Atypical Case of a Rare Disease

Reprieve

2011-05-16T10:29:00.001-04:00

Hemoglobin 14.1 (WHO normal 14.0-17.3)
Absolute neutrophil count 2.25 (normal 1.4-7.5)
Absolute lymphocyte count 0.34 (normal 0.8-5.0)
White blood cell count 2.93 (normal 4-10)
Red blood cell count 4.68 (normal 4.5-5.9)
Platelet count 158 (normal 150-450)

Went back to the NIH last week for another round of tests. Since flow cytometry on April 19 was slightly positive (0.15% HCL in the peripheral blood), everybody was expecting I'd be positive again on May 11 and I'd be starting 8 weeks of rituxan on May 18.

So I got started planning for it, and a whole new round of communication snafus with my UM doctor ensued. Too boring to tell here, but wow is this communication system messed up. It's exactly like a game of telephone: I speak to the nurse, who speaks to the physician's assistant, who maybe speaks to the doctor but usually doesn't. The PA speaks to the nurse, who speaks to me. The cycle takes two or three days if it works (and the callback rate is only about 70 percent). Almost every time, some important piece of information gets garbled or ignored. My final trick — on a doctor friend's advice — was to email the doctor directly. That produced an immediate (though quite irritated) response.

The problem is that there's no system for handling what I'm doing — a clinical trial at one institution in another state, with some of the treatment and followup here. They each have their routines, and they're not compatible. And exception handling is not the strong point.

But anyway — on to the good, no, the great — no, the amazing news. After all that, the flow cytometry on May 11 was negative for HCL. That's not the end of the story; it's the bone marrow biopsy that's definitive. Dr. Kreitman's email:

Paul:

Congratulations are in order in that your bone marrow is consistent with a complete remission! There are some B-cells which could be minimal residual HCL cells in the marrow but they are very few in number. Since the blood flow cytometry is negative and your counts are also consistent with complete remission, you would NOT get rituximab at this time and instead we would monitor your blood flow every 6 months.

Best wishes and congratulations!

So it's over, for now. I'm very relieved. I wasn't anticipating problems, but there's no denying that Rituxan is a very serious drug that can damage or kill you. The longer I can put off taking it, the better. No telling how long this remission will last, but in some people with complete remissions— at least 9 out of 350, in one long-term study — it never comes back. (Dozens of those 350 patients were lost to follow-up, so we don't know how many of them may also have never had a relapse.)

And it's summer. Though you wouldn't know it here in Ann Arbor, where it's 45 degrees and raining.

Positive

2011-05-02T11:32:00.000-04:00

My April 19 flow cytometry showed hairy cells again — 0.15% of my white blood cells, to be exact.

I would've been surprised if the flow had stayed negative, so this is expected. It means that I'll probably need to start rituxan after my 6-month followup visit to NIH on May 11. Depends on the results of the flow cytometry they do that day, plus the results of the bone marrow biopsy (I hate those...)

Not too worried about it. I've arranged to have all the treatments here at UM, so it won't be so stressful. According to Dr. K, the side effect levels are directly related to the disease burden, so I should have very few and it shouldn't have much effect on my everyday life.

Had a pneumonia scare a few weeks ago, but it turned out to be only a bad cold and bronchitis that hung on for a while. All gone now except an occasional cough. Feeling pretty good, especally now that spring is here.

Test, again

2011-03-16T19:58:00.003-04:00

Hemoglobin 14.7 (WHO normal 14.0-17.3)
Absolute neutrophil count 1.7 (normal 1.4-7.5)
Absolute lymphocyte count 0.4 (normal 0.8-5.0)
White blood cell count 3.3 (normal 4-10)
Red blood cell count 4.74 (normal 4.5-5.9)
Platelet count 200 (normal 150-450)

Excellent counts, except for the expected low lymphocytes. Doing well, feeling normal and strong.

Learned that they may not, in fact, treat me on May 11, since my current status is complete remission.

They'll do another flow cytometry on April 19. Flow results will come back around April 22; if I'm still at zero hairy cells in the peripheral blood, they won't start rituxan on May 11.

The default assumption is that hairy cells will reappear by April 19. But that's not a certainty. One of Dr. B's patients got a partial remission (like me) on the first round of treatment, went through another round of treatment 3 years later — and then had a complete remission lasting 12 years and still going! That guy was in the office earlier the same day as my appointment last week.

Dr. K confirmed that while it's rare to have a CR follow a PR, it's not unheard of.

And I wouldn't mind being unheard of.

Zero

2011-02-16T07:01:00.001-05:00

Hemoglobin 14.4 (WHO normal 14.0-17.3)
Absolute neutrophil count 1.7 (normal 1.4-7.5)
Absolute lymphocyte count 0.2 (normal 0.8-5.0)
White blood cell count 2.4 (normal 4-10)
Red blood cell count 4.47 (normal 4.5-5.9)
Platelet count 175 (normal 150-450)

These counts are actually OK. WBC is low, but mainly because of the expected low lymphocytes; neutrophils (more important) are normal. RBC is just barely subnormal, within the error bar. Hemoglobin is just fine, thanks, and correlates with how I'm feeling, i.e. healthy and normal. Incredibly, I've only had one cold this winter, and it lasted less than 2 days.

But today's real news — came in last night from my NIH research blood draw last week — is the flow cytometry:

The patient has a history of hairy cell leukemia and is being evaluated post therapy. The white blood cell count is 12.4 K/uL with 10 percent lymphocytes and 13.1 percent monocytes (02/09/2011). There is no definitive immunophenotypic evidence of hairy cell leukemia. The B-cells are polyclonal with no evidence of atypical antigen expression.

Yow!

This doesn't mean the HCL is gone. It could still be (and almost certainly is) hiding in the bone marrow, since the flow cytometry only measures the peripheral blood But it does mean the levels are quite low now. They may go down even further before I start rituxan in May. That's what we want — rituxan's good at cleaning up stragglers, but not so good at the frontal assault.

Undead

2011-01-07T06:16:00.004-05:00

Hemoglobin 12.7 (WHO normal 14.0-17.3)
Absolute neutrophil count 3.4 (normal 1.4-7.5)
Absolute lymphocyte count 0.3 (normal 0.8-5.0)
White blood cell count 4.6 (normal 4-10)
Red blood cell count 3.91 (normal 4.5-5.9)
Platelet count 183 (normal 150-450)

Almost a month since my last post (blog followers, sorry about that). I am undead. Not a zombie — just not dead.

All kinds of things have happened, of course. Went back to the NIH for a bone marrow biopsy and a cardiac MRI. Here's the BMB report:

Cellularity is 40-90%. Megakaryocytes are adequate. M:E ratio is 2:1. Myeloid and erythroid maturation are progressive. Immunohistochemical staining shows infiltrate of atypical lymphoid cells, which are positive for CD20, CD79a and TRAP and involve less than 10% of marrow. Aggregates over 25 B-cells are not present. CD3 staining shows mild increase in T-cells (CD20=CD3).

I won't try to translate all of this — main thing is that the percentage of bone marrow with HCL in it went from about 80% to 10% in a month. That's pretty good. In the peripheral blood (that's the circulating blood, as opposed to blood in tissues), HCL dropped from 15.3% to 0.03% on flow cytometry. It should continue to drop steadily over the next few months, until my next BMB in May just before I start rituxan treatments.

The cardiac MRI showed nothing significantly wrong with my heart.

Blood counts are looking pretty good. I had another test two days before the one listed above and it was even better; hemoglobin at 13.5 and lymphocytes at 0.4. Lymphocytes will probably stay in this subnormal range for the next 2 years, an expected effect of cladribine. Fortunately there do not seem to be any clinical consequences.

No more blood tests for a month! After well over 300 needle sticks since September, I am really looking forward to not being stabbed in the arm for a while.

As for how I’m feeling: most symptoms are gone or much diminished. No night sweats. No longer hearing my pulse most of the time. Occasional nighttime leg cramps and headaches, but they're very minor compared with a month ago. A chest x-ray last week showed that the pneumonia in my left lung has finally cleared, much to my relief since if it hadn't they were getting ready for another CT scan and antifungals.

The one thing nagging at me right now is a minor, hardly noticeable ache in the left side of my ribcage. I mentioned this to Dr. Washer when I saw her on Monday and it cost me 6.5 hours. If you're over 40, you cannot walk into a hospital and say "chest pain" without ending up in the ER being evaluated for a heart problem. But the cardiac enzyme tests were negative, and since I've already had a heart stress test and a cardiac MRI, both negative for heart problems, they decided it was probably musculoskeletal and released me. Probably it's either (a) a side effect of the pneumonia or (b) related to starting up yoga and weightlifting again after a couple of months of being mostly idle. I've been doing a lot of backbends, which really stretch out your ribcage, so it could be strained intercostal muscles or connective tissue. It's strangely persistent, though, and hard to localize. If it keeps up for another week or 10 days I'll have it checked out. I'm still on Bactrim and acyclovir, prophylactics for pneumonia and shingles, respectively.

Best thing is being back at work. I'm teaching two courses, finishing off the process of leading some job searches, writing some short pieces. Feeling good about all this, though it's stressful, and looking forward to truly complete recovery, which is probably still a few weeks away.

I/O

2010-12-09T14:38:00.002-05:00

Input

Blood: 4 pints
Saline: 4 liters
Heparin: 6 shots
Neupogen: 2.1 grams
Levaquin: 8.75 grams
Bactrim: 5 grams
Acyclovir: 16 grams
Zysor: 3x/day by IV, 8 days
Voriconazole:
Norco: 150 mg
Tylenol: ~5 grams
Ibuprofen: ~10 grams
Acupuncture needles: ~300
Radiation (3 chest x-rays, 2 CT scans): ~1000 millirems (average yearly background exposure is ~300 millirems)
Sestamibi (radioactive dye): 5 ml
Iodine (radioactive contrast agent): 500 ml solution
Nuclear magnetic resonance (MRI): ~3.5 hours
Ultrasound: ~1.5 hours

Output

Blood: ~750 ml (~150 tubes @ 5-10 ml per tube)
Urine: ~700 ml
Bone marrow (2 biopsies): ~100 ml aspirate, 2 solid cores
Lung tissue (bronchoscopy): trace amount
Hospital time: 8 days inpatient, 10 days outpatient (full time)
Blood draw appointments: 18
Acupuncture appointments: 10
Arranging logistics of NIH, HCL-related childcare, canceled events and trips, etc.: ~200 hours
Money: much more than I care to think about. The NIH clinical trial pays my airfare and part of my hotel bill, but we also bought air tickets for Gabrielle, my mother, and Luka for the first round at Bethesda. Then I came back for 3 nights. We also brought my mother to Ann Arbor to help out for 3 weeks.

Neutrophils!

2010-12-06T11:00:00.001-05:00

Hemoglobin 9.6 (WHO normal 14.0-17.3)
Absolute neutrophil count 9.3 (normal 1.4-7.5)
White blood cell count 9.8 (normal 4-10)
Red blood cell count 2.89 (normal 4.5-5.9)
Platelet count 216 (normal 150-450)

Wow. Thank you Neupogen. White blood count normal; neutrophils well above normal; platelets nicely in the middle of the range. Hoping that the blast of neutrophils will take out the pneumonia. Which it will only do if the pneumonia's bacterial in origin. Had a TB test this morning; we'll see what that brings.

