Outcomes of pregnancy in women with cystic fibrosis (CF) taking CFTR modulators - an international survey.

Nash, Edward F and Middleton, Peter G and Taylor-Cousar, Jennifer L (2020) Outcomes of pregnancy in women with cystic fibrosis (CF) taking CFTR modulators - an international survey. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society. ISSN 1873-5010. (In Press) This article is available to all UHB staff and students via ASK Discovery tool http://tinyurl.com/z795c8c by using their UHB Athens login IDs

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Official URL: https://www.cysticfibrosisjournal.com/inpress

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.

Item Type: Article
Additional Information: This article is available to all UHB staff and students via ASK Discovery tool http://tinyurl.com/z795c8c by using their UHB Athens login IDs
Subjects: WI Digestive system. Gastroenterology
Divisions: Planned IP Care > Respiratory Medicine
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Depositing User: Mrs Yolande Brookes
Date Deposited: 13 Mar 2020 14:42
Last Modified: 13 Mar 2020 14:42
URI: http://www.repository.uhblibrary.co.uk/id/eprint/2927

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