The Behavioural and Psychological Symptoms of Huntington’s Disease

The Behavioural and
Psychological Symptoms
of Huntington’s Disease
- A practical guide to assist in caring for a
person with HD
This project has been produced by The Academic Unit for
Psychiatry of Old Age in conjunction with NARI and was generously
supported by a University of Melbourne Dreamlarge Knowledge
Transfer Project Grant
1
For more information
Contact Dr Anita Goh
goha@unimelb.edu.au
9816 0513
Fax: 9816 0477
Acknowledgements
We would like to acknowledge the participation of the Australian
Huntington’s Disease community on this project.
A note to the reader
The case studies contained in this booklet are taken from several
sources, including from our research, focus groups with people
with HD and health professionals, from the available literature, and
also adapted from actual real-life cases. In each case names and
identifying features have been changed in order to protect privacy.
The opinions expressed in this booklet are those of the contributing
authors and not to be construed as the opinions or
recommendations of the publishers or sponsors.
Full prescribing information must be consulted on any of the
medications or procedures described herein, and the advice does
not replace the advice of your health professionals.
2
About NARI
The National Ageing Research Institute (NARI) aims to be the
centre of excellence in Australia for research into ageing and
improving the quality of life and health of older people. NARI
conducts research in the community, hospital and residential care
settings. NARI holds expertise in:
• clinical research and application of research to best practice
• Preventive and public health, and health service evaluation
• Community and professional education
• Clinical research into diseases of ageing
A pioneer in Australia for ageing research, NARI is recognised
nationally and internationally for its work in several key fields.
NARI’s approach to its mission is closely linked to the values and
priorities of older people.
http://www.mednwh.unimelb.edu.au/
About AUPOA
The Academic Unit for Psychiatry of Old Age (AUPOA) is a unit in
the Department of Psychiatry, the University of Melbourne, and
also St Vincent’s Aged Mental Health Service (SVAMHS). AUPOA
provides clinical leadership, ongoing training, and knowledge
transfer and service to the community. As a recognised leader in
research and innovation in Psychiatry of Old Age the AUPOA
conducts ongoing upskilling and training programs for GPs, health
professionals and other service providers. The Academic Unit
operates the Normanby Unit SVAMHS clinics including the Speech
and Communication Clinic, Memory and Related Disorders Clinic,
HD clinic and Psychotherapy Clinic. Through the SVAMHS Research
Clinic the unit conducts national and international clinical research
projects, which provides access to new evidence-based treatments
and participation in national and international research studies.
http://www.aupoa.unimelb.edu.au/
3
Foreword
Huntington’s disease (HD) is a profoundly incapacitating, and
ultimately fatal, neurodegenerative disease. HD is presently
incurable, so the current goal is to allow affected individuals to live
as well as possible with the illness, to maximise functional
independence and quality of life for the person with HD and their
carers and family members.
The Behavioural and Psychological Symptoms of HD (BPSHD) are
often not well understood by family, health professionals and the
community, but have been considered the presenting symptoms of
HD in up to half of all people with HD, and can precede the classical
motor symptoms by up to a decade. BPSHD are often the most
distressing part of the condition and thus a good understanding of
these are crucial to good clinical practice, and so that those
involved can be effectively informed how to manage these stressful
and common manifestations of HD. A more thorough
understanding of the BPSHD is also essential as they are often the
most amenable to targeted treatment and intervention (both nonpharmacological and pharmacological), which can lead to a better
quality of life for the people living with HD and also for family
members and carers, and also potentially dramatically delay
institutionalisation and reduce high health care costs.
This booklet develops our research and over 30 years of
accumulated clinical knowledge through our HD clinic (Professor
Edmond Chiu) to provide a much-needed and accessible resource.
It is designed to raise the profile and improve the recognition,
understanding and management of the BPSHD amongst health
professionals and HD community. It is written for people affected
by HD, which includes family members (partners, children, parents,
sisters, brothers), friends, caregivers and those professionals who
give advice and care to these communities. We hope that this
booklet contributes to the improved management and quality of life
for those affected by HD.
Dr Anita Goh
Chief Investigator
4
Contents
1. Huntington’s Disease
5
1.1 What is HD?
2. The BPSHD
2.1
2.2
2.3
2.3
2.4
2.5
What are the BPSHD?
Causes of the BPSHD
Neuropathology in the BPSHD
Cognitive Deficits in the BPSHD
Psychological Contributors to the BPSHD
Social and Environmental Contributors to the
BPSHD
3. The Common BPSHD
3.1
3.2
3.3
3.4
6
15
Affective Symptoms
Perceptual symptoms & Mania
Personality Symptoms
Unawareness, Denial, & Lack of Insight
4. Treatment and Management of the BPSHD 36
4.1
4.2
The role of Caregivers
Strategies for dealing with the BPSHD
5. Guide to identifying and measuring the BPSHD
47
6. The Research Team
50
7. Resources
52
8. Further Reading
56
5
1. Huntington’s Disease
1.1 What is HD?
Reproduced
from
the
'Huntington
number'
http://www.nature.com/nrg/journal/v6/n10/images/nrg1686-f1.jpg
of
Neurographs
Huntington’s disease is a currently incurable genetic
neurodegenerative disorder that usually manifests in early to
middle adulthood. The genetic abnormality consists of expansion
(over 36) in the trinucleotide repeat CAG in the HD gene on
chromosome four, which codes for the normal protein huntingtin.
HD is considered a tripartite neurodegenerative disorder, with
motor, cognitive, and psychiatric changes. Common symptoms are
uncontrollable movements, poor balance, slurred speech, difficulty
swallowing, thinking (cognitive) difficulty, and personality change.
However, despite significant well-known behavioural and
psychological symptoms of HD (BPSHD) being highly prevalent, it is
typically diagnosed on the basis of family history, motor symptoms,
with progression and severity of the illness generally measured by
motor deterioration and detailed neuropsychological assessment of
cognition.
6
2. The Behavioural and
Psychological Symptoms of HD
(BPSHD)
2.1 What are the BPSHD?
Source http://i41.tinypic.com/5upz4m.jpg
BPSHD are part of the disease process and present severe problems
to those with HD, their families and caregivers, and wider society.
Apathy, depression, lack of insight, disinhibition, impulsivity,
obsessive and compulsive symptoms, anxiety, frustration,
irritability, and aggression are all common BPSHD. Social conduct
is often altered and there is frequently severe breakdown in
relationships. People with BPSHD are often unfairly stigmatised as
self-centred, lacking in sympathy and empathy and mentally
inflexible.
7
KEY POINTS
• Various BPSHD occur at different phases of the illness
• Not all symptoms are experienced by all
• Symptoms can wax and wane
• The environment can contribute to the BPSHD
• Detailed knowledge of the BPSHD could lead to earlier and
more accurate diagnosis and appropriate management
2.2 Causes of the BPSHD
There are multiple causes of the BPSHD, with underlying factors
including a combination of the following:
1. Neurobiological
factors
–
neurochemical
and
neuropathology, current health status, presence of
coexistent medical or psychiatric syndromes, such as
delirium, urinary tract infections, arthritis, insomnia,
dehydration.
2. Cognitive factors – cognitive impairment
3. Psychological factors – personality traits, response to stress
4. Social factors – changes in family structure, leaving work,
changes in routine, stigmatisation and exclusion
5. Environmental factors – changes in caregiver, moving
house, unmet needs of the person with HD, such as the
presence of pain, wetness, fatigue, or fear.
