What is von Willebrand Disease ?
Von Willebrand disease (VWD) 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 VWD, 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 (adhesive) to help the platelets stick together and form a blood clot.
Von Willebrand factor also carries clotting factor VIII, another important protein that helps your blood clot. Factor VIII is the protein that's inactive or missing in haemphilia, another clotting disorder. VWD is more common and usually milder than haemophilia. In fact, VWD is the most common of all the inherited bleeding disorders. It occurs in about 1 out of every 100 to 1,000 people. VWD affects both males and females, while haemophilia mainly affects males.
Types of von Willebrand Disease
There are three major types of VWD.
In type 1 VWD, you have a low level of the von Willebrand factor, and you may have lower levels of factor VIII than normal. This is the mildest and most common form of the disease. About 75% of people who have VWD have type 1.
In type 2 VWD, 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. This makes knowing the exact type of VWD that you have very important.
In type 3 VWD, you usually have no von Willebrand factor and low levels of factor VIII. Type 3 is the most serious form of VWD, but it's very rare.
What Causes von Willebrand Disease ?
Von Willebrand disease (VWD) is almost always inherited. Your parents pass the gene for the disease on to you. You can inherit type 1 or type 2 VWD when only one of your parents passes the gene on to you. You usually inherit type 3 VWD only if both of your parents pass the gene on to you. Your symptoms may be different from your parents'symptoms. Some people carry the genes for the disease but don't have symptoms. They still can pass the disease on to their children. Some people develop a form of VWD later in life as a result of other medical conditions. This form of VWD is called acquired von Willebrand syndrome.
What Are the Signs and Symptoms of von Willebrand Disease ?
The signs and symptoms of von Willebrand disease (VWD) depend on the type and severity of the disease. Many people have such mild symptoms that they don't know they have the disorder.
If you have type 1 or type 2 VWD, 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
People with type 3 VWD may have all of the symptoms listed above, as well as severe bleeding episodes for no reason.
• Heavy menstrual bleeding is often the main symptom of VWD for women. Doctors call this menorrhagia
How is VWD treated ?
Treatment is required in case of persistent bleeding.
• Desmopressin (DDAVP) is a synthetic hormone that you usually take by injection or nasal spray. DDAVP works for most patients who have type 1 VWD and for some who have type 2 VWD.
• Von Willebrand factor replacement therapy is an infusion of a concentrate of von Willebrand factor and factor VIII into a vein in your arm.
Treatments for Women
Treatments for women who have VWD with heavy menstrual bleeding include:
• • Combined oral contraceptives (birth control pills
Living with von Willebrand Disease
Preventing bleeding and staying healthy are important if you have von Willebrand disease (VWD). You should:
• Avoid over-the-counter medicines that can affect blood clotting, including aspirin, ibuprofen, and other nonsteroidal anti-inflammatory drugs (NSAIDs).
• Always check with your doctor before taking any medicines.
• Tell your doctor and dentist that you have VWD. Your dentist can talk to your doctor about whether you need medicine before dental work to reduce bleeding.
• Exercise regularly and maintain a healthy weight. Exercise helps keep muscles flexible. It also helps prevent damage to muscles and joints. Always stretch before exercising.
Always check with your doctor before starting any exercise program. Since your parents, brothers and sisters, and children may also have von Willebrand disease, you should consider getting them tested.
Bleeding disease card
You should always carry a "Bleeding disease card" which will be given to you after the diagnosis