Speech and Language Therapy in Huntington’s Disease Rita Twiston Davies, Clinical Lead SLT Oxfordshire Health NHS Foundation Trust

Why Speech and Language Therapy? As the most highly co-ordinated human activity Speech is often affected relatively early in the disease. (DYSARTHRIA) Swallowing impairment can endanger life, result in malnutrition and /or dehydration (DYSPHAGIA) Either condition affects quality of life, limiting social interaction and impacting on health and well-being.

HD What an SLT sees: chorea ataxia Word-finding difficulty fatigue frustration Weak voice Slow verbal processing akathisia HD ataxia clumsiness Facial expression limited depression rigidity chorea dysarthria dysphagia Muscle weakness Loss of voluntary movement control

Clinical features Like Parkinson’s disease: loss of facial expression, shuffling gait, festination and “freezing”, impairment of voluntary movement Like MS: disease progresses in bouts of deterioration followed by periods of stability Like MND: muscle weakness and fatigue Sensory impairment implicit in muscular impairment Slowness of cognitive processing but verbal comprehension preserved to varying degrees

How does working with people with HD differ? Motivation/compliance Disease progression Combination of physical, psychiatric/psychological features Long time-scale of the disease Families

How HD affects communication Dysarthria: Involuntary movements + impaired co-ordination of voluntary movements lead to Poor co-ordination of breathing and voice, affecting volume, and pacing of speech Pitch variation of speech reduced Reduced co-ordination of oral and facial muscles, affecting intelligibility of speech and facial expression. Unpredictable speech patterns, further reducing intelligibility. Impaired Language processing: Comprehension of words is generally retained but slows down This affects memory of the message So “information overload” happens more easily Retrieval of vocabulary is impaired (word-finding difficulty) Impacting on speed and accuracy of responses/information –giving Verbal communication generally becomes more effortful. Plus initiation of verbal responses becomes impaired

How HD affects eating, drinking and swallowing Increased calorie intake Effects of medication- reflux, nausea etc Incoordination of oro-facial muscles and Respiration /swallow timing Sensory impairment Longer mealtimes/increased fatigue Reduced independence- supported feeding Reduced oral hygiene –tooth brushing difficult Reduced ability to protect airway causing increased risk of airway penetration/choking Increased risk of aspiration leading to chest infection Decreased inhibition related to challenging behaviour around mealtimes.

Feeding decisions in HD- an MDT approach Sooner or later oral intake becomes an issue for people with HD. Independence Social interaction Health –nutrition, hydration,medication,risk management Assessing risk Supporting feeding –when, how, what? Supplements Enteral feeding- decisions about P.E.G Decisions involve SLT, Physio, OT, Dietetics, Nursing, carers/family as well as doctors.

SLT within the MDT Role :not comprehensive and subject to variation! To support team in providing optimal and timely care To assess and give appropriate strategies for managing risk arising from behaviour (communication)/dysphagia To support person with HD as required, in a manner acceptable to them To monitor disease progression as it affects communication and /or dysphagia and offer intervention/support whenever appropriate To support families/carers in managing risk and ensuring well-being as far as possible To be there when needed – the end and the beginning of all our roles

Interaction Teamwork is essential for any complex condition. My key people from the wider team: Physios = information about muscle tone positioning, especially for meals Hydro –a good place for voice /breathing work! OTs= adaptive equipment sensory and cognitive function information/discussion all the “techie “ bits I can’t do in computer sessions with patients! Dietician =should be joined at the hip for HD! Menus, textures,weight management, supplements etc etc Nurses= Who are THERE and KNOW! Music/ Art therapists= who help me think “outside the box” about facilitating communication

What does an SLT do? HD a year, at the outside. Assess- HD is both progressive and dynamic; individual baselines needed formal/informal assessment for communication and eating,drinking,swallowing Advise- guidelines for support and risk management-to person and carers strategies for person with HD Carers/families Liaise- wherever and whenever needed- regular exchanges of information essential to keep track of changes. Train – most of my colleagues in Oxfordshire will see one person with HD a year, at the outside. Treat? In HD this is not a given. Audit needed of uptake of SLT with people with HD.

