JOPHON NCPD (June/July 2026) Medication Adherence in Pediatric Sickle Cell Disease and Hematopoietic Stem Cell Transplantation: An Integrative Review of Literature-Medication Adherence in Pediatric Sickle Cell Disease and Hematopoietic Stem Cell Transplantation: An Integrative Review of Literature

Medication Adherence in Pediatric Sickle Cell Disease and Hematopoietic Stem Cell Transplantation: An Integrative Review of Literature

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This integrative review examined medication adherence among children and adolescents with sickle cell disease (SCD) and hematopoietic stem cell transplant (HSCT) recipients. Searches of PubMed, CINAHL, and PsycINFO identified 18 eligible studies published through May 2025: 12 on SCD and 6 on HSCT. No study specifically investigated adherence among patients with SCD after HSCT.<br /><br />Adherence varied widely. For SCD medications, including hydroxyurea and antibiotic prophylaxis, reported adherence ranged from 10% to 89%. After HSCT, adherence ranged from 40% to 73%. Rates differed according to how adherence was measured; self-reports generally suggested higher adherence than objective measures such as pharmacy refill records, electronic monitoring, or drug levels.<br /><br />Across both populations, nonadherence was linked to worse clinical outcomes. In SCD, lower hydroxyurea adherence was associated with more pain crises, acute chest syndrome, hospital and emergency visits, longer hospital stays, and poorer quality of life. Following HSCT, nonadherence was associated with increased infection risk and, in one study, graft-versus-host disease.<br /><br />The review highlights suboptimal adherence as a significant concern and identifies a critical evidence gap for children with SCD undergoing transplantation. Comparisons across studies are limited by inconsistent measurement methods and generally small samples. The authors recommend research to identify adherence barriers and facilitators and evaluate tailored strategies to support medication use. Improving adherence may help reduce complications and improve outcomes in pediatric hematology and transplant care.

Keywords

sickle cell disease

hematopoietic stem cell transplant

medication adherence

children and adolescents

hydroxyurea

antibiotic prophylaxis

nonadherence

clinical outcomes

pediatric hematology

systematic review