Spinal Muscular Atrophy Update in Best Practices: Recommendations for Treatment Considerations.

Background and Objectives: Spinal muscular atrophy (SMA) is an autosomal recessive disorder caused by biallelic variants of the Survival Motor Neuron 1 gene (SMN1) that affects approximately 1 in 15,000 live births. Availability of 3 SMN-enhancing treatments for SMA has led to urgency to review how clinicians and patients use these treatments for SMA, while additional research and real-world data and experience are being collected. This work describes important factors to assist with decision-making for SMN-enhancing treatments. Methods: A systematic literature review was conducted on SMN-enhancing treatments for SMA and related studies. A working group of American and European health care providers with expertise in SMA care identified barriers and developed recommendations through a modified Delphi technique with serial surveys and feedback through virtual meetings to fill gaps for information where evidence is limited. A community working group of an individual living with SMA and caregivers provided insight and perspective on SMA treatments and support through a virtual meeting to guide recommendations. Results: The health care provider working group and the community working group agreed that when determining whether to start, change, add, or discontinue a treatment, essential considerations include patient and family/caregiver perspective, and treatment safety and side effects. When initiating treatment for patients newly diagnosed with SMA, important patient characteristics are age and Survival Motor Neuron 2 gene (SMN2) copy number. Furthermore, when initiating, changing, or adding treatment, current clinical status and comorbidities drive decision-making. When considering a medication or treatment plan change, unless there is an urgent indication, a treatment and associated patient outcomes should be monitored for a minimum of 6-12 months. When determining a treatment plan with an adolescent or adult with SMA, consider factors such as quality of life, burden vs benefit of treatment, and reproductive issues. Access to care coordination and interdisciplinary/multidisciplinary care are essential to treatment success. Discussion: Sharing information about current knowledge of treatments and shared decision-making between health care providers and patients living with SMA and caregivers are essential to overcoming barriers to providing SMN-enhancing treatments.

Nutrition support in children with medical complexity and chronic critical illness: A narrative review.

Children with medical complexity (CMC) and children with chronic critical illness (CCI) represent growing populations with high healthcare use and dependence on specialized care, both in the hospital and community setting. Nutrition assessment and delivery represent critical components of addressing the short-term and long-term health needs for these populations across the care continuum. This article provides a framework and reviews existing literature for the assessment of nutrition status and subsequent delivery of nutrition prescriptions in CMC and children with CCI. The specific aims are to (1) describe the epidemiology of health services experience for CMC and children with CCI, with a focus on their nutrition outcomes; (2) detail how to assess their nutrition status and energy requirements; (3) review methods of delivery of the nutrient prescription; (4) introduce perioperative considerations; (5) highlight examples of special populations of CMC and children with CCI; and (6) propose future research initiatives to improve nutrition and overall outcomes for these populations.

An algorithm for discontinuing mechanical ventilation in boys with x-linked myotubular myopathy after positive response to gene therapy: the ASPIRO experience.

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, with more than half requiring invasive ventilator support. Historically, ventilator-dependent children with neuromuscular-derived respiratory failure of this degree and nature, static or progressive, are not expected to achieve complete independence from mechanical ventilator support. In the ASPIRO clinical trial (NCT03199469), participants receiving a single intravenous dose of an investigational gene therapy (resamirigene bilparvovec) started showing significant improvements in daily hours of ventilation support compared with controls by 24 weeks post-dosing, and 16 of 24 dosed participants achieved ventilator independence between 14 and 97 weeks after dosing. At the time, there was no precedent or published guidance for weaning chronically ventilated children with congenital neuromuscular diseases off mechanical ventilation. When the first ASPIRO participants started showing dramatically improved respiratory function, the investigators initiated efforts to safely wean them off ventilator support, in parallel with primary protocol respiratory outcome measures. A group of experts in respiratory care and physiology and management of children with XLMTM developed an algorithm to safely wean children in the ASPIRO trial off mechanical ventilation as their respiratory muscle strength increased. The algorithm developed for this trial provides recommendations for assessing weaning readiness, a stepwise approach to weaning, and monitoring of children during and after the weaning process.

Achieving Enteral Autonomy in Children with Intestinal Failure Following Inpatient Admission: A Case Series.

