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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.)

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: Sharing data across institutions is critical to improving care for children who are using long-term mechanical ventilation (LTMV). Mechanical ventilation data are complex and poorly standardized. This lack of data standardization is a major barrier to data sharing., Objective: We aimed to describe current ventilator data in the electronic health record (EHR) and propose a framework for standardizing these data using a common data model (CDM) across multiple populations and sites., Methods: We focused on a cohort of patients with LTMV dependence who were weaned from mechanical ventilation (MV). We extracted and described relevant EHR ventilation data. We identified the minimum necessary components, termed "Clinical Ideas," to describe MV from time of initiation to liberation. We then utilized existing resources and partnered with informatics collaborators to develop a framework for incorporating Clinical Ideas into the PEDSnet CDM based on the Observational Medical Outcomes Partnership (OMOP)., Results: We identified 78 children with LTMV dependence who weaned from ventilator support. There were 25 unique device names and 28 unique ventilation mode names used in the cohort. We identified multiple Clinical Ideas necessary to describe ventilator support over time: device, interface, ventilation mode, settings, measurements, and duration of ventilation usage per day. We used Concepts from the SNOMED-CT vocabulary and integrated an existing ventilator mode taxonomy to create a framework for CDM and OMOP integration., Conclusion: The proposed framework standardizes mechanical ventilation terminology and may facilitate efficient data exchange in a multisite network. Rapid data sharing is necessary to improve research and clinical care for children with LTMV dependence., (© 2022 Wiley Periodicals LLC.)

Background: Youth with cystic fibrosis (CF) and pulmonary exacerbation (PEx) often experience weight loss, then rapid weight gain. Little is known about body composition and its relationship to functional outcomes during this critical period., Methods: Twenty CF youth experiencing PEx were assessed on the day following admission and 7-17 days later at discharge for body mass index (BMI), fat mass index (FMI), lean mass index (LMI), skeletal muscle mass index (SMMI), and functional measures: percent predicted forced expiratory volume in 1 second (FEV1) (ppFEV1), maximal inspiratory and expiratory pressures (MIPs and MEPs), and handgrip strength (HGS). Changes from admission to discharge and correlations among body composition indices and functional measures at both times are reported., Results: Upon admission, participant BMI percentile and ppFEV1 varied from 2 to 97 and 29 to 113, respectively. Thirteen had an LMI below the 25th percentile and nine had a percent body fat above the 75th percentile. BMI and FMI increased significantly (p = 0.03, 0.003) during hospitalization. LMI and SMMI did not change. FEV1 and MIPS increased (p = 0.0003, 0.007), independent of weight gain, during treatment. HGS did not improve., Conclusions: Many youth with CF, independent of BMI, frequently carried a small muscle mass and disproportionate fat at the time of PEx. During hospital treatment, weight gain largely represented fat deposition; muscle mass and strength did not improve. A need for trials of interventions designed to augment muscle mass and function, and limit fat mass accretion, at the time of PEx is suggested by these observations., (© 2022 Wiley Periodicals LLC.)

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: 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.)

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: 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.)

Objectives: Discharging a child home on long-term ventilation (LTV) via tracheostomy is complex and involves multiple healthcare providers across healthcare sectors. To date, there has been a paucity of data with respect to the experiences of families transitioning a child home on LTV. Our objective was to explore the perceptions of family caregivers (FCs) who have completed a newly developed LTV discharge pathway as they transitioned home., Methods: We conducted 11 semi-structured interviews with FCs. Interviews focused on FC's experience with the training process, perception of competency from a knowledge and skill perspective, and opportunities for improvement. Interviews were audiotaped, transcribed verbatim, coded, and analyzed using an inductive thematic analysis approach., Results: Eight mothers and three fathers of ten children participated. Six primary themes were identified: (1) making an informed decision, (2) transitioning to rehabilitation, (3) building capacity for self-care, (4) coordinating case management, (5) readying for discharge home, and (6) experiencing home care., Conclusion: Overall, FCs felt that the preparation and transition support obtained through the application of a standardized LTV discharge pathway allowed successful attainment of new knowledge and skills necessary to care for their child with LTV at home., (© 2021 Wiley Periodicals LLC.)

