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Hi Everyone,
I’ve just returned from one of the most rewarding weeks I’ve ever had in my role as Executive Director of The Isaac Foundation. I’ll save updating on all that was the MPS and Adulthood Conference for another blog posting. For now, I wanted to give you an update on Isaac’s health as the conference, for me, was overshadowed by the news we received yesterday (and while I was still away) that Isaac requires and will be undergoing double corneal transplants soon.
This news was shocking and upsetting to us, but not surprising. We’ve always known that this was on the horizon - or that the possibility of this was on the horizon. But he’s been so stable in his health during the past 6 months that it left our minds as a possibility. But stable isn’t quite the right word - he’s improved since January, improved considerably. I guess this is why the news we got yesterday was a tough to take.
First off - Isaac’s being incredibly brave. Please know that. Me? Not so much, but I’ll stay strong because it’s what I have to do. He expressed very eloquently that how his eyes are important to him because he need to be able to read (he’s a veracious reader and it’s unimaginable to him that that could be put in jeopardy.) He wants the surgery to protect that hobby, which is both incredibly cute and heartbreaking.
Now - corneal transplant…what is this and why are we here now?
Kids with MPS accumulate a buildup of cellular waste in their bodies known as glycosaminoglycans, or GAGS. Enzyme Replacement Therapy (ERT) helps to break those GAGS up and clear them out of the body. But sometimes ERT doesn’t prevent the clouding (caused by the GAGS) that takes place in the eyes. And Isaac has always had corneal clouding. Quite severe, in fact, but it’s been severe since he was very young. In fact, if you were to look at Isaac’s eyes today, they don’t have much colour - they are grey due to the clouding.
Isaac’s glasses have corrected his vision over the course of the past numbers of years. The clouding has continued to get worse - to the point where the doctors haven’t been able to see into his eyes for about 2 years. But still - his vision has been stable. A big change happened, however, over the past 6 months. Vision in his left eye has deteriorated considerably - 20/30 down to 20/80, and this is quite concerning. The result? A decision to move on with the transplants that we thought were still a number of years off, in the very least.
Doctors are very good at doing this surgery, though I have to admit that I’m still very scared and worried. But our MPS community is amazing, and many parents are rallying around us with love and support - something I’m truly grateful for. And Isaac’s friend since childhood, who also suffers from MPS, has had both of her cornea transplanted. Her family will be a great resource for us as we embark on this latest battle, and she will be able to talk to Isaac about what to expect.
And quite serendipitous - when I arrived home, there was an invitation in my email for me to tour one of the best (if not THE Best) transplant clinic for kids with MPS in the US. I’m excited to attend and, perhaps, garner a bit of information on the process along the way. The clinic specializes in bone-marrow transplants and stem-cell transplants, but they be a wealth of information for me for this comparatively smaller and less serious procedure.
Anyhow - I feel a tad better now that I’ve written this and the decision has been made. It was hard to get the news while I was away. All I wanted to do was hug my boy, struggle together with my family, and be here for each other when we needed it most. It was a tough night last night, and a long flight home. But as soon as I arrived at the school to pick up my boys, they both jumped into my arms and hugged me for what seemed like an eternity. And Isaac looks good, and strong, and - as he always does - brave. He can do this, and so can I.
Isaac shared the news with his best friend at school, Amy. I’m so thankful that he has someone he can trust to talk to, and I’m sure it made him feel better to share the news with his friends.
I’m listening to Danny Michel as I type this blog update - poignant because he’s playing at our upcoming Gala For A Cure. The song below is called “Just The Way I Am”, and it’s providing the perfect soundtrack for my frame of mind right now as I think about Isaac, his bravery, and the joy I know he takes in having a supportive friend to talk to when things get tough. It’s below for you to listen to as well - it really is a beautiful (and perfect) song for this posting.
Thanks for letting me ramble on. I’ll update with more information when I can. Thanks for always being here for us and our kids.
With Love,
A.