Symptoms

Everybody with anemia (a generic term for low red blood counts) gets headaches and fatigue. Me too. But here's a little catalog of some more exotic symptoms, more or less in the order they occurred starting in March 2010.

Some of these may not be directly related to HCL. Yet everything on this list was also present as I slowly crashed in the spring and summer of 2007, before my first treatment — and disappeared for 2 years or more after cladribine.

• Increase in heart rate during exercise. See last post. Almost certainly an effect of low hemoglobin.
• Nighttime leg cramps. I started getting these years ago, maybe around 2003, long before HCL. Lots of perfectly healthy people start getting these as they age, and nobody knows exactly why. They tested me for peripheral artery disease and it came up negative. The cramps came and went for years, but in 2007 they got worse and worse, waking me up every 2 hours or so all night, every night. They're totally brutal — come on in a flash, jerk you right out of bed. If you can stop screaming and stand up, they vanish within 10-15 seconds, as your legs fill up with blood. Probably it's low hemoglobin and/or low red count. The legs, furthest from the heart, aren't getting enough oxygen; eventually they cramp to try to force some blood through the veins. My cramps stopped completely about 3 months after cladribine.
  
• Night sweats. Also common for leukemia patients. In summer 2007, I woke up drenched in sweat almost every night. They started again 2-3 months ago. Haven't been nearly as bad this time — damp, never soaking — and they've actually more or less stopped in the last 2 weeks.
• Hearing my pulse. On Bastille Day 2007, we went to see the fireworks in Uzès, a small city in the south of France. We had a great view but we were way too close to the launchers; it felt like an artillery barrage. My ears rang for weeks. I know all about tinnitus (ringing in the ears) from years of electric guitar and rock concerts. This time, though, it wasn't just ringing. I started hearing my pulse in my left ear. Squish, squoosh, squish. Apparently it's not uncommon for tinnitus sufferers to have this, so I chalked it up to the fireworks. But unbeknownst to me, the HCL was already far along. 2 months after cladribine, I realized that the sound had disappeared altogether. This August it came back again. And it's getting louder. Going to sleep, squish squoosh, feels like lying at the bottom of a swimming pool next to a slowly churning pump. Why? My guess: as my blood gets thinner, the blood vessels get less taut — something like the difference between a (soundless) hose blasting water at full strength and the gurgling you hear when the water's running too slowly to completely fill the hose.
• Lightheadedness. That's not even really the right word. Not dizzy, don't feel like I'm going to fall over. I can still think and talk just fine. Just a feeling of not being all there.

None of this persisted after treatment in 2007.

Not all symptoms are bad. Two more:

• Joy in small things. I like this one a lot. No mortal fear this time; I know I'll recover. Yet I'm sharply aware of mortality, and right now I'm taking a quiet pleasure in everything. My partner, my beautiful child. Work, colleagues, friends, family. Cold air, changing leaves. Bicycles, walking, fixing things. Even email.
• Weight loss. I hit 185 lbs in 2004, when Luka was a year old. We were living in South Africa, where you're always at a braai eating sausages. I felt like pork city and really had to work to drop that weight. For the last 6 years I've been cycling between two set points, 173-175 lbs and 163-165 lbs. I'll go a year or two at 174, which feels chunky. Then over a summer I'll focus on eating better, count calories for a few weeks, and drop back to 164, which feels about right for a 5'10" active guy. I'll hang there for a year or two; then it creeps back up again, usually during a winter when I'm always cold and working too hard.
  

This summer I'd been struggling through the weight-loss phase. Got down to 166 but couldn't break through it. In the last 2 months, though, it suddenly got a lot easier. Part summer and healthier food, part carpentry; I built my son a playhouse. I love that kind of project, working outside, moving all day long, using my hands and my strength. In fact, I love it so much I forget to eat. The combination of high output and low input often takes off a few pounds in a week or two. In my next life, I'm coming back as a contractor.

But now I've rather suddenly dropped below my 164 set point, down to 160-161. This has been almost impossible in the past, but this time it just happened, no effort on my part. I suspect there's a disease process at work here. So I'm focusing on eating a bit more. Fortunately, a cup or two of dark chocolate mousse at night puts me right back to 165 in a few days. You don't want to be underweight if you're at risk for pneumonia, which I will be in a month or so, after treatment. So I'll keep monitoring. And wolfing down chocolate mousse. Should that prove necessary.

I've certainly had worse problems.

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.

Full normal jacket

At my last blood test, back in August, I got worried. My blood counts all dropped at once. Still in the normal range, but everything was down. I thought oh no, here it comes. Dr. B gave me a sickly smile. Those counts bounce around all the time, he said. Oh yeah — and I didn't see anything that even looked like a hairy cell on the slide.

Yeah sure. Nice try, I thought. Let's pretend. A lot of HCL patients end up back in chemo right around the 2-year mark. I figured on getting ready to be one of them. Beginning of the end, blah blah.

But no. Not this time. My last test, in October: white counts, red counts, platelets, neutrophils — everything except lymphocytes smack in the middle of the normal range. And lymphocytes at 0.8! That's normal too, for the first time in 2 years.

So I'm doing fine. And I'm happy. Working too hard. Finally finished my endless book (coming out in March 2010); just sent the page proofs back last Monday. Sweating under the overload of coursework, committees, childcare, travel, new research, meetings, everything else. But doing just fine.