Download IntellectualDevelopmentalDisabilities.GNRS5

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INTELLECTUAL AND
DEVELOPMENTAL
DISABILITES
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OBJECTIVES
Know and understand:
• The definition of intellectual disability (ID) and its
prevalence among older adults
• How to recognize and manage psychiatric, mental, and
behavioral disorders in older adults with ID
• Ways to overcome barriers to diagnosis and treatment
• Common comorbidities found in individuals who have a
developmental disability (DD), with or without ID
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TO P I C S C O V E R E D
• Nomenclature
• Prevalence
• Diagnostic and Treatment Issues
• Psychiatric and Mental Disorders in Aging Adults
with ID
• Medical Disorders
• Social Conditions
• Developmental Disabilities and Comorbidity
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NOMENCLATURE
• Definition of ID: deficits in intellectual abilities that
impact functioning in 3 areas
 1. conceptual skills
 2. social and interpersonal skills
 3. Self-management skills
Not everyone with a DD has ID
• This slide set focuses on individuals with ID who may or
may not have a comorbidity such as cerebral palsy,
epilepsy, or autism spectrum disorder
• The term “mental retardation” has been replaced with
“intellectual disability” in the DSM V
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PREVALENCE OF ID
• Life expectancy for people with ID has
increased substantially
 1930: Average age of death 19
 1990s: life expectancy increased to 66y
• Prevalence currently 1%–2% in the US
 For people 60 yr, this number expected to
double by 2030
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ID AND MENTAL ILLNESS
• Adults with ID have similar risk factors for mental
illnesses as their peers without disability but may have
additional risks depending on the cause of their mental
disability
• Older adults who were raised in institutions or did not
benefit from modern medical care are also at greater
risk
• The prevalence of psychiatric disorders among adults
with ID is about 5 times that of age-matched controls
(10%–40%)
• Among the most common disorders is dementia
BARRIERS TO DIAGNOSIS
AND TREATMENT (1 of 2)
• Limited self-awareness
 Estimate the degree to which the patient is aware of his or her
problem, condition, or feelings
 Avoid complex questions and high-level vocabulary
• Limited communication ability
 Receptive and expressive abilities can be comparable or
quite different
 Differences in these abilities is a characteristic feature of
some conditions
 Consult collateral sources of information, such as family or
caregivers who can provide histories and other data
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BARRIERS TO DIAGNOSIS
AND TREATMENT (2 of 2)
• Diagnostic overshadowing
 The idea that the patient’s symptoms and behavior are
attributed to their intellectual disability, leaving comorbid
conditions undiagnosed and untreated
 Diagnostic overshadowing is a barrier to critical thinking,
and clinicians should guard against it when presented with
a difficult situation
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PSYCHIATRIC AND MENTAL DISORDERS
IN AGING ADULTS WITH ID
• The occurrence and severity of psychiatric
disturbances can vary by age and comorbid
conditions
• Some symptoms may improve as the patient ages or
develops comorbid problems
• Some behaviors or conditions can worsen with age
through various processes
PROBLEM BEHAVIOR CAN SIGNAL
PHYSICAL ILLNESS IN ADULTS WITH ID
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• In people who are lower-functioning or have an
expressive communication disorder, a new behavioral
concern can be a sentinel sign of a physical disorder
• As a general rule, before determining that a new
problem behavior should be treated with a
psychotropic medication or intervention, physical
causes should be excluded
• New-onset self-injurious behavior can be a particularly
important clue to occult illness
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DEMENTIA AND ID
• Individuals with ID have a higher overall prevalence of
dementia than is found in age-matched controls in the
general population
• All causes of dementia are possible, but some are
more likely than others
 Down syndrome
 “Pugilistic dementia” associated with repeated
self-injuring blows to the head from
coup/contracoup effects
INCIDENCE OF DEMENTIA IN ADULTS
WITH DOWN SYNDROME
• There is an association and a significantly increased
risk of dementia and Down syndrome
• Nearly 100% of adults with Down syndrome have
developed the characteristic histologic
neuropathology of dementia by age 40; however, it is
not typical to develop overt dementia at this young an
age
• It is common for at least 50% of adults with Down
syndrome who are ≥60 years old to have clinical
evidence of dementia
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LIFE EXPECTANCY OF ADULTS WITH
DEMENTIA AND DOWN SYNDROME
Median age of death, yr
