The road of the elephant...and where it goes next

Ok, so I'm FINALLY getting the world caught up on my health status. I apologize for seeming to be "mysterious," but I'm trying to come to terms with things myself and I just haven't felt well enough to focus on keeping everyone in the loop. But I do appreciate your prayers and patience as I work through a lot. I feel kind of lost in this medical jungle, once again. I've had many times in 16 years where a big medical decision was placed in my lap, obviously. I mean, you don't choose to go through a 5 organ transplant lightly. But in that case I had no choice, as I saw it, because it wasn't going to make things worse...and usually my doctors had an opinion on it or would provide me with some kind of guidance...

I'm not in the situation at this point where I will make things permanently worse if I go with the route I have been considering, but it's just that I've been here before and it's difficult for me to think that four years after a life saving transplant, I am in a situation where it has become necessary again and makes me wonder about the future over the next year or two...

As you know, I was in the hospital nine days last week. I went in dehydrated and malnourished because for almost a year, gradually worsening over time, I have been unable to eat very well due to my combo of problems of dumping syndrome and gastroparesis. Treating one worsens the other and the diets are opposite of each other too!

Last year I spent three months on TPN (IV nutrition) and developed sepsis twice in that amount of time, once requiring a lifeline to Pittsburgh. I have a history of this which is how I lost all my veins and ended up needing the transplant in the first place. Since then I have learned more about conditions I may very well have which make me so susceptible to infection, but being on immuno-suppressants because of my transplant puts me at even higher risk for developing sepsis and lowers my odds of being able to fight it off as well.

Since stopping the TPN, I have dropped in weight down to 85 pounds. I am weak and have fallen a lot. Fortunately, I have not broken any bones considering I have osteoporosis! I have been losing my hair and other parts of my body have started to show signs of poor nutrition. In addition, some of my other symptoms pre-transplant have returned to some of my other organs. I have chronic body pain, nausea, and abdominal pain which keep me awake at night uncomfortable. I lose everything I eat whether as a result of dumping syndrome or gastroparesis. I am following the diet(s) the best I can, eating small portions, but even a few crackers will kick off an attack so bad I am miserable for hours. I am not getting down 500 calories a day, if even close to that. Part of my problem is I can't have sugar, so even my fluids don't provide me with any calories!

For weeks, perhaps even a few months, I have had a thought on my mind which I brought up with my mom a couple of times, but really shoved behind me because I just felt like I wasn't ready yet and that surely there was a way around this. I'd go to Pittsburgh and think about bringing it up, but then I'd change my mind feeling that perhaps with the little tweaking, or different perspectives from other doctors, that we'd hit something and I'd be able to turn this around on my own. But that hasn't happened and instead I've just continued to have more problems and get even weaker.

During my admission last week, obviously the question of long-term nutrition came up. I have, literally, been blacklisted by the Hershey Med Interventional Radiology Department for all central lines. Basically, they don't even want to look at me because they sent me back to my room in 2004 saying I had nothing for them to access anymore. So, TPN is out in the eyes of Hershey because they don't know where to put a line, and for the time being, Pittsburgh as well because I am too high of an infection risk. I don't even want to consider that option anyway...

While talking to the doctors last week, the suggestion was made to put down an NJ tube for feedings for a few days just to see how I tolerated those and to see if I could get in some nutrition that way. It was then that I mentioned I had actually been considering talking to my doctors about placing a j-tube again for nutrition, having no idea if this would be a way to receive nutrition and cut back on symptoms by not having to eat, but I was concerned about having surgery and causing damage to my new organs for something I wasn't even sure would work. A j-tube is a feeding tube which is surgically implanted directly into the small bowel, bypassing the stomach. I have been unsure if this would work since I know I have some small bowel problems, but dumping syndrome and GP are primarily in the stomach, even though DS causes more small bowel, pancreatic, and cardiac type symptoms. However, I had never considered giving an NJ tube a test drive. An NJ tube is a feeding tube that goes through the nose and bypasses the stomach and feeds directly into the small bowel. It can be placed without requiring surgery. A j-tube surgery would create more adhesions and lead to a higher risk of bowel obstructions. The problem with an NJ tube is that it has to be replaced frequently, is not ideal long-term, and is very visible! Vanity aside at this stage in my life (yeah, an IV line in the neck isn't exactly attractive either!), it's an uncomfortable thing to have a tube in your nose all the time, although not something I haven't done before.

