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. Primary outcomes were feasibility (recruitment and delivery of intensive intervention) and safety. Secondary outcomes included six-minute walk test; 60-second sit-to-stand; grip strength; physical activity; pain; EQ-5D-5L; length of stay; and readmissions. Data were collected at baseline, week 3, and week 10 post-LTx. ClinicalTrials.gov #NCT03095859. RESULTS: Of 83 LTx completed during the trial, 49% were eligible and 48% provided consent. Median age was 61 years {range 18-70}; waitlist time 85 days [IQR 35-187]. Median time to first mobilization was 2 days [2-3]. Both groups received a median of 10 [7-14] standard interventions post-randomization. A median of 9 [6-18] individual intensive interventions were attempted (86% successful), the most common barrier being medical procedures/investigations (67%). No intervention-related adverse events or between-group differences in secondary outcomes were observed. CONCLUSIONS: Acute, intensive physiotherapy was feasible and safe post-LTx. This trial provides data to underpin definitive trials to establish efficacy.

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. METHODS: Multicentre 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. RESULTS: 107 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. CONCLUSION: A 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 NUMBER: ACTRN12617001009303, 13 July 13 2017.

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. We used multiple linear regression to test whether demographics and/or lung function correlated with exercise capacity. RESULTS: A total of 49 participants: 38 adults (18 male), percent predicted (pp) forced expiration in one second (FEV1 ) 29-109, aged 22-48 years and 11 children (6 male), ppFEV1 68-107, aged 10-15 years were included. Levels reached (mean (SD) [range]) were 10.2 (2.4) [6-15] (adults), 10.1 (2.5) [7-14] (children); desaturation (change between baseline and peak-exercise SpO2 ): was 8.4 (3.8 [0-15]% (adults), 2.0 (2.0) [0-7]% (children). A total of 8 (21%) adults and no children desaturated <90% SpO2 . At least one criterion for maximal effort was reached by 33 (84%) adults and 10 (91%) children. There were no adverse events. The A-STEP was straightforward to use and carried out by one operator. A total of 26 (68.4%) adults and 7 (63.6%) children completed the test within the recommended 8-12 min. All participants completed a minimum of 6 levels, and completed the test before the final 16th level. In adults, ppFEV1 and ppFVC correlated with the level reached (r = 0.55; p = <0.001 and r = 0.66, p = <0.0001) and desaturation (r = 0.55, p = <0.001 and r = 0.45, p = <0.005). CONCLUSION: In adults and children with stable CF, the A-STEP was feasible, safe, and operationally easy to use for the assessment of exercise capacity, without floor or ceiling effects. In adults, lung function correlated with exercise capacity.

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. Bronchiectasis severity was assessed using the bronchiectasis severity index (BSI) and chest high-resolution computed tomography (HRCT). RESULTS: Sixty participants with bronchiectasis (mean [SD] forced expiratory volume in 1 s of 73.2 [25.5] %predicted) were included. Greater severity of chronic rhinosinusitis symptoms (based on SNOT-22) was moderately associated with impaired cough-related quality of life (according to the Leicester cough questionnaire; all r > -.60) and impaired bronchiectasis-specific quality of life (based on the quality of life-bronchiectasis questionnaire), with impaired physical function (r = -.518), less vitality (r = -.631), reduced social function (r = -.546), greater treatment burden (r = -.411), and increased severity of respiratory symptoms (r = -.534). Chronic rhinosinusitis symptoms were unrelated to disease severity according to the BSI (r = .135) and HRCT scoring (all r < .200). The severity of chronic rhinosinusitis symptoms was not affected by sputum color (p = .417) or the presence of Pseudomonas aeruginosa colonization (p = .73). CONCLUSIONS: In adults with bronchiectasis, chronic rhinosinusitis has a consistent and negative impact on both cough-related and bronchiectasis-specific quality of life.

