PURPOSE: The need for pediatric post-acute facility care (PAC) is growing due to technological advances that extend the lives of many children, especially those with complex medical needs. The objectives were to describe [1] the types and settings of PAC; [2] the clinical characteristics of the pediatric patients requiring PAC; and [3] perceptions of PAC care delivery by clinical staff. METHODS: An online survey was administered between 6/2018 to 12/2018 to administrative leaders in PAC facilities that have licensed beds for children and who were active members of the Pediatric Complex Care Association. Survey topics included types of health services provided; pediatric patient characteristics; clinical personnel characteristics; and perceptions of pediatric PAC health care delivery. RESULTS: Leaders from 26 (54%) PAC facilities in 16 U.S. states completed the survey. Fifty-four percent identified as skilled nursing facility/long-term care, 19% intermediate care facilities, 15% respite and medical group homes, and 12% post-acute rehabilitation facilities. Sixty-nine percent of facilities had a significant increase in the medical complexity of patients over the past 10 years. Most reported capability to care for children with tracheostomy/invasive ventilation (100%), gastrostomy tubes (96%), intrathecal baclofen pump (89%), non-invasive positive pressure ventilation (85%), and other medical technology. Most facilities (72%) turned away patients for admission due to bed unavailability occasionally or always. Most facilities (62%) reported that insurance reimbursement to cover the cost of providing PAC to children was not acceptable, and most reported that it was difficult to hire clinical staff (77%) and retain staff (58%). CONCLUSION: PAC in the U.S. is provided to an increasingly medically-complex population of children. There is a critical need to investigate financially-viable solutions for PAC facilities to meet the patient demands for their services and to sufficiently reimburse and retain staff for the challenging and important care that they provide.

Objective To compare daytime and nighttime use and outcome for non-invasive respiratory intervention (NIRI) during initial admission to a pediatric post-acute care hospital (PACH). Method Retrospective examination of initial PACH admissions (October 2018 through September 2020) for infants and children requiring NIRI during the day and night. Measures included: 1) Demographics (e.g. age, diagnostic group); 2) Daytime and nighttime NIRI type (supplemental oxygen therapy via low flow nasal cannula or positive airway pressure (PAP) via high-flow nasal cannula, continuous positive airway pressure (CPAP), or biphasic positive airway pressure (BiPAP) at admission and discharge; and 3) Outcome (reduction, increase, or no change) in NIRI support for daytime and nighttime from admission to discharge. Results Thirty-eight infants and children (mean age=3.64 years; premature infants, n=20, other diagnoses, n=18) were included. For the total sample (n=38), daytime vs. nighttime NIRI type was significantly different (p<.001). At both admission and discharge, supplemental oxygen was the most common NIRI during the day, while PAP was most common at night. From admission to discharge, 7 (18%) children had a positive change (reduced NIRI) during the day, while 9 (24%) had a positive change at night. At discharge, 11/38 (29%) children required no daytime NIRI, while 4/38 (11%) required no day or night NIRI. Conclusion NIRI type differs between day and night at PACH admission and discharge. Reductions in NIRI were achieved during the day and at night from PACH admission to discharge for premature infants and for children with congenital or neurological diagnoses.

Background/objectives While outcomes for pediatric T-cell acute lymphoblastic leukemia (T-ALL) are favorable, there are few widely accepted prognostic factors, limiting the ability to risk stratify therapy. Design/methods Dana-Farber Cancer Institute (DFCI) Protocols 05-001 and 11-001 enrolled pediatric patients with newly diagnosed B- or T-ALL from 2005 to 2011 and from 2012 to 2015, respectively. Protocol therapy was nearly identical for patients with T-ALL (N = 123), who were all initially assigned to the high-risk arm. End-induction minimal residual disease (MRD) was assessed by reverse transcription polymerase chain reaction (RT-PCR) or next-generation sequencing (NGS), but was not used to modify postinduction therapy. Early T-cell precursor (ETP) status was determined by flow cytometry. Cases with sufficient diagnostic DNA were retrospectively evaluated by targeted NGS of known genetic drivers of T-ALL, including Notch, PI3K, and Ras pathway genes. Results The 5-year event-free survival (EFS) and overall survival (OS) for patients with T-ALL was 81% (95% CI, 73-87%) and 90% (95% CI, 83-94%), respectively. ETP phenotype was associated with failure to achieve complete remission, but not with inferior OS. Low end-induction MRD (<10(-4)) was associated with superior disease-free survival (DFS). Pathogenic mutations of the PI3K pathway were mutually exclusive of ETP phenotype and were associated with inferior 5-year DFS and OS. Conclusions Together, our findings demonstrate that ETP phenotype, end-induction MRD, and PI3K pathway mutation status are prognostically relevant in pediatric T-ALL and should be considered for risk classification in future trials. DFCI Protocols 05-001 and 11-001 are registered at as NCT00165087 and NCT01574274, respectively.

