The 2025 European Cystic Fibrosis Society position statement on physical activity assessment in cystic fibrosis.

Background: Recent advances in the measurement of physical activity have significantly enhanced the analyses and interpretation in relation to health and well-being. Thus, we sought to revise and expand the 2015 position statement on the measurement of physical activity and provide guidance to clinicians and researchers for measuring physical activity in cystic fibrosis (CF) clinical practice and research.

Methods: This study was registered with the International Prospective Register of Systematic Review (PROSPERO) database (CRD42022292165).

Nonpharmacological Interventions for Dysfunctional Breathing in Adults: A Systematic Review.

Background: Dysfunctional breathing is common and leads to worse asthma outcomes.

Objective: To describe the characteristics of nonpharmacological interventions to treat dysfunctional breathing, and evidence for their efficacy.

Methods: We searched electronic databases (MEDLINE, Embase, Cumulative Index to Nursing and Allied Health Literature, Cochrane Central Register of Controlled Trials, and Physiotherapy Evidence Database) to identify studies that involved nonpharmacological interventions for people with the diagnosis of dysfunctional breathing.

Preferences and perspectives regarding telehealth exercise interventions for adults with cystic fibrosis: A qualitative study.

Objective: Physical activity and exercise are key components in the management of cystic fibrosis (CF). Completing exercise programs online may minimize the risk of cross-infection and increase access for people with CF. This study aimed to understand the perspectives of people with CF regarding intervention content for a telehealth exercise program.

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.

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.

Steps Ahead: Optimising physical activity in adults with cystic fibrosis: A pilot randomised trial using wearable technology, goal setting and text message feedback.

Background: Regular participation in physical activity (PA) is encouraged for people with Cystic Fibrosis (CF). This study aimed to assess the effectiveness of an intervention using wearable technology, goal setting and text message feedback on PA and health outcomes in people with CF.

Methods: This was a pilot randomised trial conducted at University Hospital Limerick.

Web-based physical activity promotion in young people with CF: a randomised controlled trial.

Background: Physical activity levels are known to decline following hospitalisation for people with cystic fibrosis (pwCF). However, optimal physical activity promotion strategies are unclear. This study investigated the effect of a web-based application (ActivOnline) in promoting physical activity in young pwCF.

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 30 June and 1 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.

Feasibility of the A-STEP for the assessment of exercise capacity in people with cystic fibrosis.

Objectives: To evaluate feasibility of the Alfred Step Test Exercise Protocol (A-STEP) for the assessment of exercise capacity in adults and children with cystic fibrosis (CF); in adults to test whether demographics and/or lung function correlated with exercise capacity.

Methods: Adults and children with stable CF from two centres completed the A-STEP (a recently developed incremental maximal-effort step test). Feasibility was evaluated by: usefulness for exercise capacity assessment (measures of exercise capacity were: level reached, exercise-induced desaturation, and achievement of at least one maximal effort criteria); safety; operational factors; time to complete; floor and/or ceiling effects.

Post-operative, inpatient rehabilitation after lung transplant evaluation (PIRATE): A feasibility randomized controlled trial.

Background: Postoperative rehabilitation is crucial following lung transplantation (LTx); however, it is unclear whether intensive rehabilitation is feasible to deliver in the acute setting. We aimed to establish the feasibility and safety of intensive acute physiotherapy post-LTx.

Methods: This feasibility trial randomized 40 adults following bilateral sequential LTx to either standard (once-daily) or intensive (twice-daily) physiotherapy.

Physical Activity and Sedentary Behavior in Adults With Cystic Fibrosis: Association With Aerobic Capacity, Lung Function, Sleep, Well-Being, and Quality of Life.

Background: Physical activity (PA) and sedentary behavior (SB) have marked impact on key prognostic indicators such as aerobic capacity and lung function in people with cystic fibrosis (CF) and may have associations with sleep, well-being, and health-related quality of life (HRQOL).

Methods: This observational study assessed PA, SB, aerobic capacity, spirometry, sleep, well-being, and HRQOL in adults with CF at University Hospital Limerick. PA and SB were assessed using an accelerometer that was worn for 7 days.

The clinical impact of self-reported symptoms of chronic rhinosinusitis in people with bronchiectasis.

Background: Chronic rhinosinusitis affects 62% of adults with bronchiectasis and is linked to greater bronchiectasis severity. However, the impact of symptoms of chronic rhinosinusitis on disease-specific and cough-related quality of life is unknown.

Methods: In this cross-sectional study, adults with stable bronchiectasis and chronic rhinosinusitis symptoms completed the sinonasal outcome test-22 (SNOT-22), quality of life-bronchiectasis questionnaire, and Leicester cough questionnaire.

The AWESCORE, a patient-reported outcome measure: development, feasibility, reliability, validity and responsiveness for adults with cystic fibrosis.

Background: Quality of life has improved dramatically over the past two decades in people with cystic fibrosis (CF). Quantification has been enabled by patient-reported outcome measures (PROMs); however, many are lengthy and can be challenging to use in routine clinical practice. We propose a short-form PROM that correlates well with established quality-of-life measures.

