Changes at Nexviazyme Infusion #6

Hi there,

Today, I’m receiving my sixth infusion of Nexviazyme. I haven’t made any blog posts since my second infusion in late November, shortly after the change from Lumizyme. There were definitely some fits and starts getting things going but everything has been smooth sailing since.

I’m making this entry because a couple of things have changed.

First, I’ve been in regular communication with my Pompe specialists since making the switch in medications. My doctors are prescribing a measured approach to this new medication relative to other Pompe patients. My regimen thus far has involved infusion rate increases every thirty minutes until I reach my max rate. With Lumizyme, those increases were implemented every fifteen minutes and, near as I can tell, most other Pompe patients are increasing their Nexviazyme rates at fifteen minute intervals. Also, my doctors initially required a two-hour observation period following my infusions to ensure that I don’t have an adverse reaction.

After my first three infusions without incident, my doctors agreed to reduce my observation period to one hour. We’re continuing with that practice for now.

But this week, my doctors have agreed to reduce the intervals between rate increases to fifteen minutes. So my infusions start at 30 mils per hour. Then, every fifteen minutes, we increase by 60 mils to a maximum rate of 210 mils. This will reduce my overall infusion time by over thirty minutes, which is fine by me.

The more exciting news, though, is that I’ve noticed a change in my breathing strength over the past two weeks!

If you’re dropping in on this blog without having read earlier entries or some of the background information I’ve provided, I’m dependent on a non-invasive ventilator 24/7. This has pretty much been the case for the past six or seven years. Even so, there are times when I have my vent off … when I’m showering, dressing, brushing my teeth, etc. Generally, after having my vent off for even a short while, I run short of breath and can even get a bit panicked because I’m having a hard time catching my breath.

In the normal ebb and flow of living with Pompe Disease, I have good days and bad days. Some days, I feel more energetic, and things seem easier. More often, though, there are days I just feel depleted, and things seem more labored, weak, and difficult. I’m used to those peaks and valleys and just kinda ride with them.

I’ve documented elsewhere in this blog that Nexviazyme infusions produce a dramatically different short-term reaction than Lumizyme did … at least for me. Lumizyme tended to put me in a very sedated state beginning about two hours into my four-hour infusions. That haze would last through most of the day following my infusion days. Nexviazyme, on the other hand, produces a subtle but distinct improvement in my energy and mental clarity that lasts well through the day following an infusion day. Over these past twelve weeks, I’ve noticed that my overall stamina lasts a little bit longer after each infusion.

The more exciting news, though, is that my breathing strength appears to be very gradually improving. Again, I’m accustomed to the normal to-and-fro of good days and bad days. But about ten days ago, I noticed that, when I had my vent off, it was easier to breathe on my own and I didn’t feel panicked after having it off for a few minutes. At first, I attributed this experience to just having an easier day. But after it happened several days in a row, it caught my attention. Now, almost two weeks later, there appears to be a discernible pattern. With slightly improved breathing, I’m also feeling more alert and upbeat.

For the past several years, my medical team has stopped conducting Pulmonary Function Tests (PFTs) mainly because I’m ventilated all the time. If I remove my ventilator and try to run through the regimen of PFTs, it’s a given that things are going to look strained. The last time I underwent PFTs, it was determined that my breathing strength was about 23% of normal.

I meet with my Pompe specialists in the next few weeks and I believe we’re going to get comprehensive muscle tests to establish a new baseline. I’m going to ask if we should get new PFT results, also to get a new baseline. If my breathing is, in fact, improving, we need to be able to measure against a reliable score.

In the moment, though, I’m elated! I wasn’t expecting any improvement from this new medication … and certainly not this quickly. The best expected results of Enzyme Replacement Therapy (ERT) are really stability. I was simply hoping to see my clear decline over these past few years level off. That I’m actually feeling better … for the first time in years … is truly something to celebrate!

I’ll keep you posted as things evolve. But the news is all good!

Thanks for following along.

~ Brian

3 thoughts on “Changes at Nexviazyme Infusion #6

  1. How exciting I too am on my Trilogy24/7 since my falls and hospitalization at the start of Covid. I used to be able to be off the Trilogy when up in wheelchair. I’m hoping that is what improves for me! I too get panicked too. Makes it hard to eat and brush my teeth. I recently got a nose mask for daytime and that has helped , but go to my full face at night. I’m talking to my neurologist next week. I’m hopeful. Thank you!

    Liked by 1 person

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