Patent

Intriguing: a lawsuit involving my disease paved the way for the patenting of genetic sequences and engineered organisms:

In 1976 a patient at the University of California medical center began treatment for hairy-cell leukemia. The doctors recognized that his blood might have special properties for the treatment of leukemia and, in 1981, they were granted a patent in the name of the University of California on a T-cell line — that is, a sequence of genetic information — developed from the patient's blood; the potential value of the products derived from it was estimated at three billion dollars. The patient sued the university for ownership of the T cells and the genetic information, but the California Supreme Court ruled against him. The court reasoned that the University of California was the rightful owner of the cell line because a naturally occurring organism (on which his claim rests) is not patentable, whereas the information scientists derive from it is patentable because it is the result of human ingenuity. (Hardt & Negri, Multitude, 183.)

The landmark case was John Moore v. the Regents of the University of California.

Singing bowl

• Hemoglobin 8.9 (normal 14.0-17.3)
• Absolute neutrophil count: 0.3 (normal 1.4-7.5)
• White blood cell count: 0.5 (normal 4-10)

Yesterday might have been the low point. Walked in the morning, for over an hour, but it completely wiped me out and I spent the entire afternoon in bed. Fatigue from this illness can't be easily described; tired but not sleepy, foggy-headed but lucid at the same time. If you've been wondering where I find energy to write this blog, well first, yesterday I didn't, and second it's somehow a thing I can do even though concentrating on other things seems completely impossible.

Hemoglobin's up slightly from Monday, though the white count is down a bit. All according to plan. They'd scheduled a transfusion for Friday, but now the nurse thinks there's no need. And I do have a bit more energy today.

Yesterday was also my 50th birthday. Not the celebration I'd hoped for. It would've been small and quiet anyway, but more joyful than I could muster in these circumstances. My beautiful Gabrielle gave me a lovely Tibetan singing bowl, deep-throated and intense, intricately decorated with Tibetan script and designs. I've got another one of these, given to me by friends 20 years ago on the occasion of another breakdown: arthroscopic surgery on my left knee — much less serious than this, but still a health crisis. There's a sweet symmetry in these two gifts.

These bowls make the purest sounds it's possible to hear. I don't play them often, because it's a sound you really need to be present to hear, to deserve.

One of my most intense dreams, ever, involved being pursued by shadowy figures through a whole series of scenes, ending in an enormous mansion where I ran from room to room evading them. At the end I came suddenly into an enormous hall filled with people, all talking loudly, a party or reception or something. On the floor in front of me lay the broken halves of a huge singing bowl, cracked down the middle as if by a lightning bolt.

I seized them in my hands and pressed them back together. Then I rang the bowl. The sound grew and grew, a titanic tone of awesome purity and power.

Everything stopped: the talking, the people, my shadowy pursuers. Time itself.

Everyone looked at me.

And then, into this immense stillness, I began to make the sound.

I could not get that dream out of my mind for months afterward. How I had healed what was broken, and with it stopped the world.

Low

• Hemoglobin 8.7 (normal 13.0-17.3)
• Absolute neutrophil count: 0.4 (normal 1.4-7.5)
• White blood cell count: 0.6 (normal 4-10)

The nurse just called to report my counts from yesterday's test. Nice; means I don't have to go pick them up, though I also want to see the DNA test and the CT scan results that weren't ready last week.

These counts will drop a bit more before they start to pick up again. Hemoglobin below 10.0 is really low; explains my constant exhaustion right now. I'm staying in bed a lot.

Infection risk is now truly severe, as predicted. Gabrielle managed to stay clear of Luka's cold for over a week, but it finally got her yesterday, despite everything, so now we have to work even harder to keep me isolated. Without many white cells, my body's response to infection won't be normal in any way. The main thing is to track my temperature and go to the hospital if I get a fever. The next week or so will be the hardest.

Have to get a repeat test on Thursday, and the nurse is scheduling a blood transfusion for Friday. May not happen; it depends on what the counts do and just how bad I feel. Transfusions have their own risks, so I don't want to do one if I don't have to, but I think on balance infection is more dangerous.

GIGO

GIGO: geek-speak for "garbage in, garbage out." What you get is only as good as what you put in.

In the case of the human body, that would be food. Everybody knows Americans have a food problem. Around 25 percent of us are clinically obese. Another 25-50 percent could stand to lose some weight. "Meals" of fast food, candy bars, soda. We don't walk, don't exercise, won't even take a flight of stairs if there's an elevator. I could go on about this, but let's get back to leukemia.

My doctor told me I could eat anything — before, during, and after chemo. (The only exception was alcohol, which can depress the immune system.) We asked him if there were things I should be eating, things that might help. He said no, they didn't know of any specifically helpful foods. He advised against supplements because he thinks they don't work, but he said (in so many words) that he had no data to back this up.

Typical. Most doctors know zip about nutrition. Med schools don't teach it. Doctors are trained, formally or informally, to see the body as a homeostat that regulates its own levels and can't be much affected by the particular food you take in. Thankfully, this is finally beginning to change.

I already wrote about the foods I can't eat until my neutrophil counts recover (fresh fruit, raw meat/fish, leftovers — basically anything that might have little nasties growing in it.) But what about food, or nutrients, that might actually help? Can I maximize my chances in the standoff that's about to start?

The best source I found is Food, Nutrition and the Prevention of Cancer: a global per-
spective (Nov. 2007, just released). This 537-page monster is a state-of-the-art report, a five-year project of the World Cancer Research Institute. A huge panel of scientists reviewed the relevant literature and summarized the consensus findings. The bibliography is 95 pages of tiny type. It goes through every type of cancer, outlining what's known about the preventive and harmful effects of various foods and food groups.

No huge surprises here, but some very clear recommendations. Excess weight — not just obesity, but even a few extra pounds — promotes cancer. Diet and exercise help in weight control. But more specifically, minimize animal-based foods of all types, especially red meat and dairy, and maximize fruits and non-starchy vegetables. Any amount of alcohol increases overall cancer risk, especially for head and neck cancers, but this effect is not large below the 2-glass-a-day margin that's protective against heart disease.

As for leukemia, it's not the same as other cancers. The report doesn't spend much time on it — just 2 pages (319-320). Intriguing here is the report that leukemia incidence is much higher (2-3x) in highly developed countries, and rates are rising. Sounds prima facie like a lifestyle disease.

Here are the leukemia-specific dietary risk/benefit factors the report identifies:

• Good: vegetables, fruits, polyunsaturated fats, alcohol
  
• Bad: milk, dairy, red meat, saturated fat, overweight/obese
  

The Cancer Center gave us a handout on "superfoods" that can help prevent cancer, or even combat the disease if you already have it. The big ones are these:

• Yogurt (live-culture only)
• Garlic
• Carotenoids (in carrots and green leafy vegetables)
• Cruciferous vegetables (broccoli, brussel sprouts, cabbage, and cauliflower)
• Legumes (beans, seeds)

The expert report discusses all this too, and I confirmed much of it on PubMed. These foods' effects aren't small, either; they're pronounced. Chemicals in garlic are the subject of very active drug research, as are the various lactobacillus strains in yogurt. When is this stuff finally going to filter through to the clinicians? They should be telling everyone, at every visit.

In reviewing this literature, I noticed a (for me) new emphasis on probiotics — yogurt and fermented foods, such as tempeh and kefir, that contain beneficial microorganisms — and "prebiotic" foods, which those organisms require in order to grow in your gut. Prebiotics are mostly fiber-containing foods such as fruits, vegetables, whole grains. Eat your oatmeal.

Did you know that 90 percent of the cells in your body — aren't yours?

