Outcomes of infants and children with bronchopulmonary dysplasia-associated pulmonary hypertension who required home ventilation.

BACKGROUND: To characterize a cohort of ventilator-dependent infants and children with bronchopulmonary dysplasia-associated pulmonary hypertension (BPD-PH) and to describe their cardiorespiratory outcomes. METHODS: Subjects with BPD on chronic home ventilation were recruited from outpatient clinics. PH was defined by its presence on ≥1 cardiac catheterization or echocardiogram on or after 36 weeks post-menstrual age. Kaplan-Meier analysis was used to compare the timing of key events. RESULTS: Of the 154 subjects, 93 (60.4%) had PH and of those, 52 (55.9%) required PH-specific medications. The ages at tracheostomy, transition to home ventilator, and hospital discharge were older in those with PH. Most subjects were weaned off oxygen and liberated from the ventilator by 5 years of age, which did not occur later in subjects with PH. The mortality rate after initial discharge was 2.6%. CONCLUSIONS: The majority of infants with BPD-PH receiving chronic invasive ventilation at home survived after initial discharge. Subjects with BPD-PH improved over time as evidenced by weaning off oxygen and PH medications, ventilator liberation, and tracheostomy decannulation. While the presence of PH was not associated with later ventilator liberation or decannulation, the use of PH medications may be a marker of a more protracted disease trajectory. IMPACT STATEMENT: There is limited data on long-term outcomes of children with bronchopulmonary dysplasia (BPD) who receive chronic invasive ventilation at home, and no data on those with the comorbidity of pulmonary hypertension (PH). Almost all subjects with BPD-PH who were on chronic invasive ventilation at home survived after their initial hospital discharge. Subjects with BPD-PH improved over time as evidenced by weaning off oxygen, PH medications, liberation from the ventilator, and tracheostomy decannulation. The presence of PH did not result in later ventilator liberation or decannulation; however, the use of outpatient PH medications was associated with later ventilation liberation and decannulation.

Respiratory culture growth and 3-years lung health outcomes in children with bronchopulmonary dysplasia and tracheostomies.

BACKGROUND: While bacteria identification on respiratory cultures is associated with poor short-term outcomes in children with bronchopulmonary dysplasia (BPD) and tracheostomies, the influence on longer-term respiratory support needs remains unknown. OBJECTIVE: To determine if respiratory culture growth of pathogenic organisms is associated with ongoing need for respiratory support, decannulation, and death at 3 years posttracheostomy placement in children with BPD and tracheostomies. METHODS: This single center, retrospective cohort study included infants and children with BPD and tracheostomies placed 2010-2018 and ≥1 respiratory culture obtained in 36 months posttracheostomy. Primary predictor was any pathogen identified on respiratory culture. Additional predictors were any Pseudomonas aeruginosa and chronic P. aeruginosa identification. Outcomes included continued use of respiratory support (e.g., oxygen, positive pressure), decannulation, and death at 3 years posttracheostomy. We used Poisson regression models to examine the relationship between respiratory organisms and outcomes, controlling for patient-level covariates and within-patient clustering. RESULTS: Among 170 children, 59.4% had a pathogen identified, 28.8% ever had P. aeruginosa, and 3.5% had chronic P. aeruginosa. At 3 years, 33.1% of alive children required ongoing respiratory support and 24.8% achieved decannulation; 18.9% were deceased. In adjusted analysis, any pathogen and P. aeruginosa were not associated with ongoing respiratory support or mortality. However, P. aeruginosa was associated with decreased decannulation probability (adjusted risk ratio 0.48, 95% CI 0.23-0.98). Chronic P. aeruginosa was associated with lower survival probability. CONCLUSION: Our findings suggest that respiratory pathogens including P. aeruginosa may not promote long-term respiratory dysfunction, but identification of P. aeruginosa may delay decannulation.

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.

Transitions of care: An aerodigestive provider assessment survey.

