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.

Background: Habitual physical activity (PA) and exercise form a cornerstone of the management of cystic fibrosis (CF), a genetically inherited pulmonary and digestive condition - whereby telehealth platforms have been proposed as a mechanism to engage remotely people with CF in PA and exercise. Methods: To test this, in early 2020, the 'ActivOnline: Physical Activity in Cystic Fibrosis Trial' (ActiOn PACT) randomised control trial was established to examine whether an online intervention was effective at increasing PA in adolescents and adults with CF. Results: The emergence of the COVID-19 pandemic in 2020 forced this trial to be paused and modified, with the adoption of online recruitment and remote assessment of outcome measures. Despite such adaptations in accord with frameworks developed by the National Institute for Health Research, this trial failed to recruit and was subsequently terminated. Conclusions: This article details the authors reflections upon the proposed reasons for lack of recruitment, including improved technology and medications for people with CF, and contextualises this finding in relation to the wider issue of non-reporting of trial results in clinical research.

Getting Your First Publication in Medical Education-Why? What? Where? How?

The process of getting one's work published is a major milestone for many in their early academic and clinical careers. However, this process can be confusing and overwhelming for many who have yet to publish themselves. There are differing motivators for publishing work in our early career stages, alongside considerations, such as what we publish, where we decide to submit work, and how we logistically undertake the submission process. This commentary provides a holistic overview for the early career medical educator, empowering them to take the bold steps toward "getting published."

Predatory publishing in medical education: a rapid scoping review.

BACKGROUND: Academic publishing is a cornerstone of scholarly communications, yet is unfortunately open to abuse, having given rise to 'predatory publishers'- groups that employ aggressive marketing tactics, are deficient in methods and ethics, and bypass peer review. Preventing these predatory publishers from infiltrating scholarly activity is of high importance, and students must be trained in this area to increase awareness and reduce use. The scope of this issue in the context of medical students remains unknown, and therefore this sought to examine the breadth of the current literature base. METHODS: A rapid scoping review was undertaken, adhering to adapted PRISMA guidelines. Six databases (ASSIA, EBSCO, Ovid, PubMed, Scopus, Web of Science) were systematically searched for content related to predatory publishing and medical students. Results were single-screened, facilitated by online reviewing software. Resultant data were narratively described, with common themes identified. RESULTS: After searching and screening, five studies were included, representing a total of 1338 students. Two predominant themes- understanding, and utilisation- of predatory publishers was identified. These themes revealed that medical students were broadly unaware of the issue of predatory publishing, and that a small number have already, or would consider, using their services. CONCLUSION: There remains a lack of understanding of the threat that predatory publishers pose amongst medical students. Future research and education in this domain will be required to focus on informing medical students on the issue, and the implication of engaging with predatory publishers.

Cardiopulmonary Exercise Testing Provides Prognostic Information in Advanced Cystic Fibrosis Lung Disease.

Rationale: Cardiopulmonary exercise testing (CPET) provides prognostic information in cystic fibrosis (CF); however, its prognostic value for patients with advanced CF lung disease is unknown. Objectives: To determine the prognostic value of CPET on the risk of death or lung transplant (LTX) within 2 years. Methods: We retrospectively collected data from 20 CF centers in Asia, Australia, Europe, and North America on patients with a forced expiratory volume in 1 second (FEV1) ⩽ 40% predicted who performed a cycle ergometer CPET between January 2008 and December 2017. Time to death/LTX was analyzed using mixed Cox proportional hazards regression. Conditional inference trees were modeled to identify subgroups with increased risk of death/LTX. Results: In total, 174 patients (FEV1, 30.9% ± 5.8% predicted) were included. Forty-four patients (25.5%) died or underwent LTX. Cox regression analysis adjusted for age, sex, and FEV1 revealed percentage predicted peak oxygen uptake ([Formula: see text]o2peak) and peak work rate (Wpeak) as significant predictors of death/LTX: adjusted hazard ratios per each additional 10% predicted were 0.60 (95% confidence interval, 0.43-0.90; P = 0.008) and 0.60 (0.48-0.82; P < 0.001). Tree-structured regression models, including a set of 11 prognostic factors for survival, identified Wpeak to be most strongly associated with 2-year risk of death/LTX. Probability of death/LTX was 45.2% for those with a Wpeak ⩽ 49.2% predicted versus 10.9% for those with a Wpeak > 49.2% predicted (P < 0.001). Conclusions: CPET provides prognostic information in advanced CF lung disease, and Wpeak appears to be a promising marker for LTX referral and candidate selection.

