In just a few days (12/6) it will be THREE YEARS since James' stem cell transplant - Happy 3rd re-birthday James!!  Each year since we have hosted a blood drive (the last 2 virtually due to COVID). 
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So with that said... the 2021 Blood Drive for James has officially begun!  I sent out an email about this a week or two ago, but the drive runs 12/1 - 12/31 across all 17 Vitalant locations and you can sign up and get details here:

We are also supporting Cal's Angels as part of the drive... first off - I know many (MANY) of you donated to the Cal's Angels Glow Walk just a month or two ago.  To be clear, we certainly aren't expecting more donations from you and want to thank you for your generosity and being amazing / helping others.

Another really cool aspect of this drive is that we are able to put giant 36x24 poster boards in every single Vitalant lobby for the month of December promoting donations to Cal's.  These poster boards have James' photo and story on them so we're hoping that the thousands of folks coming into Vitalant will see this and be inclined to make a donation.  The poster boards look like this in case you are interested:

We're hopeful to surpass the roughly 250 donors we had last year... and we have 2x the time to do it this year (the drive in 2020 was just 2 weeks long and in 2021 it's a full month).  Please spread the word as we noticed the last 2 years that many individuals came because someone in their family or a friends told them about the drive.... it's an easy way to turn 1 blood donation into 2,3,4,5... all donors also get a Free Large 1-topping Pizza and Free Pint of Ice Cream (Thanks Papa Johns and Culver's)!  Also - for any outside of the Chicago area, Vitalant is everywhere so if you go out of state just text me the location and date and I can have them manually track it.

Reminder:  If you want / are OK with your photo on the blood drive page take a picture while giving blood and either email it to contact@curiousjames.org or text it to Dan @ 630-707-1015.

In terms of how James is doing - overall things have been going REALLY well!  There are really just two things on mild/minimal concern recently:

• James keeps getting these skin rashes on his legs but they don't seem to bother him too much.  We're thinking that it's likely his GVHD acting up and have our next visit at the hospital on 12/10 so we'll get the scoop then.  They aren't too concerning right now, its more the concern of anything escalating - but we'll worry about that if it happens and I feel its unlikely.

 James is LOVING pre-school and getting so smart these days... it's fun to see him grow up and be the happy kid that he is today despite all this crap.  We're also excited that James was allowed to get the COVID vaccine, of which he just got his 2nd shot this week.  Once the CDC approves additional shots for immunocompromised kids 5-12 years old James will need one more due to his immunosuppressants etc... so we'll do that when able.

We are really looking forward to celebrating Christmas with a much healthier James this year and a much more active baby sister Maeve!  We wish you all a wonderful holiday season.  Thank you all for your love and support!!

First off - THANK YOU to so many people for your generosity and donating to the CAL'S ANGELS GLOW WALK.  It was a huge success with Team James taking the top fundraising spot... $12,671 in donations from Team James - all to going to a good (and much needed) place to help research and find a cure for childhood cancer.  This also meant James got to lead the walk for the entire event, which you can tell by the video below meant a lot to him (and us)!

 On the health front - we also had a longer clinic visit this past Friday... outside of his normal labs and monthly checkup with the stem cell team James also had a CT scan of his lungs + an appointment with his pulmonologist.  To refresh your memory, James was diagnosed with lung disease (broad / general term) a while back and put on some preventative treatments due to some concerns in the lower portion of his right lung.  These consisted of a percussive vest and nebulizer 2x a day which James has not been a fan of.  He's also on a number of medications to treat potential infections etc that could occur, which is always a concern.  For the CT James had to have an IV in for a few hours and he was a champ with having it in all afternoon.  Attached is a photo of us going down the elevator from his labs down to the CT.  He's looking like such a big kid these days!  

Great news on the CT - James' lung looks MUCH better and, while not fully resolved/normal, the doctor said he looks "great" and they are very encouraged by the progress.  No more vest, no more nebulizer!  The stem cell team also made a slight decrease to his last remaining immunosuppressant, Jakafi.  We're using the Jafaki to combat everything from this lung disease, Crohn's, and his GVHD collectively so the fact that we're now going down on this is very good news.  We're making more progress on his medications after knocking off a few big ones this year already and I'm hopeful there is a chance we could be off or near all his medications in about a year at this pace.  This is HUGE because it may mean we can finally re-start James' early childhood vaccines... he got some of these before he got sick but the stem cell transplant wiped them out and he has been unable to get them due the immunosuppressants.  Right now James is out in the world without any protection from chicken pox, measles, rubella, etc.