Objective: To provide a systematic review of the existing pediatric decannulation protocols, including the role of polysomnography, and their clinical outcomes., Methods: Five online databases were searched from database inception to May 29, 2020. Study inclusion was limited to publications that evaluated tracheostomy decannulation in children 18 years of age and younger. Data extracted included patient demographics and primary indication for tracheostomy. Methods used to assess readiness for decannulation were noted including the use of bronchoscopy, tracheostomy tube modifications, and gas exchange measurements. After decannulation, details regarding mode of ventilation, location, and length of observation period, and clinical outcomes were also collected. Descriptive statistical analyses were performed., Results: A total of 24 studies including 1395 children were reviewed. Tracheostomy indications included upper airway obstruction at a well-defined anatomic site (35%), upper airway obstruction not at a well-defined site (12%) and need for long-term ventilation and pulmonary care (53%). Bronchoscopy was routinely used in 23 of 24 (96%) protocols. Tracheostomy tube modifications in the protocols included capping (n = 20, 83%), downsizing (n = 14, 58%), and fenestrations (n = 2, 8%). Measurements of gas exchange included polysomnography (n = 13/18, 72%), oximetry (n = 10/18, 56%), blood gases (n = 3,17%), and capnography (n = 3, 17%). After decannulation, children in 92% of protocols were transitioned to room air. Observation period of 48 h or less was used in 76% of children., Conclusions: There exists large variability in pediatric decannulation protocols. Polysomnography plays an integral role in assessing most children for tracheostomy removal. Evidence-based guidelines to standardize pediatric tracheostomy care remain an urgent priority., (© 2021 Wiley Periodicals LLC.)

Background: Previous studies have shown evidence of hypoxemia and hypercapnia during cardiopulmonary exercise test (CPET) evaluation in children with congenital central hypoventilation syndrome (CCHS). However, there are no longitudinal studies which compared CPET findings to polysomnogram (PSG) or PHOX2B mutation, to date., Objectives: To describe the longitudinal CPET findings in a cohort of children with CCHS and correlate the findings to the PSG results., Methods: This retrospective study was conducted in children with CCHS followed in the Long-term Ventilation Program at SickKids, Toronto, Canada between September, 2013 and January, 2020. CCHS genetic mutation, age of diagnosis, ventilatory support, family history, disease associations of CCHS, CPETs, and PSG parameters were recorded and analyzed., Results: A total of nine patients with CCHS (46 CPETs and 46 PSGs) were enrolled. Four (44.4%) children had polyalanine repeat mutations. The mean (SD) age at the time of diagnosis and duration of ventilatory usage were 3.2 ± 3.4 years and 11.5 ± 2.8 years, respectively. All abnormal CPETs had hypercapnia in at least 1 phase of the exercise test. Hypercapnia (12/46; 26.1%) at peak of exercise was the most common abnormality. None of the children experienced an oxygen desaturation below 90%. End-tidal CO 2 (PetCO 2 ) at rest and at peak exercise in the CPETs were significantly correlated with PSG TcCO 2 while PetCO 2 at anaerobic threshold was correlated with CO 2 in pre-PSG capillary blood gas., Conclusion: Nocturnal hypoventilation may impact the CPET results in CCHS children. Serial CPETs should be considered standard clinical care for all CCHS children., (© 2021 Wiley Periodicals LLC.)

