NIV@Home: a pilot randomized controlled trial of in-home noninvasive ventilation initiation compared to a single-day admission model.

Objective: Noninvasive ventilation (NIV) is the primary treatment for respiratory insufficiency in neuromuscular disease. NIV implementation is usually conducted within hospitals; however, in-home implementation with intensive follow-up is an effective alternative. This pilot study aimed to assess model feasibility, acceptability, and NIV usage at 12-weeks after a single visit in-home implementation of NIV with remote monitoring follow-up (NIV@Home) compared to an in-hospital day admission NIV initiation plus planned polysomnography (Usual care). Methods: A single-blinded randomized controlled trial (www.anzctr.org.au ACTRN12620000682943) of adults with neuromuscular disease referred for NIV implementation. Participants were stratified by disease (MND or Other diagnoses) and bulbar symptoms before randomization to NIV@Home or Usual care, with follow-up at 12-weeks. The primary outcome was NIV usage. Secondary outcomes included feasibility, health-related quality of life, symptoms, carer burden, and NIV experience (semi-structured qualitative interviews). Results: Twenty-three participants (MND bulbar = 9, MND non-bulbar = 11, Other = 3) were randomized (NIV@Home = 9). No statistical differences were observed in the percentage of MND participants using NIV for >4 hours/day (NIV@Home = 33% vs. Usual care = 60%, p = 0.370), average use (NIV@Home = 2.4 [1.5-9.3] vs. 5.3 [1.8-7.0] hours/day, p = 0.568), or secondary outcomes. In-home NIV implementation was feasible and safe but took more therapist time (NIV@Home = 278 [270-305] vs. 172 [130-200] minutes, p < 0.001). Participants in the NIV@Home group reported substantial advantages to receiving care in home. Conclusion: In-home NIV implementation is feasible and acceptable to people with MND but requires more therapist time. Larger studies are required to determine whether there are clinically important differences between this model of NIV initiation and a traditional hospital-based model.

Effect of pulmonary rehabilitation on all-cause mortality in patients with chronic respiratory disease: a retrospective cohort study in an Australian teaching hospital.

BACKGROUND: Pulmonary rehabilitation (PR) is widely recommended for short-term benefits in chronic respiratory diseases, yet long-term outcomes remain uncertain. This retrospective cohort study addresses this gap, comparing 20-year mortality rates between PR participants and matched controls, and hypothesizing that the short-term benefits of PR contribute to improved long-term survival. METHODS: The 20-year mortality of stable chronic respiratory patients who participated in an outpatient PR program was compared with a matched control group based on the type of lung disease. Demographic and clinical variables, and the dates of deaths, were extracted and compared between two groups with two sample t-test and chi-square tests. Kaplan-Meier plots and Cox regression analyses were employed to evaluate survival differences. RESULTS: Between 2000 and 2002, 238 individuals enrolled in a pulmonary rehabilitation (PR) program (58% male, mean age ± SD: 69 ± 8 years, mean FEV1% predicted ± SD: 46 ± 21%). An equal number of people with comparable lung disease were selected as controls (88% COPD, 5% ILD). Controls had lower FEV1% predicted values (mean ± SD: 39 ± 17%, P < 0.001), smoked more (mean ± SD: 48 ± 35 pack-years, P = 0.032), and no differences in age, BMI, sex, and Index of Relative Socio-economic Advantage and Disadvantage (IRSAD). Median (IQR) follow-up time was 68 months (34-123), with 371 (78%) deaths. Univariable (HR = 1.71, p < 0.001) and multivariable (HR = 1.64, p < 0.001) Cox regression found higher mortality risk in controls. Subgroup analysis for COPD replicated these findings (HR = 1.70, P < 0.001). DISCUSSION: Despite some methodological limitations, our study suggests that clinically stable patients with chronic respiratory disease who undertake PR may have lower mortality than matched controls. TRIAL REGISTRATION: Retrospectively registered.

Ultrasound-derived rates of muscle wasting in the intensive care unit and in the post-intensive care ward for patients with critical illness: Post hoc analysis of an international, multicentre randomised controlled trial of early rehabilitation.