On the less than great news front, James also had a follow up with dermatology a few weeks ago and they essentially told us that his hair isn't coming back.  While not surprising, we've been clinging to some small sliver of hope with the Jakafi and other treatments we've been trying.  In a nutshell when James' GVHD really broke out over a year ago it attacked his hair follicles and in simple terms scarred his skin making this irreversible.  Think of it as if you were burned over your entire body and that hair would never grow back (not a great analogy but you get the point).  It has been harder recently because James is starting to get teased more often and we've had some recent instances where he has been down about it.  It really sucks knowing this is going to be a problem for him for the rest of his life - but if it was between him having hair and being on 20 medications with lung and other issues or James making all the progress he's making and no hair I'd take no hair 100 out of 100 times.  I also try to think back to the times 3 years ago when this started and how lucky we are to even be where we are today... there are many worse things in life and many larger battles that he came out victorious on.  That said, it's still not hard to be upset that it has to be this way even despite all that.

Additionally - and I hate to even bring it up after we just asked everyone to donate to the GLOW WALK - but we will be doing a 3rd annual blood drive in December, so mark your calendars.  Details are still being finalized but it will either be a multi-week virtual drive or potentially an in-person drive, likely 12/11 and 12/12, in Chicago.  If the in-person does happen we will still have a virtual component for those that don't want to/can't trek down to the city.

So to wrap it up - great progress, great update on his health, and we're feeling really good about what 2022 will have in store.  We just need to keep it going and minimize the setbacks.  Thank you as always for the love and support - you all rock!

Dan, Rita, James (aka "The Flash") and Maeve (aka "Wonder Woman")

First and foremost - James has been doing GREAT since our last update and we are making more progress weaning his medications.  James had a good clinic visit today to the "poke doctor" (it may be obvious but he calls it that because they have to draw his blood with each visit to do labs).  

As of today James is officially off of Tacro... a really strong immunosuppressant that he has been on for 2... 2 1/2 years!  We were able to get him off steroids a few months ago, now Tacro.  Next is a CT scan of his lungs in October to see if we can start eliminating some of the medications he's been on to treat his lung disease.  We're hopeful the CT looks good and we can stop using that term (the last one looked better than expected, so we're optimistic).  Big day today - definitely going to celebrate tonight!  As always James did great at the doctor and said it was a "10 out of 10" when he got home... not sure I've ever heard a kid describe a doctor's visit that way, but love the positive spirit. 

We have the Cal's Angel's GLOW WALK tomorrow night... it's always inspirational to see how many people show up for a common cause with one goal in mind - helping other families and funding research so more kids don't have to deal with this in the future.  It's also tough to see the families that still show up despite their kids passing away and that they are still there trying to make a difference.  Rita and I are SO GRATEFUL for all the donations.. we are currently at $9,731  with 121 donors to Team James!  THANK YOU!  $10, $20, $50, $100 at a time... it adds up quickly and every dollar makes a difference (and don't forget to check with your employer to see if they will match your donation).  I know asking people for money can be hard, but if there's ever a time to be annoying it's now for this cause :-).  So thanks for putting up with my updates and posts online and most importantly thank you for supporting such an amazing cause.  And thanks again to Auntie Brooke for organizing and all you efforts!

To drive more activity and donations the local paper wrote an article on James a little over a week ago and it was published on his birthday.  We appreciated that others take the time to do things like this and are always more than willing to do anything and everything if it has a shot to help the cause/others.  

I am hoping to keep this update as brief as possible, but if the past is indicative of the future that’s probably unlikely as these seem to get longer and longer each time I write them.  Some good news, some bad news with James… but before we get to that I want to give a quick plug on Cal’s Angels which is a local charity that was and continues to be a life saver for us and for many other cancer families.  Cal’s is the go-to around Lurie’s Children’s hospital… keeping not only the kids spirits up with events, gifts, wishes, etc. but also the parents.  Spending weeks/months in the hospital we used our Cal’s blankets every day to rest up and they still send out care packages to not only James, but also Rita and I a few times a year which is really cool.  Not many of these groups support you post-discharge but Cal’s gets that for most families such as ours the battle isn’t over at that point (case in point, me starting this update with the words “Day 998 since transplant). 
 
In 2019 Cal’s partnered with United to send James and 100 others kids on a trip to the North Pole from O’Hare, with Santa and all his friends.  You can see James in this video at exactly the 2:00 minute mark – somewhat unrecognizable these days as he has hair and looks a bit different then from the steroids etc.  He still talks about that trip to this day.
 
