Management of Progressive Muscular Dystrophy of Childhood

TLDR

Preserving physical status in children with progressive muscular dystrophy may enhance the effectiveness of future pharmacologic therapies. The program aims to help patients lead a normal life physically and emotionally. The intervention combines early diagnosis, close monitoring, passive stretching of joint contractures, and bracing to support ambulation. The rehabilitation program extended average ambulation from 4.4 to 8.7 years, and with eight patients still walking in braces, the overall gain is even larger.

Abstract

In a study of 27 patients with progressive muscular dystrophy of childhood, it was found that duration of ambulation from onset of symptoms could be increased from an average of 4.4 yr to 8.7 yr by a comprehensive rehabilitation program. The program included early diagnosis, close supervision of patients, a physical therapy program with emphasis on passive stretching of flexion contractures about joints, and bracing for ambulation when independent walking was no longer possible. The total increase in ambulation will be greater, since eight patients are still walking independently in braces. This positive program is directed toward assisting the patient to lead as normal a life as possible, both physically and emotionally. The better preservation of the child's physical status should increase the chance for benefit from any future specific pharmacologic therapy.