Down Syndrome and Dementia

1. Down Syndrome and Dementia

2. What is Dementia? • Two main types: • Multi infarct or vascular dementia • Alzheimer’s disease

3. Multi infarct dementia • Characterised by problems in the blood flow to areas of the brain • Typically caused by series of mini strokes and associated with hardening of the arteries • Second most common cause of dementia accounting for 25% of sufferers

4. Alzheimer’s Disease • Caused by changes in the nerve cells of the brain • Plaques and neurofibrillary tangles develop • Interference to transmission between neuron to neuron and neurons and muscle

5. Alzheimer’s Disease • Is the biggest cause of dementia accounting for 50% of cases • Although people with Down Syndrome can develop Multi infract dementia, it is Alzheimer's that they are more susceptible to

6. Alzheimer’s Disease and Down Syndrome • Discovery of increased likelihood associated with greater life expectancy. • Presence of neurofibrillary tangles in almost every person with Down Syndrome by the age of 40. • Average of dementia onset is 54.2 years • Likely to be associated with Trisomy 21

7. Prevalence of AD in people with Down Syndrome • Age group 35-49: 8% develop AD • Age group 50-59: 55% develop AD • Age group 60 plus: 75% develop AD Lai and Williams 1989

8. Alzheimer’s Disease – early stage • Loss of short term memory • Language problems – finding the right words • Performance on usual tasks deteriorates • Changes in behaviour • Disorientation

9. Alzheimer’s Disease- Mid stage • Symptoms become more obvious, particularly language skills • Disorientation (time, place, person) • Confusion resulting in frustration • Loss of self-care skills and continence • Long periods of apathy or inactivity • More severe changes in personality and social behaviour

10. Alzheimer’s Disease – Late stage • Loss of eating and drinking skills • Problems with mobility • Problems with recognising people • Incontinence • Development of seizures • Need often for 24 hour care • Increase in health problems such as pressure sores and infections

11. Communication – good practice • Keep environment calm and quiet • Approach from front and smile! • Monitor eye contact • Identify yourself and use their name • Make sure that you are seen before touch • Try to talk to the person on your own • Check if more receptive at particular times of the day

12. Communication good practice • Speak slowly and clearly • Keep language, responses and choices simple and concrete • Offer specific choices requiring ‘yes’ or ‘no’ answers • Use reminders/repetition • Allow people time to process information • Use visual aids

13. Communication – bad practice! • Distraction – television (pictures and sound); other people/interruptions • Long complicated sentences • Long notice before stressful events –anticipatory anxiety • Repeating yourself if misunderstood • Confrontational speech or body language

14. Principles of therapeutic environments • Predictable • Calm • Familiar • Appropriate level of stimulation • Structured • Adapted to the individual/makes sense to them

15. Therapeutic environment • Maintain daily routines, carrying them out at same times and places • Avoid unnecessary change • Use different spaces for different activities • Use music – calming not constant • Avoid excessive noise and commotion

16. Therapeutic environment • Build awareness of triggers for difficult behaviour or disorientation • Attention to colours to aid recognition – red orange yellow are more noticeable • Risk assessment for ‘wandering’ • Use of visual cues • Monitor reaction to mirrors, reflections from pictures

17. Therapeutic Environments • Use of lighting to counteract ‘Sundowning’ • Non glare lighting to minimise shadows • Give attention to colour of carpets and shiny floor surfaces. • Encourage failure free activities particularly in the mid stages of dementia

18. Therapeutic environments • Goal planning for specific skills • Capture current picture with regard to skills, hobbies and interests • Reminiscence • Music for relaxation and pleasure • Aromatherapy • Balance between stimulating and low arousal atmosphere

19. Types of challenging behaviour most associated with Down’s syndrome and dementia Changeable moods_ Irritability Stubbornness Mood lability-laughter to tears Withdrawal Inappropriate responses to people or events

20. Possible solutions • Look for specific triggers • Maintain regular routine • Reassurance • Explain what is happening • Monitor mood, sleeping patterns, eating • Rule out other causes and treat where necessary • Distractions • Special care during personal care

21. Aggression/Unusual behaviours Lashing out, verbal aggression Sexualised behaviour Screaming, shouting, crying, repetitious talk Storing , hoarding, throwing things away Inappropriate urination and defecation Resorting to the floor

22. Possible response/solutions Reassure Reduce demands Breakdown tasks Distraction with desired activities/redirect Don’t approach quickly or from behind Look for triggers Pain or discomfort Check previous history (interests/known helpful approaches/ history of abuse?) Reduce possible irritants- alcohol; caffeine

23. Reasons for Wandering • Disorientation • Physical discomfort • Boredom • Searching • Separation Anxiety • Reactivating previous activities • Night time wandering • Attention seeking/looking for help • Apparent aimlessness

24. Coping with challenges • Collecting data (ABC charts) • Establishing purpose/ function of behaviour • Monitoring / changing environment • Effective distracters • Monitoring own body language/ tone of voice/ use of personal space