Agreement between left and right middle cerebral artery blood velocity responses to incremental and constant work-rate exercise in healthy males and females.

Objective.To quantify the agreement between left and right middle cerebral artery blood velocity (MCAv) responses to incremental and constant work-rate exercise in adults.ApproachSeventeen healthy adults (23.8 ± 2.4 years, 9 females) completed a ramp incremental test to exhaustion on a cycle ergometer, three 6-minute transitions at a moderate-intensity, and three at a heavy-intensity, all on separate days. Bilateral MCAv was measured throughout using transcranial Doppler ultrasonography, with left and right MCAv data analysed separately. Data were analysed at baseline, gas exchange threshold, respiratory compensation point and exhaustion during ramp incremental exercise. MCAv responses to constant work-rate exercise were analysed using a mono-exponential model, to determine time- and amplitude-based kinetic response parameters.Main ResultsLeft and right MCAv responses to incremental and constant work-rate exercise were significantly, strongly and positively correlated (r≥ 0.61,P< 0.01). Coefficient of variation (left versus right) ranged from 7.3%-20.7%, 6.4%-26.2% and 5.9%-22.5% for ramp, moderate and heavy-intensity exercise, respectively. The relative change in MCAv from baseline was higher in the right compared to left MCAv during ramp, moderate and heavy-intensity exercise (allP< 0.05), but the effect sizes were small (d≤ 0.4). Small mean left-right differences were present during ramp incremental exercise at all time-points (<6 cm s-1; <4%), and for all kinetic parameters during moderate and heavy-intensity exercise (<3 cm s-1, <3%, <4 s).SignificanceThese findings demonstrate similarities between left and right MCAv responses to incremental and constant-work rate exercise in adults on a group-level, but also highlight individual variation in the agreement between left and right MCAv exercise responses.

Expert guidelines on exercise and physical activity for people with cystic fibrosis.

Research has shown that there is a lack of confidence and understanding in how to use exercise for managing cystic fibrosis. This editorial discusses the key points of a consensus statement that highlights what is and is not known about the relationship between cystic fibrosis and exercise.

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

To conduct a systematic review to evaluate the effects of physiotherapy exercises delivered via telemedicine on lung function and quality-of-life in people with Cystic Fibrosis (CF). The databases AMED, CINAHL and MEDLINE were searched from December 2001 until December 2021. Reference lists of included studies were hand-searched. The PRISMA 2020 statement was used to report the review. Studies of any design reported in the English language, included participants with CF, and within outpatient settings were included. Meta-analysis was not deemed appropriate due to the diversity of interventions and heterogeneity of the included studies. Following screening, eight studies with 180 total participants met the inclusion criteria. Sample sizes ranged from 9 to 41 participants. Research designs included five single cohort intervention studies, two randomised control trials and one feasibility study. Telemedicine-based interventions included Tai-Chi, aerobic, and resistance exercise delivered over a study period of six to twelve weeks. All included studies which measured percentage predicted forced expiratory volume in one second found no significant difference. Five studies measuring the Cystic Fibrosis Questionnaire-Revised (CFQ-R) respiratory domain found improvements, however, did not meet statistical significance. For the CFQ-R physical domain, measured by five studies, two studies found an improvement, although not statistically significant. No adverse events were reported across all studies. The included studies indicate that telemedicine-based exercise over 6-12 weeks does not significantly change lung function or quality-of-life in people with CF. Whilst the role of telemedicine in the care of pwCF is acceptable and promising; further research with standardised outcome measures, larger sample sizes and longer follow-up are required before clinical practice recommendations can be developed.

Validity and repeatability of cardiopulmonary exercise testing in interstitial lung disease.

