Update on Management and Outcomes of Congenital Diaphragmatic Hernia.

Infants with congenital diaphragmatic hernia (CDH) benefit from comprehensive multidisciplinary teams that have experience in caring for the unique and complex issues associated with CDH. Despite prenatal referral to specialized high-volume centers, advanced ventilation strategies and pulmonary hypertension management, and extracorporeal membrane oxygenation, mortality and morbidity remain high. These infants have unique and complex issues that begin in fetal and infant life, but persist through adulthood. Here we will review the literature and share our clinical care pathway for neonatal care and follow up. While many advances have occurred in the past few decades, our work is just beginning to continue to improve the mortality, but also importantly the morbidity of CDH.

Respiratory function after birth in infants with congenital diaphragmatic hernia.

OBJECTIVE: To characterise the transitional pulmonary physiology of infants with congenital diaphragmatic hernia (CDH) using measures of expiratory tidal volume (TV) and end-tidal carbon dioxide (ETCO2). DESIGN: Prospective single-centre observational study. SETTING: Quaternary neonatal intensive care unit. PATIENTS: Infants with an antenatal diagnosis of CDH born at the Children's Hospital of Philadelphia. INTERVENTIONS: TV and ETCO2 were simultaneously recorded using a respiratory function monitor (RFM) during invasive positive pressure ventilation immediately after birth. MAIN OUTCOME MEASURES: TV per birth weight and ETCO2 values were summarised for each minute after birth. Subgroups of interest were defined by liver position (thoracic vs abdominal) and extracorporeal membrane oxygenation (ECMO) treatment. RESULTS: RFM data were available for 50 infants from intubation until a median (IQR) of 9 (7-14) min after birth. TV and ETCO2 values increased for the first 10 min after birth, but intersubject values were heterogeneous. TVs were overall lower and ETCO2 values higher in infants with an intrathoracic liver and infants who were ultimately treated with ECMO. On hospital discharge, survival was 88% (n=43) and 34% (n=17) of infants were treated with ECMO. CONCLUSION: Respiratory function immediately after birth is heterogeneous for infants with CDH. Lung aeration, as evidenced by expired TV and ETCO2, appears to be ongoing throughout the first 10 min after birth during invasive positive pressure ventilation. Close attention to expired TV and ETCO2 levels by 10 min after birth may provide an opportunity to optimise and individualise ventilatory support for this high-risk population.

Use of ventilation/perfusion mismatch to guide individualised CPAP level selection in preterm infants: a feasibility trial.

OBJECTIVE: To measure within-subject changes in ventilation/perfusion (V'/Q') mismatch in response to a protocol of individualised nasal continuous positive airway pressure (CPAP) level selection. DESIGN: Single-arm, non-randomised, feasibility trial. SETTING: Three centres in the Children's Hospital of Philadelphia neonatal care network. PATIENTS: Twelve preterm infants of postmenstrual age 27-35 weeks, postnatal age >24 hours, and receiving a fraction of inspired oxygen (FiO2) >0.25 on CPAP of 4-7 cm H2O. INTERVENTIONS: We applied a protocol of stepwise CPAP level changes, with the overall direction and magnitude guided by individual responses in V'/Q' mismatch, as determined by the degree of right shift (kilopascals, kPa) in a non-invasive gas exchange model. Best CPAP level was defined as the final pressure level at which V'/Q' improved by more than 5%. MAIN OUTCOME MEASURES: Within-subject change in V'/Q' mismatch between baseline and best CPAP levels. RESULTS: There was a median (IQR) within-subject reduction in V'/Q' mismatch of 1.2 (0-3.2) kPa between baseline and best CPAP levels, p=0.02. Best CPAP was observed at a median (range) absolute level of 7 (5-8) cm H2O. CONCLUSIONS: Non-invasive measures of V'/Q' mismatch may be a useful approach for identifying individualised CPAP levels in preterm infants. The results of our feasibility study should be interpreted cautiously and replication in larger studies evaluating the impact of this approach on clinical outcomes is needed. TRIAL REGISTRATION NUMBER: NCT02983825.

