Book 5, Chapter 2, Verse 11
[My apologies to anyone who has been trying to follow my story and who might have been wondering why I haven’t updated it in a while. I started to compose it (in my head) last March, actually started writing it in June, but then got distracted by some travels, some minor illnesses and, well, just being a bit lazy when it came to actually sitting down and writing. But, now I’ll get it done.]
This story is getting pretty boring. But, that’s a good thing. Even a great thing, as that means things are going so well that there’s rarely anything to report that seems very interesting. I am way overdue in getting an update out and appending it to my on-line blog, but I just haven’t felt like there was anything of interest to report. But, now it’s been so long that I’m getting messages asking if I’m okay. And, there have been some changes, so an update is in order.
Last March of this year I celebrated two anniversaries. They were both very significant and the first was entirely the result of the second one.
The first anniversary I celebrated was that as of last March, I have been living with Chronic Lymphocytic Leukemia for 13 years! I don’t know if that sounds as incredible to you as it is to me. But when I first got sick and was diagnosed with CLL, I truly didn’t expect to live more than another 5 to 7 years or so. Many of you know that my father had the same disease and died within about five years. When he was diagnosed, back in 1976, there was no cure for the disease. And, 25 years later, when I was diagnosed, there STILL was no cure. I fully expected to be dead in a few years.
But many things have happened. I was fortunate enough, if I can use that term, to get this miserable disease at just about the time that multiple researchers were finding some more effective treatments. Not cures, mind you, but more effective treatments. Up until that time there were many good folks who advocated for not even trying to treat the disease. The feeling was that the patients would die within about 6 years, whether they were treated or not. So, the reasoning went, why torture them with treatments which aren’t going to help them live longer.
But, I was able to get a number of experimental treatments over the years that have kept me going for more than my expectations. I won’t go into the details, as they are all recounted in some excruciating detail on my blog postings, but I have been able to go from one new treatment to another, and just when it has looked like I was out of options, new options have appeared. I am one lucky guy. One of my friends in Miami says I have reminded him of a surfer who just keeps catching another wave when one runs out. Yeah, it’s really been something like that.
But that brings me to the second anniversary I celebrated in March. About four years ago I got some very, very bad news. I had been dealing with CLL for about 9 years at the time, and my treatments had been fairly successful at keeping the disease at bay for a few years at a time. That was at least in part because I had one of the more favorable chromosome types. This stuff is described in detail elsewhere in my blog, but there are at least five or more major different chromosome types among CLL patients, plus many other complex mixtures of chromosome types. I had, up until that time, a normal male chromosome type, the 46XY chromosomes. But, about four years ago I found that the disease had mutated to a different, much more deadly type called the 17p deletion (also described in detail elsewhere). With this mutation, which does not typically respond well to chemotherapy, my life expectancy was said to be 12 to 18 months, if untreated…and most treatments weren’t expected to work.
So, with my back against the wall, and with no good treatments to fall back on, I prepared for the only real option I had left, a stem cell transplant. Now, these transplants (also called bone marrow transplants) can be lifesaving and many folks are alive today only because they had such a transplant. But, and this is a big “but,” many folks have died from attempting the kind of transplant I would need. The death rate for a transplant from an unrelated donor is said to be anywhere from 25%-50% or so, depending in who you talk to. But, with no other options, I was evaluated in the transplant clinic at M. D. Anderson and signed all the papers to authorize them to start the search for a matching donor.
But, the very next day, which was in March three years ago (the other anniversary I celebrated last March), I was accepted into a clinical trial of a new drug, a drug so new that it didn’t even have a name, being called simply PCI 32765, its developmental code name. It had been tried on about 140 other very sick folks and seemed to be working very well for many of them, so I signed up to be in the trial, one of the next 40 patients to try this new stuff.. At the time, I was very sick, having large tumors in my belly, chest, under my arms and in my neck. But, within the first week of starting this new drug, which I was taking in combination with Rituxan, I could tell it was working. My swollen belly was shrinking and the lumps under my arms and on my neck were getting smaller.
And, for me, it has kept on working. I have now been on it for over three years, making me one of the patients who has taken it the longest. There are still about 90 folks out there who have been taking it for over four years now, but with every day, we’re making history, proving this stuff works and helping to find out how long it might continue to work.
It’s not a magic bullet for everyone, however. Of the 40 folks who were in my study group, within two years four had died. I don’t know if it was from disease progression (like Richter’s Transformation), bleeding or infection, or what, but 10% of my group is no longer with us.
