Polycystic Kidney Disease (PKD) Support Group
Polycystic kidney disease (PKD) is a progressive, genetic disorder of the kidneys. It occurs in humans and other organisms. PKD is characterised by the presence of multiple cysts (polycystic) in both kidneys. The disease can also damage the liver, pancreas and rarely the heart and brain.
We have all missed you so much and ecstatic that you are well enough to come back to the group! Thank you so much for all your advise but there is one thing no one knows about yet and that is, we have joined planet fitness again, so walking is back in my life again! I had to do something I was feeling just awful, UTI's at my age, no appetite! I won't bore you with the rest of it, but we are trying and hard! My appetite is returning and I am positive I am going to make it! I will be going to the kidney doc in April, we shall see what he thinks if this old broad then! Please tell us about yourself, how you have been, etc., you know the drill. Anxiously awaiting to hear from you again. Welcome back Ruth!!! Lots if love, Norma ...
so very pleased to hear from you. I hope you will feel well enough to visit us often; I know how difficult things have been for you. It seems like madness that with all of the problems you have, even though your eGfr is down to 14 you still can't be listed for transplant. Talk about sticking too rigidly to rules........I think of you often, as my liver rules and is a constant reminder of others with enormous livers.
Norma, walking my dogs is what keeps me (almost) sane these days. My youngest little dog is a great encouragement and spurs me on to go out even when I feel horrible. It wears me out but it's worth it. Every day I give myself a pat on the back that I made it; one day at a time, eh? Keep,up the good work and I hope you feel the benefits.
Good to hear from you both,
Love Chewitt
My pain is still well managed with methadone (20 mg three times a day) and I have oxycodone immediate release (aka roxycodone) for breakthrough pain. My stomach no longer expands at all so I'm chronically dehydrated, and probably significantly malnourished as well (the dehydration hiding the fact I'm malnourished as dehydration actually caused serum albumin levels to artificially increase and despite my dreadful diet, my labs seem to indicate that I have great albumin levels for someone in my condition (how I have no idea, since I barely eat anything and am full after a couple of sips of water, or bites of food).
Of course my husband is as wonderful as ever and no, I'm not sharing him with anyone! I simply can't imagine going through this without him and the level of support he provides. I just wish everyone had someone like him in their lives.
Norma,
I'm glad to hear you're walking again. I know how much that helps your back. I've never had a UTI (as an adult), but my one kidney infection was more than enough and I can't imagine what it's like to deal with continuous UTIs over and over again, especially if the doctor keeps giving you the same treatment each and every time. I know my kidney infection was sheer misery that landed me in the hospital for a week (back in 1993). According to the hospital, I gained 15 pounds, but that was all fluid as they discontinued the diuretic I was on at the time. A week later, I not only lost the 15 pounds of water weight, but discovered I had lost 10 lbs while hospitalized (had no appetite and hospital food most assuredly did nothing to stimulate my appetite!! You have so much gumption and get up and go, I KNOW you're going to make it! And remember, you're still a youngster! You're definitely not an "old broad"!
Chewitt,
I noticed your comment a while ago about liver transplants and survival rates. Each transplant center has their own survival rates, and of course you want and need to go to a center that has extensive experience with Polycystic Livers and not all centers do. But when you look at those rates, keep in mind that the average patient who gets a new liver is sick to begin with, often on the verge between life and death. That is NOT the case with us with PKD/PLD. Other than our massive organs, we're actually very healthy and need the new liver due to a combination of quality of life and the fact our polycystic livers are causing damage and compression of all of our other internal organs (especially if there is any evidence of pulmonary hypertension!). So since we start out much healthier, our survival rates are significantly higher in the across the board. Yes, we have our own unique issues, such as the possibility of more bleeding (thanks to the fact we have a huge liver that has to removed and its very "sticky" and very vascular, so it there's a greater chance of more bleeding and it takes much longer to remove than a shriveled up cirrhotic liver). But in the long run, we survive much longer than the average patient, and a liver only transplant usually requires only one immunosuppressant (the liver is amazing and the body accepts it much more readily than any other organ; after a year or so in the body, liver cells from the donor liver can be found throughout the body (I don't remember the name of the process, or much of anything else at this point thanks to both nephron fog and medications), but it contributes to the body's acceptance of the donor liver. So keep those facts in mind when considering a liver transplant.
