On 12/24 we returned to clinic for more labs and more fluids and headed home by lunchtime.  James' constant bathroom issues persisted on Christmas Eve and Christmas Day with a fever coming and going... by Christmas night we were considering brining him down to the ER but after talking to the doctors a few times on the phone we decided to wait it out overnight.  James was improving slightly but took a bit of a tailspin on the Saturday the 26th.  Although there are closer hospitals to us our goal is always to get to Lurie's as his needs are fairly specialized with his medical history so I raced him downtown to the ER.  When we arrived his heart rate was pretty elevated and they had the room filled with medical staff immediately which is never a great sign.  That said, we stayed very calm as this wasn't our first rodeo and knew that James was in OK shape despite the urgency from past experience.  The team placed an IV and got fluids going... eventually admitting us around 10pm to the 17th floor.  We've run quite a few tests to figure out what is going on and the running hypothesis at the moment is either an infection or a virus yet again.  On the virus front, everything has come back negative outside of some cultures that just take a long time (day/days) to get a read on.  James had an x-ray earlier today and will have an EKG and heart echo tomorrow.  The EKG and echo are things we needed to do soon anyways as there are certain routine checks such as this with all the medications James is on / his prior cancer diagnosis.  The good news is these are not invasive so they should be a breeze tomorrow.

The team drew an insane amount of labs a few hours ago to check everything under the sun so we're hoping we get some direction on the likely cause soon.  That said, sometimes no one really knows and we're OK with that as well if he just starts to feel better.  He's been remarkably patient with everyone and when he isn't feeling crappy has been singing and being a happy four year old.

Although you can't understand him, this is James being a good and saying "Hey guys, I'm doing alright"

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.

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,
​
Dan, Rita, James and Baby Maeve

Update from Rita:

Hello Team James!

At the time of Dan's last update, James had just come home from the hospital and we had FINALLY gotten off prednisone (the oral steroid James has been taking since March of 2019).  We are so thrilled to report that James has tolerated the transition off prednisone to hydrocortisone very well.  We have not noticed any skin GvHD flares.  His appetite has returned and he is back to his baseline weight (right around 29 pounds).

Speaking of James' weight,  we had a marathon day of doctor's appointments on Monday, and the first appointment was with James' endocrinologist to discuss the plan for his growth.  Thankfully, James has grown a little bit since he was last measured by endocrinology in May of 2019.  In fact, James' growth rate is on par with other 4 year olds.  It is just that his growth was paused for so long and now he has to try and catch up.  You might remember that back in May, James' doctor ordered a bone age x-ray of James' left hand and the results showed that his bone age was that of a 2 year 2 month old. when his actual age was 3 years and 9 months.  Since James did grow a little bit and he is now off prednisone (which is known to stunt growth), the hope is that James will really start to grow on his own without any other interventions.  This would be the best case scenario, as interventions could include Human Growth Hormone, many more tests, more blood draws, etc...  We really want to reduce the number of specialists that James is required to see to give him as close to a "regular" early childhood as possible.

After endocrinology, James and I headed  over to Lurie to check in with stem cell, physical therapy and dermatology.   James is considered to be in remission from his JMML and that means he will be evaluated by physical therapy as part of his survivor treatment plan. PT will check in on a yearly basis to assess his strength and range of motion.  So much has happened in the past 2 years that it is sometimes hard even for me to remember that PT was once one of the many specialists that James was required to see.   I try not to dwell too much on the extreme hardships James  has had to endure, but it is also amazing to consider all the hurdles he has overcome.  This little guy was once required to have therapy to help him gain strength to walk, step up and down, climb and get around like the average 2 year old would.  He once had orthotics in his shoes to help stabilize his ankles, and now he runs faster and longer than ever before!  The physical therapist was very happy with how well James is doing physically, especially considering his recent hospitalization.  She tested the range of motion in his feet and hands, and gave me the happy news that she did not see any deficits.

Following the PT evaluation, the dermatologist came in to take a look at James' scalp and discuss options to stimulate his hair growth.  As Dan has alluded to before, no one can really tell us whether James' hair loss is permanent and no one can know for sure what exactly caused the hair to fall out.  We know that James battling the flu earlier this year caused his skin GvHD to flare, but the dermatologist suspects that one of the strong chemotherapy medications James  was exposed to ( Busulfan) may also have played a part in killing his hair follicles.  Either way, the first line of treatment is something you have all probably heard of, Rogaine.  We ordered some over the counter and are going to start to apply it to James' scalp daily.  The dermatologist recommends trying this for 6-9 months and warned that Rogaine has been shown to be effective in only 1/3 of patients, so there is not much else to do but wait, hope and pray.  The plan is for us to return in 6 months to see if any progress has been made, and also discuss the introduction of Latisse to stimulate James' eyelashes and eyebrow growth.  