Chapter 2: the BPSHD
The BPSHD can result in:
• suffering,
• increased costs of care
• premature institutionalisation
• excess disability (i.e. people with BPSHD function at a lower
level than those without)
• significant loss of quality of life for the person with HD and
their family, friends, and caregivers.
8
2.3 Neuropathology in the BPSHD
Many of the BPSHD are similar in people with HD due to the part of
the brain affected by the disease. There have been studies that
suggest that the BPSHD are not simply the result of wide-ranging
brain pathology, but are due to damage to specific areas of the
brain. However, although the BPSHD generally have an organic
basis, there are no definite conclusions linking specific BPSHD to
specific changes in the brain. Much more research is necessary.
Chapter 2: the BPSHD
The main site of HD neuropathology is the basal ganglia, in
particular the caudate nucleus. This is located deep in the brain
and has multiple connections with other parts of the brain.
Functions include regulating information, and involvement in
mood, cognition, and motor skill. The caudate has been found to
have many connections with the frontal lobes.
SOURCE: http://www.colorado.edu/intphys/Class/IPHY3730/image/figure5-31.jpg
Damage to the caudate nucleus and its circuits contribute to the
BPSHD. The primary functions of the caudate is to regulate, control
and direct information in the brain, and as the caudate degenerates,
the brain struggles to regulate how much movement, how much
9
feeling, and how much thinking is required in a certain situation.
So when the circuits are not working as they should, the flow and
transfer of messages are impaired. As HD progresses and more
brain cells die in these areas and more circuits are damaged, neural
processes become even more difficult. For example, because the
caudate acts to regulate information, if it is faulty, as in HD,
everyday behaviours can become problematic: being slightly angry
or annoyed is normal, but full blown temper tantrums are not;
worrying or being a little concerned about something is also
normal whereas being obsessional about something can be a
problem.
Chapter 2: the BPSHD
MRI scans are commonly taken to see the brain structures affected
in HD. The picture below shows severe shrinkage of putamen and
caudate nucleus in a person with Huntington’s disease (right)
compared with a person with no disease (left). The relevant areas
are traced with white, and the much smaller caudate in the person
with HD is noticeable.
Source: http://www.psychopharma.eu/?p=252
MRI image showing severe atrophy of putamen and caudate nucleus in
a person with Huntington’s disease (right) compared with someone
with no disease (left)
Source: http://hdroster.iu.edu/AboutHD/brainAndHD.asp
Above is an image in which a slice of a brain affected by HD (left) is
next to a slice from a brain from someone without HD (right). The
striatum (the caudate nucleus, putamen, and nucleus accumbens),
on the left, is severely shrunken in the brain of the person affected
by HD. The cerebral cortex is also shrunken.
2.3
Cognitive Deficits in the BPSHD
The cognitive (thinking) functions generally affected in HD are
judgment, communication, slowed processing of information in the
brain and especially affected are executive functions (see later). As
the disease progresses, memory deficits tend to appear. Cognitive
problems tend to worsen over time and as the disease progresses,
the ability to concentrate becomes more difficult and the person
may have difficulty keeping track of things, making decisions,
Chapter 2: the BPSHD
10
11
answering questions, and may lose the ability to recognize familiar
objects.
Executive function skills are those skills that help us to organise,
sequence, prioritise, and multi-task, and are crucial in planning, self
monitoring, flexibility, attention, abstract thinking, judgement,
problem solving, rule acquisition, initiating appropriate actions,
and inhibiting inappropriate actions. As the caudate deteriorates,
so do its connections to the frontal lobes, the areas of the brain
which are key to executive functions. Things can become mixed-up
without the master organiser and take much longer than usual, and
people with HD often become easily overwhelmed by too much
information or stimulation because of the frontal lobe dysfunction.
People with HD often have difficulty learning new things, planning
and prioritizing, and multitasking.
Leo struggles to sequence tasks, especially when distracted,
whilst his ability to form new memory and to recall old
memories is relatively well preserved. Thus Leo is able to do the
laundry with a list of instructions posted in the laundry room,
but cannot manage whilst also trying to make a meal or talk on
the phone.
Attention. Through its connections to the frontal lobes, the caudate
is also involved in divided attention, and in filtering information as
it arrives. Disruptions of this circuit results in difficulties in
regulating the flow of incoming information and thus can result in
being easily overwhelmed, difficulty doing more than one thing at a
time, and difficulty switching attention from one thing to another.
Example
Distracting influences reduce Mischa’s ability to accomplish
tasks. For example, she is able to eat easily in a quiet
environment, but has great difficulty eating in a noisy, crowded
room with both a television set and a radio playing.
Chapter 2: the BPSHD
Example
12
Chapter 2: the BPSHD
Regions of the Brain
Source: http://www.unlimitedbrain.com/maps_program/brain_tools_functions/
13
Memory. The memory problems in HD are typically characterised
by impairments in initially learning new information, and also in
recalling learned information. These memory deficits are likely to
be secondary to the executive function problems, which result in
inefficiencies in the skills of organising and sequencing to-beremembered information.
Communication. As Huntington's disease advances the ability to
communicate diminishes. Common problems include difficulty
pronouncing the words due to dysarthria, with poor articulation
and slurring of words, slowing in the production of words, poor
initiation of speech, difficulty organising their thoughts to be
converted into speech, and comprehension of what is being
communicated to them. People with HD also commonly have
difficulty finding the right words to use and take longer than usual
to communicate and respond. Due to the damage to the motor
areas of the brain, speech is often disorganised, poorly timed, and
poorly sustained by breath. As words become sparser, the content
is still there, but often lacking pronouns and adjectives. Eventually
the person may not be able to use speech to communicate.
Communication problems are often the source of much frustration
and exhaustion, for both the person with HD and the other person
in the conversation. Many BPSHD are exacerbated by the
frustration of poor communication.
Chapter 2: the BPSHD
HD often does not present with a true memory deficit with
forgetting of information. People with HD can learn, but with
generally slower and effortful repeated learning. They benefit from
cues and prompts and recognition.
14
2.4 Psychological Contributors to BPSHD
Someone’s personality style may have a major role in the
development and expression of the BPSHD. For example, someone
who was always controlling or aggressive is more likely to have
traits exacerbated by HD and develop these as BPSHD.
2.5 Social and Environmental Contributors to
BPSHD
These include:
- Relocation (for example moving house can cause significant
disorientation and disturbed behaviour for many months
following a move)
- Caregiver distress and poor interpersonal relations
between the person with HD and caregiver (for example
when given excessive demands)
Chapter 2: the BPSHD
Individuals with HD can be more sensitive to changes in their
environments and stress, particularly as cognitive function starts to
be impaired.
15
3. The common BPSHD
There are many ways in which the BPSHD can be grouped,
including in symptom clusters, function, or altered behaviour. This
booklet presents them in symptom clusters.
16
3.1 Affective (Mood-related)
Symptoms
Apathy/ Indifference / lack of motivation
Definition: The apathy characterised in HD is typically that of
indifference or lethargy, and the individual may seem amotivated,
lack spontaneity, and have diminished concern for things he or she
used to care about. It has been called the absence of the internal
drive to accomplish tasks.
People with apathy show:
• A lack of interest in daily activities and personal care
• No interest in his or her surroundings
• Lack of enthusiasm for things that they used to enjoy
• Reduction in the ability to initiate conversation or activities
• Decreased social interaction
• Decreased facial expression
• Monotonic speech
• Decreased emotions
• Decreased initiative
The inability to “get going” and “get started” can be one of the
earliest symptoms (like a car without a starter motor), and wellinformed caregivers can help initiate tasks that the person with HD
can then complete themselves.
