Dementia & people with Dementia & people with Down’s syndromeDown’s syndrome

Dr Nicola Jervis, Clinical Dr Nicola Jervis, Clinical Psychologist, Manchester Psychologist, Manchester Learning Disability Learning Disability Partnership 22/6/05Partnership 22/6/05

AimsAims

• To provide a brief introduction to the To provide a brief introduction to the literature on Dementia and people with literature on Dementia and people with Down’s syndrome Down’s syndrome

• To discuss the development of a To discuss the development of a screening project to assist the diagnosis screening project to assist the diagnosis of dementiaof dementia

• To discuss the results of the screening To discuss the results of the screening project to dateproject to date

Life expectancyLife expectancy

• Life expectancy of people with Life expectancy of people with Down’s syndrome is increasing e.g Down’s syndrome is increasing e.g 1927 = LE 9 yrs, 2000 = LE 57 yrs1927 = LE 9 yrs, 2000 = LE 57 yrs

• More likely to experience the More likely to experience the effects of ageingeffects of ageing

Prevalence of dementia Prevalence of dementia (Alzheimer’s Disease)(Alzheimer’s Disease)

• Approximation for people with DS:Approximation for people with DS: 10% - 40 - 49 yrs (Holland et al, 1998)10% - 40 - 49 yrs (Holland et al, 1998) 40% - 50 - 59 yrs40% - 50 - 59 yrs 55% - 60 – 69 yrs (Turk et al, 2001)55% - 60 – 69 yrs (Turk et al, 2001)• General population = 10% of those aged General population = 10% of those aged

above 65 yrsabove 65 yrs• LD population without DS = 13% 50 – LD population without DS = 13% 50 –

65 yrs, 22% 65+ yrs65 yrs, 22% 65+ yrs• Likely to be underestimatesLikely to be underestimates

Dementia Diagnosis – ICD Dementia Diagnosis – ICD 10 10

• Decline in memory (especially learning Decline in memory (especially learning new information)new information)

• Decline in other cognitive abilities e.g. Decline in other cognitive abilities e.g. planning and organisingplanning and organising

• Awareness of the environment (absence Awareness of the environment (absence of clouding of consciousness long of clouding of consciousness long enough to demonstrate decline)enough to demonstrate decline)

ICD 10 continuedICD 10 continued

• Decline in emotional control/ Decline in emotional control/ motivation or change in social motivation or change in social behaviour in one of a) apathy, b) behaviour in one of a) apathy, b) irritibility, c) emotional lability, d) irritibility, c) emotional lability, d) coarsening of social behaviourcoarsening of social behaviour

• Duration – decline present for at Duration – decline present for at least 6 months least 6 months

Alzheimer’s DiseaseAlzheimer’s Disease

• Above criteriaAbove criteria• Exclusion of other potentially Exclusion of other potentially

treatable causestreatable causes• Gradual onset and continuing Gradual onset and continuing

decline over timedecline over time

Difficulties with Difficulties with assessmentassessment

• General population assessments often General population assessments often applied to people with learning applied to people with learning disabilities, norms are not relevant, disabilities, norms are not relevant, often floor effectsoften floor effects

• People with LD’s often have difficulties People with LD’s often have difficulties with these prior to any decline e.g. with these prior to any decline e.g. orientation qu’sorientation qu’s

• Few assessments designed specifically Few assessments designed specifically for people with a LDfor people with a LD

RecommendationRecommendation

• Given these difficulties it has been Given these difficulties it has been recommended by the AAMR and the recommended by the AAMR and the IASSID that IASSID that

‘‘all adults with intellectual disability be all adults with intellectual disability be evaluated using standardised evaluated using standardised procedures to assess memory, other procedures to assess memory, other cognitive functions, and adaptive/ cognitive functions, and adaptive/ maladaptive behaviour at least once in maladaptive behaviour at least once in early adulthood’early adulthood’

Why?Why?

