Reshma Amin

SickKids

Introduction Children with medical complexity (CMC) have chronic, intensive care needs managed by many healthcare practitioners. Medical advances have enabled CMC to survive well into adulthood. However, the availability of supports as CMC transition into the adult care system remain suboptimal, contributing to poor care coordination, and discontin...

Background Homecare nursing is considered essential to the care for children with medical complexity (CMC) given their intense daily care demands and dependence on medical technology. While patients, families, and healthcare providers (i.e., paediatricians, nurse practitioners) rely on homecare nurses (HCNs), there is limited research exploring fac...

Consideration of the best practices for transition from pediatric to adult neuromuscular health is essential given the growing number of individuals with neuromuscular disease surviving into adulthood due to advances in medical care (Menon et al., Neuromuscul Disord 32(3):206–212, 2022), rehabilitation, and pulmonary ventilation techniques. For exa...

X-linked myotubular myopathy (XLMTM) is a rare, life-threatening congenital myopathy. Most (80%) children with XLMTM have profound muscle weakness and hypotonia at birth resulting in severe respiratory insufficiency, the inability to sit up, stand or walk, and early mortality. At birth, 85-90% of children with XLMTM require mechanical ventilation,...

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 use and predictors of highest costs using robust health administrative data. Objectives: To describe patterns of healt...

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 care...

Rationale: Information is limited about the association between obstructive sleep apnea (OSA) and mental disorders in children. Objectives: In children, (1) to evaluate the association between OSA and new mental healthcare encounters; (2) to compare mental healthcare encounters two years post- to pre-OSA treatment initiation. Methods: We condu...

Study objectives: Our aim was to characterize the 14 and 6 like spike wave activity seen on electroencephalogram (EEG) in children with Prader-Willi syndrome (PWS) undergoing polysomnogram (PSG). Methods: We performed a retrospective review of children with PWS and healthy controls who underwent diagnostic PSGs between January 1, 2007 to Decembe...

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...

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 accept...

Unlabelled: 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...

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 t...

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 hypov...

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 caregi...

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 P...

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 presc...

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 vent...

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....

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 b...

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 Ontari...

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 h...

Objective Early screening and diagnosis of nocturnal hypoventilation can slow progression to diurnal hypercapnia and mortality in children with neuromuscular disease (NMD). However, gold standard, laboratory-based polysomnography (PSG) testing is a limited resource. Therefore, we evaluated the diagnostic accuracy of ambulatory transcutaneous carbon...

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 relat...

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 identif...

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 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...

Background 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. However, the impact of this novel therapy on the progression of scoliosis is not known. The ob...

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: Pragma...

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...

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 sy...

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 challengin...

PurposeVolume-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 VA...

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 imp...

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...

Primary Subject area Respirology Background Despite the large morbidity and potential mortality associated with tracheostomy tube decannulation failure, there are currently no consensus guidelines on pediatric tracheostomy decannulation. This has led to wide practice variation that is largely based on expert option. This is the largest review of p...

Study objectives: Individuals with Duchenne muscular dystrophy (DMD) frequently develop sleep-disordered breathing (SDB). Non-invasive ventilation (NIV) is often prescribed for SDB treatment based on the American Academy of Sleep Medicine (AASM) criteria. In 2018, DMD disease specific criteria for SDB were established. Our study aimed to examine t...

PurposeThe 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...

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 caregive...

Introduction: The COVID-19 pandemic has been an unprecedented threat to our health care system. Clinicians had to pivot and develop creative and timely "virtual" solutions to provide clinical care. Our aim was to develop a standardized approach to virtual "mask fitting" for children who are either being initiated or are already on existing long-te...

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 sympt...

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 yea...

Abstract 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 pal...

Objectives: Discharging a child home on long term ventilation (LTV) via tracheostomy is complex and involves multiple healthcare providers across healthcare sectors. To date, patient and family feedback of a newly developed LTV discharge pathway has been anecdotal. Our objective was to explore the perceptions of family caregivers (FCs) that have co...

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 descri...