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. METHODS: We evaluated the utility of a 10-item score (AWESCORE) by measuring reliability, validity and responsiveness in adults with CF. The questions were developed by thematic analysis of survey questions to patients in a single adult CF centre. Each question was scored using a numerical rating scale 0 to 10. Total scores ranged from 0 to 100. Test-retest reliability was assessed over 24 h. To determine validity, comparisons were sought between stable subjects and those in pulmonary exacerbation, and between AWESCORE and Cystic Fibrosis Questionnaire - Revised (CFQ-R). Responsiveness to pulmonary exacerbation in individual subjects was evaluated. RESULTS: Five domains, each with two questions, were identified for respiratory, physical, nutritional, psychological and general health. A total of 246 consecutive adults attending the outpatient clinic completed the AWESCORE. Scores were higher during clinical stability compared to pulmonary exacerbation (mean± sd): 73±11 versus 48±11 (p<0.001). Each domain scored worse during an acute exacerbation (p<0.001). No differences in reliability were observed in scores on retesting using Bland-Altman comparison. The CFQ-R scores (mean±sd: 813±125) and AWESCORE (81±13) were moderately correlated (Pearson's r=0.649; p=0.002). CONCLUSIONS: The AWESCORE is valid, reliable and responsive to altered health status in CF.

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. Subjects assisted in selecting: step height, start rate, increments, stage and test duration parameters. Equipment to externally pace and time the test and measure exercise parameters were selected. Reasons for stopping, criteria for achieving a maximal test, and key outcome measures were determined. Documentation to record and standardize the test and instructions to set up the metronome and timer App were developed. Infection control practices were considered. RESULTS: Eight subjects were recruited to develop the Alfred Step Test Exercise Protocol (A-STEP) on a 20 cm portable step. The A-STEP package included a pretest information sheet, clinical assessment and instructions, recording worksheet, and the metronome/timer instructions. The test started at 18 steps/min. Each level increased by two steps/min to a maximum of 48 steps (Level 16). Results were presented as mean (SD) [range] for: age 30.63 (5.89) [21-39] years; FEV1 58.13 (18.33) [32-89]%; levels: 10.31 (3.29) [6-15.5]. The A-STEP required space of 2 m2 and complied with current infection control guidelines. CONCLUSIONS: The A-STEP is a new incremental maximal step test to assess exercise capacity in pwCF, without floor or ceiling effects. It addresses the issues of space restrictions and the need for strict infection prevention in the clinical setting.

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 (FEV1) % pred >40%. We assessed the clinical utility of LUM/IVA in all eligible adult CF patients with FEV1 % pred <40% treated for at least 1 year under a single-centre managed access programme. METHODS: Following clinical optimisation, eligible patients (n=40) with FEV1 % pred <40% were commenced on LUM/IVA and monitored for tolerance and clinical outcomes, including health service utilisation, pulmonary function, weight and body composition. 24 patients reached 1 year of treatment by the time of evaluation. Six patients discontinued due to adverse events (five for increased airways reactivity) and three underwent lung transplantation. RESULTS: In comparison with the year prior to LUM/IVA commencement, significant reductions (median per year) were observed in the treatment year in the number of pulmonary exacerbations requiring hospitalisation (from 3 to 1.5; p=0.0002), hospitalisation days (from 27 to 17; p=0.0002) and intravenous antibiotic (IVAB) usage days (from 45 to 27; p=0.0007). Mean±sd change in FEV1 % pred was -2.10±1.18% per year in the year prior, with the decline reversed in the year following (+1.45±1.13% per year; p=0.035), although there was significant heterogeneity in individual responses. Mean±sd weight gain at 1 year was 2.5±4.1 kg (p=0.0007), comprising mainly fat mass (mean 2.2 kg). The proportion of patients severely underweight (body mass index <18.5 kg·m-2) decreased from 33% at baseline to 13% at 1 year (p=0.003). CONCLUSION: This real-world evaluation study demonstrated benefits over several clinical domains (infective exacerbations requiring hospitalisation, IVABs, pulmonary function decline and nutritional parameters) in CF patients with severe lung disease.

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 ± 4.3 kg/m2) were recruited for a single-center, double-blind, placebo-controlled, 28-d, crossover study of ivacaftor, followed by an open-label extension (OLE) for 5 mo. Eleven patients underwent measurements 2 y later. The study variables included weight, BMI, and body composition (including fat-free mass [FFM] and fat mass). RESULTS: After 28 d of treatment with ivacaftor, weight increased by 1.1 ± 1.3 kg, BMI by 0.4 ± 0.5 kg/m2, and FFM by 1.1 ± 1.2 kg (all P < .005) with no change in fat mass. Differences between 28-d changes on ivacaftor and placebo were not statistically significant. In the following 5 mo of the OLE, there were significant increases in weight (1.2 ± 1.9 kg; P < .05) and fat mass (1.5 ± 1.9 kg; P < .01), but not in FFM. Between baseline and the end of the OLE, the total weight gain was 2.5 ± 2.4 kg (P < .005), comprised of 0.9 ± 1.5 kg FFM (P < .05) and 1.6 ± 1.8 kg fat mass (P < .005). For the 11 participants who were followed for a further 2 y, no further changes in mean weight, BMI, or body composition parameters between 6 mo and 2 y later were observed. CONCLUSIONS: Small gains were seen in FFM in the first month of ivacaftor treatment. Weight, BMI, and fat-mass gains in the first 6 mo on ivacaftor plateaued by 2.5 y. The metabolic and clinical consequences of weight and fat-mass gains remain to be determined.

