As a patient with Pompe disease, I have been doing enzyme replacement therapy (ERT) with the drug Lumizyme® for about 7 years. I will always remember my very first infusion.
I was admitted to the hospital because the possible reactions from the drug made us take serious precautions. Doctors wanted to monitor me overnight and see how my body reacted. My clearest memory is waking up the next day in the hospital bed with the sun shining through the window and feeling this burst of energy. It’s hard to explain to someone who doesn’t have Pompe, but if I have to try to explain it, we normally feel like we had run a marathon and we’re tired and don’t have the strength to do anything.
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So after my first infusion, it felt like the complete opposite, like I could run a marathon and had the strength to do anything. Even though, of course, I can’t walk and still need a ventilator. But just being able to feel that energy was very satisfying – satisfying enough to convince me that ERT was something I needed.
I had my first infusion recently with the latest drug for Pompe, Nexviazyme®. I have spoken to others who have already been on the new drug to see if they have seen a difference in their body or if there were any side effects to be aware of. Talking to others about the infusion before getting it definitely scared me a little.
Everyone was having different reactions; some would break out in hives, some had an upset stomach, some had trembling in their hands, and everyone had a different reaction. Some even said there was no difference between the old drug and the new drug, while others saw a clear difference. I’ve always felt like my body reacts differently than everyone else’s, no matter what medication I took. And that feeling alone is what still made me excited about starting a new drug, but at the same time, cautious.
It took me months to find the right infusion center and the right transportation because they didn’t want me going there in my wheelchair and going back home in my wheelchair. There were concerns about possible reactions on my way home in my chair, so the safe way for us to do this was going by ambulatory. That means being transported by stretcher. I’m not sure, but I don’t think that’s common at all. However, I never asked anyone about their transportation to treatment centers, so I’m not sure.
I get there in the morning. They put me in a small emergency room instead of an actual hospital room. It seemed like they were ready for me because everything was on time; they checked my vitals and gave me premeds right away. With my home infusion, I’m given my premeds with a syringe through my port; in the infusion center, it’s given through an IV bag, so of course, it takes a little time, but only an hour which wasn’t bad. So once that was done, they gave me the Nexviazyme® right away.
Within the first 10 minutes of getting the new drug through the IV bag, I felt the medicine. In a weird way, I could smell and taste it, and I thought soon I’m going to have a reaction or at least vomit because it was a pretty nasty taste. Even though I couldn’t exactly taste it in my mouth, I still could taste it. But thankfully, I didn’t throw up, I had no reaction, and I just went to sleep. So after staying a couple of hours after the infusion to make sure I didn’t have any reactions, I went home, ate dinner, and then went to bed.
And that was my first infusion with the new drug – smooth and easygoing, the opposite of what a few others experienced. I think it’s good to share experiences with treatment, but it’s also good to understand that you might experience different reactions from everyone else. I can’t say I see a difference just yet, for sure, but I think I see a positive change in my breathing.
