The Occupational Therapist and Huntington’s Disease Clare Cook MSc, Dip COT Explain work for out pt neuropsychiatry service. HD one pt group we work with others include epilepsy, NEAD, somatoform disorders (Medically unexplained functional symptoms) CFS As a team we see approx 300 people with HD and these people form the largest part of my case load.

Session Outline Introduction to occupational therapy in HD Development of practice guidelines Research Due to time constraints this afternoon I will be looking very briefly at occupational therapy with people who have Huntington’s disease, I will explain about the development of the best practice guidelines for OTs that I have been working on, and some research that we are currently undertaking in our department.

“The basic goals of occupational therapy intervention for persons with dementing illnesses are (a) to maintain, restore or improve functional capacity; (b) to promote participation in activities that optimise physical and mental health; and (c) to ease caregiving activities.” (American OT association,1994) I thought I would start with this quote from the American OT association. This is rather an old quote but seemed to encapsulate what I try to do with people with HD who are referred to our service. To restore and improve function Promote participation in occupations Ease caregiving and provide support to carers I am aware that there are a few OTs in the audience and hope they would agree with this.

Where to find an OT Health and social care departments Wheelchair services Neurology clinics Inpatient units (mental health/physical health) Specialist teams Community mental health teams Palliative care teams The person with HD may come across OTs in these different areas and it is possible that more than one OT may be involved in care at any one time.

Why Have an OT in the Team? Trained in both mental health and physical health OTs specialise in occupational performance Maintaining skills and independence Of course there are many benefits to having an OT in your team but here are just a few. We focus on the things people do, we try to enable people to participate in daily activities, interests and roles within their home and community. This might include things like going to work, walking the dog, preparing a meal, getting dressed or catching the bus.

Interventions Vary according to: Particular difficulties experienced by the service user Which activities are meaningful and purposeful to the individual Social support Resources available Interventions vary here are a couple of examples of people I am working with at the moment: Give examples (Ian) Alan is in the late stages of HD and living in residential care, he has lost a lot of weight, his choreiform movts make it difficult to sit in a chair and he tries to get up out of the chair to walk but is no longer weight bearing. I have been working with the care staff to look at suitable seating, shower chair for this gentleman and supporting the manager in applying for continuing healthcare funding. (Brian) Dave is in the mid stages of HD. He has only most minor physical changes but numerous cognitive changes which have resulted in changes in his personality and behaviour. He lives in an upstairs flat with his wife who has a below knee amputation and uses a wheelchair. Brian enjoys walking, gardening and fishing but is unable to do these activities on his own, I have been working with a support agency to enable Dave to get out of the house more and participate in these activities and also monitoring triggers to his agitation and bursts of aggression whilst supporting his wife and family to manage this.

Strategies Compensatory Environmental Cognitive/behavioural (care givers) Case management/care coordination Maintaining safety Managing risk Compensatory strategies such as using calendars, reminders, labels to compensate for changes in organisational and planning skills. Environmental changes such as recommending level access showers or converting a downstairs room into a bedroom when the stairs become too dangerous, As a team we do not discharge people with HD so in many cases the OT takes on the role of care co-ordinator and regularly reviews the service user to monitor any changes in needs.

Where’s the evidence? So, where is the research evidence to support the need for occupational therapy in teams? Well, there is very little research to support the use of occupational therapy with people with HD as I have discovered trying to put together some best practice guidelines for OTs.

This is the document so far… This document is being developed as part of the EHDN, standards of care OT group. The groups consisted mainly of OTs from the UK with 2 ots from Sweden, one from the States and e mail contact with 2 OTs in Holland.

Systematic Literature Review Medline Embase Cinahl Amed PsychInfo Cochrane library Internet search (Google scholar) We started by carrying out a systematic review of the current literature using these data bases.

Search Terms Huntington’s disease, AND Occupational therapy / occupation / independence / rehabilitation / activities of daily living / adaptations / home adaptation / equipment / leisure / work / employment / feeding / posture / seating / wheelchairs / splinting / sensory / multi-sensory / manual handling.   And using these search terms

Findings 10 Articles No RCTs Pilot studies Case studies We found 10 articles No RCTs One pilot study and several case studies

Effectiveness of physiotherapy, occupational therapy and speech pathology for people with Huntington’s disease: A systematic review (Bilney et al 2003) I wont go through all the articles but One of the articles was this one by Bilney

3 articles on OT ’71,’81, ’91, no RCTs Small numbers of participants All in latter stages of disease in nursing homes Showed no improvement in function but unable to show if deterioration had been prevented Need for further research He found 3 articles all very old – it seems from this article that research into therapy and HD only takes place on a 10 yr cycle Small number of participants Late stage HD Identified need for further research

Community based OT for patients with dementia and their caregivers: randomised controlled trial. (Graff et al 2006) In order to write our guidelines we consulted literature from other diagnostic groups such as dementia.

10 sessions of OT over 5 weeks compared to a control group of no OT 135 participants 10 sessions of OT over 5 weeks compared to a control group of no OT Outcome measures – AMPS, IDDD,SCQ Significant improvement in scores which remained significant after 3 months Assessment of Motor and Process Skills Interview for Deterioration in Daily living skills in Dementia Sense of Competence Questionnaire

“occupational therapy will result in less dependence on social and healthcare resources and less need for institutionalisation” (Graff et al 2006) They conclude that…

Guidelines Part 1 Process of developing the guide Background Information So, back to the guidelines 2 parts Section one looks at the process of developing the guidelines and includes a section from a carer of one of our service users. It also look at an overview of HD including the aetiology, prevelance, signs and symptoms.