BACKGROUND AND AIMS: Muscle wasting results in weakness for patients with critical illness. We aim to explore ultrasound-derived rates of change in skeletal muscle in the intensive care unit (ICU) and following discharge to the post-ICU ward. DESIGN: Post hoc analysis of a multicentre randomised controlled trial of functional-electrical stimulated cycling, recumbent cycling, and usual care delivered in intensive care. METHOD: Participants underwent ultrasound assessment of rectus femoris at ICU admission, weekly in the ICU, upon awakening, ICU discharge, and hospital discharge. The primary outcome was rate of change in rectus femoris cross-sectional area (ΔRFCSA) in mm2/day in the ICU (enrolment to ICU discharge) and in the post-ICU ward (ICU discharge to hospital discharge). Secondary outcomes included rate of change in echo intensity (ΔEI), standard deviation of echo intensity (ΔEISD), and the intervention effect on ultrasound measures. Echo intensity is a quantitative assessment of muscle quality. Elevated echo intensity may indicate fluid infiltration, adipose tissue, and reduced muscle quality. RESULTS: 154 participants were included (mean age: 58 ± 15 years, 34% female). Rectus femoris cross-sectional area declined in the ICU (-4 mm2/day [95% confidence interval {CI}: -9 to 1]) and declined further in the ward (-9 mm2/day [95% CI: -14 to -3]) with a mean difference between ICU and ward of -5 mm2/day ([95% CI: -2, to 11]; p = 0.1396). There was a nonsignificant difference in ΔEI between in-ICU and the post-ICU ward of 1.2 ([95% CI: -0.1 to 2.6]; p = 0.0755), a statistically significant difference in ΔEISD between in-ICU and in the post-ICU ward of 1.0 ([95% CI, 0.5 to 1.5]; p = 0.0003), and no difference in rate of change in rectus femoris cross-sectional area between groups in intensive care (p = 0.411) or at hospital discharge (p = 0.1309). CONCLUSIONS: Muscle wasting occurs in critical illness throughout the hospital admission. The average rate of loss in muscle cross-sectional area does not slow after ICU discharge, even with active rehabilitation.

Breathing control training for functional seizures: A multi-site, open-label pilot study.

There are no well-validated treatments for functional seizures. While specialist psychotherapy is usually recommended, the evidence for its benefit is qualified, and it can be difficult to obtain. Given the association between hyperventilation and functional seizures we explored an alternative modality, breathing control training, in a multi-site open label pilot trial. Participants with functional seizures over the age of 16 received an hour of breathing training from a respiratory physiotherapist, with a half-hour booster session a month later. Seizure frequency and Nijmegen scores (a measure of hyperventilation) were reported at baseline and follow-up, 3-4 months later. Eighteen subjects were recruited, and 10 completed follow-up. Seven of these 10 had improved seizure frequency, and 3 did not (Wilcoxon signed rank test, p = 0.09), with seizure frequency correlating with Nijmegen score (Spearman's rank correlation = 0.75, p = 0.034). The intervention was well tolerated, with no adverse events reported. These preliminary results support a potentially new approach to treating functional seizures that should prove cost-effective and acceptable, though require confirmation by a randomised controlled trial.

Management of Sleep-Disordered Breathing in a Spinal Cord Injury Rehabilitation Center: Model of Care Adaptation and Implementation.

Background: Obstructive sleep apnea (OSA) is highly prevalent and poorly managed in spinal cord injury (SCI). Alternative management models are urgently needed to improve access to care. We previously described the unique models of three SCI rehabilitation centers that independently manage uncomplicated OSA. Objectives: The primary objective was to adapt and implement a similar rehabilitation-led model of managing OSA in an SCI rehabilitation center in Australia. Secondary objectives were to identify the local barriers to implementation and develop and deliver tailored interventions to address them. Methods: A clinical advisory group comprised of rehabilitation clinicians, external respiratory clinicians, and researchers adapted and developed the care model. A theory-informed needs analysis was performed to identify local barriers to implementation. Tailored behavior change interventions were developed to address the barriers and prepare the center for implementation. Results: Pathways for ambulatory assessments and treatments were developed, which included referral for specialist respiratory management of complicated cases. Roles were allocated to the team of rehabilitation doctors, physiotherapists, and nurses. The team initially lacked sufficient knowledge, skills, and confidence to deliver the OSA care model. To address this, comprehensive education and training were provided. Diagnostic and treatment equipment were acquired. The OSA care model was implemented in July 2022. Conclusion: This is the first time a rehabilitation-led model of managing OSA has been implemented in an SCI rehabilitation center in Australia. We describe a theory-informed method of adapting the model of care, assessing the barriers, and delivering interventions to overcome them. Results of the mixed-methods evaluation will be reported separately.

