Re-run

• Hemoglobin 12.3 (WHO normal 14.0-17.3)
• Absolute neutrophil count 1.2 (normal 1.4-7.5)
  
• White blood cell count 2.5 (normal 4-10)
• Platelet count 95 (normal 150-450)

Lots has happened since my last post 10 months ago. My book finally came out. Saw my partner through finishing her book. (Well, mostly finishing. Book publishing drags on and on, long after you think you're done.) Worked hard, played hard, loved my kid like crazy. Had some great vacation time. Paris, Avignon, Capitol Reef, Santa Fe, Taos, Point Reyes, Big Sur.

Now, here it comes again.

I actually felt it coming back in March, long before it showed up in the tests. Exercise seemed harder than it should be. My pulse went up too fast and just kept pounding. I went in for a blood test off the schedule, riling up the clerical staff and annoying the nurses. White and red cell counts were subnormal, but just barely. Hemoglobin and hematocrit still looked normal, though, so I chalked it up to being out of shape from a winter spent doing more walking than aerobics. Stopped worrying.

But in July, everything was down. Hemoglobin 12.8, WBC 2.4, RBC 3.56. Not good, but still outside the treatable range. I bought a bike computer and started monitoring my pulse carefully. Even on a flat ride, going fast but not really pushing, my pulse would hit 160 in a couple of minutes — and stay there. Pushing, I could drive it to 175, no problem.

I'm 52, so that's pretty high. When I'm normal, 145-150 would be a more typical range for me on a hard ride, and I'd have to drive myself to the limit to hit 170. I got worried that I might damage my heart, though I could not find any research about exercising with low hemoglobin. (Probably most people are not stupid enough to try.) So I set an arbitrary goal of not exceeding 165 and keeping the average pulse around 155, and that seemed ok. I keep asking doctors, but nobody seems to know. (Please comment on this post if you do!)

Since July there's been a gradual slide in the counts, so I saw it coming. Had a bone marrow biopsy a week ago: 80-90% hairy cells. Interestingly, the cells' antigen profile has changed. According to Dr. B, that's because cladribine destroyed the population most vulnerable to it in the first round, so the new hairy cells grew from the ones it couldn't kill — the ones more resistant to the drug. Evolution at work, inside your body.

Looks like I'm going to enter a clinical trial at the National Cancer Institute under Dr. Robert Kreitman. I trust Dr. B, but he's merely a leukemia expert, and there are dozens of leukemias. Kreitman is a hairy cell leukemia expert — he doesn't do anything else. Jon Howard's blog about his experiences in this trial is absolutely great. (Corresponding with Jon is actually what led me to join the trial. I gather from the nurses at the NCI that I'm not his first recruit.)

Kreitman's trial is testing a 5-day course of cladribine plus 8 weeks of rituxan (once a week). Cladribine is the heavy artillery; it kills most of the hairy cells and breaks up the clumps they form. Then rituxan mops up the rest. The trial has two arms:

• cladribine with 8 weeks of rituxan immediately following
• cladribine, then wait 6 months, then 8 weeks of rituxan

You get randomized into one of the arms when you're accepted into the trial, so I won't know for a while which one I'll be doing. Rituxan (generic name, rituximab) is a monoclonal antibody. It binds to the CD-20 antigen on the cell surfaces and they die. The exact mechanism of action is uncertain. (Maybe it kills them, maybe they kill themselves.) It kills other B-cells, too, but the clinical consequences are minimal.


The idea of the second trial arm is that most people reach their lowest hairy cell counts about 6 months after cladribine. Dr. B isn't sure that's the best idea — he says that in all other forms of leukemia, concomitant administration of purine analogs and rituxan seems to work best. But hairy cell is unusual, so he might be wrong. Hence the trial.

I'll have to go to Bethesda, MD for 5 days to start the trial, probably in the first week of November. First two days, they test for everything. CT scan, EKG, MRI, chest x-ray, PFT (whatever that is), blood tests, etc.. Third and fourth days, cladribine (and rituxan if you're assigned to that trial arm). After that you go home and do the rest of the treatments there (i.e., at your local hospital, not in your house. You have to be in an infusion center to get this stuff, since it can kill you if something goes wrong.) They provide all the drugs. At $20,000 a dose for rituxan, that's no small thing.

The treatment plan's great, there's followup care until you die, it's nearly free, the leader of the trial is one of the two best hairy cell leukemia researchers in the world, and you can do most of the tests and treatment at home. Hard to argue with that.

My attitude: this is a chronic disease. While your counts are good, you live your life without worry. It comes back, you treat it. Almost every HCL patient dies of something else. This regimen could bring the disease level down to undetectable, at least for a while. There's maintenance therapy — rituxan, as needed — that has a serious shot at keeping it that way forever. This is as close to a cure as I am likely to get.