Long-term survival following initiation of home non-invasive ventilation: a European study.

INTRODUCTION: Although home non-invasive ventilation (NIV) is increasingly used to manage patients with chronic ventilatory failure, there are limited data on the long-term outcome of these patients. Our aim was to report on home NIV populations and the long-term outcome from two European centres. METHODS: Cohort analysis including all patients established on home NIV from two European centres between 2008 and 2014. RESULTS: Home NIV was initiated in 1746 patients to treat chronic ventilatory failure caused by (1) obesity hypoventilation syndrome±obstructive sleep apnoea (OHS±OSA) (29.5%); (2) neuromuscular disease (NMD) (22.7%); and (3) obstructive airway diseases (OAD) (19.1%). Overall cohort median survival following NIV initiation was 6.6 years. Median survival varied by underlying aetiology of respiratory failure: rapidly progressive NMD 1.1 years, OAD 2.7 years, OHS±OSA >7 years and slowly progressive NMD >7 years. Multivariate analysis demonstrated higher mortality in patients with rapidly progressive NMD (HR 4.78, 95% CI 3.38 to 6.75), COPD (HR 2.25, 95% CI 1.64 to 3.10), age >60 years at initiation of home NIV (HR 2.41, 95% CI 1.92 to 3.02) and NIV initiation following an acute admission (HR 1.38, 95% CI 1.13 to 1.68). Factors associated with lower mortality were NIV adherence >4 hours per day (HR 0.64, 95% CI 0.51 to 0.79), OSA (HR 0.51, 95% CI 0.31 to 0.84) and female gender (HR 0.79, 95% CI 0.65 to 0.96). CONCLUSION: The mortality rate following initiation of home NIV is high but varies significantly according to underlying aetiology of respiratory failure. In patients with chronic respiratory failure, initiation of home NIV following an acute admission and low levels of NIV adherence are poor prognostic features and may be amenable to intervention.

BPAP is an effective second-line therapy for obese patients with OSA failing regular CPAP: A prospective observational cohort study.

BACKGROUND AND OBJECTIVE: Continuous positive airway pressure (CPAP) is the most common treatment for obstructive sleep apnoea (OSA), but many patients fail long-term therapy. Bilevel positive airway pressure (BPAP) is a potential alternative. We hypothesized that BPAP could improve treatment adherence and outcomes in patients who cannot tolerate CPAP. METHODS: Patients with OSA who failed CPAP (usage < 4 h/day) and were referred to a tertiary sleep centre between 2014 and 2017 for BPAP were included. Age, gender, body mass index (BMI), co-morbidities, CPAP use and reasons for failure, Epworth Sleepiness Scale (ESS), sleep study data, spirometry data and average maximum nightly compliance were recorded. RESULTS: A total of 52 patients with OSA requiring CPAP > 15 cm H2 O (71% male, age: 58 (15) years, BMI: 42.6 (10.1) kg/m2 , apnoea-hypopnoea index (AHI): 51.1 (30.4)/h) were studied; 62% had respiratory co-morbidities affecting nocturnal breathing including obesity hypoventilation syndrome and COPD; 25% had neuromuscular conditions; and 17% had cardiovascular disease. CPAP was used for 199 (106-477) days prior to referral for BPAP. Reasons for CPAP failure were intolerant pressures (23%), uncontrolled symptoms (23%), mask problems (21%), adverse effects (13%), claustrophobia (8%), co-morbidities (8%) and other issues (4%). Lower expiratory positive airway pressures were needed with BPAP compared to CPAP (10 (8-12) vs 16.8 (15.7-19.2) cm H2 O, P = 0.001); patients achieved better adherence to BPAP (7.0 (4.0-8.5) vs 2.5 (1.6-6.7) h/night, P = 0.028) and better symptom control (ESS: 4.0 (1.0-7.0) vs 10.0 (6.0-17.0) points, P = 0.039). CONCLUSION: In patients with moderate-severe OSA who fail CPAP therapy due to low adherence, BPAP is well tolerated and achieves sufficient control of sleep-disordered breathing and its symptoms.

Nutrition and Exercise Rehabilitation in Obesity hypoventilation syndrome (NERO): a pilot randomised controlled trial.

