Evidence map›Paper›PMID 40603159›Full record

ArticleJournal of cystic fibrosis : official journal of the European Cystic Fibrosis Society2025

Elexacaftor/tezacaftor/ivacaftor prescription in lung transplant recipients with cystic fibrosis in the US.

Nora C Burdis, Miranda C Bradford, Sonya L Heltshe, Tijana Milinic, Oliver J McElvaney, Erika D Lease, Christopher H Goss, Siddhartha G Kapnadak, Brandon L Guthrie, Kathleen J Ramos

Abstract read
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In one paragraph

Article in Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

0numbers the graph read from it
0cells of the map it votes in
0citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

0 citing papers in PubMed.

No citing paper in PubMed yet.

4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

10 authors.

Nora C BurdisDivision of Pulmonary, Critical Care, and Sleep Medicine, Department of Medicine, University of Washington, Seattle WA, United States.
Miranda C BradfordSeattle Children's Research Institute, Biostatics Epidemiology and Analytics in Research (BEAR) Core, Seattle, WA, United States.
Sonya L HeltsheDivision of Pulmonary and Allergy, Department of Pediatrics, University of Washington, Seattle WA, United States; CFF TDNCC Seattle Children's Research Institute, Seattle, WA, United States.
Tijana MilinicDivision of Pulmonary, Critical Care, and Sleep Medicine, Department of Medicine, University of Washington, Seattle WA, United States.
Oliver J McElvaneyDivision of Pulmonary, Critical Care, and Sleep Medicine, Department of Medicine, University of Washington, Seattle WA, United States.
Erika D LeaseDivision of Pulmonary, Critical Care, and Sleep Medicine, Department of Medicine, University of Washington, Seattle WA, United States.
Christopher H GossDivision of Pulmonary, Critical Care, and Sleep Medicine, Department of Medicine, University of Washington, Seattle WA, United States; Division of Pulmonary and Allergy, Department of Pediatrics, University of Washington, Seattle WA, United States; CFF TDNCC Seattle Children's Research Institute, Seattle, WA, United States.
Siddhartha G KapnadakDivision of Pulmonary, Critical Care, and Sleep Medicine, Department of Medicine, University of Washington, Seattle WA, United States.
Brandon L GuthrieSchool of Public Health, Department of Global Health, University of Washington, Seattle, WA, United States.
Kathleen J RamosDivision of Pulmonary, Critical Care, and Sleep Medicine, Department of Medicine, University of Washington, Seattle WA, United States; CFF TDNCC Seattle Children's Research Institute, Seattle, WA, United States. Electronic address: ramoskj@uw.edu.

Funding

Translational Research Center to Expedite Novel Therapies in Cystic FibrosisP30DK089507 · NIDDK · SEATTLE CHILDREN'S HOSPITAL · PI Christopher Hooper Goss, Lucas R Hoffman · 2010 to 2026
$21.4M
Palliative Care Research Training GrantT32HL125195 · NHLBI · UNIVERSITY OF WASHINGTON · PI KROSS, ERIN KATHRYN, SHARMA, RASHMI K. · 2015 to 2024
$3.2M
Preparation for lung transplant discussions and decisions among people with cystic fibrosisR01NR020470 · NINR · UNIVERSITY OF WASHINGTON · PI Kathleen Jessica Ramos · 2023 to 2026
$2.9M
Pre-transplant health coaching to improve patient-reported outcomes in lung transplant candidatesR33HL158811 · NHLBI · MAYO CLINIC ROCHESTER · PI Cassie Colleen Kennedy · 2023 to 2026
$2.1M
NHLBI NIH HHS R33 HL158811NHLBI NIH HHS T32 HL125195NIDDK NIH HHS P30 DK089507NINR NIH HHS R01 NR020470
6 · The paper itself

Abstract

backgroundElexacaftor/tezacaftor/ivacaftor (ETI) has dramatically improved pulmonary and extrapulmonary manifestations of cystic fibrosis (CF). ETI clinical trials excluded lung transplant (LTx) recipients and current post-transplant prescribing practices are not evidence-based. We sought to identify the prevalence of new ETI prescriptions among CF LTx recipients in the United States (U.S.) and factors associated with ETI prescription after LTx.

methodsWe performed a retrospective cross-sectional study of LTx recipients through December 2022 using the Cystic Fibrosis Foundation Patient Registry. Recipients were alive as of October 2019, had an ETI-eligible genotype, and were not prescribed ETI before LTx. Logistic regression was used to assess factors associated with ETI prescription. CF Center prescribing patterns were categorized based on the proportion of LTx recipients who were prescribed ETI after LTx at each center.

resultsOverall, 488/1666 (29.3 %) of patients were prescribed ETI after LTx. The presence of sinus disease (OR 2.12, 95 % CI 1.51-2.99) and BMI<18.5 kg/m

conclusionsETI was newly prescribed to almost 30 % of eligible LTx recipients in the U.S. Even when including patient clinical characteristics in the model, CF center prescribing pattern was one of the strongest factors associated with ETI prescription after LTx.

Indexed as

AminophenolsBenzodioxolesChloride Channel AgonistsCystic FibrosisDrug PrescriptionsIndolesLung TransplantationPractice Patterns, Physicians'PyrazolesPyridinesPyrrolidinesQuinolonesAdultCross-Sectional StudiesDrug CombinationsFemaleAminophenolsBenzodioxolesChloride Channel AgonistsDrug CombinationselexacaftorIndolesivacaftorPyrazolesPyridinesPyrrolidinesQuinolonesCFTR modulatorCystic fibrosisElexacaftorIvacaftorLung transplantationTezacaftor

Identifiers

PMID40603159
PMCPMC12282660

What OpenQuestion holds

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.