United welcomes more than 100 kids for fantasy flight to North Pole | WGN-TV (wgntv.com)
 
The Cal’s glow walk, their most important fund raiser, is on 9/25 and we’re going again this year to support the cause and raise some money.  Team James, organized by James’ aunt(ie) Brooke is looking for anyone willing to throw a few bucks in and/or spread the word.  And of course if you want to join the team (virtually or with us in person) all are welcome!  Link below (great job Brooke)!  If you do donate, also check to see if your company does a matching contribution for Cal’s Angels.  Believe it or not, only 4% of the federal budget for cancer research is dedicate to childhood cancer.  Cal’s helps to fill that gap, and helps a ton of families in the process.
 
www.givegab.com/teams/team-james
 
So on to James.  We had a really good clinic visit this past Friday with James’ stem cell team.  He’s been handling his medication weans very well over the past few months and has been completely off steroids for a month or two now with no adverse effects.  The next major medication we’ve been tackling is Tacrolimus, another heavy immunosuppressant that keeps James’ immune system at bay… and not functioning properly.  Outside of short and long term side effects that are always a risk (infection being one that’s always high on the radar for anyone with a suppressed immune system), getting James off this drug is also the next step in his recovery towards tackling the next.. and the next… until we can hopefully at some point say that for the last time.  The doctors agreed to take James’ dosage down to 0.6ml (he takes this every 12 hours and has for a couple years now).  For perspective, his prior dosage was 1.2ml and the last few months it has sat around 1.8ml.  In just a few month span that’s a major downward trend and James is so close to getting off.  We left that appointment Friday with the goal of James, nearly 3 years, finally being able to kick this to the curb at his next appointment in 4 weeks!  As we’ve experienced with attempting to get James off steroids so many times these things rarely go according to plan, so we were pessimistically preparing for something to go sideways while hoping and praying it wouldn’t.
 
Which leads me to the less than ideal part of this update.  James began experiencing similar Crohn’s / GI issues recently and they started to amp up this past Saturday and then even more so on Sunday.  James went to the bathroom about a dozen times Saturday and then Sunday it increased even more.  Tack on a fever around 101-102 which got up to 103.4 Sunday night and things haven’t been great the last few days.  We re-started James on steroids today, but that should be short term fix which they call a stress dose.  He’ll be on them as long as he isn’t doing well, and then we can quickly wean them down and get him off of them again.  James is in good spirits but it really hurts him a lot to go to the bathroom, which is often, so that’s not easy and you feel for him when he’s constantly afraid to go/crying but he can’t help but have to constantly.  The sliver lining is that he shrugs it off quickly and gets back to being the James we know. Today was his first day of school… well, it was supposed to be… so we’re bummed about that for his sake and not being able to be there with the other kids.  We pulled him for the week as we try to figure this out / help him and we’ll take another shot at school hopefully next Tuesday after Labor Day.  No mention (yet) of going backwards on the Tacro medication wean, so we are hopeful we can find a way to get this under control and get back on track.
 
And now for my comments that likely won’t be popular to everyone reading this, but that’s ok.  If you aren’t vaccinated please for the sake of those around you get it done.  James and others like him will never be safe from COVID-19 or many other illnesses if we willfully allow them to persist more than they need to.  We can’t control everything, but we can get this country and the world in a much better place if everyone would start thinking more about their neighbors and less about themselves.  We already had to pull James from his original school and put him somewhere else at the last second because they couldn’t commit to a mask mandate for the kids.  I totally get that it’s not fun wearing a mask and you want to see your kids smile, but have some perspective.  If that’s the biggest stressor in your life, life is pretty good for you.  I am not trying to polarize anyone with these comments, but just ask that you put yourself in other peoples shoes before making a decision on what the right thing to do is.
 
As always, thank you for all the continued support… can’t thank everyone enough and we’ll keep you looped in in the coming days/weeks.
 
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(Update written mostly by Rita.. thank you Rita!)

Hello Team James!

Apologies again for such a long time between updates, but for the most part, the delay has been due to James having a fun-filled and doctor free summer!  

As of our last update (all the way back in April), we were hoping that James' new medication (Jakafi) would help to alleviate some of James' gut issues and we could continue tapering him off steroids. For the last few months, James has been on a low dose of hydrocortisone and we had been slowly reducing the dose in an attempt to help wake up James' adrenal system.   The adrenal system is responsible for producing cortisol, a hormone that helps your body regulate many things: metabolism, immune system, blood pressure, response to stress, etc.  As the doctors have explained it, James' adrenal system has essentially been sleeping for the last 2+ years while he has been on prednisone (a synthetic steroid similar to cortisol) and then hydrocortisone.   

The withdrawal of steroids has to be slow to avoid symptoms of withdrawal (like headaches, weakness, fatigue) and also keep his GVHD from kicking in and attacking his body.  Unfortunately, in mid May, James did experience some symptoms that manifested themselves as headaches and vomiting.   We had to employ a "stress dose" or triple his regular amount of steroids for a few days to help James' body recover from the withdrawal, and then we slowly started the process again.  Our endocrinologist put in an order for hydrocortisone "sprinkles", or doses so small that they have to be ordered from a special pharmacy, so that we could make only slight reductions in the dosing to help James' body adjust.  In order to administer these doses, we actually sprinkled the medicine out of the capsule it came in and into applesauce.  James would then have a few spoonfuls of the applesauce.  I know we've shown  pictures of the array of medications James has to take daily (there are many, plus several vitamins and pills), and here we go adding in another strange thing into his routine.  James handled it so well, without complaint, which is just amazing.  We sometimes forget how little he still is and how much we ask of him, yet he (usually) complies without much complaint.  Now, trying to get him to take a bath or stop playing at a playground, that can be a different story.... but that is true of all 4 year olds, and we love when James can be included in that "all 4 year olds" group.