BACKGROUND: Respiratory management of obesity hypoventilation syndrome (OHS) focusses on the control of sleep-disordered breathing rather than the treatment of obesity. Currently, there are no data from randomised trials of weight loss targeted rehabilitation programmes for patients with OHS. INTERVENTION: A 3-month multimodal hybrid inpatient-outpatient motivation, exercise and nutrition rehabilitation programme, in addition to non invasive ventilation (NIV), would result in greater per cent weight loss compared with standard care. METHODS: A single-centre pilot randomised controlled trial allocated patients to either standard care or standard care plus rehabilitation. Primary outcome was per cent weight loss at 12 months with secondary exploratory outcomes of weight loss, exercise capacity and health-related quality of life (HRQOL) at the end of the rehabilitation programme to assess the intervention effect. RESULTS: Thirty-seven patients (11 male, 59.8±12.7 years) with a body mass index of 51.1±7.7 kg/m2 were randomised. At 12 months, there was no between-group difference in per cent weight loss (mean difference -5.9% (95% CI -14.4% to 2.7%; p=0.17)). At 3 months, there was a greater per cent weight loss (mean difference -5% (95% CI -8.3% to -1.4%; p=0.007)), increased exercise capacity (6 min walk test 60 m (95% CI 29.5 to 214.5) vs 20 m (95% CI 11.5 to 81.3); p=0.036) and HRQL (mean difference SF-36 general health score (10 (95% CI 5 to 21.3) vs 0 (95% CI -5 to 10); p=0.02)) in the rehabilitation group. CONCLUSION: In patients with OHS, a 3-month comprehensive rehabilitation programme, in addition to NIV, resulted in improved weight loss, exercise capacity and QOL at the end of the rehabilitation period, but these effects were not demonstrated at 12 months, in part, due to the limited retention of patients at 12 months. TRIAL REGISTRATION NUMBER: Pre-results; NCT01483716.

Parasternal electromyography to determine the relationship between patient-ventilator asynchrony and nocturnal gas exchange during home mechanical ventilation set-up.

INTRODUCTION: Patient-ventilator asynchrony (PVA) can adversely affect the successful initiation of non-invasive home mechanical ventilation (HMV). The aim of this observational study was to quantify the prevalence of PVA during initiation of HMV and to determine the relationship between PVA and nocturnal gas exchange. METHOD: Type and frequency of PVA were measured by surface parasternal intercostal muscle electromyography, thoracoabdominal plethysmography and mask pressure during initiation of HMV. Severe PVA was defined, as previously, as asynchrony affecting ≥10% of breaths. RESULTS: 28 patients (18 male) were enrolled aged 61±15 years and with a body mass index of 35±9 kg/m(2). Underlying diagnoses were neuromuscular disease with or without chest wall disease (n=6), obesity related chronic respiratory failure (n=12) and COPD (n=10). PVA was observed in all patients with 79% of patients demonstrating severe PVA. Triggering asynchrony was most frequent, observed in 24% (IQR: 11-36%) of breaths, with ineffective efforts accounting for 16% (IQR: 4-24%). PVA types were similar between disease groups, with the exception of auto-triggering, which was higher in patients with COPD (12% (IQR: 6-26%)). There was no correlation observed between PVA and time spent with oxygen saturations ≤90%, mean oxygen saturations or transcutaneous carbon dioxide levels during overnight ventilation. CONCLUSIONS: Severe PVA was identified in the majority of patients, irrespective of pathophysiological disease state. This was not associated with ineffective ventilation as evidenced by gas exchange.

Neural respiratory drive predicts clinical deterioration and safe discharge in exacerbations of COPD.

