Von Willebrand Disease

von Willebrand disease is the most common inherited bleeding disorder, affecting about 1% of all people. Unlike hemophilia, vWD is inherited autosomally, so it can occur equally among females and males. There are three types of vWD: type 1, type 2 (several subtypes), and type 3. Having von Willebrand disease means that a person either produces too little of a blood protein called von Willebrand factor or a person’s body does not produce the protein at all. von Willebrand factor helps the body stop bleeding by helping to form blood clots. vWD can be very difficult to diagnose and the testing is complicated. It is very important to go to a qualified treatment center where a hematologist and experienced staff can conduct the testing.

Incidence

vWD occurs in approximately 1-2 out of every 100 people, affecting 1%-2% of the world’s population.

Symptoms

¨ Easy bruising
¨ Frequent nosebleeds
¨ In women, long/heavy menstrual periods (over 6 days)
¨ Excessive bleeding from the mouth
¨ Abnormal bleeding from minor cuts and scrapes
¨ Prolonged bleeding after surgery
¨ More serious symptoms include joint and muscle bleeding

Severity

Of the three types of vWD, type 1 is the most common, affecting 70%-80% of people with vWD. A person with type 1 vWD usually has mild to moderate bleeding symptoms since von Willebrand factor levels are lower than normal but function correctly.

Type 2 vWD, on the other hand, accounts for approximately 15-30% of people with vWD. With type 2, vWF levels are usually normal but they do not function correctly.

Type 3 vWD is the rarest form of the disease, affecting about 1% of people with vWD. A person with type 3 vWD produces little, if any, vWF and usually has the most severe bleeding symptoms.

Treatment

The treatment of vWD is based on the type and symptoms of the disease. The goal of treatment is to correct the clotting problem, which is usually done by raising the levels of vWF and factor XIII. Right now the only vWF concentrate that is approved by the United States is Humate-P, which is an antihemophilic factor/von Willebrand Factor complex. Humate-P is derived from human plasma and given by infusion. Another treatment is DDAVP, which is a medicine that works by helping the body to release stored vWF into the bloodstream and increasing levels of factor XIII. DDAVP is usually only effective in vWD patients with type 1 vWD. Another alternative for people with mild or moderate vWD is Stimate. It is essentially DDAVP in a nasal spray. Because it is sprayed rather than injected, it is much easier to use. Local clotting agents are also used to help correct clotting problems. These agents are called antifibrinolytics. These medicines do not help the body form blood clots, but they help protect blood clots and hold them in place. They are often used in addition to other medicines. Antifibrinolytics can be taken as an injection or by mouth (tablet, capsule, elixir or mouthwash). Oral contraceptives can also increase vWF levels.

Prognosis

With adequate care, people with vWD should be able to lead very healthy lives. Proper diagnosis and treatment is, of course, essential. vWD is often misdiagnosed and misunderstood. Working with a doctor that specializes in bleeding disorders, a hematologist, is extremely important. Also, self-educating and advocating is the only way to insure the best treatment and overall quality of life.