We describe cases of intestinal failure wherein inpatient admission was critical toward enteral autonomy. We performed a retrospective chart review of 6 children with long-term parenteral nutrition dependence who were weaned from parenteral nutrition after admission. Admissions included feeding and medication titration, interdisciplinary care, and a home parenteral nutrition team consultation.

Characteristics and Outcomes of Home-Ventilated Children Undergoing Noncardiac Surgery.

OBJECTIVES: To determine the frequency of children with chronic respiratory failure (CRF) and home ventilator dependence undergoing surgery at a tertiary children's hospital, and to describe periprocedural characteristics and outcomes. METHODS: We conducted a retrospective cohort study of patients with CRF and home ventilator dependence who underwent noncardiac surgery from January 1, 2013, to December 31, 2019. Descriptive statistics were used to report patient and procedural characteristics. Univariable and multivariable analyses were used to assess for factors associated with 30-day readmission. RESULTS: We identified 416 patients who underwent 1623 procedures. Fifty-one percent of patients used transtracheal mechanical ventilation (trach/vent) support at the time of surgery; this cohort was younger (median age 5.5 vs 10.8 years) and more complex according to American Society of Anesthesiologists status compared with bilevel positive airway pressure-dependent patients. Postoperatively, compared with bilevel positive airway pressure-dependent patients, trach/vent patients were more likely to be admitted to the ICU with longer ICU length of stay (median 5 vs 2 days). Overall 30-day readmission rate was 12% (n = 193). Presence of chronic lung disease (adjusted odds ratio 1.65, 95% confidence interval 1.01-1.69) and trach/vent dependence (adjusted odds ratio 1.65, 95% confidence interval 1.02-2.67) were independently associated with increased odds for readmission. CONCLUSIONS: Children with CRF use anesthetic and surgical services frequently and repeatedly. Those with trach/vent dependence have higher hospital and ICU resource utilization. Although overall mortality for these patients is quite low, underlying diagnoses, nuances of technology dependence, and other factors for frequent readmission require further study to optimize resource utilization and outcomes.

Influenza and COVID-19 Vaccination Rates Among Children Receiving Long-Term Ventilation.

This cohort study examines rates of vaccination for COVID-19 and for influenza among children receiving long-term home ventilation.

Pediatric pulmonology year in review-Pediatric pulmonary critical care.

Pediatric pulmonary critical care literature has continued to grow in recent years. Our aim in this review is to narrowly focus on publications providing clinically-relevant advances in pediatric pulmonary critical care in 2023.

Perioperative Health Interventions in Children With Chronic Neuromuscular Conditions Undergoing Major Musculoskeletal Surgery: A Scoping Review.

BACKGROUND AND OBJECTIVES: Children with chronic neuromuscular conditions (CCNMC) have many coexisting conditions and often require musculoskeletal surgery for progressive neuromuscular scoliosis or hip dysplasia. Adequate perioperative optimization may decrease adverse perioperative outcomes. The purpose of this scoping review was to allow us to assess associations of perioperative health interventions (POHI) with perioperative outcomes in CCNMC. METHODS: Eligible articles included those published from January 1, 2000 through March 1, 2022 in which the authors evaluated the impact of POHI on perioperative outcomes in CCNMC undergoing major musculoskeletal surgery. Multiple databases, including PubMed, Embase, Cumulative Index of Nursing and Allied Health Literature, Web of Science, the Cochrane Library, Google Scholar, and ClinicalTrials.gov, were searched by using controlled vocabulary terms and relevant natural language keywords. Preferred Reporting Items for Systematic Reviews and Meta-Analyses Extension for Scoping Reviews guidelines were used to perform the review. A risk of bias assessment for included studies was performed by using the Risk of Bias in Non-randomized Studies of Interventions tool. RESULTS: A total of 7013 unique articles were initially identified, of which 6286 (89.6%) were excluded after abstract review. The remaining 727 articles' full texts were then reviewed for eligibility, resulting in the exclusion of 709 (97.5%) articles. Ultimately, 18 articles were retained for final analysis. The authors of these studies reported various impacts of POHI on perioperative outcomes, including postoperative complications, hospital length of stay, and hospitalization costs. Because of the heterogeneity of interventions and outcome measures, meta-analyses with pooled data were not feasible. CONCLUSIONS: The findings reveal various impacts of POHI in CCNMC undergoing major musculoskeletal surgery. Multicenter prospective studies are needed to better address the overall impact of specific interventions on perioperative outcomes in CCNMC.

