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Spinal muscular atrophy (SMA) and Duchenne muscular dystrophy (DMD) are neuromuscular disorders that affect muscular function. The most common causes of morbidity and mortality are respiratory complications, including restrictive lung disease, ineffective cough, and sleep-disordered breathing. The paradigm of care is changing as new disease-modifying therapies are altering disease trajectory, outcomes, expectations, as well as patient and caregiver experiences. This article provides an overview on therapeutic advances for SMA and DMD in the last 10 years, with a focus on the effects of disease-modifying therapies on respiratory function., Competing Interests: Disclosure Dr R. Amin is a committee member of the American College of Chest Physicians and a pediatrics committee member of the American Thoracic Society. R. Amin holds research grants from Canadian Institutes of Health Research, Canada, Cure SMA Canada, Canada, Muscular Dystrophy Canada, Canada, VHA Home Healthcare, Canada, Boehringer-Ingelheim, Germany, Medigas, Canada, ProResp, Canada, Baxter Corporation Endowment Fund for Home Care, United States, and Ontario Ministry of Health and Long-Term Care, Canada. Dr L. Xiao is a committee member of the American College of Chest Physicians. Dr. Xiao holds research grants from Muscular Dystrophy Canada, Baxter Corporation Endowment Fund for Home Care, International Pediatric Sleep Association, United States, and the Sleep Research Society Foundation, United States., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Respiratory sequelae are a frequent cause of morbidity and mortality in children with NMD. Impaired cough strength and resulting airway clearance as well as sleep disordered breathing are the two main categories of respiratory sequelae. Routine clinical evaluation and diagnostic testing by pulmonologists is an important pillar of the multidisciplinary care required for children with NMD. Regular surveillance for respiratory disease and timely implementation of treatment including pulmonary clearance techniques as well as ventilation can prevent respiratory related morbidity including hospital admissions and improve survival. Additionally, novel disease modifying therapies for some NMDs are now available which has significantly improved the clinical trajectories of patients resulting in a paradigm shift in clinical care. Pulmonologists are 'learning' the new natural history for these diseases and adjusting clinical management accordingly., Competing Interests: Disclosure The authors have indicated they have no potential conflicts of interest to disclose., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Rationale: Information is limited about the association between obstructive sleep apnea (OSA) and mental health disorders in children. Objectives: In children, 1 ) to evaluate the association between OSA and new mental healthcare encounters; and 2 ) to compare mental healthcare encounters 2 years after to 2 years before OSA treatment initiation. Methods: We conducted a retrospective longitudinal cohort study using Ontario health administrative data (Canada). Children (0-18 yr) who underwent diagnostic polysomnography (PSG) 2009-2016 and met criteria for definition of moderate-severe OSA (PSG-OSA) were propensity score weighted by baseline characteristics and compared with children who underwent PSG in the same period but did not meet the OSA definition (PSG-No-OSA). Children were followed until March 2021. Weighted cause-specific Cox proportional hazards and modified Poisson regression models were used to compare time from PSG to first mental healthcare encounter and frequency of new mental healthcare encounters per person time, respectively. Among those who underwent adenotonsillectomy (AT) or were prescribed and claimed positive airway pressure therapy (PAP), we used age-adjusted conditional logistic regression models to compare 2 years post-treatment to pretreatment odds of mental healthcare encounters. Results: Of 32,791 children analyzed, 7,724 (23.6%) children met criteria for moderate-severe OSA. In the PSG-OSA group, 7,080 (91.7%) were treated (AT or PAP). Compared with PSG-No-OSA, the PSG-OSA group had a shorter time from PSG to first mental healthcare encounter (hazard ratio, 1.08; 95% confidence interval [CI], 1.05-1.12) but less frequent mental healthcare encounters in follow-up (rate ratio, 0.92; 95% CI, 0.87-0.97). OSA treatment (AT or PAP) was associated with lower odds of mental healthcare encounters 2 years after treatment initiation compared with 2 years before (odds ratio, 0.69; 95% CI, 0.65-0.74). Conclusions: In this large, population-based study of children who underwent PSG for sleep disorder assessment, OSA diagnosis/treatment was associated with an improvement in some mental health indicators, such as fewer new mental healthcare encounters compared with no OSA and lower odds of mental healthcare encounters compared with before OSA treatment.

Cardioneuroablation has emerged as a potential alternative to cardiac pacing in selected cases with vasovagal reflex syncope, extrinsic vagally induced sinus bradycardia-arrest or atrioventricular block. The technique was first introduced decades ago, and its use has risen over the past decade. However, as with any intervention, proper patient selection and technique are a prerequisite for a safe and effective use of cardioneuroablation therapy. This document aims to review and interpret available scientific evidence and provide a summary position on the topic., Competing Interests: Conflict of interest: none declared., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Study Objectives: Our aim was to characterize the 14 and 6 like spike wave activity seen on electroencephalograms (EEG) in children with Prader-Willi syndrome (PWS) undergoing polysomnograms., Methods: We performed a retrospective review of children with PWS and healthy controls who underwent diagnostic polysomnograms between January 1, 2007, and December 31, 2020, at SickKids, Toronto, Canada. EEGs from the polysomnograms were reviewed for the presence of the 14 and 6 like spike wave activity and its characteristics. Clinical correlation of the EEG variant with sleep-disordered breathing indices from the polysomnograms was also evaluated., Results: A total of 94 children with PWS and 50 healthy controls were included. The median age and interquartile range for the cohort was 1.42 (0.6, 4.2) years. There were 50 (53.2%) males in the PWS cohort. The EEG variant prevalence in this cohort was 51.0% (n = 48) in children with PWS and 0% for the healthy controls. 14 and 6 Hz like spike wave activity was bilateral in 52% (25/48) children with PWS. The waves had a negative deflection in almost all patients, 44/48 (92%), with PWS. It was predominantly located in the frontal leads for children with PWS, 23/48 (47.9%). It most frequently occurred during non-rapid eye movement stage 2 sleep for children with PWS, 25/48 (52.0%). The mean (standard deviation) frequency was 6.8 (0.97) Hz. The median (interquartile range) length of the waves was 1.1 (0.8, 1.4) seconds in children with PWS. There was no correlation between the presence of the EEG variant and sleep-disordered breathing indices in children with PWS., Conclusions: The 14 and 6 Hz like spike wave activity EEG variant was present in more than 50% of a pediatric cohort of children with PWS compared with 0% in healthy children. This EEG variant did not appear to be associated with sleep-disordered breathing indices in children with PWS and is of unknown clinical significance., Citation: Alzaid M, Sunkonkit K, Massicotte C, Otsubo H, Amin R, Al-Saleh S. 14 and 6 Hz like spike wave activity is a common finding in young patients with Prader-Willi syndrome. J Clin Sleep Med . 2024;20(8):1227-1232., (© 2024 American Academy of Sleep Medicine.)

Rationale: Home mechanical ventilation (HMV) is an advanced medical therapy offered to children with medical complexity. Despite the growing pediatric HMV population in North America, there are limited studies describing healthcare utilization and predictors of highest costs using robust health administrative data., Objectives: To describe patterns of healthcare utilization and costs in children receiving HMV over a 14-year period in Ontario, Canada., Methods: We conducted a retrospective population-based cohort study (April 1, 2003 to March 31, 2017) of children aged 0-18 years receiving HMV via invasive mechanical ventilation (IMV) or non-invasive ventilation (NIV). Paired t-tests compared healthcare system utilization and costs two years before and two years after HMV approval. We developed linear models to analyze variables associated with children in the top quartile of health service utilization and costs., Results: We identified 835 children receiving HMV. In the two years after HMV approval compared to the 2 years prior, children had decreased hospitalization days (median 9 (IQR 3-30) versus 29 (6-99), p<0.0001) and ICU admission days (6.6 (1.9-18.0) versus 17.1 (3.3-70.9), p<0.0001) but had increased homecare service approvals (195 (24-522) versus 40 (12-225), p<0.0001) and outpatient Pulmonology visits (3 (1-4) versus 2 (1-3), p<0.0001). Total healthcare costs were higher in the two years after HMV approval (mean $164,892 (SD $214,187) versus $128,941 ($194,199), p<0.0001). However, all-cause hospital admission costs were reduced ($66,546 ($142,401) versus $81,578 ($164,672), p<0.0001). Highest total 2-year costs were associated with IMV (OR 3.45, 95% CI 2.24-5.31; reference NIV), number of medical devices at home (OR 1.63, 95% CI 1.35-1.96; reference no technology), and increased healthcare costs in the year prior to HMV initiation (OR 2.23, 95% CI 1.84-2.69)., Conclusions: Children progressing to the need for HMV represent a worsening in their respiratory status that will undoubtedly increase healthcare utilization and costs. We found that the initiation of HMV in these children can reduce inpatient healthcare utilization and costs but can still increase overall healthcare expenditures, especially in the outpatient setting.

