Von Willebrand Disease

What is von Willebrand disease?

Von Willebrand is a bleeding disorder. The disease can cause bleeding problems because blood does not clot well after an injury.

Normally, pieces of blood cells (platelets) clump together after an injury. This helps form a clot and stops bleeding. A protein called von Willebrand factor helps blood clot.

In von Willebrand disease, there’s not enough of this protein or the protein does not work right. The platelets do not stick together, which can cause too much bleeding. A similar bleeding disorder that most people have heard of is hemophilia.

  • Type 1: Von Willebrand factor is fairly normal, but there is not enough of it. Children with this type may also be low on another clotting factor, called factor VIII. This is the most common type of the disease. It tends to be mild.

    Type 2: Von Willebrand factor is abnormal. There are several kinds of type 2 von Willebrand disease. Some are more severe than others.

    Type 3: There is little or no von Willebrand factor. Often a child with this disease type also has very little factor VIII. This type of von Willebrand disease is the most serious. It is extremely rare.

  • Von Willebrand disease is caused by a change (mutation) in a gene that makes von Willebrand factor. Von Willebrand factor is a protein that helps platelets stick to damaged areas of blood vessels.

    The abnormal gene is passed down from a mother or father or both parents.

    Usually, children who get the gene from just 1 parent will have type 1 or type 2 disease. They may have no symptoms or mild symptoms. But sometimes their symptoms are very serious. Even within a family, some people may have more problems with their disease than others.

    Children who get the gene from both parents will likely have type 3 disease. Their symptoms are usually serious.

Von Willebrand Disease at Seattle Children’s

Experts at our Cancer and Blood Disorders Center focus on diagnosing and treating blood disorders in children and teens, including von Willebrand disease.

Our goals are to achieve the best possible outcome for your child and to lessen how much the illness affects them.

We work with you, your child, your family and your child’s primary doctor to get your child the right care and services.

Please contact the center at 206-987-2106 for more information, a second opinion or to make an appointment.

  • Our doctors are nationally known for treating children who have blood disorders. The doctors who guide your child’s care are board certified in pediatric hematology-oncology. This means they are approved to give the specialized care your child needs and they constantly expand their knowledge about blood disorders.

    We offer a full range of services for children with von Willebrand disease, including medicine and clotting factors. In rare cases, a child may need blood transfusions. We also help your child prevent bleeding.

    We care for your whole child. We don’t just treat their disease. Our bleeding disorders team also includes nurses, social workers and physical therapists. Children with severe disease may also receive care from experts in nutrition, pain management, psychiatry and emotional health.

  • Our specialty is treating children’s conditions while helping them grow up to be healthy and productive. Children don’t react to illness, injury, pain and medicine in the same way as adults. They need – and deserve – care designed just for them.

    Our doctors have special training in how to diagnose and treat children with blood disorders. Our experts base their treatment plans on years of experience and the newest research on what works best – and most safely – for children.

  • A diagnosis of von Willebrand disease can be stressful. We help take positive steps right away by offering same-day appointments for children with urgent needs. New patients whose needs are not urgent usually can be seen within 1 or 2 weeks.

    During visits, we take time to explain your child’s condition. We help you fully understand your treatment options and make the choices that are right for your family.

    Our child life specialists and social workers help your child and your family through the challenges of this condition. We connect you to community resources and support groups.

    Read about how we can support you and your child with financial counseling, schooling, housing, transportation, interpreter services and spiritual care.

  • For the 27th year in a row, Seattle Children’s ranks among the nation's best children's hospitals, according to U.S. News & World Report.

    Our physician-scientists help set national standards for care of young people with blood disorders.

    Seattle Children’s provides the most advanced treatments in our region. Our experience helps us recommend the right treatment at the right time to have the best results for your child and your family.

Symptoms of Von Willebrand Disease

Signs and symptoms of von Willebrand disease can be mild or serious. It depends on which type of the disease your child has.

