PT - JOURNAL ARTICLE AU - Christina Keßler AU - Angelika Dübbers AU - Jörg Große-Onnebrink AU - Heymut Omran TI - Pregnancy in two patients with cystic fibrosis treated with lumacaftor/ivacaftor AID - 10.1183/13993003.congress-2019.PA4517 DP - 2019 Sep 28 TA - European Respiratory Journal PG - PA4517 VI - 54 IP - suppl 63 4099 - http://erj.ersjournals.com/content/54/suppl_63/PA4517.short 4100 - http://erj.ersjournals.com/content/54/suppl_63/PA4517.full SO - Eur Respir J2019 Sep 28; 54 AB - Introduction: The effect of CFTR modulators on the fecundity of patients with cystic fibrosis (CF) is unknown. Good research practice prohibits participation of patients attempting pregnancy in clinical trials, resulting in a lack of safety data. We report two pregnancies in CF-patients on lumacaftor/ivacaftor.Cases: Case 1 is 29 years old, homozygous for F508del, body mass index (BMI) 20,3 kg/m2, FEV1 58,6 % pred. She was on lumacaftor/ivacaftor since Febuary 2016 using hormonal contraceptives unregularly and against medical advice. The CFTR-modulator was discontinued when pregnancy was detected at 8 weeks gestation. FEV1%pred declined to 45,6 at week 32 and weight gain was only 4 kg. After repeated pulmonary exacerbations needing iv-therapy, a caesarean section was performed at 35+2 weeks. The infant needed ventilatory support due to prematurity but was otherwise healthy, birth weight 2690 g. After two months FEV1%pred was 48, BMI 20,1 kg/m2.Case 2 is also 29 years old, homozygous for F508del, BMI 20,6 kg/m2, FEV1%pred 39,2 with CF-diabetes. She had unprotected sexual intercourse for over a decade. After treatment with lumacaftor/ivacaftor for two years, a pregnancy was diagnosed at 20 weeks gestation. The patient decided to continue lumacaftor/ivacaftor. FEV1%pred at 36 weeks was 43,9 and weight gain was 9 kg. She gave birth to a healthy girl, birth weight 2440 g via planned caesarean section at 37 weeks. After two months FEV1%pred was 39,2, BMI 20,3 kg/m2.Conclusions: There might be an effect of CFTR-modulators on fecundity and the course and outcome of pregnancy in CF-patients. More data are needed to support the safety or risk for infants of mothers treated with CFTR-modulators.FootnotesCite this article as: European Respiratory Journal 2019; 54: Suppl. 63, PA4517.This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).