1. Dementia with a Focus on Older Adults with Down Syndrome Presented By: Ronald Lucchino, PhD rvluc@comcast.net

2. Purpose Develop observational skills of the changes related to dementia, specifically Alzheimer’s disease Understand how Alzheimer's disease (AD) in the older adult with Down syndrome increases the risk of Ambulatory Care Sensitive Conditions (ACSC) and hospitalization Understand how this risk can be reduced by implementing management strategies

3. Alzheimer’s disease and adults with disabilities Increased risk of Alzheimer’s disease in the general population (an age-associated condition) due to aging The general older adult with I/DD has the same rate and changes as the general population The exception is Down syndrome; there is a greater risk of Alzheimer’s disease Caregivers, staff, and health and social care practitioners are not prepared to deal with dementia in the older adult with Down syndrome

4. What is Dementia?

5. Dementia – “loss of mind” There is no such thing as “just a little senile.” Forgetfulness may accompany normal aging. Many older healthy adults have reduced concentration, difficulty recalling names and remembering where things were left. When memory loss and confusion begin to interfere with daily living it is not a part of normal aging but due to age-associated changes or diseases.

6. Dementia (cont’d) Dementia is defined as: a) a state of impaired short- term memory, b) loss of cognitive abilities, and c) changes in personality/behavior which are persistent and interfere with levels of social activities, routine activities of daily living, and/or occupational function. The clustering of behavioral changes, memory loss and decline in cognitive abilities defines the type of dementia but does not imply causation or prognosis.

7. Examples: Personality / Behavior Changes Apathetic (voluntary/willful refusal to cooperate) Passive and withdrawn (reduced responsiveness) Reduced Cognition Skills Reduced handling of complex tasks (abstract thought, planning, problem solving) Reduced reasoning ability (poor judgment, inability to respond to problems) Reduced spatial and orientation ability (organizing objects, finding way in familiar surroundings) Reduced language skills (finding words, following conversation) Short term memory decline Store, retain, recall information (noticed early decline)

8. Two Categories of Dementia

9. Acute Dementia - Reversible Chronic Dementia - Irreversible –Chronic secondary dementia –Chronic primary dementia Non-Progressive – Vascular Progressive – Neurodegenerative

10. Overlapping Dementia An individual may have more than one dementia, making it harder to differentiate the types of dementia and intervention strategies. This should be noted for the aging population with I/DD (evidence indicates that older adults with Down syndrome may only suffer from Alzheimer’s with a very low incident of possible Lewy Bodies disease (LBD) dementia).

11. What is Acute Dementia?

12. Acute Dementia (reversible) Remove the cause and the symptoms disappear Causes: –delirium –depression –dehydration * –hypothyroidism * –illness, inflammation, or infection * –isolation (social) –lack of sleep * –low body temperature * –medication(s) * –poor nutrition –sensory deprivation * * indicates high risk for acute dementia in older adult with Down syndrome

13. Acute Dementia (cont’d) General I/DD populations are at risk for acute dementia, which may be confused with Alzheimer’s Disease (AD), increasing the difficulty in diagnosis and management (diagnostic over-shadowing). Older adults with Down syndrome are at a higher risk for acute dementia by having age- related and associated changes misdiagnosed as Alzheimer’s (diagnostic over-shadowing).

14. Chronic Secondary Dementia (Low Incidence in Adults with Down Syndrome)

15. Secondary condition to a primary disease Dementia symptoms may be unique to the primary disease Examples of primary diseases that have associated secondary dementias: –AIDS –Alcoholism –Diabetes (possible high risk for adults with Down syndrome) –Huntington’s disease –Parkinson’s disease –Syphilis

16. Primary Chronic Dementias

17. Types of Primary Chronic Dementia 60% 20% 15% 5%

18. Caused by multi-infarctions or mini-strokes in the brain; accumulation of stokes leads to progressive decline in cognitive, memory and personality function (not clear if this is an issue for the older adult with Down syndrome) Causes: Lifestyle/Diet –Hyperinsulinemia (high insulin levels), dyslipidemia (high levels of fat), hyperglycemia (high sugar levels), hypertension Effects –Mini-strokes (clot/hemorrhagic) in various areas of the brain –New symptoms with each new incident (20% + of all dementias) Can affect aging adults with I/DD due to poor lifestyle (diagnostic over-shadowing) Chronic Non-Progressive (Vascular) Dementia

19. Chronic Non-Progressive Example Mary, a 50-year-old adult with Down syndrome and mild I/DD, is independent with full time job. Her employer describes Mary’s work as good, but noticed that her speech is slightly slurred and she seems to have some problems finding words. He noticed this two days ago and it has not improved, nor has it worsened. Note that the change occurred within a short time (two days) but did not worsen. –Symptom of vascular dementia

