A late preterm pregnancy in a patient with cystic fibrosis (CF): case report

Background. Literature suggests that maternal and fetal pregnancy outcomes are satisfactory for cystic fibrosis (CF) affected women with well controlled clinical features. We present a case highlighting that even in CF patients in stable conditions the risk of severe clinical deterioration in the course of pregnancy is not to be overlooked.
Case presentation. A 25-year-old woman with CF and a history of a previous pregnancy ended in miscarriage. She presented a pre-pregnancy BMI of 21.1 kg/m², FEV1 60%, and was colonized by MSSA, therefore resulting in a low risk profile for pregnancy. The patient’s general conditions progressively worsened, with four pulmonary infective exacerbations, a gradual decrease of pulmonary function, need of noninvasive oxygen support, and deterioration of nutritional status despite parenteral nutrition (weight before pregnancy 56 kg, at 36 weeks 54 kg). Anticipation of delivery was decided. A healthy female child was delivered by elective cesarean section at 36 + 0 weeks, Apgar 1’ 9-5’ 10, weight at birth 2850 g. She was discharged at 4 days with 12.6% loss of the birth weight and mild jaundice. In the following days feedings were switched to infant formula exclusive nutrition, due to lack of breast milk. The patient was discharged 9 days postpartum, after a 12-day i.v. antibiotic cycle, with improved general conditions and basal pulmonary function comparable to usual, pre-pregnancy, standards.
Conclusions. Even in CF women with a low risk profile before pregnancy, a careful monitoring of pregnancy and active collaboration between the CF center, anesthesiology and the Gynecological/Obstetrical Unit is highly recommendable.