting juice.
The second core feature of autism is in the area of restrictive and repetitive
behaviors. Individuals with ASD may exhibit an abnormal or intense
preoccupation with routines or patterns. For instance, a child may line up blocks
or toy cars for hours without playing with others or using these toys in pretend
play. Additionally, the child may scream or cry in reaction to someone joining
the child’s play or moving the lined-up toys. Obsessions with specific topics are
common, such as showing an unusual interest in elevators or numbers and letters
(Strock, 2004). Additional common characteristics include rigidity in daily
routines; abnormal, stereotyped behaviors such as repetitive hand-flapping or
body rocking; and unusual, nonfunctional preoccupation with parts of an object
(e.g., spinning the wheels of a toy car without showing interest in any other type
of play with this toy) (American Psychiatric Association, 2013a; Strock, 2004).
All children diagnosed with ASD demonstrate some combination of these
social, social language, and behavioral symptoms. However, the range of
severity and functional presentation of these symptoms is vast. The DSM-5
provides additional specifiers to more accurately capture the impact of these
symptoms on individual functioning that is useful for treatment planning and
research purposes. First, the presence or absence of intellectual disability,
language disorder, associated medical condition, and/or catatonia is captured.
Then, severity level for the two core symptoms is captured on a three-point scale
from mild to severe. Examples of function within this hierarchy, adapted from
the DSM 5, are included in Table 3.1.
TABLE 3.1 Levels of Severity within the Autism Spectrum

The DSM-5 provides for one umbrella diagnosis of ASD and offers new
categorical descriptors to pinpoint the specific presentation of this disorder for
an individual. Since the degree of functional impairment varies widely for
individuals with autism, this has the potential to allow for more individualized
treatment. However, the move from having five diagnostic categories (i.e.,
Asperger’s syndrome, pervasive developmental disability not otherwise
specified, childhood disintegrative disorder, Rett’s syndrome, and autism
disorder) is not without controversy. Detractors are concerned that diagnosis may
be more restricted and that people being newly diagnosed with autism may not
fit the new criteria and may not have access to services and support previously
provided (Kulage, Smaldone, & Cohn, 2014). The impact of this major change
will be followed for years to come, and the evaluation of the usefulness of this
new diagnostic structure will be determined by the successful identification of
children in need of services to mediate the functional limitations that are often
caused by symptoms of autism.
Co-occurring Conditions
The core symptoms of autism cause many challenges to engagement in everyday
life for individuals with ASD and the people who love and support them. These
challenges can be compounded by co-occurring conditions that are commonly
found in individuals ASD. Sensory processing disorder and possibly related fine
and gross motor deficits, food selectivity, sleep disturbances, and GI issues can
create additional stressors that profoundly influence the quality of life in this
population.
Sensory Processing Disorder
Hypo- and hyperreactivity to sensory information and/or unusual preoccupation
with the sensory aspects of the environment are included in the diagnostic
criteria in the DSM-5 under restricted and repetitive behaviors. However, many
children with autism have sensory processing problems that go beyond hypo-
and hyperreactivity. Also, beginning with the pioneering work of the prominent
occupational therapist Jean Ayres, sensory processing disorders are of special
interest to occupational therapists and are therefore covered separately here.
In the 1970s, Jean Ayres, an occupation therapist with training in
neuroscience and education, developed the theory of sensory integration to
explain learning differences in children who struggled with interpreting
sensation from their bodies and the environment (Ayres, 1994). Today, estimates
of atypical sensory processing in children with ASD range as high as 95%
(Baranek, David, Poe, Stone, & Watson, 2006). Sensory processing disorders are
organized into several subtypes that manifest in difficulty modulating sensory
experiences in order to maintain a calm alert state (i.e., under- or
hyporesponsivity [SUR], over- or hyperresponsivity [SOR], sensory seeking
[SS], or craving [SC]), difficulty with motor skills (i.e., dyspraxia and postural
instability), and difficulty with discrimination of the quality of sensory stimulus
(Miller, 2014). The severity and type of sensory processing problems in children
with ASD contribute substantially to success in daily functioning. Table 3.2
contains the sensory processing disorder subtypes with behavioral examples and
the connection between the subtype and children with ASD.
TABLE 3.2 Sensory Processing Disorder in Children with ASD

Fine and Gross Motor Impairment
As described in the table above, children with autism have motor impairments
that may be related to their ability to use sensation to plan, guide, and execute
movement. Fine and gross motor impairments include problems in skilled
movement, hand-eye coordination, speed, praxis and imitation, posture, and
balance (Dawson & Watling, 2000). A particularly debilitating abnormality in
individuals with autism includes deficits in motor imitation skills despite intact
perceptual and motor capacities, which are most apparent in younger groups of
children (Williams, Whiten, & Singh, 2004). Furthermore, these children
perform poorly on tasks of executing a sequence of movements, such as a
sequence of hand or facial actions in imitation (Hughes, 1996). Reduced stride
lengths, increased stance times, increased hip flexion at toe-off, reduced knee
extension at initial ground contact, abnormal heel strikes, and decreased knee
extension and ankle dorsiflexion at ground contact were noted in an earlier study
of children with autism (Vilensky, Damasio, & Maurer, 1981).
In addition, a disproportionate number of children with autism display
ambiguous hand preference (~40%) long past the age that dominant hand
preference typically develops. A person who is ambidextrous will usually choose
one hand for a specific task (e.g., left hand for writing, right hand for throwing a
football). An ambiguous hand preference, however, refers to switching hands
within the same activity (Hauck & Dewey, 2001). When this behavior persists
into the school-age years, it may indicate abnormal functioning of the brain.
Food Selectivity
Children with ASD often have an extremely self-restricted diets and disruptive
behaviors during mealtime (Schreck, Williams, & Smith, 2004). These children
may refuse to eat more than 5 to 10 very specific foods. For example, a child
with ASD might insist on eating a specific brand and shape of macaroni and
cheese at every meal. They may insist that food be arranged on the plate a
particular way with foods not touching other foods. They may refuse to eat any
foods from a particular food group and have meltdowns and/or gag or vomit
when encouraged to eat a nonpreferred food. It is common for children with
autism and food selectivity to refuse to eat any fruits and vegetables (Suarez &
Crinion, 2015). This behavior causes worry and stress for caregivers and can
make mealtime unpleasant for the entire family (Suarez, Atchison, & Lagerwey,
2014). Also, depending on the degree of food refusal, food selectivity can
threaten health and development due to lack of consumption of essential
nutrients (Bandini et al., 2010).
Sleep Disturbance
Poor quality sleep is common in the ASD population and up to approximately
70% have some form of sleep disturbance (Souders et al., 2009). Children with
autism may resist going to bed and require extensive time to fall asleep (Liu,
Hubbard, Fabes, & Adam, 2006). They may have long periods of awakenings,
awaken many times during the night, or wake-up very early in the morning.
Causes for sleep disturbance are unknown but may be related to abnormalities in
the areas of the brainstem that regulate sleep and in melatonin levels (Malow et
al., 2012). Children with ASD who are poor sleepers have more difficulty with
daytime disruptive behavior than children who sleep well (Malow et al., 2006;
Wang et al., 2015). In addition, sleep disturbance in children with ASD has
implications for the sleep quality of the entire family. This can lead to additional
stress on a family already struggling with core symptoms of autism.
Since the symptom variability within the autism spectrum differs widely
between individuals, the course and prognosis depend on the individual’s
presentation. Many children with autism can have significant functional
improvement with treatment. However, most individuals tracked longitudinally
retain characteristics of the diagnosis throughout the span (Lord & McGee,
2001). Several prognostic indicators can partially predict functional outcomes
for individuals with this disorder.
Intellectual Disability
Some children with autism also have decreased mental capacity limiting their
ability to plan, problem solve, think abstractly, and/or comprehend complex
ideas (The Arc, n.d.). Intellectual abilities are measured with assessments that
provide an intelligence quotient (IQ) and scores below 75 indicate significant
impairment in thinking skills. The prevalence of intellectual disability in
children with ASD is likely in the 15% to 20% range (Fernell, Eriksson, &
Gillberg, 2013) with estimates ranging as high as 68% (Yeargin-Allsopp et al.,
2003). IQ is the number one predictor of later adaptive functioning in children
with autism (Fernell et al., 2013). For example, the previous DSM (IV) included
a diagnosis of Asperger’s, which was characterized by social and behavioral
challenges without presentation of cognitive deficits. Longitudinal studies
suggest that prognosis is better for the Asperger’s disorder with more individuals
able to function independently in employment and self-sufficient living
(American Psychiatric Association, 2000).
Language
A child’s language level is also a strong predictor of functioning over the course
of the lifespan. A child’s ability to spontaneously, meaningfully, and consistently
combine words into phrases or sentences before 5 years of age is a good
prognostic indicator of cognitive, language, adaptive, and academic achievement
measures (Lord and McGee, National Research Council, 2001). It is important to
observe that the child is able to spontaneously construct sentences rather than
echo others’ utterances (echolalia) or use memorized chunks of language to
communicate. In addition, a child’s use of joint attention has been found to be a
predictor of language outcome. Joint attention is the ability to use eye contact
and gestures in order to share experiences with others. Children who fail to use
early gestural joint attention (e.g., failing to point at an object and to turn to his
mother to determine if his mother shares his interest) seem to struggle in the
development of meaningful language. In Lord and McGee’s review of autism
research (2001), findings implied that early joint attention, symbolic play, and
receptive language were strong predictors of a child’s future outcomes. Another
study in this review examined severity of repetitive, stereotyped behaviors and
social symptoms and found that the severity later predicted adaptive functioning.
Motor Skills
Early success in hand-eye coordination may predict vocational abilities later in
life. Fine motor skills also predicted later leisure pursuits (Lord & McGee,
2001). Children with autism who displayed a definite hand preference performed
significantly better on motor, language, and cognitive tasks (Hauck & Dewey,
2001). The ability to imitate body movements has been linked to expressive
language development, and imitation of actions with objects predicted later
levels of play abilities (Lord & McGee, 2001). The more established behavioral
challenges become without intervention, the more these problems persist and
worsen.
Surgical/Medical Management
Diagnosis
Autism is now diagnosed at a younger age than ever before because
characteristics of autism have become more defined and better recognized.
Researchers have employed at least two research methods to identify early
indicators of ASD (Boyd, Odom, Humphreys, & Sam, 2010). Retrospective
studies, using home videos of children later diagnosed with autism, and
prospective studies of the younger siblings of children with autism (due to the
genetic likelihood of siblings both having autism) have been used to develop an
understanding of early behavioral warning signs. Key indicators include delay or
disorder in social behaviors like smiling socially or response to one’s name.
Coordination of verbal and nonverbal communication (e.g., verbalization with a
point or eye gaze, showing objects to others) is often difficult or absent in very
young children that are eventually diagnosed with autism. Finally, atypical play,
including spinning objects or preoccupations with parts of objects, can be an
early indicator. Receiving a diagnosis at an early age is optimal because the
sooner the disorder is recognized, the more likely the child can make dramatic
reductions in symptoms and gains in learning (Lord & McGee, 2001; Strock,
2004). In addition, parents are able to receive valuable support and education
from professionals and other family members.
A child is usually referred for an assessment because those who interact
closely with him (e.g., family members, pediatrician, teachers) may observe
warning signs either specific to autism or to a development delay in one or more
areas. When these concerns become apparent, the child is initially screened,
usually by primary care providers or early child care professionals, to look for
the “red flags” that may indicate autistic behaviors. Published screening
instruments for children with autism include
The Checklist for Autism in Toddlers (CHAT) (Baron-Cohen, Allen, &

Gillberg, 1992)
The Autism Screening Questionnaire (ASQ) (Berument, Rutter, Lord,
Pickles, & Bailey, 1999)
The Screening Tool for Autism in Two-Year-Olds (Stone, Coonrod, &
Ousley, 2000)
Australian Scale for Asperger’s Syndrome (Garnett & Attwood, 1998)
Pervasive Developmental Disorders Screening Test, Stage 1 (PDDST-I)
(Siegel, 1998a)
The Modified Checklist for Autism in Toddlers (M-CHAT) (Robins et al.,
2001).
If the child does not pass the screening, this indicates that enough warning signs
of autism are present to warrant a thorough assessment for autism. Since each
child on the ASD displays a unique matrix of strengths and weaknesses, no two
children on the autism spectrum will look the same. Therefore, critical to an
accurate diagnosis is an assessment with clinicians who are experienced in
identifying the characteristics of autism. Additionally, a thorough diagnosis with
a team of professionals can gather insight into each child’s skills across several
areas of development, which, in turn, helps with intervention planning. An
autism evaluation team frequently consists of a developmental pediatrician, a
psychologist, a speech and language pathologist, and often an occupational
therapist. The following elements should be included in every sensitive,
comprehensive evaluation of a child with autism:
1. History: Though autism is not known to result from complications during

pregnancy, it is important to discuss any unusual pre- or perinatal events to
rule out other disorders. Because of autism’s genetic implications, it is
important to determine if other family members have been diagnosed with
autism, psychiatric concerns, or developmental disorders. The history
portion should also include questioning about autism-specific behaviors in
the areas of social, language, behavioral, play, cognitive, and sensory
processing abilities (Rapin, 1997).
2. Medical history: During this portion of the assessment, parents report
when their child’s developmental milestones were reached (e.g., what age
their child said his or her first word, learned to walk), if regression of
developmental skills occurred at any point, or if any other medical
problems are occurring (e.g., psychiatric, sleeping, or eating problems)
(Filipek et al., 1999).
3. Physical/neurological examination: Other illnesses such as fragile X
syndrome, tuberous sclerosis, or congenital rubella need to be ruled out,
since these disorders may look similar to autism (Rapin, 1997). The
physician also checks for other medical illnesses (e.g., GI disorders, ear
infections), measures head circumference, gives a general physical
examination, examines mental status, verifies that cranial nerves function
normally, and performs a motor examination (Filipek et al., 1999).
4. Parent interviewing: Many diagnostic tools are available to gain parents’
insight into their child’s autism-specific behaviors. A clinician should also
ask parents about their overall impression of their child, since more
general questions may reveal further insight beyond the scope of these
tools (Filipek et al., 1999; Lord and McGee, National Research Council,
2001).
Parent Interview Tools:
The Autism Diagnostic Interview: Revised (Lord, Rutter, & LeCouteur,

1994)
Functional Emotional Assessment Scale (FEAS) (Greenspan, DeGangi, &
Wieder, 1999)
The Gilliam Autism Rating Scale (GARS) (Gilliam, 1995)
The Pervasive Developmental Disorders Screening Test, Stage 2 (PDDST-
II) (Siegel, 1998b)
5. Tests in language development. Examples of tools include the following:
Preschool Language Scales (PLS) (Zimmerman, Steiner, & Pond, 2011)

Clinical Evaluation of Language Fundamentals (CELF) (Wiig, Semel, &
Second, 2013)
6. Tests in cognitive development:
Mullen Scales of Early Learning (MSEL) (Mullen, 1995).

Wechsler Intelligence Scale for Children (WISC) (Wechsler, 2003)
Bayley Scales of Infant and Toddler Development, Third Edition (Bayley-
III) (Bayley, 2005).
7. Tests in other developmental areas including sensory processing, motor,

and adaptive skills.
Sensory Profile 2 (Dunn, 2014)

Peabody Developmental Motor Scales, Second Edition (PDMS-2) (Folio
& Fewell, 2000)
Quick Neurological Screening Test 3 (QNST-3) (Mutti et al.)
Vineland Adaptive Behavior Scales (Sparrow, Cicchetti, & Balla, 2006)
8. Formal and informal observation of the child interacting with others. This
is considered one of the most valuable pieces of the evaluation process,
since it typically reveals the qualitative impairments of the child (such as
lack of eye contact, limited initiation of interaction, or difficulty
transitioning between tasks). A diagnostic instrument should be used that
examines autistic behaviors while the clinician observes the child’s
interests and interactions with others. The Autism Diagnostic Observation
Schedule (ADOS) (Lord & Rutter, 2000) is considered a gold standard
instrument for this purpose.
9. Audiological testing: An audiologic evaluation is necessary to rule out
hearing disorders. Children with autism are often unresponsive to verbal
auditory stimuli, and it is important to determine that this behavior is not
caused by a hearing impairment. If hearing loss co-occurs with autism,
language comprehension may be further impacted than with a diagnosis of
autism alone (Filipek et al., 1999).
10. Other testing: Certain tests may prove beneficial to specific
circumstances. For instance, an electroencephalograph (EEG) may be
needed if the child is suspected of having seizures (Filipek et al., 1999).
Some children are assessed through university studies, and MRIs are
typically included in these autism assessments (Bauman, 2004; Filipek et
al., 1999). Genetics testing may be appropriate for parents who are
considering having another child (Filipek et al., 1999). Some parents are
also concerned about heavy metal contamination and wish to pursue a lead
screening for their child.
Medical/Surgical Treatment
Overall, treatment for autism does not heavily rely on medical intervention, and
surgical interventions are not practiced for this disorder. Intensive early
intervention is most effective in reducing problem behaviors while increasing
language, social, sensory, motor, and cognitive skills (Lord and McGee, National
Research Council, 2001; Rapin, 1997; Rogers, 1996). A variety of approaches
are available, ranging from highly structured to naturalistic. Because no two
children with autism present with the same set of symptoms, no one treatment
plan is successful for all children with autism. Effective intervention must
account for the child’s individual strengths and challenges and must consider
functional skills to be generalized across a variety of settings in the child’s life.
Substantial literature supports early intervention for children 0 to 3 years as the
most beneficial time to connect new pathways for more appropriate functioning
and behavior, though an individual may continue to make substantial gains
following this period of development (Lord and McGee, National Research
Council, 2001; Rapin, 1997; Rogers, 1996). Because it is beyond the scope of
this book to discuss these approaches, this chapter focuses on current knowledge
of medical interventions meant to accompany intervention techniques.
Pharmacologic Therapies
Use of medication does not cure the core social, language, and repetitive
behavioral deficits of autism because “in most cases, the brain has undergone
atypical cellular development dating from the earliest embryonic stages” (Rapin,
2002, p. 303). Research in this area is presently limited or inconclusive. In
addition, many parents and clinicians are cautious because use of
pharmacotherapy with children presents the risk of harmful side effects. Lindsay
and Aman (2003) reviewed existing literature on pharmacologic intervention and
found that when certain medications are used appropriately, behavioral
dysregulations such as hyperactivity, irritability, anxiety, and perseveration may
be reduced. For instance, risperidone, an atypical antipsychotic agent, shows
promising results in emerging research for reducing tantrums, irritability,
aggression, and self-injurious and repetitive behaviors (Gordon, 2002; Lindsay
& Aman, 2003; Pediatric Psychopharmacology Autism Network, 2002).
Because autism is likely a genetic disorder determined before the child is born,
pharmacologic treatments could probably not “undo” this disorder. Therefore,
these treatments are not investigated to replace educational services but rather to
supplement them. For those children who respond successfully to medication,
behavioral and educational intervention may be even more beneficial since they
do not struggle as greatly with challenging behaviors.
Medical Conditions
Medical intervention is necessary for any co-occurring medical conditions, such
as seizures or GI disorders. Children with autism need to be monitored closely
for behaviors that may reflect a medical condition and should receive a complete
medical workup and treatment through a physician who specializes in the child’s
medical condition.
Complementary and Alternative Medicine

Complementary and alternative medicine has gained popularity in the past
decade as a supplementary treatment to educational services. Complementary
and alternative medicine is defined as “a broad domain of healing resources
that encompasses all health systems modalities and practices and their
accompanying theories and beliefs, other than those intrinsic to the politically
dominant health system” (Levy et al., 2003, p. 418). Those who support
complementary and alternative medicine believe the methods target underlying
medical difficulties, such as GI and sleep disorders, which are not addressed
through educational intervention. The goal of many of the complementary and
alternative medicine treatments is to aid in the associated problems of autism,
rather than claim to cure the disorder. Statistics reveal that 30% to 50% of
children with autism in the United States are using complementary and
alternative medicine; however, approximately 9% are using potentially harmful
treatments, and 11% are using multiple complementary and alternative medicine
treatments (Levy et al., 2003). Several studies are currently underway to
examine the effectiveness and the risks of these treatments since many parents
have reported a decrease in associated problems (e.g., GI problems) and an
increase in developmental skills. Table 3.3 summarizes the potentially harmful
side effects of these methods. The following sections describe commonly used
complementary and alternative medicine treatments.
TABLE 3.3 Potential Side Effects of Complementary and Alternative

Medicine
Levy, S. E., & Hyman, S. L. (2003). Use of complementary and alternative medicine for children with
autistic spectrum disorders is increasing. Pediatric Annals, 32(10), 685–691.
http://dx.doi.org/10.3928/0090-4481-20031001-10. Reprinted with permission.
Supplements
The use of vitamins and minerals to address ASD concerns purports that because
children with autism experience GI inflammation and intestinal disorders, their
ability to absorb nutrients is thereby reduced. As a result, development that relies
on nutrients such as vitamins A, B1, B3, B5, biotin, selenium, zinc, and
magnesium is altered. Frequently used supplements include vitamin C, cod liver
oil (for vitamins A and D), and the combination of vitamin B6 with magnesium
(Autism Society of America, 2004).
Gluten-Free, Casein-Free Diet

Parents and professionals who support the theory that gluten and casein
negatively impact a child’s development recommend a diet that completely
eliminates these products (McCandless, 2003). Parent reports of improvement
have been inconsistent, as some parents claim to see significant improvements in
their child’s behavior and/or developmental skills (e.g., improved eye contact),
while others report that no change occurs by implementing this diet. Data is
currently limited and inconclusive. One review of five available articles
researching the effectiveness of gluten- and casein-free diets for behavioral
symptom management found no positive effect from this diet (Hurwitz, 2013).
Other Common Complementary and Alternative Medicine Treatments

for Autism
Secretin: The human body naturally produces secretin, which is a hormone
produced in the small intestine that stimulates secretion by the pancreas and
liver. The use of extra secretin through injections became a popular
complementary and alternative medicine treatment before it was
scientifically analyzed, and studies now show few changes for children with
ASDs who have used the extra hormone injection (Levy & Hyman, 2003).
Chelation: Because mercury poisoning produces symptoms similar to those
seen in autism, mercury and other heavy metals have been suggested as
causes of autism. Though research has not found a link between mercury
and autism, those who believe their children have experienced metal
poisoning may choose chelation, a process to remove toxins from a child’s
system (Levy & Hyman, 2003).
Antibiotic treatment: Immune system dysfunctions and antibiotic treatments
have been targeted as possible causes of autism, and those who believe in
these theories use further antibiotic treatment to alter the course of the
symptoms in autism (Levy & Hyman, 2003).
Antifungal treatment: Yeast overgrowth in the colon is hypothesized to
cause many medical disorders, including autism, with a low-sugar diet and
the use of probiotic agents (which encourage helpful intestinal bacteria)
used as treatments (Levy & Hyman, 2003).
The effectiveness of complementary and alternative medicine stands largely

unproven and highly controversial, though attempts are underway for further
studies since many parents report positive results by using these methods.
Complementary and alternative medicine treatments are intended to supplement
educational services rather than to replace them. Clinicians are currently
encouraged to use an empathetic stance with families providing complementary
and alternative medicine to their children, though no alternative method should
be administered without the guidance of an experienced physician.
The presence or absence of intellectual and language impairments, the severity
of the autism symptoms, and the influence of coexisting sensory, psychiatric, and
motor deficits all have significant implications on the degree of functional
impairment present in an individual with an ASD diagnosis. For example, ADLs
like dressing and eating can be difficult if the individual is overresponsive to the
sensations of clothing or food and/or has difficulty with maintaining the
necessary postural stability to support motor planning of these tasks. IADLs like
shopping or driving are difficult and even dangerous if the individual does not
have adequate focused attention and emotional regulation to make in the
moment safe decisions. Participation in the educational system can be hampered
by difficulty with memory functions for learning and socialization skills to
develop healthy relationships with peers. Play and leisure activities are
sometimes limited by restricted and repetitive behaviors, making participation
outside of the home stressful. Finally, individuals with autism often have
difficulty obtaining and maintaining employment due to the complex nature of
these tasks and the constellation of symptoms that make professional behaviors
challenging. In summary, ASD is a pervasive, lifelong disability that can impact
every area of occupational performance. However, intervention can facilitate
functional changes to significantly improve the quality of life of the individual
with autism and their family.
Global Impairment
Autism is considered a global impairment, meaning that it does not reflect
damage as a result of one specific lesion in the brain. Since autism likely affects
several regions of the brain, this global impact means that autism impairs
multiple areas of a child’s development. As a result, all areas of occupation are
often impacted by this disorder.
Specific Mental Functions
Each individual with autism presents with different areas of strengths and
weaknesses; therefore, individuals with an ASD diagnosis show various
combinations of concerns in their mental skills. Concerns in this area can range
from very severe, where the individual will need constant support and
supervision throughout the lifespan, to very mild, where the individual can
achieve complete financial and interpersonal independence. However, despite
the great variability in symptom presentation, several areas of mental function
are commonly impaired to a greater or lesser extent depending on the level of
ASD severity.
Children with autism often have difficulty regulating their own emotions and
often experience features of or possibly comorbid psychiatric disorders (Leyfer
et al., 2006). The most common issue is specific phobia. Children with autism
may experience phobias of typically innocuous things like crowds, bridges, or
loud noises. In addition, attention deficits are nearly universal in this population.
Unique patterns of attention include distractibility, disorganization, intense
preoccupation for preferred, self-initiated activities for unusual lengths of time,
and lack of boredom for repeating same action or play schema (Rapin, 1991).
Features of obsessive-compulsive disorder are also often present in many
children with autism. They may need to perform a routine a certain way, like
walking through the same side of a double door to get to the lunchroom at
school, or not be able to move on until a task is completed, like needing to pick
all of the dandelions on the playground before they can play. Finally,
approximately 10% of children with autism also have symptoms of major
depression with episodes of things like sadness, hopelessness, crying, and/or flat
affect (Leyfer et al., 2006). Difficulty regulating emotion in this population can
lead to emotional lability (e.g., swinging from laughing to crying without
apparent reason), heightened anxiety with temper tantrums, and/or aggressive
behaviors such as hitting and biting, self-injury (e.g., head banging, hitting self),
or self-stimulatory behaviors (body rocking).
A child with autism often shows a scattered pattern of memory functions
(Hill, Berthoz, & Frith, 2004). Overall, “memory performance of individuals
with autism becomes increasingly impaired as the complexity of the material
increases” (Minshew & Goldstein, 2001, p. 1099). Children with autism also use
fewer organizational strategies, relying on stereotyped rules regardless of the
task’s complexity (Minshew & Goldstein, 2001). Therefore, a child with autism
performs more poorly on tasks with higher complexity. Word recall is more
significantly impacted than digit recall, and these children often have difficulty
recalling activities in which they have recently participated (Boucher, 1981).
However, certain areas of memory remain intact, particularly in the areas of
visual and rote memory. Rote memory describes the memorization and use of
previously heard chunks of language rather than the spontaneous generation of
language.
In the area of perceptual functioning, individuals with ASDs perceive
sensory stimuli, but often process and react abnormally to it. Integration of
perceptual and sensorimotor information allows individuals to respond
appropriately with physical and emotional responses. In autism, this integration
does not occur in the same efficient way; therefore, this population of individuals
has difficulty responding with typical emotional and behavioral responses to the
sensory stimuli around them. Between 30% and 100% of individuals with autism
demonstrate deviant sensory-perceptual abilities (Dawson & Watling, 2000).
As mentioned above, cognitive impairments or intellectual disabilities are
common in, though not universal to, the autism population. Individuals with
cognitive deficits typically demonstrate scattered skills; in other words, they may
present with strong skills in some areas with significant concerns in other areas.
These children often have difficulty sequencing a series of items, imitating the
actions and words of others meaningfully, generalizing concepts across a variety
of situations, demonstrating theory of mind, and playing with toys appropriately
and symbolically (Lord and McGee, National Research Council, 2001). In her
1991 literature review of autistic features, Rapin described that children with
autism tend to have better visual-spatial skills than auditory verbal skills on IQ
tests. Children in this population may show above-average skills in very specific
areas, such as calculating numbers, completing puzzles, or demonstrating rote
verbal memory, while demonstrating overall cognitive impairment. Some
children with autism are able to read at a young age with minimal instruction,
but they have little or no understanding of what they read. This unusual
occurrence is known as hyperlexia (Rapin, 1991).
Studies examining higher level cognitive functions have revealed that
executive functioning skills such as forward planning, cognitive flexibility, and
the use of assistive strategies (e.g., creating a mnemonic such as a rhyme to
assist in remembering information) in learning are impacted for those who have
autism (Gordon, 2002).
As previously described, children with autism typically show language
delay. Deviations from typical language are noted across all diagnostic
categories. Children on the autism spectrum typically have limitations in using
language in appropriate contexts and for social purposes. Some children may be
highly verbal and articulate though literal, echolalic, and repetitive; other
children may remain nonverbal or use very little speech (Rapin, 1997).
Additionally, comprehension of language is affected and exacerbated by a
decreased motivation to use language for interacting with others. Often, these
children are challenged to understand what topics interest others and have
difficulty interpreting nonverbal language such as gestures and facial
expressions, as well as abstract language such as jokes or idioms. For those who
are verbal, intonation of their voices may sound unnatural as a result of a
singsong or monotone pattern. Speaking at unusually loud volumes is another
unusual characteristic of children with autism. Though the words may be clear,
content may be memorized chunks of language or may focus on topics that are
not relevant to a conversation partner. Echolalia is also common for verbal
children (Rapin, 1991). Some children do not master fluent speech and their
sounds in words are difficult to understand. Decoding the sounds that they hear
may be severely compromised; therefore, these children may not understand
what they hear and, in turn, are unable to produce the sound accurately (Rapin,
1997).
Sensory Functions and Pain
“The experience of being human is imbedded in the sensory events of everyday
life. When we observe how people live their lives, we discover they characterize
their experiences from a sensory point of view” (Dunn, 2001, p. 608). As
described above, sensory processing is particularly challenging in individuals
with autism and additional information is included here. If a child’s sensory
system does not interpret stimuli in a typical way, it is easy to understand why
this individual may react to the world differently. Sensory processing problems
should be addressed through early intervention to reduce these abnormal
behaviors (Lord and McGee, National Research Council, 2001; Dunn, 2001).
Visual perception is usually an area of relative strength and may be used to
compensate for challenging areas. For instance, the integration of vestibular,
visual, and somatosensory afferent systems is needed to maintain upright
postural stability. Molloy, Dietrich, and Bhattacharya (2003) measured the
postural stability in children with autism and found that these children relied on
visual cues to help them maintain stability. When these visual cues were omitted,
children had difficulty maintaining their upright balance and reducing their sway.
One area of visual processing that is consistently impaired is integrating details
of a figure into a whole (Deruelle, Rondan, Gepner, & Tardit, 2004). For
instance, if given a line drawing of a house made up of geometric shapes, these
children focus on the shapes rather than seeing the image of a house.
Functions Related to the Digestive System
Children with autism may be at a higher risk for experiencing GI problems such
as reflux or gastritis, with persistent gas, diarrhea, and constipation also
frequently reported (Horvath et al., 1999; Levy et al., 2003; McQueen & Heck,
2002). Additionally, hypotheses exist regarding functioning of the digestive
system, such as increased intestinal permeability that allows absorption of
morphine-like compounds from gluten and casein. The buildup of these
substances theoretically results in the social withdrawal and stereotypical
behaviors seen in autism, but as previously described, this theory has not been
proven (Levy & Hyman, 2003).
Urinary and Reproductive Functions
In children with autism, the urinary tract is usually typical in its structure and
function. However, for those children with cognitive and sensory impairments,
toilet training is often complicated. Children with autism often learn to toilet
train at a later age than typically developing children; evidence problems such as
fear, pain, confusion, frustration, and constipation when learning to train; urinate
or defecate in inappropriate places; and experience difficulty when a change of
routine occurs or when entering an unfamiliar bathroom (Ruble & Dalrymple,
1993).
Because individuals with autism have difficulty interacting, inappropriate
sexual behavior is also a concern. Close relationships with others are challenging
for these individuals; therefore, few person-oriented behaviors are noted.
Additionally, discouraging inappropriate sexual behaviors may be difficult. The
most frequently reported inappropriate sexual behaviors include public
masturbation or public touching of one’s own private parts (Ruble & Dalrymple,
1993; Van Bourgondien, Reichle, & Palmer, 1997).
Brain Structure
The most significant abnormalities in a child’s body structure include the
anomalies of the brain that are currently under investigation. Likely caused by
genetic abnormalities, these deviations in the brains of individuals with autism
are not yet clearly defined, nor is it clear precisely how the differences cause the
characteristics of autism. Studies have found consistent abnormalities in the
cerebellum, frontal lobes, brainstem, and limbic system (Bauman & Kemper,
1996). Research is also examining potential deviations in the way different
regions of the brain communicate (Piven et al., 1997).
CASE-STUDY 1
At 1 year of age, Jacob was brought to an early intervention clinic because
of delays in motor and cognitive skills. An initial evaluation conducted by
an occupational therapist, educator, and speech-language pathologist
confirmed these concerns. In the area of gross motor skills, Jacob was
delayed in learning to walk and demonstrated moderate hypotonia in his
trunk. Fine motor concerns included tactile defensiveness of wet or sticky
substances and delays in grasping and manipulating objects appropriately.
Cognitively, Jacob showed little interest in playing with toys or imitating
the words and actions of others. Language impairments were not yet
observed since Jacob frequently vocalized, babbled, and expressed his
feelings through behaviors such as smiling or crying. At this young age,
ASD was not initially suspected. Jacob was clearly a delightful little boy
who showed a strong attachment to his parents and was interested in
watching children and other adults in his environment.
Jacob was placed in a playgroup at the clinic where his occupational
therapist and educator worked directly with him, while a speech-language
pathologist monitored his language. As time passed, further concerns
became evident. His language skills failed to further develop; as a result,
first words did not emerge. Nor was he using gestures to indicate what he
wanted. At the age of fourteen months, he evidenced limitations in
language comprehension and speech production, and his use of eye contact
decreased. As the months progressed, he showed frustration more
frequently through crying, banging his head, throwing himself on the
ground, and arching his back to pull away from a person trying to hold him.
His feeding skills were limited since he was not able to bring his hand to his
mouth, and he demonstrated extreme sensitivities to many tastes and
textures. He did not show an interest in other self-help skills such as
learning to bathe or dress himself. While other children his age learned to
imitate motor movements in songs, Jacob seemed to content to only listen
to the songs. He did not demonstrate typical play skills for his age such as
exploring how toys worked, taking toys in or out of a container, or taking
turns with others. However, musical or flashing toys captured his attention
for long periods of time. While he was engrossed with these toys, he
evidenced unusual, repetitive behaviors such as rocking his body back and
forth.
Through early intervention by his therapists and parents, Jacob’s gross
motor skills improved during the following months and he successfully
learned to walk at 18 months. He delighted in walking through his home,
through the early intervention center, and outside. His eye contact
improved, becoming more spontaneous and consistent. His repertoire of
sounds increased and he vocalized to take a turn in songs or games; in
addition, he began signing “more” to request something desirable to happen
again. He learned to play with toys in a more functional manner, including
stacking rings and blocks and taking toys in and out of containers. Yet other
skills continued to be challenging. When he was left to play on his own, he
repetitively turned the pages of books or walked aimlessly around a room.
He showed hypersensitivities to touching or mouthing certain textures,
limited motor or imitation skills, difficulty understanding others, a lack of
verbal words, and repetitive behaviors during play.
Currently, at slightly older than 2 years of age, Jacob continues to
struggle in several areas of development. However, he has made steady
progress in these developmental skills, and his family and team are
encouraged that he will continue to make significant gains. More
importantly, despite being faced with more challenges than typically
developing children, Jacob is a young boy who is often able to share and
express joy with others.
CASE-STUDY 2
Patrick is a 38-year-old man who was diagnosed with autistic disorder at
two-and-a-half years of age. His mother was first concerned he had a
hearing impairment, as he did not seem to understand what people said to
him, did not respond to a fire truck siren, placed his ear close to the
refrigerator, and acted out with negative behaviors. Behavioral and
developmental concerns persisted after testing ruled out a hearing
impairment. A psychologist specializing in autism assessed Patrick’s
developmental skills and behaviors and diagnosed Patrick with autistic
disorder.
Patrick communicated nonverbally for several years, usually by
grabbing a person’s hand and leading the person to an item he wanted. He
had a very limited diet as a young child, eating only peanut butter, raisins,
and yogurt. In time, he increased his repertoire of foods. When Patrick was
three, he began attending a local school for children with autism; at the age
of eight, he attended a school for children with a variety of special needs.
Patrick began using words at the age of nine. He transferred to a local
public high school at the age of 18, participating in the school’s autism
program and working in the school’s kitchen to learn to work with others.
He graduated high school when he was 26, receiving a diploma of
completion for the school’s autism program.
Following high school, Patrick moved into a condominium with two
other men diagnosed with autism; he currently maintains this living
situation. Patrick and his roommates receive 24-hour supervision. He also
works five days per week at a therapy center that encourages functional
skill building. He works alongside eight or nine other adults with autism
and receives supervision in his daily tasks, which include shredding legal
documents and inserting newspapers into plastic sleeves. To prevent
overwhelming stimuli, the room is kept dim and without extraneous sound.
As with all individuals diagnosed with autism, Patrick shows personal
strengths and unique challenges, with his own personality shining through.
Patrick has achieved several goals in activities of daily living and
continues to work toward independence. He dresses himself, with the
occasional need for help with fasteners. He toilets independently, though
requires assistance with wiping after a bowel movement. He washes
himself with verbal prompting from a supervisor and climbs in and out of
the tub independently. He applies deodorant and cologne and combs his hair
when directed. Once a supervisor has prepared his toothbrush, Patrick
brushes his teeth for two minutes, needing occasional reminders to not
swallow the toothpaste. He carries groceries into the house, takes out the
trash, and wipes the table after meals.
Patrick displays unusual and stereotypical behaviors, such as rapidly
bouncing or tapping a small ball on a tabletop, spinning lids, and repeatedly
blowing up balloons. He eats his food very quickly and needs verbal
reminders to eat at a slower pace. In the car, he often bends down to play
with the hardware beneath his seat. Recently, Patrick has shown negative
behaviors, such as destruction of property or soiling himself. His family and
staff believe these behaviors are likely Patrick’s way of showing grief from
his father’s death last year.
Socially, Patrick gets along well with his family, supervisors, and
roommates, though he has few verbal interactions with his roommates. His
family frequently brings him to community outings such as sports events;
Patrick shows interest and enjoyment during these outings. He reciprocates
another person’s smile easily. He occasionally initiates and maintains eye
contact with another person, but not with typical duration or frequency. He
often responds to a speaker, but does not engage in back-and-forth
conversation.
Regarding other developmental skills, Patrick currently understands
more language than he uses. He follows familiar one-step directions, though
sometimes needs repetition from the speaker to complete the request. He
comprehends simple questions that relate to his interests and daily routines.
Patrick communicates through single words and phrases. He names familiar
people and objects, answers simple questions, and requests desired items
and actions. He greets when prompted by another person. In the area of fine
motor skills, Patrick does not demonstrate a hand preference. He writes his
first name, last name, and the word “love” to sign letters and cards. Patrick
shows many strengths in his gross motor skills. He enjoys playing a variety
of sports, including swimming and throwing a ball back and forth with a
partner. He loves to shoot basketball hoops (dribbling is more difficult for
him), which was a favorite activity he had shared with his father. He
experiences some difficulty with coordination and motor planning.
Cognitively, Patrick has recently learned the days of the week and loves to
look at calendars. He can recite the alphabet and recognizes a few familiar
written words. He does not yet tell time.
Patrick lives a very active life, filled with family, supportive staff, and
opportunities to contribute to his community. His mother and professional
team continuously work together to create new goals for Patrick, and he
meets these challenges. He is a delightful and admirable individual.
RESOURCES
Organizations
Autism Society of America
7910 Woodmont Ave., Suite 300
Bethesda, MD 20814-3067
www.autism-society.org/site/PageServer
Autism Speaks
http://www.autismspeaks.org
Web site includes a “100 day kit” for families whose children have recently been
diagnosed with autism.
Centers for Disease Control and Prevention
http://www.cdc.gov/ncbddd/autism/index.html
Web site includes fact sheets and other helpful information for families,
individuals with autism, healthcare providers, etc.
First Signs
http://www.firstsigns.org/
Web site includes valuable information on autism and an ASD video glossary
National Alliance of Autism Research
99 Wall Street, Research Park
Princeton, NJ 08540
www.naar.org
National Institute of Mental Health Office of Communications 6001
Executive Blvd
Room 8184, MSC 9663
www.nimh.nih.gov/publicat/autism.cfm
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Lord, C., & McGee, J. P. (Eds.). (2001). Educating children with autism.
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Quill, K. A. (2000). Do-Watch-Listen-Say: Social and communication
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Siegel, B. (2003). Helping children with autism learn: Treatment approaches for
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Volkmar, F. R. (Ed.). (2005). Handbook of autism and pervasive developmental
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C H A P T E R
4 Intellectual Disability
Michelle A. Suarez Ben J. Atchison
KEY TERMS
Cortical atrophy Craniostenosis Cytomegalovirus Down syndrome Fragile X
syndrome Hydrocephaly Hyperphenylalaninemia Hypoxia PICA Spina bifida
Tay-Sachs disease Teratogenic Toxemia
Jeffrey, aged 3 years, was brought to Early On at the local school district for a
developmental evaluation. His parents report that his pediatrician is concerned
about his language development. He smiled and interacted with examiners but
was unable to express his wants and needs or follow single-step directions. An
interview with his parents revealed that Jeffrey was unable to dress or undress
and was not self-feeding. He was diagnosed with an intellectual disability and
enrolled in early childhood special education where he is working toward
language and self-care goals. His family hopes he will eventually attend
mainstream kindergarten with special education support.Michael, now 2, was
diagnosed by his pediatrician shortly after his birth as having Down syndrome.
He exhibits the physical characteristics such as a round face, flattened nose
bridge, abnormally small head, low-set ears, short limbs, and abnormally shaped
fingers. His motor development is delayed and his low muscle tone has made
learning to walk difficult. Intellectual disability in children with Down syndrome
is inevitable but varies in degree of severity. Michael’s early intervention
program provides services for Michael as well as parent support and
education.Kelly, now in second grade, is pleasant and likable. Teachers have
been concerned about her inability to write letters or sound out words since
kindergarten, and as the curriculum becomes more challenging, she is falling
further behind. She has trouble maintaining friendships and often struggles to
join peers in play. School staff conducted multidisciplinary team evaluation and
diagnosed Kelly with an intellectual disability. She was placed in a special
education classroom where the material is appropriate for her cognitive ability
and learning pace.
Definition
The term intellectual disability (ID) has evolved from the previous diagnosis of
mental retardation. This terminology change reflects the shift from a view that
classifies “mental retardation” as a personal trait residing solely within the
individual to a holistic perspective that includes the capabilities of the person
within the context of the environment. The official definition of the disability is
as follows and includes three specific criteria:
“Intellectual disability is characterized by significant limitations both in
intellectual functioning and in adaptive behavior as expressed in conceptual,
social, and practical adaptive skills. This disability originates before age 18.”
(American Psychiatric Association, 2013)
Intellectual Functioning
The first component of this definition, intellectual functioning (or intelligence),
is the general mental capability of an individual. This includes the ability to
reason, plan, problem solve, think abstractly, comprehend complex ideas, and
learn from experience. While it has its limitations, the accepted measure of
intelligence is that which is determined by an intelligence quotient (IQ) score,
which involves administration of standardized tests given by a trained
professional. In the 2010 publication of the American Association on Intellectual
and Developmental Disabilities (AAIDD), a “significant limitation in intellectual
functioning” was defined as two standard deviations below the mean, in the
context of the standard error and strengths and limitations of the specific
instrument (IQ test) used in assessment (American Association on Intellectual
and Developmental Disabilities, 2010). The IQ score is not a complete
representation of human functioning and must be considered in the context of
adaptive behavior, health, participation, and context. Therefore, clinical
judgment must be used to interpret scores when considering diagnosis or service
provision.
Adaptive Behavior
Adaptive behavior is the most related domain of concern among occupational
therapists. Thus, it is our focus of assessment and intervention for persons with
ID. As defined by the AAIDD, “adaptive behavior is the collection of
conceptual, social, and practical skills that people have learned so they can
function in their everyday lives” (AAIDD, 2010). Significant limitations in
adaptive behavior impact a person’s daily life and affect the ability to respond to
a particular situation or to the environment. Table 4.1 provides specific examples
of these three areas, which are published by the AAIDD.
TABLE 4.1 Adaptive Skills
Limitations in adaptive behavior can be determined by using standardized tests

referenced to the general population, including people with disabilities and
people without disabilities. On these standardized measures, significant
limitations in adaptive behavior are operationally defined as performance that is
at least two standard deviations below the mean. In contrast to IQ scores for
determining intellectual abilities, adaptive behavior is measured with the focus
on typical performance and not maximum performance. In other words, the
criteria critical to measuring limitations in adaptive behavior is how a person
typically performs, not performance potential.
Onset before the Age of 18
The final component of the definition of ID is that it begins early in life, and
therefore, a diagnosis of ID is made in childhood. A diagnosis of ID is not
considered in adult-onset degenerative diseases such as dementia or those
associated with traumatic brain injury. In addition, children under the age of 5
years who have delays in developmental milestones and intellectual functioning
are sometimes given a temporary diagnosis of global developmental delay
(GDD) that requires reassessment after age 5 (American Psychiatric Association,
2013). This diagnosis recognizes that accurate assessment of intellectual
functioning is often not possible during this early childhood period, and
therefore, the lifelong intellectual functioning prognosis is difficult to determine.
In considering this definition, the AAIDD notes that there are five
assumptions that need to be considered in the diagnostic process (American
Association on Intellectual Disability, 2010):
1. Limitations in present functioning must be considered within the context of
community environments typical of the individual’s age peers and culture.
2. Valid assessment considers cultural and linguistic diversity as well as
differences in communication, sensory, motor, and behavioral factors.
3. Within an individual, limitations often coexist with strengths.
4. An important purpose of describing limitations is to develop a profile of
needed supports.
5. With appropriate personalized supports over a sustained period, the life
functioning of the person with ID generally will improve.
Etiology
The causes of ID may be classified according to when they occurred in the
developmental cycle (prenatally, perinatally, or postnatally) or by their origin
(biomedical vs. environmental) (Shapiro & Batshaw, 2011). There are hundreds
of causes of ID, yet despite knowing the many factors that contribute to ID, in a
large proportion of cases the cause remains unknown. The ability to determine
cause is highly correlated with the level of the ID. The etiology of ID is much
less likely to be known with individuals who are mildly intellectually disabled
(IQs of 50 to 70) than with those who are severely affected (IQs of <50)
(AAIDD, 2010).
In a large United States population-based study describing probable causes
of ID in school-aged children, the following results were obtained (Matilainen,
Airaksinen, & Monomen, 1995):
No defined cause 78.0%

Prenatal conditions 12.4%
Genetic 7.1%
Perinatal conditions 5.9%
Intrauterine/intrapartum 5.2%
Postneonatal events 3.6%
Teratogenic 2.9% (factors that can disrupt fetal development)
Central nervous system (CNS) birth defects 1.5%
Other birth defects 0.8%
Neonatal 0.7%
Prenatal factors that can cause ID include genetic aberrations, birth defects that
are not genetic in origin, environmental influences, or a combination of factors
(Shapiro & Batshaw, 2011). Up to 50% of the individuals diagnosed with ID
may have more than one causal factor (AAIDD, 2010). With genetic aberrations,
the problem is either with the genes, which are the basic unit of heredity, or the
chromosomes, which carry the genes. Each nongerm cell (cells other than the
ovum and spermatozoa) contains 23 pairs of chromosomes, including one pair of
sex chromosomes that determine the sex of the person. Males have an X and a Y
chromosome, and females have two X chromosomes (Shapiro & Batshaw,
2011).
In many cases of ID, the gene or chromosome that has caused the condition
can be identified specifically. In fact, more than 350 inborn errors of metabolism
that result from genetic changes have been identified. Many of these metabolic
errors lead to ID (Sumar & Lee, 2011). The two most common genetic causes of
ID are Down syndrome and fragile X syndrome. Down syndrome is generally
caused by an extra 21st chromosome, and fragile X syndrome is the result of a
mutation at what is known as the fragile site on the X chromosome (Sumar &
Lee, 2011). In other cases, the specific genetic aberration has not been identified.
Factors such as higher incidences of a condition in specific families or increased
recurrence rates among siblings suggest that the defect is genetic (Sumar & Lee,
2011).
Birth defects that are not considered genetic in origin also can contribute to
or cause ID. These could include such things as malformation of parts of the
CNS (e.g., cortical atrophy, hydrocephaly, spina bifida, craniostenosis)
(Shapiro & Batshaw, 2011), congenital cardiac anomalies (Rogers et al., 1995),
or metabolic disorders not associated with a genetic defect (e.g.,
hypothyroidism) (Reuss, Paneth, & Pinto-Martin, et al., 1996). Environmental
factors may also be involved in prenatal development of ID. They may include
exposure to chemical agents, such as alcohol or nonprescription drugs ingested
by the mother during the pregnancy, maternal conditions such as
hyperphenylalaninemia, a rare form of phenylketonuria caused by increased
levels of phenylalanine amino acid (Mayo Clinic, 2015), toxemia, hypertension,
and diabetes, or to congenital infections such as cytomegalovirus, which is a
common herpesvirus infection that has a wide range of symptoms from
asymptomatic to fever and to severe signs involving the eyes, brain, or other
internal organs; rubella; and syphilis (Taylor, 2003).
Genetic Causes
Genetic causes can be divided into two types: single gene disorders and
chromosomal abnormalities. In single gene disorders, there is a problem with the
quality of the genetic material; a specific gene is defective. In chromosomal
abnormalities, the problem is with the quantity of material. There is either too
much or too little genetic material in a specific chromosome.
Single Gene Disorders
Single gene disorders follow specific patterns of transmission: autosomal
dominant, autosomal recessive, or sex linked. Table 4.2 presents the transmission
patterns and risk factors associated with each type.
TABLE 4.2 Single Gene Disorders
The autosomal dominant type is caused by a single altered gene. Either parent
may be a carrier, or there may have been a spontaneous mutation of the gene.
Dominant inheritance occurs when one parent passes on the defective gene. This
occurs even if the other parent passes a healthy gene. Because the defective gene
can be passed by either parent, there is a 50% risk of the child being affected in
each pregnancy (Rogers, 2008). An example of this type of inherited disorder is
tuberous sclerosis.
In the autosomal recessive type, both parents are carriers but show no
outward signs or symptoms of having the disorder. Inheritance occurs when both
parents pass the defective gene to their offspring. Each pregnancy has a 25% risk
of the child being affected (Rogers, 2008). Examples of this type of disorder are
phenylketonuria (PKU) and Tay-Sachs disease. With X-linked disorders, the
affected gene is on the sex chromosomes, specifically the X chromosome, and
can occur in either parent. Because males have only one X chromosome, if the
father has an affected gene, he will always have the disorder and cannot be a
carrier. Because the female has two X chromosomes, she can either be a carrier
of the disorder (if only one X chromosome is affected) or have the disease
herself (if both X chromosomes are affected). A carrier mother has a 25% risk of
having an affected son. If the father has the affected gene, all his daughters will
be carriers, but his sons will not be affected (Rogers, 2008). Examples of X-
linked disorders are Duchenne muscular dystrophy, fragile X syndrome, Lesch-
Nyhan syndrome, and Hunter’s syndrome.
Chromosomal Aberrations
Chromosomal aberrations include missing or extra chromosomes, either in part,
such as a short arm, or the total chromosome, as is found in the trisomal types.
Either the autosomes or sex chromosomes can be affected, with the autosomal
type resulting in more serious neuromotor impairments (National Human
Genome Research, 2015).The most common are trisomies 21, 18, and 13. The
patterns of transmission are not as readily identified as those of specific gene
defects.
Environmental Influences
Prenatal Factors
There are numerous environmental causes of ID in the prenatal period, including
maternal infections such as rubella, cytomegalovirus, toxoplasmosis, and
syphilis. Low birth weight that results from prematurity or intrauterine growth
retardation can also be a contributing factor. Maternal factors associated with
low birth weight include smoking, lack of prenatal care, infections, poor
nutrition, toxemia, and placental insufficiency. Exposure to industrial chemicals
or drugs, including certain over-the-counter (OTC) prescriptions and illegal
substances, also can affect birth weight, particularly during the first trimester of
pregnancy.
Perinatal Factors
Two major causative factors of ID in the perinatal period are mechanical injuries
at birth and perinatal hypoxia, which refers to reduced oxygen supply.
Mechanical injuries are caused by difficulties of labor because of malposition,
malpresentation, disproportion, or other labor complications that result in tears
of the meninges, blood vessels, or other substances of the brain. Factors that
cause perinatal hypoxia or anoxia include premature placental separation,
massive hemorrhage from placenta previa, umbilical cord wrapped around the
baby’s neck, and meconium aspiration. Very premature infants also may have
impaired respiration or an intracranial hemorrhage that can result in brain
damage.
If a mother has an active case of herpes simplex II and is shedding the virus
at the time of delivery, the baby can acquire the infection in the birth canal,
which can cause severe developmental disability. This can be avoided by testing
to determine whether the mother has an active case and, if so, delivering by
cesarean section.
Postnatal Factors
Traumas or infections that result in injury or a lack of oxygen to the brain are a
major cause of ID during the postnatal period. Traumas include near-drowning
or strangulation, child abuse, and closed head injuries. Early severe psychosocial
deprivation (i.e., attachment disorder, removal from the family home) is a
significant factor ID etiology (Shevell & Sherr, 2008). Infections include
encephalitis and meningitis. ID that results from meningitis caused by
Haemophilus influenzae is now preventable, however, with the introduction of
the Haemophilus influenzae type B (HiB) vaccine (Baraff, Lee, & Schriger,
1993).
ID is one of the most frequently occurring developmental disabilities. Estimates
of the prevalence of ID in this country range from 1% to 3%. Most professionals
associated with the AAIDD accept a prevalence of 2.5%, and they recognize that
the prevalence varies with chronological age (Rogers, 2008). The most recent
data in the DSM-5 reports indicate a prevalence rate of approximately 1%,
which is lower than previous estimates of 2% to 3%. This reduced number is
likely due to the fact that IQ is no longer relied on as the specific factor in
diagnosis of ID (American Psychiatric Association, 2013). Boys are 1.5 times
more likely to be diagnosed with ID than girls, which may be related to the sex-
linked genetic disorders that result in ID (Harbour & Maulik, 2010).
Signs and Symptoms
ID often occurs in tandem with, or as a secondary manifestation of, another
diagnosis. One study’s results found that two-thirds of the children with severe
ID (IQ <50) had an additional neurological diagnosis; <20% of children with
mild ID were found to have an additional neurological diagnosis. These
diagnoses included conditions such as cerebral palsy, epilepsy, and hearing and
visual impairments.
ID is defined by the AAIDD as a condition that is present from childhood
(age 18 or younger), with (IQ) two standard deviations below the mean as
measured on a standardized test and significant limitations in adaptive skills.
Adaptive skills must be two standard deviations below the mean on a
standardized test in conceptual, social, or practical skill areas (AAIDD, 2010).
As previously stated, adaptive skill areas include communication, self-care,
home living, social/interpersonal skills, leisure, health and safety, self-direction,
functional academics, use of community resources, and work. Adaptive skills
should be assessed in all of the individual’s performance contexts. Someone with
limited intellectual function who does not have adaptive skill deficits is not
considered intellectually disabled (AAIDD, 2010).
There are currently two major systems of classification for ID, and both have
similar characteristics. The American Psychiatric Association (APA) publishes
the Diagnostic and Statistical Manual of Mental Disorders (DSM), and the 5th
edition was published in 2013. This system is frequently used for diagnostic
purposes. It classifies ID based on two major criteria. The first is intellectual
functioning, which is determined by standard IQ testing. A score that is less than
two standard deviations below the mean constitutes the cutoff for a cognitive
deficit. The second criterion is deficits in adaptive functioning. This means that
the individual has difficulty with skills required to live independently in a safe
and responsible manner.
In addition to the DSM-5 diagnostic criteria, the AAIDD (previously the
American Association on Mental Retardation) developed a system of
classification based on adaptive skill levels and supports needed to function
(AAIDD, 2010). The AAIDD system, because it focuses on function and
supports needed in adaptive skills across performance contexts, is very useful for
occupational therapy practice.
In the traditional system, ID is classified according to the severity of the
impairment in intellectual functioning. This is determined through standardized
intelligence testing. To be considered intellectually disabled, the person’s
performance on these tests must be two standard deviation units or more below
the mean. The levels of ID as identified by IQ tests are mild, moderate, severe,
and profound. Approximately 85% of individuals with ID are in the mild range,
10% are in the moderate range, 4.0% are in the severe range, and 2.0% are in the
profound range of function (King, Toth, Hodapp, & Dykens, 2009). The DSM-5
classifies four different degrees of mental retardation: mild, moderate, severe,
and profound. These categories are based on the functioning level of the
individual (American Psychiatric Association). It is important to remember that
not all individuals in a particular classification will function at exactly that level.
The classification system developed by the AAIDD involves a three-step
process. The first step is to have a qualified person administer standardized
intelligence and adaptive skills assessments that are appropriate for the
individual’s age, communication abilities, and cultural experience. The second
step is to describe the individual’s strengths and weaknesses across the
dimensions of (1) intellectual and adaptive behavior skills, (2)
psychological/emotional considerations, (3) physical/health/etiological
considerations, and (4) environmental considerations. The third step is to have
the interdisciplinary team determine needed supports across these four
dimensions. Supports are classified based on level of intensity and include
intermittent, limited, extensive, and pervasive. Intermittent support is provided
on an “as-needed” basis. Limited support occurs over a limited time span.
Extensive support is assistance provided on a daily basis in a life area. Pervasive
support refers to the need for support in all life areas across all environments on
a daily basis (AAIDD, 2010).
In addition to the performance deficits produced by ID and the associated
conditions already mentioned, a high proportion of individuals with ID also have
some form of mental illness. Estimates of prevalence of mental illness among
people with ID range from 10% to 20% to 40% to 70% (King et al., 2009). Some
of the common types of mental illness seen in people with ID include personality
disorders, affective disorders, psychotic disorders, avoidant disorder, paranoid
personality disorder, and severe behavior problems that may include self-
injurious behavior (Einfeld, Ellis, & Emerson, 2011). Several misconceptions
about people with ID may complicate or prevent appropriate care for their
mental illness, including the beliefs that people who are intellectually disabled
cannot also be mentally ill, do not experience normal feelings and emotions, and
are not affected by changes in their environment. Substance abuse problems,
especially with alcohol, may be overlooked. Because of limited communication
skills and limitations in abstract thinking caused by the ID, the diagnosis of
mental illness and mental health problems can be a very difficult process and is
frequently inexact. Good communication with caregivers and significant others
in the life of the individual with ID is essential.
ID is generally considered a lifelong condition, but the course and prognosis will
vary depending upon the cause(s) of the disability and access to resources (Beers
& Berkow, 1997; Behrman, Kliegman, & Nelson, 1992). In terms of life
expectancy, people with mild ID live as long as the general population (Shevell
& Sherr, 2008). However, people with more profound ID are less likely to reach
old age. This is likely due to more serious neurological deficits and associated
disorders.
Most cases of ID are nonprogressive, that is, once the initial insult to the
brain occurs, there is no further damage (Beers & Berkow, 1997; Behrman et al.,
1992). The emphasis is on managing the medical aspects of the condition and
helping individuals to achieve their highest potential. However, certain genetic
conditions (e.g., muscular dystrophy and Tay-Sachs disease) are progressive,
with incremental loss of function and, in some cases, associated early death.
Those with Down syndrome experience degenerative changes in the brain,
beginning at about age 40 years, that eventually result in progressive dementia
similar to Alzheimer’s (Carr, 2012). The goal for these individuals is to help
them achieve the highest level of independence and maintain it as long as
possible.
One significant issue that can impact prognosis in people with ID is the
presence of stigma in our society. This group often encounters negative
stereotypes and prejudice from society that can lead to isolation and
discrimination (Werner, 2015). Lower self-esteem and social exclusion can
result. In addition, self-reported stigma from people with ID is strongly
associated with symptoms of anxiety and depression in this population (Ali,
King, Styrdom, & Hassiotis, 2015). However, social inclusion can mediate the
impact of stigma and support psychological well-being (Simplican, Leader,
Kosciulek, & Leahy, 2015). For example, membership in organizations like the
Special Olympics can increase self-esteem, increase quality of life, and reduce
stress (Crawford, Burns, & Fernie, 2015).
Another important factor in the improvement of the prognosis for people
with ID is access to services that focus on strengths and increase adaptive
functioning. With these services, it is possible for individuals with mild ID to
gain adaptive behavior skills through remedial programs to the extent that they
no longer meet the diagnostic criteria for being intellectually disabled, although
their intellectual function has probably not changed significantly (AAIDD,
2010).
Diagnosis
An evaluation must be performed to determine whether a person meets the
criteria for being intellectually disabled. Besides ascertaining that the onset of
the condition occurred before age 18, there are two main aspects to this process.
The first part involves administration of appropriate standardized intelligence
tests by a qualified individual. The selection of the specific standardized
instrument should be based on factors such as the individual’s social, linguistic,
and cultural background (AAIDD, 2010). The individual’s IQ is interpreted in
the context of their level of adaptive behavior.
The second aspect of the process is the evaluation of adaptive behavior as it
relates to the targeted adaptive life skill areas. Adaptive functioning is an
individual’s ability to cope with life demands and meet societal expectations for
independence depending on age group (AAIDD, 2010). The skills needed for
adaptive behavior become more complex and varied as the person ages. For
instance, eating and dressing independently are major skills for the young child,
but the child does not need to be able to use a telephone or manage money.
Evidence for deficits and strengths in adaptive function should be gained from
one or more independent, reliable sources who are familiar with the individual’s
abilities in different performance contexts. This information should be used to
complete a standardized scale designed to provide a composite “picture” of the
individual’s adaptive function. As with the selection of an intelligence test, care
should be taken that the Adaptive Behavior Scale chosen is appropriate for the
individual’s sociocultural background, education, associated handicaps,
motivation, and cooperation level (AAIDD, 2010). There are more than 200
adaptive behavior measures and scales. The most common scale is the Vineland
Adaptive Behavior Scales, Second Edition (VAB-II) (Sparrow, Balla, &
Cicchetti, 2005), which purports to assess the personal and social skills needed
for everyday living. It is an indirect assessment in that the respondent is not the
individual in question but someone familiar with the individual’s behavior. The
VAB-II measures four domains: communication, daily living skills, socialization,
and motor skills, and an optional Maladaptive Behavior Index. An Adaptive
Behavior Composite is a combination of the scores from the four domains. A
second scale frequently used to assess adaptive behavior is the Diagnostic
Adaptive Behavior Scale (DABS) formerly titled the Adaptive Behavior Scale
(AAIDD, 2010). This measure was developed by the AAIDD to assess
conceptual, social, and practical skills and focuses on the critical “cutoff area”
for the purpose of ruling in or ruling out a diagnosis of ID.
There is no drug treatment for the condition of ID; however, medications may be
needed for some of the conditions that may occur in tandem. In the past,
psychotropic medications, and particularly antipsychotics, were often used in an
attempt to manage challenging behaviors (Glover, Bernard, Brandord, Holland,
& Strydom, 2014). However, due to negative side effects and limited evidence
that these medications actually decreased these target behaviors, this practice has
declined. Instead, psychotropic medications are now often used to treat
diagnosed concomitant mental health problems (Tsiouris, Kim, Brown, Pettinger,
& Cohen, 2013). A large-scale study of psychotropic drug use in people with ID
by Tsiouris et al. (2013) revealed that 45% were receiving an antipsychotic (e.g.,
risperidone, olanzapine) to treat psychosis and bipolar disorder, 23% were
receiving an antidepressant (e.g., Prozac, Zoloft) for an affective disorder, and
16% were receiving an antianxiety agent (e.g., clonazepam).
In addition to medications for treatment of mental health disorders,
approximately 30% people with ID also have epilepsy, which may require the
use of medications to reduce seizure activity (Lewis et al., 2000). Medications
include Tegretol for generalized tonic-clonic seizures and tranquilizers like
clonazepam for short-term emergency treatment. Unfortunately, diagnosis of
epilepsy in people with ID can be complicated by communication and tolerance
issues in this population that make standard testing like electroencephalogram
magnetic resonance imaging difficult (McCarron, O’Dwyer, Burke, McGlinchey,
& McCallion, 2014). Misdiagnosis can increase the risk for serious, sometimes
life-threatening consequences of untreated seizure activity. Vigilance and
professionals that are trained in recognizing symptoms of seizures in this
population can increase appropriate medication dosage and seizure management.
Finally, neuromuscular dysfunction (e.g., spasticity) is seen in children with
ID who have comorbid cerebral palsy. Several medications are sometimes
helpful for increasing active and/or passive range of motion for increased
function and prevention of contractures (Smith & Kurian, 2012). For example,
botulinum toxin (e.g., Botox) is injected directly into the muscle group and will
provide a short-term reduction in tone that can be used to strengthen antagonist
muscle groups and facilitate the development of functional movement patterns.
One longer-lasting medication for tone reduction is intrathecal baclofen. This
medication is sometimes delivered by a pump directly into the spinal fluid and
can relax spastic muscle groups. All medications have potential side effects and,
when given, require close monitoring by the prescribing physician.
Impact of Occupational Performance
Virtually all areas of occupational performance and many client factors can be
affected by ID, depending on the cause and severity of the ID. As stated
previously, the diagnostic criteria for individuals with ID included three
categories of adaptive behavior (conceptual, social, and practical skills)
(AAIDD, 2010). These areas (communication, language, interpersonal skills,
social responsibility, recreation, friendships, daily living skills, work, and travel)
fall into all occupational performance areas including (basic) activities of daily
living, instrumental activities of daily living, work, play, leisure, and social
participation.
Although all of the occupational performance areas and client factors can be
influenced by ID, those that are affected will depend on factors such as the
presence of additional medical diagnoses and the severity of the diagnosis. It is
imperative that the clinician be informed about the specific diagnosis that
accompanies the identification of ID to determine the associated client factors
that are involved.
For example, occupational therapists often work with persons who have
Down syndrome. Babies with Down syndrome often have hypotonia, or poor
muscle tone (National Down Syndrome Society, 2012). Because they have a
reduced muscle tone, which results in oral motor dysfunction such as protruding
tongue, feeding babies with Down syndrome is often difficult. Hypotonia may
also affect the muscles of the digestive system, in which case constipation may
be a problem. Atlantoaxial instability, a malformation of the upper part of the
spine located under the base of the skull, is present in some individuals with
Down syndrome. This condition can cause spinal cord compression as well as
craniovertebral instability (Brockmeyer, 1992). Additionally, half of children
with Down syndrome are diagnosed with a congenital heart defect (National
Down Syndrome Society, 2012). In addition to hearing disorders, visual
problems also may be present early in life. Cataracts occur in approximately 3%
of children with Down syndrome but can be surgically removed.
Approximately half of the children with Down syndrome have congenital
heart disease and associated early onset of pulmonary hypertension or high
blood pressure in the lungs (National Down Syndrome Society, 2012).
Echocardiography may be indicated to identify any congenital heart disease. If
the defects have been identified before the onset of pulmonary hypertension,
surgery has provided favorable results. Seizure disorders, though less prevalent
than some of the other associated medical conditions, still affect between 5% and
13% of individuals with Down syndrome, a 10-fold greater incidence than in the
general population. Congenital hypothyroidism, characterized by a reduced basal
metabolism, an enlargement of the thyroid gland, and disturbances in the
autonomic nervous system, occurs slightly more frequently in babies with Down
syndrome. Recent studies indicate that 66% to 89% of children with Down
syndrome have a hearing loss of >15 to 20 decibels in at least one ear, due to the
fact that the external ear and the bones of the middle and inner ear may develop
differently in children with Down syndrome (NIH, 2006). Given that 85% of all
ID cases fall in the mild range of cognitive impairment, global and specific
mental functions will be most affected with this population. The following case
studies illustrate how ID affects an individual’s area of occupational
performance in different stages of the life cycle.
CASE-STUDY 1
K.R. is a 21-year-old young woman with Down syndrome. Despite the fact
that she has reached the legal age of adulthood, she is still in the process of
transitioning from the developmental stage of adolescence to young
adulthood. She still receives special education services but is also working
on developing work and job skills through traditional vocational services.
Her “disability” is expected to be lifelong.
K.R.’s condition was identified at birth. She has received continuous
support and direct services to facilitate development of her abilities since
then. She has always lived at home with her parents and still does so. She
has her own room and bathroom at home and generally has exclusive use of
the family room for her leisure pursuits. Her parents are professionals who
are actively involved in their professions and the community. They are very
realistic about her abilities and extremely supportive, allowing her to make
most of her life choices. K.R. has a younger sister who is now away at
college. K.R. has always been exposed to and involved in many social and
cultural opportunities in the community, both with her family and on her
own. Her social circle includes friends with and without disabilities, and
she has several close friends as well as many social acquaintances.
K.R. has recently declared her life goals to be getting a job, getting an
apartment of her own, and spending leisure time at the local community
drop-in/recreational center for individuals with disabilities. Her mother
feels that with appropriate supports, all of these goals are attainable.
K.R. is independent in most areas of personal care. As a result of
limited fine motor coordination that seems to be complicated by visual-
perceptual deficits, she needs assistance with regulating the water
temperature for her bath, fastening zippers and buttons, and tying her shoes.
She also occasionally needs reminders to straighten her clothing and brush
the back of her hair. She has a speech impediment that makes it difficult for
individuals who are unfamiliar with her to initially understand her. K.R. has
learned to adapt to this limitation and works very hard to make people
understand what she is trying to communicate. When cued, she will slow
down and work on enunciating her words. She is independent in functional
mobility, but because of problems with depth perception, she is very
cautious when climbing steps and negotiating between different surface
levels. She is able to travel independently in community areas she is
familiar with and can ride the community “Dial-a-Ride” bus if the ride is
arranged for her. Her mother feels that K.R. would be aware of danger in
her home, such as a fire, and would get out of the house. She does not
consistently answer the phone when it rings, although she is capable of
doing so. For this reason, she is generally not allowed to be at home alone
because her parents have no way of checking in with her if she does not
answer the phone.
K.R. is able to perform many home management tasks and has recently
become motivated to attempt more activities given her desire to have an
apartment of her own. She generally dislikes “housework” but knows it is
necessary in order to be a good roommate. She folds laundry and puts it
away, and is learning to sort light and dark clothing and operate the washing
machine. Her mother questions her ability to make judgments about what
should or should not go into the dryer, however. She is able to sweep,
vacuum, and dust but is not thorough, and this may be a result of her lack of
interest and/or her visual-perceptual impairment. She keeps her bathroom
clean and makes her bed. She is currently dependent in meal preparation,
and this is the area that will probably require the most support for her to
live in her own apartment. She cannot safely regulate stove and oven
temperatures because of her fine motor problems. At the current time, K.R.
attends school in a self-contained special education classroom in the local
high school for half a day, focusing on vocational and prevocational skills,
and spends the other half-day at Goodwill Industries in a work adjustment
trial placement. K.R. has told her mother that she no longer enjoys going to
school and would rather go to Goodwill. In addition to school and
vocational activities, K.R. volunteers at the local community theater and at
her church office doing clerical tasks. She is very interested in obtaining
employment and would prefer to work at a video store, a music store, at the
mall, or Pizza Hut. She is very interested in clerical tasks and has been
working at her father’s medical practice putting monthly billings in
envelopes to be mailed. K.R. has many leisure interests and activities. She
enjoys music and videos, likes to eat out, participates in Special Olympics,
goes to a community center for structured activities, and socializes with her
friends. She prefers not to do strenuous physical activities, probably
because of the difficulty she has as a result of generalized hypotonia. She
has participated in team sports at the center but does so mostly for the social
interaction. K.R. is very aware of her limitations and takes herself out of
situations that she knows will be difficult or where she might not succeed.
CASE-STUDY 2
F.B. is a 60-year-old man who is intellectually disabled. He has a history of
behavior problems, including PICA and obsessive-compulsive–type
behaviors of picking at his skin and clothing and stuffing toilets. He was
retired from a sheltered workshop approximately 18 months ago when the
emphasis of the program shifted to work readiness for community
placement. It was determined that he would not be a good candidate for
community work because of his age and lack of necessary supports (e.g.,
transportation). He currently attends a day program for social/leisure
activities. F.B. has no known family and has spent most of his life in public
residential institutions or in adult foster care (AFC) homes. He currently has
a court-appointed guardian who makes most significant life decisions for
him, including those regarding medical care and living arrangements. His
guardian supports and encourages F.B. to make his wishes known about
how he would like to spend his leisure time and allowed him input into his
last housing change. In addition to his intellectual limitations, F.B. also
experiences fairly frequent medical problems related to his obsessive-
compulsive behaviors (e.g., skin infections). Medical problems, which may
be age related, are emphysema and frequent fractures of bones in his lower
extremities. F.B. currently lives in an AFC with 11 other adults who are
developmentally disabled. His home is only required by law to provide
basic care, and so it does not offer training in or support for participation in
many home management tasks. His social groups generally consist of other
adults with developmental disabilities or paid paraprofessional staff, either
at home or the day program. His cultural experiences have been very
limited because of his background.
F.B. is independent in most self-care tasks but needs supervision when
using the bathroom because of his history of stuffing the toilet. He needs
assistance and supports for taking medication owing to his cognitive
limitations. He also needs very close monitoring of health status because he
has a very high pain tolerance. He tends to prefer solitary activities but has
become more verbal, social, and outgoing since going to the day program.
He generally seeks out staff to interact with, and this usually takes the form
of teasing. He will share activities with day program peers if prompted and
has shown protective behaviors toward clients who are more limited and
vulnerable than he is. Although he is verbal, he has a speech impediment
that makes it difficult for people who are unfamiliar with him to understand
what he is saying. He is independently ambulatory; however, he has
reduced endurance as a result of an old hip fracture and neuropathy of the
right lower extremity caused by a degenerative disease in the lumbar spine.
He is dependent for all community mobility as a result of cognitive
limitations and lack of experience and training. It is not clear whether he
understands emergency situations, but he is cooperative with emergency
drill procedures at the day program. It is likely that he would need ongoing
supervision to maintain his personal safety.
Because of a lack of experience and opportunity, F.B. is dependent in
all home management tasks. He has no responsibility for caring for others
but seems to be very aware when one of his peers needs assistance or
protection and alerts staff to these needs. He has been retired from the
vocational arena for 18 months and now attends a day program that
emphasizes social and leisure activities.
F.B. is generally not open to exploring new activities and has to be
coaxed and teased by staff to try them. He generally prefers solitary
activities and appears to enjoy assembly activities that result in a finished
product like picture puzzles and building with Erector Set components. He
does not seem to be very interested in watching television or listening to
music but does enjoying going on automobile rides with his guardian,
especially when she drives her convertible with the top down.
RESOURCES
American Association on Intellectual and Developmental Disabilities 444 No.
Capitol St., NW, Suite 846
Washington, DC, 20001
The American Association on Intellectual and Developmental Disabilities
(AAIDD)501 3rd Street, NW Suite 200Washington, D.C. 20001Telephone: (202)
387-1968Fax: 202-387-2193
Baker, B., Brightman, A., Blacher, J., et al. (2004). Steps to independence:
Teaching everyday skills to children with special needs (4th ed.). Baltimore, MD:
Paul H. Brookes.
The Arc
500 E. Border St., Suite 300
Arlington, TX, 76010
Tel: (817) 261-6003; TTY: (817) 277-0553
Fax: (817) 277-3941
Behrman, R., & Kliegman, R. (Eds.) (2012). Nelson essentials of pediatrics (4th
ed.). Philadelphia, PA: WB Saunders.
Case-Smith, J. (2015). Occupational therapy for children (7th ed.). St. Louis,
MO: Mosby.
The Council for Exceptional Children 1920 Association Drive
Reston, VA 22091-1589
National Down Syndrome Congress 1605 Chantilly Dr., Suite 250
Atlanta, GA, 30324
Tel: (800) 232-NDSC
National Down Syndrome Society 666 Broadway
New York, NY, 10012
Tel: (212) 460-9330; toll free: (800) 221-4602
President’s Committee for People with Intellectual Disabilities US Department
of Health & Human Services 330 Independence Ave., SW
Tel: (202) 619-0634
People First International (self-advocacy group) 1340 Chemeketa St., NE
Salem, OR, 97301
Tel: (503) 588-5288
Special Olympics International, Inc.
1350 New York Ave., NW, Suite 500
Tel: (202) 628-3630
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C H A P T E R
5 Muscular Dystrophy
Jennifer L. Forgach Andrea L. Washington
KEY TERMS
Amyotrophic Lateral Sclerosis (ALS) Atrophy
Autosomal dominant
Autosomal recessive
Charcot-Marie-Tooth (CMT) disease Contracture
Corticosteroid
Creatine kinase
Dystrophin
Electromyography (EMG)
Familial
Gower’s maneuver
Gower’s sign
Lumbar lordosis
Muscle biopsy
Neuromuscular disorder
Progressive
Spinal Muscular Atrophy (SMA) Valley sign
Winged scapula
X-linked
J.B. is 6 years old and has a 3-year-old brother and a 1-year-old sister. J.B.’s
mother is concerned because he does not seem to be “keeping up” with his peers.
He gets tired quickly, has had an increased number of falls, and has never
learned how to jump. J.B. recently had to quit his soccer team due to increased
fatigue. J.B.’s mother takes him to his pediatrician who notes that J.B. has
enlarged calves, has proximal muscle weakness, and uses Gower’s maneuver to
rise off the floor. Gower’s maneuver refers to the inability to rise up off the floor
without using the upper extremities to walk up the thighs to assist with hip
extension. This is often seen in multiple types of muscular dystrophy.The
pediatrician refers J.B. and his siblings for blood work, for genetic testing, and to
neurology/muscular dystrophy (MD) specialty clinic for further assessment.J.B.
arrives to the MD clinic, and the neurologist meets with J.B. and his family. His
blood work shows elevated creatine kinase (CK) levels >6,500 IU/L, while his
3-year-old brother’s are normal at around 150 IU/L. CK is a steroid hormone
produced by the adrenal cortex or synthesized for medical purposes;
administered as drugs, they reduce swelling and decrease the body’s immune
system. Genetic testing reveals that J.B. is positive for Duchenne muscular
dystrophy. His younger brother tests negative, and his 1-year-old sister is found
to be a carrier. J.B. is referred to occupational and physical therapy and is
recommended to follow up with the physician in 6 months. Although the above
scenario illustrates one type of MD, MD is not strictly one disease.MD is one of
the most prolific neuromuscular disorders, which include a number of
conditions that affect the muscles and/or direct nervous system control. It is
actually a grouping of 9 different disorders with more than 30 different subtypes.
MD can broadly be defined as a group of hereditary diseases that weaken the
muscles. They are characterized by progressive muscle weakness, defects in
muscle protein, and/or the death of muscle cells and tissues. The progressive
muscle weakness is caused by the lack, or absence, of the structural protein
dystrophin. Dystrophin is a vital part of the intracellular protein complex that is
responsible for maintaining the shape and structure of the muscle fiber. In
general terms, dystrophin acts as the “glue” to hold the muscle together. Without
this “glue,” the muscle breaks down causing the progressive weakness that is the
hallmark characteristic of MD (Emery, 1994, 2001; http://www.mda.org 2015). It
is the distribution and the progression of the muscle weakness that is used to
distinguish between the nine types (Fig. 5.1). The nine main types of MD that
have been identified are (http://www.mda.org 2015) as follows:
Duchenne muscular dystrophy (DMD)

Becker muscular dystrophy (BMD)
Emery-Dreifuss muscular dystrophy (EDMD)
Limb-girdle muscular dystrophy (LGMD)
Facioscapulohumeral muscular dystrophy (FSHD)
Myotonic muscular dystrophy (MMD)
Oculopharyngeal muscular dystrophy (OPMD)
Distal muscular dystrophy (DD)
Congenital muscular dystrophy (CMD)
Although there are currently nine identified forms of the disease, this chapter
will highlight six types of MD most commonly seen by occupational therapists.
Figure 5.1 Typical distribution of muscle weakness seen in different types of

muscular dystrophy. Left to right: Duchenne/Becker, Emery-Dreifuss, limb-
girdle, facioscapulohumeral, distal, oculopharyngeal.
Duchenne Muscular Dystrophy
Although DMD is genetically present at birth, symptoms of the disease may not
present themselves until the child is 3 to 4 years of age. DMD affects only males
and, as will be discussed later, is an X-linked recessive inherited condition
(Biggar, 2006). This is a type of inheritance, also referred to as sex linked, that
occurs when a mother who carries the affected gene passes it onto her son.
DMD is caused by an absence, or deficiency, of dystrophin. Initial symptoms
include delayed motor development, proximal weakness, and increased fatigue.
These symptoms manifest themselves as a waddling gait, enlarged calf muscles,
increased falls, and failure to develop the ability to run or jump (Emery, 1994,
2001). Also present in 90% of males with DMD is the “valley sign.” This is a
depressed area on the posterior axillary fold that can be seen when the patient
abducts shoulders to 90 degrees, elbows flexed to 90 degrees, with bilateral
hands pointing upward (Fig. 5.2).
Figure 5.2 The valley sign: Note the depressed area of the left posterior axillary
region seen beneath the deltoid when the individual abducts shoulders to 90
degrees, elbows flexed to 90 degrees, with bilateral hands pointing upward.
The pattern of muscle weakness is always bilateral and symmetrical. It

progresses from proximal to distal and affects the lower extremities prior to
upper extremities. The rate of progression varies from person to person. The
individual can alternately go through periods of both rapid progression and
slowed or no progression. Most individuals with DMD will be wheelchair
dependent by 12 years of age. DMD affects all voluntary skeletal muscles, as
well as cardiac and pulmonary muscles (Emery, 2001). Of note, up to one-third
of the individuals with DMD may have cognitive impairments. Current research
indicates that this may also be related to the lack of dystrophin (Ashraf & Wong,
2005; Emery, 1994, 2001).
As muscle wasting progresses, active range of motion decreases, and the
individual can be left with contractures, which result in abnormal shortening of
muscle tissue that renders the muscle highly resistant to stretching at the elbows,
hips, and knees, as well as severe spinal deformities. The joint contractures are
often at a 90-degree angle later in the disease process, indicative of prolonged
wheelchair use.
Becker Muscular Dystrophy
Like Duchenne, BMD is characterized by progressive muscle weakness,
affecting primarily males, as an X-linked recessive inherited condition. While
DMD mutations cause virtually no functional dystrophin production, BMD
produces dystrophin that is partially functional. BMD is also genetically present
at birth, but onset of symptoms can vary widely from 2 to 40 years of age.
Symptoms most often appear, however, between 6 and 18 years of age.
Individuals with BMD often demonstrate delayed ambulation, difficulty
climbing stairs, “toe walking,” muscle cramps, and fatigue as their early
symptoms (Emery, 1994, 2001; http://www.mda.org 2015; Stockley, Akber,
Bulgin, & Ray, 2006).
Muscle wasting/weakness progresses proximal to distal and generally occurs
at a slower rate than in DMD (Stockley et al., 2006). It is usually symmetric,
starting in the muscles of the pelvic girdle and thighs. Eventually, it extends to
the trunk and upper extremities. The calves and forearms remain preserved until
later stages of the disease. Facial muscles are not affected. Eventually, heel cord
contractures (from toe walking) and lumbar lordosis, which is an inward
curvature of the lower vertebral column, are seen due to the individual
attempting to compensate for pelvic weakness (Emery, 2001).
Affected men can become wheelchair dependent in their 30s. Some may
never require the use of a wheelchair, managing daily life with the use of
compensation and adaptations.
Limb-Girdle Muscular Dystrophy
Limb-girdle muscular dystrophy accounts for approximately half of the
identified subtypes of MD (Cardamone, Darras, & Ryan, 2008). LGMD is
classified based on age of onset, rate of progression, and type of inheritance.
Most forms of LGMD are of autosomal recessive inheritance that occurs when
both parents carry and pass on the affected gene to their children. Up to 10% of
cases are autosomal dominant, which refers to a type of inheritance that occurs
when a child inherits a normal gene from one parent and an affected gene from
the other parent.
The autosomal recessive forms exhibit more severe symptoms with a faster
decline and loss of function, with an onset usually in childhood (Emery, 2001;
http://www.mda.org 2015).
Other forms of limb-girdle muscular dystrophy have their onset in later
adolescence, or even into adulthood. Adult-onset forms of LGMD are less severe
and progress more slowly. Childhood forms can clinically resemble DMD.
Males and females are equally affected. Dystrophin levels are normal; however,
there is a lack of structural, dystrophin-associated glycoproteins. There are no
associated cognitive deficits with LGMD (Bonnemann, 2005).
Progression of muscle weakness is not always symmetrical. It first affects
the muscles of the pelvis and shoulders, “the limb girdle.” Clinical symptoms are
often first identified by a “waddling” gait. Muscle wasting is usually slow,
however variable. While individuals with severe cases can lose the ability to
ambulate in their early adolescence, others may never have more than
complaints of muscle cramping in their lower extremities. Cardiopulmonary
complications may appear in later stages of the disease (Bonnemann, 2005;
Emery, 2001; http://www.mda.org 2015).
Typical presentation includes enlarged calves, severe lordosis with scoliosis,
proximal muscle weakness, and a positive Gower’s sign. Gower’s maneuver can
be described as the inability to rise off the floor without using the upper
extremities to “walk up” the thighs to assist with hip extension (Fig. 5.3). When
a person is observed to use this technique to get up off the floor, it is documented
as having a positive Gower’s sign (Bonnemann, 2005).
Figure 5.3 The Gower’s maneuver: The sequence of movements utilized by
individuals with proximal weakness needed to rise off the floor. Named for
William Richard Gower who first described this technique in 1879.
Myotonic Muscular Dystrophy
MMD is marked by teen or adult onset. Only 50% of the individuals affected
live beyond 50 years of age. MMD is an autosomal dominant inherited disorder,
affecting both males and females. Progression of muscle weakness is slow
(Emery, 1994; http://www.mda.org 2015).
Muscle wasting/weakness begins in the face, lower legs, forearms, hands,
and neck. Delayed relaxation after muscle contraction is a common symptom.
This can be seen by “locking up” of the muscles, followed by a slowed
relaxation (Emery, 1994; McNally & Pytel, 2007). In addition, MMD affects the
gastrointestinal system, vision, heart, and/or respiratory system. Thirty percent
of individuals affected with MMD will die from cardiac complications. Cardiac
symptoms often precede neurological symptoms. Cognitive impairments may
also be associated with this type of MD (McNally & Pytel, 2007).
Facioscapulohumeral Muscular Dystrophy
Facioscapulohumeral muscular dystrophy is the third most common type of the
dystrophies (Padberg & van Engelen, 2009). It is an autosomal dominant
inherited disorder, affecting both males and females. Onset of symptoms is quite
variable, appearing anywhere from the age of 7 to the age of 20. The earlier the
disease onset, the more severe the symptoms, and the faster the disease will
typically progress (Van der Maarel, Frants, & Padberg, 2007).
It initially affects the facial, shoulder, and upper arm muscles with
progressive weakness. Weakness begins with the facial muscles and progresses
down the body (Padberg & van Engelen, 2009). Weakness, unlike the other
dystrophies, is not usually symmetrical, with one side more affected than the
other. Presentation of facial symptoms include the inability or difficulty with
closing the eyes, asymmetry of the mouth with smiling, drooping of the corners
of the mouth at rest, and inability to whistle or pucker, and atrophy, a wasting of
tissue that results in decreased muscle mass of the facial muscles, is clearly
present (Emery, 1994; Wohlgemuth et al., 2006). As the disorder progresses into
the shoulder region, winged scapula with severe muscle atrophy is present (Van
der Maarel et al., 2007). A winged scapula is a physical symptom when the
medial border of a person’s scapula is abnormally positioned outward and
backward. It is directly an indication of shoulder girdle weakness.
Shoulders appear depressed due to severe muscle weakness (especially in the
biceps and triceps), and the individual is ultimately unable to raise his or her
arms against gravity. With some individuals, the disease can progress to pelvic
muscles and eventually affect their gait. This is most often seen in individuals
with early onset of the condition. Many others never have any pelvic
involvement and never lose the ability to ambulate (Iosa et al., 2007; Padberg &
van Engelen, 2009). This type of MD is not associated with any cardiac
complications or cognitive impairments (Emery, 1994).
Emery-Dreifuss Muscular Dystrophy
EDMD is a less statistically common form of MD. Like BMD and DMD, it is
most often of X-linked recessive inheritance. EDMD primarily affects boys and
usually present by the age of 10. Individuals with EDMD display a unique
pattern of muscle weakness characterized by the formation of muscle
contractures before any significant muscle weakness is recognized by the
individual. Contractures of the heel cords, elbows, and muscles of the posterior
neck are most common. Tightness of the heel cords may result in toe walking
and an increased number of trips and/or falls. Elbows that are contracted to a 90-
degree angle are the “most important diagnostic clue” of EDMD to physicians
(Brown, Piercy, Muntoni, & Sewry, 2008). Eventually, rigidity of the spine and
posterior neck prevents an individual from flexing the body forward. Formation
of contractures and the subsequent muscle weakness is generally symmetrical on
the body (Brown et al., 2008; Emery, 1994; http://www.mda.org 2015;
Yazdanpanah, Javan, Nadimi, & Ghaffarain Shirazi, 2007).
Significant cardiac complications are also highly characteristic of EDMD. It
is often necessary to surgically implant a pacemaker to regulate heart and pulse
rate. Similar to the other forms of MD, it is often the cardiac and pulmonary
complications that cause death in mid-adulthood (Gayathri, Taly, Sinha, Suresh,
& Gorai, 2006). It is important to note, however, that with early intervention and
proper medical management, individuals with EDMD may live an average life
span (Emery, 1994).
Etiology
Most muscular dystrophies are familial, meaning there is some family history of
the disease. All of the muscular dystrophies are inherited genetic disorders.
Muscular dystrophies can be inherited in one of three ways as listed below. It is
important to note, however, that some forms may be inherited in multiple ways;
http://www.mda.org 2015;
1. X-linked recessive
Also referred to as sex linked, occurs when a mother who carries the
affected gene passes it onto her son. Although the mother carries the
affected gene on one of her X chromosomes, she may never show
symptoms since it is a recessive trait.
Muscular dystrophies inherited this way: Duchenne, Becker, Emery-
Dreifuss
2. Autosomal recessive
Occurs when both parents carry and pass on the affected gene
Muscular dystrophies inherited this way: Emery-Dreifuss, limb-girdle,
oculopharyngeal, distal, congenital
3. Autosomal dominant
Occurs when a child inherits a normal gene from one parent and an affected
gene from the other parent
Muscular dystrophies inherited this way: Emery-Dreifuss, limb-girdle,
facioscapulohumeral, myotonic, oculopharyngeal, distal, congenital
It is estimated that at least 1 in every 5,000 people in the population are currently
affected with some form of MD. The actual prevalence of the disease is most
likely higher than this as many forms of MD are often misdiagnosed, not yet
identified, or overlap clinical symptoms with other disorders. Therefore, specific
current statistical information is difficult to obtain (Cardamonne et al., 2008;
Emery, 1994, 2001; Kohler et al., 2009; McNally & Pytel, 2007). Table 5.1 is an
approximation of available statistical figures in research literature today. It
should be used only as a tool to compare the occurrence of one type of MD to
another.
TABLE 5.1 Incidence and Prevalence of Specific Types of Muscular

Dystrophy
It is most important to recognize from Table 5.1 that Duchenne and Becker MD
are by far the most common types and affect only males. Other forms, like limb-
girdle and Emery-Dreifuss, have statistically unknown or unreliable data. This
may be due to Emery-Dreifuss being less common than the other forms, and the
many different subtypes of limb-girdle. MD occurs worldwide affecting all
races, ages, and genders. Depending on the region of the world, or country, a
certain form of MD may be very common or very rare (Mah et al., 2014).
Signs and Symptoms
Refer to each of the individual descriptions of the different types of MD for
specific signs and symptoms as it relates to each of the various subtypes. As
mentioned, decreased strength, muscle cramping, and decreased gross motor
skills are the common link between all forms, with each form having its own
unique presentation. As previously defined, the Gower’s maneuver and the
“valley sign” are two key clinical indicators that may be evident in any form of
MD with proximal muscle weakness (Emery, 1994, 2001).
There are other progressive neuromuscular disorders that mimic MD and are
often mistaken for MD in their early stages. Some of these include spinal
muscular atrophy (SMA) (a progressive or worsening motor neuron disease
that results in generalized muscle weakness and is most severe in the muscles
closest to the center of the body), myasthenia gravis (MG), Pompe disease, and
Charcot-Marie-Tooth (CMT) disease (a hereditary, slowly progressing disease
of the peripheral nerves). An individual may present with muscle weakness and
wasting and some loss of sensation in the feet, legs, hands, and forearms. This
often results in contractures and scoliosis mitochondrial myopathy and
amyotrophic lateral sclerosis (ALS). These disorders exhibit the same
symptoms at onset including muscle cramping, generalized progressive
weakness, and joint stiffness and are often treated in neurological clinics
alongside individuals with MD (http://www.mda.org 2015).
Diagnosis
MD is often first suspected by a primary care physician when a patient presents
with complaints of muscle weakness, increased falls, muscle cramps, or
increased fatigue. It is at that point that clinical assessment becomes a vital part
of the diagnostic process.
The patient is physically assessed by the physician. Based on the distribution
of the weakness in conjunction with other aforementioned physical symptoms,
MD may be suspected. At this point, the physician will begin to order more
testing to confirm diagnosis.
The following three diagnostic tests are typically used to confirm or rule out
an MD diagnosis (Emery, 1994, 2001; Stockley et al., 2006):
Blood work to perform genetic testing and reveal CK levels. CK levels

become elevated when there is muscle damage in the body; therefore,
elevated levels will indicate that there is muscle wasting occurring. Normal
CK levels average around 100 to 200 IU/L. The levels in Duchenne MD, in
comparison, can be elevated 50 to 100 times this amount (Biggar, 2006;
Emery, 1994, 2001).
Electromyography (EMG) is a diagnostic tool used for testing the
electrical activity of muscle to evaluate the current electrical activity
produced by the skeletal muscles. An EMG examination can be used to
confirm the diagnosis of dystrophy and distinguish this from other types of
neuromuscular diseases. It cannot reveal what type of dystrophy a patient
may have (Emery, 2001).
Muscle biopsy is used to examine the cellular makeup of the muscle tissue
and look at the differences in muscle fibers, fiber size, fiber splitting, and
fiber necrosis. Muscle biopsy can be used to distinguish the type of
dystrophy, based on subtle differences in the muscle tissues (Emery, 1994).
These are not stand-alone tests; therefore, it is important to complete a thorough

clinical and neurological exam to identify any patterns of muscle weakness, test
reflex responses and coordination, and identify contracted muscles (Wren,
Blumi, Tseng-Ong, & Gilsanz, 2007). If one child is diagnosed with MD, all
siblings and parents should be tested for the condition as well.
Each of the muscular dystrophies follows its own unique course. Each type has a
variable prognosis with most ultimately resulting in a shortened life span.
Prognosis will depend on not only the primary diagnosis but also what specific
subtype of the disorder is present. For instance, limb-girdle MD has up to 15
different subtypes. Some of these subtypes have a normal life expectancy, while
others have a shortened life span. The course and prognosis of each of the
muscular dystrophies examined in this chapter are discussed individually below.
While the figures represent an average of current statistical data, research and
medical advances are beginning to extend life expectancies (http://www.mda.org
2015).
Duchenne muscular dystrophy: Males affected with DMD have an average
life expectancy of late teens to early 20s. The cause of death is usually
secondary to respiratory and/or cardiac complications (Biggar, 2006; Emery,
1994; 2001; Kohler et al., 2009).
Becker muscular dystrophy: Less severe than DMD, males affected with BMD
demonstrate a much slower progression. Life expectancy varies greatly, but
average is between middle and late adulthood, approximately 25 to 30 years
after onset of the condition (Emery, 2001; Stockley et al., 2006). If cardiac
aspects are minimal, or adequately controlled, a normal life span is possible.
Emery-Dreifuss: Disease progresses slowly, often with cardiac and respiratory
complications. Individuals may live an average life span if proper cardiac
management is received (Cardamonne et al., 2008; Chen, 2008).
Limb-girdle muscular dystrophy: Progresses slowly. Although symptoms may
appear in childhood, they often do not appear until adolescence or even
adulthood. In the most severe form, muscles deteriorate rapidly, and life
expectancy may be only into the early 30s. Otherwise, an individual may live
a normal life span. The course of limb-girdle is by far the most variable of the
dystrophies (Bonnemann, 2005; Emery, 1994, 2001; http://www.mda.org
2015).
Facioscapulohumeral: Symptoms appear around age 20. Disease progresses
slowly with some periods of rapid decline. Course of disease may spread over
many decades, with life expectancy being normal (Padberg & van Engelen,
2009; Van der Maarel et al., 2006).
Myotonic: Disease progresses slowly with course spanning up to 50 to 60
years. Life expectancy typically into late adulthood (Cardamonne et al.,
2008).
Currently, there is no cure or specific treatment that can reverse or stop the
progression of any form of MD; however, vast amounts of research is being
conducted in this area. Symptom management will have the greatest impact on
enhancing a person’s quality of life and possibly increasing their life expectancy.
The primary goal of treatment with MD is maintenance of independence for as
long as possible (Emery, 2001).
Respiratory Maintenance
As mobility and muscle functioning decreases, respiratory maintenance becomes
increasingly difficult. The individual will have decreased ability to use the
voluntary muscles of the thorax to fully inhale, or cough, causing inability to
keep the lungs clear. The individual may require suctioning, cough assist, or
antibiotics to preserve lung function. They are high risk for pneumonia and other
respiratory infections. Monitoring for signs/symptoms of aspiration is an
important part of pneumonia prevention (Kohler et al., 2009; Thompson, 1999).
Skin Integrity
Skin integrity becomes a risk factor with decreased mobility. When skin integrity
is compromised, the individual is at increased risk for infection, further tissue
damage and loss of joint mobility. Careful skin inspection should be
incorporated into one’s daily routine. Severe infections can lead to death if not
properly managed.
Pain
Pain should be assessed in all individuals with MD. While most types of MD are
not directly associated with pain, some physical symptoms may contribute to
discomfort (Emery, 1994). Initial feelings of muscle cramping or spasms may
occur. The pain cycle may initially worsen when a stretching or strengthening
program is initiated. It is advantageous to complete these exercises on a regular
basis as the body will tolerate the stretches with less discomfort. In fact, it is
common for an individual with MD to request joint stretching as a source of pain
relief. As the disease progresses, pain levels should be continually monitored
and assessed as increased or localized pain could be indicative of further
concerns. Adequate pain management is essential for enhancing quality of life as
well as promoting maximum participation in daily tasks.
Nutrition
Good nutrition and hydration are essential to maintain overall health and can aid
in the maintenance of skin integrity. Nutrition can also assist in controlling
obesity, which can be detrimental to function and mobility. People with MD are
at high risk for obesity, secondary to lack of mobility and loss of muscle
function. Safe maintenance of nutrition becomes a priority as oral and
pharyngeal musculature may also be weakened in some forms of MD. As a
result, swallowing can become unsafe. Individuals should be closely monitored
for signs of dysphagia so that diet can be modified or a feeding tube inserted
(Cardamonne et al., 2008; Thompson, 1999).
Occupational and/or Physical Therapy
Therapy should be initiated as soon as the diagnosis is confirmed to train the
patient and family in an extensive home exercise program. A baseline of
activities of daily living (ADL) skills and mobility performance should also be
obtained. It is important to initiate therapy prior to the formation of contractures
or muscle tightness (Thompson, 1999). Therapy is an essential component in
preventing or slowing loss of strength, range of motion, and function. Another
major role of therapy is to assess current equipment needs for home and
community use. The role of occupational or physical therapy becomes
increasingly important as the disease progresses and specific individual needs
change. Evaluation and provision of splints and orthotics as needed to prevent
joint contracture formation should further be considered at the time of the
evaluation.
Drug Intervention
Corticosteroids are steroid hormones produced by the adrenal cortex or
synthesized for medical purposes. When these hormones are administered as
drugs, they reduce swelling and decrease the body’s immune system may
decrease muscle damage and assist with preserving respiratory function.
Although they present possible benefits, they are often associated with side
effects that must be closely monitored, including the tendency to gain weight.
While corticosteroids are widely used, there continues to be controversy.
Significant research is being conducted regarding whether the use of steroids has
any effect on life expectancy. In addition to corticosteroids, there are other
medications that are often prescribed by a physician for treatment of MD.
Immunosuppressant drugs can be used to delay muscle degeneration.
Anticonvulsants can be used to control muscle activity. Antibiotics can be used
to control respiratory infections. Over-the-counter analgesics can be used for
pain management and cramping (Biggar, 2006).
Surgical Management
Surgical management often includes corrective and/or preventative surgeries.
Some of these may include, but are not limited to, contracture release, scoliosis
repair, cardiac stability, respiratory assist, or spinal stabilization. Spinal
deformities can physically reduce a person’s ability to breathe and should be
closely monitored. Pacemakers can be surgically implanted to control heart
rhythms as needed. Tracheostomies may need to be performed if a person
requires ventilator assistance or suctioning of secretions. Feeding tubes, either
temporary or permanent, may need to be considered if dysphagia is a concern
(Kohler et al., 2009; Thompson, 1999).
Advances in Medical Management
Current scientific research is being conducted seeking the causes of and
treatments for neuromuscular diseases. There are currently three main research
methodologies being completed: gene replacement therapy, genetic modification
therapy, and drug-based therapy. Gene replacement therapy directly addresses
the lack of dystrophin by “replacing” it with a closely related protein that is not
affected by the gene mutation. Genetic modification therapy, however, acts to
“skip” or “read through” the genetic mutation by manipulating the protein to
result in a partially functional dystrophin. Lastly, drug-based therapy (i.e.,
steroids) seeks to delay muscle wasting by promoting muscle growth or reducing
the damage caused by inflammation. As research and clinical trials continue to
advance, there is expected to be a greater impact on prognosis and overall
quality of life (Braun, Wang, Mack, & Childers, 2014; Mah et al., 2014; Ulane,
Teed, & Sampson, 2014).
As described in the Occupational Therapy Practice Framework, 3rd edition,
occupations, client factors, performance skills, performance patterns, context,
and environment may all be affected by MD. These are most dependent on the
type and the stage of the disease. As previously mentioned, each type of MD has
its own unique progression and variable severity. Each individual may have any
combination of these different occupational deficits.
Activities of Daily Living (ADL)/Instrumental
Activities of Daily Living (IADL)
The individuals with MD may initially be independent with their ADL/IADLS
skills; however, a decline in function is anticipated, especially with more severe
forms. Maintaining the basic underlying skills of strength and ROM will keep
the individual independent longer. As active movement becomes more difficult,
the person will require assistance from a caregiver to complete basic tasks such
as dressing, grooming, and bathing. Toileting may become a cumbersome task.
This can be due to physical limitations making toilet transfers difficult or due to
loss of muscle control causing incontinence. Often with more severe forms of
MD, the individual is required to wear protective toileting garments.
Eventually, the use of adaptive equipment such as commode chairs, shower
chairs, bathing aides, mechanical lifts, ADL equipment, and
wheelchairs/mobility equipment may be necessary. These pieces of equipment
will help increase independence and make caring for the individual easier and
safer. Home modifications such as ramps, grab bars, and bedrails should be
considered to promote independence and safety within an individual’s home
environment. In less severe forms, the individuals may maintain the ability to
complete the task themselves but may require increased time to do so. Energy
conservation, or task adaptation, should be a consideration for these individuals
(Kohler et al., 2009). Tasks such as driving, community mobility, health
management and maintenance, meal preparation, and shopping may also require
adaptation or modification to allow for some level of participation. This will
most likely be an area of dependence for most individuals in later stages of the
disease.
Education
School is a major concern as the majority of MD cases are diagnosed in early
childhood or early adolescence. Most forms of MD do not involve cognitive
impairments, and therefore, physical limitations ultimately become the barrier at
school (Donders & Taneja, 2009; Thompson, 1999). Often the child with MD
fatigues quickly, has difficulty keeping up with peers, and may require physical
assistance to get through the school day. A one-on-one aid may be necessary to
assist with maneuvering the wheelchair, toileting, assisting with meals, and
completing basic educational tasks.
Increased absences due to illness may be common. This, combined with
wheelchair/adaptive equipment use and the decreased physical ability to
participate in the same activities as peers, may contribute to social isolation.
Work
Older individuals may face many of the same barriers as children in school.
Social isolation, environmental barriers, fatigue, an inability to keep up with
work activities, and requiring physical assistance for some basic tasks may be
common in the workplace.
Obtaining and maintaining employment and having the ability to change
jobs are more difficult for these individuals due to physical limitations,
decreased job opportunities, and employer misperceptions. Some individuals
with severe MD may lose, or never have, the physical ability to work. Others
may maintain employment with altered responsibilities/adaptations or with
relatively little interruption at all.
Play/Leisure
A child’s primary responsibility is to play. Many individuals diagnosed with MD
are children, and this area becomes a significant area of concern. It is important
to encourage play, in any capacity, to ensure pleasure, learning, and
development. A child with physical impairments may require modifications to
his or her leisure activities to promote success and enjoyment.
For individuals of all ages, leisure activities such as computer and video
game play, text messaging, and reading are commonly enjoyed as hand function
is often preserved well enough to complete these tasks. It is beneficial to also
incorporate and encourage gross motor movements into leisure activities (i.e.,
balloon volleyball, painting on an easel, etc.) as a means to preserve muscle
strength and function (Thompson, 1999).
Psychosocial
MD affects everyone within the family, not solely the affected individual. The
emotions, fears, and anxieties experienced by the individual are shared by the
entire family. Parents often face feelings of guilt, helplessness, and fear over the
anticipated loss of their child. Fear and anxiety are often a concern for the
younger sibling as they watch the deterioration of their older sibling, with the
anticipation that they will soon, too, face the same fate.
As the affected individual loses function, and the loss of independence
becomes more evident, the individual often struggles with anger and frustration.
Individuals who face severe functional loss often experience feelings of
depression. Suicidal ideations may become a concern as the individual faces the
loss of his or her perceived role (Bostrom & Ahlstrom, 2005; Chen, 2008;
Poysky & Kinnett, 2008).
CASE-STUDY 1
A.F. is 32 years old. When she was 13 years old, she began to notice that
her hips and lower back would fatigue quickly and that she was having
minor trouble with her balance. Her primary care physician felt that it was
“growing pains” and told her that he was sure it would pass. A year later,
A.F. continued to have problems, and her symptoms worsened. A.F.
returned to her physician and, at that time, blood work and a muscle biopsy
were conducted. A.F. was eventually diagnosed with limb-girdle muscular
dystrophy.
On this day, A.F. presents to the muscular dystrophy clinic per
recommendation of her primary care physician. The occupational therapist
evaluates A.F. It was revealed that A.F. has had significant functional
decline in the last year. She was previously walking with a walker for
household distances. She is now using a borrowed wheelchair for all
ambulation. A.F. can independently complete a stand-pivot transfer from
her wheelchair to the examining table with increased time allotted. Manual
muscle testing scores as follows: grossly 3/5 bilateral shoulder strength,
3+/5 strength at bilateral elbows, and 4/5 in the wrists and hands.
A.F. currently has a shower chair in the bathtub that she borrowed from
her grandmother. However, A.F. further reports that she is no longer able to
step over the side of the tub. She is now wearing a diaper due to her
inability to get to the toilet quickly enough. She attempts to dress herself
seated at the edge of the bed but is unable to don her socks or shoes as a
result of leg weakness and poor sitting balance. A.F. currently lives alone
and does not have family close by to assist her. She has hired a maid to
keep the house clean and assist with weekly laundry. She recently had to
quit her job as a school teacher due to her physical decline. She is now
significantly concerned about her finances.
She reports that she is feeling isolated at home. She does not identify
any leisure interests other than watching television. She states that it is too
much work to get out of the house, so she just stays home. A.F. is anxious
about her recent decline and is not knowledgeable about her condition or
prognosis. She is not currently taking any medications except an over-the-
counter analgesic, as needed.
CASE-STUDY 2
H.M. is a 12-year-old male diagnosed with Duchenne muscular dystrophy.
He is currently an inpatient at a children’s hospital. He presented to the ER
with complaints of difficulty breathing. He subsequently required intubation
and eventually a tracheostomy.
Occupational therapy was consulted to evaluate H.M. and to provide
recommendations to his family. A review of the medical record reveals that
H.M. was diagnosed with muscular dystrophy at the age of 3. He is
currently on prednisone, digitalis, a multivitamin, a diuretic, and an
analgesic as needed. He is the oldest of four children, with two other
brothers also diagnosed with DMD. He had spinal stabilization surgery
approximately 1 year ago and releases of bilateral knee contractures about 6
months ago.
Upon entering H.M.’s hospital room, the occupational therapist
completes a parent interview and receives the following information: H.M.
is currently in the 7th grade in a POHI classroom. He lives in a two-story
home, with first floor bedroom and bathroom. They have a ramp outside to
enter the home. H.M. has not received therapy in almost a year. Parents are
concerned that H.M. has outgrown his current wheelchair and is not
properly supported by his seating system. Parents also report that H.M. has
been gaining weight, making all wheelchair and surface transfers
increasingly difficult. Prior to admission, H.M. required variable assistance
for all ADL, IADL, and transfers. There is no current home exercise
program in place.
Upon interviewing H.M., he appears to be delayed cognitively,
requiring simple questions and multiple cues to follow one-step commands.
He reports no pain throughout his day. He spends his time watching
television, playing video games, and texting his friends on his cell phone.
H.M. states that he would like to be more independent within his school and
home environments.
Evaluation findings as follows: H.M. presents with decreased upper
extremity active range of motion and strength, as he is unable to raise his
arms over his head or fully straighten his elbows. He also presents with
contractures of bilateral shoulders, elbows, and wrists. H.M. demonstrates
the ability to isolate all fingers and thumb movements. He presents with
decreased grip strength bilaterally. He currently demonstrates the ability to
feed himself with a spoon; however, he is observed to lower his head to the
plate. H.M. is coughing and choking during meals with minimal chewing
observed. He currently requires maximal assistance for all bathing/dressing
tasks, which is being completed in bed at this time. Bed mobility is
impaired. He requires maximum assistance to transfer from supine to edge
of bed and is unable to maintain sitting balance without support.
The inpatient occupational therapist made the following
recommendations to H.M. and his family: Referrals were made to
outpatient occupational and physical therapy following discharge. A
thorough feeding evaluation is recommended with possible feeding tube
placement, as he is showing signs/symptoms of aspiration. A new
wheelchair and seating system evaluation is to be completed. H.M.’s
parents were also provided an initial home exercise program for safe
positioning, daily stretching, and mobilization. The social work department
was contacted to address possible psychosocial and coping needs.
Acknowledgments
We would like to thank Lawrence C. Banko for his contributions. Lawrence was
responsible for drawing all sketches/illustrations used in this chapter.
Online Resources
Muscular Dystrophy Association www.mda.org
Centers for Disease Control and Prevention www.cdc.gov
Parent Project Muscular Dystrophy www.parentprojectmd.org
National Institute of Health www.nih.gov
Muscular Dystrophy Canada
www.muscle.ca
Muscular Dystrophy Family Foundation www.mdff.org
Mayo Clinic Web site
www.mayoclinic.org
Patient and family support group www.dailystrength.org/muscular-
dystrophies/support-group
Cincinnati Children’s Hospital Web site www.cincinnatichildrens.org
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C H A P T E R
6 Attention Deficit Disorder
Shelley Mulligan
KEY TERMS
Clinical presentations Cognitive-behavior therapy DAMP
Developmental coordination disorder Executive Functions Hyperactive-impulsive Inattentive
Methylphenidate Multimodal intervention
Samuel is 7 years of age and lives at home with his parents and 12-year-old
sister. Samuel just completed his first few months of second grade and had
begun to come home from school often in tears, and stating that he hates school.
A phone call to his teacher revealed that he was struggling with math, reading,
and writing. His teacher also said that he has trouble sitting still, and
concentrating, and that he was not getting along well with the other children. His
mother recognized that Samuel tended to be impulsive and rarely would sit still,
unless he was playing his favorite video game. She found that he was easily
frustrated and often irritable with frequent temper outbursts, which had gotten
worse over the past few months.As an infant and very young child, Samuel was
quite easy to care for, although he was always full of energy and had trouble
following bedtime routines and sleeping through the night. His mother felt that
Samuel needed more help than did other children his age to complete basic self-
care skills like dressing and bathing or putting his belongings away. He liked
playing outside with his bike, scooter, and any type of ball play and preferred
gross motor games including rough and tumble play, although he was accident-
prone. Samuel had joined a baseball team the previous spring, which did not go
well. He had trouble following directions, often pushed or bothered the other
children, and was often just running around. Eventually, he withdrew from the
program. Samuel is a healthy boy, and his medical history is unremarkable with
the exception of a fractured radius, caused by a fall off of his bike 2 years prior.
He had not ever received therapy or special education services.Based on his
second grade teacher’s concerns, and parent concerns, he was referred for an
evaluation by the school district’s special education team including a school
psychologist, special education teacher, and occupational therapist. Outside of
school, he was evaluated by a developmental pediatrician known for her
expertise with children with learning and attention disorders, and she diagnosed
him with attention deficit hyperactivity disorder (ADHD), combined
presentation. The label of ADHD combined is given to adults and children who
exhibit both attention and hyperactive-impulsive symptoms. Samuel’s evaluation
by the school team indicated that Samuel had a poor attention span and that he
needed frequent one-on-one assistance to initiate and complete tasks. He tended
to interrupt the conversations of others, and the volume of his speech was loud.
Executive function deficits consistent with the ADHD-combined presentation
were noted, including inattention, disorganization, and poor awareness and
problem solving. Standardized testing revealed that he had normal intelligence
with IQ scores falling in the low-average range (88 to 92), with a slightly higher
verbal than performance IQ. However, his math and reading skills were below
average. Sensory and motor deficits were identified with respect to auditory
processing, and motor planning, and were believed to contribute to his
challenges with fine and gross motor skills, and social play.His mother was most
concerned regarding Samuel’s school performance, and she was also worried
about his emotional well-being and self-esteem. Samuel was determined to be
eligible for special education and related services based on his ADHD diagnosis
and his challenges at school, and subsequently, an individualized education
program (IEP) was developed and implemented. His pediatrician recommended
that he begin a trial of Ritalin, but his parents wanted to gather more information
about the use of stimulant medication prior to agreeing to a trial of medication.
Definition and Description
ADHD is the most common neurobiological disorder that manifests in
childhood, and it often continues into adolescence and adulthood (Wolraich et
al., 2011). ADHD is characterized by persistent and maladaptive symptoms of
inattention, hyperactivity, and impulsivity (APA, 2013). The average age of
onset is 7 years of age, and boys are four times more likely than girls to have the
disorder. ADHD is placed in the latest edition of the Diagnostic and Statistical
Manual for Mental Disorders, Fifth Edition (DSM-5) by American
Psychological Association, (2013) within the section describing
neurodevelopmental disorders. This is a change from previous versions of the
DSM, which had placed ADHD within the disruptive behavior disorders. ADHD
includes three subtypes: combined, predominantly inattentive, and
predominately hyperactive-impulsive, which in the DSM-5 are referred to as
clinical presentations rather than subtypes. This subtle change in semantics
from previous versions shifts the disorder from one with distinctly delineated
subtypes to one that is more fluid, which better accommodates the changes in
presentation or symptoms that often occur over time in the same individuals with
ADHD. A helpful addition in the DSM-5 is that examples of how symptoms of
the disorder may present in adolescents and adults are included, which gives a
little more recognition of the disorder in older children and adulthood. A
summary of the symptoms are listed below, and a more thorough and detailed
description is available in the DSM-5 (APA, 2013).
Inattentive Symptoms of ADHD
Often fails to give close attention to details or makes careless mistakes in
schoolwork, work, or other activities (e.g., overlooks or misses details)
Often has difficulty sustaining attention in tasks or play activities and
remaining focused during activity
Often does not appear to listen when spoken to directly (e.g., mind seems to
wander)
Has difficulty following through on instructions with task completion; often
gets sidetracked
Has poor organizational skills and has difficulty managing sequential tasks
Avoids or is reluctant to engage in tasks that require sustained mental effort
such as schoolwork
Often loses necessary items like a cell phone, keys, or glasses
Is often easily distracted by extraneous or unimportant stimuli
Is often forgetful in daily activities
Hyperactive-impulsive symptoms of ADHD
Often fidgets with hands, taps hand or feet, or squirms in a chair
Often gets up and out of one’s seat when remaining seated is expected
Often runs about, or feels restless
Often has difficulties engaging in leisure activities quietly
Is often “on the go” or is uncomfortable being still for an extended time
Often talks excessively
Often blurts out answers before questions have been completed
Often has difficulty waiting or taking turns
In addition to the criteria listed above related to symptomatology, there are other
factors to consider when evaluating an individual for the possibility of an ADHD
diagnosis. In the DSM-5, criteria state that several inattentive or hyperactive-
impulsive symptoms must be present prior to 12 years of age (which is 5 years
later than what was previously used as a criterion). In order to be diagnosed with
ADHD, five or six inattentive or hyperactive-impulsive symptoms as listed
above must be observed in two or more settings (e.g., home or school). Thus,
symptoms must be evident in more than one context, but not necessarily so
severe as to result in impairments in an individual’s functioning in multiple
contexts. There also must be clear evidence that symptoms interfere with or
reduce the quality of the individual’s social functioning, academic performance,
and/or ability to perform their desired and necessary occupations (APA, 2013).
This requirement relates to the negative impact that the clinical symptoms must
have on an individual’s daily functioning.
DSM-5 criteria are slightly more lenient than are earlier versions of the
manual, which required evidence of clinically significant impairment in social,
academic, or occupational functioning. Removing the need for clinically
significant impairment may make it easier for an individual to meet full
diagnostic criteria for ADHD under the new DSM-5 criteria and perhaps will
potentially increase the percentage of the population who qualify for the
diagnosis. Diagnosticians must also rule out alternative explanations for core
symptomatology as an individual’s ADHD symptoms cannot be accounted for
by another mental disorder. Symptoms consistent with an ADHD diagnosis
cannot occur only in conjunction with or be better accounted for by another
mental disorder such as schizophrenia or other psychotic disorder (APA, 2013).
Although ADHD can certainly be diagnosed in conjunction with other disorders
(e.g., autism spectrum disorders), the diagnosis of ADHD should not be used if
the symptoms are better accounted for by another existing condition. Finally,
DSM-5 criteria require that the level of severity of an individual’s ADHD be
specified when the diagnosis is made, as mild (few symptoms beyond those
needed to make the diagnosis; only minor impairment in functioning), moderate
(between mild and severe), or severe (many symptoms in excess of those needed
for the diagnosis, and marked impairments in functioning resulting from
symptoms).
Types of ADHD
ADHD Combined
The label of ADHD combined is given to adults and children who exhibit both
attention and hyperactive-impulsive symptoms. Children younger than 17 years
of age must exhibit at least six inattentive and six hyperactive-impulsive
symptoms, while individuals who are 17 years of age or older require only five
symptoms of each category. Therefore, an important change in the DSM-5
diagnostic criteria reflects the understanding that a reduction in symptoms often
occurs with increasing age and that a slightly lower threshold in symptoms is
sufficient to reliably diagnose the disorder in adults.
ADHD Predominately Inattentive
This clinical presentation is given to individuals with sufficient inattentive
symptoms (six symptoms if under 17 years of age; five symptoms if 17 years of
age or older) but insufficient hyperactive-impulsive symptoms.
ADHD Predominately Hyperactive-Impulsive
This clinical presentation is given to adults and children who have sufficient
hyperactive-impulsivity symptoms (six symptoms if under 17 years of age; five
symptoms if 17 years of age or older) and insufficient inattention symptoms.
ADHD: Other Specified or Unspecified
This ADHD category is reserved for individuals who show some symptoms
characteristic of the disorder, but who do not meet diagnostic criteria for any of
the other three clinical presentations.
Etiology of ADHD
The specific underlying causes of ADHD remain largely unknown, although
many studies suggest that genetics play a significant role (Williams, Franke,
Mick, & Faraone, 2012). Like many other neurobiological disorders, ADHD
most likely results from a combination of genetic factors and environmental
factors with nutrition, brain injuries, or characteristics of the social environment
being possible contributing factors (NIMH, 2012). Many regions of the brain,
and chemicals (neurotransmitters) within the brain, have been implicated in
ADHD, with the neurotransmitter dopamine receiving the most attention
(Antshel et al., 2011). The prefrontal cortex, which serves many cognitive and
executive functions (e.g., attention, concentration, inhibition), has a high
concentration of dopamine, and it has been hypothesized that individuals with
ADHD may not produce sufficient levels of dopamine (Antshel et al., 2011).
Identifying the specific gene or genes that contribute to ADHD has proven
difficult because any single gene variant among the hundreds or thousands of
implicated genes makes only a very small contribution to genetic vulnerability
(Ross, 2012).
The prevalence of ADHD has steadily increased over the past 20 years and is
now reported as affecting 11% of children across the United States (APA, 2013).
Therefore, supports and clinical services for individuals with ADHD continue to
be an important area for health care professionals including occupational
therapy. Prevalence estimates for ADHD in children have, however, fluctuated
over time and as a result of survey (data gathering) methodologies ranging from
as low as 3% to as high as 20% of children. From 1997 to 2012, there was quite
a steady 3% to 5% increase per year (retrieved from the Center for Disease
Control, 2013). The Centers for Disease Control and Prevention (2014) estimates
that ADHD impacts the lives of 9.5% of school-aged children. The National
Institutes of Health (NIH) estimates that ADHD currently affects about 4% to
5% of adults. Wolraich and colleagues (2011) reported that approximately 8% of
children have ADHD. Although studies have consistently shown that the number
of children being diagnosed with ADHD is increasing, the reasons for this
upward trend are unclear (Wolraich et al., 2011). Visser et al. (2013) did an
extensive review and analysis of parent report data from surveys conducted by
the National Survey of Children’s Health between 2003 and 2011, which also
showed the increasing prevalence of ADHD. In 2011, according to Visser et al.,
11% of children 4 to 17 years of age from the United States had received an
ADHD diagnosis (6.4 million) at some time, and among those who had been
given an ADHD diagnosis, 83% were reported as still having the disorder
(Visser et al., 2013). Visser and colleagues also reported that 69% of children
with ADHD were taking medication for their symptoms; this equates to 6.1% of
the US population or approximately 3.5 million children. These authors noted an
overall increase of 42% in diagnosis of ADHD from 2003 to 2011 and a 28%
increase in the use of medications to treat the disorder from 2007 to 2011. Taken
together, these multiple data sources estimate the prevalence of ADHD at
between 8% and 12% of children and from 4% to 6% of adults. Approximately
50% of children continue to meet the criteria for ADHD as adults.
It is important to keep in mind that normal child behavior often includes
having a high activity level, being easily distracted, and acting impulsively. All
children mature at different rates, and their personalities, temperaments, and
energy levels are variable, which makes it challenging to discern true ADHD
from normal behavior. At what point does one begin to perceive child behavior
as a medical condition or pathological, rather than just as a variant of normal
behavior? This is a fascinating and important question, and the idea that ADHD
represents the extreme end of a normal continuum for behaviors related to
attention, inhibition, and the regulation of activity level (rather than
psychopathology) has been considered by some researchers (Holmberg,
Sundelin, & Hjern, 2013). When considering the diagnosis, it is often helpful to
be reminded that ADHD symptoms usually appear prior to age seven, and for a
diagnosis to be made, symptoms must be significant enough that they negatively
impact one’s daily functioning in more than one setting or context.
Co-occurring or Comorbid Conditions
ADHD is often comorbid with motor, sensory, learning, mood, anxiety, and
disruptive behavior disorders in children and adults, and it has also been found to
be associated with substance abuse in older adolescence and adults (Antshel et
al., 2011). Kaplan and colleagues (2001) reported that as many as 80% of
children with ADHD are at risk of having at least one other disorder, such as
learning disorders, developmental coordination disorder (DCD), oppositional
behavior disorder, depression, and/or anxiety.
Motor disorders have been reported in as many as 40% to 60% of
individuals representing all three subtypes of ADHD, with slightly more
representation in the combined clinical presentation (Egeland, Ueland, &
Johansen, 2012; Piek & Dyck, 2004). Despite the high rate of movement
problems in children with ADHD, the DSM-5 (APA, 2013) does not explicitly
recognize the comorbidity between developmental coordination disorder,
which is difficulty coordinating movements (DCD) and ADHD. Rather, it is
suggested that the motor skill problems in children with ADHD most likely are
due to their ADHD symptoms, namely, distractibility and impulsiveness, rather
than a true motor disorder. However, many children with ADHD meet criteria
for DCD, which is characterized by a marked impairment motor coordination
that is not due to a medical condition or developmental disorder and which
interferes significantly with academic achievement or activities of daily living.
Piek, Pitcher, and Hay (1999) found an association between inattentive
symptoms and fine motor ability, and Pitcher, Piek, and Hay (2003) provided
evidence to suggest that motor deficits in ADHD were a result of a specific
motor deficit rather than of ADHD symptomatology.
Disorders of attention and learning have also been associated with sensory
processing disorders or sensory integration dysfunction (Dunn & Bennett, 2002;
Mangeot et al., 2001; Pfeiffer, Daly, Nicholls, & Gullo, 2015). Yochman, Parush,
and Ornoy (2004) conducted an investigation comparing the sensory processing
abilities of preschool children aged 4 to 6 years with and without ADHD.
Results indicated that children with ADHD had an increased risk of sensory
processing difficulties such as overresponsivity and/or underresponsivity to
sensory input, as well as deficits with visual perception (Yochman, Parush, and
Ornoy, 2004). Studies have also shown that ADHD is associated with deficits in
somatosensory processing (Parush, Sohmer, Steinberg, & Kaitz, 1997), including
adverse reactions to tactile stimuli, and motor planning. More recently, Pfeiffer,
Daly, Nicholls, and Gullo (2015) found that children with ADHD were much
more likely to exhibit sensory processing challenges in all areas of sensory
processing than neurotypical children. Furthermore, their study suggested that
children with ADHD were more likely to display problems with higher level
functions believed to be dependent in part on efficient sensory processing,
including social participation and motor planning. The close association between
attention deficits and impairments with sensory, motor, and perceptual functions
is further supported by the work of Gillberg (2003) and Hellgren and colleagues
(1993, 1994) who have published widely on what they refer to as a condition
called DAMP, or deficits in attention, motor control, and perception.
Researchers over the years have questioned whether individuals with this
common clustering of problems represent a single disorder or whether they
represent an attention disorder with co-occurring disorders.
A number of mental health disorders are also commonly seen in children and
adults with ADHD including mood, anxiety, and disruptive behavior disorders.
In adults with bipolar disorder, the incidence of co-occurring ADHD has been
reported to be between 10% and 21% (Perroud et al., 2014; Perugi et al., 2013;
Wingo & Ghaemi, 2007). Consistent with these reports, epidemiological studies
of adults with ADHD have shown bipolar disorder to approximately 20% of
subjects (Halmoy et al., 2010). Several hypotheses have been raised to explain
this higher-than-chance association ranging from overlapping dimensions, such
as impulsivity and increased activity level, to shared genetic vulnerability
(Youngstrom, Arnold, & Frazier, 2010). Comorbidity between ADHD and
bipolar disorder has been associated with early-life onset of bipolar, with higher
numbers of depressive and mixed episodes, fewer asymptomatic periods, and
with worse outcomes (Karaahmet et al., 2013; Tamam, Karakus, & Ozpoyraz,
2008). In addition, individuals with both disorders show higher rates of other
psychiatric comorbidities, such as anxiety and substance abuse (Tamam,
Karakus, & Ozpoyraz, 2008).
ADHD is considered a lifelong disorder although studies suggest that only
approximately 50% of children continue to meet the criteria for ADHD into
adulthood (Visser et al., 2013), and the clinical presentation of the disorder
changes over time. Overt behaviors associated with overactivity or
hyperactivity and impulsivity tend to subside gradually with age, while
difficulties with attention and sustained focus and disorganization more often
continue into adulthood. In adulthood, a more inward type of hyperactivity has
been reported such as restlessness and difficulty sleeping, and impulsivity may
present as impatience or being ill tempered. However, it is important to note that
relatively little is known about the course of the disorder in adulthood, as there
have been few longitudinal studies examining long-term outcomes, and the
course of the disorder throughout adulthood.
There is some evidence to suggest that predictors of ADHD recovery in
adults include severity of the ADHD symptoms at age 21 and level of education
(Barkley, 2006; Kuriyan et al., 2013). Education outcomes of older children with
ADHD are somewhat discouraging, with 30% to 50% of children with ADHD
receiving special education. Children with ADHD have higher school dropout
rates (23% to 40%) and lower grade point averages than do neurotypical peers,
and fewer students with ADHD enter college (Kent et al., 2011). As adults, they
engage in more risk-taking behaviors, have poorer driving records, and have
more problems in the workplace. Individuals with comorbid oppositional defiant
disorder are at higher risk for deficient driving habits and other risk-taking
behavior (Barkley, 2006; Kent et al., 2011; Kuriyan et al., 2013).
On a more positive note, despite these findings, many individuals who have
symptoms into adulthood learn to cope with and manage their symptoms well
and live productive, happy lives. Levrini and Prevatt (2012) provide a number of
helpful strategies, including suggesting that adults with ADHD harness their
excess energy and creativity positively with careers that require a lot of stamina
or multitasking and that fit well with whatever they excel at and find most
interesting.
Diagnosis
No single test is available to diagnose a child or adult as having ADHD, and
diagnosis is made based on observations and reports of behavior, rather than on
medical or biologically based tests. This process inherently involves a degree of
subjectivity, which may account for some of the discrepancies in data related to
incidence and prevalence estimates. A licensed health professional or team of
professionals typically gathers information about the individual and his or her
behavior and environment. For children, parents often first bring their concerns
to their child’s pediatrician who may refer the family to a mental health
specialist with experience in childhood mental disorders, such as a pediatric
neurologist, clinical psychologist, developmental pediatrician, or child
psychiatrist.
Clinical practice guidelines for diagnosing ADHD from the American
Academy of Pediatrics relative to ADHD describe the diagnostic process as
involving ruling out other possibilities for the ADHD symptoms or behaviors
such as undetected hearing problems, learning disorders, or other medical
problems that might affect thinking and behavior (Wolraich et al., 2011).
Sensory integration deficits have also been identified as resulting in a similar
clinical presentation as ADHD (Miller, Neilson, & Schoen, 2012; Parush,
Sohmer, Steinberg, & Kaitz, 2007). Information gathered throughout the
evaluation process includes a detailed description of the individual’s symptoms;
when they began; how severe, excessive, and persistent they are; and how they
affect aspects of the person’s daily life and activities. Caregivers are asked if
they perceive that their child’s behaviors happen more often than do those of the
child’s peers and if they are present across multiple contexts such as school,
home, and community settings (NIMH, 2012). Specialists conducting
evaluations pay close attention to the individual’s behavior in highly structured
versus unstructured situations, performance when situated in environments that
are busy versus quiet, and behavior when the individual is engaged in activities
he or she enjoys versus those the individual does not particularly like.
Main methods of gathering information for a diagnostic evaluation of a child
for ADHD include review of relevant medical and education records,
interviewing the parent and child (if old enough), administering child behavior
rating scales, parent self-report measures, and psychological testing (Wolraich
et al., 2011). Descriptive, qualitative information about parental concerns
regarding the child’s behavior helps to narrow the focus of the evaluation, and
presents the degree of distress that the child’s behaviors are having on the family
unit. Parents provide essential demographic information, relevant family medical
history, an overview of the child’s developmental course and skills, and medical
and school history, as well as a detailed history of the child’s ADHD symptoms.
Purposes of interviewing children vary depending on the age and developmental
level of the child, but they always provide an opportunity to observe the child’s
behavior and to learn about the child’s temperament, personality, interests, and
daily activities.
A number of well-researched assessment tools or scales that offer normative
data across age groups and that are valid and reliable for quantifying the
opinions of those who know the child well are available. Two commonly used
scales that have been validated with children are the Behavior Assessment
System for Children (BASC-2; Reynolds & Kamphaus, 2005) and the
Achenbach System of Empirically Based Assessment (ASEBA; Achenbach,
2014). For adults, the ADD Rating Scale (Brown, 2001) and the Adult Self-
Report Scale (Adler, Kessler, & Spencer, 2003) are commonly used.
Psychological testing to determine an individual’s learning style, levels of
cognitive functioning, or intelligence testing such as the administration of a test
that yields an intelligence quotient or IQ is another component of the evaluation
process. Tests of academic achievement in specific subject areas such as math
and reading may also be administered to school-aged children, while other
psychological tests might focus psychosocial-emotional skills and other mental
processes or behaviors. Often, such testing is conducted to identify coexisting
conditions such as learning disorders and to supplement the other sources of data
used to diagnose an individual with ADHD.
Finally, standard medical evaluation procedures including genetic testing
may be necessary to rule out other medical conditions and childhood psychiatric
disorders that might produce ADHD-like symptoms. Allied health, discipline-
specific evaluations may be recommended such as those that address vision and
hearing, speech/language, auditory processing, sensory and motor skills, and
occupational functioning and adaptive behavior. Such evaluations not only
provide useful information that contribute to the diagnostic process but also are
useful for guiding intervention recommendations and plans.
Professionals working with adults need to consider a wider range of
symptoms because symptoms in adults tend to be more varied and less clear-cut
as those seen in children and coexisting conditions are often present. As noted
earlier, diagnostic criteria include that ADHD symptoms must appear prior to
age 12 and continue into adulthood (APA, 2013). Therefore, gathering reliable
retrospective accounts of childhood ADHD symptoms is necessary and may be
challenging. Spouses or partners, parents, or others who know the adult well are
sometimes interviewed. Clinical symptoms of inattention and
hyperactivity/impulsiveness are explored in detail to gauge the severity and
pervasiveness of the symptoms and how ADHD symptoms impact the
individual’s functioning in work and leisure pursuits and social relationships.
Finally, similar to the evaluation process for children, adults should go through
appropriate psychological testing, behavior rating scales, and medical
evaluations to rule out other possible explanations for their symptoms and to
identify any comorbid conditions. In addition to common comorbidities such as
learning, anxiety, and mood disorders, adult ADHD has been associated with
conditions such posttraumatic stress disorder, substance abuse, and obsessive-
compulsive disorder (Knouse, Cooper-Vince, Sprich, & Safren, 2008).
Medical, Pharmacological, Behavioral, and
Educational Interventions for Individuals
with ADHD
Although the management of individuals with ADHD has progressed
significantly over the past 20 years, considerable work remains to help children
and adults with this disorder. There is no cure for ADHD, but interventions can
significantly reduce the negative effects of ADHD symptoms. The presentation
of ADHD can be markedly different among children and adults, necessitating
intervention programs to be individualized. A number of intervention options are
considered, and a multimodal approach using a number of different
interventions in an effort to reduce ADHD symptoms and enhance daily
functioning is typically applied. Intervention programs consider the most current
and effective research-based therapies, as well as caregiver/individual
preferences and concerns. All care plans should include education about ADHD
and its various treatment options (medication and behavior, psychotherapies, and
other treatments), linkage with community supports and resources, and school-
based interventions for school-aged children. Specific medical and nonmedical
intervention approaches are discussed in more detail below.
Medical and Pharmacological Interventions for
ADHD
Pharmacotherapy remains the cornerstone for ADHD intervention for all age
groups, with advances in long-lasting stimulant medications being the most
common (DeSousa & Kalra, 2012). Earlier research emphasized
pharmacological interventions as being most efficacious, with little additional
value placed on behavioral and other nonpharmacological interventions
(American Academy of Pediatrics, 2011). However, more recent literature
suggests that the interpretations of earlier studies may have undervalued these
interventions, and recommendations clearly continue to support a multimodal
approach to care (American Academy of Pediatrics, 2011). Stimulant
medications such as methylphenidate (MPH), more commonly known by the
trade name Ritalin, and mixed amphetamine salts (MAS) are the most commonly
used drugs for treating ADHD in both children and adults (Barkley, 2006). They
work by increasing levels of dopamine and norepinephrine in the brain, and the
medication has a calming effect, which improves inattention, impulsivity, and
overactivity (DeSousa & Kalra, 2012). Stimulant medications come in different
forms, such as a pill, capsule, liquid, or skin patch and may be short-acting,
long-acting, or have extended-release capacities. In each of these varieties, the
active ingredient is the same, but it is released differently in the body. For
example, long-acting or extended-release forms often allow a child to take the
medication just once a day before school, so they don’t have to make a daily trip
to the school nurse for another dose. Trade names of some of the more
commonly used FDA-approved medications for children include Adderall,
Concerta, Dexedrine, and Ritalin. Side effects of the medication tend to be minor
such as decreased appetite, sleep problems, anxiety, mild head ache or stomach
upset, and irritability (DeSousa & Kalra, 2012). Side effects can often be
managed by modifying dosage or may subside over time.
Clinical research trials have been conducted to measure the effects of long-
acting forms of MPH such as Concerta, Daytrana, and Ritalin and mixed salts
amphetamine such as Adderall XR with older children (DeSousa & Kalra,
2012). Long-acting formulations have been found to be equally as efficacious as
immediate-release forms for adolescents and older children (Biederman et al.,
2007; Faraone & Buitelaar, 2010; Greenhill et al., 2002; McGough et al., 2005;
Spencer, Greenbaum, Ginsberg, & Murphy, 2009). Overall, single daily dosing is
associated with greater compliance for all kinds of medications. In studies in
which individuals were treated with both amphetamine and MPH, approximately
50% of participants responded equally to both MPH and amphetamine, whereas
almost half responded preferentially to one of the classes of stimulants, with the
overall positive response rate being as high as 85% (DeSousa & Kalra, 2012).
Unfortunately, there is no way to predict which drug will work for a particular
individual, emphasizing the importance of tailoring interventions, using a trial-
and-error approach, and a process of careful monitoring with ongoing
modifications as needed.
Stimulant medications, including extended-release forms, are also frequently
prescribed for adults with ADHD, although not all stimulant medications have
been FDA approved for adults. Antidepressants, including tricyclics, such as
amitriptyline, and atypical antidepressants, such as Wellbutrin, are also
sometimes used to treat adults with ADHD (Barkley, 2006). Adult prescriptions
for ADHD medications require special considerations because adults are more
likely than children to be taking other medications for conditions such as
diabetes or heart disease, which may interact negatively with their ADHD
medications. Children and adults who are prescribed medication should be
monitored carefully, because a one-size-fits-all approach does not apply for all
individuals with ADHD and much remains to be learned regarding the long-term
effects of these medications. More information on dosage and management of
medication are provided by the American Academy of Child and Adolescent
Psychiatry (2007) and by DeSousa and Kalra (2012) and are presented in the
clinical practice guidelines for ADHD issued by the American Academy of
Pediatrics (2011).
Nonpharmacological and Behavioral Interventions for
ADHD
Nonpharmacological and behavioral interventions include psychological
counseling and other therapy services for targeting disruptive or challenging
behaviors. Specific skills are also often addressed such as social and
organizational skills, executive and other cognitive functions, coping skills, and
sensory-motor and/or language skills. Specific occupational performance
challenges may also be addressed Therapy programs and counseling services for
individuals with ADHD often focus on teaching problem-solving strategies and
suggesting and implementing accommodations for managing the challenges of
everyday life in work, school, or other community settings. Education on
important topics that can be addressed in group and individual sessions with
older teens or adults might include relevant neurobiology and neurophysiology,
medication management, motivation and initiation of activities, and strategies to
enhance attention, concentration, self-esteem, and emotional regulation.
Behavior and psychosocial therapies represent a broad set of interventions
that are typically delivered by psychologists or other child behavior specialists.
These therapies aim to alter the individual’s behavior by modifying either the
environment or an individual’s patterns of interaction with others or patterns of
thinking (American Academy of Pediatrics, 2011). Specific behavioral
techniques may be taught and applied by parents, teachers, peers, or the
individual with ADHD himself or herself. Such techniques typically involve the
application of consistent consequences to desired behavior (rewards) and to
undesirable behavior (negative reinforcement, or punishment). For example, a
program described and evaluated by Curtis, Chapman, Dempsey, and Mires
(2013) targeted children ages 7 to 10 years with ADHD and their families. The
program emphasized parent training, instruction in self-regulation strategies,
goal setting, and rehearsal of targeted behaviors for improving the child’s
understanding of behavioral expectations.
Cognitive-behavioral therapies (CBT) focus on modifying dysfunctional
thought patterns and are based on the idea that cognitive thought processes can
be monitored and adapted and that they do affect an individual’s behavior
(Antshel et al., 2011). For example, an individual may be taught to use a process
of “stop and think” before acting, or to focus on the positive consequences of
task completion for enhancing persistence and motivation. Programs often
include training in organizing and planning such as the use of a day planner,
strategies for reducing distractibility, enhancing coping strategies especially in
stressful situations, and the development of problem-solving skills (Safren et al.,
2010). A specific type of cognitive-behavioral therapy called meta-cognitive
therapy is sometimes applied with older teens or adults (see Josman &
Rosenblum, 2011; Solanto and colleagues, 2010). This intervention applies
cognitive-behavioral principles in conjunction with techniques such as
reflection, evaluation, and adaptation of ways of thinking and problem solving.
Finally, another nonmedical intervention, peer or life coaching, is becoming
a popular intervention for adults with ADHD (Knouse, Cooper-Vince, Sprich, &
Safren, 2008). Peer coaching is an individualized approach that focuses largely
on personal goal setting, strategy selection, and accountability. Advocates of
coaching distinguish it from “traditional” therapy by describing coaching as
more action oriented rather than insight oriented, as the adult works through
specific problems or strives to reach concrete goals. Coaching sessions tend to
be briefer than sessions using cognitive-behavioral therapies and can be
delivered on as “as needed basis,” rather than on a more regular basis. Specific
goals might be targeted such as finding a more satisfying job or living a healthier
more balanced lifestyle. Exercises or activities might be implemented to
determine what brings the most meaning to one’s life as a process of discovery,
followed by the development of a plan for engaging in those meaningful
activities.
Parent Education and Training Programs
Parent training programs are designed for parents of preschool- and school-aged
children with ADHD. They focus on teaching strategies for fostering positive
child-parent relationships and for managing challenging or disruptive behaviors
and provide parents with support and education about the disorder (Charach et
al., 2013). While different programs have some unique attributes, they all aim to
help parents manage their child’s problem behavior by emphasizing the use of
effective discipline strategies involving rewards and nonpunitive consequences.
Parent training programs also promote positive child-parent relationships by
increasing parents’ understanding of their child’s temperament, and awareness of
their own parenting style. Helping parents understand why their children behave
the way that they do is a first important step in the process. Parents learn how
their child’s symptoms interact with their own parenting styles/ways of
interacting and with unique stress factors. Specific problem behaviors are then
targeted typically through the teaching and application of behavioral techniques,
primarily associated with positive reinforcement (Sanders, Bor, & Morawska,
2007). Parent training programs aim to promote a positive and caring
relationship between parents and their child, reduce the child’s problem
behaviors, and enhance overall parenting skills (Charach et al, 2011; Matos,
Bauermeister, & Bernal, 2009; Thompson et al., 2009).
Educational and Classroom Interventions
School-related interventions are a critical component of a comprehensive
intervention program for school-aged children with ADHD, as children with
ADHD are more likely to have poorer grades, higher dropout rates, and higher
absenteeism as compared to their peers without the disorder (DuPaul &
Weyandt, 2009; DuPaul, Weyandt, & Janusis, 2011). School-aged children
whose ADHD symptoms affect their ability to participate fully and benefit from
public education programs in the United States are eligible to receive an IEP,
including special education and related support services through the Individuals
with Disabilities Education Act (IDEA; U.S. Department of Education, 2004).
Under the U.S. Rehabilitation Act of 1973, federal legislation that extended civil
rights protection to people with disabilities, children with ADHD who need only
reasonable accommodations (rather than specialized instruction) to succeed in
school may receive such accommodations through a 504 plan rather than
through an IEP.
Behavioral programs are commonly implemented in schools and follow the
same basic behavioral principles of reinforcement that are used in other settings.
Teacher praise or token reinforcements in the form of stickers or points that can
be exchanged for rewards such as time spent in preferred activities (e.g.,
computer time or recess) are commonly used (DuPaul, Weyandt, & Janusis,
2011). Alterations in curricula, environmental adaptations, or academic
interventions, which facilitate a child’s interest, motivation, and ability to persist
when challenged can also be implemented. For example, teachers might provide
their ADHD students with clear expectations and rules, give students choices, or
reduce task demands by breaking assignments into smaller or shorter
components. Preferential seating (seating the child in a place that is relatively
free from distraction or close to the teacher for frequent monitoring or cueing) or
creating a designated quiet area is often helpful (DuPaul, Weyandt, & Janusis,
2011).
Training in specific skills such as general study and organizational skills,
note taking, self-regulation, and social skills has also been found to be effective
(DuPaul, Weyandt, & Janusis, 2011). There are a number of computer-assisted
math, reading, and writing programs that have been shown to enhance the on-
task behavior and overall performance of children with ADHD (DuPaul,
Weyandt, & Janusis, 2011). Peer tutoring or other peer-mediated interventions
have also been used successfully in school-based settings and have been found
not only to positively impact school performance but also to improve social
skills and friendships. Typically, this intervention involves pairing a child with
ADHD with a higher achieving student who provides instruction and feedback to
the student with ADHD (Daley & Birchwood, 2010; Plumer & Stoner, 2005).
For young preschool-aged children, symptoms of ADHD often hinder their
ability to play effectively with peers, control and manage their behavior in the
home and community, participate in a variety of learning activities, and perform
basic self-care skills. For older children, challenges in their roles as students are
particularly common, as well as the quality of their performance of basic and
instrumental activities of daily living. For adults with ADHD, social and
community participation, sustaining meaningful relationships, and functioning in
the workplace may be negatively affected by symptoms of the disorder. It is also
important to note that common coexisting conditions, such as learning, sensory,
motor, mood, and/or anxiety disorders when present, also impact one’s ability to
perform one’s daily occupations, and it is often difficult to discern what
condition is most responsible for certain problems.
The main occupations of humans include activities of daily living (such as
personal self-care activities like bathing, dressing, and eating), instrumental
activities of daily living (such as homemaking tasks and money management
tasks), rest and sleep, education, work, play, leisure, and social participation
(American Occupational Therapy Association, 2014). For individuals living with
ADHD, symptoms affecting the ability to attend, concentrate, adapt, plan,
organize, and control emotions may impair performance in any of these
occupational areas. School performance and student roles have probably
received the most attention for individuals with ADHD (DuPaul & Weyandt,
2009) with challenges noted with academic work, classroom behavior, study
skills, and motor skills including handwriting.
Children and adolescence with ADHD may exhibit problems with learning,
initiating, organizing basic self-care tasks such as completing a morning routine
of grooming, and dressing in a timely and thorough manner (Dunn, Coster,
Cohn, & Orsmond, 2009; White & Mulligan, 2005). For adults, instrumental
activities of daily living, social participation, and work occupations are most
often impacted. Adults with ADHD have been shown to have greater risk for
lower socioeconomic status and lower rates of professional employment
(Cermak & Maeir, 2011). Driving is also an area that has been given attention in
the ADHD population, as teens and adults with ADHD have been shown to be at
a higher risk for accidents and violations than drivers without the disorder
(Reimer, Mehler, D’Ambrosio, & Fried, 2010).
Participation in leisure activities and play skills is also often compromised in
children and adults with ADHD. Shimoni, Engel-Yeger, and Tirosh (2010), for
example, found that boys with ADHD reported less enjoyment from their leisure
activities than did their typical peers. Cordier, Bundy, Hocking, and Einfeld
(2009) studied the play behaviors of children 5 to 11 years of age with ADHD
and found that they had more difficulties in social play and had less interpersonal
empathy in comparison with typical peers. Habits, routines, and roles supporting
one’s engagement in his or her occupations may also be disrupted in individuals
with ADHD. Interventions targeting these areas may help those with ADHD
overcome poor habits such procrastination or change disruptive behavioral
patterns characterized by beginning many new projects, but then not following
through with them to completion. Environmental and contextual factors such as
clutter and crowds, poor lighting, and/or excessive noise hinder the performance
of all individuals. However, the negative influence of these environmental
factors on one’s occupational performance may be exaggerated in individuals
with ADHD. Sociocultural environments or expectations within a particular
context may also be particularly challenging for individuals for ADHD such as
those that are highly unstructured, require long periods of sedentary work, or
lack of interesting activity or stimulation. Successful engagement of their
occupations may therefore be enhanced by modifying or addressing
environmental and/or contextual elements
Summary
This chapter has provided information on the diagnosis, etiology, clinical
presentation, prevalence, and prognosis of ADHD. The many ways in which
ADHD symptoms commonly impact one’s occupational performance or the
ability to successfully participate in one’s daily life was also described, along
with an overview of methods of evaluation and treatment for both children and
adults. It is important to emphasize that ADHD is a complex condition and every
client with the disorder is unique. Best practice is described as implementing a
multimodal intervention approach that is individually tailored to meet the
unique presentation of each client, with consideration of the client’s own goals
and priorities, and in concert with the other medical, pharmacological,
psychological, and/or educational interventions that the individual might be
receiving. Despite continual advances in our understanding of the disorder, and
its typical course throughout one’s life, there is still much to be learned about the
nature of the disorder and how best to treat children and adults with ADHD.
An adult case study and a child case study are presented below to illustrate
how ADHD may present in specific individuals, and to provide an opportunity
for you to work through possible evaluation methods, models of intervention,
and potential outcomes. As you read through the case studies, it may be helpful
to address and be thinking about the following questions: (1) What type of
ADHD does this individual present with, and how do ADHD symptoms affect
his or her ability to carry out their required and desired occupations? (2) Was the
evaluation conducted comprehensive, or would there be other information that
would have been helpful to gather to assist in guiding intervention? (3) What
medical, psychological, educational, or occupational intervention strategies or
approaches would be appropriate to consider for this individual?
CASE-STUDY 1
Cory, at 5 years of age, was recently diagnosed with ADHD-combined
presentation by his pediatrician. His parents were having difficulty
managing his behavior in the home including frequent temper outbursts,
hyperactivity, and perceived immaturity in his play skills and inability to
carry out basic self-care tasks. He was started on Dexedrine to help manage
his symptoms, and he was planning to start kindergarten in the fall, in 4
months time. He had not ever received therapy or support services in the
past, although he had attended a community preschool program two
mornings a week for 3 months when he was 4 years of age. His medical
history included frequent ear infections and upper respiratory infections and
was otherwise unremarkable. Height and weight were average, and he was
followed regularly by his pediatrician. Cory lived at home with his parents
and two siblings, 2 and 8 years of age. His mother stayed at home to
manage the household and care for the children, and his father worked as a
university professor. His father had a history of ADHD, and both parents
were quite knowledgeable about the disorder. Interviews with his parents
revealed that Cory was full of energy and protested consistently when he
did not get his way, often to the point of being aggressive. Medication had
settled his behavior somewhat, although his mother was concerned
regarding side effects including loss of appetite and sleep problems.
During the weekdays, he spent most of his time playing with his 2-year-
old sister. He usually got up in the morning around 7:00 and had an
afternoon nap early afternoon for 2 to 3 hours. He enjoyed playing with
construction toys like big blocks and with his train set. He liked playing
outside on the swing set, playing with balls of any kind (running and
kicking), and riding his two-wheeled bike with training wheels. Cory
needed assistance with his self-care including dressing and toileting and
only recently was toilet trained, although he continued to have occasional
accidents. His mother reported that Cory was able to assist with brushing
his teeth and washing his face. He was able to eat using a spoon and fork,
although his mother stated that he was a very messy and picky eater and
rarely would sit at the dining room table for more than about 5 minutes.
Cory often protested when it was time to get dressed or during grooming
tasks. His mother said that helping Cory with basic self-care tasks and
taking the children out on the weekends to the park, the mall, or other
community settings were very exhausting, and she was hoping to get some
ideas on how to better manage Cory’s behavior.
Cory was referred for occupational therapy at an outpatient pediatric
clinic, and his occupational therapy evaluation consisted of informal
interview with his mother; observations of sensory processing, play, and
motor skills; and administration of the three standardized assessments
including the Sensory Processing Measure, Preschool Home Form (Parham,
Ecker, Kuhaneck, Henry, & Glennon, 2010); the Miller Function and
Participation Scale, Home Form (Miller, 2006); and the Brief Rating
Inventory of Executive Functions, Preschool Form, by Giola, Isquith, Guy,
and Kenworthy (2000). The evaluation revealed that Cory presented as a
happy, talkative child who was full of energy with an increased activity
level. Instructions often needed to be repeated during standardized testing,
and Cory experienced difficulty persisting at a task when challenged, often
saying “No I can’t. I am all done.” Cory tended to move quickly from one
activity to the next with limited productive and sustained play at any one
activity, and he behaved impulsively. His gross motor skills fell within the
low-average range, and visual-motor and fine motor skills both fell
significantly below average. Sensory processing concerns on the Sensory
Processing Measure were noted in the hearing, touch, balance, and social
participation areas and included sensation-seeking behaviors (especially
excessive movement), tactile hypersensitivity, and not being able to
adequately filter irrelevant noise and other stimuli.
His mother reported that she wanted assistance with managing Cory’s
impulsivity, lack of attention to tasks, and noncompliance. Her requests of
him were often ignored, or he would have a temper outburst that involved
screaming and sometimes hitting or pinching her. She was also concerned
that he did not share and take turns well when playing with peers and was
fearful that Cory might hurt his younger sister when interacting with her at
home. His lack of independence with basic self-care skills and fine motor
skills was also of concern, and she was worried that he would experience
difficulties when starting kindergarten. His assessment revealed that during
play, Cory insisted on controlling the play situation, always wanting to
assume the leader role. Observations of play indicated limited problem-
solving strategies, minimal creative and goal-directed play, and need for
control and for immediate gratification.
In summary, the occupational therapy evaluation indicated that Cory
had some coexisting sensory integration deficits, including decreased
visual-motor integration and sensory modulation deficits. Executive
function deficits consistent with the ADHD-combined presentation were
noted, including inattention, disorganization, increased activity level,
impulsivity and lack of self-control, and poor problem solving. His
executive functioning and sensory motor problems impacted his ability to
perform many of his daily occupations including play, his performance of
basic self-care skills, and, most importantly, his ability to cope with stress,
regulate his behavior, and exhibit self-control especially when asked to
perform an undesirable or challenging task. Despite these challenges, Cory
was very interested in his environment and seemed eager to please others,
and many developmental areas and skills were progressing typically, such
as use of language and gross motor skills.
CASE-STUDY 2
Amanda was diagnosed with ADHD predominately inattentive presentation
when she was a high school student and had been taking stimulant
medication off and on since then. At 26 years of age, she was referred for
occupational therapy by her counselor, a clinical psychologist, to address
some challenges she was having in the workplace and with community
living skills. She was living in an apartment with a roommate and was
receiving counseling for anxiety and to assist in managing her symptoms
associated with ADHD. Occupational therapy evaluation methods included
informal interviews with Amanda and her mother, which were conducted
separately; a workplace observation; administration of the Canadian
Occupational Performance Measure (Law et al., 2005); and administration
of the Behavior Rating Inventory of Executive Function, Adult Form (Roth,
Isquith, & Gioia, 2006).
Evaluation results revealed that Amanda was employed full time as an
administrative assistant for a property management company. Her parents
lived about an hour away in a neighboring town; she had two adult siblings
who lived in other states. Socially, she spent leisure time with her roommate
and reported having a few other friends whom she saw regularly. She
worked out at a gym regularly and enjoyed listening to music and watching
movies. Aside from ADHD, her medical history was unremarkable with no
major illness or hospitalizations. During the weekdays, she typically
worked 10 AM to 6 PM. She typically got up at 9 AM and took public transit
(bus) to and from work. Although she had a driver’s license, she did not
have a car. She enjoyed cooking dinner and often would prepare dinner for
both her and her roommate. In the evenings, she often went to the gym and
watched TV. On weekends, she often visited her parents and enjoyed
catching up on her sleep. Evaluation results indicated that Amanda wanted
to perform better with her job, and she expressed a need to improve her
organizational skills. She reported that she always felt busy doing things
and had a lot of energy but that tasks never seemed to get done. She
enjoyed starting new projects in the home and at work, but often, there was
little follow-through to completion. She was easily distracted and had not
developed effective ways of prioritizing and organizing her time. Results
from the Behavior Rating Inventory of Executive Function, Adult Version
(BRIEF-A; Roth, Isquith, & Gioia, 2006), indicated some executive
function deficits in the ability to plan and organize tasks and with working
memory. Her ability to self-monitor her behavior was scored in the
dysfunctional range, while emotional control, inhibition, and ability to shift
and adapt were scored within the average range.
A worksite, 1-hour observation revealed that Amanda had a demanding
job that required her to frequently multitask (talk on the phone, schedule
appointments, meet with clients, problem solve tenant complaints and
concerns, assist contractors, organize records, etc.). She would often begin a
task, get interrupted, and then forget to go back and complete what she was
doing. The office space was very open, bright, and cheery, although the
reception area and her space were very close to the waiting area, which was
noisy most of the time. Amanda said that she always felt stressed at work
and felt as though she was always behind and not meeting expectations. She
was capable of doing the tasks asked of her, but just did not have the time to
get everything done, and she was fearful of getting fired. There was one
other person who worked in the front office area. They seemed to have a
good, comfortable working relationship, and each had their own
responsibilities and also shared some responsibilities. Amanda reported that
she most wanted to improve her financial situation and improve her time
management and efficiency at work and ability to cope with work.
Evaluation results suggested deficits in her ability to sustain focus and
attention to tasks and decreased organizational and problem-solving skills.
RESOURCES
Recommended Books
Taking charge of ADHD, revised edition: The complete authoritative guide for
parents, by Russell Barkley Taking charge of adult ADHD, by Russell Barkley
with Christine Benton ADHD answer book: Professional answers to 275 of the
top questions parents ask, by Susan Ashley Overcoming ADHD without
medication: A parent and education’s guide association for youth, children, and
natural psychology The disorganized mind: Coaching your ADHD brain to take
control of your time, tasks and talents, by Nancy Ratey Rethinking ADHD:
Integrated approaches to helping children at home and at school, by Ruth
Schmidt Neven Succeeding with adult ADHD: Daily strategies to help you
achieve your goals and manage your life, by Levrini and Prevatt (2012),
American Psychological Association.
Recommended Organizations and Web sites National Institutes of Mental
Health
www.nimh.nih.gov/health/publications/attention-deficit-hyperactivity-
disorder/index.shtml
Center for Disease Control
www.cdc.gov/ncbddd/adhd/research.html
Children and Adults with Attention deficit/Hyperactivity Disorder
(CHADD) www.chadd.org
Attention Deficit Disorder Association
www.add.org
ADHD Aware
www.adhdaware.org
American Psychiatric Association
www.psych.org
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C H A P T E R
7 Sensory Processing Disorder
Ben J. Atchison Brandon G. Morkut
KEY TERMS
Dyspraxia Neuronal pruning Nosology
Sensory craving Sensory deprivation Sensory discrimination Sensory
modulation Sensory overresponsivity Sensory underresponsivity
Somatosensation
Since the age of two, it seemed that 4-year-old Thomas responded to stimuli
around him in a way that was unusually intense. For example, when a sudden
noise occurred, such as turning on the vacuum or the blender or when someone
knocked on the door or rang the doorbell, he would stop and fall to the ground
with excessive outbursts of emotion, with his hands over his ears crying “Stop,
stop.” Consoling him after this happened was very difficult. After being dressed
for the day, he would often remove his clothing, preferring to play in his diaper,
and often took that off as well. Attempts to put his clothing back on were always
a battle as he fussed and cried and took his clothes off after being dressed again.
Mealtimes were increasingly challenging as well as his food preferences were
limited to smooth and soft textures. Clearly, it was going to be a challenge for
Thomas to begin his preschool program in a few months. After describing his
behaviors to a close friend whose child had similar difficulties, Thomas’ parents
were advised to look into the possibility that he was experiencing something
called a sensory processing disorder (SPD).
In the early 1960s, the late Dr. A. Jean Ayres, an occupational therapist and
neuroscientist at the University of Southern California, reported a series of
clinical studies that emphasized the connection between the important roles of
sensory processing in learning and behavior. Beginning with this statement in
her classic textbook, Sensory Integration and Learning Disorders, published in
1972, Ayres noted: “Learning is a function of the brain; learning disorders are
assumed to reflect some deviation in neural function” (p. 1). Ayres described a
theoretical model of sensory integration that included treatment guidelines. She
was insistent that while her theory was built on brain and behavioral research, it
was formative as theories must be and that her findings were tentative and not to
be considered conclusive. From her pioneering work in the mid 20th century to
the present time, a series of scientific investigations have been published, mostly
by occupational therapists, to expand theoretical constructs of sensory
processing as well as develop informal and formal assessment procedures and
intervention guidelines for children and adults with what Ayres initially
identified as sensory integration dysfunction (SID).
Miller, Anzalone, Lane, Cermak, and Osten (2007) published the seminal
article Concept Evolution in Sensory Integration: A Proposed Nosology for
Diagnosis. Based on input from focus groups of occupational therapy scholars
and empirical evidence, the authors proposed that the term SID be replaced with
SPD. Further, three SPD phenotypes were described: sensory modulation
disorder (SMD), sensory-based motor disorder (SBMD), and sensory
discrimination disorder (SDD), each with subtypes (as shown in Fig. 7.1). These
typologies will be described in detail in the section on signs and symptoms.
In conjunction with this proposed SPD nosology, a term used to classify
medical conditions, Miller and her colleagues also recommended a more well-
defined distinction regarding terminology. They proposed that the theory be
identified as sensory integration theory, the disorder as SPD, and that
intervention be referred to as occupational therapy using a sensory integration
approach (OT-SI). It is important to note that Miller and her colleagues were
explicit, as was Ayres, that further scholarly dialogue and research on the
proposed nosology were to be expected and required for continued evolution of
SPD concepts.
Etiology
While there is no specific identified cause of SPD, there are a number of studies
that point to extrinsic and intrinsic factors that result in the difficulty of the
central nervous system (CNS) to detect, register, and process sensory
information. Preliminary research continues to hypothesize that heredity,
environmental factors, prenatal conditions, and perinatal complications (e.g.,
jaundice, forceps/vacuum delivery, and fetal distress) are strong factors for SPD.
Miller (2014) designed a small research pilot (N = 26) to identify possible
correlation between heredity and SPD and found a relationship between SPD
and heredity as evidenced by 92% of the sample children having at least one
parent who identified that his or her own behaviors were indicative of SPD.
Forty percent of the participant’s mothers recognized personal attributes
associated with SPD in comparison to 37% of fathers.
One of the most significant environmental factors believed to influence SPD
is sensory deprivation or a considerable lack of quality sensory stimulation, as
evidenced by children living in Eastern European orphanages (Miller, 2014). As
a result of immature sensory systems at birth (e.g., tactile, proprioceptive, visual,
auditory, and vestibular), substantial alterations take place postnatally for all
humans. During infancy, connections between sensory neurons continue to be
established to allow sensory systems develop functionally by way of appropriate
modifications in neural pathways. Early repetitive exposure to nonthreatening
environmental events, which promote sensory processing and spontaneous
activity, is required to facilitate the maturation of neuronal circuits. Studies have
also shown that exposure to sensory-rich experiences during childhood is
essential in the exponential speed of and overproduction of neuronal
development. This overproduction, in turn, promotes neuronal pruning.
Neuronal pruning is a necessary process to eliminate the overproduction of
sensory neurons that are generated mostly in infancy through middle childhood.
This allows better efficiency in learning new information and explains, in part,
the neuroplasticity of brain development (Fox, Levitt, & Nelson, 2010). When
infants are deprived of sensory enrichment, the natural process of
overproduction and pruning does not occur. By way of example, it was
discovered that children living in the former communist bloc within Eastern
European orphanages were not often provided adequate conditions for typical
sensory development to occur, which resulted in poor developmental and
atypical behavioral outcomes (Lin, Cermak, Coster, & Miller, 2005). Research
data suggest that internationally adopted children who were institutionalized 12
months or more demonstrated severe SPD patterns of dysfunction when
compared to those adopted at <8 months with minimal or no institutional
placement. Those in the former group experienced higher reactivity and aversion
to tactile, vestibular, proprioceptive, visual, and auditory input as well as
difficulty with motor planning skills, or dyspraxia (Wilbarger, Gunnar,
Schneider, & Pollak, 2010).
Other significant factors that are believed to contribute to SPD are chronic
exposure to pre- and postnatal stressors and neurodevelopmental difficulties.
Studies have shown that chronic prenatal stress and alcohol exposure have been
identified to impact sensory processing in primates. Furthermore, studies of
humans have indicated that maternal stress hormones (e.g., adrenaline and
cortisol) can cross the placental barrier resulting in altered neurotransmitter
activity, thus facilitating abnormal sensory modulation in offspring (Brunton,
2013). Research outcomes reported by Atchison (2007) identified significant
SMDs in children who experienced prenatal exposure to alcohol in conjunction
with postnatal abuse. Among the same population, Henry, Sloane, and Black-
Pond (2007) found severe neurodevelopmental deficits in language, memory,
visual processing, motor skills, attention, and behavior.
Presently, there is a lack of strong empirical evidence to determine a
definitive etiology for SPD. However, emerging evidence continues to suggest
that SPD reduces a child’s ability to engage in daily activities, such as social
interaction, self-care, learning, and play (Schaaf et al., 2010). Hypothesized risk
factors associated with SPD include the following (Ben-Sasson, Carter, &
Briggs-Gowan, 2009; Keuler, Schmidt, Van Hulle, Lemery-Chalfant, &
Goldsmith, 2011; May-Benson, Koomar, & Teasdale, 2009; Schneider et al.,
2007, 2008, 2009; Wickremasinghe et al., 2013):
Low birth weight (<4.5 lb)

Prematurity (<36 weeks gestation)
Maternal illness
Maternal use of medications
Living with a single parent
Lower socioeconomic status
The search continues for evidence that SPD is a unique disorder with distinct
etiologic factors and pathomechanics. At the time of this publication, research
scientists at major centers around the world, from a variety of scientific
disciplines, are conducting primate studies, rat studies, and anatomical and
genetic studies. Studies of specific patterns of sensory modulation, using precise
measures such as electroencephalography, neuroimaging, and electrodermal
reactivity, which is a measure of stress and emotion, are being conducted and
reported in the literature (Miller, 2014). For example, recent research (2013)
discovered, by way of specialized neuroimaging technology, that the brains of
boys with SPD were found to have unique abnormalities in the posterior white
matter including the posterior corpus callosum, posterior corona radiata, and
posterior thalamic radiations. These structures are implicated in interconnections
of auditory, visual, and tactile systems as well as the two cortical hemispheres
(Owen et al., 2013). An excellent resource for recent and current studies are
archived and continually updated at: www.spdfoundation.net.
Performance dysfunction related to sensory processing has been identified in all
age groups, intellectual levels, and socioeconomic groups. In a systematic
review of the literature related to prevalence of SPD, Ahn, Miller, Milberger, and
McIntosh (2004) examined the prevalence of SPD among 1,800 kindergartners
attending a Colorado suburban public school for the 1999–2000 school year. The
Short Sensory Profile (SSP) (Dunn, 1999) was administered to 1,796 parents,
which resulted in 703 surveys completed and returned, yielding a 39% return
rate. Of the 703 children, results indicated 96 children (13.7%) met criteria for
an SPD evaluation. Based on the study’s empirical evidence, researchers
cautiously suggest that approximately 220,000 kindergartners in the United
States may demonstrate a critical threshold of symptoms to warrant an
evaluation to rule out SPD (Miller, 2014). In addition, a longitudinal study (N =
1,312) (Ben-Sasson et al., 2009) investigated the prevalence of sensory
overresponsivity (SOR) in a demographically proportionate Connecticut
elementary school. Data collection methods included the Sensory Over-
Responsivity Scales (SensOR; Schoen, Miller, & Green, 2008) in conjunction
with additional parent-report instruments. Parent-rated surveys were distributed
at three predetermined periods among the sample population beginning at (1) 11
to 42 months of age, (2) 22 to 56 months, and (3) 7 to 11 years old. Final data
analysis indicated 148 (16.5%) children of the sample population acquired
notably elevated SOR scores, as defined by identifying four or more unsettling
sensations on the SensOR (Schoen et al., 2008). Investigation of demographic
risk factors that may influence SOR symptoms identified the population with
elevated SOR results (N = 148). They were more likely to be of minority
ethnicity, of lower economic status, and living with a single parent and to have
experienced considerably lower birth weight and gestational period. In addition,
76.4% (N = 113) of the population with elevated SOR results identified tactile
input as the most bothering sensation (Ben-Sasson et al., 2009). Even though
evidence supports the existence of SPD as an isolated disorder, Miller (2014)
and colleagues validated SPD as a “combination disorder” (p. 267) reflective of
more than one SPD pattern and/or subtype. In an additional study, Miller (2014)
investigated the existence of co-occurrence among the subtypes of SMD and
patterns of SPD in children ages three to 12 years old (N=200). Results indicated
that 59% of the sample population possessed combined subtype symptoms of
SMD in conjunction with 70% of subjects validating combined patterns of SPD.
The most frequent SPD pattern/subtype combinations involve motor planning
difficulty with sensory craving (SC), motor postural disorder, and sensory
underresponsivity (SUR) with postural disorder (Miller, 2014).
Disruption in sensory processing has also been most frequently recognized
in children with autism spectrum disorders (ASDs), attention deficit
hyperactivity disorder (ADHD), learning disabilities, fragile X syndrome, and
developmental/complex trauma. Tomchek and Dunn (2007) investigated sensory
processing dysfunction in children between ages 3 and 6 years (N = 281)
diagnosed with ASD. Results indicated that 95% of the children with ASD
displayed some degree of sensory processing dysfunction in the pattern of SMD
in conjunction with SOR as the co-occurrence subtype most frequently identified
with ADHD (i.e., 40% to 60%) and fragile X.
Signs and Symptoms
Participation in daily activities requires the CNS complex process of organizing
internal and external sensations to allow for a functional response that is
meaningful and appropriate to a given demand. Sensory processing includes the
detection, modulation, integration, interpretation, and organization of sensory
sensations (Bialer & Miller, 2011). It is postulated that individuals who display
SPD symptoms encounter errors in the analysis of sensory information in the
brain. Since all humans experience interpretation errors in sensory processing, it
is the deficiency of habituation and adaptation to these sensations that results in
chronic manifestations of poorly organized behavior responses. Therefore,
intervention is only recommended when the inability to process and organize
sensory input significantly impacts one’s capability to facilitate a functional
response toward a daily occupation. Schaaf and Miller (2005, p. 1) note, “The
goal of intervention is to improve the ability to process and integrate sensory
information and to provide a basis for improved independence and participation
in daily life activities.” Furthermore, three patterns of SPD have been identified,
each with subtypes as described below and illustrated in Figure 7.1.
Figure 7.1 Typologies of sensory processing disorder. (Adapted from Sensory
Processing Disorder: A Conceptual Model. From Miller, L. J., Anzalone, M. E.,
Lane, S. J., Cermak, S. A., & Osten, E. T. (2007). Concept evolution in sensory
integration: A proposed nosology for diagnosis. American Journal of
Occupational Therapy, 61(2), 135–140.)
Pattern 1: Sensory Modulation Disorder
Sensory modulation allows for purposeful and successful participation in
activities of daily living, which is dependent on the central and autonomic
nervous system’s ability to maintain “system harmony” through a complex
process of regulating and organizing the intensity of sensory input to facilitate a
functional graded response. However, a disorder in sensory modulation occurs
when difficulty with regulating and organizing external or internal sensory
stimuli results in a maladaptive emotional or motor response. Evidence suggests
that SMD can impact one or more of the seven sensory systems: vestibular,
tactile, visual, proprioception, auditory, olfactory, and/or gustatory as supported
by laboratory measures of altered physiological responses to specific stimuli
(James, Miller, Schaaf, Nielsen, & Schoen, 2011). Within SMD, three subtypes
have been identified including SOR, SUR, and SC.
Subtype 1: Sensory Overresponsivity

This subtype is characterized by responses to stimuli that are more intense or
have a longer duration than typically expected and may occur in only one or
among multiple sensory modalities. Oftentimes, the behaviors that children
demonstrate are judged to be intentional because the response sets are often
context dependent. For example, the same type of auditory stimulus that sets off
an adverse behavioral reaction in the classroom but is not observed at home will
often lead to the conclusion that the child can control the behavior when in fact it
is automatic and unconscious. At home, the child may have more control of an
auditory stimulus where, in the classroom, the same stimulus occurs without
expectation. Repetition of this unexpected stimulus can create a cumulative
effect, resulting in a range of aggressive reactions to complete avoidance or
shutdown in the stimulus environment. Miller et al. (1999) demonstrated, in a
laboratory model, that activation of the sympathetic nervous system is a marker
of SOR, specifically noted in the exaggeration of the fight or flight responses
typically experienced in stressful situations. Persons with SOR often
demonstrate inflexibility and hypervigilance and tend to be difficult to console
when they are in “overresponsive” mode. SOR is often seen in children with
autism, fragile X syndrome, ADHD, and mood disorders (Schaaf et al., 2010).
Subtype 2: Sensory Underresponsivity

