Von Willebrand Disease

Von Willebrand disease (VWD) is a blood 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.

VWD affects both males and females. It is the most common inherited bleeding disorder. 

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

VWF also carries clotting factor VIII, another protein that helps in clotting, to the area of injury.

VWF also protects factor VIII in the blood so it does not get broken down too quickly.

Without VWF, people can have bleeding due to platelet plugs not working well and bleeding from low factor VIII levels.

Not all patients with VWD will have low factor VIII levels.

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

Type 1: The most common type of VWD. There is not enough VWF in the bloodstream.

Type 2: The next most common type of VWD. There may be enough VWF, but it does not work correctly. VWF is made of long strands called multimers. In type 2 VWD, there may be a problem with the multimers working correctly.

Type 2A: VWF does not form the correct multimers and does not stick to the platelets.

Type 2B: VWF multimers stick too tightly to the platelets, which causes the body to get rid of the platelets with the VWF. This results in a low level of platelets and VWF that doesn’t work correctly.

Type 2M: VWF multimers are normal, but VWF is not able to stick to the platelets.

Type 2N: VWF cannot carry factor VIII normally and this causes low factor VIII levels.

Type 3: The rarest form of VWD. There is little or no VWF in the blood. This type is the most severe form of the disease with little to no VWF and low factor VIII levels.

  • Easy bruising
  • Frequent nose bleeds
  • Bleeding after dental work
  • Bleeding following a surgery or procedure
  • Heavy menstrual periods
  • Less common is bleeding in the joints or muscles

Most people with VWD will not need regular treatment. The goal of treatment for VWD is to make levels of VWD in the blood normal at times of bleeding or prior to surgical or dental procedures. Treatment is based on the type and severity of the disease.

DDAVP

  • This medication is given by vein or inhaled through the nose in spray form.
  • DDAVP causes the body to release stored von Willebrand factor and factor VIII.
  • DDAVP is not a blood product.
  • It cannot be used for all types of VWD.

Children will need to be tested with DDAVP in our clinic before regular use.

Clotting Factor Concentrate

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

Types of Factor Concentrate:

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

Antifibrinolytics

(aminocaproic acid, tranexamic acid)

  • Prevent the clot that is formed from breaking down too quickly
  • Do not increase the level of VWF in the blood
  • Available in liquid or pill form or IV
  • Can be used to treat:
    • Nosebleeds
    • Mouth bleeds
    • Heavy menstrual period bleeding
    • After dental procedures

Nasal Saline Spray or Gel

This helps keep the nose moist to prevent nose bleeds.

Hormone Medicines or Intrauterine Devices (IUDs)

These are used to decrease the amount of menstrual period bleeding.

Keep your provider’s phone number handy in case of emergencies.

Visit your doctor for a regular yearly check-up.

Remain physically active. Most kids with VWD can participate in most sports and activities. Try to avoid high contact sports like football.

Avoid using nonsteroidal anti-inflammatory drugs (NSAIDs) like ibuprofen, naproxen and aspirin. These drugs can make you bleed more.

  • You are having bleeding concerns
  • At least two weeks before any surgical or dental procedure
  • Prior to travel if you have required treatment for your VWD in the past

Last Updated 02/2020

Reviewed by Lisa Littner, Hematology Education Specialist

Who treats this.

Learn about how the Bleeding Disorders Evaluation Clinic can help your child.