Non-invasive Ventilation Adherence Research Articles

Factors Associated With Change in S3-NIV Score Over Time in People With Chronic Respiratory Failure Treated With Long-Term Home Noninvasive Ventilation

IntroductionMonitoring changes in symptoms over time during long-term nocturnal home non-invasive ventilation (NIV) using patient-reported outcome measures is crucial. This study aimed to identify factors associated with changes in the S3-NIV total score, its two domains (“respiratory symptoms” and “sleep and NIV-related side effects”) and individual item responses. MethodsWe conducted a retrospective, longitudinal data analysis of a cohort of adults with chronic respiratory failure treated with NIV. Data were obtained from a French homecare provider. Multivariate linear and multinomial ordinal mixed effect models were used to identify factors associated with changes in S3-NIV scores over time. ResultsMedian follow-up was 2 years for 2135 participants. Each participant completed a median of five S3-NIV questionnaires; totaling 11,359 analyzed questionnaires. Type of respiratory condition, sex, age and time since NIV initiation were associated with change in S3-NIV score over time. NIV adherence was not associated with total S3-NIV score but high adherence was associated with more severe respiratory symptoms and an improvement in sleep and NIV-related side effects during the follow-up. Intensity of pressure support was associated with a lower total S3-NIV score and more side effects. Face masks and supplemental oxygen were associated with a lower S3-NIV total score. ConclusionChanges in S3-NIV scores over time are associated with the individual's characteristics and NIV settings. Analysis of the two domains and individual items of the S3-NIV could increase understanding of the difficulties experienced by people on NIV.

P042 Lean Six Sigma Approach to Improve Non-Invasive Ventilation Patient Care

Abstract Introduction A lean six-sigma (LSS) approach to clinical redesign looks at aspects of patient flow, value to consumer and process inefficiencies. We used LSS quality management approach for clinical redesign of our non-invasive ventilation patient care. Current recommendations suggest that patients using non-invasive ventilation (NIV) require therapy for a minimum of 4 hours per night for optimal health benefits, with less than this amount defined as low adherence. This information is collated for each patient at each clinic visit. The aims of this study were to improve the identification of patients on non-invasive ventilation that are poorly adherent to paediatric specialist support, decrease the time taken for entry of NIV adherence data for all patients and evaluate process/es for scalability for future growth. Methods A LSS approach was used with identification of variation, waste and patient flow concerns in diagnostic stage. Rapid improvement events, diagnostic and solution design workshops were conducted to determine root cause, key issues and possible solutions. A preferred solution was adopted through team collaboration. Results A diagnostic stage identified two key areas: a 45% increase in telehealth and 162 man-hours of manual data entry for patient care. The solution initiated was to extract NIV machine data remotely and automate data collection. Discussion Healthcare is well suited to using the lean six-sigma approach to facilitate team collaboration in all aspects of clinical redesign of a healthcare pathway. Automating aspects of patient care may improve patient health and staff satisfaction.

Impact of Previous Continuous Positive Airway Pressure Use on Noninvasive Ventilation Adherence and Quality in Obesity Hypoventilation Syndrome: A Pragmatic Single-Center Cross-Sectional Study in Martinique.

