The ideas presented in this chapter are a reflection of the author’s experience over a five-year period working with people living with Huntington’s disease (HD). During this period, different approaches were explored to find those which offered maximum support to clients in their differing stages of the condition. Approaches employed needed constant re-evaluation, as HD is typified by a gradual change in abilities over an extended period of deterior-ation. A definition and description of the symptoms of HD are given, fol-lowed by behaviours and responses observed in music therapy sessions presented in case study format.
Definition and description of Huntington’s disease
Huntington’s disease is a chronic, progressive, hereditary disease affecting the central nervous system, stemming from damage to the basal ganglia within the brain. It causes motor, cognitive and emotional disorders in the affected individual, and although the average age of onset is 36–45 years of age, symptoms may begin at any time of the affected person’s life, from childhood to old age (Folstein, 1989). It is an autosomal dominant disorder, meaning each child born to a parent with the HD gene has a 50 per cent chance of also carrying the gene and developing the disease. It is not HD which causes death for the affected person; secondary illnesses, such as pneumonia or cardio-respiratory illness, have been found to be the most common causes of death (Harper, 1991; Folstein, 1989).
The illness is characterised by involuntary movements and abnormality of voluntary movements, gradual deterioration of certain cognitive skills, and emotional disorders causing complex social consequences for the individual and their family or support network.
Movement problems may involve large, ‘choreic’ movements or cause rigid-ity and slowness involving arms, legs, fingers, trunk, neck, head and face.
Speech becomes dysrhythmic, occurring in bursts with pauses in mid-phrase, and also slower and dysarthric, causing the listener difficulty in interpreting
the spoken message. Difficulty in initiating speech causes considerable delay in responding verbally to even a simple request or command. Swallowing problems develop, and in the later stages the use of direct feeding methods such as nasogastric tube or gastrostomy may be necessary to ensure adequate nutrition.
Early cognitive symptoms are likely to involve memory problems and an inability to organise information, and progressively result in decreased abil-ities in problem-solving, initiation, and concentration. The individual will experience increasing difficulty in verbal expression, the ability to change from one task to another, retrieval of information and memories, and the speed with which they can process information and act on it. There is a marked loss of spontaneity and a reduced ability to participate in novel situations. Conscious related functions such as knowing and insight, however, may be relatively well preserved even into the most advanced stages of the illness (Shoulson, 1990).
Psychiatric disorders associated with HD may be seen years before motor or cognitive symptoms have caused the illness to be diagnosed, resulting in distress and often stigma for the affected individual and their family. Depres-sion, irritability and apathy are the most often exhibited symptoms, with other psychiatric conditions such as schizophrenic symptoms, delusional dis-orders, mania and anxiety commonly being seen (Folstein, 1989; Morris, 1991). Behavioural problems and personality changes caused by emotional disorder may include sexual inappropriateness, aggressive behaviour, suicidal tendencies, and drug and alcohol abuse. The psychosocial effects on the indi-vidual, their spouse, children and support network can be tremendously complicated.
Literature review
A review of the research and descriptive literature reveals that music therapy is particularly beneficial as part of music and movement programmes (Rainey Perry, 1983; Groom and Dawes, 1985), relaxation programmes (Rainey Perry, 1983), or to facilitate speech through singing (Erdonmez, 1976; Hoskyns, 1981; Rainey Perry, 1983). Music therapy also may act as a catalyst for dis-cussion and emotional expression through songwriting or by encouraging the group or individual to choose songs with themes or lyrics with which they identify (Curtis, 1987; Dawes, 1985a, 1985b). This last technique, described as
‘counselling-oriented music therapy’, appears to be relevant when working with people in the early or advanced stages of HD, justifying it as an attract-ive and useful therapeutic tool when working with a client over a long period of time. This is particularly so considering that other media and activities usually become unrealistic or impossible for the client to participate in as they become increasingly disabled.
Judging from the literature, it would certainly seem that using familiar
songs offers a more easily measurable activity for research or evaluation pur-poses in therapy programmes, particularly important in the current climate of proving ‘effectiveness’ to service purchasers. However, the use of instru-mental improvisation is only briefly described (Hoskyns, 1981), and leaves a question as to what the benefits are of this activity with this population, particularly considering the certainty of increasing physical debility and the likelihood of cognitive degeneration.
Music therapy as part of a treatment programme
Music therapy can be seen to offer a range of experiences which may be denied to the person in their everyday life due to the physical, communication and cognitive changes they are experiencing.
Even when large choreic movements or minimal movements become impossible because rigidity render functional tasks impossible, playing musical instruments provides a purpose for attempting exploration of the environment. For clients with large, uncontrolled voluntary movements, using instruments with a large surface area – such as bass xylophones or large-headed drums and cymbals – optimises success in achieving a sound for the often enormous effort involved. For those with minimal rigid move-ments, the use of instruments suspended on a stand, such as triangle or windchimes, positioned close to the hand allows independence in achieving feedback for the involved effort. Other instruments provide maintenance and development of fine motor skills, such as the use of keyboard, or strumming instruments which involve fine finger movements or the manipulation of a plectrum. Encouraging decision-making through offering a choice of instrument is considered to be an added creative and expressive component to exploration of the environment, particularly when there may be few areas in their life where the client is still able or allowed to express choice or make decisions.
