Management of end stage respiratory failure in duchenne muscular dystrophy

Abstract

There were 31 Duchenne patients placed on overnight mouth intermittent positive pressure ventilation for severe respiratory insufficiency at the average age of 19.9 years. Most patients had vital capacities less than 200 cc at their last evaluations. Of these, 23 patients are alive at the average age of 27 years and live in the community, although they are dependent on assisted ventilation 24 hours a day. There were 8 patients who died at the average age of 30 years. Although normocapnic during the day, the presence of symptomatic nocturnal hypoventilation or pCO₂ over 55 mmHg documented by continuous overnight capnograph study indicates the need for introducing overnight respiratory assistance. Mouth intermittent positive pressure ventilation alone or in combination with other techniques of ventilatory assistance can prolong life while allowing optimal function, attainment of higher levels of education, and home management of patients with Duchenne muscular dystrophy.