Services for Juvenile Huntington's Disease
Research type
Research Study
Full title
Assessment and satisfaction of services for Juvenile Huntington's Disease and modelling alternative methods of service organisation
IRAS ID
137435
Contact name
Oliver William John Quarrell
Contact email
Sponsor organisation
Sheffield Children's NHS Foundation Trust
Research summary
Huntington's disease is an inherited progressive neurodegenerative disorder. Juvenile Huntington’s disease (JHD), defined as onset = 20 years, is a rare form of the condition. Given the progressive nature of the condition, the needs of patients/familes change over time. On average, patients with JHD have a more complex clinical picture and more widespread neuropathology than those with the more typical adult onset form of HD. There is no disease modifying treatment so interventions are symptomatic and supportive. Whilst there are no evidenced based guidelines for the management of JHD, there are recommendations that patients should be managed within a multi-disciplinary team. The patients are widely dispersed; consequently, clinicians caring for a young person with the condition are often doing so for the first time. The multi-disciplinary services for a particular patient are organised locally on an ad hoc basis. Our previous qualitative studies have indicated that this can be a problem for families. The aim of this study is to identify a relevant sample of patients and estimate the economic cost of the current arrangement. Using a combination of interviews and questionnaires we will obtain the views of patients/carers, and professionals delivering services, on the current service provision as well as their perception of improvements which could be made. We will also specifically seek their views on two models of care provision:concentrating expertise within a multidisciplinary clinic delivered in a traditional face to face method or by delivering the expertise using tele-health technology. We will model the economic costs of these alternative methods of delivering care. At the end of the study we will have an evidence base for best practice and will be in a position to design an intervention to assess alternative models of care. We will also attempt a direct estimate of the prevalence of JHD.
REC name
Yorkshire & The Humber - Leeds West Research Ethics Committee
REC reference
14/YH/1032
Date of REC Opinion
29 Jul 2014
REC opinion
Further Information Favourable Opinion