Keegan continues to be his usual enigma-wrapped-in-a-riddle self. There was an error in his TPN last night, so after much discussion (including with the transplant cardiologist on service directly calling our room to go over his decision...yeah!!), we did not run anything except his antibiotics overnight. We held some of his diuretics today to see how he would do. Low and behold, you stop the diuretics, and he blows up like a little balloon. Fluid status is and has always been a problem with a solitary kidney. It's so hard to find his "happy place." His kidney lab values did get a little better today, so we are more optimistic that we were not dealing with him simply being on the verge antibiotic toxicity. Big relief.
So tonight will be the last dose of antibiotics. The plan then is to see how he handles the first 24 hours off them, play with his fluid status a bit as we are able, and watch his labs closely for another looming flare. If all is calm tomorrow, we are all banking on a Saturday discharge mid-day after all his labs and vitals have come in. Then he will have the weekend off his TPN and get some trough-level labs on Monday morning. This plan errs on the side of caution and experience and respects the fact that it's more emotionally harmful to leave and come back right away. Very reassuring, to say the least.
We also had our meeting with Keegan's rheumatologist, immunologist, and transplant cardiologists today. I really struggle to put all that information into words, yet alone out on this blog. The ultimate plan for right now is to stay the course on his regular dose of steroids and anakinra. His chromosomal microarray labs should be back in the next three weeks. Those will hopefully tell us if we are on the right track with the IL-1 blockers, or if we should switch gears. The caveat being that the microarray could also tell us nothing at all. However, if they indicate that the next "big drug" we have been discussing is really the next best option, we will have a lot of work to do to get Keegan ready for that transition (more to be explained later, if and when that choice is made). This line infection has put us all on edge because even though the cultures may be negative, we know from past experience that the bacteria can "hide out" on his line and reappear later. If it's going to do that, we would like to see it happen in the next few weeks while we wait on the results of the microarray. Then we will have to discuss the need to put a new, clean port in before starting drugs that put him at a greater risk of sepsis.
Now, if the microarray doesn't give us as much guidance as we hope, there will be another set of genetic immunodeficiences that we will be running to look for answers. The kicker is that if he has one of these combined immunodeficiencies, the next "big drug" we are considering would be the absolute wrong thing to try. All of this assumes that we can continue to control flares during the waiting with steroid pulses.
(Are you still with me?)
The general idea we got from the rheumatologist and immunologist is that it seems most likely Keegan has some sort of underlying native immune disorder that he would have had whether he was transplanted or not. The fact that we are following protocols for solid organ transplant patients and he has not rejected his heart means that this dysfunction is coming from a different part of his immune system altogether. Additionally, the proof that his GI problems are caused by an autoimmune response supports the theory that it is something he has, rather than something we induced. Whether or not science and technology has a way to find out where that problem is and what to do about it is another story entirely.
I'm sure that none of that made sense to anyone other than me. In fact, maybe it doesn't even make that much sense to me! BUT, the general gist for now is that we stay the course and see what he does while we wait on these last bits of information. This was the perfect time to get these heads together though because we needed to know (1) everyone was on the same page and (2) what is being done to save time and protect Keegan while we wait.
No matter what happens, this is shaping up to be a long, hard summer for our Bug. Tonight, we are trying to keep our sights on the short-term goal of getting him home on Saturday. I know we say it all the time, but thank you once again for every prayer and well-wish sent Keegan's way. We certainly could not do it without our faith and without the encouragement of our family and friends. Thank you!