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The use of elexacaftor/tezacaftor/ivacaftor in patients with cystic fibrosis post-liver transplant: a case series
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  • Hunter Ragan,
  • Elizabeth Autry,
  • Taryn Bomersback,
  • Jennifer Hewlett,
  • Lauren Kormelink,
  • Julie Safirstein,
  • Laura Shanley,
  • Lisa Lubsch
Hunter Ragan
Goldfarb School of Nursing at Barnes-Jewish College

Corresponding Author:hunterragan50@gmail.com

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Elizabeth Autry
University of Kentucky Medical Center
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Taryn Bomersback
Alberta Health Services
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Jennifer Hewlett
Children’s Hospital of Philadelphia
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Lauren Kormelink
University of Kentucky Health Care Pharmacy Services
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Julie Safirstein
Hospital of the University of Pennsylvania
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Laura Shanley
Children’s Hospital of Philadelphia
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Lisa Lubsch
Southern Illinois University Edwardsville - School of Pharmacy
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Introduction Cystic fibrosis (CF) related liver disease (CFLD) manifests as a wide spectrum of hepatobiliary disease and can progress to need liver transplantation. Elexacaftor/tezacaftor/ivacaftor (elx/tez/iva) is a cystic fibrosis transmembrane conductance regulator (CFTR) modulator which has superior efficacy compared to previously approved modulators. Use of elx/tez/iva, should be approached with caution in individuals with CFLD or following liver transplantation due to possible increases in LFTs and drug-drug interactions with several immunosuppressant medications. Objective The purpose of this case series was to explore if the use of elx/tez/iva is safe and tolerable in patients with CF post-liver transplantation. Methods A retrospective case series including patients prescribed elx/tez/iva following liver transplantation and an immunosuppressive regimen consisting of drug therapy metabolized by P-glycoprotein was completed. Results Ten patients at six CF centers with a median age of 22.1 years (range 14-43.4 years) and median time from transplant of 6.9 years (range 0.6-22 years) were included. Most patients (8, 80%) received a reduced or full dose of elx/tez/iva for a mean duration of 10.4 months (range 7-12 months). Fluctuations in LFTs occurred in all patients (10, 100%) and led to therapy discontinuation in two patients (20%). Elx/tez/iva initiation resulted in elevations in tacrolimus trough concentration in 7 patients (70%). Most patients who tolerated elx/tez/iva had symptomatic and quality of life improvement, increased body-mass-index, and maintained or improved lung function. Conclusion Initiation of elx/tez/iva in patients with CF who received a liver transplantation may be safe with clinical benefits.
10 Jun 2021Submitted to Pediatric Pulmonology
18 Jun 2021Submission Checks Completed
18 Jun 2021Assigned to Editor
21 Jun 2021Reviewer(s) Assigned
04 Jul 2021Review(s) Completed, Editorial Evaluation Pending
07 Jul 2021Editorial Decision: Revise Major
08 Oct 20211st Revision Received
09 Oct 2021Submission Checks Completed
09 Oct 2021Assigned to Editor
09 Oct 2021Reviewer(s) Assigned
17 Oct 2021Review(s) Completed, Editorial Evaluation Pending
17 Oct 2021Editorial Decision: Revise Minor
31 Oct 20212nd Revision Received
01 Nov 2021Submission Checks Completed
01 Nov 2021Assigned to Editor
01 Nov 2021Reviewer(s) Assigned
06 Nov 2021Review(s) Completed, Editorial Evaluation Pending
13 Nov 2021Editorial Decision: Accept
30 Nov 2021Published in Pediatric Pulmonology. 10.1002/ppul.25779