Saline at lower tonicity in cystic fibrosis (SALTI-CF) trial comparing 0.9% versus 3% versus 6% nebulised saline.

BACKGROUND: In people with cystic fibrosis (CF), regular nebulisation of 6% or 7% saline improves lung function; however, these concentrations are not always tolerable. Clinically, some CF patients report using lower concentrations of saline to improve tolerability, yet the effects of lower concentrations are unknown. This study therefore aimed to evaluate the relative effectiveness and tolerability of 0.9% versus 3% versus 6% saline nebulised twice daily with an eFlow rapid nebuliser. METHODS: This was a randomised, blinded, placebo-controlled, parallel-group, multicentre study where subjects inhaled 4 mL of 0.9%, 3% or 6% saline twice daily for 16 weeks. The primary outcome was forced expiratory volume in 1 s. The secondary outcomes were: forced vital capacity (FVC) and forced expiratory flow at 25-75% of FVC; quality of life; exercise capacity; acquisition or loss of bacterial organisms in expectorated sputum; tolerability of nebulised saline; pulmonary exacerbations; and adverse events. RESULTS: 140 participants were randomised to 0.9% (n=47), 3% (n=48) or 6% (n=45) saline. 134 participants (96%) contributed to the intention-to-treat analysis. 3% saline significantly improved lung function and increased the time to first pulmonary exacerbation compared with 0.9% saline but did not improve quality of life. 6% saline had similar benefits to 3% saline but also significantly improved quality of life compared with 3% saline. Only 6% saline delayed the time to intravenous antibiotics for pulmonary exacerbation. Tolerability and adherence were similar. CONCLUSIONS: Dilution of 6% saline to 3% maintains the benefits for lung function and exacerbation prevention; however, the positive impacts of 6% saline on quality of life and time to i.v. antibiotics for pulmonary exacerbations are lost.

Elicitation interview study to identify salient beliefs about exercise participation in adults with cystic fibrosis.

OBJECTIVES: Conduct an elicitation study, using the Theory of Planned Behaviour framework, to identify salient beliefs about exercise participation in adults with cystic fibrosis (CF). Specifically, identify attitudes on advantages and disadvantages of exercise (behavioural beliefs); individuals and groups who apply social pressure to exercise (normative beliefs); and perceived control over facilitators and barriers to exercise (control beliefs) for adults with CF. DESIGN: Qualitative interviews using open-ended, structured questions. SETTING: Adult CF clinic in a large Australian hospital. PARTICIPANTS: Sixteen adults with CF, three relatives/friends of adults with CF and six CF clinic staff. RESULTS: The most common positive attitudes about exercise were to keep fit and healthy (68%) and feel better and happier (60%), and negative attitude was to feel breathless (36%). Social pressure to exercise mainly came from parents/family (72%) and friends (52%), and 60% of participants reported that no-one discourages exercise. Having someone to exercise with (44%) and be encouraged (36%) were the most common facilitators of perceived control to exercise, whereas being unwell (96%) and not having sufficient time (56%) were the most common barriers. CONCLUSIONS: Attitudes, social pressure and perceived control to exercise for adults with CF were similar to beliefs previously reported by the general population and some patient groups. A number of CF-specific exercise beliefs, mainly related to pulmonary function, were also reported. These findings can help develop questionnaires for larger groups of adults with CF, interpret relationships between exercise beliefs and participation, and inform clinicians to target interventions to increase exercise participation.

Management of Australian Adults with Bronchiectasis in Tertiary Care: Evidence-Based or Access-Driven?

