Survey
* Your assessment is very important for improving the workof artificial intelligence, which forms the content of this project
* Your assessment is very important for improving the workof artificial intelligence, which forms the content of this project
1 MENTAL RETARDATION/ INTELLECTUAL DISABILITY AND DEVELOPMENTAL DISABILITES 2 OBJECTIVES Know and understand: • The definition of mental retardation (MR) / intellectual disability (ID) and its prevalence among older adults • How to recognize and manage psychiatric, mental, and behavioral disorders in older adults with MR / ID • Ways to overcome barriers to diagnosis and treatment • Common comorbidities found in individuals who have a developmental disability (DD), with or without MR / ID 3 TO P I C S C O V E R E D • Nomenclature • Prevalence • Psychiatric and Mental Disorders in Aging Adults with MR / ID • Medical Disorders • Social Conditions • Developmental Disabilities and Comorbidity 4 NOMENCLATURE • Definition of MR / 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 MR / ID • This slide set focuses on individuals with MR / ID who may or may not have a comorbidity such as cerebral palsy, epilepsy, or autism spectrum disorder • The term “mental retardation” will be replaced with “intellectual disability” in the upcoming DSM V 5 PREVALENCE OF MR / ID • Life expectancy for people with MR / 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 MR / ID • Adults with MR / 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 MR / ID is about 5 times that of age-matched controls (10%–40%) • The occurrence and severity of psychiatric disturbances vary with age and comorbidities 6 7 DEMENTIA AND MR / ID • Individuals with MR / 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 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 8 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 9 10 DIAGNOSIS AND TREATMENT OF DEMENTIA IN PEOPLE WITH MR / 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 MR / ID There is no evidence of efficacy in this population 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 MR / 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 MR / ID can lose or become less adept with some adaptive behaviors 11 12 MALADAPTIVE BEHAVIORS • Examples: withdrawal, self-injury, stereotypy • Severe or frequent in up to 50%–60% of adults with MR / 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 MR / 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 13 BARRIERS TO DIAGNOSIS AND TREATMENT (2 of 2) • Diagnostic (aka comorbid) overshadowing The presence of MR / 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 IV-TR and ICD-9 criteria to the level of MR / ID for each diagnosis 14 DIAGNOSIS OF MENTAL DISORDERS IN OLDER ADULTS WITH MR / 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 15 TREATMENT OF MALADAPTIVE BEHAVIORS IN MR / 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 16 PRINCIPLES OF MEDICATION MANAGEMENT • Treating major mental illness in older adults with MR / 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” 17 18 MEDICAL DISORDERS • Adults with MR / ID have more medical problems than age-matched individuals About 5 medical conditions per person People with severe MR / ID have even more About 2/3 of community-dwelling people with MR / ID have chronic conditions or major physical disability, 50% of which go undetected • Visual or hearing impairments are particularly common in people with MR / ID They increase with age and affect about 25% PROBLEM BEHAVIOR CAN SIGNAL PHYSICAL ILLNESS IN ADULTS WITH MR / ID • 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 19 20 LIFE EXPECTANCY • Life expectancy for adults with MR / ID is ~65 yr Decreases with increasing severity of MR / 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) 21 SOCIAL CONDITIONS • At least 80% of adults with MR / 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) System/condition Change with developmental disabilities Management strategies Mental retardation/ Intellectual disability Two thirds of patients with DD suffer from MR / ID, many in the mild-to-moderate 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 22 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 23 DEVELOPMENTAL DISABILITIES AND COMORBIDITY (3 of 5) System/condition Change with developmental disabilities 24 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 25 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 26 27 S U M M A RY ( 1 o f 2 ) • An increasing number of individuals with MR / 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 MR / 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. 28 S U M M A RY ( 2 o f 2 ) • In individuals with MR / 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 MR / 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 MR / ID. However, these conditions can contribute to maladaptive behaviors and affect an individual’s quality of life. 29 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. 30 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. 31 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. 32 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. 33 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. 34 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. 35 GRS Slides Editor: Annette Medina-Walpole, MD, AGSF GRS8 Chapter Author: Mark H. Fleisher, MD GRS8 Question Writer: Mark H. Fleisher, MD Medical Writers: Beverly A. Caley Faith Reidenbach Managing Editor: Andrea N. Sherman, MS Copyright © 2013 American Geriatrics Society SlideSlide 35 35