Mechanical ventilation of patients with late stage Duchenne muscular dystrophy: management in the home.

Death in the late stage of Duchenne muscular dystrophy is most frequently a consequence of respiratory failure. Since muscles of ventilation become weakened the bellows mechanism fails insidiously. Patients exhibit symptoms of ventilatory insufficiency, the first to be noticed are those associated with CO2 retention: dyspnea, nightmares, increased heart rate, and increased blood pressure. Ten patients with late stage Duchenne muscular dystrophy have been supplied with mechanical aid for ventilatory assistance. The age of onset of respiratory distress needing mechanical assistance varied from 10 to 20 years. Meaningful survival after allegedly reaching the end stage has been from 2 to 7.5 years with an average of 3.4 years. With a caring family, these patients can have a meaningful life, even though they require continuous mechanical ventilatory aid.