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My last surgery was 2 years ago and my very first surgery was when I was 12 years of age. Many of my procedures were minor. For example, kidney stone surgery, which is something I’ve had a few times since my body is prone to kidney stones. I’ve always healed very well and literally every surgery I had was a success. But before every surgery, I always had many questions and anxiety, not from me but from my doctors. 

Here’s the thing about having surgery when you have Pompe disease, your surgeon doesn’t necessarily know anything about your disease. And here’s the thing about having surgery when you have Pompe and are on 24-hour ventilation, your entire surgical team is very nervous about operating on you because they don’t know everything about your condition. All your doctors will come together sharing important information about you before your surgery, whatever your surgery is, but chances are they are going to get a little frustrated looking for the answers to their questions.

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Instead of going over all my surgeries, I’ll review my last surgery, which was a Mitrofanoff procedure. That’s when an entrance is created in the lower belly pelvis area to allow a catheter to enter your bladder, creating a way to urinate. This is a very unique procedure. I have 2 ways to urinate. It’s a lifesaver to me personally, and the surgery was very successful; the healing was tough, but it was worth it. 

Before my surgery, I had conversations with everyone that was involved in the operating room, but the only thing they were really concerned about is my breathing. That’s a big thing for me in my case, and you know, during any surgery, you have a team monitoring you and one specific person watching your oxygen level and breathing. I wanted to make sure I voiced everything I could about my ventilator and the settings and told everyone how to use it.

They did mention to me that I might wake up with a breathing tube in my mouth which I actually did, and it scared me a little not to be on my usual breathing machine but a tube down my throat. I had those before from previous major surgeries, and all I could remember is that uncomfortable feeling and pain from getting that tube pulled out. I never know exactly how my surgeries go because all I’m ever told is that everything went well, which is good enough for me.

Pompe disease is rare, but it’s not a complicated disease. Maybe I’m just saying that because I live with it, but to me, the issue is pretty standard. Muscle weakness. That’s the main issue. My muscles are weak, and now the reason my muscles are weak is where the complexity may come in. I think doctors look up my disease and read maybe a couple of paragraphs on it and think they understand it. And maybe they do.

However, it always has to be considered that every patient is different. I cannot say this enough, every patient is different even when we share the exact same disease, and even if we were diagnosed at the same time, our bodies are different. So even though doctors know health and treatment, it’s important that they are aware of your individual health and treatment. When they pulled the breathing tube out of my throat after my surgery, they didn’t even have my ventilator on and ready to put on me. I had to wait as I’m struggling to breathe while a nurse figured out how to turn it on and put the mask on my face. That came from a nurse assuming she knew what she was doing and that I was just like any other patient she dealt with before, so yes, this goes for nurses as well: treat me as an individual.