"Blue Lightning Jeep" and me on a summer day in 2010 when I had an NJ tube
"Gastro what? Oh, I have that. Everybody does. That's just reflux. Just take some Tums. Pseudo- HUH? Oh. Just avoid salad and you'll be fine. Salads makes me sick, too. I have IBS. Five organ transplants? Why did you need five organ transplants? Kidney? And what else?"
"No, no, no...!!! It's not that simple!"
Having one rare illness is frustrating enough, but when you have a number to deal with, it only adds to the amount of hair you pull out in a day...if you have any hair to begin with after meds, malnutrition, and stress. Trying to explain it all to medical personnel, even in some of the best hospitals is challenging enough, not to mention trying to explain it to your typical layperson.
I have suffered from a number of conditions for 18 years that have left me unable to eat, work, or function in any normal capacity. My life has pretty much been swallowed up by long medical terminology and complicated scenarios. As if figuring out how to treat one rare condition isn't difficult enough, getting doctors together on the same page for a number of conditions is like getting a Steelers fan to convert to a Ravens fan- it just doesn't happen!
In 1994 I was diagnosed with a condition known as gastroparesis, or GP. It literally means "paralyzed stomach." Gastro- Stomach. Paresis- Paralysis. I also suffer from Chronic Intestinal Pseudo-Obstruction, or CIP/CIPO. CIP literally means "false intestinal obstruction." Pseudo- false. Obstruction, well, doesn't that speak for itself? CIP is a condition in which the small bowel forms blockages/obstructions in the absence of a mechanical obstruction. Basically, when I eat, food gets stuck in my small bowel and while it doesn't actually obstruct the bowel, it blocks it off completely so that nothing can go through. Thus the term "pseudo-obstruction" means "false" obstruction because it acts like an actual bowel obstruction. In spite of how easy the terminology seems to me to understand, it's not to most people who are not surrounded by medical terminology and even medical personnel. It's even more complicated to treat!
These two conditions led to my need for a five organ transplant. No. Not FIVE organ transplants. ONE transplant of five different organs. And no, a kidney was not one of them...they do transplant other organs besides kidneys...
Picture of me which appeared in the Pittsburgh Post-Gazette in Dec 2005, three months before my transplant. Taken at Montefiore Hospital.
On March 13, 2006 I was given the gift of life at the University of Pittsburgh Medical Center when I received a small bowel, stomach, pancreas, liver, and duodenum due to total gut failure. My entire GI tract had quit working, I was 62 pounds, and starving to death. I had been on TPN, a form of IV nutrition, for years and lost all my veins for central lines due to frequent sepsis. The TPN caused liver damage resulting in my need for a new liver.
After the transplant, I also developed a condition known as dumping syndrome. The pyloric sphincter was damaged during the transplant and that means that sometimes my food empties TOO fast from my stomach- quite the opposite of gastroparesis. So, not only am I juggling a number of rare conditions, I am dealing with conditions that are opposite of each other! The diets are complete opposites and I never know which one is going to act up that day, or even from one hour to the next! But it doesn't end there!
On top of the GI issues, I also have a rare heart condition known as long QT syndrome. Long QT is an electrical abnormality of the heart. Although genetic, this condition is triggered by low potassium and certain medications. Which medications? Any medication used to stimulate the GI tract to digest (motility medications) and most anti-nausea medications. The medication used to treat my dumping syndrome also has a warning that it can cause sudden cardiac death in people who have a long QT interval. Been there, done that on the motility medications. I don't recommend it. Aye, aye, aye! Plus, that medication also CAUSES gastroparesis. So, if I try to treat the dumping syndrome, I only make the gastroparesis worse AND I put myself at risk for heart complications.
As if that weren't enough, I also lose potassium at an alarming rate, even IV potassium. There is a possibility I have another rare genetic condition, a hypokalemic periodic paralysis, which causes my body to shift potassium from my bloodstream and into my muscles and causes drastic and sudden drops of potassium, even in IV form and when not exhibiting any loss through my GI tract or kidneys. Again, low potassium can trigger my long QT and send me into potentially fatal heart arrhythmia's. I am rare. I am very rare. Not only is this combination of problems rare, perhaps even non-existent in anyone but me, but not too many people have survived six episodes of cardiac arrest!
In addition to all of those, I juggle a number of other conditions which are the result of years of malnutrition, treatments, or caused by some rare, underlying condition yet to be discovered. Basically, I am a mystery. A conundrum. Unique. Complicated. Special. Oh, the number of terms that have been used to describe me over the years are are numerous as the pages in my medical records!
Just imagine trying to explain one of these to a physician on a trip to the ER, much less getting into all of them and how they interact and work against each other. Then imagine being half asleep and sick, tired, and trying to explain it all! One doctor thinks his or her treatment option is a must, while the other claims that it will aggravate something else and isn't an option. Then a third chimes in that neither will work well, and this other treatment is the only way to go. Finally, a fourth comes to the conclusion that nothing should be done. Just live with it. It has gotten to the point that my doctors ask me what to do now. I don't know. Just let me get some sleep. I'm tired of all these questions. It's just....complicated!
I am the founder and president of the Gastroparesis Patient Association for Cures and Treatments, Inc. G-PACT is a 501(c)(3) non-profit organization founded on August 23, 2001 to help fight gastroparesis and chronic intestinal pseudo-obstruction, collectively known as digestive tract paralysis or DTP. Check out our website at www.g-pact.org, like us on FB at www.facebook.com/GPACT, and follow us on Twitter at 4GPACT.
