Understanding VWD
Von Willebrand Disease (VWD) is a hereditary disorder caused by either missing or having a low amounts of the clotting protein von Willebrand factor (VWF). VWD is the most common bleeding disorder, affecting up to 1% of the US population (approximately 1 in every 100 people). VWD occurs equally across all races and ethnicities and genders, although women may show more symptoms due to heavy menstrual bleeding.
Symptoms of VWD
The main symptoms of VWD are frequent nosebleeds that last longer than 10 minutes, easy bruising with bruises that are raised and larger than a quarter, bleeding from injuries that last longer than 10 minutes, and heavy bleeding after procedures such as surgery or dental extractions. Females often experience heavy menstrual periods that last longer than average and heavy bleeding after childbirth or miscarriage.
There are three main types of VWD based on qualitative (function-related) or quantitative (amount-related) defects in VWF. A fourth type, acquired VWD, is not hereditary.
- Type 1 VWD is found in 60%-80% of patient
People with type 1 VWD have a quantitative deficiency of VWF. Levels of VWF in the blood range from 20%-50% of normal. The symptoms are usually mild. - Type 2 VWD is found in 15%-30% of patients
People with type 2 VWD have a qualitative deficiency in their VWF. Type 2 is broken down into four subtypes: type 2A, type 2B, type 2M and type 2N, depending on the presence and behavior of multimers, molecular chains of VWF. Symptoms are mild to moderate. - Type 3 VWD is found in 5%-10% of patients
People with type 3 VWD have very low levels or no VWF. 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.
Diagnosing VWD
Many patients with bleeding disorders are diagnosed and treated at one of the federally funded hemophilia treatment centers (HTCs) that are located 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.
A medical health history is important to help determine if other relatives have been diagnosed with a bleeding disorder or have experienced symptoms. A combination of blood tests are used for diagnosis, including a VWF antigen test, tests that measure clotting time, the ability to form a clot, and measure platelet function. Patients with VWD typically have < 50% of normal VWF in their plasma. After VWD is confirmed, a test to determine the exact type is performed.
It should be noted that diagnostic testing to confirm von Willebrand Disease (VWD) may have to be repeated because levels of VWF fluctuate. VWF can rise due to stress, exercise, the use of oral contraceptives, pregnancy and hyperthyroidism.
Treating VWD
Von Willebrand factor replacement therapy may be ordered. Factor VIII is rich in von Willebrand factor and is another option. Recombinant factor products are developed in a lab through the use of DNA technology instead of using human-derived pools of donor-sourced plasma. Factor products are infused intravenously through a vein in the arm or a port in the chest.
Aminocaproic acid prevents the breakdown of blood clots. It is often recommended before dental procedures, to treat nose and mouth bleeds, and for heavy or prolonged menstrual bleeding. It is taken orally, as a tablet or liquid. The Medical and Scientific Advisory Council (MASAC) of the National Hemophilia Foundation 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.
Desmopressin acetate (DDAVP®) is the synthetic version of vasopressin, a natural antidiuretic hormone that helps stop bleeding. In patients with types 1 and 2 VWD, it can be used for joint and muscle bleeds, for bleeding in the mucous membranes of the nose and mouth, and before and after surgery. It comes in an injectable form and a nasal spray.
Websites:
- Centers for Disease Control and Prevention
- National Bleeding Disorders Foundation
- National Heart, Lung and Blood Institute
- World Foundation of Hemophilia
- Hope for Hemophilia
Support Groups:
References:
Last reviewed 08/18/2026