Children who experience SUR experience challenges in the feed-forward
mechanism of the CNS, lacking a central predictive set of actions in given
situations (Miller, 2014). It is as if there is a lack of ability to detect incoming
sensory information, which is often misinterpreted as the child having “lazy”
tendencies and being unmotivated. The infant and toddler with SUR are often
considered to be “an easy baby” as there seems to be less demands on the
caregiver. However, this same child shows less interest in active exploration of
the environment, which can best be explained by a hypoaroused manner, which
manifests in a lack of natural, exploratory sensory behavior and may be
manifested in inefficient tactile and proprioceptive sensory responses, which are
typically robust in developing infants and toddlers (Quake-Rapp & Atchison,
2011). It is soon discovered that the child with SUR requires a more intense and
extended duration of sensory stimuli to reach an optimal level of arousal. Most
caregivers will intuitively “turn up the volume” of sensory stimuli to address this
behavior. A child with SUR often demonstrates difficulty with sustained task
performance as the lack of a consistent interaction with sensory input, which can
lead to SDDs, motor planning difficulty, or both (Miller, 2014).
Subtype 3: Sensory Craving

While it is clear in the human growth and development literature, including
teratologic studies, that humans seek sensory input to make meaning of the
external environment, children with this subtype of SMD seek sensory input
with an approach that results in erratic, disorganized behavior. The child in the
physician’s office who wants to touch the reflex hammer, stethoscope, and other
available items within reach is different than the child who runs about the office,
turning things over and generally creating concern among those in the room. The
behavior appears to be impulsive, unsafe, and boundary-free, and the child will
energetically engage in physical actions that add to his or her craving for sensory
input. Oftentimes, this child is considered to be “fearless” as he or she engages
in constant spinning, jumping, and running into objects with a “crashing and
bashing” approach. When the necessary boundaries are enforced and thus unable
to meet sensory needs, children with SC may become highly agitated and
explosive. With extreme cases, the ability to learn is compromised, interpersonal
relationships become very difficult to sustain, and activities of daily living are
disrupted (Quake-Rapp & Atchison, 2011).
Miller describes SC as an active seeking of sensation that is “insatiable” and
often results in significant disorganization and less-than-acceptable behavior
(Miller, 2014). These are children who are removed from the classroom for
behavioral concerns as early as preschool. Other children often complain of the
child with SC behaviors due to their aggressive social interaction and intrusive
interruption of others’ play space. Children who crave sensation often have a
need for increased volume when viewing a video and will speak in a loud voice
or shrill, frenetic pattern of verbal communication. When on the playground,
these children cannot get enough of the varied stimulation from the equipment
whether it be rotation or angular or vertical stimulation that comes from
jumping, climbing, swinging, and twirling. Oftentimes, these children are
described as being very active, yet this high activity is beset with disorganized
and less purposeful outcomes than a child engaged in typical, robust play. Miller
(2014) suggests that there are two distinct groups of sensory cravers with the
first being more common, with an observed need for “more and more”
stimulation that is difficult to control, or self-regulate. The second type is less
common but includes those who seek out extreme sensory experiences and
experience self-regulation of their craving tendencies. This group of sensory
cravers has outcomes that are less disorganized, with more purpose as seen in
“extreme sports” activities such as cliff diving, parachuting, high-speed auto
racing, and snowboarding.
Pattern 2: Sensory-Based Motor Disorder
Subtype 1: Postural Disorders
Those body functions that are dependent on smooth and efficient processing of
vestibular and kinesthetic input are often disrupted among children with SPD,
resulting in difficulty with maintaining normal postural tone. Smooth control of
movement and endurance may be compromised, particularly in antigravity
activity. Poor balance between agonist and antagonist muscle groups, poor
balance reactions in both static and dynamic challenges, poor weight shifting and
trunk mobility, and deficits in smooth oculomotor control may be present in
children with sensory-based motor disorder (SBMD). Children with either
sensory seeking or underresponsivity may demonstrate behaviors associated with
SBMD. The arousal level of the person, whether it is SOR or SUR, may result in
postural control problems and difficulty with motor planning. The child who
typically maintains a slumped position while attempting to complete a
handwriting task and quickly losing a sustained effort to do so is typical of a
child with this pattern of SPD (Miller, 2014).
Subtype 2: Dyspraxia
Dyspraxia, or difficulty with motor planning, is the inability to plan unfamiliar
or novel tasks, resulting in a clumsy, awkward, maladaptive response. The key
idea here is that the task is novel. Asking a person to perform a task that is done
routinely, such as buttoning a button on a shirt, would be an evaluation of fine
motor coordination and less motor planning ability. When attempting a motor
task that is not in one’s repertoire of acquired skills, the person with dyspraxia
will demonstrate significant awkwardness in the use of hands and body to
perform the task. It is clearly a sensory-based problem in that persons with
dyspraxia appear to be unsure of where their body is in space and may have
difficulty with timing, grading, and executing movement to complete the task
with success. Most people have experienced the awkwardness of learning new
dance steps. After a series of repetitions, a more accurate use of the body is felt
and observed in most individuals. It is true, of course, that some of us will never
learn to dance! That, however, may be tied to a lack of interest rather than poor
somatosensory processing. Usually, there are other motor planning challenges
that one can succeed in, unless dyspraxia prevents success in every new motor
activity attempted. Many children with dyspraxia are creative and highly verbal
and are safe when engaged in fantasy games that don’t require “doing”
something specific. Children with dyspraxia often prefer passive activity such as
watching television, playing on computers, or reading to the exclusion of any
activities that challenge motor planning abilities. The fear that emerges when
being required to participate in physical education activities at school or other
settings is real for a child. This may result in repeated feelings of failure, leading
to significant impairment of confidence and self-esteem.
Pattern 3: Sensory Discrimination
SDDs are reflected by difficulty with qualitative processing of sensory stimuli
(Miller, 2014). It is not uncommon to have a combination of strengths and
weaknesses in sensory discrimination, which leads to the idea that one is a
“visual” or “auditory” learner or learns best “by doing.” Commonly, descriptions
of sensory discrimination and assessments emphasize visual, auditory, and tactile
perception. Visual discrimination includes such skills as pattern recognition and
visual-spatial analysis. Pattern recognition problems include difficulty in the
recognition of differences in similar shapes such as ovals versus circles or in the
reversal of letters and numbers that persists after the age of six. Problems in
visual-spatial analysis are observed in attempts to solve simple puzzles and
replicate simple designs and can ultimately impact on the ability to adequately
develop handwriting skills. Auditory discrimination includes the ability to
differentiate similar sounds such as b and d and words such as with and which,
while tactile perception is often described as the ability to identify an object with
vision occluded, using the integration of tactile and visual discrimination to do
so, such as reaching into one’s pocket and retrieving a quarter, rather than a
penny, for the parking meter.
A unique feature of the SDD typology is the inclusion of the somatic senses,
which includes proprioceptive and vestibular discrimination and referred in
sensory processing language as somatosensation. Normal somatosensation, a
term to describe the combination of tactile, proprioceptive, and vestibulary
sensory processing, provides for a foundational body scheme, which Ayres
(1972) classically described as the “blueprint for movement,” enabling adequate
processing of feed-forward skills for planning movement and postural stability.
The awareness of where one’s body is in space in relation to the external
environment, and the excursion, speed, precision, and gradation of movement
needed to complete a motor task is a function of somatosensory discrimination.
Children who experience SOR as well as SUR may have difficulty with this
aspect of sensory discrimination, which can create difficulties in body scheme
and motor planning.
The course and prognosis of SPD are impacted by recognition by the child’s
healthcare providers that the behaviors observed are not willful misbehavior on
the part of the child. Oftentimes, children who demonstrate behaviors associated
with SPD are immediately determined to have a behavioral disorder or
something that is a result of ineffective parenting. Recognizing that SPD is a
neurological disorder is critical in meeting the needs of the child. At the very
least, providing a comprehensive effort to rule out SPD when behaviors suggest
this diagnosis is essential. The child with SPD is different than the typical child,
and the associated behaviors are not merely a matter of “noncompliance” or
“oppositional defiance.” Attempts to use a behavioral modification approach or a
“talk therapy” approach to change difficult behaviors or at worst using capital
punishment will very likely result in escalation of difficult behaviors.
Comorbidity studies have been published, which provide some
understanding of the course of SPD. Children diagnosed with ADHD experience
a diminished cortical response to sensory input as compared to typical children
(Reynolds, Lane, & Gennings, 2009). Children with a dual diagnosis of ADHD
and the SOR have been found to demonstrate anxiety more than do children with
only ADHD as indicated by higher scores on the Revised Children’s Manifest
Anxiety Scale (Reynolds & Lane, 2007). Children with ASD with higher
degrees of sensory overresponsivity were found to have less competence in daily
activities including school performance (Reynolds, Bendixen, Lawrence, &
Lane, 2011). Atypical sleep patterns were found to relate to atypical sensory
behaviors among children with ASD with predictive accuracy of 87.5% by way
of behavioral and physiologic measures (Reynolds, Lane, & Thacker, 2011).
A common question among caregivers of children diagnosed with SPD is
whether the challenges identified will be resolved as the child ages. The general
answer is that we learn to adapt or compensate for these challenges but the
challenges remain. A review of the literature by Abernathy (2010) suggests that
while adults with a history of SPD will develop compensatory strategies to
manage the specific challenges, quality of life can be compromised, yet there are
strategies that an occupational therapist with expertise in OT-SI can suggest to
bring positive results. Dunn’s work in the last two decades has been helpful in
providing an understanding of how SPD can be described and evaluated across
the lifespan. Her most recent work has resulted in the development of a set of
standardized tools to determine both caregiver-generated and self-report data to
provide a way in which occupational therapists can document and identify
specific sensory processing challenges (Dunn, 2014).
Diagnosis
It is important to note that three different diagnostic manuals include SPD or
very close descriptions of this disorder, as a legitimate diagnosis. These include
the Diagnostic Classification of Mental Health and Developmental Disorders of
Infancy and Early Childhood, Revised (DC: 0-3R) (Zero to Three, 2005), the
Diagnostic Manual for Infancy and Early Childhood of the Interdisciplinary
Council on Developmental and Learning Disorders (ICDL, 2005), and the
Psychodynamic Diagnostic Manual (PDM Task Force, 2006).
While the efforts to include SPD in the fifth edition of the Diagnostic and
Statistical Manual (DSM-5) was not successful, the application process resulted
in an exponential increase in the recognition of the term SPD as well as
facilitating the most significant degree of research on SPD in history (Miller,
2014). Funding by the Wallace Research Foundation has resulted in the
formation of the collaborative SPD Scientific Work Group, composed of
investigators with strong records of NIH-funded research at world-renowned
centers, which has led to major contributions to the knowledge base of SPD
including work toward the validation of the SPD diagnosis (Brett-Green, Miller,
Gavin, & Davies, 2008; Brett-Green, Miller, Schoen, & Nielsen, 2010; Davies &
Gavin, 2007; Schoen, Miller, Brett-Green, & Nielsen, 2009).
The most common tools used to diagnose SPD are behavioral measures
including clinical observations, caregiver-generated sensory profiles, and
standardized assessments to determine challenges as well as strengths in sensory
processing. A key to the diagnostic process is to clearly identify the impact of
sensory challenges on adaptive behavior. Clearly, everyone can identify an
idioscyncratic behavior that interferes with optimal efficiency in daily living, yet
compensatory strategies are typically deployed to lessen the impact on function.
This would not be considered an SPD. However, when that perceived challenge
overwhelms a person’s ability to engage and participate in everyday occupations,
an analysis of sensory processing is necessary to identify not only the source of
the challenge but also the support needed to potentially enable better adaptation.
Despite recognition by the American Academy of Pediatrics that
occupational therapy for SPD is a promising and effective intervention, there is a
lack of consensus among healthcare providers that a distinct diagnosis of SPD
exists (Zimmer et al., 2012). In an effort to produce evidence for a distinct,
stand-alone condition, several important studies have been published that
suggest this. A longitudinal study of all infants born in New Haven, Connecticut
were followed from birth to their eighth birthday. After excluding all cases of
developmental delays, autism, and genetic disorders, it was determined that 75%
of the remaining group of children presented with SPD signs and symptoms
only, suggesting a stand-alone condition (Carter, Ben-Sasson, & Briggs-Gowan,
2011). A study by Van Hulle, Schmidt, and Goldsmith (2012) completed a
phenotypic and behavior-genetic analysis on 970 seven-year-old children to
delineate comorbidity between childhood psychopathology and SOR. Diagnostic
surveys included ADHD, conduct disorder, oppositional defiant disorder,
agoraphobia, general anxiety, obsessive-compulsive disorder, panic disorder,
separation anxiety, social phobia, specific phobia, depression, enuresis,
trichotillomania, selective mutism, and pica behavior. Fifty-eight percent (58%)
of children who screened positive for SOR did not qualify for one of the
diagnosis included, and 68% of those who screened positive for
psychopathologic diagnoses did not screen positive for SOR, suggesting that
SOR exists independent of childhood psychiatric disorders.
Medical and Surgical Management
Early identification of SPD by a pediatrician, family doctor, or other health
provider is essential so that referral to a qualified occupational therapist can
occur. Historically, it has not been uncommon for a child who presented with
SPD signs and symptoms to be given a medical diagnosis due to the lack of any
diagnostic criteria for SPD. Interestingly enough, the policy paper published by
the American Academy of Pediatrics suggested that pediatricians not diagnose
children with SPD until further evidence of a distinct diagnosis was validated. In
the same statement, however, the authors indicated that intervention was
potentially effective, which initiated an increased frequency of diagnosis of SPD
by pediatricians (Miller, 2014). It is essential that occupational therapists educate
ephysicians and other health care providers about SPD and provide screening
checks that can be used to identify children who could benefit from an
assessment of sensory processing to rule out SPD. Children who demonstrate
signs and symptoms of SPD have often already been medically diagnosed with
ADHD, which may result in being prescribed stimulant medications such as
Adderall and Ritalin. These medications can reduce impulsiveness and
hyperactivity, leading to improved concentration, sustained time on task, and
more purposeful responses.
However, not all physicians necessarily select medication as the primary
regimen of treatment for children with SPD. In some cases, they may prescribe
an initial trial of OT-SI to determine if the child’s response lessens or even
negates the need for medication. It is not uncommon for children with SPD who
experience sleep difficulties to be prescribed melatonin, which is an over-the-
counter natural supplement that can enable the child to acquire an improved state
of sleep. It has been suggested that children with SPD may not naturally generate
and regulate adequate levels of melatonin due to an inefficiency of serotonin, a
neurotransmitter that regulates the natural release of melatonin (M. Sloane,
personal communication, February 15, 2016).
The end result of typical sensory processing is effective participation and
engagement in occupation. As noted earlier, unless analysis of sensory
processing impacts on the child’s functional abilities in occupational
performance, further intervention is not felt to be necessary. However, for most
children, the presence of one type of SPD can indeed impact on occupational
performance. Each area of occupation is significantly impacted when the ability
to process sensory information and produce an adaptive, meaningful, and
efficient response is compromised.
Play
Play is the main occupation of children and requires skills that are grounded in
all sensory modalities. Knox, an occupational therapist, developed a
comprehensive analysis of play for ages 0 to 72 months and is useful in creating
a play profile based on four dimensions including management of space,
management of materials, symbolic and pretense, and social participation in
play. Each dimension, when analyzed from a sensory processing framework,
clearly requires modulation, sensory-based motor functions, and sensory
discrimination in order for purposeful play to develop. By way of example, the
following describes some of the specific tasks within the first three dimensions
for a child 36 to 48 months that are all dependent upon sensory processing to
acquire.
Space Management: Gross Motor and Interest

(Intentionality)
Gross motor includes smooth walking, jumping, climbing, running with
graded acceleration and deceleration of movement, hopping on one foot,
and catching and throwing a ball using shoulder movements. Effective
modulation of the visual, vestibular, and proprioceptive sensory
mechanisms is foundational to anticipation, initiation, and maintenance of
gross motor activities. The more success a child feels, the higher the degree
of interest in engaging and participating in gross motor play, which of
course leads to development of new and more challenging gross motor
activities. Transitional, quality movement is dependent upon the child’s
ocular-postural function that is dependent on smooth modulation of
vestibular-visual dyad. The need for acceleration and deceleration, holding
and placing of the extremities in isolated positions, along with smooth
execution of gross motor movement is the direct result of tactile-kinesthetic
integration superimposed on vestibular-ocular mechanisms.
Strong interest in novelty and drawn fine motor activities. With developing
intersensory skills, the child is clearly drawn to try out new tasks as he or
she receives feedback from the outcomes of earlier engagement and
participation.
Material Management: Manipulation, Construction,

Purpose, and Attention
Small muscle activity—hammers, sorts, and inserts small objects
Makes simple objects, takes apart objects, and combines play materials
Shows interest in a completed project
Plays with single object for about 10 minutes
Interest in a variety of fine motor activities is further facilitated by the

emergence of precision hand prehension and manipulation patterns integrated
with higher-level visual discrimination tasks that allow for improved pattern
recognition and spatial relation skills
Pretense-Symbolic; Imitation and Dramatization

Increased mimicry in play, for example, domestic play; past experiences are
dramatized
Pretending with toys; develops story sequence with toys
Creates and enacts multiple characters with feelings that primarily include
anger and crying
Pretense requires motor planning skills to express ideation of observations and

past experiences. It’s not uncommon for parents to enjoy observing their toddlers
trying to “help” when they are engaged in household tasks through imitation of
their actions. Sequential storytelling, typically by way of doll play, demonstrates
motor planning abilities as the child sequences actions and verbal exchanges
between the objects that become personified.
Difficulty with these developmental tasks can be often be tied to challenges
in sensory modulation including hyperresponsive and hyporesponsive, dyspraxia
and postural integrity, and difficulty with higher-order visual-perceptual and
cognitive skills that are grounded in sensory discrimination.
Basic Activities of Daily Living
Basic activities of daily living (BADLs) are primarily habitual and predictive.
Thus, these tasks are typically less challenging for someone with intact sensory
processing. However, for a person with SPD, the multisensory components of
bathing/showering, dressing, eating, feeding, personal hygiene and grooming,
and toileting can be significantly challenging. For example, SOR to visual,
tactile, proprioceptive, olfactory, auditory, and gustatory sensations can create
severe food selectivity issues when the ability to detect, register, and modulate
incoming stimuli is compromised. Difficulty with dressing for children and
adults can be related to tactile sensitivity to texture, tags in shirt collars, and
wearing hose, ties, and clothing that is too lightweight but also includes
problems with motor planning as can often be observed with children who
cannot sequence and organize their bodies in space to put on clothing, use
fasteners, or tie shoes independently. Bathing can be significantly challenging
for a child with SOR who becomes increasingly distressed at the sound of water
rushing from the faucet as well as discomfort from being washed with a face
cloth and having hair shampoo applied, all of which can be perceived as
threatening and cause significantly negative reactions in a child with SOR.
Instrumental Activities of Daily Living
Instrumental activities of daily living (IADLs) require more complex cognitive
level skills that emerge primarily from sensory-based motor and sensory
discrimination abilities. Persons with dyspraxia may experience difficulty with
planning and organizing steps to accomplish basic life skills due to poor sensory
discrimination across all sensory mechanisms. Going shopping, which can result
in being in a crowded facility, having to use an escalator or elevator, and
standing in line to wait for checking out, can be distressing for someone with
SOR. Dining out can be difficult due to the sounds, smells, and crowded
sensation to which a person with SOR is particularly sensitive or can be very
challenging for parents of a child with SS who can’t regulate in the midst of
opportunities to explore by running around the restaurant, going up to guest’s
tables, throwing items from the table, and needing to be in constant motion while
at a table. Riding in an automobile may result in complaints of feeling nauseated,
especially when riding in the back seat. The crowded, rapid movement of a
crowded airport can be intolerable for those with SOR as the sensory chaos that
ensues from entrance to takeoff overwhelms the sensory responsivity of a person
with SPD.
Rest and Sleep
The lack of a balance of rest and other areas of occupation can lead to sleep
problems in all people. The cycle of the “4 As (awake, alert, attending, and
asleep)” is compromised when factors in the environment interfere with this
cycle. The need for a consistent “diet” of sensory enrichment is key to healthy
rest and sleep as it helps the nervous system maintain a modulated state. The
child with SOR to movement, along with proprioceptive and tactile stimuli, will
more likely prefer to engage in much more passive visual activity as opposed to
outdoor play. Alternatively, the child who tends to crave sensory input, resulting
in overstimulation—especially in the evening hours—may have difficulty with
reduction of an arousal state, creating sleep disturbance. External factors, such as
direct and ambient auditory, visual, and even olfactory sensation, can impact
significantly on sleep patterns as well.
Education
Supporting the educational needs of children with various disabilities has
historically been a major focus of occupational therapists who work with school-
age children and adolescents. Ayres’ early work was directed toward children
with learning disabilities (Ayres, 1972). Five decades after initiating her
pioneering work focused on this population, occupational therapists continue to
rule out SPD as a factor in readiness for specific academic skills among a variety
of educational diagnoses. All three typologies have implications for educational
competencies. Common problems include a child’s inability to attend to
classroom demands and difficulty being in one position without transition. There
are varied demands that a child needs to be able to consistently show
competence in order to succeed in school. These include performing self-care
tasks without assistance, maintaining care of personal belongings, moving
through the day without significant fatigue, organizing and sequencing oral and
written directions, ability to correctly interpret social and environmental cues
from teachers and peers, and staying “on task” without being consistently
distracted. SPD can easily compromise these and other educational-related tasks
that can be addressed with working on “just right” challenges that are at the level
of the child’s developmental level.
Work
The antecedent behaviors and aptitudes necessary for the occupational
performance of work can be significantly impacted by SPD. Due to modulation
difficulty, fatigue from poor postural stability, motor planning problems, and
higher-level discrimination difficulties, even mainstream jobs can be a challenge
for a person with SPD.
May-Benson (2009) described the impact of SPD on adults, noting that
functional limitations secondary to SPD impacts on an adult’s occupational
performance. Studies by Pfieffer (2002) and Moore and Henry (2002) reported
on the negative influence of poor sensory processing and motor coordination
problems on occupational choices, including the ability to obtain employment
and engage in leisure activities (as cited by May-Benson, 2009). May-Benson
and Patane (2010) found more than half of their study participants chose to be
homemakers specifically because of their sensory processing difficulties, even
though all had advanced educational degrees. In their study, it was noted that
respondents indicated that sensory processing problems impacted on intimate
relationships, leading in one case to problems having children. Lastly, driving a
car was commonly reported as being severely restricted by sensory processing
problems (May-Benson, 2009) and poor motor performance (Cousins and
Smyth, 2003, as cited in May-Benson, 2011). By way of interview data, Heufner,
Cohn, and Koomar (2010) reported on the negative impact of mothers’ sensory
processing problems on parenting their children with SPD during mealtimes,
waking and bedtime routines, and participation in child-selected leisure
activities, creating disruption that impacted the entire family.
Social Participation
Persons with modulation difficulty, whether it be sensory overresponsiveness,
sensory underresponsiveness, or sensory craving, will experience significant
challenges in developing and maintaining socially acceptable behaviors.
Examples of challenging behaviors include aggressive reactions to sensory
stimuli, particularly tactile sensation, and avoiding interaction due to fear and
discomfort with particular types of environmental stimuli, that is, sounds, smells,
lights, and colors derail. Embedded in play development is the ability to develop
and sustain interpersonal relationships that are apparent very early on with the
first episode of infant regard for a caregiver by way of eye contact, “molding,”
or positive physical response to being gentling by a caregiver through later
stages of group interaction skills that develop in early childhood. While
reporting on a small sample (N = 12), a study by Cosbey, Johnston, and Dunn
(2010) suggests that boys with SPD demonstrate some variation in social
participation that differs from those of peers without a diagnosis of SPD. By way
of a case study report of an adult male with sensory defensiveness and dyspraxia,
ADLs and work performance were compromised by organizational and
sequential abilities along with challenges experienced in dating and social
interactions. These difficulties were found to impact on positive self-esteem as
well.
CASE-STUDY 1
Hector and Anna were very pleased when their first child, Jose, was born.
Even though Hector expressed his neutrality about having a boy or a girl, he
was elated to have a son. For the first year, Hector and Anna understood
now what their friends with children had told them about having to expect
being “sleep deprived” as they continued to have difficulty with
establishing a regulated state after he was fed, held for a while, and
beginning to fall asleep. It was unexpected that Jose would resist a pacifier
when he would be returned to his crib, which they had been told was often
necessary and helpful to help calm a baby, especially after having been fed
and diapered. He cried for long periods of time when put down in his crib.
Attempts to provide a pacifier was met with complete resistance as he
would eject it from his mouth and cry with increasing intensity. They would
pick him up and rock him, which would eventually result in his falling
asleep. However, soon after they returned him to his crib, he would cry with
increasing intensity until he was held again. While calming quickly,
attempts to quietly, slowly, and carefully lay him down again after falling
asleep again were frequently unsuccessful. Attempts to console Jose with a
pacifier was unsuccessful. He simply would not retain it. The fact that Jose
simply could not tolerate or accept the pacifier was troubling. Exhausted,
Hector and Ana were at a loss as to why Jose could not be consoled with a
pacifier. All their friends with infant children were often distraught when a
pacifier went missing as they could not calm their baby without it. During a
well-infant appointment with their nurse practitioner, she ruled out
problems with his oral intake as his weight gain was within the normal
curve. Recently, she had worked with another case similar to Jose in which
an occupational therapist has provided suggestions for calming. Jose had
always been able to latch when breast-feeding, and while it took longer than
expected to finish the process as Jose took “breaks” throughout the feeding
process, he did acquire enough intake to allow for proper nutritional needs.
He was beginning to take in cereal, but this was very time consuming even
though the reflexive tongue protrusion was diminished. Ruling out a
physical problem, the PNP provided them with a referral for an evaluation
to obtain a sensory profile of Jose and determine challenges in specific
sensory modulation that would indicate both internal and external factors
that were possibly creating Jose’s dysregulation. In addition, she advised
Jose’s parents that the occupational therapist would provide strategies to
support his challenges with oral motor regulation.
CASE-STUDY 2
Robert has just started his first semester at his local community college and
is feeling increasingly anxious about whether or not he will make it in
college. Throughout his educational experiences from K to 12, Robert had
always experienced difficulty with being in large groups, and sitting and
listening to his teacher’s presentations that went beyond 20 minutes. He
would often become restless and fidgety, sometimes feeling like his body, in
his words, “was about to explode.” At times, he noticed that his discomfort
was heightened if there were smells coming out of the school cafeteria, if
his teachers or other students had perfume or cologne, or if there were
ambient noises in the room or outside the classroom that others didn’t seem
to notice or in which they were not bothered. He dreaded classes in which
the instructors used PowerPoint presentations—especially those that were
mostly text based with minimal graphics to illustrate points presented. He
would easily get distracted and miss important points that were likely to be
on exams. Furthermore, if the teacher had a high, grating voice—like the
professor of his English Literature course—he literally would get up, grab
his things, and leave the room. She had already asked him to come to her
office when this happened for the third time and advised him that his grade
would suffer significantly if he continued to leave her class. Robert knew
that he was at risk for failing if he did not figure out how to cope and adapt
to his challenges. He made an appointment with his advisor who readily
recognized that Robert could benefit from consultation with an
occupational therapist who was working with high school and college
students who had sensory processing disorders. He provided Robert with
general information about SPDs and suggested that it would be helpful to
have an assessment. He provided Robert with a survey to fill out in advance
of his visit with the OT: the Adolescent Adult Sensory Profile, which would
begin to identify the sensory modulation typology that interfered with
Robert’s ability to succeed in his classroom activities as well as his
participation in classroom group activities. Most importantly, as he
suggested to Robert, the assessment could lead to some supportive
strategies that might alleviate the challenges he was experiencing.
RECOMMENDED BOOKS
Miller, L. J. (2011). No longer a secret: Unique common sense strategies for
with sensory or motor challenges. Arlington, TX: Sensory World.
Sensory Integration and Learning Disorders, by A. Jean Ayres Published in
1972. This is the classic textbook of Dr. Ayres in which she presents the
neuroscience of sensory integration, evaluation, and intervention approaches for
what is now termed sensory processing disorder. Western Psychological
Disorders.
Kranowitz, C. (2006). The out-of-sync child: Recognizing and coping with
sensory processing disorder. New York: Perigee.
Kranowitz, C. (2006). The out of sync child has fun: Activities for kids with
sensory processing disorder. New York: Perigee.
Miller, L. J. (2006). Sensational kids: Hope and help for children with sensory
processing disorders. New York: Perigee.
Sensoryplanet.com. A social network community for persons with SPD.
SPDFoundation.net. The “gold standard” for resource information on sensory
processing including the theory of sensory integration, the diagnosis of sensory
processing disorder, and the treatment of SPD known as occupational therapy
using a sensory integration approach (OT-SI), research archives, bibliographies,
and all other resources for the professional community.
SPDFoundation.net/families.html. Resource site that provides information for
parents of children with SPD as well as for anyone wanting to know more about
sensory processing disorder and treatment.
SPDUniversity.org. A comprehensive site for online courses, webinars, and links
to educational resource for professionals.
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0876
UNIT
2
Mental Conditions
The Mental Conditions Unit includes mental conditions as specified by the
Diagnostic and Statistical Manual of Mental Disorders, 5th Edition. These
chapters focus on conditions that are diagnosed in both adults and pediatrics.
People with these conditions may be referred to occupational therapy for the
specified mental condition or have the mental condition as a secondary
diagnosis. Each chapter provides information about the etiology, incidence and
prevalence, signs and symptoms, course and prognosis, diagnosis,
medical/surgical management, and impact on occupational performance of these
conditions. Case illustrations are used to provide examples of lives affected by
the conditions. The conditions included in this unit are the following:
Chapter 8: Mood Disorders
Chapter 9: Schizophrenia and other Psychotic Disorders
Chapter 10: Anxiety Disorders
Chapter 11: Neurocognitive Disorders
Chapter 12: Complex Trauma
Chapter 13: Obsessive-Compulsive and Related Disorders
Chapter 14: Somatic Symptoms and Related Disorders
Chapter 15: Feeding and Eating Disorders
Chapter 16: Substance-Related and Addictive Disorders
C H A P T E R
8 Mood Disorders
Ann Chapleau Jennifer Harrison
KEY TERMS
Affect
Anhedonia
Avolition
Cyclothymia
Depression
Dysphoria
Euphoria
Flight of ideas Grandiosity
Hypomanic episode Major depressive episode Mania
Manic episode
Mixed episode
Prodromal
Psychomotor agitation Psychomotor retardation Psychosis
Unipolar depression
Helen sits in her rocking chair in her small living room, smoking a cigarette. The
room is dimly lit by the television screen. Her silver hair is disheveled, and her
sweat suit is stained and dirty. She has several days’ worth of Meals on Wheels
containers on the coffee table, mostly uneaten. A picture of her deceased
husband is on the end table, as are various photos of her grown daughter, who
lives in another state. Helen has not left her home in over 6 months, except for
doctor visits. She worked as a tool and die operator for 14 years after her
daughter went to kindergarten, but quit after she was hospitalized for depression
for the second time when her daughter left for college. She thinks of these two
times in the hospital as her “change of life” difficulties, but realizes that her
symptoms have continued off and on for the last two decades. She has early-
stage emphysema, but has not been able to stop smoking. She no longer knits or
bakes, two of her favorite hobbies.Ten years ago, she tried a newer
antidepressant at the behest of her daughter. It seemed to help, but her mouth
was too dry and she had difficulty feeling alert in the morning when she woke
up. That, paired with the high co-pay for the medication, led to her decision to
stop taking it after 4 months. At her last doctor’s visit, her physician asked her if
she was feeling depressed again, but Helen was too ashamed to tell him how sad
and lonely she had been feeling for months. She wanted to tell him that there
wasn’t any point in living anymore and that her life doesn’t have any meaning,
but she remained silent. At home, she is overwhelmed by feelings of guilt. She
had been raised to keep a tidy home and be active, and not to feel sorry for
oneself, but she feels too tired to do anything except watch television.Mood
disorders are among the most disabling and prevalent illnesses worldwide. The
World Health Organization (WHO) estimates that major depressive disorder
(MDD) and bipolar disorder (BPD) are among the leading causes of disability,
and depression is the leading cause of disability worldwide in terms of years lost
to disability (WHO, 2012a). Depression often strikes people younger than do
other chronic illnesses and impacts an estimated 350 million worldwide (WHO,
2012b). Despite the prevalence and impact on global health care costs, as well as
quality of life for individuals and their families, mood disorders often go
undetected and untreated.
Mood disorders represent a spectrum of mood disturbances, from the extremely
low mood of depression to the extremely elevated mood of mania. The
American Psychiatric Association (APA) provides a classification system known
as the Diagnostic and Statistical Manual of Mental Disorders, now in its fifth
edition (DSM-5; APA, 2013), to identify symptoms using universal terminology.
Depressive disorders include MDD and dysthymic disorder, which are often
referred to as unipolar depression, as they do not include mood variances on
the other end of the mood spectrum. MDD, often referred to as major depression,
is characterized by symptoms such as sadness, hopelessness, guilt, irritability,
and cognitive impairments, like poor concentration and difficulty making
decisions. A diagnosis of MDD requires the presence of a major depressive
episode, which is a period of depressed or irritable mood, with additional
symptoms lasting at least 2 weeks, resulting in severe impairments in
functioning.
The BPD spectrum includes BPD I, BPD II, and cyclothymia, which all
involve some degree of elevated mood and usually a history of at least one major
depressive episode. In addition, the DSM-5 includes substance-induced BPD and
BPD related to a medical condition (APA, 2013). BPD I is characterized by
mood swings from extremely high (mania) to extremely low (depression). Manic
behaviors include euphoria, irritability, grandiosity, decreased sleep,
impulsivity, and distractibility, which significantly interfere with daily
functioning. A diagnosis of BPD I is warranted when an individual presents with
either a manic or mixed episode. A manic episode is a highly elevated or
irritable mood lasting at least 1 week, with or without psychotic symptoms such
as delusions and hallucinations. Severity of symptoms results in significant
impairment in functioning, which may require hospitalization. A mixed episode
is characterized by the presence of both manic and major depressive symptoms
almost daily for at least 1 week, resulting in rapid mood cycling with or without
psychotic symptoms.
BPD II can be characterized by the presence or history of an MDD and at
least one hypomanic episode. Hypomania involves similar but less intense
mood and energy elevation than does mania.
Cyclothymia represents a chronic (at least 2 years), but less severe, mood
disturbance involving both hypomanic behaviors and depressive symptoms that
do not meet the criteria for either a manic or major depressive episode (Fig. 8.1).
Figure 8.1 Mood spectrum.
Terms to describe the clinical symptoms of mood disorders include
Affect: the display of emotion, particularly facial expression

Anhedonia: lack of interest in previously pleasurable activities
Avolition: lack of drive or ambition to complete goal-directed tasks or
activities
Dysphoria: a depressed or negative mood state
Euphoria: highly elevated, exaggerated mood
Flight of ideas: rapidly changing, disconnected thoughts
Grandiosity: inflated sense of self-esteem or importance
Hypomania: elevated mood that is less intense than full mania
Psychomotor agitation: increased physical movements that are purposeless
and reflective of an agitated or anxious state (e.g., wringing hands,
fidgeting, pacing)
Psychomotor retardation: abnormally slowed or reduced movements or
speech
Psychosis: the presence of delusions or hallucinations without insight
Etiology
Despite ongoing research, there is still no one known cause of mood disorders.
Both MDD and BDP are widely believed to result from a complex
combination of biological, genetic, and psychosocial factors. Many factors can
influence the development of mood disorders, including an individual’s genetic
makeup, biology, other co-occurring medical and psychiatric conditions,
cognitive abilities, personality, support systems, personal history, exposure to
stress, and coping strategies. Additionally, mood disorders can be triggered by
childbirth, seasonal changes, substance use, and other medical conditions.
Biological Factors
Much of the research over the past 50 years has focused on biological changes in
brain function (National Institute of Mental Health, 2014; U.S. Department of
Health and Human Services, 1999). Current research using advanced
technologies of brain imaging to examine structures and brain activity provides
evidence that mood disorders are disorders of the brain. Functional magnetic
resonance imaging (fMRI), positron emission tomography (PET), photon
emission computer tomography (PECT), and magnetic resonance spectroscopy
(MRS) studies reveal abnormal functioning in regions of the brain that regulate
mood, sleep, thinking, appetite, and behavior (Franklin, Carson, & Welch, 2015).
In studies of MDD, functional abnormalities have been found in limbic
system, the brain region that represents centers of emotion. These parts of the
limbic system include the amygdala, hippocampus, insula, regions of the anterior
cingulate cortex, and the dorsolateral prefrontal cortex (Cole et al., 2010; Gos et
al., 2010; McKinnon et al., 2009). Functional abnormalities, including reduced
cortical thickness of the frontal and temporal lobes and within the limbic system,
have also been found in studies of BPD (Kruger, Seminowicz, Goldapple,
Kennedy, & Mayberg, 2003; Oertel-Knöchel et al., 2015). In a systematic review
of controlled studies with BPD, changes in the hippocampus were also found
(Otten & Meeter, 2015). These abnormalities are consistent with studies in
which both MDD and BPD subjects demonstrated blunted or decreased
behavioral and physiological reactivity to sad or negative stimuli, such as
pictures and videos (Foti, Olvet, Klein, & Hajcak, 2010; Miklowski & Johnson,
2006).
The role of neurotransmitters, such as serotonin, acetylcholine, and
melatonin, in mood disorders has been studied for several decades.
Neuroimaging and genetic studies have focused on the role of genes that may
predispose an individual to dysregulated levels of neurotransmitters. Current
research focuses on neurotransmitter systems, such as the effects of dysregulated
dopamine, noradrenaline, and serotonin transporters and receptors in the brain
and research related to the improvement of primary and augmented medication
therapies for MDD and BPD (O’Leary, Dinan, & Cryan, 2015; Owenby, Brown,
& Brown, 2011).
Biological abnormalities of the brain, however, may not necessarily be the
cause of a mood disorder, but may develop as a result of the disorder. For
example, some researchers believe that chronic stress can trigger depression, as
the body continually responds to stress through neurochemical changes
(Hennessy, Schiml-Webb, & Deak, 2009; Kumar et al., 2015). One such stress
response is the excessive secretion of cortisol, which can interfere with the
limbic-cortical systems. The challenge for researchers is to differentiate which
abnormalities are causal factors and which develop in response to environmental
factors that trigger neurochemical changes.
Both MDD and BPD are known to run in families as heritable traits, but
researchers have had minimal success in isolating any specific responsible
genes. Genetic variations associated with both MDD and BPD support the
growing belief among researchers that mood disorders involve a combination of
altered genes that interact with environmental factors, such as stress, to explain
why some family members with a known genetic risk develop either MDD or
BPD while other family members do not (McMahon et al., 2010). The research
on biomarkers for MDD and BPD also shows promise, which could lead to
improved diagnosis in primary care and other first-line providers based on
laboratory testing and not simply clinical presentation or family history (Redei &
Mehta, 2015).
Twin studies also support the genetic link in mood disorders (Gillespie et al.,
2015). In studies of MDD, heritability was found to contribute 30% to 40% of
the risk for depression, with as much as 60% in adolescent depression (Dolan, de
Kort, van Beijsterveldlt, & Boomsma, 2014; Kendler, Gatz, Gardner, &
Pedersen, 2006; Kendler et al., 2011; Nivard et al., 2015). In studies of BPD,
heritability is even higher. Identical twins have a concordance rate of 57%, while
the concordance rate for fraternal twins is 14% (Miklowitz & Johnson, 2009).
Psychosocial Risk Factors
In addition to the role of biological and genetic factors in the development of
mood disorders, the role of stressful life events has been widely studied. For
example, traumatic events, such as childhood sexual, physical, or emotional
abuse, increase the risk of both MDD and BPD (Maniglio, 2010; Miklowitz &
Johnson, 2009). Other risk factors include exposure to war, disaster,
displacement, parental mental illness, domestic violence, physical or sexual
assault, involvement in a serious accident, death of a loved one, chronic work
stress, and caregiver stress (Maniglio, 2010; Wingo et al., 2010). This
relationship appears to be related to stress and inflammatory processes in the
brain, which are associated with depression (Bufalino, Hepgul, Aguglia, &
Pariante, 2013; Denson, Spanovic, & Miller, 2009; Irwin & Cole, 2011).
However, it is important to remember that the majority of people who experience
life stressors do not develop a mood disorder and that not all mood disorders are
triggered by a traumatic event. For those who experience traumatic life events, it
is estimated that 20% will develop MDD and that estimate is higher for those
who experience more profound trauma (Monroe & Reid, 2009).
MDD is both common and widespread, with a 12-month prevalence rate of 7%,
a lifetime prevalence of 20%, and a relapse rate of more than 50% (APA, 2013;
Gotlib & Hamilton, 2008). The 12-month prevalence for BPD is much lower.
Community samples in the United States can range from 0.4% to 0.6% (APA,
2013), or from 3.9% to 10%, based on inclusion criteria of the full bipolar
spectrum (Miklowitz & Johnson, 2009).
In any given year, 20.9 million (9.5%) adults in the United States will
experience a mood disorder, with 14.8 million suffering from MDD and 5.7
million suffering from BPD (Kessler, Chiu, Demler, & Walters, 2005; Kessler et
al., 2010). Women are more frequently diagnosed with MDD than are men
(Gavin et al., 2010; Kessler et al., 2010). Additionally, lower socioeconomic
status is associated with both MDD and BPD (Dijkstra-Kersten, Biesheuvel-
Leliefeld, van der Wouden, Penninx, & van Marwijk, 2015; Schoeyen et al.,
2011). Low socioeconomic status is also related to lower rates of treatment and
higher rates of medication nonadherence (Alekhya et al., 2015).
Signs and Symptoms
Both MDD and BPD are marked by a complex set of symptoms that interfere
with daily functioning. Moreover, both disorders are associated with high rates
of suicide, the 14th leading cause of death worldwide (Clements et al., 2013;
O'Connor & Nock, 2014), making a proper diagnosis essential for treatment
purposes.
Major Depressive Disorder
According to the APA (APA, 2013), the following are the typical symptoms of
MDD:
Depressed mood
Anhedonia
Weight loss
Altered sleep
Change in psychomotor behavior
Fatigue/loss of energy
Feelings of worthlessness or guilt
Impaired cognition
Thoughts of death or suicide
Children with depressive disorders may not exhibit the same set of symptoms as
do adults. Symptoms may include irritability, negativity, acting clingy or overly
needy, behavioral problems in school, refusing to attend school, and fear of a
parent’s death (U.S. Department of Health and Human Services, 2014).
Bipolar Disorder
According to the APA (2013), the following are the typical symptoms of BPD:
Grandiosity
Minimal need for sleep
Excessively talkative or having pressured speech
Racing thoughts or flight of ideas
Distractibility
Excessive goal-directed activity or psychomotor agitation
Impulsivity or participation in dangerous or risky activities
The course of illness and prognosis of both MDD and BPD is varied and is
complicated by co-occurring conditions such as anxiety, cardiovascular and
pulmonary disease, diabetes, and obesity, which can be related to lifestyle,
limited access to care, and the effects of long-term psychotropic medication use
(Carnethon et al., 2007; Colton & Manderscheid, 2006). In addition, individuals
with mood disorders are more likely to abuse alcohol and/or drugs, which can
negatively impact prognosis (Boschloo et al., 2010; Conway, Compton, Stinson,
& Grant, 2006).
Average age of onset for MDD is in the mid- to late-20s (Beesdo et al., 2009).
Approximately one-third of those diagnosed with MDD will develop a chronic
course of the illness, often lasting throughout their lifetime (Institute of
Medicine, 2009). Although MDD is commonly regarded as an adult illness, there
is growing evidence that depressive symptoms meeting diagnostic criteria can be
present in adolescence, childhood, and even early childhood/preschool years
(Hammen, 2009; Luby, 2010). Childhood depression carries a higher risk for
recurrence (30% to 50%) during adolescent years and early adulthood (Costello
et al., 2002; Thapar, Collishaw, Pine, & Thapar, 2012) and can also be a risk
factor for more severe depression in adulthood, as well as among the children of
depressed adults (Mars et al., 2015; Weissman et al., 1999).
During adolescence, rates for depression are equally common among boys
and girls, but after age 13, the rate doubles or triples for girls. This higher rate
continues into adulthood for women until after middle age (Costello et al., 2002;
Mars et al., 2015; Thapar et al., 2012). Adolescents who experience an initial
depressive or manic episode are at a higher risk of conversion to BPD (Beesdo et
al., 2009).
MDD among older adults is common, representing approximately 10% of
older adults (Lyness, Caine, King, Cox, & Yoediono, 1999). While the
prevalence of diagnosed depression appears to decline with age, risk of
depression and suicide increases when co-occurring physical illness or pain
affects ability to function (National Institute of Mental Health, 2007). About one
in three older adults with MDD develops a chronic course of the illness (Licht-
Strunk et al., 2007; Penkunas & Hahn-Smith, 2015).
Bipolar Disorder
Age of onset for BPD is earlier than in MDD, with an average age of 17.5 years
(Beesdo et al., 2009). BPD can occur in childhood and early adolescence, as
well. A prodromal period, ranging from 1.8 to 7.3 years, before full onset of
BPD, has been noted in a number of studies (Skjelstad, Malt, & Holte, 2010).
Symptoms of dysregulated mood and fluctuations in energy increase in intensity
during this period.
Longitudinal studies of BPD indicate that a full, functional recovery from a
first episode is uncommon. There is a high risk of relapse, recurring episodes,
and mood cycling during the first 2 years of the illness. In addition, suicidal
behavior is higher during this early phase (Clements et al., 2015; Nordentoft,
Mortensen, & Pedersen, 2011; Salvatore et al., 2007). Sixty percent of people
with BPD will experience a recurrence within the first 2 years, while as many as
75% will experience recurrences within the first 5 years. In addition, many will
suffer residual symptoms, such as depression, between these episodes
(Miklowitz, 2007).
Those who experience an initial depressive or mixed episode are at greater
risk for more severe depression and morbidity later in the illness than are those
who initially experience a manic episode. In general, the course of the BPD is
highly variable, but early treatment is associated with a better prognosis
(Clements et al., 2015).
Rates of suicide among people with BPD are higher than in any other
psychiatric condition: four times higher than among those with MDD and 15
times higher than among the general population. As many as 60% of people with
BPD attempt suicide at least once during the course of the illness, and 5% die as
a result of suicide (Clements et al., 2015; Miklowski & Johnson, 2006).
Diagnosis
A diagnosis of MDD can be determined when an individual has experienced at
least one major depressive episode, in the absence of any manic, mixed, or
hypomanic episodes (APA, 2013). Symptoms must represent a significant
change in typical functioning that interferes with daily living. According to the
APA (2013), a depressive episode must include at least five of the symptoms
listed previously during a 2-week period, and at least one of the symptoms
observed must be either depressed mood or loss of interest or pleasure.
Bipolar Disorder
The BPDs can be differentiated by the experience of either a depressive or manic
episode. A diagnosis of BPD I is warranted when an individual has experienced
at least one manic or mixed episode, with manic mood as the dominant
presentation. The individual may have had at least one major depressive episode,
as well. A classification of BPD II is determined when the individual has had at
least one depressive episode and at least one hypomanic episode, but no manic
or mixed episode. BPD II features a predominantly depressed mood. As in
MDD, symptoms of BPD must represent a change from previous level of
functioning. The disturbed mood must last a minimum of 1 week and include at
least three of the symptoms listed previously (APA, 2013).
Pharmacology for Major Depressive Disorders
Antidepressant medications work to regulate neurotransmitters, particularly
serotonin and norepinephrine. The newest and most widely prescribed category
is the “second-generation” antidepressants called selective serotonin reuptake
inhibitors (SSRIs) and serotonin and norepinephrine reuptake inhibitors
(SNRIs). SSRIs and SNRIs are the current medications of choice due to lesser
side effects than seen with “first-generation” tricyclics and monoamine oxidase
inhibitors (MAOIs).
Unfortunately, side effects of all antidepressants can range from minor to life
threatening, depending on the individual, and can result in medication
noncompliance. Side effects can include nausea, headache, agitation, sexual
dysfunction or loss of drive, dry mouth, constipation, blurred vision, and
sedation.
MAOIs require strict adherence to diet and medication restrictions, as certain
foods and medicines can interact with the MAOIs, resulting in increased risk of
stroke.
Pharmacology for Bipolar Disorder
Medications for mood stabilization can vary, based on the need to treat acute
symptoms versus maintenance treatment. Monitoring of medication use is
important to ensure that the mood stabilizer does not cause an episode at the
opposite end of the spectrum. Lithium was the first approved medication for
BPD and has been the most commonly used medication for mood stabilization
for decades. Extensive research still supports its efficacy (Miklowski & Johnson,
2006).
Other mood stabilizers found to be effective include valproic acid or
divalproex sodium, known as Depakote, and anticonvulsant medications, such as
lamotrigine (Lamictal), gabapentin (Neurontin), topiramate (Topamax), and
oxcarbazepine (Trileptal). Potential side effects include sedation; weight gain;
tremors; dry mouth; excessive thirst, or polydipsia; restlessness; acne; gastric
irritation; and kidney problems.
A treatment regime for acute mania may consist of a combination of lithium,
divalproex, or carbamazepine and an atypical antipsychotic medication
(Spielmans et al., 2013). Atypical antipsychotic medication side effects can
include extensive weight gain, sedation, dizziness, blurred vision, skin rash, and
sun sensitivity (Table 8.1).
TABLE 8.1 Common Medications and Side Effects

Electroconvulsive Therapy
Convulsive, or shock, therapy has been used in psychiatry for decades. Early use
was often associated with tragic results, including permanent severe blunting of
affect, permanent memory loss, and even death. Electroconvulsive therapy
(ECT) has evolved over time, as medical advances have increased our
knowledge of the intricacies of the brain. Currently, ECT is considered a safe,
effective treatment for MDD and BPD, but is primarily used with those who are
treatment resistant to pharmacology (Ottosson & Odeberg, 2012; Taylor, 2007;
Travino, McClintock, & Hussain, 2010). The mortality rate for ECT is about 2
deaths per 100,000 treatments and is associated with complications from
anesthesia (Fink & Taylor, 2007).
The ECT procedure involves the use of anesthesia prior to induction of a
controlled seizure. The seizure is evoked by administration of an electrical shock
using electrodes attached to the scalp. ECT is usually administered in a series,
over the course of several days or weeks, depending on the individual and
severity of symptoms. Although effective, it is still not clear how ECT works.
Researchers believe the antidepressive effects may result from an increase in
monoamines and serotonin levels, increases in neurotrophic factors that can
include cellular improvements and norepinephrine and serotonin receptor
expression, and/or increases in anticonvulsant action, which can increase opioids
(Ottosson & Odeberg, 2012; Trevino et al., 2010).
Repetitive Transcranial Magnetic Stimulation
Repetitive transcranial magnetic stimulation (rTMS) is a newer intervention for
treatment-resistant depression (Gross et al., 2007). rTMS involves a noninvasive
procedure of creating a magnetic field that passes through the skull inducing
electrical currents in the brain that activate specific nerve cells. The procedure
can be repeated daily over a period of days or weeks. It has proven successful in
reducing depressive symptoms and improving remission rates (George et al.,
2010; Holtzheimer et al., 2010; McGirr, Van den Eynde, Tovar-Perdoma, Fleck,
& Berlim, 2015). Based on recent successful clinical trials, an rTMS device for
the treatment of MDD has been approved by the U.S. Food and Drug
Administration. rTMS is used either singly or in combination with
pharmacotherapy and is associated with fewer cognitive and memory side effects
when compared with ECT (George et al., 2010; McGirr et al., 2015).
Mood disorders, particularly those at the extreme ends of the mood spectrum,
can significantly interfere with daily functioning. Symptoms of both MDD and
BPD interfere with one’s ability to work, socialize, recreate, sleep, eat, and learn.
The impact on occupational performance, however, can vary greatly based on
factors such as severity of symptoms, responsiveness to treatment, number of
episodes, co-occurring conditions, and age of onset. For example, an adult with a
high level of premorbid functioning, who experiences a single episode and
responds well to intervention, may successfully return to work and family roles.
For many individuals, however, there are numerous barriers to occupational
performance once symptoms occur.
Activities of Daily Living
The ability to care for personal health such as eating well, following a healthy
diet, sleeping, and exercising regularly can be impaired. Previously maintained
daily routines can be disrupted. Symptoms of avolition and anhedonia seen in
depressive episodes can decrease motivation for exercise, proper nutrition,
grooming, and hygiene. Symptoms of distractibility, impulsivity, and
psychomotor disturbances that are characteristics of a manic or hypomanic
episode can interfere with the quality of self-care. Additionally, sleep patterns
are often disturbed, with individuals not getting adequate sleep, sleeping
excessively, or experiencing poor sleep quality. Weight gain or loss is common
and can be related to either changes in daily habits or long-term medication use.
Early onset in childhood or adolescence, a time of developing skills and
interests, coupled with high rates of recurrence throughout adulthood, can limit
one’s ability to develop educational, social, and work skills. For individuals who
continue to experience residual symptoms between episodes, it can be even more
difficult to re-engage in meaningful life roles. The individual dealing with
recurring depressed, manic, or cycling moods has great difficulty managing daily
tasks that can include work, school, parenting/caregiving, home maintenance,
and healthy leisure.
Many individuals with mood disorders lack the educational and training
requirements needed for a successful career. Moreover, persons suffering from
depressive episodes are likely to experience symptoms of anhedonia, avolition,
and cognitive difficulties, which decrease motivation and ability to work. Studies
reveal that those diagnosed with MDD or BPD are less likely to work outside the
home, either full- or part-time (Mojtabai et al., 2015; Substance Abuse and
Mental Health Services Administration [SAMHSA], 2009), and those who do
work experience lowered productivity and increased absenteeism (Kessler et al.,
2006; Mojtabai et al., 2015; SAMHSA, 2011). An evidence-based practice for
supported employment for adults with mental illness including MDD and BPD
has demonstrated substantial improvements in employment outcomes as well as
decreased hospitalization, arrest, and incarceration (SAMHSA, 2011).
Children and adolescents with early onset of a mood disorder may
experience symptoms that can negatively impact school performance, such as
lower scores on achievement tests and impaired peer interactions, including
social withdrawal or fighting and disruptive behavior. Studies have shown that
high school students with depression, particularly females, are more likely to
drop out of high school and are less likely to enroll in college (Fletcher, 2007).
Young adults with depression are also more likely to disenroll in college
especially when engaged in substance use (Arria et al., 2013).
Involvement in healthy leisure activities is usually limited, as well. Those
with MDD are likely to socially isolate and cease participation in activities due
to symptoms of anhedonia and avolition. During a manic or hypomanic episode,
individuals may experience an initial burst of creative, goal-directed energy, but
they are more likely to engage in risky or dangerous activities and, over time, to
become more unable to organize time and activities. Interventions such as the
best practice IMPACT (Improving Mood—Promoting Access to Collaborative
Treatment) have substantial evidence of effectiveness in combating avolition and
anhedonia in older adults with MDD or BPD (Penkunas & Hahn-Smith, 2015).
In general, those who experience recurring episodes, limited treatment
success, co-occurring medical or psychiatric conditions, and lack of healthy
support systems are more likely to demonstrate an overall decline in functioning.
Early intervention and ongoing access to treatment and other support networks
can improve one’s ability to maintain meaningful and productive roles and to
experience a greater quality of life through social participation.
CASE-STUDY 1
Phyllis spent excessive hours at her new human resources job, planning
how to take over the company. She was visibly irritated with what she
perceived as organizational inefficiencies and spoke about her concerns to
her employers. Her grandiosity was evident to all of her coworkers. She
drafted numerous versions of detailed proposals to “take the company to the
next level.” Despite working long hours at her computer each day, she
failed to complete the tasks she was hired to do. She was continually
distracted by her big plans. In addition, she began shopping for new clothes
that she felt would be in keeping with her elevated status. She ran up large
credit card bills and was in financial trouble. Phyllis found it difficult to
sleep regularly and often stayed up until 3:00 or 4:00 AM working on her
computer, although she rarely accomplished what she set out to do. She
tried using over-the-counter sleep aids and Melatonin and started drinking a
few glasses of wine before bed, but she worried that she would develop a
dependency. For these same reasons, she was reluctant to see a physician
about her mood issues, even though she began to recognize that her
behavior was perceived as odd by her colleagues.
When she shared her business ideas with family members, they gave
her feedback about how unrealistic she was being and urged her to focus on
her job. She responded defensively, unable to recognize how her behavior
was negatively affecting her work. Although she was highly talented in her
field, she was fired after 3 months on the job. After she was fired, the other
areas of her life began to spiral out of control. She missed two job
interviews related to not being able to sleep because she was up all night
preparing. Phyllis felt this lack of sleep was associated with beginning to
have visions at night that she could communicate directly with leaders of
industry with her thoughts. When she disclosed these thoughts to her best
friend, her friend convinced her to talk to her family doctor. Her family
doctor referred her to a psychiatrist, and she was diagnosed with BPD with
psychotic features. She began treatment with medication and cognitive-
behavioral therapy and, within 4 months, was able to return to the job
market where she obtained a job in a similar industry as she had worked
before. Although she still had times when her mood shifted outside of her
“normal bandwidth,” she found that medication, therapy, regular yoga,
checking in with three good friends at least weekly for check-ins, and
avoiding alcohol allowed her to live the kind of life she was proud of.
CASE-STUDY 2
Justin is a 9-year-old boy who was admitted to the child and adolescent
psychiatric inpatient unit at the local community mental health center. He
had been living in a foster home since being removed from his biological
parents’ home at age 7, due to neglect.
His foster parents describe Justin as quiet and passive in his interactions
with others, avoiding eye contact and wearing his hair long, over his eyes.
They reported that Justin spends most of his free time in his room, alone,
watching television or playing video games. Schoolwork is a daily struggle
for Justin’s foster parents, as they indicate he is disinterested in school and
avoids completing his assignments. They report he “throws temper
tantrums” when they make him do homework. He frequently complains of
having an upset stomach when it is time to get ready for school in the
morning. During the initial interview with the occupational therapist, Justin
shared that he felt he was “bad” and that was why he had been removed
from his biological parents’ custody.
During his stay in the inpatient unit, Justin completed a more
comprehensive evaluation. He reported that he has times when he feels like
he does not want to wake up and has considered hanging himself in his
room. He described times when he cut himself on his thighs or calves to
feel a sense of relief and calm. He reported a lack of motivation and energy
and that he feels unworthy of friendship or the love that his foster parents
and grandparents give to him. Justin talked about wanting to sleep all day
most weekends, often not eating except when someone was watching him
or would notice his lack of appetite.
Once stable and no longer considered a suicide risk, Justin was
discharged from the hospital. He continues to work with an occupational
therapist, a social worker, and his family physician. In addition, he joined a
group at school focused on resiliency. He gradually started talking to a few
boys in his grade and joined an Olympics of the Mind group after school.
Within 2 years, he and his foster parents decided to pursue adoption, and he
happily reported to his support team that he understood that he was a really
cool kid and that “my parents are pretty lucky to be able to adopt me…they
got that going for them….so there’s that.”
RESOURCES
National Alliance on Mental Illness (NAMI) 3803 N. Fairfax Dr., Ste. 100
Arlington, VA 22203
Phone: 703-524-7600
Fax: 703-524-9094
www.nami.org
National Institute of Mental Health (NIMH) Science Writing, Press, and
Dissemination Branch 6001 Executive Boulevard, Room 8184, MSC 9663
Phone: 1-866-615-6464
Fax: 301-443-4279
www.nimh.nih.gov
NARSAD
60 Cutter Mill Road, Suite 404
Great Neck, NY 11021
Phone: 516-829-0091
Fax: 516-487-6930
www.narsad.org
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C H A P T E R
9 Schizophrenia and Other
Psychotic Disorders
Ann Chapleau
KEY TERMS
Affect Alogia Anhedonia Antipsychotic medication Asociality Avolition
Catatonia Delusions Disorganized behavior Disorganized thinking Dysphoria
Extrapyramidal syndrome Hallucinations Negative symptoms Paranoia
Premorbid functioning Prodromal phase Psychosis Tardive dyskinesia
Megan is a 12-year-old girl, in the 6th grade at middle school. She was born in
Texas, but moved with her mother and four siblings to Michigan when she was
five. Megan’s father was jailed in Texas for running a methamphetamine
laboratory in their family home. The family moved in with Megan’s
grandmother in the Detroit area, and her mother was able to find part-time
employment in hotel housekeeping. Megan had difficulty adjusting to her new
living situation and starting school for the first time. She had trouble following
classroom rules such as sharing supplies and toys. In the second and third grade,
she struggled with learning to read and write. Her IQ score was 85. She did not
develop any close friendships. She struggled academically with most subjects,
but excelled in art, often drawing or painting very dark and strange images that
she could not explain. She became increasingly withdrawn and disinterested in
peer-related activities such as participating in sports and going to the
movies.When Megan was 12, she was referred for psychological testing, which
revealed that she had the clinical symptoms of psychosis. Megan revealed to the
psychologist that Satan had appeared to her on multiple occasions. She was
tormented by voices telling her that she was evil and should commit suicide. She
was seen by a psychiatrist who diagnosed her with early-onset schizophrenia,
admitting her to a local child and adolescent psychiatric hospital for medication
and psychotherapy.
Schizophrenia is one of the most severe, complex, and debilitating of all mental
health disorders. It is a progressive disorder that can be treated, but not cured.
This lifelong brain disorder is characterized by periods of psychosis, which is
the presence of delusions or hallucinations without insight, and/or disorganized
thoughts/speech, abnormal motor behavior, and diminished volition and
emotional expression. It is also marked by a progressive decline in daily living
skills, including work and education skills, social/relationship skills, and basic
self-care abilities (American Psychiatric Association [APA], 2013). This chapter
will focus primarily on the disorder of schizophrenia. A brief overview of other
psychotic disorders considered part of the schizophrenia spectrum will be
provided at the end of the chapter.
Etiology
There is no one single factor found to be the cause of schizophrenia. There are a
number of models that attempt to explain the multiple factors that can contribute
to the development of the disorder. Current research supports a genetic
vulnerability as well as environmental triggers (Karl & Arnold, 2014; Kaur et
al., 2014; Leask, 2004). Findings from advanced brain imaging suggest the
presence of genetic markers, reduced brain activity in the frontal and temporal
regions, and structural abnormalities in all regions of the brain, including
enlarged ventricles, reduced volume of gray matter in the cerebral cortex, and
decreased size of the hippocampus and thalamus (De Peri et al., 2012;
Lieberman et al., 2008). The role of key neurotransmitters, including dopamine,
glutamate, and serotonin, is being explored to determine their role in genetic
alterations (Choi & Tarazi, 2010; Sawa & Snyder, 2002). Emerging research also
suggests that abnormal cortical-subcortical brain connectivity may play a key
role (Woodward & Cascio, 2015).
Complications in prenatal development or during delivery, affecting brain
development, have also been associated with schizophrenia (Brown & Derkits,
2010; Cannon, Jones, & Murray, 2002; Gyllenberg et al., 2015; Walker, Kestler,
Bollini, & Hochman, 2004). Additionally, complications in later brain
development, including chronic cannabis use and exposure to trauma or other
stress, have also been linked to schizophrenia (Duhig et al., 2015; Veen et al.,
2004). Longitudinal studies have focused on the pattern of structural changes in
the brain and have found progressive anatomical changes both prior to and
following onset of the illness (Arango et al., 2008; Kempton, Stahl, Williams, &
DeLisi, 2010; Sun et al., 2009).
Despite major progress in research, the cause of schizophrenia and other
psychotic disorders remains a mystery. With new technology including
molecular genetics, in vivo brain imaging, and advancements in
psychopharmacology, there is hope for discovering the cause or causes of
schizophrenia that can ultimately lead to advancements in prevention and
treatment.
Schizophrenia affects more than 21 million people worldwide (World Health
Organization [WHO], 2014) and 2.5 million American adults (Treatment
Advocacy Center, 2009). The incidence of schizophrenia is low, with a median
value of 15.2/100,000 people. The lifetime prevalence is estimated to be 0.3% to
0.7% (APA, 2013). Schizophrenia crosses all racial, geographical, and
socioeconomic boundaries. Some studies, however, indicate that migrant status,
lower economic status, residing in a higher latitude or urban setting, and male
gender are factors associated with a higher incidence and prevalence (McGrath,
Saha, Chant, & Welham, 2008; Schmitt, Malchow, Hasan, & Falkai, 2014).
Signs and Symptoms
Despite scientific advancements that reveal neurobiological abnormalities of the
brain, schizophrenia continues to be misunderstood in the general public.
Negative stereotypes are reinforced by the unique nature of the signs and
symptoms of this disorder. Schizophrenia affects the brain, which regulates
impulse control, judgment, affect, and social skills. Perhaps most important,
schizophrenia affects one’s self-awareness. As a result, people with
schizophrenia are more likely to have difficulty recognizing and accepting that
they have the disorder. This lack of self-awareness is similar to anosognosia, a
lack of awareness of neurological damage, often seen in individuals following
stroke or other acquired brain injuries. Lack of self-awareness can lead to
resistance to treatments that could help reduce or eliminate symptoms.
Symptoms of schizophrenia vary greatly, but fall within the following five
categories: delusions, hallucinations, disorganized thinking/speech, grossly
disorganized or abnormal motor behavior, and negative symptoms (APA, 2013).
Delusions are fixed beliefs that, even in the face of contradictory evidence,
are typically due to a misinterpretation of an event or experience. People
with schizophrenia may experience more than one type of delusion at a time
or at different points in time during the course of the illness. Delusions are
categorized as bizarre or nonbizarre. Bizarre delusions are characterized by
beliefs or events that are clearly impossible and not related to everyday life
experiences, such as a belief that aliens have impregnated the person while
he/she was asleep. An example of a nonbizarre delusion is the belief that all
coworkers are talking about the person. The individual may interpret all
private conversations observed at work to be about him or her. Delusional
content can include any of the following types:
Persecutory: The most common of all delusions are delusions in which
one believes himself or herself to be victimized, ridiculed, or placed
under surveillance by known or unknown persons. Some people who
suffer from persecutory delusions, such as paranoia, believe that
someone is attempting to poison them, leading to a refusal to eat or
drink. Another example is thought broadcasting, in which the
individual is convinced that outside forces are able to transmit, or
broadcast to others, his or her inner thoughts.
Referential: These are also fairly common forms of delusions. In this
form of delusion, one believes that common cues from the
environment, such as facial expressions of celebrities on television,
casual comments in daily conversations, or newspaper stories, are
specifically targeted to the individual, holding special meaning or a
message for him or her.
Somatic: This form of delusion is marked by beliefs that involve the
person’s body. For example, a person may believe he or she has
received a secret operation while under anesthesia or that she is
pregnant, despite evidence to the contrary.
Religious: People who suffer from religious delusions may believe
they are Jesus Christ or are acting out direct orders from God.
Grandiose: Examples of this delusion include believing that one is all-
powerful or important, believing that one is acting on secret orders
from the President of the United States, or believing oneself to be a
genius or multimillionaire.
Erotomania: This is a belief that another person, often one unknown to
the individual, is in love with the individual.
Hallucinations are the experience of particular sensations that are not real
to others and that are experienced while awake. The most common form is
auditory, in which an individual hears voices or sounds. The individual may
perceive voices in the external environment, or inside his or her head, often
more than one voice conversing, or speaking directly to the person. Voices
can be taunting and cruel, sometimes commanding the individual to
perform certain acts, such as harming self or others, or can be familiar
voices that are perceived as friendly companions. Visual hallucinations
involve seeing images of people or objects in the environment. The
individual may describe seeing shadowy figures or the image of a dead
body. Other less commonly seen forms of hallucinations include olfactory
(smell), tactile (touch), and gustatory (taste) hallucinations.
Disorganized thinking: Speech content, which can provide clues about
thought processes, may encompass any of the following types of
disorganized thinking:
Loose associations: answers that begin to veer “off track” of the
original questions
Tangential: unrelated comments or answers
Incoherent: often referred to as “word salad” and is seen in more
severe cases
Grossly disorganized behavior or catatonia is unpredictable, socially
inappropriate behavior that interferes with daily activities. Examples of
disorganized behavior include agitated or angry outbursts with no known
provocation, sexually acting out in public (e.g., masturbation), and
difficulties performing goal-directed tasks such as meal preparation or
grooming. Catatonia is an abnormal motor behavior characterized by a loss
of responsiveness to environmental cues. The individual may assume rigid
or bizarre postures and resist attempts made to move or reposition him or
her. Excessive, nonpurposeful motor activity may also be observed. In
extreme cases, the individual appears to be completely unresponsive, as in a
catatonic stupor.
Negative symptoms are features that represent an absence of function or
experience. Two prominent features of schizophrenia include diminished
emotional expression and avolition. Diminished emotional expression is
restricted facial expressions, eye contact, speech intonations, and
movements that are typically used to convey meaning or emphasize speech.
Avolition is a lack of motivation to engage in social or productive activities.
Other negative symptoms include alogia (impoverished speech),
anhedonia (loss of pleasure in previously enjoyed activities), and
asociality (decreased interest in socialization and maintenance of
relationships).
Cognitive Symptoms
Memory, attention, language, and executive function, such as abstract reasoning
and planning skills, are affected by schizophrenia. Use of neuroimaging and
cognitive tests has revealed impaired neurocognitive functioning in completing
tasks requiring use of frontal and temporal lobes (Keefe & Harvey, 2012;
Lieberman et al., 2008). Individuals with schizophrenia demonstrate reduced
ability to process visual stimuli (Koethe et al., 2009) and to respond to
environmental stimuli, due to motor skills deficits (Walker et al., 2004). This
limited visual processing may lead to deficits in social cognition, which is the
ability to recognize, interpret, and act upon social cues during social interactions.
There is a substantial body of research supporting the social cognitive deficits in
this disorder (Chung, Mathews, & Barch, 2011).
Mean IQ scores range from 80 to 85, which is significantly lower than the
norm of 100 for the general population (Jibson, Glick, & Tandon, 2004). People
with schizophrenia also lack skills in identifying social cues and solving social
problems. In general, research has not yielded any specific cognitive deficit
present in all individuals with schizophrenia. Impairments appear generalized
and vary greatly from person to person.
Affective Symptoms
Flattened or inappropriate affect is a common symptom of schizophrenia, and
people with schizophrenia are more likely to demonstrate difficulties in
identifying and expressing emotions. Studies have found that people with
schizophrenia have a limited ability to scan and recognize facial expressions of
others (Bediou et al., 2007). This, coupled with a limited ability to identify social
cues, can lead to maladaptive social functioning.
Mood disturbances are also commonly seen. Dysphoria, depressed, anxious,
or angry mood can be present during or following a psychotic episode and may
require intervention. Individuals who develop insight into the severity of their
illness are at greater risk of becoming depressed or experiencing demoralization.
Onset of symptoms can be gradual or acute, but there are usually earlier signs of
dysfunction in both premorbid functioning, the period from birth to the
prodromal phase. The prodromal phase can range from weeks or months to
years before full onset of symptoms. Studies of premorbid functioning reveal
subtle signs of problems in motor development, affect, and school performance.
Impairments in cognitive functioning are often documented in school records
and formal testing much earlier in life (MacCabe et al., 2013; Russell et al.,
1997). Some individuals do not present with significant premorbid “clues,” but
begin to show early signs of psychosis and unusual behavior in the prodromal
phase. Nonspecific clinical symptoms seen in the prodromal phase can include
affective changes such as depressed or anxious mood, irritability, insomnia, and
cognitive changes such as impaired concentration and difficulty attending to
tasks. Late-emerging symptoms in the prodromal phase can include
suspiciousness, brief hallucinations, and perceptual difficulties (Kulhara,
Banerjee, & Dutt, 2008).
The course of the illness varies greatly, with multiple episodes and
changeable symptoms. The APA provides a classification of the longitudinal
course that can be applied 1 year after initial onset. The course can be classified
as a first, multiple, or continuous episode, in either an acute episode or in partial
or full remission.
Some individuals are able to maintain independent living and work
competitively, while others experience a chronic decline in functioning resulting
in the need for 24-hour supervision and care. Approximately 10% to 20% of
individuals are able to maintain remission for 5 years after the first psychotic
episode, but the majority experience continued relapses (Jibson et al., 2004).
Noncompliance with medication and chronic substance abuse significantly
increase the risk for relapse (Alvarez-Jimenez et al., 2012). Relapses, which are
associated with deterioration in daily functioning, can also be triggered by lack
of available psychosocial treatment and support, noncompliance with
psychosocial treatment, and environmental stressors (Jibson et al., 2004). Over
time, with multiple psychotic episodes, negative and cognitive symptoms may
become more prominent while delusions and hallucinations are more likely to
decrease in intensity in late middle age (Lieberman et al., 2008).
Other factors that can negatively impact prognosis include male gender,
having a gradual and early onset of symptoms, a family history of schizophrenia,
poor premorbid functioning, dysfunctional family relationships, and experience
of abuse or neglect (Jibson et al., 2004; Walker et al., 2004). Even when
medication has been shown to reduce or even stop symptoms such as
hallucinations and delusions, cognitive impairments, such as decreased executive
functioning and abstract reasoning, are more likely to persist. Individuals with
more severe cognitive loss are apt to experience decreased occupational
performance and quality of life than do those who demonstrate cognitive
improvements (Jibson et al., 2004; Tolman & Kurtz, 2012).
People with schizophrenia die, on average, 12 to 25 years earlier than do
those in the general population. These deaths are usually related to pulmonary,
cardiac, and infectious diseases and cancer (Colton & Manderscheid, 2006;
Crump, Winkleby, Sundquist, & Sundquist, 2013). Complications from
antipsychotic medications, self-neglect, fear of health institutions, lack of access
to health care, and increased suicide rate (Meltzer, 2002) are all contributing
factors to this disparity in life expectancy. Individuals with schizophrenia are
also at a higher risk of death from a motor vehicle accident or when killed by a
car as a pedestrian (Treatment Advocacy Center, 2009).
Diagnosis
The criteria for a diagnosis of schizophrenia require the presence of symptoms
for a period of at least 1 month, with some clinical signs present for at least 6
months (APA, 2013). The age of onset for schizophrenia is between 16 and 30
years of age and is typically earlier for males than for females. Diagnosis after
age 45 is unusual (National Institute of Mental Health [NIMH], 2009). Early-
onset schizophrenia, however, can be diagnosed in childhood or adolescence.
Childhood onset refers to a diagnosis of schizophrenia before the age of 13 and
is characterized by an insidious onset, rather than a clear first episode, with
multiple neurodevelopmental impairments present prior to onset of psychotic or
negative symptoms (Arango et al., 2008). Adolescent onset usually has a clear
first episode, with a mean onset age of 15 (Arango et al., 2008).
The focus of treatment is to reduce or eliminate symptoms and to provide
environmental supports to enhance quality of life. The primary treatment for
symptom reduction continues to be medication, although technological advances
show promise for other medical or surgical approaches to treatment.
Pharmacological Treatment
Prior to the 1950s, treatment consisted of institutionalization, including physical
restraints, seclusion, and even lobotomy. There was no medication for relief of
clinical symptoms. Thorazine was the first antipsychotic medication introduced
in the 1950s. Other similar medications, referred to as “first-generation” or
“typical” antipsychotics, were also quickly developed. This first group of
medications, which included Haldol, Prolixin, and Navane, proved effective in
decreasing positive symptoms of schizophrenia by blocking dopamine receptors.
Unfortunately, the side effects were often severe, including extrapyramidal
syndrome (abnormal movements similar to Parkinson’s disease), tardive
dyskinesia (motor abnormalities such as writhing movements), cardiac
problems, and heavy sedation.
The first atypical antipsychotics were introduced in the late 1980s. Atypical
antipsychotics differ from the typical or first-generation antipsychotics in that
they occupy different neurotransmitter receptors. Clozapine (Clozaril) was
introduced in 1989 and received FDA approval in 1990. It is considered to be
effective in treating psychotic symptoms for individuals who were previously
unresponsive to other first-generation medications. A major drawback, however,
is the potential for serious side effects, including development of
agranulocytosis, which requires regular blood work to monitor white blood cell
count. Despite its effectiveness, it is not a favored medication among people
with schizophrenia due to the medical risk, additional side effects,
inconvenience, and excessive cost (Jibson et al., 2004).
Other atypical antipsychotics introduced since the 1990s include risperidone
(Risperdal), olanzapine (Zyprexa), quetiapine (Seroquel), ziprasidone (Geodon),
aripiprazole (Abilify), and paliperidone (Invega). They also are less likely to
cause motor abnormalities such as extrapyramidal effects, but there can be other
side effects including sedation, mild hypotension, weight gain, akathisia
(restlessness), dry mouth, and constipation (Lieberman et al., 2005; NIMH,
2009).
Serious side effects from medications contribute to a decreased quality of
life. In addition to symptoms such as constipation, blurred vision, and impaired
sleep, individuals may also experience motor impairments such as tardive
dyskinesia, which is characterized by involuntary facial and body movements.
For some, tardive dyskinesia remains a permanent condition even when
medications are stopped.
Repetitive Transcranial Magnetic Stimulation
While medications have been successful in reducing symptoms such as
hallucinations and delusions, there has been no proven treatment for negative
symptoms. Transcranial magnetic stimulation (TMS) was developed in the
1980s to study brain function. It involves creating a magnetic field that passes
through the skull, creating a current in the brain that activates nearby nerve cells.
A coil of wire, wrapped in plastic, is held to the head, while a capacitor is
discharged to create the magnetic field. Researchers learned that repeated
applications (repetitive transcranial magnetic stimulation [rTMS]) over the
course of several days appear to affect brain activity. The magnetic field can be
targeted to specific regions of the brain where nerve cells are associated with
psychiatric symptoms. Recent studies of rTMS, while inconclusive, show
promise in reducing negative symptoms of schizophrenia (Diabac-de Lange,
Knegtering, & Aleman, 2010; Matheson, Green, Loo, & Carr, 2010). Other
studies of rTMS have also shown effectiveness in reducing positive symptoms of
auditory hallucinations, when used in conjunction with antipsychotic medication
(Bagati, Haque Nizamie, & Prakash, 2009).
Electroconvulsive Therapy
Electroconvulsive therapy (ECT) is a medical procedure that consists of
inducing a seizure by administration of electrical shock using electrodes attached
to the scalp. Anesthesia is used for this brief procedure. It is generally
administered in a series over the course of days or weeks. The mortality rate for
ECT is approximately 2 deaths per 100,000 treatments and usually are associated
with anesthesia complications (Fink & Taylor, 2007). ECT is used for treating
severe symptoms, such as catatonia, that do not respond to medication. Studies
indicate some short-term benefits in global functioning when used in conjunction
with antipsychotic medication (Tharyan & Adams, 2005). A more recent
systematic study, however, examined randomized controlled trials of ECT
compared to sham ECT (Poublon & Haagh, 2011), a placebo treatment, and
found that the sham ECT group also demonstrated some improvements over
time, suggesting a placebo effect. Side effects of ECT include confusion,
experienced immediately after the procedure, as well as short-term memory loss
for about 1 to 2 weeks.
Other Psychotic Disorders
In addition to schizophrenia, there are several other psychotic disorders as
classified by the APA (2013). Schizophrenia and the following disorders all
share the common feature of active psychosis.
Schizophreniform Disorder
Clinical features are nearly identical to schizophrenia with the exception of two
differences: (1) Total duration of the illness is more than 1 month but less than 6
months, and (2) occupational performance deficits may not be present. About
one-third of all people diagnosed with schizophreniform disorder will experience
remission within the 6-month period, but the remaining two-thirds are likely to
eventually be diagnosed with either schizophrenia or schizoaffective disorder.
Schizoaffective Disorder
Clinical symptoms of schizophrenia are present, including delusions or
hallucinations, but at some point in the course of the illness, a major depressive,
manic, or mixed episode occurs. There are two subtypes of schizoaffective
disorder, depending on the mood presentation: bipolar type, which includes a
manic or mixed episode, and depressive type. Age of onset is typically in early
adulthood, but can range from adolescence to late life.
Delusional Disorder
Symptoms include the presence of delusions lasting at least 1 month. Auditory
or visual hallucinations may be present, but are not prominent. Olfactory or
tactile hallucinations may be both present and prominent if they relate to the
delusion. For example, if a person believes he or she has an unknown infectious
disease, the person may experience related body sensations or odors. Activities
of daily living skills are not significantly impaired, although work, social, and
relationship problems can occur as a result of the delusional beliefs. For
example, those who experience jealous or persecutory delusions may
demonstrate angry or violent behavior. Age of onset for delusional disorders is
highly variable, from adolescence to late life. The course of the illness can also
be variable, from chronic to full remission within several months.
Brief Psychotic Disorder
While symptoms can emerge in adolescence or early childhood, the average age
of onset is mid-30s. The disorder is characterized by a sudden onset of psychotic
symptoms or highly disorganized or catatonic behavior lasting between 1 day
and 1 month. The brief episode is followed by a return to premorbid functioning.
There may or may not be a precipitating stressor, such as death of a loved one or
a traumatic experience in war combat. Individuals experience great confusion
and dramatic mood shifts. Occupational performance can be significantly
impaired, and there is an increased risk of suicide due to impulsivity.
Psychotic Disorder Due to Another Medical Condition
In this disorder, delusions or hallucinations are a direct result of a general
medical condition, such as epilepsy, brain lesions, Huntington’s disease, hepatic
or renal disease, lupus, or auditory or visual nerve injuries. The course can be
varied, from a single episode to recurrent. Even when the underlying medical
condition is resolved, psychotic symptoms can continue, particularly in cases of
brain injury.
Substance/Medication-Induced Psychotic Disorder
Clinical features include hallucinations or delusions directly due to effects of a
drug or exposure to a toxin. In cases of intoxication or withdrawal from an
abused drug, the hallucinations and delusions are more severe and present well
beyond what would be expected during the
intoxication/withdrawal/detoxification stages. Onset can result from a single use
of a substance or following prolonged use.
Catatonia Specifier
The presence of multiple catatonia symptoms may be present in a number of
disorders. When it accompanies another mental disorder (Catatonia Associated
with Another mental disorder) or is the result of a medical condition (Catatonic
Disorder Due to Another Medical Condition), the diagnostic code will be
included. A category of Unspecified Catatonia is used when some catatonic
symptoms are present and disruptive to daily functioning but do not meet full
criteria or there is not enough clinical information to make a more thorough
diagnosis.
Other Specified Schizophrenia Spectrum and Other
Psychotic Disorder
This diagnostic category is used when individuals present with some psychotic
symptoms and significant functional impairments that do not meet the full
criteria for any specific psychotic disorder. For example, the individual may
present with only auditory hallucinations. In documenting the diagnosis, the
healthcare provider specifies this as the reason for the “other” diagnosis.
Unspecified Schizophrenia Spectrum and Other
Psychotic Disorder
Similar to the “other” diagnostic category described above, this diagnosis applies
to individuals whose symptoms do not meet full criteria for any of the other
schizophrenia spectrum disorders. It differs in that the healthcare provider cannot
specify the reasons for the limited diagnosis due to a lack of thorough clinical
information.
Because schizophrenia typically presents in early adulthood, a period of
developing new roles and responsibilities such as career, life partnership,
independent living, and parenthood, its lifelong effects are profound. Functional
abilities vary greatly, but can be positively affected by environmental factors
such as the presence of social support systems, financial assistance, and
opportunities for housing or work.
Overall, quality of life is significantly lower for people with schizophrenia,
as they represent lower socioeconomic status, are typically unemployed or
underemployed, and are at higher risk of homelessness and incarceration (WHO,
2009). They are more likely to be victims of crime, especially violent crimes,
and to abuse alcohol and/or drugs. Approximately 50% of all people with
schizophrenia have a co-occurring diagnosis of substance abuse or dependence
(Volkow, 2009). Smoking rates are also high among those with schizophrenia,
estimated as high as 60% to 90% (Hahn et al., 2012), and as high as three times
the general population (Williams & Foulds, 2007). One reason for the increased
smoking rates may be self-medication. Nicotine has been found to stimulate the
brain’s dopamine receptors, positively impacting sensory processing abilities,
including decreased startling response and eye-tracking deficits (Winterer,
2010).
Basic Activities of Daily Living
The ability to manage personal health is significantly compromised.
Antipsychotic medications can cause weight gain, cardiac problems, and other
health risks. Many individuals have impaired motor functioning, visual
processing deficits, and cognitive deficits, which, coupled with avolition and
anhedonia symptoms, can limit interest or ability to participate in traditional
exercise, nutrition, or smoking cessation programs.
Avolition, anhedonia, and disorganized behavior can also affect one’s ability
to initiate and complete self-care tasks such as grooming and hygiene. Impaired
visual processing and difficulty interpreting visual stimuli can be associated with
excessive application of makeup or ineffective attempts to bathe or shave
thoroughly.
The majority of people with schizophrenia do not live or work independently.
The ability to work or succeed in school; to manage a household including
cooking, cleaning, laundry, and budgeting; or to function as a caregiver requires
multiple skills such as higher level cognitive processes, sensorimotor skills, and
social interaction skills, all of which can be affected by the symptoms of
schizophrenia. Those who experience repeated relapses over time, which often
involve hospitalization, demonstrate an overall decline in functioning, which
limits opportunities for independent living.
Education
There is some evidence of impaired academic performance in premorbid
functioning as well as during the prodromal phase, with standardized testing and
general IQ scores in a significantly lower range than the general population
(Fuller et al., 2002; Jibson et al., 2004). Many individuals experience their first
acute onset in young adulthood, which can derail plans for post–high school
education or graduation. In fact, worldwide, about one-third of all people with
schizophrenia do not graduate from high school (WHO, 2009). While
antipsychotic medication can be effective in treating symptoms such as
hallucinations and delusions, there is no intervention that has been shown to
create meaningful improvements in cognitive functioning. For the vast majority
of those with schizophrenia, ongoing cognitive impairments persist and can have
a major impact on academic success.
Work
Competitive work situations require multiple job-specific skills, the ability to
learn new information quickly, and the ability to interact effectively with
coworkers, customers, and superiors. Individuals with schizophrenia are more
likely to lack college education or other technical or skilled trade certifications.
Even with supported employment assistance, people with schizophrenia are
more likely to be placed in minimum wage jobs such as fast-food work, which
requires the ability to process and respond quickly while maintaining effective
communication with coworkers. These work environments are often too
demanding for the individual with active symptoms of schizophrenia. Successful
work environments provide structured tasks, additional time allotted to complete
work, the ability to work part-time hours, and opportunities for job coaching,
particularly when new expectations are introduced.
Leisure and Social Participation
Symptoms of anhedonia and avolition can play a crucial role in limiting
involvement in leisure activities. Psychosocial functioning may be impaired by
both symptoms of asociality and affective symptoms, such as flattened affect and
the ability to recognize and reciprocate appropriate social cues. People with
schizophrenia typically have less financial and transportation resources for
recreation, fewer social contacts, and less interest in leisure activities. As a
result, they are more likely to be socially isolated. In fact, those living in
independent or semi-independent apartments with fewer opportunities for social
participation and support report higher levels of loneliness than do those living
in group homes (Schwartz & Gronemann, 2009). Additionally, engagement in
physically active forms of leisure has been found to be associated with lower
negative emotion (McCormick, Snethen, Smith, & Lysaker, 2012).
CASE-STUDY 1
Joe, a thin, middle-aged man, can often be seen walking about downtown,
wearing a cap fashioned out of aluminum foil, pulling a handcart loaded
with assorted household items and clothing. He is well known to the mental
health community and is a familiar sight to local residents. Joe grew up in a
middle class family, the only child of parents who were both accountants.
His parents described him as a happy baby and a bright student, who had
attended college to study engineering. But things began to change for Joe
and his family when he moved 1 hour away to the state university when he
was 19. Although his teen years were marked by a gradual withdrawal from
friends and family, he began to demonstrate more socially bizarre behaviors
while at college and when home on weekends. He started dressing in dark,
heavy, hooded clothing even in warm weather and developed an intense
preoccupation with watching the C-SPAN television channel. His parents
were concerned about his behavior, but hoped it was a temporary phase of
adapting to his changing life.
During winter break of his sophomore year in college, while his parents
were vacationing in Mexico, Joe was seen by neighbors in the park near his
family home wearing camouflage and sleeping in a small pup tent. Police
were called, and Joe hid in his tent, refusing to speak to them. He was
forcibly removed and taken to the local psychiatric hospital for emergency
admission. During his evaluation by the on-call psychiatrist, he revealed
that he had moved out of his home because he had discovered “bugs”
planted throughout the house. He believed that he was under surveillance
by the CIA and was worried that his parents had been arrested. He reported
that he had been suspicious of a plot against him for quite some time, but
that this final “discovery” was the confirmation for which he was waiting.
When the staff arranged for him to speak to his parents, who reassured him
they were only on vacation, Joe was convinced that CIA agents were
impersonating their voices. A physical exam including blood testing was
conducted to rule out any medical or drug-related cause of the psychosis.
His parents were interviewed to obtain pertinent social and medical history.
They shared that he had experienced some problems over the past 2 years in
getting along with roommates and was only sporadically attending his
classes, resulting in low grades. Joe was diagnosed with schizophrenia, first
episode, currently in acute episode and admitted to the inpatient program
under court order. He was given antipsychotic medications and discharged
to his parents upon their return home. He was not able to return to college
as planned. He attempted to enroll at the local community colleges for
various classes, but was never able to complete the courses.
Joe has been in and out of the psychiatric hospital numerous times in
the past 25 years. His admissions are typically preceded by him not taking
his medications, which results in a return of delusions that he is under CIA
surveillance. He has never been employed and has never had a significant-
other relationship. He is suspicious of others, and so he will not consent to
living in a room and board facility or group home. His attempts at
independent living in community apartments are usually short-lived. He has
been evicted for inability to pay rent as well as for unsafe conditions in the
residence, such as hoarding and not cleaning. He resides for brief periods
with his parents and has had numerous episodes of street homelessness.
CASE-STUDY 2
Maggie was diagnosed with schizoaffective disorder, depressive type, at
age 20, during her last year of college, when she began hearing voices
telling her she was ugly and stupid. She became increasingly depressed and
eventually attempted suicide. She was hospitalized at a local medical
hospital as there was no mental health inpatient facility or program in the
area. The attending doctor prescribed an aggressive course of psychotropic
medication. After a 48-hour stay, when Maggie was no longer actively
suicidal, she was discharged with a prescription and a referral to follow up
with outpatient counseling. She took the medication as prescribed for 3
months, but experienced serious side effects such as gaining 20 lb,
abnormal eye movements, and eventually developing diabetes. Although
she had only been an occasional cigarette smoker, she began chain-smoking
and began smoking marijuana almost daily. She described her cigarette and
marijuana smoking as a way to cope with her constant anxiety.
During this initial 3-month period, Maggie was unable to return to
school or her 10-hour-per-week work-study job she had held at the college
bookstore. She told her parents she would take the medication but that she
wasn’t going to counseling “with all those crazy people.” She moved back
in with her parents and no longer socialized with her dorm roommates or
college friends. She summarized her feelings during that time stating, “I felt
so lonely and isolated. I was embarrassed about how I looked and I was too
nervous to be around anyone. I mostly watched TV and ate a lot of junk
food. I was so afraid I was going to be like this for the rest of my life. I
thought about suicide, too.” After talking with a family friend who was a
mental health case manager, Maggie researched the Recovery Center, a
community drop-in support program in a nearby town, and she began
attending regularly. With help from the staff and peer support specialists
over the past 2 years, Maggie has learned to advocate for herself, working
with a psychiatrist to find a better medication at a lower dosage with fewer
side effects. She participates in cognitive-behavioral therapy, focusing on
learning healthier coping strategies. She would also like to learn yoga and
be more active physically.
Maggie returned to school part-time and completed her bachelor’s
degree. While she still has what she calls her “bad days,” she has been
stable and has not been rehospitalized. She continues to live at home but
has a goal to get her own apartment. She is now passionate about helping
others and has been hired as a peer leader at the Recovery Center. The
people she has met there have become a strong support system for her. She
often speaks to community groups as part of a panel of peer support
specialists, working to end the stigma of mental illness. Maggie says of her
mental illness, “This is not the road I envisioned for myself, but it has
shaped who I am and I am a better person for it.”
RESOURCES
National Alliance on Mental Illness (NAMI) 3803 N. Fairfax Dr., Ste. 100
Arlington, VA 22203
Phone: 703-524-7600
Fax: 703-524-9094
www.nami.org
National Institute of Mental Health (NIMH) Science Writing, Press, and
Dissemination Branch 6001 Executive Boulevard, Room 8184, MSC 9663
Phone: 1-866-615-6464
Fax: 301-443-4279
www.nimh.nih.gov
Brain and Behavior Research Foundation 90 Park Avenue, 16th Floor
New York, NY 10016
Phone: 800-829-8289
https://bbrfoundation.org
Torrey, E. F. (2001). Surviving schizophrenia: A manual for families, consumers,
and providers. New York: Harper Collins Publishing.
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C H A P T E R
10 Anxiety Disorders
Christine K. Urish
KEY TERMS
Agoraphobia Gamma-aminobutyric acid (GABA) Diagnostic and Statistical
Manual of Mental Disorders, Fifth Edition (DSM-5) Fear Generalized anxiety
disorder Panic attack Panic disorder Phobia Social anxiety disorder
A s the occupational therapist arrives on the inpatient psychiatric unit, she finds
Jillian pacing up and down the hallway. Jillian was admitted last evening as a
result of her significant functional decline in all areas of occupational
performance. She has been diagnosed with generalized anxiety disorder by her
psychiatrist. “I know my psychiatrist has written a referral for me to attend
occupational therapy; I know all about occupational therapy,” she states. “I have
worked in this hospital on the pediatrics unit for quite some time. I know the
occupational therapists come to the pediatrics unit and play with the children and
try to get them to move and interact with their parents. I don’t need to play. I am
so anxious. I worry all the time, and it seems as if I worry about everything. I
don’t really think occupational therapy and play will help me at all. I cannot
sleep or rest, and I am very worried that I will be fired because I’m not
functioning as I should because I am anxious and tired.” The occupational
therapist suggests Jillian come with her to discuss occupational therapy services
and to complete an initial interview. The occupational therapist wants to
determine how Jillian’s anxiety is impacting her ability to do everyday things.
As the occupational therapist walks down the hall, Jillian responds by saying,
“I’m not able to be a nurse anymore; then what will I do?” “This play therapy is
not going to help my anxiety, I cannot do anything right and I am certain this is
not going to help me.” “What in the world was my psychiatrist thinking when he
ordered me to attend occupational therapy groups?” “How in the world is this
going to help me at all?” Jillian is obviously thinking that occupational therapy
is one-dimensional. In her mind, occupational therapy is about play. The
occupational therapist knows that she will have to explain to Jillian how
occupational therapy services on the pediatrics unit differ from an inpatient
psychiatric setting. She also knows that she will have to do her best to try and
convince Jillian that occupational therapy can be beneficial to her. The
occupational therapist feels that this information could assist Jillian in improving
her performance skills and decreasing her anxiety. The occupational therapist
looks forward to the challenge of working with Jillian both individually and in a
group of other patients diagnosed with anxiety disorders.If Jillian will learn
relaxation techniques and mindfulness strategies and develop her skill at using
them, through participation in occupational therapy services, her level of anxiety
may decrease. She may find that she is better able to sleep at night, and thus, her
energy level and concentration may improve. The occupational therapist also has
a feeling that Jillian may be overly focused on her success and performance at
work, and that Jillian’s overall life balance—and more specifically her leisure
lifestyle—may be limited. As an occupational therapist, she is concerned about
Jillian’s overall ability to balance her work, activities of daily living,
instrumental activities of daily living (IADLs), continuing education required by
her employment status as a nurse, sleep and rest, leisure, and social participation.
Although Jillian thinks that all occupational therapy includes is play, this
therapist will work with Jillian to assist her in understanding that a balance of
occupations, occupational performance and occupational engagement, is
essential to being productive.
Introduction
This chapter discusses anxiety disorders: how they are classified, are diagnosed,
and differ from “normal” feelings. Evidence-based research on intervention will
be presented throughout the chapter. The impact of anxiety disorders on
performance skills in occupation will be examined through the detailed case
studies at the end of the chapter. Learning resources for development of
additional knowledge are provided at the end of the chapter as well.
Anxiety is defined as “apprehension of danger, and dread accompanied by
restlessness, tension, tachycardia, and dyspnea unattached to a clearly
identifiable stimulus” (Dirckx, 2005). It is important to distinguish fear from
anxiety. Fear is similar in that it is an alerting response to a known, external,
definite threat. Anxiety is a response to a threat that is unknown, vague, and
internal, and it can lead to conflicted feelings (Sadock, Sadock, & Ruiz, 2015). It
is normal to have some degree of anxiety in our lives: “Will I get a raise during
my review with my boss?” “Will my dress be appropriate for the social occasion
to which I am driving?” “Will I get a good grade on the test I recently
completed?” Most often, someone can be nervous or anxious about a number of
life events but be able to perform daily occupations without incident. In our day-
to-day existence, we experience anxiety, whether or not we recognize it as
anxiety. Anxiety can motivate us into action. A certain amount of anxiety or
anxious feelings is quite normal. For example, “I’m anxious about my
performance review at work at the end of the month, so I will go in early or stay
late this week to make sure I am caught up on things” or “I think my clothing is
getting tighter; I seem to have put on a few pounds. I need to spend more time
exercising to lose some weight and improve my physical appearance.” Anxiety,
however, can also be pathological, when we worry incessantly about things that
we cannot control or change. When this incessant worry begins to negatively
impact our ability to work, learn, or socialize, anxiety may be considered
pathologic. Anxiety symptoms may vary from individual to individual (Sadock
et al., 2015).
Classification of Anxiety Disorders
The Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition
(DSM-5) has established criteria to determine if anxiety or anxiety-related
conditions are pathologic (American Psychiatric Association [APA], 2013). The
criteria present both physical and psychological symptoms that must be met for a
diagnosis to be made. A significant change from DSM IV-TR to DSM-5 was the
change from a large category of anxiety disorders in which 11 disorders were
classified. The DSM-5 classifies the previous anxiety disorders in three large
categories. These include anxiety disorders, obsessive-compulsive and related
disorders, and trauma and stress-related disorders. This chapter focuses on the
first category, anxiety disorder. For information on obsessive-compulsive and
related disorders, refer to Chapter 13. Complex Trauma is addressed in Chapter
12. Occupational therapists treat clients diagnosed with these disorders in a
variety of mental health settings including inpatient, partial hospitalization, and
outpatient settings. Occupational therapists also treat clients in a variety of
nonmental health settings in which they may observe anxiety symptoms in the
clients they treat across the life span. If an occupational therapist is observing a
client with anxiety symptoms, they should not be overlooked, as they can
significantly impact occupational engagement and performance as well as
overall health and quality of life.
Although occupational therapists do not make psychiatric diagnoses, a
thorough understanding of the criteria for making such a diagnosis is important
for the clinician to facilitate clinical observations. When considering the
classification of anxiety disorders within the DSM-5, the following diagnoses
are included:
Separation anxiety disorder

Selective mutism
Specific phobia
Social anxiety disorder, previously known as social phobia
Panic disorder
Agoraphobia
Generalized anxiety disorder
Substance/medication-induced anxiety disorder
Anxiety disorder due to another medical condition
Panic Disorder
Panic disorder includes short, sudden attacks of fear, fear of losing control, or
terror (Johns Hopkins, 2016). The diagnosis of panic disorder includes panic
attacks, which recur and are unexpected. A panic attack is described as an
abrupt surge of intense fear/intense discomfort, which can peak within minutes
and at which time four (or more) of the following symptoms occur:
Heart palpations, increased heart rate or pounding heart

Sweating
Shaking or trembling
Sensation of shortness of breath or feeling of smothering
Feeling of choking
Chest discomfort or pain
Nausea or distress in abdomen
Feelings of dizziness, light-headedness, or faint
Chills or sensation of increased heat
Numbness or sensation of tingling
Feelings of not being in reality or being separate or detached from oneself
Fear of loss of control
Fear of death
At least one of the panic attacks has to be followed by 1 month or more of one or
both of the following criteria. An ongoing, persistent concern or worry about
additional panic attacks or the consequences of having additional panic attacks.
The individual fears he or she will lose control, is experiencing a heart attack, or
is “going crazy.” Further, the individual experiences a significant maladaptive
change in behavior related to previous attacks, for example, engaging in
behavior in an attempt to facilitate not having an attack such as avoiding
exercise or situations in which they are unfamiliar. It is important to note that the
symptoms and associated behavior are not able to be attributed to the
physiological effects of a substance (street drugs or prescribed medication) or
another medical condition such as a heart condition or hyperthyroidism.
Phobia
Phobia refers to irrational fears that lead individuals to often avoid certain
objects and specific situations all together (Substance Abuse and Mental Health
Services Administration [SAMHSA], 2015). The diagnosis of phobia includes
the individual presenting with marked and persistent fear or anxiety regarding an
object or situation such as flying on an airplane, sight of certain animals,
receiving an injection, sight of blood, or being at a certain elevation/height. It is
important to note that in children, this fear or anxiety can be expressed through
behavior that includes crying, tantrums, clinging, and lack of movement (APA,
2013). The object or situation that is feared by the individual almost always
provokes instant fear or anxiety. As a result, the object or situation is actively
avoided by the individual, or alternatively the individual may endure exposure to
the object or situation with intense fear or anxiety. When considering the level of
fear or anxiety regarding the object or situation, it is considered out of proportion
to the actual danger posed by the specific object or situation and to the
sociocultural context (APA, 2013).
Individuals with phobia demonstrate fear, anxiety, or avoidance, which is
persistent and must last more than 6 months. The fear, anxiety, or avoidance of
the object or situation causes significant distress in social, occupational, or other
areas of life function. It is common for individuals to have multiple specific
phobias (APA, 2013). It is important to note that agoraphobia is not included in
this category of phobia, rather it is a single category of anxiety disorder.
There are five different types of specific phobias most often observed in
adult clinical populations (Fadem, 2012). These phobias, in descending order of
frequency seen in clinical settings, include situational, natural environment,
blood-injection injury, animal, and other types. Situational phobias include fears
of tunnels, bridges, using public transportation, flying in an airplane, and being
in closed places. Situational phobias are usually more common in adults than in
children. Natural environment phobias include fear of natural occurrences such
as lightning, thunder, heights, and deep water. These fears often present in
childhood. Blood-injection-injury phobias focus on the fear of receiving an
injection or treatment, which requires some invasive bodily procedure. The
specific phobia of animals is the fourth most frequently seen phobia in adult
clinical populations. This phobia includes insects in addition to animals. The
final type of specific phobia is categorized as other type that includes loud
sounds, falling, contracting an illness, and choking. A fear of costume characters
in children is also considered in this category (Fadem, 2012).
The diagnosis of social phobia, now known as social anxiety disorder, is a
marked, persistent fear or anxiety regarding being in one or more social
situations where the individual has the potential to be scrutinized by others. This
can include social interactions such as meeting unfamiliar individuals, engaging
in conversation, being observed by others while eating or drinking, and
performing in front of others such as giving a speech. It is important to note that
in children, the anxiety must occur in settings that include peers, not just adult
interactions (APA, 2013). The person with social anxiety disorder has the fear of
his or her anxiety showing and as a result being negatively evaluated, which will
result in humiliation and embarrassment. As a result of their anxiety and the
negative evaluation of others, they will be rejected or offend others (APA, 2013).
Persons with social anxiety disorder experience fear or anxiety in nearly all
social situations. Therefore, the individual will go to great lengths to avoid social
situations or will endure situations with extreme fear or anxiety. The fear or
anxiety experienced is out of proportion to the actual threat of the social
situation and to the sociocultural context (APA, 2013). Fear, anxiety, or
avoidance is persistent and lasts 6 months or longer and causes a significantly
negative impact upon social, occupational, or other significant area of life
functioning (APA, 2013). It is important to note that the fear, anxiety, or
avoidance cannot be attributed to physiological effect of a substance such as a
prescribed medication or a drug of abuse, or another medical condition. The fear,
anxiety, or avoidance is not better explained by symptoms present in another
mental disorder such as panic disorder, body dysmorphic disorder, or autism
spectrum disorder. If another medical condition is present, such as Parkinson’s
disease, obesity, or disfigurement from an injury or serious burn, the fear,
anxiety, or avoidance is unrelated or considered excessive (APA, 2013). This
diagnosis has one specifier: whether or not the fear is limited to speaking or
performing in public.
Agoraphobia
Agoraphobia in the past was considered within the DSM IV-TR within the
diagnostic category of phobia. Within the DSM-5, agoraphobia is classified as a
separate diagnosis. An individual diagnosed with agoraphobia will demonstrate
fear or marked anxiety in two of five situations which include the following:
Use of public transportation (e.g., plane, train, automobile, bus, ship)

Being in an open space (e.g., bridge, parking lot, open marketplace)
Being in an enclosed area (e.g., shopping mall, theatre, movie cinema)
Being in a crowd or standing in line
Alone outside of the home (APA, 2013)
The individual diagnosed with agoraphobia fears and avoids these situations
because of thoughts that escape may be very difficult or that help may not be
available in the event of developing panic-like symptoms or other symptoms that
would incapacitate or embarrass the individual, such as the fear of falling or the
fear of incontinence, common among the elderly. The agoraphobic situations are
avoided, a companion is required, or the situation may be endured, however,
with great fear or anxiety. The fear or anxiety experienced by the individual is
out of proportion to the actual danger posed by the agoraphobic situations and to
the sociocultural context (APA, 2013). To be diagnosed with agoraphobia, the
symptoms must persist more than 6 months and cause significant distress or
impairment in social, occupational, or other important area of life functioning. If
the individual has another medical condition (e.g., Parkinson’s disease or
inflammatory bowel disease), the fear, anxiety, or avoidance is excessive (APA,
2013).
Generalized Anxiety Disorder
This disorder is diagnosed when an individual has excessive worry and anxiety,
which occurs more often than not for a period of at least 6 months. This
excessive worry or anxiety can include a number of events or activities such as
school or work performance or family concerns (APA, 2013). Individuals have
difficulty controlling their worry, and they characteristically also have three or
more of the following six symptoms:
1. Feelings of being on edge, restless, or keyed up 2. Becoming easily fatigued
3. Feeling as if his or her mind is going blank and difficulty with
concentration 4. Irritability
5. Tension in muscles
6. Difficulty with sleep, which can include falling asleep, staying asleep, or
restless sleep (APA, 2013)
It is important to note that in children only one of the aforementioned items is
required for a diagnosis. The concern of the individual with generalized anxiety
disorder is not related to another DSM-5 disorder such as OCD (anxiety about
contamination) or weight gain as in anorexia nervosa (APA, 2013). Anxiety,
worry, or accompanying physical symptoms cause clinically significant distress
or impairment in social, occupational, or other important areas of life
functioning (APA, 2013). The symptoms cannot be attributable to another
medical condition (e.g., hypothyroidism) or due to physiological effects of a
substance (e.g., prescribed medication or drug abuse).
When considering generalized anxiety disorder in children, worry tends to
focus on competence or quality of performance. Anxiety disorders may coexist
with other disorders such as depression, substance abuse, eating disorders,
schizophrenia, personality disorders, or other anxiety disorders (National
Institute of Mental Health [NIMH], 2016). Comorbidity of another mental illness
was high when individuals were diagnosed with social phobia and generalized
anxiety disorder (Brown, Campbell, Lehman, Grisham, & Mancil, 2001). It is
important to note that comorbidity between depression and anxiety often goes
unnoticed (Bouchard & Verrier, 2005). In individuals diagnosed with an anxiety
disorder, up to 45% are diagnosed with major depression at some point in time in
their lives. The comorbidity between generalized anxiety disorder and panic
disorder is known to exceed 55% (Bouchard & Verrier, 2005). It is important to
note that individuals diagnosed with depression do not develop a secondary
anxiety disorder, rather individuals with anxiety disorder are known to more
often develop a comorbidity of depression (Bouchard & Verrier, 2005).
Individuals diagnosed with social phobia also had high levels of comorbidity
with avoidant personality disorder (Rettew, 2000). When considering anxiety
disorders from a comprehensive perspective, it is important to note that anxiety
disorders may coexist with cancer and heart disease as well (NIMH, 2016). It is
important for clinicians to consider comorbidity to select the most effective
intervention for individuals with anxiety disorders and other coexisting
conditions in everyday practice.
Etiology
The understanding of the causes of anxiety has increased in recent years as
neurochemical and physical pathways of fear and anxiety have been critically
examined (Brantley, 2007). Despite this, more research is needed as we are far
from having a clear understanding of these complex conditions. In examining the
causes of anxiety disorders, one must consider the powerful interaction between
biology, cognitive/emotional influences, and stress. The likelihood of developing
an anxiety disorder includes a combination of life experiences, psychological
traits, and genetic factors. Anxiety disorders, such as panic disorder, have a
stronger genetic basis than do other disorders, although at this time-specific
genes have not been identified (United States Surgeon General [USSG], 1999).
Women are more at risk for being diagnosed with anxiety disorders than men,
although it is not clear as to why. Some researchers suggest the role of gonadal
steroid. Other researchers suggest that women’s response to stress and their
exposure to a wider range of life events is different from those experienced by
men (USSG, 1999). There are three major etiologic considerations in examining
anxiety disorders. These include biological factors, genetic factors, and
psychosocial factors. Three major schools of psychosocial theory have
contributed to the understanding of the causes of anxiety: psychoanalytic,
behavioral, and existential. Each of these frameworks presents conceptual and
practice applications for the treatment of anxiety disorders. According to the
biological theories of anxiety, an excessive autonomic reaction is present with
increased sympathetic tone. Increased catecholamines are released in addition to
increased production of norepinephrine metabolites. Gamma-aminobutyric
acid (GABA) levels are decreased, and this causes central nervous system
hyperactivity. Additionally, a decrease in serotonin causes anxiety, and increased
dopaminergic activity is related to anxiety. The temporal cortex activity in those
with anxiety is decreased. The center of the brain, locus caeruleus, is hyperactive
when anxiety is present, especially during a panic attack (Sadock et al., 2015).
Magnetic resonance imaging evidence indicates that individuals with panic
disorder have pathology in the temporal lobes of the brain, in particular, in the
hippocampus (Sadock et al., 2015). Biological research into the etiology of
generalized anxiety disorder has focused on GABA and serotonin receptors.
There are also indications that genetics may play a role in the etiology of
anxiety disorders. Despite the fact that well-controlled research studies into
panic disorder are limited, available genetic research has indicated that nearly
half the individuals with panic disorder have at least one relative affected with an
anxiety disorder (Sadock et al., 2015). Approximately 5% of individuals with
high levels of anxiety have a variant of the gene associated with serotonin
metabolism (Sadock et al., 2015). Phobias also seem to be more common among
family members. More specifically, the blood-injection–type phobia has a
significantly high familial tendency. Further, first-degree relatives with social
phobia are three times more likely to be diagnosed with social phobia than those
who have first-degree relatives without a mental disorder (Sadock et al., 2015).
From a psychoanalytic perspective, anxiety is viewed as developmentally
related to childhood fears of disintegration and is related to the fear of loss of a
loved one or an object or the fear of castration. Clinicians critically examine
possible triggers when working with individuals with anxiety disorders. In
people diagnosed with phobias, from a psychoanalytic perspective, the
individual attempts to repress. When this fails, other defense mechanisms such
as avoidance are called upon (Sadock et al., 2015). Individuals diagnosed with
generalized anxiety disorder, according to the psychoanalytic perspective, have
unresolved unconscious conflicts. Behavioral theories propose that anxiety is a
response that is learned from exposure to parental behavior or through the
process of classical conditioning. Anxiety disorders include faulty, distorted, or
counterproductive thinking patterns (Sadock et al., 2015).
The success of treating individuals with anxiety disorders using a behavioral
approach lends credence to this approach in intervention (Sadock et al., 2015).
Anxiety is acquired through classical conditioning and observational learning
and maintained through operant conditioning. Learning theory is of significance
in the treatment of phobias and provides clear explanation for many symptoms
experienced by the phobic individual (Sadock et al., 2015). According to the
cognitive-behavioral school of thought, an individual with generalized anxiety
disorder has an incorrect and inaccurate perception of danger. This inaccuracy is
facilitated by selective attention to negative information within the environment,
distortion in information processing, and an inability to cope. From an existential
perspective, there is no one specifically identifiable stimulus that facilitates the
feeling of chronic anxiety in an individual. Anxiety, according to an existential
approach, occurs when the individual becomes aware of profound feelings of a
lack of meaning in his or her life. This lack of meaning for some individuals is
more fear provoking than are thoughts of death. With increased concerns of
bioterrorism and nuclear attacks, existential concerns in society have been noted
to be increasing (Sadock et al., 2015). Occupational therapy professionals are
well suited to address anxiety from an existential perspective as we critically
examine meaning and occupation in an individual’s day-to-day existence. The
cumulative and long-term effects of stress contribute to the development of
anxiety and anxiety disorders (Sadock et al., 2015). Chronic stress places an
individual at serious risk for physical illness, emotional, social, and spiritual
dysfunction. Clearly, unmanaged chronic stress needs to be identified and
addressed (Brantley, 2007).
In the United States, 40 million persons experience an anxiety disorder in 1 year
(SAMHSA, 2015). Generalized anxiety disorder affects 6.8 million adults, which
is approximately 3.1% of the US population (Anxiety and Depression
Association of America [ADAA], 2014). Panic disorder affects 6 million
individuals, with women being more likely to experience this diagnosis than
men. Less than 30% of individuals who suffer from anxiety disorders, however,
seek treatment (Lepine, 2002). Phobias affect 19 million individuals, with
women being twice as likely to be affected as men (ADAA, 2014; World Health
Organization [WHO], 2013). Phobias typically begin in childhood; the median
age is 7 years of age at onset (ADAA, 2014). The annual cost of anxiety
disorders is estimated to be $42 to $47 billion per year, which is one-third of the
total cost of all mental illnesses (ADAA, 2014). Of the total cost, $23 billion
(54% of cost) is spent in nonpsychiatric medical costs (physician office visits,
emergency room costs). Persons diagnosed with anxiety disorders are three to
five times more likely to visit their physician and are six times more likely to be
hospitalized. These are significant health care costs, in addition to potential for
lost productivity for an employer/company. Approximately $4.1 billion (10%) is
spent in indirect workplace costs, $1.2 billion (3%) in mortality costs, and $0.8
billion (2%) in pharmaceutical (prescription) costs. Costs to the workplace are
attributed more to lost productivity rather than absenteeism. Other than phobia,
all anxiety disorders were found to be associated with impairment in work
performance. It is important to note that for every one dollar spent in the United
States on the treatment of anxiety and depression, a return of four dollars is
realized in improved health and ability to work (World Health Organization
[WHO], 2016).
Panic Disorder
This diagnosis affects 5 million American adults (NIMH, 2016). Women are two
to three times as likely to be diagnosed with panic disorder as men. An
underdiagnosis of panic disorder in men, however, may skew this distribution
(Sadock et al., 2015). The male-to-female ratio for panic disorder without
agoraphobia is 1:1 and agoraphobia is 1:2 (Sadock et al., 2015). The ethnic
differences present in panic disorder are small. One social factor contributing to
the development of a panic disorder is relational, for example, experiencing
separation or divorce. The mean age for diagnosis of a panic disorder is 25 years
of age (Sadock et al., 2015). This disorder often begins in late adolescence or
early adulthood (NIMH, 2011).
Phobia
Overall, phobias are the most common mental disorder in the United States with
19.2 million American adults being diagnosed with a specific phobia (NIMH,
2016). Specific phobia is considered to be more common than social phobia. In
women, specific phobia is the most common mental disorder. In men, the most
common mental disorder is substance-related disorders, with specific phobia
being the second most common mental disorder (Sadock et al., 2015). Despite
the fact that phobias are the most common mental disorder, a significant number
of persons do not seek help for their phobia(s) or are misdiagnosed upon seeking
medical attention (Sadock et al., 2015). In considering social anxiety disorder, 15
million American adults are diagnosed with this disorder. Men and women are
equally likely to experience social anxiety disorder that usually begins in
childhood or early adolescence (NIMH, 2016).
Studies indicate 6.8 million American adults experience this disorder (NIMH,
2016), with twice as many men as women impacted (Sadock et al., 2015). The
lifetime prevalence for this diagnosis is 5%. In anxiety disorder clinics,
approximately 25% of the clients treated are diagnosed with generalized anxiety
disorder (Sadock et al., 2015). Lifetime prevalence rate for anxiety disorder is
28.8% (Kessler et al., 2005). When considering individuals older than age 55,
the best estimate prevalence rate based on Epidemiologic Catchment Area
(ECA) data for anxiety disorders was 11.4%. Prevalence of simple phobia in
those older than 55 years of age was 7.3%, and panic disorder was 0.5%. The
prevalence rates reported indicate that anxiety disorders are common in the
general population, with anxiety disorders having the earliest age of onset of
mental disorders (Kessler et al., 2005). Prevalence rates for anxiety disorders
according to gender are between one-third and two-thirds higher in women
(WHO, 2013). Rates of anxiety disorders in women could be due to the
perception women hold regarding stressful life events (USSG, 2005). Further,
the World Health Organization (WHO) identified gender-specific risk factors,
which include gender-based violence (including sexual violence), subordinate
social status, socioeconomic disadvantages specific to women, and
responsibilities to care for others (WHO, 2013).
Signs and Symptoms
It is important to consider the messages presented by society as we examine the
signs and symptoms of anxiety disorders. Messages such as “Don’t Worry, Be
Happy” abound. It is something that individuals may subscribe to in trying to
keep their symptoms “under control,” so no one will recognize they are
experiencing a great deal of internal turmoil and distress (Roth & Fonagy, 2004).
In making a diagnosis of an anxiety disorder, presenting symptoms are the
primary consideration. Many anxiety disorders may present with similar physical
symptoms. In some disorders, however, the symptoms are more severe than in
others. Although all individuals may experience some degree of fear, worry, and
anxiety, health care providers need to critically examine the diagnostic criteria
relative to the degree of symptoms present and the duration of symptoms.
Panic Disorder
The diagnosis of panic disorder includes experiencing a panic attack. Symptoms
of a panic attack include heart pounding; increased sweating; feelings of
trembling or shakiness; feeling short of breath; feeling as if choking will occur;
chest tightness; pain; discomfort; abdominal discomfort; distress or nausea;
feeling faint, light-headed, or dizzy; feelings of unreality or depersonalization;
fear of going crazy or losing control; fear of death; sensation of tingling or
numbness; hot flashes; or chills. Individuals who experience at least four of
these symptoms, which appear quickly and peak within 10 minutes, are said to
have experienced a panic attack. To obtain the diagnosis of panic disorder, an
individual must have experienced four attacks within 4 weeks or one attack
within the last month with ongoing worry or concern of when another attack will
strike (SAMHSA, 2015). Panic attacks typically are short lived but can last up to
10 minutes in duration. Rarely, attacks will last up to 1 hour and can occur while
sleeping. Individuals with panic disorder who reported childhood physical abuse
were more likely to have additional comorbid psychiatric diagnoses including
depression and had a higher likelihood of attempting suicide (Friedman et al.,
2002).
Phobia
Symptoms of a phobia include many of the physical symptoms previously
presented in panic disorder, including sweating, increased heart rate, and
trembling (SAMHSA, 2015). Phobias are traditionally classified by the specific
fear through the use of Greek or Latin prefixes. For example, acrophobia is the
fear of heights, ailurophobia is the fear of cats, pyrophobia is the fear of fire, and
xenophobia is the fear of strangers (Sadock et al., 2015). Diagnosis of a phobia
should indicate which type of phobia (animal, natural environment, blood-
injection, situational, other) (APA, 2013). If the person experiences extreme
anxiety when faced with the feared situation or object, the individual’s daily
routine, social activities, and interpersonal relationships are impacted, and,
because of these fears, a diagnosis may be made by a qualified and trained
professional. Phobias may impact many different areas of occupation depending
on the specific feared stimulus of the individual. In social anxiety disorder, the
symptoms present are similar to those for specific phobia. In addition, the
extreme anticipatory anxiety regarding performance or social situations
negatively impacts cognition and leads to actual or perceived poor performance
in the situations evoking fear, which only further perpetuates the cycle of anxiety
(APA, 2013).
Panic Disorder
The age of onset for panic disorder is typically early to middle adulthood (Pigott,
2003). However, onset can occur at childhood, early adolescence, or midlife
(Sadock et al., 2015). Panic disorder is associated with increased risk of
agoraphobia and depression. One relationship factor identified as contributing to
panic disorder is the history of a recent divorce or separation (Sadock et al.,
2015). Comorbidity of depression, substance abuse, and other anxiety disorders
are associated with poor prognosis. Considered a chronic condition, the course
of panic disorder is variable across clients as well as within a single individual.
Studies that have examined intervention for panic disorder are not easily
interpreted because of the inability of researchers to control for the effects of
treatment (Sadock et al., 2015). However, 30% to 40% of individuals have
presented as symptom free after long-term follow-up. One-half of individuals
with this diagnosis continue to have symptoms, but these symptoms are mild
enough to not significantly impact their lives. Approximately 10% to 20% of
individuals continue to have symptoms despite treatment (Sadock et al., 2015). It
is important that clinicians use a psychometrically sound instrument such as the
Panic Disorder Severity Scale to monitor clients and determine if symptoms are
recurring to facilitate treatment modification. By responding to recurrence of
symptoms, a complete relapse may be avoided (Rouillon, 1996). However,
whereas some individuals may experience episodic outbreaks with years of
remission between, still others may experience continuous severe symptoms
(APA, 2013).
Phobia
The most predominant phobias among women are animal, natural environment,
and situational-specific phobias; whereas blood-injection injury is experienced
equally by both genders (APA, 2013). Females are more frequently affected than
males with the ratio of 2:1; however, this varies depending on phobic stimuli
(APA, 2013). Phobias are the most common anxiety disorder. Individuals may
commonly avoid the feared stimulus and may go to extreme lengths to avoid the
feared stimulus (Sadock et al., 2015). Depression is a comorbid condition in
approximately one-third of people with phobia. Animal phobia, natural
environment phobia, and blood-injection phobia appear to peak at childhood.
Other phobias have a peak onset at early adulthood. Approximately 75% of
individuals have phobias about one or more situation or object (APA, 2013).
Phobias have been found to run in families, especially blood-injection phobia
(Sadock et al., 2015). Limited data exist regarding the course of specific phobia
despite being the most common anxiety disorder. Individuals frequently do not
seek treatment for this condition and may live with anxiety for many years. The
condition appears to remain constant and does not appear to have the waxing and
waning progression that is seen in other anxiety disorders. For individuals who
do seek treatment, a positive response to intervention was associated with better
long-term outcomes (Curtis, Magee, Eaton, Wittchen, & Kessler, 1998). One
study that examined outcomes of individuals diagnosed with phobia 10 to 16
years after treatment challenged the notion that recovery from this diagnosis is
characterized by complete and lasting remission from symptoms (Curtis et al.,
1998).
Social Anxiety Disorder
The age of onset for individuals with social phobia is childhood or adolescence,
and the individual may experience symptoms of the disorder for many years.
Parental psychopathology, including social anxiety disorder, depression, and
parenting style (overprotection or rejection), has been associated with
development of social anxiety disorder in youth (Judd, 1994). This disorder is
commonly associated with substance dependence, depression, avoidant
personality disorder, panic disorder, and generalized anxiety disorder (Lieb et al.,
2000). Social anxiety disorder is more common in women than in men; however,
in clinical samples, the reverse is often true (Sadock et al., 2015). Social anxiety
disorder has onset before other psychiatric conditions (Long, 2011).
Additionally, psychiatric conditions complicate approximately one-third of those
diagnosed with social anxiety disorder. Early intervention of social anxiety
disorder may prevent the onset of other psychiatric conditions (Long, 2011).
Without intervention, the course of social anxiety disorder is chronic and
unremitting. A significant concern relative to this disorder is the strong tendency
of society, including mental health professionals, to trivialize this disorder
(Lipsitz, Mannuzza, Klein, Ross, & Fyer, 1999).

The age of onset for generalized anxiety disorder is variable and difficult to
pinpoint. It can occur as early as childhood (Sadock et al., 2015). Individuals,
when questioned about their symptoms, often recall feeling anxious as long as
they can remember. This condition tends to worsen without treatment and
especially during times of increased stress (Fadem, 2012). One-third of
individuals with generalized anxiety disorder symptoms seek psychiatric
intervention. More often, these individuals present to family practitioners,
cardiologists, internists, or gastroenterologists from whom they are seeking relief
for the somatic concerns that they are experiencing as a result of this condition
(Sadock et al., 2015). Because of a high incidence of comorbidity of generalized
anxiety disorder with other psychiatric conditions, a specific course and
prognosis are difficult to identify. Generalized anxiety disorder can be viewed as
a chronic condition in which the individual may experience symptoms that can
be lifelong (Sadock et al., 2015).
At present, there are no specific laboratory tests that can be used to diagnose
anxiety. Society has become more aware and accepting of identification and
treatment of mental illness over the last several years. This is apparent through
public health initiatives targeted at addressing mental health concerns, such as
National Mental Illness Awareness Week, Anxiety Screening Day, and
Depression Screening Day, as well as through the increasing amount of
information regarding mental illness, diagnosis, and treatment available via the
Internet (Long, 2011; NIMH, 2016; SAMHSA, 2015). Current guidelines for
treatment of severe mental illness can be found in four different categories
(Richards, Klein, & Carlbring, 2003). These categories vary depending on the
score and stringency for which the guidelines rely on research evidence. The
categories include recommendations, comprehensive treatment options,
algorithms, and expert consensus guidelines. In 1998, comprehensive treatment
options were developed for panic disorder by the APA (Mellman et al., 2001).
However, the strength of evidence presented in support of these treatment
options is less stringent than Patient Outcomes Research Team (PORT)
recommendations. PORT treatment recommendations for schizophrenia were
developed by the U.S. Agency for Health Care Policy and Research. The PORT
project critically examined literature, which was then followed by expert review.
The PORT recommendations contain very specific evidence of efficacy of
treatment interventions that supported the utilization of these interventions. The
APA guidelines developed for panic disorder were by a professional organization
and did not require the evidence considered for inclusion to be as stringent as the
guidelines used for the development of PORT. As a result, the APA treatment
guidelines are less prescriptive than the PORT treatment recommendations.
Algorithms, a single rule or set of rules, are used when solving a problem.
Medication algorithms are considered within practice guidelines. Algorithms
provide practitioners with a step-by-step approach to clinical decisions
considering medications. At present, the Texas Medication Algorithm Project
has the most extensive collection of medication algorithms for persons with
mental illness. Unfortunately, there are no algorithms for anxiety disorder
treatment. Algorithms do exist, however, for schizophrenia, bipolar disorder, and
major depressive disorder (Mellman et al., 2001). The last category includes
expert consensus guidelines. These are recommendations based upon surveys
completed by a comprehensive array of experts in the treatment of identified
conditions. These guidelines do not rely on critical analysis of research
literature. The rationale provided for the development of these expert guidelines
is related to the fact that research literature at times does not address specific
points in treatment decision making. At present, expert treatment guidelines exist
for panic disorder (APA, 2013). In addition to expert treatment guidelines for
practitioners, guidelines have been developed for patients and families regarding
these diagnoses (APA, 2016). People with anxiety disorders are three to five
times more likely to seek the care of a physician and six times more likely to be
hospitalized for a psychiatric disorder (Copeland, 2003). From an occupational
therapy perspective, their functional abilities would be assessed, and it would be
determined how their anxiety was impacting activities of daily living, IADLs,
sleep/rest, education, work, play, leisure, and social participation (American
Occupational Therapy Association [AOTA], 2014). The occupational therapist
may consider administering an activity configuration, role checklist, interest
checklist, or self-assessment of occupational functioning to determine functional
deficits (Reed, 2014). Assessments such as the Canadian Occupational
Performance Measure, Occupational Performance History Interview—2.1, and
the Occupational Self Assessment are appropriate considerations (Reed, 2014).
The most common treatments for anxiety disorders include a combination of
pharmacologic and psychological interventions with the exception of specific
phobia for which there is no good pharmacologic treatment (Fadem, 2012).
Remission is the ultimate goal for the treatment of anxiety disorders (Kjernisted
& Bleau, 2004). Occupational therapy intervention is targeted at changing
performance deficits in areas of occupation as a result of the symptoms
experienced by the person (Reed, 2014). Occupational therapy intervention may
also examine activity demands and modifications that could be made to the
activity or environment to assist the individual in independence in areas of
occupation (AOTA, 2014). Although some individuals may continue to
experience symptoms of anxiety throughout their lives, occupational therapy
intervention can be focused toward the development or reestablishment of
meaningful daily routines. Engagement in meaningful occupation can serve to
facilitate adaptation, which can lead to improved health and wellness, quality of
life, and positive life satisfaction. It is important to remain client centered as
interventions that may reduce anxiety in one individual may prove ineffective for
another. Medications utilized to treat anxiety disorders include anxiolytics and
antidepressants, specifically SSRIs (Sadock et al., 2015). One should be cautious
when working with clients who are on benzodiazepines who have addiction
concerns, as these medications are highly addictive. Further, older adults are at
risk of falls when on benzodiazepines because of side effects impacting balance
as a result of the half-life of the medication. Side effects of benzodiazepines
include sedation and fatigue, cognitive and memory impairments, delayed
reaction time, impaired balance and coordination, hangover effects, withdrawal,
and abuse potential. For individuals diagnosed with panic disorder,
antidepressant medications such as paroxetine hydrochloride (Paxil), which is a
selective SRI, or benzodiazepines such as alprazolam (Xanax) are approved by
the Food and Drug Administration and are often prescribed (Fadem, 2012;
Sadock et al., 2015). Paxil has been shown to be effective with individuals
diagnosed with social phobia. Benzodiazepines may be used for social phobia on
a very short-term basis (Sadock et al., 2015). Some research has indicated that
cognitive-behavioral interventions are superior to pharmacologic approaches,
whereas other research indicates the contrary (Sadock et al., 2015). In
considering cognitive-behavioral interventions for anxiety disorders, a variety of
interventions exist. Findings from one study indicated that when treating
individuals with panic disorder, progress can be obtained through the use of a
self-help workbook and brief therapist contact (Hecker, Losee, Robertson-Nay,
& Maki, 2004). An emphasis on family and client psychoeducation, focusing on
symptomatology, nature and course of panic disorder, and lifestyle
modifications, has been suggested as evidence-based interventions that cost less
(in Australia) than the cost of drug therapy over a 1-year period of time
(Andrews, Oakley-Browne, & Castle, 2003). The efficacy of cognitive-
behavioral therapy (CBT) has been examined through evidence-based research.
It is important to note that CBT interventions are often coupled with
pharmacotherapy; however, within individuals diagnosed with panic disorder,
use of medications may interfere with CBT intervention approach (Shearer,
2007). The APA’s comprehensive treatment guidelines for panic disorder
indicate that most individuals can be treated on an outpatient basis and may
rarely require hospitalization (APA, 2013). Cognitive-behavioral interventions
suggested by the APA include the use of psychoeducation, panic monitoring,
breathing monitoring, anxiety management skill development, cognitive
restructuring, and in vivo exposure. Establishing and maintaining a therapeutic
alliance with the individual diagnosed with panic disorder is viewed as a key
element in successful intervention as well as educating the individual on the
early signs of relapse.
Panic Disorder
From an occupational therapy perspective, individuals with panic disorder may
need assistance in the area of IADLs. People may be fearful of leaving the
house, and therefore, community mobility may be impaired. Systematic
desensitization can be useful but very stressful for individuals in addressing this
fear (Andrews, Oakley-Browne, Castle, Judd, & Baillie, 2003). Relaxation
training, including deep breathing, progressive muscle relaxation, visualization,
and autogenic training, is an effective intervention utilized by occupational
therapy practitioners for individuals with panic disorder and phobias (Bonder,
2010). Although relaxation therapies such as visualization, deep breathing,
meditation, and progressive muscle relaxation have been shown to decrease
anxiety, there is limited research (Conrad & Roth, 2007).
Phobia
When considering other available interventions for phobia, 70% to 85% of
individuals responded with clinically significant improvement when exposure
was used as a behavioral intervention. The addition of cognitive components
appeared to add little efficacy (Roth & Fonagy, 2004). Therapist-directed
exposure was suggested rather than self-directed exposure to the feared stimulus.
Although some studies have used virtual reality techniques as intervention for
the treatment of phobias, this may be cost prohibitive and technically
challenging but could provide a clinic with the ability to expose the client to a
wide range of feared stimuli (North, North, & Coble, 1998).

When considering evidence-based intervention for social anxiety disorder, five
cognitive-behavioral treatments including exposure therapy, cognitive
restructuring, exposure coupled with cognitive restructuring, social skills
training, and relaxation were compared and found to be moderately effective
with no differences obtained at the end of the study or at the follow-up (Federoff
& Taylor, 2001). Further, both individual and group interventions were found to
be equally beneficial to individuals with this diagnosis. Some obstacles to
effective treatment for social anxiety disorder are as follows: the individual’s
avoidance of treatment because of fear, shame, or stigma. Limited screening to
assess social anxiety disorder is available at present. Assessment and
intervention may be directed toward somatic complaints expressed by the
individual rather than the social anxiety disorder. Physicians lack knowledge of
effective treatment options or they trivialize the client’s concerns or view them
as unchangeable (Bruce & Saeed, 1999). Despite these facts, cognitive-
behavioral therapy is useful when treating social phobia (NIMH, 2016). Both
cognitive therapy and exposure therapy present good efficacy, and the
combination presents the largest effect sizes in meta-analytic reviews (Taylor,
1996).
Limited research has been completed examining the link between sensory
defensiveness in adults and increased anxiety (Kinnealey & Fuiek, 1999). It is
hypothesized that many symptoms of sensory defensiveness may be
interchangeable with psychiatric disorders including generalized anxiety
disorder. Research has explored the use of sensory integration interventions for
decreasing anxiety levels by using deep pressure, tactile, and proprioceptive
activities (Kinnealey, Oliver, & Wilbarger, 1995).

Generalized anxiety disorder can be addressed using cognitive therapy focusing
on education and lifestyle alterations focusing on how the external environment
influences internal feelings. Addressing diet (caffeine intake), medication use
(over-the-counter medications may increase anxiety), and the need for regular
exercise can also be helpful in addressing generalized anxiety disorder
(Kjernisted & Bleau, 2004). Rational/cognitive approaches that focus on
assisting the person in replacing negative self-statements with more positive
ones can be effective with individuals diagnosed with generalized anxiety
disorder. Time management activities that assist the individual in prioritizing
activities may assist in decreasing anxiety as well. Expressive activities such as
journal writing, drawing, or other craft activities can provide a mechanism for
the individual to communicate his or her feelings and may assist in the
development of coping skills (Reed, 2014). Clinicians should be mindful
regarding the length of intervention provided to individuals with anxiety
disorders. Outpatient sessions between 6 and 7 weeks were deemed too short by
clients in two different studies (Prior, 1998; Rosier, Williams, & Ryrie, 1998).
Structured course content should include opportunities for skill development,
communication, and practice with ongoing monitoring by the clinician.
Cognitive aspects of intervention such as relaxation training and assertiveness
were found to be most beneficial per client report. An 8-week course was felt to
allow more time for learning and practice of the cognitive aspects of the course
deemed most important by those diagnosed with anxiety disorders (Rosier et al.,
1998).
Impact on Client Factors and Occupational
Performance
The impact of anxiety disorders on client factors is dependent on the specific
disorder with which the client is diagnosed. In the area of mental functions,
specifically global mental functions, sleep, temperament, and energy can be
impacted by the diagnosis of an anxiety disorder. In the area of specific mental
functions, the following areas are impacted: attention, reduced recall (memory),
impaired ability to make associations, time management, problem solving,
decision making, and emotional functions in the area of self-control (Sadock et
al., 2015). From a sensory perspective, individuals with an anxiety disorder may
demonstrate increased startle response. Physical signs present in anxiety disorder
from a neuromuscular and movement-related perspective include feeling shaky,
muscle tension, backache, headache, and fatigue. From a cardiovascular and
respiratory perspective, symptoms include tachycardia and hyperventilation,
which can lead to syncope (passing out). From a gastrointestinal perspective,
clients may experience signs of diarrhea and have the feeling of an upset
stomach or “butterflies” in the stomach. When considering the genitourinary
system and reproductive functions, clients may experience urinary frequency and
decreased libido, which could relate to reproduction or lack of desire for sexual
relationships (APA, 2013; Fadem, 2012; Sadock et al., 2015).
Panic Disorder
The symptoms an individual may experience during a panic attack can have a
negative impact upon the many different areas of occupation, including care of
others, care of pets, childrearing, community mobility, safety and emergency
maintenance, shopping, sleep/rest, formal educational participation, job
performance, and leisure and social participation (AOTA, 2014). A common
concern of individuals who experience panic disorder is anticipation of when the
next attack will occur. This fear can significantly alter daily roles, habits,
routines, and rituals. This can have a negative impact upon their ability to initiate
performance in daily activities. The individual who experiences a panic disorder
and has children may worry about the well-being of his or her children. He or
she may feel as if he or she is dying while having an attack. Those experiencing
symptoms of panic disorder may begin restricting themselves to their residence
for fear of having a panic attack while in public, and this behavior can facilitate
agoraphobia.
Social anxiety disorder impacts the areas of educational participation, such as
having to get up in front of class and give a presentation. Job performance, as
well as other social roles and responsibilities, may be impaired (Fischler &
Booth, 1999). Communication skills may be negatively affected because of the
individual’s significant level of anxiety. Individuals with social phobia may
experience low self-esteem due to their inability to perform up to self-imposed
standards, yet they frequently do not seek assistance for their concerns.
Individuals with this disorder may be characterized by others as “nervous” or
“ineffective” in social situations. To increase the potential for individuals with
social anxiety disorder to experience success, the occupational therapist should
work toward establishing environmental control to reduce anxiety regarding the
unknown. Individuals may benefit from working alone initially to reduce self-
consciousness and at their own pace. Positive feedback for effective performance
is essential. Working with individuals diagnosed with social phobia to self-
disclose diagnosis to supportive individuals may assist in reducing self-
consciousness and embarrassment when symptoms arise (Fischler & Booth,
1999).

Any area of occupation can be impacted by generalized anxiety disorder. The
individual may express excessive worry about himself or herself and his or her
own health and well-being (IADLs) or his or her educational participation, job
performance, as well as social participation. This anxiety disorder is one that
many people express as similar to the gray cloud of worry that follows them
everywhere and as such impacts every area of occupation.
CASE-STUDY 1
Jillian is a 44-year-old registered nurse. She has worked in nursing for 20
years on a hospital pediatrics unit. For the last 7 months, for more days than
not, Jillian finds it difficult to control her level of anxiety and worry. She is
worried about getting along with coworkers, pleasing the physicians with
whom she works, and interacting appropriately with the parents of the
children for whom she is providing care. Additionally, Jillian has been
worrying about her children’s school performance and the fact that many
companies in her community are downsizing. Although her husband has
frequently reassured her that his position is stable, she cannot help but
worry that he will lose his job or be demoted. With the economy being on a
downhill slide, this serves to increase Jillian’s fears regarding employment
and finances. Jillian also worries about her difficulty attending to her tasks
as a nurse. She finds her mind going blank, and she has difficulty
concentrating on what a physician is saying to her while she is doing
rounds. Three times during the last month, she has recorded physician’s
orders incorrectly. The unit secretary has caught these errors and brought
them to Jillian’s attention. Jillian is demonstrating difficulty responding in
an appropriate fashion to safety concerns, which are typically presented in
her workplace such as “code blue.” She feels others know of her problems.
She is concerned about her relationships with her coworkers since she is so
preoccupied by her anxiety, and she finds herself irritable at work and has
“snapped” at a several coworkers over the last few months. Jillian has been
having extreme difficulty falling and staying asleep. She has been
awakening 2 to 3 hours early and is not able to fall back to sleep. As a
result, she feels fatigued and has been experiencing increased muscle
tension, backaches, and frequent headaches. Jillian describes her ongoing
keyed-up feelings of restlessness like “walking on eggshells.” Jillian
expresses frustration in her lack of ability to relax and inability to decrease
her anxiety. Jillian expresses little appetite and has experienced noticeable
weight loss over the last 7 months. At home, Jillian has not been preparing
meals for her family as she had in the past and rather has been relying
solely on frozen dinners and takeout food for family dining. Jillian picks up
the house but does not clean per se. She used to keep her house immaculate,
but for the past several months, she has verbalized feelings of fatigue and
lack of desire to keep things clean in her home. Although she expresses
numerous interests including scrapbooking, cooking, reading, and walking,
she has not been able to participate in these leisure activities because of her
anxiety level and accompanying fatigue. Jillian had previously been active
as a volunteer at her church and involved in activities of her son’s football
league, but at this time, she feels so overwhelmed that she is unable to
complete these tasks. She has been caring for her children marginally. She
has experienced difficulty helping them with their school work and has
been distant or short with them in her communication. Jillian’s husband and
friends too have noticed a change in Jillian’s behavior. Although they have
been supportive of and encouraging Jillian, they do not know what to do or
how to assist her in diminishing her level of worry. Jillian agrees to go on
family outings in the community with her husband and children, but at the
last minute, she backs out. When asked why, she provides little to no
explanation for the last minute change of plans. Jillian’s marriage is “on the
rocks” because she is experiencing difficulties in communicating to her
husband and he is starting to wonder if she may be having an affair as she
appears so anxious all of the time. Infrequently, she will speak to friends on
the phone but has not gone to social activities with her friends in months.
Financially, Jillian and her family have money in their savings, live
comfortably, and have been saving for their children’s college education
and their retirement. Despite these facts, Jillian spends between 4 and 6
hours each weekend reviewing the financial status of the family and
constantly worrying about expenses that are considered by many others to
be routine expenditures. Jillian has seen a psychiatrist who has prescribed
medication and supportive psychotherapy 1 week ago. Despite Jillian’s
complaints of fatigue, the psychiatrist encouraged her to try to walk one to
two times per week as physical exercise is beneficial to those with
generalized anxiety disorder. Additionally, deep breathing exercises were
reviewed in attempts to decrease her level of anxiety. Jillian plans to
continue to pursue therapy on an outpatient basis with a psychologist and
occupational therapist for relaxation training and to critically examine life
stressors and develop effective coping mechanisms to deal with her current
level of anxiety.
Phobia
Emma is a 9-year-old female who is in fourth grade. She lives with her
mother, grandmother, and brother Larry. Emma is a high achieving student
in school, who has many friends and enjoys a variety of after-school
activities such as Girl Scouts, playing soccer, and computer games. Three
years ago, Emma’s parents divorced and her mother and brother moved
from Oregon to Iowa. In Iowa, in the spring and summer when it rains,
often times there are severe thunderstorms. Emma had never experienced
extreme thunder and lightning until she moved to Iowa. In May of this year,
Emma missed 12 school days due to thunderstorms and her ongoing and
persistent worry regarding her safety during a severe storm episode. When
the weather becomes ominous, Emma will head for the basement of her
home and will not come out until she is 100% certain that there is no
chance of thunder or lightening. Even when Emma’s mother shows her on
the Weather Channel on television, there are no storms in the area and the
most recent storm is over, Emma will persist in staying in the basement.
Emma worries she will be struck by lightning, and her irrational fear keeps
her from engaging in many occupations during stormy weather. Not only
does Emma’s fear negatively impact her engagement in school and
extracurricular activities, she will not eat and has been experiencing bouts
of constipation. Emma refuses to eat or drink as there is no bathroom in the
basement, and she does not want to have to leave the basement due to her
extreme fear. Emma’s mother after several missed school days due to this
behavior contacted the school counselor. The counselor reported Emma’s
irrational fears have negatively impacted her school performance and peer
engagement. At school, if Emma hears thunder, she immediately runs to the
bathroom which is in an interior location on the lower level of the school
and will not leave. The school counselor stated she is worried as the other
children are beginning to “tease” Emma about her phobia of storms. The
counselor has tried to work with Emma and has taught her deep breathing
but feels that more intervention is needed. Emma too is concerned as her
performance and attention at school is “off” as she appears to always be
staring out the window, examining the current weather situation. The school
counselor suggested that Emma’s mother seek assistance at the local mental
health center. The mental health center employs an occupational therapist
who considers Emma in the context of her school and home environments
and how her phobia is negatively impacting her engagement in desired
occupations. Together with the psychologist, the OT and client (Emma and
her family) develop strategies for Emma to try in an attempt to ally her
fears and anxieties. The psychologist utilizes virtual reality technology and
the occupational therapist uses behavioral and sensory interventions. The
OT works with Emma on developing a sensory box that she can use to self-
soothe when her negative thoughts/feelings seem to take over. Further, the
OT instructs Emma’s mom on strategies she can use at home from a
behavioral perspective to reinforce the use of the sensory box and positive
coping techniques. The school counselor reported that Emma has appeared
more engaged in school, spending less time attending to the weather
outside, and was able to remain in her classroom for a longer time period
when a storm was occurring. Emma did not rush to the bathroom, rather she
was able to leave her classroom and stand/sit in the hallway so she could
continue to hear the classroom instruction. Together with the psychologist
and OT and the mental health center, the school counselor is hopeful to
continue the progress Emma has made in addressing her irrational fears of
electrical storms and improve her school performance, after-school activity
engagement, and peer relationships.
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C H A P T E R
11 Neurocognitive Disorders
Joyce Fraker Jayne Yatczak
KEY TERMS
Agnosia
Agrammatica Amyloid precursor protein (APP) Analgesic Anomia
Aphasia
Apraxia
Asterixis Anxiolytics: medications used to treat anxiety Beta-amyloid plaques
Circumlocution Computerized tomography Diffusion tensor imaging
Disinhibition Dyslipidemia Dysnomic aphasia Dysgraphia Dysphagia
Electroencephalography Executive function Hyperreflexia Hyperorality
Hypersexuality Lability Logopenic Long-term memory Magnetic resonance
imaging (MRI) Magnetic resonance volumetric Magnetic resonance
spectroscopy Neurofibrillary tangles Neuroleptic Paraphasia Paratonia Personal
episodic memory
Corrine slowly entered Roland’s room and gently awakened him for his morning
activities of daily living (ADL). Roland opened his eyes and looked at her with
an expression of disorientation and fear. Corrine had known him for almost a
year, and though he could never remember her name, he usually greeted her with
a smile of recognition. Today was different. Roland appeared tense, with agitated
movements of his arms and legs. Corrine sat next to him, stroked his arms
rhythmically, and told him her name. “It is such a beautiful day; let’s open the
curtain and look at the view,” she told him. She opened the curtains and handed
him his glasses. Roland put them on without difficulty and turned his vacant
gaze toward the window. Within moments, Roland relaxed, and his limbs
calmed.
Corrine said, “Let’s get dressed. I will help you put on your clean pants.”
She lightly touched a leg to cue him to lift it so she could place his foot in the
pant leg. Roland looked at her, anxious and bewildered. “What do you want me
to do?” he cried. Corrine lightly touched his foot, directing him to lift it. Roland
tightly gripped his upper thighs and he began to shake. He pleaded, louder this
time, “I can’t move them, what do you want me to do?” Corrine gently took his
hand and began stroking his arm. “Let’s just rest for a moment,” she said. After
he had visibly calmed, Corrine gently lifted each foot and placed them into the
pant legs. She positioned his walker and said, “You can help us stand.” She
lightly guided him by the elbow, and he came to a stand. “Thank you for doing
such a good job,” Corrine told him, and helped him pull on the pants. “Now all
we need are shoes, and then we can get some breakfast.” Roland looked at her,
again without recognition, the fear now replaced with calm.
Key Terms
Positron emission tomography Procedural memory Prodromal Prosopagnosia Recent memory
Remote memory Semantic memory Semantic variant speech Short-term memory Somnolence
Sundowning Tau
Topographical orientation
Description and Definitions of Neurocognitive
Disorders
In 2013, the Diagnostic and Statistical Manual of Mental Disorders, fifth edition
(DSM-5), redefined dementia and most cognitive disorders as “neurocognitive
disorders,” abbreviated as NCD. Although dementia is still a recognizable term,
officially what was known as vascular dementia, frontotemporal dementia, and
dementia with Lewy bodies are now called, respectively, vascular neurocognitive
disorder (vascular NCD), frontotemporal neurocognitive disorder
(frontotemporal NCD [FTNCD]), and neurocognitive disorder with Lewy bodies
(NCDLB). NCD due to Alzheimer’s disease can be simply stated as Alzheimer’s
disease (AD).
NCDs include a range of conditions including delirium, disorders formerly
defined as dementias, and NCD due to traumatic brain injury (TBI). This chapter
will focus on the types of NCD most commonly seen by occupational therapists
with the exceptions of NCD due to TBI, which is more completely covered in
another chapter.
Delirium
Description and Definition of Delirium
The diagnostic criteria for delirium are as follows: (a) disturbance in attention
with decreased ability to focus, sustain, or shift attention; (b) an additional
change in cognition (such as memory loss, disorientation, language disturbance)
or the development of perceptual disturbance (hallucinations, paranoid
thoughts); (c) the disturbance quickly develops, usually within hours or days,
and the severity of symptoms fluctuate throughout the course of a day; (d) the
changes in attention and cognition cannot be explained by a preexisting or
developing NCD; and (e) there is medical evidence that the disturbance is either
caused by a medical condition or developed during intoxication or withdrawal
from a substance; such substances include alcohol, prescription medication, and
illegal substances such as cocaine or hallucinogens (American Psychiatric Association,
2013).
It is not unusual for delirium to occur when a person is being treated, or is in

need of treatment, for an acute physical condition. A high fever can bring on
delirium, causing the person to be confused, to misinterpret shadows, to be
disoriented (especially in a hospital environment, which is unfamiliar and
possibly frightening), and to be unable to express thoughts and needs clearly.
These symptoms might fluctuate, just as the degree of a fever might fluctuate
during the course of a day. An asymptomatic urinary tract infection is often
suspected when a delirium occurs without clear signs of a medical problem. The
delirium ends after the acute medical condition clears.
Etiology of Delirium
Delirium is caused by one or more underlying medical conditions. Although a
fever can bring on a delirium, the rise in body temperature is merely a symptom
of an underlying medical condition. Medical conditions associated with delirium
are many and varied. They include adverse medication side effects; substance
intoxication or withdrawal; infection; metabolic disturbances; traumatic head
injury; vascular injury; cancer; and inflammatory disorders (Peterson et al., 2001,
2008). Risk factors for delirium include increased severity and complexity of
physical illness, use of many prescription medications, extremes of age (older or

younger), and the presence of a baseline cognitive impairment, such as an NCD
(Hales & Yudofsky, 2003; Samuals & Neugroschl, 2008). Delirium can manifest in
confusion and disorientation following surgery and, in some cases, may be the
first sign of a more serious NCD such as NCD with Lewy bodies (Weiner & Lipton,
2009). In fact, delirium is a frequent finding during recovery from hip fractures,
stem cell transplant, acquired immunodeficiency syndrome (AIDS), and terminal
cancer (Hales & Yudofsky, 2003).
Incidence and Prevalence of Delirium

Estimating the incidence and prevalence of delirium is difficult because it is
often overlooked or left untreated. Sadock, Sadock, and Ruiz (2014) state that
delirium is commonly under-recognized by health care workers. In adults aged
55 and older, delirium may be present in as many 1% of dwelling adults, 10% of
emergency department patients, 80% of critical care patients and 80% of patients
in end of life care. According to the DSM-5, 14% to 24% of individuals admitted
to hospitals have delirium as well as up to 60% of individuals in nursing homes
or postacute care settings (American Psychiatric Association, 2013).
Signs and Symptoms of Delirium

The prodromal symptoms of delirium can include restlessness, anxiety, sleep
disturbance, and irritability. Clinical features of delirium include altered arousal
and disturbance of the sleep-wake cycle. The person may have daytime
sleepiness, nighttime agitation, difficulty falling asleep, or wakefulness
throughout the night; in some cases, there may be a complete reversal of the
night-day sleep-wake cycle. Perception may be altered, with misperceptions,
illusions, delusions, or hallucinations. The wrinkled pattern of a blanket may
appear to be an object; a shadow in the corner seems to move and is perceived as
threatening. The person will likely have decreased attention, impaired memory,
or disorientation to time or place; there can be rapid shifts in emotion as well as
periods of agitation (American Psychiatric Association, 2013). Neurological abnormalities
can include dysgraphia (inability to write a sentence), constructional apraxia
(evidenced by inability to draw a clock face), dysnomic aphasia (difficulty
naming objects), motor abnormalities (tremor, asterixis, or hand-flapping
tremor), myoclonus or muscle spasms, and reflex and tone changes (Hales &
Yudofsky, 2003).
The three subtypes of delirium are hyperactive, hypoactive, and mixed.

