Saturday, October 23, 2004

von Wille--wha?

Learn along with me about this most common of bleeding disorders called von Willebrand Disease. Yeah, I'd never heard of it before, either. Then my DD was diagnosed with it.

Murphy's Law, I believe it's called.

Due to a change in my husband's employment, our health insurance was ending June 30th of this year. No big deal, we thought; we'd secure some basic coverage for the kids and even more basic for us. We are, thankfully, almost never ever sick, and have no medical issues, so we weren't panicked.

On July 1st of this year, I got a call from our pediatrician. Seems the blood test they performed at Rainbow's 2nd Year Check-Up came back with a little surprise: she tested positive for von Willebrand Disease.

"Mellie Helen, you've just run out of health insurance. Now what're you gonna do?"
"I'm gonna get my daughter a life-threatening disease!"


von Willebrand Disease is a genetic disorder (really it is a disorder, but was named "Disease" in the 20s, so who's gonna change the name after 80 years?) wherein the platelets of one's blood decide to do an Anti-Velcro act. When you get a cut, your little platelets are supposed to rush to the scene to bond together, hugging and embracing one another just long enough to let other cells form what's called a fibrin clot, thereby allowing your body to make one of those attractive natural Band-Aids called "scabs". BUT -- if those first platelets fail to bond together, then the fibrin clot fails, and the entire thing just falls apart. Try painting with watercolors on a slick plastic surface without first priming it properly, and you'll see the problem here.

The reason those platelet party poopers may not be clinging to each other for dear life (literally!) could be due to a low "factor" count (means there's not enough von Willebrand factor in the blood to do the job), or it could be that there's plenty of factor, but they don't behave properly (perhaps they were never disciplined properly in vitro). Whatever the cause, the result is more bleeding than you really want to experience.

Rainbow, thankfully, has the mildest form of this disorder, which means she will live a mostly normal life, punctuated with sudden nosebleeds, and tinted with colorful bruises all over her body, created by anybody gently exhaling in her general direction. The hemotology center has provided me with a letter addressed "To Whom It May Concern" explaining the ramifications of her disorder, so that her Sunday School teachers, playgroup leaders, etc., won't be misled to think that we use Rainbow as a bocci ball and consequently report us to Child Services on a weekly basis. She will occasionally require infusions of medicine (which means DH and I get to learn how to prepare and give an I.V. -- Golly Bob Howdy!). But for the most part, she'll get to have a Regular Kid life.

Since it's genetic, and nobody on either side of the family seemed to have any issues with bleeding disordered whatsoever, I had myself tested, and discovered that -- WHO KNEW? -- I have this disorder too! My factor count is only 1% higher than Rainbow's, and both of us are WAY below the "normal" level. Yet for some reason, my activity is "normal". That is to say, although like my daughter, I too have a very low amount of factor, the amount I have does the job of a normal person's factor. Which is why I've gone 42 years blissfully unaware that I am diseased. (Or is that "disordered"? Naw, that can't be it; I have in fact known that I've been disordered for years now...)

Merlin and the Astronaut-in-Training have been tested: Merlin is well within the "normal" range, but Astro has the lowest possible numbers to still be considered within the normal range, and that could likely mean a false negative for him. He prolly should be retested, but since he has no symptoms, we're holding off doing that again until we have to (e.g., before any scheduled surgery).

What this all means is that the disorder was passsed down through me. But how did I get it?

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