Day + 628

​All – as usual, it’s taken us much longer than usual to write an update than intended.  You’d think with COVID we would all have more time while working from home etc. but somehow it seems we have less... not sure how that works.  The math doesn’t add up but it sure seems like reality!
 
As many of you know we had a new baby girl, Maeve, join our family and it’s been a fantastic two+ weeks just getting to enjoy life for a while with our newly expanded family.  Little sleep has turned into less sleep… but once you’re at a certain amount of sleep depravity it’s all really the same.  I think Dunkin Donuts stock may have moved a few points the last couple of weeks just on our consumption alone.  I attached two videos to this update: (1) is when James was told he had a baby sister literally minutes after she was born and (2) is when they first met.  James couldn’t be at the hospital due to COVID and Maeve came a bit earlier than expected so it took us 5 days to get her home from the NICU to meet James and the rest of our families.  It was a little hectic at the hospital… after we arrived I had to run outside and draw James’ meds for the next few days on a picnic bench outside since our amazing mom’s were taking him while we waited for the baby to arrive.  After crushing through drawing about 40-50 syringes of various meds I rushed back upstairs and Maeve was here shortly thereafter.  Always an adventure.

Thank you all again for all the positive thoughts and prayers for James over the past few weeks.  We’ve been pretty concerned about James’ AEC and keeping his GvHD at bay.  At the time of the last update, the AEC count was over 5,000 (normal range being below 500).  As a result, we essentially tripled James’ steroids and went way up is dosage on another one of his immunosuppressant drugs, Tacro.  On the good news front his AEC came down to 2,670 the next week (finally) and then decreased again to 1,620 the week after.  Unfortunately, the following week we saw very little movement and it hovered around the same range.  We’re now almost a month and a half out from his med increases and have our next follow-up tomorrow morning.  While hopeful, I am still skeptical (hopefully wrong) that it will be in the normal range.  James’ skin seems to be a bit bumpier lately and I am preparing myself that the AEC may still be high and causing his GvHD to flare a bit, albeit it mildly.  Again, I hope I am wrong and we get to go down on his medications a bit after the clinic visit tomorrow.
 
What does this mean for James?  We go back to where we have been since May of 2019… trying to be patient with the slow steroid wean process and work toward getting James off the many medications that he remains on now 2+ years into this endeavor.  The doctors frequently tell us that stem cell transplant recovery is a delicate balance: trying to wean medications while keeping complications like his chronic GvHD in check.  A few steps forward, and then one or two steps back.  
 
Practically, this means we will continue to have to wait to address James' lack of growth, as there is not much endocrinology can offer while James continues to be on prednisolone (steroids).  This also means we will have to wait to address his hair loss as our dermatologist is concerned the skin GvHD remains active and the introduction of additional medications may exacerbate symptoms.
 
This is frustrating as James will be turning 4 in a few weeks yet is smaller than kids half his age.  In previous updates, we mentioned how James is starting to become more aware of his size and appearance in comparison to other kids his age. Over the past month we have had the first, of what we know will be many, conversations with him about how his body and what it has been through.  He also has made some comments recently about why he doesn’t have hair or eyebrows.  Not the easiest questions to answer but we’re doing our best!  James, being the amazing kid he is, isn't upset but is more curious and just wants an answer to his question.  For now, he accepts the explanation that he was very sick when he was 2, and although he is much better now, his body is still recovering.  He doesn't harp on it and continues to have his usual zest for life.  We hope his joyful energy will always be here, but it is hard not to think of the many challenges he may face as he gets a bit older.
 
Moving forward, we will try our best to be patient with the medication weaning process and are grateful for how well James has been doing since the steroid increase.  He is full of energy and his appetite, while still great (although we know not many almost 4 year olds have the best appetites or variety of food preferences), is much better.  James has been busy playing with friends and having sleepovers and dance parties with his cousins.  His favorite game these days is "Monster Tag", which basically involves anyone chasing him around while roaring like a monster.  Typically, we try to run James around to get him tired for a good night’s sleep but it ends up that we’re the ones doing the most of the running. 
 
I would say “I’ll post an update after the results tomorrow” from James’ labs and visit to Lurie’s, but in true fashion lately I am sure that update will be a few days out (at best).  In any case, we really to truly appreciate all the continued support.  Even though we feel fortunate 99% of the time there is the 1% of the time that catches you off guard and you get down a bit on the whole situation, especially for James’ sake.  If it wasn’t for all of you the 1% would be a much greater number.