PURPOSE: Australian data regarding the management of patients with bronchiectasis is scarce. We sought to compare the management of adults with bronchiectasis attending tertiary Australian centres with recent national and international guidelines. METHODS: The Australian Bronchiectasis Registry is a centralised database of patients with radiologically confirmed bronchiectasis unrelated to cystic fibrosis recruited from 14 tertiary Australian hospitals. We excluded children (<18 years) and those with incomplete data, leaving 589 adults for cross-sectional analyses. We compared the proportion of patients receiving certain therapies, as compared to the proportion eligible for those treatments according to the current guidelines and baseline clinical information available from the registry. RESULTS: Pulmonary rehabilitation was attended by 22%, although it was indicated in 67% of the cohort. Airway clearance was undertaken in 52% of patients, although 71% reported chronic productive cough. Sputum bacterial culture results were available for 59%, and mycobacterial culture results were available for 29% of the cohort. Inhaled antibiotics were used in half of potentially eligible patients. Despite guideline recommendations against routine use, inhaled corticosteroids were used in 48% of patients. Long-term macrolides were used in 28% of participants. CONCLUSIONS: Discrepancies exist between guideline recommendations and real-world treatment of bronchiectasis in Australia, even in tertiary centres. These findings suggest the need for increased patient referral to pulmonary rehabilitation, increased attention to airway clearance, increased collection of sputum samples (especially for mycobacterial culture) and rationalisation of inhaled corticosteroid use. These findings encourage a review of treatment access and will inform ongoing education to promote evidence-based care for people living with bronchiectasis.

Australian adults with bronchiectasis: The first report from the Australian Bronchiectasis Registry.

BACKGROUND: /objective: There are no large, multi-centre studies of Australians with bronchiectasis. The Australian Bronchiectasis Registry (ABR) was established in 2015 to create a longitudinal research platform. We aimed to describe the baseline characteristics of adult ABR participants and assess the impact of disease severity and exacerbation phenotype on quality of life (QoL). METHODS: The ABR is a centralised database of patients with radiologically confirmed bronchiectasis unrelated to cystic fibrosis. We analysed the baseline data of adult patients (≥18 years). RESULTS: From March 2016-August 2018, 799 adults were enrolled from 14 Australian sites. Baseline data were available for 589 adults predominantly from six tertiary centres (420 female, median age 71 years (interquartile range 64-77), 14% with chronic Pseudomonas aeruginosa infection). Most patients had moderate or severe disease based on the Bronchiectasis Severity Index (BSI) (84%) and FACED (59%) composite scores. Using Global Lung function Initiative-2012 reference equations, the majority of patients (48%) had normal spirometry; only 34% had airflow obstruction (FEV1/FVC < LLN). Disease severity scores (BSI and FACED) were negatively correlated with QoL-Bronchiectasis domain scores (rs between -0.09 and -0.58). The frequent exacerbator phenotype (≥3 in the preceding year) was identified in 23%; this group had lower scores in all QoL-B domains (p ≤ 0.001) and more hospitalisations (p < 0.001) than those with <3 exacerbations. CONCLUSIONS: The largest cohort of Australian adults with bronchiectasis has been described. Using contemporary criteria, most patients with bronchiectasis did not have airflow obstruction. The frequent exacerbation trait connotes poorer QoL and greater health-care utilisation.

Side lying during nebulisation can significantly improve apical deposition in healthy adults and adults with mild cystic fibrosis lung disease: a randomised crossover trial.

BACKGROUND: In people with and without Cystic Fibrosis (CF), does side lying during nebulisation change: the proportion of the dose loaded in the nebuliser that is deposited in the lungs; the uniformity of deposition throughout the lungs; or the apical drug density as a percentage of the drug density in the remaining lung? Do these effects differ depending on the degree of lung disease present? METHODS: A randomised crossover trial with concealed allocation, intention-to-treat analysis and blinded assessors, involving 39 adults: 13 healthy, 13 with mild CF lung disease (FEV1 > 80%pred), and 13 with more advanced CF lung disease (FEV1 < 80%pred). In random order, 4 mL of nebulised radioaerosol was inhaled in upright sitting and in alternate right and left side lying at 2-min intervals, for 20 min. RESULTS: Compared to sitting upright, lung deposition and the uniformity of deposition were not significantly altered by side lying in any of the three groups. In sitting, the density of the deposition was significantly less in the apical regions than in the rest of the lung in all participants. Side lying significantly improved apical deposition in healthy adults (MD, 13%; 95% CI, 7 to 19), and in minimal CF lung disease (MD, 4%; 95% CI, 1 to 7) but not in advanced disease (MD, 4%; 95% CI, - 2 to 9). CONCLUSION: Alternating between right and left side lying during nebulisation significantly improves apical deposition in healthy adults and in adults with mild CF lung disease, without substantial detriment to overall deposition. TRIAL REGISTRATION: ACTRN12611000674932 (Healthy), ACTRN12611000672954 (CF) Retrospectively registered 4/7/2011.