Lots of gentle hugs and love to you both,
Ruth
I'm glad that you at least have your pain under control and that you have such a supportive husband. I'm keeping my fingers crossed for things to follow the hoped for path quickly and smoothly for you. You've been very strong for such a long time, let's hope it won't be for too much longer.
Lots of love
Chewitt
You obviously are a survivor and your body is compensating to run so regardless of the odds, you will come through the dual transplant once you get it.
I don't come on much but when I do I always enjoy reading your posts and keeping up with you.
Here's some healing thoughts and strength to get you through.
In terms of anemia, I developed anemia caused by the kidney disease when my function was about 50%. My nephrologist (the fabulous, wonderful one) was stumped and ran basically every test known to man. I had a colonoscopy to rule out any type of internal bleeding in that system, a plethora of labs to check iron levels, B-12 (pernicious anemia), B-6 and folate (all of them work in tandem to keep your red blood cells developing and the shortage of one can throw everything out of whack. It's important to note that while your kidneys are the main producer of erythropoietin, your liver also produces some, and our livers are anything but normal when it comes to doing the right thing, despite having normal labs. BTW, does your doctor run a PT/INR on you, checking your coagulation factors?. It's one of the main indicator of a healthy liver. The standard liver panel (ALT, AST, etc.) looks for indications of inflammation in the liver. But PT/INR looks at the actual function of the liver and one of the primary things the liver does is to produce coagulation factors. If it's not producing coagulation factors properly, those numbers will be off, and will also indicate your liver isn't functioning properly. The INR is also part of the MELD score, model for end stage liver disease, along with serum creatinine, and total bilirubin.
I did not get an EGD at that time (have had one since) as my nephrologist had no reason to believe that I had what is called portal hypertension. This occurs when the portal vein, which brings blood to liver from the digestive organs, gets occluded (in our case due to the intense mass of cysts) and instead develops large veins (called varices) that go from the non-occluded portion of the portal vein and reach across the esophagus and stomach to get around the blockage. These varices are fragile and can bleed easily, but if you had them, most likely you would already know. Your voice would most likely have changed a bit, you'd be out of breath (not just fatigued, but literally out of breath) and if a varice bleeds, you would know it as the bleeding usually causes nausea and vomiting, which then aggravates the varices even more. In fact the bleeding can be life threatening due to the amount of blood that flows through these varices from the portal vein. I sincerely hope you do not have any of these problems, but it's worth getting the test done just to be sure.
In my case, the transplant hepatologist simply listened to me talk at our first meeting, did some very gentle probing of my abdomen (no digging at all, and was able with that one encounter to say without reservation that I did not have portal hypertension or any varices whatsoever. This was despite the fact I had and still have problems with catches in my breath that now have become a natural part of my breathing pattern, with some exceptions.
Despite the fact the transplant hepatologist sees multiple liver transplant patients each and every day, I wasn't so convinced, especially as my "transplant buddy", a good friend who had also has PKD/PLD and went through a liver/kidney transplant at Mayo Jacksonville did have portal hypertension. Obviously his case was different and as a radio personality, we were able to hear the catches in his voice while he was on air, and the change in his breathing pattern (I doubt most people noticed, but as friends knowing what he was going through, we did). He's now 5 years post transplant and other than a single mild rejection episode at the 5 month point (when they changed his medication AND changed his labs to every 3 months at the same time, thus not catching the fact the medication change was not a good thing for him fast enough). Less learned, do not make medication changes without doing labs once a month until you're sure the medication changes are effective and have not caused a rejection episode! He's doing terrific; just bummed that they couldn't remove his kidneys (they were too sticky to remove during the transplant surgery and there is no valid reason to remove them now other than vanity) so he never got back that flat Marine abdomen he used to have!
