Newly diagnosed Stage 4 liver disease

Just wanted to say hello, I have been reading all of your posts and the wonderful information from Hector especially.  You really seem to know the ins and outs of Cirrhosis.  I was diagnosed in January 2012 with SLE Lupus and it was blamed for my low blood platelets.  After a regimen of Prednisone and Imuran my platelets did not recover and I was still  having fatigue and bruising. An ultrasound revealed an enlarged spleen and liver and my RA still kept treating me for just the Lupus,which I am ANA positive and have the Lupus criteria.  Needless to say I changed RA's in August and she jumped on the suspect of the liver being the major culprit for the platelets.  After a CT scan, liver biopsy and endoscopy I find myself faced with liver disease, medium varices in the distal third of the esophagus as well as portal hypertension and gastritis along with an ulcer. There were no bleeders thank God.  I have been put on the beta blocker Corgard 20mg and Protonix 40mg for the first line of defense. There were no ascites, thank God again.  I have been put on a low sodium diet which I anticipated from reading up on my disease stage.
The question is still Why do I have this liver disease?  Complications of the Lupus being undiagnosed and years of elevated liver profiles with no cause.  I had a liver biopsy in 2005 which said I had mild steatosis that was not expected to progress.  But it did, anyway, I am reeling with emotion as I am only 53....which 3 small grandchildren that I would like to see grow up.
My son wants to be a liver donor and the closest facility to my location is Toulane in New Orleans, LA.  But I ready that New York Presbyterian does laparoscopy transplants on some patients and this sounds really interesting.  Not sure if they are doing adult to adult yet but I am going to check it out.
My MELD is 11 right now and my CTP score is 6 so I am hanging in there and want to keep hanging in there.  My GI said the largest problem is the Lupus factor and the unknown.  All hepatitis including auto immune is negative.  Everyone doctor shakes their head and says the reasons are unknown and my case is complicated at best.  My GI is doing all the preliminary tests so he can send them with me to a hepatologist.  
Sorry so lengthy but I am trying to soak in and learn everything I can about this nasty diagnosis.
I have cried for the last 3 weeks so I am just now trying to start fighting back.  Thanks for listening all!

Teri

[HectorSF]

Teri,

I can not comment on anything but liver disease as I have no experience or knowledge of the other conditions that you speak of.

Hector, you asked me about a few things......

1.  No ascites
2.  No internal bleeding
3.  No vomiting
4.  No swelling except in joints and from the Prednisone
5.  No memories issues out of the ordinary, I have always made lists
6.  Medium Varices
7.  Portal Hypertension
8.  Jaundiced while on Imuran
9.  Mildly enlarged spleen

These items are all consistent with compensated cirrhosis.

When a patient has cirrhosis AND portal hypertension it is quite common for them to have a low platelet counts. (My platelet counts have never been above 70,000 since 2007). The platelets are sequester in the enlarged spleen.

"People with cirrhosis end up having a problem with portal hypertension and an enlarged spleen, and a reduced platelet count in the bloodstream. As time goes by, the liver may try to repair itself by growing new cells. If there is a lot of scar tissue already present--- the new cells grow between scar tissue (and result in abnormal nodules). (The nodules and scar tissue can further interfere with blood flow through the liver). So over time people with advanced cirrhosis can end up having a problem with more and more abnormal nodules and scar tissue forming...which interferes even more with blood flow through the liver.....which makes the spleen continue to enlarge....and the platelet count continue to drop."

"The obstructed flow of blood through the portal vein (portal hypertension) causes the spleen to enlarge by causing an increase in pressure inside the vessels of the spleen. The spleen stores red and white blood cells and platelets (fragment of cells that are used to clot blood). An enlarged spleen traps platelets lowering levels in the blood which can lead to the inability of blood to clot. This is called Thrombocytopenia and is common in people with cirrhosis. "

"The Natural History of Cirrhosis
http://www.clinicaloptions.com/Hepatitis/Annual%20Updates/2008%20Annual%20Update/Modules/Garcia-Tsao/Pages/Page%201.aspx

There are 2 main stages in the natural history of cirrhosis: compensated and decompensated cirrhosis, defined by the absence or presence of ascites, variceal hemorrhage, encephalopathy and/or jaundice. Compensated and decompensated cirrhosis are 2 separate entities with distinct clinical presentations, prognostic indicators, outcomes, and mortality.
Cirrhosis represents the end stage of any chronic liver disease and can remain compensated for many years before the development of a decompensating event. Decompensated cirrhosis is marked by the development of any or all of the following complications: variceal hemorrhage, coagulopathy, hepatic encephalopathy, ascites, spontaneous bacterial peritonitis, hepatorenal syndrome, hyponatremia, hypoalbuminemia, and/or hepatic encephalopathy. Because of its poor prognosis, the only effective therapy for decompensated cirrhosis is liver transplantation."