60
49
50
40
30
25
20
10
0
1983
1997
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DIAGNOSIS AND TREATMENT
OF DEMENTIA IN PEOPLE WITH ID
• Dementia is diagnosed according to the same criteria
as in the general population:
 Establish cognitive and adaptive deterioration
 Demonstrate deficits on exam (preferably with longitudinal
follow-up showing progression of deficits)
 Exclude other possible causes of deterioration and other
mental disorders
• Body of evidence is increasing for modest palliative
efficacy in this population of cholinesterase inhibitors
(donepezil, rivastigmine, galantamine) and the
glutamate receptor antagonist (memantine)
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ADAPTIVE BEHAVIORAL DIFFICULTIES
• Types of adaptive behaviors:
 Conceptual (eg, language, reading, writing)
 Social (eg, rules, sense of responsibility)
 Practical (eg, job skills, eating, dressing)
• In general, the greater the severity of ID, the lower the
level of adaptive abilities, but these abilities can be
improved over time with behavioral supports
• Like any group, aging adults with ID can lose or
become less adept with some adaptive behavior skills
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MALADAPTIVE BEHAVIORS
• Examples: withdrawal, self-injury, stereotypy
• Severe or frequent in up to 50%–60% of adults with ID
 Can persist for years
• The proportion decreases with age, for various reasons
 Exception 1: In Down syndrome the proportion is
higher and the incidence of behavioral problems
increases with the degree of ID
 Exception 2: Aggression is similarly frequent in all
age groups; presentation is extremely variable
DIAGNOSIS OF MENTAL DISORDERS
IN OLDER ADULTS WITH ID
• Follow the same principles of history-taking and
examination that apply for the general population,
with the caveats discussed on the previous 2 slides
• Mental disorders often present as behavioral
changes
 The reports of family or other caregivers are
extremely important
 Consider a change in staff, residential or
vocational setting, or family health as a
precipitating factor for behavioral changes
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TREATMENT OF MENTAL DISORDERS IN
OLDER ADULTS WITH ID
• The appropriate treatment or response might be
instructional or behavioral
 Preferred behavior programs reward desirable
behavior using positive reinforcement techniques
• Pharmacologic intervention may be necessary for the
safety of the patient or those nearby, in particular for
physical aggression
 Very few medications are approved for the most
common and challenging behaviors, and
prescribing medications off-label is common
PRINCIPLES OF
MEDICATION MANAGEMENT
• Treating major mental illness in older adults with ID is
similar to treating the general population
• Change only one medication at a time
• Start new medications at a low dosage and monitor the
results (“start low, go slow”)
• If possible, taper and ultimately D/C all medications
• Avoid antipsychotic medications
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MEDICAL DISORDERS
• Adults with ID have more medical problems than agematched individuals
 About 5 medical conditions per person
 People with severe ID have even more
 About 2/3 of community-dwelling people with ID
have chronic conditions or major physical disability,
50% of which go undetected
• Visual or hearing impairments are particularly common
in people with ID
 They increase with age and affect about 25%
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LIFE EXPECTANCY
• Life expectancy for adults with ID is ~65 yr
 Decreases with increasing severity of ID
 Decreases with comorbidities such as inability to
ambulate, lack of feeding skills, and incontinence
• The most common causes of death are CVD,
respiratory disorders, cancer, and dementia
(particularly in Down syndrome)
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SOCIAL CONDITIONS
• At least 80% of adults with ID are cared for at home by
aging family members
• About 40% of eligible individuals are not served by the
formal service system
 Can lead to crisis when the family can no longer
provide care or manage a behavioral problem
• About half of adults with ID and a behavior problem
eventually need a different living arrangement
 In a typical system, more than half of families have
not made plans for future care
DEVELOPMENTAL DISABILITIES
AND COMORBIDITY (1 of 5)
Change with
developmental disabilities
Management strategies
Intellectual disability
Two thirds of patients with DD
suffer from ID, many in the mild-tomoderate range
Evaluation and referral to
specialized services to
maximize intellectual potential
Growth retardation
Usually found in patients with
moderate to severe disabilities; it
may present as short stature,
inability to gain weight, lack of
sexual development, or failure to
thrive
Medical evaluation for
treatable causes
Sensory impairment
Nearly 90% of patients have
impairments in hearing, vision, and
speech. Strabismus is common, as
is dysarthric speech.