There is a strong relationship between obstructive sleep apnea (OSA) and obesity hypoventilation syndrome (OHS). When OHS is combined with severe OSA, treatment consists of continuous positive airway pressure (CPAP), followed by noninvasive ventilation (NIV) in the case of CPAP failure. Currently, the impact of a previous use of CPAP on the quality of NIV is unknown. We conducted a cross-sectional study with OHS patients, to assess the quality of NIV according to previous CPAP use. We included 75 patients with OHS on NIV (65 women, 87%). Among these, 40 patients (53.3%) who had had prior CPAP (CPAP+ group) were compared to the remaining 35 patients (46.7%) (CPAP- group). Key characteristics were comparable between the CPAP+ and the CPAP- groups: age at diagnosis of OHS was 67 ± 3 vs. 66 ± 4 years (p = 0.8), age at inclusion was 73 ± 15 vs. 69 ± 15 years (p = 0.29), number of comorbidities was 3.7 ± 1.2 vs. 3.3 ± 1.5, the Charlson index was 5.1 ± 2 vs. 4.6 ± 1.8, and BMI was 41.6 ± 7.6 kg/m2 vs. 41.2 ± 8.2, respectively, all p > 0.05. Follow-up length was greater in CPAP+ vs. CPAP- patients (5.6 ± 4.2 vs. 2.9 ± 2.9 years, p = 0.001). The quality of NIV based on daily adherence, pressure support, apnea-hypopnea index (AHI) and leaks was similar in both groups. Reduced adherence (less than 4 h daily) was found in 10 CPAP+ patients (25%) versus 7 CPAP- patients (20%), p = 0.80. NIV efficacy was also similar. This study found no difference in the quality of NIV or in adherence between patients who had had prior CPAP and those who had not. Previous CPAP does not appear to improve the quality of NIV.

Psychological Factors Influencing Adherence to NIV in Neuromuscular Patients Dependent on Non Invasive Mechanical Ventilation: Preliminary Results.

Non-invasive ventilation (NIV) is associated with improvement of both morbility and mortality in patients affected by neuromuscular diseases with chronic respiratory failure. Several studies have also shown that long-term NIV positively impacts the patient's quality of life and perception of disease status. Its effectiveness is likely related to the adherence to NIV. Several factors, patient- and not patient-related, may compromise adherence to NIV, such as physical, behavioral, familiar, and social issues. Few data are currently available on the role of psychological factors in influencing NIV adherence. In this pilot study, we evaluated the adherence to NIV in a group of 15 adult patients with neuromuscular diseases (Duchenne muscular dystrophy, myotonic dystrophy, and amyotrophic lateral sclerosis) in relation to their grade of depression assessed by the Beck Depression Inventory (BDI) questionnaire. Other data were collected, such as clinical features (age and sex), use of anxiolytic drugs, the presence of a family or professional caregiver, the quality of patient-physician relationship, the beginning of psychological support after BDI screening, and the family acceptance of NIV. NIV adherence was definied as the use of NIV for at least 4 h per night on 70% of nights in a month. The overall rate of NIV adherence was 60%. Based on the BDI questionnaire, patients who were non-adherent to NIV had a higher rate of depression, mainly observed in the oldest patients. The acceptance of NIV by the family and positive physician-patient interaction seem to favor NIV adherence. Depression can interfere with NIV adherence in patients with neuromuscolar diseases.

Mortality in individuals with COPD on long-term home non-invasive ventilation

BackgroundReal-world evidence regarding survival of patients with chronic obstructive pulmonary disease (COPD) using chronic non-invasive ventilation (NIV) is scarce. Research questionHow do obesity and other factors relate to mortality in patients with COPD on chronic NIV? Study designand Methods: We retrospectively analyzed data from COPD patients enrolled in a home ventilation program between 2014 and 2018. Survival was compared between obese and non-obese groups using the Kaplan-Meier method. Factors associated with mortality were identified using multivariable Cox proportional regression analyses with Least Absolute Selection and Shrinkage Operator (LASSO) regularization. Univariable analyses were also done stratified by obesity. ResultsMedian survival was 80.0 (95% CI: 71.0-NA) months among obese (n = 205) and 30.0 (95%CI: 19.0–42.0) months in non-obese (n = 61) patients. NIV adherence was high in both groups. Mortality was associated with male gender [HR 1.44], chronic opioids or benzodiazepines use [HR 1.07], home oxygen use [HR 1.82], fixed pressure mode of ventilation [HR 1.55], NIV inspiratory pressure [HR 1.05], and thoracic cancer [HR 1.27]; obesity [HR: 0.43], age [HR 0.99] and NIV expiratory pressure [HR 0.94] were associated with decreased mortality. In the obese, univariable analyses revealed that chest wall disease, thoracic cancer, home oxygen use, FEV1% predicted, and ventilation parameters were associated with mortality. In the non-obese, male gender and respiratory comorbidities were related to mortality. InterpretationObesity is associated with improved survival in COPD patients highly adherent to NIV. Other factors associated with mortality reflect disease severity and ventilator parameters, with differences between obese and non-obese patients.