RATIONALE: Hospitalised patients with acute exacerbation of COPD may deteriorate despite treatment, with early readmission being common. OBJECTIVES: To investigate whether neural respiratory drive, measured using second intercostal space parasternal muscle electromyography (EMGpara), would identify worsening dyspnoea and physician-defined inpatient clinical deterioration, and predict early readmission. METHODS: Patients admitted to a single-site university hospital with exacerbation of COPD were enrolled. Spirometry, inspiratory capacity (IC), EMGpara, routine physiological parameters, modified early warning score (MEWS), modified Borg scale for dyspnoea and physician-defined episodes of deterioration were recorded daily until discharge. Readmissions at 14 and 28 days post discharge were recorded. MEASUREMENTS AND MAIN RESULTS: 120 patients were recruited (age 70 ± 9 years, forced expiratory volume in 1 s (FEV1) of 30.5 ± 11.2%). Worsening dyspnoea, defined as at least one-point increase in Borg scale, was associated with increases in EMGpara%max and MEWS, whereas an increase in EMGpara%max alone was associated with physician-defined inpatient clinical deterioration. Admission-to-discharge change (Δ) in the normalised value of EMGpara (ΔEMGpara%max) was inversely correlated with ΔFEV1 (r = -0.38, p < 0.001) and ΔIC (r = -0.44, p < 0.001). ΔEMGpara%max predicted 14-day readmission (OR 1.13, 95% 1.03 to 1.23) in the whole cohort and 28-day readmission in patients under 85 years (OR 1.09, 95% CI 1.01 to 1.18). Age (OR 1.08, 95% CI 1.03 to 1.14) and 12-month admission frequency (OR 1.29, 1.01 to 1.66), also predicted 28-day readmission in the whole cohort. CONCLUSIONS: Measurement of neural respiratory drive by EMGpara represents a novel physiological biomarker that may be helpful in detecting inpatient clinical deterioration and identifying the risk of early readmission among patients with exacerbations of COPD. TRIAL REGISTRATION: NCT01361451.

Current opinions on non-invasive ventilation as a treatment for chronic obstructive pulmonary disease.

PURPOSE OF REVIEW: This review examines the current reports, the evidence and the issues surrounding the use of non-invasive ventilation (NIV) for the treatment of chronic obstructive pulmonary disease (COPD) in both the acute and domiciliary setting. RECENT FINDINGS: With the increasing use of NIV, more recent studies have focused on investigating the outcomes of our current practice. Although overall morbidity and mortality outcomes in the acute setting have improved, patients who initially stabilize but then deteriorate during an acute exacerbation of COPD have a poor prognosis. The focus must be on phenotyping this high-risk group to investigate other potential rescue treatments, including extracorporeal carbon dioxide removal. Indeed, phenotyping appears to favour the obese COPD patient, which may have a protective role in reducing the risk of NIV failure and recurrent hospital admissions. Randomized controlled trial evidence to support the use of NIV in a domiciliary setting as a treatment for COPD is awaited, and until the data from a number of ongoing clinical trials are available, the wide variation in global practice will continue. Increased understanding of patient ventilator asynchrony has improved domiciliary NIV set up, which is expected to enhance the tolerability of NIV, promoting patient adherence. SUMMARY: NIV is the established standard of care to treat acute hypercapnic exacerbations of COPD postoptimal medical management. NIV as a long-term treatment for COPD remains controversial based on the evidence from the published randomized controlled trials. With increasing experience of NIV therapy, patient outcomes are improving; however, further work is still required to better characterize and target the patients who will most benefit from NIV.

Digital peer-to-peer support programme for informal caregivers of people living with motor neuron disease: study protocol for a multi-centre parallel group, single-blinded (outcome assessor) randomised controlled superiority trial.