Apart from the cosmetic concerns of having no hair, James' alopecia has other uncomfortable side effects.  He is constantly rubbing his eyes because he has no eyelashes to provide his eyes with extra protection.  He is also extremely sensitive to water getting in his eyes because it probably feels a lot different than it would for you or me.  His eyes are also frequently dry, even more so in the winter months, and anyone with young kids knows that trying to put eye drops in to relieve dry eyes in kids is no easy task.  Dan and I were both involved in trying to get James to let us put eye drops in, and let's just say James won out in the end.   He is one strong willed kid!!

The stem cell team reviewed James' labs and most have recovered back to normal ranges after his hospitalization which is a good thing.  James' AEC level is still in the abnormally high range (3000) so the team thinks he is still having some skin Gvhd, but is just monitoring the AEC for now.  

James was very happy to learn that we do not have to go back to the doctor again for 3 more weeks. While he is usually such a good sport about going to the doctor, the older he gets, the more he remembers and with that comes fear of things that happen at the doctor, like being poked with a needle.  James is really upset about "all the pokes" and of course we don't blame him.  Unfortunately, this is something that not even Airheads or jellybeans can distract him from.  He always handles it like a champ, but it is another example of the after effects of JMML that James will have to endure, likely for life.  Regular blood draws, doctors examining him and comparing him to "normal" kids... it will be a lot.  I say this not to be a downer, but as a reminder of what James, and all cancer survivors endure.

On that note, what can we do to help James and other cancer patients?  We can donate blood!  If you haven't yet, please consider signing up to donate blood using the link below.  Blood donations always fall during this time of year, and even more so this year due to Covid.  Rest assured that blood donation is safe.  If you are healthy and able, please consider donating.

https://www.curiousjames.org/blood-drive-2020.html#/

I want to end with a genuine THANK YOU to everyone who reads these updates, keeps James and our family in their thoughts and prayers, participates in the blood drives and who has supported us these last few years.  Two years ago, on the Saturday after Thanksgiving,  James was taking a nap in our apartment, and I was beginning to pack and get organized for our hospital admission the following Monday.  In that quiet hour, I was trying to choose books and small toys to bring with us and found myself sobbing on the floor, overwhelmed by the enormity of the battle we were facing.  In two days, we were going to check our little boy into the hospital and subject him to the harshest chemotherapy drugs and the brutal stem cell transplant process, all with no definite discharge date in sight.  We had no idea if James, or if we, would survive it physically or emotionally.  

I remember that moment vividly. I know that every year around Thanksgiving, I will remember how scared I was and how grateful I am that James is still with us and of how many people helped us get to another Thanksgiving... this year marks #2 since stem cell transplant on 12/6/18.  Thank you all so much!  We are always grateful for you.  We wish everyone a Happy Thanksgiving and hope everyone has a great holiday, even in these very strange Covid times. 

Team James - 

I know many of you have been wondering how James is doing and I am thrilled to say that things are going very well.  We are back home as of Wednesday afternoon and James is on the mend.  His energy is coming back day by day and he has a few pounds to regain but other than that things are great after our 5 day stay at Lurie.  All signs are looking this this was 100% Rotavirus and it just hit James really hard because of his challenged immune system.  This is tremendous news for a number of reasons.  First and foremost, nothing more serious was causing the issues James was experiencing.  The brain MRI came back with zero issues and looking great.  Second, there is an explanation for why James was not feeling well.  In this journey there are often unknowns and for those of you that have had to deal with medical issues personally our in your family/circle of friends you know that the unknowns can sometimes be harder to deal with that bad news.  And last but certainly not least, James' GVHD did not rear it's head as his immune system activated to fight off the virus.  This is really critical and a very positive outcome... the last few times James has been sick his GVHD has flared up and caused all kinds of other issues (case in point, killing his hair follicles and attacking his skin).  None of that happened this time.  James' immune system was able to slowly fight off the virus without attacking the rest of his body at the same time.  Granted it's not "normal" to spend nearly a week in the hospital on IVs with Rotavirus, but the that out of the fold and everything else has been very positive.  

