Maternal and Perinatal Outcomes in Pregnant Women with Cystic Fibrosis

Abstract

Objectives To assess the perinatal and maternal outcomes of pregnant women with cystic fibrosis (CF) and severe lung impairment. Methods This was a series of cases aiming to review the maternal and fetal outcomes in cases of singleton pregnant women with a diagnosis of CF. We have included all of the cases of singleton pregnancy in patients with CF who were followed-up at the obstetrics department of the Medical School of the Universidade de São Paulo, between 2003 and 2016. The exclusion criteria were the unattainability of the medical records of the patient, and delivery at other institutions. A forced expiratory volume in 1 second < 50% was considered as severe lung impairment. We have also analyzed data regarding maternal hospitalization and respiratory exacerbations (REs). Results Pregnant women with CF and severe lung impairment did not present an association with spontaneous prematurity, fetal growth restriction or fetal demise. All of the cases involved multiple RE episodes requiring antibiotic therapy. The median (range) of events per patient was of 4 (2–4) events. Conclusion Cystic fibrosis patients with severe lung impairment may achieve successful term pregnancies. However, pregnancies of women with CF are frequently complicated by REs, and this population may require hospital admission during the course of the pregnancy. Cystic fibrosis patients should be followed by a specialized team with experience in treating respiratory diseases.

Predictive factors for premature birth and respiratory exacerbation in pregnancies of women with cystic fibrosis

“…The main adverse maternal outcome observed in the present study was respiratory exacerbation during pregnancy (84.6% prevalence rate). Similar results have been reported by previous studies, 13 , 24 and only one case-control study indicated no difference (p > 0.2) between rates of respiratory exacerbation between pregnant and non-pregnant women with CF. However, the sample size was small and modest differences between these groups may have been lost.…”

“…Despite these findings, maternal-fetal outcomes in women with CF have not yet been exhaustively explored, 12 and few Brazilian studies are available for building an arsenal of evidence capable of leading to strong conclusions. 13 …”

Effects of modulator therapies on endocrine complications in adults with cystic fibrosis: a narrative review

“…The rates of spontaneous miscarriage in women with cystic fibrosis appear comparable to the general population 78 . There is evidence that pregnancy overall does not affect survival in cystic fibrosis; 79 however, higher rates of Caesarean delivery, diabetes, preterm birth, congenital anomalies, 80,81 and pulmonary decline in the mother have been reported 82 …”