Day +844

It's been a little over a month since our last update... per usual Rita and I have been a bit behind on our updates.  Honestly this tends to happen because we're usually waiting to get clarity on a few things before updating everyone, but you'd think we would be used to the drill of not letting that stop us since clarity rarely occurs and often there are new things that pop up before we resolve the initial areas of concern that we're waiting to update everyone on.

2021 has been a challenging year thus far with new complications and issues arising on top of the old ones that we are still trying to beat and get past.  Don't get me wrong, there is still a lot to be thankful for and many great memories have already been made this year.  As much as we try to focus on the positives it's hard to not let the negatives frustrate and eat away at you with constant worry about what the future may hold.  It's increasing difficult to google "how many days since December 6th, 2018" when I sit down to write these and not feel a sense of frustration and sadness for all James has had to ensure over the last 844 days since transplant + his original diagnosis and time in the hospital for a few months preceding that... so we'll call it 964 days but who's counting.  It's also hard constantly seeing my iPhone show pictures for this time 1,2,3 years ago (I know it's trying to help me cherish the memories) and have so many bad ones scroll across my phone along with the good ones.  It's been almost a year on the don't since GVHD took James' hair so seeing those photos fade away is hard as well.  We've been really holding out hope that James may be able to at least grow eyebrows or eyelashes but it's looking pretty unlikely at this point.  He's aware of it but other kids his age haven't started to judge him yet for this or his height, etc but we know it's coming and that's hard to pre-feel the pain that he is likely going to experience growing up.  That said, we are SO THANKFUL that he will grow up... that he is here... and that he's such a funny and happy go lucky kid most days.

Despite all this, James continues to find ways to live it up and make the most of life.  We've been able to do a few sleepovers with his grandparents and cousins this year which has been amazing.  They are really terrific with James and he looks up to them more than anything in the world (well, except for his grandmas).  

At the time of our last update James has battle some pretty major GI issues - we had an endoscopy (tube down the throat, thru stomach and into the intestine + a colonoscopy done a little over 2 months ago.  The results were better than expected... the photos of the issue looked essentially like the inside of your cheek and there was inflammation but nothing overly concerning at the time.  James was put on a new, temporary, medication to help calm his GI down at that time.  Since that time we've tried to get James off this medication 3x but within a day or two of stopping it his symptoms become unbearable and he's back in the bathroom 20+ times a day and often multiple times per hour at it's worst.  The good news is that as soon as we go back on the medication it helps significantly (although helping a bit less this third time around)... but with all this going on we knew something was not right and this wasn't the solution.  

Last week James went back into Lurie's for the same procedure again to get a re-look and the results were dramatically different than last time.  There were very obvious ulcers and deterioration of his issue... needless to say as soon as we saw the photos post-procedure last Thursday we knew this was likely fairly serious and not something that was going to resolve easily.  Going into this we had a pretty good sense that there were not any really great potential outcomes... out thoughts were that it was one of the following:

(1) Infection.  We didn't think this was likely, given how long he's been dealing with this and that his GI was always been somewhat of a problem.  That said, any infection is a big deal for James so we were hopeful this wasn't the issues

(2) GVHD in his gut.  Again, not a good outcome and pretty serious.  The signs are not pointing to this as the likely outcome as it's not presenting how the Dr's would expect for GVHD

(3) Some type of autoimmune disorder affecting his gut.  This had been positioned as a potential outcome last time as well, so it was in the back of our minds.

Below are photos of the tissue from his past Thursday, and you can see rather easily it doesn't not look healthy.  We had a better understanding now why he is so uncomfortable and at times in pain.

Apologies for the photos - but it put in perspective what else James now has to deal with... on top of everything else.

The consensus is that this is IBD (Irritable Bowel Disease).  

Per WebMD (this is easier than me trying to explain it)

"Inflammatory bowel disease (IBD) is a group of conditions that cause swelling and irritation in your digestive tract, such as Crohn’s disease and ulcerative colitis. Irritable bowel syndrome (IBS) is the term for symptoms that happen when the contents of your large intestine move too quickly or too slowly.
IBD is what doctors call a structural disease. That means physical damage causes your symptoms. Doctors can see chronic inflammation or ulcers when they look at your gut with an X-ray, endoscopy, surgery, or biopsy.

Crohn’s disease, one of the main forms of IBD, usually causes pain in the lower right side of your belly. The other main form, ulcerative colitis, leads to pain in the left side of your belly."

We are waiting for some more details from his medical team to confirm the diagnosis and treatment plan... which we hope to have this week.  It was positioned to us as a likely lifelong condition and it can happen to around 1% of patients post-stem cell transplant with immune system complications.  I must admit that it's hard to take this one on the chin along with his GVHD and new lung issue that popped up a couple months ago.  The percussive vest that James has to wear twice a day shakes his entire body (while also wearing his nebulizer the entire time) and causes him a lot of stress.  In my head it's hard to not go down the rabbit hole of how many issues his stem cell transplant, which saved his life, has caused / continues to cause him.

The three treatment options we discussed were essentially:

1 - a pill that James would take daily or twice a day (not sure) to address this 
2 - a shot that we would give him every 14 days
3 - an IV infusion that he would need for a few hours every other month

It's my understanding that those meds are essentially in order in terms of least to most aggressive treatment.  It's hard to imagine they will start with #1 (but never say never) given some of the recurring issues and that this is classified as a moderate case as it's presented today (on a scale of mild, moderate, severe).  We can certainly handle any of the options and take them in stride... we're at the hospital quite often anyways so if we need to do infusions it's just one more thing on the list and they are drawing blood each time anyways so placing an IV for a few hours isn't a huge leap.  The hard thing to grasp is that these treatments could be lifelong... it's hard to not have an end in sight on this stuff and also worry about what the next unknown issues is lurking around the corner that he may have to face.

As you can likely tell by the length of this post I could go on for hours but will call it quits for tonight.  It may not seem like it after reading this but I we're all staying positive and most importantly focused on keeping James' spirits up (often with too many TV shows and the occasional Sour Patch Kid).  We appreciate all the support and will keep everyone posted once we know what the plan is.  My apologies for any typos - this one is too long to proofread :-)