End stage liver disease

@twocatmama, Welcome to Connect. I am a transplant recipient. 10 years ago I received both a liver and a kidney trnsplant due to PSC (Primary Sclerosing Cholangitis) I had 3 of the symptoms that your loved one is experiencing. It doesn't seem to matter what name is given for the liver disease, or whether it has no known cause (idiopathic liver disease) like your loved one, the symptoms are commom for all of us.

Is your loved one receiving care for his liver disease? Is he being consideded for a liver transplant? What are some questions that you would like to ask?

He is still being managed with diruetics, a thoracic catheter which we drain daily up to 1000 ml. He just feels terrible. He’s lost nearly 70 pounds and has neuropathy which causes him to walk very slowly with a cane. The condition hit him like a cyclone last October with no warning. He is being followed by a transplant hospital. Initially he was told he was good for 5 years, but they might be rethinking that now. Doctor said if the current treatment doesn’t work, they would consider a shunt before the transplant list. I think my question has to do with life after transplant,

@twocatmama, Unfortunately, no one can predict how long it will be until our liver fails to where we need a transplant. And no one has any promise of the outcome if /when we get one. I will share a little of my story
When my symptoms hit, they also hit with a vengence. I had no other health conditions, but my liver disease took both my liver and my kidney. I was placed on dialysis and had other interventions along the way. It was a difficult period of time for me and for my husband, my caregiver. My transplant team was exceptional in their knowledge and care that they provided. My family and friends gave me the strength to keep on going when I felt like I couldn't go anymore. One of my best decisions ever was when I said that I wanted to be evaluated for a transplant. Because of my transplant, I am healthy and active once again.

You are not alone in your search for answers and support. I want to invite some others who have experienced the liver transplant process as a recipient or as a spouse of a recipient. I post the question that you asked, "How is your life after your transplant?"
@jodee, @gaylea1, @rrichardparker, @parmcat, @des46893, @mickj, @frankw54, @gary1864, @rodney9999 , @contandwell

Hi, @twocatmama and I also welcome you to Connect. I am a liver transplant recipient, my transplant occurred in September 2016. I presume if the diagnosis is idiopathetic, he does not have cirrhosis? I had non-alcoholic cirrhosis (also called NASH cirrhosis). It seemed to come on suddenly, having "mental changes" -- hepatic encephalopathy -- was what brought it to the forefront. Prior to that I did have other symptoms but they were not diagnosed as cirrhosis symptoms. These were declining platelet counts (which was considered to be ITP - "idiopathic thrombocytopenic purpura"), fatigue, shaky hands (which was diagnosed as "essential tremor"), excruciating cramps in my thighs -- these were infrequent, thankfully, but when I had them they were horrible, and fluid retention in my feet at times. That seemed to happen primarily when I traveled. All of these should have added up when I had my first episode of hepatic encephalopathy, HE, but unfortunately it took a long time before a doctor did finally put them all together. After my diagnosis I went to a hepatologist and she told me that I probably had cirrhosis for 10 years, it takes a long time to present itself.

Are the mental changes this person is experiencing causing them to be irrational and possibly combative? Mine did, and I ended up in the hospital a few times with them prior to finally having a diagnosis. It seems as if the symptoms a person has can vary from one individual to the next. If you have any questions, please feel free to ask. Those of us who have been there are happy to be able to help anyone who is experiencing these problems or to help the person who is a caretaker or closely associated with the person.

After transplant all of the symptoms disappeared. Life is good and I am grateful every day for how well I feel now.
Please let us know how this person is doing.
JK

I waited 17 months on the wait list and 2 year from diagnosis until I received my liver. The symptoms prior to surgery were quite intense and I couldn't wait to be transplanted. While I was nervous about the transplant surgery I came to terms with it knowing it was my only hope. I was extremely calm when my call came as I was more than ready. The surgery itself was 8.5 hours but I came out of it feeling great. The actual recovery from that surgery was quick and relatively painless. My staples were removed 4 weeks after surgery. I had complications post surgery and it was a difficult time as I underwent 4 ERCPs and one major surgery. These have been resolved. I am now 7 months post transplant and beginning to feel lile my old self. My transplant team and family kept me going through the entire process.
My life is so much better now as the 2 prior years were awful. If you have questions or concerns this group is always here to share their experiences and supply tips on the whole process. I wish the best for all those waiting for a transplant.