Regular evaluation of hearing,
vision, and speech; correction
of deficits
System/condition
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DEVELOPMENTAL DISABILITIES
AND COMORBIDITY (2 of 5)
System/condition
Change with
developmental disabilities
Management strategies
Dental/oral
conditions
Poor dentition and oral health are
very common
Oral hygiene and tooth
brushing; regular dental visits
Thyroid problems
Thyroid problems can be a cause or
a result of developmental disability
Regular testing and treatment
as indicated
Spinal deformities
Kyphosis, scoliosis, and lordosis are Monitoring of body habitus;
common among patients with
physical therapy
muscle weakness and spasticity
Seizure disorders
Half of patients may suffer from
some type of seizure disorder
Diagnosis; anticonvulsant
medications
Degenerative joint
disease
Chronic muscle spasticity and
mobility limitations often lead to
osteoarthritis and joint disease.
Strength and functional status may
be prematurely impaired.
Physical therapy, occupational
therapy, pain management
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DEVELOPMENTAL DISABILITIES
AND COMORBIDITY (3 of 5)
System/condition
Change with
developmental disabilities
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Management strategies
Osteopenia and
osteoporosis
Lack of weight bearing leads to these
chronic conditions in patients who are
unable to ambulate
Promotion of mobility
(physical therapy); adequate
calcium and vitamin D
supplementation, screening
and treatment of
osteoporosis
Chronic pain
syndromes
Muscle abnormalities and associated
spinal deformities often result in
chronic pain syndromes. Sensory
abnormalities can result in the inability
to describe the type, location, and
source of the pain.
Regular monitoring of
function and behavior to
detect possible painful
conditions; pain management
DEVELOPMENTAL DISABILITIES
AND COMORBIDITY (4 of 5)
System/condition
Change with
developmental disabilities
Management
strategies
Functional decline
Aging patients with cerebral palsy and other
Physical therapy,
similar conditions often develop fatigue, pain, occupational therapy,
weakness, and overuse syndromes that
pain management
result in premature loss of function. This is
referred to as post-impairment syndrome and
often requires a reduction in work hours,
increase in assistance or use of adaptive
devices, and/or nursing-home placement.
Cardiac and
pulmonary
conditions
Patients with cerebral palsy and other similar
physical disabilities typically require 3−5
times the energy level of unimpaired adults,
predisposing patients to premature
conditions of aging, such as hypertension,
heart failure, and coronary artery disease
Monitoring for
hypertension,
shortness of breath,
angina; risk factor
management;
engaging in regular
physical activity and
healthy diet
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DEVELOPMENTAL DISABILITIES
AND COMORBIDITY (5 of 5)
System/condition
Change with
developmental disabilities
Management strategies
GI conditions
Gastroesophageal reflux disease and
constipation are common; constipation
can be chronic and severe
Monitoring; medications;
fiber-rich diet; exercise
Incontinence
Many patients are incontinent of bowel
and bladder from childhood, but others
develop these problems with age
Screening for treatable
causes; identifying functional
impairments that can limit
toileting
Depression and
mood disorders
Patients with cerebral palsy are 4
times more likely to develop
depression as age-compared other
adults. The stress associated with
multiple disabilities is a risk factor, as
is the premature decline in functional
status associated with the disorder.
Regular screening;
counseling and/or
medications for those
diagnosed with mood
disorder
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S U M M A RY ( 1 o f 2 )
• An increasing number of individuals with ID are
surviving into adulthood and old age.
• Maladaptive behaviors, as well as difficulties in learning
and retaining new skills of coping and adaptation, are
significant problems for adults with ID and,
consequently, for their caregivers.
• Impairments in receptive and expressive communication
and coexisting cognitive limitations can contribute to
difficulties in the diagnosis and treatment of medical,
psychiatric, and behavioral problems.
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S U M M A RY ( 2 o f 2 )
• In individuals with ID, disease states and physiologic
changes related to age can exacerbate or attenuate
maladaptive behaviors.
• Therapeutic interventions for maladaptive behaviors
or psychiatric illnesses that coexist with ID can
include medications and behavioral therapies.
• The term “developmental disability” can be applied to
a variety of medical conditions that are not defined by
ID. However, these conditions can contribute to
maladaptive behaviors and affect an individual’s
quality of life.
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CASE 1 (1 of 3)
• A 60-year-old man with an intellectual disability who lives in a
residential facility, accompanied by a new staff person
• Staff person reports that her colleagues have noted the following
over the past few days:
 An increase in self-injurious behaviors (biting himself, hitting his head on
the wall), both at the residence and at the workshop he attends daily. No
serious injury has occurred.