Introduction: Obstructive sleep apnea (OSA) is an important and evolving area in the pediatric population, with significant sequelae when not adequately managed. The use of positive airway pressure (PAP) therapy is expanding rapidly and is being prescribed to patients with persistent OSA post adenotonsillectomy as well as those children who are not surgical candidates including those with medical complexity., Areas Discussed: This article provides a state-of-the-art review on the diagnosis of pediatric OSA and treatment with positive airway pressure (PAP). The initiation of PAP therapy, pediatric interface considerations, PAP mode selection, administration and potential complications of PAP therapy, factors influencing PAP adherence, the use of remote ventilation machine downloads, considerations surrounding follow-up of patients post PAP initiation and evaluation of weaning off PAP will be reviewed. The literature search was conducted via PubMed, Cochrane Library and Google Scholar databases through to March 2024., Expert Opinion: Further research is required to address barriers to adherence. Further innovation of home monitoring devices for both the diagnosis and assessment of OSA is required, given the limited pediatric sleep medicine resources in several countries worldwide.

Background: Nasogastric tube (NGT) feeding is used for children unable to tolerate adequate nutrition orally to sustain growth and development. This vulnerable population is at risk of gaps in care because they often lack a medical home due to the transitional nature of the technology. This study explores perspectives and challenges of family caregivers (FCs) of children requiring NGTs transitioning from hospital to home., Methods: Semi-structured qualitative interviews were conducted with FCs at the Hospital for Sick Children and Children's Hospital of Eastern Ontario. Research ethics approval was obtained (SK REB# 1000064641, CHEO REB# 19/133X). Written informed consent was obtained., Results: Thirteen interviews revealed FCs feeling overwhelmed and uncomfortable with first communication of the NGT but learned to manage NGTs with training and virtual care support over time. Initial transition home was described as challenging due to physical, emotional, and financial strain associated with constant management of NGTs., Conclusions: Our study describes the importance of emotional support and additional time for decision making during initial communication to FCs of their child's NGT need, and access to specialized healthcare professionals after transitioning home. Future programs should focus on personalized education and psychosocial support for FCs of children with NGTs at home., Impact: This study delves into the challenges faced by family caregivers (FCs) of children requiring nasogastric tube (NGT) feeding when returning home from the hospital. There is a pressing need for more time for FC decision-making and emotional support during the initial communication of the need for an NGT for their child. In addition, FCs require ongoing 24/7 support including access to healthcare professionals specialized in NGT care after the initial transition home. The study highlights the need for personalized education and psychosocial supports for FCs of children with NGTs to improve their experiences at home., (© 2024. The Author(s), under exclusive licence to the International Pediatric Research Foundation, Inc.)

Competing Interests: The authors have no conflicts of interest to disclose.

Purpose: Tonsillectomy is one of the most common surgical procedures performed in children. Since most clinical practice guidelines (CPGs) are designed to support surgical decisions, none are specifically designed for the perioperative management of children undergoing tonsillectomy. We aimed to identify and analyze the existing CPGs with recommendations for the perioperative management of children undergoing tonsillectomy by conducting a systematic review., Source: We searched Embase, MEDLINE, MEDLINE ePub Ahead of Print, and CINAHL for relevant articles published from inception to 3 August 2022. The inclusion criteria were: 1) CPG of perioperative recommendations for tonsillectomy under general anesthesia in children, 2) CPG that include at least one evidence-based recommendation, 3) peer-reviewed CPG published in English after 2000. We extracted data on baseline characteristics of each CPG and general recommendations for perioperative interventions or complications., Principal Findings: Out of five eligible CPGs, AGREE II and REX confirmed that two CPGs were high quality while only one of the two was recommended for implementation without modifications. Most of the recommendations were for pain management. Acetaminophen was the only medication recommended in all five CPG. Except for the oldest CPG, the CPG all supported of the use of nonsteroidal anti-inflammatory drugs and steroids as a pain adjunct., Conclusions: Acetaminophen, nonsteroidal anti-inflammatory drugs, and steroids are recommended in the perioperative management of pediatric tonsillectomy. Future CPG should further clarify the safe use of opioids based on severity of obstructive sleep apnea and in the context of opioid-sparing techniques, such as dexmedetomidine, high-dose dexamethasone, and gabapentinoids., Study Registration: PROSPERO (CRD42021253374); first submitted 18 June 2021., (© 2024. Canadian Anesthesiologists' Society.)

Background: Clinical management of ventilator-assisted individuals (VAIs) was challenged by social distancing rules during the COVID-19 pandemic. In May 2020, the Long-Term In-Home Ventilator Engagement (LIVE) Program was launched in Ontario, Canada to provide intensive digital care case management to VAIs. The purpose of this qualitative study was to explore the acceptability of the LIVE Program hosted via a digital platform during the COVID-19 pandemic from diverse perspectives., Methods: We conducted a qualitative descriptive study (May 2020-April 2021) comprising semi-structured interviews with participants from eight home ventilation specialty centers in Ontario, Canada. We purposively recruited patients, family caregivers, and providers enrolled in LIVE. Content analysis and the theoretical concepts of acceptability, feasibility, and appropriateness were used to interpret findings., Results: A total of 40 individuals (2 VAIs, 18 family caregivers, 20 healthcare providers) participated. Participants described LIVE as acceptable as it addressed a longstanding imperative to improve care access, ease of use, and training provided; feasible for triaging problems and sharing information; and appropriate for timeliness of provider responses, workflows, and perceived value. Negative perceptions of acceptability among healthcare providers concerned digital workload and fit with existing clinical workflows. Perceived benefits accorded to LIVE included enhanced physical and psychological safety in the home, patient-provider relations, and VAI engagement in their own care., Conclusions: Study findings identify factors influencing the LIVE Program's acceptability by patients, family caregivers, and healthcare providers during pandemic conditions including enhanced access to care, ease of case management triage, and VAI safety. Findings may inform the implementation of digital health services to VAIs in non-pandemic circumstances., Competing Interests: The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2024.)

Aim: The aim of the present study was to assess the stain removal ability and color stability of three distinct dentifrices on artificially stained enamel surface., Materials and Methods: This study included 75 intact, healthy premolars free of dental caries that were extracted during orthodontic therapy. The samples were allowed to dry for 6 hours after being submerged in the prepared tea solution for roughly 18 hours every day. Then this procedure was repeated for seven successive days. All samples were randomly divided into three experimental groups with 25 samples in each group. Group I: control dentifrice, group II: dentifrice containing hydrogen peroxide, group III: dentifrice containing papain and bromelain. A specially designed toothbrushing simulator was used to brush every sample in the relevant group. Using a spectrophotometer and a measurement program, color measurement was evaluated after staining process after 4 weeks and 8 weeks of teeth cleaning. Using a profilometer, the surface roughness values (Ra) were assessed., Results: After 8 weeks of brushing of stained samples, the color stability was better in dentifrice containing hydrogen peroxide (1.14 ± 0.11) followed by dentifrice containing papain and bromelain (1.22 ± 0.08) and control group (1.30 ± 0.09). And after 8 weeks of brushing of stained samples, the surface roughness was more in dentifrice containing hydrogen peroxide (0.237 ± 0.02) followed by dentifrice containing papain and bromelain (0.229 ± 0.13) and control group (0.207 ± 0.05)., Conclusion: The present study concluded that the dentifrice containing hydrogen peroxide showed a superior whitening effect on the stained enamel surface than dentifrice containing papain and bromelain and control dentifrice., Clinical Significance: The development of various dentifrice products has been greatly aided by the increased demand for an improved esthetic appearance. Teeth's natural color and any external stains that could accumulate on the tooth surface combine to determine a tooth's color. Additionally, the use of whitening dental pastes to remove external stains has grown in favor. With the development of these whitening toothpastes, dentifrices' ability to lessen or eliminate extrinsic dental stains has increased. How to cite this article: Mishra D, Kamath DG, Alagla M, et al . Evaluation of Stain Removal Efficacy and Color Stability of Three Different Dentifrices on Artificially Stained Enamel Surface-An In Vitro Study. J Contemp Dent Pract 2024;25(1):68-71.