Background: The care of mechanically ventilated pediatric patients is complex and burdensome. It is essential to adequately support the family caregivers of these children to optimize outcomes; however, there is no literature describing interventions for caregivers in this population., Research Question: This study described a novel behavioral health program and examined its impact on family caregiver engagement and psychological distress on a pediatric inpatient chronic ventilator unit., Study Design and Methods: Electronic chart review was completed with retrospective and prospective participant enrollment for the purposes of longitudinal evaluation of caregiver engagement. For analytic purposes, participants were grouped into three categories: (1) preprogram, (2) postprogram, and (3) postprogram with completion of psychotherapy., Results: The behavioral health program was associated with increased caregiver participation in rounds, t = 7.76, p = < .001. Parents who completed a course of psychotherapy within the behavioral health program demonstrated reduced time to training completion (F = 5.89; p < .01), higher staff-rated caregiver engagement (F = 3.69; p < .05), and significantly reduced levels of caregiver distress (t = 2.09; p < .05)., (© 2021 Wiley Periodicals LLC.)

Background: Children using home mechanical ventilation (HMV) live at home with better quality of life, despite financial burden for their family. Previous studies of healthcare utilization and costs have not considered public and private expenditures, including family caregiver time. Our objective was to examine public and private healthcare utilization and costs for children using HMV, and variables associated with highest costs., Methods: Longitudinal, prospective, observational cost analysis study (2012-2014) collecting data on public and private (out-of-pocket, third-party insurance, and caregiving) costs every 2 weeks for 6 months using the Ambulatory Home Care Record. Functional Independence Measure (FIM), WeeFIM, and Caregiving Impact Scale (CIS) were measured at baseline and study completion. Regression modeling examined a priori selected variables associated with monthly costs using Andersen and Newman's framework for healthcare utilization, relevant literature, and clinical expertise. Data are reported in 2015 Canadian dollars ($1CAD = $0.78USD)., Results: Forty two children and their caregivers were enrolled. Overall median (interquartile range) monthly healthcare cost was $12 131 ($8159-$15 958) comprising $9929 (89%) family caregiving hours, $996 (9%) publicly funded, and $252 (2%) out-of-pocket (<1% third-party insurance) costs. With higher FIM score (lower dependency), median costs were reduced by 4.5% (95% confidence interval: 8.3%-0.5%), adjusted for age, sex, tracheostomy, and daily ventilation duration. Note: since the three cost categories did not sum to the total statistically derived median cost, the percentage of each category used the sum of median public + caregiver lost time + private out-of-pocket + third-party insurance as the denominator., Conclusions: For HMV children, most healthcare costs were due to family caregiving costs. More dependent children incur highest costs. The financial burden to family caregivers is substantial and needs to considered in future policy decisions related to pediatric HMV., (© 2020 Wiley Periodicals LLC.)

Objective: Respiratory compromise in congenital muscular dystrophy (CMD) occurs, in part, from chest wall contractures. Passive stretch with hyperinsufflation therapy could reduce related costo-vertebral joint contractures. We sought to examine the impact of hyperinsufflation use on lung function and quality of life in children with CMD., Study Design: We conducted a randomized controlled trial on hyperinsufflation therapy in children with CMD at two centers. An individualized hyperinsufflation regimen of 15 minutes twice daily using a cough assist device over a 12 months period was prescribed. We measured lung function, quality of life, and adherence. To demonstrate reproducibility, pulmonary function was measured twice on the same day. A mixed-effects regression model adjusting for confounders was used to assess the effects of hyperinsufflation., Results: We enrolled 34 participants in the study; 31 completed the trial (n = 17 treatment group and n = 14 controls). Participants in the treatment group demonstrated a relative gain in lung volume measured at 4 and 8 months, but not at 12 months. The control group required increases in the maximum insufflation pressures to achieve maximum lung volumes while the treatment group did not. Adherence was best early in the study, peaking at the first visit and decreasing at subsequent visits. Caregiver-reported quality of life was higher in the treatment group., Conclusion: Hyperinsufflation therapy is effective in increasing and sustaining lung volume over time. Adherence, however, was inconsistent and difficult to maintain. Further research should determine if improved adherence leads to sustained benefits of hyperinsufflation., (© 2020 Wiley Periodicals LLC.)