Non-invasive ventilation versus oxygen therapy in cystic fibrosis: A 12-month randomized trial.

BACKGROUND AND OBJECTIVE: No published studies have examined the long-term effects of non-invasive ventilation (NIV) in cystic fibrosis (CF). Our primary aim was to determine if adults with CF and sleep desaturation were less likely to develop hypercapnia with NIV ± O2 compared to low-flow oxygen therapy (LFO2 ) or meet the criteria for failure of therapy over 12 months. We studied event-free survival, hospitalizations, lung function, arterial blood gases (ABG), sleep quality and health-related quality of life. METHODS: A prospective, randomized, parallel group study in adult patients with CF and sleep desaturation was conducted, comparing 12 months of NIV ± O2 to LFO2 . Event-free survival was defined as participants without events. Events included: failure of therapy with PaCO2 > 60 mm Hg, or increase in PaCO2 > 10 mm Hg from baseline, increases in TcCO2 > 10 mm Hg, lung transplantation or death. Outcomes were measured at baseline, 3, 6 and 12 months, including lung function, ABG, Pittsburgh Sleep Quality Inventory (PSQI), SF36 and hospitalizations. RESULTS: A total of 29 patients were randomized to NIV ± O2 (n = 14) or LFO2 (n = 15) therapy for 12 months. Of the 29 patients, 18 met the criteria for event-free survival over 12 months. NIV ± O2 group had 33% (95% CI: 5-58%) and 46% (95% CI: 10-68%) more event-free survival at 3 and 12 months than LFO2 group. No statistically significant differences were seen in spirometry, ABG, questionnaires or hospitalizations. CONCLUSION: NIV ± O2 during sleep increases event-free survival over 12 months in adults with CF. Further studies are required to determine which subgroups benefit the most from NIV.

Effects of exercise and airway clearance (positive expiratory pressure) on mucus clearance in cystic fibrosis: a randomised crossover trial.

Exercise improves mucus clearance in people without lung disease and those with chronic bronchitis. No study has investigated exercise alone for mucus clearance in cystic fibrosis (CF). The aim of this study was to compare the effects of treadmill exercise to resting breathing and airway clearance with positive expiratory pressure (PEP) therapy on mucus clearance in adults with CF.This 3-day randomised, controlled, crossover trial included 14 adults with mild to severe CF lung disease (forced expiratory volume in 1 s % predicted 31-113%). Interventions were 20 min of resting breathing (control), treadmill exercise at 60% of the participant's peak oxygen consumption or PEP therapy (including huffing and coughing). Mucus clearance was measured using the radioaerosol technique and gamma camera imaging.Treadmill exercise improved whole lung mucus clearance compared to resting breathing (mean difference 3%, 95% CI 2-4); however, exercise alone was less effective than PEP therapy (mean difference -7%, 95% CI -6- -8). When comparing treadmill exercise to PEP therapy, there were no significant differences in mucus clearance from the intermediate and peripheral lung regions, but significantly less clearance from the central lung region (likely reflecting the huffing and coughing that was only in PEP therapy).It is recommended that huffing and coughing are included to maximise mucus clearance with exercise.

The use of an alternate side lying positioning strategy during inhalation therapy does not prolong nebulisation time in adults with Cystic Fibrosis: a randomised crossover trial.

