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a Sleep and Ventilation Unit, Respiratory Support
Service, Royal Brompton and Harefield NHS Trust, London SW3
6NP, UK, b Department of Paediatrics and Neonatal Medicine, Hammersmith
Hospital, London W12, UK
Correspondence to: Dr A K Simonds.
Received 9 February 1998; Returned to authors 1 May 1998; Revised version received 8 June 1998; Accepted for publication 17 June 1998
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.
METHODSNocturnal 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.
RESULTSOne 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 non-progressive disorders using NIPPV.
CONCLUSIONNasal 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.
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