Impact of COVID-19 pandemic on adherence to noninvasive ventilation in children.

The aim of this study was to measure changes in adherence to noninvasive ventilation (NIV) in children during the first year of the COVID-19 pandemic. Retrospective chart review of children (0-18 years) using home NIV through the Stollery's Pediatric NIV program in Edmonton, Canada during March 2019 to March 2021. Demographics, clinical characteristics, and adherence information from machine downloads were collected prior to and during the first year of the COVID-19 pandemic. Paired t tests and Chi-square compared adherence prepandemic and during pandemic and repeated analysis of variance tests compared adherence pre- and 0-6 and 6-12 months during pandemic. Eighty-two children met inclusion criteria (62% male; age 8.6 ± 4.6 years). Overall, there were no changes in NIV adherence during pandemic (average NIV minutes pre- and during pandemic of 390 ± 219 and 405 ± 300 minutes, respectively). When separated into increased vs decreased adherence groups, adherence differences pre- compared to during pandemic became significant, with no differences across demographic/clinical variables or prepandemic adherence. There were no changes in NIV adherence during the initial 6 months of pandemic compared to prepandemic, but NIV use significantly increased by 9-57 minutes during the following 6-12 months. Bilevel positive airway users had longer NIV use than continuous positive airway pressure users at all time points, with an overall increasing trend over time. Despite the significant life disruption created by COVID-19 and changes to virtual care, children using home NIV maintained adequate adherence. Qualitative research is needed to understand the nuances of using NIV during the pandemic and potential advantages of virtual care for support of these children and families. Halperin H, Chalifour M, Bedi PK, etal. Impact of COVID-19 pandemic on adherence to noninvasive ventilation in children. J Clin Sleep Med. 2023;19(1):179-188.

Home-Based Adaptation to Night-Time Non-Invasive Ventilation in Patients with Amyotrophic Lateral Sclerosis: A Randomized Controlled Trial

Background: Initiation to Non-Invasive Ventilation (NIV) in amyotrophic lateral sclerosis (ALS) can be implemented in an inpatient or outpatient setting. Aims: We aimed to evaluate the efficacy of adaptation (the number of needed sessions) to home-based NIV compared to an outpatient one in ALS in terms of arterial carbon dioxide (PaCO2) improvement. NIV acceptance (mean use of ≥5 h NIV per night for three consecutive nights during the adaptation trial), adherence (night-time NIV usage for ≥150 h/month), quality of life (QoL), and caregiver burden were secondary outcomes. Methods: A total of 66 ALS patients with indications for NIV were involved in this randomized controlled trial (RCT): 34 underwent NIV initiation at home (home adaptation, HA) and 32 at multiple outpatient visits (outpatient adaptation, OA). Respiratory function tests were performed at baseline (the time of starting the NIV, T0) together with blood gas analysis, which was repeated at the end of adaptation (T1) and 2 (T2) and 6 (T3) months after T1. NIV adherence was measured at T2 and T3. Overnight cardiorespiratory polygraphy, Short Form Health Survey (SF-36), Caregiver Burden Inventory (CBI), Caregiver Burden Scale (CBS), and Zarit Burden Interview (ZBI) were performed at T0, T2, and T3. Results: Fifty-eight participants completed the study. No differences were found between groups in PaCO2 at T1 (p = 0.46), T2 (p = 0.50), and T3 (p = 0.34) in acceptance (p = 0.55) and adherence to NIV at T2 and T3 (p = 0.60 and p = 0.75, respectively). At T2, the patients’ QoL, assessed with SF-36, was significantly better in HA than in OA (p = 0.01), but this improvement was not maintained until T3 (p = 0.17). Conclusions: In ALS, adaptation to NIV in the patient’s home is as effective as that performed in an outpatient setting regarding PaCO2, acceptance, and adherence, which emphasizes the need for further studies to understand the role of the environment concerning NIV adherence.

