Me

“But still and forever she remains defective and defeated. Not all the spirit and ingenuity in the world, not all the substitutions or compensations..., can alter in the least her continuing and absolute loss...”

- The Man Who Mistook His Wife For A Hat
By Oliver Sacks

Eating, Swallowing

My swallowing muscles and chewing muscles have definitely deteriorated noticeably in the last 4 years or so. The first time I got something stuck in my esophagus, it blocked all other swallowing so I had to suction out saliva that built up very quickly. I went to the hospital to get it out.  That happened several more times, that I went to the hospital to get something removed.  It's a ridiculous ordeal, because the doctors always want to try things that we've already tried on previous visits that didn't work.  The treatments that did work were putting a large tube down my throat and either pushing the food down into my stomach, suctioning it out or both. Twice,out of approximately four visits, they used a large tube connected to a camera to get the food out.  The first time was without anesthetic and it literally felt like torture. The second time they used twilight sleep, so I was not conscious and it was a much better experience.  This way they also confirmed that I didn't have other problems, besides weakened muscles.  The first time, the doctor said, "Well, we ruled out cancer." I thought, "Holy fuck, I didn't even know cancer was an option to think about!"

Since then I've learned how to get things out of my throat myself with my suction machine. I just put a suction catheter down through my mouth and keep at it until everything comes out and I can swallow again.  It can take 30 mins to an  hour to get it all, but it's better than 5 hours in the emergency room.

I'm also more careful about taking smaller bites and concentrating on chewing my food, so that I'm not careless about swallowing food that isn't chewed enough.  I avoid steak, because I find it harder to chew and it takes much more effort to chew properly.  I also avoid pizza that doesn't have a thin crust and don't eat the end crust part at all. Other things I am extra careful with are shrimp, hot dogs, and any kind of meat that isn't extremely tender. I have to be careful with anything that might get stuck that wouldn't resolve itself, like meat and cheese.  Usually bread, crackers, and things like that can be broken down by saliva and if they don't block completely I just wait until it is ready to go down. I also have trouble with large pills, and try to get liquid medications if possible, so I can put them through the stomach tube.

It's Fucking Hard

I wrote this in response to a post that a woman made on a forum I belong to. The forum is a group of women who have Pompe (glycogen storage disease type 2), and we share information, offer support, and generally talk about our lives.  The woman I responded to was scared of the amount of muscle damage that showed up in a recent medical test, and was worried she might lose the ability to walk.  I wanted to share it here, because it contains a lot of my own fears and feelings about the disease.




I think all muscle damage is scary. I don't think I am speaking for myself saying that we all want to retain as much function as possible for as long as possible. I think that part of the reason this forum exists is to get each other through the fact that, muscle damage and loss of function is inevitable and frightening.

I go through a grieving process every time I have to give something up, whether it was walking, or going to university, or a hundred other different things. It's so hard.

Some days I'm sure that the progression has to stop, because it's already taken so much away; how could it possibly take more? Some days I think that if I lose another function or some specific function that I won't' be able to live with it and I think, "how can I go on?" But then it does happen, I lose it, and I do go on, and it's hard as hell.

I use the support of my family, friends, and health professionals to get through it. Some days it feels like nobody understands, and how could they possibly understand what it's like to feel your body slowly betraying you, or being so tired you can barely think. Honestly, a lot of times they can't, and that is another good reason this forum exists, because the people here can understand. We can be scared together, we can grieve together, and we can go on.