CLF: Let’s start from the beginning, can you discuss how you were diagnosed with PSC and your experience living with the disease? 

Jefferson: I was diagnosed with PSC in 2001, loosely tied to bowel disease, which I was diagnosed with at the same time. But, if I reflect back, I had symptoms as early as the 1980s. Being a young adult at that time, I wasn’t inclined to sort things out with my health and I ignored the issue. My take – don’t pretend that something that isn’t normal, is normal. Now as a manager, I encourage my employees to investigate the issue and seek out help and answers when they are not feeling well. 

I eventually went to see my general practitioner because there were times that I wasn’t feeling great. I was a social drinker like many people, and I didn’t feel great after drinking certain types of alcohol and eating certain types of food. I realized that fatty foods and alcohol weren’t for me, and I ruled them out after the year 2000.  

After my diagnosis, I took prescribed medication and visited a liver specialist yearly, and basically lived a comfortable life for 20 years. I would ask my doctor if there was anything more that I could do to manage my disease and would be told to “keep doing what you are doing, look out for signs of jaundice, and one day you will need a liver transplant.” I continued to stay active during this time, taking up many outdoor activities including sailing, skiing, cycling, and running. 

CLF: Can you share about your turning point when a manageable diagnosis became an urgent need for transplantation? 

Jefferson: Everything was fine until it wasn’t fine. In March 2019, I started feeling drained. I was tired all the time and I had digestion issues. I just wasn’t feeling great. At first, I didn’t associate it with PSC. I chalked it up to working too hard and not getting enough sleep. One day my boss stopped me to say I didn’t look well, and I joked that she was working me too hard. 

As this feeling of unwell got worse, I started booking appointments with specialists. One day, while still waiting for my appointment with one specialist, I looked down at my legs to see that they were swollen. I didn’t recognize my lower body. I recall looking over to my wife and saying that it was time to go to the hospital. I still didn’t have jaundice, so I didn’t think it was PSC at this point. Blood tests at the hospital showed that I had crazy high liver functions – ten times the upper limit – and low hemoglobin. I found out that I had been bleeding internally for months. 

From this point on to early 2020, I was in the hospital almost every two weeks from internal bleeding. If I look back at photos from this time, it’s literally a family ski trip, a hospital visit, a vacation, and then the hospital again. I was coordinating my life around frequent trips to the hospital; I needed a transplant. 

CLF: How did you go about finding a living liver donor? 

Jefferson: During this time of frequent hospitalizations, we realized it was time for some self-advocacy. My wife knew someone who had just lost her husband to the same disease, and he sadly ran out of time waiting for a liver. We were diligent about researching and learning more about the liver, PSC, and transplantation. We sought out mentors to talk about the journey and what timelines for transplantation were like. My wife heard that Dr. Gideon Hirschfield, one of the University Health Network’s (UHN) hepatologists, was speaking at an upcoming patient meeting and she wanted me to go. I was hesitant to go at first, because I had a hard time being honest with myself about the state of my diagnosis but, went I did. 

I pulled Dr. Hirschfield aside at the start of the talk and after asking him some questions, I realized I needed him to be my doctor. First steps were to meet with the pre-transplant team at the UHN. With assistance from the team and my new hepatologist, I made it to the transplant list on February 1, 2020. PSC usually doesn’t have a high MELD Score, so living donation was my best chance as I needed a transplant to live. Unofficially I was told that I was looking at 48 months to live if I didn’t get a transplant. 

Time was of the essence. I was told that it takes up to six weeks to work up a potential donor, and you often need eight potential donors to find a full match and only one donor can be worked up a time. It was incredibly humbling asking for help, and I felt all kinds of emotions during this time. I wondered if I was worthy of a donation. I felt guilty about someone else undergoing a major surgery for my gain. I also worried that no one would answer my call for help. 

We spread the word widely until the day that we were told that my friend Tim was a perfect match. Tim is my hero. Strangers, friends, and family came forward to put their name on the list. The principal at my son’s school even shared my story and call for a living donor in their weekly newsletter. 

CLF: What would you share with someone in a similar position?

Jefferson: I would say to get the word out wide and well. My wife created a website and email that were both ready to launch for the day that I got on the transplant list. Time on the transplant list can be a touch unbearable as you are not notified of how many potential donors have come forward or where they are in the process of testing for a match. Be patient and be prepared to wait. Trust the process and trust humanity – others will help.