A longitudinal study of the SF-36 version 2 in Friedreich ataxia.
OBJECTIVE
The Medical Outcomes Study 36 item Short-Form Health Survey (SF-36) is one of the most commonly used patient reported outcome measure. This study aimed to examine the relationship between SF-36 version 2 (SF-36V2) summary scores and Friedreich ataxia (FRDA) clinical characteristics, and to investigate the responsiveness of the scale, in comparison with the Friedreich Ataxia Rating Scale (FARS), over 1, 2 and 3 years.


METHODS
Descriptive statistics were used to examine the characteristics of the cohort at baseline and years 1, 2 and 3. Correlations between FRDA clinical characteristics and SF-36V2 summary scores were reported. Responsiveness was measured using paired t tests.


RESULTS
We found significant correlations between the physical component summary (PCS) of the SF-36V2 and various FRDA clinical parameters but none for the mental component summary. No significant changes in the SF-36V2 were seen over 1 or 2 years; however, PCS scores at Year 3 were significantly lower than at baseline (-3.3, SD [7.6], P=.01). FARS scores were found to be significantly greater at Years 1, 2 and 3 when compared to baseline.


CONCLUSIONS
Our findings suggest that despite physical decline, individuals with FRDA have relatively stable mental well-being. This study demonstrates that the SF-36V2 is unlikely to be a useful tool for identifying clinical change in FRDA therapeutic trials.
Bruce Lefroy Centre for Genetic Health Research, Murdoch Childrens Research Institute, Parkville, VIC, Australia.
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Bruce Lefroy Centre for Genetic Health Research, Murdoch Childrens Research Institute, Parkville, VIC, Australia.|School of Psychological Science, Faculty of Medicine, Nursing and Health Sciences, Monash University, Clayton, VIC, Australia.|Department of Paediatrics, University of Melbourne, Parkville, VIC, Australia.|Department of Occupational Therapy, Monash Health, Clayton, VIC, Australia.
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Corben
Bruce Lefroy Centre for Genetic Health Research, Murdoch Childrens Research Institute, Parkville, VIC, Australia.|Department of Paediatrics, University of Melbourne, Parkville, VIC, Australia.|Department of Neurology, Royal Children's Hospital, Parkville, VIC, Australia.
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Bruce Lefroy Centre for Genetic Health Research, Murdoch Childrens Research Institute, Parkville, VIC, Australia.|School of Psychological Science, Faculty of Medicine, Nursing and Health Sciences, Monash University, Clayton, VIC, Australia.|Department of Paediatrics, University of Melbourne, Parkville, VIC, Australia.|Department of Clinical Genetics, Austin Health, Heidelberg, VIC, Australia.
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Delatycki
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D005621:Friedreich Ataxia / Q000453:epidemiology / Q000473:pathology* / Q000628:therapy; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D017063:Outcome Assessment, Health Care
Friedreich ataxia; SF-36; health status; rating scales