Transitioning children using home invasive mechanical ventilation from hospital to home: Discharge criteria, disparities, and ethical considerations.

Children using home invasive mechanical ventilation (HIMV), a valuable therapeutic option for chronic respiratory failure, constitute a growing population. Transitioning children using HIMV from hospital to home care is a complex process that requires a multidisciplinary approach involving healthcare professionals, caregivers, and community resources. Medical stability, caregiver competence, and home environment suitability are essential factors in determining discharge readiness. Caregiver education and training play a pivotal role in ensuring safe and effective home care. Simulation training and staged education progression are effective strategies for equipping caregivers with necessary skills. Resource limitations, inadequate home nursing support, and disparities in available community resources are common obstacles to successful HIMV discharge. International perspectives shed light on diverse healthcare systems and challenges faced by caregivers worldwide. While standardizing guidelines for HIMV discharge may be complex, collaboration among healthcare providers and the development of evidence-based regional guidelines can improve outcomes for children using HIMV and their caregivers. This review seeks to synthesize literature, provide expert guidance based on experience, and highlight components to safely discharge children using HIMV. It further assesses disparities and divergences within regional and international healthcare systems while addressing relevant ethical considerations.

Improved tracheostomy-dependent patient outcomes after implementation of the Pediatric Resident Education in Pulmonary (PREP) Boot Camp.

INTRODUCTION: Children with tracheostomies are high risk for morbidity and mortality. Pediatric resident physicians are not routinely taught skills to care for this vulnerable patient population. Few reports link educational interventions to improved patient outcomes. This study evaluates the impact of an intensive educational training program on pediatric residents' observed skills and tracheostomy-dependent patient outcomes. METHODS: Pediatric post-graduate year 2 (PGY2) resident physicians rotating through the inpatient pediatric pulmonology month at Children's Hospital Colorado July 2018-2019 participated in the Pediatric Resident Education in Pulmonary (PREP) Boot Camp, an intensive educational program with an interactive lecture and simulation experience on patients with tracheostomy-dependence. PGY2s who partook in PREP and PGY3s who rotated before PREP initiation were invited to be studied. Primary outcomes included: (1) resident skills assessed by direct observation during simulation encounters and (2) rates of intensive care unit (ICU) transfers in tracheostomy-dependent patients following acute events before and after introduction of PREP. We hypothesized that increased education would enhance resident skills and improve patient outcomes by decreasing the rate of ICU transfers. RESULTS: PGY2 residents retained skills learned during PREP up to 11 months following initial participation, and significantly outperformed their PGY3 counterparts. There was a significant decrease in ICU transfer rate in patients with tracheostomies admitted to the pulmonary team during the 19 months following initiation of PREP. CONCLUSIONS: Enhanced early education may improve resident physicians' ability to care for complex patients with tracheostomies and could improve outcomes in this high-risk population.

Classifying multicenter approaches to invasive mechanical ventilation for infants with bronchopulmonary dysplasia using hierarchical clustering analysis.

INTRODUCTION: Evidence-based ventilation strategies for infants with severe bronchopulmonary dysplasia (BPD) remain unknown. Determining whether contemporary ventilation approaches cluster as specific BPD strategies may better characterize care and enhance the design of clinical trials. The objective of this study was to test the hypothesis that unsupervised, multifactorial clustering analysis of point prevalence ventilator setting data would classify a discrete number of physiology-based approaches to mechanical ventilation in a multicenter cohort of infants with severe BPD. METHODS: We performed a secondary analysis of a multicenter point prevalence study of infants with severe BPD treated with invasive mechanical ventilation. We clustered the cohort by mean airway pressure (MAP), positive end expiratory pressure (PEEP), set respiratory rate, and inspiratory time (Ti) using Ward's hierarchical clustering analysis (HCA). RESULTS: Seventy-eight patients with severe BPD were included from 14 centers. HCA classified three discrete clusters as determined by an agglomerative coefficient of 0.97. Cluster stability was relatively strong as determined by Jaccard coefficient means of 0.79, 0.85, and 0.77 for clusters 1, 2, and 3, respectively. The median PEEP, MAP, rate, Ti, and PIP differed significantly between clusters for each comparison by Kruskall-Wallis testing (p < 0.0001). CONCLUSIONS: In this study, unsupervised clustering analysis of ventilator setting data identified three discrete approaches to mechanical ventilation in a multicenter cohort of infants with severe BPD. Prospective trials are needed to determine whether these approaches to mechanical ventilation are associated with specific severe BPD clinical phenotypes and differentially modify respiratory outcomes.

