Malena Cohen-Cymberknoh1, Bar Gindi Reiss2, Joel Reiter3, Noah Lechtzin4, Joel Melo5, Gema Pérez5, Hannah Blau6, Huda Mussaffi6, Hagit Levine6, Lea Bentur7, Michal Gur7, Galit Livnat8, Javier Perez Miranda9, Eva Polverino9, Francesco Blasi10, Stefano Aliberti10, Micha Aviram11, Inbal Golan Tripto11, Elie Picard12, Michal Novoselsky13, Hagai Amsalem13, Drorith Hochner Celnikier13, Eitan Kerem3, Michal Shteinberg8. 1. CF Center, Hadassah-Hebrew University Medical Center, Jerusalem, Israel. Electronic address: malena@hadassah.org.il. 2. Faculty of Medicine, Hebrew University, Jerusalem, Israel. 3. CF Center, Hadassah-Hebrew University Medical Center, Jerusalem, Israel. 4. Johns Hopkins University School of Medicine, Baltimore, MD, USA. 5. Instituto Nacional del Tórax, Santiago, Chile. 6. Graub CF Center of Schneider Children's Medical Center, Petach-Tikva and Sackler School of Medicine, Tel-Aviv University, Israel. 7. CF Center, Ruth Rappaport Children's Hospital, Rambam Medical Center, Haifa, Israel. 8. (7)Pulmonology institute and CF Center, Carmel Medical Center and the Technion- Israel Institute of Technology, Haifa, Israel. 9. CF Center, Hospital Universitari Vall d'Hebron Barcelona, Spain. 10. Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Adult CF Center, and Department Pathophysiology and Transplantation University of Milan, Italy. 11. Soroka Medical Center, Beer Sheva, Israel. 12. Pediatric Pulmonary Unit, Shaare Zedek Medical Center, Jerusalem, Israel. 13. Department of Obstetrics and Gynecology, Mount Scopus, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.
Abstract
BACKGROUND: With increasing longevity and quality of life in adults with Cystic fibrosis (CF), growing maternity rates are reported. Women with severe CF are becoming pregnant, with unpredictable maternal and fetal outcomes. AIM: To determine how baseline disease severity, pancreatic insufficiency (PI) and Pseudomonas aeruginosa (PA) infection affect fertility, the pregnancy course, delivery, neonatal outcome, and subsequent disease progression. METHODS: A multicenter-retrospective cohort study. Data on patients that had been pregnant between 1986-2018 was collected from ten CF centers worldwide. Disease severity [mild or moderate-severe (mod-sev)] was defined according to forced expiratory volume % predicted in 1 second (FEV1) and body mass index (BMI). Three time periods were compared, 12 months prior to conception, the pregnancy itself and the 12 months thereafter. RESULTS: Data was available on 171 pregnancies in 128 patients aged 18-45 years; 55.1% with mod-sev disease, 43.1% with PI and 40.3% with PA. Women with mod-sev disease had more CF-related complications during and after pregnancy and delivered more preterm newborns. However, FEV1 and BMI decline were no different between the mild and mod-sev groups. A more rapid decline in FEV1 was observed during pregnancy in PI and PA infected patients, though stabilizing thereafter. PI was associated with increased risk for small for gestational age infants. CONCLUSION: Baseline disease severity, PA infection and PI have an adverse impact on infant outcomes, but do not impact significantly on disease progression during and after pregnancy. Consequently, pregnancies in severe CF patients can have a good prognosis.
BACKGROUND: With increasing longevity and quality of life in adults with Cystic fibrosis (CF), growing maternity rates are reported. Women with severe CF are becoming pregnant, with unpredictable maternal and fetal outcomes. AIM: To determine how baseline disease severity, pancreatic insufficiency (PI) and Pseudomonas aeruginosa (PA) infection affect fertility, the pregnancy course, delivery, neonatal outcome, and subsequent disease progression. METHODS: A multicenter-retrospective cohort study. Data on patients that had been pregnant between 1986-2018 was collected from ten CF centers worldwide. Disease severity [mild or moderate-severe (mod-sev)] was defined according to forced expiratory volume % predicted in 1 second (FEV1) and body mass index (BMI). Three time periods were compared, 12 months prior to conception, the pregnancy itself and the 12 months thereafter. RESULTS: Data was available on 171 pregnancies in 128 patients aged 18-45 years; 55.1% with mod-sev disease, 43.1% with PI and 40.3% with PA. Women with mod-sev disease had more CF-related complications during and after pregnancy and delivered more preterm newborns. However, FEV1 and BMI decline were no different between the mild and mod-sev groups. A more rapid decline in FEV1 was observed during pregnancy in PI and PA infected patients, though stabilizing thereafter. PI was associated with increased risk for small for gestational age infants. CONCLUSION: Baseline disease severity, PA infection and PI have an adverse impact on infant outcomes, but do not impact significantly on disease progression during and after pregnancy. Consequently, pregnancies in severe CF patients can have a good prognosis.
Authors: Raksha Jain; Amalia Magaret; Phuong T Vu; Jill M VanDalfsen; Ashley Keller; Alexandra Wilson; Melissa S Putman; Nicole Mayer-Hamblett; Charles R Esther; Jennifer L Taylor-Cousar Journal: BMJ Open Respir Res Date: 2022-06