A Boxing Match On A High Wire

Testdriver here, posting from afar.

Gracie's Mom, Noodles, Noni and Muntz are all holding it together in Minneapolis and I'm here in Philly, a million miles away, with my two healthy, but likely plague-ridden babies, who are not allowed to visit immune compromised people on a BMT unit, and would probably not mix well with lots of tubes and cords and things that beep around, anyway.

From my phone updates, I can hear us collectively holding our breath. We're waiting, waiting, waiting for cell counts to creep up, waiting for fluid balances to normalize, waiting for meds on drips to be titrated ever-closer to Gracie's exact need for them. Gracie just got knocked down by the medical blow of a lifetime, and we're all holding our breath, watching her try to get her feet back under her, watching her reel and stagger, and imagining how her head must be spinning.

The balancing act that is maintaining Gracie's body's stability right now is really something to behold, a high-wire act like nothing you can believe.

It starts with a side effect of chemotherapy--immune suppression--served as the main course, in order to erase her body's ability to make blood and other fast growing cells, but that means wiping out ALL KINDS of fast growing cells (the reason chemo was originally developed and used in cancer patients--kills those fast-growing tumor cells) like the lining of the mouth--and the rest of the GI tract, the hair, the lining of the urinary tract, various organ tissues, everything.

So now that those cells are also compromised, there are new problems. Mouth sores are one very painful problem--mediated along with other painful problems--by narcotic pain medications. Narcotics, particularly in people as small as Gracie (easy to overdose a little baby) have to be carefully, carefully titrated, to avoid their own cascade of side effects, the most dangerous of which is respiratory depression. It's a delicate balance. She's gotta breathe.

It's also important to protect that urinary tract. If you can't make urine, you're pretty much sunk, so keeping the chemo from compromising her urinary tract function is extremely important. How to do it? Get that stuff through her body and out as fast as you can. To dilute it, she has to have LOTS of fluids through her IV, and then she gets loop diuretics to help her punch-drunk kidneys move all that fluid out. Sounds sensible and simple, but it's another crazy balancing act. If the diuretics are too weak, and she gets more fluid than her kidneys can manage, the extra fluid will back up in her blood stream and leak into her lungs (the "wet lung" we keep hearing about) impairing her breathing again. Too much diuretic and there is danger of her losing important electrolytes (like potassium) which are essential to muscular function, and importantly the function of her heart muscle. So, these electrolyte levels also have to be carefully balanced.

She gets an antibiotic drip, which prevents invading bacteria, or even her own native cultures, from growing out of control and infecting her immune compromised body. She continues to receive enzyme replacement therapy, which provides her with the critical enzyme until the donor cells can start growing in her bone marrow and producing enzyme on their own. I'm sure she gets much more besides, these are just the list we've discussed on the phone.

All this is to illustrate how absolutely miraculous the success of this treatment is for children like Gracie. It's a monumental undertaking for all concerned to maintain artificially these balances that, for most of us, nature maintains effortlessly. As the graft takes and her cell counts improve, Nature will take over again, where medicine is doing all it can to keep up.

I know it's starting to sound like there's an echo in here, but we are so grateful to everyone who's following Gracie's journey, and for everyone who thinks about her and prays for her, and loves her. We can't thank you enough.