A learning health system describes a team, provider or group of providers who develop learning from delivering routine health care in order to drive learning and improvement1Hardie T et al.Developing learning health systems in the UK: Priorities for action. The Health Foundation, 2022(https://doi.org/10.37829/HF-2022-I06)Crossref Google Scholar. CFHealthHub (www.cfhealthhub.com) is a learning health system designed to optimise CF care using quality improvement, digital data and tailored behaviour change interventions to optimise outcomes for people with CF, support treatment adherence and to deliver low-burden adherence research. A key aspect of CFHealthHub is a community of practice of CFHealthHub-trained clinicians at each of the 15 CFHealthHub CF centres delivering both clinical and research activities. To describe the feedback CFHealthHub-trained clinicians provided on the value of the community of practice. The CFHealthHub community of practice meet for an hour weekly, sharing knowledge and learning. One meeting focused on understanding clinicians' perspectives of the value of these meetings. A virtual whiteboard with open questions, followed by group discussion, identified ideas and themes. Themes of feedback for the questions were: Tabled 1QuestionResponses/ThemesWhat do you find useful about the CoP meetings?Maintains motivation, community, sharing learning, staying informed.What could be improved?More quality improvement sharing and training, more centre-led meetings, more sub-projects, themed weeks.What to cover in future meetings?Post-modulator challenges and changes, CFHealthHub-clinicians' roles in other centres, behaviour change case studies, strategic updates, journal club, medicines optimisation and sustainability.Other thoughts and suggestions.Using effective meeting skills with roles is helpful, record meetings for non-attendees, circulate slides/resources after meetings, thanks for the enthusiasm, approachability and encouragement. Open table in a new tab Clinicians valued weekly community of practice meetings. The timetable for future meetings will include topics suggested in the feedback, such as journal club and encouraging different centres to share their specific experiences, challenges and solutions. The community of practice is a key aspect of delivering both the clinical and research functions of CFHealthHub and provides an exemplar for other multi-centre clinical and research programmes.

Individual CF centres have few people not prescribed CFTR modulators (CFTRm). The CFHealthHub Learning Health System includes 15 UK CF centres and approximately 1400 people with CF, of which over 100 are not prescribed CFTRm. This allows collaborative study of the James Lind research priority question "What options are available for those not able to take current CFTR modulators?"1. Preventative inhaled therapies are effective2 but difficult to adhere to. Could optimising the use of inhaled therapy be an option for those not able to take CFTRm? To describe patterns of inhaled therapy use for the non-CFTRm population and for those prescribed CFTRm, within CFHealthHub. Real-time electronic data capture from data-logging nebulisers are collected as part of the CFHealthHub and National Efficacy-Effectiveness CFTR Modulator Optimisation (NEEMO) research programmes. Routinely collected clinical and inhalation data were analysed, for the six months before and six months after 21st August 2020 (UK commissioning of Elexacaftor/Tezacaftor/Ivacaftor[ETI]), to describe patterns of inhaled therapy use. In this preliminary analysis, data were available for 822 participants, with 753 in the CFTRm group and 69 in the non-CFTRm group. In the CFTRm group 356 (47.3%) were male with mean(SD) age 31.2years(10.1), while in the non-CFTRm group 35 (50.7%) were male with mean(SD) age 31.5years(13.2). There was a higher use of inhaled therapies in the CFTRm group before and after UK commissioning of ETI. Following ETI commissioning, inhaled therapy use dropped in the CFTRm group while use rose in the non-CFTRm group. Tabled 1Preventative inhaled therapy use per week (mean number of inhalations)Pre 6 months, mean (SD)Post 6 months, mean (SD)CFTR modulator group11.5 (11.6)10.4 (11.3)Non-CFTR modulator group7.6 (7.8)8.6 (8.5) Open table in a new tab Patterns of preventative inhaled therapy use differed between those prescribed CFTRm and those not prescribed CFTRm. CFHealthHub provides a unique opportunity to pay attention to optimising treatment for people who are not able to take current CFTR modulators.