"Anyone, hear this, I read from another web site that you can lose up to 75 percent of your liver to cirrhosis and it still can rejuvenate.  What I do not understand is that the ultrasound tech told me that blood is still getting to my liver so there has to be some part that is salvageable, I just want to know what part."

All organ in the body have more capacity than they need to function properly. The liver can lose a large percentage of its functioning liver cells and still manage all of its functions. 80-90% liver must stop functioning for hepatic failure. This is why just having scarring doesn't cause the liver to fail. When you have compensated cirrhosis as you do. Even though many of the functioning liver cells (hepatocytes) no longer function due to them being scarred you have no complications of your cirrhosis. When the functioning be impaired we call that decompensated cirrhosis. This is went the liver can no longer carry out all of its functions. This is when the complications of cirrhosis manifest themselves. Ascites, bleeding varices, hepatic encephalopathy etc. etc.

"What I do not understand is that the ultrasound tech told me that blood is still getting to my liver so there has to be some part that is salvageable, I just want to know what part."

Blood is always getting to the liver if not your liver would fail and you would die. Like when the blood supply is cut off to the brain. The brain tissue dies along with the patient. As the liver gets more scarred with time less and less blood flows through the liver. It then starts flowing through collateral veins. The is what varices are.

The bottom line is you are not receiving the best healthcare that is available for your liver disease. Cirrhosis is a very complicated disease and can potentially be fatal if not managed properly. The best thing you can do for yourself as all cirrhotic patients should do is to get a hepatologist at a transplant center to manage their liver disease. The fact is a gastroenterologist is not qualified to manage a patient with cirrhosis, period. A good gastroenterologist would tell you as much.
I would go to The Tulane Abdominal Transplant Clinic in New Orleans, LA and get evaluated to find out if you are ill enough to be listed. You may not be that ill but at least you will know your current condition and they will follow you over time until you are ill enough to be listed. It could be a relief t know you don't need a transplant any time soon even if you in time have a living donor transplant.

"A transplant evaluation is the process by which you prepare for transplant. It includes a history and physical exam, psychosocial assessment, nutritional consultation, and financial evaluation. The first appointment will take approximately 1-2 hours. This is just the first step in the evaluation for many patients, as you will need additional tests and consultations with other physicians. Your Transplant Coordinator will be able to tell you what additional tests you may need before you leave after this first appointment, but sometimes we may not be able to tell you until after the whole team reviews your case. The formal liver transplant evaluation usually takes 2-3 days and is done on an outpatient basis unless an emergency situation exists.
Your Transplant Coordinator will call you after all your results have been received and have been reviewed by our team at our Candidate Selection Meeting. This is a meeting of the whole team to review your case and to make a decision about transplant for you. The liver transplant selection committee meets weekly. Within a week or two after we have received all the results of your tests, you can expect to hear from us. We will first get clearance from your insurance carrier before we place you on the waiting list. Once we do, you will get a letter in the mail telling you this. We will also send a letter to your referring physician and/or primary care physician. If you do not receive a letter from us, you should call us."

Your gastroenterologist or primary care physician can make the transplant referral, but anyone, including you, can call to make the transplant appointment.
When you get a hepatologist you will know the status of all of your health conditions and will have someone who can answer all of your questions over the years that you will probably have cirrhosis.
You have already had one doctor who may have damaged your liver more by using medicine that is toxic to the liver. When you have a hepatologist ALL changes in medicine, diet, etc. that can impact your liver must be approved by them first and they will work with your other doctors. To continue to rely on unqualified doctors is a risk no person with cirrhosis should ever do if they want to maintain their health for as long as possible.

Cheers!
Hector

[Dee1956]

Hello, welcome to the forum.  I am so sorry for your pain. It took me quite a while to wrap my brain around the cirrhosis
There are knowledgeable people on the forum who will be here to help as soon as they are awake.:)
I just wanted to say hello, hang in there
Dee

[HectorSF]

Teri,

There are many causes of liver disease. Various forms of hepatitis are but 3 causes of cirrhosis. Autoimmune, hep B and hep C. You have steatosis that has progressed. Steatosis is simple fatty liver disease and can be caused by many things. Only a very good GI or a hepatologist who knows your history and current health condition can diagnose the cause of the accumulation of fat (fatty infiltration) in the liver cells (hepatocytes).

