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. 2022;27(5):467-469.
doi: 10.5863/1551-6776-27.5.467. Epub 2022 Jul 6.

If At First You Don't Succeed, Trikafta Again

India Loyd  1 , Nicole Papac  2 , Jason Hirshburg  2 , Jarad Levin  2 , Jennifer Dannelley  3 , Janislynn Dorris  3 , Jason Stratton  1   2   3 , Nighat Mehdi  3
Affiliations

If At First You Don't Succeed, Trikafta Again

India Loyd et al. J Pediatr Pharmacol Ther. 2022.

Abstract

Adverse reactions, including severe cutaneous reactions, to cystic fibrosis transmembrane conductance regulator (CFTR) modulators have been described in the literature. Herein we present a drug eruption in response to elexacaftor/tezcaftor/ivacaftor (brand name, Trikafta) in a 7-year-old male with cystic fibrosis, followed by desensitization and successful continuation. A review of the literature outlining similar cases is provided. Attempting to mitigate and manage drug reactions to CFTR modulators is essential because they represent vital and irreplaceable therapies for individuals with cystic fibrosis (CF).

Keywords: CFTR modulator; cystic fibrosis; desensitizat urticaria; elexacaftor; ivacaftor; tezacaftor.

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Conflict of interest statement

Disclosures. The authors declare no conflicts or financial interest in any product or service mentioned in the manuscript. The authors had full access to all patient information in this report and take responsibility for the integrity and accuracy of the report.

Figures

Image.
Image.
Urticarial drug rash in young male.
See this image and copyright information in PMC

References

    1. Elborn JS. Cystic fibrosis. Lancet . 2016;388(10059):2519–2531. - PubMed
    1. Zaher A, ElSaygh J, Elsori D et al. A review of Trikafta: triple cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy. Cureus . 2021;13(7):e16144. - PMC - PubMed
    1. Ridley K, Condren M. Elexacaftor-tezacaftor-ivacaftor: the first triple-combination cystic fibrosis transmembrane conductance regulator modulating therapy. J Pediatr Pharmacol Ther . 2020;25(3):192–197. - PMC - PubMed
    1. Griese M, Costa S, Linnemann RW et al. Safety and efficacy of elexacaftor/tezacaftor/ivacaftor for 24 weeks or longer in people with cystic fibrosis and one or more F508del alleles: interim results of an open-label phase 3 clinical trial. Am J Respir Crit Care Med . 2021;203(3):381–385. - PMC - PubMed
    1. Zemanick ET, Taylor-Cousar JL, Davies J et al. A phase 3 open-label study of elexacaftor/tezacaftor/ivacaftor in children 6 through 11 years of age with cystic fibrosis and at least one F508del allele. Am J Respir Crit Care Med . 2021;203(12):1522–1532. - PMC - PubMed

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