Pregnancy outcomes in patients from a Scottish Adult Cystic Fibrosis Unit taking elexacaftor/tezacaftor/ivacaftor,2020–present

Abstract

Background

Elexacaftor/tezacaftor/ivacaftor (ETI) was made available to eligible women in September 2020 by NHS Scotland.

Methods

Retrospective data collection for the 13 pregnancies in women taking ETI from the West of Scotland Adult Cystic Fibrosis Unit, September 2020–December 2023.

Results

Mean pre-pregnancy FEV1 was 2.26L, 70% predicted (range 1.25–3.19); (38–86% predicted). Mean FEV1 post-pregnancy was 2.29L, 71% predicted (range 1.49–3.40); (45–92% predicted). The mean age at conception (29 years) and mean percentage predicted FEV1 (70%) were higher than in other UK studies. Two pregnancies resulted in miscarriage, the remaining 11 pregnancies resulted in a live birth. Seven women had a pulmonary exacerbation of CF during pregnancy. Three of four women with FEV1 < 60% predicted had uncomplicated pregnancies with no pulmonary exacerbations.

Conclusion

We demonstrate that people with CF and varying spectrums of lung disease who take CFTR modulators can have uncomplicated pregnancies with positive lung function outcomes.