Managing established bronchopulmonary dysplasia without using routine blood gas measurements.

OBJECTIVE: Routine blood gas measurements are common in infants with severe bronchopulmonary dysplasia (sBPD) and are a noxious stimulus. We developed a guideline-driven approach to evaluate the care of infants with sBPD without routine blood gas sampling in the chronic phase of NICU care (after diagnosis at 36 weeks PMA). STUDY DESIGN: We examined blood gas utilization and outcomes in our sBPD inpatient care unit using data collected between 2014 and 2020. RESULTS: 485 sBPD infants met inclusion criteria, and 303 (62%) never had a blood gas obtained after 36 weeks PMA. In infants who had blood gas measurements, the median number of total blood gases per patient was only 4 (IQR 1-10). We did not identify adverse effects on hospital outcomes in patients without routine blood gas measurements. CONCLUSIONS: We found that patients with established BPD could be managed without routine blood gas analyses after 36 weeks PMA.

Multidisciplinary bronchopulmonary dysplasia care.

INTRODUCTION: Bronchopulmonary dysplasia (BPD) is a chronic respiratory disease in neonates and infants, which often presents with multisystem organ involvement, co-morbidities, and prolonged hospital stays. Therefore, a multidisciplinary chronic care approach is needed in the severest forms of BPD to optimize outcomes. However, this approach can be challenging to implement. The objective of this article is to review and synthesize the available literature regarding multidisciplinary care in infants and children with established BPD, and to provide a framework that can guide clinical practice and future research. AREAS COVERED: A literature search was conducted using Ovid MEDLINE, CINAHL, and Embase and several components of multidisciplinary management of BPD were identified and reviewed, including chronic care, team development, team members, discharge planning, and outpatient care. EXPERT OPINION: Establishing a core multidisciplinary group familiar with the chronicity of established BPD is recommended as best practice for this population. Acknowledging this is not feasible for all individual centers, it is important for clinical practice and future research to focus on the development and incorporation of national consulting services, telemedicine, and educational resources.

Respiratory management of established severe bronchopulmonary dysplasia.

Respiratory management of infants with established severe BPD is difficult and there is little evidence upon which to base decisions. Nonetheless, the physiology of severe BPD is well described with a predominantly obstructive pattern. This pulmonary dysfunction results in prolonged exhalatory time constants and thus ventilator management must be focused on maintaining adequate oxygenation and ventilation through achieving full exhalation. This approach is often difficult to maintain in acute care settings and a culture of chronic care focused on slow change and steady progress is imperative. Once respiratory stability is achieved, the focus should shift to growth and development and avoidance of care practices and medications that impair neurodevelopment.

Tracheostomy in Severe Bronchopulmonary Dysplasia-How to Decide in the Absence of Evidence.

Infants with the most severe forms of bronchopulmonary dysplasia (BPD) may require long-term invasive positive pressure ventilation for survival, therefore necessitating tracheostomy. Although life-saving, tracheostomy has also been associated with high mortality, postoperative complications, high readmission rates, neurodevelopmental impairment, and significant caregiver burden, making it a highly complex and challenging decision. However, for some infants tracheostomy may be necessary for survival and the only way to facilitate a timely and safe transition home. The specific indications for tracheostomy and the timing of the procedure in infants with severe BPD are currently unknown. Hence, centers and clinicians display broad variations in practice with regard to tracheostomy, which presents barriers to designing evidence-generating studies and establishing a consensus approach. As the incidence of severe BPD continues to rise, the question remains, how do we decide on tracheostomy to provide optimal outcomes for these patients?

Bronchodilator responsiveness and dysanapsis in bronchopulmonary dysplasia.

Background: The incidence of bronchopulmonary dysplasia (BPD) following preterm birth is increasing. Bronchodilators are often used to treat patients with BPD with little evidence to guide therapy. The aim of this study was to test the hypothesis that there are infant pulmonary function test (iPFT) parameters that can predict subsequent bronchodilator response in infants with BPD. Methods: Subjects in this study were part of a patient group in which we reported three BPD phenotypes (obstructive, restrictive and mixed) based on iPFT data. From that group, a cohort of 93 patients with iPFT data including bronchodilator response was eligible for this study. Results: Bronchodilator responsiveness was found in 59 people (63%) in the cohort. There were no differences in demographics between the responders and non-responders. There was no difference in forced vital capacity (FVC) between the two groups. Responders had significantly lower forced expiratory volume in 0.5 s (FEV0.5) and FEV0.5/FVC (p<0.005) and greater indices of hyperinflation than did non-responders (p<0.005). Logistic regression modelling found that pre-bronchodilator FEV0.5 and functional residual capacity/total lung capacity were significantly associated with bronchodilator response. The magnitude of response to bronchodilators was negatively correlated (R= -0.49, R2= 0.24, p<0.001) with the FEV0.5. The median dysanapsis ratio in responders (0.08, 95% CI 0.05-0.19) was significantly (p=0.005) smaller than in non-responders (0.18, 95% CI 0.06-0.38). Conclusion: These findings demonstrate that there are pulmonary function test parameters associated with bronchodilator response. Responders had evidence of greater dysanaptic lung growth than non-responders.

