New Horizons in Emergency Department Management of Pediatric Sickle Cell Disease.

ABSTRACT: Sickle cell disease (SCD) is an important topic for emergency medicine audiences because complications of the disease account for a large proportion of hematologic emergencies that are seen in the emergency department each year. Early recognition and aggressive management of emergency complications of SCD can help to reduce the morbidity and mortality associated with this disease. Although the treatment recommendations for some complications of SCD are based on expert opinion, there has been advancement in the understanding of the pathogenesis of the disease and evidence regarding the treatment options available for managing acute complications. This continuing medical education article will provide a summary of the clinical manifestation and management of the most common acute complications of SCD: infection, vaso-occlusive episode, acute chest syndrome, splenic sequestration, stroke, and priapism.

Building a Physician-Scientist Pathway for Underrepresented Medical Students Through a Summer Research Experience.

PROBLEM: There is a need to increase the number of physician-scientists from underrepresented in medicine (URiM) groups. To engage URiM medical students, a committee of pediatric departmental leaders at the Children's Hospital of Philadelphia created the Summer Underrepresented in Medicine Medical Student Research program. This 8-week, onsite research and clinical experience takes place during the summer between students' first and second years of medical school. APPROACH: Applications were solicited between 2019-2023 through nationwide outreach to medical school deans and members of URiM student organizations. Accepted students were assigned a mentor to lead their research and clinical exposure. A curriculum highlighting aspects of academic medicine was developed. Students received a $3,000-$5,500 stipend for in-person participation. In 2020 and 2021, adjustments were made (e.g., virtual programming) to avoid interruptions during the COVID-19 pandemic. OUTCOMES: In the 2019-2023 application cycles, 298 students applied. Of 128 students who participated, 78 (61%) completed a postprogram survey. Students' survey feedback was positive. They indicated the program met expectations (mean rating = 1.3; scale: 1 = strongly agree to 5 = strongly disagree). Students reported they learned valuable information/skills (mean = 1.3) and that participation was worth time spent away from other responsibilities (mean = 1.3). The 2019 cohort (n = 12) achieved a 100% residency match rate. In addition, 4 (33%) of these students reported they are obtaining additional degrees or are performing research. After the program, many mentors continued to include students in their research projects. NEXT STEPS: Next steps include incorporating a standardized, scored rubric for selecting applicants; adding 3 lead mentors, an executive committee, and a faculty advisory board; establishing earlier pathway programming (e.g., at elementary and middle school levels); continuing to track/support alumni throughout their careers; and pursuing federal funding to expand the program.

Return visit rates after an emergency department discharge for children with sickle cell pain episodes.

BACKGROUND: High return visit rates after hospitalization for people with sickle cell disease (SCD) have been previously established. Due to a lack of multicenter emergency department (ED) return visit rate data, the return visit rate following ED discharge for pediatric SCD pain treatment is currently unknown. PROCEDURE: A seven-site retrospective cohort study of discharged ED visits for pain by children with SCD was conducted using the Pediatric Emergency Care Applied Research Network Registry. Visits between January 2017 and November 2021 were identified using previously validated criteria. The primary outcome was the 14-day return visit rate, with 3- and 7-day rates also calculated. Modified Poisson regression was used to analyze associations for age, sex, initial hospitalization rate, and a visit during the COVID-19 pandemic with return visit rates. RESULTS: Of 2548 eligible ED visits, approximately 52% were patients less than 12 years old, 50% were female, and over 95% were non-Hispanic Black. The overall 14-day return visit rate was 29.1% (95% confidence interval [CI]: 27.4%-30.9%; site range 22.7%-31.7%); the 7- and 3-day return visit rates were 23.0% (95% CI: 21.3%-24.6%) and 16.7% (95% CI: 15.3%-18.2%), respectively. Younger children had slightly lower 14-day return visit rates (27.3% vs. 31.1%); there were no associations for site hospitalization rate, sex, and a visit occurring during the pandemic with 14-day returns. CONCLUSION: Nearly 30% of ED discharged visits after SCD pain treatment had a return visit within 14 days. Increased efforts are needed to identify causes for high ED return visit rates and ensure optimal ED and post-ED care.

