Published ahead of print on June 16, 2005, doi:10.1164/rccm.200503-322OC Am. J. Respir. Crit. Care Med., Volume 172, Number 8, October 2005, 1032-1036 A more recent version of this article appeared on October 15, 2005
Submitted on March 1, 2005 Quality of Life, Physical Disability, and Respiratory Impairment in Duchenne Muscular DystrophyMalcolm Kohler1,1 Pulmonary Division, Department of Internal Medicine, University Hospital of Zurich, Zurich, Switzerland * To whom correspondence should be addressed. E-mail: pneubloc{at}usz.unizh.ch.
Rationale: Duchenne muscular dystrophy (DMD) leads to progressive, generalized paresis, and to respiratory failure in the second decade of life. The assumption that severe physical disability precludes an acceptable quality of life is common but has not been specifically evaluated in DMD. Objectives: The purpose of the study was to investigate the quality of life in relation to physical disability, pulmonary function, and the need for assisted ventilation in DMD. Methods: In 35 DMD patients aged 8 to 33 years, we assessed physical disability by a score ranging from 9 (no disability) to 80 (complete dependence on care and technical aids), pulmonary function, and health related quality of life by SF-36 questionnaires. Measurements and main results: All patients required a wheelchair, and help for dressing and eating. Fourteen patients were on long-term noninvasive positive pressure ventilation. In ventilated patients, mean ±SD FVC was 12±10 % predicted, the physical disability score was 65±7. Corresponding values in spontaneously breathing patients were 48±25 % predicted, and 51±7 % predicted, respectively (P<0.05 for both comparisons between groups). SF-36 physical function scores were massively reduced in both groups (1±2, and 0±0), but vitality, role emotional, social function, and mental health scores were nearly normal (67 to 98), and did not differ between groups. Conclusions: Quality of life in DMD is not correlated with physical impairment nor the need for noninvasive positive pressure ventilation. The surprisingly high quality of life experienced by these severely disabled patients should be taken into consideration when therapeutic decisions are made. Key words: muscular diseases, respiratory therapy, noninvasive ventilation, chronic respiratory failure, hypoventilation
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