Positive Airway Pressure and Metabolic Markers in Children With Obstructive Sleep Apnea.

BACKGROUND: Increasing evidence suggests an association between childhood obstructive sleep apnea (OSA) and metabolic syndrome, with more research available on the potential impacts of positive airway pressure (PAP) on metabolic markers in children. The purpose of this systematic review is to provide a systematic synthesis of the evidence on the effect of PAP use on metabolic markers in children with OSA. METHODS: A search strategy with terms for "OSA" and metabolic markers in pediatrics was run to systematically assess 5 databases until August 26, 2022. Two reviewers independently screened eligible articles, extracted data, and conducted quality appraisal. Meta-analysis was done using random-effects models. Body mass index (BMI), glycemic, lipid, cardiovascular, and other metabolic and inflammatory markers were reported. RESULTS: Sixteen studies (N = 1,213) were included, 15 observational studies and 1 randomized controlled trial (RCT); most reported outcomes in children with obesity. Meta-analysis of 4 studies found no changes in BMI at median average follow-up of 12 months after PAP initiation. A reduction in heart rate and blood pressure parameters was demonstrated in several studies in children with OSA with and without obesity at a median average follow-up of 4.9 months after PAP initiation. Research in echocardiographic outcomes is limited, including one RCT in children with Down syndrome and OSA showing no changes in heart rate variability parameters. Evidence of improvements in glycemic and/or lipid control, liver enzymes, and inflammatory markers with PAP therapy is even more limited and of limited clinical importance. Risk of bias was moderate to critical and outcome evidence very low. CONCLUSIONS: Although evidence on effects of PAP on metabolic markers in children with OSA is encouraging, available literature is limited. Longitudinal studies are still required to further assess the long-term influence of PAP on metabolic and inflammatory markers, particularly in children with obesity.

Parents' experiences of their children's medical journeys with tracheostomies: A Focus Group Study.

Objectives: Children living with a tracheostomy have among the most complex medical care needs in Canada. The focus of this study was to gain a contemporary understanding of key aspects of these children's medical journeys. Methods: We conducted a qualitative constructivist grounded theory study using semi-structured focus groups with parents whose children are living in the community with a tracheostomy. Parents were recruited from the Stollery Children's Hospital Tracheostomy Clinic, which serves a geographically wide and diverse population. Results: Three focus groups were completed, including a total of 12 participants. Key themes leading up to tracheostomy related to contextual understanding, experiences of inclusion, and perceptions of proportionality. Parents discussed the preparedness for how a tracheostomy would affect their child, their own involvement in recovery, and the education needed for their child's medical care. Navigating hospital units related to inconsistencies in care, accommodations of families' needs, and confidence in care received. Finally, living in the community was the focus of much of the participants' discussions including coping with system-related issues, limited homecare and medical support, cost of care, and connections with the broader community of parents of children with complex medical needs. All themes encircled the family's deeply felt responsibility to care for their child. Conclusions: From both patient- and family-centered care perspectives, there exist individual and systemic issues related to the care delivery for children with a tracheostomy. It is in particular in the community where there is a severe deficiency of support afforded to these children and their families.

Tracheostomy and long-term invasive ventilation decision-making in children: A scoping review.

An increasing number of children are surviving critical illnesses requiring tracheostomy/long-term ventilation (LTV). This scoping review seeks to collate the available evidence on decision-making for tracheostomy/LTV in children. Systematic searches of electronic databases and websites were conducted for articles and reports. Inclusion criteria included: (1) children 0-18 years old; (2) described use of tracheostomy or tracheostomy/LTV; and (3) information on recommendations for tracheostomy decision-making or decision-making experiences of family-caregivers or health care providers. Articles not written in English were excluded. Of the 4463 records identified through database search and other methods, a total of 84 articles, 2 dissertations, 1 book chapter, 3 consensus statement/society guidelines, and 8 pieces of grey literature were included. Main thematic domains identified were: (1) legal and moral standards for decision-making; (2) decision-making models, roles of decision-makers, and decisional aids towards a shared decision-making model; (3) experiences and perspectives of decision-makers; (4) health system and society considerations; and (5) conflict resolution and legal considerations. A high degree of uncertainty and complexity is involved in tracheostomy/LTV decision-making. There is a need for a standardized decision-support process that is consistent with a child's best interests and shared decision-making. Strategies for optimizing communication and mechanism for managing disputes are needed.

