{"doi":"10.1002/ajh.26696","title":"Adherence to <scp>NHLBI</scp> guidelines for the emergent management of <scp>vaso‐occlusive</scp> episodes in children with sickle cell disease: A multicenter perspective","abstract":"To the Editor: Patients with sickle cell disease (SCD) commonly experience vaso-occlusive pain episodes (VOE), which accounts for 78% of all emergency department (ED) visits among patients with SCD.1 SCD-VOE pain is often under-, and inconsistently, treated in the ED.2 In 2014, the National Heart, Lung, and Blood Institute (NHLBI) put forth guidelines to direct the care of patients presenting with VOE.3 Key guideline recommendations include: (1) triage as high priority with rapid evaluation of patients presenting with VOE, (2) use of parenteral opioids for moderate-to-severe pain with administration ≤30 min after ED triage or ≤ 60 min after registration, (3) pain reassessment and subsequent parenteral opioid dosing every 15–30 min until pain is controlled, (4) the administration of non-steroidal anti-inflammatory drugs (NSAIDS) as an analgesic adjuvant (if no contraindications exist), and (5) in euvolemic patients with SCD-VOE who are unable to drink fluids, intravenous hydration at no more than maintenance rate to avoid over-hydration. Our objective was to assess adherence to all NHLBI recommendations and the impact of timely opioid administration on hospital admission in a study of 20 academic pediatric EDs in the United States and Canada. A cross-sectional study was performed at 20 academic, pediatric EDs across the United States and Canada (19 in the United States and one in Canada) (Supplemental Methods). International Classification of Disease Ninth and Tenth Revision code ED diagnoses of VOE were used to identify consecutive patients. To include the traditionally more severe genotypes of SCD, children with sickle cell anemia (hemoglobin-SS disease or hemoglobin Sβo-Thalassemia) aged 3–21 years who presented with VOE pain of any severity were included. Children who were diagnosed in the ED with acute chest syndrome or pregnancy were excluded. To determine adherence to the 2014 NHLBI recommendations, the date and time of: ED arrival, triage, patient rooming, initial pain assessment, intravenous (IV) catheter placement, first parenteral opioid administration (including intranasal fentanyl at some sites), pain reassessment, and second parenteral opioid administration were collected. Assigned Emergency Severity Index (ESI) triage score, type of parenteral opioid administered, IV fluid administration, and disposition from the ED were collected. ED arrival (i.e., the earliest time of ED arrival, registration, triage time, or time to room, whichever came first) was used for time to outcome measures. Descriptive statistics were calculated for adherence to each of the 2014 NHLBI recommendations for the management of acute VOE. Univariable logistic regression was used to assess factors associated with receipt of first parenteral opioid within ≤60 min and the relationship between time of opioid administration and hospital admission. Kruskal-Wallis and Wilcoxon rank-sum tests were used to test for associations between continuous variables and Chi-Square tests were used to test for associations between categorical variables. All statistics were conducted using SAS version 9.4. Over half of the 400 patients were female (n = 215, 54%) (median age 14.6 years [IQR 9.8, 17.6]). Most patients had hemoglobin-SS (92%, n = 367) and 8% (n = 33) had hemoglobin Sβo Thalassemia. MedED arrival times to evaluation and treatment were as follows: time to first pain assessment 8 min (IQR 3, 20), time to IV catheter placement 52 min (IQR 33, 81), time to first parenteral opioid administration 62 min (IQR 37, 98), and time from first to second parenteral opioid 67 min (IQR 46, 101). The proportion of children who received NHLBI recommended evaluation and treatments is in Table 1. Most patients were assigned the recommended ESI triage score of ≤2. Pain was assessed and documented in 99% (n = 395) of all charts. Most patients had pain reassessed (n = 363, 91%). Pain reassessment after the administration of the first parenteral opioid occurred at a median ","journal":"American Journal of Hematology","year":2022,"id":263434,"datarank":0.0,"base_score":0.0,"endowment":0.0,"self_citation_contribution":0.0,"citation_network_contribution":0.0,"self_endowment_contribution":0.0,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":13,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.9559,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2022-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":376325,"name":"David C. Brousseau","orcid":"0000-0001-7995-6504","position":1,"is_corresponding":false},{"id":920390,"name":"Fahd A. Ahmad","orcid":"0000-0001-5919-6835","position":2,"is_corresponding":false},{"id":447442,"name":"Jonathan E. Bennett","orcid":"0000-0002-8460-990X","position":3,"is_corresponding":false},{"id":730687,"name":"Seema Bhatt","orcid":null,"position":4,"is_corresponding":false},{"id":920391,"name":"Amanda Bogie","orcid":"0000-0003-0642-9310","position":5,"is_corresponding":false},{"id":302517,"name":"Kathleen Brown","orcid":"0000-0001-5768-2098","position":6,"is_corresponding":false},{"id":290456,"name":"T. Charles Casper","orcid":null,"position":7,"is_corresponding":false},{"id":921064,"name":"Laura L. Chapman","orcid":null,"position":8,"is_corresponding":false},{"id":920392,"name":"Corrie E. Chumpitazi","orcid":"0000-0001-5323-2743","position":9,"is_corresponding":false},{"id":298769,"name":"Daniel M. Cohen","orcid":"0000-0002-0352-7989","position":10,"is_corresponding":false},{"id":333557,"name":"Carlton Dampier","orcid":"0000-0002-7738-2620","position":11,"is_corresponding":false},{"id":377640,"name":"Angela M. Ellison","orcid":null,"position":12,"is_corresponding":false},{"id":735265,"name":"Hartmut Grasemann","orcid":"0000-0003-0331-9642","position":13,"is_corresponding":false},{"id":268708,"name":"Robert W. Hickey","orcid":"0000-0002-7708-9512","position":14,"is_corresponding":false},{"id":519039,"name":"Lewis L. Hsu","orcid":"0000-0002-3156-2378","position":15,"is_corresponding":false},{"id":333558,"name":"Peter A. Lane","orcid":"0000-0001-5887-6222","position":16,"is_corresponding":false},{"id":380284,"name":"Nitya Bakshi","orcid":"0000-0002-0876-9506","position":17,"is_corresponding":false},{"id":921065,"name":"Sara Leibovich","orcid":null,"position":18,"is_corresponding":false},{"id":920393,"name":"Prabhumallikarjun Patil","orcid":"0000-0002-2855-1555","position":19,"is_corresponding":false},{"id":778614,"name":"Elizabeth C. Powell","orcid":"0000-0003-3147-9889","position":20,"is_corresponding":false},{"id":672558,"name":"Rachel Richards","orcid":"0000-0002-4872-0037","position":21,"is_corresponding":false},{"id":921066,"name":"Syana Sarnaik","orcid":null,"position":22,"is_corresponding":false},{"id":81115,"name":"Debra L. Weiner","orcid":"0000-0001-8323-8134","position":23,"is_corresponding":false},{"id":333554,"name":"Claudia R. Morris","orcid":"0000-0001-6019-2858","position":24,"is_corresponding":false},{"id":921067,"name":"the SCD Arginine Study Group and PECARN","orcid":null,"position":25,"is_corresponding":false},{"id":451033,"name":"Chris A. Rees","orcid":"0000-0001-6449-0377","position":0,"is_corresponding":true}],"reference_count":8,"raw_metadata":null,"created_at":"2026-07-19T00:26:33.413850Z","pmid":"36054566","pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}