Select Committee on Scottish Affairs Minutes of Evidence

Memorandum Submitted by Scottish Huntington's Association (16 January 1998)

INTRODUCTION

The Scottish Huntington's Association

  The Scottish Huntington's Association (SHA) is a national voluntary organisation which provides information and support to families with Huntington's Disease, their carers and professional staff in Scotland. (See appendices for further details).

Huntington's Disease

  Huntington's Disease is an inherited, progressive neurological degenerative disease of the central nervous system which is characterised by severe physical, psychological and behavioural impairment for which there is no cure. Each individual who carries the faulty Huntington's Disease gene will develop Huntington's Disease, usually between the ages of 30 and 45, and each of their offspring has a 50 per cent risk of inheriting the Huntington's Disease gene.

  Huntington's Disease has a profound impact on the lives and capabilities of those who suffer from it, their families who are at risk of inheriting it, and their carers.

Complex needs

  There is now strong evidence to suggest that as a client group suffering from a relatively rare disease people with Huntington's Disease do have very complex needs which are absolutely disproportionate to their numbers.

Major long term impact

  A recent comprehensive report (HMSO—NHS Advisory Service (HAS) Thematic Review "Heading for Better Care", August 1996) disclosed that in England and Wales this client group has a major long term impact on the health and social service agencies, requiring major long term commitment with a much clear strategic approach to commissioning and provision of services.

  The report studied three client groups which had certain common features relating to gaps in services (the three groups being Acquired Brian Injury, Early Onset Dementia and Huntington's Disease). The HAS team visited six districts in England and Wales in the process of the review . In doing so it was able to generate a picture of current service shortcomings and strengths which would, in their opinion, reasonably represent the picture at a national level.

Scotland—similarities and differences

  Scottish family members with Huntington's Disease have similar medical management problems to those referred to in the HAS report. However, in Scotland the health and social services system is different to that of England and Wales. In addition there has not been a suitable in-depth review of the specific needs of those with Huntington's Disease.

Presenting key priority issues

  The issues raised within the HAS report reflects the experience of the Scottish Huntington's Association and we therefore request an opportunity to prepare a presentation for the Scottish Affairs Committee outlining the key priority issues.

  The enclosed report summarises the principal issues affecting the Huntington's Disease client group and offers recommendations to address these.

HUNTINGTON'S DISEASE—A SPECIAL APPROACH

  Huntington's disease is a very complex condition. Its most characteristic feature is chorea i.e., jerky, random involuntary movements that disrupt normal control and may lead to total dependence on others as the disease progresses.

  The muscular inco-ordination of the facial and upper body muscles result in speech, language and swallowing difficulties.

  In addition many individuals affected by Huntington's Disease suffer from weight loss and require additional dietary supplements of between 4,000 to 5,000 calories per day. This is believed to be cause of the amount of energy expended from the involuntary movements described above.

  The complex nature of the condition makes obvious the weakness inherent in service provision presently available in Scotland. The needs of Huntington's Disease families involve different providers, i.e., often both health care and social services. Currently these needs are given a low priority because the client group is numerically small. This situation prevails despite the fact that the needs are varied, complex, disproportionate to numbers and, on an individual basis, are likely to continue and intensify over a period of 20 to 25 years from the onset of symptoms.

KEY ISSUES RELATING TO HUNTINGTON'S DISEASE

  Lack of assessment and inaccurate diagnosis of behavioural problems, often resulting from paucity of specialist expertise.

  There is a complete lack of facilities for rehabilitation, day care, respite care and long term care. Patients are often placed in facilities which do not offer rehabilitation or appropriate care management. This places patients at risk because of mismanagement of clinical issues such as choking episodes. A lack of residential respite care facilities is commonly mentioned by carers. It is often reported that the only respite care offered is in a home for elderly people with dementia or in acute psychiatric unit.

  Affected individuals and carers persistently report a lack of sufficient centres of expertise and specialism. Huntington's Disease families are particularly keen to receive information, advice and genetic counselling, given the high risk (50 per cent) of Huntington's Disease sufferer's children inheriting the disease.

  Lack of co-ordination of care presents major problems for families as they are frequently passed from one care provider to another.

  There appears to be a need for properly defined care pathways for Huntington's Disease families as there is a marked disparity throughout the country in the standard of care provided.

RECOMMENDATIONS

  The Scottish Huntington's Association would like to make the following recommendations to address the key problems identified.

  1. The development of a Working Party or Select Group to investigate further the needs of the Huntington's Disease client group in Scotland.

  2. A strategic approach to the commissioning of services for Huntington's Disease families should be devised so that a comprehensive co-ordinated approach to service provision can be produced.

  3. Recognition to be given to the HAS report's recommendations that funding need not come from new resources but rather the reallocation of funding in a structured way to provide a specialist service to meet this client group's complex needs.

  4. Health and Social Services need to adopt an integrated approach to develop collaborative multi-disciplinary services for people with Huntington's Disease.

  5. The views of individuals, families and voluntary group members involved with Huntington's Disease are extremely important. Such views should be sought throughout the commissioning and planning process, as the Huntington's Disease client group are in an ideal position to express their needs and concerns about their own future care provision. Such a process would help to ensure that those services are truly inclusive of user led views.

  The Scottish Huntington's Association—as representative of this client group—consider it essential that the association are involved in future discussion about the needs of those with Huntington's Disease in Scotland.

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Prepared 27 May 1998