Von Willebrand Disease

What is Von Willebrand Disease

Von Willebrand disease is the most common bleeding disorder. It occurs when the body makes too little of the von Willebrand factor (VWF)—a protein that helps blood to clot—or when the VWF does not work the way it should.

VWF is a carrier for another protein that helps blood to clot (factor 8). When VWF levels are low or when the VWF is not working properly, factor 8 levels are low as well and the body is less able to form stable blood clots.

There are four major types of von Willebrand disease:

• Type 1. This is the mildest and most common type. People with this type have a low level of the VWF along with decreased factor 8 activity.
• Type 2. This type happens when the VWF does not work as it should.
• Type 3. This is a severe and rare type. People with this type usually have no VWF.
• Acquired von Willebrand disease. This type happens when von Willebrand disease occurs as a result of another condition.

What are the causes?

This condition may be caused by:

• A gene that is passed along from a parent (inherited).
• A medical condition, such as an autoimmune disease or cardiovascular disease.

What increases the risk?

This condition is more likely to develop in people who have a parent with this condition.

What are the signs or symptoms?

Symptoms of this condition include:

• Easy bruising.
• Nosebleeds that take longer than usual to stop.
• Bleeding from the gums after a dental procedure.
• Heavy menstrual bleeding in women.
• Blood in the stool (feces).
• Blood in the urine.
• Excessive or prolonged bleeding:
  • From a cut.
  • After an accident or surgery.
  • After childbirth.
• Bleeding into muscles. This creates a painful, full, firm muscle.
• Bleeding into joints. This creates a painful, swollen joint.

Some people do not have any symptoms.

How is this diagnosed?

To diagnose this condition, your health care provider will ask about your symptoms and whether members of your family have a history of bleeding that lasts longer than normal. Your health care provider will also do tests to check the ability of your blood to clot, the level of the VWF and factor 8 in your blood, and how well the VWF works. He or she may also order genetic tests to confirm the diagnosis.

How is this treated?

Treatment for this condition depends on the type of von Willebrand disease and its severity. Often, treatment is not needed. If treatment is needed, it may involve:

• Medicines to increase the production of the VWF. These may be given through an injection, an IV tube inserted in a vein, or a nasal spray.
• Medicines to help your blood to clot (clotting factor concentrates). These may be given after an injury, surgery, or another event that causes bleeding.
• Medicine applied directly to cuts (fibrin glue) to assist the blood-clotting process.
• Medicines to prevent blood clots from breaking down too soon.
• Contraceptive medicines to prevent heavy menstrual bleeding in women.

Follow these instructions at home:

Medicine

• Take over-the-counter and prescription medicines only as told by your health care provider.
• Always check with your health care provider before you take any medicine. You will need to avoid over-the-counter medicines that can affect the clotting process. Some of these are:
  • Aspirin and other drugs that contain salicylates.
  • NSAIDs.

Exercise

• Exercise regularly and maintain a healthy weight. Exercise helps keep muscles flexible and helps prevent damage to muscles and joints. Activities that are usually safe for people with this condition include biking and walking. Activities that are not safe include contact sports like football, soccer, hockey, and wrestling.
• Always check with your health care provider before you start any exercise program.

General instructions

• Tell your dentists, doctors, and all other health care providers who treat you that you have von Willebrand disease. There are things that your health care provider can do to prevent or reduce bleeding during and after procedures. For example, medicine can be given during dental procedures to reduce bleeding.
• Wear a medical ID bracelet to alert health care providers that you have von Willebrand disease.
• If you cut yourself, have a nosebleed, or are bleeding for any other reason, apply consistent pressure to the area until the bleeding has stopped completely. This may take several minutes.

Get help right away if:

• You have bleeding that does not stop when you follow your treatment plan.