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"

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!