Also - thank you to everyone that has signed up for the blood drive in December.  We're ~50 enrollments so a good start.  Last year we hit 253 and there is no reason we can't beat that number this year with all your support.  THANK YOU!

- James, Dan, Rita and Baby Maeve

​

All - I am writing to give a brief update on James' MRI as I know many of you have asked how things are going and if we had any results yet.  James was supposed to get this done Monday but it kept getting pushed back due to over-scheduling, etc.  James didn't sleep much last night (translation -> not at all) but fortunately it wasn't due to pain he was just restless.  His steroids are about 3x normal and he took a pretty solid nap in the late afternoon so I wasn't surprised.  I served as his waterboy all night and carried him to and from the bathroom about every hour or two.  King James at his best.  I give this context because I finally flagged our nurse down and asked if we could do his MRI at 4 am this morning.  We were both up anyways and I figured we could beat the crowd and get it over with.

James did amazing... he was a little confused at first when he was getting strapped to the board and put into the machine but got through the entire thing without crying, screaming, or even showing any remote signs of being afraid.  I was very impressed considering he just turned for and an MRI is a little daunting when you can't understand what they heck is going on around you as a little kid.  We got the results this afternoon and everything looks totally normal.  This was a huge relief for us as you can imagine.

We're still stuck here on fluids and some other IV meds but James has been on the up and up.  We're hoping for a great night tonight and a bit more sleep...  and possible discharge tomorrow.  If that doesn't happen, then likely Thursday (but my money is on tomorrow).  James will be moving to a new steroid once we are out of here and off his prednisolone which is also news to our ears.  We've been working towards this for 18 months.  Although he isn't off steroids, getting off prednisolone and onto a less potent one is a very positive step.  We're hoping to handles this with flying colors and we can eventually get him off steroids all together before the end of 2020 (gotta shoot high :-))

Thanks everyone for all the support and thoughts - and also for the great turnout thus far on sign up for the blood drive!  We really appreciate everyone taking the time to sign up to donate - it means a lot of us and I am sure it will to James one day when he is old enough to understand and appreciate all the support he's had throughout this journey.

October has been an up and down month... each time I have been ready to write an update something else changes (either for better or for worse) and I've delayed writing an update for a few days each time as a result.  Unfortunately right now is one of the "for worse times" as we are back in the hospital and admitted back at Lurie's.  I'll get into details on that in a moment.

Rewind to the last few weeks.  James has been having headaches followed by vomiting throughout October which has landed us back at the hospital a few times in the last 3 weeks.  These headaches have come out of nowhere with immediate onset - basically a 180 from feeling great to feeling really, really crappy.  Turns out that each time it has been medication related.  One of the medicines James' takes to suppress his immune system, Tacrolimus, requires a delicate balance to keep in therapeutic range.  Too low and it doesn't do it's job and James graft-versus-host-disease flares up causing skin and other issues.  Too high and it's highly toxic - which is what was occurring each time.  The only way to balance out his dosage is through blood draws (labs) to check his levels, which has to be done at the hospital.  It's kind of a guessing game and hence the need to come back on a regular basis to re-check his labs.  James calls drawing labs "getting poked" and is not a big fan but always handles it like a champ and is super brave.  The hospital uses a butterfly needle to access his veins and it's not a permanent IV that is put in.  They draw 4-5 vials of blood (he has a lot of labs drawn every time because of all his meds, hence a decent amount of blood needs to be drawn) then its done and the needle is out.

Fast forward to this past week and James was feeling really crappy again for the third time in 2 weeks.  Vomiting, sharp stomach pains, etc.  We assumed it was his Tacro level yet again and got him into clinic the next day to check his labs/numbers.  It is standard procedure with any immunocompromised kid feeling sick to check for literally everything under the sun each time (viruses, infections, and so on) and James came back with a positive for Rotavirus.  This is a pretty common virus in kids and it's pretty rough to get through for a regular, healthy kid.  James has actually had it before (during transplant) and it was not fun.  Most kids are vaccinated for Rotavirus at a few months old... James was when he was born but those vaccines were wiped out with his stem cell transplant.  Unfortunately he has not been able to be revaccinated since for this or really anything for that matter because of all the immunosuppressants he is on to control his GVHD.  This has hit James really hard.  Ultimately James was sent home and we were told to monitor him closely... he took a pretty rough turn with a 103.9 fever and no sleep for 2 straight nights along with lots of vomiting etc.  He was exhausted to the point where he couldn't stand or hold his head up much so we got him to the ER and his vitals were not in a great place.  His heart rate was 190+, his respirator rate was about 3x normal, and he was severely dehydrated with about 6-7% bodyweight loss since we were there just 24ish hours earlier.  The doctors worked pretty quickly to get an IV in for fluids, which is always a challenge for James.  He has very small veins and the dehydration makes them almost non-existent.  Getting an IV placed is much harder than the butterfly needles I mentioned earlier and almost always takes a few tries across both arms, which is what happened per usual this time around.