Of the trillions and trillions of cells in a typical human body — at least 10 times as many cells in a single individual as there are stars in the Milky Way — only about 1 in 10 is human. The other 90 percent are microbial. These microbes — a term that encompasses all forms of microscopic organisms, including bacteria, fungi, protozoa and a form of life called archaea — exist everywhere. They are found in the ears, nose, mouth, vagina, anus, as well as every inch of skin, especially the armpits, the groin and between the toes. The vast majority are in the gut, which harbors 10 trillion to 100 trillion of them.

This comes from Fat Factors, an astonishing NY Times Magazine story published in summer 2006. (You need a subscription to read the story, but if you have trouble getting access, email me.) Too much to review here. The punch line:

“Humans are superorganisms... whose metabolism represents an amalgamation of microbial and human attributes.” [Scientists] call this amalgamation — human genes plus microbial genes — the metagenome.

If this is true — and it seems totally obvious once you learn that we're unable to digest almost anything without the gigantic colonies of microbes in our guts, with which we've co-evolved over countless millennia — we are only starting to understand human health. And it will not be at all surprising to learn that probiotics can play a major role in preventing cancer.

Over

Chemo's over. About 6:15 PM yesterday my chemo pump began beeping insistently. Infusion ended, it read. All 200 ml injected. I pushed the "silence" button, but the thing kept on beeping every two minutes, even after I tried to turn it off.

We were in the middle of dinner, so I just kept pushing the "silence" button while we finished eating and drove to the hospital for my 7 PM appointment. I would've been out of there in 15 minutes, but they didn't have an explicit order to pull my PICC line, so they had to page a nurse.

As they peeled away the layers of tape and dressing, I was happily surprised to see that my skin — buried under that stuff for 10 days — looked normal. Just a bit red in a couple of spots. No gangrene, thanks.

Last blood draw through the PICC before they pulled it. (From now on it'll be needles, but I'd rather get poked than wear that f-ing thing any longer.) Pulling the line out was a sensation I'd rather not repeat, making a kind of zp-zp-zp-zp-zp-zp sound as they dragged all 47 cm of it through my poor vein. Thankfully, this only lasted five seconds. Then it was done.

Half-life of 2-CdA is 5.4 hours. It's mid-day on Tuesday now, about 18 hours post-chemo. So my blood levels are now less than one-eighth of what they were yesterday, but there's still some drug in my system. I felt completely blitzed when I woke up today. I'm guessing the chemo fatigue effect will linger another day or so. Will try to get my blood test results later and see how the hemoglobin looks.

The highlight of my day today: an unassisted, non-Saran-wrapped shower. YES.

Standoff

Almost half of HCL patients experience a relapse at some point. As one study put it, "the relapse-free survival curve does not appear to reach a plateau." In other words, even if I'm clear for 10 years, it can still come back. I'll need tests every 6-12 months for the rest of my life.

Since 2-CdA almost certainly does not eradicate the disease completely in any patient, why do 50 percent never relapse? Many, especially those diagnosed at later ages than mine, die of something else first. This could be anything, but my risk of a second cancer — of some other type — is now 6-7x greater, probably because of the disease itself rather than the treatment. (My absolute risk remains low, however.)

Still, a large proportion of patients never relapse, even though some small amount of the disease likely remains in their bodies. Nobody knows why. Somehow their bodies keep the disease in check, even though it once grew out of control. It's a stand-off.

So. What can I do? Exercise. Avoid radiation, benzene, pesticides, tobacco, mononucleosis: all things I was going to do anyway. I'll start refusing some routine dental X-rays. (Not all; my teeth are too bad not to check up on them once in a while.) Drink coffee and alcohol, in moderation. Don't get too stressed.

Stay happy. Give. Love.

Thanks-giving

Less than 12 hours until I'm liberated from the chemo pump I've been dragging around all week. I can't tell you how happy I'll be to get rid of the tube in my arm. So far so good: no fever, no pump alarms, no rash, no other symptoms except for the flattening fatigue.

I'll go over at 7 PM for the disconnect. They'll take some blood through my PICC line, then yank it out. I've had that thing for 10 days, and even though the infernal itching eventually subsided I am really looking forward to getting rid of it. Blood test results should be in by mid-day tomorrow, which should be close to the nadir of my neutrophil count. Platelets, red cells, and hemoglobin should be down too. Will report.

Yesterday I managed another long slow walk, over an hour, though I had to sit down a couple of times. Felt pretty woozy the rest of the day, but couldn't fall asleep. Luka's over his cold, so I spent real time with him, the first since he got sick 9 days ago. At dinnertime he asked, out of the blue, "Mommy and Daddy, when will I go to graduate school?" I put him to bed last night with stories about my life at 5, 6, 7 years old.

The end of Thanksgiving weekend seems like a good time to give thanks to everybody who's helped us through this. I've been overwhelmed by the response. Still haven't answered most of the hundreds of emails, though I hope to start doing that soon.

Here are just a few specifics: Gabrielle, who's done almost nothing but help me since this all began. Yan, for the superb food operation; I'm starting to think my colleagues should abandon teaching and open a restaurant instead. My parents, for everything. My uncle Larry, a radiologist, retired but always my family's first line of defense when it comes to helath problems. Half a dozen cousins, for medical consultation. Jennifer, for putting me on to Sloan Kettering. My parents' many friends, who've sent dozens of emails.

Marcia and Kirk, for support and referrals to Marcia's hematologist/oncologist brothers. John, for the awesome brownies (got me through the first few days). The Big Brain (Gabrielle's friends from MIT). Lauren and Maya, who sent me a Sony Playstation as well as Destined for Destiny: the Unauthorized Autobiography of George W. Bush. Don, who's white-knuckling through a medical ordeal of his own, and Elaine, who's doing it with him. Todd, who always understands. My brothers. Kali and Paul. Joel, for endless consulting. Susan and TR. My always-elegant Dutch colleagues, who sent flowers. Donna, who's getting through her own big-time disappointment but still finds understanding. Steve Schneider. The parents of Luka's friends. Tirtza. My workgroup: Steve, Geof and Leigh, Chris, Tom, David, Steve C., Archer.

At the School of Information: our deans, who understood how all-consuming a fight like this must be, and cut my teaching load to make time for me to get through this. Michael and Yan, who agreed to pick up my slack by taking over a crucial course. Ann. Former students and GSIs, for staying in touch. Jim, our facilities guy and a total mensch.

And many, many more. Thank you all.

Why

Short answer: nobody knows. Only a few risk factors have been identified for any kind of leukemia, much less the rare ones like mine.

Radiation exposure: my teeth are terrible, so I've had a lot of dental X-rays. Could have something to do with it — X-rays aren't high-level exposure, but there's no safe level of radiation (ask Gabrielle). Benzene: we're talking working at a gasoline refinery, not inhaling fumes while filling up your tank. Cigarette smoking: I smoked for 4 years, off and on, but I quit when I was 21 and never smoked again. Pesticides, dioxins, other chemicals most people probably don't encounter at home or in desk jobs. That's about it. Electro-magnetic fields, for example living under a high-voltage power line: evidence suggestive, but inconclusive (and I've never lived under a power line). Some people think even home electric current, and devices, can be a risk factor, but if that were true we'd have an epidemic on our hands.

How about vices? Start with coffee, to which I've been happily addicted most of my adult life. I used to live in Santa Cruz, California, many of whose citizens consider coffee just shy of heroin on the scale of consumable evil. So did I, for a few years.

But if you think coffee's bad for you, think again. Cruising PubMed brings some astonishing results. In the last ten years, coffee's been found to reduce, rather substantially, your risk of many kinds of cancers: colon, liver, pancreas, kidney, stomach. It also reduces your risk of Parkinson's disease, cognitive decline in old age, possibly Alzheimer's. It can even lower some coronary risk factors (e.g. cholesterol), though it does raise inflammation levels, which may be a risk factor. (All this is at moderate consumption levels, up to 300-400 mg caffeine/day. That's 3-4 American diner coffees, 4-5 espressos, 1-2 large Starbucks-type drip coffees. Above that, watch out.) The caffeine itself isn't the principal active agent in this risk reduction; it's other chemicals, so decaf might be just as good for you.