Autism and neurodevelopmental disability risks in children with tracheostomies and ventilators.

BACKGROUND/OBJECTIVE: Infants who survive prematurity and other critical illnesses and require continued invasive mechanical ventilation (IMV) postdischarge (at home) are at high risk of developmental delays and disabilities. Studies of extremely preterm cohorts (<28-week gestation) demonstrate rates of 25% for intellectual disability (ID) and 7% for autism spectrum disorder (ASD). Rates of ASD and ID in children with IMV are unknown. This study aimed to determine neurodevelopmental disability risk in a cohort of children with postdischarge IMV. DESIGN/METHODS: A consecutive series of children with IMV were assessed 1 month, 6 months, and 1 year after discharge. Cognitive, social, and communicative domains were assessed by a Developmental and Behavioral Pediatrician using (1) clinical adaptive test/clinical linguistic and auditory milestone scale (CAT/CLAMS) of the capute scales; (2) pediatric evaluation of disability inventory computer adaptive test (PEDI-CAT); and (3) modified checklist for autism in toddlers, revised (MCHAT-R). Red flag signs and symptoms of ASD using DSM-V criteria were noted. Longitudinal testing was reviewed. Expert consensus impressions of evolving ASD and/or ID were determined. RESULTS: Eighteen children were followed for 1 year; at 1 year, the median age (range) was 23 (17-42) months. Children were 44% male, 33% non-Hispanic White, 39% non-Hispanic Black, and 28% Hispanic. Fifteen (83%) children were prematurity survivors. Median (range) developmental quotients (DQs): full-scale DQ 59 (11-86), CAT DQ 66.5 (8-96), and CLAMS DQ 49.5 (13-100). Twelve (67%) children were highly suspicious for ASD and/or evolving ID. CONCLUSIONS/SIGNIFICANCE: This cohort of children with at-home IMV demonstrates a higher risk of ASD and ID than prior premature cohorts. Larger investigations with longer follow-up are needed.

Outcomes for Pediatric Oncology and Hematopoietic Stem Cell Transplantation Patients Who Undergo Tracheostomy Placement: A Pediatric Health Information System Database Cohort Study, 2009-2020.

OBJECTIVES: To describe the epidemiology, surgical complications, and long-term outcomes after tracheostomy in pediatric oncology and/or hematopoietic stem cell transplantation (HSCT) patients in U.S. Children's Hospitals. DESIGN: Retrospective cohort from the Pediatric Health information System (PHIS) database, 2009-2020. SETTING: The PHIS dataset incorporates data from 48 pediatric hospitals in the Children's Hospital Association. PATIENTS: Patients 0-21 years old with an oncologic diagnosis and/or underwent HSCT, received a tracheostomy, and were discharged from hospital between January 1, 2009, and December 31, 2020. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: There were 1061 patients included in the dataset, and 217 (20.5%) had undergone HSCT. The annual prevalence in tracheostomy usage did not change over the study period. The majority of patients (62.2%) underwent tracheostomy early (< 30 d) in the admission and those who underwent the procedure later (> 90 d) had a significant increase in mortality (52.6% vs. 17.6%; p < 0.001) and mechanical ventilation (MV) at discharge (51.9% vs. 24.5%; p < 0.001) compared with the early tracheostomy patients. Complications reported included tracheostomy site bleeding (< 1%) and infection (24%). The overall rate of MV at discharge was 32.6% and significantly associated with chronic lung (adjusted odds ratio [OR], 1.54; 95% CI, 1.03-2.32) and acute lung disease (OR, 2.18; 95% CI, 1.19-3.98). The overall rate of mortality was 19.6% within the cohort and significantly associated with HSCT (OR, 5.45; 95% CI, 3.88-7.70), diagnosis of sepsis (OR, 2.09; 95% CI, 1.28-3.41), and requirement for renal replacement therapy (OR, 2.76; 95% CI, 1.58-4,83). CONCLUSIONS: This study demonstrated a static prevalence of tracheostomy placement in the cohort population relative to the increasing trends in other reported groups. Regardless of underlying diagnosis, the study patients incurred substantial morbidity and mortality. However, tracheostomy specific complication rates were comparable with that of the general pediatric population and were not associated with increased odds of mortality within this population.

The myotubular and centronuclear myopathy patient registry: a multifunctional tool for translational research.