Prior to the introduction of remote spirometry devices, people with cystic fibrosis (CF) would typically attend clinic appointments every three months or acutely where a lung function test may be performed. Evaluation of the frequency and grading of spirometry results was undertaken following the introduction of the NuvoAir Air Next lung function device. The primary objective of this service evaluation was to provide real-world spirometry data on the grading of lung function tests for the recently introduced NuvoAir Air Next spirometry device, as well as lung function test frequency data. Spirometry data was collected from 86 patients from one adult CF centre in the UK over a one-year period. Grading of data (A-G) for individual lung function tests completed on the NuvoAir Air Next was analysed. In addition, the mean frequency and mean time between lung function tests were measured using data from both CareFusion and NuvoAir Air Next lung function devices. Lung function results recorded between 1 April 21 to 31 March 22 using the NuvoAir Air Next demonstrated 77.2% (N = 322 out of 417) fall within an acceptable A-C grading. Furthermore, frequency data of lung function tests using the Carefusion and NuvoAir Air Next (N = 616) demonstrated that, on average, 7 lung function tests were performed annually with a time of 65 days between each test. This data provisionally suggests that this is more frequent than the previous 3 monthly recommendation. The service evaluation provides real-world spirometry NuvoAir Air Next data from one adult CF centre. Increasing lung health monitoring through more frequent lung function testing may contribute to providing timely treatment to prevent lung function decline in CF populations. As remote spirometry is established across CF centres it would be advantageous to undertake a multi-centre review to provide consensus on remote and centre use of lung function monitoring in the future.

Background: During the COVID-19 pandemic, people with cystic fibrosis (pwCF) were considered clinically vulnerable & asked to shield. A national scheme supported purchase of spirometers enabling pwCF to do home spirometry to support virtual clinics. PwCFattending the York Hull adult CF centre (YHACFC) were provided with NuvoAir spirometers. Objectives: To evaluate how the change from clinic to home spirometry impacted on the experiences of pwCF & clinicians, focusing on anxiety levels & use of time. Methods: Online questionnaires were sent to pwCF & clinicians at YHACFC. Mock scenarios established potential differences in time taken for home spirometry (HS) & clinic spirometry (CS). Results: 28/70 pwCF & 8/15 clinicians responded. PwCF & clinicians were confident that pwCF could use HS. Frequently used words for both groups were “quick,” “convenient” & “easy” when describing HS. When describing CS, “expertise” & “helpful” featured for pwCF and “time”, “stressful” & “accurate” for clinicians. PwCF reported the main benefit of CS was regular contact with clinicians. 62.5% of clinicians agreed that the change to HS gave better time efficiency & 87.5% agreed clinic flow was more efficient with HS. Timed scenarios indicated 7½ minutes per test saved with HS. Most clinicians believed pwCF are anxious with spirometry, 50% linking anxiety to the clinic setting. Most pwCF reported never being anxious about HS or CS. Only 4% reported anxiety with CS;however, when describing CS, the words “anxious” & “worry” were used frequently. The preference for 82% of pwCF for future care was access to a combination of spirometry in the home & clinic. Conclusion: • Home spirometry saved time for pwCF & clinicians. • Virtual technology has an important & developing role in the care of pwCF but relationships remain valued. • Style of questioning affects response from pwCF about spirometry anxiety. • Disparity between pwCF reported anxiety & clinicians’ perceptions of anxiety needs further investigation.

Background: CFDigiCare is a community of practice using digital adherence data, FEV1 and BMI to inform clinical decision-making and comprehensive digital behaviour change tools to support patient self-care. The 16 UK adult CF centres that form CFDigiCare work together using improvement science to embed digital data into routine care. The collaborative uses the Dartmouth Microsystems approach to build quality improvement (QI) capability Most CFDigiCare teams attended a one-day class-based QI training pre-COVID-19, with subsequent coaching delivered online. We discuss digital QI refresher training and coaching to the nationally dispersed improvement collaborative during the COVID crisis. Methods: 1-hour QI refresher training was delivered via MS Teams. Dartmouth improvement ramp activities were undertaken during subsequent 3 sessions. Results: Average attendance was 9. We explored two areas for improvement, teams participated in group ‘Fishbone’ exercises, silent brainstorming, discussed change ideas and planned PDSA cycles. Staff rated the meetings 9.4/10, commenting: • Useful QI demonstration and collaboration of ideas • Jamboard is a useful tool for gathering ideas • Can deliver it to the team • Good feedback for PDSAs Attendance was high with staff valuing practical interactive sessions using facilitated MS Teams calls and tools such as Google Jamboard. Virtual sessions connected clinicians from 16 centres providing a national “hive brain” perspective on system optimisation. The Dartmouth microsystems paradigm focused attention on specific aims, providing a structure to deliver iterative change. Virtual connectivity allowed QI to continue despite COVID-19. Familiarity with MS Teams increased over time with shared mastery of the technology a clear improvement outcome. Conclusion: Digital technology not only supports the delivery of QI training and coaching but can enhance it;clinical teams quickly mastered platforms such as MS Teams and Google Jamboard.