Objective: Adolescents receiving long-term home mechanical ventilation (HMV) who survive into adulthood must transition to adult health care services. Lack of transition readiness is reported to result in poor health outcomes. The objective of this study is to explore longitudinally the pediatric-to-adult health care transition experience involving a transition program for adolescents receiving HMV including transition readiness, barriers, facilitators, and modifiable features., Design: A prospective qualitative longitudinal interview study of adolescent and family caregiver dyads recruited through a pediatric-to-adult HMV transition program jointly established by two collaborating health centers: The Hospital for Sick Children and West Park Healthcare Centre in Toronto, Canada. Eligible dyads were interviewed at three time points: pretransition, transition, and 12-months posttransition. Interviews were transcribed verbatim and analyzed using directed content analysis methods., Results: Ventilator-assisted adolescents (VAAs) and caregiver participants perceived a lack of transition readiness in their ability to manage health communication and coordination across multiple adult providers. Transition facilitators included early transition discussion, opportunities for VAAs to speak directly with HMV providers during appointments, receipt of print informational materials regarding adult services, and a joint pediatric-adult team handover meeting. Modifiable transition barriers included lack of other specialist referrals, insufficient information about adult homecare service funding, and limited involvement of family doctors. Unresolved transition barriers resulted in perceptions of service fragmentation., Conclusions: Although the pediatric-to-adult HMV transition program conferred benefits service fragmentation was perceived. Transition barriers may be overcome through early planning and staged transition with all specialists, community providers, and the family and adolescent working in collaboration., (© 2020 Wiley Periodicals, Inc.)

There has been a significant increase in the past few decades in the number of children receiving noninvasive positive airway pressure (PAP) therapy at home. At present, PAP therapy can be successfully used in children of all ages, for a variety of indications. Data acquired from PAP devices is clinically useful, providing objective information regarding adherence, leak, and efficacy of PAP therapy. However, guidelines outlining a standardized approach to interpretation of PAP device data in pediatrics is currently lacking. Given the rapidly expanding use of PAP therapy in pediatric practice, we aim to provide an overview of the interpretation of data reports, otherwise called "data downloads," from PAP devices and illustrate how they can be used to guide clinical care., (© 2019 Wiley Periodicals, Inc.)

Objectives: To quantify health service utilization including assessment, monitoring, and treatment of respiratory complications of children with neuromuscular disease (NMD), identifying practice variation and adherence to guideline recommendations at a population level., Methods: North American population-based cohort study (2003-2015) of children with NMD using hospital diagnostic and physician billing codes within health administrative databases., Results: We identified 18 163 children with NMD. Mean (SD) age was 7.8 (5.6) years with 40% ≤5; 45% were female. Most common diagnoses were cerebral palsy (50%) and spina bifida (16%); 8% had muscular dystrophy. From fiscal years 2003-2014, 15 600 (86%) children went to an emergency department on average 3.5 times every 3 years; 6575 (36%) for respiratory reasons. 8788 (48%) were admitted to hospital with 2190 (12%) for respiratory reasons and 2451 (13%) required intensive care. Respiratory specialist outpatient visits occurred for 2226 (12%) children on average 6.5 visits every 3 years; 723 (4%) had in-hospital respiratory specialist consultation. Pulmonary function testing was conducted in 3194 (18%) children on average 2.4 times every 3 years; sleep studies in 1389 (8%)., Conclusion: In this population-based study of children with NMD, healthcare utilization for respiratory complications was considerable. Frequency of respiratory specialist consultation, monitoring of respiratory function and sleep disordered breathing was variable but on average reflected professional society recommendations. Children with NMD are frequent ED users suggesting a need to improve community and social supports. We did not detect reduced access to respiratory monitoring or specialist consultation in adolescents transitioning to adult services., (© 2018 Wiley Periodicals, Inc.)

