Debulking: the amazing photographic evidence

I mentioned recently the rather astonishing debulking effect that methylprednisolone -- being used to treat autoimmune hemolytic anemia -- has had on my chronic lymphocytic leukemia. Sometimes a picture is worth a thousand words, so I am posting two pictures today in lieu of writing 2,000 words.

The top photo is me on February 7, before the AIHA came calling and any treatment was done. Someone was kind enough to say that my thick neck made it look like I had been working out, but the only workout was being done by the CLL, which was ever expanding itself into lymph node masses.

The second photo was taken March 25, a little more than a week after starting treatment with 72 mg of methylprednisolone for the AIHA (and also 20 mg/m2 of low-dose Rituxan three times a week). Who is that man with the neck?

Now, imagine that sort of lymph node impaction throughout my abdomen -- I have no doubt that the bothersome pelvic nodes I have written about in the past were in a similar mass -- and you know why I slimmed down there, too, and no longer need my "maternity clothes." Altogether, I have lost 22 pounds since starting the steroids, which are now tapered to 16 mg daily. (As to the AIHA, I am doing OK but the red counts are still not normalized.)

I hope these photos show what unmutated, 11q-deleted clones of "the good cancer" can do. Often we patients get used to a slow change in our appearance and forget what we looked like before the lymph nodes began to swell. And while this shows what the CLL visibly did, another big part of the story is what it did that I couldn't see -- compromised my immune function, allowing something, probably an infection, to trigger the AIHA.

By the way, I made this note to myself on the third day of methylprednisolone therapy: "After two days of steroid, nodes reduced to about the least since initial (Rituxan) treatment three years ago. Six pounds lost." In other words, the debulking occurred substantially within the first few days of steroid therapy. Today, only two palpable, almond-sized lymph nodes remain in the fleshy area under my jawline, both vastly reduced from before.

The challenge ahead, after recovering fully from the AIHA, is to maintain my slimmer physique by good diet and exercise and to keep the CLL at bay. Seeing the massive bulk that was built up in me, and coping with the hidden consequences of the disease running amok which suddenly came to the fore as AIHA, has caused me to do a little thinking about the possibility of using something a little stronger to solidify my remission.

In the meantime, here are the photos, before and after:

A spring in my step (or the weight loss follies)

This morning’s e-mails contained a spam message titled “End the annoying obesity now.” I had to laugh, as I have just discovered my own secret to rapid weight loss, namely methylprednisolone. Maybe I should run out and patent the “CLL Miracle Diet” before some doctor -- like Tom Kipps or Januario Castro -- gets inspired by the example of Dr. Robert Atkins and decides to it.

One side effect of taking steroids can be weight loss, especially if you are a bulky CLL patient like me. My experience, dropping 20 pounds in nine days -- about 10% of my body weight -- is consistent with what has happened to some other patients I know about.

As you will recall, I began taking 72 mg of methylprednisolone (MP) daily starting March 14 for a sudden case of autoimmune hemolytic anemia (AIHA). High doses of steroids (1 mg/kg prednisone up to 2X daily) are the standard frontline treatment for AIHA -- and in my case they are working. (For those of you keeping score: My hemoglobin was up to 11.3 as of yesterday, as opposed to its nadir of 8.9 on March 14, and I am almost starting to feel normal, or as normal as I get. Hematocrit went from a low of 26.9 on March 12 to 34.8 yesterday. Haptoglobin levels, which had been as low as 10, were up into the 70s after only two days on the steroids, 34–200 being the standard range.) I expect my red counts to be normalized very soon, and one of these days I will report on my theory as to why I developed AIHA in the first place.

The AIHA reared its ugly head -- I am reminded of the old Monty Python sketch "Nobody expects the Spanish Inquisition” -- just as I was undertaking low-dose Rituxan three times a week for 12 weeks as part of a plan to deal with CLL. My hem/onc and I had planned to add some low-dose MP toward the middle of that process to squeeze CLL out of the nodes and spleen, after first getting the peripheral blood counts down. This was the plan anyway, and it was completely upended by the AIHA diagnosis. In the immortal words of my doctor, who came rushing into the infusion room with scripts in hand, “How would you like to start the steroid part early?”

This turn of events has interesting consequences for the CLL as well as the AIHA. Readers of this blog know that I have long been interested in the concept of Rituxan + HDMP (high dose methylprednisolone), the protocol that has been used successfully in chemo-naïve patients at UC San Diego (and thus the source of my reference to Drs. Kipps and Castro in the first paragraph). CLLers following this protocol also know there is debate in the medical and patient community about its advisability, with some people swearing by it and others swearing you have to be crazy to do it. My own experience with MP at much lower doses, as well as with Rituxan in combination, is giving me some interesting insights into the issue. I promise to go into them in detail.

