Infusion Day 1.8

5:30pm (PST) – 15 November 2021

Home Sweet Home

My first Nexviazyme infusion is complete and a success.

I completed my two-hour observation period at 5:00pm and am now home and unpacked from a very long day.

I’ll debrief further tomorrow. There are some very noticeable differences already between Nexviazyme and Lumizyme … rather positive ones, I think.

For now, though, I’m going to settle in … turn on Monday Night Football … and call it a day!

I’d like to thank all of you again for the good wishes, affirmations, and encouragement today.

#LetHopeFlow

~ Brian

Infusion Day 1.7

3:00pm (PST) – 15 November 2021

Nexviazyme Infusion #1 Is On The Books!

My second bag of meds just finished up. I’m now starting my two-hour observation period.

They’re keeping my IV in with a dextrose solution drip for the first hour of my wait. This is to keep my infusion line open in case I have a reaction. That way, they can immediately infuse me with Solu-Medrol. I rather doubt that’s going to happen. Today has gone very smoothly.

It looks like my total infusion time on this new drug, even with the slower increases in the first hour, was just about four hours. By comparison, the total infusion time for my Lumizyme infusion was about three hours and forty minutes. So pretty comparable.

I’ll admit, though, that I’m not feeling particularly energized. I had a lot of energy invested in this big day and I’m pretty tired. But I’m also feeling like today was a big success.

I’m sure gonna sleep well tonight!

Thanks again for all of the good wishes on social media, emails, and messages today.

I’ll plan to do one more entry to end this long day, when I’m settled in at home. Fortunately, I live less than ten minutes away from the infusion center.

~ Brian

Infusion Day 1.6

2:00pm (PST) – 15 November 2021

In the Home Stretch!

About an hour ago, we completed the infusion of my first bag of Nexviazyme. Remarkably, it took just two hours and twenty minutes to finish that half of my overall infusion. When infusing with Lumizyme, my first back typically took about two hours. Because we were using a slower infusion rate with Nexviazyme, I had expected that first back to take considerably longer.

Typically, my second bag of Lumizyme would take a little over an hour and half. I’m now almost an hour into my second back of Nexviazyme. So we’ll see how long it take to finish this up.

This first day on the new drug requires that I stay at the infusion center for a two-hour observation period following the completion of the treatment. So I have quite awhile yet to go today. Even though I’m not that excited about a two-hour wait afterwards, I’ll be feeling a great sense of satisfaction in having gotten through this first treatment.

Timing aside, all is going well with this first infusion of Nexviazyme. All of my vital signs are stable and at good levels and I’m feeling fine.

There has been so much anticipation around this day and I’m sure I’m going to be pretty tired when I get done today.

My spirits are really high, though, and I’m looking forward to getting home this evening after this long day.

Infusion Day 1.5

12:15pm (PST) – 15 November 2021

I’ve reached my maximum infusion rate of 210ml per hour.

All vitals are good.

I’m feeling good too!

I’ll stay at this rate until this first Nexviazyme infusion is done. Probably in an hour or so, I’ll reach the end of my first bag of medication and we’ll switch me over to the second.

Hope is Flowing, big time!

~ Brian

Infusion Day 1.4

11:40am (PST) – 15 November 2021

Just completed my first hour of my first Nexviazyme Infusion!

All systems GO!

We just increased my infusion rate to 150ml per hour. In a half hour, I’ll be at my maximum rate.

All vital signs are good!

The hospital pharmacist just stopped by to say hello. She is relatively new to Sierra Nevada Memorial Hospital but we’ve met and talked before. She reported that mixing Nexviazyme is so much easier than Lumizyme. Both of these medications need to be reconstituted by hand. Lumizyme was packaged in smaller vials and the pharmacy had to hand-mix forty vials for my dose. Nexviazyme is packed in larger vials so the pharmacy only has to mix about half that number. So they’re very happy about that. The Pharmacist also told me that my second bag of meds is already mixed and delivered. So when this first bag is done, we’re ready to move right over to the second. No waiting!

My first four years of Lumizyme infusions were administered at UC Davis Medical Center in Sacramento. They’re a huge operation and have three separate infusion centers. During my time with them, they were infusing eight or nine Pompe Disease patients as well a several with other Glycogen Storage Diseases (e.g., Fabry Disease, Gaucher’s Disease). They were a well-oiled machine at UCD.

Since I moved back home to Grass Valley in 2016 and relocated to Sierra Nevada Memorial Hospital, I’m the only patient at this hospital with Pompe Disease (or any GSD, for that matter) and it’s a big deal for this relatively small hospital to be treating me here. Over these five years, all of the infusion nurses, the pharmacy staff, and the hospital administrators have taken a real interest in my case, my care, and these treatments. They’ve been remarkably embracing of treating me and I’ve made a lot of friends here.

When I lived in Chico, the local hospital – Enloe Medical Center – refused to take on this medication or to administer my infusions. That decision was based at least in part on the exorbitant price of these infusions – roughly $75,000 each. The fact that my hometown hospital didn’t bat an eye at taking on this treatment and the way they’ve always worked tirelessly to accommodate me has always made my infusions here seem special.

As I mentioned in an earlier post, there’s a lot of excitement here today as we start this new treatment!

Feeling very grateful today!

~ Brian

Infusion Day 1.3

11:10am (PST) – 15 November 2021

First 30 minutes completed without incident.

Infusion rate increased from 30ml to 90ml per hour. We’ll go another 30 minutes at this rate and, if all goes well, we jump up to 150ml per hour. The max rate for this infusion is 210ml per hour. When we hit that rate, we stay there until the infusion is done.