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. METHODS: This was a measurement study in 62 inpatients post-LTx (31 acute postoperative; 31 medical readmissions). Interrater reliability was assessed with 2 STS tests undertaken by different assessors at baseline. Known group validity was assessed by comparing STS repetitions in postoperative and medical groups. Content validity was assessed using comparisons to knee extensor and grip strength, measured with hand-held dynamometry. Criterion validity was assessed by comparison of STS repetitions and 6-minute walk distance postoperatively. Responsiveness was assessed using effect sizes over inpatient admission. RESULTS: Median (interquartile range) age was 62 (56-67) years; time post-LTx was 5 (5-7) days postoperative and 696 (244-1849) days for medical readmissions. Interrater reliability was excellent (intraclass correlation coefficient type 2,1 = 0.96), with a mean learning effect of 2 repetitions. Repetitions were greater for medical at baseline (mean 18 vs 8). More STS repetitions were associated with greater knee extensor strength (postoperative r = 0.57; medical r = 0.47) and 6-minute walk distance (postoperative r = 0.68). Effect sizes were 0.94 and 0.09, with a floor effect of 23% and 3% at baseline (postoperative/medical) improving to 10% at discharge. Patients incapable of attempting a STS test were excluded, reducing generalizability to critical care. Physical rehabilitation was not standardized, possibly reducing responsiveness. CONCLUSIONS: The 60-second STS demonstrated excellent interrater reliability and moderate validity and was responsive to change postoperatively. IMPACT: The 60-second STS represents a safe, feasible functional performance tool for inpatients post-LTx. Two tests should be completed at each time point.

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. The statement summarises current knowledge and proposes topics for future research, but does not make specific clinical recommendations.

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. 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. METHODS: Adolescents 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. DISCUSSION: This 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 REGISTRATION: Clinical trial registered on July 13, 2017 with the Australian and New Zealand Clinical Trials Register at (ACTRN12617001009303).

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. During invasive ventilation, NAC, dornase alfa and saline had no effect on mucus. Postoperatively, mucus characteristics improved with NAC (n = 10). Ambroxol lowered LOS (mean difference 4 days) and halved complications following lung carcinoma resection (n = 140). Heparin improved ventilator-free days (n = 130, mean difference 3.9-4.6) and intensive care LOS (n = 223, 3.2 days), but not ventilator-acquired pneumonia. CONCLUSION: Dornase alfa, hypertonic saline and NAC were ineffective for atelectasis/mucus plugging while intubated. More data are required to support using NAC, ambroxol and heparin during acute illness.

Accumulating physical activity in at least 10-minute bouts predicts better lung function after 3-years in adults with cystic fibrosis.

Achieving physical activity guidelines by undertaking multiple bouts of moderate-vigorous physical activity ≥10 min duration, but not shorter periods of activity, was independently associated with less decline in FEV1over 3 years among adults with CF http://ow.ly/yk6930ivCV8.

Effect of Upper Limb Rehabilitation Compared to No Upper Limb Rehabilitation in Lung Transplant Recipients: A Randomized Controlled Trial.

