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.

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.

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.

A Comparative Analysis of Sensory Return in Delayed-Immediate Versus Immediate Neurotized Deep Inferior Epigastric Perforator Flap Breast Reconstruction.

BACKGROUND: Breast reconstruction patients who anticipate adjuvant radiation are not suitable candidates for immediate deep inferior epigastric perforator (DIEP) flap reconstruction due to the risk of flap fibrosis, shrinkage, and fat necrosis. Rather, many of these patients undergo delayed-immediate, or "babysitter," reconstruction, where a tissue expander is placed first as a temporizing measure during adjuvant therapy before definitive flap reconstruction. In this study, we aim to compare sensory changes in delayed-immediate to immediate DIEP flap patients. METHODS: Ninety-one patients, including 26 patients (46 breasts) with "babysitter" procedures and 65 patients (120 breasts) with immediate DIEP flaps, were prospectively identified at their preoperative visit. For both cohorts, baseline level (t = 0) is defined as before mastectomy. RESULTS: "Babysitter" patients underwent final-stage neurotized flap reconstruction on average at 12 months after initial tissue expander placement (range, 3-18 months). At 18 month after mastectomy (6 months after DIEP), delayed-immediate patients had comparable sensitivity measurements as immediate DIEP flap patients in all regions of the breast (P > 0.05). For delayed immediate patients, at 18 months postoperatively, sensitivity measurements were comparable with baseline levels only in the outer superior, outer medial, and outer lateral regions of the breast (P > 0.05). At 24 months postoperatively, cutaneous thresholds were comparable with baseline in all regions of the breast except the inner inferior region (P > 0.05), following a similar sensory recovery trajectory as immediate DIEP flap patients. CONCLUSIONS: In patients who undergo "babysitter" procedures, the combination of sensory return from the native mastectomy skin flap along with the neurotized DIEP flap yields sensory recovery comparable with immediate DIEP flap patients after definitive flap reconstruction. When final-stage flap reconstruction occurs by 12 months after mastectomy, sensation can return beginning 24 months postoperatively, or even sooner in some regions of the breast.

A Pilot Study Comparing Sensation in Buried Versus Nonburied Deep Inferior Epigastric Perforator Flaps.

BACKGROUND: Neurotized deep inferior epigastic perforator (DIEP) flaps have been shown to improve sensory recovery after mastectomy and reconstruction. With the recent trend toward nipple-sparing mastectomies, sensation likely originates within the buried DIEP flap and then innervates the breast skin. In contrast, for patients undergoing skin-sparing mastectomies, the DIEP flap skin is preserved, brought up to the surface, and directly innervated. In this study, we aim to evaluate inner breast region sensation between patients whose DIEP flap is buried and whose DIEP flap skin is brought to the surface. METHODS: Seventy patients who underwent mastectomy with immediate reconstruction using the DIEP flap were prospectively identified. Of these, 60 patients underwent nipple-sparing mastectomy with buried DIEP flap reconstruction while 10 patients underwent skin-sparing mastectomy with nonburied DIEP flap reconstruction. Patients in both cohorts received nerve grafting using the 70 × 1-2-mm Avance Nerve Graft in identical fashion. Sensitivity evaluation was performed in five inner breast regions (corresponding to the nonburied DIEP flap area). RESULTS: In the buried DIEP cohort, at 6 months postoperatively, there was a statistically significant difference in inner breast region sensitivity measurements compared with baseline levels ( P < 0.001). In contrast, in the nonburied DIEP cohort, at 6 months postoperatively, sensation in the inner breast region was comparable with preoperative baseline levels ( P = 0.236). At 24 months postoperatively, inner breast region sensitivity measurements in both cohorts were comparable with preoperative baseline measurements ( P > 0.05). CONCLUSIONS: Neurotized DIEP flap skin raised directly to the surface confers earlier sensory recovery than buried DIEP flaps. In patients who undergo skin-sparing mastectomies with nonburied DIEP flap reconstruction, they can expect significantly better sensation in the inner regions of the breast at 6 months postoperatively. In patients who undergo nipple-sparing mastectomies with buried DIEP flap reconstruction, they can expect sensation in the inner breast to return to preoperative baseline levels at a later time point-beginning as early as 24 months postoperatively.

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.

Comparing Autologous to Device-Based Breast Reconstruction: A Pilot Study of Return in Breast Sensation.