After clearing the option with my transplant doctors, I let Hershey move ahead with the NJ tube placement (thus the elephant comments and pics began!) :). My thinking is that if we bypass the pyloric sphincter, my DS will not be aggravated and my nutrition will stay in. DS is the result of damage that occurred to my pyloric sphincter during the transplant. The pyloric sphincter is the valve at the bottom of the stomach that regulates the rate at which food empties from the stomach into the small bowel. I think if we bypass the pyloric sphincter and infuse nutrition directly into the small bowel, I won't be nearly as symptomatic and perhaps absorb some of the calories that I receive this way. Unfortunately, I didn't get the opportunity to give it a true test in the hospital. Being there over a weekend, especially a holiday weekend, always slows things down! Plus, it took them a while to get the tube in the right place so I lost a couple days that way. Once in place, tube feedings had to be started very slowly because my gut is not used to handling much and was being introduced to new things. In two days, I was backed up and not handling the feedings well so I was switched to another formula. Sometimes this makes a difference. But, they were quickly stopped again because I was not able to have anything in my gut for a while in order to go through testing and biopsies. A couple days later I was discharged and the tube was pulled out. However, on the small amount of tube feedings I was able to tolerate, I did feel some better and was less symptomatic. I don't know if this was the result of the IV meds I was on, the fact that the tube was actually working, that I was eating less because of the tube feeds taking away my appetite, or what. But, I want to try again...I'm at a point of desperation. I just don't feel well at all. I'm tired. I ache all over. I'm weak and short of breath. The scariest thing is that my potassium has been running low.

For those of you who know my history, I have a long QT interval (electrical abnormality in my heart) and went into cardiac arrest 6x in the years before my transplant as a result of either meds or low, even low-normal potassium. I fear I am entering that phase again of having to constantly focus on my potassium and worry about what every heart palpitation I experience may mean. Heart palpitations come with dumping syndrome, and I don't want to live with that constant fear when I am dumping, while I know it is important to have a healthy concern. I may start going back to my cardiologist again given the current situation or at least have routine EKG's to ensure that I am not returning to an unsafe situation with my heart. Either way, my body is feeling and showing signs of poor nutrition more and more every day and I am struggling to get by and frustrated by the fact that my activities are starting to become more limited. I am still doing as much as I can, but clearly my concentration is worsening, I am not out of bed for very long periods of time, I have concerns about driving many days, and going up the stairs requires a lot of effort again. I've had to pull my HP parking permit back out, something I only used on rare occasions until recently. All of this has been tough for me. While I have been through it before, half of my life has been filled with medical drama, and I have been very fortunate in terms of my body not REJECTING my organs, this is what I see as a major setback and almost a return to my status before going through the transplant. Sure, complications are always expected. When you have a transplant you go in knowing that you are trading one set of problems for another. I just had hoped I wouldn't be trading the set of problems for the exact, or similar, set of problems again...certainly not anything that would stop my ability to eat! I love to eat!

Having said that, the NJ tube is in no way curative or a way for me to eat better. The main goal at this point is to help me get back on track nutritionally and to feel physically stronger and more functional. It won't have an impact on my symptoms if I do eat. The only thing it may do is make my gut stronger and improve my nutrition so that perhaps it will eventually be able to function better simply by being more nourished. That was also the goal of the TPN. The other thing is, my transplant doctor isn't convinced this is going to work. In fact, he thinks it may make the DS worse. I don't think it will because if we pass the pyloric sphincter, we are passing the problem that causes the dumping, but we are in new territory here with all of this and he just doesn't know. He has said it is something I can try, he won't be upset or think poorly of me for doing it, he just won't do it himself because he doesn't have confidence in it. But, he has said my doctors in Hershey can do it if we would like to try it.

So, I am scheduled for an appointment with my PCP this Friday at 3 PM. I had wanted to get in with her on Thursday because I am feeling really lousy, but she's not in on Thursdays and I chose to wait a day and see her rather than see the PA on Thursday. Technically, I should go in under GI, but I know I won't get an appointment with my GI doctor fast enough and I'm already in as bad of shape now as I was on admission two weeks ago. I'll present this option to my PCP and we'll go from there on Friday, but I think the plan is pretty much set as I see it. The good thing with the NJ tube is we can test it for a while and see if it works and all it can do is just not work or make me feel sicker...it won't do any permanent damage to my organs. If it does end up working and we see that it will be a long term need, I will then talk to Dr. Kareem about the possibility of the surgical placement of a more permanent j-tube (I am not comfortable with anyone but Kareem or Costa operating on my beautiful organs!) and also the possibility of having a g-tube placed again so that at least I could enjoy eating and get rid of the contents without vomiting or experiencing the miserable dumping syndrome symptoms if I do get sick.

