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.
ABSTRACT
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.
Full text
Available for:
FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SAZU, SBCE, SBMB, UL, UM, UPUK
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.
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).
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.
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.
•Body composition in adults with cystic fibrosis who carry the G551 D mutation was tracked during ivacaftor treatment.•Weight and fat-free mass increased after 1 mo on ivacaftor, as well as weight on placebo.•Fat mass and further weight increases were seen after 6 mo of ivacaftor treatment.•Attenuation of weight and fat-mass gains were seen after a further 2 y of treatment.•More work is needed on the impact of cystic fibrosis transmembrane conductance regulator modulators on nutrition management in cystic fibrosis.
Full text
Available for:
GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
In cystic fibrosis (CF), a defect in ion transport results in thick and dehydrated airway mucus, which is difficult to clear, making such patients prone to chronic inflammation and bacterial ...infections. Physiotherapy using a variety of airway clearance techniques (ACTs) represents a key treatment regime by helping clear the airways of thickened, adhered, mucus and, thus, reducing the impact of lung infections and improving lung function. This article aims to bridge the gap between our understanding of the physiological effects of mechanical stresses elicited by ACTs on airway epithelia and the reported effectiveness of ACTs in CF patients. In the first part of this review, the effects of mechanical stress on airway epithelia are discussed in relation to changes in ion transport and stimulation in airway surface layer hydration. The second half is devoted to detailing the most commonly used ACTs to stimulate the removal of mucus from the airways of patients with CF.
To investigate the effects of a supervised longer- (14wk) versus shorter-duration (7wk) rehabilitation program after lung transplantation (LTX).
Randomized controlled trial.
Outpatient rehabilitation ...gym setting.
Post-LTX patients aged ≥18 years (N=66; 33 women; mean age, 51±13y) who had undergone either single LTX or bilateral LTX.
Outpatient rehabilitation program consisting of thrice-weekly sessions with cardiovascular training on bike ergometer and treadmill plus upper and lower limb strength training.
Measures were taken at baseline, 7 weeks, 14 weeks, and 6 months by assessors who were blinded to group allocation. Functional exercise capacity was measured by the 6-minute walk test (6MWT). Strength of quadriceps and hamstrings was measured on an isokinetic dynamometer and recorded as average peak torque of 6 repetitions for both muscles. Quality of life (QOL) was assessed with the Medical Outcomes Study 36-Item Short-Form Health Survey.
Of the participants, 86% had bilateral LTX and 41% had primary diagnosis of chronic obstructive pulmonary disease. The 6MWT increased in both groups with no significant difference between groups at any time point (mean 6mo 6MWD: short, 590±85m vs long, 568±127m; P=0.5). Similarly, at 6 months, there was no difference between groups in quadriceps average peak torque (mean, 115±38Nm vs 114±40Nm, respectively; P=.59), hamstring average peak torque (57±18Nm vs 52±19Nm, respectively; P=.36), or mental or physical health domains of quality of life.
Shorter duration (7wk) of rehabilitation achieves comparable outcomes with 14 weeks of supervised rehabilitation for functional exercise capacity, lower limb strength, and quality of life at 6 months after LTX.
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.
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.
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.
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.
ACTRN12617001009303, 13 July 13 2017.
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.
Full text
Available for:
BFBNIB, FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SAZU, SBCE, SBMB, UL, UM, UPUK
Abstract
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.
To investigate the effect of a supervised upper limb (UL) program (SULP) compared to no supervised UL program (NULP) after lung transplantation (LTx).
Randomized controlled trial.
Physiotherapy gym.
...Participants (N=80; mean age, 56±11y; 37 46% men) were recruited after LTx.
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.
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.
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.
UL rehabilitation results in short-term improvements in pain and muscle strength after LTx, but no longer-term effects were evident.
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.
Full text
Available for:
FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SAZU, SBCE, SBMB, UL, UM, UPUK
PDF
