Primary Biliary Cholangitis

UPDATE AUGUST, 2021

I was admitted to the local hospital for bleeding varices on Sunday 8/8/21 and was transferred to the IU Health University Hospital 8/9/21. I underwent surgery to stop the bleeding on Tuesday 8/10 and was told I could possibly get a liver within the next few days due to my MELD score climbing from 27 to 36.  On Thursday 8/12 around 10:00 I was told I had to go NPO at 13:00 for a possible procedure but my nurse couldn’t tell me why. We started getting excited and were waiting for my transplant coordinator to call. All of a sudden these two super perky nurses came in with their “Donate Life” shirts on display. They said there was an organ procurement that afternoon and they needed to draw 40 tubes of blood to get me prepped in case the liver was in good shape. We were so excited and called my dad and brother who dropped everything at work and drove the 3 hours to get to me as soon as possible.  Then the waiting game started.  We were so anxious. My bedside nurse had never gotten anyone ready for transplant so he was just as excited as us!! He kept checking every hour and finally around 18:15 we found out the liver was good!!!  They rolled me to the OR around 20:30 and started the countdown at 21:00.  My liver was placed early in the morning Friday the 13th (my new lucky day)!!   By Friday afternoon they already downgraded me from ICU status and I walked in the hall Friday afternoon and got up to a chair that evening. On Saturday I walked to my new transplant stepdown room.  I started doing laps Saturday afternoon and continued getting up and moving as much as possible. By Monday night I had walked 17 laps (3/4 mile) and when my doctors came in on Tuesday morning they said everything looked great and they would be sending me home later that afternoon!! We were stunned. At 4 days post op I was discharged.  My follow up labs looked great on Thursday and I got for my first all day clinic visit on Monday.  We are so very grateful for the staff at IU Health Transplant and my donor and their family. They truly saved my life during the hardest time of their lives. I am going to keep pushing myself and get better every day.  I will continue my work with the ALF to promote liver health and living liver donation. I also plan on volunteering with #DonateLifeAmerica as well!!

UPDATE, JULY 2021

By 2017 things still were not improving and I started have vision issues and was taken off the new medication and they continued to adjust my medications, changed my work schedule, etc. I went from being a 33 yr old nurse working as a charge nurse on a busy open heart ICU to working 8 hour shifts during the day and eventually had to transition to a new area due the toll the stress was putting on my body. In December 2017 I married my best friend and we had plans of having children and building a long life together. At an appointment in May 2018 I was told it was too dangerous for me to try to have children due to my liver showing cirrhosis (permanent damage) and they also diagnosed me with Auto Immune Hepatitis overlapped with my PBC and added my medication regimen. It was a crushing blow to know I could not have my own children but my husband and I decided to see how I did on the new medications and then look into adoption if things improved. By late 2019 my numbers continued to worsen and testing showed my liver was fully cirrhotic. My doctor said he was going to try using everything he could to help prevent needing a liver transplant. My spleen was enlarging and causing anemia and issues with my blood and I started getting transfusions several times a year to help my levels. In October 2020 we were told there was nothing else that could work and I need to start testing for liver transplant. I met with my transplant physician and started a battery of tests to make sure I would tolerate transplant.

On February 15, 2021 I was officially listed for liver transplant. Unfortunately PBC/AIH does not normally result in a high MELD score so I was listed pretty low in ranking. Since that time my score has been increasing and I am having more symptoms, fatigue, nausea, GI issues, etc. The good news is that in July 2020 IU Health University Hospital started doing living donor liver transplants and I have been approved to receive a living donor transplant. Now I am waiting to be matched with someone who is altruistically donating or someone that volunteers to go through the testing to match and donate with me. I am needing to find someone who has Type A blood and fits the parameters. I feel like my life is in holding pattern right now waiting for transplant and going through the roller coaster of symptoms and health issues that go along with cirrhosis and ascites. My husband and I would love for me to get better and be able to adopt and expand our family once I am well!