Background: Improved exercise capacity (EC) and normal glucose tolerance (NGT) are independently associated with favorable outcomes in CF, however, little information on this relationship exists in patients with CF., Methods: Cardiopulmonary exercise tests, oral glucose tolerance tests (OGTT), and HbA1c values measured within a 12-month period were evaluated on 83 pediatric patients diagnosed with CF. Patients were categorized as having NGT, abnormal glucose tolerance (AGT), or CF-related diabetes (CFRD)., Results: EC decreased as severity of glucose intolerance increased across NGT, AGT, and CFRD groups (P = 0.02). Compared to patients with NGT, patients with CFRD had lower peak VO 2  mL/kg/min (33.0 ± 7.3 vs 41.3 ± 9.4, P = 0.01), lower VO 2 % (81 ± 20 vs 93 ± 17, P = 0.03), and higher HbA1c (6.9 ± 1.7 vs 5.4 ± 0.4, P < 0.01). There was a positive association with age and FEV 1 % with EC in the 17 patients with CFRD. In the 66 patients without diabetes, peak EC was positively associated with FEV 1 % and negatively associated with age, fasting insulin, and insulin 120 min. After accounting for age and FEV 1 %, multivariate analyses indicated that insulin and glucose values at 120 min predicted EC., Conclusions: These data provide evidence that poor glucose tolerance is associated with lower EC in pediatric patients with CF. There was a significant relationship between glucose and insulin values obtained by OGTT with EC in a sample of non-diabetic patients with preserved lung function. Future studies are warranted to confirm these findings and investigate the potential role of exercise in the management or prevention of CFRD., (© 2017 Wiley Periodicals, Inc.)

Objective: To evaluate the contents of parent-created emergency tracheostomy kits and identify deficiencies., Methods: This was an observational study. Data on emergency tracheostomy kits were abstracted for 30 consecutive children who had a tracheostomy tube in situ during an outpatient clinic visit with the Division of Respiratory Medicine and/or the Department of Otolaryngology-Head and Neck Surgery at the Hospital for Sick Children between February 1 and October 30, 2016. A checklist of 12 essential items based on expert consensus was used to evaluate each tracheostomy kit., Results: Emergency tracheostomy kits from all children were missing at least one item from the 12-item checklist. Nineteen (63%) kits had three or more critical items missing. All kits had the same size tracheotomy tube. Twenty-two (73%) kits did not have a half size smaller tracheostomy tube. Fifteen (50%) were missing a manual resuscitation bag and four (13.3%) were missing a suction machine. Children who had tracheostomy tube in situ for ≥4 years were more likely to have ≥3 missing items in their kit (43.4%) compared to those who had tracheostomy tube for <4 years (20%), (χ2 (1) = 9.85, P = 0.0017)., Conclusion: Maintenance of a fully stocked emergency tracheostomy kit can save a child's life. It is incumbent upon healthcare providers to ensure ongoing reassessment of knowledge and skills required to care for a child with a tracheostomy tube and to regularly review the components of a child's emergency tracheostomy kit., (© 2017 Wiley Periodicals, Inc.)

Objective: Caregivers of children using home mechanical ventilation (HMV) via tracheostomy require appropriate knowledge and skills. Existing training curricula are locally developed and content variable. We sought to develop a competency checklist to inform initial training and subsequent assessment of knowledge and skills of family caregivers., Methods: We used a 2-step process. Candidate items were generated by synthesis of a scoping review, existing checklists, with additional items suggested by an eight member inter-professional group representing pediatric HMV programs across Canada. Following removal of duplicate items, we conducted a three-round Delphi to gain consensus on items for the KidsVent Checklist., Results: The scoping review and checklists from five HMV programs identified 18 domains and 172 items; one additional domain and 83 additional items were identified by our expert group who also classified domains as mandatory or optional. We recruited 95 clinicians representing 12 Canadian paediatric HMV programs to participate in Delphi round 1 (response rate 72%; 84%, and 100% for subsequent rounds). Importance rating of the 255 items reduced them to 246 items. In the final checklist, the 19 domains comprised 14 mandatory (189 mandatory items) and 5 optional domains (57 optional items)., Conclusions: We have developed the KidsVent checklist using rigorous consensus building methods, informed by participants with diverse geographic and inter-professional representation. This checklist represents knowledge and skills required to safely care for children using tracheostomy ventilation at home. Further study is required to explore the impact of this checklist on outcomes of this growing group of technology-dependent children., (© 2017 Wiley Periodicals, Inc.)

