1 INTELLECTUAL AND DEVELOPMENTAL DISABILITESSuggestions for Lecturer -1-hour lecture -Use GNRS slides alone or to supplement own teaching materials. -Refer to GNRS for further content and for strength of evidence (SOE) levels. -Refer to Geriatrics At Your Fingertips for updated information on patient evaluation and management. -Supplement lecture with handouts. -The GNRS Teaching Slides reflect care that can be provided to older adults in all settings. The words patient, resident, and older adult have been used interchangeably, as have the words provider, clinician, and primary care provider. Given the continually ongoing changes in health care today, some of the guidelines around reimbursement may have changed since publication. -Please note that the term “intellectual disability” has replaced the term “mental retardation” in the DSM-5.

2 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 Topic

3 TOPICS COVERED Nomenclature Prevalence Diagnostic and Treatment IssuesPsychiatric and Mental Disorders in Aging Adults with ID Medical Disorders Social Conditions Developmental Disabilities and Comorbidity Topic

4 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 Intellectual disability, as used in the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition (DSM-5), is defined as deficits in intellectual abilities that impact adaptive functioning in three areas: 1) conceptual skills, such as reading, writing, math, reasoning, and knowledge; 2) social and interpersonal skills, such as empathy, communication, and maintaining friendships; and 3) self-management skills, including personal care, recreation, and job skills. Cognitive processes such as attention and memory remain intact. Intellectual disability does not have a specific age requirement; however, the symptoms must occur during the developmental period and result in an inability to meet standards for personal independence. Diagnosis of an intellectual disability is based on the severity of the intellectual impairment and its impact on adaptive function and support needed. While the DSM-5 no longer considers specific IQ scores in the grading of symptom severity, an IQ of approximately 70 or below based on formal testing is suggestive of an intellectual disability. Topic Slide 4

5 Life expectancy for people with ID has increased substantiallyPREVALENCE 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 The number of individuals with intellectual disability surviving into old age is increasing because of generally better health care overall, including earlier detection and treatment of some conditions. It is difficult to quantify the prevalence of older adults with intellectual disability because of methodologic considerations and heterogeneity of conditions; however, it is estimated that intellectual disability has a worldwide prevalence of approximately 1%. Prevalence rates vary by age and by severity of intellectual disability. Individuals with severe intellectual disability are estimated at 6 per 1,000. It is even more problematic to consider the issues from the perspectives of other cultures and standards in areas other than Europe and North America. More recently, there are studies from Australia, New Zealand, China, Taiwan, and Israel; however, there is very little information about prevalence, morbidity, and mortality of people with intellectual disability living in developing countries. In general, longevity decreases with severity of intellectual impairment, certain comorbid conditions (eg, seizure disorders, Down syndrome), and the general health of the population and socioeconomic status of the country or culture. Topic Slide 5

6 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 The literature on older adults with intellectual disability and mental illness (other than dementia) has been relatively sparse over the last few decades. This makes it even more problematic to determine accurate numbers for several reasons: definitions have changed (ie, through various editions of the DSM and various versions of the International Classification of Diseases), health care has improved, studies have been conducted in different countries or regions, standard methodologies are lacking, and the relative numbers of institutionalized versus community-based individuals have changed. There are many reports of greater than expected rates of certain mental illnesses or behavioral disorders associated with specific physical illnesses or genetic disorders. For example, older adults with autistic spectrum disorders exhibit higher rates of compulsive behaviors requiring psychiatric treatment. Topic Slide 6

7 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 Clinicians face many diagnostic and treatment challenges when seeing patients with intellectual disability with or without comorbid issues of behavioral symptoms or mental illness. The best treatment requires an accurate, or at least the most likely, diagnosis, and that requires obtaining the best history. Unfortunately, all too often the patient’s history and his or her subjective reporting are limited or unavailable. The availability of a family member or caregiver who knows the individual well is extremely valuable when making a diagnosis and developing an effective treatment plan. Barriers to communication can exist for both clinician and patient. Under these circumstances, the clinician should try to be as effective as possible by recognizing the limitations of the patient. For the purposes of this discussion, these limitations are grouped into three broad categories: self-awareness, communication abilities, and diagnostic overshadowing. Topic Slide 7

8 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 Topic Slide 8

9 PSYCHIATRIC AND MENTAL DISORDERS IN AGING ADULTS WITH IDThe 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 In a study of individuals with Down syndrome who subsequently developed Alzheimer disease, these individuals were far more likely to suffer from psychologic and behavioral symptoms with more rapid decline in functional status than those who did not develop dementia. Some symptoms may improve as the patient ages or develops comorbid problems. In adults with Down syndrome, it is not uncommon to have complaints of significant obsessive and compulsive symptoms. This can be the primary focus of concern from young adulthood through the fifth or sixth decade of life or until symptoms of dementia become evident. As the dementia worsens, the anxieties of obsessions and compulsions can wane, and as memory function worsens, those symptoms are often lost or of little concern. It is also true that some behaviors or conditions can worsen with age through various processes. Individuals with intellectual disability experience the same disorders of aging as others but possibly with reduced coping mechanisms. For example, they may be more affected by chronic pain conditions or by vision or hearing loss. Topic Slide 9