Coffee tends to be a health negative mainly in countries where people boil coffee, such as Sweden and Greece (but what would you expect? Boiled coffee's terrible.) If you don't believe me, just go to PubMed and enter "coffee cancer" or "coffee cardiovascular" in the search bar. Obviously if coffee's making you jittery, tense, or murderous, you probably shouldn't drink it, but not because it'll hurt you physically.

Alcohol? Same thing. Most recent research points to general benefits from drinking up to two glasses a day, not just of wine but of any kind of alcohol. It lowers coronary risk, colon cancer risk. Typically it's a J-shaped curve: non-drinkers on the left, risk dropping with 1-2 glasses/day of alcohol, then rising steeply into a danger zone with heavy drinking. The resveratrol in red wine specifically attacks leukemia cells (not necessarily the kind I have, but those are little studied). A couple of case-control studies of adult-onset leukemia showed that moderate levels of beer and liquor consumption exerted a considerable protective effect.

Those studies concluded that moderate red wine consumption somewhat increased risk (~1.5-2x). However, the evidence is imperfect (not controlled, e.g., for dietary differences), and the biggest study by far (649 leukemia patients) took place in Italy. Different lifestyle, eating habits, gene pool, medical tradition, everything. Hmmm.

Those studies addressed the most common types of leukemia: acute and chronic myeloid, acute and chronic lymphocytic. As for hairy cell, it's hard to find enough people with a rare disease to get a statistically significant result. I couldn't discover much in the literature. A 1985 case-control study — based on 45 HCL patients — concluded:

There was no association found for cigarette smoking, alcohol or coffee consumption and hairy cell leukemia. With respect to occupational risk factors... reported exposure to organic chemicals in the workplace was significantly greater among both sexes of the cases than among their respective matched controls (relative risk (RR) = 3.10). Other variables found to be significantly associated with hairy cell leukemia were farm birthplace (RR = 4.20), anemia (RR = 4.29), migraine (RR = 4.80), infectious mononucleosis (RR = 9.00), and routine use of aspirin (RR = 3.41).

None of this looks like me. No significant chemical exposure that I know of. Never had mono, migraine, or anemia; wasn't born on a farm; don't use aspirin. The only other HCL-specific risk factor I could locate in the literature was benzene, in Japanese workers.

So the answer to why, for me, will always remain nobody knows. No reason. It just happened.

Today's pattern was like yesterday: felt OK in the morning, took a long walk, wiped out by 1 PM. Slept most of the afternoon, this time a sleep so heavy it felt like I was lying under an elephant. OK through dinner, then crashed again. Just 2 more days to get through.

Wait

Pretty good energy this morning. Walked around the neighborhood for an hour with Gabrielle, circling around not too far from home just in case. The walk wiped me out, so I took a 2.5-hour nap.

Both Dr. A and the nurses stressed not to stop physical activity. If an infection happens in the next few weeks I'll need to be in the best possible shape to get through it. But the crushing waves of fatigue are real, for one thing. For another, even if my hemoglobin weren't so far down, I just can't imagine doing heavy aerobic exercise with a catheter dangling in the vena cava right outside my heart (see PICC line). Even gentle yoga poses have gotten difficult; I can't warm up my muscles enough to loosen them for stretching (low hemoglobin effect). After the nap I tried taking another walk, but had to head back after 10 minutes.

Reached the chemo halfway point this morning. The pump control panel says it's pushed 105 ml of my total 210 ml of 2-CdA. Monday evening, 7 PM, they'll disconnect me and take another blood test. I'm contemplating going over for the blood draw separately, in early afternoon, so I can get the test results when I go back for the disconnect.

After that it's just waiting. Guarding against infection, and waiting some more. Around week 2 they'll put me on a prophylactic antibiotic to prevent pneumonia. And then I'll wait some more. Yesterday I found a description of the 2-CdA clinical trial results on RxList.

In the trials, half of all patients achieved normal white counts — ANC of 1.5 or higher — within 5 weeks from Day 1 of treatment. Normal's great, and of course I want to get there, but I've been functioning for weeks, maybe months, infection-free on a count of 0.6-0.7. So if my count comes up even to 1.0, I'll stop worrying about it much. Platelets come back even faster, within 2 weeks. (And a good thing too. I remember noticing back in Amsterdam, last summer, that shaving cuts kept bleeding for an amazingly long time; now I know why.) Hemoglobin takes a bit longer — 8 weeks. That's the one I really can't wait to see. I think a lot of my noticeable symptoms — fatigue, muscle tightness and soreness, cramps, general weakness — come from this.

But maybe I won't be in the early half of the statistical spread. Maybe age, good physical condition, etc. plays in my favor on this. Maybe not.

That's all I have energy for today. Tomorrow I want to write about the question that's been nagging at me: why?

Sledgehammer

Thanksgiving Day. Snow falling early this morning. Around 11 AM, the fatigue suddenly landed on my head like a sledgehammer. Some combination of the disease and the chemo. Right now the 2-CDA is murdering every white blood cell it can find, red cells and platelets too. My hemoglobin's probably dropped again. Climbing just one flight of stairs knocks me out now.

Took a long nap and recovered some energy. Mid-afternoon I walked — slowly — for 45 minutes, the first time I've spent outdoors since Sunday. (Monday was chemo hookup, all day. Tuesday and Wednesday it rained.) It felt great to be outside, in the sweet calm of a major holiday. But I was teetering a bit by the time I got home.

I'm still quarantined upstairs. Luka's getting over his cold, but still coughing and sneezing occasionally. So I've got to stay out of his way at least one more day. I'm missing him desperately, but the timing may work out well.

The nadir of my white cell counts should come 5-10 days after chemo started, i.e. between this Saturday and next Thursday. Usually after Luka's been sick, he won't catch something else for at least 10 days. So if he recovers by Saturday, I should be able to play with him safely through the riskiest period of this treatment.

Apology

After the near-overdose of 2-CdA (see Chemo), Gabrielle called a friend of ours, a distinguished professor at the hospital (and also a clinician), to talk about the incident. You should think about calling this in, he said. People die of chemo overdoses. Oncologists are very sensitive to these things. The incorrect drug order (about 12% more drug than I needed) probably wouldn't have hurt me too much. But the medical system needs to know about breakdowns like this, in case other people might get hurt by the same error.

Gabrielle called Dr. A's office the afternoon after the chemo hookup and told them what happened. The nurse said she would get an urgent message to the doctor. Around 8 PM, he called back, from his home phone. (His name showed up on the caller ID.) He immediately apologized for the mixup, and took full responsibility. He'd been looking at my chart on Careweb, the UM electronic patient record system. Somehow, when he went to check my weight, he'd looked at another patient's record without noticing. (!!)

After the mistake, he'd gone back to check my record. My hypothesis of a pounds-to-kilos conversion error — something that might have been systemic, not unique to my case — was wrong. My record correctly listed my weight at 76 or 77 kg at each visit; nowhere did the number 84 kg appear. He apologized again, several times, very gracefully. We make mistakes, he said. We are not God.

Dr. A went on to say that he thought trust between a doctor and his patient was extremely important. He hoped my trust in him would not be undermined by this episode. I said yes, it did undermine our trust, but we accept your apology. He said he'd understand if I decided I wanted another doctor now, and he would help set that up if I asked him to.

All this was entirely correct, as the French would say. Impeccable behavior. But not the end of the story. Gabrielle pointed out that he only called to apologize after we called him; he'd known about the mixup the previous afternoon, since he'd had to revise the drug order.

