First off I need to explain what Scleroderma does to the esophagus. Most of the esophagus is made of smooth muscle tissue; this tissue creates waves called peristalsis that move food along to the stomach. At the bottom of the esophagus is the Lower Esophageal Sphincter (LES) which is essentially the door to the stomach. Its purpose is to clear esophageal content into the stomach, and keep acid from splashing back into the esophagus. In Scleroderma thick fibrosis builds in place of your smooth muscle tissue causing extreme atrophy, or weakness, of these important muscles. With this atrophy your muscles eventually shut down completely, leaving your esophagus unable to move food without the help of gravity, and in severe cases, weakening so critical the LES can no longer empty the esophagus contents into the stomach properly causing massive back up in the esophagus. Typically these findings can only be found when a patient is lying down flat, though in end stage motility disease they can be found even when a patient is standing straight up.
This can all be documented by a study called a Fluoroscopy Esophagram, also known as a Barrium Swallow. Basically, it is a giant video x-ray that watches barrium move through your esophagus. I have had many, many, many of these studies over the last four years since my esophageal motility is at such serve end stage involvement. All the radiologists find my imaging incredibly intriguing! lol Today I had the pleasure *insert sarcasm* of having yet another one of these lovely tests done to asses my current situation as I seem to be aspiration more frequently then normal. The radiologist and his team got me all ready for the study and explained the procedure, I expressed to him that I wouldn't be able to do the laying down part of the test and he asked me why. I told him my esophagus has absolutely zero peristalsis to which he chuckle a bit saying "I am sure that's not true! Everybody has some! We will get you better numbers than zero miss." I just shook my head and asked them to start the test standing up. We started imaging with a tiny teaspoon of barium -- then they asked me to swallow. "Swallow honey!" They yelled again, "Swallow!" Well, I was swallowing. "Ohhhh," I hear from the radiology room. "Wow, her esophagus doesn't work at all, are you seeing this?!" I watched as all the interns huddled into the room to view my apparently very exciting failed organ.
They asked me to attempt to swallow another teaspoon of barium for those who missed it -- that was a mistake. By the second teaspoon I started coughing, burping, and grabbing my chest. "Oh wow, we have to stop," I hear from the other room. My bum food pipe which has no muscle tone was unable to empty that barium into my stomach fast enough and it was now all backed up into my esophagus. We stopped the test and the radiologists and physicians had me sit down to rest while we waited for the ounce of fluid to move slowly into my stomach. Ten minutes later, I finally had passed the fluid from my esophagus to the stomach -- which should happen instantaneously. If you do the math: in order to not aspirate fluid into my lungs I would need to space drinking 1 cup (8oz) of water over an 8 hour period. Insane.
This explains all my recent aspiration; anytime I consume more than about one ounce of fluid my esophagus backs up almost all the way to my upper esophageal sphincter. All the doctors in the room suddenly handled me with 'little kid gloves' and puppy dog eyes, expressing how sorry they were for the "torture." I just kind of laughed in between coughing fits because I had already told them how this would end, they just refused to believe me. After that they sent me on my way telling me to never have more than two teaspoons of anything at one time or I would backup my organs and aspirate. Awesome. Somehow my esophagus has gotten even worse then it was two years ago. I had no idea you could get worse than zero. Urg. So if you're wondering why I am tube fed, it is because my esophagus sucks and cannot handle more than an ounce at a time without ruining my lungs -- and they are already pretty good at ruining themselves! lol
On a happier note I have a date for my first Stem Cell Transplant Consult! August 10th I will meet a physician at City Of Hope in California to evaluate my candidacy for the STAT trial. This isn't a guarantee of acceptance, but simply a meeting to evaluate my condition. If I am accepted I will have to fly back down for rigorous testing to be sure my organs can handle the stress of the transplant.
I am also happy to share that another physician has taken interest in my case! A doctor in Chicago from the ASSIST Trail wants to evaluate me as well. This isn't my first choice due to its lengthy travel, but I do believe the physician is extremely skilled at his craft -- and I would like to see what he has to say about my case. I haven't gotten a date for my consult yet, but I did submit all my new patient paperwork this week. I even received an email from his nurse that stated the physician was "eager" to see me. Hopefully that means soon!
I am extremely grateful to have two physicians interested as I now have the ability to compare experiences and make an informed decision on what I feel will be best for my health. I will keep you all updated as things unfold! Thank you to each and every person who shared, or donated to my You Caring Transplant Fund! You have all made it possible to receive the second consult by funding 600 dollars over my goal! Having two options will insure the best possible outcome for my complicated situation. Thank you from the bottom of my heart!