The symptoms of apathy can be mistaken for those of depression,
as both can present with diminished interest, psychomotor
retardation, and lack of energy and motivation. However, apathy is
more characterised by lack of motivation without the sadness,
dysphoria (an emotional state characterized by anxiety, depression,
or unease) or vegetative symptoms (e.g. insomnia, fatigue, impaired
attention) of depression. Apathy can be a frustrating symptom for
17
others as people may feel that the person with HD is capable of
performing an activity, but “won’t”.
Strategies
•
•
•
•
Chapter 3: the common BPSHD
•
Remember that apathy does not equal laziness.
Educate the family and friends about the person with HDs
difficulty beginning or initiating tasks and their role in
initiating and encouraging them to begin tasks.
The use of a regular routine is helpful.
It is important to differentiate between apathy and
depression, as the management of each disorder differs.
Try and be motivational for the person with HD.
Source: Apathy, by Leslie Bruce. http://www.lesliebruceartandillustration.com/id1.html
18
Source: http://lifeinthefastlane.com/wp-content/uploads/2010/02/depression.jpg
Depression is common in HD due to both the emotional response to
the diagnosis and symptoms of HD, and its effect on a person’s life
and role; and also the biological changes in HD. Depression can
frequently be an initial manifestation of Huntington's disease.
Depression can also occur in the middle to later stages of
Huntington's disease as a person is confronted with their
diminished ability to do things and by social isolation.
It is important to differentiate depression from demoralization,
transient changes in mood caused by negative life events, such as
bereavement, and from some of the symptoms of Huntington's
disease itself. However, as the disease progresses, diagnosis
becomes more difficult because of increasing language and
Chapter 3: the common BPSHD
Depression / Suicidality
19
communication difficulties that limit the identification and
description of emotional state, and many of the symptoms of HD
that occur as part of the illness resemble depressive symptoms (e.g.
apathy, weight loss, sleep disturbance, agitation), making it difficult
to diagnose depression.
There can be frequent requests for help, complaining, and
negativism. Negativism is a refusal to do things- sometimes when
someone doesn’t understand the situation, this can lead to
stubbornness, resistance to care and uncooperativeness.
Depression should be considered when:
• There is a pervasive depressed mood and loss of interest or
pleasure in previously enjoyed activities
• There are changes in self-attitude, such as feelings of
worthlessness, guilt, and hopelessness
• There are self-deprecatory statements
• There are appetite and sleep disturbances, particularly loss
of appetite and early morning awakening
• There is agitation or tearfulness
• There is a drop-off in functional abilities in excess of the
stage of illness
• There is a slowing of speech and movement.
• There are expressed wishes to die
• There is a history of depression prior to illness onset
In severe cases of depression, delusions or hallucinations may be
present, the context of which tend to match their depressed mood.
Chapter 3: the common BPSHD
Definition: Depression in HD is usually manifest as low mood, low
self esteem, anxiety, suicidal ideation, and by pessimistic outlook.
There is usually a loss of pleasure in activities that normally
produce pleasure. Apathy, withdrawal from activities, social
isolation, changes in appetite and weight (non-depressed people
with HD often have a great appetite), and mood swings can all be
components of depression in HD.
20
Grieving and Coping with Loss
It is essential to promptly manage the symptoms of depression as
depression can be associated with significant morbidity, including
isolation, appetite and associated additional weight loss, sleep
impairment, muscular deconditioning, lowered mobility, and
reduced self care abilities. Not only can depression be easily
treated, but by treating it the quality of a person's life can be greatly
improved. The consequences of not treating depression can
include: worsening the depression, loss of contact with friends and
family and the very real risk of suicide.
Risk factors for suicide:
• Depressed mood
• Lack of, or withdrawal of, support
• Increase in stress or upcoming stressful event
• Withdrawal and desire to be alone
• Talk about ending it all or not having to deal with it
anymore
• Talk about death
• Talk about or active pursuit of a plan
• Putting their life in order
• Saying goodbye
Chapter 3: the common BPSHD
HD is a degenerative disease and at every stage there is likely to be
an issue that requires grieving and coping with multiple losses.
This grieving process is common in both person with HD and their
family members.
Examples include coping with the loss of:
• paid employment and associated self esteem
• hobbies due to cognitive or motor impairments
• ability to eat and swallow solid food
• the vision of the future
• income
• independent living
21
Suicide prevention:
• Be open and talk to the person about it – it will not cause
them to think about it more than they are already
• Ensure adequate personal and social support
• Bring them to a mental health specialist, and keep them in
the loop of mood changes
• Remove or lock away all potential means from their
surroundings (e.g. rope, guns, medications, hose, solvents)
• Emergency numbers by telephone
• Arrange for supervision if carers cannot be around when
needed
•
•
•
•
•
•
•
•
•
Take the person with HD outside the home for activities
Social contacts from other people whose company they
enjoy
Regular exercise
Bright sunlight
Increase and encourage activities that the person with HD
enjoys. What did they enjoy and find pleasurable in the
past? Modify that activity if necessary to their current level
of functioning.
Establishing a daily routine can be helpful to combat
negativism.
Encourage person with HD to talk about pleasant things
and happy events
Provide a cheerful environment
For more severe cases, an antidepressant can be used in
conjunction with behavioural strategies.
Chapter 3: the common BPSHD
Strategies
22
"Tired of fighting" / 2008 Ruth Batke. Source.
http://en.artoffer.com/_images_user/3770/55779/large/Ruth-Batke-Emotions-AggressionEmotions-Depression-Contemporary-Art-Contemporary-Art.jpg
As part of the neuropathology and cognitive decline in HD, a person
with HD often has difficulties with their ability to modulate
emotion or control wide fluctuations in their emotions. This means
that people with HD can sometimes have an excessive and sudden
emotional response or behaviour. Frustration, agitation, and anger
are usually triggered by an actual event, but the person with HD
can no longer control the intensity of their response. These can be
sudden angry outbursts, verbal aggression (shouting, swearing),
and threats of, and actual physical aggression. Just as they can
rapidly escalate into severe anger, they can also calm down just as
quickly (intermittent explosive disorder).
Cognitive, medical, psychological, environmental factors affect
agitation. Risk factors for this include severe cognitive impairment,
older age, long standing personality traits, and BPSHD such as
hallucinations and delusions.
Chapter 3: the common BPSHD
Agitation / Aggression
23
Causes of outbursts (from Haupt, 1996)
Organic variables
Brain pathology
Neurotransmitter dysfunction
Environmental variables
Unidentified noise
Inadequate lighting
Moving to unfamiliar places
Adversarial management style
Agitation
Agitation is frequent in those with moderately impaired cognitive
function, and become less evident in the advanced stages of HD
(mostly due to the deteriorating physical and neurological status).
Most agitated behaviours signal discomfort and discontent
Chapter 3: the common BPSHD
Psychological variables
New environment
Realisation of illness
Reduced ability to communicate
Acting out psychotic distress
Increase of established personality traits
Difficulties in the relationship to caregiver
24
Examples of agitated behaviour (adapted from the CohenMansfield Agitation Inventory, 1989)
General restlessness
Repetitive mannerisms
Pacing
Trying to get to a different place
Handling things inappropriately
Hiding things
Inappropriate dressing/undressing
Verbally non-aggressive behaviours
Negativism
Does not like anything
Constant requests for attention
Verbal bossiness
Complaining / whining
Relevant interruptions
Irrelevant interruptions
Repetetive sentences
Physically aggressive
behaviours
Hitting
Pushing
Scratching
Grabbing things
Grabbing people
Kicking
Biting
Verbally Aggressive
Behaviours
Screaming
Cursing
Temper outbursts
Making strange noises
Aggression
Definition: an unprovoked or disproportionate act of hostility
which can either be verbal or physical. Some people suffering from
HD may become emotionally volatile and become less able to
control their emotions. This may be manifested as increased
irritability, frustration, or episodes of uncharacteristic
explosiveness and/or aggressiveness. For example, when
confronted with the denial of their desire a person with
Huntington's disease may respond with a temper tantrum instead
of being reasonable and measured.