Predictors of NIV-related adverse events in children using long-term noninvasive ventilation.

BACKGROUND AND OBJECTIVES: An increasing number of children with diverse medical conditions are using long-term noninvasive ventilation (NIV). This study examined the impact of demographic, clinical, and technology-related factors on long-term NIV adverse events in a large cohort of children using long-term NIV. METHODS: This was a multicenter retrospective review of all children who initiated long-term NIV in the province of Alberta, Canada, from January 2005 to September 2014, and followed until December 2015. Inclusion criteria were children who had used NIV for 3 months or more and had at least one follow-up visit with the NIV programs. RESULTS: We identified 507 children who initiated NIV at a median age of 7.5 (interquartile range: 8.6) years and 93% of them reported at least one NIV-related adverse event during the initial follow-up visit. Skin injury (20%) and unintentional air leaks (19%) were reported more frequently at the initial visit. Gastrointestinal symptoms, midface hypoplasia, increased drooling, aspiration and pneumothorax were rarely reported (<5%). Younger age and underlying conditions such as Down syndrome, achondroplasia, and Duchenne muscular dystrophy were early predictors of unintentional air leak. Younger age also predicted child sleep disruption in the short term and ongoing parental sleep disruption. Obesity was a risk factor for persistent nasal symptoms. Mask type was not a significant predictor for NIV-related short- or long-term complications. CONCLUSIONS: This study demonstrates that NIV-related complications are frequent. Appropriate mask-fitting and headgear adaptation, and a proactive approach to early detection may help to reduce adverse events.

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

STUDY OBJECTIVES: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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. CITATION: Halperin H, Chalifour M, Bedi PK, et al. Impact of COVID-19 pandemic on adherence to noninvasive ventilation in children. J Clin Sleep Med. 2023;19(1):179-188.

Respiratory characteristics in children with spinal muscular atrophy type 1 receiving nusinersen.

BACKGROUND: Spinal muscular atrophy type 1 (SMA1) is a neuromuscular disorder with a natural history of chronic respiratory failure and death during infancy without ventilation. Recently, disease-modifying therapies such as nusinersen have improved disease trajectory. However, objective data on the trajectory of polysomnography outcomes, the relationship between motor scores and respiratory parameters, respiratory technology dependence and healthcare utilization in children with SMA1 remain to be elucidated. METHODS: This was a retrospective observational study of children with SMA1 receiving nusinersen between October 2016 and February 2021 at two tertiary care hospitals in Canada. Baseline polysomnography data, motor scores, respiratory technology, and unanticipated healthcare utilization were examined. RESULTS: Eleven children (five females, two SMN2 copies each) were included. Median (interquartile range [IQR]) age at diagnosis was 3.6 (2.8-5.0) months and age at diagnostic polysomnogram following nusinersen initiation was 9.4 (5.3-14.0) months. Nusinersen was initiated at a median (IQR) age of 5.4 (3.4-7.6) months and 8/11 children had respiratory symptoms at that time. Diagnostic polysomnography data showed a median (IQR) central apnea-hypopnea index (AHI) of 4.1 (1.8-10.0) and obstructive AHI of 2.2 (0-8.0) events/h. We observed an inverse relationship between motor scores and central apnea-hypopnea indices. All children required ventilatory support at the end of the study period. CONCLUSION: This study showed abnormal polysomnography parameters and need for ventilation despite nusinersen suggesting ongoing need for regular monitoring with polysomnography. Understanding the respiratory disease trajectory of children undergoing treatment with nusinersen will inform decision-making regarding optimal timing of ventilatory support initiation.

