Definition
Von Willebrand disease is the most common hereditary bleeding disorder.
Symptoms
• Abnormal menstrual bleeding
• Bleeding of the gums
• Bruising
• Nose bleeds
• Skin rash
Causes & Risks
Von Willebrand disease is caused by a deficiency of von Willebrand factor. Von Willebrand factor helps blood platelets clump together and stick to the blood vessel wall, which is necessary for normal blood clotting.

Von Willebrand disease affects both men and women. Most cases are mild. Bleeding may occur after surgery or when you have a tooth pulled. Aspirin and other nonsteroidal anti-inflammatory drugs (NSAIDs) can make this condition worse. Bleeding may decrease during pregnancy.

A family history of a bleeding disorder is the primary risk factor. In women with heavy or prolonged menstrual bleeding, von Willebrand is more common in Caucasian women than in African American women. The majority of women with heavy or prolonged menstrual bleeding do NOT have Von Willebrand disease.
Tests & Diagnostics
Tests that may be done to diagnose this disease include:
• Bleeding time (is prolonged)
• Platelet aggregation test
• Platelet count (may be normal)
• Ristocetin cofactor test (the primary assay test used to diagnose von Willebrand disease)
• Von Willebrand factor level (level is reduced)
This disease may also alter the results of the following tests:
• Factor VIII level
• Von Willebrand factor multimers (parts of the von Willebrand factor protein molecule)
Von Willebrand disease may be hard to diagnose. Low von Willebrand factor levels and bleeding do not always mean you have von Willebrand disease.

Treatments
Medications such as desamino-8-arginine vasopressin (DDAVP) can be given to raise the levels of von Willebrand factor, which will reduce the tendency toward bleeding.

Some types of von Willebrand disease do not respond to DDAVP. Tests should be done to determine a patient's specific type of von Willebrand disease before trauma or surgery occurs. A trial of DDAVP can be done before surgery to test whether von Willebrand factor levels increase.

The drug Alphanate (antihemophilic factor) is approved to decrease bleeding in patients with the disease who must have surgery or any other invasive procedure.
Blood plasma or certain factor VIII preparations may also be used to decrease bleeding.

Drugs
DDAVP is the most common treatment for people with type 1 VWD. About 80% of people with type 1 VWD respond to DDAVP therapy. Treatment with DDAVP can also be used to treat some people with type 2 VWD. Patients with Type 2B VWD should not be treated with this medication since DDAVP can induce dangerous platelet clumping. Type 3 VWD should not be treated with DDAVP since this medication does not increase the level of vWF in type 3 patients. DDAVP should only be used in people who have been shown to be responsive through a pre-treatment trial transfusion with this medication.

DDAVP can be administered intravenously or through a nasal inhaler. DDAVP has relatively few side effects although some people may experience facial flushing, tingling sensations, and headaches after treatment with this medication. Often treatment with this medication is only required prior to invasive surgeries or dental procedures.

Complications
Bleeding (hemorrhaging) may occur after surgeries or other invasive procedures.
If you have von Willebrand disease, do not take nonsteroidal anti-inflammatory drugs (NSAIDs), such as aspirin or ibuprofen, without talking to your health care provider.

Prevention
If one parent has been diagnosed with an autosomal dominant form of VWD or both parents are carriers for an autosomal recessive form of VWD, then prenatal testing can be considered. If the parent with an autosomal dominant form of VWD possesses a detectable gene change or both parents who are carriers for an autosomal recessive form of VWD possess detectable mutations, then DNA testing of their fetus would be available. DNA testing can be performed through amniocentesis or chorionic villus sampling. If the DNA change in the parent(s) is unknown then prenatal testing can sometimes be performed through biochemical testing of blood obtained from the fetal umbilical cord, which is less accurate and is associated with a higher risk of pregnancy loss.

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