Clinician attitudes and practices on pregnancy planning and care in cystic fibrosis

Abstract

People with cystic fibrosis (CF) are living longer and healthier lives as a result of cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies, and are pursuing pregnancy. As the number of pregnancies in CF continue to increase, clinician attitudes and practices regarding care of pregnant people with CF remain largely unknown. To evaluate the current attitudes and practices of CF clinicians regarding pregnancy planning and care in CF. We conducted a national survey investigating practice patterns related to pregnancy care in CF. We used descriptive statistics to summarize responses and pairedt-tests to compare population means. A total of 93 clinicians completed the survey. Eighty-six percent of respondents believed family planning and pregnancy discussions should start before the age of 21 years, of which 67% believed these discussions should occur prior to age 18 years. Our results demonstrate variability in CF clinician comfort and management of various aspects of pregnancy care in CF including 1) potential complications of pregnancy 2) continuation of chronic CF therapies 3) continuation of CFTR modulators during pregnancy and lactation, and 4) approach to treatment of pulmonary exacerbation during pregnancy. As more people with CF pursue pregnancy in the era of CFTR modulators, CF providers should be initiating discussions surrounding pregnancy early and often. Establishing best practices in the management of pregnancy in CF, expanding peri‑pregnancy expertise within the CF community, and future studies investigating the maternal-fetal effects of CF therapies are needed.