• Helps to monitor people against there Helps to monitor people against there own baselineown baseline

• Assesses people prior to any Assesses people prior to any deterioration taking placedeterioration taking place

• Increases the validity of carer scalesIncreases the validity of carer scales• Need to use direct and indirect Need to use direct and indirect

assessmentsassessments• Sims (2002) discusses the ethics of Sims (2002) discusses the ethics of

baseline (prospective) assessments baseline (prospective) assessments

Baseline screening project Baseline screening project in Manchesterin Manchester

• Aim: To assess everyone in Manchester Aim: To assess everyone in Manchester known to MLDP with Down’s syndrome known to MLDP with Down’s syndrome over the age of 25 yrs (approx. 116 over the age of 25 yrs (approx. 116 people)people)

• Would expect around 20 people to have Would expect around 20 people to have dementia based on researchdementia based on research

• Multidisciplinary contribution to Multidisciplinary contribution to assessment, assistant psychologist post assessment, assistant psychologist post allocated to complete assessmentsallocated to complete assessments

Measures- Semi structured Measures- Semi structured interviewinterview

• Semi structured Interview (areas Semi structured Interview (areas covered : daytime occupation, covered : daytime occupation, concerns, support person needs, concerns, support person needs, those involved, physical/ mental those involved, physical/ mental health concerns, health concerns, skill/mood/behavioural changes, skill/mood/behavioural changes, medication, life events) medication, life events)

Health ChecklistHealth Checklist

• Aim: to identify conditions that might Aim: to identify conditions that might mimic dementia symptoms early on.mimic dementia symptoms early on.

• Detailing how often areas need to be Detailing how often areas need to be assessed, when last assessed, problems assessed, when last assessed, problems identified, need for reassessment, actionsidentified, need for reassessment, actions

• Health areas – hearing, vision, thyroid, Health areas – hearing, vision, thyroid, dental, heart, height/ weight, woman’s dental, heart, height/ weight, woman’s health, skin (consider epilepsy and health, skin (consider epilepsy and diabetes symptoms also) – regular checks diabetes symptoms also) – regular checks recommendedrecommended

Communication Communication assessmentassessment

Focuses on:Focuses on:• Channels of communication person usesChannels of communication person uses• What supports persons understandingWhat supports persons understanding• Level of understanding of spoken Level of understanding of spoken

languagelanguage• Concerns around eating/ drinking/ Concerns around eating/ drinking/

swallowing swallowing • Changes noticedChanges noticed

Functional/ mobility Functional/ mobility assessment assessment

Focuses on:Focuses on:• History of falls, support needed History of falls, support needed

with transfers, use of stairs, aids with transfers, use of stairs, aids and adaptations, mobility and adaptations, mobility difficulties, etc.difficulties, etc.

Carer assessments - DMRCarer assessments - DMR

• Dementia questionnaire for Persons with Dementia questionnaire for Persons with Mental Retardation (Evenhuis, Kengan Mental Retardation (Evenhuis, Kengan and Eurlings, 1990)and Eurlings, 1990)

• Assesses short term/ long term memory, Assesses short term/ long term memory, spatial & temporal orientation, speech, spatial & temporal orientation, speech, practical skills, mood, activity & interest, practical skills, mood, activity & interest, behaviour concernsbehaviour concerns

• Norms for PWLD for first and repeated Norms for PWLD for first and repeated assessmentsassessments

Carer Assessment - ABS – Carer Assessment - ABS – RC 2RC 2

• AAMR Adaptive Behaviour Scale AAMR Adaptive Behaviour Scale Residential and Community- 2Residential and Community- 2ndnd Ed. Nihira, Ed. Nihira, Leland, Lambert, 1993)Leland, Lambert, 1993)

• Changes in adaptive behaviour often first Changes in adaptive behaviour often first signs notedsigns noted

• Part 1 - Assesses independent functioning, Part 1 - Assesses independent functioning, physical development, self direction, etc.physical development, self direction, etc.