Tolerance of one-way in-line speaking valve trials in ventilator dependent children.

BACKGROUND: Despite the advantages of tracheostomy placement in children requiring prolonged mechanical ventilation, vocalization and verbal communication remains limited in this population of children. The lack of these essential elements during a critical period of development can have a negative impact on overall development. In ventilator dependent children, in-line speaking valves (ISV) provide an opportunity for initiating speech and communication. The objective of this study is to examine patient characteristics and risk factors associated with tolerance and success of ISV trials performed with mechanically ventilated children. METHODS: A retrospective cohort study was conducted at a large, tertiary care children's hospital to evaluate the outcomes of ISV trials in ventilator-dependent children with tracheostomies, from 2009 to 2019. The primary endpoints were tolerance of the initial ISV assessment, and successful completion of a trial. We compared demographic and clinical characteristics among children that had a successful ISV trial to those that did not. RESULTS: Eighty-nine patients were included, 56 (62%) were male and 33 (38%) were female. Overall, 76 (85%) patients completed an ISV assessment and trial successfully during their hospitalization. The number of attempts before completing a successful trial varied with 41 (46%) patients succeeding on the first attempt. Children that underwent a tracheostomy for airway obstruction were more likely to fail. CONCLUSIONS: Ventilator-dependent children with complex comorbidities demonstrate excellent tolerance of in-line speaking valves. Patients should be selected for ISV trials in a multidisciplinary setting. Airway obstruction as an indication for tracheostomy placement is a significant predictor of failure for ISV trials.

Perspectives on developing and sustaining a small baby program.

The Small Baby Program at Nationwide Children's Hospital was launched in 2004 in response to a need for better care for infants born extremely preterm. Standardization of care, decreased variability, multidisciplinary support, and robust research and quality improvement have allowed us to greatly improve our outcomes. In addition to the numerous medical and technological advances during this time, a strong commitment to kangaroo care and family-centered care have been integral to the growth and success of our program. The following review of the program aims to highlight the above areas while detailing the specific processes that have contributed to its ongoing success. Key areas of focus have been on respiratory management, neurodevelopmental care, and nutritional optimization. The implementation and continued refinement of the Small Baby Program has allowed us to improve the survival of extremely preterm infants, decrease certain morbidities, and improve long-term neurodevelopmental outcomes.

Noninvasive Respiratory Severity Indices Predict Adverse Outcomes in Bronchopulmonary Dysplasia.

OBJECTIVE: To test the hypothesis that elevated respiratory severity indices will identify patients with severe bronchopulmonary dysplasia (BPD) at the greatest risk for adverse in-hospital outcomes. STUDY DESIGN: This was a retrospective cohort study. A modified respiratory severity score (mean airway pressure × fraction of inspired oxygen) and a modified pulmonary score (respiratory support score × fraction of inspired oxygen + sum of medication scores) were calculated in a consecutive cohort of patients ≥36 weeks of postmenstrual age with severe BPD admitted to a referral center between 2010 and 2018. The association between each score and the primary composite outcome of death/prolonged length of stay (>75th percentile for cohort) was assessed using area under the receiver operator characteristic curve (AUROC) analysis and logistic regression. Death and the composite outcome death/tracheostomy were analyzed as secondary outcomes. RESULTS: In 303 patients, elevated scores were significantly associated with increased adjusted odds of death/prolonged length of stay: aOR 1.5 (95% CI 1.3-1.7) for the modified respiratory severity score and aOR 11.5 (95% CI 5.5-24.1) for the modified pulmonary score. The modified pulmonary score had slightly better discrimination of death/prolonged length of stay when compared with the modified respiratory severity score, AUROC 0.90 (95% CI 0.85-0.94) vs 0.88 (95% CI 0.84-0.93), P = .03. AUROCs for death and death/tracheostomy did not differ significantly when comparing the modified respiratory severity score with the modified pulmonary score. CONCLUSIONS: In our referral center, the modified respiratory severity score or the modified pulmonary score identified patients with established severe BPD at the greatest risk for death/prolonged length of stay, death, and death/tracheostomy.

