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Medication Adherence in Pediatric Sickle Cell Disease and Hematopoietic Stem Cell Transplantation: An Integrative
Misty Evans1, Samereh Abdoli2, Micah Skeens3,4
1School of Nursing, Vanderbilt University, Nashville, TN, USA.
Insights
Medication adherence is crucial for children with sickle cell disease (SCD) undergoing hematopoietic stem cell transplantation (HSCT). This review found no studies on adherence in pediatric SCD patients post-HSCT, highlighting a significant research gap.
Area of Science:
- Pediatric Hematology
- Hematopoietic Stem Cell Transplantation
- Sickle Cell Disease Management
Background:
- Hematopoietic stem cell transplantation (HSCT) is an emerging curative option for pediatric sickle cell disease (SCD).
- Medication adherence is vital for managing SCD and post-HSCT care, yet remains a challenge.
- No existing synthesis evaluates adherence patterns specifically in pediatric SCD patients after HSCT.
Purpose of the Study:
- To identify and synthesize existing research on medication adherence in pediatric patients with SCD and/or undergoing HSCT.
- To highlight the research gap concerning adherence in pediatric SCD patients post-HSCT.
Main Methods:
- An integrative review was conducted using PubMed, CINAHL, and PsycINFO.
- Searched for studies measuring medication adherence in individuals ≤21 years with SCD and/or HSCT.
- Included studies reporting adherence rates; excluded intervention or drug development trials.
Main Results:
- Eighteen studies met inclusion criteria (12 on SCD, 6 on HSCT).
- No studies specifically addressed medication adherence in pediatric SCD patients post-HSCT.
- Reported adherence rates varied widely: 10%-89% for SCD and 40%-73% for HSCT.
- Nonadherence was linked to increased complications in both populations.
Conclusions:
- A significant research gap exists regarding medication adherence in pediatric SCD patients following HSCT.
- Findings underscore the need for targeted adherence interventions to improve outcomes.
- Addressing this gap is crucial for advancing nursing practice and optimizing care in pediatric hematology and HSCT.
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.
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