Long-Term Non-invasive Ventilation in Children With Down Syndrome: A Systematic Review.

Context: Children with Down syndrome are at risk for obstructive sleep apnea, which may not be resolved by adenotonsillecotmy, as well as other respiratory disorders that may impact breathing during sleep. Long-term non-invasive ventilation, including continuous and bilevel positive airway pressure delivery, is an alternate treatment strategy. Objective: To assess the use and outcomes of long-term non-invasive ventilation in children with Down syndrome including comparison to other children using long-term non-invasive ventilation. Data Sources: The search strategy for the scoping review used Medical Subject Headings (MeSH) and free-text terms for "child" and "non-invasive ventilation." MEDLINE (Ovid), Embase (Ovid), CINAHL (Ebsco), Cochrane Library (Wiley), and PubMed databases were searched (1990-2021). Study Selection: The scoping review results were searched to identify studies including data on at least three children with Down Syndrome using long-term non-invasive ventilation. Data Extraction: Study characteristics, subject characteristics, technology type, and outcome measurements were extracted. Results: A total of 28 articles included 543 children with Down syndrome using long-term non-invasive ventilation. Children with Down syndrome accounted for 18% of children using long-term non-invasive ventilation. Data on efficacy, feasibility, and adherence in children with Down syndrome are comparable to other children. Children with Down syndrome may have greater difficulty initiating long-term non-invasive ventilation, longer time to establish use, and a higher rate of inability to establish use. Outcome data is limited but suggest favorable impact on cardiac function and attention. Limitations: Articles related to long-term non-invasive ventilation use in adolescents and young adults may have been excluded. Conclusions: Children with Down syndrome make up a significant portion of the population of children using long-term non-invasive ventilation. While there is more limited data available with respect to the use and outcomes for children with Down syndrome compared to the other children, long-term non-invasive ventilation is an effective and well-tolerated therapy with no clear differences in the use or outcomes for children with Down syndrome. Additional work is needed to understand potential challenges around establishing long-term non-invasive ventilation use in children with Down syndrome. Systematic Review Registration: https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=206533, identifier: CRD206533.

Feasibility of split night polysomnography in children to diagnose and treat sleep related breathing disorders.

STUDY OBJECTIVES: The gold standard test for diagnosis of sleep related breathing disorders (SRBD) in children is diagnostic polysomnography (PSG). This is often followed by a titration PSG to identify optimal non-invasive ventilation (NIV) pressures. Access to pediatric PSG is limited, resulting in delays to diagnosis and initiation of treatment. Split-night PSGs (snPSG) combine a diagnostic and titration PSG into a single night study. Although described in adults, the pediatric literature on this topic is sparse. The objective of this study was to describe a large cohort of children who utilized snPSG to diagnose SRBD and initiate NIV. METHODS: This multi-center study analyzed clinical and PSG data from children with SRBD who had initiated NIV following a snPSG. Data from diagnostic and titration portions of the snPSG were analyzed separately. RESULTS: The study included 165 children who initiated NIV following a snPSG. The majority of children (61.8%) were initiated on NIV for upper airway obstruction. The population included children with medical complexity, including those with central nervous system disorders (17.0%), musculoskeletal/neuromuscular disorders (12.1%), and cardiac disorders (1.2%). Moderate to severe SRBD was present in 87.2% of children with a median apnea-hypopnea index (AHI) of 16.6 events/hour (IQR: 8.2, 38.2). The median AHI was reduced on treatment to 7.6 events/hour (IQR: 3.3, 17.1), with fewer subjects meeting criteria for severe SRBD. CONCLUSIONS: snPSG is technically feasible in children, facilitating the diagnosis of SRBD and initiation of NIV, even in those with high medical complexity.

Outcomes of Long-Term Noninvasive Ventilation Use in Children with Neuromuscular Disease: Systematic Review and Meta-Analysis.

