What does von Willebrand (VWF) factor do in our blood?

  • VWF has two main functions:
    • VWF helps sticky cells in our blood called platelets stick to areas of injury and form an initial plug to stop bleeding
    • VWF also carries clotting factor VIII (8) to bring it to areas of injury and protects factor VIII in the blood so factor VIII does not get broken down too quickly
  • With low VWF, people can have more bleeding and bruising because platelet plugs don’t work like they are supposed to

Facts about low VWF:

  • It is a category for people with somewhat low VWF levels (between 30 and 50%) who have increased bleeding and bruising symptoms
  • VWF can be low because there is a problem with how it is made and released from cells or may be related to blood type
  • Though there is less VWF (low), VWF works like it is supposed to
  • Low VWF isn’t always genetic (passed down from parent to child through information in the cells called the VWF gene)
  • Both males and females can have low VWF
  • Having low VWF is different than having von Willebrand Disease
  • It is more common and usually milder than von Willebrand Disease (VWD)

Symptoms of Low von Willebrand Factor

  • Easy bruising
  • Frequent nose bleeds
  • Bleeding after dental work
  • Bleeding following a surgery or procedure
  • Heavy menstrual periods
  • Some people have lower levels of VWF but do not have bleeding symptoms
  • Sometimes symptoms of low VWF are so mild that it might be hard to tell if your child has it

Diagnosis of Low von Willebrand Factor

  • There is no single test for diagnosing low VWF
  • Your child’s doctor will order several blood tests to test the level of VWF and test how VWF works
  • Blood tests will be done to look at the VWF multimers (strands of VWF) and check factor VIII levels to make sure your child doesn’t have VWD
  • Low VWF can be difficult to diagnosis and sometimes your child may need to have repeat blood tests in order to get a clear answer

Treatment of low Von Willebrand Factor

  • Most people with low VWF will not need regular treatment
  • The goal of treatment for low VWF is to make the levels of VWF in the blood normal at times of bleeding or before surgical or dental procedures
  • Treatment is based on the type and severity of bleeding or the planned surgery
DDAVP
  • A medication 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
  • Children will need to be tested with it 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 a vein

Types of Factor Concentrate:

Made from plasma

  • Factor made from plasma from healthy blood donors
  • Factor made from 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

Other treatments:

Antifibrinolytics (aminocaproic acid, tranexamic acid)

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

Nasal saline spray or gel: to help 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

Special Instructions

  • 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.

Call Your Doctor If:

  • 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 low VWF in the past