Dementia in People with Intellectual Disability: diagnostic and management challenges

1
Dementia in People with Intellectual Disability
diagnostic and management challenges

• Chris Perkins
• Psychiatrist
• MHSOP and Dual Disability
• BOPDHB

2
Longevity

• UK Currently 12 of ID population aged gt65 by
  2040 expect it to be 25 (UK) (7)
• 2 of population gt 65 will have ID
• 1063 people with ID gt55 years in NZ in 1993 (Hand
  1995) numbers increasing
• In 2003 half IHC users in Central Region gt55 (8)

3
Consequences of increased life-span

• More disorders of old age mental and physical
  including dementia
• Ageing parents no longer able to continue care at
  home
• Need residential options suitable for retired
  people with ID
• Older people with ID fall between disability
  and age-related stools in service provision

4
Down Syndrome

• 44 live to gt 60 years
• 14 gt 68 years (10)
• Incidence of dementia in Down syndrome 15-40
  (1)
• 30-35 years 3.4
• 40-49 years 10.3
• 50-59 years 40 (11)

5
Dementia in Down Syndrome

• Extra Chromosome 21 ? extra gene for amyloid
  precursor protein ? people with DS secrete 1.5x
  amount of amyloid than non-DS
• gt35 years all people with DS have classical
  plaques and tangles but not all get AD

6
Other causes ID

• ? Incidence same as rest of population (2)
• Making the diagnosis in person with ID

7
Who makes the diagnosis?

• GPs dont feel confident
• Psychiatrists dont want to see people with ID
• (Psycho) geriatricians see some? (But lt65)
• Dual disability providers?
• (Often seems to be the support worker or manager
  of group home)

8
Why make the diagnosis?

• For all the usual reasons
• Education of person, family and support workers
• Support e.g. via Alzheimers NZ
• To access additional care (age-related)
• Planning
• Medication?

9
Response to medication (13)
10
And how?

• Informant information caregivers, family
• Physical and MSE
• Check list?
• Diagnostic criteria adapted to people with ID

11
DSM 4

• Memory impairment
• aphasia,
• apraxia,
• agnosia,
• executive function
• Decline in functioning
• Not something else

12
Diagnostic Criteria DC-LD

• Loss of memory, both short and long term
• Cognitive decline especially loss of skills of
  judgment and understanding
• Change in adaptive behaviour which is recognised
  by loss of skills in self care
• Language difficulties, both of expressive
  language and comprehension

13
DC-LC

• Apraxia
• Agnosia
• Excluded as criteria because of difficulty in
  measurement
• (but are they more difficult to measure than
  memory?)

14
Neurological signs supportive of diagnosis

• Frontal release signs
• Epilepsy
• Late onset seizures in DS (prevalence 82-84)
  (1)(3)
• Depth perception?
• Dysarthria, dysphasia, Parkinsonian features,
  spastic paresis of limbs, contractures in DS (3)

15
People with DS and dementia may show..

• In early stages
• General slowing
• Personality change
• Apathy and loss of motivation
• More like frontal lobe dementia
• Not the same degree of behavioural disturbance (
  but similar types?)

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Differential Diagnosis

• Depression may cause loss of cognitive skills
  and regression, (may be prodromal for dementia)
  Anxiety?... Phobic d/os 5.8
• Delirium
• Physical disorders (e.g. hypothyroidism in DS)
• Sensory problems (cataracts and otosclerosis in
  DS)
• Medication polypharmacy common

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Problems making diagnosis

• Lack of longitudinal history ? failure to
  recognise decline need to have informant who
  knows person gt6 mo
• MMSE unhelpful? Repeated neuropsych. Testing
• Diagnosing depression / physical problems e.g.
  more difficult if poor communication
• But basically the same as people with normal IQ

18
Checklist of observed changes in older
people (IHC NZ Inc)
19

• Service user
• known for gt1 year
• Useful info. for
• assessors not to
• formally diagnose an
• individual

20
(No Transcript)
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Referrals for ? dementia in person with ID

• 9 referrals to MHSOP and DDS over last year.
  Underlying request is often for placement in
  residential care? questions
• Are people with ID similar in age and interests
  even if they do have age-related disorder?
• Are younger people with ID suitably placed in
  residential care with OP?
• What about ageing in place?