BACKGROUND: Inhalation of nebulised medications is performed in upright sitting to maximise lung volumes. The pattern of deposition is poor for inhaled medications in people with Cystic Fibrosis. The pattern tends to be non-uniform and typically the upper lobes receive a reduced dose compared to the rest of the lung. One strategy that has been proposed as having the potential to improve homogeneity of deposition is to adopt an alternate side lying position for the inhalation procedure. This study sought to determine whether, among adults with Cystic Fibrosis, there is any disadvantage to delivery time of nebulised medications with a strategy of alternate side lying, compared to upright sitting. METHODS: A randomised crossover trial with concealed allocation, intention-to-treat analysis and blinded assessors was undertaken. The participants were 24 adults with stable Cystic Fibrosis. They inhaled 4 mL of normal saline via an LC Star™ nebuliser twice within 24 h. In random order, participants sat upright throughout nebulisation, or alternated between left and right side lying at each minute during the nebulisation period. The nebuliser was stopped and weighed each minute until the residual volume was reached. The primary outcome was the time required for 3.5 mL to be delivered. The secondary outcomes were: respiratory rate; ratio of the volume delivered on right and left sides; and calculation of how long the periods in side lying can be extended without causing greater than 20% discrepancy in dose delivered in the two positions. RESULTS: The delivery time did not significantly differ between sitting and side lying (mean difference 0.58 min, 95% confidence interval (CI) -1.40 to 0.24). There was no significant correlation between delivery time, lung function or subject height (all R2 < 0.4). Increasing side lying duration from 1 to 2 min did not significantly impact the dose delivered on each side. Turning each 3 min however, significantly worsened the disparity (mean ratio 1.32, 95% CI 1.24 to 1.40). CONCLUSION: Side lying during inhalation therapy does not prolong nebulisation time. 2-min periods should provide an equal dose in the two side lying positions. TRIAL REGISTRATION: Prospectively registered on 4 July 2011; ACTRN12611000672954 .

Hypoglycaemia in cystic fibrosis: An analysis of a single centre adult cystic fibrosis clinic.

BACKGROUND: Hypoglycaemia in cystic fibrosis (CF) is known to occur during oral glucose tolerance tests (OGTT) and continuous glucose monitoring, however demographic, clinical and mechanistic data are limited. The aims of this study were to review patient electronic medical records (EMR) in order to 1) describe patient characteristics of a university teaching hospital CF clinic, 2) determine the prevalence of hypoglycaemia on OGTT and explore associations with demographic and clinical characteristics, and 3) explore patient reported symptoms suggestive of hypoglycaemia documented in the EMR. METHODS: Adults who attended the RPA CF clinic between January 2009 to April 2016 were included in the study. The prevalence of hypoglycaemia on OGTT was determined and clinical and demographic data were compared to age, sex and glucose tolerance matched controls. Reported symptoms suggestive of hypoglycaemia documented in EMR were qualitatively explored. RESULTS: Hypoglycaemia on OGTT was prevalent in 25 (3 fasting and 22 reactive) of 169 patients who had an OGTT. They were heavier, less likely to have pancreatic insufficiency and had a lower insulin response at 2-h. Another 14 patients reported symptoms suggestive of hypoglycaemia in their EMR. No patient appropriately suppressed insulin at 2-h on OGTT. CONCLUSIONS: This study identified two potentially different presentations of hypoglycaemia occur in different clinic sub-populations. Knowledge gaps in the aetiology and triggers of hypoglycaemia remain.

Effects of treadmill exercise versus Flutter® on respiratory flow and sputum properties in adults with cystic fibrosis: a randomised, controlled, cross-over trial.