BACKGROUND: Peer support is effective in improving psychological well-being of family caregivers of people with conditions such as dementia, cancer, and brain injury. However, there are limited data on effective psychological interventions for family caregivers of people living with motor neurone disease. Our objective is to evaluate the efficacy of a virtual peer support programme for improving caregiver psychological wellbeing and caregiving related outcomes. METHODS: We will conduct a multi-centre parallel group randomised controlled superiority trial. Using a multi-modal recruitment strategy, we will recruit informal caregivers from UK MND clinics, in-patient units, and hospices. We will randomise (1:1, stratified by gender) participants to either a 12-week virtual peer support programme or usual care comprising provision of online information resources publicly available via the MND Association website. Peer support programme elements will be delivered via a secure digital e-platform aTouchAway™ (Aetonix, Canada). Our target sample size is 160 (80 each arm). Our primary outcome is the Hospital Anxiety and Depression Scale (HADS) assessed at 12 weeks (primary endpoint). Secondary outcomes that will also be assessed at 12 weeks include the Zarit Burden Interview, Pearlin Mastery Scale, Personal Gain Scale, Positive Affect Scale, and the Brief COPE. Outcome assessors will be blinded to allocation. Tertiary outcomes include perceived usability (1 item 9-point Likert scale) and acceptability (semi-structured qualitative interviews) of the peer support programme. Intervention fidelity measures will comprise frequency, type (text, audio, video), and duration (audio and video) of peer support contact downloaded from the aTouchAway AWS server. We will use a mixed-effects linear model to test the effect of the intervention on the primary outcome. Secondary outcomes will be analysed using linear regression. We have ethical approval (21/NW/0269) from the North-West Research Ethics Committee, UK. DISCUSSION: This single-blinded randomised controlled trial will determine the effect of a virtual peer support programme on caregiver psychological wellbeing and caregiver burden. This study will examine the impact of a virtual peer support intervention on quality-of-life measures in informal caregivers of individuals with MND living in the community. TRIAL REGISTRATION: ClinicalTrials.gov: NCT04695210.

Tracheostomy ventilation in motor neurone disease: a snapshot of UK practice.

Introduction: Motor neurone disease (MND) is characterized by rapidly progressive motor neurone degeneration which leads to muscle wasting. Mortality and morbidity are due to respiratory muscle failure which may be offset by ventilation. The aim of this observational study was to quantify the number and characteristics of patients living with MND choosing tracheostomy ventilation (TV) in the UK. Methods: Long-term ventilation services in the UK were invited to undertake a retrospective 5-year audit of MND patients under their care between April 2013 and March 2018 who had TV. Patient characteristics, the time spent on ventilation, hospital length of stay, discharge destination, and survival data were collected. Results: Sixty-eight MND patients were initiated on TV over the 5-year period. Eighty-one percent of patients received TV in an emergency setting with more than a third of these undiagnosed at presentation. Patients choosing elective TV were more likely to be male (85%) have a bulbar presentation (54%) and an increased survival of 10 months over the observation period. The mean length of hospital stay post TV was 136 days. Two-thirds of patients were discharged to their own home. Conclusion: Very few patients living with MND in the UK are currently receiving TV. In those who choose TV, there may be a survival advantage to planning an elective procedure. Despite the long inpatient stay and high care costs involved a majority of patients survived and were discharged to their own home.

The impact of the COVID-19 pandemic on home mechanical ventilation services: A national survey.

The COVID-19 pandemic has caused major disruption to healthcare services globally. We present the findings of a national survey of home mechanical ventilation (HMV) services in England and Wales. 30 HMV services (60%) responded. There was a significant reduction in outpatient services with 93% of services not offering routine face-to-face appointments, although most centres were able to offer emergency appointments for ventilation review and set-up. HMV inpatient capacity was reassigned, and HMV service staff re-deployed in the majority of centres (97%). The initial wave of the COVID-19 pandemic left a service backlog of a median of 87 outpatient appointments [range 0-1500] and a median of 4 patients (range 0-100) awaiting NIV set-up.

Long-term adherence to home mechanical ventilation: a 10-year retrospective, single-centre cohort study.