BACKGROUND: Cardiopulmonary exercise testing (CPET), and its primary outcome of peak oxygen uptake (VO2peak), are acknowledged as biomarkers in the diagnostic and prognostic management of interstitial lung disease (ILD). However, the validity and repeatability of CPET in those with ILD has yet to be fully characterised, and this study fills this evidence gap. METHODS: Twenty-six people with ILD were recruited, and 21 successfully completed three CPETs. Of these, 17 completed two valid CPETs within a 3-month window, and 11 completed two valid CPETs within a 6-month window. Technical standards from the European Respiratory Society established validity, and repeatability was determined using mean change, intraclass correlation coefficient and typical error. RESULTS: Every participant (100%) who successfully exercised to volitional exhaustion produced a maximal, and therefore valid, CPET. Approximately 20% of participants presented with a plateau in VO2, the primary criteria for establishing a maximal effort. The majority of participants otherwise presented with secondary criteria of respiratory exchange ratios in excess of 1.05, and maximal heart rates in excess of their predicted values. Repeatability analyses identified that the typical error (expressed as percent of coefficient of variation) was 20% over 3-months in those reaching volitional exhaustion. CONCLUSION: This work has, for the first time, fully characterised how patients with ILD respond to CPET in terms of primary and secondary verification criteria, and generated novel repeatability data that will prove useful in the assessment of disease progression, and future evaluation of therapeutic regimens where VO2peak is used as an outcome measure.

Normal reference values for aerobic fitness in cystic fibrosis: a scoping review.

Objective: The importance of aerobic fitness (VO2peak) in cystic fibrosis (CF) is well established, and regular exercise testing is recommended. To standardise VO2peak, a 'percentage of predicted' (%pred) derived from normative reference values (NRV), as promoted by the 2015 European Cystic Fibrosis Society Exercise Working Group (ECFS EWG), can be reported. However, the NRVs used in CF and their relative frequency is unknown. Method: A scoping review was performed via systematic database searches (PubMed, Embase, Web of Science, SciELO, EBSCO) and forward citation searches for studies that include people with CF and report VO2peak as %pred. Studies were screened using Covidence, and data related to patient demographics, testing modality and reference equations were extracted. Additional analyses were performed on studies published in 2016-2021, following the ECFS EWG statement in 2015. Results: A total of 170 studies were identified, dating from 1984 to 2022, representing 6831 patients with CF, citing 34 NRV. Most studies (154/170) used cycle ergometry, 15/170 used treadmills, and the remainder used alternative, combination or undeclared modalities. In total, 61/170 failed to declare the NRV used. There were 61 studies published since the ECFS EWG statement, whereby 18/61 used the suggested NRV. Conclusion: There is a wide discrepancy in NRV used in the CF literature base to describe VO2peak as %pred, with few studies using NRV from the ECFS EWG statement. This high variance compromises the interpretation and comparison of studies while leaving them susceptible to misinterpretation and limiting replication. Standardisation and alignment of reporting of VO2peak values are urgently needed.

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

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.

The effect of exercise intensity and cardiorespiratory fitness on the kinetic response of middle cerebral artery blood velocity during exercise in healthy adults.

The aim of this study was to compare the kinetic response of middle cerebral artery blood velocity (MCAv) to moderate- and heavy-intensity cycling in adults, and explore the relationship between maximal oxygen uptake (V̇o2max) and MCAv kinetics. Seventeen healthy adults (23.8 ± 2.4 yr, 9 females) completed a ramp incremental test to exhaustion on a cycle ergometer to determine V̇o2max and the gas exchange threshold (GET). Across six separate visits, participants completed three 6-min transitions at a moderate intensity (90% GET) and three at a heavy intensity (40% of the difference between GET and V̇o2max). Bilateral MCAv was measured using transcranial Doppler (TCD) ultrasonography and analyzed using a monoexponential model with a time delay. The time constant (τ) of the MCAv response was not different between moderate- and heavy-intensity cycling (25 ± 10 vs. 26 ± 8 s, P = 0.82), as was the time delay (29 ± 11 vs. 29 ± 10 s, P = 0.95). The amplitude of the exponential increase in MCAv from baseline was greater during heavy-intensity cycling (23.9 ± 10.0 cm·s-1, 34.1 ± 14.4%) compared with moderate-intensity cycling (12.7 ± 4.4 cm·s-1, 18.7 ± 7.5%; P < 0.01). Following the exponential increase, a greater fall in MCAv was observed during heavy-intensity exercise compared with moderate-intensity exercise (9.5 ± 6.9 vs. 2.8 ± 3.8 cm·s-1, P < 0.01). MCAv after 6 min of exercise remained elevated during heavy-intensity exercise compared with moderate-intensity exercise (85.2 ± 9.6 vs. 79.3 ± 7.7 cm·s-1, P ≤ 0.01). V̇o2max was not correlated with MCAv τ or amplitude (r = 0.11-0.26, P > 0.05). These data suggest that the intensity of constant-work rate exercise influences the amplitude, but not time-based, response parameters of MCAv in healthy adults, and found no relationship between cardiorespiratory fitness and MCAv kinetics.NEW & NOTEWORTHY This is the first study to model the MCAv kinetic response to moderate- and heavy-intensity cycling in healthy adults. This study found that the amplitude of the exponential rise in MCAv at exercise onset was greater during heavy-intensity exercise (∼34%) compared with moderate-intensity exercise (∼19%), but the time-based characteristics of the responses were similar between intensities. Higher cardiorespiratory fitness was not associated with a greater or faster MCAv response to moderate- or heavy-intensity exercise.