The side effects have been, at least for me, fairly minimal. I have had intermittent but persistent pains in my joints, usually in the small joints of the hands and feet, but also sometimes in the larger joints as well; wrist, knee, hip, etc. The pains can be significant but are usually transient and last but a few days before disappearing. And I have days, many of them, when I feel significantly tired, even very, very tired. But there can be more serious problems. This drug, now called ibrutinib, and more recently approved by the FDA for general use and trade-named Imbruvica, can also cause heart rate abnormalities, most commonly a condition called atrial fibrillation, or A-fib. I have had two episodes of A-fib over the last year and a half, but both times the condition resolved on its own within less than 24 hours, so I didn’t have to be cardioverted, or “shocked” to restore a normal rhythm.
During the last two years, this medication has worked so well for me that my blood counts are near-normal, with my white blood cell count being about 5,000 at every monthly blood test (normal is about 3,000 to 10,000 or so). So, since my counts are staying normal, and because I am having, at least intermittently, a significant side effect like the A-fib, Dr. Keating has reduced my dose of ibrutinib from the usual three capsules daily, to just one capsule daily. We’ll see how my counts look after a month or so and see if I can stay on the reduced dose or whether I might need to go up to two capsules daily.
As an aside, I should mention that when this drug was first studied in the very first 140 patients, the researchers were comparing a dose of six capsules daily against a dose of three capsules daily. They found, early on, that three worked just as well as six, and caused fewer side effects, so all patients from that time on were given the lower, three capsule dose, which is what I was started on.
However, no one, to my knowledge, has done a controlled study to compare how effective one or two capsules are compared to the standard three daily capsules. I know that since the drug has become more widely available, after the FDA’s approval last year, many folks or their docs have tried reduced doses of the ibrutinib. In fact, one of my CLL “pen pals,” who was having horrible rashes with the drug, completely on his own, and not even at his doctor’s recommendation, reduced his dose to two daily and his blood counts have continued to improve over the last year, even though he had very high white blood cell count numbers when he started on the drug. Dr. Keating noted to me that he has a number of folks on reduced doses, many even as low as the one capsule daily that I am now getting.
This could be significant on many fronts. First, if this lower dose works as well as the three capsules a day, then one could expect fewer side effects with no apparent reduced efficacy. Secondly, this drug is VERY expensive. The list price for ibrutinib is $92 a capsule in the USA, or $276 daily. That’s close to $100,000 a year. If patients could be eventually maintained on just one capsule daily, that would be a significant savings for the patients and their health insurance providers.
I’m happy to have been so stable on the ibrutinib, as I truly think it has saved my life, but while I’m doing well on it, I am having some other issues. Anyone who is diagnosed with CLL is told, early on, that they are now at increased risk for a second, or more, malignancy. For men, the greatest risk is for skin and prostate cancers. Well, a couple of years ago I developed a skin cancer, a small squamous cell carcinoma of my left forearm. It was easily taken care of but now I have to get twice a year full body checkups from my dermatologist.
But, more recently, my blood test level for my PSA (prostate specific antigen) has gone up, more than double last’s number. A visit with the urologist revealed a possible small irregularity of my prostate. But, a repeat blood test a few weeks later showed that the blood test had returned to normal levels. I just had a third test and will see the urologist yet again in a couple of weeks to see where the level has gone. It looks, however, like I’m going to have to have some prostate biopsies to see if I have a prostate cancer, or to prove that I don’t. I don’t really care for the idea of the biopsies, but it probably shouldn’t be TOO big a deal. I hope.
Okay, I’m back and I’m going to finish this up. But first I need to update the “updates,” as things have changed yet again. First, let me follow up on the prostate issue. Since I wrote all this I have had a series of prostate tests and the levels have gone down, down and down, to completely normal levels. This has happened after a couple of courses of antibiotics, so one presumes that the cause of the abnormal PSA tests was a mild prostate infection. So, no prostate biopsies yet, and I’m to get another PSA blood test next January followed by an exam. Hopefully all will still be normal.
Secondly, the trial of taking just one capsule of the ibrutinib daily didn’t last very long. After one month, my white count was up to 6,700. Now, this is a completely normal number for most folks, but my white blood cell counts had been consistently between 4,500 and 5,500 for the last two years or so on the standard, three capsules daily dose of the drug. So, the rise to 6,700 might have been significant. I reported this result to the folks at MDA and I was put on an increased dose of two capsules daily, about five weeks ago.