Pediatric Simulation of Intrinsic PEEP and Patient-Ventilator Trigger Asynchrony During Mechanical Ventilation.

BACKGROUND: Intrinsic PEEP during mechanical ventilation occurs when there is insufficient time for expiration to functional residual capacity before the next inspiration, resulting in air trapping. Increased expiratory resistance (RE), too rapid of a patient or ventilator breathing rate, or a longer inspiratory to expiratory time ratio (TI/TE) can all be causes of intrinsic PEEP. Intrinsic PEEP can result in increased work of breathing and patient-ventilator asynchrony (PVA) during patient-triggered breaths. We hypothesized that the difference between intrinsic PEEP and ventilator PEEP acts as an inspiratory load resulting in trigger asynchrony that needs to be overcome by increased respiratory muscle pressure (Pmus). METHODS: Using a Servo lung model (ASL 5000) and LTV 1200 ventilator in pressure control mode, we developed a passive model demonstrating how elevated RE increases intrinsic PEEP above ventilator PEEP. We also developed an active model investigating the effects of RE and intrinsic PEEP on trigger asynchrony (expressed as percentage of patient-initiated breaths that failed to trigger). We then studied if trigger asynchrony could be reduced by increased Pmus. RESULTS: Intrinsic PEEP increased significantly with increasing RE (r = 0.97, P = .006). Multivariate logistic regression analysis showed that both RE and negative Pmus levels affect trigger asynchrony (P < .001). CONCLUSIONS: A passive lung model describes the development of increasing intrinsic PEEP with increasing RE at a given ventilator breathing rate. An active lung model shows how this can lead to trigger asynchrony since the Pmus needed to trigger a breath is greater with increased RE, as the inspiratory muscles must overcome intrinsic PEEP. This model will lend itself to the study of intrinsic PEEP engendered by a higher ventilator breathing rate, as well as higher TI/TE, and will be useful in ventilator simulation scenarios of PVA. The model also suggests that increasing ventilator PEEP to match intrinsic PEEP can improve trigger asynchrony through a reduction in RE.

Association of Racial Disparities With In-Hospital Outcomes in Severe Bronchopulmonary Dysplasia.

Importance: Bronchopulmonary dysplasia (BPD) is the most common serious morbidity of preterm birth. Short-term respiratory outcomes for infants with the most severe forms of BPD are highly variable. The mechanisms that explain this variability remain unknown and may be mediated by racial disparities. Objective: To determine the association of maternal race with death and length of hospital stay in a multicenter cohort of infants with severe BPD. Design, Setting, and Participants: This multicenter cohort study included preterm infants enrolled in the BPD Collaborative registry from January 1, 2015, to July 19, 2021, involving 8 BPD Collaborative centers located in the US. Included patients were born at less than 32 weeks' gestation, had a diagnosis of severe BPD as defined by the 2001 National Institutes of Health Consensus Criteria, and were born to Black or White mothers. Exposures: Maternal race: Black vs White. Main Outcomes and Measures: Death and length of hospital stay. Results: Among 834 registry infants (median [IQR] gestational age, 25 [24-27] weeks; 492 male infants [59%]) meeting inclusion criteria, the majority were born to White mothers (558 [67%]). Death was observed infrequently in the study cohort (32 [4%]), but Black maternal race was associated with an increased odds of death (adjusted odds ratio, 2.1; 95% CI, 1.2-3.5) after adjusting for center. Black maternal race was also significantly associated with length of hospital stay (adjusted between-group difference, 10 days; 95% CI, 3-17 days). Conclusions and Relevance: In a multicenter severe BPD cohort, study results suggest that infants born to Black mothers had increased likelihood of death and increased length of hospital stay compared with infants born to White mothers. Prospective studies are needed to define the sociodemographic mechanisms underlying disparate health outcomes for Black infants with severe BPD.

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.

Established severe BPD: is there a way out? Change of ventilatory paradigms.