In the interim, suffice it to say that my AIHA treatment plan has had big consequences for my CLL, which brings us back to the weight loss. As you debulk, the weight has to go somewhere. Fortunately, methylprednisolone has lympholytic activity -- that is, it kills off CLL cells -- which are passed as urine. But it is by no means a miracle worker in this department. There are three compartments where CLL hides: the lymphatic system (including spleen), the bone marrow, and the peripheral blood. Steroids push the CLL out of the first two; what isn’t killed circulates in the peripheral blood, driving the absolute lymphocyte count upwards. If you let the CLL just float there, once you have stopped the steroids it is only a matter of time -- a couple of weeks, a month -- before the white trash is back in the other compartments, making babies and cleaning up after the hurricane.

This is why my hem/onc and I will meet Monday to go over the situation; my MP dosage was cut by 50% two days ago and I will probably be off of it entirely soon. Perhaps we will consider upping the Rituxan dosages to take advantage of this window of opportunity.

Not all of the weight loss is CLL. Muscle wasting is one side effect of steroids and it appears to be part of the problem in my case. (Muscles can be built back up, so it isn’t permanent.) This is why I ended up at a General Nutrition Center store yesterday, buying one of those clown-sized jars of “the world’s most powerful weight gain formula.” High protein, low glucose, a thousand calories in every 16 delectable ounces.

Indeed, the challenge for the past week has been to eat enough calories. Like almost everyone reading this, most of my life has been spent with the opposite problem. Suddenly I find myself in a bizarro world where ingesting exessive calories is a good thing. The other day, for the first time in my life, I ate steak and eggs for breakfast, swimming in butter. It is a sad day when you troll the nutritional information sheet for Burger King looking for the highest-calorie sandwich you can find (Triple Whopper with Cheese, 1230 calories). For once I am free -- nay required -- to eat with abandon, but I have to avoid things with lots of sugar, as steroids can raise glucose levels. I have a glucose meter at home and while my levels have gotten a little high at times, they have stayed well away from the magic number of 200, which is when we radio Houston and tell them we have a problem.

So I am eating more and enjoying it less. On the plus side, I haven’t looked this trim in about 20 years. There is an opportunity here, post-steroid, to build back muscle and stay at a lower, healthier weight. And, of course, there is an opportunity here to deal with the CLL in a more meaningful way than I have in the past, since I debulked much better than I would have imagined.

In the meantime, I have a spring in my step -- both from my returning red counts and my lighter weight. Thanks to all of you who have written me with your messages of support, as well as with some invaluable advice about managing life with steroids. The one thing I keep saying about chronic lymphocytic leukemia is that we are all in this journey together, and that our caring for one another makes an incalculable difference. I have learned first-hand recently that this is true.

Now I think I'll go have lunch. Bucket of chicken, anyone?

It's raining WHAT?

There’s a road in California pasture country called Brown Material Road. It’s one of my favorite road names, and there’s little doubt about the nature of the brown material that the name refers to. Well, Marilyn and I have been driving down our own Brown Material Road lately. Upping the metaphorical ante, let’s just say we’ve been caught in a shitstorm of events, the sort of telescoping of crap in a short frame of time that can tax your ability to cope on every level. A friend of mine was kind enough to provide a weather map showing a shitstorm over our home in Arizona, and it is posted below for your amusement, and mine in retrospect. (Hopefully it will not head your way as it meanders toward its usual resting place, Washington, D.C.)

Suffice it to say that one element of the storm is called Autoimmune Hemolytic Anemia, which I suspected I had on a Friday and which I began treatment for on the following Wednesday. So far, the treatment (steroids at a rather high dose, backed by cautionary antibacterial and antifungal drugs) seems to be working. This all sounds so easy as I write it, now that I have had a chance to catch my breath (oh God, an anemia pun of all things)! I promise to go into the experience in detail one of these days, as there is a lot to tell (not the least of which is the way this all is playing into my current CLL treatment plan, which was about to get started when the AIHA hit.)

