{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,12]],"date-time":"2026-10-12T13:45:26Z","timestamp":1791812726280,"version":"4.3.4"},"reference-count":11,"publisher":"The Pediatric Pharmacy Association","issue":"5","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,10]]},"abstract":"\n OBJECTIVE<\/jats:title>\n In the United States, approximately 100,000 people currently live with sickle cell disease. Despite the prominence of disease, there remains room for enhancing patient care. The use of hydroxyurea has improved patient outcomes, showing a decrease in vaso-occlusive crises, acute chest syndrome, the need for blood transfusions, and mortality. Other strides in management include antibiotic prophylaxis for younger patients, optimization of immunizations, and institution of novel agents.<\/jats:p>\n <\/jats:sec>\n \n METHODS<\/jats:title>\n The purpose of this study was to evaluate pediatric patients with sickle cell disease at a single institution by assessing adherence to the prescribing practices of evidence-based pharmacologic therapy. The primary outcome was evaluation of prescribing practices of hydroxyurea or erythrocytapheresis, antibiotic prophylaxis, and immunizations. Secondary outcomes include the prescribing of novel therapies, the evaluation of hydroxyurea dosing, and the comparison of vaccination rates with a previous study completed at the same institution. Data collected include active therapy with hydroxyurea or erythrocytapheresis, supportive care with antibiotic prophylaxis for patients younger than 5 years, and vaccination adherence per current guideline recommendations.<\/jats:p>\n <\/jats:sec>\n \n RESULTS<\/jats:title>\n A total of 104 patients were included. Of those reviewed, 79% were prescribed hydroxyurea, and 17% were receiving erythrocytapheresis. Supportive care results showed 100% of patients younger than 5 years were receiving appropriate antibiotic prophylaxis. On comparison with a previous institutional \u00adanalysis, those up-to-date on vaccinations were 66% vs 9.4% (p < 0.05).<\/jats:p>\n <\/jats:sec>\n \n CONCLUSIONS<\/jats:title>\n Results demonstrated high prescribing rates of therapy with either hydroxyurea or \u00aderythrocytapheresis and antibiotic prophylaxis but highlighted the continued opportunity to improve \u00advaccination rates.<\/jats:p>\n <\/jats:sec>","DOI":"10.5863\/jppt-25-00092","type":"journal-article","created":{"date-parts":[[2026,10,12]],"date-time":"2026-10-12T12:50:45Z","timestamp":1791809445000},"page":"622-627","source":"Crossref","is-referenced-by-count":0,"title":["Assessment of Evidence-Based Pharmacologic Therapy for Pediatric Patients With HbSS Disease at a Single Pediatric Institution"],"prefix":"10.5863","volume":"31","author":[{"given":"Rachel","family":"Gustafson","sequence":"first","affiliation":[{"name":"Norton Children\u2019s Hospital, Department of Pharmacy, Louisville, KY"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexis","family":"Hamelink","sequence":"additional","affiliation":[{"name":"Norton Children\u2019s Hospital, Department of Pharmacy, Louisville, KY"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joshua","family":"Elder","sequence":"additional","affiliation":[{"name":"Norton Children\u2019s Hospital, Department of Pharmacy, Louisville, KY"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"3883","reference":[{"issue":"7","key":"JPPT-25-00092-b01","series-title":"P T","first-page":"417","article-title":"Review of medication therapy for the prevention of sickle cell crisis","volume":"43","author":"Riley","year":"2018","unstructured":"Riley TR, Boss A, McClain D, et al. Review of medication therapy for the prevention of sickle cell crisis. P T. 2018;43(7):417\u2013421, 437."},{"issue":"9778","key":"JPPT-25-00092-b02","series-title":"Lancet","first-page":"1663","article-title":"A multicenter randomised controlled trial of hydroxyurea (hydroxycarbamide) in very young children with sickle cell anaemia","volume":"377","author":"Wang","year":"2011","unstructured":"Wang WC, Ware RE, Miller ST, et al. A multicenter randomised controlled trial of hydroxyurea (hydroxycarbamide) in very young children with sickle cell anaemia. Lancet. 2011;377(9778):1663\u20131672."