Objectives: To determine whether children with neuromuscular disorders using long-term noninvasive ventilation (NIV), continuous or bilevel positive airway pressure, have improved health outcomes compared with alternative treatment strategies. Data Sources: This systematic review is an extension of a scoping review. The search strategy used Medical Subject Headings and free-text terms for "child" and "noninvasive ventilation." Studies of humans from 1990 onward were searched in MEDLINE (Ovid), Embase (Ovid), CINAHL (Ebsco), Cochrane Library (Wiley), and PubMed. The results were reviewed for articles reporting on neuromuscular disorders and health outcomes including mortality, hospitalization, quality of life, lung function, sleep study parameters, and healthcare costs. Data Extraction: Extracted data included study design, study duration, sample size, age, type of NIV, follow-up period, primary disease, and primary and secondary outcome measures. Studies were grouped by primary disease into three groups: spinal muscular atrophy, Duchenne muscular dystrophy, and other/multiple neuromuscular diseases. Data Synthesis: A total of 50 articles including 1,412 children across 36 different neuromuscular disorders are included in the review. Mortality is lower for children using long-term NIV compared with supportive care across all neuromuscular disease types. Overall, mortality does not differ when comparing the use of NIV with invasive mechanical ventilation, though heterogeneity suggests that mortality with NIV is higher for spinal muscular atrophy type 1 and lower for other/multiple neuromuscular diseases. The impact of long-term NIV on hospitalization rate differed by neuromuscular disease type with lower rates compared with supportive care but higher rates compared with supportive care use for spinal muscular atrophy type 1, and lower rates compared with before NIV for other/multiple neuromuscular diseases. Overall, lung function was unaltered and sleep study parameters were improved from baseline by long-term NIV use. There are few data to assess the impact of long-term NIV use on quality of life and healthcare costs. Conclusions: Long-term NIV for children provides benefit for mortality, hospitalizations, and sleep study parameters for some sub-groups of children with neuromuscular disorders. High risk of bias and low study quality preclude strong conclusions.

Who is using noninvasive ventilation? A descriptive study examining the population enrolled in a pediatric noninvasive ventilation program.

DESIGN AND METHODS: This study used a retrospective design and involved reviewing the charts of infants and children enrolled in the noninvasive ventilation (NIV) program at a quaternary pediatric hospital located in Western Canada in 2017. Demographic and clinical variables were collected, along with variables related to adherence to NIV therapy. For data storage and analysis purposes, a comprehensive database was created. Descriptive statistics were used to analyze and better understand patterns within the data. RESULTS: Findings included a comprehensive description of the population of infants and children enrolled in this NIV program in 2017, including demographic and clinical variables as well as follow-up and adherence data. This study identified that the NIV program at this pediatric center has unique characteristics which provide an exciting opportunity for further research into the population that requires NIV support. PRACTICE IMPLICATIONS: This study presents new knowledge, gathered by examining the clinical characteristics of a pediatric population that requires NIV, which can be used to inform practice, support NIV program planning, and health resource allocation, as well as suggest directions for future research on pediatric NIV therapy.

Predictors of longitudinal outcomes for children using long-term noninvasive ventilation.