Aggressive behaviours can be particularly disturbing to the family
unit because they can be a source of fear and tension.
Chapter 3: the common BPSHD
Physically non-aggressive behaviours
25
•
•
•
Physical and verbal aggression is more likely to occur in
those with poor social relationships
Verbal aggression is related to depression and health
problems
Physical aggression is more common in those with severe
cognitive impairment and in men
•
•
•
•
•
•
•
•
•
•
•
•
•
•
Avoid stressful and provoking situations
Avoid getting agitated in response
Use a calm, gentle, reassuring voice
Approach the agitated person calmly from the front
Tell the person what is going to happen in delivering care
Touch can either be reassuring or provoking
Use non-threatening postures/gestures (e.g. be at eye-level)
Avoid confrontation and arguing (it will likely be futile and
cause the situation to escalate)
Avoid physical restraint
Use distractions. Ask unrelated questions, use music
Disengage. Change activities. Leave the room if necessary.
The reasons behind the agitation must be discovered so
that appropriate social, environmental, behavioural, or
medical intervention can be made.
Be consistent. If you initially decline a request (e.g., to
allow them to watch a television show), they respond
angrily and then you give in to their original demand, you
have taught them that being aggressive allows them to
accomplish their goals. Once a pattern of behaviour is
established it may become difficult to break.
Try not to concentrate on talking about the aggressive
behaviours, try to focus on positive, beneficial behaviours.
By focusing on the inappropriate behaviours you may
actually be reinforcing them and de-emphasizing the
desired behaviours.
Chapter 3: the common BPSHD
Strategies
26
•
•
In severe cases, pharmacological agents may be necessary
as an adjunct to other strategies. Medication may be
effective.
Stay safe. When aggression reaches the point where you
are concerned about harm to yourself, to the affected
person or to others, seek professional help immediately.
Have relevant telephone numbers handy.
Anxiety and stress
Anxiety symptoms are often worse when in new situations and
some may develop a phobia related to embarrassment about visible
symptoms. As thought processes become less flexible, those with
HD may be anxious by even small departures from routine. They
may worry for days in advance about what to wear when going to
the hairdresser or whether to attend a family function.
Strategies
•
•
•
•
•
•
•
•
•
Stick to agreed established routines
When giving directions, one manageable step at a time
Remain positive and encouraging
Tell the person about plans and changes in plans in advance
Remove excessive stimulation from the environment.
Discontinue the anxiety-provoking activity
Provide calming background music and participation
(instruments, dancing, singing). Even severely impaired
people often retain musical abilities and appreciation.
stress reduction activities (e.g. participation in long
standing religious activities, pet and art therapy).
In severe cases, medication may be necessary.
Chapter 3: the common BPSHD
Anxiety in HD is often typically characterised by nervousness,
restlessness, fidgeting, shallow breathing, sweating, fear, panic,
rapid heart rate, and repetitive thoughts about bothersome topics.
Other symptoms can be irritability, overt aggression, psychomotor
agitation, and constant crying. Behaviours such as pacing, chanting,
and repetitive tapping may reflect underlying anxiety.
27
Source: http://lifeinthefastlane.com/wp-content/uploads/2010/03/art_59_full-240x300.jpg
Psychotic symptoms
Psychotic symptoms reflect a loss of touch with reality. Symptoms
can include seeing, hearing, smelling, or tasting things that are not
there (hallucinations), paranoia, and delusional thoughts. These
are rare BSPHD. When they are present, they generally occur after
the motor symptoms. Psychotic symptoms tend to present early in
the course of the illness, are frequent in those with moderately
impaired cognitive function, and become less evident in the
advanced stages of HD (mostly due to the deteriorating physical,
cognitive, and neurological status)
Hallucinations
Hallucinations can vary between benign misperceptions of reality
to horrifying experiences that have no basis in reality. They can
Chapter 3: the common BPSHD
3.2. Perceptual Symptoms &
Mania
28
involve sounds (especially voices), visions, olfactory (smells),
gustatory (taste) and tactile (feeling of being touched). Paranoia
can be associated with hallucinations or even trigger
hallucinations.
People with Huntington's disease may have benign, or nonbothersome (to them) hallucinations. An example of a benign
tactile hallucination is that of someone who feels that the chorea of
his legs is caused by ghosts. Another example is someone who may
stare off into space and laugh at something that appears to be just
in front of them.
•
•
•
•
•
Hallucinations:
distort the ability to understand the outside world
can impair functional ability
can adversely affect their relationships
can be very distressing
can be associated with paranoia
Delusions
Delusions are:
• strongly held false beliefs
• believed in spite of invalidating evidence
• thoughts about unreal situations and relationships
• are a risk factor for physical aggression
• capable of exerting a lot of inappropriate influence on
someone’s life.
The person with delusions usually expresses ideas or beliefs with
unusual persistence or force. For example, someone can think that
someone is out to get them, watching them, or reading their mind.
Chapter 3: the common BPSHD
In those with moderate cognitive impairment, an association may
exist between visual misperceptions and hallucinations. An
examination of auditory and visual function is an essential part of
assessment of any person suffering hallucinations.
29
Mania
People with HD may become manic, displaying elevated or irritable
mood, impulsiveness, overactivity, decreased need for sleep, and
grandiosity.
Symptoms include increased levels of activity,
pressured speech, uncharacteristic cheerfulness, large and
inappropriate purchases, and a return of sexual interest after a long
period of an inability to experience pleasure or impotence.
Example
Jack became very talkative with increased use of swear words and
grandiosely talking up his skills and abilities, spending money on
unnecessary goods and giving away money to strangers
•
•
•
•
•
Treatment is often challenging due to the person denying
that there is a problem.
Frequently orient person with HD to surroundings and
provide orientation cues
Engage with a neuropsychiatrist or psychiatrist to monitor
symptoms
In some cases, medication may be necessary. Antipsychotic
and anticonvulsant medication can be effective.
Medications should be a second line of treatment after
attempts have been made to understand or modify the
behaviour. Newer antipsychotic medications appear to be
at least as effective as conventional neuroleptics, but with
fewer side effects.
Those with HD are more susceptible to the toxic effects of
medications, so frequently consult a qualified professional.
Experience of Psychosis: By Maureen Oliver. Source http://lifeinthefastlane.com/
Chapter 3: the common BPSHD
Strategies
30
3.3. Personality Symptoms
Disinhibition & Impulsivity
Behaviours that can occur due to disinhibition and impulsivity:
• Easily distractible
• Emotionally unstable
• Poor insight and judgement
• Alcohol and drug abuse
• Crying
• Euphoria
• Behavioural outbursts, aggression, and irritability
• Self-destructive behaviour
• Sexual disinhibition
• Intrusiveness
• Wandering
• Shoplifting
• Gambling
• Impulse buying
• Other unrestrained behaviours
Example
Lisa spent half her rent money on a new sound system she didn’t need
Chapter 3: the common BPSHD
Disinhibition has been associated with impulsive and inappropriate
behaviours. People who are disinhibited have trouble controlling a
sudden desire to do or say something that comes to mind, even if
the behaviour is inappropriate, hurtful, or repetitive.