P027 The clinical role and outcomes of non-invasive ventilation in motor neuron disease: An Australian tertiary hospital experience

Abstract Introduction In motor neuron disease (MND), non-invasive ventilation (NIV) in patients who develop respiratory muscle weakness improves both quality of life and survival. This study aimed to evaluate the current practice and outcomes of NIV use in MND patients in an Australian tertiary hospital. Methods The medical records of all MND patients who attended a specialist multidisciplinary clinic requiring NIV treatment between January 2015 and January 2020 were retrospectively analysed. Progress to date: Forty-five patients have been analysed with a mean age at time of NIV commencement of 61±10(SD) years, 67% were male, 33% were current or past smokers and 7% had OSA with previous CPAP use. MND onset was limb in 58%, bulbar in 36% and respiratory muscle in 7%. Riluzole was prescribed in 47% and PEG/RIG insertion performed in 47%. At time of NIV commencement, 82% were symptomatic and 47% hypercapnic. No patient was commenced based on functional testing alone. NIV adherence (usage ≥4hours/night) was observed in 80%. NIV non-adherence was associated with bulbar subtype (p=0.02) and empirical NIV initiation (p<0.01) on univariate analysis. Average survival from NIV commencement was 17±22(SD)months. Average survival on NIV in adherent patients was 19±24(SD)months and non-adherent patients was 2±2(SD)months, although this did not reach statistical significance (p=0.1). Intended outcome & impact Overall clinical practice and outcomes of NIV use in this study is comparable to literature. The factors influencing NIV tolerance and adherence require further study to optimise outcomes in MND patients with respiratory muscle weakness.

Characterization of sleep-disordered breathing in children with Duchenne muscular dystrophy by the American Academy of Sleep Medicine criteria vs disease-specific criteria: what are the differences?

Individuals with Duchenne muscular dystrophy (DMD) frequently develop sleep-disordered breathing. Noninvasive ventilation is often prescribed for sleep-disordered breathing treatment based on the American Academy of Sleep Medicine (AASM) criteria. In 2018, DMD disease-specific criteria for sleep-disordered breathing were established. Our study aimed to examine the clinical interpretation differences using these different criteria. We performed a multicenter, retrospective chart review of children with DMD followed at The Hospital for Sick Children, Toronto, Canada, and Rady Children's Hospital, San Diego, California, who underwent polysomnography from August 1, 2012, to February 29, 2020. Baseline characteristics and polysomnography data were summarized using descriptive statistics. Agreement for the diagnosis of sleep-disordered breathing evaluated by kappa statistics and sensitivity/specificity analysis was assessed. One hundred five male children with DMD (mean ± SD age: 12.1 ± 3.8 years; body mass index z score: 0.2 ± 2.3) were included. The proportions of children with DMD that met at least 1 AASM criterion and at least 1 DMD criterion were 45.7% and 67.6%, respectively. We found that 32.4% of children met neither AASM nor DMD criteria. Overall agreement between AASM and DMD criteria was moderate (k = 0.57). There was almost perfect agreement in sleep apnea diagnosis (k = 0.90); however, there was only slight agreement in hypoventilation diagnosis (k = 0.12) between AASM and DMD criteria. There were more children with DMD diagnosed with nocturnal hypoventilation and prescribed noninvasive ventilation using DMD criteria compared with AASM criteria. Future studies should address whether the prescription of noninvasive ventilation for children with DMD based on both criteria is associated with different clinical outcomes. Hurvitz MS, Sunkonkit K, Massicotte C, Li R, Bhattacharjee R, Amin R. Characterization of sleep-disordered breathing in children with Duchenne muscular dystrophy by the American Academy of Sleep Medicine criteria vs disease-specific criteria: what are the differences? J Clin Sleep Med. 2022;18(2):609-615.