There has been increasing interest in utilizing volume assured pressure support (VAPS) modes of ventilation for children, which historically had only been favored in adult populations. In addition to patients with obesity hypoventilation syndrome, newer pediatric populations for which it has recently been prescribed include congenital central hypoventilation syndrome and children with neuromuscular disease such as Duchenne muscular dystrophy and spinal muscular atrophy. Given its expanding use in pediatrics, greater familiarity with VAPS is essential for pediatric pulmonologists and sleep physicians. This review article will highlight methods of initiation for this mode, specific ventilator settings, discussion of suitable pediatric patient populations, ventilator titrations via formal polysomnograms and detailed ventilator data downloads specific interpretation. Finally, common challenges to be aware of and how to troubleshoot relevant machine alarms will be reviewed., (© 2023 Wiley Periodicals LLC.)

Aim: The purpose of the present study was to evaluate the impact of various chemical solvents on bond strength of orthodontic brackets., Materials and Methods: One hundred healthy human premolars with undamaged buccal surfaces that were extracted for orthodontic purposes were gathered. Using 37% orthophosphoric acid, primer, and Transbond XT adhesive, ceramic 0.018" × 0.022" slot orthodontic brackets were adhered to the tooth surface. Following thermocycling, all samples were divided into four groups, with 25 samples in each group: group I: control; group II: application of ethanol; group III: application of acetone; and group IV: application of dimethyl sulfoxide (DMSO). Following the debonding tests, a double-ocular stereomicroscope was used to inspect the tooth surfaces. Additionally, adhesive remnant index (ARI) values were evaluated at 40× magnification. Data were recorded and statistically analyzed., Results: The bond strength was lesser in acetone applied group (16.18 ± 3.64) followed by DMSO applied group (22.08 ± 2.86), ethanol applied group (24.36 ± 4.02), and control group (27.14 ± 3.68). There was a highly significant difference found between the chemical solvents group. The ARI score 3 was present in control (12%), ethanol (8%), and DMSO (4%), and it was absent in acetone applied group. The ARI score 0 was more in acetone applied group (24%)., Conclusion: The present study concluded that the reduced debonding force was found with the application of acetone solvent followed by DMSO, ethanol, and control groups. Applying acetone can be a substitute technique to help with ceramic bracket debonding., Clinical Significance: Orthodontic bracket debonding cannot occur without shear bond strength (SBS). The need for an ideal debonding technique for ceramic brackets without negative consequences arises from the risk of enamel damage that frequently follows the process. Acetone treatment prior to ceramic bracket debonding could be an alternate clinical technique to preventing enamel damage and facilitating debonding. How to cite this article: Bhushan R, Singh S, Sam G, et al . Effect of Different Chemical Solvents on Bond Strength of Orthodontic Brackets: An In Vitro Study. J Contemp Dent Pract 2023;24(12):940-943.

Background: The COVID-19 pandemic led to an unprecedented need for virtual healthcare that was safe, acceptable and feasible to deliver. In May 2020, we launched the Long-term In-Home Ventilator Engagement (LIVE) program for ventilator assisted individuals using ventilators hosted on an e-platform in Ontario, Canada., Objectives: To assess the acceptability, appropriateness, feasibility and usability of the LIVE program reported by patients, family caregivers, and healthcare providers (HCP)., Design and Methods: We conducted a cross-sectional study. We provided HCPs participating in the LIVE program anonymized questionnaires (Acceptability of Intervention Measure (AIM), Intervention Appropriateness Measure (IAM), Feasibility of Intervention Measure (FIM), and mHealth App Usability (MAUQ). Patients and family caregivers completed the AIM and MAUQ. Questionnaires were administered via an e-platform., Results: We recruited 105/251 (42%) patients and family caregivers and 42/48 (87.5%) HCPs. Patients and caregivers rated a mean (SD) overall AIM score of 4.3 (0.7) (maximum score 5; higher scores indicate greater acceptability) and a mean (SD) overall MAUQ score of 5.8 (1.5) (maximum score 7; higher scores indicate greater useability). HCPs rated a mean (SD) overall AIM score of 4.3 (0.7), IAM score of 4.3 (0.8), FIM score of 4.2 (0.7) and overall MAUQ score of 5.6 ± 1.5. There were no differences in AIM ((4.3 (0.7) vs 4.3 (0.8), p  = 1) or MAUQ (5.8 (1.5) vs 5.6 (1.5), p  = 0.5) scores between patients/ family caregivers and HCPs., Interpretation: This study suggests that the LIVE program was acceptable, appropriate, feasible, and usable from the perspective of patients, family caregivers and HCPs., Competing Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2023.)