The secondary fatty liver diseases include those that occur in other types of liver disease. Thus, alcoholic liver disease (ALD) is the most frequent secondary fatty liver disease. Secondary fatty liver can also occur in chronic viral hepatitis C (HCV), chronic viralhepatitis B (HBV), chronic autoimmune hepatitis (AIH), and Wilson's disease. (In AIH, the body's immune defense system mistakenly attacks its own liver. In Wilson's disease, an accumulation of copper injures the liver.) In all of these secondary fatty liver diseases, fatty liver is associated with other liver abnormalities distinct from NAFLD and is thought to result from liver cell injury.
Another type of secondary fatty liver disease is unrelated to other specific liver diseases. In these cases, the accumulation of liver fat is due to disturbances in the body's processing (metabolism) of fat (lipid) rather than to direct injury to the liver cells. Such causes include certain drugs (e.g.,prednisone), some gastrointestinal operations (bariatric surgery) for obesity, malnutrition, and genetic defects in proteins that process (metabolize) lipids. As described below, all of the secondary causes of fatty liver disease must be ruled out before attempting to establish the diagnosis of NAFLD. All of the secondary causes of fatty liver disease must be ruled out before attempting to establish the diagnosis of Nonalcoholic Fatty Liver Disease (NAFLD). This is why NAFLD is considered a diagnosis of exclusion.

I have met a number of patients who have had liver transplants where the exact cause of their liver failure was never determined.

I have never heard of a laparoscopy liver transplant. Liver transplant takes on average 6-8 hours of surgery it is not a simple operation. In fact it is one of the most complicated surgeries there is. Worrying about being cut open is really the least of person’s problems with End-Stage Liver Disease facing death. advanced cirrhosis destroys the body in many ways. No one that get a transplant vanity survives the progress. We learn to live with it. It is part of the disease. The only people that get transplants are those who who will soon be dead without a transplant. In my opinion, having scarring under your rib cage is trivial compared to getting a chance to remain alive. How many people get a second chance at life? Very few.

My son wants to be a liver donor and the closest facility to my location is Toulane in New Orleans, LA.  
That could help solve the long wait time issues many of us experience. Be sure to weight the risks vs the benefits and make sure you understand what could happen to your son. I had my 21 year old cousin offer. But I turned him down as I couldn't live with the small possibility of him being injured perhaps for the rest of his life in some way. Also he will have that scar for the rest of his life. So am I am still waiting for a cadaver liver. 15 months after being diagnosed with liver cancer (HCC).

“My GI said the largest problem is the Lupus factor and the unknown.”
If lupus is your main health issue, then be sure to focus on that.  

My MELD is 11 right now and my CTP score is 6 so I am hanging in there and want to keep hanging in there.  My GI said the largest problem is the Lupus factor and the unknown.  

“I have cried for the last 3 weeks so I am just now trying to start fighting back.”
That is the right attitude! We all have cried many times over the lose of your health and former lives. No one gets to transplant that hasn’t struggled with life and death issues unless they have sudden acute liver failure. Liver disease and liver transplant is not easy but it is doable. It sounds like you are taking care of yourself. There are many things you can do to maximize your health and a lot has to do with your attitude. Attitude is sometimes the only thing we have control over. We cry when we need to cry and then we pick ourselves up and fight on another day. One day at a time. Take it step by step. Liver disease can be an opportunity to refocus your life and learn how to live with serious adversity. It is not all bad. I have learned more from dealing with my cirrhosis and liver cancer then I have learned from any other experience in my life by far. And I am a better and happier person for it.

If you have particular questions feel free to ask.

Hang in there!
Hector

[Teri001]

Hi Dee,

Thank you for the welcome.  I do feel like I have been on a roller coaster for the past few weeks.  It is a lot to take in when I had no idea what was going on in my body and attributing all of the symptoms to Lupus.
I am sure I will have so many questions as time goes by and I learn more about what is happening to me.

Thanks,

Teri

[Teri001]

Thank you Hector for all of the valuable information.  I have read so many sites that I am in overload right now.  
I did not mean to sound vain by mentioning the laparoscopic surgical procedure at New York Presbyterian.  I all ready have a 7 inch scar from gall bladder removal and a 6 inch scar from a complete hysterectomy so I have my war injuries.  I was very interested in the recovery time difference but as I said I have not spoken to them and do not know if they do adult to adult surgery in this form yet.  I was thinking of my son and less scarring for him.  It is a big decision for him and I did not ask him, he has been adamant about being a donor if possible since we received the news and I encourage him to keep thinking and researching until we actually go to a liver transplant facility to start the process.
I will do what it takes to salvage what liver I have and look forward to asking many questions of a hepatologist to see what my options are.  Every week since the diagnosis has been another procedure and more tests and I want to be the best patient I can possibly be.  I have been hit by a huge curve ball as you all have and I am in this fight for my life and for the liver God gave me.  If there is a viable part I want it to survive and need to know my chances of survival w/wo a liver transplant.  I know I have cirrhosis, but do not know how much of my liver is working and what I can do to extend that life?
I pray that you will get a liver very soon and will have a wonderful success story to speak of.  You have so much knowledge to share and it is comforting to someone like me that is still reeling from the diagnosis.
Thank you so much for the welcome and I will think of questions to ask I am sure as I organize my thoughts.

