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.

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.

Risk Factors for Foster Care Placement in Patients with Bronchopulmonary Dysplasia.

OBJECTIVE: Bronchopulmonary dysplasia (BPD) is a major cause of morbidity in neonates and can be associated with long hospitalization and high health care utilization. This extremely stressful situation can be difficult for many families and caregivers. The high-risk situation combined with increased medical complexity can result in involvement of Department of Child Services (DCS) and even foster care placement. This study seeks to define risk factors for DCS involvement and foster care placement in children with BPD. STUDY DESIGN: A retrospective study of children born at less than 32 weeks of gestation born between 2010 and 2016, on oxygen at 28 days of life and discharged home from a tertiary care center. RESULTS: A total of 246 patients were identified. DCS was involved in 49 patients with 13 requiring foster care placement. The most common correlated risk factors that were identified for DCS involvement were maternal THC (tetrahydrocannabinol) positivity, hospital policy violations, maternal mental health diagnosis, and home insecurity. Home insecurity (p < 0.005) and amphetamine use (p < 0.005) were associated with foster care placement. CONCLUSION: There are numerous risk factors for both DCS and foster care placement. The identification of these risk factors is important to help establish services to help families and identify potential biases to avoid. KEY POINTS: · There were both substance-related and non-substance-related risk factors for DCS involvement.. · Home insecurity and maternal amphetamine use were risk factors associated with foster care placement.. · This study fills the knowledge gap of risk factors for DCS and foster care placement in BPD..

Growth and nutrition in children with established bronchopulmonary dysplasia: A review of the literature.

BACKGROUND: Bronchopulmonary dysplasia (BPD) remains the most common late morbidity of preterm birth. Clinical care and research have largely focused on the pathogenesis and prevention of BPD. Preterm infants who develop BPD have significant medical needs that persist throughout their hospital course and continue after discharge, including those associated with growth and nutrition. The objective of this study was to review the available literature on nutrition and growth in infants with established BPD and to identify the knowledge and research gaps to provide direction for future studies. METHODS: We conducted a literature search in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines using Ovid MEDLINE, CINAHL, and Embase. Titles, abstracts, and full texts were independently reviewed by the authors and selected based on predetermined inclusion/exclusion criteria. Results were summarized qualitatively. RESULTS: Excluding duplicates, 2315 articles were identified. Thirty articles were selected for inclusion. We identified the following key components of nutrition support and clinical care: energy expenditure, growth, and metabolism; body composition; enteral nutrition; supplements; parenteral nutrition; and respiratory outcomes. CONCLUSIONS: Despite a large body of literature describing the role of growth and nutrition in the prevention of BPD, research is lacking with respect to interventions and management in the population with established BPD. Thus, organized approaches for clinical interventions and trials with respect to growth and nutrition in infants and young children with established BPD are needed. These studies should include multiple centers because of the small numbers of patients with BPD at each site.

Adjusting ventilator settings to avoid air trapping in extremely premature infants reduces the need for tracheostomy and length of stay.

Despite the improving understanding of how lung mechanics and tidal volume requirements evolve during the evolution of bronchopulmonary dysplasia (BPD), clinical management continues to be heterogeneous and inconsistent at many institutions. Recent reports have examined the use of high tidal-volume low respiratory rate strategies in these patients once disease has been well established to help facilitate their eventual extubation and improve their long-term neurodevelopmental outcomes. In this retrospective observational research study, we describe how intentional adjustment of ventilator settings based on patient lung mechanics by an interdisciplinary BPD team improved the care of the at-risk population of infants, reduced the need for tracheostomies, as well as length of stay over a period of over 3 years. The team aimed to establish consistency in the management of these children using a high tidal volume, low-rate approach, and titrating PEEP to address the autoPEEP and bronchomalacia that is frequently observed in this patient population.

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.

Characteristics of infants or children presenting to outpatient bronchopulmonary dysplasia clinics in the United States.

