Background: Physical exercise is an important part of regular care for people with cystic fibrosis (CF). It is unknown whether such exercise has beneficial or detrimental effects on nutritional status (body composition). Thus, the objective of this review was to evaluate the effect of exercise on measures of nutritional status in children and adults with CF. Methods: Standardized reporting guidelines for systematic reviews were followed and the protocol was prospectively registered. Multiple databases were utilized (e.g., PubMed, Scopus, and CINHAL). Two reviewers independently reviewed titles/abstracts and then the full text for selected studies. Results: In total, 924 articles were originally identified; data were extracted from 4 eligible studies. These four studies included only children; pulmonary function ranged from severe to normal, and the majority of participants were at or below their recommended weight. Exercise training did not worsen nutritional status in any study; two studies that included resistance exercise reported an increase in fat-free mass. Three of the four studies also reported increased aerobic capacity and/or muscle strength. Conclusions: Exercise training can produce positive physiologic changes in children with CF without impairing their nutritional status. In fact, resistance exercise can help improve body mass. Much less is known about how exercise may affect adults or those who are overweight.

The aim of this study was to investigate the dose–response relationship between physical activity and health-related quality of life (HRQoL) in a large population-based sample of people with chronic disease. We analysed the data of 29,271 adults (15,315 women) who were diagnosed with chronic diseases and participated in the Welsh Health Survey (Wales, UK; data collection 2011–2015). Participants were classified, based on their weekly minutes of moderate-to-vigorous physical activity (MVPA), into four groups as follows: inactive (no MVPA), insufficiently active (<150 min/week), sufficiently active (≥150–<300), and very active (≥300). The main outcome was HRQoL measured via the Short-Form 36 Health Survey (SF-36). This study found a curvilinear association between MVPA and HRQoL and a dose–response relationship for the perception of general health and vitality domains. Compared to inactive participants, those who were very active had higher HRQoL scores (coefficient = 12.54; 95% confidence interval [CI] 11.39–13.70), followed by sufficiently active (coefficient = 11.70; 95% CI 10.91–12.49) and insufficiently active (coefficient = 9.83; 95% CI 9.15–10.51) participants. The fully adjusted regression model showed curvilinear associations between MVPA and the domains of SF-36. Future research should find ways to motivate people with chronic diseases to engage in physical activity. The evidence to support regular exercise in individuals with chronic diseases in all age groups is strong and compelling, and patients should be encouraged to regularly devote more time to physical activity in order to improve their health and well-being.

Background: The aims of this study were to evaluate the methodological quality of interventional exercise studies in individuals with cystic fibrosis (CF) and link the reported outcome measures of these studies to the International Classification of Functioning, Disability and Health (ICF) model. Methods: Electronic searches of PubMed, Scopus, Embase, CINAHL, SPORTDiscus, and Cochrane review were performed in the spring of, 2022. The methodological quality of studies was evaluated using the Effective Public Health Practice Project Quality Assessment Tool (EPHPP). Outcome measures were linked to the categories and sub-categories of the ICF model using ICF linking rules. Results: We screened 4,769 studies and extracted data from 89 eligible manuscripts. Of these. 41% were one group, pre-post design; 36% were RCTs; 11% were two-group pre-post design; 6% were controlled clinical trials; and 6% were either case reports or case series. Using the (EPHPP), 49% were ranked as high quality, 34% moderate, and 17% low. In addition, body function was the most addressed ICF category followed by activities/participation and, subsequently, body structure. Significance: This review supports the recommendation of exercise as an intervention to improve a variety of outcomes, ranging from Body structure (e.g., body composition) to Participation (e.g., engaging in community, social, and civic life). We recommend that investigators review the spectrum of potential ICF outcomes when designing rehabilitation trials, to both reflect the potential effects of exercise on a variety of outcomes, but also to facilitate mechanistic studies that are able to link which body structures and body functions are most important to improving patient outcomes at the activity and participation domains. Addressing environmental factors within the ICF is also important for people with CF, such as resource allocation towards exercise and rehabilitation resources. Finally, this review highlights the need for more vigorous methodological designs to minimize risk of bias and improve quality of exercise studies in CF.

Background: Exercise is an important part of a healthy lifestyle for people with cystic fibrosis (CF). Exercise testing is also recommended as part of a routine assessment to provide physical activity counseling/exercise prescription. Body composition assessment is also an important assessment to can also guide dietary counseling. Prior studies have found that exercise testing and exercise training programs are underutilized in CF care centers, and no one has assessed the prevalence of body composition assessment. Thus, the purpose of this study was to develop and validate a survey to explore the barriers and facilitators that obstruct or promote the use of exercise and body composition testing in the CF care centers. Methods: Seventy-nine individuals with expertise in exercise and CF were invited to participate in a Delphi study. They rated the importance and quality of a 106-item survey on exercise and body composting testing and exercise training and shared their feedback. Results: Thirteen subject matter experts (SMEs) agreed to participate in the study, which lasted 3 rounds, but only 6 of them were in the final round. Of the 106 original items, 46 were kept with revisions, 58 were revised, 2 items were dropped, and 8 items were added, resulting in a final survey of 114 items. Conclusions: This Delphi study validated a survey to be used in CF care centers to provide valuable information about the benefits and barriers to exercise testing, exercise training, and body composition assessment in people with CF in the CFTR-modulator era.