Feasibility of long-term home noninvasive ventilation program in a general pediatric unit: 21 years' experience in Hong Kong.

Long-term home noninvasive ventilation (NIV) is increasingly employed in children with sleep-disordered breathing and chronic respiratory failure. While studies suggest its successful implementation in tertiary care centers, little is known about the situation in a general care setting. Hence, we aim to evaluate the clinical profiles of these children in a general pediatric unit over the past two decades. Data collected retrospectively on patients younger than 18 years old receiving long-term home NIV from January 1, 1997 to December 31, 2017 in a Hong Kong regional general pediatric unit were reviewed. The number of children on home NIV increased more than 10-fold over the past two decades. In total, 114 children were commenced on NIV during the 21-year period. Upper airway obstruction was the most common cause (77%), followed by neuromuscular diseases (16%), pulmonary disorders (4%), and abnormal ventilatory control (3%). Continuous positive airway pressure was the most common NIV type (59%). To date, 46% of the children remained in our NIV program, while 18% discontinued NIV support. NIV adherence increased significantly with follow-up (median of 78.6% and 82.5% at baseline and last follow-up, respectively). Sixty-five percent of the children used NIV for at least 4 h on 70% of the days monitored. Higher body mass index was associated with lower adherence. Pediatric home NIV is feasible in the general care setting with good outcomes and adherence. As the demand for NIV service grows, input from local hospitals will be of increasing importance and should be considered upon healthcare planning.

The role of noninvasive ventilation in the management of type II respiratory failure in patients with myotonic dystrophy.

Type 1 myotonic dystrophy (DM1) causes sleep disordered breathing and respiratory failure due to a combination of obstructive sleep apnoea, reduced central drive and respiratory muscle weakness. Noninvasive ventilation (NIV) is commonly used for treating respiratory failure in neuromuscular disease; however, there have been few studies assessing the role of NIV in DM1. The aim of this retrospective service evaluation was to investigate the impact of NIV adherence on hypercapnia and symptoms of hypoventilation in patients with DM1. Data on capillary carbon dioxide tension (PCO2), lung function, adherence to NIV and symptoms of hypoventilation were obtained from the records of 40 patients with DM1. Mean capillary PCO2 significantly reduced from 6.81±1.17 kPa during supervised inpatient set-up to 5.93±0.82 kPa after NIV set-up (p<0.001). NIV adherence reduced from 7.8 (range: 1.0–11.0) h per 24 h during supervised inpatient set-up to 2.9 (0–10.4) h per 24 h in the community. Overall 72% of patients used NIV <5 h per 24 h during follow-up, including 11% who discontinued NIV completely. There was no correlation between adherence to NIV and changes in capillary PCO2. Patients who reported symptomatic benefit (50%) had higher adherence than those who did not feel benefit (p<0.05). In conclusion, in patients with myotonic dystrophy with Type II respiratory failure maintaining adherence is challenging.

Determinants of usage and nonadherence to noninvasive ventilation in children and adults with Duchenne muscular dystrophy.