INTRODUCTION: Bronchopulmonary dysplasia (BPD) is a common respiratory sequelae of preterm birth, for which longitudinal outpatient data are limited. Our objective was to describe a geographically diverse outpatient cohort of former preterm infants followed in BPD-disease specific clinics. METHODS: Seven BPD specialty clinics contributed data using standardized instruments to this retrospective cohort study. Inclusion criteria included preterm birth (<37 weeks) and respiratory symptoms or needs requiring outpatient follow-up. RESULTS: A total of 413 preterm infants and children were recruited (mean age: 2.4 ± 2.7 years) with a mean gestational age of 27.0 ± 2.8 weeks and a mean birthweight of 951 ± 429 grams of whom 63.7% had severe BPD. Total, 51.1% of subjects were nonwhite. Severe BPD was not associated with greater utilization of acute care/therapies compared to non-severe counterparts. Of children with severe BPD, differences in percentage of those on any home respiratory support (p = .001), home positive pressure ventilation (p = .003), diuretics (p < .001), inhaled corticosteroids (p < .001), and pulmonary vasodilators (p < .001) were found between centers, however no differences in acute care use were observed. DISCUSSION: This examination of a multicenter collaborative registry of children born prematurely with respiratory disease demonstrates a diversity of management strategies among geographically distinct tertiary care BPD centers in the United States. This study reveals that the majority of children followed in these clinics were nonwhite and that neither variation in management nor severity of BPD at 36 weeks influenced outpatient acute care utilization. These findings suggest that post-neonatal intensive care unit factors and follow-up may modify respiratory outcomes in BPD, possibly independently of severity.

Feeding Outcomes for Infants with Bronchopulmonary Dysplasia Discharged on Nasogastric Feeds.

OBJECTIVE: While previous studies regarding neonatal home feeding regimens have demonstrated a more favorable prognosis for nasogastric (NG) versus gastrostomy tubes (GT), institutional practices of discharge on NG feeds are varied, particularly for infants with bronchopulmonary dysplasia (BPD). Little is known regarding the risk factors for patients treated with GT postdischarge in premature infants with BPD. Our objective was to identify frequency and risk factors for treating premature infants with BPD discharged on NG feeds with GT. STUDY DESIGN: In this retrospective study, we included infants born at 30 weeks' gestational age with BPD transitioning from a tertiary care center to home on NG feeds from 2010 to 2016. RESULTS: Of the 86 infants included in this study, 25 (29%) underwent GT placement at a median age of 264 days postdischarge. Fourteen (56%) were able to remove the GT at a median age of 979.5 days. Infants not requiring GT placement postdischarge were found to have a significantly higher ccipitofrontal circumference (p = 0.0089) and length (p = 0.0166) at discharge compared with infants with GT. CONCLUSION: NG feeding for infants with BPD appears to be a viable treatment with fewer patients (29%) requiring GT placement. Gestational age and abnormal magnetic resonance imaging results were found to have a significant association with GT placement postdischarge.

Correlation between the clinical severity of laryngomalacia and endoscopic findings.

OBJECTIVE: To correlate the clinical severity of laryngomalacia (LM) with endoscopic findings, swallowing evaluations and polysomnography in a cohort of patients. Method: We conducted a retrospective analysis between 2017-2018 on a cohort of patients diagnosed with upper airway obstruction (UAO), stridor, noisy breathing or laryngomalacia. This study took place at the Pediatric Pulmonology Department, Riley Children's Hospital, Indianapolis, United States of America. RESULTS:   There were 157 patients with laryngomalacia included in the study. Patients with severe LM were significantly younger than those with mild LM (p=0.0214) and moderate LM (p=0.0220). Subjects with type I of LM were significantly older than type III (p=0.0051). When associations were tested between polysomnogram (PSG) variables and clinical severity, there were significant associations with age at PSG. The overall apnea-hypopnea index (AHI) in mild (p=0.0103) and moderate (p=0.0242) were significantly lower than the severe group. The rapid eye movement (REM) AHI was significantly lower in moderate cases than severe (p=0.0134). The end-tidal carbon dioxide (EtCO2) peak was significantly lower in mild cases than severe (p=0.0141). The total sleep time (TST) peripheral capillary oxygen saturation (SpO2) 90% occurs in both mild (p=0.0197) and moderate (p=0.0498) were significantly lower than the severe group. CONCLUSIONS: The severity of the clinical manifestations of LM did not correlate with the different endoscopic types in our study. The presence of cyanosis was associated with type III LM. Rapid eye movement AHI and EtCO2 in polysomnogram were remained significantly associated with clinical severity.

Retrospective Analysis of Factors Leading to Pediatric Tracheostomy Decannulation Failure. A Single-Institution Experience.