James' vitals are significantly improved and back into a normal range.  James' heart rate around 110 and his oxygenation is near perfect.  Cognitively he has been a bit slower to recover - he has not been as attentive.. not making a lot of eye contact... slower to respond etc.  Rita and I have obviously been concerned about this, especially with how dehydrated James was Friday night.  The doctors would like to do an MRI on his brain to check and make sure everything looks good.  This was supposed to be today, but given it's pushing 5 PM and we've been through this many times I can confidentially say it will be tomorrow at this point.  James just turned 4 so sitting still through an MRI is a tall task.  The last few times he has had to go under anesthesia for this and we're hoping to avoid that this time around if possible but it will be up to James if he's able to handle it or not without being put under.  Those machines are pretty loud and frightening for some adults so I totally understand why it's near impossible to get a kid to sit motionless in there for an extended period of time.

As today as gone on James does seem more alert than he did this morning, so we're optimistic the MRI will be find and this is all residual effect of the Rotavirus.  Because James doesn't have a great way to fight anything due to all his suppressing meds and a lack of ability to mount an internal defense on his own due to being on steroids for a year and half viruses such as this are a much larger challenge for him that it would be for a healthy four year old.

James' is getting sick of the 24x7 IVs, beeping and being bedridden but taking it in stride as much as possible. He is not happy to be here, which is what we want to see as it means he's eager to get better and get out of here.

We'll make sure to update everyone in the next 24-48 hours with more details.  In the meantime, I also wanted to take this moment to please ask everyone to sign up for James' Blood Drive (Click HERE to sign up!).  As many of you know, we held a blood drive in 2019 and had over 250 blood donations in the two day drive (THANK YOU) as well as many sign ups for Be The Match.  Without many, many blood transfusions James would not be with us today.  Times are tough right now for everyone... COVID, jobs, homeschooling, not being able to see your families, you name it... we all have struggles.  This is a free way to give back and no matter how bad life seems for you right now, it's worse for someone else and you can help make it a little better for them.  Blood donations are way down and already challenging in December to begin with.  The drive will be for two full weeks in December... 12/5 though 12/20 and you can visit any Vitalant center you want in either Chicago or Pittsburgh.  Why this week in December?  December 6th is the two year anniversary of James' "re-birth" and stem cell transplant.  There are many people in need, probably more than ever, of blood product at a time when donations are on the decline.  One ask is that if you intend to sign up please do it now rather than waiting so we can plan accordingly and have a good read on resources needed.  AKA don't wait to sign up, do it today... we and others need you.  Please share the sign up site with any many people as possible.  5-10 sign ups a day over the next 60 days = 300-600 donations.  Every single person counts and the only way we can get to those numbers is with your help sharing the message.

Lastly - thanks for all the support.  Appreciate everything more than you know.

September is Childhood Cancer Awareness Month. Amazingly (and not in a good way) only 4% of cancer research funding is dedicated to childhood cancer.  Cal’s Angels is an organization that has helped us/James immensely over the last two years and their mission is to fill in that gap.

Kudos to my sister-in-law Brooke Mulcahy for her fundraising efforts... today is the last day. Consider making a donation BY CLICKING HERE if you’re willing and able...

The final 24 hours of fundraising... anything helps!

Happy 4th Birthday James!
 
That’s right – James turns 4 years old today… he is truly one of the most amazing kids I have ever met, and I am not just saying that because he’s our son (although admittedly I have a TON of bias in that statement J).  James is extremely caring, very smart with a huge vocabulary well beyond his years and simply loves life.  It’s not to say he doesn’t have his moments… I mean he is a four year old… but James is genuinely a great kid and I have no doubt will be just as amazing, compassionate and caring as he continues to get older.
 
