What is Von Willebrand Disease?

If you’ve never even heard of Von Willebrand Disease (VWD) you are probably fortunate than not to know anyone with it or have it yourself. It is an inherited bleeding disorder that affects the blood’s ability to clot.

As you may know, if your blood cannot clot, and there is bleeding after an injury or surgery or even during menstruation or pregnancy your body can have a difficult time when it comes time to stop bleeding.

Typically when a blood vessel is injured and starts to bleed small blood cells manufactured in marrow called platelets clump together to stop the bleeding by forming a plug (clot) where the bleeding is occurring.

Types of VWD

There are three types of VWD: Type 1 is a low level of the Von Willebrand factor, and you may not notice that you have a disorder. This is the most common type of VWD. You have the factor VIII just lower levels than is normal.

Then there is Type 2 VWD. In this type of the disorder the factor does not work in the normal way and different gene mutations included in this type act differently.

In Type 3, you have no Von Willebrand factor and you also have low levels of factor VIII. This is the form of VWD that is the most serious type to have, fortunately it is very rare.

Individuals with VWD Type 1 typically only need intervention if they have surgery otherwise they are symptom free. However, any trauma, or surgery such as a tooth extraction requires treatment including medications and medical therapies to treat the condition. If an individual has Type 3 they will require emergency treatment if they start bleeding or they will bleed to death.

It is vitally important to not only receive the correct diagnosis of Von Willebrand Disease but to diagnose the correct Type that you have 1, 2 or 3. early diagnosis is very important so that the blood disorder is recognized before any bleeding incident should occur.

Signs and Symptoms of Willebrand Disease

Usually if the disorder is mild enough signs and symptoms will be difficult to spot. Individuals with Type 1 or Type 2 VWD may exhibit the following signs and symptoms: frequent bruising even when the injury is slight, frequent or hard to stop nosebleeds, lots of bleeding after a dental procedure, for women – heavy or long menstrual bleeding, blood in stools, blood in urine, heavy bleeding after being cut and heavy bleeding after surgery.

Those who have Type 3 VWD will show the same signs and symptoms of those with Type 1 or 2 but will also have life threatening bleeding episodes or times when bleeding goes into soft tissue or into the joints where it causes swelling and severe pain.

Menorrhagia

The symptom women notice the most is very heavy menstrual periods, often called, “menorrhagia”. This is where the bleeding with clots are larger than 1″ in diameter, they become anemic or show a low blood iron count and need to change pads or tampons more frequently than normal (more than once an hour). If you notice any of these signs or symptoms speak with your doctor about your concerns.

It can be difficult to diagnose Von Willebrand disease. Typically there is a trauma or surgery in which it becomes difficult to control the bleeding at which time the doctor or other medical professional may become suspicious of the diagnosis.

Once the disease is suspected, a medical history will be taken, give a physical examination and run a combination of blood tests that will help in diagnosing the disorder. Some of the tests that may be run are the Von Willebrand factor antigen, the Von Willebrand factor ristocetin cofactor activity test, Factor VIII clotting activity test, the Von Willebrand factor multimers test and the platelet function test.

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