Objective: Spinal muscular atrophy (SMA) is a neuromuscular disorder that manifests with motor deterioration and respiratory complications. The paradigm of care is shifting as disease-modifying therapies including nusinersen, onasemnogene abeparvovec and risdiplam alter the disease trajectory of SMA. The objective of this study was to explore caregivers' experiences with disease-modifying therapies for SMA., Design: Qualitative study including semistructured interviews with caregivers of children with SMA who received disease-modifying therapies. Interviews were audio recorded, transcribed verbatim, coded and analysed using content analysis., Setting: The Hospital for Sick Children (Toronto, Canada)., Results: Fifteen family caregivers of children with SMA type 1 (n=5), type 2 (n=5) and type 3 (n=5) participated. There were two emerging themes and several subthemes (in parentheses): (1) inequities in access to disease-modifying therapies (variable regulatory approvals, prohibitively expensive therapies and insufficient infrastructure) and (2) patient and family experience with disease-modifying therapies (decision making, hope, fear and uncertainty)., Conclusion: The caregiver experience with SMA has been transformed by the advent of disease-modifying therapies. Consistent and predictable access to disease-modifying therapies is a major concern for caregivers of children with SMA but is influenced by regulatory approvals, funding and eligibility criteria that are heterogenous across jurisdictions. Many caregivers described going to great lengths to access therapies, highlighting issues related to justice, such as equity and access. This diverse population reflects contemporary patients and families with SMA; their broad experiences may inform the healthcare delivery of other emerging orphan drugs., Competing Interests: Competing interests: RA is a committee member of the American College of Chest Physicians and a paediatrics committee member of the American Thoracic Society, and holds research grants from Canadian Institutes of Health Research, Cure SMA Canada, Muscular Dystrophy Canada, VHA Home Healthcare, Boehringer-Ingelheim, Medigas, ProResp, Baxter Corporation Endowment Fund for Home Care, and Ontario Ministry of Health and Long-term Care. LX reported receiving research funding from The Hospital for Sick Children Clinician-Scientist Training Program, American Thoracic Society ASPIRE fellowship, Baxter Corporation Endowment Fund for Home Care, International Pediatric Sleep Association, and the Sleep Research Society Foundation during the conduct of the study., (© Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Objectives: To determine outcomes following adenotonsillectomy for obstructive sleep apnea (OSA) and the impact of motor and swallowing impairment on respiratory complications in children with Cerebral Palsy (CP)., Methods: A retrospective review of children with CP and sleep disordered breathing (SDB) who underwent adenotonsillectomy (2003-2021) was performed. Children with CP were age-matched to children without CP. Motor and swallowing function was assessed using the Gross Motor Functional Classification System (GMFCS) and the Eating and Drinking Ability Classification System (EDACS). The primary outcome was postoperative obstructive apnea-hypopnea index (OAHI). Secondary outcomes were cure rate, complications, and need for additional interventions., Results: Ninety-seven children with CP were assessed for SDB, and 74 underwent polysomnography. Moderate or severe OSA was found in 49% (36/74). Adenotonsillectomy was performed in 30% (29/97). All children who underwent adenotonsillectomy experienced an initial reduction in OAHI (31.7/h to 2.9/h, p < 0.0001). Children with CP were less likely to achieve an OAHI<1 compared with children without CP (62.5% vs 81.8%, p = 0.23). Children with CP had more postoperative complications (43.5% vs. 8.7%) and greater odds of respiratory complications compared with children without CP (OR 8.9 95% CI 2.1-37.9). Children with CP and a GMFCS score of 5 and EDACS score between 3 and 5 had more respiratory complications post-adenotonsillectomy compared to those with GMFCS<5 (p = 0.002) and EDACS<3 (p = 0.031)., Conclusion: Children with CP had an improved OAHI initially following adenotonsillectomy but had higher rates of post-adenotonsillectomy complications. Respiratory complications after adenotonsillectomy were more common in children with motor and swallowing impairment. Findings may provide better preoperative planning for caregivers., Competing Interests: Declaration of competing interest None., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Background: Spinal muscular atrophy (SMA) is a genetic disorder that may result in neuromuscular weakness and respiratory insufficiency. Gene replacement therapy has changed the trajectory of this condition, but long-term outcomes related to sleep disordered breathing are not known., Methods: This was a retrospective review of infants with SMA identified via newborn screening who subsequently received onasemnogene abeparvovec at the Hospital for Sick Children (Ontario, Canada). Polysomnograms were conducted at the time of confirmed diagnosis as well as regularly thereafter., Results: Eleven children (4 female) were identified via newborn screen (7 with 2 copies of the SMN2 gene and 4 with 3 copies of the SMN2 gene) and received onasemnogene abeparvovec at a median age of 3.6 weeks. All eleven infants met criteria for sleep disordered breathing based on their first completed polysomnograms but improved over time. Three infants required respiratory technology, including a premature infant who was prescribed nocturnal supplemental oxygen therapy for central sleep apnea and two symptomatic infants with neuromuscular weakness who required nocturnal noninvasive ventilation. We did not find a correlation between motor scores and polysomnogram parameters., Conclusion: Children treated with onasemnogene abeparvovec have reduced sleep disordered breathing over time. Polysomnograms revealed abnormal parameters in all children, but the clinical significance of these findings was unclear for children who were asymptomatic for sleep disordered breathing or neuromuscular weakness. These results highlight the need to evaluate both motor scores and respiratory symptoms to ensure a holistic evaluation of clinical status., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Objectives: This qualitative descriptive study explores the experiences of family caregivers (FCs) of children with medical complexity who are initiated on new medical technology in the hospital and transition to new daily life at home. The study aims to investigate FCs' response and readiness for medical technology use, the value of education and transition support and the challenges associated with managing new medical technology in the home., Study Design: A qualitative descriptive approach was used to conduct and analyse 14 semistructured interviews with a group of FCs composed of 11 mothers and 3 fathers. Content analysis was used to analyse transcripts of the caregiver interviews. The study was conducted at a tertiary paediatric hospital in Toronto, Canada., Results: Our study revealed three main themes: FC's response and readiness for medical technology use, the value of education and transition support for initiation of new medical technology and the challenges associated with managing new medical technology in the home. FCs expressed emotional distress related to coping with the realisation that their child required medical technology. Although the theoretical and hands-on practice training instilled confidence in families, FCs reported feeling overwhelmed when they transitioned home with new medical technology. Finally, FCs reported significant psychological, emotional and financial challenges while caring for their technology-dependent child., Conclusions: Our study reveals the unique challenges faced by FCs who care for technology-dependent children. These findings highlight the need to implement a comprehensive education and transition programme that provides longitudinal support for all aspects of care., Competing Interests: Competing interests: No, there are no competing interests., (© Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Polysomnography (PSG) is the gold standard for the diagnosis of pediatric sleep-disordered breathing (SDB). However, the literature characterizing the indications for inpatient PSGs and the impact on clinical decision-making is limited., Objective: To determine the indications, results, and outcomes for children undergoing inpatient PSGs at our institution., Methods: We performed a retrospective review of children aged 0-18 years who underwent inpatient diagnostic PSGs between July 2018 and July 2021 at SickKids, Toronto, Canada. Baseline characteristics, indications, and management were reviewed and characterized by descriptive statistics., Results: Eighty-eight inpatient PSGs were performed in 75 children (male 62.7%). Median (interquartile range) age and body mass index z-score were 1.5 (0.2, 10.8) years and 0.27 (-1.58, 2.66), respectively. The most common indication for inpatient PSG was initiation and titration of ventilation (n = 34/75, 45.3%). Of the 75 children, 48 (64%) had multiple complex chronic conditions (CCCs). Sixty children (80%) underwent a baseline PSG for either the entire night or a portion of the night. Of these studies, 54 (90%) had clinically significant SDB of which isolated obstructive sleep apnea (OSA; 17/60, 28.3%) was the most common. The following management was undertaken for the 54 patients with SDB; respiratory technology (88.9%), surgical intervention (31.5%), positional therapy (1.9%), intranasal steroids (3.7%), and no further intervention (5.6%), respectively., Conclusions: Our study highlights that inpatient PSG was an important diagnostic tool resulting in directed medical and surgical management. Future multicenter studies are needed to compare indications for inpatient PSGs across institutions to develop evidence-based clinical practice guidelines., (© 2023 Wiley Periodicals LLC.)

Background: Respiratory failure is a significant concern in neuromuscular diseases (NMDs). This CHEST guideline examines the literature on the respiratory management of patients with NMD to provide evidence-based recommendations., Study Design and Methods: An expert panel conducted a systematic review addressing the respiratory management of NMD and applied the Grading of Recommendations, Assessment, Development, and Evaluations approach for assessing the certainty of the evidence and formulating and grading recommendations. A modified Delphi technique was used to reach a consensus on the recommendations., Results: Based on 128 studies, the panel generated 15 graded recommendations, one good practice statement, and one consensus-based statement., Interpretation: Evidence of best practices for respiratory management in NMD is limited and is based primarily on observational data in amyotrophic lateral sclerosis. The panel found that pulmonary function testing every 6 months may be beneficial and may be used to initiate noninvasive ventilation (NIV) when clinically indicated. An individualized approach to NIV settings may benefit patients with chronic respiratory failure and sleep-disordered breathing related to NMD. When resources allow, polysomnography or overnight oximetry can help to guide the initiation of NIV. The panel provided guidelines for mouthpiece ventilation, transition to home mechanical ventilation, salivary secretion management, and airway clearance therapies. The guideline panel emphasizes that NMD pathologic characteristics represent a diverse group of disorders with differing rates of decline in lung function. The clinician's role is to add evaluation at the bedside to shared decision-making with patients and families, including respect for patient preferences and treatment goals, considerations of quality of life, and appropriate use of available resources in decision-making., (Copyright © 2023 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.)

Objective/background: Moderate-to-severe obstructive sleep apnea (OSA) is highly prevalent in children with obesity and/or underlying medical complexity. The first line of therapy, adenotonsillectomy (AT), does not cure OSA in more than 50% of these children. Consequently, continuous positive airway pressure (CPAP) is the main therapeutic option but adherence is often poor. A potential alternative which may be associated with greater adherence is heated high-flow nasal cannula (HFNC) therapy; however, its efficacy in children with OSA has not been systematically investigated. The study aimed to compare the efficacy of HFNC with CPAP to treat moderate-to-severe OSA with the primary outcome measuring the change from baseline in the mean obstructive apnea/hypopnea index (OAHI)., Participants/methods: This was a single-blinded randomized, two period crossover trial conducted from March 2019 to December 2021 at a Canadian pediatric quaternary care hospital. Children aged 2-18 years with obesity and medical complexity diagnosed with moderate-to-severe OSA via overnight polysomnography and recommended CPAP therapy were included in the study. Following diagnostic polysomnography, each participant completed two further sleep studies; a HFNC titration study and a CPAP titration study (9 received HFNC first, and 9 received CPAP first) in a random 1:1 allocation order., Results: Eighteen participants with a mean ± SD age of 11.9 ± 3.8 years and OAHI 23.1 ± 21.7 events/hour completed the study. The mean [95% CI] reductions in OAHI (-19.8[-29.2, -10.5] vs. -18.8 [-28.2, -9.4] events/hour, p = 0.9), nadir oxygen saturation (7.1[2.2, 11.9] vs. 8.4[3.5, 13.2], p = 0.8), oxygen desaturation index (-11.6[-21.0, -2.3] vs. -16.0[-25.3, -6.6], p = 0.5) and sleep efficiency (3.5[-4.8, 11.8] vs. 9.2[0.9, 15.5], p = 0.2) with HFNC and CPAP therapy were comparable between conditions., Conclusion: HFNC and CPAP therapy yield similar reductions in polysomnography quantified measures of OSA severity among children with obesity and medical complexities., Trial Registration: NCT05354401 ClinicalTrials.gov., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Introduction/aims: Non-invasive ventilation (NIV) is routinely prescribed to support the respiratory system in Duchenne muscular dystrophy (DMD) patients; however, factors improving NIV usage are unclear. We aimed to identify predictors of NIV adherence in DMD patients., Methods: This was a multicenter retrospective analysis of DMD patients prescribed NIV and followed at (1) The Hospital for Sick Children, Canada; (2) Rady Children's Hospital San Diego, USA; and (3) University of California San Diego Health, USA, between February 2016 and October 2020. The primary and secondary outcomes were 90-day period NIV adherence and clinical and socioeconomic predictors of NIV adherence., Results: We identified 59 DMD patients prescribed NIV (mean ± SD age = 20.1 ± 6.7 y). Overall, percentage of nights used, and average nightly usage, were 79.9 ± 31.1% and 7.23 ± 4.12 h, respectively. Compared with children, adults had higher percentage of nights used (92.9 ± 16.9% vs. 70.4 ± 36.9%; P < .05), and average nightly usage (9.5 ± 4.7 h vs. 5.3 ± 3.7 h; P < .05). Non-English language (P = .01), and absence of deflazacort prescription (P = .02) were significantly associated with higher percentage of nights used while Hispanic ethnicity (P = .01), low household income (P = .02), and absence of deflazacort prescription (P = .02) were significantly associated with higher nightly usage. Based on univariable analysis, older age and declining forced vital capacity were associated with increased percentage of nights used and increased average nightly usage., Discussion: Certain clinical and socioeconomic determinants had a significant impact on NIV adherence in DMD patients, providing insight into those at risk for high versus low compliance with respiratory therapy., (© 2023 The Authors. Muscle & Nerve published by Wiley Periodicals LLC.)