Thanks,

Teri

[rpooo]

Hello Teri and Welcome to MedHelp’s Cirrhosis of the Liver Community!

I am sorry to hear of your diagnosis and surely understand your concerns since having cirrhosis also puts us in a similar circumstance.

I see Hector has already done a terrific job as usual covering all of your questions/concerns so there I little I can add. Below I posted a link for you to take a look at, there is a lot of very good information provided that will help you stay on the right track and learn what lifestyle changes are necessary.

It is not uncommon for people to live for many years with cirrhosis depending on their particular situation. Diet, exercise, medications, vitamin supplements and routine monitoring are all key in getting the most you can from your liver in its current condition.

Obtain a copy of each lab test bring it home and place it in a folder. Learn what efforts you can make to change these enzymes that are out of range. Compare each one to the one before it and see the difference you have made. This can be quite rewarding and you are the one to benefit! You can even go as far as to create a spreadsheet as I have done here to gain a better prospective of your process. http://www.medhelp.org/user_photos/show/294808?personal_page_id=1830091

----------------------------------------------------------------------------------------------------
"I have cirrhosis, but do not know how much of my liver is working and what I can do to extend that life?"

http://www.webmd.com/digestive-disorders/tc/cirrhosis-treatment-overview

----------------------------------------------------------------------------------------------------

It sounds like you are on the right track, learning all you can about this disease. We will be glad to do our best along the way to share what we have learned also :) I will be hoping the best for you Terri, take care!

Randy

[HectorSF]

Yes I am sorry that you are still going through the initial shock of your diagnosis. You have the right attitude and that counts for a whole lot.

As Randy mentioned if you post your blood test results, imaging reports etc. we can help you figure out how ill you are.
Have you had complications from your cirrhosis such as fluid build up in your lower legs and feet?
Have you had fluid buildup in your abdomen so your look pregnant?
How you ever vomited blood or had internal bleeding?
Have your eyes or skin turned yellow?
Have you go lost in a familiar place and not know why you are there. Or be unable to remember family and friends names. Have you ever gone into a coma?
These are are complications of advanced decompensated cirrhosis. Most people that are eligible for transplant have a number of these conditions repeated before being able to be sick enough to get on a transplant list. Although living donor transplants are usually done before the liver disease get too advanced. A MELD score below 25.

Of course as far as transplant is concerned it is all about your MELD score. The range is 6-40. 40 being the sickest. That is how it is determined if you need a transplant to begin with. If your MELD score is under 15 a transplant is more risky then living with the cirrhosis. So a MELD score 15 is commonly used to determine who can even be listed.

Cirrhosis covers a lot of variety of liver damage. From those who don't have complete scarring and no symptoms to those with many life-threatening complication and are on deaths door (MELD score around 40). So please try not to assume the worse. More likely than not you are healthier than you imagine. Only an evaluation at a liver transplant center will give you a full picture of your health and degree of illness.

Jun 28, 2010
"NewYork-Presbyterian Hospital/Columbia University Medical Center is the only center in the country performing removal of liver tissue from the donor via laparoscopy in certain individuals. Surgeons have used this approach to perform living donor liver transplantation from an adult to a child, and they are working to expand this program to adult-to-adult transplants in the next year."
So it is used on the donor in some cases.

I have lived at least since 2007 with cirrhosis and if it wasn't for my liver cancer, I could carry on for some time to come although after 3 years of being disabled I really want to get things moving.

Hang in there things many not be as dire as you thing.


Cheers!
Hector

[HectorSF]

Here is some info on Living Donor Liver Transplant from one of the top surgeons at my transplant center.

Living Donor vs Cadaverous Liver

http://www.ucsfbenioffchildrens.org/education/interview_roberts_living_donor_liver_transplant/index.html

Andrew Schorr:
Well, nobody wants to get there of course, and so let's take a few minutes now and understand the benefits and the risks of living donor liver transplants. First of all for the patient we understand it gives them really the chance of life and a higher quality of life when otherwise, they can be so sick. Thinking about the patient, are there particular risks for them and having a transplant from a living donor that would be any different than if it were a cadaverous liver?