Also, this was not Dr. A's first mistake. At the second visit, when he gave us the diagnosis and the treatment plan, Gabrielle had asked about foods I should and should not eat. Dr. A basically said I could eat anything, except supplements.

This is not true. Patients with neutropenia (low neutrophil count) in the 0.5-1.4 range should not eat raw foods, especially meat, sushi, and fruits that can't be peeled, due to the risk of infection. I'm at 0.7 right now. He should have given me this advice immediately. And once chemo started and my neutrophil count began to drop further, below the 0.5 "severe risk" threshold, I can't eat fresh fruit at all (only canned).

There are a lot of other, very specific things to know about foods to avoid, and also foods to consume: lots of protein, garlic, ginger... I won't go into it all here. If Dr. A didn't know about this, he should have. If he did know but couldn't remember details, he should have sent us to the nurses or the Oncology Dept. dietitian right around the corner from his office.

Dr. A's apology was menschlich, for sure. But this second error amplifies my concern that he's treating this case too cavalierly: an easy cancer, good prognosis, easy treatment. No big deal.

He might also have been trying to avoid having our concern about the near-overdose escalate into what the hospital calls an "incident report." So far, it's all between him and us and the nurses. Were I to file a formal report, that could damage his reputation. Anybody's to be excused for self-preserving instincts, but all this adds up to me wanting to find a new doctor. I'll sit on this a few days before I decide. Dr. A offered us an easy way out, and we can certainly take it.

Chemo

So now I'm hooked up to this pump, 24 hours a day for the next 7 days. It's dispensing 2-CdA at 1.2 ml/hr. That's about one big drop every hour. Wikipedia on 2-CdA:

"A purine analog, it... mimics the nucleoside adenosine and thus inhibits the enzyme adenosine deaminase, which interferes with the cell's ability to process DNA. It is easily destroyed by normal cells in the body except for blood cells, with the result that it produces relatively few side effects and results in very little non-target cell loss."

The pump weighs maybe 3 pounds, comes in a cute black fanny pack specially made to hold the pump, some extra AA batteries, and however much of the 4-foot tube you want to stow in there. You get to figure out how best to wear it: under your clothes, coming out the end of your sleeve or through your collar, etc. It seems indestructible, looks made to military specs. If something goes wrong an alarm starts to beep and instructions appear on the screen. The most common things are kinks in the tubing or air in the line. There's a 24-hour nurse help line.

So far, so good. I feel — absolutely nothing. I thought it would be hard to sleep with this thing, but I put the pump in the middle of the bed and really hardly noticed it except when turning over. No alarms yet.

Today I'm wearing it under my clothes, with the fanny pack in front. Wearing it in back seems too dangerous — easy to bang it into something — and makes it hard to sit down, anyway. I think I'll try to start working again tomorrow, but for today I'm just going to get used to this, take a nap, try to help Gabrielle, who has been working herself to the bone.

Luka's come down with a nasty cough, and we hear scary reports of a bacterial bronchitis making the rounds of Ann Arbor schools. So I'm quarantined upstairs, sleeping in the guest room to keep out of his way. Until Luka gets well, Gabrielle has all the childcare, plus everything else she has to do: errands, food shopping, cooking, organizing childcare and housework help, not to mention trying to do her job.

The food operation started up last night: friends and colleagues bringing meals to help out. Ann Z started things off with a delicious roast pork loin, green beans, and homemade applesauce. I did not expect to be eating so well!

Don't know who's reading this blog, but I want all of you to know — and there are dozens of you — how immensely, deeply, profoundly grateful I am for all your help. I'm swamped with emails and phone calls, so if you don't hear back from me right away just know that I am reading everything, hearing all the messages, and feeling amazingly supported and loved. Eventually I hope I'll be able to answer you all individually, but for now I have to focus on getting well. You're magnificent.

911

We were sitting in an exam room yesterday, waiting for Dr. A, when the following announcement came across the hospital intercom:

We are experiencing a temporary interruption in paging services. To report a cardiac arrest or medical emergency, dial 911.

Words fail me.

Just try to imagine exactly how this might work. It makes a good mind-bender, a bit like the circularity problem with time travel.

SNAFU

In case you don't know the origin of that acronym, look here.

Chemo hookup day (yesterday) got pretty interesting, though not in a good way.

Hookup was supposed to happen at 10:30 AM, right after my visit with Dr. A. We sat down to wait in the Infusion Clinic. (That name makes me think of chamomile tea, but you don't want these infusions in your cup if you can help it.) I wore my NIOSH-95 surgical mask: great at keeping out viruses, but man is that thing a bear to wear. The metal nosepiece grinds into your cartilage, and breathing through it takes real effort. If you breathe too shallowly, you'll just recycle your own CO2 and then pass out. More bald kids in scrubs, one of them, about 4 years old, squealing in delight while Mom chased her into the infusion clinic. Happiness, even here.

At noon, a nurse finally appeared. She told us that the hospital's Home Med department handles the pump, the drug supply, and all the ancillary equipment. Until recently Home Med was a separate company, and its offices are on the other side of town, three miles from the hospital. Dr. A's office had failed to process the order for drugs and supplies earlier, so Home Med had just received it. Over the weekend we'd learned from the Visiting Nurses that Home Med was also supposed to have delivered chemo spill supplies, extra dressings, and heparin syringes to our house. They never got that order either. According to Home Med, this happens all the time. They even have a whole system — drivers, delivery people, a protocol — for working up "same day" prescriptions and delivering them fast.

So after teaching us how to use the chemo pump, the nurse sent us home to wait some more. We returned at 2:30, waited for another hour. A linebacker type wearing blue scrubs finally appeared with a Macy's-style two-handle shopping bag full of supplies. They got ready to plug me in.

Then one of the nurses eyeballed me, as I was rolling up my sleeve, and said (in a slightly disbelieving tone), so, you really weigh 185 lbs? No, I said, I was 168 in my clothes just a few hours earlier, right here in Hematology/Oncology. On my home scale I'm 165-166.

All three nurses began to scramble, furiously looking up records and making phone calls. One led me down the hall to weigh me again. 171 lbs. after a big lunch and a lot of water.

How much drug they give you depends on how much you weigh: so many milliliters of drug for each kilogram of body weight. If I weigh 185, I get one amount; if I weigh 168 I get less. They're allowed a 10% "fudge factor," they said. But the difference between 168 and 185 crossed over that line.

I think I know exactly what happened here. That morning, Hematology weighed me in at 168 lbs. Somewhere along the line, somebody converted pounds to kilos — and simply divided by 2 (instead of 2.2, the correct factor), recording my weight as 84 kg (should have been 76). Since the dose calculation uses kilos, 84 kg became the basis for the incorrect dose calculation. [Posted later: this turned out to be wrong.]

They were about to give me an overdose. If that nurse hadn't had such a good eye for body weight, I would have gone home with too much medicine dripping into my blood. If she hadn't known how to correctly convert kilos to pounds, she might not have spotted it at all, since her eye for kilos probably isn't as good.

Now they had to get the doctor to revise the prescription and send it in, then wait for their pharmacy to fill it. So they sent us home again, promising that Home Med would deliver the drug straight to our door, and Nurse M would come by to hook me up. This finally happened around 7:30 PM, eleven hours after we'd originally left for the hospital that morning.

Once again, nurses rule. They're the ones that track the supplies, check the doses, watch for errors, and even more important, know how to work around the problems.

Commitment

In the hospital right now, waiting for the Home Medics to deliver my pump and chemo supplies to the nurse so she can hook me up. It’s 2:30.

We got here at 8:45 this morning. They took a blood draw through the PICC (so no needles; very convenient.) Then we saw Dr. A for the first time since the diagnosis.