OBJECTIVE: To investigate the effect of a supervised upper limb (UL) program (SULP) compared to no supervised UL program (NULP) after lung transplantation (LTx). DESIGN: Randomized controlled trial. SETTING: Physiotherapy gym. PARTICIPANTS: Participants (N=80; mean age, 56±11y; 37 [46%] men) were recruited after LTx. INTERVENTIONS: All participants underwent lower limb strength thrice weekly and endurance training. Participants randomized to SULP completed progressive UL strength training program using handheld weights and adjustable pulley equipment. MAIN OUTCOME MEASURES: Overall bodily pain was rated on the visual analog scale. Shoulder flexion and abduction muscle strength were measured on a hand held dynamometer. Health related quality of life was measured with Medical Outcomes Study 36-item Short Form health Survey and the Quick Dash. Measurements were made at baseline, 6 weeks, 12 weeks, and 6 months by blinded assessors. RESULTS: After 6 weeks of training, participants in the SULP (n=41) had less overall bodily pain on the visual analog scale than did participants in the NULP (n=36) (mean VAS bodily pain score, 2.1±1.3cm vs 3.8±1.7cm; P<.001) as well as greater UL strength than did participants in the NULP (mean peak force, 8.4±4.0Nm vs 6.7±2.8Nm; P=.037). At 12 weeks, participants in the SULP better quality of life related to bodily pain (76±17 vs 66±26; P=.05), but at 6 months there were no differences between the groups in any outcome measures. No serious adverse events were reported. CONCLUSIONS: UL rehabilitation results in short-term improvements in pain and muscle strength after LTx, but no longer-term effects were evident.

Mucoactive agents for chronic, non-cystic fibrosis lung disease: A systematic review and meta-analysis.

Inhaled mucoactive agents are used in respiratory disease to improve mucus properties and enhance secretion clearance. The effect of mannitol, recombinant human deoxyribonuclease/dornase alfa (rhDNase) and hypertonic saline (HS) or normal saline (NS) are not well described in chronic lung conditions other than cystic fibrosis (CF). The aim of this review was to determine the benefit and safety of inhaled mucoactive agents outside of CF. We searched Medline, Embase, CINAHL and CENTRAL for randomized controlled trials investigating the effects of mucoactive agents on lung function, adverse events (AEs), health-related quality of life (HRQOL), hospitalization, length of stay, exacerbations, sputum clearance and inflammation. There were detrimental effects of rhDNase in bronchiectasis, with average declines of 1.9-4.3% in forced expiratory volume in 1 s (FEV1 ) and 3.7-5.4% in forced vital capacity (FVC) (n = 410, two studies), and increased exacerbation risk (relative risk = 1.35, 95% CI = 1.01-1.79 n = 349, one study). Some participants exhibited a reduction in FEV1 (≥10-15%) with mucoactive agents on screening (mannitol = 158 of 1051 participants, rhDNase = 2 of 30, HS = 3 of 80). Most AEs were mild and transient, including bronchospasm, cough and breathlessness. NS eased symptomatic burden in COPD, while NS and HS improved spirometry, HRQOL and sputum burden in non-CF bronchiectasis. Mannitol improved mucociliary clearance in asthma and bronchiectasis, while the effects of N-acetylcysteine were unclear. In chronic lung diseases outside CF, there are small benefits of mannitol, NS and HS. Adverse effects of rhDNase suggest this should not be administered in non-CF bronchiectasis.

Airway-Clearance Techniques in Children and Adolescents with Chronic Suppurative Lung Disease and Bronchiectasis.

Common symptoms of chronic suppurative lung disease or bronchiectasis in children and adolescents are chronic cough with sputum production, retention of excess secretions in dilated airways, and a history of recurrent infections. Clinical management includes the prescription of airway-clearance techniques (ACTs) to facilitate mucociliary clearance, optimize sputum expectoration, relieve symptoms, and improve well-being. A wide range of ACTs are available for selection, and these strategies may be applied in isolation or in combination. The choice of technique will depend in part on the age of the child, their clinical state, and factors which may influence treatment adherence. While the evidence base for ACTs in children and adolescent with these conditions is not robust, the current available evidence in addition to clinical expertise provides guidance for technique prescription and clinical effect. An overview of the most commonly applied ACTs, including their physiological rationale and discussion of factors influencing prescription in children and adolescents is outlined in this review.

Physiotherapy for cystic fibrosis in Australia and New Zealand: A clinical practice guideline.

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.

Physical activity participation by adults with cystic fibrosis: An observational study.