Hyperactive delirium is characterized by agitation, restlessness, and excessive
emotional reactivity or emotional lability. Hypoactive delirium has much
different features such as somnolence, withdrawal, decreased responsiveness,
and apathy. Mixed delirium is seen when the person has symptoms of both
hyperactive and hypoactive delirium within the same day. The person with
hypoactive delirium makes little or no demands on staff and may not be
diagnosed or treated for delirium as quickly as the person with hyperactive
delirium. The occurrence of delirium, and especially hypoactive delirium, is
associated with increased length of hospital stay and increased morbidity (Girard,
Pandharipande, & Wesley, 2008).
Course and Prognosis of Delirium

Delirium has a rapid onset. For persons who have delirium, many meet
diagnostic criteria within the same day that they developed their first symptoms,
and most meet criteria within 48 hours of emergence of symptoms. The course
usually fluctuates. There can be lucid intervals as well as periods of confusion
and anxiety within the course of a day or even hours. It is not unusual for
symptoms to worsen in the evening or at night, and this phenomenon is known
as sundowning (Sadock, Sadock, & Ruiz, 2014).
Delirium usually has a brief duration of less than a week, though the
symptoms will persist as long as any condition causing the delirium is present.
After treating the causative factor, symptoms of delirium usually recede in three
to 7 days. For older patients, the delirium can last longer and take as many as
two weeks to resolve (Sadock, Sadock, & Ruiz, 2014). The possible outcomes for
delirium range from full recovery to death (Hales & Yudofsky, 2003). It should be
noted that a person does not die from delirium. For a person who is declining
with a terminal illness, it is not uncommon for delirium to develop as the body’s
life-sustaining systems begin to fail. It is also important to note that there is a
risk for falls that accompanies delirium. The person hospitalized postoperatively
or for a serious illness may attempt to get out of bed even though he or she may
not have the needed strength, balance, or coordination. Disturbed thought
processes including impulsivity, emotionality, confusion, and fear may also lead
a person with delirium to remove lines and tubes necessary for his or her medical
treatment or engage in other, potentially dangerous behavior (Schneider & Levenson,
2008).
Medical Management of Delirium

Treatment for delirium involves treating any underlying cause or medical
condition. It is important to withdraw any sedatives or most medications that act
on the central nervous system (CNS). One exception to this rule is when the
delirium is related to withdrawal from alcohol or sedatives, in which case the use
of anxiolytics is indicated. In other patients with delirium, treatment may
include the neuroleptic haloperidol (Schneider & Levenson, 2008). Since delirium is
thought to be a reflection of a neurotransmitter dysfunction, antipsychotics may
restore the acetylcholine/dopamine balance (Seitz & van Zyl, 2006). The person with
delirium requires extra supportive physical care, including attention to nutrition
and hydration, and maintenance of a safe and quiet environment. If hospitalized,
it is also helpful for the person to have positive, orienting cues, such as familiar
pictures or things nearby, and frequent contact with family or loved ones
(Sadock, Sadock, & Ruiz, 2014).
For a person who is normally alert with intact memory, the symptoms of
delirium are a significant cause for concern and should be considered a medical
emergency. If the person is recovering from a medical condition that resulted in
delirium, care should be taken that long-term decisions, such as guardianship
and nursing home placement, be avoided until the delirium has cleared. Consider
the possibility of an individual who has a delirium caused by a prescribed
medication, such as a sedative. If the delirium is not accurately diagnosed and
the medication routine changed, the person could be assessed as being unable to
live independently. Delirium is a temporary condition whose course usually ends
as the person becomes medically stable.
Dementia: Alzheimer’s Disease
Description and Definitions of Dementia: Alzheimer’s
Disease
Historically, memory impairment has been the hallmark symptom of dementia.
Many years of research and technology developments have added to the
knowledge base in this field. In 2013, the American Psychiatric Association
published the DSM-5, which did more than just rename dementia as a NCD.
Now, the various types of NCD are differentiated by much more complex
symptoms of cognitive impairment, classified as neurocognitive domains. Table
11.1 gives an overview of the neurocognitive domains, which includes complex
attention, executive function, learning and memory, language, perceptual-motor,
and social cognition (American Occupational Therapy Association, 2014; American Psychiatric
Association, 2013).
TABLE 11.1 Neurocognitive Domains

American Psychiatric Association. (2013). Diagnostic and statistical manual of mental disorders (5th ed.).
Washington, DC: Author.
American Occupational Therapy Association. (2014). Occupational therapy practice framework: Domain
& process (3rd ed.). Bethesda, MD: Author. doi.org/10.5014/ajot.2014.682006
The DSM-5 differentiates major and mild NCDs within each type of NCD. A
significant criterion for this differentiation is the level of function. For the NCD
to be a major NCD, independence in instrumental activities of daily living
(IADLs) is impaired. In a mild NCD, there may be capacity for independence in
IADLs though the person may need more time, more effort, and compensatory
strategies (American Psychiatric Association, 2013).
This chapter will focus primarily on the NCD most often seen by the
occupational therapist: NCD due to AD. The remaining types of NCDs will be
reviewed in a briefer format.
Etiology of Alzheimer’s Disease

The cause of AD is poorly understood, and, at present, there is no perfect
biological marker that is diagnostic of AD in a living person (Minati, Edinton,
Bruzzone, & Giaccone, 2009). The clinical diagnosis is one of exclusion, made after
ruling out alternate etiologies, such as cardiovascular disease or Parkinson’s

disease, and after considering current and past medical and psychiatric
conditions, surgeries, metabolic abnormalities, and pharmacotherapy (Burke et al.,
2015). The best diagnostic tools for confirming a diagnosis of AD, such as
magnetic resonance imaging and spectroscopy which refer to the measurement

of radiation intensity, diffusion tensor imaging, cerebrospinal fluid analysis,
positron emission tomography, and electroencephalography, can be difficult and
expensive. But even these advanced tests yield only 60% to 90% specificity and
sensitivity (Minati et al., 2009). The best tools for diagnosing AD, such as magnetic
resonance imaging (MRI) and spectroscopy, diffusion, positron emission
tomography, and electroencephalography can be difficult and expensive.
However, even these advanced tests yield only 60to 90% specificity
development and utilization of superior diagnostic tools have not been a priority
to researchers due to the lack of highly effective treatment options following a
positive diagnosis (Minati et al., 2009). However, much research has focused on
associated features and laboratory findings relevant to AD in an attempt to
discover its origin(s). This chapter will review some of the major findings now
being studied.
Neuropathology of Alzheimer’s Disease
Neuroimaging tools, such as MRI, are being used to detect the earliest changes
of AD, to differentiate AD from other forms of NCDs, and to improve methods
for clinical trials in AD and related disorders (Jack, C., et al., 2015; DeCarli, 2001). Some
of these physical findings are cortical atrophy, widened sulci, and ventricular
enlargement (Hales & Yudofsky, 2003). This shrinking of brain structure is a result of
neuronal loss, which seems to be caused by neurofibrillary tangles and beta-
amyloid plaques which are tau proteins Amyloid deposits are part of the
histopathological definition of AD; therefore, a great deal of weight is put on the
biomarkers of amyloid in AD research. Beta-amyloid plaques are caused by a
chemical accident or the defective breakdown of a benign substance known as
amyloid precursor protein (APP). APP lives in various parts of the body,
including the brain, and its role in cellular function is unknown. As part of its
mysterious function, APP regularly gets broken down into much smaller soluble
components and is washed away with other decomposed tissues and chemicals.
Under some conditions that are not understood, the breaking apart does not
proceed correctly. The outcome produces sticky, insoluble shards of beta-
amyloid. These shards stick to each other, attracting fragments of dead and dying
neurons, and slowly decline into dense, misshapen plaques (Shenk, 2003). Beta-
amyloid plaques collect outside and around the neurons. It is believed that this
accumulation causes physical damage to axons and that prolonged neuronal
response to this injury ultimately leads to the development of neurofibrillary
tangles and neuronal death (Vickers, Dickson, & Adlard, 2000). Amyloid has a
preference for depositing in regions of the brain that support complex cognitive
function. An active area of AD research is testing the relationship between
amyloid levels and cognitive function.
Neurofibrillary tangles are made up of another contaminated protein called
tau. Tau normally serves as connectors or “railroad ties” for a track-like
structure that transports nutrients and other important molecules throughout the
cell body of every neuron. The contaminated or tangled tau somehow becomes
hyperphosphorylated, a corruption resulting in several extra molecules of
phosphorous. Without the stabilizing railroad ties of normal tau, the tracks bend
into a twisted mess. Under the weight of the nutrients being transported, the
tracks buckle, and the damage increases. Inside the neuron, this twisted debris
gets worse as the filaments of track keep twisting around each other. As cell
communication and nourishment are lost, the neuron begins to wither. The cell
body, axons, and dendrites disintegrate, and as a result, thousands of synapses
vanish (Bear, Connors, & Paradiso, 2001; Shenk, 2003). It is well documented that the
brains of persons with AD have an abundance of the abnormal structures of beta-
amyloid plaques and neurofibrillary tangles (Shenk, 2003). Filamentous structures
known as “Hirano bodies” found primarily in the hippocampus are also
associated with the disease (Neugroschl, Kolevzon, Samuels, & Marin, 2004).
The structural changes just described do not cause AD but are the end
product of a pathological process. The challenge for researchers is to discover
what causes this degeneration. The loss of neurons occurs throughout the brain
but is significant in the cerebral cortex. The cerebral cortex is responsible for
higher brain functions, including thinking, judgment, reasoning, speech, and
language. Plaques and tangles are also dense in the hippocampus, which plays a
role in attention and memory (Cohen & Eisdorfer, 2001).
It is possible that amyloid has a function as a repair protein. Amyloid levels
have been noted to increase when there is some injury to the brain. Some
researchers contend that beta-amyloid plaques do not lead to AD but, in fact,
might be a by-product of the AD process (Shenk, 2003).
Genetic Predisposition of Alzheimer’s Disease

Unlike some diseases, AD is not caused by a single gene. Recent advances in
understanding the human genome combined with technologic advances in
methods of analysis have revealed new genes associated with AD risk. More
than one gene mutation is seen in AD, and multiple genetic loci associated with
increased risk have been identified over the past few years. The role of the innate
immune system was identified in 2013 by the discovery that rare variants in
TREM2, a microglial surface receptor, are associated with a significantly
increased risk of AD (Ryan, Rossor, & Fox, 2015). In most cases, genes alone are not
sufficient to cause AD. The two types of AD are early onset and late onset. Early
onset refers to AD that manifests before the age of 65, and late onset refers to
AD seen at or after age 65. Less than 5% of AD is early onset, and this form of
the disease is often inherited (Rocchi, Pellegrini, Siciliano, & Murri, 2003; U.S. Department
of Health and Human Services, 2004).
Three genes have been identified as responsible for the rare, early-onset,
familial form of AD. Mutated chromosome 21 causes an abnormal APP to be
produced. Mutated chromosome 14 causes an abnormal protein called presenilin
1 to be produced. Mutated chromosome 1 causes yet another abnormal protein,
presenilin 2, to be produced (National Institute on Aging, 2003). It appears that these
mutations increase the likelihood that beta-amyloid will be snipped from the
APP, causing more of the sticky beta-amyloid to be formed (Cohen & Eisdorfer, 2001).
Even if only one of these genes inherited from a parent is mutated, the person
will almost certainly develop early-onset AD. This means that in these families,
children have a 50% chance of developing early-onset AD if one parent has this
disease.
In the more common, late-onset AD, the apolipoprotein E genotype is the
strongest risk factor (Karch & Goate, 2015). Although the apolipoprotein E genotype
is equally common in men and women, it has a much stronger effect in women.
The Apolipoprotein E, or APOE allele is an example of a completely biological
factor that interacts with biological and gender-related factors such as hormones,
education, behavioral preferences, type of occupation, and physical activity and
increases the risk of AD (Rocca, Mielke, Vemuri, & Miller, 2014). The apolipoprotein E
gene is found on chromosome 19. This gene codes for a protein that helps carry
cholesterol in the bloodstream. The apolipoprotein E gene comes in several
forms, called alleles. The three most common alleles are apolipoprotein E 2,
apolipoprotein E 3, and apolipoprotein E 4. A person inherits one apolipoprotein
E allele from each parent. One or two copies of the 4 allele increases the risk of
getting AD. However, having the 4 allele does not mean that AD is certain, as
some persons with even two of the 4 allele do not develop the disease. The rarer
2 allele may be associated with having a lower risk for AD and later age at onset
(Blazer, Steffens, & Busse, 2004; Karch & Goate, 2015; U.S. Department of Health and
Human Services, 2004). AD may also be linked to mutations in the gene that
encodes for insulin-degrading enzyme, which helps to break down beta-amyloid.
Other genes related to AD include those involved in intracellular APP trafficking
and the creation of apolipoprotein neural receptors and transmembrane proteins
that influence calcium levels and beta-amyloid production (Minati et al., 2009).
Down’s syndrome is a risk factor for AD. Individuals with Down’s
syndrome carry an extra copy of chromosome 21, which contains the APP gene.
As a result, researchers believe that APP processing in neuronal membranes is
abnormal leading to the formation of beta-amyloid plaques and eventual AD
neuropathology. In fact, the brains of almost all individuals with Down’s
syndrome over the age of 40 show anatomical changes associated with AD, and
as many as 66% will develop clinically detectable dementia in their 50s (Beacher et
al., 2009). In another study, it was reported that 32.1% of a sample of individuals
with Down’s syndrome between the ages of 55 and 59 had dementia, 17.7%
between the ages of 50 and 54, and 8.9% between the ages of 45 and 49 (Coppus et
al., 2006). Due to preexisting learning disabilities, AD is more difficult to detect
and treat in this population (Beacher et al., 2009).
More recently, a possible link between environmental conditions in early
brain development and future manifestation of AD has been proposed. The
“Latent Early-life Associated Regulation” or “LEARn” model posits that factors
such as diet, toxin exposure, and hormones early in one’s development may
disrupt DNA structure in a manner that influences the way certain genes are
regulated. In support of this model, animal studies have shown that exposure to
lead early in life may upregulate the expression of APP in old age (Lahiri, Zawia,
Sambamurti, & Maloney, 2008; Wu et al., 2008).
Neurotransmitter Abnormalities in Alzheimer’s Disease

There are a number of neurotransmitters that are associated with AD. Most
changes are related to the glutamatergic system, with most changes in the
activation and expression of NMDA and the acetylcholinergic system. Changes
are also found in other central neurotransmitter systems including the
serotoninergic, dopaminergic, and noradrenalinergic systems. Changes in these
systems are most probably the result of global brain atrophy seen as the disease
progresses. The neurotransmitter acetylcholine is proposed to be responsible for
the cognitive symptoms of AD and is implicated in the progression of AD.
Neurons that contain acetylcholine are known as cholinergic neurons, and many
of these are bunched together to form tracts. Cholinergic tracts radiate
throughout the entire brain. Autopsy shows that for a person with AD, many
cholinergic tracts throughout the entire brain have been destroyed. The enzyme
choline acetyltransferase (CAT), which is needed to form acetylcholine, is
seriously reduced in the brains of persons with AD. This reduction of CAT is
greatest in areas of the brain where there is a dense amount of plaques and
tangles (U.S. Department of Health and Human Services, 2004). Although the
cognitive impairments of AD have been traditionally attributed to the
degeneration of cholinergic systems, alterations in the neuronal histaminergic
system have been increasingly recognized as contributing to the cognitive
impairments seen in AD (Zlomuzica et al., 2015). The neuronal histaminergic system
is an area where further research may lead to the development of more effective
drugs for the treatment of cognitive symptoms. Other neurotransmitter systems
found to be altered in people with AD include norepinephrine, GABA, and
glutamate (Neugrosch et al., 2004).
Cardiovascular Risk Factors in Alzheimer’s Disease

Although vascular dementia accounts for 15% to 25% of all dementia cases,
there is now a concern that cardiovascular disease is also linked to AD. As
people are living longer with AD, and living long enough to be diagnosed with
AD at age 80 and 90, these groups are also at risk for developing cardiovascular
disease. Some see this group as having a “mixed dementia,” or a combination of
AD and vascular disease (Cohen & Eisdorfer, 2001). Currently, the question is not
whether the two disease processes are mixed but whether the vessel damage is
causal or consequential to the degeneration of neural tissue (Stone, Johnstone,
Mitrofanis, & O’Rourke, 2014). Increasing evidence suggests the neuropathology and
cognitive decline of AD is caused by clinically silent bleeding from small
cerebral vessels. Studies have established that the presence of infarcts in eersons
with AD results in lower cognitive function, especially in the area of episodic
memory of specific experiences and events (Gore-lick et al, 2011). (U.S.
Department of Health and Human Services, 2004). In autopsy studies, 60% to
90% of AD cases exhibit variable cerebrovascular pathology (Kawas & Brookmeyer,
2001). Researchers have also found the use of statins, the most common type of
cholesterol-lowering drug, to be associated with a lower risk of developing AD

(Rocchi et al., 2003). Research has linked cases of familial AD with vessel damage
indicating that these dementias may possibly be forms of vascular dementia, as
well. Recent advance in the resolution of cerebral imaging has made it possible
for researchers to detect silent micro bleeds, which do most of the damage in
vascular dementia. The vulnerability of the brain to the pulse is linked to the
high metabolism of the brain. To enable the high volume of blood flow, the
resistance of the brain’s vessels is low making it vulnerable to the long-term beat
of the heart. Loss of elasticity, vascular remodeling, and arterial stiffness also
increase the intensity of the pulse and correlate with cognitive decline in aging.
Ways to protect the brain from the long-term effect of the pulse include
controlling blood pressure and diabetes and minimizing arteriosclerosis and
atherosclerosis.
Other Potential Causes of Alzheimer’s Disease

One of the theories of aging suggests that over time, damage from a kind of
molecule called a free radical can build up in neurons and cause a loss of
function. Free radicals can be helpful in fighting infection, but too many can
injure cells by changing other nearby molecules, such as those in the neuron’s
cell membrane or in DNA. This process can lead to a chain reaction, releasing
more free radicals and causing more damage. This is called oxidative damage
and may contribute to AD by upsetting the proper flow of substances in and out
of cells, by disrupting cell metabolism, and/or by interfering with other cell
processes such as protein synthesis, stress response, and enzyme-catalyzed
reactions (National Institute on Aging, 2003; Reed, Sultana, & Butterfield, 2010). Some
researchers are focusing on a possible connection between oxidative stress and
beta-amyloid, the principal component of plaques found in the brain of the
person with AD (Varadarajan, Yatin, & Aksenova, 2000). To support the role of oxidative
stress in contributing to AD, researchers have found that antioxidant defense is
altered in the brains of persons with mild cognitive impairment (MCI), a
condition that is often a precursor to AD (Reed et al., 2010).
Researchers are also studying the possible role of inflammation, because
cells and compounds that are known to be involved in inflammation are found in
AD plaques. Some scientists think that inflammation is harmful and sets off a
cycle of events that lead to the death of neurons. Others believe that some
aspects of the inflammatory process may be a helpful part of the brain’s natural
healing efforts (National Institute on Aging, 2003). Research is focused on even more
factors that may be related to AD, including trace elements, the role of the
immune system, and the possibility of a viral connection. The role of numerous
misfolded proteins in the neuropathology of AD along with other
neurodegenerative diseases such as Parkinson’s disease, Huntington’s disease,
and amyotrophic lateral sclerosis (ALS/Lou Gehrig’s disease) is also a topic of
scientific inquiry (Kanehisa, Limviphuvadh, & Tanabe, 2010).
Incidence and Prevalence of Alzheimer’s Disease
It is estimated that there are 5.3 million Americans with AD. While most persons
with AD are 65 or older, there are about 200,000 individuals under age 65 who
have younger-onset AD (Alzheimer’s Association, 2015). According to population-
based studies, 600,000 Americans with AD died in 2010, and in 2011, it was the
sixth leading cause of death (Mitchell, 2015). With the aging of the baby boomer
population and medical advances, it is predicted that by 2050, there will be 18
million Americans with NCD; within this group, 14 million will have NCD due
to AD. AD increases in prevalence with increasing age, and women tend to have
a higher incidence of AD. For persons age 85, 11% of men and 14% of women
have AD. At age 90, the rate increases to 21% for men and 25% for women.
While AD is the most common form of NCD, approximately 10% to 15% of
persons with AD have a coexisting vascular dementia (Sadock et al., 2014).
In addition to age and gender, there are numerous factors that appear to
create a higher risk for AD (Hales & Yudofsky, 2003; Neugrosch et al., 2004; Reitz et al., 2010;
Shah et al., 2009). These factors include the following:
1. Low educational level

2. History of head trauma with loss of consciousness 3. History of depression
4. Late maternal age
5. Environmental and occupational exposure (e.g., to aluminum) 6. History of
electroconvulsive therapy (ECT) 7. Alcohol abuse
8. Analgesic abuse 9. Long-standing physical inactivity 10. Vascular risk
factors (e.g., hypertension, high cholesterol) 11. Type II diabetes
12. Black or Hispanic ethnicity
13. High waist-to-hip ratio
Minati et al. state that TBI is increasingly believed to be a risk factor for the
development of AD due to the accumulation of beta-amyloid and tau pathology
that follows neural degeneration. They also indicate that cerebrovascular
abnormalities may increase the chances of being diagnosed with AD. However,
they assert that the most important risk factors are advanced age and genetic
predisposition (Minati et al., 2009).
Related to these risk factors, there also appear to be several variables that
may reduce the risk of developing AD (Breitner & Albert, 2009; Neugrosch et al., 2004; Shah
et al., 2009). These include the following:
1. Exposure to antioxidants (such as vitamin E) 2. Regular consumption of

fish
3. Estrogen replacement therapy in postmenopausal women 4. Use of
nonsteroidal anti-inflammatory drugs (NSAIDs) 5. Use of specific
antihypertensive drugs 6. Use of statins
7. Use of specific histamine blockers 8. Physical activity
9. Exposure to education, cognitive training, and mental stimulation
However, the effectiveness of some of these agents is still a matter of scientific
debate.
Signs and Symptoms of Alzheimer’s Disease
The signs and symptoms of NCD due to AD include a range of cognitive
functions: attention, executive function, learning and memory, language,
perceptual motor skills, and social cognition. These symptoms impact virtually
all performance skills. Since AD is a progressive disease, the first signs can be
mistaken for normal aging. Even though there are small changes in process skills
with normal aging, a healthy older adult can continue to learn and problem-solve
at any age. Researchers are generating new information on normal aging versus
mild NCD. A person with mild NCD will have more impairment of process
skills than is expected with normal aging but will not yet meet the diagnostic
criteria for major NCD due to AD.
Defining the progressive stages of AD is an arbitrary process, and various
researchers use different ideologies. Reisberg, a prominent researcher in the
study of AD, has proposed seven stages (Reisberg & Sclan, 1992). These stages range
from stage 1, in which there is no impairment, to stage 7, which is very severe
decline. Most researchers use a three-stage approach (Goldman, Wise, & Brody, 2004;
Samuels & Neugroschl, 2004; Shenk, 2003). The Clinical Dementia Rating (CDR) is used
to measure dementia (Hughes, Berg, & Danziger, 1993). This scale defines three levels
of dementia or NCD as mild, moderate, and severe.
This chapter will use the three-stage approach, which correlates with the
DSM-5’s levels of severity for NCDs: mild, moderate, and severe. The first
stage, or early stage, is a mild NCD and is what used to be referred to as mild
cognitive disorder. The second or middle stage occurs at the onset of major
NCD, with a moderate level of severity. The late stage is severe major NCD. It is
important to remember that each person with AD is a unique individual, with
unique concerns, needs, and strengths. Although such individuals do not always
fit into a rigid classification system, using the three stages will give the reader
general guidelines for better understanding the problems facing the person with
AD and his or her caregivers.
Course and Prognosis of Alzheimer’s Disease
Mild Stage
For mild NCD due to AD, the early or mild stage usually lasts 2 to 3 years
(Gauthier, 2002). The cognitive changes in the mild stage require compensatory
strategies and accommodations for the individual to maintain independence. The
person who recognizes that his or her memory is impaired may be better able to
make necessary adaptations. Eventually all cognitive domains are impacted in
the early or mild stage.
Complex Attention
The person takes longer to perform familiar tasks and begins making mistakes. It
becomes difficult to do two things at once, such as talk while driving or cook
while following a recipe.
Executive Function
The person in the mild stage of AD begins to have difficulty with IADLs. Bills
may be forgotten, and having automatic bill payment becomes a necessary
strategy. Medication management may need to be monitored by a caregiver.
Problems with planning, organizing, sequencing, and abstracting become
apparent as the person begins to have difficulty in the workplace or following
written directions such as a recipe (Hales & Yudofsky, 2003). For example, the person
may begin to prepare a meal, become distracted, and forget to complete it; in
another example, the person preparing food might be unable to properly
sequence the steps or leave out important ingredients.
Learning and Memory

Short-term memory is significantly impaired, which makes new learning very
difficult. The person forgets tasks, loses the thread of a conversation, and
misplaces things. Long-term memory begins to be impaired (Hales & Yudofsky,
2003). The person might be able to remember a phone number long enough to
repeat it but will forget it if there is any delay in using it. Procedural memory,
such as knowing how to write, will remain intact. However, other types of
memory begin to show deficits including personal episodic memory, which is
time-related information about one’s self, such as where and if one ate yesterday
evening; semantic memory, such as remembering the name of a common
object; and general knowledge, such as remembering the name of the highest
mountain in the world.
Language.
Aphasia, an abnormal neurological condition in which language is impaired,
appears in the mild stage. The person can usually maintain sentence structure,
though it becomes less complex. Poor semantic memory leads to difficulty with
word retrieval (the ability to name an object when shown a picture of it) and
word list generation (e.g., being able to quickly name words in a common
category or beginning with the same letter). When forgetting a word, the person
may substitute inappropriate words, making sentences incomprehensible. This
would be an example of paraphasia, which means saying the wrong word,
substituting a word that sounds alike, or using a word in the same category as the
intended word. Anomia is the phenomenon in which a person searches in vain
for a word and says “thingamajig” or just gives up. Circumlocution may occur,
in which the person tries to express an idea by talking around the intended word
with extensive description and elaboration (Liu, McDowd, & Lin, 2004).
Perceptual-Motor Abilities
Visuospatial abilities decline in the mild or early stage of AD. The person starts
to have difficulty with topographical orientation, getting lost in a familiar
neighborhood or failing to recognize a familiar intersection. There may be some
disorientation within the home, such as putting the frying pan in the freezer or
the wallet in the dishwasher (Bear et al., 2001).
Social Cognition
It is not unusual to see personality changes. The family notices that the person is
more rigid and irritable and less spontaneous or adventurous. Some persons with
AD become suspicious, thinking that one’s things are being stolen or that his or
her spouse is being unfaithful. The person may become less attuned to body
language and seem inconsiderate. Depression is seen in up to 25% of people in
the mild stage of AD. Depression or anxiety may lead to withdrawal from
routine social activity. In some cases, delusional thinking may emerge (Kovach,
1997).
Moderate Stage
In the middle or moderate stage of AD, which can last from 2 to 10 years, there
is continued decline across all neurocognitive domains. While a person in the
mild stage of AD may be able to live independently, by the moderate stage there
is a severe impact on function. All areas of performance skills begin to show
deficits, psychiatric symptoms increase, and behavior disturbances arise.
Complex Attention
There is difficulty with maintaining attention in environments with multiple
stimuli such as loud noises and bright or changing light. Attention cannot be
divided between two tasks, such as walking and talking simultaneously. The
ability to focus attention for a length of time will be reduced, with the need for
prompts or redirection several times a minute.
Executive Function
The person in the moderate stage of AD is no longer able to problem-solve. The
ability to plan, weigh consequences, consider alternative strategies, and judge
outcomes is lost. The caregiver, who is now responsible for complex tasks and
problem solving, may be able to guide and prompt the person with AD through
some of the steps as a way to keep him or her engaged in meaningful tasks.
Learning and Memory

Recent and remote memory worsens. The person may think he or she is back at
an earlier stage of life and become focused on a past worry, such as getting the
children to school on time. The person becomes less bothered by the memory
loss. There is disorientation to place and time, and in the course of decline, the
person may not recognize his or her own face in the mirror, much less friends, or
family members a condition known as prosopagnosia. New information is not
retained for more than a few moments. There is difficulty organizing thoughts
and thinking logically, and inability to cope with new or unexpected situations.
Thinking is concrete, with no ability to take into account ideas or objects that are
not present (National Institute on Aging, 2003; Shenk, 2003; Zgola, 1999).
Language
There are increased symptoms of aphasia, and the person loses fluent language.
Language is limited to the concerns of the moment or reminiscing about the past.
There may be diminished verbal responsiveness, or verbalizations may be
impulsive and inappropriate. There is difficulty understanding simple questions
or instructions. The person has trouble following a conversation and may be
unable to keep track of his or her own thoughts or words (Frank, 2003). Internal
speech is part of executive function and is necessary for an individual to make
plans or mentally problem-solve. A person who can no longer use words
effectively loses this ability of rational planning. His or her actions will become
impulsive and disorganized, requiring step-by-step direction and supervision for
any occupation (Zgola, 1999).
Perceptual-Motor Skills
Visuospatial abilities continue to decline. Visual inattention, which is seen in the
mild stage, begins to seriously limit function in the moderate stage (Liu et al., 2004).
The person gets lost in familiar environments, and is unable to become oriented
if he or she moves to a new environment. Constructional skills are compromised,
and the person is unable to sort out the arms and legs of garments while trying to
get dressed. There is a loss of ability to judge depth and distance. The person
may step highly over a mark on the ground or choose to walk around it. He or
she may not be able to distinguish furniture from designs on the carpet or
interpret changes in flooring. These visuospatial impairments can lead to falls.
Judging direction and distance is problematic, resulting in knocking things over
when trying to grasp them or grasping at thin air (Zgola, 1999).
Social Cognition
There is little or no awareness of social standards, as manifested by immodest
dress or inappropriate topics of conversation. The person will not be able to
consider the needs of a minor or a pet. He or she will need the caregiver to assist
with community participation such as doctor appointments, shopping, and
restaurant outings.
Psychiatric Symptoms
Psychiatric symptoms that emerged in the mild stage worsen. Depression and
anxiety are frequently seen, and the presence of depression is associated with
increased mortality. The person may lose control over his or her emotions,
having outbursts of fear or anger. Visual hallucinations are not uncommon, and
auditory hallucinations can also occur. Sleep is disturbed, with increased daytime
napping and frequent nighttime wakefulness (Kovach, 1997).
Behavior Disturbances
Behavior disturbances increase the likelihood that the individual will need
nursing home placement. It is in the moderate stage of AD that wandering and
agitation become a problem (National Institute on Aging, 2003). The person paces,
seemingly without a goal or destination. Pacing could be a sign of stress or
anxiety, or perhaps it is the person’s need for activity and exercise. Agitation
could be the person’s only way to respond to fear or frustration (Kovach, 1997).
Loss of impulse control manifests in sloppy table manners, undressing at
inappropriate times or places, and vulgar or rude language (Vickers et al., 2000).
There is a loss of social propriety and disinhibition, a failure of the filtering
system that determines which thoughts to keep to one’s self and which thoughts
to act upon. This inability to inhibit impulses can cause offense to others or
become dangerous if the person with AD acts upon aggressive impulses (Zgola,
1999).
Severe Stage
The severe or late stage of AD can last as long as 8 or more years. At this stage,
the person with AD is fully dependent on others for basic ADLs, such as
bathing, dressing, and eating. Motor skills are affected, and the person eventually
becomes immobile and incontinent.
Complex Attention
Attention is now measured in seconds, and the caregiver will need to give
frequent but simple cues for performing simple actions such as eating or lifting
an arm during dressing. Awareness of the environment is very limited. The
person in the severe stage of AD eventually may only respond to pain, hunger,
and fear.
Executive Function
All problem-solving and planning skills are seriously impaired, and purposeful,
goal-directed occupation is lost.
Learning and Memory

There is no ability to create new memories and little or no recognition of close
family members. Despite these deficits, it is still important to bring into the
environment personal and meaningful objects such as mementos and family
pictures.
Language
Speech is limited to one or two words, or speech and vocabulary may be totally
unintelligible. Over time, speech will decline to the point where it is lost. The
person no longer smiles or communicates with facial expression (Shenk, 2003).
There can be instances of moaning or crying, and since these are universal
sounds of distress, it is important for caregivers to explore the possible causes of
discomfort. Receptive language is also seriously impaired. The person cannot
process the meaning of words but may respond to a calm and soothing tone of
voice or touch.
Perceptual-Motor Skills
In the severe stage of AD, if the person attempts to ambulate, he or she will be at
risk for falling. Neurological symptoms develop, including hyperreflexia,
apraxic gait, and frontal release signs (grasp and snout reflexes). Eventually, the
person becomes bedbound with loss of postural control. Paratonia is a primitive
reflex in which there is involuntary resistance in an extremity in response to a
sudden passive movement. Thus, if a caregiver quickly moves the person’s arm,
he or she automatically resists the movement (Goldman et al., 2004). Seizures may
occur, and contractures, pressure ulcers, urinary tract infections, and pneumonia
may develop from immobility. There is incontinence of bladder and bowel.
Appetite decreases, and eventually, the person develops dysphagia, or the loss
of ability to chew and swallow (Kovach, 1997). The person begins to experience
agnosia, having difficulty recognizing objects and familiar people.
Social Cognition
Social interaction is very limited. With a few words, and by taking the person’s
hands, a family member or caregiver may be able to capture the person’s
attention momentarily. Despite the loss of productive communication, the person
may benefit from gentle hugs and caresses from a loved one.
Psychiatric Symptoms
The sleep cycle is very disturbed as the person spends 60% of time sleeping,
including much of the daytime. Hallucinations persist for some (Kovach, 1997).
The course of AD tends to be slowly progressive, with the loss of about 3 points
per year on the Mini Mental State Examination (MMSE), a standard cognitive
assessment instrument (Neugrosch et al., 2004). The stages outlined in the previous
section of this chapter illustrate the progression of the signs and symptoms of
AD. A 2004 study reported the average survival time following diagnosis of AD
to be 4 to 6 years. On average, those who are newly diagnosed live
approximately half as long as do people of the same age without AD. However,
some people can survive as long as 20 years after the first appearance of
symptoms (Alzheimer’s Association, 2009; Varadarajan et al., 2000).
AD progresses until death. Death is usually a result of complications, such as
infection, or the eventual inability of the individual to maintain nutrition and
hydration. Despite suspected underreporting, AD is the 6th leading cause of
death in the United States (Alzheimer’s Association, 2015).
Diagnosis of NCD
There are four primary criteria in the diagnosis of NCDs. First, there is a decline
in performance in one or more of the cognitive domains. These cognitive
domains include complex attention, executive function, learning and memory,
language, perceptual-motor abilities, and social cognition (see Table 11.1)
(American Occupational Therapy Association, 2014; American Psychiatric Association, 2013). If there
is a mild decline in performance, the diagnosis will be a mild NCD; if there is a
significant decline in cognition, the diagnosis will be a major NCD. The
diagnostician determines that there is a decline in performance based on 1.
Concern of the individual or the observation of someone who knows the
individual, and 2. Cognitive impairment documented by standardized
neuropsychological testing, or other quantified clinical assessment (American
Psychiatric Association, 2013).
The second criterion for NCD is the finding that cognitive deficits cause
some degree of impairment in occupational functioning. For a mild NCD, the
cognitive deficits do not necessarily interfere with independent living. However,
the individual will begin to have difficulty with IADLs, especially those
requiring executive function such as financial or medication management. The
person may be able to use compensatory strategies or may begin to need some
assistance. Routine ADLs remain intact. For a major NCD, the cognitive deficits
interfere with independent living; the individual loses the ability to manage
IADLs and will need assistance or total care for ADLs.
The third criterion specifies that delirium cannot be the only cause of the
cognitive impairment. The fourth criterion rules out other disorders, such as
depression or schizophrenia as the cause of the cognitive impairment (American
Psychiatric Association, 2013). AD is not the only type of NCD. In fact, the diagnosis of
mild or major NCD includes a specifier for each type of NCD. These specifiers
include AD, frontotemporal lobar degeneration, Lewy body disease, vascular
disease, TBI, substance/medication use, HIV infection, prion disease,
Parkinson’s disease, Huntington’s disease, other medical condition, multiple
etiologies, and unspecified (American Psychiatric Association, 2013).
The diagnosis is a time-consuming process, but an important one, as the
thorough medical investigation may reveal a condition that can be treated. The
possibility of reversible dementia will be discussed later.
The National Institutes of Health (NIH) (National Institute on Aging, 2003) has
outlined the following steps in diagnosing AD:
1. A detailed patient history should include a description of how and when the
symptoms developed, the patient’s and family’s medical condition and
medical history, and an assessment of the patient’s emotional state and
living environment.
2. Interviewing family members or close friends, which can provide
information on how behavior and personality have changed.
3. Physical and neurological examinations and laboratory tests, which help
determine neurological functioning and identify possible non-AD causes of
dementia.
4. A computerized tomography (CT) scan or a magnetic resonance
imaging (MRI) test, which can reveal changes in the brain’s structure and
function that indicate mild or early AD.
5. Neuropsychological tests that measure memory, language skills, and other
cognitive functions, which help indicate what kind of cognitive changes are
occurring.
Medical Management
General Treatment Approach
Goldman et al. (2004) describes the following four pillars of complete dementia care:
1. Supportive care for the patient

2. Supportive care for the family and/or caregiver 3. Disease treatment
4. Symptom treatment, including cognitive, mental, and behavioral symptoms
Supportive care includes assessing the environment for aspects of safety, as well
as looking at how the environment can improve function and well-being. Very
early in the disease process, the person with AD needs to discuss financial and
medical concerns such as wills and advance directives. It is important to
determine the level of care that is needed, who will provide the care, and
additional supports such as day programs. The family may need to help make
decisions regarding ability to drive and work. Maintaining the person’s dignity
and privacy is always important. The person with AD will also need ongoing
medical care and evaluation of vision and hearing.
Minati et al. (2009) summarizes a number of evidence-based interventions that
may be helpful in the treatment of persons with AD. These include individual-
and group-based structured activities, psychological therapy, and cognitive
rehabilitation. Cognitive strategies based on implicit learning include spaced
retrieval, vanishing cues, and errorless learning. However, it is important to note
that this type of “learning” depends on implicit aspects of cognition not as
rapidly affected by the disease; such treatments should be used only to the extent
that they are therapeutic for the individual. Other cognitive interventions include
cognitive stimulation, reminiscence therapies, and external memory aids. These
authors assert that “person-centered therapeutic interventions” are helpful in
addressing emotional issues that underlie much of the problem behavior and
distress associated with AD for both the patient and caregiver (Minati, 2009).
Support for the caregiver is critical. Approximately 80% of people with
dementia are cared for by their families in the community (Neugrosch et al., 2004).
Caring for someone who is progressively losing memories and skills can be
stressful, sad, and frightening. Caregiving can become a 24-hour-a-day job.
Complicating the stress of caring for a person with AD is the tendency of
caregivers to withhold the diagnosis from friends and family and the social
isolation that may result from embarrassing behavior or functional decline
(Neugrosch et al., 2004). For caregivers, the stress of caregiving leads to increased
risk of depression, sleep disturbance, and substance abuse and can indirectly
result in patient neglect (Neugrosch et al., 2004). The Alzheimer’s Association can
refer caregivers for support, resources, and advocacy.
Disease treatment is meant to target the etiology or cause of the progressive
decline in AD. The etiology of AD is still unknown. Former President Ronald
Reagan brought national attention to the disorder when, in 1994, he announced
that he had been diagnosed with AD. Following his death, his widow, Nancy
Reagan, publicly campaigned for increasing dementia research funds and for
limiting obstacles to stem cell research. Until the causes of AD are better
understood, medical management of the vascular process continues to be
important not just for persons with vascular dementia but also for AD.
Symptom treatment targets cognitive decline, psychiatric symptoms
(including psychosis or depression), and behavior disturbances. As the disease
progressed and the symptoms become more distressing, it is imperative for the
caregiver to understand the reasons for behavior changes and finds ways to
support the individual with AD while keeping him or her safe. The occupational
therapist has an important role in helping the caregiver to consider the individual
as a whole person, to explore environmental stressors and adaptations, and to
discover productive ways to perform occupations. Occupation-based symptoms
management will be discussed in the next section.
Pharmacotherapy can also be useful for some symptoms. The use of
medications will not stop the disease process, but relieving the associated
symptoms can comfort the person with AD and improve the quality of life for
both patient and caregivers. Pharmacotherapy strategies will also be discussed.
Behavioral and Environmental Management

According to Hilgeman, Burgio, and Allen (2009), the environment surrounding a person
with AD should be modified so as to restore the balance between the challenges
presented and the person's current level of function. By creating an appropriate
level of demand, the physical environment can serve to help maintain function
and simultaneously provide stimulation and comfort to the individual with AD.
Environmental considerations should be made for declining cognitive function
as well as sensory loss and the potential for pain and discomfort. Some specific
suggestions from Hilgeman et al. (2009) include
1. Reducing the size of space and number of individuals 2. Simplifying visual
and auditory stimuli 3. Providing choices/options
4. Increasing the amount of familiar and meaningful stimuli 5. Providing
redundant cues in multiple modalities 6. Increasing the sensory contrast of
important stimuli
An appropriate environment is perhaps one of the most effective ways to manage
problem behaviors of persons with AD. Hilgeman et al. (2009) point out that
aggression is often a result of too much or too little stimulation, the anticipation
or experience of pain, and frustration with one's inability to perform daily tasks
or effectively communicate. They suggest breaking up long periods of low or
high arousal activities with sensory stimulation or relaxation, providing orienting
cues, emphasizing nonverbal communication, and working to relieve immediate
discomforts. Addressing other underlying factors such as pain and depression
may also help ameliorate agitated behavior. Neugrosch et al. (2004) add that
infections, constipation, delirium, and medication or substance intoxication may
also lead to agitated behavior in persons with AD. They suggest strategies such
as promoting exercise, socialization, recreation, and predictable routines.
Another way to address troublesome behavior, such as agitation, is to consider
the "ABCs" (Neugrosch et al., 2004; Xie et al., 2009). This refers to the antecedents, the
nature of the behavior itself, and the consequences. By considering factors that
frequently lead up to the behavior, changes can be made to try to avoid the issue
before it begins. Some changes may involve simplifying the task, providing
different cues, or offering choices. The consequences of behavior should also be
considered so that uncooperative behavior, such as acting out in avoidance of a
task, is not reinforced, and positive behavior is not punished or ignored.
The DICE approach is an occupation-based method of interpreting and
managing symptomatic behavior for persons with major NCD (Fraker, Kales, Blazek,
Kavanaugh, & Gitlin, 2014). The occupational therapist or other health professional
guides the caregiver through the four steps of DICE: Describe, Investigate,
Create, and Evaluate. Using the example of difficult behavior during bathing (the
patient strikes out at the caregiver), the therapist first asks the caregiver to
“describe” in detail the context (who, what, when, how); the social and physical
environment; the perspective of the patient as much as possible; and the degree
of distress felt by both caregiver and the patient. In the second step,
“investigate,” the therapist explores other possible factors, such as the patient’s
pain, medications, medical conditions, sleep, and sensory factors. The therapist
also explores the caregiver’s expectations and social and cultural factors. In the
third step, “create,” the therapist collaborates with the caregiver to create
strategies to enhance communication, simplify the task, modify the environment
and stimuli, and create meaningful activities for the patient. The therapist may
need to educate the caregiver in how much support is needed for the patient to be
successful. After the caregiver implements the strategies, the last step is
“evaluate.” In this essential step, the therapist and caregiver review the
outcomes, determine which strategies were safe and effective, and if other
strategies need exploration. In the case of a patient becoming combative during
bathing, strategies such as warming the bathroom, undressing and sponge
bathing only part of the body at a time, using favorite scents, and timing the bath
when the patient is most relaxed are examples of strategies that may be
productive.
Patients with AD may engage in a variety of problematic behaviors other
than or in addition to aggression and agitation. Some examples include
wandering, grabbing others, and swallowing inedible objects. A helpful guide for
dealing with these behaviors based on Allen's Cognitive Disability Model and
the 25 cognitive levels and modes is written by Pollard (2005) and listed as a
recommended learning resource at the end of the chapter.
Pharmacotherapy
Prescription drugs are frequently used in managing symptoms such as impaired
cognition, depression, delusions and hallucinations, and agitation or aggression.
Medications commonly used in treating cognitive symptoms work as
cholinesterase inhibitors. These include donepezil (Aricept), rivastigmine
(Exelon), and galantamine (Reminyl). Inhibiting acetylcholinesterase leaves
more acetylcholine in the synapse, which facilitates the activity of remaining
neurons. The various cholinesterase inhibitors tend to perform equally in
comparison group studies, but it is possible for individuals to respond more
favorably to one drug than another (Farlow & Boustani, 2009). In a study of patients in
the mild stage of AD, those given cholinesterase inhibitors were treated with less
antipsychotics and less anxiolytics than those not taking cholinesterase inhibitors
(Fereshtehnejad, Johnell, & Eriksdotter, 2014). These medications do not inhibit cell loss,
but research does show that there is improvement in cognitive function,
behavioral symptoms, and ADL (Wang et al., 2014). This improvement seen in
increased function in ADLs also results in delayed placement in nursing homes
(Peterson, Stevens, & Ganguli, 2001). Some studies show that treatment with
cholinesterase inhibitors can restore cognition to the level seen about 6 months
prior to the start of treatment (Liu et al., 2004). This means that the person may be
able to again do the things he or she could do half a year ago, such as dressing
with verbal prompts only. However, these treatments cannot halt the progression
of AD, and cognitive decline will continue. Common side effects of
cholinesterase inhibitors include nausea, vomiting, diarrhea, and loss of appetite
(Farlow & Boustani, 2009).
Memantine (Namenda) is another medication used in an attempt to slow the
progression of AD. It works by blocking excess glutamate, a chemical involved
in memory function (National Institute on Aging, 2003). Memantine has few long-term
side effects but may cause temporary confusion or sedation while the dosing is
being adjusted. Memantine by itself does not enhance cognitive function (Wang et
al., 2014). A combination of memantine and a cholinesterase inhibitor is the
preferred treatment for moderate to severe AD (Fan & Chiu, 2014). However, as AD
progresses and the person is no longer able to share meaningful interactions,
medication may be discontinued (Farlow & Boustani, 2009).
At the present, pharmacological advances in the treatment of AD include
large-scale clinical trials of an antihistamine drug called Dimebon (latrepirdine).
This agent appears to have mitochondrial stabilization properties that promote
neuronal function and inhibit cell death, leading to improved cognition in people
with AD. Investigators are also exploring drug treatments that target amyloid-
beta production and aggregation, but so far, success has been minimal (Massoud &
Gauthier, 2010). Fan and Chiu (2014) assert that a crucial drawback in researching AD
drugs is that the patients do not receive treatments early enough in the
progression of NCD. For treatments to be most effective, patients, families,
and/or clinicians must recognize the early signs and symptoms of AD and begin
the diagnostic and treatment process.
Depression is seen in as many as 25% to 30% of persons with dementia.
Depression can occur in the early stage of AD and, if untreated, can lead to
earlier institutionalization and death, aside from the emotional suffering. Select
serotonin reuptake inhibitors (SSRIs) are the first line of treatment for
depression, followed by the use of agents with dual effects on the serotonin and
norepinephrine systems (Peterson et al., 2001). Many SSRIs have anticholinergic side
effects, so fluoxetine (Prozac) and sertraline (Zoloft) are usually most
appropriate for individuals with dementia (Farlow & Boustani, 2009). The
accumulation of research evidence suggests that antidepressants should be used
only after nonpharmacologic approaches have been tried, with the exception of
severe depression with suicidal symptoms (Leong, 2014).
Delusions and hallucinations can begin in the mild stage of AD but are more
common in the moderate stage. Low-dose neuroleptics are sometimes used, and
the atypical neuroleptics such as risperidone (Risperdal) and olanzapine
(Zyprexa) are less likely to cause side effects than the typical antipsychotic
medications (Peterson et al., 2001). Corbett (2014) argues that risperidone is the only
neuroleptic that should be used for persons with AD, and only in cases of a
preexisting psychosis or severe aggression. However, the use of these
medications is controversial due to increased risk of stroke and death as well as
only limited, if any, long-term improvements (Ballard, Corbett, Chitramohan, & Aarsland,
2009; Corbett, Burns, & Ballard, 2014; Kales et al., 2012; Minati et al., 2009).
Disruptive behavior such as agitation and aggression is often a sign of a

medical problem. Urinary tract infections or pneumonia can create delirium with
agitation or aggression. Ruling out these and other concerns, such as
environmental stress or pain, is the first step in reducing problem behaviors. If
the behavior is truly caused by the underlying dementia, low-dose neuroleptics,
such as risperidone and olanzapine, and the antidepressant aripiprazole may be
beneficial (Gallagher & Hermann, 2015). However, the use of neuroleptics in this
situation may not be the best approach. Psychological interventions and
caregiver training should be the first management strategies used, prior to
pharmaceutical interventions (Ballard et al., 2009; Corbett et al., 2014; Madhusoodanan & Ting,
2014).
Mild Stage
With the progressive loss of performance skills, AD affects increasing areas of
occupation and performance patterns. Although this section outlines the
expected impact of AD on client factors, it is important to remember that each
individual will perform differently. Someone who has always been flexible and
adaptive may compensate in ways that support function; another person who is
prone to anxiety or has fewer coping skills might have more significant
functional losses.
In the mild stage, ADLs remain intact. The first signs of memory impairment
manifest in IADLs. The person experiencing neurocognitive deficits cannot
adequately or safely fulfill the responsibilities of child-rearing or caring for
others. When orientation to place is impaired, mobility within the community
may no longer be safe. The person easily becomes lost in a new environment.
The person with mild AD will be disoriented while vacationing or traveling
away from home, and sometimes, this is when family members begin to note the
symptoms of AD. Financial management begins to deteriorate. The person may
forget to pay a bill or may misplace it. Shopping becomes a problem when the
person is disoriented, loses track of what he or she is trying to purchase, and has
difficulty making the money transactions. Compensations can include shopping
only in a familiar neighborhood and using a credit or debit card.
Other IADLs may also be impacted. Meal preparation can be a problem if
the person starts the task, gets distracted, and then forgets to finish preparing and
even eating the meal. Health management can be a problem if the person forgets
to make or keep appointments or forgets to take medication. Home management
may not be a problem if there are well-established routines and no unexpected
problems arise. However, routine car maintenance might be neglected, and
response to problems and emergencies might be less organized.
The ability to drive is an IADL that deserves special attention. As a general
rule, persons with mild stage dementia who wish to continue to drive should
have their driving skills evaluated. Many states offer driving assessments
through their state departments. The Family Caregiver Alliance (2002) advises families
to observe for behavioral signs that the person with dementia is no longer able to
drive with safety. It is possible to determine a person is no longer safe to drive
when he or she:
1. Has become less coordinated
2. Has difficulty judging distance and space 3. Gets lost or feels disoriented in
familiar places 4. Has difficulty engaging in multiple tasks 5. Has increased
memory loss, especially for recent events 6. Is less alert to things happening
around him or her 7. Has mood swings, confusion, or irritability 8. Needs
prompting for personal care 9. Has difficulty processing information 10.
Has difficulty with decision making and problem solving
Memory loss impacts performance in education. Learning becomes very
difficult, if not impossible. Reading is problematic. The person is likely able to
read but will have difficulty retaining or remembering what is read. Work is also
seriously impacted. Tasks that are routine may remain intact, but poor attention
will impact the ability to get work started, attend to all details, and follow the
task through to completion. As job performance deteriorates, relationships with
co-workers and supervisors can become strained. Some employers make efforts
to arrange workloads and expectations in order to maintain the worker in
employment as long as possible. In other cases, the person in the mild stage of
AD may have to seek early or medical retirement. The person will likely need
assistance in determining benefits and making plans for productive retirement.
Volunteer exploration and participation will be difficult. New routines are
difficult to establish, and the task demands, as with regular employment, may be
too high for the person with memory and other cognitive impairments.
Leisure exploration will be difficult for the same reasons. Learning new
tasks or routines, even for leisure, will be very difficult. The person might be
able to maintain established leisure patterns that have little demand for problem
solving. Other activities may be given up; attending the bridge club becomes
stressful instead of relaxing when it is hard to remember your partner’s name or
what cards were just played.
The person with mild stage AD will find social participation no longer easy
or enjoyable. The family begins to notice that the person avoids social contact, is
rigid or irritable, and is no longer spontaneous. He or she may drop out of
community and family events, or attend without actually participating. The
changed behavior could be a result of depression, deteriorating language skills,
or fear of embarrassment. For the same reasons, intimacy and sexual expression
will be diminished.
In the mild stage, motor skills will be unaffected. As cognition declines, the
process skills of temporal organization are impacted. It is easy to be distracted in
the middle of a task and then forget to continue, sequence, and properly
terminate it. If the task is new or unfamiliar, adaptation may be too demanding.
Communication and interaction skills are impaired as language problems
develop, and the person may no longer be able to clearly articulate thoughts and
needs.
Performance patterns begin to deteriorate. The person might rigidly cling to
habits or routines as he or she becomes aware of, and attempts to compensate
for, memory loss. Another person may begin to neglect habits and routines; the
garbage is not taken out, or the person stops going to church. Roles begin to
change: a grandmother can no longer baby-sit but needs help to go shopping; a
father can no longer help his family do the taxes, even though he worked all of
his life as an accountant.
Cultural and spiritual contexts remain strong, but participation begins to
decline as a result of memory impairment and communication difficulties.
Family and friends need to provide ever-increasing support to ensure the person
is included in family, cultural, and spiritual events.
Client factors that are impacted in the mild stage are the mental functions:
memory, orientation, perception, higher-level cognition, mental functions of
language, and calculation. Self-concept will be impacted as the individual fears
embarrassment or worries that his or her competence is declining.
Moderate Stage
In the moderate stage of AD, there is impairment in all areas of occupation, and
the person can no longer live alone. ADLs are not always attended to, and, when
attempted, performance may be poor or inadequate. The person may attempt to
shower but have difficulty setting the water temperature. Showering may consist
of just getting wet, with no attention to the need for soap or shampoo. If
shampoo is used, regulation is poor, with too much or too little used, using it on
the top of the head only or forgetting to rinse. Dressing requires decision making
such as choosing attire based on weather and occasion and the sequence of
donning each article. The person with AD might make mistakes in these
decisions, as well as in understanding the need to remove dirty clothing and
replace with clean. Although the person begins misplacing eyeglasses and
dentures in the mild stage, by now, these articles might be lost or even unnoticed.
Toilet hygiene is no longer productive as the person may neglect to clean the
body or properly refasten clothing. Eating difficulties and weight loss typically
begin in the moderate stage of AD (Neugrosch et al., 2004). The sleep-wake cycle is
often disturbed, and wandering in the night can create a crisis for caregivers.
IADLs are neglected or performed without proper sequence and completion.
Home management tasks that are repetitive, such as folding towels, can be
performed after someone else has sorted, laundered, and dried them. The person
is dependent in areas of community mobility, financial management, and
shopping. Some cleaning and cooking tasks may be done with supervision and
direction. Safety is a major concern for the person in the moderate stage of AD.
At first, he or she may be safe if left home alone for an hour. As the symptoms
progress, there can be risk for wandering, letting a stranger in the house, or
setting a fire while trying to cook.
There is a loss of ability to perform in areas of work or education. Leisure
participation is limited to activities that do not require problem solving or
decision making such as singing or going out with a friend or family member to
church or a restaurant. In fact, friends or family are needed for any social
participation as the person will have difficulty initiating or maintaining social
interaction outside of his or her immediate environment.
Motor skills remain intact in the mild stage. By the moderate stage, some of
these skills are affected. The decline in visuospatial skills leads to many
problems. Positioning and reaching are not always effective. Poor judgment of
distance, direction, and floor or ground surfaces creates fall risk. By the middle
stage, all process skills are impacted. Attention is limited, and serious memory
impairment compromises all aspects of knowledge, temporal organization,
organizing space and objects, and adaptation.
In the mild stage, the person begins to have problems with communication
and interaction skills. By the moderate stage, information exchange is limited
because of memory impairment and aphasia. As a result, social interactions are
affected, often becoming limited to caregivers. Communication may be driven
by anxiety, and it is not unusual for the individual to perseverate, asking the
same question or expressing the same worry over and over again.
Performance patterns are severely limited. The individual may attempt to
engage in habits and may be successful in some such as brushing hair or teeth.
Habits that require problem solving or adaptations, such as setting an alarm
clock or sewing a button, will be less productive if attempted at all. While most
people have established routines for workdays, weekends, and holidays, the
person in the moderate stage of AD can no longer differentiate days. This loss of
routine can contribute to the person’s anxiety and depression. Caregivers can
help by maintaining structure in the day, as well as creative use of meaningful
occupations. Roles continue to be lost, and family and friends need to support
the most significant roles by reminiscing and affirming the individual’s
importance.
Cultural contexts begin to diminish in moderate stage AD. Personal and
temporal contexts may be confused, and some days, the person may believe he
or she is in an earlier stage of life. Some cultural, social, and spiritual contexts
may remain intact, but as the condition progresses, there is less attention given to
beliefs and values that defined the individual.
Client factors of mental functions are seriously impaired. Orientation to
person, place, and time is affected. Personality changes; instead of spontaneity,
there is disinhibition; instead of motivation and goal direction, there is apathy or
anxiety; instead of fluent verbal and nonverbal communication, there is
disinterest or perseveration. There is progressive impairment in all cognitive
functions. The person may have hallucinations or delusional ideas. Emotions are
not well regulated, and there can be outbursts of anger, fear of an imagined
threat, or crying spells that come and go suddenly.
Severe Stage
During the severe stage of AD, all areas of occupation diminish and are lost. The
person is fully dependent in all ADLs and can no longer ambulate with safety.
All performance skills and patterns are impaired, and eventually the person loses
all functional capacity. Speech is reduced to a few words and then lost entirely.
The person may moan or cry, and caregivers need to assess this as a possible
response to pain, discomfort, or psychosis. There is no awareness or
understanding of cultural, social, or spiritual contexts. Mental functions are
completely impaired, and now there is serious impact on neuromusculoskeletal
and movement-related functions. Nerve cell damage takes a toll on muscle
strength and tone, and voluntary movement is limited. Once the ability to
swallow is lost, the family must make the decision to provide artificial life
support or allow the natural course of the illness to proceed to death.
It is important to note that individuals with AD will show a wide variance in
the signs and symptoms within these stages. This is true for several reasons. An
individual with a strong set of beliefs and values may maintain these ideas
longer than expected; the woman who always took care and pride in her
appearance will maintain grooming habits in the moderate stage; someone who
has always been organized may adapt for memory loss more productively.
Conversely, a formerly shy person may become very impulsive and disinhibited
or become even more withdrawn. The course of the disease is also dependent
upon the extent and the areas of neuronal damage.
Non-Alzheimer’s Neurocognitive Disorder
Several other types of non-Alzheimer’s NCDs may be seen by occupational
therapists. These types include
Vascular NCD
Frontotemporal NCD
NCD with Lewy bodies
Reversible NCD
Vascular NCD
Weiner and Lipton (2009)
state that vascular NCD is second to AD as the most
common type of NCD, accounting for approximately 10% to 20% of late-onset
cases. However, some researchers suggest that “mixed” AD and vascular
dementia may be the most common form, and in Japan, China, and Russia,
vascular dementia is more common than AD. While there is a slightly greater
risk of AD for women, vascular dementia is more likely to occur in men,
although this gender difference becomes less prominent with age (Szoeke et al.,
2009). For persons having a stroke, 20% to 30% are diagnosed with NCD within
3 months. The prevalence of vascular NCD increases from 13% at age 70 to
more than 44% at age 90 (American Psychiatric Association, 2013).
Vascular NCD can be caused by large multi-infarcts, which are blockages in
the large vessels of the brain. Lacunar strokes, in which the small arteries are
affected, can also cause vascular NCD. Lacunar strokes affect very small areas
of tissue, and people with a history of arrhythmias, or irregular heartbeat
rhythms, may be especially at risk for this problem (Cohen & Eisdorfer, 2001). While
factors such as hypertension, diabetes, dyslipidemia or abnormal lipid levels,
coronary artery disease, and nicotine dependence have been known to contribute
to vascular NCD, nicotine use is a completely preventable risk factor and should
be a focus of clinical intervention as well as public health campaigns (Chandra &
Anand, 2015).
Frontotemporal Neurocognitive Disorder
Memory/learning and perceptual-motor symptoms are less prominent in
FTNCD. This form of dementia was first identified by Pick in 1906 and was
known as Pick’s disease (Kertesz, 2004). Some researchers refer to this condition as
Pick’s complex and believe that it includes an overlapping of the following
syndromes: primary progressive aphasia, corticobasal degeneration, progressive
supranuclear palsy, and motor neuron disease (Kertesz, 2003). The most prominent
feature of FTNCD is a change in character and social conduct. The DSM-5
specifies two types of FTNCD: behavioral variant and language variant. In
behavioral variant FTNCD, there is a decline in personal hygiene and
socialization. Insight is usually impaired as is social cognition. Mental rigidity
and inflexibility combined with distractibility can cause the person to appear
memory impaired, when, in fact, memory may yet be intact. There may be
perseverative and stereotyped behavior, such as humming or hand rubbing.
Utilization behavior refers to touching or grasping anything within sight and
inappropriate use of objects such as trying to drink from an empty cup.
A striking feature of behavior variant FTNCD is the combination of
disinhibition with apathy, although, at various stages of the disease, one or the
other symptom may predominate. Disinhibited behaviors manifest as abnormal
demonstration of sexual acting-out, or hypersexuality hyperorality (a condition
created by oral insertion of objects), and utilization behavior. For the middle-
aged or older person, hypersexuality may consist of verbalizations or gestures.
Hyperorality can be seen in excessive overeating or in developing a limiting
food preference, such as eating only milk and bananas. Some may grab food, eat
from other’s plates, or eat inedible objects. Another group of behaviors are
sometimes referred to as "negative symptoms" because they represent a lack in
behavior. These include apathy, amotivation, indifference, and flat affect. There
may be striking disinterest in the affairs of the family. The person may neglect to
change clothing or lack the ability to attend to any task to completion (Mosimann &
McKeith, 2003). However, episodic memory is generally less impaired in
comparison with AD (Xie et al., 2009).

In language variant FTNCD, speech is impaired. There are three subtypes
commonly seen: semantic variant (impaired single word comprehension, poor
object and/or person knowledge, errors in reading aloud);
agrammatica/nonfluent variant (grammatical simplification and errors, effortful
halting speech); and logopenic variant (impaired single word retrieval, impaired
repetition of phrases and sentences).
This disorder was often mistaken for AD, as its course is progressive.
Survival is usually shorter and decline is faster in FTNCD than it is in AD.
Neurocognitive Disorder with Lewy Bodies

NCDLB has often been misdiagnosed as AD or delirium or viewed as
Parkinson’s disease plus AD. Lewy bodies are microscopic spherical neuronal
inclusion bodies within the cytoplasm of a cell. In NCDLB, Lewy bodies are
found in the cortical and subcortical structures of the brain distinguishing
NCDLB from Parkinson’s disease, in which Lewy bodies are found in the
substantia nigra (Neugrosch et al., 2004).
Just as with AD, the etiology of NCDLB is unknown. The exact mechanism
through which Lewy bodies form is not clearly understood. It is thought that
Lewy bodies cause neuronal degeneration and eventually lead to cell death (Bras,
2015). Examination at autopsy reveals, in addition to Lewy bodies, the presence
of senile plaques. Unlike the findings of AD, there are sparse neurofibrillary
tangles (Mosimann & McKeith, 2003). Familial aggregation may occur, but in most
cases of NCDLB, there is no family history (American Psychiatric Association, 2013).
Reversible Neurocognitive Disorder

The idea that NCD may be reversible is a topic of debate. Most researchers
today believe that reversible NCD is very uncommon (Knopman & Jankowiak, 2005).
In many cases, what seemed to be a reversible NCD was simply some other
cause of cognitive impairment that was misdiagnosed as NCD or dementia.
Burke et al. refer to the term of reversible NCD as a misnomer and state that
NCD should only be used to identify cognitive impairment in cases of
irreversible degenerative brain disease (Burke, Sengoz, & Schwartz, 2000). A possible
exception to this rule is in cases of substance-induced NCD. The person who
begins to show signs of substance-induced NCD but is able to achieve stable
abstinence prior to age 50 may show substantial and even complete recovery of
neurocognitive functions (American Psychiatric Association, 2013).
It is a fact that cognitive impairment can be misdiagnosed as NCD. Having a
potentially treatable impairment misidentified, and left untreated, could have
drastic and dire consequences for the person’s function and way of life. As a
result, it is important to review what some may refer to as reversible NCD and
others see as reversible cognitive impairment.
CASE-STUDY 1
Mrs. L. was born in South America. She was bilingual, speaking her native
Spanish as well as flawless English. In her early years, she had the luxury
of maids and a cook. She was a widow before her two children were grown.
At age 60, her adult daughter died, leaving Mrs. L. as the primary caregiver
for her two young grandchildren. Soon after her daughter’s death, Mrs. L.
fled her native land for political reasons, bringing her orphaned
grandchildren to live in New York. After raising them, she continued to live
with her adult granddaughter, G.
Mild Stage
When Mrs. L. was 82, G. married and her new husband moved into their
home. Mrs. L. had always told G. that “when you marry and no longer need
me, it will be my time to die.” She seemed angry at G. and showed her
irritation by complaining. Nevertheless, she continued to prepare the family
meals, make beds, and do some of the laundry. In spite of failing vision, she
was aware of the need to dust. She continued to take impeccable care of her
appearance, always setting her hair at night and never going out without
wearing hose and high heels. Her social life was full, with friends and
neighbors frequently visiting. She took pride in serving tea and dessert to
her weekly women’s support group who met at her apartment. There were
times when Mrs. L. misplaced her dentures (in odd places such as in a plant
pot) or forgot to use detergent when washing the dishes. She was often
anxious and needy when G. came home from work, and she became
defensive and easily upset when questioned about mistakes. Her
granddaughter attributed these aberrations to Mrs. L.’s increased stress as a
result of the marriage.
Moderate Stage
At age 86, Mrs. L. broke her hip; her granddaughter had a new, colicky
baby; and the family moved to a larger apartment in the same complex. As
a result of these crises, Mrs. L. seemed to have slowed down and became
more dependent on G. She began making more obvious mistakes, such as
using the toilet brush to scrub the floor and neglecting to wash the dishes
before putting them away. G. took over the housekeeping and cooking
duties.
Mrs. L.’s hearing and sight continued to decline, and she complained
about “nothing ever being right.” G. got her books on tape, but Mrs. L.
could not learn to operate the tape player. She continued to entertain her
weekly women’s group, basking in the compliments from her friends for the
tasty desserts that they knew were now being made by G.
Mrs. L. became a picky eater, putting catsup on everything, including
salads and fruit. She was still very particular about her appearance, but she
began to need help doing her hair and became neglectful of her denture
care. She could still fold laundry, make beds, and take her bath
independently.
There were times when Mrs. L. became fearful of G.’s husband,
accusing him of wanting to hurt her granddaughter.
At age 91, the family moved out of the apartment complex, where they
had lived for many years, to a new home. Mrs. L. was no longer able to
make impromptu visits to her neighbors, and her circle of friends now
rarely visited. Mrs. L. was afraid to use anything in the new kitchen,
especially the unfamiliar gas stove. Her appetite continued to be poor, and
she was losing weight. Strategies to enable her to make her own tea and
toast were unsuccessful, and she would forget to eat food left for her in the
refrigerator when G. went to work.
Mrs. L. began to have incontinence of bowel and bladder, yet she would
resist her granddaughter’s suggestions that she needed to shower or bathe.
Ironically, when Mrs. L. fell and broke her wrist, she became amenable to
assistance. Insurance covered about 6 weeks of home health care, and Mrs.
L. seemed to enjoy the attention.
Severe Stage
At age 92, it was clear that Mrs. L. could not be left alone. She continued to
be incontinent; she couldn’t lift herself from the toilet and seemed afraid to
use a bedside commode. When her granddaughter came home from work,
she would follow her about, repeating the same question over and over. She
was not eating lunch or dinner. Her granddaughter, pregnant with her
second child, was having difficulty bathing Mrs. L. On one occasion, they
both ended up falling on the bathroom floor during a bathing session. In-
home care proved unaffordable, and G. made the difficult decision to place
Mrs. L. in a nursing home. The nursing home, known for its excellence,
proved to be a positive move for Mrs. L. She regained some of her weight
and seemed much less anxious. Mrs. L.’s deep-rooted social personality and
gift for charming those around her made a reappearance. This served to
ensure that a constant stream of staff and residents dropped by her room for
brief chats. When G. and her family visited, Mrs. L. remained cool, as if to
reprimand G. for sending her away.
Within a year, Mrs. L. was not always able to recognize G. when she
came to visit. Sometimes, she mistook G. for her daughter. Eventually, she
completely lost the ability to recognize G. Her granddaughter continued to
visit with her own children, who delighted in playing word games with Mrs.
L. The youngest child, himself learning to speak, would say a few words or
phrases to Mrs. L., which she would mimic. This would set both children to
giggling, in turn pleasing Mrs. L. to have had such an amusing effect on the
children.
At age 94, Mrs. L. seemed no longer able to speak English. Her
verbalizations in Spanish were limited to a few words, which were
sometimes unintelligible. She was no longer able to ambulate and needed
total care for feeding. Shortly after her 95th birthday, Mrs. L. died
peacefully of heart failure.
CASE-STUDY 2
Mr. B. is a 71-year-old African American who is cared for at home by his
wife. Mrs. B. describes him as having been a quietly dignified person
before the onset of his illness. He enjoyed spending time with his family,
including his four children and other members of his extended family.
As a young college graduate in the 1960s, Mr. B. was unable to find
work in his field of engineering but did get an unskilled job with the phone
company. His supervisor recognized his talents and abilities, and, over time,
he had a series of promotions that eventually took him to the position of
personnel manager.
When Mr. B. was 60, his company was bought out and he was given an
early retirement. Soon after this, he started taking classes in cabinet making
and began working for an architect who was building a local church.
At age 64, Mr. B. began making errors in measurement in his work. He
was aware of, and troubled, by this difficulty. He decided to cut back his
hours at work. Mrs. B. noted at this time that he sometimes appeared
confused. During family gatherings, he was not only quieter than his usual
self, but he would actually distance himself by sitting in another room.
Although he was still driving, he began misplacing things. Mrs. B. had
some concerns about these behaviors but attributed them to the stress of life
changes.
It was when Mr. B. was no longer able to read a ruler that Mrs. B.
began to realize something was seriously wrong. At about this time, Mr. B.
had an episode in which he got lost on the way to the dentist. Mrs. B. turned
to her family physician for help, and an 18-month period of medical and
neurological testing ensued.
At age 66, Mr. B. had a stroke. During hospitalization, the diagnosis of
vascular dementia was finally made. When Mr. B. left the hospital, he was
able to walk and perform his self-care activities independently.
At first, things went well at home. Both Mr. and Mrs. B. adjusted to a
routine in which she would make sure that he was up, dressed, and had
breakfast before she left for work. His lunch was prepared and left for him
to retrieve from the refrigerator. One day, Mrs. B. came home from work
and found that the stove had been left on. At this point, she realized that Mr.
B. was no longer safe when left alone, and she enrolled him in a day-care
program.
About 8 months later, Mrs. B. made the decision to stop working. The
day program cost almost as much as her pay. It was becoming more and
more difficult to help Mr. B. get dressed and out of the house every
morning. In fact, Mr. B. started choosing to undress himself several times a
day. He began having problems speaking and understanding what others
said to him. Mrs. B. felt he was losing his personality; he wasn’t able to
focus on conversation or show interest, even when his grandchildren
visited. He was still ambulating with help. He began having difficulty
swallowing, was losing weight, and was having bladder infections. He was
no longer continent of bowel or bladder.
At age 69, Mr. B. had another stroke. Despite the efforts of the
rehabilitation service, he could no longer ambulate. He returned home in a
wheelchair. His home did not have a ground floor bedroom, so Mrs. B.
converted the living room to his bedroom. For about 1 month, his insurance
provided a home health aide who would give Mr. B. a weekly bed bath.
Eventually, Mrs. B. took over this responsibility, along with complete care
for dressing and feeding him. Mr. B. often did not recognize Mrs. B. and no
longer recognized his children.
During a recent visit to the outpatient clinic, Mrs. B. talked about the
difficulty of getting through each day. “I rarely get out unless one of the
family members can stay with my husband, and even then, I’m just too
tired. I think about the reality that someday I may need to put him in a
nursing home. I’ll do it when it gets to the point where I’m no longer able
to take care of him or if my health goes bad. But if that happens, I’ll lose
our life savings—it’s not much, but enough to help pay bills with my social
security check.” She also related an incident that illustrated the small daily
frustrations of caring for her husband: “I was feeding him lunch, and he
reached for the glass of milk with his left hand. I helped place the glass in
his outstretched hand, but he began raising his right hand to his mouth as if
to drink. Obviously, that wasn’t working, so I took the glass out of his left
hand and put it in his right hand. This must have totally confused him, and
he just looked at me as if to say ‘what did you do that for?’ I felt so helpless
and so bad that I couldn’t even help him.”
Mr. B., who at 71 is still handsome, sits straight and tall in his
wheelchair, appearing to emanate dignity and calm, faintly smiling and
nodding as his wife tells their story.
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C H A P T E R
12 Complex Trauma
Brandon G. Morkut Ben J. Atchison
KEY TERMS
Alexithymia Complex trauma Developmental trauma disorder Dissociation
Parentification Polyvictimization Resiliency Transgenerational Trauma
According to her stepgrandmother, 9-year-old Brittney was removed over a year
ago from her biological mother’s and stepfather’s care due to neglect, subjection
to chronic domestic violence, and exposure to methamphetamine. This situation
resulted in her current placement with her stepgrandmother. Brittney’s
stepgrandmother reports Brittney’s biological father, mother, grandmother, and
stepfather frequently used methamphetamine. Additionally, Brittney’s biological
father was violent toward her mother while she was pregnant and continued the
erratic episodes of violence after Brittney was born, an event her biological
father failed to attend. She also stated that a year after Brittney was born, her
biological father left Brittney and her mother, which resulted in Brittney’s
mother marrying her stepfather. Over the years, she lived with her stepfather and
mother, Brittney demonstrated significantly poor school attendance due to living
in multiple residences that included a motel, an apartment in which they were
evicted from due to producing a filthy and unsafe living environment, a “shack
in the country,” and eventually a camper without running water that was infested
with cockroaches. Presently, Brittney is described by her stepgrandmother as
struggling academically in all areas, lacking efficient problem-solving skills,
grappling to understand cause and effect, and having few friends at school. Her
stepgrandmother continues to explain that Brittney visits her mother 4 hours per
week at the public library and visits her stepfather in jail with her mother every
other week. Furthermore, her stepgrandmother stated that Brittney has not seen
or had contact with her biological father since he left her at the age of 1 year.
Brittney is unaware of the fact that her biological father is in jail as a result of
drug abuse and that, in addition to Brittney, he has fathered four other children.
Prior to the 19th century, child abuse was not only acceptable but was,
essentially, legally sanctioned. At that time, children did not receive equal legal
status like domesticated animals with regard to protection against cruelty and/or
neglect. It was not until 1962 that the term “battered child syndrome” became
part of the medical terminology and not until 1976 that all of the states in the
United States had adopted laws mandating the reporting of suspected child
abuse.
Trauma, from the Greek word meaning “wound,” was originally used in
medicine for a serious physical injury, but it is more widely used now to refer to
emotional shock following a stressful event or an experience that is deeply
distressing. The American Heritage Dictionary defines trauma as “a serious
injury or shock to the body, as from violence or an accident,” which is what
comes to mind when thinking of “trauma care” or a “trauma unit.” This chapter
is focused on the second component of this definition, which is “an emotional
wound or shock that creates substantial, lasting damage to the psychological
development of a person, often leading to neurosis, and an event or situation that
causes great distress and disruption” (American Heritage, 2011).
In 2013, the American Psychiatric Association published the Diagnostic and
Statistical Manual of Mental Disorders 5th edition (DSM-5) in response to an
abundance of new research data and practical knowledge related to mental health
disorders (American Psychiatric Association, 2013). To represent children who
experienced or witnessed the traumatic event(s), the DSM-5 Task Force
established posttraumatic stress disorder for children 6 years and younger under
the classification of a trauma- and stressor-related disorder (American
Psychiatric Association, 2013). Posttraumatic stress disorder (PTSD) was
originally created and published in the DSM-III in 1980 in response to the large
number of Vietnam veterans returning home with psychiatric problems. Until the
publication of the DSM-5, PTSD was the only trauma-related diagnosis used for
both children and adults (van der Kolk, 2005).
The main attributes used to describe PTSD for children 6 years and younger
include experiencing, witnessing, or learning about death, violence, or injury,
especially toward a caregiver, one or more times. As a result of the trauma
experience, children may encounter intrusive thoughts, avoidance, or negative
emotional reactions to reminders of the traumatic event in conjunction with
physiological dysregulation, as demonstrated by disturbance in sleep,
hypervigilance, concentration difficulties, and angry outbursts (American
Psychiatric Association, 2013). Unfortunately, there continues to be a population
of children who continually experience a dark and unsafe world as a result of
chronic exposure to interpersonal violence, neglect, abuse (e.g., physical,
emotional, or sexual), and maltreatment, in addition to a caregiver who
repeatedly fails to provide protection or is unavailable during times of extreme
stress. As a result, these children often receive a cluster of diagnoses to identify
their complex behaviors and negative emotional reactions, which can impair
their ability to successfully participate in age-appropriate daily activities (e.g.,
school, play, self-care, toilet training, etc.) and to develop positive peer
relationships. Frequent inaccurate disorders include bipolar disorder, attention
deficit/hyperactivity disorder (ADHD), anxiety disorders, oppositional defiance
disorder (ODD), conduct disorder, obsessive-compulsive disorder (OCD), and
sensory processing disorder (SPD). Furthermore, the impact of receiving
multiple mental health diagnoses most often leads to interventions focusing on
behavioral control. This can limit a clinician’s ability to provide effective
research-based interventions as reflected by addressing symptoms based on a
single etiology (e.g., complex trauma).
In 2009, the Consensus Proposed Criteria for Developmental Trauma
Disorder (DTD) was established by the National Child Traumatic Stress
Network (NCTSN)-affiliated Task Force led by Bessel A. van der Kolk, MD,
and Robert S. Pynoos, MD (van der Kolk, 2009, 2014). The cornerstone of the
Task Force was to develop proposed criteria that reflect the true reality and
outcomes in children exposed to chronic interpersonal trauma experience. The
authors of the Task Force hoped that this new set of criteria would lead to the
acceptance of the diagnosis of DTD in the publication of the revision of DSM-5.
This more complete and holistic set of criteria would result in a more
comprehensive approach to intervention so that children would receive the
proper care to improve their quality of life and ability to appropriately react to
daily experiences (van der Kolk et al., 2009). Extensive review of empirical
literature, surveys of NCTSN clinicians, discussions with clinical experts, and
preliminary analysis of data representing thousands of children in various
clinical and child service system settings (e.g., state child welfare systems,
juvenile detention centers, and inpatient psychiatric settings) allowed the
NCTSN Task Force to construct a proposed set of criteria for DTD, which
contained the most clinically significant symptoms displayed by children
following complex trauma. DTD symptoms include affective and physiological
dysregulation, attentional and behavioral dysregulation, and self- and relational
dysregulation (van der Kolk, 2014). Furthermore, to meet the criteria for DTD
exposure, the child must have experienced or witnessed multiple adverse events
over a minimum period of 1 year that begins in early childhood or adolescent, in
conjunction with chronic exposure to interpersonal violence and significant
interruptions of protective caregiving, which may include exposure to severe and
repeated emotional abuse (van der Kolk, 2005, 2014). Unfortunately, several
months after submitting their proposal for DTD, it was rejected by the DSM
subcommittee with consensus that no new diagnosis was required to fill a
“missing diagnostic niche” and “The notion that early childhood adverse
experiences lead to substantial developmental disruptions is more clinical
intuition than a research-based fact” (van der Kolk, 2014, p. 16).
Clinicians, including occupational therapists, who treat children with a
history of chronic trauma, have increasingly called for a diagnosis that more
adequately addresses the multiple domains of concern that result from
developmentally adverse interpersonal trauma because the multitude of signs
and symptoms cannot be accurately described by current diagnoses. As of the
date of the publication of this chapter, the term complex trauma most accurately
describes and represents children and adolescent subjected to repetitive adverse
experiences. As defined by experts in the field of childhood trauma, complex
traumas “describe both a constellation of causal risk factors involving repeated
interpersonal trauma by caregivers early in life; and the resulting dysregulation
that occurs across a range of areas including emotional, behavioral,
interpersonal, physiological, and cognitive functioning” (Greeson et al., 2011, p.
93). Therefore, this chapter will focus on the complex trauma because this is the
condition most relevant to occupational therapy practice.
Etiology
Complex trauma can be the result of multiple exposure to traumatic events,
maltreatment, or polyvictimization beginning in early childhood or adolescent,
occurring within the context of unpredictable, uncontrollable, and violent
environments in conjunction with inconsistent or absent protective caregiving.
The abuse, whether it is physical, sexual, or emotional, and neglect a child
experiences from a caregiver are often transgenerational, that is, the caregiver’s
actions are based on his or her own previous parenting experiences (Perry,
2001). The most deadly form of child trauma is caused by neglect, as death can
occur from accidents that occur in the absence of supervision or during
abandonment. Failure to seek necessary medical attention in cases of injury,
illness, or a life-threatening medical condition places a child at risk for death as
well. Fatal injuries are caused by a variety of traumatic actions including severe
head trauma, such as violently shaking an infant or small child; forceful
punching of the fist to the abdomen, chest, or head; scalding; intentional
drowning; suffocation; poisoning; and starvation.
Multiple risk factors related to complex trauma have been identified (Center
for Disease Control and Prevention, 2015; World Health Organization, 2014):
Parental risk factors include young or single parents, those who did not
graduate from high school, and those who either were abused themselves as
children or endured a severely dysfunctional home life. Difficulty providing
quality care to a large number of dependent children can also increase the
risk of abuse.
Adults with psychiatric disorders such as depression and bipolar disorder
are more likely to abuse children.
A common theme when interviewing abusive individuals is their unrealistic
expectations of infant or child development. Often, they expect maturation
of developmental milestones significantly beyond the age of the child. This
is especially true for toilet training expectations.
The perpetrator’s childhood: Approximately 80% of offenders were
themselves abused as children.
Substance abuse: Children in alcohol-abusing families are nearly four times
more likely to be mistreated, almost five times more likely to be physically
neglected, and 10 times more likely to be emotionally neglected than
children in non–alcohol-abusing families.
Family support systems: Other factors include the disintegration of the
nuclear family and violence between other family members and, in
addition, the loss of child-rearing support from the extended family
members.
Children at higher risk for abuse include infants who are felt to be “overly
fussy,” as well as children with congenital anomalies, chronic/recurrent
conditions, and children with chronic diseases as well as children with
learning disabilities, speech/language disorders, and intellectual disability.
Specific “trigger” events that occur just before many fatal parental assaults
on infants and young children include an infant’s inconsolable crying,
feeding difficulties, a toddler’s failed toilet training, and exaggerated
parental perceptions of acts of “disobedience” by the child.
Family income strongly correlates to incidence rates. Children from
families with annual incomes below $15,000 per year are more than 25
times more likely than are children from families with annual income above
$30,000 to be harmed or endangered by abuse or neglect. Poverty clearly
predisposes a person to child abuse. However, it must be recognized that all
data available can only be based on reported cases. It is very likely that
trauma exposure exists among other classes as well, but those families are
often protected by position and wealth and thus their cases do not
necessarily become part of a community’s child protective services system.
Parents’ or caregivers’ characteristics that may increase child maltreatment
include difficulty bonding with their newborn, involvement in criminal
activity, and inability to provide quality nurturing to their child.
Multiple nonbiological, transient caregivers living in the home.
Neglect and medical neglect are most often attributed to female caretakers,
while sexual abuse is most often associated with male offenders.
Younger children are at risk: If the child is unwanted, 67% of abused
children are <1 year old, and 80% are <3 years
Adopted and foster children are at higher risk.
Child maltreatment most often occurs within the home (Perry, 1997) and an
estimated 91.6% of maltreated children are victimized by one or both parents
(US Department of Health & Human Service, 2016). Bruce Perry, MD, PhD,
best describes infants and children raised in violent and erratic settings as
“incubated in terror” (Perry, 1997, p. 2). Data collected during the 2014 financial
fiscal year and analyzed by the National Child Abuse and Neglect Data System
(NCANDS) indicate that an estimated 702,000 victims experienced child abuse
and neglect. Child victims were similar for both boys (48.9%) and girls (50.7%).
Three-quarters (75%) of maltreated children were victims of neglect, while 17%
of the children experienced physical abuse and 8.3% were victims of sexual
abuse. Approximately 75% of sexual abuse is inflicted upon girls. Girls also are
more likely to suffer from sexual abuse, emotional abuse, and neglect. Boys, on
the other hand, are more likely to experience physical trauma (other than sexual
abuse). Data from the Child Maltreatment 2014 report (US Department of Health
& Human Service, 2016) also indicate more than one-half (54.1%) of
perpetrators were women and 44.8% were men. In addition, mothers (40.7%)
were the most frequent perpetrator of maltreatment in relation to 20.5% of
fathers. With regard to age of perpetrator, 83.2% of perpetrators were between
the ages of 18 and 44 years in conjunction with ages 25 through 34 years
representing the highest group of perpetrators.
Unfortunately, an estimated 1,580 children nationally died from abuse and
neglect during the 2014 financial fiscal year. Children <1 year old were the most
vulnerable to maltreatment, and almost three-quarters (70.7%) of all child
fatalities were younger than 3 years old. Boys had a higher child fatality rate
than did girls, and 79.3% of child fatalities involved at least one parent. Further,
2014 child maltreatment data indicate that 72.3% of child fatalities were due to
neglect, and 41.3% of child deaths were the result of physical abuse.
Retrospectively, 1.1% of the child fatalities were caused by emotional and sexual
abuse (US Department of Health & Human Service, 2016).
Signs and Symptoms
Complex trauma is composed of seven domains of impairment observed in
children subjected to reoccurring trauma exposure. Those domains include
attachment, biology, affect regulation, dissociation, behavioral regulation,
cognition, and self-concept (Cook et al., 2007).
Attachment
The most significant signs of exposure to interpersonal trauma are maladaptive
behaviors related to the disruption of attachment. The type of relationship
between a child and caregiver influences the response to present and future
emotional and physical experiences. A normal child-caregiver relationship
develops into a secure attachment in about 55% to 65% of the general population
(Cook, Blaustein, Spinazzola, & van der Kolk, 2003). In a secure attachment, the
caregiver responds to stressful situations by providing the child a safe
environment and a sense of protection. This in turn influences the child’s ability
to appropriately regulate affect and behavior. Over 80% of children who
experience chronic trauma demonstrate insecure attachment patterns (Cook et
al., 2003). Repeated exposure to unpredictable and uncontrollable stressful
environments, in conjunction with erratic, hostile, rejecting, or abusive
caregiving, is a precursor to an insecure child-caregiver attachment (Cook et al.,
2007). Insecure attachment and associated signs can be subcategorized into three
typologies: avoidant, ambivalent, and disorganized.
Avoidant attachment develops as a result of persistent caregiver rejection
and failure to provide basic emotional and physical support to his or her child.
These children often become skilled at distancing their emotions in conjunction
with avoiding the establishment of meaningful relationships with peers and
adults. Children with ambivalent attachment experience a range of predictable
patterns of detachment and neglect to excessive intrusiveness from their parents.
Disorganized attachment develops when children are traumatized by repeated
exposure to uncontrollable and unpredictable stress, for example, physical or
sexual abuse, in conjunction with the consistent absence of a nurturing, reliable,
and protective caregiver. Children who demonstrate difficulty organizing an
adaptive response to daily experiences, due to childhood trauma, display
significant challenges regulating their emotions. In addition, they experience
problems in managing stress and developing empathy (Cook et al., 2007).
It has been hypothesized that disorganized attachment interferes with
efficient neurodevelopmental connections between the right and left hemispheres
of the orbital prefrontal cortex, which is responsible for regulating emotions,
conscious decision-making, and social behavior (Cook et al., 2003). Other brain
structures associated with the development of attachment that can be affected by
complex trauma include the amygdala, hippocampus, hypothalamic-pituitary-
adrenal (HPA) axis, and the neurotransmitters oxytocin, dopamine, and
norepinephrine, which impact, respectively, on threat detection, new memory
and learning, response to threat detection, and release of hormones critical for
healthy emotional states that are critical for affective development (Henry,
Sloane, & Black-Pond, 2007). Attachment dysfunction leads to intense
preoccupation with the safety of the caregiver or other loved ones and difficulty
tolerating reunion with them after separation. Other outcomes that emerge due to
poor attachment include the following (Cook et al., 2003):
Problems with personal boundaries

Chronic feelings of distrust and suspicion
Social isolation
Establishing and maintain healthy relationships thought life
Difficulty attuning to other people’s emotional states and perspective taking
Biology
As a result of being in a state of chronic fear and stress, the child’s brain alters its
typical bottom-up organization (i.e., brainstem, midbrain, limbic structure, and
cerebral cortex) and redirects neural systems to support repeated survival-based
reactions or “flight, fight, or freeze” to stressful experiences. Due to repeated
activation of the HPA axis, which controls stress responses, traumatized children
can habitually be in a continuous hyperarousal state (Gaskill & Perry, 2014;
Perry, 2001). Other brain structures impacted by maltreatment include the
thalamus, which is vital for sensory and motor signal relay and the regulation of
consciousness and sleep. The thalamus plays a role in controlling the motor
systems of the brain, which are responsible for voluntary bodily movement and
coordination and the corpus callosum, the structure connecting the two cerebral
hemispheres (Gaskill & Perry, 2014). In a study of 900 children with a history of
maltreatment, caregiver-generated survey data of sensory processing indicated
difficulty with sensory modulation. It has been postulated that sensory
modulation disorder is a result of difficulty in the brain’s ability to filter and
inhibit extraneous environmental stimuli (Bialer & Miller, 2011). Survey data
indicated 53.1% of the sample population demonstrating signs associated with
difficulty filtering auditory sensations, 51.7% displaying sensory-seeking
responses, and 23.8%demonstrating patterns of sensory overresponsivity to
tactile input (Atchison, 2007).
Affect Regulation
A common deficit observed in children with complex trauma histories is their
inability to distinguish and interpret internal states of arousal in conjunction with
difficulty accurately identifying emotions, for example, mad, happy, and sad, in
self and others (Cook et al., 2003). Impaired affect regulation is the result of
exposure to inconsistent displays of affect and behavior (e.g., a cheerful
expression combined with rejecting behavior) or to inconsistent responses to
display of emotion (e.g., infant distress is united inconsistently with rejection,
anger, nurturance, or detachment) (Cook et al., 2007). Alexithymia, or the
impaired capacity to describe emotions or bodily states, is a persistent sign of
affect dysregulation. This includes difficulty describing internal states and
communicating basic needs such as hunger or elimination, as well as expressing
emotions, wishes, and desires (Sayar, Kosfe, Grabe, & Topbas, 2005; Way,
Yelsema, Van Meter, Hyter, & Black-Pond, 2007). Due to difficulty with
identifying, interpreting, and regulating emotions, traumatized children may
avoid emotional situations including positive experiences, display dissociation,
or chronic emotional “numbing” and develop maladaptive coping strategies
(Cook et al., 2007).
Dissociation
Dissociation is a predominate attribute in children exposed to complex trauma.
Essentially, dissociation is a loss in the ability to process and integrate
information and experiences. As a result, conscious awareness fails to respond to
physical sensations, disconnection between thought and emotion is facilitated,
and involuntary repetitive behavior may emerge (Brand & Lanius, 2014). As
described by Putnam (1997), dissociation serves three primary functions during
exposure to overwhelming trauma, which is evident by a protective “freeze”
response, suppression of painful emotions and memories, and detachment from
one’s self. Unfortunately, a child who habitually dissociates as a coping strategy
can intensify difficulties with self-concept, behavior management, and affect
regulation (Cook et al., 2007).
Behavioral Regulation
Traumatized children often demonstrate difficulty regulating their behavior to
participate in age-appropriate daily activities. It is important to note that
traumatized children’s maladaptive behavior patterns are reflections of their
difficulty adapting to significant stress (Cook et al., 2003). Maladaptive
behaviors include difficulty with arousal regulation and modulation, which is
demonstrated by the lack of and recovery from extreme affective states such as
fear, anger, and shame that emerge from chronic trauma exposure. These
affective states may be manifested by prolonged and extreme emotional tantrums
on one side of the spectrum to catatonic-like immobilization. Disorders of bodily
functions may occur including disturbances in sleeping, eating, and elimination
(Egger, Costello, Erkanli, & Angold, 1999; Glod, Teicher, Hartman, & Harakal,
1997; Noll, Trickett, Susman, & Putnam, 2006). Furthermore, the alteration of
affective states may lead to misperception of risk, combined with poor impulse
control and difficulty understanding consequences of negative behaviors. When
experiencing a sense of threat, maladaptive attempts at self-soothing may
include head banging, body rocking, and compulsive masturbation (Ford et al.,
2000). Self-harm among chronically traumatized children and adolescents has
been described in the literature. These include dangerous actions such as setting
fires, sexual promiscuity, and actions of self-harm such as cutting, picking, and
burning one skin and other self-mutilation actions (Brown, Houck, Hadley, &
Lescano, 2005).
Cognition
Exposure to maltreatment can be associated with impairment in cognitive
function, which can be identified by late infancy. By early childhood, maltreated
children may display less cognitive flexibility and problem-solving skills, which
can affect their future academic growth and performance (Cook et al., 2003;
Egeland, Sroufe, & Erickson, 1983).
Further attributes related to cognitive dysfunction as a result of complex
trauma include the following (Cook et al., 2003, 2005):
Delayed language development

Problems with object constancy
Difficulties with sustained attention to task to complete a task
Visual perceptual problems
Difficulty understanding complex visual-spatial patterns
Lack of persistent curiosity
Self-Concept
The sense of feeling worthy and belonging and being accepted and the
perception of being capable of engaging and completing novel and challenging
tasks are frequent deficients in complexly traumatized children (Ferrer & Fugate,
2003). The early caregiver relationship significantly influences the development
of an unblemished sense of self. These factors include a responsive caretaker to
the child’s needs and a caretaker who offers quality and frequent touch and eye
contact. In childhood, the development of a healthy self-concept is facilitated by
the child being able to safely explore and interact successfully within his or her
environment, ask questions without feeling they are an annoyance, and engage in
make-believe play (Ferrer & Fugate, 2003). Common symptoms associated with
an impaired self-concept identified in traumatized children consist of feeling
unsuccessful, powerless, helpless, incompetent, and unlovable (Cook et al.,
2003). As a result of a lack of reoccurring positive experiences to develop a
healthy self-concept beginning in early infancy through adolescent by adulthood,
these children can endure a substantial degree of self-blame (Liem & Boudewyn,
1999).
Complex trauma is marked by observable alterations in behavior across multiple
domains with prognosis directly related to the extent of exposure, the application
of appropriate interventions, and resiliency factors. Signs and symptoms and
associated impairments are characterized by progressive deterioration with
episodic signs of symptom severity in childhood and adolescence, as well as
persistent challenges across the lifespan in many cases as evident by the Adverse
Childhood Experiences (ACE) study. The ACE study examined 17,337 middle-
class multiethnic Kaiser Permanente health care insurance members by means of
a general medical questionnaire that included 10 questions related to potential
adverse experiences, that is, witness to parental violence, household dysfunction,
neglect, and abuse (Felitti & Anda, 2010). Results identified that the number of
adverse experiences in the first 18 years of life increased the probability to
develop cancer, heart disease, diabetes, liver disease, and stroke. Elevated ACE
scores were also associated with depression, alcoholism, drug use, obesity,
cigarette smoking, sexual promiscuity, and domestic violence (van der Kolk,
2005). To analyze adult death rates, a 14-year follow-up was initiated, and data
indicated that participants with an ACE score of 6 or higher exhibited a shorter
lifespan of almost 20 years compared to participants with ACE score 0 (Felitti &
Anda, 2010). The multiple consequences that emerge as a result of chronic
trauma and impact on the course are detailed in a seminal white paper by the
NCTSN Complex Trauma Task Force titled Complex Trauma in Children and
Adolescents (Cook et al., 2003). Two major considerations for course and
prognosis are directly related to the extent of neurobiologic damage and
resiliency factors.
Neurobiologic
The impact of chronic trauma on brain development has been reported in the
literature with the primary benefit of these studies being a better understanding
of the scope of the problems associated with trauma exposure as well as
informing clinicians about the intervention approaches necessary to improve the
prognosis for healing and recovery. Ito, Teicher, Glod, and Harper (1993)
reported that children exposed to chronic trauma had left hemisphere EEG
abnormalities in anterior, temporal, and parietal areas. Taylor et al. (2006) found
decreased amygdala activation among children who experienced detached
emotional engagement from parents. In a task requiring identification of
emotions, Taylor also found a significant positive correlation between the
activation of the amygdala and right ventrolateral prefrontal cortex, a finding
that indicates reduced inhibition of the amygdala.
Curtis and Cicchetti (2007) found that maltreated children categorized as
nonresilient had decreased left hemisphere activation when compared to resilient
maltreated children and decreased left parietal activity compared to
nonmaltreated children. Neuroendocrine changes have been documented in the
aftermath of childhood interpersonal trauma.
Bevans, Cerbone, and Overstreet (2008) found that exposure to childhood
trauma was related to alterations in diurnal cortisol variation. Cortisol, a steroid
hormone, is released in response to stress and acts to restore homeostasis.
However, prolonged cortisol secretion results in significant physiological
changes including immunological and neurological changes.
Cicchetti and Rogosch (2001) found that maltreated children with
internalizing problems and coexisting internalizing and externalizing problems
had elevated cortisol compared to nonmaltreated children. Neuroimaging studies
have indicated reduced growth of the hippocampus and limbic abnormalities as
well as diminished growth in the left hemisphere and compromised function of
the corpus callosum, the structure that allows for efficient interhemispheric
connectivity (Teicher, 2000). Henry et al. (2007) summarized brain structures
affected as a result of chronic trauma, which are illustrated in Table 12.1.
TABLE 12.1 Impact of Complex Trauma on Specific Brain Areas

Recognition of the brain behavior connection linking trauma and
neurodevelopmental function is critical to occupational therapists working with
this population so that appropriate intervention is provided to improve the
prognosis for healing and engagement and participation in occupations.
Resiliency Factors
Human resiliency refers to the ability of an individual to recover from adverse or
traumatic events in a manner that is adaptive and nonpathologic. While the risks
of pathologic responses are indeed great among children who have experienced
trauma, there is the potential to gain competence across a variety of domains if
provided with the necessary intervention, including those that address both
internal and external factors (Cherry & Galea, 2015; Masten, 2014). Resilience
is most threatened by the loss of organic and relational protective systems, which
occur in response to traumatic events. The extent of brain damage and associated
cognitive, perceptual, and self-regulatory dysfunction, along with severely
compromised caregiver relationships, are key factors that compromise resilience
(Teicher, Anderson, & Polcari, 2002). In addition, loss of motivation to seek out
interpersonal relationships, to interact with one’s environment, and to learn and
to develop new skills greatly inhibits recovery. However, supportive
relationships, family connections, and cognitive resources help protect one and
serve as “inoculations against adversity” (Kagan, 2004). Several factors have
been found to be the most critical for promoting resilience, including (a) positive
attachment and connections to emotionally supportive and competent adults
within a child’s family or community, (b) development of cognitive and self-
regulation abilities, (c) positive beliefs about oneself, and (d) motivation to act
effectively in one’s environment (Luthar, Cichetti, & Becker, 2000; Masten,
2014; Wyman, Sandler, Wolchik, & Nelson, 2000).
Medical Management
Due to the impact of chronic polyvictimization, or multiple types of trauma, the
child may demonstrate signs of comorbid psychiatric disorders. These common
mental health disorders include posttraumatic stress disorder, depression,
attention deficit/hyperactivity disorder, oppositional defiant disorder, anxiety
disorders, bipolar disorder, dissociative disorders, and personality disorders.
Traditional medical management of trauma will include psychopharmacological
intervention, which, in best practice, is prescribed as an adjunct to psychosocial
treatment modalities. Medication should only be used in conjunction with
trauma-specific treatment and not in the absence of it. In a survey sample
representing 1,699 children, ages infancy through 18 who were exposed to
chronic trauma, it was determined that most trauma intervention occurs in an
outpatient setting with varied treatment modalities (Pynoos et al., 2008). The
frequency of intervention modalities identified is as follows:
Weekly psychotherapy (78%)

Self-management/coaching (62%)
Family therapy (56%)
Play therapy (55%)
Expressive therapies (41%)
Pharmacotherapy (27%)
Community outreach (25%)
Traditional models of intervention are typically provided by either a social

worker or a psychologist; however, there are existing models of assessment and
intervention that are grounded in interdisciplinary and transdisciplinary
approaches, which include several professional disciplines on a team including
occupational therapy (Hyter, Atchison, Henry, Sloane, & Black-Pond, 2001;
Koomar, 2009; Richardson et al., 2015).
Occupational performance areas are significantly compromised as a result of
complex trauma, Evidence of compromised client factors has been determined
including those related to motor and praxis skills, sensory-perceptual skills,
emotional regulation skills, cognitive skills, and communication and social
skills, which impact on all areas of occupation (Richardson et al., 2015;
Richardson, Henry, Sloane & Black-Pond, 2008; Atchison, 2007; Pynoos et al.,
2008). In addition, the physical context in which the trauma occurs can greatly
impact the child’s ability to engage in an activity in the same or similar location,
due to overwhelming reminders or “trauma triggers” of the event (Petrenchik,
2015).
Personal Activities of Daily Living (PADL)
Bathing
As a result of physiological manifestations of stress, regulations of bodily
functions are compromised leading to difficulties in PADL. Stress responses are
especially impacted by children with a history of sexual abuse while being
bathed as well as being physically abused by immersion in hot or cold water.
Toileting
Encopresis, or the act of passing feces in inappropriate places such as in clothing
or other places, is known to occur more frequently among children who have
experienced sexual abuse, although it is not a specific indicator of child abuse.
Enuresis, or the repeated voiding of urine in the clothing and in inappropriate
places, often accompanies encopresis among traumatized children. Whether
intentional or not, it is essentially an expression of the child’s only mechanism of
control in the midst of complete submission to the perpetrators of abuse.
Feeding and Eating

There are many relational factors between a primary caregiver and a child with
regard to nutritional intake, beginning in the initial attachment process in
infancy. A negative nurturing relationship with an infant including lack of
attunement, irritability, depression, and other maternal problems will result in
significant maladaptive, disorganized responses. These include difficulty with
oral mechanisms such as suckling, sucking, swallowing and breathing, rhythm,
food refusal, and overactive response to certain smells and tastes. Toddlers will
often present with severe dental decay, or “bottle rot,” a condition resulting from
persistently being bottle fed with high concentrates of sugar while dental
development is taking place. Children exposed to chronic trauma are at a higher
risk for developing an eating disorder as they grow older. In a home where
physical or sexual abuse is taking place, the child may turn to an eating disorder
to gain a sense of control. Similar to the psychodynamics associated with
encopresis and enuresis, they are able to control their food intake or their weight.
Children who are compulsive eaters or those who hoard and hide food are
usually using food to help them deal with feelings of anger, sadness, hurt,
loneliness, abandonment, fear, and pain.
Personal Hygiene and Grooming
Complex trauma often results in children who lack the attention needed to
provide proper hygiene and grooming, which often comes to the attention of
school personnel and may facilitate a referral to child protective services. This
may be the result of incompetent parents who lack the intellectual requirements
to facilitate a child’s awareness of healthy grooming or willful neglect of one,
but not all, children among a set of siblings.
Sexual Activity
Reactions to chronic sexual abuse include significant dysfunction in sexual
activity including hypersexuality, which is often demonstrated by a
preoccupation with sexual organs of self, parents, and others expressed in
drawings and in language. Children with a history of molestation are seven times
more likely to become drug/alcohol dependent (Pynoos et al., 2008). In a study
on the effects of sexual abuse of 938 adolescents admitted to residential,
therapeutic communities for the treatment of substance abuse and related
disorders, 64% of the girls and 24% of the boys reported histories of sexual
abuse (Hawke, Jainchill, & DeLeon, 2000).
Instrumental Activities of Daily Living (IADLs)
Childhood maltreatment has a significant impact on IADLs. A common
occurrence among a group of siblings in an abusive and neglectful environment
is for one child, usually the oldest, to take on the role of a parent, referred to as
parentification as there is an absence of an adult care provider willing or able to
provide care for others. Oftentimes, neglected children are exposed to dangerous
in-home situations such as fire hazards, firearms, and insect and animal
infestation from parents operating methamphetamine labs. Parents might become
physically or mentally unable to care for a child. Other times, alcohol or drug
abuse may seriously impair judgment and the ability to keep a child safe.
Rest and Sleep
Exposure to trauma often results in substantial difficulty with rest and sleep
patterns as the child often exhibits hypervigilance and overactive. Multiple
exposures to violence and trauma result in autonomic and endocrine
hyperarousal, which are observed overreactions to stimuli. This may include
being easily startled and craving high-risk, stimulating, or dangerous activity, all
of which impair the balance of play, work, and rest. The NCTSN clinician
survey (Pynoos et al., 2008) found that 73% of children with complex trauma
histories experienced sleep disturbances, a finding that has been supported by
additional studies (Egger et al., 1999; Glod et al., 1997; Noll et al., 2006). While
not a common occurrence, victims of chronic abuse may experience “sleep
terrors,” which are also called “night terrors.” In a typical episode, an individual
will sit up in bed and begin to scream or shout, which may include kicking and
thrashing. The child may say or shout nonsensible exclamations with an intense
fearful expression with eyes wide open and heart racing. In addition, the child
may sweat, breathe heavily, and be very tense. Evidence further indicates
increased bed-wetting among complexly traumatized children (Petrenchik, 2015,
cited in Humphreys et al., 2009).
Education
In a study that included a sample of 9,336 children receiving trauma intervention
across the United States, 41% had academic problems including behavior
problems in school settings, including preschool programs (Pynoos et al., 2008).
Academic functioning is a significant area of developmental competence
beginning with preschool to higher education. In addition to intellectual abilities,
success is significantly tied to a child’s ability to regulate internal events or
experience and to effectively interact with peers and teachers. By preschool,
trauma-exposed children demonstrate problems in both of these areas as
demonstrated by poor frustration tolerance, a higher incidence of anger and
noncompliance, and significantly higher dependency on others for support (Cook
et al., 2005; Egeland et al., 1983; Vondra, Barnett, & Chichetti, 1990). In
elementary school, children are more likely to avoid challenging tasks, and thus
are overly reliance on teachers’ guidance and feedback (Shonk & Cicchetti,
2001). Complexly traumatized children may further demonstrate negative
attention-seeking behaviors, poor attendance, and incomplete homework
assignments (Petrenchik, 2015). By middle school and high school, they are
more likely to be considered having a lack of motivation and learning below
average, and there is a higher incidence of disciplinary referrals and suspensions
(Eckenrode, Laird, & Doris, 1993). Developmental delays and
emotional/behavioral dysregulation, learning disabilities, and intellectual
impairment that cannot be accounted for by neurological or other factors that are
experienced by children exposed to trauma can profoundly affect their school
performance (Cook et al., 2005). A cycle is often created whereby a student’s
lack of success in school reduces his or her self-esteem and increases a lethargic
response that is often perceived by adults as laziness or willful disobedience. A
variety of school intervention programs, with a focus on creating trauma-
informed or trauma-sensitive classrooms, have been utilized and are described in
the literature as a means of addressing these students’ needs (Atchison, 2011;
Macy, Macy, Gross, & Brighton, 2003; Stein et al., 2003). The key components
of a trauma-sensitive classroom that emerge from descriptions of intervention
programs for children exposed to chronic trauma include
Establishing and maintaining a safe classroom

Predictability
Acquiring affective regulation skills through positive adult interactions
Considering the classroom’s level of sensory input (i.e., bright-colored
posters, objects hanging and moving from the ceiling, noise volume, and
rich-stimulating scents).
Assistance in making meaning of students’ experiences
Unfortunately, there exists a paucity of awareness in most school systems

regarding the need for classroom-based intervention that addresses the child as
the victim of chronic abuse. The child, because of the many challenges he or she
has in response to trauma experiences, acts out and behaves in ways that are
interpreted as willful and thus being labeled as oppositional defiant or other
diagnoses that lay blame solely on the child, leading to continuous conflicts with
school personnel. The lack of appropriate intervention leads to academic
underperformance, nonattendance, disciplinary problems, a high dropout rate,
and failure to complete diplomas, which ultimately leads to poor success in
vocational pursuits.
Work
Persons exposed to reoccurring early childhood trauma typically show
disinterest in work, with ill-defined employment interests and poor employment-
seeking skills or vocational interests. Acquisition of job performance skills, due
to the lack of antecedent skills that typically emerge from home as well as
academic environment, leads to the inability to get or keep jobs. Persistent
conflict with coworkers or supervisors is also a significant barrier to successful
work performance. Other factors, which influence low work performance and
employment, include impaired emotional regulation during stressful situations
and an absence of self-awareness as noted by a lack of appropriate hygiene and
social skills (Petrenchik, 2015).
Play and Leisure
The lack of acquisition in the components of interpersonal competence, poor
self-concept, difficulty with social communication, sensory processing disorders,
and intellectual impairment have a significant impact on play exploration,
constructive play, and symbolic play. Typical early childhood behavior among
those exposed to complex trauma will often reflect traumatic events, which may
include sexual acting out or violent play with dolls, seemingly disorganized and
nonpurposeful interaction with items that may indicate a reenactment of events.
Persistent themes may be noted as the child is essentially reliving the event in an
attempt to control or gain mastery over fears that continue to create fear or that
overwhelm the child. The child may be easily triggered by environmental
stimuli, including other children and adults, which result in rage and physical
aggression during play sessions with other children or alone (Petrenchik, 2015).
As the child transitions to adolescence and adulthood, the pursuit of leisure
interests is compromised due to these maladaptive play behaviors.
In a sample of 9,336 children with a history of trauma who were receiving
intervention across the United States, 48% were reported to have had difficulty
with social engagement within the home and in the community (Pynoos et al.,
2008). Within peer groups, there is a significant degree of isolation, deviant
affiliations, persistent physical or emotional conflict, avoidance/passivity,
involvement in violence or unsafe acts, and age-inappropriate affiliations or style
of interaction. Family interaction is marked by interpersonal conflict,
avoidance/passivity, running away, detachment and surrogate replacements,
attempts to physically or emotionally hurt family members, or nonfulfillment of
responsibilities within the family. Many individuals with complex trauma
histories are at high risk for dysfunctional social participation within their
communities. This includes a high incidence of arrests and recidivism, detention,
convictions, incarceration, violation of probation or other court orders,
increasingly severe offenses, crimes against other persons, and disregard or
contempt for the law and conventional moral standards (Pynoos et al., 2008). In
addition, any inappropriate, avoiding, or aggressive behaviors as a result of poor
mental health can greatly impact the child’s ability to develop and maintain
healthy peer relationships (Petrenchik, 2015).
CASE-STUDY 1
Meghan is a 6-year-5-month-old girl who has been living with her
stepgrandmother and infant stepbrother for over a year as a foster
placement. Primary concerns for Meghan include her emotional outburst,
which often results in Meghan throwing objects and breaking toys. In
addition, Meghan has been displaying highly sexualized behaviors toward
her stepbrother. Meghan was removed from her biological father and
stepmother due to chronic neglect, physical and sexual abuse, and exposure
to drugs and domestic violence. On one occasion, Meghan once was forced
to put her soiled pants on her head, then in her mouth, and eventually forced
to swallow some of her own feces. The stepgrandmother noted that Meghan
does not have any contact with her biological mother. Meghan’s father was
attending weekly visits with her and then suddenly stopped showing up.
Meghan frequently states she misses her father, and the stepgrandmother
noted that Meghan was making good progress until her father stopped
attending visits. The stepgrandmother did not have any information related
to Meghan’s prenatal care, development, and medical history but stated her
biological mother and father both had a history of drug and alcohol abuse.
Currently, Meghan does not take medication or display signs of fine and
gross motor challenges. However, Meghan is struggling academically as
indicated by her inability to read and write her name, follow multistep
directions, and problem-solve efficiently. Her difficulty with sustained
attention has also been observed.
Meghan demonstrates irregular sleep patterns causing her to wake in
the middle of the night, which occasionally results in her kicking her
bedroom walls and dismantling her room. Meghan has verbally expressed
aggression by telling her stepgrandmother that she was going to shoot
people and stab her stepbrother. She lacks the awareness of “stranger
danger” and continues to display sexualized behaviors as evident by talking
to her dolls in a sexual manner and has removed the diaper from her
stepbrother and proceeded to touch him inappropriately.
CASE-STUDY 2
David is an 11-year-9-month-old adolescent male residing with his adoptive
mother, her fiancé, and adoptive 4-year-old sister. At 2 years old, David was
placed with his adoptive mother as a foster placement and then adopted by
her 1 year later at the age of 3 years. Prior to being adopted, David resided
in five different foster homes. The adoptive mother’s concerns for David
include aggression and lack of remorse as evident by David threatening to
suffocate his 4-year-old sister with a pillow a few days prior to the start of
the new school year. The incident resulted in a 5-day inpatient stay at a
mental health facility followed by outpatient appointments at Community
Mental Health. Currently, David wears a hat due to trichotillomania, a
compulsive disorder in which a person constantly pulls out his or her hair.
His other diagnoses include fetal alcohol syndrome (FAS) and obsessive-
compulsive disorder (COD).
David was born 2 months premature and removed from his biological
mother at birth as a result of testing positive for cocaine in his system. It is
unknown who David’s father is as his biological mother reportedly has had
frequent multiple sex partners. David last visited his biological mother at
her house when he was 7 years of age in the company of his adoptive
mother. The visit was brief as a result of excessive use of alcohol by
individuals in the home, including his biological mother.
David’s adoptive mother reports that he is a good reader and speller and
enjoys puzzles and drawing. His drawings are frequently similar to that of
an elementary child. David’s adopted mother further states that he has a
first grade math competency level and demonstrates a limited
understanding of cause and effect and difficulty with time orientation. His
recent full scale IQ score was 68. His problem-solving skills are limited to
simple challenges. David is suspected of having some sensory processing
challenges, specifically in regard to sounds as noted by his aggressive
response toward individuals making noise he perceives as threatening and
irritating. When David is upset, he yells, slams doors, and attacks his sister
and mother. David is easily frustrated and does not process his emotions
well. His adoptive mother states she has rarely seen him cry. David does
laugh and smile at times but most often presents with a flat affect.
RESOURCES
American Professional Society on the Abuse of Children (APSAC) 350 Poplar
Avenue
CHO 3B-3406
Elmhurst , IL 60126
877-402-7722
www.apsac.org
The Annie E. Casey Foundation
701 St. Paul Street
Baltimore, MD 21202
The Child Trauma Academy
5161 San Felipe, Suite 320
Houston, TX 77056
866-943-9779
www.childtrauma.org
The Doris Duke Charitable Foundation 650 Fifth Avenue, 19th Floor
New York , NY 10019
212-974-7000
www.ddcf.org
National Children’s Traumatic Stress Network Center for Mental Health
Services
Substance Abuse and Mental Health Services Administration Department of
Health and Human Services 5600 Fishers Lane
Parklawn Building, Room 17C-26
Rockville, MD 20857
www.nctsn.org
The National Institute for Trauma and Loss in Children 42855 Garfield Road
Suite 111
Clinton Township, MI 48038
www.starrtraining.org/contact-tlc
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C H A P T E R
13 Obsessive-Compulsive and
Related Disorders
Cynthia A. Grapczynski
KEY TERMS
Obsessive-compulsive disorder Body dysmorphic disorder Hair-pulling disorder
Skin-picking disorder Hoarding disorder Obsessions
Compulsions
Repetitive behaviors
Trichotillomania
Excoriation
Betsy, a 23-year-old on-again–off-again student, waited impatiently for the
occupational therapist to contact her. Betsy has been admitted to an outpatient
mental health facility to begin treatment for her obsessive-compulsive disorder
(OCD). Betsy’s OCD began when she was about 12 years old, coming on quite
suddenly and quickly becoming debilitating. Betsy’s OCD is related to
contamination issues, and she cannot touch anything that she has not cleaned
herself, including food, and she showers four to five times a day, with
intermittent hand washing between showers. As a result, Betsy’s appearance is
disheveled; her hair is dry and flyaway; her hands and face are dry and scaly
with some spots reddened and some bleeding. She wears the same clothes all the
time because these are the only clothes she believes have been decontaminated
appropriately. Because she will only eat food she has cleaned and prepared for
herself, Betsy is very thin; she is also very tired from the compulsive ritualistic
behaviors that take up so much of her time. Betsy completed high school under
great duress, with many absences due to her compulsions. She has attempted
college, but her compulsive behaviors were so demanding that she has had to
drop out.The occupational therapist is a member of the team that will be treating
Betsy for OCD with cognitive-behavioral therapy and exposure response
therapy. Attempts have been made before to treat Betsy, but she has been unable
to complete the program. This is her third attempt, and she is hoping to be able
to be successful this time. The occupational therapist is supposed to work with
Betsy on relaxation therapy, mindfulness, and energy conservation, of which
Betsy is skeptical. Betsy believes that her thoughts are out of her control and the
anxiety she experiences because of her obsessions is so profound that she does
not believe there is any hope for her to ever find relief. She believes that no one
understands the extent of her suffering, and she is often short-tempered with
family and others. Betsy has very few friends for this reason and the fact that her
hand washing is disruptive to any social activities in which she might engage.
Obsessive-compulsive disorder (OCD) is the primary disorder identified in the
Diagnostic and Statistical Manual of Mental Disorders, 5th Edition [DSM-5]
(American Psychological Association [APA], 2013) category of Obsessive-
Compulsive and Related Disorders (OCRDs). The DSM-5 has made changes in
the description and the classification of OCD, one of which is that OCD is now
leading the new diagnostic category of OCRDs. A second significant change is
that the DSM-5 does not require that people recognize the senselessness or
excesses of their obsessions and compulsions. However, it does allow a way to
distinguish degree of insight or lack thereof in OCD, through the use of
specifiers, which may help to improve differential diagnoses (Abramowitz &
Jacoby, 2014). These changes also reflect more precisely clinical symptoms and
distinguish severity among symptoms (Schieber, Kollei, de Zwaan, & Martin,
2015; Snorrason, Stein, & Woods, 2013).
OCRDs include all disorders marked by obsessive thoughts and/or repetitive
behaviors, including OCD, and five other related disorders. The related disorders
included in the OCRD category include (a) body dysmorphic disorder (BDD),
(b) hoarding disorder, (c) hair-pulling disorder, (d) skin-picking disorder,
and (e) other specified and OCRDs (APA, 2013). The related disorders are
included due to similarities of symptoms and characteristics. These include the
following: (a) some empirical evidence that supports inclusion; (b) the primary
symptom of all these disorders is repetitive behaviors; (c) all have similar age of
onset, comorbidity patterns, and family loading; (d) all share brain circuitry and
neurotransmitter abnormalities; and (e) all have overlapping treatment response
profiles (Abramowitz & Jacoby, 2014; Schieber et al., 2015). Each of these
OCRDs will be discussed individually.
Obsessive-Compulsive Disorder
OCD is a common, heterogeneous, neuropsychiatric disorder that is experienced
by both adults and children (Grant, 2014; National Alliance on Mental Illness
[NAMI], 2013). The hallmark of this disorder is the presence of obsessions
and/or compulsions that cause anxiety and interfere with daily functioning and
relationships (NAMI, 2013). Obsessions consist of persistent, unwanted,
intrusive thoughts that can only be resolved, mostly, but not always, by
compulsions, which are irrational excessive behaviors or mental acts repeated
again and again. These repetitive behaviors are expected to resist or somehow
control or banish obsessive thoughts to prevent feared consequences
(Abramowitz & Jacoby, 2014; Bokor & Anderson, 2014; Jukel, Siebers, Kienast,
& Mavrogiorgou, 2014; NAMI, 2013). These obsessions and/or compulsions
must be upsetting, create problems in the completion of daily life activities, and
last at least 1 hour every day to meet the DSM-5 criteria.
Body Dysmorphic Disorder
BDD presents as a severe preoccupation with a perceived defect in physical
appearance that results in distress and serious social or occupational functioning
impairment (Schieber et al., 2015; Veale & Bewley, 2015). Although the
perceived flaw may be a normal variation, or appear slight to the objective
observer, it nevertheless causes “…enormous shame or interference in a person’s
life” (Veale & Bewley, 2015, p. 2278). Additionally, BDD can result in bodily
harm, due to overexercise and unnecessary surgeries (NAMI, 2013).
The obsession with the physical aspect causes repetitive behaviors or
mental acts such as mirror checking, rumination, and camouflaging, to hide or
distract from the perceived defect, which, on average, occur 3 to 8 hours per day
(Phillips, 2014). While BDD is considered a significant psychiatric diagnosis, it
is seldom seen in the mental health clinic unless there is co-occurring
depression, inability to leave the home, or risk of suicide (Veale & Bewley,
2015). According to Pavan et al. (2008), the individual with BDD focuses
emotionally charged attention on a specific part of the body. This body part is so
despised or disgusting to the individual that this focus can interfere with
relationships and social functioning, damage the self-esteem, and severely
impact quality of life (Buhlmann, Teachman, Gerbershagen, Kikul, & Rief,
2008; Marques et al., 2011). Comorbidities include OCD, depression (80%
lifetime), substance abuse, and suicidal ideation, to name the most common
(Pavan et al., 2008).
Hoarding Disorder
Hoarding disorder (HD) is a condition that impedes the ability of an individual
to discard apparently unnecessary possessions, value notwithstanding, and
creates clutter that interferes with the safe use of the living space (Nordsletten &
Mataix-Cols, 2014; Saxena et al., 2011). Extreme distress is created by the idea
of throwing anything away, and can make the living space unhealthy (NAMI,
2013; Saxena et al., 2011). The accumulation of articles is often disorganized,
substantially compromising intended use of living space, keeping individuals
from sleeping in their bed, sitting in the living room, or cooking in the kitchen
(Nordsletten & Mataix-Cols, 2014; Saxena et al., 2011).
Individuals with this condition often have poor insight and are not open to
seeking help due to the distress caused by the thought of discarding anything,
despite serious social and financial distress (Mataix-Cols, 2014; NAMI, 2013).
HD can be associated with OCD, but more often is found to appear
independently. Individuals with HD show higher levels of disability related to
work and relationships (Saxena et al., 2011). Older individuals with HD
frequently have worse general health and more medical problems of a chronic
nature as well (Nordsletten & Mataix-Cols, 2014; Saxena et al., 2011).
Hair-Pulling Disorder
Hair-pulling disorder (HPD), also known as trichotillomania, is a disorder in
which individuals engage in repeatedly pulling out their hair, resulting in
significant hair loss or thinning of the hair (Ricketts, Brandt, & Woods, 2012).
This hair pulling is self-induced and recurrent and is a result of an “increasing
sense of tension” before the pulling is begun (Jacob, 2013, p. 162). Hair can be
pulled out with the thumb and forefinger, or it can be scratched or rubbed out, or
various implements can be used such as tweezers, pencils, and hairbrushes—
even needles in some extreme cases (Jacob, 2013).
HPD creates significant psychosocial impairment and may create physical
problems associated with eating the hair that can be dangerous (NAMI, 2013;
Tung, Flessner, Grant, & Keuthen, 2015). Medical complications can occur, such
as infection, permanent hair loss, and gastrointestinal obstruction in the event
that the hair is ingested and hairballs are formed. Psychosocial domains are also
affected including self-esteem, body image, emotions, and social functioning
(Jacob, 2013; Ricketts et al., 2012). The disorder is recognized as one that has a
significant stigma attached because it is both visible to others and is perceived to
be a disorder that can be controlled by the individual (Ricketts et al., 2012).
Skin-Picking Disorder
Skin-picking disorder (SPD), also known as excoriation, is the presence of
recurrent skin picking resulting in noticeable skin lesions. Individuals with this
disorder pick at their skin relentlessly, to the extent that it may use up several
hours during the day. People with this disorder can be seen to have open wounds
on their bodies, with myriad scabs, scars, and tissue damage from previous
picking (Craig-Muller & Reichenberg, 2015; Grant et al., 2012).
SPD, left untreated, can lead to other dermatologic problems, including
infection and even the need for amputation. Triggers to skin picking can include
stress, anxiety, boredom, anger, and/or a perception of a blemish by the
individual (Craig-Muller & Reichenberg, 2015). SPD often co-occurs with
related disorders such as BDD and hair-pulling disorder, and many individuals
with SPD are found to have other lifelong psychiatric disorders as well (Odlaug,
Hampshire, Chamberlain, & Grant, 2015).
Etiology
The etiology of the OCRDs is unclear and complex in all types of these
disorders. There do appear to be genetic, neurobiological, and sociocultural
factors that work together in the development of these disorders; however, each
disorder has a unique combination of factors identified (NAMI, 2013; Pavan et
al., 2008; Saxena et al., 2011).
The etiology of OCD is unknown, and there is no way to tell which symptoms or
specific elements of OCD will predict later development of the condition (Jukel
et al., 2014). There is some information to indicate that genetics may play a part
in this illness, to the extent that if a parent or sibling has the condition, there is a
25% chance that an individual will develop it as well (NAMI, 2013).
Additionally, there are indicators that brain activity in several different areas of
the OCD brain is abnormal due to a unique response to serotonin. A single gene
has not been isolated, but rather genes that may affect serotonin, glutamate, and
dopamine appear to be involved (Bokor & Anderson, 2014).
OCD can also occur in the wake of physical or physiological damages to the
body. For example, a stroke affecting the basal ganglia, caudate, and posterior
frontal lobes, or a serious traumatic injury (accident or experience of violence),
and occasionally pregnancy can bring on symptoms of OCD. Psychosocial
trauma, particularly related to posttraumatic stress disorder (PTSD), can bring on
OCD that includes a high rate of self-injurious behavior, panic disorder, and
compulsive buying (Bokor & Anderson, 2014). Pediatric autoimmune
neuropsychiatric disorder associated with streptococcus (PANDAS) can also be
related to the onset or the exacerbation of OCD symptoms in children, along
with a variety of other symptoms such as separation anxiety and mood disorders.
Occasionally, exposure to GABHS, a type of streptococcus bacteria that causes
Sydenham chorea, another neurobiological disorder of childhood, can result in
symptoms of OCD, along with irregular involuntary movements seen in choreic
conditions (Bokor & Anderson, 2014). Any of these insults to the system can
cause hyperactivity in the orbitofrontal cortex, anterior cingulate gyrus,
thalamus, and striatum, leading to OCD (Seibell, Hamblin, & Hollander, 2015).
The cause of BDD is unknown. Research, although inconsistent, indicates that
these individuals may have abnormalities in visual processing, executive
functioning, and difficulty recognizing emotional facial expressions. These
abnormalities result in the individual actually seeing things differently from
others (Phillips, 2014). Specifically, there appears to be impairment in the brain
circuitry at the frontostriatal and temporoparietal-occipital areas, which are
related to how facial images and emotional information are processed (Pavan et
al., 2008). These abnormalities indicate less effective connections in various
areas of the brain leading to poor insight about the source or reality of BDD
(Phillips, 2014).
Additionally, in the sociocultural realm, individuals with BDD appear to
have unhealthy attitudes associated with physical appearance and maladaptive
beliefs about the importance of appearance. Many of these attitudes and beliefs
appear to be based on past teasing or childhood neglect/abuse (Buhlmann et al.,
2008; Phillips, 2014). All of these elements, when taken together, suggest a
multifactorial causation and complex clinical picture in BDD (NAMI, 2013;
Phillips, 2014).
Hoarding Disorder
The cause of HD appears to be genetic, without specific genes identified for
about 50% of compulsive hoarding; however, it is still considered to have a
familial component (Gilliam & Tolin, 2010). Research is inconsistent when
considering specific genes that predispose individuals to hoarding. One common
factor seen in HD is high rates of traumatic events and/or abuse in childhood,
sometimes reported by those individuals diagnosed at a later age, that appears to
precede onset, but this has not been empirically identified as a definitive cause of
HD (Mataix-Cols, 2014).
The presence of other disorders such as brain lesions, autism spectrum
disorders, and dementia can present with symptoms of HD. Research, however,
indicates no specific relationship to these other disorders. HD appears to be
independent of these and other obsessive-compulsive disorders, with more
global impairment noted in HD and an overall poorer response to treatment than
these other disorders (Gilliam & Tolin, 2010; Nordsletten & Mataix-Cols, 2014;
Saxena et al., 2011).
The cause of HPD is not known; however, research in the last 10 years suggests
both biological and behavioral factors are involved, with a relationship to certain
abnormalities in the brain including alteration in frontostriatal thalamic
pathways. These abnormalities are similar to those found in SPD and OCD;
however, there appear to be other areas of the brain that are involved in HPD
(Roos, Fouche, Stein, & Lochner, 2015).
Behavioral theorists suggest a strong influence from sociocultural issues may
also be part of the etiology of HPD. Issues related to family conflicts, stress, or
depression may result in HPD as a learned response that ultimately develops into
a habit (Jacob, 2013). The relationship of HPD to SPD may be quite strong, as
SPD is frequently a co-occurring disorder in approximately 38% of those with
HPD (Grant et al., 2012; Ricketts et al., 2012).
SPD’s etiological information is scarcer than that found in the other OCRDs, but
also does suggest an underlying neurological dysfunction. Specifically, recent
imaging studies showed “…abnormalities in white matter tracts, involving top
down motor generation and suppression…” (Snorrason et al., 2013, p. 406). This
white matter is close to the anterior cingulate cortices, and the abnormalities
seen are quite similar to those found in hair-pulling disorder (Odlaug et al.,
2015).
A recent functional imaging study conducted with individuals having skin-
picking disorder showed “…significant underactivation in distributed neural
circuitry including the bilateral dorsal striatum, bilateral anterior cingulate, and
right frontal regions” of the brain (Odlaug et al., 2015, p. 5). These results
suggest that SPD is associated with both structural and functional abnormalities
in the areas of the brain related to the development of habits, monitoring action,
and control of inhibitory processes (Odlaug et al., 2015).
OCD occurs in roughly 1% to 2% of the US population. Similar rates are noted
in the United Kingdom, Canada, Puerto Rico, German, Korea, and New Zealand,
but Taiwan has a slightly lower prevalence (Soomro, 2012). However, a recent
study of OCD in Singapore (Subramaniam, Abdin, Vaingankar, & Chong, 2012)
showed a lifetime prevalence of 3.0% and a 1-year prevalence of 1.1%. In
Singapore, being younger and being divorced or separated had a higher
association with OCD than did others in the study.
Males appear to be more often affected in childhood, but women are more
often affected in adulthood (Seibell & Hollander, 2014). According to Seibell
(2015), OCD is “…one of the top 20 causes of illness-related disability
worldwide for individuals between the ages of 18 and 44 years” (p. 290). Seibell
et al. (2015) noted that many individuals with OCD endure their condition in
silence, as individuals average 9 years in treatment before the appropriate
therapeutic treatment can be obtained.
Risk factors for OCD include a family history of the condition, being single,
and being in a higher socioeconomic category. Additionally, individuals who
have anxiety, depression, alcohol/substance abuse, and eating disorders may
have an underlying OCD. More than 2% of the US population is expected to
develop OCD in their lifetimes (Johnson & Blair-West, 2013; NAMI, 2013).
BDD is considered a fairly common disorder in the general population. In the
United States, prevalence was found to be approximately 2.4%; in Germany,
prevalence was recently found to be 1.7% to 1.8% (Marques et al., 2011;
Schieber et al., 2015). These rates for BDD are higher than those for either
anorexia nervosa or schizophrenia and occur equally in both sexes (Veale &
Bewley, 2015). According to Phillips (2014), BDD rates for suicidal ideation,
suicidal attempts, and completed suicides are comparatively high, but treatment
research on this disorder is significantly behind that for similarly common and
serious disorders.
BDD is frequently comorbid with a major depressive disorder, poor insight,
social phobia, and information processing differences. BDD can be
misdiagnosed as an eating disorder if the body preoccupation relates to weight,
as the mean age of onset is 16.4 years, and a number of studies indicate that
BDD is common among students, both male and female. Research has indicated
that many individuals report sensitivity related to their appearance throughout
their lives, with up to 80% experiencing depressive disorders and 78%
experiencing suicidal ideation (Pavan et al., 2008; Phillips, 2014; Veale &
Bewley, 2015).
Hoarding Disorder
Reports of prevalence of hoarding disorder (HD) varied widely in previous
publications, as clinically significant compulsive hoarding is identified as quite
common. The most recent research indicated a population prevalence of 1.3% to
1.5% in the United States (Nordsletten et al., 2013). Comorbidities of HD
include anxiety disorder (~75%) and attention deficit hyperactivity disorder,
along with a greater number of chronic medical conditions. The majority of
people with HD report for treatment in their 50s despite the fact that symptoms
may appear decades earlier (Mataix-Cols, 2014; Saxena et al., 2011).
Risk factors related to HD appear to include having a family member with
the same disorder, stressful and/or traumatic life events, and environmental
influences. There is not adequate research to support the popular idea that
material deprivation in childhood creates a predisposition to developing
hoarding disorder (Mataix-Cols, 2014).
Hair-pulling disorder (HPD) is a severely underresearched and poorly
understood condition, and prevalence has not been established in the pediatric
population, although the condition appears between the ages of 7 and 14
(Harrison & Franklin, 2012; Kaplan, 2012). The condition is more common in
women than in men, with a total lifetime prevalence of 0.6% to 3.4% and a 1-
year prevalence of 1% to 2%, especially among college-age individuals
(Harrison & Franklin, 2012; Jacob, 2013; Kaplan, 2012; Rogers et al., 2014).
HPD is seen comorbidly in up to 60% of reported cases, most often with
depression, generalized anxiety, and OCD. A recent study revealed that severity
of depression was a major predictor of life disability in HPD, along with
avoidance and emotional distress (Tung et al., 2015). Only 5% of individuals
with HPD report any relationship to posttraumatic stress disorder (Kaplan,
2012).
Skin-picking disorder has only recently been recognized as an independent
syndrome, and research on prevalence has been limited. Past prevalence reports
estimated SPD to range from 1.4% to 1.5%, with the age of onset usually
between 30 and 40 years of age (Craig-Muller & Reichenberg, 2015; Odlaug et
al., 2015). A recent study by Monzani et al. (2012) looked at prevalence of SPD
among a sample of 2,518 twins in the United Kingdom and established
prevalence with empirically derived cutoffs. Results indicated that SPD was a
“…relatively prevalent problem, particularly among women…” (p. 605), with a
prevalence of 1.2% among the twins, who were all female.
Among those with SPD, there is a higher rate of the presence of other
grooming disorders. A comorbidity of BDD is pronounced in SPD, with research
estimates between 32% and 44.9% (Grant et al., 2012).
Signs and Symptoms
The primary symptoms of OCD include intrusive, irrational thoughts or impulses
that occur repeatedly, known as obsessions, and repetitive acts that briefly
relieve the stress associated with obsessive thoughts, known as compulsions.
These obsessive thoughts cause great anxiety in the affected individual, and the
repetitive acts, some of which are ritualistic, are attempts to reduce the anxiety
by neutralizing the obsessive thought (Abramowitz & Jacoby, 2014). The
obsessive/compulsive cycle can be vicious, generally lasting more than 1 hour,
intensifying the disease process and significantly interfering with daily life
processes (NAMI, 2013; Seibell & Hollander, 2014).
Leonard and Reimann (2012) point out that it is important to listen carefully
to patients for potential compulsions that may not be as rule driven, prolonged,
or repetitive. Some mental acts or subtle behaviors may be completed in
response to an obsessive thought of which the patient is unaware. Furthermore,
according to Johnson and Blair-West (2013), “any recurrent anxiety-provoking
thoughts that are seen as intrusive and involuntary, and are recognized as
exaggerated, excessive, or against the person’s own belief system should be
reviewed as possible OCD symptoms” (p. 607).
Symptoms of OCD begin in childhood, teen, and young adult years and
include common categories or dimensions. These include (a) contamination,
related to washing and cleaning rituals; (b) symmetry, related to ordering and
positioning rituals; (c) counting, related to the need to complete a behavior a
specific number of times; (d) harm, related to the fear of hurting others; and (e)
forbidden thoughts/images/scrupulosity, related to sex, violence, and religion
(Johnson & Blair-West, 2013; Seibell & Hollander, 2014). Also, while OCD
appears similar across cultures, the culture may have an influence on the content
of obsessions and compulsions. For example, studies show more aggressive and
religious obsessions in the Middle East and Brazil (Grant, 2014). Individuals
with OCD may also show deficits in executive functioning, motor function
impulsivity, and cognitive flexibility.
The primary symptom of BDD is a highly emotionally charged focus on a
particular part of the body, where an individual identifies one or more perceived
flaws that are described as “ugly,” “hideous,” and/or otherwise intolerable
(Pavan et al., 2008; Phillips, 2014). Preoccupation with the offending body part
lasts for 3 to 8 hours per day and results in repetitive behaviors such as constant
mirror checking, excessive grooming, reassurance seeking, and camouflaging of
the defect. Furthermore, the exaggerated preoccupation with the defect produces
a disproportionate emotional reaction that is akin to shame. Individuals with
BDD also report a pervasive sense of inadequacy about their bodies, and the
anxiety produced with social encounters can be debilitating, inhibiting social life
and occupational activities, such as school or work (Buhlmann et al., 2008;
Veale & Bewley, 2015). Self-esteem is noted to be seriously eroded, as well
(Phillips, 2014).
While the repetitive behaviors seen in BDD are similar to those found with
OCD, there are differences to note. For example, some compulsions in BDD can
consist of mental acts, such as comparing oneself with others. In the use of
camouflage, the affected individual will check and recheck the camouflage (e.g.,
makeup, hair, clothing) to ensure it is still hiding the defect. Additionally, there
is evidence that individuals with BDD have maladaptive beliefs about the
importance of appearance in life, and self-referential thinking, along with
accessory symptoms such as reference ideas and abnormal tactile sensations
(Pavan et al., 2008; Rieke & Anderson, 2009).
Hoarding Disorder
Hoarding is exemplified by an obsessional fear of losing items of significance
that may become important at a later time, along with a disproportionate
emotional attachment to possessions (Saxena et al., 2011). Symptoms include the
urge to save things, difficulty discarding things, excessive acquisition,
disorganization of items with no cohesive theme, and clutter to the point that
activities of daily living are impaired. Function is significantly impaired as well,
and there is emotional distress tied both to difficulties with function and with the
idea of discarding any possessions (Mataix-Cols, 2014; Nordsletten & Mataix-
Cols, 2014; Saxena et al., 2011). Additional symptoms noted include deficits in
attention, decision making, and categorization (Gilliam & Tolin, 2010).
Symptoms occur early in life and generally increase in severity over time.
Poor insight among those with HD further interferes with the individual’s ability
to see the seriousness of the situation. Symptoms that appear later in life are
often related to other medical, neuropsychiatric, or neurodegenerative diseases
(Mataix-Cols, 2014).
The primary symptom of hair-pulling disorder (HPD), also called
trichotillomania, is the self-induced removal of hair on the body. The primary
areas chosen for pulling include the scalp, the eyebrows, and eyelashes. Hair
pulling can be implemented with a variety of tools, from the thumb and
forefinger to use of tweezers and even needles to dig out hair (Jacob, 2013; Tung
et al., 2015). Episodes can occur while the individual is relaxed, known as
automatic HP, or stressed, known as focused HP, but the common denominator is
that the individual experiences an increasing sense of tension prior to actually
pulling out the hair (Jacob, 2013). Recent research indicated a complex
relationship to exist between the severity of HPD, degree of depression, and
functional impairment (Tung et al., 2015). HPD most commonly affects women,
with onset usually in adolescence (Ricketts et al., 2012).
Individuals with skin-picking disorder (SPD), also known as excoriation, spend a
significant amount of their time, on a daily basis, picking at their skin. Many
such individuals report that picking takes up several hours each day, sometimes
causing them to be late for, or miss, school, work, or social engagements (Grant
et al., 2012; Jacob, 2013). Skin picking in SPD has been shown to be repetitive
to the point of creating skin lesions and tissue damage that ultimately causes
emotional distress and functional impairment in social, occupational, and daily
living activities. SPD can lead to complicated dermatologic conditions, scarring,
infection, and need for amputation in severe cases (Craig-Muller & Reichenberg,
2015; Grant et al., 2012).
Factors that may trigger the urge to pick include stress, anxiety, boredom,
fatigue, and anger. Sometimes, the feel or look of the skin may trigger picking,
and emotional regulation or reactivity has been shown to predict picking activity
(Grant et al., 2012).
OCD is responsible for significant suffering, impairment, and debilitation and is
the 4th most common mental illness in the United States (Seibell et al., 2015).
OCD is often chronic and has a significant impact on social and occupational
functioning, often starting in early childhood or adolescence. Fifty percent of
patients report their first behavioral changes, such as anxiety or a lack of self-
trust, appearing before the age of 20. Later onset is associated with increased
feelings of responsibility and exact attention to details, along with a need for
order and cleanliness, difficulty with decision making, and often a later diagnosis
of depression (Jukel et al., 2014).
A serious problem with the disorder is that of misdiagnosis, related to the
shame produced by the illness, and lack of recognition. OCD is often comorbid
with other psychiatric diagnoses such as schizophrenia, Tourette’s syndrome,
posttraumatic stress disorder, generalized anxiety disorder, and some
neurological disorders, making diagnosis complex (Bokor & Anderson, 2014).
Many patients go for up to 9 to 10 years before the condition is correctly
diagnosed, with an average of 17 years elapsing before appropriate treatment is
initiated (Seibell et al., 2015; Subramaniam et al., 2012).
Unless OCD is treated, the course of the illness is chronic, although
symptoms can wax and wane. However, remission rates are low (20%) without
treatment, and occupational and social impairment is the norm (Jukel et al.,
2014; Seibell et al., 2015). The course of the illness with treatment has been
shown to be shorter in duration and to have higher rates of symptom remission
(up to 40%) and an enhanced quality of life (Jukel et al., 2014; Wheaton,
Schwartz, Pascucci, & Simpson, 2015).
OCD can also have an episodic course, appearing during the initial years of
the disease; however, the chronic course is more common after that. Over time,
almost half of individuals with OCD experience some symptom improvement.
Research indicates that approximately 24% of individuals experience a partial
remission after about 2 years. In children, however, “…the rate of persistent, full
OCD was 41%…with greater persistence, related to earlier onset, longer
duration and a history of inpatient treatment” (Soomro, 2012, p. 3). A case report
by Sinha, Bakhla, Patnaik, and Chaudhury (2014) described a seasonal course of
OCD, with symptoms appearing in October and resolving themselves by April or
May. The patient did experience an increase in severity of symptoms over time
and was treated with a combination of exposure response prevention therapy
(ERP) and phototherapy. This combined 14-day therapy resulted in “…complete
remission of OCD symptoms” (p. 161).
BDD is a serious mental health disorder, but seldom seen clinically unless there
is a comorbid disorder of depression, anxiety, or risk of suicide (Veale &
Bewley, 2015). Despite this fact, individuals with BDD are substantial
consumers of healthcare resources, but are seldom satisfied with their
experiences. The reason for this dissatisfaction, according to Pavan et al. (2008),
is because these individuals usually present themselves to dermatologists and
plastic surgeons to resolve their perceived physical deficits, often associated with
distaste, decreased social functioning, and psychological discomfort. Without
appropriate treatment, however, BDD is a chronic malady that is “…associated
with markedly poor functioning and quality of life and with high rates of
suicidality” (Phillips, Menard, Quinn, Didie, & Stout, 2012, p. 1109). Some
researchers, however, report that individuals with BDD, whether treated or not,
show little to no difference in quality of life or functional disability (Marques et
al., 2011).
Despite the commonality of BDD (1.7% to 2.4% nationwide), little is known
about the course of the illness or any predictors of the course. A recent
prospective naturalistic study of BDD’s course and predictors over 4 years, by
Phillips et al. (2012), revealed significant information about the course of BDD
and its predictors. The results indicated, over 4 years, with 88% receiving mental
health treatment at some time during the follow-up period: (a) Both partial
remission and no remission of BDD symptoms were more frequent than full
remission; (b) more severe BDD symptoms at intake predicted a lower chance of
remission; (c) BDD comorbid with a major depressive disorder, personality
disorder, or delusional BDD beliefs did not predict greater chronicity of BDD;
and (d) even with remission, a majority of these individuals relapsed.
Hoarding Disorder
Hoarding disorder (HD) symptoms appear in childhood and adolescence with
60% reporting symptoms by age 12 and 80% reporting symptoms by age 18
(Gilliam & Tolin, 2010). The course of hoarding disorder appears to be chronic,
with a high likelihood of comorbid mental and physical healthcare problems, as
it occurs more often with depression, social anxiety, and generalized anxiety than
with other conditions (Gilliam & Tolin, 2010; Nordsletten et al., 2013; Saxena et
al., 2011). Compulsive hoarding is associated with significant problems with
social, familial, and work-oriented activities, with the more severe hoarding
associated with lower overall income. Quality of life in HD, including safety,
daily activities, family contact, and general life satisfaction in one study, was
shown to be significantly lower than that in individuals with OCD (Saxena et al.,
2011).
A recent study Nordsletten et al. (2013) indicated that individuals with HD
were likely to be older and unmarried as well as impaired enough by a physical
or mental health condition that they could claim disability benefits. Mataix-Cols
(2014) indicated that untreated HD is chronic and becomes progressively worse
over time, with interference in daily functioning occurring at about mid-20s and
clinical significance occurring by the mid-30s. The majority of individuals with
HD appear for treatment in their mid-50s (Nordsletten & Mataix-Cols, 2014).
The course of hair-pulling disorder (HPD) is complicated by whether the hair
pulling is automatic and high focused, or low focused, as well as whether or not
there are other body-focused repetitive behaviors present (Tung et al., 2015).
Those individuals with HPD that is high focused appear to have greater
depression and stress, as do high automatic pullers (Flessner et al., 2008).
According to Tung et al. (2015), the severity of any depression in the HPD
population was a strong predictor of significant social disability, though many of
the individuals in this study were not formally diagnosed with depression.
Additional predictors of work and social disability included interference or
avoidance of activities by individuals with HPD, along with distress caused by
HPD leading to impairment in social and leisure activities, family life, and home
responsibilities. Intensity of hair pulling also influences perceptions of those
individuals with HPD (Ricketts et al., 2012).
Like most of the OCRDs, SPD has a chronic course, which worsens with time,
and is difficult to manage. The disorder may fluctuate depending on the stress
level, but continues for many decades and is consistently present (Snorrason et
al., 2013). Individuals with SPD pick at their skin daily, sometimes for several
hours, causing damage to the skin, infection, and sometimes even disfigurement
(Grant et al., 2012; Monzani et al., 2012; Snorrason et al., 2013). It is also
considered a stigmatizing disorder (Ricketts et al., 2012).
SPD is often comorbid with other psychiatric disorders and causes distress as
well as functional disability, particularly in the case of skin ulcerations and
infections, which are common (Monzani et al., 2012). Contributing to the
chronicity of the disorder is the lack of awareness of SPD and therefore lack of
appropriate treatment. Fewer than 20% of individuals with SPD report seeking
treatment. Most people with SPD report other family members having some kind
of grooming disorder, as well (Craig-Muller & Reichenberg, 2015; Grant et al.,
2012; Monzani et al., 2012).
Diagnosis
The diagnosis of OCD is generally made when an individual reports the presence
of repetitive thoughts and actions to neutralize those thoughts, occurring
repeatedly throughout the day and taking up 5 to 8 hours of time daily. Diagnosis
of OCD can be made if either obsessions or compulsions are present, despite
recommendations from the field to require both for an OCD diagnosis, to
improve diagnostic accuracy and treatment (Leonard & Reimann, 2012).
There is a specifier related to degree of insight for the individual with OCD
that allows for differentiation between OCD and other psychotic disorders, as
well as to distinguish degree of severity of OCD. This specifier separates those
who have good insight and recognize their fears as not real, from those
individuals who have poor insight and believe their obsessions may be real, and
those with no insight, who are considered to have a delusional belief that their
fears are very real (Abramowitz & Jacoby, 2014). Abramowitz and Jacoby
(2014) also note a tic-related specifier that helps to distinguish a combination of
OCD with tics, which is characterized by a physical problem that is relieved by
the motoric tic response. However, diagnosis may be difficult due to shame on
the part of the patient, the stigma associated with diagnosis of a mental disorder,
and misdiagnosis by the health care provider (Seibell et al., 2015).
A key assessment used for identifying obsessive-compulsive behaviors is the
Yale-Brown obsessive-compulsive scale (YBOCS). The YBOCS is a 10-item
semistructured questionnaire designed to measure the severity of OCD
symptoms. Both obsessions and compulsions are measured, looking at five
different parameters: (a) time, (b) interference, (c) distress, (d) resistance, and (e)
control. This measure has been shown to be reliable and valid in a number of
research studies (Conelea, Schmidt, Leonard, Riemann, & Cahill, 2012).
Other rating scales used include the National Institute of Mental Health
General Obsessive-Compulsive Scale, the Maudsley Obsessive-Compulsive
Inventory, and the Leyton Obsessional Inventory. Other mental illness scales are
also available but are not specific to OCD (Bokor & Anderson, 2014).
Furthermore, according to Bokor and Anderson (2014), obsessions and
compulsions must consume a minimum of 1 hour per day, must not be a
response to another medication or to substance abuse, and must not be due to
any other psychiatric disorders.
Key diagnostic criteria for BDD to meet DSM-5 criteria include three factors: (a)
significant preoccupation with a perceived physical defect for at least 1 hour per
day; (b) the defect must cause the individual substantial distress, interfering with
daily life, including social and occupational areas of function; and (c) repetitive
behaviors or mental acts like rumination must be present (Pavan et al., 2008;
Phillips, 2014).
The diagnosis of BDD may not be easy to make, as many individuals with
this disorder may not discuss the degree of their concerns unless they are asked
directly. Veale and Bewley (2015) note that diagnosis requires a careful review
of an individual’s history, particularly the degree of preoccupation with the body,
how much it interferes with daily life, level of distress associated with the
preoccupation, and whether the individual avoids certain activities because of
body preoccupation. It is also suggested that the clinician observe carefully for
attempts to camouflage body parts with clothing or makeup (Pavan et al., 2008;
Phillips, 2014).
There are two specifiers that need to be considered in the diagnosis of BDD.
These include muscle dysmorphia and insight regarding BDD beliefs. The
muscle dysmorphia specifier identifies individuals who believe their body builds
are too small or lack adequate muscle development, despite a normal or very
muscular appearance. This belief could persist in the presence of other non–
muscle-focused preoccupations as well. The insight specifier addresses the level
of insight an individual has related to BDD beliefs. This specifier has three
levels: (a) with good or fair insight, (b) with poor insight, and (c) with absent
insight/delusional beliefs and contributes to distinguishing degrees of severity
(Phillips, 2014; Schieber et al., 2015).
Hoarding Disorder
Key diagnostic criteria for HD include (a) persistent difficulty discarding
possessions, (b) marked distress associated with discarding possessions, (c)
congestive clutter that impedes normal use of living spaces and compromises
their intended use, (d) significant distress or impairment at the suggestion of
discarding, (e) symptoms that are not attributable to other conditions, and (f)
symptoms not attributable to other relevant psychiatric conditions (DSM-5,
2013). The attachment that these individuals have to their possessions is
attributed to potential value or usefulness, sentiment, and a desire not to create
waste (Gilliam & Tolin, 2010; Mataix-Cols, 2014).
Additional information that can be useful in making the diagnosis of HD
includes observations made by the clinician or by reliable family members about
the condition of the home of individuals suspected of having HD. These
observations can demonstrate floor to ceiling stacks of newspapers, books, mail,
etc., that allow only a small path to be travelled within the living space;
possessions piled on beds, seating areas, tables, and even kitchen appliances; and
no apparent organization to the collections. Reports from family members may
indicate that they themselves, or others, have intervened to help individuals
discard possessions. These reports, along with photographs, may be helpful, as
well (Nordsletten & Mataix-Cols, 2014). Additionally, it is important for the
clinician to differentiate HD from normal collecting. One particularly important
differentiation is the characteristic disorganization and clutter associated with
HD, along with very little relationship seen among the myriad possessions
(Mataix-Cols, 2014).
There are two specifiers that exist for HD, excessive acquisition and insight.
Neither of these is required for the diagnosis, but they help to identify specific
areas of concern for the clinician. Excessive acquisition refers to the continued
accumulation of possessions that are unnecessary or for which there is no space.
The insight specifier is the same as noted in other of the OCRDs: (a) good or fair
insight, (b) poor insight, or (c) no insight/delusional (Mataix-Cols, 2014).
Key diagnostic criteria for HPD in the DSM-5 include (a) recurrent pulling out
of one’s own hair leading to noticeable hair loss, (b) repeated attempts to
decrease or stop pulling, (c) the disturbance is not better accounted for by
another mental disorder and is not due to a general medical (e.g.,
dermatological) condition, and (d) the disturbance causes clinically significant
distress or impairment in social, occupational, or other important areas of
functioning (APA, 2013). A diagnosis of hair-pulling disorder requires
individuals meet all four criteria.
While the DSM-5 has attempted to recategorize hair-pulling disorder, the
requirement for meeting all four criteria has come under review, based on the
idea of a dimensional framework, which would put diagnostic criteria on a
spectrum or continuum. Using a dimension framework, researchers have argued
that it would be easier to predict relapses by showing systematic variances along
a continuum (Houghton et al., 2015).
There are several assessments that can be used for diagnosis including the
National Institute on Mental Health Trichotillomania Severity Scale, which has
adequate psychometrics, and the Clinical Global Impression Severity Scale,
which has good psychometrics. The latter assessment is often used to measure
treatment outcomes (Houghton et al., 2015).
Key criteria for SPD include (a) the presence of skin lesions, (b) clinically
significant distress or functional impairment, and (c) repeated attempts to stop or
decrease picking (Snorrason et al., 2013). It is important when considering SPD
that the clinician looks closely to distinguish SPD from other disorders such as
primary pruritic skin disorders systematic conditions related to chronic,
generalized pruritus, and other psychocutaneous syndromes, such as depression,
anxiety, and/or delusional infestation (Craig-Muller & Reichenberg, 2015). It
can also be misdiagnosed as OCD or BDD (Grant et al., 2012). Skin picking is
also found to coexist many times with other grooming disorders, and lesions
may vary from several to several hundred, depending on severity. Fingernails are
used to create most lesions. Some individuals, however, report using tools to
pick, such as knives, tweezers, pins, and needles (Grant et al., 2012).
It is important to note that skin damage and infections are quite common.
Some individuals could experience disfigurement caused by picking, making
SPD a significant mental health problem (Snorrason et al., 2013). Therefore, it is
imperative that clinicians ask “problem-specific questions,” including personal
and family history, general acuity of the problem, frequency of picking, time
spent picking, awareness of picking, body parts involved, when picking occurs,
attempts to resist picking, and degree of distress evoked by picking (Craig-
Muller & Reichenberg, 2015).
The Skin-Picking Impact Scale (SPIS) developed by Keuthen et al. (2001)
has shown good psychometrics and has been used effectively in the diagnosis of
SPD (Craig-Muller & Reichenberg, 2015). Recently, a short version of the SPIS,
called the SPIS-S, has been developed and appears to be more effective with
individuals whose picking extends beyond the face (Snorrason et al., Olafsson
2013).
Research has shown that the neurobiology of OCD is complex, making medical
management complex, as well. The orbitofrontal cortex, anterior cingulate gyrus,
and caudate nucleus of the brain are all involved in OCD. Furthermore,
abnormalities in neurotransmitters like serotonin, dopamine, and glutamate are
also apparent contributors to OCD. The most effective treatment for OCD is a
combination of cognitive-behavioral therapy (CBT), exposure response
prevention (ERP) therapy, and pharmacotherapy (Bokor & Anderson, 2014;
NAMI, 2013).
CBT is a type of psychotherapy that addresses dysfunctional emotions,
maladaptive behaviors, cognitive processes, and differentiation of rational and
irrational thinking. ERP encourages the individual to confront images that
provoke anxiety and then conscious opt to NOT implement a compulsive
behavior. This combination of CBT and ERP has had the most long-term success
in the treatment of OCD (Seibell & Hollander, 2014). Individuals in treatment
must adhere closely to instructions to ensure that learning occurs. Any kind of
avoidance or subtle distraction may weaken the outcome (Grant, 2014).
When the CBT/ERP combination is not enough, selective serotonin reuptake
inhibitors (SSRIs) are also used (Bokor & Anderson, 2014). The compounds
with FDA approval include paroxetine, sertraline, fluoxetine, fluvoxamine, and
clomipramine (Bokor & Anderson, 2014; Seibell & Hollander, 2014). There are
additional combination strategies that may be used for adults with comorbid
depression or other mental illness. Adaptations of all of these treatment methods
must occur if they are to be used in children and adolescents (Franklin & Foa,
2011). Psychoeducation, reduction of psychosocial stress, and guided self-help
are strategies that can be used in the treatment of children with OCD, along with
CBT, behavioral therapy, and SSRIs (Ferren, Palmes, & Kaplan, 2013; Thomsen,
2013).
Neuromodulation is being researched for OCD based on a deeper
understanding of brain circuitry in some psychiatric conditions. The need for
neuromodulation is due to individual OCD resistance to the usual treatment at a
level of approximately 40% to 60%. Neuromodulation is preferable to more
invasive methods, such as surgery, and includes transcranial magnetic
stimulation, which modulates cortical and subcortical function to increase or
decrease cortical excitability. These treatments are considered experimental
because the pathophysiology of OCD remains unclear (Pallanti, Marras, &
Grassi, 2015; Pittenger, 2015).
While BDD is related to OCD, it is less responsive to ERP, and this approach is
unlikely to be successful. What is suggested includes some basic strategies such
as motivational interviewing, focus on patients’ functional impairment, and
discouraging patients from seeking cosmetic dermatological, dental, or surgical
treatment (Phillips, 2014). Cognitive-behavioral therapy (CBT) that is specific to
BDD, following a protocol that lasts over 6 to 24 sessions, is the recommended
treatment (National Institute for Health and Clinical Excellence, 2005).
According to Veale and Bewley (2015), this plan is based on several randomized
controlled trials, demonstrating that CBT is “…more effective in improving
severity of body dysmorphic disorder, based on blinded assessment by
clinicians” (p. 2280). The aim of treatment is to decrease preoccupation with the
body to reduce self-focused attention and to address faulty beliefs and
assumptions. This process includes self-monitoring of thoughts and behaviors.
Some individuals may be helped by SSRIs over a 3-month period used in
addition to the focused CBT (Pavan et al., 2008).
Hoarding Disorder
Management of this condition is best done through a multifaceted psychological
approach based on CBT, blending motivational enhancement techniques, and
goal setting with behavioral modification and skills training (NAMI, 2013;
Nordsletten & Mataix-Cols, 2014). CBT for HD uses a variety of strategies to
address motivational issues; information processing deficits; beliefs about, and
attachment to, possessions; and behavioral avoidance (Gilliam & Tolin, 2010).
According to Gilliam and Tolin (2010), when using the CBT for HD
strategies, it is imperative that goal setting is addressed at the outset of treatment
to ensure that the individuals can identify personally relevant reasons to make
changes in their hoarding behaviors. Simultaneous with CBT, the individual
must begin the sorting and discarding process, which requires extensive therapist
involvement asking challenging questions and employing behavioral
experiments to help address barriers to treatment. More research is needed on
both psychological treatment and pharmacological treatment, particularly due to
the high cost of medications (NAMI, 2013). A lot of uncertainties remain
relative to hoarding in children and adolescents, family-centered intervention,
and the value of pharmacologic treatments (Mataix-Cols, 2014).
Hair-Pulling and Skin-Picking Disorders
HPD and SPD are combined here because recent neuroimaging research has
shown substantial similarities between the two disorders, particularly in the
frontostriatal circuitry of the brain, and the medical management, therefore, is
similar. The areas of the brain involved appear to be related to stimulus-response
habit formation. There are, however, some distinct differences in brain structures
between HPD and SPD, including differences in brain volume and cortical
thickness (Roos, Grant, Fouche, Stein, & Lochner, 2015).
Habit reversal training (HRT) and cognitive-behavioral therapy (CBT) used
singly and in combination have been successful in treating both HPD and SPD
(Rogers et al., 2014). However, there is a gap between implementation of
treatment and documentation of efficacy. One of the problems is that there are
not many competent providers of HPD/SPD treatment, as evidenced by the “…
lack of providers in 12 states and only 1 provider for another 14 states”
(Keuthen, Tung, Reese, Raikes, Lee, & Mansueto, 2015, p. 11). Another
significant issue is that various racial and ethnic groups respond differently to
treatment, especially to web-based, self-help programs (Falkenstein, Rogers,
Malloy, & Haaga, 2015).
The TLC Foundation for Body-Focused Repetitive Disorders, formerly
known as the Trichotillomania Learning Center, endorses a behavioral treatment
approach that includes HRT along with CBT, and medications (TLC Foundation,
2016). This combination of behavioral therapy and medication has been shown
to be more effective than either HRT or CBT alone. Habit reversal training
(HRT) is a behavioral treatment approach developed in the 1970s and has been
found to be useful in treating HPD and SPD over the short term, but is less
effective long term (Golomb et al., 2011). HRT includes awareness training,
competing response training, contingency management, relaxation training, and
generalization training. HRT used in combination with CBT and medications
that affect serotonin and/or glutamate production in the brain has been shown to
be effective for HPD and SPD as well. Other treatment strategies are being
explored (Jacob, 2013; Rogers et al., 2014).
Because the OCRDs relate directly to functional behaviors, each of them has a
significant impact on client factors and occupational performance. Client factors
as defined by the Occupational Therapy Practice Framework, 3rd Edition
(AOTA, 2014) include “…capacities, characteristics, or beliefs that reside with
the person…” (p. 57). Client factors include (a) values, beliefs, and spirituality,
(b) body functions, and (c) body structures. These factors can affect and be
affected by performance skills, through influence on motivation and individual
perceptions of value as well as through physical and physiological perceptions of
the world. This section of the chapter will address client factors and discuss how
the OCRDs impact each factor and ultimately impact occupational performance.
Values, Beliefs, and Spirituality
This category of client factors relates to cultural beliefs and values, what is held
as true by an individual, and the way that individuals express meaning and
purpose and connectedness in their lives. As can be seen from the descriptions of
the OCRDs, this category of client factors is affected heavily. In OCD, for
example, beliefs about what is safe (contamination), what is orderly
(sequencing), what is repugnant (unacceptable behaviors like harming a friend),
and what is moral (sexual and religious beliefs) affect social encounters, how
one maintains a home, how one carries out ADLs, and how one relates to others
(Bokor & Anderson, 2014; Saxena et al., 2011). In BDD, beliefs about one’s
attractiveness or its importance can interfere with both social and work activities.
These beliefs can also have an impact on style and type of clothing or attire
identified as appropriate In HD, beliefs about what is valuable and what one can
or cannot do without become important, along with an individual’s sense of
power over one’s own behaviors (Gilliam & Tolin, 2010). In HPD and SPD,
issues related to honesty with self, as well as issues of power, are present
(Snorrason et al., 2013). In all of the OCRDs, issues related to the expression of
meaning and purpose and connected to the world and to others are a strong
presence, which the public does not understand or accept. Furthermore, the
media tends to create and reinforce faulty conceptualizations of mental illness
(Fennel & Boyd, 2014).
Body Functions
All of the OCRDs affect body functions, including mental functions, both
specific and global, volunteer activities, play, leisure, and social participation.
Mental Functions
Each of the OCRDs has a unique neurobiology that affects a different area of the
brain and therefore impacts mental functions slightly differently. So, for
example, all of the OCRDs affect higher level cognition, such as judgment,
concept formation, metacognition, cognitive flexibility, insight, attention, and
awareness (Abramowitz & Jacoby, 2014; Mataix-Cols, 2014; Phillips, 2014). So,
for example, research in HD has shown cognition deficits in judgment, cognitive
flexibility, and insight (Grant, 2014; Phillips, 2014; Tung et al., 2015).
Concentration and attention are very involved in OCD, along with perceptions,
thoughts, global, and other mental functions. Depending on the severity of the
OCRD, one’s state of awareness and alertness may be affected, along with
temperament and personality and energy level.
Sensory Functions
Sensory functions have also been researched in OCD, to examine how
individuals process sensory input. According to Rieke and Anderson (2009),
adults with OCD have greater sensory sensitivity and sensation avoiding than do
other adults. This includes difficulty ignoring stimuli and responding readily.
This difficulty suggests that adults with OCD have less efficient sensory
processing patterns, which may be linked to neurocognitive processes. Also,
because clinicians do not know what the individual with BDD actually sees, “…
abnormalities in visual processing” are suggested, according to Phillips (2014, p.
326).
Skin and Related Functions

Individuals with HPD and SPD may have limited understanding of the protective
nature of skin and hair, if they are unaware of their behavior (Rogers et al.,
2014). Even with awareness, because these two disorders may be comorbid with
BDD or another mental illness, the ability to interpret and act on that knowledge
may be impaired, as well (Phillips, 2014; Snorrason et al., 2013).
Body Structures
In this category, all of the OCRDs have a relationship to the structure of the
nervous system, as discussed earlier in the chapter. The research demonstrating
brain abnormalities and neurotransmitter differences is testament to this
connection and to the importance of understanding the stigma, the shame, the
suffering, and the disability that creates the devastation of mental illness
(Pallanti et al., 2015; Pittenger, 2015, Seibell, 2015).
The impact of OCRDs on occupational performance can be profound,
depending on the severity of symptoms, the client factors involved, and the
duration of the illness. In terms of performance skills, such things as positioning
and transporting objects may be problematic for someone with a hoarding
disorder. Process skills are much more involved, related to ability to attend,
heed, make choices, initiate, sequence, and organize activities, any and all of
which may be impaired, as in someone who is preoccupied with skin picking or
some other body concerns. Social interaction skills are seriously eroded in all of
the OCRDs, as indicated earlier in the chapter, including the ability to engage in
social interactions.
Performance patterns in terms of habits, routines, rituals, and roles may all
be seriously impaired, as the OCRDs related directly to these categories. For
example, rituals may be overstressed, while routines may be erratic due to
interference from obsessive thoughts or compulsive actions. The ability to
perform in a particular role may also be impaired, as in attempting to play in the
mud with a child when a contamination obsession is present, or attempting to
prepare a meal for family when the kitchen is cluttered with things that don’t
belong there, but for which there is no other space.
It is evident that client factors, performance skills, and performance patterns
can all be affected by the presence of OCRDs and can significantly impact one’s
ability to live comfortably in one’s own home or even in one’s own skin. Being
cognizant of the many complexities involved in living a functional life is
important in dealing with individuals having OCRD.
CASE-STUDY 1
Eileen is 28-year-old interior designer. She has worked in her field for about
6 years, but she has had seven jobs in that length of time. Eileen has always
been very conscious of her appearance, although she has never been happy
with it. From the time she was little, Eileen has been aware of what she
believes is a serious flaw in her appearance. When she turned 13, this flaw
became more of an issue for her because she suddenly became interested in
boys and she was sure no one would be interested in her because of this
flaw. Eileen has tried numerous ways to conceal the flaw, but is never
satisfied with the result.
The flaw is located on her left ear. At the top of the ear where it would
normally curl, for about one-half inch along the curled edge, Eileen’s ear is
flat. When she was little, it was easy to cover with her hair, but she would
never let her mother put her hair in pigtails or braids or a ponytail that
others girls wore. If her mother was insistent, Eileen would wear earmuffs
all day until she could take out the braids or pigtails. If anyone noticed it or
asked about it, she said she had “cold ears.”
As Eileen has gotten older and wanted to try different hairstyles, she
has become acutely aware of this defect, to the point that she spends hours
every day focused on it. Getting through high school was very difficult for
Eileen, because she was so self-conscious about her appearance. She
refused to present in class and used to sit with her face downcast, her head
resting in her hand, and her hand covering her ear. She did not date in high
school because she was so self-conscious that she was unable to make eye
contact with anyone who spoke to her, which earned her a reputation as a
snob. Eileen developed one good friend in high school, Kate. Kate was
willing to listen to Eileen’s concerns and would sympathize to a degree.
However, once Kate went to college, she changed, and told Eileen that she
had a “problem” and needed help to deal with it. This event prompted
devastation in Eileen, and she cried for weeks, expressing feelings of
worthlessness. Eileen and Kate have not spoken since that time. While
Eileen was studying for her interior design career, she arranged all her
classes for later in the day, so she would have plenty of time to work on
covering up her flawed ear.
Since getting her design credential, Eileen has had numerous jobs. The
reason is that she usually quits or loses the job because she is unable to
keep her appointments in a timely fashion and often has difficulty focusing
on client conversations and desires due to her preoccupation with her ear
flaw. It typically takes Eileen 3 hours to prepare for work. Every morning
when she is getting ready for work, she spends most of her time fussing
with her hair to cover the flawed part of her ear and then moving around the
room, as she might do if she was working at a client’s home, to see if the
flaw is revealed. If she does find that certain movements display the ear,
then Eileen attempts to control her movements more closely, resulting in
stilted and awkward movements that actually draw more attention to her
than usual. During the workday, Eileen is so obsessed with her hair and her
ear, that she frequently checks herself in store windows, the car mirror, and
in the bathroom. Many days, she wears a hat to cover her flaw and then has
to spend time rearranging or adjusting it. These behaviors have resulted in
client requests for another designer and have cost her the job in several
cases. The loss of her last job resulted in another significant episode of
depression.
As a result of her obsession with her ear flaw and her inability to work
or concentrate on anything else, Eileen has no social life. She does not have
the money to live independently, so she lives at home with her widowed
mother, a teacher. Eileen’s mother has tried numerous strategies to help
Eileen address her concerns, even agreeing to take her to a plastic surgeon,
particularly following Eileen’s most recent depressive episode. The surgery
proposed by the physician, however, was so expensive that Eileen could not
afford it, and since she is over age, she is no longer covered by her mother’s
insurance. The physician did recommend a psychiatric consult, based on
Eileen’s depression, which her mother thought would be a good idea. This
encounter has convinced Eileen that her preoccupation with her ear flaw
may actually be abnormal and that perhaps she should think about seeing a
psychiatrist, especially since she has already experienced two depressive
episodes. Eileen has agreed to think about it, primarily because she is so
unhappy and has found herself recently thinking about suicide.
CASE-STUDY 2
Jerry is a disabled veteran of the Gulf War. He is 52 years old and comes
from a large family with seven siblings, all of whom live in the same area
where Jerry lives. Jerry has never married, and when he returned from the
Gulf War with a disability, he was invited to live with his sister and her
family. Recently, Jerry was asked to leave his sister’s home because he had
accumulated so much stuff that he had filled up his own space and had
begun to encroach on the family space. It was decided that Jerry needed his
own place. Because Jerry is disabled, he is unable to work, but he does
receive disability and a small military pension, enabling him to live
independently in a subsidized housing environment. His apartment is one
bedroom, with a living/dining area, bathroom, and kitchenette. It is about
550 square feet.
According to Jerry’s sister, he has always been a “collector” of sorts,
not of anything particular, but rather of odds and ends that appealed to him,
or that had some sentimental value. He has saved a lot of his papers and
grade school report cards, along with a great deal of his high school
memorabilia. He has saved similar memorabilia from the activities of his
many nieces and nephews, special events that he has attended, posters,
correspondence, newspaper and magazine articles he has found interesting,
and so on. When he was younger, his collectibles could be contained in
several boxes in his room, or in his closet, and his mother would
periodically suggest that he get rid of some stuff. He never did, but he
would just compress his stuff so it fit into fewer boxes.
Since Jerry has been back home after his military experience and
several months in the hospital because of his injury, his collecting has
become excessive, notes his sister. She states that she had to ask him to
move because his room was filled with boxes and also loose papers he had
saved—cards, articles, more correspondence, and souvenirs from his recent
volunteer work with the Boys and Girls Club. She objected when she could
not get into the room to run the vacuum, and he agreed to move some things
to the family garage. But that became impossible as well, said his sister, so
she asked him to move.
Jerry’s other siblings refuse to go to his house because there is no place
to sit in the living/dining room area or the kitchenette. There is a small
stove, but that is covered with magazines and unopened mail and is
currently not usable. Jerry generally does not cook anyway, preferring to eat
directly from the refrigerator, in which he keeps ready-to-eat foods such as
yogurt, cheese and crackers, some fruit, and other things he can eat from the
carton. He likes to order Chinese food once every couple of weeks if he
can. The tabletop is covered with mail and magazine articles, while
newspapers are stacked 6 to 7 ft high all through the house. Even in the
bedroom, part of the bed, along with the floor, the dresser tops, and one
chair, is covered with both boxes and loose papers. It is difficult to get into
the bathroom, but there is a slender path to the toilet and sink. The
bathtub/shower is full of boxes of memorabilia.
Outside of his volunteer work, Jerry has few interests and he does not
socialize much. He has never been comfortable in social situations,
preferring his own company or the company of family. Jerry is good with
children, which allows him to continue his work with the Boys and Girls
Club. He does visit his siblings for family gatherings, but it is difficult for
him to get around because of his disability. So his interests are focused
inside the house including reading, watching television, maintaining
correspondence with old military buddies, and looking at pictures and other
stuff from the children of his family members.
The family has suggested both cleaning up Jerry’s apartment and even
helping him to do it, but he has refused any assistance, saying that he would
be afraid they would discard something that was really important to him or
that he might need later. The family has also suggested that his hoarding
may be a mental health problem, but Jerry doesn’t see it that way. He has,
however, been seeing a counselor for a recent episode of depression, and his
collecting/hoarding behavior was broached in that meeting. Jerry agreed to
let the counselor visit him in his apartment, but he is uncertain if he will be
able to do anything to change the living environment or lifestyle with which
he is comfortable.
LEARNING RESOURCES
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C H A P T E R
14 Somatic Symptoms and Related
Disorders
Michael J. Urban
KEY TERMS
Chronic pain Cognitive-based therapy (CBT) Conversion disorder (functional
neurological symptom disorder) Depression Dystonic movements Factitious
disorders Hypochondriasis Illness anxiety disorder Malingering Munchausen’s
syndrome Munchausen’s syndrome by proxy Occupational performance Pain
Psychological factors Somatic symptom disorder
Mr. Smith is a 30-year-old man who works at a local construction company.
While at work one day, he was helping to move some 8 foot 2 × 4 pieces of
lumber, which was a normal job function. After moving 50 boards, he noticed
his back was sore and he started to have difficulty with getting in and out of his
truck or going to stand from sitting in a chair. Mr. Smith the next day called out
of work to rest his back. After 2 days of no resolve, he went to see his doctor as
instructed by his supervisor and was diagnosed initially with a lumbar strain
needing therapy.
After completing 4 weeks of the physical therapy for his back and being out
of work, Mr. Smith was referred to an occupational therapist for work hardening
as his body had become deconditioned. His pain was still reported to be at an
8/10 constantly with difficulty with any kind of bending or performing
transitions from a seated or supine surface. The report for transition from
physical therapy showed all the therapeutic exercises to stretch his back and
modalities that were used. The occupational therapist started to conduct the
evaluation that comprised a full check of Mr. Smith’s full body active range of
motion (AROM), passive range of motion (PROM), strength, sensation, hobbies,
leisure pursuits, interests, work classification, job description, ability to complete
his activities of daily living (ADLs) and instrumental activities of daily living
(IADLs), and current cognitive status, which included memory and risk of
depression. The evaluation also reviewed his current physical capacity and
mechanics to complete basic lifting, pushing, pulling, carrying, and functional
positional and mobility ability. Each client factor, performance pattern, and
performance skill noted to be a weakness was identified in relation to the impact
on his ability to complete his basic daily needs and work functions.
Testing noted that Mr. Smith clinically and objectively demonstrated signs
consistent with depression and several inconsistencies and self-limiting
behaviors. He was noted to have a 10% loss of full forward trunk flexion and
poor body mechanics and posturing for daily habitual routines. With functional
testing, Mr. Smith often reported having 10/10 pain and reported he could not lift
the 10-lb box from the table to carry to another table. The occupational profile
noted that Mr. Smith enjoyed spending time with his 4-month-old son since his
injury and his injury has allowed his wife to return to work earlier to a higher
paying job. When showing pictures of him carrying his son to the occupational
therapist, he disclosed that his son weighed 21 lb.
Somatosensory sensation can be viewed as subjective and objective in its own
right as we all perceive pain differently. Pain is a physical suffering or
discomfort caused by illness or injury (American Psychiatric Association, 2013).
It is a reaction to some kind of physical, biological, or emotional trauma that
disrupts the harmony of our everyday life.
Somatic symptom disorder is a disorder where the physical symptoms
cannot be fully explained by a general medical condition, another mental
disorder, or the effects of a substance. They could be the result of a factitious or
a malingering disorder. Malingering is the intentional production of false or
grossly exaggerated physical or psychological problems (American Psychiatric
Association, 2013). Despite the unknown origins, they are noted to cause
significant impairments to everyday engagement in social occupational
performance and all areas of daily living. Occupational performance is an act
accomplishing a selected action (The American Occupational Therapy
Association, 2014). The Diagnostic and Statistical Manual of Mental Disorders
5th Edition (DSM-5) uses the term somatic symptom and related disorders as a
broad classification grouping a variety of disorders. Changes were made to the
classifications from DSM-IV to DSM-5 to consolidate similar conditions into
one classification cluster (see Table 14.1).
TABLE 14.1 Somatic Symptom Disorder and Related Disorders
When medical providers think about a somatic disorder, they view it as chronic
pain without a known cause (American Psychiatric Association, 2013). Chronic
pain is pain that causes clinically significant distress or impairment in social,
occupational, or other important areas of functioning for a duration >6 months
(American Psychiatric Association, 2013). As occupational therapy practitioners,
we must think about the areas of daily functioning that have been impaired.
Individuals in this classification often display an increased attention to their
bodily sensations with the attributions of those sensations expressed negatively.
These two cognitive components foster the individual learning the help-seeking
behaviors associated with the sick role that are only reinforced through attention
and/or sympathy. The level of distress caused by the fixation developing or even
acquiring a serious illness or debilitating impairment preoccupies the individual
into severe forms of self-limiting and habit-forming behaviors. Table 14.2
compiles the most common symptoms reported by an individual with no medical
root cause noted.
TABLE 14.2 Most Common Symptoms Reported with Somatic Disorders

Etiology
There is no known cause for somatic sensory and related disorders, as the
physical complaints cannot be fully explained by a medical condition or
substance abuse. These disorders are neither testable nor physically or medically
identifiable and thus are difficult to treat by medical professionals (Crane et al.,
2012). Due to the unknown origin, physicians report this classification of
disorders as difficult to identify and treat properly (Crane et al., 2012; de Waal,
Arnold, Eekhof, & van Hemert, 2004; Voigt et al., 2012). The impact on a client
can result in the loss of physical, social, and occupational performance. Most
clients diagnosed with a form of somatic-related disorder typically have a history
or an associated diagnosis of depression and/or anxiety, which could exacerbate
the condition (Crane et al., 2012; de Waal et al., 2004). Depression is a lack of
interest or pleasure in daily activities for more than a 2-week period and
represents a change from previous functioning (American Psychiatric
Association, 2013).
There is no difference in prevalence by gender, age, or ethnicity (Crane et al.,
2012). For people with somatoform disorders, costs are two times higher than
those with other medical conditions in the United States (Crane et al., 2012).
Some literature does suggest that somatoform symptoms and related disorders
are the third most common psychiatric condition behind depression and anxiety
(Crane et al., 2012; de Waal et al., 2004; Douzenis & Seretis, 2013; Neng &
Weck, 2013). Current literature cannot accurately document the incidence and
prevalence rates due to the difficulty with properly diagnosing the disorders due
to their unknown origin and the recent changes from DSM-IV to DSM-5, which
increased the identification of individuals who are psychologically impaired
(Voigt et al., 2013).
Signs and Symptoms
Complaints of pain that cause significant distress and disability with daily
occupations are abundant in all areas of health care practice. The correlation
between chronic pain and psychiatric disorders is well known but hard to treat
due to the subjectivity in nature of the complaints (American Psychiatric
Association, 2013; Crane et al., 2012; de Waal et al., 2004; Dimsdale et al.,
2013; Douzenis & Seretis, 2013). A key characteristic stems around the
excessive fixation or concern the client has about physical symptoms or health
(Crane et al., 2012; de Waal et al., 2004; McCabe et al., 2009; Neng & Weck,
2013). Somatic symptom disorder, illness anxiety disorder, conversion disorder,
and psychological factors affecting other medical conditions all derive from this
overall notion but with slight variations (Arnold, de Waal, Eekhof, & van
Hemert, 2006; Bener, Al-Kazaz, Ftouni, Al-Harthy, & Dafeeah, 2013; de Waal et
al., 2004; Dimsdale et al., 2013; Katz, Rosenbloom, & Fashler, 2015). These
characteristics prominently appear in a medical setting rather than a mental
health care setting.
Somatic Symptom Disorder
Clients with somatic symptom disorder may present with one or multiple
complaints of muscle and joint pains, low back pain, tension headaches, chronic
fatigue, non–cardiac-related chest pain, palpitations, nonulcer dyspepsia,
irritable bowel, dizziness, and insomnia (American Psychiatric Association,
2013; Bener et al., 2013; Crane et al., 2012; de Waal et al., 2004; Kroenke,
Spitzer, deGruy, & Swindle, 1998). Clients also are noted to have a higher
medical history that includes depression, irritable bowel syndrome,
fibromyalgia, chronic pain, posttraumatic stress disorder, antisocial personality
disorder, and a history of sexual or physical abuse. Due to the physical fixation,
this classification can be difficult to identify and can greatly impact a client’s
overall occupational performance with daily tasks.
Illness Anxiety Disorder
Clients diagnosed with hypochondriasis have been reclassified as illness
anxiety disorder under the DSM-5 (American Psychiatric Association, 2013).
Hypochondriasis is a preoccupation with fears of having or the idea that one has
a serious disease based upon the person’s misinterpretation of bodily symptoms
(American Psychiatric Association, 2013). A key characteristic factor is the
preoccupation of having or acquiring a serious illness. Clients in this cluster
have a high anxiety about health leading to excessive or maladaptive health
behaviors, such as repeatedly checking their bodies for signs of illness or
avoiding the doctor altogether. Clients in this classification tend not to be
relieved by negative test results, and the reassurance of negative findings from
doctors does not decrease the client’s anxiety.
Conversion Disorder
Conversion disorder is often referred to as functional neurological symptom
disorder or psychogenic (American Psychiatric Association, 2013; Arnold et al.,
2006; Crane et al., 2012; Dimsdale et al., 2013; Katz et al., 2015) . A client with
conversion disorder is characterized by one or more symptoms related to a motor
or sensory impairment when all medical testing is considered normal or the
symptoms reported are inconsistent in nature. Typical motor symptoms are
tremors, dystonic movements, gait abnormalities, abnormal limb posturing, and
weakness or paralysis. Dystonic movement is a neurological movement disorder
in which sustained muscle contractions cause twisting and repetitive movements
or abnormal postures (American Psychiatric Association, 2013). Sensory
symptoms include altered, reduced, or an absence of hearing, visual, or skin
sensation. Other symptoms can include reduced or absence of speech volume,
sensation of a lump in the throat, and altered articulation. Examples of loss of
motor function could present with a client who is unable to abduct the shoulder
greater than 40 degrees when tested but then can use the same arm to scratch the
back of the head, which uses approximately 85 to 95 degrees of shoulder
abduction.
Psychological Factors Affecting Other Medical
Conditions
Psychological factors affecting other medical conditions are noted to be
behavioral or psychological factors that can exacerbate or have an influence on
the pathophysiology of the medical condition (American Psychiatric
Association, 2013). Characteristics associated in this classification include poor
adherence to medical recommendations, coping styles, patterns of interpersonal
interactions, psychological distress, and other maladaptive behaviors that impact
denial of a medical condition. Examples of these factors can include chronic
occupational stress leading to increased risk for hypotension to conditions with a
clear medical etiology impacted by the psychological factors such as knowingly
ignoring the signs of a heart attack. This classification helps to clarify the impact
that lifestyle, personality, or occupational stressors have on a medical condition.
In this classification, individuals can choose to ignore or continue to engage in
the factors that have a negative impact on their overall general well-being and
health. If the behaviors or psychological components are deemed to stem from
the medical condition, a more appropriate diagnosis and classification will be
provided. Simple examples of this disorder classification would be the impact of
a chronic stressful lifestyle on irritable bowel syndrome.
Factitious Disorders
The last defined classification for somatic symptoms and related disorders is
factitious disorders (American Psychiatric Association, 2013). Factitious
disorders are falsifications of an illness for oneself or others. In this
classification, the individual reports various symptoms to obtain a diagnosis
from a doctor, often to gain attention. In some cases, the individual is able to
convince the doctor to perform surgery to the extent of loss of limb as an act of
mutilation. Factitious on others differs from factitious on self in that the
caregiver will project false reports of symptoms on behalf of someone in his or
her care such as a child or elder. The end result can be unnecessary medical
procedures including surgery. Individuals with a type of factitious disorders use
to be diagnosed with Munchausen’s syndrome (factitious on self) or
Munchausen’s syndrome by proxy (factitious on others) (Ferrara et al., 2014).
Munchausen’s syndrome was named after Karl Friedrich Hieronymus Von
Munchausen, a German cavalry officer who was known for exaggerating his
adventures to attract attention. People who exaggerate their symptoms do so not
for financial gain but for increased sympathy (American Psychiatric Association,
2013; Ferrara et al., 2014). Munchausen’s syndrome by proxy is the
exaggerating of an illness, typically upon a child, to gain sympathy for the
caregiver (American Psychiatric Association, 2013; Ferrara et al., 2014).
Individuals with factitious disorder could go to extremes to alter medical test
results to help support their subjective complaints. Examples of this could
include placing blood in their urine sample and physical self-harm. The
differential diagnosis for factitious and all related disorders is challenging for
health care professionals due to there being little to no evidence of the individual
deceiving the doctor into believing that the symptoms are real despite the lack of
medical documentation to support claims (Ferrara et al., 2014). Occupational
therapy practitioners as part of an interdisciplinary medical team need to be
cognizant of any inconsistencies noted during functional tasks. Early return to
functioning and appropriate diagnosis and medical treatment is essential to help
minimize the development of such disorders mentioned related to somatic
symptoms (Clark, Bair, Buckenmaier, Gironda, & Walker, 2007; Ferrara et al.,
2014).
Somatic symptoms and related disorders can occur across the life span in
different variations and frequencies. The nature of and pattern of somatic
complaints can be part of the normal aging process expected with the course of
maturation. Examples of this would include older adults with some health issues
having an increased worry over various symptoms than a younger individual or
healthier peer. In children, examples of normal progression for somatic
complaints are noted with common recurrent abdominal pain, fatigue, or
headaches but without any worry of fixation of the somatic complaints
(American Psychiatric Association, 2013).
Illness anxiety disorders are noted to have an unclear development and
course but are noted to be rarer in children than adults (American Psychiatric
Association, 2013; Bener et al., 2013). One explanation for this difference in
children versus an adult is the increase in anxiety symptoms as people age, with
the most common anxiety fixating on memory loss (American Psychiatric
Association, 2013; Bener et al., 2013). Conversion disorders tend to have
increased onset of nonepileptic attacks in the 30s, while motor symptom onset
peaks in the 40s (American Psychiatric Association, 2013). Factitious disorders
are noted to have an onset in early adulthood after a hospitalization, which are
often lifelong in nature (American Psychiatric Association, 2013; Ferrara et al.,
2014).
Because of the psychological complexity involved, somatic symptoms and
related disorders often result in a poor prognosis (Simms, Prisciandaro, Krueger,
& Goldberg, 2012). Due to the complexity and severity noted with each disorder,
the ability to provide proper medical treatment often is limited. Medical
treatment is further complicated by the individual’s tendency to not remain
constant with one health care provider long enough to receive a proper diagnosis
(Crane et al., 2012; de Waal et al., 2004; Simms et al., 2012). To be successful in
treating an individual, the health care provider needs to be knowledgeable and
tactful in the approach to diagnosing and treating the disorders (Arnold et al.,
2006; de Waal et al., 2004; Simms et al., 2012). If the individual feels the
provider does not believe his or her reported symptoms or if the provider accuses
the individual of making up the symptoms, the patient-provider rapport will be
destroyed, thus yielding a poor prognosis over the long term (Crane et al., 2012;
de Waal et al., 2004; McCabe et al., 2009; Neng & Weck, 2013).
Since the prognosis and duration is relatively unknown, the occupational
therapy practitioner needs to be able to build a strong rapport with all individuals
to allow for proper assessments of impairments of occupational performance.
The ability to train an individual in how to adapt to the changes in his or her
medical conditions can help redesign daily occupational performance patterns to
compensate for any perceived impaired client factors. Through the use of
compensatory techniques and a complete occupational profile, occupational
therapy practitioner can help to remediate the impact of any perceived
impairments. Thus, the outcomes of impaired occupational performance are
more likely to improve while helping the individual and the medical team with
proper medical management of the condition.
Diagnosis
Individuals with somatic symptom disorder typically have one or more somatic
symptoms that are distressing or have a significant impact on daily functioning.
Pain is the most common and specific form, but symptoms can range from
specific complaints of pain to complaints of general fatigue (de Waal et al.,
2004). The nature of the complaint can sometimes represent normal bodily
functions and does not have to necessarily indicate severe pathology. Diagnosis
is made based on the three criteria in the DSM-5 being met with the intensity
from mild to severe corresponding to the number of symptoms reported in the
second criterion (see Table 14.3).
TABLE 14.3 DSM V Diagnostic Criteria
From American Psychiatric Association. (2013). Diagnostic and Statistical Manual of Mental Disorders
(5th ed.). Washington, DC: Author.
Somatic symptoms are assessed based on psychological responses such as the

client’s emotions, thinking, and behavior of the symptoms. Diagnosing a client’s
symptom as unexplained is no longer an accepted practice as it was in the past
because often symptoms need to be further examined (American Psychiatric
Association, 2013; Dimsdale et al., 2013; Katz et al., 2015; Voigt et al., 2013).
Client’s suffering from somatic symptoms may feel that professionals are not
viewing their complaints seriously and will report lower satisfaction levels with
their medical care (American Psychiatric Association, 2013). To diagnose
somatic symptoms and related disorders, the physicians cannot discredit the
seriousness of the root cause as psychological and thus need to concurrently rule
out all root physiological causes. Once all physical systems of possible
alignment are ruled out, the physician will initiate a psychiatric treatment
approach, which would require referral to appropriate services and
pharmacological treatment. Table 14.3 details the DSM-5 diagnostic criteria the
physician uses to properly diagnose a patient with a type of somatic symptom
disorder.
Medical management for somatic symptom disorder can include treating the
somatic symptoms, reducing the health-related anxieties and preoccupations
with health concerns, and modifying or substituting the maladaptive illness
behaviors. The nature of the subjective complaints may not be clearly associated
with a physical cause but more of a learned sick role psychologically manifested.
To help determine if pain is related to a form of somatic sensory disorders, the
medical providers need to look holistically at the patient to see if the subjective
complaints and reports of physical impairments with daily tasks increase with
emotional distress and psychological disturbances (Katz et al., 2015). The
danger in jumping straight to dismissing the patient’s complaints and diagnosing
a mental condition can destroy the medical rapport between the provider and the
patient. The recognition and early diagnosis of mental health conditions is
considered essential in the proper management to help medical providers to not
improperly diagnose a patient with a form of somatic symptom disorder (Bener
et al., 2013).
The clinical association between somatic symptom disorders and other
mental health conditions is high. An example of comorbidities associated with
somatic symptom disorders is anxiety, stress, and depression (American
Psychiatric Association, 2013; Bener et al., 2013; Katz et al., 2015; Wolfe,
Walitt, Katz, & Häuser, 2014). It has been noted that psychological disorders
increase with age with the peak age group typically to be males aged 45 to 54
years old (Bener et al., 2013; Douzenis & Seretis, 2013; Katz et al., 2015). The
lack of clear methods on how to assess each criterion for somatic symptom
disorders often leads the medical providers to rely on their clinical judgment to
properly diagnose patients, thus impacting effective medical management by
providers (Bener et al., 2013; Katz et al., 2015; Knaster, Karlsson, Estlander, &
Kalso, 2012; Kroenke et al., 1998).
Medical diagnosis and management is essential for positive outcomes.
Cognitive-based therapy (CBT) has been noted as an effective means to treat
patients with various forms of somatic symptom disorders (Arnold et al., 2006;
Pfingsten, Hildebrandt, Leibing, Franz, & Saur, 1997; Riebel, Egloff, &
Witthöft, 2013). CBT is a multimodal approach to treatment, which includes a
focus on at least three of the following: relaxation, stress management, goal
setting/contracting, self-monitoring, self-talking, assertiveness training,
modeling, imagery, pacing, and/or family training as interventions that can be
delivered as individual or group sessions (Arnold et al., 2006; Chang &
Compton, 2013; Pfingsten et al., 1997; Riebel et al., 2013). CBT as an
occupational therapy technique would focus on stress management, activity
regulation, emotional awareness, cognitive restructuring, and improving
interpersonal communication (Arnold et al., 2006; Chang & Compton, 2013;
Pfingsten et al., 1997; Raine et al., 2015; Willmuth & Callahan, 2002). The goals
for CBT are to focus on the reduction of physiological arousal through relaxation
techniques, promote engagement in pleasurable and meaningful occupations,
increase self-awareness of emotions associated with the somatic complaints,
modify dysfunctional beliefs, and enhance communication of thoughts and
emotions associated with symptoms.
In moderate to severe cases, psychiatric consultation should be included to
allow for proper differential diagnosis of other mental health conditions that may
be influencing the somatic complaints. Medications used to treat somatic
symptom disorder focus on the comorbidities of symptoms such as depression
and anxiety with the prescription of tricyclic antidepressants, serotonin reuptake
inhibitors, serotonin-noradrenaline reuptake inhibitors, antipsychotics, and
general analgesics if warranted. Selective serotonin reuptake inhibitors (SSRIs)
and serotonin-noradrenaline reuptake inhibitors (SNRIs) are the first line of
treatment for patients with predominant pain symptoms and may also include
anticonvulsants such as gabapentin or pregabalin. These medications are used to
block or limit serotonin reuptake, thus blocking the stimulation of nerves for
pain, depression, and anxiety. Common brand names are noted in Table 14.4.
TABLE 14.4 Common Brand Medication Names for Somatic Symptoms

and Related Disorders
Occupational therapy in the treatment of somatic symptoms and related disorders

is critical and essential to help promote the well-being and engagement with
daily occupations. The Practice Framework 3rd Edition (The American
Occupational Therapy Association, 2014) views pain as generalized or localized
sensations that can be signaling potential or actual damage to bodily structures.
The role of occupational therapy as part of an interdisciplinary team to help treat
patients with reports of various pain syndromes is vital given our skill set of
analyzing the impact of somatic symptoms on client factors and occupational
performance.
The American Occupational Therapy Association (AOTA) defines occupational
performance as the skills observed while clients engage and move within their
environment to complete their daily occupations (The American Occupational
Therapy Association, 2014). To best understand the impact of somatic symptoms
and related disorders on occupational performance, one must have an
understanding of the impact of pain syndromes and the perception of pain on
client factors, which influence performance. When we think of pain, it is easy to
understand the cause and impact on the client if it is associated with an acute
injury or surgical procedure, but after the bodily structures heal and the presence
of pain still remains, the perplexity related to the chronic pain is unclear.
The occupational therapy practitioner is skillfully trained to analyze the
client through a different lens related to how the client must meet the demands of
the tasks to properly engage in the task. When looking at the client with reports
of chronic pain, the first thing an occupational therapy practitioner must review
is the client’s medical history to have an understanding of what root etiology
there was leading up to the current state. The review of the medical chart helps
the practitioner to identify possible chronic conditions that may have
permanently altered the basic bodily functions, such as a herniated disc with
nerve root impingement that is leading to structural integrity issues with the
functions of the joints, bones, and nerves of spine. In this situation, even with
surgery, the length of nerve root impingement, severity of the injury, and the
surgical versus nonsurgical medical treatment will start to paint a clearer picture
of what functional limitations a client may have when attempting to engage in
his or her daily occupations.
Regardless of surgical intervention or not, a person with reports of chronic
pain due to a herniated disc could present with decreased joint alignment, altered
sensations, and muscular impairment (strength, tone, reflexes, mass, etc.) along
the impinged nerve root distribution. Due to pain and changes with soft tissue
integrity from the injury, the client may learn various maladaptive methods to
engage in daily occupations to the fullest potential. Using a full functional
assessment of the client, the occupational therapy practitioner is able to analyze
how the clients move and participate in their daily occupational demands.
Through early identification of maladaptive posturing and body mechanics, the
practitioner is able to help the client reduce the impairments through remedial
and compensatory techniques. It is imperative to assess the client’s perception of
his or her physical impairments related to occupational performance.
The traditional medical model uses medications and surgical interventions to
try to correct soft tissue abnormalities and may overlook the mental component
of pain. Some models use health psychologists as part of the interdisciplinary
team to address this component. Occupational therapy uses a holistic approach
through a functional lens to assess and treat the client. The client’s perception of
perceived disability related to pain has an impact on the overall occupational
performance. The modification and correction of abnormal postures during the
engagement in occupations can reduce the strain on compromised joint integrity
and soft tissue, thus breaking the negative cycle for pain. When thinking of pain
and the impact on occupational performance, the occupational therapy
practitioner needs to understand and articulate the impact of the perception of
pain, physical impairments, and mental integrity in relation to occupational
performance.
A visualization of the client factors that can impact occupational
performance is represented by the symbol for the Olympics (see Fig. 14.1). Each
ring represents a different factor that can increase a client’s pain including stress,
anxiety, depression, poor sleep, poor body mechanics/ posture, and muscle
tension/weakness related to the client’s impairments. As one component in the
client’s life starts to spin more out of control, such as stress or poor body
mechanics, it causes the rest of the components to spin faster, thus leading to an
increased perception of pain and disability.
Figure 14.1 Factors that increase a client’s pain.

