Impact of Elexacaftor/Tezacaftor/Ivacaftor on Cystic Fibrosis Liver Tests in a Real-World Cohort with and Without Baseline CFLD

Bowen, Megan E.; Sample, E.; Collins, Ashley; Singhal, Anju; Brown, Alison

doi:10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2028

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“…Physicians described 7 cases of adults who developed biliary colic after treatment with ETI, including 6 who required cholecystectomy, an outcome also observed in one pregnant woman who used ETI during pregnancy [48 ▪▪ ,49]. Although ETI has been successfully used in pwCF with known baseline CF-related liver disease (based on ultrasound and/or biopsy), a case report of an adult with previously undetected preexisting liver disease developed acute liver failure requiring liver transplant following the reintroduction of ETI after initial discontinuation for elevated liver function tests [50,51]. Anecdotally, increased anxiety has also been reported with ETI use.…”

Section: Challenges and Care Management In The Elexacaftor/tezacaftor...mentioning

confidence: 95%

Triple combination cystic fibrosis transmembrane conductance regulator modulator therapy in the real world – opportunities and challenges

Barry

Taylor‐Cousar

2021

Current Opinion in Pulmonary Medicine

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Purpose of reviewThe purpose of this review is to describe on-going and upcoming real-world studies that will aid the cystic fibrosis (CF) community in understanding the long-term efficacy, safety and challenges in utilizing this therapy and managing care.Recent findingsThe triple combination of elexacaftor, tezacaftor and ivacaftor (ETI) has been demonstrated to improve lung function, weight and quality of life in children and adults with CF with at least one copy of Phe508del. Treatment with ETI will potentially be available for approximately 90% of the CF population and change the face of CF.SummaryIn spite of early demonstrations of short-term efficacy and safety, for medications that may be given for much of someone's life, continued assessment of these outcomes is necessary. Furthermore, the CF community must evaluate and address the issues that arise with increased longevity including parenthood, preventive care management and the potential comorbidities of aging.

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Section: Challenges and Care Management In The Elexacaftor/tezacaftor...mentioning

confidence: 95%

Triple combination cystic fibrosis transmembrane conductance regulator modulator therapy in the real world – opportunities and challenges

Barry

Taylor‐Cousar

2021

Current Opinion in Pulmonary Medicine

View full text Add to dashboard Cite

show abstract

Impact of Elexacaftor/Tezacaftor/Ivacaftor on Cystic Fibrosis Liver Tests in a Real-World Cohort with and Without Baseline CFLD

Cited by 1 publication

References 0 publications

Triple combination cystic fibrosis transmembrane conductance regulator modulator therapy in the real world – opportunities and challenges

Triple combination cystic fibrosis transmembrane conductance regulator modulator therapy in the real world – opportunities and challenges

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