Development of a small baby unit to improve outcomes for the extremely premature infant.

Survival and outcomes for extremely premature (EP) infants have improved and even infants born at 23 and 24 weeks that were previously considered non-viable are now routinely surviving. This review describes our particular institution's basis for and process of creating and sustaining a small baby program for a quaternary, referral-based neonatal intensive care unit. Through multi-disciplinary collaboration, small baby guidelines were developed that established uniform care and optimized evidence-based practice for the care of this unique patient population. A focus on parent-centered care while removing noxious stimuli for the patient has improved neurodevelopmental outcomes. Data collection, quality improvement, and ongoing research are incorporated in the small baby program to establish and sustain best practices and outcomes for the EP patient. Through the establishment of a small baby unit, we have improved survival, decreased short-term morbidities, and improved neurodevelopmental outcomes for the EP infant in our region.

In-hospital respiratory viral infections for patients with established BPD in the SARS-CoV-2 era.

OBJECTIVE: Our objective was to test the hypothesis that in-hospital respiratory viral infections (RVI) would be significantly lower in a cohort of patients with established bronchopulmonary dysplasia (BPD) exposed to a severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection prevention protocol when compared to historical controls. STUDY DESIGN: On April 1, 2020, we implemented a universal infection prevention protocol to minimize the risk of nosocomial SARS-CoV-2 transmission in a dedicated BPD intensive care unit. We performed a retrospective cohort study and included patients with established BPD, as defined by the 2019 Neonatal Research Network criteria, admitted to our center who underwent real-time polymerase-chain-reaction RVI testing between January 1, 2015 and March 31, 2021. We excluded patients readmitted from home. We compared the proportion of positive tests to the number of tests performed and the distribution of viral respiratory pathogens in the pre- and post-SARS-CoV-2 eras. RESULTS: Among 176 patients included in the study, 663 RVI tests were performed and 172 (26%) tests were positive. The median number of tests performed, measured in tests per patient per month, in the SARS-CoV-2 era was not significantly different compared to the pre-SARS-CoV-2 era (0.45 vs. 0.34 tests per patient per month, p = .07). The proportion of positive RVI tests was significantly lower in the SARS-CoV-2 era when compared to the pre-SARS-CoV-2 era (0.06 vs. 0.30, p < .0001). No patients tested positive for SARS-CoV-2 in the SARS-CoV-2 era. CONCLUSIONS: Infection prevention measures developed in response to the SARS-CoV-2 pandemic may reduce the risk of RVIs in hospitalized patients with established BPD.

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.

Evaluation of Short-term Outcomes of Tracheostomy Procedures in a NICU Population With High Ventilator Settings.

OBJECTIVE: To investigate whether tracheostomy placement in infants requiring high ventilator pressure is safe and effective. STUDY DESIGN: Case series with chart review. SETTING: Tertiary children's hospital. METHODS: Fifty ventilator-dependent neonatal intensive care unit patients who underwent tracheotomy from 2009 to 2018 were included. Patients requiring high ventilator pressures were compared to those requiring low ventilator pressures. Demographics, comorbidities, and surgical and clinical data were recorded. RESULTS: Thirty-two percent (n = 16) had low ventilator settings at the time of tracheostomy tube placement, and 68% (n = 34) had high ventilator settings. The median peak inspiratory pressure of the high ventilator group was 29.5 cm H2O, positive end-expiratory pressure (PEEP) was 8 cm H2O, mean airway pressure was 13 cm H2O, pressure support (PS) was 14 cm H2O, PS above PEEP was 6 cm H2O, and inspiratory time was 0.65 seconds. The high ventilator cohort had a higher median age at the time of surgery compared to the low ventilator group (P = .02). Female patients were more likely to have high ventilator settings (P = .02). There were no intraoperative complications or deaths within the first 7 days of tracheostomy tube placement. Pneumonia incidence and rate of mortality during admission did not vary by ventilator settings (P = .92 and P = .94, respectively). CONCLUSION: Few differences in tracheostomy tube placement outcomes were observed for patients with high ventilator settings compared to low ventilator settings. These data demonstrate that patients requiring high ventilator pressures can benefit from tracheostomy tube placement with no additional short-term risks.

In-hospital outcomes of late referrals for established bronchopulmonary dysplasia.

