The personal experience of juvenile Huntington's disease: an interpretative phenomenological analysis of parents' accounts of the primary features of a rare genetic condition.

There has been a paucity of research into the psychosocial impact of juvenile Huntington's disease (JHD) on the child and the family. The study reported here is part of larger project that aimed to address this and investigate the social and health care needs of those affected by JHD. Ten semistructured interviews with the main caregiver(s) were carried out and were analyzed using the qualitative methodology interpretative phenomenological analysis. The main themes arising from the analysis are reported here: first becoming aware something is wrong; physical symptoms; speech and communication difficulties; behavioral problems; a slow but relentless process. These are discussed in relation to extant literature. We hope the article will be helpful to clinicians working with families where a child is affected by JHD and also contribute to the general literature on understanding symptoms in childhood illness.

Using research to develop care for patients with Huntington's disease.

Huntington's disease (HD) is a progressive neurodegenerative disorder affecting approximately 5-10 per 100,000 people in the UK (Harper, 1992). This dominantly inherited genetic condition causes movement disturbance (especially chorea), depression and dementia. The complex physical, mental, psychological and social problems caused by HD can result in particular difficulties in care. The course of the disease may span 15-20 years from diagnosis to death, necessitating long-term nursing care. The NHS and Community Care Act 1990 has made statutory provision for patients to be cared for at home if they so wish, placing a greater onus of care on the primary healthcare team. A survey of 25 patients in Somerset who had been diagnosed with HD was recently undertaken to ascertain the health and social care needs of patients and their carers. Of a possible 300 care needs in 12 categories (e.g. housing, dietary advice, carer support), 73 unmet needs were identified in this group. Using the survey as a basis, this article describes the steps taken in Somerset to ensure that the future needs of this group of patients are fulfilled. This model may be appropriate for the care of patients with other complex long-term diseases.