BACKGROUND: Poor breast sensation is common after mastectomy and reconstruction. In this study, we aim to define the temporal pattern of sensory changes after reconstruction and to compare the return in sensation between autologous and device-based reconstruction. METHODS: Women undergoing mastectomy with immediate reconstruction, with either tissue expander (TE) or neurotized deep inferior epigastric perforator (DIEP) flap, were prospectively identified at their preoperative or postoperative visit at defined time points. Neurosensory testing was performed in 9 breast regions using the AcroVal pressure-specified sensory device to determine 1 point-static cutaneous thresholds. Sensitivity data were averaged between patients at each time point and plotted over time. RESULTS: Eighty-seven patients (153 breasts) were included in this study, including 41 women (75 breasts) with DIEP flap and 46 women (78 breasts) with TE. The groups were comparable in age, body mass index, breast size, chemotherapy, radiotherapy, and number of revisional breast surgeries (P > 0.05). Compared with preoperative baseline, mean cutaneous thresholds in DIEP flap patients were comparable in most outer breast regions at 18 months postoperatively and in the entire breast, except the inner inferior region, at 3 years (P > 0.05). In contrast, mean cutaneous thresholds in TE patients continued to be significantly worse in the entire breast at 5 years postoperatively compared with baseline (P < 0.05). CONCLUSIONS: Autologous breast reconstruction is superior in sensory recovery. Patients undergoing DIEP flap can expect sensory return to preoperative levels by 3 years, with sensation returning by 18 months in some areas of the breast. Patients undergoing device reconstruction should expect a slower and more unpredictable return in breast sensation.

Prepectoral Breast Reconstruction Without the Use of Acellular Dermal Matrix: A 3-Year Review.

INTRODUCTION: Acellular dermal matrix (ADM) is frequently used during prepectoral tissue expander-based breast reconstruction. However, there has been a paucity of literature describing the experience of prepectoral reconstruction without the accompanying use of ADM. We seek to highlight our institutional experience with immediate prepectoral tissue expander placement without the use of ADM in breast reconstruction. METHODS: A retrospective, single-institution review of patient records was performed to identify all patients who underwent either skin sparing or nipple-sparing mastectomy with immediate tissue expander placement without the use of ADM. Demographics including age, body mass index, comorbidities, history of smoking or steroid use, perioperative radiation or chemotherapy, intraoperative details, and complication profiles during the tissue expander stage were retrospectively collected and analyzed. At the time of tissue expander placement, all mastectomy flaps were evaluated clinically and with indocyanine green laser angiography. Postoperative outcomes were tracked. RESULTS: Between 2017 and 2020, 63 patients (for a total of 108 breasts) underwent either skin sparing (16%) or nipple-sparing mastectomy (84%) with immediate prepectoral tissue expander without ADM placement. Fourteen percent of breasts developed postoperative cellulitis, 19% of breasts developed skin compromise, and 5% required a postoperative revisional procedure that did not result in immediate expander explant. There was a 13% (n = 14 breasts) explant rate occurring at a mean time of 74 days. Of those breasts that developed skin compromise, 45% went on to require eventual explant. Patients in the study were followed for an average of 6.3 months. CONCLUSIONS: Immediate prepectoral breast reconstruction using tissue expanders without ADM offers a viable alternative to established reconstructive paradigms. The major complication rate for prepectoral reconstruction without the use of ADM (17%) was found to be comparable with our historical subpectoral tissue expander reconstruction with ADM use. Tissue expander explant rates were also comparable between the prepectoral without ADM (13%) and the subpectoral with ADM cohorts. These preliminary data suggest that immediate breast reconstruction with tissue expander placement without accompanying ADM is viable alternative in the breast reconstructive algorithm.

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.

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.

Prepectoral Dual-Port Tissue Expander Placement: Can This Eliminate Suction Drain Use?