BACKGROUND AND OBJECTIVE: Studies in children with cystic fibrosis (CF) suggest greater physical activity (PA) is associated with a slower rate of decline in respiratory function. In adults with CF, objectively measured PA time and its relationship to long-term clinical outcomes of respiratory function and need for hospitalization are unknown. METHODS: PA measured objectively (SenseWear armband), pulmonary function, exercise capacity (Modified Shuttle Test-25) and CF-related quality of life (CFQ-R) were assessed in 65 adults (34 male; mean age 28 years) with CF during a stable phase. A sub-group of these participants undertook additional measurement of PA at hospital discharge for a respiratory exacerbation. RESULTS: Median daily habitual moderate-vigorous PA (MVPA) time was 31-min (IQR:15-53). Participants who accumulated ≥30-min MPVA daily experienced fewer hospital days (P = 0.04), better exercise capacity and higher FEV1 at 12 months (P ≤ 0.001). Daily, fewer females than males accrued ≥30-min MVPA (P = 0.02). Compared with those who did not, participants who accumulated 30-min MVPA in bouts ≥10-min (n = 21) recorded better FEV1 (P = 0.02) and exercise capacity (P = 0.006), and reduced hospital admissions (P = 0.04) and hospital days (P = 0.04) at 12 months. MVPA participation declined significantly 1 month post-hospital discharge (median 12 min (4-34); P = 0.04). CONCLUSION: Adults with CF are able to achieve recommended MVPA targets of 30mins/day; however, a significant gender difference in activity time is apparent. Greater time in MVPA is related to more positive clinical outcomes over 12 months. Whether increasing PA levels can improve clinical outcomes in adults with CF warrants further investigation. See Editorial, page 404.

Optimising inhaled mannitol for cystic fibrosis in an adult population.

ABSTRACT: There has been remarkable progress in the treatment of cystic fibrosis (CF) patients over the past 20 years. However, limitations of standard therapies have highlighted the need for a convenient alternative treatment to effectively target the pathophysiologic basis of CF-related disease by improving mucociliary clearance of airway secretions and consequently improve lung function and reduce respiratory exacerbations. Mannitol is an osmotic agent available as a dry powder, dispensed in a convenient disposable inhaler device for the treatment of adult patients with CF. Inhalation of mannitol as a dry powder is thought to change the viscoelastic properties of airway secretions, increase the hydration of the airway surface liquid and contribute to increased mucociliary and cough clearance of retained secretions. In two large phase 3 studies [1, 2], long-term use of inhaled mannitol resulted in a significant and clinically meaningful improvement in lung function relative to control in adult CF subjects and had an acceptable safety profile. Clinical experience with inhaled mannitol confirms that it is safe and effective. A minority of patients are unable to tolerate the medication. However, through training in proper inhaler technique and setting clear expectations regarding therapeutic effects, both the tolerance and adherence necessary for long term efficacy can be positively influenced. EDUCATIONAL AIMS: To discuss the importance of airway clearance treatments in the management of cystic fibrosis.To describe the clinical data that supports the use of mannitol in adult patients with cystic fibrosis.To highlight the role of mannitol tolerance testing in screening for hyperresponsiveness.To provide practical considerations for patient education in use of mannitol inhaler. KEY POINTS: Inhaled mannitol is a safe and effective option in adult patients with cystic fibrosis.Mannitol tolerance testing effectively screens for hyperresponsiveness prior to initiation of therapy.Physiotherapists and respiratory therapists play an integral role in the introduction and maintenance of dry powder inhalation therapy.Patient training and follow-up is important for optimising longer term adherence.

Prevalence and impact of urinary incontinence in men with cystic fibrosis.

OBJECTIVES: To determine the prevalence and impact of urinary incontinence (UI) in men with cystic fibrosis (CF). DESIGN: Prospective observational study. SETTING: Adult CF clinics at tertiary referral centres. PARTICIPANTS: Men with CF (n=80) and age-matched men without lung disease (n=80). INTERVENTIONS: Validated questionnaires to identify the prevalence and impact of UI. MAIN OUTCOME MEASURES: Prevalence of UI and relationship to disease specific factors, relationship of UI with anxiety and depression. RESULTS: The prevalence of UI was higher in men with CF (15%) compared to controls (10%) (p=0.339). Men with CF and UI had higher scores for anxiety than those without UI (mean 9.1 (SD 4.8) vs 4.7 (4.1), p=0.003), with similar findings for depression (6.8 (4.6) vs 2.8 (3.4), p=0.002) using the Hospital Anxiety and Depression Scale. CONCLUSIONS: Incontinence is more prevalent in adult men with CF than age matched controls, and may have an adverse effect on mental health. The mechanisms involved are still unclear and may differ from those reported in women.