Von Willebrand Disease

What is von Willebrand Factor?
von Willebrand factor (VWF) is a clotting protein in the blood.  It is produced in the cells that line blood vessels and then is released into the blood stream.  VWF has two functions; One is to bind factor VIII, the clotting protein that is low in the most common form of hemophilia.  When factor VIII is bound to VWF, it is protected from being destroyed in the blood.  The second function of VWF is to bind to platelets and enable them to function normally in making a platelet plug and clot.

Why do People with von Willebrand Disease Have Bleeding Problems?
Bleeding occurs when blood vessels are damaged, allowing blood to escape through holes in the blood vessels walls.  A healthy body acts quickly to stop the bleeding.  VWF acts like a "glue" to help platelets stick to the damaged areas of the blood vessel wall.  The first platelets that bind to this area attract other platelets to form a platelet "plug."
    People with VWF have bleeding problems because their blood forms incomplete or unstable platelet plugs. They may also have difficulties with making a fibrin clot.  The fibrin clot is a mesh or gauze-like covering on the platelet plug that protects the damaged blood vessel wall.  In order for this clot to form, a series of clotting factors must interact.  One of these clotting factors is factor VIII. VWF carries and protects factor VIII from breakdown in the blood.  If VWF is present at low levels or does not work properly there is deficient binding to factor VIII and the levels of factor VIII may also be low, and bleeding occurs because an insufficient fibrin clot forms.

Is von Willebrand Disease the Same in Every Patient?
No.  There are many different types of VWD.  In addition, most types of VWD can range from mild or severe.  Some people with a particular type of VWD may have almost no symptoms, while others may experience major bleeding problems.
   

What are the Signs and Symptoms of von Willebrand Disease?
VWD can be a mild disorder associated with few, if any symptoms.  Sometimes, affected individuals are not identified until they have excessive bleeding following a serious injury, accident, invasive dental work, or surgery.  In other cases, diagnosis of one family member may lead to the identification of VWD in other family members as well.
    Bleeding in persons with VWD usually involves the mucous membranes, the delicate tissues that line such body passages as the nose, mouth, uterus, vagina, stomach, and intestines.  Some of the usual symptoms of VWD include frequent nosebleeds, easy bruising, heavy menstrual flow, and excessive bleeding following surgery, childbirth, or dental work.  Bleeding may occur in the absence of an injury.  Bleeding from the stomach or intestines or blood in the urine may also be a problem.  Bleeding into the joints or muscles is rare in people with mild VWD but can occur in people with severe type 3 VWD, as they have very low factor VIII levels.
    Usually, people with very low VWF levels or significant qualitative abnormalities in their VWF will have the most severe symptoms.  Other factors, such as blood type and hormone levels, can also affect VWF levels in the blood.  A person's symptoms may change throughout his or her life.  However, the type of VWD cannot change.

How Does a Person Get von Willebrand Disease?
von Willebrand disease is known as an inherited or genetic disorder, meaning it is passed down through families via information in cells, called genes. (There are rare cases in which it is acquired during one's lifetime, usually in conjunction with other serious health problems such as lupus or certain malignancies.)  VWD occurs when the information in the gene is incorrect.  The result is that either not enough VWF protein is produced or the protein that is made is abnormal.

How is von Willebrand Disease Diagnosed?
VWD may be a difficult disorder to diagnose.  In particular, it is often hard to distinguish individuals with mild VWD from those without.  People whose symptoms are fairly mild may live for many years without a diagnosis or perhaps may never be diagnosed.  It may also be hard to distinguish between VWD and other disorders, such as qualitative platelet disorders.  In some cases, people with these conditions have many of the same symptoms as individuals with VWD.
Laboratory tests are an important part of VWD diagnosis.  Repeat testing is often necessary in order to get a clear answer.  This is because the level of VWF may change on a day-to-day basis.  In certain situations, the amount of VWF is temporarily increased.  In particular, higher than usual VWF levels are often seen in:
                » people who are under stress (as are many people who are having 
                   laboratory test performed!) or who exercise a lot
                » women who are pregnant, using birth control pills, or nursing
                » persons who have an overactive thyroid (hyperthyroidism)
                » individuals who have just had surgery or a blood transfusion

How is von Willebrand Disease Treated?
Treatment for an individual with VWD depends on the type of VWD he or she has, the symptoms and their severity.
    For minor bleeding problems, such as bruising, treatment may not be necessary.  A nosebleed may be stopped by pinching the bridge of the nose for 10 to 15 minutes.  Some bleeds in the mouth or nose may be treated with medicine applied at the bleeding site.  Then, once a clot is formed, oral medication can be used to help keep the blood clot in place longer.  Women with heavy menstrual bleeding may benefit from oral estrogens or Stimate nasal spray.  All of these medications require a prescription from a physician.
    More serious bleeding problems may require other forms of treatment.  Possible complications of excessive bleeding include weakness and fatigue from loss of blood, anemia (low blood count requiring iron therapy or a red blood cell transfusion), or life-threatening hemorrhage following surgery or trauma.

I Just Found Out that I have VWD and I'm Really Upset.  What Should I do?
For some people, the emotional trauma of finding out that they have VWD is one of the most difficult parts of having this disorder.  The process of coming to terms with the diagnosis of VWD is different for each person.  Talking with friends, health-care professionals, and other people with VWD may help you sort out your feelings and reactions.  Remember you are the same person as before the diagnosis.  The only difference is that now you know why you have bruising  or bleeding.
    

Above are excerpts from the National Hemophilia Foundation's 
"von Willebrand Disease Just the FAQs".  

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