Woke up this morning at 5:50 AM — first time in a while. Feeling almost normal. My main goal right now is not to overdo it so I end up back in the hospital. Waiting for the hemoglobin to come up more; that might take another two weeks.

So I'm done with two drugs: Levaquin ended today, and they took me off Neupogen. (We'll see whether the neutrophils can hold up on their own now; they should.) Down to Bactrim and acyclovir. Perhaps getting off the drugs will help me feel better, too.

Tomorrow, back to the NIH for a bone marrow biopsy and a cardiac MRI.

Home!

2010-12-03T11:49:00.012-05:00

Hemoglobin 9.5 (WHO normal 14.0-17.3)
Absolute neutrophil count 1.7 (normal 1.4-7.5)
White blood cell count 1.9 (normal 4-10)
Red blood cell count 2.93 (normal 4.5-5.9)
Platelet count 161 (normal 150-450)

Got out of the hospital on Tuesday evening. Neupogen — aka filgrastim or "granulocyte colony-stimulating factor (GCSF)" — worked like magic. I got a shot on Monday around 4 PM. By Tuesday morning's 8:30 AM blood draw, the neutrophil count had gone from a severely deficient 0.3 to a near-normal 1.1. The next day, after one more shot, neutrophils were normal. (Counts above are from Weds. morning.)

Waiting for results from today's test now. Neutrophils should be much higher. As Dr. Kreitman explained in an email:

The rapid increase with GCSF suggests that the bone marrow is hypocellular, the HCL cells mostly killed off, and the ANC precursors delayed in growing in possibly due to lack of endogenous local growth factors. We sometimes give GCSF just to initiate ANC growth into the vacated marrow and then stop when the ANC is > 1.5. In the setting of serious infection, though, we stop GCSF when the ANC is > 5. In my experience, it won’t take long to get there, perhaps tomorrow or the following day. We have seen it go to 30 within a few days.

Platelets are also normal now — and that's all me, not the GCSF. Other counts on the rise as well. Yahoo!

As for the pneumonia, they're still not sure what it is. My fabulous infectious disease doctor, Laraine Washer, consulted with several people, including a fungal specialist; they decided it was probably not fungal, but they still aren't sure. There's a thin possibility it's tuberculosis. (I spent a year in South Africa, where it's common.) Another test coming on that one next week. Some of the cultures they took can take up to 8 weeks to grow out, so it'll be a while, and we may never know. They're talking about doing another CT scan in a month. Hate the thought of all that radiation, but since this disease has been symptomless so far, it's the only real way to be sure it's improving.

The approach right now is wait and see if it gets better, or if I develop symptoms. (Right now, there's nothing except a very infrequent, unproductive cough.) They sent me home on a massive dose of Levaquin (antibiotic), and I'm still taking acyclovir (antiviral, prophylactic against shingles and herpes) and Bactrim (prophylactic against certain kinds of pneumonia). Maybe my rising neutrophils will take care of it on their own now.

Totally exhausted. It's a cliché to say this, but a hospital is pretty much the worst place in the world to heal. The two things people need most — sleep and good food — are nearly impossible to come by in there. The Guantanomo-like sleep deprivation really wore me down. At home, I slept 14 hours Tuesday night. Got up and went for blood tests, came home and slept another 2 hours — overkill, since then I had insomnia on Wednesday night, but Thursday was another 10-hour night. Yet I'm still wiped out.

Until the neutrophils come up a bit more, I have to inject myself with Neupogen every day. You grab some belly fat, squeeze it, punch the needle in and inject. It actually hardly hurts at all, but there's still a long moment just before the needle jab when I think am I really gonna do this?

Very weak, too. Lost 5 pounds in the hospital, though it's been quick to come back, what with finishing off the amazing dark chocolate birthday cake Luka and Susan D baked for me. Hemoglobin's coming up now, but still pretty low, so the best I can manage is a couple of 30-minute walks each day and some very, very gentle yoga. Can't tell you how good it feels to get upside down again, hanging from the inversion swing. Hospitals don't make that easy either.

Limbo

2010-11-29T10:10:00.006-05:00

Hemoglobin 8.6 (WHO normal 14.0-17.3)
Absolute neutrophil count 0.3 (normal 1.4-7.5)
White blood cell count 0.6 (normal 4-10)
Red blood cell count 2.68 (normal 4.5-5.9)
Platelet count 133 (normal 150-450)

Still stuck in the hospital. Probably they will not let me out until my neutrophils hit 0.6. So yesterday's 0.4 and today's 0.3 look like setbacks. Still, the average case starts seeing a neutrophil rebound on day 21, which will be tomorrow.

That's an average, so I might be slower, but I do think it's coming soon. I've felt stronger today and yesterday. And the real harbinger is the platelets at 133, up 23 points from just 2 days ago. The platelet concentration dropped for a while after my transfusion, but that's because they were diluted by the new red blood. (All the measures are X per unit of volume, so if you add one thing the concentration of everything else drops.) Since platelets are the first cell line to come back after chemo, this is a very good sign.

Finally got a nurse who really responded to my request for people to stay the f- out of my room in the middle of the night, so I slept OK between 11:30 and 6:40, and then went back to sleep until 9 even with a few further interruptions for blood draw, vitals, and pills.

On the pneumonia, still waiting for a final call. The infectious disease doc says the high-res CT shows a slight improvement over the NIH scan from 2.5 weeks ago. That's good news. The problem is that it's not definitive for fungal vs. bacterial pneumonia. The CT people lean heavily toward bacterial, but there is some nodularity that could indicate fungi. My ID doc is discussing today with a pneumonia specialist, but she thinks they will probably keep me on VFend (antifungal) for a while just in case.

This drug's nasty side effect is "visual disturbances." For me, that meant reading along on a black and white page and suddenly seeing everything highlighted in gray. It looked so much like MS Word highlighting that I actually held the page up to the light backwards to see if the highlighting was real. Then colors became lighter and somewhat attenuated, and everything shimmered slightly. Fortunately this only lasted about an hour. Another, much more fun effect appeared when going to sleep. In the half-awake state you reach on the way down, vivid color images materialized, shifted, grew. I soon realized I could control them, to a degree — distort them, morph them into new images, alter the colors. Interesting, and might make for some nice high-res color dreams. I once took mefloquine, an antimalarial, for 6 weeks and got this effect. Some of the coolest dreams of my life, much enhanced by having them in a tent under the stars of southern Madagascar.

They've backed off the VFend dose and we'll see how that goes.

Hoping to get some work done today, but I can feel a headache looming and think I'd better try to sleep some more first.

Lift team

2010-11-27T09:49:00.018-05:00

I live in a fat state. In 2009, over 70 percent of Michigan men and 56 percent of Michigan women were overweight or obese. We don't have the highest rates in the nation — that would be Louisiana and Mississippi — but we're right up there. When you think Michigan, think big chunky cars and big chunky people.

Those figures are probably even higher here in the hospital, not just among the patients but among the hospital staff. I've had over a dozen nurses, male and female, since I've been here and only two them even approached a normal body mass index. Practically everyone I see on the floor here is carrying a substantial spare tire. Or two, or three or four. One nurse (probably 5'5" and pushing 300 lbs) was so heavy she literally couldn't stand up for long; every time I saw her she was leaning or sitting on something. This woman didn't breathe. She panted.

One nurse told me that they used to train in how to lift patients, but in recent years they have started doing "lift teams" instead. Partly this is to protect the nurses' backs. But it's also about the fact that the patients have gotten too heavy for anyone to lift alone. And the fact that the nurses themselves are now too heavy and out of shape to lift people anyway.

I find the American obesity epidemic deeply frightening. We're living through what Diane Vaughan called the "normalization of deviance." Now that the majority of people are fat, it's coming to seem normal. Imagine: our children are growing up obese. They never, ever experience how it feels to live in a body that's not overburdened. I have relatives who are way overweight; if you dare to bring it up with them, they'll blame the body mass index tables (certainly approximative, but still a good guide). They just don't take into account a build like mine, they'll say. I don't see that much fat on me. (Those are quotes.) They literally don't see it.

It's infrastructural now. Japanese car manufacturers make bigger seats for the American car market. Clothing manufacturers change the standards for sizes so bigger people can continue to feel the same size. Plates and bowls get larger, so they can fit more food, so we eat more. (And in fact the size of the plate or bowl directly affects how much people eat before they feel full.) Tent-like clothing — vast hoodies, giant T-shirts, baggy pants — has become fashionable. And so on.

I've gained and lost 20+ pounds, so I know a little bit about how it feels to live in a body that's too heavy. It sucks. You're sluggish, you feel vaguely disgusting, you eat all the time because you can't tell the difference between feeling bad and being hungry. You can't tell when you're full. Your thighs rub together when you walk. Your activity level drops, which makes you fatter, which makes your activity level drop more. I've had all those experiences at only 20+ lbs above my best weight. I can't begin to imagine what it's like to be 100 lbs or more overweight. To hazard a guess, I'd think you just stop feeling your body very much.

I also know how hard it is to take off weight, and how easy it is to give up the effort. And I can't imagine how much harder that effort would be if you needed to lose not 20, but 70 or 90 or 150 pounds. Better to stop the problem at the source, in childhood — but overweight parents raise overweight children, and the cycle goes on.

If we could just get rid of calorie-laden "drinks," that'd be a start. Just before they try to supersize you, the fast-food people always ask: Do you want a drink with your meal? They're not asking if you'd like some water. They want to know if you'd like 200-300 calories' worth of corn syrup.

Nobody's overweight because they like it that way. This is a social disease, not an individual one. Worse, it's a disease that has been sold to us in the guise of instant happiness and convenience. Engineered flavors, designed to be addictive. Fabulous packaging. Advertising that links junk products to joy and bliss, much of it aimed directly at children.

As Joseph Stiglitz puts it, the American economic system privatizes gains, but socializes losses. Unfortunately, with respect to obesity it's the other way round. Your weight gain is delivered practically gratis by a society that's normalized it (massively abetted by the food industry and its relentless marketers), but when it comes to weight loss, you're on your own.

I've struggled with it too, for many years. Everything I've learned boils down to two simple principles: energy balance — the fundamental input-output equation — and frequent feedback. Things like the Atkins diet hold a few nuggets of useful knowledge (e.g. what kinds of foods reduce hunger most, thus helping you stick to a reduced calorie load). But no matter what anybody tells you and how badly you want to believe it, there's no magic combination of foods that will keep your weight down permanently if you don't pay attention to the only things that matter: honestly accounting for how many calories you take in and how many you put out (energy balance), and weighing yourself every day (feedback).

Recently the Tap&Track iPod app has been doing the job for me. It's an amazing tool, because unlike traditional diets where you limit your calorie count to a fixed number each day (thus starving on days where you do a lot), you keep a running daily count of calories (energy) in and exercise (energy) out. So if you want to eat more, you can burn more and buy yourself a cookie, or whatever. "Exercise" here includes all kinds of things, like cleaning the house, mowing the lawn, and raking leaves, as well as the usual sports.

