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Update from Rita:

Hello friends and family!  We hope everyone is doing well and staying safe.  It has been a busy few months for our family since the last update (all the way back in March- back even before quarantine started!).  Details below. 

Over the past several weeks, we have been closely monitoring James' skin for any signs of GVHD flares.  Thankfully, his system seems to be handling the new immunosuppressant drug (Imbruvica) very well and we have not seen any red or dry patches pop up.  As Dan explained in previous updates, Imbruvica is actually a form of chemotherapy normally used in adults, so we had been worried about potential side effects.  So far, James has had a few bouts of nausea, but we have been able to keep them at a minimum.  We are so thankful James' system has taken to the Imbruvica, as this is key to getting him off oral steroids.  We still aren't seeing any signs of his hair growing back and he has lost most of what he was still hanging on to but he's young enough that he doesn't care.  It is a little difficult with everyone knowing or assuming he is is sick when we're out and about... but there isn't much of that going on with the quarantine lockdown anyways!

Speaking of steroids, we have been looking forward to our next monthly stem cell clinic so the team could evaluate James' skin and continue to taper his steroid dose.  We had our appointment today and are very happy to report that we were able to wean down the dose again... we're SO eager to get him off of them as it's been 15+ months already.  The goal is to have James completely off the higher potency oral steroid by the end of July. and move him to a different (and less strong/potent) steroid simply for maintenance.  This is exciting news for us, as the prolonged steroid use has undoubtedly contributed to James' lack of growth since August of 2018.  

To address this issue, James is now being followed by the endocrinology team at Lurie.  During our visit to Lurie this morning, we went to the endocrinology clinic so James could be weighed and his height could be measured.  The endocrinology team will be following his growth by 6 month intervals to evaluate additional steps we can take to help James catch up to other kids his age.  James also had a bone age x-ray and additional labs done and we are waiting on the results.  We're ager to get the results from the bone age test as it will give us some insight into his bone health and what we should expect going forward.  Once we have these, the endocrinology team can tell us what potential next steps might be (wait and watch, introduce Human Growth Hormone, etc.) to help him grow.

We also got to check in our with lung doctor, Dr. Laguna, during today's visit.  In true James form, he happily demonstrated how his lungs have been functioning by sprinting up and down the clinic hallways :)  Dr. Laguna was thrilled with his energy levels and also emphasized the major milestone he has had- it has been well over one year since James needed to be hospitalized for anything associated with his respiratory system.  Dr. Laguna mentioned this as a solid example that his respiratory system can handle other illnesses (cold, flu) without needing oxygen support.  The plan moving forward will be to continue his prophylactic (preventative) lung medications for 6 months after he is weaned off steroids.  After we reach the 6 month milestone, the team will likely want to do another chest CT to look at his lungs, and if everything looks good, he should be able to come off his lung medications.    

Our doctors also gave us the green light to allow James to go back to a preschool/daycare environment in the fall.  We are really happy they feel he is healthy enough to be in a full-day school environment.  James really loves being around other kids and learning.  All in all, today's visit was very positive and we are hopeful we will get more good news and a more definitive treatment plan from Endocrinology soon.

What else has been James been up to lately?  Enjoying his new house in Lombard!  Our family bought a house in Lombard and moved in on 4/30.  As some of you know, we had been planning to move back in 2018 to be closer to our families, give James more space and have room for our family to grow.  After James got sick, we put our move on hold to focus on his recovery, so this has been a long time coming.  

It has been a hectic few months, but the timing of the move couldn't be better- the three of us in our condo during quarantine could get stifling at times to say the least!!  James is loving having space to play with his toys and to run around.  We also got so lucky and have amazing neighbors with young kids.  While we will miss the city and Southport, it is amazing to see James being able to run around with other kids in the front or back yard.  We also love exploring the walking trails and many parks that Lombard has to offer.  

Having the additional space will also come in handy this fall... we are so excited to share that Baby #2 will be joining us in September!  James is still coming around to the idea of having a little brother or sister, but we know he will be a great big brother.  We cannot wait to meet the new member of the family!

Sending love to you all!  We are hopeful we will have more positive updates to share after our June clinic visit.  Thank you all again for the support!

Rita & Dan