von Willebrand Disease

von Willebrand Disease

Lee este articulo

Everyone has to deal with bruises and bloody noses from time to time. But for people with a condition called von Willebrand disease, these things can sometimes be a problem.

Some people with von Willebrand disease never even know they have it because the symptoms are so mild. People with more severe forms of the disease are less likely to have problems if they get the proper diagnosis and treatment.

What Is von Willebrand Disease?

When people have von Willebrand disease (vWD for short) their blood doesn't clot properly. That means cuts and wounds can't scab over as well, so they bleed longer than normal.

Bleeding is usually a sign that a blood vessel has been cut or torn. Normally, when someone bleeds, small cells in the blood called platelets plug the hole. With the help of calcium, vitamin K, and a protein called fibrinogen, the platelets create a mesh to hold the plug in place and close the wound. As this mesh dries, it hardens into a scab.

A substance in the blood called von Willebrand factor helps platelets stick to damaged blood vessels. Special proteins known as clotting factors are also needed to help blood clot — von Willebrand factor includes one of these clotting factors, called factor VIII.

People with vWD have bleeding problems because the levels of von Willebrand factor or factor VIII in their blood are abnormal. In some cases, the factors don't work the way they're supposed to.

The disease is named after Erik von Willebrand, the doctor who first identified it. It's similar to another bleeding condition called hemophilia. Both conditions are rare. Unlike hemophilia, which usually affects only guys, both girls and guys can have vWD.

The Types of von Willebrand Disease

There are different kinds of vWD:

  • In type 1, a person has less von Willebrand factor in the blood than normal. This is the most common and mildest form of the disease. In fact, it might be so mild that the person never knows he or she has vWD. People with type 1 vWD sometimes have mild bruising or nosebleeds, and they can bleed a lot with injuries, surgery, or when they have a tooth pulled.
  • In type 2, the level of von Willebrand factor in the blood is normal, but it doesn't work properly. This can lead to moderate bleeding problems.
  • People with type 3 vWD have severe bleeding problems. They have no measurable von Willebrand factor in the blood and very low factor VIII levels.
  • In pseudo or platelet-type vWD, the person's platelets are abnormal, making them stick to von Willebrand factor too well. This causes clotting problems due to low numbers of platelets and levels of von Willebrand factor.

What Causes It?

A genetic disorder, von Willebrand disease is passed down from parent to child. If a parent has vWD, a child has a 50% chance of getting the gene for the condition.

When a child has type 1 or type 2, it usually means he or she inherited the gene from one parent. With type 3, the child usually inherits genes for the disease from both parents.

Signs and Symptoms

Signs of von Willebrand disease can include:

  • bruising easily
  • unusually heavy periods or other abnormal menstrual bleeding in girls
  • bleeding from the gums, nose, and lining of the intestines
  • prolonged oozing of blood from cuts
  • bleeding too much or for too long after a tooth is pulled or tonsils are removed

Mild cases of von Willebrand disease can be hard to diagnose. If a doctor thinks you have vWD, he or she will examine you and ask about your medical history. Your medical history includes things like your past health, your family's health, and any medicines you're taking. The doctor also may send a blood sample to a lab for tests.

What to Do

Having von Willebrand disease doesn't usually mean big life changes. People with more severe vWD should avoid contact sports like football and hockey, but other sports and activities are usually OK.

If someone with vWD starts bleeding, it's usually enough to put pressure on the area and wait for the bleeding to eventually stop. For nosebleeds, pinch the soft part of the nose to help stop the bleeding.

Girls with vWD who have started their periods might want to carry extra pads or even a change of clothes in case of accidents. Sometimes, a girl's doctor may prescribe birth control pills to help control heavy menstrual bleeding.

If you have vWD, talk with your doctor before taking medicine for pain or fever. Don't take aspirin and ibuprofen because they interfere with platelet function and can increase the risk of bleeding. It's usually OK to take acetaminophen for pain or fever, since it has no effect on platelet function.

Medicines for vWD

Some people with more serious vWD may need to take medicines. The most common medicine for type 1 von Willebrand disease is called desmopressin. It causes a temporary increase in the von Willebrand factor level in the blood. It can be given in two ways: by injection or by being sniffed into the nose. Desmopressin may also help some people with type 2 von Willebrand disease.

People with type 3 (and some with type 2) disease need a medicine called Humate-P. It contains both factor VIII and von Willebrand factor. Humate-P is injected into a vein. Patients with type 1 also might need a shot of Humate-P in certain situations, like after major surgery or a serious accident.

Other medicines, like Amicar, control bleeding by keeping blood clots from breaking down too quickly.

Most of the time, people with von Willebrand disease can do everything their friends do. Speaking of friends, if you have vWD, it doesn't hurt to let the people in your life (like friends, teammates, or coaches) know that you have it. Von Willebrand disease isn't contagious — you can't give it to anyone — and it can help to know someone has your back.

Reviewed by: Elana Pearl Ben-Joseph, MD
Date reviewed: January 2014

Related Articles
Note: All information is for educational purposes only. For specific medical advice, diagnoses, and treatment, consult your doctor.
© 1995-2014 KidsHealth® All rights reserved. Images provided by iStock, Getty Images, Corbis, Veer, Science Photo Library, Science Source Images, Shutterstock, and Clipart.com