Cystic fibrosis (CF) is the commonest life-threatening genetic disease affecting people of northern European descent. In the UK, it affects approximately one newborn in 2,400. Babies born with the disease, if untreated, cannot digest their food normally, fail to thrive, and are subject to severe, repeated and chronic lung infections which are the usual cause of death. When CF was first described in the 1930s, life expectancy was less than five years.

John Dodge (Dept of Child Health, University of Wales Swansea, UK) and his colleagues report on the way in which this outlook has improved during the intervening years. The authors followed up successive birth cohorts of people suffering from CF who were born between 1968 and 1995, and who had been entered into a national registry.

This registry included all patients known to the health services, whether or not they attended specialist CF clinics. Death certificates for all persons in the UK where CF was mentioned as a main or contributory cause were compared with registry data. Enquiries were made about the small number of deceased who were previously unknown, and any necessary corrections were made before survival and life expectancy were calculated.

The findings confirmed those of previous studies and demonstrated that modern management of CF is increasingly effective in prolonging life. About 250-300 children in the UK are born with CF annually, of whom all but about three survive the first decade and fewer than 20 fail to reach young adulthood. Each successive birth cohort since 1968 had an improving outlook and there is nothing to suggest that this trend will not continue. Even if no "breakthrough" in treatment occurs, on average a baby with CF born since 2000 could expect to survive for 50 years or more, a ten-fold increase since 1940.

Title of the original article:
Cystic fibrosis mortality and survival in the UK: 1947-2003

The European Respiratory Journal is the peer-reviewed scientific publication of the European Respiratory Society (more than 8,000 specialists in lung diseases and respiratory medicine in Europe, the United States and Australia).

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