The Effect of Dysglycaemia on Changes in Pulmonary and Aerobic Function in Cystic Fibrosis.

Cross-sectional studies have reported lower pulmonary and aerobic function during exercise in people with cystic fibrosis-related diabetes (CFRD) compared to non-CFRD counterparts. However, this association has yet to be longitudinally investigated. Therefore, this study examines these differences over time between people with cystic fibrosis (CF) of differing glycaemic status. Annual review data, including cardiopulmonary exercise tests and pulmonary function tests, were retrospectively analysed at baseline (T0, n = 82) and at a one-year follow-up (T1, n = 54). Data was analysed in three groups: normal glucose tolerance (NGT), impaired glucose tolerance (IGT), and CFRD. Further analyses were undertaken, with a dichotomous split of NGT and a combined IGT/CFRD group. At baseline, a significant reduction in the majority of variables, including forced expiratory volume in one second (FEV1) and maximal oxygen uptake (VO2max), was observed in the CFRD (n = 19) group compared to NGT (n = 58). At follow-up, no significant differences were observed, and no interaction effect between CFRD status and time was identified. FEV1 and VO2max presented with varying directions and magnitudes of change within patients. In summary, patients with CFRD have a reduced aerobic and pulmonary function compared to non-CFRD counterparts, although such changes disappeared at follow up. Varying responses for FEV1 and VO2max highlight the need to consider both variables as independent markers of function in CF.

The impact of COVID-19 upon the delivery of exercise services within cystic fibrosis clinics in the United Kingdom.

OBJECTIVES: The COVID-19 pandemic has resulted in unprecedent changes to clinical practice, and as the impact upon delivery of exercise services for people with cystic fibrosis (CF) in the United Kingdom was unknown, this was characterised via a national survey. METHODS: An electronic survey was distributed to healthcare professionals involved in the exercise management of CF via established professional networks. RESULTS: In total, 31 CF centres participated. Findings included significant reductions in exercise testing and widespread adaptation to deliver exercise training using telehealth methods. Promisingly, 71% stated that they would continue using virtual methods of engaging patients in future practice. CONCLUSION: These findings highlight adaptation to the COVID-19 pandemic and the need to develop sustainable and standardised telehealth services to manage patients moving forwards.

Differences in cerebrovascular regulation and ventilatory responses during ramp incremental cycling in children, adolescents, and adults.

Regulation of cerebral blood flow during exercise in youth is poorly understood. This study investigated the cerebrovascular and ventilatory responses to a ramp incremental cycle test to exhaustion in 14 children (means ± SD age: 9.4 ± 0.9 yr), 14 adolescents (12.4 ± 0.4 yr), and 19 adults (23.4 ± 2.5 yr). Middle cerebral artery blood velocity (MCAv), partial pressure of end-tidal CO2 ([Formula: see text]), and ventilatory parameters were analyzed at baseline, gas exchange threshold (GET), respiratory compensation point (RCP), and exhaustion. The increase in minute ventilation relative to CO2 production during exercise was also calculated (V̇e/V̇co2 slope). Relative change from baseline (Δ%) in MCAv was lower in children, compared with adolescents and adults at GET [15 ± 10% vs. 26 ± 14%, and 24 ± 10%, respectively, P ≤ 0.03, effect size (d) = 0.9] and RCP (13 ± 11% vs. 24 ± 16% and 27 ± 15%, respectively, P ≤ 0.05, d ≥ 0.8). Δ%MCAv was similar in adults and adolescents at all intensities and similar in all groups at exhaustion. The magnitude of the V̇E/V̇co2 slope was negatively associated with Δ%MCAv at GET and RCP across all participants (P ≤ 0.01, r = -0.37 to -0.48). Δ%[Formula: see text] was smaller in children and adolescents compared with adults at GET and RCP (P ≤ 0.05, d ≥ 0.6). In children, Δ%[Formula: see text] and Δ%MCAv were not associated from baseline-GET (r¯ = 0.14) and were moderately associated from RCP-exhaustion (r¯ = 0.49). These relationships strengthened with increasing age and were stronger in adolescents (baseline-GET: r¯ = 0.47, RCP-exhaustion: r¯ = 0.62) and adults (baseline-GET: r¯ = 0.66, RCP-exhaustion: r¯ = 0.78). These findings provide the first evidence on the development of the regulatory role of [Formula: see text] on MCAv during exercise in children, adolescents, and adults.NEW & NOTEWORTHY This is the first study to observe similar increases in cerebral blood flow during incremental exercise in adolescents and adults. Increases in cerebral blood flow during exercise were smaller in children compared with adolescents and adults and were associated with a greater V̇E/V̇co2 slope. This study also provides the first evidence on the progressive development of the regulatory role of end-tidal CO2 on cerebral blood flow during exercise during the transition from childhood to adulthood.