Anyhow, despite the hepatologists insistence that I didn't have portal hypertension or varices, I talked with my nephrologist who was very surprised they didn't do an EGD and to ally both of our concerns, I had an EGD done. It was to be done with sedation, but alas, I no longer respond to the form of sedation they use in the clinic (at least not the kind they use without an anesthesiologist present). So I was wide awake during the entire process. Scary? Yes. But I had a technician decide to hold my hand and talk to me the entire time, reminding me to breath through my nose and telling me what they were doing and looking at (I like to see things on a screen, but that's not an option when they have a tube down you're throat). While it felt like hours, it was only a 3-minute exam, start to finish. I had no varices, no portal hypertension and they were absolutely fascinated by the fact my stomach didn't expand no matter how much air they pumped into it!
You WILL respond to the sedation, so you wont' have any problems. And let your doctor know about your fear and ask if you can have something to take ahead of time so you're less anxious when you get there. Unless the British system is cruel, they're going to give you some form of sedation, so you'll have to have a driver with you anyhow as they won't let you drive home afterwards on your own (in my case for the colonoscopies and EGD I had to have my driver with me when I signed in; if he wasn't there, they wouldn't do the procedure).
The fear of the test is far worse than the test itself. The same holds true for a colonoscopy. In the case of the latter, it's the prep that's the most miserable part. I had one done last week and I assure you, the prep was dreadful and tasted horrid; I only finished about 60% of it, but I had also been on a low-residue diet for 5 days prior and barely eat anything anyhow, so there wasn't that much to clear out. I had one done 11 years ago with the standard sedation and woke up with a couple of minutes of getting the sedation (and that was when I was sedative nave), so I just watched the test on the TV screen. This time they gave me heavy duty sedation with propofol (the closest thing to general anesthesia without requiring intubation) and all I remember about the test is telling the anesthesiologist I had a metallic taste in my mouth and my lips felt a bit numb, then waking up in the GI recovery room, asking repeatedly if they had done the test! Even that evening I still wasn't quite sure about the test, despite having the results, complete with pictures in my hand. I'm happy to report that despite the expectations of a contorted twisted colon and the possibility of not being able to reach the terminal end where the colon meets the small intestines, none of this was true. While my liver has done a number on everything else in my abdomen and pelvis, all it's done to my colon is push it out to the sides and up to the top, compressing it in a few places, but there were no tortuous twists or turns and I was in and out of the colonoscopy suite in a bit over 20 minutes (with the average time with being 10 minutes). So it ended up being far easier than expected, thank goodness, but I still want to sleep through the next one too!
You'll do just fine during your tests. The thought of them is far worse than the reality. Just be sure your doctor does all the labs first and prescribes some good anxiolytics for you to take before your tests, so you are less anxious to begin with and the sedatives work better during the test. Alas, these are tests that you need to get done, they make perfect sense from an educated patient perspective (mine) and as long as the EGD is negative, you never have to get another one done. And the colonoscopy is a once every 10 year test (even for transplant patients). Get them done and you'll be good to go for at least 10 years!
Lots of love and very gentle hugs,
Ruth
I want to ask you all, can't the anemia be from kidney failure which is treated by Epogen shots? As my function was decreasing, and during dialysis, I was taught how to give myself the Epo shots, and it did bring up the blood count. At this point, I don't know how much my liver is failing, will check next visit, but I know between the kidneys and the liver, I have one full abdomen.
As well Chewitt, I want to encourage you as Ruth did about the fear. It is NORMAL to have the fear, but we do need to decide ahead of time how much free range we allow it to take. I do need to have another colonoscopy, as it has probably been a good 5 years now, but when I had it before my workup for a transplant, I read the Dave Barry Journal below, and it is so hilarious I have to share it. But I agree with Ruth, the prep for it is the worse, but the actual colonoscopy is a piece of cake, really.