Long‐term mechanical ventilation (MV) is defined as the use of MV for more than 6 hours per day for at least 3 weeks. Children requiring long‐term MV include those with neuromuscular disease, central dysregulation, or lung dysfunction. Such children with medical complexity may be at risk for ventilator‐induced diaphragmatic dysfunction. Ventilator‐induced diaphragmatic dysfunction has been described in adult patients requiring acute MV with ultrasound (US). At this time, diaphragmatic US has not been evaluated in the pediatric post–acute care setting or incorporated into weaning strategies. We present 24 cases of children requiring long‐term MV who underwent diaphragmatic US examinations to evaluate for ventilator‐induced diaphragmatic dysfunction.

Key Points Childhood B-ALL patients, including those with VHR features, had favorable outcomes on DFCI 05-001 risk-stratified therapy. IKZF1 deletion was an independent predictor of inferior outcome, including among patients with low end-induction MRD.

BACKGROUND: Recovery from respiratory illness (RI), a common reason for hospitalization, can be protracted for some children because of high illness severity or underlying medical complexity.OBJECTIVE: We assessed which children hospitalized with RI are the most likely to use post-acute facility care (PAC) for recovery.METHODS: Retrospective analysis of 609,800 hospitalizations for patients in 43 US children's hospitals between 2010-2015 for RI, identified with the Agency for Healthcare Research and Quality Clinical Classification System. Discharge to PAC was identified using Centers for Medicare & Medicaid Services Discharge Status Codes. We compared patient characteristics by PAC use with generalized estimating equations.RESULTS: There were 2660 (0.4%) RI hospitalizations resulting in PAC transfer (n = 2660, 0.4%). Discharges to PAC had greater percentages of technology assistance (83.2% vs and mechanical ventilation (52.7% vs 9.1%), P < 0.001 for all. The highest likelihood of PAC use occurred with >= 11 vs no chronic conditions (odds ratio [OR] 11.7 [95% CI, 8.0-17.2]), >= 9 vs no therapeutic medication classes (OR 4.8 [95% CI, 1.8-13.0]), and existing tracheostomy (OR 3.0, 95% confidence interval [CI], 2.6-3.5). Median (interquartile range [IQR]) acute-care length of stay (LOS) for children most likely to use PAC was 19 (8-56) days; LOS remained long (median 13 [6-41] days) for children with the same attributes (n = 9448) not transferred to PAC.CONCLUSIONS: Children with RI who are most likely to use PAC have a high prevalence of multiple chronic conditions, multiple medications, and medical technology. Future investigations should assess the supply of PAC against the demand of hospitalized children with RI who might need it. (C) 2017 Society of Hospital Medicine

TPS10576 Background: CUDC-907 is an oral first-in-class small molecule inhibitor of histone deacetylases (HDACs) and phosphatidylinositol-3-kinases (PI3Ks), two enzyme classes commonly implicated in pediatric malignancies. Preclinical data demonstrate that inhibition of these enzymes decreases tumor growth across a range of histologies. Data from preclinical and clinical studies suggest that down-regulation of Myc or Mycn signaling may be important in the antineoplastic effects of CUDC-907. Myc or Mycn signaling appears to drive a number of pediatric cancers, heralds a poor prognosis in many of these diseases and has proven difficult to target. CUDC-907 has completed adult phase I testing in patients with hematologic malignancies. The drug was tolerable using a 5 days on/2 days off (5/2) dosing strategy, with a recommended phase II dose of 60 mg. Diarrhea, fatigue, nausea and thrombocytopenia were the most commonly reported side effects. Partial and complete responses were observed in patients with Myc-altered diffuse large B cell lymphoma. Methods: This study is a phase I, open-label, multicenter trial of CUDC-907 in patients 1-21 years of age with relapsed/refractory solid tumors, brain tumors and lymphomas (NCT02909777). The primary objectives are to determine the recommended phase II dose, describe toxicities, and describe pharmacokinetic parameters of CUDC-907 in this population. Other objectives include evaluation of disease response and exploration of the pharmacodynamic effects of CUDC-907. Patients receive CUDC-907 orally on a 5/2 schedule in 28-day cycles, with a pediatric mini-tab formulation available for younger children. Part A consists of a standard 3+3 design evaluating up to three dose levels. Following dose escalation, Part B consists of two expansion cohorts for patients with Mycn/Myc-driven neuroblastoma or mature B-cell lymphoma. Up to 44 patients may be enrolled across Parts A and B. Detailed pharmacokinetic testing is required in the first two cycles. Optional pharmacodynamic testing will quantify histone acetylation, Myc protein, and phospho-S6 in serial blood samples. Enrollment began in October 2016 and is ongoing. Clinical trial information: NCT02909777.