  • If your child’s disease is mild, you may not notice any sign of bleeding problems until a serious injury or surgery. Most children have a mild type.
  • If your child’s disease is severe, you will likely notice symptoms of bleeding problems early, even as a baby.

Symptoms may include:

  • Nosebleeds that happen often and last longer than normal
  • Easy bruising
  • Bleeding that lasts longer than normal, for example, after a cut, a tooth is pulled or surgery
  • For girls, heavy menstrual periods

Too much bleeding can be dangerous. But even if your child has a severe type of von Willebrand disease, treatment can help prevent serious problems.

Diagnosing Von Willebrand Disease

If your child seems to have a bleeding problem, your doctor will ask questions to find out about:

  • Bleeding that happens for no clear reason
  • Bleeding from a minor injury that lasts a long time
  • Bleeding that stops for a while but starts again

Your doctor will also check your child for other signs of bleeding problems and other health issues that can affect bleeding.

Your doctor will order blood tests to learn:

  • How much von Willebrand clotting factor your child has
  • Whether your child’s von Willebrand factor is normal and how well it works
  • How much factor VIII your child has
  • How many platelets your child has
  • How well the platelets are working
  • Sometimes it can be tricky to diagnose the disease, especially in mild cases. When a child is diagnosed with von Willebrand disease, we usually consider testing other family members who might have it. Testing parents may help us better understand a child’s risk of bleeding.

    We often refer parents to our partners at Bloodworks Northwest to be evaluated. Bloodworks also can talk to you about the risk of passing von Willebrand disease to children in future pregnancies.

Treating Von Willebrand Disease

Your child may not need any treatment for von Willebrand disease unless they have problems with bleeding too much.

If your child needs medicine or clotting factor into their vein (infusion), they can get care without having to spend a night in the hospital.

  • Our outpatient infusion unit is staffed by expert nurses and is also open on weekends.
  • Sometimes we teach families to mix and give infusions at home. This may be done with a butterfly needle or an intravenous (IV) line that we place before a child goes home.

Seattle Children’s offers these treatment options:

  • To prevent bleeding: A medicine called desmopressin (brand name Stimate) helps increase clotting. Doctors can give it to your child before any planned surgery or dental work. This medicine can be taken:

    • As a spray into your child’s nose when they need it
    • Through a vein (IV)

    Desmopressin does not work well for all children. Your doctor may give your child a test dose to see if it increases their levels of von Willebrand factor enough. We do not use it in children under 3 years old.

    To reduce bleeding: Medicines that help keep blood clots from breaking down are called antifibrinolytic (AN-te-fi-BRIN-o-LIT-ik). They are used mainly to reduce bleeding after:

    • An injury
    • Having a tooth pulled
    • Surgery
  • Your child may need a combination of von Willebrand factor and factor VIII made from donor blood. This is an option if your child needs major surgery and they do not respond well to desmopressin, a medicine that helps increase clotting.

    Clotting factors are given into a vein in your child’s arm.

  • If your child has a lot of bleeding because of von Willebrand disease, they may need a blood transfusion.

    Transfusing blood means giving red blood cells from a healthy donor. Your child receives the blood through a vein in their arm.

  • We talk with you and your child about ways to prevent excess bleeding. Your child may need to:

    • Avoid injury, such as not playing certain contact sports.
    • Reduce bleeding during menstruation. Girls who have very heavy menstrual periods because of von Willebrand disease may want to take birth control pills to decrease the buildup of the lining of the uterus. Some girls use the desmopressin nasal spray on the heaviest days of their period to decrease bleeding.
    • Avoid medicines that increase bleeding, such as aspirin and ibuprofen (Advil). Your child’s doctor can give you details about which medicines to avoid.

    If your child needs surgery or dental work, we create a treatment plan so it can be done safely.

Contact Us 

If you would like an appointment, ask your child’s primary care provider for a referral.

If you have a referral or would like a second opinion, call the Cancer and Blood Disorders Center at 206-987-2106.

Providers, see how to refer a patient.