Improved survival of extremely preterm newborn infants has increased the number of infants at risk for developing bronchopulmonary dysplasia (BPD). Despite efforts to prevent BPD, many of these infants still develop severe BPD (sBPD) and require long-term invasive mechanical ventilation. The focus of research and clinical management has been on the prevention of BPD, which has had only modest success. On the other hand, research on the management of the established sBPD patient has received minimal attention even though this condition poses large economic and health problems with extensive morbidities and late mortality. Patients with sBPD, however, have been shown to respond to treatments focused not only on ventilatory strategies but also on multidisciplinary approaches where neurodevelopmental support, growth promoting strategies, and aggressive treatment of pulmonary hypertension improve their long-term outcomes. In this review we will try to present a physiology-based ventilatory strategy for established sBPD, emphasizing a possible paradigm shift from acute efforts to wean infants at all costs to a more chronic approach of stabilizing the infant. This chronic approach, herein referred to as chronic phase ventilation, aims at allowing active patient engagement, reducing air trapping, and improving ventilation-perfusion matching, while providing sufficient support to optimize late outcomes. IMPACT: Based on pathophysiological aspects of evolving and established severe BPD in premature infants, this review presents some lung mechanical properties of the most severe phenotype and proposes a chronic phase ventilatory strategy that aims at reducing air trapping, improving ventilation-perfusion matching and optimizing late outcomes.

Development and validation of a novel informational booklet for pediatric long-term ventilation decision support.

OBJECTIVES: To provide accessible, uniform, comprehensive, and balanced information to families deciding whether to initiate long-term ventilation (LTV) for their child, we sought to develop and validate a novel informational resource. METHODS: The Ottawa Decision Support Framework was followed. Previous interviews with 44 lay and 15 professional stakeholders and published literature provided content for a booklet. Iterative versions were cognitive tested with six parents facing decisions and five pediatric intensivists. Ten parents facing decisions evaluated the booklet using the Preparation for Decision Making Scale and reported their decisional conflict, which was juxtaposed to the conflict of 21 parents who did not read it, using the Decisional Conflict Scale. Twelve home ventilation program directors evaluated the booklet's clinical sensibility and sensitivity, using a self-designed six-item questionnaire. Data presented using summary statistics. RESULTS: The illustrated booklet (6th-grade reading level) has nine topical sections on chronic respiratory failure and invasive and noninvasive LTV, including the option to forgo LTV. Ten parents who read the booklet rated it as helping "Quite a bit" or more on all items of the Preparation for Decision Making Scale and had seemingly less decisional conflict than 21 parents who did not. Twelve directors rated it highly for clinical sensibility and sensitivity. CONCLUSIONS: The LTV booklet was rigorously developed and favorably evaluated. It offers a resource to improve patient/family knowledge, supplement shared decision-making, and reduce decisional conflict around LTV decisions. Future studies should validate it in other settings and further study its effectiveness.

Pulmonary Manifestations of Congenital Heart Disease in Children.

This review addresses how anomalous cardiovascular anatomy imparts consequences to the airway, respiratory system mechanics, pulmonary vascular system, and lymphatic system. Abnormal formation or enlargement of great vessels can compress airways and cause large and small airway obstructions. Alterations in pulmonary blood flow associated with congenital heart disease (CHD) can cause abnormalities in pulmonary mechanics and limitation of exercise. CHD can lead to pulmonary arterial hypertension. Lymphatic abnormalities associated with CHD can cause pulmonary edema, chylothorax, or plastic bronchitis. Understanding how the cardiovascular system has an impact on pulmonary growth and function can help determine options and timing of intervention.

Therapy at 30 days of life predicts lung function at 6 to 12 months in infants with congenital diaphragmatic hernia.

