Von Willebrand disease

Our pediatric hematologists diagnose and treat many children with von Willebrand disease, a bleeding disorder. We have the experience to care for children who are experiencing a range of von Willebrand disease symptoms, from mild to complex.

At Advocate Children’s Hospital, our care team brings treatment and support together in a child-friendly environment. We help you manage symptoms at home and support your child with advice and strategies as they move into adulthood.

About von Willebrand disease

Childhood von Willebrand disease is caused by inherited changes, or mutations, to the VWF gene. These genetic changes affect a protein called von Willebrand factor, which helps blood clots to form and stop bleeding after an injury. Some of the changes lead to lower levels of the protein, while others stop it from working well.

Von Willebrand disease is the most common bleeding disorder although it may not be diagnosed in people who have very mild symptoms.

Von Willebrand disease symptoms

Von Willebrand disease symptoms range from being very mild to being so severe that they are life-threatening. The most common von Willebrand disease symptoms include:

• Easy or excessive bruising
• Frequent nosebleeds
• Heavy menstrual bleeding
• Long-lasting bleeding after surgery, dental work, small injuries or childbirth
• Blood in the stool or urine

Types of Von Willebrand disease

There are three types of von Willebrand disease:

• Type 1: This form is relatively mild. Children with type 1 disease have up to half as much von Willebrand factor in their blood.
• Type 2: Children with this type of von Willebrand disease have problems with the way the protein is structured or how it works. They usually have mild to moderate symptoms.
• Type 3: In this severe form of disease, children have almost no useful von Willebrand factor in their blood. Your child may develop spontaneous bleeding with no injury involved.

Von Willebrand disease diagnosis

Sometimes doctors have difficulty identifying von Willebrand disease, especially if a child’s von Willebrand disease symptoms are mild. Our doctors diagnose and treat many children, giving us the experience to help get an accurate diagnosis as quickly as possible.

If von Willebrand disease runs in your family, our doctors might recommend evaluating your child before symptoms even arise. Doctors may need to do multiple tests because von Willebrand factor levels can rise and fall.

Diagnosis involves various blood tests that help doctors measure the amount of von Willebrand factor and how it’s working. They include:

• Antigen test: Measures the level of von Willebrand factor in the blood.
• Von Willebrand factor activity tests: Shows how well the von Willebrand factor is functioning.
• Factor VIII activity test: Measures factor VIII, a blood protein that interacts with von Willebrand factor to help clotting.
• Multimers test: Provides information on how much von Willebrand factor is in the blood and its structure.
• Platelet function tests: Looks at how well platelets are working in the blood.

Von Willebrand disease is often complex. Our doctors will get you the right diagnosis and the latest treatments to manage von Willebrand disease symptoms.

Von Willebrand disease treatments

Our doctors work with your family to determine the best treatment plan for your child’s von Willebrand disease symptoms. Treatment focuses on two major areas: increasing the amount of von Willebrand factor in your child’s blood and preventing heavy bleeding.

Some common treatments for von Willebrand are:

• Desmopressin: This hormone helps the body increase von Willebrand factor. Your child’s doctor may give your child a shot (injection) or a nasal spray.
• Medications: Doctors may recommend drugs that keep clots from breaking down or slowing the breakdown.
• Fibrin glue: Doctors typically use this sealant if a child has a wound that won’t stop bleeding. Surgeons and dentists also sometimes use it for people with von Willebrand disease, since it can help stop bleeding during procedures.
• Birth control or IUD: Girls may need treatment for heavy menstrual bleeding, including birth control pills or an IUD that includes hormones.

How to handle common situations

Our care team helps you learn how to handle everyday situations that may arise with von Willebrand disease. But call our doctors or go to the emergency room if you can’t figure out why your child is bleeding. Also do so if your child begins bleeding excessively.

Many routine situations require extra planning when your child is diagnosed with or suspected to have a bleeding disorder. Some common situations are:

• Circumcision: If there is a family history of von Willebrand disease, talk to your pediatrician before doing a circumcision.
• Aspirin or NSAIDs: Your child should take acetaminophen for pain and fever rather than nonsteroidal anti-inflammatory drugs such as aspirin or ibuprofen.
• Bleeding: If your child has a mild wound, put pressure on the area and hold it until the bleeding stops.
• Nosebleeds: These are a common part of life when dealing with a bleeding disorder. Ask your child to lean forward and pinch the soft part of their nose until bleeding stops.
• Dental work: Let your child’s dentist know about the diagnosis before having any work done. The dentist will take special precautions and may prescribe medication before a procedure.
• Heavy periods: Some girls have menstrual periods that are extremely heavy or last a long time. If so, you can schedule an appointment with an adolescent medicine doctor or a gynecologist who can help manage these problems.

Transitioning into adulthood with von Willebrand disease

Early preparation is a great way to set your child up for successful management of von Willebrand disease symptoms in adulthood. This preparation includes learning how to manage their treatment plan, their medical supplies and even potential insurance issues.

Our care team helps your child define their goals. We also help them prevent complications on their own and manage urgent needs, especially if they plan to move away from home.