Diabetic MedicineVolume 37, Issue 7 p. 1205-1208 Letter Some young adults with cystic fibrosis-related diabetes may safely stop insulin without any adverse clinical sequelae C. Ogbolu, C. Ogbolu orcid.org/0000-0002-7960-9673 Human Development and Health, Faculty of Medicine, University of Southampton, Southampton, UKSearch for more papers by this authorI. Arregui-Fresneda, I. Arregui-Fresneda Southampton NHS Foundation Trust, Wessex Adult Cystic Fibrosis Service, Southampton, UKSearch for more papers by this authorT. Daniels, T. Daniels orcid.org/0000-0002-5249-5100 Southampton NHS Foundation Trust, Wessex Adult Cystic Fibrosis Service, Southampton, UKSearch for more papers by this authorR. I. G. Holt, R. I. G. Holt orcid.org/0000-0001-8911-6744 Human Development and Health, Faculty of Medicine, University of Southampton, Southampton, UKSearch for more papers by this author C. Ogbolu, C. Ogbolu orcid.org/0000-0002-7960-9673 Human Development and Health, Faculty of Medicine, University of Southampton, Southampton, UKSearch for more papers by this authorI. Arregui-Fresneda, I. Arregui-Fresneda Southampton NHS Foundation Trust, Wessex Adult Cystic Fibrosis Service, Southampton, UKSearch for more papers by this authorT. Daniels, T. Daniels orcid.org/0000-0002-5249-5100 Southampton NHS Foundation Trust, Wessex Adult Cystic Fibrosis Service, Southampton, UKSearch for more papers by this authorR. I. G. Holt, R. I. G. Holt orcid.org/0000-0001-8911-6744 Human Development and Health, Faculty of Medicine, University of Southampton, Southampton, UKSearch for more papers by this author First published: 01 March 2020 https://doi.org/10.1111/dme.14288Citations: 1Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat Citing Literature Volume37, Issue7July 2020Pages 1205-1208 RelatedInformation

Rationale: 25-hydroxyvitamin D (25OHD) exerts immunomodulatory effects on lung health1. Therefore, this study investigated if 25OHD is a predictor of lung function in people with Cystic Fibrosis (pwCF).

Airway clearance techniques (ACTs) are recommended in cystic fibrosis (CF) to prevent accumulation of secretions and lung infection. "Can exercise replace chest physiotherapy for people with CF?" is one of the CF community's top 10 research questions. We conducted an online survey of the CF community to gather data on current ACT use, recommendations, reported adherence levels and exercise strategies used. There were 488 respondents: 194 (40%) people with CF (pwCF), 141 (29%) family and 153 (31%) healthcare professionals (HCPs) (mostly physiotherapists). Only 10/285 (4%) of pwCF do no exercise at present and 163/303 (54%) already incorporate exercise into ACTs. ACTs were omitted by 128/267 (48%) of pwCF when they exercised. Nearly all (110/129, 93%) of HCPs currently recommend exercise to support ACTs. A trial replacing some or all ACTs with exercise, was supported by 80/110 (73%) of HCPs, with an additional 9/110 (8%) willing to consider in selected patients.

Objectives: Ventilatory parameters during CPET are important in people with cystic fibrosis (CF).They are associated with prognosis and help guide exercise prescriptions.CFRD is associated with worse prognosis and poorer lung function.However, little is known about how CF-related dysglycaemia affects ventilatory function during exercise.This study aimed to investigate ventilatory parameters during CPET across the dysglycaemic spectrum of CF.Methods: 76 people with CF (18 paediatric with normal glucose tolerance [NGT], 30 adults with NGT, 9 adults with impaired glucose tolerance [IGT] and 19 adults with CFRD) completed a combined ramp incremental and supramaximal verification cycle test to determine maximal O2 uptake (V O2max), ventilatory drive (ΔVE/ΔV CO2), breathing reserve (VE/MVV), peak ventilatory equivalents for O2 (VE/V O2peak) and CO2 (VE/V CO2peak), change in arterial O2 saturation (ΔSpO2) and peak dyspnoea.Results: V O2max relative to body mass was significantly different between groups ( p < 0.02, n2 > 0.13), with the paediatric NGT group having a significantly higher V O2max compared to adults with CFRD (+2.86 mLžkgžmin-1 , p < 0.05).A moderate effect size was observed between groups for V E/MVV ( p = 0.05, n2 > 0.10), but not ΔVE/ΔV CO2 ( p > 0.05, n2 < 0.01), V E/V O2peak ( p > 0.05, n2 = 0.01) or VE/V CO2peak ( p > 0.05, n2 = 0.05).There were significant negative and positive correlations between V E/MVV and ΔSpO2 (r = -0.31,p = 0.01), and V E/MVV and peak dyspnoea (adult data only; r = 0.34, p = 0.01) respectively. Conclusion:More adults with CFRD experienced ventilatory limitation during exhaustive CPET compared to their NGT peers.The implications of these observations are unknown.However, associations between V E/MVV, ΔSpO2, and dyspnoea at exhaustion may implicate ventilatory abnormalities as a potential barrier to exercise in people with CFRD and may need consideration when prescribing exercise.