Understanding the Relationships Between Sleep Quality and Depression and Anxiety in Neurotrauma: A Scoping Review.

Sleep problems, depression, and anxiety are highly prevalent after a spinal cord injury (SCI) and traumatic brain injury (TBI) and may worsen functional outcomes and quality of life. This scoping review examined the existing literature to understand the relationships between sleep quality, depression, and anxiety in persons with SCI and TBI, and to identify gaps in the literature. A systematic search of seven databases was conducted. The findings of 30 eligible studies reporting associations between sleep quality and depression and/or anxiety after SCI or TBI were synthesized. The included studies were mostly cross-sectional and employed a range of subjective and objective measures of sleep quality. Poor subjective sleep quality and insomnia tended to be significantly associated with increased levels of depression and/or anxiety, but no such associations were reported when sleep quality was measured objectively. Two longitudinal studies observed worsening depressive symptoms over time were related to insomnia and persistent sleep complaints. Two interventional studies found that treating sleep problems improved symptoms of depression and anxiety. The findings of this review suggest that sleep and psychopathology are related in persons with neurotraumatic injuries. This has important therapeutic implications, because individuals may benefit from therapy targeting both sleep and psychological issues. More longitudinal and interventional studies are warranted to further understand the direction and strength of the relationships and how they impact patient outcomes.

Incidence and Risk Factors of Pneumonia in Individuals With Acute Spinal Cord Injury: A Multi-national, Multi-center, Prospective Cohort Study.

OBJECTIVE: To describe the occurrence of pneumonia in individuals with acute spinal cord injury (SCI) and identify its key predictors. DESIGN: Multi-centric, longitudinal cohort study. SETTING: 10 specialized SCI rehabilitation units in Europe and Australia. PARTICIPANTS: Eligible were 902 men and women with acute SCI, aged 18 years or older, with cervical or thoracic lesions and not dependent on 24-hour mechanical ventilation; 503 participated in the study (N=503). INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: We assessed demographics and lesion related parameters at study entry, and any pneumonia events throughout inpatient rehabilitation. Respiratory function, decubitus, and urinary tract infections were assessed at 1, 3, and 6 months post injury as well as at discharge from inpatient rehabilitation. Time to event (pneumonia) analyses were done using the Kaplan-Meier method, and potential predictors for pneumonia were analyzed with multivariable survival models. RESULTS: Five hundred three patients with SCI were included, with 70 experiencing at least 1 pneumonia event. 11 participants experienced 2 or more events during inpatient rehabilitation. Most events occurred very early after injury, with a median of 6 days. Pneumonia risk was associated with tetraplegia (hazard ratio [HR]=1.78; 95% confidence interval [CI] 1.00-3.17) and traumatic etiology (HR=3.75; 95% CI 1.30-10.8) American Spinal Injury Impairment Scale (AIS) A (HR=5.30; 95% CI 2.28-12.31), B (HR=4.38; 95% CI 1.77-10.83), or C (HR=4.09; 95% CI 1.71-9.81) lesions. For every 10 cmH2O increase in inspiratory muscle strength, pneumonia risk was reduced by 13% (HR=0.87; 95% CI 0.78-0.97). CONCLUSION: Pneumonia is a major complication after SCI with the highest incidence very early after injury. Individuals with traumatic or AIS A, B, or C tetraplegia are at highest risk for pneumonia.

The complexity of multidisciplinary respiratory care in amyotrophic lateral sclerosis.