20. Chronic Primary Neurodegenerative Pre-Senile Dementia

21. Prior to the age of 64 Frontal-temporal lobe diseases (Pick’s) –Not reported in the I/DD populations Dementia with Lewy Bodies disease (LBD) –Recent evidence suggests that LBD may occur in older adults with Down syndrome and may overlap with Alzheimer’s disease Creutzfeldt-Jakob dementia –Not reported in the I/DD populations

22. Chronic Primary Neurodegenerative Senile Dementia (Alzheimer’s Disease)

23. Alzheimer’s Disease: Risk Factors in the Adult with Down syndrome 21st chromosome - mutated gene on chromosome increases risks (mutated gene 100% in the Down syndrome population but only 60% diagnosed by age 60) Unknown if vascular diseases in adults with Down syndrome increases the risk of Alzheimer’s disease Acute dementia may increase the risk of mimicking, masking or exacerbating Alzheimer’s disease Symptoms may be misdiagnosed as Alzheimer's disease since other conditions, such as acute dementia, may mimic those symptoms (diagnostic over-shadowing)

24. Alzheimer’s Disease: Key Findings in Adults with Down syndrome Adults with Down syndrome comprise 10-12% of the population with I/DD but by age 60 account for 60% of Alzheimer’s Disease. Late onset seizures in about 12%

25. Age of Onset of Alzheimer’s Disease Average onset age about 53 for adults with Down syndrome – 65 for others 25% of adults with Down syndrome diagnosed with AD by ages 45-60 60 - 65% of adults with Down syndrome diagnosed between 60-70 and <5% over 80+ years

26. Duration of Alzheimer’s in Adults with Down syndrome compared to the I/DD and General Populations Adults with Down syndrome have a 5-8 year duration time for the disease from diagnosis to death The general and the mild to moderate populations with I/DD have a duration of AD from 5- 17+ years from diagnosis until death Average Years

27. Summary of differences between AD in adults with Down syndrome and general population Rate of Occurrence Much higher prevalence & neuropathology indicative of AD Onset and Duration Earlier onset and shorter duration Behavioral Changes Personality change and memory loss Neurological Signs Late onset seizures

28. Why the Increase in Alzheimer’s Disease? In the past, the younger adults with Down syndrome did not reach the upper age levels resulting in low incidents of Alzheimer’s Disease –With the increase of life expectancy into middle age and beyond, the morbidity rate is increasing No inevitability about the onset of the disease –Studies suggest that 40% of adults with Down syndrome will not develop clinical symptoms of Alzheimer's disease

29. Alzheimer’s Disease Example You notice that over the last six months there has been a slow change in Tom’s appearance and personality. He does not shave unless he is urged to do so and it appears his ability shave properly has declined. At first the changes were not really noticeable until you look back over time. Note that the changes were slow and not noticeable at first but noticed over time.

30. Alzheimer’s Disease: Specific Early Stage Changes in adults with Mild to Moderate Down syndrome (different than other aging adults with I/DD and general aging populations) Key Early Stage in order of change - Slow Onset (1-2 years) –Difficulty with or loss of speech, loss of ability to find words, repetition of words, questions, or phrases –Decline in activities of daily living skills (ADL) Key indicator in the early stage of Alzheimer’s –Some deterioration in work performance –Changes in personality Long periods of inactivity or apathy

31. Alzheimer’s Disease: Specific Early Stage Changes in adults with Mild to Moderate Down syndrome (cont’d) Loss of interest in favorite hobbies Memory loss is not the first symptom of AD in the older adults with Down syndrome but occurs later (in the general population it is the key indicator) Noticeable decline in learned skills Changes in mental processes – slowing of thinking, reasoning and judgment (increase disorientation and confusion)

32. Alzheimer’s Disease: Specific Mid-Stage Changes in adults with Mild to Moderate Down syndrome (cont’d) Severe change in personality Marked decline in long-term memory Marked decline in mental processes - such as thinking, reasoning and judgment

33. Alzheimer’s Diseases: Specific Late-Stage Behavioral Changes in Adults with Mild to Moderate Down syndrome Late-stage (1 year) –Loss of basic skills (eating or drinking) –Loss of ability to walk –Total bowel and urinary incontinence

34. Alzheimer’s Disease: Specific Behavioral Changes in the Older Adult with Severe to Profound Down Syndrome Changes may be very subtle and pass unnoticed because of non-verbal communication and the disabilities may mask symptoms of Alzheimer’s Disease –Social withdrawal, reduced responsiveness –Apathy/behavior - personality changes –Impaired attention

35. Interventions for Reducing Behavioral Issues

36. Age 60+ 0 Increasing skills before dementia Increasing skills before dementia Decline in skills after dementia Decline in skills after dementia + Change - Retrogressive Changes Earliest skills learned are the last to be lost conversely the newest skills are first to be lost.