Objective: The primary objective was to quantify psychosocial risk in family caregivers (FCs) of children with medical complexity (CMC) during the COVID-19 pandemic using the Psychosocial Assessment Tool (PAT). The secondary objectives were to compare this finding with the average PAT score of this population before the COVID-19 pandemic and to examine potential clinical predictors of psychosocial risk in FCs of CMC., Design: Cross-sectional study., Participants: FCs of CMC were recruited from the Long-Term Ventilation Clinic at The Hospital for Sick Children, Toronto, Ontario, Canada. A total of 91 completed the demographic and PAT questionnaires online from 10 June 2021 through 13 December 2021., Main Outcome Measures: Mean PAT scores in FCs were categorised as 'Universal' low risk, 'Targeted' intermediate risk or 'Clinical' high risk. The effect of sociodemographic and clinical variables on overall PAT scores was assessed using multiple linear regression analysis. Comparisons with a previous study were made using Mann-Whitney tests and χ 2 analysis., Results: Mean (SD) PAT score was 1.34 (0.69). Thirty-one (34%) caregivers were classified as Universal, 43 (47%) as Targeted and 17 (19%) as Clinical. The mean PAT score (1.34) was significantly higher compared with the mean PAT score (1.17) found prior to the COVID-19 pandemic. Multiple linear regression analysis demonstrated an overall significant model, with the number of hospital admissions since the onset of COVID-19 being the only variable associated with the overall PAT score., Conclusion: FCs of CMC are experiencing significant psychosocial stress during the COVID-19 pandemic. Timely and effective interventions are warranted to ensure these individuals receive the appropriate support., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Background and Objectives: Children with medical complexity have been disproportionately impacted by the COVID-19 pandemic and the associated changes in healthcare delivery. The primary objective of this study was to gain a thorough understanding of the lived experiences of family caregivers of children with medical complexity during the pandemic., Methods: We conducted semi-structured interviews with family caregivers of children with medical complexity from a tertiary pediatric hospital. Interview questions focused on the aspects of caregiving for children with medical complexity, impact on caregiver mental and physical well-being, changes to daily life secondary to the pandemic, and experiences receiving care in the healthcare system. Interviews were conducted until thematic saturation was achieved. Interviews were audio recorded, deidentified, transcribed verbatim, coded and analyzed using content analysis., Results: Twelve semi-structured interviews were conducted. The interviews revealed three major themes and several associated subthemes: (1) experiences with the healthcare system amid the pandemic (lack of access to healthcare services and increased hospital restrictions, negative clinical interactions and communication breakdowns, virtual care use); (2) common challenges during the pandemic (financial strain, balancing multiple roles, inadequate homecare nursing); and (3) the pandemic's impact on family caregiver well-being (mental toll, physical toll)., Conclusions: Family caregivers of children with medical complexity experienced mental and physical burden due to the intense nature of their caregiving responsibilities that were exacerbated during the pandemic. Our results highlight key priorities for the development of effective interventions to support family caregivers and their children., (© 2023. The Author(s).)

Objectives: The incidence, as well as the predictors of mortality, for children receiving home mechanical ventilation (HMV) using population-based data in Canada is a current knowledge gap. Our objectives were to describe HMV incidence and mortality rates, and associations of demographic and clinical variables on mortality., Methods: Using Ontario health and demographic administrative databases, we conducted a retrospective cohort study (April 1, 2003-March 31, 2017) of children aged 0 to 17 years receiving HMV via invasive mechanical ventilation and noninvasive ventilation. We identified children with complex chronic conditions. We used data from Census Canada to calculate incidence rates and Cox proportional hazards modeling to assess for predictors of mortality., Results: We identified 906 children with a mean (SD) crude incidence rate of 2.4 (0.6) per 100 000 for pediatric HMV approvals that increased by 37% over the 14-year study period. Compared with children who were invasively ventilated, we found mortality was associated with noninvasive ventilation (adjusted hazard ratio [aHR], 1.9; 95% confidence interval [CI], 1.3-2.8). Mortality was highest in children from families in the lowest income quintile (aHR, 2.5; 95% CI, 1.5-4.0), those with neurologic impairment complex chronic conditions (aHR, 2.9; 95% CI, 1.4-6.4), those aged 11 to 17 years at HMV initiation (aHR, 1.5; 95% CI, 1.1-2.0), and those with higher health care costs in the 1 year before HMV initiation (aHR, 1.5; 95% CI, 1.3-1.7)., Conclusions: The incidence of children receiving HMV increased substantially over the 14-year period. Demographic variables associated with increased mortality were identified, suggesting areas requiring greater attention for care providers., (Copyright © 2023 by the American Academy of Pediatrics.)

Introduction: Due to the increased risk of obesity for boys with Duchenne muscular dystrophy (DMD), recent guidelines recommend that dietary intake is carefully managed. Parents play an important role in the development of their child's eating behaviours and patterns. However, despite what is known about the increased risk of obesity for children with DMD, little is known about parental feeding behaviours in this population. The objective of this study was to qualitatively explore the experiences of parents of children with DMD around their child's weight management and understand what influences their feeding behaviours., Methods: This paper reports a secondary data analysis. Semi-structured, individual interviews were conducted and analysed using qualitative description., Results: Thirteen parents were interviewed for the study. Three themes were developed: (1) parent responses to healthcare provider interactions, (2) mixed emotions contributing to feeding approach and (3) variable parenting feeding styles. Within the third theme, two subthemes arose including (1) control and preoccupation and (2) striking a balance., Conclusion: Given the potential impact of higher weights on the progression of DMD, it is important that healthcare providers explore feeding behaviours with families. However, it is essential that healthcare providers consider the impact of these conversations on parents, as well as the broader issues that may place additional pressure on the lives of families., (© 2022 John Wiley & Sons Ltd.)

Chronic respiratory failure is a common endpoint in the loss of respiratory muscle function in patients with progressive neuromuscular disease (NMD). Identifying the onset of hypoventilation is critical to allow for the timely introduction of ventilator support and effectively manage respiratory failure [1-3]. While there are accepted criteria governing the diagnosis of hypoventilation during polysomnography (PSG) [4], there is concern that criteria are insufficient for identifying hypoventilation in the earlier stages of respiratory insufficiency related to NMD. The purpose of this project was to identify more sensitive criteria for identifying hypoventilation., Methods: Fifteen pediatric pulmonologists with broad experience in managing patients with NMD, 10 of whom were board certified in and practice sleep medicine, were assembled and performed a review of the pertinent literature and a two-round Delphi process with 6 domains (Table 1)., Results: Within the 6 domains there were three pertinent items per domain (Table 2). There was clear agreement on findings on history (morning headaches) and pulmonary function testing (FVC < 50% or awake TcCO2 > 45 mmHg) indicating a high concern for nocturnal hypoventilation. There was close agreement on the definitions for nocturnal hypercapnia and hypoxemia. PSG criteria were identified that indicate a patient is likely in the transitional phase from adequate ventilation to hypoventilation., Discussion: We identified a set of clinical criteria that may allow for more sensitive diagnosis of hypoventilation in NMD and earlier initiation of non-invasive ventilation leading to a reduction in the respiratory morbidity in progressive NMD. These criteria need to be further and more broadly validated prospectively to confirm their utility.

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., (© 2022 Wiley Periodicals LLC.)

