Physiotherapy management is a key element of care for people with cystic fibrosis (CF) throughout the lifespan. Although considerable evidence exists to support physiotherapy management of CF, there is documented variation in practice. The aim of this guideline is to optimize the physiotherapy management of people with CF in Australia and New Zealand. A systematic review of the literature in key areas of physiotherapy practice for CF was undertaken. Recommendations were formulated based on National Health and Medical Research Council (Australia) guidelines and considered the quality, quantity and level of the evidence; the consistency of the body of evidence; the likely clinical impact; and applicability to physiotherapy practice in Australia and New Zealand. A total of 30 recommendations were made for airway clearance therapy, inhalation therapy, exercise assessment and training, musculoskeletal management, management of urinary incontinence, managing the newly diagnosed patient with CF, delivery of non‐invasive ventilation, and physiotherapy management before and after lung transplantation. These recommendations can be used to underpin the provision of evidence‐based physiotherapy care to people with CF in Australia and New Zealand.

Although problems with the hands are well recognized in SSc, foot problems also occur in the majority of patients, and can be a cause of major disability. Care of the feet is therefore an important part of the management of SSC.

Pulmonary exacerbation treatment aims to eradicate increased respiratory symptoms and recover acute loss in lung function. Current treatment strategies remain suboptimal, with conventional intravenous antibiotics and intensive physiotherapy failing to achieve this in 25% of patients [1]. Despite this worrying statistic, optimising recovery from acute pulmonary exacerbations has not been a focus of recent cystic fibrosis (CF) research efforts. There is a lack of adjunct evidence-based therapies for use in this setting [2] and strategies to optimise airway clearance with physiotherapy have been largely overlooked, despite common use in the outpatient setting [3,4]. Inhaled dry-powder mannitol (IDPM), a mucoactive agent, improves mucociliary clearance [5], mucus rheology, and hydration and surface properties of mucus [6]. In the CF outpatient setting, IDPM treatment improves lung function, both in the short (>2 weeks) and long (>12 months) term [3,7]. Its utility in in-patient pulmonary exacerbation care is unclear. In this pilot study, we investigated feasibility and safety of IDPM as an adjunct therapy to standard in-patient hospital care for children with pulmonary exacerbation. Efficacy was also explored using both conventional respiratory function outcomes and additional sensitive measures of peripheral airway function.A double-blind, randomised, placebo-controlled pilot study was conducted of twice daily IDPM (10×40 mg) or very low dose IDPM (10×5 mg nonrespirable mannitol, termed "control" hereafter) administered for 12 consecutive days. Eligibility criteria for recruitment were age ⩾6 years, admission to hospital for an infective pulmonary exacerbation (defined using Fuchs' criteria 4) and baseline forced expiratory volume in 1 s (FEV1) ⩾40% predicted (Global Lungs Initiative reference equations). Exclusion criteria included concurrent haemoptysis, hypertension, supplemental oxygen, oral corticosteroids, surgery or recent commencement of a mucolytic agent (within 3 months of admission). During hospital admission, subjects received combination i.v. antibiotic therapy (typically an aminoglycoside plus a β-lactam), daily airway clearance sessions and CF multidisciplinary team input, as clinically indicated.

BackgroundRegular 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.MethodsAdolescents 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.DiscussionThis 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 registrationClinical trial registered on July 13, 2017 with the Australian and New Zealand Clinical Trials Register at (ACTRN12617001009303).

BackgroundPhysical 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.MethodsMulticentre randomised controlled trial with assessor blinding and qualitative evaluation. People with CF (12–35 years) admitted to hospital for a respiratory cause were eligible and randomised to the 12-week ActivOnline intervention (AO) or usual care (UC). The primary outcome was change in device-based time spent in moderate-to-vigorous physical activity (MVPA) from baseline to post-intervention. Follow-up was at 6 months from hospital discharge when qualitative evaluation was undertaken.Results107 participants were randomised to AO (n=52) or UC (n=55). Sixty-three participants (59%) contributed to the intention-to-treat analysis. Mean (SD) age was 21 (6) years (n=46, <18 years). At baseline, physical activity levels were high in both groups (AO 102 (52) vs UC 127 (73) min/day). There was no statistically significant difference in MVPA between groups at either timepoint (post-intervention mean difference (95% CI) −14 mins (−45 to 16)). Uptake of the intervention was low with only 40% (n=21) of participants accessing the web application.ConclusionA web-based application, including individualised goal setting, real-time feedback and motivation for behavioural change, was no better than usual care at promoting physical activity in young pwCF following hospital discharge. High levels of baseline physical activity levels in both groups, and limited engagement with the intervention, suggest alternative strategies may be necessary to identify and support young pwCF who would benefit from enhanced physical activity.Trial registration numberACTRN12617001009303, 13 July 13 2017.