Thankfully, James tolerated the rest of the wean incredibly well.  We are incredibly happy to report that as of 7/16/21 James is completely OFF oral steroids!  It's a huge accomplishment that has taken us roughly 2 1/2 years to achieve with a million setbacks but we finally crossed the finish line and it feels amazing to have some tangible progress.  

We were able to take our first quick trip as a family of four to Milwaukee, take James to a White Sox Game, and enjoy fireworks with the neighbors.  James was so full of energy, he was challenging all the neighborhood kids to foot races in the streets while the fireworks display went on, well past 10:00!! It was amazing to see him feeling so great.  He didn't win a single race, but it didn't stop him from lining up again.

On 7/14, we gave James his last "sprinkle" dose, and on 7/16, we headed to Lurie for a marathon day of lab draws, x-rays, and a follow up CT scan of James' lungs.  Turning first to the labs, all of James' labs look really great.  We are far out from transplant, but it is still very much a relief to see white blood cell counts in the normal range.  The endocrinologist let us know that James' cortisol level is pretty low (~ non-existent), but this is expected.  His body is still figuring out how to produce cortisol on his own.   Thankfully, our doctor thinks James is safe to remain OFF steroids, and we will monitor him again next month.  If James does get sick (high fever, vomiting), then we have a plan in place to give his body a stress dose of steroids to help his body respond to the illness/stressor.  We also have some back-up (needle) injection medications that we can go to if things go south quickly but thankfully have never had to go there.

James also had a bone age x-ray done which is just a quick x-ray of the left hand.  You might remember that his last bone age study was done in May of 2020.  At the time, James' bones looked like that of a 2 year old, rather than the 3 and 1/2 year old he was.  The endocrinologist was encouraged by this, noting that the study suggested that James is going to grow for an additional 1-2 years after the rest of his peers stop growing.  While he likely won't be very tall as an adult, we are encouraged that he will be able to catch up to most of his peers, just with some extra time.  The x-ray taken on 7/16/21 showed about the same results, that James is still about 1.5 years behind in growth.  The endocrinologist is encouraged that James is growing a bit and was especially excited to hear that his feet are growing, as this is a good indication that his other bones will follow suit.  For now, the plan is to wait and watch, and we'll take it.

Now to the big news from the appointment.... the CT.  Our last CT from December of 2020 showed cloudiness in James' right lower lobe.  Our pulmonologist is not sure what that cloudiness is, but it got her worried enough to prescribe preventative breathing treatments.  The goal of the treatments was to keep any secretions from building up in that area and causing an infection in the lung.   

I think we have alluded to what the breathing treatments entail, and they are not fun.  James is asked to wear a percussive vest that litterally shakes his entire body for 30 minutes twice a day.  To be honest, we rarely (aka never) hit those targets, but we do what we can.  I would liken it to a mini jack hammer type vibration that shakes your whole body.  Not comfortable for anyone, let alone a kid.  While wearing the vest, he is also asked to put a mask over his face and breath in albuterol solution, followed by saline solution.  He doesn't mind the mask, but combined with the vest it causes him a ton of stress.  Needless to say, this is not James' favorite activity, and it has been challenging for us to force him to do it on a daily basis.  We did our best, and went into the CT hoping we would have positive results.  This was also James' first CT without anesthesia so we were crossing our fingers he could handle it and stay still without putting him under (which he did).

I am so happy to report that James' CT looked "very good" per our doctors! The cloudiness and findings in the right lower lobe were not just improved, but they were GONE.  Completely!  The pulmonologist was very encouraged and recommended that we continue the breathing treatments for another 3 months and reassess.  We will do the CT scan again, and if the results are good, we can hopefully stop the breathing treatments all together. 

With this happy news, our stem cell team also felt comfortable with starting to wean James off his other big immunosuppressant drug, Tacrolimus.  James has been on "tacro" since his stem cell transplant on 12/06/2018.  We have always been intent on getting James off this drug as safely and as quickly as possible,  but this is even more of a priority now given the pandemic we are all living through.  All these drugs have a laundry list of potential side effects (think of the last drug commercial you saw and x10 with all this meds) as well as putting him at some major risks when it comes to things like infection / the potential inability to fight off any serious illness.  As is always the case in cancer treatment/recovery, we try not to look too far ahead and focus on the small wins, and we had some major wins this month as we chipped away at the long list of medications.

