LONG-TERM REHABILITATION IN ADVANCED STAGE OF CHILDHOOD ONSET, RAPIDLY PROGRESSIVE MUSCULAR-DYSTROPHY

  • 1 January 1981
    • journal article
    • research article
    • Vol. 62  (7) , 328-331
Abstract
The most common cause of death in patients with rapidly progressive neuromuscular disease has been respiratory failure. In 120 patients with early childhood onset, rapidly progressive muscular dystrophy, 29 were in the most advanced stage requiring mechanical ventilation 24 h/day and had vital capacities of < 10% predicted for age and height. Of these patients with severely weakened bulbar innervated muscles, 10 were receiving positive pressure ventilation via indwelling tracheostomy tubes for an average of 3 yr. The average age of the 29 patients was 27 yr with a range of 15-54 yr. Of the 29 patients, 24 live in the community. The management of and potential for improved quality of life and increased longevity in these patients are discussed.

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