Hopefully by sharing my story I will find my liver buddy or someone else waiting to get a liver will get matched as well! I know everything is in God’s hands and things will work out how they are supposed to. I want to help inform the public that they can be a living liver donor. The liver is the only organ that can regenerate and after donating a portion of liver the donor’s liver will return to normal size in about 2 months! Please consider signing up for testing in your state if they offer living liver donors and help improve the lives of the many patients waiting for liver donation.

Learn more about Living Donor Liver Transplantation by visiting the ALF “Greatest Gift” Living Donor Liver Transplant Information Center.

ORIGINAL POST

In February 2014, I started itching like crazy, and after 5 months of not getting any answers other than “allergies” or “you are imagining the itch,” I landed in the Emergency Department with jaundice of the skin and eyes.  I was told that I had hepatitis, and to “deal with it.”

I pushed for a GI consult, and the physician that heard my case immediately ordered labs and called me that night stating that she had an idea about what was going on, but that she was waiting for confirmation.  She also prescribed medicine for my itching.  Four days later, I was in her office, and was told that I had Primary Biliary Cirrhosis, that there was no cure, but my symptoms could be treated.

As a cardiac ICU nurse, it was hard to digest having a disease that could not be fixed.  I am used to giving a medication or treatment, and seeing results in 5-10 minutes.  Since I had never heard of PBC, I started googling it immediately. Bad move.  Googling medical diseases only brings up the bad stuff.  Scares the bejeezus out of people.  I then went to the American Liver Foundation website, and immediately got some relief and found support.

Luckily, I had a fantastic doctor who listened to my concerns, and a family that was willing to do whatever necessary to keep me healthy.  The fatigue I was experiencing was incredible.  I had to switch from being a 12/hour night shift RN to days, then decrease my hours to 8hr/day.  It definitely helped, and I have been blessed with wonderful coworkers that have helped me through that transition.  I remember listening to the American Liver Foundation’s PBC webinar, and thinking “wow, I am not crazy other people feel this way too!”

In November 2014, after watching my LFTs continue to climb, my doctor said that it was time to be referred to a transplant center.  Talk about scary.  My mother and I immediately rallied the wagons, and had the prayer circles going, as we waited to get in with a transplant center.   I couldn’t believe the hoops that I had to jump through to see a specialist.  IU Health had a 3-4 month waitlist, and my insurance would only pay for their facility to treat me.  We took a leap of faith, drove down to Vanderbilt, and met with a wonderful doctor who made some medication adjustments until I could get in at IU Health.  Two weeks later, one of the physicians that I work with made some calls and got me into IU Health.

With my medication adjustments, my labs were already better, and I was not considered for a transplant.  Hooray!  By February 2015, my LFTs were the lowest that they have been in almost a year.  In June, my numbers jumped up, and I met with my IU Health liver specialist.  He has recommended me for a drug trial that has been amazing so far.  They are seeing this medication reversing cirrhosis, and practically curing PSC and PBC.  I am being screened right now, and I hope I will be lucky enough to get the trial medication.  Either way, by participating in the trial, I know that I am helping other people like me who are needing a better treatment or cure.

This has definitely been an eye-opening experience.  I definitely take the LFTs into account when I am caring for my patients, and now I have firsthand experience dealing with insurance and trying to get patients into specialists.  I hope that I can provide support and comfort to people dealing with PBC and other chronic diseases.  The American Liver Foundation provided much needed answers, comfort and support in my time of need.  I can’t think of a better cause to be associated with and to raise money for.  I know that there are lots of people in the community dealing with much more severe liver issues, and I hope that we can provide them the comfort and support that I received, and hopefully continue to fund research for a cure.  I’ve been an EMT, RN and now I am a physician extender.  I love to help people, no matter what I am going through.  Life is much better with a smile and a positive attitude!  If I can touch one person with my message, and make a difference to one person, that makes my day.