What is Von Willebrand Disease?

Von Willebrand disease (VWD) is a bleeding disorder that is passed down from parent to child through information in the cells called genes.

Von Willebrand disease is caused by not having enough von Willebrand factor (VWF) or having VWF that does not work properly. Von Willebrand factor is a protein that helps the blood form clots to stop bleeding.

Von Willebrand disease affects both males and females. It is the most common inherited bleeding disorder.

How von Willebrand Disease Causes Bleeding

Von Willebrand factor helps sticky cells in our blood, called platelets, stick to areas of injury and form an initial plug (clot) to stop bleeding.

It also carries clotting factor VIII (8), another protein that helps in clotting, to the area of injury and protects factor VIII in the blood so it does not get broken down too quickly.

Without von Willebrand factor, people can bleed due to platelet plugs not working well and bleeding from low factor VIII levels.

Not all patients with von Willebrand disease will have low factor VIII levels.

Von Willebrand Disease Diagnosis

Von Willebrand disease is diagnosed by blood tests that check the level of von Willebrand factor in your blood and how it is working. Repeat blood testing may be needed because levels can be affected by illness or other things that stress the body. Blood tests can tell your doctors what type of von Willebrand disease you have.

Von Willebrand Disease Types

• Type 1: The most common type. There is not enough von Willebrand factor in the bloodstream.
• Type 2: The next most common type. There may be enough von Willebrand factor, but it does not work correctly. Von Willebrand factor is made of long strands called multimers. In Type 2 patients, there may be a problem with the multimers working correctly.
• Type 2A: Does not form the correct multimers and does not stick to the platelets.
• Type 2B: Multimers stick too tightly to the platelets, which causes the body to get rid of the platelets with the factor. This results in a low level of platelets and von Willebrand factor that doesn't work correctly.
• Type 2M: Multimers are normal, but von Willebrand factor is not able to stick to the platelets.
• Type 2N: Von Willebrand factor cannot carry factor VIII normally and this causes low factor VIII levels.
• Type 3: The rarest form of von Willebrand disease. This type is the most severe form of the disease with little to no von Willebrand factor and low factor VIII levels.

Von Willebrand Disease Symptoms

The three main symptoms that are characteristic of von Willebrand disease include bruising, nosebleeds and bleeding more than normal or more than other people do. Some details about when bruising and bleeding may be problematic are:

Bruising

• Easy bruising (bruising without a known injury).
• Bruises that get bigger days after the injury.
• Bruises that do not heal or fade within 7-10 days.
• Bruises that are painful and decrease the use of the area (meaning the arm or leg with the bruise cannot be moved easily).

Nosebleeds

• Frequent nosebleeds (having several nosebleeds in a week, having one nosebleed every month, and/or having a nosebleed that stops, but starts up again).
• Nosebleeds that start without an injury.
• Nosebleeds that last longer than 20 minutes.

Bleeding

• Bleeding after dental work.
• Bleeding of the gums.
• Bleeding after a surgery or procedure (not expected based on the surgery).
• Heavy menstrual periods.
• Periods that last longer than seven days.
• Irregular periods.
• Changing a pad or tampon more often than every one to two hours.
• Passing clots larger than a quarter.
• Periods that cause iron deficiency anemia, or a need for a blood transfusion.
• Periods that cause missed days of school or work due to bleeding.
• Bleeding in the joints or muscles (this is less common).
• Family history of a bleeding disorder.

Von Willebrand Disease Treatment

Most people with von Willebrand disease will not need regular treatment. The goal of treatment is to make levels of von Willebrand factor in the blood normal at times of bleeding or prior to a surgical or dental procedure. Treatment is based on the type and severity of the disease, and includes:

DDAVP (Desmopressin acetate)

DDAVP is a medication given by vein or inhaled through the nose in spray form. It causes the body to release stored VWF and factor VIII. DDAVP cannot be used for all types of VWD, and children will need to be tested with DDAVP in our clinic before regular use.

Clotting Factor Concentrate

This is medicine that contains VWF with or without factor VIII. It can only be given through an IV.

Types of Factor Concentrate:

• One type of factor is made from plasma from healthy blood donors. Plasma is tested for safety, but it could contain viruses. It contains VWF and factor VIII.
• Another type of factor (recombinant factor) is made in a laboratory, not from human blood. There is no risk of infection. It only contains VWF.

Antifibrinolytics (aminocaproic acid, tranexamic acid)

Antifibrinolytics prevent clots from breaking down too quickly. They do not increase the level of VWF in the blood. Antifibrinolytics are available in liquid or pill form or IV, and can be used to treat:

• Nosebleeds
• Mouth bleeds
• Heavy menstrual period bleeding
• Bleeding after dental procedures

Nasal saline spray or gel can help keep the nose moist to prevent nose bleeds.

Hormone medicines or intrauterine devices (IUDs) can be used to decrease the amount of menstrual period bleeding.

Von Willebrand Disease Prognosis

Children with von Willebrand disease have good long-term outcomes. With excellent care and proper treatment, most people with von Willebrand disease can lead normal, healthy lives. Children with von Willebrand disease should remain physically active. Most children with VWD can participate in most sports and activities. They should avoid high-contact sports like football, and talk about physical activity restrictions with their doctor. People with von Willebrand disease should not use nonsteroidal anti-inflammatory drugs (NSAIDs) like ibuprofen, naproxen and aspirin. These drugs can make you bleed more.

Let all your child's healthcare providers, including their dentist, know that your child has von Willebrand disease. This will help them to plan if your child must have surgery or a procedure.

When to Call Your Doctor:

• If you are having bleeding concerns
• At least two weeks before any surgical or dental procedures
• Prior to travel if you have required treatment for your von Willebrand disease in the past