Abstract: Four studies (199 participants) met the inclusion criteria and were predominantly conducted in children with cystic fibrosis. Only one study had a combined cohort of adult and paediatric participants. The description of study methods was inadequate to assess the risk of bias, particularly with regard to blinding of assessors and selective reporting. One study was conducted in an inpatient setting with follow up in the outpatient setting; while the remaining three studies were conducted in individuals with stable respiratory disease in the outpatient setting. All included studies used exercise training to promote participation in physical activity, with the duration of the intervention period ranging from 18 days to three years. No improvement in physical activity participation was reported with any intervention period less than or equal to six months. Improvements in physical activity participation were only seen where follow up occurred beyond 12 months. There was no significant impact on quality of life from any of the intervention strategies. Although participation in physical activity is generally regarded as beneficial for people with cystic fibrosis, there is a lack of evidence regarding strategies to promote the uptake and the continued participation in physical activity for this population. This review provides very limited evidence that activity counselling and exercise advice, undertaken over at least six months, to engage in a home exercise programme may result in improved physical activity participation in people with cystic fibrosis. Further research is needed to determine the effect of strategies such as health coaching or telemedicine applications, in promoting the uptake and adherence to regular participation in physical activity. In addition, establishing the ideal duration of any interventions that promote physical activity, including exercise training programmes, will be important in addressing issues relating to participation in physical activity for people with cystic fibrosis.

Plain-language summary: This review aimed to evaluate the strategies that encourage people with cystic fibrosis to participate in daily physical activity. There were four included studies with a total of 199 participants which investigated the effect of exercise training on participation in physical activity. These were mostly conducted in children. The study methods and results were not clearly reported, so it was difficult to tell if the results were influenced by the way in which participants were assessed, or the nature of the outcomes reported. The training programmes ranged from 18 days to three years. In two studies the exercise training programmes were supervised and in two studies they were unsupervised and home-based. Due to differences in the study design and the outcomes measured, we could not combine data from different studies. None of the studies reported any improvement in participation in physical activity when the exercise training lasted less than six months. There was very limited evidence that using a home-exercise programme, for at least six months after receiving activity counselling and exercise advice, improved participation in physical activity in people with cystic fibrosis. No training program showed significant effects on quality of life. It is unknown whether strategies such as health coaching or Internet-based advice may help promote regular participation in physical activity in people with cystic fibrosis.