The components and their connectedness to one another when associated with
occupational performance can help the medical team provide the best treatment
strategies to help the client achieve his or her optimal occupational performance.
Occupational therapy plays a vital role in identifying the impacted client factors
and performance patterns that are leading to an altered perception by the client
related to occupational performance. Explaining to the client how to safely
engage to his or her fullest limits related to impaired joint and muscular systems
can help decrease the stress and anxiety often expressed and noted through self-
limiting behaviors due to a fear of increased pain while engaging in daily
occupations. The ability for the client to learn how to control one factor in the
Olympic ring example can help to eventually slow the negative impact of the
other rings that are impacted.
Through the use of meaningful and purposeful occupations, a client can
become lost in the task and work within his or her limits to safely complete daily
occupations that were perceived to be unmanageable. Opportunities for this can
be promoted though the occupational therapy session in various settings to
include work hardening/return-to-work programs, outpatient clinics, subacute
clinics, and pediatric settings. To understand the role of occupational therapy in
various settings, we will examine 2 clients and how their perception of pain and
disability related to somatic symptoms and related disorders may present in a
pediatric and an adult case.
CASE-STUDY 1
Ben is a 10-year-old boy who was in an accident and sustained a fracture
with mild medial and ulnar nerve involvement to his dominant right radius
and ulna while playing on the school playground. He was casted for 4 to 6
weeks after being set with internal fixation to protect the nerves and soft
tissue. Ben has poor pain tolerance and favored his right upper extremity by
not using it while casted leading to increased joint stiffness and atrophy in
the right hand. He was referred to occupational therapy after the school
teacher noted that he could not use a utensil for school tasks after the cast
was removed and he was asking for help to don his coat.
The occupational therapist reviewed the medical history provided by
the orthopedist and observed Ben in the classroom. He has no restriction,
and the orthopedist has encouraged him and his family to actively use his
right hand. His parents, however, sympathized with his reports of pain, thus
promoting him to not use his arm to complete his work for school if his arm
hurt. Ben was observed to not cross or transfer any objects past midline to
use his right upper extremity. He was noted to hold his right upper
extremity in severe shoulder protraction with elevation leading to tightness
in the upper trapezius and pectoralis major and minor, which also lead to
shoulder impingement from the abnormal posturing. The changes in his
body mechanics due to an abnormal posturing of his dominant upper
extremity lead to increased anterior shoulder pain and tenderness over the
anterior bursae.
As a result, Ben was noted to have decreased active and passive range
of motion (ROM), impaired strength across the right upper extremity equal
to 50% loss of full active ROM. Due to the ongoing pain and the perception
that if he used his right upper extremity he would hurt himself again, Ben is
at risk for developing complex regional pain syndrome. Ben also reported
that he liked to have others write and type papers for him and to not have to
do any house chores. When asked to play with a building block set using
his right hand, he started to cry as he expressed an increase in pain in his
hand. Ben’s family reported that he used to love to play with his 1-year-old
puppy but now could not hold him due to his injury. The occupational
therapist noted in the evaluation that when Ben was engaged and
completely distracted during functional testing, he had full passive ROM
and 75% of full active ROM in all the joints of the right upper extremity.
CASE-STUDY 2
Sara is a 36-year-old female veteran who presented to her primary care
physician with complaints and signs of increased muscle twitching and
decrease motor coordination in all four extremities. Sara, who just retired
from the military, denies any injury or trauma while in the service to have
led to the cause of these problems. Preliminary testing does not support a
general organic cause due to pathology. Sarah is referred to neurology for
further testing for which the doctor states clinically the symptoms present
like the early onset of amyotrophic lateral sclerosis (ALS), but medical
testing and electromyography (EMG) studies do not support the diagnosis.
Throughout testing, Sara starts to display an increase in symptoms for
which the neurologist noted and starts to ask her about her feelings about all
of the testing and results. Sara reports that the added medical appointments
for testing have increased her stress and anxiety over having ALS. The
neurologist refers Sara to a psychologist to help her with the added stress
and to rule out the possibility of symptoms stemming from a psychological
component.
Sara is angered by the referral but still attends the appointment for the
psychologist. Testing now notes a positive screen for depression and
anxiety along with Sara reporting that she was a victim of an assault upon
leaving the service. The stress, anxiety, and fear of being a victim have left
her unable to work, properly care for personal hygiene, or take care of her
home. The psychologist has a close working relationship with the therapy
department and refers Sara to occupational therapy for assistance with
reengagement into daily functional tasks. The occupational therapist upon
evaluation notes generalized poor personal hygiene, impaired
accommodation to various settings, a hyperactive startle reflex, 5/5 strength
and full active ROM in all extremities, slowed mental processing, and
reduced self-awareness. The medical record was reviewed, and the therapist
notes through the medical chart and semistructured interview that the
symptoms increased with stress and appeared to have manifested after the
trauma of an assault. Sara is working with the psychologist on the
psychological impact of the assault, but the physical manifestations have
precluded her from being able to perform self-care. Due to the muscle
twitching impacting her gross and fine motor coordination, she is a fall risk
during bathing and dressing. Her impaired gross and fine motor
coordination also leads to frequent dropping of items and precludes the use
of utensils for self-feeding. Sara would love to be able to bathe and dress
again within a reasonable amount of time.
LEARNING RESOURCES
Somatic Symptom Disorders YouTube Video
https://www.youtube.com/watch?v=h5eHWYJTlsE
Somatic Symptom Disorders Summary and Screening Tool
http://www.aafp.org/afp/2007/1101/p1333.html
Treatment of Functional Impairment in Severe Somatoform Pain Disorder
Article http://jpepsy.oxfordjournals.org/content/26/7/429.full
Conversion Disorder
https://www.nlm.nih.gov/medlineplus/ency/article/000954.htm
Conversion Disorder CBS This Morning YouTube Clip
https://www.youtube.com/watch?v=gbQK8ucIcAw
Danielle’s Journey with Conversion Disorder YouTube
https://www.youtube.com/watch?v=6xhypWbI0bk
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C H A P T E R
15 Feeding and Eating Disorders
Amy Wagenfeld Linda M. Olson
KEY TERMS
Anorexia nervosa Avoidant/restrictive food intake disorder Bulimia nervosa
Cognitive-behavioral therapy Eating disorder Family-based therapy Feeding
disorder Pica Rumination
Lucy was admitted to 4C, the inpatient psychiatric unit at her regional hospital.
At admission, Lucy is 5′6" tall and weighs 90 lb. This is Lucy’s second day in
the unit, and her occupational therapist (OT) has just arrived for her initial
interview. Lucy is frantic. She does not want to be on the unit; she wants to go
for a long run. She is not allowed beyond the end of the hallway, which she has
estimated to be 50 yards long. If she briskly walks back and forth for 2 hours,
she might actually burn off some calories. But so far, whenever she starts to
walk, a nurse gently guides her back to her room. Good thing the OT has arrived;
maybe he will help her figure out ways to get exercise, because that is what
matters right now.With intense societal pressure to be thin and social media
perpetuating this trend, so-called celebrity “role models” often succumb to the
pressure and place excessive personal value on thinness and weight control. The
domino effect is that achieving extreme thinness can become a goal for those
who idolize celebrity role models. Negatively comparing oneself with the
societal “ideal” can lead to a distorted self-image (Treasure, Claudino, & Zucker,
2010) and, for too many, eating disorders. This chapter discusses six feeding
and eating disorders: how they are diagnosed and classified and their
prevalence and clinical presentations. Evidence-based research on the causality
and intervention of feeding and eating disorders will be explored. The impact of
feeding and eating disorders on occupational performance will be discussed
throughout the chapter as well as in the case studies at the conclusion of the
chapter.
Activities that contribute to health and occupational performance include eating
a balanced diet, engaging in meaningful leisure pursuits, exercising in
moderation, and participating in fulfilling social relationships. Food restricting
or purging behaviors “prevent clients from engaging in activities that contribute
to health” (Costa, 2009, p. 13). Many people experience internal conflict with
regard to eating at some point in their lives, but not to the point at which it
threatens good health and occupational performance. According to the DSM-5
(American Psychiatric Association [APA], 2013), feeding and eating disorders
include anorexia nervosa, bulimia nervosa, binge eating, pica, rumination,
and avoidant/restrictive food intake disorder. Eating disorders (particularly
anorexia nervosa, bulimia nervosa, and binge eating disorder) are among the
most common health concerns in young people, particularly female adolescents
and young women (Treasure et al., 2010), but to a lesser extent, men are also
impacted (Grave, 2011). Most adults seeking treatment for eating disorders are
in their 20s or 30s, having dealt with their issues for on average 8 years
(Murphy, Straebler, Basden, Cooper, & Fairburn, 2012). Eating disorders are
understood to arise from a combination of behavioral, biological, emotional,
psychological, interpersonal, and social factors (Costa, 2009). While each of the
feeding and eating disorders has different clinical presentations, these six
disorders feature severe disturbances in eating behavior that clinically impede an
individual’s life. Life-threatening effects of eating disorders can be electrolyte
imbalance, cardiac arrhythmias, and intercurrent infarction (Treasure et al.,
2010). Anxiety, low self-esteem, obsessional thoughts and actions, feelings of
low self-worth, and perfectionism are classic characteristics associated with
eating disorders. Hypotension and bradycardia, constipation, GI issues, and
long-term estrogen deficiency are common side effects of anorexia nervosa
(Grave, 2011). Other serious long-term effects of anorexia nervosa and bulimia
nervosa include reproductive issues, osteopenia and osteoporosis, impaired brain
growth, and dental health.
On a more systemic level, eating disorders can threaten to take over the
entire family system; the family becomes consumed by it and has limited
community engagement. Mealtimes become war zones. For children and
adolescents with eating disorders, constant prompting to eat can lead to created
dependency (Clark & Nayar, 2012).
Anorexia Nervosa
Anorexia nervosa is described as the oldest recorded eating disorder, with
accounts of it dating back to the 19th century. Anorexia nervosa presents as a
profound refusal to maintain a normal body weight at 85% of expected weight,
excessive concern with and distorted body image, and failure to recognize that
there is a problem (Quiles-Cestari & Pessa Ribeiro, 2012; Trudeau, 2016, in
press). It is a relentless pursuit of thinness through diet and exercise with an
accompanying intense and unremitting fear of gaining weight or becoming fat,
despite being extremely thin (Treasure et al., 2010; Walsh, 2011). Food is always
on the minds of those with anorexia nervosa. For example, a person might
obsess about the number of calories, the fat content, or how many minutes of
exercise it will take to burn off a desperately desired cookie.
Bulimia Nervosa
Bulimia nervosa features episodic and recurring binge eating and purging of
food to avoid weight gain. Behaviors such as self-induced vomiting, misuse of
laxatives, diuretics, enemas, or other medications to avoid weight gain are
hallmarks of bulimia nervosa (Treasure et al., 2010; Trudeau, in press; Walsh,
2011). Fasting or excessive exercise to compensate for high caloric intake is
common in individuals with bulimia nervosa as well as anorexia nervosa. Like
anorexia nervosa, a distorted perception of body image (shape and weight) is
common for individuals with bulimia nervosa.
Binge Eating Disorder
Binge eating disorder was first described in the medical literature in 1952
(Walsh, 2011). Binge eating disorder is a condition in which there are both
recurrent episodes of excessive eating within a discrete period of time and a
perceived sense of lack of control over the eating. Most individuals with binge
eating disorder tend to be “overweight, obese, and middle-aged” (Walsh, 2011,
p. 526). The difference between binge eating disorder and bulimia nervosa is that
individuals with binge eating disorder do not engage in compensatory behaviors
like purging or misuse of laxative and diet pills. A transition from restrictive
eating to binge eating is common, but going from binge eating to severely
restricted eating is less so (Treasure et al., 2010).
Pica
Pica is a dangerous form of self-injurious behavior. It is described as a perverted
appetite for nonnutritive, nonfood substances such as clay, soil, laundry starch,
hair, chalk, crayons, and ashes (Aparna, Austin, & Mattews, 2012; Tabers,
2013). The word pica is derived from the Latin for magpie, a species of bird that
eats whatever it finds (Aparna et al., 2012). Pica can lead to death or life-
threatening consequences due to choking or intestinal obstruction and may
require surgery to remove inedible objects (Williams & McAdam, 2012). It can
also lead to poisoning, burns, and parasitic infections and cause significant harm
to dental and digestive structures.
Rumination
Rumination is the nonpurposeful regurgitation of recently ingested food from the
stomach to the mouth, where it is either rechewed, reswallowed, or expelled
(Eckern, Stevens, & Mitchell, 1999; Mousa, Montgomery, & Alioto, 2014). It
has been recognized in the medical literature for over 100 years (Eckern et al.,
1999). Regurgitation typically happens during or immediately following a
feeding. The sensation associated with the regurgitation is felt as pressure, the
need to belch, nausea, pain, or discomfort (Mousa et al., 2014). Individuals with
rumination typically describe the process as being effortless with no gagging or
retching (Mousa et al., 2014). Those with rumination understand that the food is
undigested yet report that it tastes normal. Lacking social conventionality,
rumination is often a very secretive behavior (Eckern et al., 1999). It is not life
threatening but can have medical and psychological ramifications for the
individual and family.
Avoidant/Restrictive Food Intake Disorder
Avoidant/restrictive food intake disorder is a new diagnostic category in the
DSM-5 (APA, 2013). It replaces and extends the category of Feeding Disorder of
Infancy or Early Childhood in the DSM-IV. The reason for the change was to
expand the category to make the disorder applicable across the life span and to
make the diagnostic criteria more explicit (APA, 2013). While typically seen in
children and adolescents, avoidant/restrictive food intake disorder can also be
diagnosed in adults (Bryant-Waugh & Kreipe, 2012). Avoidant/restrictive food
intake disorder may present in a variety of ways; some individuals eat only a
very narrow range of foods, while others restrict food intake in response to
emotional crises, unpleasant experiences, or untoward feelings (Bryant-Waugh
& Kreipe, 2012). Food avoidance is not driven by cognitive misperceptions
about weight or body shape, nor is it the result of lack of availability of food or a
culturally sanctioned practice (Bryant-Waugh & Kreipe, 2012; Katzman,
Stevens, & Norris, 2014). Those with avoidant/restrictive food intake disorder
are more likely to have a coexisting medical condition such as autism spectrum
disorder, attention deficit hyperactivity disorder, anxiety disorder, and obsessive-
compulsive disorder than individuals with anorexia nervosa or bulimia nervosa
(Bryant-Waugh & Kreipe, 2012; Fisher et al., 2014). It has been suggested that
there are five types of food refusal. They include
Learning dependent food refusal

Medical complications–related food refusal
Selective food refusal
Fear-based food refusal
Appetite awareness and autonomy-based food refusal (Dovey and
colleagues as cited in Kreipe & Palomaki, 2012)
An individual with avoidant/restrictive food intake disorder is dependent on

enteral feeding and/or oral nutritional supplements and may demonstrate notable
interference with psychosocial function (Bryant-Waugh & Kreipe, 2012; Norris
& Stevens, 2014).
Etiology
The heritable characteristics of eating disorders are strong. Twin and family
studies show the incidence rates to be between 50% and 83% (Treasure et al.,
2010). Research also indicates that eating disorders represent a complex
interaction of physiological, genetic, psychological, social-emotional, and
cognitive factors. There is a strong link between coexisting mental health
conditions such as depression and anxiety, borderline personality disorder,
affective disorder, substance abuse (the latter two more closely associated with
bulimia nervosa) (Treasure et al., 2010), and eating disorders. Further, there is a
relationship between general adversity (abuse, neglect) and increased risk of
developing an eating disorder (Treasure et al., 2010). Parallels are also noted
between eating disorders and addictions; both have associated tendencies such as
compulsive behaviors, diminished self-control, and engagement in repetitive
behaviors despite knowing the negative consequences (Costa, 2009).
For some individuals with bulimia nervosa and binge eating disorder,
overeating may be considered a form of self-regulation (Gardiner & Brown,
2010). An explanation for this behavior is that when people are under stress,
eating helps to balance the body’s biochemistry; serotonin levels increase and
cortisol decreases (Gardiner & Brown, 2010). From a sensory regulation
perspective, people who are hyporesponsive to sensory input (tactile and
proprioceptive) may be drawn to consuming large amounts of highly textured
and flavored foods (or nonnutritive substances) in an effort to modulate
(Gardiner & Brown, 2010). This potential causation of eating disorders could
apply to individuals with bulimia nervosa, binge eating disorder, and pica.
Although there are similarities, there are also etiological characteristics specific
to each eating disorder. A description of etiology specific to each eating and
feeding disorder follows.
The cause of anorexia nervosa is multifactorial and includes genetics,
sociocultural, familial, and individual factors and/or experiencing an adverse
event, personality, and psychological vulnerability (Quiles-Cestari & Pessa
Ribeiro, 2012). Like anorexia nervosa, the cause of bulimia nervosa is most
likely multifactorial and includes sociocultural, familial, and individual
characteristics as well as psychological factors (Frank, 2015).
One suggested cause of binge eating disorder may be a genetic mutation of
the melanocortin 4 receptor gene, which makes a protein that stimulates hunger
(Branson et al., 2003). A mutation in this gene may lead to reduced protein
production so that the body (inaccurately) feels excessive hunger (Branson et al.,
2003). Like anorexia nervosa and bulimia nervosa, binge eating disorder may
also have psychological roots, for example, negative emotions, loneliness, and
anger (Zeek, Stelzer, Linster, Joos, & Hartmann, 2011).
The practice of pica has been reported as far back as 1800 BCE in regions of
the Far and Middle East (n.a., 2011). Since the time of Hippocrates, it has been
proposed that pica is a symptom of anemia, but it has never been proven (Aparna
et al., 2012). The question remains as to whether iron deficiency is a cause or
effect of pica (Aparna et al., 2012). No further etiology for pica has been
reported in the medical or psychological literature.
The cause of rumination is not understood. Rumination is commonly
believed to be behavioral, that is, a learned disorder that allows for voluntary
relaxation of the diaphragm that leads to belching and regurgitation of food
(Talley, 2011).
Although not for certain, avoidant/restrictive food intake disorder may be
caused by mechanical oral motor factors such as low tone or coordination, tactile
defensiveness, previous history of choking, and/or a conditioned response to
gastrointestinal issues that preclude food intake (Kreipe & Palomaki, 2012).
These issues combined with an individual’s medical history and, for children,
caregiver response to these issues and subsequent learned behaviors may
exacerbate avoidant/restrictive food intake disorder (Kreipe & Palomaki, 2012).
It is estimated that up to 24 million people of all ages and genders have anorexia,
bulimia, or binge eating disorder in the United States with the highest incidence
rate of anorexia nervosa and bulimia nervosa occurring between the age of 10
and 19 years (Renfrew Center Foundation for Eating Disorders, 2003). With
only 35% of people with anorexia, bulimia, or binge eating disorder receiving
specialized treatment (Noordenbox, 2002), the incidence rate for these three
eating disorders cannot be considered absolute. More specific information on
incidence and prevalence by diagnosis follows.
Anorexia Nervosa
Anorexia nervosa was previously thought to impact only young Caucasian
women with upper socioeconomic status. It actually affects both men and
women of all ages, races, and ethnicities across the world (Costa, 2009), but
mostly younger Caucasian women. The prevalence rate is reported at a range
between 0.3% and 1% (Hoek, 2007; Renfrew Center Foundation for Eating
Disorders, 2003). Comorbidity is the rule rather than the exception, with anxiety
and depression the most common. It has been estimated that one-fifth of people
with attention deficit hyperactivity disorder (ADHD) and autism spectrum
disorder have anorexia nervosa (Treasure et al., 2010).
Bulimia Nervosa
A 12-month prevalence of bulimia nervosa among young females is reported at
1% to 1.5% (APA, 2013; Hay & Claudino, 2010; Hoek, 2007). Prevalence is
highest among young adults since it peaks in older adolescence and early
adulthood. There is approximately a “10:1 ratio of females versus males” (APA,
2013, p. 347). Like anorexia nervosa, comorbid psychological conditions such as
anxiety and depression may also be present in individuals with bulimia nervosa.
The 12-month prevalence of binge eating disorder in the United States adult
population is 1.6% female and 0.8% male. It is more prevalent among
individuals seeking weight loss treatment than in the general population and is
equally as prevalent among white females as ethnic minorities (APA, 2013, p.
351).
Pica
Pica has been observed in people across all cultures and age groups (Aparna et
al., 2012). In the United States, it appears to be more common among
individuals living in the Southeast. Considered by some to be a learned behavior,
it is more frequently noted in individuals with autism spectrum disorder, Prader-
Willi syndrome, and psychological conditions such as stress and anxiety (APA,
2013; n.a., 2011). It is difficult to determine the prevalence rate because people
are reluctant to admit to engaging in pica and the definitions of pica vary
(Blinder, 2008) because of varied cultural practices.
Rumination
Originally, it was thought that rumination occurred only in intellectually
impaired children or adults, but recent research suggests that healthy individuals
with no cognitive challenges can also be diagnosed with rumination syndrome
(Mousa et al., 2014). To that end, there have been no systematic incidence
studies on rumination in humans (Talley, 2011).
There has been limited study of the prevalence of avoidant/resistant food intake
disorder (Nicely, Lane-Loney, Masciulli, Hollenbeak, & Ornstein, 2014). Studies
have noted rates range from 1.4% to 5% for youth and adolescents treated at
eating disorder facilities (Fisher et al., 2014; Norris et al., 2013; Ornstein et al.,
2013). Being a new category in the DSM-5 (APA, 2013), future prevalence study
has been recommended.
Signs and Symptoms
There are a number of signs and symptoms common among the six eating and
feeding disorders. They are presented below in Table 15.1.
TABLE 15.1 Signs and Symptoms Associated with Feeding and Eating
Disorders
From Eckern et al. (1999), Gardiner and Brown (2010), Grave (2011), Mousa et al. (2014), and Treasure et
al. (2010).
Although eating disorders have the highest mortality rate of any mental disorder,
like diagnostic rates, the mortality rate of eating disorders varies depending on
studies and reports (Crow et al., 2009). Part of the reason why there is a large
variance in the reported number of deaths caused by eating disorders is that
those with an eating disorder may ultimately die of heart failure, organ failure,
malnutrition, or suicide. Often, the medical complications of death are reported
instead of the eating disorder that compromised a person’s health.
The complex nature and frequency of relapse of anorexia nervosa make its
prognosis the bleakest among eating and feeding disorders (Miller & Golden,
2010). Over 20% of individuals with anorexia nervosa continue to present with
the condition at long-term follow-up. Vomiting and purgative abuse have been
shown to lead to the worst prognosis, while excessive exercise and dieting does
not necessarily portend poor prognosis (Steinhausen, 2008). It has been found
that the outcome for adolescent onset is more positive than for adult onset
(Steinhausen, Boyadjieva, Griogoroiu-Serbanescu, & Neumarker, 2003). For
younger individuals, a positive parent-child relationship has protective qualities
to diminish poor outcomes for both anorexia nervosa and bulimia nervosa
(Steiner & Martine, 2012). (Herzog et al., 1999) and colleagues’ longitudinal
study of 246 patients with anorexia nervosa and bulimia found that 74% of
individuals with bulimia nervosa achieved a full recovery compared to 33% of
those with anorexia nervosa. Partial recovery occurred in 99% of individuals
with bulimia nervosa and 83% of those with anorexia nervosa. Relapse rate was
found to be approximately 33% for both anorexia nervosa and bulimia nervosa
(2003). Recovery from anorexia nervosa becomes more difficult the longer it
persists, yet the opposite is true for bulimia nervosa (Treasure, Claudino, &
Zucker, 2010). Coexisting obsessive-compulsive characteristics add to the
chronicity of both anorexia nervosa and bulimia nervosa (Steiner & Martine,
2012). The prognosis for binge eating disorder is more favorable than for
bulimia nervosa. The remission rate is >50% (National Association of Anorexia
Nervosa and Associated Disorders, 2015).
Pica may stop spontaneously after several months or require dopamine-
regulating medication to control the behaviors (National Library of Medicine
[NLM], 2015a). It tends to be more persistent and leads to poorer prognosis in
individuals with developmental disabilities than the general population.
Rumination may also stop spontaneously or require psychotherapy or aversive
conditioning (NLM, 2015a). Like pica, for individuals with developmental and
intellectual disabilities, the prognosis for rumination tends to be poorer than for
the general population (NLM, 2015b). The prognosis for individuals with
avoidant/resistant food intake has not been reported in the literature, but early
treatment is very important. Left untreated, it may lead to anorexia nervosa or
bulimia nervosa (Sheppard Pratt Center for Eating Disorders, 2015).
Until clients are ready to make behavioral changes, they will not recover
from an eating disorder. Recovery also depends on hope, healing, empowerment,
and connection (Clark & Nayar, 2012). Recovery from an eating disorder has
been described as “when individuals with a history of an eating disorder appear
indistinguishable from healthy controls” (Bardone-Cone et al., 2009, p. 3). From
an occupational therapy perspective, recovery is dependent upon achieving a
balance between work, leisure, and self-care (Crist, Davis, & Coffin, 2000).
Diagnosis
Anorexia Nervosa
The diagnostic criteria for anorexia nervosa include “restriction of caloric intake
relative to body requirements that leads to significantly low body weight in
relationship to age, sex, developmental trajectory, and physical health status”
(APA, 2013, p. 339). Suicide rate is high for individuals with anorexia and is
reported at 12 per 100,000 annually (APA, 2013). The ICD-9-CM (International
Statistical Classification of Diseases and Related Health Problems) code for
anorexia nervosa is 307.1. The ICD-10-CM codes vary based on subtype;
restricting type is F50.01 and binge eating/purging type is F50.02.
Bulimia Nervosa
Bulimia nervosa is diagnosed when an individual engages in binge eating and
compensatory purging behaviors at least one time per week for 3 months and
does not occur exclusively during episodes of anorexia nervosa (APA, 2013).
Eating occurs within a discrete amount of time (e.g., 1 hour). Food intake is
significantly larger than what would be typically expected, and the individual
experiences a sense of loss of control of the eating (APA, 2013). Compensatory
behaviors occur such as vomiting, misuse of laxative and diuretics, fasting,
and/or excessive exercise (APA, 2013). Unlike those with anorexia nervosa,
individuals with bulimia nervosa tend to be of normal weight or slightly
overweight (Frank, 2015). An individual with bulimia nervosa presents with
self-evaluation that is “overly influenced by body shape and weight” (APA,
2013, p. 345). The ICD-9-CM code for bulimia nervosa is 307.51 and the ICD-
10-CM code is F50.2.
Binge eating disorder refers to recurring episodes of eating quickly, with loss of
control, and ingesting significantly more food in a discrete period of time (e.g., 1
hour) than most people would eat under similar circumstances regardless of
hunger or satiety. It is associated with marked distress and occurs, on average, at
least once a week over 3 months (APA, 2013). Binge eating disorder is not
associated with inappropriate compensatory behaviors, nor does it occur
exclusively during the course of anorexia nervosa or bulimia nervosa (APA,
2013, p. 351). The ICD-9-Cm code is 307.51, and the ICD-10-CM code is F50.8.
Pica
To be diagnosed with pica, the individual must have engaged in eating nonfood
items for at least 1 month (APA, 2013; Aparna et al., 2012). The ICD-9-CM
code for pica is 307.52, and the ICD-10-CM codes for childhood pica are F98.3
and F50.8 for adults.
Rumination
Rumination is diagnosed via the Rome III criteria, which includes symptoms
such as regurgitation and rechewing or expulsion of food, with no retching for at
least 1 month, with onset at least 6 months prior to diagnosis. While most often
diagnosed in children with intellectual disabilities, adult cases in the general
population have been documented. Rumination does not occur exclusively
within the context of anorexia nervosa, bulimia nervosa, binge eating disorder, or
avoidant/restrictive food intake disorder (APA, 2013; Mousa et al., 2014). The
ICD-9-CM code for rumination is 307.53, and the ICD-10-CM code is F98.21.
Nutritional insufficiency and weight loss resulting from excluding specific food
groups or inadequate vitamin or mineral intake must occur for a diagnosis of
avoidant/restrictive food intake disorder (Bryant-Waugh & Kreipe, 2012). There
are no validated assessments to diagnose avoidant/restrictive food intake
disorder. Assessment is typically via interview with the client (or family if child
is young), weight and height measurement, and assessment of clinical intake. An
avoidant/restrictive food intake disorder cannot be associated with an
individual’s self-perception of his/her body weight or shape, associated with a
concurrent medical condition such as food allergy or intolerance, or better
explained by psychiatric conditions such as schizophrenia, in which an
individual might have delusions about the safety of his or her food (Bryant-
Waugh & Kreipe, 2012; Norris & Stevens, 2014, p. 445), cultural practices, or
with the presence of another eating disorder (APA, 2013). The ICD-9-CM code
for avoidant/restrictive food intake disorder is 307.59 and the ICD-10-CM code
is F50.8.
Interpersonal challenges tend to worsen self-esteem. These challenges can be the
catalyst to engage in atypical eating behaviors as a maladaptive way to gain self-
control (Murphy et al., 2012). Fully manifesting eating disorders tend to occur in
the context of or are amplified by traumatic interpersonal events (Murphy et al.,
2012).
Psychological therapies are the typical means for managing eating disorders.
Self-help groups and family interventions are the main types of therapy
intervention for individuals with eating disorders (Gardiner & Brown, 2010).
Cognitive-behavioral therapy (CBT) is the most common psychological
intervention for adults and family-based therapy (FBT) for children, youth, and
adolescents (Kosmerly, Waller, & Robinson, 2015; Treasure et al., 2010). Both
are evidence-based treatment approaches for individuals with eating disorders.
These highly structured interventions have been shown to be more effective than
less structured psychological treatment approaches. If psychotherapy services
are not available or are ineffective, the next treatment option for those with
eating disorders (anorexia nervosa or bulimia nervosa) may be the use of
medications such as antidepressants or mood stabilizers (Treasure et al., 2010).
In the case of rumination, a muscle relaxant such as baclofen may be used to
“reduce regurgitation and belching… and decrease how often the lower
esophageal sphincter relaxes” (Mousa et al., 2014, p. 4). For some eating
disorders, particularly pica, applied behavioral analysis treatment techniques are
often the preferred treatment method.
When there is a concern regarding an individual’s physical or emotional
safety, especially in individuals with anorexia nervosa, inpatient hospitalization
may be required. The goal of this hospitalization would be to reestablish a safe
nutritional status and address emotional issues.
Typical treatment for avoidant/restrictive food intake disorder includes
psychological or behavioral interventions, nutritional counseling, and medical
monitoring (Katzman & Stevens, 2014). Avoidant/restrictive food intake
disorder can be managed clinically using food or drink supplements high in
calories and fat or via enteric feeding (Bryant-Waugh & Kreipe, 2012).
The effect of an eating disorder negatively influences occupational performance.
Behaviors are obsessive and maladaptive. Eating disorders globally impact a
person’s ability and drive to perform adaptive occupations (Clark & Nayar,
2012). While loss of occupational role is a key area of concern for all individuals
with eating disorders, anorexia nervosa, in particular, can literally consume a
person and lead to a singular focus on food to the exclusion of all else in daily
life (Trudeau, 2016). Diminished engagement in purposeful occupation is noted
and accompanied by unreasonably high self-expectations. It is not uncommon
for an individual with an eating disorder to lose sight of what is (was) fun and
important for him or her. Personal causation is at issue. Isolation is common.
Roles that are lost may include friend, spouse, parent, worker, and hobbyist
(Quiles-Cestari & Pessa Ribeiro, 2012). This loss leads to a dearth of
interpersonal connections and even, due to the obsessive inward nature of the
disorders, lack of empathy for others.
A client-centered approach to occupational therapy intervention for
individuals with eating and feeding disorders entails finding meaning and
purpose in life. It can also focus on self-esteem because it is a critical contributor
to recovery from eating disorders and an important part of the treatment process
(Karpowicz, Skerseter, & Nevonen, 2009). Engagement in social and community
activity bolsters self-esteem. Intervention can be directed at increasing
socialization, a life function often lost when an individual is entrenched in an
eating disorder (Clark & Nayar, 2012). Motivational interviews may help a client
with an eating disorder prepare to authentically engage in making adaptive
behavioral changes. Occupational therapy can focus on enhancing self-efficacy
and self-concept, identifying role deficits, and identifying positive role models
(Gardiner & Brown, 2010). For instance, with anorexia nervosa and bulimia
nervosa, exercise may become the focal point of a client’s life—all day, every
day. The occupational therapy practitioner must become skilled at understanding
the client’s desire and motivation to engage in an activity and the activity
demands and associated contraindications. Striking a balance between autonomy
and safety is paramount. Too much high-intensity activity will perpetuate
undesired behaviors and weight loss. An occupational therapy practitioner needs
to be aware of this persistent drive and work with the individual to reevaluate the
value of such behavior. It becomes, in essence, helping the client find balance
between engaging in moderate exercise and engagement in other meaningful
work and leisure activities. This entails client-centered intervention to engage in
meaningful active recreation that stops before becoming excessive, to increase
socialization, to set limits, and to reduce anxiety and depression (Gardiner &
Brown, 2010).
Conclusion
To varying extents, all aspects of daily life are negatively impacted by an eating
disorder. Meal preparation, grocery and clothes shopping, interpersonal
relationships, work, and leisure all suffer. An important role that occupational
therapy practitioners can perform when working with clients with eating
disorders is to help them organize their lives in order to find a workable balance
between engaging in daily skills that they need to do in order to achieve a sense
of personal fulfillment (Costa, 2009). Eating disorders are serious mental health
conditions that warrant careful and sensitive client-centered treatment from an
interdisciplinary team of health practitioners.
CASE-STUDY 1
Lucy is a 38-year-old manager of a local bank in a midsized town in the
Pacific Northwest. Lucy works about 50 hours a week, where she
supervises 10 employees, and is responsible for employee training and
customer service. Lucy has been married to Zachary, an elementary school
music teacher, for 15 years. They have three children, ages 12, 6, and 2, all
conceived via in vitro fertilization. They try to share household tasks, but
due to Lucy’s long work hours, Zachary ends up doing almost all of it,
including child care. Lucy used to love to cook, garden, run, do Zumba,
hike, bike, and swim. Girls’ night at the local dinner theatre was also
something Lucy looked forward to every month. Now, she spends her time
being in motion: walking, pacing, and marching in place when she is at
work, home, the grocery store, and everywhere. She goes to the gym 6 days
a week before work, where she does strength training and cardio workouts.
Before bed, she does 100 sit-ups and 50 push-ups as well as prepares
lunches for everyone for the next day. Her favorite lunch is two cups of raw
chopped chard and a whole sliced cucumber. Sometimes, she also brings
celery sticks. She used to pack some carrots, but decided that while they
were tasty, she really did not need to eat them. She is thirsty, but doesn’t
like to drink much because it makes her stomach seem huge when she looks
down.
Lucy’s story actually begins when she was 12 years old. Lucy is the
oldest child in a family with six children. Life was very hectic at home, and
there was often a lot of strife between Lucy’s parents and siblings. She was
always an overachiever, always seeking to be the best at everything she did,
from playing the viola and softball to being on student council and having
the most friends. Lucy sought out praise and approval and was loath to
make a mistake, much less be chastised for it. No matter what she achieved,
it never seemed to be enough.
The summer after seventh grade, Lucy’s aunt and uncle invited her to
come to Nova Scotia to babysit their four children while they rehearsed for
a musical theatre troupe. The hours were long and the responsibility intense
for a 12-year-old girl. She had wrestled with whether to accept the job or
not. It was a chance to go someplace she had never been, but going would
mean a summer away from friends and her routines, which were important
to her. And she was getting intense pressure from her parents to say yes,
which ultimately, being the good girl, she agreed to go. Once she arrived in
Nova Scotia, she realized that she was in over her head. Her aunt and uncle
were absent for much of the day and night. The children were hard to take
care of, on top of making meals and trying to keep the summer cottage
clean and tidy. There was no time for Lucy to do what she wanted to do,
except for an occasional day off, when she hiked five miles each way into
town to look at shop windows as she longed to be home with her family and
back into her routine.
While in Nova Scotia, Lucy began to experience a great deal of comfort
in restricting what she ate and found that it gave her an incredible high.
Finally, she could control something in what had become a very chaotic
life. She tried to convey how she felt to her mom, but was dismissed and
told to stay the course and honor her commitment; after all, she was a good
girl and needed to remember that. By the end of the summer, Lucy went
from being a healthy 85-lb girl who had her first period 3 months before the
Nova Scotia trip to a 63-lb waif, whose period ultimately stopped for 3
years. When her parents and siblings came to pick her up for their end of
the summer vacation, her parents were horrified, but they had no idea what
to do. So, they did nothing. Lucy continued to bask in the glory of being in
charge of her weight and loved how she could go for a day without eating
and exercise seemingly nonstop. She was in full control of herself, but also
felt very out of control at the same time, which was confusing. Over the
years, Lucy would cycle through times when she could allow herself to eat,
and then, following another traumatic life event or when she felt her busy
life spinning out of control, the cycle would begin again.
CASE-STUDY 2
Milo is 4 years old and was diagnosed with autism spectrum disorder
(ASD) when he was 3 years old. He lives in Vermont with his mother and
two older sisters. Both sisters are typically developing and attend the local
elementary school. Milo’s cousin Jonas also has ASD. Milo was verbal
until age two, when he began to lose his speech. Although Milo’s Mom
(Candice) was puzzled, she attributed Milo’s loss of speech to having two
older sisters who spoke for him. Although his motor skills developed
normally, there was a certain quality to them that Candice at times thought
were just not right, but again, she shrugged it off by telling herself that Milo
was his own person and was just developing according to “his” plan. And
didn’t boys usually develop more slowly than girls? At least it seemed that
way with what Candice had experienced with her daughters. When first
enrolled in preschool at age 2 years 6 months, his teachers immediately
identified that things were not “right” with Milo. He did not talk, avoided
interacting with his peers, walked only on his toes, and preferred to twirl his
fingers and, at every chance he could, to eat dirt and rocks. Outside play
was scary for the teachers; one needed to be by Milo’s side at every minute
to make certain that Milo was not putting dirt or rocks in his mouth. They
did not know how to stop this behavior and were very concerned that Milo
would get sick or, worse, to even choke on something or eat something
poisonous and die. When the lead teacher called Candice in for a
conference, the classroom teaching staff expressed their concerns about
Milo’s behaviors and strongly recommended that Milo get evaluated to
determine what might be causing these behaviors. Reluctantly, Candice
agreed. It took her several months to process that Milo might have a
condition, so time went by and no evaluation was done. When she was
ready, Candice made an appointment with her pediatrician, and after
extensive testing, Milo was diagnosed, at age 3, with ASD and pica.
CASE-STUDY 3
It is just another typical Friday night for 28-year-old Treena. After a long
day of working as a front desk clerk at a hotel, she heads to the grocery
store. She tells herself that this time it will be different. No more buying
three gallons of mint chocolate chip ice cream; instead, she is going to buy
a large platter of preprepared fruits and eat them while doing what she does
every Friday night: watching reruns of a fashion show in which the hosts
help women learn to dress to their best potential for their body type. She
longs to have a friend to join her, but it seems like they are all busy with
their own lives and their own families. No one wants to hang out with her.
It is the story of her life, it seems.
At the store, Treena heads to the produce aisle and, after a lot of
deliberation, selects a platter of fruit that comes with a pint of fruit dip.
This, she thinks, will be the start of a new me. But since she was already at
the store, it was logical to pick up a few more things that were on sale,
including frozen pancakes and her favorite brand of peanut butter. As she
was passing through the frozen food aisle, she saw the ice cream and
decided to buy it, but promised herself that she could portion control and
not eat a full gallon while watching a 60-minute episode of the fashion
show. Besides, she was going to eat her fruit. And that would be enough.
Once home, she puts away her groceries and jumps into the shower,
noticing that the waistband of her pants had made a deep impression on her
stomach, maybe deeper than ever. After comfortably settling onto the couch
with the television remote in hand, she began to think about the ice cream.
“No, fruit first,” Treena told herself. So she ate the platter of fruit, dipping
pieces of fruit in the caramel dip and then swirling each piece in the peanut
butter she had picked up on sale. Then, despite being full, she opened the
mint chocolate chip ice cream and had a taste, then another, and then, by the
time the fashion television show had ended, the fruit was gone, the
container of dip was empty, half of the peanut butter had been eaten, and
there was one spoonful left of ice cream. So she ate it. And then she cried.
“Was this any way to live?” she asked herself.
RESOURCES
National Eating Disorders Association
http://www.nationaleatingdisorders.org/resource-links
Center for Eating Disorders http://www.center4ed.org/resources.asp
National Association of Anorexia Nervosa and Associated Disorders
http://www.anad.org/resources/
Academy for Eating Disorders http://www.aedweb.org/
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C H A P T E R
16 Substance-Related and Addictive
Disorders
Emily Raphael-Greenfield
KEY TERMS
Addiction
Co-occurring disorders
Cravings
Delirium tremens
Fetal alcohol syndrome
Intoxication disorder
Impaired control
Pharmacological criteria
Risky use
Social impairment
Substance abuse
Substance/medication-induced disorders Substance-related disorders
Substance use disorder
Tolerance
Withdrawal
Withdrawal disorder
Laura, an 80-year-old Caucasian woman, is being seen daily by an occupational
therapist (OT) in a subacute facility. She has broken her hip and successfully
undergone hip replacement surgery. Before her fall, she was living by herself in
a four-bedroom house where she and her husband raised their children. Her
husband passed away 10 years ago, and none of her three children live nearby.
The OT has observed that Laura keeps to herself in the subacute rehabilitation
facility and never has any visitors. The therapist encourages Laura to describe
her daily life prior to her accident and gently probes for details surrounding her
fall. Laura is hesitant to discuss how she used to spend her days and the fall, but
she liked the therapist and admitted to her that she was very lonely and was
having a hard time living by herself and taking care of her house. When asked if
she had any friends, she shook her head and said that she used to but not
anymore. She told the therapist that she had worked as an accountant for 30
years while also raising her children. She used to see her children and
grandchildren often, but she said, “My family is angry with me.” When asked
why, the client shook her head and said sadly, “I spoiled things.”The therapist
knew that the client was not depressed because she had been screened with a
depression scale, but she had never been screened for alcohol use, which is
linked with falls and other orthopedic injuries. The therapist decided to
administer the CAGE Questionnaire, which is a standardized screening tool for
alcohol use disorders that can be administered by any health care provider
(Silver, 2015). She asked Laura the following four questions:Have you ever
thought you should Cut down on your drinking? Although Laura was upset by
the question, she began to describe how she and her husband were social
drinkers and occasionally shared a bottle of wine with friends. Since his death,
she had begun to drink alone. She described herself as a late-onset drinker who
had lost her tolerance for alcohol. She had tried to stop her drinking but had
failed. (Yes.)Have people Annoyed you by criticizing your drinking? Laura
stated with great embarrassment that her children were critical of her drinking,
and for the last 3 years, they had refused to allow her to see her grandchildren
whom she adored. She was extremely annoyed with them. (Yes.)Have you ever
felt bad or Guilty about your drinking? Laura felt that she has really messed up
her life, especially by spoiling her relationship with her family. (Yes.)Have you
ever had a drink first thing in the morning to steady your nerves or to get rid of a
hangover (Eye opener)? Laura started to cry and said that the morning she fell
and broke her hip, she had started the day with a drink. (Yes.)The therapist
totaled her score, which was a four, which is considered clinically significant.
The OT reported her findings to the medical team, which included a psychologist
who considered a diagnosis of alcohol use disorder for Laura. Because Laura
was in a subacute rehab facility, she was in a controlled environment where
alcohol was prohibited, which was going to help her remain abstinent in the
short run. Her therapist also knew that building a trusting relationship with Laura
was going to help her patient face and overcome her substance use problem in
the long term. Another part of Laura’s plan consisted of collaborating with her to
design a discharge plan, which included not only continuing outpatient physical
rehabilitation but also setting her up in a day program that treated substance use
problems, locating self-help services such as Alcoholics Anonymous (AA), and
reinvolving her family in her life to help her recover from this late-onset alcohol
use disorder.
Substance-related disorders and addiction are widespread phenomena that affect
all sectors of a population and OT specialty areas on a national and global basis
(AOTA, 2014). Because OTs are likely to encounter clients with addiction in all
treatment settings, a background in signs and symptoms as well as familiarity
with medical interventions will enable our profession to make a unique
contribution to improving our clients’ occupational performance (Thompson,
2007).
DSM-5 changed the classification system of substance-related disorders
from that used in DSM-IV-TR. DSM-5 has retained the two main categories of
substance-related disorders—substance use disorders and substance-induced
disorders—but dropped the terminology of substance dependence and substance
abuse. Substance abuse usually is defined as the state that occurs when the use
of the substance leads to failure in carrying out a variety of occupational roles,
interpersonal and legal problems, and risky behaviors (APA, 2000). DSM-5’s
newer system of classification for all classes of substances is based on the
pattern of use and its associated behavioral and psychological factors. The three
types of disorder listed in the DSM-5 for all substance classes are Use disorders,
Intoxication disorders, and Withdrawal disorders (APA, 2013).
Clients are now described as having substance use problems, which are
further categorized into two phases: intoxication and withdrawal. Substance use
disorder refers to irreversible brain circuit changes in severe disorders and
repeated behavioral relapses and strong cravings in the presence of substance-
related stimuli (APA, 2013). Intoxication disorder refers to the short-term
symptoms associated with current use of the substance, including the
problematic behaviors and psychological changes. Withdrawal disorder
describes the symptoms that occur immediately and for a longer period after a
client who has been heavily using a substance stops ingesting. These withdrawal
symptoms also cause suffering and problematic functioning (APA, 2013).
There are 10 classes of drugs that are encompassed by the DSM-5
substance-related disorders: alcohol; caffeine; cannabis; hallucinogens;
inhalants; opioids; sedatives, hypnotics, and anxiolytics; stimulants; tobacco; and
other substances and nonsubstance (gambling). DSM-5 describes diagnostic
criteria and specifiers for each drug class. The classes are presented
alphabetically (see Table 16.1). All drugs that are taken in excess activate the
neurons in the brain’s reward system to release the chemical dopamine at higher
levels than do naturally occurring stimuli (NIDA, 2015b). This reaction is so
intense that normal activities are ignored. The drugs produce a pleasurable
feeling state, which is referred to as a “high.” Rather than stimulating the reward
system in the brain through adaptive behavior, drugs of abuse directly trigger the
reward circuits (APA, 2013). Gambling is included because it also activates the
brain’s reward system and produces similar behavioral patterns (Bonder, 2015).
When a person is diagnosed with a substance use disorder and other psychiatric
conditions, this is referred to as co-occurring disorders (CODs) (Moyers,
2011).
TABLE 16.1 Substance-Related Disorders
From American Psychiatric Association (APA). (2013). Substance-related and addictive disorders. In
Diagnostic and statistical manual of mental disorders (5th ed.). Author. doi:
10.1176/appi.books.9780890425596.249120
Substance use is defined as the consumption of the substance by any of the

following means: orally, smoking, inhaling, or intravenously. Substance use
produces long-term physiological, cognitive, and behavioral symptoms in
contrast with the immediate effects of intoxication. Clients are diagnosed with
substance use disorders when they exhibit disturbed patterns of behavior after
ingesting a substance. DSM-5 criteria for diagnosing substance use disorders are
organized into four categories: impaired control, social impairment, risky use,
and pharmacological criteria (APA, 2013).
Impaired control (APA, 2013) is defined as taking larger amounts than
intended over time, many unsuccessful attempts to reduce use and also a
persistent desire to decrease or discontinue, and one’s daily activities revolve
around obtaining the substance and/or recovering from its use. Cravings are the
desire for the drug that precludes thinking of anything else and are associated
with specific reward pathways in the brain and occur more likely in an
environmental context of previous use. Cravings are documented by asking the
client if there was a time when he or she could not think of anything else other
than getting the substance. Cravings are viewed as a signal of an impending
relapse (APA, 2013). Social impairment (APA, 2013) is defined as persistent
substance use associated with the inability to carry out key occupational roles,
serious social and interpersonal problems, and withdrawal from leisure and other
family activities. Risky use (APA, 2013) is defined as consuming the substances
even when it is physically hazardous and despite knowing that the substance use
is causing persistent physical, legal, and/or psychological difficulties.
Pharmacological criteria (APA, 2013) are defined as tolerance and withdrawal.
Tolerance is defined as requiring increased amounts of the substance in order to
get the desired effect or a significantly reduced effect when usual dosage is
consumed; tolerance is difficult to determine by history alone and is established
by laboratory tests. Withdrawal is defined as a syndrome that occurs when
blood or tissue concentrations decline in a person who has used heavily, which
makes him or her more likely to consume again to relieve symptoms. For most
classes of substances, a past history of withdrawal is associated with a more
severe clinical picture. When a person is experiencing both the symptoms of
tolerance and withdrawal, he or she is described as having an addiction.
Substance-induced disorders include intoxication, withdrawal, and other
substance/medication-induced mental disorders, including substance-induced
psychotic disorder, substance-induced depressive disorder, substance-induced
bipolar disorder, substance-induced anxiety disorder, substance-induced OCD
and related disorders, substance-induced sleep problems, substance-induced
sexual dysfunction, substance-induced delirium, and substance-induced
dementia (see Table 16.2). According to the DSM-5, these types of substance-
induced disorders are usually reversible, but severe, temporary, brief states and
related to the ingestion of a specific substance; conditions usually disappear
within 1 month or so of cessation of acute withdrawal, severe intoxication, or
use of the medication. All of the 10 classes of substances that produce substance
use disorders can produce the substance/medication-induced mental disorders,
but these disorders can also be caused by many other medications commonly
used in medical practice (APA, 2013) (see Table 16.3). Common characteristics
of these types of substance-/medication-induced disorders include appearance of
clinically significant symptoms of a relevant mental disorder that occur within 1
month of a substance intoxication or withdrawal or taking a medication,
capability of the substance/medication to produce a mental disorder, and
presence of significant problems in several areas of occupational functioning
(APA, 2013).
TABLE 16.2 Substance-Induced Disorders
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TABLE 16.3 Medication-Induced Disorders
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Intoxication disorders usually consist of changes in perception, wakefulness,

attention, thinking, judgment, psychomotor behavior, and interpersonal behavior.
The type of intoxication sought determines the route of administration. The most
intense intoxication is produced by rapid and efficient absorption into the
bloodstream intravenously, smoking, or intranasal “snorting.” These three
methods of administration are more likely to lead to an escalating pattern of
withdrawal. Short-acting substances, rather than longer acting ones, have a
higher potential for the development of withdrawal (APA, 2013).
Withdrawal disorders occur after a person who has used a substance for a
long period stops ingesting the substance. Withdrawal disorders are described by
specific behavioral, psychological, and physical symptoms that can last hours,
days, and weeks and can cause significant distress and occupational dysfunction.
When a substance such as heroin, which has prolonged, uncomfortable
withdrawal symptoms, is used, it may make treatment of the disorder more
challenging (Bonder, 2015).
Medications or substances can cause serious central nervous system (CNS)
side effects, such as dystonic reactions, confusion, and dizziness, but the side
effects disappear once the medication or substance is eliminated from the body.
The criteria for diagnosing substance/medication-induced mental disorders
involve gathering descriptions of symptoms, a history of substance/medication
use, a mental status exam, and lab findings (APA, 2013).
OTs need to be aware of the medications that can cause CNS side effects
because the cognitive, behavioral, and physical symptoms that they cause may
occur in any acute, rehabilitation, and community practice site (Bonder, 2015).
One example is medication-induced movement disorders, such as dystonia,
akathisia, and tardive dyskinesia. All of these movement disorders are caused by
the use of typical antipsychotic medications, and their appearance can further
stigmatize clients. The movement disorders are usually controlled by rebalancing
medication regimens. Dystonia is defined as prolonged muscle contractions.
Akathisia is defined as restlessness and fidgeting. Tardive dyskinesia is defined
as involuntary athetoid and choreiform movements of the tongue, face, and
limbs.
Etiology
There are five factors that help to explain the causes and origins of substance-
related disorders: biological and genetic, temperament, psychological
explanations, sociocultural factors, and age (Fig. 16.1). The neurological
pathways that are implicated in all substance use disorders are the mesolimbic
dopamine reward pathway and the prefrontal cortex (Bonder, 2015). The reward
pathway is changed by substance use, which causes unrestrained cravings
(Gutman, 2006). The reward response is changed so that “go” signals are
enhanced and “stop” signals are inhibited. The prefrontal cortex controls
decision making, which becomes impaired with substance use (Clay, Allen &
Parran, 2008) (see Fig. 16.2).
Figure 16.1 Intrinsic and extrinsic factors affecting etiology. (From Rundio, A.,
& Lorman, B.. (2015). Core curriculum of addictions nursing (3rd ed.).
Figure 16.2 The anatomical brain. (From Herron, A., & Brennan, T. K.. (2015).
The ASAM essentials of addiction medicine (2nd ed.). Philadelphia, PA:
The genetic factors underlying the etiology of alcohol disorders are more
thoroughly understood and researched than any of the other substances (Sells,
Stoffel & Plach, 2011). In early 20th century America, the cause of alcohol
disorders was considered to be a moral failing. It is now considered to be a
disease (Bonder, 2015). There is no single gene but rather a variety of genes that
are thought to make a person vulnerable to developing alcoholism (NIAAA,
2005a). Based on biological relative and adoptive parent studies with twins, it is
thought to be a combination of genetic predisposition triggered by environmental
issues. The onset of familial alcoholism is earlier, and its prognosis is poorer
than patterns of nonfamilial alcoholism. Women diagnosed with alcohol use
disorders have a later onset, drink smaller amounts, and progress more rapidly
with the disease (Bonder, 2015). The genetic factors linked to drug disorders are
more complex, but genes are considered important in explaining the level of
risky behavior associated with these disorders (Sells et al., 2011).
The development of substance use disorders in adulthood is associated with
childhood and adolescent “difficult” temperaments (NIAAA, 2005a). Youth with
self-regulation problems, which translate into behavioral problems in the
classroom and the community, may self-medicate with alcohol, drugs, and illicit
use of medications. Research on adults with sensory modulation disorders
demonstrated that a majority used CNS depressant substances, including alcohol,
sedatives, hypnotics, and anxiolytics, while 15% to 18% used stimulants or a
combination of both (Quadling, Maree, Mountjoy, Bosch, & Kotkin, 1999).
Psychological theories that explain the etiology of substance use include
theories of behaviorism and cognitive behavior (NIAAA, 2005a). Classical
conditioning explains how drinking and drug use become linked to person,
location, and time of day. Operant conditioning explains how substance use
becomes paired with stressors as triggers and perceived calm after use. These
theories explain how the user discounts negative consequences because they
occur later and are not connected with the positive effects of using. There is also
research with children that reveals the power of modeling where young users
learn their alcohol use behaviors from observing and imitating what others are
doing in real life and in the media (NIAAA, 2005a).
Sociocultural influences on the etiology of alcohol and drug use include the
relationship between alcohol and drug use and family, peer, and social
relationships. It is not clear if this relationship is causal or just associative. The
influence of family has been researched, for example, if a child grows up in a
family where substance use adversely impacts family functioning, the child is at
greater risk for developing substance use problems. There is a link between
adolescents who have early adverse experiences such as parental divorce,
parental drinking, a parental psychiatric disorder, or sexual abuse and
adolescents trying to cope by using substances (Monti et al., 2005). Economic
and sociological explanations rather than neurochemical explanations have also
been posited for the high rates of usage among inner-city minority populations
by neuroscientist Hart (2013) who has studied how the availability of cheap
illicit substances and absence of healthier alternative occupations and racist law
enforcement in low-resource neighborhoods explain addiction patterns rather
than the hard wiring of brains.
Researchers have found protective factors such as supportive extended
family members, a child’s calm temperament, or positive family rituals can
interrupt the cycle (NIAAA, 2005a). The influence of peers is profound during
mid to late adolescence when it is most likely that alcohol and drugs are tried for
the first time, due to greater independence, need for peer approval, availability of
substances, and greater tolerance, for example, on college campuses.
Adolescents report that they use more substances because they are bored and
have positive associations with intoxication. They also believe that substances
foster increased sociability and reduce anxiety (NIAAA, 2005a). There is a
strong link in women between histories of sexual and physical abuse and alcohol
use disorders (Maniglio, 2011). If women use alcohol excessively during
pregnancy, there is a greater risk for fetal alcohol syndrome, a condition in
which the infant exhibits CNS damage and retardation (Jones & Streissguth,
2010). Alcohol is often used to self-medicate symptoms of other psychiatric
disorders, which results in dual diagnosis or co-occurring disorders.
There are two age groups that deserve special attention in terms of etiology:
college age students and older adults. Excessive drinking on campuses is now
reported to be at epidemic proportions, particularly among males, ages 18 to 25,
even though drinking among college-bound students is lower than that among
their non–college-bound peers because of the pressure to achieve. College
campuses have traditionally been considered safe places to drink and use
substances, but there are numerous negative consequences associated with this
permissive environment, including serious injuries, death, sexual harassment and
assault, problems for roommates and friends, destruction of property, academic
problems, anxiety, and depression (National Center on Addiction and Substance
Abuse, 2007). Because of their use of alcohol in combination with prescription
and over-the-counter medications, older adults also present with particular
difficulties. While the majority of older adults do not drink or use drugs at all or
only in moderation, research has shown that in adults over 60, 15% of men and
12% of women drank more than the recommended guidelines (Adams, Barry &
Fleming, 1996).
Prevalence is the percentage of persons within a particular population who have
a specific disease or illness at a given time. Incidence is the percentage or rate of
new cases within a population during a particular time period (Center for
Substance Abuse Treatment, 2007). The prevalence of Americans aged 12 or
older, who reported being current alcohol drinkers, was 52.2%, which was
similar to the rate in 2012 (52.1%). Nearly one-quarter or 22.9% of persons aged
12 or older in 2013 were described as binge alcohol users in the 30 days prior to
the survey. Binge drinking is defined as five or more drinks on the same
occasion. The rate in 2013 was similar in 2012 (23.0%). Heavy drinking, which
is defined as five or more drinks on each of 5 days, was reported by 6.3% of the
population aged 12 or older, which was similar to the rate of heavy drinking in
2012 or 6.5% (SAMHSA, 2014).
An estimated 24.6 million Americans aged 12 or older in 2013 used an illicit
drug during the month prior to the survey, which represents 9.4% of the
population aged 12 or older, according to the National Survey on Drug Use and
Health (NSDUH) (Fig.16.3). Illicit drugs include marijuana, cocaine, heroin,
hallucinogens, inhalants, psychotherapeutics, or the nonmedical use of
prescription medications (SAMHSA, 2014). The most commonly used illicit
drug was marijuana, which was used by 19.8 million users over the last month,
representing 80.6% of illicit drug users (Fig.16.4).
Figure 16.3 Percentage using any illicit drug. (From Ries, R. K., Fiellin, D. A.,
Miller, S. C., & Saitz, R. (2014). The ASAM principles of addiction medicine
(5th ed.). Philadelphia, PA: Lippincott Williams & Wilkins.)
Figure 16.4 Prevalence of different drugs. (From Ries, R. K., Fiellin, D. A.,
Miller, S. C., & Saitz, R. (2014). The ASAM principles of addiction medicine
Young adults, aged 18 to 25, used illicit drugs at the highest rate (21.5%) in
2013, after that youths aged 12 to 17 (8.8%), and then adults aged 26 or older
(7.3%). This represents a decrease from 2012 in the number and percentage of
current illicit drug users among youths aged 12 to 17 from 2.4 million (9.5%) in
2012 to 2.2 million (8.8%) in 2013 (SAMHSA, 2014; National Center on
Addiction and Substance Abuse, 2011).
The prevalence of alcohol and illicit drug use is higher among males than
females. In 2013, 57.1% of males aged 12 or older were current drinkers
compared to females (47.5%). Among youths aged 12 to 17, however, the
percentage of males and females who were current drinkers was 11.2% and
11.9%, respectively. The incidence for both male and female youths was lower
than those of 2012 (12.6% and 13.2%, respectively). In the young adult group,
ages 18 to 25, 62.3% of males and 56.9% of females were reported to be current
drinkers in 2013. In this same age group in 2013, 44.4% of males and 31.4% of
females reported binge drinking in 2013. The rate of binge drinking for females
aged 18 to 25 decreased from the incidence reported in 2012 (33.2%), while
binge drinking among males in this age group remained the same. Among
persons aged 26 or older, an estimated 62.2% of males and 50.1% of females
reported current drinking in 2013, but in this age group, the rate of binge
drinking for males was approximately twice the rate for females (30.7% vs.
14.7%) (SAMHSA, 2014).
The prevalence of alcohol use among pregnant women aged 15 to 44 in
2012–2013 included an annual average of 9.4% reporting current alcohol use,
2.3% reporting binge drinking, and 0.4% reporting heavy drinking. Alcohol use
during the second and third trimesters in 2012–2013 was reportedly lower than
during the first trimester (5.0% and 4.4% vs. 19.0%) (SAMHSA, 2014).
Among persons aged 12 or older in 2013, the prevalence of current illicit
drug use was 3.1% in Asians, 8.8% in Hispanics, 9.5% in Whites, 10.5% in
Blacks, 12.3% in American Indians or Alaska Natives, 14.0% in Native
Hawaiians or other Pacific Islanders, and 17.4% in persons reporting two or
more races. Between 2002 and 2013, the incidence of current illicit drug use
increased from 8.5% to 9.5% for whites, and among blacks, the rate increased
from 2004 (8.7%) to 2013 (10.5%) (SAMHSA, 2014).
Prevalence of substance use is also explained by biological, genetic, and
socioeconomic factors. Epidemiological research has demonstrated differences
in metabolism and absorption rates of alcohol in different ethnic groups (Wall,
Luczak, & Hiller-Sturmhofel, 2016). For example, Asians have the lowest risk of
alcohol use disorders, while Whites experience the highest risk. Hispanics have
higher rates and Asians have lower rates of heavy drinking patterns. Hispanics
and Blacks compared to Whites and Asians are more likely to experience health
and social problems from drinking. Access to health care, preventive services,
and alternatives to drug use are less available and underfunded for lower
socioeconomic groups in this society (Hart, 2013). Hart also emphasizes that
only about 10% to 15% of substance users, whether they use alcohol,
prescription medications, or illicit drugs, become addicted and 75% of substance
users do not develop these serious dysfunctional patterns. Underserved
minorities face more discrimination, stronger legal consequences, and increased
incarceration when they use drugs recreationally than do the Caucasian youth
population. There are also fewer resources for healthy living and treatment in
inner cities and rural areas, which makes advanced states of substance-related
disorders harder to treat in these areas (Sells et al., 2011).
The prevalence of adult US citizens with a COD is approximately five
million (Center for Substance Abuse Treatment, 2007). Studies of clients in
mental health settings found that 20% to 50% had a lifetime co-occurring
substance use disorder, while other studies of individuals in substance abuse
treatment found that 73% had co-occurring mental health disorders during their
lifetimes. The majority of these mental health disorders are described as
depressive, bipolar, posttraumatic stress, and personality disorders. Clients
diagnosed with more than one mental health disorder are not uncommon (Center
for Substance Abuse Treatment, 2007).
Signs and Symptoms
Alcohol
Alcohol is a CNS depressant that causes a brief period of intoxication followed
by slowing of cardiac and respiratory responses over time. Overdose and death
can occur from a single episode when there is a very high level of intake, for
example, during fraternity hazing (Bonder, 2015). The signs and symptoms are
organized into alcohol use disorders, alcohol intoxication disorders, and alcohol
withdrawal disorders within DSM-5 (see Table 16.4). Alcohol use disorders have
the following signs and symptoms: within a 12-month period, the use of alcohol
leads to significant psychological distress and problems in functioning, including
increased tolerance and cravings; a great deal of time spent acquiring, using, and
recovering from alcohol consumption; unsuccessful efforts to decrease or stop its
use; inability to carry out important occupational roles; serious problems with
interpersonal functioning and relationships; leisure and recreational activities not
developed or given up; and continued use despite its physical and legal risks and
its negative effect on the body and mind (APA, 2013). The signs and symptoms
for alcohol intoxication include recent consumption of alcohol followed by
inappropriate sexual or aggressive behavior, mood lability, impaired judgment,
slurred speech, incoordination, unsteady gait, nystagmus or involuntary eye
movement, impaired attention or memory, or stupor or coma. The signs and
symptoms of alcohol withdrawal include stopping consumption of alcohol after a
prolonged and heavy use, and within several hours or days of cessation, a person
experiences autonomic hyperactivity or sweating and increased pulse rate; hand
tremors; insomnia; nausea or vomiting; transient visual, tactile, or auditory
hallucinations; psychomotor agitation; anxiety; or generalized tonic-clonic
seizures (loss of consciousness, stiffness of muscles, and jerking movements)
(APA, 2013). Mild degrees of withdrawal symptoms are common and are
usually managed in the community without medical interventions. In
approximately 5% of people with chronic alcohol use disorders who terminate
their drinking, the condition of delirium tremens (DTs) develops, which
consists of seizures, hallucinations, and severe tremors, which can lead to death.
Alcohol withdrawal can produce life-threatening symptoms such as DTs, and the
protocol is currently to admit these patients with moderate and severe
withdrawal symptoms to the hospital (Elliott, Geyer, Lionetti & Doty, 2012).
TABLE 16.4 Substance Use Disorders
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Alcohol use has been analyzed by the amount of substances consumed by males
and females (NIAAA, 2010) (see Table 16.5). The amount of alcohol consumed
has also been standardized by NIAAA and defined as one unit or standard drink.
One standard drink consists of 0.5 to 0.6 fluid ounces or 1.2 tablespoons of
absolute ethanol (see Table 16.6).
TABLE 16.5 Drinking Types by Gender
From National Institute on Alcohol Abuse and Alcoholism (NIAAA). (2010). Rethinking drinking: Alcohol
and your health. Bethesda, MD: Author. Retrieved from http://rethinkingdrinking.niaaa.nih.gov/ NIH
Publication No. 13–3770 on June 27, 2015
TABLE 16.6 A Standard Drink
Adapted from National Institute on Alcohol Abuse and Alcoholism. (n.d.). “What is a standard drink?”
Retrieved from http://pubs.niaaa.nih.gov/publications/Practitioner/pocketguide/pocket_guide2.htm on
August 21, 2015.
The vast and changing numbers of abused drugs make its classification more
complex than that of alcohol use. Rather than being categorized by amounts and
patterns of consumption, drug use is categorized by classes of drugs and whether
they are prescription or nonprescription drugs (Sells et al., 2011). Classifications
of drugs also change as new drugs are created and their trends of usage shift. In
the late 1970s, the United States experienced a peak in drug use that was
followed by a national decline in the 1980s. After an increase in the 1990s, the
use has been relatively stable for the last two decades. While Ecstasy, considered
a club drug, reached its popularity in the 1990s, it was surpassed by oxycodone,
a painkiller, in the last decade (SAMHSA, 2014). Figure 16.5 shows a
comparison of drug use worldwide.
Figure 16.5 Comparison of drug use worldwide. (From Ries, R. K., Fiellin, D.
A., Miller, S. C., & Saitz, R. (2014). The ASAM principles of addiction medicine
Caffeine
Caffeine is the most commonly consumed mind-altering substance globally. OTs
are rarely involved in the treatment of this disorder, except to assist clients to
find alternative activities that produce desired positive outcomes. For example,
an OT may help a client learn how to socialize without drinking coffee if a client
is experiencing negative side effects. Although research is beginning to
document its health benefits, there are still people who experience adverse
reactions and cannot stop using it, as with other addictive substances (Higdon &
Frei, 2006; Juliano & Roland, 2004). There is no caffeine use disorder because
that would include an inordinate number of people. The signs and symptoms for
caffeine intoxication include restlessness, nervousness, excitement, insomnia,
flushed face, diuresis, gastrointestinal disturbance, muscle twitching,
disorganized thinking or speech, cardiac rhythm changes, excessive energy, and
psychomotor retardation (APA, 2013). The symptoms of caffeine withdrawal
must take place within 24 hours of the last caffeine intake and include headache,
difficulty concentrating, fatigue, depressed mood, and flu-like symptoms. The
signs and symptoms of caffeine intoxication disorder and caffeine withdrawal
disorder are summarized in Table 16.4.
Cannabis
The most commonly used illicit drug, cannabis (Fig. 16.6), also called weed, pot,
ganja, and a large number of other slang terms, is made from the shredded leaves
and flowers of Cannabis sativa, which is a hemp plant (SAMHSA, 2014). In
2013, marijuana was used by 81% of current illicit drug users and the only drug
used by 64.7% of them. A number of states are legalizing marijuana for
medicinal and recreational purposes, which means that its use is gaining greater
acceptance in American society. Its therapeutic benefits, as well as its adverse
health effects, are still being researched. It is smoked in the form of rolled
cigarette joints, water pipes, and marijuana cigars, brewed in tea, mixed into
foods when dispensed for medicinal purposes, and served as concentrated resins.
The mind-altering or psychoactive chemical in cannabis is delta-9-
tetrahydrocannabinol (THC). THC is similar to cannabinoid neurotransmitters
that occur in the body and activate the following areas of the brain: hippocampus
and orbitofrontal cortex, which are responsible for forming new memories and
learning complex new tasks; the cerebellum and the basal ganglia, which
regulate balance, posture, coordination, and reaction time; and the body’s
dopamine reward system, which contributes to the pleasurable “high” sought by
recreational users (NIDA, 2015b).
Figure 16.6 Picture of marijuana plant. (From Ries, R. K., Fiellin, D. A., Miller,
S. C., & Saitz, R. (2014). The ASAM principles of addiction medicine (5th ed.).
Pleasant experiences with cannabis are not universal. Instead of euphoria and
relaxation, some users experience anxiety, fear, distrust, panic, hallucinations,
delusions, and a loss of a sense of personal identity (NIDA, 2015b). These
adverse reactions often occur when an excessive amount is taken, the user is
inexperienced, or the potency is high. Over the past three decades, the potency of
marijuana is increasing, rising from 3.7% in the 1990s to 9.6% in 2013. These
levels have been established from THC content in confiscated cannabis samples
(NIDA, 2015b). There is some research that demonstrates a link between
cannabis use and psychotic disorders such as schizophrenia if there is a
preexisting genetic vulnerability. There are also studies that show marijuana use
can exacerbate symptoms in people who have already been diagnosed with
schizophrenia (Radhakrishnan, Wilkinson, & D’Souza, 2014). Because cannabis
use impairs judgment, motor coordination, and reaction time, research has
established a connection between its use and problems with driving accidents,
including fatal ones (NIDA, 2015b). There is also evidence that marijuana can
be addictive; about 9% of people who use it cannot stop using it even though it
interferes with many occupational roles and areas. There is also growing
evidence that marijuana use during development can cause permanent adverse
changes in the brain, which has implications for its use during pregnancy on the
developing fetus and during adolescence when there is a potential loss of IQ on
intelligence tests and the development of learning problems (NIDA, 2015b). The
physical effects of cannabis use include breathing problems and increased heart
rate (NIDA, 2015a). The signs and symptoms of cannabis use disorder, cannabis
intoxication disorder, and cannabis withdrawal disorder are summarized in Table
16.4.
Hallucinogens
Hallucinogens are taken by the user to escape. These drugs are found in plants
and mushroom and have been used in religious ceremonies for centuries. Their
chemical structure is similar to neurotransmitters such as acetylcholine and
serotonin (NIDA, 2014b). Some of the general effects of this class include
hallucinations, depersonalization/derealization, distortions of time and
perception, mood swings, elevated body temperatures, and seizures. Lysergic
acid diethylamide (LSD) is one of the most powerful hallucinogens and was
discovered in 1938. LSD is not considered to be an addictive drug because
although it produces tolerance, it usually does not result in compulsive drug-
seeking behavior. Unlike most other drugs and because there is so much
variation in their composition, hallucinogens have an unpredictable and therefore
dangerous effect on individuals. The fact that this class of drugs is produced in
laboratories and is readily available contributes to its variability. Although PCP
developed a bad reputation as a street drug in the 1960s due to its adverse
reactions during intoxication (violence and suicidality), people still abuse it
because it causes intense feelings of strength, power, invulnerability, and
numbing of the mind (NIDA, 2014b).
More recently, the hallucinogen or synthetic cannabinoid, K2, which is
marketed as fake marijuana, potpourri, or herbal high, has been gaining in
popularity because users believe it is natural and safer than other drugs. It is,
however, not natural and has been created in laboratories and never meant for
human ingestion. It is sold cheaply in small colorful packages with comical
characters, meant to appeal to youth, and is easily available in tobacco and
smoke shops. K2 contains a mixture of herbs that have been laced with
psychoactive chemicals. While the Federal Government has classified some of
the synthetic cannabinoids as illegal to sell, laboratories quickly change the
formula to avoid these banned substances and sell them as potpourri, labeled as
not meant for consumption. Standard urine toxicology tests cannot detect K2. It
is thought to stay in the body for prolonged periods of time and to have a similar
chemical structure as carcinogens, which are cancer-causing substances (NIDA,
2012a).
The risk of overdose with K2 is high because the amount of psychoactive
agents varies within each batch. The length of its effect varies from 30 minutes
to 2 hours. It causes the following signs and symptoms, including euphoria and
relaxation, perceptual and sensory distortions, hallucinations, poor
concentration, drowsiness, confusion and agitation, difficulty forming thoughts,
loss of motivation, no concern for the future, emotional lability, aggression,
depression, anxiety, paranoia, headaches, tachycardia, hypertension, heart
attacks, nausea and vomiting, electrolyte abnormalities, seizures, and syncope
(fainting) (NIDA, 2012a). Withdrawal can take 6 hours or longer and may
require hospitalization. The signs and symptoms of hallucinogen use disorder,
hallucinogen intoxication disorder, and hallucinogen withdrawal disorder are
summarized in Table 16.4.
Hallucinogens have the potential for long-term cognitive damage. Although
most people experiment with this class of drugs and are deterred from further
use by the negative consequences after a few times, other people use them
heavily for a longer period of time and develop hallucinogen persisting
perception disorder. Diagnostic criteria include persistent perceptual changes,
sometimes described as flashbacks, memory loss, depression, loss of speech,
thinking problems, weight loss, and liver malfunction (APA, 2013).
Inhalants
Inhalants are chemical vapors or gases that when inhaled cause psychoactive
effects. These include a broad range of volatile solvents and gas products (e.g.,
airplane glue, paint thinner, nail polish remover), aerosols (e.g., hairspray),
anesthetics (e.g., nitrous oxide), and nitrites (e.g., poppers, room odorizers).
Inhalants are abused by inhaling either through the nose or through the open
mouth (SAMSHA, 2003). Because of their easy availability due to the difficulty
of restriction of use of legal, inexpensive, everyday substances, inhalants have
become popular with teens. Because the inhalants are so readily available, many
young people do not consider them harmful or understand their negative
consequences. While teens more frequently inhale glue and lighter fluid, adults
inhale nitrous oxide and room odorizers. Lifetime prevalence by gender and race
is higher for Caucasian males (8.9%) and females (9.8%) than for Black males
(5%) and females (6.8%) (SAMSHA, 2003). People who abuse inhalants seek
treatment in emergency rooms rather than treatment in hospitals. Signs of
inhalant use include redness or sores around the mouth or nose, chemical breath
odor, dizzy appearance, excess salivation, and unexplainable collection of
inhalable substances. Even with first use, lethal and debilitating effects can occur
including sudden death, cardiac effects, coma, seizures, brain damage, and lead
poisoning (SAMSHA, 2003). With longer term use, there is evidence of
neurological damage causing decreased concentration, memory impairment, and
learning difficulties, but the research has not established if these changes are
transitory or long term (SAMSHA, 2003). The signs and symptoms of inhalant
use disorder, inhalant intoxication disorder, and inhalant withdrawal disorder are
summarized in Table 16.4.
Opioids
Opioids include narcotics, which were developed to block or reduce pain and
produce drowsiness for legitimate medical procedures. Users of nonprescribed
opioids seek withdrawal and relaxation, and they typically have a low tolerance
for pain, anxiety, and stimulus thresholds. There are illicit opioids, such as
heroin, and other substances that are prescription drugs prescribed for cough
suppressants or for relief of pain or sensation (analgesics and anesthetics) that
are used for nonmedical purposes (Bonder, 2015). Most people begin with using
prescriptions for controlling pain but change to nonprescription use or heroin
because of lower costs and lack of availability of prescribed medication (Rabin,
2014). Although heroin use is less frequent than that of the prescription
medications, it has extremely serious health and addictive consequences. Heroin
is an opioid made from morphine extracted from the poppy plant. Heroin can be
consumed by injection, snorting or sniffing, or smoked. These three methods
rapidly deliver the drug to the brain, which results in changes in the brain and
addiction whereby uncontrollable cravings occur heedless of consequences. The
opioid binds with opioid receptors in the brain and body, which are involved in
the perception of pain and reward, as well as in the brainstem, which is
responsible for controlling blood pressure, arousal, and respiration (NIDA,
2014d). Overdose with heroin often results from the suppression of breathing,
which results in hypoxia in the brain (loss of oxygen) that can have serious
psychological and neurological effects. Research has demonstrated destruction
of the brain’s white matter after heroin use, which may impair decision making,
emotional regulation, and ability to manage stress (NIDA, 2014a, 2014d). There
are a number of serious health problems caused by heroin use, including fatal
overdose, pneumonia, irreversible damage to vital organs (lungs, liver, kidney,
brain), spontaneous abortion, HIV, hepatitis, collapsed veins, heart lining and
valve infections, abscesses, constipation, stomach cramps, and liver or kidney
disease (NIDA, 2014d). The signs and symptoms of opioid use disorder, opioid
intoxication disorder, and opioid withdrawal disorder are summarized in Table
16.4.
CNS Depressants
Sedatives, hypnotics, and anxiolytics are drugs that decrease activity in the brain
and are therefore termed CNS depressants (NIDA, 2014e). CNS depressants are
used as medications to treat anxiety and sleep disorders. These medications
include benzodiazepines (Valium, Xanax, Halcion, Restoril, and ProSom), which
are used for short- but not long-term treatment of anxiety or sleep because of
their risk for developing tolerance and addiction. This class also includes
nonbenzodiazepine sleep medications (Ambien, Lunesta, and Sonata), which are
thought to have fewer side effects and less risk of developing addiction than do
the benzodiazepines. The last class of CNS depressants is the barbiturates
(Mebaral, Luminal Sodium, and Nembutal), which are used in surgery, have a
higher risk of overdose, and therefore are not used for sleep or anxiety problems
(NIDA, 2014e). The CNS depressants increase the neurotransmitter gamma-
aminobutyric acid (GABA) neural activity, which has an inhibitory effect on the
brain, resulting in a drowsy or a calming effect.
There are two patterns of developing substance use disorders with this class
of drugs. First, the drugs are prescribed for a specific purpose and the person
develops tolerance and cravings over time, or second, the person obtains the
drugs illegally and they are not used in a prescribed fashion (Bonder, 2015).
When a person stops taking CNS depressants, the body can experience a
rebound effect, including seizures. While withdrawal from benzodiazepines is
not life threatening, it can be with barbiturates. Stopping CNS depressants and
combining CNS depressants with other medications should be closely monitored
(NIDA, 2014e). The signs and symptoms of sedatives, hypnotics, and anxiolytics
use disorder; sedatives, hypnotics, and anxiolytics intoxication disorder; and
sedatives, hypnotics, and anxiolytics withdrawal disorder are summarized in
Table 16.4.
Stimulants
Stimulants involved in substance use disorders consist of amphetamines and
cocaine (NIDA, 2013). Amphetamines can be prescribed for the treatment of
ADHD; methamphetamine and cocaine are never prescribed and always used
illicitly (Bonder, 2015). Cocaine is an addictive stimulant derived from the coca
plant. In a powdered form, it is either inhaled through the nose (snorted) or
mixed with water and injected. Crack is the crystallized form of cocaine that is
heated to form vapors that are smoked. Injecting or smoking cocaine produces a
more intense, but shorter-lasting, sensation than does snorting. To extend the
length of the high, people often binge, taking bigger doses over a short period of
time (NIDA, 2013). Cocaine increases the level of dopamine in the synapses in
the brain, which amplifies its effects, causing the “high.” The other bodily
systems affected by cocaine include blood vessels, body temperature,
gastrointestinal problems, strokes, heart attacks, death, and risk for contracting
HIV through risky sexual behavior. Snorting leads to loss of a sense of smell,
nosebleeds, swallowing problems, and runny nose. Injecting cocaine can lead to
increased risk for contracting HIV, hepatitis C, and other blood-borne illnesses.
Cocaine binging can lead to severe paranoia. When combined with other drugs
or alcohol (polydrug use), cocaine poses a high risk for a serious overdose
(NIDA, 2013). The signs and symptoms of stimulant use disorder, stimulant
intoxication disorder, and stimulant withdrawal disorder are summarized in
Table 16.4.
Tobacco
Tobacco is a stimulant and causes one in every five deaths in the United States,
serious illnesses attributable to smoking for 16 million Americans, and the
deaths of 41,000 nonsmokers from secondhand smoke each year (NIDA, 2014c).
Nicotine is the stimulant, which is smoked, chewed, or inhaled. Nicotine
stimulates the adrenal gland, which releases the hormone epinephrine. This
stimulates the CNS, increasing respiration, blood pressure, and heart rate.
Nicotine also stimulates the production of dopamine. Cigarette smoking, which
is a mixture of many chemicals that are carcinogenic, causes 90% of lung cancer
cases in the United States (NIDA, 2014c). Other medical complications include
oral cancers, emphysema, heart disease, leukemia, cataracts, and pneumonia. On
average, people who are smokers shorten their lifespan by 10 years. Smoking by
pregnant women and maternal smoking is associated with increased risks of
miscarriage, stillborn, premature infants, and learning and behavioral problems
in older children (NIDA, 2014c). If a person quits smoking, there are immediate
positive health consequences, including reduced risk of heart disease, cancer,
and stroke. The signs and symptoms of tobacco use disorder and tobacco
withdrawal disorder are summarized in Table 16.4. There is no category of
tobacco intoxication disorder (APA, 2013).
Gambling
Gambling, a non–substance-related disorder, shares many of the same criteria as
do substance-related disorders even though it does not involve ingestion of a
substance. The signs and symptoms include impulsivity, unsuccessful attempts
to cut down, preoccupation with spending time gambling, a return to gambling
even after significant losses, attempts to conceal a gambling habit from others,
negative impacts on relationships and job functioning, and persistent
participation in gambling despite financial risks (APA, 2013). The signs and
symptoms of gambling use disorder are summarized in Table 16.4.
Alcohol
Although it is thought to be an intractable condition, only a small percentage of
longtime chronic users will experience serious psychological and physical
problems including liver damage, cognitive impairment, peripheral neuropathy,
cardiomyopathy, stroke, sleep disorders, pancreatitis, cancer, injuries, drowning,
homicide, suicide, fatal falls and/or burns, domestic violence, sexual assaults,
motor vehicle crashes, depression, homelessness, unemployment, social isolation
and loss of contact with family, and incarceration. Those who use both alcohol
and drugs have a poorer outcome (Bonder, 2015; NIAAA, 2010). Unsafe use of
alcohol and drugs is associated with increased levels of violence and conflict,
and the presence of conflict increases the risky consumption of alcohol and
drugs (World Health Organization, 2008).
Most people with this disorder have a more auspicious prognosis and
improve (see Fig. 16.7). They may benefit from four types of evidence-based
treatments, including brief interventions, cognitive-behavioral therapy,
motivation interviewing, and 12-step programs (Stoffel & Moyers, 2004).
Prognostic factors include cultural attitudes toward drinking, availability and
price of alcohol, stress levels and impaired ways of coping with stress, heavy
peer pressure regarding substance use, and cognitive distortions, which
exaggerate the positive effects of alcohol (APA, 2013; see Fig. 16.8).
Figure 16.7 Managing addiction. (From Lowinson, J. H., Ruiz, P., Millman, R.
B., & Langrod, J. G. (2004). Substance abuse. Philadelphia, PA: Lippincott
Figure 16.8 Alcohol-related problems and prevention. (From Ries, R. K.,
Fiellin, D. A., Miller, S. C., & Saitz, R. (2014). The ASAM principles of
addiction medicine (5th ed.). Philadelphia, PA: Lippincott Williams & Wilkins.)
In adolescents, alcohol use disorder is often comorbid with conduct disorder.

Ninety percent of individuals develop alcohol use disorder before the age of 40.
The 10% of older adults who become chronic users develop more severe
intoxication with lower levels of consumption and develop other medical
complications (APA, 2013; Adams et al., 1996). Fetal alcohol spectrum
disorders (FASDs) are the physical, mental, behavioral, and learning disorders
that occur in an individual whose mother consumed alcohol during pregnancy. It
occurs in approximately 1% of the live birth population in the United States
(SAMSHA, 2007). Alcohol damages the developing fetus more than do other
substances that are used illicitly. FASDs are the most preventable cause of
developmental disabilities in children. Most individuals with FASD do not have
the facial features associated with this illness (short palpebral fissures, indistinct
philtrum, and thin upper lip). The extent of the disorder depends on when and
how much alcohol the mother consumed during the pregnancy. The behavioral
outcomes can include poor judgment and impulsivity, depression, and substance
and alcohol abuse (SAMSHA, 2007).
Caffeine
Because the half-life of caffeine is approximately 4 to 6 hours, the symptoms of
caffeine intoxication and withdrawal are short-lived and do not present with
long-term consequences. There are several exceptions to this benign description
of its course: if a person consumes more than 5 grams of caffeine at one time, he
or she will require immediate medical attention; older adults grow more
sensitive to the intense effects of caffeine and complain about increased
sleeplessness and restlessness; children and adolescents with low body weight
may be at increased risk for caffeine intoxication, especially if they are
consuming caffeine drinks, such as soft drinks and energy drinks (APA, 2013).
Cannabis
The development of cannabis use disorder most commonly occurs during
adolescence and early adulthood. Early onset of use of cannabis before the age
of 15 is considered a serious risk factor for future externalizing and internalizing
mental health disorders, including depression and conduct disorders (APA,
2013). In adolescence, the onset of the disorder is usually gradual. Cannabis,
along with tobacco and alcohol, are generally the first substances tried by
adolescents with cannabis used more often than alcohol because its behavioral
and cognitive consequences are considered less harmful. This may explain the
rapid transition from cannabis use to cannabis use disorder. While milder cases
of cannabis use disorder among teenagers arouse disapproval by peers, parents,
and school staff, severe cases result in more solitary and daily consumption that
is hidden from others leading to mood, energy level, and appetite changes and
serious impairment in school functioning, including drop in grades, truancy, loss
of interest in previous activities, and deficits in prosocial behaviors. Adults
diagnosed with cannabis use disorder involve patterns of daily consumption,
medical and psychosocial problems, and unsuccessful attempts to stop using
(Volkow et al., 2014; APA, 2013; Cousijn et al., 2012; D’Souza, Sewell, &
Ranganathan, 2009).
Hallucinogens
When phencyclidine (PCP) is the primary drug used among those admitted to
substance use treatment settings, the patients are younger, have lower
educational levels, and tend to be from the two coastal regions of the United
States (APA, 2013). The continual use of PCP is associated with high risk of
injuries from fights, falls, and other accidents as well as suicide; psychosis;
intense rage; deficits in memory, language, and cognition; neurological and
cardiovascular toxicities; intracranial hemorrhage; and, rarely, cardiac arrest
(APA, 2013). Early-onset Ecstasy users are more likely to be polydrug users than
those who begin using this hallucinogen later (APA, 2013). With other
hallucinogens, the course is usually associated with experimentation, limited use,
and high rates of recovery (APA, 2013).
Inhalants
Inhalant use generally declines after adolescence and diminishes in early
adulthood (APA, 2013). While 10% of American children 13 to 17 years old
report the use of inhalants one time, <0.5% of the same age group progress to
inhalant use disorder and exhibit multiple mental health problems, including
personality disorders and suicidal ideation and attempts (APA, 2013).
Opioids
Most problems associated with opioid use disorder are reported in late
adolescence and early adulthood. The typical course of use of opioids persists
for many years, punctuated by periods of remission. Successful treatment and
abstinence is often followed by relapse. While 20% to 30% of opioid users
achieve long-term abstinence, the mortality rates for long-term users may be as
high as 2% per year (see Fig.16.9). Increasing age is associated with decreased
prevalence due to early mortality rates and a diminution of symptoms after age
40. Ninety percent of Vietnam veterans were successfully treated for opioid use
disorder after deployment, but these veterans also developed alcohol use and
amphetamine use disorders and suicidality (APA, 2013).
Figure 16.9 Evidence of improvement with treatment. (From Ries, R. K.,

Fiellin, D. A., Miller, S. C., & Saitz, R. (2014). The ASAM principles of
addiction medicine (5th ed.). Philadelphia, PA: Lippincott Williams & Wilkins.)
CNS Depressants
Use of sedatives, hypnotics, and anxiolytics generally begins in adolescence and
early adulthood when some individuals use these types of substances to achieve
a “high” (APA, 2013). There are two courses that sedative, hypnotic, and
anxiolytic use disorders generally follow. The most common course is where
individuals who are often using other substances such as alcohol, opioids, and
stimulants escalate occasional use of these substances especially in social
situations like parties until they meet criteria for diagnosis. In this situation, there
are long-term cognitive and interpersonal difficulties as well as severe
withdrawal symptoms. A less common course is where individuals in their 40s
who have been prescribed sedatives, hypnotics, or anxiolytics by a medical
provider for anxiety, insomnia, or somatic complaints experience tolerance and
self-administer higher doses until they meet criteria for diagnosis. These
individuals will often seek prescriptions from several providers. As individuals
age, the use of sedatives, hypnotics, and anxiolytics poses greater risks, both in
terms of motor coordination and cognitive deficits and the metabolism of
substances. Individuals with neurocognitive disorders such as dementia are more
likely to experience intoxication and toxic side effects at lower dosages (APA,
2013).
Stimulants
Stimulant use disorders are more common in young people ages 12 to 25 than in
those over 26 years old (APA, 2013). Methamphetamines are often prescribed to
control weight gain, treat attention deficit disorder, and improve school, work, or
athletic performance. Some individuals will begin by using medications
prescribed for others. Patterns of use can be daily or episodic. In addition, there
are binges where individuals consume these substances for hours and days until
their supply is depleted. When amphetamine-type stimulants are taken orally and
cocaine is administered intranasally, there is a more gradual course established
over months and years compared to stimulant smoking and intravenous use,
which results in a rapid progression to a severe stimulant use disorder. As people
use more stimulants, there is a decrease in pleasure and increase in dysphoria
(APA, 2013).
Tobacco
Most adolescents in the United States experiment with tobacco, and by age 20,
20% of them become daily users (APA, 2013). It is rare for people over 21 to
initiate smoking. The symptoms of tobacco use disorder appear quickly after
tobacco is consumed. About 80% of individuals with this disorder attempt to
stop, but 60% resume and only 5% achieve abstinence. Most people do not
achieve abstinence until after age 30 (APA, 2013).
Gambling
Gambling use disorders develop gradually over many years and at earlier ages in
males compared to females (APA, 2013). Females with gambling use disorder
are more likely than males to have comorbid depressive, bipolar, and anxiety
disorders and to seek treatment. Most individuals with this disorder report
problems with one to two different types of gambling but with more trouble with
one type than the other. The frequency and the amount of money involved do not
determine the severity of the gambling use disorder. Some people can gamble
large amounts of money once a month and not have a gambling use disorder,
while others who wager small amounts on a daily basis meet criteria for this
disorder. Gambling can increase in response to stress, depression, substance use,
or abstinence. The course may follow periods of heavy gambling and serious
problems, total abstinence, and sometimes nonproblematic gambling, which may
lead to a false sense of invulnerability (APA, 2013).
Diagnosis
Substance-related disorders are divided into substance use disorders and
substance-induced disorders. The diagnosis of substance use disorders is based
on an atypical configuration of behavior that stems from the use of a substance.
Criteria A described in DSM-5 pinpoint the four types of behavior necessary in
order to make a substance use diagnosis: impaired control, social impairment,
risky use, and pharmacological criteria. Two examples that cause significant
distress or impaired functioning must be present within a 12-month period (APA,
2013). These key characteristics were defined and explained earlier in the
chapter. Pharmacological criteria include not only behaviors associated with
tolerance but also physiological changes that are detected by laboratory tests, for
example, high blood levels of the substance occurring with little evidence of
intoxication. Withdrawal is also diagnosed with a combination of history and
laboratory tests. The physiological signs of withdrawal are easily measured for
the following substances, alcohol, opioids and sedatives, hypnotics, and
anxiolytics, whereas the laboratory detection of withdrawal for the stimulants,
tobacco, and cannabis is less obvious. The behavioral and physiological signs of
withdrawal from PCP, other hallucinogens, and inhalants are not detectable
(APA, 2013). DSM-5 also includes a number of specifiers to diagnose these
disorders with greater precision. These specifiers consist of severity of use (mild,
moderate, severe), level of remission (early, sustained, maintenance, controlled
environment), use of more than one substance at a time, presence of other mental
disorders, and presence or absence of perceptual disturbances (APA, 2013).
The criteria for diagnosing substance-induced disorders are divided
separately into the characteristics for intoxication and for withdrawal, which are
included within the descriptions of the 10 classes of substances in DSM-5.
Intoxication is diagnosed based on the following criteria. Criterion A describes a
reversible substance-specific syndrome caused by the ingestion of the substance.
Criterion B specifies the behavioral and psychological changes that occur with
intoxication. Criterion D specifies that the signs and symptoms are not
attributable to another medical or psychiatric condition. Withdrawal is diagnosed
based on the following criteria. Criterion A identifies the key behavioral,
psychological, physiological, and cognitive characteristics that result from the
cessation or reduction in heavy use of a substance. Criterion B highlights the key
functional areas of distress and impairment that occur as a result of withdrawal.
Criterion D specifies that the signs and symptoms are not attributable to another
medical or psychiatric condition (APA, 2013).
Evidence-based intervention strongly suggests the combined use of
medications and psychosocial support (Fig. 16.10) for the most effective
treatment of substance-related disorders (Stoffel & Moyers, 2004). Medications
are primarily used for the following substance-related disorders: alcohol, opioid,
and tobacco. There is often a pattern where the use of medications for these
disorders competes with the use of the abused substance to block or diminish its
effect (Paxos & Dugan, 2015).
Figure 16.10 Continuum of Care Model. (From Lowinson, J. H., Ruiz, P.,
Millman, R. B., & Langrod, J. G. (2004). Substance abuse. Philadelphia, PA:
Alcohol
The four FDA-approved medications for alcohol-related disorders are
acamprosate (Campral), disulfiram (Antabuse), oral naltrexone (ReVia), and
extended-release injection naltrexone (Vivitrol), but only a small percentage of
US residents take these medications (SAMSHA, 2010). They are prescribed to
enable a client to be abstinent, not as a means to taper the withdrawal from
alcohol. The abrupt cessation of alcohol ingestion by a client who has been a
heavy user can have very serious health consequences, including seizures and
neurological injury.
Acamprosate is given to clients who are seeking complete abstinence and are
currently not using to help decrease cravings and reduce other uncomfortable
symptoms, including insomnia, restlessness, and mood changes, which might
cause a relapse (NIAAA, 2005b). It requires the client to take 2 to 3 pills a day
for an extended period. The side effects are generally mild, except when a person
first starts taking this medication, at which time there can be acute side effects,
including diarrhea, nausea, anxiety, and depression, which may entail suicidal
ideation or attempts that must be closely monitored (SAMHSA, 2010; Paxos &
Dugan, 2015). Since it does not interfere with opioids, acamprosate is
appropriate for clients who are receiving opioid maintenance therapy or taking
opioids. There is also no interaction with other medications. Clients with severe
kidney problems should not take this medication. Otherwise, it is a safe
medication with no overdose risk and no potential for abuse and can be
continued even if a person relapses and requires detoxification (SAMSHA,
2010).
Naltrexone can be taken orally or by injection. It works as an opioid
antagonist (blocker) that reduces the positive effects and cravings of alcohol and
is effective in individuals who have a history of opioid use but are now abstinent.
It has not been found effective in individuals using other illicit substances and
should not be taken by those currently using illegal opioids or on methadone
maintenance therapy (SAMHSA, 2010; Sells et al., 2011). Pills are taken orally
once daily. Side effects of oral naltrexone are mild, usually subside over time,
and include nausea, vomiting, headache, dizziness, fatigue, and drowsiness.
Extended-release injectable naltrexone is provided once monthly. It is
recommended for those who are motivated to maintain abstinence, have
problems adhering to treatment regimens, and have the means to pay for its high
costs. The most prominent and bothersome side effect of the injectable form is
painful skin reactions at the site of the injection, which require immediate
medical attention (SAMHSA, 2010).
Disulfiram has been used to treat alcohol use disorders for over 60 years. It
reduces the desire to use alcohol by adversely impacting the user’s metabolism
and other bodily systems (Sells et al., 2011). If a person consumes alcohol while
taking disulfiram, he or she will experience a toxic reaction even if the alcohol is
prepared in food. The person can become sweaty, have difficulty breathing, and
experience blurred vision, head and neck throbbing, confusion, vertigo or
syncope, nausea, flushing, tachycardia, and restlessness. Severe reactions
include life-threatening cardiac and respiratory problems, such as heart attacks,
seizures, and death (SAMHSA, 2010). The reaction varies from moderate to
severe in individuals, typically occurs 10 to 30 minutes after alcohol intake, and
can last up to 2 weeks. Individuals taking disulfiram should carry a medical alert
card indicating that they are taking it and providing emergency procedures to
follow in case of a severe reaction. The drug is considered most effective if a
person is trying to be abstinent or in risky drinking situations (NIAAA, 2005b).
It is taken as a tablet once daily. Side effects are generally mild and usually
disappear after 2 weeks and include drowsiness, a metallic taste, dermatitis,
headache, and impotence (SAMHSA, 2010). There are two new promising
medications for alcohol use disorders, but they are still in clinical trials,
topiramate and baclofen (Sells et al., 2011).
Opioids
Medications used to treat opioid use disorders include methadone (Dolophine), a
combination of buprenorphine and naloxone (Suboxone), buprenorphine
(Butrans), and naloxone (Narcan). Methadone is a synthetic, long-lasting opioid,
which has been used in the treatment of opioid addiction for more than 40 years.
Methadone mimics the actions of opioids by binding to opiate receptors to
prevent cravings and reduce the chances of relapse. This medication is
controversial because it is also addictive, but it does not lead to overdose,
infections, and death (Sells et al., 2011). It is usually dispensed as a premixed,
colored liquid and distributed only at clinics and treatment facilities to minimize
its abuse. Methadone prescriptions increased by 700% between 1998 and 2006
(Center for Substance Abuse Treatment, 2009). When methadone is used
properly, it is considered very safe. Typical side effects include faintness,
dizziness, nausea, vomiting, perspiring, and drowsiness. Adverse events can
occur when an individual is simultaneously abusing other substances, such as
benzodiazepines, other opioids, cocaine, or alcohol, or there are interactions with
other medications. Because methadone is long-lasting and is slowly released into
the bloodstream from the liver and other tissues, toxicity or poisoning can occur
if a patient takes methadone faster than the body can metabolize it or if the dose
is increased too quickly (Center for Substance Abuse Treatment, 2009). Twelve
months is considered the minimum treatment for methadone maintenance
(NIDA, 2012b). The combination of buprenorphine and naloxone is also used to
treat opiate addiction. Buprenorphine binds to opiate receptors to prevent highs
and withdrawal symptoms, and naloxone is an opiate receptor antagonist, which
blocks the opiate from binding to its receptor. Sublingual pills, which are used as
tablets under the tongue, are used as maintenance therapy. Typical side effects
from this combination include headache and other pain, diarrhea, rhinitis,
sleeplessness, and liver damage, which must be closely monitored (Paxos &
Dugan, 2015).
Tobacco
The prescription medications, varenicline (Chantix) and bupropion (Wellbutrin),
are used for the treatment of tobacco addiction as well as nicotine replacement
therapy (NRT) (patches, gum, lozenges, and nasal spray). Varenicline is the most
recently approved medication for smoking cessation. It acts as a partial
agonist/antagonist at nicotine receptors in the brain, which means that it partially
stimulates the nicotine receptor but not enough to release dopamine so the
person does not get pleasurable effects and decreases cravings (NIDA, 2012b).
Typical side effects include nausea, constipation, flatulence, vomiting, abnormal
and/or strange dreams, sleep disturbances, significant mood changes, and
allergic reactions (Paxos & Dugan, 2015). Bupropion was discovered to decrease
tobacco craving in patients who were depressed (NIDA, 2012b). Its most
prominent side effects include dry mouth, nausea, stomach and headaches, light-
headedness, ringing in the ears, decreased interest in sex, weight loss, skin rash,
and sore throat. NRT provides low levels of nicotine, which prevent withdrawal
symptoms that make it difficult for people to quit. Using more than one of the
replacement therapies in combination has been found to be more effective
(NIDA, 2012b). The patches, which are applied once a day, provide consistent
levels throughout the day, while gum, lozenges, nasal spray, and inhalers deliver
sporadic dosages. It is important to follow NRT protocols for maximal effect, for
example, the gum must be chewed until the perception of a prickly taste and then
placed in the cheek until the nicotine is absorbed. Side effects of NRT include
unusual oral tastes, and if a person has a heart problem, he or she must use only
low doses (Paxos & Dugan, 2015).
The severity level of use of the 10 classes of substances determines their impact
on occupational performance. Individuals with mild use disorders probably can
participate in most or all occupations. Individuals diagnosed with severe use
disorders will face barriers in most or all areas of occupation. This section will
summarize the common difficulties faced by users of all substances at the severe
level in each occupation as well as highlight particular difficulties that
accompany specific classes of substances.
ADLs
The primary areas of ADLs impacted by severe levels of all substance use
disorders are hygiene, grooming, time management, and sexual activity (Carifio,
2014; Sells et al., 2011). The avolition characteristic of cannabis users can
express itself as a decreased interest or disregard for grooming or personal
hygiene. There may also be inappropriate choices of clothing for the weather or
occasion. Sexual activity may be impacted and expressed as risky behavior or
lowering of sexual drive or interest. Stimulant users believe they have the
capacity to outperform other people in ADLs when in actuality their
performance is poor (Martin, Bliven, & Boisvert, 2008).
IADLs
The primary IADLS impacted include communication management, driving and
community mobility, health management and maintenance, medication
management, financial management, and home establishment and management
(Sells et al., 2011). Acute intoxication involving motor skills and reflexes can
interfere with driving cars, riding bicycles, taking public transportation, and
operating machinery. Doctors and clinics may be avoided to get around drug
testing, and medication compliance activities may be abandoned. Complex
financial and home management activities may be abandoned due to cognitive
impairments and a focus on substance obtaining and using. The user’s ability to
provide care for others or supervise others is often compromised. Safety
awareness is negatively impacted by lack of awareness of the environment.
Stimulant users believe they have the capacity to outperform other people in
IADLs when in actuality their performance is poor. Individuals who are using
substances illicitly will often become involved in theft, prostitution, or drug
dealing in order to obtain drugs or money for substances (Hoppes, Bryce, &
Peloquin, 2013; Stoffel & Moyers, 2001).
Rest and Sleep
All three areas of this occupation (rest, sleep preparation, and sleep
participation) are adversely impacted by a disrupted sleep-wake cycle, poor
orientation to time, and impaired sequencing skills, which characterize most of
the classes of substance use (Sells et al., 2011). Strange nightmares and insomnia
often characterize this area of occupation.
Education and Work
Both education and work are negatively impacted by higher rates of truancy and
absenteeism, tardiness, accidents, poor performance, lack of volition, and the
inability to follow through with commitments. Dropping out of school and job
loss and turnover often result from substance use disorders, which lead to
numerous negative consequences, including financial difficulties, unstructured
days that perpetuate poor self-esteem, and, in some cases, incarceration
(Gutman, 2006; Hoppes et al., 2013). Employment opportunities may not be
pursued due to fear of failing drug testing. Cannabis use is identified with
motivational syndrome, which can affect advancement in education and work
arenas.
Play and Leisure
Substance use disorders that develop in early adolescence interrupt typical
developmental milestones in play exploration and the acquisition of leisure
activities, which can aid in stress management and patterns of healthy living in
adulthood. The primary focus of substance users becomes obtaining the drug and
getting high. Money spent on the seeking and consuming substances is no longer
available for play and leisure participation. When individuals stop using
substances, they often are at a loss for how to structure their days and manage
their time. When intoxicated or in withdrawal, individuals often experience
anhedonia or engage in risky behaviors such as driving recklessly or
participating in unprotected sexual activity (Heuchemer & Staffan, 2006; Knis-
Matthews, 2003).
The inability to regulate emotions and poor orientation to person, place, time,
self, and others as well as poor ADLs and IADLs often lead to impaired
relationships and roles with family, friends, co-workers, peers, and community.
Family members especially become angry with the users because their aberrant
behavior violates trust and negatively impacts the entire family structure. For
example, parents who are heavy users cannot carry out their roles (Knis-
Matthews, 2003). Individuals with substance use disorders will often self-isolate
because of embarrassment, fear of failing, lack of hope, slurred speech,
dysphoric or apathetic mood, inability to understand what someone else says,
lowered frustration, and poor judgment. During intoxication, users can become
either more social or more withdrawn, while during withdrawal, mood and
physiological difficulties can negatively impact social participation (Boisvert,
Martin, Grosek, Clarie, 2008; Gutman, 2006; Hoppes et al., 2013; Sells et al.,
2011).
Case Study 1
Case Illustration
Adolescent with Stimulant or Opioid Disorder Donald is an 18-year-old
African American adolescent male who is living in a homeless shelter in a
medium-sized northeastern city. He grew up with his mother who had been
addicted to heroin when he was younger, but she is now involved in a
methadone maintenance program. He never knew his father. His
grandmother has been a stabilizing influence in his life, and he maintains
contact with her. Donald was diagnosed with ADHD in elementary school
and was placed in special education classes. At age 10, he was prescribed
the stimulant Adderall, an amphetamine, but living in shelters made follow-
up by a psychiatrist difficult. He would get Adderall from other kids at his
high school before exams and when papers were due. Donald was athletic
and played on the varsity basketball team in high school. He liked to
socialize with friends, and when he attended parties, he joined the crowd
and smoked cannabis. He liked the relaxation that came with marijuana and
began to use it on a daily basis. Since moving into the shelter, he has been
experimenting with smoking K2, a hallucinogen, which he likes because it
is sold as potpourri and as a natural substance and is very cheap. He can
purchase it at a local tobacco store and not have to worry about getting
arrested when making purchases of illicit drugs from dealers on the street.
He also believes that the K2 is safer because it is sold in a package, whereas
the marijuana sold on the street is unregulated and can sometimes contain
toxic substances.
Donald’s views about K2 changed drastically after the last time he tried
it. He experienced very severe symptoms for more than an hour, including
hallucinations, tachycardia, nausea and vomiting, and syncope. He was
taken to the emergency room of the local hospital by the shelter staff. When
he sobered up from this episode, an OTR who worked in the ER
approached him about his problems with polysubstance use. She asked him
what his daily schedule looked like and what his short- and long-term goals
were. He said he had been a B student in school and enjoyed playing
basketball and that he wanted to attend college. The OTR asked him what
steps he was taking to achieve his goal. Donald said, “None, and I think I
need some help.” He called his grandmother and spoke to her and his
mother, both of whom advised him to enroll in an inpatient program for
substance use treatment. They also suggested that he try attending Narcotics
Anonymous Meetings, which would provide him with daily support once
he was discharged. Each of them promised to be there for him during this
rough stretch. They also expressed their strong belief in him and his ability
to recover from these substance-related problems.
Case Study 2
Adult with Disabilities— Sedatives,
Hypnotics, and Anxiolytics
Jose is a 50-year-old Hispanic man who has been deaf since birth. He lives
by himself in an apartment in a large city in the southwestern United States.
His family consists of his sister and elderly parents who live nearby. He
uses sign language and reads lips in order to communicate. He was enrolled
in special education in elementary and secondary school while growing up.
For college, he moved away from the Southwest and attended Gallaudet
University for the Deaf in Washington, DC, where he majored in political
science. He loved his college experience and thrived in the deaf community.
He formed lifelong friendships at Gallaudet. After graduation, he was able
to obtain a job in the federal government, in the Department of Education.
He worked in Washington, D.C., for 25 years, successfully supporting
himself. His leisure interests included swimming and advocacy work for the
deaf community. When he was in his 40s, his elderly parents became ill and
he decided to return to the Southwest to help his sister care for them.
Jose did not anticipate that he would have difficulty finding a new job.
The arduous job search depleted his savings and left him feeling extremely
anxious and vulnerable. He consulted with his internist about his symptoms,
which included insomnia and intense feelings of self-doubt and agitation
during job interviews. The internist referred him to a psychiatrist who
prescribed Restoril for improving his sleep and Xanax to be used when he
became symptomatic before an interview. The sedative and anxiolytic
medications relieved his symptoms, but he found that he needed to take
more Xanax at other times besides job interviews. Whenever he was in a
new situation or meeting people for the first time, he felt he performed
better if he took some Xanax, which he began to self-administer in higher
doses. His original psychiatrist would not prescribe additional anxiolytic
medication unless Jose started therapy. Jose was not eager to be in therapy
but agreed to see a therapist who knew how to communicate with people
who are deaf. His sister searched the city for someone with these
qualifications and could not find either a therapist or a treatment program
that had expertise in working with clients who are deaf or any clients who
abused substances and had a physical or sensory disability. In the
meantime, Jose went to several other providers who gave him prescriptions
for the sedative and anxiolytic medications.
After several years of this course, Jose noticed that he was experiencing
difficulty with motor coordination, cognitive deficits, and the metabolism of
the substances. His sister was especially concerned because when Jose
stopped using, he experienced increased anxiety attacks, lethargy, and
emotional lability. She decided to contact an old friend of Jose’s from
Gallaudet who was a psychiatrist, Dr. Powers. When Dr. Powers heard what
was going on with her old friend, she made arrangements that he be
admitted immediately to a local detox center because she was very
concerned about the likelihood of seizures that can accompany sedative,
hypnotic, and anxiolytic withdrawal disorders. She reassured Jose’s sister
that they would work together to find Jose a therapist and substance use
recovery program that were specifically trained to work with clients who
are deaf and living with disabilities.
Acknowledgments
Kathryn Reynolds, OTS, Marcy Schlissel, OTS, Allison Schubert, OTS, and
Zachary Schluger, OTS
RESOURCES
https://medicine.wright.edu/citar/sardi
This is the SARDI Program (Substance Abuse Resources & Disability Issues). It
is on the Web site of the Wright State University’s Boonshoft School of
Medicine. Its purpose is to improve the quality of life for people living with
physical disabilities who face behavioral problems. Through participatory
research, it has produced several unique programs: HIV services for people who
are minorities and have physical disabilities; Deaf Off Drugs that targets the deaf
community; programs for formerly incarcerated individuals who also have HIV;
tobacco education and cessation programs for people with disabilities; and
research resources for professionals working with people with disabilities who
also have substance-related disorders.
http://www.smartrecovery.org/meetings/olschedule.htm
The SMART Program (Self Management and Recovery Training) is a free
nonprofit supported outpatient treatment based on Ellis’ Rational-Emotive-
Behavioral Therapy. This is a form of cognitive-behavioral therapy that teaches
skills for recovery. It is a form of self-help but based on scientific research rather
than dependency on faith; it has been called non–12-step rehab. It includes
weekly meetings, individual therapy, and the use of medications to treat
substance use disorders.
Alcoholics Anonymous Online Intergroup http://aa-intergroup.org/index.php
Online resources for the self-help approach advocated by Alcoholics
Anonymous (AA), including online chat rooms, immediate assistance, a global
and local directory of meetings, upcoming conferences, and AA literature.
Narcotics Anonymous Chat and Online Meetings for Drug Addicts
http://www.12stepforums.net/na
An online group with immediate 24-hour/7 days a week access for all people
with drug use disorders to find support and fellowship.
Baylor College of Medicine Center for Research on Women with Disabilities
http://www.bcm.edu/crowd
Online site for the Center for Research on Women with Disabilities (CROWD)
whose mission is to advance the quality of life of women with disabilities by
developing and disseminating information. It is a consortium of local and
national consumer advocates, medical advisors, and researchers. Some of the
topics they have investigated include health behaviors (weight management,
smoking cessation), access to health care, violence, and abuse.
Minnesota Chemical Dependency Program for Deaf and Hard of Hearing
Individuals http://www.mncddeaf.org
This program was started in 1989 to provide services for the deaf community
who are struggling with any form of chemical dependency. They provide direct
services as well as train providers to deliver specialized care to this population.
Yale–CASA National Center on Addiction and Substance Abuse
http://www.casacolumbia.org/
CASA–Columbia is teaming up with Yale University’s Medical School and
Public Health to form a new research, policy, and education center on addiction.
National Institute on Drug Abuse (NIDA) http://www.drugabuse.gov/about-
nida
This is the federal government’s preeminent organization to fund research on
addiction. Their role is to also disseminate results of research to improve
prevention and intervention services as well as to inform policy makers.
National Institute on Alcohol Abuse (NIAA) http://www.niaaa.nih.gov/
The mission of this federal government institute, which is part of NIH, is to lead
the nation’s efforts on clinical and basic science intramural and extramural
research on alcohol addiction through grants, contracts, and cooperative
agreements. NIAA coordinates research with national and international bodies.
In addition, its research mission is to discover the basic science of alcohol
addiction in order to establish evidence-based programs of prevention, treatment,
and health services.
Substance Abuse Mental Health Services Administration (SAMHSA)
http://www.samhsa.gov/
This is the agency within the federal Department of Health and Human Services
charged with the public health agenda of prioritizing the behavioral health of the
US population. Its purpose is to fund and organize a national effort to reduce the
impact of substance abuse and mental illness on individuals and communities by
making research, information, and services more easily accessible. Its primary
purpose is to facilitate recovery for these populations. Its online site is home to
easily accessible and valuable evidence-based resources for consumers, families,
and professionals.
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UNIT
3
Physical Conditions
The Physical Conditions Unit includes the most common physical conditions
that clients have who are treated by occupational therapists as determined by the
National Board of Certification in Occupational Therapy. These chapters focus
on conditions that are typically diagnosed in adults, but may also be seen in
pediatrics. People with these conditions are typically referred to occupational
therapy for the specified physical condition as a primary diagnosis, but they may
also have the physical condition as a secondary diagnosis. Each chapter provides
information about the etiology, incidence and prevalence, signs and symptoms,
course and prognosis, diagnosis, medical/surgical management and impact on
occupational performance of these conditions. Case illustrations are used to
provide examples of lives affected by the condition. The conditions included in
this unit are the following:
Chapter 17: Cerebrovascular Accident
Chapter 18: Cardiopulmonary Disorders
Chapter 19: Diabetes
Chapter 20: Acquired Brain Injury
Chapter 21: Burns
Chapter 22: Progressive Neurodegenerative Disorders
Chapter 23: Rheumatic Diseases
Chapter 24: Spinal Cord Injury
Chapter 25: Orthopedics
Chapter 26: Low Vision Disorders
Chapter 27: Cancer
Chapter 28: Obesity
C H A P T E R
17 Cerebrovascular Accident
Mylene Schriner Joan Ziegler Delahunt
KEY TERMS
Agnosia
Aneurysm
Apraxia (motor planning)
Associated reactions
Ataxia
Atherosclerosis
Deep vein thrombosis
Dysarthria
Dysphagia
Embolism
Flaccidity
Hematoma
Homonymous hemianopsia (hemianopia) Hemiparesis
Hemiplegia
Hemorrhagic stroke
Hypotonus
Ischemia
Neural plasticity
Spasticity
Thrombus
Transient ischemic attacks (TIAs) Unilateral inattention (neglect)
M.V. is a 68-year-old native New Yorker. She was born to Irish and Italian
parents on the lower eastside of Manhattan. She attended private preparatory
schools and readily was accepted at Columbia University, where she studied
fashion marketing and design. She received a second undergraduate degree in
business from Barnard College and immediately entered the hectic, high-energy
world of finance, within the world of women’s fashion and apparel. M.V. never
married, finding her life fulfillment more in the glitter and glass world than in
diapers and the seeming melancholia of suburbia. She worked ungodly hours,
often never stopping for lunch, and sometimes missing dinner altogether.
Timelines, deadlines, corporate demands, and the constant need to exceed quotas
in order to appease shareholders made her job monetarily rewarding but
extremely stressful in overt as well as insidious ways. M.V.’s weight was a
constant stressor for her. She seemed to gain 2 to 3 lb each time she walked past
a Baskin-Robbins ice cream store. She was always dieting, or going to diet, or
thinking that she should be on a diet, but the Coke Zero did not make a
discernible dent in her ever-increasing midriff. In addition to her weight, she also
had an ongoing relationship with cigarettes. She continued to be a pack-a-day
smoker despite all of the Relays for Life, pink ribbons, and St. Jude’s
commercials that seemed to trumpet their anticancer messages directly at her
whenever she gave them a second glance. M.V. was 30 lb overweight and has
had high blood pressure since she was a sophomore in college. M.V. was on her
usual path from the subway station at 54th St. and Lexington Avenue to her
office, when she began to sense that something was happening to her that she
had never before experienced. The first sensation was one of a warm drape being
pulled over her head; the air she was breathing became thick and difficult for her
to draw into her chest. Then there was a bit of a tingle in her right hand, as
though the pinky and ring fingers suddenly decided to fall asleep together, the
bag of bagels she was carrying to share with her co-workers slipped to the
pavement unnoticed by M.V. As she attempted to make sense of what was
happening to her, breathing became more challenging, like drawing thick soup
through a too-thin straw. She leaned on a nearby storefront and vomited. As she
leaned there for a moment, a man came to her side and spoke in a language she
seemingly had never heard before, she thought “was he speaking Russian,
Latvian, certainly someone here speaks English?” Within a minute or two, M.V.
was being guided to the pavement by two people who appeared to be wearing
uniforms; possibly they worked for a Russian circus? She was now lying on the
pavement feeling frightened, embarrassed, and very, very confused. There was
no way she could move her right hand or arm, and her right lower body seemed
to have floated away from her and onto another part of the universe. M.V. was
taken to a hospital by ambulance, with much of that event lost to her memory.
What is happening to M.V.? Is there enough evidence to suspect that a stroke
may be evolving? This chapter will provide insight into M.V.’s condition and
help provide answers to these questions.
A stroke, or brain attack, results from an interruption in the blood flow to the
brain from either a blocked or ruptured blood vessel. The consequence is an
inadequate supply of oxygen and nutrients to this vital organ. Even a brief
disruption of this blood flow can lead to brain damage. Medical practitioners use
the term “cerebrovascular accident,” often abbreviated as CVA, for stroke. A
CVA can occur in any part of the brain, the cerebral hemispheres, the
cerebellum, or the brainstem. The site and extent of the affected area, or infarct,
determines loss of function.
CVAs are divided into two main types: ischemic and hemorrhagic.
Ischemic strokes are characterized by blockages (the term ischemia refers to the
lack of blood supply) and include atherothrombotic, lacunar, and embolic
infarctions, in that order of frequency. Hemorrhagic strokes include intracerebral
and subarachnoid hemorrhages (Broderick et al., 2007). Both types of stroke
lead to the death, or infarction, of brain tissue. CVA terminology involves a
complex composition of pathophysiological entities that include thrombosis,
embolism, and hemorrhage (Saenger & Christenson, 2011). In most cases, a loss
of blood supply is the result of long-standing degeneration of the body’s blood
vessels. Less commonly, a CVA occurs because of an inborn abnormality or
weakness of the brain’s vascular supply (Ferguson, 2010). A brief review of
cerebral circulation will help understand the impact of each type of stroke.
Cerebral Circulatory System
The blood supply of the brain is extremely important because the brain is one of
the most metabolically active organs of the body. Although it constitutes only
2% of the body’s weight, the brain receives approximately 17% of the cardiac
output and consumes about 20% of the oxygen used by the entire body (Lundy-
Ekman, 2007).
In the brain, the arteries of the anterior circulation supply the front, top, and
side portions of the cerebral hemispheres. The brainstem and cerebellum, as well
as the back and undersurface of the cerebral hemispheres, are supplied by the
posterior circulation. These two areas of circulation are further categorized into
the extracranial portions (arising from outside the skull and traveling toward the
brain) and the intracranial portions (arising from within the skull) (Lundy-
Ekman, 2007).
Extracranial Vessels
Extracranial anterior circulation consists of the two carotid arteries, which travel
in the front of the neck on each side of the trachea and esophagus (Lundy-
Ekman, 2007). The word “carotid” is derived from the Greek word “karos”
meaning “to stupefy,” or render unconscious, indicating the significance of this
main artery in maintaining consciousness and brain function (Qureshi et al.,
2007). The right common carotid artery arises from the innominate artery. The
left common carotid artery originates directly from the aortic arch. Around the
fifth or sixth vertebrae, these common carotid arteries divide into external
carotid arteries, whose branches supply the face and its structures, and the
internal carotid arteries, which supply the eyes and the cerebral hemispheres
(Lundy-Ekman, 2007; Qureshi, 2007).
The vertebral arteries arise from the subclavian arteries and make up the
extracranial posterior circulation. They remain within the vertebral column for
part of their course from about C6 to C2. The vertebral arteries enter the cranium
through the foramen magnum (Lundy-Ekman, 2007; Qureshi, 2007).
Intracranial Vessels
The internal carotid arteries enter the skull through the carotid canal and form an
S-shaped curve called the carotid siphon (Qureshi et al., 2007). The artery then
enters the subarachnoid space by piercing the dura mater. It gives rise to the
ophthalmic arteries, which supply the eyes; the posterior communicating
arteries, which join with the posterior circulation; and the anterior cerebral
arteries, which supply the orbital and medial surfaces of the frontal lobes and
part of the basal frontal lobe white matter and caudate nucleus. The internal
carotid artery also branches off to create the middle cerebral arteries, which
supply almost the entire lateral surface of the frontal, parietal, and temporal
lobes, as well as the underlying white matter and basal ganglia (Lundy-Ekman,
2007). The middle cerebral artery is the largest of the terminal branches of the
internal carotid artery and is the direct continuation of this vessel.
The vertebral arteries enter the cranium within the posterior fossa and travel
along the side of the medulla, where they produce their longest branch, the
posterior inferior cerebellar artery. This artery supplies the lateral medulla and
the back of the undersurface of the cerebellum (Alastruey, Parker, Peiró, Byrd, &
Sherwin, 2007). The two vertebral arteries then join at the junction between the
medulla and pons to form the single midline basilar artery (Alastruey et al.,
2007). The basilar artery gives off penetrating arteries to the base of the pons and
two vessels (the anterior inferior and superior cerebellar arteries), which supply
the upper and anterior undersurfaces of the cerebellum (Alastruey et al., 2007).
At the level of the midbrain, the basilar artery bifurcates into the two posterior
cerebral arteries (Alastruey et al., 2007). As they circle the brainstem, these two
arteries give off penetrating branches to the midbrain and thalamus and then
divide into branches that supply the occipital lobes as well as the medial and
undersurfaces of the temporal lobes (Alastruey et al., 2007). One of the branches
of the posterior cerebral artery, the calcarine artery, is of special significance
because it is the main supplier of blood for the visual area of the cortex (Lundy-
Ekman, 2007).
Communicating Arteries
The right and left carotid vessels connect with each other when they enter the
brain, each sending out a small lateral branch that meets in the space between
them. These are the anterior communicating arteries. They also branch backward
to join with the right and left posterior cerebral arteries, called the posterior
communicating arteries. This communicating vascular interchange is known as
the circle of Willis, which is pictured in Figure 17.1. It protects the brain should
one of the four major supplying arteries coming up through the neck be blocked
(Alastruey et al., 2007). This important anatomical feature is named for Dr.
Thomas Willis and was first described in the mid-17th century (Zazulia, 2009).
Starting from the midline anteriorly, the circle consists of the anterior
communicating, anterior cerebral, internal carotid, posterior communicating, and
posterior cerebral arteries, from which it continues to the starting point in reverse
order (Zazulia, 2009).
Figure 17.1 Arteries of the brain. (From the American Chart Co.)
When one major vessel supplying the brain is slowly occluded, either within the
circle of Willis or proximal to it, the normally small communicating arteries may
slowly enlarge to compensate for the occlusion (Lundy-Ekman, 2007). This
system is imperfect, however, and it often fails to prevent strokes. In many
individuals, the same atherosclerotic processes that caused a stroke also may
damage communicating arteries. In addition, only about one-fourth of strokes are
caused by a blockage of the major neck vessels (Qureshi et al., 2007). For
approximately one-third of the population, the communicating artery may be
insufficient or even absent (Alastruey et al., 2007). Such anomalies are more
common in those who have strokes than in the general population and may be
linked to increased risk of stroke in persons who also have atherosclerosis (Van
Kooij, Hendrikse, Benders, De Vries, & Groenendaal, 2010).
Types of Cerebrovascular Accident
Ischemic Stroke
Ischemic stroke is the most common type of CVA accounting for 88% of cases
(Go et al., 2013). Cerebral infarction, or brain tissue death, results when
circulation to an area of the brain is obstructed, with the result being ischemia.
Ischemic strokes are classified as thrombotic, embolic, or lacunar strokes. The
damaged area has two components: the tissues that have died as a result of blood
supply loss and the peripheral area in which there may be temporary dysfunction
as a result of edema. Edematous brain tissue sometimes recovers slowly and
gradually, resulting in a reappearance of function after a period of 4 to 5 months
(Go et al., 2013). In the past, prognoses for functional recovery have been
limited to this time frame. However, recent research offers promising evidence
that recovery is possible months and even years post CVA (Hankey et al., 2007).
Explanations focus on the brain’s ability to reroute neural pathways, a
phenomenon known as neural plasticity (Huttenlocher, 2009; Takatsuru et al.,
2009). Current research supports that recent advances in functional imaging of
human brain activity of clients with a CVA demonstrate that the cortical
hemisphere contralateral to the infarction lesion serves a vital role in the
recovery process and that the most optimal recoveries are linked with the
greatest return toward the normal state of brain functional organization (Hara et
al., 2015). Further, neural plasticity is evident with positively impacting
functional outcomes for clients with past CVA (Rodríguez-Mutuberría et al.,
2011).
The actual physiologic events that follow an ischemic stroke occur in
characteristic steps. First, the membrane surrounding each affected neuron leaks
potassium (a mineral necessary for producing electrical impulses) and adenosine
triphosphate (ATP, an energy-producing biochemical found in the body). Fluid
quickly accumulates between the blood vessel and neuron, making it difficult for
oxygen and nutrients to pass from the bloodstream into the damaged neuron. The
initial injury produces a vicious cycle in which more cellular injury results.
Irreversible cell death will occur in 5 to 10 minutes if oxygen and nutrients are
unable to reach them from the bloodstream or in a slightly longer period if blood
flow is only partially interrupted. These dead cells form a zone of infarction that
will not regenerate (Minger et al., 2007). Downstream from the infarct zone is a
zone of injury (penumbra) (Bose et al., 2008; Gonzalez, 2006). This area may be
served by collateral blood vessels and is capable of returning to normal
functioning. A third area that reacts differently to the stroke process may also
exist. In this area of hyperemia, the blood vessels are congested and swollen and
also may have the potential for recovery.
Thrombosis
Cerebral thrombosis occurs when a blood clot forms in one of the arteries
supplying the brain, causing vascular obstruction at the point of its formation.
The size and location of the infarct depends on which vessel is occluded and the
amount of collateral circulation. Thrombosis occurs most frequently in blood
vessels that have already been damaged by atherosclerosis (Ho, Huang, Khor, &
Tay, 2008).
Atherosclerosis is a gradual degenerative disease of the blood vessel walls. It
is a pathologic process rather than a normal effect of human aging (Ho et al.,
2008). Rough, irregular fatty deposits form within the intima and inner media of
the arteries and often lead to the generation of a thrombus, or blood clot. This is
the most common cause of CVA, with stenosis, or narrowing of the blood
vessels, resulting in much fewer cases (Ho et al., 2008). Large-vessel
atherosclerosis accounts for 60% of ischemic stroke (Bang et al., 2010). Because
the body’s blood vessels have a significant reserve capacity, ischemic strokes do
not usually occur until the vessel is two-thirds blocked (Bang et al., 2010). The
impact of atherosclerosis on the vascular system is considerable; it is also a
major risk factor for heart disease.
To understand the process that produces a mass of degenerated, thickened
material (plaque) called atheromas, imagine a glue bottle that has been allowed
to collect the residuals of dried glue. The more clogged the cap of the bottle
becomes, the more difficult it is for the glue to flow through. Squeezing the glue
through the opening can push already dried glue more firmly against the
opening. The opening will become smaller and smaller until it closes completely
or bursts from the increased pressure.
In the cerebral circulation, atherosclerosis and thrombus formation are most
likely to occur in areas where blood vessels turn or divide, such as the origins of
the internal carotid artery and the middle cerebral artery and the junction of the
vertebral and basilar arteries (van Kooij et al., 2010).
Cerebral thrombosis often causes stuttering or progressive symptoms that
occur over several hours or days. Onset during sleep is common. Often, a patient
notices mild arm numbness at night and then awakens the next morning with
paralysis. Transient ischemic attacks (TIAs) precede actual infarction about
half the time (van Kooij et al., 2010).
Lacunar Strokes/Penetrating Artery Disease

Lacunar strokes are small infarcts, usually lying in the deep brain structures,
such as the basal ganglia, thalamus, pons, internal capsule, and deep white
matter (Porter & Kaplan, 2010). Approximately 25% of ischemic strokes are the
result of damage to these deep structures (Porter & Kaplan, 2010). Within a few
months of onset of a lacunar stroke, a small cavity (“lacune” in French) is left
(Porter & Kaplan, 2010).
Lacunar infarcts or lacunes result from occlusion of a single penetrating
artery and account for approximately a quarter of cerebral infarctions (Arboix,
2011). Lacunar infarcts range in size from 2 to 15 mm (Porter, 2010). Because of
their small size, minimal neurological symptoms are often present, and many
such strokes go undetected. Recent findings indicate, however, that long-term
prognosis is not good for lacunar strokes, and the recovery rate is similar to that
for other types of CVA (Basile et al., 2006). Typically, lacunar strokes produce
purely motor deficits (weakness or ataxia), purely sensory deficits, or a
combination of sensory and motor deficits (Arboix, Blanco-Rojas, & Martí-
Vilalta, 2014; Sacio, 2006). Symptoms do not usually include aphasia, changes
in cognition or personality, loss of consciousness, homonymous hemianopsia,
or seizures (Basile et al., 2006). The most consistently identified risk factor for
lacunar infarction is hypertension, and treatment is aimed at controlling it (Basile
et al., 2006).
Embolism
Embolism occurs when a clot that has formed elsewhere (thrombus) breaks off
(embolus), travels up the bloodstream until it reaches an artery too small to pass
through, and blocks the artery (Montaner et al., 2008). At this point, the effects
of the embolus are similar to those produced by thrombosis. Embolic materials
that travel to the arteries of the brain can originate from many sources, including
the aortic arch and arteries arising from it, the extracranial carotid and vertebral
arteries, and thrombi in the heart. Cardiac-source emboli occur in approximately
20% of ischemic strokes and are referred to as cardiogenic (Cho et al., 2009).
Many cardiac abnormalities can give rise to a cerebral embolism, including atrial
fibrillation (AF), coronary artery disease, valvular heart disease, and
arrhythmias. Cardiac surgery is also a cause (Cho et al., 2009). The middle
cerebral artery is by far the most common destination of cardiac emboli,
followed by the posterior cerebral artery (Cho et al., 2009).
In contrast to thrombotic strokes, embolic strokes typically occur during
daytime activity (Cho, 2007; Montaner et al., 2008). The embolism can be
precipitated by a sudden movement, or even a sneeze, which raises blood
pressure and dislodges the clot. Clinical symptoms are usually maximal at onset,
but in some cases, the neurological symptoms improve or stabilize somewhat
and then worsen as the embolus moves and blocks a more distal artery. A history
of TIAs is rare. Seizures may be associated with embolic strokes (Cho et al.,
2007) (Fig. 17.2).
Figure 17.2 Types of stroke. (From Hickey, J. (2013). Clinical practice of

neurological and neurosurgical nursing (7th ed.). Philadelphia, PA: Lippincott
Hemorrhagic Stroke
Approximately 20% of CVAs are hemorrhagic (Amarenco, Bogousslavsky,
Caplan, Donnan, & Hennerici, 2009). Hemorrhagic strokes are caused by a
rupture in a blood vessel or an aneurysm, with resultant bleeding into or around
cerebral tissue. An aneurysm is a bulging or outpouching of a wall of an artery
as a result of weakness in the vessel wall; it is prone to rupture at any time.
While fatality rates for hemorrhagic strokes are higher than are those for
ischemic strokes, recent findings indicate that patients often make a better
recovery after a hemorrhagic stroke (Amarenco et al., 2009). Hemorrhagic
strokes are more common in young people than are ischemic strokes as the
vessel wall anomaly is often congenital. There are two types of hemorrhagic
strokes. An intracerebral hemorrhage refers to bleeding directly into brain
substance, whereas a subarachnoid hemorrhage is bleeding occurring within the
brain’s surrounding membranes and cerebrospinal fluid (CSF) (Feigin, Lawes,
Bennett, Barker-Collo, & Parag, 2009). These two types of hemorrhage differ in
incidence, etiology, clinical signs, and treatment. Age, ethnicity, and
hypertension are strongly linked to intracerebral hemorrhagic stroke
(Grysiewicz, Thomas, & Pandey, 2008).
Intracerebral Hemorrhages
Intracerebral hemorrhage results in bleeding directly into the brain and accounts
for a high percentage of deaths because of CVA (Feigin et al., 2009). It may
occur in any part of the brain and is most commonly linked to hypertension.
Other causes include blood vessel abnormalities, such as arteriovenous
malformations or aneurysms, or trauma (Locksley, 2010). Release of blood into
brain tissue and surrounding edema will then disrupt the function of that
particular brain region (Locksley, 2010). Blood irritates the brain tissue and
causes swelling, or it may form a mass called a hematoma. In either case, the
increased pressure on brain tissue can rapidly destroy them. Factors that increase
the risk of intracerebral hemorrhage include blood and bleeding disorders such
as hemophilia, sickle cell anemia, and leukemia; use of anticoagulants; and liver
disease (Porter & Kaplan, 2010).
Clinical signs of intracerebral hemorrhage are usually focal, that is, unlike
cerebral infarcts, hemorrhagic bleeds do not follow the anatomic distribution of
blood vessels but move spherically through the tissue planes (Locksley, 2010).
Typically, they develop suddenly, often during activity (Amarenco et al., 2009).
While extremely small hemorrhages may go undetected, a large hematoma
causes headache, vomiting, convulsions, and decreased levels of alertness
(Ohwaki et al., 2004). Stupor and coma are common signs of very large
hemorrhages and indicate a poor prognosis, especially among individuals over
85 (Grysiewicz et al., 2008). Nevertheless, recovery is possible.
Cerebral injury caused by intracerebral bleeding is a result of the damaging
effect that the abnormal presence of blood has on the neurons. In addition to the
irritant of the blood, abnormal pressure on neurons distorts their normal
architecture. It also prevents oxygen and nutrients from passing to the cells from
the bloodstream. Eventually, bleeding will stop and a hard clot will form. During
a period of months, the clot slowly recedes, breaks down, and is absorbed by the
body’s white blood cells (Zhao, Grotta, Gonzales, & Aronowski, 2009). If not
damaged by increased pressure, the tissues irritated by the blood may heal,
leading to a positive outcome, perhaps even full recovery (Zhao et al., 2009).
Subarachnoid Hemorrhages
Subarachnoid hemorrhages account for about 3% of all CVAs and are slightly
more common in women (De Rooij et al., 2007). About 95% are caused by the
leakage of blood from aneurysms (Grysiewicz et al., 2008). A combination of
congenital and degenerative factors, usually at the points of origin or
bifurcations of arteries, can precipitate formation of an aneurysm (Ohwaki et al.,
2004). Blood may break through the weak point of the aneurysm at any time
and, because of the force of arterial pressure, spread quickly into the CSF
surrounding the brain. A subarachnoid hemorrhage also may be caused by
bleeding from an arteriovenous malformation, which is an abnormal collection
of vessels near the surface of the brain. Other less common causes of
subarachnoid hemorrhages are hemophilia, excessive anticoagulation therapy,
and trauma to the skull and brain (Amarenco et al., 2009). The extravasated, or
escaped, blood irritates the meninges, and intracranial pressure is increased
owing to extra fluid in the closed cranial cavity. This can lead to headache,
vomiting, and an altered state of consciousness. Sleepiness, stupor, agitation,
restlessness, and actual coma are various manifestations of reduced
consciousness. Headaches are usually severe and are described as the worst in
the patient’s life (Perry et al., 2013). Subarachnoid hemorrhages are diagnosed
through one of these three methods subarachnoid blood on computed
tomography (CT) scan, xanthochromia in CSF, or red blood cells in the final
tube of CSF, with positive angiography findings (Perry et al., 2013) (Fig. 17.3).
Figure 17.3 Ischemic versus hemorrhagic stroke. (From Walton, T. (2010).
Medical conditions and massage therapy. Philadelphia, PA: Lippincott Williams
& Wilkins.)
Etiology
Research suggests that ten risk factors are associated with 90% of the risk of
CVA including history of hypertension, smoking, waist-to-hip ratio, diet risk
score, regular physical activity, diabetes mellitus, alcohol intake, psychological
stress, and cardiac problems. These risk factors were all significant for ischemic
stroke, whereas hypertension, smoking, waist-to-hip ratio, diet, and alcohol
intake were significant risk factors for intracerebral hemorrhagic stroke
(O’Donnell et al., 2010). A number of risk factors are associated with the
likelihood of CVA and resemble those for heart disease, which stands to reason
as atherosclerosis is an underlying cause for both conditions. While some of
these risk factors are inborn or otherwise unavoidable, many are related to
lifestyle and behavioral choices. Specific interventions that lower blood
pressure, eliminate smoking, and promote physical activity and a healthy diet
could substantially reduce the burden of CVA (O’Donnell et al., 2010).
Ethnicity—Although all minority groups are at risk, CVA death rates for
African Americans is more than twice the risk of stroke compared with
other ethnicity groups, and women are at a greater risk for CVA than men
(Saenger & Christenson, 2009). Recent data also indicate that blacks living
in the South have a greater risk of dying from CVA than do those who live
in the northern states (Voeks et al., 2008).
Age—Risk for CVA increases with age, especially after age 65. People with
high blood pressure and who exhibit the other risk factors listed in this
chapter are increasingly vulnerable as they age. Younger people are not
immune, either: approximately 28% of CVAs occur in individuals younger
than age 65 (Bhat et al., 2008; Goldstein et al., 2011).
Heredity—A family history of CVA, particularly on the father’s side,
increases one’s risk (Bhat et al., 2008). Genetic factors account for 7% to
20% of cases of subarachnoid hemorrhage, and some researchers
recommend that people with more than one close relative who suffered a
hemorrhagic stroke be screened for aneurysms (Locksley, 2010).
Obesity—Being overweight is a known risk factor for hypertension and
diabetes mellitus and also is associated with CVA. Weight that is centered
in the abdomen (so-called apple shape) has a particularly high association
with CVA as well as heart disease, while individuals whose weight is
distributed around the hips are less at risk (Lavie, Milani, & Ventura, 2009).
A sedentary lifestyle is associated with rising levels of obesity and has been
implicated in occurrence of CVAs, as well as hypertension (Bhat et al.,
2008).
Hypertension—Called the “silent disease,” hypertension has long been
acknowledged as the most significant controllable risk factor for both CVA
and heart attack (Bhat et al., 2008). Hypertension occurs in 25% to 40% of
the US population (Bhat et al., 2008). Symptoms of hypertension are not
clearly identifiable: an occasional headache, dizziness, or light-headedness,
which may indicate hypertension, can easily be attributed to other factors.
Chronically elevated blood pressure exerts pressure on cerebral vessels,
often resulting in lacunar infarctions or intracerebral hemorrhage
(Amarenco et al., 2009; Ohira et al., 2006). Hypertension also has been
implicated in the atherosclerotic process because it drives fatty substances
into the arterial walls, making them brittle, narrowed, and hardened
(Zazulia, 2009). The impact of hypertension increases with age (Bhat et al.,
2008).
Smoking—Smoking doubles the risk of CVA (Goldstein et al., 2011).
Quitting smoking reduces the likelihood of CVA, and there is some
evidence to suggest that 5 years after quitting smoking, individuals lower
their risk of having a CVA to nearly that of people of whom have never
smoked (Basu, Glantz, Bitton, & Millett, 2013).
TIAs—A TIA is considered an important risk factor for an impending CVA.
Survivors of a TIA represent a population at increased risk of subsequent
stroke (Furie et al., 2011). Approximately 30% of all patients who have had
a TIA are at risk of having a CVA within 2 years.
Geographic location—The highest death rates from CVAs in the United
States are in North and South Carolina, Georgia, Northern Florida,
Alabama, Mississippi, and Tennessee. Specific pockets of high death rates
in Texas, Oklahoma, and all of the Hawaiian Islands have been noted
(Voeks et al., 2008). This geographical strip, often termed the “stroke belt,”
is the source of numerous studies on environmental, cultural, or other
geographically determined risk factors. A person who grows up in a high-
risk area and then moves to a lower-risk area as an adult continues to carry
the greater likelihood of having a CVA. This has led to speculation that the
causes may be diet related, cultural, or possibly even related to water supply
or altitude (Glymour, Avendaño, & Berkman, 2007).
Diabetes mellitus—This disease is more common in CVA patients than in a
normal population of similar age (Sarwar et al., 2010). Duration of diabetes
is independently associated with ischemic stroke risk adjusting for risk
factors. The risk increases 3% each year and triples with diabetes ≥10 years
(Banerjee et al., 2012).
Oral contraceptives—Women who have taken birth control pills, especially
those with a high estrogen content, have an increased risk for CVA as they
become older. Current use of combined oral contraceptive pills is associated
with increased odds of venous thromboembolism and ischemic stroke, but
not hemorrhagic stroke or myocardial infarction (Urrutia et al., 2013).
Hyperlipidemia—Hyperlipidemia, including elevated triglycerides and LDL
cholesterol, has long been suspected to be a risk factor for CVA (Furie et al.,
2011).
AF is increasing in incidence in the United States and its presence increases
the risk of developing CVA (Nichols, 2014).
Asymptomatic carotid bruits—Bruit is an abnormal sound or murmur heard
when a stethoscope is placed over the carotid artery. This slushing noise
indicates turbulent blood, often caused by a significant degree of stenosis.
Carotid bruit clearly indicates increased CVA risk. Complete occlusion of
the carotid artery sometimes follows, resulting in a CVA (Bhat et al., 2008).
Prior CVA—Approximately one-quarter of the 795,000 CVAs that occur
each year are secondary incidents (Furie et al., 2011).
Heart disease—Diseased heart (whether it be chronic disease, acute heart
attacks, or prosthetic heart valves) increases the risk of CVA. Independent
of hypertension, people with heart disease have more than twice the risk of
CVA than do people with normally functioning hearts (Ohira et al., 2006).
AF, a condition in which the heart produces an irregular rhythm, is also a
known risk factor for CVA. Both persistent and paroxysmal AFs are strong
predictors of first as well as recurrent CVA. In the United States, 75,000
cases of CVA per year are attributed to AF (Furie et al., 2011).
Infections and inflammation—Inflammation occurring with various
infections, often mild, has been associated with CVA. Studies examining
the relationship between periodontal disease and CVA are inconclusive
(Lockhart et al., 2012). Research is under way to determine whether other
infections that produce arterial inflammation can lead to CVA or heart
disease. For example, chronic infection with Chlamydia pneumoniae, which
causes mild pneumonia in adults, has been linked with higher risk for CVA
(Elkind et al., 2006a).
Alcohol and drug abuse—Numerous studies indicate that moderate
consumption of alcohol decreases the risk of CVA and heart disease;
however, excessive consumption and total abstinence are associated with
higher risk (Elkind et al., 2006b). Studies have demonstrated an association
between alcohol and ischemic stroke, ranging from a definite independent
effect to no effect (Furie et al., 2011). Cocaine and methamphetamine abuse
are major factors in the incidence of CVA in young adults (Westover,
McBride, & Haley, 2007). Use of anabolic steroids for bodybuilding is also
associated with increased risk of CVA (Santamarina, Besocke, Romano,
Loli, & Gonorazky, 2008).
Sleep apnea—Sleep apnea is a common disorder in which the throat
becomes obstructed during sleep, interfering with normal breathing and
sleep. Sleep apnea is worsened by obesity and may contribute to the
narrowing of the carotid artery. Obstructive sleep apnea impacts 15 million
American adults and is most commonly associated with CVA and
cardiovascular disorders including hypertension and arrhythmia (Somers et
al., 2008) (Fig. 17.4).
Figure 17.4 Left and right hemiplegia. (From Wilkins, E. M. (2011). Clinical
practice of the dental hygienist. Philadelphia, PA: Lippincott Williams &
Wilkins.)
CVA is the fourth leading cause of death in the United States, killing nearly
130,000 Americans each year or 1 of every 20 deaths (CDC, 2015; Towfighi &
Saver, 2011), surpassed only by heart diseases, cancer, chronic lower respiratory
disease, and accidents (Kochanek, Murphy, Xu, & Arias, 2014). The most
common cause of disability, CVA is the most widespread diagnosis among
clients seen by occupational therapists for the treatment of physically disabled
adults (Legg et al., 2007). An estimated 600,000 to 730,000 people in the United
States suffer an episode each year, and about 4.5 million stroke survivors are
alive today (Xie et al., 2006). Approximately 50% to 70% of people regain
functional independence after a CVA; however, 15% to 30% of those who
survive an ischemic or hemorrhagic stroke suffer some permanent disability (Xie
et al., 2006). Of the two main types, hemorrhagic strokes occur much less
frequently (20% of all strokes) than do ischemic strokes, which account for
about 80% of all strokes (Xie et al., 2006). Depression affects approximately
one-third of stroke survivors (Hackett, Yapa, Parag, & Anderson, 2005;
Townend, Tinson, Kwan, & Sharpe, 2010). Impact on family and caregivers is
also enormous. If adequate social services and support are not available,
caregivers assume the burden for functional tasks that the patient is unable to
perform. Numerous studies identified caregiver mental health and the amount of
time and effort necessary of the caregiver as significant determinants of
caregiver burden (Rigby, Gubitz, & Phillips, 2009).
Despite these grim statistics, there is some good news. With the exception of
subarachnoid hemorrhage, between 1950 and 1990, all types of CVA have shown
a significantly decreased incidence (Chiuve et al., 2008). This may be partly the
result of increased control of risk factors such as hypertension, diabetes mellitus,
and heart disease (Chiuve et al., 2008). Individuals who have had a CVA now
live almost twice as long; interestingly, one study even reported that long-term
survivors appeared to be less depressed than did the comparison group (Patel,
McKevitt, Lawrence, Rudd, & Wolfe, 2007). Fewer people now die of CVA,
notwithstanding the continuing debate as to whether this decline is the result of
the occurrence of fewer CVAs or better medical treatment (Carandang et al.,
2006). However, it should be noted that the absolute number of CVAs occurring
in the United States is rising, probably a result of the aging of the population
(Carandang et al., 2006). Moreover, dramatic increases in obesity, among
children as well as adults, offer grave cause for concern, given the close links
determined between obesity and vascular disease (Grysiewicz et al., 2008;
Lawlor & Leon, 2005).
Pediatric Stroke
CVA in young children occurs in about 1.2 to 13 cases per 100,000 children per
year as compared to 240 cases per 100,000 in the adult population (Grysiewicz
et al., 2008; Tsze & Valente, 2011). It is more frequent in children younger than
2 years of age, as CVA is 17 times more common in the perinatal period than
later in childhood and beyond (Nelson, 2007). Childhood CVA incidence is 5 to
8 per 100,000 children annually with approximately 50% ischemic including
arterial ischemic stroke (AIS) or cerebral sinovenous thrombosis (CSVT)
(Kitchen et al., 2012). The effects of CVA among children are similar to those
described in adults. Sickle cell disease, which affects blood-clotting
mechanisms, and genetic disorders are the next most common known causes.
Additional risk factors for pediatric stroke include infection, vascular, vasculitis,
oncologic, trauma, and drugs (Tsze & Valente, 2011). Residual neurological
deficits, which extend into adulthood, are reported in 50% to 90% of children
and include motor, language, and cognitive deficits (Kitchen et al., 2012) (Fig.
17.5).
figure 17.5 Pediatric stroke CT and MRI of a 2-year-old girl who awoke with
left hemiparesis and right-gaze preference. A. A wedge-shaped area of
hypodensity is seen on axial CT. MRI shows (B) T2 hyperintensity and (C)
corresponding diffusion restriction, indicative of an ischemic stroke in the
distribution of the right middle cerebral artery. (From Kline, A. M., & Haut, C.
(2015). Lippincott certification review: Pediatric acute care nurse practitioner.
Signs and Symptoms
Neurological Effects of CVA
An occlusion that causes a serious CVA can occur anywhere in the extracranial
or intracranial system, but the most common site is in the distribution of the
middle cerebral artery and its branches in the cerebrum. The majority of cerebral
CVAs occur in the left hemisphere compared to right hemispheric (Hedna et al.,
2013). It is important to note that even in individuals with the same neurological
deficit, the impact of disability is different, depending on the individual’s life
situation.
Stroke Warning Signs

To educate the public, the American Heart Association and National Stroke
Association distribute pamphlets listing the warning signs of an impending
serious CVA (American Heart Association [AHA], 2010). Public knowledge of
the FAST (Face drooping, Arm weakness, Speech difficulty, Time to call 911)
CVA warning signs awareness campaign is effective (Robinson, Reid, Haunton,
Wilson, & Naylor, 2012). These include
Sudden numbness or weakness of the face, arm, or leg; especially on one

side of the body
Sudden confusion, trouble speaking, or understanding
Sudden trouble seeing in one or both eyes
Sudden trouble walking, dizziness, and loss of balance or coordination
Sudden severe headache with no known cause
Some general medical symptoms related to type of CVA were discussed under
Etiology. Signs and symptoms also depend on the size and location of the injury,
and neurologists can often predict location by the symptoms the individual
displays. However, it is important to remember that a CVA is complex, and each
individual may experience a unique constellation of symptoms. Relying on
stereotypical models of CVA leads to generalized and often inappropriate
therapy.
Left-Sided Cerebral Injuries: Middle Cerebral Artery
The left cerebral hemisphere controls most functions on the right side of the
body because of the decussation of motor fibers (decussation of the pyramids) in
the medulla. These fibers that cross, or decussate to the opposite side, form the
lateral corticospinal tract. The rest of the fibers descend ipsilaterally, forming the
anterior corticospinal tract (Fujimura, Kaneta, Mugikura, Shimizu, & Tominaga,
2007). The proportion of crossing fibers varies from person to person, averaging
about 85% (Fujimura et al., 2007).
A CVA in the region of the middle cerebral artery in the left cerebral
hemisphere may produce the following symptoms:
1. Loss of voluntary movement and coordination on the right side of the face,
trunk, and extremities.
2. Impaired sensation, including temperature discrimination, pain, and
proprioception on the right side (hemianesthesia).
3. Language deficits, called aphasia, in which the patient may be unable to
speak or understand speech, writing, or gestures. The breakdown of
language function is complex; the many types of aphasia will be discussed
later in this chapter.
4. Problems with articulation of speech because of disturbances in muscle
control of the lips, mouth, tongue, and vocal cords (dysarthria).
5. Blind spots in the visual field, usually on the right side.
6. Slow and cautious personality.
7. Memory deficits for recent or past events (Boyd & Winstein, 2003).
Right-Sided Cerebral Injuries: Middle Cerebral
Artery
The right cerebral hemisphere controls most of the functions on the left side of
the body and also is responsible for spatial sensation, perception, and judgment.
Injury to the middle cerebral artery of the right cerebral hemisphere may produce
a combination of the following deficits:
1. Weakness (hemiparesis) or paralysis (hemiplegia) on the left side of the
body (face, arm, trunk, and leg) 2. Impairment of sensation (touch, pain,
temperature, and proprioception) on the left side of the body 3. Spatial and
perceptual deficits
4. Unilateral inattention (neglect), in which the patient neglects the left side
of the body and/or the left side of the environment 5. Dressing apraxia, in
which the patient is unable to relate the articles of clothes to the body
(Suzuki et al., 2006) 6. Defective vision in the left halves of visual fields or
left homonymous hemianopsia in which there is defective vision in each
eye (the temporal half of the left eye and the nasal half of the right eye) 7.
Impulsive behavior, quick and imprecise movements, and errors of
judgment (Suzuki et al., 2006)
Anterior Cerebral Artery Stroke
The territory of the anterior cerebral artery is rarely infarcted because of the side-
to-side communication provided by the anterior communicating artery in the
circle of Willis (Young, 2009). Symptoms of an anterior cerebral artery CVA
include
1. Paralysis of the lower extremity, usually more severe than that of the upper
extremity, contralateral to the occluded vessel 2. Loss of sensation in the
contralateral toes, foot, and leg 3. Loss of conscious control of bowel or
bladder
4. Balance problems in sitting, standing, and walking
5. Lack of spontaneity of emotion, whispered speech, or loss of all
communication 6. Memory impairment (Thompson & Morgan, 2013)
Vertebrobasilar Stroke
The vertebrobasilar system of arteries supplies blood primarily to the posterior
portions of the brain, including the brainstem, cerebellum, thalamus, and parts of
the occipital and temporal lobes. This posterior circulation is not divided into
right and left halves, as in the anterior circulation (Coward et al., 2007;
Schneider & Olshaker, 2012). An occlusion here might produce
1. A variety of visual disturbances, including impaired coordination of the
eyes 2. Impaired temperature sensation
3. Impaired ability to read and/or name objects
4. Vertigo, dizziness
5. Disturbances in balance when standing or walking (ataxia) 6. Paralysis of
the face, limbs, or tongue
7. Clumsy movements of the hands
8. Difficulty judging distance when trying to coordinate limb movements
(dysmetria) 9. Drooling and difficulty swallowing (dysphagia) 10.
Localized numbness
11. Loss of memory (Boyd & Winstein, 2003) 12. Drop attacks in which there
is a sudden loss of motor and postural control resulting in collapse, but the
individual remains conscious (Parry et al., 2009).
TIAs in this area are common in the elderly. The vertebral arteries travel up to
the brainstem through a bony channel in the cervical vertebrae. In older adults,
osteoarthritis may develop in the cervical bones, causing narrowing of the
cervical canal, especially when the head is extended or rotated (Parry et al.,
2009).
Wallenberg’s Syndrome
Wallenberg’s syndrome is a classic brainstem stroke that also is referred to as
lateral medullary syndrome (Chen, Khor, Chen, & Huang, 2011). It occurs as the
result of an occlusion of a vertebral or cerebellar artery. CVAs in this area may
produce contralateral pain and temperature loss, ipsilateral Horner’s syndrome
(sinking of the eyeball, ptosis of the upper eyelid, and a dry, cool face on the
affected side), ataxia, and facial sensory loss. Ischemia to the ipsilateral cranial
nerve fibers VIII, IX, and X results in palatal paralysis, hoarseness, dysphagia,
and vertigo with no significant weakness (Raymond & Louis, 2015). Brainstem
strokes often result in coma because of damage to the centers involved with
alertness and wakefulness (reticular system) (Young, Aminoff, Hockberger, &
Wilterdink, 2012). A hemorrhage into the brainstem area is rare, quickly
accompanied by loss of consciousness, and usually fatal. Among patients who
survive brainstem stroke, however, recovery is often good (Young et al., 2012).
Other Complications of CVA
Secondary conditions may occur in addition to these deficits. These are
important manifestations of the patient’s recovery and rehabilitation and may
actually be more disabling than the CVA itself (Creutzfeldt, Holloway, & Walker,
2012). Awareness is key to reducing the risk of the following complications.
Seizures
Seizures are among the most common neurological sequelae of CVA. About
10% of all individuals who experience a CVA experience seizures, from CVA
onset until several years later (Silverman, Restrepo, & Mathews, 2002). Brain
scars that result from CVA may irritate the cortex and cause a spontaneous
discharge of nerve impulses that may generalize to a full grand mal convulsion
(Leone et al., 2009). Seizures develop in up to 10% of patients with CVA and are
more common with embolic than thrombotic infarcts (Leone et al., 2009).
Anticonvulsant drugs are sometimes used in patients with early seizures, but
their use is controversial (De Reuck, Hemelsoet, & Van Maele, 2007).
Infection
Alteration of swallowing function, aspiration, hypoventilation, and immobility in
the patient with CVA often lead to pneumonia (Prass, Braun, Dirnagl, Meisel, &
Meisel, 2006). Changes in bladder function may lead to bladder distention and
urinary tract infection (Westendorp, Nederkoorn, Vermeij, Dijkgraaf, & van de
Beek, 2011). Impaired sensation and inadequate position changes may result in
pressure sores (decubitus) and consequent infection of these areas (Turhan,
Atalay, & Atabek, 2006).
Thromboembolism
Immobility of the legs and prolonged bed rest often lead to thrombosis of
dependent leg veins (MacDougall, Feliu, Boccuzzi, & Lin, 2006). In deep vein
thrombosis (DVT), local pain and tenderness may develop in the calf, with
some swelling and a slight increase in temperature. If the thrombosis is confined
to the calf, it may not be serious. However, if the thrombosis spreads up toward
the groin to involve the veins in the pelvis, there is a very real possibility of a
clot breaking off into the bloodstream. The clot will then travel through the right
side of the heart and enter the lungs through the pulmonary arteries, resulting in
sudden collapse and death owing to obstruction of the pulmonary arteries
(MacDougall et al., 2006). Early mobilization of the patient is of utmost
importance in preventing DVT and subsequent pulmonary embolism.
In addition to the above list, it is important to be aware that symptoms
resulting from a partial reduction or temporary change in the blood flow to the
brain are extremely important warning signs for CVA (Fogle et al., 2008).
Several of these conditions are discussed below.
Transient Ischemic Attacks
TIAs result from a temporary blockage of the blood supply to the brain. The
symptoms occur rapidly and last for <24 hours. Seventy-five percent of TIAs
last <5 minutes. The specific signs and symptoms depend on the portion of the
brain affected but may include fleeting blindness in one eye, hemiparesis,
hemiplegia, aphasia, dizziness, double vision, and staggering. Carotid artery
disease and vertebral basilar artery disease may lead to TIAs (Dharmasaroja &
Intharakham, 2010). The main distinction between TIAs and CVA is the short
duration of the symptoms and the lack of permanent neurological damage. Ten to
fifteen percent of clients who experience a TIA will have a CVA within 3
months, with half occurring within 48 hours (Easton et al., 2009; Giles &
Rothwell, 2007; Wu et al., 2007). Without preventive treatment, a third of those
who suffer TIAs will go on to have a CVA within 5 years (Mattace-Raso et al.,
2006). Thus, it is crucial to detect the cause of a TIA and begin appropriate
intervention promptly (Sehatzadeh, 2015). The long-term impact of TIAs have
been found to be more debilitating (Croot et al., 2014; Moran et al., 2013).
Residual problems include anxiety, depression, and fatigue, which need to be
addressed before they become disabling. Rehabilitation can play a significant
role in decreasing the impact of this type of neurological event (Heron, Kee,
Donnelly, & Cupples, 2015).
Small Strokes
In some cases, the symptoms of a TIA may last longer than 24 hours. If they last
a day or more and then completely resolve, or if they leave only minor
neurological deficits, they are called small strokes, or lengthy TIAs (Easton et
al., 2009). Often, the remaining neurological deficits are barely noticeable. Like
TIAs, however, these small strokes are important warning signs that a more
serious CVA may occur (Fogle et al., 2008). A small stroke that completely
resolves is called a reversible ischemic neurological deficit (RIND). An episode
that lasts more than 72 hours and leaves some minor neurological impairments is
called a partially reversible ischemic neurologic deficit (PRIND). The
mechanism of injury in RIND and PRIND is the same as that for a CVA or TIA.
Like ischemic strokes, RINDs typically occur in the morning; since blood
pressure is low during sleep, sudden increases in blood pressure upon arising
may cause problems (Metoki et al., 2006).
Many small strokes are not reported to a medical practitioner, which makes
the exact frequency of occurrence of these strokes difficult to determine. It is
important to recognize the symptoms of a small stroke so that it can be treated
early, reducing the risk of more permanent injury.
Subclavian Steal Syndrome
This is a rare condition caused by a narrowing of the subclavian artery that runs
under the clavicle. Symptoms occur when the arm on the side of the narrowed
vessel is exercised. Usually, movement of the arm produces light-headedness,
numbness, and weakness. Other neurological symptoms also may be present. In
this syndrome, blood is “stolen” from the brain and instead is delivered to the
exercised arm. It is a warning sign that advanced atherosclerosis may be present
in the arteries throughout the body, including the cerebral arteries (McIntyre,
2009).
CVAs result in anoxic damage to nervous tissue that causes various neurological
deficits, depending on where the blood supply was lost. If neuronal cell death
occurs, it is considered irreparable and permanent, as no way has yet been found
to regenerate nerve cells (Yuan, Lipinski, & Degterev, 2003). However, the
nervous system has a high level of plasticity, especially during early
development, and individual differences in neural connections and learned
behaviors play a major role in functional recovery. No two brains can be
expected to be structurally or functionally identical (Carmichael, 2006).
Spontaneous recovery may occur as edema subsides or viable neurons reactivate.
Recovery also may occur with physiologic reorganization of neural connections
or developmental strategies. Reorganization of surviving central nervous system
structures facilitates behavioral recovery, such as changes in interhemispheric
lateralization, activity of association cortices linked to injured zones, and the
organization of cortical representational maps (Hara et al., 2015). Any injury
brings different factors into play, affecting axonal and dendritic sprouting or
collateral rearrangement, synaptic formation, the excitability of neurons,
“substitution of parallel channels,” and “mobilization of redundant capacity”
(Huang, Shen, & Duong, 2010). Recovery from neurological deficits thus
depends on the etiology and size of the infarct (Carmichael, 2006).
Approximately 90% of neurological recovery occurs within 3 months, with the
rest occurring over a more extended time (Fonarow et al., 2014). It should be
noted that recovery from hemorrhagic strokes proceeds more slowly, however
(Steiner et al., 2011).
Accuracy in the prediction of function or rate of return is difficult because of
individual variability of anatomy and extent of brain damage, as well as
differences in types of CVA, learning ability, premorbid personality and
intelligence, and motivation (Fonarow et al., 2014). Generally, the prognosis for
recovery of function is greater in young clients, possibly because the young
brain has more plasticity or because the young are generally in better physical
condition.
Secondary complications are important to recovery and rehabilitation as the
occurrence of these complications may impact CVA outcomes (Al-Khaled &
Eggers, 2013). These complications are discussed in this chapter and include
depression, seizures, infection, bowel/bladder incontinence, thromboembolism,
shoulder subluxation, painful shoulder, shoulder-hand syndrome, abnormal
muscle tone, and associated reactions and movements.
Individuals with good sensation, minimal spasticity, some selective motor
control, and no fixed contractures seem to make the greatest improvements in
functional abilities. If an individual has no concept of the affected side and
cannot localize stimuli to the affected side, or if he or she has fecal or urinary
incontinence, the outlook for independence is generally poor. A small percentage
of clients may experience additional CVAs, which affect their recovery outcomes
(Bhalla & Birns, 2015).
Long-term complications are associated with CVA (Kuptniratsaikul,
Kovindha, Suethanapornkul, Manimmanakorn, & Archongka, 2013). It has been
estimated that three-quarters of all clients affected by CVA will have at least one
physical or cognitive issue within 1 year of the diagnosis. Different diagnostic
conditions, which have been documented, involve musculoskeletal issues
including pain, spasticity, shoulder subluxation, and contractures as well as
psychological disorders (Kuptniratsaikul et al., 2013).
Diagnosis
The diagnosis of CVA requires knowledge of the incidence of the different types
of stroke and awareness of the presence of the complicating factors mentioned
above. Symptoms must be carefully noted from the patient or, if the patient is too
ill, frightened, or confused, from the family (Hand, Kwan, Lindley, Dennis, &
Wardlaw, 2006). Neurologists, neurosurgeons, and some internists are the
specialists usually involved in this acute diagnostic phase of treatment (Hand et
al., 2006). A number of diagnostic techniques are used to distinguish CVA from
other potential causes of observed symptoms and to assist in determining the
location of lesions (Sacco et al., 2013).
The physical examination of the client with a suspected CVA or TIA
includes a search for possible cardiac sources of emboli by listening to the heart
and arteries of the neck. Also useful in the determination of cardiac-source
emboli are electrocardiography (ECG), echocardiography, and monitoring for
arrhythmias. In addition to cardiac testing, various neuroimaging techniques (see
below) and analysis of blood and CSF may be performed (Feldmann et al.,
2007). A neurological examination assists in determining the neurological
disability and usually includes evaluation of higher cortical function (memory
and language), level of alertness, reflexes, visual and oculomotor system,
behavior, and gait (Feldmann et al., 2007). Other diagnostic methods include
noninvasive studies of blood vessels and invasive techniques requiring injection
of dye into the arterial system, for example.
Neuroimaging Techniques
CT and magnetic resonance imaging (MRI) are invaluable noninvasive tools that
depict pathological changes in the brain in clients with CVA (Tarpley, Frank,
Tansy, & Liebeskind, 2013). One or the other of these is almost always used at
some point for every client with a suspected CVA. CT and MRI are also capable
of showing zones of edema and the shifting of intracranial material (Kidwell &
Wintermark, 2010). Negative results of these tests may indicate that the ischemia
is reversible (Feldmann et al., 2007). CT scans and MRI are the most reliable
tools for determining intracranial hemorrhages and infarctions (Tarpley et al.,
2013).
Computed Tomography
CT is a type of radiographic examination that is widely used for analysis of
cerebral injury (Kidwell & Wintermark, 2010). CT scans are employed to
differentiate between a hemorrhagic stroke and an ischemic stroke (Kidwell &
Wintermark, 2010). While they are useful in clarifying the location and the
mechanism and severity of CVA, they are not particularly sensitive to subtle
ischemic changes such as lacunar strokes and microbleeds (Kidwell &
Wintermark, 2010). It is the most common tool used in the diagnosis of CVA
caused by hemorrhage, but research is finding that MRI is more effective
(Kidwell & Wintermark, 2010). It can be diagnostic in clients with TIAs, but
MRI is the preferred method of evaluation if available (Sidorov, Feng, & Selim,
2014).
Magnetic Resonance Imaging

MRI is more sensitive than a CT scan and does not expose the client to radiation.
It provides detailed pictures of the brain by using a magnetic field. MRI provides
better detection for ischemic areas than does a CT scan as well as distinguishes
whether a hemorrhage is new or chronic (Arsava, 2012). MRI can provide
information about all aspects that are being affected when the CVA is detected
from tissue health to the circulatory system of the brain, which in turn can help
determine the best CVA treatment for the client (Arsava, 2012). Research also
shows that MRI may assist in determining the candidacy of the patient for CVA
treatment when the length of time the CVA has been occurring is unknown
(Tarpley et al., 2013).
Positron Emission Tomography (PET scan)
Positron emission tomography, or PET scan, is being used experimentally. This
scan shows how the brain uses oxygen and glucose, which are the main energy
elements of the brain, and can indicate the effects of CVA on the brain
(Bunevicius, Yuan, & Lin, 2013). Currently, PET scans are seldom used in the
management of acute CVA, but research exists supporting the use of PET as
another tool in assessing CVA progression to determine management
(Bunevicius et al., 2013). The use of PET/MRI together has been found to be
particularly sensitive and more precise in guiding treatment decisions (Werner et
al., 2015).
Noninvasive Study of Blood Vessels