INTRODUCTION: Congenital diaphragmatic hernia (CDH) is associated with variable degrees of lung hypoplasia. Pulmonary support at 30 days postnatal age was found to be the strongest predictor of inpatient mortality and morbidity among CDH infants and was also associated with higher pulmonary morbidity at 1 and 5 years. It is not known, however, if there is a relationship between the need for medical therapy at 30 days of life and subsequent abnormalities in lung function as reflected in infant pulmonary function test (iPFT) measurements. OBJECTIVE: We hypothesized that CDH infants who require more intensive therapy at 30 days would have more abnormal iPFT values at the time of their first infant pulmonary function study, reflecting the more severe spectrum of lung hypoplasia. METHODS: A single-institution chart review of all CDH survivors who were enrolled in a Pulmonary Hypoplasia Program (PHP) through July 2019, and treated from 2002 to 2019 was performed. All infants were divided into groups based on their need for noninvasive (supplemental oxygen, high flow therapy, noninvasive mechanical ventilation) or invasive (mechanical ventilation, extracorporeal membrane oxygenation) respiratory assistance, bronchodilators, diuretic use, and pulmonary hypertension (PH) therapy (inhaled and/or systemic drugs) at 30 days. Descriptive and statistical analyses were performed between groups comparing subsequent lung function measurements. RESULTS: A total of 382 infants (median gestational age [GA] 38.4 [interquartile range (IQR) = 37.1-39] weeks, 41.8% female, 70.9% Caucasian) with CDH were enrolled in the PHP through July 2019, and 118 infants underwent iPFT. The median age of the first iPFT was 6.6 (IQR = 5.3-11.7) months. Those requiring any pulmonary support at 30 days had a higher functional residual capacity (FRC) (z) (P = .03), residual volume (RV) (z) (P = .008), ratio of RV to total lung capacity (RV/TLC) (z) (P = .0001), and ratio of FRC to TLC (FRC/TLC) (z) (P = .001); a lower forced expiratory volume at 0.5 seconds (FEV0.5) (z) (P = .03) and a lower respiratory system compliance (Crs) (P = .01) than those who did not require any support. Similarly, those requiring diuretics and/or PH therapy at 30 days had higher fractional lung volumes, lower forced expiratory flows and Crs than infants who did not require such support (P < .05). CONCLUSIONS: Infants requiring any pulmonary support, diuretics and/or PH therapy at 30 postnatal days have lower forced expiratory flows and higher fractional lung volumes, suggesting a greater degree of lung hypoplasia. Our study suggests that the continued need for PH, diuretic or pulmonary support therapy at 30 days can be used as additional risk-stratification measurements for evaluation of infants with CDH.

Decisions for Long-Term Ventilation for Children. Perspectives of Family Members.

Rationale: The decision whether to initiate or forgo long-term ventilation for children can be difficult and impactful. However, little has been published on the informational and decisional needs of families facing this decision.Objectives: To assess what families with children with chronic respiratory failure and life-limiting conditions need and want for informed decision-making.Methods: English- and Spanish-speaking parents who were facing (contemporaneous decision makers) or had previously faced (former decision makers) a decision regarding invasive or noninvasive long-term ventilation for their children were recruited using convenience sampling. Patients who were older and cognitively capable also were invited to participate. We performed semistructured interviews using an open-ended interview guide developed de novo to assess parents' decisional needs and experiences. Qualitative data analysis used a thematic approach based on framework analysis, and thematic saturation was a goal.Results: A sample of 44 parents and 2 patients from 43 families was interviewed. All contemporaneous decision makers (n = 28) favored or believed that they would choose long-term ventilation. Fifteen of 16 former decision makers chose long-term ventilation. Thematic saturation was achieved from the perspective of parents who favored or chose long-term ventilation. Four domains were identified: parents' emotional and psychological experiences with decision-making, parents' informational needs, parents' communication and decision support needs, and parents' views on the option not to initiate long-term ventilation. For most parents, making a decision regarding long-term ventilation was stressful, even though they articulated goals and values that could/did guide their decision-making. In general, parents wanted comprehensive information, including what life would be like at home for the child and the family. They wanted their medical providers to be honest, tactful, patient, and supportive. Parents reported that they felt being presented with the option not to initiate was acceptable.Conclusions: In this study, we identified specific informational and decision-making needs regarding long-term ventilation that parents facing decisions feel are important. These data suggest that providers should present families with comprehensive, balanced information on the impact of long-term ventilation and, when the child has a profoundly serious and life-limiting condition, explore the option not to initiate long-term ventilation.

