Hemophilia 101.1

The boys have been self-infusing (infusing their medicine into their veins all by themselves) for a long time. #1 is 11 and #2 is 9. They've been self-infusing since ages 8 & 7 respectively. It's a fairly complex process since they have to reconstitute the lyophilized (basically, freeze-dried) recombinant Factor 9 so that it can be infused in a vein. They mix/prepare the product for infusion, select a vein on their bodies that looks good for poking, poke the vein with a butterfly needle and slowly inject the factor into their bloodstream. Then they withdraw the needle, hold pressure on the infusion site and clean up. Cleaning up means disposing of the items properly. We have a sharps container (about 10 times bigger than that picture) for any bio-hazardous waste and pitch the other stuff in the garbage. We also must keep an infusion record (data log) of each infusion, when we did it, why we did it, what lot # of medicine we used and the # of units that were infused.

This infusion log is vital. Should a problem arise with the medicine they are using, we would be alerted of a recall. The only way of knowing if we are affected by the recall is if we have maintained sterling infusion logs. Many of the men and women who were infected with HIV and Hepatitis through tainted plasma-derived (made from human plasma)factor were not able to trace their infection to specific contaminated lots of factor. This, combined with the blood shield laws that treat a blood or tissue product differently than other drugs (for liability and negligence purposes), made it impossible individuals to sue over blood products that were knowingly tainted and distributed.

It's simply amazing to me that I can tell the boys "you have to do a poke" and they can trot...or scramble...upstairs and do it on their own! Sometimes they infuse while I'm cooking supper and we talk and chat through the whole thing. Sometimes they head upstairs and fight with each other the whole time: "That's MY tourniquet"..."Give me that saline"..."quit moving the sharps away from me"....

This was a process that, at the very beginning, involved calling our Hemophilia Treatment Center and asking if what had just happened (the injury) warranted an infusion. Then I'd call the Emergency Room at the hospital and tell them who I was and that I was on my way with my kid with hemophilia. (People with hemophilia SHOULD NEVER WAIT IN A WAITING ROOM...just because you don't see blood gushing out of an open wound does NOT mean that blood is not slowly oozing into the cranium, spine or knee joint.) Then we'd get to the ER, check in and head into a room. Where, inevitably, a new doctor would show up who would have to ask 100 questions while I assertively started mixing the factor...when he finally decided that we should infuse (after I'd told him, and the nurses had told him) we'd have to wait to decide which vein would be used to infuse the medicine.

And let me tell you, chubby little 9 month olds don't have good veins. Except right on their scalps. So I'd tell them to use a scalp vein. And they'd balk. And I'd say o.k., try an elbow. And it wouldn't work. Then I would INSIST that they use the scalp vein. And the doctor would tell ME, then, to restrain my child while they stabbed him. Um no. Go get a couple more people. I do the whispering in the ear and the consoling...but no restraining.

That lasted for about 12 months (birth-12 months) for each kid. Then we had ports inserted into their bodies and we were able to infuse them at home. Which was FUCKING AWESOME!!!

But port infusions aren't easy. Sterile technique, heparin flushes, betadine, sterile fields....a whole slew of things to make that work and keep the boys safe.

Now? It's a 10 minute procedure. And I can send them upstairs to do it themselves. They are in charge of their own care. We help, we oversee, we encourage...but they do it.

It's awesome. And I'm so proud of them!