Reassuring, and illuminating. The CT scan results hadn't come back at the beginning of the visit, but they arrived halfway through. My spleen is enlarged, at least a little. The section he measured (right there in front of us, using a “ruler” on the screen) was 13.5cm; normal is 12. He also said that 10-15% of HCL patients don’t have enlarged spleens. I hadn’t read that myself, but it’s possible.

So my case is still atypical, but at least more indicators are hitting the mark. Fibrous, non-aspirable bone marrow. Flow cytometry positive for HCL, except for the CD10 expression. Cell morphology positive. And now a slightly enlarged spleen — not the enormous, painful spleen most people get, but it still points in the right direction.

I told Dr. A about my background in science and technology studies, a bit about my research. Then I described my reading over the last week. Dr. A said you might know more than I do about hairy cell now. I have to treat over 300 different diseases, but you only need to know about one. Check, right answer.

I gave him the Lancet article on the annexin-1 genetic marker test for HCL. He had not seen it. But he also wouldn't know about it anyway, because that’s diagnostic stuff, and that’s not what he does. The degree of specialization, and the disconnection it creates, astounds me: I think I’d have trouble treating a patient based on somebody else’s diagnosis if I didn’t know exactly how the diagnosis was reached, what could be wrong with it, and how it could be better. Dr. A promised to show the article to the hematopathologist and get his reaction. But he also said that UM would not do the test if it's not FDA-approved and commercially available. (I hadn’t thought about FDA approval for diagnostic tests, but yes, of course they need to do that.) So maybe it’s too new.

I told him about the clinical trials of rituxin, BL22, and HA22. I also told him about the Patient from Hell, who fought for (and won) rituxin maintenance therapy for his lymphoma. Are you interested in these trials? Dr. A asked me, bemusedly. I told him I was, but only after we see what happens with the first-line 2-CdA treatment. If we get a complete remission with 2-CdA, I can afford to wait and see — let the new drugs work their way through the system, find out about success rates and side effects, then decide if the disease ever returns. About half of all patients don’t get a relapse, or at least not one they die of. Either they die of something else first, or the disease doesn’t cause symptoms and doesn’t have to be treated.

Dr. A nodded vigorously. He doesn’t believe it’s possible, or even desirable, to try to eliminate all the cancer cells. A few new cancer cells grow in our bodies every day; the question isn’t whether they’ll be there, but whether they’ll escape the body’s SWAT team, start a cartel, and take over. On rituxin maintenance therapy, he thinks that in the long run they’ll abandon it. Cancers eventually mutate some kind of resistance to all chemotherapy, so eventually they always need a new drug.

Dr. A is a bit of a fatalist. I said this disease very often comes back in 3, 5, 8 years. He said 3, 5, 8 years — who knows what could happen in that time? A car accident, another cancer, a heart attack… You should stop researching this. If the treatment doesn't work, or if it comes back later, then you can start researching it again.

Interpreting him generously, I see real wisdom here. What am I going to do if I still have residual disease? I can worry and research, even though I’ll have (maybe) no symptoms and no doctor would treat me. Or I can stop worrying, get on with my life, and rely on periodic testing to tell me when to worry again. If 2-CdA works the first time, it very often works the second time too. And by then — in 3, 5, 8 years — maybe some new drug will be ready to rock.

All this convinces me of two things. One, my diagnosis isn't perfect, but it's reliable enough that I can stop wondering about it. Two, I’m ready to commit.

Just in time. Soon the nurse will come to hook up my pump.

Nurses

On Friday, at the hospital, I got a crash course on living with a PICC line. The dressing has to be changed periodically, and I have to flush the line with heparin every day. It's complicated: everything needs to stay sterile, and I have to avoid pulling the line out by accident. To take a shower, I have to Saran wrap my arm and lock it down with medical tape.

Yesterday two Michigan Visiting Nurses showed up to do the 24-hr. dressing change. Nurse R pulled out a huge stack of paperwork, most of it designed for people who will be wearing their PICC lines for many weeks or months. In the stack were some surprises. The nurses expected me to have a bag of supplies containing 5 days worth of new dressing kits, heparin syringes, and blunt needles, but all the hospital had given me was one dressing kit, one heparin syringe, and no needles. They showed me a prescription they'd been faxed for all this stuff; according to them it was something the hospital should have handled without me having to ask for it. In fact I never saw that prescription at all.

They also had virtually no information from Oncology, even though Dr. A had mentioned that the Visiting Nurses would come by for this purpose. So they didn't know what I had, or how long I'd need their services. But Oncology never sends us anything, they said. They talked about other left hand-right hand problems too, even within the hospital. As usual in most of my hospital experiences, the nurses knew a lot more than the doctors about exactly what patients would need, and they have workarounds — I've got a bag in my car... — to get you the supplies.

We learned a lot. Gabrielle and my excellent friend and colleague Yan have planned out food supplies for the next couple of weeks, with colleagues and friends, many many of them, signing up to deliver dinners on Yan's wiki (thankyouthankyouthankyou, everybody!!!). Gabrielle had already sent out some guidelines on good/bad foods, but the nurses told us more. Nurse S had worked on the cancer ward. No fresh fruit or vegetables, no raw food of any kind, she said. (Man am I going to miss sushi). Anything I eat needs to be cooked, or processed in some other germ-assassinating fashion (pasteurized juices, etc.).

They also advised us to have Luka take off his school clothes immediately on coming home, to quarantine the airborne viruses and bacteria that have fallen on his clothes during the day. Only paper towels in the bathroom and kitchen. Nobody should use the same bath towel twice, especially me. Dishwashing only with rubber gloves, for me, and maybe not at all so long as the PICC line is in (can't risk splashing dishwater on it).

With no white cells and low platelets, even a tiny cut could put me in the hospital if it gets infected. This'll be tough for me. I like to fix things and bang around with tools. We have an old house, so there's lots of fixing to do. I'm used to always having a few little cuts and scrapes on my hands; mostly I don't even notice them when they happen. Same thing with shaving cuts, so I think I'll only shave every other day. (If the beard gets too long between shaves, the risk of cuts actually goes up.)

These elaborate precautions are a far cry from Dr. A's rosy picture: No contact sports, he said, but you'll be able to do almost anything else. It all comes down to how much you want to minimize risk. Maybe nothing we do will stop me getting some secondary infection — but wouldn't it be better if I didn't? Days I spend in the hospital will grind even harder on Gabrielle and Luka, not to mention raising everybody's anxiety level.

And as everybody knows by now, the hospital is the last place you want to be when you're at extreme risk of infection. Every year, 2 million of the 35 million patients admitted to US hospitals catch something in the hospital that they didn't have when they went in there. More than 26,000 of them die of it.

CT scan

Banana or berry? The choices, the choices. We're talking about barium sulfate milkshakes here, 2 pints you have to drink before the CT scan. The barium shows up white on the CT scan; helps them avoid confusing your bowels with the rest of your guts.

CT is computed tomography, an X-ray technique for building a 3-dimensional image of your insides. (Same thing as CAT, which is just an older abbreviation meaning "computer-assisted tomography.") In my case, we're looking for spleen or lymph node enlargement, secondarily problems with the liver and other organs.

The scanner is a huge white donut, reminding me of the Stargate on the old TV series. You lie on a robotic bed with your arms above your head. The bed levitates you upward, then slides your body into the hole in the scanner. I half expect to emerge in another dimension.

Meanwhile, the tech hooks up the world's biggest hypodermic to my PICC line. It's a foot long and 2 inches in diameter, made of clear plastic. It dangles from a giant articulated steel arm attached to the ceiling, looking like a mad dentist's drill and reminding me way too much of Marathon Man. It's filled with a radiocontrast dye that will circulate rapidly through my blood.