Dr. Roberts:
The first thing the recipient, the patient who is going to get the transplant, should know is that there are different risks associated with getting a living donor transplant versus a cadaver transplant, and a picture's worth a thousand words.

The blood vessels in the bile ducts enter the liver with a trunk. So they have kind of a big round part of the blood vessels and bile ducts. Then, as they come into the liver, they divide in branches very similar to the way a tree branches. When we take out part of the living donor liver, we have to leave them with the trunk of the tree and the branch to the part of the liver we leave behind because without it they couldn't survive.

We can only use a branch to say the right side of the liver. We get the branch of the bile duct and the branch of the blood vessels to that side of the liver, and as with branches in a tree, the size of the branch is always less than the trunk, so that you have a small branch versus a large trunk. In surgery, when we sew things together, it's easier to sew things together that are bigger, have a bigger diameter like the trunk of a tree, than things that are smaller in diameter like a branch.

When we sew together the branches of the blood vessels and the bile ducts, there's a greater chance that things can go wrong. Most of those problems that occur are problems with where we sew the bile duct together because it's very thin and doesn't have a very good blood supply, and so patients who get a living donor transplant have an advantage in that they don't have to wait until they get really sick. They can get transplanted on an elective basis. What happens is that the risk of complications related to where we sew together the bile duct is about twice as high as if you got a cadaver liver. That's the main difference between getting a piece of a liver and a whole liver is really that the branches that we have to work with are smaller, and there are more troubles associated with them.

Now it doesn't mean that those problems lead to the patient, the recipient, losing the new piece of liver, but it can mean more time in the hospital, more time with the radiologists as we try and take care of these problems and potentially, a return trip to the operating room. But over time, all these things usually heal up and it just means more time in the hospital and in the physicians offices until these things heal.

Evaluation and Risks to the Donor

Andrew Schorr:
Of course, we should say, as patients and families consider this is your team is at the top tier of experience with this sort of transplant. So certainly as surgeons like to say, people can feel that they are in good hands, that you're going to do the very best that's available anywhere. What about on the donor side? What are things for someone who's considering being a living donor? What do they need to consider so they go in with their eyes open? What evaluation happens to make sure that they're right for this procedure?

Dr. Roberts:
Well, the first thing we do with the living donor is to 1) send them what we call a health history questionnaire. It's a questionnaire that asks them about general health issues, and what we want to do is make sure that the patient is healthy. They have to be between the ages of 18 and 55, and then they have to have a blood type that's compatible with the recipient. That means if the recipient is type O, they have to be type O. If the recipient is type A, they could be type O or type A. So there are some rules in regard to blood type to make sure that they're compatible.

At that point, if they're compatible and it doesn't look like they have medical problems, we have a number of things that happen.

One is that they seek what we call the Independent Donor Advocate. In our institution, this is a social worker whose responsibility is to make sure that the donor gets through the process with a good understanding of the issues regarding donation of a piece of liver in general and then issues regarding their specific case. They see an internist who looks at them for their general health. They see two surgeons, myself and Dr. Asher who does the donor surgery, to discuss their particular case. We do a lot of blood tests, and we then do a number of X-rays. One set of X-rays is to look at the blood vessels to the liver to make sure we can safely cut out the right or left lobe of the liver, and then another set looks at the bile duct so we can make sure that the bile duct has the right anatomy to be able to donate a piece of liver.

Not everybody has an anatomy that allows them to donate a piece of liver. That doesn't mean that their liver will ever cause them trouble. It just means that there is no way we can cut that piece of liver and safely give it to somebody else.

There is a risk of death associated with donating a piece of liver. It's about one in 500 we think for the risk of death. The risk of death of donating a kidney is about one in 3000, so this is a riskier operation than donating a kidney. The stakes are usually higher for the recipient of the transplant because unlike kidney failure, where you have a dialysis machine, in liver failure we don't have that kind of machine that allows a patient to survive until they can get a cadaver organ.

So there's a risk of death, there is a risk of complications associated with any surgery, and there are complications associated with this surgery.

There's going to be pain related to having an incision, and people really need to think that they're going to have to take some time out of their life until they get back to feeling normal. We have surveyed our donors and have asked them how long did it take you to feel 100 percent again? When the donors responded, about half of them said that within six weeks they felt back to 100 percent. Three quarters of the people felt they were back 100 percent by three months, and the last quarter took six months before they felt that they'd recovered back to 100 percent. Now, when people talk about being 100 percent that's a psychological feeling of, you know; I feel well, I'm doing everything I want to do; because people are going to be changed because they now have a scar across their abdomen that they didn't have before. So those are the kinds of things that I think the donor needs to understand about as they're going forward with this surgery.