Motor neurone disease/amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder with no known cure, where death is usually secondary to progressive respiratory failure. Assisting people with ALS through their disease journey is complex and supported by clinics that provide comprehensive multidisciplinary care (MDC). This review aims to apply both a respiratory and a complexity lens to the key roles and areas of practice within the MDC model in ALS. Models of noninvasive ventilation care, and considerations in the provision of palliative therapy, respiratory support, and speech and language therapy are discussed. The impact on people living with ALS of both inequitable funding models and the complexity of clinical care decisions are illustrated using case vignettes. Considerations of the impact of emerging antisense and gene modifying therapies on MDC challenges are also highlighted. The review seeks to illustrate how MDC members contribute to collective decision-making in ALS, how the sum of the parts is greater than any individual care component or health professional, and that the MDC per se adds value to the person living with ALS. Through this approach we hope to support clinicians to navigate the space between what are minimum, guideline-driven, standards of care and what excellent, person-centred ALS care that fully embraces complexity could be. Educational aims: To highlight the complexities surrounding respiratory care in ALS.To alert clinicians to the risk that complexity of ALS care may modify the effectiveness of any specific, evidence-based therapy for ALS.To describe the importance of person-centred care and shared decision-making in optimising care in ALS.

Prevalence of central sleep apnea in people with tetraplegic spinal cord injury: a retrospective analysis of research and clinical data.

STUDY OBJECTIVES: Over 80% of people with tetraplegia have sleep-disordered breathing (SDB), but whether this is predominantly obstructive or central is unclear. This study aimed to estimate the prevalence of central sleep apnea (CSA) in tetraplegia and the contributions of central, obstructive, and hypopnea respiratory events to SDB summary indices in tetraplegia. METHODS: Research and clinical data from 606 individuals with tetraplegia and full overnight polysomnography were collated. The proportions of different respiratory event types were calculated; overall and for mild, moderate, and severe disease. The prevalence of Predominant CSA (Central Apnea Index [CAI] ≥ 5 and more central than obstructive apneas) and Any CSA (CAI ≥ 5) was estimated. Prevalence of sleep-related hypoventilation (SRH) was estimated in a clinical sub-cohort. RESULTS: Respiratory events were primarily hypopneas (71%), followed by obstructive (23%), central (4%), and mixed apneas (2%). As severity increased, the relative contribution of hypopneas and central apneas decreased, while that of obstructive apneas increased. The prevalence of Predominant CSA and Any CSA were 4.3% (26/606) and 8.4% (51/606) respectively. Being male, on opiates and having a high tetraplegic spinal cord injury were associated with CSA. SRH was identified in 26% (26/113) of the clinical sub-cohort. CONCLUSIONS: This is the largest study to characterize SDB in tetraplegia. It provides strong evidence that obstructive sleep apnea is the predominant SDB type; 9-18 times more prevalent than CSA. The prevalence of CSA was estimated to be 4%-8%, significantly lower than previously reported.

The role of lung volume recruitment therapy in neuromuscular disease: a narrative review.

Respiratory muscle weakness results in substantial discomfort, disability, and ultimately death in many neuromuscular diseases. Respiratory system impairment manifests as shallow breathing, poor cough and associated difficulty clearing mucus, respiratory tract infections, hypoventilation, sleep-disordered breathing, and chronic ventilatory failure. Ventilatory support (i.e., non-invasive ventilation) is an established and key treatment for the latter. As survival outcomes improve for people living with many neuromuscular diseases, there is a shift towards more proactive and preventative chronic disease multidisciplinary care models that aim to manage symptoms, improve morbidity, and reduce mortality. Clinical care guidelines typically recommend therapies to improve cough effectiveness and mobilise mucus, with the aim of averting acute respiratory compromise or respiratory tract infections. Moreover, preventing recurrent infective episodes may prevent secondary parenchymal pathology and further lung function decline. Regular use of techniques that augment lung volume has similarly been recommended (volume recruitment). It has been speculated that enhancing lung inflation in people with respiratory muscle weakness when well may improve respiratory system "flexibility", mitigate restrictive chest wall disease, and slow lung volume decline. Unfortunately, clinical care guidelines are based largely on clinical rationale and consensus opinion rather than level A evidence. This narrative review outlines the physiological changes that occur in people with neuromuscular disease and how these changes impact on breathing, cough, and respiratory tract infections. The biological rationale for lung volume recruitment is provided, and the clinical trials that examine the immediate, short-term, and longer-term outcomes of lung volume recruitment in paediatric and adult neuromuscular diseases are presented and the results synthesised.

