IMPACT OF NASAL VENTILATION ON SURVIVAL IN HYPERCAPNIC DUCHENNE MUSCULAR-DYSTROPHY

Background-Respiratory failure is the commonest cause of death in pati ents with Duchenne muscular dystrophy (DMD). Life expectancy is less t han one year once diurnal hypercapnia develops. This study examines th e effects of nasal intermittent positive pressure ventilation (NIPPV) on survival in symptomatic Duchenne patients with established ventilat ory failure. Methods-Nocturnal NIPPV was applied in 23 consecutive pat ients with DMD of mean (SD) age 20.3 (3.4) years who presented with di urnal and nocturnal hypercapnia. Results-One year and five year surviv al rates were 85% (95% CI 69 to 100) and 73% (95% CI 53 to 94), respec tively. Early changes in arterial blood gas tensions following NIPPV o ccurred with mean (SD) Po-2 increasing from 7.6 (2.1) kPa to 10.8 (1.3 ) kPa and mean (SD) Pco(2) falling from 10.3 (4.5) kPa to 6.1 (1.0) kP a. Improvements in arterial blood gas tensions were maintained over fi ve years. Health perception and social aspects of SF-36 health related quality of life index were reported as equivalent to other groups wit h nonprogressive disorders using NIPPV. Conclusion-Nasal ventilation i s likely to increase survival in hypercapnic patients with Duchenne mu scular dystrophy and should be considered as a treatment option when v entilatory failure develops.