Effects of Elexacaftor/Tezacaftor/Ivacaftor on cough frequency, physical activity patterns, and sleep quality in adolescents and adults with cystic fibrosis
Year: 2026
Type: article
Study design: Clinical Trial
Abstract: BACKGROUND: Cystic fibrosis (CF) transmembrane conductance regulator modulators improve lung function, however, effects on cough frequency, physical activity, and sleep have not been assessed in clinical studies. METHODS: After a 12-week, phase 4 pilot feasibility study, we conducted a 13-week, phase 3b, open-label study in participants with CF aged ≥12 years previously naïve to elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) to assess the effects of ELX/TEZ/IVA on cough frequency, physical activity, and sleep using wearable cough monitors and actigraphy sensors (VX-445-126). RESULTS: In study VX-445-126 (N = 81), participants treated with ELX/TEZ/IVA experienced a 91.7% reduction in cough frequency (cough events per day) (95% CI, 89.2% to 93.6%; 241.3 coughs per day at baseline to 20.3 coughs per day) from baseline at the average of week 8 through week 12 (primary endpoint). Total steps per day (secondary endpoint) increased by 638 (95% CI, 298 to 977) at the average of week 8 through week 12. Time spent above sedentary physical activity per day, time spent on moderate-to-vigorous physical activity per day, and total mean activity count per day all increased from baseline at the average of week 8 through week 12 (other efficacy endpoints). While no changes in sleep activity patterns were observed in actigraphy data, patient-reported measures of sleep quality improved (other efficacy endpoints). ELX/TEZ/IVA was generally safe and well-tolerated. CONCLUSIONS: ELX/TEZ/IVA treatment led to a > 90% reduction in daily cough frequency, with sustained improvements in physical activity and better perceptions of sleep quality.
Source: Journal of Cystic Fibrosis
Authors: Eva Van Braeckel, Andrew T. Braun, Nicholas J. Simmonds, Peter J. Barry, Jane C. Davies, Marie E. Egan, Allen Lapey, Marcus A. Mall, Edward F. McKone, Daniel Peckham, Marijke Proesmans, Bonnie Ramsey, Saioa Vicente Santamaria, Daniel Smith, Jennifer L. Taylor-Cousar, Elizabeth Tullis, Elke De Wachter, Nick Withers, Neil Ahluwalia, Sarah Conner, Mark Jennings, Yiyue Lou, Tanya G. Weinstock, Dominic Keating
Institutions: Ghent University Hospital, Ghent University, University of Wisconsin–Madison, Guy's and St Thomas' NHS Foundation Trust, Royal Brompton Hospital, Imperial College London, University of Manchester, Manchester University NHS Foundation Trust, Yale University, Massachusetts General Hospital, German Center for Lung Research, Charité – Universitätsmedizin Berlin, St. Vincent's University Hospital, St James's University Hospital, Universitair Ziekenhuis Leuven, Seattle Children's Hospital, Hospital Universitario Ramón y Cajal, Prince Charles Hospital, National Jewish Health, St. Michael's Hospital, Vrije Universiteit Brussel, Universitair Ziekenhuis Brussel, Royal Devon University Healthcare NHS Foundation Trust, Vertex Pharmaceuticals (United States), Alfred Health, Monash University
Language: English
Subfield: Pulmonary and Respiratory Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: hybrid
Estimated APC paid: $4,370
OpenAlex ID: W7147486356