Comparison of two pregnancies with and without elexacaftor-tezacaftor-ivacaftor in a woman with cystic fibrosis

Chamagne, Matthieu; Farabet, Clémence; Grenet, Dominique; Ayoubi, Jean Marc

doi:10.1016/j.resmer.2022.100953

Cited by 4 publications

(10 citation statements)

References 7 publications

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“…Thirteen case reports included pregnant pwCF who remained on IVA (four), LUM/IVA (three), or ELE/TEZ/IVA (six) throughout all three trimesters 12,18–27 . One pregnant person on IVA and another pregnant person on LUM/IVA remained on modulator therapy throughout all three trimesters for two separate pregnancies 22,23 .…”

Section: Resultsmentioning

confidence: 99%

“…Likewise, a case report by Chamagne et al reported the impact of ELE/TEZ/IVA on maternal and fetal outcomes for a pwCF who delivered one infant before and one infant after ELE/TEZ/IVA initiation 27 . Marked improvement was observed in ppFEV 1 (37%–53% 1 month after modulator initiation; 60% at predelivery), pulmonary exacerbations requiring antibiotic therapy and hospitalization (four pre‐ELE/TEZ/IVA vs. one post‐ELE/TEZ/IVA), as well as increase in body weight during pregnancy 27 …”

Section: Resultsmentioning

confidence: 99%

“…Three case reports disclosed that cataracts were not observed infants exposed to modulator therapy in‐utero, whereas the remaining case reports lacked mention of the presence or absence of cataracts in the newborn 18–27 . Similarly, cataracts were not detected in a small cohort of infants who underwent a formal evaluation after exposure to CFTR modulators in‐utero 29,30 .…”

Section: Resultsmentioning

confidence: 99%

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Use of CFTR modulators in special populations, part 1: Pregnancy and lactation

Elijah,

Fitzgerald,

Phan

2023

Pediatric Pulmonology

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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‐CFTR 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: “special population (pregnancy, lactation, breastfeeding)” AND “ivacaftor OR lumacaftor OR tezacaftor OR elexacaftor”; “cystic fibrosis transmembrane conductance regulator” AND “off label drug use.” 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‐utero 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‐term safety remains unclear, necessitating ongoing observation, and reporting by care teams. As such, treatment decisions should be individualized and coproduced.

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

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Use of CFTR modulators in special populations, part 1: Pregnancy and lactation

Elijah,

Fitzgerald,

Phan

2023

Pediatric Pulmonology

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“…In another study, Chamagne and collaborators compared two pregnancies of a woman with CF receiving or not ETI therapy in each case [ 112 ]. During the first pregnancy, she was 32 years old, not using ETI therapy, and had chronic infection with P. aeruginosa and methicillin-resistant S. aureus , pancreatic insufficiency, and CFRD.…”

Section: Clinical Outcomes Of Eti Therapy In Case Reports Observation...mentioning

confidence: 99%

“…There was a membrane rupture at 34.5 weeks of gestation and a caesarian section was performed. They were able to return home 11 days after childbirth [ 112 ].…”

Section: Clinical Outcomes Of Eti Therapy In Case Reports Observation...mentioning

confidence: 99%

Elexacaftor-Tezacaftor-Ivacaftor: A Life-Changing Triple Combination of CFTR Modulator Drugs for Cystic Fibrosis

et al. 2023

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Cystic fibrosis (CF) is a potentially fatal monogenic disease that causes a progressive multisystemic pathology. Over the last decade, the introduction of CF transmembrane conductance regulator (CFTR) modulator drugs into clinical practice has profoundly modified the lives of many people with CF (PwCF) by targeting the fundamental cause of the disease. These drugs consist of the potentiator ivacaftor (VX-770) and the correctors lumacaftor (VX-809), tezacaftor (VX-661), and elexacaftor (VX-445). In particular, the triple combination of CFTR modulators composed of elexacaftor, tezacaftor, and ivacaftor (ETI) represents a life-changing therapy for the majority of PwCF worldwide. A growing number of clinical studies have demonstrated the safety and efficacy of ETI therapy in both short- and long-term (up to two years of follow-up to date) and its ability to significantly reduce pulmonary and gastrointestinal manifestations, sweat chloride concentration, exocrine pancreatic dysfunction, and infertility/subfertility, among other disease signs and symptoms. Nevertheless, ETI therapy-related adverse effects have also been reported, and close monitoring by a multidisciplinary healthcare team remains vital. This review aims to address and discuss the major therapeutic benefits and adverse effects reported by the clinical use of ETI therapy for PwCF.

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Cystic fibrosis year in review 2022

Kim,

Lyman,

Savant

2023

Pediatric Pulmonology

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Remarkable medical advancements have been made for people with cystic fibrosis (CF) in recent years, with an abundance of research continuing to be conducted worldwide. With concern for limitations in access to highly effective CFTR modulators, as well as the recent Coronavirus Disease‐19 pandemic, there has been a consistent effort to understand and improve CF screening, disease burden, diagnosis, and management. Our aim in this review is to present articles from 2022 with an emphasis on clinically relevant studies. We hope this will serve as a broad overview of the research published in the past year.

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Comparison of two pregnancies with and without elexacaftor-tezacaftor-ivacaftor in a woman with cystic fibrosis

Cited by 4 publications

References 7 publications

Use of CFTR modulators in special populations, part 1: Pregnancy and lactation

Use of CFTR modulators in special populations, part 1: Pregnancy and lactation

Elexacaftor-Tezacaftor-Ivacaftor: A Life-Changing Triple Combination of CFTR Modulator Drugs for Cystic Fibrosis

Cystic fibrosis year in review 2022

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