{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T19:46:49Z","timestamp":1791229609629,"version":"4.1.0"},"reference-count":43,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2023,10,3]],"date-time":"2023-10-03T00:00:00Z","timestamp":1696291200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2023,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Safety and efficacy data regarding cystic fibrosis transmembrane conductance regulator (CFTR) modulator use in the setting of pregnancy or breastfeeding remains lacking due to exclusion from key trials and lack of multicenter prospective and retrospective studies in the post\u2010CFTR modulator era. A scoping review of English articles from the period of January 1, 2012, to July 31, 2023, was conducted utilizing PubMed and EmBase databases with the following terms: \u201cspecial population (pregnancy, lactation, breastfeeding)\u201d AND \u201civacaftor OR lumacaftor OR tezacaftor OR elexacaftor\u201d; \u201ccystic fibrosis transmembrane conductance regulator\u201d AND \u201coff label drug use.\u201d Search results were reviewed by title and abstract for duplications and relevance. Relative to pregnancy or breastfeeding, a total of 18 publications were included for review. Majority of case reports and surveys concluded maternal and infant health were preserved throughout gestation. Likewise, breastfeeding infant case reports show possible changes in liver function and lens opacities, though risk may be increased with both in\u2010utero and breastfeeding exposure. Ivacaftor (IVA) and lumacaftor (LUM) concentrations in fetal cord blood and maternal blood were found to be equivalent. Yet, low concentrations of IVA and LUM were detectable in breastmilk and infant plasma. Current safety data surrounding CFTR modulator use in the setting of pregnancy and lactation is relatively reassuring; however, long\u2010term safety remains unclear, necessitating ongoing observation, and reporting by care teams. As such, treatment decisions should be individualized and coproduced.<\/jats:p>","DOI":"10.1002\/ppul.26706","type":"journal-article","created":{"date-parts":[[2023,10,4]],"date-time":"2023-10-04T08:33:01Z","timestamp":1696408381000},"page":"3377-3385","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Use of CFTR modulators in special populations, part 1: Pregnancy and lactation"],"prefix":"10.1002","volume":"58","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3933-473X","authenticated-orcid":false,"given":"Joseph","family":"Elijah","sequence":"first","affiliation":[{"name":"Department of Pharmacy Services Michigan Medicine Ann Arbor Michigan USA"},{"name":"Department of Clinical Pharmacy College of Pharmacy, University of Michigan Ann Arbor Michigan USA"},{"name":"School of Pharmacy and Pharmaceutical Sciences Bouve College of Health Sciences, Northeastern University Boston United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Linda J.","family":"Fitzgerald","sequence":"additional","affiliation":[{"name":"Department of Pharmacy Services Michigan Medicine Ann Arbor Michigan USA"},{"name":"Department of Clinical Pharmacy College of Pharmacy, University of Michigan Ann Arbor Michigan USA"},{"name":"Sanofi Medical Affairs Bridgewater New Jersey USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4968-1460","authenticated-orcid":false,"given":"Hanna","family":"Phan","sequence":"additional","affiliation":[{"name":"Department of Pharmacy Services Michigan Medicine Ann Arbor Michigan USA"},{"name":"Department of Clinical Pharmacy College of Pharmacy, University of Michigan Ann Arbor Michigan USA"},{"name":"Susan B. Meister Child Health Evaluation and Research (CHEAR) Center University of Michigan Ann Arbor Michigan USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,10,3]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0039-1694021"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2022.10.008"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.2570460"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.2475911"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.2772657"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.biocel.2014.02.004"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1186\/1465-9921-4-8"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.3390\/jpm11050418"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1569-1993(22)00693-2"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajog.2014.07.018"},{"key":"e_1_2_9_12_1","article-title":"Association between unplanned pregnancies and maternal exacerbations in cystic fibrosis","author":"Peng G","year":"2023","journal-title":"J Cyst Fibros"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2021.12.004"},{"key":"e_1_2_9_14_1","unstructured":"Kalydeco\u2122. 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