AI Article Synopsis
- Respiratory failure significantly impacts mortality rates in muscular dystrophy patients, but non-invasive ventilation is improving treatment outcomes.
- A study analyzed 817 muscular dystrophy-related deaths in England and Wales from 1993 to 1999, finding no annual increase in mortality despite an increase in median age at death.
- Respiratory failure was a major cause of death in 82% of cases, mostly during acute infections, suggesting a need for enhanced respiratory service capabilities to handle approximately 100 new cases of respiratory failure annually.
Article Abstract
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.
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| http://dx.doi.org/10.1016/j.rmed.2005.09.030 | DOI Listing |
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