Duchenne muscular dystrophy (DMD) is a neuromuscular disorder that leads to chronic respiratory insufficiency and failure. Use of home noninvasive ventilation (NIV) has been linked to improved outcomes including reduced mortality. Despite the importance of NIV, factors promoting optimal NIV usage and determinants of nonadherence have not been rigorously examined. Moreover, given that respiratory issues in DMD span between childhood and adulthood, examination across a broad age group is needed. The objectives of this study were to (1) evaluate NIV usage across a broad spectrum of patients with DMD, including both children and adults, and (2) identify biological and socioeconomic determinants of NIV usage and NIV nonadherence. We performed a retrospective review of all patients with DMD from February 2016 to February 2020 who underwent evaluation at associated pediatric and adult neuromuscular disease clinics. NIV use was determined objectively from device downloads. A priori, we defined nonadherence as < 4 hours use per night, quantified as the percentage of nights below this threshold across a 30-day period within 6 months of a clinic visit. We also assessed the average hours of NIV usage over this time period. Predictors examined included demographics, social determinants, and pulmonary function. 33 patients with DMD were identified, 29 (87%) of whom were using NIV (13 age < 21 years). Mean age was 22.9 ± 6.6 years (range 13-39 years), body mass index was 23.4 ± 10.4 kg/m2, and seated forced vital capacity was 23% ± 18% predicted. Mean nightly NIV usage was 7.4 ± 3.8 hours and mean percentage of nonadherent nights was 13% ± 30%. In univariable analysis, age did not predict use. Those with lower forced vital capacity had higher NIV usage hours (P = .01) and a trend toward less nonadherence (P = .06). Higher estimated household income demonstrated a trend toward increased usage hours and less nonadherence (both P = .08). Multivariable analysis found increased usage hours were predicted best by higher income, higher inspiratory positive airway pressure, and higher bicarbonate. Nonadherence was higher in those with lower income or higher forced vital capacity. In this cohort of adult and pediatric patients with DMD, most individuals were using NIV. While usage hours were higher with lower lung function, substantial variability remains unexplained by examined factors. Nonadherence was observed in some individuals, including those with advanced disease. Further investigations should focus on evaluating patient-oriented outcomes to define optimal NIV usage across the spectrum of disease and determine strategies to counteract issues with nonadherence. Hurvitz MS, Bhattacharjee R, Lesser DJ, Skalsky AJ, Orr JE. Determinants of usage and nonadherence to noninvasive ventilation in children and adults with Duchenne muscular dystrophy. J Clin Sleep Med. 2021;17(10):1973-1980.

570 Comparing NIV Adherence in Early versus Advanced Stage Sleep Disordered Breathing for Children with Neuromuscular Disease

Abstract Introduction Children with neuromuscular disease (NMD) typically develop progressive sleep disordered breathing (SDB), including obstructive sleep apnea (OSA), nocturnal hypoxemia and/or hypoventilation, due to loss of upper airway muscle tone and weakness of respiratory muscles. Commonly, the SDB initially presents during rapid eye movement (REM) sleep, as this stage is associated with physiological muscle atonia, but then progresses to non-REM (NREM) sleep and ultimately daytime respiratory insufficiency. Non-invasive ventilation (NIV) is currently the treatment of choice for children with NMD and SDB. However, the use of NIV in REM-related SDB is less demonstrated and adequate therapy adherence is unclear. The aim of this study is to determine differences in NIV adherence in children with early (REM) versus advanced (non-REM) SDB. Methods Children (0–18 years) diagnosed with NMD and using NIV for the past 10 years were included. Demographic, clinical, technology-related, and sleep study data were collected from medical charts and polysomnography reports. Adherence data (mean hours of NIV use and % days NIV was used &amp;gt;4hrs) were collected from NIV machine downloads. Children were categorized into two groups based on based on their apnea-hypopnea index (AHI) ratio between REM and NREM sleep. Children with REM-SDB were defined as a REM/NREM AHI ratio of ≥ 2. Children with NREM-SDB were defined as a REM/NREM AHI ratio &amp;lt; 2. Results A total of 14 children (9 REM-SDB and 5 NREM-SDB) were included in the analysis. Both groups were comparable with respect to demographic, clinical, and technology-related characteristics. A total of 24 adherence reports were available for the cohort (16 REM-SDB and 8 NREM-SDB). The mean hours of NIV use per night was comparable between the REM-SDB and NREM-SDB groups (9.2±1.3hrs vs. 9.0±0.4hrs respectively), but the percent days NIV was used &amp;gt;4hrs was higher in the NREM-SDB group (68.7±9.6 vs. 93.0±2.7, p=0.03). Conclusion NIV adherence was high for children with both REM-SDB and NREM-SDB. While hours of NIV use were comparable between both groups, suggesting good NIV tolerance through the night, children with REM-SDB had a lower percentage of days with NIV use &amp;gt;4hr, suggesting less willingness to use the therapy. Support (if any):

Nasal versus oronasal masks for home non-invasive ventilation in patients with chronic hypercapnia: a systematic review and individual participant data meta-analysis

BackgroundThe optimal interface for the delivery of home non-invasive ventilation (NIV) to treat chronic respiratory failure has not yet been determined. The aim of this individual participant data (IPD) meta-analysis...

