Speech and Language Therapy in Huntington’s Disease

Rita Twiston Davies, Clinical Lead SLTOxfordshire Health NHS Foundation Trust

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.

What an SLT sees:chorea fatigue

dysarthria

ataxia

dysphagiaMuscle

weakness

rigidity

HD depression

Facial expressionlimited

Word-finding difficultyWeak

voice

akathisiaclumsiness

frustration

chorea

Loss of voluntary movement control

Slow verbal processing

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 fatigueSensory impairment implicit in muscular

impairmentSlowness 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 communicationDysarthria: 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 approacho Sooner or later oral intake becomes an

issue for people with HD. o Independenceo Social interactiono Health –nutrition, hydration,medication,risk managemento Assessing risko Supporting feeding –when, how, what?o Supplementso Enteral feeding- decisions about P.E.Go

o 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?

• 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?

o NOW!o Don’t wait for behavioural change due to

frustration at communication difficulties!o Don’t wait for person to choke/become

malnourished/dehydrated/ have repeated chest infections

o 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)

How communication has changed for the individual

CarersFamily members

Effort, concentration, loss

Less depth in conversation

Change in understanding, need for adjustment

Variability, lack of initiative

Person with HD

How HD had changed communication for participants

Negative influences

CarersFamily members

Speed of others’ communication

Fewer people to talk with

Personality change

Lack of eye contact

Emotional Load, depression, stress

Timing of information

People with HD

What each group found helpful

Positive influences on communication

CarersFamily members

People with HD Speaking more

Support Sense of community, Trust

Stimulation

Activities, memories, questions

Need for increased participation

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 :KeyguardsSwitch accessPredictive 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 drinksHow she feltYes/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