Is there hope? Maybe. I've started seeing calorie counts displayed directly on menus (in Maryland, not in Michigan). There are rumblings about a federal soft drink tax as part of a solution to our budget crisis. Foodies continue to revive the joy of slow food, eaten slowly, for nourishment and deep sensual pleasure rather than an ersatz flavor fix.

But the problem is so damn big. I fear it's even bigger than we are. So to speak.

Fever

2010-11-26T21:58:00.006-05:00

Hemoglobin 8.8 (WHO normal 14.0-17.3)
Absolute neutrophil count 0.5 (normal 1.4-7.5)
White blood cell count 0.7 (normal 4-10)
Red blood cell count 2.75 (normal 4.5-5.9)
Platelet count 110 (normal 150-450)

It's late and I don't have much energy to write tonight. Long story short, went home for a lovely Thanksgiving dinner, but by the time it was over I was running a fever of 101 and had to go right back to the hospital. Been there ever since. Another hellish night. Fever at 3:30 AM, stayed up until 5:30 AM, nurses came in for drugs and blood tests starting at 6:30 AM. Zoned out all day as a result. Working my way through an entire season of Lie to Me on Netflix.

Gabrielle rescued the CD of my chest CT scan from Fedex. The hospital mail service was not open today, so they weren't planning to deliver it until Monday. She brought it in and we paged the docs. That got their attention fast, partly because Gabrielle leaned over the nurse's shoulder while she wrote the page and ordered her to label it "urgent" (respond in 5 min)— as opposed to "routine" (respond in 30 min) or FYI (no need to respond). The two attendings came in together less than half an hour later. They say the scan shows a substantial lung infiltrate, but it does not look like a typical fungal infection. They want to do another CT scan, this time a high-res one.

They've put me on an antifungal in case that's the cause of the pneumonia. Tomorrow's CT results should help them decide whether to keep me on that for a long time — 3 months (!!) — or for a much shorter time. Since antifungals can fry your liver and make you hallucinate, the tradeoff against the substantial radiation dose from the high-res CT seems worth it.

So as of today, my daily drug regime is:

Zosyn: antibiotic, one of the ones they hold in reserve for drug-resistant bacteria
Bactrim: antibiotic, prophylactic against certain strains of pneumonia
Acyclovir: antiviral
Vfend (voriconazole): antifungal
Heparin: to prevent blood clots from all the lying around in the hospital. Given by injection. Burns like liquid fire. They prefer to give you shots in the belly; more effective there, according to the nurse. Ow, ow, ow.
Norco: painkiller, hydrocodone plus acetaminophen. Very nice, but like all narcotics if you use it more than a few times in a row you start having to jack up the dose to get the same effect.

Neutrophils holding steady at 0.5. Slight upticks in the WBC, RBC, and hemoglobin. Hope those turn out to be trends.

More tomorrow.

Thanksgiving

2010-11-25T14:08:00.004-05:00

Hemoglobin 8.5 (WHO normal 14.0-17.3)
Absolute neutrophil count 0.5 (normal 1.4-7.5)
White blood cell count 0.6 (normal 4-10)
Red blood cell count 2.65 (normal 4.5-5.9)
Platelet count 105 (normal 150-450)

Last night far better than the first one. Antibiotic drip over at 10:30 PM, vitals at midnight, nothing else until the next antibiotic drip at 6:30 AM.

Today's picture looks more hopeful. Uptick in the neutrophils to 0.5 puts me on the border between "severe" and "moderate" neutropenia. If there's another uptick tomorrow, they might release me.

Meanwhile, the infectious disease doc has ordered another raft of tests. She's taking the possibility of fungal pneumonia seriously, though not saying it's likely yet. That would be a bear. Means a lung biopsy to confirm, another CT scan, then months of antifungal drugs. Another possibility is that it's a bacterial pneumonia that's already resolved but hasn't fully cleared (which can take 6 weeks). Comparison of my chest X-ray here with the NIH CAT scan should at least show whether the affected area is smaller, larger, or about the same as when that scan was taken on Nov. 8.

Good thing I might be getting out of here soon. Today, NY Times —

Study Finds No Progress in Safety at Hospitals: The study, conducted from 2002 to 2007 in 10 North Carolina hospitals, found that harm to patients was common and that the number of incidents did not decrease over time. The most common problems were complications from procedures or drugs and hospital-acquired infections. [The study] focused on North Carolina because its hospitals, compared with those in most states, have been more involved in programs to improve patient safety.
But instead of improvements, the researchers found a high rate of problems. About 18 percent of patients were harmed by medical care, some more than once, and 63.1 percent of the injuries were judged to be preventable. Most of the problems were temporary and treatable, but some were serious, and a few — 2.4 percent — caused or contributed to a patient’s death, the study found.

Stuck

2010-11-24T18:49:00.023-05:00

Hemoglobin 8.5 (WHO normal 14.0-17.3)
Absolute neutrophil count 0.4 (normal 1.4-7.5)
White blood cell count 0.5 (normal 4-10)
Red blood cell count 2.58 (normal 4.5-5.9)
Platelet count 105 (normal 150-450)

In the hospital. Stuck.

Woke up on Tuesday morning feeling awful, with a fever. 7:30 AM it was 99.5, 9:30 AM it was 101.5. I had been planning to call in about a transfusion anyway, and there's no better way to light a fire under that process than to report a fever of over 101. By 10:30 I was in the hospital getting a blood draw.

Weirdly, by 11:30 AM, when they first took my vitals, the fever was only about 99. Very soon my temp was in its normal 97.5 range, and it did not go up again, despite no medications of any kind until about 1:30 PM.

Hemoglobin was 7.6. They signed me up to get 2 more units of blood, but meanwhile, my fever pushed the urgent-care system over the line into a standard protocol for dealing with neutropenic fevers. Another chest x-ray showed the pneumonia is still there despite the Levaquin. That rang a lot of alarms. They filled me up with saline and gave me a giant IV dose of azithromycin. By about 3 PM I was admitted as an in-patient.

More insane delays and communication issues. The PA kept paging people who took hours to call back. The in-patient floors filled up and no one was available to transport me down there, though I could easily have walked. Result: I didn't get into the in-patient unit until about 5:30 PM, 7 hours after arrival. After that, incredibly, it still took 3 more hours to get the blood that had been ordered at 12:30 PM. The transfusion started at 8:30 PM. It lasted until 3:15 AM. During a transfusion, they take vital signs every 15-30 minutes, so this was a night from hell. Plus, after the transfusion, there was another antibiotic. Then at 6:00 in the morning a nurse arrived to take more blood — with a needle, from the other arm. You try going back to sleep after that, even on Vicodin.

Felt absolutely awful until about 11:30 AM, after many more interruptions. I don't think I got more than 1 hour of uninterrupted sleep that night. The transfusion helped a little, but it only raised my hemoglobin 1 point, so I still don't feel great. It'll be more narcotics tonight.

More docs, more case histories. They called in Infectious Diseases (ID), who got started on communicating with NIH. Damn good thing I got here Tuesday, instead of Weds, the day before Thanksgiving.

Upshot: in this situation the standard protocol for most leukemias/neutropenias is to keep the patient until her neutrophils go above 0.5. Mine have been at 0.4 for 2 weeks. They could go up tomorrow, but they might not hit 0.5 for another week or more. So I could be in here a long time. The ID people are waiting for fungal cultures from the NIH, which might take another 2.5 weeks to give final results. They're doing more cultures of their own, too, and the NIH is shipping us its CT scan of my lungs. Still, tomorrow's Thanksgiving and I doubt any big decisions will be taken, certainly not releasing me.

So I'll be here tomorrow. Eli's coming up from DC just for this weekend; hope it doesn't suck too badly for him. It does sound like I can get a "day pass" to go home for a few hours for Thanksgiving dinner. That'll be great. But I'll still have to come back.

Praying I can keep the nurses mostly out of my f-ing room tonight. At least I got the antibiotic schedule changed from 8 PM - 4 AM to 10 PM - 6 AM. I'll have to somehow fend off the others, with their insatiable zombie quest for my vital signs. Perhaps an outward-facing row of metal spikes backed by coils of razor wire.

Unfortunately, I can't lay land mines this close to the bed.

Communication

2010-11-22T08:26:00.004-05:00

Last few days a blur of work, naps, walks with my mom. Pneumonia seems to be clearing, though I'm still coughing just a little. Night sweats still happening, but less often; none the last 2 nights. I'm feeling a little weaker each day; probably the hemoglobin continues to drop. One flight of stairs now requires a 30-second pause to catch my breath. Standing up fast, even sitting up from lying down, makes my heart pound. As long as I stay still, I feel OK except for the ever-present headache.

Faxed Dr. B about the possibility of another blood transfusion this week. My next scheduled blood tests are on Wednesday, but if I try to get a transfusion that day — the day before Thanksgiving — it might be very slow or even impossible.

Maybe he'll get back to me, maybe not.

Communication has slightly improved since we developed this fax system: I fax him, try to keep it very brief, he dispenses orders to the nurses and one of them calls me back a few days later to confirm. On the other hand, when my neutrophil count dropped below 0.5 last week, the lab report noted that a lab tech had called the nurse and read her back the results to be sure she knew. Her name was on the note. Yet nobody called me.

From the point of view of education, it doesn't matter. I already know what sub-0.5 neutrophils mean, and what I need to do while they're this low. But it's still more than a little disturbing when a patient develops a very dangerous condition and the nurse does not check in. I still suspect that despite my doctor's reassurance, I'm seen as an irritating pest who's asking for more than he deserves. (It sure feels that way.) Or, they think I'm "being treated somewhere else," as they put it on one piece of correspondence I saw. Could also be the nurse herself. One of the two I've been seeing seems much less consistent about follow-through than the other (and she was the one named on the lab report.)

Getting in about 6 hours/day of work before I crash.

Platelets!

2010-11-17T10:40:00.011-05:00

Hemoglobin 8.6 (WHO normal 14.0-17.3)
Absolute neutrophil count 0.4 (normal 1.4-7.5)
White blood cell count 0.5 (normal 4-10)
Red blood cell count 2.58 (normal 4.5-5.9)
Platelet count 135 (normal 150-450)

All going as expected.

Platelets keep climbing — which is great, because it means the cladribine is working: they're first to recover. But everything else is down. Neutrophils now well into the danger zone at 0.4. In 2007, it took about 3 weeks for the neutrophils to come up to near normal again, but I'll be happy to get back above 0.5.

Could be heading for another transfusion next week, or even sooner, if hemoglobin gets below 8 again. Still, since yesterday afternoon I've felt remarkably normal. Slept until 7 AM both yesterday and today (first time in 6 weeks). That really helped, though the night sweats were bad last night. Been working, though not breaking any speed records.

Now, half past twelve, I'm slowing down and heading for a nap.