The
awareness of social conventions may wane, resulting in (for
example) lewd or rude comments to others, and behaviour can lead
to financial problems. Some people with HD no longer have the
‘inhibiting’ emotions of shame, embarrassment and fear that help
keep social behaviour in check.
31
Strategies
•
•
•
•
Remember, the person is not doing this intentionally
A routine and daily schedule can reduce confusion and
outbursts by giving them predictability.
Stay calm and not react emotionally
Seek medical advice
Perseveration, Obsessive-Compulsive behaviour,
Inflexibility & Self-Centeredness
A person with HD may be perseverative or often become “stuck” on
an idea or activity. They can be rigid in their behaviour and
struggle to change, adapt, and/or alter routines. Examples include a
person with HD who perseverates about obtaining cigarettes.
While a true diagnosis of OCD is not common in HD, many of the
symptoms are often reported. An obsession is a constant concern
or worry about one or more things that may or may not be
grounded in reality. A compulsion is a feeling of needing to o
actually performing a task over and over. Compulsions, like
obsessions can be grounded in reality. If you realize that your
memory is not what it used to be, you may need to check several
times to be certain that you have locked the door.
When obsessions or compulsions get to the point that they are
consuming too much of one's time or they get to the point where
the compulsion of obsession is in itself worrisome, something
should be done. Sometimes, calm rational discussions with the
person can help them to confront the fear that has led to their
obsession and allow them to cope.
Chapter 3: the common BPSHD
Some people with HD appear to have an inability to see things from
another person’s point of view. They can appear to be self-centred
and selfish.
32
Strategies
•
•
•
•
Make sure their underlying needs are being met (that
the repeated actions are not made in the hopes of being
understood).
Try and reduce feelings of frustration and irritation if
their requests are denied.
Use distraction. Use favourite items or foods of the
person with HD to distract and help shift them off the
topic they are stuck on.
Try and use humour
Chapter 3: the common BPSHD
Frustration & Irritability
Source: "Frustration” Graphics –Gold Jordan Recchia. http://www.santaclaritaartists.org
As mentioned in the section on the affective BPSHD, some people
suffering from HD may become emotionally volatile and become
less able to control their emotions. This may be manifested as
increased irritability, frustration, or episodes of uncharacteristic
explosiveness and/or aggressiveness.
33
The person’s entire personality is often described as changed due
to these symptoms.
Frustration may be caused by clumsiness or difficulties with
communication, and also due to physical factors such as hunger and
fatigue.
If someone with Huntington's disease is irritable, it is important
that the cause is figured out. This is especially true when the
disease has advanced to the point that communication is difficult.
Underlying causes of triggers can include:
- pain
- discomfort
- hunger
- thirst
- communication difficulties
- awareness of deterioration of function
- boredom
- loss of role
- changes in routine
- depression
Strategies
•
•
•
respond calmly, diplomatically, and avoid
confrontation
Keep the environment calm and structured
For extreme irritability, anxiolytic medication can
be effective.
Chapter 3: the common BPSHD
Irritability is typically manifested by verbal outbursts, threatening
behaviours, aggression, and inflexible/uncooperative behaviour.
Bad-tempered outbursts and irritability are among the most
common and troublesome BPSHD, with family members
complaining that the person with HD becomes irritable for no
obvious reason, and that during these periods the slightest
provocation (such as a small disagreement or trivial nuisance) will
provoke an outburst of angry or even violent behaviour.
34
Altered Sexuality
These BPSHD often cause distress and embarrassment for the
caregiver. A lowered sex drive is a common BSPHD. Decreased sex
drive can be secondary to apathy, depression, and amotivation.
Increased sex drive can also occur, and occasionally, people with
HD may desire and pursue excessive sexual activity or engage in
inappropriate sexual behaviours, such as public masturbation, or
voyeurism. Increased promiscuity can be a result of disinhibition,
impulsivity and/or poor judgement.
Strategies
•
•
•
•
•
•
Be matter-of-fact. Try not to react with distress or
shock.
Unclothing behaviours may occur because the person
with HD is confused as to where they are or have
forgotten why or how to dress. Their clothes may be
uncomfortable. Bring the person some clothes or
change their wardrobe accordingly.
Distract the person
Prevent future episodes of unwanted sexualised
behaviour by increasing involvement in other activities.
HD does not end one’s sexuality
Keep open communication about it
Chapter 3: the common BPSHD
The brain is no longer able to properly regulate the amount and
appropriate expression of sexual drive a person has, resulting in
too much, or too little. The neurotransmitter and hormone balance
in the brain may also be disrupted by HD pathology.
35
3.4. Unawareness, Denial, &
Lack of Insight
People with HD may:
• Have reduced capacity for self-reflection and insightfulness.
• Deny having HD or accept this reality and accept their
symptoms.
• be unable to recognize their own disabilities or to evaluate
their own behaviour and limitations.
This can result in anger as some people with HD cannot understand
why they can no longer, say, drive or work.
The denial and unawareness is likely caused by a combination of
the normal psychological response to a distressing situation, and
also organic changes to the brain (e.g. damage to circuits
connecting caudate nucleus to frontal lobes). Unawareness can
also be a defence against depression.
Examples:
Trevor denies needing help and refuses all home help, despite being
bruised from multiple falls.
Marianna has significant cognitive impairment, but describes it as
“normal, and all part of the wear and tear of ageing”
Strategies
•
Remember, the unawareness is not intentional.
Chapter 3: the common BPSHD
Definition: a lack of self awareness and an inability to evaluate
one’s own performance may cause individuals to be unaware of
mistakes that are obvious to others.
36
4. Treatment and Management
of the BPSHD
BPSHD are treatable and are often more amenable to therapy
than other symptoms of HD
4.1 The role of Caregivers
Caregivers care for the person with HD, and for themselves. Both
family caregivers and professional caregivers can be affected by the
BPSHD. Although this booklet focuses on family caregivers, many
of the concepts can be translated to professional caregivers.
Difficult BPSHD can increase caregiver burden (more so than the
motor or cognitive impairments) and the relationship between the
caregiver and the person with HD significantly determines whether
at-home care can be maintained.
Caregivers are at high risk for developing psychological distress
and feelings of depression and anxiety. The demands of caretaking
have been found to aggravate existing vulnerabilities to illness,
affect immune system, increase blood pressure, and exacerbate
feelings of stress. Carers need to be well and healthy –
psychologically and physically, to be the best caregiver they can be.
KEY POINTS
• Caregiver distress and poor relationships can exacerbate
BPSHD
• One BPSHD may be stressful for one caregiver, but not for
another. It depends on the appraisal by the caregiver.
• It is important that the caregiver and family member needs
are addressed as this enables them to continue caring for
the person with HD at home.
• Caregivers are people too.
37
TIPS FOR CAREGIVERS, FAMILY MEMBERS, and FRIENDS
• Make and take personal time. Don’t forget to do the things
you enjoy. Deliberately include this in the schedule of
activities and prioritise.
• Seek support from family, friends, neighbours, spiritual
counsellors, agencies
• Get enough sleep
• Don’t neglect personal health care – go to the doctor as
needed
• Don’t take negative behaviours or comments personally
• Delegate - allow others to do easier tasks so this leaves
more energy for the harder ones.
• Consider practical supports such as Meals on Wheels,
council cleaning, respite, day centres.