The machine begins to whir ominously. I can see rotating things through a little window. The tech has left the room. Now the giant hypodermic begins to move, injecting me with the dye. The tech has warned me that I might feel a hot flash or other weird sensations. I do, but they're not very dramatic.

At the top of the machine are two cartoon faces. The open-mouthed face lights up green, the one with closed lips and puffed-out cheeks lights up yellow. As the faces illuminate, alternately, a pre-recorded voice intones breathe and hold your breath while the bed slides my body back and forth through the scanner. The bed stops just before ripping my up-reaching arms off on the edges of the doughnut.

2.5 hours in the waiting room, 5 minutes in the scanner and it's all over. 8 hours in the hospital (Friday); time to go home. I feel worn from within, and worried. This nightmare is getting more real.

80-90

Dr. A returned my phone call, but not until I was already in the hospital and out of range of cell signal. On the annexin-1 genetic marker for HCL, I’m not familiar with it, he says, but the hematopathologist is very confident that HCL is what you have.

This does not exactly boost my confidence in Dr. A. Remember, only a handful of articles on HCL come out every year. If you had a patient with a rare disease, and you only had to scan 10 or 15 articles to brush up on the latest research, wouldn’t you do that? Exactly how much does he know about hairy cell? The annexin-1 article is three years old, and it’s from The Lancet, the British equivalent of the New England Journal of Medicine. Not exactly junk science.

Here’s my fear. To cancer specialists, the odds in my case look really good. Hardly anything else gets an 80-90% remission rate. So to them, I’m in great shape, an easy case, not to worry. (Dr. A even talked about a “cure rate,” a phrase that should be banned from cancer wards.) They’ve got great stats. They’ve got a protocol.

But I’m not a statistic. To me, their 80-90% is like lining up 10 people in front of a firing squad, then announcing cheerfully Excellent news! We’re only going to shoot 2 of you! How would you react?

As for protocols: I’ve been thinking a lot about standards recently. (You can download my writing on this at my website.) Standards and protocols are technopolitical settlements: temporary, rough agreements about how to understand something, or do something, or make something. 120V AC, TCP/IP, HDTV, FM, AM, QWERTY, HTML — all great things, important steps toward valuable infrastructure. All also things about which people fought; argued; won and lost colossal economic, intellectual, and personal struggles.

If standards and protocols settle controversies, ipso facto they help people stop thinking about something, at least until they’re forced to think about it again. In science and technology studies, we call this black-boxing. It's very valuable. The ability to stop thinking about complicated choices by putting them into a black box that cranks out a decision automatically — we need this to get by, in an ultra-complicated world, with our incredibly limited bandwidth (attention) and low-reliability, kilobyte-size memories.

Standards and protocols are especially useful for things you don’t encounter very much. Such as a rare disease with a pretty good treatment plan. The hematopathologist is very confident — but the ANXA1 test is nearly 100 percent accurate, if the research is to be believed. Which sounds better to you: very confident, or 100 percent accurate?

When Dr. A sees me on Monday morning, I’ll be packing a stack of article abstracts. And my question will be how much Dr. A is willing to learn — from me.

PICC line

I reported to the hospital at 1 PM to get a PICC line (a peripherally inserted central catheter) placed in my arm. Long delay while the tech had trouble getting one into the patient in the bed next to mine. A quarter of that guy's face was black — not black as in race; black as in black and blue — so I knew he was in bad shape.

My turn. The tech found a vein with an ultrasound scanner, then threaded the catheter in, roughly 2 feet of thin purple tubing. A "power PICC," they call it around here; it can take more pressure than the standard lines, so they can use it with pumps. On the first try the thing went in OK, but then they X-rayed my chest to examine the placement and the last 2 centimeters had curled up, maybe gone into a small vein. So she had to pull it partway back out, then re-thread. Not much more pain than a blood draw; no sensation at all in the vein, only at the entry point.

The line starts on the underside of my left upper arm and runs all the way to the cavo-atrial junction. That's the venous return to the heart. The idea is to deliver drugs and other things, like the radiocontrast dye for my CT scan, directly to a place where there's a lot of rapidly moving blood. This dilutes the medication immediately and lessens the risk of damaging the vein with the drug. It also stops me having to get injections all the time. A little green plastic fixture sticks out of the dressing, for an IV or pump hookup. I wear an elastic sleeve to hold the whole contraption in place.

Instructions include no golf, bowling, weight lifting, bow and arrow. Admittedly none of these were in my plans this week, but it's good to be warned. Also no duct tape. Not sure I want to know about why that one's on the list.

Differential diagnosis 2

So I'm going into the hospital at 1 PM, to get my PICC line, learn how to use it, and have a CT scan. No food after 12 today.

Right now I'm waiting around for Dr. A to call. I'm still wondering about the precision of this diagnosis. I thought the CT scan would be part of the diagnosis, but I emailed Dr. F and she said no, it would only give them a baseline with which to follow the progress of the disease. I want to know that they're treating me for the right disease, since this one's features overlap with other B-cell lymphomas. (I hope they are right, since the prognosis for any other lymphoma is worse.)

Sounds like there is a near-100 percent accurate gene expression profiling test for HCL (Lancet 363, 2004, 1869-1871): the annexin-1 (ANXA1) marker. So I'm going to be sure Dr. A knows about this, and also ask him about the CT scan again. If they don't find any spleen enlargement, at this stage in my own knowledge that would raise a big red flag.

Here's a picture. If you read the caption you'll see why you need an expert to understand all this.

Choosing poison

Ok, now for the all-consuming thing: treatment.

In the last week, I've read dozens of websites and probably fifty medical journal articles. Remember, I study science and technology. I founded the UM Science, Technology & Society Program, which also covers medicine. I write about climate modeling, global data networks, and information infrastructure (see my website for more.) So I'm totally at home with ultra-technical subjects I haven't been trained in, and I am a very quick study.

One good thing about having a rare disease is that the number of journal publications is small. Pubmed dredges up only 30 or so articles on hairy cell leukemia since 2000. The National Cancer Institute website offers both a patient version and a health professional version; I read the latter (thanks Mom), which links to dozens of articles.

So. The first-line treatment for HCL is cladribine, aka 2-CdA. Doctors like it because it leads to complete or partial remission in 80-90% of HCL cases. It's administered over 7 days by a pump through a PICC line, a catheter that will run through an artery in my arm all the way to my heart. As chemotherapy drugs go, this one has limited side effects. No vomiting. My hair won't even fall out. (Damn!) On the other hand, about half of all patients get a high fever, caused by blood cell death as the drug does its work.

Two other drugs, pentostatin and alpha-interferon, also work against HCL. Alpha-interferon is out of fashion now. Pentostatin requires a much longer course of treatment — 3-6 months instead of one week — and its success rate is no better than cladribine. So they hold these in reserve in case the cladribine doesn't work.

Two other drugs are in clinical trials now. Rituximab, a monoclonal antibody used widely against B-cell lymphomas, doesn't work very well by itself against HCL, but it's being tried in combination with cladribine to eradicate the dregs of the disease. It kills off all your B-cells; then they grow back from scratch, as it were (if you're lucky). Early results on this combination look extremely good. BL22, a recombinant immunotoxin, consists of an "edited" bacterial toxin attached to a protein that binds to CD22 on the cell surface. It's basically a little mail-bomb that delivers itself, then kills the cell. A newer version of this idea, HA22 — not the deluxe clown shoe, in case you were wondering — is more than twice as toxic to the B-cells without increasing toxicity to the rest of the organism. It's in Phase I.

Every doctor I've talked to is pushing cladribine alone as a first-line treatment. Since my anemia is getting really bad, and the clinical trials of ritux and BL22 are in Texas and Maryland respectively, I don't seem to have much choice — for the present. It's not as simple as it sounds. In another post I'll write about the advice of the Patient from Hell, who's doing rituximab as maintenance therapy for his lymphoma.

