Any trip to BATCC where you get good news is a good trip. My good news is that, in spite of my apprehensions, I am pretty stable and doing well after 15 months on the Ruxolitinib. My White Blood Cell count is high, of course, but less than the last trip as last time I was coming off the pneumonia and the relapse. Apparently the Ruxolitinib pulls collected cancer lymphocytes from your lymph nodes and releases them into your bloodstream, which raises your WBC, but reduces your lymph nodes in size. Mine are noticeably smaller. This is a good thing.
Chronic Lymphocytic Leukemia and Small Lymphocytic Lymphoma are the same disease. The different names are used to differentiate how it expresses itself. The CLL shows up primarily in your bone marrow and blood with some swelling of lymph nodes. SLL shows up in your bone marrow and the lymphocytes tend to collect primarily in your lymph nodes, making them swell so you look like a chipmunk that has filled his mouth with too many peanuts. No one, however, with the swollen lymph nodes thinks of it as chipmunkish; there’s no cuteness about it.
Relapses of CLL tend to lean more towards the SLL side, though they do not have to. One can have CLL/SLL at the same time. The disease is very personal and makes itself at home in your body in its own unique way, since it is your chromosomes and genes that have mutated, and though many mutations are identical from one person to the next, yours can have its own twist that makes it unique to your body. Your cancer is as unique as your DNA.
So, here’s what follows: I still have CLL. I will continue to have CLL. It is progressing and in all likelihood will continue to progress, but it does not have to. I am doing well on the clinical trial with the Ruxolitinib. If and when I fail to do well on the Ruxolitinib, or the trial is discontinued, and my blood numbers go south (bad) or north (up/still bad) at an accelerating pace, they are prepared to treat me with new front line therapies and they expect the treatment will produce another remission. Since I was in remission for over five years, my chances of a second remission are very good. The more one is treated, the more probability of one becoming refractory, or resistant to treatment. One’s unique cancer can become quite crafty, learning to resist treatment. Our cells in our bodies sort of have a life of their own, reproducing themselves, with their own will to live. They react to poisons by allowing the newer ones that have mutated and become poison resistant to survive. The poison resistant ones reproduce and multiply while the poison sensitive ones die off.
The particular life-saving poisons I took for chemotherapy was a triad of drugs. Two of them were truly chemotherapy drugs, Fludarabine and Cyclophosphamide. The third was Rituximab, an immunotherapy drug that targeted a protein called CD20, which is only found on the surface of the cancerous Lymphocytes. Rituximab also targets a few other proteins and is used to treat Psoriasis, Psoriatic Arthritis, and Rheumatoid Arthritis, all autoimmune diseases where your B cells become confused and attack your own body tissues like sailors on shore leave attack the portside watering holes, rather than attacking and destroying real pathogens. Rituximab is quite useful.
Since I was treated (in 2010), they mostly no longer initially use any chemotherapy agents, though they can if they need to. Medicine is a practice and different oncologists and hematologists practice differently. Today, most CLL treatment is immunotherapy consisting of an immunotheraputic drug called Ibrutinib, or Ibrutinib combined with Rituximab or a third one called Idelasib. There are several clinical trials involving the use of Ibrutinib with a newer drug called Venetoclax. This seems to be the direction things are headed. Dr. Gooday said he would treat me with Ibrutinib and Venetoclax if I needed treatment now.
With all the remarkable things happening with CLL treatment there are still pitfalls, as the CLL can morph into something worse, whether a worse form of CLL called Richter’s Transformation, an aggressive and hard to treat form with a poor prognosis, or a genetic change unique to you, producing higher risk forms of CLL that are more resistant to treatment (such as a deletion at chromosome 17p or 11q). While I do not have a 17p or 11q deletion, it does not mean I can’t develop one. What I do have is an elevated level of an enzyme called ZAP-70, which is tested for to show the real risk called Unmutated IgVH, since there is a correlation between elevated Zap-70 and Unmutated IgVH status. I had the mutation status done early in my CLL experience, and the University of California San Diego and Dr. Kipps, one of the top places and one of the top CLL researchers, confirmed that I am Unmutated IgVH. I also have elevations of enzymes CD-38 and Beta2 Microglobulin. All of these things are what are known as prognosticators, and while they are used to predict outcomes and provide physicians with some direction on how they should go about treating the CLL patient, they are not guarantees of anything. CLL patients are living longer. I am one of them. I am beyond the median for my particular subset of CLL and am one of those who are helping the median climb.