Efficacy and adherence of noninvasive ventilation treatment in children with Down syndrome.

Children with Down syndrome (DS) have an increased prevalence of obstructive sleep apnea (OSA). Noninvasive ventilation (NIV) is a common modality of OSA treatment in this cohort. This study aimed to measure adherence and efficiency of NIV delivery in children with DS. This was a retrospective cohort study involving 106 children with confirmed OSA and home NIV with downloadable data capacity. Children were divided into DS (n = 44) and non-DS cohorts (n = 62). Adherence, clinical outcomes apnea-hypopnoea index (AHI), positive airway pressure delivery, and leakage were recorded and compared between DS and non-DS cohorts and within the DS cohort based on past surgical history. Significantly greater NIV usage was observed in the DS cohort, they showed more consistent use with an increased percentage of days used relative to their non-DS counterparts (78.95 ± 2.26 vs. 72.11 ± 2.14, p = .031). However, despite greater usage, poorer clinical outcomes in the form of increased AHI (p = .0493) was observed in the DS cohort, where significantly greater leakage was also shown 41.00 ± 1.61 L/min versus 36.52 ± 1.18 L/min (p = .022). Twenty children with DS had prior cardiac surgery; compliance across all parameters was significantly reduced relative to those without. These data confirm that satisfactory NIV adherence is achievable in children with DS. However, we have identified excessive system leak at the machine-patient interface as a factor, which could undermine NIV efficacy in children with DS.

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

IntroductionAlthough 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...

Long-term Noninvasive Ventilation in Obesity Hypoventilation Syndrome Without Severe OSA: The Pickwick Randomized Controlled Trial

Noninvasive ventilation (NIV) is an effective form of treatment in obesity hypoventilation syndrome (OHS) with severe OSA. However, there is paucity of evidence in patients with OHS without severe OSA phenotype. Is NIV effective in OHS without severe OSA phenotype? In this multicenter, open-label parallel group clinical trial performed at 16 sites in Spain, we randomly assigned 98 stable ambulatory patients with untreated OHS and apnea-hypopnea index< 30 events/h (ie, no severe OSA) to NIV or lifestyle modification (control group) using simple randomization through an electronic database. The primary end point was hospitalization days per year. Secondary end points included other hospital resource utilization, incident cardiovascular events, mortality, respiratory functional tests, BP, quality of life, sleepiness, and other clinical symptoms. Both investigators and patients were aware of the treatment allocation; however, treating physicians from the routine care team were not aware of patients' enrollment in the clinical trial. The study was stopped early in its eighth year because of difficulty identifying patients with OHS without severe OSA. The analysis was performed according to intention-to-treat and per-protocol principles and by adherence subgroups. Forty-nine patients in the NIV group and 49 in the control group were randomized, and 48 patients in each group were analyzed. During a median follow-up of 4.98 years (interquartile range, 2.98-6.62), the mean hospitalization days per year ± SD was 2.60 ± 5.31 in the control group and 2.71 ± 4.52 in the NIV group (adjusted rate ratio, 1.07; 95%CI, 0.44-2.59; P= .882). NIV therapy, in contrast with the control group, produced significant longitudinal improvement in Paco2, pH, bicarbonate, quality of life (Medical Outcome Survey Short Form 36 physical component), and daytime sleepiness. Moreover, per-protocol analysis showed a statistically significant difference for the time until the first ED visit favoring NIV. In the subgroup with high NIV adherence, the time until the first event of hospital admission, ED visit, and mortality was longer than in the low adherence subgroup. Adverse events were similar between arms. In stable ambulatory patients with OHS without severe OSA, NIV and lifestyle modification had similar long-term hospitalization days per year. A more intensive program aimed at improving NIV adherence may lead to better outcomes. Larger studies are necessary to better determine the long-term benefit of NIV in this subgroup of OHS. ClinicalTrials.gov; No.: NCT01405976; URL: www.clinicaltrials.gov.