Transition of patients with neuromuscular disease and chronic ventilator-dependent respiratory failure from pediatric to adult pulmonary care.

Improvements in medical care have allowed many children with neuromuscular disease and chronic respiratory failure to survive into adulthood. There are currently no published guidelines to facilitate transition from pediatric to adult respiratory care in this population. The transition process in neuromuscular disease and chronic respiratory failure is uniquely challenging in that the patients are often declining in health and losing independence as they approach adulthood. Barriers to transition include lack of access to adult providers, incompatible health insurance, loss of resources within patients' medical structures, absence of transition preparation, and patient and family insecurity with a new healthcare system. The six core elements and optimal time frame of transition should be applied, with special consideration of the psychosocial aspects associated with neuromuscular disease. Successful transition revolves around information, open communication between young adults and their medical care team, and individualized planning to ensure optimal health and quality of life.

Transitioning from an ICU ventilator to a portable home ventilator.

There is a variety of portable ventilators on the market, each with its' own features. A clinician needs to understand the unique characteristics of the ventilators available in his or her region, as well as the nuances of primary and secondary settings for these portable home ventilators in order to create a comfortable breath that allows for adequate gas exchange for the patient. Understanding the interplay of the portable home ventilator and the ventilator circuit is also a key component of transitioning a patient to a portable home ventilator. This review details characteristics of some of the more commonly used machines in the United States, as well as the settings to be considered in supporting a child with chronic respiratory failure outside of the hospital. As more patients are being discharged from the hospital with mechanical home ventilation, new ventilators are being developed that expand upon features of current machines.

Identifying and treating intrinsic PEEP in infants with severe bronchopulmonary dysplasia.

RATIONALE: Infants with severe bronchopulmonary dysplasia (sBPD) and airway obstruction may develop dynamic hyperinflation and intrinsic positive end-expiratory pressure (PEEPi ), which impairs patient/ventilator synchrony. OBJECTIVES: To determine if PEEPi is present in infants with sBPD during spontaneous breathing and if adjusting ventilator PEEP improves patient/ventilator synchrony and comfort. METHODS: Interventional study in infants with sBPD. PEEPi measured by esophageal pressure (Pes) and pneumotachometer, during pressure-supported breaths. PEEP i defined as the difference between Pes at start of the inspiratory effort minus Pes at onset of inspiratory flow. The set PEEP was adjusted to minimize PEEP i . "Best PEEP" was the setting with minimal wasted efforts (WE), an inspiratory effort seen on the Pes waveform without a corresponding ventilator breath. FiO 2 and SpO 2 measured pre- and post-PEEP adjustment. Sedation requirements evaluated 72 hours preprocedure and postprocedure. RESULTS: Twelve infants were assessed (gestational age, 24.9 ± 1.4 weeks; study age, 48.8 ± 1.5 weeks, postmenstrual age). Mean baseline ventilator PEEP was 16.4 cm H2 O (14-20 cm H 2 O). Eight infants required an increase, one, a reduction, and three, no change in the set PEEP. For the eight infants requiring an increase in set PEEP, there was an 18.9% reduction in WE and a reduction in FiO 2 (0.084 ± 0.058) requirements in the subsequent 24 hours. Conditional sedation was reduced in five infants postprocedure. No adverse events occurred during testing. CONCLUSION: PEEPi is measurable in infants with sBPD with concurrent esophageal manometry and flow-time tracings without the need for pharmacological paralysis. In those with PEEP i , increasing ventilator PEEP to offset PEEP i improves synchrony.

Home Oxygen Therapy for Children. An Official American Thoracic Society Clinical Practice Guideline.

