The Exeter Activity Unlimited statement on physical activity and exercise for cystic fibrosis: methodology and results of an international, multidisciplinary, evidence-driven expert consensus

Abstract

Background The roles of physical activity (PA) and exercise within the management of cystic fibrosis (CF) are recognised by their inclusion in numerous standards of care and treatment guidelines. However, information is brief, and both PA and exercise as multi-faceted behaviours require extensive stakeholder input when developing and promoting such guidelines. Method On 30th June and 1st July 2021, 39 stakeholders from 11 countries, including researchers, healthcare professionals and patients participated in a virtual conference to agree an evidence-based and informed expert consensus about PA and exercise for people with CF. This consensus presents the agreement across six themes: (i) patient and system centred outcomes, (ii) health benefits, iii) measurement, (iv) prescription, (v) clinical considerations, and (vi) future directions. The consensus was achieved by a stepwise process, involving: (i) written evidence-based synopses; (ii) peer critique of synopses; (iii) oral presentation to consensus group and peer challenge of revised synopses; and (iv) anonymous voting on final proposed synopses for adoption to the consensus statement. Results The final consensus document includes 24 statements which surpassed the consensus threshold (>80% agreement) out of 30 proposed statements. Conclusion This consensus can be used to support health promotion by relevant stakeholders for people with CF.

Survey of exercise testing and training in cystic fibrosis clinics in the UK: a decade of progress

“…The anticipated health gains for people on ETI, along with the potential for weight gain, are expected to lead to an increased focus on exercise for its management of CF, such as promoting airway clearance therapy, 43 and the multitude of extrapulmonary health benefits. 38 This study demonstrates a shift in availability of exercise testing and training for pwCF over the past decade but also highlights that the progress made requires to be built on with improvements still being required in regard to test selection, test standardisation and offering tailored exercise programmes to people with CF. It would be good to see these outstanding issues being tackled quickly to meet this prescient need.…”

“… 35 Considering this increased value of exercise professionals is alongside increasing recognition for the health benefits associated with exercise by society in general, perceived benefit of CFTR modulator treatments on perspectives on quality of life and health status 36 and the recognised benefit of exercise professionals by pwCF themselves; 37 CF MDTs should consider the incorporation of exercise specialists 27 or clinical exercise physiologists 35 into their teams. 38 These roles exist in countries outside of the UK, 39–41 and it is likely their prevalence will increase within the National Health Service, given a recent charter and push for standardised roles. 35 …”

Adapting, restarting, and terminating a randomised control trial for people with cystic fibrosis: Reflections on the impact of the COVID-19 pandemic upon research in a clinical population

“…Whilst a disappointing final result for the exceptional time and effort placed into the running of a RCT, this also remains an important finding, and helps guide the CF community towards finding ways of engaging people with CF in PA and exercise, and how to improve these trials for improvement of health status, particularly in hard-to-reach clinical groups. Identifying these optimal modes (should they even exist) has been identified as a focal point by researchers and the wider CF community [ 64 ], and therefore, the present work can still contribute towards this research field, despite the issues described herein.…”

A systematic review to explore how exercise-based physiotherapy via telemedicine can promote health related benefits for people with cystic fibrosis

“…The frequency of telemedicine guided exercises varied from once a week to daily and the intensity of exercise was only stated in three studies [26,28,33]. The majority of the studies utilised a combination of aerobic exercises, rather than focussing on one method [26][27][28][29][30][31]33], and this heterogeneity in programmes may be due to the unique characteristics of pwCF, consolidating how they may require more specific individualised exercise programmes; whereby individualisation of programmes has been recently advocated for by international experts in exercise management of CF [37].…”