OBJECTIVE: To create, validate, and apply an aerodigestive provider assessment survey. METHODS: A survey assessing provider knowledge and current practice in the transition of patients with chronic aerodigestive disorders from pediatric to adult care was drafted by a multidisciplinary expert panel. Once agreement of the initial survey items was obtained, the survey was distributed to a national multidisciplinary panel of aerodigestive experts for review. Responses from the national panel were systematically quantified and a content validity index (CVI) was calculated. A final survey was developed and distributed to pediatric and adult aerodigestive providers. RESULTS: From the initial 22 items presented to the national panel, 20 of the initial questions were included in the final instrument. Two additional questions were developed as a result of feedback from the expert panel. All items included in the survey had an Item Content Validity Index (I-CVI) of >0.85. The average Scale CVI in proportion to the average proportion of relevance (S-CVI/Ave) for the tool was 0.88. The average Scale CVI in proportion to universal agreement (S-CVI/UA) was 0.52. The survey was then administered to pediatric and adult specialty providers at our institution. Twenty-two providers completed the final survey. CONCLUSION: The content validity index measurements from this newly developed survey suggest that it is a valid tool for assessing current knowledge and practice in care transitions among patients with complex aerodigestive needs. The survey developed in this project has been used to identify knowledge gaps and process issues that can be addressed to ease the transition of adolescents from pediatric specialty care into adult specialty care.

Factors associated with liberation from home mechanical ventilation and tracheostomy decannulation in infants and children with severe bronchopulmonary dysplasia.

OBJECTIVE: To identify factors associated with the timing of ventilator liberation and tracheostomy decannulation among infants with severe bronchopulmonary dysplasia (sBPD) who required chronic outpatient invasive ventilation. STUDY DESIGN: Multicenter retrospective study of 154 infants with sBPD on outpatient ventilators. Factors associated with ventilator liberation and decannulation were identified using Cox regression models and multilevel survival models. RESULTS: Ventilation liberation and decannulation occurred at median ages of 27 and 49 months, respectively. Older age at transition to a portable ventilator and at discharge, higher positive end expiratory pressure, and multiple respiratory readmissions were associated with delayed ventilator liberation. Surgical management of gastroesophageal reflux was associated with later decannulation. CONCLUSIONS: Ventilator liberation timing was impacted by longer initial admissions and higher ventilator pressure support needs, whereas decannulation timing was associated with more aggressive reflux management. Variation in the timing of events was primarily due to individual-level factors, rather than center-level factors.

Feeding Disorders in Children With Tracheostomy Tubes.

BACKGROUND: We aimed to describe feeding dysfunction in a group of children with tracheostomy. METHODS: Single-center, retrospective chart review of all children with a tracheostomy who were evaluated by our interdisciplinary feeding program. Demographic and diagnostic data, nutrition variables, acceptance of food consistencies, as well as 2 validated psychometric instruments for assessment of feeding dysfunction were analyzed. RESULTS: Thirteen tracheostomy-dependent children (5/13; 38% ventilator dependent) were evaluated at a median age of 51 months (interquartile range [IQR], 26-69). The majority of children (8/13; 62%) underwent evaluation after decannulation. Four children (30%) had a history of a cuffed tracheostomy tube. Eleven children (85%) used a speaking valve prior to decannulation, only 2 of whom started before initial discharge with a tracheostomy. Children with a tracheostomy had low-median weight- and height-for-age z-scores (-1.27 and -1.73, respectively), with normal-median body mass index (BMI)-for-age z-score (0.175). Children received 75% of feedings via tube feeding (IQR, 13%-97%). Compared with other children with feeding disorders, children with tracheostomy had delays in initial acceptance of most food textures and general diet, and the Mealtime Behavior Questionnaire showed significantly worse overall scores (P = .01), and the About Your Child's Eating survey showed significantly higher parental perception of resistance to eating (P = .0001). CONCLUSION: Requirement of enteral nutrition, poor oral-feeding skills, chronic malnutrition, and worse mealtime behaviors are associated with tracheostomy. A history of ventilator dependence, cuffed tracheostomy, and inpatient speaking valve-use were infrequently associated with interdisciplinary feeding-program evaluation.

Polysomnography Provides Useful Clinical Information in the Liberation from Respiratory Technology: A Retrospective Review.

