Screening Adults with Downs Syndrome for Dementia

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Screening Adults with Downs Syndrome for Dementia

• Dr Sarah Whitwham
• Clinical Psychologist
• Learning Disability Service, Plymouth

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• Life Expectancy
• England, 1929 - Average life expectancy was 9
  years.
• Today, about 80 of people with Downs Syndrome
  live to over 50 years old.
• People are now surviving into their 60s and 70s.

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Age-related risk of Alzheimers
Disease Prevalence by age of onset (Alzheim
ers Disease International, Feb 03)
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• Downs Syndrome Alzheimers
• Virtually all people with DS over 40 show
• characteristic brain changes of AD - although not
  
• all show clinical signs.
• Average age of onset of AD is 54 years.
• Interval from diagnosis to death approximately 5
  years.

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• Downs Syndrome and Dementia Briefing for
  Commissioners
• Turk Dodd 2001 suggest
• A register of people with DS is needed.
• Baselines of cognitive adaptive functioning are
  conducted before age 30.
• LD teams must develop specialist skills in this
  area.
• They must offer training to other professionals
  and front line staff and carers.
• Co-ordination between agencies - independent
  providers, social services, LD health team,
  hospital teams, etc.

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• Plymouth Screening Project
• The identification of all people with Downs
  Syndrome in Plymouth (may be used as a research
  cohort in the future.)
• Develop a screening protocol.
• Commence one-off screening assessments
• for all adults with Downs Syndrome.

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Establishing a register

• Write to all residential homes, day care centres
  and Care Managers asking for names of people with
  DS.
• Keep names on a Data Base

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• The Psychology Screen
• Three stages
• 1. Review historical information on file (up to
  4 hours)
• 2. Assessment battery with the individual face
  to face (1-2 hrs)
• 3. Interview with an informed carer (1-2 hr)
  Behaviour, mood,daily living skills,life-events,ca
  re-giver burden (CAS-ID) and the DMR.

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Consent or Assent

• Ethical Committee approval.
• Informed consent can the person understand the
  risk, the co-operation with assessments and
  having the results held on file? If so they can
  give/withhold consent.
• Assent where the person cannot give informed
  consent, a next of kin or carer is asked to agree
  (assent) to the person participating.
• Extra consent to use video tape for training.

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Two groups

• Those being screened while still well so that we
  have a baseline for the future
• Offered to every adult with DS.
• Those being more fully assessed because there are
  concerns

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Care Pathway Plymouth

• If there are concerns about possible dementia
• Community Nurse conducts health assessment geared
  to dementia screening.
• Bloods are taken to rule out thyroid dysfunction
  and other problems.
• Cognitive screen by Clinical Psychologist to
  assess cognitive level, adaptive behaviour, mood.
• Assessment by Psychiatrist to make differential
  diagnosis and refer for CT scan/EEG if necessary.

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Frequency of screening

• Anyone who is referred because there are concerns
  is screened. Re-screening can be from 3months to
  several years depending on results.
• Everyone identified with DS will be offered a
  one-off baseline screen. Re-screens will be set
  for when they are 40 years old, unless concerns
  before then.

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182 Downs Syndrome names received
5 moved out of area/cannot find
12 not Downs Syndrome
164 Downs Syndrome names on Data-Base
15 refused consent/assent
149 consented / assented
 
49 Referred Cohort all screened regularly
100 New Cohort of whom 87 screened
6 referred with concerns (4 dementia, 2 other)
3 died
2 died
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Age groups of 165 adults with DS
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• Final thoughts
• Diagnosis may have come some time after the
  actual onset of the symptoms.
• The length of time between diagnosis of
  Alzheimers Disease and death can be as short as
  3 - 5 years.
• The drugs available work best when given early in
  the course of the disease.
• Will allow us to respond appropriately to
  changing needs as soon as possible.