IMPORTANCE Acute care hospitals are challenged to provide efficient, high-quality care to children who have medically complex conditions and may require weeks or months for recovery. Although the use of home health care (HHC) and facility-based postacute care (PAC) after discharge is well documented for adults, to our knowledge, little is known for children. OBJECTIVE To assess the national prevalence of, characteristics of children discharged to, and variation in use across states of HHC and PAC for children. DESIGN, SETTING, AND PARTICIPANTS Retrospective analysis of 2,423,031 US acute care hospital discharges in 2012 for patients ages 0 to 21 years from the nationally representative Agency for Healthcare Research and Quality Kids' Inpatient Database. MAIN OUTCOMES AND MEASURES Discharges to HHC (eg, visiting or private-duty home nursing) and PAC (eg, rehabilitation facility) were identified from Centers for Medicare and Medicaid Services Discharge Status Codes. We compared children's characteristics (eg, race/ethnicity and number of chronic conditions) by discharge type using generalized linear regression. RESULTS The median age of participants was 3 years (interquartile range, 0-13 years), and 45.6% were female. Of 2,423,031 US acute care hospital discharges in 2012 for patients ages 0 to 21 years, 122,673 discharges (5.1%) were to HHC and 26,282 (1.1%) were to PAC facilities. Neonatal care was the most common reason (44.5%, n = 54,589) for acute care hospitalization with discharge to HHC. Nonneonatal respiratory, musculoskeletal, and trauma-related problems, collectively, were the most common reasons for discharge to PAC (42.9%, n = 11,275). When compared with PAC, more discharges to HHC had no chronic condition (34.4% vs 18.0%, P < .001) and fewer discharges to HHC had 4 or more chronic conditions (22.5% vs 37.7%, P < .001). In multivariable analysis, Hispanic children were less likely to use PAC (0.8% vs 1.1%; odds ratio [OR], 0.9 [95% CI, 0.8-0.9]) or HHC (3.3% vs 5.5%; OR, 0.8 [95% CI, 0.7-0.8]) compared with other children. Children with 4 or more chronic conditions compared with no chronic conditions had a higher likelihood of HHC use (11.0% vs 4.4%; OR, 2.9 [95% CI, 2.8-3.0]) and PAC (3.9% vs 0.8%; OR, 4.5 [95% CI, 4.3-4.9]). After case-mix adjustment, there was significant (P < .001) variation across states in HHC (range, 0.4%-24.5%) and PAC (range, 0.4%-4.9%) use. CONCLUSIONS AND RELEVANCE Home health care and PAC use after discharge for hospitalized children is infrequent, even for children with multiple chronic conditions. It varies significantly by race/ethnicity and across states. Further investigation is needed to assess reasons for this variation and to determine for which children HHC and PAC are most effective.

