8/20/18. Disclosures. Objec&ves. Alzheimer s Disease. Alzheimer's Disease in People with Intellectual Disabili8es IHCA Convention and Expo

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1 Disclosures 2 No financial disclosures Alzheimer's Disease in People with Intellectual Disabili8es IHCA Convention and Expo September 11, 2018 Brian Chicoine, MD, Medical Director, Adult Down Syndrome Center Objec&ves Understand the diagnosis of Alzheimer's disease in individuals with intellectual disabilities. Be able to use strategies, including environmental changes, to optimize function in the individual's home setting. Stimulate discussion regarding adjustment in goals of care and the effect of the changes in the individual has on those who live with him/her and/or care for him/her 3 Adult Down Syndrome Center Alzheimer s Disease What is the link between dementia and Alzheimer s disease? In theory can affect anyone with an intellectual disability Why is there a focus on people with Down syndrome? 6 1

2 Life Expectancy Life expectancy: 1907: 9 years 1984: 28 years Today: 60 years One of the oldest well-documented individuals with Down syndrome, 83 years old at death Chicoine, B. & McGuire, D. (1997) Longevity of a woman with Down syndrome: a case study. Mental Retardation 35(6): Older Adults Differences in health condi3ons and age of onset Osteoarthri3s and Cerebral palsy Alzheimer disease and Down syndrome Younger More common Behavioral changes Seizures What is Alzheimer Disease? What is the Association between Down syndrome and Alzheimer Disease? Progressive neurological condition Affects the brain Is a type of dementia Microscopically, the characteristic changes of the brain are plaques and tangles Nearly all people with Down syndrome have plaques and tangles by age 40. All people with DS over age 60 Neuropathological Changes of Alzheimer s Disease in a Person with Down s syndrome DMA Mann In The Neuropathology of DemenIa MM Esiri and JH Morris University Press: Cambridge 1997 Alzheimer s demen.a: Key diagnos.c issues In our prac*ce Memory deterioration Loss of previously mastered skills Incontinence Gait apraxia Dysphagia (swallowing problems) Seizures (77% vs 2%) Weight loss Withdrawal, apathy and/or other psychological or personality changes Average age of onset 52.3 years Average age of death years Average from onset to death- 3.6years (compare to approximately 5.9 years) Cumulative survival Age Women with Down syndrome Women without Down s yndrome Cumulative survival rates for average-risk women (without Down syndrome) and women with Down syndrome in the absence of breast cancer-related deaths Glasson E, Sullivan S, Hussain R, Petterson B, Montgomery P, Bittles A. The changing survival profile of people with Down's syndrome: implications for genetic counselling. Clinical genetics. 2002;62(5):

3 What if I suspect Alzheimer Disease? Look for a pa8ern of decline Rule-out other poten=al causes Is it Alzheimer Disease: Approach to a person with Decline in skills History and physical Medication review Mental Health/Psychosocial evaluation Labs: Thyroid, vitamin B12, Chemistry panel, Celiac Xrays: Lateral cervical spine, CT/MRI of brain Sleep study(?) The Interac+on Between Physical and Mental Health Any and all behavior change should be viewed as a possible communication tool Pain Pain tolerance/reduced responsiveness Pain perception Delayed Localization Higher tolerance but greater intensity at some point and affected by other factors Martínez-Cue, Carmen; Baamonde, Carmela. A murine model for Down syndrome shows reduced responsiveness to pain. NeuroReport, Vol 10, Issue 5, April 1999, Hennequin, M et al. Pain expression and stimulus localization in individuals with Down's syndrome. The Lancet, Volume 356, Issue 9245, Dec 2000, Nanda C. de Knegt, Frank Lobbezoo, Carlo Schuengel, Heleen M. Evenhuis, Erik J.A. Scherder; Self-Reported Presence and Experience of Pain in Adults with Down Syndrome, Pain Medicine, Volume 18, Issue 7, 1 July 2017, Pages , More Common Health Condi0ons Hearing impairment Vision impairment, keratoconus, cataracts Dysphagia (swallowing) Sleep apnea Infections Skin Lung/Respiratory More Common Health Conditions Continued Gastrointestinal Gastroesophageal reflux Celiac Constipation Hernias Urinary Retention Undescended testicle Orthopedics Atlanto-axial instability Osteoarthritis 3

4 More common, con*nued Cancer Leukemia Testicular cancer Autoimmune Mental Health and Neurologic Depression Obsessive Compulsive Disorder Alzheimer s disease Autism Regression Conditions that are Less Common Hypertension Atherosclerotic disease Cancer, solid tumors Who does Alzheimer Disease Effect? The pa7ent The family The health care team The direct care personnel 22 Treatment Cholinesterase inhibitor (donepezil/aricept, galantamine/razadyne, etc.) Meman>ne (Namenda) Hanney et al. Meman>ne for demen>a in adults older than 40 years with Down's syndrome (MEADOWS): a randomised, double-blind, placebo-controlled trial Lancet Feb 11;379(9815): Symptom Evaluation and Treatment Sleep Melatonin, Trazodone Urinary incontinence Assess for UTI, urinary retention, and overactive bladder Gait changes Arthritis, cervical subluxation (AAI) 4

5 Symptom Evalua-on and Treatment Mood changes Assess for pain, environmental s-muli, sleep Depression Selec-ve serotonin reuptake inhibitors (eg sertraline/zolob; citalopram/celexa) Serotonin and norepineprhine reuptake inhibitor (eg duloxe-ne/cymbalta) Symptom Evalua-on and Treatment Aggressive behavior Assess for pain, sleep, environment Fear Seizure medications Anti-anxiety medications (eg lorazepam/ativan, clonazepam/klonopin) Anti-psychotics (eg risperidone/risperdal) Symptom Evaluation and Treatment Seizures Myoclonic jerking An;-seizure medica;ons leve;racetam/keppra Doing what can s8ll be done Friends and family Physical exercise Social engagement Crea8ve arts Sensory s8mula8on Bingo Pace Provide best quality of life Down Syndrome Scotland Publica4on 29 Na#onal Down Syndrome Society Publica#on 30 5

6 Challenging behaviors Caregiver triggers (e.g. reasoning) Physical triggers (e.g. pain) Environmental triggers (e.g. noise) Communica)on skills Eliminate background noise Talk one-on-one Make direct eye contact (level of the person) Don t correct or argue Take )me to listen to words and tone Validate feelings Slow down words and ac)ons Communica)on skills, cont. Resources Short simple sentences Cues and gestures Limit choices Break down tasks Use gentle touch Web site h*ps:// (or search for Adult Down Syndrome Center) Facebook h*ps:// Contact Laura Chicoine, Project Manager, Research and EducaLon Laura.Chicoine@advocatehealth.com