Development of the A-STEP: A new incremental maximal exercise capacity step test in cystic fibrosis.

Background: Exercise testing is important in people with cystic fibrosis (pwCF). The aim was to develop an incremental maximal step test to assess exercise capacity across the range of pwCF, without floor or ceiling effects, within restrictions of space, and infection prevention.

Methods: The step test was developed in adults with stable CF.

Body composition and weight changes after ivacaftor treatment in adults with cystic fibrosis carrying the G551 D cystic fibrosis transmembrane conductance regulator mutation: A double-blind, placebo-controlled, randomized, crossover study with open-label extension.

Objectives: In patients with cystic fibrosis (CF) who carry the G551D mutation, treatment with ivacaftor improves lung function and weight; however, short- and long-term impacts on body composition have not been well studied.

Methods: Twenty adults with CF carrying the G551D mutation (mean ± standard deviation body mass index [BMI] 23.3 ± 4.

Lumacaftor/ivacaftor-associated health stabilisation in adults with severe cystic fibrosis.

Introduction: Lumacaftor/ivacaftor (LUM/IVA) has been shown to improve clinical outcomes in cystic fibrosis (CF) patients homozygous for Phe508del with forced expiratory volume in 1 s (FEV) % pred >40%. We assessed the clinical utility of LUM/IVA in all eligible adult CF patients with FEV % pred <40% treated for at least 1 year under a single-centre managed access programme.

Methods: Following clinical optimisation, eligible patients (n=40) with FEV % pred <40% were commenced on LUM/IVA and monitored for tolerance and clinical outcomes, including health service utilisation, pulmonary function, weight and body composition.

Steps Ahead: optimising physical activity in adults with cystic fibrosis: Study Protocol for a pilot randomised trial using wearable technology, goal setting and text message feedback.

Physical activity (PA) and exercise are widely documented as key components in the management of cystic fibrosis (CF). In recent years there have been significant improvements in telehealth, in particular; wearable technology,  smartphone use and remote monitoring, all of which may have potential to impact on PA in adults  with CF. The objective of this pilot randomised trial is to explore the effect of wearable technology, which is remotely monitored, combined with personalised text message feedback and goal setting, on PA in adults with CF.

The Utility of the Sit-to-Stand Test for Inpatients in the Acute Hospital Setting After Lung Transplantation.

Objective: Measurement of physical function is important to guide physical therapy for patients post-lung transplantation (LTx). The Sit-to-Stand (STS) test has proven utility in chronic disease, but psychometric properties post-LTx are unknown. The study aimed to assess reliability, validity, responsiveness, and feasibility of the 60-second STS post-LTx.

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.

Positive expiratory pressure physiotherapy for airway clearance in people with cystic fibrosis.

Background: Chest physiotherapy is widely prescribed to assist the clearance of airway secretions in people with cystic fibrosis (CF). Positive expiratory pressure (PEP) devices provide back pressure to the airways during expiration. This may improve clearance by building up gas behind mucus via collateral ventilation and by temporarily increasing functional residual capacity.

ERS/TSANZ Task Force Statement on the management of reproduction and pregnancy in women with airways diseases.

This European Respiratory Society/Thoracic Society of Australia and New Zealand statement outlines a review of the literature and expert opinion concerning the management of reproduction and pregnancy in women with airways diseases: asthma, cystic fibrosis (CF) and non-CF bronchiectasis. Many women with these diseases are now living into reproductive age, with some developing moderate-to-severe impairment of lung function in early adulthood. The statement covers aspects of fertility, management during pregnancy, effects of drugs, issues during delivery and the post-partum period, and patients' views about family planning, pregnancy and parenthood.

Childbearing concerns, information needs and preferences of women with cystic fibrosis: An online discussion group.

Objective: The life expectancy of women with cystic fibrosis (CF) has increased, enabling many to reach reproductive age. However, little is known about the childbearing concerns and information needs of women with CF or their preferences for information about the effect of their condition and its treatment on childbearing. The aim of this study was to identify the childbearing concerns and related information needs and preferences of women with CF.

Dornase alfa during lower respiratory tract infection post-lung transplantation: a randomized controlled trial.

Lung transplant (LTx) recipients are at risk of lower respiratory tract infection (LRTI), while altered physiology may lead to difficulty clearing sputum. Mucoactive agents alter sputum properties and facilitate mucociliary clearance; however, there are no randomized controlled trials (RCTs) studying this post-LTx. This RCT evaluated the safety and efficacy of nebulized dornase alfa during LRTI post-LTx.

Mucoactive agents for adults with acute lung conditions: A systematic review.

Background And Objectives: Inhaled mucoactive agents are used to enhance airway clearance, however efficacy and safety are unclear in adults with acute respiratory conditions.

Methods: We systematically reviewed randomized controlled trials assessing respiratory function; safety; length of stay (LOS); mucus; radiology; and oxygenation.

Results: No adverse events were reported for dornase alfa (n = 63), N-acetylcysteine (NAC, n = 50), ambroxol (n = 140), hypertonic saline (n = 33), heparin (n = 384), mannitol (n = 20) or isotonic saline.