37. Suggested Interventions: Early to Mid Stages of Alzheimer’s Disease Routine and familiarity is important –“age in place” Adaptations to environment or program Normal activities, but made simpler Structure and support to daily routines

38. Suggested Interventions: Early to Mid Stages of Alzheimer’s Disease (cont’d) More guidance or supervision Explicit simple directions, instructions or cues Build on skills with similar activities –Use knowledge and activities that were learned early

39. Enhanced Observation Skills

40. First incidents that lead to suspicion of dementia in group homes Wandering Trouble finding words Apathetic - uncooperative Falling Decline in general abilities Short-term memory loss Increased aggression Increased conflicts with peers 40

41. What to do when Dementia is Suspected

42. Use a screening instrument to look for signs, if it comes up positive, then: - Ask for an assessment to be done - By whom?... an Agency MD, local MD, psychologist, nurse, or other person who may do a formal assessment to validate suspicions If the assessment comes up positive, then: - Ask for a diagnostic work-up - Ask for confirmatory information and determination that it is dementia…

43. Challenges to Diagnosis and Care

44. Older Individuals with I/DD may not be able to report signs and symptoms (communication problems)

45. Subtle changes may not be observed

46. Older Individuals with I/DD may not be able to report signs and symptoms (communication problems) Subtle changes may not be observed Commonly used dementia assessment tools are not relevant for people with I/DD

47. Older Individuals with I/DD may not be able to report signs and symptoms (communication problems) Subtle changes may not be observed Commonly used dementia assessment tools are not relevant for people with I/DD Difficulty of measuring change from previous level of functioning

48. Older Individuals with I/DD may not be able to report signs and symptoms (communication problems) Subtle changes may not be observed Commonly used dementia assessment tools are not relevant for people with I/DD Difficulty of measuring change from previous level of functioning Conditions associated with I/DD maybe mistaken for symptoms of dementia (Diagnostic overshadowing)

49. Alzheimer’s Disease: Prognosis

50. Prevention –No intervention to prevent the onset or progression of AD –Exercising the brain and body may delay some of the memory and cognitive decline Treatment –No treatment available –Question if Aricept or Memantine medications may be effective in reducing memory loss Diagnosis –Presently there is no diagnostic procedure - only upon autopsy of the brain

51. Assessment for Alzheimer’s Disease

52. Document baseline adult function by age 35. By age 35, each individual's medical record should ideally include detailed information on his or her adult abilities and intellectual, social and behavioral function. Watch for changes in day-to-day function. Reduced enthusiasm for daily activities, loss of interest in social interactions, and changes in personality and behavior are often early signs of an underlying decline in thinking skills. Consider professional assessment by a dementia expert. A variety of cognitive tests have been used to evaluate thinking changes in adults with Down syndrome. Experts caution that cognitive tests should never be used as the only benchmark to diagnose dementia in a person with Down syndrome. Rule out other causes of symptoms. Thyroid problems, depression, chronic ear and sinus infections and sleep apnea can also cause changes in a person's thinking and functioning.

53. Adaptive Behavior Dementia Questionnaire (ABDQ) Assessment for Adults with Developmental Disabilities (AADS) Dementia Questionnaire for People with Learning Disabilities (DLD) Dementia Scale for Down Syndrome (DSDS) Dementia Screening Questionnaire for Individuals with Intellectual Disabilities (DSQIID) DMR – Pinter RAI-Intellectual Disability (Pinter RAI ID), assesses key domains of interest to service providers relative to a person with an intellectual disability (ID) Adaptive Behavior Dementia Questionnaire (ABDQ)

54. Usable by support staff and caregivers to note presence of key behaviors associated with dementia Picks up on health status, ADLs, behavior and function, memory, self-reported problems Available in several languages ‘NTG-Early Detection Screen for Dementia’ (NTG-EDSD) Pages ①② : Basic information Pages ③④ : Information about function and indicators of problem areas associated with dementia Page ⑤ : Coincident conditions Page ⑥ Medications & Comments

55. Intervention Strategies

56. 1. Develop a protocol to evaluate and document observed change 2. Develop an educational packet on Alzheimer’s dementia in older adult with Down syndrome to be distributed to the healthcare practitioners, hospital, and/or nursing home staff. 3. Develop a training program to educate formal and informal caregivers and older adults with I/DD regarding the prevention of acute dementia, and the symptoms of Alzheimer’s disease. 4. Develop an approach that includes the input of health concerns, and health care interventions from older adult with Down syndrome and general adults with I/DD. 5. Establish a base line evaluation protocol that include four activities in which the older adults with Down syndrome are involved and review it at specific intervals to determine changes. - Videotaping the individual is one of the most effective evaluation tools. The video can be shown to the professional healthcare practitioner documenting changes over time.