Background: Nontuberculous mycobacteria (NTM) have a particular affinity for patients with cystic fibrosis (CF). Recent studies suggest a possible relationship between acquiring NTM and the level of environmental water in a given area. We sought to determine if there is an association between household proximity to water and NTM in children with CF., Materials and Methods: An IRB-approved retrospective chart review was completed on 150 children with CF in Florida. Inclusion criteria required regular follow-up, at least two acid-fast bacilli cultures, and a consistent home address over a 3-year period. The distance from each patient's home to the nearest body of water was measured using ArcMap®, a Geographic Information System, and the mean distance to water for NTM-positive and NTM-negative groups were compared. A stepwise backwards logistic regression was used to evaluate for predictors of NTM-positivity., Results: Of the 150 CF patients, 65 met inclusion criteria and 21 (32.3%) tested positive for NTM. Comparison of the mean distance to water for NTM-positive versus NTM-negative groups revealed a cutoff of 500 meters. On the logistic regression, CF patients who lived within 500 meters of water were 9.4 times more likely to acquire NTM (P = 0.013). Other significant predictors included a history of Aspergillus fumigatus (OR 7.9, P = 0.011) and recent history of Pseudomonas aeruginosa (OR 2.5, P = 0.007)., Conclusions: In the regions studied, children with CF who live closer to water are more likely to acquire nontuberculous mycobacteria. Future studies in other geographic areas are needed to determine if these results are generalizable. Pediatr Pulmonol. 2017;52:324-330. © 2016 Wiley Periodicals, Inc., (© 2016 Wiley Periodicals, Inc.)

There has been a dramatic increase in the past few decades in the number of children receiving noninvasive positive airway pressure (PAP) therapy at home. Although PAP therapy was first prescribed for children with obstructive sleep apnea, the indications have rapidly widened to include treatment for central hypoventilation syndromes, neuromuscular and chest wall disorders as well as primary respiratory diseases. Given the rapidly expanding use of PAP therapy in children, pediatric pulmonologists need to be familiar with the indications, technical and safety considerations as well as potential complications and challenges that may arise when caring for children using PAP therapy. This review article covers the definition of PAP therapy, modes, interfaces, devices, indications, contraindications, suggested settings, complications as well as the factors influencing the adherence., (© 2015 Wiley Periodicals, Inc.)

Background: We report on the long-term survival of children initiated on invasive and noninvasive positive pressure ventilation (NiPPV) before the age of 1 to assess the safety and efficacy of long-term ventilation at home., Methods: A chart review was performed of children initiated on long-term home mechanical ventilation (LTHV) before the age of 1 year, at The Hospital for Sick Children (SickKids), Canada, between January 1991 and April 2014., Results: We report on 51 children. Twenty-five children (49%) received NiPPV and 26 (51%) received invasive mechanical ventilation via tracheostomy (IMV). There was one NiPPV initiation between 1991 and 2001, the rest were in subsequent years. Most children had a "musculoskeletal disorder" in the NiPPV cohort, n = 14 (56%) and a "central nervous system" disorder in the IMV cohort, n = 13 (50%). The pCO2 improved with the initiation of NiPPV, P = < 0.0001. Of the 25 subjects initiated on NiPPV, eight (32%) are currently being followed as compared to 22 (84%) in the IMV cohort. Seven (28%) of the NiPPV group were weaned off ventilation as compared to three (11.5%) in the IMV cohort. There were two NiPPV treatment failures. There were more deaths in the NiPPV cohort: eight (32%) versus two (7.6%) in the IMV cohort. Four of the deaths in the NiPPV cohort were in children in whom a palliative approach was taken. None were due to NiPPV technical failure., Conclusions: Based on this long-term follow-up study, NiPPV use in infants appears to be a viable long-term ventilation strategy., (© 2015 Wiley Periodicals, Inc.)