James was an absolute champ throughout the 4 plus hour clinic visit at Lurie.  As mentioned earlier he was able to do this with anethsia... now we can tell James that he will get to go on the "donut" ride (what James called the CT machine) , versus telling him he can't eat or drink from the night prior, which was REALLY fun for all of us (heavy sarcasm).  So. Much. Better!

Unfortunately we have not seen any re-growth of James' hair.  Starting JAKAFI (medication) was kind of our last hope for hair regrowth.  75%+ of patients with full hair loss (alopecia) had regrowth with JAKAFI so we've been optimistic that it could help James.  As time has gone by we've looked more and more into the clinical results and in all those patients regrowth was seen by Week 8 or well prior...and we are about 12 weeks out today with zero regrowth.  Our team has told us to be patient and give it 6 months, which would put us at about October.   At this point, the chances seem slim, but we continue to hold out hope and pray that his hair will return.  James has been thru the ringer so we're holding onto some hope that maybe he just needs a little more time to recover.  If not, so be it.  It's been a little difficult lately as James has made comments here and there asking when he will have hair or why he doesn't have hair.  We were chatting the other day and he told me that we're both boys and boys have hair so he was a little confused as to why he didn't.  Little kids, unintentionally, can be pretty direct as well and that's going to be hard on him.  He was a bit upset after preschool one day when some other kids his age were calling him bald.  No harm intended on their part as they don't know and are just curious, but we're going to have to stay really close to this as he grows up as being a kid is hard enough with this on top of you.  That said, there are tons of people out there with other medical issues, disabilities, etc that would KILL for having no hair as their only problem in life.  And other kids that are no longer with us.  If James comes out of this thing on the other side and the only lasting issue is no hair I'll take that every day of the week.  

In the meantime, James continues to have a fun filled summer.  He loves going to TBall with his buddies, going for nighttime walks with our family and swimming with his cousins.  He also apparently loves drinks in giant cups, as you can see below (side note: James always says "The McCarthy boys are slammers" when discussing his beverages).  We are looking forward to celebrating his sister's first birthday (already!! It goes so fast) and then heading to Michigan for one more family vacation before pre-school begins.  

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.

It's been a little over a month since our last update... per usual Rita and I have been a bit behind on our updates.  Honestly this tends to happen because we're usually waiting to get clarity on a few things before updating everyone, but you'd think we would be used to the drill of not letting that stop us since clarity rarely occurs and often there are new things that pop up before we resolve the initial areas of concern that we're waiting to update everyone on.

2021 has been a challenging year thus far with new complications and issues arising on top of the old ones that we are still trying to beat and get past.  Don't get me wrong, there is still a lot to be thankful for and many great memories have already been made this year.  As much as we try to focus on the positives it's hard to not let the negatives frustrate and eat away at you with constant worry about what the future may hold.  It's increasing difficult to google "how many days since December 6th, 2018" when I sit down to write these and not feel a sense of frustration and sadness for all James has had to ensure over the last 844 days since transplant + his original diagnosis and time in the hospital for a few months preceding that... so we'll call it 964 days but who's counting.  It's also hard constantly seeing my iPhone show pictures for this time 1,2,3 years ago (I know it's trying to help me cherish the memories) and have so many bad ones scroll across my phone along with the good ones.  It's been almost a year on the don't since GVHD took James' hair so seeing those photos fade away is hard as well.  We've been really holding out hope that James may be able to at least grow eyebrows or eyelashes but it's looking pretty unlikely at this point.  He's aware of it but other kids his age haven't started to judge him yet for this or his height, etc but we know it's coming and that's hard to pre-feel the pain that he is likely going to experience growing up.  That said, we are SO THANKFUL that he will grow up... that he is here... and that he's such a funny and happy go lucky kid most days.

Despite all this, James continues to find ways to live it up and make the most of life.  We've been able to do a few sleepovers with his grandparents and cousins this year which has been amazing.  They are really terrific with James and he looks up to them more than anything in the world (well, except for his grandmas).  

At the time of our last update James has battle some pretty major GI issues - we had an endoscopy (tube down the throat, thru stomach and into the intestine + a colonoscopy done a little over 2 months ago.  The results were better than expected... the photos of the issue looked essentially like the inside of your cheek and there was inflammation but nothing overly concerning at the time.  James was put on a new, temporary, medication to help calm his GI down at that time.  Since that time we've tried to get James off this medication 3x but within a day or two of stopping it his symptoms become unbearable and he's back in the bathroom 20+ times a day and often multiple times per hour at it's worst.  The good news is that as soon as we go back on the medication it helps significantly (although helping a bit less this third time around)... but with all this going on we knew something was not right and this wasn't the solution.  