Bedside exclusion of pulmonary embolism in children without radiation (BEEPER): a national study of the Pediatric Emergency Care Applied Research Network-Study protocol.

Background: The Pulmonary Embolism Rule Out Criteria (PERC) Peds rule, derived from the PERC rule, was derived to estimate a low pretest probability for pulmonary embolism (PE) in children but has not been prospectively validated. Objective: The objective of this study was to present a protocol for an ongoing multicenter prospective observational study that evaluates the diagnostic accuracy of the PERC-Peds rule. Methods: This protocol is identified by the acronym, BEdside Exclusion of Pulmonary Embolism without Radiation in children. The study aims were designed to prospectively validate, or if necessary, refine, the accuracy of PERC-Peds and D-dimer in excluding PE among children with clinical suspicion or testing for PE. Multiple ancillary studies will examine clinical characteristics and epidemiology of the participants. Children aged 4 through 17 years were being enrolled at 21 sites through the Pediatric Emergency Care Applied Research Network (PECARN). Patients taking anticoagulant therapy are excluded. PERC-Peds criteria data, clinical gestalt, and demographic information are collected in real time. The criterion standard outcome is image-confirmed venous thromboembolism within 45 days, determined from independent expert adjudication. We assessed interrater reliability of the PERC-Peds, frequency of PERC-Peds use in routine clinical care, and descriptive characteristics of missed eligible and missed patients with PE. Results: Enrollment is currently 60% complete with an anticipated data lock in 2025. Conclusions: This prospective multicenter observational study will not only test whether a set of simple criteria can safely exclude PE without need for imaging but also provide a resource to fill a critical knowledge gap about clinical characteristics of children with suspected and diagnosed PE.

Faculty Perspectives on Diversity, Equity, and Inclusion: Building a Foundation for Pediatrics.

ABSTRACT: Pediatric departments and children's hospitals (hereafter pediatric academic settings) increasingly promote the tenets of diversity, equity, and inclusion (DEI) as guiding principles to shape the mission areas of clinical care, education, research, and advocacy. Integrating DEI across these domains has the potential to advance health equity and workforce diversity. Historically, initiatives toward DEI have been fragmented with efforts predominantly led by individual faculty or subgroups of faculty with little institutional investment or strategic guidance. In many instances, there is a lack of understanding or consensus regarding what constitutes DEI activities, who engages in DEI activities, how faculty feel about their engagement, and what is an appropriate level of support. Concerns also exist that DEI work falls disproportionately to racial and ethnic groups underrepresented in medicine, exacerbating what is termed the minority tax. Despite these concerns, current literature lacks quantitative data characterizing such efforts and their potential impact on the minority tax. As pediatric academic settings invest in DEI programs and leadership roles, there is imperative to develop and use tools that can survey faculty perspectives, assess efforts, and align DEI efforts between academic faculty and health systems. Our exploratory assessment among academic pediatric faculty demonstrates that much of the DEI work in pediatric academic settings is done by a small number of individuals, predominantly Black faculty, with limited institutional support or recognition. Future efforts should focus on expanding participation among all groups and increasing institutional engagement.

Diversity among research coordinators in a pediatric emergency medicine research collaborative network.

We conducted a survey study of clinical research coordinators (CRCs) at the member institutions of the Pediatric Emergency Care Applied Research Network, to determine the demographic and linguistic characteristics of CRCs around the network, and any perceived impact of those characteristics on their duties. A total of 53/74 CRCs completed the survey. Most respondents identified as "female," "white," and "not Hispanic/Latino." Most respondents felt that their race/ethnicity and their ability to speak a language other than English would positively impact recruitment. Four female respondents felt that their gender hindered their recruitment efforts and their sense of belonging within the research team.

A Qualitative Systematic Review of Pediatric Patient and Caregiver Perspectives on Pain Management for Vaso-Occlusive Episodes in the Emergency Department.