I didn't think there could be worse GI conditions than GP and CIP. I still don't think there are, except when it comes to dumping syndrome too. They are all horrible...and a combo of any is a nightmare. I experience all three at various times, or all three at the same time too. I know it is odd, but that's the way it is. Dumping syndrome alone is totally disabling. Even before I started to develop signs of GP and CIP again, DS had me flat on my back many many many days. Now I don't know what is going to do what. At least with just one I knew how to eat to manage things more easily, but it got to a point where even just the dumping became unmanageable. Now with all three interacting, it complicates things more...and by treating one, we make the others worse. My hope is that, even if not ideal, the CIP and other potential small bowel issues do not prevent me from being able to feed through the NJ, and if necessary, J-tube as I try to find a way to improve and stabilize my health and not fall into a severe level of poor nutrition or greater weight loss (I was 62 at time of transplant). My osteoporosis has worsened in the past year already. My body doesn't even have what it did pre-transplant to bounce back from as much. I'm not in as bad of shape as I was pre-transplant by any means, although I do have days I feel as bad as that, but the trauma to my body over the years has just added up and I don't feel like I have as much to fight back with for any major complications with treatment options or anything caused by malnutrition or just the overall problems related to the native condition which started this all in the first place (still unknown, pursuing Mitochondrial Disease which could include Andersen's-Tawil Syndrome, POTS, and a few other things fitting the puzzle pieces together).

It's late so I'm starting to ramble and get off track. Basically, that's where it stands...I'm going to try the NJ tube on a longer term basis and see if I can get a reasonable amount of tube feeding into my system over 24 hours/day with fewer symptoms. If so, it's worth keeping an NJ tube in at home for a while too. If effective, I may seek the placement of a permanent J-tube, possibly with g-tube so I can at least enjoy eating some. I have problems with NJ tubes sometimes not holding in place and creeping back up into my stomach, which clearly creates problems, but it is a fixable problem too. The NJ is only temporary and will only last a few weeks or months...but perhaps that is long enough to get me nutritionally stable and eating better. Also, I don't know that it's even going to work. This is going to be a period of trial and error as it will take a while to get feedings up to a decent level while my gut adjusts, and I don't know if symptoms will improve or not.

I also don't know the status of my small bowel and whether my worst symptoms really do originate mostly in the stomach, or how many originate in the small bowel too. My prayer is that my stomach is the biggest problem and I don't have to deal with the additional problem of major small bowel issues too so that I can handle the tube feeds and not have to worry about TPN, potassium and cardiac issues as much, and will have this option for nutrition until my stomach decides to cooperate! It's complicated...it's very complicated. And it's frustrating too. But it is what it is and it's just one more thing I will deal with and get through. I had just hoped that I would never reach this point in my life again, or at least not this quickly, but I'm tough and I'll deal. It won't stop me. I'll keep plugging away with all my activities, living life as normally as I possibly can in spite of it all.

I will always keep fighting for a cure for these problems, even if I will not personally benefit. I'll just keep being me...and take what comes as it comes, one day at a time, even an hour and a minute at a time. I'll enjoy the good days as always, and on the bad days, look forward to the good days. Nothing has changed in that way...my life isn't over, I don't regret going through the transplant at all, and I'm still optimistic and love life! This is just a bump in the road, or as my friend Nancy likes to say "Transplant patients don't have bumps, they have potholes!" Very true! But there's a way to look at potholes too. Even though potholes can be big enough to throw your sysyem out of whack, they can be fixed and filled too...not always perfectly or smoothly, or through the easiest means, but there are alternatives and options and the road can still continue and be traveled safely.

Thank you for your love, support, and interest as I pursue this next step on Friday. I respect my transplant physician and he is my hero. But, I also feel like it is time to branch out and step into this. Perhaps I will learn something that he can use...if it works for me, it may work for others. If it doesn't, he's right and I'll know that. But I have to try...I can't think that it might be an option to help me feel better nutritionally and not try it. Even if he won't be involved himself, I am grateful he has given me his support to try it if my doctors in Hershey are willing to work with me on it.