Health- related quality of life in patients with Duchenne Muscular Dystrophy

Introduction: Advances in symptomatic therapy have improved the physical status and life span of patients with Duchenne Muscular Dystrophy (DMD). However, very little is known about quality-of-life in this patient population.

Patients and methods: 36 patients with DMD (age range 10–24 years) completed either the DISABKIDS Questionnaire for the age group 10–16 years (18/36 boys) or the SF 36 for the age group older than 16 years (18/36 boys). To assess symptoms of depression in children and adolescents, the DIKJ (Depressionsiventar für Kinder und Jugendliche) was utilized; for adults the BDI (Beck Depression Inventory). Patients' scores were compared to published norms. Neurological examination included Vignos scales.

Results: In the older 18 patients (16–24 years) physical aspects of quality-of-life assessed with the SF36 were significantly reduced (p<0.001) compared to published normative data. Interestingly, psychological aspects of quality of life were unaltered in our sample. In our younger 18 patients (10–16 years), we also observed significantly decreased physical quality-of-life, also when compared to normative data from other chronic conditions. Social and emotional aspects of quality-of-life were also significantly impaired (p<0.001). Clinically relevant symptoms of depression were not observed in either the younger or older patient samples.

Conclusion: An increase in both psychological and emotional impairment is expected while the physical impairment is constantly growing at the same time in the older group. However, this hypothesis is not supported by our results. While the illness is progressing, only the physical quality of life remains impaired, whereas the psychological quality of life appears to be no longer affected, possibly because of psychological coping mechanisms.