Of the types of bleeding disorders, hemophilia — not von Willebrand disease (VWD) — is probably what first comes to mind. And yet, VWD is actually the most common bleeding disorder. VWD is found in up to 1% of the U.S. population; in other words, 3.2 million (or about 1 in every 100) people in the United States have the disease.
With this in mind, it’s important to address key questions about VWD, understand how it differs from hemophilia, and recognize its symptoms and treatments. Read on for a breakdown of the bleeding disorder.
What Is von Willebrand Disease?
Named after Finnish physician Erik von Willebrand, VWD is an inheritable bleeding disorder that, like hemophilia, is a result of missing or low levels of certain factor proteins that are needed to make a person’s blood clot properly. People with VWD are either missing or low in the clotting protein known as von Willebrand factor (VWF) — or the VWF doesn’t work as it should.
VWF is supposed to bind to factor VIII, another clotting protein, and platelets in blood vessel walls. This process helps form a platelet plug during the clotting process. People with VWD are not able to form this platelet plug, or it will take longer to form.
There are three main types of VWD:
- Type 1: The most common and mild of the three, affecting 60% to 80% of people with VWD. Type 1 results in low levels of VWF and either mild or no symptoms.
- Type 2: Effects 15% to 30% of people with VWD. In this case, levels of the VWF protein are normal, but it doesn’t operate as it should. This could happen for a few different reasons, such as VWF not being the right size, VWF attaching to platelets at the wrong time, or VWF attaching to platelets but not factor VIII.
- Type 3: Found in 5% to 10% of people with VWD, the third type is the most severe. People with type 3 have little or no VWF and usually suffer from severe bleeds.
There is a fourth type, known as acquired VWD, that isn’t hereditary. This type found in adults comes after a diagnosis of an autoimmune disease — such as lupus — or from heart disease, some types of cancer, or certain medications.
What Are the Differences Between VWD and Hemophilia?
Hemophilia and VWD are both passed on genetically from parent to child, though VWD is much more common and usually milder than hemophilia. Another difference: Men and women have an equal chance of getting VWD, whereas hemophilia is much rarer in women than in men.
And compared with hemophilia, people with VWD are less likely to bleed into their joints. VWD most often causes easy bruising and nosebleeds, and women with VWD can experience very heavy menstrual periods.
What Are the Symptoms of von Willebrand Disease?
Von Willebrand disease shares many of the same symptoms as hemophilia. Symptoms of VWD include:
- Frequent nosebleeds that last longer than 10 minutes
- Bleeding from cuts or injuries that lasts longer than 10 minutes
- Bruising easily, with bruises that are raised and larger than a quarter
- Heavy bleeding after any surgery
- For women, girls, and those who menstruate: Heavy periods, also called heavy menstrual bleeding, (having to change one pad or tampon every hour) or periods that last longer than seven days; and heavy bleeding after childbirth or miscarriage
VWD is diagnosed through a combination of blood tests including a VWF antigen test — which measures the amount of VWF in the blood — tests that measure clotting time and ability to form a clot, and tests measuring platelet function.
If VWD is discovered, an additional test is given to determine the type. The recommended facilities for diagnosis and treatment are any federally funded hemophilia treatment centers.
What Are the Treatments for VWD?
VWD is treated in several ways depending on the diagnosis and severity, and some bleeds are mild enough not to require treatment at all. The most common treatment is desmopressin acetate (DDAVP), a drug that stimulates the release of VWF from cells and increases the level of factor VIII, but was recently recalled from one manufacturer. As with hemophilia, more severe forms of VWD are treated with factor replacement therapy.
Birth control pills can be taken to reduce menstrual bleeding and increase the levels of VWF and factor VIII in the blood. Antifibrinolytics, medications that keep blood clots from breaking down, are another treatment option.