Roadkill

2010-11-15T22:00:00.001-05:00

Dr. K said to start taking antibiotics, even though nothing's turned up on the blood cultures or the bronchoscopy. So I got both Bactrim and Levaquin going in the morning. Apprehensive about the latter, but so far no obvious side effects, except maybe a contribution to the general state of fatigue. On the positive side, platelets are going up already, so now I can take ibuprofen, which I find a lot more effective than acetaminophen.

Slept most of the day, except for a one-hour walk.

Last day

2010-11-14T23:00:00.003-05:00

Hemoglobin 9.3 (WHO normal 14.0-17.3)
Absolute neutrophil count 0.5 (normal 1.4-7.5)
White blood cell count 0.62 (normal 4-10)
Red blood cell count 2.91 (normal 4.5-5.9)
Platelet count 130 (normal 150-450)

NIH, day 7. The last day. Got there at 9:15, did bloodwork and settled in for the 2-hour cladribine drip.

A while later another guy took the chair next to me: Greg, another patient in the Kreitman trial. First-timer, a bit younger than me, very vigorous-seeming, a data manager from Wisconsin. Like me, first diagnosis came at a blood test for a routine physical. He'd started feeling fatigued, needing naps, but didn’t think of them as symptoms. Scary blood counts — 0.35 absolute neutrophils.

Greg got the other trial arm, so he'd done rituxan on Wednesday. Said he shook and shivered, but they didn't start him until late at night so he'd managed to sleep through it without even telling the nurses, who could've doped him up with Demerol, until the next morning. We traded cards, maybe will stay in touch.

My counts came back. Hemoglobin back up to 9.3, thanks to the transfusion. But 0.5 on the neutrophils. Not as low as they got in 2007 (0.3); probably will get lower before they get higher. That means I'm in "severe neutropenia" territory now (0.0-0.5). Have to stay away from crowds and anyone who's sick. Also have to stop eating anything raw. No more sushi, no more rare-cooked meat. Fortunately there have been very few cases of infections reported in HCL patients, so I'll hope I don't become another one, but it does mean a long period — perhaps 3 weeks — when I won't be going out of the house much.

Finished up the drip, slept through about an hour of it. Left the hospital a little after noon, found lunch with my mom and joined Gabrielle and Luka at the National Zoo. Luka had been there for hours and was ready to leave before we even arrived, so except for a lion and a tiger I didn't really see anything.

Back to Bethesda, took a cab to the airport and flew home.

Blood culture

2010-11-13T23:00:00.004-05:00

Hemoglobin 6.8 (WHO normal 14.0-17.3)
Absolute neutrophil count 0.73 (normal 1.4-7.5)
White blood cell count 0.91 (normal 4-10)
Red blood cell count 2.06 (normal 4.5-5.9)
Platelet count 123 (normal 150-450)

NIH, day 6. By the time I got to the day hospital at 8:45 on Saturday morning, my hemoglobin was at 6.8, the lowest it's ever been (normal: 14.0). I felt bad, but not worse than before, so I was a bit surprised.

The nurse hooked me up to my cladribine drip and went off to see about a blood transfusion. Bronchoscopy results won't be final for a month, but most of the preliminary results were in — all negative. I asked the nurse to get in touch with Infectious Diseases, who had promised to call my cellphone but never did.

Long story short — I got a transfusion, 2 units. It took all afternoon to get the blood and drip it in.

Meanwhile, Infectious Diseases never called back. I think the nurse paged them at least 4 times.

Finally, at the very end of the day, she discovered a doctor's note in the files, written about 1 PM. So. Early in the week they took blood cultures, 2 little bottles with a nutrient gel. The idea is that if something grows in both bottles, that critter is probably living in your bloodstream. If something grows in one bottle, but not the other one, there's a possibility it's a false positive, a contaminant such as staph bacteria from the nurse's hands. (We're covered in them all the time, no matter how much we wash, so that wouldn't be a surprise.)

That's what happened to me. 68 hours after the cultures, something grew in 1 out of 2 bottles. The ID person consulted with Dr. Kreitman and they decided to do more blood cultures, said I didn't need to start antibiotics. Wait and see.

Left the hospital at 6:45, went to dinner with Eli, an old family friend now 19 and a college sophomore. Had to bow out of dinner early from fatigue. Took Luka back to the hotel. While I took a bath, he sat on the toilet seat and we discussed what superpowers we'd most like to have. He wants to be invisible. Me, I'd settle for unbreakable. (But it sure would be nice to be able to fly.)

Bronch

2010-11-12T22:00:00.007-05:00

Hemoglobin 8.1 (WHO normal 14.0-17.3)
Absolute neutrophil count 0.79 (normal 1.4-7.5)
White blood cell count 1.1 (normal 4-10)
Red blood cell count 2.48 (normal 4.5-5.9)
Platelet count 114 (normal 150-450)

NIH, day 5. Slept OK last night but woke up at 5:45 with a raging low-hemoglobin headache. In to the hospital at 8:30. Checked in at the day hospital, got an IV placed — big needle this time, so they could push sedatives for the bronch — and blood drawn for the daily CBC (complete blood count).

Thought we’d have to go check in to the pulmonary unit, but instead they came to us, right away. Pulmonary nurse took a history, went off to check me in. A few minutes later she came back with the pulmonologist, who went through the whole history again, probing for anything that might explain the lung infection. He was interested in my night sweats. (I’d chalked those up to HCL, but then remembered the first time I had them, during a case of pneumonia in the Netherlands in 2007.) Said he was sure it wasn’t cancer. Thought it was probably pneumonia but surprised that there were no symptoms. So maybe it’s something atypical.

Some of the bronch results will come back tomorrow, but the full results will take a month. Some of the possible critters take that long to reproduce. Anyway, we'll make a decision about antibiotics tomorrow, after the gram stain results come back.

Into the bronch room. The techs wired me up, fired up a nebulizer filled with lidocaine. I inhaled lidocaine mist for about 10 minutes. Stuff tastes unbelievably nasty, like a blend of aspirin and burning tires. Meanwhile, they got ready to snake the bronchoscope through my left nostril and into my lungs. I remember them injecting my IV with fentanyl. 7 seconds of bliss and happiness, then nothing but a blur of hands and instruments over my face. They told me later I’d coughed a lot — which can’t make scoping someone easier — so they loaded me up with extra fentanyl. Too bad I couldn’t enjoy it. Once it was over, they loaded me into a wheelchair and sent me back to the day hospital for my daily dose of cladribine.

Dr. Kreitman came by to check in. Proposed giving me a blood transfusion tomorrow, since my hemoglobin’s at 8.2 and I have to fly on Sunday. Good plan, I thought. I was going to try to power through it, but I remember the utter horror of sub-8 hemoglobin from last time. Wasn’t looking forward to that. Effects of the transfusion might last 3 weeks and keep me in a better range while the bone marrow recovers and starts to produce new blood.

Left the hospital around 3:45 PM and went to the hotel, where my mother and Luka were waiting for us, having arrived a couple of hours earlier. I took a nap until 7:15, then we all went to dinner at Redwood in downtown Bethesda — highly recommended. Eating in classy restaurants (on Thursday we tried Green Papaya, also very good) after chemo feels extremely weird. But we have to eat — so why not?

3 stories

2010-11-11T17:19:00.006-05:00

NIH, day 4. Easiest day yet. Nothing to do but chemo, and working out how to handle the antibiotic for whatever's going on in my left lung (see yesterday's post).

Showed up at the day hospital just before 10 AM. Very efficient and competent nurse placed another IV, drew some blood without me even noticing, plugged in the cladribine and off I went.

Meanwhile, the sweet but unconfident young med student from Infectious Diseases showed up before we even got around to raising the antibiotic issue. She asked about any new symptoms (none), listened to my lungs, heard nothing. I told her about my fears about Levaquin — tendon rupture, insomnia, leg cramps — and my conversation with Dr. K last night.

Story #1, from her: I'll consult with Dr. B, the attending. She fired off a text message. I think what he will say is that the side effects you describe are very rare and that it's the best option for you. Turns out that Infectious Diseases is a sort of rotation for her; she's trying out different specialties, mainly working on internal medicine. Didn't realize Infectious Diseases would be so interesting. Dr. B makes it all into a mystery. It's fascinating.

A few minutes later, med student returned with NCI Fellow from yesterday. (All without me saying a word.)

Story #2: this time, no hedging. I've prescribed Levaquin throughout my residency. Never had anyone complain about insomnia. The tendon rupture issue is real, but it's more common among children. I told her about the lawsuit against the company that makes Levaquin; 400+ people suing over tendon ruptures over a 10-yr period. But how many people took the drug without side effects? I've never seen a tendon rupture.

The other problem, she said, is that you can take Levaquin orally, but all the other broad-spectrum pneumonia options involve an IV for 7 days. I could see right away that would be a major headache. You can always try the Levaquin, and if you start to develop side effects, you can stop right away and we'll do something else. That made sense to me. I told her I'd try it. Said I'd take it during the day instead of at night, at least hope to avoid the insomnia that way.

An hour later, Dr. B came in, along with med student and NCI Fellow, and a third woman who said nothing and was never introduced. He listened to my lungs for a bit, then motioned med student over to listen while he held the stethoscope in position. This time, she heard it: crackling in the left lung.

Dr. B's from Madrid. Beautiful accent, great manner, great communication. And a really different story. We loved this guy.

Story #3, from Dr. B: first, he confirmed NCI Fellow's line on Levaquin. Fine drug, widely prescribed, and the other options are all combinations of oral pills and IV delivery. But next: We know something's happening in your lung. We don't know what it is. We don't know how long it's been there, how it's changed. Has it been there 10 days, or a month? Is it getting better, or worse? You have no symptoms, no fever, no cough. I nodded and confirmed. So we don't need to treat you right this minute. It's not like you're coughing up blood.

What could be causing this fuzzy spot on the CT scan? It isn't cancer. That shows up as a dark mass, not a fuzzy infiltrate. But it might not be pneumonia either. It's possible that because you're immuno-compromised, some of the bacteria from your mouth — bacteria that are normal there — have gotten into your lungs where they don't belong.

So I say: you're already scheduled for a bronch tomorrow. There's no need to treat right away. Why don't we do the bronch and find out exactly what it is? If it's mouth bacteria, we have a lot of other options for antibiotics.

Now that's a good idea. I'll take it, I said.

The interesting thing here: the three different levels of experience, and the three stories they produced.

Story #1, inexperienced apprentice: defer to someone else's judgment, but predict that it'll be an attempt to persuade me to stick to the original plan. Don't mention other possibilities (maybe don't know them). Call for backup.
Story #2, real but limited experience, journeywoman: describe the other available options and explain why Levaquin makes the most sense. Reassure the patient about the unlikelihood of side effects, but acknowledge their reality. Offer one option: try it and see.
Story #3, deep experience, guildmaster: Look again at the entire situation. Reopen all avenues. Does the patient really need this antibiotic, right now? Is there another way to think about what's going on? Realize that there's an unexplored, but simple and obvious possibility: wait, find out more about the condition, use that knowledge to decide what's best.

There's a life lesson in that one, I think.

