What is von willebrand disease?

Von Willebrand disease is a bleeding disorder. When you have this problem, it takes longer for your blood to form clots, so you bleed for a longer time than other people.

Normally when a person starts to bleed, small blood cells called platelets go to the site of the bleeding. These cells clump together to help stop the bleeding. If you have von Willebrand disease, your blood doesn't clot well. This happens because you don't have a certain protein in your blood. Or you may have low levels of the protein or a form of it that's not normal. The protein is called the von Willebrand factor. It helps your blood to clot by helping the platelets stick together.

The disease can range from mild to severe. It is mild in most people. It can stay the same or get better or worse as you get older.

Bleeding a lot is the main symptom of von Willebrand disease. How severe the bleeding is will be different for each person.

When the disease is mild, symptoms include:

• Frequent nosebleeds.
• Some bleeding from the gums.
• Heavy menstrual periods in women.
• Bruises that appear for no reason.
• Heavy bleeding after an injury or surgery.

When the disease is more severe, you may also have:

• Blood in the urine.
• Bruising easily.
• Black, tarry, or bloody stools.
• Bleeding into the joints, which causes stiffness, pain, and swelling. This symptom is rare.

Von Willebrand disease can be hard to diagnose. Some people who have mild von Willebrand disease bleed about the same amount as other people do. You may not notice any symptoms until you bleed a lot after an injury, dental procedure, or surgery.

Your doctor will ask you how often and how much you bleed. If your doctor thinks you may have a problem with clotting, your doctor may suggest:

• Blood tests that measure von Willebrand factor activity levels or von Willebrand factor antigen.
• Genetic testing to look for gene changes and types of von Willebrand disease.

Treatment depends on the type of von Willebrand disease you have, how much you bleed, and your risk for heavy bleeding.

If you have von Willebrand disease, your treatment may include:

• Desmopressin medicine (such as DDAVP, Stimate) which helps your body release more of the von Willebrand clotting factor into your blood.
• Replacement therapy, which replaces the clotting factor in your blood.
• Medicines to help stop blood clots from breaking down.
• Birth control pills, or an intrauterine device (IUD) that contains hormones, to help control heavy menstrual periods.

If you have von Willebrand disease, you most likely will need to take extra care to treat and prevent bleeding episodes. Avoid NSAIDs and blood thinners. Try to stay at a healthy weight and keep active. This helps keep your muscles and joints healthy. Avoid sports or activities where injury and bleeding are likely, such as football and hockey.

Tell all your doctors and other health professionals, such as your dentist, that you have this disease. Doctors need to know about it before you have any procedures, because you may be at risk for dangerous bleeding.

Most large hospitals have a "bleeding disorders" resource center. Learn the phone number to the center closest to you, and carry it with you.

Take your medicines as prescribed. Avoid blood thinners and nonsteroidal anti-inflammatory drugs (NSAIDs) like aspirin and ibuprofen. Try to prevent injury. You may need to avoid contact sports like football and hockey. Tell all your health providers, including dentists, that you have this disease. And wear medical alert jewelry or carry a wallet card.