Lung health and pregnancy in the cystic fibrosis (CF) modulator era

<bold>Introduction:</bold> Women with CF (wwCF) have improved health on elexacaftor/tezacaftor/ivacaftor (ETI), and pregnancy rates are rising. <bold>Methods:</bold> We reviewed all pregnancies at our centre since 2020 in relation to lung function at baseline and at delivery, use of ETI, CF exacerbations, planning for pregnancy and outcomes. <bold>Results:</bold> 30 wwCF had 38 pregnancies (5 in 2020, 7 in 2021, 9 in 2022 and 17 in 2023) at a mean age of 27.5 (18.5-43) years. Mean BMI was 25.5 (18.6-34.4); 17 had Pseudomonas, 11 Staphylococcal and 2 Burkholderia infections. Mean baseline FEV1 was 79.3(45-121)%predicted: 5 stopped ETI and their FEV1 fell by a mean 13.6 (5-28)%; in those continuing ETI, FEV1 was stable or improved by a mean 4.35 (−3-+27)%. One wwCF had recurrent exacerbations and FEV1 fell from 96% to 62%; 5 needed intravenous antibiotics for exacerbations, 9 had CF diabetes pre-pregnancy and 8 developed gestational diabetes. 3 had had in-vitro fertilisation (2 for subfertility and one with preimplantation genetic tests as partner was a carrier of CF); one same-sex couple used insemination with donor sperm. 12 pregnancies were not planned and 3 had terminations. There were 6 miscarriages; delivery was by Caesarean section in 8, induction at 38 weeks in 9, and 3 were premature at 34-35 weeks gestation, with some pregnancies on-going. All babies were well with no CF or anomalies. Over the 4 years 2 wwCF had advice against pregnancy because of FEV1 <25%; one has now had two children via surrogacy. <bold>Conclusions:</bold> Many wwCF are now healthy, opt to continue ETI and tolerate pregnancy well. Stopping ETI results in a fall in FEV1. Some wwCF have advanced disease that makes pregnancy inadvisable but can consider options such as adoption or surrogacy.