Evaluation of the Treatment of Stenotrophomonas maltophilia in Tracheostomy-Dependent Pediatric Patients.

OBJECTIVE: Tracheostomy-dependent pediatric patients will often have respiratory cultures positive for Stenotrophomonas maltophilia (multidrug-resistant Gram-negative rod). There are limited data available to guide treatment in this population. The objective of this single-center, retrospective study was to evaluate if antibiotic treatment of S maltophilia improved clinical outcomes in tracheostomy-dependent pediatric patients. METHODS: We included tracheostomy-dependent pediatric patients who had a respiratory culture positive for S maltophilia. Patients were divided into 2 groups: 1) treatment and 2) no treatment. RESULTS: Forty patients with 55 encounters were included in this study. S maltophilia was treated with sulfamethoxazole-trimethoprim in 20 encounters (19 patients) and no antimicrobial treatment was given in 35 encounters (30 patients). The time to return to stable respiratory status was 5 days (0-10) (median [range]) in the treated group and 4 days (0-19) in the untreated group (p = 0.52). There was no statistically significant difference in time to baseline respiratory status between patients treated and those not treated for S maltophilia. There was no difference in hospital length of stay between patients who were or not treated. CONCLUSIONS: Based on these results, these data would suggest that there might not be a benefit to treating cultures positive for S maltophilia in tracheostomy-dependent pediatric patients.

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.

Pediatric Ethics and Communication Excellence (PEACE) Rounds: Decreasing Moral Distress and Patient Length of Stay in the PICU.

This paper describes a practice innovation: the addition of formal weekly discussions of patients with prolonged PICU stay to reduce healthcare providers' moral distress and decrease length of stay for patients with life-threatening illnesses. We evaluated the innovation using a pre/post intervention design measuring provider moral distress and comparing patient outcomes using retrospective historical controls. Physicians and nurses on staff in our pediatric intensive care unit in a quaternary care children's hospital participated in the evaluation. There were 60 patients in the interventional group and 66 patients in the historical control group. We evaluated the impact of weekly meetings (PEACE rounds) to establish goals of care for patients with longer than 10 days length of stay in the ICU for a year. Moral distress was measured intermittently and reported moral distress thermometer (MDT) scores fluctuated. "Clinical situations" represented the most frequent contributing factor to moral distress. Post intervention, overall moral distress scores, measured on the moral distress scale revised (MDS-R), were lower for respondents in all categories (non-significant), and on three specific items (significant). Patient outcomes before and after PEACE intervention showed a statistically significant decrease in PRISM indexed LOS (4.94 control vs 3.37 PEACE, p = 0.015), a statistically significant increase in both code status changes DNR (11 % control, 28 % PEACE, p = 0.013), and in-hospital death (9 % control, 25 % PEACE, p = 0.015), with no change in patient 30 or 365 day mortality. The addition of a clinical ethicist and senior intensivist to weekly inter-professional team meetings facilitated difficult conversations regarding realistic goals of care. The study demonstrated that the PEACE intervention had a positive impact on some factors that contribute to moral distress and can shorten PICU length of stay for some patients.

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.

Nurse practitioner coverage is associated with a decrease in length of stay in a pediatric chronic ventilator dependent unit.

AIM: To hypothesize a dedicated critical care nurse practitioner (NP) is associated with a decreased length of stay (LOS) from a pediatric chronic ventilator dependent unit (PCVDU). METHODS: We retrospectively reviewed patients requiring care in the PCVDU from May 2001 through May 2011 comparing the 5 years prior to the 5 years post implementation of the critical care NP in 2005. LOS and room charges were obtained. RESULTS: The average LOS decreased from a median of 55 d [interquartile range (IQR): 9.8-108.3] to a median of 12 (IQR: 4.0-41.0) with the implementation of a dedicated critical care NP (P < 1.0001). Post implementation of a dedicated NP, a savings of 25738049 in room charges was noted over 5 years. CONCLUSION: Our data demonstrates a critical care NP coverage model in a PCVDU is associated with a significantly reduced LOS demonstrating that the NP is an efficient and likely cost-effective addition to a medically comprehensive service.

An Official American Thoracic Society Clinical Practice Guideline: Pediatric Chronic Home Invasive Ventilation.

BACKGROUND: Children with chronic invasive ventilator dependence living at home are a diverse group of children with special health care needs. Medical oversight, equipment management, and community resources vary widely. There are no clinical practice guidelines available to health care professionals for the safe hospital discharge and home management of these complex children. PURPOSE: To develop evidence-based clinical practice guidelines for the hospital discharge and home/community management of children requiring chronic invasive ventilation. METHODS: The Pediatric Assembly of the American Thoracic Society assembled an interdisciplinary workgroup with expertise in the care of children requiring chronic invasive ventilation. The experts developed four questions of clinical importance and used an evidence-based strategy to identify relevant medical evidence. Grading of Recommendations Assessment, Development, and Evaluation (GRADE) methodology was used to formulate and grade recommendations. RESULTS: Clinical practice recommendations for the management of children with chronic ventilator dependence at home are provided, and the evidence supporting each recommendation is discussed. CONCLUSIONS: Collaborative generalist and subspecialist comanagement is the Medical Home model most likely to be successful for the care of children requiring chronic invasive ventilation. Standardized hospital discharge criteria are suggested. An awake, trained caregiver should be present at all times, and at least two family caregivers should be trained specifically for the child's care. Standardized equipment for monitoring, emergency preparedness, and airway clearance are outlined. The recommendations presented are based on the current evidence and expert opinion and will require an update as new evidence and/or technologies become available.

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.

Profound hypoxemia and pulmonary hypertension in a 7-month-old infant: late presentation of alveolar capillary dysplasia.

OBJECTIVE: To report a case of an oldest previously asymptomatic infant diagnosed with alveolar capillary dysplasia who lived a relatively normal life until 7 months of age. DESIGN: Descriptive case report. SETTING: Intensive care unit of a tertiary care children's hospital. PATIENT: Seven-month-old female infant with profound hypoxemia and pulmonary hypertension. CONCLUSION: Alveolar capillary dysplasia should be considered with a high index of suspicion in an infant who presents with pulmonary hypertension beyond the neonatal period and for which no anatomical cause can be found. Early consideration of open lung biopsy may prevent using costly, invasive, and probably ineffective procedures such as extracorporeal membrane oxygenation.

The congenital porto-caval fistula: a unique presentation and novel intervention.

Congenital porto-caval fistulas are uncommon vascular malformations with a varied clinical presentation beginning in infancy and spanning late adulthood. We report a 14-year-old male who presented with dyspnea and cough. His past medical history was significant for a chronic non-immune hemolytic anemia, thrombocytopenia, coagulopathy and a learning disability. He was found to have severe pulmonary hypertension and hyperammonemia associated with a large congenital porto-caval fistula. The abnormal vessel was occluded via endovascular covered stent placement in the vena cava. His pulmonary hypertension has improved remarkably while his chronic anemia, thrombocytopenia and ammonia have normalized to allow improved cognitive performance.

Primary unilateral pulmonary hypoplasia: neonate through early childhood - case report, radiographic diagnosis and review of the literature.

Unilateral pulmonary hypoplasia is a rare cause of respiratory distress in the neonate. It is usually secondary to other causes such as diaphragmatic hernia. We present a case of a newborn with primary hypoplasia of the right upper lobe who was later found to also have tracheobronchomalacia. We describe the clinical course through early childhood.