Last week James went back into Lurie's for the same procedure again to get a re-look and the results were dramatically different than last time.  There were very obvious ulcers and deterioration of his issue... needless to say as soon as we saw the photos post-procedure last Thursday we knew this was likely fairly serious and not something that was going to resolve easily.  Going into this we had a pretty good sense that there were not any really great potential outcomes... out thoughts were that it was one of the following:

(1) Infection.  We didn't think this was likely, given how long he's been dealing with this and that his GI was always been somewhat of a problem.  That said, any infection is a big deal for James so we were hopeful this wasn't the issues

(2) GVHD in his gut.  Again, not a good outcome and pretty serious.  The signs are not pointing to this as the likely outcome as it's not presenting how the Dr's would expect for GVHD

(3) Some type of autoimmune disorder affecting his gut.  This had been positioned as a potential outcome last time as well, so it was in the back of our minds.

Below are photos of the tissue from his past Thursday, and you can see rather easily it doesn't not look healthy.  We had a better understanding now why he is so uncomfortable and at times in pain.

Apologies for the photos - but it put in perspective what else James now has to deal with... on top of everything else.

The consensus is that this is IBD (Irritable Bowel Disease).  

Per WebMD (this is easier than me trying to explain it)

"Inflammatory bowel disease (IBD) is a group of conditions that cause swelling and irritation in your digestive tract, such as Crohn’s disease and ulcerative colitis. Irritable bowel syndrome (IBS) is the term for symptoms that happen when the contents of your large intestine move too quickly or too slowly.
IBD is what doctors call a structural disease. That means physical damage causes your symptoms. Doctors can see chronic inflammation or ulcers when they look at your gut with an X-ray, endoscopy, surgery, or biopsy.

Crohn’s disease, one of the main forms of IBD, usually causes pain in the lower right side of your belly. The other main form, ulcerative colitis, leads to pain in the left side of your belly."

We are waiting for some more details from his medical team to confirm the diagnosis and treatment plan... which we hope to have this week.  It was positioned to us as a likely lifelong condition and it can happen to around 1% of patients post-stem cell transplant with immune system complications.  I must admit that it's hard to take this one on the chin along with his GVHD and new lung issue that popped up a couple months ago.  The percussive vest that James has to wear twice a day shakes his entire body (while also wearing his nebulizer the entire time) and causes him a lot of stress.  In my head it's hard to not go down the rabbit hole of how many issues his stem cell transplant, which saved his life, has caused / continues to cause him.

The three treatment options we discussed were essentially:

1 - a pill that James would take daily or twice a day (not sure) to address this 
2 - a shot that we would give him every 14 days
3 - an IV infusion that he would need for a few hours every other month

It's my understanding that those meds are essentially in order in terms of least to most aggressive treatment.  It's hard to imagine they will start with #1 (but never say never) given some of the recurring issues and that this is classified as a moderate case as it's presented today (on a scale of mild, moderate, severe).  We can certainly handle any of the options and take them in stride... we're at the hospital quite often anyways so if we need to do infusions it's just one more thing on the list and they are drawing blood each time anyways so placing an IV for a few hours isn't a huge leap.  The hard thing to grasp is that these treatments could be lifelong... it's hard to not have an end in sight on this stuff and also worry about what the next unknown issues is lurking around the corner that he may have to face.

As you can likely tell by the length of this post I could go on for hours but will call it quits for tonight.  It may not seem like it after reading this but I we're all staying positive and most importantly focused on keeping James' spirits up (often with too many TV shows and the occasional Sour Patch Kid).  We appreciate all the support and will keep everyone posted once we know what the plan is.  My apologies for any typos - this one is too long to proofread :-)

Update from Rita:

Hello Team James,

We apologize for the long delay in writing James' update, but unfortunately 2021 has not been too kind to James. We are in a better place now, but our family has spent the last month and a half in and out of the hospital/specialist appointments/clinic visits, etc.  During these weeks, James has suffered from severe GI issues, headaches, fevers, lack of sleep... the list goes on.  I won't mince words.  The last month has been agony.  But, in true James fashion, his amazing spirit continued to shine through and he has come out on the other side smiling.

So much has happened in the last weeks that it is hard to keep track of all the details, but I will do my best to break it down and not write a crazy long update.  As everyone knows, James' health situation is complicated and always evolving.  Currently, we have 2 main issues that we are tackling.  There are many other issues (alopecia, skin GvHD, elevated medication doses, stunted growth) that we are monitoring and employing a more "wait and see and slowly make changes"  approach.

ISSUE #1:  Build up of secretions/damage to the right lung

You might remember that during his last hospital stay in December, James had a CT of his chest which revealed a build up of something (doctors still are not sure what) in the lower right lobe.  (We confirmed with the team that it is the right lobe, not the left as we had indicated in the last update).  The doctors were concerned that this build up could lead to infection, so the plan was to closely monitor any symptoms, including fever.  

