Presentation Details
Osteoporosis and Fragility Fractures in Hemophilia and von Willebrand disease – a large national claims database study

Divyaswathi Citla Sridhar1, Brandi Dupervil2, Laura Schieve2, Sanjay Ahuja3, Marilyn Manco-Johnson4, Roshni Kulkarni5, Suchitra Acharya6, Meera Chitlur7, Patricia Tobase8, Anjali Sharathkumar9, Binh Le2, Mike Soucie2.

1University of Arkansas for Medical Sciences/ Arkansas Children's hospital, Little Rock, AR, USA.2Centers for Disease Control and Prevention, Atlanta, GA, USA.3Case Western Reserve University, Cleveland, OH, USA.4Hemophilia & Thrombosis Center, University of Colorado Anschutz Medical Campus, Aurora, CO, USA.5Centers for Bleeding and Clotting Disorders, Department of Pediatrics, Michigan State University, Lansing, MI, USA.6Cohen Children's Medical Center, Northwell Hemostasis and Thrombosis Center, New Hyde Park, NY, USA.7Division of Hematology/Oncology, Children’s Hospital of Michigan, Detroit, MI, USA.8University of California San Francisco, San Francisco, CA, USA.9Stead Family Department of Pediatrics, Carver College of Medicine, University of Iowa Health Care, Iowa City, IA, USA

Abstract


Background: Osteoporosis is characterized by qualitative skeletal changes and reduction in bone mass resulting in bone fragility and higher risk of fractures. The risk of reduced bone mineral density in persons with hemophilia (PwH) has been described in several small single center and multi-center studies. Data on bone health outcomes in persons with von Willebrand disease [PwvWD] is very limited. Our study compares the prevalence of osteoporosis and bone fragility fractures in hemophilia and von Willebrand disease [vWD] with the general population in the US. Methods: This retrospective study analyzed data from 2 large datasets: the MarketScan Multi-State Medicaid Database [MD] and MarketScan Commercial and Medicare Supplemental Claims Database CD. Cases were individuals between 11-64 years of age in 2021 with at least 1 inpatient diagnosis code ICD-9 or ICD-10 or ≥ 2 non rule-out outpatient diagnosis codes > 30 days apart of hemophilia A or B, or vWD between 2015-2021, and who were continuously enrolled in a health plan for 12 months during 2021. Controls were individuals that met age and continuous enrollment criteria with no bleeding disorder diagnostic codes between 2015-2021. We compared prevalence of osteoporosis and fragility fractures, among cases and controls, overall and within age and sex subgroups. Additionally, prevalence of fragility fractures was assessed according to whether the patient also had another clinical risk factor for poor bone health MD dataset only. Individuals with disorders associated with risk of fragility fracture e.g., malignant neoplasm were excluded.
Results: Fragility fractures[Table 1]: Hemophilia: Prevalence of fractures among PwH was significantly higher than controls, 3.7% vs 1.9%, [p <0.05]  in CD and 8.1% vs 2.7%, [p <0.05] in MD. When categorized by age, prevalence of fractures was significantly higher among 31-45y and 46-64y in both CD and MD. Hypertension and vitamin D deficiency rates were significantly higher among PwH with fractures compared to controls with fractures in the MD.
vWD: Prevalence of fractures among PwvWD was significantly higher than controls 4.0% vs 1.9%, [p <0.05] in CD and 4.3% vs 2.7%, [p <0.05] in MD. When categorized by age, prevalence of fractures was significantly higher in all three age groups, in both databases.
Osteoporosis [Table 2]: Hemophilia: Prevalence of osteoporosis in PwH was significantly higher than controls 1.1% vs 0.3%, [p<0.05] in the MD. When categorized by age, prevalence of osteoporosis was significantly higher in 46-64y in the MD 8.4% vs 1.4%, [p<0.05]. vWD: Prevalence of osteoporosis in PwvWD was significantly higher than controls 1.1% vs 0.6%, [p <0.05] in CD and 0.6% vs 0.3%, [p <0.05] in MD. When categorized by age, prevalence of osteoporosis was significantly higher in 46-64y in the MD 4.7% vs 1.4%, [p<0.05] and 31-45y in the CD 4.1% vs 0.1%, [p<0.05]. Conclusions: The prevalence of fragility fractures and osteoporosis is significantly higher among PwH and PwvWD. These data highlight the importance of screening patients with these bleeding disorders for reduced bone mineral density, identifying additional risk factors for poor bone health, and providing education to prevent fractures.

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