Mark Nimmer

The number of people with sickle-cell disease in the United States: national and state estimates.

Sickle-cell disease is not a reportable condition, making it difficult to ascertain the number of affected individuals. We estimated the number of people with sickle-cell disease for the United States and each individual state, adjusting for increased mortality. US Census population data for each of the 50 states plus the District of Columbia were obtained. The published prevalence of sickle-cell disease for blacks and Hispanics of either Mexican or non-Mexican ancestry was applied. Analysis revealed 89,079 (95% confidence interval: 88,494―89,664) people with sickle-cell disease in the United States, 80,151 black and 8928 Hispanic. The state with the highest sickle-cell population was New York with 8308, followed by Florida with 7539, and Texas with 6765 people with sickle-cell disease. This study provides important information for researchers and policymakers attempting to better plan for the care of the sickle-cell population.

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 Sβ(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.

Outpatient follow‐up and rehospitalizations for sickle cell disease patients

Rehospitalization rates are increasingly used as quality indicators for a variety of illnesses, including sickle cell disease. While one small, single center study suggested outpatient follow‐up with a pediatric hematologist was associated with fewer rehospitalizations, no study has examined the effect of post‐discharge outpatient follow‐up on rehospitalization rates across ages and beyond a single site.

Dissatisfaction with hospital care for children with sickle cell disease not due only to race and chronic disease

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.

Allergy Testing in Children With Low-Risk Penicillin Allergy Symptoms

Our utilization of a penicillin allergy questionnaire identified children at low risk for true allergy who were subsequently tested and whose results were found to be negative. BACKGROUND: Penicillin allergy is commonly reported in the pediatric emergency department (ED). True penicillin allergy is rare, yet the diagnosis results from the denial of first-line antibiotics. We hypothesize that all children presenting to the pediatric ED with symptoms deemed to be low-risk for immunoglobulin E-mediated hypersensitivity will return negative results for true penicillin allergy. METHODS: Parents of children aged 4 to 18 years old presenting to the pediatric ED with a history of parent-reported penicillin allergy completed an allergy questionnaire. A prespecified 100 children categorized as low-risk on the basis of reported symptoms completed penicillin allergy testing by using a standard 3-tier testing process. The percent of children with negative allergy testing results was calculated with a 95% confidence interval. RESULTS: Five hundred ninety-seven parents completed the questionnaire describing their child’s reported allergy symptoms. Three hundred two (51%) children had low-risk symptoms and were eligible for testing. Of those, 100 children were tested for penicillin allergy. The median (interquartile range) age at testing was 9 years (5–12). The median (interquartile range) age at allergy diagnosis was 1 year (9 months–3 years). Rash (97 [97%]) and itching (63 [63%]) were the most commonly reported allergy symptoms. Overall, 100 children (100%; 95% confidence interval 96.4%–100%) were found to have negative results for penicillin allergy and had their labeled penicillin allergy removed from their medical record. CONCLUSIONS: All children categorized as low-risk by our penicillin allergy questionnaire were found to have negative results for true penicillin allergy. The utilization of this questionnaire in the pediatric ED may facilitate increased use of first-line penicillin antibiotics.

Parent-Reported Penicillin Allergy Symptoms in the Pediatric Emergency Department

OBJECTIVE Children often present to the pediatric emergency department (ED) with a reported penicillin allergy. The true incidence of pediatric penicillin allergy is low, and patients may be inappropriately denied first-line antibiotics. We hypothesized that more than 70% of reported penicillin allergies in the pediatric ED are low risk for true allergy. METHODS Parents of children presenting to the pediatric ED with parent-reported penicillin allergy completed an allergy questionnaire. The questionnaire included age at allergy diagnosis, symptoms of allergy, and time to allergic reaction from first dose. The allergy symptoms were dichotomized into high and low risk in consultation with a pediatric allergist before questionnaire implementation. RESULTS A total of 605 parents were approached; 500 (82.6%) completed the survey. The median (interquartile range) age of the children at diagnosis was 1 year (7 months, 2 years); 75% were diagnosed before their third birthday. Overall, 380 (76%) (95% confidence interval 72.3, 79.7) children had exclusively low-risk symptoms. The most commonly reported symptoms were rash (466, 92.8%) and itching (203, 40.6%). Of the 120 children with one or more high-risk symptom, facial swelling (50, 10%) was the most common. Overall, 354 children (71%) were diagnosed after their first exposure to penicillin. Symptom onset within 24 hours of medication administration occurred in 274 children (54.8%). CONCLUSIONS Seventy-six percent of patients with parent-reported penicillin allergy have symptoms unlikely to be consistent with true allergy. Determination of true penicillin allergy in patients with low-risk symptoms may permit the increased use of first-line penicillin antibiotics.

Impact of emergency department care on outcomes of acute pain events in children with sickle cell disease

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 and >60 min, opioid doses in the highest and lowest tertiles, and oral opioid initiated within 24 and >24 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.

The accuracy of using ICD-9-CM codes to determine genotype and fever status of patients with sickle cell disease

To the Editor: ICD-9-CM codes are commonly used by health services researchers and administrators investigating quality metrics to identify patients, but no study has determined their accuracy for classifying patients with sickle cell disease (SCD) by genotype and fever status [1–4]. We conducted a retrospective chart review to compare ICD-9-CM codes to manual chart review for patients 3 months to 21 years old with SCD who presented to the Emergency Department (ED) at a Midwest urban children’s hospital between January 1, 2004 and December 31, 2012. Charts were reviewed from all 6,286 visits with a discharge diagnosis of SCD or SCD with crisis (i.e., 282.41, 282.42, 282.6X), whether the visit resulted in ED

The proportion of potentially preventable emergency department visits by patients with sickle cell disease.

Background: Emergency department (ED) visits by children with sickle cell disease (SCD) are often classified as urgent based on resource utilization. This classification may not accurately reflect the potentially preventable nature of SCD visits. We sought to determine the proportion of SCD crisis-related pediatric ED visits that are possibly preventable. Procedure: We reviewed 2 years of ED visits with a diagnosis of SCD with crisis at a hospital with an established sickle cell program. Criteria for preventable visits were predefined by pediatric hematologists. Non–pain-related chief complaints requiring emergent evaluation or painful episodes preceded by 2 opioid doses were considered not preventable; others were potentially preventable. Results: The study included 603 visits by 187 patients; 33% were potentially preventable. Overall, 29% of visits were emergent based on non–pain-related emergent complaints. Of the remaining pain-related visits, 26% were preceded by 2 or more doses of opioids at home. Visits by children with asthma were 0.58 times as likely to be preventable, due to more non–pain-related emergent chief complaints (32%) and more children (36%) taking 2 or more opioid doses. Conclusions: Approximately two thirds of SCD crisis-related pediatric ED visits are not immediately preventable; that percentage is higher in children with asthma.

The Benefits and Challenges of Preconsent in a Multisite, Pediatric Sickle Cell Intervention Trial

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.