BUSTING AT THE SEAMS TO SHARE THIS

It's time to share our big, BIG news with you all. Colby will receive his first Sprinraza injection on September 11^th, just a couple of weeks from now! How about them apples?! I've known about this for a couple of weeks. However, Cincinnati Childrens called once to reschedule. Plus I had an over-the-phone consult with Anesthesia yesterday, and wanted to make sure there were no surprises with that. So I've been sitting on it, making sure it is a total go, and it seems to be. Oh my, the thoughts and emotions running through my little brain right now!

Here's how this will all go down. Colby will get 4 “loading doses” of Spinraza, the first being Sept. 11. Then 2 weeks later on Sept 25. Then a couple weeks later on Oct 12 and his 4^th loading dose is scheduled for Nov 15^th. Yep, we're going to be very, very busy in the near future. After the loading doses, Colby will receive the drug every 120 days forever. Ok, here's the part that freaks me out: the drug is administered via lumbar puncture (LP.) Couldn't just crush up a pill and flush it through Colby's g-tube, could we? Nope, of course not, that would be too easy. The procedure will be done down in the Interventional Radiology (IR) department. I've heard wonderful things about the IR doctor who has given other kiddos this drug from other SMA moms. They've been so busy down in IR giving Spinraza that he has also been training his fellow to do some of the LPs. But I know for a fact the doctor, not the fellow, will be administering Colby's Spinraza.

And how do I know this? Let's just say oftentimes nothing is easy with Colby. Most patients receive this drug with no anesthesia. They lie on their sides or back, are given only a local numbing cream to prepare to get the LP. Well, if you remember, Colby had spinal fusion surgery a few years ago, meaning of course his spine is FUSED. So the trick is to find an opening big enough in that fusion to administer the drug. The IR doctor has looked at Colby's back/spine x-rays and consulted with the ortho doctor who did Colby's fusion. He has said that for Colby, this will be a “very difficult procedure” and that he will be handling it, not the fellow. And Colby will need to have anesthesia. That brings up another can of worms of worries because SMA patients aren't supposed to fast, and of course when you're having anesthesia you have to do that. I've been in debate with the neurologist on whether or not Colby needs to be admitted to the hospital the night before to get PPN for the procedure. She insists he doesn't need it. I insist he does. So it appears we've reached an impasse. She called me yesterday and said now don't worry, Amy, you'll see everything will be fine. I cannot stand when people talk to me like that! Of course SHE'S not worried, it's not HER child undergoing anesthesia and having a lumbar puncture procedure done. I'll be so freaking glad to have this first Spinraza done!

I keep talking to Colby about all this. Trying to reassure him that many other kiddos have had this done and that I would never agree to something for him unless I thought it would help him. People keep asking me how he feels about it. Honestly, it's hard to tell. I've told him several times if he's scared or has questions to please use his eye gaze and let me know. That hasn't happened, so I guess I'm to assume he's ok with it.

What a way to end SMA Awareness Month, huh? To share the news that Colby will soon be receiving a drug to treat his SMA is still mind blowing to me. We've waited 15 years for this. Honestly I thought there would never be a treatment for SMA during Colby's lifetime. And here we are, just a couple weeks away from Colby receiving the first and only FDA approved drug to treat SMA. Yes, I'm scared to death about the whole anesthesia/LP stuff. Yes, I'm scared because we don't really know what to expect in improvements for Colby. We're rolling the dice and taking a huge chance. But I can't let me fear take over the decision to do something positive for Colby. And I certainly can't let Colby sense that fear. So, we're moving forward with Spinraza, and I'm praying I'm making the right decision for Colby. We'll soon find out, won't we?