Shortly after the last update from 1/4/21,  James developed unexplained fevers (101-103.3) and wasn't feeling great.  On 1/12/21, we were admitted to the hospital so that James could undergo a bronchoscopy to make sure there was no active infection in the right lung.  We were able to get the bronch done on 1/14/21, after three long days of sitting in the hospital with an irate and very bored 4 year old.  During the stay, the team ran many tests on James' blood and stool and ruled out all sorts of infections and viruses, including COVID.   By the time of discharge on 1/14, James had started to feel a little better.  By the weekend, James was feeling great and was even able to go sledding with his cousins. ​

The bronchoscopy results came back with no sign of active infection.  This was good news, but that still left the question of what is going on in in his lung per the very obvious signs of abnormalities on his CT scan?  Our lung doctor couldn't tell us for certain, but the suspicion is that James' lung sustained some lasting damage from the month and half he was on a ventilator when he was first diagnosed back in 2018.  She suspects the damage to the lung makes it difficult for James to fully clear mucus and secretions (something those of us with healthy lungs do all day long, without even realizing we are doing it).  The concern is that the secretions will start to pile up in the lung and put James at risk for serious lung infections.  It's a bit disheartening to see "new" damage show up and evolve in his lungs... and we're a bit fearful of the fact that these issues are popping up 2 1/2 years since his time on the ventilator.  We do wonder what it means in terms of any further evolution of lung disease...  but no one knows if this is it or if it will worsen.

So, what do we do now that we know the right lower lobe isn't functioning as well as it should?  The lung specialist recommended that James use a percussive vest and albuterol therapy targeted at the damaged part of the lung to proactively help the lung clear secretions.  Due to complications that I will get into later in the update, we weren't able to obtain the vest/albuterol treatments until this week, but so far, James has been such a trooper about the respiratory treatments. We try to make the process a little more enjoyable for him and James picks a show to watch while the vest or his dad rapidly pounds or vibrates his chest.  While all of this is happening, he also has to wear a nebulizer mask over his mouth. The goal is to work up to James wearing the vest and nebulizer for 30 minutes, two times per day... which the doctors know is a very tall order for anyone, especially a 4 year old.  James is allowing about 20 minutes at a time so far, and we are thrilled with how well he is handling it.  Of course we will do anything from James but adding another hour of treatment each day on top of all his current needs is going to be a challenge for all of us.

The plan for James' lung treatment is to monitor his lung function over the next 6 months while he continues his daily vest/albuterol treatments.  Hopefully, we won't have any episodes of coughing, trouble breathing, etc.  At about the 6 month mark, James will have another CT of his chest, and we will go from there.

Below is a video of the vest he will need to wear during these treatments.  For context, this is on the LOW setting!

ISSUE 2:  What  is going on inside James' GI tract?

After discharge from the hospital on 1/14, James had a fantastic 5 day span.  He was medically cleared to return to school and had a blast playing in the snow at Grandma Marilyn's.  He was full of energy, eating and playful.  On Wednesday, 1/20, James developed another fever.  He really began to feel crummy that night, and the next day we were back at clinic with fever, headache, and tummy complaints.  James' blood was drawn, he was given IV antibiotics and fluids, and we were sent home.  Almost minutes after leaving, we received a call that James had tested positive for COVID.  As you can imagine we were extremely surprised.  James always wears a mask out in public, at school and at the hospital- we had no idea how he could have contracted COVID.  We were also extremely worried.  We were worried not only about how COVID would affect James, a medically complicated kid with a suppressed immune system, but also terrified of how it might affect anyone James had come in direct contact with when he would have been most contagious (in the two days before he began showing symptoms, per his doctors).  James actually still had an IV in his arm when he left the hospital as we were supposed to return the next day again for more fluids as well.  Dan removed if from his arm when we got home and James as always was a champ about this.

The entire family immediately went into quarantine mode.  We monitored James closely over the weekend, and he seemed to be having only a "mild" case of COVID, with bouts of coughing and fever here and there.  But on the whole, he seemed to be improving and his fevers dissipated.  Unfortunately, by Wednesday, 1/27, James' diarrhea really began to accelerate. The poor kid was going (and this is not an exaggeration) 20-30 times per day.  It was every hour, then every half hour, then every 15 minutes.  Anyone who has ever had food poisoning or a really bad stomach virus knows how miserable this is for an adult... imagine a 4 year old boy spending hours in the bathroom every day.  The challenge here is that this went on for weeks, not a day... hard to imagine.

For some reason, James' body kept sending him signals that he had to go.  We had countless episodes of sitting on the potty for 15 minutes, getting up and getting tucked back into bed, only for James to be convinced he had to go again minutes later.   It was an awful, never ending cycle of him not only feeling terrible, but also getting very little sleep day after day after day.  We were concerned about his nutrition as well as his body's ability to keep up with all of this day after day.  James' body was telling him he had to go, causing him to strain so hard trying to empty his system.  This eventually caused his rectum to begin prolapsing again.  Dan and I had to explain to James, in a way that he could understand, that he couldn't push too hard because sometimes pushing too hard would cause part of his body to slip out a bit.  It seems too crazy to be true, but James now knows the word "rectum" and how it is a part of your body that you want to keep inside.