BACKGROUND: Sleep-disordered breathing (SBD) can be associated with hypercapnic respiratory failure (HRF). Home Mechanical Ventilation (HMV) is the preferred long-term treatment for patients with chronic HRF. We reviewed the database of a large tertiary referral centre for HMV to study the long-term adherence to HMV in chronic hypercapnic patients. METHODS: Data on adherence and characteristics of patients who received HMV for the treatment of SDB were collected over a decade using electronic patient records. The primary outcome parameter in this study was annual non-adherence rate (patients with HMV usage of <4 hours/night in the service divided by the number of all new patients of the same year), secondary outcomes were patients' characteristics and reasons for low adherence. HMV adherence clinics were established to improve uptake. RESULTS: Two thousand and two hundred twenty-eight patients with HRF were under active follow-up on HMV at the end of the recording period. In contrast, a total of 1,900 patients had their HMV contracts terminated over the course of a decade (due to non-adherence, transfer to other services or death). Out of those, 222 patients {62 [52-72] years, body-mass index, BMI 40 [35-43] kg/m2, 58.1% male, Epworth Sleepiness Scale, ESS 9 [4-15] points, 4% oxygen desaturation index, 4%ODI 32 [20-71] × hour-1, TcCO2 6.6 [6.0-7.2] kPa} met the non-adherence criteria (nocturnal usage 0-4 hours). The annual non-adherence rate was 25.5% of all new setups in 2010, and declined to 3.4% in 2019 (relative reduction of 86%, P<0.001). Patients with Obstructive Sleep Apnoea/Obesity Hypoventilation Syndrome (58.2%), Neuromuscular Diseases (NMD) (26.8%) and COPD (13.6%) accounted for most cases of this non-adherent cohort. The vast majority of the patients (96.1%) were established on full-face masks. In 23.4% of patients, substantial weight loss (>10%) was the most common reason for low adherence; general displeasure (21.3%), uncontrolled symptoms (12.8%), claustrophobia (6.7%), mood (4.8%) and mask intolerance (4.3%) caused problems as well. CONCLUSIONS: Non-adherence to HMV in patients with chronic HRF can affect significant proportions of patients. However, the non-adherent rate substantially decreases when individual treatment solutions are offered in multi-disciplinary clinics.

Time-to-death in chronic respiratory failure on home mechanical ventilation: A cohort study.

BACKGROUND AND OBJECTIVE: Home mechanical ventilation (HMV) is used in heterogeneous conditions underlying chronic hypercapnic respiratory failure, but there are sparse data on long-term clinical outcomes. The aim was to systematically analyse the time and the circumstances of death on HMV. METHODS: All-cause mortality data of HMV patients were prospectively collected between 2008 and 2018 in a large tertiary centre. Data were categorised into diagnostic groups including neuromuscular disease (NMD), chest wall disease (CWD), chronic obstructive pulmonary disease (COPD), obesity hypoventilation syndrome (OHS), overlap syndrome of COPD and OSA (overlap) and other group. The primary outcome was time-to-death from initiation of HMV. RESULTS: 1210 deaths were recorded over a 10-year period. Median time-to-death was 19.5 [6-55] months and differed between groups (Kruskal Wallis p < 0.001). CWD (98.5 [23.5-120] months) and slowly progressive NMD (64.5 [28-120] months) had the longest time-to-death on HMV, while OHS (33 [13-75] months) and overlap syndrome (30.5 [14.5-68.5] months) had a longer median time-to-death than COPD (19.5 [7-42.5] months) and motor neurone disease (7 [3-14] months). Daily adherence to HMV of greater than 4 h/night was associated with better outcomes (10 [3-24] vs. 30 [10-76] months; p < 0.001). 43% with confirmed location of death died outside the hospital. CONCLUSIONS: The time-to-death on home mechanical ventilation varies widely across disease groups with chronic respiratory failure and seems to be associated with daily usage time. TRIAL REGISTRATION: researchregistry.com UIN: researchregistry4122.

Patient ventilator asynchrony and sleep disruption during non-invasive ventilation.

Patient-ventilator asynchrony (PVA) are a common phenomenon affecting all patients receiving long-term domiciliary ventilation. The interruption of synchrony between the patient and the ventilator has been reported to be a possible cause of nocturnal sleep disruption leaking to arousals, awakenings and reduced periods of stage 3 and REM sleep overnight. The cause of PVA is multi-factorial driven frequently by leak at the interface, changing respiratory breathing patterns and changes in sleep stages. It currently remains unclear as to whether the PVA is the cause of sleep fragmentation or if PVA is purely a marker of unsuccessful ventilatory control, patient discomfort and underlying sleep disruption.

The clinical usefulness of a self-administered questionnaire for sleep-disordered breathing in patients with neuromuscular disease.