The Myotubular and Centronuclear Myopathy Registry is an international research database containing key longitudinal data on a diverse and growing cohort of individuals affected by this group of rare and ultra-rare neuromuscular conditions. It can inform and support all areas of translational research including epidemiological and natural history studies, clinical trial feasibility planning, recruitment for clinical trials or other research studies, stand-alone clinical studies, standards of care development, and provision of real-world evidence data. For ten years, it has also served as a valuable communications tool and provided a link between the scientific and patient communities. With the anticipated advent of disease-modifying therapies for these conditions, the registry is a key resource for the generation of post-authorisation data for regulatory decision-making, real world evidence, and patient-reported outcome measures. In this paper we present some key data from the current 444 registered individuals with the following genotype split: MTM1 n=270, DNM2 n=42, BIN1 n=4, TTN n=4, RYR1 n=12, other n=4, unknown n=108. The data presented are consistent with the current literature and the common understanding of a strong genotype/phenotype correlations in CNM, most notably the data supports the current knowledge that XLMTM is typically the most severe form of CNM. Additionally, we outline the ways in which the registry supports research, and, more generally, the importance of continuous investment and development to maintain the relevance of registries for all stakeholders. Further information on the registry and contact details are available on the registry website at www.mtmcnmregistry.org.

Safety and efficacy of gene replacement therapy for X-linked myotubular myopathy (ASPIRO): a multinational, open-label, dose-escalation trial.

BACKGROUND: X-linked myotubular myopathy is a rare, life-threatening, congenital muscle disease observed mostly in males, which is caused by mutations in MTM1. No therapies are approved for this disease. We aimed to assess the safety and efficacy of resamirigene bilparvovec, which is an adeno-associated viral vector serotype 8 delivering human MTM1. METHODS: ASPIRO is an open-label, dose-escalation trial at seven academic medical centres in Canada, France, Germany, and the USA. We included boys younger than 5 years with X-linked myotubular myopathy who required mechanical ventilator support. The trial was initially in two parts. Part 1 was planned as a safety and dose-escalation phase in which participants were randomly allocated (2:1) to either the first dose level (1·3 × 1014 vector genomes [vg]/kg bodyweight) of resamirigene bilparvovec or delayed treatment, then, for later participants, to either a higher dose (3·5 × 1014 vg/kg bodyweight) of resamirigene bilparvovec or delayed treatment. Part 2 was intended to confirm the dose selected in part 1. Resamirigene bilparvovec was administered as a single intravenous infusion. An untreated control group comprised boys who participated in a run-in study (INCEPTUS; NCT02704273) or those in the delayed treatment cohort who did not receive any dose. The primary efficacy outcome was the change from baseline to week 24 in hours of daily ventilator support. After three unexpected deaths, dosing at the higher dose was stopped and the two-part feature of the study design was eliminated. Because of changes to the study design during its implementation, analyses were done on an as-treated basis and are deemed exploratory. All treated and control participants were included in the safety analysis. The trial is registered with ClinicalTrials.gov, NCT03199469. Outcomes are reported as of Feb 28, 2022. ASPIRO is currently paused while deaths in dosed participants are investigated. FINDINGS: Between Aug 3, 2017 and June 1, 2021, 30 participants were screened for eligibility, of whom 26 were enrolled; six were allocated to the lower dose, 13 to the higher dose, and seven to delayed treatment. Of the seven children whose treatment was delayed, four later received the higher dose (n=17 total in the higher dose cohort), one received the lower dose (n=7 total in the lower dose cohort), and two received no dose and joined the control group (n=14 total, including 12 children from INCEPTUS). Median age at dosing or enrolment was 12·1 months (IQR 10·0-30·9; range 9·5-49·7) in the lower dose cohort, 31·1 months (16·0-64·7; 6·8-72·7) in the higher dose cohort, and 18·7 months (10·1-31·5; 5·9-39·3) in the control cohort. Median follow-up was 46·1 months (IQR 41·0-49·5; range 2·1-54·7) for lower dose participants, 27·6 months (24·6-29·1; 3·4-41·0) for higher dose participants, and 28·3 months (9·7-46·9; 5·7-32·7) for control participants. At week 24, lower dose participants had an estimated 77·7 percentage point (95% CI 40·22 to 115·24) greater reduction in least squares mean hours per day of ventilator support from baseline versus controls (p=0·0002), and higher dose participants had a 22·8 percentage point (6·15 to 39·37) greater reduction from baseline versus controls (p=0·0077). One participant in the lower dose cohort and three in the higher dose cohort died; at the time of death, all children had cholestatic liver failure following gene therapy (immediate causes of death were sepsis; hepatopathy, severe immune dysfunction, and pseudomonal sepsis; gastrointestinal haemorrhage; and septic shock). Three individuals in the control group died (haemorrhage presumed related to hepatic peliosis; aspiration pneumonia; and cardiopulmonary failure). INTERPRETATION: Most children with X-linked myotubular myopathy who received MTM1 gene replacement therapy had important improvements in ventilator dependence and motor function, with more than half of dosed participants achieving ventilator independence and some attaining the ability to walk independently. Investigations into the risk for underlying hepatobiliary disease in X-linked myotubular myopathy, and the need for monitoring of liver function before gene replacement therapy, are ongoing. FUNDING: Astellas Gene Therapies.