BACKGROUND: Treadmill exercise and airway clearance with the Flutter® device have previously been shown to improve mucus clearance mechanisms in people with cystic fibrosis (CF) but have not been compared. It is therefore not known if treadmill exercise is an adequate form of airway clearance that could replace established airway clearance techniques, such as the Flutter®. The aim of this study was to evaluate respiratory flow, sputum properties and subjective responses of treadmill exercise and Flutter® therapy, compared to resting breathing (control). METHODS: Twenty-four adults with mild to severe CF lung disease (FEV1 28-86% predicted) completed a three-day randomised, controlled, cross-over study. Interventions consisted of 20 min of resting breathing (control), treadmill exercise at 60% of the participant's peak oxygen consumption and Flutter® therapy. Respiratory flow was measured during the interventions. Sputum properties (solids content and mechanical impedance) and subjective responses (ease of expectoration and sense of chest congestion) were measured before, immediately after the interventions and after 20 min of recovery. RESULTS: Treadmill exercise and Flutter® resulted in similar significant increases in peak expiratory flow, but only Flutter® created an expiratory airflow bias (i.e. peak expiratory flow was at least 10% higher than peak inspiratory flow). Treadmill exercise and Flutter® therapy resulted in similar significant reductions in sputum mechanical impedance, but only treadmill exercise caused a transient increase in sputum hydration. Treadmill exercise improved ease of expectoration and Flutter® therapy improved subjective sense of chest congestion. CONCLUSIONS: A single bout of treadmill exercise and Flutter® therapy were equally effective in augmenting mucus clearance mechanisms in adults with CF. Only longer term studies, however, will determine if exercise alone is an adequate form of airway clearance therapy that could replace other airway clearance techniques. TRIAL REGISTRATION: Australian and New Zealand Clinical Trials Registry, Registration number # ACTRN12609000168257 , Retrospectively registered (Date submitted to registry 26/2/2009, First participant enrolled 27/2/2009, Date registered 6/4/2009).

A randomised trial of hypertonic saline during hospitalisation for exacerbation of cystic fibrosis.

BACKGROUND: The mucoactive effects of hypertonic saline should promote exacerbation resolution in people with cystic fibrosis (CF). OBJECTIVES: To determine the effects of hypertonic saline inhalation during hospitalisation for exacerbation of CF on length of stay, lung function, symptoms, oxygenation, exercise tolerance, quality of life, bacterial load and time to next hospitalisation. METHODS: 132 adults with an exacerbation of CF were randomised to inhale three nebulised doses a day of either 4 mL 7% saline or a taste-masked control of 0.12% saline, throughout the hospital admission. The primary outcome measure was length of hospital stay. RESULTS: All participants tolerated their allocated saline solution. There was no significant difference in length of stay, which was 12 days in the hypertonic saline group and 13 days in controls, with a mean between-group difference (MD) of 1 day (95% CI 0 to 2). The likelihood of regaining pre-exacerbation FEV1 by discharge was significantly higher in the hypertonic saline group (75% vs 57%), and the number needed to treat was 6 (95% CI 3 to 65). On a 0-100 scale, the hypertonic saline group had significantly greater reduction in symptom severity than the control group at discharge in sleep (MD=13, 95% CI 4 to 23), congestion (MD=10, 95% CI 3 to 18) and dyspnoea (MD=8, 95% CI 1 to 16). No significant difference in time to next hospitalisation for a pulmonary exacerbation was detected between groups (HR=0.86 (CI 0.57 to 1.30), p=0.13). Other outcomes did not significantly differ. CONCLUSIONS: Addition of hypertonic saline to the management of a CF exacerbation did not reduce the length of hospital stay. Hypertonic saline speeds the resolution of exacerbation symptoms and allows patients to leave hospital with greater symptom resolution. TRIAL REGISTRATION NUMBER: ACTRN12605000780651.

Physical activity in people with asbestos related pleural disease and dust-related interstitial lung disease: An observational study.

