Saturday, December 03, 2005

An Orwellian illness

I have a new name for CLL: Orwell's Disease.

In George Orwell’s 1984, nothing was as it seemed to be. “Newspeak” was the language used by the authorities to control society, and good citizens were supposed to accept contradictions as if they made perfect sense.

Fortunately, society never reached the point that Orwell describes -- or hasn't yet -- but misleading language is everywhere about us. People with CLL hear terms like “good cancer” and “complete response” and “normal karyotype,” which seem to imply things that are, in fact, not the case. We are even told that CLL is "incurable," when in fact it is curable. In CLL, black is white and white is black with frightening regularity. Orwell could not have invented a more brilliantly contradictory disease if he had tried.

What makes this a serious problem is that these terms are often taken at face value, and they become part of the process by which we make treatment decisions. So getting clear on what they mean -– and don’t mean -– can be a matter of life and death.

"Good cancer"

“Good cancer” is my nominee for the Mother of All Oxymorons. Enduring this phrase is a rite of passage for most people with CLL. It is harmful on two levels: First, it can lead people who don’t have CLL to think that those of us who do have little to worry about. Second, it can convince us of that, too.

It is all too tempting, and too easy, to wander blithely along, reassuring oneself that CLL is no big deal. There are no doubt many of our brethren out there right now doing so, and some of them are in for a very rude surprise.

Only about one third of people with CLL die of causes unrelated to CLL, according to a current American Society of Hematology report by Guillaume Dighiero entitled CLL Biology and Prognosis. (Things are no doubt worse for younger patients like myself, who face the challenge of contending with the disease for several decades.) CLL takes many forms, and for every patient with “indolent” disease there is another suffering through the vagaries of therapy, the disappointment of relapse, and complications that can include uncontrollable skin cancer, anemia, shingles, and Richter’s Transformation. And did I mention death by simple infection?

Yes, on the sliding scale of cancers there are worse things to have. I’m grateful I wasn’t hit by a train, thank you. But being hit by a bus still hurts. CLL is a bitch and there’s nothing “good” about it. Even those with more cooperative cases have compromised immunity and face the stress of not knowing if and when the disease will worsen. The Dighiero report states: “Even in this very good prognosis group (Binet Stage A, mutated IgVH), progression is observed for one-third of these patients and CLL-related death for 10% of them.”

That’s for those with the very best prognostics for our “good” cancer.

"Complete response"

"Complete response" is another mind-bender. “Complete,” according to, has four definitions, of which two apply here: “Absolute; total.” And “having come to an end; concluded.”

In the ancient days of CLL, say 1996, response to treatment was determined by blood counts and physical examination, bone marrow biopsy and aspirate. Indeed the NCI Working Group Guidelines, written in 1996 and still in use today, say you have a "complete response" if your BMB still shows 30% lymphocytes. There are other criteria to meet as well: If you don't have palpable (felt by hand, as opposed to examined by CT scan) swelling of the lymph nodes, spleen, and liver; if you have no "B" symptoms such as night sweats; and if you meet certain minimal requirements for levels of hemoglobin, neutrophils and platelets -- and you maintain all these things for two entire months -- that is said to be a "complete response."

As you can see, the bar is not set terribly high, and why the doctors who wrote the guidelines chose the term “complete,” I don’t know, since all patients undergoing chemotherapy relapse, which is a clue that the response isn’t really complete to begin with. (As Homer Simpson would say, "D’oh!")

At any rate, we now have more sophisticated testing, such as polymerase chain reaction (PCR). PCR has improved our detection ability by a hundred fold, so we can now see CLL cells that we couldn’t detect before. But the language hasn't changed, so people with CLL cells as determined by PCR are still having “complete” responses.

They can call it "complete," but it's not. It's less incomplete than "partial."

And, of course, as is typical with CLL, all PCR tests are not equal; one method may provide the joyous news of being PCR negative while another determines that there are CLL cells lurking (all of this with interesting consequences for how the end results of clinical trials are determined: Did treatment X really work that well, or would a different PCR test show it to be less effective than reported?).

And, of course, future advances may change everything. PCR could be further refined or even outdated, replaced by something else. No doubt incomplete responses will still be "complete," though.

"Normal" FISH test results

"Normal" karyotype has entered the CLL vernacular thanks to the FISH test. If you don't test positive for the loss of a bit of important genetic material
(11q, 17p, Trisomy 12, 13q) on the common FISH test then you are considered to have a "normal" karyotype (genetic profile). But that doesn't mean your karyotype is normal. If it was, you wouldn't have CLL. It means they didn't test for your particular deletion and that you have one of unknown and uncharted consequence.