Andrew Schorr:
Dr. Roberts, I know your team has really immense experience comparatively in doing this, and since January of 2000 you've done over a hundred of these living donor liver transplants. You must feel that the donors really have given an incredible gift and have been very courageous as they've done this.

Dr. Roberts:
Yes, I think we are always in awe of what the donors have done in terms of providing of themselves to the recipients. It really is a heroic act because people take on themselves not only a risk of death but also pain-and-suffering in order for their loved one to get the benefit of the liver transplant.

[ceanothus]

Hector, thank you so much for posting that info about transplant. I'm hoping I don't have to go there but I really appreciate the opportunity to prepare for that possibility. Teri, we are all different, and my cirrhosis has a different source than yours (mine is from hepatitis c), but it might encourage you to hear that I've had the second stage of cirrhosis since at least 2004, it is still compensated, and I can still hope that it might improve a bit if the source of the damage is eliminated. I hope they can figure out what is really causing your liver damage and stop the damage from accumulating further. Do you have other symptoms of lupus besides the positive ANA? I ask because I also have a positive ANA along with poly arthralgias, and my rheumatologist has said I MIGHT have lupus, but it is most likely an autoimmune response to the Hepatitis C virus. I send my very best wishes your way.

[Teri001]

Thanks so much for all of the wonderful advice and information.  I am trying my best as I said to learn and fight back against this liver disease.  My next big question to my GI is to see both of the ultrasounds and their interpretations by the radiologists.  My GI said that there had been a big change in my liver since my ultrasound and I did not ask which one. I am now beginning to say stop and then ask questions, making lists, and writing down answers so I will be able to be more informed.  
You all ask me some questions and I will try to answer them as best I can. For years I have had liver profiles that were out of whack even after being treated for high cholesterol, they never really settled down but I was told they were not significant for anything else, obviously that was not true.

Moving on to December 2011 my internist did routine labs and my platelets were 53000. I was immediately referred to an oncologist/hematologist and was being tested for leukemia.  We have a history of cancer in our family that is out of control so I held my breath an waited. Lab tests concluded no cancer, but inflammation of 69% and tremendous leg pain and swelling in joints, hair loss, bruising, and gum bleeding, light sensitivity. So, I was sent to an RA which promptly starting running arthritis panels as well as auto immune diseases.  I gave so much blood and he tested everything from Dengue Fever to Lyme Disease to Legionnaires etc.  I did not have the standard facial rash but had a positive ANA and a 1.8 titer along with the inflammation and several other criteria for Lupus, thus the diagnosis.

My RA started me on Prednisone and Imuran.....and the platelet count would not come back up....So he increased the doses as high as 60 mg of Prednisone and 150 mg of Imuran.  My oncologist started getting very uneasy as my liver profiles started rising rapidly.....Imuran being a very bad no no for anyone with liver disease.  Needless to say I changed RAs and believe that saved my life...I was jaundiced, my eyes blurred, light sensitivity to where I would have to squint even with just the tv on, I would sunburn in about 4 minutes, it was awful....

Now, fast forward to my first GI visit in September with the killer diagnosis of you have cirrhosis and liver disease and I hit the fetal position because I was so wrapped up in the Lupus diagnosis and how to treat it but now I cannot have any treatment until everyone consults on what is ok for me to take and not to take.

Hector, you asked me about a few things......

1.  No ascites
2.  No internal bleeding
3.  No vomiting
4.  No swelling except in joints and from the Prednisone
5.  No memories issues out of the ordinary, I have always made lists
6.  Medium Varices
7.  Portal Hypertension
8.  Jaundiced while on Imuran
9.  Mildly enlarged spleen

I wonder what long term damage Imuran could have had on me and now that I have been off it for two months if some of my labs will start looking better.  I sure hope so.

Please ask me more questions.......sorry so lengthy.....

Anyone, hear this, I read from another web site that you can lose up to 75 percent of your liver to cirrhosis and it still can rejuvenate.  What I do not understand is that the ultrasound tech told me that blood is still getting to my liver so there has to be some part that is salvageable, I just want to know what part.  



Teri

[ceanothus]

I just googled Imuran, and I am really glad you have changed your RA. Between Imuran and high doses of Prednisone, it sounds like that doctor was taking a lot of risks with your health. Have you definitely been tested for HCV? I also had a 1:8 titter for ANA, and lots of inflammation and swollen joints, and in my case all those symptoms plus the elevated liver enzymes led to my eventual dx with HCV, though I did have some detours along the way using lots of different RA drugs (but not Imuran or Prednisone). I'm sure they must have already tested you for HCV, but I can't help but think of it anyway – there just seems to be some similarities.