OBJECTIVES: The primary objective of this study is to describe the experiences of pediatric patients with sickle cell disease (SCD) and their caregivers who have presented to the emergency department (ED) for management of vaso-occlusive pain events. METHODS: We conducted a qualitative systematic review. The search protocol was developed to identify both published and unpublished literature that met inclusion/exclusion criteria. Included articles were primary hospital-based research with study populations that included (but were not limited to) pediatric patients aged 21 years or younger and qualitative or mixed-method analysis. RESULTS: Four themes were identified: (1) patients and caregivers perceive the ED as the last resort; (2) health care professionals in the ED lacked knowledge about SCD but rejected patients' and caregiver's attempts to share experience or advocate for their needs; (3) patients' accounts of pain are doubted because they do not always have "typical" signs of pain; and (4) caregivers identify racism as a reason for suboptimal care in the ED. CONCLUSIONS: There are multiple opportunities to improve management for vaso-occlusive pain events in the ED, including education of health care providers about SCD and complications, partnership between patients/caregivers and providers, and efforts to reduce the impact of systemic racism on health care delivery.

Intranasal fentanyl and discharge from the emergency department among children with sickle cell disease and vaso-occlusive pain: A multicenter pediatric emergency medicine perspective.

Children with sickle cell disease (SCD) commonly experience vaso-occlusive pain episodes (VOE) due to sickling of erythrocytes, which often requires care in the emergency department. Our objective was to assess the use and impact of intranasal fentanyl for the treatment of children with SCD-VOE on discharge from the emergency department in a multicenter study. We conducted a cross-sectional study at 20 academic pediatric emergency departments in the United States and Canada. We used logistic regression to test bivariable and multivariable associations between the outcome of discharge from the emergency department and candidate variables theoretically associated with discharge. The study included 400 patients; 215 (54%) were female. The median age was 14.6 (interquartile range 9.8, 17.6) years. Nineteen percent (n = 75) received intranasal fentanyl in the emergency department. Children who received intranasal fentanyl had nearly nine-fold greater adjusted odds of discharge from the emergency department compared to those who did not (adjusted odds ratio 8.99, 95% CI 2.81-30.56, p < .001). The rapid onset of action and ease of delivery without intravenous access offered by intranasal fentanyl make it a feasible initial parenteral analgesic in the treatment of children with SCD presenting with VOE in the acute-care setting. Further study is needed to determine potential causality of the association between intranasal fentanyl and discharge from the emergency department observed in this multicenter study.

Diagnosis and Management of Pediatric Venous Thromboembolism: New Therapies on the Horizon.

ABSTRACT: The incidence of venous thromboembolism (VTE) is increasing in pediatric patients. Prompt recognition and evaluation of VTE in young patients could prevent significant morbidity or mortality. In contrast to VTE in adults, current treatment guidelines are largely based on expert opinion as limited randomized controlled trial data exist about the appropriate management in pediatric patients with traditional anticoagulants. However, recently approved direct-acting oral anticoagulants in adults are also being investigated in pediatric VTE and these data could inform future evidence-based treatment principles. Thus, healthcare providers must be well informed about the management of pediatric VTE and the data from these trials to date. This continuing medical education article will provide a summary of management of pediatric VTE with particular emphasis on emerging direct-acting oral anticoagulants.

CTA utilization for evaluation of suspected pulmonary embolism in a tertiary pediatric emergency department.

OBJECTIVE: To evaluate changes in the utilization of computed tomography angiography (CTA) for evaluating suspected pulmonary embolism (PE) and the positive rate of ancillary for those studies negative for PE in the last 13 years. MATERIALS AND METHODS: A retrospective review of patient ≤ 20 years of age who underwent a chest CT angiography to rule out PE was performed in a 13-year-period. CT angiographies were grouped into three categories: Positive for PE, negative for PE and positive for ancillary findings, and negative for any pathology. From the exams with ancillary findings, we examined how many of these had a chest radiograph perform within 24 h prior to the CTA and how many of them had an impression stating the same conclusion as the CTA. RESULTS: 307 chest CT angiographies for suspected PE were included. 50 (16%) were reported as positive for PE and 91 (30%) were negative for PE but positive for ancillary findings. The most frequent ancillary findings were pneumonia (n = 26) and pleural effusion (n = 11). Out of 91, 73 patients had a previous chest radiograph and 28 of them reported a similar diagnosis than the CTA. The number of CT angiographies indicated for PE increased by 3.2 studies per year. The rate of CT angiographies positive for ancillary findings (slope = 1.5) and positive for PE (slope = 0.3) remained similar throughout the same period. CONCLUSIONS: CTA orders for PE have been increasing without any increased detection of PE or ancillary findings in children.