This study aimed to measure the levels of physical activity (PA) in people with dust-related pleural and interstitial lung diseases and to compare these levels of PA to a healthy population. There is limited data on PA in this patient population and no previous studies have compared PA in people with dust-related respiratory diseases to a healthy control group. Participants with a diagnosis of a dust-related respiratory disease including asbestosis and asbestos related pleural disease (ARPD) and a healthy age- and gender-matched population wore the SenseWear(®) Pro3 armband for 9 days. Six-minute walk distance, Medical Outcomes Study 36-item short-form health survey and the Hospital Anxiety and Depression Scale were also measured. Fifty participants were recruited and 46 completed the study; 22 with ARPD, 10 with dust-related interstitial lung disease (ILD) and 14 healthy age-matched participants. The mean (standard deviation) steps/day were 6097 (1939) steps/day for dust-related ILD, 9150 (3392) steps/day for ARPD and 10,630 (3465) steps/day for healthy participants. Compared with the healthy participants, dust-related ILD participants were significantly less active as measured by steps/day ((mean difference 4533 steps/day (95% confidence interval (CI): 1888-7178)) and energy expenditure, ((mean difference 512 calories (95% CI: 196-827)) and spent significantly less time engaging in moderate, vigorous or very vigorous activities (i.e. >3 metabolic equivalents; mean difference 1.2 hours/day (95% CI: 0.4-2.0)). There were no differences in levels of PA between healthy participants and those with ARPD. PA was reduced in people with dust-related ILD but not those with ARPD when compared with healthy age and gender-matched individuals.

Non-invasive ventilation used as an adjunct to airway clearance treatments improves lung function during an acute exacerbation of cystic fibrosis: a randomised trial.

QUESTION: During an acute exacerbation of cystic fibrosis, is non-invasive ventilation beneficial as an adjunct to the airway clearance regimen? DESIGN: Randomised controlled trial with concealed allocation and intention-to-treat analysis. PARTICIPANTS: Forty adults with moderate to severe cystic fibrosis lung disease and who were admitted to hospital for an acute exacerbation. INTERVENTION: Comprehensive inpatient care (control group) compared to the same care with the addition of non-invasive ventilation during airway clearance treatments from Day 2 of admission until discharge (experimental group). OUTCOME MEASURES: Lung function and subjective symptom severity were measured daily. Fatigue was measured at admission and discharge on the Schwartz Fatigue Scale from 7 (no fatigue) to 63 (worst fatigue) points. Quality of life and exercise capacity were also measured at admission and discharge. Length of admission and time to next hospital admission were recorded. RESULTS: Analysed as the primary outcome, the experimental group had a greater rate of improvement in forced expiratory volume in 1 second (FEV1) than the control group, but this was not statistically significant (MD 0.13% predicted per day, 95% CI -0.03 to 0.28). However, the experimental group had a significantly higher FEV1 at discharge than the control group (MD 4.2% predicted, 95% CI 0.1 to 8.3). The experimental group reported significantly lower levels of fatigue on the Schwartz fatigue scale at discharge than the control group (MD 6 points, 95% CI 1 to 11). There was no significant difference between the experimental and control groups in subjective symptom severity, quality of life, exercise capacity, length of hospital admission or time to next hospital admission. CONCLUSION: Among people hospitalised for an acute exacerbation of cystic fibrosis, the use of non-invasive ventilation as an adjunct to the airway clearance regimen significantly improves FEV1 and fatigue. TRIAL REGISTRATION: ANZCTR 12605000437662.

Exercise training for asbestos-related and other dust-related respiratory diseases: a randomised controlled trial.

BACKGROUND: The study aimed to determine the short and long-term effects of exercise training on exercise capacity and health-related quality of life (HRQoL) compared to usual care in people with dust-related pleural and interstitial respiratory diseases. No previous studies have specifically evaluated exercise training in this patient population. METHODS: Participants with a diagnosis of a dust-related respiratory disease including asbestosis and asbestos related pleural disease were recruited and randomised to an eight-week exercise training group (EG) or a control group (CG) of usual care. Six-minute walk distance (6MWD), St George's Respiratory Questionnaire (SGRQ) and Chronic Respiratory Disease Questionnaire (CRQ) were measured at baseline, eight weeks and 26 weeks by an assessor blinded to group allocation. RESULTS: Thirty-three of 35 male participants completed the study. Sixty-nine percent of participants had asbestos related pleural disease. At eight weeks, compared to the CG, the EG showed a significantly increased 6MWD (mean difference (95%CI)) 53 metres (32 to 74), improved SGRQ total score, -7 points (-13 to -1) and increased CRQ total score, 6.4 points (2.1 to 10.7). At 26 weeks significant between-group differences were maintained in 6MWD, 45 metres (17 to 73) and CRQ total score, 13.1 points (5.2 to 20.9). CONCLUSION: Exercise training improved short and long-term exercise capacity and HRQoL in people with dust-related pleural and interstitial respiratory diseases. CLINICAL TRIAL REGISTRATION NUMBER: ANZCTR12608000147381. Date trial registered: 27.03.2008.

