Presentation on theme: "Speech and Language Therapy in Huntington’s Disease"— Presentation transcript:
1 Speech and Language Therapy in Huntington’s Disease Rita Twiston Davies, Clinical Lead SLTOxfordshire Health NHS Foundation Trust
2 Why Speech and Language Therapy? As the most highly co-ordinated human activitySpeech 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.
3 HD What an SLT sees: chorea ataxia Word-finding difficulty fatigue frustrationWeak voiceSlow verbal processingakathisiaHDataxiaclumsinessFacial expressionlimiteddepressionrigiditychoreadysarthriadysphagiaMuscle weaknessLoss of voluntary movement control
4 Clinical featuresLike Parkinson’s disease: loss of facial expression, shuffling gait, festination and “freezing”, impairment of voluntary movementLike MS: disease progresses in bouts of deterioration followed by periods of stabilityLike MND: muscle weakness and fatigueSensory impairment implicit in muscular impairmentSlowness of cognitive processing but verbal comprehension preserved to varying degrees
5 How does working with people with HD differ? Motivation/complianceDisease progressionCombination of physical, psychiatric/psychological featuresLong time-scale of the diseaseFamilies
6 How HD affects communication Dysarthria: Involuntary movements + impaired co-ordination of voluntary movements lead toPoor co-ordination of breathing and voice, affecting volume, and pacing of speechPitch variation of speech reducedReduced 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 downThis affects memory of the messageSo “information overload” happens more easilyRetrieval of vocabulary is impaired (word-finding difficulty)Impacting on speed and accuracy of responses/information –givingVerbal communication generally becomes more effortful.Plus initiation of verbal responses becomes impaired
7 How HD affects eating, drinking and swallowing Increased calorie intakeEffects of medication- reflux, nausea etcIncoordination of oro-facial muscles andRespiration /swallow timingSensory impairmentLonger mealtimes/increased fatigueReduced independence- supported feedingReduced oral hygiene –tooth brushing difficultReduced ability to protect airway causing increased risk ofairway penetration/chokingIncreased risk of aspiration leading to chest infectionDecreased inhibition related to challenging behaviour around mealtimes.
8 Feeding decisions in HD- an MDT approach Sooner or later oral intake becomes an issue for people with HD.IndependenceSocial interactionHealth –nutrition, hydration,medication,risk managementAssessing riskSupporting feeding –when, how, what?SupplementsEnteral feeding- decisions about P.E.GDecisions involve SLT, Physio, OT, Dietetics, Nursing, carers/family as well as doctors.
9 SLT within the MDT Role :not comprehensive and subject to variation! To support team in providing optimal and timely careTo assess and give appropriate strategies for managing risk arising from behaviour (communication)/dysphagiaTo support person with HD as required, in a manner acceptable to themTo monitor disease progression as it affects communication and /or dysphagia and offer intervention/support whenever appropriateTo support families/carers in managing risk and ensuring well-being as far as possibleTo be there when needed – the end and the beginning of all our roles
10 InteractionTeamwork is essential for any complex condition. My key people from the wider team:Physios = information about muscle tonepositioning, especially for mealsHydro –a good place for voice /breathing work!OTs= adaptive equipmentsensory and cognitive function information/discussionall 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
11 What does an SLT do? HD a year, at the outside. Assess- HD is both progressive and dynamic; individual baselines neededformal/informal assessment for communication and eating,drinking,swallowingAdvise- guidelines for support and risk management-to person and carersstrategies for person with HDCarers/familiesLiaise- 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 withHD a year, at the outside.Treat? In HD this is not a given. Audit needed of uptake of SLT with people with HD.
12 Evidence base for SLT intervention Evidence base? What evidence base?GothenburgFOTTRosenbek and JonesEHDN Standards of Care Working Party guidelinesNeed for research into efficacy of management techniques
13 Aims of SLT for people with HD SAFETY: majority of deaths are from choking/aspiration leading to pneumoniaFUNCTION: use it or lose it –maintaining movement also sustainssensationWithdrawal from communication enhances difficultyQOL: Eating, socialising, communicating –essential pleasures.People with HD need to know that they are still members of the human race
14 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 infectionsDO refer as early as possible after diagnosis, preferably when still pre-symptomatic
15 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 initiateVolume 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.
16 Recent communication research: Results of Gothenburg study into effects of HD (IJLCD 2011) Person with HDEffort, concentration, lossVariability, lack of initiativeLess depth in conversationChange in understanding, need for adjustment
17 How HD had changed communication for participants People with HDSpeed of others’ communicationEmotional Load, depression, stressFewer people to talk withLack of eye contactTiming of informationPersonality change
18 What each group found helpful People with HDSpeaking moreSupportNeed for increased participationSense of community, TrustActivities, memories, questionsStimulation
19 Supporting communication Low tech aidsPeople to talk withPicture shopping listsWeekly planners/activity chartsCommunication chartsCommunication/life booksMemo boardsPhoto remindersTalking MatsActivities!Not so low-tech aidsButtons/ switchesVoice amplifiersVOCAsComputers with :KeyguardsSwitch accessPredictive lexicons
20 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 drinksHow she feltYes/No.Here she is being shown pictures to indicate mood: happy, sad, so-so.
21 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.
22 Summary People with HD need a multi-disciplinary approach They are more likely to work with the MDT if first contact is pre-symptomaticIntervention will be sporadic, according to disease progressionOutcomes 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.
23 ReferencesIJLCD 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 DisordersPubl; Plural Publishing -Clinical Dysphagia series- OxfordEuropean Huntington’s Disease Network: Standards of Care Working Group – in preparation.Huntington’s Disease Association publications: Communication; Eating and Drinking