Von Willebrand disease is an inherited bleeding disorder. It affects your blood's ability to clot. If your blood doesn't clot, you can have heavy, hard-to-stop bleeding after an injury. The bleeding can damage your internal organs or even be life threatening, although this is rare.

In Von Willebrand disease, you either have low levels of a certain protein in your blood, or the protein doesn't work the way it should. The protein is called von Willebrand factor, and it helps the blood clot.

Normally, when one of your blood vessels is injured, you start to bleed. Small blood cells called platelets clump together to plug the hole in the blood vessel and stop the bleeding. Von Willebrand factor acts like glue to help the platelets stick together and form a blood clot.

Von Willebrand disease is more common and usually milder than hemophilia. In fact, Von Willebrand disease is the most common of all the inherited bleeding disorders. It occurs in about 1 out of every 100 to 1,000 people. Von Willebrand disease affects both males and females, while hemophilia mainly affects males.

Types of von Willebrand Disease:

• Type 1: the mildest and most common form of the disease. In type 1, patients have a low level of the von Willebrand factor.
• Type 2: the von Willebrand factor doesn’t work the way it's supposed to. Type 2 is divided into subtypes: 2A, 2B, 2M, and 2N. Different gene mutations cause each type, and each is treated differently.
• Type 3: patients have no von Willebrand factor. Type 3 is the most serious form of Von Willebrand disease, but it’s very rare.

Symptoms

Most people with Von Willebrand disease have type 1, a mild form. This type usually doesn't cause life-threatening bleeding, and you may need treatment only if you have surgery, tooth extraction, or trauma.

If you have type 1 or type 2 Von Willebrand disease, you may have the following mild-to-moderate bleeding symptoms:

• Frequent large bruises from minor bumps or injuries
• Frequent or hard to stop nosebleeds
• Extended bleeding from the gums after a dental procedure
• Heavy or extended menstrual bleeding in women
• Blood in your stools from bleeding in your intestines or stomach
• Blood in your urine from bleeding in your kidneys or bladder
• Heavy bleeding after a cut or other accident
• Heavy bleeding after surgery

People with type 3 Von Willebrand disease may have all of the symptoms listed above, as well as severe bleeding episodes for no reason. These bleeding episodes can be life threatening if not treated right away. They also may have bleeding into soft tissues or joints, causing severe pain and swelling.
Heavy menstrual bleeding is often the main symptom of Von Willebrand disease for women. Doctors call this menorrhagia. They define it as:

• Bleeding with clots larger than about 1-inch in diameter
• Anemia or low blood iron
• The need to change pads or tampons more than every hour

Medicines are used to:

• Increase the release of von Willebrand factor into the bloodstream
• Replace von Willebrand factor
• Prevent the breakdown of clots
• Control heavy menstrual bleeding in women

Treatments for women who have Von Willebrand disease with heavy menstrual bleeding include:

• Combined oral contraceptives (birth control pills)
• A levonorgestrel intrauterine device. This is a contraceptive device that contains progestin. It’s placed in the uterus (womb).
• Aminocaproic acid or tranexamic acid. These antifibrinolytic drugs can reduce bleeding by slowing the breakdown of blood clots.

For some women who are done having children or don’t want children, endometrial ablation is performed. This procedure destroys the lining of the uterus. It has been shown to reduce menstrual blood loss in women with Von Willebrand disease.