Tracheostomy Timing During Pediatric Cardiac Intensive Care: Single Referral Center Retrospective Cohort.

OBJECTIVES: To describe associations between the timing of tracheostomy and patient characteristics or outcomes in the cardiac ICU (CICU). DESIGN: Single-institution retrospective cohort study. SETTING: Freestanding academic children's hospital. PATIENTS: CICU patients with tracheostomy placed between July 1, 2011, and July 1, 2020. INTERVENTIONS: We compared patient characteristics and outcomes between early and late tracheostomy based on the duration of positive pressure ventilation (PPV) before tracheostomy placement, fitting a receiver operating characteristic curve for current survival to define a cutoff. MEASUREMENTS AND MAIN RESULTS: Sixty-one patients underwent tracheostomy placement (0.5% of CICU admissions). Median age was 7.8 months. Eighteen patients (30%) had single ventricle physiology and 13 patients (21%) had pulmonary vein stenosis (PVS). Primary indications for tracheostomy were pulmonary/lower airway (41%), upper airway obstruction (UAO) (31%), cardiac (15%), neuromuscular (4%), or neurologic (4%). In-hospital mortality was 26% with 41% survival at the current follow-up (median 7.8 [interquartile range, IQR 2.6-30.0] mo). Late tracheostomy was defined as greater than or equal to 7 weeks of PPV which was equivalent to the median PPV duration pre-tracheostomy. Patients with late tracheostomy were more likely to be younger, have single ventricle physiology, and have greater respiratory severity. Patients with early tracheostomy were more likely to have UAO or genetic comorbidities. In multivariable analysis, late tracheostomy was associated with 4.2 times greater mortality (95% CI, 1.9-9.0). PVS was associated with higher mortality (adjusted hazard ratio [HR] 5.2; 95% CI, 2.5-10.9). UAO was associated with lower mortality (adjusted HR 0.2; 95% CI, 0.1-0.5). Late tracheostomy was also associated with greater cumulative opioid exposure. CONCLUSIONS: CICU patients who underwent tracheostomy had high in-hospital and longer-term mortality rates. Tracheostomy timing decisions are influenced by indication, disease, genetic comorbidities, illness severity, and age. Earlier tracheostomy was associated with lower sedative use and improved adjusted survival. Tracheostomy placement is a complex decision demanding individualized consideration of risk-benefit profiles and thoughtful family counseling.

Real-world analysis of healthcare resource utilization by patients with X-linked myotubular myopathy (XLMTM) in the United States.

