I am sleepless in Sedona, up in the middle of the night, thinking. It is the writer’s curse to be filled with ideas at inconvenient times.
I have written a lot about chemotherapy in this blog without actually having had any (Rituxan being immunotherapy, and a lot easier on the system). Now I am having it, and am coping with both the joy of success –- watching my red counts recover rapidly and the color, such as it was, return to my cheeks –- and some of the side effects: I am highly sensitive to vincristine as I have some numbness in my fingers after just one dose, and it was a half-dose. The cyclophosphamide is challenging me with hyperglycemia –- which, on top of the effects of the steroids, means I am on a Draconian no-glucose diet, at least temporarily.
But I don’t care. The big picture is that my back was to the wall and I did what I had to do and I am glad it is working. That big picture is the thing we CLL patients constantly try to grasp, yet there are so many mirages in CLL Bizarro World that we are often left clutching a fistful of illusions.
So I want to say some things about chemotherapy and also about CLL, provide some perspective I have been gaining.
The CLL lullaby
The first brain muddle we patients get into is that we all have the same disease. There was a time a couple of decades ago, when CLL was poorly understood, when that appeared even to the experts to be the case. And when it also appeared that survival and response to treatment had more to do with luck than anything else. Now, while luck plays a role in everything in life, we know today that CLL is not the same for all of us.
A reader of this blog wrote me and said, “I know someone who has had CLL for 30 years.” When I first was diagnosed I began trolling the internet for stories of people who had had CLL for a long time, concrete examples to hold on to, and I began to write them down in a page in a journal. And then as time went on, I came to know some of these people myself. More power to them. They are alive after all this time because they have very indolent CLL, possibly this asymptomatic lymphocytosis that Dr. Terry Hamblin writes about, possibly something a little worse than that but still so low-level as to be kept in check by their own immune systems or to respond to therapy for a long, long time. These are the people with IgVH mutated status who also have things like a deletion on one arm of the 13q gene, and who are CD38 and ZAP-70 negative –- Chaya Venkat’s Bucket A and A+. For the most part they do not have great secrets to tell us about how to overcome CLL. They are not geniuses; they are fortunate. They overcome CLL because for them it is a low-level, indolent disease -– the very picture of the “good cancer” –- and it is not really the same disease that the rest of us have.
This is why prognostic tests are important. They do not provide a complete picture, but they do give us the outlines. They do not always follow true to form –- you can have good markers and bad disease, and more rarely bad markers and not-so-bad-disease –- but they generally do. There are no doubt prognostic factors that have yet to be ironed out, discovered, entered into the CLL vernacular that will one day show us more. But today’s tests -– IgVH mutational status, FISH, CD38, ZAP-70 as done at a reliable research institution as opposed to a commercial lab –- will tell you a lot about your CLL.
Not getting them done is, to my mind, just plain stupid. When you’re first diagnosed and you’re feeling scared and vulnerable for a while, I can see holding off. But after you’ve gotten your CLL sea legs and realized that you’re not going to keel over tomorrow, it’s time to grow up and get real. Knowing your prognostic score, as it were, gives you more of a fighting chance when it comes to choosing the right risk-adapted therapy and in planning a long-term strategy.
That is, of course, if you acknowledge and act upon the results and don’t go putting your head in the sand when you hear them. But putting our heads in the sand is one thing we do because we are human, and also because we are encouraged to do it from the very beginning. We are told we have the “good cancer,” which leads to complacency. Even if we are not aware of it, it gives us subconscious permission to ignore CLL, to downgrade it, to pretend it won’t hurt us. (The fact that most of us also have a period of years before the disease begins to significantly impact our lives reinforces this misconception.) We are told “you will probably die with it, not because of it.” Well, that is true if you have an indolent form. Or if you are diagnosed at, say, 70, when your natural life span is starting to skate on statistically thinner ice anyway.
But that is not true for us younger people, and I know people diagnosed with this disease who are in their 20s. I know a young mother who is in hospice. I know that for those of us who are younger than 60, unless we are blessed with Bucket A CLL, most of us will die because of the disease and not with it, and we will die earlier than we would otherwise. Except for those who are saved by a transplant, which is the only cure we have for now. So therein lies hope, and it is where mine resides.
Transplants are tricky. They might cure you, they might kill you. Chaya has written with cautious optimism about them. Terry reminds us that life after a transplant can be hard to endure. Like so much having to do with CLL, the results are individual. Some people do well with minimal fuss, others struggle with nasty issues of graft versus host disease. My view is that the risk is better than the alternative.
Which brings us back to chemo, sort of, except that I have another tangent first:
CLL is a confusing world in which patients still have trouble getting it into their heads that one size does not fit all, and that we do not all leave the starting gate at diagnosis in the same shape (and that clonal evolution can also change the order of things). Unfortunately, this thinking is reinforced by legions of local hem/oncs who have the same idea. CLLers form a small percentage of their practices and they deal with much more dramatic cancers, where life and death is indeed compressed into months or a few years. In that perspective, CLL is comparatively a good cancer. But as John Byrd has been heard to say, the only good cancer is the one you don’t have.
The bigger point here is that in my unscientific estimation from reading countless patient accounts, roughly half the hem/oncs out there are incompetent to treat CLL and its complications to an adequate standard. They are not up on the current thinking on prognostics, they are not up on the latest treatments, they don’t even know to put patients undergoing treatment on prophylactic meds, they’re fairly clueless about the laundry list of complications such as AIHA and ITP, they have no sense of the subtleties of when to end “watch and wait” and when to start treatment, they are so undereducated and inexperienced as to have little ability when it comes to the art of medicine as it relates to CLL. My history of having to fire doctors is not uncommon.
