Diagnosed in 1981 at 26 years old

I came down with FHC myself, probably before most of you were born. It was diagnosed, on July 10, 1981, by the late, Dr. Thomas E. Starzl. I’m sure many of you have heard of him as he is known as the “father of organ transplantation”. Now, at the time, I worked on Wall Street in New York doing Financial Analysis. One night, while living in New Jersey, I just felt like I had severe stomach muscle cramps – and I could not fall asleep. Now mind you, for the previous 3-4 months, I had been having some digestive issues, and had experienced loose bowels over that period of time but did not think I was sick. Anyway, I drove myself over to a local ER at a hospital that was maybe 2-3 miles from my apartment. Once they sort of went through triage, the admitting doctor did just that, to my shock. He said he didn’t know what was wrong with me, but some bloodwork indicated I was anemic, so he admitted me.

Over the course of the next two weeks, the docs conducted a variety of tests, only to discover a tumor in my abdomen. My father, who was doing consulting work for a client in the area, did some checking/asking and found this liver specialist in New York City at Mt. Sinai. His name was Dr. Fenton Schaffner. Dr. Schaffner upon hearing something about my condition, told my father that it sounded to him like surgery may be needed, and that he should get in touch with a doctor in Pittsburgh, PA; Dr. Thomas Starzl.

So, off to Pittsburgh we go, on Tuesday, July 7, 1981. We arrive at the hospital, Presbyterian University Hospital, now known as UPMC Presbyterian. Dr. Starzl was the most unassuming person you would ever want to meet-(and was also the most amazing, interesting, thoughtful, driven person I ever met). Sort of acted like he had not a care in the world. He asked a few questions, did a cursory exam and left. He came back to my room the next morning to announce that he had scheduled me for surgery for Friday afternoon, July 10.

Now as you know, if you are having surgery, that means no food. So all day Friday I am just hungry, but waiting to be taken down for the procedure. We go, they put me under, etc. I wake up, now it is maybe about 4:30 or so. My parents say they have some bad news, and the doctor couldn’t remove the tumor. So, a bit flustered, I said – well, what next? A minute or two later, (maybe it was 10 minutes, can’t remember), Dr. Starzl comes in…he is very apologetic but tells me that he could not get the tumor as it had completely enveloped my liver, and getting the tumor would mean probably taking a good 65% of my liver, and he thought that would probably not be successful anyway. Later, I find that I had a Fibrolamellar tumor, and in subsequent years, Dr. Starzl told me that I had the biggest liver he had ever seen. So, he doesn’t miss a beat, and says that his suggestion for a safer procedure, would be to do a liver transplant. A liver transplant? It is 1981, who had ever heard of a liver transplant? Sure, I knew about heart transplants and kidney transplants – had heard of those and had heard those were kind of dangerous things. But, a liver transplant, and the doc is so matter-of-fact about the whole thing; so, I said, “sure”. Then, I asked him, “when can we get that scheduled because I really need to get back to New Jersey?” I’m sure he just looked at me, sort of incredulously, and replied “as soon as we can”. Now, I didn’t know that I had to wait for them to a) find a donor (only cadavers then) and b) the donor had to be a match to my tissues, blood type, whatever. But I was the most naive person when it comes to medical issues, or healthcare in general, that you would ever meet. I thought a liver transplant was no more serious than a tonsillectomy to be honest with you.

It is now probably, oh 5;15-5:30 on Friday the 10th. My brothers and sisters had come to Pittsburgh by now – seeing as it is Friday, and my best friend from childhood came as well, he lived in St. Louis at the time. We all hang out on Saturday. I don’t feel great, kind of weak, but don’t really feel that sick either. We did some walking around the hospital and I think Dr. Starzl peeked in and talked to me a couple of times. About 5:45pm or so, right after I have eaten dinner; the nurses on the unit come to inform me that they hear that a liver has been found for me. Great I think, (although then getting me ready for surgery was not fun). Anyway, a couple of minutes later, Dr. Starzl comes to my room (I think I was in room to myself but really don’t remember), and says a few things… yes, he has found a liver and he is going to need to go and get it soon. OK, then he asks if I have any questions or concerns and tells me the nurses are going to bring me a consent form to sign, and again asks if I have any questions. None really I tell him, so he starts walking to the door saying “well, I need to go and get the liver”, remember still speaking in this very calm, matter-of-fact tone, and I said, “don’t drop it”. He turns, and I think gives me that same incredulous look and walks out.

The transplant went great, no issues, no rejection, nothing. The procedure took about 18 hours because my liver was just so big, but when I woke up the next day, Sunday the 12th, I was already on the road to recovery, which took another month while in the hospital and then 3 more weeks before I started graduate school in September. I think that I was just so much at ease because Dr. Starzl just gave off this aura, basically, that he was going to will everything to be successful for me and that I would be fine. And it was, he just refused to let me die, and seeing as he spent so much time and energy on my case, I always had this fear of failing him by getting sick or, worse, dying from side effects of my liver transplant. He was driven, and so was I. I followed directions, never forgot my meds (I think I have missed maybe 25 doses in total, since my liver transplant). About 2 1/2 weeks after the transplant, my parents asked him a question in the hallway outside my room. He dragged them into the room and had them ask again. They were concerned for my long term well being, Dr. Starzl told them, right in front of me, that I would be” bouncing my grandchildren off my knees”. I did not ever think otherwise, but they obviously had, no worries (but no grandchildren yet, I married at 40, have a son who is 22 and a daughter of 16).

Now, it is almost 38 years later
, there are maybe, what say 10-12 people in the US who have had a liver transplant longer than I have, and all of them were transplanted as children, I was the first successful adult liver transplant recipient.

Unfortunately, UNOS regs now prohibit cadaver liver transplants for patients with FHC, don’t know about living donors. Many of the liver transplant docs think that is somewhat ridiculous as that was my diagnosis, I had the transplant and have not had issues since (although toxicity from my immunosuppressant, CyclosporinB caused kidney issues that led to kidney transplant in 1999).

Thanks, God Bless you all.