22
Referrals to MHSOP and DDS

• 3 no dementia
• 2 delirium (? Underlying dementia)
• 4 dementia ( 2 new diagnoses)
• Referral source MHS 2, ID Sector 5. GP2

23
From MHS

• 1. M 65 mild ID, schizophrenia and deterioration
  in mobility. No dementia Mental Health
  Residential Provider not able to manage poor
  mobility. Geriatric assessment, OT, placed in RH
• Problem was really MH providers reluctance to
  adapt to persons physical needs

24
From MHS

• 2. M 45 No dementia family history of AD,
  cerebral atrophy on CT brain no history
  suggestive of dementia (or of current mental
  illness). Long-term challenging behaviour .
  Placed with ID provider who may or may not manage
  behaviour
• Main issue lack of suitable ID beds for people
  with behaviour challenges in BOP .

25
From ID Provider (5)

• 1.M. 61 delirium (underlying dementia DS) needed
  Rx for UTI, also anxiety. Group home couldnt
  manage (respite, additional staff unavailable due
  to funding issues) transferred permanently to RH
  
• Issue lack of flexibility to provide a temporary
  increase in care

26
From ID residential provider

• 2.M 43 recurrent chest infections, delirium,
  possible dementia house has no daytime staff
  Resident still had to go out when ill. May be
  transferred to retirees home when bed
  available.
• Problem Lack of daytime support staff, lack of
  accommodation for people who can no longer attend
  work

27
From ID residential provider

• 3. M 58DementiaLiving in group home, up at
  night. T/F to RH with mother (who has AD)
  Behavioural difficulties? St III ? picked on by
  other residents and deteriorating mobility ?
  Hospital level care. Rapid course of illness
• Multiple moves. Would be better in
  psychogeriatric hospital care, but no beds

28
From ID residential provider

• 4. F 48 probable dementia, many years
  institutionalised very disabled, blind, mute,
  resistive DS developed epilepsy, difficult to
  get history, possible decrease in skills.
• Disagreement between management and care staff
  about history and whether she could be adequately
  cared for in group home.
• Moderate ID, dependency and perceptual problems
  made diagnosis difficult

29
From ID residential provider

• 5. M 58 dementia difficult behaviour, no
  overnight staff,
• Staff felt they could cope mangers wanted him in
  Stage 111
• Where is he now?

30
From GP (1)

• 1. F 45 dementia
• DS lives with ageing mother, sister supportive
  (WG / PM) explicitly looking for L-T placement?
  ID house for older people
• Good history, caring family, making decisions for
  future

31
From GP

• 2. M 62 Lives in RH Spec. endorsement
  cyproterone. Our decision to assess moderately
  severe ID very limited range of activities, but
  no clear deterioration. Probably no dementia. No
  change in management
• Institutionalised long-term, no family, no
  advocate, impoverished life?

32
Issues for our clients

• Variable quality of information available at
  group home
• Limited function and communication,
  institutionalisation and perceptual problems make
  diagnosis difficult
• No family for history and decision-making
• Mangers see things differently from support
  workers
• Adult MHS get desperate for placement for people
  with ID!
• Behaviour problems seemed similar to people with
  pre-morbid normal IQ

33
Issues continued

• Lack of suitable ID beds for people with
  behaviour challenges
• Difficulty providing a temporary increase in care
• Lack of daytime support staff
• Lack of specific accommodation for older people
• Multiple changes in accommodation

34
Group Homes

• Maximum number of residents 5
• Houses are small. If one resident is disruptive
  it is hard to get away (c.f. with RH)
• Staffing at night nil, sleep-over, wake-over
  . If wake-over expected to be working? disturbs
  residents

35
Group Homes

• Group homes may not have daytime staff they are
  geared for working age population (There is 1
  group home for OP, not necessarily with dementia,
  in Tauranga)
• Group homes cant (or dont) readily access
  additional help if residents frail or ill
• Funding comes via MOH directly. Some DHB services
  not available