• Learn about HD, it’s effects and management
• Learn practical strategies about organisation in the home,
and appropriate exercise and diet.
• Join community support groups (e.g. Huntington’s Victoria
Alzheimer’s Association, Carers Australia).
• Develop and maintain personal and family support
networks
• Plan for the future, including what to do in an emergency,
• Get financial and legal affairs sorted as early as possible.
• Learn about entitlements (social services, carer benefits)
• Seek help from professionals (e.g. case worker or social
worker for linking in with resources, nurse or personal
care assistant to help with daily tasks of medications or
bathing)
• Use regular respite services. This is best used to prevent
the caregiver feeling stressed and will enable to give care
for longer. There is in-home care (where someone comes
into the home to spent time with the person with HD),
overnight or residential respite (one night to several
weeks), day care, and inpatient stays.
Psychological coping mechanisms
- Talk about it. Talking and ventilating feelings about the
situation will help in managing stress, guilt, and resentment
feelings, and/or feelings of martyrdom. Anger and
resentment are emotions commonly felt by caregivers
- Learn effective coping mechanisms (e.g. problem solving,
stress relief, positive appraisal of situations)
- Join a support group. It helps to discuss issues with people
going through similar problems, learn new strategies for
dealing with the BPSHD, and to see that some problems are
not unique
- Develop means of dealing with the anxiety, depression, and
other negative effects of caregiving (e.g. counselling,
relaxation training, stress management skills, baths, yoga).
- Try and keep a sense of humour and laugh a little each day,
preferably with the person with HD.
Source: http://ahuli.info/wp-content/uploads/depression-anxiety-recognize-before-late.jpg
The BPSHD are recognisable,
understandable, and treatable.
Chapter 4: Treatment and management
38
39
4.2. Strategies for dealing with the BPSHD
In managing the BPSHD, involve the person with HD as much as
possible
• Work together
• Start with small goals and work up from there
• Management plans must be tailored for the individual
• Don’t expect change overnight
• Don’t give up if the first plan doesn’t work – try another
one!
• Be prepared to always evaluate, update, and modify plans
• Engagement with health professionals and also support
groups can help to teach family members techniques to deal
or work with the problems.
Your behaviour towards the person with BPSHD has a direct
and significant impact on them
Pharmacological Management
Medication is typically used for moderate to severe BPSHD. They
are most effective when used in conjunction with the nonpharmacological strategies described below.
When considering using medication in a person with HD,
consideration must be given to their age and the disease-related
changes. For example, nutritional deficiencies and the degree of
neuropathology can make those with HD more sensitive than
others to the effects of the drug. Some medications can increase
sedation and the risk of falls. Low starting doses, slow dose
titration, and careful monitoring of side effects is recommended.
Review of the medication regimen is also recommended at 12 week
intervals, in order to measure efficacy (effect of the frequency and
severity of the symptom) and its side effects.
Keep your medical professional involved.
40
Non-Pharmacological Management
Non-pharmacological approaches are the first line treatment for
the BPSHD. These strategies are typically used for mild to moderate
BPSHD.
The problem solving approach to management of the BPSHD
1. assess and identify the problem. Describe the behaviour
and note the frequency. Is it measurable? How severe is it?
2. identify triggers.
• When does the problem occur?
• Where does the problem occur?
• What precedes the behaviour?
• Who was involved?
• What emotion was expressed (e.g. fear, frustration,
anxiety)?
Compile possible causes of the unwanted behaviour. Possible
causes include: cognitive overload, environmental change,
individual contributions.
3. identify consequences
• what follows the behaviour?
4. generate solutions. be creative!
Develop of list of possible workable responses to the behaviour.
5. rehearse the solution
6. implement
7. evaluate
8. monitor
Chapter 4: Treatment and management
The first step in the management of the BPSHD involves the careful
assessment and correction of any physical, psychosocial, or
environmental triggers or perpetuating factors. It is helpful for
caregivers to try and understand why the person with HD is
behaving that way, and to determine the triggers for the unwanted
behaviour. Take the time to work out the antecedents and possible
reasons for symptoms, if any. Look at what the behaviour
represents and what it accomplishes.
41
Be flexible and creative. Allow many attempts and use a trial and
error process. Be ready to try several strategies. What works with
one person may not be appropriate for another and individuals
with HD may react differently. The chosen approach needs to be
chosen keeping in mind personality styles. For example, in some
cases, humour could be used, or in others diversionary tactics, in
others, reassurance. Some may feel that touch is comforting and
quieting but some may find touch as an invasion of personal space.
Try and engage the person with HD in activities that reflect their
unique personalities. Realistically evaluate what a person with
Huntington's disease is capable of and what they are interested in
doing. They may not be able to paint as well as they used to, but
they may still enjoy the act of painting. Activities of everyday life,
such as bathing, food preparation and other acts may become more
difficult for them to accomplish. If these acts can be modified, the
person can still maintain the feelings of accomplishment and
independence that come with the ability to help themselves. For
example, if they can no longer sort the clothes properly when doing
the wash, perhaps they can still do the wash if someone else sorts
the clothes.
Environmental interventions
The person with HD may have more difficulty than before adapting,
thus the environment can be adapted to them. Try and make the
environment familiar, constant, and non-stressful.
Establish and Maintain a routine.
Routines help to externally structure the day as the internal
structuring system is not as good as it used to be in those with HD.
A lot of people with HD benefit from having a regulated daily
environment that is consistent, structured, and predictable. If there
must be change, make the changes gradual, allowing the person
with HD time to become familiar with changes to their habits.
Chapter 4: Treatment and management
Remember and maintain the person with HD’s personality
42
The BPSHD also tend to worsen with fatigue. People with HD tend
to use more energy doing everyday activities, so ensure daily
routines include rest time. Fatigue sets in as the day progresses so
the afternoon may be dominated by less activity.
A weekly routine is also important, with scheduled activities.
Example:
Monday: day centre, in home carer coming
Tuesday: at home day, gardening,
Wed: Church group, in home carer coming
Thurs: carer outing
Friday: at home day, in home carer coming
:
Source: http://pages.prodigy.net/bjbservices/images/Schedule.JPG
Chapter 4: Treatment and management
Saturday: library day, Grandkids visiting
Sunday: Church and church group lunch, movie night at home
43
1
2
3
4
5
Example.: Moving into assisted living.
Discuss the possibility of a move early– so the idea does not
come as a surprise when the person is more affected.
Make sure the person gets some say in the decision making
process. Give them the option of choosing from a few different
places.
Bring photos, personal items, and other objects (even furniture)
that are familiar to the person.
Arrange furniture in the same configuration and orientation.
Use the same bed sheets.
Avoid overloading and overstimulation
In HD, it becomes easy for the brain to overload, as it is not working
as efficiently as it could. Ensuring the environment is not too busy
and overstimulating will help the person with HD focus.
Example room considerations:
o Remove as many distractions as you can.
o Switch off the television when engaged in an activity
o One person and one voice at a time
o Remove unnecessary furniture
o Use soft lighting and calm colours
o Use rugs and carpets to absorb sound
o Use calming music
o Make sure lighting is adequate
o Take into consideration longstanding preferences
o Avoid loud prints, abstract designs, confusing
mirror placements. loud telephones or alarms,
changing room layouts
Chapter 4: Treatment and management
These routines should be written or printed and placed in a highly
visible position and should have priority and the routine only
broken in unavoidable circumstances. If the routine does have to
be altered, try to limit the associated stress by telling the person
with HD well ahead of time, only making changes one at a time, and
keeping as much familiar as possible.
