CHEST Guidelines-Cystic-Fibrosis-Transmembrane-Conductance-Regulato

Cystic-Fibrosis-Transmembrane-Conductance-Regulato

Pdf Summary

The advancement in cystic fibrosis (CF) management has improved life expectancy and quality of life for people with CF (pwCF), allowing them to reach adulthood, pursue careers, and have families. CF management has transitioned from supportive therapies to include cystic fibrosis transmembrane conductance regulator (CFTR) modulators, which correct and potentiate the defective CFTR protein. Notable medications, such as ivacaftor and the combination therapies elexacaftor-tezacaftor-ivacaftor, have substantially improved lung function and quality of life. However, CF impacts both fertility and pregnancy outcomes, historically posing significant reproductive challenges for women with CF (wwCF), primarily due to respiratory and extrapulmonary complications.<br /><br />A recent study by Jain et al. examined the effects of CFTR modulators during pregnancy, indicating improved maternal outcomes, such as increased lung function and reduced need for assisted reproductive technologies. The study suggests CFTR modulators could improve fertility, possibly through enhanced respiratory health.<br /><br />The safety of CFTR modulators in pregnancy is still under investigation, as concerns about potential embryotoxicity and effects on infants, like liver enzyme disturbance and cataracts, remain. The ongoing "MAYFLOWERS" study aims to further assess these outcomes. Despite safety uncertainties, improved maternal health from modulator use could favorably impact fetal outcomes, making continued use during pregnancy increasingly common among wwCF.<br /><br />The editorial highlights the evolving landscape of CF management, weighing the benefits of CFTR modulators against potential fetal risks. It emphasizes that while more systematic safety data are needed, the improved maternal health associated with these treatments tends to guide the decision toward continued use, potentially offering improved outcomes in both maternal and infant health contexts.

Keywords

cystic fibrosis

CF management

CFTR modulators

ivacaftor

elexacaftor-tezacaftor-ivacaftor

fertility

pregnancy outcomes

maternal health

Jain et al. study

MAYFLOWERS study