BACKGROUND: Noninvasive ventilation (NIV) is a first-line therapy for sleep-related breathing disorders and chronic respiratory insufficiency. Evidence about predictors that may impact long-term NIV outcomes, however, is scarce. The aim of this study is to determine demographic, clinical, and technology-related predictors of long-term NIV outcomes. METHODS: A 10-year multicentred retrospective review of children started on long-term continuous or bilevel positive airway pressure (CPAP or BPAP) in Alberta. Demographic, technology-related, and longitudinal clinical data were collected. Long-term outcomes examined included ongoing NIV use, discontinuation due to improvement in underlying conditions, switch to invasive mechanical ventilation (IMV) or death, patient/family therapy declination, transfer of services, and hospital admissions. RESULTS: A total of 622 children were included. Both younger age and CPAP use predicted higher likelihood for NIV discontinuation due to improvement in underlying conditions (p < .05 and p < .01). Children with upper airway disorders or bronchopulmonary dysplasia were less likely to continue NIV (p < .05), while presence of central nervous system disorders had a higher likelihood of hospitalizations (p < .01). The presence of obesity/metabolic syndrome and early NIV-associated complications predicted higher risk for NIV declination (p < .05). Children with more comorbidities or use of additional therapies required more hospitalizations (p < .05 and p < .01) and the latter also predicted higher risk for being switched to IMV or death (p < .001). CONCLUSIONS: Demographic, clinical data, and NIV type impact long-term NIV outcomes and need to be considered during initial discussions about therapy expectations with families. Knowledge of factors that may impact long-term NIV outcomes might help to better monitor at-risk patients and minimize adverse outcomes.

Does obstructive sleep apnoea contribute to obesity, hypertension and kidney dysfunction in children? A systematic review protocol.

INTRODUCTION: Childhood obstructive sleep apnoea (OSA) is a highly prevalent disorder that may directly contribute to the development of obesity, hypertension and renal injury. Although those associations seem to be clearer in adults, studies in children have revealed conflicting results and updated synthesis of the evidence is lacking. The aim of this systematic review is to summarise the available evidence on the effect of OSA on obesity, systemic blood pressure and kidney function, to help to elucidate whether respiratory interventions to correct OSA would have the potential to improve those outcomes. METHODS AND ANALYSIS: A systematic literature review search was created by a medical librarian and peer-reviewed by a second librarian prior to running. Ovid Medline, Ovid Embase, CINAHL via EbscoHOST, Wiley Cochrane Library and ProQuest Dissertations and Theses Global were searched on 25 February 2020. Titles and abstracts will be screened by two independent reviewers for inclusion, followed by full-text screening of relevant articles. Studies in children will be included if they report data on OSA and weight, systemic blood pressure or kidney parameters. The extracted data will be combined for analysis and the information subcategorised in groups based on outcome. Risk of bias will be determined using tools specific to study methodology and certainty of the evidence using the Grading of Recommendations, Assessment, Development and Evaluations approach. ETHICS AND DISSEMINATION: This study will provide essential information for healthcare professionals to better understand the relationship between childhood OSA and changes in body mass index, systemic blood pressure and kidney function indicators. Our findings will be disseminated through conferences and publications. The results of this review may guide the initiation of new strategies and the development of future research studies. This research did not involve human subjects and therefore did not undergo research ethical review. PROSPERO REGISTRATION NUMBER: CRD42020171186.

Does non-invasive ventilation change metabolic markers in children with obstructive sleep apnoea? A systematic review and meta-analysis study protocol.

INTRODUCTION: Obstructive sleep apnoea (OSA) is not only common within paediatrics but is associated with critical childhood metabolic morbidity such as obesity, cardiovascular disease and glucose tolerance impairment. Increasing evidence suggests an association between childhood OSA and metabolic syndrome such as markers of cardiovascular disease, systemic hypertension, glucose intoleranceand increased lipid profile. Recent studies have targeted changes in metabolic markers in children using non-invasive ventilation (NIV) but no systematic reviews are available to summarise this emerging evidence. The purpose of this systematic review is to provide systematic synthesis of the evidence on the effect of NIV use on metabolic markers in children with OSA. METHODS AND ANALYSIS: A systematic search of electronic databases and grey literature will include paediatric interventional studies (random controlled trials, cohort studies) with and without a comparison group. Two reviewers will independently undertake the two step process of title/abstract and full-text screening. Data will be extracted and assessed, with aggregate data being reported. When the data allow, meta-analysis will be performed. ETHICS AND DISSEMINATION: There are no ethical concerns with this systematic review, as data have previously been published. This review will inform clinicians taking care of children with OSA and obesity/metabolic syndrome about the potential effects of NIV therapies on metabolic markers and has the potential to change the approach to childhood OSA and obesity. Results of this systematic review will be submitted for dissemination in abstract and manuscript form.

