It’s been quite a while, but I told myself I wasn’t going to apologize anymore for my lack of updates. Hopefully everyone understands that “no news is good news” at this point. The good news off the bat is that life for Will and the family have moved back to something resembling normal, whatever that is.
My last update was in October and while we have continued to have ups and downs, the periods of quiet in between are growing longer and longer each time. Our once three times a week appointments at Phoenix Children’s hospital are now six weeks apart. Will’s once daily pill regimen of thirty pills has dwindled down to eight a day.
On top of that, in May Will officially graduated from the Bone Marrow Transplant team, having successfully completed his transplant. It took a little longer than normal for Will due to an overabundance of caution from everything his body went through over the transplant process and the complications and medications he went through during that. They have primarily been concerned if there are any lingering Graft Vs Host Disease signs and will probably always stay vigilant on that front.
So in May we formally made the transition from the BMT Team to the IHC (Immunohematology Clinic) Team at Phoenix Children’s. The Immunohematology Clinic is a specialized clinic focused on the diagnosis, treatment, and education of children with primary immunodeficiencies. It combines expertise in pediatric allergy, immunology and hematology to provide coordinated care. My dad used to work for Intermountain Health Care in Utah so I get very confused hearing our doctors down here saying IHC!
This change brought some mixed emotions to us. Fearful for the transition but beyond thrilled that we already know/love and have worked with the doctors who head up the Phoenix Children’s IHC team, including Dr. Miller who has been a lifesaver through this whole process. She literally saved Will’s life in my eyes by identifying his RASGRP1 Diagnosis, the plan of attack for treatment to fix it, and was there with us when he was in the hospital for transplant.
It was weird to say goodbye to the transplant phase, something that has defined our family so heavily these past few years. It was October 2022 when we first noticed the red spots in Will’s eyes that became the first domino to fall in his journey. It’s amazing to look back at it and be on this side of it almost three years later, but as you can imagine, all of this has put us on edge. Were we really just going back to life as normal (there’s that word again)?
Well yes and no. During our May visit with Dr Miller and the IHC team she gave us a bit of a reality check (in a good way).
- Yes, Will had successfully made it through his transplant
- Yes, he had no functioning immune system at the beginning of all of this because of the mutation, and the transplant was the best move to fix that
- Yes, his body is showing positive signs of recovery with a once again functioning immune system
- Yes but…
- But there have only been ten cases of RASGRP1 in history and Will is the first ever (That’s right ever!) to have had been diagnosed as RASGRP1 before transplant.
So, while there is a plan in place for bone marrow transplants to help kids with malfunctioning immune systems, a plan to know what to expect and what to watch for in the future, there can’t be a plan for Will when he is literally one of 10 RASGRP1 cases.
With that reminder came the guidance that we really will never be out of the woods with Will on this, and in turn the approval that it’s okay to still be on edge over all of this. They know what to expect in a post Bone Marrow Transplant world for most patients a year down the road, five years down the road, 10 years, etc… but they can’t know that for Will and never will. He’s the author of this playbook.
The immediate concern has been resolved, and his immune system is starting to work, but just like making a baby, it takes time to build a fully functioning immune system, and it will take time until Will’s is fully functional even if we see positive trends now.
The vigilance that we have had since October 2022 will need to remain (to a lesser extent of course) and every illness or ailment will need to be looked at through the lens of Will’s unique situation. In one way it’s overwhelming to get that news, in another it’s a relief. Relief because we were probably going to feel that way anyway due to our shared experiences and the PTSD that has come from it. Relief because we have a reason to be concerned.
So where does that leave the future for Will? It will be about like it is now, just knowing that normal won’t be normal and there is no normal for any of us (you reading this included!). For as amazing, resilient and strong as the human body is, it’s an equally fragile and finicky beast. It amazes me how any of us can even get out of bed in the morning when you think about everything that must go right just to live each day.
That’s not a doom/gloom mindset or depression (although I’m sure there is enough of that to share!). Just a reality check that we must live for each moment and tomorrow isn’t promised for any of us. Never go to bed angry or without letting your loved ones know they are just that. While you would think our lived experiences wouldn’t require a reminder of that, I really do need one from time to time.
This week we had the opportunity to celebrate Will’s 13th birthday. We did another another escape room, visited a cat café (which is a café filled with cats’ thanks for asking!) and ate some cake. It’s amazing how quickly he is turning into an attitude-filled teenager but we love him even more. He is still the same Will he was when this started almost three years ago but maturing. He is still overwhelmingly happy most of the time or at least doesn’t like to show sadness. He still loves all animals and hates needle pokes although he has become an expert at coping with them. He is still packing a dry sense of humor and sharp wit, able to process things beyond his years in a scientific manner and can boil down even the most complex challenges of life into its simplest form. He still doesn’t understand why many people do the things they do, doesn’t have patience for little kids and gets overstimulated in loud places. (His ever precent noise cancelling headphones have been a lifesaver for public life and if you are ever wondering what he is listening to its been “EPIC:The musical” for the last four months). He’s still my baby, and I still love and protect the heck out of him at every turn like I do all my kids.
We continue to be grateful for everything. Life has given us a truly amazing set of challenges/opportunities to learn and grow from. I’ve said it before but we are completely different people today than we were ten years ago, five years ago, two years ago, heck I think I’ve probably changed a little since I started writing this last week. I’m grateful for all of it and it sounds masochistic but I’m thankful to have the opportunity to go through trauma and so clearly crystalize what is important in life. Maybe that is the lesson from all of this. Never be so set in your ways that you can’t learn to change. Embrace change, because that evolution and growth is what life is all about. Love those around you and never let them wonder how you feel. Overall, love everyone more than you love yourself. If the world could just take a step back from selfishness for a bit and truly love one another, unconditionally, we might all be in a much better place.
Updates going forward will probably remain spread out, but Will’s journey will never really end and so as long as this site is up, I’ll keep updating it from time to time. Thank you all for your support (past, present and future). None of us make it through this journey of life alone and that comes from someone who hates to rely on people. Another life lesson I have learned!