Successful Pregnancy in a Patient With Advanced Cystic Fibrosis Lung Disease and <i>Burkholderia cenocepacia</i> Colonization

Cystic fibrosis (CF) affects multiple aspects of health, including reproduction and fertility. This report details a case of a 38-year-old woman with several complications from her CF, including advanced CF lung disease, pancreatic exocrine insufficiency, asthma, and airway colonization with Burkholderia cenocepacia, who became pregnant and successfully delivered a baby boy without significant complications. This was an unplanned pregnancy 4 years after initiation of elexaftor/tezacaftor/ivacaftor therapy, a CF transmembrane conductance regulator modulator therapy that has significantly improved various health outcomes in patient populations with CF, including the reproductive health of women.