Sunday, June 27, 2010

The Elephant Returns

A lot has happened since my last blog. I have since become a long-term elephant! I was re-admitted to Hershey Med just six days after discharge the weekend before. I never really felt like much was accomplished during the first nine day stay. I went to my doctor the following Friday and I was really weak, pale, thin, and dry. She admitted me with failure to thrive and instructions to have a GI consult and place another NJ for me to go home with. This time I was even more frustrated because I had to go back knowing that I had pretty much wasted nine days the week before and was feeling no better. But, I was also hopeful that they would be more thorough and that by keeping the NJ in for a while longer would give me a better chance of feeling better in the long run.

I was in the hospital for eight days, many of which were really tough as I felt from almost the beginning that I was in quite a fight to get the doctors to grasp what I am dealing with and all the various complex issues I am aware of that needed to be considered. I was worried that my concerns weren't being addressed. I know that these doctors don't know me because I have dealt with Pittsburgh so much since 2004, and only my doctors pre-transplant know of the unusual complications I develop, but I was flustered that they didn't seem to grasp that I am not the typical patient and my body does not react normally to anything! Finally, I felt like they started to listen once my labs started showing some funky results, pretty much like I told them they would! I only wish I got paid what they do for the work that I do and my out-of-the box thinking on how to treat myself! HA!

After about three days on tube feeds through the NJ I started to show signs of significant "refeeding syndrome." Refeeding syndrome is what happens when the body has been starving for a long time and is starting to receive better nutrition. My phosphorous dropped pretty low. This happens to me almost every time I am restarted on nutritional support of any kind. It's actually a good sign because it means that my body is absorbing the increased nutrition, but it slows the process of feeding down because it can also be dangerous to refeed too quickly. Over the course of the week, my potassium and magnesium fluctuated, something else I told them would happen, even with high rates of IV replenishment! It's part of my conditions that my doctors have been baffled by...they've never seen a patient drop potassium like I do w/o kidney issues, w/o severe diarrhea, and while receiving high loads via IV. It's one reason I am pursuing Andersen's-Tawil syndrome (a Hypokalemic periodic paralysis) as part of a mitochondrial disease...dropping potassium like that is a norm!

My feedings were gradually increased and a GI consult was made. I went home on a tube feeding rate not quite satisfactory, but it was more than I had been getting in nutrition when admitted. I've been working on increasing the rate on my own at home. My success has been limited, but I am up 5 cc's an hour...I think, although occasionally I get sick and have to slow things down. They would like me to get up to a total of 30 cc's/hour, but I'm on 15-20 right now and not sure if I will be able to go much higher. I'm still getting close to 1,000 calories a day over the course of 24 hours because I am on Nutren 2.0 which is thicker than the typical formula used and has 500 calories per 8 oz. It's been a little bit of an adjustment to be back on 24 hour nutrition, but not as much as I thought it would be. I was used to my non-backpack carrying days, but now I pretty much cart it around with me again without even thinking much about it!

I have noticed that it seems to be helping me some. I am still very symptomatic when I eat, but that's not new and certainly tube feedings don't help the underlying problems, just help with one of the complications of having them- it's no worse than it was. It's giving me the nutrition I need without the pressures of having to eat and knowing I will just get sick and lose most of it anyway. So, the pressure is off to try to force foods when I am not feeling well. It's keeping me hydrated, and I do have a bit more energy. Plus, it gives me a chance to have better control over my nausea by giving me a way to get liquid phenergan directly into my small bowel where it is more quickly and easily absorbed. I am pretty anemic right now, almost to transfusion levels, and I DO feel symptomatic from that, but not as much as I could if not for the nutrition I am receiving. I'm having trouble getting up legs get weak, I get short of breath, I have almost fallen a lot because of weakness, getting up in the morning is tough, and I am tired out by 7 PM, but I have more energy for more hours during the day overall. When we get my counts back up, be it through transfusion or other means, I am sure I will feel a whole lot better too!

On Monday, my tube got clogged! I joke about my tube as being my elephant trunk. My mom and I tried for a total of 3 hours to unclog it using all the options we knew of, but were unsuccessful. On Tuesday I had an appointment with internal med, but they said they couldn't do anything so I had to go to the ED. They put me in total isolation because of the immune-suppression and I was placed in a psych room with nothing in it but a fixed bed and mattress! No TV, no electrical outlets for my phone to charge, no people to watch, padded walls, high was REALLY freaky and I was in there for about 7 hours as they used "clog zapper" to try to unclog my nose! I tell people now to never complain of a clogged nose to me because I will not be sympathetic! I'll laugh, and hand them clog zapper and diet soda! The doctor came in before the second attempt and informed me that if they could not get my tube unclogged, I'd have to be admitted for the placement of another one. I wasn't happy about this feeling like it is one of the easier procedures I go through and should definitely be do-able in the ED! Finally, he told me that in a "normal" person he would be more comfortable doing it, but not in someone with my history. When I told him the nurses on the floor placed it, he did more research and decided he would try to put one in himself. After a few more hours and three attempts and x-rays later, finally it was over and they let me go home...but not without a little mishap on the way out! As I was leaving I started to bleed very quickly from one of my IV sites and had to go back to get it stopped. My blood has been extra thin lately, and i think it's because they have been giving me Fragmin shots in the hospital as a pre-caution because of my factor V (blood clotting problem) and the fragmin doesn't seem to leave my system very quickly. So, after an extra half hour detour, I finally headed home about 1 AM.

