Thursday, July 11, 2013

Oh boredom how we miss you!!

There were these two glorious months of peaceful medical boredom.  At first we were holding our breath just waiting for the next shoe to drop.  Then as the weeks went on we started relaxing a little, too afraid to really start counting how long it had been or get too relaxed.  Then sometime around the 6 week mark since anyone had any issues we started to really enjoy it and think maybe all the initial crazy transplant drama had passed and we had achieved the utopia of boredom.  Well... it was fun while it lasted!

The drama all started with Max's biopsy which had been this ongoing debate and topic of conversation. At Stanford they recommend a 6 month, 1 year and 2 year protocol biopsy of the new kidney just to make sure everything is ok.  It is possible for things to be going on that are completely asymptomatic that you have to see inside the kidney to realize.  It is a standard procedure in California but not here.  Addie's 6 month mark coincided with Max's evaluation and since she was under 2, we managed to fly her out and have it done without any expense so she had hers there. And got a perfect report, no issues!  Then in June we were approaching Addie needing a 1 year biopsy and Max needing a 6 month one.  Flying four to California just wasn't feasible so we ended up doing Max first here since he had not yet had one and had many complications.  We were still going back and forth about whether to even biopsy Addie at this point.

So Max had his biopsy and the actual biopsy went very smoothly, no complications.  They kept him overnight for observance and right when we were about to pack him up to head home, the doctor came in and let us know that the initial report indicated something was wrong.  Within a few hours they told us that the biopsy showed that his body was in the process of trying to reject the kidney and also that he had an infection, presumably a UTI that had spread to the kidney, as well.

Most people know that you can reject a transplanted organ but I know that before we started this transplant experience I did not really understand what that meant.  I guess I figured that one day you were fine and the next day your body had rejected the organ and it was done.  What I now understand is that rejection is a slow process that can be stopped but not reversed at any time.  Basically the body identifies the new organ as an intruder like it would bacteria or something making you sick.  So it will start killing it cell by cell.  But think about how tiny bacteria is when you are sick and it still takes your body a long time to kill.  Then by comparison there is this large adult sized kidney inside of Max, well his body is not going to be able to wipe it out quickly. 

So all we really know right now is that the first biopsy showed that cells were being attacked and killed.  Right now we don't really know the extent of what damage has already happened.  His labs show his kidney function is still good so his kidney is still working.  Mind you he has a lot more kidney in him than he really needs.  He could easily get by on one little baby kidney and instead he has a big adult kidney so he could have actually experienced damage to a significant percent of the kidney and would still maintain okay kidney function.  So until we have been able to stop the rejection and re biopsy, we don't really know the whole story.

Even though he can lose a lot of the kidney and still be ok, it is still far from ideal.  Transplanted kidneys get damaged with time from the medications and some degree of mild but unavoidable chronic rejection.  The life of a transplanted kidney varies but with a parent donor I guess we could be looking at 10-20 years which is a big range! But anyway damage from rejection would decrease that time significantly.  So aside from any current concerns with rejection, it is a big deal to us because it would be costing him years of having his special parent kidney.

But hopefully we did catch it nice and early and have been able to stop it.  Essentially to stop rejection you have to wipe out the entire immune system.  Transplant patients dread this fine line of needing enough of an immune system to not be chronically sick but not tolerating too much of one that could really get going on the kidney.  So the reaction to a rejection episode it to highly suppress him so the rejection cannot proceed.  We have done that by adding a steroid medication on top of his other medications.  A lot of transplant patients are always on prednisone the steroid.  It's been a really blessing they have avoided it thus far since it does have side effects and is harder on their weak immune systems.  But for now it looks like it is here to stay for Max.

Please pray for him because the past 5 days he has actually been really irritable and upset and not sleeping well.  We are hoping this could be due to the IV antibiotics he has still been on for the infection which will now stop.  If it is the steroid causing it his discomfort and behavior change and it is permanent, well we are all going to lose our minds! So please pray for comfort and rest for sweet Max and that he will get his sunny disposition back soon :)

The same week that we found out about Max's rejection, the doctor also talked to us about Addie's liver enzymes.  Addie's liver enzymes get checked with her other bloodwork just as part of the panel. Starting in around April, we started noticing both Max and Addie's being elevated.  I think initially we noticed Max's and then realized Addie's were also high.  Normally this number should be below 40 and theirs were in the 100s instead.  Hoping this could be transitory, we just waited and kept following it.

Two weeks ago Addie's number rose into the 300s and just waiting didn't really seem like a good plan anymore.  Max's continues to be elevated in the 100s.  Our kidney doctors looked at all their medications and everything they could think of to try and understand why this showed up for both kids around the same time with no history of it.  So they thought we needed to go talk to the liver team.  Since Addie's numbers are considerably higher and she is not having other kidney rejection drama we decided to address the liver stuff specifically with her and hope that an answer we get could then be generalized to Max also.

Having been through everything we have, I try to stay optimistic but certainly have developed a good sense of realism about me.  So while I certainly hoped that we would go yesterday and they would say oh yeah let's fix this one medication and it will all go away and we never have to talk about it again, I was bracing myself for that not to be the case.  And it went pretty much like I worried- there aren't any obvious reasons but it is a serious concern.  A lot of their liver numbers are fine so their livers are working.  And they are really well and happy and not at all yellow! So it's not a case of full blown liver failure, just to make sure everyone knows that and isn't worried.  They are ok.  BUT, these labs due indicate that their livers are inflamed and not clearing all things as well as they should. And given it seems to have been going on for 2-3 months now, ignoring it or hoping it goes away doesn't seem to be a real option.  Left untreated their livers would presumably get worse and accumulate damage and they wouldn't stay as happy and healthy as they currently are.

Because they both have a similar pattern beginning at the same time, it seems to suggest an infection of some sort that they both contracted, possible one from the other one.  So they are running a lot of tests and exploring a lot of viruses including a very rare form of Hepatitis (Hep E, which I don't know if anyone has ever heard of!).  It will be a couple of weeks before we get final results back.  We would need to know what the virus is to know how to treat it.  Most likely treating it would be a tricky decision since the best way to deal with the virus may not actually be ideal for the kidney.  So please pray that we make the best decisions as we get more information.

There may not be a virus.  It could also be a strange autoimmune response whereby the body confusingly starts attacking the liver instead of the new kidney.  Or it could be some sort of intestinal issue that is affecting the liver.  Right now we really are not sure.  So we will wait on bloodwork, get a liver ultrasound and quite possibly go ahead and get a liver and kidney biopsy for Addie.

The good thing is that Addie is so happy, full of energy and otherwise very healthy.  She is not sick right now and if this liver issue had not shown up in her labs, we would have had no idea from her behavior.  So we are thankful that she is feeling well and we pray that we can figure this out and treat it with minimal pain or discomfort for her and before she would develop symptoms or big problems as a result.

So yeah that is what all is new with us.  We just need lots of prayers right now with these new issues and as we try to manage them all.  We know these kiddos are serious fighters and have been through worse and we know God has taken excellent care of them in the past so we know everything will be ok.  We just are praying for minimal hospitalizations and complications in the process and that they will remain happy as we go through these new issues.  We are blessed that I am off work right now and that we have Al's parents out for a month so we have a lot of support!  And we really appreciate all the people praying for us and checking on them.  It means so much!