Respiratory Outcomes for Ventilator-Dependent Children With Bronchopulmonary Dysplasia.

OBJECTIVES: To describe outpatient respiratory outcomes and center-level variability among children with severe bronchopulmonary dysplasia (BPD) who require tracheostomy and long-term mechanical ventilation. METHODS: Retrospective cohort of subjects with severe BPD, born between 2016 and 2021, who received tracheostomy and were discharged on home ventilator support from 12 tertiary care centers participating in the BPD Collaborative Outpatient Registry. Timing of key respiratory events including time to tracheostomy placement, initial hospital discharge, first outpatient clinic visit, liberation from the ventilator, and decannulation were assessed using Kaplan-Meier analysis. Differences between centers for the timing of events were assessed via log-rank tests. RESULTS: There were 155 patients who met inclusion criteria. Median age at the time of the study was 32 months. The median age of tracheostomy placement was 5 months (48 weeks' postmenstrual age). The median ages of hospital discharge and first respiratory clinic visit were 10 months and 11 months of age, respectively. During the study period, 64% of the subjects were liberated from the ventilator at a median age of 27 months and 32% were decannulated at a median age of 49 months. The median ages for all key events differed significantly by center (P ≤ .001 for all events). CONCLUSIONS: There is wide variability in the outpatient respiratory outcomes of ventilator-dependent infants and children with severe BPD. Further studies are needed to identify the factors that contribute to variability in practice among the different BPD outpatient centers, which may include inpatient practices.

Validation of an outpatient questionnaire for bronchopulmonary dysplasia control.

INTRODUCTION: Despite bronchopulmonary dysplasia (BPD) being a common morbidity of preterm birth, there is no validated objective tool to assess outpatient respiratory symptom control for clinical and research purposes. METHODS: Data were obtained from 1049 preterm infants and children seen in outpatient BPD clinics of 13 US tertiary care centers from 2018 to 2022. A new standardized instrument was modified from an asthma control test questionnaire and administered at the time of clinic visits. External measures of acute care use were also collected. The questionnaire for BPD control was validated in the entire population and selected subgroups using standard methodology for internal reliability, construct validity, and discriminative properties. RESULTS: Based on the scores from BPD control questionnaire, the majority of caregivers (86.2%) felt their child's symptoms were under control, which did not differ by BPD severity (p = 0.30) or a history of pulmonary hypertension (p = 0.42). Across the entire population and selected subgroups, the BPD control questionnaire was internally reliable, suggestive of construct validity (albeit correlation coefficients were -0.2 to -0.4.), and discriminated control well. Control categories (controlled, partially controlled, and uncontrolled) were also predictive of sick visits, emergency department visits, and hospital readmissions. CONCLUSION: Our study provides a tool for assessing respiratory control in children with BPD for clinical care and research studies. Further work is needed to identify modifiable predictors of disease control and link scores from the BPD control questionnaire to other measures of respiratory health such as lung function testing.

Standardizing electronic health record ventilation data in the pediatric long-term mechanical ventilator-dependent population.

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.

Approaches to Interdisciplinary Care for Infants with Severe Bronchopulmonary Dysplasia: A Survey of the Bronchopulmonary Dysplasia Collaborative.