Hi,

My brother is 40 years old has been diagnosed with cirrhosis . His liver is failing and has been having episodes of high ammonia as high as 331 as of this morning. Its the 3rd time now and the doctors pretty much have it under control .. but my question is ...Is this a common issue? Ammonia getting that high and for my brother its every week. He is on a strict diet and takes all of his meds and has bowels movement often, sometimes up to 10 times a day due to the lactulose. He will stay in the hospital for 3 days at a time then out for 5 then back to the ICU again. Its so horrible to see him go through this. His episode seem more severe every time. He cannot be put on a list for transplant until he is clean at least 6 months.. his last drink was 04/26/21. if anyone has information that can share pls do. We are loss. He seems to do well one minute then the next he is in ICU with his ammonia issue. ..thank you

@mandosssss, I would like to welcome you to Mayo Connect. I think that your brother is a lucky guy to have you as a sister. Being diagnosed and enduring the difficulties of cirrhosis is an awful thing to experience, both as a patient and as a loved one. I was diagnosed with a liver disease that caused my cirrhosis and my eventual transplant in 2009.

Your brother is fortunate that he has a dedicated group of doctors who are working with him to get his ammonia levels under control. The condition that you describe is caused by a buildup of toxins in the brain (hepatic encephalopathy). A liver damaged by cirrhosis isn't able to clear toxins from the blood as well as a healthy liver can. These toxins can then build up in the brain and cause mental confusion and difficulty concentrating. The lactulose is the medication that is used to control this buildup. However, it does come with bothersome side effects as you are witnessing.

I want to invite you to join a discussion in the Transplant Group where @contentandwell, @jodeej, @gaylea1, @amyintucson, @kltchrmn have shared their experiences with lactulose for hepatic encephalopathy.
-Lactulose question
https://connect.mayoclinic.org/discussion/lactalose-question/

Will your brother be evaluated for a liver transplant after the 6 month period?

@mandosssss @rosemarya
I too welcome you to Connect. As Rosemary commented, your brother is very fortunate to have you looking out for him.

I had HE episodes somewhat frequently until I went to a hepatologist and she prescribed xifaxan for me. When I started taking that I was able to drop lactulose for about 8 or 9 months and had no HE episodes. When they resumed I had to start taking lactulose with xifaxan, obviously my liver had deteriorated further.

Something I did not do myself but I think could be helpful is to track what your brother is eating, and when he has an HE episode see if there is a common food that could be leading to it. I think that perhaps beef was a problem for me. I was told it was OK to eat it in small quantities, which I did, but maybe I was just more sensitive to it than other people with cirrhosis.

My cirrhosis was from fatty liver, not alcohol, but once you have cirrhosis it doesn't matter what the cause was, it's all the same. I had a number of hospital stays, generally two or three days each time. I went to my local hospital which of course is not as knowledgeable about cirrhosis and HE so frankly the treatment there was not good, they just seemed to think I was a belligerent old lady. If you are close enough to the hospital where he will be getting a transplant I would advise you to have him brought there.

I really empathize with what you and your brother are going through. Having HE episodes was the worst part of cirrhosis for me and even now, almost 5 years after my transplant, thinking of those days brings tears to my eyes.

Please feel free to ask any questions you need to have answered, either here or by private message.
JK

Hi,
Thank you so much for responding ...Yes my brother is on a strict diet low sodium, no beef and since he is diabetic low sugar everything. He has had an issue with his ammonia levels for the last 3 weeks, 1st week 200 ammonia level then 314, then last week 336. Every single time he ends up in critical care. In a state like coma for at least 2 days. So very scary and painful to see.

He is on a lactulose and antibiotics and now on L-Ornithine-L-Aspartate , it is supposed to work hand N hand with the lactulose. Getting these ammonia levels in order is so hard. You all give me hope that he will one day have a better quality of life.

We have had issues with getting a doctor and specialist that can work together with us. I read about liver teams. Is this something that hospitals have? or is it just a way of classifying your own team.

@mandosssss Is your brother seeing a hepatologist yet? If not that should be the next step I think.

Even though he does not yet qualify for a transplant he could be seen at a hospital that does transplants, they know the most about all of this. That's where the term "liver team" comes from. You have a team of doctors who coordinate together. It generally involves a hepatologist, a transplant surgeon, and possibly other specialists if he has other existing problems that need to be addressed prior to being a transplant candidate. I would imagine in his case there might be someone who could be helpful to him in his battle to stay away from alcohol. Prior to transplant, after I went through all of the tests to qualify, I only saw my hepatologist.

Has any mention been made of xifaxan? For me, and from what I have heard from others, that seems to be the most effective remedy for HE. It is very expensive but depending on his insurance the cost might be doable. I was on Medicare and still had to pay about $700 a month for it until I hit the "donut hole" and the cost went way down. With a drug that costly it doesn't take long to get to the donut hole. I have no idea how private insurance handles the cost.
JK