Pregnancy in cystic fibrosis: Review of the literature and expert recommendations

J Cyst Fibros. 2022 May;21(3):387-395. doi: 10.1016/j.jcf.2021.07.019. Epub 2021 Aug 26.

Abstract

Cystic fibrosis (CF) was historically a disease largely afflicting children. Due to therapeutic advancements, there are now more adults with CF than children. In the past decade, medications including Cystic Fibrosis Transmembrane conductance Regulator (CFTR) modulators became available that treat the underlying cause of CF and are dramatically improving lung function as well as quality and quantity of life for people with CF. As a result, more women with CF are becoming pregnant. We gathered a panel of experts in CF care, family planning, high risk obstetrics, nutrition, genetics and women with CF to review current literature on pregnancies and to provide care recommendations for this unique population.

Keywords: Cystic fibrosis; Family planning; Fertility; Parenthood; Pregnancy.

Publication types

  • Review
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Child
  • Cystic Fibrosis Transmembrane Conductance Regulator / genetics
  • Cystic Fibrosis Transmembrane Conductance Regulator / metabolism
  • Cystic Fibrosis* / epidemiology
  • Cystic Fibrosis* / genetics
  • Cystic Fibrosis* / therapy
  • Female
  • Humans
  • Ion Transport
  • Pregnancy

Substances

  • Cystic Fibrosis Transmembrane Conductance Regulator