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)
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Swallowing impairment can endanger life, result
in malnutrition and /or dehydration
(DYSPHAGIA)
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Either condition affects quality of life, limiting
social interaction and impacting on health and
well-being.
What an SLT sees:
chorea
Word-finding
difficulty
fatigue
Weak
voice
frustration
Slow verbal
processing
akathisia
Facial expression
limited
H
D
clumsiness
ataxia
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
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How HD affects communication
Dysarthria: Involuntary movements +
impaired co-ordination of voluntary
movements lead to
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Poor co-ordination of breathing and
voice, affecting volume, and pacing
of speech
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Pitch variation of speech reduced
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Reduced co-ordination of oral and
facial muscles, affecting
intelligibility of speech and facial
expression.
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Unpredictable speech patterns,
further reducing intelligibility.
Impaired Language processing:
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Comprehension of words is
generally retained but slows down
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This affects memory of the message
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So “information overload” happens
more easily
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Retrieval of vocabulary is impaired
(word-finding difficulty)
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Impacting on speed and accuracy of
responses/information –giving
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Verbal communication generally
becomes more effortful.
Plus initiation of verbal responses becomes impaired
How HD affects eating, drinking and
swallowing
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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
o
Sooner or later oral intake becomes an issue for
people with HD.
o
Independence
Social interaction
Health –nutrition, hydration,medication,risk management
Assessing risk
Supporting feeding –when, how, what?
Supplements
Enteral feeding- decisions about P.E.G
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Decisions involve SLT, Physio, OT, Dietetics,
Nursing, carers/family as well as doctors.
SLT within the MDT
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Role :not comprehensive and subject to variation!
 To support team in providing optimal and timely care
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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
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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?
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Assess- HD is both progressive and dynamic; individual baselines needed
formal/informal assessment for communication and
eating,drinking,swallowing
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Advise- guidelines for support and risk management-to person and carers
strategies for person with HD
Carers/families
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Liaise- wherever and whenever needed- regular exchanges of
information essential to keep track of changes.
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Train – most of my colleagues in Oxfordshire will see one person with
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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
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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
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SAFETY: majority of deaths are from choking/aspiration leading to
pneumonia
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FUNCTION: use it or lose it –maintaining movement also sustains
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sensation
Withdrawal from communication enhances difficulty
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QOL:
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People with HD need to know that they are still members of the human
race
Eating, socialising, communicating –essential pleasures.
What Is the right time for SLT intervention?
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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
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*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
Person with HD
Effort,
concentration, loss
Variability, lack
of initiative
Less depth in
conversation
Family members
Change in
understanding,
need for
adjustment
Carers
How HD had changed communication for
participants
Negative influences
People with HD
Speed of others’
communication
Emotional Load,
depression,
stress
Fewer people to
talk with
Family members
Personality change
Lack of eye
contact
Timing of
information
Carers
What each group found helpful
Positive influences on communication
People with HD
Speaking more
Support
Stimulation
Family members
Need for
increased
participation
Sense of
community,
Trust
Activities,
memories,
questions
Carers
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
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People with HD need a multi-disciplinary approach
They are more likely to work with the MDT if first contact is presymptomatic
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
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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