Supporting the Development of Adherence and Self-Management in Pediatric Severe Hemophilia

Objective: Hemophilia is a complex medical condition in which home-based self-management by children, adolescents, and their families is the standard of care. However, research on hemophilia self-management is limited. Because of its complexity and self-management standard, understanding the development of adherence and self-management in hemophilia may provide insights into supporting pediatric patients and their families in managing complex health care needs that can be generalized and adapted for other conditions. Methods: A topical review and retrospective case report are provided to illustrate issues in the development of adherence and self-management, and to highlight clinical applications, for pediatric patients with severe hemophilia. Results: Individual, social, and contextual factors influence adherence in pediatric severe hemophilia. Interventions that support individual autonomy and competence, such as medical play and education, and social-contextual supports, such as community-building and technological supports, have been shown to improve adherence and self-management in pediatric severe hemophilia, particularly when tailored to patients' individual needs. Conclusions: Though the literature on adherence and self-management in pediatric severe hemophilia is limited, evidence of positive effects of adherence and self-management interventions is promising and could be applied to management of other complex conditions. Future work should examine emerging adults, and roles of health literacy, executive functioning, and pediatric psychologists in adherence and self-management in pediatric severe hemophilia.