44
Do’s and Don’ts
Do’s
•
•
•
•
•
•
•
•
suddenly change the environment
suddenly change established routine
be rigid or controlling
be overly demanding
be critical
ignore needs
get angry if the person with HD forgets something
be aggressive
become exasperated or frustrated
maintain routine
maintain stable environment
involve the person in day-to-day activities
foster a sense of individualism and personhood in the
person with HD
try and give person a degree or semblance of control and
decision making in their life and environment
remember and maintain the person with HD’s personality
Remember it is not easy for the person with HD tom
communicate due to speech problems, word finding
difficulty, and lack of self awareness.
Focus on what they CAN do, not what they CAN’T.
Caregiver characteristics:
• be patient
• be unhurried
• be flexible and adaptable. If the person with HD wants to do
something a certain way, wear a certain article of clothing,
that’s fine.
• Keep sense of humour!
• Be creative in coming up with potential strategies
Chapter 4: Treatment and management
Don’ts
•
•
•
•
•
•
•
•
•
•
•
•
•
•
•
•
•
•
Be empathic, kind, caring, and warm towards person with
HD
Try to understand the reasons and triggers behind the
behaviour
Maintain realistic expectations of them and their abilities
Be tolerant.
Be respectful of the person with HD as a person with
feelings and thoughts and emotions
Try not be feel shame over a loved one’s illness, and hide
the illness
Maintain education of the symptoms of HD
Maintain own identity and support network
Seek help and respite when needed – including
psychological and social support
Communication Tips
• Use short, simple sentences
• Give one step commands
• Allow the person more time than usual to absorb
information, answer questions and to communicate.
• Keep your voice steady and talk at a lower pitch, at a
reasonable rate.
• Avoid sentences phrased in the negative (eg. “stay inside”
instead of “don’t go outside”)
• Repeat yourself if necessary
• Use visual cues, demonstration, and non-verbal gestures
and communication – smile, touch, nodding, shaking head.
• Maintain eye contact
• Be at eye level
• Give choices, but limit them to avoid confusion and
decrease frustration. Amend the way you ask questions. For
example, instead of asking, “Where do you want to go
today?” ask “Would you like to go to the café or your
daughter’s house today?”
• Avoid confrontation
• Avoid correction
Chapter 4: Treatment and management
45
46
•
•
•
•
•
•
Be calm, gentle and matter-of-fact
Flashcards may be used for communication, where the
person with HD can point.
Listen actively and make listening a priority.
Try to not become frustrated at communication difficulties.
Give cues and prompts when necessary, but not all the time.
Break instructions and directions down into small steps.
Avoid overwhelming sentences and paragraphs.
Consider speech therapy input, including the use of aids
such as picture boards.
KEY POINTS
• Compromise - Sometimes the solution is to relax one's
expectations.
• The person with HD is not being purposefully provocative.
• The BPSHD are not under their control
• The person with HD may be forgetful, but they are not being
irresponsible
• The person with HD may be irritable, but they are not being
unappreciative
• The person with HD may question you repeatedly, but they
are not being deliberately annoying
Important!
Remember that someone with Huntington's disease is still a
person. They may not be the same as they were several years
ago, but they deserve to be treated with respect and
understanding to help preserve the quality and dignity of their
life.
HD does not take away someone’s uniqueness and
individuality.
The disease is not the person.
Chapter 4: Treatment and management
•
•
47
5. Guide to identifying and measuring
the BPSHD
The BPSHD should be monitored and the following recorded over a
2 week period.
1. Identify the target BPSHD
•
•
•
•
•
•
•
•
•
•
•
What is the problem?
When does it happen?
How often does the behaviour occur?
Where does it happen the most?
Who is present when it happens?
What time is it?
How severe is the behaviour?
How distressed is the person with HD? (rate from 1
to 10, 10 being most distressed)
How distressed are you? (rate from 1 to 10, 10
being most distressed)
Was there a trigger?
What happened immediately prior?
2. Develop a Plan
Behaviour:
Intervention
Rationale
By whom?
Outcome
Criteria
48
3. Plan evaluation
•
•
•
•
•
Is the plan successful?
Is the behaviour occurring less?
Is the behaviour ceasing faster?
Is the person becoming less distressed?
If no, develop a new plan!
Example Plan
Behaviour: Potential for Physical Violence
Signs and Symptoms:
Demanding attention, raised voice,
aggressive posturing, accusing and blaming others, being overly
critical
Rationale
By
whom?
Calmly
Provide support and Husband
express
be non-threatening
and
concern and
caretaker
care
Bob
Remain close Provide support and Bob
by until Mary help relieve Mary’s
calms down
fears.
Allow Mary to Provide comfort and Bob
express
her soothing
thoughts
Turn
lights
down Avoid
overstimulation
Bob
Outcome
criteria
Mary remains
safe
Mary
becomes only
mildly
agitated
Mary
is
agitated for
less than 5
minutes
Mary
will
start
to
control
her
behaviour,
with
assistance
Chapter 5: Guide
Intervention
49
Remain calm,
clear,
and
concise when
talking
to
Mary
Do not rush
Mary
Avoid
overstimulation
Touch
tends
soothe Mary
Bob
There will be
fewer violent
responses
to
Establish a sense of
trust,
conveys
attention,
and
facilitates
communication
Establish a sense of
trust,
conveys
attention,
and
facilitates
communication
Reduce the feeling of
being forced or
pushed to do things
or act a certain way
Keep Mary and
others safe
Remove
all
potential
harmful items
from Mary’s
reach
Reinforce
Reward
positive
non-violent
behaviours
to
behaviour
discourage negative
outbursts
Promote
To
control
and
regular
channel
Mary’s
exercise
energy
Give Mary her Positive enjoyment
knitting
of
a
favourite
activity
Chapter 5: Guide
Remove
distracting
things (switch
off television)
If possible, put
a comforting
hand on her
shoulder
Listen
to
Mary’s
concerns
50
6. The Research Team
Dr. Anita Goh
Dr. Anita Goh is a qualified clinical
neuropsychologist who has established both a
clinical and academic career. She is a Research
Fellow at Academic Unit for Psychiatry of Old
Age, The University of Melbourne, and also
works as a clinician at the Melbourne
Neuropsychiatry Centre, Royal Melbourne
Hospital. Dr. Goh is a staff member of the
international Huntington’s Study Group (HSG)
and a member of Huntington’s Research Group
Victoria. Her work in international research has
resulted in publications and presentations at
national and international forums.
Contact Anita on goha@unimelb.edu.au
Professor Edmond Chiu, AM
Professor Chiu founded our Huntington’s
Clinic in 1972, and has a long record of
clinical work, advocacy, and conducting HD
research –leading to an award of Member of
the Order of Australia in 1988. He is
founding chair of the Huntington Research
Group Victoria. He is a member of HSG, and
was awarded the Marjorie Guthrie award in
1990 by the HD Society of America for his
contributions to the USA HD community. He
“retired” in December 2004 but continues to
engage in HD and Alzheimer’s disease
research in the AUPOA.
Contact Ed on e.chiu@unimelb.edu.au
51
Associate Professor Olga Yastrubetskaya
Olga is the Manager of the Research
and Specialist Clinics conducted by the
Academic Unit for Psychiatry of Old
Age, University of Melbourne. Olga is a
member of the HSG, a founding member of
the Huntington’s Research Group of Victoria,
and an Honorary Senior Fellow of the
National Ageing Research Institute (NARI).
