Rate of improvement of CF life expectancy exceeds that of general population—Observational death registration study

Abstract Background It is unclear why cystic fibrosis (CF) survival has improved. We wished to quantify increases in CF median age of death in the context of general population survival improvement. Method Death registration data analysis (US, England & Wales (E&W)—1972–2009). Results CF med...

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Bibliographic Details
Published in:Journal of cystic fibrosis 2014-07, Vol.13 (4), p.410-415
Main Authors: Hurley, Matthew N, McKeever, Tricia M, Prayle, Andrew P, Fogarty, Andrew W, Smyth, Alan R
Format: Article
Language:English
Subjects:
Adolescent
Adult
Age Distribution
Age Factors
Cause of Death - trends
Child
Child, Preschool
Cystic fibrosis
Cystic Fibrosis - mortality
England - epidemiology
Female
Healthcare
Humans
Infant
Life Expectancy
Male
Middle Aged
Mortality
Original
Population Surveillance - methods
Pulmonary/Respiratory
Registries
Retrospective Studies
Survival
Survival Rate - trends
United States - epidemiology
Wales - epidemiology
Young Adult
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Summary:Abstract Background It is unclear why cystic fibrosis (CF) survival has improved. We wished to quantify increases in CF median age of death in the context of general population survival improvement. Method Death registration data analysis (US, England & Wales (E&W)—1972–2009). Results CF median age of death is higher in US than E&W and greater for males, opposite to that of death from all causes. CF median age of death has increased by 0.543 life years per year (E&W, US combined (95% confidence interval 0.506, 0.582)). The difference in median age at death between those dying from all causes and CF decreased in both territories. CF median age of death for males is greater than for females in both territories. This gap has not narrowed. Conclusion The median age of death of people with CF is improving more rapidly than that of the general population in US and E&W.
ISSN:1569-1993
1873-5010
DOI:10.1016/j.jcf.2013.12.002