American Thoracic Society 2019 Pediatric Core Curriculum.

The American Thoracic Society Pediatric Core Curriculum updates clinicians annually in pediatric pulmonary disease in a 3 to 4 year recurring cycle of topics. The 2019 course was presented in May during the Annual International Conference. An American Board of Pediatrics Maintenance of Certification module and a continuing medical education exercise covering the contents of the Core Curriculum can be accessed online at www.thoracic.org.

Mask interfaces for home non-invasive ventilation in infants and children.

The selection of the mask interface for non-invasive ventilation (NIV) is recognized to be an essential part for therapy success. While nasal masks are the first recommended option in children and adults, there are indications for other mask types such as intolerance or complications from nasal masks. Evidence comparing performance, adherence and complication risk among mask interfaces in pediatrics is, however, scarce and information is often extrapolated from adult studies. Given this gap in knowledge and the lack of guidelines on NIV initiation in children, mask selection often relies on the clinicians' knowledge and expertise. Careful mask selection, a well-fitting headgear and time investment for mask desensitization are some important recommendations for adequate mask adaptation in children. Frequent mask-related complications include nasal symptoms, unintentional leak, mask displacement, skin injury, and midface hypoplasia. Close monitoring and a pro-active approach may help to minimize complications and promote the optimal use of home NIV.

Long-term Non-Invasive Ventilation in Infants: A Systematic Review and Meta-Analysis.

BACKGROUND: The use of long-term non-invasive ventilation (NIV) to treat sleep and breathing disorders in children has increased substantially in the last decade; however, less data exist about its use in infants. Given that infants have distinct sleep and breathing patterns when compared to older children, the outcomes of infants on long-term NIV may differ as well. The aim of this study is to systematically review the use and outcomes of long-term NIV in infants. METHODS: Ovid Medline, Ovid Embase, CINAHL (via EbscoHOST), PubMed, and Wiley Cochrane Library were systematically searched from January 1990 to July 2017. Studies on infants using long-term NIV outside of an acute care setting were included. Data were extracted on study design, population characteristics, and NIV outcomes. RESULTS: A total of 327 studies were full-text reviewed, with final inclusion of 60. Studies were distributed across airway (40%), neuromuscular (28%), central nervous system (10%), cardio-respiratory (2%), and multiple (20%) disease categories. Of the 18 airway studies reporting on NIV outcomes, 13 (72%) reported improvements in respiratory parameters. Of the 12 neuromuscular studies exclusively on spinal muscular atrophy type 1 (SMA1), six (50%) reported decreased hospitalizations and nine (75%) reported on mortality outcomes. Risk of bias was moderate to serious, and quality of the evidence was low to very low for all studies. Most studies had an observational design with no control group, limiting the potential for a meta-analysis. CONCLUSION: The outcomes reported in studies differed by the disease category being studied. Studies on airway conditions showed improvements in respiratory parameters for infants using NIV. Studies on neuromuscular disorder, which were almost exclusively on SMA1, reported decreased hospitalizations and prolonged survival. Overall, it appears that NIV is an effective long-term therapy for infants. However, the high risk of bias and low quality of the available evidence limited strong conclusions.

Longitudinal changes in clinical characteristics and outcomes for children using long-term non-invasive ventilation.