Evidence base for SLT intervention Evidence base? What evidence base? Gothenburg FOTT Rosenbek and Jones EHDN Standards of Care Working Party guidelines Need for research into efficacy of management techniques

Aims of SLT for people with HD SAFETY: majority of deaths are from choking/aspiration leading to pneumonia FUNCTION: use it or lose it –maintaining movement also sustains sensation Withdrawal from communication enhances difficulty QOL: Eating, socialising, communicating –essential pleasures. People with HD need to know that they are still members of the human race

What Is the right time for SLT intervention? NOW! Don’t wait for behavioural change due to frustration at communication difficulties! Don’t wait for person to choke/become malnourished/dehydrated/ have repeated chest infections DO refer as early as possible after diagnosis, preferably when still pre-symptomatic

How HD progression limits communication *Body posture is altered by choreic movements and impoverished control of voluntary movements- affecting gestural support for speech *The eyes remain a primary source of communication but impaired postural control may limit this *Facial expression becomes more limited and chorea may resultin grimacing *Speech becomes harder to initiate Volume and pitch of voice are harder to control and intonation is less varied – no subtlety in meaning *Awareness of the subtexts of other people’s communication is impaired – their tone of voice, facial expression etc. *Finding the right word is impaired –affecting the fluency of the message/ losing the thread completely *Language processing slows down *Information can be retained, if given in a manageable way. *Verbal reasoning is likely to be impaired- affecting capacity.

Recent communication research: Results of Gothenburg study into effects of HD (IJLCD 2011) Person with HD Effort, concentration, loss Variability, lack of initiative Less depth in conversation Change in understanding, need for adjustment

How HD had changed communication for participants People with HD Speed of others’ communication Emotional Load, depression, stress Fewer people to talk with Lack of eye contact Timing of information Personality change

What each group found helpful People with HD Speaking more Support Need for increased participation Sense of community, Trust Activities, memories, questions Stimulation

Supporting communication Low tech aids People to talk with Picture shopping lists Weekly planners/ activity charts Communication charts Communication/life books Memo boards Photo reminders Talking Mats Activities! Not so low-tech aids Buttons/ switches Voice amplifiers VOCAs Computers with : Keyguards Switch access Predictive lexicons

Low Tech Communication support – an example The lady in this picture was diagnosed with HD over 20 years previously. She showed such enthusiasm for a Life Book, produced with the support of the SLT Assistant and ward staff, that we tried using A5 size pictures so she could communicate what mattered to her at that time: Her choice of drinks How she felt Yes/No. Here she is being shown pictures to indicate mood: happy, sad, so-so.

A Higher-Tech approach This man has good verbal skills but is showing early impairment of language so his voice has been recorded, using everyday requests and information that he has selected, on a Go-Talk aid. He is learning to use pictures to link to these phrases as the time is approaching when he will find it hard to read and pictures will facilitate his use of a VOCA such as this.

Summary People with HD need a multi-disciplinary approach They are more likely to work with the MDT if first contact is pre-symptomatic Intervention will be sporadic, according to disease progression Outcomes are seen in what hasn’t happened- no chest infections- as well as in what has –still able to self-feed, for example. This disease affects everyone differently – broad patterns apparent but timing of onset, rate of progression, personal reactions etc vary. Effective Communication is key, for the individual and for their support network. Gene therapy/cure still a long way off –research into effective management lacking and needed.

References IJLCD 2011: papers by Hartelius et al and Ulrika Ferm on communication in HD and on Use of Talking Mats in HD. Yorkston, Miller, Strand 1995:Management of speech and swallowing in degenrative diseases. Publ; Pro-Ed, Austin Texas. Rosenbek and Jones 2009: Dysphagia in Movement Disorders Publ; Plural Publishing -Clinical Dysphagia series- Oxford European Huntington’s Disease Network: Standards of Care Working Group – in preparation. Huntington’s Disease Association publications: Communication; Eating and Drinking