Objectives: Individuals dependent on long-term mechanical ventilation (LTMV) for their day-to-day living are a heterogenous population who go through several transitions over their lifetime. This paper describes three transitions: 1) institution/hospital to community/home, 2) pediatric to adult care, and 3) active treatment to end-of-life for ventilator-assisted individuals (VAIs)., Methods: A narrative review based on literature and the author's collective practical and research experience. Four online databases were searched for relevant articles. A manual search for additional articles was completed and the results are summarized., Results: Transitions from hospital to home, pediatric to adult care, and to end-of-life for VAIs are complex and challenging processes. Although there are several LTMV clinical practice guidelines highlighting key components for successful transition, there still exists gaps and inconsistencies in care. Most of the literature and experiences reported to date have been in developed countries or geographic areas with funded healthcare systems., Conclusions: For successful transitions, the VAIs and their support network must be front-and-center. There should be a coordinated, systematic, and holistic plan (including a multi-disciplinary team), life-time follow-up, with bespoke consideration of jurisdiction and individual circumstances.

Objectives: To describe the current clinical practice patterns of Canadian pediatric respirologists at pediatric tertiary care institutions regarding chronic tracheostomy tube care and management of home invasive ventilation., Methods: A pediatric respirologist/pediatrician with expertise in tracheostomy tube care and home ventilation was identified at each Canadian pediatric tertiary care center to complete a 59-item survey of multiple choice and short answer questions. Domains assessed included tracheostomy tube care, caregiver competency and home monitoring, speaking valves, medical management of tracheostomy complications, decannulation, and long-term follow-up., Results: The response rate was 100% (17/17) with all Canadian tertiary care pediatric centers represented and heterogeneity of practice was observed in all domains assessed. For example, though most centers employ Bivona™ (17/17) and Shiley™ (15/17) tracheostomy tubes, variability was observed around tube change, re-use, and cleaning practices. Most centers require two trained caregivers (14/17) and recommend 24/7 eyes on care and oxygen saturation monitoring. Discharge with an emergency tracheostomy kit was universal (17/17). Considerable heterogeneity was observed in the timing and use of speaking valves and speech-language assessment. Inhaled anti-pseudomonal antibiotics are employed by most centers (16/17) though the indication, agent, and protocol varied by center. Though decannulation practices varied considerably, the requirement of upper airway patency was universally required to proceed with decannulation (17/17) independent of ongoing ventilatory support requirements., Conclusion: Considerable variability in pediatric tracheostomy tube care practice exists across Canada. These results will serve as a starting point to standardize and evaluate tracheostomy tube care nationally., (© 2022 Wiley Periodicals LLC.)

Purpose: The quality and length of life for boys with Duchenne muscular dystrophy (DMD) has improved due to medical advancements, but obesity has emerged as and may pose a risk to their physical health. Clinical guidelines recommend attention to weight management, but healthcare professionals (HCPs) find implementing them in clinical care challenging. Little information is available about the perspectives of children with DMD and their families around weight management. This study explored the key priorities of children with DMD, their parents, and HCPs who treat them, around weight management., Methods: Qualitative, individual, semi-structured interviews were conducted and analyzed using a qualitative descriptive approach., Results: Participants included parents of children with DMD ( n  = 13), children with DMD ( n  = 10), and HCPs ( n  = 14). Theme one: "Competing priorities between healthcare providers, parents, and boys" contained two sub-themes: (i) Body mechanics and function; and (ii) Psychosocial well-being. Theme two: "The realities of living with Duchenne's Muscular Dystrophy," with subthemes: (i) Striving for normality; (ii) The trajectory of DMD; (iii) The labour associated with DMD., Conclusion: HCPs, parents, and boys have diverging worldviews around weight management, highlighting the importance of integrating the priorities of families into care, even when not aligned with guideline recommendations.IMPLICATIONS FOR REHABILITATIONHCPs must understand the competing priorities in the lives of children with DMD and their families when discussing weight, weight management, and lifestyle changes.Quality of life and living a "normal" life are prioritized by children and families over the surveillance and time demands of lifestyle routines recommended by clinicians for weight management.Weight management recommendations should be based upon the individual needs and priorities of the family.

Context: Children and adults with advanced cardiac or respiratory disease may benefit from specialized palliative care (SPC), but there has been little SPC research in this area., Objectives: To explore pediatric cardiologists' and respirologists' (pediatric clinicians) beliefs about and referral practices to SPC and compare these results to adult cardiologists and respirologists (adult clinicians)., Methods: Pediatric and adult clinicians were sent a survey exploring SPC referral practices and beliefs. Responses were summarized with descriptive statistics. Pediatric and adult clinicians' responses were compared using Pearson's chi-square test., Results: The response rate was 56% (989/1759); 9% (87/989) were pediatric clinicians. Pediatric clinicians were more likely than adult clinicians to be female, work in an academic center, and experience fewer patient deaths (P<0.001). Pediatric clinicians reported better access to SPC clinical nurse specialists, spiritual care specialists and bereavement counselors (P<0.001), while adult clinicians reported better access to palliative care units (P<0.001). Pediatric clinicians referred to SPC earlier, while adult clinicians tended to refer after disease directed therapies were stopped (P<0.001). More than half of all clinicians felt patients had negative perceptions of the phrase "palliative care". Although most clinicians were satisfied with SPC quality (73-82%), fewer adult clinicians were satisfied with SPC availability (74 vs. 47%; P<0.001). Fewer pediatric clinicians felt that SPC prioritized oncology patients (13 vs. 53%; P<0.001)., Conclusion: There are important differences between pediatric and adult clinicians' beliefs about and referral practices to SPC. This may reflect unique features of pediatric diseases, provider characteristics, care philosophies, or service availability., (Copyright © 2022 American Academy of Hospice and Palliative Medicine. Published by Elsevier Inc. All rights reserved.)

Background: The Family Integrated Care (FICare) program adapted for Alberta (AB) level II neonatal intensive care units (NICUs) aims to increase parental involvement and support during their NICU stay. The experience of fathers of preterm infants in a FICare program is currently unknown., Purpose: To describe the experiences of fathers of preterm infants born at 320/7 to 346/7 weeks' gestational age with AB FICare., Methods: A qualitative substudy of a multicenter prospective cluster randomized controlled trial of FICare in 10 level II NICUs across Alberta. Fathers of preterm infants participated in a semistructured interview after discharge when their infants were at least 2 months' corrected gestational age. Journal entries written by fathers while in the NICU from the FICare intervention sites were also collected. Data were analyzed thematically and the interview and journal data were triangulated., Findings: Thirteen fathers (9 from the FICare intervention and 4 from standard care) participated in semistructured interviews and there were 24 journals collected. Seven themes emerged: fear of the unknown, mental preparation, identifying the father's role, parenting with supervision, effective communication, postneonatal intensive care transition, and family life. Fathers enrolled in AB FICare attributed their level of confidence and positive neonatal intensive care experience that continued postdischarge to the care and attention they received during hospitalization., Conclusion: AB FICare may improve experiences for fathers of preterm infants in the NICU with continuation postdischarge. Future research should include designing and evaluating father-specific NICU programs., (Copyright © 2022 The Authors. Published by Wolters Kluwer Health, Inc.)

With the growing prevalence of obesity in the pediatric population, reports of its severe complications are increasing. Obesity hypoventilation syndrome is an uncommon disorder in children with altered respiratory mechanics, sleep-disordered breathing, and impaired ventilatory responses leading to persistent hypercapnia. Presentation is varied, and children may remain relatively asymptomatic until challenged with a respiratory infection, when they may present with acute respiratory failure. With increasing use of genetic testing in pediatric patients, our knowledge of potential contributors to hypoventilation syndromes is growing. Although mutations in the paired-like homeobox 2B gene are known to be causative of congenital central hypoventilation syndrome, other genes may also contribute to hypoventilation phenotypes. We report one of the youngest reported patients with obesity hypoventilation syndrome in pediatrics, with a proposed congenital predisposition for central hypoventilation derived from a deletion in the brain-derived neurotrophic factor gene., Citation: McCoy J, Karp N, Brar J, Amin R, St-Laurent A. A novel case of central hypoventilation syndrome or just heavy breathing? J Clin Sleep Med. 2022;18(9):2321-2325., (© 2022 American Academy of Sleep Medicine.)

