Von Willebrand Disease (VWD)
Von Willebrand disease (VWD) is an inherited disorder that affects
the blood's ability to clot.
VWD is almost always inherited.
This means it is passed from parents to children. People with the disorder may have
levels of a blood protein called von Willebrand factor. It helps the platelets in
stick together to stop bleeding. Sometimes von Willebrand factor is in the blood but
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
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
Your child's healthcare provider
will check your child for signs of bruising and bleeding. They will ask many questions
about your child's symptoms and about your family history. Samples of your child's
will be taken to check:
Complete blood count
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
Von Willebrand factor antigen
These tests measure the amount and activity of von
Willebrand factor in your child's blood.
This evaluates how well blood platelets are working.
Factor VIII clotting
This test determines the amount of factor VIII in the
Long-term medicine is often not
needed. If your child has repeated bleeding inside their body (for instance, 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
One medicine is desmopressin. It is
a hormone that causes the body to release more von Willebrand factor into the
bloodstream. It is given through an IV (intravenously) into your child's vein, or
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.
Possible complications of VWD
- Severe bleeding with pain and swelling
- Anemia or low levels of red blood cells or hemoglobin, the part of the blood that
- Heavy menstrual periods
- Problems with pregnancy
You can take these steps to reduce
your child's risk for bleeding:
- Don't give your child medicines such
as aspirin and nonsteroidal anti-inflammatory drugs (NSAIDs) like ibuprofen. These
can cause bleeding in children with VWD. Ask your child's healthcare provider for
list of medicines and supplements to avoid.
- Discourage your child from taking part
in certain contact sports. Injuries may lead to bleeding. Talk with your child's
provider about what sports and activities are safe.
- Have your child wear a medical
identification (ID) bracelet at all times.
- Make all of your child's healthcare
providers, dentists, and pharmacists aware of your child's condition.
- Educate all caregivers, from teachers
to sports coaches, about the disease and treatment in case of an emergency.
When to Call a Healthcare Provider
Call your child's healthcare provider if your child has bleeding that
you can't stop. If your child has pain or swelling, call the provider right away.
- VWD is an inherited disorder that
affects the blood's ability to clot.
- Most people with VWD have a mild
- VWD is diagnosed with blood
- Medicines may be used to stop bleeding
or to prevent bleeding with planned procedures.
- People with VWD should not take
aspirin or NSAIDs.
- All caregivers should be educated about your child's condition
and what to do in an emergency.
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.