},{"issue":"1","key":"JPPT-25-00092-b03","series-title":"Am J Hematol","first-page":"77","article-title":"Hydroxyurea effectiveness in children and adolescents with sickle cell anemia: a large retrospective, population-based cohort","volume":"92","author":"Quarmyne","year":"2017","unstructured":"Quarmyne MO, Dong W, Theodore R, et al. Hydroxyurea effectiveness in children and adolescents with sickle cell anemia: a large retrospective, population-based cohort. Am J Hematol. 2017;92(1):77\u201381."},{"key":"JPPT-25-00092-b04","series-title":"Evidence-Based Management of Sickle Cell Disease: Expert Panel Report 2014","first-page":"29","author":"Buchanan","year":"2014","unstructured":"Buchanan GR, Yawn BP, Afenyi AN, et al. Evidence-Based Management of Sickle Cell Disease: Expert Panel Report 2014. National Heart, Lung, and Blood Institute, US Department of Health and Human Services; 2014:29\u201330."},{"issue":"25","key":"JPPT-25-00092-b05","series-title":"N Engl J Med","first-page":"1593","article-title":"Prophylaxis with oral penicillin in children with sickle cell anemia: a randomized trial","volume":"314","author":"Gaston","year":"1986","unstructured":"Gaston MH, Verter JI, Woods G, et al. Prophylaxis with oral penicillin in children with sickle cell anemia: a randomized trial. 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J Pediatr Pharmacol Ther. 2020;225(1):39\u201346."},{"issue":"12","key":"JPPT-25-00092-b08","series-title":"Blood Adv","first-page":"2656","article-title":"American Society of Hematology 2020 guidelines for sickle cell disease: management of acute and chronic pain","volume":"4","author":"Brandow","year":"2020","unstructured":"Brandow AM, Carrol CP, Creary S, et al. American Society of Hematology 2020 guidelines for sickle cell disease: management of acute and chronic pain. Blood Adv. 2020;4(12):2656\u20132701."},{"issue":"2","key":"JPPT-25-00092-b09","series-title":"Blood Adv","first-page":"327","article-title":"American Society of Hematology 2020 guidelines for sickle cell disease: transfusion support","volume":"4","author":"Stella","year":"2020","unstructured":"Stella T, Chou MA, Ross MF, et al. American Society of Hematology 2020 guidelines for sickle cell disease: transfusion support. Blood Adv. 2020;4(2):327\u2013355."},{"key":"JPPT-25-00092-b10","article-title":"Evaluation and diagnosis of penicillin allergy for healthcare professionals: antibiotic use","year":"2017","unstructured":"Evaluation and diagnosis of penicillin allergy for healthcare professionals: antibiotic use. Centers for Disease Control and Prevention. October 31, 2017. Accessed April 20, 2024. https:\/\/www.cdc.gov\/antibiotic-use\/clinicians\/Penicillin-Allergy.html"},{"key":"JPPT-25-00092-b11","article-title":"NIH statement on new FDA-approved gene therapies for sickle cell disease","year":"2023","unstructured":"NIH statement on new FDA-approved gene therapies for sickle cell disease. National Heart Lung and Blood Institute, US Department of Health and Human Services. 2023. Accessed April 22, 2024. https:\/\/www.nhlbi.nih.gov\/news\/2023\/nih-statement-new-fda-approved-gene-therapies-sickle-cell-disease"}],"container-title":["The Journal of Pediatric Pharmacology and Therapeutics"],"original-title":[],"link":[{"URL":"https:\/\/jppt.kglmeridian.com\/view\/journals\/jppt\/31\/5\/article-p622.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/jppt.kglmeridian.com\/downloadpdf\/journals\/jppt\/31\/5\/article-p622.xml","content-type":"text\/html","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/jppt.kglmeridian.com\/downloadpdf\/journals\/jppt\/31\/5\/article-p622.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,10,12]],"date-time":"2026-10-12T12:50:45Z","timestamp":1791809445000},"score":1,"resource":{"primary":{"URL":"https:\/\/jppt.kglmeridian.com\/view\/journals\/jppt\/31\/5\/article-p622.xml"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,10]]},"references-count":11,"journal-issue":{"issue":"5"},"URL":"https:\/\/doi.org\/10.5863\/jppt-25-00092","relation":{},"ISSN":["2331-348X","1551-6776"],"issn-type":[{"value":"2331-348X","type":"electronic"},{"value":"1551-6776","type":"print"}],"subject":[],"published":{"date-parts":[[2026,10]]}}}