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Types

Von Willebrand Disease

Von Willebrand disease (VWD) is an inheritable bleeding disorder. Many different proteins are needed to make a person’s blood clot successfully. People with VWD are either missing or low in the clotting protein von Willebrand factor (VWF) – or it doesn’t work as it’s supposed to. For a person to make a successful clot, VWF binds to factor VIII (8), another clotting protein, and platelets in blood vessel walls. This process will help 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. The condition is named after Finnish physician Erik von Willebrand, who first described it in the 1920s.

VWD is the most common bleeding disorder, affecting up to 1% of the US population – or approximately 1 in every 100 people. It occurs equally in men and women. However, women may be more symptomatic due to heavy menstrual bleeding (periods). VWD occurs equally across all races and ethnicities.

The guidelines for VWD were updated in 2021. Click here to read the guidelines in full.

What are the Symptoms of VWD?


The main symptoms of VWD are:

  • Frequent (more than 5 a year) 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
  • Being told you are “low in iron” or have been treated for anemia.
  • Heavy bleeding after any surgery including dental surgery
  • Having someone in your family who has one or more of these symptoms
  • Have someone in your family who has been diagnosed with a bleeding disorder such as von Willebrand disease or hemophilia.
  • 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 7 days
    • Heavy bleeding after childbirth or miscarriage

What are the Types of VWD?


There are three main types of VWD. A fourth type, acquired VWD, is not hereditary.

  • Type 1 VWD is found in 60%-80% of patients. People with type 1 VWD have a low levels of VWF in their blood. Levels of VWF in the blood range from 20%-50% of normal. The symptoms are usually mild. There is one subtype called Type 1C where the VFW has increase clearance leading to prolonged bleeding.
  • Type 2 VWD is found in 15%-30% of patients. People with type 2 VWD have a normal levels of VWF, but the factor doesn’t function as it should. Type 2 is broken down into four subtypes: type 2A, type 2B, type 2M and type 2N, depending on the specific way the VWF is defective. Symptoms are mild to moderate.
  • Type 3 VWD is found in 5%-10% of patients. People with type 3 VWD have a very low levels or no  VWF in their blood.. Some people with this type of VWD may also be low in factor VIII (factor eight). Symptoms are typically severe, and include spontaneous bleeding episodes, often into their joints and muscles.
  • Acquired VWD. This type of VWD in adults results after a diagnosis of an autoimmune disease, such as lupus, or from heart disease or some types of cancer. It can also occur after taking certain medications.

How is VWD Diagnosed?


A combination of blood tests are used to diagnose VWD, 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. Some of these tests may have to be repeated, because the levels of VWF can change due to stress, exercise, the use of birth control pills, pregnancy, and hyperthyroidism.

People with VWD usually have less than 50% of normal VWF in their blood. After a diagnosis of VWD is discovered, an additional is given to determine the type. The best place for patients with bleeding disorders to be diagnosed and treated is at one of the federally funded hemophilia treatment centers (HTCs) that are spread throughout the country. HTCs provide comprehensive care from skilled hematologists and other professional staff, including nurses, physical therapists, social workers and sometimes dentists, dieticians and other healthcare providers. In addition, HTCs often have specialized labs that can run more accurate VWD testing.

How is VWD Treated?


Treatment for VWD depends on the diagnosis and severity. Some bleeds are mild enough not to require treatment at all.
The most common treatment for VWD is DDAVP (desmopressin acetate), which stimulates the release of VWF from cells. This also increases the level of FVIII (factor 8). DDAVP comes in two forms:

Injectable DDVAP: This is injected into a vein or just under the skin, and is used to treat milder forms of VWD, usually type 1.

Nasal spray: A high-dose nasal spray is used to treat milder forms of VWD, usually type 1.
NOTE: A DDAVP nasal spray under the brand name Stimate was subject to a recall in 2020. To date, Stimate manufacturer Ferring Pharmecuticals has not resupplied the product. Learn more about efforts to produce and supply an alternative generic desmopressin-based nasal spray.

It is important to know that DDAVP is an antidiuretic, which means it causes the body to retain water. Because of this, it’s important to restrict fluids so you don’t develop a condition known as hyponatremia, reduced sodium in the bloodstream.

More severe forms of VWD are treated with factor replacement therapy, which are either plasma-derived (made from human blood products) or recombinant (synthetic). These clotting factor products are rich in VWF and factor VIII (factor 8). Clotting factor products are injected into a vein in the arm to replace the missing factor in the blood. They may also be used to treat mild VWD in people who do not respond to DDVAP.

Hormone therapy, such as birth control pills, or oral contraceptives, can be taken to reduce heavy menstrual bleeding. The hormones in birth control pills can increase the levels of VWF and factor VIII (factor 8) in the blood.

Aminocaproic acid and tranexamic acid prevent the breakdown of blood clots. These drugs are often recommended before dental procedures, to treat nose and mouth bleeds, and for heavy menstrual bleeding. Antifibrinolytics are taken by mouth, as a tablet or liquid. MASAC recommends that a dose of clotting factor be taken first to form a clot, then aminocaproic acid, to preserve the clot and keep it from being prematurely broken down.

Living with VWD


There's a lot to know about living with a bleeding disorder like hemophilia. Visit NBDF’s Steps for Living to explore resources, tools, tips and videos on living with hemophilia through all life stages. Organized by life stages, Steps for Living provides information on recognizing the signs of bleeds in children, help on navigating school issues, how to exercise safely, helping teens manage their bleeding disorder, information on workplace accommodations, and much more. There are downloadable checklists, toolkits, videos, and more.

222 S. Rainbow Blvd
Suite 203
Las Vegas, Nevada 89145

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