Von Willebrand Disease Explained

Von Willebrand Disease is a hereditary disorder that currently affects approximately one to two percent of the population. It is a condition that affects platelet function, and is signified by the presence of an abnormality of the von Willebrand factor in the patient’s blood. The disease gets its name from Erik von Willebrand, a physician who discovered a new bleeding disorder in Sweden and Finland.

What Causes von Willebrand Disease?

In people suffering from von Willebrand disease, a protein that is responsible for allowing blood platelets to stick together is considered abnormal. This protein inhibits clotting, particularly at the site of an injury. This leads to excessive bleeding and is quite similar to hemophilia. Blood flow will eventually stop, but this loss of blood can lead to other complications.

What is Unique About von Willebrand Disease?

Unlike hemophilia, women and men are equally affected by the disease. It is passed on genetically, but it is also possible to develop acquired von Willebrand disease later in life. This is usually due to antibodies that form and attack the proteins that assist platelets in clotting. It is also possible to develop von Willebrand disease through other diseases, such as rheumatoid arthritis, kidney disease, cancer and systemic lupus erythematosus.

If a person has von Willebrand disease, they have a fifty percent chance of passing the gene on to their children. Even if the symptoms of von Willebrand disease, the risk for transmission does not decrease.

How Many Types of von Willebrand Disease Exist?

There are four distinct types of von Willebrand Disease. They include:

Type One – This is the most mild and common form of the disease. The level of von Willebrand factor is much lower in people with type one and other factors that can make the disease worse are lower as well.

Type Two – This type is signified by an actual abnormality in the von Willebrand factor itself. This type is subdivided into two classifications, Type IIa and Type IIb. In Type IIa, platelets do not bind together, while in Type IIb, they may actually have an increase ability to bind together.

Type Three – This is the most severe type of von Willebrand disease. A complete lack of the von Willebrand factor usually occurs. This is the most rare form of von Willebrand disease, affecting only 10% of the total sufferers of the disorder.

Pseudo – This type closely resembles Type IIb, but instead of having the defect present in the von Willebrand factor, the platelets themselves are abnormal.

How is von Willebrand Disease Diagnosed?

Since the disease is hereditary, if parents of a child have experienced heavy nosebleeds, increase bleeding after surgical procedures or a history of bruising easily, diagnosis is made easier. These factors as well as laboratory tests assist a doctor in determining whether a child has von Willebrand Disease. Once this has been determined, further testing is necessary to determine which type of the disease is present.

How is von Willebrand Disease Treated?

Von Willebrand disease is rarely fatal, and there are several treatment options. The treatment will depend on the type as well as the severity of the symptoms. Intranasal medication, such as desmopressin acetate or DDAVP is an effective method of treating mild cases of von Willebrand disease. Other treatments include an infusion of what is called a “viral inactivated factor VIII” that has forms of von Willebrand factor that are found in Humate-P, Alphanate and Koate DVI. This is normally recommended if a patient with von Willebrand Disease requires surgery or is bleeding excessively.

Avoiding unnecessary trauma is recommended, particularly for children. Menstruating teens will also need to take more precautions. Heavy contact sports are not recommended if the case of von Willebrand Disease is severe.

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