Cordon sanitaire

Once I start chemo next Monday, for a month or so I'll have basically no immune system. With an absolute neutrophil count of 0.6, I'm already on the borderline between "mild risk" (0.5-1) and "severe risk" (0-0.5) for infection. They used to give blood transfusions automatically when people hit the level I reached weeks ago.

Viruses like colds aren't the main problem. Instead, the main enemy is already within my body. Bacteria, like those that live in my gut and on my skin. Fungus, like athlete's foot. And dormant viruses like herpes zoster, the chickenpox virus, that can reactivate and cause shingles. (I had chickenpox as a child, so I guess this is not unlikely.) During this period, if I get a fever of 100.5° or more, I go straight to the emergency room and I'm automatically admitted to the hospital.

So Gabrielle has been building a cordon sanitaire around me. All this week I haven't gone out in public much. When I do, I avoid contact (handshaking, close-up conversation) with people I meet. I carry little bottles of Purell in all my outerwear, and I wash my hands a lot. The timing rule, by the way, is to sing "Happy Birthday" twice while you wash. (Got that from my mom.) I'm humming "Six Blade Knife" instead.

As for visitors, we haven't had any since last week, especially kids. Luka won't be having play dates here until this is all over. (Whatever that means, when you have cancer.) With tradespeople and our house cleaner, we ask them to use Purell before entering. Gabrielle put a scary sign on the front door: "Immune compromised patient at risk — please use sanitizer and face wipes immediately on entering the house."

A year ago, when the possibility of another flu pandemic was in the news a lot, I read that hospital-grade surgical masks (plus hand washing) would be your best bet to avoid infection. I also read about how difficult it might be to buy those masks if a pandemic did start and there was a run on them. So I laid in a big box of NIOSH-95 masks. Now that seems prescient. Anybody who visits while I'm in chemo, or in the following weeks, will need to wear one. Gabrielle sent some to my family, who'll be coming for Christmas, to wear on the trip here, since airplanes are basically bottles of germs. I'm doubtful they'll achieve 100 percent coverage, but anything is probably better than nothing. Overkill, maybe, but we're not taking any chances.

Sunday night, the last night before chemo starts, I'm going to a concert at the Ark. I'll wear a mask to the show. I'll get weird looks, I'm sure, but that's a small price to pay for avoiding infection right now.

Army

Symptoms came back yesterday afternoon. Tried to take a long walk with Gabrielle; didn't get very far. It's hard to describe: not exactly weakness, not exactly fatigue, not exactly light-headed. Just an uneasy feeling that if I do too much I might not make it. The hemoglobin must be dropping.

Today, we did take a long walk — but uphills make my heart pound, immediately, and after even a couple of dozen stairs I grind to a complete halt.

I've had amazing luck. What if I hadn't gone in for an annual physical last month? Exactly when would I have started to feel this stuff as symptoms of something dangerous, instead of just flu, a cold, not enough sleep, too much to drink last night, whatever? I'm severely anemic now; I would've needed a blood transfusion right away if I'd waited much longer.

In the Cancer Center last week, I thought: there's an army fighting with me. Doctors, nurses, scientists, therapists, even the f-ing insurance company — hundreds, thousands of people working on the knowledge, the drugs, the antibodies, the cure. Not all-powerful, yet still mighty. None of it's about me personally, of course. But I'll choose to think of it that way.

Then there are the other armies: my parents, my extended family (including half a dozen doctors, even a Mayo Clinic oncologist), my friends with their stories of illness and recovery. Steve Schneider, my climatologist colleague and friend, who survived mantle cell lymphoma and then wrote The Patient From Hell. My friend Marcia's brothers, both hematologist/oncologists: I never met them, but they read my emails, looked over my lab reports, talked to me on the phone about the diagnosis, the treatment, clinical trials of new drugs.

Platoons, regiments, battalions. Generals and footsoldiers. Field medics and walking wounded. Weapons of war. Weapons of love.

Symptom

10.30.2007:

• Hemoglobin 10.2 (normal 14.0-17.3)
• Absolute neutrophil count: 0.6 (normal 1.4-7.5)

Last Friday, Nov. 9, I woke up, rolled out of bed. And staggered, just a bit. Then again, on the way to the bathroom. Not exactly dizzy, just a bit light-headed — and not really in control. Called the hospital. Hemoglobin went down a bit this week, I think she said 9.3 on Nov. 6, so I'm starting to feel it. (Here's a picture of hemoglobin.)

Lightheaded off and on all day. A strange sensation, not at all like being about to pass out or unable to think. I stayed clearheaded, didn't stagger or lose coordination again. Just a sort of airy feeling, like I'm dissolving at the edges.

Sensation was gone the next day, and hasn't returned. Actually I'm feeling good now. But climbing stairs or carrying heavy things makes my heart pound, hard, right away, and I need to sit down after just a few minutes.

Differential diagnosis

Two years ago, i.e. last Tuesday, November 6, we went back to the UM Cancer Center to get my diagnosis.

Blessedly, we didn't wait long.

In came Dr. F, Dr. A's assistant: 35-ish, smart, warm, pretty, cheerful without pretense. Under other circumstances it might be a pleasure to know her. The previous week she'd corkscrewed two fat needles deep into my upper left hipbone, a total of five times, digging out bone marrow samples.

About the bone marrow biopsy: she'd said it would feel like dental work. That was about right. First lidocaine, a snake of fire under the skin, then into the bone itself. Then a few minutes later the big needles. Heavy pressure; grinding noises via bone conduction. Dr. F said she'd do one core and one aspiration (sucking out fluid), but the aspiration didn't work. After three tries, she took a second core instead. On her way out, Gabrielle asked her whether the failed aspiration meant anything. Dr. F replied, "Sometimes it does mean something, but usually it's just a fluke." Sometimes it does mean something — I kept hearing that in my head all week, especially after I found the web pages about hairy cell. Fibrous bone marrow, difficult to aspirate. This can't be me. This isn't happening.

She shook my hand, sat down quickly, said "Take a deep breath, but don't worry." (Don't worry?!?) "It's not what you think. You have leukemia, but it's hairy cell leukemia. That's the good kind." The good kind?!? This is CANCER, you witch... "It's very treatable. It's not life-threatening in the short term. Cure rates are very high. 80, even 90 percent."

I don't remember much of what happened after that. Dr. A came in and confirmed what Dr. F had said. My first question — the basic question I build my life around — was how do you know? If you've watched a lot of House, TV's genius doctor from hell, you've heard about differential diagnosis: how doctors tell one disease from another, really really similar one. Not science; art. They do a lot of shooting in the dark, then turning the lights on to see what they hit. If they hit anything.

For HCL, they use several techniques. (I've probably got some of this wrong.) First there's flow cytometry, in which a laser scanner detects refraction patterns from the cell surfaces, looking for protein markers. Next there's visual inspection, under a microscope, looking at the cell morphology. That's where "hairy cell" (see the picture) gets its name: the cells have teeny little hairs, so they bind together into a fibrous mass. (That's why Dr. F couldn't get an aspirate.) Then there's clinical presentation: symptoms. Pancytopenia, i.e. low blood count in all cell lines, is typical, and that makes HCL unlike all other leukemias. Most people with HCL also have an enlarged spleen, often massively enlarged and painful, or enlarged lymph nodes, or both. Finally, certain sequences in the lymphocyte DNA can help determine which type they are. But ultimately it's the hematologist's judgment, based on experience. Better pray he's right.

Hairy cell leukemia (HCL) is rare. Very rare. Out of over 44,000 cases of leukemia diagnosed each year in the United States, only about 600 are HCL. That's about 1.2% of an already rare cancer.