Out of the hospital by 12:30. Wiped out, back to the hotel for a 1.5 hour nap. Then a long slow walk.

This go-round, without the anxiety of a PICC line (which made me feel like I really shouldn't move much, even though I think the intent of the portable pump was the opposite), I'm focusing on keeping somewhat active throughout the treatment. Circulating the cladribine can't hurt. I want poison to reach every cell of bone marrow. Light exercise will certainly do that better than lying on my back in a hotel room.

Chemo, again

2010-11-10T23:00:00.005-05:00

Hemoglobin 8.8 (WHO normal 14.0-17.3)
Absolute neutrophil count 0.82 (normal 1.4-7.5)
White blood cell count 1.23 (normal 4-10)
Red blood cell count 2.67 (normal 4.5-5.9)
Platelet count 121 (normal 150-450)

NIH, day 3. Counts picked up a bit from Monday. Feeling tired but OK.

Gabrielle arrived around 10:30 PM Tuesday, after a delayed flight. So great, so reassuring and important, to have her with me. But the consequence was late bedtime, early wakeup; I mostly don't make it past 6 AM any more. Today it was more like 5:10.

I called Rita, Dr. Kreitman's nurse, on the way to the NIH. Plan for the day: get blood work, see an infectious disease specialist around 9 AM. Induced sputum test at noon, and then, if I'm cleared for treatment, meetings with the clinic staff and randomization. Start treatment at last.

Well that's a long day. In addition to the usual CBC, the blood lab took some "blood cultures," trying to grow the nasty little things that might be hanging around in my lungs without my permission.

Nurse who took my vitals informed me that NIH stands for "Not In a Hurry." Not my experience so far; everything I've done the last two days ran more or less on time, with no more than a 20-minute wait for anything. But now I'm in the clinic, on the 12th floor. Things are definitely different at this altitude.

The infectious disease people showed up a little after 9, as promised. First got examined by a very nice and well-intentioned — but also clearly very green (tentative, overly wordy, unsure of herself 25-year-old) — med student. She took my medical history, asked a lot of questions, probed for anything besides the June bronchitis episode I described in yesterday's post. Nothing else seemed to explain the apparent pneumonia in my left lung. Which I do not feel, at all, in any way. Was cycling a lot all summer, even into early October, no coughing or crackling or anything like that.

I've had a lot of bronchial infections in my life. They started in my 20s when I hadn't smoked anything for years and led a mainly vegetarian lifestyle of monklike purity, at least on the bodily front. There've been years with 2 or 3 of these monsters, coughing my lungs out for weeks. Probably I've had at least one infection like this in 17 or 18 of the last 25 years. Usually they come on at the tail end of an ordinary cold; it descends into the lungs and refuses to come out.

So I'm used to the drill. Take a lot of expectorant, don't do anything to irritate the lungs, eat a lot of fruits and vegetables, drink a lot of water. Usually that regime works pretty well and I'm not feeling sick after 10 days, though often the cough will linger for up to 4-6 weeks.

Next came the NCI Fellow, rough equivalent of a resident in an academic hospital. What a difference in confidence and style. Maybe 5-8 years older than the student, but so much more self-assured. She too couldn't find anything, in either the history or the physical exam, to suggest another explanation than the June bronchitis/pneumonia. Heard nothing in my lungs. But, she said, we have this finding. The CT scan and the chest X-ray both clearly show infiltrate in your left lung. They aren't diagnostic, but we're pretty sure.

What to do? How to tell exactly what's causing the fuzzy patch on the CT? So we'll do an induced sputum test, but we may also want to do a bronch. That's a bronchoscopy; they stick a camera into your lungs and look around in there. That does not sound like a picnic in the park. The fellow and the student consulted with the attending by phone. He agreed with the fellow's plan to bronch me, and they also decided to start treating me for "community-acquired" pneumonia. That means the kind of pneumonia you get outside a hospital, not the ultra-dangerous antibiotic-resistant supergerms lurking in the lungs of inpatients.

We waited around the clinic until noon. Talked to Mike, the first hairy cell patient I've ever actually met, a long-haired woodworker from Redding CA with a gentle presence and a lovely smile. Mike's had HCL for 21 years. Had his spleen removed; that bought him 5. Then he had cladribine and bought another 10. Second round of cladribine bought 5 more, but the third only bought 3. His doctors wouldn't let him do cladribine again. Mike tried to get into the Kreitman trial, but there was a months-long delay in responding. By then his docs had decided to give him rituxan straight up, 8 shots. His counts went up for 3 months, then dropped right down again. 2 months of treatment, $42,000 worth of Genentech-donated drugs, for 3 months of recovery. His platelets are around 35, so he can't work; Mike could bleed to death from a shaving cut. Finally he got into a Kreitman NCI trial, a different one from mine, testing bendamustine plus rituxan. Small trial so far, but 5 out of 6 patients already achieved complete remission on this plan.

Good luck, Mike. Live long and prosper.

Noon, the induced sputum test. You inhale saline mist for 20 minutes in hopes of irritating your lungs enough to cough something up. I went the full 20 minutes, with Gabrielle reading The Collectors of Lost Souls out loud in lieu of horrible daytime TV. Nothing. Not a single real cough.

Lunch, back to the clinic at 1:30, wait. Around 2:45 it was our turn. Another NCI Fellow, confirmation of everything in my record. Then Kreitman and Rita came in too. The moment of truth. Drum roll, as Rita said. They decided they can start treatment despite the pneumonia, but they want the bronch. That'll happen on Friday. Signed consent forms and Rita went off to randomize me. It's a blind process; they have no control over how it happens. They don't even know how it happens. They send off a form to an office somewhere else in the hospital, wait half an hour or so, and the forms come back with a randomization.

Over an hour later Rita came back. Conclusion: I'm in the delayed-rituxan arm. So I get cladribine now, and in 6 months, if I still have detectable disease, I get 8 weeks of rituxan. (Since my remission on the first course of cladribine was only partial, chances are near 100 percent that I'll get the rituxan.) It's physically plausible that this is the best arm to be in. Hairy cells continue to die off until the 6-month mark. Since the rituxan can't penetrate clumps of hairy cells, it may be best to hit them when their numbers are smallest. Wipe out the stragglers.

Off to the day hospital (closes at 8 PM, doesn't admit inpatients.) We were alone. A very kind but also very incompetent nurse tried to hook up an IV, couldn't push saline through it. Eventually called another nurse who messed with the plastic connectors. Sudden spurt of blood and saline; the first nurse shrieked, then immediately apologized — a really unprofessional reaction. The second nurse, clearly much more capable, removed the IV and placed another one in the other arm, and off I went, cladribine at last.

Rita stopped by around 7:15 on her way home. We won't see her again on this trip, since she's off for the next 4 days. Rita's great; manages an amazing number of details and doesn't ever seem to lose her cool. Last thing she said, on her way out the door: Don't hesitate to call Dr. Kreitman. He really is 24/7.

We finally left the hospital around 8:45 PM. We'd been there more than 12 hours.

Last item of the day. Around 10 PM I got ready to take Levaquin, the antibiotic I'd been prescribed for pneumonia. But the warning labels genuinely scared me: possible tendon rupture (including up to 3 months after taking the drug), insomnia, leg cramps, lots of others. Since I have insomnia and leg cramps already, and since physical exercise is crucial to my sanity, this began to sound like something I really did not want to do.

So I called Dr. Kreitman, at 10:30 PM. Apologized profusely for the late call, but he didn't balk at all. Said he'd never seen these side effects and that Levaquin was very commonly prescribed. But he also said he didn't think treating the pneumonia was so urgent. Talk to the infectious disease people tomorrow and see what they say. Maybe they can find a substitute, like ciprofloxacin. That's what I'll do.

Amazing. He really is 24/7.

Pneumonia?

2010-11-09T22:00:00.001-05:00

NIH, day 2. Not so packed as yesterday. First appointment at 8:30 AM, though that didn't help me sleep past 6. Morning tests required fasting again.

They wanted a 24-hour urine test, so I had to drag 4 liters of the stuff around in an expandable brown plastic container. Turned it in at the phlebotomy lab and went off to my first test, an abdominal ultrasound, measuring all the organs and taking sectional views of them, looking for badness.

Onward. Chest X-ray: stand up, face the white board, grab the handles, shoot. Turn sideways, shoot again, you're done. So far so good, but the next test, a CT scan of everything from my neck to my pelvis, was more elaborate.

9:40 AM, drink half of a banana-flavored barium sulfate milkshake. 20 minutes later, drink the rest. 40 minutes after that, into the scanner room in my blue paper hospital garb. They placed a new IV line, then hooked me up to the world's biggest hypodermic, filled with an iodine solution. Into the scanner, laid out like a fish ready for cleaning. The platform pulls you into the scanner, then out again, while the giant X-ray wheel whirs around you. The hypodermic suddenly compresses, fast. The iodine feels cold and metallic on its way in. Tried not to think about all that radiation. The docs call it "taking a picture," which downplays the risk factor way too much — most people's main "optional" exposure to radiation comes from medicine. CT scans give you 100 times the radiation of a chest X-ray, which is already way more than you should take lightly. Think about all those dental X-rays going right through your head. This is not a joke.

Around 11:30, after the CT scan, I finally got to eat. Didn't feel as awful as yesterday since I'd had more sleep and it was earlier, but I was still really hungry.

Last appointment of the day: a pulmonary function test. I huffed and puffed into a little plastic mouthpiece that measured exhaust gases and volume of air expelled, stuff like that. In between huffs, the tech used her cell to call out for some danishes she needed for the next morning. This took several calls, during some of which she directed me to puff, hold, expel by raising her eyebrows and gesturing dramatically with her cellphone, looking like the conductress of a medical orchestra. By the time I left I knew her breakfast menu by heart.

Everything was over by 1 PM. Wiped out, I went back to the hotel, did some email while I waited for the room to be cleaned, fell asleep until 4:30. Took a slow walk around downtown Bethesda.

Around 7 PM, Dr. Kreitman called my cell. Are you still at the NIH, by any chance? They'd found a spot on my chest X-ray that looked like pneumonia. No, I was about to have dinner. But I can come back up there if you want. Back in June, I had a bout of bronchitis — or pneumonia, as it now seems. Coughed a lot for a week or so, I took Mucinex (guaifanesin, an expectorant) religiously for a while, and the cough very gradually slacked off. But that cough never disappeared altogether. This needs investigating. They can't treat somebody with an active pneumonia. When were you scheduled to come in tomorrow? Noon, I told him. That's way too late. I think you'd better get here at 8AM. We'll get an infectious disease specialist to look at this. Maybe do an induced sputum test. OK, I said. We'll be there.

Treatment starts tomorrow. Yow. I am really ready to start shooting hairies.

NIH: testing

2010-11-09T06:16:00.014-05:00

Hemoglobin 8.5 (WHO normal 14.0-17.3)
Absolute neutrophil count 0.65 (normal 1.4-7.5)
White blood cell count 1.4 (normal 4-10)
Red blood cell count 2.65 (normal 4.5-5.9)
Platelet count 111 (normal 150-450)

These blood counts suck. And I feel like roadkill.