I am three years past the median survival rate for those CLL patients with Unmutated IgVH. I can explain to you the mechanisms of how the B-cell receptors in Mutated versus Unmutated IgVh works, and how they work with our own T-Cells to help program the B-Cells to do their job properly, but you don’t want me explaining it, nor do you likely want to hear it. CLL is a disease of the B White blood cells. They already don’t work properly and grow until all your bone marrow will produce is bad B cells. When I was treated the first time, 78% of my bone marrow was involved, and this happened rather quickly, accelerating once it started, though it turns out I had had the CLL longer than anyone thought, as revealed by an unrelated CT Scan done two years before my diagnosis. That CT scan showed enlarged lymph nodes and an enlarged spleen though the radiologist did not see that at the time, so intent was he on finding a kidney stone.
Well, you need lots and lots of Red Blood cells pretty badly, and T-Cells, Neutrophils, and NK cells, and platelets, and plasma. You can’t live on bad B cells alone, nor even good B cells. Red blood cells and their protein compound Hemoglobin have to carry the oxygen to your tissue cells so they can carry on with their cellular respiration, else a formerly living you winds up to be merely a collection of dead cells. Red Blood cells are why your blood is red. Your white blood cells only collect where you have an infection, though they are legion in your bloodstream. We have an ugly vernacular name for them. We call them “pus”. What an ugly name for something so valuable as your white blood cells. You’ll die quickly as you watch too many of your red blood cells drain out. You’ll likely die in a couple of days without your white blood cells, or the complete loss of any one of the several types of white blood cell. Every component of your blood is vital.
With that said, there’s still lots to worry about. I could worry over it until it paralyzes me with fear, but allowing myself that self-indulgence produces nothing good, doesn’t help deter the cancer, nor help me live a productive life. We worry about today. There is enough for today that we cannot spend the time or energy to focus on what MAY be in our future, as we do not know. I have every expectation that things will continue as they are, or if not, effective treatment will be available. Yes, my already unfavorable prognosticators can turn even more unfavorable. Yes, I can get into the mode of needing treatment more often, with each round being less effective, causing more complications until I become entirely refractory, meaning nothing works anymore. Then, the outcome is grim. But the outcome of my pneumonia in April could have been grim. The heart attack I could have could be grim. A fit of apoplexy could overtake me in the grimmest sort of way. The drive to work in the morning could be grim. The inadvertent 40,000 volt electric shock I got last Thursday from a piece of my own test equipment could have been grim. I survived, though I daresay I will keep more distance between me and the energized test lead than I did last Thursday, which is another story for another blog post.
We cannot and must not yield to grimness. I won’t, at least not until I have to. I understand all the negative things that can turn on a dime, but I will worry about them when I face them. Right now, I am still thinking about the girl in the elevator at BATCC. She had grimness on her mind. Her mother was filled with cheerful platitudes that served no purpose other than to make her very ill daughter feel worse because she could not even entertain the thought of them. I doubt they helped the mother, either, though a wise friend of mind pointed out that that may have been the mother’s only coping mechanism. We all cope as best we can. Some are better at it than others. We must lend a hand where we can, and frequently a hand is better than a word. Ask Job if you will. He’ll tell you.
At any moment, I expected her mother to say, “When the going gets tough, the tough get going.” I expect her daughter may have vomited on all of us in that elevator had her mother said that. I wouldn’t have blamed her if she had.
Shown smiling with me is my wonderful Advanced Practice Research Nurse Alice. She is Dr. Gooday’s right arm. Alice has my vote. She is a healer. She loves me, but she loves all her patients, though I like to think I am special. Of course, being beyond the median makes me special. I hope to maintain my special status in spite of unfavorable special statuses that are determined to foil that. It is my hope, I said.
No grimness here. But I have a sincere burden and prayers for that special young lady and her mother I stumbled onto an elevator ride with. I can’t get her out of my mind. I am feeling what she is feeling, and I don’t like it. It is with deep regret that in spite of looking for her the next day, I did not see her face to face. I see her now, though. I see her plain as day.
Whenever you walk in to BATCC, upright, unescorted, and unsupported by mechanical devices, under your own motive power, without oxygen tanks and generators, without tubes and drains, and without masks, you are doing remarkably well. It is a powerful reminder of your good fortune that makes you feel guilty about the misfortunes of others.
“Why me?” I wonder to myself, sometimes out loud, not as a whine about having gotten the cancer, but why I am still alive and doing rather well..
“Well, why not you?” the entire universe shouts back at me, adding, “What makes you think they are not you?”
You see, the universe has all the time in the world. It is I who do not. It is I who has to take time’s measure. If you have an infinite supply, how would you measure it?
I’ve gotten used to that idea. I can live with it.
Better than that, I can laugh at myself, at my own foolishness, at my own sense of self-importance and self-indulgence.
“Why not me?” I ponder.
It is not surprising to me that I do not have the answer. It relieves me of a lot of responsibility.
©2017 Mississippi Chris Sharp