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. 2022 Sep 6;19:14799731221121670. doi: 10.1177/14799731221121670

Table 3.

Final consensus statements and associated agreement from group members.

Statement Agree n (%) Do not agree n (%) Abstain n (%)
The rationale for physical activity and exercise in CF – Why are these factors important for people with CF?
1 Physical activity and exercise contribute to increased survival in people with cystic fibrosis and can also improve an individual’s sense of overall well-being 36 (95) 2 (5) 0 (0)
2 Engagement in physical activity and exercise has the potential to positively influence the cost associated with hospitalisations, exacerbations, and antibiotic use 34 (89) 3 (8) 1 (3)
Health benefits of physical activity and exercise in CF – How does changing these factors benefit people with cystic fibrosis?
3 Physical activity and exercise can improve cardiovascular health in people with cystic fibrosis, however there is limited research on this topic 33 (87) 3 (8) 2 (5)
4 Resistance training improves limb muscle strength in people with cystic fibrosis 35 (92) 2 (5) 1 (3)
5 Exercise of adequate frequency and progressive intensity improves fitness in people with cystic fibrosis 36 (95) 2 (5) 0 (0)
6 Health-related quality of life is an important and holistic outcome measure that should be included in physical activity and exercise intervention research, but the clinical effectiveness of physical activity and exercise interventions on the different domains of health-related quality of life remains unclear 36 (95) 0 (0) 2 (5)
Measurement of physical activity and exercise in CF – How do we monitor these factors?
7 Accelerometers provide accurate information regarding the volume and accumulation of physical activity, particularly when tailored for use in people with cystic fibrosis, but self-report data is still required to provide contextual, and complementary, information 34 (89) 2 (5) 2 (5)
8 There are many safe, effective, and informative options for exercise testing in people with cystic fibrosis regardless of age or disease severity 37 (97) 1 (3) 0 (0)
9 Lab-based tests provide greater insights to the determinants of exercise capacity, but field-based tests can provide useful information if lab-based tests are not available 35 (92) 2 (5) 1 (3)
10 Standardised methods of exercise testing and physical activity assessment should be utilised to enable within- and between-person comparisons, facilitating the individualisation of physical activity promotion and/or exercise prescription 36 (95) 1 (3) 1 (3)
Prescription of physical Activity and exercise in CF – How do we facilitate change and get people with cystic fibrosis moving (more)?
11 An individualised and comprehensive training program, undertaken at a moderate intensity or higher, as part of the ongoing therapeutic routine is recommended in people with cystic fibrosis 35 (92) 2 (5) 1 (3)
12 Lifelong adherence to habitual physical activity and exercise across the lifespan is important and challenging, due to the many competing and time-consuming activities 34 (89) 4 (11) 0 (0)
13 The provision of exercise within cystic fibrosis care is becoming increasingly important and requires additional staffing and expertise at the level required to offer annual exercise testing and prescription, including the option of cardiopulmonary exercise testing. Standardisation of the education, training and roles and responsibilities of exercise specialists and other healthcare professionals responsible for the provision of exercise services is required 36 (95) 2 (5) 0 (0)
14 Exercise training delivered remotely by video calling is feasible and acceptable to people with cystic fibrosis in a small number of pilot studies, and clinical teams could consider its use as a supplement to face-to-face contact 37 (97) 1 (3) 0 (0)
Clinical considerations for physical activity and exercise in CF – What must we also consider when prescribing activity and exercise for people with cystic fibrosis
15 Exercise with huff or cough improves mucus clearance, however there is no medium- or long-term evidence if exercise can adequately replace airway clearance techniques in people with cystic fibrosis 38 (100) 0 (0) 0 (0)
16 The clinical efficacy of CFTR modulator therapy on the fitness and physical activity behaviours of people with cystic fibrosis warrants further study. Including exercise testing measurements as standard alongside routinely collected clinical data within cystic fibrosis registries may begin to allow a better understanding of the physiological effects of modulator therapies 32 (84) 3 (8) 3 (8)
17 Physical activity and exercise are strongly recommended for people with cystic fibrosis. Although the risk is minimal, guidance should be provided to all people with cystic fibrosis around hydration, nutrition, exercise intensity and duration, based on the specific activity 36 (95) 1 (3) 1 (3)
18 Both energy intake and hydration should be adjusted to meet the elevated caloric and fluid needs of an active person with cystic fibrosis. Every exercise/diet plan should be discussed with the physician and dietician to meet the individual’s body mass index targets, caloric goals, and diet preferences 34 (89) 2 (5) 2 (5)
Future directions for physical activity and exercise in CF
19 The prescription of physical activity and exercise should be individualised for people with cystic fibrosis. All aspects of disease management and personal patient preferences should be considered 36 (95) 1 (3) 1 (3)
20 Clinical trials in cystic fibrosis should include outcomes associated with physical activity and exercise to understand how lifestyle parameters can be affected by pharmaceutical and non-pharmaceutical interventions 36 (97) 1 (3) 0 (0)
21 Future trials of physical activity and exercise should seek to include outcomes that are currently under-reported, such as cardiovascular health, mental health, endocrine, inflammatory function, and patient reported outcomes, to help further evaluate the effects of physical activity and exercise upon overall health status 38 (100) 0 (0) 0 (0)
22 Future research should examine: A. Whether exercise can adequately replace airway clearance techniques.
B. The interactive effects of CFTR modulators, physical activity, and exercise
C. How to improve adherence, including overcoming barriers, identifying enablers, and developing routines, to physical activity and structured exercise programmes
a) 32 (84)
b) 34 (89)
c) 33 (87)

All values are presented in percentages, following voting process whereby n =38 group members cast votes. Totals may not always equal 100% due to rounding. Excluded statements provided in Supplemental File 3.