Pregnancy in Cystic Fibrosis-Past, Present, and Future

Abstract

The introduction of mutation-specific therapy led to a revolution in cystic fibrosis (CF) care. These advances in CF therapies have changed the disease profile from a severe incurable disease with limited survival to a treatable disease with improved quality of life and survival into adulthood. CF patients are now able to plan their future, including marriage and parenthood. Side by side with the optimism, new issues and concerns are arising, including fertility and preparation for pregnancy, maternal and fetal care during pregnancy, and post-partum care. While cystic fibrosis transmembrane regulator (CFTR) modulators show promising results for improving CF lung disease, data on their safety in pregnancy are still limited. We performed a literature review on pregnancy in CF from the past, with the first described pregnancy in 1960, through the current fascinating changes in the era of CFTR modulators, to ongoing studies and future directions. Current advances in knowledge give hope for improved outcomes of pregnancy, towards the best possible prognosis for the mother and for the baby.

Keywords: CFTR modulators; cystic fibrosis; pregnancy.

Figure 1

Summary of recommendations pre-conception, during pregnancy, and post-partum. CFRD = cystic fibrosis-related diabetes; BMI = body mass index; FEV1 = forced expiratory volume in one second; GER = gastro-esophageal reflux; IV = intravenous; LTX = lung transplantation.