Recognition of dementia in people with learning disabilities

Transcription

1 Recognition of dementia in people with learning disabilities Dr Karen Dodd Co-Director, Services for People with Learning Disabilities Associate Director, Therapies Learning Disabilities / Consultant Clinical Psychologist January 2016

2 Down Syndrome Trisomy 21

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4 Life Expectancy

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6 The link with Down s syndrome? Down s Syndrome is a genetic disorder caused in 96% of cases by a extra copy of chromosome 21 Evidence of triad on post mortem over the age of 30 years Chromosome 21 has the Amyloid Precursor Protein (APP) gene Gene for Family history of Alzheimer s is also on Chromosome 21 (and 1 and 14) ApoE status may be relevant

7 Current Prevalence and Survival London Consortium study Median age of onset = 55.4 years 50% of those who develop dementia are diagnosed in their 50 s Median survival time= 4.1 years (95% CI: 3.6, 4.6) Age at diagnosis highly predictive of survival time Sex not predictive

8 Dementia Prevalence Rates 80 Comparison of dementia prevalence rates by age Prevalence (%) General population Intellectual disabilities Down's syndrome Age

9 Current thinking re prevalence in DS 80% of people with DS will have dementia at age 60 (McCarron 2014) Prevalence - 21/2 times more in people with DS than in people with learning disabilities without DS Prevalence rates may drop with increasing age Alzheimers disease is now the leading cause of death for people with DS

10 Issues Brain reserve some people have passive brain reserve - brain mass, size and synapse count, inherent ability active reserve - can improve networks through occupation, activity. People with learning disabilities may not have either passive or active reserve. Some risk factors less common in people with DS - eg smoking and ischiemic heart disease.

11 Conditions that Mimic Dementia Physical Health issues Uncontrolled Epilepsy Nutrition vitamin B12 & Folate Electrolyte abnormalities Hypothyroidism Sensory Impairments Visual Impairments (recognition, lost skills) Hearing Impairments (misunderstanding) Mental Health Problems Depressive illness (pseudo-dementia) Psychotic disorder (decline in function) Chronic Anxiety (catatonia) Medication Anticholinergics, psychotropics, AED s, Pain meds, antihistamines and benzodiazepines

12 Conditions that Mimic Dementia II Sleep Disturbance Sleep Apnoea or other sleep disturbances Life Events Loss of parents, moves, day-care changes etc Impact of poor social / physical environment Abuse Loss of skills and regression Loss of skills and regression Acute Organic Brain Syndrome Confusional state secondary to pain, chronic infection or head injury

13 How do you know if it is dementia? Need to check and treat: thyroid problems - thyroid should be tested annually depression other mental health problems sensory difficulties both vision and hearing which should be checked at least every 2 years physical problems e.g. cancer, infections environmental changes neurological conditions psychological problems polypharmacy people taking many medications can show side effects that mimic the early signs of dementia Only if all these have been checked, and symptoms for more than 6 months, should you begin to think dementia.

14 Presentation in People with DS Most common form of dementia in people with DS is Alzheimer s disease. It is known that the brain pathology of Alzheimer s disease is almost universally found in later life in people with Down s syndrome. Vascular disease, and the risk of vascular dementia is rare. Dementia in people with DS may present atypically with changes in behaviour and/or personality that can precede the full clinical picture of dementia by some years (see next slide). Dementia in people with Down s syndrome may be associated with the onset of seizures for the first time in that person s life (78 98%). The middle and later course of Alzheimer s disease in people with Down s syndrome are similar to those experienced by people in a similar stage of dementia but without pre-existing learning disabilities. The time course of dementia in people with DS with dementia has been reported to be more rapid than in the general population.

15 Early signs in people with DS Evidence that people with DS have a frontal-like dementia (perhaps in their 30 s) prior to developing full Alzheimer s disease. Frontal-like dementia will affect: Mood Behaviour Executive function e.g. planning, initiative, problem solving, reasoning Personality Social skills Memory and language are not affected

16 Atypical presentations in people with DS Anecdotal reports of people with DS in teens or early adult who deteriorate, often after a life event, and never or, only after months or years, recover. This has not been reported in the literature but the characteristics of the decline may superficially resemble that of dementia or depressive illness but it neither seems to progress (as would be expected with dementia) or resolve (as would be expected with depressive illness). The clinical picture is dominated by the development of a general slowness in mental and/or physical activity, apparent loss of interest in previous activities, and a lowered level of functioning. Unclear how such problems should be best conceptualised. If depressive illness is a possible factor, a trial of anti-depressant medication may be indicated, with careful monitoring of outcomes. Check that there is no progressive disorder e.g. dementia. Try to help the person progressively back to their previous functioning.

