Pregnancy and perinatal outcomes for women with Cystic Fibrosis: a UK
population-based cross-sectional study, 2003-2017
Abstract
Objective To estimate the pregnancy rates and outcomes for women with
cystic fibrosis (wwCF) in the UK compared to the general population and
to explore the impact of the introduction of disease modifying
treatments on pregnancy rates. Design A population-based cross-sectional
study. Setting Electronic records of UK CF Registry Data
(~99% of all CF), and conceptions data for England and
Wales (E&W). Population All women aged 15-44 years who were pregnant
between 2003-2017. Methods We calculated 3-yearly crude and age-specific
pregnancy rates per 1,000 women years (wys), pregnancy rates for wwCF
with a G551D mutation before and after Ivacaftor was introduced in 2012
and compared live birth rates. Main outcome measures Crude rates, age
specific fertility, and maternal morbidity. Results The overall
pregnancy rate was 23.5 (95% CI 21.9-25.3) per 1,000 wys,
~3.4fold difference to E&W women (77.7). This pattern
was evident in the age specific rates, except for those aged 40-44 years
where the difference in rates was much less (wwCF 8.2 per 1,000 wys vs.
13.3 in E&W). LB rate differences mirrored pregnancy rates (wwCF 17.4
per 1000 wys vs. 61.4 E&W women). Following the introduction of
Ivacaftor, pregnancy rates in wwCF with G551D increased from 29.5 to
56.9 per 1000wys (2012-2014 to 2015-2017). Conclusions Pregnancy rates
in wwCF are about a third of the rates in the general population but on
the rise following the introduction of Ivacaftor. There is no indication
that there is a reduced chance of a live birth in wwCF who become
pregnant.