NIH, day 1. Got up at 5:30 AM and arrived at the NIH hospital at 6:10 (thank the gods for the time change on Sunday, which lessened the pain). Security screening. Then on to phlebotomy. (No, not lobotomy, though I'm starting to feel as if I'd had one). I was the tech’s first appointment of the morning, so I got to watch her struggle with her computer. Try, reboot, fail, try again, fail, reboot, success. So much for health informatics.

My record showed screen after screen of tests. Her labelmaker spit out 23 labels, one for each tube of blood she drew. Amazing skill she had, holding on to the IV with one hand while putting filled tubes into the rack and grabbing new ones two at a time. Seemed like she had three hands.

Next, an EKG. Hook up a few electrodes, lie back on the bed, turn on the machine, bam, you’re done. They only needed ten heartbeats’ worth of recording.

On to the dreaded sestamibi stress test. They inject you with a radioactive dye, then scan your heart. You lie inside a gamma camera for 20 minutes, with your arms raised over your head. (Yes, your arms go completely to sleep.) You wait an hour and a half, go back. Now they hook you up to a heart monitor, about a dozen electrodes. Some shaving of chest hair involved in this one. Once you’re hooked up, they record a resting baseline. Then you get on a treadmill. Here's a lousy drawing that shows the setup.

It starts low and slow, but they speed it up and raise the incline every 2-3 minutes until you are really sailing along, striding uphill at 3.5 mph. After about 8 minutes I exceeded the 143 bpm threshold they were shooting for — 85 percent of the theoretical maximum for my age — but they kept me going 3 more minutes until my heart rate hit 156 and I was sweating and breathing hard. The doctor and two techs cleverly distracted me from the effort with small talk.

By now it was 12:30 and I’d had nothing but water since 9PM the previous night. Finally I could eat. Half a liter of glucose by IV, plus a bit of ginger ale and some graham crackers. Wait another half hour, then back to the gamma camera for another 20 minute scan.

Off to the salad bar, which I regretted, since some of the vegetables turned out to have been frozen. But it was good to finally eat.

Next, echocardiogram — basically a sonogram of the heart. This one was cool, since I could see the images on the tech’s screen as he slid the ultrasound wand around on my chest. The valves looked like two boys break-dancing.

Met Dr. Kreitman and we went over my medical records, which he had a hard time sifting through because UM delivered them in order by document type, rather than by date.

Finally the last test, an MRI of the spine. Mike, the tech, wedged my head into the headrest with some pieces of foam rubber so I couldn’t move my neck at all. Then I had to lie completely still for 45 minutes. I forgot to take my silver rings off and they began vibrating like crazy when the scan started, so I pressed the little rubber bulb. Turns out that was an over-reaction; it’s really just for emergencies since you can simply talk and the tech will hear you.

Out of the hospital at 6 PM. I had been there almost 12 hours. Back to the hotel, Argentine steak dinner, bed.

Europe

2010-11-05T08:04:00.003-04:00

Countdown: 3 days to the NCI clinical trial.

Spent last week in France and Belgium. Gave 3 talks, 2 in French and one in English with bilingual slides. Good audiences: in Paris, at the Ecole Normale Supérieure, about 70-80 people, probably 1/4 faculty and the rest students. At the Centre Koyré, also in Paris, a combined seminar of about 25. In Brussels, at a colloqium on "Climate Controversies," around 120, perhaps more. Great reception, lots of interesting colleagues.

Mastering French and worming my way into French intellectual culture has been something of a life project for me. I took Latin and Spanish in school, but I didn't start French until my first year in college. Spent a semester in Paris in 1978, then went back for a year in 1980-81 and worked for an American consulting firm. The job required me to interview numerous French tire tycoons (yes, there are tire tycoons). Since then I've probably spent another 1.5-2 years altogether in France and francophone Africa. Still, they say it takes 10,000 hours of practice to master any expert skill, and I'm still learning. The hardest thing is technical vocabulary, of which there's quite a bit in the climate science world.

From the HCL point of view, the trip was tough but OK. Wore an N-95 face mask on both plane flights. That's a lot of breathing effort, sucking air through a piece of paper. At least these masks had exhaust valves, so my glasses didn't fog up and the exhale wasn't quite as much work as the inhale. Had a 1st-class seat on the outbound night flight, so I slept as well as one can on a plane. Got in probably 4-4.5 hours of good sleep and hence was able to deliver my talk with reasonable coherence at 6 PM on the day of arrival. The return flight was economy class, but miraculously I ended up in a middle row with 2 empty seats beside me, so I could lie down and sleep, which I did, twice.

Didn't do much in Paris or Brussels besides tweak Powerpoints, give talks, and rest. I did get to walk around for an hour late the first night. Got pretty wiped out during the colloquium in Brussels, but it was so interesting I just couldn't leave.

Anyway, very glad I got the chance to do this. I'd been invited back in March, and I really didn't want to have to back out. My blood counts held up, just barely, and by means of obsessive handwashing (and the face masks) I managed not to get sick.

I'm really needing the treatment now. Drenching night sweats almost every night; I've started wearing a terry-cloth bathrobe to bed to absorb it all. Near-perpetual headache. Sleep's a lost cause.

Still having a hell of a time getting responses from my doctor. I sent him a stern fax yesterday, expressing my frustration and asking him to call me before I leave town on Sunday. We'll see.

Logistics

2010-10-21T09:12:00.004-04:00

Countdown: 19 days to the NCI clinical trial.

Hemoglobin 10.5 (WHO normal 14.0-17.3)
Absolute neutrophil count 0.8 (normal 1.4-7.5)
White blood cell count 1.7 (normal 4-10)
Red blood cell count 3.06 (normal 4.5-5.9)
Platelet count 103 (normal 150-450)

After another few prods, the two docs finally talked. Not sure what happened there, but at least now it seems to be settled that I can do the NCI clinical trial and get most of the treatment here, at the University of Michigan Hospital.

Lots of irritating stumbling blocks. Dr. B canceled an appointment, but nobody told me until I arrived at the hospital. Explanation (or excuse): we thought you were being treated elsewhere. Not true — I was still deciding, and the decision hung on whether my doc here would continue to treat me like his own patient. Felt very much like a door being slammed in my face.

More unreturned phone calls. Finally on Tuesday I went in for another blood test. I'm Dr. B's new nurse, said the very nice person who brought out the lab results. I understand you're being treated elsewhere. So I went through the whole story again, for the 10th time: no, not yet. Want to do it, but first, need to know that the arrangements for drugs and infusions will work. The docs have to talk. Above all, I don't want to do it if Dr. B won't keep treating me here.

The other nurse was OK, but this one seemed to genuinely listen. She made a lot of notes and went off immediately to contact with Dr. B. 3 hours later, the docs had talked. The path starts to look smoother.

Now, lots and lots of logistics. Flying to DC, staying there Nov. 8-14, getting all the cladribine infusions down there since our infusion center isn't open on Sundays. My partner's coming down on Tuesday night, staying the rest of the week so she can be there for the treatments. Meanwhile my amazing-but-true mother's coming up here to take care of Luka the ninja warrior, who turns 8 next week. On Friday, she and Luka will fly to DC as well; Luka can see the White House and the Air & Space Museum, and, oh yeah, his dad.

Some good days, some bad days. Yesterday I made it through most of a 7-hour faculty retreat, but couldn't quite manage the last hour. Went home and took a nap. Headaches hanging around, stormclouds on the horizon; not always active, but always possible.

Cracks

2010-10-13T08:58:00.006-04:00

Countdown: 26 days to the NCI clinical trial.

Yesterday I spent 5 hours on arrangements. And barely got started.

Logistics of the trip. How to handle child care while I'm away and after my immune system crashes, probably a week or two after treatment. When my partner will and won't be there. When, and how long, we're going to need help from my mother and others.
Due diligence with the NCI nurse. Who pays for the trial? Any vested interests of the doctor in Genentech (maker of Rituxan)? What's the drug company's role? How long will the study continue? What happens if the trial leader dies or moves on? What happens if I leave the country for a year or more? Etc. etc.
Getting the UM medical records system to release my records to the NCI
And on and on and on.

Unbelievable how much time it takes.

Cracks between the systems. As I said in my last post, getting into this trial puts me at the intersection of three huge, complex organizations: the UM hospital, the National Cancer Institute, and the insurance company.
Tests and some treatment happens at the NCI; that's all fine, I'm in their system and I expect it will work OK.

No matter what, I'll get 5 days of cladribine infusions. The first 2 at the NCI, then I come home. The next 3, here at UM. The first infusion is scheduled for a Wednesday, which means the last one has to happen on a Sunday.

But the UM infusion center isn't open on Sunday. Does that matter? Could I do it Monday instead? Answer: yes, it matters for the clinical trial, because the trial needs the treatment conditions to be as close to identical as possible. Homework: communicate with Dr. B's nurse to see whether there's some way to get an infusion here on Sunday; if not, reschedule the treatments. Maybe stay at the NCI for all 5 infusions, but then we're talking 8 days instead of 5 at the NCI, which has a big effect on my family, since my partner wants to be near me for all 5 treatments.

Once I'm back here, there'll be more infusions no matter what. The NCI supplies the drugs, but UM is supposed to administer them. How do they get here? Who transports and delivers them? Most important for me: who pays for the infusion services, which might cost up to $1500-2000 a pop? If this isn't set up correctly it won't be covered by insurance and I'll be stuck with the bill.

I'll have many, many blood tests — some done here in our lab, some sending blood to Maryland like I did the other day. Who draws the blood? Who pays? How far off the map will the system let me go? How much irregularity and extra time will my doctor here tolerate?

This is at least a half-time job right now.

Holding pattern

2010-10-09T07:15:00.008-04:00

Countdown: 31 days to the NCI clinical trial.

Hemoglobin 10.8 (WHO normal 14.0-17.3)
Absolute neutrophil count 1.0 (normal 1.4-7.5)
White blood cell count 1.6 (normal 4-10)
Red blood cell count 3.06 (normal 4.5-5.9)
Platelet count 94 (normal 150-450)

Down and up and down again. They bounce around, says Dr. B. This week's test: a large-ish drop in hemoglobin and RBC, which accounts for the increased fatigue, headaches, and occasional lightheadedness. Having good days and bad days now. I'd say I feel OK about 85-90 percent of the time. Cutting back on work and preparing to wind down much more. But still going strong on most fronts.

Fortunately immune counts are still OK. ANC at 1.0 is considered above the threshold for treatment in the NCI clinical trial and no major risk for infection. Lymphocytes are down to 4.0 (normal 0.8-5.0) — low, but I've been that low before, and according to Dr. B there's no clinical consequence.

All my counts are still higher than when I was diagnosed in 2007. Then I went 26 days between diagnosis and starting chemo, so I don't see any problem making it to November 8. The docs seem OK with it too.

Nonetheless, trying to avoid infections at all costs. Lots of handwashing and Purell, not shaking hands so much, getting out of the room if there's coughing going on. It'll be great if I can make it to November 8 without getting sick.