OBJECTIVE: Bronchopulmonary dysplasia (BPD) remains the most common late morbidity for extremely premature infants. Care of infants with BPD requires a longitudinal approach from the neonatal intensive care unit to ambulatory care though interdisciplinary programs. Current approaches for the development of optimal programs vary among centers. STUDY DESIGN: We conducted a survey of 18 academic centers that are members of the BPD Collaborative, a consortium of institutions with an established interdisciplinary BPD program. We aimed to characterize the approach, composition, and current practices of the interdisciplinary teams in inpatient and outpatient domains. RESULTS: Variations exist among centers, including composition of the interdisciplinary team, whether the team is the primary or consult service, timing of the first team assessment of the patient, frequency and nature of rounds during the hospitalization, and the timing of ambulatory visits postdischarge. CONCLUSION: Further studies to assess long-term outcomes are needed to optimize interdisciplinary care of infants with severe BPD. KEY POINTS: · Care of infants with BPD requires a longitudinal approach from the NICU to ambulatory care.. · Benefits of interdisciplinary care for children have been observed in other chronic conditions.. · Current approaches for the development of optimal interdisciplinary BPD programs vary among centers..

Daycare Attendance is Linked to Increased Risk of Respiratory Morbidities in Children Born Preterm with Bronchopulmonary Dysplasia.

OBJECTIVES: To test the hypothesis that daycare attendance among children with bronchopulmonary dysplasia (BPD) is associated with increased chronic respiratory symptoms and/or greater health care use for respiratory illnesses during the first 3 years of life. STUDY DESIGN: Daycare attendance and clinical outcomes were obtained via standardized instruments for 341 subjects recruited from 9 BPD specialty clinics in the US. All subjects were former infants born preterm (<34 weeks) with BPD (71% severe) requiring outpatient follow-up between 0 and 3 years of age. Mixed logistic regression models were used to test for associations. RESULTS: Children with BPD attending daycare were more likely to have emergency department visits and systemic steroid usage. Children in daycare up to 3 years of age also were more likely to report trouble breathing, having activity limitations, and using rescue medications when compared with children not in daycare. More severe manifestations were found in children attending daycare between 6 and 12 months of chronological age. CONCLUSIONS: In this study, children born preterm with BPD who attend daycare were more likely to visit the emergency department, use systemic steroids, and have chronic respiratory symptoms compared with children not in daycare, indicating that daycare may be a potential modifiable risk factor to minimize respiratory morbidities in children with BPD during the preschool years.

A Literature Review of Feeding Disorders in Children with Tracheostomies and Ventilators.

Feeding disorders and gastrostomy use are highly prevalent in children with invasive mechanical ventilation (IMV) due to both common risk factors (eg, prematurity, neurological disorders) and resultant experiential deprivation (eg, long hospitalizations, delayed feeding experiences). Feeding in children with IMV is complicated by the presence of a tracheostomy, lung vulnerability, and medical complexity. The potential comorbidity of swallowing difficulties (dysphagia) and atypical early feeding experiences can result in complex feeding disorders. In this review of pediatric feeding disorders in children with invasive mechanical ventilation (IMV), we identify gaps in clinical translational research for this patient population and opportunities for improving evidence-based management. To improve long-term feeding outcomes and maximize oral feeding in this vulnerable population, children would benefit from earlier feeding opportunities during critical developmental windows, standardized protocols for advancing oral feeding, and involvement of intensive, comprehensive therapies throughout hospitalizations and early childhood. [Pediatr Ann. 2022;51(7):e291-e296.].

Posterior tracheopexy for preterm infants with severe bronchopulmonary dysplasia and severe tracheobronchomalacia: A case series.

We report a series of four patients with severe bronchopulmonary dysplasia (BPD) who underwent posterior tracheopexy for severe tracheomalacia (TM). While posterior tracheopexy is an established surgical treatment for TM associated with tracheoesophageal fistula, it has not been previously described in TM associated with BPD. There were no significant intraoperative or postoperative complications from the surgeries. Three of the four patients required tracheostomy and mechanical ventilation, which may reflect the degree of lung disease and other multisystem comorbidities in these patients. More investigation is needed to determine whether posterior tracheopexy is an effective surgical option for TM related to BPD.

Insurance coverage and respiratory morbidities in bronchopulmonary dysplasia.

