Day +760

Welcome to 2021!  As you all know, at the time of my last update James was having a rough go and we were admitted at Lurie's trying to figure out how to help him. We ended up being admitted for 4 days after being in and out of the hospital much of the week prior as well.  While admitted we did a whole host of tests - an EKG, Heart Echo, X-Ray, CT scan, endoscopy, colonoscopy and an exam from the ophthalmology team.

You may be wondering why the medical staff is looking at the inner workings of every part of James' body and there are really two primary reasons.... (1) looking for signs of GVHD in his GI tract, in his eyes, in his lungs, etc... and (2) looking for signs of any infections in the same areas.  James was pretty dehydrated and lost a decent amount of body weight - he was down to 27 pounds which is not much (understatement) for a 4 year old and hence the 4 days of IV fluids while we were admitted.  The GI team saw signs of inflammation but nothing extensive.  The general guidance was that it could be minor GVHD, but it was more likely irritation from a virus or infection that they just couldn't pinpoint.  The good news was the suspicion for GVHD in his GI tract was/is low and they also ruled out some other auto-immune type diseases which were equally concerning.  This is one of those times where no answer is better than either of those answers and "we don't know" is more reassuring than the alternative bad news.

Eyes looked pretty good but showing some signs of dryness.  Its a simple fix with eyedrops for now (although it is very challenging to get James to allow us to put the drops in) and they'll keep monitoring it, but again no suspicion of GVHD.  James has not had any vision problems but the teams routinely check this stuff out as GVHD can start to attack other areas of your body.  With James it was initially his lungs and over the past year or so it's been his skin.  As a reminder, GVHD is essentially his immune system attacking his body - which we control by severely depressing James' immune system to not allow it to function properly.  Over time, the goal is to very slowly and gradually reduce these medications with hopes that they will eventually get along.  In the short term (which has been 2 years and 1 month since transplant, so not sure "short term" is the proper verbiage anymore) James has had to deal with the side effects of all these medications as well as the increased risk of infection or illnesses being pretty dangerous to his well-being.

On to the lungs now... we had little concern with the CT scan as James' lungs have really given us no indications of any trouble recently.  Everything he's been dealing with has been either skin or GI related.  We were surprised to hear that the CT came back with some concerns of what the images showed in his left lung.  I had a follow up with James at Lurie's today to discuss this and essentially he has build up in the bottom of his left lung.  He hasn't been running consistent fevers, so the concern of an immediate infection is low.  There are pockets of air and liquid that shouldn't be hanging around... and the worry is that over time that the build up can get infected.  The below photo (left hand side) shows James' lungs in 02-2020 and then (right hand side) in 12-2020.  As you an see in the red circled area, there are some new developments where the white area is... which is the concern.  Your lungs should be black throughout with some white lines etc (blood vessels)... what you see below isn't normal.  That large circle in the middle is James' heart.

For anyone this is not good news... but for James this is worse news given (1) all his immunosuppressants and (2) his history of lung disease.  We'll know more in the coming days but the plan is either to monitor over the next few weeks/months and follow up with some x-rays to see if it's progressed or improved or put James under again for a bronchoscopy to collect some tissue etc and see what's down there.  For the "bronch" the team essentially puts a tube down James' throat and into his lungs, washes it with water, then collects some of the tissue for examination.  Given they were just down his airway last week for the endoscopy we're hoping to avoid this if possible but we'll follow their lead on how immediate the concern is or if we can wait it out a bit.  The main thing is that James is in a safe place - so we'll put him through it if it is needed to ensure that's the case.  

Aside from the lung concerns, to combat the GI issues (James was going to the bathroom about 20-30 times per day over Christmas) the team tripled his steroid dose while we were admitted... and for a few days after returning home.  After a few days of being home we moved James back down to his normal steroid dose (the steroid he is on now is called hydrocortisone-a much less potent steroid than what James has been on for the past 18 months)  and within a day his body was not very happy.  While glad to be home, we were pretty concerned about the immediate flaring of his skin GVHD all over his body.  Below is a picture of James' chest, but his arms, legs, and head  all looked just as angry.

The good news is that James tends to react well to an increase in steroids.  It's a blessing and a curse because we can never seem to get off of them as a result, but on the blessing side it's been a way to get him back to some sort of baseline.  We contacted Lurie's right away and went back up to 3x his steroid dose later that day.  James takes these meds three times per day so they can make an impact pretty quickly... I am glad to report his skin looks much better today (the above photo was from Friday) and the plan is to keep him on his 3x dose for the foreseeable future.  Although we don't love tripling his steroids it's actually a better outcome than some of the alternatives that were initially on the table when we spoke with the doctors Friday.  We're very fortunate and glad that James' skin improved dramatically from Friday to today and that the doctors felt reassured at his clinic appointment.  The two alternatives are (1) introducing a new chemotherapy drug called Jakafi (strange name I know) as kind of a last ditch effort before the last alternative which is photopheresis.  We're really hoping and praying to avoid Photopheresis as it would require James' to have a port put back in, which is a permanent Central IV Line and spend two full days at the hospital each week for minimally the next few months and then on a lesser frequency for many months after that.  Photopheresis involves using a machine, similar to dialysis, that takes James' blood from his body, separates it into it's core components (red blood cells, white blood cells, platelets), then treats the white blood cells with a medication and UVA irradiation and then the blood is returned back to his body.   It's been a mental struggle to think this is on the table after all this time and all he's been through.  We are not there yet, but it's been brought up a few times as these issues persist so we're a bit on edge of this even being a possible solution.  We'll continue to stay positive but I wanted to give a brief overview of what's being discussed so you can get some idea of the stressors of day to day life and decisions when it comes to diseases such as this.

And on a closing note  James' energy and appetite have been great the last few days.  He has been in a great mood and is back to his cheerful , sweet self- below is a photo of James and Maeve to end this post with a smile.  He loves his sister very much and is always trying to care for her and cheer her up when she is crying.

We'll keep you posted as we learn more... but happy to be home and looking forward to a hopefully healthier 2021 for James and all of you!

- Dan, Rita, James and Maeve