The Impact of Ageing & Dementia for People with Down Syndrome. Evelyn Reilly Clinical Nurse Specialist Dementia

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1 The Impact of Ageing & Dementia for People with Down Syndrome Evelyn Reilly Clinical Nurse Specialist Dementia

2 We need to support a rapidly expanding older population with Down syndrome.

3 Ageing & Down syndrome Ageing impacts on people with Down syndrome at an earlier age, and those over the age of 40-years are at high risk for developing complex health conditions.

4 Common Medical Issues Sensory Loss Hypothyroidism Obstructive Sleep Apnoea Osteoarthritis Osteoporosis Coeliac Disease Alzheimer s Disease

5 Alzheimer s Disease Alzheimer s disease is a type of dementia that gradually destroys brain cells, affecting a person s memory and their ability to learn, make judgements, communicate and carry out basic daily activities. It is characterised by a gradual decline that progresses through 3 Stages: early, middle and late stage disease.

6 Alzheimer s dementia and Down syndrome (DS) It has been universally agreed that the prevalence of Alzheimer s type dementia in person s with Down s syndrome exceeds that of the general population. 15% - 45% of people with D.S over the age of 40 years will be affected.

7 Why? Years 12 Years 18 Years 30 Years 21 st Century 60 Years & Beyond.

8 Age Specific Prevalence Rates Age Group Incidence years 0% years 3.4% years 7.5% years 21.4% years 48.4% > 60 years 70.5% (Schupf 2001)

9 Comparative Rates of Dementia Down s syndrome, I.D., General Population D S ID G P Holland 2011

10 DoC Memory Clinic: 189 Assessed 54 people (28.5%) had dementia 17 people (8.9%) had dementia queried 25 people (13.2%) were at high risk of developing dementia

11 The Research Journey

12 Trisomy 21 and Alzheimer s Disease Trisomy Trisomy 21 accounts for 95% of DS APP (amyloid) gene on Chromosome 21 Amyloid Cascade Hypothesis

13 Trisomy 21

14 Plaques and Tangles the Hallmarks of Alzheimer Disease PLAQUES are dense, insoluble deposits of protein, found surrounding the neurons In A.D. Plaques develop in the Hippocampus (memory) TANGLES A special kind of protein, Tau, makes the communication system throughout the brain stable In A.D, Tau is changed chemically which will lead to the collapse of the neurons transport system

15 Neurons

16 Alzheimer s Disease

17 The use of Advanced Neuroimaging MRI Structure PET Amyloid What is the relationship between brain amyloid accumulation, the loss of brain tissue and the development of dementia (Alzheimer s disease)?

18 Alzheimer s Disease

19 Alzheimer s Dementia Memories Fade Functional Decline Changes in Personality Disorientation Loss of Self

20 Symptoms of Dementia

21 Early Stage Short term memory loss Difficulty learning & retrieving new information Expressive language changes Receptive language changes Difficulty planning & sequencing familiar tasks Personality changes

22 Early Stages Behaviour changes Spatial disorientation Worsening fine motor control Decline in work productivity Difficulty doing complex tasks requiring multiple steps Depressed mood

23 Middle Stages Decreased ability performing everyday tasks & self care skills. Decline in short term memory with preserved long term memory Increased disorientation to time and place Worsened ability to express and understand language Difficulty recognising familiar people & objects

24 Middle Stages Poor judgement & worsened attention to personal safety. Mood & Behaviour fluctuations Erratic sleep/wake cycle Physical changes related to progression of the disease: New onset seizures Urinary incontinence Compromised swallow Mobility changes

25 Advanced Stage Dementia Significant memory impairment Dependency on others for all personal care tasks Increased immobility Profound loss speech Loss of mechanics of chewing & swallowing leading to aspiration & pneumonia Full incontinence. Increased survival at advanced dementia Advance Care Plan

26 What can we do? Establish a Baseline In order to observe change effectively, we must be informed about what the person was capable of doing at his or her very best. The baseline is used as a basis of comparison to document change Note self care skills, personal achievements, academic/employment milestones, talents, skills, hobbies.

27 Memory Clinic Screening people with DS over the age of 35- years on an annual basis. Make a consensus diagnosis with the MDT Formulate a management plan Offer advise Follow up longitudinally Training & Education (Family; Staff; Peers) Strategic Planning for Future Care.

28 Personalising Care The emphasis must begin with the person themselves and not the therapy or technique: Know the person What works for the individual Their likes and dislikes Their strengths and needs Their unique life story Their personality.

29 Personalising Care Each Person s experience of dementia is as unique as their fingerprint To be a Person is to have a story to tell. (Isak Dinesen)

30 Improving Outcomes by Developing & Implementing Standards for Care

31 Dementia & Down syndrome

32 A 20 Year Longitudinal Perspective 77 Females with Down Syndrome Aged 35 years+ First screened 1996 Annual assessment for dementia (ICD-10 criteria) in Memory Clinic Comprehensive diagnostic work up and consensus diagnosis

33 Summary of Key Findings Over the 20 year follow-up period 97.4% - developed dementia Age of onset: 55 years (SD 7.07) 96.7% - persons with moderate ID developed dementia 100% - Persons with severe ID developed dementia None had dementia confirmed prior to age 40 years

34 % dementia Risk Trajectory According to Age Age 65 = 88% Risk Age 55 = 45 % Risk Age 50 = 23 % Risk Age (Yrs)

35 Dementia in Down syndrome: Mortality 74.7% (56 of 75 with dementia) died by 2015 Dementia duration 6.6 years (SD=3.3) if they had died; Average age on onset = 55 years Dementia duration 7.74 years (SD=5.4) if still alive; Average age of onset = 56 years No significant difference in duration or in age of onset between those with dementia who had moderate and those who had severe ID.

36 Co-Morbidities 100 Dementia %

37 Epilepsy and Dementia in DS While only 10% of the generic population with AD will go on to develop epilepsy, the figure for people with Down syndrome and dementia is reported to be as high as 80%.

38 Dementia and Epilepsy 77.9% ( 60 of the 75 with dementia) had epilepsy Life Time Prevalence LTP Dementia LTP Epilepsy % Age (yrs)

39 Dementia, Epilepsy & Depression 48% of those with dementia were also reported to have depression. Life Time Prevalence 100 LTP Dementia LTP Epilepsy LTP Depression % Age (yrs)

40 A Strategic Challenge for ID Services Substantial Increased Risk of Dementia >50years Changing Demographics Challenges to current service model Memory Clinic-Baseline Screening and Annual Review A need to Restructure Residential & Day Programmes. A need to upskill staff at all levels to respond to changing needs. End of Life Concerns. Potential for Preventive Treatments Brain Training; Healthy Lifestyle (HELLO BRAIN.EU) Strategic Service Planning is Critical.

41 Remember. Care is better now for people with Intellectual Disability and they are living longer..this is a success story and we should celebrate it Professor Mary McCarron Trinity College