OBJECTIVE: To determine the in-hospital outcomes for patients with established bronchopulmonary dysplasia (BPD) referred late for severe disease. STUDY DESIGN: Retrospective cohort study of patients with established BPD referred to our center after 36 weeks PMA. RESULT: Among 71 patients with BPD referred to our center after 36 weeks PMA between 2010 and 2018, the median PMA was 47 weeks (IQR, 42, 53) and the median respiratory severity score was 8.1 (IQR 4.5, 11.0) on admission. Survival in this cohort was 92%. Most survivors were discharged home without the need for positive pressure respiratory support (77%) or pulmonary vasodilators (89%). For survivors, we observed a significant improvement in median z-scores for length (-6.7 vs -3.3, p < 0.0001) between admission and discharge. CONCLUSION: Despite presenting relatively late with a high degree of illness severity, nearly all patients in this cohort survived to hospital discharge with improvement in comorbidities.

Short-term complications and long-term morbidities associated with repeated unplanned extubations.

OBJECTIVE: To describe characteristics associated with repeated unplanned extubations, short-term complications and outcomes, and longer-term morbidities including acquired subglottic stenosis. STUDY DESIGN: Cohort study including neonates admitted to a tertiary care neonatal intensive care unit who experienced an unplanned extubation in a 5-year period. RESULTS: We reviewed 588 events involving 300 patients. Ten percent had airway trauma with reintubation, 42% required ≥2 reintubation attempts, and 39% led to increased baseline oxygen. Increased odds of repeated events were seen in patients with bronchopulmonary dysplasia and were associated with higher rates of tracheostomy and longer length of stay. The 9% of patients diagnosed with acquired subglottic stenosis had more unplanned extubations, higher rates of airway trauma and tracheitis, and were an older gestational age at birth. CONCLUSION: Unplanned extubations lead to short- and long-term morbidities. Certain patient characteristics are associated with increased odds of repeated events and the development of acquired subglottic stenosis.

Synergistic effects of sepsis and prematurity on neonatal postoperative mortality.

INTRODUCTION: Compared with term neonates, preterm babies are more likely to die from sepsis. However, the combined effects of sepsis and prematurity on neonatal postoperative mortality are largely unknown. Our objective was to quantify the proportion of neonatal postoperative mortality that is attributable to the synergistic effects of preoperative sepsis and prematurity. METHODS: We performed a multicentre, propensity-score-weighted, retrospective, cohort study of neonates who underwent inpatient surgery across hospitals participating in the United States National Surgical Quality Improvement Program-Pediatric (2012-2017). We assessed the proportion of the observed hazard ratio of mortality and complications that is attributable to the synergistic effect of prematurity and sepsis by estimating the attributable proportion (AP) and its 95% confidence interval (CI). RESULTS: We identified 19 312 neonates who realised a total of 321 321 person-days of postsurgical observations, during which 683 died (mortality rate: 2.1 per 1000 person-days). The proportion of mortality risk that is attributable to the synergistic effect of prematurity and sepsis was 50.5% (AP=50.5%; 95% CI, 28.8-72.3%; P < 0.001). About half of mortality events among preterm neonates with sepsis occurred within 24 h after surgery. Just over 45% of postoperative complications were attributable to the synergistic effect of prematurity and sepsis when both conditions were present (AP=45.8; 95% CI, 13.4-78.1%; P<0.001). CONCLUSION: Approximately half of postsurgical mortality and complications were attributable to the combined effect of sepsis and prematurity among neonates with both exposures. These neonates typically died within a few days after surgery, indicating a very narrow window of opportunity to predict and prevent mortality. CLINICAL TRIAL NUMBER AND REGISTRY: Not applicable.

Predicting survival in infants born at <27 weeks gestation admitted to an all referral neonatal intensive care unit: a pilot study.

BACKGROUND: There are no available predictive models for survival in extremely preterm (EP) infants admitted to a referral Level IV neonatal intensive care unit (NICU) after the first day of life (DOL). OBJECTIVE: To determine if there are clinical variables present at admission that are associated with survival in an outborn EP population. STUDY DESIGN: Data were analyzed from EP infants admitted before DOL 100 from 2008 to 2016 (n = 744). RESULTS: We found that prophylactic indomethacin (OR 1.98 (1.20-3.25) p = 0.007), admit DOL (OR 1.05 (1.02-1.08) p < 0.001), and birth in Franklin County, (OR 2.02 (95% CI, 1.04-3.90) p = 0.04) were all associated with survival. Using ROC analysis, the area under the curve for the provisional survival score was 0.69 (95% CI, 0.64-0.75; p < 0.0001). CONCLUSIONS: There are factors on admission to a referral Level IV NICU associated with survival in EP patients.