Day +732

Day +732.. also known as 2 years and 1 day since James' stem cell transplant. It's an incredible number in a few ways... first and foremost, we truly thought that after James made it through transplant we'd be past the complications and medications within 6 months. That's the pessimistic view. However, 732 days ago if you told me James would still be with us today I'd take any situation, complications, or curveball on the table to make that happen. It's truly a miracle that James is here today - are we feel absolutely grateful and lucky to be able to say those words... and for all your support over such a long stretch.

As many of you know, it's been a winding road from the get go and we expect more of the same as we continue moving forward. Rita and I laid in bed last night and briefly talked about those moments in his hospital room two years ago last night. It wasn't a long conversation but the vivid memories that get into your head are worth 10,000 words. The uncertainty of the situation. Getting the strength to walk again on his own. The life or death moments both before transplant and immediately following it. This week two years ago was a very tough one with James' in a very serious battle against VOD. I remember distinctly for a moment a few days after transplant sitting in the break room on the 17th floor with some doubt in my mind for just a split second if he was going to make it before I kicked that thought out of my head and got back into his room to be by his side. Tough times for sure and I'd go back and read those updates right now but it would certainly side track me from finishing this update for at least a few hours (and I don't stay up too late these days) so that's for another day. If you have the time and energy jump over our old fundrazr site (https://fundrazr.com/f1OA33?ref=ab_37TAU9) and sort the updates from old to new and you'll see how lucky we are today. One of these days I'll find the time to get all those updates into a better format on this site.

We've met and talked to so many families over the last few years, months, days and some outcomes are good and others aren't. To those out there that lost a loved one or child - we still think of you every day and love you all... can't imagine the pain but you've got your entire network and this one behind you even still.

One of the early things we started to do when James was in the PICU... about a week or two into this journey... was look for ways to cheer others up and truthfully provide us some distraction for even a moment from what we were facing. It started in a pretty simple manner with us loading up on snacks for the medical staff and other parents and kids on the floor. It was pretty common to have the cleaning crew or overworked nurses and doctors swing by our room to grab something (we had a pretty mean food selection :-) during their undoubtedly stressful long shifts. During rounds we'd push snacks on the team of 15-20 doctors and nurses as we talked about the plan for the day. I'd say half truly wanted the snacks and 1/2 we forced them on, but it made everyone feel good.

A few months later once James was back for his stem cell transplant we would cart him around the floor in his blue wagon with his gigantic medical pole and hand out candy to other kids and nurses on the floor.  

We tried to keep that goodwill going once we were out... whether talked to other families that just had the worst news of their lives dropped on them, or looking to other ways to help and give back through James' annual blood drive.  As most of you know, we held a drive in 2019 in Chicago at two local churches with a big afterparty at D'Agostino's Pizza (thank you Scott!) on our (previous) hometown street of Southport Ave.  The day of the drive we got the honor of meeting James' donor, Spencer, along with his wife Diana and mom Pam that morning.  The photo on the left is the first time James met Spencer and you can tell they were immediately best buds.  At that drive we had 253 blood donors and over 50 sign-ups for Be The Match.

Fast forward to 2020 and we're off to an amazing start... and in the middle of a pandemic... thanks to all of you!  We're sitting around 240 enrolled, which should translate into 210-220 donations with no-shows or those turned away at time of donation due to mediations, illness, low iron etc.  THANK YOU so much to all our family, friends, co-workers and strangers that have giving up some time to donate blood as well as registered on Be The Match.  We know we ask a lot of you all at times... THANK YOU from the bottom of our hearts.  Please continue to spread the word and we can blow this thing out of the water!  On that note, if you take a photo while donating email it to contact@curiousjames.org or text it to me at 630-707-1015 and I'll post it on the blood drive sign up site.  Additionally, thanks for your patience on the sign-up process.  We're doing our best to keep up with the enrollment emails and I know it may take a day or two to get a response... we are all adapting to the pandemic so doing the best we can with what we have to work with :-) 

Vitalant, the organization running the drive, has been fantastic and really helped us get the word out.  The recent segment on NBC has driven significant activity to the drive and I believe a plug will be on WGN TV tomorrow as well.  Special thanks to Holly and Dave over there for getting the message out as well as Lauren Petty at NBC.

So with all that said - I also feel in necessary to give everyone an update on James.  We took him in for his clinic visit today and it was slightly less than ideal.  Before I go further, everything is OK and James is in a safe and good place - hence the "less than ideal" and not bad comment.  James has been really challenged over the past 18 months to find a way forward in his battle against GVHD.  It's mainly been solved with medication, immunosuppressant, chemo drugs, and a lot of patience with some up's and down's.  There are many, many variables we look at on his blood draws/labs but recently we've been trying to figure out why his ENC is ~ 2,000-3,000 when 500 or less is normal.  A few months ago his ENC spiked to nearly 5,000 and he had a pretty good flare of his GVHD and we ended up tripling his steroid dose to combat it...setting us back a few months on his med weans.  Fortunately we were able to FINALLY get him off prednisone about a month ago and onto a less potent steroid that should hopefully allow him to start to grow a bit (and it's easier on his body).  For the last week or so James has been a bit itchier than usual... often a sign of his GVHD wrecking some minor level of havoc on his skin.  James' labs today showed a jump to nearly 10,000 on his ENC.  Additionally his white blood cell count jumped up to ~22,000 which is definitely high.  The doctors aren't 100% sure what to make of all this.  In the short term, we've paused weaning his medication further and will return in 2 weeks for a re-look at all his number.  Best case is the next two weeks go well and this normalizes a bit, which is certainly possible.  Although you can't read too much into numbers they aren't meaningless either so it's a bit of a mental battle of balance overreaction and under reaction to find some kind of balance that makes sense.  

I did ask quite a few questions and was reassured from some of the answers.  For starters, I asked about James' JMML returning and was told that is highly unlikely this far in the game... two years in with nothing in site.  Secondary cancers from all his medications and from the stem cell transplant itself are always a possibility, but there is nothing at all to indicate that's the case.  The working hypothesis is that it's his GVHD rearing it's head yet again so we're just hoping that over the next week or two we don't run into any major issues.  Sometimes there is a calm before the storm and the numbers can be a precursor to what's next.  Other times, not so much.. so again don't want to think too far ahead here and let's wait and see what shakes out.  At his next visit on 12/21 we're really hoping these numbers are trending in the right direction or we may need to start up the prednisone again or potentially look at new and more invasive ways to combat his GVHD.  Neither are good options for James... it took a year and half to get off the prednisone and the alternative treatments require a the replacement of a permanent IV line and multiple full infusion days at the hospital every week.  We'll keep you all posted as things progress... we're very hopeful these worries are just that and not reality.  The last few paragraphs are more stream of consciousness and not necessarily reality at this point - it's all the things on our minds but chances are James will figure out how to navigate this on his own and we'll back on a straighter path forward.

In the meantime - keep sharing the details on James' Blood Drive and let's all keep helping those that aren't as fortunate as we are right now.  

CLICK HERE TO SIGN UP FOR THE BLOOD DRIVE

Thanks and much love,
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Dan, Rita, James and Baby Maeve