Psychosocial Outcomes and Quality of Life in Patients with Hemophilia a Without Inhibitors: The HemoLIFE Study

Background: Hemophilia adversely affects several health domains and impairs the daily life of both patients and caregivers. Objectives: To assess the impact of hemophilia A without inhibitors on humanistic outcomes in adult and young patients and their caregivers in a real-life setting. Methods: This was a 12-month multicenter prospective observational study conducted in 18 Spanish hospitals. Patients who were diagnosed with hemophilia A (PWHs), without inhibitors, and who were 12 years of age or older, and their caregivers were included in the study. Results: A total of 85 PWHs (mean age: 33 years) and 12 caregivers participated in the study; 51 PWHs completed it, representing a 40% lost-to-follow-up rate. Twenty-five percent of PWHs showed maladjustment at study completion, with 'leisure time' and 'work/studies' being the most affected domains. Quality of life was particularly impaired in the sport, physical health and future areas. 'Lying/sitting/kneeling/standing' and 'leisure activities and sports' were the most impaired functions. Productivity was mainly affected by presenteeism in adult PWHs; 40 painful joint bleeding episodes were reported. Active strategies were mostly used for coping with chronic pain, and anxiety and/or depression were present in more than 10% of PWHs. Anxiety and depression were more frequently reported by caregivers. Conclusions: The study results suggest sustained impairments in adaptation to their disease, quality of life, and functionality in PWHs without inhibitors, especially related to leisure and sports activities, and a nontrivial proportion of them presented clinical levels of depression and anxiety. Overall impairment was more marked in adults than in children. In addition, due to the limited number of caregivers, their results must be considered exploratory.