Well, my friends, the long wait appears to be over.

After completing just over nine years of Lumizyme infusions (237 infusions!), I’m scheduled to receive my first infusion of Nexviazyme tomorrow – 15 November 2021. Until three months ago, Lumizyme was the only FDA-approved treatment for Late Onset Pompe Disease; Nexviazyme received FDA approval in August of this year.

I’ll arrive at the Ambulatory Treatment Center (ATC) at Sierra Nevada Memorial Hospital at 10am (Pacific). I’ll receive a simple premedication of Tylenol; I was able to talk my team out of administering a preliminary course of Benadryl. They’ll have Benadryl and Solu-Medrol on hand in the event of an adverse reaction. After receiving the Tylenol, we’ll start my IV with a drip of dextrose and water. This is an interesting change from the protocol for Lumizyme. Lumizyme is reconstituted in saline whereas Nexviazyme is reconstituted in dextrose (a sugar water solution).

I’ve communicated directly with three fellow Pompe patients who have made the switch from Lumizyme to Nexviazyme and all three have reported an energetic boost after their first Nexviazyme infusion – likely a result of the sugar water solution. It will be interesting to experience that possible aftereffect. I’m typically in a very subdued and quiet space that begins about an hour into a Lumizyme infusion and continues for the rest of that day and the following day. If an energy boost accompanies this new treatment, that will be one of the first changes I’ll notice.

I have a long relationship with the folks at the ATC and the pharmacy at Sierra Nevada. I phone them at 7am on infusion days and confirm that I will be there by my scheduled time. The pharmacy staff begins mixing my medication right then so it’s ready to go when I arrive. All systems are in place for that to take place tomorrow as well.

After starting my IV tomorrow and receiving a 30-minute drip of dextrose and water – enough time for the Tylenol to activate – I’m anticipating that my first Nexviazyme infusion will be flowing by about 11am (Pacific).

I’m bringing my laptop with me tomorrow and plan to provide some blog updates as this first infusion rolls out.

I’m very excited about this first Nexviazyme infusion! I can still remember the day that I received my first Lumizyme infusion in September of 2012 and the overwhelming sense that I was reclaiming my life. This threshold on my long road of Pompe Disease diagnosis and treatment feels like that all over again.

I’d like to thank the many members of my medical team who have worked so hard and collaboratively, together and with me, to get us to this moment. I’d also like to thank my family and my friends who have supported me through this crazy process. Most of all, though, I’d like to tip my hat to my fellow Pompe Warriors. This is an extremely rare disease and, as such, there aren’t many of us in the world. We are a community that sticks together, helps each other out, lifts each other up, shares information and experiences, and takes care of one another. No one would ever wish to be diagnosed with this decidedly unkind disease. But if there is one benefit, it’s that Pompe Disease gains you immediate entry into the Fellowship of Pompe Warriors. No one could ever ask for more.

“Alone, We Are Rare – Together, We Are Strong.”

More tomorrow as I begin the next stage on the long trail of a Pompe Warrior!

#LetHopeFlow

~ Brian