Edward F Nash1, Peter G Middleton2, Jennifer L Taylor-Cousar3. 1. West Midlands Adult Cystic Fibrosis Centre, University Hospitals Birmingham NHS Foundation Trust, Bordesley Green East, Birmingham, United Kingdom. Electronic address: edward.nash@heartofengland.nhs.uk. 2. Westmead Clinical School, University of Sydney, Sydney, Australia. 3. National Jewish Health, Internal Medicine and Pediatrics, Pulmonary, Denver, Colorado, United States.
Abstract
BACKGROUND: As their long-term prognosis improves, women with CF are increasingly choosing to have children, but the safety of CFTR modulators in pregnancy and breastfeeding is currently unknown. METHODS: A survey was sent to lead clinicians of adult CF centres in Europe, the United Kingdom (UK), United States of America (USA), Australia and Israel requesting anonymised data on pregnancy outcomes in women using CFTR modulators before and during pregnancy and lactation. RESULTS: We identified 64 pregnancies in 61 women taking IVA (n = 31), LUM/IVA (n = 26) or TEZ/IVA (n = 7), resulting in 60 live births. In 44 pregnancies, CFTR modulators were either continued throughout pregnancy or temporarily stopped and then restarted. Two maternal complications were deemed related to CFTR modulator therapy; cessation of modulator therapy resulted in clinical decline in 9 women prompting resumption of therapy during pregnancy. No modulator-related complications were reported in infants exposed in utero and/or during breastfeeding. CONCLUSIONS: CFTR modulators were reported to be generally well tolerated in pregnancy and breastfeeding, with only 2 maternal complications that were deemed related to CFTR modulator therapy. Women stopping CFTR modulators in pregnancy may experience a decline in clinical status and in the cases identified in this survey, restarting therapy led to a clinical improvement. Current experience remains limited and longer-term prospective follow-up is required to exclude delayed adverse effects.
BACKGROUND: As their long-term prognosis improves, women with CF are increasingly choosing to have children, but the safety of CFTR modulators in pregnancy and breastfeeding is currently unknown. METHODS: A survey was sent to lead clinicians of adult CF centres in Europe, the United Kingdom (UK), United States of America (USA), Australia and Israel requesting anonymised data on pregnancy outcomes in women using CFTR modulators before and during pregnancy and lactation. RESULTS: We identified 64 pregnancies in 61 women taking IVA (n = 31), LUM/IVA (n = 26) or TEZ/IVA (n = 7), resulting in 60 live births. In 44 pregnancies, CFTR modulators were either continued throughout pregnancy or temporarily stopped and then restarted. Two maternal complications were deemed related to CFTR modulator therapy; cessation of modulator therapy resulted in clinical decline in 9 women prompting resumption of therapy during pregnancy. No modulator-related complications were reported in infants exposed in utero and/or during breastfeeding. CONCLUSIONS:CFTR modulators were reported to be generally well tolerated in pregnancy and breastfeeding, with only 2 maternal complications that were deemed related to CFTR modulator therapy. Women stopping CFTR modulators in pregnancy may experience a decline in clinical status and in the cases identified in this survey, restarting therapy led to a clinical improvement. Current experience remains limited and longer-term prospective follow-up is required to exclude delayed adverse effects.
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
Authors: Traci M Kazmerski; Natalie E West; Raksha Jain; Ahmet Uluer; Anna M Georgiopoulos; Moira L Aitken; Jennifer L Taylor-Cousar Journal: Pediatr Pulmonol Date: 2021-08-18
Authors: Kate E O'Connor; Dana L Goodwin; Andrew NeSmith; Bryan Garcia; Christina Mingora; Sigrid L Ladores; Steve M Rowe; Stefanie Krick; George M Solomon Journal: J Cyst Fibros Date: 2021-01-19 Impact factor: 5.527