River has Type 1 von Willebrand Disease (vWD)
What is vWD?
VonWillebrand Disease is a blood disorder that prevents the blood from clotting properly. It is similar to hemophilia, except instead of being short on platelets (the cells that form a blood clots), he is short on the proteins that hold those platelets in place, which is known as the von Willebrand factor. In simpler terms, when injured, his body tries to form blood clots, but they don't stick in place.
What are the implications?
River can lead a normal, everyday life. The disorder shows up most frequently as bruising, especially during the toddler years. His vWD is not a real concern unless he sustains a significant injury or needs to have surgery. In that case, he has to have an infusion to provide his blood with extra factor.
How did we discover his vWD?
Just before River's first birthday, I realized that he had had a number of strange bleeding issues: blood in his stools, a persistent bloody nose, small scratches continuing to bleed for days, and blood from his urethra.
None of these were particularly alarming on their own, but the combination of them prompted me to ask the pediatrician to run some labwork and find out if the seemingly random bleeding was more than coincidental. It was. The initial labwork showed that he had a deficiency of the vonWillebrand factor. She referred us to the hematologist at Arkansas Children's Hospital, Dr. Stine, and a repeat blood test from their clinic confirmed the results. He has Type 1 vWD, which is the least severe. For both blood tests, the results took approximately 2 weeks to come in.
What has this meant for our everyday?
Even though we have not yet needed to take him to the ER for an infusion, the medication he will need in that scenario is not readily available at most ERs or pharmacies. His hematologist set us up with a home healthcare service, which is keeping us supplied with the medication for that someday in the future when we'll need it. We have 2 doses of Alphanate in our refrigerator, and we take them along with us when we are going out of town, even for day trips. We also have another medication called Amicar, which is an oral medication used to stop mouthbleeds from teething or split lips, bit tongues, etc... Both medications have a shelf life good for the next year, but the woman at Caremark assured me that we will most likely use both doses before they expire. That's not so reassuring.
River also wears a medical i.d. bracelet to alert other to his condition in case of emergency, and we keep a notification card near his carseat in our car.
It's tempting to try and keep him in a bubble, but it doesn't matter how careful we are, injuries will happen. That is a part of life. The irony is that the more active we allow him to be, the less likely he is to injure himself.
Of course, he won't be able to play any contact sports, although, some could argue that life with Liam is a contact sport, and he won't be able to join the military or donate blood. But other than that, he can lead a normal life. He'll just have more bumps and bruises to show along the way.
You can see all of my vWD - related posts here.