RATIONALE: There is a lack of evidence regarding factors associated with failure of tracheostomy decannulation. OBJECTIVES: We aimed to identify characteristics of pediatric patients who fail a tracheostomy decannulation challenge Methods: A retrospective review was performed on all patients who had a decannulation challenge at a tertiary care center from June 2006 to October 2013. Tracheostomy decannulation failure was defined as reinsertion of the tracheostomy tube within 6 months of the challenge. Data on demographics, indications for tracheostomy, home mechanical ventilation, and comorbidities were collected. Data were also collected on specific airway endoscopic findings during the predecannulation bronchoscopy and airway surgical procedures before decannulation. We attempted to predict the decannulation outcome by analyzing associations. MEASUREMENTS AND MAIN RESULTS: 147 of 189 (77.8%) patients were successfully decannulated on the first attempt. Tracheostomy performed due to chronic respiratory failure decreased odds for decannulation failure (odds ratio = 0.34, 95% confidence interval = 0.15-0.77). Genetic abnormalities (45%) and feeding dysfunction (93%) were increased in the population of patients failing their first attempt. The presence of one comorbidity increased the odds of failure by 68% (odds ratio = 1.68, 95% confidence interval = 1.23-2.29). Decannulation pursuit based on parental expectation of success, rather than medically determined readiness, was associated with a higher chance of failure (P = 0.01). CONCLUSIONS: Our study highlights the role of genetic abnormalities, feeding dysfunction, and multiple comorbidities in patients who fail decannulation. Our findings also demonstrate that the outcome of decannulation may be predicted by the indication for tracheostomy. Patients who had tracheostomy placed for chronic respiratory support had a higher likelihood of success. Absence of a surgically treatable airway obstruction abnormality on the predecannulation bronchoscopy increased the chances of success.

Tracheostomy Following Surgery for Congenital Heart Disease: A 14-year Institutional Experience.

BACKGROUND: Tracheostomy following congenital heart disease (CHD) surgery is a rare event and associated with significant mortality. Hospital survival has been reported from 20% to 40%. Late mortality for these patients is not well characterized. METHODS: We performed a retrospective observational study of patients who had a tracheostomy following CHD surgery (excluding isolated patent ductus arteriosus ligation) between January 2000 and December 2013. Patients were categorized into single-ventricle or biventricular physiology groups. Demographics, genetic syndromes, pulmonary disease, and comorbidities were collected. Outcomes including hospital survival, long-term survival, and weaning from positive pressure ventilation are reported. Bivariate and time-to-event models were used. RESULTS: Over a 14-year period, 61 children (0.9% incidence) had a tracheostomy placed following CHD surgery. There were 12 single-ventricle patients and 49 biventricular patients. Prematurity, genetic syndromes, lung/airway disease, and other comorbidities were common in both CHD groups. Gastrostomy tubes were used more frequently in biventricular physiology patients (91.8%) versus single-ventricle patients (66.7%, P = .04). Survival to hospital discharge was 50% in the single-ventricle group compared to 86% in biventricular patients (P = .01). Long-term survival continued to be poor in the single-ventricle group comparatively (three years, 27.8% vs 64.8%, P = .01). Gastrostomy tube placement was independently associated with survival in both groups (P = .002). CONCLUSION: Tracheostomy is performed following many types of surgery for CHD and is commonly associated with other comorbidities. Both hospital and long-term survival are substantially lower in children with single-ventricle physiology as compared to patients with biventricular physiology.

Use of polysomnography to assess safe decannulation in children.

BACKGROUND: Tracheostomy is a lifesaving procedure to secure the airway and provide respiratory support. The decision to decannulate has classically been an individual physician decision without consensus among experts. The objective of this retrospective study was to assess the safety and efficacy of a standard institutional protocol that utilizes the sleep laboratory to assist in the decannulation process. METHODS: Between 2006 and 2013, patients were identified using a clinical database of decannulation studies. A protocol, finalized in 2005, was implemented for each decannulation attempt. In brief, all patients eligible for decannulation based on physician's assessment undergoes bronchoscopy. Once bronchoscopy findings reveal that the patient's airway is free of significant obstruction, decannulation is conducted in the sleep laboratory. The stoma is covered by an occlusive dressing and respiratory parameters are measured awake and asleep during the day and overnight by polysomnogram (PSG). The patient undergoes re-cannulation if the study shows significant obstruction, hypoventilation, or prolonged desaturation. RESULTS: A total of 210 decannulation attempts were performed on 189 patients (16 patients had multiple attempts). One hundred sixty-seven (79.5%) decannulation attempts were successful. Of those successfully decannulated, four (2.4%) were recannulated within 6 months. PSG parameters, specifically the apnea-hypopnea index, percent of total sleep time with oxygen saturation levels less than 90%, and lowest oxygen saturation levels were significantly associated with successful decannulation. No deaths occurred. CONCLUSIONS: We present a safe and successful decannulation protocol that includes bronchoscopy coupled with PSG evaluation of the patient with the stoma decannulated and covered by an occlusive dressing. Pediatr Pulmonol. 2016;51:796-802. © 2016 Wiley Periodicals, Inc.

Physiologic findings in children previously ventilator dependent at home due to bronchopulmonary dysplasia.

