Gastro-Oesophageal Reflux in Noncystic Fibrosis Bronchiectasis

The clinical presentation of noncystic fibrosis bronchiectasis may be complicated by concomitant conditions, including gastro-oesophageal reflux (GOR). Increased acidic GOR is principally caused by gastro-oesophageal junction incompetence and may arise from lower oesophageal sphincter hypotension, including transient relaxations, hiatus hernia, and oesophageal dysmotility. Specific pathophysiological features which are characteristic of respiratory diseases including coughing may further increase the risk of GOR in bronchiectasis. Reflux may impact on lung disease severity by two mechanisms, reflex bronchoconstriction and pulmonary microaspiration. Symptomatic and clinically silent reflux has been detected in bronchiectasis, with the prevalence of 26 to 75%. The cause and effect relationship has not been established, but preliminary reports suggest that GOR may influence the severity of bronchiectasis. Further studies examining the implications of GOR in this condition, including its effect across the disease spectrum using a combination of diagnostic tools, will clarify the clinical significance of this comorbidity

Guidance and standard operating procedures for functional exercise testing in cystic fibrosis

Regular exercise testing is recommended for all people with cystic fibrosis (PwCF). A range of validated tests, which integrate both strength and aerobic function, are available and increasingly being used. Together, these tests offer the ability for comprehensive exercise evaluation. Extensive research and expert consensus over recent years has enabled the adaptation and standardisation of a range of exercise tests to aid the understanding of the pathophysiology related to exercise limitation in PwCF and has led to the development of novel exercise tests which may be applied to PwCF. This article provides expert, opinion-based clinical practice guidance, along with test instructions, for a selection of commonly used valid tests which have documented clinimetric properties for PwCF. Importantly, this document also highlights previously used tests that are no longer suggested for PwCF and areas where research is mandated. This collaboration, on behalf of the European Cystic Fibrosis Society Exercise Working Group, represents expert consensus by a multidisciplinary panel of physiotherapists, exercise scientists and clinicians and aims to improve global standardisation of functional exercise testing of PwCF. In short, the standardised use of a small selection of tests performed to a high standard is advocated

Identifying Priorities for Physiotherapy Research in the UK: the James Lind Alliance Physiotherapy Priority Setting Partnership

Objectives To identify unanswered questions for physiotherapy research and help set and prioritise the top 10 generic research priorities for the UK physiotherapy profession; updating previous clinical condition- specific priorities to include patient and carer perspectives, and reflect changes in physiotherapy practice, service provision and new technologies. Design The James Lind Alliance (JLA) Priority Setting Partnership (PSP) methodology was adopted, utilising evidence review, survey and consensus methods. Participants Anyone with experience and/or an interest in UK physiotherapy: patients, carers, members of the public, physiotherapists, student physiotherapists, other healthcare professionals, researchers, educators, service providers, commissioners and policy makers. Results Five hundred and ten respondents (50% patients, carers or members of the public) identified 2152 questions (termed “uncertainties”). Sixty-five indicative questions were developed from the uncertainties using peer reviewed thematic analysis. These were ranked in a second national survey (1,020 responses (62% were complete)). The top 25 questions were reviewed in a final prioritisation workshop using an adapted nominal group technique. The top 10 research priorities focused on optimisation (top priority); access; effectiveness; patient and carer knowledge, experiences, needs and expectations; supporting patient engagement and self-management; diagnosis and prediction. Conclusions This study is currently the UK's most inclusive consultation exercise to identify patients‘and healthcare professionals‘priorities for physiotherapy research. The exercise deliberately sought to capture generic issues relevant to all specialisms within physiotherapy. The research priorities identified a range of gaps in existing evidence to inform physiotherapy policy and practice. The results will assist research commissioning bodies and inform funding decisions and strategy

Standards for the care of people with cystic fibrosis; establishing and maintaining health

This is the second in a series of four papers updating the European Cystic Fibrosis Society (ECFS) standards for the care of people with CF. This paper focuses on establishing and maintaining health. The guidance is produced using an evidence-based framework and with wide stakeholder engagement, including people from the CF community. Authors provided a narrative description of their topic and statements, which were more directive. These statements were reviewed by a Delphi exercise, achieving good levels of agreement from a wide group for all statements. This guidance reinforces the importance of a multi-disciplinary CF team, but also describes developing models of care including virtual consultations. The framework for health is reinforced, including the need for a physically active lifestyle and the strict avoidance of all recreational inhalations, including e-cigarettes. Progress with cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy is reviewed, including emerging adverse events and advice for dose reduction and interruption. This paper contains guidance that is pertinent to all people with CF regardless of age and eligibility for and access to modulator therapy.</p

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