Quality of life in Friedreich ataxia: what clinical, social and demographic factors are important?
Version of Record online: 7 JUL 2007
European Journal of Neurology
Volume 14, Issue 9, pages 1040–1047, September 2007
How to Cite
Wilson, C. L., Fahey, M. C., Corben, L. A., Collins, V. R., Churchyard, A. J., Lamont, P. J. and Delatycki, M. B. (2007), Quality of life in Friedreich ataxia: what clinical, social and demographic factors are important?. European Journal of Neurology, 14: 1040–1047. doi: 10.1111/j.1468-1331.2007.01881.x
- Issue online: 20 AUG 2007
- Version of Record online: 7 JUL 2007
- Received 10 January 2007 Accepted 29 May 2007
- Friedreich ataxia;
- health status;
- quality of life
The aim of this study was to examine the impact of Friedreich ataxia (FRDA) on quality of life (QOL) using a generic tool to explore factors potentially associated with health status. Sixty-three individuals with genetically confirmed FRDA, self completed the Medical Outcomes Study 36 item Short Form Health Survey Version 2 (SF-36V2) and were assessed using the FRDA Rating Scale. Disease-specific, demographic, and social characteristics were also recorded. SF-36V2 results were compared with Australian population norms. Sample subgroups of disease severity and age at disease onset were reviewed. Physical and mental component summaries were examined in relation to clinical and social characteristics using multiple linear regression. QOL is significantly worse in individuals with FRDA compared with population norms. Those with severe disease did not perceive a lower QOL than those with mild or moderate disease except in their physical functioning. A later age of onset and increased disease severity were negatively associated with physical QOL, whilst, increased disease duration was positively associated with mental QOL. There were limitations associated with the use of SF-36V2 in the FRDA population. Further exploration of health-related QOL and FRDA may benefit from the use of a more appropriate generic tool.