All this speaks to why you should see a CLL expert. I’ve seen a few. They may tell you different things about what to do if you need treatment, but there will be a sound reason behind each of their approaches. One must remember that even our expert doctors can only make the best possible guess as to what is right, and that the art of medicine does not guarantee a particular outcome. ("We like to pretend that medicine is an exact science, and that is hogwash,” Dr. Michael Keating told a friend of mine.) This is true of doctoring in general, and I would be remiss if I did not say that there are many good, dedicated local hem/oncs like my Dr. Belle that do all they can do to provide excellent care in our oft-befuddling landscape.
So, seeing an expert is essential. As is finding a local doctor who does their best to stay current on CLL and who is willing to work with you to understand it even better. The quality of your doctor(s) is a prognostic factor that influences your overall survival just like IgVH and the other medical tests.
Now, back to chemo. Like many patients, I started out after diagnosis being afraid of the word. “Chemo” is a loaded term in our society. It’s what desperate, really sick people do. It means a bald head that makes you stand out in a crowd. It means being attached to bags of fluids in a room filled with people who are fighting for their lives. It means relatives who suddenly look scared, friends who whisper. It means chemicals with a laundry list of potential side effects getting into your body. It is all these monsters crawling out from under the psychic bed, shouting at you in the face: This is serious! You could die!
The subconscious idea that we CLLers have a “good,” indolent cancer predisposes us to not want to believe that such a serious thing as chemo is necessary or even right for us. After all, many CLLers do go on and on and on without it. But we are not all equal out of that starting gate, remember?
I have long had trepidations about the side effects of chemotherapy, not so much the annoying, mostly controllable stuff such as bald-headedness, peripheral neuropathy, and high glucose. More the uncontrollable risk of developing disease resistance to further therapy, a mucked up p53 pathway, an immune system so suppressed that Richter’s gets going, the big picture kind of stuff. This is why I have sided with the school of thought that believes in graduated treatment, soft-glove first. Arguments of merit can be made for the opposite view, the “nail it” approach. I do not fault those who go that way. I am happy when I hear a patient say, “I had FCR and my report now shows no detectable CLL anywhere in my body.”
I have also had trepidations because I have seen chemo be, for lack of a better term, abused. Abused by those local hem/oncs who really should not be treating CLLers, the kind who treat too soon and who treat with, say, single-agent fludarabine –- years after CLL experts agree that it is no longer the right approach.
I have expressed all these things in this blog, but I have not expressed enough of the following, so it stands here to be said:
Chemotherapy is an invaluable tool that should be used when it is needed. It should not be avoided at all possible cost. It should be used at the right time, which is tricky in CLL, and which is why you need to see a CLL expert and/or have a good local doctor who understands the current guidelines for treatment and who has some grasp of the art of medicine. And which is why you yourself should educate yourself as much as you can.
Timing is everything; my AIHA made the decision easy for me. I am glad the chemo was there to deal with the problem. When macrophages are eating your red cells with the ferocity usually found only at Nathan’s annual hot dog-eating contest, chemo starts looking mighty user-friendly.
When I had my first round of R+CVP a couple of weeks ago and the cyclophosphamide went in and the vincristine was injected, it was simple, it was painless, it all came in clear liquid just like the Rituxan, just like the saline. That is how it looked physically. What was being injected, psychically, was power, was purpose, was hope. Drugs ceased being a compendium of potential side effects –- the trees -– and started looking more like something that would prolong my existence –- the forest.
As Chaya points out always, there is no free lunch. Perhaps I have set in motion some things that I will not be happy about later. But in the risk-reward scale, there is no doubt in my mind that the rewards were worth the risks. Sometimes we must fight fire with fire and either may burn us.
Finding out when the rewards are worth the risk to you is one of the keys to success in battling CLL. Taking action in that window of time is the right thing to do. I once wrote about opportunity costs, a concept that I got from talking to Chaya and her husband PC, whose familiarity with so many patient case histories has given them a good perspective on the lay of the land.
Not taking action, refusing to believe things are as bad as they are, hanging on to some subconscious idea that CLL is still a “good,” indolent disease that Joe Blow has been living with for 30 years without chemo so why can’t I, that is foolishness. Lullaby and goodnight.
The best rule of thumb I can come up with is this: If you have aggressive disease, treat it aggressively. If you have progressing disease that is getting you into trouble, treat it as intelligently as you can, but treat it.
And do not go it alone. Go it with a good local doctor, go it by seeing an expert, go it by reading CLL Topics religiously, go it by reading Dr. Hamblin’s blog and keeping abreast of his answers on the ACOR CLL List, go it by hashing out ideas with other patients in places like CLL Forum.
Sometimes I think I have spent too much time online, reading all those things, connecting with patients, learning from experts. Then, this year, when push came to shove, I found that I knew some things that had a profound and important impact on the quality of my medical care and therefore on my life. So stay online and learn, humbled though you will always be by the vastness of what you still don’t know.
Patient, you must be ever vigilant. You will never know all the answers but you must learn to ask the right questions at the right time.
Knowledge is power, wisdom is the proper application of that power. That is what to strive for.
Now, I need to go back to sleep.
CLL Society, LLS and the Cleveland Clinic Present a Free Patient and Caregiver Educational Forum on Nov. 11 on Chronic Lymphocytic Leukemia - The CLL Society, LLS and the Cleveland Clinic are hosting a Patient and Caregiver Forum on Nov. 11. It is going to be a super educational experience with C...
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