As speech becomes increasingly difficult for others to understand, and the content of speech repetitive and ‘stuck’ or seemingly meaningless, it is likely that attempting any verbal communication is a highly frustrating experience for the client. Music-making, either through singing or instrumental playing, can alleviate this to some extent by providing opportunities for recognition of the individual and creative expression through a non-verbal medium.
As the individual experiences difficulty in communicating, changes in behaviour, and changes in their physical abilities, they are likely to become increasingly isolated. Maintaining regular employment becomes more dif-ficult, and the individual may become increasingly self-conscious about their
‘abnormal’ behaviour. Opportunities for socialising decrease, and relation-ships with family and friends are often affected. The opportunity to build meaningful relationships with others, which may be severely impaired in usual settings, may be possible in a music therapy setting. This can be the case
particularly for people who have had an active background in music-making or appreciation.
Lastly, music-making can act as a powerful motivator even when the person’s ability to initiate other activities is impaired.
Music therapy in the early stages of HD
In the early stages of the illness, the person living with HD is likely to be maintaining a semblance of an independent lifestyle, still living in their own home, and possibly still employed. Physical symptoms may not be causing serious disability; however, they will be noticeable in the affected person’s walking pattern, facial expression, and on attempting any task. Commonly, cognitive changes cause problems with short-term memory and tasks which require the abilities to organise, calculate, or carry out complex processes. The individual may experience behavioural or psychiatric changes causing bizarre, unreasonable, or aggressive behaviour which seriously affects the spouse or children living with them, and can result in marital breakdown.
Emotional lability, confusion and social withdrawal are the most commonly reported responses to the dramatic changes occurring in the affected individual (Tyler, 1991).
The picture of the person living in the early stages of the illness, therefore, is one of increasing isolation and emotional turmoil as the individual attempts to adjust to the changes happening to them. If insight is impaired, they may be experiencing confusion as to why situations around them do not make sense, or why others are behaving in such an inexplicable way, telling them what to do. Previous occupations or pastimes gradually become impossible due to the physical changes occurring, communicating with speech becomes increasingly frustrating, and the possibility of experiencing something novel or pleasurable appears greatly diminished.
In the author’s experience, clients at this stage may benefit from individual rather than group therapy, giving them the space to take risks and time to reflect on how they are feeling or what they are experiencing. Placing clients in a group can be difficult, as being with other HD clients at different stages can be confronting and painful as they view where they will ‘end up’. Placing the client in a group with people with other conditions can also be problem-atic if it heightens their isolation when other group members are intolerant of the HD client’s difficulty in communicating, or find their choreic movements disturbing.
The main aim of the session may be to engage the client in a musical activity by focusing on the creative and expressive qualities of the music, attempting to use less-structured music such as instrumental improvisation where possible. The client is encouraged to explore as widely as possible, in terms of instrument choice and musical style, although their right to express a constant preference is respected. All tasks involve active participation, and
the music therapist needs to assess continually the client’s ability to organise information, remember previous material, interact musically, and the ten-dency to perseverate on material. The therapist aims to draw out the client’s strengths in the sessions, and to work towards maintaining and developing these. Areas which need particular consideration include the client’s ability to initiate change, which can affect their ability to stop playing at the end of a musical activity or change from one activity to another, as perseverative mannerisms in the music may continue from one task to the next. Memory deficits combined with an inability to stop playing may cause turn-taking activities to be inappropriate, as even simple instructions are not retained or followed. Using familiar songs, the music therapy session may provide a safe environment in which to vocalise, which may be becoming increasingly difficult in other situations.
Case study: ‘Joseph’
‘Joseph’, a man in his mid-forties with HD, was referred to music therapy on his admission to a day care facility, having been recently diagnosed. He was reported to be experiencing changing behaviour due to his illness, and increasing isolation, compounded by an ‘uncertain future’. He was still ambulant, although he walked with large swaying movements, and any inten-tional movement in his arms was clumsy and jerky due to ataxia. His speech was slurred, and the content perseverative, often being repetitive and limited in variation. He was reported to have moderately impaired memory, and had started to exhibit some delusional beliefs. When referred to music therapy, the team hoped that it would provide an ‘emotional outlet and means of expres-sion’ for him, as his verbal communication appeared limited. He had no previous musical experience.
In 16 months of attending music therapy sessions, Joseph stated that he enjoyed sessions, and appeared to gain confidence in his new-found skill.
Activities had centred around instrument playing, largely in improvisation with the therapist.