A Multiyear Cross-sectional Study of Guideline Adherence for the Timeliness of Opioid Administration in Children With Sickle Cell Pain Crisis.

STUDY OBJECTIVE: The National Heart, Lung, and Blood Institute evidence-based guidelines for timeliness of opioid administration for sickle cell disease (SCD) pain crises recommend an initial opioid within 1 hour of arrival, with subsequent dosing every 30 minutes until pain is controlled. No multisite studies have evaluated guideline adherence, to our knowledge. Our objective was to determine guideline adherence across a multicenter network. METHODS: We conducted a multiyear cross-sectional analysis of children with SCD who presented between January 1, 2016, and December 31, 2018, to 7 emergency departments (EDs) within the Pediatric Emergency Care Applied Research Network. Visits for uncomplicated pain crisis were included, defined with an International Classification of Diseases, Ninth Revision (ICD-9) and ICD-10 code for SCD crisis and receipt of an opioid, excluding visits with other SCD complications or temperature exceeding 38.5°C (101.3°F). Times were extracted from the electronic record. Guideline adherence was assessed across sites and calendar years. RESULTS: A total of 4,578 visits were included. The median time to first opioid receipt was 62 minutes (interquartile range 42 to 93 minutes); between the first and second opioid receipt, 60 minutes (interquartile range 39 to 93 minutes). Overall, 48% of visits (95% confidence interval 47% to 50%) were guideline adherent for first opioid. Of 3,538 visits with a second opioid, 15% (95% confidence interval 14% to 16%) were guideline adherent. Site variation in adherence existed for time to first opioid (range 22% to 70%) and time between first and second opioid (range 2% to 36%; both P<.001). There was no change in timeliness to first dose or time between doses across years (P>.05 for both). CONCLUSION: Guideline adherence for timeliness of SCD treatment is poor, with half of visits adherent for time to first opioid and one seventh adherent for second dose. Dissemination and implementation research/quality improvement efforts are critical to improve care across EDs.

Clinical variables that increase the probability of pulmonary embolism diagnosis in symptomatic children.

BACKGROUND: Pulmonary embolism (PE) in children carries a significant morbidity and mortality. We examined previously described factors in 2 cohorts of children tested for PE and identified novel factors. METHODS: We combined data from 2 retrospective cohorts. Patients up to age 21 years were included who underwent imaging or D-dimer testing for PE, with positive radiologic testing being the gold standard. Combined predictor variables were examined by univariate analysis and then forward stepwise multivariable logistic regression. RESULTS: The combined data set yielded 1103 patients with 42 unique predictor variables, and 93 PE-positive patients (8.4%), with a median age of 16 years. Univariate analysis retained 17 variables, and multivariable logistic regression found 9 significant variables with increased probability of PE diagnosis: age-adjusted tachycardia, tachypnea, hypoxia, unilateral limb swelling, trauma/surgery requiring hospitalization in previous 4 weeks, prior thromboembolism, cancer, anemia, and leukocytosis. CONCLUSION: This combined data set of children with suspected PE discovered factors that may contribute to a diagnosis of PE: hypoxia, unilateral limb swelling, trauma/surgery requiring hospitalization in previous 4 weeks, prior thromboembolism, and cancer, age-adjusted tachycardia, tachypnea, anemia, and leukocytosis. Prospective testing is needed to determine which criteria should be used to initiate diagnostic testing for PE in children.

Hydroxyurea Use for Sickle Cell Disease Among Medicaid-Enrolled Children.

