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A History of Hemophilia A: A Bleeder’s Story (Part 4)

Editor: This is the next episode of our continuing story about one person’s life with hemophilia. He’s an older guy, so many of his struggles are a thing of the past. However, new challenges are always around the corner and so it can be beneficial to hear other’s stories. Their lives may be a template for us to follow (or not to follow, depending on outcomes) in our own struggles with life in general and hemophilia in particular.

“I’ve left the most difficult of my health issues for last. The reason for this is two-fold: being the most difficult issue to face it is also the most difficult to talk about, and it is an ongoing issue, so I don’t really know what the end of the story is. I can only tell you about the struggles I’ve had dealing with it to date.

“To the best of my recollection the final issue began in the spring of my junior year in high school. No guarantees that is correct. I have lost a lot of my short-term memory and even my long-term memory is not what it once was. I had a pretty bad pain develop in my right side. We decided to get it looked at by my HTC hemophilia docs, because it was unlike any other pain I had. They determined that I had ‘non-A non-B’ hepatitis. I spent a couple of weeks in the hospital and recovered. I didn’t think too much about it until much later.

“I think it was in the early nineties that they classified Hepatitis C. They must have had some way of typing it long after the original infection. I know that eventually they had the ability to do viral load testing for Hepatitis C. Perhaps that is how they nailed it down. To me, it was just a piece of information to put in the back of my mind. When at last in the 2000’s they came up with interferon treatments for Hep C, we tried with no success to use those early treatments. My first attempt was with interferon and ribavirin if memory serves. We saw an immediate drop in the viral load, then it leveled off, and eventually started to climb back up to where it was: an unsuccessful treatment. It would have been nice to get rid of this pesky virus, but it wasn’t a big deal to me. I had survived this infection for the better part of two decades. No big deal.

“The next attempt to annihilate the virus was with PEGylated interferon, which made it stay in the system much longer and they hoped it would be a success. Alas, I must have had the mother of all hepatitis C versions because the results were almost identical. The lifechanging event came in 2013. A new type of drug became available known as protease inhibitors that showed real promise. We know that when the trial started for the version I got I had a hepatitis C viral load measured in the millions. We started the protease inhibitor with a mix of hope and trepidation. I didn’t learn this right away, but they told me later that my one-week viral load was zero. I had gone from millions of copies of the virus to zero in one week.

“After my 12 weeks of treatment, I had to wait a full year to be declared free of hepatitis C. And that’s just what happened. Whew. That was one last thing to worry about. Less than a year after that I would have the subdural hematoma that was a life changing experience all its own. Two years after the subdural hematoma they found a small (less than 2 cm) mass in the upper lobe of my liver. Not really a cause for concern at that size, but certainly something we would pay much closer attention to. I was hooked up with a new HTC that handled liver transplant as well as hemophilia, and I was enrolled with the liver transplant team. I still see my long-term HTC for hemophilia treatments.

“Now here, my memory isn’t so great, but I will tell of the watch period as best as I can. We took MRIs of the liver every six months, with a CT scan on the odd three-month periods. We watched it diligently. We watched it for three years. There was no change in the size or composition of the mass. We began to hope that it was a benign mass of some sort. Three years with no change was a big deal.

“A year and a half ago, the mass changed dramatically. It burst its capsule and grew about twice its former size. It invaded a blood vessel in the top lobe of the liver. There was now no longer any speculation. It was cancer. It was hepatocellular carcinoma. It was serious.

“We had only one tumor and it was rather contained. We were concerned about the blood vessel invasion, but I quickly learned that, whereas in most of the body veins carry blood to the heart to be recirculated, in the liver veins carry blood into the liver to be cleansed. That is my understanding, but it could well be that all blood is carried first to the liver and then from there on to the heart. So, a portal vein invasion didn’t necessarily mean that the tumor cells would have an easy route out of the liver. Subsequently we seem to have been incredibly lucky in that tumor cells appear NOT to have left the liver.