Background The prevalence of respiratory-technology dependent children is increasing although for most children the goal is liberation from technology. Liberation from home mechanical ventilation (HMV) and decannulation strategies vary due to the lack of clinical practice standards. The primary objective of this study was to describe our practice utilizing a polysomnography (PSG) in the liberation from respiratory-technology process. Methods Retrospective study of tracheostomized children with and without HMV who underwent an evaluation for decannulation between January 2006 and June 2016. Patient demographics, indication for tracheostomy, indication for PSG, PSG results and interventions performed after the PSG were collected. RESULTS: We identified 153 decannulation attempts in 148 children. Ninety-nine children had a tracheostomy only and 49 children had a tracheostomy with HMV. There were 190 PSGs performed. Almost two-thirds of the children (N = 92) had at least one PSG, 37 children (25%) had two and 19 children (13%) had more than 2 PSGs. Children with tracheostomy and HMV had more PSGs compared to children with tracheostomy only. PSGs were performed at four points: (1) prior to tracheostomy placement (N = 23); (2) to titrate HMV (N = 19); (3) off-HMV support (N = 43); and with a capped tracheostomy (N = 101). Most of the off-HMV PSGs (N = 39) were favorable for discontinuing HMV. About two-thirds of the capped PSGs (N = 73) were favorable for decannulation; of the unfavorable capped PSGs (N = 28), thirteen required airway surgeries following the unfavorable PSG. CONCLUSION: : Overnight PSG provides useful information to the liberation process, particularly when determining readiness for discontinuing HMV and decannulation.

Liberation from home mechanical ventilation and decannulation in children.

BACKGROUND: The prevalence of children requiring outpatient invasive long-term mechanical ventilation is increasing. For some children, liberation from home mechanical ventilation (HMV) and decannulation is the desired outcome. This study describes our experience liberating tracheostomy and HMV (T-HMV) dependent children from respiratory technologies. METHODS: We reviewed charts of T-HMV dependent children who were cared for at our institution and decannulated between July 1999 and December 2011. Patient characteristics, diagnoses, and important steps leading to decannulation were recorded. RESULTS: Forty-six children achieved HMV independence and decannulation. The most common indications for T-HMV were lower airway and parenchymal lung disease. The median ages at tracheotomy, initiation of HMV, initiation of tracheostomy collar (TC) trials, HMV independence, and decannulation were 3.5, 6.0, 12.0, 25.5, and 40.5 months, respectively. Twenty-five children (54%) skipped either using a speaking valve, tracheostomy capping, or both without increased likelihood of recannulation. (P = 0.03). Common procedures prior to decannulation were airway surgery, bronchoscopy, and polysomnography (n = 30, 46, and 46 children, respectively). A median of 9.5 clinic visits and 5 hospitalizations occurred from initial hospital discharge to just prior to decannulation. HMV was primarily weaned as an outpatient. CONCLUSION: Liberation from respiratory technology is a complex, multi-step process that can be accomplished in medically complex children with varying underlying disease processes at relatively young ages. Five major steps (tracheotomy, initiation of HMV, initiation of TC trials, HMV independence, and decannulation) performed in conjunction with clinic visits, procedures, and home nursing support were integral in the successful decannulation process. Pediatr Pulmonol. 2016;51:838-849. © 2016 Wiley Periodicals, Inc.

Pleural effusion in a child with a ventriculoperitoneal shunt and congenital heart disease.

We present the unique case of an 8 month old infant who required extracorporeal membrane oxygenation (ECMO) after neonatal repair of tetralogy of Fallot. While on ECMO, he developed grade 3 intraventricular hemorrhage resulting in hydrocephalus requiring ventriculoperitoneal (VP) shunt placement at 5 months of life. He presented to cardiology clinic with a 2-month history of poor weight gain, tachypnea, and grunting and was found to have a large right sided pleural effusion. This was proven to be cerebrospinal fluid (CSF) accumulation secondary to poor peritoneal absorption with subsequent extravasation of CSF into the thoracic cavity via a diaphragmatic defect. After diaphragm repair, worsening ascites from peritoneal malabsorption led to shunt externalization and ultimate conversion to a ventriculoatrial (VA) shunt. This is the second reported case of VA shunt placement in a child with congenital heart disease and highlights the need to consider CSF extravasation as the cause of pleural effusions in children with VP shunts.