Another real fear I had was after my kidney transplant, I was terrified of the stint removal, where the doctor would have to go up through the bladder and take the stint out between the bladder and the kidney. I decided that every time I started fearing that, I was going to put it off, and not let it take hold. As well, I had to get it out of there in Seattle so that I could come home to Alaska. Well, it was so fast, and the doctor was so good about it, quick as a wink it was over with. I think he had only enough time to say what a great job my surgeon did on putting it in. "That Dr. Precht does such good work" and then, it was done. I told myself after that, "remember, worry and fear don't do you much good at all".
Colonoscopy JournaL by Dave Barry
ABOUT THE WRITER
Dave Barry is a Pulitzer Prize-winning humor columnist for the Miami Herald.
Colonoscopy Journal:
I called my friend Andy Sable, a gastroenterologist, to make an appointment for a colonoscopy.
A few days later, in his office, Andy showed me a color diagram of the colon, a lengthy organ that appears to go all over the place, at one point passing briefly through Minneapolis.
Then Andy explained the colonoscopy procedure to me in a thorough, reassuring and patient manner.
I nodded thoughtfully, but I didn't really hear anything he said, because my brain was shrieking, 'HE'S GOING TO STICK A TUBE 17,000 FEET UP YOUR BEHIND!'
I left Andy's office with some written instructions, and a prescription for a product called 'MoviPrep,' which comes in a box large enough to hold a microwave oven. I will discuss MoviPrep in detail later; for now suffice it to say that we must never allow it to fall into the hands of America 's enemies.
I spent the next several days productively sitting around being nervous.
Then, on the day before my colonoscopy, I began my preparation. In accordance with my instructions, I didn't eat any solid food that day; all I had was chicken broth, which is basically water, only with less flavor.
Then, in the evening, I took the MoviPrep. You mix two packets of powder together in a one-liter plastic jug, then you fill it with lukewarm water. (For those unfamiliar with the metric system, a liter is about 32 gallons). Then you have to drink the whole jug. This takes about an hour, because MoviPrep tastes - and here I am being kind - like a mixture of goat spit and urinal cleanser, with just a hint of lemon.
The instructions for MoviPrep, clearly written by somebody with a great sense of humor, state that after you drink it, 'a loose, watery bowel movement may result.'
This is kind of like saying that after you jump off your roof, you may experience contact with the ground.
MoviPrep is a nuclear laxative. I don't want to be too graphic, here, but, have you ever seen a space-shuttle launch? This is pretty much the MoviPrep experience, with you as the shuttle. There are times when you wish the commode had a seat belt. You spend several hours pretty much confined to the bathroom, spurting violently. You eliminate everything. And then, when you figure you must be totally empty, you have to drink another liter of MoviPrep, at which point, as far as I can tell, your bowels travel into the future and start eliminating food that you have not even eaten yet.
After an action-packed evening, I finally got to sleep.
The next morning my wife drove me to the clinic I was very nervous. Not only was I worried about the procedure, but I had been experiencing occasional return bouts of MoviPrep spurtage. I was thinking, 'What if I spurt on Andy?' How do you apologize to a friend for something like that? Flowers would not be enough.
At the clinic I had to sign many forms acknowledging that I understood and totally agreed with whatever the heck the forms said. Then they led me to a room full of other colonoscopy people, where I went inside a little curtained space and took off my clothes and put on one of those hospital garments designed by sadist perverts, the kind that, when you put it on, makes you feel even more naked than when you are actually naked..
Then a nurse named Eddie put a little needle in a vein in my left hand. Ordinarily I would have fainted, but Eddie was very good, and I was already lying down. Eddie also told me that some people put vodka in their MoviPrep..