A Randomized Controlled Clinical Trial of Lung Volume Recruitment in Adults with Neuromuscular Disease.

Rationale: Clinical care guidelines advise that lung volume recruitment (LVR) be performed routinely by people with neuromuscular disease (NMD) to maintain lung and chest wall flexibility and slow lung function decline. However, the evidence base is limited, and no randomized controlled trials of regular LVR in adults have been published. Objectives: To evaluate the effect of regular LVR on respiratory function and quality of life in adults with NMD. Methods: A randomized controlled trial with assessor blinding was conducted between September 2015 and May 2019. People (>14 years old) with NMD and vital capacity <80% predicted were eligible, stratified by disease subgroup (amyotrophic lateral sclerosis/motor neuron disease or other NMDs), and randomized to 3 months of twice-daily LVR or breathing exercises. The primary outcome was change in maximum insufflation capacity (MIC) from baseline to 3 months, analyzed using a linear mixed model approach. Results: Seventy-six participants (47% woman; median age, 57 [31-68] years; mean baseline vital capacity, 40 ± 18% predicted) were randomized (LVR, n = 37). Seventy-three participants completed the study. There was a statistically significant difference in MIC between groups (linear model interaction effect P = 0.002, observed mean difference, 0.19 [0.00-0.39] L). MIC increased by 0.13 (0.01-0.25) L in the LVR group, predominantly within the first month. No interaction or treatment effects were observed in secondary outcomes of lung volumes, respiratory system compliance, and quality of life. No adverse events were reported. Conclusions: Regular LVR increased MIC in a sample of LVR-naive participants with NMD. We found no direct evidence that regular LVR modifies respiratory mechanics or slows the rate of lung volume decline. The implications of increasing MIC are unclear, and the change in MIC may represent practice. Prospective long-term clinical cohorts with comprehensive follow-up, objective LVR use, and clinically meaningful outcome data are needed. Clinical trial registered with anzctr.org.au (ACTRN12615000565549).

The impact of sleep quality on health, participation and employment outcomes in people with spinal cord injury: Analyses from a large cross-sectional survey.

BACKGROUND: Poor sleep is common in people with spinal cord injury (SCI), yet little is known about its impact on employment and participation outcomes. OBJECTIVES: This study aimed to (1) describe the sleep quality of a large sample of Australians with SCI and compare the results to data from an adult control and other clinical populations; (2) examine associations between sleep quality and participant characteristics; and (3) explore the relationship between sleep and outcomes. METHODS: Cross-sectional data from the Australian arm of the International Spinal Cord Injury (Aus-InSCI) survey from 1579 community-dwelling people aged >18 years with SCI were analysed. Sleep quality was assessed with the Pittsburgh Sleep Quality Index (PSQI). Relationships between participant characteristics, sleep quality and other outcomes were examined with linear and logistic regression. RESULTS: The PSQI was completed by 1172 individuals; 68% reported poor sleep (global PSQI score >5). Subjective sleep quality in people with SCI was poor (mean PSQI = 8.5, SD 4.5) when compared to adults without SCI (PSQI = 5.00, SD 3.37) and with traumatic brain injury (PSQI = 5.54, SD 3.94). Financial hardship and problems with secondary health conditions were significantly associated with worse sleep quality (p < 0.05). Poor sleep quality was strongly associated with lower emotional wellbeing and energy, and greater problems with participation (p < 0.001). Individuals engaged in paid work reported better sleep quality (mean PSQI = 8.1, SD 4.3) than unemployed individuals (mean PSQI = 8.7, SD 4.6; p < 0.05). Following adjustment for age, pre-injury employment, injury severity and years of education, better sleep quality remained strongly associated with being employed (OR 0.95, 95% CI 0.92 to 0.98; p = 0.003). CONCLUSIONS: This study demonstrated pervasive and impactful relationships between sleep quality and important SCI outcomes. Poor sleep quality was strongly associated with worse emotional wellbeing and vitality, unemployment and lower participation. Future studies should aim to determine whether treating sleep problems can improve outcomes for people living with SCI.

