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?

CT Images

This image shows the blood clot that was in RJ's brain (circled in yellow) and the shift that was occurring, pushing the left side (shown on the right here) of his brain over, and beginning to push the right side over as well.The red line is where the midline should be, the curved white line to the left is the midline of the brain (the division between the two halves of the brain) The clot measured 10 cm (4 in.) by 6 cm (2.25 in.) and went in about 5 cm (2 in) at this point, which is about the deepest spot.         

This image was taken right after surgery, and shows the dog-bone-shaped brackets holding in place the section of skull that had to be removed in order to remove the clot. There are 32 staples (the track of parallel white lines) and the yellow line is the drain tube that went into his skull to create negative pressure in the cavity left by the removal of the clot. This essentially "sucked" his brain back over into that space while also draining the fluids that were accumulating from the surgery. The tube was removed the following morning, the staples 10 days later, and he gets to keep the brackets & screws forever.

One month and 10 days later...

RJ went for his day of follow-up appointments today, starting at 9:30 with a blood draw. We followed that with a visit to the Hematologist, who apparently scheduled the appointment with me, but forgot to put it into their computers. Not a problem, we were supposed to see them at 10 and got in at around 10:30 or so instead. Everything there was good, there weren't any results back from the blood work yet but there was nothing from the previous tests to raise concerns. RJ hasn't had any post-operative bleeding or bruising issues, and has been feeling great so that appointment was quick and easy. We finished there at 11 and the CT scan was scheduled for 1:15, so we headed down to the lobby to get drinks (no food for RJ yet).

We were having drinks when their seating area outside the cafe started filling up, so we decided to head down to radiology early. We got down there and registered, and they had a slow spot so RJ got his scan done early and we got disks with the scans :) Since we were out of there before 1 and the Neurosurgeon appointment wasn't until 2:15, we went to get something to eat (thinking we were stuck with McDonald's), and the ad for SmashBurger caught RJ's eye in the elevator. Guess where we went for lunch.... Not McDonald's, that's for sure!

After that we headed to the Neurosurgeon's office and watched part of "Horton Hears a Who" in the lobby while waiting. That appointment also went very well, there is almost no evidence left of the clot removal aside from the titanium brackets & screws, and a little tiny space (comparatively) left from the surgery. The space is about the size of the tip of the pinky, from the last knuckle to the tip, according to the doctor. He will need to go for another CT and follow-up visit 3 months from now, but otherwise everything looks good. He can drive with an adult after he's finished tapering off the Keppra, and then on his own a couple weeks after that as long as he doesn't have any problems. We will need to keep watching for seizures but there's been none so far.

When we were leaving there the Hematologist called with the results of 2 of the 3 blood tests drawn this morning, the Factor 11 test is negative, but the Von Willebrand's showed the same numbers as in the hospital, meaning he does have a mild form of this disease.


I'll post about that later, for now I have to go get RJ so we can go to college :) He managed to finish the first trimester with a 4.4 GPA, even being gone and having brain surgery :) Way to go RJ!!