Background and Objectives: The introduction of spinal muscular dystrophy (SMA)-modifying therapies, such as antisense oligonucleotide therapy, has changed the natural history of SMA. Most reports on treatment outcomes have focused on motor scores and respiratory function. The objective of this study is to document the development and progression of scoliosis in patients with SMA1 treated with nusinersen., Methods: A descriptive single-center study was conducted in patients with SMA1 who were treated with nusinersen before 6 months of age. Data were collected on patients who met criteria, including age at the first nusinersen dose, number of nusinersen doses, degree of scoliosis, respiratory parameters, feeding route, and motor scores at baseline and follow-up. The Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP-INTEND) was subanalyzed using axial (AxS) and appendicular motor (ApS) scores to evaluate a possible correlation between scoliosis and axial muscle strength., Results: From our cohort, 31 percent (11/35) of patients had a diagnosis of SMA1. Sixty-three percent (7/11) met the inclusion criteria. All patients (7/7) showed initial improvement in their CHOP-INTEND scores in correlation with improvement on the ApS. Despite this, most patients did not show improvement in the AxS. Subsequently, all patients developed scoliosis in the first year of life with Cobb angles that ranged between 18° and 60°. Furthermore, total CHOP-INTEND scores had dropped in 2 patients alongside the development of a Cobb angle of >40°., Discussion: Despite the significant improvement in functional motor assessment in patients with SMA1, there is a progression of significant scoliosis despite treatment. Subsequently, lack or minimal improvement on the axial CHOP-INTEND scores may predict worsening on the total motor scores., (Copyright © 2022 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.)

Purpose: The hypoxic challenge test (HCT) is used to evaluate safety for air travel in individuals with respiratory disease by breathing in 15% oxygen for 20 min. Our aim was to determine if a prolonged HCT, lasting 120 min, identified more individuals with neuromuscular disease at potential risk than the standard HCT lasting 20 min., Methods: This was a cross-sectional study. All of the clinical testing took place at SickKids, Toronto, Canada. Patients were included in the study if they had a diagnosis of NMD, greater than 6 years of age, resting oxygen saturation ≥ 94%, and partial pressure of carbon dioxide (pCO2) ≤ 45 mmHg. Notable exclusion criteria were left ventricular ejection fraction < 30%, presence of a tracheostomy, and use of non-invasive ventilation for more than 12 h daily. Participants underwent a standard HCT as well as the prolonged HCT on the same day., Results: Twenty-three patients consented to the study. One patient was withdrawn because he was unable to follow the study procedures. The 22 study participants had a mean age of 14.9 years (standard deviation (SD) of 5 years). Seventeen (77%) participants were male. Two participants were withdrawn on the day of testing due to hypercapnia. Twenty participants completed the standard and prolonged HCTs. None of the participants had a positive standard or prolonged HCT., Conclusion: Our results suggest that performing a standard or prolonged HCT may, in fact, not be of clinical utility in individuals with less severe NMD., (© 2021. The Author(s), under exclusive licence to Springer Nature Switzerland AG.)

Objective: To evaluate the immediate and sustained knowledge retention and sense of self-efficacy of homecare nurses following completion of a standardized competency-based tracheostomy education course. Safe discharge of children requiring tracheostomy with or without ventilation relies on the competence of homecare nurses., Study Design: Pragmatic, randomized controlled trial of 44 homecare nurses. Participants were randomized into the intervention group ( n = 21), which received the tracheostomy course, or the control group ( n = 23), which received an enterostomy and vascular access course. Multiple-choice question (MCQ) knowledge assessments and self-efficacy questionnaires were administered to both groups pre-course and post-course at 6 week, 3 month, 6 month, and 12 month follow-ups., Results: Twenty participants in the intervention group and 19 in the control group were included. Four withdrew from the study and two crossed over from the control into the intervention arm. The change in mean self-efficacy scores (total score = 100) was significantly higher in the intervention group than in the control group at 6 weeks (intervention (mean ± SD): 18.6 ± 14.5; control: 6.6 ± 20.4; p = 0.04) and 3 months (intervention: 19.6 ± 14.2; control: 5.2 ± 17.0; p = 0.007), and trended higher at 6 months (intervention: 18.0 ± 14.5; control: 6.9 ± 24.1; p = 0.1). The change in mean MCQ assessment scores (total score = 20) trended higher in the intervention group than in the control group at 6 weeks (intervention (mean ± SD): 1.8 ± 2.2; control: 1.6, ± 2.9; p = 0.8)., Conclusions: Homecare nurses who attended the tracheostomy course demonstrated a higher sense of self-efficacy at long-term follow-up., Clinical Trial Registration: www.ClinicalTrials.gov, identifier: NCT04559932., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Shi, Orkin, Walsh, Chu, Keilty, McKay, Mocanu, Qazi, Ambreen and Amin.)

Objectives/hypothesis: Assess the risks and benefits of adenotonsillectomy (AT) for obstructive sleep apnea (OSA) in children with cerebral palsy (CP)., Study Design: Systematic review., Methods: We conducted a systematic review of Medline, Embase, and Cochrane Central Registry from 1946 to 2021. Broad search concepts included cerebral palsy, pediatric, tonsillectomy/adenoidectomy, and sleep. Additional articles were identified by searching reference lists. Studies on the safety and efficacy of AT for OSA management in children with CP were included., Results: Fifteen articles met inclusion criteria. Articles were classified into one or more of four themes: intraoperative risk (n = 1), postoperative risk (n = 3), postoperative care requirements (n = 6), and surgical outcomes (n = 7). No intraoperative anesthetic complications were reported. Postoperatively, respiratory complications including pneumonia were common and necessitated additional airway management. Following AT, children with CP required close postoperative observation, experienced increased lengths of stay, and had increased odds of unplanned intensive care unit (ICU) admission. Benefits following AT were improvement in OSA as measured by a reduction in obstructive apnea-hypopnea index (OAHI) as well as improved quality of life in some; however, many patients went on to require tracheostomy due to persistent OSA., Conclusions: Children with CP who undergo AT have a significant risk of developing a postoperative respiratory complication. Realistic counseling of families around increased perioperative risks in this population is imperative and close postoperative monitoring is critical. Many children will obtain a reduction in OAHI, but additional surgical management is often required, including tracheostomy. Further research is needed to determine the best management strategy for OSA in children with CP. Laryngoscope, 132:687-694, 2022., (© 2021 The American Laryngological, Rhinological and Otological Society, Inc.)

Determining the timing for decannulation in children with a tracheostomy is a complex process, as the appropriate timing varies based on the initial indication for the tracheostomy tube as well as individual patient characteristics. The original condition for which a tracheostomy was created may improve over time with decannulation being a very important long-term goal for many families and multidisciplinary teams. However, decannulation is an inherently risky procedure associated with morbidity and mortality. Therefore, careful planning is required to ensure the safety of the procedure. Although routine airway endoscopy is an important component of decannulation protocols, guidelines are less prescriptive regarding the definition of a complete endoscopic airway evaluation and the routine use of polysomnography. This review will summarize the important PRO and CON arguments of integrating polysomnography into pediatric decannulation protocols., (© 2021 Wiley Periodicals LLC.)

Background Overview and Rationale: We co-developed a multi-component virtual care solution (TtLIVE) for the home mechanical ventilation (HMV) population using the aTouchAway™ platform (Aetonix). The TtLIVE intervention includes (1) virtual home visits; (2) customizable care plans; (3) clinical workflows that incorporate reminders, completion of symptom profiles, and tele-monitoring; and (4) digitally secure communication via messaging, audio, and video calls; (5) Resource library including print and audiovisual material., Objectives and Brief Methods: Our primary objective is to evaluate the TtLIVE intervention compared to a usual care control group using an eight-center, pragmatic, parallel-group single-blind (outcome assessors) randomized controlled trial. Eligible patients are children and adults newly transitioning to HMV in Ontario, Canada. Our target sample size is 440 participants (220 each arm). Our co-primary outcomes are a number of emergency department (ED) visits in the 12 months after randomization and change in family caregiver (FC) reported Pearlin Mastery Scale score from baseline to 12 months. Secondary outcomes also measured in the 12 months post randomization include healthcare utilization measured using a hybrid Ambulatory Home Care Record (AHCR-hybrid), FC burden using the Zarit Burden Interview, and health-related quality of life using the EQ-5D. In addition, we will conduct a cost-utility analysis over a 1-year time horizon and measure process outcomes including healthcare provider time using the Care Coordination Measurement Tool. We will use qualitative interviews in a subset of study participants to understand acceptability, barriers, and facilitators to the TtLIVE intervention. We will administer the Family Experiences with Care Coordination (FECC) to interview participants. We will use Poisson regression for a number of ED visits at 12 months. We will use linear regression for the Pearlin Mastery scale score at 12 months. We will adjust for the baseline score to estimate the effect of the intervention on the primary outcomes. Analysis of secondary outcomes will employ regression, causal, and linear mixed modeling. Primary analysis will follow intention-to-treat principles. We have Research Ethics Board approval from SickKids, Children's Hospital Eastern Ontario, McMaster Children's Hospital, Children's Hospital-London Health Sciences, Sunnybrook Hospital, London Health Sciences, West Park Healthcare Centre, and Ottawa Hospital., Discussion: This pragmatic randomized controlled single-blind trial will determine the effectiveness and cost-effectiveness of the TtLIVE virtual care solution compared to usual care while providing important data on patient and family experience, as well as process measures such as healthcare provider time to deliver the intervention., Trial Registration: ClinicalTrials.gov NCT04180722 . Registered on November 27, 2019., (© 2022. The Author(s).)