Functional exercise capacity and health-related quality of life in people with asbestos related pleural disease: an observational study.

BACKGROUND: Functional exercise capacity in people with asbestos related pleural disease (ARPD) is unknown and there are no data on health-related quality of life (HRQoL). The primary aims were to determine whether functional exercise capacity and HRQoL were reduced in people with ARPD. The secondary aim was to determine whether functional exercise capacity was related to peak exercise capacity, HRQoL, physical activity or respiratory function. METHODS: In participants with ARPD, exercise capacity was measured by the six-minute walk test (6MWT) and incremental cycle test (ICT); HRQoL by the St George's Respiratory Questionnaire and physical activity by an activity monitor worn for one week. Participants also underwent lung function testing. RESULTS: 25 males completed the study with a mean (SD) age of 71 (6) years, FVC 82 (19)% predicted, FEV1/FVC 66 (11)%, TLC 80 (19)% predicted and DLCO 59 (13)% predicted. Participants had reduced exercise capacity demonstrated by six-minute walk distance (6MWD) of 76 (11)% predicted and peak work rate of 71 (21)% predicted. HRQoL was also reduced. The 6MWD correlated with peak work rate (r=0.58, p=0.002), St George's Respiratory Questionnaire Total score (r=-0.57, p=0.003), metabolic equivalents from the activity monitor (r=0.45, p<0.05), and FVC % predicted (r=0.52, p<0.01). CONCLUSIONS: People with ARPD have reduced exercise capacity and HRQoL. The 6MWT may be a useful surrogate measure of peak exercise capacity and physical activity levels in the absence of cardiopulmonary exercise testing and activity monitors. TRIAL REGISTRATION: ANZCTR12608000147381.

Shared Pseudomonas aeruginosa genotypes are common in Australian cystic fibrosis centres.

Recent molecular-typing studies suggest cross-infection as one of the potential acquisition pathways for Pseudomonas aeruginosa in patients with cystic fibrosis (CF). In Australia, there is only limited evidence of unrelated patients sharing indistinguishable P. aeruginosa strains. We therefore examined the point-prevalence, distribution, diversity and clinical impact of P. aeruginosa strains in Australian CF patients nationally. 983 patients attending 18 Australian CF centres provided 2887 sputum P. aeruginosa isolates for genotyping by enterobacterial repetitive intergenic consensus-PCR assays with confirmation by multilocus sequence typing. Demographic and clinical details were recorded for each participant. Overall, 610 (62%) patients harboured at least one of 38 shared genotypes. Most shared strains were in small patient clusters from a limited number of centres. However, the two predominant genotypes, AUST-01 and AUST-02, were widely dispersed, being detected in 220 (22%) and 173 (18%) patients attending 17 and 16 centres, respectively. AUST-01 was associated with significantly greater treatment requirements than unique P. aeruginosa strains. Multiple clusters of shared P. aeruginosa strains are common in Australian CF centres. At least one of the predominant and widespread genotypes is associated with increased healthcare utilisation. Longitudinal studies are now needed to determine the infection control implications of these findings.

Arm exercise training in chronic obstructive pulmonary disease: a randomised controlled trial.