And my case isn't normal. For one thing, my hairy little bastards are expressing protein CD10. This only happens in 10-15% of HCL. For another, even though virtually all HCL cases involve spleen enlargement, I don't have an enlarged spleen or lymph nodes, except for one very slightly enlarged node under my left arm. We're still waiting on the DNA tests that may (or may not) show other markers. On Friday night I'll have a CT scan, which might pick up spleen or lymph node enlargement the doctors can't feel with their hands. Yahoo. (What are you doing this Friday night?)

So I'm an atypical case of a rare disease, if they're even right about what I have. Maybe 60-80 people per year get exactly the version (cell protein expression) that I have. Maybe half that number get exactly my symptoms (or lack thereof). Maybe.

The statistics I hoped would save me — ...usually you get what most people get. Must be hepatitis C, like 3 million other Americans... — run against me now. It's hard to get enough data on typical HCL patients to build good statistics. So where do I stand?

I'm on my own out here, drifting in the current and looking for land.

Fire alarm

Three weeks ago, October 24, 2007, I had blood drawn for a routine physical exam. My doctor (I'll call her Dr. L; don't need any enemies in the medical world here) had already seen me for the office visit part of the exam.

Everything came up clean, except for one thing. For the last year or so I've had frequent nighttime leg cramps, but they've been getting worse since around May, happening every F-ing night, waking me up as much as 10-12 times. Stretching, massage, various electrolytes and other supplements: nothing worked. Believe me when I tell you that being jerked bolt upright by a leg cramp 10 times every night will not make you healthy, wealthy, or wise. Except for that, though, I felt normal, strong — in pretty good shape for a guy pushing 50 and managing life with an almost-5-year-old boy at home.

The next day, Oct. 25, Dr. L called me in the morning and said she wanted me to come back in for another blood test, right away. All my blood cell lines were well below normal: red cells, white cells, platelets, hemoglobin. I also had an elevated level of CK, a muscle enzyme that can be a marker of heart disease, but also of generalized muscle inflammation. Dr. L thought overexercise probably explained my high CK reading, so she ordered me to stop all heavy exercise.

One piece of good news: a cholesterol reading of only 150. Since my cholesterol has been over 200 for the last few years, 150 was quite a surprise — such a surprise that I just stared at the test results for a long time. Obviously, I concluded, they must have mixed up my blood with somebody else's. The repeat test would come back normal.

No. Not a mistake. The second test came back just like the first one. Now the fire alarm began to clang. Dr. L said the test meant my bone marrow wasn't producing enough new cells. She wouldn't speculate on why, but she kept saying she was "concerned." When a doctor says she's "concerned," you know something's wrong. You also know that it's probably worse than you think. Dr. L started calling me a lot, at night, even over the weekend, on her cellphone, to check that I didn't have a fever or feel weak or have any other symptoms. If I got a fever, she said, go to the ER right away. Stay away from crowds and public places. Start washing your hands all the time. She said she wanted me seen at the hematology clinic ASAP.

At first, this seemed impossible. Hematology said they were backlogged and it would be 4-6 weeks before I could be seen. Couldn't it wait, since I wasn't having symptoms? Dr. L kept calling and pushing, though, for which I will be forever grateful. Just doing her job, advocating for her patient with system, but not something a lot of doctors would have done. Sunday night, Oct. 28, she called on her cellphone to say I had an appointment the following morning.

OK, fine, still probably some sort of temporary thing. I'd get over it, like I always have. I felt normal.

Maybe I'll say a bit about me and the body I live in now. I'm 49, 5'10", 165 lbs., male, of mainly Welsh and English ancestry. I still have most of my hair. People always say I look young, even 10-15 years younger than I am. Except for a couple of years trying hard to feel cool while shaving with an unfiltered Camel drooping from the corner my mouth, smoke burning my eyes, I've always been physically active. I started long-distance running at 14. Aikido (I have a black belt), Iyengar yoga (over 25 years now), weightlifting, cycling, swimming, hiking... I do a lot, and I'm generally a healthy guy. I drink maybe a bit too much nice wine and dark roast coffee, but I eat well and I take my vitamins. I've always thought I'd live to 100. My basic philosophy is that exercise can cure anything.

Knowing that my blood counts were down so much, though, some suspicions began to creep in. I contracted a bad case of pneumonia last February, during a sabbatical in the Netherlands, and it took 6-8 weeks to recover. But I never got quite back to where I'd been before the pneumonia. Ever since, my pulse seemed to rise too fast, and stay too high, during exercise. For the first time ever, I had to take rest breaks while climbing even 2-3 flights of stairs. My muscles felt harder to stretch, and they stayed sorer longer after weightlifting or intense yoga sessions. And then there were the leg cramps, getting worse and not relieved by any of the usual treatments. I'd thought of all this as aging + stress. Now I was thinking low hemoglobin = poor oxygen transport.

Back to the story. Dr. L made me confess to every pill I was taking. These included multivitamins, vitamin C, co-enzyme Q-10, alpha lipoic acid, and a few other nutrients; calcium and magnesium for the leg cramps (useless, except as placebos). They also included L-tryptophan and 5-HTP for chronic insomnia.

Now, 15 years ago a contaminated batch of L-tryptophan from a Japanese manufacturer caused a rare blood disorder called eosinophilia myalgia syndrome (ESM) in an indeterminate number of people (between 3000 and 60,000, depending on whom you ask). In ESM, your body makes too many eosinophils (one kind of white blood cell), which then attack your nerves and muscles, causing pain and inflammation. Most people got over their ESM when they stopped taking the bad tryptophan, but not everyone did: some still suffer from it, and apparently — I now know — it's not entirely certain that the contaminant, rather than the tryptophan itself, caused the disease. The FDA took tryptophan off the market for more than a decade, but it's available again now with a better-monitored manufacturing process. Fortunately, my eosinophil count was on the low end of the normal range, and I don't have myalgia. So this wasn't it, but I worried about it anyway. I stopped taking everything, including alcohol and caffeine, right away. (Well, actually I backed down gradually on the coffee, but now I've been completely off it for a week.)

On Halloween morning, Oct. 29 — my son's 5th birthday — I went in for my hematology appointment at the University of Michigan hospital. Fortunately we'd held Luka's party the day before.

Hematology is in the UM Hospital's Cancer Center. Not a place I'd recommend visiting, if you can help it. The waiting room is filled with people who look pale and worn, some wearing surgical masks to guard against infection, others wearing wool caps to cover hairless heads. A faint smell of alcohol (the ubiquitous Purell) permeates the air. Worst is seeing the kids, some in wheelchairs, some with long scars across shaved skulls. The staff were dressed up in Halloween costumes and getting ready for a party with the kids. To me everything took on a wavy look, like precarious broadcast TV coming in from a faraway station.

Amazing: the genuine humanity of the staff in this clinic. Nobody pretends everything's fine here — what would be the point? — but they're cheerful and open anyway. They look you in the eye. They seem to know how dark it is inside you and they're determined to shine their living light in there, even for a few seconds.

On my two hours in the clinic and my bone marrow biopsy, I'll spare you the blow-by-blow —which exactly describes what it felt like — except for this bit. The hematologist, Dr. A, said it might just be that some virus, hepatitis B or C, or some unknown virus, had knocked out my immune system temporarily and it was taking some time to come back up. Nice theory; I would have been glad to leave at that moment. But, he said, acute leukemia is definitely a possibility. More or less exactly like that, he said it. I am preparing you for the possibility of bad news. And as he left us: you will not hear from me again, whatever the results, until our next appointment. I don't believe in telling people they have leukemia on the phone. So he left a door open for hope.

We hung in that doorway all week, but in my dark hours — and there were many of them — I knew what Dr. A's parting comment really meant. He was going to tell me I had leukemia.