Part 2 Supporting Participation Strategies… Optimising activities Self Efficacy Roles & relationships Work Social Recreational and Leisure activities Driving Community living skills and Outdoor mobility Strategies… Physical Factors Cognitive Factors Behavioural Factors Optimising activities Mobility and falls prevention Transfers and hoisting Bed mobility and sleep safety Postural management Eating & drinking Self care Domestic skills Fatigue End of life care 24 hour postural management Manual handling and minimizing risk Alternative living arrangements Section two is the actual guidelines which start by looking at strategies for physical, cognitive and behavioural factors impacting on engagement, motivation and learning. Which includes things such as break down complex tasks, use calendars, lists, use prompts to aid retrieval, eliminate external stimuli, identify triggers to irritability and avoid confrontations. It then goes into optimising activities which concentrates on personal care issues, transfers, mobility, domestic tasks etc Supporting participation considers wider issues of relationships, coping with accessing community resources, work and driving And the final section looks at 24 hr postural management, manual handling and minimising risk and alternative living arrangements. We also include mental capacity and power of attorney in this section but I think this may move to an earlier part of the document as it should be considered and revisited throughout the disease trajectory rather than at the end stage.

Current Challenges Evidencing statements Ratification Publication Lack of literature as mentioned earlier Basing doc on Expert concensus to evidence statements – through our standards of care group and peer review at the COT conference last June. Ratification COT, COTEC, HDA Publication of whole doc on EHDN, COT Abbreviated doc in conjunction with other AHPs in SoC group ie SLT, Dietitans, dentists

Need for more research into Huntington's disease and occupational therapy The process has really highlighted the need for good quality research into OT and HD

A Study to validate the Assessment of Motor and Process Skills (AMPS) for use with people who have Huntington’s disease To start this process in Birmingham we have employed a research OT to carry out a study to validate the AMPS for use with people with HD.

The Assessment of Motor and Process Skills (AMPS) An observational assessment Used to measure the quality of ADL performance Standardized on over 100,000 clients worldwide Just a quick outline of this measure The AMPS is an OT tool which is an observational assessment, used to evaluate people in the context of familiar and relevant tasks. The AMPS is used to measure the quality of task performance. The AMPS is a standardized assessment that can be used with clients aged three or older, with any diagnosis or disability. It is currently in use in Europe, North and South America, Asia, Nordic Countries, Australia, New Zealand, Israel, the United Kingdom, and South Africa.

An AMPS Assessment Begins with an interview to determine the tasks that are familiar and relevant to the client There are 85 standardized AMPS tasks, ranging from easy to hard Includes the observation of at least two chosen ADL tasks A core concept in the AMPS is the importance of client choice, as engagement in chosen and meaningful activities is at the core of occupational therapy. Client motivation and performance is believed to be best when he or she can choose the activity. An AMPS assessment begins, therefore, with an interview of the client so that the therapist can determine which standardized AMPS tasks are familiar, relevant and of sufficient challenge to the client. For an AMPS assessment, the client at least 2 AMPS tasks to perform from a subset of tasks offered by the therapist. The therapist must also ensure that the tasks offered as choices are hard enough to offer a “just right” challenge to the person – not too easy and not too hard. AMPS tasks include a variety of ADL tasks – personal ADL (eating, dressing, grooming, etc) and domestic ADL (simple to complex cooking, home maintenance, outdoor tasks, etc)

The AMPS uses Rasch analysis to analyse the data The AMPS uses Rasch analysis to analyse the data. This process takes into account the task difficulty and the rater severity to produce these graphs. It is hard to see the detail but this is the person’s score for ….. Yellow bar is the range for people without a disability in this age group.

Research Study Test/retest Participant vs. control Relationship with similar scales The AMPS has been widely used and researched with a variety of conditions including dementia, MS and brain injury but no studies so far have validated its use in HD. I think that this is a more sensitive tool to use to assess everyday performance skills and will give more detailed and useful information than existing measures such as Total functional capacity or the Functional Assessment currently used in battery of tests used within the EHDN registry. The study design will include: a test/retest design to look at the sensitivity of the tool on repeated measures Participant vs control – to look at specific performance skill areas of people in late premanifest, early and middle stage of the disease (suspect may show difficulties in temporal organisation skills and organisation of space and objects but will retain skills associated with applying knowledge.) Look at how AMPS compares with similar scales such as physical performance test, TFC, Mohost, registry scores (Motor and Cog)

Hypotheses AMPS is a valid assessment to use with people who have HD AMPS is a sensitive measure which can detect change in performance skills AMPS could potentially be used to assess the impact of disease modifying drugs on a person’s ADL performance skills Go through slide I believe that there is a role for occupational therapists to work with people with HD across the disease trajectory. This role changes with the person’s needs and as OTs we need to demonstrate the work we do using good quality qualitative and quantitative research. I know that I have not really spoken much about my role clinically as an OT but I wanted to share with you some of the other parts of my role in developing our service.. I am happy to answer any questions now or during the panel discussion.

Thank you for listening clare.cook@bsmhft.nhs.uk