Exercise intolerance in cystic fibrosis-the role of CFTR modulator therapies.

Jessica E Caterini, Felix Ratjen, Alan R Barker, Craig A Williams, Kate Rendall, Jane E Schneiderman, Greg D Wells

Journal: Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2022;21(2):282-292

PMID: 34955387

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Abstract

Exercise intolerance is common in people with CF (pwCF), but not universal among all individuals. While associated with disease prognosis, exercise intolerance is not simply a reflection of the degree of lung disease. In people with severe CF, respiratory limitations may contribute more significantly to impaired exercise capacity than in those with mild-moderate CF. At all levels of disease severity, there are peripheral factors e.g., abnormal macro- and micro-vascular function that impair blood flow and reduce oxygen extraction, and mitochondrial defects that diminish metabolic efficiency. We discuss advances in understanding the central and peripheral mechanisms underlying exercise intolerance in pwCF. Exploring both the central and peripheral factors that contribute to exercise intolerance in CF can help inform the development of new therapeutic targets, as well as help define prognostic criteria.

Address: Translational Medicine Program, SickKids Research Institute, Toronto, ON M5G 0A4, Canada; Queen's Medical School, Kingston, ON K7L 3N6, Canada.; Translational Medicine Program, SickKids Research Institute, Toronto, ON M5G 0A4, Canada; Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, ON M5G 1X8, Canada; Division of Respiratory Medicine, The Hospital for Sick Children, University of Toronto, Toronto, ON M5G 1X8, Canada.; Children's Health and Exercise Research Centre, Sport and Health Sciences, University of Exeter, Exeter EX1 2LU, UK.; Translational Medicine Program, SickKids Research Institute, Toronto, ON M5G 0A4, Canada.; Division of Respiratory Medicine, The Hospital for Sick Children, University of Toronto, Toronto, ON M5G 1X8, Canada; Faculty of Kinesiology & Physical Education, University of Toronto, Toronto, ON M5S 1A1, Canada.; Translational Medicine Program, SickKids Research Institute, Toronto, ON M5G 0A4, Canada. Electronic address: [email protected].

DOI: 10.1016/j.jcf.2021.11.011

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MeSH Terms: Cystic Fibrosis, Cystic Fibrosis Transmembrane Conductance Regulator, Exercise, Humans