Background L-asparaginase is a universal component of treatment for childhood acute lymphoblastic leukaemia, and is usually administered intramuscularly. Pegylated Escherichia coli asparaginase (PEG-asparaginase) has a longer half-life and is potentially less immunogenic than the native Escherichia coli (E coli) preparation, and can be more feasibly administered intravenously. The aim of the Dana-Farber Cancer Institute Acute Lymphoblastic Leukaemia Consortium Protocol 05-001 (DFCI 05-001) was to compare the relative toxicity and efficacy of intravenous PEG-asparaginase and intramuscular native E coli l-asparaginase in children with newly diagnosed acute lymphoblastic leukaemia.Methods DFCI 05-001 enrolled patients aged 1-18 years with newly diagnosed acute lymphoblastic leukaemia from 11 consortium sites in the USA and Canada. Patients were assigned to an initial risk group on the basis of their baseline characteristics and then underwent 32 days of induction therapy. Those who achieved complete remission after induction therapy were assigned to a final risk group and were eligible to participate in a randomised comparison of intravenous PEG-asparaginase (15 doses of 2500 IU/m(2) every 2 weeks) or intramuscular native E coli l-asparaginase (30 doses of 25 000 IU/m(2) weekly), beginning at week 7 after study entry. Randomisation (1: 1) was unmasked, and was done by a statistician-generated allocation sequence using a permuted blocks algorithm (block size of 4), stratified by final risk group. The primary endpoint of the randomised comparison was the overall frequency of asparaginase-related toxicities (defined as allergy, pancreatitis, and thrombotic or bleeding complications). Predefined secondary endpoints were disease-free survival, serum asparaginase activity, and quality of life during therapy as assessed by PedsQL surveys. All analyses were done by intention to treat. This study is registered with ClinicalTrials. gov, number NCT00400946.Findings Between April 22, 2005, and Feb 12, 2010, 551 eligible patients were enrolled. 526 patients achieved complete remission after induction, of whom 463 were randomly assigned to receive intramuscular native E coli l-asparaginase (n=231) or intravenous PEG-asparaginase (n=232). The two treatment groups did not differ significantly in the overall frequency of asparaginase-related toxicities (65 [28%] of 232 patients in the intravenous PEG-asparaginase group vs 59 [26%] of 231 patients in the intramuscular native E coli l-asparaginase group, p=0.60), or in the individual frequency of allergy (p=0.36), pancreatitis (p=0.55), or thrombotic or bleeding complications (p=0.26). Median follow-up was 6.0 years (IQR 5.0-7.1). 5-year disease-free survival was 90% (95% CI 86-94) for patients assigned to intravenous PEG-asparaginase and 89% (85-93) for those assigned to intramuscular native E coli l-asparaginase (p=0.58). The median nadir serum asparaginase activity was significantly higher in patients who received intravenous PEG-asparaginase than in those who received intramuscular native E coli l-asparaginase. Significantly more anxiety was reported by both patients and parent-proxy in the intramuscular native E coli l-asparaginase group than in the intravenous PEG-asparaginase group. Scores for other domains were similar between the groups. The most common grade 3 or worse adverse events were bacterial or fungal infections (47 [20%] of 232 in the intravenous PEG-asparaginase group vs 51 [22%] of 231 patients in the intramuscular E coli l-asparaginase group) and asparaginase-related allergic reactions (14 [6%] vs 6 [3%]).Interpretation Intravenous PEG-asparaginase was not more toxic than, was similarly efficacious to, and was associated with decreased anxiety compared with intramuscular native E coli l-asparaginase, supporting its use as the front-line asparaginase preparation in children with newly diagnosed acute lymphoblastic leukaemia.

PURPOSE:We assessed the toxicity and efficacy of dexamethasone and a novel dosing method of Escherichia coli L-asparaginase (EC-Asnase) in children and adolescents with newly diagnosed acute lymphoblastic leukemia (ALL).PATIENTS AND METHODS:Patients achieving complete remission (CR) on Dana-Farber Cancer Institute ALL Consortium Protocol 00-01 were eligible for random assignment to 1) dexamethasone or prednisone, administered as 5-day pulses, every 3 weeks, and 2) weekly EC-Asnase, administered as a 25,000 IU/m(2) fixed dose (FD) or individualized dose (ID) starting at 12,500-IU/m(2), adjusted every 3 weeks based on nadir serum asparaginase activity (NSAA) determinations.RESULTS:Between 2000 and 2004, 492 evaluable patients (ages 1 to 18 years) enrolled; 473 patients (96%) achieved CR. Four hundred eight patients (86%) participated in the corticosteroid randomization and 384 patients (81%) in the EC-Asnase randomization. With 4.9 years of median follow-up, dexamethasone was associated with superior 5-year event-free survival (EFS; 90% v 81% for prednisone; P = .01) but higher rates of infection (P = .03) and, in older children, higher cumulative incidence of osteonecrosis (P = .02) and fracture (P = .06). ID EC-Asnase had superior 5-year EFS (90% v 82% for FD; P = .04), but did not reduce the frequency of asparaginase-related toxicity. Multivariable analysis identified both dexamethasone and ID EC-Asnase as independent predictors of favorable EFS.CONCLUSION:There was no overall difference in skeletal toxicity by corticosteroid type; dexamethasone was associated with more infections and, in older children, increased incidence of osteonecrosis and fracture. There was no difference in asparaginase-related toxicity by EC-Asnase dosing method. Dexamethasone and ID EC-Asnase were each associated with superior EFS. Monitoring NSAA during treatment with EC-Asnase may be an effective strategy to improve outcome in pediatric ALL.