Despite some of the challenges James is still facing we absolutely feel grateful and thankful and fortunate that we’re able to celebrate with him today.  He’s faced more adversity in the last 24 months than many of us experience in a lifetime.  As some of you may remember on James’ 2nd birthday a miracle truly happened.  We were celebrating James’ birthday with family, but without James, on the 11th floor at Lurie’s… singing happy birthday, eating cake and taking videos to show him back in his room where he remained on a breathing machine.  Well, he didn’t want to accept that and we had a call from his room during that party that James was ready to give it a go and breath on his own after nearly a month and a half with that machine keeping him alive… he was removed from the ventilator that day and has never seen it since.  That was the ultimate birthday present… hard to top that one.
 
It is hard not to make James’ life story about his illness and obstacles.  We really try to avoid falling into this trap, although it may not seem that way when you read these updates.  Part of the reason for that is we’re writing to keep everyone updated on James’ progress and hence it inherently comes out in these updates that way.  Day-to-day James lives his life just like any other kid… and with just as much energy, if not more, than most.  As anyone with kids his age knows, chasing him around is a full-time job.  He loves his cousins, aunts, uncles, grandparents, neighbors/friends etc. and REALLY loves his grandmas that care for him every day.  One really positive aspect of the past two years has been James developing an amazing bond and relationship with his grandmas.  That’s easy for me to say since they have basically given up their retirement and large chunks of their lives to help care for him but he wouldn’t change it for the world and looks forward to seeing them single every day.
 
We had a birthday celebration this past weekend in our front yard with immediate family and neighbors.  It was raining, but honestly, I think that made it even more fun for the kids…. A few pictures are attached below.  As you can see, James was in his element and told us many times the next day how much fun he had on his birthday weekend.  Per James, his favorite part of his birthday was "eating candy."
 

In terms of James’ health – things are about status quo.  Our next appointment is in about 10 days and we’re hoping to step down on his steroids at that time to inch back down to where we were a few months ago.  The doctors are really determined to find a way to get James off steroids… they know his current medication regiment isn’t good for him long term, but balancing that with what he needs right now makes it a very challenging balancing act.  We’re really hoping that James can handle further weaning of his steroids without issue and the next 2-3 months will be critical as we work through it.  Avoiding illness is really important as that’s one major factor in what flares up his immune system and causes these bouts of his GVHD rearing its head to occur.  Thank you again to everyone that keeps up with James' story, for your support, and always keeping James in your thoughts and prayers.  We don't say it often enough!
 
So, with that said – to my best friend… happy 4th Birthday.  I love you more than you’ll ever be able to understand and am the luckiest Dad on the planet to have you in my life.  I know your mom feels the same.  As promised, you can have Mike and Ike’s for breakfast and fried chicken for dinner (don’t judge, it’s his birthday).  Looking forward to what this next year brings and we’ll face any obstacles together, but 99% of the time life is going to be great so let’s enjoy it.  Love you buddy.

​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.

​Hello Team James!  We have waited a bit to write an update as this month has included a lot of ups, downs, and unknowns.  While James had a great start to his summer, enjoying strawberry picking, swimming at Grandma Kathy's pool, sleepovers with his cousins, neighborhood play dates and fun at summer camp, July has brought some setbacks.
 
As we mentioned in our last update, we were on track to reduce James' oral steroid (prednisolone) down to a physiologic dose at our clinic appointment on 6/24.  A physiological dose is essentially what his adrenal gland would normally make if he wasn’t on steroids to do it for him.  James has been on steroids to combat his GvHD for about a year and half, and we’ve been told that it takes roughly the same amount of time (another year and a half) for his adrenal glands to kick back in, hence the need to keep him on a small dose in the meantime while his body works through that.  Additionally, we have some shots that we can give him if his body needs it quickly (won’t go into detail into how/when that would occur).  After all this time and James working so hard to get reduce his steroid dosage over the past few months we finally got him down to that dose on 6/24.  We were so excited…only one month until we could potentially move James to a less potent steroid and finally (FINALLY!) start weaning down other medications.  As I stated earlier, we have been working toward this step since early 2018 and were anxious to start tackling other issues (lack of growth being #1 on the list since these steroids have prevented him from growing since then).
 
Unfortunately, as we have experienced in the past, stem cell transplant recovery is not without highs and lows, steps forward and steps back. After the Fourth of July, James started to experience bouts of nausea and headaches, seemingly out of nowhere.  James would be able to run around and play all day, and then within minutes of being his playful, energetic self, he would begin to complain that his head hurt and start vomiting.  With no other associated fever or other symptoms, we began to suspect that James' Tacrolimus (Tacro) levels were off.  You may remember that Tacro is one of the three immunosuppressant drugs (prednisolone and Imbruvica (chemo pill) are the other two) that James has been on to help keep his new immune system in check.  We brought James to clinic on 7/13 after our doctors told us to pause Tacrolimus for 2 days to see if that would help reduce some of his symptoms.  
 
