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INTRODUCTION The National Heart, Lung, and Blood Institute guidelines for sickle cell disease (SCD) pain crisis management recommend opioids within 60 minutes of emergency department (ED) registration and every 30 minutes thereafter until acute pain is managed. These guidelines are based on expert opinion without published, supporting data. OBJECTIVE To evaluate the association between timely ED opioid administration and hospitalization rates in children with SCD. METHODS Retrospective cohort of children presenting to a children's hospital ED with SCD pain between January 1, 2014, and April 30, 2018. Visits were extracted using ICD codes, chief complaints, and receipt of at least one opioid, and then reviewed to confirm the visit was an uncomplicated pain crisis. The primary outcome was hospitalization, yes or no. Generalized estimating equations were used to determine adjusted odds of hospitalization for the timely administration of initial and second doses of opioids. RESULTS Of the 902 eligible visits, 368 (40.8%) resulted in hospitalization. The mean (SD) age was 11.9 (± 5.2) years. The first opioid was administered within 60 minutes of arrival in 601 (66.6%) visits. The second opioid was administered within 30 minutes of the first in 84 (12.3%) visits. Receipt of the first opioid within 60 minutes of arrival was not associated with decreased hospitalization (1.30 [0.96-1.76]). However, receipt of the second dose within 30 minutes of the first was associated with decreased hospitalization (0.56 [0.33-0.94]). CONCLUSION This study suggests an association between children with SCD receiving a second dose within 30 minutes of the first opioid dose and decreased hospitalizations.

Objective Controversy exists regarding which febrile children with sickle cell disease (SCD) should receive a chest x-ray (CXR). Our goal is to provide data informing the decision of which febrile children with SCD presenting to the emergency department (ED) require a CXR to evaluate for acute chest syndrome (ACS). Methods Retrospective chart review of children ages 3 months to 21 years with SCD presenting to the ED at one of two academic children's hospitals with fever ≥38.5°C between January 1, 2010, and December 31, 2012. Demographic characteristics, respiratory symptoms, and laboratory results were abstracted. The primary outcome was the presence of ACS. Binary recursive partitioning was performed to determine predictive factors for a diagnosis of ACS. Results A total of 185 (10%) of 1,837 febrile ED visits met ACS criteria. The current National Heart, Lung, and Blood Institute (NHLBI) consensus criteria for obtaining a CXR (shortness of breath, tachypnea, cough, or rales) identified 158 (85%) of ACS cases, while avoiding 825 CXRs. Obtaining a CXR in children with NHLBI criteria or chest pain and in children without those symptoms but with a white blood cell (WBC) count ≥18.75 × 109/L or a history of ACS identified 181 (98%), while avoiding 430 CXRs. Conclusion Children with SCD presenting to the ED with fever and shortness of breath, tachypnea, cough, rales, or chest pain should receive a CXR due to high ACS rates. A higher WBC count or history of ACS in a child without one of those symptoms may suggest the need for a CXR. Prospective validation of these criteria is needed.

The impact of emergency department (ED) treatment on outcomes of sickle cell disease (SCD) acute pain hospitalizations is not well described. We investigated whether length of stay (LOS) and change in health-related quality of life (HRQL) are affected by initial opioid dose and time to administration. We conducted secondary analyses of data from the randomized-controlled Magnesium for children in Crisis (MAGiC) trial. The primary outcome was LOS. Secondary outcome was change in HRQL, assessed using PedsQL SCD Pain and Hurt and Pain Impact Domains measured in ED and at discharge. Independent variables were (1) time to first IV opioid, (2) total initial opioid dose (mg/kg/hr of morphine equivalents administered between ED and first study drug), and (3) Time to first oral opioid. Spearman correlations determined the associations with LOS. Using two-sample t-tests, we compared mean change in HRQL scores between IV opioid initiated within 60 and60 min, opioid doses in the highest and lowest tertiles, and oral opioid initiated within 24 and24 hr. Two hundred and four patients participated at 8 sites. Mean (SD) age was 13.6 (4.7) years. Earlier initiation of oral opioids was strongly correlated with shorter LOS (r = 0.61, P 0.01). Higher initial opioid dose was weakly correlated with longer LOS (r = 0.34, P 0.01). Higher initial opioid doses (6 vs -2.2; P = 0.01) and oral opioids initiated within 24 hr (5.7 vs -1.7, P = 0.04) were associated with larger mean change in HRQL at discharge. Prospective trials evaluating the impact of ED care on outcomes of pain hospitalizations could improve SCD pain treatment. Am. J. Hematol. 91:1175-1180, 2016. © 2016 Wiley Periodicals, Inc.

Enrollment of patients in sickle cell intervention trials has been challenging due to difficulty in obtaining consent from a legal guardian and lack of collaboration between emergency medicine and hematology. We utilized education and preconsent in a pediatric multisite sickle cell intervention trial to overcome these challenges. Overall, 48 patients were enrolled after being preconsented. Variable Institutional Review Board policies related to preconsent validity and its allowable duration decreased the advantages of preconsent at some sites. The utility of preconsent for future intervention trials largely depends on local Institutional Review Board policies. Preeducation may also benefit the consent process, regardless of site differences.

Background A previous study reported increased dissatisfaction with hospital care for children with sickle cell disease (SCD); however, its small size excluded determining whether race and chronic disease explained the difference. Procedure At hospital discharge, parents of children with SCD completed a survey assessing satisfaction with their child's hospital care. Results were compared to three years of satisfaction surveys for children with asthma or admitted to a general pediatrician's service collected as quality improvement for the hospital. The primary outcome was parent reported dissatisfaction with care. A chi-square was used to compare dissatisfaction between SCD and each comparison group. Results Parents of 639 children were included, 34 children with SCD, 124 with asthma, and 481 general pediatric patients. Parents of children with SCD were more often dissatisfied with their child's care compared to children with asthma (32.4% vs. 16.9%, P