Noninvasive procedures used to evaluate both extracranial and intracranial blood
flow include duplex ultrasonography as well as color-flow and transcranial
Doppler ultrasound. These techniques can localize and determine the
approximate size of the lesions within the arteries (Aaslid, Huber, & Nornes,
2010).
Duplex ultrasonography is useful in detecting the presence and severity of
disease in the common and internal carotid arteries and in the subclavian and
vertebral arteries in the neck. This scan can reliably differentiate between minor
plaque disease, stenosis, and occlusive lesions. It is an excellent method of
monitoring the progression or regression of atherosclerotic disease in the neck
(Vassileva, Daskalov, & Stamenova, 2015).
Color-flow Doppler ultrasound is used because it is effective in showing
lesions of the carotid and vertebral arteries (Nasr, Ssi-Yan-Kai, Guidolin,
Bonneville, & Larrue, 2013). Transcranial Doppler ultrasound gives information
about pressure and flow in the intracranial arteries (Dwedar et al., 2014). A
recent study found that use of this type of ultrasound may play a therapeutic role
in restoring blood flow velocity even before the use of thrombolytic therapy
(tPA) and, due to the noninvasive nature provided, it may be another viable
method to utilize (Dwedar et al., 2014).
Invasive Techniques
Cerebral angiography involves radiography of the vascular system of the brain
after injecting a dye or other contrast medium into the arterial blood system.
Computer-generated images are then produced that can show the entire visible
length of cerebral arteries, as well as, the nature, location, and extent of
pathological changes. This technique is now safer than before (<1% incidence of
mortality and serious morbidity); however, it is recommended when noninvasive
techniques have failed to yield a conclusive diagnosis or when surgery is being
planned or considered (Kaufmann et al., 2007). The use of angiography has been
found to be effective when decisions about the best therapeutic method are
required in terms of delivering thrombolytic agents intravenously or intra-
arterially (Porelli et al., 2013).
Analysis of CSF can be helpful in determining the severity and functional
outcome of an acute ischemic stroke within the first few hours by examining the
levels of cytokines and nitrates present (Beridze, Sanikidze, Shakarishvili,
Intskirveli, & Bornstein, 2011). These elements are associated with
inflammatory damage, which can occur from the ischemia.
Other Diagnostic Techniques
ECG is another diagnostic technique, which can be used due to the high
incidence of heart disease in clients with CVA (Salmani, Prarthana, Bandelkar, &
Varghese, 2014). With acute stroke patients, changes are noted with ECG
recordings, and different wave patterns may be indicative of mortality rates
(Salmani et al., 2014); however, more research needs to be performed. Other
diagnostic techniques used for CVA include electroencephalography (EEG),
single photon emission tomography (SPET), and other special cardiac and
coagulation tests that are useful in detecting unusual heart and blood disorders
that can bring on a CVA (Petrella, Coleman, & Doraiswamy, 2003). After the
type of CVA has been determined based on the diagnostic tools used, further
management may be indicated in the form of medical or surgery. If not, a
neurologist will evaluate the brain-damaged person’s ability to function.
Rehabilitation often will begin at that point to return the client to the highest
possible level of independent functioning (Rodríguez-Mutuberría et al., 2011).
At present, the treatment of acute CVA is limited to management of the results of
the primary event and preventive measures against further injury or occurrence
(Zhao et al., 2009). The most effective management of CVAs is constantly
evolving as new drugs, imaging techniques, and surgical interventions are being
developed (Sacks et al., 2013). Before the CVA can be treated, it must be
accurately identified as either cerebral infarction or cerebral hemorrhage, since
interventions that are beneficial with one type of CVA may be potentially
dangerous to the other (Tansy et al., 2015). Therefore, careful and exact
diagnosis must be made first. Drug management of CVA is constantly evolving.
Common categories of drugs used to minimize the damage of cerebral infarction
are described in the following sections.
Antiplatelet Therapy
Aspirin is often prescribed to patients when the vascular lesion is not severely
stenotic and the individual has not had a CVA (Bégot et al., 2013). Use of two
antiplatelets including aspirin reduces the possibility of a CVA in those who have
had a previous CVA or TIA, as well as those who are at high risk for CVA more
significantly than does just aspirin use in the short term (Tan et al., 2015). But
long-term efficacy of dual use of antiplatelets has been found to be detrimental
(Xie, Zheng, Zhong, & Song, 2015). There is an additional concern with
increased risk of hemorrhage so the benefits must be examined to ensure they
outweigh the risk (Tan et al., 2015). It also benefits clients who have had a mild
CVA but is not as effective in those with moderate or severe CVAs (Sacco et al.,
2006a, 2006b). Aspirin is clearly not suitable for clients with cerebral
hemorrhage or those at risk of bleeding. Aspirin is relatively safe and
inexpensive. Studies have looked at the efficacy of aspirin use in low doses as a
preemptive therapy and found a benefit in the prevention of cardiovascular
diseases (Stegeman, Bossuyt, Yu, Boyd, & Puhan, 2015).
Anticoagulants
Anticoagulants inhibit clotting by interfering with the activity of chemicals in
the liquid portion of blood that are essential for the coagulation process (Mant et
al., 2007). Short-term (2 to 3 weeks) heparin therapy is prescribed for clients
with complete blockages of large arteries, as heparin is effective in preventing
the formation of emboli (Diener et al., 2006). For longer-term treatment (1 to 3
months), the drug warfarin may be used to prevent blockages in areas that cannot
be treated by surgery (Mant et al., 2007).
Thrombolytics (tPA)
Thrombolytic therapy (tPA), used for dissolution of an occluding thrombus, is
frequently applied in the acute treatment of myocardial infarction as well as in
the treatment of CVA. It is effective before extensive brain infarction has
occurred, so it is only appropriate in CVA patients whose arterial damage has
been identified early (Demchuk & Bal, 2012). The potential benefit must be
weighed against reperfusion damage, a bleeding tendency, and the possibility of
reocclusion. The Food and Drug Administration (FDA) has approved the use of
tPA for treatment of acute CVA within 3 hours of onset (Demchuk & Bal, 2012).
However, studies are researching whether administration of thrombolytics
beyond the 3-hour limit will still produce favorable outcomes in CVA recovery
(Emberson et al., 2014). At this time, there is an indication that clients will still
improve in their functional outcomes after receiving thrombolytics up to 5 hours
after initial CVA symptoms appeared (Emberson et al., 2014). Much more
research needs to occur in regard to the time of administration as well as
methods of administration. Another area of recent interest is determining the
location of the infarct to administer thrombolytics either intravenously or intra-
arterially, which can affect how well a client recovers (Berkhemer et al., 2015).
Also, newer research is examining the efficacy of moving from using time limits
to analyzing the health of brain tissue in determining the administration of
thrombolytics (Demchuk & Bal, 2012; Leiva-Salinas et al., 2013).
Surgical Interventions
In some cases, surgical treatment may be the best choice for the client. The
neurosurgeon must carefully consider many factors before surgery is performed,
including the client’s overall health and life expectancy. Carotid endarterectomy
(CEA) was among the most commonly performed vascular surgeries in the
United States, but now, carotid artery stenting (CAS) is used more often due to
the lower invasive properties of the procedure (Al- Damluji, Nagpal, Stilp,
Remetz, & Mena, 2013). During the CEA procedure, the diseased vessel is
opened, the clot is removed, and an artificial graft is put in place (Mendonça et
al., 2014). During CAS, a stent, which is a narrow tube, is inserted in the carotid
to expand the artery and, in turn, increase the blood flow. CEA has become a less
common treatment option for patients with moderate to severe stenosis of the
carotid artery due to the common use of statins in the United States (Al-Damluji
et al., 2013). Statins are a preemptive medication used to lower blood
cholesterol, which decreases the risk of a CVA occurring.
Subarachnoid hemorrhages are often caused by ruptured aneurysms or
AVMs. Surgical clipping or coiling is the most effective treatment of these
anomalies. If the patient survives the initial bleeding, the goal of surgery is to
correct the problem before bleeding recurs. If surgery is performed, there is an
increased probability of a successful outcome for the client (Garbossa et al.,
2012). In intracerebral hemorrhage, small hematomas usually resolve
spontaneously, but the chance of a recurrence is greatly increased (Chou et al.,
2012). Surgery of these smaller hematomas has been investigated, but there are
increased risks and more research needs to be performed (Kim & Ko, 2014).
Clipping versus coiling can affect the patient cognitive outcomes (Latimer,
Wilson, McCuskey, Caldwell, & Rennie, 2013). Endovascular coiling appears to
affect the patient less cognitively than those patients who underwent
neurosurgical clipping and demonstrated more global deficits.
Large hematomas, however, often produce death. Some lesions may expand,
causing gradually increasing neurological signs. These expanding lesions can be
drained surgically if they are near the surface of the brain, especially in the
cerebral or cerebellar white matter. Generally, hemorrhages are evacuated only if
they are large and life threatening or when surgery is necessary to treat an
aneurysm, tumor, or AVM, but of those who experience this type of bleed, 66%
do not undergo surgery (Hobson et al., 2014).
Superficial temporal artery bypass is a new, more delicate surgical therapy
for preventing future CVAs (Chou et al., 2012). The procedure begins with
craniotomy to expose the brain; then a scalp artery is connected to an intracranial
artery microsurgically. This operation is extremely challenging to perform and is
thus performed less frequently than CEA. Surgeons who do the procedure,
however, are enthusiastic about the results and claim that it revascularizes the
brain better than does endarterectomy, and studies have determined that there is a
0% chance of a recurrent CVA (Chou et al., 2012).
Treatment of Secondary Effects
Specific pathophysiologic sequelae, or outcomes, follow the occurrence of any
type of CVA. Treating these secondary effects is crucial to medical and
functional recovery. Two of these are cerebral edema and ischemia. Oxygen
therapy to reduce hypoxia, vasodilation to improve blood flow through ischemic
areas, therapeutic hypertension, and hemodilution therapy are some of the
treatments used for ischemia. Hemodilution results in a significant rise in
cerebral blood flow and increased oxygen transfer (Vilela & Newell, 2008).
Edema often complicates ischemic strokes and must be controlled, because
most deaths during the first week after a massive CVA are caused by extensive
cerebral edema and increased intracranial pressure (Treggiari, Walder, Suter, &
Romand, 2003). This pressure can displace the cerebrum downward and
interfere with the functioning of the midbrain and lower brainstem, which
control such basic vital functions as respiration and heart action (Treggiari et al.,
2003).
Corticosteroid therapy can cause a significant reduction in interstitial
cerebral edema, reducing swelling and improving outcomes after CVA (Thomas
et al., 2007). However, a review of the literature indicates that there is no clear
basis for evaluating the effect of corticosteroid treatment for people with acute
ischemic strokes (Veltkamp et al., 2005).
The Occupational Therapy Practice Framework (OTPF) 3rd edition includes
client factors, which are particular capacities, characteristics, or beliefs that
define a person and influence performance in occupations (American
Occupational Therapy Association [AOTA], 2014). The impact of CVA on an
individual is unique and may affect client factors. Deficits in sensory, motor,
mental, communication, and emotional client factors may be compounded by
secondary complications, such as infection or depression. Together, these
significantly impact an individual’s performance in all areas of occupation:
activities of daily living (both basic and instrumental), work, play, leisure, and
social participation.
Sensory Functions
Sensory functions can be affected at the very basic level of awareness and at the
point of processing and modulation of sensory input. Loss of protective tactile
functions, such as diminished awareness of temperature and pain, is a common
concern for an individual with a CVA. Loss of these functions poses a safety
risk. Individuals with proprioceptive dysfunction may show asymmetrical
posture, have difficulty maintaining balance, appear to forget affected body
parts, be unable to describe position or movement of limbs, and be susceptible to
joint damage (Lima et al., 2015). Individuals with a loss of tactile sensation may
demonstrate a lack of awareness of body parts simply because they forget what
they cannot feel. They are also vulnerable to damage of affected body parts,
particularly skin breakdown. Moreover, diminished tactile function hinders
resumption of motor activities. Depending on the location of the infarct,
individuals may experience diminished vestibular function, which will limit
mobility efficiency and safety. Impaired balance may cause difficulties in
assuming and maintaining a vertical posture and in automatic adjustments to
changes of position and antigravity movement. As a result, individuals
demonstrate an asymmetrical posture at rest, leaning or falling to the hemiplegic
side during mobility, or fail to use normal protective reactions when falling
(Kim, Kim, & Kang, 2015; Mitsutake et al., 2014).
Defects in visual field functions may impair reading, even in the absence of
language dysfunction. For example, clients with right cerebral lesions may find
reading difficult or impossible, because visuospatial deficits hinder tracking or
following the line of print across the page. Clients with homonymous
hemianopsia on either side are unable to respond to people, objects, or the
environment on the affected side. They may bump into objects or be startled by
their sudden appearance. Individuals experiencing visual inattention have
difficulty scanning and shifting their gaze, particularly toward the affected side
(Goodwin, 2014) (Fig. 17.6).
Figure 17.6 Homonymous hemianopsia. (From Timby, B. K., Smith, N. E.
(2013). Introductory medical-surgical nursing (11th ed.). Philadelphia, PA:
Perceptual deficits may be difficult to understand for patient and family alike,
but their impact on the client’s ability to resume independent function may be
profound. Depending on the location of the lesion, or infarct, such deficits
include visual agnosia and visuospatial agnosia—difficulty in understanding the
relationship between objects and between self and objects (Zhang, Kedar, Lynn,
Newman, & Biousse, 2006). Individuals affected in this manner are unable to
find their way in a familiar environment; they cannot trace a route on a map,
cannot pick out objects from a cluttered environment, cannot copy drawings or
simple construction, and may have difficulty in functional (spatial) tasks, such as
dressing and reading a newspaper. A condition where the client “pushes” away
from the nonhemiparetic side is thought to be due to an impairment in the
client’s perception of their vertical orientation (Mansfield et al., 2015). This
behavior could affect participation in occupations by impairing client factors
such as mobility.
Agnosia for sounds may also occur, so that the individual cannot understand
or confuses nonverbal sounds (Jang, Kim, Park, & Lee, 2014; Tabuchi, 2014;
Urbanski, Coubard, & Bourlon, 2014). Another important loss is astereognosis,
which affects functional use of the affected hand whenever vision is occluded:
tasks such as finding keys or coins in a pocket or a glass on a bedside table when
it is dark may be difficult (Murray, Camen, Gonzalez, Bovet, & Clarke, 2006).
The location of the infarct determines which functions are lost and which
remain intact. For example, somatagnosia, in which an individual has no
awareness of his or her own body and its condition, is commonly seen in right
parietal lobe lesions. Deficits that result from right cerebral injuries often cause
unilateral perceptual problems of the left body side and space, such as unilateral
body inattention. Unilateral body inattention (or neglect) is different from
unilateral spatial (or visual) inattention as the first represents a lack of awareness
of one side of the body, usually the left side, and the latter is a visual
unawareness of part of the visual field. Lesions in the left cerebral hemisphere,
however, cause bilateral problems, such as right/left discrimination (Connell,
2008). Impairment of the left parietal lobe results in reduced motor planning
(apraxia), whereby individuals are unable to adjust movement of their own body
parts for intentional movements. Yet impairment of the right parietal lobe causes
an inability to adjust the position of external objects. Frontal lobe lesions may
also result in impaired motor planning, in which sequencing of movement
becomes difficult. Individuals with impaired motor planning may be unable to
carry out a verbal request (for even a simple task such as combing the hair),
although often they can perform such tasks automatically (Jax, Rosa-Leyra, &
Buxbaum, 2014). They may perseverate, that is, persist in purposeless
movement, or they may be unable to complete a required sequence of acts, copy
gestures and drawings, or carry out simple spatial constructional tasks (Beis et
al., 2004) (Fig. 17.7).
Figure 17.7 Examples of impaired performance on a test for copying given to a
patient with unilateral neglect. (From Hickey, J. (2013). Clinical practice of
neurological and neurosurgical nursing (7th ed.). Philadelphia, PA: Lippincott
Motor Functions
Sensory loss seldom occurs in isolation but typically accompanies the loss of
motor functions. The combination of sensory and motor loss impairs limb
recovery and overall participation in occupations (Meyer, Karttunen, Thijs, Feys,
& Verheyden, 2014). Motor dysfunction because of CVA usually results in
changes in muscle tone that render normal movement impossible. It has been
commonly said that hypotonicity (flaccid hemiplegia) gives way to hypertonicity
(spastic hemiplegia); occasionally, there is progress into a final stage, in which
normal movement patterns re-emerge (Mikołajewska, 2012).
The muscle tone of individuals with an intact central nervous system
operates within a range that permits effective voluntary movement. Normal
muscle tone is high enough to stabilize and maintain a person through an
activity, while at the same time low enough to allow ease of movement. This
variability of tone allows mobility to be superimposed on stability (Gogola,
Saulicz, Kuszewski, Matyja, & Myśliwiec, 2014).
Abnormal tone may be termed either low or high. Hypotonus, or flaccidity,
is felt as too little resistance, or floppiness. When released, the extremity will
drop. At the other extreme, when there is too much resistance, hypertonus, or
spasticity, is felt. Spasticity is the result of hyperactive reflexes and loss of
moderating or inhibiting influences from higher brain centers (Florman, Duffau,
& Rughani, 2013; Trompetto et al., 2014). Spasticity may be aggravated by pain,
emotional upset, or efforts to hurry. Spasticity is never isolated to one muscle
group but is always a part of what is known as either an extensor or flexor
synergy, that is, a grouping of stereotypical movements. These movement
patterns usually consist of a flexion pattern in the arm (scapular retraction and
depression, shoulder adduction and internal rotation, elbow flexion, forearm
pronation, wrist flexion, finger and thumb flexion and adduction) and an
extension pattern in the leg (pelvis rotated back and internal rotation, knee
extension, foot plantar flexion and inversion, toe flexion and adduction).
Abnormal tone is not limited to the extremities, however, and is manifested
in the head and trunk. The head is usually flexed toward the hemiplegic side and
rotated so that the face is toward the unaffected side. The trunk is rotated back
on the hemiplegic side with side flexion of the hemiplegic side (Karthikbabu et
al., 2012; Liao, Liaw, Wang, Su, & Hsu, 2015). These typical patterns of
spasticity interfere with the normal, smooth, efficient, and coordinated
movement necessary for locomotion in and manipulation of the environment
including daily occupations (Fujita et al., 2015) (Figs. 17.8 and 17.9).
Figure 17.8 Posture of client with right-sided stroke. (From Walton, T. (2010).
Medical conditions and massage therapy. Philadelphia, PA: Lippincott Williams
& Wilkins.)
Figure 17.9 Posture of a client with left-sided stroke. (From Salter, R. B. (1998).
Textbook of disorders and injuries of the musculoskeletal system. Philadelphia,
PA: Lippincott Williams & Wilkins.)
If untreated, spasticity may lead to contractures (Chang et al., 2013). Spasticity

can be debilitating for clients if it interferes with participation in occupations or
causes pain. Different types of pharmacological approaches can be used
including botulinum toxin and diazepam to decrease the effects of the spasticity,
and research continues to investigate different forms of tone management
(Dashtipour, Chen, Walker, & Lee, 2015; Francisco & McGuire, 2015).
Addressing the return of motor function is an important part of rehabilitation,
and a number of theories and treatment methods have been developed. In recent
years, one approach that is being studied for its effectiveness is known as
constraint-induced therapy (CIMT). CIMT has been found to be highly effective;
however, there are questions about its practical use as CIMT is a highly
restrictive and time-intensive program and is not a technique that can be used by
clients who are severely impaired (Marque, Gasq, Castel-Lacanal, De Boissezon,
& Loubinoux, 2014). Modified CIMT (m-CIMT) programs have been studied
where the client does not have the unaffected arm restrained for as long as
dictated in CIMT programs and success has been documented (Park, Lee, Cho,
& Yang, 2015) but this research is ongoing.
The hemiplegic shoulder is also a common concern. Typical problems
include shoulder subluxation, pain, and immobility. Because of the unstable
nature of the glenohumeral joint, the anatomy of the shoulder is particularly
vulnerable to problems. Normally, this lack of stability is partly compensated for
by a strong surrounding musculature. However, subluxation is inevitable once
the surrounding musculature of the shoulder, especially the so-called rotator cuff
muscles, has been damaged (Huang et al., 2012; Kim et al., 2014).
After a CVA, changes in muscle tone and movement, the position of the
scapula, and joint capsule stability allow the pull of gravity to draw the head of
the humerus out of the glenoid fossa of the scapula, resulting in shoulder
subluxation (Huang et al., 2012). Clients with hemiplegia have lost voluntary
movement in muscles such as the supraspinatus, infraspinatus, and posterior
fibers of the deltoid. In addition, the muscles that support the scapula in its
normal alignment are affected, which leads to a change in angulation of the
glenoid fossa (Fig. 17.10).
Figure 17.10 Shoulder subluxation. (From Oatis, C. (2008). Kinesiology: The
mechanics and pathomechanics of human movement (2nd ed.). Philadelphia, PA:
Another typical complication is “the painful shoulder.” This condition may

either develop quickly after a CVA or at a much later stage. It presents with
flaccid or spastic muscle tone and with or without subluxation. In hemiplegia,
the normal, coordinated, and timed movement of the scapula and humerus
(scapulohumeral rhythm) has been disturbed by abnormal and unbalanced
muscle tone (Cho, Kim, & Joo, 2012). The typical hemiplegic postural
components of depression and retraction of the scapula and internal rotation of
the humerus are especially important to the mechanism of pain. Fear of pain
during passive movement of the arm will further increase abnormal flexor tone,
which can become a vicious cycle (Kim, Jung, Yang, & Paik, 2014). A
chronically painful shoulder can lead to shoulder-hand syndrome, also known as
reflex sympathetic dystrophy (RSD). This complex condition produces severe
pain, edema of the hand, and limitations in range of motion on the involved side
(Li et al., 2012). These complications not only interfere with movement, but they
may have profound emotional consequences.
Another motor dysfunction caused by CVA is the presence of associated
reactions. Associated reactions in hemiplegia are abnormal reflex movements of
the affected side that duplicate the synergy patterns of the arm and leg
(McMorland, Runnalls, & Byblow, 2015). These movements may be observed
when the client moves with effort, is trying to maintain balance, or is afraid of
falling. A flexor pattern of involuntary movement in the arm is often observed
when the individual yawns, coughs, or sneezes. Associated reactions also are
seen when new activities, such as running or putting on socks, are attempted
after a CVA. They are stereotyped reactions and may occur even if no active
movement is present in the limb. The limb returns to its normal position only
after cessation of the stimulus and usually does so gradually (Bhakta, O’Connor,
& Cozens, 2008).
Unlike associated reactions, associated movements accompany voluntary
movements and are normal, automatic postural adjustments. They reinforce
precise movements of other parts of the body or occur when a great amount of
strength is required. They are not pathological and can be stopped at will.
Associated movements often can be observed in the unaffected extremities of
individuals with CVA who are trying new activities (Bhakta et al., 2008).
Other motor dysfunctions include orofacial weakness, which may cause
difficulties in expression, speech (dysarthria), mastication, and swallowing
(dysphagia) (Yanagida, Fujimoto, Inoue, & Suzuki, 2015; Yang, Choi, & Son,
2015). Additionally, bladder or bowel incontinence may result from a
communication disorder or from disruption of normal routine and diet, lack of
awareness of body function, or emotional disorder (Daniels et al., 2009;
Kovindha, Wyndaele, & Madersbacher, 2010).
Mental Functions
Within mental function, the Practice Framework (OTPF) incorporates the areas
of affective, cognitive, and perceptual. Cognition encompasses information
processing, including attention, comprehension, and formation of speech (Gillen
et al., 2015) with cognition being a foundational determinant of occupational
performance, social participation, and well-being (AOTA, 2013). Severe CVAs
often result in cognitive deficits that affect global and specific mental functions
(Hallevi et al., 2009). Statistically, overall prevalence of cerebral infarct resulting
in cognitive impairment ranges from 31% to 77% (Jaillard, Naegele, Trabucco-
Miguel, LeBas, & Hommel, 2009). Milder CVAs may have a more subtle impact
on mental function, however. Commonly used psychometrics are not always
sensitive to the wide range of mental functions and process skills that may
permit effective occupational performance: initiation, recognition, attention,
orientation, sequencing, categorization, concept formation, spatial operations,
problem solving, and learning abilities (Nys et al., 2007). Moreover, basic visual
deficits also have an impact on cognitive performance. For example, visual
attending and scanning deficits lead to a decrease in the efficiency required for
cognitive performance (Hoffmann, Bennett, Koh, & McKenna, 2010;
Pendlebury, Cuthbertson, Welch, Mehta, & Rothwell, 2010). In summary, CVAs
have the potential to cause deficits in perception and cognition that impact
functional independence (Gillen et al., 2015).
Emotional Functions
As many as 50% of all clients who experience a CVA may encounter a stroke-
related psychological or emotional disorder (Hildebrand, 2015). Carota and
Bogousslavsky (2009) organized these disorders into the four categories: (1)
affective and mood disorders, such as depression, poststroke emotionalism, and
generalized anxiety disorders; (2) behavioral and personality changes such as
anger, irritability, apathy, sexual changes, and obsessive-compulsive disorder; (3)
cognitive and behavioral disintegration such as acute confusional state and
delirium; and (4) perception-identify disorders of the self or of other people and
places. Significant depression has been recorded in 30% to 50% of stroke
survivors (Berg, Lönnqvist, Palomäki, & Kaste, 2009). These changes often are
a major cause of concern to relatives and the individual. While depression is
often viewed as a natural and understandable consequence of reduced function
caused by CVA, proper treatment can result in observable improvement.
Depression is more frequent and severe with lesions in the left hemisphere, as
compared with right hemisphere or brainstem strokes (Williams et al., 2007).
Both organic and psychological factors are probably involved in poststroke
depression (Williams et al., 2007).
Emotional lability, sudden and extreme shifts of mood, may be the result of a
release of inhibition. The individual may switch from laughing to crying for no
apparent reason. Excessive crying is the most common problem and is frequently
the result of organic emotional lability rather than depression or sadness over
perceived losses. Organic emotional lability is characterized by little or no
obvious relation between the start of emotional expression and what is
happening around the person (Provinciali et al., 2008).
Catastrophic reactions are outbursts in which frustration, anger, and
depression are combined. When individuals cannot perform tasks that used to be
very easy, they may be unable to inhibit emotional expression and may begin
sobbing, expressing a sense of hopelessness (Carota, 2008; Thompson & Ryan,
2009). Outbursts and emotional difficulties are to be expected after CVA.
Relatives and families should be told that a tendency to cry easily or get upset
will improve with time. Families and therapists need to develop a positive,
understanding attitude if the individual is to overcome psychological sequelae.
The psychosocial impact of CVA on patients and families can be lessened with
increased social support and access to services once the individual has been
discharged to home and community (Ferro, Caeiro, & Santos, 2009).
CASE-STUDY 1
Initial Presentation
Janet is a 67-year-old female who retired 5 months ago from her occupation
of president of a trucking company. She and her husband had built the
company from the ground up starting with two trucks 30 years ago. When
Janet retired, the company had 500 trucks. She handed the reins to her twin
sons who have been “in training” for the past 5 years. Janet’s job meant she
was at her desk for long hours each day, as well as on call on weekends.
The job could be stressful when trailer deliveries did not occur at the time
promised or trucks would break down. There was also a high turnover with
drivers, and she was always trying to fill positions. Her husband, the CEO,
also retired from the same company on the same day. Janet has never been
one to exercise. She would just go home and watch TV being so mentally
exhausted from her days of dealing with people and problems. Janet has
been gaining weight over the past 10 years, but she was not concerned. She
eats fast food for dinner at least 4 out of the 5 work days and never has time
for breakfast. Her lunch consist of coffee and whatever people have brought
in for snacks and desserts. She cooks meals at home on the weekends, but
she feels now that she is home more she will be able to start cooking more
healthy meals. At this time, she has not been able to start with home
cooking since she and her husband have been eating out a lot as their
retirement treat. But, she plans to start meal planning soon. Her doctor kept
telling her she needed to lose weight and was concerned especially since
her BP has been consistently in stage 1 hypertension averaging 150/90 for
her past 4 yearly physical exams. Her doctor wants her to take medications
to address this issue, but Janet does not want to take any drugs. She just
does not believe in pharmaceuticals. Now that she has all this extra time,
she believes she will be able to start walking and participate in a consistent
exercise program. She feels her BP will be fine and she will be able to lose
all the weight. If only she could get motivated to start… “Tomorrow” she
tells herself every day.
Sunday afternoon, Janet began to get a headache located in the back of
her head. She also began to feel a little nauseated, and as she walked to her
bed to take a nap, she ran into the doorway to her bedroom. “That was
clumsy of me” she thought as she lay down on her bed and closed her eyes.
Two hours later, Janet woke up with a worse headache. She also noticed as
she looked up at her ceiling that she could not see her light, which is located
to the left of the bed. She turned her head to the left and then saw the light.
She thought to herself that maybe she had never been able to see the light
and started to get out of bed to go get some aspirin for her headache. As she
started to stand up from her bed, she lost her balance and fell to her left side
on her bed. “I just can’t believe how clumsy I am today” she thought to
herself as she tried to stand again. This time she fell to the floor with a
crash that brought her husband running into the bedroom. Her husband
lifted her up to the bed and asked her what happened. Janet could not figure
out why and how she had ended up on the floor. She started to stand again
and realized how heavy her left arm felt. Looking at her husband as he
stood in front of her, she realized she could only see part of him. So strange
she thought to herself, what is going on and why is he standing there?
Janet’s head was hurting so she tried to lay back down but ended up
flopping onto her left side on the bed. Her husband could see something
was wrong with her and wondered if she had hit her head when she fell. She
was not making sense to him, and she wanted to go back to sleep. He
wondered if she had a concussion. He reached for the phone and dialed 911
asking for an ambulance while trying to keep her talking since he knew that
she needed to stay awake if she had hit her head. Janet was not making
much sense to him. She was talking about packing for a vacation and going
to Mexico. The ambulance arrived in 5 minutes and proceeded to take her
and her husband to the emergency room of the local hospital.
Hospital Course
Once in the emergency room, the ER doctor started a preliminary
evaluation by asking Janet and her husband questions. Janet was clearly
confused by the questions, and her verbalizations were full of made-up
words and slurred speech. Her husband expressed his concern that she had
hit her head when she fell. The doctor did not see any external signs of
trauma and her visual exam showed a left visual homonymous hemianopia.
Due to this finding, the doctor activated the stroke response team. Nursing
took a finger stick to assess blood sugar and started an IV. The stroke
response team arrived and ordered a noncontrast CT scan, cardiac workup,
and blood work including CBC, Chem-7, and PT/INR. Results from the CT
scan determined an infarct had occurred on the right posterior cerebral
artery located in the occipital lobe as well as extending into the right
internal capsule and thalamus. Since her husband reported she had
complained of a headache about 3 hours ago, tPA was started at the time.
Janet was transferred to the ICU where she was monitored by nurses.
Forty-eight hours later, orders were sent to occupational, physical, and
speech therapies to evaluate Janet. Occupational therapy noted that she had
left homonymous hemianopsia, her left UE strength was weak including her
grip and she is left-hand dominant, she had decreased sensation on her left
UE, and she was oriented to self only. Janet was able to independently wash
her face and brush her teeth after setup using her right hand as she sat in a
chair. Since she was in the ICU, the OT did not evaluate her dressing skills.
When she spoke, some of her words did not make sense, but she followed
all one-step commands consistently. Physical therapy’s assessment
documented her left LE strength was decreased, but she was able to bear
weight on both her legs when she stood up. Her standing and sitting balance
were also decreased, and the therapist always had to have her hand on
Janet’s transfer belt. Ambulation was not attempted. The speech therapist
assessed Janet’s swallow and found it to be functional. Her speech was
slurred, and she appeared to have problems using the correct words. Janet
stayed on the ICU unit one more day and was then transferred to the neuro
step down unit. The therapists continued to see her for ADLs, mobility, and
speech articulation. Within 3 days, Janet was ready to be transferred to the
rehabilitation unit where she would receive at least 3 hours of therapy a day.
Janet’s husband and sons were very happy with this transition as they all
agreed Janet would be going home after an inhospital rehabilitation stay.
The goals were to get Janet as independent as possible in her mobility and
daily living skills.
CASE-STUDY 2
Initial Presentation
During a basketball game, Danny Wiley, a 9-year-old boy developed a
sudden onset of weakness and clumsiness on his left side of his body. He
quickly noticed that he was unable to dribble the ball using his left hand. At
the same time, he felt like his head was going to pop off of his body due to
the immense pain and pressure that he felt. Danny’s coach noticed that
Danny was weaving down the court and that his ball skills were
significantly worse than normal. Danny, who was his star forward, was
unable to control the ball or his body at all. Danny’s coach quickly signaled
a time-out to the referee. As soon as Danny’s coach ran over to him, Danny
had collapsed onto the gym floor. Emergency medical services were called
immediately and arrived minutes later.
Hospital Course
Within 30 minutes, Danny and his family were in the emergency room at
the local hospital. In the ER, Danny’s blood pressure was elevated and he
was disoriented. Initial physical examination revealed left-sided weakness
and sensory loss more apparent in Danny’s arm compared to his leg. The
admitting doctor evaluated Danny and advised that he be seen by a pediatric
neurologist. The neurological examination revealed decreased grip strength
in Danny’s left hand, poor finger dexterity, and slowing of rapid alternating
movements such as being able to turn his left hand palm up and palm down
quickly.
The ER doctor ordered a CT scan of Danny’s head, which came back
showing internal bleeding. The neurology team advised that Danny be
admitted for inpatient hospitalization to allow more testing as they
suspected a CVA. Danny and his parents were very worried but trusted the
neurology team. MRI with MR angiography (MRA) validated right AIS. A
shunt was inserted to relieve the pressure on his brain, and an angiogram
revealed an arteriovenous malformation (AVM), an abnormal collection of
blood vessels, in the medulla area of the brainstem. Following shunt
surgery, Danny recovered in the pediatric intensive care unit or PICU and
was kept in a chemically induced coma. He was on a ventilator because
doctors were not sure he could breathe on his own. Danny’s family was full
of worry and questioned how this CVA would affect their son’s life. A
couple of days later, Danny’s eyes opened and he asked his mom, “Where
am I?” At that time, Danny’s doctor ordered occupational therapy (OT),
physical therapy (PT), and speech therapy (SLP) to evaluate Danny’s
current functioning.
The therapists assessed Danny in the PICU the day the order was
received. PT watched how Danny moved his right leg in bed, but noted how
he was unable to move his left leg by himself. When PT assisted Danny in
sitting at the edge of his bed, Danny required moderate assistance to stay
upright and kept leaning his body toward the left. OT observed Danny
brushing his teeth and washing his face with his right hand with help to set
up the toothbrush with paste and wring out the washcloth. The SLP
observed Danny talking and remembering the names of his friends who
came by for a visit. Danny’s family and doctors were pleased to see that
Danny demonstrated no memory impairments. However, Danny was unable
to move his left arm and leg without great effort. He was sent to a
rehabilitation center for therapy to regain motor functions with the goal of
returning to school and basketball.
RESOURCES
National Stroke Association
www.stroke.org
American Stroke Association
www.strokeassociation.org
National Institute of Neurological Disorders and Stroke
www.ninds.nih.gov
Children’s Hemiplegia and Stroke Association
www.chasa.org
Pediatric Stroke Network
www.pediatricstrokenetwork.com
American Heart Association
www.americanheart.org
Family Caregiver Alliance
www.caregiver.org
Stroke Rehabilitation Provider Resource
http://www.stroke-rehab.com/
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C H A P T E R
18 Cardiopulmonary Disorders
Reese Martin
KEY TERMS
Angioplasty
Arrhythmia
Atherosclerosis
Atrial fibrillation
Bradycardia
Bronchitis
Congestive Heart Failure
Coronary artery bypass grafting (CABG)
Cyanosis
Diastolic
Dyspnea
Emphysema
Hypertension
Hypoxemia
Myocardial Infarction
Systolic
Tachycardia
Nadine looked down at her feet and saw that they were really swollen today. She
also felt more short of breath than she did a couple of days ago but assumed it
was because she had been working hard to move some of her belongings into her
daughter’s home as she was going to help take care of her grandchildren for a
few weeks. A couple of months ago, her doctor had told her that she had signs of
congestive heart failure (CHF), and although she thought the name of the
condition sounded serious, she was told she could control it. She had no time to
think about it at the moment though, as she was focused on helping her daughter.
Over the next few days, her shortness of breath and fatigue worsened—and so
did her swollen ankles. One night, Nadine felt like she couldn’t breathe if she lay
flat in her bed, so she decided to sleep in her recliner. The next morning, she
decided to go see her doctor. While being weighed prior to her session, Nadine
noticed that she had gained 4 lb even though she had been eating less. During
their meeting, her doctor explained that her heart was not pumping hard enough
to circulate her blood normally and that fluid was building up in her lungs and
other parts of her body.
Occupational therapists often work with clients who have either a primary or
secondary diagnosis that involves the cardiopulmonary system. This chapter
reviews introductory anatomy and physiology information about the lungs and
heart and defines common conditions that negatively impact these important
structures. Common conditions to be further discussed in this chapter include
coronary artery disease (CAD), CHF, chronic obstructive pulmonary disorder,
myocardial infarctions (MIs), and hypertension.
Definitions and Description
The Cardiopulmonary System: An Overview
The cardiopulmonary system consists of the lungs, the heart, and their
interconnections. Cardiopulmonary function enables the heart and lung to
maintain flow and regulation of blood between these vital organs—a process that
relies on their connection via the pulmonary artery. In this section of the chapter,
a brief overview of these systems and their connections, as well as the
interaction of these organs as a functional unit that is the cardiopulmonary
system, are discussed. In addition, cardiopulmonary vital signs will be discussed,
which are essential in understanding how the heart and lungs function.
Lungs
The respiratory system includes the nose, throat, trachea, and lungs. Inhalation
of air contains oxygen and other gases. Oxygen is a basic gas that every cell in
the body requires to maintain life. To begin the inhalation process, the
diaphragm muscle pulls down into the chest cavity creating a vacuum for air to
enter. Air moves through the lungs, where small sacs called alveoli perform the
gas exchange. The lungs consist of two spongy, balloon-like parts. The right
lung contains three lobes and is slightly larger than the left lung, which contains
two lobes. In the lungs, the oxygen from each breath is transferred to the
bloodstream and sent to all the body’s cells as life-sustaining fuel. Each cell in
the body exchanges oxygen cells for waste, gas, and carbon dioxide. The carbon
dioxide travels back to the lungs where the waste gas is removed from the
bloodstream and exhaled from the body. This vital process, called gas exchange,
occurs automatically within the body (Fig. 18.1). The respiratory system has
several protective mechanisms. The epiglottis, a thin flap of tissue that covers
the trachea during each episode of swallowing, prevents foreign materials from
entering the air passages that lead to the lungs. Inhaled air is warmed and
moistened for optimal humidity within the nose and mouth and then cleaned by
mucus, which lines the airway structures. Cilia, hairlike structures, remove the
mucus up and out of the system (American Lung Association, n.d.).
Figure 18.1 How lungs function. (From Anatomical Chart Company.)
Heart
The heart is the main pumping station of the circulatory system, working to
provide needed nutrients and oxygen to all organs of the body. The heart
attempts to provide the right amount of blood at the proper rate to make sure an
adequate amount of oxygen is delivered to all of the organs. The amount of
oxygen needed throughout the body varies depends on the tasks being
completed. For example, increased oxygen is needed in the gastrointestinal
system when digesting after a meal. Also, voluntary muscle groups require more
oxygen while exercising. Heart rate changes in order to meet these needs.
The heart contains four chambers: the two upper chambers (atria) that collect
blood and the two lower chambers (ventricles) that pump blood out of the heart.
Beginning with the right atrium, oxygen-depleted blood enters the upper
chamber after circulating through the body, continuing to the right ventricle and
then entering the lung to receive oxygen. Oxygen-rich blood returns from the
lungs to the left atrium, continues to the left ventricle, and is then expelled to the
body via the bloodstream. Muscular walls surround the four chambers and
valves of the heart to keep the blood moving in the right direction and prevent
backflow. Refer to Figure 18.2 for a visualization of the process of the blood
flow through the heart.
Figure 18.2 Blood flow through the heart. The arrows show the direction of
blood flow. Red vessels carry oxygen-rich blood and blue vessels carry oxygen-
poor blood. (From Anatomical Chart Company.)
Combination of Lungs and Heart

It is vital that the heart and lungs work in tandem in order to efficiently transport
oxygen and nutrients to all systems of the body. The lungs receive deoxygenated
blood from the heart, where the blood circulates through the alveoli within the
lungs in order to gather oxygen from air inhaled into the lungs and remove the
carbon dioxide waste. The lungs then return the oxygenated blood to the heart in
order to be pumped back out to the rest of the body. Due to the direct connection
between these two organs, conditions impacting one can cause impairments of
the other.
Cardiopulmonary Vital Signs
Cardiopulmonary vital signs measure cardiac pulse rate, respiratory rate, blood
pressure, oxygen saturation, and temperature. These measurements help detect
potential medical issues as well as monitor how the body tolerates activities.
When working with various cardiopulmonary disorders, vital signs are often
monitored closely to determine a patient’s tolerance to activities (John Hopkins
Medicine, n.d.).
Pulse Rate
The pulse rate, often referred to as a heart rate, is the number of times the heart
beats per minute (BPM). As the heart pushes blood through the arteries, the
arteries expand and contract with the flow of the blood. This can be measured by
palpation, or the manual pressing of the examiner’s fingers on arteries located
close to the surface of the skin. The outcome of this is the heart sounds that
occur as the heart forces blood through the arteries. The pulse can be found on
the wrist, inside of the elbow, or neck. To check a pulse rate, count the beats felt
in 60 seconds by firmly pressing the fingertips of your first and second digits. A
normal resting heart rate for healthy adults ranges from 60 to 100 BPM. Adult
females tend to have a higher resting heart rate than do adult males.
Arrhythmias are any changes from the normal sequence of electrical impulses
in the heart, causing the heart to beat too slow, too quickly, or irregularly. If the
heart doesn’t beat appropriately, blood is not adequately pumped throughout the
body, which may lead to organs inefficiently working or becoming damaged
(“Tachycardia, Fast Heart Rate,” 2015). Although 60 to 100 BPM is considered
normal, athletes may have a heart rate as low as 40 BPM without experiencing
problems. A heart rate <60 BPM in adults is called bradycardia. A heart rate
higher than 100 BPM in adults is called tachycardia. Atrial fibrillation (AFib)
is an irregular, rapid heart rate that can lead to poor blood flow to the body. This
occurs when the upper chambers of the heart quiver and beat irregularly, leading
to ineffectively moving the blood to the ventricles. This can cause the blood to
slow down and pool, which increases the risk for blood clots, stroke, and heart-
related complications, including heart failure (Mayo Clinic Staff, 2015).
Respiratory Rate
The number of breaths a person takes per minute is the respiratory rate. The rate
is measured by counting the number of breaths for 1 minute by counting how
many times the chest rises typically when a person is at rest. The average adult
takes 15 to 20 breaths per minute (American Lung Association, n.d.).
Blood Pressure
Blood pressure is the force of blood pushing against the artery walls. Blood
pressure consists of two numbers written as a fraction and is recorded as
millimeters of mercury (mm Hg). The recording represents how high the
mercury column on a mercury manometer, an older manual blood pressure
device, is raised by the pressure of the blood. Blood pressures are now
commonly taken with a blood pressure cuff with a simple dial and a stethoscope.
The top number, also known as the systolic, measures the pressure in the arteries
when the heart muscle contracts or beats. The systolic factor is usually the higher
of the two numbers. Diastolic, the bottom number, measures the pressure in the
arteries when the heart muscle is resting between beats and refilling with blood.
One’s blood pressure rises with each heartbeat and falls when the heart relaxes
between beats. For an adult over 20, a normal blood pressure should be <120/80
mm Hg. Hypertension, or high blood pressure, is a prevalent chronic disease
that is often asymptomatic. Early-stage hypertension may present with dull
headaches, dizzy spells, or increased nosebleeds. These signs and symptoms
usually do not occur until high blood pressure has become severe or life
threatening. Hypertension will be discussed in further detail later in this chapter.
Hypotension, low blood pressure, can cause symptoms of dizziness and light-
headedness, and severe cases can be life threatening. Blood pressure is
considered low if the systolic (top number) is 90 mm Hg or below or if the
diastolic (bottom number) is 60 mm Hg or less. Hypotension can occur for
various reasons, such as dehydration or pregnancy, but is generally treatable
once the cause of the low blood pressure is identified (American Heart
Association, 2014).
Oxygen Saturation
In order to function properly, your body needs a certain level of oxygen
circulating in the blood to cells and tissues. Oxygen saturation is a percentage
measure of the amount of oxygen in which the blood is carrying. Oxygen
saturation can be measured by testing a sample of blood from an artery by way
of a pulse oximeter, which is a small device that usually clips to a finger. Normal
pulse oximeter readings range from 95% to 100%. Values under 90% are
considered low, or hypoxemic. Hypoxemia, or low blood oxygen, describes a
lower than normal level of oxygen in your blood (Mayo Clinic, n.d.).
Prevalent Cardiopulmonary Disorders
Coronary Artery Disease
CAD develops when your coronary arteries, the blood vessels that supply the
heart with blood, oxygen, and nutrients, become damaged. CAD occurs when
plaque, which is the by-product of cholesterol, fat, and calcium deposits and
other substances, builds up in the arteries that supply blood to the heart. Over
time, the plaque hardens and leads to narrowing of arteries, or stenosis, reducing
the rate of blood flow to the heart. This process is also known as atherosclerosis
(Fig. 18.3).
Figure 18.3 A. Deterioration of a coronary artery from deposits of fatty
substances that roughen the vessel’s center. When a thrombus (blood clot) forms
above the plaque, complete blockage of the artery produces a myocardial
infarction or heart attack. A coronary artery bypass graft (CABG) creates a new
“transportation route” around the blocked region to allow the required blood
flow to deliver oxygen and nutrients to the previously “starved” surrounding
heart muscle. The saphenous vein from the leg is the most commonly used
bypass vessel. CABG involves sewing the graft vessels to the coronary arteries
beyond the narrowing or blockage, with the other end of the vein attached to the
aorta. Medications (statins) lower total and LDL cholesterol, and daily low-dose
aspirin (81 mg) reduces post-CABG artery narrowing beyond the insertion site
of the graft. Repeat CABG surgical mortality averages 5% to 10%. B.
Angioplasty procedure to fix a blocked coronary artery. (From McArdle, W. D.,
Katch, I., & Katch, V. L. (2013). Exercise physiology (8th ed.). Philadelphia, PA:
Etiology
Causes of the CAD are thought to be a combination of lifestyle choice and
genetics. CAD is thought to begin with damage or injury to the inner layer of a
coronary artery. The damage may be caused by smoking, high cholesterol, high
blood pressure, diabetes, and obesity or sedentary lifestyle, or radiation therapy
to the chest. Age and family history are additional risk factors.

According to the Center for Disease Control (2015), heart disease is the leading
cause of death for both men and women. Coronary heart disease is the most
common type of heart disease in the United States, resulting in death in over
three hundred and seventy thousand people annually (Center for Disease
Control, 2015).
Signs and Symptoms

At first, the decreased blood flow to the heart may not cause any symptoms. As
plaque builds up in the coronary arteries, a patient may develop angina (chest
pain), shortness of breath, and/or fatigue with exertion. A complete blockage of a
coronary artery may lead to a heart attack.

CAD progresses and slowly worsens with time; the disease can go unnoticed
until an artery is blocked to the point of a heart attack. Over time, the disease can
weaken the heart muscle, which may lead to heart failure, a serious condition
where the heart cannot adequately pump blood. Arrhythmias can also develop as
the disease progresses.
Medical management for CAD is commonly a combination of lifestyle changes,
medication, and, if severe enough, surgical interventions. Lifestyle changes
include smoking cessation, healthy diet, and active lifestyle, losing weight, and
stress reduction. Medications utilized for CAD include cholesterol-modifying
medications; aspirin or a blood thinner; beta-blockers, which can slow the heart
rate and decrease blood pressure; and nitroglycerin tablets to open coronary
arteries and decrease chest pain. When the artery blockage is severe enough,
certain procedures to restore and improve blood flow are needed. An
angioplasty is a procedure in which a catheter with a deflated balloon is inserted
into the blocked artery (Fig. 18.3). Once in place, the balloon is inflated,
compressing the plaque to the sides of the artery, opening up the artery, and
improving blood flow to the heart. A stent, a small metal mesh tube, is then
inserted into the narrowed artery. Some stents release medicine to help keep the
artery open. When CAD is causing significant blockage, one may have a
coronary artery bypass graft (CABG) (Fig. 18.4). A CABG is a procedure in
which arteries or veins from other parts of the body, oftentimes from the leg, are
removed and surgically attached to bypass the blocked arteries in the heart. In
order to perform surgery on a patient’s heart, the sternum is often cracked open
and wired back together after surgery. After the surgery, patients generally have
sternal precautions: no pushing, pulling, or lifting anything more than 5 to 10 lb
(surgeon dependent) in order for the sternum to adequately heal. Patients with
CAD often attend cardiac rehabilitation, a medically supervised program
consisting of exercise training as well as educating the patient about the disease.
Occupational therapists are often involved in these programs (National Heart,
Lung, and Blood Institute, 2014).
Figure 18.4 Coronary artery bypass surgery. Two types of bypasses are
illustrated: (1) the left internal mammary artery originates from the left
subclavian artery, and in this schematic, it is anastomosed to the left anterior
descending (LAD) coronary artery distal to obstructing plaque; (2) one end of a
saphenous vein graft is sutured to the proximal aorta and the other end to the
right coronary artery (RCA) distal to a stenotic segment. (From Lilly, L. S.
(2015). Pathophysiology of heart disease (6th ed.). Philadelphia, PA: Wolters
Kluwer.)
Congestive heart failure
Congestive heart failure, or heart failure (CHF), is a chronic, noncurable
condition where the heart does not beat strongly enough to maintain adequate
blood flow throughout the body causing organs to become oxygen deprived and
leading to fluid retention throughout the body.
Etiology
Certain conditions such as narrowed arteries (CAD) or chronic high blood flow
can gradually lead to the heart muscles becoming too weak or stiff to fill and
pump blood efficiently. According to the American Heart Association (2014),
CAD is the most common cause of CHF. Other common risk factors that may
lead to CHF include heart attacks causing damage to the heart muscles,
congenital birth defects, arrhythmias, high blood pressure, infection of the heart,
diabetes, thyroid problems, obesity, alcohol or drug abuse, or certain types of
chemotherapy.

According to CDC, about 5.1 million Americans are diagnosed with heart
failure. Heart failure is one of the most common reasons people aged 65 and
older go into the hospital. In 2009, heart failure was a contributing cause of
death for one in nine deaths in the United States (Center for Disease Control,
2015).
Signs and Symptoms

Signs and symptoms of CHF include dyspnea, a sensation of having impaired
breathing such as shortness of breath, due to exertion or when lying down,
fatigue and weakness, and reduced activity/exercise tolerance. A patient may
also experience chest pain, arrhythmias, coughing, or wheezing. Patients
diagnosed with CHF often suffer from fluid retention causing sudden weight
gain, edema in lower extremities, ascites (swelling of the abdomen), and
increased need to urinate at night. Additional symptoms include lack of appetite,
nausea, as well as difficulty concentrating or decreased alertness (Mayo Clinic,
n.d.).
CHF is a chronic diagnosis that requires lifelong management. Shortness of
breath, an initial symptom of the condition, is often mistaken for a normal
process of aging. As the condition progresses, shortness of breath worsens and
overall endurance declines. Although treatment may improve various symptoms,
the disease slowly progresses depriving the body of much needed oxygen and
may eventually lead to death. The Center for Disease Control (2015) estimates
that about half of people who develop heart failure in the United States die
within 5 years of diagnosis. Early diagnosis and treatment can improve quality
and length of life.
Treatment can help improve the various symptoms associated with the disease
leading to an increased quality of life, increase lifespan, and reduce the chances
of sudden death. Specific treatment is determined via identifying and treating the
underlying cause of the heart failure. Similar to all cardiopulmonary conditions,
lifestyle changes can decrease the rate of progression of the disease. Lifestyle
changes include diet modifications such as limiting sodium intake and
monitoring portions, exercising regularly, and smoking cessation. According to a
systematic review of various research articles, authors report that although
length of life was not significantly impacted, men with mild to moderate CHF
who participated in regular exercise had a better quality of life and better overall
fitness than did those who did not (Davies et al., 2010). Medications are often
utilized to help minimize the symptoms associated with CHF. Medications may
include angiotensin-converting enzyme (ACE) inhibitors, which expand the
blood vessels to decrease the heart’s workload, diuretics to reduce fluid
overload, and dioxin to help the heart contract properly. When CHF becomes
severe, surgical interventions may be indicated. If the CHF is caused by
inadequate blood flow to the muscles in the heart, a CABG surgery or
angioplasty (stent placement) or heart valve replacement or repair may be
considered in order to improve blood circulation. A pacemaker insertion may
also be indicated to help maintain a consistent heartbeat.
Chronic Obstructive Pulmonary Disease
Chronic obstructive pulmonary disease (COPD) is an overarching term, which
refers to a group of lung diseases characterized by airflow obstruction that
interferes with normal breathing. Emphysema and chronic bronchitis are the
most prevalent conditions that compose COPD, and they often coexist. A normal
functioning lung resembles an inflated balloon with tension and pressure to
allow for proper air exchange. COPD may cause the lungs to lose their elasticity,
resembling a somewhat deflated balloon. Walls of the airways may also become
inflamed or damaged, or mucus within the airways can build up and lead to a
blockage. The damage of COPD leads to decreased airflow. With emphysema,
the walls between air sacs are damaged causing the air sacs to lose their shape.
Damage can also destroy the walls of the air sacs, leading to fewer and larger air
sacs instead of many tiny ones in a normal lung. When this occurs, the quantity
of gas exchange in the lungs is reduced. Bronchitis causes the lining of the
airways to become irritated and inflamed causing the lining to thicken. Buildup
of mucus leads to difficulty breathing.
Etiology
The National Heart, Lung, and Blood Institute (n.d.) consistently indicates that
cigarette smoking is the leading cause of COPD. Long-term exposure to other
lung irritants—such as air pollution, chemical fumes, or dust—may also
contribute to COPD.

The Center for Disease Control reports that chronic lower respiratory disease,
primarily COPD, was the third leading cause of death in the United States in
2011. Fifteen million Americans report that they have been diagnosed with
COPD. Chronic bronchitis affects people of all ages, although people aged 65
years or more have the highest rate at 64.2 per 1,000 persons. In 2011, more
women reported a diagnosis of emphysema than did men and were twice as
likely to be diagnosed with chronic bronchitis. In 2011, 3.3 million men (29.6
per 1,000 population) had a diagnosis of chronic bronchitis compared to 6.8
million women (56.7 per 1,000 population). Prevalence seems to be highest in
the Midwest and Southeast states (Center for Disease Control, 2014).
Signs and Symptoms
Typical symptoms of COPD include persistent chronic cough with increased
mucus production, dyspnea (especially with exertion), wheezing, chest tightness,
frequent respiratory infections, and fatigue. COPD often goes undetected until
the condition is moderately to severely advanced. Severe COPD may cause
edema in the lower extremities, weight loss, or decreased muscle endurance.
Cyanosis, a bluish color to the skin (usually evident in lips and fingernails), is
usually due to decreased oxygen levels. The severity of symptoms is dependent
on the severity of lung damage. An American Lung Association (n.d.) survey
revealed that half of all COPD patients (51%) indicate that their condition limits
their ability to work. It also limits them in normal physical exertion (70%),
household chores (56%), social activities (53%), sleeping (50%), and family
activities (46%).

COPD is a progressive and irreversible disease. As the disease worsens,
increased difficulty breathing leads to decreased oxygen levels throughout the
body. The heart may become enlarged due to the strain with decreased oxygen
levels, and changes in blood pressures may also be present. Increased shortness
of breath and difficulty with daily tasks are often thought as symptoms of normal
aging leading to a delay in accurate diagnosis and medical management. With
the delay in diagnosis, decreased lung function has already occurred or began.
The progression of the disease can be slowed and quality of life maintained via
lifestyle changes such as smoking cessation or avoiding irritants, managing
symptoms, medication management, and simplifying daily tasks by way of
energy conservation techniques.
COPD is diagnosed through a history of exposure to toxins and lifestyle choices
as well as a series of functional pulmonary tests to determine lung function. A
spirometry test, the most common pulmonary measure, is used to assess how
well the lungs work by measuring how much air is inhaled and exhaled and the
pace of exhalation. A chest radiograph and computerized tomography (CT) scan
can visualize emphysema and lead to a diagnosis of COPD.
Although COPD is a progressive disease, medical management can help
manage symptoms, reduce the risk for exacerbations or complications, and
improve quality of life. The most critical treatment of managing COPD is
smoking cessation to prevent the disease from continuing to worsen. Various
medications may be utilized to help manage symptoms. Bronchodilators, often
prescribed as inhalers, relax the muscles around the airways to alleviate
coughing and decrease dyspnea. Inhaled and oral steroids reduce airway
inflammation and help prevent exacerbations. Antibiotics are often prescribed to
prevent exacerbations as respiratory infections such as pneumonia can aggravate
symptoms of COPD. Oxygen therapy may be indicated with moderate to severe
COPD to help maintain adequate oxygen levels. Patients with COPD may be
referred to a Pulmonary Rehabilitation Program, which combines education,
exercise training, nutrition advice, and personal counseling to improve quality of
life and decrease hospitalizations.
Despite consistent management, acute exacerbations (worsening of
symptoms for days to weeks) may occur and lead to lung failure if not
adequately and promptly treated. Respiratory infections or exposure to irritants
are often the cause of COPD exacerbations. Some forms of severe emphysema
that are not adequately managed with medications and lifestyle changes may
undergo surgical interventions such as lung volume reduction surgery or a lung
transplant.
Myocardial Infarction
A myocardial infarction (MI), commonly referred to as a heart attack, occurs
when the blood flow that brings oxygen to the heart muscle is severely reduced
or completely occluded causing the heart muscle tissue to be without oxygen.
This results in varying degrees of physical destruction to that tissue (Fig. 18.5).
Atherosclerosis, which is the formation of plaque within the arterial wall, is the
leading cause of MI. Atherosclerosis may rupture and leak cholesterol and other
substances into the bloodstream. A blood clot, or thrombus, forms at the site of
the rupture, which can occlude all blood flow through that coronary artery. A
spasm of a coronary artery is an additional cause of MI, which shuts down blood
flow to the heart muscle. These spasms are often a result of tobacco or illicit
drug use. Spontaneous coronary artery dissection, or a tear in the arterial wall,
may also lead to an MI (Mayo Clinic, 2015).
Figure 18.5 A heart attack occurs when one or more of the coronary arteries
become blocked, preventing blood from reaching the myocardium. The lack of
oxygen and nutrients causes the tissue that is supplied by the affected artery to
die. (From Carter, P. J. (2011). Lippincott textbook for nursing assistants (3rd
ed.). Philadelphia, PA: Lippincott Williams & Wilkins.)
Etiology
Key risk factors for heart attacks include smoking, high blood pressure, elevated
levels of cholesterol, diabetes, obesity and poor diet, sedentary lifestyles, and
excessive alcohol intake (Mayo Clinic, 2014).

About every 34 seconds, a single incident of MI occurs in the United States
(American Heart Association, 2014). The Center for Disease Control (2015)
reports that every year, about seven hundred and thirty five thousand Americans
are diagnosed.
Signs and Symptoms

The most common sign of a heart attack is chest discomfort or pain, which may
spread to the arm, neck, or jaw. Shortness of breath, nausea/vomiting, cold
sweats, and fatigue are additional warning signs of a heart attack (Fig. 18.6). The
American Heart Association reports that women, more often than men,
experience shortness of breath, neck/jaw or back pain, and nausea or vomiting.
Knowing the warning signs of a heart attack is essential to prevent death. A
study in 2005 revealed that 92% of respondents recognized chest pain as a major
sign of a heart attack, and only 27% of those in the study were aware of the
additional signs of a heart attack (Center for Disease Control, 2008).
Figure 18.6 Heart attack symptoms include chest pain, possible arm pain,
sweating, shortness of breath, nausea, and light-headedness. (From Delaet, R.
(2011). Introduction to health care & careers. Philadelphia, PA: Lippincott

Cardiac damage caused by an MI can be limited if adequate medical
management is acquired in an appropriate amount of time. With adequate
medical management and lifestyle modifications, many people survive heart
attacks and recover to return to full, active lives. Given a cardiac event, one is at
higher risk for additional MIs (Mozzafarian & Benjamin, 2015).
Early medical management can prevent or limit heart muscle damage. Medical
personnel can initiate treatment of an MI prior to admission to the hospital.
Aspirin is a first-line treatment to thin blood and prevent further clotting.
Nitroglycerin, typically a sublingual medication, is used for chest pain and
reduction of cardiac output and acts to improve blood flow through the coronary
arteries. An MI is often diagnosed via electrocardiogram (ECG) and blood tests
following admission. An ECG records the electric activity of the heart and
confirms, in part, that a cardiac event has occurred or is in process, as injured
heart muscles do not create normal electrical impulses. When the heart has been
damaged, certain enzymes slowly leak into the bloodstream, which can be
identified in a blood test. Once the diagnosis is confirmed, treatment is initiated
to restore blood flow to the heart. Thrombolytic medicine, commonly referred to
as “clot busting” medicine, is used to dissolve blood clots that are blocking the
coronary arteries. For best results, the medicines should be administered within
hours of the start of the symptoms of a heart attack. Once the location of the
blockage is identified, a patient may undergo a coronary angioplasty, a procedure
used to open blocked arteries. Smoking cessation, like all cardiac diseases, is
encouraged. Lifestyle changes may also be recommended, such as increasing
exercise/activity.
Hypertension
Hypertension, or high blood pressure, is an increase in the amount of force that
is pushing against the artery walls as the heart pumps blood. A blood pressure
consists of two readings, a diastolic and systolic number. Normal blood pressure
is considered to be 120/80 mm Hg. Hypertension is likely to be diagnosed when
one or both of the numbers are persistently above 140/90. Blood pressures above
120/80 mm Hg but below 140/90 mm Hg will result in a diagnosis of
prehypertension.
Etiology
There are two types of hypertension—primary and secondary. Primary
hypertension is diagnosed when there is no identifiable cause and tends to
develop gradually over years. Secondary hypertension is caused by an
underlying condition, tends to have a sudden onset, and causes higher blood
pressures than does primary hypertension. Various conditions that may lead to
secondary hypertension include kidney problems, adrenal gland problems,
thyroid problems, congenital blood vessel defects, certain medications, illegal
drugs, chronic alcohol use, and sleep apnea (Mayo Clinic, n.d.).

It is estimated that 29% or one of three American adults has high blood pressure.
Hypertension is most prevalent in African American women and older
populations as blood vessels become increasingly compromised with age. High
blood pressure increases the risk for various life-threatening conditions such as
heart attack, stroke, heart failure, and kidney disease (Mozzafarian & Benjamin,
2015).
Signs and Symptoms

Hypertension is often asymptomatic and can be undiagnosed and, therefore,
untreated for several years. Although asymptomatic, high blood pressure can
cause damage to the heart, kidneys, and other body structures. Those with early-
stage hypertension may present with dull headaches, dizzy spells, or increased
nosebleeds. These signs and symptoms usually do not occur until high blood
pressure has become severe or life threatening (Mayo Clinic, n.d.). Someone
experiencing a hypertensive crisis, which is defined as a systolic measure of 180
or higher or diastolic of 110 or higher, may present with severe headache and
anxiety, shortness of breath, and nosebleeds. Because hypertension often has no
signs or symptoms, high blood pressure is often identified when readings are
taken by a health care provider. Several blood pressure readings will be taken at
various times before a diagnosis is made as blood pressures can fluctuate
throughout the day (American Heart Association, 2014).

Hypertension can often be adequately controlled with medication and lifestyle
changes. Uncontrolled hypertension increases risk for various life-threatening
conditions such as heart attack, stroke, heart failure, and kidney disease.
The goal of treating hypertension is to lower blood pressure to decrease risk of
various complications described above. Lifestyle change is the first defense line
when treating hypertension. These changes include dietary changes (decreasing
salt intake, drinking water), increasing physical activity, smoking cessation, and
limiting alcohol intake. Medications can also help maintain a lower blood
pressure. Medications prescribed depend on the type of hypertension as well as
other medical conditions. Diuretics are commonly utilized to help the kidneys
eliminate water and sodium, which reduces blood volume. Various other
medications help relax the blood vessels making it easier for blood to flow
through, decreasing the workload on the heart (Mayo Clinic, n.d.).
Each of the cardiopulmonary disorders impacts occupational performance across
all domains. Common symptoms throughout the cardiac disorders described here
include shortness of breath, fatigue, and decreased activity tolerance. These
symptoms may cause increased difficulty managing all occupations and lead to a
decreased quality of life. Additionally, patients commonly have precautions after
cardiac procedures, which may impact occupational performance. Common
precautions after a coronary artery bypass grafting surgery and angioplasties
include no pushing, pulling, or lifting anything more than 5 to 10 lb. Functional
transfers and bed mobility are modified to maintain these precautions.
Household chores, work tasks, and play/leisure activities are often limited after
these procedures due to lifting restrictions. Occupational therapists play a lead
role when working with patients with cardiac disorders including assessing one’s
ability to safely complete activities of daily living, educating patients regarding
energy conservation and work simplification techniques, and assisting with
progressing back to normal activity by a program of strengthening and
endurance training.
CASE-STUDY 1
Kenny, a 58-year-old male, worked as a tow truck driver who spent 50 to 60
hours a week on the road towing cars after accidents. In the middle of a
high-stress 12-hour workday, Kenny began to feel severe chest heaviness
while on his way to his next job. Kenny did not think much of the pain,
figured it was heartburn from lunch, and continued on with his workday.
The chest heaviness, however, did not go away and began traveling to his
arms and neck. Kenny began having cold sweats and feeling nauseous. He
decided to go to the emergency room.
Once Kenny arrived at the emergency room, Kenny was given medicine
to alleviate chest pain and decrease workflow for the heart. Blood draws
and an EKG were completed and both came back abnormal; Kenny was
diagnosed with an acute MI. Kenny was given medicine to help break up
the blood clot that was preventing blood flow. Once his condition was
stabilized, Kenny was taken to the cardiac catheter lab and underwent a
coronary angioplasty in order to open the blocked arteries.
Kenny had to stay in the hospital for 2 days in order to be monitored by
cardiologists. Kenny was seen by cardiac rehabilitation therapists to provide
education and guidelines for home as well as dieticians to discuss diet
recommendations such as limiting salt intake. Kenny was able to go home
and continue living independently after a couple of short days in the
hospital. Once he completed outpatient cardiac rehabilitation and had a
follow-up appointment with his cardiologist, Kenny was able to return to
work.
CASE-STUDY 2
Ruth, a 71-year-old female, is a retired secretary who lives with her spouse.
Over the past several weeks, she has been experiencing increased shortness
of breath with activities and increased overall fatigue. The shortness of
breath and decreased activity level lead to significant overall weakness, and
Ruth made an appointment with her doctor to discuss her concerns. The
doctor ordered a cardiac stress test, which came back abnormal, so a cardiac
catheter procedure was completed. The procedure revealed severe
multivessel coronary artery disease. Due to the severity of the blockages to
the arteries, the doctor recommended a coronary artery bypass graft.
After discussion with her doctors and family, Ruth decided to have the
surgery and was admitted to the hospital. After the surgery, Ruth remained
in the hospital for approximately 1 week where she was monitored by the
cardiologists. After the surgery, Ruth worked with therapists. Because of
her surgery, Ruth was unable to use her arms to help herself get in and out
of bed or on and off the toilet. Because Ruth required more help with her
basic care and was having difficulty with transfers and mobility, she went to
a rehabilitation facility (skilled nursing facility) when she was medically
stable to leave the hospital.
Ruth stayed at the skilled nursing facility for 2 weeks until she was able
to safely care for herself and return home. Once she was home, her spouse
continued to assist her as needed, such as providing transportation to and
from appointments and helping with household chores and meal
preparation.
Resource List
American Heart Association
http://www.heart.org/HEARTORG/
American Lung Association
http://www.lung.org/lung-disease/
National Heart, Lung, and Blood Institute
http://www.nhlbi.nih.gov/
The Mayo Clinic
http://www.mayoclinic.org/
Center for Disease Control
http://www.cdc.gov/heartdisease/index.htm
REFERENCES
American Heart Association. (2014). Why blood pressure matters. Retrieved
from
http://www.heart.org/HEARTORG/Conditions/HighBloodPressure/WhyBloodPressureMatters
Blood-Pressure-Matters_UCM_002051_Article.jsp, on August 4, 2014.
American Heart Association. (n.d.). About heart attacks. Retrieved from
http://www.heart.org/HEARTORG/Conditions/HeartAttack/AboutHeartAttacks/About-
Heart-Attacks_UCM_002038_Article.jsp
American Lung Association. (n.d.). Chronic obstructive pulmonary disease
(COPD) fact sheet. American Lung Association. Retrieved from
http://www.lung.org/lung-disease/copd/resources/facts-figures/COPD-Fact-
Sheet.html
CDC. (2008). Disparities in adult awareness of heart attack warning signs and
symptoms—14 states, 2005. MMWR, 57(7), 175–179.
Center for Disease Control. (2015). Heart disease fact sheet. Retrieved from
http://www.cdc.gov/dhdsp/data_statistics/fact_sheets/docs/fs_heart_disease.pdf,
on February 19, 2015.
Centers for Disease Control and Prevention. National Center for Health
Statistics: National Health Interview Survey Raw Data, 2011. Analysis
performed by the American Lung Association Research and Health Education
Division using SPSS and SUDAAN software.
Davies, E. J., Moxham, T., Rees, K., Singh, S., Coats, A. J. S., Ebrahim, S., et al.
(2010). Exercise training for systolic heart failure: Cochrane systematic review
and meta-analysis. European Journal of Heart Failure, 12, 706–715. doi:
10.1093/eurjhf/hfq056
John Hopkins Medicine. (n.d.). Retrieved from
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Mayo Clinic. (2014). Heart attack causes. Mayo Clinic Retrieved from
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20019520, on November 2014.
Mayo Clinic. (2015). Heart failure symptoms. Mayo Clinic. Retrieved from
http://www.mayoclinic.org/diseases-conditions/heart-
failure/basics/symptoms/con-20029801, on January 17, 2015.
Mayo Clinic. (n.d.-a). Low blood pressure (hypotension). Mayo Clinic. Retrieved
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pressure/basics/definition/con-20032298
Mayo Clinic. (n.d.-b). High blood pressure (hypertension) treatments and drugs.
Mayo Clinic. Retrieved from http://www.mayoclinic.org/diseases-
conditions/high-blood-pressure/basics/treatment/con-20019580
Mayo Clinic Staff. (2015) Atrial fibrillation. Mayo Clinic. Retrieved March 18,
2015 from http://www.mayoclinic.org/diseases-conditions/atrial-
fibrillation/basics/definition/con-20027014
Mozzafarian, D., & Benjamin E. (2015). Heart Disease and Stroke Statistics—
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e29–322. doi: 10.1161/CIR.0000000000000152
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September 29, 2014.
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topics/topics/heartattack/lifeafter
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Fast-Heart-Rate_UCM_302018_Article.jsp#, on April 21, 2015.
C H A P T E R
19 Diabetes
Joanne Phillips Estes
KEY TERMS
Autonomic neuropathy Diabetic foot Diabetic ketoacidosis End-stage renal
disease Fasting plasma glucose Hemoglobin A1c
Hygiene hypothesis Hyperglycemia Hypertension Hypotension Insulin
Nephropathy Nocturia
Peripheral artery disease Peripheral neuropathy Polydipsia Polyphagia Polyuria
Prediabetes Retinopathy Type 1 diabetes Type 2 diabetes
Stella is a 61-year-old white female who retired from her job as a grocery clerk
about a year ago. Since retiring, Stella spends most of her time watching TV and
reading. She also replaced cooking balanced meals with eating processed
convenience foods, which contributed to an 80-lb. weight gain. Her children
have been encouraging Stella to see her physician for a long-overdue checkup,
but Stella has resisted doing so. She maintained that she does not need to see a
doctor because other than being thirsty and needing to urinate often, she feels
fine and perceives herself to be healthy. Ultimately, Stella agreed to have a
physical exam in order for her and her husband to be able to purchase life
insurance policies.
Stella saw her physician for a complete physical exam, including fasting
blood tests. The results of her checkup stunned Stella. Her weight of 258 lb and
body mass index of 29 kg/m2 classifies Stella as obese. Stella’s blood pressure
reading of 155/99 mm Hg resulted in a diagnosis of hypertension. Additionally,
results of her blood tests produced diagnoses of hyperlipidemia (total serum
cholesterol level = 250 mg/dL) and type 2 diabetes mellitus (HbA1c = 7.1%).
Stella’s doctor prescribed antihypertensive, cholesterol-lowering, and glucose-
reducing medications. He also strongly advised her to add a moderate exercise
routine such as walking 30 minutes per day in order to increase her activity
level.
Diabetes mellitus, more commonly known as diabetes, refers to a group of
metabolic conditions characterized by a malfunction in the body’s ability to
make insulin, to use insulin, or a combination of both (Canivell & Gomis, 2014).
Insulin is a hormone produced by the pancreas and functions to regulate glucose
metabolism. Insulin transports glucose into the body’s cells where it is used for
growth and energy. Without insulin, glucose builds up in the bloodstream
producing a condition known as hyperglycemia. The excess glucose is
ultimately excreted in the urine (Fig. 19.1). Without insulin to transport it, organs
and tissues do not receive glucose causing the body to break down its own fat or
lipids to produce an energy source. The by-products of this process are known as
ketones that are released in the bloodstream. Total lack of insulin is potentially
lethal, and a chronic high blood glucose level has devastating effects on multiple
tissues and organ systems. The American Diabetes Association (ADA) classifies
diabetes into four disease entities (i.e., type 1, type 2, gestational diabetes, and
other specific types) plus a prediabetes entity (ADA, 2015a). Historically, the
two most prevalent entities (type 1 and type 2 diabetes) were distinguished by
etiology and age of onset (Tuomi et al., 2014). Advances in understanding of the
underlying pathophysiology along with sociocultural trends have led to the
creation of subclassifications (Thomas & Philipson, 2015) and deem age to
continue to be an important but no longer a discriminating factor (ADA, 2015a;
Ducloux, Safraou, & Altman, 2015).
Figure 19.1 Type 1 diabetes mechanism of hyperglycemia.
Type 1 Diabetes
Type 1 diabetes, formerly known as insulin-dependent or juvenile-onset
diabetes mellitus, most frequently occurs in children and accounts for 5% to
10% of people with diabetes (ADA, 2015b). Type 1 diabetes is a condition of
complete insulin deficiency and requires insulin replacement for survival (Craig
et al., 2014). Onset of the disease is abrupt with the individual initially
presenting in an acutely ill state and oftentimes with a life-threatening condition
known as diabetic ketoacidosis (Fritsch et al., 2011) resulting from a buildup of
ketones in the bloodstream. Type 1 diabetes is further classified according to
pathogenesis with type 1A associated with an autoimmune destruction of
pancreatic beta cells and type 1B lacking the same (Canivell & Gomis, 2014). A
third subtype, latent autoimmune diabetes of adults (LADA), refers to an
autoimmune-based form of diabetes that occurs in adults aged 35 years or older
(Thomas & Philipson, 2015).
Type 2 Diabetes
In type 2 (formerly known as non–insulin-dependent or adult-onset) diabetes
mellitus, the pancreas secretes insulin but insulin resistance is present and the
amount of insulin may be insufficient, producing a chronic state of
hyperglycemia (ADA, 2015a). Type 2 accounts for 90% to 95% of all cases of
diabetes (ADA, 2015b) and typically occurs with increasing age and in people
who are obese (Ducloux et al., 2015). Onset of type 2 diabetes is gradual, and in
asymptomatic cases, the disease may go undetected for years (ADA, 2015a).
Historically, type 2 diabetes diagnoses were limited to adults. A marked increase
in prevalence of obesity in children and adolescents both in the United States
and globally has resulted in dramatic increases in type 2 diabetes for these age
groups (Pulgaron & Delamater, 2014).
Gestational Diabetes
Gestational diabetes is a transient form of diabetes mellitus that first appears
during pregnancy and typically requires no further treatment after delivery
(ADA, 2015a). Women with gestational diabetes are seven times more likely to
develop type 2 diabetes at some future point as compared to non–gestational
diabetes counterparts (Bellamy, Cases, Hingorani, & Williams, 2009).
Postpartum weight loss is associated with mitigating deterioration in glucose
metabolism and perhaps delaying a future diagnosis of type 2 diabetes (Ehrlich
et al., 2014).
Other Specific Types
The ADA (2015a) classifies other types of diabetes according to etiology.
Genetic defects of the beta cell include two subtypes: maturity-onset diabetes of
the young (MODY) and neonatal diabetes. MODY presents in people before age
25 as hyperglycemia due to impaired insulin secretion (ADA, 2015a). Neonatal
diabetes appears in the first 6 months of life and may be transient (ADA, 2015a).
Other etiologic-based conditions include those resulting from pancreatic trauma
or diseases (e.g., pancreatitis, cystic fibrosis, carcinoma), drugs (e.g.,
glucocorticoids, thyroid hormone), infections (e.g., congenital rubella), or
genetic syndromes (e.g., Down’s syndrome, Klinefelter’s syndrome) (Craig et
al., 2014).
Prediabetes
People with prediabetes are in an intermediate zone between normal blood
glucose levels and meeting diagnostic criteria for diabetes (Egan & Dinneen,
2014). These individuals present with impaired fasting glucose levels (100 to
125 mg/dL) and impaired glucose tolerance levels (140 to 199 mg/dL) and are at
risk for developing diabetes in the future (ADA, 2015a). Prediabetes is also
associated with obesity and increased risk for cardiovascular disease (ADA,
2015a). Lifestyle modifications such as increasing one’s physical activity level
and losing weight (Tabák, Herder, Rathmann, Brunner, & Kivimäki, 2012) and
eating a healthy diet (Ley, Hamdy, Mohan, & Hu, 2014) can delay or prevent
progression to type 2 diabetes.
Etiology
Type 1
The type 1 diabetes disease process is precipitated by an autoimmune response
whereby antibodies are produced that destroy pancreatic insulin-producing cells
known as beta cells (Canivell & Gomis, 2014). Etiological factors are
multifaceted and overlapping; however, specific roles of some remain unknown,
unclear, or controversial (Atkinson, Eisenbarth, & Michels, 2014; Canivell &
Gomis, 2014; Craig et al., 2014; Nielson, Krych, Buschard, Hansen, & Hansen,
2014). Current theories include etiological factors focusing on a strong genetic
susceptibility coupled with a variety of environmental factors thought to trigger
or drive the disease process. Three categories of environmental factors include
viral infections, infant diet, and exposure to microbial toxins. Some research
points to a clinically significant association between viral infections such as the
Enterovirus and the autoimmune response in type 1 diabetes (Yeung, Rawlinson,
& Craig, 2011). However, other studies have found no association (Canivell &
Gomis, 2014). Infant diet, specifically ingestion of cow’s milk; a short duration
of breast-feeding; and early introduction of gluten-based cereals are thought to
be inciting factors (Nielson et al., 2014). Exposure to microbial toxins in the
form of ingested nitrites and nitrates in food (e.g., processed meat products) and
water may also be triggering factors (Akerblom et al., 2002). Theories relating
the hygiene hypothesis (Liu, 2015) to the autoimmune response in diabetes are
emerging in the literature (Canivell & Gomis, 2014). The hygiene hypothesis
purports that improved hygiene practices are decreasing childhood exposure to
infectious agents and subsequent opportunities for immune system development
leading to a rise in autoimmune diseases (Liu, 2015). At the same time, exposure
to environmental pollutants (Janghorbani, Momeni, & Mansourian, 2014) and
vitamin D deficiency are also thought to play a role; however, evidence for the
same is weak (Canivell & Gomis, 2014).
Type 2
Beta cell malfunction in type 2 diabetes has a clear genetic element (Kahn,
Cooper, & Del Prato, 2014). Nevertheless, etiological factors related to age,
obesity, sedentary lifestyle (ADA, 2015a), and other lifestyle factors are
prevalent in the literature. Obesity affects metabolism by releasing certain
substances including nonesterified fatty acids (NEFAs) that are associated with
insulin resistance (Al-Goblan et al., 2014). From 1994 to 2010, the percentage of
adults with diagnosed diabetes who were obese increased from 34.9% to 56.9%
(Centers for Disease Control [CDC], 2015). Body fat distribution is also
associated with insulin resistance such that those with more abdominal fat are at
higher risk (Al-Goblan et al., 2014). Lifestyle choices such as prolonged
television watching (Grøntved & Hu, 2011) and habitual consumption of sugar
beverages (Imamura et al., 2015) are associated with new-onset diabetes. Finally,
the pathophysiology of obstructive sleep apnea may also play a part in abnormal
insulin metabolism (Morgenstern et al., 2014).
Children and Adolescence Incidence
The 2014 National Diabetes Statistics Report provides data based on 2009 to
2012 data (CDC, 2014a). Approximately 18,346 (ADA, 2015b) youth (rate of 22
per 1,000) (CDC, 2014a) are newly diagnosed with type 1 diabetes and 5,089
(ADA, 2015b) youth (rate of 10 per 1,000) (CDC, 2014a) with type 2 diabetes
annually. Non-Hispanic White youth had the highest rate of newly diagnosed
cases of type 1 diabetes (28 per 100,000), with type 2 being extremely rare in
this group of youth under age 10 (CDC, 2014a). The highest rates of newly
diagnosed type 2 cases occurred in American Indians/Alaskan natives (28 per
100,000), non-Hispanic African Americans (23 per 100,000), and Hispanics (17
per 100,000) (CDC, 2014a). For these groups, the incidence of type 2 diabetes
was greater than that of type 1.
Adult Incidence
In 2012, 1.7 million new cases of diabetes were diagnosed in people aged 20 or
older in the United States, with the majority of cases in the 45- to 64-year-old
age range (CDC, 2014a). The age-adjusted incidence of diabetes was slightly
higher for men (11.2 per 100,000) than for women (7.5 per 100,000) aged 20 to
74 years from 2007 to 2010 (Menke et al., 2014). Incidence for African
American, Hispanic, and Asian Americans is 2 to 3 times that of Caucasian
Americans (Kanaya et al., 2014).
Prevalence of Diabetes
Approximately 29.1 million Americans or approximately 9.3% of the population
now have diabetes (ADA, 2015b). Trends in prevalence of diabetes have steadily
risen such that there was a fourfold increase in the number of adults diagnosed
between 1980 and 2012 (CDC, 2014b), and if present trends continue, 1 in 3
adult Americans could have diabetes by 2050 (CDC, 2014b). Prevalence is
associated with race and ethnicity. For type 1 diabetes, non-Hispanic White
people have the highest while African Americans and people of Asian descent
have the lowest prevalence (CDC, 2014b). Prevalence of type 2 diabetes is
highest among American Indians/Alaskan natives (15.9%), followed by non-
Hispanic African Americans (13.2%), Hispanics (12.8%), Asian Americans
(9.0%), and non-Hispanic Caucasians (7.6%) (CDC, 2014a). Type 1 diabetes
occurs equally among males and females (Dabelea et al., 2014), with type 2
prevalence slightly higher for men (10.6%) than women (7.6%) (Menke et al.,
2014).
Signs and Symptoms
Classic symptoms of type 1 diabetes are those caused by hyperglycemia:
polydipsia (increased thirst), polyuria (frequent urination), polyphagia
(increased hunger), weight loss, and blurred vision (ADA, 2015a). Additional
symptoms of type 1 may include muscle cramps, irritability, emotional lability,
headaches, anxiety attacks, abdominal pain or discomfort, diarrhea or
constipation, and altered school and work behaviors. The individual may also
present with ketonuria. Ketones are waste products from the body’s breakdown
of fat for energy, high concentrations of which leads to DKA (ADA, 2015a).
DKA is an acute and potentially life-threatening condition that may appear as the
first symptom of previously undiagnosed diabetes (Demirci, Cosar, Ciftci, &
Sari, 2015). Clinical signs of DKA include dehydration; tachycardia (rapid heart
rate); tachypnea (rapid breathing); deep, sighing respirations with fruity breath
odor; nausea and vomiting; abdominal pain; and confusion or drowsiness
(Wolfsdorf et al., 2014). The number of deaths due to DKA decreased by 19.8%
from 1980 to 2009 (CDC, 2015).
Because onset of type 2 diabetes is gradual, levels of hyperglycemia may not
be severe enough to produce noticeable symptoms (ADA, 2015b). DKA is rare
in type 2 diabetes but can present concurrently with other conditions such as
infections, stroke, or pancreatitis (Demirci et al., 2015). However, symptoms
related to chronic hyperglycemia and subsequent systemic complications can
occur, for example, nephropathy, neuropathy, and retinopathy. These symptoms
may be present upon diagnosis if preceded by long periods of hyperglycemia
(Powers, 2008).
For people diagnosed with diabetes, longevity and quality of life are currently
better than in the past owing to improvements in insulin delivery regimens,
medication treatment for hyperlipidemia (i.e., excessive concentration of fats or
lipids in the bloodstream), and lifestyle modifications (Miller et al., 2012).
However, life expectancy remains lower overall for persons with diabetes as
compared to those who do not have the disease (Jørgensen, Almdal, &
Carstensen, 2013). Duration of diabetes and advancing age are predictive of
morbidity and mortality rates (Huang et al., 2013). From 2002 to 2011, mortality
rates declined for males with type 1 diabetes by 6.6% and for females by 4.8%
(Jørgensen et al., 2013). One study approximated an 11-year lower life
expectancy for men with type 1 diabetes and a 13-year lower life expectancy for
women (Livingstone et al., 2015). Children and young adults diagnosed with
type 2 diabetes lose approximately 15 years of remaining life expectancy
(Rhodes et al., 2012). For people with type 2 diabetes, mortality is associated
with onset and duration of complications, especially macrovascular-related
complications (ADA, 2015a).
Hypoglycemia
During the course of the disease, people with diabetes may experience periods of
hypoglycemia. Hypoglycemia or insulin shock is a condition of too much
insulin or oral hypoglycemic medication and not enough glucose in the
bloodstream (Powers, 2008). Risk factors for severe hypoglycemia include
hypoglycemia unawareness, longer durations of insulin replacement therapy,
cognitive deficits, age (e.g., early childhood or adolescence), and low
socioeconomic status or health literacy (Canadian Diabetes Association [CDA],
2013). Symptoms include, but are not limited to, trembling, sweating, hunger,
anxiety, confusion, drowsiness, dizziness, and difficulty concentrating (CDA,
2013). Mild hypoglycemia can be frightening to patients (ADA, 2015a) and lead
to less-than-optimal blood glucose control. Severe hypoglycemic states are
dangerous as they can result in falls or automobile accidents (ADA, 2015a). To
treat hypoglycemia, the individual needs to ingest some form of carbohydrate
that contains sugar, for example, orange juice, cola, and candy (CDA, 2013).
Some strategies that can prevent hypoglycemia are education, careful self-
monitoring of blood glucose levels, diet and exercise adjustments, and
medication modifications (Seaquist et al., 2013).
Hyperglycemia
Hyperglycemia is a condition of too little insulin causing abnormally high blood
glucose levels. If untreated, the patient is at risk for entering into a diabetic
coma. Mortality from hyperglycemia increases with age typically due to the
presence of a comorbid condition (myocardial infarction, cerebral vascular
accident, sepsis). Treatment depends on insulin to reverse metabolic
abnormalities and successful treatment of the comorbid conditions.
Hyperglycemia has a direct toxic effect on body tissues (Powers, 2008). Few
diseases have the same potential for damaging as many organ systems and
producing impairments as does diabetes (Fig. 19.2). In 1993, the National
Institutes of Health completed a 9-year study called the “Diabetes Control and
Complications Trial (DCCT).” Results of this study showed that intensive
insulin therapy slows the development of long-term complications (Nathan,
2014). Prior to this landmark study, it was unclear whether tight glucose control
was beneficial. Damage occurs in microvascular and macrovascular structures as
well as autonomic and peripheral nerves. These complications are acute and
chronic and affect the quality and duration of life for people with diabetes.
However, rates of major complications have substantially declined since 1990
(Gregg et al., 2014).
Figure 19.2 Impact of potential diabetes complications on body functions.
Macrovascular Complications
Hypertension
Stage 1 hypertension (HTN) is defined as blood pressure > 140/90 mm Hg
(Powers, 2008). Although it varies according to age, type of diabetes, racial and
ethnic groups, and obesity, HTN affects over two-thirds of people with type 2
(Ferrannini & Cushman, 2012) as well as young adults with type 1 diabetes who
have an underlying disorder such as nephropathy (ADA, 2015a). Those with
type 2 diabetes commonly develop HTN associated with central obesity (Lago,
Singhy, & Nesto, 2007). HTN combined with diabetes produces significant risk
for cerebrovascular disease, retinopathy, and end-stage renal disease (ADA,
2015a). The ADA recommends that people with diabetes should generally target
blood pressure goals at <140/90 mm Hg; however, lower targets (i.e., <130/80
mm Hg) may be appropriate for younger people (ADA, 2015a).
Cardiovascular Disease
People with diabetes have a higher risk for cardiovascular disease (CVD) and
CVD is a major cause of morbidity and mortality (ADA, 2015a; Wannamethee,
Shaper, Wincup, Lennon, & Sattar, 2011). Along with accelerated
atherosclerosis, typical cardiac risk factors (e.g., HTN, smoking, abnormal
cholesterol level, obesity, and inactivity) are common in people with type 2
diabetes (ADA, 2015a). Having diabetes for longer duration (Wannamethee et
al., 2011) and older age (Halter et al., 2014) further the risk of CVD for people
with diabetes. Thus, CVD is becoming more common in people with type 1
diabetes as these individuals are living longer (Melendez-Ramirez, Richards, &
Cefalu, 2010). Women with diabetes have a 40% greater chance of developing
CVD as compared to men (Peters, Huxley, & Woodward, 2014). Despite these
trends, the number of people with diabetes diagnosed with acute myocardial
infarction (MI) decreased by 67.8% from 1990 to 2010 (Gregg et al., 2014).
Cerebrovascular Disease
Similarly, accelerated atherosclerosis is an important risk factor for
cerebrovascular accident (CVA) as having diabetes doubles the risk of CVA and
1 in 8 to 9 cases of CVA is attributable to diabetes (Luitse, Biessels, Rutten, &
Kappelle, 2012). The rates of CVA in people with diabetes dramatically
decreased (58.9 fewer cases per 10,000 people) from 1990 to 2010 (Gregg et al.,
2014); however, women have a 27% greater relative risk as compared to men
(Peters et al., 2014). Despite a general decline in incidence, people with diabetes
and comorbid CVA have poorer long-term outcomes especially if they present in
a hyperglycemic state (Luitse et al., 2012). Dyslipidemia (i.e., elevated
cholesterol) and HTN (Powers, 2008) combined with lifestyle choices such as
smoking, inactivity, heavy alcohol consumption, and unhealthy diets increase the
risk of CVA (Luitse et al., 2012).
Peripheral Artery Disease

Peripheral artery disease (PAD) is a condition of decreased arterial blood flow
to the extremities, stomach, and kidneys (Deshpande, Harris-Hayes, &
Schootman, 2008; Neschis & Golden, 2015). PAD occurs at an earlier age and at
a faster rate in people with diabetes (Deshpande et al., 2008; McDermott, 2015)
with those in the age 40- to 49-year-old age range who have at least one other
risk factor for atherosclerosis at higher risk (Neschis & Golden, 2015). Severity
of PAD increases with duration of diabetes and presence of neuropathy
(Deshpande et al., 2008). Two categories of symptoms include intermittent
claudication (pain or discomfort during walking or exercise that resolves with
rest) and resting pain (Neschis & Golden, 2015). PAD predisposes people with
diabetes to impaired wound healing (Kolluru, Bir, & Kevil, 2012), tissue
hypoxia, and decreased mobilization of white blood cells to infected tissues
(Goodman, 2009), all of which contribute to diabetes being the leading cause of
nontraumatic lower extremity amputation (Powers, 2008).
Microvascular Complications
Diabetic Retinopathy
Retinopathy, or damage to the eye producing visual impairment, is a key
indicator of microvascular complications and as such of the impact of diabetes.
Diabetic retinopathy (DR) is the leading cause of blindness in adults aged 20 to
65 years (Ding & Wong, 2012). In 2011, approximately 19% of adults aged 18
years or older with diabetes had DR, which represented a 6% drop since 1997
(CDC, 2015). Of this same group, 15.6% of Hispanics, 17% of Caucasians, and
20.7% of African Americans with diabetes reported visual impairment (CDC,
2015). Similarly, females (19%) were more likely than males (16%) with
diabetes to report visual impairment (CDC, 2015). Mechanisms leading to visual
loss include macular edema, new vessel hemorrhage, retinal detachment, or
neovascular glaucoma (Fraser & D’Amico, 2015). Risk factors include longer
duration of diabetes, HTN, hyperglycemia, obesity, and puberty (Olafsdottir,
Anderson, Dedorsson, & Stefánsson, 2014). Most people with DR are
asymptomatic until late stages, and rate of progression can be rapid. Regular
screening and early diagnosis and treatment, including intensive insulin control
(McCulloch, 2015), can slow the disease process (Fraser & D’Amico, 2015).
Diabetic Nephropathy
Diabetic nephropathy results from structural and functional changes in the
kidney that lead to chronic kidney disease, the progression of which can be
slowed by optimal interventions (Bakris, 2015) of strict blood pressure and
glycemic control (Mailoux, 2015). Diabetes is the leading cause of end-stage
renal disease (ESRD), a condition of nonfunctioning kidneys. Twenty to forty
percent of people with diabetes were diagnosed with kidney disease (ADA,
2015a) with equal risk for type 1 and type 2 diabetes (Bakris, 2015). However,
the number of people with diabetes and comorbid ESRD dropped 28% from
1990 to 2010 (Gregg et al., 2014) likely due to advances in treatment. Family
history of diabetes, Black race, Mexican American or Pima Indian heritage, poor
blood glucose control, HTN, and cigarette smoking increase the risk for diabetic
nephropathy (Bakris, 2015). The presence of diabetic retinopathy can be used to
screen for and diagnose diabetic nephropathy (He et al., 2013). For people with
ESRD, renal replacement therapy in the form of dialysis or kidney transplant is
required for survival with transplantation offering the most optimal outcomes
(Mailoux, 2015).
Neurologic Complications
Peripheral Neuropathy
Up to 50% of people with long-standing type 1 or type 2 diabetes have damage
to peripheral nerves, a condition known as diabetic peripheral neuropathy
(DPN) (Powers, 2008; Tesfaye & Selvarajah, 2012) with painful DPN affecting
up to 25% of people with diabetes (Tesfaye et al., 2011). Risk factors include
duration of diabetes, poor glycemic control, and cardiovascular risk factors such
as abdominal obesity, hypertension, and hyperlipidemia (Singh, Kishore, &
Kaur, 2014; Jaiswal et al., 2013; Tesfaye & Selvarajah, 2012). Cigarette smoking
is also positively associated with DPN (Clair, Cohen, Eichler, Selby, & Rigotti,
2015). The most common symptoms include burning pain; stabbing, prickly, or
tingling sensation; or numbness and lack of protective sensation (ADA, 2015a;
Martin et al., 2014). Pain is worse at night, and symptoms are more common in
lower extremities than in upper (Tesfaye et al., 2011). Severe pain can interfere
with sleep, and subsequent daytime sleep debt and daytime pain negatively
impact daily functioning (Tessfaye et al., 2011). Intensive insulin therapy and
tight glycemic control reduce the risk of DPN (Martin, Albers, & Pop-Busui,
2014).
Autonomic Neuropathy
Damage to autonomic nerves that innervate organs leads to diabetic autonomic
neuropathy (DAN) that affects multiple systems in the body including the
cardiovascular, gastrointestinal, and genitourinary systems (ADA, 2015a). DAN
produces a wide spectrum of symptoms and onset is gradual and progressive
(Freeman, 2013; Vinik & Erbas, 2013). Cardiac autonomic neuropathy (CAN) is
a serious complication that affects up to one-third of people with type 2 diabetes
and increases risk of cardiovascular complications and sudden death (Vinik,
Erbas, & Casellini, 2013). Clinical manifestations of CAN include resting
tachycardia (rapid heart rate), orthostatic hypotension (i.e., drop in blood
pressure upon standing), exercise intolerance, greater risk for intraoperative or
perioperative cardiovascular incidents, increased incidence of myocardial
infarction (MI), and lower rate of post-MI survival (ADA, 2015a; Vinik et al.,
2013).
Autonomic neuropathy of the gastrointestinal system produces mild to
severe symptoms that occur more in people with long-standing diabetes and
poorly controlled hyperglycemia (Vinik & Erbas, 2013). Diabetes can cause an
increase or decrease in gastric motility. The latter leads to gastroparesis, a
condition of delayed gastric emptying (Freeman, 2013). Gastroparesis interferes
with the relationship between glucose absorption and insulin administration and
influences absorption of orally ingested medications (Freeman, 2013). Patients
may experience cramping, bloating, heartburn, loss of appetite, nausea,
vomiting, constipation, and diarrhea (Vinik & Erbas, 2013). Genitourinary
involvement produces bladder dysfunction in the form of decreased sensation
that results in decreased voiding frequency, urinary retention, and subsequent
increased risk of urinary tract infections (Vinik & Erbas, 2013). Sexual
dysfunction is common in men with diabetes over the age of 50 with erectile
dysfunction the most frequent issue (Isidro, 2012). Erectile dysfunction is
typically the first sign of DAN for this age group (Isidro, 2012).
Neurodegeneration
Recent literature provides evidentiary support for a link between type 2 diabetes
and neurodegenerative diseases such as Alzheimer’s disease (Verdile, Fuller, &
Martins, 2015) in that people with type 2 diabetes are at higher risk for
developing Alzheimer’s disease (Hao et al., 2015). Current thought postulates
that type 2 diabetes can exacerbate the neurodegenerative process but the exact
mechanism is not well understood (Verdile et al., 2015). Metabolic abnormalities
linked with diabetes (e.g., hyperglycemia, hypercholesterolemia) are also
associated with AD (Verdile et al., 2015). Although more research is needed, one
hypothesis points to shared genetic risk factors that cross both conditions (Hao et
al., 2015).
Other Complications
Diabetic Foot
Multiple sequelae of diabetes merge to make foot ulcers and infections common
leading to a condition known as diabetic foot. Etiological factors leading to foot
ulcers include repetitive stress on the skin with body weight and activity level
increasing the pressure (Noor, Zubair, & Ahmad, 2015). Autonomic neuropathy
results in dry, cracked skin making it more prone to ulceration and allowing
easier access for infection entry (Powers, 2008). Sensory polyneuropathy
diminishes pain and temperature perceptions causing lesions to go unnoticed
(Noor et al., 2015). PAD impairs blood supply needed for healing, and
hyperglycemia reduces host defenses (Powers, 2008). Trauma to the foot
combined with these risk factors results in lesions that can be slow to heal and
subject to a secondary infection (Noor et al., 2015). Infections may extend to
deeper soft tissues, bones, joints, and systemic circulation (Noor et al., 2015)
(Fig. 19.3). Management of mild infections involves antimicrobial therapy with
surgical intervention in the form of debridement or revascularization (Powers,
2008). Severe infections leading to gangrene tissues require limb amputation.
Risk factors for foot ulcers or amputation include being male; having diabetes >
10 years; smoking; and presence of PAD, peripheral neuropathy, nephropathy, or
foot structure abnormalities (ADA, 2015a; Powers, 2008). The CDC reports a
3.3/1,000 incidence rate of lower extremity amputation–related hospital
discharges for people with diabetes, which represents a 5.6/1,000 drop since
1988 (CDC, 2015). Despite this decline in incidence rates, the number of lower
extremity amputation–related hospital discharges for people with diabetes
increased by 24% from 1988 to 2009 (CDC, 2015). This increase is likely due to
the increasing numbers of people diagnosed with diabetes.
Figure 19.3 Diabetic ulcer of the heel. (From Goodheart, H. P. (2003).
Goodheart’s photoguide of common skin disorders (2nd ed.). Philadelphia, PA:
Periodontal Disease
Diabetes and periodontal disease have a bidirectional relationship (Chapple &
Genco, 2013) in that diabetes increases the risk of periodontitis especially for
people with type 2 diabetes (Bascones-Martinez et al., 2011) and periodontitis
negatively affects diabetes outcomes (Borgnakke, Ylostalo, Taylor, & Genco,
2013). It has been suggested that increased incidence of periodontal disease in
this population is due to inhibition of cellular mechanisms that destroy bacteria
in the mouth or to impaired functioning of reparative (i.e., wound-healing) cells
(Álamo, Soriano, & Pérez, 2011).
Diagnosis
Abnormal plasma glucose levels serve to screen for and diagnose both type 1
and type 2 diabetes (ADA, 2015a). The ADA (2015a) published threshold levels
for four types of blood glucose tests, any of which may affirm a diagnosis of
diabetes or prediabetes (Fig. 19.4). In 2009, the ADA added glycated
hemoglobin A1c (HbA1c) as a diagnostic indicator for diabetes (Atkinson et al.,
2014). An HbA1c level shows the average blood glucose concentration level over
a 3-month period (Atkinson et al., 2014) and is commonly used to screen for and
diagnose diabetes in adults. Some research has raised questions as to the validity
of HbA1c levels for diagnosing type 2 diabetes in children and adolescents (Buse
et al., 2013). The ADA acknowledges this limitation but still recommends using
HbA1c as a diagnostic indicator for children and adolescents (ADA, 2015a).
Fasting plasma glucose (FPG) levels are measured following a minimum of an
8-hour period of no caloric intake (ADA, 2015a). For the 2-hour plasma glucose
(2-h PG), blood glucose levels are checked following a 75-g oral glucose
tolerance test (OGGT). Finally, random plasma glucose (RPG) levels that exceed
the diagnostic threshold concurrent with presentation of hyperglycemic crisis or
classic symptoms indicate a clinical diagnosis of diabetes (ADA, 2015a). One
additional blood test result indicative of type 1 diabetes is the presence of
pancreatic autoantibodies; however, most laboratories are not equipped to
measure all autoantibodies and may produce false-negative results (Chiang,
Kirkman, Laffel, & Peters, 2014) (Table 19.1).
TABLE 19.1 American Diabetes Association Plasma Glucose Level

Diagnostic Thresholds
Note: American Diabetes Association (ADA). (2015a). Standards of medical care in diabetes—2015.
Diabetes Care, 38(Suppl. 1), S1–S90. Retrieved from
http://professional.diabetes.org/admin/UserFiles/0%20-
%20Sean/Documents/January%20Supplement%20Combined_Final.pdf.
Medical Management
Type 1
Management Regimens
There is no known cure for diabetes; therefore, medical management focuses on
developing individually tailored, culturally sensitive, and age and
developmentally appropriate care plans (Chiang et al., 2014). Management
regimens center on insulin replacement, glycemic control, assessment of and
prevention of complications, and attention to lifestyle and psychosocial issues.
The ADA recommends intense and ongoing diabetes self-management education
(DSME) to promote knowledge and skills necessary for effective glycemic
control and self-care (ADA, 2015b). DSME topics include medications, self-
monitoring of blood glucose levels (SMBG), physical activity, complications,
nutrition, risk reduction (e.g., foot care, smoking), and psychosocial issues
(Powers et al., 2015).
Insulin Replacement and Glycemic Control

Until insulin was characterized and manufactured in 1921, diabetes was a death
sentence. Insulin replacement uses combinations of rapid-acting (e.g., Humalog),
short-acting (Humulin S), intermediate-acting (Humulin I), and long-acting
(Lantus) insulin that can be delivered by syringe, pen, continuous subcutaneous
insulin infusion (CSII) or insulin pump, or inhalation (Leong, Velusamy, &
Choudhary, 2014). For children, the goal of insulin replacement is to achieve as
near-normal glycemic control as possible as indicated by HbA1c levels targeted
at <7.5% for all pediatric age groups (Chiang et al., 2014). Insulin injection
schedules depend on the individual’s age, compliance level, and severity of
disease, with the ADA recommending three or more injections of prandial
(rapid-acting or mealtime) insulin and one to two injections of basal (long-
acting) insulin daily (Chiang et al., 2014). Approximately 40% of people with
type 1 diabetes use CSII (Pickup, 2011), advantages of which include the ability
to alter insulin infusion to meet physiological requirements, provide more
precise insulin delivery, and be connected to continuous glucose monitoring
sensors (Leong et al., 2014). Inhaled insulin advantages include simple delivery
and rapid action; however, concerns related to precision of dose and secondary
effects on lungs remain (Leong et al., 2014).
Surgical options include pancreatic transplants or pancreatic islet cell
transplants. Pancreatic transplants occur in one of three categories: simultaneous
kidney/pancreas transplant (SKP), pancreas after kidney transplant (PAK), or
pancreas alone transplant (PAT) (Gruessner, Sutherland, & Gruessner, 2012).
Long-term follow-up studies show positive trends in pancreatic function (i.e.,
insulin secretion) that have steadily improved over time (Gruessner et al., 2012)
and improvements in glycemic control following all three categories of
transplants (Gruessner et al., 2012; Lehman et al., 2015). The benefits of being
free of insulin dependency and avoiding secondary complications of diabetes
outweigh transplant surgical risks (Gruessner et al., 2012). Although currently
considered experimental (Atkinson et al., 2014), islet cell transplantation may be
a less-risky option for those who are poor candidates for organ transplantation
(Chiang et al., 2014). Short-term outcomes are more favorable than long-term as
patients are insulin free for 3 years on average (Barton et al., 2012).
Management of Lifestyle and Psychosocial Issues

Attention to lifestyle factors such as diet and physical activity levels/exercise
along with psychosocial issues is important to the management of type 1
diabetes. People with type 1 diabetes should participate in nutrition therapy
comprised of education related to how food impacts blood glucose, how to avoid
hypoglycemia or hyperglycemia resulting from food-exercise-insulin
interactions, and how to adapt food-related plans to a various situations (Chiang
et al., 2014). The ADA has published nutrition guidelines (ADA, 2015a) that
recommend a healthy, balanced diet and monitoring intake of carbohydrates for
people with type 1 diabetes (Chiang et al., 2014). Long delays between meals
should be avoided, as not eating in a predictable pattern consistent with one’s
insulin regimen may cause hypoglycemia. Frequent, small snacks at the time of
peak insulin action should also be taken to avoid hypoglycemia. The many
health benefits associated with increased physical activity and exercise along
with recommendations that all children engage in 60 minutes of physical activity
per day apply to people with type 1 diabetes (Chiang et al., 2014). However, for
those with type 1 diabetes, exercise may induce states of hyperglycemia or
hypoglycemia and thus requires diligence in frequently monitoring blood
glucose levels and adjusting insulin dosages and/or food intake accordingly
(Chiang et al., 2014). Finally, assessment and management of psychosocial
issues (e.g., depression, anxiety related to hypoglycemia or hyperglycemia,
eating disorders, family tensions) are important to ensure that individuals with
type 1 diabetes can responsibly and effectively manage their diabetes care
(Chiang et al., 2014).
Type 2
Management of type 2 diabetes focuses on supporting lifestyle changes related
to nutritional strategies, exercise programs, and prevention and/or treatment of
complications, along with glycemic control (Chatterjee & Davies, 2015). As
with type 1 diabetes management, patient education plays a crucial role in
effectively managing type 2 diabetes (Coppola, Sasso, Bagnasco, Giustina, &
Gazzaruso, 2015). Medical nutritional therapy in the form of controlling the
amount and types of fats, carbohydrates, and proteins is recommended for
weight reduction and prevention or reduction of atherosclerotic vascular disease
(ADA, 2015a). A Mediterranean-style diet (i.e., consumption of monosaturated
and omega-3 fatty acids, fresh fruits and vegetables, high fiber, and vegetable
protein) can reduce insulin resistance in people with type 2 diabetes (Esposito &
Guigliano, 2014).
The health-promoting benefits of increased physical activity and regular
exercise are well documented. The ADA recommends a minim of 150 minutes
of moderately intense exercise physical activity or exercise per week (ADA,
2015a). For patients with type 2 diabetes, regular exercise reduces insulin
resistance, promotes cardiovascular health, and prevents/improves obesity,
anxiety, and depression (Inzucchi et al., 2012).
The targets for glycemic control are HbA1c levels of ≤7.5% for children and
adolescents with type 2 diabetes and ≤7.0% for most adults (ADA, 2015a). A
more stringent goal (i.e., <6.5%) may be recommended for certain individuals
(e.g., those with recent diagnosis, long life expectancy, or those without
cardiovascular disease) if the goal can be met without risk of hypoglycemia
(ADA, 2015a). Less stringent targets (≤8.5%) may be indicated for older adults
who have advanced complications, are in otherwise poor health, or have shorter
life expectancy (Chiang et al., 2014). When diet and exercise are ineffective in
controlling blood glucose levels, individuals take oral glucose-lowering agents.
Several classes of these medications are prescribed and dosages individualized
for patients. Metformin (Glucophage) is typically the first line of intervention
(Chatterjee & Davies, 2015) and is used to decrease liver output of glucose
(ADA, 2015a). Glimepiride (Amaryl) and repaglinide (Prandin) stimulate insulin
secretion and pioglitazone (Acta) increases sensitivity of skeletal muscle tissues
to insulin (ADA, 2015a). Individuals with type 2 diabetes may initially respond
to oral hypoglycemics but then not respond well after years of therapy. This
could be the result of decreased compliance with diet and exercise programs,
progression of pancreatic failure to produce insulin, complications from
comorbid medical conditions or medications, or development of tolerance to
medications. Insulin therapy is indicated at that point.
Self-Monitoring of Blood Glucose Levels
SMBG is crucial for both type 1 and type 2 diabetes. Self-monitoring requires
that individuals with diabetes take active control of their own health and well-
being, increases understanding of the impact of diet and physical activity, and
allows for more immediate adjustments to insulin therapy in order to prevent
hypoglycemia (Chiang et al., 2014). People with type 1 diabetes should monitor
their blood glucose levels up to 6 to 10 times per day including but not limited to
before and after meals and exercise, before bedtime, when they suspect low
blood glucose levels, and oftentimes prior to driving (Chiang et al., 2014).
People with type 2 diabetes who are insulin dependent should monitor before
breakfast, dinner, and bedtime, with the goal of monitoring being to avoid
hyperglycemia.
Technology plays an important role in SMBG. Portable glucose meters are
available that take blood for sampling, give a digital readout of glucose levels,
and have a computerized memory for record keeping. Newer intermittent
glucose monitoring devices can be preprogrammed to calculate rapid-acting
insulin doses (Cavan et al., 2014) and connect to insulin pumps and mobile
phones (Leong et al., 2014). The use of mobile phone apps to assist with
diabetes management is becoming more prevalent, and research shows
promising trends in some patient outcomes, for example, self-efficacy and self-
management behaviors (Holtz & Lauckner, 2012). However, further research is
needed to establish evidence as to the impact of their usage on glycemic
outcomes especially for people with type 1 diabetes (Peterson, 2014).
Performance
Body Functions
Diabetes and its subsequent complications potentially affect most body
functions. Specifically, diabetes affects mental functioning in several ways due
to both acute and chronic complications. Acutely, insulin and blood glucose
levels influence the level of arousal. Symptoms of hypoglycemia include
drowsiness and fatigue. Variations in blood glucose levels can also cause mood
swings and irritability.
Chronic complications affect those with both type 1 and type 2 diabetes.
Adults with type 1 diabetes are prone to diminished intelligence, attention,
psychomotor speed, cognitive flexibility, and visual perception (McCrimmon,
Ryan, & Frier, 2012). Adults with type 2 diabetes are more prone to learning and
memory deficits (McCrimmon et al., 2012) and at higher risk for developing
Alzheimer’s disease (Verdile et al., 2015). Emotional regulation is also impacted.
Depression and diabetes are common comorbid conditions (Holt, de Groot, &
Hill Gordon, 2014), especially for females with type 1 diabetes over the age of
40 (Navmenova, Mokhort, & Makhlina, 2014), and youth (Walders-Abramson,
2013) and adults with type 2 diabetes (Saddiqui, 2014). Some research points to
stresses related to living with diabetes as partially responsible for this
comorbidity (Holt et al., 2014) in that the presence of complications increased
the risk for depression (Saddiqui, 2014). At the same time, while other research
showed an increase prevalence of mild to moderate depression in people with
diabetes, there was no correlation between a diagnosis of depression and disease
duration and presence of complications (Trento et al., 2012).
Sensory functions are affected by retinopathies and neuropathies.
Retinopathy visual impairment can ultimately lead to blindness (Fraser &
D’Amico 2015). Neuropathy affects sensory functioning such that people with
diabetes often experience pain that may be severe (Tesfaye et al., 2011) along
with diminished touch and pressure sensations in the upper and lower
extremities (Noor et al., 2015; Shah, Clark, McGill, & Mueller, 2015). In one
study, people with diabetes had greater prevalence of decreased shoulder range
of motion and muscle strength and decreased grip and pinch strength than those
without diabetes (Shah et al., 2015). Autonomic neuropathy leads to
compromised functioning of cardiovascular (Freeman, 2013), gastrointestinal
(Vinik & Erbas, 2013), genitourinary (Isidro, 2012), and integumentary systems
(Powers, 2008).
Occupational Performance
Effective functioning in multiple occupational performance areas impacts the
disease process and subsequent quality of life and is also impacted by living with
diabetes. Functioning in specific activities of daily living (ADLs) that affect
disease outcomes includes personal hygiene and grooming (i.e., personal
hygiene, dental care) and personal device care (i.e., blood glucose meters, insulin
syringes). Risk of diabetic foot complications can be reduced by daily inspection
and care of feet. The link between periodontal disease and diabetes (Álamo et
al., 2011) makes proper dental hygiene imperative. The disease process also
impacts ADL functioning as approximately 10% of adults age 60 years or older
reported needing assistance with ADLs in 2011 (CDC, 2015). Lower extremity
neuropathies affect functional mobility. Percentages of adults over age 18 living
with diabetes reported difficulty with the following functional mobility tasks in
2011: 37% walking a quarter mile, 28% climbing 10 steps, 38% standing for 2
hours, and 44% stooping, bending, or kneeling (CDC, 2015). Sexual activity
dysfunction is reported for women and men. Women with diabetes are more
likely to experience problems with sexual functioning and satisfaction (Copeland
et al., 2012) and men problems with low desire and erectile dysfunction (Isidro,
2012).
Successful management of multiple instrumental activities of daily living
(IADLs) tasks affects the diabetes disease process and is affected by the same.
Almost 16% of adults over age 60 with diabetes report needing assistance with
IADLs (CDC, 2015). Meal preparation must meet dietary guidelines. Tasks that
require cognitive functioning (e.g., managing medication routines, financial
management) may be difficult due to fluctuations in blood glucose levels.
Neuropathies can impact the ability to use communication devices (e.g., writing
or typing) or perform home management tasks (e.g., meal preparation).
Retinopathy, neuropathy, and fluctuating blood glucose levels may make driving
and community mobility difficult or prohibitive. Health management and
maintenance are perhaps the most important IADL tasks for people with diabetes
(Powers et al., 2015). People with diabetes must develop and adhere to effective
routines to manage the multifaceted care demands (Fritz, 2014; Pyatak, 2011),
including dietary and nutritional issues (Ley et al., 2014), physical fitness
(Balducci et al., 2014), medication management (Strom Williams et al., 2014),
and reduction of health risk behaviors such as smoking cessation (Clair et al.,
2015).
People with diabetes are at higher risk for sleep problems than those without
diabetes (Plantinga, Rao, & Schillinger, 2012) that can interfere with glycemic
control and limit quality of life (Surani, Brito, Surani, & Ghamande, 2015).
Problems include nocturia (nighttime urination), restless leg syndrome, apnea,
and inadequate amount of sleep (Plantinga et al., 2012). Education, work, play,
and leisure participation routines must incorporate diabetes self-management
including SMBG and adhering to insulin regimens. This may be challenging for
children and young adults with type 1 diabetes whose activity levels directly
influence blood glucose levels and insulin needs (Pyatak, 2011) and for whom
peer acceptance and pressures are issues (Chiang et al., 2014). Finally, social
participation may be limited by fatigue and decreased mobility (Schmader,
2001); however, one study in China showed a positive association between
cognitive social capital (i.e., support, reciprocity, cohesion) and quality of life for
people with type 2 diabetes (Hu et al., 2015).
CASE-STUDY 1
Jalen is a 15-year-old African American male diagnosed with obesity
(weight of 270 lb) and HTN during a mandatory presports physical exam.
The physician referred him to an internal medicine doctor for a more
thorough exam. The results of this checkup confirmed the previous
diagnosis of HTN (BP reading of 160/100 mm Hg). Blood test results
produced additional diagnoses of hyperlipidemia (total cholesterol level of
260 mg/dL) and type 2 diabetes (FPG = 138 mg/dL). His physician
prescribed Lipitor (atorvastatin; cholesterol lowering), 20 mg per day;
Maxzide (hydrochlorothiazide; anti-HTN), 50 mg twice per day; and
Glucophage (metformin; glucose lowering), 500 mg twice per day
medications. He further recommended that Jalen routinely check his blood
glucose levels and lose weight. Jalen met with a nurse and a dietician for a
series of intensive training sessions for diabetes self-management education
(DSME). Training topics centered on medications, SMBG, physical
activity, complications, nutrition, risk reduction (e.g., foot care, smoking),
and psychosocial issues (Powers et al., 2015). Jalen attended some but not
all of the scheduled sessions. During several of the training sessions, he
appeared to be drowsy and occasionally drifted off to sleep. When asked
about his apparent lack of interest in the education, Jalen stated that having
diabetes is no big deal for his mother and grandmother, so it will be no big
deal for him.
Jalen’s mother is single and works two part-time jobs to support him
and his older sister and younger brother. Together with his maternal
grandparents, they live in a subsidized, three-bedroom apartment in a large,
inner-city urban neighborhood. Family history is significant as both Jalen’s
mother and maternal grandmother are obese and have a long-standing
history of HTN and type 2 diabetes. Jalen’s grandmother is living with
several complications of diabetes, including retinopathy and lower
extremity PAD that causes pain and diminished sensation. She has ESRD
and receives hemodialysis treatment 3 d/wk. Due to his own poor health
issues, Jalen’s grandfather can provide only minimal assistance with his
wife’s care. Jalen’s mother is overwhelmed with work and parental care-
taking responsibilities. She is exhausted by the end of the day, so meals
consist of fast or processed foods.
Recently, Jalen’s school attendance has been erratic as he often skips to
hang out with his friends. When not with his friends, Jalen isolates himself
in his bedroom playing computer games and listening to music through
earbuds. Lacking parental guidance, Jalen typically eats convenience foods
and drinks sugary soft drinks. His medication compliance is poor as he
often forgets to take his pills or does not take the prescribed dosages. Jalen
continues to smoke cigarettes (1 pack/d) and drink alcohol with his friends
(3 to 4 d/wk). His mother is worried about him because he seems sad all the
time.
Jalen presently shows no signs of systemic complications from
hyperglycemia. However, his weight, HTN, smoking, alcohol abuse, and
poor compliance with medication regimen place him at high risk for rapid
development of the same (e.g., cardiovascular disease, peripheral artery
disease, peripheral neuropathies, and end-stage renal disease). His lack of
parental support and adult supervision contributes to a high risk of
morbidity and mortality from diabetic complications.
CASE-STUDY 2
Tammy is a 39-year-old Caucasian female diagnosed with type 1 diabetes at
age 11. Her medical history includes long-standing history of HTN and
hyperlipidemia, and she was diagnosed with ESRD at age 36. Since then,
she has been on a three times per week regimen of outpatient hemodialysis.
Tammy has decreased vision due retinopathy, and she complains of leg pain
due to lower extremity peripheral neuropathy. Six months ago, Tammy had
an acute myocardial infarction. Since then, she complains of fatigue and
lack of interest in participating in activities that she used to enjoy.
Tammy’s medication regimen is extensive. She injects 10 units of
Lantus (long-acting insulin) BID and Humulin (short-acting insulin) three
to four times per day as indicated by SMBG results. Tammy takes 20 mg of
Lipitor (cholesterol lowering), 5 mg of amlodipine (anti-HTN), 1
Nephrocap (prescription multivitamin), 17 g MiraLax (laxative) dissolved
in water, 325 g ferrous sulfate (anemia) twice a day, and 3,667 PhosLo
(phosphate binder) three times per day with meals. Calcium binds
phosphorus with ingested foods to prevent phosphorus from going into the
body; normal kidney function eliminates extra phosphorus in the body.
Tammy must also adhere to fluid and diet restrictions. Fluids are
restricted to 1,800 mL/d. Dietary restrictions consist of limiting phosphorus
(i.e., dairy foods, cola), potassium, salt, and carbohydrates. Tammy has a
history of poor adherence to these restrictions, especially the fluid
restrictions as she typically starts hemodialysis in a state of fluid overload.
This raises her blood pressure, and the dialysis staff has difficulty removing
the excess fluid without risks of cramping and hypotension. She
communicates little information to the staff or her physician during dialysis,
spending most of the time trying to sleep.
Tammy lives with her parents who transport her to dialysis. She has
never been married and is currently unemployed, although her employment
history consisted of several part-time jobs, primarily as a retail clerk. She
has two older sisters and one younger brother, all of whom are married and
have children of their own. Tammy’s social interactions are centered on her
family as she enjoys spending time with her nieces and nephews and often
attends their sporting events with her parents. She has few friends of her
own age as her past friends are busy with family responsibilities of their
own and Tammy finds that she has little in common with them now.
Tammy’s hobbies consist of watching TV and occasionally knitting.
Tammy is right-hand dominant and receives dialysis through her LUE.
She has moderate pain and numbness in her LUE because of an ischemic
neuropathy that is the result of decreased blood flow because blood is
diverted to her dialysis access site. She has full bilateral upper extremity
active range of motion but decreased peripheral sensation (tactile, pain, and
temperature). Tammy has a moderate decrease in bilateral grip and pinch
strength, with her left side weaker than her right. Tammy recently stepped
on a tack, did not feel it, and subsequently developed an infection that is not
responding to antimicrobial medication. Her physician told her that she will
likely have to undergo a below-knee amputation.
Tammy’s parents transport her to and from dialysis as declining vision
and lower extremity neuropathy deem her unsafe to drive. She ambulates
and transfers independently but with increasing difficulty. Tammy is
independent in all activities of daily living (ADLs) and prefers to prepare
her own meals rather than eat food her mother prepares. She lives in a
double-story house with a half bathroom on the main level and her bedroom
and full bathroom on the upper level. Climbing the steps is becoming
difficult such that Tammy only does so to go to bed at night.
Tammy is moderately but increasingly disabled by the physical and
emotional ramifications of diabetes, retinopathy, neuropathies, renal failure,
and cardiac status. This affects her roles as daughter, sister, and aunt. Her
parents are concerned about Tammy’s declining health and her future ability
to care for herself as they are aging and dealing with their own health
issues. While she can currently access her home environment, she may not
be able to do so in the near future. Socially, Tammy is becoming withdrawn
and disinterested in participating in the few previous activities she used to
enjoy. The context of her occupational performance further reflects the
impact that diabetes has on decreasing her quality of life.
RESOURCES
American Diabetes Association (ADA)
www.diabetes.org) Centers for Disease Control (CDC)
www.cdc.gov) The Mayo Clinic
http://www.mayoclinic.org/diseases-conditions/diabetes/basics/definition/con-
20033091
National Institute of Diabetes and Digestive and Kidney Disease (NIDDK)
www.niddk.nih.gov) National Kidney Foundation (NKF)
www.kidney.org)
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C H A P T E R
20 Acquired Brain Injury
Gerry E. Conti
KEY TERMS
Anterograde amnesia Aspiration
Benign tumor
Coma
Contrecoup injury Decerebrate rigidity Decorticate rigidity Dysautonomia
Executive function Heterotopic ossification Hydrocephalus
Malignant tumor
Metastasis
Mild TBI
Moderate TBI
Retrograde amnesia Severe TBI
It had been a hot summer day, the work was hard, and J.D.’s construction job
required every ounce of his strength and endurance. He’d missed lunch and was
roofing in the heat as the final part of his day, the tar radiating heat upward while
the sun beat down on this back. At last it was over. J.D. picked up Kathy, his
girlfriend of 4 years, and headed for the beach. Five hours and many beers later,
they sped home on familiar secondary roads. He negotiated the first part of an S-
curve fine, but his reflexes were too slow to manage the second curve when a
rabbit ran into the road. Overcompensating, he lost control of the car, slamming
it up against a tree. In an instant, his life changed. Kathy was killed. J.D., age 20,
survived. At the hospital, he was diagnosed with a traumatic brain injury.
Acquired brain injury (ABI) is defined by the World Health Organization as
“damage to the brain, which occurs after birth and is not related to a congenital
or a degenerative disease.” These impairments may be temporary or permanent
and cause partial or functional disability or psychosocial maladjustment (Brain
Trauma Foundation, 2007). ABI includes the conditions of traumatic brain injury
(TBI), brain tumors, and cerebrovascular accidents (CVA). This chapter will
address TBI and brain tumors; CVA is discussed in a separate chapter.
Traumatic Brain Injury
TBI is defined by the Diagnostic and Statistical Manual of Mental Disorders
(American Psychiatric Association, 2013) as a mild, moderate, or severe
brain trauma with specific characteristics that include at least one of the
following: loss of consciousness, posttraumatic amnesia, disorientation and
confusion, or, in more severe cases, neurological signs (e.g., positive
neuroimaging, a new onset of seizures or a marked worsening of a preexisting
seizure disorder, visual field cuts, anosmia, hemiparesis). To be attributable to
TBI, the neurocognitive disorder must present immediately after the brain injury
occurs or immediately after the individual recovers consciousness after the
injury and persist past the acute post-injury period.
TBI therefore involves a complex matrix of physical, cognitive, and
neurobehavioral changes that may have a lifetime effect on a person’s ability to
participate in necessary and preferred occupations. Many functions are
compromised, including the ability to produce coordinated movement, speak,
remember, reason, and alter behavior in response to the environment (Wehman et
al., 2003a). The combination of these changes makes TBI a major public health
problem (Rutland-Brown, Langlois, Thomas, & Xi, 2006).
The extent of disability is typically identified within 48 hours of medical
evaluation and is based on loss of consciousness or coma, posttraumatic
amnesia, and disorientation and confusion at initial assessment (American
Psychiatric Association, 2013). The following definitions will be used for this
chapter.
Mild TBI: Loss of consciousness of <30 minutes, posttraumatic amnesia of
<24 hours and disorientation and confusion, and a Glasgow Coma Scale
(GCS) score of 13 to 15.
Moderate TBI: Loss of consciousness of 30 minutes to 24 hours,
posttraumatic amnesia of 24 hours to 7 days, and a GCS score of 9 to 12.
Severe TBI: Loss of consciousness of more than 24 hours, posttraumatic
amnesia of more than 7 days, and a GCS score of 3 to 8 (American
Psychiatric Association, 2013).
Mild TBI represents 80% of all brain injury, and about 85% of these people will
recover without intervention over a 3-month period (Greenwald & Rigg, 2009).
Persons with mild TBI typically report symptoms such as headaches, dizziness,
fatigue, visual disturbance, memory, and executive function difficulties during
the first week following injury. However, for some individuals, these difficulties
persist from 3 months to a lifetime, causing significant distress and disruption of
daily activities (Ponsford, Willmott, & Rothwell, 2000).
The onset of TBI is sudden, following a single-incident neurologic insult,
and results in both primary and secondary brain damage. Primary brain damage
may be focal (localized) or diffuse and is created by direct impact, acceleration,
deceleration, and rotation of the brain; by intrusion into the brain by a
penetrating object, such as a gunshot; or by blast waves from an explosion
(Maas, Stochetti, & Bullock, 2008). Focal lesions are limited in scope and are
associated with direct impact of short duration such as occurs with gunshot.
Diffuse lesions occur throughout multiple brain areas and may result from an
explosion, motor vehicle accidents, or sport collisions. Diffuse axonal injuries
(DAI) may occur with head collisions at about 15 miles per hour or greater
(Meythaler, Peduzzi, & Eleftheriou, 2001). DAI therefore can occur with high-
speed running collisions with others, as in football, soccer, or hockey, as well as
with higher-speed motor vehicle accidents. Motor vehicle accidents typically
result in both coup and contrecoup injuries. With these injuries, direct damage is
incurred as the cerebrum rotates on the more stable brainstem while accelerating
from the force of impact. The cerebrum strikes the skull (coup), and then
reaccelerates in the opposite direction to strike the skull at an opposite location
(contrecoup). This continues until the force of impact has been absorbed (Drew
& Drew, 2004).
The mechanism for DAI is stretching and shearing of brain cell axons, and
typically results in profound coma and a poor outcome (Maas et al., 2008).
Injury to the tracts leading from the hypothalamus and/or pituitary stalk results
in medical complications of hyperventilation, hormonal changes, electrolyte
disturbances of salt and water, altered temperature regulation, and dysfunctional
awareness of hunger (Adams, Graham, & Murray, 1982; Dawodu, 2005;
Meythaler et al., 2001). DAI is characterized by small lesions in and shearing of
white matter tracts of the cerebral cortex, such as the sensory and motor cortices
and the frontal lobe, and the corpus callosum, brainstem, and cerebellum (Maas
et al., 2008). Common cognitive deficits associated with these structures include
deficits in working memory and processing speed and memory, as well as
limited planning and executive functions (Meythaler et al., 2001; Salmond &
Sahakian, 2005). A common motor deficit is difficulty with bilateral integration
due to callosal damage, which is frequently disrupted in TBI (Hulkower, Poliak,
Rosenbaum, Zimmerman, & Lipton, 2013).
Secondary damage, with differing pathophysiological mechanisms, occurs
within hours and days of impact. Factors leading to secondary damage may
include inflammatory responses, raised intracranial pressure, and decreased
cerebral blood flow or ischemia (Maas et al., 2008). Increased intracranial
pressure results in swelling, which cannot be accommodated within the rigid
structure of the skull. Ischemia occurs when blood vessels can no longer provide
sufficient blood to the brain.
Medical complications are frequent and impair recovery following TBI.
Following a high-impact TBI, such as occurs with a motor vehicle accident,
persons may have single or multiple seizures (Frey, 2003), and hydrocephalus
(De Bonis, Pompucci, Mangiola, Rigante, & Anile, 2010). Fifty percent of
people with severe TBI have extremity injuries including lacerations and
fractures (Probst et al., 2009), resulting in casting of extremities. The incidence
of deep vein thrombosis following TBI is up to 54% (Cifu, Kaelin, & Wall,
1996; Probst et al., 2009; Rimel & Jane, 1983). Systemic complications may
include the cardiovascular, respiratory, immunological, hematological, and
endocrinological systems (Wijayatilake, Sherren, & Jigajinni, 2015). As a result
of respiratory dysfunction, nasal intubation or tracheostomy may be required,
and the person is at risk for pneumonia. Neurogenic bowel and bladder disorders
require catheterization, while limited body mobility requires close monitoring to
avoid the development of decubiti (Dijkstra, Kazimier, & Halfens, 2015).
Etiology
The three leading causes of TBI are falls, motor vehicle accidents, and violence.
Falls are the most common cause of TBI, but motor vehicle accidents are the
most common cause of severe TBI. Children below the age of 5 and elderly
adults over 85 years are the most commonly seen groups with TBI in hospital
emergency departments (Gordon et al., 2006). According to a major statewide
study from 2003 to 2006, males accounted for about 61% of all motor vehicle
accidents, both with and without the presence of alcohol or substance use
(Rochette, Conner, & Smith, 2009). Among children involved in motor vehicle
accidents resulting in moderate to severe injury, half were unrestrained at the
time of injury (National Highway Traffic Safety Agency, 2006). In the United
States, survivors of violence, the second major cause of TBI, are more likely to
be men, single, unemployed, and from a minority background (Bogner,
Corrigan, & Mysiw, 2001; Bushnik, Hanks, Kreutzer, & Rosenthal, 2003).
Severity of injury is related to cause. Surviving vehicle crash victims tend to
be injured more severely than do survivors of either falls or violence and to have
additional injuries such as long bone fractures and plexopathies; falls are more
often associated with mild injury. Intoxication, which is present in one-third to
one-half of individuals at the time of injury, and substance use are significantly
negatively correlated with outcome. Persons who were intoxicated when injured
also tend to be hospitalized longer and have greater severity of injury, greater
incidence of death, and a lower cognitive status at the time of discharge, as well
as greater periods of postacquired amnesia (Bogner et al., 2001; Corrigan, 1995;
Cummings, Rivara, Olson, & Smith, 2006; Cunningham, Maio, Hill, & Zink,
2002). People injured by violence tend to have more severe injuries and poorer
community reintegration after injury (Gordon et al., 2006).
The great incidence of TBI around the world has led the World Health
Organization to refer to TBI as a “silent epidemic” (Binder, Corrigan, &
Langlois, 2005). An estimated 1.7 million Americans sustain a TBI each year. Of
these, about 52,000 will die, 275,000 will be hospitalized, and between 80,000
and 90,000 persons will experience lifetime disability (American Psychiatric
Association, 2013).
The incidence of disability increases with the severity of brain injury.
Permanent disability occurs in about 10% of those with mild TBI, 66% of those
with moderate TBI, and 100% of those with severe TBI (Jallo & Narayan, 2000).
Additionally, age, gender, and ethnicity affect the incidence rate. At greatest risk
for injury are young men between the ages of 15 and 24, who are twice as likely
as women of the same age to sustain a TBI (American Psychiatric Association,
2013). It is not known, however, whether gender affects the severity of and
outcome from TBI (Ragnarsson, 2006). Age groups that show an increased
incidence of TBI include adults older than 75 years and children below the age
of 5 (Centers for Disease Control and Prevention, 2001). A review of current
evidence suggests that injury in older adults may result in greater impairment
and more limited recovery (Ragnarsson, 2006). Inner-city environments have
higher incidence rates (Bruns, 2003), with persons of American Indian/Alaskan
and African American ethnicity having the highest rates from TBI of all races
(Corrigan, Selassie, & Orman, 2010). It is uncertain whether this incidence is
due to issues related to socioeconomic status or to lack of medical and
rehabilitation opportunities (Ragnarsson, 2006).
The costs of TBI, both individually and for society, are staggering. The
estimated economic cost of TBI in the United Stated in 2010, including both
direct and indirect health care costs, was $76.5 billion (Centers for Disease
Control and Prevention, 2014). As only 20% to 50% of TBI survivors are
employed (Injury, 1999), an additional $1 billion annually may be incurred due
to lost wages, lost income taxes, and increased public assistance (Johnstone,
Mount, & Schopp, 2003). However, researchers have used different economic
analysis methodologies, and historical results have been inconsistent
(Humphreys, Wood, Phillips, & Macey, 2013). Nevertheless, it is clear that TBI
results in significant costs to society, the individual, and the family during the
acute phase of recovery and through lifetime costs.
Signs and Symptoms
People with TBI experience a wide range of deficits, depending on the location
and severity of their injuries. The following is a description of the medical,
motor, cognitive, psychosocial, and cranial nerve signs and symptoms associated
with TBI.
Medical Complications
Seizures are a frequent medical complication of moderate or severe TBI
(Ragnarsson, 2006) and are typically classified as immediate, early, or late
seizures, depending on whether they occur within 24 hours, 1 week or after 1
week, respectively (Najafi, Tabesh, Hosseini, Akbari, & Najafi, 2015). Risk
factors for seizures include the severity of the injury, time since injury,
decompressive craniectomy, and brain contusion with subdural hematoma, and
being older than 65 (Huang, Liao, Chen, & Ou, 2015; Najafi et al., 2015). The
risk of seizures varies for different groups, however. For example, seizure rates
of up to 53% have been identified after military injuries, while a 4% seizure
incidence may occur with a small intracranial injury (Frey, 2003). The overall
incidence of not-inflicted TBI seizures in children is 15% to 17%, while the
incidence for inflicted TBI seizures is 48% to 65% (Park & Chugani, 2015). The
risk of a first seizure for someone of any age continues to be elevated for more
than 10 years after brain injury (Annegers, Hauser, & Coan, 1998).
Posttraumatic hydrocephalus is the most common medical complication
following TBI, with rates of incidence varying from approximately 2% to 45%
(Gordon et al., 2006). Those with more severe injuries and those who have
undergone decompressive craniotomies are at more risk to develop posttraumatic
hydrocephalus (Fig. 20.1).
Figure 20.1 Hydrocephalus in a 58-year-old patient. (From Rowland, L. P.
(2005). Merritt’s neurology (11th ed.). Philadelphia, PA: Lippincott Williams &
Wilkins.)
Dysautonomia is also frequently seen after severe TBI and is characterized by

hypertension, tachycardia, increased body temperature and blood pressure,
profuse sweating, and decerebrate or decorticate posturing (Gordon et al., 2006;
Hendricks, Heeren, & Vos, 2010). People experiencing dysautonomia have been
shown to have longer rehabilitation lengths of stay, longer periods of
posttraumatic amnesia, and lower Glasgow Outcome Scale scores (Baguley,
Nicholls, & Felmingham, 1999; Gordon et al., 2006). (Table 20.1)
TABLE 20.1 Glasgow Coma Scale

Best possible total score = 15; worst possible total score = 3.
It has been estimated that up to 20% of people with TBI have deep vein
thrombosis on admission to the hospital (Carlile et al., 2010). Deep venous
thrombosis (DVT) results from prolonged immobilization. DVT can give rise to
pulmonary emboli, which is the most common preventable cause of hospital
death in TBI (Anderson, Wheeler, & Goldberg, 1991; Gordon et al., 2006). Brain
injury involves associated cerebral and brainstem depression or destruction that,
in turn, affects the person’s level of consciousness. Mild brain injury may result
in a relatively short loss of consciousness. Coma, defined as an alteration of
consciousness associated with decreased arousal and awareness of all stimuli
(Posner, Saper, Schiff, & Plum, 2007), is typically present following moderate to
severe TBI.
Either diffuse cerebral hypoxia or extensive cortical damage, with minimal
to no impairment of the brainstem, may result in a vegetative state. In this state,
the individual’s eyes may be open and follow a moving object, and the limbs
may move but without apparent purpose. The person will not respond to pain or
simple verbal requests, however, and there is no evidence of cortical function
related to voluntary movement (Bazarian, McClung, & Shah, 2005). Persons in
such a vegetative state may live briefly or for years.
Motor Deficits
Damage to the brainstem between the vestibular nuclei and the red nucleus
produces decerebrate rigidity, defined as an extensor posture of all extremities
and/or the trunk. When the brainstem is intact despite severe cortical damage,
decorticate rigidity is present, with flexion of the upper extremities and
extension of the lower extremities (Fig. 20.2). Abnormal reflexes complicate
movement patterns. During deep coma, brainstem reflexes may result in
grimacing to noxious stimuli, which may be accompanied by a change in
postural tone in the extremities. These deficits decline as coma lightens, and
motor disturbances reflecting neural damage become apparent. These deficits
may include quadriparesis, hemiplegia, or monoplegia, with or without
fluctuating muscle tone or spasticity, as well as disorders of coordination.
Decorticate rigidity (top) and decerebrate rigidity (bottom). (From Pellico, L. H.

(2012). Focus on adult health. Philadelphia, PA: Lippincott Williams &
Wilkins.)
Spasticity is characterized by velocity-dependent increase in muscle tone

resulting from hyperexcitability of the stretch reflex (Kandel, Schwartz, Jessell,
Siegelbaum, & Hudspeth, 2012). Spasticity is common in adults after moderate
and severe TBI, interfering with limb mobility and performance capabilities
(Burnett, Watanabe, & Greenwald, 2003; Gordon et al., 2006); 65% of children
also have spasticity following TBI (Dumas, Haley, Carey, Ludlow, & Rabin,
2003). With immobility, heterotopic ossification, or bone formation at an
abnormal soft tissue site, may form at synovial joints surrounded by spastic
musculature, particularly the hips, knees, and elbows (Gordon et al., 2006).
Coordination deficits include tremor and ataxia. Tremor types include
cerebellar, resting, essential, and physiologic. Cerebellar or intention tremors are
slow tremors that occur at the end of purposeful movement and are associated
with ataxia, hypotonia, and balance disorders. They tend to occur in trunk and
proximal muscles with intentional movement, at a frequency of approximately 4
to 6 per second. Resting tremors are correlated with striatal damage and involve
a pill-rolling movement at rest, occurring at a similar rate. Essential tremors are
slow constant tremors that typically affect more distal musculature, occur at a
frequency of 8 to 12 per second, and increase with anxiety and maintained
positions. Physiologic tremor is present in every person and occurs at the same
rate as essential tremor. It can be exacerbated by fatigue, stress, strong emotions,
caffeine, and fever. Post acquired ataxia is a result of damage to the sensory,
equilibrium, or cerebellar systems and is present in 20% to 30% of persons
sustaining DAI (NINDS, 2015).
Cognitive Deficits
Cognitive deficits are among the most common, difficult, and long-lasting
consequences of all levels of TBI in both adults and children. Limited memory,
especially, is typically present from coma through the person’s lifespan.
Retrograde and anterograde amnesia affect learning and cognitive
rehabilitation. Retrograde amnesia, or memory loss prior to the accident, may
gradually but incompletely improve. Anterograde amnesia, defined as the
inability to learn new long-term declarative information, is typically the last to
improve.
As coma subsides, cognitive deficits become apparent. These may include
difficulties with sustained attention, concentration, memory, comprehension,
reasoning, self-monitoring and impulse control, other-awareness, and executive
functions. Executive functions involve the ability to formulate context-
appropriate goals and to initiate, plan and organize, sequence, and adapt
behavior based on the anticipated or actual consequences of actions (Cicerone,
Dahlberg, & Kalmar, 2000; Hawley, 2004).
Other effects of cognitive dysfunction are apparent and include difficulty
performing routine activities of daily living (ADL), learning new motor routines,
and adapting to new or cognitively demanding situations (Cicerone et al., 2000).
Motor learning, or the ability to relearn previously well-known or learn new
adaptive motor skills, is often functional during the rehabilitation process despite
memory loss because it is located in the cerebellum.
Psychosocial Deficits
Neurobehavioral deficits occur as a result of cognitive deficits interacting with
brain dysfunction. These deficits are typically seen whether the TBI is mild,
moderate, or severe (Cicerone et al., 2000; Hawley, 2004) and include
impulsivity, perseveration, irritability, poor control of temper, aggression,
disinhibition, and apathy (Noggle & Pierson, 2010; Ylvisaker et al., 2007).
Limited self-awareness or a lack of insight may slow rehabilitation progress, as
well as the ability to participate successfully in academic, vocational, and/or
social roles. Depression and loss of self-esteem are a common problem and may
be particularly prevalent in children with TBI as they age. An increasing
awareness of their deficits coupled with decreased academic achievement (Fay et
al., 1994) may lead to depression in children. In one large sample of 722
outpatients with TBI, major depressive disorder was found in 42% of the sample
(Kreutzer, Seel, & Gourley, 2001); another large study of 666 people found the
incidence to be 27% (Seel, Kreutzer, & Rosenthal, 2003). Neither time since
injury nor severity of injury is correlated with depression (Gordon et al., 2006).
With depression comes the potential for suicide. Suicide rates for people with
TBI vary between 2.7 and 4.1 times that of the general population when matched
for age and gender (Engberg & Teasdale, 2004; Teasdale & Engberg, 2001). A
diagnosis of TBI and evidence of aggression and hostility are predictive of
suicide attempts (Gordon et al., 2006). Posttraumatic stress disorder is also seen
in people with TBI. Studies have found that symptoms of posttraumatic stress
disorder were related to the person’s level of self-awareness, but not to severity
of injury, years of education, intelligence, or memory impairment (Gordon et al.,
2006). Additional psychiatric conditions correlated with TBI include substance
abuse and aggressive behavior and agitation. Aggressive behavior has been
shown to be three times greater in people with TBI compared to people with
multiple trauma (Baguley et al., 1999). Other psychiatric diagnoses associated
with TBI include borderline, avoidant, paranoid, obsessive-compulsive, and
narcissistic personality. The onset of these disorders is independent of severity of
injury, age at injury, and time since injury (Gordon et al., 2006).
Visual rather than perceptual deficits are more common in the person with
TBI. Such problems may include diplopia, problems with accommodation and
convergence, visual field deficits, saccadic dysfunction, and strabismus
(Goodrich, Flyg, Kirby, Chang, & Martinsen, 2013).
Cranial Nerve Dysfunction

As the cranial nerves originate from the brainstem, TBI typically results in
damage to both the sensory and motor functions of these nerves. Lower levels of
coma may permit only assessment of cranial nerves III, VI, and VII (Keane &
Baloh, 1992). Pupillary reflexes are important early indicators of brain damage.
The absence of a pupillary reflex in response to light by an unconscious patient
is an indication of damage to the midbrain, from which the oculomotor nerve
(III) originates. A fixed dilated pupil, indicative of pressure on the oculomotor
nerve, is frequently seen following moderate to severe TBI (Kandel et al., 2012).
Cranial nerve II is the optic nerve and it transmits visual information from
the eye to the brain. The functions of cranial nerve II are acuity and processing
of the visual fields. Damage to this nerve may result in homonymous
hemianopsia, or difficulty seeing objects on the left or right hemispheres. If the
damage occurs at the level of the optic chiasm, bitemporal hemianopsia can
occur, which involves loss of the lateral part of vision in both eyes. As coma
subsides, significant visual deficits typically become apparent, because of
damage to the oculomotor (III), trochlear (IV), and abducens (VI) nerves. These
deficits include binocular, oculomotor, accommodative, refractive, and eyelid
movement dysfunction, as well as nystagmus, ptosis, and diplopia (Ciuffreda,
Ludlam, & Kapoor, 2009). Indeed, the composite signs of diplopia, blurred
vision, visual field loss, decreased oculomotor skills, and seeing movement in
the stable external environment has been termed posttrauma vision syndrome.
Double vision has been called the hallmark of visual deficits for persons with
TBI and often results in the individual closing one eye to eliminate double
vision.
Loss of the sense of smell (cranial nerve I) occurs in 7% of the people with
brain injuries, as a result of damage to the olfactory nerve (Tao &
Shenbagamurthi, 2012). Anosmia, or the absence of smell, is especially common
following frontal or occipital blows, as nerve endings cross through the thin and
easily fractured cribriform plate of the ethmoid bone in the nose (Tao &
Shenbagamurthi, 2012). Recovery of smell is not universal and is often
incomplete. A study of persons with TBI by Costanzo and Becker found that
only 33% of TBI victims improved in smell function. If recovery occurs, it
typically occurs between 6 and 12 months postinjury (Burnett et al., 2003;
Costanzo & Becker, 1990).
Damage to the ear is recognized as a common deficit associated with TBI;
high-frequency hearing loss is correlated with severity of cortical injury (Munjal,
Panda, & Pathak, 2010). Eighty to 90% of individuals with TBI who receive a
longitudinal fracture of the temporal bone will experience a conductive hearing
loss as a result of damage to the vestibulocochlear nerve (VIII). Positional
vertigo has been found in 15% to 90% of people with mild brain injury (Ahn et
al., 2011).
As oral feeding is attempted, damage to the glossopharyngeal (IX) and vagus
(X) nerves in the medulla may become apparent. Dysfunction results in an
absent or depressed gag reflex and decreased movement of the palate and uvula.
This decreased oral-motor movement makes swallowing hazardous and may
necessitate continued use of nasogastric or gastrostomy feeding tubes. In a
seminal study of swallowing disorders in brain-injured patients, Lazarus and
Logemann found that 81% of patients had a delayed or absent swallowing reflex,
50% demonstrated limited tongue control, and 33% had slowed peristalsis
(Lazarus & Logemann, 1987). Morgan, in a more recent study of dysphagia in
children with TBI, found that the incidence of dysphagia ranged from 1% in
those with mild TBI to 68% to 76% for those with severe TBI (Morgan, 2010).
Aspiration, or pathologic inhalation of food or mucus into the respiratory tract,
was found in one-third of all persons with TBI. The presence of aspiration is
highly correlated with the development of pneumonia, which may be life
threatening. In later stages of recovery, there may be hypotonia of the oral
musculature, resulting in drooling, limited lip closure and tongue control,
pocketing of food in the cheek, and a delayed swallow trigger (Morgan, 2010).
Response to and recovery from TBI tend to be highly individual, due to the
variety of neuropathological effects that may be present, as well as individual
factors of age, gender, and preinjury history. Persons with TBI, in general, may
expect a reduced life expectancy by 9 years (Harrison-Felix et al., 2015).
Furthermore, TBI “accounts for the greatest number of total years lived with a
disability resulting from trauma” (p. 728) (Maas et al., 2008).
Significant functional, emotional, behavioral, and social difficulties remain
for many years following injury. Useful factors in determining a person’s
prognosis are the trauma score, the GCS score, the presence of certain
biomarkers, and the presence or absence of hypoxia. In addition, consideration
of neuroimaging studies and electrodiagnostic findings, length of coma and
duration of posttraumatic amnesia help determine general psychosocial and
functional outcomes (Gordon et al., 2006).
The GCS was the first scale developed to predict both mortality and outcome
for the comatose patient and remains the best known and widely accepted scale
of coma (Kornbluth & Bhardwaj, 2011). The Disability Rating Scale (DRS) (Fig.
20.3) has expanded on this information to provide a quantitative assessment of
the disability of patients with severe brain injury. The DRS includes eight
categories, including assessments of the cognitive components of self-care
activities, the general level of functioning/dependence on others, and
psychosocial skill/employability (Rappaport, Hall, & Hopkins, 1982). The DRS
has demonstrated high interrater and test-retest reliability, as well as concurrent
and predictive validity (Pretz, Malec, & Hammond, 2013).
Figure 20.3 Disability rating scale.
The Levels of Cognitive Functioning Scale (LCFS) (Hagan, Malkmus, &

Durham, 1972) (Fig. 20.4) is used in many rehabilitation programs. This scale
classifies the admitted patient into one of eight levels of cognitive functioning
and has been shown to have good interrater and test-retest reliability (Gouvier,
Blanton, & LaPorte, 1987). Limitations for the scale are that it does not
adequately reflect small changes in recovery, may not accurately place a patient
with characteristics of two or more categories, and is less accurate at higher
levels (Wright, 2000).
Figure 20.4 Levels of cognitive functioning scale.
Level I of the LCFS is a period of dense unresponsiveness to all external stimuli.

In level II, an inconsistent, nonpurposeful, and often delayed response to
external stimuli is seen. Responses may be gross body movements,
vocalizations, or physiologic changes such as sweating. Visual tracking of large
objects is present, but the eyes appear unfocused. In level III, the level of
localized response, there is an inconsistent but specific response to a strong
stimulus such as pain or a bright object. An inconsistent response to simple
verbal commands may be present. The person may respond to discomfort by
pulling at nasogastric or catheter tubing. At level III, observational assessments
of deficits of vision and/or visual perception, somatosensation, and movement
may become feasible. Level IV is a highly variable stage, which may last for
shorter or longer periods of time for the individual person. In level IV, the
confused-agitated level, the person is confused and agitated, primarily
responsive to internal stimuli, and unable to cooperate with treatment. Behavior
may be aggressive, explosive, and nonpurposeful, with incoherent verbalization.
As this behavior may be out of character for the person, it can upset family and
friends and provide challenges for the treatment team. No short-term memory is
present. Attention is severely limited and is frequently driven by external
stimuli. In the absence of motor deficits, sitting, standing, reaching, and
ambulating are possible but do not occur purposefully or consistently on request
(Hagan et al., 1972).
At level V, the confused-inappropriate- nonagitated level, more consistent
motor response to requests becomes possible. Agitated and exaggerated behavior
may still occur, especially in response to external stimuli. An inability to
maintain selective attention is present, and frequent redirection is needed for any
task completion. Simple social and automatic communication is possible but
only for short periods of time. Memory is severely impaired and initiation is
often limited. While the person may be physically able to complete simple self-
care and feeding, verbal supervision is needed to accomplish tasks. The use of
some selected formal or standardized assessments may become possible at level
V.
In stages VI through VIII, the injured person becomes increasingly more
aware of his or her person, the external environment, and other persons and is
able to intentionally plan movement sequences. Responses to requests become
consistently more appropriate, and the supervision level decreases for previously
learned tasks. New academic learning is generally impaired until level VIII
(Hagan et al., 1972).
Other factors, such as memory loss, age, and intracranial pressure, are also
associated with outcome. Postinjury amnesia of <1 day suggests a mild injury,
whereas amnesia lasting more than 1 day is indicative of a more severe injury. A
younger age at injury improves both the chance of survival and overall outcome
in adults. In children, higher death rates are associated with younger ages, and
mortality below the age of 1 year is great, with abuse common as the primary
cause (Craig, Campbell, Richards, Ventureya, & Hutchison, 2004).
Acute Phase
Physicians from neurology, neurosurgery, internal medicine, or orthopedics may
direct overall medical management in the acute phase. The focus of acute
medical management is preservation of life, management of secondary
complications and the prevention of secondary damage. Maintaining an effective
airway and maintaining circulatory function are critical life-preserving steps
immediately after injury. An endotracheal tube is typically placed to support
breathing. After arrival at the hospital, diagnostic tests are begun to identify the
location and severity of all injuries. The patient typically receives a
computerized axial tomography scan (CAT). If this reveals an intracranial
hematoma, immediate surgical decompression is performed. Constant
monitoring of consciousness occurs, as the duration and depth of coma are
significant indicators of both mortality and morbidity (Carlile et al., 2010).
Diagnosis and management of secondary diagnoses also occur upon arrival at
the hospital. Most persons with severe head injury have additional injuries
(Eapen, Allred, O'Rourke, & Cifu, 2015). A common secondary complication
from the brain injury is hydrocephalus, which is a serious complication for up to
4five percent of individuals with TBI (Eapen et al., 2015). Fractures are common
as well, and 16% to 59% of persons with TBI have a concomitant spinal cord
injury (Macciocchi, Seel, Thompson, Byams, & Bowman, 2008). In this case,
immediate medical management is needed for both a brain injury and a high-
level spinal cord injury .
Intensive care medical management is constant. An indwelling urinary
catheter is placed and closely monitored. About one-third of those hospitalized
with TBI aspirate food into their lungs, resulting in aspiration pneumonia. These
persons usually have a delayed or absent swallowing reflex (Lazarus &
Logemann, 1987; Logemann et al., 1994; Mackay et al., 1999). A nasogastric
tube is positioned and used for high-caloric feeding for people with swallowing
dysfunction. Close attention to skin integrity is essential, and the person’s total
body position is changed frequently. Vigorous respiratory therapy is typically
implemented to prevent additional pulmonary problems (Wijayatilake et al.,
2015).
Ongoing management of common medical complications occurs in the acute
phase. Prophylactic medication for seizures is provided typically for only the
first 7 days following injury and then discontinued unless the person has
recurring seizures. As a result of rigid abnormal posturing and other motor
disturbances, many persons with TBI develop contractures of the neck, trunk,
and/or extremities. The longer the duration of coma, the greater is the potential
for the development of contractures, heterotopic ossification, and DVTs.
Treatment to prevent these complications commonly includes range of motion
and splinting in the acute phase. Medical intervention for spasticity in the acute
phase includes physical and pharmacological interventions. Commonly
prescribed medications include baclofen, tizanidine, dantrolene, and botulinum
toxin (Greenwald & Rigg, 2009).
Rehabilitation interventions in the acute phase may begin as soon as
neurological stability is achieved. The focus of acute rehabilitation is to prevent
joint deformity and to provide graded and specific sensory stimulation, with the
assumption that selective sensory input may speed or improve neurological
recovery.
Rehabilitation
Admission to an inpatient rehabilitation unit is needed for people with moderate
to severe TBI. Reasonable and necessary requirements for admission to an
inpatient rehabilitation facility are (1) medical stability, (2) the need for close
medical supervision, and (3) the need for active and ongoing intensive therapy
by multiple therapy disciplines (Department of Health and Human Services,
2015). Inpatient rehabilitation requires tolerance for at least 3 hours of two or
more therapies 5 to 7 days per week; subacute rehabilitation requires the ability
to participate for 0.5 to 2 hours each day. Intensive rehabilitation is usually
directed by a physiatrist, a physician specializing in rehabilitation medicine.
Goals of the rehabilitation program are to maximize the person’s function,
minimize additional physical or psychosocial impairments, and prevent
complications (Greenwald & Rigg, 2009). Along with the primary physician,
core rehabilitation members include specialists in occupational therapy, physical
therapy, speech/language pathology, nursing, neuropsychology, and social work.
In 2005, a major review of multidisciplinary rehabilitation of adults with TBI
found “strong evidence of benefit from formal intervention,” with earlier
functional gains from more intensive programs (Turner-Stokes, Pick, Disler &
Wade, 2015).
The long-term rehabilitation goals in occupational therapy are to re-establish
occupational performance skill, sensorimotor integration and control, and the
improvement of perceptual, cognitive, and communication skills for daily
function. Where remediation is not possible or when maximal neurological
recovery is assumed to be complete, the use of compensation strategies may be
appropriate. As basic goals are accomplished, discharge from the hospital may
occur, with more advanced skills learned on an outpatient basis. Outpatient
occupational therapy goals include instrumental ADL, further community
reintegration, and work reentry.
Impact of TBI on Occupational Performance
All areas identified in the Occupational Therapy Practice Framework are
affected with TBI. The deeply comatose person, with cognitive levels I through
III, shows depressed function in all areas of meaningful function. With further
recovery, improvement in performance skills and patterns, as well as in client
factors, may occur and enable the performance of preferred or required
occupations. In a study of 1,170 records from the TBI Model Systems database,
Bushnik et al. (2003) found that individuals with TBI as a result of a vehicular
accident were initially admitted with significantly lower Functional
Independence Measure motor scores than those who were admitted because of
violence, falls, or other causes. However, at discharge from rehabilitation, no
significant differences were found among patients in the four etiology groups for
most psychosocial and functional outcome measures. In fact, 85% to 96% of all
patients demonstrated sufficient basic ADL skills to live in a supervised private
residence upon discharge.
Despite the acquisition of basic ADL skills, independence in community
living is difficult for many people after TBI. A study of 147 survivors of severe
TBI 4 years after injury found that 12% of participants were still impaired in
common ADL after 4 years. These activities included the items of incontinence,
grooming, toileting, bathing, dressing, and feeding as well as transfers, walking,
and climbing. More than 50% of survivors needed help for activities such as
using a bank account, paying household bills, managing appointments, and
keeping track of money. Participants reported regular contacts with siblings,
other relatives, and closest friends, but less often with other acquaintances. Few
people participated in a professional activity (Jourdan et al., 2015).
Work
Many people have major difficulties returning to a productive life after TBI
whether the injury is mild or severe. Roffolo, Friedland, and Dawson (1999)
reported that while 42% of people with mild brain injury returned to work, only
12% returned to their premorbid level of employment. Cognitive and behavioral
issues were cited for this decreased function. Bushnik et al. found the
unemployment rate for persons with TBI because of violence to be 70%,
significantly greater than the rate of approximately 50% for those with TBI from
all other causes (Bushnik et al., 2003). An additional factor independently
predicting poorer productivity outcomes was preinjury substance abuse (Sherer,
Bergloff, & High, 1999).
For those with moderate to severe injuries, the ability to return to work has
been inconsistently correlated with self-awareness. Sherer et al. (1998), in a
multicenter TBI Model System study, found that limited self-awareness
accounted for a substantial proportion (0.31) of the variance in positive
vocational outcomes, while Coetzer and du Toit (2002) found no comparable
correlation in their study of 40 people with TBI of varying levels of severity. In
the absence of definitive findings to date, the difficulty of people with TBI to be
aware of their deficits and the effect of their actions on others should be included
in planning for return to productivity. Indeed, psychosocial skills that affect
social integration show a stronger correlation with successful return to work than
do either cognitive or sensorimotor skills, or any combination of the three factors
(Conti, 1992).
Neither gender nor race has been consistently linked to productivity
outcomes. A review by van Reekum found conflicting evidence that females had
poorer outcomes, partly based on varying definitions of productivity (van
Reekum, 2001). A major study of race and productivity (Sherer, Nick & Sander,
2003) found that African Americans and other people from minority
backgrounds had less productive outcomes than did Whites, but race alone
accounted for little of the variance in productivity. Race and productivity had
confounding associations with preinjury productivity, educational level, and
cause of injury. The DRS (Rappaport et al., 1982) has been found to predict
employment. In a study of 145 persons with TBI, Cope, Cole, and Hall (1999)
found that 62% of those with scores of one to three on rehabilitation admission
were employed or in school at 1 year after discharge, while 39% of those with
scores of 4 to 6 and only 11% of those with a DRS score of 7 to 20 were
similarly employed. For those returning to work, supported part-time
employment has been shown to be a viable and cost-effective option (Cope et al.,
1999). However, limited employment opportunities with typically lower wages
and decreased work hours often result in the need for public assistance
(Johnstone et al., 2003; Wehman et al., 2003b).
Driving
Physical disabilities; cognitive, visual, or perceptual dysfunction; and self-
awareness deficits can significantly impair driving function. A number of studies
have attempted to identify factors that predict those persons with TBI who may
successfully return to driving, with inconclusive results (Coleman, Rapport, &
Ergh, 2002; Pietrapiana et al., 2005; Rapport, Coleman, & Hanks, 2008).
Severity of injury and duration of coma have not shown to impact driving
consistently. One study found that persons more likely to return to driving
included those discharged from rehabilitation with independence or modified
independence in scores on the Functional Independence Measure (Fisk,
Schneider, & Novak, 1998). Neuropsychological testing may provide insight into
driving potential, but these findings may be limited by the person’s level of self-
awareness (Novack et al., 2010). Nevertheless, about 50% of people with
moderate to severe TBI have resumed driving within 5 years, with most driving
within the first year of injury (Novack et al., 2010; Rapport, Hanks, & Bryer,
2006). Tamietto and colleagues (2006) note, however, that as many as two-thirds
of these may resume driving without any formal examination. In a study
comparing nondrivers to drivers with TBI, Rapport et al. (2006) found that
nondrivers who wanted to drive rated themselves as physically and cognitively
fit to drive, despite cognitive skills that were significantly worse than drivers
with TBI. In discussing this finding, Rapport et al. (2006) stated that “even
nondrivers rate their current driving abilities as better than average (that) may
reflect unawareness of deficit, denial, resistance to role change or accurate self-
perception” (p. 42).
Tumors of the Central Nervous System
Descriptions and Definitions
Brain tumors, another category of ABI, have increased in the past few decades.
Fortunately, this appears to be due to enhanced neuroimaging techniques and
improved medical treatment options (Fisher, Schwartzbaum, Wrensch, &
Wiemels, 2007) rather than a rise in natural incidence. Tumors are classified as
primary or secondary and malignant or benign. The site of origin for the tumor is
considered its primary site, even when the tumor has spread to other parts of the
body or brain (secondary site). Malignant tumors are composed of abnormal
cells that multiply rapidly, with the ability to invade, or metastasize, into other
tissues. Brain tumors, conversely, rarely metastasize beyond the brain. There are
four general categories of malignant brain tumors: gliomas, meningiomas, germ
cell tumors, and sellar region tumors (Bondy et al., 2008; Fisher, Schwartzbaum,
Wrensch, & Berger, 2006). Benign tumors, on the other hand, are not cancerous
and they do not invade other body tissues or spread to other body parts. Benign
tumors may become life threatening as they cause increasing deficits with cell
growth, because they press upon nearby structures and tissues (Fisher et al.,
2007). This section will discuss malignant primary brain tumors. However, the
signs and symptoms of, and diagnosis, and treatment for, a secondary brain
tumor are similar.
Etiology
Chemical changes in brain cells lead to the formation of brain tumors. However,
why these changes occur is not fully understood. Most brain tumors develop for
no apparent reason and are not associated with anything the person did or did not
do.
The incidence of brain tumors in the United States has increased over time
and differs according to gender, age, race and ethnicity, and geography. In the
United States, the median age at diagnosis among all patients diagnosed with a
primary brain tumor between 1998 and 2002 was 57 years. Gliomas are
approximately twice as common among Caucasians as compared with African-
Americans, as are germ cell tumors, while the incidence of glioma among non-
Hispanics was greater than that of Hispanics (Fisher et al., 2007). The lowest
age-adjusted average annual incidence of all CNS tumors is found in Virginia,
while the highest incidence is present in Colorado (Fisher et al., 2007). Gender
appears to be associated with risk for varying types of tumors. Men are more
susceptible to glioma and germ cell tumors, while women are twice as likely as
men to have meningiomas. In addition, relatively consistent research results
show that being premenopausal confers a greater meningioma risk than being
postmenopausal (Fisher et al., 2007).
High levels of ionizing radiation are strongly associated with tumor
development, as was found following the atomic bombing of Hiroshima in
World War II (Fisher et al., 2007). Types of tumors associated with radiation
include glioma, schwannoma, and pituitary tumors. In the past, infants and
children have been treated with radiation in the treatment of tinea capitis and
skin hemangioma and have shown increased risk for nerve sheath tumors,
meningioma, and pituitary adenoma. Taken as a whole, however, exposure to
high levels of radiation is rare.
Many studies suggest that the risk for glioma is reduced as a result of
allergies and immune-related conditions. This may arise from the anti-
inflammatory effects of cytokines present in allergic and autoimmune diseases
(Fisher et al., 2007). Inconsistent results have been found for exposure to other
environmental factors. Studies of cell phone use and glioma risk do not generally
support the conclusion that cell phone use causes brain tumors (Inskip et al.,
2001; Kan, Simonsen, Lyon, & Kestle, 2007). However, long-term studies are
needed to determine definitively any risk factor between cell phone use and
glioma. Inconsistent, minimal, or no evidence is present to suggest any
relationship with increased brain tumor risk and head trauma, certain dietary
supplements, alcohol consumption, tobacco smoking, cellular phone use, and
exposure to electromagnetic fields (Fisher et al., 2006, 2007). Inconclusive
studies suggest that exposure to neurogenic or carcinogenic substances may be a
risk factor. Therefore, occupations involving these substances may have a higher
risk for brain tumors. Such occupations may include agricultural works,
firefighters, petroleum and gas workers, construction workers, and janitors
(Wrensch, Minn, Chew, Bondy, & Berger, 2002).
About 22,070 persons were diagnosed with primary malignant brain tumors in
2009, including 12,060 men and 10,060 women (Central Brain Tumor Registry
of the United States, 2014). An estimated 3,420 new cases of primary brain
tumors were expected to occur in children in 2015, with the rate higher in boys
than girls (Central Brain Tumor Registry of the United States, 2014). Incidence
differs according to gender, age, race and ethnicity, and geography. Adult men
tend to have higher rates than do women of primary malignant brain tumors,
while nonmalignant meningioma is more common in women (Bondy et al.,
2008).
Age affects the survival rate for both the youngest and oldest people.
Childhood cancer is the second leading cause of death (after accidents) in
children between 1 and 14 years (American Cancer Society, 2014). They are also
the third leading cause of death in young adults ages 20 to 39 (Jemal, Siegel, Xu,
& Ward, 2010). In general, and for most tumors, the 5-year survival rate
increases with age (Bondy et al., 2008) through the fifties and early sixties.
Studies of race and ethnicity are confounded by issues related to low
socioeconomic status. African Americans are more likely to develop tumors than
any other racial or ethnic group, and death rates from cancer are 34% higher for
African American men and 17% higher for African American women than their
Caucasian counterparts. Hispanic people tend to have lower incidence rates for
cancer. These figures must be placed in context with concerns of poverty. About
25% of African Americans and Hispanic Americans lived below the poverty line
during the period from 2007 to 2011. By comparison, only 11% of non-Hispanic
White people lived below the poverty line (Macartney, Bishaw, & Fontenot,
2013). Furthermore, one in five African Americans and one in three Hispanic
persons were uninsured and therefore less able to receive needed treatment.
Finally, issues of health disparity may play a role in the provision of health care.
Racial and ethnic minorities tend to receive a lower quality of care than do
Whites, even when insurance status, income, age, and severity of conditions are
comparable (Institute of Medicine, 2002).
Cancer rates tend to be higher in more developed countries, partly due to
greater technology resources for evaluation and treatment.
Signs and Symptoms
Signs and symptoms of brain tumors include fatigue, sleep disturbance, pain,
mood disorders, and cognitive dysfunction. Fatigue may be the single most
significant problem with brain tumors, resulting in increased daytime sleep and
decreased or interrupted nighttime sleep. Nightly sleep disturbance is reported
by up to 50% of people with brain tumors (Liu, Page, Solheim, Fox, & Chang,
2009). One study of people with recurrent malignant gliomas found that as many
as 94% reported severe fatigue (Osoba, Brada, Prados & Yung, 2000).
Headache is the most common type of pain experienced, with up to 50% of
people with gliomas reporting severe pain. Mood disorders accompanying brain
tumors include anxiety and depression. Depression particularly has been linked
to survival, and yet, one study found that only 60% of people who reported this
to their physician received antidepressants (Litofsky et al., 2004). Problems with
memory and executive functions are found in almost half of all people with
glioma tumors (Liu et al., 2009).
In general, survival from any tumor is lowest in the oldest age groups. When
types of tumors are examined, the relative survival probability of those with
glioblastomas is the lowest, with a 37.7% 2-year survival rate and a 30.2% 5-
year survival rate. The survival rates for benign meningioma are much less well
known but appear to be significantly better. One estimate is that the 5-year
survival rate for meningioma may be 81% for people aged 21 to 64 years and
56% for those 65 or older at diagnosis (McCarthy, Davis, & Freels, 1998).
Diagnosis
Diagnosis begins with the onset of unexplained symptoms. As tumor cells
multiply in the brain, they create pressure on and irritate normal brain tissue. As
a result, two-thirds of those with primary brain tumors report symptoms as just
described. Amazingly, about one-third of all people with brain tumors have no
symptoms initially.
The presence of symptoms is typically assessed by a neurologist, with the
use of such diagnostic procedures as magnetic resonance imaging (MRI),
computerized tomography (CT), positron emission tomography (PET), or
biopsy. The MRI uses an extremely strong magnet and radio waves to produce
brain images. It may or may not be used with angiography, in which a dye is
inserted into the bloodstream to differentiate between healthy and tumor tissue.
People receiving an MRI may not have pacemakers or metal implants or be
allergic to the dye used in angiography. CT scans involve multiple x-rays of the
brain from different angles. From this, a computerized three-dimensional model
of the brain can be displayed. Iodine is commonly used as a contrast agent to
enhance the image. As a result, this may not be the best diagnostic procedure for
people with allergies, diabetes, or a heart, kidney, or thyroid condition. PET
scans are typically an ancillary diagnostic tool to either the MRI or PET scan.
Finally, a biopsy of the suspected brain tissue may be made. Following the
biopsy surgery, histologic analysis of brain cell tissue occurs (American Cancer
Society, 2015d).
In most cancer, an important next step is to determine the stage of the tumor.
However, brain tumors rarely spread to other organs, although they may spread
within the brain. Therefore, there is no formal staging system for the prognosis
of brain tumor. Instead, the oncologist will use the following information to
determine the outlook: the type of tumor, the grade of the tumor (how quickly it
can be expected to spread), the person’s age, the person’s functional status
(whether symptoms are present or not), the size and location of the tumor, the
feasibility of surgical removal of the tumor, and whether there is evidence of
spread to other parts of the brain and/or spinal cord (American Cancer Society,
2015d).
Management of brain tumors may involve surgery, radiation therapy, and/or
chemotherapy. Surgery is now more sophisticated than in the past. It can now be
guided at least partially by the use of MRI, to determine locations of important
brain areas and their distance from a tumor. Surgery also may be directly aided
by image-guidance, which provides better visualization of the brain area
(American Cancer Society, 2015d).
Radiation therapy uses x-rays, gamma rays, electron beams, or protons to
destroy cancer cells. Because radiation can be localized, it affects only the body
part being treated. It may be used alone or in combination with surgery or
chemotherapy, where some drugs may actually make cancer cells more sensitive
to radiation. Radiation may be given externally, using a linear accelerator, or
internally, where a radioactive source in an implant is surgically placed near the
tumor (American Cancer Society, 2015a, 2015d).
Significant improvements have occurred to radiation therapy recently,
allowing smaller doses of radiation and more precise placement of the radiation.
Stereotactic radiosurgery is not really surgery but the delivery of precise
radiation to a brain site guided by MRI or CT scans. Nearby tissue is affected as
little as possible. Types of stereotactic radiosurgery include using a moving
linear accelerator, the Gamma Knife, or the use of accelerator-delivered proton
and helium ions. Stereotactic radiosurgery typically uses just one treatment
session to deliver the full radiation dose (American Cancer Society, 2015a).
Common side effects of radiation therapy, requiring medical management,
include fatigue, fever/chills, and a sore or dry mouth. Because radiation therapy
to the brain may increase the chance of tooth cavities, ongoing dental
consultations are advised. Hair loss also is a common side effect (American
Cancer Society, 2015b). While this is not a dangerous side effect, it is often
distressing to the person.
Newer approaches to chemotherapy are also available. Traditionally,
chemotherapy drugs have had difficulty crossing the blood-brain barrier.
Research is currently underway to modify these drugs to successfully cross the
barrier, so that they can reach more easily the bloodstream in the brain. Some
targeted drug treatments have been developed that focus on specific
abnormalities within cancer cells. These treatments are very new and are still
undergoing rigorous study in clinical trials (American Cancer Society, 2015c).
The schedule for chemotherapy is dependent on a number of factors and may
be provided daily, weekly, or even monthly. After each treatment cycle, a break
is provided to allow the body time to rebuild healthy new cells and recuperate
from the strong chemicals used. Common side effects of chemotherapy include
fatigue, nausea, vomiting, sore mouth, diarrhea or constipation, loss of appetite,
pain or difficulty swallowing, swelling in hands or feet, itching or rash, shortness
of breath, cough, muscle or joint pain, or numbness in hands or feet (American
Cancer Society, 2015b).
In addition to chemotherapy, the medications most commonly prescribed for
brain tumors are steroids and anti-epileptic drugs. Steroids are used to reduce
brain edema and ameliorate the person’s symptoms. They may be prescribed at
diagnosis or before or after surgery. Common steroids include dexamethasone,
Decadron, prednisone, or methylprednisone. Short-term side effects include
insomnia, weight gain and increased appetite, mood swings, and irritability. Side
effects from long-term use include cataracts, osteoporosis, muscle weakness, and
diabetes (University of California San Francisco, 2012).
Antiepileptic medications may be used as a precautionary measure or in
response to seizure activity. Common anticonvulsant drugs include Dilantin,
Tegretol, Depakote, Keppra, Neurontin, Topamax, phenobarbital and Lamictal.
Side effects from these medications include fatigue, weakness, nausea and
vomiting, and incoordination.
Complementary and alternative therapies may also be used, not to replace
medical treatment but to lessen symptoms. Complementary therapies may
include stress management, relaxation and imagery training, meditation,
acupuncture, herbal medicine, and massage.
Education and support are critical throughout the course of treatment.
Support groups may be helpful to both the person with cancer and his or her
caregivers. Additionally, individual or family counseling may provide additional
support and individual-specific information.
While rates of survival have improved for some types of cancer, most people
consider a tumor to be deadly. Values and beliefs may be in conflict. For
example, a highly valued commitment to honesty may conflict with the desire
not to cause others distress. A basic belief in fairness may be shaken by the
timing of the diagnosis. Spiritual beliefs may be overturned, or spirituality may
grow.
Mental functions are likely to decline, as well as movement-related skills,
vision, and/or communication, depending on the site of the tumor. Pain is likely
to increase. Fatigue and side effect from medical treatment may make
participation in all daily activities and preferred occupations difficult. While
ADL may or may not remain intact for a considerable amount of time,
instrumental activities such as work and leisure pursuits are likely to suffer fairly
quickly from both physical dysfunction and cognitive deficits. Rest may be
disrupted and daytime sleep ineffective in decreasing perceptions of fatigue. In a
study comparing people with malignant glioma to age- and gender-matched
people with lung cancer, greater problems with vision, motor function,
communication, headaches and seizures were identified by the people with
glioma (Klein, Taphoorn, & Heimans, 2001).
Both TBI and brain tumors result in physical, cognitive, and social-
emotional deficits affecting all areas of occupation. For the occupational
therapist, assessment and intervention are challenging, at times frustrating, and
ultimately rewarding.
CASE-STUDY 1
John, age 20, survived an automobile accident, with a moderate TBI. After
2 weeks each in intensive care and an acute medical unit, he is to begin
intensive rehabilitation. His occupational therapist cannot get reliable
information from him, so she relies on a medical record review for his
medical history and a discussion with his mother to identify his previous
occupations.
His mother does not seem to be very aware of his activities. She states
that he has had three garage mechanic jobs in the last 2 years. She says he
seemed to get tired of a routine and didn’t get along well with his bosses.
He moved into her two-bedroom apartment about 6 months ago so he could
start saving money for a new car. He does not participate in any home care
tasks but does help with the rent. Leisure activities included fixing up his
old car and “hot-rodding” around. John’s mother does not care for many of
his friends, but becomes tearful when asked about his relationship with
Kathy. She says they were planning to be married in the fall.
The medical record review reveals that John sustained a right tibia-
fibula fracture with the TBI. After 3 days of general unresponsiveness, he
began to obey simple commands (LCFS III). In a few days, he became
agitated and confused. A few words could be understood, including swear
words. He persistently pulled out both his urinary catheter and his
nasogastric tube.
The agitation has lessened somewhat, but he continues to be
intermittently disoriented, as when he calls his occupational therapist
“Kathy.” There is a 1- to 2-second delay before any requested movement.
His gaze appears divergent. When fatigued or perhaps frustrated, he is
irritable and the therapist has used protective measures she was taught to
protect both herself and John from injury as he jabs his fist out toward her.
Motor deficits include poor sitting balance and right hemiplegia, with
moderate to severe spasticity. The left upper extremity is within functional
limits in range of motion and strength. Transfers require moderate
assistance.
John is independent in eating but requires assistance for all other tasks.
He requires moderate assistance and verbal cuing for showering using a
shower chair, donning and doffing a T-shirt, and transferring to and from
the bed and wheelchair. Wheelchair mobility is slow, but he can wheel
himself from one area to another with verbal cuing for the route. John is
unable to read, and perseveration is apparent when writing his name. John
has come a long way, but there is still a longer way to go to achieve
maximal possible independence.
CASE-STUDY 2
As a part of routine fourth-grade vision screening, the school nurse
noticed that Jesus appeared to have some vision problems that weren’t
present last year. Jesus said things seemed fuzzy. She sent a note home with
Jesus to his mother but never received a response. Six weeks later, his
teacher sent Jesus to see the school nurse again, this time because of
ongoing complaints of a headache. While with the nurse, Jesus vomited and
said that he had been routinely vomiting for the last few weeks. He had no
fever. Alarmed, the school nurse drove Jesus home to the trailer in which he
and his mother, brother and two sisters lived.
She spoke to the mother and stressed the need for immediate evaluation
by a physician. Maria, his mother, stated that she had no physician and no
insurance, and that the only medical care she and her family received was at
the free clinic, open only on Tuesday afternoons and evenings. The school
nurse wrote out information for the doctor there, and Maria promised to
take Jesus to the next clinic.
The physician at the clinic examined Jesus and immediately set Jesus
up for an evaluation by a pediatric oncologist. Social workers at the clinic
assisted in finding transportation, and assured Maria that medical care
would be provided despite her inability to pay. The visit to the pediatric
oncologist provided a diagnosis of glioma. This message was sent to the
social worker at the clinic, where there was a telephone. The social worker
drove to Maria’s to give Maria this information and to plan how to achieve
the medical care Jesus needed. School was put on the back burner. Jesus
entered a regional children’s cancer center. Maria arranged for the other
children to stay with an aunt and stays near the center.
It was a confusing and fun and sad time for Jesus. He missed his friends
and his family, but he enjoyed the activities of the center. He did not like
how he felt after radiation therapy, and it was hard to understand that
something that made him feel so bad could make him better. He was glad
his mother was around and, for the first time in years, spent time sitting on
her lap.
Jesus came home, to a great family celebration. Maria ended the
celebration after an hour, seeing that Jesus was tired. But Jesus was happy
to be home. He didn’t care that he had no hair, because he was beginning to
feel better, even though he tired easily. He missed his friends at school and
hoped to go back soon, but he knew he still couldn’t see well. Maria feared
for the future of Jesus and her family and worried about bills that would
certainly come.
RESOURCES
Brain Injury Association of America
1608 Spring Hill Road
Suite 110
Vienna, VA 22182
Phone: 703-761-0750
[email protected]
http://www.biausa.org
National Institute of Neurologic Disorders and Stroke (NINDS).
Traumatic Brain Injury Information Page
http://www.ninds.nih.gov/disorders/tbi/tbi.htm
Family Caregiver Alliance/National Center on Caregiving 785 Market St.
Suite 750
San Francisco, CA 94103
Tel: 415-434-3388, 800-445-8106
[email protected]
http://www.caregiver.org
American Brain Tumor Association
8550 W. Bryn Mawr Ave.
Suite 550
Chicago, IL 60631
773-577-8750, CareLine: 800-866-2282
[email protected]
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C H A P T E R
21 Burns
Rebecca Ozelie
KEY TERMS
Allograft Autograft Burn shock Debridement Dermis Epidermis Fluid creep
Fluid resuscitation Full-thickness burn Hypermetabolism Hypertrophic scar
Keloids Partial-thickness burn Subcutaneous tissue Superficial burn Total body
surface area (TBSA) Zone of coagulation Zone of hyperemia Zone of stasis
Sam, age 15, is scheduled for a burn dressing change at the outpatient burn
clinic. Sam suffered 46% mixed partial- and full-thickness burns to his face,
neck, chest, and bilateral arms 4 months ago when he added gasoline to a brush
fire. The resulting explosion set Sam’s clothes on fire. Sam panicked and started
running. A neighbor who heard the explosion from inside his house tackled Sam
and smothered the flames with a blanket. Sam spent 2 months as an inpatient on
the burn unit where he received extensive grafting to his face, neck, arms, and
chest. He was discharged 2 months ago but continues to have scattered small
open areas. Sam arrives at the clinic, accompanied by his mother, and
immediately requests assistance to remove his coat. When he is asked to try to
remove his coat himself, he is able to complete the task but is noted to have
extremely limited range of motion (ROM) and severe bilateral axillary
contractures. It is noted that both his clothing and temporary compression
garment have been cut apart at the arms and shoulder and duct-taped together.
When asked why this was done, he replies that it is easier to remove his clothes
this way. His mother states, “I told him he shouldn’t do that.”
Anatomy and Physiology of the Skin
An understanding of burn injury must begin with a review of the anatomy and
physiology of the skin. The skin is the largest organ of the body (Latenser &
Kowal-Vern, 2002). The skin has three anatomical layers: epidermis, dermis,
and the subcutaneous tissue. The thin nonvascular outer layer, called the
epidermis, consists of layers of epithelial cells. This layer serves as a barrier to
bacteria and moisture loss. Beneath the epidermis is the thicker dermis, which
makes up the bulk of the skin. Housed within the dermis are hair follicles, blood
vessels, sweat glands, nerve endings, and sebaceous glands, which play an
integral part in the functions of the skin (Fig. 21.1). The functions of the skin
include (McGraft & Uitto, 2010)
Figure 21.1 Anatomy of the skin. (From Archer, P., & Nelson, L. A. (2012).
Applied anatomy & physiology for manual therapists. Philadelphia, PA:
Protection against infection