10 PROBLEM BEHAVIOR CAN SIGNAL PHYSICAL ILLNESS IN ADULTS WITH IDIn 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 Self-injurious behavior to the ears can be a sign of otitis externa or otitis media. Self-injurious behavior to the eyes can be a clue to vision loss or changes. Delaying attention for a treatable vision condition, eg, presbyopia, can cause permanent loss through self-injury, such as a detached retina or corneal scarring. Topic Slide 10

11 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 Topic Slide 11

12 INCIDENCE OF DEMENTIA IN ADULTS WITH DOWN SYNDROMEThere 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 Topic Slide 12

13 LIFE EXPECTANCY OF ADULTS WITH DEMENTIA AND DOWN SYNDROMEMedian age of death, yr The figures on this slide come from data on nearly 18,000 individuals with Down syndrome, compiled by the Centers for Disease Control and Prevention. Based on data from death certificates, standardized mortality odds ratios (SMOR) of people with Down syndrome were more likely to show congenital heart defects (SMOR=29.1), dementia (SMOR=21.2), hypothyroidism (SMOR=20.3), or leukemia (SMOR=1.6) than those of people without Down syndrome. Apart from leukemia and testicular cancer, the risk of other malignant diseases was low in people with Down syndrome. Most individuals with Down syndrome and dementia now die during the sixth decade of life. Topic Slide 13

14 DIAGNOSIS AND TREATMENT OF DEMENTIA IN PEOPLE WITH IDDementia 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) Interest has been growing in attempting to demonstrate the efficacy of medications such as cholinesterase inhibitors (donepezil, rivastigmine, galantamine) and the glutamate antagonist memantine in individuals with intellectual disability. One randomized trial showed minimal benefit with the use of donepezilOL for patients with Down syndrome who developed progressive dementia (SOE=C). Another study failed to demonstrate any benefit from use of memantine in a placebo-controlled randomized trial of patients with dementia secondary to Down syndrome (SOE=A). It may be that Alzheimer disease in Down syndrome is diagnosed relatively later in disease progression than in non-Down syndrome patients with Alzheimer disease, and potential benefits may be attenuated. Topic Slide 14

15 ADAPTIVE BEHAVIORAL DIFFICULTIESTypes 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 Adaptive behaviors are learned social and practical skills concerned with daily functions. Limitations in these skills have a negative impact on people’s lives; however, these skill abilities are not fixed in place or easily assigned to a particular level of ID. Topic Slide 15

16 MALADAPTIVE BEHAVIORSExamples: 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 Maladaptive behaviors are observable phenomena that are counterproductive or disruptive for the individual. Various other terms are sometimes used to describe these acts, including target behaviors (behaviors targeted for extinction) or challenging behaviors. Topic Slide 16

17 DIAGNOSIS OF MENTAL DISORDERS IN OLDER ADULTS WITH IDFollow 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 It is important not to overdiagnose and therefore overtreat an individual’s presentation. Because insight, judgment, and adaptive or coping skills are limited, individuals may be more likely to “act out,” which may be incorrectly perceived as a serious symptom of illness when, in fact, it may just be frustration. Medication may not be called for in a situation in which supportive therapy and time could lead to resolution. Medication can be used, however, to create a window of opportunity to make behavioral supports or strategies more effective. A change in staff, residential or vocational setting, or family health should be reported and considered as a precipitating factor for all behavioral changes. The concepts of applied behavioral analysis are important tools to use in determining cause and effect of problem behaviors. Topic Slide 17

18 TREATMENT OF MENTAL DISORDERS IN OLDER ADULTS WITH IDThe 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 Topic Slide 18

19 PRINCIPLES OF MEDICATION MANAGEMENTTreating 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 It is beyond the scope of this slide set to discuss the various treatment options for such a diverse patient group. Autism spectrum disorders probably represent the largest diagnostic group among aging individuals for whom medication management is common and difficult. Self-injurious behaviors are certainly the most common reason medications are considered. Self-injurious behaviors include several potentially life-threatening behaviors that can cause damage to the brain, eyes, and ears, as well as the potential for systemic infections. Topic Slide 19

20 MEDICAL DISORDERS Adults with ID have more medical problems than age-matched 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% Topic Slide 20

21 LIFE EXPECTANCY Life expectancy for adults with ID is ~65 yrDecreases 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) Topic Slide 21

