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Von Willebrand Disease

Von Willebrand Disease

Von Willebrand disease (VWD) is an inherited disorder that affects the blood's ability to clot. People with VWD may experience heavy bleeding after an injury, leading to physical damage or even death. It is a cause of bleeding in early life and is often associated with family members who also have bleeding problems. But young children and adolescents without a family history of bleeding may suddenly have a bleeding problem. The disorder occurs as often in males as it does in females.

About von Willebrand disease

VWD is almost always inherited—it is passed from parents to children. People with the disorder may have low levels of a blood protein called von Willebrand factor, which helps the platelets in blood stick together to stop bleeding. Sometimes von Willebrand factor is present, but not working well. VWD may also cause problems with another clotting protein in the blood known as factor VIII.

Illustration of blood components
Von Willebrand factor is a protein in the blood that helps platelets stick together and form blood clots. (Click to Enlarge)

Types of VWD

VWD is divided into three types:

  • With type 1, the most common form, your child has low levels of von Willebrand factor and possibly low factor VIII levels.

  • With type 2, your child has von Willebrand factor in the blood, but it doesn't work properly.

  • With type 3—the most severe, yet the rarest form—your child has no von Willebrand factor and low levels of factor VIII. This type can cause significant bleeding in your child, from infancy into childhood.

Symptoms

The symptoms of VWD may vary, depending on type. Type 1 and type 2 generally have many of these symptoms:

  • Frequent or prolonged nosebleeds

  • Large bruises from minor bumps or injuries

  • Blood in the stool or urine

  • Heavy bleeding after a cut or surgery

  • Heavy menstrual bleeding  

In addition to these symptoms, children with type 3 VWD usually have severe bleeding episodes with no known cause. Because of these episodes, babies are often diagnosed during their first year.

Diagnosis

If you suspect that your child has VWD, your doctor will probably do a physical exam to check for signs of bruising and bleeding and ask you about your medical history since the disease is passed through the genes. The following blood tests are commonly used to diagnose VWD:

  • Von Willebrand factor antigen. This test measures the amount of von Willebrand factor in your child's blood.

  • Platelet function test. This evaluates how well blood platelets are working.

  • Factor VIII clotting activity. This test determines the amount of factor VIII present in the blood.

Treatment

Some children with a mild form of VWD do not need ongoing treatment; they may only need help after a medical procedure, including dental work, or after an accident that causes bleeding. Others may need to take medications.

One common drug used to treat VWD is desmopressin, a hormone that causes the body to release more von Willebrand factor and factor VIII into the bloodstream. Another treatment is von Willebrand factor replacement therapy, which infuses von Willebrand factor and factor VIII into the body intravenously. Certain antifibrinolytic drugs—medicines that prevent blood clots from being destroyed—may also be prescribed. Aminocaproic acid and tranexamic acid are two examples of these drugs.

If your child has a more serious form of VWD in which heavy bleeding is more likely, educate all caretakers, from teachers to sports coaches and the school nurse, about the disease and needed treatment in the event of an emergency.

Prognosis

Consider these steps to reduce your child's risk for bleeding and to live a healthier life:

  • Avoid giving your child blood-thinning medications like aspirin and ibuprofen.

  • Discourage your child from participating in strenuous sports like football, hockey, and weightlifting.

  • Have your child wear a medical ID bracelet.

  • Make doctors, dentists, and pharmacists aware of your child's condition, as well as teachers, coaches, and other school personnel.

 

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