The aim of this study was to compare the effects of arm endurance training, arm strength training, a combination of arm endurance and strength training, and no arm training on endurance arm exercise capacity. A randomised controlled trial was undertaken with chronic obstructive pulmonary disease subjects randomised into one of four groups to complete 8 weeks of training: (a) arm endurance training (endurance group) consisting of supported and unsupported arm exercises, (b) arm strength training (strength group) using weight machines, (c) a combination of arm endurance and arm strength training (combined group), or (d) no arm training (control group). The primary outcome measurement was endurance arm exercise capacity measured by an endurance arm crank test. Secondary outcomes included functional arm exercise capacity measured by the incremental unsupported arm exercise test and health-related quality of life. A total of 52 subjects were recruited and 38 (73%) completed the study. When comparing the arm endurance group to the control group, there was a significant increase in endurance time of 6 min (95% CI 2-10, p < 0.01) following the interventions. When comparing the combined group to each of the control, endurance and strength groups, there was a significantly greater reduction in dyspnoea and rate of perceived exertion at the end of the functional arm exercise test for the combined group following the interventions. The mode of training to be favoured to increase endurance arm exercise capacity is arm endurance training. However, combined arm endurance and strength training may also be very useful to reduce the symptoms during everyday arm tasks.

Adults with cystic fibrosis prefer hypertonic saline before or during airway clearance techniques: a randomised crossover trial.

QUESTION: Among adults with cystic fibrosis, does the timing of hypertonic saline relative to airway clearance techniques affect lung function, perceived efficacy, tolerability, or satisfaction with the entire airway clearance regimen, and is the preferred timing regimen stable over time? DESIGN: A randomised crossover trial with concealed allocation, intention-to-treat analysis, and blinded assessors. PARTICIPANTS: 50 adults with cystic fibrosis and stable lung function at the end of a hospital admission. INTERVENTION: Participants performed 3 sessions of airway clearance techniques per day for 3 days. On each day, participants were randomised to inhale hypertonic saline either before, during, or after the airway clearance techniques. Participants readmitted within one year repeated the 3-day study. OUTCOME MEASURES: The primary outcome was the change in forced expiratory volume in one second (FEV(1)) from before to 2 hours after an entire airway clearance session. Secondary outcomes were change in forced vital capacity, perceived efficacy, tolerability, satisfaction, adverse events, and adherence. RESULTS: All 50 participants completed the study. The effects on lung function were non-significant or were of borderline statistical significance favouring inhalation of hypertonic saline before airway clearance techniques. Satisfaction was rated significantly worse on a 100mm scale when hypertonic saline was inhaled after the airway clearance techniques: mean differences 20mm (95% CI 12 to 29) compared to before the airway clearance techniques and 15 mm (95% CI 6 to 24) compared to during the techniques. Perceived effectiveness showed similar effects but other outcomes were unaffected. All 14 participants who were readmitted repeated the study and most preferred the same timing regimen. Conclusion People with cystic fibrosis could be encouraged to time hypertonic saline before or during airway clearance techniques to maximise perceived efficacy and satisfaction, even though lung function may not be better with these timing regimens. TRIAL REGISTRATION: ACTRN12611000673943.

Cystic fibrosis in Australia, 2009: results from a data registry.

OBJECTIVES: To describe the demographics, clinical features and outcomes among people with cystic fibrosis (CF) in Australia and to estimate incidence of the disease. DESIGN AND SETTING: Cross-sectional analysis using data from the Australian Cystic Fibrosis Data Registry for 2009. MAIN OUTCOME MEASURES: Numbers of diagnoses, pulmonary and anthropometric measurements, microbiological culture results, rates of hospitalisation and transplantation, and numbers of medical complications and deaths. RESULTS: In 2009, data were submitted on 2986 people (48% female). Median age was 17.6 years and 49% of people were aged 18 years or over. Seventy-eight people were newly diagnosed. Fourteen people died and 14 people underwent lung transplantation in the year. Lung function and nutrition were relatively normal among children but deteriorated (more rapidly) among adolescents. With increasing age, progressive respiratory disease was apparent, and the frequency of CF-related complications and use of health care resources increased. In all age groups, there was a wide range in severity of lung disease and nutritional status. CONCLUSIONS: CF remains a progressive respiratory disease and is associated with multisystem complications. The acceleration in disease severity in adolescence and early adulthood suggests that better treatment at these stages is required to further improve survival.