Conditions Basics

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.

What are the types?

There are three major types of the disease. They range from mild to severe.

• In type 1, you are missing some von Willebrand factor. This is the most common type. It usually causes mild bleeding episodes. You may not know you have the disease, and you may not need treatment.
• In type 2, you have the von Willebrand factor, but it doesn't work as it should. This usually causes mild to moderate bleeding episodes.
• In type 3, you don't have the von Willebrand factor or you have a very small amount. This type is rare. It usually causes severe bleeding episodes. Bleeding may happen without any reason and in more than one place in your body.

What causes it?

Von Willebrand disease usually is passed down through families (inherited). If you have the disease, your doctor may suggest that your family members get tested for it too.

It's also possible to get the disease later in life. This is called acquired von Willebrand disease. This rare form of the disease isn't inherited. Instead, it seems to be caused by certain diseases or medicines that decrease the amount of von Willebrand protein in your blood.

What are the symptoms?

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.

How is it diagnosed?

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.

How is von Willebrand disease treated?

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.