Contact Olga on olgay@unimelb.edu.au
David is the Director of the National Ageing
Research Institute and The University of
Melbourne Professor of Ageing and Health. He
co-founded Victoria's first memory clinic, and
his main research and clinical interests are
detection and management of Alzheimer's
disease, new therapies, and care of the
depressed elderly. He leads the Australian
Imaging Biomarkers and Lifestyle study of
Ageing, a $20M 6-year collaborative study.
Contact dames@unimelb.edu.au
Professor Nicola Lautenschlager
Nicola holds the position of University of
Melbourne Professor & Chair of Old Age
Psychiatry at the Department of
Psychiatry, where she is also Head of the
Academic Unit for Psychiatry of Old Age.
Nicola is the Director for the St. Vincent’s
Health Aged Psychiatry Service. Her
research focus is early diagnosis of
cognitive impairment and intervention
trials for older adults to improve mental
health outcomes.
Contact nicolatl@unimelb.edu.au
Chapter 6: Research Team
Professor David Ames
52
7.
Resources
Huntington’s Victoria provides
services in Victoria for people with
Huntington’s disease (HD). They:
Provide information and
advice to help people with
HD and their families make
decisions about their care
• Connect people to the
services and support they
need
Assist people with HD to live independently for as long as
possible
Provide expert support for clients attending specialist HD
clinics
Provide support for carers and families
Advocate to government policy makers and deliverers of
services for improved access to services for people with HD
Educate health professionals and direct care staff about
how to support people with HD
Increase understanding and knowledge of HD in the
community
Support research that improves the quality of life of people
affected by HD
•
•
•
•
•
•
•
•
Contact them at:
16 Wakefield Street, Hawthorn, 3122
P.O Box 2112, Hawthorn, VIC 3122
Telephone: 03 9818 6333
1800 063 501 (Toll free)
Fax: 03 9818 7333
http://www.ahda.com.au/
(Website includes downloadable information sheets)
53
Australian Huntington’s Disease Association
(National)
Huntington’s Australia is the website of the Australian Huntington’s
Disease Association (National). It provides information about the
Association, and acts as an entry to the websites of the state
Huntington’s Disease associations. Services are provided to
Australian families through state associations, each of which is
independently incorporated and managed. The Australian
Huntington’s Disease Association (National) is an unincorporated
body, the objectives of which are:
• to promote a national profile for Huntington’s Disease
Associations.
• to provide a forum for planning by state associations.
• to advocate in areas of national priority.
• to develop national policies and standards.
• to promote uniformity of purpose amongst state
associations.
• to benchmark best practice in areas of national priority.
• to maintain relations with like-minded organisations
world-wide.
• to disseminate information nationally and internationally
AHDA State Associations
NSW - http://www.ahdansw.asn.au/
Post Office Box 178, West Ryde, NSW, 1685
Phone: 02 9874 9777
Freecall (Regional New South Wales): 1800 244 735
Email: hdassoc@ahdansw.asn.au
QLD - http://www.qahda.com/
385 Ipswich Road ( PO Box 635) ANNERLEY, Q. 4103
Phone: 07 3391 8833
Email: admin@huntingtonsqld.com
Chapter 7: Resources
http://huntingtonsaustralia.asn.au/
54
South Australia and NT - http://huntingtonssa.org/
PO Box 580 North Adelaide, SA 5006
Phone: 08 8352 2202
Email: mail@huntingtonssa.org
Western Australia - http://www.huntingtonswa.org.au/
The Niche, Aberdare Rd, Nedlands, WA, 6009
Phone: 08 9346 7599
Email: ahda@cnswa.com
Tasmania – Huntington’s Tasmania
PO Box 1168, Burnie, TAS 7320
Phone: 03 6431 3403
Email: hdatas@bigpond.com
Victorian Huntington’s Disease Clinics
Normanby Unit, St. George’s Campus
283 Cotham Road, Kew Vic 3101
Telephone: (03) 9816-0508
Consultant: Dr Andrew Churchyard
Cabrini Health
183 Wattletree Rd
Malvern Vic 3144
Phone : 9500 9899
Consultant: Dr Andrew Churchyard
Neuropsychiatry Unit HD Clinic
2nd Floor, John Cade Bldg,
Royal Melbourne Hospital
Grattan St, Parkville 3052
Tel: (+61) 03 9342 8750/4033
Email: info@neuropsychiatry.com.au
Psychiatry Consultants: Dr Dennis Velakoulis, Dr Ramon Mocellin,
Dr Mark Walterfang
Neuropsychology: Dr Anita Goh
Chapter 7: Resources
St Vincent’s Aged Mental Health Service HD Clinic
55
NSW Huntington’s Disease Clinic
The Huntington Disease Clinic
Westmead Hospital
Darcy Rd, Westmead NSW 2145, Australia
Telephone: (02) 9845 6544 or (02) 9845 6699.
Consultant: Dr Elizabeth McCusker
Queensland Huntington’s Disease Clinic
South Australian Huntington’s Disease Clinic
Paediatric Genetic Clinic
Level 4, Flinders Medical Centre
Bedford Park 5042 South Australia
Phone: (08) 8161 7375
Consultants: Dr E Thompson, Dr L McGregor
Chapter 7: Resources
Royal Brisbane and Women's Hospital
Level 7, Ned Hanlon Building
Metro North Health Service District
Telephone: (07) 3636 7036
Outpatients: (07) 3636 3111
Email: RBWH-Feedback@health.qld.gov.au (with the subject
"Neurology")
Consultants: Dr John O’Sullivan, Dr Pam McCombe
56
8. Further Reading
Neuropsychological and neuropsychiatric aspects of
Huntington’s disease. By David Craufurd and Julie Snowden.
Published in P Harper, G Bates & L Jones (eds.) Huntington's
disease 3rd edition, Oxford: Oxford University Press 2002,
pages 62-94.
Psychiatric disorders in preclinical Huntington’s disease.
By Camille L Julien, Jennifer C Thompson, Sue Wild, Pamela
Yardumian, Julie S Snowden, Gwen Turner, and David Craufurd.
Published in the Journal of Neurology, Neurosurgery and
Psychiatry, 2007, 78, pages 939-943.
Neuropsychiatry of Huntington’s disease and other basal
ganglia disorders. By Adam Rosenblatt and Iracema Leroi.
Published in Psychsomatics, 2000, 41, pages 24 – 30.
Depression and Stages of Huntington’s disease. By Jane
Paulsen and colleagues. Published in Journal of
Neuropsycahitry and Clinical Neurosciences, 2005, 17, pages
496-502
Psychopathology in Verified Huntington’s disease gene
carriers. By E. van Duijn, E.M. Kingma, and R.C. van der Mast.
Published in Journal of Neuropsychiatry and Clinical
Neurosciences, 2007, 19, pages 441-448.
Behavioural abnormalities contribute to functional decline
in Huntington’s disease. By JM Hamilton, DP Salmon, J Corey
Bloom, A Gamst, JS Paulsen, S Jerkins, MW Jacobson, and G
Peavy. Published in the Journal of Neurology, Neurosurgery and
Psychiatry, 2003, 74, pages 120-122.
Understanding behaviour in Huntington’s disease (2nd
edition). By Jane S Paulsen. Published by The Huntington
Society of Canada 1999
57
NOTES
58
The information in this booklet is intended as a general guide only
and you should obtain professional advice if you have specific
questions.
The opinions expressed in this document are those of the
writers.
Author: Dr Anita Goh
Research Fellow and Clinical Neuropsychologist
The University of Melbourne
January 2011