Baseline Characteristics and Self-Reported Use of Chronic Daily Therapies of the Home Reported Outcomes With CFTR Modulator Therapy (HERO-2) Cohort.

Georgene E Hergenroeder¹, Kevin J Psoter², Cynthia D Brown³, Josh Ostrenga⁴, Christine Ford⁵, Lisa Bendy⁶, Bradley H Rosen³, Nell Meosky Luo⁷, Connie Zhang⁷, Kathryn A Sabadosa⁴, Clement L Ren¹

¹Division of Pulmonary and Sleep Medicine, Department of Pediatrics, Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.

²Division of General Pediatrics, Department of Pediatrics, Johns Hopkins School of Medicine, Baltimore, Maryland, USA.

³Division of Pulmonary, Critical Care, Sleep, and Occupational Medicine, Department of Medicine, Indiana University School of Medicine, Indianapolis, Indiana, USA.

⁴Cystic Fibrosis Foundation, Bethesda, Maryland, USA.

⁵Division of Pulmonary and Critical Care Medicine, Department of Medicine, Johns Hopkins School of Medicine, Baltimore, Maryland, USA.

⁶Division of Pulmonary, Allergy, and Sleep Medicine, Department of Pediatrics, Riley Hospital for Children, Indiana University School of Medicine, Indianapolis, Indiana, USA.

⁷Folia Health Inc, Boston, Massachusetts, USA.

PubMed DOI

Abstract

BACKGROUND: The HERO-2 study is a prospective, observational study investigating chronic daily therapy (CDT) changes among people with cystic fibrosis (PwCF) ≥ 12 years of age taking elexacaftor/tezacaftor/ivacaftor (ETI). The goal of this analysis was to describe baseline characteristics and patterns of CDT discontinuation reported at study enrollment and identify clinical features associated wit…

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Mentioned Companies

Folia Health
affiliation

Other Funding

This research was supported by the Cystic Fibrosis Foundation, The Cystic Fibrosis Foundation (BROWN21AB0). Initial results of this study were presented in abstract form at the 2023 North American Cystic Fibrosis Conference in Phoenix, Arizona.

MeSH Terms

Humans0Cystic Fibrosis*Male0Female0Prospective Studies0Aminophenols*Child0Adolescent0Benzodioxoles*Quinolones*Self Report0Indoles*Drug Combinations0Young Adult0Adult0Cystic Fibrosis Transmembrane Conductance Regulator0Pyrrolidines0Pyrazoles*Pyridines0Patient Reported Outcome Measures0Chloride Channel Agonists*

Keywords

burden of carechronic daily therapiescystic fibrosis transmembrane conductance regulatory (CFTR) modulator therapyelexacaftor/tezacaftor/ivacaftortreatment de‐escalation

Article Info

PMID: 40662498
DOI: 10.1002/ppul.71190
Received: January 16, 2025
Accepted: June 28, 2025

How to Cite

Hergenroeder GE et al. Baseline Characteristics and Self-Reported Use of Chronic Daily Therapies of the Home Reported Outcomes With CFTR Modulator Therapy (HERO-2) Cohort. Pediatr Pulmonol2025;60(7):e71190. doi:10.1002/ppul.71190

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