Prevention of pulmonary morbidity for patients with Duchenne muscular dystrophy

Study objective: To evaluate the effects of a new respiratory management protocol on respiratory morbidity and hospitalization rates for patients with Duchenne muscular dystrophy (DMD). Design: A retrospective cohort study. Methods: Using a protocol in which oxyhemoglobin desaturation was prevented or reversed by the use of noninvasive intermittent positive pressure ventilation (IPPV) and assisted coughing as needed, the hospitalization rates and days for 24 protocol DMD ventilator users were compared with those of 22 nonprotocol DMD tracheostomy IPPV users. Results: The 22 conventionally managed patients were hospitalized a mean of 72.2+/-112 days when undergoing tracheostomy. This included a 16.1+/-5.4-day period of translaryngeal intubation. The 24 protocol patients were hospitalized a mean of 6.0+/-2.4 days (p<0.005) when beginning ventilator use. Over their next 126.2 patient-years of ventilator use, the 24 protocol patients had significantly lower rates of hospitalization (p<0.008) and hospitalization days (p<0.005) than had the tracheostomy IPPV users over a 167.2 patient-year period. This is true although 14 of the 24 protocol patients went on to require 24-h noninvasive IPPV for 4.5+/-3.6 years. Five of the 14 have yet to be hospitalized. Conclusion: The use of inspiratory and expiratory aids can prolong survival while significantly decreasing the pulmonary morbidity and hospitalization rates associated with conventional resort to tracheostomy IPPV.