17 Assessment in adults with DS Medical assessment e.g. hearing, sight, blood tests, CAT/MRI scans Cognitive assessment e.g. orientation, memory, learning, language, visuo-spatial skills Adaptive assessment e.g. daily living skills, social and communication skills Behavioural assessment e.g. changes in behaviour, personality, unusual or challenging behaviours Background history: medication, medical history, health status, past abilities, risk factors e.g. head injury, family history of Alzheimer s Psychosocial/ psychiatric assessment e.g. mental health problems, recent life events, bereavement, social and physical environment

18 Issues in establishing premorbid functioning A core feature of dementia is a decline from a baseline level of the person s functioning. Establishing pre-morbid skills, abilities and personality can be challenging in the intellectual disability population due to: variance in cognitive functioning and abilities, frequent poor record keeping from childhood the possible lack of consistent involvement of family or staff throughout the person s lifespan.

19 Recognising early signs Signs of early dementia can be subtle and require careful observation to identify concerns in a timely way. Families and staff carers can often be so close to the person that they become less able to recognise minor changes in functioning through adapting to the person s needs.

20 Assessment process 1. History and information gathering 2. Mental State examination 3. Physical examination 4. Physical investigations 5. Environmental assessment Leads to a Formulation

21 History and information gathering Nature of the presenting problem(s), origin, rate/pattern of progress (sudden or gradual), presence of seizures and other associated conditions, impact on the person s overall functioning and personality. History of significant physical and medical history including past and present medical conditions, e.g. diabetes, hypertension, thyroid or cerebrovascular disease, B12 deficiency. History of or current presence of psychiatric symptoms such as depression, anxiety or other mental health problems. Record developmental history and best level of historic functioning. Ascertain if there are any previous neuropsychological test data on record and compare data with previous assessment results. Record historic daily living skills, interests/hobbies/skills and details of personality.

22 Family history: dementia or other mental health and medical conditions (particularly in first-degree relatives). Assess for psychosocial issues, changes or life events. These include house moves, health decline/death of loved ones, change of caregivers, changes to, or retirement from work/day services. Information gathering should be undertaken through a combination of informant interview (preferably with a family member, when relevant and appropriate) or an informant who has known the person well for a period of six months at least) and directly from the person where possible.

23 Mental State examination Observation of: level of alertness orientation to time, place and person any evidence of alterations in consciousness, psychomotor activity, mood, thoughts, evidence of any abnormal mental beliefs or experiences, and perceptual abnormalities. evaluation of memory and other cognitive functions via formal assessment

24 Physical examination Where possible a complete physical examination should be undertaken. This should occur in the context of primary care, although support may be provided by specialist services. The key issues are: Cardiovascular system focal deficits, evidence of cardiovascular accident etc. Detailed neurological examination (focal deficits, gait abnormalities, speech abnormalities etc.). Endocrine system: signs of hypothyroidism. Careful recording of historic/newly added/current medications (with particular attention to those that are psychoactive, antiepileptic, sedating or anticholinergic).

25 The physical health issues could be addressed in these individuals by a combination of: Brief physical checks e.g. blood pressure, pulse. Observations for any evidence of physical health issues. Observational tools (e.g. pain questionnaires); Information from carers e.g. The OK Health Checks (Matthews, 2006), Health Action Plans (DH, 2001) or bespoke nursing assessments developed to meet the needs of local intellectual disability services can be used to structure this process.

26 Physical investigations Recommended routine investigations are: Full blood count. Urea & electrolytes. Blood sugar. Thyroid function tests. Liver function tests. B12 and Folate level. Lipid profile. Sensory screening vision and hearing. Optional tests are: Electro Encephalograph (EEG) Neuro-imaging Electro Cardiograph (ECG)

27 Neuroimaging The most consistent structural change in the early stage of Alzheimer s disease is the atrophy of the medial temporal lobe. People with Down s syndrome have medial temporal lobe atrophy even without dementia. However, normative values have not yet been established, so neuro-imaging currently has limited value in the early diagnosis of Alzheimer s disease in people with Down s syndrome. Its value is mainly to rule out structural lesions other than atrophy (e.g. space occupying lesions). It should therefore be used only when the clinical picture suggests the possibility of such lesions.

