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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 in the blood, 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. This is the mildest and most common form. Your child has low levels of von Willebrand factor and may have low levels of factor VIII.
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 normally.
Type 3.This is 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 the 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 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 with a nasal spray. 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. This can help control heavy menstrual bleeding.
What are possible complications of VWD?
Possible 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?
You can take these steps to reduce your child's risk for bleeding:
Not giving your child blood-thinning medicines such as aspirin and nonsteroidal anti-inflammatory drugs (NSAIDs) like ibuprofen.
Discouraging your child from taking part in sports like football, hockey, and weightlifting. Injuries may lead to bleeding.
Having your child wear a medical identification (ID) bracelet.
Making all of your child's healthcare providers, dentists, and pharmacists aware of your child's condition.
Educating all caregivers, 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 healthcare provider 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 not take aspirin or NSAIDs.
Tips to help you get the most from a visit to your child’s healthcare provider:
Know the reason for the visit and what you want to happen.
Before your visit, write down questions you want answered.
At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you for your child.
Know why a new medicine or treatment is prescribed and how it will help your child. Also know what the side effects are.
Ask if your child’s condition can be treated in other ways.
Know why a test or procedure is recommended and what the results could mean.
Know what to expect if your child does not take the medicine or have the test or procedure.
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.
Online Medical Reviewer:
Amy Finke RN BSN
Online Medical Reviewer:
L Renee Watson MSN RN
Online Medical Reviewer:
Richard LoCicero MD
Date Last Reviewed:
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