What is von Willebrand Disease (VWD)?

Von Willebrand disease (VWD) is a blood disorder in which people with VWD either have a low level of the protein called von Willebrand factor (VWF)  in their blood or the VWF protein doesn’t work the way it should. This leads to increased bleeding.  When a person has VWD, because the VWF doesn’t work the way it should,  a blood clot which normally forms to stop bleeding might take longer to form or does not form the way it should, and bleeding might take longer to stop. This can lead to heavy, hard-to-stop bleeding. Although rare, the bleeding can be severe enough to damage joints or internal organs, or even be life-threatening.

The disease is named after Erik Adolf von Willebrand, a Finnish doctor who traveled to a small island in Finnland and was the first to map a family where there were 23 out of 66 members who had the disease. many had died from bleeding.

How often does von Willebrand Disease happen?

VWD is the most common bleeding disorder. It is found in over 3 million people in the US or  up to 1% of the U.S. population (about 1 in every 100. Although VWD occurs among men and women equally, women are more likely to notice the symptoms because of heavy or abnormal bleeding during their menstrual periods and after childbirth.

VWD is a gentic disease which means that most people who have VWD are born with it. It almost always is inherited, or passed down, from a parent to a child. VWD can be passed down from either the mother or the father, or both, to the child. It is rare that a person is born with  VWD without a family history of the disease. This usually happens when a “spontaneous mutation” occurs, a change in a person's genes. In addition to being born with VWD, it is rare that a person who is not born with VWD can acquire it or have it first occur later in life when a person’s own immune system destroys his or her VWF, often as a result of use of a medication or as a result of another disease. If VWD was acquired later in life, VWD cannot be passed to the baby.

There are three types of VWD, type 1, type 2, and type 3. Type 1 is the least severe one and type 3 is the most severe one.

What are the major signs of von Willebrand Disease?

In general, VWB disease is associated with increased bleeding. You may have nosebleeds that won't stop, you may have an injury that won't stop bleeding, or surgery with excess bleeding like after dental surgery.

Women and von Willebrand Disease

Women with VWD often have longer and heavier mentrual periods (Menorrhagia) or they bleed more than usualy during childbirth or postpartum. You might want to track your periods by writing down the dates of your periods and how heavy you think your flow is (maybe by counting how many pads or tampons you use). Do this before you visit the doctor so that you can give the doctor as much information as possible.

Diagnosis of Von Willebrand Disease

The doctor will start by asking questions about personal and family histories of bleeding and also will check for unusual bruising or other signs of recent bleeding. The next step is to order some blood tests that will measure how the blood clots. A combination of blood tests is often needed to diagnose the disease and if the blood is clotting properly. These tests may show if there is a bleeding disorder, but more tests are needed to tell the type of bleeding disorder present. Initial screening tests are often normal in VWD, and more specific tests are required.

The tests will provide information about the amount of clotting proteins present in the blood and if the clotting proteins are working properly. Because certain medications can cause bleeding, even among people without a bleeding disorder, the doctor will ask about recent or routine medications taken that could cause bleeding or make bleeding symptoms worse.

Initial screening tests include:

• Complete Blood Count (CBC)
• Activated Partial Thromboplastin Time (APTT) Test
• Prothrombin Time (PT) Test
• Fibrinogen Test

More specific diagnostic tests are required to diagnose which bleeding disorder is there. Often these tests need to be repeated several times before an accurate diagnosis can be made. This is because the levels of clotting factors in the blood vary over time as a result of changes the body might be reacting to―such as stress, pregnancy, and infections―that can affect the test results. It is often difficult to make a specific diagnosis in and shortly after pregnancy because pregnancy changes often the blood tests.

The blood tests that a doctor can order to diagnose VWD (or another platelet disorder) include:

• Factor VIII clotting activity―To measure the amount of factor VIII in the blood
• Von Willebrand factor antigen―To measure the amount of VWF in the blood
• Ristocetin cofactor or other VWF activity―To measure how well the VWF works
• Von Willebrand factor multimers―To measure the makeup or structure of the VWF
• Platelet aggregation tests―To measure how well the platelets are working

Treatment of von Willebrand Disease

There is no one single treatment for Von Willebrand disease (VWD) because it is a complex disorder. Good quality medical care from doctors and nurses who know a lot about the disorder can help prevent some problems.

Often the best choice is a comprehensive hemophilia treatment center (HTC). Alternatively, a specialist in blood diseases called a hematologist could be consulted. Although it is called a hemophilia treatment center, HTCs provide care to address all issues related to VWD. The team consists of physicians (hematologists or blood specialists), nurses, social workers, physical therapists and other health care providers, who are specialized in the care of people with bleeding disorders, including VWD.

Find more resources on bleeding disorders here.