ArticlePediatric pulmonology2026
Management Strategies for Hepatotoxicity Associated With Elexacaftor/Tezacaftor/Ivacaftor: Results of a Provider Survey.
Article in Pediatric pulmonology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
backgroundElexacaftor/tezacaftor/ivacaftor (ETI) has significantly improved lung function and the clinical course of people with cystic fibrosis (PwCF) but also can cause elevations in liver function tests (LFTs). While the prescribing information (PI) provides some recommendations for holding ETI or hepatically adjusting doses based on certain criteria, clinicians are often faced with clinical scenarios that do not meet those criteria (e.g. PwCF with CF liver disease [CFLD, now termed CF hepatobiliary involvement (CFHBI)] that do not meet Child-Pugh Class B or C criteria). The purpose of this survey was to describe clinical practices regarding LFT monitoring and ETI dose adjustments in relation to hepatotoxicity and LFT abnormalities in PwCF with and without baseline CFLD or elevations in LFTs across adult and pediatric CF Foundation accredited care centers and affiliate programs (CFF-ACCAP) in the United States.
resultsThere were 53 unique respondents with representation from physicians, pharmacists, and advanced practitioners. When managing ETI in response to transaminase elevations that didn't meet PI recommendations for holding ETI in PwCF without CFLD or baseline LFT elevations, 51% of respondents would not change ETI therapy, and 41% would repeat LFTs, usually within a 2-4 week time frame. For those who met PI recommendations for pausing ETI, 49% of respondents would repeat LFTs in 1 month. In PwCF with baseline CFLD that doesn't meet Child-Pugh Class B or C criteria or baseline LFT elevations, 74% of respondents initiated ETI at full dose, and 79% of respondents checked LFTs earlier than the PI recommendations.
conclusionDespite ETI's remarkable impact on pulmonary and extrapulmonary CF manifestations, the CF care team faces clinical challenges related to hepatotoxicity as well as appropriate monitoring and dosing of ETI in clinically gray scenarios. The results of this survey demonstrate the need for a multidisciplinary guideline to navigate these complex situations.
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