Is your kid a bleeder? It might be Von Willebrands disease. Von Willebrands is the most common bleeding disorder and yet very few people actually know about it. It is estimated that 1% of the population has it. (In medicine, that’s a huge number; that means 1 or 2 people in your neighborhood have it). As is the case with many disease names in medicine, this one is named for the doctor who discovered the condition, Dr. Erik von Willebrand. As an aside, if I ever discover a disease, I’m definitely not naming it after myself. I don’t ever want to be associated with a disease, the cure perhaps.

What is von Willebrand disease?

In a nutshell, von Willebrand disease (vWD) is an inherited bleeding disorder where the affected person has excessive bleeding. There is a factor in the blood called von Willebrand factor (hence the name) that sticks to platelets and helps in the clotting of blood. So if you are missing the factor (or don’t have enough or it doesn’t work properly), then your blood won’t clot well and you’ll be a bleeder. Do not get this confused with Hemophilia (another bleeding disorder); they are different.

Technically speaking, there are 3 major categories of von Willebrand disease, cleverly named Type 1, Type 2, and Type 3. Type 1 is a partial deficiency of the von Willebrand factor and accounts for 75% of the cases out there. Gratefully, it is the most mild of the types. Type 2 is a functional defect (normal levels, but they don’t work right) and type 3 is a complete deficiency in the von Willebrand factor.

Signs and Symptoms:

While all kids get scrapes, cuts, bruises, and the occasional bloody nose, kids with von Willebrand disease get it significantly worse. Common signs include:

1. Prolonged or frequent bloody noses (not the 5 minute bloody nose 1-2 times a year, I’m talking the 10+ mins, 5+ times a year)
2. Lots of bruises (often unexplained or in unusual locations)
3. Prolonged or excessive bleeding during or after surgical procedures (e.g., circumcision, teeth being pulled, tonsillectomy, etc.)
4. Simple cuts or scrapes that seem to bleed or ooze forever
5. Heavy, long periods (the super plus pad type, that last 7+ days)
6. Easy bleeding with minor trauma (like brushing teeth or flossing)

Who gets it?

Von Willebrands is an equal opportunity disease. Boys and girls get it the same. (Unlike hemophilia which affects only boys). You don’t catch or develop von Willebrands, you’re born with it (it’s inherited from the get-go).

What’s the risk of passing it on?

If your child has von Willebrand disease, then mom or dad likely also have the disease. In the genetic world, it is considered autosomal dominant (meaning there is a 50% chance of passing it on to your child if one parent has it). In complete disclosure, it is a little more technical than that because of the different types (type 1 and 2 can come from one parent, but type 3 needs usually requires inheritance from both parents). This strong genetic link is part of what makes it so prevalent.

How is it diagnosed?

Unfortunately, the only way to officially diagnose von Willebrand disease is through a blood test. There are various blood antigen and activity tests that your doctor will know about. The trick is that the levels aren’t always the same (they can temporarily rise and fall). So sometimes the tests will have to be repeated to get an actual diagnosis.

How is it treated?

1. Prevent bleeds (as much as possible)
2. Learn to properly stop bleeding when it occurs (e.g., apply firm, prolonged pressure to cuts, nosebleeds, etc.)
3. Inform doctors/surgeons before any surgery or procedure. Sometimes doctors will have to use a medication called desmopressin (DDAVP) which will temporarily cause a rise in the von Willebrand factor (and factor VIII) levels to help a patient be able to clot better during a surgery.
4. Avoid non-steriodal antiflammatory medications (like ibuprofen/Motrin) because they inhibit platelet function which can increase bleeding risk.

My kid was just diagnosed, how stressed should I be?

For most people (the 75% who have type 1), a diagnosis of von Willebrand disease is not that bad. It will not change your child’s expected life span and for the most part, will not dramatically change his/her quality of life. It is worth knowing, so you can let doctors know before your child undergoes any procedures or surgeries. It will also help you to not freak out when you have a bloody situation that takes a while to get to stop. Otherwise, in terms of diseases- it’s totally doable. Out of your own curiosity, you may want to test you and dad. See who is too blame (insert sarcasm here).