Exploring the psychological distress and resilience of youth diagnosed with hemophilia: a scoping review

Abstract

Hemophilia is a bleeding disorder characterized by a deficiency in coagulation factors, typically diagnosed prenatally or in childhood when moderate or severe. This untreatable chronic disease poses social, emotional, and physical challenges, with resilience opportunities as youth transition towards greater autonomy developmentally. The Resilience in Illness Model, developed for youth with cancer, emphasizes coping approaches. No current work has been done to systematically synthesize the extant literature regarding resilience among youth with hemophilia. To explore and map peer-reviewed literature, a systematic scoping review was conducted to examine the relationship between psychological distress and resilience in hemophilic youths (ages 10 to 24). An academic librarian supported a search of quantitative and qualitative research from five databases (EMBASE, Ovid MEDLINE, CINAHL, ProQuest PsycINFO, and Web of Science) yielding 12,010 initial studies. After screening, 22 studies were selected for data extraction and final inclusion. Resilient coping (e.g., practicing spirituality, courageous optimism, problem-focused), participation in physical activity, and social support were found to consistently be associated with lower distress levels (depression, anxiety) and improved outcomes (e.g., quality of life, self-efficacy). Resilience interventions (promoting gym use, empowerment, CBT skills) showed promise for both mental health and pain outcomes. There was inconsistency in resilience conceptualizations, measures, and intervention approaches. Given the significance of resilience in the relationship between hemophilia and mental health, greater research is merited to address both physical pain, health, and distress simultaneously. There needs to be a focus on resilience-enabling environments, that includes social support, social norms, and spirituality, integrated within healthcare systems, as currently, an individual-based approach dominates. Recommendations include the need to consider systems support and an interactional view of resilience. Interventions may be aimed at caregivers, healthcare practitioners, transition services, and education, as well exploring digital mental health technologies. An integrated, resilience-oriented pediatric care pathway needs longitudinal study.