Prevention loss of body fluid
Control of body temperature
Functioning as an excretory organ
Production of vitamin D
Helping to determine personal identity
Pathophysiology of Burns
In addition to understanding the anatomy and physiology of the skin, it is
important to understand the pathophysiology of burns. An understanding of what
kind of physiological response a burn will induce is critical to the management
of burns. The two primary factors that influence the amount of tissue destruction
that occurs following a burn injury are temperature and duration of exposure
(McGraft & Uitto, 2010). The tissue damage that occurs following a burn injury
can be divided into three zones (Kowalske, 2011). The zone of coagulation is
the area exposed to the most amount of heat and ensues the most damage. This is
the area of irreversible tissue destruction. Surrounding this is the zone of stasis,
where damage results in decreased tissue perfusion. The tissue in this zone may
be salvageable, and the main goal of burn resuscitation is to increase tissue
perfusion here and prevent any irreversible damage. The outer zone is referred to
as the zone of hyperemia. The tissues in these outer zones are damaged but with
proper care should recover and heal. Without proper care of the tissue in the
zone of hyperemia, further damage may result and increased tissue loss can
occur (Kowalske, 2011; Rowan et al., 2015). The aim of care after a burn injury
is to reduce or prevent dermal ischemia, thereby avoiding further tissue death.
The residual necrotic layers of skin destroyed by direct heat damage or the injury
occurring secondary to heat damage is referred to as eschar.
Etiology
The most common cause of the burns is fire/flame (43%), scald (34%), contact
(9%), electrical (4%), chemical (3%), and other (7%). The majority of burns
occur in the home (73%). The other places burns occur include occupational
(5%), street/highway (5%), recreational/sport (5%), and other (9%) (American
Burn Association, 2015).
Incident and Prevalence
The American Burn Association (2015) reports that 486,000 people seek
medical treatment for burns each year. This estimate is derived from hospital
admission and emergency department data. It is acknowledged that this number
is an estimate range because some burns may have been treated at home, in the
community, and at clinics. Sixty percent of the estimated acute hospitalizations
for burns were admitted to 128 burn centers and the other 40% to the nation’s
4,500 acute care hospitals. Those hospitals average fewer than three burn
admissions per year. Of the 60% admitted to the nation’s burn centers, there is a
96.7% survival rate with 69% male and 31% female. The mean age is 30 years
(Veeravagu et al., 2015). Caucasians represented the largest ethnic group
affected (59%) followed by African American (20%), Hispanic (14%), and
others (7%).
Signs and Symptoms
Depth of Burn
The depth of a burn injury reflects how deep into the skin layers a burn extends
and the duration of hot contact (Fig. 21.2). Understanding the depth of burn
injury will influence projected survival rates, healing time, treatment, and scar
formation.
Figure 21.2 Depth of burn injury. (From Pellico, L. H. (2012). Focus on adult
health. Philadelphia, PA: Lippincott Williams & Wilkins.)
Superficial Burn Injury

A superficial (first-degree) burn injury involves only the epidermal layers of the
skin (Kowalske, 2011). This burn is characterized by redness and pain. The
wound is dry and does not form blisters. The wound blanches readily and is
exquisitely sensitive to air and/or light touch. A superficial burn injury can result
from a variety of causes such as a sunburn or flash from an explosion.
Partial-Thickness Burn Injury (Superficial vs. Deep)

A partial-thickness (second-degree) injury destroys the epidermal layer and
extends down into the dermal layer of the skin. The differentiation between
superficial partial thickness and deep partial thickness is dependent on how deep
the burn extends into the dermal layer. A superficial partial-thickness burn has
damage in the upper layers of the papillary dermis (Lloyd, Rodgers, Michener, &
Williams, 2012). A sunburn that peels is the depth of a superficial partial-
thickness burn. These burns are characterized by clear blisters and weeping, wet
skin. The burn will blanch and is painful when touched.
A burn to the deeper layers of the dermis is classified as a deep partial-
thickness burn. Deep partial-thickness burns affect the entire epidermis and
dermis but spare the base of the hair follicle (Kowalske, 2011). This type of burn
will appear white and will not blanch when touched. Deep-partial thickness
burns are often extremely painful because there can be exposed nerve endings
due to the depth of the burn.
Full-Thickness Burn Injury

A full-thickness (third-degree) burn injury destroys the entire epidermal and
dermal layers of the skin and extends down into subcutaneous fat. A full-
thickness injury may be a variety of colors. The wound can be charred black,
cherry red, tan, or pearly white in color. This wound may present with small
fragile, thin-walled blisters that break easily and do not increase in size. Overall,
the wound is dry and leathery hard in texture (Lloyd et al., 2012). Since nerve
endings are destroyed, the wound is initially insensate but remains sensitive to
deep pressure. Because burn wounds often have a mixture of differing depths,
pain is not a good indicator of the depth of wound. This wound will leave a
residual scar and is at severe risk for contracture formation (Morgan, Bledsoe, &
Barker, 2000).
Deep Full-Thickness Burn Injury

Deep full-thickness (fourth-degree) burns destroy all skin layers and extend into
the muscle, tendon, or bone. This type of burn will be characterized by a charred
or mummified appearance (Kowalske, 2011). Deep full-thickness wounds are
challenging to close and can result in partial or total loss of function (Sahin et
al., 2012). When this type of burn involves severe damage to the underlying
structures, amputations may be warranted.
Inhalation Injury
Inhalation injuries most commonly occur in a setting of thermal injury of an
enclosed space. Signs and symptoms of inhalation injuries are typically based on
observations of the following: the presence of facial burns, singed nasal hair,
darkened oral mucosa, hoarse voice, wheezing, hypoxia, and/or cough (Dries &
Endorf, 2013; Mlcak, Suman, & Herndon, 2007). Medical diagnosis of an
inhalation injury is typically completed with a fiberoptic bronchoscopy.
A common concurrent form of injury is carbon monoxide toxicity (Mlcak et
al., 2007). Almost all products release carbon monoxide during combustion. It is
an odorless, colorless gas that has a greater affinity for binding hemoglobin than
oxygen, thus displacing oxygen and leading to asphyxia (Dries & Endorf, 2013).
During a fire, the concentration of oxygen typically drops to 10% to 15%, at
which point death from asphyxia occurs (Barillo, 2009). Hydrogen cyanide is
produced during combustion of multiple household materials and can be inhaled
during a fire. Hydrogen cyanide also decreases oxygen consumption. Carbon
monoxide and cyanide toxicity can interfere with oxygen transport at the cellular
level and affect electron transport within cells leading to death (Dries & Endorf,
2013).
Burn Shock
A complication that may occur in patients with burns in excess of 20% total
body surface area (TBSA) is burn shock (Atiyeh, Dibo, Ibrahim, & Zgheib,
2012). Burn shock can occur within 48 to 72 hours after injury (Parihar, Parihar,
Milner, & Bhat, 2008). Unlike other causes of shock, the problem is not a loss of
blood but rather the fluid or plasma portion of the circulating blood volume.
Immediately following a burn injury, an increase in capillary permeability allows
fluid in the intravascular space to shift into the interstitial space producing burn
wound edema. The effect on the cardiovascular system is a marked increase in
peripheral vascular resistance accompanied by a decrease in cardiac output. This
shift is greatest in the first 12 hours postinjury but continues for 72 hours
postinjury (Atiyeh et al., 2012). After the first 72 hours, capillary wall function
returns and gradually burn wound edema will shift back into the intravascular
volume and be excreted by the kidneys. In the presence of a burn wound >25%,
this fluid shift occurs throughout the body and edema develops in areas that have
not been burned (James, 2012). In burns <25%, the fluid shift is usually confined
to the burn area.
Hypermetabolism
Hypermetabolism is a significant concern for patients with burns. A large burn
injury triggers a significant and prolonged stress response in the body and
initiates the release of catecholamines, cytokines, and insulin (Abdullahi &
Jeschke, 2015). The release of these hormones initiates and mediates a
hypermetabolic response and increased energy expenditure and protein turnover
in the body. This hypermetabolic state will result in increased energy catabolism,
skeletal muscle catabolism, immune deficiencies, peripheral lipolysis, and
reduced bone mineralization and growth. Energy expenditure is most significant
in the first weeks postburn and then begins to decrease (Lavrentieva, 2016).
Infection
The skin provides a barrier to the external environment and offers metabolic and
immunological support. Injury to this barrier disrupts the innate immune system
and increases susceptibility to infection (Church, Elsayed, Reid, Winston, &
Lindsay, 2006). The leading cause of death following a severe burn injury is
infection (Chaudhari, Upadhyay, Bambhaniya, & Patel, 2015). The typical
systems of an infection (fever, tachycardia, and leukocytosis) are commonly
observed in persons with burn injuries, without the presence of an infection.
Therefore, the American Burn Association (Greenhalgh et al., 2007) proposed
indicators that are more specific for sepsis in burns. These include
Temperature (>39°C or <36°C), progressive tachypnea (>25 breaths/minute

not ventilated or >12 L/minute ventilated)
Progressive tachycardia (>110 beats/minute)
Thrombocytopenia (<100,000/μL, applied only after day 3
postresuscitation)
Hyperglycemia (untreated glucose >200 mg/dL, >7 units/hour insulin
infusion, or >25% increase in insulin dosing over 24 hours)
Enteral feeding intolerance (abdominal distention, residuals over two times
the feeding rate, or diarrhea >2,500 mL/24 hours)
In the event three or more of these indicators are present, the infection site
should be identified and antibiotics should be initiated. The use of topical
antibiotics, early excision and grafting of the wound areas, and strict sterile
techniques also aid in the prevention of infections.
Scars
Scars are fibrous tissue that replaces normal tissue after injury. Typical scars are
initially red in color and fade as the fibrous tissue begins to develop (Heppt et
al., 2015). Burn scars begin to form as wounds close. Burn scars are prone to
hypertrophic scar formation. Hypertrophic scars result from uncontrolled
production of fibroblasts and excess in deposition of collagen tissue (Heppt et
al., 2015). Collagen is a basic structural fibrous protein found in all tissue.
Hypertrophic scars typically present as red, raised, and rigid and generally do not
extend past the injury site (Heppt et al., 2015). Hypertrophic scars are
differentiated from keloid scar formation by the fact that they remain within the
boundary of the original wound and will eventually fade in color, flatten, and
become more pliable as they mature.
People who are most susceptible to hypertrophic scars are those who have a
genetic predisposition, have African ethnic origin, and have burns in areas of the
body involved in stretch or motion such as the shoulders and truck and areas of
prolonged healing (Heppt et al., 2015). Keloids are excessive fibrosis, nodular
proliferations that project beyond the margins of the original injury (Heppt et al.,
2015). Keloids are tender and painful and can be difficult to treat. In addition,
keloids can cause physical, cosmetic, psychological, and social concerns (Viera,
Vivas, & Berman, 2012). Keloids can occur in all skin types, but increased
prevalence is noted in the African population, and genetic predisposition may
impact keloid formation (Viera et al., 2012).
Contractures
Wound healing involves three processes: epithelialization, connective tissue
deposition, and contraction. Wound contraction is an active process generated by
fibroblasts and myofibroblasts and is one of the most powerful mechanical
forces in the body. Burn scar contracture is the shortening and tightening of the
burn scar. It is estimated that approximately 40% of those that suffer burn
injuries will develop scar contractures (Ehanire et al., 2013). Burn scar
contracture deformities are most problematic over large joints. They can severely
limit ROM and interfere with the ability to perform activities of daily living
(ADLs) (Niedzielski & Chapman, 2015). Additionally, contractures can be
disfiguring and painful and cause itching (Ehanire et al., 2013). Mature burn
scars do not have the capacity to stretch like normal skin. Children with large
full-thickness injuries may develop burn scar contractures years after their initial
burn injury as they literally grow out of their skin.
The course and prognosis of a burn injury depends on the depth and surface area
of the burn. A superficial burn will generally heal within 3 to 4 days as the
epidermis sloughs, and there will not be any residual scarring (Herdon, 2012). A
superficial partial-thickness burn will generally heal within 2 weeks, and
typically, there is no scarring; however, there are often pigmentation changes
(Kowalske, 2011). A deep partial-thickness burn will heal in 3 weeks or longer
and commonly result in significant scarring and contractures (Lloyd et al., 2012).
Because both layers of the skin are destroyed in the full-thickness burn injury,
the wound will not heal spontaneously and the healing time is dependent upon
the availability of donor sites. A full-thickness burn injury has the potential to
affect all body systems. Organ failure, sepsis, pneumonia, and cellulitis are
common complications from burn injuries. Sixty percent of deaths from burn
injuries are attributed to multisystem organ failure (Veeravagu et al., 2015).
A common cause of death in persons with burn injuries is inhalation injuries
(Mlcak et al., 2007). The presence of an inhalation injury can increase the burn
mortality rate by 20%, and there is an increased predisposition to pneumonia
(Mlcak et al., 2007). Burn injuries can also lead to a rapid and significant loss of
intravascular fluid into the interstitial space. If this is not managed, it can lead to
progressive organ dysfunction and ultimately death.
Diagnosis
The depth of the burn wound is not always clear on admission, and burn depth is
often not accurately assessed. A clinical examination of burn depth has been
found to be only 70% accurate (Bezuhly & Fish, 2012). Laser Doppler scanning,
histological assessments, and indocyanine green fluorescence are some of the
new technologies being used to increase the accuracy of burn depth
determination.
In addition to evaluating the depth of a burn, it is important to accurately
estimate the total body surface area (TBSA) involved in the burn injury. Burn
depth and burn size will guide medical and therapeutic management of the
burns. There are three common methods used to estimate extent of burn: the
Lund and Browder scale, the rule of nines, and the rule of palms. The Lund and
Browder scale (Fig. 21.3) is a diagram that consists of a two-dimensional figure
of a human body, front and back. The figure is divided into sections, each of
which represents a defined percentage of the total body surface. The evaluator
documents the injury on the diagram, estimates the amount of each section the
injury occupies, and manually adds these numbers to determine the TBSA. The
Lund and Brower scale adjusts for patients of different ages (Williams et al.,
2013). This evaluation of TBSA can be time consuming and has limited
accuracy.
A.Rule of nines. B.Lund and Browder scale. (From Irwin, R. S., Lilly, C. &
Rippe, J. M. (2013). Irwin and Rippe’s manual of intensive care medicine (6th
The rule of nines (Fig. 21.3) is a quick method that may be used to estimate
extent of burn. It divides the body surface into areas representing 9% or
multiples of 9%. This method is also susceptible to user error and often
overestimates the percentage of TBSA (Sheng et al., 2014).
The rule of palms is defined as the surface of the patient’s palm that is
roughly 1% of their TBSA (Sheng et al., 2014). Similar to the other methods, the
rule of palms also has validity concerns. This method can result in an
overestimate of the TBSA of 10% to 20% (Amirsheybani et al., 2001).
An accurate assessment of the TBSA is critical to determining care and
resuscitation methods for persons that have sustained burns. As such, several
computer-based methods have been developed and researched. Studies have
shown that the computer-based methods can be up to 99.9% accurate in
determining TBSA (Sheng et al., 2014). Mobile systems for TBSA evaluation
and remote assessment have also been developed as a means to provide
telehealth and impact the quality of the treatment in burns (Parvizi et al., 2014).
Medical Management
Major burn injuries are considered one of the most severe examples of trauma
and often require specialized care not available at all hospitals (Bezuhly & Fish,
2012). Since 2006, the American Burn Association identified criteria (Table
21.1) for burn injuries that should be transferred to a hospital with a designated
burn/trauma unit, capable of providing the specialized care required by
significant burn injuries.
TABLE 21.1 American Burn Association Criteria for Burn Center Referral
Inhalation Injuries
Treatment of inhalation injury is aimed at maintaining adequate oxygenation
through the administration of humidified oxygen by mask to maintain an oxygen
saturation level >90%. Additionally, bronchial hygiene therapy, chest
physiotherapy, early ambulation, and airway suctioning are used as part of
inhalation injury treatment protocols (Mlcak et al., 2007). Bronchoscopies are
also used in severe inhalation injuries and have been found to be effective in
decreasing the duration of mechanical ventilation compared to patients who did
not have a bronchoscopy (Carr, Phillips, & Bowling, 2009). Intubation and
ventilator support may be indicated in the presence of severe respiratory
obstruction or restrictive defects. In the presence of restrictive eschar (dark,
crusty, dead skin) on the chest or abdomen, the patient’s respiratory status may
become compromised and the patient may require an escharotomy.
Escharotomies are incisions through the eschar down to viable tissue to release
the restriction and allow for expansion of the chest wall during inspiration and
expiration (Kupas & Miller, 2010).
Management of carbon monoxide toxicity includes administration of oxygen
via a facemask or artificial airway at 100% oxygen. Hyperbaric oxygen is also
used as a treatment mechanism, but it is difficult to monitor a patient and provide
initial burn care while in the hyperbaric chamber (Dries & Endorf, 2013).
Cyanide toxicity is managed with aggressive treatment to support cardiovascular
function and may include the utilization of cyanide antidotes (Dries & Endorf,
2013).
Fluid Resuscitation
Early aggressive fluid resuscitation, which is the administration of intravenous
fluid, is required for stabilization. The fluid replacement of choice is crystalloid,
or lactated Ringer solution. Ringer solution contains sodium, chloride, calcium,
potassium, and lactate (Rowan et al., 2015). The goal of fluid resuscitation is to
maintain the intravascular volume in sufficient amounts to ensure adequate
perfusion and oxygenation to all tissues and organs with the least amount of fluid
needed.
Several fluid resuscitation practices are used. A survey found that the
Parkland formula is the most commonly used method (69.3% of respondents),
followed by the Galveston (8.9%), Brooke (6.9%), and Warden hypertonic
formulas (5.9%) (Greenhalgh, 2010). These formulas calculate the predicted
fluid needs of patients based on their burn size and weight over the course of the
first 24 hours. According to the Baxter (Parkland) formula, the patient should
receive 4 mL/kg body weight/% burn, as a volume of fluid needed for the initial
24-hour fluid resuscitation. Adequate assessment of the effectiveness of fluid
resuscitation is important, as excessive fluid overloading can cause the
phenomenon known as fluid creep. It is important to avoid fluid overloading
because this may be lead to compartment syndrome. Compartment syndrome is
internal edema within a part of the body or often an extremity that can decrease
circulation to the structure and thus putting them at risk. To avoid fluid
overloading, burn resuscitation is commonly monitored by urine output. Hourly
urine output in adults should be 30 mL/hour and 1 to 1.5 mL/kg/h in pediatrics
(Pukar, Rajshakha, Mewada, & Lakhani, 2015).
Delayed or inadequate fluid replacement results in hypovolemia, tissue
hypoperfusion, shock, and multiple organ failure. Inadequate fluid resuscitation
can lead to hypovolemia and renal failure. Once fluid resuscitation is initiated,
tissue edema in or directly surrounding the burned area may occur. If
circumferential burn wounds are present on the extremities, distal areas should
be checked frequently for compromised blood supply.
Nutritional Support
Nutritional support is needed to meet the resulting increase in basal energy
expenditure due to hypermetabolism. Multiple formulas are available to
determine the required caloric needs. Most patients cannot consume enough
calories through eating and require nutritional support via the enteral route
(nasogastric feedings). Early initiation of nutritional support via enteral feedings
will help reduce the risk of infection and mortality (Williams et al., 2009).
Burn Wound Management
Burn wound management involves the surgical debridement of nonviable skin
and wound coverage in the form of skin grafts. Debridement is the cleansing
and removal of nonadherent and nonviable tissue. Eschar, the dead tissue that
sheds from healthy tissue, facilitates bacterial access and acts as the common
denominator for burn sepsis. Daily cleansing and debridement of the burn wound
is necessary to decrease the potential for burn wound sepsis, to facilitate healing,
and to prepare the wound for grafting if this procedure is needed to achieve
wound closure.
Debridement is a painful procedure, and it is important to make sure the
patient has been medicated with analgesics and/or sedative medication prior to
starting the dressing change. Commonly used analgesics include morphine,
fentanyl, or codeine. A common drug given to sedate the patient is ketamine.
Anxiolytics, such as diazepam or midazolam, are drugs given to control anxiety.
The pain experienced at dressing changes can make a person feel anxious or
stressed (Woo, 2010). Anxiety can influence pain perception. The use of
anxiolytics can be beneficial in reducing anticipatory anxiety regarding future
dressing changes. It has been found that anxiety and pain can impact the patient
and delay the healing process (Upton & Andrews, 2014). Research is being done
to examine interventions that can minimize pain during dressing changes.
Nonpharmaceutical interventions utilized include virtual reality, massage
therapy, sensory focusing, guided imagery, relaxation techniques, and music
therapy.
Hydrotherapy is a form of wound cleansing in which water is used as a
means of decontamination of the burn site. There are concerns with the risk of
infection during the use of hydrotherapy as the patient’s wounds are typically
open and deep. Due to this concern, the prevalence of hydrotherapy use at burn
centers has decreased since 1990 (83% vs. 95%), yet continues to be used at the
majority of centers (Davison, Loiselle, & Nickerson, 2010). Showering methods
are being a more common alternative to hydrotherapy (Davison et al., 2010).
Showering methods and hydrotherapy assist in cleaning wounds, removing
topical creams, and facilitating dressing change. A mild soap, soft washcloth,
tweezers, and scissors are tools utilized to aid in debridement. To decrease the
potential for hypothermia, time in the bath is limited and the temperature of the
room should be kept at 85°F or higher (29°C) (Herdon, 2012).
Once the wound has been cleansed, nonadhesion, absorbent, and
antimicrobial dressing should be applied. The burn dressings act as a barrier to
the environment to prevent against infection and can assist in the management of
wound fluids. Dressing changes are commonly performed twice weekly or on
alternate days (Selig et al., 2012). If outer bandages become saturated with
drainage, however, it is necessary to replace them with dry outer bandages to
prevent the wicking of bacteria down to the surface of the wound.
Topical dressings are used to provide protection from contamination and
from physical damage, allow gas exchange and moisture retention, and provide
comfort (Rowan et al., 2015). There are several topical agents one can choose
from that can encourage a moist but not wet environment to promote
epithelialization and maintain moisture retention (Warner, Coffee, & Yowler,
2014). Use of silver-containing compounds has become increasingly popular
(Warner et al., 2014). Silver-containing topical agents have antimicrobial
properties and have been found to reduce wound pain (Abboud et al., 2014).
Silver toxicity, resistance, and skin discoloration are concerns with the use of
silver agents, but more research is needed to determine the true impact (Sterling,
2014). One antimicrobial agent commonly used is silver sulfadiazine 1%
(Silvadene). Silvadene is a broad-spectrum antimicrobial agent effective against
gram-positive and gram-negative bacteria with some antifungal activity.
Silvadene does not penetrate burn eschar. It controls bacterial growth only on the
surface of the wound, has few side effects, and is usually well tolerated by the
patient.
The standard of care for wound management has included early excision and
grafting (Rowan et al., 2015). Research has shown that excision within 24 to 48
hours reduces blood loss, indication, length of hospital stay, and mortality
(Saaiq, Zaib, & Ahmad, 2012). Additionally, timely closure of wounds can
prevent dehydration and assist in reducing the risk of infection (Mahjour et al.,
2015). Grafting priorities are influenced by location and size of the burn. If the
patient requires long-term intravenous fluid administration, skin grafting may be
needed on the chest to allow for insertion of a central line. Hands, because of
their functional importance, are given grafting priority. Faces and ears have a
dense cross section of dermal appendages and are given additional time to
ascertain if healing will occur without surgical intervention (Kao & Garner,
2000).
If the patient does not have available donor sites, the burn wound can be
excised down to viable tissue and temporarily closed through the application of
an allograft, xenograft, or skin substitute. An allograft is donor skin taken from
another living or deceased person. The body will predictably reject the allograft
from the wound bed 7 to 12 days after placement due to immunologic
incompatibility between the burn victim and the cadaver donor (Leto Barone et
al., 2015). These temporary means of wound closure provide the time needed to
achieve a permanent method for closing the wound.
In full-thickness injuries, the risk of bacterial entrance and fluid and heat loss
through the wound continues until the wound is closed either temporarily or
permanently. Split-thickness skin grafts from an uninjured donor site of the
patient (autograft) provide quick and permanent closure of the wound (Rowan
et al., 2015). An autograft is the surgical transplantation of the patient’s own skin
from one area to another. Donor skin is taken from areas of unburned skin. The
harvested skin is 0.008 to 0.012 in. in thickness. This leaves a wound, referred to
as a donor site, that takes about 7 to 10 days to heal. Once healed, donor sites
can be reharvested but will take longer to heal. Burn eschar is excised down to
viable tissue, and bleeding is controlled prior to placement of the skin graft.
A split-thickness skin autograft can be applied as a mesh graft or a sheet
graft. A mesh graft is a graft that has had small holes placed evenly throughout
the graft, which allows it to be expanded (2:1, 3:1). Use of a mesh graft allows
more area to be covered than the actual size of the donor skin taken. The scar
from a mesh graft often has a crosshatch appearance. Because of this, mesh
grafts are avoided in areas where there are concerns about the appearance of the
scar, such as on the face or hands. Split-thickness grafts can be applied as a sheet
graft in areas with aesthetic concerns. A sheet graft is a graft in which the donor
skin has been laid intact over the area to be grafted. Sheet grafts are traditionally
used in burn wounds of up to 20% TBSA to limit the potential cosmetic impact
(Nikkhah, Booth, Tay, Gilbert, & Dheansa, 2015). While sheet grafts are
preferred because of cosmetic reasons, limited availability of donor sites in large
burn injuries limits their use.
In large burns with limited availability of donor sites, cultured epithelium
can be used to achieve wound closure. A biopsy of unburned skin is taken and
sent to a laboratory that can grow cultured epithelium. It takes 3 to 4 weeks for
cultured epithelium to be available for grafting. The resulting grafts can be
fragile and sensitive to infection. Cultured epithelium grafts can also be
expensive, and they require a complex application process. These cultured
epithelium grafts are often reserved for patients with 50% or greater TBSA burns
(Nguyen, Potokar, & Price, 2010). Epidermal substitutes are also commercially
available (e.g., Alloderm, Biobrane) and can be used to replace the dermis or
epidermis. These substitutes can be only a few cell layers thick and do not
contain all the typical components of human dermis (Fang, Lineaweaver, Sailes,
Kisner, & Zhang, 2014). Use of stem cells to enhance wound healing is an
emerging area of research in the field of burn management.
Immediately after grafting, with either an autograft or cultured epithelium,
the graft is fragile and susceptible to loss. Factors that can cause graft failure
include shearing/motion and increased edema (Lorello, Peck, Albrecht, Richey,
& Pressman, 2014). To prevent loss of graft, the grafted area is often
immobilized in a functional position and remains in that position until there is
the establishment of circulation to the grafted area (Lorello et al., 2014). If grafts
are placed on the chest or back, the bandages are sutured to the body to decrease
the risk of shearing when repositioning the patient. Extremities are elevated to
prevent/minimize edema formation. Immobilization time is variable, and each
physician will have his or her own preference. ROM to the grafted area is
avoided until the graft is stable, which is usually about 4 to 6 days after surgery.
A burn injury is a devastating injury that may have a significant impact on
occupational performance immediately and long after the wound is healed. The
impact of a burn injury on an individual’s occupational performance is
influenced by the size, location, and depth of the burn injury. A burn injury
affects a number of client factors and can profoundly impact an individual’s
ability to independently perform ADLs, instrumental activities of daily living
(IADLs), education pursuits, work, play, leisure, and/or social participation
activities. Burn injuries that have the greatest potential to impact occupational
performance include deep partial-thickness or full-thickness burns that result in
more severe scar formation, contractures, and joint restrictions and burns that
involve major joints and account for a high TBSA.
During the initial evaluation, it is important to complete a thorough
evaluation as soon as possible. Several client factors and performance skills
should be critically evaluated so that the most effective treatment plan can be
developed to ensure the optimal occupational performance for the client.
Client Factors
A significant deficit experienced after a burn injury is decreased joint mobility
and joint function. This includes the patient’s passive and active ROM to any
joints that may have been involved in a burn injury in addition to surrounding
joints. During the emergency phase and fluid resuscitation, edema formation is
expected and may be profound in patients. The edema formation will impact
joint function and mobility leading to difficulties with ADLs and functional
activities. Joint mobility and function are also impacted due to the formation of
scarring and contractures. Scars that cross a joint may often lead to reduced
ROM in that joint. It has been found that as little as a 10% reduction in joint
ROM will significantly impair joint function (Fearmonti et al., 2011).
Muscle power and muscle endurance are client factors that are also
commonly impacted after a burn injury. Burn survivors often experience long
periods of immobilization during the acute phase of the medical management.
Additionally, the body experiences many physiological responses as it heals
from a burn injury. Both the prolonged periods of immobilization and the body’s
physiological responses can reduce a person’s muscle endurance and muscle
power. Muscle power and endurance may also be limited due to scar formation
and limited joint ROM. If minimal joint range is available or the joint’s mobility
is limited due to scar formation, the muscle power exerted by the joint may be
limited. Limited muscle endurance and power can significantly impact many
facets of one’s occupational performance. Research suggests that participation in
structured progressive resistance exercise and cardiopulmonary endurance
programs can aid in rapid restoration of functional independence (Paratz,
Stockton, Plaza, Muller, & Boots, 2012).
Another client factor that is important to evaluate with this population is
mental functions. Due to the traumatic and catastrophic nature of a burn injury,
burn survivors may suffer symptoms of posttraumatic stress disorder, depression,
and body image and self-image dysmorphia. A study of hospitalized burn
patients found 55% of patients suffered from at least one mental disorder at a 6-
month follow-up (Palmu, Suominen, Vuola, & Isometsä, 2011). The specific
mental functions of “emotional” and “experience of self and time” are often
overshadowed in persons who sustain burn injuries as medical personnel are
focusing on survival. Support and interventions focused on loss, grief,
acceptance of body image and self-image, posttraumatic stress disorder, anxiety,
and depression should be provided. These psychosocial mental function-focused
interventions are necessary to achieve a burn survivor’s highest level of
occupational performance. Palmu, Partonen, Suominen, Vuola, and Isometsä
(2015) found that burn survivors’ level of functioning was predicted strongly and
consistently by mental disorders, most specifically depression.
It is important to recognize and assist both patients and families in dealing
with the psychological and psychosocial impact of a major burn injury. Burn
support groups can be helpful in assisting patients and families in dealing with
the lifelong disfigurement and dysfunction that may result from a major burn
injury. Having patients meet with a burn survivor can help them realize what
challenges they may face and ways to overcome these challenges. Family
members may experience many emotions such as fear, guilt, or sadness. If these
emotions are not addressed, family members may feel the need to “take care of
their loved one” and to do things for the patient that he or she needs to do
independently. Having family members meet with another burn survivor and
their families will help prepare them for the challenges ahead and assist them to
deal with their emotions. This preparation will enable the family to provide the
patient both the support and independence they will need to achieve the best
outcome.
It is critical that early interventions, comprehensive home programs and
patient and family education, and discharge recommendations are provided to
patients to address and prevent a significant impact on client factors to limit the
impact on a person’s occupational performance.
CASE-STUDY 1
Lyla, a 5-year-old girl, is playing outside when her father arrives home. He
is having difficulty with his car, which is overheating. Unaware that Lyla
has come over to watch him work on the car, he removes the radiator cap.
Hot radiator fluid and steam strikes Lyla, and she sustains a 32% mixed
partial- and full-thickness burn injury to her face, neck, chest, and scattered
area on her bilateral arms.
During the emergency phase, she required fluid resuscitation and
intubation to maintain her airway during the initial fluid shift and resulting
swelling. She was extubated on postburn day 3 and has had no further
problems with her airway. Three weeks have passed, and Lyla has had two
surgeries. During the first surgery, mesh grafts were placed on her chest and
arms. During the last surgical procedure, a sheet graft was placed on her
neck. Her grafts are intact and healing well, but scattered open areas remain
at the margins of the grafts. Lyla has donor sites on her bilateral thighs and
buttock, which are healing without difficulty.
Neuromuscular components of ROM, strength, and soft tissue integrity
have all been affected by the burn injury Lyla sustained. She has
participated in ROM exercises since she was extubated. ROM exercises
were halted for a few days, after the grafting procedures, to facilitate
adherence of the graft. Currently, Lyla has some restriction of ROM to
bilateral axillary areas and is at risk for hypertrophic scarring. While she
currently does not demonstrate any restriction of neck ROM, she is at great
risk for developing contractures in this area. ADLs and play or leisure
activities are the occupational performance areas that have been affected.
Currently, Lyla has few limitations on ROM to most joints, but because of
the significant pain and anxiety she experiences during ROM, she is
hesitant and resistant to movement. Lyla’s parents express a great deal of
difficulty with the discomfort that Lyla has experienced during therapy and
are hesitant to make her do things that may cause pain. Lyla’s father
expresses a lot of guilt surrounding the circumstances that caused the burn
injury.
CASE-STUDY 2
Rahul is a 45-year-old male who was employed as an electrician. He
was working outdoors with a coworker on a nonfunctional power box when
it exploded, and he was engulfed in flames that caused third-degree burns to
75% of his body. He sustained partial- and full-thickness burns to his head,
neck, chest, abdomen, bilateral upper extremities, genital and perineal area,
and bilateral upper legs. His ankles and feet were spared as they were
protected by his work boots. During the explosion, Rahul also sustained
inhalation injuries.
Rahul was taken to an urban, local burn facility and remained in the
burn ICU for more than 3 months. During this time in the burn ICU, he
received early aggressive fluid resuscitation. Due to excessive fluid
overloading, he experienced fluid creep. The fluid creep led to abdominal
compartment syndrome and required an emergency fasciotomy to release
the excessive pressure. In addition, Rahul was intubated and received
ventilator support due to severe respiratory obstruction from the inhalation
injury. Rahul underwent over 20 grafting procedures to assist with
management of his burn injuries. After 3 months, he was transferred to a
local rehabilitation hospital.
At the rehabilitation facility, he presented with extremely low
endurance, significant pain, and symptoms of depression, which limited his
ability to participate in the required 3 hours of therapy per day. Due to his
prolonged period of decreased mobility in the ICU and poor participation at
the rehabilitation facility, Rahul began to demonstrate significant limitations
in his head, neck, and bilateral upper extremity ROM. Contractures and
hypertrophic scarring were present in the bilateral axilla and anterior neck.
Rahul also presented with webbing of the fingers on his left hand. He
experienced persistent open areas on the dorsum of the hands, which
limited his ability to use them functionally. Rahul was dependent to
maximal assistance with all ADLs.
Rahul is married and has two children aged 5 and 8 years. His facial
burns are frightening to his children, and his depression has begun to
impact his marriage. Rahul has begun to pull away from his family and
requested that they no longer visit him at the rehabilitation center. Because
Rahul was injured at work, he is receiving worker’s compensation benefits
and plans to stay at the rehabilitation facility for approximately 12 weeks.
RESOURCES
Herndon, D. N. (Ed.) (2012). Total burn care (4th ed.). London: WB Saunders.
Associated online support at: www.totalburncare.com
American Burn Association
311 S. Wacker Drive, Suite 4150 Chicago, IL 60606
Tel: 312-642-9260
Fax: 312-642-9130
E-Mail: [email protected]
www.ameriburn.org
Shriners Burn Institutes
2900 Rocky Point Dr
Tampa, FL 33607-1460
Tel: 813-281-0300
http://www.shrinershospitalsforchildren.org/care/burn-care
The Phoenix Society for Burn Survivors, Inc 1835 R W Berends Dr SW
Grand Rapids, MI 49519-4955
Tel: 800-888-2876 or 616-458-2773
Fax: 616-458-2831
www.phoenix-society.org
Burn Summer Camps for Kids
(Information available at Kids Camps) 909 N. Sepulveda Blvd., 11th Floor El
Segundo, CA 90245
Tel: 877-242-9330
Fax: 310-280-5177
www.kidscamps.com/special_needs/burn.html
Children’s Burn Foundation
5000 Van Nuys Boulevard, Suite 450
Sherman Oaks, CA 91403
Tel: 818-907-2822
Fax: 818-501-4005
Toll free: 800-949-8898
www.childburn.org
International Burn Foundation 6915 Ebenezer Church Road
Hillsborough, NC 27278
Tel: 919-471-4714
www.internationalburnfoundation.org
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C H A P T E R
22 Progressive Neurodegenerative
Disorders
KEY TERMS
Benign Demyelination Dopamine Familial Lewy body pathology Motor neurons
Myelin sheath Non–tremor-dominant PD
Plaques Preclinical period Primary progressive Prodromal period Relapsing-
remitting-progressive Relapsing-remitting-nonprogressive Sporadic Substantia
nigra Symptomatic period Tremor-dominant PD
Shortly after the birth of her second child, Joan started getting the sensation of
pins and needles in her hands and feet. Within a couple of months, she noticed
some numbness and weakness in her arms and legs. She was having difficulty
walking for long distances and began to worry that she might drop her 2-year-old
son or even her newborn. When the children napped in the afternoon, she found
herself slumped on the couch for a much-needed rest. Convinced that this was
just part of her postpartum recovery, Joan did not inform her doctor of this
difficulty. However, when she found herself struggling one day to focus on the
words of her son’s bedtime story, she decided to seek medical advice.Over time,
Joan found out that she had a progressive neurodegenerative disorder (PND)
called multiple sclerosis (MS). PNDs are a group of diseases that affect various
areas of the central nervous system (CNS), are chronic in nature, and cause a
deterioration of function over time. This chapter discusses three of the most
common PNDs: MS, Parkinson’s disease (PD), and amyotrophic lateral sclerosis
(ALS).There is little known about the underlying etiology and there is no known
cause of any of these three PNDs, but research indicates that the etiology is a
combination of interrelated factors (Armon, Kurland, Beard, O’Brien, & Mulder,
1991; Kenealy, Pericak-Vance, & Haines, 2003). These include genetic
predisposition, viruses, antigens, infectious diseases, and environmental factors.
A genetic predisposition is suspected because these diseases are more prevalent
among families and certain racial groups. Viruses, antigens, or infectious
diseases and their resulting autoimmune response also may be involved as a
cause of these PNDs (Nylander & Hafler, 2012). Specific viruses have not been
isolated, but particular interest has focused on a viral subgroup called
retroviruses. What causes them is unknown, and they can remain silent for years
before the onset of a disease. Environmental factors, including exposure to such
toxins as lead or pesticides, have also been associated with a higher incidence of
PND (Armon et al., 1991; Kamel et al., 2006). None of these factors, however,
have been isolated as the single cause of any of the PNDs.
Multiple Sclerosis
MS is a debilitating immunological and neurodegenerative disease in which the
genetically susceptible person’s own immune system attacks the myelin sheath
that surrounds the brain, spinal cord, and optic nerve (Kalb, 1996; Kenealy et al.,
2003; Nylander & Hafler, 2012). This process is called demyelination. MS is
characterized by chronic inflammation and diffuse demyelination not only in the
white matter but also in the gray matter and the axons (Compston & Coles,
2002). Demyelination of the neurons in the CNS results in scar tissue formation
or plaques that reduce the axons’ ability to conduct impulses (Kenealy et al.,
2003). The location of demyelination varies from person to person. The visual,
motor, sensory, cognitive, psychological, and bowel and bladder systems can be
affected. See Figure 22.1.
Figure 22.1 Nerve cell in multiple sclerosis. (From Anatomical Chart Co.)
Etiology
Why the attack on the myelin sheath begins is unknown because the exact cause
of MS is unknown. It is hypothesized to be a combination of genetic
susceptibility and environmental factors, such as a virus or infection (Compston
& Coles, 2008; Murray, 2002). The latest evidence regarding MS suggests that a
viral infection triggers the immune system to wage an attack on the nerve cells
of people who are genetically susceptible (Hanson & Cafruny, 2002). MS is
polygenetic and although it does not follow an obvious pattern of inheritance
(Wakerley, Nicholas, & Malik, 2012), it has a familial recurrence rate of 20%
and is more common in first-degree relatives versus more distant relatives
(Compston & Coles, 2008). Recent research has identified some potential
specific genetic associations with the HLA gene on chromosome 6, and certain
leukocyte antigen types (DR15 and DQ6) may play an important role in the
abnormal response of the T cells (Wakerley et al., 2012). Other factors that may
contribute to the cause of MS include smoking (Hernan, Olek, & Ascherio,
2001), a lack of ultraviolet light exposure resulting in vitamin D deficiency
(Berlanga-Taylor, Disanto, Ebers, & Ramagopalan, 2011), heavy metal toxins in
the environment (Noonan et al., 2010), and a history of viral infections such as
mononucleosis and Epstein-Barr virus (Compston & Coles, 2008; Wakerley et
al., 2012).
MS is the most common nontraumatic neurodegenerative disorder among adults
under 40 years of age (Carrithers, 2014). It affects approximately 1% of the
population of the United States, and there is evidence that those numbers are
increasing (Kalb, 1996; Kenealy et al., 2003; Noonan, Kathman, & White,
2002b). Currently, an estimated 400,000 people in the United States have MS
(Harrison, 2014) and 100 per 100,000 in the United Kingdom. The distribution
of prevalence varies geographically. The closer a person lives to the equator, the
less likely he or she is to have MS (Matthews, 1993; Noonan et al., 2010). For
example, in the southern United States, the rate of incidence is 20 to 39 for every
100,000 persons. In the northern United States and Canada, the rate is more than
40 out of every 100,000 persons (Matthews, 1993). If a person migrates from a
location near the equator prior to age 15, the incidence of MS is reduced, which
contributes to the theory of a possible childhood infection having a role in the
onset (Hanson & Cafruny, 2002) or of the influence of limited exposure to
ultraviolet light (Noonan et al., 2010).
The incidence of MS also varies according to gender. MS affects females
more often than males at a ratio of between 2 and 3 females for every 1 male
with the disease (Hanson & Cafruny, 2002; Harrison, 2014). The current trend
supports an increasing prevalence among women with approximately 125 per
100,000 women and 40 per 100,000 men in the United States (Noonan et al.,
2002b; Wakerley et al., 2012).
In general, MS has a global distribution with the frequency increasing as the
distance from the equator increases and it occurs more frequently among people
of European ancestry than other white racial groups (Compston & Coles, 2008).
Even within northern countries, there is a distribution of increasing incidence in
the northern parts of the countries (Wakerley et al., 2012). People of
Scandinavian and Scottish descent are most susceptible to MS. It is twice as
common among Caucasians as other races and it is rare among people of
Mongolian, Japanese, Chinese, Native American, Eskimo, African Black and
Aborigine descent (Hanson & Cafruny, 2002; Wakerley et al., 2012). If a person
migrates from a low-risk region to a high-risk region before 15 years of age, the
low risk associated with the person’s region of birth remains (Compston &
Coles, 2008).
Studies of offspring, twins, siblings, and adopted children demonstrate that
the closer a relation genetically, the more likely a person is to have MS
(Compston & Coles, 2008; Neilsen et al., 2005). The incidence of MS decreases
as biological relationship decreases. If a person has MS, the identical twin, full
siblings, half siblings, and offspring have increased risk of also having MS in
that order (Compston & Coles, 2008; Kenealy et al., 2003). In adoption studies,
increased risk was only noted in biological relatives (Compston & Coles, 2008).
Signs and Symptoms
Because MS can affect nearly any part of the CNS, the signs and symptoms of
MS can vary greatly (Harrison, 2014). In general, the motor, sensory, visual, and
autonomic systems are affected by MS. Some specific symptoms of MS include
visual deficits such as diplopia (double vision) or unilateral optic neuritis,
sensory disturbances such as dysesthesia or paresthesia, urinary incontinence or
retention, erectile dysfunction, muscle weakness, gross and fine motor
incoordination, pain, weakness, spasticity, fatigue, ataxia, dysphagia, dysarthria,
vestibular dysfunction, and cognitive or emotional disturbances (Compston &
Coles, 2008; Wakerley et al., 2012). Each person with MS has symptoms that
result from lesions in specific areas of the CNS. The types of symptoms, their
intensity, and their effects on the person’s functional status are highly
individualized (Delisa, Hammond, Mikulic, & Miller, 1985). Table 22.1 includes
a summary of common signs and symptoms associated with the various lesion
sites.
TABLE 22.1 Common Symptoms, Signs, and Medical Management of

Multiple Sclerosis
Adapted from Compston, A., & Coles, A. (2008). Multiple sclerosis. The Lancet, 372, 1502–1517.
Visual disturbances often are among the earliest signs of MS (Rocca, Messina, &
Filippi, 2013). They usually appear as a partial loss of vision (scotoma), double
or blurred vision, or ocular pain (Harrison, 2014). Sudden loss of vision with
pain in or behind the eye is caused by optic neuritis. These early symptoms may
subside after 3 to 6 weeks without any residual deficit. For others, visual loss
may be insidious and painless. Nonetheless, nearly 80% of all persons who have
MS have some loss of visual acuity. Oculomotor control may also be affected
due to lesions of the supranuclear connection to the oculomotor nuclei in the
brainstem. As a result, the person loses horizontal eye movement either
unilaterally or bilaterally.
The individual with MS can experience a variety of other sensory
disturbances such as numbness; impairment of vibratory, proprioceptive, pain,
touch, and temperature sensation; and distortion of superficial sensation.
Because of these sensory losses, the person also may lose various perceptual
skills such as stereognosis, kinesthesia, and body scheme (Umphred, 1990).
Fatigue is the most common complaint and is often identified as the most
debilitating symptom (Filippi et al., 2002). Increased energy is required for
nerves to conduct their impulses in a demyelinated nervous system, making it
difficult for the individual to initiate movement and perform sustained activities.
The individual also may experience muscle weakness. As the disease progresses,
the person requires more frequent rest periods between activities, and decreased
levels of activity lead to further debilitation.
Approximately 40% to 70% of individuals with MS experience some change
in their cognitive ability (Rocca et al., 2013). Short-term memory, attention,
processing speed, visuospatial abilities, verbal fluency, and executive functions
have all been identified as deficit areas related to MS (Bobholz & Rao, 2003;
Lyros, Messinis, Papageorgiou, & Papathanosopoulos, 2010). As with all aspects
of MS, there is considerable variability among individuals with the disease
depending on the area of the brain affected (Bobholz & Rao, 2003). An
emotional component to this disease results in some individuals having bouts of
depression, euphoria, or lability caused by lesions in the frontal lobes of the
brain (Feinstein, 2006).
MS is the most common PND found in young adults and is usually diagnosed
between the ages of 20 and 40 years (Matthews, 1993). Peak age of onset is
about 30 years, and it is rarely seen in children or diagnosed in adults older than
age 60 (Wakerley et al., 2012). The clinical course of this disease is variable
among individuals, but it can be roughly organized into four types or patterns
including benign, relapsing-remitting-nonprogressive, relapsing-remitting-
progressive, and primary progressive.
The first type of MS is benign, in which the person experiences one or two
episodes of neurological deficits with no residual impairments. This person’s
chance of remaining symptom free increases with each nonsymptomatic year.
The next pattern of progression is relapsing-remitting-nonprogressive. In this
pattern, the person returns to the previous level of function after each
exacerbation with no residual deficits. With the third type, relapsing-remitting-
progressive, however, the person has some residual impairment with each
remission. The course of this type is unpredictable with varied patterns of
exacerbation and remission. Finally, there is the primary progressive pattern,
which involves a steady decline in function without remissions and
exacerbations. Approximately 65% to 85% of people have one of the relapsing-
remitting forms of MS in which they experience neurological symptoms
followed by complete or partial recovery (Hanson & Cafruny, 2002; Harrison,
2014), but about 25% of these individuals will develop a progressive form of MS
in which they do not experience remissions (Wakerley et al., 2012).
Approximately 10% to 20% experience the primary progressive pattern of
decline in function without periods of remission of symptoms from the outset
(Compston & Coles, 2008; Harrison, 2014; Noonan et al., 2010; Nylander &
Hafler, 2012).
The course of MS varies not only among the subtypes but also among
individuals. In general, progression of symptoms begins around 40 years of age
and the clinical course evolves over several decades (Compston & Coles, 2008).
Ten years after the disease onset, about 10% will be wheelchair bound and about
50% will be unable to work (Wakerley et al., 2012).
The median time from disease onset to death is around 30 years (Compston
& Coles, 2008). This prognosis, however, depends on the time of onset. People
diagnosed with MS in their 20s have a prognosis of approximately 46 to 60
years, whereas people diagnosed with MS in their 60s have a prognosis of
approximately 13 to 22 years (Hurwitz, 2011).
Diagnosis
The diagnosis of MS involves excluding alternate neurological conditions that
share the symptomatology of MS such as systemic lupus erythematosus,
neuromyelitis optica, acute disseminated encephalomyelitis, and
neurosarcoidosis (Kenealy et al., 2003; Rocca et al., 2013; Wakerley et al.,
2012). Because there are no specific MS biomarkers, the physician will use a full
assessment of clinical symptoms combined with testing to aid in diagnosis
(Harrison, 2014). The McDonald criteria combine the assessment of these
clinical symptoms with the outcome of magnetic resonance imaging (MRI) and
cerebral spinal fluid (CSF) analysis to make a definitive diagnosis of MS
(Harrison, 2014; Wakerley et al., 2012). According to these criteria, there are
three combinations of clinical symptoms and test results that can provide the
diagnosis of MS. The first criterion is two or more relapses of clinical symptoms
and evidence of two or more objective lesions. The second criterion that may be
used includes one relapse and evidence of one objective lesion, plus a positive
CSF with 2+ lesions as demonstrated on MRI or a further relapse. The last
criterion includes insidious neurological progression suggestive of MS plus a
positive CSF and MRI evidence of 9+ lesions in the brain, 2+ lesions in the
spinal cord, or 4 to 8 lesions in the brain and one lesion in the spinal cord.
Surgical intervention is not part of the routine care given for MS. There are some
medications, however, currently available to provide some overall disease
modification, and several medications are used to alleviate the myriad symptoms
caused by MS (Carrithers, 2014). Disease-modifying drugs such as interferon
β-1b, glatiramer acetate, azathioprine, and fingolimod show some potential to
provide a reduction in relapses in the progression of MS (Carrithers, 2014;
Harrison, 2014; Lyros et al., 2010; Wakerley et al., 2012). Second-line disease-
modifying treatments that promote antibodies include natalizumab, rituximab,
and alemtuzumab (Carrithers, 2014). Possible future treatments include antiviral
medications, vaccinations, transplantation of Schwann cells, cell lines or stem
cells, and/or gene therapy (Noseworthy, 2003). Other antibody treatments, such
as anti-LINGO-1, are being tested for the potential to regenerate myelin
(Ledford, 2015). Teriflunomide to inhibit rapidly dividing T cells and
alemtuzumab to kill T cells show some promise in the reduction of relapses
(Wakerley et al., 2012).
There are several medications prescribed to manage the symptoms of MS.
See Table 14.1 for a complete list. Beta-blockers may be prescribed to treat
spasticity. Anticholinergic drugs are used to treat difficulty swallowing. Several
different antidepressant drugs may be prescribed to treat changes in affect.
Various painkillers may be prescribed and steroids may be used to reduce nerve
inflammation (Harrison, 2014). Cannabis extract has been used to treat both pain
and spasticity (Wakerley et al., 2012). Acetylcholinesterase inhibitors such as
donepezil, rivastigmine, and galantamine, which were developed for the
treatment of Alzheimer’s disease, have shown some promise for alleviating
cognitive symptoms such as attention, information processing, and
memory/learning (Bobholz & Rao, 2003; Lyros et al., 2010).
Nonpharmacological interventions that have been found to alleviate some
symptoms of MS include cognitive rehabilitation, occupational therapy, physical
therapy, psychotherapy, and early nursing education (Bobholz & Rao, 2003;
Harrison, 2014; Lyros et al., 2010; Wassem & Dudley, 2003). Lifestyle changes,
such as a healthy diet and adequate sleep, may be helpful (Harrison, 2014). Low-
impact exercise with a gradual increase in intensity, duration, and frequency was
also found to be effective for reducing fatigue in adults with MS (Neill, Belan, &
Reid, 2006). Bone marrow transplants intended to reset the immune system have
also shown promise for reducing relapses, but because of the neurodegenerative
aspect of MS, this treatment has not been able to thwart the disease progression
(Wakerley et al., 2012).
Parkinson’s Disease
Parkinson’s disease (PD) is a PND characterized by death of dopaminergic
neurons in the substantia nigra pars compacta and the presence of Lewy bodies
(Kalia & Lang, 2015; Zhang, Dawson, & Dawson, 2000). The substantia nigra,
which is located in the basal ganglia, produces dopamine, a neurotransmitter,
and transports it to the dorsal striatum (see Fig. 22.2). The decrease in dopamine
leads to deficits in the speed and quality of motor movements, postural stability,
cognitive skills, and affective expression (DeLong, 2000; Duvoisin & Sage,
1996). In addition to dopaminergic denervation, there is also evidence of
serotonergic loss as well (Politis & Niccolini, 2015). The progression of
serotonergic denervation, however, happens at a slower pace.
Figure 22.2 Pathophysiology of PD. (From Smeltzer, S. C., Bare, G. B., Hinkle,
J. L. (2008). Brunner & Suddarth’s textbook of medical surgical nursing (11th
Neuronal degeneration progresses beyond the substantia nigra and the brainstem
and can affect other neurotrasmitter systems (Zhang et al., 2000). Lewy body
pathology is a process in which proteins abnormally fold and form intracellular
inclusions within the cell body and neurons (Kalia & Lang, 2015). The
progression of Lewy body pathology stereotypically progresses through six
stages over time and throughout the CNS beginning with the peripheral nervous
system and progressing through the pons, spinal cord gray matter, midbrain,
basal forebrain, limbic system, thalamus, and temporal cortex before affecting
multiple cortical regions of the brain (see Table 22.2) (Braak et al., 2003).
Neuroinflammation is another typical feature of PD (Kalia & Lang, 2015).
TABLE 22.2 Braak Model of Lewy Pathology in PD
There are variations in the clinical and pathological presentation of PD, and
there is overlap with other parkinsonian disorders (Albanese, 2003). Two
subtypes of PD have been proposed that include tremor-dominant PD with
relative absence of other motor symptoms and nontremor-dominant PD with
akinetic-rigid syndrome and postural instability gait disorder (Kalia & Lang,
2015).
Etiology
The underlying cause of PD is unknown although there is some evidence that
implicates an interaction of genetic and environmental factors (Kalia & Lang,
2015; Kamel et al., 2006). Two types of PD include familial PD in which there
is a genetic association and the more common type called sporadic PD. Several
genetic factors, such as linkages to chromosomes 2 and 4, have been identified
in familial PD (Macphee & Stewart, 2012; Zhang et al., 2000). As many as 43%
of people with familial PD have an affected relative, and a predisposition for
developing PD was found to significantly more common in monozygotic twins
versus dizygotic twins (Macphee & Stewart, 2012). The involvement of genetic
factors in sporadic PD has not been fully established, but increasing evidence
points to an interaction with environmental factors (Kalia & Lang, 2015; Kamel
et al., 2006).
The environmental factors that have been associated with PD include dietary
intake, exposure to environmental elements, and a history of head trauma. Diet
may not be a primary cause of PD, but many studies have found an association
between diet and the risk for PD suggesting a role in the susceptibility to the
disease (Gao et al., 2008). Some examples include the following: exposure to
iron sources and intake of too much iron supplement was associated with
increased risk of PD (Logroscino, Gao, Chen, Wing, & Ascherio, 2008),
increased dairy consumption was associated with increased risk of PD in men
(Chen et al., 2007), and higher total blood cholesterol levels were associated
with decreased risk of PD in women (de Lau, Koudstaal, Hofman, & Breteler,
2006). The use of beta-blockers and antipsychotics also has been found to
enhance the risk of PD as does the consumption of well water (Kalia & Lang,
2015).
Exposure to various environmental elements is also a risk factor for
developing PD. Several studies have found that living in rural areas, drinking
well water, having an agricultural occupation, and being exposed to pesticides
can each increase a person’s risk for PD (Kalia & Lang, 2015; Kamel et al.,
2006; McCormack, Thiruchelvam, Mannig-Bog, Thiffault, Langston, 2002;
Priyadarshi, Khuder, Schaub, & Priyadarshi, 2001).
Parkinson’s disease (PD) is the second most common neurodegenerative
disorder (Kalia & Lang, 2015; Zhang, Dawson, & Dawson, 2000). Worldwide,
the prevalence of PD is estimated at 1% of the population above the age of 60
years, with estimates as high as one million cases in the United States (Connolly
& Lang, 2014; Kamel et al., 2006). The prevalence and incidence increase
greatly with age to around 2% in the population over 80 years of age (Macphee
& Stewart, 2012). Because of the aging of the world’s population, the prevalence
of PD is expected to continue to increase. PD is more prevalent in Europe, North
America, and South America compared to African, Asian, and Arabic countries
(Kalia & Lang, 2015).
The incidence of PD ranges from 10 to 18 per 100,000 people per year
(Kalia & Lang, 2015). PD affects males slightly more than females with a 3:2
ratio and varies among races. In the United States, the incidence is greatest
among people who are Hispanic, followed by Caucasians, Asians, and African
Americans in descending order (Kalia & Lang, 2015). PD is more common in
urban counties than in rural counties and is more concentrated in the Midwestern
and Northeastern United States (Willis et al., 2010).
Signs and Symptoms
There are motor, cognitive, psychiatric, and autonomic symptoms associated
with PD. The primary motor symptoms are resting tremor, muscle rigidity, and
bradykinesia (Albanese, 2003; Connolly & Lang, 2014) (see Table 22.3). The
most obvious and familiar of these symptoms is tremor, which is usually noted
initially in the hand on one side and sometimes in the foot. In the hand, the
movement is frequently described as “pill-rolling.” The tremors are usually
variable, and they disappear when the person is asleep or calmly resting and they
increase under stress or intense mental activity (Duvoisin & Sage, 1996).
Infrequent eye blinking is often an early sign of PD followed by progressive loss
of facial expression. Other early nonmotor symptoms include hyposmia (reduced
olfactory sense), fatigue, depression, rapid eye movement (REM), sleep behavior
disorder, and constipation (Connolly & Lang, 2014).
TABLE 22.3 Primary and Secondary Symptoms, Signs, and Medical

Management of PD
Secondary symptoms of PD include gait disturbances referred to as a
“festinating gait” (short-stepped or shuffling with reduced arm swing), dexterity
and coordination difficulties, involuntary immobilization, micrographia (small
handwriting), cognitive impairments (visuospatial, memory, and frontal lobe
functions), sensory loss, muffled speech (Liu et al., 2015), frequent swallowing,
postural instability, poor balance, oculomotor impairments, reduced perception
of and expression of emotions, sleep disturbances (Politis & Niccolini, 2015; Yu,
Tan, & Wu, 2015), reduced bowel and bladder function, painful cramping,
sexual dysfunction, low blood pressure, seborrhea, depression or anxiety, and
fatigue (Breitenstein, Van Lancker, Daum, & Waters, 2001; Carbon & Marie,
2003; Chen, Schernhammer, Schwarzschild, & Ascherio, 2006; Rearick,
Stelmach, Leis, & Santello, 2002). This array of symptoms varies among people
with PD. It is highly unlikely that any person with PD would develop all of the
symptoms listed above. Some people may not experience a specific symptom,
whereas for another person, that symptom might be a major complaint. For
example, some people may experience cognitive deficits, such as executive
functions impairment, as the initial symptoms, while others may never
demonstrate any cognitive decline.
PD is usually first diagnosed when a person is older than 50 years with the
average onset age at about 60 years. It rarely affects people younger than age 40
(Duvoisin & Sage, 1996; Macphee & Stewart, 2012). As with MS, the
progression of PD differs with each person. In general, PD is a slow, progressive
disorder (Macphee & Stewart, 2012). The three phases of the disease include the
preclinical period when neurons have begun to degenerate, but no symptoms
are yet evident (Albanese, 2003; Macphee & Stewart, 2012). In this first phase,
there may be some peripheral neuroinflammation that can be found with medical
testing, but the implications of this are still being researched (Chen, O’Reilly,
Schwarzschild, & Ascherio, 2008). The second phase of PD is the prodromal
period that can last months or even years. During this phase, generalized
symptoms such as depression, anxiety, constipation, REM, behavior disorder,
and fatigue may appear (Kalia & Lang, 2015; Macphee & Stewart, 2012). The
third phase is the symptomatic period when the classic motor PD symptoms are
evident followed by progressive nonmotor symptoms (Albanese, 2003; Kalia &
Lang, 2015; Macphee & Stewart, 2012). According to a clinical scale by Hoehn
and Yahr, the third phase can be divided into five stages, as follows.
Stage I: Signs of PD are strictly one-sided, affecting one side of the body only.
Stage II: Signs of PD are bilateral and balance is not impaired.
Stage III: Signs of PD are bilateral and balance is impaired.
Stage IV: PD is functionally disabling.
Stage V: The person is confined to bed or a wheelchair.
Although there is a general progressive worsening of symptoms, the progression
through these stages is variable for each person. Usually, a person will have PD
for 15 to 20 years before entering the most severe stages (Kalia & Lang, 2015).
Some people may be in the first asymptomatic, preclinical phase and remain
there until death (Albanese, 2003). There are also fluctuations within each stage.
The loss of function is not a linear progression. Each person experiences some
periods of improvement scattered throughout the progressive loss of function.
Because of advances in medical treatment, life expectancy is not significantly
affected by a diagnosis of PD.
Diagnosis
Historically, a definitive diagnosis of PD could only be made postmortem and
there are still no diagnostic tests that allow for a definitive diagnosis in the early
stages (Albanese, 2003; Kalia & Lang, 2015). Therefore, a person may initially
be given a diagnosis of probable PD. To determine a diagnosis of PD, the
physician observes the current clinical symptoms, eliminates other
neurodegenerative diseases as the cause of those symptoms, and evaluates the
person’s response to medications used to treat PD (Macphee & Stewart, 2012).
The main clinical symptoms that denote PD are bradykinesia plus rigidity and
resting tremor (Kalia & Lang, 2015). Additional clinical assessment may include
standard tests for olfactory function and REM sleep behavior disorder.
Recent advancements in the development of medical diagnostic tools have
aided in a more definitive diagnosis as the disease progresses. Positron emission
tomography (PET) and single photon emission computed tomography (SPECT)
can be used to assess the density of presynaptic dopaminergic terminals within
the striatum (Kalia & Lang, 2015), and genetic testing may be used to determine
familial PD (Merims & Freedman, 2008). Positive genetic test results without
the presence of symptoms, however, do not indicate a definitive diagnosis of PD
(Kalia & Lang, 2015).
There are no medications that inhibit the progression of PD. The medications
usually prescribed to treat the primary motor symptoms of PD aim to increase
the concentrations and uptake of dopamine. These medications include
levodopa, dopamine agonists, and monoamine oxidase type B inhibitors
(Connolly & Lang, 2014; Kalia & Lang, 2015). Specific medications in these
categories may include levodopa-carbidopa, levodopa-benserazide, piribedil,
ropinirole, bromocriptine, and rasagiline. There are many side effects associated
with long-term use of these medications, and those include motor and nonmotor
fluctuations, nausea and loss of appetite, dyskinesia, impulsive and compulsive
behaviors, hallucinations, and psychosis (Connolly & Lang, 2014; Kalia & Lang,
2015).
There are several surgical procedures that have been developed to treat the
symptoms of PD. These procedures include thalamotomy, pallidotomy, deep
brain stimulation, and continuous device-aided drug delivery (DeLong, 2000;
Katzenschlager, 2014; Merims & Freedman, 2008). Thalamotomy is a surgical
procedure in which heat via an electrode or gamma-knife radiosurgery is used to
destroy part of the thalamus. The thalamus is an area of the brain involved in
movement. A thalamotomy can reduce tremors associated with PD and may
sustain the improvement for over 10 years. A pallidotomy is a surgical procedure
in which heat via an electrode or gamma-knife radiosurgery is used to destroy
part of the globus pallidus. The globus pallidus is also an area of the brain
involved in movement. A pallidotomy can reduce tremors, shuffling gait, flat
affect, rigidity, and slowness of movement. These symptoms may be
dramatically reduced following this procedure, and the effects may last for at
least 5 years. Deep brain stimulation (neurostimulation) is the implantation of a
type of “brain pacemaker” that delivers electrical impulses to the subthalamic
nucleus, the internal globus pallidus, or the thalamus to reduce tremors
associated with PD. The potential side effects of this procedure include
depression, slurred speech, tingling in the head and hands, and problems with
balance. The generator usually needs to be replaced every 3 to 5 years.
Continuous device-aided drug delivery involves implanting a subcutaneous or
gastrostomy pump to provide continuous delivery of levodopa (Katzenschlager,
2014).
Other medical management techniques that are being explored are gene
therapy to replace enzymes involved in dopamine synthesis or to enhance the
survival of dopamine neurons (Bohn, 2000), the use of growth factors pumped
directly into dopamine-deficient areas of the brain (Mayor, 2002), and stem cell
or fetal cell transplantation to generate dopamine-producing cells in the
substantia nigra (Bega & Krainc, 2014; Merims & Freedman, 2008; Politis &
Lindvall, 2012). While the stem cell or fetal cell transplants have shown promise
for long-term motor improvements, the use of this treatment has been limited by
problems with tissue availability and standardization of grafts (Bega & Krainc,
2014; Politis & Lindvall, 2012).
Amyotrophic Lateral Sclerosis
ALS, also known as Lou Gehrig disease, is a fatal, progressive, degenerative
motor neuron disease in which scars form on the upper motor neurons in the
corticospinal pathways and the functionally linked lower motor neurons in the
motor nuclei of the brainstem and the anterior horn cells of the spinal cord
(Douaud, Filippini, Knigh, Talvot, & Turner, 2011; Przedborski, Mitsumoto, &
Rowland, 2003; Weydt, Weiss, Moller, & Carter, 2002). Neurodegeneration is
also found in the corpus callosum, extramotor cerebral regions, and the
frontotemporal lobes (Douaud et al., 2011). The actual mechanism of
neurodegeneration remains a mystery, but significant progress has been made
with the identification of several genetic mutations including mutation in the
superoxide dismutase 1 gene seen mostly in the familial type of ALS (Kuehn,
2011). There is speculation that cytosolic and mitochondrial pathway
dysfunction results in the buildup of proteins that lead to motor neuron death
(Shaw, Al-Chalabi, & Neigh, 2001). There is also evidence of an inflammatory
component in the mechanisms of the disease process (Weydt et al., 2002). See
Figure 22.3.
Figure 22.3 Motor neuron changes in amyotrophic lateral sclerosis (ALS).
(From Anatomical Chart Co.)
There are two types of ALS: sporadic and familial. Sporadic ALS does not have
a known genetic link whereas familial ALS does. Sporadic ALS accounts for
90% of cases while the remaining 10% are the familial type (Gupta, Prabhakar,
Sharma, & Anand, 2012).
Etiology
There is no known cause of ALS, but it is speculated that several disorders with
several causes lead to this motor neuron disease (Weydt et al., 2002). The two
types of ALS, familial and sporadic, may have different causes. Only 10% of
cases are familial (Zoccolella, Santamato, & Lamberti, 2009). Researchers have
discovered a genetic mutation as one cause for familial ALS (Kuehn, 2011;
Shaw et al., 2001). There is also evidence of genetic causes using familial
patterns of susceptibility. Family history of a first- or second-degree relative with
ALS is a significant risk factor for the disease.
In most cases, however, ALS occurs sporadically and is presumed to be
acquired. There is no known cause of sporadic ALS, but there is some
speculation regarding viral, retroviral, and environmental causes (Shaw et al.,
2001). There is also some evidence of genetic susceptibility with higher rates of
ALS among relatives of people with other common neurodegenerative disorders
(Kiernan et al., 2011). Other identified risk factors associated with sporadic ALS
include occupational exposure such as chemicals or electromagnetic fields
(Weisskopf et al., 2005), lead exposure (Kamel et al., 2003), military service
(Mehta et al., 2014), cigarette smoking (Gupta et al., 2012; Weisskopf et al.,
2004), alcohol use (Gupta et al., 2012), physical activity, trauma (Mehta et al.,
2014), and dietary consumption with increased fat and glutamate intake
associated with increased risk and fiber intake with reduced risk (Nelson,
Matkin, Longstreth, & McGuire, 2000). Recent research has found that rural
residence alone is not a risk factor for ALS, but participation in agricultural
activities, such as farming, has a significant association with developing the
bulbar form of ALS possibly due to exposure to agricultural chemicals (Furby,
Beauvais, Kolev, Rivain, & Sebille, 2009). Some of the aforementioned risk
factors may also be related to the familial type of ALS as environmental
exposure may be combined with genetic susceptibility (Kamel et al., 2003).
Worldwide, ALS affects white males who are older than 60 years more than any
other group (Mehta et al., 2014). The prevalence rate of people with ALS is
estimated to be 5.4 per 100,000 people in Europe (Chiò et al., 2013) with
worldwide numbers difficult to track (Kiernan et al., 2011). Recent prevalence
numbers for the United States indicate a prevalence of 3.9 cases per 100,000
persons (Mehta et al., 2014). Some estimates state that as many as 30,000
Americans are diagnosed with ALS annually (Weisskopf et al., 2004). Sporadic
ALS affects males more often than females with a ratio of 1.7:1 and an overall
lifetime risk of 1:350 for men and 1:400 for women (Kiernan et al., 2011;
McCombe & Henderson, 2010; Mehta et al., 2014; Shaw et al., 2001), but there
is evidence that this gender difference is decreasing over time (Noonan, Hilsdon,
White, Wong, & Zack, 2002a). The incidence among males and females for
familial ALS is relatively the same (Kiernan et al., 2011). Ongoing investigation
indicates the possibility of increasing prevalence of ALS in the United States
(Noonan et al., 2002a) and worldwide (Przedborski et al., 2003). Reduced
frequency of ALS has been noted among people of Hispanic origins (Kiernan et
al., 2011; Mehta et al., 2014). The age groups with the lowest ALS frequency are
ages 18 to 39 years and ages >80 years (Mehta et al., 2014).
Signs and Symptoms
The most common initial symptom of ALS is weakness of the small muscles of
the hand or an asymmetrical foot drop (Beresford, 1995). Night cramps,
particularly in the calf muscles, also may be present. Progressive loss of muscle
movement, spasticity, difficulty speaking and swallowing, loss of emotional
control, and reduced body temperature regulation are common (Beresford,
1995). Although often overlooked, there is now evidence of significant cognitive
deficits such as reduced executive functions and changes in language abilities or
personality related to frontotemporal involvement (Kiernan et al., 2011). The
progressive neurodegeneration of the CNS eventually leads to respiratory failure.
The signs and symptoms of ALS are progressive, most commonly in a distal
to proximal pattern. The symptoms can be divided into three areas, including
lower motor neuron, corticospinal tract, and corticobulbar tract dysfunction (see
Table 22.4). The lower motor neuron dysfunction symptoms include focal and
multifocal weakness, atrophy, cramps, and muscle twitching. Muscle spasticity,
weakness, and hyperresponsive reflexes are associated with corticospinal tract
dysfunction (Kiernan et al., 2011; Nelson et al., 2000). Slow, labored, and
distorted speech, often with a nasal quality, hyperactive gag reflex, tongue
atrophy, and dysphasia, are seen with bulbar lower motor neuron involvement
(Kiernan et al., 2011; Beresford, 1995).
TABLE 22.4 Signs and Symptoms of Amyotrophic Lateral Sclerosis

The age of onset of ALS occurs between 16 and 77 years, but it is usually
diagnosed when a person is between the ages of 55 and 75 years (Noonan et al.,
2002a). The peak age of onset is 58 to 63 years for sporadic ALS and 47 to 52
years for familial ALS (Kiernan et al., 2011). The course of ALS is usually
progressive and rapid. The duration of survival after diagnosis is usually 1 to 5
years, with a mean survival of 3 years (Beresford, 1995; Weisskopf et al., 2004).
Nearly 50% of people die within 30 months from the onset of symptoms
(Kiernan et al., 2011). Life expectancy is dependent on the type of ALS
(sporadic or familial), rate of disease progression, early presence of respiratory
failure, and nutritional status (Kiernan et al., 2011). The younger a person is and
the more mild the symptoms at the time of diagnosis, the longer the course.
There is some evidence of a “resistance in ALS,” in which a person may
demonstrate improvements and live longer than 10 years. This is seen in
approximately 10% to 16% of people with ALS (Mitsumoto Hanson, & Chad,
1988). Death is usually from respiratory failure, often precipitated by pneumonia
(Kiernan et al., 2011; Weydt et al., 2002).
Diagnosis
It is difficult to diagnose ALS in the early stages because the symptoms are
similar to many other neuromuscular disorders (Gupta et al., 2012). A definitive
diagnosis requires a complete neurological examination with
electrophysiological and radiological testing, which can take several months and
delay much-needed intervention (Gupta et al., 2012). As with the other PNDs,
there is not a specific diagnostic test for ALS and the physician must piece
together clinical symptoms, electromyogram (EMG) results, and tests to exclude
other causes of the clinical presentation (Beresford, 1995; Kiernan et al., 2011).
The EMG findings will include motor denervation and fasciculation (twitching)
with intact sensory responses. Although MRI of the CNS is usually used to rule
out other causes of the symptoms (Kiernan et al., 2011), some recent successful
identification of ALS has been found by combining structural and functional
MRI results to identify cerebral network connectivity failure (Douaud et al.,
2011). Blood tests are usually normal. Cerebrospinal fluid is often normal but
may show raised protein levels.
The medical treatments that are currently available do little to alter the fatal
course of ALS (Weydt et al., 2002). Riluzole is the only medication that has been
approved by the U.S. Food and Drug Administration to treat ALS (Mehta et al.,
2014). There is evidence that riluzole helps people stay in the milder stages for a
longer time and it is the only medication that increases survival, although for
only a modest amount of time (3 to 6 months) (Kiernan et al., 2011; Zoccolella
et al., 2009). Riluzole, however, does not provide significant relief from the
symptoms of ALS (Mehta et al., 2014). The medications prescribed to treat the
symptoms of ALS include antispasmodic medications, nonsteroidal anti-
inflammatory medications, and antibiotics. Several medications to treat ALS are
in the clinical phases of development (Bucchia et al., 2015). The most promising
of these experimental medications is dexpramipexole, which is used to protect
motor neurons and is currently approved for use with people who have PD
(Bucchia et al., 2015). Neural stem cell therapy is also being explored as a
means to replace motor neurons (Bucchia et al., 2015).
Gastrostomy and noninvasive positive-pressure ventilation have been shown
to increase both quality and possibly length of life (Przedborski et al., 2003).
Nebulizers with N-acetylcysteine may be used to thin saliva, and low-dose
radiation and botulinum toxin injections into the salivary glands are sometimes
used to treat drooling (Kiernan et al., 2011).
There is some evidence that attending a clinic staffed by a multidisciplinary
team (including neurologists; specialist nurses; physical, occupational, and
speech therapists; and a pulmonologist, nutritionist, psychologist, and social
worker) is effective for improving quality and length of life (Kiernan et al.,
2011; Traynor, Alexander, Corr, Frost, & Hardiman, 2003). Palliative care may
also be very important for people with ALS and their families. It has been
estimated that 62.4% die in a hospice-supported environment (Przedborski et al.,
2003).
Impact of Conditions on Occupational
Performance
Each of these PNDs is progressive and can affect all occupations, client factors,
performance skills, and performance patterns. The extent of this effect depends
on the stage and severity of the disease. In each case, a person may have any
combination of the deficits listed.
Self-care skills are affected by changes in the person’s sensorimotor skills.
Changes are usually noted in gross and fine motor coordination, postural control,
muscle tone, endurance, and sensation (except in ALS). Loss of independence in
bathing, dressing, personal device care, and toilet hygiene may occur. Toileting
can become problematic for persons with MS or PD because of the loss of
bladder and bowel control. The individual may experience any combination of
the complications noted earlier in this chapter.
Eating may be difficult, either because the person loses the strength and
coordination to self-feed or because of chewing or swallowing difficulties
(dysphagia). The latter is caused by weakness or incoordination of the
pharyngeal musculature, which also can make it difficult for an individual to
ingest oral medications.
Functional mobility is another critical concern. Neuromuscular and motor
problems make ambulation difficult or impossible, either independently or with
assistive devices, even in an electrically propelled wheelchair. Acquiring
alternate methods of mobility requires the ability to adapt. The person must be
able to change motor patterns, requiring concurrent new and varied perceptual
and cognitive strategies. At the same time, the individual is challenged
psychologically to make the necessary adjustments to new and different types of
mobility. As the person’s function decreases, issues of home and work
accessibility must be considered, and the necessary adaptations must be made to
maintain performance in activities of daily living.
Deficits in neuromusculoskeletal and movement-related functions and motor
skills due to PNDs usually lead to reduced ability to perform all instrumental
activities of daily living. In addition to neuromusculoskeletal losses, reduced
sensory, cognitive, and perceptual functions (Kiernan et al., 2011) may also
interfere with independence in this area. In the early stages of these diseases,
adaptations need to be made to afford the person the opportunity to maintain
function for as long as possible. Most of these tasks, however, eventually need to
be delegated to other members of the household, further creating issues of
dependence and loss of role function.
A normal activity for many persons, including those with PND, is the care of
others, including a spouse or significant other, children, or older, dependent
adults. The individual with a PND may have increasing difficulty fulfilling this
role. In fact, he or she may have to rely on these care receivers to provide
support and care, creating a major role reversal. These changes in
responsibilities can be very stressful for all concerned; they challenge everyone’s
ability to maintain the integrity of relationships.
Education
Because of the age of onset for these PNDs, many people will not be involved in
formal educational activities. Those who are may gradually experience a reduced
ability to physically participate due to decline in underlying
neuromusculoskeletal and movement-related functions and motor skills. Deficits
in cognitive and perceptual functions may further limit learning ability.
Adaptations such as computer-based courses for formal and informal education
may be a viable option.
Work
All client factors have the potential to affect work activities. Work is a crucial
area of occupational performance and, for many adults, is an important part of
self-identity. As motor skills decline, the ability to perform specific work tasks
also declines. “Invisible symptoms” such as fatigue, weak or blurred vision, and
difficulties with bladder control often confound the issue. Coworkers may not
understand why someone who does not look ill cannot work. Again, this affects
the person psychologically, with changes in societal roles and self-concept. This
is particularly true for an individual whose job requires a high degree of physical
stamina and skill. For example, assembly workers or truck drivers may lose their
jobs fairly early in the course of these diseases. An individual who has been the
breadwinner of the family and whose identity is closely tied to physical strength
and endurance may have serious adjustment problems. Cognitive deficits also
may make it difficult for the person to function and continue to find satisfaction
in work.
Play and Leisure
Many leisure activities can be affected by the changes that result from PNDs.
Fatigue and reduced exercise capacity are common (Kiernan et al., 2011).
Alternative leisure activities must be explored as more and more performance
deficits occur. A balance between work and play should be maintained as long as
possible. However, if the person can no longer engage in usual work and daily
living activities, it is even more critical to have meaningful and fulfilling leisure
pursuits. These activities will grow in importance as a means of self-
actualization and satisfaction.
Communication, mobility, sexual dysfunction, and eating problems may all
affect the person’s normal socialization with individuals or groups. Dysarthria or
imperfect articulation is caused by a lack of control of the tongue and other oral
muscles essential to speech. This problem can affect the person’s ability to
communicate thoughts, needs, and desires and can limit social interaction
(Kiernan et al., 2011). The individual may lose upper extremity function, making
it difficult to compensate for speaking problems with written communication.
Sexual dysfunction may also be present. Because of depression and
diminished self-concept, the person may no longer feel attractive, which causes
problems in sexual expression. Also, loss of specific motor and sensory function
can affect physical performance.
Because of the unpredictable nature of the course of each PND, potential
dependency issues are ongoing problems. This may lead to secondary
psychosocial issues caused by these lifestyle changes. PNDs require an initial
social-psychological adjustment as well as continual readjustment because of
erratic progression of symptoms. A person who was active and outgoing may
have a diminished self-concept because of the inability to engage in activities
that were once of interest and value. The result is a variety of role changes in the
family or society.
Role expectations, which exist in every social situation, are ways of
behaving or reacting that fit with one’s self-image and the expectations of others.
These include attitudes, activities, and patterns of decision making, expression of
feelings, and meeting the needs of significant others. Some individuals with loss
of bladder control may avoid going out in public. Mothers may be unable to care
for their children. Some may come to see themselves as no longer useful or
attractive to others. Marriages may break up under the strain of living with
PNDs. Occasionally, individuals with PNDs threaten suicide. An individual with
a PND must think seriously about current role expectations and how these might
be threatened by the PND.
CASE-STUDY 1
Jennifer is a 38-year-old woman who was diagnosed with MS 5 years
before this hospital admission. She was admitted because she noticed a
progressive deterioration of function during the past 6 months. The main
problems she identifies are an increase in fatigue, difficulty with bowel and
bladder function, and several falls. She complains of feeling moody and
forgetting information.
She is married and has two children, an 11-year-old girl and a 5-year-
old boy. She is self-employed as a graphic designer, which requires her to
spend many hours typing on the computer and talking via phone and face-
to-face with clients. Her husband works full time as a school counselor and
has been very supportive. At the time of admission, he seemed
overwhelmed.
Jennifer tries to do her morning self-care but is finding it more difficult
and frustrating. Getting dressed is particularly fatiguing, and she admits that
at times, she goes to bed fully dressed to avoid having to get dressed in the
morning. She has been using a manual wheelchair off and on for the past 2
years.
Her daughter is currently helping with the laundry, cooking, and simple
cleaning. Jennifer states that she has problems doing household tasks
because she must hold onto something stable before reaching for, or lifting,
an object. She currently enjoys no leisure activities. At one time, she liked
to knit, but it has become too frustrating to be pleasurable.
Jennifer complains of bladder urgency but often cannot void. She also
has a mild dysarthria, spasticity of the lower extremities, weakness of the
upper extremities (able to move against gravity withstanding minimal
resistance), poor sitting balance, poor fine and gross motor coordination,
blurred vision, and loss of stereognosis and light touch. When the
occupational therapist spoke with her, it became apparent that she did not
comprehend the nature and course of MS. She is feeling frustrated and
depressed about her recent decline of function. Jennifer recently began
receiving interferon - 1b treatment. She is currently taking Tylenol,
Senokot, Metamucil, Colace, heparin, and multivitamins and is using
Dulcolax suppositories.
CASE-STUDY 2
Cal is a 72-year-old man with stage III PD. He has recently experienced a
severe loss of balance and functional mobility. He reports difficulty moving
quickly and gracefully. He also complains of poor handwriting, problems
sleeping, and numbness in both hands.
Cal is a single, retired accountant who lives independently in a two-
story home. His bedroom and bathroom are on the second floor. There are
four steps to enter the home. He has no children. He has one sister who
lives within walking distance of his home. She, however, is suffering from
arthritis and has difficulty offering much assistance.
Cal has been caring for himself thus far, but his sister reports she is
concerned about his safety, especially with activities such as cooking and
bathing. She reports that he has fallen on several occasions recently and
spends most of his time sitting in his chair in his living room. His sister
brings him meals as often as possible, but is unable to bring meals in the
morning or during bad weather. Her children live out of state and are only
able to offer assistance to him during occasional visits when they try to do
some of the major household chores such as painting, repairs, and cleaning.
Cal was once interested in music and art. He played the piano and
painted with watercolors. He reports that he has not participated in these
leisure activities for “a long time.” He also played tennis on a regular basis
at the local club. He is still a member of the club but has not been there in
more than a year.
Cal is reportedly self-conscious of his illness and, therefore, does not
like to go out in public very much. His sister, who has always been very
close to her brother, expresses concern about his “depression and lack of
motivation.” She states that she has tried on several occasions to get him to
go to local musical concerts or museums, but she feels he is just too
depressed. He is currently taking levodopa-carbidopa. His sister, however,
reports that he does not consistently take his medication because it “makes
him feel sick.”
CASE-STUDY 3
Tomas is a 48-year-old man who has recently been diagnosed with ALS.
Six months before being referred to O.T. services, he began to experience
some weakness in his hands and he began dropping objects, such as tools.
He reports loss of strength in his arms and legs. The weakness in his legs
has become so severe that he now uses a borrowed wheelchair part of the
time. He complains of difficulty sleeping due to cramps in his legs. He also
reports that he has lost almost 20 lb in the last 6 or 8 months.
Tomas is married and has two sons, ages 6 and 4. He independently
owns and operates a lawnmower shop at which he sells and repairs
lawnmowers. He reports significant difficulty performing the repair parts of
his job because of weakness in his hands and arms. Most of the repairs have
become backed up in the shop and he is considering hiring assistance or
sending the work to another shop. His wife is employed full-time as a legal
secretary. She has helped with the business as much as possible by
completing some of the bookkeeping after hours. She is very busy caring
for their boys and trying to maintain the household. She appears to be very
supportive of Tomas, but also seems very burdened by her responsibilities.
Tomas has many interests in sports and outdoor activities. He has been
racing in “Iron Man” triathlons and marathons for the last several years. He
was a high school track and cross-country star. He enjoys biking and
camping. Every summer, he and his family take a 2-week trip to a remote
location where they hike and camp. He is also supportive of his sons’ sports
events and enjoys teaching them various sports. Last year, he was a soccer
coach for his oldest boy’s team.
The course and prognosis of ALS have been explained to Tomas and
his wife, and they have reportedly been discussing future plans. They are,
however, “hoping for a miracle.” At this time, he is taking riluzole and pain
relievers to reduce the pain from the cramping in his legs.
RESOURCES
Multiple Sclerosis
National Multiple Sclerosis Society http://www.nmss.org/
The Multiple Sclerosis Foundation http://www.msfacts.org/
The Multiple Sclerosis Association of America http://www.msaa.com/
Parkinson Disease
National Parkinson Foundation
http://parkinson.org/
Parkinson’s Disease Foundation, Inc.
http://pdf.org/
The American Parkinson Disease Association, Inc.
http://www.apdaparkinson.com/
Amyotrophic Lateral Sclerosis
The ALS Association
http://www.alsa.org/
World Federation of Neurology Amyotrophic Lateral Sclerosis
http://www.wfnals.org/
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C H A P T E R
23 Rheumatic Diseases
Timothy M. Mullen
KEY TERMS
Boutonniere deformity Chondropenia Crepitus Disease-modifying antirheumatic
drug (DMARD) Energy conservation Fibromyalgia Gout
Guarding Hyperuricemia Joint protection Juvenile rheumatoid arthritis
Osteoarthritis Pannus
Rheumatoid arthritis Systemic lupus erythematosus Swan-neck deformity
Grace Kennedy is a very pleasant 68-year-old retired woman living in a rural
community. She is recently widowed and is finding more difficulty maintaining
her independence. Grace belongs to a very supportive parish whose members
have supported many of her instrumental activities of daily living (IADLs),
including shopping, meal preparation, housework, laundry, and paying bills. Her
adult children live several hours away and spend as many weekends with her as
possible to support her bathing and other hygiene activities of daily living. Grace
recounts gradually losing the ability to grasp and pinch small objects and
progressing to larger objects. Opening jars, functioning zippers, and fastening
buttons eventually became impossible. From there, gross motor challenges
become apparent. Transitions in and out of bed, from a chair and off, and on the
toilet were slow and full of effort. She began to feel helpless and thought she
would lose her independence.
Seeing the apparent decline of independence, Grace’s children intervened
and arranged for an in-home occupational therapy assessment. The occupational
therapist (OT) spent considerable time evaluating Grace’s needs and focusing on
her preferences. The OT also had Grace demonstrate all IADLs and activities of
daily living (ADLs) required for her to live independently. Subsequently, the OT
had a meeting with Grace and her family and made several recommendations to
optimize her ability to stay at home. The meeting focused on what could be
adapted to maintain independence and what could be outsourced. Grace had no
strong desire to maintain some basic IADLs; therefore, the family agreed to look
into a service to provide housekeeping, meal preparation, and grocery shopping.
The OT then demonstrated several assistive devices that could aid in writing,
pulling zippers, gripping, and opening objects. The concept was to make items
larger and lighter so manipulations were easier and more sustainable. The OT
further recommended devices and grab bars to facilitate transitions.
Descriptions and Definitions
Rheumatic disease is an umbrella category of over 100 different individual
diagnoses identified by the pathology of normal muscle, joint, nerve, and
connective tissue mechanics. The root definition rheuma in Latin means
discharge of mucous membranes because in early days, it was thought to be the
origin of all aches and pains associated with muscles and joints. The science of
rheumatology has greatly advanced since the early days of medicine (American
Heritage Dictionary). It is now well known that rheumatic diseases range from
autoimmune responses to wear-and- tear–type pathologies. The more common
diagnoses include rheumatoid arthritis (RA) (Garrod 1890, 2001), osteoarthritis
(OA), fibromyalgia, gout, and systemic lupus erythematosus.
Rheumatoid Arthritis
In the year 1800, a 28-year-old resident physician from France, Augustin Jacob
Landré-Beauvais, described in his dissertation what modern medicine now calls
rheumatoid arthritis (Landré-Beauvais, 2001). He began to differentiate the
symptoms and characteristics from gout. It was not until 1859 that an English
physician named Alfred Garrod identified the presence of excessive uric acid in
the blood of patients with gout but the absence of this characteristic in those with
other forms of arthritis. In 1890, Archibald Garrod, son of Alfred, first coined
the diagnosis “rheumatoid arthritis” in his book Treatise on Rheumatism and
Rheumatoid Arthritis.
Based on modern definitions, scientists studying paleopathology now study
eight specific characteristics of skeletons related to RA. These characteristics
include subchondral cysts; erosions/periarticular sinuses in affected joints;
rebuilding and/or presence of osteophytes; severe periarticular bone
fragmentation or sinuses; ulnar deviation of the metacarpophalangeal (MCP)
joints; traces in cartilage-supporting bone tissues of a multiarticular joint, as
evidence of the disease’s effect on all the articular facets of the joint
(distinguishing it from OA); osseous ankylosis of joints (especially carpal and
metacarpal); eburnation; and multiple joints that are bilaterally affected
(Entezami, Fox, Clapham, & Chung, 2011). Common locations and symptoms of
RA are depicted in Figure 23.1.
Figure 23.1 Common locations and symptoms of RA. (From Anatomical Chart
Company.)
Societal costs associated with RA are significant. It has been determined that
annual costs are $39.2 billion. This can be broken down into direct, indirect, and
intangible costs. The direct costs total $19.3 billion, with the burden shared
across employers (33%), patients (28%), the government (20%), and caregivers
(19%). Intangible costs consist of premature mortality ($9.6 billion) and quality
of life deterioration ($10.3 billion) (Birnbaum et al., 2010).
Osteoarthritis
Degenerative joint disease, more commonly called osteoarthritis, is, as the
name indicates, inflammation of the bone and joints (The American Heritage
Science Dictionary, 2015). Hippocrates described general incidence of joint pain
but not in detail. It was not until British physician William Musgrave first
detailed OA and its effects in 1715 in his published work De arthritide
symptomatica (Cameron, 1998). Since, OA has been subdivided into primary
and secondary forms. Primary OA consists of the typical wear and tear
associated with eroding the joint cartilage over time. Secondary OA involved an
injury or insult to the joint, such as a fracture, that leads to premature erosion of
the joint cartilage. Common locations and symptoms of OA are depicted in
Figure 23.2.
Figure 23.2 Common locations and symptoms of OA. (From Anatomical Chart
Company.)
Societal costs can be broken down into direct and indirect costs. It is estimated
that combined, these costs average over $5700 per person annually (Maetzel, Li,
Pencharz, Tomlinson, & Bombardier, 2004). Thirteen point two billion dollars
alone can be assigned to costs associated with employment (Buckwalter,
Saltzman, & Brown, 2004). Absenteeism costs society approximately $10.3
billion (Kotlarz, Gunnarsson, Fang, & Rizzo, 2010). Murphy and Helmick
estimate that hospitalization costs for total knee and total hip arthroplasties
combined cost $42.2 billion in 2009 (Murphy & Helmick, 2012).
Fibromyalgia
Scottish surgeon, William Balfour, first described the symptoms of fibromyalgia
in 1816. In 1904, the work by a pathologist Ralph Stockman presented
inflammatory changes in the intramuscular septa on patient biopsies, and
separately, Sir William Gowers coined the term fibrositis to describe tender
points in patients with low back pain. The modern definition evolved in 1972
when physician Hugh Smythe detailed fibromyalgia as widespread pain and
tender points (Inanici & Yunus, 2004). Common locations of tenderness with
fibromyalgia are depicted in Figure 23.3.
Figure 23.3 Common locations of tenderness with fibromyalgia.
Silverman et al. studied the economic burden associated with fibromyalgia and
often found it accompanied RA. They quantified the direct costs of fibromyalgia
as nearly $11,000 annually per patient but nearly doubled at $19,395 when both
diagnoses were present. Indirect costs, such as work absence, are significant for
this disease (Silverman et al., 2009).
Gout
In 2640 BC, the Egyptians were the first to describe podagra, what modern
medicine calls gout. Later, Hippocrates wrote about the disease as the
“unwalkable disease” because of its primary impact on the first
metatarsophalangeal joint. Throughout history, it was associated with an
excessive lifestyle of rich foods and alcohol consumption and often referred to as
the “disease of kings.” In medieval times, it was believed that this excess would
cause a “drop” or flow into the joint; therefore, the Latin derivative of drop or
gutta was applied and the origination of gout (Nuki & Simkin, 2006). Common
locations of gout include the first metatarsal phalangeal joint, the interphalangeal
joints of the hands and the elbows.
It has been estimated that annual costs directly attributable to gout alone
exceed $4 billion. In addition, indirect costs have an added societal burden
related to lost wages and productivity due to absenteeism totaling $2.6 billion.
While not quantified, the indirect costs to caregivers and other indirect costs are
also substantial (Wertheimer, Morlock, & Becker, 2013).
Systemic Lupus Erythematosus
Systemic lupus erythematosus was first described by Hippocrates through his
work on cutaneous ulcers. The word lupus in Latin means wolf and was applied
to these patients as a descriptor by Rogerious in the thirteenth century to
describe his patients whose facial lesions bore a strong resemblance to a wolf’s
bite (Boltzer, 1983). It wasn’t until 1833 when Biett began to clarify the
categories and descriptions of lupus erythematosus. In 1872, Kaposi’s work
clarified the variations into discoid and systemic forms leading to the modern
definition of systemic lupus erythematosus (Smith & Cyr, 1988). Systemic lupus
erythematosus tends to impact similar joints associated with RA.
Systemic lupus erythematosus also is a significant burden on society. Direct
and indirect costs per patient range from $4,453 to $52,415 (Meacock, Dale, &
Harrison, 2013).
Etiology
Healthy articular joint anatomy and function is the foundation for understanding
pathology associated with rheumatic diseases. The healthy joint has a smooth
well-lubricated articular surface whether it is a hinge joint, pivot joint, or ball
and socket–type joint. Figure 23.4 identifies the standard components of most
joints. Typically, the soft tissue includes muscles, tendons, joint capsule, and
ligaments, which provide the mobility and stability of the joints. The
cartilaginous joint surface and synovial fluid provide the necessary lubrication
and durability for high-repetition load bearing. The combined structure is
smooth, stable, and efficient.
Figure 23.4 Normal, healthy joint.
It is apparent with RA that a combination of genetic and environmental factors

leads to autoimmune reactions within the body (Gibofsky, 2012). If left
untreated, these reactions result in the formation of synovitis and tenosynovitis,
which often lead to joint damage and ligament laxity, which can subsequently
create muscle imbalance (Horsten & Ursum, 2010). The infiltration of infectious
agents into the synovial tissues triggers a chronic inflammatory process, and
ensuing immune reaction leads to cell degradation of cartilage and bone tissue
(Baecklund, Askling, Rosenquist, Ekbom, & Klareskog, 2004). Depicted in
Figure 23.5 is the typical appearance of a hand with RA. The swelling around
the joints is often quite visible. Pannus is formed from the synovial lining,
which is a substance that causes the soft tissue destruction. Subsequently, the
cartilage and bone erode creating a narrowed joint space and joint instability.
Figures 23.6 and 23.7 describe and depict the cellular response to the disease
state.
Figure 23.5 Typical impact of rheumatoid arthritis in the hand. (From
Anatomical Chart Company.)
Figure 23.6 Synovial lining cells multiply, creating a mass called pannus.
Substances in this mass further damage the underlying cartilage, which softens,
weakens, and ultimately is destroyed. The waste products of cartilage cell
destruction further stimulate the inflammatory process. New phagocytes rush to
the area to clean up the debris. Some lymphocytes and other mononuclear cells
are mistakenly rendered capable of attacking cartilage. Lysosomal enzymes and
collagenase are released, thus perpetuating the abnormal process. (Reprinted
with permission from the Arthritis Foundation. (1988). The AHPA Arthritis
Teaching Slide Collection (2nd ed.).)
Figure 23.7 In the development of inflammatory rheumatic diseases, the normal
protective process of inflammation goes awry. Lymphocytes can no longer
distinguish between antigens and healthy tissue, and they secrete substances that
cause the synovial lining to become inflamed. Phagocytes become overloaded
with immune complexes and release lysosomal enzymes into the joint fluid. The
enzymes then attach to the cells of the joint lining, eventually destroying them.
(Reprinted with permission from the Arthritis Foundation. (1988). The AHPA
Arthritis Teaching Slide Collection (2nd ed.).)
OA, often synonymous with degenerative joint disease, has polygenic hereditary
component as well as an environmental component. New research is linking
genes to the development of synovial joints and may be able to predict onset and
specific joint involvement (Sandell, 2012). Aging and other environmental
factors can also lead to OA following period of chondropenia or a loss of
cartilage faster than the rate of self-repair (Lohmander, Saxne, & Heinegard,
1994). Acute injury, mechanical stress, and even immobilization can lead to
elevated levels of degradative enzymes in synovial fluid that remain present for
years following the insult (Dahlberg, Friden, Roos, Lark, & Lohmander, 1994;
Ghosh, Sutherland, Taylor, Pettit, & Bellenger, 1983). The extent determines
whether the process becomes progressive or not (Dahlberg et al., 1994). Figure
23.8 depicts chondropenia at the cellular level leading to OA. Figure 23.9 is a
depiction of cartilage degradation typical of knee OA.
Figure 23.8 Chondropenia at a cellular level leading to osteoarthritis. (From
Moskowitz, R. W., Altman, R. D., Buckwalter, J. A., Goldberg, V. M., &
Hochberg, M. C. (2006). Osteoarthritis (4th ed.). Philadelphia, PA: Wolters
Kluwer.)
Figure 23.9 Depiction of cartilage degradation typical of knee osteoarthritis.
(From Anatomical Chart Company.)
Disabling pain and tenderness that impact muscles, tendons, and joints are
commonly associated with fibromyalgia. While the incidence is rapidly
increasing, the etiology remains unclear (Jahan, Nanji, Qidwai, & Qasim, 2012).
It is theorized that genetic, immunological, and hormonal factors may play an
important role, but it is still uncertain (Bellato et al., 2012). Unlike typical
rheumatic diseases, fibromyalgia is not characterized by joint inflammation.
Gout is a common inflammatory arthritis caused by crystallization of uric
acid within the affected joints. Long associated with diet high in purines, it can
give rise to elevations in blood uric acid. When synthesis and excretion cannot
balance with dietary intake, hyperuricemia results. Hyperuricemia can also be
attributed to insulin resistance syndrome, hypertension, nephropathy, and similar
conditions. Chronic elevation of urate levels can lead to permeation and
crystallization within the joint (Choi, Mount, & Reginato, 2005). In Figure
23.10, the urate crystals are depicted within a knee joint and the erosive impact it
can have on cartilage.
Figure 23.10 Erosive impact of urate crystals on the cartilage of a knee joint.
(From Anatomical Chart Company.)
Systemic lupus erythematosus remains an autoimmune disorder without certain

etiology. Many studies have been performed to identify the potential trigger of
the autoimmune response including allergies, infections, and exposure to certain
agents, but nothing is conclusive. The impact on joints is very similar to that of
RA (Harvey, Shulman, Tumulty, Conley, & Schoenrich, 1954).
Rheumatic diseases lead to significant disability worldwide; arthritis is the
leading cause of disability in the United States (Bolen et al., 2010). RA has been
reported to affect approximately 1% of the world population (Gibofsky, 2012).
While the prevalence of RA increases with age, it can occur at any age. Most
commonly, it will be diagnosed between the ages of 40 and 70 years (Helmick,
et al., 2008). Studies show RA equally impacts all races, ethnic groups, and
gender (Escalante & del Ricon, 2001). In order to be considered juvenile
rheumatoid arthritis, the onset must occur prior to the age of 16, with
involvement of one or more joints for at least 6 weeks (Gewanter, Rohnmann, &
Baum, 2005). Typical onset occurs between ages 1 and 6, with no gender bias.
Children may have the diagnoses with or without the presence of rheumatoid
factor in their blood. Those with a presence have a higher likelihood of greater
disease severity (Colbert, 2010).
While RA has a significant impact on the world, OA is far more prevalent.
Studies indicate that 10% to 15% of the world population has the disease.
Approximately 27 million people in the United States alone are impacted with
OA, with expected exponential growth due to our aging population and the
increased incidence of obesity (Neogi & Zhang, 2013). Studies show the impact
varies by gender and race depending on the involved joint. The knee remains the
most common site for OA, and it is estimated to impact 47% of women and 40%
of men during their lifetime. Similarly, for hand OA, it is estimated that 9.7% of
women and 4% of men will be affected. A recent study identified that African
American men had a 32.3% incidence of hip OA while 23.8% of Caucasian men
had hip OA. Chinese women were more likely to develop knee OA over
Caucasian women (46.6% compared to 34.8%), while Caucasians had a higher
incidence of hand and hip OA (47% compared to 85% for hip OA and 0.8%
compared to 4.5% for hand OA) (Neogi & Zhang, 2013).
Fibromyalgia can often accompany OA and RA, and in fact, it is one of the
more common diagnoses referred to a rheumatologist. Studies indicate
prevalence worldwide ranges from 1% to 4.7%. Fibromyalgia is more common
in women than men, and the incidence increases with age (Jones, Atzeni,
Beasley, Flüß, Sarzi-Puttini, & Macfarlane, 2015). It has been reported that the
prevalence in the United States is 0.5% in men and 3.4% in women (Vincent et
al., 2013).
The most common form of inflammatory arthritis is gout. Gout has been
reported to affect 1% to 4% of the world population (Helmick et al., 2008;
Lawrence et al., 2008). In the United States, it has been reported that 8.3 million
people are affected by gout. Men and African Americans are significantly more
likely to be affected (Singh, 2013).
Several studies report the proportion of the population with systemic lupus
erythematosus is increasing while the frequency of new cases is decreasing.
Internationally, Northern Ireland has the highest prevalence of systemic lupus
erythematosus while people of Black Caribbean descent have the highest
incidence and prevalence. Females are six times more likely to be affected by
systemic lupus erythematosus than are males (Rees et al., 2014).
Signs and Symptoms
RA is quite variable in its onset and progression by individual. While some
experience sudden onset of symptoms, some experience a gradual onset, some
experience exacerbations and remissions, some experience mild symptoms, and
some experience severe symptoms. RA can affect one joint or have polyarticular
involvement. It can affect joints symmetrically or asymmetrically. Most
frequently, RA is polyarticular with the initial signs being that of fatigue and
generalized weakness (Kasper et al., 2012).
The inflammatory process is the primary source of the signs and symptoms.
Table 23.1 depicts the most common presentation for each stage of the
inflammatory joint disease process ranging from acute to chronic-inactive.
Following fatigue and generalized weakness is often the loss of range of motion.
Joint edema may impede normal joint articulation in early stages. Joint guarding
or self-bracing is also a frequent response in those with RA and Juvenile
Rheumatoid Arthritis (JRA) due to pain associated with movement. Limiting
range of motion may in turn lead to adhesions, which mechanically limit joint
movement (Gewanter et al., 2005). As the disease progresses, contractures may
develop, joint erosions progress, and subluxation, ankylosis, or dislocation may
ensue. Typically, RA affects the smaller joints with higher synovium to cartilage
ratio (Matschke, Murphy, Lemmy, Maddison, & Thom, 2009). The MCP and
proximal interphalangeal joints are most severely impacted by RA (Fouque-
Aubert, Chapurlat, Miossec, & Delmas, 2010). A swan-neck deformity results
when the volar plate of the Proximal interphalangeal joint (PIP) joint elongates
and allows the extensors to force the PIP joint into hyperextension, which causes
an imbalance, and the Distal interphalangeal joint (DIP) joint subsequently
moves into flexion. A boutonniere deformity results when the central slip of the
extensor tendon erodes from its attachment on the middle phalanx. The inability
to extend the PIP joint allows the lateral bands to migrate, creating PIP flexion
and DIP hyperextension. The anatomy of this injury has the appearance of a
buttonhole, which gives rise to its name. When either of these deformities
remains uncorrected for a long period of time, it becomes contracted or fixed.
Figure 23.11 depicts a typical presentation of a rheumatoid hand with an index
finger boutonniere deformity as well as middle and ring finger swan-neck
deformity. Figure 23.12 is a diagram of each of the deformities that frequently
develop.
TABLE 23.1 Signs and Symptoms by Stage of the Inflammatory Disease
Process
Figure 23.11 Rheumatoid hand with an index finger boutonniere deformity and
middle and ring finger swan-neck deformities. (From St. Clair, E. W., Pisetsky,
D. S., & Haynes, B. F. (2004). Rheumatoid arthritis. Philadelphia, PA: Wolters
Kluwer.)
Figure 23.12 Diagram of a swan-neck and boutonniere deformity. (From St.

Clair, E. W., Pisetsky, D. S., Haynes, B. F. (2004). Rheumatoid arthritis.
Philadelphia, PA: Wolters Kluwer.)
While less common, the small joints of the feet are impacted in a similar fashion.
Figure 23.13 depicts a common presentation of the toes of an individual with
RA.
Figure 23.13 Typical presentation of feet in an individual with RA. (From St.
Clair, E. W., Pisetsky, D. S., Haynes, B. F. (2004). Rheumatoid arthritis.
Philadelphia, PA: Wolters Kluwer.)
In the progression of the disease, the soft tissue allows for deformity of small
joints as previously discussed, but the erosive properties continue on the bony
structure until they become dislocated and perhaps ankylosed.
Figure 23.14 shows a picture of the typical zigzag deformity found in the
hands of a person with severe RA. The prolonged inflammation surrounding the
joint, tendons, and ligaments degrades the structures to the point of laxity,
imbalance, and erosion. The typical pattern of imbalance allows significant
radial deviation of the radiocarpal joint as well as ulnar deviation of the MCP
joints of the index through small fingers. This disease is truly erosive on bony
structures.
Figure 23.14 Zigzag deformity: radial deviation of the wrist and ulnar deviation
of the MCP joints. (From Thorne, C. H., Gurtner, G. C., Chung, K., Gosain, A.,
Mehrara, B., Rubin, P., & Spear, S. L. (2013). Grabb and Smith’s plastic surgery
(7th ed.).)
While it is less common in other areas of the body, it is still quite significant.
The presentation often begins with pain and tenderness and localized
inflammation. Prolonged presence can lead to tendonitis, tenosynovitis, joint
capsule inflammation, and joint degradation. Following pain and tenderness, it is
common to observe reduced range of motion. The elbow and forearm can be
susceptible to limitations in flexion, extension, pronation, and supination. It can
also impact the shoulder with the intricate ball and socket joint structure, which
allows the performance of complex ADLs. If the cervical spine is involved,
specifically C1 and C2, the presentation includes headaches with pain and loss
of motion and may produce life-threatening conditions (Niere & Jerak, 2004).
Figures 23.15 and 23.16 characterize cervical degeneration for RA.
Figure 23.15 Neck abnormalities in RA.
Figure 23.16 Cervical subluxations.
If the lower cervical spine is involved, resulting in nerve root compression, the
signs may include numbness in the extremities and muscle weakness with
varying severity.
As RA progresses untreated, individuals may develop an abnormal gait
pattern resulting from hip involvement (Williams, Brand, Hill, Hunt, & Moran,
2010) (Fig. 23.17). As hip involvement advances, typical ADLs begin to become
more difficult including climbing stairs, sitting, donning and doffing garments,
and positioning during sexual activity (Josefsson & Gard, 2010). RA
manifestation in the knee is very similar to that in the hip including pain and
localized edema, often presenting as a limp and progressing to loss of motion
and difficulty with ADLs.
Figure 23.17 Erosive nature of RA on a hip joint. (From Anatomical Chart
Company.)
Often confused with RA because of similar presentation, OA primarily impacts

the knees, hips, hands, and spine. Typically, pain will first appear localized to a
particular joint. Visible and palpable edema will appear next, often accompanied
with point tenderness about the joint involved. Individuals with OA will
typically describe significant joint stiffness following any prolonged period of
inactivity including sitting or lying down to sleep. It is also common for
individuals to complain of postactivity flare-ups of edema and pain. Upon
examination, more advanced cases of OA will present with crepitus during
range of motion, which is the crunching feeling or sound coming from the
articular surface (Hochberg et al., 1995). As with other rheumatic disease,
individuals with OA often guard motion and avoid use of the painful joint
leading to a more rapid development of muscle weakness and increase feeling of
fatigue.
Fibromyalgia can exist in conjunction with other rheumatic disease or
independent of them. It is a very complicated condition that is characterized by
chronic widespread pain, point tenderness, headaches, fatigue, poor sleep, lower
abdomen cramps, and depression. Severity and presence of other comorbid
factors are significant to the presentation of fibromyalgia (Vincent et al., 2013).
Presentation can occur unilaterally or bilaterally and most commonly affect the
neck, buttocks, shoulders, arms, the upper back, and the chest. Symptoms can be
chronic or episodic typically tied to some type of physical or psychological
stressor (Jahan et al., 2012).
Gout presents clinically very similar to RA with its typical joint destructive
properties beginning with inflammation. The symptoms of gout are usually more
intermittent than progressive but, if left untreated, can be quite destructive.
Individuals typically complain of joint-specific pain first that is accompanied by
edema, erythema, and increased localized temperature. This will subsequently
lead to stiffness. Most commonly, the big toe will be affected bilaterally or
unilaterally. Gout may manifest in other parts of the feet and ankles, knees, wrist,
hands, and elbows (Hall, Barry, Dawber, & McNamara, 1967). The images in
Figure 23.18 (left and right) show the characteristic localized edema and
erythema associated with gout of the big toe and a digit, respectively. The onset
is often quite rapid.
Figure 23.18 Localized (A) edema and (B) erythema associated with gout.
(Courtesy of Tim Mullen.)
One common symptom of all chronic rheumatic diseases is depression. Research

suggests that there is a direct correlation with severity of pain and fatigue and the
tendency to experience depression. Furthermore, as participation in ADLs
diminishes in line with disease progression, the more likely an individual is to
experience depression (Wolfe & Michaud, 2009).
The physical manifestations and individual responses to RA present across a
wide spectrum. Surveys suggest that the onset is typically insidious and function
declines with severity (Adams, Burridge, Mullee, Hammond, & Cooper, 2004).
While 20% of adults diagnosed with RA may go into remission in the first year,
the majority will develop a chronic progressive disease (Goronzy et al., 2004). In
contrast, people diagnosed with JRA have a 70% to 90% likelihood of making a
satisfactory recovery with only a small chance of recurrence as an adult
(Gewanter et al., 2005). While remission is possible, research has identified the
following prognostic indicators: (a) number and length of remission, (b) levels of
rheumatoid factor, (c) presence of subcutaneous nodules, (d) extent of bone
erosion seen radiographically at the initial evaluation, and (e) sustained disease
activity for more than 1 year. Additionally, women diagnosed prior to age 50 had
a worst prognosis for developing a severe and chronic form of RA (Iikuni et al.,
2009). Table 23.2 identifies the criteria necessary to be considered in remission
from RA.
TABLE 23.2 Criteria for Remission in Rheumatoid Arthritis
These criteria are intended to describe either spontaneous remission or drug-induced disease suppression,
which stimulates spontaneous remission. To be considered for this designation, a patient must have met the
American Rheumatism Association criteria for definite or classic rheumatoid arthritis at some time in the
past. No alternative explanation may be involved to account for the failure to meet a particular requirement.
For instance, in the presence of knee pain that might be related to degenerative arthritis, a point for “no joint
pain” may not be awarded.
The course and progression of OA widely vary based on whether the onset was
insidious or traumatic. The degenerative process has previously been described
and is progressive in nature. Research suggests the rate of progression may be
slower when the baseline is more severe than when the disease is mild.
Progression has also found to be faster in joints more distal when compared to
proximal joints (Hochberg, 1996). Comorbidities also play a significant role in
disease progression. The greater the body mass index, the more rapid the
progression (Neogi & Zhang, 2013). Presence of Heberden’s nodes correlated
with progression of cartilage loss when monitoring x-ray images longitudinally.
When osteophytes and sclerosis are present on baseline radiographs compared to
radiographs when only osteophytes were present, there was a greater progression
of joint degeneration including narrowing of the joint space, increased angular
deformity, as well as worsening symptoms. Interestingly, there is not always a
significant correlation between progressing radiographs and worsening
symptoms (Hochberg, 1996). This all leads to a widely varying presentation of
OA. With modern treatments, symptoms can be reduced and progression can be
impeded. Joint replacement surgeries can eliminate localized OA and
dramatically improve the prognosis.
Contrary to the progressive nature of other rheumatic disease, fibromyalgia
is not progressive and does not impact morbidity rates. The onset varies widely
based on comorbidities. Fibromyalgia can begin as a widespread effect or
manifest unilaterally and progress bilaterally. Multiple etiological factors make it
difficult to identify and treat. Current research is focusing on psychosocial
management and biochemical, metabolic, and immunoregulatory abnormalities.
Until more is understood about the disease itself, the prognosis is difficult to
determine (Jahan et al., 2012).
Gout has episodic and chronic components. Typical acute attacks have been
previously prescribed and, if left untreated, can be very progressive and
destructive to joints. If diagnosed and treated early, long-term effects can be
eliminated. The American College of Rheumatology (ACR) recently put out
guidelines suggesting dietary recommendations as well as pharmaceutical
recommendations. They also stressed the importance of identifying all related
comorbidities to determine potential secondary causes for the hyperuricemia.
Their dietary recommendations included lists of foods to avoid, limit, and
encourage. Because of the episodic nature of gout, it is suggested that these
dietary standards be maintained as a means of preventing or limiting the
potential return of symptoms (Khanna et al., 2012).
As any of the rheumatic diseases progress, function declines in work, play,
and leisure. The lack of ADL independence will cause a faster decline. There is
no known cure for these diseases; however, early diagnosis and subsequent
treatment offer the best outcomes. Modern treatments can delay or prevent the
destructive nature of these diseases; provide functional, emotional, and social
supports to facilitate independence; and enhance quality of life.
Diagnosis
A comprehensive history of symptoms and detailed timeline of the disorder
provide necessary details for identification and diagnosis. Unfortunately,
individuals will self-manage for 2 to 4 years prior to seeking medical
intervention (Kumar et al., 2007). As previously indicated, differentiation
between RA and other rheumatic diseases can pose significant difficulty. In order
to support differential diagnosis, criteria were created to aid physicians
(Symmons, 2007). See Table 23.3.
TABLE 23.3 Diagnostic Criteria for Rheumatoid Arthritis
As indicated in the table, a detailed physical examination, blood analysis,

radiographic analysis, and tissue/fluid biopsy will be required to aid in the
diagnosis of RA.
OA has degenerative and acute onsets. If an insult to the joint occurs, an
individual is likely aware. The initial injury typically heals and normal activity
resumes, yet persistent pain can linger. Radiographic evaluation will aid in the
diagnosis. If the onset is insidious from overuse or biological factors, diagnosis
may be more difficult without the use of radiographic imaging. Detailed history
and physical examination are also important. Research has shown that habitual
vigorous exercise increases the incidence of OA. Certain sports and occupations
have been linked to soft tissue damage within a joint leading to OA. The best
indicator for knee OA is malalignment as seen in radiographs. If an individual
has a leg length discrepancy of at least 2 cm, they have a higher likelihood of
incidence of OA. Finally, if the shape of the bone or joint departs from normal
anatomy, biomechanical loads can be imbalanced leading to increased joint wear
leading to OA (Neogi & Zhang, 2013).
Diagnosing fibromyalgia is inherently more challenging because there are no
laboratory or radiographic analyses that can make a confirmation. History and
physical examination are essential (Bellato et al., 2012). In an effort to aid
diagnosis of fibromyalgia, the following criteria were created. In order to be
considered fibromyalgia, an individual must have a Widespread Pain Index
(WPI) score greater or equal to seven and a symptom severity scale score greater
or equal to five or a WPI score between three and six and a symptom severity
scale score greater or equal to 9. In addition, these symptoms must be present at
these levels for a minimum of 3 months. Finally, the individual must not have
any other disorder that could be causing the symptoms primarily or secondarily.
The WPI focuses on frequency and location of pain and tenderness and the
symptom severity scale focuses on level of fatigue and cognitive involvement in
the symptoms (Wolfe et al., 2010).
Gout can be diagnosed through history and physical examination and
confirmed with blood work and laboratory analysis. If the disease has progressed
or has chronic episodes of flare-ups, it may be important to have radiographic
evaluation to determine if any joint erosion has occurred. Advanced imaging
such as CT scans may be more sensitive for detecting early changes when
compared to standard radiography. It is important to be aware of the frequency
of symptoms as well as the number of joints involved (Khanna, 2012).
Significant advances in pharmaceutical agents have enhanced the medical
management of RA exponentially. Physicians typically focus on managing six
factors including relief of pain and joint stiffness; reduction of edema;
preservation of normal joint function, musculature, and soft tissue support
structures; minimization of the unintended consequences of the medication;
promotion of normal growth and development; and maintenance of ADL
independence. If early diagnosis can occur and proper interventions can be
initiated within the first 2 years, disease progress may be halted and in some
cases reversed (Finckh et al., 2009).
The ACR collaboratively with the European League Against Rheumatism
developed medication criteria. Research shows synthetic disease-modifying
antirheumatic drugs (DMARDs) should be the first medication introduced as it
has the best chance to achieve low disease activity or remission. Individuals
should be regularly monitored for treatment modification for maximum efficacy.
Methotrexate is the primary synthetic DMARD that has proven to be highly
effective. If methotrexate is not tolerated or contraindications exist, injectable
gold, sulfasalazine and leflunomide can also be considered. Additionally,
DMARDs can be used alone or as a combination therapy for possible added
benefit if the course of the disease does not seem to be altering. Combination
therapy can also include glucocorticosteroids (GCs). GCs can supplement the
DMARDs and also have excellent anti-inflammatory properties. If treatment
targets are not being met or if there are poor prognostic indicators present, a
biological DMARD can be added to the treatment. While synthetic DMARDs
target the T cells to control the inflammatory process, biologic DMARDs focus
directly on the intracellular inflammatory process. Often, biological medications
include tumor necrosis factor (TNF) inhibitor as some individuals have better
responses with and some have better responses without these inhibitors.
Research is lacking on how to taper use of these costly medications if remission
is achieved (Smolen et al., 2010).
Similarly, the treatment of OA is focused on inflammation reduction.
Nonsteroidal anti-inflammatory drugs (NSAIDs) are widely used because they
are effective at managing both inflammation and pain. Most NSAIDs are
nonselective and act by inhibiting cyclooxygenase (COX) enzymes. Newer
medications have become selective due to gastrointestinal (GI) complications
found in early medications. These are known as coxibs and target just the COX-
2 enzymes. Further advancement discovered that protecting prostaglandins in the
GI tract was key to tolerating these medications for acute and chronic symptoms.
These medications are COX-inhibiting nitric oxide donators (CINODs) and have
proven to be very effective for pain modulation and inflammation reduction
while maintaining GI safety (Schnitzer, Kivitz, Lipetz, Sanders, & Hee, 2005).
For the treatment of fibromyalgia, NSAIDs and opioids are typically
initiated to manage the pain and improve physical function prior to diagnosis
confirmation. These medications have limited benefit. Research suggests the
most effective treatment includes antidepressants and neuromodulating
antiepileptic medications. Pregabalin, duloxetine, and milnacipran are the only
medications approved for the treatment of fibromyalgia (Bellato et al., 2012).
Research is now focusing on the stress adaptation response along the
hypothalamic-pituitary axis and aberrant pain processing (Jahan et al., 2012).
NSAIDs are often used as a frontline treatment for gout, but the development
of xanthine oxidase inhibitors (XOI) to treat hyperuricemia revolutionized
outcomes. These medications act to reduce urate levels and can reverse the
development of tophaceous deposits (Nuki & Simkin, 2006). Recently, the ACR
put together guidelines for the management of gout. At the time of diagnoses,
the individual is directed to adopt new dietary standards previously discussed
and evaluated for all possible causes of hyperuricemia with a comorbidity
checklist, current medications are reviewed to make sure that is not the cause,
and finally, upon history and physical examination, if the individual has been
found to have frequent presentations and/or have palpable tophi, a urate-
lowering treatment plan must be initiated. XOI treatment is initiated, typically
allopurinol or febuxostat. The treatment plan continues until targeted urate levels
are achieved (Khanna, 2012).
Because of the pain involvement and chronic nature of the disease process,
most rheumatic disease can have a component of depression. It is imperative to
consider this in all treatment approaches within the medical model. Psychosocial
factors can degrade pain tolerance, causing a worsening of the disease process.
Identifying and managing these components are critical to coping with chronic
pain (Backman, 2006).
Surgery
The management of rheumatic disease focuses primarily on medical
management, environment modification, adaptive techniques and tools, and
psychosocial interventions, but when those fail to prevent the erosive properties
of the disease, surgical intervention is recommended. Every surgical procedure
has its own inherent risks and complications. Individuals should understand
these risks and complications and have reasonable expectations for the
outcomes. Simple procedures dealing with synovitis issues like trigger finger
and carpal tunnel surgery are extremely effective with full functional outcomes.
Larger procedures, such as arthrodesis or joint replacement, fully remove the
damaged bone but do not restore full function. While joint replacements often
provide significant pain relief, deformity correction, and functional
improvements, they may not restore every facet of previous function. It is
important that individuals are prepared for the outcomes and the recovery
process before entering into any surgery (Grotle et al., 2010). Common joint
replacements include hip, knee, and thumb carpometacarpal, MCP, and PIP
joints.
Figure 23.19 shows an x-ray of an individual following implant resection
arthroplasty using a silicone implant of their MCP joints. Figure 23.20 depicts a
pyrocarbon implant inserted in a PIP joint. Figure 23.21 depicts a tendon
interposition arthroplasty for the thumb. The arthritic trapezium is removed and
replaced with a harvested tendon sutured to maintain the space previously
occupied by the trapezium. Figure 23.22 depicts an x-ray where the hip has
undergone a total hip arthroplasty.
Figure 23.19 An x-ray of an individual following implant resection arthroplasty
using a silicone implant of his or her MCP joints. (Courtesy of Timothy M.
Mullen.)
Figure 23.20 A pyrocarbon implant inserted in a PIP joint. (Courtesy of Timothy
M. Mullen.)
Figure 23.21 A tendon interposition arthroplasty for the thumb. (Courtesy of

Timothy M. Mullen.)
Figure 23.22 An x-ray where the hip has undergone a total hip arthroplasty.
(From St. Clair, E. W., Pisetsky, D. S., Haynes, B. F. (2004). Rheumatoid
arthritis. Philadelphia, PA: Wolters Kluwer.)
In 172 AD, Galen wrote, “employment is nature’s best physician and essential to
human happiness.” The roots of occupational therapy are founded in this notion
and aim to maximize occupational performance (Wright, 1947). Trombly
suggests identifying all deficits in occupational performance first in order to set
priorities (Trombly, 1995). When considering rheumatic diseases, occupational
deficits often include fatigue, difficulty with fine motor ADLs, pain, and lifestyle
changes. Quality of life must be maximized.
After all activities that make up an individual’s occupational performance
have been identified, it is important to categorize which of them are necessities
and which are preferred. The goal is to maintain an individual’s ability to engage
in the preferred occupation. Employing concepts of energy conservation can be
key to this concept. Educating proper technique and balancing rest and activity
in an individualized plan are imperative to maintaining quality of life in those
with disabling conditions (Furst, Gerber, Smith, Fisher, & Shulman, 1987). In
coordination with energy conservation, using joint protection principles is also
effective. Altering work methods, using orthotics, implementing assistive
devices, and educating clients can reduce the impact on the involved joints and
maximize independence. Some examples of joint protection include larger
handles on utensils for writing and food preparation, carrying heavy objects in
bags using larger joints versus holding larger items with fingers alone, and
wearing resting orthotics to sleep and functional orthotics to support activities
that require force like a Silver Ring Splint in someone with a swan-neck
deformity. Joint protection can reduce pain and localized inflammation and help
preserve the integrity of joint structures (Hammond & Freeman, 2001).
Lifestyle management must include the above interventions to mechanically
deal with rheumatic disease, but managing psychosocial functioning completes
the quality of life balance. Prolonged pain and feelings of hopelessness can lead
to maladaptive coping techniques. Research suggests that nearly half of all
individuals with RA report psychosocial issues (Bai et al., 2009).
Managing individuals with rheumatic diseases must be an interdisciplinary
approach focused on the individual’s medical needs, performance limitations,
and psychosocial issues. Maximizing quality of life through the most advanced
diagnostic and treatment approaches is fundamental to success. A diagnosis of
any rheumatic disease does not have to be a terminal sentence with our modern
approach.
CASE-STUDY 1
James Lochlan is an 11-year-old middle school student and active in club
sports and recreational activities. Lately, he has been coming home from
school complaining of back pain, knee pain, and hand weakness. He takes
lengthy naps and has difficulty making it to evening soccer practice. He has
good days where he was able to fully participate and bad days that
prevented him from active engagement. After several months, his parents
took him to his pediatrician. The physician ordered lab work and made a
referral to occupational therapy and a child psychologist. The lab work
revealed rheumatoid factor, and James was referred to a rheumatologist for
evaluation and treatment. Meanwhile, the psychologist employed modified
coping techniques for the times when he was too fatigued and the OT
worked out a schedule that spread out the periods of activity and inactivity
and educated him and his parents on some adaptive devices and strategies
to manage the times of extreme fatigue. The rheumatologist was able to
establish a pharmaceutical program that allowed him to go into remission
approximately 2 years after his initial diagnosis.
CASE-STUDY 2
Dorothea Kapp is a 47-year-old female who considers herself to be a typical
suburban mother with two teenage children. She works full-time as an
elementary school teacher. She began noticing increasing tenderness in her
index fingers of both hands. Within a week, the ends of her fingers were
quite swollen and red and she began to notice something similar in her right
big toe, to the point that she was unable to wear her shoe. She assumed it
was an infection that would go away in a day or two. After 2 weeks, she
was unwilling to weight-bear on her right foot and she was losing the
ability to bend her index fingers. When describing her symptoms to her
primary care physician, a referral was made to a rheumatologist. The
rheumatologist took blood and aspirated the edematous area on her right
foot and sent the samples out for laboratory evaluation. The rheumatologist
referred her to occupational therapy for management. The OT was able to
determine Dorothea’s goals were to be able to continue teaching, cooking
for her family, and caring for herself independently. The OT recommended
a modified metatarsal shoe, which applied no direct pressure to the area of
inflammation and had a hard sole to better disperse the pressure for weight
bearing. The OT also made custom orthotic tip protectors so that if her
fingers were bumped or she needed to use them for ADLs, the pressure was
dispersed and the areas of tenderness were fully protected. Built-up foam
handles with various inner diameters were issued for ease of manipulations
of things like toothbrush, pencils, dry-erase marker, razor, and eating
utensils. She was taught joint protection techniques and was encouraged to
ask her family for help with some household chores. She was prescribed
allopurinol, and within 4 weeks, the red, erythematic painful joints were
unnoticeable and she was able to resume normal activities.
Learning Resources
American Juvenile Arthritis Organization (AJAO) Tel: 404-872-7100, 1-800-
568-4045
www.arthritis.org
Arthritis Foundation (AF)
Tel: 404-872-7100, 800-568-4045
www.arthritis.org
The European League Against Rheumatism (EULAR) EULAR Executive
Secretariat
Seestrasse 240
8802-Kilchberg
Switzerland
Tel: +41-44-716-30-30
[email protected]
Higher Education and Training for People with Handicaps (HEATH)
George Washington University
HEATH Resource Center
2121 K Street NW, Suite 220
Washington, D.C. 20037
Tel: 800-544-3284
www.heath.gwu.edu
National Chronic Pain Outreach Association (NCPOA) P.O. Box 274
Millboro, VA 24460
Tel: 540-862-9437; Fax: 540-862-9485
www.chronicpain.org
Support Groups
Contact the Arthritis Foundation for local groups across the United States of
America
Professional Organizations
American College of Rheumatology (ACR)/Arthritis Health Professionals
Association (AHPA) 1800 Century Place
Suite 250
Atlanta, GA 30345
Tel: 404-633-3777; Fax: 404-633-1870
[email protected]
American Occupational Therapy Association (AOTA) 4720 Montgomery
Lane
P.O. Box 31220
Tel: 301-652-7711, 800-377-8555 (TDD), 1-800- SAYAOTA www.aota.org
The American Orthopedic Society for Sports Medicine 6300 N. River Road
Suite 500
Rosemont, IL 60018
Tel: 847-292-4900
www.sportsmed.org
American Podiatric Medical Association, Inc.
9312 Old Georgetown Road
Tel: 1-800-FOOTCARE
www.apma.org
American Physical Therapy Association (APTA) 111 N. Fairfax Street
Alexandria, VA 22314-1488
Tel: 1-800-999-2782, 703-683-6748 (TDD) www.apta.org
Arthritis Center
University of Missouri-Columbia
MA427 Health Sciences Center
1 Hospital Drive
Columbia, MO 65212
Tel: 314-882-8738
National Arthritis and Musculoskeletal and Skin Diseases Information
Clearinghouse (NAMSIC)
9000 Rockville Pike
PO Box AMS
Bethesda, MD 20892
Tel: 301-495-4484
www.niams.nih.gov/
AliMed Rehabilitation Products, Inc.
297 High Street
Dedham, MA 02026-9135
Tel: 1-800-255-2160
www.alimed.com
Amigo Mobility International, Inc.
6692 Dixie Highway
Bridgeport, MI 48722-9725
Tel: 1-800-248-9131
[email protected]
DeRoyal/LMB, Inc.
P.O. Box 1181
San Luis Obispo, CA 93406
Tel: 1-800-541-3992
Independent Living Aids, Inc.
27 East Mall
Plainview, NY 11803
Tel: 1-800-537-2118
Invacare Corporation
899 Cleveland Street
Elyria, OH
American Fibromyalgia Syndrome Association, Inc AFSA
7371 E Tanque Verde Rd
Tucson, AZ 85715
http://www.afsafund.org/
Lupus Foundation of America, Inc.
2000 L Street, N.W., Suite 410
Washington, DC 20036
http://www.lupus.org
Scleroderma Foundation
300 Rosewood Drive, Suite 105
Danvers, MA 01923
http://www.scleroderma.org
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C H A P T E R
24 Spinal Cord Injury
Laura V. Miller Rosanne DiZazzo-Miller “… of the many
forms of disability which can beset mankind, a severe
injury or disease of the spinal cord undoubtedly
constitutes one of the most devastating calamities in
human life” (Guttmann, 1976).
—Sir Ludwig Guttmann, pioneer in 20th-century management of spinal cord injury “The future lies in our
own hands, and if a challenge should enter our life, it is important to remember we have tremendous
strength, courage, and ability to overcome any obstacle.”
—Douglas Heir, Esq., Attorney-at-Law (personal communication, December 1994)
KEY TERMS
Autonomic dysreflexia (hyperreflexia) Catheterization Cauda equina Credé’s
maneuver Decubitus ulcers Deep vein thrombosis Heterotopic ossification
Peristalsis Reflex arc Spina bifida Spinal shock
The full impact of the preceding quotes may not strike the reader unless the
whole story is known. The latter author, Doug Heir, sustained a spinal cord
injury (SCI) at age 18. He dove into a pool to save a boy who appeared to be
drowning. The boy was only playing, but Doug’s injury resulted in tetraplegia.
Decades later, Doug has become known for being many things, among them an
author, US ambassador to the Soviet Union, cover athlete for Wheaties cereal,
associate legal editor of the National Trial Lawyer, and a gold medalist in the
1988 Olympics in Seoul, South Korea—an impressive list of accomplishments
for someone who sustained “one of the most devastating calamities in human
life!”The goals of the health care team should include empowering clients to
take charge of their futures. To accomplish this, the health professional must
understand the complexities of the diagnosis. This chapter explores the
ramifications of spinal cord injuries, beginning with a brief overview of the
central nervous system (CNS) and surrounding structures.
Overview of CNS and Related Structures
The brain and spinal cord make up the CNS. The spinal cord receives sensory
(afferent) information from the peripheral nervous system and transmits this
information to higher structures (i.e., the thalamus, cerebellum, cerebral cortex)
in the CNS. Descending motor (efferent) information, originating from the
cortex, is also transmitted by the spinal cord back to the peripheral nervous
system. The consistency of the spinal cord has been compared to a ripe banana,
and it is fortunate that the spinal cord and cerebral cortex are protected by bony
structures. Whereas the skull protects the brain, the vertebral column protects the
spinal cord. The vertebral column is composed of 33 vertebrae, with 7 cervical
vertebrae in the neck region (C1 through C7); 12 thoracic vertebrae in the chest
region (T1 through T12); 5 lumbar vertebrae in the midback region (L1 through
L5); 5 sacral vertebrae (S1 through S5), which are actually fused in the lower
back and pelvic region; and 4 fused coccygeal vertebrae that make up the
coccyx, or tail bone (Fig. 24.1). There are 31 pairs of spinal nerves, which exit
from the spinal cord and branch to form the peripheral nervous system. The
nerves exit through the openings formed between each two vertebrae. The spinal
nerves are named according to the vertebrae above or below the point of exit.
Note that spinal nerves C1 through C7 exit above the corresponding vertebrae,
whereas the remaining spinal nerves (C8 through S5) exit below the
corresponding vertebrae. Thus, although there are seven cervical vertebrae, there
are eight cervical spinal nerves. The actual spinal cord ends just below the L1
vertebra. However, some spinal nerves continue and exit beyond the point where
the spinal cord ends. Because of their visual resemblance, this bundle of nerves
is referred to as the cauda equina, which is Latin for horse’s tail (Grundy,
Tromans, & Jamil, 2002). The meningeal covering of the spinal cord, which
contains the cerebrospinal fluid (CSF) that bathes the structures of the CNS, also
extends past the end of the spinal cord to the L4 vertebral level. The CSF-filled
meningeal space between L2 and L4, referred to as the lumbar cistern, is the site
where diagnostic or therapeutic lumbar punctures, that is, spinal taps, are
performed, because the spinal cord is not present, yet CSF is accessible.
Figure 24.1 The spinal cord, spinal nerves, and vertebral column. (From Moore,
K. L., Agur, A. M. R., & Dalley, A. F. (2013) Clinically oriented anatomy (7th
Sensory and Motor Tracts
The terms tract, pathway, lemniscus, and fasciculus all refer to bundles of nerve
fibers that have a similar function and travel through the spinal cord in a
particular area. It is important to know the names, locations, and functions of
these tracts to understand the possible outcomes of an SCI at a given level.
Figure 24.2 shows the location of major tracts within a cross section of the spinal
cord.
Figure 24.2 Cross section of cervical spinal cord, shown in relation to
surrounding vertebral structures. (From Cohen, B. J. (2012). Memmler's
structure and function of the human body (10th ed.). Philadelphia, PA:
Lippincott Williams & Wilkins.) A. Spinal cord vertebral level and innervation.
B. Spinal cord cross-section diagram. C. Spinal cord cross-section image
Two basic types of nerve tissue make up the spinal cord. Gray matter is located
centrally and resembles a butterfly in cross sections of the cord. Gray matter is
composed of cell bodies and synapses. White matter encompasses most of the
periphery of the cord and contains the ascending and descending pathways.
Table 24.1 provides a more detailed description of the functions of the various
sensory and motor pathways that travel through the white matter of the spinal
cord. It may be helpful to remember that many pathways are named according to
their origin and the location of their final synapse (e.g., spinocerebellar,
corticospinal).
TABLE 24.1 Noninclusive Listing of Ascending and Descending Pathways
aCalled the posterior column.
Each pair of spinal nerves carries specific motor and sensory information. In
general, the cervical nerves (C1 through C8) carry afferent and efferent impulses
for the head, neck, diaphragm, arms, and hands. The thoracic spinal nerves (T1
through T12) serve the chest and upper abdominal musculature. The lumbar
spinal nerves (L1 through L5) carry information to and from the legs and a
portion of the foot, and the sacral spinal nerves (S1 through S5) carry impulses
for the remaining foot musculature, bowel, bladder, and the muscles involved in
sexual functioning. Table 24.2 and Figure 24.3 present a more detailed outline of
muscles innervated by each level of the spinal cord and a dermatomal
segmentation (sensory map) of the body.
TABLE 24.2 Spinal Cord Innervations/Function

MCP, metacarpophalangeal; IP, interphalangeal.
Figure 24.3 Dermatome map. (From Hoppenfeld, J. D. (2014). Fundamentals of
pain medicine. Philadelphia, PA: Lippincott Williams & Wilkins.)
Reflex Arc
Most nerve impulses move up the spinal cord to the brain and back through the
cord to the peripheral nerves. However, some impulses directly enter the cord
through the dorsal nerve root, synapse, and exit by the ventral nerve root. This
causes certain muscle functions or responses to occur without direction from the
brain. A simple example of this “looping” can be seen in the knee-jerk reflex. If
the knee is tapped with a reflex hammer, the knee will extend without any
influence from the brain. The stimulation by the hammer causes afferent
impulses to enter the cord, synapse, and exit, causing a contraction of the muscle
fibers (Fig. 24.4). This activity is called a reflex arc. In people with an intact
spinal cord, afferent nerve impulses also travel to the brain almost
instantaneously. This allows an awareness, or “feeling,” of the initial stimulation
(knee tap) and subsequent response (knee jerk). This concept is important to an
understanding of SCIs. It explains why some individuals with SCI continue to
have reflexes but do not have voluntary control of their muscles. It also explains
why others have no reflexes at all below the level of their injury. This is
discussed in much greater detail in the section on classification of injuries.
Figure 24.4 Knee-jerk reflex. (From Anderson, M. K. (2012). Foundations of
athletic training (5th ed.). Philadelphia, PA: Lippincott Williams & Wilkins.)
Etiology
Historically, many demographic sources attempted to count the number of
people who have sustained SCIs. Since 1973, the National Spinal Cord Injury
Database has been in existence, making strides in collecting comprehensive data
on a national level. In 1985, the Centers for Disease Control and Prevention
(CDC) began promoting surveillance mechanisms at state and national levels for
the collection and reporting of these data. Prior to this time, data related to
etiology and incidence of SCI in this country were inconsistently collected and
lacked uniformity; advancements are continuing to be made in this area.
The leading cause of SCI in the United States is motor vehicle accidents,
followed by falls and acts of violence (Fig. 24.5). Sports-related injuries account
for most of the remaining SCIs, with diving being historically the most common
(and preventable) cause (Table 24.3). Understanding the different contexts in
which SCI occurs is critical throughout the course of occupational therapy
evaluation and intervention. For example, inner-city populations in particular
comprise more SCI secondary to violence than in other environmental contexts.
In fact, in the United States, gunshot wounds (GSW) are the third most common
cause of SCI (Mayo Clinic, 2011; National Institute of Neurological Disorders
and Stroke, 2013). Occupational therapists need to be aware of the culture of
violence, environmental influences, and challenges with access to resources that
are often unsupportive of individuals with SCI secondary to GSW (DiZazzo-
Miller, 2015). Occupations take place throughout the dynamic union of client
factors, performance skills, and performance patterns (AOTA, 2014) and are
unique to each and every person with an SCI.
Figure 24.5 Etiologic distribution of spinal cord injury since 2015. National
Spinal Cord Injury Statistical Center (NSCISC). (2015). Recent trends in causes
of spinal cord injury. Birmingham, AL: University of Alabama at Birmingham.
Retrieved from
https://www.nscisc.uab.edu/PublicDocuments/fact_sheets/Recent%20trends%20in%20causes%
TABLE 24.3 Comparison of Select Sports-Related Spinal Cord Injuries

Developed with data from The National Spinal Cord Injury Statistical Center. 2014 Annual Statistical
Report, p. 48. University of Alabama at Birmingham, 2014.
Analyzing the etiology of SCIs helps target prevention programs. Public

awareness of the effects of using substances while operating a vehicle is
certainly heightened. Tougher penalties for driving under the influence of
cognitive altering substances have been enacted, and many states have adopted
seat belt, child restraint, and “distracted” driving legislation. All of these efforts
have the potential to reduce the leading cause of SCI. Grant monies have even
been awarded to hospital-based programs that evaluate the home environments
of senior citizens for safety. Their recommendations may reduce the risk of falls
—a major cause of SCI in the elderly. An innovative effort sponsored by the
University of Michigan Health System involves airing public service
announcements on the prevention of diving injuries before the “coming
attractions trailers” at popular movies for teens during the summer months.
Although much of the literature focuses on trauma, there are many
nontraumatic causes of spinal cord damage. Developmental conditions, such as
spina bifida, which is a congenital neural tube dysfunction resulting in an
incomplete closing of the vertebral column and spinal cord agenesis, may yield
many of the same clinical signs as traumatic SCI. Acquired conditions, such as
bacterial or viral infections, benign or malignant growths, embolisms,
thromboses, and hemorrhages— even radiation or vaccinations—can also lead to
damage of spinal cord tissue.
Incidence rates for SCI in the United States are estimated at 40 cases/million
population/year, excluding those who die at the scene of an accident (The
National SCI Statistical Center [NSCISC], 2013). This translates to about 12,000
new cases of SCI every year. The statistics indicate that over 80% of people who
sustain spinal cord injuries are male; notably, the mean age at time of injury has
increased from 28.7 years in the 1970s to the present mean age of 34.7 years
(NSCISC, 2014). Seasonal sports cause fluctuations in etiology and incidence
statistics throughout a given year, and some urban hospitals are reporting that a
disproportionate number of their SCI cases are caused by acts of violence
(NSCISC, 2014). In the United States, of the spinal cord injuries reported to the
national database since 2010, 63% of individuals were identified as White, 24%
Black, 2% Asian, and 10% Hispanic (NSCISC, 2014). One may be tempted to
conclude from these statistics that Caucasians are at higher risk for sustaining
spinal cord injuries—but this would be erroneous. When compared to the
composition of the general population, spinal cord injuries have a higher
incidence among non-Whites—specifically among Blacks where the general
population is 12% compared to 24% who acquire SCI (NSCISC, 2014).
Signs and Symptoms
Sensory Functions
The two major classifications of SCI are complete and incomplete. A complete
SCI occurs with a complete transection of the cord. In this case, all ascending
and descending pathways are interrupted, and there is a total loss of motor and
sensory function below the level of injury. The injury also may be referred to as
an upper motor neuron (UMN) injury, if the reflex arcs are intact below the level
of injury but are no longer mediated by the brain. UMN lesions are characterized
by (a) a loss of voluntary function below the level of the injury, (b) spastic
paralysis, (c) no muscle atrophy, and (d) hyperactive reflexes (Fig. 24.6).
Figure 24.6 A diagrammatic representation of the reflex arc. (From Barash, P.
G., Cullen, B. F., & Stoelting, R. K. (2012). Clinical anesthesia (5th ed.).
Complete injuries below the level of the conus medullaris (Fig. 24.1) are referred
to as lower motor neuron (LMN) injuries, because the injury has affected the
spinal nerves after they exit from the cord. In fact, injuries involving spinal
nerves after they exit the cord at any level are referred to as LMN injuries. In
these injuries, the reflex arc cannot occur, because impulses cannot enter the
cord to synapse. As a result, LMN injuries are characterized by (a) a loss of
voluntary function below the level of the injury, (b) flaccid paralysis, (c) muscle
atrophy, and (d) absence of reflexes. UMN and LMN injuries may be complete
or incomplete. There also may be a mixture of UMN and LMN signs after an
incomplete lesion in the lower thoracic/upper lumbar region. The following
section discusses incomplete injuries in greater detail.
Incomplete Injuries
If damage to the spinal cord does not cause a total transection, there will still be
some degree of voluntary movement or sensation below the level of injury. This
is known as an incomplete injury, which may be further categorized according to
the area of the spinal cord that was damaged and the clinical signs that are
present.
Anterior Cord Syndrome
This syndrome results from damage to the anterior spinal artery or indirect
damage to anterior spinal cord tissue (Fig. 24.7). Clinical signs include loss of
motor function below the level of injury and loss of thermal, pain, and tactile
sensation below the level of injury. Light touch and proprioceptive awareness are
generally unaffected (Hayes, Hsieh, Wolfe, Potter, & Delaney, 2000).
Figure 24.7 A cross section of the spinal cord illustrating the damage that causes
anterior cord syndrome. The anterior artery is involved, resulting in damage to
most areas, with the exception of the posterior columns. (From Pellico, L. H.
(2012). Focus on adult health. Philadelphia, PA: Lippincott Williams &
Wilkins.)
Brown-Séquard’s Syndrome
This syndrome occurs when only one side of the spinal cord is damaged (Fig.
24.8). A hemisection of this nature frequently is the result of a penetrating (e.g.,
stab, gunshot) wound. The clinical signs of Brown-Séquard’s syndrome
generally include
Figure 24.8 Cross section of the cord, illustrating the damage that results in
Brown-Séquard syndrome. (From Pellico, L. H. (2012). Focus on adult health.
Ipsilateral loss of motor function below the level of injury.
Ipsilateral reduction of deep touch and proprioceptive awareness. (There is a
reduction rather than loss as many of these nerve fibers cross.) Contralateral
loss of pain, temperature, and touch.
Clinically, a major challenge presented by Brown-Séquard’s syndrome is that
the extremities with the greatest motor function have the poorest sensation.
Central Cervical Cord Syndrome
In this lesion, the neural fibers serving the upper extremities are more impaired
than those of the lower extremities (Fig. 24.9). This occurs because the fibers
that innervate the upper extremities travel more centrally in the cord and, as the
name of the syndrome implies, the central structures are the ones that are
damaged (Fig. 24.2). Injury to the central portion of the spinal cord is often seen,
along with structural changes in the vertebrae. Most commonly, hyperextension
of the neck, combined with a narrowing of the spinal canal, results in this type of
injury. Because arthritic changes can lead to spinal canal narrowing, this
syndrome is more prevalent in aging populations. The signs of central cord
syndrome often include:
Figure 24.9 Cross section of the cord, illustrating the damage resulting in central
cervical cord syndrome. (From Pellico, L. H. (2012). Focus on adult health.
Motor and sensory functions in the lower extremities less involved than in the
upper extremities.
Improvements in intrinsic hand function are generally evidenced last, if at all
(Ackerman, Foy, & Tefertiller, 2013).
A potential for flaccid paralysis of the upper extremities, as the anterior horn
cells in the cervical spinal cord may be damaged. Because these are synapse
sites for the motor pathways, an LMN injury may result.
Cauda Equina Injuries
Cauda equina injuries do not involve damage to the spinal cord itself but rather
to the spinal nerves that extend below the end of the spinal cord (Fig. 24.1).
Injuries to the nerve roots and spinal nerves that constitute the cauda equina are
generally incomplete. Because this type of injury actually involves structures of
the peripheral nervous system (exiting spinal nerves), there is some chance for
nerve regeneration and recovery of function if the roots are not too severely
damaged or divided. These injuries are usually the result of direct trauma from
fracture dislocations of the lower thoracic or upper lumbar vertebrae. Clinical
signs of cauda equina injuries include
Loss of motor function and sensation below the level of injury.
Absence of a reflex arc, as the transmission of impulses through the spinal
nerves to their synapse point is interrupted. Motor paralysis is of the LMN type,
with flaccidity and muscle atrophy seen below the level of injury. Bowel and
bladder function are also areflexic (American Spinal Injury Association, 2008).
Conus Medullaris Injuries
Conus medullaris injuries are similar to cauda equina injuries. In many cases, it
is very difficult to distinguish between these two types of injuries. They can both
cause similar signs and symptoms such as local, referred, and radicular pain, loss
of sphincter control, and gluteal and lower extremity sensation and weakness
(Byrne, Benzel, & Waxman, 2000). Clinical signs of conus medullaris injuries
include the following:
Loss of motor function and sensation below the level of injury, although
typically not severe.
Absence of a reflex arc, as the transmission of impulses through the spinal
nerves to their synapse point is interrupted. Motor paralysis is of the LMN type,
with flaccidity and muscle atrophy seen below the level of injury. Bowel and
bladder incontinence and sexual dysfunction are typically more severe than
cauda equina injuries (Byrne et al., 2000).
Complete Versus Incomplete Injuries
In both complete and incomplete injuries, the terms quadriplegia, tetraplegia,
and paraplegia may be used to further describe the impact of the injury.
Quadriplegia refers to lost or limited function of all extremities as a result of
damage to cervical cord segments. The American Spinal Injury Association
(ASIA) advocates the term tetraplegia over quadriplegia. Tetraplegia refers to
impairment or loss of motor or sensory function in the cervical segments of the
spinal cord that is the result of damage of neural elements within the spinal
canal. Tetraplegia causes impairment of function in the arms as well as in the
trunk, legs, and pelvic organs. It does not include brachial plexus lesions or
injury to peripheral nerves outside the neural canal (American Spinal Injury
Association/International Medical Society of Paraplegia, 2013). Paraplegia,
which refers to lost or limited function in the lower extremities and trunk
depending on the level of injury, occurs after lesions to thoracic, lumbar, or
sacral cord segments. Spinal cord injuries are frequently classified further, based
on the ASIA Impairment Scale (American Spinal Injury
Association/International Spinal Cord Society, 2013), which contains the
following levels:
Level A Complete; no motor or sensory function is preserved in the sacral
segments S4 through S5.
Level B Sensory incomplete; sensory but not motor function is preserved below
the neurological level and extends through the sacral segments S4 through S5.
Level C Motor incomplete; motor function is preserved below the neurological
level, and the majority of key muscles below the neurological level have a
muscle grade <3.
Level D Motor incomplete; motor function is preserved below the neurological
level, and the majority of key muscles below the neurological level have a
muscle grade of ≥3.
Level E Normal; motor and sensory function is normal.
A manual muscle test is performed to assess the strength of muscles and aid in
the determination of the extent and nature of injury. Usually, strength is graded
using the following scale (Moroz, 2015):
0Total paralysis
1Palpable or visible contraction 2Active movement, full range of motion (ROM)
with gravity eliminated 3Active movement, full ROM against gravity 4Active
movement, full ROM against moderate resistance 5Normal active movement,
full ROM against full resistance NT Not testable
A prognosis implies that one can forecast or predict the outcome and chances for
recovery from a particular disease or traumatic injury. That is somewhat
challenging for SCIs. Although some aspects of SCI are highly predictable (e.g.,
specific muscle functions impaired with a complete lesion at C5), other aspects
are much more vague.
Part of the ambiguity lies in the definitions of the term “recovery.” One
definition is to get back, or regain, which tends to be the client’s focus. Another
definition for recovery stresses compensation, which is often the thrust of the
health care professional working with SCI. While the physiological prognosis of
SCI will be discussed here, the clinician should always be aware, and
acknowledge the validity, of the client’s perspective on recovery. To “regain”
and to “compensate for” are dramatically different frames of reference. When
discussing prognosis with a person who has survived an SCI, the clinician
should always be truthful but must also be acutely aware of the impact of what
the client is hearing. Perhaps the most crucial indicators of an individual’s
functional outcome are personal characteristics such as motivation, use of
support systems, and coping mechanisms. The clinician must be skilled at
fostering these strengths while at the same time providing accurate information.
Posttraumatic Complications
Spinal Shock
The period of altered reflex activity immediately after a traumatic SCI is known
as spinal shock. As a result of injury, spinal cord segments below the level of
the lesion are deprived of excitatory input from higher CNS centers. What is
observed clinically during this phase is a flaccid paralysis of muscles below the
level of injury and an absence of reflexes. The bladder is also flaccid, requiring
catheterization, which is a procedure to enable flow of urine by way of
insertion of a latex tube into the bladder by way of the urethra when there is no
voluntary control of the bowel. Depending on the level of the injury, the person
with an SCI may require a ventilator because of lost or temporarily interrupted
innervation to the diaphragm, intercostals, and abdominal muscles.
Spinal shock generally lasts from 1 week to 3 months after injury. Once
spinal shock subsides, the areas of the spinal cord above the level of the lesion
operate as they did premorbidly. Below the level of the lesion, reflexes will
resume if the reflex arc is intact. This is an important concept to understand.
Unlike a plant, which may die entirely if its stem is cut in half, the spinal cord is
still alive and functional above and below the level of injury. The problem is one
of communication; the brain cannot receive sensory information beyond the
lesion site and cannot volitionally control motor function below that point.
After spinal shock subsides, there is often an increase in spasticity, especially
in the flexor muscle groups. The reflex arc “fires,” and the brain is unable to
interfere. After this phase, there may be a period of 6 to 12 months after injury
when an increase in the spasticity of the extensor groups is common. Usually,
after 1 year postinjury, the wide fluctuations in tone will cease. An array of
complications can greatly affect the prognosis of a person who has sustained an
SCI. Some of the more common medical complications are addressed in the next
section.
Respiratory Complications
People with spinal cord injuries at or below the level of T12 generally have a
normal respiratory status. Injuries above that level, however, compromise the
respiratory system to some degree. The abdominal musculature is innervated by
segments T7 through T12, the intercostal muscles are served by segments T1
through T12, and the diaphragm is innervated by C4. People with complete
injuries above C4 usually need a respirator. Some may be candidates for a
phrenic nerve stimulator if the nerve shows the ability to conduct an impulse.
Generally, people with complete injuries at C4 and below do not use respirators,
but respiratory complications may persist. Breathing may be shallow, and the
ability to cough productively may be compromised. Various deep-breathing and
assisted-coughing techniques may be taught, along with other procedures to keep
the lungs clear. Prevention and early management of respiratory complications
are crucial. Currently, respiratory complications are the most common cause of
death following SCI (Brown, DiMarco, Hoit, & Garshick, 2006).
Autonomic Dysreflexia (Hyperreflexia)
As implied by its name, autonomic dysreflexia, or hyperreflexia, involves an
exaggerated response of the autonomic nervous system (ANS). A function of the
ANS is the integration of body functions in the “fight-or-flight” response—heart
rate, blood vessel constriction/dilation, regulation of glands, and smooth muscle.
Autonomic dysreflexia usually occurs in people with spinal cord injuries above
the T6 level. Signs to look for include a sudden, pounding headache, diaphoresis,
flushing, goose bumps, and tachycardia followed by bradycardia. These signs
are caused by an irritation of nerves below the level of injury. Common sources
of irritation include an overfull bladder or bowel, urinary tract infections (UTIs),
or decubitus ulcers. Even irritations such as ingrown toenails can trigger the
response. These irritations would be bothersome to a person with an intact spinal
cord—he or she would feel uncomfortable and act to remedy the situation. But
the person with an SCI lacks this feeling, and autonomic dysreflexia is the
body’s way of warning that something is wrong below the level of the injury.
The most important aspect of managing autonomic dysreflexia is to find the
cause and alleviate it. This may require emptying the bladder, checking for
obstructions in external urinary drainage tubing, assessing for bowel impaction,
or evaluating for other factors. It helps to decrease blood pressure if the person
assumes an upright position. Most people with tetraplegia will experience an
episode of autonomic dysreflexia at least once, but if the signs of autonomic
dysreflexia appear frequently, medication may be indicated. Autonomic
dysreflexia may appear suddenly and must be managed promptly. Because the
blood pressure may elevate dramatically, there is risk of stroke or death if the
situation is ignored or mismanaged. Although autonomic dysreflexia may be
more prevalent in the initial months following injury, it has the potential of being
an ongoing complication, and people with SCI must be educated about
symptoms and management (Russo-McCourt, 2009).
Postural Hypotension
In contrast to autonomic dysreflexia, blood pressure decreases in postural
hypotension. This condition, often seen in people who have sustained cervical or
thoracic SCIs, also may be referred to as orthostatic hypotension (Krassioukov,
Warburton, Teasell, & Eng, 2009). Blood tends to pool distally in the lower
extremities as a result of reduced muscle tone in the trunk and legs. The
symptoms of postural hypotension frequently occur when a person attempts to
sit up after prolonged periods of bed rest. Symptoms include light-headedness,
dizziness, pallor, sudden weakness, and unresponsiveness. Preventive measures
include the use of antiembolism hosiery and abdominal binders, which
externally assist circulation. Also, assuming an upright position slowly can help
avoid these symptoms. If symptoms do occur, a semireclined or reclined position
should be maintained until the symptoms subside.
Deep Vein Thrombosis
Deep vein thrombosis (DVT) can be a serious complication in many types of
medical conditions. It is a potential complication in SCI for three main reasons:
reduced circulation caused by decreased tone, frequency of direct trauma to legs
causing vascular damage (e.g., repeated trauma during transfer or bed mobility
activities), and prolonged bed rest. Edema is often seen in SCI for the same
reasons. Clinical signs of DVT may include swelling in the lower extremities,
localized redness, and a low-grade fever. However, a DVT may be relatively
asymptomatic on bedside evaluation. Vigilant medical screenings for DVT
should be performed in all cases of SCI. An undetected and unmanaged DVT
may result in an embolism and death. In people with SCI, it appears the greatest
risk of DVT is seen within the initial 2 weeks postinjury (American Spinal Injury
Association, 2008; Consortium for Spinal Cord Medicine, 2006).
Thermal Regulation
It has already been seen that damage to the spinal cord can disrupt the ANS,
possibly resulting in autonomic dysreflexia. Thermal regulation is another
function of the ANS that can be disturbed after SCI. Maintaining the appropriate
body temperature is often a problem for people whose injuries are above T6.
During the first year after injury, the body tends to assume the temperature of the
external environment. This condition is called poikilothermia (Wood, Binks, &
Grundy, 2002). In time, some adjustment usually occurs. Cold weather often
causes discomfort, as blood vessels below the level of injury do not constrict
sufficiently to conserve the body’s heat. Conversely, excessive sweating may
occur above the level of injury in warmer weather but not below, which hampers
the body’s efforts to prevent hyperthermia. Because of this, extreme
temperatures should be avoided, and attention should be given to the extent and
type of clothing worn in all conditions.
Musculoskeletal
Spasticity
In people who have UMN lesions, increased tone appears in muscles below the
level of injury after spinal shock subsides. Virtually all individuals with cervical
cord injuries experience spasms, and the percentage of those experiencing
spasms gradually decreases as the level of the lesion descends along the spinal
cord column— with flexors and extensors experiencing the majority of spasms
(Christopher & Dana Reeve Foundation, 2015b). An increase in spasticity can be
triggered by a variety of factors including infections, positioning, pressure sores,
UTIs, and heightened emotional states. Spasms are not necessarily
disadvantageous. The ability to trigger spasms can help some individuals
maintain muscle bulk, circulation, bowel and bladder management, transfers,
and other activities of daily living (ADL). Excessive spasticity, however, may
result in contractures, pain, and a reduced ability to participate in activities. At
this point, pharmacological or surgical options may be recommended.
Heterotopic Ossification (Ectopic Bone)