Although L ‐asparaginase (ASP) is associated with several toxicities, its myelosuppressive effect has not been well characterized. On DFCI ALL Consortium Protocol 05‐01 for children with newly diagnosed acute lymphoblastic leukemia, the Consolidation phase and the initial portion of the Continuation phase were identical for standard risk patients, except ASP was given only during Consolidation. Comparing the two treatment phases revealed that low blood counts during Consolidation with ASP resulted in more dosage reductions of 6‐mercaptopurine and methotrexate. The myelosuppressive effect of ASP should be considered when designing treatment regimens to avoid excessive toxicity and dose reductions of other critical chemotherapy agents. Pediatr Blood Cancer 2012; 59: 925–927. © 2012 Wiley Periodicals, Inc.

BACKGROUND:Children with tracheotomy receive health care from an array of providers within various hospital and community health system sectors. Previous studies have highlighted substandard health information exchange between families and these sectors. The aim of this study was to investigate the perceptions and experiences of parents and providers with regard to health information management, care plan development and coordination for children with tracheotomy, and strategies to improve health information management for these children.METHODS:Individual and group interviews were performed with eight parents and fifteen healthcare (primary and specialty care, nursing, therapist, equipment) providers of children with tracheotomy. The primary tracheotomy-associated diagnoses for the children were neuromuscular impairment (n = 3), airway anomaly (n = 2) and chronic lung disease (n = 3). Two independent reviewers conducted deep reading and line-by-line coding of all transcribed interviews to discover themes associated with the objectives.RESULTS:Children with tracheotomy in this study had healthcare providers with poorly defined roles and responsibilities who did not actively communicate with one another. Providers were often unsure where to find documentation relating to a child's tracheotomy equipment settings and home nursing orders, and perceived that these situations contributed to medical errors and delayed equipment needs. Parents created a home record that was shared with multiple providers to track the care that their children received but many considered this a burden better suited to providers. Providers benefited from the parent records, but questioned their accuracy regarding critical tracheotomy care plan information such as ventilator settings. Parents and providers endorsed potential improvement in this environment such as a comprehensive internet-based health record that could be shared among parents and providers, and between various clinical sites.CONCLUSIONS:Participants described disorganized tracheotomy care and health information mismanagement that could help guide future investigations into the impact of improved health information systems for children with tracheotomy. Strategies with the potential to improve tracheotomy care delivery could include defined roles and responsibilities for tracheotomy providers, and improved organization and parent support for maintenance of home-based tracheotomy records with web-based software applications, personal health record platforms and health record data authentication techniques.