OBJECTIVES: The most common method of performing breast reconstruction after a mastectomy is using tissue expanders. Significant drainage that can lead to seromas and possible infection is a common sequela after mastectomies, and therefore, closed suction drains are routinely placed during the initial surgery (Vardanian et al. Plast Reconstr Surg. 2011;128:403-410). Drains, however, are associated with increased pain and discomfort for the patient and have been attributed to an increased infection rate by some authors (Degnim et al. Ann Surg. 2013;258:240-247; Saratzis et al. Clin Breast Cancer. 2009;9:243-246). We report on our experience using a dual-chamber tissue expander placed in the prepectoral space without acellular dermal matrix or other supportive material, which allows for drainage of periprosthetic fluid and avoids drain placement. PATIENTS AND METHODS: A retrospective, single-institution review of patients' records was performed for all patients who underwent prepectoral tissue expander placement between January 2018 and June 2019. Patients who had drains placed or who underwent autologous reconstruction in combination with expander placement were excluded. Thirty-nine patients were selected, with a total of 66 expander placements. Demographics including body mass index, comorbidities, history of smoking or steroid use, perioperative chemotherapy and radiation therapy, and intraoperative details and indications for surgery were retrospectively collected. Outcomes were separated into minor and major complications. Major complications were defined as complications that required surgical intervention. RESULTS: There were 51 prepectoral reconstructions with a dual-chamber tissue expander and no further surgical drain and 15 reconstructions using a standard expander with an additional closed suction drain. Overall complications for the no-drain cohort were 13.7% compared with 20% in the drain cohort (P = 0.68). Surgical site infection rate is 7.84% in the no-drain cohort compared with 13.3% in the drain cohort (P = 0.61). Mean numeric postoperative pain score at 6 hours was 3.2 in the no-drain cohort compared with 4.3 in the drain cohort (P = 0.03) and 4.17 compared with 5.6 at 12 hours, respectively (P = 0.04). Mean time to exchange of implant in the no-drain cohort was 152 days versus 126 days in the drain cohort (P = 0.38). Median follow-up times were 157 days for the no-drain cohort and 347 days for the drain cohort. CONCLUSIONS: Immediate breast reconstruction using a dual-chamber tissue expander offers a drain-free alternative to the immediate implant-based breast reconstruction. Our infection rate with 7.8% is lower than our own reported rates with subpectoral tissue expander reconstruction using either acellular dermal matrix or poly-4-hydroxybutyrate (17% and 11%). The overall complication rate is similar to historic data associated with breast reconstruction after mastectomy and suggests that dual-chamber expander placement offers a safe alternative possibly decreasing the patient's postoperative pain and discomfort that often is associated with closed suction drains (Saratzis et al. Clin Breast Cancer. 2009;9:243-246).

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.

A Standardized Clinical Pathway to Decrease Hospital Admissions Among Febrile Children With Sickle Cell Disease.

BACKGROUND AND OBJECTIVE: Recurrent hospital admissions for patients with sickle cell disease (SCD) are costly and contribute to a low quality of life for patients. We implemented a clinical pathway to safely discharge SCD patients with fever who are evaluated in the emergency department (ED) of a large tertiary care center. METHODS: An interdisciplinary team of ED and hematology physicians, nurses, and an improvement advisor developed a clinical pathway that identified febrile SCD patients at low risk of serious bacterial infection based on historical, clinical, and laboratory criteria who could be discharged from the ED. Phone follow-up was planned through the use of an automated electronic notification that was sent to an established hematology follow-up pool at the time of ED discharge. We conducted two "fake front end" trials in the ED to receive feedback on our process before full implementation. A postpathway implementation quality improvement team monitored discharge rates, phone follow-up rates and adverse events. RESULTS: In the first 9 weeks postpathway implementation, 100 SCD patients were evaluated for fever; 84 (24%) met low-risk criteria and were discharged home. This reduction in admission rate has been maintained throughout the 3 years postimplementation. Successful phone follow-up was achieved in all discharged patients within 24 hours and no adverse events were identified. CONCLUSIONS: Low-risk febrile patients with SCD can be safely discharged from the ED. An automated notification system within the electronic medical record system can facilitate patient follow-up after ED discharge. Future quality improvement efforts aimed to further reduce admissions in this population should target patients with modifiable risk factors for serious bacterial infection.

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.

A multicenter randomized controlled trial of intravenous magnesium for sickle cell pain crisis in children.

Magnesium, a vasodilator, anti-inflammatory, and pain reliever, could alter the pathophysiology of sickle cell pain crises. We hypothesized that intravenous magnesium would shorten length of stay, decrease opioid use, and improve health-related quality of life (HRQL) for pediatric patients hospitalized with sickle cell pain crises. The Magnesium for Children in Crisis (MAGiC) study was a randomized, double-blind, placebo-controlled trial of intravenous magnesium vs normal saline placebo conducted at 8 sites within the Pediatric Emergency Care Applied Research Network (PECARN). Children 4 to 21 years old with hemoglobin SS or Sß(0) thalassemia requiring hospitalization for pain were eligible. Children received 40 mg/kg of magnesium or placebo every 8 hours for up to 6 doses plus standard therapy. The primary outcome was length of stay in hours from the time of first study drug infusion, compared using a Van Elteren test. Secondary outcomes included opioid use and HRQL. Of 208 children enrolled, 204 received the study drug (101 magnesium, 103 placebo). Between-group demographics and prerandomization treatment were similar. The median interquartile range (IQR) length of stay was 56.0 (27.0-109.0) hours for magnesium vs 47.0 (24.0-99.0) hours for placebo (P = .24). Magnesium patients received 1.46 mg/kg morphine equivalents vs 1.28 mg/kg for placebo (P = .12). Changes in HRQL before discharge and 1 week after discharge were similar (P > .05 for all comparisons). The addition of intravenous magnesium did not shorten length of stay, reduce opioid use, or improve quality of life in children hospitalized for sickle cell pain crisis. This trial was registered at www.clinicaltrials.gov as #NCT01197417.