But the part of the story I want to mention today is the importance of a patient being proactive. Thanks to all the time I have wasted reading CLL patient sites and groups on the internet, I was way ahead of everyone in suspecting what was really going on, in insisting on the appropriate tests, and in getting the attention of those in the busy hem/onc’s office who needed to know and to act on my behalf. Now, sometimes I make lousy decisions, but in this case Marilyn and I played it just about right: the lesson is, if you want to get good medical attention, do whatever you have to to get the ball rolling and then monitor the process closely, making sure the ball gets to where it has to go. This made the difference between me getting help in time and me collapsing on the street and needing transfusions.

And so a word of thanks is in order. This was the first medical crisis, as it were, that I have had with my CLL. Being able to quickly access information from CLL Topics (thank you, Chaya and PC), the Professors’ Posts on ACOR (thank you, Susan and Terry), and the experiences of fellow patients on CLL Forum (thanks, Denise and all) helped save my butt. I did not have to reinvent the wheel and was able to come up to speed on the subject quickly. What a marvelous (worldwide) web we weave when first we practice to CLL deceive.

So I am now recuperating and looking at the effect of this on my CLL treatment plan -- 72 mg of methylprednisolone daily does wonders for slimming the neck and spleen and pushing crap out of the marrow, if only it could be made sort of semi-permanent in a non-toxic kind of a way . . . And, of course, there is dealing with the other non-CLL issues that have hit us like frozen blue ice falling from an airplane toilet in the sky. So while this plays out I may be rather absent from my usual internet haunts, and I may not post here quite so frequently. Other priorities demand my attention. When the shitstorm hits, you drop everything but the most immediate and essential. But I’ll be back with my story one of these days, and I expect to have some interesting things to tell. Meanwhile, watch out for brown clouds on the horizon.

Truncheons and dragons: 13 ways to fight CLL

“How long do I have, doc?” It’s a line from the movies that people with chronic lymphocytic leukemia sometimes repeat after they’ve been diagnosed, which is when life can start to seem like a B-grade B-cell melodrama.

It’s a natural question, and one that is unanswerable. CLL varies greatly in its aggressiveness and in patient response to treatment. I read the statistic somewhere that about half of all CLL patients will die of the disease, and we now know that certain prognostic test results can indicate which patients may be more likely to have disease that will progress. But that doesn’t mean they’ll die; even patients with the worst prognostics can get lucky and have a stem cell transplant that cures their CLL. Ultimately, “how long you have” is still the province of Fate rather than man.

So, to the oft-asked question “How long do I have to live?,” I offer in reply a paraphrase of John F. Kennedy: “Ask not how long you have to live. Ask what you can do to help yourself live longer.”

In that spirit, here are some suggestions that may help keep the dragon, as some people are wont to call leukemia, in its place:

1. Get yourself a good local doctor. Qualities to look for: thoroughness, thoughtfulness, a willingness to listen and learn, to read stuff you bring in off the internet, to explain things clearly, to work with a CLL expert. Qualities to avoid: rigidity and dismissiveness. In his book Come Hell on High Water (A Really Sullen Memoir), Gregory Jaynes writes about a fellow passenger on a ship: “It occurred to me that he has the self-confident bearing of a successful and God-like physician, either that or an extraordinarily secure idiot; for most of my life I’ve been incapable of telling the two apart.” Doctors aren’t infallible, and a good one -- no matter how accomplished or famous -- brings to his work a healthy dash of humility. CLL is tricky and the landscape of care is in an almost constant state of change, so it should humble everyone who toils with it.

2. Consult at least one CLL expert, preferably two at different institutions. Experts often disagree and their views may be colored by the research their own cancer center is doing and the clinical trials they are conducting. (When visiting a CLL expert, avoid getting caught up in the moment and agreeing to jump into a clinical trial without giving it some thought and research.) All this makes the good local doctor all the more important: It helps to have an honest broker who can, with impartiality and from the vantage point of distance, help you sift through both expert recommendations and the latest ideas for treating the disease.

3. Go with a friend. I hope you have a spouse or a relative or a friend who is willing to hold your hand, lend a shoulder to cry on, go to the doctor with you, help you take notes, ask questions, and sort through what CLL means on all levels. Caretakers provide emotional support as well as a second brain when dealing with complex medical issues. Remember to give them your love and gratitude, as well as this important gift: time and space of their own, away from CLL. (And if there is no one close at hand, patient websites such as CLL Forum have a number of members who are willing to help from a distance; for face-to-face contact, the Leukemia and Lymphoma Society provides local support meetings.)