Barriers and enablers to telehealth use by physiotherapists during the COVID-19 pandemic.

OBJECTIVES: To investigate the perceived barriers and enablers experienced by physiotherapists whilst delivering community and outpatient services during the COVID-19 pandemic. METHODS: Qualitative study undertaken at a University-affiliated hospital in Melbourne, Australia. Physiotherapists working in the outpatient setting participated in a semi-structured interview. Interviews were analysed using Framework Analysis, with themes mapped to the Theoretical Domains Framework (TDF). RESULTS: From 19 interviews, we identified major themes and mapped these to the TDF domains: 1. The pandemic rapidly closed the knowledge-practice gap; 2. Adaptation of existing skills and integration of new skills were required; 3. Supportive senior leadership helped the transition; 4. Capabilities and confidence improved with time; 5. Environmental factors were crucial to success or failure of telehealth; 6. Access to and delivery of care improved for some; 7. Identification of appropriate patients and future hybrid models of care; 8. Changes in work practices, role certainty and identity; 9. Development of educational resources consolidated knowledge; 10. Socialisation of telehealth and optimism for the future. Within each domain, key barriers and enablers were also identified. CONCLUSIONS: The findings of this study suggest that the delivery of community and outpatient physiotherapy via telehealth during the COVID-19 pandemic was an initial challenge. Growing knowledge, confidence and ability to problem solve barriers enabled physiotherapists to move along the continuum of 'fear' to 'triumph' as the pandemic progressed. These results can be used by clinicians, managers, and academics to guide future workforce planning, hospital environmental design, and service delivery. CLINICAL MESSAGE.

Respiratory muscle training in neuromuscular disease: a systematic review and meta-analysis.

BACKGROUND: Neuromuscular disease causes a progressive decline in ventilatory function which respiratory muscle training may address. Previous systematic reviews have focussed on single diseases, whereas this study systematically reviewed the collective evidence for respiratory muscle training in children and adults with any neuromuscular disease. METHODS: Seven databases were searched for randomised controlled trials. Three reviewers independently reviewed eligibility, extracted characteristics, results, determined risk of bias and combined results using narrative synthesis and meta-analysis. RESULTS: 37 studies (40 publications from 1986-2021, n=951 participants) were included. Respiratory muscle training improved forced vital capacity (standardised mean difference (SMD) 0.40 (95% confidence interval 0.12-0.69)), maximal inspiratory (SMD 0.53 (0.21-0.85)) and maximal expiratory pressure (SMD 0.70 (0.35-1.04)) compared to control (usual care, sham or alternative treatment). No impact on cough, dyspnoea, voice, physical capacity or quality of life was detected. There was high degree of variability between studies. DISCUSSION: Study heterogeneity (children and adults, different diseases, interventions, dosage and comparators) suggests that the results should be interpreted with caution. Including all neuromuscular diseases increased the evidence pool and tested the intervention overall. CONCLUSIONS: Respiratory muscle training improves lung volumes and respiratory muscle strength in neuromuscular disease, but confidence is tempered by limitations in the underlying research.

Efficacy of Repeating Pulmonary Rehabilitation in People with COPD: A Systematic Review.