28 Some people with intellectual disabilities may be able to go through the procedure without any other interventions, others may benefit from the use of one dose of oral anxiolytic medication such as lorazepam or diazepam an hour beforehand. Some clinicians prefer to use buccal midazolam, which provides rapid and short-term sedation and therefore may be given immediately before the procedure. Generally, the MRI procedure is longer and more anxiety provoking. In these situations, discussion with the radiologist may be helpful in deciding if CT can be used as an alternative. New generation CT scans are much more user friendly and less anxiety provoking. If the individual clearly needs neuro-imaging but is unable to cooperate in spite of all these measures, it can be undertaken under general anaesthesia. Mental Capacity issues will need to be considered.

29 Environmental assessment Quality of the person s physical environment. Staffing levels (day and night). The mix of people with intellectual disabilities in the residential and day care settings. Quality and quantity of day activities. Staff characteristics: attitudes and competence, including consistency of approach. Scrutiny/review of historic/current support package.

30 Issues with Assessment tools Assessment tools for dementia in the general population are not appropriate for people with intellectual disabilities. There is currently no agreed battery of assessments with which to assess dementia in this population and there is often great variation in screening/assessment methods. There continues to be a lack of research data to secure agreement in order for there to be better uniformity across services and for future data to be pooled and compared. Lack of research data and the nature of the intellectual disability population requires each individual to be viewed independently in terms of their own functioning.

31 Minimum direct testing with the person A validated instrument for the cognitive assessment of dementia in people with intellectual disabilities e.g. NAID, CAMCOG-DS, SIB Prospective, short and long term memory (visual and verbal). Executive functioning. Orientation. Language (expressive and comprehension). Recording of evidence of new learning.

32 Executive functioning Increasing importance of assessing executive functioning No agreement on what tests to try, but several under development / trial Can use individual tests e.g. verbal/category fluency, response inhibition tests and set/rule switching tasks such as card sorting tests Informant scales may be useful

33 Informant interviews These should aim to cover those areas of function that are known to deteriorate with the development of dementia including: short and long term memory, general mental functioning, dyspraxia and dysphasia, daily living skills personality and behaviour e.g. DLD, DSDS, DSQIID, ABDQ

34 Other measures Psychological / mental health e.g. PAS-ADD, Anxiety / depression Carer burden Life events Adaptive functioning e.g. ABAS-II, HALO, Vineland, ABS important not just about quantitive change but qualitative change

35 Explaining assessments to people with ID Assessment process must be discussed with the person with intellectual disabilities, and that their consent to participate is obtained. Need to use different communication methods, tailored to the individual, including clear verbal communication and the use of picture booklets. Need to be related to people s prior understanding of the issues, and not to cause the person anxiety Emphasis on helping people to understand the process and the support available. Assessment of the person s capacity may need to be undertaken, and where the person does not have capacity, the decision re assessment needs to be made using a best interest process.

36 Sharing the diagnosis Establishing the diagnosis is often a complex process People with intellectual disabilities need to be told about their diagnosis of dementia and given ongoing opportunities to understand their diagnosis and their experience of dementia. Family members and carers need to be informed about the diagnosis and involved as much as possible in support and management plans and, as appropriate, be given opportunities for education and training. The person s peers and friends are also important people to involve in giving information about the diagnosis and this will both help them cope and help them support the person affected by dementia. People with intellectual disabilities and their families and carers may need psychological interventions to enable them to feel emotionally supported and to understand the diagnosis.

37 Breaking the news to the person Generic breaking-bad-news models do not meet the needs of people with intellectual disabilities so we need to communicate information over time and based on chunks. A Person-centred approach aims to support the person to understand and cope with their changing experiences We want to help them be involved as much as possible in decisions about their support and care and medical treatments, including future care. These chunks need to be appropriate to the person s current and changing framework of knowledge and lived experience and they need to take into account disturbed encoding and roll-back memory. Working with the Speech and Language Therapist Specific resources: The Journey of Life and About Dementia; What is dementia

38 Contact details Dr. Karen Dodd Surrey & Borders Partnership NHS Foundation Trust, Ramsay House, West Park, Epsom, Surrey KT19 8PB