James' Tacro level was higher than expected on 7/13 and the team decided to cut his regular dose in half.  Tacro is a tough one because if it’s too low it doesn’t suppress the immune system enough and if it’s too high it’s (more) toxic to your body, hence the headaches and vomiting.  While this was happening, and possibly due to the pause in Tacrolimus dosing, James' skin GvHD began to flare and he also began to experience more vomiting, diarrhea and overall not feeling like himself.  The doctors also noticed that, while every other lab result has come back with excellent results, one of James' labs has continued to remain abnormal.  
 
The doctors explained that, since we reduced the steroid to the physiologic dose in June, James' AEC or absolute eosinophil count has started to climb.  AEC is a blood test that measures white blood cells called eosinophils.  These white blood cells become active when you have allergies, infections or other diseases.  A normal AEC is between 50-350.  To illustrate, James' AEC number on 7/2 was 840, 7/13 was 1,850 and by 7/17 it was up to 2,800.   The team became concerned that, with the temporary pause in Tacro PLUS weaning of the steroid, James' immune system was not properly checked and it allowed his underlying chronic GvHD to begin to surface.  This manifested itself in lots of tummy issues for James and his skin starting to become drier and bumpier.
 
We introduced Imbruvica a few months ago with the understanding that it would have a similar effect as the Tacro and steroids and hence could help us get off of them.  That may still be the case, but it’s hard mentally giving him this everyday if we’re still pumping all these other drugs into him on top of it and not in substitute of it.  James is too young to understand how to take the pill so we have to break it open while wearing Latex gloves and mix it into his juice cup each morning.  Although you think we’d be used to this by now with the hundreds/thousands of doses of medication James has taken over the last 24 months but this one still gets me.  Every morning I am sitting there mixing a toxic substance into my son’s Paw Patrol or Trolls juice cup with gloves on because it’s not healthy for me to touch it and then sitting there making sure he takes it all down and puts it into his body.  I probably wouldn’t have as hard of a time with it if he could take the pill and just swallow it but it just speaks to the difficulty of all this… you get used to it but it’s still never easy.
 
So... back to the plan.  In order to combat what the team suspects in a GvHD flare, we were instructed to triple James' prednisolone dose on 7/17.  Although we were happy to do anything to relieve James' symptoms, it was a pretty rough blow to kick up the steroids again when we were so close to being off them completely.  Our hopes of moving on to the less potent steroid and beginning to wean off other medications has now been put on the back burner yet again.  As many of you know, the prolonged use of steroids, combined with all he has been through, has dramatically stunted James' growth.  Although we are so grateful that James is every bit the nearly 4-year-old he is when it comes to mental, emotional and social development, physically he is the size of a 2-year-old.  He has started to make comments like “I’m bald” and “I have no eyebrows” once in a while and is also become more aware of his size as he gets a little older.  That is all pretty rough as we don’t want his life to be any harder than it’s already been the last few years.
 
Thankfully, the increase in steroids has helped James' symptoms.  Over the past few days, he has regained his appetite and energy and (fingers crossed) has been able to keep all of his food/liquids down.  Given this change in symptoms, we were fully anticipating that the AEC number would go down when we re-tested James' labs on 7/22.  Not so.....The AEC is now 5,000.
 
Any cancer patients or cancer parents know the stomach knots that come with news that blood cell counts are "abnormal" and "trending in the wrong direction".  This kind of talk always leads me back to where we were two years ago, receiving news that James' white blood cell count was abnormally and dangerously high.  Our stem cell team has reassured us that the rest of James' lab results remain in great ranges and that they do not suspect anything else going on other than his chronic GvHD beginning to flare.  This eases some worry, but we remember how fast things can change, and not in a good way, in the oncology world.
 
For now, we are grateful that James seems to be feeling much better.  He looked so good in clinic that, despite the AEC going up, the team felt safe keeping the steroid at the current level and not increasing it again.  Our plan is to bring James back on 7/29 for repeat labs.   Once again, we will ask all of you, James' army of support, to pray with us that the AEC goes down, and that we can resume our path of medication weaning.  It is extremely hard to be patient with all of this, especially knowing how much James has already had to endure. I try to remind myself to be patient with James' recovery, he really has come so far.  You really are amazing James!  We love you so much!