Day +966

(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.  

I know this has been a longer read, so I will leave you with a quick anecdote that made my day, month, maybe year!

We were watching the Olympics together the other night and it happened to be showing the end of the men's triathlon.  We were cheering on Kevin McDowell, who was having the race of his life, and eventually placed 6th overall! Kevin had the fastest time of any American to compete in the triathlon at the Olympics... ever!  Some of you may know that Kevin is from Illinois and is also a cancer survivor.  Dan and I were talking about Kevin with James, and pointed out that Kevin had to be very sick years ago.  We emphasized that he was able to not only recover, but thrive and become stronger than ever.  James took it all in and then said "so, his sickness actually made him better?"  We agreed, absolutely buddy, his sickness made him better.  Then, James said, "do you think I could do that someday?  I love to run and I am really fast."  

You can do anything James! We love you and will be with you every step.

We'll leave you with James' impromptu shirtless performance (video below) of "I'm Still Standing" from his favorite movie for 3 days, Sing.  It's a fitting song given his journey.

We hope you are all having a great summer.  Thank you for all of your love and support.