Change in exercise capacity measured by Cardio-pulmonary Exercise Testing (CPET) in Danish people with cystic fibrosis after initiation of treatment with Lumacaftor/Ivacaftor and Tezacaftor/Ivacaftor |
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| Institution: | 1. Department of Physiotherapy and Occupational Therapy, Aarhus University Hospital, Aarhus, Denmark;2. Cystic Fibrosis Centre, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark;3. Department of Paediatrics and Adolescent Medicine, Aarhus University Hospital, Aarhus, Denmark;1. Adult Cystic Fibrosis Centre, The Prince Charles Hospital, Brisbane, Australia;2. Faculty of Medicine, The University of Queensland, Brisbane, Australia;3. Translational Research Institute, Brisbane, Australia;1. Division of Paediatric Medicine, Department Paediatrics, Hospital for Sick Children, University of Toronto, Toronto, Ontario M5G 1X8, Canada;2. Division of Respiratory Medicine, Department of Paediatrics, Hospital for Sick Children, University of Toronto, Toronto, Canada;3. Division of Infectious Diseases, Department of Paediatrics, Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada;4. Translational Medicine Program, Hospital for Sick Children, University of Toronto, Toronto, Canada;5. Division of Respirology, Department of Medicine St Michael''s Hospital, University of Toronto, Toronto, Canada;1. Asthma and Airway Disease Research Center, University of Arizona, Tucson, AZ, United States;2. Department of Biostatistics, University of Washington, Seattle, WA, United States;3. Division of Medical Genetics, Department of Medicine, University of Washington, Seattle, WA, United States;4. Brotman-Baty Institute for Precision Medicine, Seattle, WA, United States;5. Division of Genetic Medicine, Department of Pediatrics, University of Washington, Seattle, WA, United States;6. Department of Pediatrics, University of Washington School of Medicine, Seattle, WA, United States;7. Department of Pediatrics, Children''s Hospital Colorado and University of Colorado Anschutz Medical Campus, Aurora, CO, United States;8. Department of Genome Sciences, University of Washington, Seattle, WA, United States;9. Department of Pediatrics, University of Arizona, Tucson, AZ, United States;10. Department of Medicine, University of Arizona, Tucson, AZ, United States;1. Stead Family Department of Pediatrics, University of Iowa Carver College of Medicine, Iowa City, IA 52242, United States;2. Department of Pathology, University of Iowa Carver College of Medicine, Iowa City, IA 52242, United States;3. State Hygienic Laboratory at the University of Iowa, Coralville, IA, United States;1. Department of Pediatrics, University of Michigan Medical School, 1150 West Medical Center Drive, Ann Arbor, MI 48109, United States;2. Department of Pediatrics, Case Western Reserve University School of Medicine, Cleveland, OH, United States;3. Department of Biostatistics, University of Michigan School of Public Health, Ann Arbor, MI, United States;4. Department of Internal Medicine, University of Michigan Medical School, Ann Arbor, MI, United States;1. Pneumologisches Studienzentrum München-West, München, Germany;2. Division of Cystic Fibrosis, CF Center Westbrandenburg, Campus Potsdam, Potsdam, Germany;3. Cardiff University, School of Biosciences, Cardiff, Wales, UK;4. SMERUD, Karenslyst alle 6, 0278 Oslo, Norway;5. AlgiPharma AS, Industriveien 33, 1337 Sandvika, Norway |
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| Abstract: | BackgroundSince 2015, when the first cystic fibrosis transmembrane conductance regulator (CFTR) modulators were approved for people with cystic fibrosis (CF) homozygous for F508del-CFTR, studies have shown improved lung function after initiation of the treatment and patients experience improved physical capacity. The aim of this study was to investigate change in exercise capacity after initiation of Lumacaftor/Ivacaftor and Tezacaftor/Ivacaftor treatment (LUM/IVA, TEZ/IVA).MethodsWe performed a single group prospective observational cohort study with follow-up at six and 12 months. The study examined change in exercise capacity in people with CF initiating treatment with LUM/IVA and TEZ/IVA, measured by cardio-pulmonary exercise testing (CPET). Inclusion criteria were people with CF homozygous for F508del-CFTR aged 12 years or older eligible for LUM/IVA and TEZ/IVA treatment from June 2017 until June 2019. Primary outcomes were change in VO2peak and maximal workload. Secondary outcomes were change in muscle strength, muscle power and body composition in a subgroup of the study population.ResultsA total of 91 patients were included in the analysis. The mean change in VO2peak and VO2peak divided by body weight from baseline to 12-months follow-up was 145.7 (91.2;200.2) ml/min and 1.07 (95% CI 0.19;1.95) ml/min/kg, respectively. The mean change in maximal workload between baseline and 12 months was 14.2 Watt (95% CI 9.1;19.2). All improvements in exercise capacity were statistically significant.ConclusionsPatients in this study improved their exercise capacity by a statistically significant increase in VO2peak and maximal workload 12 months after initiation of treatment with LUM/IVA and TEZ/IVA. |
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