Understanding adherence to noninvasive ventilation in youth with Duchenne muscular dystrophy.

Duchenne muscular dystrophy (DMD) is an X-linked, progressive neuromuscular disorder that results in chronic respiratory insufficiency and subsequently failure requiring noninvasive ventilation (NIV). Adherence to NIV in neuromuscular disorders and related barriers are poorly described. The aim of the current study was to assess NIV adherence, adherence barriers, and identify psychosocial predictors of adherence in young boys with early DMD-related sleep disordered breathing and recommended nocturnal NIV. This cross-sectional study included 42 youth with DMD with prescribed nocturnal NIV, and their caregivers. Caregivers and youth completed questionnaires assessing adherence barriers, psychosocial symptoms (eg, anxiety and depressive symptoms), and stress. Medical information pertinent to cardiopulmonary health and neurologic status at both enrollment and initiation of NIV was reviewed. Adherence to NIV, defined as percent days used and days used ≥4 hours/day was 56.1 ± 38.7% and 46.2 ± 40.6%, respectively. Average duration of use on days worn was 5.61 ± 4.23 hours. NIV usage was correlated with the severity of obstructive sleep apnea but not cardiopulmonary variables. Mask discomfort was the most commonly reported adherence barrier followed by behavioral barriers (eg, refusing to use). Multiple regression analyses revealed that internalizing behaviors (eg, anxiety and depressive symptoms) and total adherence barriers significantly predicted NIV adherence. Adherence to NIV in DMD is poor and similar to other pediatric chronic diseases. Our data suggest interventions targeting adherence barriers and patient internalizing symptoms may improve adherence to NIV in DMD.

Practical implementation of noninvasive ventilation in Amyotrophic Lateral Sclerosis: lessons learned from a clinical case series.

PurposeNoninvasive ventilation (NIV) may improve survival and quality of life in Amyotrophic Lateral Sclerosis (ALS) patients. There is a surprising paucity of practical guidelines for office-based implementation and management of NIV outside of tertiary ALS centers. We saw the need for a clinical protocol to allow feasible and consistent NIV management in this patient population.MethodsWe created a clinical protocol for office-based initiation of NIV implemented on consecutive ALS patients referred from our regional ALS multidisciplinary clinic. The protocol provided initial empiric settings using a bilevel device in volume-assured pressure support mode. A respiratory therapist (RT) initiated NIV in an office setting and made adjustments according to patient tolerance and therapy targets outlined in the protocol. Later setting changes were performed at patient or provider request. We evaluated patient adherence and efficacy via device download at 30 days and 1 year.ResultsWe present data from a case series of the first 14 consecutive patients initiated on NIV over a 20-month period. Our protocol underwent iterative modification based on clinical experience and patient feedback. Early challenges included the significant time and resource burden required to coordinate device downloads and patient follow-up. Early 30-day NIV adherence was variable (median 20 out of 30 days), while 1-year NIV adherence was excellent (median 27.5 out of 30 days).ConclusionsOur RT-driven clinical NIV protocol was feasible but labor intensive. Achieving real-world adherence of NIV in our ALS patients required iterative protocol adjustment, significant RT provider time, and tele-based follow-up.

Non-Invasive Positive airway Pressure thErapy to Reduce Postoperative Lung complications following Upper abdominal Surgery (NIPPER PLUS): protocol for a single-centre, pilot, randomised controlled trial

IntroductionPostoperative pulmonary complications (PPCs) are a common serious complication following upper abdominal surgery leading to significant consequences including increased mortality, hospital costs and prolonged hospitalisation. The primary objective of this...