Assessment Tools for Adherence to Prophylactic Haemophilia Treatment in Adult and Adolescent Patients: A Systematic Review

INTRODUCTION: Prophylactic treatment offers greater clinical benefits than on-demand therapy for patients with haemophilia, but these benefits depend on sustained adherence. Although adherence assessment is essential for understanding treatment effectiveness, a wide range of tools is used, and their characteristics, scope, and limitations have not been systematically synthesized. AIM: This systematic review aimed to identify and critically appraise assessment tools used to measure adherence to prophylactic haemophilia treatment in adult and adolescent patients. METHODS: The protocol followed PRISMA-P guidelines and was registered with PROSPERO (CRD42021273813). We conducted a systematic search across multiple databases including PubMed, Ovid EMBASE, Web of Science, and Scopus for studies published between 2000-2022 that reported on adherence to prophylaxis in adolescents and adults with haemophilia. A narrative synthesis was used to summarise key themes, variations, and the strengths and limitations of the studies. RESULTS: From the 22 studies, adherence was assessed using objective measures (prescription records), self-reported questionnaires, technology-assisted tools (electronic diaries and the Medtep Hemophilia online platform), and qualitative approaches. Self-reported questionnaires, particularly VERITAS-Pro and VERITAS-PRN, were the most frequently used adherence measures. Adherence assessment was frequently examined alongside medication supply and treatment practices, quality of life, bleeding episodes and haemarthrosis, joint health status, health beliefs and self-efficacy, environmental influences, and sociodemographic and clinical characteristics. CONCLUSION: VERITAS-Pro and VERITAS -PRN are central to the assessment of adherence to prophylactic haemophilia treatment. Their use alongside quality of life and illness perception measures supports clinical assessment and interpretation of adherence in both research and practice.