22 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 Not surprisingly, the degree of ID, physical health, and functional skills of the aging individual correlate with the degree of parental stress and burden, although maternal and family characteristics such as education and income are more correlated with overall life satisfaction and maternal well-being. Individuals with intellectual disability are now more likely to outlive their family caregivers, and caregiver succession planning becomes important for the clinician to address with the patient and the family before a crisis. Topic Slide 22

23 DEVELOPMENTAL DISABILITIES AND COMORBIDITY (1 of 5)System/condition Change with developmental disabilities Management strategies Intellectual disability Two thirds of patients with DD suffer from 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 Some developmental disabilities may not cause an intellectual disability but nonetheless can contribute to other morbidities and challenging behaviors, resulting in reduced quality of life. Among these disabilities are cerebral palsy, seizure disorders, and a host of genetic disorders too numerous to mention. Some genetic disorders have significant variability in their impact on cognitive functioning; others once thought to affect only a single generation or gender are now thought to have broader implications, such as fragile X syndrome. In general, as the degree of cognitive impairment increases, the risk of morbidity due to physical causes increases. This slide and the next 4 slides show the approximate prevalence and severity of some common conditions found in individuals who have a developmental disability, with or without ID. Topic Slide 23

24 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 common among patients with muscle weakness and spasticity Monitoring of body habitus; physical therapy 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 Topic Slide 24

25 DEVELOPMENTAL DISABILITIES AND COMORBIDITY (3 of 5)System/condition Change with developmental disabilities 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 Topic Slide 25

26 DEVELOPMENTAL DISABILITIES AND COMORBIDITY (4 of 5)System/condition Change with developmental disabilities Management strategies Functional decline Aging patients with cerebral palsy and other similar conditions often develop fatigue, pain, weakness, and overuse syndromes that 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. Physical therapy, occupational therapy, pain management 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 Topic Slide 26

27 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 Topic Slide 27

28 SUMMARY (1 of 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. Topic

29 SUMMARY (2 of 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. Topic

30 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. Topic

31 CASE 1 (2 of 3) Which one of the following is the best initial strategy for treating the behavioral disturbance? Provide support and behavioral interventions at residence and workshop. Provide support and behavioral interventions at residence, and stop workshop attendance until behavior improves. Start risperidoneOL 0.25 mg/d. Admit to inpatient psychiatric unit for observation and treatment. Topic

32 CASE 1 (3 of 3) Which one of the following is the best initial strategy for treating the behavioral disturbance? Provide support and behavioral interventions at residence and workshop. Provide support and behavioral interventions at residence, and stop workshop attendance until behavior improves. Start risperidoneOL 0.25 mg/d. Admit to inpatient psychiatric unit for observation and treatment. ANSWER: A The most appropriate treatment for this patient is emotional support and behavioral interventions at both his residence and workshop. The circumstances suggest possible triggers for the change in his behavior. He is accompanied by a new staff member who does not know him well, and his mother broke her hip and is in a nursing unit while she recuperates. Patients with intellectual disability have fewer coping strategies; they are often unable to express their emotions adequately and seek support. When caregivers assess behavior, they are more likely to attribute aggressive behavior to anger, whereas patients are more likely to describe anxiety or depression. The patient reports being sad, perhaps in response to concern about his mother and new staff (who may have replaced a favorite caregiver). Keeping him from the workshop would not likely be useful, because the behaviors are occurring in all settings. He may perceive being kept home as punishment, which may further exacerbate his behaviors. Strategies based on positive reinforcement are the standard in treating patients with intellectual disability. The new behavior began only a few days before, and there have been no serious injuries, so medication as an initial treatment is not necessary. If the behavior is extreme, short-term use of medication may be useful to attenuate symptoms while behavioral strategies are initiated. Studies of various medications, including antipsychotics, naltrexone, and clomipramine, have had mixed results in terms of benefit, and the drugs have potential for significant adverse effects. Inpatient admission is appropriate for patients who are at great risk of harm to themselves or others. It is not indicated in this case, because the patient has not caused serious harm to himself and does not have a history of aggression toward others. Inpatient admission could exacerbate symptoms, because patients with intellectual disability are sensitive to changes in their environment. Topic

33 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. Topic

34 CASE 2 (2 of 3) Which one of the following is the best tool for assessing whether this patient may have dementia? Functional Assessment Staging Test (FAST) Dementia Screening Questionnaire for Individuals with Intellectual Disabilities (DSQIID) Down Syndrome Mental Status Examination (DSMSE) Mini–Mental Status Examination (MMSE) Montreal Cognitive Assessment (MoCA) Topic Slide 34

