Physical activity and exercise have become an accepted and valued component of Cystic Fibrosis care. Regular physical activity and exercise can slow the rate of decline of pulmonary function, improve physical fitness, and enhance quality of life. However, motivating people to be more active is challenging. Supervised exercise programs are expensive and labor intensive, and adherence falls off significantly once supervision ends. Unsupervised or partially supervised programs are less costly and more flexible, but compliance can be more problematic. The primary objective of this study is to evaluate the effects of a 12-months partially supervised exercise intervention along with regular motivation on forced expiratory volume in 1 second (FEV1) in a large international group of cystic fibrosis patients. Secondary endpoints include patient reported quality of life, as well as levels of anxiety and depression, and control of blood sugar. A total of 292 patients with cystic fibrosis 12 years and older with a FEV1 ≥35% predicted will be recruited. Following baseline assessments (2 visits) patients will be randomized into an intervention and a control group. Thereafter, they will be seen every 3 months for assessments in their centre for one year (4 follow-up visits). Along with individual counseling to increase vigorous physical activity by at least 3 hours per week on each clinic visit, the intervention group will document daily exercise and inactivity time and will receive a step counter and they will record their progress with a web-based program. They will also receive monthly phone calls from the study staff. After 6 months, they will continue with the step counter and web-based program for a further 6 months. The control group will receive access to this intervention after 12 months of standardized care. Should this relatively simple program prove successful, this will be made available on a wider scale internationally.
Name: Exercise Intervention
Description: from diary
Measure: Number of upper respiratory tract infections Time: baseline to 6 months and baseline to 12 monthsDescription: from questionnaire
Measure: Days on additional oral / intravenous antibiotics Time: baseline to 6 months and baseline to 12 monthsDescription: estimated from skinfold thickness
Measure: Change in muscle mass (kg) Time: baseline to 6 months and baseline to 12 monthsDescription: estimated from skinfold thickness
Measure: Change in percent body fat Time: baseline to 6 months and baseline to 12 monthsDescription: from the revised Cystic Fibrosis health-related quality of life Questionnaire (CFQ-R questionnaire)
Measure: Change in Quality of Life scales Time: baseline to 6 months and baseline to 12 monthsDescription: from Depression Anxiety Stress Scales
Measure: Change in depression, anxiety and stress scores Time: baseline to 6 months and baseline to 12 monthsDescription: standardized oral glucose tolerance test only patients without diabetes mellitus
Measure: Change in plasma glucose concentrations 1 and 2 hours after a standardized glucose load Time: baseline to 9 monthsDescription: causality as judged by investigator
Measure: Adverse events possibly or likely related to exercise Time: baseline to 6 months and baseline to 12 monthsDescription: based on questionnaire and diary
Measure: Compliance with the exercise goal Time: baseline to 6 months and baseline to 12 monthsDescription: based on nitrogen multiple breath washout, in selected centres only
Measure: Change in lung clearance index Time: baseline to 6 months and baseline to 12 monthsDescription: based on accelerometry, in selected centres only
Measure: Change in time spent in moderate-and-vigorous physical activity Time: baseline to 6 months and baseline to 12 monthsDescription: based on dual energy x-ray absorptiometry, in selected centres only
Measure: Change in bone mineral density and body composition Time: baseline to 6 months and baseline to 12 monthsDescription: based on nuclear medicine scans, US centres only
Measure: Change in mucociliary clearance with exercise Time: baseline to 6 monthsAllocation: Randomized
Parallel Assignment
There is one SNP
- Unstable condition precluding exercise (major hemoptysis or pneumothorax within the last 3 months, acute exacerbation and iv-antibiotics during the last 4 weeks, planned surgery, listed for lung transplantation, major musculoskeletal injuries such as fractures or sprains during the last 2 months, others according to the impression of the doctor) - Cardiac arrhythmias with exercise - Requiring additional oxygen with exercise - Recent diagnosis of diabetes 3 months prior to screening or at screening - Recent changes in medication 1 month or less prior to screening (systemic steroids, ibuprofen, inhaled antibiotics, mannitol, DNAse, hypertonic saline) - At least one G551D mutation and not on ivacaftor (VX770) yet but planned start or planned stop of ivacaftor during the trial - Colonization with Burkholderia cenocepacia Inclusion Criteria: - Confirmed diagnosis of Cystic Fibrosis - Age ≥12 years - Forced expiratory volume in 1 second (FEV1) ≥ 35% predicted - Access to the internet Exclusion Criteria: - Participation in another clinical trial up to 4 weeks prior to the first baseline visit - Pregnancy/Breastfeeding - Inability to exercise - More than 4 hours of reported strenuous physical activities per week currently or up to 3 months prior to baseline measurements and not already planned within the coming 6 months. --- G551D ---
- Unstable condition precluding exercise (major hemoptysis or pneumothorax within the last 3 months, acute exacerbation and iv-antibiotics during the last 4 weeks, planned surgery, listed for lung transplantation, major musculoskeletal injuries such as fractures or sprains during the last 2 months, others according to the impression of the doctor) - Cardiac arrhythmias with exercise - Requiring additional oxygen with exercise - Recent diagnosis of diabetes 3 months prior to screening or at screening - Recent changes in medication 1 month or less prior to screening (systemic steroids, ibuprofen, inhaled antibiotics, mannitol, DNAse, hypertonic saline) - At least one G551D mutation and not on ivacaftor (VX770) yet but planned start or planned stop of ivacaftor during the trial - Colonization with Burkholderia cenocepacia Cystic Fibrosis Cystic Fibrosis null --- G551D ---