BACKGROUND: Patients with neuromuscular disease (NMD) are at risk of developing sleep-disordered breathing (SDB) with hypercapnic respiratory failure. We hypothesised that a self-administered questionnaire (SiNQ-5 scores) may be useful to assess patients who are established on treatment for NMD with SDB. METHODS: Patients attending a tertiary referral centre filled in the SiNQ-5 (range 0-10 points, lower scores indicating fewer symptoms). The questionnaire contains five questions related to breathlessness, sleep and posture. Patients with NMD and treated SDB were compared to NMD without SDB, to sleep apnoea, chronic obstructive pulmonary disease (COPD) and heart failure (HF) patients' scores, as well as a group of patients without SDB. Results were compared using Kruskal-Wallis one-way analysis of variance, with Dunn/Bonferroni post-hoc tests if comparisons were found to be statistically significant. RESULTS: A total of 265 (156 male) patients completed the assessment, 40 had NMD with treated SDB [SiNQ-5 score 3.4 (3.0) points], 11 had NMD without SDB [2.7 (2.9) points], 120 patients had obstructive sleep apnoea (OSA) [4.1 (2.6) points], 16 had COPD [3.9 (3.0) points] and 9 had HF [3.2 (2.8) points], 69 patients had other conditions with no evidence of SDB [3.0 (2.4) points; P=0.077]. Patients with NMD without SDB and those with SDB who were on treatment did not differ in their responses (P=0.417). Question #1 allowed discrimination between patients with NMD with SDB [0.8 (0.8) points] and other disorders without respiratory involvement [0.3 (0.6) points; P=0.024]. CONCLUSIONS: The SiNQ-5 scores in neuromuscular patients with SDB who are established on treatment and NMD patients without SDB, as well as in patients with other conditions leading to SDB are similar.

Sociodemographic and behavioural factors associated with body mass index among men and women in Nairobi slums: AWI-Gen Project.

BACKGROUND: Body mass index (BMI) is rising globally with a faster increase in urban areas in low- and middle-income countries. It is critical to identify modifiable risk factors for BMI to prevent the occurrence of associated health consequences. OBJECTIVE: To investigate socio-demographic, behavioural and biological factors associated with BMI in Nairobi slums. METHODS: In 2014-2015, a cross sectional study of men and women aged 40-60 years in Nairobi slums (Korogocho and Viwandani) was conducted. Data were collected on socio-demographic, behavioural and biological characteristics. Mean BMI, overweight and obesity were computed. Hierarchical multiple linear regression analysis was conducted separately for men and women to explore factors associated with BMI. RESULTS: In total, 1942 study participants (54.4%, women) with a mean age (SD) of 48.3 (5.3) years and 48.8(5.6) years for women and men respectively were recruited. Mean BMI was higher among women than men (27.6 versus 22.8; p < 0.001). More women were overweight (30.9% versus 19.6%; p < 0.001) and obese (32.1% versus 5.1%; p < 0.001) than men. Among men, BMI was independently associated with wealth index, bread consumption and self-reported diabetes and was negatively associated with current tobacco smoking, HIV and TB infections. Among women, BMI was independently associated with wealth, current non-problematic drinking, and sedentary time, but was lower among other ethnicities compared to Kikuyu, among current smokers, women with longer sleep, and those with HIV infection and tuberculosis. Wealth index contributed the most variance in BMI among women and men (10.4%, 7.5%, respectively), but behavioural factors (7.4%) among men and biological factors (6.5%) among women accounted for most of the additional BMI variance. CONCLUSIONS: Adults aged 40-60 years in the urban slums of Nairobi have a high BMI associated with wealth. Bread consumption by men and sedentary life among women are the main risky behaviours that need urgent targeted interventions.

Hot Topics in Noninvasive Ventilation: Report of a Working Group at the International Symposium on Sleep-Disordered Breathing in Leuven, Belgium.

During the last few decades, attention has increasingly focused on noninvasive ventilation (NIV) in the treatment of chronic respiratory failure. The University of Leuven and the University Hospitals Leuven therefore chose this topic for a 2-day working group session during their International Symposium on Sleep-Disordered Breathing. Numerous European experts took part in this session and discussed (1) NIV in amyotrophic lateral sclerosis (when to start NIV, NIV and sleep, secretion management, and what to do when NIV fails), (2) recent insights in NIV and COPD (high-intensity NIV, NIV in addition to exercise training, and NIV during exercise training), (3) monitoring of NIV (monitoring devices, built-in ventilator software, leaks, and asynchronies) and identifying events during NIV; and (4) recent and future developments in NIV (target-volume NIV, electromyography-triggered NIV, and autoregulating algorithms).