What is Von Willebrand Disease?

Von Willebrand disease (VWD) is a disorder that makes it hard for your blood to clot. This happens because you don’t have enough of a clotting protein called von Willebrand factor (VWF). It could also happen because you have a type of VWF that doesn’t work well. If you have von Willebrand disease, a cut, accident, or surgery can result in bleeding that’s hard to stop.

VWD is the most common inherited bleeding disorder. That means you get it from your parents. It affects an estimated 1 in 100 to 1 in 1,000 people.

Types of VWD

There are three types of inherited VWD and one type of the disorder that isn’t hereditary.

Type 1: This is the most common form of inherited VWD. About 60% to 80% of people with VWD have this type. With Type 1, you don’t have enough von Willebrand factor in your blood. Typically, you have 20% to 50% of normal levels. Symptoms of Type 1 VWD are mild.

Type 2: This is the second most common form of inherited VWD. It’s caused by your own VWD factor not working well. If you have VWD, there’s a 15% to 30% chance you have Type 2. Symptoms range from mild to moderate.

Type 3: This is the rarest form of inherited VWD. It’s found in 5% to 10% of cases. If you have this type, you typically have no von Willebrand factor and very low levels of another protein needed for clotting. Type 3 has the most severe symptoms.

Acquired: It’s possible to get this form of VWD if you have an autoimmune disease, like lupus. An autoimmune disease is one where your body’s natural defense system (immune system) fights itself. You can also get acquired VWD after taking certain medications, or from heart disease or some types of cancer.

What Causes It?

Most of the time, you inherit VWD from one or both parents. You can inherit Type 1 or Type 2 if one of your parents passes the gene on to you. You usually only get Type 3 if both of your parents pass the gene to you.

It’s possible to have the gene that causes the disorder but not have any symptoms. In that case, you can still pass the gene to your children.

What Are the Symptoms?

It depends on what type of von Willebrand disease you have.

With Type 1 and Type 2, your symptoms can range from mild to moderate. They include:

• Frequent large bruises from minor injuries
• Frequent or hard-to-stop nose bleeds
• Blood in your stool or pee (from internal bleeding)
• Heavy bleeding after a cut, accident, or minor medical procedure
• Bleeding for a long period of time after major surgery
• Heavy or long menstrual periods

If you’re a woman with VWD, you’ll have periods with clots larger than an inch in diameter. This means you may need to change your pad or tampon more than every hour. You’ll also likely develop anemia (low iron in the blood). These are symptoms of von Willebrand disease, but by themselves, they don’t prove you have VWD.

With Type 3, you may have all the symptoms of Type 1 and Type 2, plus episodes of severe bleeding for no reason. You also might experience severe pain and swelling in your soft tissues and joints because of bleeding.

How Is It Diagnosed?

If your doctor suspects you have von Willebrand disease, they’ll start with a detailed medical history. Remember, VWD is almost always inherited. Your family medical history will show if you’ve experienced typical symptoms or if other relatives have a bleeding disorder or symptoms of one.

You might have to take a clotting test to see how well your blood can form a clot and how long it takes. Your doctor may also order several blood tests, such as an antigen test. This shows how much VWF you have in your blood plasma.

Levels of VWF go up and down because of things like stress and exercise. For this reason, you might have to have these tests more than once to confirm the results.

What’s the Treatment?

There’s no cure for von Willebrand disease, but it can be treated and/or managed. A key to managing this disorder is to reduce the risk of bleeding before it starts. This means avoiding certain drugs that can thin your blood. Your doctor might recommend you avoid aspirin and medications known as NSAIDs, such as ibuprofen (Advil, Motrin) and naproxen (Aleve). Acetominophen (Tylenol) is a good alternative to aspirin and NSAIDs.

How your condition is treated will depend on how severe your symptoms are.

With Type 1, you typically only need treatment if have you have surgery, a tooth extraction, or are injured.

The most common treatment for von Willebrand disease is desmopressin acetate (DDAVP). It’s available as an injection or a nasal spray. DDAVP is a synthetic form of the hormone vasopressin. It causes the release of von Willebrand factor from your cells. A side effect of this hormone is that it causes your body to retain water. As a result, you will need to be on fluid restrictions if you’re taking the medication.

Your doctor might also recommend that you get clotting factor concentrates through an IV.

If you’re going to have a dental procedure, you may need to take aminocaproic acid or tranexamic acid. These prevent the breakdown of blood clots. You take these medications by mouth, either in liquid or pill form. You may also take them if you're bleeding from your nose or mouth, or if you have heavy bleeding with your period.

If you’re a woman with VWD and heavy periods, your treatment may also include birth control pills. These can increase the amount of von Willebrand factor in your blood. Another possible treatment is a levonorgestrel intrauterine device. This is a type of birth control that contains the hormone progestin. If you’re done having children or don’t want any, you can also have an endometrial ablation. This procedure destroys the lining of the uterus and reduces the amount of blood you lose during your period.

If you have Type 3 and are experiencing bleeding, you need to get treatment right away. Bleeding episodes can be fatal if not treated immediately.

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