Greetings!

Today, I’m settled in at the infusion center and am currently receiving my second dose of Nexviazyme. For details about how the first one went, feel free to browse my blog entries from two weeks ago.

In short, though, I had a very positive reaction to that first Nexviazyme infusion. Granted those are immediate reactions. The long-term efficacy of this new enzyme replacement therapy will be determined over time. After I started Lumizyme in September of 2012, it took a few months before I began to notice any differences. But having an immediate positive reaction after that first Nexviazyme infusion was certainly a nice “shot in the arm”!  (… a little IV humor, there, folks!)

Since that first Nexviazyme infusion two weeks ago, I encountered another bureaucratic bump in the road. It was fortunately resolved right before the Thanksgiving holiday, but it came right down to the wire again.

Before we began this transition process, it had been quite awhile since I’d been weighed. And since the dose of my enzyme replacement therapy medications is based on my body weight, it was essential that we had an accurate weight. The last time I was accurately weighed – down in Sacramento at UC Davis Medical Center – was over eighteen months ago, at which time I weighed 250 lbs. The method we used for weighing me in Sacramento, since I’m unable to stand, was to maneuver my power wheelchair onto their flat scale and subtract the known weight of my chair (443 lbs.). Since that last weigh measurement, I’ve gotten a new wheelchair, so we hadn’t yet calculated its weight. I haven’t been down to Sacramento since then, where they have the gear to lift me out of my chair and weigh the chair. My local hospital doesn’t have such a scale.

At my local hospital, we tried a couple of different methods to get my weight. Over two weeks, we got two different weights, 30 lbs. apart. We realized that neither of those weights could be considered dependable. A week ago, my local hospital acquired a reliable scale, and I went in and got weighed – 238 lbs. (Kinda nice that in two years of Covid-induced isolation, I’ve actually LOST 12 lbs!). That’s the good news!

The bad news was that this new weight measurement required that my medical orders needed to be revised. This, in turn, set in motion another tedious chain of emails, faxes, and multiple parties (my Pompe Specialist, my Primary Care Physician, the Infusion Center staff, and the hospital pharmacy) to connect the dots, order my medication, and have everything in place for today’s infusion.

But as mentioned, it all came together late last Wednesday – the day before Thanksgiving. So here I am, receiving my second Nexviazyme infusion with the correct dose for my body weight.

There’s peace and harmony in the mountains! I’m about half way through this treatment now and my observation period has been reduced from two hours to one hour. So God willin’ and the creek don’t rise, I’ll be home while the sun is still shining.

# LetHopeFlow

~ Brian