Estimating Bone Health Among Hemophilia Carriers and von Willebrand Disease Patients

Investigators address the need for understanding surrounding the relationship between poor bone health and patients who are hemophilia carriers and those with von Willebrand disease.

In the US, approximately 1.5 million individuals experience bone fractures related to osteoporosis each year. In addition to this being a major public health concern, it poses a significant financial burden.

Recently, issues with bone health have gained attention around how they relate to bleeding disorders. Individuals with hemophilia tend to have low bone mineral density that’s believed to be a consequence of prolonged immobility, recurrent hemarthrosis, decreased weight bearing, lower physical activity level and obesity.

While there’s some literature relating to bone health and hemophilia, there’s a lack of understanding around impaired bone health in individuals with von Willebrand disease (vWD) and those who are hemophilia carriers (HC). This motivated investigators to estimate the prevalence of osteoporosis, osteoarthritis and bone fractures in these individuals.

Assessing the Data

Divyaswathi Citla-Sridha, MD, University of Arkansas for Medical Sciences, and colleagues conducted a retrospective cohort study with a population level, commercial database that held electronic health record data from 26 major integrated healthcare systems representing more than 360 hospitals.

A total of 940 women who were diagnosed as hemophilia carriers and 19,580 patients with vWD were identified within the database. The primary outcome for the study was to identify the prevalence of osteoporosis, osteoarthritis, and fractures among individuals with von Willebrand disease and hemophilia carriers to compare them to controls from the database.

Investigators found 10 common risk factors related to poor bone health in the general population and patients with hemophilia–Vitamin D deficiency, obesity (BMI> / = 30), hypothyroidism, cigarette smoking, diabetes mellitus (DM), hypocalcaemia, malignancy, renal failure(RF), use of corticosteroids and use of nonsteroidal anti-inflammatory drugs (NSAIDs).

The Call for More Research

The results of the study indicate a significantly higher rate of osteoporosis, osteoarthritis and fractures among the target population. Investigators believe these data highlight the importance of screening patients for risk factors for poor bone health and then provide education to prevent these complications.

When looking at individuals who are hemophilia carriers, 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. For individuals with vWD, the prevalence of risk factors was significantly higher in cases when compared to controls.

“This study highlights the need for Haemophilia Treatment Centres to focus on the bone health in these patients and pay closer attention to other risk factors during their routine comprehensive visits,” investigators wrote. “Further prospective, multi centre studies are necessary to validate these findings."

The study “Bone health in haemophilia carriers and persons with von Willebrand disease: A large database analysis” was published in Haemophilia.