Rare Disease or Rarely Diagnosed?

New clinical guidelines aim to improve von Willebrand Disease patient outcomes.

Woman doctor talking with patient

A new international collaboration is working to develop important guidelines on how to diagnose and manage von Willebrand Disease (VWD), an inherited bleeding disorder that affects approximately 1 percent of the population (more than 75 million people worldwide).

VWD is the world’s most common bleeding disease and is caused by decreased production, absence or abnormal function of the clotting protein von Willebrand factor. For people with VWD, a cut, accident or surgery can result in bleeding that is hard to stop.

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Because VWD symptoms, like bruising from minor injuries, hard-to-stop nosebleeds and joint pain, are variable and show up in different ways at different times, many people are not diagnosed right away and may live for years with untreated bleeding. Research has shown that as many as 9-in-10 people with VWD have not been accurately diagnosed, according to the World Federation of Hemophilia.

“While VWD has no cure, it can be managed,” explains Jens Oltrogge, Head of Commercial Development Hematology, CSL Behring. “We have long known that with the right clinical guidance, physicians and hematologists around the world will be better prepared to diagnose VWD and enhance health outcomes for their patients. That’s why this effort is so important.” CSL Behring is a global leader in developing innovative therapies, including a wide range of recombinant and plasma-derived products for treating bleeding disorders.

Uniting in the effort to establish the clinical guidelines are the World Federation of Hemophilia (WFH), the International Society on Thrombosis and Haemostasis (ISTH), the National Hemophilia Foundation (NHF), the American Society of Hematology (ASH) and the University of Kansas Medical Center. As part of the collaboration, the organizations have identified hematologists, scientists and people with VWD to staff expert panels, which will help inform the recommendations.

The group intends to create evidence-based guidelines by 2020 to help physicians diagnose and manage patients with VWD and recommendations to improve care and outcomes for those patients.

People with VWD who would like to be notified of opportunities to provide input for the development of these guidelines can email vwd@wfh.org.