OK, I’ll sheepishly admit that if it’s late at night and I can’t sleep, I’ll watch the Maury Povich show. It’s more mindless than medication and a lot less expensive.
Maury, for those of you who have your TVs tuned to PBS, is a long-running talk show host whose specialty is DNA paternity testing. If there’s a woman in America who slept with 20 men two years ago and can’t figure out who the father of adorable little Brandy is, Maury will keep testing until he finds out.
Like a Kabuki dance or the Oberammagau Passion Play, each Maury set piece opens with a predictable series of events, usually involving a couple and a lot of bleeped cursing and protestations of the "I'm not your baby daddy!" variety. There are two possible outcomes after Maury rips open the envelope with the test results: One is to shout “You ARE the father!,” which usually leads to a crestfallen look on the part of a young gentleman. Alternately, Maury will shout “You are NOT the father!,” which will cause the man to do a little victory jig while the woman runs screaming from the stage.
Well, no special testing required, I am here to report that I am a lymph node baby daddy. When I began 4 mg of Decadron (dexamethasone) to control my autoimmune hemolytic anemia on Christmas Eve, I weighed 212 pounds. A week later I was down to 202. My neck was noticeably thinner, as was my abdomen. I had lost my pregnant look.
I discussed this with my doctor, Dr. Belle, during an office visit on New Year’s Eve. (In case you hadn't noticed by now, I like to mark every holiday with a special medical event.) It is common for people to gain weight on steroids but not to lose it, she said. Not unless there is a whole lot of CLL and attendant nonsense sloshing around in the body’s 600 or so lymph nodes.
This happened to me before, in March 2007, when I first took steroids to combat the initial attack of hemolysis that led to my diagnosis with AIHA. I was on 72 mg of methylprednisolone daily then (4 mg of Decadron is worth about 21 mg of MP) and lost 20 pounds in nine days. I was bulkier to start with and was doing Rituxan at the same time, which had a synergistic effect when it comes to cell kill. In both cases I spent the first couple of nights peeing like a horse.
* * *
My weight loss has slowed for now, and despite the joy of effortlessly losing ten pounds, the whole episode raises some serious questions that have bearing on my next steps in terms of therapy.
One, for example, is just how pregnant with lymph nodes am I? Do I have twins in there? Triplets? (The average baby weighs six to nine pounds at birth, so I appear to be working on a second one.)
All the usual blood-based monitoring tools -- lymphocyte count, hemoglobin, platelets, annual FISH -- give us a glimpse into the state of our disease. But especially in “bulky”or “SLL-ey” or 11q CLL patients like me -- 11q clones love to collect in the nodes -- these tests can show us just the tip of the iceberg.
It wasn’t the visible part of the berg that sunk the Titanic, after all, and more than one CLLer has awakened from testing complacency to find something going haywire fast. Count me as one of them. I was Coombs negative in November, resting on my RBC laurels. By Christmas Eve I was hemolyzing again. The Coombs was positive, reticulocytes were high, and haptoglobin was low. It was the pattern of 2007 repeating itself again.
During the year’s remission that I enjoyed following the completion of R-C(V)P therapy in December 2007, the disease began to return, creating the conditions of immune dysfunction that led to hemolysis again. I could see the lymph nodes in my neck slowly coming back. This was no surprise -- It’s a chronic disease, after all. That I was lulled into thinking all was well by looking at the numbers -- well, that was shortsighted.
* * *
A word is in order here about lulling. I had a very rough year in 2007. So when 2008 dawned with a pencil-thin neck, low lymphocyte count, and red cells on the rebound, I was prepared to embrace the good news. Despite my trip to the NCI to discuss a stem cell transplant trial, I made an effort to spend as much time as possible not thinking and worrying about CLL. Managing this disease can lead to burnout, a depletion almost as profound in its own way as the effects of leukemia upon the immune system. It did not help that I lost more friends to the disease, including a particularly good one.
And so I took a break, which gave my ever-loving and giving caregiver a break, too. I didn't look for clues. I figured no news was good news, and when I got good news on a test -- and I kept getting such news into November -- I embraced it. I knew the nodes were growing and I knew I would have to be back in this fight again, so I cherished and jealously guarded the time that I was, as they say in the military, on leave.
In the end, worrying all year probably would have made little difference to the outcome. I don't blame myself for taking some down time, especially as I was ready by dint of past experience and knowledge to jump into action immediately when needed, and especially as I have a doctor who is really and truly there when I need her. Those factors made me a little more comfortable turning my back on CLL for awhile, which means that I didn't flirt -- too much -- with danger.
* * *
In CLL, many things go on where the sun don’t shine, and this especially includes the peritoneal (abdomonal) cavity. God knows how many more lymph node babies I have in there, gumming up the works and growing into masses that not even Decadron can flush away. It can be notoriously hard to feel these nodes, or any large abdominal mass. Marilyn once had an ovarian cyst the size of a cantaloupe that she couldn’t feel until it twisted on its axis and started to abscess.
Doctors will tell you that, especially as time goes on, CLL can change in the way it behaves and the locations it chooses to hide out in. When docs refer to "the natural history of CLL," they mean the course it takes, which can in many ways be affected by the treatment we throw at it as well as the clonal evolution of the disease itself. (Clonal evolution is what happens as more bad copies of CLL cells are made, giving rise to new groups of clones with particular properties. For example, I began my CLL career without 11q and later developed it.)
One's CLL may go from being more leukemic -- present in the blood -- to congregating where it is safest from therapy, namely the deep abdominal nodes. There is a reason this is the location where Richter’s Transformation to lymphoma occurs. Not that I’m worried about that at the moment, but a constellation of such nodes provides the breeding ground for that sort of thing if sufficient T cell suppression occurs. At the very least, hidden interior bulk may be keeping my immune system so dysfunctional that the AIHA can never be put somewhat confidently to rest.
* * *
So, what to do? Well, aside from Philippine psychic surgery, there’s the CT scan, and that is what Dr. Belle and Marilyn and I have agreed to do to help us develop a treatment plan. The last time I had a thorough one was at diagnosis in 2003. I'm going in tomorrow for another.
I know there are those with reservations about radiation, but the prudent use of X-Ray technology can answer some important questions. Here I am, with 11q CLL, prone to bulkiness, having lost enormous lymph node weight, having relapsed rather suddenly. "Don't worry about what you can't see" is a rather hollow option, even though this is often repeated to us patients, sometimes by doctors. Power, such as we have it over this disease, comes from knowledge. The Titanic had a watchman who saw the iceberg in advance, just not soon enough to avoid the damage. (For a reasoned opinion on the use of CT scans and other imaging in CLL, read Dr. Terry Hamblin's blog post entitled CT Scans.)
To develop a strategy without knowing as much as I can about the enemy is wasted effort. To do treatment and say that it’s a success because all the peripheral blood numbers look fine is also folly.
We need to see as complete a picture as possible going in. Once we have that picture, we’ll have a better idea of know how to proceed. In the meantime, the steroids are holding me, just barely. My hemoglobin has been stable, at 12.1, for the last week. I feel pretty good, despite the Decadron, which gives me a wired effect that tends to make me want to dance like one of those relieved guests on Maury.
I've go to admit it's getting better: More good news about my CLL (chronic lymphocytic leukemia) - Most of my blog posts can be followed here. The full story of my CAR-T therapy, the incredible rollercoaster and the amazing results can be found there I...
3 days ago