My “new normal" began when I was diagnosed with chronic lymphocytic leukemia in the fall of 2003. This new normal has since evolved, or devolved, depending upon how you look at it. Each time I have settled into a comfort zone, things have gotten worse. There’s a reason why my sig line at CLL Forum is a quote from Gilda Radner’s Saturday Night Live character Roseanne Rosannadanna: “It’s always something. If it’s not one thing, it’s another.”
Since my diagnosis, unwelcome new wrinkles have demanded new responses, as well as renewed patience. Required, also, have been constant efforts to find psychic and emotional shelter from a disease that blithely marches on. As I have written here, hope is an active process -- but it is not always an easy one. This is the most challenging year yet, and to understand it, it helps to look back. I have had the disease long enough now to have some perspective on where it has been, and where it may go.
As bad as 2003 was -- I was diagnosed at Stage 2 with swollen lymph nodes and spleen, which threw me directly from the frying pan into the fire -- a few good things came out of it, or seemed to. I learned that I had the inner strength to deal with devastating news and the resourcefulness to learn something about a complicated medical issue. I showed a modicum of wisdom by avoiding the treatment pushed mercilessly by my first oncologist, the fludarabine-happy Dr. Lippencot. I began Googling to save my life, literally, and I discovered that there is a patient community out there full of experienced and knowledgeable people. Largely through their efforts, I found another treatment option, single-agent Rituxan, that would buy me time without burning any big bridges.
In 2003, I also had one good test result -- my CD 38 came back at 12%, or well within the negative range. This was the only prognostic test available to me at that time, and it boded well. And then there was a gray memory that came back into full color: Prior to my diagnosis, my last CBC had been in 1996. I recalled that my white count had been a little high, and that my doctor had told me that it was probably just an infection but that I might want to get my blood retested in a month. His infection comment had struck me as odd, since I didn’t feel as if I had one. But I dismissed his advice, feeling rather immortal. And, of course, I didn’t bother to get a copy of the test report. It no longer exists, since the law requires that these things be kept for only so long, and I stopped seeing this doctor long ago. But what this memory told me was that my disease had not appeared overnight, and that I had likely had it for a long time, during which it had behaved itself for the most part, perhaps slowly building up, as might a more indolent form.
2004 was, in retrospect, a breeze. I began the year with my Rituxan treatment. The progress with which my CLL cells died was so rapid that it created a buzz among the nurses in my oncologist’s office. I left treatment with a normal lymphocyte count, a spleen worthy of an anorexic, and very few visible lymph nodes. I turned my attention to telling my extended family about the disease, and I became involved in reaching out to newbies in the online patient community. Marilyn, Pyewacket, and I even enjoyed a long vacation through the Canadian Maritime provinces.
Upon our return, I underwent a couple of fancy new prognostic tests just made available to my oncologist through Impath Labs in Los Angeles. The first, a FISH test, came out “normal.” This was the second-best FISH result, and it served to confirm my belief that perhaps I had a fairly cooperative case of CLL. After all, my CD 38 was negative, I had evidently had the disease since at least 1996, and while it had slowly built up to Stage 2, it had never sent my red counts or platelets below normal, and I had never had any “B” symptoms. All that, and a mild treatment had appeared to work quite nicely. My lymphocyte count was slowly making its way back up, of course, but that was to be expected with single-agent Rituxan. If my disease could be looked at as a ship -- the SS CLL -- I was standing on the bow and feeling, if not exactly like king of the world, at least that there were no icebergs in sight.
Then the second test result came in: I was ZAP-70 positive. This gave me pause, knowing it was often a surrogate for mutational status. I liked to think then that I had one foot in Chaya’s Bucket A -- the CD 38 and FISH -- and one foot in bucket B -- the ZAP-70 and the fact that the disease had managed to progress to Stage 2. Bucket C was over there somewhere, with flies buzzing around it. And so far, I was avoiding it. At the end of 2004, I joined the board of directors of CLL Topics; there was no reason not to toast the new year with some optimism over a holiday brunch with friends amid the red rocks of Sedona.
2005 sucked, pretty much. If 2004 was punctuated by moments of ease, the year that followed seemed to ratchet up the stress at every opportunity. The new normal shifted from bizarre but comfortable to disquieting. Life with CLL became an unending process of adapting to bad stuff.
The year began with the surgical removal of a squamous cell skin cancer from the top of my head. (“It’s just a pimple,” I had been telling Marilyn. “No, it’s not!” she had insisted. Guys, women know pimples. It’s genetic. Just give in and don’t bother arguing.) I had been reading up on the heightened risk of skin cancer in CLL patients and knew that I was at even higher risk; my skin tone is akin to that of the Pillsbury Doughboy and I have always burned in the sun. In 1999, I had had my first squamous cell growth, a pinkish thing that reminded me of a toadstool, frozen off the side of my face. Now I had a second one, which had implications beyond those of vanity: My CLL-compromised immunity was not stopping the damned things. And treatments that severely impair T cell function -- that’s the thing that keeps those squamous cancers in check -- might be a problem for me. Those treatments include fludarabine and Campath, two of the biggest guns we have.
On top of this, Typhoid Charlene came to visit and gave us colds. I got mine last, and I got over it first -- all of which told me that my immune system was still pretty functional in some respects -- but my lymph nodes ballooned in response. They went back down after a week or two, but provided a visible reminder that my immunity was laboring under a new burden. My immune system was no longer an aerodynamic Prius or a sleek Mustang convertible -- it was, perhaps, starting to look like a clunky and dented 1972 Oldsmobile Cutlass, still making it from point A to point B, but not without some grinding of gears and black smoke coming out the tailpipe.
I received Rituxan again in April and May of 2005; the response, while adequate, wasn’t quite as good as before, and it did not last as long. This implied, of course, that the soft-glove approach might not be effective forever. This wasn't, of course, what I wanted to hear.
And for the first time, prior to starting treatment, I had felt a little pressure in my lower left pelvic region, where lymph nodes had begun to grow. The treatment did not ameliorate it. I was left with an occasional reminder, if I lay flat on my back, that I was sick with something. Slowly, this pressure got worse, until I noticed it when laying on my right side. It is there now, noticeable also when laying on my left side, or when sitting.
Adapting all these things into the new “new normal” took some doing but paled in comparison to the news I would soon get from Quest Diagnostics: my CLL is IgVH unmutated. I still remember the call from my oncologist’s nurse, and I can still see the scrap of paper on which I wrote the single word: “unmutated.” I can also recall the sick feeling I had in the pit of my stomach. “Unmutated” brings with it some “un” implications: “Un-controllable” “Un-stoppable” “Un-likely to have an easy time of it.”
Slowly, as test results had continued to emerge and treatment had became less effective -- renewed Rituxan ending in December fared no better than had April’s doses -- everything I had initially assumed about my CLL started to turn on its head. A newer and meaner new normal was in the offing.
I toasted the arrival of 2006 with trepidation. I told my friends that maybe it would be nice if we could just have a little time without stress, without anything eventful, without something else bad happening. I suppose it was the champagne talking.
This is getting a little long, and so there will be a “The new normal, Part 3," which will bring you up to date on my treatment quest and what the new normal is for me today.
Monoclonal B-Cell Lymphocytosis: A precursor to CLL (chronic lymphocytic leukemia) - This week I'm posting on the CLL Society website an interview that took place at ASH 2016 with Dr. Neil Kay from the Mayo Clinic in Rochester, MN where we...
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