Background: Pulmonary rehabilitation is an effective intervention for people with chronic obstructive pulmonary disease (COPD). People with COPD undertake repeat programs, but synthesis of evidence regarding such practice has not been undertaken. The aim of this systematic review was to establish the effects of repeating pulmonary rehabilitation subsequent to an initial program in people with COPD. Methods: Studies where participants with COPD undertook >1 pulmonary rehabilitation program were included, incorporating RCT (randomized controlled trial) and non-randomized studies. Electronic database searches were undertaken. Two authors independently undertook study identification, data extraction and risk of bias assessment. The primary outcome was health-related quality of life (HRQoL); secondary outcomes were exercise capacity, hospitalizations and exacerbations, adherence, mortality and adverse events. Narrative synthesis was undertaken for clinically heterogeneous trials. Data from RCTs and non-randomized studies were not combined for analysis. Results: Ten included studies (2 RCTs) involved 907 participants with COPD (n=653 had undertaken >1 program). The majority of studies were at high risk of bias. One RCT (n=33) reported no difference in HRQol after a repeat program vs usual care following exacerbation (Chronic Respiratory Disease Questionnaire dyspnea domain score MD 0.4, 95% CI -0.5 to 3). In stable patients, clinically important and statistically significant improvements in HRQoL and exercise capacity were reported after repeat programs, but of a smaller magnitude than initial programs. There was evidence for reductions in exacerbations and hospitalizations, and shorter hospital length of stay for patients who repeated a program twice in 12 months compared to those who repeated once. No data for mortality or adverse events were available. Conclusion: This systematic review provides limited evidence for benefits of repeating pulmonary rehabilitation in people with COPD, including improved HRQoL and exercise capacity, and reduced hospitalizations. However, most studies have high risk of bias, which reduces the certainty of these conclusions. Study Registration: PROSPERO (CRD42020215093).

Routine lung volume recruitment in boys with Duchenne muscular dystrophy: a randomised clinical trial.

BACKGROUND: Impaired cough results in airway secretion retention, atelectasis and pneumonia in individuals with Duchenne muscular dystrophy (DMD). Lung volume recruitment (LVR) stacks breaths to inflate the lungs to greater volumes than spontaneous effort. LVR is recommended in DMD clinical care guidelines but is not well studied. We aimed to determine whether twice-daily LVR, compared with standard of care alone, attenuates the decline in FVC at 2 years in boys with DMD. METHODS: In this multicentre, assessor-blinded, randomised controlled trial, boys with DMD, aged 6-16 years with FVC >30% predicted, were randomised to receive conventional treatment or conventional treatment plus manual LVR twice daily for 2 years. The primary outcome was FVC % predicted at 2 years, adjusted for baseline FVC % predicted, age and ambulatory status. Secondary outcomes included change in chest wall distensibility (maximal insufflation capacity minus FVC) and peak cough flow. RESULTS: Sixty-six boys (36 in LVR group, 30 in control) were evaluated (median age (IQR): 11.5 years (9.5-13.5), median baseline FVC (IQR): 85% predicted (73-96)). Adjusted mean difference in FVC between groups at 2 years was 1.9% predicted (95% CI -6.9% to 10.7%; p=0.68) in the direction of treatment benefit. We found no differences in secondary outcomes. CONCLUSION: There was no difference in decline in FVC % predicted with use of twice-daily LVR for boys with DMD and relatively normal lung function. The burden associated with routine LVR may outweigh the benefit. Benefits of LVR to maintain lung health in boys with worse baseline lung function still need to be clarified. TRIAL REGISTRATION NUMBER: NCT01999075.

Tetraplegic obstructive sleep apnoea patients dilate the airway similarly to able-bodied obstructive sleep apnoea patients.

Context/objective: Obstructive sleep apnoea (OSA) develops soon after cervical spinal cord injury (SCI) at rates higher than the general population, but the mechanisms are not understood. This study aimed to determine whether OSA in SCI is associated with altered pharyngeal muscle dilatory mechanics during quiet breathing, as has been observed in the non-SCI injured with obstructive sleep apnoea.Design: Cross sectional imaging study.Setting: Medical research institute.Participants: Eight cervical SCI patients with OSA were recruited and compared to 13 able-bodied OSA patients and 12 able-bodied healthy controls of similar age and BMI.Interventions and outcome measures: 3T MRI scans of upper airway anatomy and tagged-MRI to characterize airway muscle motion during quiet breathing were collected for analysis.Results: Considerable variation in the patterns of inspiratory airway muscle motion was observed in the SCI group, with some participants exhibiting large inspiratory airway dilatory motions, and others exhibiting counterproductive narrowing during inspiration. These patterns were not dissimilar to those observed in the able-bodied OSA participants. The increase in airway cross-sectional area of able-bodied control participants was proportional to increase in BMI, and a similar, but not significant, relationship was present in all groups.Conclusion: Despite the limited sample size, these data suggest that SCI OSA patients have heterogeneous pharyngeal dilator muscle responses to the negative pressures occurring during inspiration but, as a group, appear to be more similar to able-bodied OSA patients than healthy controls of similar age and BMI. This may reflect altered pharyngeal pressure reflex responses in at least some people with SCI.

