Implementation of near‐universal hydroxyurea uptake among children with sickle cell anemia: A single‐center experience. Issue 6 (19th March 2021)

• Record Type: Journal Article
• Authors: Karkoska, Kristine; Todd, Kevin; Niss, Omar; Clapp, Kelly; Fenchel, Lynette; Kalfa, Theodosia A.; Malik, Punam; Quinn, Charles T.; Ware, Russell E.; McGann, Patrick T.
• Is Part Of: Pediatric blood & cancer. Volume 68:Issue 6(2021)
• Page Start: n/a
• Page End: n/a
• Publication Date: 2021-03-19
• Subjects: hydroxyurea -- pediatrics -- prescriptions -- sickle cell anemia; Tumors in children -- Periodicals; Blood -- Diseases -- Periodicals; Cancer in children -- Periodicals; 618.92
• DOI: 10.1002/pbc.29008
• Languages: English
• ISSNs: 1545-5009

Abstract

Abstract: Background: Without early initiation of disease‐modifying therapy, the acute and chronic complications of sickle cell anemia (SCA) begin early in childhood and progress throughout life. Hydroxyurea is a safe and effective medication that reduces or prevents most SCA‐related complications. Despite recommendations to prescribe hydroxyurea for all children with SCA as young as 9 months, utilization remains low. Procedure: We completed a retrospective review of hydroxyurea‐prescribing practices and associated clinical outcomes at our institution over a 10‐year period before and after the 2014 National Heart, Lung, and Blood Institute (NHLBI) recommendations to use hydroxyurea for all children with SCA. Results: Hydroxyurea use more than doubled within our pediatric SCA population from 43% in 2010 to 95% in 2019. The age of hydroxyurea initiation was significantly younger during 2014–2019 compared to 2010–2013 (median 2 years vs. 6 years, p ≤ .001). With this change in clinical practice, nearly all (69/71 = 97%) children born after 2013 received disease‐modifying therapy by the end of 2019, primarily hydroxyurea (93%). Concurrently, the number of SCA‐related admissions significantly decreased from 67/100 patient‐years in 2010 to 39/100 patient‐years in 2019 ( p < .001). Conclusion: The early and universal prescription of hydroxyurea for children with SCA is the standard of care. Here, we demonstrate that a careful and deliberate commitment to follow this guideline in clinical practice is feasible and results in measurable improvements in clinical outcomes. Our approach and improved outcomes can serve as a model for other programs to expand their hydroxyurea use for more children with SCA.