Von Willebrand

Von Willebrand disease is an inherited disorder characterized by bleeding from mucous membranes (mouth, nose, throat, GI tract) and skin surfaces. The condition is caused by a defect or deficiency of a clotting protein called von Willebrand factor. This glue-like protein helps platelets in the blood stick together to form a plug over the site of an injury. Since people with vWD do not form a complete plug, bleeding continues longer.

Von Willebrand disease is different from hemophilia in that it affects males and females equally. It’s the most common inherited blood condition, and approximately 1 to 3 percent of the population is affected.

The most common symptoms of von Willebrand disease (vWD) are excessive bruising, nosebleeds, heavy menstrual cycles (lasting more than 7 days), and excessive bleeding following surgery, childbirth, or tooth extraction.

It doesn’t show up in routine blood tests. To diagnose vWD, a physician needs to order special tests. A person suspected of having vWD or another blood condition can be referred to a specialist hematologist or to a hemophilia treatment center.

A medical evaluation to diagnose vWD includes the individual’s medical history, the family medical history, a physical examination, and specific blood tests. Levels of von Willebrand factor can vary, so lab tests may have to be repeated.

Classification

vWD or Factor Level

Complications

Type I

Most Common

Reduced levels of von Willebrand factor

Bleeding symptoms are usually very mild.

Type II

Von Willebrand factor does not function properly

Patients usually have mild to moderate symptoms

Type III

Rare

Absence or very low levels of von Willebrand factor and factor VIII

Patients can experience deep and extensive tissue bleeding, similar to hemophilia – similar precautions apply.

The treatment approach will be selected by your hematologist and will depend on the type of vWB determined by blood tests and examination.

Treatments Options:

Product:

Specifics:

Amicar

This product is available in liquid or tablet form. It is primarily utilized to treat bleeding in the mouth or nose. Additionally, it is utilized for menstrual bleeding that is heavy in nature.

Desmopression Acetate

Available in a concentrated nasal spray or in an injectable formulation (Stimate or DDAVP). This product stimulates the release of vW factor from the lining of the blood vessels.

Clotting Factor Concentrations that contain von Willebrand factor and factor VIII

These treatment options must be given intravenously.

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