I’m sitting at home in the warmth of my office, looking out my window at the soaked lot of my apartment complex. I can see bright yellow and red leaves scattered over the dark pavement after a night of rain – real rain – and the first real showers the Sierra Foothills have seen since April or May.
In the next hour and a half, I’ll be venturing out into the weather to make the five-minute drive to the infusion center (the Ambulatory Treatment Center [ATC]) at Sierra Nevada Memorial Hospital … like I’ve done every other Monday for over five years. That every-other-week ritual followed four prior years at the University of California Davis Medical Center (UCDMC) Adult Infusion Center. I visited the UCDMC Adult Infusion Center on its opening day to receive my very first Lumizyme infusion in September 2012.
And here, just over nine years later, I will today receive my final infusion of Lumizyme – the enzyme replacement therapy that has extended my life. I live with a rare neuromuscular disease called Late Onset Pompe Disease. For the 26 years prior to 2012, I lived with the belief that I had some non-specific form of Muscular Dystrophy. And before that, I lived with confusion and wonder about why my body was weakening when everyone around me seemed “normal.”
It’s been a long road leading to this day.
Today will be my final Lumizyme infusion because, in two weeks, I will be one of the first longtime Lumizyme users to switch over to a new medication – Nexviazyme – that will, we hope, extend my life and wellbeing for another number of years. In the last two years of the past nine years that I’ve received Lumizyme, the stability I experienced the first seven years has begun to wane. These last two years have been marked by the gradual onset of weakness again. My shoulders, arms, neck, and diaphragm have grown weary, and I’ve lost range of motion. I’m no longer able to reach as high as I could just two years ago. Once I have my grip on an object, my arms are barely able to deliver that object from a counter or tabletop to my lap. In these past two years, I’ve learned that many longtime users of Lumizyme have also experienced this “plateauing” effect where the effectiveness of these treatments has leveled off and begun to fade.
For these past two years, we – the Pompe Disease Community – have waited for a new treatment (originally called NeoGAA) to successfully pass through clinical trials, receive FDA approval, and come to market. That process concluded just two months ago and the first Pompe patients are lining up to make the change from Lumizyme to what is now being called Nexviazyme.
I was scheduled to make that transition today. However, due to the many bureaucratic hoops of hospital and medical administration, insurance approvals, and the overwhelming expense of these treatments (roughly $75,000 per infusion), the necessary authorizations were not in place in time to order todays medication. Since that decision was made this past Thursday, those issues have since been resolved and I will receive my first Nexviazyme infusion two weeks from today – on 1 November.
Even though that milestone transition has been postponed for two weeks, today is not without ceremony. As I write this entry, the pharmacy at my hospital is mixing today’s final dose of Lumizyme – a lengthy process that involves reconstituting by hand, forty vials of medication in a saline solution. Soon, they will start my IV and I will begin the four-and-a-half-hour infusion process that has kept me alive these past nine years.
Today, the first real rain in many months has drenched the Sierra Foothills, hopefully ending a treacherous fire season that has cost our Earth and many of its inhabitants so much. Life is being restored to our area. And on this day, I receive my final life-saving infusion of Lumizyme – a treatment that has allowed me to be here sharing these thoughts. Today is a day of gratitude.
Two weeks from today, I’ll enter a new chapter in what has been a lifelong journey – the Journey of a Pompe Warrior.
And so it is.