A Systematic Review of Medication Adherence Interventions in Pediatric Sickle Cell Disease

Objective: Adherence to medication regimens is of critical importance in sickle cell disease (SCD). Most notably, data indicate that hydroxyurea, penicillin, and iron chelators increase life expectancy and decrease comorbid medical problems (e.g., strokes). However, average pediatric SCD adherence rates are only 55-74%. Studies have introduced interventions for pediatric SCD adherence, but no review has synthesized these data.

Methods: We conducted a systematic review of interventions for enhancing medication adherence in pediatric SCD. There were 9 studies that met inclusion and exclusion criteria. The Pediatric Self-Management Model provided a framework for organizing the modifiable factors targeted by existing interventions.

Results: The 9 studies had high risk of bias levels and most targeted hydroxyurea. All studies used multiple measures of adherence, the interventions were multicomponent, and most included behavioral or technological interventions. There was variability in terms of whether the intervention targeted the individual, family, community, or healthcare system.

Conclusions: Consistent with the broader adherence literature, targeting knowledge alone was insufficient in increasing adherence. Findings suggest that reminders and targeting self-efficacy were key to success. In addition, addressing multiple domains in an intervention yielded larger effects on adherence. Although these results are promising, this review highlights several limitations of the extant literature, including a paucity of intervention studies and several methodological weaknesses, such as small sample sizes, few randomized controlled trials, and variable measures of adherence. Recommendations for advancing scientific understanding of adherence promoting interventions in pediatric SCD are provided.