35 CASE 2 (3 of 3) Which one of the following is the best tool for assessing whether this patient may have dementia? Functional Assessment Staging Test (FAST) Dementia Screening Questionnaire for Individuals with Intellectual Disabilities (DSQIID) Down Syndrome Mental Status Examination (DSMSE) Mini–Mental Status Examination (MMSE) Montreal Cognitive Assessment (MoCA) ANSWER: B Diagnosing dementia in any patient is challenging, even more so in patients with intellectual disabilities. Because of the underlying cognitive deficits, the earliest signs often go unnoticed, and dementia tends to be diagnosed later in the disease process. The DSQIID has good sensitivity, specificity, and inter-rater reliability. It takes 10–15 minutes to administer, so it could be done easily during an office visit. Because of its single cut-off score, it may not be useful in cases of more advanced dementia. The risk of Alzheimer disease is significantly increased in patients with Down syndrome: up to 75% of patients are affected by age 60. The time between diagnosis and death is shorter than in other populations and is likely multifactorial: diagnosis at later stage, lower premorbid functioning, and perhaps other factors as yet not clearly defined. Nearly all patients with Down syndrome have the plaques and tangles of Alzheimer disease by age 40, although they do not manifest clinical symptoms that early. These changes are not seen in patients with intellectual disability of other etiologies, suggesting a strong pathophysiologic contribution. Because of the baseline cognitive deficits, the usual screening tests for dementia (MMSE, MoCA) are generally of little use in patients with Down syndrome or other intellectual disabilities. Many tests depend on caregiver observations rather than on direct testing of the patient. The Alzheimer FAST is a caregiver observation tool that can be used to assess progression of disease or response to treatment; it is not considered a screening tool. The DSMSE is administered to the patient. It relies heavily on verbal skills, has a floor effect, and is less sensitive than other tests. Topic Slide 35

36 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. Topic

37 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. Topic

38 CASE 3 (3 of 4) Which one of the following is the best next step in providing care for this patient? Order mammography and refer to a gynecologist for Pap smear. Have blood drawn for medication levels at end of visit. Refill prescriptions and schedule follow up in 3 months. Provide education on healthy lifestyle changes and follow up in 2 weeks. Refer for dental work and Pap smear under general anesthesia. Topic Slide 38

39 CASE 3 (4 of 4) Which one of the following is the best next step in providing care for this patient? Order mammography and refer to a gynecologist for Pap smear. Have blood drawn for medication levels at end of visit. Refill prescriptions and schedule follow up in 3 months. Provide education on healthy lifestyle changes and follow up in 2 weeks. Refer for dental work and Pap smear under general anesthesia. ANSWER: D Studies confirm that providing education on diet and exercise to the family and patient can improve lifestyle choices. Scheduling a follow-up appointment soon after the initial visit would allow the provider to develop a rapport and begin to address other health concerns without overwhelming the patient (and family) at the initial visit. An eye examination, tailored to the patient’s intellectual ability, should be attempted at a subsequent visit. Although patients with intellectual and developmental disabilities live longer than in the past, their lifespan remains shorter than that of age-matched controls without comparable disabilities. Overall life expectancy is 65 years, varying somewhat depending on the specific diagnosis. Illnesses typically related to aging (eg, heart disease, diabetes, arthritis, dementia) develop earlier in patients who have an intellectual disability or chronic physical symptoms associated with developmental disability (eg, seizure disorders, cerebral palsy, Down syndrome and other genetic abnormalities). The reasons for this include poorer access to health care because of both limited financial resources and lack of providers experienced with this population; poor diet, again because of finances or to lack of knowledge regarding healthy eating habits; more sedentary life style because of physical limitations (including vision problems), social limitations, and knowledge deficits; and physical manifestations of the underlying disorder (eg, cerebral palsy, muscular dystrophies). A patient who refuses to undress for a physical examination is unlikely to cooperate for mammography or Pap smear without being prepared about what the examinations entail. Referring the patient to another new provider may further increase anxiety without increasing the likelihood of success. Working with the patient over time and explaining the procedures in simple terms, with demonstrations when possible, may allow important screening tests to be done at a future date. In this reluctant patient, whose parents promised “no shots,” attempts to draw blood may interfere with efforts to establish a relationship. Parents should be asked to not make such a promise in the future. If the patient has had blood drawn in the past with little difficulty, laboratory orders for drug levels and routine screening can be ordered for a future date, perhaps at a laboratory in a separate location. Refilling the patient’s prescriptions does not address the need for health screening or the current history of ongoing seizures. A 3-month delay in follow-up would undermine the provider’s efforts to develop rapport with the patient and family, which will be beneficial for ongoing treatment. It is sometimes necessary to do procedures while the patient is under anesthesia, especially if the degree of intellectual disability is more severe than in this patient. However, every effort should be made to perform procedures in the usual manner. If it is necessary to do a procedure while the patient is under anesthesia, he or she should be informed beforehand, at the level of his or her ability to understand. Topic Slide 39

40 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