At first I was ticked off that I hadn't thought of this, but then I pondered what would happen if you got yourself too tipsy to make it to the bathroom, so you were staggering around in full Fire Hose Mode. You would have no choice but to burn your house.
When everything was ready, Eddie wheeled me into the procedure room, where Andy was waiting with a nurse and an anesthesiologist. I did not see the 17,000-foot tube, but I knew Andy had it hidden around there somewhere. I was seriously nervous at this point.
Andy had me roll over on my left side, and the anesthesiologist began hooking something up to the needle in my hand.
There was music playing in the room, and I realized that the song was 'Dancing Queen' by ABBA. I remarked to Andy that, of all the songs that could be playing during this particular procedure, 'Dancing Queen' had to be the least appropriate.
'You want me to turn it up?' said Andy, from somewhere behind me..
'Ha ha,' I said. And then it was time, the moment I had been dreading for more than a decade. If you are squeamish, prepare yourself, because I am going to tell you, in explicit detail, exactly what it was like.
I have no idea. Really. I slept through it. One moment, ABBA was yelling 'Dancing Queen, feel the beat of the tambourine,' and the next moment, I was back in the other room, waking up in a very mellow mood.
Andy was looking down at me and asking me how I felt. I felt excellent. I felt even more excellent when Andy told me that It was all over, and that my colon had passed with flying colors. I have never been prouder of an internal organ.
On the subject of Colonoscopies....
Colonoscopies are no joke, but these comments during the exam were quite humorous..... A physician claimed that the following are actual comments made by his patients (predominately male) while he was performing their colonoscopies:
1. 'Take it easy, Doc. You're boldly going where no man has gone before!'
2. 'Find Amelia Earhart yet?'
3. 'Can you hear me NOW?'
4. 'Are we there yet? Are we there yet? Are we there yet?'
5. 'You know, in Arkansas , we're now legally married.'
6. 'Any sign of the trapped miners, Chief?'
7. 'You put your left hand in, you take your left hand out...'
8. 'Hey! Now I know how a Muppet feels!'
9. 'If your hand doesn't fit, you must quit!'
10. 'Hey Doc, let me know if you find my dignity.'
11. 'You used to be an executive at Enron, didn't you?'
12. 'God, now I know why I am not gay.'
And the best one of all:
13. 'Could you write a note for my wife saying that my head is not up there?'
Thank you both for your invaluable input. It's always so good to hear things from the horse's mouth, as it were.
With regards to the anaemia, my Nephrologist is adamant that PKD does not cause anaemia in the same way that CKD generally does, and so he believes that there is another cause. On reflection, I do not think that a bleed in the digestive tract will be the problem. I think it far more likely that the cause is my menstrual cycle, as at 48, my hormones are being quite mean to me at the moment and have been for some time. I have said this to the consultant but he ignored me. I'm really not sure that there is any point having the endoscopy. I have no symptoms of varices or portal hypertension that you describe Ruth. If there turns out to be inflammation as a result of acid reflux, there's not much I can do about it whilst these massive organs remain in my body, squeezing my stomach up and its contents out.
The colonoscopy holds less fear for me, though I know it's horrible. As I say, though, I don't think it's necessary. I also know several people who have had these procedures without sedation. My father, for instance, drove home afterwards. It doesn't seem to be routine in the UK to sedate.
I will in any case meet with the GI consultant (if I ever receive an appointment) and discuss the whys and wherefores before making a decision.
I have had several other blood tests for folates, B12 etc and all is normal except for Hb being low. I haven't, however, had all of the liver tests that you mention - just the standard ALT etc., so I might mention this to the GI consultant, too.
Heather, thank you for sharing the account of the colonoscopy. It really made me laugh, which is good. I try to bear in mind your wise words about fear but I think I'm a bit of an ostrich. More and more I don't want to have traumatic or invasive tests to find out that I have something else that can't be fixed. I'm feeling a bit fatalistic about it all at times.
Thank you both again for your support and reassurance. I really do appreciate it.
Love Chewitt