In 2003 at the age of 38, I was diagnosed with primary biliary cholangitis, or PBC. I went for routine bloodwork which came back showing crazy high liver function and cholesterol over 400. My PCP sent me to a very young, new, “cutting edge” GI who immediately told me it was PBC and sent me for a biopsy to stage the disease. I was staged at 2. My GI then sent me for a consultation with a hepatologist in NY to make sure he was prescribing the most up to date treatment.
My first thought was that I had been given a death sentence.
I was 38 years old with a 6th grade daughter and a high school freshman son. I was beyond frightened. There wasn’t much information at that time and only one medication, which I immediately started on. In the beginning the fatigue was so bad that all I wanted to do was sleep. I had horrible migraines and nausea. The itch though was by far the worst symptom. It was nonstop and felt like thousands of ants crawling all over my body. I tried so many different things to stop it or at least to quiet it. I will admit to feeling suicidal. I felt so alone and couldn’t bear the thought of living like this. I started on a medication to address the itch, which had a calming effect. It didn’t take it away; it just made some days more bearable.
I have a large but very close family. I met my husband while still in high school. He has been the biggest source of my support along with my two children and now grandchildren. I made a conscious effort to live my life as normal as possible. My son was active in the music department and my daughter played sports. I tried to be present for any activity. My husband and I had a large pool of friends, and we did a lot of fun things from watching our children together or having parties, going away together, etc. All the while, I tried to keep myself educated about my disease and any new developments related to it.
There is a bit of a stigma, or at least there was at first, because the name of my disease used to be primary biliary cirrhosis. People just assumed you were an alcoholic because of the name. Thankfully early on in my diagnosis the name was changed to cholangitis. Still people hear liver disease and make assumptions. It’s difficult enough to deal with a progressive disease without having to explain to people it is autoimmune rather than alcohol related.
The disease has no cure. It is a progressive disease. I could tell that things were changing as I would get nauseous and vomit occasionally for no reason. I never knew when it would happen. We would go out for dinner and before the meal was even served, I would be so nauseous that I would have to take it home without eating. My doctor told me about a new drug coming down the pike that looked promising. My Alk phos and ALT and AST blood counts were never normal. They were always quite high even after years of being on treatment. After much discussion, we decided to try the new medication. My Alk phos did come down a lot but never returned to normal, and my other counts went higher. I stayed on the new drug since it was bringing my Alk phos down. We don’t know if I was put on it too late, or if I just had a bad reaction to it, but I suddenly was getting sicker. My joint pain became unbearable, and I was basically always nauseous. I stopped taking the new drug and went back to the first one.
Later that next year, I woke up one morning vomiting blood. It was my 54th birthday. I went to the ER and was admitted for a GI bleed. They banded the varices in my esophagus and released me after 4 days. I went home and literally gained 18 pounds in a matter of hours. I was now having trouble breathing and went back to the ER within a couple of hours. My blood pressure tanked. The ICU doctor was called in to take control of my care. It was at that moment the scariest moment of my life. After 10 days, I was released but very sick. My PCP whom I saw after being released told me he thought I was now in a downward spiral.
A couple of months later my daughter said, “Mom you are yellow”. I called my GI immediately and he then sent me to NY Langone to consult about a transplant. I saw the head hepatologist who then had me meet the transplant coordinator, surgeon, and social worker. They told me they would list me but that it would probably be a year at least before I would have a new liver. I have O+ blood, which is a universal donor; however, I can only receive an O+ liver. From there everything moved extremely fast.
My consultation day was May 7th. By the end of May I had 4 episodes of hepatic encephalopathy which is caused by high ammonia levels. Each episode I was hospitalized until they could bring my ammonia level down. I somehow went to all my tests and appointments and was listed June 1st. On June 4th I had another episode and was admitted until I could get a liver. I was very sick. By June 14th, they admitted me to the ICU. They had to administer a feeding tube. I had ports in my neck for dialysis as my kidneys were failing.
I knew I was dying. My husband and I had the “talk” in case a liver didn’t come available.
After two weeks in the ICU, it was getting harder to breathe on my own, and they intubated me. There are so many variables in getting the transplant that we know it only happened because God intervened. It must be a healthy liver and be O+, but it also had to be the right size for my body. I also had to be healthy enough to withstand the surgery, yet sick enough to warrant getting the liver. Somehow by the grace of God and only a miracle, a liver became available that was a match. My sweet husband also never left my side. He slept there and was there anytime I opened my eyes. My children came often, and I know that is what helped me fight to live. I was in the ICU for about 6 to 7 weeks before being healthy enough to be transferred to the acute care floor.
My rehabilitation after was brutal. I had to learn to talk, walk, feed myself, bathe myself, and go to the bathroom by myself. It was the worst time, but because God left me here, I knew I had to do everything I could to honor him and my donor. What a wonderful, selfless gift someone can give. I believe my donor saved 3 lives that day including mine.
No one prepares you for life after transplant. It is difficult to say the least. Unfortunately, I also did not know that transplant is not a cure for PBC. So, in 2021 a year and a half after my transplant, my bloodwork showed elevated liver enzymes. A biopsy confirmed that I indeed have PBC again. I immediately started on treatment again. Some say I should never have been taken off medication in the first place. I am now close to six years post-transplant and four years with PBC. So far, I don’t have any of the horrible symptoms. I suppose since bloodwork has been done monthly since transplant that it was caught as soon as it started. I will admit I was crushed when I found out. But I try to remember that I would have been dead 6 years ago and that I was left here for a reason. My husband and my children needed me. I was alive to see the birth of my second grandchild. I helped my daughter through a terrible divorce. I have seen my oldest granddaughter grow from a little toddler to a little girl. I adore my family and am blessed to be alive. I pray that when and if the symptoms of PBC come to me that I can once again fight. I am 60 years old now. While I don’t like the number, I am blessed with each birthday I celebrate.
My hero with no name is my donor. I don’t know anything about him or her. But I try to honor them every day by living.
My advice to anyone dealing with PBC is first educate yourself. There are more options available now. Know that it is not a death sentence. I am now going on 22 years with this disease. There is hope! Find support groups. It truly helps to talk to people who are going through what you are. Find good doctors. Get in with a Hepatologist. Live your life to the fullest. Hopefully new research will come through with a cure for this awful disease.
AUTHOR: Lisa Lehr