• Part 2 – Assesses potentially concerning Part 2 – Assesses potentially concerning behaviour e.g. social behaviour, self behaviour e.g. social behaviour, self abusive behaviour, conformityabusive behaviour, conformity

RCPMRCPM

• Raven’s Coloured Progressive Raven’s Coloured Progressive MatricesMatrices

• Used to assess level of ability to Used to assess level of ability to determine which individual determine which individual assessment to useassessment to use

Individual Assessments – Individual Assessments – people with a mild LDpeople with a mild LD

• Cambridge Cognitive Examination Cambridge Cognitive Examination (CAMCOG), taken from the CAMDEX – R (CAMCOG), taken from the CAMDEX – R (Roth, Huppert, Mountjoy and Tym, (Roth, Huppert, Mountjoy and Tym, 1999)1999)

• Assesses orientation, language, Assesses orientation, language, memory, abstract thinking, perception, memory, abstract thinking, perception, attention, executive functioning, praxis attention, executive functioning, praxis (includes MMSE)(includes MMSE)

• Hon et al (1999) – found scale useful to Hon et al (1999) – found scale useful to assess people with DSassess people with DS

People with a Moderate People with a Moderate Learning DisabilityLearning Disability

• Dementia Rating Scale – 2 (DRS 2) Dementia Rating Scale – 2 (DRS 2) Jurica, Leitten & Mattis (2001)Jurica, Leitten & Mattis (2001)

• Assesses attention, Initiation/ Assesses attention, Initiation/ perseveration, construction, perseveration, construction, conceptualisation, memoryconceptualisation, memory

• McDaniel & McLaughlin (2000) – McDaniel & McLaughlin (2000) – previous version sensitive to age related previous version sensitive to age related changes in people with DSchanges in people with DS

Severe Learning DisabilitySevere Learning Disability

• Test for Severe Impairment – Albert & Test for Severe Impairment – Albert & Cohen (1992)Cohen (1992)

• Areas assessed e.g. language Areas assessed e.g. language production, motor performance, production, motor performance, memory, languagememory, language

• Cosgrave et al (1998) – good reliability Cosgrave et al (1998) – good reliability and validity with people with Down’s and validity with people with Down’s syndrome with/ without Dementiasyndrome with/ without Dementia

Screening Project ResultsScreening Project Results

• 80/116 people assessed so far80/116 people assessed so far• People already known to have dementia People already known to have dementia

not assessednot assessed• 15/80 query about dementia – need 15/80 query about dementia – need

additional investigationsadditional investigations• Concerns highlighted about routine Concerns highlighted about routine

health checks not being undertaken for health checks not being undertaken for people with Down’s syndrome for people with Down’s syndrome for conditions that mimic dementia conditions that mimic dementia symptomssymptoms

Health areasHealth areas

• Hearing - 32/80 not been assessed in Hearing - 32/80 not been assessed in last 2 years (Rec.)last 2 years (Rec.)

• Vision – 20/80 not been assessed in last Vision – 20/80 not been assessed in last year (Rec.)year (Rec.)

• Thyroid – 46/80 not been assessed in Thyroid – 46/80 not been assessed in last year (Rec.)last year (Rec.)

• Heart – 55/80 not been assessed in last Heart – 55/80 not been assessed in last year (Rec.)year (Rec.)

• Dental – 8/80 not been assessed in last Dental – 8/80 not been assessed in last 6 months (Rec.)6 months (Rec.)

Reassessment stagesReassessment stages

• People will now have their own baseline People will now have their own baseline which comparisons can be made against which comparisons can be made against in the futurein the future

• Reassessment interval reduces with Reassessment interval reduces with increasing age increasing age

1.1. Aged 30 – 40 yrs, every 10 yrsAged 30 – 40 yrs, every 10 yrs2.2. Aged 40 – 50 yrs, every 5 yrsAged 40 – 50 yrs, every 5 yrs3.3. Aged 50 +, every 2 yrs Aged 50 +, every 2 yrs • If concerns noted prior to reasssessment If concerns noted prior to reasssessment

date referral made to psychologydate referral made to psychology