The impact of physical activity and exercise interventions for physical health in people with cystic fibrosis: protocol for a systematic review.

BACKGROUND: Cystic fibrosis (CF) is a genetically inherited, life-limiting condition, affecting ~90,000 people globally. Physical activity (PA) and exercise form an integral component of CF management, and have been highlighted by the CF community as an area of interest for future research. Previous reviews have solely focused on PA or structured exercise regimens independent of one another, and thus a comprehensive assessment of the physical health benefits of all PA, including exercise, interventions, is subsequently warranted. Therefore, the purpose of this review is to evaluate the effects of both PA and exercise upon outcomes of physical health and healthcare utilisation in people with CF. METHODS: A systematic review has been registered and reported in line with Preferred Reporting Items for Systematic Reviews and Meta-Analysis-P guidelines. This will include randomised control trials on the effects of PA and exercise, relative to usual treatment, upon people with CF. Primary outcomes will include variables associated with fitness, PA, lung health, inflammation, body composition, glycaemic control and patient-reported outcomes. Secondary outcomes will include adverse events and healthcare utilisation. Searches will be undertaken in Ovid MEDLINE, OVID EMBASE, PsychINFO, ERIC, SPORTDiscus, ASSIA, CCTR, CINHAL and Web of Science databases, and will be searched from date of inception onwards. Two reviewers will independently screen citations and abstracts, and full-texts, for inclusion and data extraction, respectively. Methodological quality will be assessed using the Cochrane Risk of Bias-2 tool. If feasible, random-effects meta-analyses will be conducted where appropriate. Additional analyses will explore potential sources of heterogeneity, such as age, sex, and disease severity. DISCUSSION: This systematic review will build on previous research, by comprehensively assessing the impact of both PA and exercise upon physical health and healthcare utilisation in people with CF. Results of this review will be utilised to inform discussions that will ultimately result in a consensus document on the impact of physical activity and exercise for people with CF. SYSTEMATIC REVIEW REGISTRATION: PROSPERO CRD42020184411.

Physical activity for cystic fibrosis: perceptions of people with cystic fibrosis, parents and healthcare professionals.

BACKGROUND: The benefits of physical activity (PA) for people with cystic fibrosis (pwCF) are widely accepted, yet how PA is promoted and utilised by pwCF is unclear. METHOD: An online questionnaire to explore attitudes, practices and promotion of PA in cystic fibrosis was completed by healthcare providers (HCP), pwCF and parents/caregivers. RESULTS: 351 respondents (105 HCP, 120 pwCF, and 126 parents/caregivers) from 12 countries completed the survey. Importance of PA was rated highly by the majority of respondents. Physical (e.g. health), psychological (e.g. enjoyment) and social (e.g. social interaction) factors were motives for PA for 82%, 49% and 37% of pwCF, respectively, irrespective of country. Common barriers to PA included time (49% and 36%) and tiredness (61% and 7%) for pwCF and parents/carers, respectively. pwCF also reported psychosocial barriers (e.g. stigma, demoralisation), while parents/caregivers reported structural barriers (e.g. cost). Clinical teams varied substantially in terms of the emphasis placed on PA, facilities available, staff and training, and advice given to pwCF. CONCLUSION: Despite the majority of participants rating the importance of PA highly, substantial variability was evident regarding the facilities and clinical support available to them, as well as why and how people were active. There remains a need to identify what constitutes "best practice" for PA promotion within clinics.

Desaturation during exercise is not a sufficient mechanism for prediction of osteoporosis in non-cystic fibrosis bronchiectasis.

