Management of a parturient with respiratory failure secondary to cystic fibrosis

Abstract

A 21-year-old with end-stage respiratory failure and cor pulmonale due to cystic fibrosis became pregnant. Her forced expiratory volume in 1 s (FEV1) was 17% of that predicted antenatally, and termination was strongly advised on the grounds that continuing pregnancy was likely to be fatal. She elected to continue with the pregnancy and gave birth by lower segment caesarean section at 29 weeks and 5 days' gestation. Despite a problematic postnatal course, she was successfully discharged home and, 7 months postnatally, continues to live at home with her partner and their baby. There are no previous reports of cystic fibrosis patients with this level of respiratory impairment surviving pregnancy.