One Small Step for Sickle Cell Disease: Many More to Go
Authors: Julie Kanter
Journal: Annals of Internal Medicine (2021)

EditorialsSeptember 2021One Small Step for Sickle Cell Disease: Many More to GoJulie Kanter, MDJulie Kanter, MDUniversity of Alabama at Birmingham Birmingham, AlabamaSearch for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/M21-2650 SectionsAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail Sickle cell disease (SCD), the most commonly inherited blood disorder in the United States, primarily affects a minority population and has been chronically underfunded. Despite its claim to fame as the “first molecular disease identified,” SCD has not been given sufficient attention, advocacy, or empathy (1). Until recently (2018), the only medication approved by the U.S. Food and Drug Administration for SCD was hydroxyurea, a chemotherapeutic that was accidentally noted to benefit persons with SCD through fetal hemoglobin induction, leukoreduction, and nitric oxide donation (2). Although useful in the reduction of acute painful events, hydroxyurea (and other new agents, including ...References1. Pauling L, Itano HA. Sickle cell anemia, a molecular disease. Science. 1949;110:543-8. [PMID: 15395398] CrossrefMedlineGoogle Scholar2. Charache S, Terrin ML, Moore RD, et al; Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia. Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. N Engl J Med. 1995;332:1317-22. [PMID: 7715639] CrossrefMedlineGoogle Scholar3. Salinas Cisneros G, Thein SL.. Recent advances in the treatment of sickle cell disease. Front Physiol. 2020;11:435. [PMID: 32508672] doi:10.3389/fphys.2020.00435 CrossrefMedlineGoogle Scholar4. Ballas SK, Gupta K, Adams-Graves P. Sickle cell pain: a critical reappraisal. Blood. 2012;120:3647-56. [PMID: 22923496] doi:10.1182/blood-2012-04-383430 CrossrefMedlineGoogle Scholar5. Yawn BP, Buchanan GR, Afenyi-Annan AN, et al. Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members. JAMA. 2014;312:1033-48. [PMID: 25203083] doi:10.1001/jama.2014.10517 CrossrefMedlineGoogle Scholar6. Lanzkron S, Little J, Wang H, et al. Treatment of acute pain in adults with sickle cell disease in an infusion center versus the emergency department. A multicenter prospective cohort study. Ann Intern Med. 2021;174:1207-13. doi:10.7326/M20-7171 Google Scholar7. Kanter J, Smith WR, Desai PC, et al. Building access to care in adult sickle cell disease: defining models of care, essential components, and economic aspects. Blood Adv. 2020;4:3804-3813. [PMID: 32785684] doi:10.1182/bloodadvances.2020001743 CrossrefMedlineGoogle Scholar8. Tsevat J, Moriates C. Value-based health care meets cost-effectiveness analysis. Ann Intern Med. 2018;169:329-332. [PMID: 30083766]. doi:10.7326/M18-0342 LinkGoogle Scholar Author, Article, and Disclosure InformationAffiliations: University of Alabama at Birmingham Birmingham, AlabamaDisclosures: The author has reported no disclosures of interest. The form can be viewed at www.acponline.org/authors/icmje/ConflictOfInterestForms.do?msNum=M21-2650.Corresponding Author: Julie Kanter, MD, University of Alabama at Birmingham, 1720 2nd Avenue South, Birmingham, AL 35294; e-mail, [email protected]edu.This article was published at Annals.org on 6 July 2021. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetailsSee AlsoTreatment of Acute Pain in Adults With Sickle Cell Disease in an Infusion Center Versus the Emergency Department Sophie Lanzkron , Jane Little , Hang Wang , Joshua J. Field , J. Ryan Shows , Carlton Haywood Jr. , Mustapha Saheed , Marc Proudford , Derek Robertson , Adrienne Kincaid , Lorri Burgess , Charles Green , Rebecca Seufert , Jasmine Brooks , Allie Piehet , Brandi Griffin , Nicole Arnold , Steven Frymark , Marcus Wallace , Nebras Abu Al Hamayel , Chiung-Yu Huang , Jodi B. Segal , and Ravi Varadhan Annals On Call - Vascular Occlusive Crisis: Site of Care Matters Robert M. Centor and Julie Kanter Metrics September 2021Volume 174, Issue 9Page: 1313-1314KeywordsClinical trial reportingEmergency departmentOutpatient clinicsPain managementSickle cell disease ePublished: 6 July 2021 Issue Published: September 2021 Copyright & PermissionsCopyright © 2021 by American College of Physicians. All Rights Reserved.PDF downloadLoading ...