Gestational weight gain and health-related quality of life during pregnancy and the early postpartum period in women with cystic fibrosis receiving elexacaftor/tezacaftor/ivacaftor.

Background

Highly effective CFTR modulator therapy, such as elexacaftor/tezacaftor/ivacaftor (ETI), has significantly increased pregnancy rates in women with cystic fibrosis (wwCF). Data on health-related quality of life (HRQoL) during pregnancy and the early postpartum period under ETI remain limited.

Methods

This retrospective observational study evaluated 18 wwCF receiving ETI during pregnancy. Maternal outcomes (lung function, BMI, gestational weight gain, pulmonary exacerbations) and HRQoL using the Cystic Fibrosis Questionnaire-Revised (CFQ-R) were assessed at baseline, during pregnancy, and postpartum.

Results

Maternal lung function remained stable across pregnancy and the postpartum period, while pulmonary exacerbations occurred in 50% of pregnancies. Gestational weight gain (GWG) deviated in 16 of 17 women (94%) from Institute of Medicine (IOM) recommendations: 8/17 (47%) had insufficient and 8/17 (47%) excessive GWG, while only 1/17 (6%) achieved adequate GWG. All women with insufficient GWG had a baseline BMI of 18.5-24.9 kg/m2, whereas excessive GWG occurred across all baseline body mass index (BMI) categories. In exploratory CFQ-R analyses, the largest changes were observed in the physical, respiratory, and social domains, with lower physical and social scores observed postpartum compared with baseline.

Conclusions

Pregnancy in wwCF receiving ETI was associated with preserved maternal pulmonary stability. The high rate of GWG outside recommended ranges and exploratory changes in selected HRQoL identify nutritional and patient-reported outcomes that warrant further prospective investigation.