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Elexacaftor-Tezacaftor-Ivacaftor improves exercise capacity in adolescents with cystic fibrosis
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  • Adam J. Causer,
  • Jan Shute,
  • Michael Cummings,
  • Anthony Shepherd,
  • Samuel Wallbanks,
  • Richard Pulsford,
  • Victoria Bright,
  • Gary Connett,
  • Zoe Saynor
Adam J. Causer
University of Portsmouth

Corresponding Author:[email protected]

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Jan Shute
University of Portsmouth School of Pharmacy and Biomedical Sciences
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Michael Cummings
Portsmouth Hospitals University NHS Trust
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Anthony Shepherd
University of Portsmouth
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Samuel Wallbanks
University of Portsmouth
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Richard Pulsford
University of Exeter College of Life and Environmental Sciences
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Victoria Bright
University Hospital Southampton NHS Foundation Trust
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Gary Connett
University Hospital Southampton NHS Foundation Trust
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Zoe Saynor
University of Portsmouth
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Abstract

This study investigated whether Elexacaftor-Tezacaftor-Ivacaftor (Kaftrio ®), a cystic fibrosis (CF) transmembrane conductance regulator (CFTR) modulator, could improve exercise capacity in adolescents with CF. After six weeks treatment, Kaftrio ® improved both maximal and submaximal indices of aerobic fitness. Improvements were independent of changes in ventilatory function during exercise and physical activity. Interestingly, pulmonary oxygen uptake per unit of power output was higher in two, out of three, cases who presented with more severe CF lung disease. These findings suggest improved O 2 extraction and/or consumption in the exercising muscle and demonstrate, for the first time, that short-term treatment with Kaftrio ® might improve aerobic fitness in people with CF, especially in those with more severe lung disease and deconditioning.
13 Apr 2022Submitted to Pediatric Pulmonology
13 Apr 2022Submission Checks Completed
13 Apr 2022Assigned to Editor
18 Apr 2022Reviewer(s) Assigned
03 May 2022Review(s) Completed, Editorial Evaluation Pending
03 May 2022Editorial Decision: Revise Minor
12 May 20221st Revision Received
12 May 2022Reviewer(s) Assigned
12 May 2022Submission Checks Completed
12 May 2022Assigned to Editor
05 Jun 2022Review(s) Completed, Editorial Evaluation Pending
09 Jun 2022Editorial Decision: Revise Minor
16 Jun 20222nd Revision Received
21 Jun 2022Submission Checks Completed
21 Jun 2022Assigned to Editor
21 Jun 2022Reviewer(s) Assigned
25 Jun 2022Review(s) Completed, Editorial Evaluation Pending
25 Jun 2022Editorial Decision: Accept
Nov 2022Published in Pediatric Pulmonology volume 57 issue 11 on pages 2652-2658. 10.1002/ppul.26078