Impact of nasal ventilation on survival in hypercapnic Duchenne muscular dystrophy.
BACKGROUND
Respiratory failure is the commonest cause of death in patients with Duchenne muscular dystrophy (DMD). Life expectancy is less than one year once diurnal hypercapnia develops. This study examines the effects of nasal intermittent positive pressure ventilation (NIPPV) on survival in symptomatic Duchenne patients with established ventilatory failure.


METHODS
Nocturnal NIPPV was applied in 23 consecutive patients with DMD of mean (SD) age 20.3 (3.4) years who presented with diurnal and nocturnal hypercapnia.


RESULTS
One year and five year survival rates were 85% (95% CI 69 to 100) and 73% (95% CI 53 to 94), respectively. Early changes in arterial blood gas tensions following NIPPV occurred with mean (SD) PO2 increasing from 7.6 (2.1) kPa to 10.8 (1.3) kPa and mean (SD) PCO2 falling from 10.3 (4.5) kPa to 6.1 (1.0) kPa. Improvements in arterial blood gas tensions were maintained over five years. Health perception and social aspects of SF-36 health related quality of life index were reported as equivalent to other groups with nonprogressive disorders using NIPPV.


CONCLUSIONS
Nasal ventilation is likely to increase survival in hypercapnic patients with Duchenne muscular dystrophy and should be considered as a treatment option when ventilatory failure develops.
Simonds
A K
AK
Sleep and Ventilation Unit, Royal Brompton, London, UK.
Muntoni
F
F
Heather
S
S
Fielding
S
S
D000293:Adolescent; D000328:Adult; D002245:Carbon Dioxide / Q000097:blood; D005500:Follow-Up Studies; D006699:Home Care Services; D006801:Humans; D006935:Hypercapnia / Q000209:etiology / Q000628:therapy*; D007385:Intermittent Positive-Pressure Ventilation; D008297:Male; D009136:Muscular Dystrophies / Q000150:complications*; D009666:Nose; D010100:Oxygen / Q000097:blood; D010313:Partial Pressure; D011788:Quality of Life; D012131:Respiratory Insufficiency / Q000209:etiology / Q000628:therapy
D002245:Carbon Dioxide; D010100:Oxygen