Day +597 (since transplant)

​Hello Team James!  We have waited a bit to write an update as this month has included a lot of ups, downs, and unknowns.  While James had a great start to his summer, enjoying strawberry picking, swimming at Grandma Kathy's pool, sleepovers with his cousins, neighborhood play dates and fun at summer camp, July has brought some setbacks.
 
As we mentioned in our last update, we were on track to reduce James' oral steroid (prednisolone) down to a physiologic dose at our clinic appointment on 6/24.  A physiological dose is essentially what his adrenal gland would normally make if he wasn’t on steroids to do it for him.  James has been on steroids to combat his GvHD for about a year and half, and we’ve been told that it takes roughly the same amount of time (another year and a half) for his adrenal glands to kick back in, hence the need to keep him on a small dose in the meantime while his body works through that.  Additionally, we have some shots that we can give him if his body needs it quickly (won’t go into detail into how/when that would occur).  After all this time and James working so hard to get reduce his steroid dosage over the past few months we finally got him down to that dose on 6/24.  We were so excited…only one month until we could potentially move James to a less potent steroid and finally (FINALLY!) start weaning down other medications.  As I stated earlier, we have been working toward this step since early 2018 and were anxious to start tackling other issues (lack of growth being #1 on the list since these steroids have prevented him from growing since then).
 
Unfortunately, as we have experienced in the past, stem cell transplant recovery is not without highs and lows, steps forward and steps back. After the Fourth of July, James started to experience bouts of nausea and headaches, seemingly out of nowhere.  James would be able to run around and play all day, and then within minutes of being his playful, energetic self, he would begin to complain that his head hurt and start vomiting.  With no other associated fever or other symptoms, we began to suspect that James' Tacrolimus (Tacro) levels were off.  You may remember that Tacro is one of the three immunosuppressant drugs (prednisolone and Imbruvica (chemo pill) are the other two) that James has been on to help keep his new immune system in check.  We brought James to clinic on 7/13 after our doctors told us to pause Tacrolimus for 2 days to see if that would help reduce some of his symptoms.  
 
James' Tacro level was higher than expected on 7/13 and the team decided to cut his regular dose in half.  Tacro is a tough one because if it’s too low it doesn’t suppress the immune system enough and if it’s too high it’s (more) toxic to your body, hence the headaches and vomiting.  While this was happening, and possibly due to the pause in Tacrolimus dosing, James' skin GvHD began to flare and he also began to experience more vomiting, diarrhea and overall not feeling like himself.  The doctors also noticed that, while every other lab result has come back with excellent results, one of James' labs has continued to remain abnormal.  
 
The doctors explained that, since we reduced the steroid to the physiologic dose in June, James' AEC or absolute eosinophil count has started to climb.  AEC is a blood test that measures white blood cells called eosinophils.  These white blood cells become active when you have allergies, infections or other diseases.  A normal AEC is between 50-350.  To illustrate, James' AEC number on 7/2 was 840, 7/13 was 1,850 and by 7/17 it was up to 2,800.   The team became concerned that, with the temporary pause in Tacro PLUS weaning of the steroid, James' immune system was not properly checked and it allowed his underlying chronic GvHD to begin to surface.  This manifested itself in lots of tummy issues for James and his skin starting to become drier and bumpier.
 
We introduced Imbruvica a few months ago with the understanding that it would have a similar effect as the Tacro and steroids and hence could help us get off of them.  That may still be the case, but it’s hard mentally giving him this everyday if we’re still pumping all these other drugs into him on top of it and not in substitute of it.  James is too young to understand how to take the pill so we have to break it open while wearing Latex gloves and mix it into his juice cup each morning.  Although you think we’d be used to this by now with the hundreds/thousands of doses of medication James has taken over the last 24 months but this one still gets me.  Every morning I am sitting there mixing a toxic substance into my son’s Paw Patrol or Trolls juice cup with gloves on because it’s not healthy for me to touch it and then sitting there making sure he takes it all down and puts it into his body.  I probably wouldn’t have as hard of a time with it if he could take the pill and just swallow it but it just speaks to the difficulty of all this… you get used to it but it’s still never easy.
 
So... back to the plan.  In order to combat what the team suspects in a GvHD flare, we were instructed to triple James' prednisolone dose on 7/17.  Although we were happy to do anything to relieve James' symptoms, it was a pretty rough blow to kick up the steroids again when we were so close to being off them completely.  Our hopes of moving on to the less potent steroid and beginning to wean off other medications has now been put on the back burner yet again.  As many of you know, the prolonged use of steroids, combined with all he has been through, has dramatically stunted James' growth.  Although we are so grateful that James is every bit the nearly 4-year-old he is when it comes to mental, emotional and social development, physically he is the size of a 2-year-old.  He has started to make comments like “I’m bald” and “I have no eyebrows” once in a while and is also become more aware of his size as he gets a little older.  That is all pretty rough as we don’t want his life to be any harder than it’s already been the last few years.
 
Thankfully, the increase in steroids has helped James' symptoms.  Over the past few days, he has regained his appetite and energy and (fingers crossed) has been able to keep all of his food/liquids down.  Given this change in symptoms, we were fully anticipating that the AEC number would go down when we re-tested James' labs on 7/22.  Not so.....The AEC is now 5,000.
 
Any cancer patients or cancer parents know the stomach knots that come with news that blood cell counts are "abnormal" and "trending in the wrong direction".  This kind of talk always leads me back to where we were two years ago, receiving news that James' white blood cell count was abnormally and dangerously high.  Our stem cell team has reassured us that the rest of James' lab results remain in great ranges and that they do not suspect anything else going on other than his chronic GvHD beginning to flare.  This eases some worry, but we remember how fast things can change, and not in a good way, in the oncology world.
 
For now, we are grateful that James seems to be feeling much better.  He looked so good in clinic that, despite the AEC going up, the team felt safe keeping the steroid at the current level and not increasing it again.  Our plan is to bring James back on 7/29 for repeat labs.   Once again, we will ask all of you, James' army of support, to pray with us that the AEC goes down, and that we can resume our path of medication weaning.  It is extremely hard to be patient with all of this, especially knowing how much James has already had to endure. I try to remind myself to be patient with James' recovery, he really has come so far.  You really are amazing James!  We love you so much!