Living with CLL

Yesterday, after having had my upteenth follow up at MD Anderson, I was talking with my son about our respective days and what the doctors shared with me. During our conversation, he asked me, sort of timidly, “I understand that your ‘numbers’ have been going the wrong direction, but what do you actually DIE from when you have CLL?” That was a very good question…that I’m sure many folks who don’t have cancer might wonder. 

Cancer starts when cells start to grow out of control. Cells in nearly any part of the body can become cancer and can spread to other parts of the body. Chronic lymphocytic leukemia (CLL) is the most common leukemia in adults. It's a type of cancer that starts in cells that become certain white blood cells (called lymphocytes) in the bone marrow. The cancer (leukemia) cells start in the bone marrow but then go into the blood. In CLL, the leukemia cells often build up slowly. Many people don't have any symptoms for at least a few years. But over time, the cells grow and spread to other parts of the body, including the lymph nodes, liver, and spleen. Leukemia is cancer that starts in the blood-forming cells of the bone marrow. When one of these cells changes and becomes a leukemia cell, it no longer matures the way it should and grows out of control. Often, it divides to make new cells faster than normal. Leukemia cells also don't die when they should. This allows them to build up in the bone marrow, crowding out normal cells. At some point, leukemia cells leave the bone marrow and spill into the bloodstream. This increases the number of white blood cells in the blood. Once in the blood, leukemia cells can spread to other organs, where they can prevent other cells in the body from functioning normally. This is just a scratch on the CLL surface as there are so many additional variables such as negative markers…which I have…but I won’t get into that. Suffice it to say, it makes it harder to treat.

So…the answer to my son’s question is this. An infection (usually pneumonia) is the most likely cause of death of a CLL patient. CLL impairs our immune system, so that we are not as able to resist infection as someone with a normal immune system. Either that or a shutting down of the vital organs due to the limited “good” blood flow…or lastly, negative reactions to treatments…like Richter’s Syndrome when CLL transforms into a more aggressive form of large cell lymphoma. (Though I have a dear friend where it happened the opposite way…lymphoma to leukemia.)

So what does all of this have to do with LIVING with CLL?! I guess the first thing that I wanted you to know is that unless the Lord puts His Hand on my head and says “Be Healed,” there is no cure for CLL. And we’re ok with this. We weren’t at first, obviously…but we are now. We’re all going to die of something. I just have a higher percentage of knowing what mine’s going to be from…unless I get hit by a bus or we fall off a cliff when hiking! The Lord has appointed our days. That’s why I don’t worry. He’s got this. I just need to be faithful in pursuing my best options of staying around as long as possible. 

Which brings us to yesterday. Dr. W reviewed my treatment history…Campath to reduce leukemia prior to transplant, stem cell transplant, two donor lymphoctye infustions, and two CAR T-Cell trials. Only the transplant got me into remission for a couple years. Everything else were attempts to get me back there. They all failed…BUT they bought me TIME! They worked just enough to slow things down a bit. And give my body a chance to recuperate before ramping up for the next treatment. Dr. W has given us three paths to review and research. We actually have already started the research the last time we talked about this, but yesterday’s info was more intense. So as to not completely lose the majority of you, I will not go into too much detail about each of these protocols. Just enough that it makes sense for those who have asked…. 

Aberrant signaling of the B-cell receptor pathway has been linked to the development and maintenance of B-cell malignancies. Targeted inhibition of Bruton's tyrosine kinase, or BTK, has improved clinical outcome in patients with relapsed (and treatment-naïve) CLL. Acalabrutinib is one such BTK inhibitor with somewhat fewer side effects than its predecessor, Ibrutinib. 

Apoptosis is an orderly, programmed intracellular signaling cascade that leads to cell death. BCL-2 encodes an anti-apoptotic protein and was the first oncogene identified that primarily exerted its oncogenic effects by inhibiting cell death, rather than by promoting uncontrolled cellular proliferation. Venetoclax is one of a new class of cancer treatments that operates by directly promoting the cellular process of apoptosis, or programmed cell death. It is even been shown to work well with 17p deletion (which I have). 

Lastly, Obinutuzumab is a monoclonal antibody (antineoplastic cytotoxic chemo drug) that affects the actions of the body's immune system. Obinutuzumab strengthens your immune system to help your body fight against tumor cells. Combined with other drugs, it has shown to be effective in treating CLL. I say all this because ONE of the three choices included all three of these drugs…where the first two were either just BTK inhibitors or just BCL-2 inhibitors. The new trial, which just began a couple weeks ago at MDA, offers in our opinion the best of all options, plus it’s a 2-year trial with the goal of getting back to remission and buying me some more time. My name is on the list. 

So why not start now? Well, that’s a good question, too! I have several numbers that we follow…white blood count, hemoglobin, platelets, plus my absolute neutrophils and lymphocytes (part of the WBC). While most of my numbers continue to move in the wrong direction, my hemoglobin and platelets have been on the low but steady side. So because of the toxicity of treatment, they don’t get in a big hurry to rush into things until ALL my numbers are going south. Plus, they look at physical symptoms as well such as swollen lymph nodes (check), fatigue (check), night sweats, weight loss and enlarged spleen…ok on those currently. So as is so common with CLL…we wait. Another three months. And check again. Unless my physical symptoms worsen before then. 

I know this was a LOT of information, but so many of you have asked specific questions and asked how to pray. While I appreciate prayers for “healing,” outside of a massive miracle, CLL doesn’t just go away. But it can get squashed down for a while. So that’s my prayer. Always has been. That God will give us time. Time together. Time to do the things we love. Time with our family. Time with friends. Time to travel. Time to help others. Time to build our house and retire in the country. Time to enjoy this life He has given us. 

At the end of our lives, people often reflect and ask themselves…Did I live? Did I love? Did I connect? Did I matter? Did I contribute? Life has given me a second chance…a “golden ticket.” I intend to make sure I have used it well. 

What will you do to live today? To make difference? To be intentional? Will you have any unanswered questions when your day comes? Life is about choices. Choose well.