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A History of Hemophilia A: A Bleeder’s Story (Part 5)

Editor: We have an update for you on our story tracing one hemophiliac’s journey with Hemophilia A and Hepatocellular Carcinoma. This is a personal story, so we’ve striven to keep his identity private. This is only one person’s journey and will vary greatly from every other story out there. There are plenty of similarities however, that we can enter into the story and perhaps sympathize with him, perhaps feel good about his victories and perhaps reflect on our own stories in the process.

“Since last we met I’ve had an eventful summer and am looking forward to a healthy fall. Fall has always been my favorite time of year, in spite of some personal tragedies that have struck our family in the fall. I like the cooler days and the beginning of frost. I love Thanksgiving as it is the epitome of family time and family is by far the most important thing to me these days.

“So, to continue my story where I left it in mid-July, we were looking forward to successful treatment with Lenvima (lenvatinib) to keep new tumors from establishing blood supplies, thereby keeping them from growing or perhaps even leading to their death. (Poor tumors.) The Lenvima may have very well been a helpful treatment, and indeed it may prove to be so in the future as well, but I didn’t tolerate it well. It works by messing with the formation of blood vessels and that doesn’t mix so well with a nice, severe, hemophilia diagnosis. I was able to tolerate it for several weeks before I began to bleed.

“Lenvima doesn’t seem to cause general bleeding and I don’t think I am any more susceptible to joint bleeds when I am on it. What it causes is GI tract bleeding, particularly in the nether regions. In other words, after several weeks on the lower dose of Lenvima, I had bloody stools, which sounds like a horrible Halloween game of musical chairs. We stopped the Lenvima and gave my body a couple of weeks off to regroup.

“Thinking this might be a one and done effect we tried the Lenvima again, still at the two-thirds dose that I was tolerating. This time it was only a couple of weeks before the bleeding came up again. My oncology team consulted with my hematology team and came up with a plan. I have been using extended half-life clotting factor for about four years now. The plan was to switch me to Hemlibra. Hemlibra is a topic all by itself. It may be worthy of an examination by the editing staff here at some point in the future.

“Hemlibra is not clotting factor, but it does work pretty much the same as clotting factor. Factor VIII has one major job, as I understand it. It takes factor IX and factor X and joins them together. That’s it. Other factors have been working on the clotting job before this step and still other factors continue to work until the clot is formed. Without factor VIII, the whole process falls apart and you don’t clot. Hemlibra is a protein that accomplishes one task: take factor IX and factor X and join them together. Sound familiar?

“So major differences between Factor VIII and Hemlibra? The one that has the most impact on hemophiliacs taking the treatment is the half-life. Factor VIII, at it’s best has a half-life of 22 hours. This means that you lose half of the injected Factor VIII every 22 hours. This is an average and individuals have very different results. This is why it’s imperative that you have a PK study done to see how factor stays in your body after a dose is given. This allows you to tweak your dosing to make sure you are covered with your prophylactic doses. Hemlibra has a 646 hour half-life. This means that instead of losing half the product approximately every day, you lose half of it in about 4 weeks.

“The result of all this half-life discussion is that when you take your factor on  your recommended dosing schedule, your factor soars up to the top of the chart in about an hour. Then you lose half of that in less than a day. By the time it’s time to take the next dose of factor, your levels are really low. My doctor’s have tried to keep my levels above 5%, which eliminates most spontaneous bleeding episodes. With Hemlibra, you should maintain a fairly straight efficacy line. From what I understand, and for reasons that are beyond the scope of this paper, they aim to keep you between 20% and 30% of normal clotting capacity. These percentages are from the best of my memory, which isn’t good to begin with. Don’t use these figures without verifying them!

“The purpose of all that is to explain that the hope in switching me from factor to Hemlibra was that without the deep troughs in clotting coverage perhaps I would not experience the bleeding that had occurred on factor. It looked very promising, until it wasn’t any longer. In September we resumed treatment with the Lenvima, having completed the switch to Hemlibra and the loading doses, which is the first four weeks on Hemlibra.

“We started with one third dosing of the Lenvima for the first two weeks and then increased to two thirds dosing again. Almost immediately I was back in the hospital with the very familiar symptom of blood in the stool.

“Since then my time has been spent recovering and planning our next move. What we have come up with is to restart the Lenvima on the 10th of October at only one third the dose. We will try to continue that dose, thinking that something is better than nothing. We’ve discussed adding perhaps a clotting aid such as Tranexamic Acid to keep bleeding at bay, perhaps at half dosing. This is not unheard of but I’m also not sure that it is a common use of that drug. In any case the coming days will tell us a lot about where we’ll be in a year.

“One note about the Hemlibra: I have heard anecdotal evidence of many who have had zero bleeds since beginning Hemlibra. That was not the case with me. I have had one questionable and one definite bleeding episode, in addition to the GI bleed in the short time I’ve been on Hemlibra. I’m an old guy with lots of hemophilic arthropathy, so maybe it’s not that surprising. I just want to point out that Hemlibra isn’t necessarily the be-all and end-all of bleeding. Individual results will definitely vary. My activity level has been a lot higher as well because in general I’ve felt a lot better on Hemlibra.

“I’d like to add that through this all I have maintained an attitude that this is just something that needs to be addressed and I have nothing but deep respect for the men and women on the teams that take care of all of my many health issues. I don’t know what lies ahead, but I do know that I have a wonderful life with many good family and friend connections that give meaning to what would otherwise be a rather dreary existence. My advice? Keep your loved ones close, and if you don’t have many, make new ones. That’s the best antidote for poor health I have found.”

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