Heterotopic ossification (HO) refers to the abnormal formation of bone deposits
on muscles, joints, and tendons. It occurs most often in the hip and knee and less
frequently in the shoulder and elbow. It has been estimated that 20% of all
people with SCI have some degree of ectopic bone growth (Cipriano, Pill, &
Keenan, 2009). Clinical signs of HO may include heat, pain, swelling, and a
decrease in active or passive ROM. These signs should always alert the clinician,
as they may also indicate other serious complications such as a DVT. Many
facilities that specialize in the care of people with SCI routinely provide
prophylactic medications that have shown promise in halting this abnormal
calcification. In extreme cases in which HO permanently and severely limits
ROM, surgery may be indicated.
Genitourinary Complications
UTIs are a common and dangerous complication of SCI. Prior to modern
medical management, many people with SCI who survived the initial trauma
died within a few years after injury, with one of the most common causes being
kidney failure as a result of chronic UTI. For several reasons, individuals with
SCI are prone to UTI or bladder infections. The bladder is composed of smooth
muscle, innervated by sacral segments of the spinal cord. As such, it is affected
by a loss of sensory and motor function, as are other parts of the body,
depending on the level and extent of injury. The nature of bladder function will
depend on whether the injury caused LMN or UMN deficits (Fig. 24.10). An
injury affecting the UMN bladder is also referred to as a reflex or spastic
bladder. In this case, the bladder can contract and void reflexively. Although this
action is involuntary, some people with SCI can trigger the reflex through
various stimuli, much as the knee-jerk reflex being triggered by tapping with a
reflex hammer. This is because impulses can still enter the cord below the level
of injury, synapse, and exit.
Figure 24.10 Bladder and corresponding spinal segment innervations. (From
Rhoades, R. A., & Bell, D. R. (2012). Medical physiology (4th ed.).
People with a UMN bladder may use various types of catheters and additional
techniques to ensure that the bladder does not become distended or retain urine.
They generally cannot rely on sensation to alert them that the bladder has
exceeded its normal capacity; rather, they must rely on an established voiding
schedule.
An LMN bladder may also be referred to as a nonreflex or flaccid bladder.
This type of bladder function is usually seen during the spinal shock phase and
may remain if the injury has affected the cauda equina area. With an LMN
bladder injury (Fig. 24.10), a reflexive emptying of the bladder cannot occur, as
the reflex arc is destroyed. Because the bladder is flaccid and does not
spontaneously empty, urine will accumulate continuously. People with an LMN
bladder must catheterize according to a schedule or must apply external pressure
to force urine from the bladder. The application of external pressure on the
abdomen with their fists, starting at the umbilicus and pressing downward, is
called Credé’s maneuver. Another technique for generating force is called the
Valsalva maneuver, which involves closing the glottis and contracting the
abdominal muscles, as if resisting a forceful exhalation (Somers, 2010). Chronic
use of Credé’s maneuver may lead to multiple complications, including inguinal
hernias, hemorrhoids, and vesicoureteral reflux (Chang, Hou, Dong, & Zhang,
2000; Consortium for Spinal Cord Medicine, 2006; Somers, 2010).
With either type of bladder (UMN or LMN), voiding must occur routinely
and completely. Chronic overstretching of the bladder will reduce its ability to
empty adequately. Residual urine is a breeding ground for infections that can
spread to all structures in the urinary system, including the ureters and kidneys.
Chronic infections can lead to renal calculi (kidney stones), kidney failure, and,
potentially, death. Warning signs of a UTI include urine that appears cloudy or
has excessive particles, dark or foul-smelling urine, an elevated fever, chills, or
an increase in spasticity. The best treatment of UTI is prevention— adhering to
an effective voiding schedule, using clean or sterile techniques, maintaining a
proper diet and adequate fluid intake, and prompt attention to warning signs.
Complications Associated with the Bowel
Normally, elimination occurs when stool is present in the rectum. Nerves in the
rectal musculature are stimulated, triggering a reflexive peristalsis, which is a
process of muscle contractions resulting in the movement of food through the
digestive track and a relaxation of the rectal sphincters. A bowel movement may
be prevented at this step of the process if the brain overrides this reflex, sending
down an impulse to tighten the sphincter muscles until an appropriate time. We
have all experienced the sensation of urgency caused by a full rectum, but
perhaps we have not fully appreciated our brain’s ability to allow us to forestall
the process until a socially acceptable time.
Unfortunately, an SCI can interfere with bowel function in much the same
way as it impedes the bladder. The bowel can become spastic or flaccid (Fig.
24.11). In this case, stool can be eliminated reflexively if nerves located in the
rectum are stimulated. This stimulation may be done manually through digital
stimulation or in conjunction with the use of suppositories. Establishing and
following a regular schedule for bowel management can reduce occurrences of
incontinence.
Figure 24.11 The bowel and corresponding spinal segment innervations. (From
Moore, K. L., Agur, A. M., & Dalley, A. F. (2014). Essential clinical anatomy
The bowel is usually flaccid during the phase of spinal shock and may remain in
that state if the injury involves the areas illustrated at points B and C (Fig.
24.10). As with the flaccid bladder, the flaccid bowel cannot be stimulated to
empty reflexively. Stool often remains in the rectum after attempts at evacuation,
and it may be necessary to remove it manually to prevent impaction.
Constipation or impaction may result if elimination does not occur regularly.
In addition to the general discomfort associated with this, autonomic dysreflexia
may be triggered in people with lesions above the T6 level. Diarrhea is another
complication that can be particularly frustrating for the person with an SCI who
is trying to establish a set schedule for bowel management. This condition is
always frustrating, but the majority of the general population have the benefit of
intact sensation to provide a warning. The best strategies to prevent diarrhea for
people with an SCI include making sure to use (not overuse) laxatives if they are
prescribed, eating a proper diet, maintaining adequate hydration, and following a
scheduled bowel program that reduces the chance of impaction, which can result
in diarrhea.
Dermal Complications
The skin is the largest organ of the body, and it performs essential functions in
maintaining health. Skin assists in thermal regulation, providing insulation in
cold weather and sweating to prevent hyperthermia during hot conditions. Aside
from literally keeping the body together, skin acts as a barrier to the external
environment. At the cellular level, skin provides the site for O2/CO2 exchange
through the capillary system. Keeping skin intact is essential and requires
conscious effort by a person with an SCI, as sensations that would normally
provide warning of potential skin damage, such as pain and extremes in
temperature, are not perceived below the level of injury.
The story of our own modern-day Superman, Christopher Reeve, bears
witness to the importance of skin integrity. What eventually took his life were
not the leading causes of death for people with SCI such as renal failure,
pneumonia, pulmonary emboli, or sepsis (NSCISC, 2009). What took the life of
Superman was a pressure ulcer that became infected—ultimately resulting in
organ failure.
Damage to the skin as a result of pressure sores, or decubitus ulcers, is a
major reason for hospital admissions in SCI populations. The mechanism of
injury is continued pressure due to lack of movement. Circulation is impaired
because of the pressure, and capillary exchange is impeded. This can result
rapidly in tissue necrosis. The severity of pressure sores can be classified in four
stages:
Stage I. Clinical signs are reddened or darkened skin. Damage is limited to more
superficial (epidermal and dermal) layers. At this stage, merely removing
pressure until the skin returns to its normal color can halt tissue breakdown.
Stage II. The skin now appears reddened and open. A blister or scab is present.
The scab is not a sign of healing; rather, the tissue beneath it is necrotic. This
involves the epidermal and dermal layers, as well as deeper adipose tissue.
Wound dressings may be involved at this stage, and it is imperative that pressure
be kept off the site.
Stage III. The skin breakdown is deeper, and the wound is now draining. Muscle
may be visible through the open wound. An ulcer is developing in the necrotic
tissue. In addition to wound dressings, surgical intervention may be indicated if
more conservative treatment is unsuccessful.
Stage IV. All structures, from the superficial levels to the bone, are destroyed.
Infection and bone decay occur. Surgical intervention is likely, and the person
with a decubitus ulcer at this stage often must spend weeks after a skin graft with
pressure totally removed from the involved site (Kroshinsky & Strazzula, 2015).
Pressure sores are preventable with diligent attention and preventative strategies.
A person with SCI can use, or instruct another person to assist with, a variety of
pressure relief methods. Also, visual skin inspections should be performed at
least twice daily, taking particular note of areas most prone to breakdown. These
would include areas where bony prominences (e.g., the sacrum, ischium,
calcaneus, and scapula) can add to pressure. Proper nutrition also should be
heeded, as healthy skin is less apt to break down and is more responsive to
healing in early stages of pressure sore development.
Other dermal complications, such as burns or frostbite, are prevented by
attentiveness and common sense. Even commonly encountered things, such as
space heaters or exposed plumbing under a sink, can cause severe burns to
people with an SCI without their immediate knowledge. It is important to be
aware of the environment and to rely on other, intact senses to avoid injury.
Mental Health Challenges
Prevalent throughout the SCI literature is the high rate of depression in this
population (Bombardier, Richards, Krause, Tulsky, & Tate, 2004), which,
unaddressed, was found to be associated with greater incidence of pressure
ulcers as well as decreased occupational performance (Fann et al., 2011).
Depression significantly complicates daily living and is characterized by
disinterest and feelings of worthlessness, fatigue, weight and appetite changes,
and suicidal ideations (Kalpakjan, Bombardier, Schomer, Brown, & Johnson,
2009). The Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition
(DSM-5) is used to evaluate symptoms in regard to intensity and duration (APA,
2013). It is critical for occupational therapists to immediately refer patients with
suspected depression to appropriate health care professionals and notify the
treatment team accordingly.
Medical management of an SCI should begin immediately at the onset of injury.
As typically injuries occur outside a hospital setting (roadways, lakes, sporting
events), often first responders are not medical personnel. Any person with a
suspected SCI should be immobilized with a backboard and neck brace before
transport (Cooper, 2006). Diagnostic evaluation of the vertebral column and
spinal cord must be completed to determine the next steps of intervention.
Injuries may require surgical or nonsurgical intervention to decompress, realign,
and stabilize the spine (Russo-McCourt, 2009). Surgical procedures such as
laminectomies may be performed to remove the cause of pressure on the cord
(such as a bony fragment or bullet) (Grundy et al., 2002). Spinal fusion may also
be required to achieve stability. External alignment devices may be used
following spinal surgery or may be sufficient substitutes for surgery (Wood et
al., 2002). In thoracic injuries, a TLSO (thoracolumbosacral orthosis) or
“clamshell” brace is often used. This device is a “total contact” molded 2-piece
brace, applied to the anterior and posterior of an individual to limit trunk
movement (University of California, San Francisco, 2015). Multiple devices also
exist for external stabilization of cervical injuries. These orthoses typically
restrict the chin and the posterior aspect of the skull, restricting cervical motion
(Somers, 2010). Among the most restrictive cervical devices is the “halo”
orthosis. This is composed of a metal ring affixed directly to the skull with
screws (called “pins”) at occipital and temporal points and steel bars mounted to
a body vest (Somers, 2010). The determination of the need and/or type of
orthosis required demands careful assessment of vertebral stability and the
nature of movement the device is able to restrict.
Concurrent with spinal decompression, realignment, and stabilization,
prompt pharmacologic management is vital (Russo-McCourt, 2009). In the most
acute stages following injury, recent research headlines have reported that the
use of steroids—specifically, methylprednisolone—may improve neurological
outcomes of patients with SCI if administered within the first 8 hours after injury
(Bracken, 2009). Additionally, select experimental drugs administered soon after
injury, such as GM-1 ganglioside (Sygen) and 4AP, were hoped to aid in the
preservation of damaged nerves and insulating them from the toxic level of
chemicals the body releases during trauma and possibly reduce cell death
(http://www.spinalcord.org/news). Study outcomes to date relating to the benefit
of these drugs are mixed, and some concerns have been raised about potential
adverse effects versus limited functional gains with some of these protocols.
Another very recent acute experimental intervention that has been tried to
mitigate spinal cord damage and hopefully improve outcomes is “therapeutic
hypothermia.” This process involves lowering the core body temperature to
discourage spinal cord edema and reduce metabolic demands soon after injury
occurs (Cappuccino, 2008). While this technique has been used historically for
other diagnoses, its consideration in the management of SCI is relatively new.
Perhaps the first introduction to this technique for many was during the 2007
National Football League season opening game, when millions watched live as
Buffalo Bills’ tight end Kevin Everett was involved in helmet-to-helmet contact
that resulted in a cervical SCI. The team’s orthopedic surgeon administered
almost-immediate hypothermic intervention. The technique, which Everett has
credited with his positive outcomes, was described for days in the media
(Higgins, 2007). Certainly, significant additional research needs to be conducted
in all aspects of the very acute management of SCI, but improved initial
treatments hold promise for better long-term outcomes.
On other fronts, while fetal stem cell research relating to spinal cord
regeneration is prevalent in the news, meaningful functional outcomes have been
limited. However, newer research related to stem cells found in a living donor’s
brain, spinal cord, or other structures may hold greater promise and potentially
avoid problems of cell rejection found with fetal stem cells (NINDS, 2015).
Headlines were made in the recent past regarding the treatment of spinal cord
injuries with the use of stem cells from umbilical blood. This process reportedly
resulted in a South Korean woman who had been paralyzed for 20 years being
able to perform some level of ambulation with assistance (Gallagher, 2004.).
Autologous (self-donated) bone marrow stem cell transplants have also been
shown in select reported case studies to result in functional gains and quality of
life improvements in spinal cord–injured recipients (Geffner, Santacruz, Izuretta,
2008). Additional research is being aimed at a variety of strategies, including
pharmacologic and enzyme therapy, cloning of nerve cells, and the highly
sophisticated altering of the cellular environment to encourage actual
regeneration. As stated earlier, the spinal cord is typically intact above and below
the level of injury; one goal of current research is to encourage functional
reconnection of the disrupted pathways. A different picture is presented with
cauda equina injuries or injuries involving the nerve root. These types of injuries
carry the potential for regeneration if the nerve roots are not severely damaged
or divided. The degree of regeneration that can be expected is very difficult to
predict and should be addressed on an individual basis after extensive medical
testing.
Aside from research on actual spinal cord cell replacement and regeneration,
significant work is also being done with highly technical devices to compensate
for paralysis. Retraining CNS circuits includes treatments such as epidural
stimulation (ES), functional electric stimulation (FES), robotic-assisted therapy,
and brain-computer interfaces (BCI) (NINDS, 2015). Epidural stimulation was
used on a man with paralysis from an SCI along with intensive physical therapy.
After 2 years of locomotor training, electrodes were surgically implanted over
the paralyzed area thereby simulating the brain’s signals to the spinal cord.
Although locomotor training without ES is common practice in rehabilitation,
inclusion of ES has the ability to relieve pain during training. This individuals’
success provides hope for further investigations on how ES can help regain
function in people with SCI paralysis (NINDS, 2015). People with UMN injuries
have experienced increases in their functional abilities (improved ability to
productively cough, upper extremity function, standing ability, ambulation) with
the external application of electrodes that stimulate muscle contraction through
FES. Although some may feel that the FES apparatus is cumbersome, unsightly,
time consuming, and difficult to apply, refinements are ongoing. Many people
with SCI feel that, although not ideal, the technology provides an appealing
alternative to outcomes from traditional methods of rehabilitation. Another
technological development is the use of neuroprosthetic devices, which are
surgically implanted FES systems. One device, the Freehand System, combines
internal electrodes, stimulators, sensors, and transmission coils within an
external control box. It allows a person with tetraplegia to achieve a degree of
functional prehension, enhancing such ADL as eating, writing, and phone and
computer use. Select studies have shown gains in the functional performance of
individuals with C5 to C6 tetraplegia utilizing this system (Taylor, Esnouf, &
Hobby, 2002). Research on robotic-assisted therapy is being used in various
ways for people with incomplete SCI—to investigate assisted movement and
enhanced sensation (AMES) technology by rotating the ankle and moving the
leg, as well as through determining the effectiveness of body weight–supported
treadmill training using a robotic gait trainer. Both areas of research are novel
within the field of SCI recovery (NINDS, 2015). Finally, BCI technology has the
ability to bypass damaged nerve circuits within the spinal cord, thereby
providing a link from the brain to an assistive implanted device in order to
restore paralyzed muscles with voluntary muscle control and coordination
(NINDS, 2015). Electrodes are placed in the brain and through practice in
directing their thoughts, patients can generate neural signals interpreted by the
computer and translated into movement. This is currently being researched on
people with UE paralysis as well as people with tetraplegia (NINDS, 2015).
Overall, although actual regeneration of the cord may not be on the immediate
scientific horizon, the advances in all phases of SCI management are promising.
Everyone has the right to hope for what is not yet reality, without being said to
have unrealistic expectations. Assisting someone to be hopeful, while
simultaneously working to maximize today’s function, is to truly master the art
of the therapeutic relationship.
Performance in areas of occupation relates to a person’s ability to engage in or
direct caregivers on activities that are essential and meaningful. Performance in
areas of occupation includes ADL, instrumental activities of daily living (IADL),
education, work, play, leisure, and social participation. Occupational
performance for a person with an SCI can also include directing caregivers on
how to assist with these fundamental aspects of their daily living (AOTA, 2014).
Clearly, an SCI can have a catastrophic effect on a person’s ability to function in
these areas, because it can affect body function categories that support the ability
to participate in these activities. Body function categories that may be impacted
by an SCI include sensory functions and pain, voice and speech functions,
functions of cardiovascular and respiratory systems, genitourinary and
reproductive functions, neuromusculoskeletal and movement-related functions,
functions of the skin and related structures, and select mental functions. The
importance of understanding the theory of occupational performance cannot be
overstated. We cannot begin to holistically treat a person with SCI until we can
visualize the impact that this diagnosis has on the various aspects of that
person’s life. The following sections explore occupational performance areas and
components to present a comprehensive view of the impact of SCI. Table 24.4
shows generally expected functional outcomes at various levels of complete
injuries.
TABLE 24.4 Expected Functional Outcomes of Various Levels of Complete

Injury (Noninclusive)
Developed with material from the Rehabilitation Institute of Michigan, 1996.
Grooming, Oral Hygiene, Eating, Bathing, Dressing
For a person with tetraplegia, grooming, oral hygiene, and eating may be
extremely laborious. The use of extensive adaptive devices or reliance on a
caregiver may be necessary because of deficits in sensory and neuromuscular
performance components. People with paraplegia are generally independent in
these tasks, but they must often think ahead, making sure that items are available
and sufficient time is allocated. Bathing and dressing specifically present major
challenges for people with quadriplegia. With higher-level injuries (C1 through
C4), total assistance is required. At lower levels of injury, with varying amounts
of adaptive equipment and assistance from others in some task components, the
person with quadriplegia can be a more active participant. It is extremely
important, however, for the person’s own goals and contexts to be
acknowledged. For example, if an individual with a complete C6 requires 60
minutes to dress with the use of adaptations, the question becomes, “Is this
functional?” If a person is attempting reentry into the work force or return to
school, is it “functional” to spend an entire hour, as well as all the physical
energy, in just getting dressed? Or is it a sign of greater autonomy to delegate
some tasks to a caregiver to allow participation in activities that are more
meaningful?
Secondary conditions aside, individuals with paraplegia are usually
independent in bathing and dressing. Some even have the strength to transfer to
the bottom of a tub without assistive devices.
Toileting
Managing altered bowel and bladder function are challenges for virtually
everyone with SCI. The two aspects to this challenge are the actual physiologic
management and the various techniques and equipment used in the toileting
process.
Medications may be required for physiologic management. Stool softeners
are used, as well as suppositories, to assist in evacuation. A goal of effective
bowel management is to eliminate or reduce reliance on medications. For people
with injuries at C7 or below, independence in toileting can usually be achieved
with an array of equipment that may include suppository inserters, digital
stimulators (devices that trigger reflexes to relax the rectal sphincter in UMN
injuries), catheterization devices, leg separators, mirrors, and adapted commode
chairs that allow access to the perianal area for bowel-training procedures.
Aside from medications and equipment, additional strategies include
maintaining a specific schedule for elimination, eating a healthy diet that
promotes regularity, assuming positions that facilitate elimination, and the use of
Credé’s method. If toileting appears to be tiring, then it has been portrayed
correctly! But many people with SCI become adept at its management. It may be
a very different picture, though, when a person attempts these procedures in a
community environment (i.e., restrooms at work or school), due to accessibility,
availability of equipment, and time limitations. In order to promote greater
freedom and independence, some individuals opt to explore alternatives—such
as undergoing surgical urinary diversion. Surgeries such as the Mitrofanoff
procedure create a permanent stoma in the abdomen, which can allow an
individual to catheterize through their navel. This presents benefits of easier
anatomical access and significantly less adjustment of clothing versus traditional
catheterizing. While not every individual is a candidate—and the procedure is
certainly not without risk and potential long-term complication— studies have
revealed that many who undertake it have been highly satisfied with the degree
of freedom and independence it has allowed them (Merenda, Duffy, & Betz,
2007).
The person with a higher-level cervical injury faces greater challenges.
Although the bowel and bladder management concepts are the same,
neuromuscular deficits limit performance of the tasks, even with adaptive
equipment. Generally, people with injuries above the C6 level require a caregiver
to assist with or perform functions such as transfers to adaptive commode chairs,
suppository insertion, digital stimulation, catheterization, and general perianal
care. The person with an SCI must indicate who the caregiver will be,
particularly for tasks that are socially sensitive. Even though family members
may be willing to assist, it is perfectly justifiable for the person to request
someone else as a caregiver. Some people may have no reservations about who
assists them, whereas others may feel strongly that it would negatively affect
established roles. Whatever the case, whenever feasible, the preferences of the
person with an SCI should be the deciding factor in selecting caregivers for
various tasks.
Personal Device Care
The extent of personal and adaptive devices required by an individual with an
SCI can generally be predicted by the level of injury (Table 24.4). Additional
complications, such as reduced ROM resulting from contractures, may require
use of more extensive devices than are typically seen at a particular level.
Usually, personal device care is performed by others for those with injuries at C4
and above. Those with injuries at the C5 level and below have progressively
more ability to assist in personal device care and use, but this fluctuates greatly
depending on the individual’s endurance, motivation, resources, and priorities.
Health Maintenance
Fostering a healthy lifestyle is critical, but often challenging, after an SCI. Most
people with an SCI are advised to follow lifelong exercise programs to preserve
and enhance ROM and strength, as well as to promote good cardiovascular
fitness and weight management. Many individuals report weight gains in the
months after injury, as their energy demands are greatly altered in great part by
use of a wheelchair for mobility. Attention also must be given to proper nutrition
for weight management, impact on skin integrity, and bowel and bladder
management. As mentioned earlier, routine attention to skin condition is crucial
to avoiding dermal complications. A health maintenance routine may require the
assistance of others, depending on the level of injury, for such activities as
setting up weights and equipment, performing passive ROM, pressure relief, and
aiding in skin inspection.
Socialization, Functional Communication, and
Emergency Response
With a singular diagnosis of SCI, no cognitive deficits are inherent that would
preclude a person from socializing in an appropriate contextual manner. What is
challenging, though, are the variety of barriers that may inhibit socialization.
Architectural, environmental, and transportation barriers; reduced endurance;
and increased reliance on others may discourage or actually prevent someone
from traveling to the places where they socialized before their injury.
Psychological barriers also may prevent reintegration into a premorbid social
support system, and these “barriers” are as real as physical ones.
In all but the highest of injuries, verbal communication is functional. People
with injuries at C7 or below are generally independent with a variety of forms of
communication (e.g., writing, keyboard use, phone use) without the use of
adaptive equipment. Above this level, however, adaptive devices are
progressively required.
The need to request assistance in emergencies is possible at virtually all
levels of injury, depending on the environment and adaptive equipment
available. For people with higher-level injuries, adaptive phone devices,
emergency call systems, and environmental control units make contact with
emergency agencies feasible. At the level of C4 and above, however, the
availability of a caregiver 24 hours a day remains the most appropriate safety
option. Individuals with injuries below this level may use a phone independently
with possible adaptations but may be limited in other emergency responses, such
as exiting a dwelling or attending to the physical needs of another injured
person.
Functional Mobility
People with complete injuries at the thoracic levels of injury and above usually
rely on wheelchairs for household and community mobility. Those with injuries
at C6 or above may require additional assistance in manual wheelchair
management; they may utilize a manual wheelchair with power-assist wheels, or
a power wheelchair. Individuals with higher-level quadriplegia may use adapted
orthotic devices to aid in controlling a power wheelchair, or alternative interfaces
such as “puff and sip” or chin controls to maneuver the wheelchair in the
absence of sufficient upper extremity function. Even at lower levels of injury,
while an individual may be able to navigate a manual wheelchair under certain
conditions, power wheelchairs may be considered due to community access
needs (e.g., the need to navigate a large college campus routinely). A person with
a lower level thoracic injury may be able to ambulate short distances with an
ambulation device such as a walker or Lofstrand (forearm) crutch and lower
extremity orthotic devices; however, the practicality of this in household and
community settings must be evaluated. It is important to consider the need to
avoid repetitive motion disorders in the upper extremities, which can become
problematic if overused during ambulation activities. People with sacral injuries
often can ambulate community distances without orthoses or devices.
Sometimes it is not so much the ability or inability of the person with an SCI
but the inaccessibility of the environment that limits functional mobility.
Whereas the home environment can be modified through creative thought,
planning, and finances, the community environment is much harder to change.
Although the Americans with Disabilities Act celebrated its 20th anniversary of
enactment on July 26, 2010, most would agree that compliance with and
enforcement of barrier-free environments are less than ideal. Many physically
challenged people continue to fight discriminatory situations (e.g., inaccessible
public transportation, restaurants, offices, classrooms). Ed Roberts, founder of
the first Center for Independent Living, recalled a lighter side of the
inaccessibility issue. He reminisced that early protestors in the disability rights
movement were released from police custody because jail cells were
inaccessible! (Price, 1990) In the vast majority of situations, however, an
inaccessible environment is frustrating, demeaning, and personally violating.
The health care team can do its part by helping physically, emotionally, or
cognitively challenged people to be aware of their rights, as well as by providing
them with information on advocacy groups.
Functional mobility includes driving as a consideration for many. A C5-
injured person may be able to drive with specially adapted low-effort steering,
braking, and acceleration hand controls. Usually, those with complete injuries
above the C5 level cannot drive because of physical and respiratory limitations.
People with C6 injuries and below may be able to drive independently using
hand controls and adaptive steering devices. A van with a power lift or ramp
may be recommended for someone who has difficulty transferring from his or
her wheelchair or independently stowing the wheelchair in a vehicle. Often,
finances more than need may dictate the type of transportation used, and
clinicians can be invaluable in helping clients identify what financial and
community resources may be available to help them meet their mobility needs.
Sexual Expression
As with bowel and bladder function, most people with SCI experience
alterations in their ability to perform sexually as compared with their premorbid
status. The nerves that innervate the genital area (both motor and sensory
components) originate at the sacral spinal cord levels, so in the great majority of
SCI cases, sexual function is affected.
For the most part, a person can participate in a variety of sexual activities
despite SCI, although the level of sensation and motor response will vary,
depending on the extent and level of injury. Specialists may recommend
medications, surgical implants, and sexual enhancement devices, but this is
highly individualized.
Advances in the field of fertility have improved the chances for a spinal
cord–injured male to father a child. Although fertility rates for men with SCI are
estimated at <10%, techniques such as electrostimulation to induce ejaculation in
paraplegics have proven successful in many cases (Christopher & Dana Reeve
Foundation, 2015a). Men may be well advised to delay a decision about surgical
penile implants until at least 1 year after injury. This allows time to evaluate the
full impact of the SCI on sexual function and to determine the extent, if any, of
sensory or motor return in an incomplete injury. The reproductive capabilities of
women are generally unaltered by an SCI, and women and their partners should
be made aware that the potential for pregnancy exists (Spinal Outreach Team,
2014).
Addressing sexual function should be an integral part of each person’s
treatment plan, but a distinction should be made between purely physiological
sexual performance—arousal, orgasm, and ejaculation—and sexuality, which is
the totality of a person’s attractiveness, personality, and self-perception as a
sexual being. Sexuality does not have to rely on physiology. Whereas
performance may be hampered as a result of an SCI, one’s sexuality can be quite
healthy and intact. The health care provider must make accurate information
available and identify sources of more detailed information or expertise. It is
entirely at the discretion of the person with an SCI to explore, or not explore,
options available to them.
Home Maintenance
Tasks such as household maintenance, meal preparation, shopping, cleaning,
clothing care, and safety procedures are included within the general category of
home maintenance. Usually, people with a C6-level injury or above require
assistance with all of these activities and need some personal attendant care.
People with injuries at C7 or lower can often live independently (without
attendant care) as they are able to perform their basic ADL tasks in accessible
environments. However, they typically require assistance with heavier home
maintenance tasks or activities requiring access to high or low areas.
Care of Others
An SCI can certainly make caring for others difficult, particularly if the injured
person had been the primary caregiver for a child, spouse, or parent. Although a
major goal of those with SCI is mastering personal self-care, a concurrent goal
may be the introduction of activities (e.g., diapering, bathing a child) that can
allow them to assume some premorbid roles. Often, many of these previous
responsibilities must be delegated to others. In these cases, it is ideal if the
person with SCI retains the responsibility for the verbal direction of care.
Education
People with SCI can generally resume educational activities, even while still
inpatients in rehabilitation facilities. Many specialty hospitals retain the services
of teachers from local school districts—or encourage the involvement of a
student’s premorbid teachers—and educational instruction is often scheduled
along with other therapies for elementary, secondary, and high school students,
encouraging smoother reintegration after discharge. The length of hospital
admission following an SCI has decreased dramatically in the nearly three
decades since more reliable statistics have been available; currently, the average
length of combined acute/inpatient rehabilitation hospitalization following an
SCI is 49 days (http://fscip.org//facts.htm). Certainly, a variety of factors can
impact the length of hospitalization (e.g., level of injury, secondary diagnoses,
complications, availability of specialty hospital resources), but the overall trend
toward reduced inpatient hospitalization allows for individuals to reintegrate
back into their premorbid roles and support systems much more rapidly than in
the past. There are certainly advantages—but some inherent concerns—for
children and adolescents returning more quickly to their previous school
environments after the significant life changes brought on by an SCI. The
ongoing support of the health care team can be critical in promoting successful
reintegration.
College students would most likely be able to resume their studies following
an SCI. Depending on their level of injury, however, people may reevaluate their
course of study to prepare for a more feasible career.
Adaptive writing devices, page turners, recording devices, and computers
have made returning to the classroom less intimidating. Laws have also
improved the accessibility of public buildings. It is challenging, though, for a
student with an SCI to manage bowel and bladder schedules, adjustment of
clothing, eating devices, and so forth, but with planning and the assistance of
others if needed, many individuals have successfully returned to the classroom.
Vocational
It is appropriate for a person with any level of SCI to begin to formulate
vocational options, even as an inpatient, with the members of the health care
team. Individual situations are so variable—premorbid occupation, level of
injury, educational level, other vocational interests, family support, cognitive
abilities, motivation, financial resources—making it impossible to say whether a
person with a certain level of SCI can or cannot be gainfully employed.
However, legislation mandates that work sites be accessible within reason. Also,
many employers recognize the importance of a trained employee and will make
additional accommodations to return a valued person to the workplace.
As we move from an industrial to an information society, the job market will
continue to change, with positions requiring less physical labor than in the past.
Job requirements will increasingly demand analytical thought, problem solving,
and creativity, all of which are certainly intact after an SCI!
Leisure Activities
For most people, leisure pursuits are an integral part of a meaningful life.
Although an SCI can alter the way in which one participates in leisure activities,
it does not have to change the intensity of participation.
Sports, both individual and team, are excellent leisure pursuits that are
growing in popularity for people with SCI. Virtually any sport can be
undertaken, from basketball to tennis to archery. Adaptive equipment and
modified regulations help make some sports more feasible for people with an
SCI, and these modifications should not be viewed as detracting from the
competitiveness of the sport. Consider that athletes in the general population use
“adaptive” equipment all the time. How long would a catcher last in a baseball
game without a mitt or a mask? Adaptive equipment need not detract from the
legitimacy of the contest. It is heartening to see that, even internationally, the
wheelchair athlete is recognized for excellence, with designated events in the
Olympic games. People with an SCI who want to participate have numerous
avenues open to them.
Aside from sports, opportunities for social activities from square dancing to
traveling abound. Travel agencies and tour groups have recognized the market
created by the wheelchair traveler and have responded. Most hospitals with
specialized SCI rehabilitation units have well-established programs that help
people get involved with special interest groups. Often, during the acute phase of
SCI, people cannot envision themselves participating again in the things they
enjoy. The health care professional must be available for these individuals to
encourage renewed interest in favorite leisure activities.
Several resources related to leisure activities, as well as other issues, have
been discussed in this chapter. The reader is encouraged to consult the references
and suggested readings at the end of this chapter for more information.
Additionally, Table 24.4 gives an overview of specific expected outcomes for
each level of SCI.
CASE-STUDY 1
M.L. is a 24-year-old woman who sustained an SCI during a motor vehicle
accident while on her honeymoon. Her husband was thrown from the
vehicle but received only minor injuries. M.L. was transported by
emergency medical services (EMS) to the local emergency department. She
was diagnosed with a C5 through C6 vertebral subluxation and a C6 crush
injury, resulting in a complete (ASIA-A) C6 SCI. She also sustained a left
clavicular fracture. She received nasal O2 for respiratory support. Once
stabilized, she was transferred to the specialized trauma center near her
home. A halo vest was applied, which stabilized her cervical spine. No
operative procedures were indicated at that time. After 22 days, her
endurance improved so that she could tolerate sitting upright in a chair for
up to 1 hour. She was transferred to the nearby rehabilitation facility’s SCI
unit. During her 12 weeks there, the only complications she experienced
were two episodes of autonomic hyperreflexia (apparently secondary to
hard stool in the lower rectum) and a mild UTI. Spasticity developed in her
wrists, elbows, and lower extremities.
Before her injury, M.L. was a recently graduated college student with a
liberal arts degree. She and her new husband had recently signed a 1-year
lease on an upstairs apartment close to the university, as she was
anticipating pursuing graduate studies. She had been totally independent in
all of her ADL and home management activities. Her leisure pursuits
included recreational team sports (particularly softball) and more sedentary
activities like reading and gourmet cooking. She was also involved with her
family, particularly two sisters and her parents, who live close by.
Many body functions are significantly affected by M.L.’s injury. Her
sensorimotor deficits are consistent with those anticipated for a C6
complete quadriplegia. M.L. is challenged by the
psychosocial/psychological issues facing her as a result of her SCI. She
feels that her role has changed significantly, especially in her relationship
with her husband. He has been willing to assist her in those activities in
which she is physically limited; however, his assistance in some activities—
particularly bowel management—has been difficult for her to accept. This
has been a source of frustration for her, and she has discussed with him the
possibility of hiring an attendant on a limited basis to assist with specific
activities. He resists this idea, stating that it is his desire and duty to care for
her. He took a temporary leave of absence from the family-owned
landscaping company where he is employed when she was discharged 2
weeks ago. There is no definite timetable for his return. M.L. also is
concerned about her ability to express herself sexually, as well as her
potential to have children in the future. She attended classroom sessions on
these topics in the rehabilitation facility, but she did not seek any individual
counseling. M.L. states that she probably wasn’t ready to hear anything
specific then, but she now wishes she had someone to whom she could ask
questions.
M.L. has not begun to consider her work activities or return to school.
Even before her accident, she had been undecided about a career path. She
has expressed interest, however, in exploring possible alternatives.
M.L. has stated that eventually, she might like to get involved in team
sports again. In the 2 weeks since her discharge, though, her main leisure
pursuits have been reading and watching television.
CASE-STUDY 2
H. B. is a 6-year-old boy who sustained an incomplete (Brown-Séquard’s)
SCI, as the result of being struck by a stray bullet during a drive-by
shooting in early August. He did not lose consciousness during transport to
the emergency department. It was determined radiologically that the bullet,
which entered from the right near the base of his neck/upper back, was
lodged in the C7/C8 cervical spinal canal, and surgery was performed to
remove the bullet soon after admission. H.B. is now 2 weeks postinjury and
exhibits a loss of voluntary movement, reduced touch, and proprioceptive
sensation on the right below the level of C7. He exhibits reduced pain,
thermal, and some reduction in touch awareness on the left below the level
of C7. The levels of C7 and above appear intact for both motor and sensory
function. H.B. has had an uncomplicated hospital admission at this time. It
is anticipated that he will remain in the acute children’s specialty hospital
for at least another 2 weeks and then transfer to the inpatient pediatric
rehabilitation unit.
At the time of his injury, H.B. was living with his maternal
grandmother, who is also his legal guardian. H.B. had been cocaine positive
at birth, and his biological mother had relinquished her parental rights
shortly after he was born. H.B.’s biological father is unknown. H.B.’s
grandmother is also caring for another of H.B.’s siblings, an 8-year-old
sister. H.B.’s grandmother reports that prior to his injury, he did not appear
to have any overt behavioral/cognitive challenges and had successfully
completed kindergarten. She related that he was a “normal, active boy” and
enjoyed playing any type of outdoor games, as well as watching television.
She states he did not have much exposure to computers or video games, as
the family did not own these items. She states he had been looking forward
to starting first grade, which is scheduled to begin in his district next week.
H.B. has been very cooperative with all of the health care providers
working with him; he appears to enjoy the attention he is receiving from the
staff, as well as the visitors from the neighborhood, his grandmother’s
church, and police department. His grandmother has expressed many
concerns to the health care professionals working with H.B. Her overriding
concern is fear that she will be unable to provide sufficient care— both
from a physical and financial perspective—for H.B. following his inpatient
rehabilitation stay. She related that the family residence is an older, rented
duplex, with seven steps leading up to the front porch. She stated the
interior of the residence is not conducive to any type of mobility devices, as
the hallways and doorways are narrow, and all the bedrooms are on the
second story of the unit. She also states concern about how he will be
treated when he eventually starts school; although his older sister is in the
same elementary school, H.B.’s grandmother states she has had very limited
contact with teachers/administrators at the school and has no idea what
resources are available to help him. She expressed frustration that H.B.’s
shooter has not been apprehended and shared feelings of hopelessness and
helplessness in her ability to protect H.B. and his sister from harm in the
future.
RESOURCES
Readings
Americans with Disabilities Act. Retrieved from http://www.ada.gov
Blackwell, T. L. (2001). Spinal cord injury desk reference: Guidelines for life
care planning and case management. New York, NY: Demos Medical Pub.
Fehlings, M. G., Vaccaro, A. R., Boakye, M., Rossignol, S., Ditunno, J. F., &
Burns, A. S. (2013). Essentials of spinal cord injury: Basic research to clinical
practice. New York, NY: Thieme Medical Publishers.
Field-Fote, E. C. (2009). Spinal cord injury rehabilitation. Philadelphia, PA: F.A.
Davis Co.
Hall, M. (2012). Across the street from hell: My spinal cord injury recovery.
Mark Anthony Hall.
Kirshblum, S., & Waring, W., III. (2014). Updates for the international standards
for neurological classification of spinal cord injury. Physical Medicine &
Rehabilitation Clinics of North America, 25(3), 505–517, vii. doi:
10.1016/j.pmr.2014.04.001
Mayo Clinic. (2009). Mayo clinic guide to living with a spinal cord injury. New
York, NY: Demos Health.
National Institute of Neurological Disorders and Stroke. Retrieved from
http://www.ninds.nih.gov/
Ngonyani, J. B. (2008). Living with spinal cord injury disability. Dar es Salaam,
Tanzania: Peramiho Printing Press.
Ozer, M. N. (1988). The management of people with spinal cord injury. New
York, NY: Demos Publications.
Palmer, S., Kriegsman, K. H., & Palmer, J. B. (2008). A guide for living: Spinal
cord injury (2nd ed.). Baltimore, MD: The Johns Hopkins University Press.
Pennsylvania Bar Institute. (2015). The catastrophic injury case: Quadriplegia,
paraplegia & spinal injury cases. Mechanicsburg, PA: Pennsylvania Bar
Institute.
Selzer, M. E., & Dobkin, B. H. (2008). Spinal cord injury. New York, NY:
Demos Medical Pub.; AAN Press, American Academy of Neurology.
Senelick, R. C., & Dougherty, K. (1998). The spinal cord injury handbook for
patients and their families. Birmingham, AL: HealthSouth Press.
Simonds, A. K. (2007). Non-invasive respiratory support: a practical handbook
(3rd ed.). London, UK: Hodder Arnold.
Somers, M. F. (2010). Spinal cord injury: Functional rehabilitation (3rd ed.).
Upper Saddle River, NJ: Pearson.
Sutton, A. L. (2011). Disabilities sourcebook: Basic consumer health
information about disabilities that affect the body, mind, and senses (2nd ed.).
Detroit, MI: Omnigraphics.
Tabak, H. (2005). No whining. Craig hospital spinal injury rehab: Reaching new
heights. Lincoln, NE: iUniverse Inc.
Patient/Family Resources
Alpert, M. J., & Wisnia, S. (2008). Spinal cord injury and the family: A new
guide. Cambridge, MA: Harvard University Press; Christopher & Dana Reeve
Foundation. Retrieved from
http://www.christopherreeve.org/site/c.ddJFKRNoFiG/b.4048063/k.67BA/The_Christopher_a
Jefferson University Hospitals. (2009). Spinal cord injury: Patient family
teaching manual. Retrieved from
http://www.spinalcordcenter.org/consumer/manual.html
Neville, J. B. (2012). How I roll: Life, work, and love after a spinal cord injury.
Pennsylvania, PA: Winans Kuenstler Publishing, LLC.
Spinal Cord Injury Information Network. Retrieved from
http://www.uab.edu/medicine/sci/
The Miami Project to Cure Paralysis. University of Miami School of Medicine.
P.O. Box 016960, Mail Locator R-48, Miami, FL 33101. Retrieved from
www.miamiproject.miami.edu
General Neuroanatomy Reviews
Gertz, S. D., & Tadmor, R. (2007). Liebman’s neuroanatomy made easy and
understandable (7th ed.). Austin, TX: PRO-ED.
Kiernan, J. A., & Barr, M. L. (2009). Barr’s the human nervous system: An
anatomical viewpoint (9th ed.). Philadelphia, PA: Wolters Kluwer
Health/Lippincott Williams & Wilkins.
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C H A P T E R
25 Orthopedics
Nancy Hock Heather Javaherian-Dysinger Sharon L.
Pavlovich
KEY TERMS
Arthroplasty Closed fracture Comminuted fracture Complex regional pain
syndrome (CRPS) Compound fracture Delayed union Ecchymosis Greenstick
fracture Heterotopic ossification Malunion Nonunion Open fracture Open
reduction internal fixation (ORIF) Osteoarthritis Osteopenia Osteoporosis
Pathologic fracture Remodeling Rheumatoid arthritis Volkmann’s deformity
Mary is a 68-year-old widow who lives alone in a small two-story home. Though
obese and managing diabetes, she is very active. She volunteers at her church
and the community hospital. She loves to garden, walk, play cards with her
friends, and do crossword puzzles. At home, she cooks simple meals and does
light housework as the pain in her hip and hands makes it too difficult to carry
out the activities that she did in the past. Mary was an avid square dancer, but
with the hip pain and loss of her husband, she gave up dancing, a valued
occupation.
After much encouragement from her friends and children, Mary finally went
to the doctor and told him about the severity of her pain. After a few tests, he
diagnosed her with arthritis. She started anti-inflammatory medication to manage
her pain and discomfort. Over the past year, however, her right hip became more
and more painful. She had difficulty getting out of bed in the morning and had to
ask her children to help with the housework and gardening. She cried on the
phone as she told her daughter, “I can’t do anything anymore.” She missed being
out in her garden and tending to the plants as it gave her peace and helped her
feel connected to the memories of her husband. She also missed socializing,
walking, and playing cards with her friends. When they came over to check on
her, they encouraged her to talk to her doctor again.
After seeing her doctor, who confirmed that she had severe arthritis in her
hip and degeneration, Mary chose to have an elective total hip replacement
(THR). Plain radiographs obtained from the right hip revealed severe arthritic
changes. Her physician thoroughly discussed the benefits and risks of the
surgery with her. She was seen for preassessment training, which involved
meeting with several members of the orthopedic total joint team. During this
time, she met with both an occupational and physical therapist. The occupational
therapist asked her several questions about her postoperative goals, the design of
her home, her family support, and the activities in which she liked to engage.
The occupational therapist explained that she would receive occupational
therapy after the surgery to help her regain independence in daily activities while
safely following her hip precautions. The occupational therapist told her that it
would take about 6 weeks to recover and that she should be walking with her
friends, meeting them for card night, working in the garden with possible
modifications, and, most importantly, enjoying life again.
Orthopedic conditions involve injury and disease of bones, joints, and their
related structures, which include ligaments, tendons, and muscles. The severity
of the injury determines the extent of involvement of those supporting structures.
Orthopedic conditions may be caused through a variety of circumstances and
disease including traumatic injury, motor vehicular accident (MVA), or arthritis.
Injuries during sports such as snowboarding, leisure activities including hiking,
and unexpected falls that happen while going about one’s day contribute to many
of the conditions we see in occupational therapy. Orthopedic conditions may also
be caused by rheumatic diseases such as osteoarthritis and rheumatoid
arthritis as well as osteoporosis. Osteoarthritis is a result of wear and tear
causing joint damage, whereas rheumatoid arthritis is an autoimmune disorder.
Osteoporosis is caused by a decrease or loss in bone density. As a result of these
diseases, people may need a joint replacement or arthroplasty to promote
participation in daily activities and to help improve their quality of life with
severe arthritis.
One of most common orthopedic conditions is a fracture, which is a break in
the continuity of the bone. When one considers the forces involved in an injury
such as the impact to the wrist as an older woman tries to protect herself from
falling, or from the force of a door crushing against a finger, it is easy to
understand that the ligaments and tendons surrounding the involved joint or bone
are often injured as well. Though this is common in orthopedic conditions, the
focus of this chapter is on conditions related directly to bones and joints.
Etiology
Fractures
Fractures are caused by a trauma or disease of the bone or joint. There are two
critical factors involved in the determination of a fracture: (1) the amount of
force applied to the bone and (2) the strength of the bone (Kunkler, 2002).
Forces can be high energy such as those experienced in a motor vehicle accident
(MVA) or low energy such as those experienced in a fall or through chronic
stress as seen in long-distance running. Consequently, these are called stress
fractures. The second determining factor is bone strength, which may be normal
or weakened from pathological conditions such as tumors or osteoarthritis and
osteoporosis. In addition, the age of a person and the size of the bone further
influence the bones’ ability to withstand a force. These factors negatively affect
bone density, elasticity, and supportive structures, thus making the bone more
vulnerable to fracture. For example, an older adult who has decreased bone
density from osteoporosis has a weak bone that may be unable to sustain normal
forces experienced during daily activities. Thus, the bone may fracture while the
person simply bends over or gets out of bed. This type of fracture is called a
pathologic fracture as the fracture was caused by weakening of the bone from
another disease. Osteoarthritis and osteoporosis are common conditions that
contribute to a pathologic fracture.
Osteoarthritis
Osteoarthritis, also referred to as degenerative joint disease, is a
noninflammatory joint disease that results in deterioration of articular cartilage
and the formation of new bone or osteophytes on the joint surface. These
changes often result in pain, joint edema, and impaired participation in life
activities. Osteoarthritis is the most common joint disease in the upper extremity.
Osteoarthritis of the knee tends to cause the most disability as it impacts one’s
ability to walk, go up and downstairs, and engage in leisure and work activities.
As a result of osteoarthritis, many individuals seek elective surgery for hip and
knee replacements to return to a more active and pain-free lifestyle. In the
United States, approximately 719,000 total hip and 332,000 knee replacements
are performed annually (CDC, 2015b). This number is expected to increase with
the aging of the population (National Institute of Arthritis and Musculoskeletal
and Skin Diseases, 2009). The technology of joint replacements continues to
evolve making them essential in promoting participation in daily activities and
improving quality of life in people with severe arthritis.
Other Orthopedic Conditions
Osteoporosis is a disease characterized by low bone density and deterioration of
bone. It is common in postmenopausal women due to the cessation of estrogen
production. The National Osteoporosis Foundation (2010) estimates that over 10
million people in the United States have osteoporosis. A person with
osteoporosis may fracture a bone through normal movement such as turning to
reach an item of clothing when getting dressed in the morning. The most
common sites for these fractures according to data gathered in 2005 are the
vertebrae, the wrist, the hip, and the pelvis with approximately 547,000 vertebral
fractures, 397,000 wrist fractures, and approximately 297,000 hip fractures
(National Osteoporosis Foundation, 2010). It is estimated that half of all women
and as many as one out of four men aged 50 and older will experience a fracture
related to osteoporosis (US Dept. of Health and Human Services, 2004).
Osteopenia is a reversible weakening of the bone. It is estimated that nearly
34 million people have low bone mass (National Osteoporosis Foundation,
2010). As osteopenia is a reversible condition, it is important to take advantage
of bone density screenings offered at health fairs and follow up with your
physician. Measures to help decrease the risk of osteopenia and halt further
progression into osteoporosis include a balanced diet, supplements, weight-
bearing exercise, and bone density screenings.
Heterotopic ossification (HO) is an orthopedic condition resulting in
abnormal bone formation in extraskeletal soft tissues (Moore & Cho, 2010). This
condition is often associated with traumatic injuries including severe burns,
spinal cord injuries, and head injuries as well as other bone-forming diseases
such as ankylosing spondylitis (Moore & Cho, 2010). The exact cause and
pathophysiology of HO is still unclear. The massive trauma sustained by the
body creates a whole-body response, and in some instances, fibroblasts
inappropriately start forming bone. This results in excessive bone growth near
joints causing stiffening and loss of movement. This painful and debilitating
condition is still not clearly understood, but it can have a significant impact on a
person’s functional abilities.
Fractures
In 2007, the National Center for Injury Prevention (NCIP) reported unintentional
falls as the leading cause of nonfatal injuries in the United States across all age
groups. The most common fractures that result from falls are those of the spine,
hip, forearm, leg, ankle, pelvis, upper arm, and hand (Scheffer, Schuurmans,
VanDijk, & VanDerHoof, 2008). Chung and Spilson (2001), following review of
nearly 1.5 million fractures, determined that 44% of all fractures involve the
distal radius. Falls are the leading cause of fractures among older adults (Bell,
Talbot-Stern, & Hennessy, 2000). In 2001, more than 1.6 million older adults
were treated for fall-related injuries, which included fractures and other
conditions. The 2000 census reported that there are 360,000 to 480,000 fall-
related fractures in older adults per year involving the hip, wrist, humerus,
vertebrae, and pelvis, with the most serious being a hip fracture (National Center
for Injury Prevention and Control, 2004). Older adult women sustain
approximately 80% of all hip fractures (Stevens & Olson, 2000). Population
studies project, with the rising number of older adults, a significant increase in
rates of hip fractures, which are expected to reach 289,000 annually by 2030.
This is an increase of 12%. Fortunately, the hip fracture rate declined
significantly from 1996 to 2010 (CDC, 2015a). Hip fractures, however, continue
to be a significant condition contributing to long-term functional impairment as
well as increased nursing home admissions (CDC, 2015a).
Hip fractures are one of the primary causes of disability and mortality in the
older adult. Statistics suggest that one in five patients dies within the first year of
sustaining a hip fracture (Farahmand, Michaelsson, Ahlbom, Ljunghall, &
Baron, 2005). Some people will not return to their premorbid functional level
and may rely on adaptive equipment or modifications to their daily activities
(Crottty et al., 2010). A randomized trial by Hagsten, Svensson, and Gardulf
(2004), however, found that occupational therapy intervention increased a
patient’s ability to perform activities of daily living (ADL) sooner than those
who did not receive occupational therapy. Thus, patients receiving occupational
therapy were more likely to return to an independent living situation and require
less postoperative care. This charges occupational therapy practitioners with the
role of promoting life participation and well-being among individuals who are
recovering from fractures as well as other orthopedic conditions.
Osteoarthritis
Arthritis is one of the leading causes of disability impeding people’s capacity to
engage in meaningful and necessary daily occupations due to pain, weakness,
and loss of range of motion (ROM) (Healthy People 2010, 2010). According to
the Centers for Disease Control and Prevention (2013), 52.5 million people have
arthritis worldwide. In the United States alone, it is estimated that 27 million
people age 25 and older have osteoarthritis (Lawrence et al., 2008). Arthritis was
also found to result in estimated $128 billion in medical care costs and lost
earning in the United States in 2003 (CDC, 2013). Osteoarthritis typically affects
the knee, distal interphalangeal joints, proximal interphalangeal joints, and the
first carpal metacarpal joint.
According to the National Osteoporosis Foundation, 10 million people have
osteoporosis, of which 80% are women (National Osteoporosis Foundation,
2010). Early signs of osteoporosis are seen in younger women in the form of
osteopenia. This trend emphasizes the importance of prevention and lifestyle
management as once the disease progresses beyond osteopenia to osteoporosis,
bone regeneration is no longer reversible. Nguyen, Eisman, Center, and Nguyen
(2007) identified that postural instability, quadriceps weakness, and a history of a
fall or previous fracture are shown to be significant predictors of future
osteoporotic fractures.
HO is a common complication after traumas such as fractures, arthroplasties,
traumatic amputations, spinal cord injuries, head injuries, and burns with the
incidence varying depending on the injury (Wheeless, 2015b). Though it is a
common complication, only 10% to 20% of people who develop HO will have
significant functional limitations (Bruno-Petrina, 2008). Most people will be
asymptomatic and most likely diagnosed when it is seen on an x-ray. HO usually
occurs 1 to 4 months after the traumatic injury (Wheeless, 2015b). HO may
occur after a fracture especially in those who have an open reduction internal
fixation (Bruno-Petrina, 2008). In injuries and fractures to the elbow, the
incidence may be as high as 90% (Bruno-Petrina, 2008). The incidence of HO in
hip arthroplasties is generally 50%, although only one-third of the people
experience clinically significant symptoms such as limited ROM and pain
(Kocic, Lazovic, Mitkovic, & Djokic, 2010). Revisions to hip and knee
replacements increase the risk of developing HO (Moore & Cho, 2010). With the
increase in combat-related injuries over the last several years, we have seen
more soldiers with traumatic amputations. The incidence of HO in these
amputations was found to be 63%, which is much higher than previously
expected (Potter, Burns, Lacap, Granville, & Gajewski, 2007). Fortunately, the
majority of the cases are successfully managed or the soldiers are asymptomatic.
HO occurs in 40% to 50% of people who have a spinal cord injury, with it
primarily affecting the knee, pelvis, and hip (Moore & Cho, 2010). Research
suggests it also occurs in 20% of people who have a traumatic brain injury
(Bruno-Petrina, 2008). The incidence of HO appears to be much higher in
fractures and neurological injuries involving spasticity than it does in burns.
Factors that can impact occurrence of HO in burns are found to be the
percentage and location of the burn, the amount of forceful manipulation, and
the time period of immobilization and time for wound closure (Klein, Logsetty,
& Costa, 2007).
Signs and Symptoms
Fractures
The general symptoms of a break are localized pain at the fracture site,
deformity, edema, and ecchymosis, which is often seen after 24 to 48 hours of
onset (Altizer, 2002). Ecchymosis is described as a discoloration of the skin that
is caused by bleeding. There are several types of fractures. A closed fracture
refers to a fracture that has not broken through the skin, whereas in an open
fracture, the bone breaks through the skin surface. Open fractures, also known
as compound fractures, have an increased chance for infection. The term
comminuted is applied to fractures that have two or more fragments. A
displaced fracture involves segments that have become separated or shifted from
the bone (Altizer, 2002). Greenstick fractures are often seen in children whose
bones are still soft and growing. Rather than snapping into two, the bone breaks
on one side and bends on the other. It is similar to how a young twig or tree limb
breaks, thus the name greenstick fracture.
Fractures are further described by the type of fracture line. A complete
fracture involves a break in the full continuity of the bone. An incomplete
fracture involves a partial disruption in the continuity of the bone. Such breaks
are often called cracks or hairline fractures (Altizer, 2002). A transverse fracture
occurs when the fracture line is at a right angle to the longitudinal axis of the
bone. An oblique fracture involves a fracture line that is diagonal or slanted.
Torsional stress applied to bone causes a twisting fracture line, which is called a
spiral fracture.
Distal Radial Fractures

Distal radial fractures may cause loss of sensation, strength, movement, and
limited functional use of the hand and possibly the arm. As most of these injuries
are caused by a fall on an outstretched arm, the entire upper extremity should be
examined for injury. Several different classification systems have been used to
describe distal radius fractures. These systems are used to identify fracture
fragment patterns and lead the surgeon’s management decisions (Michlovitz &
Festa, 2011). Physicians evaluate distal radius fractures based on volar or dorsal
displacement, comminution of the fracture, whether there is articular
involvement or involvement of the ulna, or whether there are additional soft
tissue injuries associated with the fracture (Chen, Huang, Trumble, & Jupiter,
2010).
Hip Fractures
A hip fracture generally refers to a fracture of the proximal femur. It may be
intracapsular or extracapsular. Intracapsular fractures involve the femoral neck
such as a subcapital or transcervical fracture (Fig. 25.1). Extracapsular fractures
involve the trochanters such as a subtrochanteric or intertrochanteric fracture. An
individual with a hip fracture may experience referred pain to the knee, be
unable to bear weight on the involved lower extremity, and will often have a leg-
length discrepancy.
Figure 25.1 Lower extremity, anterior view.
Humeral Fractures
The incidence of humeral fractures in the United States has increased as a result
of the aging of our population, increased participation in sports, and the rise of
osteoporosis (Gill, 2002). A humeral fracture may present with humeral
displacement and malposition of the distal limb. Radial nerve injury is found in
approximately 18% of humeral shaft fractures (Wheeless, 2015a). A spiral
fracture may lacerate the radial nerve, or it may be damaged by displaced bony
segments. Clinical signs of radial nerve involvement include loss of wrist
extension and impaired sensation on the dorsal aspect of the wrist. Seventy-five
to ninety percent of individuals who have closed humeral fractures have
recovery of the radial nerve in 3 to 4 months (Wheeless, 2015a).
Fractures that occur at the distal end of the humerus just above the medial
and lateral condyles are referred to as supracondylar fractures. These fractures
are commonly known as elbow fractures. The medial and lateral condyles serve
as an origin site for many muscles of the forearm and have bony connections to
the radius and ulna of the forearm. Nerves and arteries pass along this area;
therefore, a supracondylar fracture can also result in injury to muscles, nerves,
and tissue that further affect the function of the entire arm. Understandably, it is
associated with a high risk for complications such as malunion and Volkmann’s
deformity. Volkmann’s deformity is a condition that results from severe damage
to tissues and muscles caused by increased pressure in the forearm
compartments (Kare, 2008). Increased compartmental pressures may be caused
by several factors such as ischemia, compartmental bleeding, and excessively
tight bandages (Kare, 2008). Signs of ischemia include pale or bluish skin,
absence of radial pulse, decreased sensation, and severe pain. Suspected cases of
increased compartmental pressure should be immediately reported to the
physician as it can result in nerve damage and necrosis.
Scaphoid Fractures
The position of the scaphoid in the proximal row of the carpal bones makes it
particularly vulnerable to injury. It is the most commonly fractured bone in the
wrist with an incidence of 35,000 to 50,000 fractures annually (Dell, Dell, &
Griggs, 2011). Scaphoid fractures are commonly seen in young males with a
sports injury resulting in wrist hyperextension >90 degrees with radial deviation.
Fractures of the middle and proximal poles of the scaphoid are susceptible to
avascular necrosis because of its poor blood supply (Dell et al., 2011). Plain
radiographs are commonly used to classify the scaphoid fracture; however, other
radiographic procedures may be necessary to evaluate its healing.
Arthritis
There are other conditions in addition to fractures that affect the integrity of the
bone, such as rheumatic diseases. Rheumatic diseases include an array of
progressive conditions leading to impairments in joints and soft tissue. Examples
include osteoarthritis and rheumatoid arthritis. Rheumatic diseases are closely
associated with the term arthritis, which means joint inflammation. Arthritis is a
general term used to describe a host of conditions characterized by joint pain,
redness, swelling, and stiffness (Deshaies, 2006).
Osteoarthritis symptoms build up gradually and often begin with notable
aches with movement especially after inactivity. Osteoarthritis is often
characterized by joint pain, inflammation, stiffness, tenderness, limited ROM,
and crepitus, which is an audible or palpable crunching or popping in the joint
caused by the irregularity of opposing cartilage surfaces (Hochberg as cited in
Deshaies, 2006).

Osteoporosis is gradual and somewhat silent as it emerges. In the early stages of
osteoporosis, symptoms are not usually present. Over time, however, advanced
symptoms present themselves through pain, height loss, and kyphosis. The
primary clinical signs include skeletal fractures and recurring pathological
fractures (Dal Bello-Haas, 2009). Osteopenia is a predecessor to osteoporosis.
Even more silent, there are no clinical signs or presentation for osteopenia.
HO usually begins with pain, joint warmth, swelling, and decreased ROM
approximately 1 to 4 months after an injury (Adler, 2006). There may be a
palpable mass, which becomes harder as the bone forms.
The prognosis and functional outcomes for orthopedic conditions vary
depending on the condition itself and the health of the person. The general
course and prognosis of fractures are dependent upon several factors: age, type
of fracture, fracture location, severity of the fracture, and the patient’s intrinsic
motivation and premorbid health status. It is important to note, however, that
considering all of the orthopedic conditions, hip fractures are a leading cause of
morbidity and mortality in older adults (Stevens & Olson, 2000). Therefore,
occupational therapy practitioners should work closely with the referring
physician for the appropriate protocol and precautions. This statistic also
emphasizes the importance of addressing fall prevention and community
wellness programs for older adults.
The way in which the bone heals influences the patient’s course and
prognosis. There are two types of tissues that form bone: cancellous and cortical.
Cancellous bone or spongy bone is the inner layer that houses the bone’s
vascular supply. It is essential for nutrients to help form strong healthy bones.
Cortical bone is the hard outer layer of the bone that provides support and
protection. The periosteum is a dense fibrous membrane consisting of connective
tissues, elastic fibers, and nerve fibers, which line the outer surface of most
bones (Gray, 2004). Depending on the location, a fractured bone normally takes
6 to 12 weeks to heal (Kunkler, 2002).
To achieve optimal healing, it is important that the fracture site receive
immediate vascular circulation and appropriate immobilization. The healing
process involves five stages (Kunkler, 2002). First, a hematoma forms and seals
the damaged blood vessels, and then osteoclasts reabsorb the damaged bone and
tissue. The second stage is the formation of a granular or fibrocartilage tissue,
which increases the stability of the bone fragments. The third stage involves the
formation of a callus. This takes place between 2 and 6 weeks. The formation of
the callus has a significant influence on the outcome of the fracture. The fourth
stage is ossification and the formation of a bony union. The fifth or final stage is
often referred to as consolidation and remodeling occurring between 6 weeks
and 1 year. To reiterate, the amount of healing time depends on the severity of
the fracture and any premorbid health conditions. During remodeling, the bone is
ideally reshaped to its original form to enable it to resume its intended function
as best as possible.
In some instances, the bone heals abnormally. Abnormal healing can be
caused by several factors including an open fracture, severe soft tissue damage,
infection, poor vascularization, nerve damage, phlebitis, or compartment
syndrome. Such complications can result in delayed union, malunion, and
nonunion. A delayed union is when the bone takes more time to heal than is
expected; it heals slowly. It may be suspected when pain and tenderness persist
at the fracture site 3 months to 1 year after the injury (Kunkler, 2002). Delayed
unions may be caused by several factors including infection, poor
vascularization, or inadequate immobilization. Once the causing factor is
identified and corrected, the bone will typically heal. In a malunion fracture, the
fracture heals in an abnormal or deformed position. Contributing factors include
muscle imbalance and inadequate protection and positioning of the fracture
(Altizer, 2002). A malunion fracture has significant functional implications as
the person will often experience limited ROM, strength, and coordination.
Nonunion fractures refer to a fracture in which the bone is not healing. A
nonunion fracture may be caused by several factors such as vascular and tissue
damage, poor alignment, stress to the fracture site, and infection (Altizer, 2002).
The scaphoid bone has a high risk for nonunion due to its limited blood supply.
Arthritis
The course and prognosis of arthritis vary. Idiopathic osteoarthritis, which is the
most common, may show itself by osteophytes or “bone spurs” on the proximal
interphalangeal joints (Bouchard’s nodes) and distal interphalangeal joints
(Heberden’s nodes). These may become painful and inflamed, limiting
functional use. In cases where there is significant pain and joint degeneration, an
arthroplasty may be warranted. This is most common in the carpometacarpal
joint of the thumb (Cooper, 2014).
Osteopenia may progress to osteoporosis if untreated through lifestyle changes
including calcium supplements, a calcium-enriched diet, and added weight-
bearing exercise. As mentioned before, this condition is silent and will transform
into osteoporosis without any signs. Osteoporosis is irreversible. In regard to
prognosis, a person can live a productive and active life but will need to take
extra caution in joint protection and fall prevention to avoid a resulting fracture
due to the weakened bone.
In the case of HO, 10% to 20% of patients will have permanent functional
loss (Bruno-Petrina, 2008; Wheeless, 2015b). The severity of the functional
limitations will depend on the joints involved. For instance, there may be more
functional implications for a person who has had a burn and develops HO in the
dominant upper extremity versus if it develops in the hip joint of someone who
has paraplegia. The course of the condition, however, can be complicated if there
is additional nerve compression due to the ossification and lymphedema.
Medical management, which will be covered in the next section, is important in
maintaining ROM and decreasing functional limitations and pain.
Diagnosis
Following an injury with suspected fracture, a physician first performs a clinical
evaluation obtaining information on the signs and symptoms and the
circumstances surrounding the fracture. The physician usually refers the person
to radiology to confirm and determine the degree and classification of the
fracture. The radiologist also evaluates for other associated findings such as a
pathological fracture (bone weakened by a tumor), stress fracture, or other
preexisting conditions that may have affected the integrity of the bone. The
diagnosis is commonly confirmed through plain films or x-ray, though other
studies may be ordered depending on the physician, suspected injury, and
location of the suspected injury.
Fractures of the distal radius are also diagnosed through x-ray. To evaluate
the wrist for fracture, the standard views are posteroanterior, lateral, and oblique.
Scaphoid fractures can be more challenging to diagnose and, therefore, may
require what is known as a scaphoid view. Scaphoid fractures may also present
with dorsal wrist pain and tenderness with palpation to the anatomic snuffbox at
clinical examination. Given that this fracture is difficult to diagnose, a CT scan
or bone scan may be necessary (Sendher & Ladd, 2013).
Osteoarthritis can occur at any joint. Diagnosis begins with clinical exam as
patients complain of pain, stiffness, and swelling. The severity of osteoarthritis is
diagnosed by x-ray or radiographs. This demonstrates the amount of joint
damage that has occurred.
Osteoporosis is typically identified through a bone mineral density scan.
Osteopenia is often evident in bone scans, which may be used to confirm the
diagnosis (Li, Smith, Tuohy, Smith, & Koman, 2010). It may be initially
diagnosed in health screenings or when an adult has several risk factors such as
being female, smoking, consuming excessive alcohol, having a low body weight
(US Department of Health & Human Services, 2005), and a parent who has had
a hip fracture due to osteoporosis (Ahlborg et al., 2010; Khosla & Melton, 2007;
National Osteoporosis Foundation, 2010).
HO can sometimes be difficult to diagnose as it may be misdiagnosed as a
scarring or joint contracture. Patients with HO may present with a “locking
sign.” This presents at the end range of either flexion or extension of the joint.
X-ray is the most economical method to confirm the diagnosis of HO; if
suspected from clinical evaluation (Chen, Yang, Chuang, Huang, & Yang, 2009),
HO can also be diagnosed by a radiologist through bone scan, ultrasonography,
or CT scan (Moore & Cho, 2010).
The medical management of orthopedic conditions varies depending on the
specific condition. Some require conservative treatment consisting of over-the-
counter medications, splinting, and home exercise programs, while others may
require surgery. Below, we have provided a brief summary of the medical
management of the more common conditions that we have discussed in this
chapter.
Fractures
Hip Fractures
Most hip fractures require surgical intervention. The type of procedure depends
on several factors such as the severity of the fracture, prefracture functional level
of the patient, age of the patient, the presence or absence of comorbid conditions,
and the preference of the surgeon (Beloosesky et al., 2002; Lowe, Crist,
Bhandari, & Ferguson, 2010). Patients who are more active prior to their fracture
often have fewer complications and more positive results after surgery thus
emphasizing the importance of a healthy lifestyle (Beloosesky et al., 2002).
There is still mixed evidence on the morbidity and mortality rates and surgical
approach for the very elderly (Lowe et al., 2010). Therefore, physicians will
carefully consider these factors and make necessary preoperative referrals to
medically stabilize the patient while keeping in mind complications and further
discomfort that might arise with a delay in surgery (Holt, Smith, Duncan, &
McKeown, 2010).
The goal of surgical intervention for a hip fracture is to align and immobilize
the fracture site to allow for normal healing. Hip fractures are treated with closed
reduction and immobilization or open reduction and internal fixation (ORIF).
Closed reduction involves realigning the fracture fragments through manual
manipulation or traction, which may be done under general or local anesthesia
(Kunkler, 2002). Immobilization can be accomplished through casts, traction,
splints, or braces. Open reduction on the other hand involves surgically opening
and reducing the fracture site. Internal fixation is commonly done after an open
reduction to secure the fracture. Internal fixation involves securing the fracture
site with pins, rods, plates, and screws. This promotes healing and allows for
early mobilization, which can reduce complications often associated with
immobility. Some hip fractures, such as an intracapsular femoral neck fracture,
may be treated with hemiarthroplasty or a THR (Brunner & Eshilian-Oates,
2003). A hemiarthroplasty is a partial joint replacement in which the femoral
head and neck are replaced by a metal prosthesis. A total arthroplasty or THR
may be indicated depending on the patient’s age and whether the femur and
acetabulum were damaged from the injury or a preexisting disease.
Distal Radial Fractures and Humeral Fractures

Management of distal radius fractures has changed over the last 10 years
secondary to changes in fixation (Michlovitz & Festa, 2011). Distal radial and
humeral fractures may be reduced or realigned manually or with surgery. Closed
fractures may be reduced manually and immobilized with a cast. Fractures that
are unstable or cannot be manually reduced require surgery. This procedure is
called an open reduction and internal fixation (ORIF), which secures the
fracture site with screws, pins, plates, and rods. Open and comminuted fractures
often require ORIF. Figure 25.2 is a radiograph of an ORIF in which two plates
and screws were used to surgically stabilize displaced fractures of the radius and
ulna of a woman who had been in an MVA. As described earlier, all distal radius
fractures are not equal and, therefore, must be managed individually. Typically,
the immobilization phase following a distal radius fracture can last 1 to 6 weeks
(Michlovitz & Festa, 2011). During this phase, it is important to control edema
and pain while also addressing ROM in the shoulder and digits if allowed.
Complications such as nerve compression, tendon rupture, stiffness, complex
regional pain syndrome (CRPS), and pain may also impact progression and
outcome (Michlovitz & Festa, 2011). CRPS presents as chronic pain with
autonomic dysfunction. The most common precursor to this complication is
fracture (Li et al., 2010).
Figure 25.2 Open reduction internal fixation.
Scaphoid Fractures
A nondisplaced scaphoid fracture is typically immobilized with a thumb spica
cast for approximately 8 to 10 weeks (Dell et al., 2011) (see Fig. 25.3). Proximal
scaphoid fractures may require a long-arm thumb spica cast followed by the use
of a short-arm thumb spica cast to promote healing for as long as 6 weeks to 6
months. Displaced or unstable scaphoid fractures require an ORIF with a
compression screw (Dell et al., 2011). Following the ORIF, a wrist orthosis
should be used for 2 to 4 weeks for soft tissue healing. A percutaneous fixation
technique using a Herbert screw is a common and effective approach used in the
treatment of acute scaphoid fractures. This technique has been found to be
associated with a more rapid return of hand function, higher client satisfaction,
and minimal complications (Jeon, Oh, Park, Ihn, & Kim, 2003). The physician
and therapist should closely monitor the fracture to ensure proper
vascularization.
Figure 25.3 Scaphoid fracture.
Arthritis
Arthritis may be controlled with various medications, and patients should be
followed closely by a rheumatologist. Arthroplasty may be indicated in joints
that have severe pain and joint deterioration that significantly limits participation
in daily activities. Arthroplasties or joint replacements may be done in the hands,
shoulder, and other joints though it is most common in the hip and knee as they
are our weight-bearing joints.
Arthritis of the Hip

THRs are typically indicated for those who have severe arthritis causing pain
and stiffness that significantly limits the person’s participation in daily activities
or, as mentioned above, when there is damage to the acetabulum or femur during
a fall. This is a common condition seen by occupational therapy practitioners
working in acute care and inpatient rehabilitation. THRs are often seen in
individuals 55 years and older. The procedure involves replacement of the
acetabulum and ball of the femur with artificial implants. These may be secured
by bone cement or a cementless prosthesis, which allows for bone growth. Hip
replacements or arthroplasties generally last for 10 to 25 years (Roberts, 2002),
though researchers continue to evaluate implant wear.
Following a THR, there are several precautions that a patient must follow for
6 to 8 weeks after the surgery (Maher, 2014; Rasul & Wright, 2010). These
precautions include no hip adduction and rotation of the operated leg, and no hip
flexion beyond 90 degrees of the operated leg (Table 25.1). If the surgeon uses
an anterior approach, the patient must also avoid hip extension. Oftentimes,
therapists will encourage their clients to avoid hip rotation in general to ensure
that the surgical hip is protected. These precautions are currently the standard of
care; however, recent research suggests that an anterolateral surgical approach
rather than strict adherence to traditional hip precautions was more likely to be
associated with a low rate of dislocation (Peak et al., 2005). Depending on the
severity of the condition, the surgical approach and procedure, and the
orthopedic surgeon, there may be weight-bearing precautions for patients who
undergo a THRs and ORIFs. Common weight-bearing statuses include non–
weight bearing, touchdown or toe-touch weight bearing, partial weight bearing,
weight bearing as tolerated, and full weight bearing (Table 25.2).
TABLE 25.1 Total Hip Precautions

TABLE 25.2 Weight-Bearing Status
Modified from Goldstein, T. S. (1999). Geriatric orthopedics: Rehabilitative management of common

problems (2nd ed.). Gaithersburg, MD: Aspen.
Hip precautions have functional implications for the person in a variety of daily
activities. Occupational therapy practitioners must educate clients on the hip
precautions and teach them adaptations for sitting, dressing, bathing, reaching
for items, and driving among others. In regard to driving, patients are to avoid
driving for 4 to 6 weeks following surgery (Ganz, Levin, Peterson, & Ranawat,
2003). At this time, most patients have reached their preoperative driving
reaction time and continue to show improvements in reaction time for up to 1
year.
Arthritis of the Knee

The first line of management of arthritis of the knee is conservative treatment,
which often begins with over the counter anti-inflammatory medications and
heat and ice. If pain and functional limitations progress, the patient’s primary
physician or rheumatologist may prescribe medications to effectively control the
arthritis. When conservative treatment does not work, the patient may be a
candidate for a partial or total knee replacement (TKR). This is also referred to
as a knee arthroplasty. A TKR involves resurfacing the entire knee joint with
metal and plastic prosthetic components. The procedure typically involves a
femoral component, tibial plate, and a patellar button (Roberts, 2002) and may
be done unilaterally or bilaterally.
After surgery, it is important to begin the rehabilitation process (Bade, Kohrt,
& Stevens-Lapsley, 2010; Rasul & Wright, 2010). Treatment involves pain
medication and early mobilization to strengthen the quadriceps and prevent loss
of ROM. A continuous passive motion machine may be prescribed while in the
hospital or at home to help improve circulation and movement at the knee joint
depending on the patient’s postoperative health and functional abilities. Mizner,
Petterson, and Snyder-Mackler (2005), identified quadriceps weakness as a
primary impairment following TKR and found a high correlation between
quadriceps strength and functional performance over knee ROM or pain.
To maximize the results of the surgery and functional outcomes, the patient
needs education on several precautions such as avoiding putting a pillow under
the knee while in bed, resting both feet on the floor when sitting to increase knee
ROM, and wearing an immobilizer as instructed by the physician to protect the
knee joint until the muscle is strong enough to support it (Rasul & Wright,
2010). The immobilizer may be required when walking, while in bed, or at all
times. Kneeling should be avoided. Other considerations include not driving,
running, and jumping until cleared by the physician. Generally, patients are able
to return to most of their normal daily activities after 3 to 6 weeks though high-
impact activities may need to be modified. Driving may be resumed around 4 to
6 weeks as long as the patient is able to bend his or her knee enough to get in
and out of the car and have appropriate muscle strength and control to operate
the pedals. Patients need to follow up with their physician to monitor for
complications and ensure continued progress.
Occupational therapy practitioners use a holistic approach to examine specific
client factors residing within the person to understand their impact on
occupational performance (AOTA, 2014). Systems of values, beliefs and
spirituality, body functions, and body structures are client factors that are often
affected by illness or disability (AOTA, 2014). Client factors are an important
consideration when working with a person who has an orthopedic condition to
help them engage in daily occupations such as dressing, working, managing the
home, and leisure pursuits. We will discuss client factors that are commonly
affected by an orthopedic condition.
During the initial evaluation, it is important to carefully assess the nature and
circumstances surrounding the onset of the orthopedic condition. For example,
children and adults are likely to sustain fractures during play and sports
activities, whereas older adults may experience a fracture as a result of a fall or
osteoporosis. In some circumstances, however, a fracture may also be a result of
child abuse or domestic violence. The nature of the circumstance surrounding
the injury or condition may impact secondary injuries and psychosocial
involvement. It is important therefore to conduct a thorough history of the fall
and a radiologic analysis of the fracture (American Academy of Pediatrics,
2000) and, for children, to ascertain their developmental level.
Orthopedic conditions may compromise participation in several areas of
occupation including ADLs, instrumental activities of daily living (IADL), sleep,
education, work, play, leisure, and social participation (AOTA, 2014). The
degree of impairment depends upon the severity of the injury, location of the
injury, and the client’s premorbid health status. Several client factors may be
compromised by orthopedic conditions (Table 25.3). The main client factors that
will affect one’s participation in occupation are neuromusculoskeletal and
movement-related functions and structures and sensory and pain functions and
structures. Joint mobility, stability, and alignment are directly affected by a
fracture or a condition such as osteoarthritis. Therefore, in the case of fractures,
it is necessary to reduce or realign them in a timely manner. Limited joint
mobility may affect an individual’s ability to participate in daily activities such
as dressing, cooking, and cleaning. This in turn can affect one’s role
performance. For example, a woman with a distal radius fracture may initially
have difficulty fulfilling her mothering role with an infant. Until the pain and
swelling are reduced and the fracture is stabilized, she may be unable to lift her
baby and have difficulty doing things such as changing diapers.
TABLE 25.3 Client Factors Commonly Involved in Orthopedic Conditions

(AOTA, 2014)
Muscle strength and endurance are often affected by orthopedic conditions as a

result of immobilization of a joint during recovery. These limitations may also be
caused and worsened by disuse of the involved extremity. A person will often
hold their involved extremity in a dependent or guarded position and refrain
from using it. This further compounds the patient’s symptoms and often leads to
loss of ROM, strength, and pain in other joints and musculature that were
initially uninvolved. Limitations in muscle strength and endurance directly
impact one’s performance and participation in daily activities. A person
recovering from a humeral fracture may find it difficult to golf or may find that
his or her arm tires easily when painting or doing household chores.
Orthopedic conditions may affect sensation function and pain in the areas of
balance, sensation, and pain. When recovering from a THR or hip fracture, an
individual may struggle with balance as he or she tries to walk, move around in
the bathroom, and get dressed while following weight bearing precautions. As a
result, the individual may need to modify how he or she does certain activities in
order to prevent a fall. Sensation may be affected if there is nerve involvement at
the fracture site. Depending on the degree of sensory involvement, an individual
may have difficulty picking things up. Pain, as noted earlier in the discussion, is
a common sign of an injury. Pain will vary depending on the person, severity of
the injury, and complications. Sharp or continuous pain may affect an
individual’s ability to complete daily tasks. The pain experienced by people who
develop CRPS can be so overwhelming that it affects their ability to work and
carry out their life roles.
Joint pain, inflammation, and limited ROM from osteoarthritis often affect
participation in daily life activities as the integrity of the bone is compromised.
As such, an individual may require the use of adaptive equipment to help with
tasks such a dressing and grooming. Fluctuations in movement-related functions
can vary from day to day secondary to medications, pain, and efforts put forth in
energy conservation and work simplification techniques.
In addition, functions of the skin and related structures and mental functions
may be impacted by orthopedic conditions. Compound fractures that break
through the skin will result in a wound at risk for infection. This type of fracture
will involve more medical management and may consequently limit an
individual’s ability to participate in a variety of daily occupations due to pain,
wound care procedures, and type of fixation.
Mental functions such as one’s self-concept, motivation, and interests may
be impacted by an orthopedic condition. A distal radius fracture, for example,
may present with skin changes, edema, and severely limited movement in the
fingers. As a result, the individual may have difficulty doing many tasks such as
dressing, grooming, and writing. The inability to perform these daily tasks that
we often take for granted may impair one’s self-concept and thus affect a
person’s motivation such as when one withdraws from activities that he or she is
normally interested in. This emphasizes the importance for the occupational
therapy practitioner to continually assess and address the person’s values,
beliefs, and spirituality as these factors will impact the person’s coping strategies
and mental well-being throughout the healing process.
CASE-STUDY 1
Josh is a 16-year-old boy who enjoys participating in several sports during
the winter months. On a recent snowboarding venture, Josh was taken to a
local emergency room after he fell on his right arm while performing an air
stunt. The on-call physician evaluated Josh and ordered x-rays to confirm a
distal radial fracture. After confirmation of a closed fracture, the physician
manually reduced the fracture and immobilized Josh with a cast. After 5
weeks, the cast was removed and occupational therapy was ordered to
address ADL.
The occupational therapy evaluation found Josh to have impaired
strength and limited ROM in his forearm. Josh reported having difficulty
turning his forearm to dress and shares that his arm does not feel strong
enough to hold all of his school books.
Questions
1. What are three possible signs and symptoms of a distal radial fracture?
2. Approximately how long should a cast be worn for Josh’s type of
fracture?
3. What additional difficulties do you anticipate Josh may experience?
4. What client factors do you feel should be addressed first?
5. What tips can you give Josh regarding energy conservation and work
simplification?
CASE-STUDY 2
Ana is a 57-year-old woman who fractured her humerus in an MVA. She
was taken to the emergency room (ER) where the physician conducted a
clinical assessment and ordered x-rays to confirm the suspected fracture.
The radiologist report confirmed that there was a closed transverse fracture
of the middle third of the right humeral shaft. The physician was able to
reduce the fracture without surgery. He immobilized the arm in a sling.
Four weeks later, Ana still had significant pain and tenderness in her upper
arm. Radiological tests revealed that the bone was forming a malunion. The
physician determined that it was necessary to surgically reduce the fracture
and stabilize it with internal fixation. Radiologic studies showed signs of
healing 6 weeks after the second reduction as well as signs of osteopenia.
The physician wrote a prescription for occupational therapy.
The occupational therapist evaluated Ana. He noted that she held her
arm in a dependent position and was very hesitant to move it or let him
examine it. She reported significant pain and had atrophy of her upper arm
and forearm muscles. Her skin appeared shiny in comparison to her left
arm. Ana had limited movement and reported that her husband had to help
her with everything. She had been unable to return to work as an office
manager because the pain was just too great.
Recommended Readings and Resources
Books
Bonder, B. R., & Dal Bello-Haas, V. (2009). Functional performance in older
adults (3rd ed.). Philadelphia, PA: F.A. Davis.
Skirven, T. M., Osterman, A. L., Fedorczyk, J. M., & Amadio, P. C. (2011).
Rehabilitation of the hand and upper extremity (6th ed.). St. Louis, MO: Mosby.
Radomski, M. V., & Trombly-Latham, C. A. (2014). Occupational therapy for
physical dysfunction (7th ed.). Philadelphia, PA: Lippincott Williams & Wilkins.
Journals
American Journal of Occupational Therapy www.aota.org
American Journal of Orthopedics http://www.amjorthopedics.com/
Journal of American Geriatrics Society
http://www.wiley.com/bw/journal.asp?ref=0002-8614&site=1
Orthopaedic Nursing
http://journals.lww.com/orthopaedicnursing/pages/default.aspx
Websites
American Occupational Therapy Association. Evidence-Based Practice &
Research http://www.aota.org/Educate/Research.aspx
Centers for Disease Control and Prevention http://www.cdc.gov
Healthy People 2020
http://www.healthypeople.gov/hp2020/
Lewis, W. H. (Ed). Gray’s anatomy (12th ed.). New York: Bartleby.com.
Retrieved from http://www.bartleby.com/107
United States Department of Health & Human Services http://www.hhs.gov/
Wheeless, C. R. (2015). Wheeless’ textbook of orthpaedics.
http://www.wheelessonline.com
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C H A P T E R
26 Low Vision Disorders
KEY TERMS
Age-related macular degeneration Angle-closure glaucoma Aqueous humor
Cataracts Central scotomas Central vision Contrast sensitivity Glare sensitivity
Intraocular pressure Neovascular glaucoma Macula Metamorphopsia Myopia
Opacifications Open-angle glaucoma Peripheral vision Retina Trabecular
meshwork
George was leading a leisurely retirement and enjoying the time he had to kick
back and do all the things he couldn’t do while he was working. He was finally
able to spend time fishing at his cottage and taking care of his grandkids when
his daughter needed his help. George knew his vision wasn’t what it used to be,
but he started using reading glasses and felt like he was getting by. One day
while driving home from the store, he stopped at a four-way intersection and
then drove straight into the side of a car that had stopped before him. Luckily no
one was hurt, but after that, his daughter insisted that he see an ophthalmologist
who diagnosed him with a low vision disorder called age-related macular
degeneration. George was devastated. He feared he would become blind and lose
his ability to live alone.Low vision disorders are progressive diseases that lead to
chronic loss of sight and limit everyday function. Low vision disorders are one
of the most common causes of disability in United States of America with an
estimated 3 million people 40 years and older affected (Rosenberg & Sperazza,
2008). It is estimated that 80% of the population of people with low vision is
over age 65 years (Massof, 2002). Because of the aging of the population, the
number of people with low vision disorders is expected to continue to increase
with projected estimations of 5.5 million people in the United States by the year
2020 (Rosenberg & Sperazza, 2008).In the United States, the three most
common causes of low vision for people who are over 40 years old are macular
degeneration, glaucoma, and cataracts (Rosenberg & Sperazza, 2008). Because
of the prevalence of these diagnoses, most occupational therapists (OTs) will
work with people who have one of these conditions as either a primary or a
secondary diagnosis. Diabetic retinopathy is also a common low vision disorder,
but that condition will be covered in the Diabetes chapter in this textbook.Low
vision is the third most common cause of impaired function in people who are
older than 70 years (Rosenberg & Sperazza, 2008). Low vision disorders not
only limit a person’s ability to function independently but also increase the risk
of depression, social isolation, fall injuries, and a general decline in overall
health (Rosenberg & Sperazza, 2008). There is also a fear of blindness among
people with low vision. Blindness is one of the most feared diseases in the
United States (Burack-Weiss, 1992).
Macular Degeneration
Age-related macular degeneration (AMD) is a low vision disorder that results
from loss of function of the macula, the center of the retina (Bressler & Gills,
2000; Lim, Mitchell, Seddon, Holz, & Wong, 2012). There are two types of
AMD including dry AMD (atrophic/geographic atrophy) and wet AMD
(neovascular/exudative) (Lim et al.). Dry AMD is progressive atrophy of the
retinal pigment epithelium, choriocapillaris, and photoreceptors in the macula.
Wet AMD is an ingrowth of new blood vessels that break through the neural
retina and leak fluid, lipids, and blood into the subretinal space leading to fibrous
scarring (Chong et al., 2008; Hirami et al., 2009) (see Fig. 26.1).
Figure 26.1 Anatomy of the eye. (From Anatomical Chart Co.)
AMD is also classified as either early or late. Early AMD is the beginning signs
that the disease is developing and late AMD is when the disease has progressed
to the macula (Chong et al., 2009). In early AMD, the retina is unhealthy and
predisposed to visually threatening complications or late AMD (see Fig. 26.2).
Figure 26.2 Macular degeneration. (From Anatomical Chart Co.)
Etiology
Research indicates that the cause of AMD is probably a combination of genetic
and environmental factors (Chong et al., 2009; Wang et al., 2008). The
complement factor H gene and gene variants BF and C2 have been associated
with higher rates of AMD (Wang et al., 2008). Several other genes may also be
involved including vascular endothelial growth factor (Lim et al., 2012). Genetic
susceptibility, however, can be modified by altering environmental influences
that are linked to the development of AMD.
Many environmental factors including smoking; obesity; dietary intake low
in vitamins A, D, E, zinc, lutein, and omega-3 fatty acids; and ultraviolet light
exposure are linked to the development of AMD (Goodman, Parmet, Lynm, &
Livingston, 2012; Lim et al., 2012). Cigarette smoking has been found to trigger
or promote the development of AMD or contribute significantly to higher risks
for AMD (Wang et al., 2008). Diets high in red meat consumption are associated
with early AMD (Chong et al., 2009), and diets high in fish consumption are
associated with decreased risk for late AMD (Montgomery et al., 2010; Wang et
al., 2008). An unhealthy lifestyle that results in cardiovascular problems, such as
hypertension, is also associated with AMD. Long-term, regular use of aspirin is
significantly associated with an increased risk of wet AMD (Klein et al., 2012).
Ocular risk factors include darker iris pigmentation, cataract surgery, and
hyperopic refraction (Lim et al.).
AMD is the leading cause of severe vision loss for people older than 50 years in
the United States and other developed countries (Friedman et al., 2004) and is
the leading cause of vision loss worldwide (Lim et al., 2012). The prevalence of
AMD increases with age with 2% prevalence among people 40 years of age and
as many as 25% of people by age 80 years (Ambati & Fowler, 2012). Current
studies find that AMD is more prevalent in Caucasian and Asian populations.
Because the population is continuing to age, the incidence of AMD is increasing
with an expected increase to 3 million cases of late AMD in the United States by
the year 2020. Dry AMD accounts for 80% of AMD cases in Western countries,
whereas other parts of the world have higher incidence of wet AMD (e.g., Japan)
(Hirami et al., 2009).
Signs and Symptoms
The primary symptom of wet AMD is acute loss of central vision that becomes
permanent as the retina atrophies and is replaced by fibrous tissue. Wet AMD
usually results in rapid loss of central vision. Dry AMD can also result in the
loss of central vision, but the progression is much slower taking years to
develop. Initially, a person with AMD may be asymptomatic (Lim et al., 2012).
Over time, loss of visual acuity for discrimination of details, metamorphopsia
(distortion of objects), central scotomas (dark patches), increased glare
sensitivity, decreased contrast sensitivity, and decreased color vision can
develop (Rosenberg & Sperazza, 2008).
Although the loss of vision can be profound and greatly impair function,
people with AMD frequently do not notice the symptoms in the early stages. The
visual loss does not result in black or white spots but is spotty loss that is filled
in by the surrounding vision that is still intact (Table 26.1).
TABLE 26.1 Signs and Symptoms of Low Vision Disorders

The course of AMD varies from person to person. Dry AMD does not always
advance into wet AMD, but wet AMD always is preceded by dry AMD and
therefore, dry AMD is considered a risk factor for wet AMD (Ambati & Fowler,
2012). The timeline for the progression of dry or wet AMD is variable. Some
people have dry AMD for years before it develops into wet AMD and others
develop wet AMD right away (Mogk & Mogk, 2003).
Overall, 1% of people with early AMD will progress to late AMD each year
(Lim et al., 2012). So, in 5 years, 5% of people with early AMD will progress to
late AMD and in 15 years, 15% of those people will progress from early AMD
to late AMD.
The prognosis for loss of central vision due to AMD also varies. For some
people, loss of central vision will proceed rapidly and others will have AMD for
years and still maintain a functional level of central vision (Mogk & Mogk,
2003). Wet AMD progresses much more rapidly than does dry AMD (Ambati &
Fowler, 2012). Regardless of the speed of progression, AMD does not cause
total blindness. AMD results in loss of central vision, but it does not affect
peripheral vision. Therefore, the person will retain peripheral vision even if he
or she has late AMD.
Diagnosis
An Amsler grid test may be used initially by an eye doctor to screen for signs of
AMD, but diagnosis relies on more invasive tests (Goodman et al., 2012). The
diagnosis of AMD is made through examination by an ophthalmoscope. In early
AMD, the formation of drusen, small yellow deposits in the center of the retina,
can be seen when the pupil is dilated (Bressler & Gills, 2000; Lim et al., 2012).
In dry AMD, the pigmented layer of the retina slowly atrophies.
In wet AMD, abnormal new blood vessels in the choroidal layer of the eye
that nourishes the outer retina grow and proliferate with fibrous tissue within the
drusen material are detected through fundus fluorescein angiography, an
examination of the vascular system of the eye using an intravenous injection of
yellow dye. The bleeding accumulates within and beneath the retina, and the
retina atrophies or becomes replaced with fibrous tissue.
There is no medical treatment for early dry AMD. There are two medications
that have been developed in the last several years, however, that aid in the
prevention of vision loss due to wet AMD and even lead to the improvement of
vision in some cases (Lim et al., 2012). Those medications are ranibizumab and
bevacizumab. Both medications work to suppress the vascular endothelial
growth factor and are delivered through intravitreal injections usually on a
monthly basis (Lim et al., Martin, et al., 2011). In countries where these
medications are used routinely, there has been a significant reduction in legal
blindness due to AMD (Cheung & Wong, 2013). Bevacizumab (Avastin,
Genentech), although developed for the treatment of colon cancer, is also used as
it is related to the medication ranibizumab. Aflibercept was also recently
approved for the treatment of wet AMD, but it has not been widely adopted
(Cheung & Wong, 2013). In addition, high-dose regimens of zinc and
antioxidants (vitamin C, vitamin E, and beta-carotene) are recommended
(Rosenberg & Sperazza, 2008). Lipid-lowering drugs such as statins have been
tested as a treatment for AMD but have not been found to be effective in the
treatment or prevention of AMD (Chuo, Wiens, Etminan, & Maberley, 2007).
Laser treatments are used for wet AMD to burn the area of the retina with
neovascularization and photodynamic therapy, which uses the drug verteporfin
with the laser treatment to selectively destroy lesions (Bressler & Gills, 2000;
Goodman et al., 2012). This treatment is usually repeated every 3 or 4 months to
prevent growth of blood vessels.
In addition to medical treatments, people with AMD are assessed for
impairments in mobility and activities of daily living with a referral to physical
therapy or occupational therapy if necessary. They may also benefit from
psychological evaluation for anxiety or depression as persons with AMD have
been found to have significant emotional distress and profoundly reduced quality
of life (Rosenberg & Sperazza, 2008; Williams, Brody, Thomas, Kaplan, &
Brown, 1998).
Glaucoma
Glaucoma is a group of conditions with differing causes that result in damage to
the optic nerve head (King, Azuara-Blanco, & Tuulonen, 2013). This low vision
disorder is characterized by progressive loss of the ganglion cell layer of the
retina usually caused by increased intraocular pressure (IOP) (Rosenberg &
Sperazza, 2008; Wittstrom et al., 2010). Recent findings, however, reveal that
20% to 52% of people with glaucoma have IOP that measures within the normal
range (King et al.).
Three types of glaucoma include open-angle glaucoma, angle-closure
glaucoma, and neovascular glaucoma. Angle-closure glaucoma is the
obstruction by the iris to the outflow pathway of aqueous humor through the
trabecular network at the angle between the peripheral cornea and the iris (King
et al., 2013; Subak-Sharpe, Low, Nolan, & Foster, 2009). In open-angle
glaucoma, there is not an obstruction of the aqueous humor, but the damage to
the retina is caused by increased IOP. Neovascular glaucoma is a secondary
disorder that results from other diseases, such as diabetes mellitus or tumors,
which cause new blood vessels to grow and obstruct the outflow of aqueous
humor (Shazly & Latina, 2009). See Figure 26.3.
Figure 26.3 Path of aqueous flow. (From Porth, C. (2014). Essentials of
pathophysiology (4th ed.). Philadelphia, PA: Lippincott Williams & Wilkins.)
Etiology
As with AMD, glaucoma is thought to be caused by a combination of genetic
and environmental factors. A family history of glaucoma increases a person’s
chances of developing the disease (Hollands et al., 2013). Although several
genes have been identified in relation to glaucoma, they account for only a small
portion of cases (Leske, 2007). Other factors that may contribute to glaucoma
include abnormal blood pressure, diabetes, cataracts, myopia, and
hypothyroidism (King et al., 2013; McDaniel & Besada, 1996; Subak-Sharpe et
al., 2009).
Several pharmacological interventions have also been linked to angle-
closure glaucoma (Subak-Sharpe et al., 2009). Bronchodilators, antidepressants,
anticholinergics (used for overactive bladder), antihistamines, botulinum toxin,
and sildenafil (Viagra) are all pharmaceuticals that have been linked to angle-
closure glaucoma. Also, recreational drug use of cocaine and ecstasy has been
shown to cause angle-closure glaucoma.
Glaucoma is the second most common cause of blindness worldwide accounting
for 4.5 million people or 12% of cases, but it is the leading cause of blindness
among people of African descent accounting for 32% of cases (Hollands et al.,
2013; Leske, 2007; Lin & Yang, 2009). As of 2010, glaucoma accounted for 2%
of cases of visual impairment and 8% of cases of blindness globally (King et al.,
2013). It is uncommon in people under the age of 20 years with fewer than 3 per
100,000 in the United States for all forms of glaucoma (Quigley, 2011).
Open-angle glaucoma is the most common form of glaucoma in Western
cultures, but angle-closure glaucoma accounts for 50% of blindness worldwide
and is more common in Asian populations (Leske, 2007; Subak-Sharpe et al.,
2009). Although variation is noted, most studies report higher incidence and
prevalence of glaucoma in men than in women (Leske, 2007) and higher
incidence and prevalence in people of African descent (Rosenberg & Sperazza,
2008).
There is a marked increase in the prevalence of glaucoma among people
older than 40 years with an overall prevalence of 0.3%. Prevalence increases to
3.3% in people older than 70 years. Because of aging populations, the incidence
of glaucoma is expected to rise (King et al., 2013). Variations in the prevalence
of open-angle glaucoma among people older than 40 years can be seen
throughout the world with estimates of 1% to 5% in the United States, 1% to 3%
in Europe, 1% to 4% in Asia, 1% to 8% in Africa, and 2% to 3% in Australia
(Leske, 2007). The highest prevalence is in the Caribbean with 7% to 9%. Most
people in the Caribbean have ancestry linked to West Africa where open-angle
glaucoma is very common.
Signs and Symptoms
Open-angle glaucoma begins with a slow loss of peripheral vision that can
eventually lead to loss of central vision (King et al., 2013; Rosenberg &
Sperazza, 2008). Other symptoms include decreased ability to see in dim light,
decreased contrast sensitivity, poor adaptation to changes in lighting, sensitivity
to glare, blurred vision, and decreased depth perception (Lin & Yang, 2009). In
the early stages, however, many of these symptoms may not occur or may
develop so gradually that people are not aware of their condition (Beleforte et
al., 2010; Quigley, 2011). By the time visual loss appears, there is usually
already significant and permanent damage.
The symptoms of closed-angle and neovascular glaucoma, however, may
have a more rapid onset. The symptoms include significant ocular pain,
headache, nausea, vomiting, and loss of or blurred vision. As many as 75% of
people with closed-angle glaucoma may not have an acute attack but remain
asymptomatic or have such gradual loss of peripheral vision that the symptoms
are unnoticed until the disease has progressed significantly (Quigley, 2011).
Angle-closure glaucoma has three stages. The first stage is marked by contact
between the peripheral iris and the trabecular meshwork. This progresses to the
formation of adhesions, which cause increased IOP. If not treated, this may
progress to cause glaucomatous optic neuropathy, which causes significant
functional impairment of the vision (Subak-Sharpe et al., 2009). Open-angle
glaucoma tends to progress at a slower rate and the patient may not notice that
he or she has lost vision until the disease has progressed significantly (King et
al., 2013). Neovascular glaucoma begins when a fibrovascular membrane
initially obstructs aqueous outflow in an open-angle fashion and later contracts
to produce secondary angle-closure glaucoma (Shazly & Latina, 2009).
If left untreated, glaucoma will lead to blindness. If treated, however, people
with glaucoma usually retain some level of functional vision for their whole life
(Ang & Eke, 2007). In developed countries, 3% to 12% of people with glaucoma
are blind in one or both eyes (King et al., 2013).
Diagnosis
Early diagnosis and treatment are important to prevent vision loss. Diagnosis of
open-angle glaucoma is based on visual field defects, disc defects, and
abnormally increased IOP (Hollands et al., 2013; King et al., 2013; Sonnsjo &
Krakau, 1993). There is some debate about the use of IOP in the diagnosis of
glaucoma because this increase is not always seen (Heijl, 2015; Hollands et al.;
Leske, 2007; Quigley, 2011). When increase IOP is detected, however, it is used
to aid in the diagnosis. Angle-closure glaucoma is diagnosed using gonioscopy
or other screening techniques to examine the anterior chamber angle for contacts
or adhesions (Subak-Sharpe et al., 2009). Neovascular glaucoma is diagnosed
through the detection of new blood vessels in the iris, a closed anterior chamber
angle, and high IOP (Shazly & Latina, 2009).
Glaucoma is treated with topical ocular medications, such as eye drops, oral
medications, laser therapy, and surgery. The first line of treatment is usually
glaucoma drop monotherapy with different types of drops added to the regime as
needed to control IOP.
A trabeculectomy, the removal of part of the trabecular meshwork, is the
most common surgical procedure used to reduce IOP. Glaucoma filtration
surgery may also be used to decrease IOP pressure in open-angle glaucoma
(Wittstrom et al., 2010). Laser surgery, such as a goniopuncture, may be used to
perforate the trabecular meshwork to increase aqueous flow (King et al., 2013).
For angle-closure glaucoma, surgical procedures may include an iridotomy
or an iridoplasty (King et al., 2013; Subak-Sharpe et al., 2009). An iridotomy is
a procedure in which a laser is used to create a hole in the iris to allow for
drainage of the aqueous humor and an iridoplasty is a procedure in which a laser
is applied to the peripheral iris to remove adhesions to the trabecular meshwork
and open the angle. Cataract surgery to allow the iris to move away from the
drainage pathway may also be used (King et al.). Topical ocular medications
may be given to constrict the pupil and thus pull the peripheral iris away from
the trabecular meshwork. Oral medications may also be given to reduce IOP for
all types of glaucoma.
Future treatments that are currently being explored are intraocular implants
to deliver medication, therapeutic treatments that provide neuroprotection for the
retinal ganglion cells, stem cells or gene transfer to improve trabecular
meshwork outflow and reduce IOP, and stem cell transplants for retinal ganglion
cell regeneration (King et al., 2013; Quigley, 2011).
Cataracts
Cataracts are opacifications of the crystalline lens of the eye, which result in a
decreased amount of light reaching the retina. Cataracts are made up of broken
proteins that stick together and form sheathlike obstructions in the lens
(Armitage, 2015). The incoming light is scattered, and visual acuity is decreased.
While there are some variant cataract conditions such as congenital cataracts due
to rubella, the focus of this chapter will be on the most common type, age-related
cataracts (see Fig. 26.4).
Figure 26.4 Cataract. (From Dudek, R. W. (2013). High-yield embryology.

Etiology
As with other low vision disorders, the cause of cataracts is a combination of
factors. Risk factors for the development of cataracts include aging, cigarette
smoking, ocular UVB and radiation exposure, drug use, systemic diseases (e.g.,
diabetes), and dietary nutritional intake with increasing age being the strongest
risk factor by far (Rosenberg & Sperazza, 2008). Some of the drugs associated
with increased cataract risk are corticosteroids, antipsychotics, chemotherapy
agents, cholesterol-lowering medications, and tranquilizers. Heavy alcohol
consumption has been labeled a risk factor for the development of cataracts, but
recent meta-analyses refute that claim (Wang & Zhang, 2014). The use of statins
has been disputed as either increasing or decreasing the risk of cataracts, but
recent research has not found any evidence of statins being effective for reducing
the risk of cataracts and may actually contribute to the problem (Hoster, 2013).
Those studies, however, are confounded by high levels of bad cholesterol from a
poor diet, and researchers continue to recommend increased consumption of
fruits and vegetables to reduce risks.
Meat consumption when compared to the vegetarian or vegan diets is
associated with increased risk for cataracts (Appleby, Allen, & Key, 2011).
Lutein (found in dark green leafy vegetables and eggs), vitamin C, and vitamin E
intake have shown some associations with reduced risks for the development of
cataracts (Lyle, Mares-Perlman, Klein, Klein, & Greger, 1999). Iodine
deficiency and injuries to the lens are also risk factors for cataracts.
Cataracts are the third leading cause of blindness in the United States,
accounting for about 9% of all cases (Sperduto, 1994), and are a leading cause of
blindness worldwide (Wang & Zhang, 2014). Nearly 17.2% of Americans over
age 40 years are diagnosed with cataracts (Armitage, 2015). They are more
prevalent among females than males and are more prevalent among people of
African descent (Rosenberg & Sperazza, 2008; Sperduto, 1994). Cataracts also
vary in prevalence among different age groups with approximately 4% of people
aged 52 to 64 years, 28% of people aged 65 to 74 years, and 46% of people aged
75 to 85 years.
As the population ages, the number of people worldwide who will be blind
due to cataracts is expected to increase dramatically over the next few decades
(Sperduto, 1994). The number of people who are blind due to cataracts could
reach close to 40 million by the year 2025. As the ozone layer is depleted, there
is an increase in ultraviolet radiation and this is expected to also increase the
incidence of cataracts worldwide.
Signs and Symptoms
Decreased visual acuity, decreased contrast sensitivity, glare disability,
nearsightedness (myopia), and decreased color perception especially to blue
hues are all common symptoms of cataracts (Rosenberg & Sperazza, 2008).
Complaints of cloudy, blurry, or foggy vision are often noted. The vision of
people with cataracts is sometimes described as viewing the world through a
steamy glass (Armitage, 2015). Some people may experience temporary
improvement in near acuity as the shape of the lens is changed by the cataract.
When a person is young, the lens is transparent and incoming light has no
difficulty reaching the retina. Over time, however, the lens becomes less
transparent as a cloudy, opaque cataract develops in the lens. One eye is usually
affected earlier than the other, but eventually, both eyes are usually involved. If
left untreated, cataracts may lead to blindness or develop into other eye diseases
such as AMD or glaucoma (Rosenberg & Sperazza, 2008).
Diagnosis
A diagnosis of cataracts is done using pupil dilation, acuity charts, and
tonometry. Tonometry is done to examine ocular fluid pressure inside the eye.
Some cataracts can be detected with a visual examination that reveals a cloudy
lens, but pupil dilation is done to further examine the back of the eye.
Initially, the cataracts may be managed by prescription lenses, tobacco cessation,
and UV protection, but the only medical treatment for cataracts is the surgical
removal of the lens by either laser or scalpel (Armitage, 2015). When the
cataract has advanced to a stage that it interferes with a person’s ability to
function, surgery is performed to remove the molecular grout that builds up in
the eye (Armitage, 2015; Sperduto, 1994). Sometimes the lens is replaced.
Research is currently being conducted that aims to develop a steroid-based eye
drop that could dissolve cataracts (Armitage, 2015).
Impact of Conditions on Occupational
Performance
Visual function is the client factor that is impaired in low vision disorders. The
extent of the impact on occupational performance depends on the type of low
vision disorder, the stage of progression of that disorder, and the treatment that
has been received. Loss of central vision is the main concern for people with
AMD and cataracts, and loss of peripheral vision is the main concern for people
with glaucoma. For most people, cataracts will be surgically removed when they
interfere with the person’s ability to function. There are standardized
assessments, such as the Impact of Vision Impairment Scale and the Visual
Function Questionnaire, that may be useful to help therapists ascertain the
impact of a person’s visual loss on occupational performance (Lamoureux et al.,
2008; Magacho et al., 2004).
Because of the loss of central vision, the self-care skills that require visual acuity
are most affected by AMD and cataracts (Lim et al., 2012). These include skills
such as make-up application, dental care, nail care, and shaving. Reading and
inability to recognize faces are common problems (Bennion, Shaw, & Gibson,
2012). Management of medications may be difficult due to difficulty reading
labels and identifying various medications. Eating may also be visually
challenging in advanced stages of the disorders.
Safety during functional mobility is a concern for all low vision disorders.
The loss of central vision in AMD and cataracts and the loss of peripheral vision
in glaucoma can impact a person’s ability to safely navigate both within and
outside the home (Wu et al., 2010). Within the home, entryways and stairs are
especially difficult particularly at night. Adaptations, such as increased lighting,
railings, and visual contrasts, may need to be made to increase safety. Outside
the home, unfamiliar places may pose challenges for both locating points of
interest and negotiating varying surfaces.
Driving is impacted by all low vision disorders (King et al., 2013). Because
driving represents independence for most people, it can also be the most difficult
occupation to address. In addition, driving is necessary for most other
instrumental activities of daily living (IADLs) such as shopping, going to the
post office, and making appointments (Bennion et al., 2012).
Home management including cleaning, doing laundry, making home repairs,
and maintaining a yard can all be impacted by low vision disorders. Being able
to perform meal preparation accurately and safely is also a concern as loss of
visual acuity will interfere with seeing food items, utensils (e.g., knives,
measuring spoons, measuring cups), appliance settings, and recipes.
Money management for reading bills, paying bills, keeping records, and
identifying and handling money can all be impacted by a loss of central vision.
Large-print electronic systems may be useful for assisting with money
management.
Education
Because all of these low vision disorders are likely to be age related, most
people with these disorders will have completed their formal education. If
participation in education is still essential, functional mobility and reading may
interfere with successful completion of education-related occupations.
Work
The age of retirement has progressively increased, and therefore, the person may
still be involved in work occupations when low vision disorders progress to the
point that they interfere with function. Depending on the area of work in which
the person participates, visual deficits in central and peripheral vision can impact
the person’s ability to successfully complete work tasks. Work-related activities
such as reading, working on the computer, using the telephone, working on an
assembly line, and driving can all be impaired by visual deficits.
Play and Leisure
Many leisure activities can be affected by low vision disorders. Many people
with low vision disorders are retired and fill their time with leisure activities. In
addition, these leisure activities may be part of the person’s identity providing
life purpose and satisfaction. Painting, knitting, sewing, gardening, playing
games, completing puzzles, watching television, reading, and participating in
sports are just some of the many leisure activities that may be difficult for a
person with a low vision disorder.
Low vision disorders can impact social function in many ways. People with
AMD frequently complain about difficulty recognizing faces, which may lead to
feelings of humiliation and embarrassment in social situations. People with low
vision disorders may also have difficulty negotiating crowded places such as
concerts, restaurants, and ceremonies. In restaurants, they may have difficulty
reading the menu in addition to navigating the physical layout. They may
become distressed if they collide with people or objects. All of these factors may
lead the person into a life with more isolation. The combination of the loss of
independence and anxiety about the ability to function in social situations can
often lead to depression (Bennion et al., 2012). When people become depressed,
they are less likely to pursue social contact and a cycle of isolation and
depression can be worsened.
CASE-STUDY 1
Maria is a 72-year-old woman who has been referred to occupational
therapy due to vision loss. Maria was diagnosed with wet age-related
macular degeneration 2 years ago and is having difficulty functioning in her
home. The recent death of her husband, who was able to help her in the
home, has left her trying to find ways to manage on her own. She has two
children. Her daughter lives about 1 hour away with her three children, and
her son lives about 2 hours away in a large urban area. Her children are
reportedly concerned that she is no longer safe to live independently.
Maria worked for many years as a legal assistant until she retired at age
62. Since then, she has been enjoying traveling with her husband, spending
time with her grandchildren, and participating in various leisure activities,
such as knitting, reading, gardening, and playing mahjong. She was once an
avid tennis player but had to stop playing due to her vision loss.
Maria now lives alone in a four-bedroom, split-level home and would
like to remain there for as long as possible. She is having difficulty
accepting the fact that driving is dangerous for her and she tries to get by
using a GPS. She also gets embarrassed when she doesn’t recognize people,
so she has started to avoid social situations where she is expected to interact
with people who don’t know about her vision loss.
Maria has been getting monthly injections of ranibizumab since she was
diagnosed, but because they don’t improve her vision, she has difficulty
understanding why she needs more treatments. She is also taking high doses
of zinc, vitamin C, vitamin E, and beta-carotene, but she states that
sometimes she is not sure if she has taken the right pills. She has been
referred to occupational therapy for evaluation and treatment. Her primary
goal is to maintain her current lifestyle as long as possible.
CASE-STUDY 2
Bob is known among his friends as Bob the Builder. He retired from his job
as a site manager 10 years ago but has been busier than ever helping his
friends with home improvement projects. Bob’s wife says he is so busy
helping their friends that he never has time to get to their own home
improvement needs. Bob and his wife, Sharon, live in a four-bedroom
century-old farmhouse that they have been fixing up since they moved in 10
years ago. Their home is on a 40-acre lot, but they rent out the land to a
local farmer.
Bob and Sharon have been married for 15 years. They don’t have
children of their own, but both have children from previous marriages. All
of their four children live within an hour of them and they have seven
grandchildren between them.
Last year, Bob went to his eye doctor for a routine checkup. It had been
about 5 years since his last checkup. His kids were teasing him about his
outdated glasses and he decided to get a new style. Bob hadn’t noticed any
significant changes in his vision. He knew he was having some difficulty
seeing when it was dim lighting, but he thought this was just due to his eyes
getting older. He was very surprised when the eye doctor told him that he
suspected that he had glaucoma.
The eye doctor referred him to a specialist who confirmed the
diagnosis. The ophthalmologist explained that he had what is called open-
angle glaucoma and showed him diagrams of how much peripheral vision
he had already lost. Bob and his family were surprised. Bob felt healthy and
never really noticed the significant loss of vision. His mother had
glaucoma, but he didn’t connect that to the possibility that he would one
day develop it as well.
Bob uses eye drops and recently underwent surgery to reduce the
pressure in his eye. He has been referred to occupational therapy to see if
adaptations can be made to his environment and lifestyle to help him
maintain as much function as possible in the coming years.
CASE-STUDY 3
Elizabeth, who is known as Beth to her family and friends, has been dealing
with increasingly limited vision due to cataracts. Beth is 68 years old and
has lived with her partner, Sue, for the last 26 years in a high-rise condo in
an urban area. Beth has a daughter from a previous relationship and she
lives within walking distance to her mother’s home. Beth is retired from her
career as an architect, but Sue still works as a teacher and has an hour
commute to and from work.
Since her retirement, Beth spends most of her time participating in
leisure activities. She loves to paint, swim, and work out in the building’s
gym facilities. She is also an avid reader but has been frustrated with her
visual deficits when trying to read. Her daughter owns and operates a local
coffee shop, so she frequently stops by there to hang out or help out her
daughter as needed.
Beth has been very aware of her increasingly limited vision over the
past several years. She describes her vision as cloudy, blurry, and sensitive
to “bad lighting.” She started wearing glasses a few years ago, but now, her
vision loss is starting to interfere with her ability to function around the
home and within her community.
Beth’s eye doctor discussed routine eye surgery to remove the cataracts
from both eyes. Once the eye doctor discovered that Beth had scarring on
her retina in the right eye due to an old injury, however, she decided that the
surgery would only benefit the left eye. Beth, therefore, will continue to
have a significant loss of vision in her right eye even after the surgery is
done.
Beth was referred to occupational therapy for an evaluation and
intervention to assist her in making any adaptations necessary to increase
her ability to function independently in her home and her community.
RESOURCES
American Academy of Ophthalmology http://www.aao.org
American Council of the Blind http://www.acb.org
American Foundation for the Blind http://www.afb.org
MACULAR DEGENERATION
MedlinePlus
www.nlm.nih.gov/medlineplus/maculardegeneration.html
MayoClinic.com
www.mayoclinic.com/health/macular-degeneration
Macular Degeneration Foundation www.eyesight.org/
GLAUCOMA
Glaucoma Research Foundation www.glaucoma.org/
Mayo Clinic
www.mayoclinic.com/health/glaucoma
National Eye Institute
www.nei.nih.gov
CATARACTS
MedlinePlus
www.nlm.nih.gov/medlineplus/cataract.html
National Eye Institute
www.nei.nih.gov
Mayo Clinic
www.mayoclinic.com/health/cataracts
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C H A P T E R
27 Cancer
Suzänne Fleming Taylor
KEY TERMS
Allogeneic bone marrow transplant Autologous bone marrow transplant Benign
Bone marrow Bone marrow transplant Cancer
Cancer cachexia Cancer-related cognitive dysfunction (CRCD) Cancer-related
fatigue (CRF) Carcinoma
Chemotherapy-induced peripheral neuropathy (CIPN) Disseminated
intravascular coagulation (DIC) Fistula
Graft versus host disease (GVHD) In situ
Leukemia
Leukostasis Liquid tumor Lymphedema Lymphoma
Malignant
Metastatic disease Metastatic spinal cord compression (MSCC) Mirel’s scoring
Mixed-type cancer Myeloma
Neoplastic Neutropenia Osteoblastic lesion Osteolytic lesion Pathological
fracture Sarcoma
Solid tumor Superior vena cava syndrome (SVCS) Survivorship TNM
classification of malignant tumors Tumor lysis syndrome
Mark, a 49-year-old male, could not get rid of his nagging cough. As a
construction worker, he was used to the changing weather, and he had not had
trouble before. Over the next few weeks, Mark noticed he was fairly tired
throughout the day and tended to become short of breath carrying items that
previously caused him no trouble. After his roommate commented on the
noticeable weight loss, Mark decided to make an appointment with his primary
care physician. Upon learning the recent symptoms, the physician ordered an x-
ray of his lungs. The results showed an abnormal mass in the left upper lobe,
prompting the physician to recommend consultation with an oncologist. Mark
was told to expect to undergo a tissue biopsy as his physician suspected lung
cancer.
In 2014, there were nearly 14.5 million people living with cancer in the United
States, and in 2015, an estimated 1,658,370 new cases will be diagnosed,
bringing the total to nearly 16.2 million cancer survivors in the United States
(National Institutes of Health: Surveillance, Epidemiology, and End Results
Program, 2015). As cancer survivors are living longer after diagnosis, the
challenge becomes one of ensuring quality of life and optimal functional and
cognitive abilities.
The focus of this chapter is to provide an overview of cancer including
definitions, etiology, signs and symptoms, medical and surgical management
including common side effects, and the impact of cancer on occupational
performance. Readers are encouraged to utilize the resources listed to further
enhance their understanding of this complex and chronic disease.
Cancer refers to a collection of related diseases in which some of the body’s
cells divide without control and are able to invade other tissues. This occurs
when the normal process of cell division, growth, and death is not properly
regulated, allowing old or damaged cells to continue to survive, and new cells
are unnecessarily formed, leading to an abnormal growth, or neoplasm. Cancer
can start almost anywhere in the body and is typically named for the organ or
cell where it originates. For example, lung cancer originates in the lungs, breast
cancer originates in the breast, and so forth.
The basic tumor cell types are classified as benign, in situ, or malignant. The
cells in a benign tumor remain differentiated, or genetically similar to the
original cell, and therefore are unable to spread to other tissues, structures, or
organs. Since benign tumors are not able to spread, they are not considered
cancerous. In situ translates to “in position” or “in place.” This is an early stage
of cancer in which the cancerous, or neoplastic, cells remain in the original site
from which they arose. While these cells have not spread, unlike benign tumors,
they have a very high likelihood of progression to becoming undifferentiated and
spreading to surrounding tissues, structures, and/or organs. In situ may also be
referred to as premalignant. A malignant tumor invades surrounding tissues and
is usually capable of spreading to distant tissues, or metastasizing. Malignant
tumors are typically capable of recurring after removal and may recur as
metastatic disease, which means the cancer from one organ or part of the body
has spread to another organ or part of the body not directly connected. For
example, an individual may have a history of breast cancer, status post
mastectomy with chemotherapy and radiation, now in remission, and years later
have recurrence as metastatic bone disease.
Cancers are generally referred to as either a “solid tumor” or “liquid tumor.”
Solid tumors begin in a solid structure such as bone, muscle, or organ and
include carcinoma and sarcoma. Liquid tumors are also known as blood cancers
or hematological tumors. They arise from and affect the blood, bone marrow,
and lymphatic system and include leukemia, lymphoma, and myeloma.
Classification of Solid Tumor
There are hundreds of different cancers and several methods of classification.
The various methods include by site of origin, by histological (tissue) type, and
by stage and grade. Classification by site of origin simply names the primary,
specific type such as lung cancer, prostate cancer, and liver cancer. The
International Classification of Diseases for Oncology, Third Edition (ICD-O-3)
groups cancer into six categories based on histological type: carcinoma,
sarcoma, myeloma, leukemia, lymphoma, and mixed types. Carcinomas are
cancerous tumors that arise in epithelial tissues of skin, blood vessels, and lining
of the cavities and organs. Carcinomas account for 80% to 90% of all cancers.
Sarcomas are cancerous tumors that arise in supportive and connective tissues
including bones, tendons, cartilage, muscle, and fat. Myeloma is a malignant
tumor that originates in the plasma cells of bone marrow. Leukemia, which
means “white blood” in Greek, is a cancer that begins when the blood-forming
cells of the bone marrow and other blood-forming organs create an excess of
abnormal white blood cells (WBC). Lymphoma is a cancer that develops in the
glands or nodes of the lymphatic system causing abnormal cellular reproduction
of WBC called lymphocytes, which are a vital part of the immune system.
Mixed-type cancer is cancer that has different tissue types or components from
more than one type of cancer.
Staging of Solid Tumor
Solid tumors are staged to describe the severity of the cancer based on the size of
the primary tumor and whether or not it has spread in the body. The TNM
classification of malignant tumors (TNM) is the most widely used cancer staging
system (Table 27.1). This system was developed, and is maintained, by the
American Joint Committee on Cancer (AJCC). “TNM” is the abbreviation for:
the size of the tumor (T), the involvement of nearby lymph nodes (N), and the
presence of metastasis (M). This provides a quick and standardized method for
understanding the involvement of the cancer. Healthcare providers use cancer
staging to assess prognosis and determine treatment(s). Cancer staging is used in
research to determine efficacy of treatments, compare treatment centers, and as a
means of cancer surveillance and control.
TABLE 27.1 TNM Classification

After the values for TNM have been determined, the cancer is assigned an
overall stage. Staging groups use roman numerals and range from I through IV
(plus 0). Certain cancers are further defined using the letters A and B. The higher
cancer stages (III and IV) are associated with more advanced cancer and poorer
prognosis compared to the lower cancer stages (I and II).
Stage 0: carcinoma in situ; early in development, not all cancers have stage 0
Stage I: localized cancer; often has a good prognosis Stage II and III: locally
advanced; criteria for II and III dependent on type of cancer Stage IV:
metastasized; cells have become undifferentiated
It is important to note that cancers are staged when they are first diagnosed and
the stage does not change over time, even if the cancer shrinks, progresses,
becomes metastatic, or recurs after remission. This information is simply added
to the diagnosis. For example, a 76-year-old male with stage II prostate cancer
has progression of disease despite treatment. An example of an updated
diagnosis would be “Stage II prostate cancer with metastatic disease to the
pelvis.” This is to facilitate an improved understanding of treatments by stage
and associated survival rates. On a rare occasion, the cancer might be restaged to
determine the response to treatment. If this is done and the new stage is used, a
lower care “r” will appear in front of the new stage.
Classification and Staging of Liquid Tumor
Liquid tumors, also referred to as blood cancers, include leukemia, lymphoma,
and myeloma. Liquid tumors arise from and affect the bone marrow, blood cells,
and the lymphatic system. Myeloblasts and lymphoblasts are immature blood
cells in the bone marrow. Myeloblasts develop into granulocytes and
lymphoblasts develop into either B or T lymphocytes. When there is
malfunctioning or errors within these processes, immature blood cells may be
released into and accumulate in the peripheral blood. Myeloblasts in the
peripheral blood leads to myleoblastic leukemia, and lymphoblasts in the
peripheral blood leads to lymphocytic leukemia. The term “blasts” is often used
as a shortened version, indicating immature blood cells found in the peripheral
blood, and reported in percentages.There are a variety of classification systems
and staging criteria for liquid tumors, each of which is dependent upon the
disease type and often includes subtypes. The following section provides an
overview of classification and staging for liquid cancers.
Leukemia may be either acute or chronic. Acute leukemia is often classified
using a cellular or cytologic level. The classification system that is most widely
used is the French-American-British (FAB) system. This system divides acute
leukemia into eight subtypes of acute myelogenous leukemia (AML) and three
subtypes of acute lymphocytic leukemia (ALL). The World Health Organization
(WHO) classification uses chromosome translocations and evidence of
dysplasia. Other factors included in the staging are number of myeloblasts
(immature WBC) found in the blood or bone marrow.
Chronic lymphocytic leukemia (CLL) is frequently staged in the United
States using the Rai staging system, while the Binet system is widely used in
Europe. The Rai system divides CLL into five stages based upon absence or
enlargement of the lymph nodes, spleen, or liver as well as the counts of red
blood cells (RBC) and platelets. All stages have lymphocytosis or an increase in
the number of lymphocytes in the blood. The five Rai stages are as follows:
Stage 0: low risk
Stage I: intermediate risk
Stage II: intermediate risk
Stage III: high risk
Stage IV: high risk
Chronic myelogenous leukemia (CML) typically develops and progresses
slowly, over months or years, even without treatment. CML is divided into three
phases dependent upon clinical manifestation, laboratory values, and the
percentage of blood and bone marrow blasts. The chronic phase typically lasts
four to five years although some people have remained in the chronic phrase for
more than 20 years. Many people do not experience any symptoms and the
diagnosis is often by chance during blood testing for an unrelated reason,
reveling blasts of less than 10%. Treatment during this phase is through the use
of tyrosine kinase inhibitor (TKI) which targets the oncogene BCR-ABL. This
gene is not found in normal cells and it causes the growth of CML. Over several
years, CML progresses to an accelerated phase during which the disease process
speeds, leukemia cells accumulate more quickly, and the blood and bone marrow
have 10-19% blasts. This phase typically lasts from 6-24 months before
progression. Treatment depends upon treatments received thus far. An allogeneic
stem cell transplant may be the best option for those eligible. The final phase of
CML is the blast crisis, which often behaves as an acute leukemia. The blood
and bone marrow contain 20% or more blasts and symptoms are more severe.
People in blast crisis often have fevers, an enlarged spleen, weight loss, and
report a general feeling of being unwell. Treatment includes chemotherapy,
however success is difficult at this stage with fewer people reaching remission,
and remissions lasting shorter periods of time. Many times people in the blast
crisis of CML cannot be cured therefore, palliative treatments may be used to
alleviate bothersome symptoms including radiation to reduce pain from areas of
bone damage and to shrink an enlarged spleen. Approximately 85% of patients
are diagnosed in the chronic phase (Tefferi, 2006).
Lymphoma, the most common blood cancer, occurs when a subset of WBC
called lymphocytes grow abnormally. Lymphocytes are a vital part of the
immune system and are divided into B and T cells. Dependent upon the specific
lymphocyte involved, lymphoma is classified as either Hodgkin lymphoma (HL)
or non-Hodgkin lymphoma (NHL). Although the growth rate and prognostic
factors are not taken into account, the Ann Arbor staging is commonly used for
staging lymphoma. There are four stages as follows:
Stage I: one nodal group or lymphoid region Stage II: two or more nodal groups,
same side of the diaphragm Stage III: nodal groups on both sides of the
diaphragm Stage IV: spread beyond the lymph nodes, most often to the liver,
bone marrow, or lungs
Many oncologists consider NHL stages III and IV as one category as
treatment and prognosis do not differ.
Myeloma begins in the bone marrow and is a cancer of a different type of
WBC, the plasma cells, or plasma B cells. There are four types of myeloma:
Multiple myeloma: the most common, constituting approximately 90% of all
myeloma Plasmacytoma: only one site of myeloma cells evident Localized
myeloma: found in one site with extension into surrounding tissue
Extramedullary myeloma: involves tissue other than the bone marrow such as
skin
Myeloma may be considered asymptomatic (smoldering) or symptomatic.
Asymptomatic myeloma progresses slowly and there are no symptoms, while
symptomatic myeloma progresses quicker and has related symptoms including
anemia and kidney damage.
Etiology
By definition, cancer is a disease of the genes. A gene is a distinct portion of
deoxyribonucleic acid (DNA) that encodes, or instructs, the production of
proteins and ribonucleic acid (RNA), which in turn form the basis for how our
body grows and functions. Cells in our body continually grow, divide, and
replace themselves, which may lead to potential errors in coding or damage to
the DNA. Likewise, factors from the environment may damage DNA. The DNA
repair process allows the cell to identify and correct such damages. If the
damage is not repaired, the gene becomes mutated, which means the change is
permanent, and this may cause the production of faulty proteins. As the
abnormal cell continues to divide uncontrollably, it forms a neoplasm or tumor.
These gene mutations and damage to DNA that cause cancer are attributable to
factors within three general categories: genetic inheritance, environmental
carcinogens, and lifestyle choices. It is important to remember that research in
the field of oncology continues to add evidence to our understanding of the
etiology, the risk factors, and the best interventions for cancer. The following
information represents the most current understanding in the field of oncology at
the time of this writing.
Genetics
The National Institutes of Health (NIH), National Cancer Institute (NCI), reports
that inherited mutations in a person’s chromosomes, genes, or proteins play a
role in only 5% to 10% of cancers, while the majority of cancers (90% to 95%)
are attributable to environmental carcinogens and/or lifestyle choices. There are
some types of cancers that tend to recur in some family generations such as
breast, colon, ovarian, and uterine. Current research suggests that it is possible to
inherit certain genes that increase an individual’s susceptibility to certain cancers
such as early-onset breast cancer (National Cancer Institute, 2015d).
Environmental Carcinogens
The International Agency for Research on Cancer (IARC), a part of the WHO,
the United States National Toxicology Program (NTP), and the United States
Environmental Protection Agency (EPA) have ongoing efforts to identify and
educate on carcinogens. While all three of these agencies have methods of
classification of agents, the most widely used system is from the IARC:
Group 1: Carcinogenic to humans
Group 2A: Probably carcinogenic to humans Groups 2B: Possibly carcinogenic
to humans Group 3: Unclassifiable as to carcinogenicity in humans Group 4:
Probably not carcinogenic to humans
The IARC has evaluated over 900 agents and placed them in one of the above
groups. Most are listed within Groups 2A, 2B, and 3 due to the difficulty in
testing. There are over 100 agents placed in group 1, known to be carcinogenic
to humans. It is important to note that carcinogens do not cause cancer at all
times, under all circumstances. Sometimes the agent is only carcinogenic when
exposed in a certain manner such as touching versus ingesting. Others may only
cause cancer in the setting of specific genetics. Sometimes, an agent is
carcinogenic after brief exposure, while others take repeated exposure. For
specific details, readers are encouraged to visit the IARC, NTP, and EPA Web
sites directly.
Radiation
There are several different types of radiation, some of which increase the risk of
cancer. The electromagnetic spectrum of radiation ranges from low frequency to
high frequency. The lower frequencies, or radiofrequency (RF), are nonionizing.
This means there is enough energy to move atoms in a molecule enough to
vibrate, but not enough to ionize. Microwaves, radio waves, full-body scanners,
cell phones, and cell phone towers are all forms of low-frequency, nonionizing
radiation. As such, they do not have enough energy to cause cancer by directly
damaging cellular DNA. The higher frequencies, with higher energy, are
ionizing radiation. Radiation on this end of the electromagnetic spectrum has
enough energy to change an atom or molecule by removing an electron. This
level of energy is able to damage the DNA inside the cells, thus increasing the
risk of cancer. Gamma rays, x-rays, and ultraviolet (UV) waves from sunlight
are examples of ionizing radiation.
Ultraviolet Radiation
The sunlight that reaches the surface of Earth is made of two types of rays, both
of which are harmful: long-wave ultraviolet A (UVA) and short-wave ultraviolet
B (UVB). These rays cause premature aging of the skin, damage to the eyes
leading to low vision and cataracts, and they cause skin cancer. This damage
occurs because of the changes to the cellular DNA and resulting mutations. The
WHO lists UV radiation as a proven human carcinogen, as well as the leading
cause of basal cell carcinoma (BCC), squamous cell carcinoma (SCC), and
nonmelanoma skin cancers (NMSC) (Lucas, McMichael, Smith, & Armstrong,
2006). Artificial UV-emitting devices, such as tanning beds and sun lamps, are
also known to be a human carcinogen. Studies have shown the dose of UVA
emitted from tanning beds to be as high as 12 times of that of the sun. People
who use tanning beds are 2.5 times more likely to develop SCC and 1.5 times
more likely to develop BSC (Lucas et al., 2006).
Air Pollution
Air pollution occurs as a result of introducing contaminants into the atmosphere.
The most common cause of air pollution is exhaust from transportation and
factories. While air pollution had previously been recognized as a factor that
increases respiratory and heart diseases, data from the WHO’s Global Burden of
Disease Project showed 223,000 deaths from lung cancer associated with air
pollution worldwide. Following these findings, in 2013, the IARC classified
outdoor air pollution as a carcinogen (Simon, 2013). The following highlights
five common air pollutions that are carcinogenic.
Secondhand Tobacco Smoke

Secondhand tobacco smoke, or passive smoking, includes both the smoke given
off by the burning tobacco product and the exhaled smoke from the smoker and
is classified as a known human carcinogen. There have been more than 7,000
chemicals identified in tobacco smoke, including more than 250 harmful
substances. The known carcinogens in tobacco smoke include arsenic, benzene,
beryllium, carbon monoxide, ethylene oxide, hydrogen cyanide, and polonium-
210. The known carcinogens in tobacco smoke are directly linked to cancer of
the lung, esophagus, larynx, mouth, throat, kidney, bladder, liver, pancreas,
stomach, cervix, colon, rectum, and acute myeloid leukemia. It may also play a
role of increasing the risk of other cancers including breast cancer and skin
cancer (National Cancer Institute, 2015g).
Benzene
Benzene is widely used as a starting chemical for the production of plastics,
rubbers, dyes, drugs, and pesticides. It is present in motor vehicle exhaust and in
cigarette smoke. Benzene is known to cause blood cancers, in particular
leukemia, through damaging the DNA in marrow cells (Leukemia & Lymphoma
Society, 2015).
Diesel Engine Exhaust

Diesel engine exhaust is now classified as a known human carcinogen. Research
has shown an increase in the lifetime risk of lung cancer and has linked diesel
engine exhaust to an estimated 6% of lung cancer deaths annually (Vermeulen et
al., 2014). The two parts of diesel engine exhaust are gas and soot in which the
substances include carbon dioxide, carbon monoxide, nitric oxide, nitrogen
dioxide, and polycyclic aromatic hydrocarbons (PAHs) (American Cancer
Society, 2015d).
Asbestos
Asbestos, a naturally occurring group of minerals, has been widely used in the
United States since the late 1800s. Uses have included building materials, ceiling
and floor tiles, paint, plastics, vermiculite-containing garden products, and
insulation. When the tiny fibers of asbestos are disturbed and released into the
air, they can easily be breathed, allowing the fibers to accumulate in the lungs.
Regular exposure to asbestos greatly increases the risks of both lung cancer and
mesothelioma, a cancer in the membranes lining the chest and abdomen
(National Cancer Institute, 2015a). The EPA banned all new use of asbestos in
1989.
Radon
Radon is a naturally occurring radioactive gas with no color, no odor, and no
taste. As radon gas in the air breaks down, it creates radioactive elements called
radon progeny. Breathing these elements allows them to become lodged in the
lining of the lungs where they can give off radiation. While cigarette smoking is
the leading cause of lung cancer in the United States, radon is the second leading
cause (United States Environmental Protection Agency, 2015).
Lifestyle
Research has shown that a significant number of cancers may be prevented with
improved lifestyle choices (Anand et al., 2008). The lifestyle choices that
contribute to cancer include use of tobacco, excessive alcohol, increased weight,
physical inactivity, poor diet, and contracting infectious agents.
Tobacco Use
Tobacco, whether it is smoked or smokeless, is a carcinogen. Of the estimated
589,430 cancer deaths in the United States in 2015, smoking tobacco caused
approximately 171,000. This includes those smoking cigarettes and cigars and
those exposed to passive or secondhand smoke (National Cancer Institute,
2015g). Of all lung cancers, 80% to 90% occur in smokers. Researchers have
shown that smoking also contributes to cancers in the upper respiratory tract, the
esophagus, and the larynx along with the bladder and pancreas. Liver, stomach,
and kidney cancers have also been linked to smoking (Centers for Disease
Control and Prevention, 2015).
Excessive Alcohol
While there is evidence that alcohol in small amounts may have health benefits,
such as reduction of the risk of heart disease, excessive or heavy use of alcohol
negates these benefits and increases the risks of cancers (American Cancer
Society, 2015a; National Institutes of Health: NIAAA, 2000; Vincenzo,
Blangiardo, LaVecchi, & Corrao, 2015). The generally accepted definition of
moderate alcohol use is up to one drink per day for women and up to two drinks
per day for men. Excessive, or heavy, alcohol use is defined as five or more
drinks on a single occasion, and 5 or more days in the past 30 days. A meta-
analysis of over 200 studies showed consistent evidence that heavy alcohol use
significantly increased risks of cancer involving the mouth, pharynx, larynx, and
esophagus (Bagnardi, Blangiardo, La Vecchia, & Corraro, 2001). Heavy use of
alcohol also increases the risk of cancer involving the stomach, colon, rectum,
liver, breast, and ovaries (American Cancer Society, 2015a). The component that
increases the risk of cancer is the alcohol itself, chemically known as ethanol or
ethyl alcohol, not the other ingredients. The structural formula for ethanol is
CH3CH2OH and is abbreviated as EtOH. Different types of beverages have
different levels of ethanol within them; however, as far as the body is concerned,
all alcohol is the same at the unit level. As the risks of cancer are attributable to
the ethanol, the greater amounts of ethanol have higher associated risks
(Bagnardi et al., 2001). Along with alcoholic beverages, ethanol is also used as
an additive for gasoline, as a solvent in the manufacturing of varnishes and
perfumes, and as a disinfectant. Ethanol is a neurotoxic psychoactive drug that
has direct effects on the nerve cell. Additionally, once ingested, ethanol in the
liver metabolizes into acetaldehyde, a toxic chemical. Acetaldehyde encourages
liver cells to grow faster, increasing the risk of errors that could lead to
mutations in DNA. When alcohol is used in combination with tobacco, the risk
significantly increases for cancers involving the mouth, pharynx, larynx, and
esophagus, well above the risk levels associated with either tobacco or alcohol
alone (Bagnardi et al., 2001; National Institutes of Health: NIAAA, 2000). This
stems from tobacco and ethanol working in tandem. For example, ethanol
increases the absorption of tobacco into the linings of the mouth and esophagus,
increasing the exposure of those areas to the harmful effects of tobacco.
Increased Weight
Each year, close to 500,000 new cancer cases worldwide are associated with
obesity (Arnold et al., 2015; International Agency for Research on Cancer,
2002). Obesity is associated with cancers involving the breast, ovaries, uterus,
pancreas, gallbladder, prostate, colon, and rectum (Key et al., 2004). Obesity is
defined by a body mass index (BMI) of 30 or higher. Researchers have proposed
several possible mechanisms to explain the link between obesity and cancer
(National Cancer Institute, 2015f):
Obesity causes a chronic low-level inflammation, which is associated with
increased risk of cancer.
Obesity leads to increased levels of insulin and insulin-like growth factor in the
blood, which may contribute to the growth of certain tumors.
Fat cells produce hormones that may stimulate or inhibit cell growth.
Fat tissue produces excess amounts of estrogen, and high levels of estrogen are
linked with breast and endometrial cancers.
Physical Inactivity
The NCI reports that people who exercise on a regular basis have a 40% to 50%
lower risk of colon cancer and a 30% to 40% lower risk of breast cancer
compared to people who do not exercise on a regular basis. The WHO reports
that sedentary lifestyles increase all causes of mortality including the risk of
colon cancer and the risk of obesity. Physical inactivity leads to slower bowel
function, increasing the length of time the colon is exposed to potential
carcinogens. When coupled with tobacco use and poor diet, physical inactivity is
contributing to the rise of complications and mortalities with chronic diseases
including cancer (World Health Organization, 2002). Research also supports
regular exercise leading to a lower risk of lung cancer and prostate cancer.
Poor Diet
Approximately 35% of all cancers are directly related to dietary causes (Stadler,
2009) and include cancers of the breast, ovaries, uterus, gallbladder, pancreas,
colon, rectum, and prostate (World Health Organization, 2011). Poor diet
choices, which increase the risk of cancers, include foods containing high fat and
oxidized fats. The chemicals produced by certain meat preparation methods
including charcoaling, smoking, and grilling may also increase the risk of cancer.
Chemicals called PAHs are emitted from combustion of charcoal, gas, and wood.
Research has shown that lab animals exposed to PAHs had higher risks of
developing cancer in the skin, liver, and stomach (Gehle, 2009). More recently,
researchers at Oregon State University discovered compounds that are produced
by certain chemical reactions that are hundreds of times more mutagenic than the
parent compounds of PAHs (Mishamandani, 2014). When meat is exposed to
high heat, a chemical reaction occurs involving the released meat nitrates and the
PAHs, forming nitrated PAHs (NPAHs). This research showed the mutagenic
abilities of NPAHs to be as much as 432-fold increase over PAHs. Processed
meat products contain sodium nitrite as a preservative and colorant. When
sodium nitrite is exposed to high temperatures, it combines with proteins called
amines and forms nitrosamines, most of which are carcinogenic (International
Agency for Research on Cancer, 2015). Nitrosamines are also found in pickled
and salted foods. Recent studies have linked smoked meats to an increased risk
of colorectal, pancreatic, and prostate cancer (National Cancer Institute, 2015b).
Current research continues to study the risk of cancer and the role of PAHs,
NPAHs, sodium nitrites, nitrosamines, and other chemicals associated with the
processing of food.
Infectious Agents
Research over the past decades has shown that approximately 15% of cancers
are attributable to viruses (Liao, 2006; National Cancer Institute, 2015e)
including human papillomavirus (HPV), hepatitis B virus (HBV), hepatitis C
virus (HCV), and human immunodeficiency virus (HIV) (Table 27.2).
TABLE 27.2 Infectious Agents

Aging and Cancer
One of the greatest risk factors for cancer is aging (Hurria et al., 2010). Research
currently speculates that increased risk of cancer from aging may be a result of
having a greater length of time for exposure to factors that damage DNA, and it
may be also the aged tissues’ potential decreased ability to adequately complete
the DNA repair process, thus creating a cellular environment that has increased
susceptibility to mutations within an immune system that is less capable of
responding (Hurria et al., 2010; Ukraintseva & Yashin, 2003).
Survivorship, a common term referring to individuals who have been diagnosed
with cancer, begins at the time of diagnosis and continues through the person’s
end of life. In 2015, there were approximately 14.5 million cancer survivors in
the United States (National Institutes of Health: Surveillance, Epidemiology, and
End Results Program, 2015). The average lifetime risk of developing cancer in
the United States is one in two men and one in three women. It is expected that
1,658,370 new cases of cancer will be diagnosed in 2015, and this does not
include carcinoma in situ. The most common types of cancer are prostate, breast,
and lung (American Cancer Society, 2015b) (Table 27.3).
TABLE 27.3 Leading Sites of New Diagnoses—2015 Estimates
Source: American Cancer Society, 2015 Fact Sheet.

Adolescent and Young Adult Cancer (Ages 0 to 14
Years)
Approximately 10,380 new cases of childhood cancer were diagnosed in 2015 in
the United States, representing <1% of all new cancer diagnosis. An estimated
1,250 deaths in 2015, childhood cancer is the second leading cause of death
behind accidents (American Cancer Society, 2015c). Leukemia represents
approximately 25% of all childhood cancers, while brain and other central
nervous system tumors represent 24% (Kupfer, 2015).
Older Adult Oncology (Ages 65 Years and Older)
In 2010, approximately 40 million people living in the United States were aged
65 years or older, representing 13% of the general population (Agency for
Healthcare Research and Quality, 2015). The growth of the population of older
adults is projected to continue increasing such that by 2030 approximately 72
million or 20% to 25% of the general population (Agency for Healthcare
Research and Quality, 2015). As the incidence of cancer increases with age, the
majority of cancers are diagnosed in persons aged 65 years and older (Tariman,
2009; Ukraintseva & Yashin, 2003; National Institute on Aging, 2015). Rates of
cancer for aged 65 and older are as follows: males 4× greater and females 2×
greater than those aged 45 to 64 years (Baranovsky & Myers, 1986; National
Institute on Aging, 2015). As aging is complex and each person ages at a
different rate, successful cancer treatment for older adults requires an
understanding of how the natural aging process, physiological age,
comorbidities, level of function, and the expectations for treatment all play a
role. For example, age-associated decline in kidney function leads to a decrease
in the body’s ability to clear medications (Weinstein & Anderson, 2010). This
increases chemotherapy toxicity limits, which causes dosing to be lowered
compared to similar diagnoses in a younger population (Hurria et al., 2010). As a
result, the overall effectiveness of treatment is lowered. Likewise, cardiac, lung,
and neurological comorbidities increase the risk of adverse effects of
chemotherapy and radiation. Additionally, older adults tend to have rapid
progression of disease, a poorer tolerance to neutropenia, shorter remissions, and
a higher likelihood of complications (Hurria et al., 2010). However, older adults
who are healthy, active, and have fewer comorbidities have equal benefit with
equal treatments (Berger et al., 2006; Warburton, Nicol, & Bredin, 2006).
Signs and Symptoms
The signs and symptoms of cancer will depend upon the location and the size of
the cancer, as well as whether the cancer is affecting nearby tissues or organs.
General signs and symptoms of cancer include an unexplained weight loss,
lingering fatigue, coughing blood, persistent headaches, chronic pain, persistent
low-grade fever, skin changes, and repeated infections. The American Cancer
Society uses the acronym “CAUTION” as a reminder of the general symptoms
of cancer. It is important to remember that there are many other diseases that
could cause these symptoms; therefore, it is advisable to seek medical advice to
determine the underlying problem.
Change in bowel or bladder habits A sore that does not heal
Unusual bleeding or discharge (blood in urine or stools) Thickening or lump
in the breast, testicles, or elsewhere Indigestion or difficulty swallowing
Obvious change in the size, color, shape, or thickness of a wart, mole, or
mouth sore Nagging cough or hoarseness
The NIH considers cancer a chronic disease model and places emphasis on
recognizing not only the effects of cancer itself but also the short- and long-term
effects of the treatments, all of which impact quality of life. The course and
prognosis of cancer depends upon the type of cancer, the location and stage of
the cancer, the age of the individual and his or her premorbid health, and how
well the individual responds to treatment. Statistics are used to predict the future
course and outcome of cancer, as well as the likelihood of recovery. Five-year
survival rates are commonly used measures. This refers to the proportion of the
people expected to be alive, 5 years after the initial diagnosis, compared with a
demographically similar population that is without cancer. While the survival
rates for many cancers have continued to improve, cancer overall remains the
second leading cause of death following heart diseases (Table 27.4).
TABLE 27.4 Risk of Mortality 2013
Source: US Mortality Data 2013, National Center for Health Statistics, Centers for Disease Control and
Prevention, 2015.
Diagnosis
The process of diagnosing cancer involves several steps. These steps may begin
following a routine screening that produces abnormal results or if an individual
presents with lingering complaints. After gathering a recent medical history, the
physician conducts a full examination to look for abnormalities in color, texture,
lumps, swelling, tenderness, or thickening of tissue. Laboratory tests of blood,
urine, and/or other body fluids are frequently ordered. For concerns of solid
tumor, initial imaging may be conducted using computed tomography (CT)
scans, magnetic resonance imaging (MRI), or ultrasound. Nuclear medicine
scans such as positron emission tomography (PET) scans and bone scans are
used to diagnose, stage, and monitor cancer. PET scans focus on the organs of
the body, while bone scans are specific to detecting bone involvement. The
process of having a nuclear medicine scan begins with a small amount of
radioactive substance, typically injected into a vein, that is absorbed by organs
and tissues throughout the body. Cancer cells tend to use more energy than
healthy cells and thus absorb more of the radioactive substance. The nuclear
scan image shows those areas of high radioactive tracer uptake (Fig. 27.1). A
tissue biopsy may be conducted by removing a small portion of tissue from the
area in question. Experts then review the samples under a microscope to
determine if cellular changes are suggestive of cancer. At times, the physician
may recommend genetic testing for known genetic mutations linked to particular
cancers. For example, three abnormal genes, BRCA1, BRCA2, and PALB2, are
inheritable genes with a mutation that significantly increases the risk of breast
cancer in women (National Cancer Institute, 2015c).
Figure 27.1 Bone scan. Increased uptake is seen at the left iliac crest in the
setting of osteosarcoma.
Medical Management
The primary goals of cancer treatment are to remove all or as much as possible
of the tumor and prevent recurrence or spread of the primary tumor. The
recommended course of treatment(s) is determined on an individual basis and
with consideration for balancing the likelihood of curing the cancer with the
anticipated side effects of the treatment, as well as the patient’s preference.
There are situations when the cancer is identified at a late stage and is widely
metastatic or when the primary cancer is aggressive and cure is not possible, at
which point the focus becomes relieving symptoms and controlling the cancer.
Surgery
Surgery is used to diagnose, stage, treat, and/or palliate solid tumors and is the
most commonly used cancer treatment for solid tumors. There are several types
of surgeries:
Preventive: to keep cancer from occurring. Examples of preventive surgery are
removal of precancerous polyps or a mastectomy for a high-risk person.
Diagnostic (biopsy): removing some, or all, of the tumor for examination. This
can be done through a fine needle aspiration (FNA), incisional, or excisional
biopsy.
Staging: to determine the extent of the cancer. This is sometimes completed
through endoscopic or laparoscopic procedures.
Curative: the removal of cancerous tumor. This is most effective on localized
cancer and is often followed by radiation and/or chemotherapy.
Supportive: to help with other cancer treatments. An example of supportive
surgery is placing of a port or an Ommaya reservoir for chemotherapy treatment.
Palliative: is not meant to cure or prevent. The purpose of a palliative surgery is
to ease pain or disability and improve quality of life. Tumor debulking to
alleviate symptoms in gastrointestinal cancer is an example of a palliative
surgery.
Surgical interventions may damage nerve and muscle tissue, potentially leading
to weakness and sensory changes. There may also be lingering complaints of
pain. While these changes are typically localized to the surgical area(s), if left
untreated, over time the individual may self-restrict his or her daily routines
and/or roles, leading to a decline in function and in overall quality of life.
Radiation Treatment
Radiation treatment (XRT) may be used as curative, adjuvant (in addition to the
primary treatment to maximize effectiveness), neoadjuvant (given before the
primary treatment to reduce the size or extent of cancer), or palliative (to relieve
symptom burden). There are two methods of receiving radiation: internally and
externally. Internal radiation, known as brachytherapy, involves the placement of
a radioactive substance in a pellet or liquid into or at the cancerous site. External
radiation involves aiming the beam at the tumor from outside the body. This is a
multistep process including imaging to accurately locate the targeted area, a
simulation session to accurately mark the body and detail positions for the
machine, and the radiation treatments. The ionizing radiation damages the
cellular DNA of the cancerous cells, leading to cell death. The radiation beams
are strategically placed in order to focus the larger absorbed dose on the tumor.
The surrounding healthy tissue is exposed to radiation as well, but in much lower
doses. Radiation side effects are classified as early adverse effects and late
adverse effects. Early adverse effects are proportional to the dosing of radiation
and include changes to radiosensitive tissues such as oral mucosa, stomach,
small bowel, colon, vagina, and lymph nodes. Fatigue is a common complaint
that worsens as treatments progress. Erythema, a reddening of the skin, is
common across the radiation site(s). Late adverse effects can take months to
years to develop and include changes and tissue fibrosis to the radiated areas and
changes to the lymph vessels increasing the risk of lymphedema. Secondary
cancers are also possible. It is very important for individuals who received
radiation across their abdominal region to remain mindful of maintaining regular
bowel movements and using stool softeners, especially with any constipating
medications such as opioids. Constipation and passing hard stool through tissues
weakened by effects of radiation may lead to a fistula. A fistula is an abnormal
connection or pathway between structures caused by disease, injury, or surgery.
In this example, the bowel becomes weakened by radiation and cannot retain
structural integrity, forming a fistula. A common treatment for a fistula involving
the bowel is a colostomy, which may become permanent. Although developing a
fistula is not always preventable, care should be taken to decrease the risk.
Chemotherapy
Chemotherapy is a medicine that may be taken orally, topically, or by injection
intra-arterial (IA), intravenous (IV), or intraperitoneal (IP). There are over 100
chemotherapy agents, often used in combination. Induction chemotherapy, used
to induce a remission, is commonly used to treat acute leukemia and is typically
given while admitted to the hospital. Chemotherapy may also be administered on
an outpatient basis. Chemotherapy works on cells that grow and divide quickly.
As cancer cells divide more frequently than normal cells, they are more affected
by the chemotherapy. However, other cells that grow and divide quickly are also
affected including those that line the mouth and intestines and those that cause
hair to grow. This leads to many side effects, of which fatigue is the most
common. Other side effects may include nausea, vomiting, constipation,
diarrhea, taste changes, appetite changes, cognitive dysfunction, bleeding
problems, anemia, hair loss, sun sensitivity, and an increased risk of infection.
Chemotherapy-induced peripheral neuropathy (CPIN), a side effect of
chemotherapy that causes altered sensations of tingling, numbness, and
weakness impacting primarily the hands and/or feet, occurs as chemotherapy
spreads throughout the body, damaging the distal portions of the nerves. These
symptoms may occur immediately following chemotherapy or weeks to months
later and typically begin distally, progress proximally, and are bilateral.
Symptoms often get worse as treatments progress. Chemotherapy is a
cardiotoxic agent leading to long-term cardiac changes including heart failure,
arrhythmias, and hypertension. Individuals who have undergone chemotherapy
treatment are generally advised to have annual cardiac examinations.
Hormone Therapy
Hormone therapy, often used as an adjuvant therapy, is used for those types of
cancers that are hormone dependent and grow faster in the presence of particular
hormones. This includes prostate, breast, and uterine cancers. By blocking the
production or action of the hormone, the tumor growth is slowed and survival
may be extended for several months or years. Side effects of hormone therapy
are dependent upon the specific hormone; however, general side effects include
tiredness, memory problems, mood changes, thinning hair, and headaches. There
might be complaints of joint pain initially, and some hormones may cause
thinning of the bones.
New Approaches to Treatment
One new focus in cancer treatment is the use of targeted cancer therapy: drugs or
substances that target specific molecules or proteins to block the growth and
spread of cancerous cells. Targeted therapies are different from chemotherapy in
that they work on specific targets associated within the cancerous cell rather than
on rapidly dividing cells. This helps avoid healthy cells from cellular death, thus
decreasing the risk of side effects. Another difference is that targeted therapies
are cytostatic (block cell division) and chemotherapy is cytotoxic (kill the cell).
Immunotherapy, or biologic therapy, uses the body’s own immune system to
destroy cancer cells. This type of treatment is being studied in clinical trials and
is not yet widely available.
Bone Marrow Transplantation
Bone marrow transplant (BMT) is a procedure in which the damaged or
destroyed stem cells are replaced with healthy stem cells. Stem cells are
primarily found in bone marrow but may also be harvested from peripheral
blood or umbilical cord blood from newborns. For purposes of clarification,
stem cell transplant (SCT) refers to a transplantation using peripheral blood stem
cells; the procedure itself is still referred to as BMT. Bone marrow, the spongy
center of certain bones, forms three types of blood cells: RBC, WBC, and
platelets. RBC, also called erythrocytes, carry oxygen throughout the body and
bring carbon dioxide back to the lungs. WBC, also called leukocytes, help fight
infection. Platelets, also called thrombocytes, assist in the clotting factor of
blood. Lymphocytes, also formed in the bone marrow, work with the lymphatic
system to help fight infections. Cancer occurs when stem cells become damaged
and start producing abnormal cells or too few blood cells. The first BMT
occurred in 1956 between identical twins. Twelve years later in 1968, after
improved understanding of the role of human leukocyte antigens (HLA) and the
importance of matching, the first BMT was performed between siblings. Then in
1973, the first unrelated BMT was performed. There are three types of BMT:
autologous BMT, allogeneic BMT, and umbilical cord blood transplant. In an
autologous bone marrow transplant, stem cells are removed from the
individual while in remission from cancer and are kept frozen. After the
individual undergoes high-dose chemotherapy or radiation, those stem cells are
replaced to begin making new blood cells. Advantages of autologous BMTs
include the following: there is no need to locate a donor; the individual receives
his or her own stem cells and is not at risk of receiving an infection from the
donor, nor is there a risk of the graft attacking the body (graft vs. host). A
disadvantage is the increased rate of relapse or recurrence of the original cancer.
In an allogeneic bone marrow transplant the individual undergoes high-dose
chemotherapy or radiation and then receives stem cells from a HLA-matched
donor. Umbilical cord transplants are a form of allogeneic in that the stem cells
are from another individual (donor), in this case a newborn baby’s umbilical
cord. In an allogeneic BMT, the stem cells make their own immune cells, which
could help destroy any remaining cancerous cells. This is called graft versus
cancer, a preferable outcome. Graft versus host disease (GVHD) is a
significant disadvantage in allogeneic transplants and occurs when the newly
formed immune system views the recipient’s body as foreign and attacks
systems and/or organs. The most common GVHDs involve the gastrointestinal
system, the skin, and the liver (National Cancer Institute, 2015h). GVHD is
considered acute if it begins within the first 100 days posttransplant, chronic if it
begins after. This complication requires hospitalization with close monitoring,
and the treatment often includes immune-suppressing medications, steroids, and,
if the gastrointestinal system is involved, artificial nutrition. As a result, the
survivor is at high risk of an overall decline in physical and cognitive abilities as
well as from a psychosocial standpoint.
Oncologic Emergencies
Oncologic emergencies are defined as an acute and potentially life-threatening
event caused by cancer or the associated treatments. These emergencies require
immediate intervention to prevent loss of life or quality of life (Lewis,
Hendrickson, & Moynihan, 2011; McCurdy & Shanholtz, 2012).
Tumor Lysis Syndrome
Tumor lysis syndrome (TLS) refers to a group of metabolic disorders that are
potentially life threatening and includes the rapid development of hyperuricemia
(high blood uric acid), hypocalcemia (low blood calcium), and
hyperphosphatemia (high blood phosphorus); symptoms include severe muscle
weakness or paralysis, sudden mental incapacity, extrapyramidal movement
disorders, and myopathy. TLS is common in acute leukemia although it may
occur in other cancers including lymphoma. Higher numbers of blasts, or
abnormal immature WBC, increase the risk of TLS once the person begins
chemotherapy. As the malignant cells go through lysis, breaking down of a cell
membrane, the intracellular contents are released into the systemic circulation
and may exceed the abilities of the kidneys leading to this metabolic emergency.
Hyperleukocytosis and Leukostasis
Hyperleukocytosis is a high leukocyte (WBC) count, and this condition can
cause leukostasis, which is a clumping or sludging of the microcirculation that
tends toward clotting. This oncologic emergency occurs more frequently in
AML. Impairment in vascular flow and local hypoxemia is common in the
pulmonary and cerebral vascular systems. The coronary circulation may also be
affected. Early pulmonary involvement presents as mild dyspnea and respiratory
alkalosis, which is excessive alkaline, causing weakness and cramps. Cerebral
involvement can range from confusion to somnolence to frank intracerebral
bleeding and coma.
Disseminated Intravascular Coagulopathy
Disseminated intravascular coagulopathy (DIC) is an oncologic emergency
encompassing both excessive bleeding and thromboembolic events and is
common in leukemia, in particular acute promyelocytic leukemia (APL). The
characteristic trait is excess thrombin generation. This is an enzyme in blood
plasma that causes clotting by changing fibrinogen to fibrin. The typical
presentation is episodes of thrombosis rather than bleeding; however, there is a
high risk of bleeding from injury. While signs and symptoms of DIC vary as the
clots can occur throughout the body, bleeding is often the first sign of acute DIC
and includes bruising more frequently or more severe than expected, red spots
on the skin, nosebleeds or epistaxis, bleeding from the gums, coughing up blood
or hemoptysis, and dark, tarry stools.
Superior Vena Cava Syndrome
Superior vena cava syndrome (SVCS) is an oncologic emergency resulting in
diminished blood return to the heart, causing symptoms of facial edema, edema
of the neck and upper extremities, dyspnea, cough, and shortness of breath or
orthopnea and is most commonly caused by lung cancer. The initial temporary
measure is to elevate the head and apply supplemental oxygen.
Fever and Neutropenia
When the cancer involves the immune system, or immunosuppressive
chemotherapy as a treatment, patients often have a period of neutropenia, which
is defined as an absolute neutrophil count lower than 500/mm3. Neutrophils are
the most abundant type of WBC, formed from stem cells in the bone marrow,
and are an essential part of the immune system. During neutropenic states, the
immune system becomes very susceptible to infections, particularly from gram-
negative organisms, staphylococci, and fungi.
Metastatic Spinal Cord Compression
Metastatic spinal cord compression (MSCC) occurs when cancer grows on or
near the spine, causing compression on the dural sac and the contents, in
particular the spinal cord. There are approximately 12,700 new cases of MSCC
per year (Quinn, 2000). Compression of spinal cord may be due to growth of a
mass around the vertebrae, growth within the spinal column, or collapse of bone.
The thoracic region is the most common at 70%, lumbar-sacral at 20%, and
cervical at 10% (Dubey & Koul, 2009). Aggressive lymphomas such as Burkitt,
multiple myeloma, and solid tumors with high risk for bone metastases (lung,
breast, and prostate cancers) may lead to spinal cord compression(s). Back pain
is the most common symptom present; neurological changes may or may not be
present. Immediate interventions, no longer than 48 hours following symptoms,
are required to alleviate pressure on the spinal cord with the goal of preventing
permanent neurological changes (George et al., 2008). Interventions include
stabilizing and decompressing surgeries, procedures such as kyphoplasty
(surgically filling the collapsed vertebrae), local radiation, and/or high-dose
steroids. Temporary measures include restricting activity pending stabilization
and/or support of the compromised region(s).
Increased Intracranial Pressure
Mass lesions or obstructions of flow of cerebrospinal fluid by tumor tissue may
cause increased intracranial pressure. Increased intracranial pressure requires
immediate medical intervention; when left untreated, herniation and mortality
may result. The symptoms include complaints of headache, cranial nerve
symptoms, nausea and vomiting, and the onset of seizures. The initial temporary
measure is to elevate the head to 30 degrees higher than the level of the heart.
Altered Mental Status
Altered mental status, always an emergency, may occur as a result from the
primary cancer or from metastatic brain disease. It may also occur from
metabolic changes, infections, or organ failure. The symptoms range from
confusion and decreased attention through delirium and coma.
Neurological Changes
Changes in neurological status may warrant immediate medical attention. Signs
and symptoms include disorientation, complaints of dizziness or light-
headedness, complaints of blurred vision, changes in sensation, and ataxia or
change in motor planning.
Performance
Both cancer and the associated treatments have significant implications across
all client factors and areas of occupational performance (American Occupational
Therapy Association, 2015a, c). Along with optimizing physical, cognitive, and
psychosocial functioning, it is imperative for occupational therapy practitioners
to work with survivors and their family and/or caregivers to help them
understand the changing trajectory and how to live within these changes.
Effective occupational therapy interventions will make a positive impact on the
quality of life through facilitating posttreatment recovery including increasing
functional and cognitive independence, reducing or eliminating pain, instructing
on management of changes to sleep, addressing impacts on sexual abilities and
intimacy, addressing social and psychosocial impacts, assisting with return to
work, and redefining occupational roles (Penfold, 1996; Pergolotti, Cutchin,
Weinberger, & Meyer, 2014; Silver, Baima, & Mayer, 2013; Silver & Balma,
2013; Silver & Gilchrist, 2011). Additionally, patients must maintain the best
level of function possible as a loss of function, increased need for sedentary
time, or increased time in bed may preclude further treatment. The Eastern
Cooperative Oncology Group (ECOG), is one of the common scales used by
oncologists to determine if further cancer treatment is appropriate (Oken, 1983)
(Table 27.5).
TABLE 27.5 Karnofsky Performance Status

Common Effects of Cancer and Treatments
Pain
A systematic meta-analysis review of 40 years of studies showed that across all
cancers, at all stages, >50% of patients experience pain and more than one-third
rated their pain as moderate or severe (Everdingen et al., 2007). The causes of
cancer pain include spinal cord compression, metastatic bone involvement,
surgical pain, side effects of chemotherapy and radiation, psychological
responses to the diagnosis and treatments, and cancer itself such as an expanding
mass pressing on organs. Cancer-related pain syndromes include peripheral
neuropathies, axillary web syndrome (cording), and radiation fibrosis. Pain can
lead to a loss of function in routines and roles due to guarding movements, self-
restriction of activities, and generalized weakness associated with inactivity,
leading to a low quality of life. Occupational therapy plays a vital role as a part
of a multidisciplinary approach to pain management. Interventions include
patient education, training in proactive pain management, safe body mechanics,
neuromuscular re-education, muscle tension reduction training, and pacing
activities (American Occupational Therapy Association, 2015b). Pain must be
assessed during every occupational therapy session using objective measures
such as patient-reported pain ratings, adult nonverbal pain scale, and Wong-
Baker FACES pain rating scale. Documentation must also include the location,
any patient-reported description of the pain such as stabbing or burning, and if
movement leads to an increase or decrease in the complaint(s) of pain. Types of
pain include visceral, somatic, neuropathic, and mixed type. Visceral pain arises
from internal organs and is described as dull, often difficult to pinpoint. Somatic
pain arises from the skin and deep tissues and is generally described as a
musculoskeletal pain with a more precise location. Neuropathic pain arises from
nerve damage and may result in localized complaints of numbness or
hypersensitivity. Accurately understanding the patient’s complaints of pain is
necessary for the development of an appropriate intervention, as the
occupational therapy intervention will vary according to the root cause of the
pain. For example, a patient may have complaints of pain in the general hip and
gluteal region. This may stem from soft tissue tumor burden, spinal cord
compression, or metastatic bone disease. Further questioning and assessment of
the pain, coupled with the known diagnosis, will dictate if it is appropriate to
attempt standing (to don clothing or complete toileting) or if recommendations
should be made for further imaging due to concern for unstable bone. When
uncertain, err on the side of caution.
Compromised Bone
Compromised bone due to metastatic disease or primary bone cancer can lead to
pathological fractures. Almost all cancers can spread to bone, but certain cancers
commonly spread to bone including breast, lung, prostate, thyroid, and kidney.
The liquid cancer multiple myeloma arises from plasma cells in the bone marrow
and frequently causes weakening of bones. Along with facilitating overgrowth of
plasma cells that crowd the normal blood-forming cells, multiple myeloma
interferes with the cells that maintain bone integrity: osteoblasts, which form
new bone, and osteoclasts, which lyse or dissolve bone. Typically, new bone is
being formed while old bone is being dissolved. Multiple myeloma speeds the
process of osteoclasts, thus leading to weaker bones as the bones are broken
down without forming new ones. As cancer spreads to the bone, it causes bone
lesions. Osteoblastic lesions, or blastic lesions, result when cancer increases the
activity of osteoblasts, causing that area of the bone to be harder. Although
harder, the structure is abnormal and these areas break more easily than normal
bone. Prostate cancer typically causes osteoblastic lesions. Osteolytic lesions, or
lytic lesions, occur when greater numbers of osteoclasts are lysing the bone. This
causes areas so weak that the bone can break even under its own weight. Breast,
lung, and multiple myeloma cancers typically cause osteolytic lesions. Bones
that break other than by trauma are considered a pathological fracture.
Pathological fractures are managed differently than traumatic fractures, as
healing is poor due to abnormal bone structure. Internal stabilization is the most
common intervention. If there is a reason that the individual is not able to have
surgical intervention, the occupational therapy plan must include protection for
the unstable area(s), instruction of proper body mechanics to avoid weight-
bearing across the unstable area(s), instruction on the impact to the individual’s
daily routine, and issuance of adaptive equipment and durable medical
equipment as needed. Occupational therapy practitioners may be hesitant to
mobilize patients with known metastatic bone disease, but without mobilization,
the overall functional abilities including participation in their daily routine
declines due to the negative impact of inactivity. Bunting and Shea (2001)
published a study reviewing pathological fractures in 54 patients with metastatic
bone disease who were undergoing rehabilitation. Results showed a total of 16
fractures in 12 patients. Only one occurred during rehabilitation, nine were silent
fractures, and six occurred during rest. The Mirel’s scoring system is a tool used
to guide the management of bones with cancer in order to help prevent a
pathological fracture (Table 27.6). Remember, all pathological fractures were
once not fractured, and intervention including internal stabilization is
considerably easier when impending pathological fractures are identified (Figs.
27.2 and 27.3). This is done through understanding the primary diagnosis and
proper pain assessment.
TABLE 27.6 Mirel’s Scoring
Score each: site, pain, lesion, and size from 1 to 3. Total the score for clinical recommendations.
“Size” is the size/diameter of the lesion in relationship to the size of the bone; look at imaging.
Figure 27.2 Impending pathological fracture. Example wording of an imaging
report: Cortical thinning is present in the proximal humeral metaphysis. There is
no definite associated fracture or soft tissue mass. A second lucent lesion may
also be present in the left posterior lateral fifth rib. Shoulder and elbow joints are
anatomically aligned. Bone mineralization is otherwise normal. Visualized left
lung is clear. Impression: Lytic lesions in the left proximal humerus and left
posterior fifth rib compatible with patient’s known metastatic renal cell
carcinoma.
Figure 27.3 Postoperative intramedullary (IM) rod to prevent pathological
fracture. Example wording of an imaging report: The patient is status post ORIF
with placement of a long intramedullary rod and interlocking screws crossing a
4-cm lytic lesion in the proximal humeral shaft. When compared with the prior
exam, the overall size of the lesion does not appear to have changed. The lesion
shows slight interval thickening of the remaining cortex. No other lesions are
identified in the left humerus. Impression: Multiple lytic lesions as described
above. No increase in size of the above lesions and some increased margination
and perhaps increased new bone formation around the lesions suggest the
possibility of some interval healing or positive response to treatment.
Although continued mobilization is vital to maintain maximum levels of activity

and engagement with their routine and roles, there may be times when
mobilization is no longer safe due to the location of the unstable bone. At this
point, it may be most appropriate to modify the individual’s routine and roles to
a bed level. This requires significant involvement from occupational therapy in
order to successfully educate the caregivers on proper care and proper
positioning and to assist the individual in adjustment of his or her life to a bed
level.
Cancer-Related Fatigue
Cancer-related fatigue (CRF) is a common side effect of cancer and the
associated treatments. CRF impacts anywhere from 40% to 100% of people with
cancer (American Cancer Society, 2015a). CRF is not the same as a regular or
general fatigue as CRF does not get better with rest. Often described as feeling
weak and drained, minimal activity can cause exhaustion such as walking from
the bed to the bathroom or even just getting out of the bed to eat. The level of
fatigue can vary from day to day and throughout each day. CRF can last for
months and years after treatment ends (American Cancer Society, 2015a). The
hazard with CRF is the tendency for survivors to decrease daily activity, restrict
roles, and become inactive, directly and negatively impacting the overall quality
of life. Additionally, decreased functional status may preclude the survivor from
receiving continued cancer treatments such as chemotherapy. CRF is currently
understood to be a combination of cancer and the treatments. Other factors that
may contribute include low levels of certain blood cells, stress, pain, poor sleep,
lack of exercise, and poor nutrition. The National Comprehensive Cancer
Network (NCCN) provides clinical guidelines for management of CRF and
recommends assessment of fatigue at diagnosis and management of symptoms
and treatable contributing factors including pain, emotional distress, sleep
disturbance, and decreased functional status (2015). Occupational therapy’s role
in CRF includes instruction in energy conservation balanced with continued
daily activity and engagement in routines and roles; intervention to improve
strength, balance, and physical abilities to complete daily activities; and
educating on good sleep hygiene.
Cancer-Related Cognitive Dysfunction

Previously referred to as “chemo brain,” cancer-related cognitive dysfunction
(CRCD) refers to various cognitive weaknesses an individual may experience
following chemotherapy treatments (Porter, 2013; Staat & Segatore, 2005).
These cognitive changes are common following chemotherapy treatments (Raffa
et al., 2006), with current research showing that 75% cancer patients experience
mental impairments and upward of 35% persist for months or years (Janelsins et
al., 2011). CRCD includes difficulty with attention, concentration, memory, and
processing speed, areas that directly impact executive functioning (Evens &
Eschiti, 2009; Janelsins et al., 2011; Raffa, 2010, 2013).
Potential causes of CRCD include primary brain disease, metastatic brain
disease, hypoxia, fluid or electrolyte abnormalities, psychological impact of the
diagnosis, stress, impaired sleep, chemotherapy treatment, brain radiation, and
treatment duration. Occupational therapists should use standardized cognitive
assessments in order to objectively measure and track cognitive abilities. This is
particularly important at the time of diagnosis, prior to the introduction of
treatments that potentially impact cognition such as chemotherapy. This allows
both an understanding of (near) baseline cognitive abilities and the ability to
identify subtle changes that may not be recognized during general conversations.
Engaging in mentally stimulating and cognitive exercising tasks has been shown
to positively impact cognitive functioning (Evens & Eschiti, 2009; Porter, 2013;
Staat & Segatore, 2005). The role of occupational therapy for individuals with
CRCD includes education of the individual, the family/caregivers, and the
treatment team on the noted areas of impairment and the anticipated impact on
the daily routine, along with facilitating treatment plans to include cognitive
retraining. For example, a mild cognitive impairment in the area of attention and
mental flexibility places the individual at risk of error in medication
management and household finances. During the time of impairment, it is
advisable to have assistance with these tasks as errors could be detrimental to the
individual’s overall well-being. Meanwhile, the occupational therapy plan of
care includes interventions to facilitate improvement in these areas and
standardized testing to track progress.
Immobility
A variety of reasons may cause an individual to become immobile including
cancer and treatment-related fatigue, weakness, pain, depression, fear of
movement, and hospitalization(s). Regardless of the underlying reason for
immobility, the immobility itself leads to a further decline in function beginning
at a physiological level. Research has demonstrated that immobility leads to
orthostasis and thrombus formation (Olsen & Thompson, 1967) as well as an
increase in accumulation of secretions (Olsen & McCarthy, 1967). This places
the individual at an increased risk of complications including orthostatic
hypotension, which further limits tolerance for out-of-bed activity, and may
preclude the individual from receiving further chemotherapy treatments.
Additionally, prolonged immobility may lead to critical illness polyneuropathy
(CIP), a multilevel weakness with high rates of mortality (Latronico & Bolton,
2011). Immobility also leads to decreased strength, decreased joint integrity, and
an overall decrease in physical functioning (Bryant, Grigsby, Swenso, Scarbro,
& Baxter, 2007; Bunting & Shea, 2001; Chambers, Moylan, & Reid, 2009). A
landmark longitudinal study, the Dallas Bed Rest and Training study,
demonstrated the significant changes to the cardiovascular system following
immobility. In 1966, five 20-year-old men participated in the Dallas Bed Rest
and Training study. It began with a series of tests on physiological capacity,
followed by 3 weeks of bed rest. At the end of the 3 weeks, the men were
retested, underwent 8 weeks of heavy endurance training, and were retested
again. The results showed a significant decline in their cardiovascular system.
Thirty years later, the same five men were evaluated and the results were
profound. A greater deterioration in cardiovascular performance, specifically
maximal oxygen uptake (VO2max), was found after 3 weeks of bed rest than in
30 years of natural aging (McGuire et al., 2001). A final follow-up to this study
was published in 2009 (McGavock et al., 2009). The same five men, 40 years
after the original assessments, were evaluated. The net proportional decline in
maximal oxygen uptake for a period of 40 years of life was comparable with that
experienced after 3 weeks of strict bed rest as a 20-year-old. Reflect for a
moment: this study was conducted on five healthy 20-year-olds. How much
greater is impact of bed rest for an older individual, or in someone with
comorbidities, or in someone who has cancer? The role of occupational therapy
is to facilitate participation with daily routines and roles, to provide therapy
interventions to improve function, to modify tasks and/or routines as needed, and
to provide recommendations for adaptive equipment or durable medical
equipment.
Lymphedema
The lymphatic system, composed of groups of lymph nodes and a network of
lymphatic vessels throughout the body, drains excess fluids from the body’s
tissues. It also helps defend the body against disease through lymphocytes, a
type of WBC. When there is disruption to the lymphatic system following
surgery or radiation in the area of the lymph nodes, rather than circulating
through the lymphatic system, the lymph fluid builds in the fatty tissue just
under the skin, resulting in lymphedema. Lymphedema is a condition resulting
from the disrupted flow of the lymph system and is a lifelong side effect that
cannot be cured but must be managed. As examples, lymphedema may occur in
the arm following axillary lymph node dissection for breast cancer or in the leg
following inguinal lymph node dissection for a soft tissue sarcoma or cervical
cancer. Lymphedema may also occur in the genitals, the trunk, or the head and
neck region, dependent upon the surgical or radiation area. Factors that increase
the risk of lymphedema include extensive surgery or radiation, an infection in
the affected limb, trauma to the affected limb, inadequate muscle contraction,
obesity, hypertension, and chronic venous insufficiency. Lymphedema goals
include decreasing the volume in the affected area and developing the best
system to maintain the lowest volume. Patients and caregivers are educated on
risk reduction, early detection, and treatment, as well as the risk for infection and
guidelines for proper skin care. Lymphedema treatments consist of manual
lymph drainage, use of elastic sleeves and short-stretch bandaging systems to
continue decreasing volume, and ordering custom-fit compression garments.
Occupational therapists may become certified lymphedema therapists (CLT)
after participating in a training and certification program.
Psychosocial Disruption
A certain amount of anxiety, despair, and fear is normal following a diagnosis of
cancer as the individual has concerns about potential changes in daily routine
and life plans, has changes in the body (loss of hair, postoperative changes, skin
changes following radiation), has a fear of death, or has legal or financial
concerns (American Cancer Society, 2013a). However, clinical anxiety and
depression may co-occur with cancer to a greater rate than the general
population without cancer and may impact the overall medical management and
outcomes (Hewitt, Greenfield, & Stovall, 2006). Research has shown the
prevalence of clinical anxiety ranging from 19% to 24%, clinical depression
from 6% to 13%, and mixed anxiety/depression from 9% to as high as 30%
(Boyes et al., 2013; Brintzenhofe-Szoc, Levin, Li, Kissane, Zabora, 2009;
Linden, Vodermaier, MacKenzie, & Greig, 2012). The percentages are higher
when subclinical symptoms are included. The Diagnostic and Statistic Manual of
Mental Disorders, 5th edition (DSM-5) provides diagnoses for both anxiety
disorder and depressive disorder “due to another medical condition” and
includes parameters for clinical diagnosis. The role of occupational therapy is to
recognize when these changes in mood are impacting the individual’s ability to
complete his or her daily routine and to share this information with the
individual, family, and treatment team. Through examination of the individual’s
routines and roles, the occupational therapy practitioner can recommend
modifications and instruct on balance both of tasks and roles, thus encouraging
continued daily engagement in activity.
Special Considerations
As cancer and the treatments impact areas across all client factors, occupational
therapy practitioners must have special considerations for the following areas in
order to effectively and safely guide treatment sessions.
Cardiovascular Considerations
Research has shown that chemotherapy treatments and radiation across the chest
increase the risk of cardiac problems including heart failure, myocardial
ischemia, arrhythmias, hypertension, and thromboembolism (Bovelli,
Plataniotis, & Roila, 2010). Regular monitoring for cardiac signs and symptoms
is important for early detection and intervention including the following:
Complaints of chest pain.
Resting pulse is >100 beats per minute or lower than 50 beats per minute.
Resting blood pressure is >145/95 or systolic is lower than 85 mm Hg.
Swelling is noted at bilateral ankles.
If the patient is on any medication(s) that controls the heart rate, the target heart
rate during exercise will not be attainable; do not overexert, and watch for
clinical signs and symptoms such as increased breathing and diaphoresis
(sweating) or increased complaints of fatigue.
If the patient has lymphedema or is at risk for lymphedema, compression
garments should be worn on the affected limb during exercise.
If the patient has a known premorbid risk of cardiac disease, then it is medically
recommend to be supervised during exercise testing and training.
Potential Contraindications
The following list of potential contraindications is defined as potential because
patients undergoing cancer treatments may very well fall below these
parameters; however, the risk of inactivity may outweigh the risk of mobility
with these potential contraindications: deep vein thrombosis (DVT) or
pulmonary embolism (PE), oxygen saturation below 90%, orthostasis, unstable
bone, platelets below 20,000 (20k), and hemoglobin (Hgb) below 8.0. Clinical
judgment and close attention to clinical signs and symptoms associated with
these values are important during occupational therapy sessions.
Cancer and Nutrition

Consideration must be given to the patient’s nutritional status as cancer and the
treatments may lead to poor oral intake or poor metabolism of intake. At times,
patients may have complaints of decreased appetite, decreased sense of taste,
nausea, or vomiting. If the patient has been vomiting or had diarrhea within the
last 24 to 36 hours, dehydration must also be considered. When not adequately
addressed, these issues may lead to failure to thrive and/or cancer cachexia, an
often irreversible syndrome leading to substantial weight loss including skeletal
muscle and body fat (Acreman, 2009). At times, artificial nutrition may be used;
options include total parenteral nutrition (TPN) infused intravenously, a
percutaneous endoscopic gastrostomy (PEG) tube, and a nasogastric (NG) tube.
The amount of energy (calories) expended during occupational therapy sessions
and throughout the day should not exceed the number of calories the body is
processing, especially in the setting of cancer cachexia.
Cancer and Sexuality and Intimacy

