Validity and reliability concerns associated with cardiopulmonary exercise testing young people with cystic fibrosis

This is the author accepted manuscript. The final version is available from the publisher via the DOI in this record.Letter to the Edito

Relationship between (non)linear phase II pulmonary oxygen uptake kinetics with skeletal muscle oxygenation and age in 11 to 15 y olds

This is the author accepted manuscript. the final version is available from Wiley via the DOI in this recordThis study investigated in nineteen male youth (mean age: 13.6 ± 1.1 y, range: 11.7 – 15.7 y) the relationship between pulmonary oxygen uptake ( o2) and muscle deoxygenation kinetics during moderate‐ and very heavy‐intensity ‘step’ cycling initiated from unloaded pedaling (i.e. U→M and U→VH) and moderate‐to‐very heavy‐ intensity step cycling (i.e. M→VH). Pulmonary o2 was measured breath‐by‐breath and tissue oxygenation index (TOI) of the vastus lateralis using near‐infrared spectroscopy. There were no significant differences in the phase II time constant (τ o2p) between U→M and U→VH (23 ± 6 s vs. 25 ± 7 s; P = 0.36); however, the τ o2p was slower during M→VH (42 ± 16 s) compared to other conditions (P < 0.001). Quadriceps TOI decreased with a faster (P < 0.01) mean response time (MRT; i.e. time delay + τ) during U→VH (14 ± 2 s) compared to U→M (22 ± 4 s) and M→VH (20 ± 6 s). The difference (Δ) between the τ o2p and MRT‐TOI was greater during U→VH compared to U→M (12 ± 7 vs. 2 ± 7 s, P < 0.001) and during M→VH (23 ± 15 s) compared to other conditions (P < 0.02), suggesting an increased proportional speeding of fractional O2 extraction. The slowing of the τ o2p during M→VH relative to U→M and U→VH correlated positively with chronological age (r = 0.68 and 0.57, respectively, P < 0.01). In youth, “work‐to‐work” transitions slowed microvascular O2 delivery‐to‐O2 utilization with alterations in phase II o2 dynamics accentuated between the ages of 11 to 15 y

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

This is the final version. Available on open access from Wiley via the DOI in this recordObjectives: 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 UK 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

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

This is the final version. Available on open access from BMJ Publishing Group via the DOI in this recordData availability statement: Data are available upon reasonable request. Please contact the corresponding author to arrange access to study data.Objectives Regular exercise testing is recommended for people with cystic fibrosis (pwCF), as is the provision and regular review of exercise training programmes. A previous survey on exercise testing and training for pwCF in the UK was conducted over a decade ago. With the landscape of CF changing considerably during this time, this survey aimed to evaluate UK-based exercise testing and training practices for pwCF a decade on. Design Cross-sectional, online survey. Participants A survey was distributed electronically to UK CF clinics and completed by the individual primarily responsible for exercise services. Descriptive statistics and qualitative analyses were undertaken. Results In total, 31 CF centres participated, representing ~50% of UK specialist clinics. Of these, 94% reported using exercise testing, 48% of which primarily use cardiopulmonary exercise testing. Exercise testing mostly occurs at annual review (93%) and is most often conducted by physiotherapists (62%). A wide variation in protocols, exercise modalities, normative reference values and cut-offs for exercise-induced desaturation are currently used. All centres reportedly discuss exercise training with pwCF; 94% at every clinic appointment. However, only 52% of centres reportedly use exercise testing to inform individualised exercise training. Physiotherapists typically lead discussions around exercise training (74%). Conclusions These data demonstrate that the majority of respondent centres in the UK now offer some exercise testing and training advice for pwCF, representing a marked improvement over the past decade. However, continued efforts are now needed to standardise exercise practices, particularly regarding field testing practices and the translation of test results into personalised training programmes for pwCF

The effect of a novel, digital physical activity and emotional well-being intervention on health-related quality of life in people with chronic kidney disease: trial design and baseline data from a multicentre prospective, wait-list randomised controlled trial (kidney BEAM)