 Although he has no history of aggression toward others, he recently
struck out at another resident who bumped into him in the dining room.
 The behaviors significantly worsened after he visited his mother, who is
in a nursing facility recovering from hip fracture.
• The patient says that he has no pain or other physical complaint.
• When asked about his behavior, he states that he is sad but is
unable to explain in more detail.
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CASE 1 (2 of 3)
Which one of the following is the best initial strategy for
treating the behavioral disturbance?
A. Provide support and behavioral interventions at
residence and workshop.
B. Provide support and behavioral interventions at
residence, and stop workshop attendance until behavior
improves.
C. Start risperidoneOL 0.25 mg/d.
D. Admit to inpatient psychiatric unit for observation and
treatment.
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CASE 1 (3 of 3)
Which one of the following is the best initial strategy for
treating the behavioral disturbance?
A. Provide support and behavioral interventions at
residence and workshop.
B. Provide support and behavioral interventions at
residence, and stop workshop attendance until behavior
improves.
C. Start risperidoneOL 0.25 mg/d.
D. Admit to inpatient psychiatric unit for observation and
treatment.
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CASE 2 (1 of 3)
• A 55-year-old woman with Down syndrome,
accompanied by her parents, with whom she has lived
her entire life
• Parents report a change in her ability to perform tasks
that she had previously mastered.
• They do not know when the change first began, but
their son visited recently and noticed a difference
compared with when he last saw her 1 year ago.
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CASE 2 (2 of 3)
Which one of the following is the best tool for assessing
whether this patient may have dementia?
A. Functional Assessment Staging Test (FAST)
B. Dementia Screening Questionnaire for Individuals with
Intellectual Disabilities (DSQIID)
C. Down Syndrome Mental Status Examination (DSMSE)
D. Mini–Mental Status Examination (MMSE)
E. Montreal Cognitive Assessment (MoCA)
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CASE 2 (3 of 3)
Which one of the following is the best tool for assessing
whether this patient may have dementia?
A. Functional Assessment Staging Test (FAST)
B. Dementia Screening Questionnaire for Individuals with
Intellectual Disabilities (DSQIID)
C. Down Syndrome Mental Status Examination (DSMSE)
D. Mini–Mental Status Examination (MMSE)
E. Montreal Cognitive Assessment (MoCA)
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CASE 3 (1 of 4)
• A 58-year-old woman with an intellectual disability, accompanied
by her parents. They want to establish care in a practice that has
mostly female providers; the patient refuses to see male providers.
• History includes a seizure disorder. Seizures occur 1-2 times each
month, despite medication. Seizure frequency and the medication
regimen have not changed recently.
• She has lived with the family most of her life, except for a few
months in a group home in her early 20s that did not work out
because “some bad things happened.” She came to the office
today because her parents promised her ice cream after the visit
and told her that she would not get any shots.
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CASE 3 (2 of 4)
• She has never had a Pap smear or undergone mammography or
colonoscopy. She has had the same eyeglass prescription for 5
years.
• The patient refuses to undress and allows only a limited
examination. She has very poor dentition, consistent with her
refusal to go to the dentist. She is overweight. Gait is normal.
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CASE 3 (3 of 4)
Which one of the following is the best next step in
providing care for this patient?
A. Order mammography and refer to a gynecologist for
Pap smear.
B. Have blood drawn for medication levels at end of visit.
C. Refill prescriptions and schedule follow up in 3 months.
D. Provide education on healthy lifestyle changes and
follow up in 2 weeks.
E. Refer for dental work and Pap smear under general
anesthesia.
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CASE 3 (4 of 4)
Which one of the following is the best next step in
providing care for this patient?
A. Order mammography and refer to a gynecologist for
Pap smear.
B. Have blood drawn for medication levels at end of visit.
C. Refill prescriptions and schedule follow up in 3 months.
D. Provide education on healthy lifestyle changes and
follow up in 2 weeks.
E. Refer for dental work and Pap smear under general
anesthesia.
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GNRS5 Teaching Slides Editor:
Barbara Resnick, PhD, CRNP, FAAN, FAANP, AGSF
GNRS5 Teaching Slides modified from GRS9 Teaching Slides
based on chapter by Elizabeth Galik, PhD, CRNP
and Andrew Warren, MB, BS, DPhil
and questions by Rebecca Wysoske, MD
Managing Editor:
Andrea N. Sherman, MS
Copyright © 2016 American Geriatrics Society