On top of the hours being spent on the potty, we were also noticing more and more blood in James' stool- a terrifying sight for any parent.  At first it was a few times a day - and then eventually it was every time he went to the bathroom.  We brought James back to clinic in desperation mode.  We were willing to do anything we could to help him.  The team again tested his blood and stool for anything that could explain the terrible symptoms, but no answers.  In terms of treatment options, we finally got the OK to give him Imodium, but other than that, all we could really do was make sure James did not get dehydrated and wait to be seen by GI.

When the problem still had not resolved by the following week (now 14 full days of this nightmare), we were a bit in panic mode.  We were also all completely exhausted by lack of sleep and trying to take care of 2 young kids, one with horrible stomach issues, all while trying to work and keep ourselves sane.  On top of that, we were all isolating due to the COVID diagnosis.  On 2/9/21, we met with a GI doctor who recommended that James start an antibiotic called Flagyl to help with  any possible infection in the GI system and/or help with inflammation.   The doctor explained the inflammation could be caused by GvHD (although this is still not high on the list of potential causes) or some sort of inflammatory bowel disease like ulcerative colitis.  The typical treatment for inflammation in the gut is steroids, but  James is already on a triple dose of hydrocortisone and we wanted to do everything in our power to keep him from switching back to prednisone (the higher potency steroid he was on for 18 months).   

​As a side note - in addition to this James' skin GVHD was also really acting up from time to time, creating one more stressor for him to deal with.

After 3 days of Flagyl without too much of an improvement, we went back to an inflammatory bowel disease specialist to discuss next steps.  The specialist admitted that he is puzzled by James' pattern.  James had these exact same symptoms back in December and the issues seemingly resolved on their own after about a week.  Now, we were back with the same issues, possibly caused by COVID, but possibly not.  The symptoms were the same this time around, but had lasted 19 days at that point.  

It is and continues to be a mystery.  Because the specialist can't be sure what this GI issue is, he cannot know how best to treat James.  He recommended that James undergo yet another endoscopy/biopsy/sigmoidoscopy (camera down the esophagus and into the GI system) to help confirm the diagnosis.  This was scheduled for 2/18.  The specialist did note that Flagyl could take a few days to work, but if James was not improved by Monday, 2/15, we would move forward with the test.

Thankfully, James finally (finally!!!) got some relief over this past weekend.  He started going less (more like 10 times per day) , not sitting on the potty for so long, and not having to return 2-3 times in one 30 minute span.  His appetite and energy returned, and we got our happy little guy back in full force.  Things continued to improve to the point where our GI specialist agreed that we could postpone the endoscopy until James has to go under anesthesia for his 6 month lung CT or if the GI issues return.  

While we are so relieved that James is doing better, there is  a pit in my stomach about this GI issue.  Why does it keep coming back? What is this and what is causing it? How do we prevent this from ever happening again?  Is this GI issue contributing to the lack of growth?  Right now, the doctors can only guess. The suspicion is that James has some sort of GI/gut inflammation, possibly caused by one or several of his medications, possibly caused by GvHD, possibly something else or a combination of some or all of the above.   The other hypothesis is that it could be ulcerative colitis (not good) which would be yet another complication/medical issue added to the list that James would potentially have to deal with for the rest of his life.  That said, we/the doctors simply do not know what the issue is at this point and we're hoping for more details in the coming months as we're able to see how James reacts to these new treatments and (hopefully0 avoids any further episodes.

Moving forward, we will start to wean James off the Flagyl and back down off his hydrocortisone.  We are anxious to get him on lower doses of these meds as both seem to cause fluid retention and James' belly is very distended.  After what seems like 6 months of setbacks, we are really hoping we can get James back on the road to recovery, and focus on weaning medications and stimulating growth.  

The older James gets, the more I worry that his early childhood memories will be overwhelmed with doctors, hospitals, needle sticks, breathing machines, appointments and waiting rooms.... more appointments... more waiting rooms.  Because of this, we really try to live in the moment with James and immerse him in loving, happy and "normal" early childhood experiences.    James himself is largely responsible for allowing us to live in and cherish each moment with him, as his fun loving and cheerful personality brings us such joy.  When I hear the sound of him playing and making up voices for his Scooby Doo characters (his latest toy obsession) or singing along to music (he loves to sing the Fireman Sam theme song to his baby sister), it fills me up with joy because I know he is truly feeling good.  And that's really what we want for him, to feel good, GREAT even- the way every young child should when they wake up each morning.  

For now, I am so very grateful that we have gotten about one week of those "I Feel Good!" mornings and I cherish every single one.  Hoping the trend continues in this direction.

If you made it to the end of this very long update (apologies!), thank you for reading and thank you so very much for loving and supporting our family.  

Thanks!

Rita

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.

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"