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. 2022;27(5):463-466.
doi: 10.5863/1551-6776-27.5.463. Epub 2022 Jul 6.

Rechallenge of Elexacaftor/Tezacaftor/Ivacaftor After Skin Rash in Two Pediatric Patients

Emily R Diseroad  1   2 , Peter J Mogayzel Jr  3 , Alice Pan  2
Affiliations

Rechallenge of Elexacaftor/Tezacaftor/Ivacaftor After Skin Rash in Two Pediatric Patients

Emily R Diseroad et al. J Pediatr Pharmacol Ther. 2022.

Abstract

Cystic fibrosis transmembrane conductance regulator (CFTR) modulators have revolutionized care for patients with cystic fibrosis (CF). The triple combination product elexacaftor/tezacaftor/ivacaftor is a highly effective CFTR modulator that is generally well tolerated. However, in clinical trials of pediatric and adult patients, 4% to 12% developed rash after initiation of therapy. Few reports have described approaches to management of this adverse effect. In this report, we describe 2 children with CF who developed a pruritic, maculopapular rash after initiating elexacaftor/tezacaftor/ivacaftor. These patients were successfully rechallenged after rash resolution with a practical titration schedule.

Keywords: CFTR modulator; cystic fibrosis; rash.

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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, including grants, equipment, medications, employment gifts, and honoraria. PJM receives grant support from the Cystic Fibrosis Foundation, Eloxx, and Vertex Pharmaceuticals. Authors had full access to all patient information in this report and take responsibility for the integrity and accuracy of the report.

Figures

Image 1.
Image 1.
(a) Left palm and (b) lower back of case 1 on first day of elexacaftor/tezacaftor/ivacaftor therapy.
Image 2.
Image 2.
(a) Left hand and (b) left foot of case 2 on eighth day of elexacaftor/tezacaftor/ivacaftor therapy.
See this image and copyright information in PMC

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