Background: Despite expert recommendations for use, limited evidence identifies effectiveness of mechanical insufflation-exsufflation (MI-E) in addressing respiratory morbidity and resultant health care utilization and costs for individuals with neuromuscular disorders. We examined the impact of provision of publicly funded MI-E devices on health care utilization, health care costs, and survival trajectory., Methods: This is a retrospective pre/post cohort study linking data on prospectively recruited participants using MI-E to health administrative databases to quantify outcomes., Results: We linked data from 106 participants (8 age < 15 y) and determined annualized health care use pre/post device. We found no difference in emergency department (ED) visit or hospital admission rates. Following MI-E approval, participants required fewer hospital days (median [interquartile range] [IQR]) 0 [0-9] vs 0 [0-4], P = .03). Rates of physician specialist visits also decreased (median IQR 7 [4-11] vs 4 [2-7], P < .001). Conversely, rates of home care nursing and homemaking/personal support visits increased. Following MI-E, total costs were lower for 59.4%, not different for 13.2%, and higher for 27.4%. Physician billing costs decreased whereas home care costs increased. Regression modeling identified pre-MI-E costs were the most important predictor of costs after approval. At 12 months, 23 (21.7%) participants had died. Risk of death was higher for those using more medical devices (hazard ratio 1.12, [95% CI 1.02-1.22]) in the home., Conclusions: Provision of publicly funded MI-E devices did not influence rates of ED visits or hospital admission but did shift health care utilization and costs from the acute care to community sector. Although increased community costs negated cost savings from physician billings, evidence suggests costs savings from reduced hospital days and fewer specialist visits. Risk of death was highest in individuals requiring multiple medical technologies., Competing Interests: The authors disclose a relationship with IQVIA Canada., (Copyright © 2022 by Daedalus Enterprises.)

Study Objectives: 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., Methods: 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., Results: 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., Conclusions: 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., Citation: 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., (© 2022 American Academy of Sleep Medicine.)

Background: Selection of appropriate patients for transcatheter aortic valve implantation (TAVI) can be challenging. Many factors can influence post-procedure outcomes. Traditional surgical scoring systems do not discriminate effectively. Medical parameters and functional indices can characterize mortality risk. Mobility is an important predictive functional index but is largely defined using subjective criteria., Aim: To describe the relationship between mobility, objectively defined by the requirement for gait aids to ambulate, and all-cause 30-day and long-term mortality in patients undergoing TAVI., Methods: Mobility aid use was assessed in 1444 consecutive patients undergoing TAVI in a single center. Patients were categorized into "unaided," "1-stick," and "higher aid" groups based on the Brighton Mobility Index. Mortality tracking was obtained via the NHS Spine Portal in February 2021., Results: Patients were aged 82 (IQR 78-86). 66% of patients walked unaided, 26% walked with 1 stick, and 8% required more assistance (e.g., 2 sticks, a Rollator, a Zimmer frame, or a wheelchair). Overall 30-day mortality for the whole cohort was 3.5%; 1-year mortality was 12.2%. Mobility was a significant predictor of 30-day mortality (p = 0.025). Use of a higher aid was associated with a mortality odds ratio of 2.83 (95% CI: 1.39-5.74). Mobility was also a significant predictor of long-term mortality (p < 0.001). Odds ratios for 1-stick and higher aid groups were 1.45 (95% CI: 1.21-1.72) and 2.01 (95% CI: 1.55-2.60), respectively., Conclusion: Objective assessment of mobility by gait aid use predicts both short and long-term survival in patients undergoing TAVI. Increased dependence on mobility aids is associated with a worse prognosis., (© 2021 Wiley Periodicals LLC.)

Background: Premature birth affects millions of neonates each year, placing them at risk for respiratory disease due to prematurity. Bronchopulmonary dysplasia is the most common chronic lung disease of infancy, but recent data suggest that even premature infants who do not meet the strict definition of bronchopulmonary dysplasia can develop adverse pulmonary outcomes later in life. This post-prematurity respiratory disease (PPRD) manifests as chronic respiratory symptoms, including cough, recurrent wheezing, exercise limitation, and reduced pulmonary function. This document provides an evidence-based clinical practice guideline on the outpatient management of infants, children, and adolescents with PPRD. Methods: A multidisciplinary panel of experts posed questions regarding the outpatient management of PPRD. We conducted a systematic review of the relevant literature. The Grading of Recommendations, Assessment, Development, and Evaluation approach was used to rate the quality of evidence and the strength of the clinical recommendations. Results: The panel members considered the strength of each recommendation and evaluated the benefits and risks of applying the intervention. In formulating the recommendations, the panel considered patient and caregiver values, the cost of care, and feasibility. Recommendations were developed for or against three common medical therapies and four diagnostic evaluations in the context of the outpatient management of PPRD. Conclusions: The panel developed recommendations for the outpatient management of patients with PPRD on the basis of limited evidence and expert opinion. Important areas for future research were identified.

Purpose: Volume-assured pressure support in noninvasive ventilation (VAPS-NIV) is a newer mode providing automatic pressure support adjustment to ensure a constant alveolar ventilation. Previous studies have shown that NIV effectiveness depends on patient adherence and tolerance. The aim of this study was to determine the adherence and efficacy of VAPS-NIV compared to spontaneous-time (S/T) mode in pediatric patients with neuromuscular disease (NMD)., Methods: This was a prospective observational study. Children with NMD who utilized NIV at home for ≥ 3 months were recruited from the Long-term ventilation clinic at The Hospital for Sick Children, Toronto, Canada, from July 1, 2015, to July 1, 2019. Baseline characteristics, date of initiation of NIV, and pulmonary function tests were recorded. Polysomnogram (PSG) data and adherence were recorded and analyzed comparing VAPS and S/T modes., Results: Twenty children with NMD (17 male, 85%) were enrolled. The mean (SD) age at initiation of NIV was 11.6 ± 4.6 years. The median (IQR) duration of ventilation was 1.36 (0.80-2.98) years. The mean average daily usage and the median daily usage for VAPS mode and S/T mode were 8.4 ± 1.6 versus 7.2 ± 2.5 h (p = 0.012) and 8.6 ± 1.4 versus 7.8 ± 2.1 h (p = 0.022), respectively. There was no difference in sleep architecture, gas exchange, or parent proxy report of NIV tolerance between S/T and VAPS modes., Conclusion: VAPS was associated with an improvement in adherence to therapy in children with NMD compared to S/T mode. Longitudinal studies are required to evaluate long-term clinical outcomes using VAPS mode in children with NMD., (© 2021. Crown.)

Purpose: To identify current practices related to the assessment, monitoring and discussion of bodyweight, growth and obesity in neuromuscular clinics for children with Duchenne muscular dystrophy (DMD)., Methods: A cross-sectional, online survey was distributed using snowball sampling to healthcare providers working with children with DMD across Canadian neuromuscular clinics. Summary and descriptive statistics were calculated. Content analysis was performed on open text responses., Results: Thirty-seven responses were received, representing a range of healthcare disciplines. Height and weight were routinely assessed by 32/37 (87%) respondents, although only 21/37 (57%) responses reported having a clinic standard for measuring height and 23/37 (62%) for weight. While 32/36 (89%) reported discussing weight during consultations, only 13/37 (35%) felt confident doing so. Dietitians were considered the most appropriate person to discuss and manage weight with children and families, although only 17/37 (46%) reported having a dietitian involved in their clinic., Conclusions: Neuromuscular clinics could benefit from implementing consistent and recommended growth assessment practices. The development of evidence-based tools, training and protocols tailored to Duchenne muscular dystrophy should be a priority.IMPLICATIONS FOR REHABILITATIONGrowth and weight monitoring approaches vary within and between neuromuscular clinics.Additional training on discussing and managing weight are warranted across disciplines.Advocacy is required to ensure access to dietetic expertise within neuromuscular clinics.