But, just as I was starting this new dose, I got sick with a cold which progressed to a bronchitis. I’ve been coughing for several weeks now and finally started some antibiotics when I was well past the usual viral stage of the cold but was still coughing. So, last week, when I got my next blood counts done, my white blood cell counts were up to 10,400, a major increase. Now, this is still a normal number for most normal folks, but it’s higher than I’ve been in a few years, since I started the ibrutinib. The question is, did the white count go up because my CLL is no longer well-controlled at the two capsules a day dosage, or did it go up because of the bronchitis. After a bit of discussion with the folks at MDA we decided it was most likely the result of my infection, since bacterial infections can elevate your white counts, as they go to work trying to kill off the invading bacteria and such. We’ll see if the counts are still elevated when I get my next blood test done at the end of September.
I have mentioned, in the past, about the possibility of getting a CAR-T procedure, the “chimeric antigen receptor-T cell” procedure in which one’s own T white cells are harvested and modified to fight leukemia cells. It has been used in some small studies around the country with varying success, but it’s not yet ready for prime time, I’m afraid. In fact, the MDA CAR-T tests have gone off the rails. It appears that a large pharmaceutical company hired away the lead investigator and then gave MDA a large grant, and in doing so, created a conflict of interest which MDA’s lawyers are looking into. So, for now, CAR-T is not looking like it will be an option in Houston for a while.
But other therapies are coming on strong. First, there have been a couple more oral agents approved for CLL. This gives us more and more options for treating the disease, even if one of the drugs (like ibrutinib, as an example) fails, there are others to go to, like ABT 199, now known as Venetoclax. There are several patients who have been on ABT 199 until they achieved a complete remission and then, very bravely, stopped the medicine. In at least the several months since they went off the drug, the disease has failed to return. This is amazing.
And just as exciting, I think, is the advancing of the Immune Checkpoint Inhibitors. We have known for a while that in CLL and other cancers, the body’s own immune system fails to control the disease as it should. Researchers have found that there is an inhibitor on the surface of the patient’s T white cells, the ones that normally search out and control abnormal cells like cancers, and this prevents the T cells from doing their jobs properly. But, now antibodies are being developed which block this immune inhibitor, turning the cells loose against the disease. This process has been used successfully against several solid tumors, like melanoma and renal cell cancers, and the thought is that these antibodies should work well against CLL cells as well. We shall see. I’ll probably talk about this a bit more in an upcoming message.
I want to finish up with the mention of one more anniversary I’ve had recently. Last month Kathy and I celebrated our 46th wedding anniversary. That’s another truly amazing anniversary, as when I first got sick, I really didn’t expect us to be able to celebrate our 40th anniversary together. But, now I’m doing so well, and there are so many wonderful new treatments for CLL, that I’m beginning to think I’ll be around for our 50th anniversary. This has been an important union for us, and I couldn’t have gone through all the trials of dealing with this disease without Kathy at my side. She’s been with me every step of the way and has sat by my side, hour after hour and day after day as I was getting chemotherapy and feeling sick afterwards. It’s so wonderful to have her to rely on. And, we’ve had so many wonderful friends throughout this journey who have also given us support, love and prayers all these years. We have been truly fortunate.
And this has gotten too long…again…as my stories always do. But I’ll be back when there are any changes in what’s going on and maybe I’ll be able to be just a bit more concise in my writing.
“Life must be understood backwards. But it must be lived forwards –Soren Kierkegaard
PS: If you’re the praying kind, please take a moment to remember our friend and fellow TNT Teammate and Honored Hero, Doug Campbell and his wife Stacey and their daughters, in your prayers. Doug has been in Team in Training for many years and completed many distance events with the team, raising money for the Leukemia and Lymphoma Society even while battling an aggressive, incurable lymphoma. Several years ago, when he exhausted his chemotherapy options he began looking at a stem cell transplant, but a good match could not be found. His family organized many bone marrow donor drives, which will likely help many other folks in the future, but still a good match for Doug could not be found. Finally, about four years ago, I believe, a partial match was located. It wasn’t great, but it was all that was available and Doug needed treatment desperately. So, he underwent the transplant but very soon afterwards began having significant Graft Versus Host Disease (GVHD) complications, wherein the transplanted cells attack the patient’s organs. Doug has battled valiantly for several years now, getting through one crisis after another, with Stacey ever by his side. Ironically, the transplant has cured his “incurable” lymphoma but its side effects are beating Doug up badly. Now Doug is in severe heart failure, has compromised lung functions, diabetes and more, and is not doing well. He was offered the possibility of a heart transplant, but has decided that enough is enough. He has decided to forego further treatment, having fought the good fight for so many years now. His family hopes to get him home so they can be together for what precious little time he has left. He is a wonderful guy and is deserving of your prayers and so much more. Godspeed, Doug.