Abstract Abstract 3249 Background: L-asparaginase (ASP) is a universal component of treatment for childhood acute lymphoblastic leukemia (ALL). It is associated with multiple toxicities, including allergy, pancreatitis, and thrombosis. It is not thought to have a significant direct myelosuppressive effect, but its impact on the ability to tolerate other chemotherapeutic agents has not been well-characterized. Patients and Methods: Between 2005–2010, 61 patients (pts) were diagnosed with Standard Risk (SR) ALL at DFCI/Children's Hospital Boston and treated on DFCI ALL Consortium Protocol 05-01. SR pts met all of the following criteria: 1 to <10 years of age; highest pretreatment WBC < 50,000/mm3; absence of central nervous system leukemia (ie, CNS-1 or -2, only); absence of T-cell markers; absence of t(9;22), MLL gene rearrangements, and hypodiploidy; and low minimal residual disease (MRD) levels at the end of the first 4 weeks of treatment. Beginning at week 7, all SR pts began a 30-week (wk) Consolidation phase, consisting of every 21-day cycles of vincristine (day 1), dexamethasone (days 1–5), 6-mercaptopurine (6MP) (days 1–14), and methotrexate (MTX) (days 1, 8 and 15), and also including 30 consecutive wks of ASP (either weekly IM E.coli ASP 25000 IU/m2 or every 2-wk IV PEG ASP 2500 IU/m2). The Continuation phase followed Consolidation, and consisted of identical systemic and intrathecal (IT) chemotherapy, except without any ASP. Starting dose of 6MP was 50 mg/m2/day and of MTX was 30 mg/m2/dose. Doses were adjusted every 21 days to maintain absolute phagocyte (APC) nadirs > 500/mm3 and platelet nadirs > 75,000. Chemotherapy cycles were delayed until APC was ≥ 1000/mm3 or platelets were ≥ 100,000. Medical records of the 61 SR pts were reviewed to ascertain the dose modifications of MTX and 6MP made due to low or high blood counts (APC and/or platelets) and/or delays in starting cycles (defined as ≥25 day interval between cycles). 27 pts were randomized to receive IM E. coli ASP, 29 to IV PEG ASP, and 5 declined randomization (directly assigned to IM E.coli ASP). A total of 1780 chemotherapy cycles were evaluated longitudinally using generalized estimating equations, 498 during Consolidation (with ASP) and 1282 during Continuation (No ASP). Results: Median age was 3.9 years and 57% of pts were female. There was a higher proportion of cycles modified for low blood counts (ie, dose reduction of MTX and/or 6MP) during Consolidation (ASP given) compared to Continuation (No ASP) (24% vs 8%, Odds Ratio (OR) = 2.35 [95% CI 1.41, 3.94]; p < 0.01). There was a lower proportion of cycles modified due to high blood counts (ie, dose escalation of MTX and/or 6MP) during Consolidation (ASP given) compared to Continuation (No ASP) (20% vs 33%, OR =0.20 [95% CI 0.13, 0.30]; p < 0.01). Figures 1 and 2 summarize the dosing of MTX and 6-MP during Consolidation and Continuation. During Consolidation, the proportion of cycles in which pts received escalated doses of MTX (above the starting dose) was 21% compared to 62% during Continuation. Similarly, the proportion of cycles in which pts received escalated doses of 6-MP (above starting dose) was 10% in Consolidation compared to 59% in Continuation. 17% of Consolidation cycles were delayed because starting criteria were not met compared to 9% of Continuation cycles when pts were not receiving ASP (OR =2.08, 95% CI 1.18, 3.64; p=0.01). No difference in delays or dose modification for counts was detected between ASP randomization arms in either the Consolidation or Continuation phases. Conclusion: During Consolidation, when SR pts received ASP, blood counts were lower than during Continuation (no ASP), despite an otherwise identical schedule of other systemic and IT agents during these two phases. Because of low blood counts, pts received lower doses of MTX and 6MP when they were also receiving ASP, and delays in beginning chemotherapy cycles were more common. This suggests that ASP has a myelosuppressive effect, either direct (due to ASP) or secondary to ASP-induced changes in the levels or metabolism of other chemotherapeutic agents; further investigation is necessary to elucidate the precise mechanism. The myelosuppressive effect of ASP should be taken into consideration when designing regimens for pts with ALL which combine ASP with other agents to avoid excessive dose reductions and/or treatment delays. Disclosures: Sallan: Enzon Inc.: Honoraria, Research Funding. Silverman:EUSA Pharmaceuticals: Consultancy, Honoraria; Enzon Pharmaceuticals: Consultancy, Honoraria.

Objectives To identify children at risk for in-hospital mortality following tracheotomy.Design Retrospective cohort study.Setting 25 746 876 US hospitalisations for children within the Kids' Inpatient Database 1997, 2000, 2003 and 2006.Participants 18 806 hospitalisations of children ages 0-18 years undergoing tracheotomy, identified from ICD-9-CM tracheotomy procedure codes.Main outcome measure Mortality during the initial hospitalisation when tracheotomy was performed in relation to patient demographic and clinical characteristics (neuromuscular impairment (NI), chronic lung disease, upper airway anomaly, prematurity, congenital heart disease, upper airway infection and trauma) identified with ICD-9-CM codes.Results Between 1997 and 2006, mortality following tracheotomy ranged from 7.7% to 8.5%. In each year, higher mortality was observed in children undergoing tracheotomy who were aged <1 year compared with children aged 1-4 years (mortality range: 10.2-13.1% vs 1.1-4.2%); in children with congenital heart disease, compared with children without congenital heart disease (13.1-18.7% vs 6.2-7.1%) and in children with prematurity, compared with children who were not premature (13.0-19.4% vs 6.8-7.3%). Lower mortality was observed in children with an upper airway anomaly compared with children without an upper airway anomaly (1.5-5.1% vs 9.1-10.3%). In 2006, the highest mortality (40.0%) was observed in premature children with NI and congenital heart disease, who did not have an upper airway anomaly.Conclusions Congenital heart disease, prematurity, the absence of an upper airway anomaly and age <1 year were characteristics associated with higher mortality in children following tracheotomy. These findings may assist provider communication with children and families regarding early prognosis following tracheotomy.