Objective Nemaline myopathy (NM) is a rare neuromuscular condition with clinical and genetic heterogeneity. To establish disease natural history, we performed a cross-sectional study of NM, complemented by longitudinal assessment and exploration of pilot outcome measures. Methods Fifty-seven individuals with NM were recruited at two family worksho...

Objective Adenotonsillectomy (AT) is common in children with trisomy 21 but outcomes are variable. Therefore, practitioners must have accurate information regarding the risks of the procedure specific to trisomy 21 to help patients weigh the risks and benefits of surgery. The objective of this study was to better characterize morbidity and mortalit...

Background Children with medical complexity (CMC) are defined by their medical fragility, dependence on assistive technology and substantial care needs. Family caregivers of CMC have unique challenges, such as prolonged hospitalizations and poor care coordination, which result in extensive caregiver stress. There is a great need to quantify the lev...

Importance Advance care planning (ACP) is the process of discussing values and preferences for care to help inform medical decision-making. Children with medical complexity (CMC) often have a shortened life span with an unpredictable clinical course and timing of death; however, there is a paucity of literature that describes the experience of ACP...

Objective To quantify psychosocial risk in family caregivers of children with medical complexity using the Psychosocial Assessment Tool (PAT) and to investigate potential contributing sociodemographic factors. Design Cross-sectional study. Setting Family caregivers completed questionnaires during long-term ventilation and complex care clinic visi...

Background: In 2014, the Ministry of Health of Ontario, Canada, approved a program of public funding for specialist-prescribed mechanical insufflation-exsufflation (MI-E) devices for home use by individuals with neuromuscular respiratory insufficiency. Since 2014, 1,926 MI-E devices have been provided, exceeding device-use projections. Few studies...

View Supplementary Video 1 Laryngoscope, 2020

Background/Objectives Children using home mechanical ventilation (HMV) live at home for 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 healthca...

Objectives To determine whether a change in clinical management (e.g., new tracheostomy or adenotonsillectomy) occurred following a polysomnogram (PSG) in children with medical complexity (CMC) and to explore whether families’ goals of care (regarding results and treatment implications) were discussed prior to the completion of a PSG. Methods All...

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...

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 reg...

Rationale: Respiratory muscle strength in children can be assessed by maximal inspiratory pressures (MIP), maximal expiratory pressures (MEP), and sniff nasal inspiratory pressures (SNIP). However, previous studies involved small cohorts of healthy children and reported wide reference ranges. Objectives: To perform a systematic review to summari...

Background Population trends of disease prevalence and incidence over time measure burden of disease and inform healthcare planning. Neuromuscular disorders (NMD) affect muscle and nerve function with varying degrees of severity and disease progression. Objective Using health administrative databases we described trends in incidence, prevalence, a...

Cohort creation codes and additional study data. (DOCX)

Objectives: Surgical intervention for obstructive sleep apnea (OSA) in overweight and obese children may not be as effective as it is in normal-weight children. The purpose of this study was to systematically review the effects of various surgical interventions for OSA in obese children and to meta-analyze the current data. Data sources: PubMed,...

Study objectives: Polysomnography (PSG) surveillance recommendations are not being met for children with neuromuscular disease (NMD) because of limited diagnostic facilities. We evaluated the diagnostic accuracy of an ambulatory level III device as compared to a level I PSG. Methods: A cross-sectional study was conducted at a tertiary pediatric...

Spinal muscular atrophy (SMA) is the most common genetic cause of infant mortality. SMA is a spectral disorder and is categorised based on symptom onset and severity. The median life expectancy for infants with SMA presenting before 6 months of age is less than 2 years without respiratory support. To date, there is no cure for SMA. In June 2017, nu...

Objectives To investigate variability in pediatric tracheostomy tube care practice patterns and access to resources across Canada. Methods Canadian pediatric otolaryngologists-head & neck surgeons reported their own practice patterns for children with chronic tracheostomy tubes using a web-based, 29-item multiple choice and short answer questionna...

Our objective was to quantify health service utilisation including monitoring and treatment of respiratory complications for adults with neuromuscular disease (NMD), identifying practice variation and adherence to guideline recommendations at a population level. We conducted a population-based longitudinal cohort study (2003–2015) of adults with NM...

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 childre...