Tell me about Von Willebrand Disease

October 4, 2008 5:45 PM

Have you been diagnosed with Von Willebrand disease? Or do you know someone who has?

I suspect I may have it, but I would like to hear from someone who has actually been diagnosed how severe the symptoms are. I bruise incredibly easily (always have), get inexplicable nosebleeds (again, always), have very heavy periods, and my dad is a hemophiliac.

Now, I have looked online at plenty of descriptions of the disease, so I am not really looking for links to web pages. I would like to hear from someone with personal experience:
-How frequently do you get nosebleeds?
-Bleeding gums?
-How easily do you bruise? Does the force from someone, say, tapping you on the shoulder leave a mark? Does it take more force, like a pinch?

Now, I know that if I really, really wanted to find out if I had the disease, I could ask a doctor, but from what I have read, this disease does not really require treatment. If you have it, do you disagree with that statement?

Thanks.

posted by waywardgirl to Health & Fitness (4 answers total)

I have always bruised very easily (a relatively light bump of my leg against a table, for example, would be enough to leave a mild bruise for several days; a harder bump would leave a bruise that could linger for weeks or more). My gums only bled when I was bad about seeing a dentist and flossing regularly; now that I'm good about both, they don't. I used to get nosebleeds relatively frequently, but in retrospect I think they were related more to my previous tendency to get sinus infections combined with living in a cold, dry environment during the winter. My periods aren't terribly heavy.

Then I had jaw surgery about 4 years ago, nearly bled to death, and ended up in the ICU with blood literally coming out my eyes. Luckily, I was in a great hospital with an excellent surgeon, and I have a common blood type so that the transfusions weren't a problem. Change any of those circumstances, and it could have been a different outcome.

Needless to say, once I'd recovered, I went to a hematologist. He took my history, and said that his first hunch was that I probably had Von Willebrand disease, but of course they'd do the lab work to determine for sure. As it turns out, I don't have Von Willebrand's, but rather mild hemophilia A and C (i.e., I'm deficient in factors VIII and XI).

There is no day-to-day treatment necessary; however, if I have surgery again, they'll know now to give me the appropriate clotting factor(s) ahead of time and to keep plasma on hand. They'll also be able to prepare similarly if I have a baby -- and, because clotting disorders are heritable, we'll also know ahead of time to test the baby. I keep a card in my wallet in case of an accident or trauma that may cause severe bleeding, so that paramedics, etc. would be able to help me appropriately, even if I was unconscious or in shock. There are also drug interaction issues to consider; I now avoid aspirin and ibuprofen as much as possible (they're blood thinners), and my doctors know to avoid prescribing me anything that would also thin my blood (which is particularly relevant for me, as I also have a heart condition).

All of which is a long way of saying that if you suspect you have a bleeding disorder of any kind, you need to consult a doctor and request the labwork to get it ruled in or out -- especially since you have a known family history of it to begin with. Because even if you have something that doesn't require day-to-day treatment, what happened to me is proof that even a mild bleeding disorder can still threaten your life quite suddenly.
posted by scody at 6:54 PM on October 4, 2008 [1 favorite]

No it does not require treatment but if you have it you need to know. It testing is very simple and requires only a relatively small amount of blood to be collected. A couple of the test can be rather costly but you could be saved lots of problems with future dental and surgical procedures. Like scody above I have seen more than one person wind up in ICU being given massive transfusions because they had an unknown bleeding problem.
posted by bjgeiger at 7:13 PM on October 4, 2008

If your father truly has hemophilia, then you are a carrier (it is an X-linked recessive condition). Both Hemophilia A (Factor VIII) and B (Factor IX) are inherited in this manner. Perhaps the most important thing is for you to know that you are a carrier and that you can have affected children (25% risk per pregnancy).

That said, female carriers (by definition, carriers are female) can rarely have excessive bleeding. If you also had another heritable condition that predisposes you to have bleeding tendency, you would certainly be at an increased risk for bleeding (eg. von Willebrand Disease).

But then, this is the internet and not your doctor. This is all easily sorted out by a physician. With your family history and your own personal history, a visit to a Hematology specialist should be in order. These conditions are relatively easy to sort out with laboratory/coagulation studies (how I make a living). Your hematology doc will certainly know the proper ones to order.
posted by i_am_a_Jedi at 7:36 PM on October 4, 2008

It doesn't require treatment, but it's a good thing to know -- especially should you ever need surgery, or give birth to a child. (scody explained why, pretty much -- so they can make sure you don't bleed to death.)

I've been, uh, sort of diagnosed. My sister has a positive diagnosis, and considering how easily I bruise, it's almost certain I have it. The test is quite simple and your PCP can do it -- worth the time and cash, certainly.
posted by kalimac at 9:24 AM on October 5, 2008