INTRODUCTION: Bronchopulmonary dysplasia (BPD) is the primary respiratory complication of premature birth. Some preterm newborns develop chronic respiratory failure, requiring home ventilator support. While physiologic measures have been described for prematurely born children, little is known about spirometric indices in patients with severe BPD who were previously ventilator dependent at home. METHODS: We retrospectively reviewed medical charts of patients with severe BPD who were ventilator dependent at home. We excluded patients with other comorbidities that could contribute to the severity of BPD. Spirometry was performed when the patient was able to follow commands. RESULTS: Between 1984 and 2012, within our severe BPD cohort who previously required home ventilator support, 19 patients were able to perform reproducible spirometry meeting ATS/ERS acceptability criteria. Ten (52.6%) were females, 13 (68.4%) were Caucasians. Mean age at liberation from ventilation was 2.4 years (C.I. 2.0, 2.9) and at decannulation was 3.5 years (C.I. 2.9, 4.0); median age at first reproducible spirometry measurement was 6.6 years (IQR: 4.9, 8.3). Spirometry results revealed significant airway obstruction, as demonstrated by Z-scores values of -1.5 (C.I. -2.5, -0.4) for FVC, -2.7 (C.I. -3.3, -1.9) for FEV1 , and -3.6 (C.I. -4.3, -2.9) for FEF25-75 . More so, serial spirometric measurements' slopes revealed that the airway obstruction remained static over time (FEV1 slope: -0.07, P-value: 0.2624; FVC slope: -0.01, P-value: 0.9064; and FEF25-75 : 0.0, P-value: 0.8532). CONCLUSIONS: Extreme prematurity associated with severe BPD requiring home ventilator support carries significant risks of morbidity. These patients had substantially diminished respiratory function reflecting airflow abnormalities that remained static over time.

Median Household Income: Association with Mortality in Children on Chronic Ventilation at Home Secondary to Bronchopulmonary Dysplasia.

Objective: The aim of this study was to determine if living in a lower income neighborhood is associated with mortality of patients with bronchopulmonary dysplasia (BPD) on home ventilation. Methods: Patients were divided into two groups by their ZIP code-based annual household income (Z-AHI), their year of birth, and the median state household income. Survival, liberation from ventilation, and decannulation rates were analyzed between the groups. Results: Over 27 years, 94 patients met our inclusion criteria: 58 (61.7%) were in the group with lower Z-AHI, and 36 (38.3%) were in the group with the Z-AHI above the median state household. Of the patients who died, 14/15 were in the lower Z-AHI group (p=0.003). Survival probability at 60 months of age showed no significant difference between the two groups: 81% [95% CI 70.9, 91.1] for the group with the Z-AHI below the median state household, and 100% [95% CI 100.0, 90.3] for the group with higher Z-AHI (p=0.31). Conclusions: The results of this study are descriptive, as the cause of the association between mortality rate and living in an area with lower household income is not yet understood. The difference in mortality rates between groups above and below the median state income suggests a serious health disparity, which warrants further study. Additional understanding of this effect requires more complete and direct measurement of socioeconomic status and individual characteristics, and better understanding of local environmental conditions.

Outcomes of children with severe bronchopulmonary dysplasia who were ventilator dependent at home.

OBJECTIVE: To describe the incidence and outcomes of children with chronic respiratory failure secondary to severe bronchopulmonary dysplasia (BPD) on chronic positive pressure ventilation (PPV) via tracheostomy at home. METHODS: We retrospectively reviewed medical charts of patients with severe BPD who were PPV dependent at home and who were enrolled in a university-affiliated home ventilator program between 1984 and 2010. We excluded patients with other comorbidities that could contribute to the development of chronic respiratory failure. We reported the incidence of these children in Indiana and cumulative incidences of survival, liberation from PPV, and decannulation. RESULTS: Over 27 years, 628 children were cared for in our home ventilator program. Of these, 102 patients met inclusion criteria: 83 (81.4%) were alive and 19 (18.6%) were deceased. Sixty-nine patients (67.6%) were liberated from PPV, and 97.1% of them were weaned before their fifth birthday, with a median age at liberation of 24 months (interquartile range, 19-33). Similarly, 60 patients (58.8%) were decannulated, of which 96.7% completed this process before their sixth birthday, with a median age at decannulation of 37.5 months (interquartile range, 31.5-45). The incidence of children with chronic respiratory failure secondary to BPD who were PPV-dependent at home in Indiana was 1.23 per 100 000 live births in 1984 and increased to 4.77 per 100 000 live births in 2010. CONCLUSIONS: Although extreme prematurity associated with severe BPD necessitating PPV at home carries significant risks of morbidity and mortality, successful liberation from mechanical ventilation and decannulation are likely to occur.