Determining the longitudinal validity and meaningful differences in HRQL of the PedsQL™ Sickle Cell Disease Module.

BACKGROUND: Detecting change in health status over time and ascertaining meaningful changes are critical elements when using health-related quality of life (HRQL) instruments to measure patient-centered outcomes. The PedsQL™ Sickle Cell Disease module, a disease specific HRQL instrument, has previously been shown to be valid and reliable. Our objectives were to determine the longitudinal validity of the PedsQL™ Sickle Cell Disease module and the change in HRQL that is meaningful to patients. METHODS: An ancillary study was conducted utilizing a multi-center prospective trial design. Children ages 4-21 years with sickle cell disease admitted to the hospital for an acute painful vaso-oclusive crisis were eligible. Children completed HRQL assessments at three time points (in the Emergency Department, one week post-discharge, and at return to baseline (One to three months post-discharge). The primary outcome was change in HRQL score. Both distribution (effect size, standard error of measurement (SEM)) and anchor (global change assessment) based methods were used to determine the longitudinal validity and meaningful change in HRQL. Changes in HRQL meaningful to patients were identified by anchoring the change scores to the patient's perception of global improvement in pain. RESULTS: Moderate effect sizes (0.20-0.80) were determined for all domains except the Communication I and Cognitive Fatigue domains. The value of 1 SEM varied from 3.8-14.6 across all domains. Over 50% of patients improved by at least 1 SEM in Total HRQL score. A HRQL change score of 7-10 in the pain domains represented minimal perceived improvement in HRQL and a HRQL change score of 18 or greater represented moderate to large improvement. CONCLUSIONS: The PedsQL™ Sickle Cell Disease Module is responsive to changes in HRQL in patients experiencing acute painful vaso-occlusive crises. The study data establish longitudinal validity and meaningful change parameters for the PedsQL™ Sickle Cell Disease Module. TRIAL REGISTRATION: ClinicalTrials.gov (study identifier: NCT01197417 ). Date of registration: 08/30/2010.

Implicit Bias in Pediatric Academic Medicine.

OBJECTIVE: Despite known benefits of diversity, certain racial/ethnic groups remain underrepresented in academic pediatrics. Little research exists regarding unconscious racial attitudes among pediatric faculty responsible for decisions on workforce recruitment and retention in academia. This study sought to describe levels of unconscious racial bias and perceived barriers to minority recruitment and retention among academic pediatric faculty leaders. METHODS: Authors measured unconscious racial bias in a sample of pediatric faculty attending diversity workshops conducted at local and national meetings in 2015. A paper version of the validated Implicit Association Test (IAT) measured unconscious racial bias. Subjects also reported perceptions about minority recruitment and retention. RESULTS: Of 68 eligible subjects approached, 58 (85%) consented and completed the survey with IAT. Of participants, 83% had leadership roles and 93% were involved in recruitment. Participants had slight pro-white/anti-black bias on the IAT (M = 0.28, SD = 0.49). There were similar IAT scores among participants in leadership roles (M = 0.33, SD = 0.47) and involved in recruitment (M = 0.28, SD = 0.43). Results did not differ when comparing participants in local workshops to the national workshop (n = 36, M = 0.29, SD = 0.40 and n = 22, M = 0.27, SD = 0.49 respectively; p = 0.88). Perceived barriers to minority recruitment and retention included lack of minority mentors, poor recruitment efforts, and lack of qualified candidates. CONCLUSIONS: Unconscious pro-white/anti-black racial bias was identified in this sample of academic pediatric faculty and leaders. Further research is needed to examine how unconscious bias impacts decisions in academic pediatric workforce recruitment. Addressing unconscious bias and perceived barriers to minority recruitment and retention represent opportunities to improve diversity efforts.

Medulloblastoma With Obstructive Hydrocephalus in a Young Infant.

Medulloblastoma is the most common posterior fossa tumor diagnosed in young infants. The presentation of posterior fossa tumors in neonates is highly variable. We report the case of a 2-month-old child who presented with poor feeding and lethargy and was noted to have a fixed downward gaze. Head computed tomography revealed a posterior fossa mass that was pathologically consistent with a medulloblastoma. This case demonstrates the uncommon presentation of posterior fossa tumors in young infants.