Download GNRS4IntellectualDevtDisabilities

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INTELLECTUAL AND
DEVELOPMENTAL
DISABILITES
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OBJECTIVES
Know and understand:
• The definition of intellectual disability (ID) and
developmental disability (DD) and its prevalence among
older adults
• How to recognize and manage psychiatric, mental, and
behavioral disorders in older adults with ID and DD
• Ways to overcome barriers to diagnosis and treatment
• Common comorbidities found in individuals who have a
DD, with or without ID
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TO P I C S C O V E R E D
• Nomenclature
• Prevalence
• 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:
 IQ of ~70 or below based on formal test results
and
 Impairment in adaptive functioning before age 18
• 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” (intellectual developmental
disorder) in the DSM-5TM
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PREVALENCE OF ID
• Life expectancy for people with ID has increased
substantially
 1930: Average age of death 15 yr for males,
22 yr for females
 1990s: >40% live to at least age 60
• Prevalence currently 1%–2% in the US
 For people 60 yr, this number expected to double by
2030
PSYCHIATRIC AND MENTAL
DISORDERS IN ADULTS WITH ID
• Adults with ID have similar risk factors for mental illnesses
as their “normal” peers, but they may have additional risks
• Adults who were raised in institutions or did not benefit
from modern medical care are at greater than normal risk
• The prevalence of psychiatric disorders among adults with
ID is about 5 times that of age-matched controls (10%–
40%)
• The occurrence and severity of psychiatric disturbances
vary with age and comorbidities
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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 selfinjuring blows to the head from coup/contracoup
effects
Percent of people with Down syndrome
INCIDENCE OF DEMENTIA IN
ADULTS WITH DOWN SYNDROME
80
75
70
60
50
40
40
30
20
10
0
50 yr
Incidence of dementia
60 yr
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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
• Case reports have documented tolerability of
cholinesterase inhibitors and the glutamate antagonist
memantine in people with ID
 There is no evidence of efficacy in this population
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DIFFICULTIES WITH
ADAPTIVE BEHAVIORS
• Types of adaptive behaviors:
 Conceptual (eg, speaking, 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 behaviors
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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
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
 Consult collateral sources of information, such as family or
caregivers who can provide histories and other data
• Limited communication ability
 Receptive and expressive abilities can be comparable or
quite different
 Differences in these abilities is a characteristic feature of
some conditions
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BARRIERS TO DIAGNOSIS
AND TREATMENT (2 of 2)
• Diagnostic (aka comorbid) overshadowing
 The presence of ID itself makes it difficult to
diagnose and treat mental illness or challenging
behaviors
 Diagnostic overshadowing is a barrier to critical
thinking, and clinicians should remain aware of it
when presented with a difficult situation
• Diagnostic criteria are written for general population
 May need to adapt DSM-5TM and ICD criteria to
the level of ID for each diagnosis
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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
MALADAPTIVE BEHAVIORS IN ID
• The appropriate treatment or response might be
instructional or behavioral
 Preferred behavior programs reward good
behavior
• Pharmacologic intervention may be necessary for the
safety of the patient or those nearby
 Very few medications are approved for the most
common and challenging behaviors, and
prescribing medications off-label is common
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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
• Do not use second-generation antipsychotic medications
for sleep or “anxiety”
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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%
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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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
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 a DD 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
(intellectual
developmental
disorder)
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
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
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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
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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 diagnostic 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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QUESTION 1 (1 of 2)
Which statement is true of patients with Down syndrome and dementia?
A. Dementia rarely develops in people with Down syndrome because
of their shortened life span.
B. People with Down syndrome have beta-amyloid plaques and
neurofibrillary tangles years before overt signs of dementia develop.
C. Most people with Down syndrome and dementia live well into their
sixties before they die of dementia.
D. The finding of abnormal telomeres on the chromosomes of people
with Down syndrome can now be used to diagnose Alzheimer
disease years before there are symptoms.
E. Palliative medications used for people with Alzheimer disease are
appropriate for people with Down syndrome and dementia.
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QUESTION 1 (2 of 2)
Which statement is true of patients with Down syndrome and dementia?
A. Dementia rarely develops in people with Down syndrome because
of their shortened life span.
B. People with Down syndrome have beta-amyloid plaques and
neurofibrillary tangles years before overt signs of dementia develop.
C. Most people with Down syndrome and dementia live well into their
sixties before they die of dementia.
D. The finding of abnormal telomeres on the chromosomes of people
with Down syndrome can now be used to diagnose Alzheimer
disease years before there are symptoms.
E. Palliative medications used for people with Alzheimer disease are
appropriate for people with Down syndrome and dementia.
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CASE 1 (1 of 4)
• A 72-year-old man is brought to the ED at midnight by
his residential caregivers because over the past 3 days
he has had decreased sleep, increasing confusion, and
loss of some daily living skills for which he formerly
needed only minor prompts.
• History includes nonverbal, nonsigning autism and a
seizure disorder that is controlled by medication; he has
a distant history of self-injurious behavior. He is typically
modestly responsive to verbal prompts.
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CASE 1 (2 of 4)
• The caregivers report that as his sleeplessness
increased, his behaviors worsened, and he has resumed
self-injury, primarily hitting his ears with increased
frequency and intensity. He has had no change in
appetite.
• A brief but thorough review by the residence’s behavioral
specialist identified no specific antecedent trigger or
reward for these behaviors.
• The patient appears thin and frail. He sits silently rocking
and hitting his ears or sometimes his forehead.
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CASE 1 (3 of 4)
Which of the following is the most appropriate next step?
A.
Admit to the psychiatry inpatient service for medication
management.
B.
Request neuropsychological testing.
C.
Prescribe intramuscular haloperidol and lorazepam.
D.
Ask the caregivers to calm him, and request additional help to
perform physical exam and obtain blood samples.
E.
Prescribe quetiapine 50 mg and reevaluate in the morning.
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CASE 1 (4 of 4)
Which of the following is the most appropriate next step?
A.
Admit to the psychiatry inpatient service for medication
management.
B.
Request neuropsychological testing.
C.
Prescribe intramuscular haloperidol and lorazepam.
D.
Ask the caregivers to calm him, and request additional help to
perform physical exam and obtain blood samples.
E.
Prescribe quetiapine 50 mg and reevaluate in the morning.
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GNRS4 Teaching Slides Editor:
Barbara Resnick, PhD, CRNP, FAAN, FAANP, AGSF
GNRS4 Teaching Slides modified from GRS8 Teaching Slides
based on chapter and questions by Mark H. Fleisher, MD
Managing Editor:
Andrea N. Sherman, MS
Copyright © 2014 American Geriatrics Society