Up-to-date and projected estimates of survival for people with cystic fibrosis using baseline characteristics: A longitudinal study using UK patient registry data

Keogh, Ruth, Szczesniak, Rhonda, Taylor-Robinson, DC ORCID: 0000-0002-5828-7724 and Bilton, Diana (2018) Up-to-date and projected estimates of survival for people with cystic fibrosis using baseline characteristics: A longitudinal study using UK patient registry data. Journal of Cystic Fibrosis, 17 (2). pp. 218-227.

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Official URL: https://www.cysticfibrosisjournal.com/article/S156...

Abstract

Background Cystic fibrosis (CF) is the most common inherited disease in Caucasians, affecting around 10,000 individuals in the UK today. Prognosis has improved considerably over recent decades with ongoing improvements in treatment and care. Providing up-to-date survival predictions is important for patients, clinicians and health services planning. Methods Flexible parametric survival modelling of UK CF Registry data from 2011 to 2015, capturing 602 deaths in 10,428 individuals. Survival curves were estimated from birth; conditional on reaching older ages; and projected under different assumptions concerning future mortality trends, using baseline characteristics of sex, CFTR genotype (zero, one, two copies of F508del) and age at diagnosis. Findings Male sex was associated with better survival, as was older age at diagnosis, but only in F508del non-homozygotes. Survival did not differ by genotype among individuals diagnosed at birth. Median survival ages at birth in F508del homozygotes were 46 years (males) and 41 years (females), and similar in non-homozygotes diagnosed at birth. F508del heterozygotes diagnosed aged 5 had median survival ages of 57 (males) and 51 (females). Conditional on survival to 30, median survival age rises to 52 (males) and 49 (females) in homozygotes. Mortality rates decreased annually by 2% during 2006–2015. Future improvements at this rate suggest median survival ages for F508del homozygous babies of 65 (males) and 56 (females). Interpretation Over half of babies born today, and of individuals aged 30 and above today, can expect to survive into at least their fifth decade.

Item Type:	Article
Uncontrolled Keywords:	CFTR Genotype, Cystic fibrosis, Flexible parametric survival model
Depositing User:	Symplectic Admin
Date Deposited:	08 Jan 2018 07:28
Last Modified:	19 Jan 2023 06:46
DOI:	10.1016/j.jcf.2017.11.019
Open Access URL:	http://www.sciencedirect.com/science/article/pii/S...
Related URLs:	Author Publisher
URI:	https://livrepository.liverpool.ac.uk/id/eprint/3015600