4. Do everything you can to find or maintain the best health insurance possible. What good is the best treatment or most important test if your insurance won’t pay for it? Finagling good insurance is worthwhile, even if it means a career change or moving to another locale. (Readers of this blog know that my current insurance won’t pay for stem cell transplants and that I have to do something about that. Stay tuned.) A very useful website covering insurance laws and options in all 50 states is this: http://www.healthinsuranceinfo.net

5. Have your prognostic tests done so you have some idea of what you’re dealing with. These tests are IgVH mutational status, FISH, and CD 38. Making a treatment decision without knowing these is foolhardy. There is a growing body of evidence that these tests can indicate which patients are more likely to have progressing, even aggressive, disease; this evidence also shows that there are different responses to treatment depending upon these test results. The risk-based approach to CLL is the wave of the future and the future is here now. Quest Diagnostics is covered by most US insurance plans and can run all three of these tests; you usually can have the IgVH test done for free as part of a visit to a CLL Research Consortium center. Dr. Terry Hamblin’s lab in the UK can also do the tests. (ZAP-70 is a valuable test but it is in the working-out-the-testing-kinks stages; it is best done at a research institution such as UC San Diego as opposed to a commercial lab such as Quest.)

6. Become familiar with the criteria for starting treatment, especially the NCI guidelines, and remember that good doctors believe one should “treat the patient, not the numbers.” Take a look at Dr. Hamblin’s three-part blog post titled What is the aim of treatment? Learn to finesse the treatment choices in front of you, to judge their potential risks and rewards, to consider how they can be timed and staggered for your greatest benefit. Consider the opportunity cost in waiting too long or starting too soon. These skills are something that come with knowledge and experience, yet they are essential to charting the right course. Even then, there are no sure answers, just educated guesses (and making the occasional mistake can be part of the struggle). If you are in "watch and wait," use it as a time to learn.

7. Take the long view. Ask what treatment you’ll do when you relapse from this one. Make a flow chart if you have to. Strategize for the future. CLL is like baseball -- it has nine innings, not one, and it can even go into overtime. You can win the first inning -- and in CLL treatment, you almost always do -- but that won’t help in the long run if you lose the ones that follow.

8. Use the internet. Run your questions and ideas by your fellow patients in discussion groups such as CLL Forum and the ACOR list, read sites such as CLL Topics and Dr. Hamblin’s blog. Between these four sources alone there is enough information to answer all your questions, bring you up to speed on the latest in CLL care and management, and offer a valuable perspective on the choices in front of you. All you have to do is read and participate. Is your life work a little homework, a little networking on the net? Whatever you do, don’t keep your own counsel. It is said that a lawyer who represents himself has a fool for a client. The same can be said of a CLL patient who reaches conclusions and doesn’t run them by others, including doctors and fellow patients. No matter how smart or clever you think you are, CLL is a tricky business and you will benefit from sharing your suppositions with others.

9. Avoid comorbidities. Take care of yourself -- adding lung cancer or heart disease or diabetes to the CLL equation only complicates matters and narrows your choices. In other words, quite the freaking cigarettes and don’t embrace your inner glutton. The more fit you are, the better you will respond to therapy and the greater your chances of a successful transplant, if it comes to that.

10. Be proactive. If you get a strange symptom, take it seriously. If your doctor’s office is slow to respond to something, or if a nurse appears to be confused about things, or if your health insurer balks about paying for a needed test, set them straight. You can often catch more flies with honey, but be a pain in the ass when you have to. I saw a T shirt once that read: “The more I complain, the longer God lets me live.” There is a certain truth to that.

11. Don’t get freaked out by your "new normal" to the point that you make decisions based on fear or panic. Do things such as yoga, exercise, and meditation to maintain a calm, centered focus and to keep CLL in its place as only one part of your life, not the whole of it. As one patient recently put it, "Every morning I look outside to see our beautiful world. Great weather, terrible weather, it doesn't matter. I step outside and draw in a huge breath and thank God for letting me have another day here on earth. That takes the edge off anything that might cause grief the rest of the day. It's already started out as a good day." If and when the monsters start lurking in the night, remember my favorite panic button quote, from lung cancer survivor Greg Anderson in the book 50 Essential Things to Do When Your Doctor Says It's Cancer: “Panic is a projection that is not real. We are not just our fears. Our fears do not necessarily determine our future. This is significant.”

12. Don’t give up. Where there’s hope, there’s life. If you doubt what I’m saying, read Dr. Jerome Groopman’s The Anatomy of Hope. People beat the odds daily. You can, too.

13. In the final analysis, always trust your own intuition, and make no important decision without consulting it. If something doesn’t feel right, don’t do it. When you find a course of action you truly believe in, put your soul, as well as your body, into it.

"Watch and wait"