Rapidly and slowly progressive neuromuscular disease: differences in pulmonary function, respiratory tract infections and response to lung volume recruitment therapy (LVR).

INTRODUCTION: Reduced lung volumes are a hallmark of respiratory muscle weakness in neuromuscular disease (NMD). Low respiratory system compliance (Crs) may contribute to restriction and be amenable to lung volume recruitment (LVR) therapy. This study evaluated respiratory function and the immediate impact of LVR in rapidly progressive compared to slowly progressive NMD. METHODS: We compared vital capacity (VC), static lung volumes, maximal inspiratory and expiratory pressures (MIP, MEP), Crs and peak cough flow (PCF) in 80 adult participants with motor neuron disease ('MND'=27) and more slowly progressive NMDs ('other NMD'=53), pre and post a single session of LVR. Relationships between respiratory markers and a history of respiratory tract infections (RTI) were examined. RESULTS: Participants with other NMD had lower lung volumes and Crs but similar reduction in respiratory muscle strength compared with participants with MND (VC=1.30±0.77 vs 2.12±0.75 L, p<0.001; Crs=0.0331±0.0245 vs 0.0473±0.0241 L/cmH2O, p=0.024; MIP=39.8±21.3 vs 37.8±19.5 cmH2O). More participants with other NMD reported an RTI in the previous year (53% vs 22%, p=0.01). The likelihood of having a prior RTI was associated with baseline VC (%predicted) (OR=1.03 (95% CI 1.00 to 1.06), p=0.029). Published thresholds (VC<1.1 L or PCF<270 L/min) were, however, not associated with prior RTI.A single session of LVR improved Crs (mean (95% CI) increase = 0.0038 (0.0001 to 0.0075) L/cmH2O, p=0.047) but not VC. CONCLUSION: These findings corroborate the hypothesis that ventilatory restriction in NMD is related to weakness initially with respiratory system stiffness potentiating lung volume loss in slowly progressive disease. A single session of LVR can improve Crs. A randomised controlled trial of regular LVR is needed to assess longer-term effects.

Physical activity of patients with critical illness undergoing rehabilitation in intensive care and on the acute ward: An observational cohort study.

BACKGROUND: There are limited published data on physical activity of survivors of critical illness engaged in rehabilitation in hospital, despite it plausibly influencing outcome. OBJECTIVE: The aims of this study were to measure physical activity of patients with critical illness engaged in rehabilitation in the intensive care unit (ICU) and on the acute ward and report discharge destination, muscle strength, and functional outcomes. METHODS: This was a single-centre, prospective observational study. Adults with critical illness, who received ≥48 h of invasive mechanical ventilation, and who were awake and able to participate in rehabilitation were eligible. To record physical activity, participants wore BodyMedia SenseWear Armbands (BodyMedia Incorporated, USA), during daylight hours, from enrolment until hospital discharge or day 14 of ward stay (whichever occurred first). The primary outcome was time (minutes) spent performing physical activity at an intensity of greater than 1.5 Metabolic Equivalent Tasks. Secondary outcomes included discharge destination, muscle strength, and physical function. RESULTS: We collected 807 days of physical activity data (363 days ICU, 424 days ward) from 59 participants. Mean (standard deviation) duration of daily physical activity increased from the ICU, 17.8 (22.8) minutes, to the ward, 52.8 (51.2) minutes (mean difference [95% confidence interval] = 35 [23.8-46.1] minutes, P < .001). High levels of activity in the ICU were associated with higher levels of activity on the ward (r = .728), n = 48, P < .001. CONCLUSIONS: Patients recovering from critical illness spend less than 5% of the day being physically active throughout hospital admission, even when receiving rehabilitation. Physical activity increased after discharge from intensive care, but had no relationship with discharge destination. Only the absence of ICU-acquired weakness on awakening was associated with discharge directly home from the acute hospital. Future studies could target early identification of ICU-acquired weakness and the preservation of muscle strength to improve discharge outcomes.