[rpooo]

Teri,

Often people want to know how much liver function they have left.  They are told they have 20 percent of their liver function, or 15 percent of their liver function, and people don’t understand that cirrhosis is a uniform process that pretty much involves the entire liver.  You don’t usually just get cirrhosis in one spot.  Cirrhosis can’t be removed or cut out.  It is a process that involves the entire liver.  The cirrhotic process itself is separate from liver function.  You can have cirrhosis and a normally functioning liver, although the liver is anatomically scarred and damaged.  For all intents and purposes or complications that are independent of function.

The complications it can cause are due to the fact that cirrhosis causes abnormalities in the way that blood flows through the liver, which is separate and distinct from the complications you get when the liver is not functioning.  Most manifestations of cirrhosis really have to do with the fact that blood is not able to flow through the liver normally because of the structural changes that take place in the liver.  The major complications that people run into – encephalopathy, fluid retention, varices – are related not to liver function but to the fact that there is a detour and blood is bypassing the liver in many cases.  That’s something that people have a difficult time grasping.

Randy

[HectorSF]

Teri,

I can not comment on anything but liver disease as I have no experience or knowledge of the other conditions that you speak of.

Hector, you asked me about a few things......

1.  No ascites
2.  No internal bleeding
3.  No vomiting
4.  No swelling except in joints and from the Prednisone
5.  No memories issues out of the ordinary, I have always made lists
6.  Medium Varices
7.  Portal Hypertension
8.  Jaundiced while on Imuran
9.  Mildly enlarged spleen

These items are all consistent with compensated cirrhosis.

When a patient has cirrhosis AND portal hypertension it is quite common for them to have a low platelet counts. (My platelet counts have never been above 70,000 since 2007). The platelets are sequester in the enlarged spleen.

"People with cirrhosis end up having a problem with portal hypertension and an enlarged spleen, and a reduced platelet count in the bloodstream. As time goes by, the liver may try to repair itself by growing new cells. If there is a lot of scar tissue already present--- the new cells grow between scar tissue (and result in abnormal nodules). (The nodules and scar tissue can further interfere with blood flow through the liver). So over time people with advanced cirrhosis can end up having a problem with more and more abnormal nodules and scar tissue forming...which interferes even more with blood flow through the liver.....which makes the spleen continue to enlarge....and the platelet count continue to drop."

"The obstructed flow of blood through the portal vein (portal hypertension) causes the spleen to enlarge by causing an increase in pressure inside the vessels of the spleen. The spleen stores red and white blood cells and platelets (fragment of cells that are used to clot blood). An enlarged spleen traps platelets lowering levels in the blood which can lead to the inability of blood to clot. This is called Thrombocytopenia and is common in people with cirrhosis. "

"The Natural History of Cirrhosis
http://www.clinicaloptions.com/Hepatitis/Annual%20Updates/2008%20Annual%20Update/Modules/Garcia-Tsao/Pages/Page%201.aspx

There are 2 main stages in the natural history of cirrhosis: compensated and decompensated cirrhosis, defined by the absence or presence of ascites, variceal hemorrhage, encephalopathy and/or jaundice. Compensated and decompensated cirrhosis are 2 separate entities with distinct clinical presentations, prognostic indicators, outcomes, and mortality.
Cirrhosis represents the end stage of any chronic liver disease and can remain compensated for many years before the development of a decompensating event. Decompensated cirrhosis is marked by the development of any or all of the following complications: variceal hemorrhage, coagulopathy, hepatic encephalopathy, ascites, spontaneous bacterial peritonitis, hepatorenal syndrome, hyponatremia, hypoalbuminemia, and/or hepatic encephalopathy. Because of its poor prognosis, the only effective therapy for decompensated cirrhosis is liver transplantation."

"Anyone, hear this, I read from another web site that you can lose up to 75 percent of your liver to cirrhosis and it still can rejuvenate.  What I do not understand is that the ultrasound tech told me that blood is still getting to my liver so there has to be some part that is salvageable, I just want to know what part."

All organ in the body have more capacity than they need to function properly. The liver can lose a large percentage of its functioning liver cells and still manage all of its functions. 80-90% liver must stop functioning for hepatic failure. This is why just having scarring doesn't cause the liver to fail. When you have compensated cirrhosis as you do. Even though many of the functioning liver cells (hepatocytes) no longer function due to them being scarred you have no complications of your cirrhosis. When the functioning be impaired we call that decompensated cirrhosis. This is went the liver can no longer carry out all of its functions. This is when the complications of cirrhosis manifest themselves. Ascites, bleeding varices, hepatic encephalopathy etc. etc.

