Day + 886

Per usual, it's been way too long since we wrote an update on James (over a month).

The past few weeks James has been feeling better than ever... I don't think we have had a stretch this long without James really complaining about anything and it's been so great to see him just enjoying life.  Hence, we are as well!

At the time of our last update James was still battling some up's and down's with GI issues (which have been ongoing for a pretty long time) and was diagnosed with some form of "to-be-determined" irritable bowel disease.  The not-so-great news of is that we received confirmation that James' has Crohn's disease since that last update.  The good news is, as a result of this diagnosis, we've been able to figure out a treatment plan to help him, which we are very grateful for.  The spot we found ourselves in over the past few months - one of uncertainty and no clear plan on how to help James feel better - wasn't a great place to be.  Obviously for most families this diagnosis just on it's own would be tough to hear, but we really have been taking it 100% in stride and feel really optimistic just seeing James feel so much better since starting him on some new medications.  While this is typically a lifelong condition, there is some chance that when James finally beats GVHD we could see improvement in this Crohn's diagnosis and even resolution of it.  The doctors made it very clear that the expectation is that James will have this for life, but did follow up with some sliver of hope that this may not be the case.  James, always the lover of all things rare and 1 in a million, is due for some reverse fortunes here so we'll hope that this is the case sometime in the future.  If that doesn't happen, the doctor said it's likely years and not days or months into the future.

On a separate (but very good) note we were able to take a much, much needed family trip to Florida in mid-April.  We had a lot of doubts about this in the weeks up to this (not because of the 24 hour drive, but because James had rarely felt good for any long stretch of time in the past year+).  We had a moments of doubt that we may be border-line insane for even attempting this trip but we booked an AirBnB with full cancellation until 24 hours prior to check-in and decided that if we thought we could pull it off we were going to do it.  Best decision ever... it was terrific.  James' grandma's joined us on the trip and his Aunt Brooke, Uncle John and cousins Jack & Charlotte made it for a few days as well.  Hands down one of the best trips and memories in my lifetime... just hanging with family with no other distractions and James feeling great the entire trip.

Alright... doing a 180 and getting back to the discussion on his meds...

James started a regiment of Budesonide, which is a steroid used to treat a few different conditions (one being Crohn's).  This is a medication typically taken for a month or two during flares to help get things back to normal and it's worked wonders for James.  It essentially reduces inflammation, swelling and irritation... we noticed a pretty dramatic improvement in James within just a few days of starting this - so no doubt it was the reason he began to finally turn a corner.  We recently just stopped his regiment and are a bit nervous on how he will handle the next week(s) but so far so good.  If things deteriorate we can start up again right away, but hoping for the best.

James also started another new medication called Jakafi (pronounced jack-uh-fye).  This medication was just FDA approved for the treatment of GVHD in mid-2019 and only for kids 12 and up (it's still not FDA approved for kids younger than 12).  Additionally, it's only approved for acute GVHD and James has chronic GVHD.  So... to say this is a surefire bet to help is a stretch.  When James was having an impossible time getting off Prednisolone we were able to get this approved via insurance but never moved forward with it.  Like a lot of these drugs the potential side effects are many - low blood counts, risk of infections, secondary cancers, etc - so I think the medical team was waiting to see how James did moving to his new, less potent, steroid hydrocortisone which is what he is on today.  Anyways, I am giving all this detail because when the Crohn's diagnosis came up the medical minds got together after some discussions with Rita and I and we were really pushing to look for a treatment plan than may be able to treat multiple conditions at one and potentially, over time, eliminate some of his other meds.

While we aren't sure how James will respond to the Jafaki there is the potential that it could replace his hydrocortisone (steroid) and eventually his Tacrolimus (immune suppressant) while also potentially helping with both his GVHD and Crohn's.  Again, a lot of if's here, but Rita and I made the decision that this was the plan we preferred to take and the medical team was onboard so here we are.  '

Jakafi is a JAK-inhibitor... JAKs (Janus kinases) are proteins that can lead to inflammation - which is often what James is dealing with in both his GVHD and now-GI issues.  It can also decrease the levels of another protein called cytokines - which also contribute to inflammation if/when James' donor immune system is attacking his organs.

There are also some decent studies around a bi-product of this medication being hair regrowth in cases of allopecia.  It's been over a year now since James has had hair, so we're not too hopeful... but still clinging to some home.  For all these reasons we decided it was worth giving this a go and see how James responds.  This medication, like many, is very expensive so we feel fortunate to have insurance approvals in place to cover a large portion of the cost given that James is under 12 and doesn't have the exact condition it's approved to treat.

We'll keep you all posted as things progress, hopefully for the better.  James is doing great and loving life as always.