Background: Canadian longitudinal data from a pediatric domiciliary long-term mechanical ventilation (LTMV) program is lacking., Objective: Our aim was to report on the clinical characteristics and trends of children followed in one of Canada's pediatric home ventilation programs over the past 20 years., Methods: A retrospective chart review was conducted on patients receiving long-term domociliary mechanical ventilation between January 1, 1991 and December 31, 2011 in a single center. Domiciliary long-term mechanical ventilation was defined as the daily use of invasive mechanical ventilation (IMV) or noninvasive positive pressure ventilation (NiPPV) for at least 3 months, in the users' home or in a long-term residential facility., Results: Between 1991 and 2011, a total of 379 children were identified (313 [83%] with noninvasive ventilation). The median age at initiation was 9.6 years (interquartile range [IQR] 2.9-13.9), the median duration of ventilation was 2.2 years (IQR 0.8-4.9) and 53% were male. Ninety-nine percent of children were cared for at home. The reason for ventilation was "musculoskeletal" in origin for the majority of children. The number of children receiving long-term mechanical ventilation at home increased from 2 in 1991 to 156 children as of December 2011. There was a twofold increase in the number of invasive ventilation initiations in the second 10 years, n = 45 (2001-2011) as compared to the first 10 years, n = 21 (1991-2000). However, there was more than a fivefold increase in the number of noninvasive initiations in the first 10 years, n = 50 (1991-2000) as compared to the second 10 years, n = 263 (2001-2011). The largest growth was in the 13-18 years age group. There were 55 (15%) mortalities over the study period., Conclusions: In summary, our 20-year retrospective study has shown that there has been an exponential growth in the number of children receiving domiciliary LTMV with the majority of children having favorable outcomes. Our study represents a step towards developing a Canadian registry to design and implement programmatic change for this medically complex population to ensure best practice for these children as well as their families., (© 2013 Wiley Periodicals, Inc.)

Objective: Our aim was to assess the effect of treatment of early infection of P. aeruginosa on pulmonary function in pediatric CF patients., Methods: This was a retrospective cohort study of P. aeruginosa negative CF patients followed at Sick Kids from 1990 to 2007. Early P. aeruginosa infection was defined as the first respiratory culture for P. aeruginosa; patients were included if 5 years of follow-up pulmonary function data were available. Patients were divided into three groups (group 1: never infected, group 2: infected with subsequent clearance, and group 3: chronic infection or still receiving antipseudomonal antibiotics). Hierarchical linear models were used to estimate the effect of P. aeruginosa infection on spirometry. FEV(1) % predicted was the primary outcome., Results: 116 patients were included. Forty-six (40%) patients remained P. aeruginosa negative throughout the observation period, 29 (25%) patients transiently infected with P. aeruginosa, and 41 (35%) patients were either currently infected or still receiving treatment. Baseline lung function was the same for all groups. Annual decline in FEV(1) % predicted during the study period was not different (-0.6%/year for patients that were never infected and -1.3%/year among patients previously infected)., Conclusions: Lung function was not different between patients with early P. aeruginosa infection and those that never had P. aeruginosa infection. However given the slow rate of FEV(1) decline in the study population, a longer observation period and/or more sensitive outcomes measures may be required to exclude long-term detrimental effects of transient P. aeruginosa infection on lung function in CF patients., (Copyright © 2011 Wiley-Liss, Inc.)