BACKGROUND: X-linked myotubular myopathy (XLMTM) is a rare, life-threatening congenital myopathy with multisystem involvement, often requiring invasive ventilator support, gastrostomy tube feeding, and wheelchair use. Understanding healthcare resource utilization in patients with XLMTM is important for development of targeted therapies but data are limited. METHODS: We analyzed individual medical codes as governed by Healthcare Common Procedure Coding System, Current Procedural Terminology, and International Classification of Diseases, 10th Revision (ICD-10) for a defined cohort of XLMTM patients within a US medical claims database. Using third-party tokenization software, we defined a cohort of XLMTM patient tokens from a de-identified dataset in a research registry of diagnostically confirmed XLMTM patients and de-identified data from a genetic testing company. After approval of an ICD-10 diagnosis code for XLMTM (G71.220) in October 2020, we identified additional patients. RESULTS: A total of 192 males with a diagnosis of XLMTM were included: 80 patient tokens and 112 patients with the new ICD-10 code. From 2016 to 2020, the annual number of patients with claims increased from 120 to 154 and the average number of claims per patient per year increased from 93 to 134. Of 146 patients coded with hospitalization claims, 80 patients (55%) were first hospitalized between 0 and 4 years of age. Across all patients, 31% were hospitalized 1-2 times, 32% 3-9 times, and 14% ≥ 10 times. Patients received care from multiple specialty practices: pulmonology (53%), pediatrics (47%), neurology (34%), and critical care medicine (31%). The most common conditions and procedures related to XLMTM were respiratory events (82%), ventilation management (82%), feeding difficulties (81%), feeding support (72%), gastrostomy (69%), and tracheostomy (64%). Nearly all patients with respiratory events had chronic respiratory claims (96%). The most frequent diagnostic codes were those investigating hepatobiliary abnormalities. CONCLUSIONS: This innovative medical claims analysis shows substantial healthcare resource use in XLMTM patients that increased over the last 5 years. Most patients required respiratory and feeding support and experienced multiple hospitalizations throughout childhood and beyond for those that survived. This pattern delineation will inform outcome assessments with the emergence of novel therapies and supportive care measures.

Child Opportunity Index and Emergent PICU Readmissions: A Retrospective, Cross-Sectional Study of 43 U.S. Hospitals.

OBJECTIVES: To examine the association between a validated composite measure of neighborhood factors, the Child Opportunity Index (COI), and emergent PICU readmission during the year following discharge for survivors of pediatric critical illness. DESIGN: Retrospective cross-sectional study. SETTING: Forty-three U.S. children's hospitals contributing to the Pediatric Health Information System administrative dataset. PATIENTS: Children (< 18 yr) with at least one emergent PICU admission in 2018-2019 who survived an index admission. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Of 78,839 patients, 26% resided in very low COI neighborhoods, 21% in low COI, 19% in moderate COI, 17% in high COI, and 17% in very high COI neighborhoods, and 12.6% had an emergent PICU readmission within 1 year. After adjusting for patient-level demographic and clinical factors, residence in neighborhoods with moderate, low, and very low COI was associated with increased odds of emergent 1-year PICU readmission relative to patients in very high COI neighborhoods. Lower COI levels were associated with readmission in diabetic ketoacidosis and asthma. We failed to find an association between COI and emergent PICU readmission in patients with an index PICU admission diagnosis of respiratory conditions, sepsis, or trauma. CONCLUSIONS: Children living in neighborhoods with lower child opportunity had an increased risk of emergent 1-year readmission to the PICU, particularly children with chronic conditions such as asthma and diabetes. Assessing the neighborhood context to which children return following critical illness may inform community-level initiatives to foster recovery and reduce the risk of adverse outcomes.

Hospital Access Patterns of Children With Technology Dependence.

OBJECTIVES: We studied hospital utilization patterns among children with technology dependence (CTD). We hypothesized that increasing pediatric healthcare concentration requires those caring for CTD to selectively navigate healthcare systems and travel greater distances for care. METHODS: Using 2017 all-encounter datasets from 6 US states, we identified CTD visits defined by presence of a tracheostomy, gastrostomy, or intraventricular shunt. We calculated pediatric Hospital Capability Indices for hospitals and mapped distances between patient residence, nearest hospital, and encounter facility. RESULTS: Thirty-five percent of hospitals never saw CTD. Of 37 108 CTD encounters within the remaining 543 hospitals, most emergency visits (70.0%) and inpatient admissions (85.3%) occurred within 34 (6.3%) high capability centers. Only 11.7% of visits were to the closest facility, as CTD traveled almost 4 times further to receive care. When CTD bypassed nearer facilities, they were 10 times more likely to travel to high-capability centers (95% confidence interval: 9.43-10.8), but even those accessing low-capability facilities bypassed less capable, geographically closer hospitals. Transfer was more likely in nearest and low-capability facility encounters. CTD with Medicaid insurance, Black race, or from lower socioeconomic communities had lower odds of encounters at high-capability centers and of bypassing a closer institution than those with white race, private insurance, or from advantaged communities. CONCLUSIONS: Children with technology dependence routinely bypass closer hospitals to access care in facilities with higher pediatric capability. This access behavior leaves many hospitals unfamiliar with CTD, which results in greater travel but less transfers and may be influenced by sociodemographic factors.