I have a “normal” karyotype and was lulled into thinking, for some time, that I had “passed” the FISH test. It turns out that the test didn’t test for what I’ve got; maybe it’s worse than some of the common deletions, and maybe it’s better. It will just have to be a big, fat surprise.


By now, having read this far, I can hear you thinking: Dave, you’re bumming me out! So let’s look at our last example of Orwellian doublethink, where for once the truth brings with it some hope.

The word is "incurable," and we CLL dudes and dudettes have this drilled into us so much that we take
"incurable" as a matter of faith. But there are, in fact, people who have been cured by stem cell transplants. The most famous case is that of Tom McCune, who had a transplant in 1990 and has been CLL-free ever since. "Cure" is defined by the cancer gurus as ten years without a recurrence and McCune says there are other patients approaching that mark. The fact is that most CLL patients who have had transplants have had them within the last ten years, and therefore not enough time has elapsed for the cure mark to be reached. And the most recent transplantees have benefited from advances in the procedure that will probably show a higher cure rate than McCune's generation of transplant pioneers.

A study recently reported in CLL Topics shows 75% of CLL patients who have received transplants from matched unrelated donors are alive two years after the procedure. This includes a lot of people who have been through the chemo wars, and who have “failed” their FISH and other prognostic tests. (And, as Chaya writes, “the two year survival rate is pretty close to an almost-cure, the large majority of patients who make it up to that point go on to live normal lives.” )

Another study shows that people who are in better health non-CLL wise, with no “comorbidity factors,” do even better when it comes to transplants. So for us young people who stay in shape and avoid the temptation to drown our sorrows in ice cream, vodka, cigarettes, and sloth, chances are not half bad of surviving a transplant. And therefore, there is at least the possibility of being -- dare I say it -- cured?

Of course, transplants are very tricky and very hard on the body, not a choice to be taken lightly. You still need the right donor match, and nothing is a sure thing, and the cure still might kill you. But the odds are getting better, and will only continue to do so as transplants are further improved (which will only make them easier for older patients, as well).

So, to sum up, CLL is a bad cancer that chemotherapy cannot remit completely, the victims of which have abnormal genetics. And some of them can be cured.

Now, about that drink . . .


For a new report on transplants by the leader in the field, Dr. John Gribben, follow the link below. Gribben cautions that "the follow-up of most clinical trials is too short to assess whether the use of SCT can cure CLL," but the report has some encouraging statistics mixed with some cautionary ones. The light at the end of the tunnel may be the light at the end of the tunnel, or it may be an oncoming train, depending on your individual response to a transplant:

Read more on how transplants could save your life, including ways to increase the odds in your favor:

Who is Tom McCune and why is he smiling?:

CLL Biology and Prognosis by Guillaume Dighiero:

If you want to poke around a website that discusses language as used by Orwell –- and today’s continuing abuses –- visit the Newspeak Dictionary at:


Brian Levy said...

How about "Excessive White Dude"

Brian Levy said...

Sorry meant to post this on "what do we call ourselves"

David Arenson said...

I don't know, Brian. When I think of "Excessive White Dude" I think of the early days of the Jerry Springer show, when he used to interview 800-pound guys who couldn't get out of bed. They'd have to remove a wall to get the guy to the hospital. Maybe that's "Excess of White Dude" that I'm thinking of. . . .

Melissa O'Mara said...

Hi David,

I'm the wife of CLL'er Dan O'Mara, who is also coming up on his 2 year anniversary of his SCT (fully ablative, unrelated donor) at DFCI/B&W in Boston. Dan was 40 at diagnosis in January 2003, and apparently didn't have "the good CLL", much less "the good cancer". Not sure if you've followed his case, I was pretty active on the CLL list in 2003, and we started a Caringbridge
Caringbridge site for him in October 2003, which we still maintain.

Love your blog, and so does Dan. He found it today, read it, and sent me the link. Your reasons for blogging totally resonate with me. I find writing/reflecting to be very cathartic. AND, when we were in the belly of the whale, it was also a necessity, as far as I'm concerned. We love the free Caringbridge service, and particularly the guestbook. When we started in 2003, I didn't know what blogging was, then I realized that we were doing it!

So, keep sharing, reflecting, creating dialogue, and of course, dance on with CLL. And take the lead, whenever you can, in that dance! We'll be dancing too, and hopefully sharing in the dialogue.

Living fully,

Melissa O'Mara

David Arenson said...

Thank you, Melissa. I had read your site in the past; CLL is sometimes thought of as an "old people's disease," but Dan's case reminds us all that it can afflict the young, in the prime of life. Dan is lucky to have such a supportive wife and family; I'm sure it's part of the reason for his success. Now that he's almost two years post-transplant, things are looking better and better for the long term. May he be surrounded by grandchildren one day, his CLL but a distant memory!