BACKGROUND: Recent research has proposed an association between desaturation during a six minute walking test (6MWT) and osteoporosis in an elderly group of individuals with non-cystic fibrosis bronchiectasis. A causative pathway through activation of hypoxia-inducible factor 1-alpha (HIF-1α) has been proposed. COMMENTARY: Queries regarding the statistical approaches used are identified and discussed within this correspondence. These predominate around the use of linear regression models to predict osteoporosis in a group that is already osteoporotic, presenting with extreme values for bone mineral density (BMD). Further queries are raised regarding the HIF-1α pathway, and physical activity (PA) is proposed as an upstream mechanism for both reduced exercise tolerance and low BMD. CONCLUSIONS: It is suggested that osteoporosis cannot be predicted in a group that is already osteoporotic, and that PA is likely to be the causative mechanism between desaturation in the 6MWT and low BMD in non-cystic fibrosis bronchiectasis.

The feasibility of online video calling to engage patients with cystic fibrosis in exercise training.

INTRODUCTION: Physical activity, including structured exercise, is an essential component in the management of cystic fibrosis. The use of telehealth such as video-calling may be a useful method for the delivery of exercise and physical activity interventions, though the feasibility of this remains unknown. METHODS: Nine patients with cystic fibrosis (three female, six male, 30.9 ± 8.7 years) volunteered to participate. Participants completed an eight-week exercise training intervention conducted via Skype, using personalised exercises, with all sessions supervised by an exercise therapist. Feasibility was assessed by demand, implementation, practicality and acceptability. Changes in anthropometric, pulmonary, physical activity and quality of life variables were also assessed. RESULTS: Two male participants withdrew from the study, citing lack of available time. The remaining participants found use of Skype useful, with a mean satisfaction rating of 9/10, and three participants requesting to continue the sessions beyond the duration of the study. Mean compliance with sessions was 68%, with mean duration of sessions being 20 min. A total of 25% of calls suffered from technical issues such as video or audio lags. Anthropometric, pulmonary, physical activity and quality of life variables remained unchanged over the course of the study period. DISCUSSION: The use of Skype to deliver an exercise intervention to patients withcystic fibrosis was found to be technologically feasible, and acceptable among participants. Findings have implications for clinical practice and could allow care teams to engage patients remotely in exercise. Further research is required to assess the efficacy of this modality on increasing physical activity and associated health outcomes.

A web-based intervention to promote physical activity in adolescents and young adults with cystic fibrosis: protocol for a randomized controlled trial.

BACKGROUND: Regular participation in physical activity by people with cystic fibrosis (CF) promotes positive clinical and health outcomes including reduced rate of decline in lung function, fewer hospitalizations and greater wellbeing. However adherence to exercise and activity programs is low, in part due to the substantial daily therapy burden for young people with CF. Strict infection control requirements limit the role of group exercise programs that are commonly used in other clinical groups. Investigation of methods to promote physical activity in this group has been limited. The Active Online Physical Activity in Cystic fibrosis Trial (ActionPACT) is an assessor-blinded, multi-centre, randomized controlled trial designed to compare the efficacy of a novel web-based program (ActivOnline) compared to usual care in promoting physical activity participation in adolescents and young adults with CF. METHODS: Adolescents and young adults with CF will be recruited on discharge from hospital for a respiratory exacerbation. Participants randomized to the intervention group will have access to a web-based physical activity platform for the 12-week intervention period. ActivOnline allows users to track their physical activity, set goals, and self-monitor progress. All participants in both groups will be provided with standardised information regarding general physical activity recommendations for adolescents and young adults. Outcomes will be assessed by a blinded assessor at baseline, after completion of the intervention, and at 3-months followup. Healthcare utilization will be assessed at 12 months from intervention completion. The primary outcome is change in moderate-to-vigorous physical activity participation measured objectively by accelerometry. Secondary outcomes include aerobic fitness, health-related quality of life, anxiety and depression and sleep quality. DISCUSSION: This trial will establish whether a web-based application can improve physical activity participation more effectively than usual care in the period following hospitalization for a respiratory exacerbation. The web-based application under investigation can be made readily and widely available to all individuals with CF, to support physical activity and exercise participation at a time and location of the user's choosing, regardless of microbiological status. TRIAL REGISTRATION: Clinical trial registered on July 13, 2017 with the Australian and New Zealand Clinical Trials Register at (ACTRN12617001009303).