BACKGROUND: Home oxygen therapy is often required in children with chronic respiratory conditions. This document provides an evidence-based clinical practice guideline on the implementation, monitoring, and discontinuation of home oxygen therapy for the pediatric population. METHODS: A multidisciplinary panel identified pertinent questions regarding home oxygen therapy in children, conducted systematic reviews of the relevant literature, and applied the Grading of Recommendations, Assessment, Development, and Evaluation approach to rate the quality of evidence and strength of clinical recommendations. RESULTS: After considering the panel's confidence in the estimated effects, the balance of desirable (benefits) and undesirable (harms and burdens) consequences of treatment, patient values and preferences, cost, and feasibility, recommendations were developed for or against home oxygen therapy specific to pediatric lung and pulmonary vascular diseases. CONCLUSIONS: Although home oxygen therapy is commonly required in the care of children, there is a striking lack of empirical evidence regarding implementation, monitoring, and discontinuation of supplemental oxygen therapy. The panel formulated and provided the rationale for clinical recommendations for home oxygen therapy based on scant empirical evidence, expert opinion, and clinical experience to aid clinicians in the management of these complex pediatric patients and identified important areas for future research.

Tidal Breathing Measurements at Discharge and Clinical Outcomes in Extremely Low Gestational Age Neonates.

RATIONALE: The relationship between respiratory function at hospital discharge and the severity of later respiratory disease in extremely low gestational age neonates is not well defined. OBJECTIVES: To test the hypothesis that tidal breathing measurements near the time of hospital discharge differ between extremely premature infants with bronchopulmonary dysplasia (BPD) or respiratory disease in the first year of life and those without these conditions. METHODS: Study subjects were part of the PROP (Prematurity and Respiratory Outcomes Program) study, a longitudinal cohort study of infants born at less than 29 gestational weeks followed from birth to 1 year of age. Respiratory inductance plethysmography was used for tidal breathing measurements before and after inhaled albuterol 1 week before anticipated hospital discharge. Infants were breathing spontaneously and were receiving less than or equal to 1 L/min nasal cannula flow at 21% to 100% fraction of inspired oxygen. A survey of respiratory morbidity was administered to caregivers at 3, 6, 9, and 12 months corrected age to assess for respiratory disease. We compared tidal breathing measurements in infants with and without BPD (oxygen requirement at 36 wk) and with and without respiratory disease in the first year of life. Measurements were also performed in a comparison cohort of term infants. RESULTS: A total of 765 infants survived to 36 weeks postmenstrual age, with research-quality tidal breathing data in 452 out of 564 tested (80.1%). Among these 452 infants, the rate of postdischarge respiratory disease was 65.7%. Compared with a group of 18 term infants, PROP infants had abnormal tidal breathing patterns. However, there were no clinically significant differences in tidal breathing measurements in PROP infants who had BPD or who had respiratory disease in the first year of life compared with those without these diagnoses. Bronchodilator response was not significantly associated with respiratory disease in the first year of life. CONCLUSIONS: Extremely premature infants receiving less than 1 L/min nasal cannula support at 21% to 100% fraction of inspired oxygen have tidal breathing measurements that differ from term infants, but these measurements do not differentiate those preterm infants who have BPD or will have respiratory disease in the first year of life from those who do not. Clinical trial registered with www.clinicaltrials.gov (NCT01435187).

Survey of financial burden of families in the U.S. with children using home mechanical ventilation.

AIM: To describe and quantify the out-of-pocket expenses, employment loss, and other financial impact related to caring for a child using home mechanical ventilation (HMV). METHOD: We conducted a cross-sectional survey of U.S. families with children who used HMV. Eligible participants were invited to complete a questionnaire addressing household and child characteristics, out-of-pocket expenses, employment loss/reduction, and financial stress. Participants were recruited with the help of three national patient registries. RESULTS: Two hundred twenty-six participants from 32 states (152 with children who used invasive ventilation and 74 with children who used noninvasive ventilation) completed the questionnaire. Participants' median reported yearly household income was $90 000 (IQR 70 000-150 000). The median amount paid in out-of-pocket expenses in the previous 3 months to care for their child using HMV totaled $3899 (IQR $2900-4550). Reported levels of financial stress decreased as income increased; 37-60% of participants, depending on income quintile, reported moderate financial stress with "some" of that stress due to their out-of-pocket expenses. A substantial majority reported one or more household members stopped or reduced work and took unpaid weeks off of work to care for their child. CONCLUSION: The financial impact of caring for a child using HMV is considerable for some families. Providers need to understand these financial burdens and should inform families of them to help families anticipate and plan for them.