BACKGROUND: Recent publications should have resulted in increased hydroxyurea usage in children with sickle cell disease (SCD). We hypothesized that hydroxyurea use in children with SCD increased over time and was associated with decreased acute care visits. METHODS: This was a secondary analysis of the Truven Health Analytics-IBM Watson Health MarketScan Medicaid database from 2009 to 2015. The multistate, population-based cohort included children 1 to 19 years old with an International Classification of Diseases, Ninth or 10th Revision diagnosis of SCD between 2009 and 2015. Changes in hydroxyurea were measured across study years. The primary outcome was the receipt of hydroxyurea, identified through filled prescription claims. Acute care visits (emergency department visits and hospitalizations) were extracted from billing data. RESULTS: A mean of 5138 children each year were included. Hydroxyurea use increased from 14.3% in 2009 to 28.2% in 2015 (P < .001). During the study period, the acute-care-visit rate decreased from 1.20 acute care visits per person-year in 2009 to 1.04 acute care visits per person-year in 2015 (P < .001); however, the drop in acute care visits was exclusively in the youngest and oldest age groups and was not seen when only children enrolled continuously from 2009 to 2015 were analyzed. CONCLUSIONS: There was a significant increase in hydroxyurea use in children with SCD between 2009 and 2015. However, in 2015, only ∼1 in 4 children with SCD received hydroxyurea at least once. Increases in hydroxyurea were not associated with consistently decreased acute care visits in this population-based study of children insured by Medicaid.

Normal saline bolus use in pediatric emergency departments is associated with poorer pain control in children with sickle cell anemia and vaso-occlusive pain.

Vaso-occlusive pain events (VOE) are the leading cause of emergency department (ED) visits in sickle cell anemia (SCA). This study assessed the variability in use of intravenous fluids (IVFs), and the association of normal saline bolus (NSB), on pain and other clinical outcomes in children with SCA, presenting to pediatric emergency departments (PED) with VOE. Four-hundred charts of children age 3-21 years with SCA/VOE receiving parenteral opioids at 20 high-volume PEDs were evaluated in a retrospective study. Data on type and amount of IVFs used were collected. Patients were divided into two groups: those who received NSB and those who did not. The association of NSB use on change in pain scores and admission rates was evaluated. Among 400 children studied, 261 (65%) received a NSB. Mean age was 13.8 ± 4.9 years; 46% were male; 92% had hemoglobin-SS. The IVFs (bolus and/or maintenance) were used in 84% of patients. Eight different types of IVFs were utilized and IVF volume administered varied widely. Mean triage pain scores were similar between groups, but improvement in pain scores from presentation-to-ED-disposition was smaller in the NSB group (2.2 vs 3.0, P = .03), while admission rates were higher (71% vs 59%, P = .01). Use of NSB remained associated with poorer final pain scores and worse change in pain scores in our multivariable model. In conclusion, wide variations in practice utilizing IVFs are common. NSB is given to >50% of children with SCA/VOE, but is associated with poorer pain control; a controlled prospective trial is needed to determine causality.

Practice Variation in Emergency Department Management of Children With Sickle Cell Disease Who Present With Fever.

OBJECTIVES: Urgent medical evaluation is recommended for patients with sickle cell disease (SCD) and fever. Clear recommendations exist regarding certain aspects of treatment, but other areas lack evidence. We determined practice variation for children with SCD presenting with fever to the emergency department (ED). METHODS: Retrospective chart review of children ages 3 months to 21 years with SCD presenting to the ED with fever greater than or equal to 38.5°C in the ED or preceding 24 hours. Visits from 3 sickle cell centers were included. Outcomes included blood culture, complete blood count, antibiotic treatment, chest x-ray, urinalysis, electrolytes, and hospital disposition. Differences greater than 10% were considered clinically meaningful. RESULTS: The population included 14,454 visits, of which 4143 (29%) were febrile and met all inclusion criteria. A complete blood count and blood culture were obtained at 94% of visits, and antibiotics were given at 91%, with no differences among sites. Meaningful differences existed for disposition, with 52%, 43%, and 99% of patients admitted to the inpatient units at hospitals A, B, and C, respectively. Differences were seen in obtaining a urinalysis (33%, 17%, and 21%), electrolytes (2%, 50%, and 12%), and chest x-rays for patients 2 years and older (78%, 77%, 64%) for hospitals A, B, and C, respectively. CONCLUSIONS: Significant variation exists in the proportion of children who receive a urinalysis, electrolytes, chest x-ray, and, most importantly, admission to the hospital. These examples of practice variation represent potential opportunities to define best care practices for children with SCD presenting to the ED for fever.