The changes in sexuality and intimacy following a cancer diagnosis and the
associated treatments have been shown to negatively impact both the cancer
survivor and his or her partner and lead to a decreased quality of life. Multiple
factors have been identified including physical changes following surgeries and
cancer treatments, decreased libido, CRF, mood disturbances including anxiety
and depression, changes in body image, emotional distancing, and role changes
from intimate partners to patient-caregiver (Beck, Robinson, & Carlson, 2012;
Hawkins et al., 2009; Loaring, Larkin, Shaw, & Flowers, 2015; Low et al., 2009;
Rolland, 1994; Ussher, Perz., & Gilbert, 2012). Changes in sexuality and
intimacy have been reported in as many as 76% of individuals with cancer and
84% of partners caring for individuals with cancer (Hawkins et al., 2009).
Occupational therapy practitioners should address the roles of sexuality and
intimacy in order to effectively optimize the overall quality of life.
CASE-STUDY 1
Janice, a 62-year-old female, had difficulty with a persistently infected
wound that had been nonhealing for several months. Further medical
workup revealed acute myeloid leukemia with 63% circulating blasts.
Janice was immediately admitted to the hospital for induction
chemotherapy. As the 14-day bone marrow biopsy showed residual disease,
she underwent a reinduction, which was successful in helping her achieve
remission. Overall, the hospital course proceeded without medical
complications. Her function declined slightly and she had generalized
weakness, as expected, but she remained independent with her basic daily
routine. Cognitive testing during occupational therapy sessions showed a
mild cognitive impairment in executive functioning, specifically with
cognitive flexibility and working memory. She has been completing
cognitive self-exercises. Now medically stable, and after 47 days of
hospitalization, Janice is ready to return home with her husband. Due to the
lengthy medical course for acute leukemia, she had to stop working as a
real-estate assistant; therefore, she plans on spending most of her time at
home. While she enjoys socializing with her friends and is typically active
in her church, she verbalized a good understanding of the need to balance
her routine within her tolerance, as well as the importance of avoiding close
contact with individuals who may be contagious. She expressed both
excitement and apprehension regarding the medical plan for a BMT. An
HLA-matched donor has been located, and the process is scheduled for
approximately 6 weeks from now.
CASE-STUDY 2
Sonya is a 43-year-old female with stage IIa breast cancer. Unfortunately,
the disease has been refractory to multiple rounds of chemotherapy and has
continued to progress. She now has metastatic disease to the liver, spleen,
and bones. Sonya resides with her husband and two sons aged 10 and 13
years old in a single-level home. Previously an elementary school teacher,
Sonya stopped working approximately 6 months earlier due to disease
progression and became a stay- at-home mother. While this transition was
emotionally difficult for her, she took pride in keeping the home “ready for
when everyone gets home each day.” Three months ago during a hospital
admission, her occupational therapist instructed her that she could no longer
bend, could no longer lift more than 5 lb, and needed to avoid twisting her
back. This change in her routine was made due to the findings of multiple
bony lesions including at T8, which required medical intervention to
stabilize, and at L3 and L5. She was encouraged to complete her own self-
care, to remain active each day within her precautions, and to “teach her
sons” when they arrived home from school to complete portions of tasks
such as lifting laundry, placing casseroles in the oven, and vacuuming.
Sonya is now admitted to the hospital following complaints of progressive
bilateral leg weakness to the point of being unable to move and increasing
neck pain. Imaging shows a new lesion at C5 with cord edema, and there is
a concern for metastatic disease involving the CNS with differential
diagnoses including metastatic dural disease versus leptomeningeal disease.
Her mother and father are willing to have her live with them so they can
take care of her while her husband continues to work full-time to maintain
finances. Although in tremendous pain, Sonya wishes to be able to use the
bedside commode instead of a bedpan.
RESOURCES
Academy of Nutrition and Dietetics: Oncology Nutrition
http://www.oncologynutrition.org
American Cancer Society
http://www.cancer.org
American Lung Association
http://www.lung.org
American Society for Blood and Marrow Transplantation
http://www.asbmt.org
International Agency for Research on Cancer http://www.iarc.fr
Leukemia and Lymphoma Society
http://www.lls.org
National Cancer Institute: SEER Training Modules
http://www.training.seer.cancer.gov
National Cancer Institute: Comprehensive Cancer Information
http://www.cancer.gov
National Comprehensive Cancer Network http://www.nccn.org/
National Lymphedema Network
http://www.lymphnet.org
National Hospice and Palliative Care Organization http://www.nhpco.org
Occupational Therapy in Oncology
http://www.otoncology.org
Palliative Care Video
http://www.palliativecarevideo.com
World Health Organization: Cancer
http://www.sho.init/cancer/en/
RECOMMENDED READING
The Immortal Life of Henrietta Lacks by Rebecca Skloot; New York: Crown
Publishers, 2010.
The Emperor of All Maladies by Siddhartha Mukherjee; New York:
Scribner, 2010.
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C H A P T E R
28 Obesity
Shirley Blanchard
KEY TERMS
Apple ascites Apple pannus Bariatrics Body mass index Cortisol Diabesity
Fowler position Globesity Gluteal shelf Hypothyroidism Leptin
Lymphedema Metabolic syndrome Obesity
Obesity hypoventilation syndrome Orthopneic position Osteoarthritis Pear
abducted Pear adducted Posterior adipose Sarcopenia Satiety
Sleep apnea Vitamin D deficiency Waist circumference
Carl is a 56-year-old man who is a part-time assistant pastor of a small church
congregation in the rural South. With a height of 6′3″, his weight had been stable
at 240 lb until he had a sudden onset of a rare neurological condition, Parsonage-
Turner Syndrome. He was unable to return to work as a furniture machinist
secondary to the inability to perform shoulder and arm motions, perceive arm
position, and lift and carry objects. Since the diagnosis, he has gained 230 lb and
has adopted a sedentary lifestyle. He and his wife cook traditional Southern
meals such as biscuits, gravy, and fried foods. His current weight of 470 lb
makes it difficult to tolerate activity, perform functional mobility, find suitable
clothing for church, participate in home maintenance, and get in and out of the
car. He was recently hospitalized for hypertension, lower extremity cellulitis,
and edema. He is edentulous due to persistent gingivitis and periodontal disease
that resulted in complete teeth extraction. His physician prescribed antibiotics
for systemic inflammation and recommended weight loss and consultation with a
dietician. When asked about his eating habits he states, “I have to have some
enjoyment.” The client’s mental health status was not assessed.
Obesity is a public health concern and a complex social problem. The University
of Rochester Minnesota Department of Senior Health Research (URMCR)
reports that of the top ten most common health issues, being overweight and
obesity ranked second to physical inactivity and nutrition (URMCR, 2015). A
constellation of factors contribute to obesity including health behaviors, diet,
physical inactivity, and genetics. Health disparities such as environment,
socioeconomic status, health literacy, and access to health education may also
impact obesity.
Obesity is a modifiable risk factor associated with type II diabetes,
cardiovascular disease, heart disease, gallbladder and liver disease, sleep apnea,
gynecological problems (fibroid disease), neurological (such as atherosclerosis,
hypertension, and peripheral vascular disease), and musculoskeletal changes
such as osteoarthritis (OA). Metabolic syndrome and diabesity refer to the
presence of a combination of risk factors including obesity, diabetes,
hypertension, high cholesterol, elevated fasting blood sugar, high triglycerides,
systemic inflammation, and a tendency to form blood clots (Blanchard, 2012;
Kresser, 2015).
The relationship of obesity to cancer has received less attention; however,
existing evidence suggests that increased adipose tissue may increase the risk of
breast, prostrate, and colon cancer. Additionally, increased body weight increases
circulating insulin, which affects multiple types of cancer cells (Calle & Thung,
2004). Recent research examined a possible link between obesity and
Alzheimer’s disease. Chuang et al. (2015) studied 142 elders with an average
age of 83 when diagnosed with Alzheimer’s. Findings imply that those who were
overweight by age 50 tended to develop a decline in memory earlier. Higher
BMI at age 50 was related to early onset of the disease and at autopsy
demonstrated increased brain tangles. The Centers for Disease Control and
Prevention (CDC, 2015a) advise that obesity is associated with poorer mental
health outcomes and reduced quality of life. Figure 28.1 represents a summary of
associated complications and the impact of obesity on client factors and body
structure.
Figure 28.1 Complications of obesity. (From Braun, C. A., & Anderson, C M.
2011. Pathophysiology: A clinical approach. Philadelphia, PA: Wolters Kluwer.)
The terms overweight, obesity, and morbid (or extremity obesity) refer to excess
body weight (Foti, 2005). Currently, the CDC, World Health Organization
([WHO], 2015), and the American College of Sports Medicine ([ACSM], 2012)
define obesity by using the body mass index (BMI) measure. The BMI was
developed by the Nutrition/Metabolism Laboratory, Cancer Research Institute in
Boston (Blackburn & Kanders, 1987). The BMI is an international standard used
to determine degree of obesity relative to height and is calculated by multiplying
weight in (pounds) × 703 and then dividing by the height in inches squared. A
BMI < 24.9 kg/m2 is considered normal, while a BMI in excess of 25 is
considered overweight with increased risk for disease (ASCM 2000, 2014). BMI
may also be calculated electronically using the National Heart Lung and Blood
Institute (NHLBI) Guidelines (NIH Web site or with smart phone apps; graphs
are available for tracking BMI (NHLBI, 2015).
Along with waist circumference, BMI is used to describe the degree of
obesity and predict the level of disease risk. Waist size indicates the amount of
fat distribution in the abdominal area. An accumulation of body fat in this area
results in an apple shape body and is associated with increased risk of heart
disease and diabetes (ACSM, 2014). Specifically, a waist size > 35 in. or 88 cm
for women and 40 in. for men is associated with higher risk for high blood
pressure, high cholesterol, and heart disease (ACSM, 2014; CDC, 2015a). More
recently, the CDC suggest that a waist size > 35 for women and 37 for men is
associated with increased for disease (CDC, 2015b). Jacobs (2010) found that a
waist size of 47 in. or larger for men and 42 in. for women increased risk for
death compared to those with a waist size of 35 and 30 respectively. An adult
with a BMI between 25 and 29.9 kg/m2 is considered overweight, and an adult
with a BMI higher than 30 kg/m2 is considered obese (Table 28.1). A BMI
between 39 and 40 is considered morbidly obese with extremely high risk for
disease. BMI > 50 may be classified as super obesity or super morbid obesity
(Dallas Center for Obesity Surgery, 2013). The term morbid obesity refers to
patients who are 50% to 100% or 100 lb above their ideal body weight (Kolata,
2015).
TABLE 28.1 Body Mass Index, Waist Size, and Disease Risk
aClassification of disease risk based on waist circumference: <88 to >88 cm.
Adapted from American College of Sports Medicine. (2014). Classification of disease risk based on BMI
and waist circumference. ACSM’s resources for the personal trainer (4th ed., p. 311). China: Lippincott
Williams & Wilkins.
The term bariatrics is often associated with persons who are morbidly obese.
Current literature defines this condition as the medical study of the causes,
evaluation, and intervention for individuals who are obese (Foti, 2005; Purnell,
2011; Stedman’s Medical Dictionary, 2006). Medical and surgical management
of bariatrics will be discussed later in the chapter.
Childhood Obesity
Obesity among children and adolescents continues to be a health concern.
Obesity is defined in children and adolescents aged 2 to 19 years of age as a
BMI at or above the 95th percentile according to gender, age, and growth chart
(CDC, 2011). The lack of a diagnosis of obesity and an immediate plan for
intervention during annual medical examinations exposes children to the same
health conditions and comorbidities experienced by adults. Children may
develop hypertension, systemic inflammation, dyslipidemia, type II diabetes,
sleep apnea, and increased rate of obesity. Shah et al. (2015) examined
adolescent and young adults with obesity and found increased cardiovascular
risk factors (e.g. C-reactive protein, increased blood pressure, type 2 diabetes,
and arterial wall thickness). Bout-Tabaku et al. (2015) assessed musculoskeletal
pain among 233 teens average age 17 with a median BMI of 50.4. Seventy-six
percent of the teens reported musculoskeletal pain, low back pain (63%), hip
pain (31%), knee pain (49%), and ankle and foot pain (53%). Results suggest
that adolescents with morbid obesity experience musculoskeletal pain that limits
physical function and quality of life.
The American Academy of Pediatrics (APA) recommends annual monitoring
of BMI to prevent childhood and adolescent obesity (Flower, Perrin, Viadro, &
Ammerman, 2007).
Flower et al. (2007) interviewed pediatricians and parents and found lack of
assessment, identification, reporting, and discussion about children’s obesity.
Themes from focus groups also suggest that there are several barriers to using
BMI screening with children, including lack of access to accurate BMI charts
and accurate height and weight measurements, outdated growth charts, and lack
of time to calculate BMI. BMI results may also stigmatize children as being
obese and cause issues with self-esteem. This creates added psychosocial stress
and pressure to be thin (Schvey, et al., 2015), which may be further perpetuated
by the media, peers, friends, and family members. Dissatisfaction with body
image, depression, and failed attempts to achieve weight loss through dieting
may result in weight gain, altered metabolic function (insulin sensitivity), and
secretion of cortisol. Combined stressors, obesity, and psychosocial challenges
may lower school performance and reduce quality of life (Tonetti, Fabri, Filardi,
Martoni, & Natale, 2015). Today, the requirement of electronic medical records
automatically calculates BMI, tracks risk factors, and compares measurements
over time (Flower et al., 2007). BMI results may be used to raise parent and
child awareness and discuss and set goals for lifestyle change and weight
management.
Elder Obesity
Obesity among elders may result from a decline in physical activity and a
change in independent living (Blanchard & Mosley, 2010). As elders transition
from independent living to long-term care facilities and a more sedentary
lifestyle, they are often positioned in and confined to wheelchairs. Weight gain
may be associated with chronic conditions, immobility, and altered eating habits.
This immobility limits range of motion and strength and hinders energy
expenditure necessary to maintain a healthy weight. Elders who were active and
lived independently are now exposed to an increase in social eating, which also
increases BMI. Limited participation in occupations, deconditioning, and loss of
muscle mass (sarcopenia) influences physical function and metabolic rate.
Obesity among elders can negatively impact pulmonary, cardiovascular,
cognitive status (vascular dementia), cholesterol, and joint pain associated with
OA (Sperling, Laviolette, O’Keefe, et al., 2009). Long-term care facilities are
now monitoring elder BMI through the Minimum Data Set (MDS). This Centers
for Medicare and Medicaid (CMS) resident assessment tool requires a
reassessment of BMI every 30 days. Section K of the MDS monitors BMI
(CMS, 2010).
Obesity as Disease
Recently, the American Medical Association (AMA) approved the classification
of obesity as a disease. The organization defines disease as: “(1) an impairment
of the normal functioning of some aspect of the body; (2) characteristic signs
and symptoms; and (3) causes harm or morbidity” (Sales-Martinez, 2013,
paragraph 2, p. 1). The AMA and Jones (2015) of the Rudd Center for Health
Policy and Obesity further propose advantages and disadvantages to classifying
obesity as disease. Classifying obesity as a disease raises awareness, increases
insurance coverage and reimbursement for treatment, and expands federal and
private funding for research, which may lead to new public policies regarding
obesity intervention and prevention (Smith, 2013).
Proponents of the new classification support early diagnosis through primary
screening and argue that chronic obesity may be prevented through follow-up
care, weight loss, and reducing associated stigma. Obesity may also be viewed
as an addiction. The new classification suggests that persons will take the
initiative to seek intervention. However, motivation for compliance with diet,
increased physical activity, or other lifestyle changes is uncertain (Sales-
Martinez, 2013).
Opponents of the AMA decision argue that obesity is a risk factor for other
chronic diseases, that BMI does not measure fat mass, and that patients
diagnosed as overweight or obese may still be healthy. Padro, Gonzales,
Heymsfield, et al. (2015) agree that BMI does not differentiate between the
contributions of lean body mass opposed to adipose tissue. This lack of well-
defined overall body composition confounds the documented health
consequences for morbidity and mortality associated with obesity across
populations (Sales-Martinez, 2013). Therefore, it is felt that the disease
classification is not warranted. Perceived lack of control over weight results in
taking less responsibility for weight management. For example, individuals may
seek a “quick fix” through pharmacological intervention or surgery (Sales-
Martinez, 2013). Classification of obesity as a disease has the potential to reduce
stereotypes and place the responsibility on the individual; however, the
classification still must consider environmental, social, cultural, and
psychological issues that may impact this complex health issue (Sales-Martinez,
2013; Smith, 2013).
Obesity and Stigma
Existing evidence supports that persons who are obese may be shunned by
others. Stunkard and Sorensen (1993) refers to “obesity as the last socially
acceptable form of prejudice” (p. 1037). Stigma associated with obesity is seen
across population groups, among health care professionals, and across the
lifespan. Persons who are obese may experience verbal abuse and verbal and
physical bullying and be more vulnerable to depression, reduced self-esteem,
and poor self-concept (Puhl & Latner, 2007). Unfortunately, health care
professionals such as nurses, physicians and students perpetuate negative
attitudes towards persons who are obese. Many fear the doctor’s response that is
often stated as “I can’t help you if you don’t lose weight” (Bailey, 2016, p. 8).
As the BMI increases, health providers are less tolerant and attentive, do not
address health concerns in a timely manner, and view the person with obesity as
a “waste of time,” which results in negative psychosocial health and quality of
life (Teachman & Brownell, 2001; Wang, Brownell, &Wadden, 2004). Blodorn,
Major, Hunger, and Miller (2015) found that over time, constant rejection
associated with higher BMI threatened social identity and increased the
expectation of a negative response. Allison and Lee (2015) compared attitudes
toward 185 overweight female university students. Results from participants
read one of six vignettes to describe a female student with varying weights
indicated that overweight targets were viewed more negatively than average
weight or underweight targets.
Persons who are obese may be perceived as dishonest, sloppy, lacking self-
control, lazy, unattractive, intellectually impaired, gluttonous, and socially
impaired (Crandall, 1994; Flint, 2015). Persons who are obese report
discrimination in the workplace, doctor’s office, school, or other social or public
settings. Rejection, stereotyping, and disrespect results in social isolation,
overeating, and an increased potential for self-harm (Bailey, 2016; Faith, Matz,
& Jorge, 2002; Puhl & Brownell, 2006). Choice of terms such as overweight or
unhealthy weight is preferred to thick, chubby, or morbidly obese (Dutton, Tan,
Perri, et al., 2010; Tailor & Ogden, 2009). Children who are obese may
experience similar adult comorbidities and stigmatizations that perpetuate health
disparities across the lifespan (Flint, 2015).
Obesity and Depression
Obesity is considered to be a medical but not a mental health diagnosis. The
relationship between emotional state, behavior, excessive food intake, and low
energy expenditure may reflect a significant mental health or psychosocial issue.
The Diagnostic and Statistical Manual of Mental Disease Version-5 (DSM-5)
does not include overeating but focuses on binge eating (Grever, 2013; Walsh,
2010). The American Psychiatric Association included bulimia (purging
syndrome) and night eating syndrome in the most recent edition of the DSM-5,
but failed to include obesity secondary to lack of acceptance of the diagnosis as a
mental health disorder (Devlin, 2007; Grever, 2013; Walsh, 2010). Depression is
one of the most prevalent psychiatric disorders and a major contributor to the
United States (US) burden of disease. Bailey (2016) suggests that up to 80% of
persons who are obese have some level of depression. Onset of depression and
obesity may be related to geographical location and seasonal affective disorder
(SAD) (Atkinson, 2005). Faith et al.’s (2002) meta-analysis implies an
association between obesity and depression in childhood, during midlife, and
among elders.
The relationship between obesity and depression may also differ by gender
and race. Several studies support a biological and psychological basis for
overeating and weight gain. Xiang and Ruopeng (2014) correlated the BMI of
6,514 adults born between 1931 and 1941 with the Center for Epidemiology
Studies Depression Scale (CESDS). The CESDS defines symptoms of clinical
depression over a 2-week period. Results indicated that unhealthy body weight
was associated with future onset of depression. Blanchard (2009) examined the
relationship of BMI and Depression among 378 African American women.
Eighty-six percent of the sample had an average BMI of 32.78 with a high risk
for disease. CESDS scores ranged from some to severe symptoms of depression
with 73 (23.5%) reporting mild symptoms of depression, 79 (25.4%) moderate,
and 29 (9.4%) severe symptoms. Women also reported overeating when stressed.
Dixon, Dixon, and Obrien (2003) reported that weight loss following bariatric
surgery was associated with a decline in depression scores 1 to 4 years
postsurgery. Other researchers report that psychological well-being following
bariatric surgery depends on presurgery eating patterns, social support, self-
esteem, body image, coping strategies, and the amount of weight loss after
surgery (Ortega, Fernandez-Canet, Alvarez-Valseita, Cassinello, & Baguena-
Puigcerver, 2012).
Biological contributions to obesity include an increase in the stress hormone
cortisol, which is a steroid hormone that is activated in response to stress and
hypoglycemia and is believed to trigger depressive symptoms. In addition,
medications used to treat depression may result in weight gain.
Obesity and Stress
Stress and anxiety may also interfere with one’s ability to lose and manage
weight. Various types of stress may contribute to obesity including emotional
stress. Stress results in the secretion of cortisol, which has been shown to
increase central adipose tissue and secondary risk factors for disease such as
metabolic syndrome. Women may be more predisposed to stress than men due to
increased central fat. Studies are beginning to focus on secretion of cortisol in
women. Epel et al. (2015) exposed 59 women (30 with high hip to waist ratio,
and 29 with a low hip to waist ratio) to three stressful sessions and one rest
session. Women with the high hip to waist ratio secreted significantly more
cortisol and reported greater challenges when stressed than did the low hip to
ratio group. Another study examined the relationship between chronic stress,
food cravings, and BMI for a community-based sample of adults (N = 619).
Chronic stress had a significant effect on food cravings, and food cravings had a
significant effect on BMI. Findings are consistent with research that chronic
stress is related to motivation for reward-seeking behaviors and indicate that
high food cravings may contribute to stress-related weight gain (Chaol, Grilo,
White, & Sinha, 2015). Repeated stress associated with altered sleep habits and
routines such as rotating work shifts are examples of environmental factors that
may also increase weight.
Obesity and Vitamin D Deficiency
Vitamin D deficiency is common among children, adults, and elders. Research
findings support that most tissues and cells of the body have a vitamin D
receptor. Current research aims to discern the role of vitamin D in the prevention
of chronic diseases such as cancer, cardiovascular, depression, and obesity
(Holick, 2007). Vitamin D deficiency is defined as a 25-hydroxy vitamin D level
of <20 ng/mL; 30 is considered sufficient, and toxicity may occur at 150 ng/mL.
Wortsman, Matsuoka, Chen, Lu, and Holick (2000) found low levels of vitamin
D in a sample of persons who were obese and that oral vitamin D corrected the
deficiency. They further postulate that because of low physical activity levels,
persons who are obese spend less time exposed to the sun and thus have lower
vitamin D levels. Solar radiation is required for synthesis of vitamin D. It is also
thought that vitamin D deficiency is linked to osteoporosis and muscle weakness
that may be associated with observed immobility in persons who are obese
(Holic, 2007). Seppa (2013) reported that persons with low Vitamin D levels
were not prone to obesity and that losing weight could reverse vitamin D
deficiency. Vitamin D supplements may not be effective as a weight loss regime.
While vitamin D is stored in fat tissue, the mechanism for the role of vitamin D
in weight loss requires additional research.
Etiology
A variety of factors influence obesity. The consequence of physical inactivity
and its relationship to obesity is well documented in the public health literature.
An imbalance between calories consumed and energy expenditure during
occupational performance create a positive energy balance resulting in weight
gain and obesity. Even a modest energy imbalance over a sustained period of
time may increase BMI. Research has shown that losing 5% to 10% of excess
weight may prevent disease (Diabetes Care, 2011). Location of grocery stores,
food choices, and availability account for some obesity. Previously, lack of
available food resulted in storing fat to prevent starvation. Now the type of food
(such as fast, convenient, or refined) and high availability result in increased
stored fat and the prevalence of obesity (Mance, Veach, & Veach, 2013).
Genetic Factors
Genetic factors combined with lifestyle choices increase the risk or
predisposition for familial obesity. Patterns of childhood obesity are similar in
appearance to adult obesity. Family, and adoption studies attribute obesity to a
phenotype or observed characteristics of a group (e.g., family members who
resemble each other in appearance) (MOSBY’s Medical Dictionary, 1994, p.
1208). Studies examining the relationship of genetics to obesity report that 40%
to 70% of the variance in body mass is related to social environment (type of
food consumed, factors related to heredity, and physical inactivity). Clement,
Boutin, and Froguel (2002) used genome scans to identify the location of 68
obesity genes on several chromosomes that aid in the regulation of appetite and
satiety. A more recent study of genetics and obesity in 249,796 European
participants identified 32 loci or markers associated with BMI. These markers,
however, explained only 1.5% of the variance in BMI (Volkow, Wang, & Baler,
2011). Available research on genotyping and obesity has yielded small,
uncertain, and limited explanation of the contribution of genetic markers to
obesity. Inconsistent effects make it difficult to generalize results across
populations.
Obesity Hypothalamus and Hypothyroidism
The inability of the hypothalamus to recognize satiety along with thyroid
dysfunction may also result in obesity. The thyroid gland secretes the hormone
thyroxin into the blood stream that is essential to the regulation of normal body
growth and metabolism (processing food into energy). Thyroid cells absorb and
use iodine. The pituitary gland and hypothalamus both control the thyroid. When
thyroid hormone levels drop too low, the hypothalamus secretes TSH releasing
hormone (TRH), which alerts the pituitary to produce thyroid stimulating
hormone (TSH) (Sargis, 2015). Hypothyroidism occurs when cells of the
thyroid gland are damaged by inflammation resulting from an associated
autoimmune response or secondary medical interventions. The most common
cause of thyroid gland failure is called autoimmune thyroiditis (Hashimoto’s
thyroiditis), a form of thyroid inflammation caused by the patient’s own immune
system (Sargis, 2015).
An endocrinologist may diagnose hypothyroidism with a medical and family
history, risk factors, and physical examination. A definitive diagnosis may be
achieved with a blood test called a TSH; additional laboratory analyses such as
thyroxine or T4, and triiodothyronine or T3 also support the diagnosis.
Normally, the thyroid gland produces 80% T4 and 20% T3; T3 is the stronger of
the two hormones (Sargis, 2015). Combined, T3 and T4 increase the rate of
metabolism, affect body temperature, and regulate protein, fat, and carbohydrate
catabolism (conversion of nutrients into energy) in the cells of the body. Without
intervention, fatigue, weakness, weight gain, or increased difficulty losing
weight increases. The inability to burn fat normally is often referred to as having
a “fast” or “slow” metabolism (Mosby’s Medical Dictionary, 1994, p. 1555). It is
believed that weight loss may lead to decreased systemic inflammation that may
restore thyroid function (Longhi & Radetti, 2013). Pharmacologic interventions
such as synthetic hormones (Synthroid, levothyroxine sodium, liothyronine
sodium) may be used to regulate hormone function (Sargis, 2015). Care must be
taken to avoid a rapid increase in dosage to avoid symptoms of hyperthyroidism
such as nervousness, tremor, tachycardia, arrhythmia, or menstrual irregularity
(Carl, Gallo, & Johnson, 2014).
Hunger is associated with increased blood flow to the hypothalamus,
thalamus, and frontal and temporal lobes. Destruction or trauma to the
ventromedial hypothalamus, which regulates appetite and feeding behavior, may
increase food intake and reduce metabolic rate resulting in obesity. Leptin is a
hormone that is present in adipose tissue and signals receptors in the brain of
satiety, or when one is full. Diminished leptin in the brain results in impaired
signaling of satiety, thus promoting overeating. Females tend to have a higher
percent of body fat or adipose tissue than do males; thus, with low levels of
leptin they experience weight gain, increased hunger, and reduced metabolic rate
(Atkinson, 2005; Couillard et al., 2002; Meyers, Leibel, Seeley, & Schwartz,
2010). For elders, low leptin levels, increased fat tissue, and loss of muscle mass
are referred to as sarcopenia.
Gorden and Gavrilova (2003) report that leptin replacement leads to reduced
food intake, weight loss, and reduced percent body fat. Satiety has also been
achieved in both human and mouse models, but an increased resting metabolic
rate only occurred in mice, suggesting that a loss of fat mass may impact resting
metabolic rate.
Obesity and Neuroscience
Available research indicates that there is inconclusive but plausible evidence
linking the neurobehavioral effects of overeating, obesity, and addiction
(Ziauddeen, Farooq, & Fletcher, 2012). Neuroscientists are aware of neural
structures that contribute to obesity; addiction research infers that addiction to
food is similar to addiction to drugs both in behavioral and neurological response
(Ziauddeen et al., 2012). Food addiction literature reports that foods high in fat,
salt, and sugar are enjoyed by those who are obese. Processed foods may be
more addictive than foods in their natural state such as fruits and vegetables
(Benton, 2010). The addiction model supports the premise that obesity is related
to food addiction and binge eating. Binge eating is characterized by
uncontrolled, rapid consumption of large amounts of food in isolation and in the
absence of hunger despite the negative impact on health, social or financial
limitations. Multiple failed attempts to alter behavior results in feelings of guilt,
remorse, distress, and failure (Smith & Robbins, 2013). The Yale Food
Addiction Scale (YFAS) used DSM-5 addiction criteria to format questions that
identify those who may exhibit signs of addiction toward certain foods (such as
high fat, salt, and sugar). Knowledge of which foods trigger overeating and food
addiction has the potential to impact food marketing strategies across the
lifespan and may impact public policy (Gearhardt, Corbin, & Brownell, 2008).
Several neural structures are thought to contribute to addictive behavior and
obesity. The hypothalamus regulates satiety or the feeling of fullness. The
prefrontal cortex is associated with habitual and compulsive overeating; the
dorsal striatum is responsible for loss of executive control over the behavior.
Impulsive eating or initiating behavior without considering the consequences
may be related to lower levels of dopamine. Insufficient dopamine may increase
seeking of the feelings of reward and result in overeating (Volkow et al., 2002,
2011).
The more food that is consumed, the more one loses control and awareness
of the amount of food consumed. Results of positron emission tomography
studies show that striatal dopamine receptors are reduced in persons who are
obese compared to leaner counterparts and that they tend to overeat to
compensate for reduced striatal sensitivity (Mahapatra, 2010). Thus, long-term
overeating of pleasurable foods alters the chemical response in the brain and
sensitizes the brain to “crave certain foods”; thus the motivation and drive to eat
and consume certain food increases with environmental cues (Volkow & Wang,
2005; Volkow et al., 2011). Researchers are focusing on food addiction rather
than diet and the possibility of prescribing pharmacological agents to artificially
alter dopamine as an intervention for obesity. Proposed medications may be
addictive and only recommended for those with morbid obesity and urgent
health risks (Devlin, 2015).
Prevalence and Incidence
The global public health epidemic of obesity is often referred to as “globesity.”
Nationally obesity has doubled since 1980. The CDC, National Center for
Health Statistics (NCHS) Data Brief (2015b) reports that the prevalence of
obesity from 2011 to 2014 was 36% for adults and 17% for youth; for adults
aged 20 to 39 and 40 to 59, the prevalence of obesity was higher among women
than men; more than one-third of adults and youth in the United States were
obese. If people who are classified as overweight are included, the estimate
increases to 69% or two-thirds of the population (Mance et al., 2013). Harvard
medical school researchers estimate that overweight, obesity, and physical
inactivity were responsible for 1 in 10 deaths in the U.S. (Mance et al., 2013).
Regarding race, higher rates were noted with women who are African
American at 56.9%, women who are Hispanic at 45.7%, men who are Hispanic
at 39.9%, and 37.5% for men who are African American. People who are Asian
tended to have lower rates of obesity at 11.9%. The average weight for men in
the U.S. in 2011 was 88.3 kg (about 195 lb), and for women it was 74.7 kg (or
165 lb). Depending on BMI, this represents a significant increase (Mance et al.,
2013). Recent findings by Ogden, Carroll, Kit, and Flegal (2014) reveal no
significant reduction in obesity, and prevalence remains high.
Table 28.2 summarizes the prevalence of obesity across the lifespan.
TABLE 28.2 Obesity Prevalence
Adapted from the Center for Disease Control and Prevention National Center for Health Statistics Data
Brief. (2015). Prevalence of obesity among adults and youth: United States, 2011–2014. Retrieved from
http://www.cdc.gov.nchs/data/databriefs/db219.htm
Certain states and areas of the country have a high incidence of obesity No state
had a prevalence of obesity <20%; 5 states and the District of Columbia had a
prevalence of obesity between 20% and <25%; 23 states, Guam, and Puerto Rico
had a prevalence of obesity between 25% and <30%; 19 states had a prevalence
of obesity between 30% and <35%; 3 states (Arkansas, Mississippi, and West
Virginia) had a prevalence of obesity of 35% or greater; and the Midwest had the
highest prevalence of obesity (30.7%), followed by the South (30.6%), the
Northeast (27.3%), and the West (25.7%) (CDC, 2014).
Signs and Symptoms
Classic signs and symptoms of obesity include pannus and abdominal fat,
lymphedema, and OA. Obesity may also be described by the location and
distribution of adipose or fat tissue, or pannus and its characteristic
anthropometric shape. Dionne (2006) identified six body types: apple ascites,
apple pannus, pear abducted, pear adducted, gluteal shelf, and posterior
adipose. Each body type may be predisposed to challenges with activities of
daily living, functional mobility, gait, and activity tolerance. These body types
are described in Table 28.3.
TABLE 28.3Body Type & Pannus Distribution Adapted with permission

from Dionne’s (2006) Six Body Types Images and Lisle E. Veach,
PTcourses.com, 2013.
Obesity and Lymphedema
Persons with obesity may experience compromised skin integrity secondary to
skin on skin contact and impaired lymph drainage. Diabetes may further
complicate wound healing and increase the risk of infection. For clients who are
immobile, the Braden Scale may be used to predict risks for pressure sores (15
to 16 = low risk, 13 to 14 = moderate risk, 12 or less = high risk) (Braden, 2014).
A BMI > 50 often results in bilateral lower extremity edema associated with
lymphatic dysfunction. Adipose tissue is composed of adipocytes or fat cells that
produce hormones referred to as adipokines. These hormones cause a chemical
reaction that impedes the function of the lymph system causing lymphatic
leakage or lymphedema (swelling) (Greene, 2015). Obesity combined with
decreased muscle pumping action further limits lymphatic function. The number
of patent lymphatic vessels cannot keep up with the demand of new and
proliferative adipose tissue; thus, the extra weight increases resistance to the
distal and proximal flow of lymph (Bertsch, 2015; Greene, 2015). Impaired
lymph node regeneration and abnormal swelling associated with cycles of
weight loss and gain is known as primary lymphedema and may develop early in
infancy, childhood, or adolescence and continue into adulthood (Greene, 2015).
A diagnosis of lymphedema is made using lymphoscintigraphy, which verifies
backflow or blocked lymphatics (Greene, 2015).
Abdominal obesity such as that observed in the apple ascites or apple pannus
body type may compress the lymphatics in the abdominal and groin area and add
to lower extremity edema. Weight loss may improve lymphatic function but may
not reverse the condition (Bertsch, 2015). Decongestive therapy, dietary
intervention, and weight loss are recommended as primary interventions.
Obesity and Osteoarthritis
Osteoarthritis is also known as degenerative joint disease (DJD) or a “wear and
tear” disease. Clients 75 and older with obesity report higher knee pain and are
more likely to be disabled (Jordan et al., 1996). Oviatt (2009) reports that 65%
of adults with DJD are overweight or obese; 44% of those with DJD report
limited physical activity. Persons 85 years of age and older, who are obese,
account for 57% of annual hip and knee replacements. CDC Behavioral Risk
Surveillance System data from 2009 revealed that 53% of adults with DJD had
no leisure time activity, limited access to a fitness center for appropriate
exercise, and lack of fitness instructors who are knowledgeable about DJD
(Morbidity and Mortality Weekly Report 2011). BMI levels of ≥30 kg/m2
increases joint loading and causes misaligned joints and failure of weakened
quadriceps to contract adequately and absorb forces needed to transition from sit
to stand (Sowers & Karvonen-Gutierrez, 2010). Coggon et al. (2001) suggest
that those with obesity were nearly three times more likely than were those of
normal weight to develop severe knee pain over a 3-year period. Of those who
are overweight and obese, a reduction of weight by 5 kg, and achieving a BMI
within the recommended normal range, would result in an estimated 24%
reduction in knee surgeries associated with OA. Strong support for public health
initiatives aimed at reducing the burden of knee OA by controlling obesity is
needed.
Obesity Hypoventilation Syndrome
Sleep apnea is referred to as obesity hypoventilation syndrome (OHS) or
pickwickian syndrome. OHS results from excess weight compressing on the
chest, preventing breathing, and increasing the amount of carbon dioxide in the
blood; lack of oxygen contributes to poor sleep quality and hypoxia (Mance et
al., 2013). As weight increases around the neck, trunk, and abdomen, temporary
lapses in breathing compromise respiratory function. Clients with obesity may
benefit from using a larger electric hospital bed with the head of the bed elevated
between 45 degrees to 90 degrees known as the Fowler position or elevating the
head of the bed to 90 degrees and placing the arms over a bed table in a
orthopneic position. Continuous positive airway pressure (CPAP) may also be
used to achieve positive flow of air into the nasal passages in order to keep the
airway open.
Failure to achieve a sustained healthy weight with nonsurgical procedures,
pharmacological intervention, dietary changes, and some physical activity will
lead to more invasive procedures such as bariatric surgery. Outcome data focus
on the total amount of weight lost, reduction in chronic health conditions, and
number of prescribed pharmacological interventions used to treat comorbidities.
Following bariatric surgery a weight loss of 50% to 70% is expected (Health
Grades, 2013). There is a significant decrease or reversal of chronic conditions
including type II diabetes and high cholesterol; improvements in blood glucose
levels may appear within days following bariatric surgery (Health Grades, 2013).
For clients with a BMI of 35 or with comorbidities, bariatric surgery resolves
migraines (57%), hypertension (52% to 92%), cardiovascular disease (82%),
dyslipidemia (63%), metabolic syndrome (80%), type II diabetes (83%),
obstructive sleep apnea (74%), fatty liver (90%), gastroesophageal reflux disease
(72% to 98%), urinary stress incontinence (44%), gout (72%), DJD (41% to
76%), polycystic ovarian syndrome (79%), depression (55%), reduced quality of
life (95%), and mortality (89%) (Brethauer, Chand, & Schauer, 2006). Long-
term health benefits also include lower risk of cardiovascular and
cerebrovascular events with improved quality of life (Health Grades, 2013).
Quality of life following bariatric surgery depends on type of surgery and
procedure, residual intestinal and digestive problems, discomfort and pain, and
presence of excess skin folds. Clients requiring body contouring to reduce loose
skin a year following surgery reported improved quality of life (Lier, Aastrom, &
Rortveit, 2015). Occupational therapy faculty and student researchers surveyed
11 clients post bariatric surgery using an activity and functional health and well-
being assessment. Average weight loss was 105.1 with highest loss of 189 lb.
Although this was a small sample size, several themes emerged from the study.
Post– bariatric surgery participants reported a significant increase in
participation in instrumental activities of daily living (IADLs) that require
physical movement, leisure pursuits, social interaction, and health maintenance.
Even though participants achieved weight loss, some clients transferred food
addiction to alcohol or other drug use. Additionally, clients experienced
relationship changes and divorce and often reverted to previous unhealthy eating
patterns and habits (Mata, Mikkola, Loveland, & Hallowell, 2015). Ortega et al.
(2012) surveyed 60 morbidly obese clients (46 women and 14 men) 1 year post–
bariatric surgery. Findings indicate that negative preoperative body image
improved after surgery but self-esteem did not change. Overall findings suggest
that psychological intervention is needed to prepare clients for realistic
expectations following surgery and to improve postsurgical outcomes.
Diet and exercise may be recommended at various levels of intervention. Clients
at risk for a diagnosis of obesity (BMI 25) may receive primary health
screenings to increase awareness about the health risk factors associated with
obesity. Secondary intervention aims to prevent the progression of obesity (BMI
>25 to 30) from becoming a chronic health issue and tertiary intervention (BMI
> 30) attempts to maintain quality of life with chronic obesity (Garvey,
Mechanick, & Einhorn, 2014).
Following a comprehensive review of systems, modifiable risk factors, and
laboratory evaluations of blood work, clients may be prescribed pharmacological
intervention or psychiatric consultation. More often, immediate interventions for
weight loss, such as pharmacological interventions, are used to increase
motivation and promote weight loss. It is important to recognize that side effects
of some medications increase hunger, promote overeating, and result in weight
gain or obesity. Glucocorticoids produce an increase in truncal adipose tissue
and insulin and oral hypoglycemic drugs increase fat in tissues of persons with
diabetics. Antipsychotics such as phenothiazine and antidepressants (selective
serotonin reuptake inhibitors) also produce weight gain. Adrenergic antagonists
such as propranolol reduce sympathetic nervous system acidity and lead to
weight gain (Atkinson, 2005). Some of the Food and Drug Administration
(FDA)–approved medications for the treatment of obesity may cause serious side
effects and must be monitored. Currently, orlistat (Xenical) is used to absorb
fats, decrease side effects including oily and frequent bowel movements, and
increase absorption of fat-soluble vitamins (A, D, E, and K), so a multivitamin
may be prescribed. Alli is a similar, less potent over-the-counter (OTC) version
of orlistat. Qsymia, previously known as Qnexa, reduces appetite, contributes to
satiety, and alters the taste of food. Belviq alters serotonin levels in the brain and
may lead to addiction (Mance et al., 2013). Cortisol blockers are not yet FDA
approved for weight management but may be used in the future to reduce the
formation of central adipose tissue (Zeratsky, 2015).
There are many diets that claim to result in various levels of weight loss.
Popular diets used for weight loss include Atkins, Human chorionic growth
hormone (HCG) South Beach, Weight Watchers, the Zone, and Body for Life.
The Atkins diet is a low-carbohydrate, high-protein diet that burns fat for fuel.
Side effects may include reduced brain glucose and increased blood lipid levels.
HCG is extracted from the urine of pregnant women and injected, while
prescribing a restriction of calories to 500 per day. There is minimal evidence to
support the efficacy of HCG, and it is not FDA approved. The South Beach diet
is supported by research; balances carbohydrates, protein, and fat; and does not
limit fruits and vegetables, which is a disadvantage for maintaining a restricted
pattern of eating. The Zone diet requires 30% each of fats, protein, and
carbohydrates and focuses on healthy grains and fiber. Body for Life program
encourages six small meals per day to maintain stable blood sugar. Weight
Watchers is well researched and uses a point system, a balanced meal plan, and a
support group or online participation. Although there are many diets from which
to select, a 69% obesity rate continues in the U.S.
Bariatric Surgery
Bariatric surgery is often recommended for persons with super or morbid
obesity or for a BMI ≥ 40; a BMI of 35 with accompanying metabolic syndrome
or sleep apnea may also benefit from surgical intervention. The goal of bariatric
surgery is to lose 50% of body weight or achieve a healthy target weight. An
interprofessional bariatric health care team may include a bariatric physician or
surgeon, nurse, psychiatrist, dietician or nutritionist, physical therapist, and
occupational therapist.
Bariatric surgery produces a quick reduction in weight; when combined with
a healthy diet and consistent physical activity, weight loss can be sustained over
a period of time. There are two general surgical strategies: stapling and banding.
Within these two categories, there are several surgical procedures available.
Each may be associated with complications such as diarrhea, bleeding, infection,
malabsorption and lower extremity blood clots (Table 28.4; Figure 28.2).
TABLE 28.4 Advantages and Disadvantages of Different Types of Bariatric

Surgery
Figure 28.2 Two surgical procedures for morbid obesity. A. Gastric bypass with
Roux-en-Y. A horizontal row of staples creates a pouch with a capacity of 50 mL
or less. The proximal jejunum is transected and the distal end anastomosed to the
new pouch. The proximal segment is anastomosed to the jejunum. B.
Laparoscopic gastric banding. The silicone adjustable band component of the lap
band is placed around the upper part of the stomach, forming a small gastric
pouch (15 mL) to limit intake and slow gastric emptying. Size is adjusted by
injection of saline into a subcutaneous reservoir that is connected to the lap band.
(From Farrell, M., & Dempsey, J. (2010). Smeltzer and Bare’s textbook of
medical–surgical nursing (2nd ed.). Philadelphia, PA: Wolters Kluwer.)
Obesity is a modifiable risk factor that impacts multiple client factors and
performance skills, patterns, and occupational engagement. Persons with obesity
may experience changes in mental functions (mood) associated with altered
levels of serotonin. Medications used to treat depression may result in weight
gain but aid in appetite control. Excess weight aggravates symptoms of OA
resulting in loss of joint structures, destruction of cartilage, and spinal and
peripheral nerve compression. Joint pain, malalignment, reduced range of
motion, and disuse atrophy limit mobility and activity tolerance.
Diminished skin integrity is another challenge for persons with obesity.
Loose skin may cause friction and reduced skin integrity between the thighs
during ambulation. Excess skin may need to be camouflaged by wearing larger
rather than fitted clothing. Because of multiple skin folds associated with pannus
under the breasts, around the abdomen, and perineal area, it is difficult to
maintain hygiene following urination or bowel movement. Clients may become
embarrassed to have a family member or caregiver perform this task. Skin may
have poor vascularization secondary to atherosclerosis and lymphedema.
Prolonged sitting in one position or changing position may result in skin
shearing, abrasions, or other pressure sores. Clients may be dependent for
pressure relief including lateral weight shifts and raises.
Performance of occupations (such as activities of daily living) that are
usually performed in the bathroom (bathing and toileting) and self-feeding a
meal in the kitchen or dining room are now performed in the same location,
which is often in bed. These clients may experience shame and loss of self-
esteem and prefer social isolation. Loss of intimacy and lack of human touch
may also hinder self-concept. Lack of mobility and tolerance to supine and prone
positions in bed may interfere with breathing. Positioning in a wheelchair or
chair may be ill tolerated secondary to neuropathy, peripheral vascular disease,
or pressure sores related to type II diabetes. Clients may perform at a slower
pace and require a wheelchair or other mobility aide to access the community.
The increased cost for larger assistive technology such as a wheelchair and
accessible van may not be reimbursed by the insurance provider. The inability to
fit into a standard-size chair, car seat, or restaurant booth restricts driving, social
participation, educational pursuits and work.
The lack of awareness of clearly defined occupations that promote planned
energy expenditure versus energy conservation may impede progressive and
graded physical activity needed to achieve a steady reduction in weight over
time. Altered roles, habits and previously performed routines must be modified
to achieve maximum participation in occupations.
CASE-STUDY 1
Candice is a 10-year-old female who is 5′4″ and weighs 234 lb. She has
three brothers who are active in a variety of sports and are of normal
weight. Both parents are slightly overweight and believe Candice will grow
out of her obesity. Candice snacks throughout the day and prefers cookies,
candy, and fried chicken fingers for lunch. She consumes 3 to 4 cans of
sugared soda daily. During the last physician’s visit, a thyroid panel
consisting of a T4 and T3 was ordered along with a screening for
depression. The thyroid panel was negative, but Candice demonstrated
signs and symptoms of depression. She informed her mother that she has
been feeling stressed at school because she is being bullied and teased about
her weight. Her brothers usually hang out with their friends and were not
aware of the teasing and bullying. Candice has asked her parents if she can
get bariatric surgery to help lose weight so that she may be treated like
other girls. Her parents are slow to agree with the surgery because the
bullying may not change after the surgery. Candice’s parents have asked her
if she would like to transfer to another school.
CASE-STUDY 2
Mildred is an 85-year-old woman who is a resident of a long-term care
facility. She has lived at the facility for 10 years, and her BMI has increased
from 30 (260 lb) to 40 (380 lb). She has difficulty sleeping in a flat position
in bed. Her roommate informed the nurse that Mildred’s snoring and
breathing keeps her awake at night. Mildred complains of knee pain
associated with OA and increasing difficulty with standing and ambulation;
a wheelchair is used for long distances. The nursing staff noticed that her
participation in transfers to bed, toilet, and shower chair are becoming more
difficult. Over the last 3 months, the facility replaced two commodes that
cracked when the client sat down. The occupational therapist is
recommending a commode with a load capacity of 500 to 1,000 lb. The
certified nursing assistant (CNA) reported that Mildred’s feet continue to
swell and she has developed a rash between breast, abdominal, and perineal
skin folds. The facility administrator and director of nursing is concerned
about the safety and well-being of the client and staff and adherence to state
nursing home regulations. Mildred has expressed a desire to remain at this
facility because the staff treats her like family. She realizes she does not
have the willpower to lose weight and has requested to be moved to a room
that has a larger bed, commode, and mechanical lift that can handle her
weight. Patient rights indicate that the client has the right to eat and gain
weight.
Learning Resources
Center for Disease and Prevention Adolescent Growth Charts
http://www.cdc.gov/healthyweight/assessing/bmi/childrens_bmi/about_childrens_bmi.html
Center for Disease Control and Prevention BMI Calculation for BMI
Percentile Calculator for Child and Teen English Version
https://nccd.cdc.gov/dnpabmi/calculator.aspx
Bariatric Surgery Resources
http://obesityreporter.com/bariatric-weight-loss-surgery-statistics-updated-
2015/
https://asmbs.org/patients/bariatric-surgery-misconceptions
http://www.bariatric-surgery-source.com/obesity-united-states-
statistics.html
http://www.medpagetoday.com/Surgery/GeneralSurgery/39629
https://asmbs.org/resources/estimate-of-bariatric-surgery-numbers
http://www.pharmacytimes.com/publications/issue/2014/July2014/Complications-
of-Bariatric-Surgery-Dumping-Syndrome-and-Drug-Disposition
http://www.ncbi.nlm.nih.gov/pubmed/20622654
http://www.sciencedirect.com/science/article/pii/S1550728909005309
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Glossary
Activity demands: The aspects of the task that influence the performance by
the person. These demands include the objects used and their properties,
space demands, social demands, sequencing and timing, required actions,
required body functions, and required body structures.
Addiction: Chronic ingestion of substances or engagement in activities in spite
of harmful consequences; when a person experiences both the symptoms of
tolerance and withdrawal.
Affect: Observable facial expressions of a feeling or emotion. Patients may be
described as having a flat or blunt affect.
Affective and physiologic dysregulation: Behaviors include difficulty with
arousal regulation and modulation and toleration and recovery from
extreme affective states such as fear, anger, and shame that emerge from
chronic trauma exposure, manifested by prolonged and extreme emotional
tantrums on one side of the spectrum to catatonic-like immobilization.
Age-related macular degeneration (AMD): A low vision disorder that results
from loss of function of the macula, which is the center of the retina. There
are two types of AMD including dry AMD (atrophic) and wet AMD
(neovascular/exudative). Dry AMD is atrophy of the macula and wet AMD
is an in growth of new blood vessels that bleed into the subretinal space.
Agnosia: Inability to recognize the import of sensory impressions despite being
able to recognize the elemental sensation of the stimulus. Language deficits
must be absent for this diagnosis. Various agnosias correspond to several
senses and may be distinguished as auditory, gustatory, olfactory, tactile,
and visual. Specific sensory agnosias can occur when the connections are
disrupted between the primary cortical receptor region for a stimulus and
the memory of prior stimuli. For example, the inability to recognize
familiar objects by sight or touch.
Agoraphobia: “A mental disorder characterized by an irrational fear of leaving
the familiar setting of home, or venturing into the open; often associated
with panic attacks.” Commonly referred to as fear of the marketplace.
Agrammatica: Grammatical simplification and errors; effortful halting speech.
Alexithymia: The impaired capacity to describe emotions or bodily states; is a
persistent sign of affective dysregulation.
Algorithms: A step-by-step approach to treatment based upon controlled
clinical trials.
Allogeneic bone marrow transplant: The individual undergoes high-dose
chemotherapy or radiation and then receives stem cells from a HLA-
matched donor.
Allograft: An allograft (also referred to as a homograft or cadaver skin) is
donor skin taken from another person.
Alogia: Absence of speech because of mental illness.
Altruism: The unselfish concern for the welfare of others. This concept is
reflected in actions and attitudes of commitment, caring, dedication,
responsiveness, and understanding.
Alveoli: Small sacs in the lungs which perform the gas exchange. Oxygen is
moved into the blood stream from the alveoli to be carried to cells
throughout the body.
Ambiguous hand preference: Absence of a dominant hand preference; the
switching of hands within the same activity.
Aminobutyric acid (GABA): Primary inhibitory transmitter; directly involved
in regulation of muscle tone.
Amotivation: Lacking in motivation.
Amygdala: A structure that is part of the limbic system and is involved in the
emotions of aggression and fear.
Amyloid plaques: Insoluble shards of amyloid that stick to dead and dying
neurons.
Amyloid precursor protein (APP): A benign substance that lives in various
parts of the body, including the brain, and whose role in cellular function is
unknown.
Amyotrophic lateral sclerosis (ALS): A progressive neurological disorder
characterized by degeneration of motor neurons located in the ventral horn
of the spinal cord.
Analgesic: A pain medication.
Anemia: Decrease in the number of red blood cells (RBC) or less than the
normal quantity of hemoglobin in the blood.
Aneurysm: Localized abnormal dilatation of the wall of a blood vessel, usually
an artery, or in the heart, resulting in the formation of a sac. Usual causes
are the weakness of the vessel wall, resulting from atherosclerosis, or a
congenital defect. However, any injury to the middle or muscular layer of
the arterial wall can predispose the stretching of the vessel wall.
Angioplasty: is a procedure where a small mesh tube is inserted into the
coronary artery to widen the opening, thus increasing the blood flow.
Angle-closure glaucoma: The obstruction by the iris to the outflow pathway of
aqueous humor at the angle between the peripheral cornea and the iris
aqueous humor.
Anhedonia: Reduced ability or inability to experience pleasure.
Ankylosis: The fusion of a joint, often in an abnormal position, usually
resulting from destruction of articular cartilage and subchondral bone, as
occurs in rheumatoid arthritis.
Anomia: Inability to name objects.
Anterograde amnesia: The inability to learn new long-term declarative
information.
Antibodies: A specialized immune protein produced because of the
introduction of an antigen into the body. The production of antibodies is a
major function of the immune system.
Anticholinergic side effects: Most commonly dry mouth or constipation.
Antigen: A microorganism that is a uniquely configured protein that attaches to
the pathogen and stimulates the body’s production of antibodies.
Antipsychotic medications: Medications designed to reduce positive and
negative symptoms.
Anxiety: “Apprehension of danger, and dread accompanied by restlessness,
tension, tachycardia, and dyspnea unattached to a clearly identifiable
stimulus.”
Anxiolytic: A sedative or mild tranquilizer used to treat anxiety or alcohol
withdrawal.
Apathy: An absence or suppression of emotion, feeling, or concern.
Aphasia: Difficulty with expressive and/or receptive language.
Apophyseal: Refers to the articulations between the articular facets of adjacent
vertebrae, or facet joints. These joints are a frequent site of degenerative
joint disease, or osteoarthritis, spondylitic and traumatic diseases.
Apple ascites: Weight centered around the abdomen.
Apple pannus: Increased hip width and weight around the pelvis.
Apraxia: Loss of skilled purposeful movement that cannot be attributed either
to deficits in primary motor skills or problems in comprehension. It can
affect ideation and concept formation as well as programming and planning
of movement.
Aqueous humor: Thin and water-like fluid located in the anterior and posterior
chambers of the eye.
Arrhythmia: Any changes from the normal sequence of electrical impulses in
the heart, causing the heart to beat too slowly, too quickly, or irregularly.
Arteriovenous: Referring both to an artery and a vein. An arteriovenous
malformation, or AVM, is a congenital malformation characterized by an
abnormal collection of blood vessels near the surface of the brain. AVM can
lead to subarachnoid hemorrhage.
Arthritis: Inflammation or infection of a joint.
Arthroplasty: Joint replacement.
Asperger’s disorder: A neurobiologic disorder of unknown etiology in which
cognitive and communication skills develop normally or nearly normally
during the first few years of life. However, social impairment is evident,
repetitive and stereotypical behaviors are observed, and communication,
though not delayed, is marked by unusual topics of interest and rigidity.
Aspiration: Pathological inhalation of food or mucus into the respiratory tract.
Associality: A negative symptom characterized by decreased interest in
socialization and maintenance of relationships.
Associated reactions: Involuntary movements or reflexive increases in tone on
the affected side of individuals with hemiplegia. These movements replicate
synergy patterns and often appear when the individual is trying new or
stressful activities, for example, a resisted grasp by the noninvolved hand
causes a grasp reaction in the involved hand.
Astereognosis: Inability to recognize objects by touch (also called tactile
agnosia; see Agnosia).
Asterixis: A hand-flapping tremor.
Ataxia: Inability to coordinate muscle activity during voluntary movement. In
posterior column damage of the spinal cord, incoordination and a loss of
proprioception is caused by misjudgment of limb position with balance
problems. Cerebellar ataxia produces a reeling, wide-based gait.
Atheromas: An abnormal mass of fatty or lipid material with a fibrous
covering, which forms a discrete raised plaque within the inner layer
(intima) of an artery.
Atherosclerosis: A widespread form of arteriosclerosis in which deposits of
plaques (atheromas) containing cholesterol and other lipid material are
formed within the inner layer (intima) of large- and medium-sized arteries.
Athetoid (dyskinetic): Resembling athetosis or repetitive involuntary, slow,
sinuous, writhing movements. Classification of cerebral palsy in which
involuntary purposeless movement occurs when an individual attempts
purposeful motion. The abnormal movements may not only occur in the
limb being moved but also involve an “overflow” of activity to all the other
limbs with an exaggeration of reflexes.
Atrial fibrillation (Afib): Irregular, often rapid, heart rate that can lead to poor
blood flow to the body between air sacs in the lungs are damaged causing
the air sacs to lose with shape. Damage can also destroy the walls of the air
sacs, leading to fewer and larger air sacs instead of many tiny ones in a
normal lung. When this occurs, the quantity of gas exchange in the lungs is
reduced.
Atrophy: A wasting of tissue that results in decreased muscle mass.
Attentional and behavioral dysregulation: Signs and symptoms include
impairment in those competencies related to sustained attention, learning,
or coping with stress. An inability to sustain goal-directed behavior may
include a lack of curiosity, difficulties with planning or completing tasks, or
avolition.
Auditory processing: The process of the brain recognizing and interpreting
sounds in the environment. This process is responsible for the
comprehension of language.
Autistic disorder: A neurobiologic disorder with an unknown etiology,
characterized by significant delay or deviation in social interaction,
communication, and repetitive or stereotyped behaviors.
Autograft: The surgical transplantation of the patient’s own skin from one area
to another.
Autologous bone marrow transplant: Stem cells are removed from the
individual while in remission from cancer and are frozen, and after the
individual undergoes high-dose chemotherapy or radiation, those stem cells
are replaced to begin making new blood cells.
Autonomic dysreflexia (hyperreflexia): An uninhibited and exaggerated
reflex of the autonomic nervous system to stimulation. The response occurs
in about 85% of all patients who have spinal cord injury above the level of
the 6th thoracic vertebra. It is potentially dangerous because of attendant
vasoconstriction and immediate elevation of blood pressure, which in turn
can bring about hemorrhagic retinal damage or cerebrovascular accident.
Less serious effects include severe headache, changes in heart rate,
sweating and flushing above the level of the spinal cord injury, and pallor
and goose bumps below that level.
Autonomic neuropathy: A form of peripheral neuropathy. A group of
symptoms that occur when there is damage to the nerves that manage every
day body functions such as blood pressure, heart rate, bowel and bladder
emptying, and digestion.
Autosomal dominant: A disease that occurs when a child inherits a normal
gene from one parent and an affected gene from the other parent.
Autosomal recessive: A disease that occurs when both parents carry and pass
on the affected gene.
Avolition: A lack of interest or motivation to engage in goal-oriented behavior.
Babinski’s reflex: Dorsiflexion (extension) of the great toe and outspreading of
the outer toes when the sole of the foot is stroked. Normally present in
infants under the age of 6 months, in adults this reflex occurs in lesions of
the pyramidal tract.
Bariatrics: A term often associated with persons who are morbidly obese.
Benign tumor: Noncancerous, which do not invade other body tissues or
spread to other body parts. However, may become life threatening as they
cause increasing deficits with cell growth, because they press upon nearby
structures and tissues.
Beta-amyloid plaques: An accumulation of amyloid precursor proteins that
have not been correctly broken down in the body.
Body dysmorphic disorder: A severe preoccupation with a perceived defect in
physical appearance that results in distress and serious social or
occupational functioning impairment.
Body mass index: An international standard used to determine degree of
obesity relative to height and is calculated by multiplying weight in
(pounds) × 703 and then dividing by the height in inches squared.
Bone marrow: The spongy center of certain bones forms three types of blood
cells: RBC, WBC, and platelets. RBC, also called erythrocytes, carry
oxygen throughout the body and bring carbon dioxide back to the lungs.
Bone marrow transplant: is a procedure in which the damaged or destroyed
stem cells are replaced with healthy stem cells.
Boutonniere deformity: A common hand deformity secondary to the
pathologic effects of rheumatoid arthritis resulting in a combination of PIP
joint flexion and DIP joint hyperextension Bradycardia: Heart rate slower
than normal heart rate. Heart rate below 60 beats per minute.
Bradykinesia: Slowness of all voluntary movement and speech.
Brain attack: Synonym for stroke, or cerebrovascular accident.
Brainstem: connects the cerebrum with the spinal cord and includes the
midbrain, medulla oblongata, and the pons. Motor and sensory neurons
travel via the brainstem which allows for communication between brain and
spinal cord. The brainstem also controls life-supporting autonomic
functions of the peripheral nervous system.
Bronchitis: Contributing condition of chronic obstructive pulmonary disease.
Causes the lining of the airways to become irritated and inflamed causing
the lining to thicken. Buildup of mucus leads to difficulty breathing.
Bronchopulmonary dysplasia: A chronic lung disease of babies, which most
commonly develops in the first 4 weeks after birth and most often affects
babies born at least 4 weeks before term.
Burden of disease: A situation in which a group experiences an illness to a
greater degree than other subgroups.
Burn scar contracture: Forms due to the shortening and tightening of the burn
scar. Burn scar contracture deformities are the most problematic over large
joints.
Bursitis: Inflammation of the fluid-filled sac (bursa) that lies between a tendon
and skin, or between a tendon and bone.
Cancer: refers to a collection of related diseases in which some of the body’s
cells divide without control and are able to invade other tissues.
Cancer cachexia: An often irreversible syndrome leading to substantial weight
loss including skeletal muscle and body fat.
Cancer-related cognitive dysfunction (CRCD): refers to various cognitive
weaknesses an individual may experience following chemotherapy
treatments.
Cancer-related fatigue (CRF): is a common side effect of cancer and the
associated treatments. CRF impacts anywhere from 40% to 100% of people
with cancer.
Carcinoma: are cancerous tumors that arise in epithelial tissues of skin, blood
vessels, and lining of the cavities and organs.
Carotid endarterectomy: Excision of thickened atheromatous areas of the
innermost layer of an artery (intima) to improve circulation.
Carpal tunnel syndrome: Resulting from compression on the volar aspect of
the wrist, which then impinge upon the median nerve. This causes
paresthesia of the palmar aspect of the thumb, the second and third digits,
and the radial aspect of the fourth digit.
Casein: A protein found in cow’s milk. A theory in the field of autism suggests
that children with autism do not digest this protein appropriately, leading to
a buildup of morphine-like substances in the body and causing social
withdrawal and abnormal behaviors.
Cataracts: Opacifications of the crystalline lens of the eye, which result in a
decreased amount of light reaching the retina.
Catatonia: Extreme psychomotor agitation or retardation.
Catatonic behavior: Loss of responsiveness to environmental cues. A person
with catatonic behavior may assume rigid or bizarre postures and resist
attempts made to move or reposition him or her. Excessive, nonpurposeful
motor activity may also be observed. In extreme cases, the individual
appears to be completely unresponsive, as in a catatonic stupor.
Catheterization: Passage of a catheter into a body channel or cavity, especially
introduction of a catheter via the urethra into the urinary bladder.
Cauda equina: The collection of dorsal and ventral nerve roots descending
from the lower spinal cord and occupying the vertebral canal below the
cord at the L1 region.
Central scotomas: Diminished vision or visual loss in the center of the visual
field.
Central vision: Sharp, foveal vision used for reading, watching television,
driving or any activity for which visual detail is of primary importance.
Cerebellum: A region of the brain that is found at the rear of the head and is
above the brainstem. It has been traditionally known for its role in the
coordination of movement, but recent research suggests the cerebellum may
also have a role in cognitive, sensory, language, and abstract-thinking skills.
Cerebrospinal fluid assays: A diagnostic tool that measures biomarkers in
cerebrospinal fluid.
Cerebrovascular accident (CVA, stroke, brain attack): Disorder of the blood
vessels serving the brain, resulting in disruption of blood flow to parts of
the brain leading to neuronal death. Impact of CVA varies according to the
cause of the disruption (blockages or hemorrhages) and location of the
insult, as well as a variety of premorbid and psychosocial conditions.
Charcot-Marie-Tooth (CMT): An inherited neurological disorder that results
in motor and sensory peripheral neuropathy.
Chemotherapy-induced peripheral neuropathy (CIPN): A side effect of
chemotherapy that causes altered sensations of tingling, numbness, and
weakness impacting primarily the hands and/or feet, occurs as
chemotherapy spreads throughout the body, damaging the distal portions of
the nerves.
Childhood disintegrative disorder: A rare neurobiologic disorder that
develops in children who had appeared completely normal during their first
few years of life. Following its onset, language, social interaction, and self-
care skills are lost and autistic-like symptoms appear.
Chondropenia: A loss of cartilage that occurs faster than the rate of self-repair.
Chronic bronchitis: Inflammation of the bronchial tubes resulting in a
thickening of the walls, making it difficult to breathe.
Chronic obstructive pulmonary disease (COPD): A term that includes
chronic bronchitis, asthma, emphysema, and bronchiectasis. The condition
is irreversible and characterized by progressive limitation in the flow of air
in and out of the lungs.
Chronic pain: Pain that causes significant distress or impairment in social,
occupational, or other important areas of functioning for a duration of over
6 months.
Cilia: Hair-like structures in the lungs airways that help to move the mucus out
and clean the airways.
Circumlocution: The use of pantomime, nonverbal communication, or word
substitution to avoid revealing an inability to say or remember a word.
Client factors: The body functions and the body structures that reside within
the person.
Clinical presentation: With regard to ADHD, a method of describing the
disorder without rigid subtypes as previously used in the DSM-IV.
Clonus: Alternate involuntary muscular contraction and relaxation in rapid
succession.
Closed fracture: Refers to a fracture that has not broken through the skin.
Cognitive-behavior therapy: A therapeutic approach used to help patients
develop a positive pattern of thought or actions in response to identified
difficulties in everyday life.
Cogwheel rigidity: Stiff movement in one direction in small evenly spaced
segments. The tension in muscle gives way in little jerks when the muscle is
passively stretched.
Collagen: A basic structural fibrous protein found in all tissue. Excessive
deposition of tissue collagen leads to a thickening of the burn scar.
Collateral circulation: Secondary circulation that continues to an area of the
brain following obstruction of a primary blood vessel and may prevent
major ischemia.
Colles’ fracture: Fracture resulting in dorsal displacement, dorsal
comminuting, and radius shortening; often referred to as a dinner fork
deformity given its resemblance to an upside down fork.
Coma: A nonsleep loss of consciousness associated with unresponsiveness to
touch, pain, sound, or movement that lasts for an extended period.
Comminuted fracture: A fracture in which a bone in broken, splintered, or
crushed into a number of pieces.
Compensation: The use of one system or muscle group to complete the work
not capable of being completed by the usual system.
Complex regional pain syndrome (CRPS): Chronic pain condition that
typically affects one of the limbs of either the upper and lower extremity
and is believed to be caused by a disorder of the peripheral and central
nervous system.
Complex trauma: The result of multiple exposure to traumatic events,
maltreatment, or polyvictimization beginning in early childhood or
adolescent, occurring within the context of unpredictable, uncontrollable,
and violent environments in conjunction with inconsistent or absent
protective caregiving.
Compound fracture: Also referred to as an open fracture as the fractured bone
breaks through the skin surface and are more prone to infection.
Compression garment: Compression garments are the preferred conservative
method to treat -hypertrophic scars and have been in use since the early
1970s. Compression garments are thought to reduce oxygen flow to the scar
thereby decreasing collagen production.
Compulsion: “Uncontrollable impulses to perform an act, often repetitively, as
an unconscious mechanism to avoid unacceptable ideas and desires which,
by themselves, arouse anxiety; the anxiety becomes fully manifest if
performance of the compulsive act is prevented; may be associated with
obsessive thoughts.”
Computerized tomography: A computerized scanning system that reveals
changes in the brain’s structure and function.
Concrete thinking: Literal interpretation of words without understanding
abstract meanings.
Congenital: A condition existing before birth, at birth, or that develops during
the first month of life.
Congestive heart failure: The inability of the heart to function as a pump,
which results in a decreased blood flow to the tissues and congestion in the
pulmonary and circulatory systems.
Constructional apraxia: Apraxia caused by loss of visuospatial skills; for
example, difficulty drawing a clock face.
Context: The conditions that surround the person. Those conditions include
cultural, physical, social, personal, spiritual, temporal, and virtual contexts.
Contracture: Abnormal shortening of muscle tissue that renders the muscle
highly resistant to stretching, which can lead to permanent disability. In
many cases, contractures can be prevented by range of motion (ROM)
exercises (active or passive) and by adequate support of the joints to
eliminate constant shortening or stretching of the muscles and surrounding
tissue.
Contrast sensitivity: The ability to visually discern the edges or luminance of
an object.
Contrecoup injury: occurs when the brain reaccelerates in the opposite
direction after the initial strike (coup) against the skull.
Cooccurring disorders: When a person is diagnosed with a substance use
disorder and other psychiatric conditions.
Core values: in occupational therapy, seven values that are at the foundation of
the belief systems that occupational therapists use as a moral guide when
making clinical decisions.
Coronary artery bypass grafting (CABG): A process in which arteries or
vein from other parts of the body (often the legs) are “harvested” and then
surgically attached to bypass the blocked arteries near the heart.
Coronary artery disease: Tied very closely to atherosclerosis where plaque,
made of cholesterol, fat, calcium, and other substances, sticks to the inner
lining of arteries that are taking oxygen-rich blood from the heart to the
body.
Cortical atrophy: Birth defect with a nongenetic origin that can contribute to
or cause ID due to shrinkage of the posterior part of the brain.
Corticobulbar: Refers to the pathway connecting the cerebral cortex to the
brainstem. Bulbar refers to the nerves and tracts connected to the medulla;
muscles innervated include those of the tongue, pharynx, and larynx.
Corticospinal: Also referred to as the pyramidal tracts; are descending tracts
which conduct motor impulses from the brain to the spinal cord.
Corticosteroids: A class of steroids that are produced in the adrenal cortex.
Synthetic versions are produced to closely resemble these steroids and are
used for medicinal purposes.
Cortisol: A steroid hormone that is activated in response to stress and
hypoglycemia and is believed to trigger depressive symptoms.
Coup-contrecoup injury: Situation in which direct brain damage is incurred in
traumatic event as the cerebrum rotates on the more stable brainstem while
accelerating from the force of impact. The cerebrum strikes the skull
(coup), and then accelerates in the opposite direction.
Craniostenosis: Birth defect with a nongenetic origin that can contribute to or
cause ID due to premature fusion of the skull resulting in a reduction in
space for the brain to grow and in increased intracranial pressure.
Cravings: A desire for the drug that precludes thinking of anything else and are
associated with specific reward pathways in the brain.
Creatine kinase: An enzyme expressed by various tissues and cells in the
body.
Credé’s method: Use of manual pressure on the bladder to express urine,
particularly in bladder training for individuals with paralysis. The hands are
held flat against the abdomen, just below the umbilicus. A firm downward
stroke toward the bladder is repeated six or seven times, followed by
pressure from both hands placed directly over the bladder to manually
remove all urine.
Crepitus: A “crunching” feeling or sound coming from the articular surface
noted in both passive and active mobility of a given joint.
Cyanosis: A bluish color to the skin (commonly evident in lips and fingernails)
that is usually due to decreased oxygen levels.
Cyclothymia: Characterized by a chronic course of hypomania and mild
depression, this disorder presents as a mild form of bipolar disorder (BPD).
Cytomegalovirus: A herpesvirus that can result in a congenital infection.
DAMP: A condition associated with ADHD that includes deficits in attention,
motor control, and perceptual processing.
De Quervain’s disease: Pathologic process that involves extensors at the
thumb, causes severe pain and discomfort, resulting in a decrease in hand
function and the ability to grip.
Debridement: The cleansing and removal of nonadherent and nonviable tissue.
Decerebrate rigidity: An extensor posture of all limbs and/or the trunk.
Decorticate rigidity: Flexion of the upper, but extension of the lower, limbs.
Decubitus ulcers: An ulcer caused by local interference with the circulation,
usually occurring over a bony prominence at the sacrum, hip (trochanter),
heel, shoulder, or elbow. It begins as a reddened area and can quickly
involve deeper structures and become an ulcer, also called a bedsore or a
pressure sore.
Decussation: An X-shaped crossing, especially of nerves or bands of nerve
fibers, connecting parts on opposite sides of the brain or spinal cord.
Deep vein thrombosis (DVT): A thrombosis, commonly seen in the legs or
pelvis, that results from phlebitis, vein injury, or prolonged bed rest.
Deformity: Distortion of any part or general disfigurement of the body.
Delayed union: A delayed union is when the bone takes more time to heal than
is expected; it heals slowly. It may be suspected when pain and tenderness
persist at the fracture site 3 months to 1 year after the injury.
Delirium: usually a temporary disturbance in mental abilities that results in
confused thinking and reduced awareness that is often a result of an injury
and illness.
Delirium tremens (DTs): A condition caused by cessation of alcohol use
among chronic users that includes seizures, hallucinations, and severe
tremors that can be life threatening.
Delusions: Atypical and well-organized beliefs not explained by evidence or
culture.
Delusions of grandeur: Self-inflated views usually present in conversation.
Delusions of persecution: Individuals have consistent and organized beliefs
about other people intending to harm them.
Demyelination: Destruction, removal, or loss of the myelin sheath of a nerve
or nerves.
Developmental coordination disorder: Difficulty coordinating movements,
characterized by marked motor impairment in motor coordination that is not
due to a medical condition or developmental disorder and interferes
significantly with occupational performance.
Developmental trauma disorder: Describes clinically significant patterns of
behavior or syndromes that occur in an individual as a result of persistent
and chronic exposure to violent episodes including physical, sexual, and
emotional abuse resulting in functional impairment in one or more
important areas.
Diabesity: refer to the presence of a combination of risk factors including
obesity, diabetes, hypertension, high cholesterol, elevated fasting blood
sugar, high triglycerides, systemic inflammation, and a tendency to form
blood clots.
Diabetic foot: A person who has diabetes is at increased risk for slow-healing
injuries to distal extremities. It is caused by decreased vascularization; the
risk of an infection that does not heal is higher. One possible intervention is
limb amputation.
Diabetic ketoacidosis: State of medical emergency; life-threatening condition
caused by a state of relative insulin deficiency, characterized by
hyperglycemia, ketonemia, metabolic acidosis, and electrolyte depletion.
Often the first presenting sign of type 1 diabetes.
Diarthroses: are the most mobile joints and are by far the most common
articular pattern. Because these joints possess a synovial membrane and
contain synovial fluid, these joints are more commonly referred to as
synovial joints.
Diastolic blood pressure: The pressure in the arteries when the heart is at rest.
Diffusion-tensor imaging: A diagnostic tool that uses MRI methods to
produce images of the structure and organization of the connections
throughout the central nervous system.
Dignity: The importance of valuing the inherent worth and uniqueness of each
person. This value is demonstrated by an attitude of empathy and respect
for self and others.
Diplegia: Paralysis of like parts on either side of the body. In cerebral palsy,
diplegia describes involvement of the lower extremities predominantly, with
only mildly affected upper extremities.
Disease surveillance: An epidemiological practice by which the spread of
disease is monitored in order to establish patterns of progression.
Disease trajectory: Patterns of progression and functional decline that result
from a condition.
Disease-modifying antirheumatic drug (DMARD): is often the first
medication introduced as it has the best chance to achieve low disease
activity or remission.
Disinhibition: Loss of inhibition, or the cultural and social sense of self
restraint.
Disorganized behavior: Unpredictable, socially inappropriate behaviors that
interfere with daily activities. Examples include agitated or angry outbursts
with no known provocation; sexually acting out in public, that is,
masturbation; and difficulties performing goal-directed tasks such as meal
preparation or grooming.
Disorganized thinking: Thought processes that lack cohesive patterns. Speech,
which can provide clues about disorganized thought processes, may include
any of the following: loose associations (answers that begin to veer “off
track” of the original questions), tangential (unrelated comments or
answers) or incoherent (a mixture of words often referred to as a “word
salad”).
Disseminated intravascular coagulation (DIC): is an oncologic emergency
encompassing both excessive bleeding and thromboembolic events and is
common in leukemia, in particular acute promyelocytic leukemia.
Dissociation: is essentially an “out-of-body experience” in which the person
detaches from an event that is overwhelmingly stressful.
Distal radius fracture: Common fracture of the radius. Due to its proximity to
the wrist joint, often referred to as a wrist fracture.
Dopamine: A neurotransmitter that helps regulate movement and emotional
responses; a deficiency results in Parkinson’s disease.
Down syndrome: A chromosomal condition caused by the presence or all or
part of an extra 21st chromosome.
Dysarthria: Imperfect articulation of speech caused by disturbances of
muscular control resulting from central or peripheral nervous system
damage.
Dysautonomia: describes a number of conditions that affect the autonomic
nervous system.
Dysesthesia: Impairment of any sense, especially of the sense of touch; a
painful, persistent sensation induced by a gentle touch of the skin.
Dysgraphia: Inability to write due to neurological impairment.
Dyslipidemia: Abnormal levels of lipids (fats) in blood.
Dysosmic aphasia: Difficulty naming objects.
Dysphagia: Difficulty in swallowing, which may range from mild discomfort
to a seriously compromised ability to control the muscles needed for
chewing and swallowing. Dysphagia may seriously interfere with a
patient’s nutritional status. Interventions, such as positioning the patient
during feeding and use of foods of the proper consistency, are widely used.
Dysphoria: A mood disturbance resulting in a depressed mood.
Dyspnea: Difficult, labored, or painful breathing.
Dyspraxia: Difficulty with motor planning; the inability to plan unfamiliar or
novel tasks.
Dysthymia: Unlike individuals with depressive disorders, people with
dysthymia report that they have always been depressed. Characterized by
insidious onset frequently beginning in childhood, a persistent or
intermittent course, and ongoing feelings of depression over a period of at
least 2 years.
Dystonic movements: Neurological movement disorder in which sustained
muscle contractions cause twisting and repetitive movements or abnormal
postures, resulting in a clumsy, awkward or maladaptive response.
Dystophin: A protein that connects the cytoskeleton of a muscle fiber to the
surrounding extracellular matrix through the cell membrane. It is
responsible for maintaining the shape and structure of the muscle fiber.
Ecchymosis: Skin discoloration caused by escape of blood into the tissues
from ruptured blood vessels.
Echolalia: The automatic and meaningless repetition of another’s words or
phrases. In autism, this repetition may be either immediate or delayed, and
often does not appear appropriate to the conversation or activity.
Electroconvulsive therapy: The induction of a brief seizure by passing electric
current through the brain. This treatment is used mostly for depression.
Electroencephalogram: The record obtained during the amplification,
recording, and subsequent analysis of the electrical activity of the brain
using an instrument called an electroencephalograph.
Electromyography (EMG): A diagnostic tool used for testing the electrical
activity of muscles.
Embolism: Sudden blockage of an artery by a moving clot of foreign material
(embolus) lodged in place by the blood current. Emboli usually lodge at
divisions of an artery, where the blood vessel narrows, and are typically
blood clots. However, they may also be formed from globules of fat, air
bubbles, pieces of tissue, or clumps of bacteria.
Emotional lability: Inability to regulate emotions appropriately, typically
resulting in laughing or crying in unsuitable contexts.
Emphysema: The destruction of the walls of the bronchioles and alveoli,
which results in abnormal and enlarged air spaces.
End-stage renal disease: A condition of nonfunctioning kidney disease.
Energy conservation: The use of alternative methods of completing daily
activities in a manner that reduces unnecessary expenditure of physical
stress and effort.
Epidemiology: A scientific discipline devoted to studying the patterns of
communicable diseases.
Epiglottis: A thin flap of tissue in the esophagus that protects the lungs from
foreign objects by covering the trachea during swallowing.
Epstein-Barr virus (EBV): A virus causing infection and is characterized by
fatigue and general malaise. Infection with EBV is fairly common and is
usually a transient and minor thing. However, in some individuals EBV can
trigger chronic illness. It is a particular danger to people with compromised
immune systems.
Equality: The value that all individuals are perceived as having the same
fundamental human rights and opportunities. This value is demonstrated by
an attitude of fairness and impartiality.
Equinovalgus: Deformity in which the foot is plantar flexed, everted, and
abducted.
Equinovarus: Deformity in which the foot is planter flexed, inverted and
adducted.
Eschar: The residual necrotic layers of skin destroyed by direct heat damage or
the injury occurring secondary to heat damage.
Euphoria: A feeling or state of well-being or elation.
Euthymia: Normal range of mood.
Excoriation: Skin picking disorder.
Executive functioning: A broad band of skills that allow an individual to
engage in independent, self-directed behavior. Includes volition, planning,
purposeful action, and self-awareness. Executive functions involve the
ability to formulate context-appropriate goals and to initiate, plan and
organize, and sequence and adapt behavior based on anticipated or actual
consequences of actions.
Extrapyramidal symptoms: Involuntary movement, changes in muscle tone,
and abnormal posture.
Extrapyramidal syndrome: A side effect from medication that results in
abnormal movements similar to Parkinson’s disease.
Factitious disorders: Falsifications of an illness for oneself or others.
Familial: A disease for which there is a family history.
Fasting plasma glucose: Measurement of blood glucose following an 8-hour
fasting.
Fetal alcohol syndrome: A condition in which the infant exhibits CNS damage
and retardation due to intrauterine alcohol exposure.
Fibromyalgia: A condition that causes widespread pain and tender points
located in nine particular points in the body.
Fistula: is an abnormal connection or pathway between structures caused by
disease, injury, or surgery.
Fixed ideas: An individual holds an unchangeable idea.
Flaccidity: Abnormally low muscle tone, which is felt as too little resistance to
movement. Also known as hypotonus.
Flight of ideas: Accelerated speech with rapid changes in subject. Associations
are understandable unlike the lack of connections that are associated with
looseness of associations.
Fluid resuscitation: Medical practice of replenishing bodily fluid lost through
sweating, bleeding, fluid shifts, or other pathologic processes.
Food selectivity: Severe limitation in food tolerance; child may refuse to eat
more than 5 to 10 very specific foods.
Fowler’s position: An upright sitting position of 90 degrees to enable optimal
oxygenation, which is prescribed for respiratory dysfunction.
Fragile X syndrome: is a genetic cause of ID that results from a mutation at
what is known as the fragile site on the X chromosome.
Freedom: The value of allowing each individual the right to exercise choice
and to demonstrate independence, initiative, and self-direction.
Frontal lobes: Region of the brain located at the front of the cerebrum. This
region is responsible for social behavior, spontaneous production of
language, initiation of motor activity, processing sensory stimuli and then
planning reaction as a result of the input, abstract thinking, problem
solving, and judgment.
Full thickness burn: Burn injury that destroys the entire epidermal and dermal
layers of the skin and extends down into subcutaneous fat.
Gamma-aminobutyric acid (GABA): An amino acid that functions as a
neurotransmitter in the central nervous system decreasing nerve
transmission in the brain.
Gastroesophageal reflux: Caused by stomach acid coming up from the
stomach into the esophagus. A typical symptom is heartburn.
Gastrointestinal disorder: A disorder of the digestive system.
Gastrointestinal (GI) disorder: Disorder of the digestive tract including
reflux, gastritis, persistent gas, diarrhea, and constipation.
Gaze shift: Sharing attention with another person by alternating gaze between
an object and a person or following the gaze of.
Generalized anxiety disorder: Chronic, repeated episodes of anxiety or dread
accompanied by autonomic changes.
Genes: A region of DNA that carries the genetic instructions for a cell.
Genotype: Genetic makeup of a living organism.
Glare sensitivity: Visual problem in which the person has difficulty discerning
images in high light.
Globesity: The global public health epidemic of obesity.
Gluteal shelf: refers to the bulbous gluteal region that protrudes posteriorly,
impacting on supported seating position.
Gluten: A protein found in wheat products. A theory in the field of autism
suggests that children with autism do not digest this protein appropriately,
leading to a buildup of morphinelike substances in the body and causing
social withdrawal and abnormal behaviors.
Gout: A common inflammatory arthritis caused by crystallization of uric acid
within the affected joints.
Gower’s maneuver: The inability to rise off the floor without using the upper
extremities to “walk up” the thighs to assist with hip extension.
Gower’s sign: When a person is observed to use his or her upper extremities to
“walk up” the thighs to assist with hip extension, it is a positive Gower’s
sign.
Graft versus host disease (GVHD): A significant disadvantage in allogeneic
transplants and occurs when the newly formed immune system views the
recipient’s body as foreign and attacks systems and/or organs.
Grandiosity: An inflated sense of self-esteem or importance.
Graphesthesia: The ability by which outlines, numbers, words, or symbols
traced or written on the skin are recognized.
Greenstick fracture: Often seen in children whose bones are still soft and
growing. Rather than snapping into two, the bone breaks on one side and
bends on the other. It is similar to how a young twig or tree limb breaks,
thus the name greenstick fracture.
Guarding: Self-bracing is also a frequent response in those with RA and JRA
due to pain associated with movement.
Hair-pulling disorder: also known as trichotillomania is a disorder in which
individuals engage in repeatedly pulling out their hair, resulting in
significant hair loss or thinning of the hair.
Hallucinations: Disorders of perception. Atypical auditory, visual, and/or
olfactory sensory perceptions.
Health disparity: A situation in which a given population or group sharing a
constellation of characteristics manifests lower health status and poorer
health outcomes proportionate to the population as a whole.
Hematoma: A localized collection of extravasated blood, usually clotted, in an
organ, space, or tissue. Familiar, typically benign forms of hematoma
include contusions (bruises) and black eyes. Hematomas can occur almost
anywhere in the body and are especially serious when they occur inside the
skull, where they may produce pressure on the brain. The most common
types affecting the brain are epidural (above the dura mater, between it and
the skull) and subdural (beneath the dura mater, between the touch casing
and the more delicate membranes covering the tissue of the brain, the pia-
arachnoid).
Hemianopsia: Defective vision or blindness in one half of the visual field,
usually referring to bilateral defects resulting from a single lesion, often as
the result of a cerebrovascular accident (CVA). The individual is unable to
perceive objects to the side of the visual midline. Visual loss is
contralateral; that is, it is on the side opposite the brain lesion.
Hemiparesis: Paresis or weakness affecting one side of the body.
Hemiplegia: Paralysis of one side of the body, usually caused by a brain lesion,
such as a tumor, or a cerebrovascular accident. Paralysis occurs on the side
opposite the lesion, or infarct, because of decussation of most of the fibers
in the motor tracts of the brain. For example, damage to the right
hemisphere of the brain affects motor control of the left half of the body.
Hemoglobin A1c level: The average concentration of glucose in the blood over
a 6-week to 3-month time period, with levels ≥6.5% diagnostic of diabetes.
Hemorrhagic stroke: A type of stroke resulting from blood that has escaped
the normal vessels and entered brain tissue or the subarachnoid space.
Intracerebral hemorrhage is a type of hemorrhagic stroke that occurs when
blood leaking from a cerebral vessel directly enters the brain tissue.
Herd immunity: A phenomenon in which critical masses of people (or other
animal species) are rendered immune to a communicable disease and the
rampant spread of infection is curtailed.
Heterotopic ossification: The formation of bone in soft tissue and periarticular
locations. Early clinical signs include warmth, swelling, pain, and
decreased joint motion. Common joints for heterotopic ossification are the
shoulder, elbow, hip, and knee.
Hippocampus: A structure that is part of the limbic system and is involved in
long-term memory functions.
Hoarding disorder: is a condition that impedes the ability of an individual to
discard apparently unnecessary possessions, value notwithstanding, and
creates clutter that interferes with the safe use of the living space.
Homonymous hemianopsia: Loss of one half of the visual field, on the same
side, in both eyes.
Human immunodeficiency virus (HIV): A retrovirus that causes acquired
immunodeficiency syndrome (AIDS), a condition in humans in which
progressive failure of the immune system allows life-threatening
opportunistic infections and cancers to thrive.
Humeral fracture: A fracture that results in humeral displacement and
malposition of the distal limb.
Hydrocephalus: The abnormal accumulation of cerebrospinal fluid (CSF) in
the brain.
Hydrocephaly: Birth defect with a nongenetic origin that can contribute to or
cause ID due to an abnormal accumulation of cerebrospinal fluid in the
ventricles of the brain causing increased intracranial pressure and
progressive enlargement of the skull.
Hygiene hypothesis: Purports that improved hygiene practices are decreasing
childhood exposure to infectious agents and subsequent opportunities for
immune system development, leading to a rise in autoimmune diseases.
Hyperactive-impulsive: A clinical presentation of ADHD in children and
adults, characterized by signs of impulsivity and hyperactivity with less
intense signs of inattention.
Hyperflexia: Overflexion of a limb.
Hyperglycemia: An abnormally high concentration of glucose (115 to 139
mg/dL) in the circulating blood, especially with reference to a fasting level.
Hyperlexia: The ability to read words above age level expectation. Usually,
children with this ability have difficulty understanding the meaning of the
words they read.
Hyperorality: Excessive overeating.
Hyperphenylalaninemia: An environmental factor in the etiology of ID that is
the result of the maternal condition in which the presence of blood
phenylalanine levels exceed the limits of the upper reference range (2
mg/dL).
Hyperreflexia: See autonomic dysreflexia.
Hypersexuality: Disinhibition in regard to sexual impulses.
Hypertension: Persistent blood pressure measurements above the normal
systolic (140 mm Hg) or diastolic (90 mm Hg) pressures.
Hypertonicity (spasticity): Abnormal muscle tone felt as too much resistance
to movement as a result of hyperactive reflexes and loss of inhibiting
influences from higher brain centers.
Hypertrophic scar: A cutaneous condition characterized by excess scar tissue,
Hypertrophic scars occur when the body overproduces collagen, which
causes the scar to be raised above the surrounding skin; these scars take the
form of a red raised lump on the skin.
Hyperuricemia: An excess of uric acid in the blood.
Hypervigilance: Autonomic and endocrine hyperarousal which are observed
overreactions to stimuli. This may include being easily startled, craving
high-risk, stimulating, or dangerous activity; all of which impair the balance
of play, work, and rest.
Hypochondriasis: A preoccupation with fears of having or the idea that one
has serious disease based upon the person’s misinterpretation of bodily
symptoms.
Hypoglycemia: An abnormally small concentration of glucose in the
circulating blood, that is, 50 mg/dL in men and <45 mg/dL in women.
Hypomania: An episode of manic symptoms that are not severe enough to
meet the criteria for mania listed in DSM IV-TR.
Hypotension: Condition of blood pressure below the normal systolic and
diastolic measures.
Hypothyroidism: occurs when cells of the thyroid gland are damaged by
inflammation resulting from an associated autoimmune response or
secondary medical interventions.
Hypotonicity: Reduced tone; noted by minimal resistance to passive
movement.
Hypoxemia: Insufficient oxygenation of the blood.
Hypoxia: A pathological condition that results in insufficient oxygenation of
the blood.
Ideas of reference: Individuals believe that others are talking about or
referring to them.
Illness anxiety disorder: reclassified term for hypochondriasis under the
DSM-V.
Immune response: A person’s defense or resistance against infection.
Impaired control: Unsuccessful attempts to reduce use and also a persistent
desire to decrease or discontinue; one’s daily activities revolve around
obtaining the substance and/or recovering from its use.
In situ: Translates to “in position” or “in place.” This is an early stage of
cancer in which the cancerous cells remain in the original site from which
they arose.
Incentive spirometry: A machine that measures the amount (volume) of air
inhaled. This machine is used to validate chronic obstructive pulmonary
disease (COPD) as a diagnosis.
Incidence: A measure of the risk of developing a new condition within a
specified period of time. It is usually expressed as a proportion or a rate.
Infectious diseases: A group of conditions that are characterized by a set of
symptoms attributable to the introduction of a specific pathogen.
Inferior olive: Region of the brainstem that transmits error signals to the
cerebellum.
Inflammation: The initial response of the body to harmful stimuli and is
achieved by the increased movement of plasma and leukocytes from the
blood into the injured tissues. A cascade of biochemical events propagates
and matures the inflammatory response, involving the local vascular and
immune system.
Inoculum dose: The amount of pathogen necessary to produce an infection.
Insulin: A hormone produced by the pancreas and functions to regulate
metabolism.
Intoxication: Poisoning caused by ingestion of a toxic substance.
Intoxication disorders: changes in perception, wakefulness, attention,
thinking, judgment, psychomotor behavior, and interpersonal behavior.
Intraocular pressure (IOP): The fluid pressure of the aqueous humor inside
the eye.
Ischemia: Deficiency of blood resulting from the functional constriction or
actual obstruction of a blood vessel, often leading to the death (necrosis) of
the surrounding tissue.
Joint attention: The ability to use eye contact and gestures in order to share
experiences with others.
Joint protection principles: A protocol of methods used to change how one
engages in physical activity to decrease joint stress caused by excessive
force to the peripheral joints in particular.
Justice: The value of upholding of such moral and legal principles as fairness,
equity, truthfulness, and objectivity.
Juvenile rheumatoid arthritis: The most common rheumatic disease in
childhood. The diagnostic criteria for JRA are onset at age younger than 16
years, persistent arthritis in one or more joints for at least 6 weeks, and
exclusion of other types of childhood arthritis.
Kernicterus: A form of hemolytic jaundice of the newborn. The basal ganglia
and other areas of the brain and spinal cord are infiltrated with bilirubin, a
yellow-pigmented substance produced by the breakdown of hemoglobin.
Develops during the 2nd to 8th day of life.
Ketonuria: Condition in which ketone bodies are present. Ketones are waste
products from the body’s breakdown of fat for energy.
Kinesthesia: The ability and sense by which position, weight, and movement
are perceived.
Kyphosis: Abnormally increased convexity in the curvature of the thoracic
spine viewed from the side, resulting from an acquired disease, an injury, or
a congenital disorder or disease.
Lability: Rapidly shifting or changing emotions.
Latent tuberculosis: TB that exists in a dormant state.
Leptin: A hormone that is present in adipose tissue and signals receptors in the
brain of satiety, or when one is full from eating.
Leukemia: which means “white blood” in Greek, is a cancer that begins when
the blood-forming cells of the bone marrow and other blood-forming organs
create an excess of abnormal white blood cells (WBC).
Leukostasis: which is a clumping or sludging of the microcirculation that tends
toward clotting.
Lewy body pathology: A process in which proteins abnormally fold and form
intracellular inclusions within the cell body and neurons.
Limbic system: This group of subcortical structures is involved in various
emotions and memory functions.
Liquid tumor: are also known as blood cancers or hematological tumors.
Logopenic: Impairment in the ability to retrieve single words; impaired
repetition of phrases and sentences.
Long-term memory: The ability to recall information for long periods of time
without effort.
Lordosis: Forward curvature of the lumbar spine.
Lou Gehrig’s disease: Common name for Amyotrophic Lateral Sclerosis
(ALS) which is a fatal, progressive, degenerative motor neuron disease in
which scars form on the neurons in the corticospinal pathways, the motor
nuclei of the brainstem, and the anterior horn cells of the spinal cord.
Lower motor neuron: Injury to the ventral horn of the spinal cord results in
lower motor neuron damage; sign include focal and multifocal weakness,
atrophy, cramps, and muscle twitching as well as flaccid paralysis with loss
of movement, tone, and reflex activity.
Lumbar lordosis: An inward curvature of the lower vertebral column.
Lymphedema: A condition resulting from the disrupted flow of the lymph
system and is a lifelong side effect that cannot be cured but must be
managed.
Lymphoma: A cancer that develops in the glands or nodes of the lymphatic
system causing abnormal cellular reproduction of WBC called
lymphocytes, which are a vital part of the immune system.
Macula: The visual structure at the center of the retina. It is responsible for the
processing of the central vision.
Magnetic resonance imaging (MRI): A neuroimaging tool that uses radiology
to visualize detailed internal structures.
Magnetic resonance spectroscopy: A diagnostic tool that measures the levels
of different metabolites in body tissues.
Magnetic resonance volumetry: A diagnostic tool that measures the volume
of the internal structures of the body.
Major depressive episode: A period of depressed or irritable mood lasting at
least 2 weeks, resulting in severe impairments in functioning.
Malignant tumor: Composed of abnormal cells that multiply rapidly, with the
ability to invade, or metastasize into other tissues.
Malingering: Intentional production of false or grossly exaggerated physical or
psychological problems.
Malunion: Refers to nonunion of a fractured bone.
Mania: An extremely elevated mood.
Manic episode: A highly elevated or irritable mood lasting at least 1 week,
with or without psychotic symptoms such as delusions and hallucinations.
Melancholia: This term dates back to the 4th century and continues to be used
in DSM IV-TR. Refers to the following signs: anhedonia, early morning
awakening with depression worse in the morning, psychomotor agitation or
retardation, weight loss, and feelings of guilt.
Mental disorders: DSM IV-TR characterizes a mental disorder as “a clinically
significant behavioral or psychological syndrome or pattern that occurs in
an individual and that is associated with present distress or disability or
with significantly increased risk of suffering death, pain, disability, or an
important loss of freedom.”
Metabolic equivalent (MET): A MET is the amount of energy that an activity
requires, with more sedentary tasks such as watching TV or doing computer
work using 1.5 to 2 METS ranging up to some competitive sports requiring
11 or more METs.
Metabolic syndrome: refers to the presence of a combination of risk factors
including obesity, diabetes, hypertension, high cholesterol, elevated fasting
blood sugar, high triglycerides, systemic inflammation, and a tendency to
form blood clots.
Metamorphopsia: The visual distortion of objects.
Metastasis: is the spread of a disease from one organ or part to another
nonadjacent organ or part.
Metastatic disease: which means the cancer from one organ or part of the body
has spread to another organ or part of the body not directly connected.
Metastatic spinal cord compression (MSCC): occurs when cancer grows on
or near the spine, causing compression on the dural sac and the contents, in
particular the spinal cord.
Methylphenidate: A stimulant medication, commonly known as Ritalin, that
increases levels of dopamine and epinephrine in the brain to improve
attention, impulsivity, and overactivity.
Mild acquired TBI: Clinically identified as a loss of consciousness for <10
minutes or amnesia, a Glasgow Coma Scale (GCS) rating of 13 to 15, no
skull fracture on physical examination, and a nonfocal neurological
examination.
Mirels’ scoring: A tool used to guide the management of bones with cancer in
order to help prevent a pathological fracture.
Mixed episode: The presence of both manic and major depressive symptoms
almost daily for at least 1 week, resulting in rapid mood cycling with or
without psychotic symptoms.
Mixed-type cancer: Cancer that has different tissue types or components from
more than one type of cancer.
Moderate acquired TBI: Hospitalization of at least 48 hours, an initial GCS
rating of 9 to 12 or higher.
Modified constraint-induced therapy: A treatment for hemiplegia
characterized by restricted movement of the intact extremity to elicit
movement of the involved extremity.
Morbidity: A diseased state, disability or poor health due to any cause.
Mortality: The condition of being susceptible to death. A mortality rate is a
measure of the number of deaths in a given population.
MRSA: Methicillin-resistant Staphylococcus, a strain of staphylococcus that is
resistant to customary antibiotics.
Multimodal intervention: Individually tailored to meet the unique needs of
each client, with consideration of the client’s own goals and priorities in
concert with other interventions.
Munchausen’s syndrome: The exaggeration of an illness, typically upon a
child, to gain sympathy for the caregiver.
Muscle biopsy: A medical diagnostic tool used to examine the cellular makeup
of the muscle tissue, and look at the differences in muscle fibers, fiber size,
fiber splitting, and fiber necrosis.
Muscular dystrophy: A group of genetic conditions that results in progressive
weakening of the muscles. Most types of MD are multisystem disorders that
can affect the heart, gastrointestinal and nervous systems, endocrine glands,
skin, eyes, and brain.
Mutism: Inability to speak.
Myelin: The lipid substance forming a sheath around the axons of certain nerve
fibers, occurring predominantly in the cranial and spinal nerves that
compose the white matter of the brain and spinal cord. The myelin sheath is
formed by a glial cell, either an oligodendrocyte (in the central nervous
system) or Schwann cell (in the peripheral nervous system).
Myelogram: A graphic representation of the differential count of cells found in
a stained representation of bone marrow.
Myeloma: A malignant tumor that originates in the plasma cells of bone
marrow.
Myocardial infarction: commonly referred to as a heart attack, occurs when
the blood flow that brings oxygen to the heart muscle is severely reduced or
completely occluded causing the heart muscle tissue to be without oxygen
resulting in damage or death to that tissue.
Myocardial infarction (MI): caused by a lack of blood supply to the heart
muscle and tissue death occurs.
Myoclonus: Spasm of a muscle or group of muscles.
Myopia: A condition of the eye where the light that comes in does not directly
focus on the retina.
Negative symptoms: Decreases or deficits in typical behaviors, seen in mental
illness.
Neoplastic: cells remain in the original site from which they arose.
Neovascular glaucoma: A secondary disorder that results from other diseases,
such as diabetes mellitus or tumors, which cause new blood vessels to grow
and obstruct the outflow of aqueous humor.
Nephropathy: Any disease of the kidney.
Neural plasticity: The brain’s ability to create new neural pathways through
the life span. Allows for brain cells to adapt to injury and disease in
reaction to novel situations or typical environmental demands.
Neurobiologic: Of or relating to the biologic study of the nervous system. The
cause of autism is considered to be an abnormality in the structure or
function of the brain.
Neurodegenerative: Refers to degeneration of nervous tissue.
Neurofibrillary tangles: Contaminated tau protein that becomes twisted and
causes eventual cell death.
Neurogenic bowel/bladder: Dysfunction resulting from congenital
abnormality, injury, or disease process of the brain, spinal cord, or local
nerve supply to the urinary bladder or rectum and their respective outlets.
The dysfunction may manifest as partial or complete retention,
incontinence, or frequency of elimination.
Neuroleptic: A medication having antipsychotic action, affecting sensorimotor,
cognitive, and psychological functions.
Neuromuscular disorders: Diseases that affect the nerves that control
voluntary muscles.
Neuron: A nerve cell that is specialized in transmitting and receiving electrical
signals to communicate information between different regions of the body.
Neuronal pruning: A process that eliminates the overproduction of sensory
neurons that are generated in infancy through middle childhood.
Neuropathy: Pathology of the nervous system.
Neurotransmitters: Chemical transmitters, such as dopamine and glutamate,
secreted by neurons that bind receptors on nearby cells.
Neutropenia: which is defined as an absolute neutrophil count lower than
500/mm3.
Nocturia: The need to wake and urinate during the night.
Nonreflex neurogenic bladder or bowel: Also called autonomic
bladder/bowel. A neurogenic bladder/bowel resulting from a lesion or
injury in the sacral portion of the spinal cord that interrupts the reflex arc
that controls the bladder/bowel. The lesion may be in the cauda equina,
conus medullaris, sacral roots, or pelvic nerve. It is marked by loss of
normal bladder/bowel sensations and reflex activity, inability to initiate
urination/elimination normally, and stress incontinence.
Nontremor dominant PD: One of two types of Parkinson’s disease in which
there is akinetic-rigid syndrome and postural instability gait.
Nonunion: A fracture in which the bone is not healing. A nonunion fracture
may be caused by several factors such as vascular and tissue damage, poor
alignment, stress to the fracture site, and infection.
Nosology: A term used to classify medical conditions.
Nystagmus: Involuntary, rapid, rhythmic movement (horizontal, vertical,
rotatory, or mixed, i.e., two types) of the eyeball.
Obesity: Abnormal level of excess body weight.
Obesity hypoventilation syndrome: A condition that results from excess
weight compressing on the chest, preventing breathing, and increasing the
amount of carbon dioxide in the blood; lack of oxygen contributes to poor
sleep quality and hypoxia.
Obsession: “A recurrent and persistent idea, thought, or impulse to carry out an
act that is ego-dystonic, that is experienced as senseless or repugnant, and
that the individual cannot voluntarily suppress.”
Obsessive-compulsive disorder: “A type of anxiety disorder whose essential
feature is recurrent thoughts, impulses or images or compulsions (repetitive,
purposeful, and intentional behaviors performed in response to an
obsession) sufficiently severe to cause marked distress, be time consuming
or interfere significantly with the individual’s normal routine, occupational
functioning, or usual social activities or relationships with others.”
Occupations: A broad category of human activities that are typically part of
daily life. The areas include basic activities of daily living, instrumental
activities of daily living, education, work, play and leisure, and social
participation.
Opacifications: Cloudy or opaque spots in the lens.
Open fracture: A fracture in which the bone breaks through the skin surface;
also referred to as a compound fracture.
Open reduction internal fixation (ORIF): A surgical procedure which
involves the opening of and reducing the fracture site. Internal fixation is
commonly done after an open reduction to secure the fracture. Internal
fixation involves securing the fracture site with pins, rods, plates, and
screws.
Open-angle glaucoma: A low vision disorder in which retinal damage occurs
due to increased intraocular pressure.
Optic neuritis: Inflammation of the optic nerve, affecting the part of the nerve
within the eyeball (neuropapillitis) or the part behind the eyeball
(retrobulbar neuritis), usually causing pain and partial blindness in one eye.
Orthopneic position: Position of support for a person with orthopnea, for
example, propping up with pillows or soft wedges.
Orthostatic intolerance: An inability to tolerate sitting upright or standing,
related to a fall in blood pressure that causes dizziness, syncope, and
blurred vision.
Osteoarthritis: more commonly referred to as Degenerative Joint Disease as
inflammation is not the hallmark feature of this condition as it is with
rheumatoid arthritis; it is a mechanical “wear and tear” of weight-bearing
joints related to aging as well as a factor among those with obesity.
Osteoblastic lesion: occurs when cancer increases the activity of osteoblasts.
Osteolytic lesion: occurs when greater numbers of osteoclasts are lysing the
bone.
Osteopenia: Low bone mass; reversible weakening of the bone that may be
diagnosed through a bone density scan just as osteoporosis.
Osteoporosis: Disease characterized by low bone density and deterioration of
bone. It is common in postmenopausal women due to the cessation of
estrogen production.
Oxygen transport: The delivery of fully oxygenated blood to peripheral
tissues, the cellular uptake of oxygen, the utilization of oxygen from the
blood, and the return of partially desaturated blood to the lungs.
Panic: “Extreme and unreasoning anxiety and fear, often accompanied by
disturbed breathing, increased heart activity, vasomotor changes, sweating
and a feeling of dread.”
Panic attack: “Sudden onset of intense apprehension, fear, terror or impending
doom accompanied by increased autonomic nervous system activity and by
various constitutional disturbances, depersonalization, and derealization.”
Panic disorder: Short, sudden attacks of fear, fear of losing control, and terror.
Pannus: Activation of the synovial cells which accumulate and create pannus,
a malignant mass over the cartilage, leading to cartilage breakdown. This
granulation tissue continues to spread, the joint space is slowly effaced by
fibrous adhesions, and eventually fibrous ankylosis appears.
Paranoia: Persecutory delusions.
Paraphasia: A language disorder in which a person hears and comprehends
words but is unable to speak correctly.
Paratonia: Involuntary resistance to passive movement of the extremities.
Parentification: A common occurrence among a group of siblings in an
abusive and neglectful environment for one child, usually the oldest, to take
on the role of a parent, as there is an absence of an adult care provider
willing or able to provide care for others.
Parkinsonism: Characterized by tremor, muscle rigidity, slow shuffling gait,
and other classic symptoms of Parkinson’s disease.
Partial-thickness burn: Burn injury which involves part or all of the
epidermis. Referred to as second-degree burns.
Pathogen: A microorganism that compromises the health of an individual.
Most pathogens fall into three general categories: bacteria, viruses, and
fungi.
Pathologic fracture: A fracture caused by a disease such as bone cancer and
osteoporosis.
Pear abducted: Excessive weight located on the hips and upper thighs leading
to excess gluteal tissue.
Performance in areas of occupation: A broad category of human activity that
are typically part of daily life. The areas include activities of daily living,
instrumental activities of daily living, education, work, and play, leisure,
and social participation.
Performance patterns: The habits, routines, and roles that a person adopts.
Performance skills: The features of what a person does during an activity.
These skills are separated into the categories of motor skills, process skills,
and communication/interaction skills.
Peripheral artery disease: Leads to impaired wound healing, tissue hypoxia,
and decreased mobilization of white blood cells to infected tissues; occurs
at an earlier age and at a rate of two to four times higher in people with
diabetes.
Peripheral neuropathy: Most common symptoms include burning pain;
stabbing, pricking, or tingling sensation; pathologic skin sensitivity; or deep
aching pain; commonly diagnosed among people with diabetes.
Peripheral vision: Perception of images from the edges of the visual fields.
Peristalsis: The worm-like movement by which the alimentary canal or other
tubular organs with both longitudinal and circular muscle fibers propel their
contents, consisting of a wave of contraction passing along the tube.
Perseverative: Pertaining to the involuntary and pathologic repetition of verbal
or motor response.
Personal episodic memory: Recall of timerelated information about one’s self,
such as where and if one ate breakfast.
Personalized medicine: A comprehensive process to determine the best health
care options for a particular patient, deriving from a partnership between
patient and clinician.
Person-first language: The identification of a person as the first descriptor and
the disease as a secondary descriptor.
Pharmacological criteria: In terms of addiction, related to tolerance and
withdrawal.
Phenotype: Genetic markers that show in observable characteristics in
organisms.
Philosophic assumptions: Basic beliefs about focusing on the rights and
preferences of individuals, relative to their biological and social
environments.
Phobia: “Any objectively unfounded morbid dread or fear that arouses a state
of panic.”
Physiatrist: A physician specializing in rehabilitation medicine.
Pica: A medical disorder characterized by an appetite for nonfood substances.
Pica is a Latin word for “magpie,” a bird that has unusual eating habits.
Plaques: Scar tissue formation.
Pneumonia: An inflammation of lung tissue, where the alveoli in the affected
areas fill with fluid. The condition is caused by bacteria, viruses, aspiration,
or immobility.
Polydipsia: Excessive thirst that occurs frequently.
Polyphagia: Excessive eating.
Polyuria: Excessive excretion of urine.
Polyvictimization: Exposure to multiple types of trauma, the child may
demonstrate signs of comorbid psychiatric disorders.
Positive symptoms: Observable behaviors of psychosis such as hallucinations
and delusions.
Positron emission tomography: is a nuclear medicine imaging technique that
produces a three-dimensional image of the functional processes of the body.
Posterior adipose: Pannus distributed on the posterior trunk.
Posttraumatic stress disorder: Development of characteristic symptoms
following a psychologically traumatic event that is generally outside the
range of usual human experience; symptoms include numbed
responsiveness to environmental stimuli, a variety of autonomic and
cognitive dysfunctions, and dysphoria.
Practice framework: Outlines the language and constructs that describe the
occupational therapy profession’s domain of concern.
Preclinical period: Earliest of three phases of Parkinson’s disease when
neurons have begun to degenerate but not signs and symptoms are yet
evident.
Prediabetes: A condition in which people are in an intermediate zone between
normal blood glucose levels and meeting diagnostic criteria for diabetes.
Premorbid functioning: The period of time before the onset of symptoms.
Prevalence: The total number of cases of the risk factor in the population
divided by the number of individuals in the population. It is used to
estimate how common a condition is within a population over a certain
period of time.
Primary progressive: related to multiple sclerosis, in which there is a steady
decline in function without remissions and exacerbations.
Primitive reflexes: Innate primary reactions found in newborns and indicative
of severe brain damage if present beyond their usual time of disappearance.
Adult patients with closed head injury or stroke may manifest these signs;
absence on reevaluation is a sign of progress in recovery. Examples include
placing reactions, Moro reflex, grasp reflex, rooting reflex, and sucking
reflex.
Procedural learning: The ability to learn new motor, perceptual, or basic
cognitive behaviors.
Procedural memory: Recall of information on how to perform a task such as
knowing how to write or ride a bike.
Prodromal phase: The period of time when symptoms begin to emerge, which
can range from weeks or months to years before the full onset of symptoms.
Progressive: In terms of course of a disease or illness, refers to worsening,
growth, or spread of the disease
Prudence: The ability to govern and discipline oneself through the use of
reason. To be prudent is to value judiciousness, discretion, vigilance,
moderation, care, and circumspection in the management of one’s affairs, to
temper extremes, make judgments, and respond on the basis of intelligent
reflection and rational thought.
Psychomotor agitation: Repetitive and nonproductive motor activity. May
present as pacing or fidgeting.
Psychomotor retardation: Movements, reactions, and speech that are slowed.
Psychosis: The inability to distinguish fantasy from reality; delusion or
hallucinations that may be mood-congruent or mood- incongruent. Presence
of psychotic features in major depressive disorders (MDDs) indicates a
poor prognosis.
Pulse oximetry: Measures the oxygen concentration in arterial blood. The
normal range is 95% to 100%. Measurements are taken to prevent hypoxia
and to evaluate treatment.
Purkinje cells: Cells that form a layer near the surface of the cerebellum and
convey signals away from the cerebellum.
Quadriplegia: Paralysis of all four limbs (also referred to as tetraplegia).
Rales: Crackling sounds or rumbling sound caused by increased mucus which
the physician diagnoses by way of listening to the chest during breathing is.
Rapid-cycling: Four or more episodes of some type of mood disturbance
within the past year episodes meet criteria for mania, hypomania, mixed, or
depressed episodes as identified in DSM IV-TR.
Recent memory: The cognitive ability to temporarily store and manage
information that is required to carry out cognitive tasks such as learning and
reasoning. It is also referred to as short-term or working memory.
Reflex arc: A reflex, which is built in and does not need conscious thought to
take effect, is the total of any particular automatic response mediated by the
nervous system. A reflex arc is usually a simple reflex such as a knee jerk,
which involves only two nerves and one synapse. Other arcs may involve
an interneuron. When the sensory nerve ending is stimulated, a nerve
impulse travels along a sensory (afferent) neuron to the spinal cord. An
association neuron or interneuron then transfers the impulse to a motor
(efferent) neuron, which carries the impulse to a muscle, which then
contracts and moves a body part.
Relapse: Return of symptoms, resulting in rehospitalization for mental illness.
Relapsing-remitting-nonprogressive: related to multiple sclerosis, one of
several patterns in the course of the disease in which the person returns to
the -previous level of function after each exacerbation with no residual
effects.
Relapsing-remitting-progressive: related to multiple sclerosis, one of several
patterns in the course of the disease, which is marked by some residual
impairment with each remission and is unpredictable, with varied patterns
of exacerbation and remission.
Remodeling: The final stage of bone healing in which the bone is ideally
reshaped to its original form to enable it to resume its intended function as
best as possible.
Remote memory: The cognitive ability to recall information from the past. It
is also referred to as long-term memory.
Resiliency: The ability of an individual to recover from adverse or traumatic
events in a manner that is adaptive and nonpathologic.
Restrictive and repetitive behavior: In autism, an abnormal or intense
preoccupation with routines or patterns.
Retina: A structure of the central nervous system that consists of a
multilayered, light-sensitive tissue that lines the rear inner surface of the
eye.
Retinopathy: Noninflammatory degenerative disease of the retina.
Retrograde amnesia: Loss of memory of events that occurred prior to the
injury.
Rett’s disorder: A rare neurobiologic disorder that primarily affects females
and is caused by a deviation on the methyl-CpG-binding protein 2
(MECP2) gene. This disorder has a distinctive course since early
development in children is typical, followed by the deceleration of head
growth, loss of purposeful hand movements, and appearance of autistic-like
symptoms.
Rheumatoid arthritis: Arthritis refers to joint inflammation; “rheum” in
rheumatoid refers to the stiffness, general aching, weakness, and fatigue
that is experienced throughout the body.
Rheumatoid factor (RF): An antibody that is measurable in the blood.
Antibodies are normal proteins in our blood that are important parts of our
immune system; however, rheumatoid factor is an antibody that is not
usually present in the normal individual. The prognosis for those with an
RF positive factor is that they are at higher risk for erosions, nodules,
growth retardation, lack of adequate bone mineralization, anemia, and poor
functional status.
Rheumatologist: A physician who specializes in treatment of rheumatic
diseases.
Risk factors: A variable associated with an increased risk of disease or
infection.
Risky use: Consuming the substances even when it is physically hazardous and
despite knowing that the substance use is causing persistent physical, legal,
and/or psychological difficulties.
Rote memory: The process of memorizing and using language overheard from
others rather than producing spontaneously generated language.
Rumination: The nonpurposeful regurgitation of recently ingested food from
the stomach to the mouth, where it is either rechewed, reswallowed, or
expelled.
Sarcomas: are cancerous tumors that arise in supportive and connective tissues
including bones, tendons, cartilage, muscle, and fat.
Sarcopenia: For elders, low leptin levels, increased fat tissue, and loss of
muscle mass.
Satiety: Feeling of fullness.
Scoliosis: Lateral curvature of the vertebral column. This deviation of the
normally straight vertical line of the spine may or may not include rotation
or deformity of the vertebrae.
Self and relational dysfunction: One of the most significant signs of the
developmental impact of trauma; is a disruption of attachment. The type of
relationship between a child and a caregiver influences how a child
responds to present and future emotional and physical experiences.
Semantic memory: Ability to remember the name of an object.
Sensory craving: The seeking of sensory input in a manner that results in
erratic, disorganized behavior.
Sensory deprivation: Significant lack of external sensory stimulation.
Sensory discrimination: The ability to distinguish the qualitative aspects of an
external sensory stimulus.
Sensory modulation: also referred to as sensory regulation, refers to the
autonomic nervous systems’ ability to maintain “system harmony” through
a complex process of regulating and organizing the intensity of sensory
input to facilitate a functional response.
Sensory overresponsivity: responses to stimuli that are more intense or have a
longer duration than typically expected and may occur in only one or
multiple sensory mechanisms.
Sensory processing disorder: Difficulty with efficient and accurate detection,
registration, interpretation, organization, and adaptation to external and
internal sensory stimuli.
Sensory under responsivity: responses to stimuli that are significantly less
than expected resulting in longer, less efficient adaptation.
Severe acquired brain injury: Loss of consciousness and/or posttraumatic
amnesia for more than 24 hours, a GCS rating of 1 to 8.
Short-term memory: The ability to recall information after a short period of
time.
Sjogren’s syndrome: is a chronic disease of unknown etiology causing corneal
and conjunctival lesions and is characterized by dry eyes and mouth.
Skin picking disorder: also known as excoriation, is the presence of recurrent
skin picking resulting in noticeable skin lesions.
Sleep apnea: Chronic, intermittent interruption of respiration during sleep
leading to significant fatigue throughout waking hours.
Smith’s fracture: Opposite of Colles’ fracture in that the displacement from
the break is positioned toward the volar or palmar aspect of the wrist rather
than the dorsal aspect.
Social impairment: Impaired relationships and roles with family, friends,
coworkers, peers, and community.
Social phobia: “A persistent pattern of significant fear of a social or
performance situation, manifested by anxiety or panic on exposure to the
situation or in anticipation of it, which the person realizes is unreasonable
or excessive and interferes significantly with the person’s functioning.”
Solid tumors: are growths within a solid structure such as bone, muscle, or
organ and include carcinomas and sarcomas.
Somatic symptom disorder: Disorder in which the physical symptoms cannot
be fully explained by a general medical condition, another mental disorder,
or the effects of a substance.
Somatization: occurs when mental and emotional stresses become physical
complaints in the absence of an explained diagnosis.
Somatosensation: The combination of tactile, proprioceptive, and vestibular
sensory processing that provides a foundational body scheme.
Spasticity (hypertonicity): Abnormally high muscle tone, i.e., tone which is
felt as too much resistance to movement, resulting from hyperactive
reflexes or the loss of inhibiting influences from higher brain centers.
Sphygmomanometer: The instrument used to measure blood pressure.
Spina bifida: A congenital neural tube dysfunction resulting in an incomplete
closing of the vertebral column and spinal cord agenesis which may
demonstrate the same clinical conditions as traumatic spinal cord injury.
Spinal muscular atrophy (SMA): A neuromuscular disease characterized by
degeneration of motor neurons resulting in progressive muscular atrophy
and weakness.
Spinal shock: Result of an acute transverse lesion of the spinal cord that causes
immediate flaccid paralysis and loss of all sensation and reflex activity
(including autonomic functions) below the level of injury. On return of
reflex activity, there is increased spasticity of muscles and exaggerated
tendon reflexes.
Sporadic: A term used in the etiology of neurodegenerative disorders to
indicate nongenetic link. In ALS, 90% of cases are believed to be of a
sporadic type.
Stereotyped behavior: A pattern of behavior, such as hand flapping or
nonproductive exploration of objects, that is often seen in autism.
Strabismus: Deviation of the eye in which the visual axes assume a position
relative to each other different from that required by the physiological
conditions; also called squint.
Stretch reflex: Reflex contraction of a muscle in response to passive
longitudinal stretching.
Subdural hematoma: An accumulation of blood in the subdural space of the
brain, usually caused by an injury.
Substance abuse: occurs when the use of the substance leads to failure in
carrying out a variety of occupational roles, interpersonal and legal
problems, and risky behaviors.
Substance use disorder: refers to irreversible brain circuit changes in severe
disorders and repeated behavioral relapses and strong cravings in the
presence of substance-related stimuli.
Substance-related disorders: Widespread phenomena that affect all sectors of
a population and OT specialty areas on a national and global basis.
Substantia nigra: Located in the basil ganglia, this structure produces
dopamine, a neurotransmitter, and transports it to the striatum.
Sundowning: A condition in which a person with cognitive impairment
becomes confused or disoriented at the end of the day.
Superficial burn: refers to the depth of the burn; also referred to as a first-
degree burn, occurs when the top layer of skin, called the epidermis, is
burned.
Superior vena cava syndrome (SVCS): is an oncologic emergency resulting
in diminished blood return to the heart, causing symptoms of facial edema,
edema of the neck and upper extremities, dyspnea, cough, and shortness of
breath or orthopnea and is most commonly caused by lung cancer.
Survivorship: A common term referring to individuals who have been
diagnosed with cancer, begins at the time of diagnosis and continues
through the person’s end of life.
Swan-neck deformity: often seen in rheumatoid arthritis; inflammation results
in pain and edema of the PIP joint, leading to hyperextension with the DIP
in a fixed flexed position resulting in a swan neck position of the finger.
Symptomatic period: As related to Parkinson’s disease, the third phase of the
disease in which the classic motor signs are evident followed by
progressive nonmotor signs.
Synovial: Pertaining to, consisting of, or secreting synovia, the lubricating
fluid of the joints, bursae, and tendon sheaths.
Systemic lupus erythematosus: An autoimmune disease with unique facial
“butterfly” rash, joint inflammation, and fatigue.
Systolic blood pressure: The amount of force used to pump blood out of the
heart into the arterial circulation.
Tachycardia: Abnormal rapid heart rate which may indicate inefficient heart
function, reducing -circulation integrity and diminishing oxygenation of
other body structures.
Tactile defensiveness: A type of sensory defensiveness in which a person
overreacts or avoids touching certain textures.
Tangential thinking: Rapid shifting from one thought to a closely related
thought.
Tangentiality: Pattern of communicating in which the person moves off the
central point and, unlike circumstantiality, is unable to return to the point.
Tardive dyskinesia: A side effect from medication that results in motor
abnormalities such as writhing movements.
Tau: A type of protein present in the central nervous system; deterioration of
tau proteins associated with Alzheimers disease.
Tay-Sachs disease: An autosomal, recessive, single gene disorder that can
cause ID.
Temporal lobes: This region of the brain is located at the side of the cerebrum,
below the frontal lobe. Its functions include auditory processing,
comprehension, naming, verbal memory, and high-level visual processing
including object and facial recognition.
Tenosynovitis: Inflammation of the synovial lining of the tendon sheath.
Teratogenic: Environmental factors that cause malformations of an embryo or
fetus.
Theory of mind: The ability to understand another person’s thoughts, feelings,
or intentions.
Thrombus: A blood clot comprising such blood factors as platelets and fibrin;
frequently the cause of obstructions in the vascular system resulting in
medical problems such as stroke.
Tissue plasminogen activator (tPA): Medications used for dissolution of an
occluding thrombus.
TNM classification of malignant tumors: is the most widely used cancer
staging system.
Tolerance: is defined as requiring increased amounts of the substance in order
to get the desired effect or a significantly reduced effect when usual dosage
is consumed.
Topagnosia: Loss of ability to localize site of tactile sensations.
Topographic orientation: Orientation of the self within the environment.
Toxemia: An environmental factor in the etiology of ID that is the result of the
maternal condition in which high blood pressure and protein in the urine
develop after the 20th week of pregnancy (also known as preeclampsia).
Trabecular meshwork: An area of tissue in the eye at the base of the cornea
that is responsible for draining the aqueous humor from the eye via the
anterior chamber.
Transgenerational: Caregiver’s actions are based on his or her own previous
parenting experiences.
Transient ischemic attacks (TIA): A sudden episode of temporary symptoms,
typically due to diminished blood flow through the carotid arteries or
sometimes related to impaired circulation through the vertebrobasilar
vessels. TIA is a powerful -warning sign of stroke, and symptoms can range
from obvious loss of sensation or motor function to more subtle signs. See
Cerebrovascular accident.
Trauma: From the Greek word meaning wound, has been traditionally used in
medicine to indicate a serious physical injury but it is more widely used to
refer to emotional shock following a stressful event or to chronic
experience that is deeply distressing.
Tremens: See delirium tremens.
Tremor-dominant PD: One of two proposed subtypes of Parkinson’s disease
(PD) which present with a relative absence of motor impairment other than
classic tremors of PD.
Trichotillomania: Self-induced removal of hair from one’s body.
Truth: Faithful to facts and reality. Truthfulness or veracity is demonstrated by
being accountable, honest, forthright, accurate, and authentic in our
attitudes and actions.
Tuberculosis (TB): A serious and highly contagious, airborne infection usually
affecting the lungs. It is caused by mycobacterium tuberculosis.
Tumor lysis syndrome: refers to a group of metabolic disorders that are
potentially life threatening and includes the rapid development of
hyperuricemia (high blood uric acid), hypocalcemia (low blood calcium),
and hyperphosphatemia (high blood phosphorus); symptoms include severe
muscle weakness or paralysis, sudden mental incapacity, extrapyramidal
movement disorders, and myopathy.
Type 1 diabetes: A disease in which the pancreas stops producing insulin as a
result of autoimmune destruction of cells.
Type 2 diabetes: A disease where the body cells are insulin resistant.
Unilateral spatial neglect: Inattention to visual stimuli presented on the
individual’s side contralateral to a cerebral lesion. It may occur
independently of visual deficits or with hemianopsia.
Unipolar: Refers to the syndrome that consists only of major depressive
episodes without manic episodes.
Utilization behavior: Touching, grasping, or manipulating anything that is
within sight.
Valley Sign: A diagnostic observation in which two bulges are visible on either
side of a depression on the back of the shoulder when a person is asked to
abduct the shoulders to 90 degrees with 90-degree flexion of the elbows so
that hands are pointing upward.
Vegetative signs: Physiologic signs that develop in the areas of sexual activity,
sleep, appetite, and other biologic rhythms.
Visuospatial: Pertaining to the ability to comprehend visual representations
and their spatial relationships.
Vitamin D deficiency: defined as a 25-hydroxy vitamin D level of <20 ng/mL;
30 is considered sufficient, and toxicity may occur at 150 ng/mL.
Volkmann’s deformity: An orthopedic deformity that results from severe
damage to tissues and muscles caused by increased pressure in the forearm
compartments.
Waist circumference: A measure used to describe the degree of obesity and
predict the level of disease risk.
Winged scapulae: A condition in which the shoulder blade protrudes from a
person’s back in an abnormal position.
Withdrawal: defined as a syndrome that occurs when blood or tissue
concentrations decline in a person who has used heavily, which makes him
or her more likely to consume again to relieve symptoms.
Withdrawal disorders: describes the symptoms that occur immediately and
for a longer period after a client who has been heavily using a substance
stops ingesting.
X-linked: A disease that occurs when a mother who carries the affected gene
passes it onto her son. Although the mother carries the affected gene on one
of her X chromosomes, she may never show symptoms since it is a
recessive trait.
Zoonotic infection: Infection that is transmitted from animals to humans.
Index
A
AAMR. See American Association on Mental Retardation ACE (Angiotensin-
converting enzyme) inhibitors Acetylcholine
Alzheimer’s disease
mood disorder
Acetylcholinesterase inhibitors Acquired brain injury
traumatic See (Traumatic brain injury) tumor of central nervous system
definition
diagnosis, course and prognosis etiology
impact of
incidence and prevalence
medical/surgical management
signs and symptoms
Activities of daily living (ADL) in late Alzheimer disease
muscular dystrophy
traumatic brain injury
Activity demands
Acute lymphocytic leukemia (ALL) Acute myelogenous leukemia (AML)
Adaptive behavior
Adverse Childhood Experiences (ACE) Affect
Affective disorder
intellectual disability
schizophrenia
AFO (Ankle-foot orthosis)
Age-related macular degeneration (AMD) case study
course and prognosis
definition
diagnosis
etiology
signs and symptoms
types
Aging
cancer
cataracts
cerebral thrombosis
osteoarthritis
stroke risk and
type 2 diabetes
Agnosia
cerebrovascular accident
stroke
Agoraphobia
Ailurophobia. See also Phobia Alcohol
DSM-5
signs and symptoms
Alcohol abuse, stroke risk and Alexithymia
Algorithms, for mental illness treatment Allogeneic bone marrow transplant
Allograft
Alogia
Alprazolam
Altruism
Alveoli
mild stage
moderate stage
severe stage
definition
etiology of
cardiovascular risk factors
free radicals
genetic predisposition of
neurotransmitter abnormalities incidence and prevalence
neuropathology
signs and symptoms
Ambiguous hand preference
American Association on Intellectual and Developmental Disabilities (AAIDD)
American Association on Mental Retardation (AAMR) American Burn
Association, criteria for burn center referral American Cancer Society
American College of Rheumatology (ACR) American Joint Committee on
Cancer (AJCC) American Medical Association (AMA) American
Occupational Therapy Association (AOTA) American Psychiatric
Association (APA) Amnesia
anterograde
retrograde
Amphetamines
Amygdala
Amyloid precursor protein (APP) Amyotrophic lateral sclerosis (ALS) case
study
definitions
diagnosis
etiology
motor neuron change
signs and symptoms
Analgesics, for wound debridement Aneurysm
genetic factor
hemorrhagic stroke
subarachnoid hemorrhage and
Angiography, cerebral
Angioplasty
Angiotensin-converting enzyme (ACE) inhibitors Angle-closure glaucoma
Anhedonia
in major depressive disease
schizophrenia
Animal phobias. See also Phobia Ankle–foot orthosis (AFO)
Anomia
Anorexia nervosa
definition
diagnosis
etiology
signs and symptoms
Anosmia, in traumatic brain injury Anterior cerebral artery stroke. See also
Stroke Anterior cord syndrome, . See also Spinal cord injury Anterograde
amnesia
Antibiotics, for autism
Anticoagulants
intracerebral hemorrhage
for stroke
Anticonvulsants, muscular dystrophy Antidepressants
anxiety disorder
brain tumors
glaucoma
major depressive disorders
side effects
Antifungals, for autism
Antimicrobial agent, for burn wound Antiplatelet therapy, stroke Antipsychotic
medication
cataracts
schizophrenia
Anxiety disorder
case study
classification
etiology
impact on occupational performance incidence and prevalence
signs and symptoms
Anxiolytics
Aphasia
in transient ischemic attacks APP (Amyloid precursor protein (APP) Apple
ascites
Apple pannus
Apraxia
in stroke
Aripiprazole
Arrhythmias
Arteriovenous malformations (AVMs) Arthritis. See also Rheumatoid arthritis
Arthroplasty
arthritis
hip
knee
Asbestos
Asperger’s disorder. See also Autism spectrum disorders course and prognosis
diagnosis
signs and symptoms
Aspiration, traumatic brain injury Aspirin, in stroke
Associated movements
Associated reactions
Ataxia
in cerebral palsy
in lacunar stroke
motor deficits
Atheromas
Atherosclerosis
cerebral thrombosis
diabetes
stroke and
subclavian steal syndrome
Athetoid (dyskinetic)
ATP (adenosine triphosphate) Atrial fibrillation (AFib)
Attention, in schizophrenia
Attention deficit hyperactivity disorder (ADHD) behavioral interventions
case study
clinical presentations
cognitive-behavioral therapies combined
co-occurring/comorbid conditions course and prognosis
definition
diagnose
etiology
hyperactive-impulsive symptoms impact on occupational performance
inattentive symptoms
medical and pharmacological
parent training programs
predominately hyperactive-impulsive predominately inattentive
prevalence of
school-related interventions specified or unspecified
Auditory hallucinations, schizophrenia Auditory processing
Australian Scale for Asperger’s Syndrome Autism Screening Questionnaire
(ASQ) Autism spectrum disorders
case studies
characteristics
definition
etiology
brain structure/function abnormalities combination of factors
environmental factors
genetics
physiologic abnormalities
impact on occupational performance brain structure
digestive system
global impairment
mental functions
sensory functions and pain
urinary and reproductive functions incidence and prevalence
signs and symptoms
co-occurring conditions
core symptoms
fine and gross motor impairment food selectivity
sensory processing disorder
sleep disturbance
surgical/medical management
complementary and alternative medicine diagnosis
medical conditions
pharmacologic therapies
types
Autistic disorder
Autograft
Autologous bone marrow transplant Autonomic dysreflexia (hyperreflexia)
Autonomic neuropathy
Autosomal dominant single gene disorders Autosomal recessive single gene
disorder Avoidant/restrictive food intake disorder definition
diagnosis
etiology
signs and symptoms
Avolition
B
Baclofen
Bariatric surgery
Bariatrics
Basic activities of daily living (BADLs) schizophrenia
SPD
Baxter formula
Baxter (Parkland) formula
Becker muscular dystrophy (BMD) Behavior disturbances, AD
Behavior rating scales
Behavioral theory, in anxiety disorders Benign tumors
Benzene, blood cancers
Benzodiazepine
Beta-amyloid
Beta-blockers, cardiopulmonary disorders Binge eating disorder
definition
diagnosis
etiology
signs and symptoms
Bipolar disorder (BPD)
case study
diagnosis
etiology
management
electroconvulsive therapy
pharmacology for
rapid-cycling
signs and symptoms
Blasts
Blindness, in diabetics mellitus Blood glucose level, self-monitoring Blood-
injection-injury phobia. See also Phobia Body dysmorphic disorder (BDD)
definitions
diagnosis
etiology of
signs and symptoms
Bone marrow transplant (BMT) Botulinum toxin
in amyotrophic lateral sclerosis traumatic brain injury
Boutonniere deformity
Braden Scale
Bradycardia
Bradykinesia
Brain attack. See also Stroke Brain pacemaker
Brain structure/function
in autism spectrum disorders cerebral circulatory system
communicating arteries
extracranial vessels
intracranial vessels
Brainstem stroke. See also Stroke Brief psychotic disorder
Bronchitis
Bronchopulmonary dysplasia
Brown-Séquard’s syndrome
Bulimia nervosa
definition
diagnosis
etiology
signs and symptoms
Burn(s)
case study
diagnosis
etiology
impact on occupational performance incident and prevalence
medical management
fluid resuscitation
inhalation injuries
nutritional support
pathophysiology
signs and symptoms
burn shock
contractures
depth of burn
hypermetabolism
infection
inhalation injury
scars
skin anatomy and physiology
wound management
allograft
autograft
debridement
hydrotherapy
ROM
topical dressings
Burn scar contracture
Burn shock
C
Caffeine
DSM-5
signs and symptoms
Cancellous bone/spongy bone
Cancer
case study
classification and staging
liquid tumor
solid tumors
definition
diagnosis
etiology
aging
environmental carcinogens
genetics
infectious agents
lifestyle
impact on client factors and occupational performance cancer-related
cognitive dysfunction cancer-related fatigue
cardiovascular considerations compromised bone
immobility
lymphedema
nutrition
pain
potential contraindications
psychosocial disruption
sexuality and intimacy
adolescent and young adult cancer older adult oncology
survivorship
medical management
bone marrow transplant
chemotherapy
hormone therapy
radiation treatment
surgery
targeted cancer therapy
oncologic emergencies
altered mental status
disseminated intravascular coagulopathy fever and neutropenia
hyperleukocytosis and leukostasis increased intracranial pressure
metastatic spinal cord compression neurological changes
superior vena cava syndrome
tumor lysis syndrome
signs and symptoms
Cancer-related cognitive dysfunction (CRCD) Cancer-related fatigue (CRF)
Cannabis
DSM-5
schizophrenia
signs and symptoms
Cannabis sativa, 271
Carcinomas
Cardiac rehabilitation
Cardiopulmonary disease
case study
chronic obstructive pulmonary disease congestive heart failure
coronary artery disease
heart
hypertension
impact on occupational performance lungs
myocardial infarction
vital signs
blood pressure
oxygen saturation
pulse rate
respiratory rate
Cardiovascular disease (CVD), diabetes and Carotid arteries
Carotid bruit, stroke risk and Carotid endarterectomy
Case studies
age related macular degeneration anxiety disorder
attention deficit hyperactivity disorder autism spectrum disorders
bipolar disorder
cancer
cardiopulmonary disease
cerebral palsy
cerebrovascular accident
Down syndrome
feeding and eating disorders intellectual disability
low vision disorders
major depressive disorder
muscular dystrophy
obsessive-compulsive and related disorders opioids
orthopaedic conditions
osteoarthritis
radial fractures
rheumatoid arthritis
schizophrenia
sedatives, hypnotics, and anxiolytics stimulants
stroke
Casein
Casting
Cataracts
case study
definitions
diagnosis
etiology
signs and symptoms
type
Catatonic schizophrenia
Catecholamines
in anxiety disorder
burn injury
Cauda equina injuries
Center for Epidemiology Studies Depression Scale (CESDS) Centers for Disease
Control and Prevention (CDC) Central cervical cord syndrome Central
scotomas
Central vision
Cerebellar tremors
Cerebellum
Cerebral angiography
Cerebral palsy
ataxia
athetoid (dyskinetic)
case studies
characteristics
cognitive impairment
definition
diagnosis
etiology
gastrointestinal difficulties impact on client factors
mental retardation and
oral motor
pulmonary defects
risk factors
seizure disorder and
signs and symptoms
atypical motor performance
atypical posture
delayed motor development
reflex abnormalities
tone abnormalities
spastic
characteristics
definition
diplegia
hemiplegia
quadriplegia
types
visual and hearing impairments in Cerebrospinal fluid analysis
Cerebrovascular accident (CVA). See also Stroke Cerebrum
anatomy
circulatory system
Certified lymphedema therapists (CLT) Certified nursing assistant (CNA)
Charcot–Marie–Tooth (CMT)
Checklist for Autism in Toddlers (CHAT) Chelation
Chemotherapy-induced peripheral neuropathy (CPIN) Childbirth, mood
disorders following Childhood disintegrative disorder. See also Autism
spectrum disorders signs and symptoms
Childhood obesity
Chlamydia pneumoniae, stroke risk and Choline acetyltransferase (CAT)
Chondropenia
Choreiform movement
Chromosomal aberrations, intellectual disability Chronic bronchitis
Chronic lymphocytic leukemia (CLL) Chronic myelogenous leukemia (CML)
Chronic obstructive pulmonary disease (COPD) course and prognosis
definition
etiology
signs and symptoms
Chronic pain
Cilia
Circle of Willis
Circumlocution
Client factors
in cerebral palsy
diabetes mellitus
muscular dystrophy
progressive neurological disorders Clonus
Closed fracture
Clozapine
Cocaine
Cognitive impairment
in autism spectrum disorders in cerebral palsy
duchenne muscular dystrophy
facioscapulohumeral muscular dystrophy in mood disorders
myotonic muscular dystrophy
Parkinson’s disease
schizophrenia
Cognitive-behavioral therapy (CBT) in ADHD
anxiety disorder
family based therapy (FBT)
HPD and SPD
OCD
for panic disorder
somatic symptom disorder
Collagen
Collateral circulation
Color-flow Doppler ultrasound Coma
Combined ADHD
Comminuted fracture
Communicating arteries
Complementary and alternative medicine, for ASD
antibiotics
antifungals
brain injury
chelation
gluten-free, casein-free diet secretin
supplements
Complex regional pain syndrome (CRPS) Complex trauma
case study
ACE
neurobiologic
resiliency factors
definition
etiology
impact on occupational performance IADLs
PADL
medical management
signs and symptoms
affect regulation
attachment
behavioral regulation
biology
cognition
dissociation
self-concept
Compound fractures
Compulsions. See also Obsessive-compulsive disorder (OCD) Computed
tomography (CT)
in cerebral palsy
stroke
subarachnoid hemorrhage
in traumatic brain injury
Congestive heart failure (CHF) course and prognosis
definition
etiology
signs and symptoms
Constraint-induced therapy (CIMT) Context
Continuous subcutaneous insulin infusion (CSII) Contractures
burn scar
in cerebral palsy
muscular dystrophy
traumatic brain injury
Contrast sensitivity
Contrecoup injury
Conus medullaris injuries
Co-occurring disorders (CODs) “Core Values and Attitudes of Occupational
Therapy Practice Coronary artery bypass graft (CABG) Coronary artery
disease (CAD) course and prognosis
definition
etiology
signs and symptoms
Cortical atrophy
Corticosteroids
cataracts
muscular dystrophy
Cortisol
Coup injury
COX-inhibiting nitric oxide donators (CINODs) Cranial nerve dysfunction
Craniostenosis
Creatine kinase (CK)
Credé’s maneuver
Crepitus
Cultured epithelium
CVA (Cerebrovascular accident). See also Stroke Cyanosis
Cyclooxygenase (COX) enzymes Cyclothymia
Cytomegalovirus
D
DAI (diffuse axonal injuries). See also Traumatic brain injury DAMP (deficits in
attention, motor control, and perception) De arthritide symptomatica
Debridement
Decerebrate rigidity
Decorticate rigidity
Decubitus ulcers
Decussation
Deep vein thrombosis (DVT)
stroke
Deficits in attention, motor control, and perception ((DAMP)) Deformity, feet
Degenerative joint disease (DJD) Delayed union
Delirium
definition
etiology of
medical management
signs and symptoms
Delirium tremens (DTs)
Delta-9-tetrahydrocannabinol (THC) Delusional disorder
Delusions, schizophrenia
Demyelination
Depression
in ADHD
brain injury
obesity
panic disorder
phobia
TBI
Dermis
Developmental coordination disorder (DCD), in ADHD
Developmental Trauma Disorder (DTD) Diabesity
Diabetes Control and Complications Trial (DCCT)
Diabetes mellitus
classification
diabetic foot
hypoglycemia
intensive insulin therapy
macrovascular complications
microvascular complications
neurologic complications
periodontal disease
definition
diagnosis
etiology
impact on client factors
body functions
occupational performance
medical management
blood glucose level, self-monitoring type 1
type 2
signs and symptoms
stroke risk and
Diabetic coma
Diabetic foot
Diabetic ketoacidosis (DKA)
Diabetic nephropathy
Diabetic peripheral neuropathy (DPN) Diabetic retinopathy (DR)
Diagnostic Adaptive Behavior Scale (DABS) Diagnostic and Statistical Manual
for Mental Disorders, Fifth Edition (DSM-5) in ADHD
anxiety
autism spectrum disorders
avoidant/restrictive food intake disorder mood disorders
OCD
substance-related disorders
Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition (DSM-
IV) autism spectrum disorders
Diastolic
Diesel engine exhaust
Difficulty coordinating movements (DCD) Diffuse axonal injuries (DAI) Dignity
Diminished emotional expression Diplegia
Disability Rating Scale (DRS) Diseasemodifying antirheumatic drugs
(DMARDs) Disinhibition
Disorganized behavior, schizophrenia Disorganized thinking
Disorganized type schizophrenia Disseminated intravascular coagulopathy (DIC)
Distal radial fractures
Disulfiram
Dopamine
Doppler ultrasound, in stroke Down’s syndrome
Alzheimer's disease
case study
etiology
impact of occupational performance intellectual disability
DRS
Duchenne muscular dystrophy (DMD) Duloxetine, fibromyalgia
Duplex ultrasonography, in stroke Dysarthria
in cerebral palsy
multiple sclerosis
spastic quadriplegia
Dysautonomia
Dysesthesia
Dysgraphia
Dyskinetic
Dysnomic aphasia
Dysphagia
muscular dystrophy
in stroke
Dysphoria
Dyspraxia
Dystonic movements
Dystrophin
E
Eastern Cooperative Oncology Group (ECOG) Ecchymosis
Echolalia
Edema
burn wound
in ischemic stroke
Education, SPD
Elder obesity
Electroconvulsive therapy (ECT) schizophrenia
Electrodermal reactivity
Electromyography (EMG)
Embolism
Embolus
Emery-dreifuss muscular dystrophy (EDMD) Emotional lability
Emphysema
Endarterectomy
End-stage renal disease (ESRD) Energy conservation
Environmental carcinogens
air pollution
IARC
radiation
Environmental Protection Agency (EPA) Epidermis
Epiglottis
Equality
Equinovalgus
Equinovarus
Eschar
Escharotomy
Essential tremors
Ethnicity
diabetes risk and
in rheumatoid arthritis
stroke risk and
Euphoria
definition
multiple sclerosis
Evidence-based practice
Excoriation. See Skin-picking disorder (SPD) Executive function
in ADHD
mild stage
moderate stage
severe stage
definition
in schizophrenia
Exercise
in diabetes
range of motion
Extrapyramidal symptoms, antipsychotics and Extrapyramidal syndrome
Eye, anatomy of
F
Facioscapulohumeral muscular dystrophy atrophy, fascial muscles
winged scapulae
Falls
Familial ALS
Familial Parkinson’s disease (FPD) Family based therapy (FBT)
Fasciculation
Fasting plasma glucose (FPG) Fatigue
in diabetes
diabetes mellitus
multiple sclerosis
Feeding and eating disorders case study
definition
diagnosis
etiology
signs and symptoms
Fetal alcohol spectrum disorders (FASDs) Fetal alcohol syndrome
Fibromyalgia
costs
diagnosis
signs and symptoms
tenderness location
Fistula
Flaccidity
Flight of ideas
definition
in mood disorders
Fluid resuscitation
Food selectivity, autism spectrum disorders Fowler position
Fractures
definition
diagnosis
distal radial
etiology
hip See (Hip, fractures) humeral
medical and surgical management pathologic
signs and symptoms
Fragile X syndrome
intellectual disability
Framework
Freedom
French-American-British (FAB) system Freud, Sigmund
Full-thickness burn injury
G
Gabapentin
Gait disturbances, in Parkinson’s disease Galantamine
Gambling
DSM-5
signs and symptoms
Gamma-aminobutyric acid (GABA) in anxiety disorders
Ganja
Gastrointestinal (GI) disorder Gastroparesis, in diabetes
GCS (Glasgow Coma Scale)
Generalized anxiety disorder case study
characteristics
diagnosis
Genetics
in autism spectrum disorders intellectual disability
MDD and BPD
multiple sclerosis
schizophrenia
single gene disorders
stroke risk and
Geographic location
multiple sclerosis and
stroke risk and
Gestational diabetes
Glare sensitivity
Glasgow Coma Scale (GCS)
Glaucoma
definitions
diagnosis
etiology
signs and symptoms
Global developmental delay (GDD) Globesity
Glucocorticosteroids (GCs)
Glucose monitors
Glutamate, in schizophrenia
Gluteal shelf
Gluten
Gout
costs
definition
diagnosis
etiology
signs and symptoms
Gower’s sign
Graft versus host disease (GVHD) Grandiosity
Greenstick fractures
Grossly disorganized behavior or catatonia Gunshot wounds (GSW)
H
Habit reversal training (HRT) Haemophilus influenzae, intellectual disability
Hair-pulling disorder (HPD)
definition
diagnosis
etiology of
signs and symptoms
Hallucinations
Hallucinogens
DSM-5
signs and symptoms
Hand, in rheumatoid arthritis Headache, in subarachnoid hemorrhage Hearing
impairments
in cerebral palsy
Heart
Heart disease. See also Cardiovascular disease stroke risk and
Hematoma
Hemianopsia. See also Homonymous hemianopsia Hemiparesis, in transient
ischemic attacks Hemiplegia
shoulder
spastic
Hemodialysis
Hemoglobin A1c (HbA1c)
Hemorrhagic strokes
aneurysm
etiology
heredity
intracerebral hemorrhages
signs and symptoms
Heparin, for stroke
Heredity, stroke risk and
Herpes simplex II
Heterotopic ossification (HO) course and prognosis
diagnosis
etiology
signs and symptoms
Hip
fractures
medical and surgical management signs and symptoms
Hippocampus
in autism spectrum disorders in schizophrenia
Hoarding disorder (HD)
definitions
diagnosis
etiology of
signs and symptoms
Homonymous
Homonymous hemianopsia
in cerebral palsy
in stroke
Horner’s syndrome
Humeral fractures, aging
Hydrocephalus
mental retardation
Hydrocephaly
Hydrotherapy, for burn wounds Hygiene hypothesis
Hyperactive delirium
Hyperactive-impulsive symptoms, in ADHD
Hyperactivity
Hyperglycemia
Hyperlexia
Hyperlipidemia
stroke risk and
Hyperphenylalaninemia
Hyperreflexia. See also Autonomic dysreflexia Hypertension (HTN)
definition
etiology
signs and symptoms
stroke risk and
Hypertonicity (spasticity), in cerebral palsy Hypertrophic scar
Hyperuricemia
Hypoactive delirium
Hypochondriasis
Hypoglycemia
Hypomania
Hypomanic episode
Hypothyroidism
Hypotonicity, in cerebral palsy Hypoxia
in burn injuries
I
Impact on occupational performance schizophrenia
substance-related disorders and addiction ADLs
education and work
IADLs
play and leisure
rest and sleep
social participation
Inattentive symptoms, ADHD
Incomplete injuries
Infections
cerebral palsy and
stroke risk and
Inferior olive
Inflammation, stroke risk and Inflammatory joint disease
Inhalants
DSM-5
signs and symptoms
Instrumental activities of daily living (IADL). See also Occupational
performance anxiety
complex trauma
education
play and leisure
rest and sleep
work
diabetes
generalized anxiety disorder muscular dystrophy
in progressive neurological disorders schizophrenia
education
leisure and social participation work
sensory processing disorder
SPD
Insulin
function
type 1 diabetes
type 2 diabetes
Insulin shock. See also Hypoglycemia Intellectual disability (ID) adaptive
behavior
adult-onset degenerative diseases case study
definition
diagnosis
environmental influences
perinatal factors
postnatal factors
prenatal factors
etiology
environmental influences
genetic causes
impact of occupational performance incidence and prevalence
intellectual functioning
signs and symptoms
Intellectual functioning
Intelligence quotient (IQ) score
Intensive insulin therapy
Interferon b-1b
International Agency for Research on Cancer (IARC) Intoxication disorders
Intraocular pressure (IOP)
Ischemic stroke. See also Stroke embolism
lacunar strokes/penetrating artery disease thrombosis
J
Joint attention
Joint guarding
Joint protection
Justice
Juvenile rheumatoid arthritis. See also Rheumatoid arthritis K
K2
Ketamine
Ketonuria
Knee-jerk reflex
Kyphosis
L
Lability, emotional
Lacunar stroke. See also Ischemic stroke Lamotrigine
Language
in Alzheimer’s disease
late
mild stage
moderate stage
delay, in autism spectrum disorders Learning and memory
mild stage
moderate stage
Learning disorders, in ADHD
Learning theory
Leptin
Lesch–Nyhan syndrome
Leukemia
Levels of Cognitive Functioning Scale (LCFS) Levodopa, for Parkinson’s
disease Lewy body pathology
Lifestyle, cancer
excessive alcohol
increased weight
physical inactivity
poor diet
tobacco use
Limb-girdle muscular dystrophy (LGMD) autosomal dominant
autosomal recessive
Limbic system
Lithium
Little, William
Long-term memory
Lordosis
Low birth weight, cerebral palsy and Low vision disorders
case study
cataracts
glaucoma
impact of conditions on occupational performance activities of daily living
education
instrumental activities of daily living play and leisure
work
macular degeneration See (Age-related macular degeneration) Lower motor
neuron (LMN) injuries Lumbar lordosis
Lund and Browder scale
Lungs
Lymphedema
Lymphoblasts
Lymphoma
M
Macula
Magnetic resonance imaging (MRI) bipolar disorder
in cerebral palsy
in multiple sclerosis
Major depressive disorder (MDD) case study
diagnosis
etiology
management
pharmacology for
repetitive transcranial magnetic stimulation signs and symptoms
Major depressive episode
Malignant tumors
Malingering
Malunion deformity
Malunion fracture
Manic episode
MAOIs (Monoamine oxidase inhibitors) Maturity-onset diabetes of the young
(MODY) Measles-mumps-rubella (MMR) vaccination
Medications
Melatonin, mood disorder
Mental retardation, cerebral palsy Mesh graft
Metabolic syndrome
Metamorphopsia
Metastasize
Metastatic disease
Metastatic spinal cord compression (MSCC) Methadone
Methylphenidate (MPH), in ADHD
Milnacipran
Mirel’s scoring system
Mixed amphetamine salts (MAS) Mixed delirium
Mixed episode
Mixed-type cancer
Modified Checklist for Autism in Toddlers (M-CHAT) Monoamine oxidase
inhibitors (MAOIs) Mood disorders. See Bipolar disorder (BPD); Major
depressive disorder (MDD) Motor deficits, in traumatic brain injury Motor
functions
in autism spectrum disorders and in traumatic brain injury
Motor neurons
Multiple sclerosis (MS)
case study
definitions
diagnosis
etiology
signs and symptoms
Munchausen’s syndrome
Muscular dystrophy
case study
definition
diagnosis
etiology
impact of conditions on client factors activities of daily living
education
play/leisure
psychosocial
work
medical management
drug intervention
nutrition
occupational and/or physical therapy pain
respiratory maintenance
skin integrity
signs and symptoms
surgical management
Myelin
Myeloblasts
Myeloma
Myocardial infarction
definition
etiology
signs and symptoms
Myopia
Myotonic muscular dystrophy (MMD) N
Naltrexone
National Center for Health Statistics (NCHS) National Child Abuse and Neglect
Data System (NCANDS) National Comprehensive Cancer Network
(NCCN) National Heart Lung and Blood Institute (NHLBI) National
Institutes of Health (NIH) Cancer
Diabetes Control and Complications Trial National Osteoporosis
Foundation (2010) National SCI Statistical Center (NSCISC) National
Survey on Drug Use and Health (NSDUH) National Toxicology
Program (NTP) Natural environment phobias. See also Phobia
NCTSN Task Force
Neoplastic
Neovascular glaucoma
Nephropathy
Neural plasticity
Neurocognitive disorders (NCD) case study
delirium
Neurodegeneration
Neuroleptic
Neuromuscular disorders
Neuromuscular dysfunction, intellectual disability Neuronal pruning
Neurons
Neurostimulation
Nicotine replacement therapy (NRT) Noncompliance
Nonsteroidal antiinflammatory drugs (NSAIDs), osteoarthritis Nontremor-
dominant PD
Nonunion fractures
Nosology
Nutritional support, in burn injuries Nystagmus
O
Obesity
body mass index
case study
childhood
complications
definitions
depression
disease
elder
etiology
genetic factors
hypothalamus and hypothyroidism neuroscience
impact on occupational performance medical and surgical management
prevalence and incidence
signs and symptoms
body types
lymphedema
obesity hypoventilation syndrome osteoarthritis
stigma
stress
stroke risk and
in type 2 diabetes risk and
waist size
Obesity hypoventilation syndrome (OHS) Oblique fracture
Obsession
Obsessive-compulsive disorder (OCD) case studies
definitions
diagnosis
etiology of
signs and symptoms
Obsessive-compulsive and related disorders (OCRDs) body dysmorphic disorder
definitions
diagnosis
etiology of
signs and symptoms
case studies
hair-pulling disorder
definition
diagnosis
etiology of
signs and symptoms
hoarding disorder
definitions
diagnosis
etiology of
signs and symptoms
obsessive-compulsive disorder course and prognosis
definitions
diagnosis
etiology of
signs and symptoms
occupational performance
body structures
mental functions
sensory functions
skin and related functions
values, beliefs and spirituality skin-picking disorder
definition
diagnosis
etiology of
signs and symptoms
Occupation
Occupational performance
in amyotrophic lateral sclerosis anxiety disorder
attention deficit hyperactivity disorder in bipolar disorders
burns
cancer
cardiopulmonary disease
in cerebral palsy
complex trauma
diabetes mellitus
feeding and eating disorders generalized anxiety disorder in major
depressive disorder obsessive-compulsive and related disorders
rheumatoid arthritis
schizophrenia
Occupational therapist (OT)
Occupational therapy
core values
person-fist language
philosophical assumptions
professional language
Occupational Therapy Practice Framework (OTPF) Opacifications
Open fracture
Open reduction and internal fixation (ORIF) Open-angle glaucoma
Opioids
case study
DSM-5
signs and symptoms
Oppositional behavior disorder, in ADHD
Optic neuritis
Oral contraceptives, stroke risk and Oral glucose tolerance test (OGGT)
Orthopaedic conditions
case-studies
fractures See (Fractures) heterotopic ossification
osteoarthritis
osteoporosis
Orthopneic position
Osteoarthritis
case study
common locations and symptoms course and prognosis
definition
diagnosis
etiology
societal costs
Osteoblastic lesions
Osteolytic lesions
Osteopenia
Osteoporosis
definition
diagnosis
etiology
signs and symptoms
P
Pain
Pallidotomy
Panic attack
Panic disorder
diagnosis
signs and symptoms
Pannus
Paranoia, schizophrenia
Paraphasia
Parentification
Parkinson’s disease (PD)
case study
definitions
diagnosis
etiology
lewy body pathology
pathophysiology
signs and symptoms
Paroxetine
Partial-thickness burn injury Pathological fracture
Patient Outcomes Research Team (PORT) Pear abducted
Pear adducted
Pediatric autoimmune neuropsychiatric disorder associated with streptococcus
(PANDAS) Pediatric stroke
Penetrating artery disease. See also Lacunar stroke Penumbra
Perceptual-motor
Perceptual-motor abilities
Perceptual-motor skills
Performance in areas of occupation. See also Occupational performance
Performance patterns
Performance skills
Perinatal factors, intellectual disability Periodontal disease, as risk factor for
stroke Peripheral artery disease (PAD) Peristalsis
Personal activities of daily living (PADL) bathing
feeding and eating
hygiene and grooming
sexual activity
toileting
Personal episodic memory
Person-first language
Pervasive developmental disorder not otherwise specified (PDD-NOS). See also
Autism spectrum disorders Pervasive Developmental Disorders Screening
Test, Stage 1 (PDDST-I) PET. See Positron emission tomography
Phencyclidine (PCP)
Phenobarbital
Phenylketonuria (PKU)
Phobia
case study
definition
diagnosis
signs and symptoms
Physiologic tremor
PICA
Pica
definition
diagnosis
etiology
signs and symptoms
Play, sensory processing disorder material management
pretense-symbolic; imitation and dramatization space management
Polydipsia
Polyphagia
Polyuria
Positron emission tomography (PET) bipolar disorder
Posterior adipose
Postnatal factors, intellectual disability Posttrauma vision syndrome
Posttraumatic hydrocephalus
Posttraumatic stress disorder (PTSD) Postural disorders
Pot
Practice framework, occupational therapy Preclinical period
Prediabetes
Pregabalin
Pregnancy, risk factors for cerebral palsy Prematurity, cerebral palsy and
Premorbid functioning
Prenatal factors, intellectual disability Primary hypertension
Primary progressive
Primitive reflexes
Prior stroke, stroke risk and Procedural memory
Prodromal
Prodromal period
Prodromal phase
Progressive neurodegenerative disorder (PND) amyotrophic lateral sclerosis See
(Amyotrophic lateral sclerosis) case study
impact of conditions on occupational performance activities of daily living
education
instrumental activities of daily living play and leisure
work
multiple sclerosis See (Multiple sclerosis) Parkinson’s disease See
(Parkinson’s disease) Prudence
Psychomotor agitation
Psychomotor retardation
Psychosis. See also Schizophrenia Psychosocial risk factors, mood disorder
Pupillary reflexes, in traumatic brain injury Purkinje cells
Q
Quadriplegia, spastic
Quetiapine
R
Radial fractures
case study
signs and symptoms
Rai system
Rancho Los Amigos Scale
Random plasma glucose (RPG)
Range of motion (ROM), in burn injuries Reflex arc
Reflex sympathetic dystrophy Relapse, in schizophrenia
Relapsing-remitting nonprogressive Relapsing-remitting-progressive
Remodeling
Repetitive transcranial magnetic stimulation (rTMS) Rest and sleep, SPD
Resting tremors
Retina
Retinopathy
Retrograde amnesia
Rett’s disorder. See also Autism spectrum disorders Reversible ischemic
neurological deficit (RIND) Rheumatoid arthritis (RA)
anatomy
case study
diagnosis
etiology
history
signs and symptoms
cervical degeneration
feet
hip
index finger boutonniere deformity inflammatory joint disease
swanneck deformity
zigzag deformity
surgery
Rhizotomy
Riluzole
RIND (reversible ischemic neurologic deficit) Risperidone, in autism spectrum
disorders and Ritalin, in ADHD
ROM (range of motion), in burn injuries Rote memory
Rumination
definition
diagnosis
etiology
signs and symptoms
S
Sarcomas
Sarcopenia
Satiety
Scaphoid fractures
Schizoaffective disorder
Schizophrenia
case study
definition
diagnosis
etiology
impact on occupational performance activities of daily living
education
instrumental activities of daily living leisure and social participation
work
pharmacological treatment
repetitive transcranial magnetic stimulation signs and symptoms
affective symptoms
cognitive symptoms
delusions
disorganized thinking
grossly disorganized behavior or catatonia hallucinations
negative symptoms
Schizophreniform disorder
Scoliosis
Secondary hypertension
Secondhand tobacco smoke
Secretin
Sedatives, hypnotics, and anxiolytics case study
signs and symptoms
Seizures
in cerebral palsy
Selective dorsal rhizotomy (SDR) Selective serotonin reuptake inhibitors
(SSRIs) Self-monitoring of blood glucose levels (SMBG) Semantic
memory
Sensory craving
Sensory deprivation
Sensory discrimination disorder (SDD) Sensory disturbances, multiple sclerosis
Sensory Integration and Learning Disorders Sensory integration deficits, in
ADHD
Sensory integration dysfunction (SID) Sensory modulation disorder (SMD)
sensory craving
sensory overresponsivity
sensory underresponsivity
typologies
Sensory overresponsivity (SOR) Sensory processing disorder (SPD) case study
diagnosis
etiology
impact on occupational performance BADLs
education
IADLs
play
rest and sleep
work
sensory discrimination
sensory modulation disorder
sensory-based motor disorder Sensory underresponsivity (SUR)
Sensory-based motor disorder dyspraxia
postural disorders
sensory discrimination
Serotonin and norepinephrine reuptake inhibitors (SNRIs) Serotonin, in mood
disorder
Sheet graft
Short Sensory Profile (SSP)
Silver sulfadiazine
Single gene disorders
Situational phobias
Skin, in diabetes
Skin-picking disorder (SPD)
definition
diagnosis
etiology of
signs and symptoms
Skin-Picking Impact Scale (SPIS) Sleep apnea
Sleep disturbance
Smoking
schizophrenia
stroke risk and
Social cognition
mild stage
moderate stage
Social participation, SPD
Social phobia
diagnosis
etiology
signs and symptoms
Somatic symptom disorder
case study
conversion disorder
definition
DSM-5 diagnostic criteria
DSM-IV vs. DSM-5
etiology
factitious disorders
illness anxiety disorder
impact of occupational performance incidence and prevalence
medical management
psychological factors
signs and symptoms
symptoms
Somatosensation
Somnolence
South Beach diet
Spasticity. See also Hypertonicity Spina bifida
Spinal cord injury
case study
cauda equina injuries
CNS
complete versus incomplete injuries conus medullaris injuries
etiology 455
instrumental activities of daily living care of others
education
functional mobility
functional outcomes
grooming, oral hygiene and eating health maintenance
home maintenance
leisure activities
personal device care
sexual expression
socialization, functional socialization, functional response toileting
vocational
posttraumatic complications
autonomic dysreflexia (hyperreflexia) bowel function
deep vein thrombosis
dermal complications
genitourinary complications
mental health challenges
musculoskeletal
postural hypotension
respiratory complications
spinal shock
thermal regulation
reflex arc
sensory and motor tracts
signs and symptoms
anterior cord syndrome
Brown-Séquard’s syndrome
central cervical cord syndrome sensory functions
Spinal muscular atrophy (SMA) Spiral fracture
Spirometry
Splinting, in cerebral palsy Sporadic ALS
Sporadic Parkinson’s disease (SPD) Sports-related spinal cord injuries Ssensory-
based motor disorder (SBMD) Standard medical evaluation procedures, in
ADHD
Static encephalopathy. See also Cerebral palsy Stereotyped behavior
Stimulants
case study
DSM-5
signs and symptoms
Strabismus
Stretch reflex
Stroke. See also Vascular dementia case study
children
complications
infection
seizures
thromboembolism
definitions
diagnosis
invasive study
neuroimaging techniques
noninvasive study
physical examination
etiology
impact on occupational performance emotional functions
mental functions
motor functions
sensory functionsff
lacunar strokes/penetrating artery disease medical/surgical management
anticoagulants
antiplatelet therapy
surgical interventions
thrombolytics
treatment of secondary effects signs and symptoms
anterior cerebral artery stroke middle cerebral artery
neurological effects
small strokes
subclavian steal syndrome
transient ischemic attacks
vertebrobasilar stroke
Wallenberg’s syndrome
Subarachnoid hemorrhages
Subclavian steal syndrome
Subcutaneous tissue
Sublingual pills
Substance abuse, stroke risk and Substance use disorders
definition
impaired control
pharmacological criteria
risky use
social impairment
tolerance
Substance-induced disorders
intoxication disorders
withdrawal disorders
Substance/medication-induced psychotic disorder Substance-related disorders
alcohol
DSM-5
signs and symptoms
caffeine
DSM-5
signs and symptoms
cannabis
DSM-5
signs and symptoms
continuum of care model
diagnosis
etiology
gambling
DSM-5
signs and symptoms
hallucinogens
DSM-5
signs and symptoms
impact on occupational performance ADLs
education and work
IADLs
play and leisure
rest and sleep
inhalants
DSM-5
signs and symptoms
intoxication disorder
opioids
case study
DSM-5
signs and symptoms
sedatives, hypnotics, and anxiolytics case study
DSM-5
signs and symptoms
stimulants
case study
DSM-5
signs and symptoms
tobacco
DSM-5
signs and symptoms
withdrawal disorder
Substantia nigra
Suicide
in schizophrenia
Sundowning
Superficial burn injury
Superior vena cava syndrome (SVCS) Supracondylar fractures
Sustained attention
Swan-neck deformity
Symptomatic period
Synthetic cannabinoid
Systemic lupus erythematosus direct and indirect costs
etiology
T
Tachycardia
Tactile defensiveness
Tardive dyskinesia
Tau
Tay-Sachs disease
TBI Model Systems database
Temporal artery bypass
Teratogenic
Thalamotomy
The Screening Tool for Autism in Two-Year-Olds Theory of mind
Thorazine
Thrombolytic medicine
Thrombosis, cerebral. See also Ischemic stroke Thrombus
Tobacco
DSM-5
signs and symptoms
Tonic labyrinthine reflex (TLR) Topiramate
Topographical orientation
Total body surface area (TBSA) Total Hip Precautions (THP)
Total hip replacement
Total knee replacement (TKR) Toxemia
Toxins, in progressive neurological disorders t-PA
Trabecular meshwork
Transcranial Doppler ultrasound, in stroke Transcranial magnetic stimulation
(TMS), schizophrenia Transgenerational
Transient ischemic attacks (TIA) as predictor of stroke
stroke risk and
Transverse fracture
Trauma
complex See (Complex trauma) intellectual disability
mood disorder
schizophrenia
Traumatic brain injury
case study
coma
definition
etiology
mild
moderate
rehabilitation
driving
impact of occupational performance inpatient facility
work
severe
signs and symptoms
cognitive deficits
cranial nerve dysfunction
medical complications
motor deficits
psychosocial deficits
Tremor
in Parkinson’s disease
types
Tremor-dominant PD
Trichotillomania. See Hair-pulling disorder (HPD) Trichotillomania Learning
Center in California Tricyclic antidepressants
Triglycerides, stroke risk and Truth
Tumor lysis syndrome (TLS)
Type 1 diabetes, . See also Diabetes mellitus Type 2 diabetes. See also Diabetes
mellitus Tyrosine kinase inhibitor (TKI) U
Ultrasonography, stroke
Unipolar depression
University of Rochester Minnesota Department of Senior Health Research
(URMCR) Unwalkable disease
Upper motor neuron (UMN) injury V
Valley sign
Varenicline
Vascular dementia
Vegetative state
Verbal fluency, in schizophrenia Vertebrobasilar stroke. See also Stroke
Vigilance
Vineland Adaptive Behavior Scales, Second Edition (VAB-II) Visual
hallucinations
Visual impairments
cerebral palsy
in intellectual disability
Visuospatial deficits, in stroke Volkmann’s deformity
W
Waist circumference
Wallenberg’s syndrome
Warfarin, for stroke
Wear and tear disease
Weed
White matter
Widespread Pain Index (WPI) score Withdrawal disorders
World Health Organization (WHO) classification Wound healing
Wrist
fractures
X
Xanthine oxidase inhibitors (XOI) Xenophobia. See also Phobia X-linked
disorders
Y
Yale Food Addiction Scale (YFAS) Yale-Brown obsessive-compulsive scale
(YBOCS) Z
Zigzag deformity
Ziprasidone
Zone of coagulation
Zone of hyperemia
Zone of stasis

Conditions in Occupational Therapy

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Conditions in Occupational Therapy

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CONDITIONS

Diane Powers Dirette, PhD, OTL, FAOTA

Library of Congress Cataloging-in-Publication Data

—Diane Powers Dirette

Ben J. Atchison, PhD, OTR/L, FAOTA Professor and Chair

Ann Chapleau, DHS, OTRL

Gerry E. Conti, PhD, OTRL, FAOTA

Joan Ziegler Delahunt, OTD, MS, OTR/L

Diane Powers Dirette, PhD, OTL, FAOTA

Kathryn Ellsworth, MA, CCC-SLP

Joanne Phillips Estes, PhD, OTR/L

Jennifer L. Forgach, MS, OTR/L

Joyce Fraker, MS, OTR/L

Cynthia A. Grapczynski, EdD, OTR, FAOTA

Jennifer Harrison, PhD, MSW

Heather Javaherian-Dysinger, OTD, OTR/L

Reese Martin, MS, OTR/L

Laura V. Miller, MS, OTR/L, CDI, CDRS

Brandon G. Morkut, MS, OTR/L

Timothy M. Mullen, PhD, OTR/L, CHT

Shelley Mulligan, PhD, OTR/L, FAOTA

Linda M. Olson, PhD, OTRL

Rebecca Ozelie, DHS, OTR/L, BCPR

Sharon L. Pavlovich, MAM, COTA/L

Emily Raphael-Greenfield, EdD, OTRL, FAOTA Assistant Professor

Michelle A. Suarez, PhD, OTR/L

Suzänne Fleming Taylor, PhD, MBA/HCM, OTR/L

Michael J. Urban, MS, OTR/L, MBA, CEAS, CWCS

Christine K. Urish, PhD, OTR/L, BCMH, FAOTA Professor

Andrea L. Washington, BS, OTR/L

Mary Steichen Yamamoto, MS, OTL

Jayne Yatczak, PhD, OTRL

In this fifth edition of Conditions in Occupational Therapy: Effect on

To review key terms and their definitions, visit

UNIT 1 • PEDIATRIC CONDITIONS

UNIT 2 • MENTAL CONDITIONS

UNIT 3 • PHYSICAL CONDITIONS

Lindsey is finishing her course work in occupational therapy and is now

Each individual has a right to a meaningful existence: the right to live in

TABLE 1.1 Occupational Therapy Practice Domains

1. Spastic is characterized by hypertonicity. Deep tendon reflexes are present

Since the functioning of these abilities is impacted in individuals with autism, it

Support for Genetic Etiology

TABLE 3.1 Levels of Severity within the Autism Spectrum

TABLE 3.2 Sensory Processing Disorder in Children with ASD

The Checklist for Autism in Toddlers (CHAT) (Baron-Cohen, Allen, &

1. History: Though autism is not known to result from complications during

The Autism Diagnostic Interview: Revised (Lord, Rutter, & LeCouteur,

5. Tests in language development. Examples of tools include the following:

Preschool Language Scales (PLS) (Zimmerman, Steiner, & Pond, 2011)

6. Tests in cognitive development:

Mullen Scales of Early Learning (MSEL) (Mullen, 1995).

7. Tests in other developmental areas including sensory processing, motor,

Sensory Profile 2 (Dunn, 2014)

Complementary and Alternative Medicine

TABLE 3.3 Potential Side Effects of Complementary and Alternative

Gluten-Free, Casein-Free Diet

Other Common Complementary and Alternative Medicine Treatments

The effectiveness of complementary and alternative medicine stands largely

TABLE 4.1 Adaptive Skills