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Observational Study
. 2014 Jul;13(4):410-5.
doi: 10.1016/j.jcf.2013.12.002. Epub 2014 Jan 10.

Rate of improvement of CF life expectancy exceeds that of general population--observational death registration study

Matthew N Hurley  1 , Tricia M McKeever  2 , Andrew P Prayle  3 , Andrew W Fogarty  2 , Alan R Smyth  3
Affiliations
Observational Study

Rate of improvement of CF life expectancy exceeds that of general population--observational death registration study

Matthew N Hurley et al. J Cyst Fibros. 2014 Jul.

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.

Keywords: Cystic fibrosis; Healthcare; Mortality; Survival.

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Figures

Fig. 1
Fig. 1
Median age of death in England & Wales and the US for CF and all-cause mortality with milestones in care , .
Fig. 2
Fig. 2
Linear regression model of differences between CF and all-cause childhood (1–19 years) mortality.
See this image and copyright information in PMC

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