Abstract
Introduction: Sickle Cell Disease (SCD) is a chronic genetic disorder characterized by recurrent pain episodes and complex management needs. Poor medication adherence and limited self-management are frequent challenges. The increasing use of digital health technologies offers promising opportunities to enhance adherence, self-care, and patient engagement in SCD management. Methods: This systematic review was conducted in accordance with PRISMA guidelines and registered in PROSPERO (CRD42025639596). Comprehensive searches of PubMed, EMBASE, Scopus, Web of Science, Cochrane Library, Ovid, Clinical Key, and Google Scholar identified studies published from January 2000 to February 2025. Eligible studies included randomized controlled trials, quasi-experimental, cohort, and cross-sectional designs evaluating digital interventions aimed at improving medication adherence or disease management in SCD. Study quality was assessed using the Joanna Briggs Institute (JBI) Critical Appraisal Checklists. Results: Twenty-three studies involving 1,841 participants met the inclusion criteria. Interventions included mobile applications, SMS reminders, web-based platforms, and telehealth tools. Most reported improvements in medication adherence, disease knowledge, self-efficacy, and pain management. However, studies were generally small, short-term, and of moderate-to-high risk of bias, with user engagement declining over time. Evidence from low-resource settings was limited. Conclusion: Digital health interventions show encouraging potential to support medication adherence and self-management in SCD, particularly through mobile and cognitive behavioral therapy–based tools. Nevertheless, the evidence remains preliminary. Larger, theory-based, and longer-term trials are needed to confirm their effectiveness, sustainability, and accessibility in lower-resource contexts.