December 9, 2011

What's von Willebrand Disease?

Okay, this is the question everyone wants to know the answer to, as far as I can tell.

This is a problem with one of the 13 factors that cause normal clotting of the blood (with injury: i.e. cuts, scrapes, etc.) There's a protein called von Willebrand factor (vWF) that is either deficient or dysfunctional that causes the disease. This means that the platelets in the blood don't attach to the vessel wall the way they should and so clotting isn't as quick as it should be.

RJ is lucky enough to have a mild form of this disease, and it really shouldn't cause problems for him in day to day life. He may bleed a little longer than most people when he has an injury, but not excessively so. He will just have to make sure that his doctors are aware of the condition before any scheduled surgery, and he needs to notify his hematologist before surgery, after trauma, and after an injury. There's a nasal spray medication that they will be trying with him to see if it makes a difference.

The disease is hereditary, but we don't know from where it was inherited. From what I read on Medscape, it looks like he has Type I, which is the mildest of the 3 main forms. The disease affects about 125 people per million (So RJ's one of 125 in a million). There are also some reports that bleeding tendency decreases with age. Since this is a bleeding disorder, aspirin and ibuprofen shouldn't be used.

All in all, his case is mild and not enough to cause him to change his normal activities. We will simply have to be very aware of seemingly mild trauma (bumped head) and he should keep track of headaches for severity and frequency from now on, just to be safe. Easy enough to manage, right?

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