Trends in survival from muscular dystrophy in England and Wales and impact on respiratory services.
Respiratory failure is an important terminal event in muscular dystrophy, but increasingly is effectively treated by non-invasive ventilation. This study was designed to assess mortality statistics in this patient group in order to get an indication of future demand. Mortality data for all deaths from muscular dystrophy registered by death certification in England and Wales between 1993 and 1999 were analysed. In total, 817 deaths from muscular dystrophy were registered between 1993 and 1999. Annual number of deaths was unchanged over this period. Median age at death (interquartile range) for all cause muscular dystrophy increased from 20 (17-42.5) years in 1993, to 26 (17.5-63) years in 1999. Respiratory failure was the primary or contributory cause of death in 82% of cases. Two thirds of these deaths were during acute infection. We can expect 100 patients with muscular dystrophy to develop respiratory failure in England and Wales each year, so non-invasive ventilation services probably need to be able to provide for 0.2 new patients per 100,000 population annually. Respiratory services also need to provide adequate monitoring and early treatment of infection in these patients.
Calvert
L D
LD
Nottingham Assisted Ventilation Group, University and City Hospitals, Nottingham, NG7 2UH, UK. lori.calvert@uhl-tr.nhs.uk
McKeever
T M
TM
Kinnear
W J M
WJ
Britton
J R
JR
D000293:Adolescent; D000328:Adult; D002423:Cause of Death; D003695:Delivery of Health Care / Q000639:trends; D004739:England / Q000453:epidemiology; D005260:Female; D006301:Health Services Needs and Demand / Q000458:organization & administration; D006331:Heart Diseases / Q000150:complications / Q000401:mortality; D006801:Humans; D016015:Logistic Models; D008297:Male; D008875:Middle Aged; D009136:Muscular Dystrophies / Q000150:complications / Q000401:mortality* / Q000628:therapy; D012138:Respiratory Therapy / Q000592:standards; D012141:Respiratory Tract Infections / Q000150:complications / Q000401:mortality; D012306:Risk; D015996:Survival Rate; D014852:Wales / Q000453:epidemiology