• Raksha Jain, Giselle Peng, MinJae Lee, Ashley Keller, Sophia Cosmich, Sarthak Reddy, Natalie E West, Traci M Kazmerski, Jennifer L Goralski, Patrick A Flume, Andrea H Roe, Denis Hadjiliadis, Ahmet Uluer, Sheila Mody, Sigrid Ladores, and Jennifer L Taylor-Cousar.
• University of Texas Southwestern Medical Center, Dallas, TX. Electronic address: Raksha.Jain@utsouthwestern.edu.
• Chest. 2024 Sep 27.

BackgroundCystic fibrosis (CF) transmembrane conductance regulator (CFTR) modulators are available to the majority of people with CF in the United States; little is known about pregnancy outcomes with modulator use. The aim of this retrospective study was to determine the impact of CFTR modulators on maternal outcomes.Research QuestionDoes pregnancy differentially affect outcomes in female subjects with CF with and without CFTR modulator exposure?Study Design And MethodsData on pregnancies from 2010 to 2021 were collected from 11 US adult CF centers. Multivariable longitudinal regression analysis was performed to assess whether changes in percent predicted FEV1 (ppFEV1), BMI, pulmonary exacerbations (PEx), and Pseudomonas aeruginosa prevalence differed from prior to, during, and following pregnancy according to CFTR modulator use while adjusting for confounders. Infant outcomes are also described based on maternal modulator use.ResultsAmong 307 pregnancies, mean age at conception was 28.5 years (range, 17-42 years), prepregnancy ppFEV1 was 74.2, and BMI was 22.3 kg/m2. A total of 114 pregnancies (37.1%) had CFTR modulator exposure during pregnancy (77 with highly effective modulator therapy [HEMT] and 37 with other modulators). The adjusted mean change in ppFEV1 from prepregnancy to during pregnancy was -2.36 (95% CI, -3.56 to -1.16) in the unexposed group and 2.60 (95% CI, 0.23 to 4.97) in the HEMT group, with no significant change from during pregnancy to 1 year postpregnancy. There was an overall decline in ppFEV1 from prepregnancy to postpregnancy in the no modulator group (-2.56; 95% CI, -3.62 to -1.49) that was not observed in the HEMT group (1.10; 95% CI, -1.13 to 3.34). PEx decreased from prepregnancy to postpregnancy in the HEMT group, and BMI increased from prepregnancy to during pregnancy in all groups but with no significant change postpregnancy. Missing infant outcomes data precluded firm conclusions.InterpretationWe observed superior pregnancy and postpregnancy pulmonary outcomes in individuals who used HEMT, including a preservation of ppFEV1, compared with those unexposed to HEMT.Copyright © 2024 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.

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