“We decided the best route to take was something called a radioembolization of the liver. This is a procedure where, accessing the liver through the femoral artery and working a tube into the direct area of the tumor, they place a number of radioactive beads into and around the tumor itself. Sounds simple, right? I gather it is not so simple, but my interventional radiologist is one of the best in the world. He was able to place the beads precisely where they needed to be to kill the tumor and allow almost all the rest of the liver to remain functioning normally.

“It was a successful procedure, and we went back to monitoring the liver. The thought was that we would have at least gained a couple of years in the fight against the cancer and perhaps have dealt a knock out blow. Yay!

“After ten months of close follow-up and monthly treatments to boost the immune response to any cancer cells remaining in the liver, we discovered something else that was very disturbing. In the upper lobe but dispersed from the original tumor site were six new tumors.  These were small but we felt it would be best to deal with them as quickly as possible. These new tumors were discovered in December of 2020.

“In January we decided the best route would be chemoembolization, a procedure very like the radioembolization we used before, but placing instead of radioactive beads, small amounts of a chemotherapy agent with blobs of a fatty nature that would block the blood vessels. The tumors were too diverse and too many to successfully navigate a radioembolization procedure without killing off a large portion of the liver. We underwent the procedure in the middle of January, 2021. By that time it was 10 (or possibly 12) tumors that were treated.

“I am a hemophiliac, so I am familiar with pain. Lots of pain. Pain primarily in my joints. The radioembolization introduced me to something called referred pain. I learned that the liver itself doesn’t feel pain, but that it refers pain to nearby areas. The trick with referred pain is that you can’t track the source of the pain, because it isn’t where it feels like it is. And when you try to put your hand on the pain, it isn’t there. It’s a good way to drive yourself crazy, feeling pain that isn’t actually there. The pain from the radioembolization was about 12 out of 10. The worst pain I had ever felt up to that time. Knee bleeds, ankle bleeds, elbow bleeds (for me the worst of all) paled in comparison.  Even the cryptococcal meningitis, which had redefined pain for me at the time I had it in 1994, paled in comparison to the pain of the radioembolization. Granted, the pain was slow onset, something like 4 or 5 weeks from the embolization. It did last a couple of weeks or maybe longer. I thought I now knew what real pain was and it wasn’t friendly. Now, with the chemoembolization, came pain that was easily twice as bad as the radioembolization pain. It was not 4 or five weeks in coming to fruition either. Maybe three days at most. Now the good news about it is that it only lasted a little more than a week. The bad news is I never imagined so much pain could fill a person. Now I know that there are hundreds of terribly painful procedures, and many are probably worse than chemoembolization, but I certainly hope I never meet them. This was ‘two Dilaudid or two Oxycodone every two hours’ pain.

“Pretty soon they started thinking about new ways to torture me. Ahem, I mean new treatments to keep the tumors from coming back. Obviously, the immune therapy we had tried had been unsuccessful at keeping the tumors at bay. We tried a drug called Lenvima. I can now laugh a little at this. It was sorta like taking a wound, say from being skewered by a flaming hot length of iron, and trying to make it all better by sewing the wound up with no anesthesia and using flaming needles to do the sewing up with. That was a normal dose of Lenvima. We cut it by a third. Now I am tolerating the Lenvima with little to show for it besides some general fatigue. I’m beginning to see an end to the pain at least. Hopefully the Lenvima will be as effective as advertised. My most excellent interventional radiologist says brightly that if the tumors reappear, we’ll just treat again. And again. And again. I’m not so sure about that. I don’t really know if I survived the last treatment. I think if my count is correct I’m on something like life number 11 of my nine lives.

“Here’s looking forward to number 12. I know that this is not the end of the story for me. There may be many treatments in the future, or we may have knocked out the worst of it and be able to go on in a maintenance mode. I may have many years left and lots of work to accomplish, or the end might be close by. In any case, I intend to live my life to the fullest and enjoy every moment!”

Editor: This may or may not be the end of this story, perhaps a pause. We’d like to keep up with progress and share in triumphs and struggles. We may have more of this story or perhaps some others would care to share their inspirational stories. All stories will be handled with the utmost care to preserve the anonymity of the author.

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