As I start to navigate between the UM hospital and the National Cancer Institute, I am getting worried about falling into the crack between the two systems. UM works great on its own, I know from experience. Don't doubt the NCI does too. But these are two very big, very complicated systems with many moving parts. They're not designed to work with each other, and nobody in one system knows much about how the other one works.

In this trial, the NCI supplies the drugs but UM administers them to me. UM has to supply blood tests, and occasionally blood and bone marrow samples, which get Fedex-ed to Maryland. (Did you know you can Fedex blood samples at room temperature? Just stuff them into a cardboard tube, packed in tight so the tubes don't break. No idea why that doesn't ruin the samples.) Every transfer between the two has to work, and somebody — probably me — has to ensure they do. I dumped the blood samples into the Fedex drop box myself last week, wondering what would happen if they missed the pickup. But it worked just fine. Still, walking out of the hospital with a bag of blood felt truly strange.

Then there's the Third Man, the one I've been blissfully ignoring until now: my insurance company. I have no idea, yet, how that plays into this scene. From the company's point of view, a clinical trial is probably great; the trial bears almost all the costs, which in this case is going to save the company at least $100,000 at the discounted price it probably pays for Rituxan ($20K a pop at retail), certainly more than that if you throw in all the blood work and the test marathon I'll get at the NCI.

Symptoms

2010-10-07T08:54:00.005-04:00

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

2010-09-28T08:52:00.011-04:00

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

2009-11-15T06:47:00.005-05:00

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.

Balance

2008-04-28T09:40:00.015-04:00

Hemoglobin 13.5 (WHO normal 14.0-17.3) — normal by UM standards
Absolute neutrophil count 2.4 (normal 1.4-7.5) — NORMAL
White blood cell count 3.1 (normal 4-10) — subnormal but expected
Platelet count 201 (normal 150-450) — NORMAL

These counts are from April 9 (see new charts). The fact that I'm just getting around to posting them, three weeks later, gives you an idea of how much less urgent this all seems now. White blood count's low because of low lymphocytes (0.4, normal ≥ 1.2), which we expect to see for another 18 months or so. (This carries no clinical implications, so nobody's worried. Not even me.) Everything else looks great.

They actually had to make me go in for the blood test. I thought Dr. B had put me on a 3-month schedule, but the nurses thought otherwise, so I grumbled but I went. I won't have another until mid-May, at my next followup with Dr. B.

It's been 6 weeks since my last headache. Once in a while I get a twinge that reminds me the underlying condition's still hanging around, but otherwise I am absolutely, completely normal now. I can do anything. Bring it on.

Median time to relapse is 8 years. Retreatment usually works. Some people never need more treatment. And I have a normal life expectancy. That shakes out as victory — which is never permanent anyway, so why the hell not.

In fact I feel better than I have in 2 years. The other day I suddenly realized I'm taking stairs 2 at a time again, something I did all my life until I gradually started slowing down as my hemoglobin dropped. I made up all kinds of excuses — aging, tired that day, didn't want to sweat in my work clothes. But I sometimes wondered why I couldn't just bound on up the stairs, like I always had.

Anyway. Balance. I had in mind "balance sheet," adding up a bit of what I learned from all this. Today that seems like a bigger job than I want to tackle, so I'll just throw out this:

Time is short, and precious. Don't waste it wishing you were somebody else.

If you've got a good life, you don't need another one. Just do what you do, use what you have, give everything you can to everyone who needs you. Chop wood. Carry water.

Objection

2008-04-08T10:35:00.004-04:00

Passed on to me by a friend:

Conscientious Objector
by Edna St. Vincent Millay

I shall die, but
that is all that I shall do for Death.
I hear him leading his horse out of the stall;
I hear the clatter on the barn-floor.
He is in haste; he has business in Cuba,
business in the Balkans,
many calls to make this morning.
But I will not hold the bridle
while he cinches the girth.
And he may mount by himself:
I will not give him a leg up.

Though he flick my shoulders with his whip,
I will not tell him which way the fox ran.
With his hoof on my breast, I will not tell him where
the black boy hides in the swamp.
I shall die, but that is all that I shall do for Death;
I am not on his pay-roll.

I will not tell him the whereabouts of my friends
nor of my enemies either.
Though he promise me much,
I will not map him the route to any man's door.
Am I a spy in the land of the living,
that I should deliver men to Death?
Brother, the password and the plans of our city
are safe with me; never through me
Shall you be overcome.

Endgame?

2008-03-25T14:31:00.027-04:00

Too soon to declare victory.

But since I started acupuncture treatments, 12 days ago, I've had no headaches to speak of. (Well, I did have one, but it felt more like a hangover from taking a muscle relaxant at 3:30 AM.) Tension in my face and jaw slowly diminished. That's still hanging around, but at a low level, doesn't distract me much now and seems a bit lighter every day. Teeth mostly stopped hurting, too.

I had three followup appointments, 3-4 days apart. Yesterday the acupuncturist declared my case closed, except for a tuneup in 2 weeks.

Also, no drugs at all now, except nortriptyline for chronic insomnia. (If you read the link, don't freak out — my dose is only 15 mg, not enough to trigger the increase in suicidal tendencies that can accompany this drug when it's used as an antidepressant at 50-100 mg. And I had the insomnia long before HCL.)

One of my colleagues wrote:

I know about headaches, had them for 1.5 years every day. It was hard; I even talked to my own headache, as if it was a person controlling me. At some point, however, it disappeared, slowly. And when it was gone, I even missed it, strangely enough. I asked where are you? Of course, it was a blessing to live without it again. I hope you will find the same grace. I wish you love, strength and courage.

I know what he means. Mine lasted from the beginning of December until the middle of March. Three and a half months. You have no idea how good it feels to be able to just say Fine, thanks when somebody asks me how I'm doing. Pain makes very boring conversation.

Yesterday I spent 7.5 straight hours in social situations — meetings, a talk, a dinner. Wasn't sure I'd make it, but I did. Three weeks ago I couldn't go out at all, could hardly talk to my family, had to keep going off to lie down and try to relax my face. The slightest facial expression felt like the Nazi dentist from Marathon Man testing his drills on my head. When that's happening you think it will never stop. Thought I'd ruined my teeth. Started imagining living with dentures at age 50. Began contemplating a permanent headache, daily pain, a lifetime of pureed food — somehow accommodating to that. Somebody else I know has had more or less constant pain for a decade or so, from neck and back problems. I can't imagine how she survives it — much less taking the time to read this blog and write me sympathetic emails. Where does that come from?

The last few days have been almost like normal. Almost like before leukemia. Not quite. That one's not so easy to forget. And I'm still a bit nervous about the headaches coming back. If I go a month without them, I might get ready to drop my guard.

Acupuncture

2008-03-17T15:18:00.015-04:00

The week that followed my previous post consisted mainly of me lying in bed moaning like a dying warthog. Headaches from the third circle of hell. My temples screamed. The slightest facial expression made my jaw muscles clamp down so tightly I could hardly open my mouth. My teeth ached constantly, moving every couple of hours from one tooth or area to another just to keep me on my toes. My diet consisted entirely of soup and fruit smoothies, though even the soup made my teeth hurt. Vicodin and muscle relaxants would knock me out for a while, but it wouldn't be long before I was back in the inferno.

Then, last Thursday, I went to see an acupuncturist.

I'd tried this a few weeks earlier with a different practitioner, but the effects were minor at best. Then the guy tried to sell me on a weird enzyme-nutrition theory, so I didn't go back. This time I went to someone I'd seen seven years ago, for hip muscle spasms.

Having lived in Santa Cruz, CA for 9 years, I'm not a newbie. You can get treated by students at a local acupuncture school there for not very much money. I probably did 15 or 20 sessions altogether. The complaints I went in with were ones that probably nobody could do much about: chronic knee pain, most likely from a torn meniscus, and tinnitus (ringing in the ears) thanks to my previous life as a rock musician. The treatments didn't fix my problems, but I enjoyed them. Often I'd fall asleep on the table.

Two conflicting thoughts always run through my head when I do this. One is placebo, placebo, placebo. He's creating an atmosphere, reassuring me, pulling on the power of my desperation and my hope. And now I'm lying here relaxing for half an hour looking like a pincushion. Of course I'm going to feel better, but nothing's really happening. The other thought is this technique works directly on the nervous system. Western approaches treat nerves with drugs that affect the entire system all at once, but acupuncture acts on individual nerves related to specific sensations. Of course its effects are real.

This time, the effect was so dramatic that I simply can't believe it was placebo. I went into the office with a splitting headache, pain in my teeth, hurting so much I wanted to curl up and die. No bullshit, no explanation, no pep talk; Brodie just said let's see if we can get you feeling better and went to work. The first needle went into the top of my left foot, but I felt it mainly in my jaw. Most of the needles went into my hands and feet; a few into the sides of my head and my ear. This was a different pattern from what the other acupuncturist had tried (and I didn't feel much from his needles). I lay on the blissfully warm heated table for an hour, and all the tension and the pain simply melted away.

I left the office with no headache at all. Over the course of the day, the pain in my teeth gradually diminished. When I woke up the next morning that pain was gone, for the first time in 3.5 months — and it has not returned. Tension in my jaw muscles did continue, coming in waves lasting an hour or so. But though this got quite uncomfortable and distracting, it was not actually painful.

Felt the beginnings of a renewed headache on Monday, plus more jaw tension. Had another treatment and again, the next day was a very good day, no pain and much reduced jaw tension. I'll do this again a couple of times and see where it leads.

Now, I'm not a controlled experiment. Who knows if this would work the same way for you. I'm blasting away with every weapon I can think of, all at the same time, including muscle relaxants, ibuprofen (for inflammation), nortryptilene, self-hypnosis, etc., and so would you if you were me. But following the acupuncture I've tapered off the ibuprofen to just 4 pills a day, and mostly off the muscle relaxants too. No Vicodin, either.

Nothing else I've tried has been nearly so dramatic. It was, in a word, like magic.

While little is known about the mechanisms by which acupuncture may act, a review of neuroimaging research suggests that specific acupuncture points have distinct effects on cerebral activity in specific areas that are not otherwise predictable anatomically. — Lewith et al., in Evidence-based complementary and alternative medicine: eCAM 2:3 (2005), 315-9.

Head case

2008-03-06T09:18:00.012-05:00

So I've made my decision. Or maybe I should say that now a decision seems possible, for the first time. I'm going to stop worrying about HCL. Whatever happens will happen. I can't control it, and grinding away won't help. So stop.

I studied the beautiful martial art of aikido for 13 years, got a black belt, taught it at Apple Computer for several years — would still be doing it if my knees hadn't had enough. I've practiced yoga for 24 years, almost half my life. These are intensive physical disciplines requiring enormous, extremely precise control of the body. Every muscle, every tiny movement, the expression on your face, the motion of your breath are all part of the whole. You have to master all of it. The miracle is that you can.

Yet the further you go, the more you realize that controlling the body is merely a baby step toward something much, much harder: controlling what happens in your mind. In aikido, it's controlling fear and reflex; staying relaxed, easy, joyful with an attacker bearing down on you, eyes wide open and aware while you're spinning, rolling, flying upside down. Blending with the energy of an attack, channeling it into smooth, clear motion.

