Bone health in haemophilia carriers and persons with von Willebrand disease: A large database analysis

Introduction: Persons with haemophilia(PWH) have been shown to have low bone mineral density likely the result of prolonged immobility, recurrent hemarthrosis, decreased weight bearing, lower physical activity level and obesity. Bone health has been poorly characterized in haemophilia carriers (HC) and persons with von Willebrand disease (PWvWD). Aim: To estimate the prevalence of osteoporosis, osteoarthritis and bone fractures in HC and PWvWD and identify risk factors for poor bone health. Methods: This is a retrospective study using a population level, commercial database - Explorys (IBM Watson Health, Cleveland, USA). We compared prevalence rates of osteoporosis, osteoarthritis and fractures among cases (HC or PWvWD) and controls (general population without an underlying bleeding disorder) from 1999 to 2020. Prevalence of common risk factors for poor bone health were compared among cases and controls. Results: Among 72,917,850 active persons in the database, we identified 940 women with the diagnosis of HC and 19,580 PWvWD. Among HC and PWvWD, prevalence of osteoporosis, osteoarthritis and fractures were significantly higher in cases, when compared to controls. In HC, the prevalence of vitamin D deficiency, obesity, hypothyroidism, smoking, diabetes mellitus, hypocalcaemia, corticosteroid use, malignancy, renal failure and Nonsteroidal anti-inflammatory drugs (NSAID) use were significantly higher among the cases. In PWvWD, the prevalence of risk factors was significantly higher in cases when compared to controls. Conclusion: The prevalence of osteoporosis, osteoarthritis and fractures is significantly higher among HC and PWvWD. This data highlights the importance of screening patients for risk factors for poor bone health and provide education to prevent these complications.