He expressed a preference in each session for the metallophone and the electric piano on ‘pipe organ’ mode, and appeared to have some difficulty in exploring more than one instrument per session, tending to stay on the first chosen one unless prompted to change. Unless activities had a definite begin-ning and ending, such as a song-based activity, he had difficulty stopping playing when the music ended, appearing to rely on visual prompts such as the therapist changing body posture away from the instrument. He was able to repeat only the simplest rhythms given, such as three or four crotchet beats, and when given a more complex pattern would return the correct number of sounds played but with no rhythmic organisation (i.e. six quavers and a crotchet would be repeated as seven ‘sounds’). This was felt to be due to a combination of difficulty with organising the information and problems with
co-ordination, as if the given pulse was kept slow he was able to repeat it with greater accuracy. He also had difficulty participating in turn-taking activities, being unable to stop playing in each of his turns, although this reduced somewhat if the therapist was sharing the same instrument. Generally he became completely engrossed in his own playing and appeared to have little awareness of the therapist’s music regardless of dramatic dynamic, rhythmic or harmonic variations. His playing on the two chosen instruments involved repeated descending and ascending scales, or repeated playing of small areas on an instrument, such as three notes descending on the piano or metallo-phone, or three or four strings on the autoharp or guitar, despite being physically able to reach the full range.
Overall, his playing showed a marked lack of spontaneity, with repeated patterns from which he seemed unable to depart, and a tendency to organise his playing spatially.
The changes which gradually emerged during the treatment period included becoming more aware of the end of a piece of music, particularly the familiar ‘welcome chant’ in which he started to anticipate the end. He also extended his playing of the keyboard to use two hands, although he remained unable to co-ordinate his movements to do this with the metallophone. The patterns he played on the keyboard expanded from simple scales to more complex ones combining sustained notes and slow trills or leaping intervals of fourths or thirds using both hands; however, these appeared to become as perseverative in nature as the scale patterns. In the last few weeks of the 16-month treatment period his playing became much louder, more energetic, and broke away more from his repetitive patterns. However, his playing con-tinued to lack spontaneity in reaction to the therapist’s music. This dramatic change in his playing occurred at a time when a change in his living environ-ment caused him to confront the painful reality of his illness, causing aggressive outbursts at home. The team felt that in his music he was starting to express the many difficult feelings which he was unable to express verbally.
Music therapy in the middle stage of HD
People in the middle stages of the illness may still be ambulant, although have increasing difficulty maintaining their balance. It is more likely that a wheel-chair is necessary. Choreic movements become more obvious, and the indi-vidual is more dependent for overall care. Communication is severely impaired, as speech is poorly articulated, inappropriate in content, or may occur only in bursts. If cognitive skills allow, the person may be able to use a communication aid, although this would also be dependent on their willing-ness to use it. Poor initiation can affect speech and behaviour in general, requiring an immense amount of time for a delayed response to even a simple request. Cognitive deficits may come into play here as well, as the person may have increasing difficulty organising and processing information,
concentrating on tasks presented, or remembering instructions given.
Behaviour may be generally unpredictable, and requests can result in an absence of response, refusal to comply, or in angry or aggressive outbursts.
In the author’s experience, group treatment can be beneficial with the per-son in the middle stages of the illness, as this allows for a natural ‘time out’
from activities when there are lapses in attention or concentration. Group activities can also give the client the amount of time needed for their delayed responses, using others’ turns as a prompt or visual model of the activity.
Choice-making may be the focus of tasks at this stage, particularly with instruments which can be chosen without the need for speech. Music, whether for instrumental activities or singing, needs to be highly structured to facilitate organisation of information, either using familiar songs, improvisa-tions based on familiar melodies, or tasks where there is a gap within the musical framework for making sounds on an instrument. The framework provided by familiar songs can also aid clients who have memory deficits, and can facilitate more spontaneous participation for those with initiation dif-ficulties. Songs are also particularly useful for those who no longer have functional speech, encouraging verbalisation of well-rehearsed lines. Alter-natively, activities may remain non-verbal in the form of instrument playing.
Case study: ‘Caroline’
‘Caroline’ was a 50-year-old woman who was hospitalised when her living situation was considered to place her at risk of danger, no longer being able to care for herself or organise her daily activities. This was also complicated by her living with immediate family members who were thought to be show-ing early behavioural symptoms of HD. On admission to the hospital she was very withdrawn and refused to participate in any form of activity by becom-ing verbally aggressive. Her contact with music therapy began with occa-sional attendance of a group with other HD clients who were in more advanced stages of the disease.
She later started to attend another music therapy group with people who had other conditions, and hence different types of abilities and difficulties.
Her speech was nearly impossible to understand due to dysarthria, and she had impaired awareness of others, resulting in poor social skills. Because of this she initially received mixed reactions from the others in the group who could all speak clearly and had trouble understanding why she did not respond verbally to their greetings and other interactions. She was, however, highly responsive to the instruments, and expressed great pleasure through her facial gestures during instrumental activities which helped the others in the group to warm in their interactions with her. Although her memory and
Her speech was nearly impossible to understand due to dysarthria, and she had impaired awareness of others, resulting in poor social skills. Because of this she initially received mixed reactions from the others in the group who could all speak clearly and had trouble understanding why she did not respond verbally to their greetings and other interactions. She was, however, highly responsive to the instruments, and expressed great pleasure through her facial gestures during instrumental activities which helped the others in the group to warm in their interactions with her. Although her memory and