Evidence map›Paper›PMID 41158496›Full record

ArticleERJ open research2025

Exploring reduced pulmonary exacerbation rates in cystic fibrosis: a pandemic or modulator effect?

Anne L Stephenson, Jenna Sykes, Sanja Stanojevic, Xiayi Ma, Josh Ostrenga, Jonathan Todd, Al Faro, Alex Elbert, Bruce Marshall, Kathleen J Ramos and 4 more

Abstract read
In one paragraph

Article in ERJ open research, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 3 papers.

0numbers the graph read from it
0cells of the map it votes in
3citing 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

3 citing papers in PubMed.

  1. Article
  2. Article
  3. Article
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

14 authors.

Anne L StephensonDivision of Respirology, St Michael's Hospital, University of Toronto, ON, Canada.
Jenna SykesDivision of Respirology, St Michael's Hospital, University of Toronto, ON, Canada.
Sanja StanojevicDepartment of Community Health and Epidemiology, Dalhousie University, Halifax, NS, Canada.ORCID https://orcid.org/0000-0001-7931-8051
Xiayi MaDivision of Respirology, St Michael's Hospital, University of Toronto, ON, Canada.
Josh OstrengaCystic Fibrosis Foundation, Bethesda, MD, USA.
Jonathan ToddCystic Fibrosis Foundation, Bethesda, MD, USA.
Al FaroCystic Fibrosis Foundation, Bethesda, MD, USA.
Alex ElbertCystic Fibrosis Foundation, Bethesda, MD, USA.
Bruce MarshallCystic Fibrosis Foundation, Bethesda, MD, USA.
Kathleen J RamosDivision of Pulmonary, Critical Care and Sleep Medicine, Department of Medicine, University of Washington, Seattle, WA, USA.
Elizabeth CromwellCystic Fibrosis Foundation, Bethesda, MD, USA.
Bradley S QuonCentre for Heart Lung Innovation, University of British Columbia, Vancouver, BC, Canada.
Christopher H GossDivision of Pulmonary, Critical Care and Sleep Medicine, Department of Medicine, University of Washington, Seattle, WA, USA.
Ranjani SomayajiDept. of Medicine, University of Calgary, Calgary, AB, Canada.

Funding

Translational Research Center to Expedite Novel Therapies in Cystic FibrosisP30DK089507 · NIDDK · SEATTLE CHILDREN'S HOSPITAL · PI Christopher Hooper Goss · 2010 to 2026
$21.4M
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
FDA HHS R01 FD006848NIDDK NIH HHS P30 DK089507NINR NIH HHS R01 NR020470
6 · The paper itself

Abstract

Background: The impact of elexacaftor-tezacaftor-ivacaftor (ETI) on pulmonary exacerbations (PEx) in cystic fibrosis (CF) is difficult to estimate since ETI availability coincided with the COVID-19 pandemic. Later access to ETI in Canada created a natural experiment to adjust for the impact of the pandemic on PEx rates. The objective for this study was to compare PEx rates pre- and post-ETI in the USA using Canada as a control group. Methods: This retrospective cohort study utilised data from the US and Canadian CF patient registries from 1 January 2015 to 31 December 2022. Individuals with at least one F508del mutation and ≥12 years old were followed. Poisson regression was used to estimate PEx rates per 100 person-years of follow-up. A difference-in-difference approach was used to estimate the impact of ETI alone. Results: Longitudinal data from 22 590 US individuals and 3271 Canadians were analysed. Pre-pandemic, the PEx rate was significantly higher in the USA (70.6, 95% CI 70.1-71.2) compared to Canada (53.1, 95% CI 51.9-54.3). During the COVID-19 pandemic, the PEx rate decreased to 30.2 (95% CI 28.8-31.8) in Canada; whereas in the USA, the rates decreased to 14.7 (95% CI 14.3-15.1). After adjusting for covariates, the ETI effect was 2.48 times (95% percentile interval 2.26-2.70) that of the pandemic effect. Conclusions: Public health measures imposed during the pandemic resulted in a reduction in the number of PEx for both countries; however, the impact of ETI on reducing the rate of PEx was more than double the effect of the COVID-19 pandemic.

Identifiers

PMID41158496
PMCPMC12557442

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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.