OBJECTIVES: To describe longitudinal trends in long-term non-invasive ventilation (NIV) use in children including changes in clinical characteristics, NIV technology, and outcomes. METHODS: This was a multicenter retrospective cohort of all children started on long-term NIV from 2005 to 2014. All children 0 to 18 years who used NIV continuously for at least 3 months were included. Measures and main outcomes were: 1) Number of children starting NIV; 2) primary medical condition; 3) medical complexity defined by number of comorbidities, surgeries and additional technologies; 4) severity of sleep disordered breathing measured by diagnostic polysomnography; 5) NIV technology and use; 6) reasons for NIV discontinuation including mortality. Data were divided into equal time periods for analysis. RESULTS: A total of 622 children were included in the study. Median age at NIV initiation was 7.8 years (range 0-18 years). NIV incidence and prevalence increased five and three-fold over the 10-year period. More children with neurological and cardio-respiratory conditions started NIV over time, from 13% (95%CI, 8%-20%) and 6% (95%CI, 3%-10%) respectively in 2005-2008 to 23% (95%CI, 18%-28%) and 9% (95%CI, 6%-14%, p = 0.008) in 2011-2014. Medical complexity and severity of the sleep-disordered breathing did not change over time. Overall, survival was 95%; mortality rates, however, rose from 3.4 cases (95% CI, 0.5-24.3) to 142.1 (95% CI 80.7-250.3, p<0.001) per 1000 children-years between 2005-2008 and 2011-2014. Mortality rates differed by diagnostic category, with higher rates in children with neurological and cardio-respiratory conditions. CONCLUSIONS: As demonstrated in other centers, there was a significant increase in NIV prevalence and incidence rate. There was no increase in medical complexity or severity of the breathing abnormalities of children receiving long-term NIV over time. The mortality rate increased over time, maybe attributable to increased use of NIV for children with neurological and cardio-respiratory conditions.

Long-term non-invasive ventilation therapies in children: A scoping review.

Long-term non-invasive ventilation (NIV) is a common modality of breathing support used for a range of sleep and respiratory disorders. The aim of this scoping review was to provide a summary of the literature relevant to long-term NIV use in children. We used systematic methodology to identify 11,581 studies with final inclusion of 289. We identified 76 terms referring to NIV; the most common term was NIV (22%). Study design characteristics were most often single center (84%), observational (63%), and retrospective (54%). NIV use was reported for 73 medical conditions with obstructive sleep apnea and spinal muscular atrophy as the most common conditions. Descriptive data, including NIV incidence (61%) and patient characteristics (51%), were most commonly reported. Outcomes from sleep studies were reported in 27% of studies followed by outcomes on reduction in respiratory morbidity in 19%. Adverse events and adherence were reported in 20% and 26% of articles respectively. Authors reported positive conclusions for 73% of articles. Long-term use of NIV has been documented in a large variety of pediatric patient groups with studies of lower methodological quality. While there are considerable data for the most common conditions, there are fewer data to support NIV use for many additional conditions.

Long-term non-invasive ventilation therapies in children: a scoping review protocol.

INTRODUCTION: Non-invasive ventilation (NIV) in children has become an increasingly common modality of breathing support where pressure support is delivered through a mask interface or less commonly through other non-invasive interfaces. At this time, NIV is considered a first-line option for ventilatory support of chronic respiratory insufficiency associated with a range of respiratory and sleep disorders. Previous reviews on the effectiveness, complications and adherence to NIV treatment have lacked systematic methods. The purpose of this scoping review is to provide an overview of the evidence for the use of long-term NIV in children. METHODS AND ANALYSIS: We will use previously established scoping methodology. Ten electronic databases will be searched to identify studies in children using NIV for longer than 3 months outside an intensive care setting. Grey literature search will include conference proceedings, thesis and dissertations, unpublished trials, reports from regulatory agencies and manufacturers. Two reviewers will independently screen titles and abstracts for inclusion, followed by full-text screening of potentially relevant articles to determine final inclusion. Data synthesis will be performed at three levels: (1) an analysis of the number, publication type, publication year, and country of publication of the studies; (2) a summary of the study designs, outcomes measures used; (3) a thematic analysis of included studies by subgroups. ETHICS AND DISSEMINATION: This study will provide a wide and rigorous overview of the evidence on the use of long-term NIV in children and provide critical information for healthcare professionals and policymakers to better care for this group of children. We will disseminate our findings through conference proceedings and publications, and evaluate the results for further systematic reviews and meta-analyses.