Short-term effects of Lumacaftor/Ivacaftor (Orkambi™) on exertional symptoms, exercise performance, and ventilatory responses in adults with cystic fibrosis
Abstract Rationale Lumacaftor/ivacaftor (LUM/IVA) modestly improves lung function following 1 month of treatment but it is unknown if this translates into improvements in exercise endurance and exertional symptoms. Methods Adult CF participants completed a symptom-limited constant load cycling test...
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doaj-4b861c2f63ec4e6386752372ee3caecf2020-11-25T02:48:58ZengBMCRespiratory Research1465-993X2020-06-012111410.1186/s12931-020-01406-zShort-term effects of Lumacaftor/Ivacaftor (Orkambi™) on exertional symptoms, exercise performance, and ventilatory responses in adults with cystic fibrosisBradley S. Quon0Andrew H. Ramsook1Satvir S. Dhillon2Reid A. Mitchell3Kyle G. Boyle4Pearce G. Wilcox5Jordan A. Guenette6Clinician-Scientist, UBC Centre for Heart Lung Innovation, Providence Health Care Research Institute, University of British Columbia, St. Paul’s HospitalClinician-Scientist, UBC Centre for Heart Lung Innovation, Providence Health Care Research Institute, University of British Columbia, St. Paul’s HospitalClinician-Scientist, UBC Centre for Heart Lung Innovation, Providence Health Care Research Institute, University of British Columbia, St. Paul’s HospitalClinician-Scientist, UBC Centre for Heart Lung Innovation, Providence Health Care Research Institute, University of British Columbia, St. Paul’s HospitalClinician-Scientist, UBC Centre for Heart Lung Innovation, Providence Health Care Research Institute, University of British Columbia, St. Paul’s HospitalClinician-Scientist, UBC Centre for Heart Lung Innovation, Providence Health Care Research Institute, University of British Columbia, St. Paul’s HospitalClinician-Scientist, UBC Centre for Heart Lung Innovation, Providence Health Care Research Institute, University of British Columbia, St. Paul’s HospitalAbstract Rationale Lumacaftor/ivacaftor (LUM/IVA) modestly improves lung function following 1 month of treatment but it is unknown if this translates into improvements in exercise endurance and exertional symptoms. Methods Adult CF participants completed a symptom-limited constant load cycling test with simultaneous assessments of dyspnea and leg discomfort ratings pre- and 1 month post-initiation of LUM/IVA. Results Endurance time, exertional dyspnea and leg discomfort ratings at submaximal exercise did not change significantly. There was a significant inverse correlation between changes in leg discomfort and endurance time (r = − 0.88; p = 0.009) following 1-month of LUM/IVA. Conclusions Overall, 1-month of LUM/IVA did not increase endurance time or modify exertional dyspnea or leg discomfort ratings. However, individuals who experienced a reduction in leg discomfort following LUM/IVA had an improvement in endurance time. Future studies with a larger sample size are needed to verify these findings and to assess the long-term effects of LUM/IVA on exercise outcomes. Trial registration ClinicalTrials.gov Identifier: NCT02821130 . Registered July 1, 2016.http://link.springer.com/article/10.1186/s12931-020-01406-z |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Bradley S. Quon Andrew H. Ramsook Satvir S. Dhillon Reid A. Mitchell Kyle G. Boyle Pearce G. Wilcox Jordan A. Guenette |
spellingShingle |
Bradley S. Quon Andrew H. Ramsook Satvir S. Dhillon Reid A. Mitchell Kyle G. Boyle Pearce G. Wilcox Jordan A. Guenette Short-term effects of Lumacaftor/Ivacaftor (Orkambi™) on exertional symptoms, exercise performance, and ventilatory responses in adults with cystic fibrosis Respiratory Research |
author_facet |
Bradley S. Quon Andrew H. Ramsook Satvir S. Dhillon Reid A. Mitchell Kyle G. Boyle Pearce G. Wilcox Jordan A. Guenette |
author_sort |
Bradley S. Quon |
title |
Short-term effects of Lumacaftor/Ivacaftor (Orkambi™) on exertional symptoms, exercise performance, and ventilatory responses in adults with cystic fibrosis |
title_short |
Short-term effects of Lumacaftor/Ivacaftor (Orkambi™) on exertional symptoms, exercise performance, and ventilatory responses in adults with cystic fibrosis |
title_full |
Short-term effects of Lumacaftor/Ivacaftor (Orkambi™) on exertional symptoms, exercise performance, and ventilatory responses in adults with cystic fibrosis |
title_fullStr |
Short-term effects of Lumacaftor/Ivacaftor (Orkambi™) on exertional symptoms, exercise performance, and ventilatory responses in adults with cystic fibrosis |
title_full_unstemmed |
Short-term effects of Lumacaftor/Ivacaftor (Orkambi™) on exertional symptoms, exercise performance, and ventilatory responses in adults with cystic fibrosis |
title_sort |
short-term effects of lumacaftor/ivacaftor (orkambi™) on exertional symptoms, exercise performance, and ventilatory responses in adults with cystic fibrosis |
publisher |
BMC |
series |
Respiratory Research |
issn |
1465-993X |
publishDate |
2020-06-01 |
description |
Abstract Rationale Lumacaftor/ivacaftor (LUM/IVA) modestly improves lung function following 1 month of treatment but it is unknown if this translates into improvements in exercise endurance and exertional symptoms. Methods Adult CF participants completed a symptom-limited constant load cycling test with simultaneous assessments of dyspnea and leg discomfort ratings pre- and 1 month post-initiation of LUM/IVA. Results Endurance time, exertional dyspnea and leg discomfort ratings at submaximal exercise did not change significantly. There was a significant inverse correlation between changes in leg discomfort and endurance time (r = − 0.88; p = 0.009) following 1-month of LUM/IVA. Conclusions Overall, 1-month of LUM/IVA did not increase endurance time or modify exertional dyspnea or leg discomfort ratings. However, individuals who experienced a reduction in leg discomfort following LUM/IVA had an improvement in endurance time. Future studies with a larger sample size are needed to verify these findings and to assess the long-term effects of LUM/IVA on exercise outcomes. Trial registration ClinicalTrials.gov Identifier: NCT02821130 . Registered July 1, 2016. |
url |
http://link.springer.com/article/10.1186/s12931-020-01406-z |
work_keys_str_mv |
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