Résumé :
|
Objective : To examine the relative impact of inherited muscle disorders (IMD) on patients' quality of life using the Medical Outcome Study 36-item short survey (SF-36). Methods : 27 patients were assessed using the SF36. Scores for eight health domains were compared to a normal population adjusted for age and gender. The longitudinal effect within the group and the relationship with respiratory function were also assessed. Results : Patients with IMD had significantly worse health in six of the domains when compared to the normal population (physical functioning, physical role limitation, bodily pain, general health perception, vitality and social functioning. In two domains (emotional role limitation and mental health) patients with IMD had scores that were significantly greater than normal. Vital capacity had a significant correlation to physical functioning and general health perception. Longitudinal assessments two years later revealed significant declines in three domains (general health perception, emotional role limitation and mental health). Conclusions : In patients with IMD, reduced vital capacity is associated with impaired quality of life related to physical factors. Despite these physical limitations, emotional aspects of quality of lifewere good, although they declined over time.
|