Research shows positive impact of CFTR modulators on pregnancy outcomes in people with cystic fibrosis

DENVER - A recent retrospective study published in CHEST(Opens in a new window) evaluated the effects of cystic fibrosis transmembrane conductance regulator (CFTR) modulator use on pregnancy outcomes in individuals with cystic fibrosis (CF). Conducted across 11 U.S. adult CF centers, including National Jewish Health as one of the two lead sites, the study analyzed data from 307 pregnancies between 2010 and 2021. A class of drugs called CFTR modulators improve CFTR protein function in people with CF, vastly improving the lung health, weight and quality of life. The main goal of the study was to determine how CFTR modulators impact maternal health during and after pregnancy.

“This is the biggest study in the modern era of CF pregnancies," said Jennifer Taylor-Cousar, MD, pulmonologist and senior author of the study. Dr. Taylor-Cousar also is the Co-Director of the Adult CF Program at National Jewish Health. “There used to be 200 or fewer pregnancies for people with CF per year in the U.S. because women with CF died before childbearing age or couldn’t sustain the pregnancy due to their disease. Pregnancy outcomes seem to be better for women who use modulators, and babies who were exposed to modulators during pregnancy were generally healthy,” said Dr. Taylor-Cousar.

The researchers looked at medical records of women who were either on or off modulators who became pregnant in the last 10 years. Pregnant individuals with CF who used highly effective CFTR modulators showed improvements in lung function during pregnancy, with a significant positive change in lung function [as measured by percent predicted FEV1 (ppFEV1)], while those without modulator use saw a decline in lung function.

These results suggest that CFTR modulator use during pregnancy may have a beneficial effect on maternal health, particularly lung function, and are likely safe for the exposed babies. Because the results were only studied at a relatively small number of centers and the results were assessed for a relatively short period of time, the study highlights the need for further studies on modulator use during pregnancy and its long-term effects on both mothers and infants.

With 41 sites participating, The Maternal and Fetal Outcomes in the Era of Modulators (MAYFLOWERS) study is ongoing and represents the next step in this research. The study is prospectively following more than 300 women throughout pregnancy and after the baby is born.
 

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