The Behavioural and Psychological Symptoms of Huntington’s Disease
Transcription
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 [email protected] 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 [email protected] 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 [email protected] 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 [email protected] 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 [email protected] 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 [email protected] 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: [email protected] QLD - http://www.qahda.com/ 385 Ipswich Road ( PO Box 635) ANNERLEY, Q. 4103 Phone: 07 3391 8833 Email: [email protected] 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: [email protected] Western Australia - http://www.huntingtonswa.org.au/ The Niche, Aberdare Rd, Nedlands, WA, 6009 Phone: 08 9346 7599 Email: [email protected] Tasmania – Huntington’s Tasmania PO Box 1168, Burnie, TAS 7320 Phone: 03 6431 3403 Email: [email protected] 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: [email protected] 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: [email protected] (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