BACKGROUND: Physical activity and emotional self-management has the potential to enhance health-related quality of life (HRQoL), but few people with chronic kidney disease (CKD) have access to resources and support. The Kidney BEAM trial aims to evaluate whether an evidence-based physical activity and emotional wellbeing self-management programme (Kidney BEAM) leads to improvements in HRQoL in people with CKD. METHODS: This was a prospective, multicentre, randomised waitlist-controlled trial, with health economic analysis and nested qualitative studies. In total, three hundred and four adults with established CKD were recruited from 11 UK kidney units. Participants were randomly assigned to the intervention (Kidney BEAM) or a wait list control group (1:1). The primary outcome was the between-group difference in Kidney Disease Quality of Life (KDQoL) mental component summary score (MCS) at 12 weeks. Secondary outcomes included the KDQoL physical component summary score, kidney-specific scores, fatigue, life participation, depression and anxiety, physical function, clinical chemistry, healthcare utilisation and harms. All outcomes were measured at baseline and 12 weeks, with long-term HRQoL and adherence also collected at six months follow-up. A nested qualitative study explored experience and impact of using Kidney BEAM. RESULTS: 340 participants were randomised to Kidney BEAM (n = 173) and waiting list (n = 167) groups. There were 96 (55%) and 89 (53%) males in the intervention and waiting list groups respectively, and the mean (SD) age was 53 (14) years in both groups. Ethnicity, body mass, CKD stage, and history of diabetes and hypertension were comparable across groups. The mean (SD) of the MCS was similar in both groups, 44.7 (10.8) and 45.9 (10.6) in the intervention and waiting list groups respectively. CONCLUSION: Results from this trial will establish whether the Kidney BEAM self management programme is a cost-effective method of enhancing mental and physical wellbeing of people with CKD. TRIAL REGISTRATION: NCT04872933. Registered 5th May 2021

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

This is the final version. Available on open access from SAGE Publications via the DOI in this recordData availability statement: All results are presented within the manuscript and supplementary files.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.Cystic Fibrosis Trus

Exercise testing and training in cystic fibrosis clinics in the United Kingdom: a 10-year update

This is the author accepted manuscript. The final version is available from Elsevier via the DOI in this record

WS08.1 The utility of oxygen uptake efficiency as a marker of aerobic fitness in children with cystic fibrosis

This is the author accepted manuscript. The final version is available from Elsevier via the DOI in this record

The impact of plasma 25-hydroxyvitamin D on pulmonary function and exercise physiology in cystic fibrosis: a multicentre retrospective study

This is the author accepted manuscript. The final version is available from Wiley via the DOI in this recordBackground 25‐hydroxyvitamin D (25OHD) may exert immunomodulatory effects on respiratory health, which may translate to improvements in exercise physiology. Thus, we aimed to investigate whether plasma 25OHD is associated with lung function and aerobic fitness in people with cystic fibrosis (pwCF). Methods A multi‐centre retrospective review of pwCF (>9 years old) attending the Royal Hospital for Sick Children (Edinburgh) or Wessex CF‐Unit (Southampton) was performed between July 2017 to October 2019. Demographic and clinical data were collected. Plasma 25OHD measured closest in time to clinical cardiopulmonary exercise testing (CPET) and/or spirometry (forced expiratory volume FEV1% predicted) was recorded. Pancreatic insufficiency was diagnosed based on faecal elastase of <100 µg/g. We performed multiple‐regression analysis with aerobic fitness outcomes [peak oxygen uptake (VO2peak)] and FEV1% predicted as primary outcomes. Results Ninety pwCF [mean±SD age: 19.1±8.6 years, 54 (60%) children, 48 (53%) males and 88 (98%) Caucasian] were included. 25OHD deficiency and insufficiency was 15 (17%) and 44 (49%) respectively. 25OHD deficiency and insufficiency was significantly associated with pancreatic insufficiency (χ2(4.8); p = 0.02). Plasma 25OHD was not significantly associated with FEV1% predicted [R2 = 0.06; p= 0.42; 95%; CI (‐0.09 ‐ 0.19)] or VO2peak [R2= 0.04; p= 0.07; 95% CI (‐011 ‐ 0.005)] in all pwCF. However, 25OHD was significantly associated with both FEV1% [R2= 0.15; p= 0.02; 95% CI (1.99 ‐ 2.64)] and VO2peak [R2= 0.13; p= 0.05; 95% CI (‐0.26 – (‐0.005)] in the paediatric cohort. Conclusion We showed that 25OHD is associated with improved lung function and aerobic fitness in children and adolescents with CF. Mechanistic and high‐quality prospective studies including both lung function and aerobic fitness as primary outcomes are now warranted.NHS Lothian Research and Development Offic