36
ID residential providers cant directly access

• District nursing
• Ancillary services e.g. OT, dietitian, physio.
• Personal cares (per NASC)
• 28/7 residential respite
• Dementia daycare
• These are available to PWD and ID living in own
  home.
• ID provider can pay for services and then get
  reimbursed from MOH and can get client reassessed
  to allow for increased levels of staffing in house

37
Cant provide for people with dementia

• Who wander, especially at night
• Are terminal, frail, medically ill, need hospital
  level care
• (Similar to other people with dementia)

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Service issues

• Historically in US (and NZ) more interest in
  children See MOH 2001 The Clinical Assessment
  and Management of Children, Young people and
  Adults with Down Syndrome 4/69 pages devoted to
  adults)
• Conflict over responsibility for PWD- ageing or
  ID services?
• Who is primary client--person with ID, ageing
  parent, family or ID service provider?
• What is and appropriate service model in home
  care? Specific facilities? Generic dementia
  services? How to plan for transition? (4)

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Health of Older People Strategy 2002

• services need to develop to support ageing in
  place offering people the opportunity to
  continue to live safely in their community.
• This includes. Ongoing support for those who
  are disabled.

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The Clinical Assessment and Management of
Children, Young people and Adults with Down
Syndrome (MOH 2001) re Alzheimers Disease

• All efforts should be made to maintain the
  person in their current accommodation and
  occupational settings (with appropriate
  modifications) before moving to more suitable
  accommodation. Involvement of geriatric services
  and contact with specialised support services
  should occur (p 28-29)

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Assessment process for Older People MOH Oct 2003

• Acknowledges increasing numbers of older people
  with ID
• Suggests assessors have specialist training in
  the ID area and support from specialists with
  expertise in the disability field

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WHO Ageing and Intellectual Disabilities_
Improving Longevity and Promoting Healthy Ageing

• National policies should recognise the
  contribution of adults with ID to the greater
  population, and where appropriate specialised
  services and assistance should be provided, and
  equitable access to generic services should be
  ensured
• p 20

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Residential Circumstances Of Older (55)
Australians with Intellectual Disability
44
Edinburgh Principles for people with ID and
dementia (5)

• Promote utmost QOL base services on
  person-centred approach
• Individual strengths, capabilities, skills and
  wishes should be overriding consideration in
  decision-making
• Involve individual and family in all phases of
  assessment, service planning and provision
• Ensure appropriate diagnostic, assessment and
  intervention services available

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Edinburgh Principles

• 5. Plan and provide supports and services that
  optimise remaining in chosen home and community
• 6. Ensure same access to services and supports as
  those in general population affected by dementia
• 7.Ensure generic, cooperative and proactive
  strategic planning

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The words are right

• Where to from here?

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Recommendations (5)

• Need trained personnel to do diagnostic work-up /
  standardised assessment instrument
• ID Carers need to be upskilled
• Bring providers together / avoid thinking
  compartmentally ID /or geriatric services
  responsible?
• Register of people with ID and dementia for
  planning

48
Recommendations

• Staffing / funding for increased levels care
• Maintain community supports
• Advocacy / political activism
• Research evaluation of care models
• Examine conflicts around differing philosophies
• Prevention

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Summary

• People with ID are living long enough to develop
  dementia
• Diagnosis similar to that for other people,
  though some challenges
• Current ID residential care is often not suitable
  for people with dementia
• We need to support ageing in place for people
  with ID and dementia

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References

• Prasher Corbett 1993
• Janicki Dalton 2000
• Lai and Williams 1989
• McCallion Janicki 1997
• Wilkinson and Janicki 2002
• WHO Ageing and Intellectual disabilities WHO/
  MSD/HPS/MDP/00.3
• Davidson, Prasher Janicki (2003) P2.
• Webb Rogers (2002)
• Hand Reid (1996)
• Chicione and McGuire (1997)
• MOH 2001
• Holland et al (1998)
• Torr J (2006)