"What I do not understand is that the ultrasound tech told me that blood is still getting to my liver so there has to be some part that is salvageable, I just want to know what part."

Blood is always getting to the liver if not your liver would fail and you would die. Like when the blood supply is cut off to the brain. The brain tissue dies along with the patient. As the liver gets more scarred with time less and less blood flows through the liver. It then starts flowing through collateral veins. The is what varices are.

The bottom line is you are not receiving the best healthcare that is available for your liver disease. Cirrhosis is a very complicated disease and can potentially be fatal if not managed properly. The best thing you can do for yourself as all cirrhotic patients should do is to get a hepatologist at a transplant center to manage their liver disease. The fact is a gastroenterologist is not qualified to manage a patient with cirrhosis, period. A good gastroenterologist would tell you as much.
I would go to The Tulane Abdominal Transplant Clinic in New Orleans, LA and get evaluated to find out if you are ill enough to be listed. You may not be that ill but at least you will know your current condition and they will follow you over time until you are ill enough to be listed. It could be a relief t know you don't need a transplant any time soon even if you in time have a living donor transplant.

"A transplant evaluation is the process by which you prepare for transplant. It includes a history and physical exam, psychosocial assessment, nutritional consultation, and financial evaluation. The first appointment will take approximately 1-2 hours. This is just the first step in the evaluation for many patients, as you will need additional tests and consultations with other physicians. Your Transplant Coordinator will be able to tell you what additional tests you may need before you leave after this first appointment, but sometimes we may not be able to tell you until after the whole team reviews your case. The formal liver transplant evaluation usually takes 2-3 days and is done on an outpatient basis unless an emergency situation exists.
Your Transplant Coordinator will call you after all your results have been received and have been reviewed by our team at our Candidate Selection Meeting. This is a meeting of the whole team to review your case and to make a decision about transplant for you. The liver transplant selection committee meets weekly. Within a week or two after we have received all the results of your tests, you can expect to hear from us. We will first get clearance from your insurance carrier before we place you on the waiting list. Once we do, you will get a letter in the mail telling you this. We will also send a letter to your referring physician and/or primary care physician. If you do not receive a letter from us, you should call us."

Your gastroenterologist or primary care physician can make the transplant referral, but anyone, including you, can call to make the transplant appointment.
When you get a hepatologist you will know the status of all of your health conditions and will have someone who can answer all of your questions over the years that you will probably have cirrhosis.
You have already had one doctor who may have damaged your liver more by using medicine that is toxic to the liver. When you have a hepatologist ALL changes in medicine, diet, etc. that can impact your liver must be approved by them first and they will work with your other doctors. To continue to rely on unqualified doctors is a risk no person with cirrhosis should ever do if they want to maintain their health for as long as possible.

Cheers!
Hector

[Teri001]

Thanks, for all of your encouragement and advice.  I am scheduled to see my GI again on Tuesday and at that point we will decide which transplant facility and hepatologist I will be seeing.  It will most probably be Toulane for expense purposes as well as the living donor questions.  
I saw my internist on Thursday and he was the first doctor that has said something positive about the way I look and that I have a bumpy road but a winnable one.  I needed to hear that, I have been so down and over stuffed with information.  
So, I am going to try to take a breath and step away from the obvious.  I have so many other problems besides my health to take care of and my stress level is at capacity.  
Thank you for the support.

Teri

[Teri001]

HI,

Yes, I am having a hard time grasping all of this.  It will take time and a new way of thinking to understand the depth of this diagnosis.  As I said before I had just grasped the Lupus diagnosis and all of the treatments thus far for the Lupus have gravely impacted the liver so it will be a rough ride.

[Teri001]

Thank you so much for your thoughts and concerns.  I have been tested for all types of hepatitis and all are negative.  There is not much to be done as this point until I see a hepatologist and hope that I have a much more rounded view as how to treat several conditions at once.  Right now they are all running together and I have no idea how to separate them nor do the four physicians I have.  I am ready for one person to take lead and call all the shots along with myself to get the best possible care for someone dealing with broad spectrum problems.
I hope you do not have Lupus on top of everything else.  It is a complication that you just do not need but if it turns out you do, then I am sure your doctors will address it very quickly and will be very careful not to put you on medication that will make your liver any worse.
I was in a catch 22, because the Lupus was finally diagnosed first so all meds went toward remission for that disease and in the mean time the dropping platelets were not even related to the Lupus but to the liver and spleen and it has made things definitely worse.
I would stay away from Imuran if at all possible and I am sure your doctors would not let you near it anyway.  The side effects alone of that drug are horrid and of course it just hurt me in my individual case.  
Best of luck on that and please keep me posted.

Teri