Tracheobronchomalacia Is Associated with Increased Morbidity in Bronchopulmonary Dysplasia.

RATIONALE: Tracheobronchomalacia is a common comorbidity in neonates with bronchopulmonary dysplasia. However, the effect of tracheobronchomalacia on the clinical course of bronchopulmonary dysplasia is not well-understood. OBJECTIVE: We sought to assess the impact of tracheobronchomalacia on outcomes in neonates with bronchopulmonary dysplasia in a large, multi-center cohort. METHODS: We preformed a cohort study of 974 neonates with bronchopulmonary dysplasia admitted to 27 neonatal intensive care units participating in the Children's Hospital Neonatal Database who had undergone bronchoscopy. In hospital morbidity for neonates with bronchopulmonary dysplasia and tracheobronchomalacia (N=353, 36.2%) was compared to those without tracheobronchomalacia (N=621, 63.8%) using mixed-effects multivariate regression. RESULTS: Neonates with tracheobronchomalacia and bronchopulmonary dysplasia had more comorbidities, such as gastroesophageal reflux (OR=1.65, 95%CI 1.23- 2.29, P=0.001) and pneumonia (OR=1.68, 95%CI 1.21-2.33, P=0.002) and more commonly required surgeries such as tracheostomy (OR=1.55, 95%CI 1.15-2.11, P=0.005) and gastrostomy (OR=1.38, 95%CI 1.03-1.85, P=0.03) compared with those without tracheobronchomalacia. Neonates with tracheobronchomalacia were hospitalitized (118 ± 93 vs 105 ± 83 days, P=0.02) and ventilated (83.1 ± 91.1 vs 67.2 ± 71.9 days, P=0.003) longer than those without tracheobronchomalacia. Upon discharge, neonates with tracheobronchomalacia and BPD were more likely to be mechanically ventilated (OR=1.37, 95CI 1.01-1.87 P=0.045) and possibly less likely to receive oral nutrition (OR=0.69, 95%CI 0.47-1.01, P=0.058). CONCLUSIONS: Tracheobronchomalacia is common in neonates with bronchopulmonary dysplasia who undergo bronchoscopy and is associated with longer and more complicated hospitalizations.

Decisions around Long-term Ventilation for Children. Perspectives of Directors of Pediatric Home Ventilation Programs.

RATIONALE: The decision of whether to initiate or forgo long-term ventilation (LTV) for children with life-limiting conditions can be complex and impactful. Providers are responsible for helping families to understand the consequences of their options and guiding them through shared decision-making, but little has been published on how to do this. OBJECTIVES: To assess how directors of pediatric home ventilation programs facilitate shared decision-making with families facing decisions of whether to initiate or forgo LTV for their children with life-limiting conditions. In addition, to assess directors' perspectives on these families' decisional needs. METHODS: Purposeful recruiting of directors/codirectors of pediatric home ventilation programs at children's hospitals was used. We performed semistructured interviews using an open-ended interview guide developed de novo to assess their approach to informed, shared decision-making around LTV and their perspectives on these decisions. Qualitative data analysis was conducted using a thematic approach based on framework analysis in which thematic saturation was achieved. RESULTS: A sample of 15 experienced physician directors across North America was interviewed. All (15/15) inform families of the potential benefits and burdens/risks of LTV for the child and of the option to forgo LTV. All stress to families the physical, emotional, and social impact of caring for a child using LTV on the family; 12 directors also highlight the financial impact. All recommend that decision-making around LTV should be interdisciplinary, initiated early, and not rushed; nine described their approach as guided by the family's goals for the child and their family. All recommend that providers be transparent, candid, active listeners, and supportive. All directors believe that the family's decision should be respected, but vary in the extent to which they recommend an option to families. They described barriers to decision-making that stem from families, providers, and other sources. CONCLUSIONS: As providers who follow children using LTV, directors of pediatric home ventilation programs have perspectives regarding the decisional needs of these families and how to meet them that can help inform and shape the practices of other providers who assist families facing this decision.