On Wednesday morning I had an appointment with my gastroenterologist. We discussed the tube feedings and I told them they have been helpful and I haven't been getting as sick. We discussed reasons why this might be the case, and what to do long term. As expected, he has recommended the surgical placement of a j-tube to replace the NJ. I had planned on using the NJ as long as possible before making a decision, but he feels like I should know within a week if it is helping for certain and has recommended that I have one placed much sooner. I'm not quite ready to approach my Pittsburgh doctors with this yet or even make a definitive decision myself on it, but I do feel better and I know that I wouldn't without the help of these feedings. I'd still be sick all the time and struggling to eat. My docs know that if I don't stay on tube feedings that I will just be in the hospital every week at this point and that's not acceptable and definitely not a way to live!

It's tough for me to think about going back to a j-tube, but at the same time if it helps me feel stronger I think I'm prepared for it. I can't imagine going through surgery within the next couple of weeks...while I am prepared for it, I'm not quite ready...if that makes sense?? I don't want any doctor but Costa or Kareem to mess with my organs and I'm not sure how on board they will be for this. Besides that, any surgery is very risky for me and could create complications due to inability to heal, high risk of infection, and bowel obstructions. I think it could take a little while to get things in motion on their, well, I guess I SHOULD get started making plans...

After I went to my GI appointment, they drew 11 tubes of blood. Since those labs showed anemia on Wed, who knows what my counts will be on Monday when rechecked. In spite of that, I surprised myself by having enough spunk to make it to do a couple of hours of volunteer work at LCBC and hold an all volunteer board meeting on Wednesday evening for G-PACT.

The volunteer meeting was productive. We discussed a lot of ideas and it was the first ever all volunteer meeting we have had. It gave people a chance to connect and actually talk to people and discuss ideas over the phone. These meetings always tire me out, but I did well, especially considering the long day and late night I had on Tuesday! I took Thursday and devoted it to catching up on my G-PACT work and on Friday I helped out at LCBC again. Today (well, yesterday...Saturday...) I went to the LCBC kidMin picnic and that was a lot of fun! Today I plan to return to the Wheelhouse for the first time in a while and hopefully make it to Life group in the evening. So, in spite of it all, I'm still living and loving life...elephant nose and all!

My LONG time prayers were answered this week when a professional web designer offered to redesign our website and include an entire social network dedicated to GP and CIP, blogs, mobile content, and so many other very cool interactive features I don't have the experience to work on or the time to want to try to learn! He has GP and has offered his time for free because it will really help us out! Now anyone can add content, not just me, so the website will be a lot more current now and provide so many features which can really take us to the next level. It wasn't a hard decision for me to agree to let him do it! I have been looking for someone of his caliber to work on our site for a really long time! I can't keep up with ours in addition to all the administrative work I have and other life activities!

At the picnic on Saturday, one of my friends and motorhead, Michael Johnson, pulled the top off of Blue lightning for me! Talk about a blast coming home! I have been waiting for MONTHS for this day! I'm so excited! Summer can now begin...a month later than usual for me, but at least it is time for a couple months of fun in the sun! :)

I'm going to close here now, but did want to make one request...if you are on Facebook, please go to the Chase Community Giving Challenges page and "like" it. Then please vote for G-PACT! We are in the running for $20 K and every vote is crucial to make sure we don't lose out on these funds! We can do so much with that money! Please take a few minutes of your time to vote for us! If you are a "friend" of mine, please check out my notes for more info on it!

Off to bed I must will be a long, tiring day today...Sunday if I don't get off few hours ago! Sigh...oh well...I'll make up for it sometime. I can't WAIT to return to the Wheelhouse and hang with my kids! What's funny is I dropped by the WH last week to say hey. The kids were excited, but then one of the twins (who is a mess!!!) stopped and blurted out "what happened to you?" I started to say that I was sick and I had this in my nose to help me eat (I'm going to go with the whole elephant thing with them...) and Melissa jumped in and said "this is what happens when you don't eat your vegetables!" This kid got a look on his face like he didn't know whether to believe her or not...It was really really funny. We were still laughing about it on Monday!

Thursday, June 10, 2010

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 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'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 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.