INTRODUCTION: Preterm infants and young children with bronchopulmonary dysplasia (BPD) are at increased risk for acute care utilization and chronic respiratory symptoms during early life. Identifying risk factors for respiratory morbidities in the outpatient setting could decrease the burden of care. We hypothesized that public insurance coverage was associated with higher acute care usage and respiratory symptoms in preterm infants and children with BPD after initial neonatal intensive care unit (NICU) discharge. METHODS: Subjects were recruited from BPD clinics at 10 tertiary care centers in the United States between 2018 and 2021. Demographics and clinical characteristics were obtained through chart review. Surveys for clinical outcomes were administered to caregivers. RESULTS: Of the 470 subjects included in this study, 249 (53.0%) received employer-based insurance coverage and 221 (47.0%) received Medicaid as sole coverage at least once between 0 and 3 years of age. The Medicaid group was twice as likely to have sick visits (adjusted odd ratio [OR]: 2.06; p = 0.009) and emergency department visits (aOR: 2.09; p = 0.028), and three times more likely to be admitted for respiratory reasons (aOR: 3.04; p = 0.001) than those in the employer-based group. Additionally, those in the Medicaid group were more likely to have nighttime respiratory symptoms (aOR: 2.62; p = 0.004). CONCLUSIONS: Children with BPD who received Medicaid coverage were more likely to utilize acute care and have nighttime respiratory symptoms during the first 3 years of life. More comprehensive studies are needed to determine whether the use of Medicaid represents a barrier to accessing care, lower socioeconomic status, and/or a proxy for detrimental environmental exposures.

Single-cell transcriptional analysis of human endothelial colony-forming cells from patients with low VWF levels.

von Willebrand factor (VWF) plays a key role in normal hemostasis, and deficiencies of VWF lead to clinically significant bleeding. We sought to identify novel modifiers of VWF levels in endothelial colony-forming cells (ECFCs) using single-cell RNA sequencing (scRNA-seq). ECFCs were isolated from patients with low VWF levels (plasma VWF antigen levels between 30 and 50 IU/dL) and from healthy controls. Human umbilical vein endothelial cells were used as an additional control cell line. Cells were characterized for their Weibel Palade body (WPB) content and VWF release. scRNA-seq of all cell lines was performed to evaluate for gene expression heterogeneity and for candidate modifiers of VWF regulation. Candidate modifiers identified by scRNA-seq were further characterized with small-interfering RNA (siRNA) experiments to evaluate for effects on VWF. We observed that ECFCs derived from patients with low VWF demonstrated alterations in baseline WPB metrics and exhibit impaired VWF release. scRNA-seq analyses of these endothelial cells revealed overall decreased VWF transcription, mosaicism of VWF expression, and genes that are differentially expressed in low VWF ECFCs and control endothelial cells (control ECs). An siRNA screen of potential VWF modifiers provided further evidence of regulatory candidates, and 1 such candidate, FLI1, alters the transcriptional activity of VWF. In conclusion, ECFCs from individuals with low VWF demonstrate alterations in their baseline VWF packaging and release compared with control ECs. scRNA-seq revealed alterations in VWF transcription, and siRNA screening identified multiple candidate regulators of VWF.

Ventilatory Strategies in Infants with Established Severe Bronchopulmonary Dysplasia: A Multicenter Point Prevalence Study.

We performed a point prevalence study on infants with severe bronchopulmonary dysplasia (BPD), collecting data on type and settings of ventilatory support; 187 infants, 51% of whom were on invasive positive-pressure ventilation (IPPV), from 15 centers were included. We found a significant center-specific variation in ventilator modes.

Outpatient Respiratory Management of Infants, Children, and Adolescents with Post-Prematurity Respiratory Disease: An Official American Thoracic Society Clinical Practice Guideline.

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

Mechanical Ventilation During Chronic Lung Disease.

For infants with the most severe forms of chronic lung disease, regardless of etiology, chronic mechanical ventilation can provide stability, reduce acute respiratory events, and alleviate increased work of breathing. This approach prioritizes the baby's growth and development during early life. Once breathing comfortably, these infants can tolerate developmental therapies with the goal of achieving the best neurocognitive outcomes possible.