We take my vital signs at each rate increase – blood pressure, heart rate, temperature, oxygen saturation levels. Everything is in normal range and I’m feeling great!

All ahead full!

I’ll check back in 30 minutes at my next increase.

btw … thanks for all of the supportive comments on FB today!

~ Brian

Infusion Day 1.2

10:40am (PST) – 15 November 2021

Nexviazyme Infusion #1 Underway!

I’ll infuse at 30 ml/hr. for 30 minutes then ramp up to 90 ml/hr.

This is a slower rate than I was using with Lumizyme. My increases were every 15 minutes on Lumizyme but we’re starting with a slower rate on this first Nexviazyme infusion, just to be safe.

Fingers Crossed!

These past two years have led to this moment … and we’re off and running!

#LetHopeFlow!

~ Brian

Infusion Day 1.1

10:20am (PST) – 15 November 2021

I’ve arrived at the Infusion Center.

My only premedication today is Tylenol, which I’ve now taken.

My IV is started and I’m on a drip of dextrose and water. I believe we run this dextrose drip for 30 minutes before beginning the actual infusion.

My first bag of Nexviazyme has been mixed and delivered so it’s ready to go when we reach 30 minutes. I’m a big guy. I’m 6’4” and weigh about 250 lbs. Because this medication dose is mixed based on body weight, my overall dose is relatively larger than the average. These enzyme infusions are very viscous and are known to clog the inline filters between the bag and my arm. Because my dose is so large, they divide my infusion into two bags, each with its own filter. That reduces the possibility of clogging the filters … which would require that we halt the infusion and replace the filter.

I had the timing of Lumizyme infusions down to about five minutes. Today, we start a whole new protocol, so I have no idea how long this is going to take.

There’s quite a bit of excitement at the Infusion Center today. I’ve worked with this team for over five years, and they are all as invested in the start of this new treatment as I am!

More later!

~ Brian

Infusion Day 1.0

8:10am (PST) – 15 November 2021

I’ll be leaving for the infusion center in about 45 minutes.

And as I sit in the quiet of my office, I can’t help but reflect on the path that has brought me to this place … this moment.

I was diagnosed with Pompe Disease in May 2012 after living 26 years believing I had Muscular Dystrophy. That discovery opened the door to a treatment that could stop this progressive condition from worsening. I received my first Lumizyme infusion that September.

I remember the enormity of that September day. I recall feeling like my dreams had come true. I had literally woken up one day to learn that I no longer had MD. I had something else – something called Pompe Disease – but this was treatable. I sat in that infusion center and received my first Lumizyme infusion and felt, like I do today, that things were about to change … that I was given a second lease on this life that I cherish so deeply.

Every two weeks for the past nine years, I’ve returned to an infusion center to receive an enzyme replacement. And these Lumizyme infusions have lived up to their promise. They saved my life from the rapid decline I was experiencing in 2012. I’m here today, writing this entry, because of Lumizyme. These treatments stabilized my condition and gave me a decade of life I otherwise would not have had.

A little over two years ago, though, that stability began to falter. The progressive weakness that had been an everyday part of my life leading up to 2012 started to return. Very slowly over a series of months, I wasn’t able to reach as high as I had been. My grip strength was failing, and I began to drop things more.

I promptly reported these experiences to my Pompe specialist. I came to learn that it was not uncommon for Pompe patients who had been receiving Lumizyme for many years – so-called long-haulers – were increasingly reporting a plateauing effect. The stability they (no, we) had been experiencing began to fade and the muscle weakness began to creep back in.

I learned that many Pompe patients responded to that plateauing effect by increasing their Lumizyme dosage. Some began to infuse weekly; some began to receive double doses. Despite my urgent pleading, my Pompe specialist wouldn’t consider making those changes for me. As I’ve had to do at several junctions in my life, I took it on myself to pursue some changes. I contacted the leading Pompe specialists at Duke University and consulted with them. They provided me with the latest published papers on this plateauing effect and how to adjust dosages to address the problem. I provided these studies to my Pompe specialist – to no avail. She continued to delay.

About a year ago, I found a new specialist. And for the past year, we have compiled all new baseline measures in anticipation of the release and approval of a new enzyme replacement therapy. Originally called Neo-GAA in clinical trials, that new drug was approved three months ago and given the name Nexviazyme. A month after Nexviazyme received FDA approval, I met with my new Pompe specialist, and she approved me to transition from Lumizyme over to this new drug.

These past couple of months have involved a lot of medical bureaucracy … approval of the new drug by my hospital … insurance approvals … billing approvals … and volumes of paperwork to complete each of those steps. My first Nexviazyme infusion was originally scheduled for October 18th. That was delayed to November 1st. And that, in turn, was delayed to today – November 15th. This past Thursday, in the final hour before my hospital had to order my medication for today, the final approvals came through and opened to door to today’s Nexviazyme infusion.

In the next couple of hours, I’ll begin that infusion. Today will be my 238th enzyme replacement therapy. Today will be my first Nexviazyme infusion. I’m confident all will go well today. Even so, I can’t ignore the enormity of this moment. Everything is riding on the success of this new treatment. It’s not an overstatement to say that today marks the threshold of the rest of my life.

Time to sign off. Time to leave for the infusion center. My next entry will be from there … with an IV in my arm … and a bag of Nexviazyme hanging from a post next to me.

Off I go!

First Nexviazyme Infusion Day – Live Updates.

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