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

Abstract

BackgroundWith the emergence of haploidentical transplantation, children with sickle cell disease (SCD) are increasingly pursuing hematopoietic stem cell transplantation (HSCT) as a curative treatment approach. However, suboptimal medication adherence remains a persistent challenge in both SCD and HSCT populations. Adherence to complex medication regimens is crucial to reducing the risk of severe complications and early mortality, yet no synthesis has evaluated patterns of adherence among patients with SCD post-HSCT.MethodWe conducted an integrative review, searching PubMed, CINAHL, and PsycINFO from April 2025 to May 2025 for studies measuring medication adherence in children (≤21 years) with SCD and/or HSCT. Studies were included if they reported adherence rates and excluded if they involved adherence interventions or drug development trials.ResultsEighteen studies met inclusion criteria: 12 addressed SCD and 6 focused on HSCT. No studies specifically examined adherence in patients with SCD post-HSCT. Adherence rates to disease-modifying medications in SCD ranged from 10% to 89%, while post-HSCT adherence ranged from 40% to 73%. Nonadherence was associated with increased complications across both populations.DiscussionThese findings highlight a critical research gap and provide a foundation for targeted adherence interventions. Addressing this gap is essential to advance nursing practice, improve adherence, and optimize clinical outcomes in pediatric hematology and HSCT care.