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Von Willebrand Disease (VWD)

What is VWD?

Von Willebrand disease (VWD) is an inherited disorder that affects the blood's ability to clot.

What causes VWD?

VWD is almost always inherited. This means it is passed from parents to children. People with the disorder may have low levels of a blood protein called von Willebrand factor. It 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.

There are 3 main types of VWD:

  • Type 1. The mildest and most common form. Your child has a low level of von Willebrand factor and possibly a low factor VIII levels.
  • Type 2. There are 4 subtypes of Type 2 VWD. Each is treated differently. Your child has von Willebrand factor in the blood, but it doesn't work properly.
  • Type 3. The most severe and rarest form. Your child has no von Willebrand factor and low levels of factor VIII. This type can cause serious bleeding in your child, from infancy into childhood.

What are the symptoms of VWD?

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

  • Frequent or long-lasting nosebleeds
  • Large bruises from minor injuries
  • Blood in the stool or urine
  • Heavy bleeding from cuts, dental work, or surgery
  • Heavy menstrual bleeding  

Children with type 3 VWD also have these symptoms. But, they may also have  severe bleeding in their muscles, joints, or other areas of the body. This bleeding causes pain and swelling. Because of these episodes, babies are often diagnosed during their first year.

How is VWD diagnosed?

Your child's healthcare provider will check your child for signs of bruising and bleeding. He or she will ask many questions about your child's symptoms and about your family history. Samples of your child's blood will be taken to check:

  • Complete blood count (CBC).  A complete blood count checks the red and white blood cells, blood clotting cells (platelets), and sometimes, young red blood cells (reticulocytes). It includes hemoglobin and hematocrit and details about the red blood cells.
  • Prothrombin time (PT) and partial thromboplastin time (PTT). Both of these tests check for bleeding and clotting problems. 
  • Von Willebrand factor antigen and activity. These tests measure the amount and activity 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.

How is VWD treated?

Long-term medicine is usually not needed. If your child has repeated bleeding inside his or her body (for example, in the joints), long-term medicine may be needed. Medicines may be used to stop bleeding, should it occur. They may also be used to prevent bleeding with planned surgery or dental work. 

One medicine is desmopressin. It is a hormone that causes the body to release more von Willebrand factor into the bloodstream. It is given intravenously (IV), into your child's vein, or into your child's nose. Another treatment is von Willebrand factor replacement therapy. Certain antifibrinolytic medicines—medicines that prevent blood clots from being destroyed—may also be prescribed.

Menstruating girls with VWD may be treated with birth control pills to help control heavy menstrual bleeding.

What are the complications of VWD?

Complications of VWD include:

  • Severe bleeding with pain and swelling
  • Anemia or low levels of red blood cells or hemoglobin, the part of the blood that carries oxygen
  • Heavy menstrual periods
  • Problems with pregnancy

How is VWD managed?

Consider these steps to reduce your child's risk for bleeding:

  • Avoid giving your child blood-thinning medicines like aspirin and nonsteroidal anti-inflammatory drugs (NSAIDs) like ibuprofen.
  • Discourage your child from taking part in sports like football, hockey, and weightlifting. Injuries may lead to bleeding.
  • Have your child wear a medical identification (ID) bracelet.
  • Make doctors, dentists, and pharmacists aware of your child's condition.
  • Educate all caretakers, from teachers to sports coaches, about the disease and treatment in the event of an emergency.

When should I call my child's healthcare provider?

Call your child's healthcare provider if your child has bleeding that you cannot stop. If your child has pain or swelling, you should call your child's doctor right away.

Key points about VWD

  • VWD is an inherited disorder that affects the blood's ability to clot. 
  • Most people with VWD have a mild form. 
  • VWD is diagnosed with blood tests.
  • Medicines may be used to stop bleeding or to prevent bleeding with planned procedures. 
  • People with VWD should avoid aspirin and NSAIDs.

Next steps

Tips to help you get the most from a visit to your child’s healthcare provider:
  • Before your visit, write down questions you want answered.
  • At the visit, write down the names of new medicines, treatments, or tests, and any new instructions your provider gives you for your child.
  • If your child has a follow-up appointment, write down the date, time, and purpose for that visit.
  • Know how you can contact your child’s provider after office hours. This is important if your child becomes ill and you have questions or need advice.
Von Willebrand Disease (VWD) - WellSpan Health

Online Medical Reviewer: Freeborn, Donna, PhD, CNM, FNP
Online Medical Reviewer: Holloway, Beth, RN, M.Ed.
Last Review Date: 2015-02-19T00:00:00
Last Modified Date: 2016-07-28T00:00:00
Published Date: 2016-07-28T00:00:00
Last Review Date: 2011-12-01T00:00:00
© 2016 WellSpan Health. All Rights Reserved. This information is not intended as a substitute for professional medical care. Always follow your healthcare professional's instructions.

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