In yoga it's ending thought, in savasana (corpse pose). You just lie there on your back, resting, with your arms and legs splayed out and your eyes closed. Sounds easy. But the point of the pose is to clear the mind of thoughts, simply inhabiting the body, feeling the breath rise and fall. No past, no future. Only the present moment, here, now.

Eventually you learn: savasana's the hardest pose of all. Harder than 10-minute headstands, back bends, splits, extreme contortions of all sorts. In those poses you can't help but be in the present. You're concentrating so hard, against so much powerful physical sensation, that you almost don't have a choice. (Though even here your mind can wander.)

Worrying's the same. You know it doesn't do any good, but you keep right on going anyway. You're addicted. You decide to stop, but your vigilance gives out after 2 minutes and once again you're fretting away, mainlining anxiety, doing your drug. Your six-blade knife, do anything for you. You worry in your sleep, while you're working out, at the movies — even, secretly, while saying goodnight to your child, whispering to your partner, laughing with your friends. And you just can't stop. Not under your control. Maybe you blame it on somebody else, usually the person who loves you most. Maybe you blame yourself. Either way, now you're worried about worrying.

In my case, I can stop the conscious thoughts to some degree. But these infernal f-ing headaches just keep hanging around, stormclouds on the inner horizon, an embodied form of worry. The muscles in my face seem to be in permanent spasm. Hard little knots of fear, bundles of pain. Here's a partial list of what I've tried:

Self-hypnosis recordings on relieving TMJ and headaches
Binaural beat recordings
Hot tubs
Saunas
Professional massage
Self-massage (ma roller, fingertips)
Yoga
Inversion sling
Moist heat pack
Cold pack
Ibuprofen
Vicodin
Ativan
Alcohol/no alcohol
Caffeine/no caffeine
Inane TV shows
Trashy mystery novels
Intermediate bite splint (thin, hard)
Soft bite splint
Books on trigger points and TMJ

All this, but the headaches and the teeth-clenching still go on. Anything that induces facial expressions — basically any social interaction, even phone calls — ends with the sides of my head on fire, flaming golf balls wedged in my cheeks.

I know some people live with headaches forever. But now that the HCL is (temporarily) behind me, I'm determined to wipe them out. Maybe this will just happen, slowly, as an unconscious process. Right now I'm trying a week of heavy-duty ibuprofen, vicodin, muscle relaxants, bed rest, on the theory that if I can knock out the pain long enough maybe the muscle spasms will ease. Various appointments with specialists are pending, but I don't have high hopes. Ultimately this has to come from within.

It's going to take mind control. And that is just, basically, incredibly hard.

Yo-yo

2008-03-03T17:50:00.012-05:00

Hemoglobin 13.3 (WHO normal 14.0-17.3) — normal by UM standards
Absolute neutrophil count 4.1 (normal 1.4-7.5) — NORMAL
White blood cell count 5.1 (normal 4-10) — NORMAL
Platelet count 218 (normal 150-450) — NORMAL

Wow. I've never been through a bounce like this one. I feel like a human yo-yo.

Saw Dr. B today. His news is nowhere near as bad as they'd told me a week ago. In fact, the news is great — not perfect, but great.

So. The morphology — visual examination of my bone marrow biopsy, under a microscope — had revealed a small nodule, entirely normal in a 50-year-old guy. They stained the sample to find out what was in it. One of the stains showed CD-20, the cell surface protein that's typical of hairy cells. They determined that 5-10 percent of the bone marrow space consisted of CD-20 positive cells. That's what Dr. B's PA reported to me as "5-10 percent hairy cells."

For whatever reason (who the hell cares, now), she misinterpreted this result. CD-20's expressed on all B-cells, including the normal ones. So the 5-10 percent figure says exactly nothing about the percentage of hairy cells in my bone marrow.

What about the flow cytometry, the laser-based protein marker detector, far more accurate than morphology? On the flow, things looked, in fact, PRETTY DAMN GOOD. Less than 2 percent hairy cells! Yow! Dr. B said this is somewhere between a complete remission and a partial remission, in other words, a very very good partial remission — not at all what I'd been living with all week. Boing-a boing-a boing-a; this time the bounce is up. Sometimes you're the bug, but sometimes, sometimes, you get to be the windshield. Splat.

On top of that, my blood counts look great. Low lymphocytes (0.4), but we knew that. Almost normal red cells (4.43; normal is 4.5). Hemoglobin at 13.3, which UM counts as normal. Probably both of those will still rise for another week or two. Everything else well inside the normal range. I haven't seen a test like that in years.

What to do next? Dr. B's answer: nothing. No more treatments. Watch and wait. He calculates that the risks of rituxan aren't worth the additional benefit, especially because nobody's really sure, yet, whether there actually is any additional benefit from 2-CdA + rituxan. Most people in my category typically go years, even many years, before a remission (though some don't, and I'm mindful that I could always turn out to be in that camp).

We ended up leaving it that I'll go back for one more bone marrow biopsy in 3 months, see where I stand then. (Don't have to, but I want to, for psychological more than for medical reasons.) It's even possible that by then the 2 percent will drop further, though it could also go higher. But unless it really shoots up, I'm set for a while, and convinced that waiting is the right thing to do.

More later, after I celebrate. Yo-yo.

There is a girl in New York City
Who calls herself the human trampoline
And sometimes when I'm falling flying
Or tumbling in turmoil I say
Whoa so this is what she means...
She means we're bouncing into Graceland

Decision theory

2008-03-02T11:29:00.011-05:00

To tux or not to tux: that is the question.

Rituxan (trademark name; generic is rituximab) is a monoclonal antibody. "Monoclonal" simply means that the antibodies are identical (cloned). These artificially produced antibodies bind to the CD-20 protein complex on the surface of B-cells, including hairy cells. This dupes the immune system into thinking those cells are foreign, so it attacks them, with the same responses it would deploy against invading bacteria.

The drawback: rituxan can't discriminate between normal B-cells and hairy cells. It's genocide; the stuff just kills them all (or rather, it paintballs them and leaves your own body to do the dirty work on itself). Then the body grows new B-cells. You hope, you pray, that the new population doesn't include any hairy cells, or at least not so f-ing many.

In HCL, rituxan doesn't work very well all by itself. 8 weekly doses does markedly better than 4, but the response rates using rituxan alone, in previously untreated patients, are nowhere near as good as 2-CdA. But what about a combination of the two?

Those of you who've followed this blog may remember that early on, I considered enrolling in a clinical trial now underway at MD Anderson in Houston. They're testing a regimen of 1 week 2-CdA + 8 weeks of ritux starting 28 days later. In 13 patients treated with this regimen,

the CR rate was 100% with eradication of MRD (minimal residual disease)... in all but 2 patients after completion of the sequential therapy. No additional toxicity was observed with the combination compared with historical experience with 2-CdA alone (Hematol Oncol Clin N Am 20 (2006) 1125–1136).

That sounds pretty exciting, but it's only 13 people. Statistically, 2-CdA alone would have produced 9 or 10 CRs in a group that size anway. Still, if this trial works out, this regimen might become the new front-line therapy in a few years. If I do 8 weeks of rituxan now, my treatment would be almost clinically equivalent.

Let's be realistic here: this is not aspirin. My HCL friends haven't experienced serious side effects, apart from the few hours during and after the infusion (see Experience). But bad stuff does happen. Patients have died from cardiac arrest and kidney failure. A small number — all of them already suffering from lupus — came down with progressive multifocal leukoencephalopathy, a horrifying virus that kills brain cells and, eventually, you. You can get pulmonary toxicity and other nasty effects. However, most of the patients who've used this drug were being treated for other leukemias and lymphomas (not for HCL), so they were a lot sicker than I am and thus more likely to suffer side effects. Still, the reality of risk has to be faced. (That sentence sounds a lot nicer in passive voice.)

So here's my decision theory, so far.

Reasons to tux:

A good-to-excellent chance of achieving complete remission (less than 1 percent hairy cells).
A fair-to-good chance of achieving complete molecular remission (no detectable hairy cells).
If I'm gonna do it, now's the time. Ritux doesn't do well once the population of hairy cells gets larger. If I wait, I'll have to do another round of 2-CdA before I can get much benefit from rituxan. On top of that, I have the flexibility in my life and schedule right now to do the treatment without drastic effects on my job and my family.
A fair-to-good chance of a CR lasting 5 years or longer. New, better, more targeted drugs might be ready by then. If they are, I might not have to drag my body (and my family, and my friends, and you) through another round of 2-CdA.

Reasons not to tux:

In HCL, the relationship between the degree of remission and its duration — the time until symptoms reappear — is not straightforward. I'm at 5-10% hairy cells now. It's entirely possible I could stay this way for 2-4 years, or more, without treatment. Conversely, even if I achieve a complete molecular remission, the HCL could still come back in less than 5 years.
Immediate risks from rituxan infusions.
Longer-term rituxan side effects. I'm still coping with daily post-2CdA headaches (see my blog entries from January). Headaches are a low-grade side effect, not life-threatening; the medical literature barely tracks them, so there's no real way to know if the 2-CdA might have caused them. (In higher doses than I received, 2-CdA is neurotoxic, hence my suspicions.) Rituxan might make them worse, or slow my recovery, or both. Can't know in advance.
What WAID and John said, about not doing a treatment just to get nicer numbers, and about handling HCL as a chronic disease.

Seeing Dr. B tomorrow. My main questions for him: what's your clinical experience with rituxan? Side effects? What's the timeline for the other drugs now under development? What do you think of their chances for success?

And most of all — if you had HCL, what would you do?

Experience

2008-02-29T07:29:00.014-05:00

The only thing for it is to think.

This week I re-read everything I could find about Rituxan: journal articles, drug information sheets, blog entries, cancer forums. I also asked other HCL patients about their experience.

Once again I'm outside the norm. 85 percent of patients — 17 in 20 — get a complete remission after one treatment with 2-CdA. 10 percent — 2 in 20 — get a partial remission. That would be me. The other 5 percent — 1 in 20 — get no benefit at all, or not enough to matter.

WAID (stands for whatamidoing), in California, is in the 5 percent. Following diagnosis in 2003, at age 31, she tried 2-CdA twice. It did nothing. Then she did a couple of rounds of Rituxan (only), with unimpressive effects. Her latest regimen is ritux + alpha interferon, which seems to be working a bit better. The Rituxan gives her a mild, temporary case of anemia, knocking her hemoglobin down about 1.5 points and reducing her white blood counts, but not dramatically. So far, her counts bounce back after 2 months or so. I've had slightly more problems every time I've taken it, but overall I still have no complaints. She can control the side effects during the infusion with antihistamines and steroids. In my experience, the reports have been much scarier than the actual experience.

Judging by her many responses on the HCL discussion board, WAID knows a lot. Her advice: Since I assume you'd rather treat yourself, as a whole human being, instead of just creating a prettier set of numbers on the lab report at any cost, I'd say you should ignore the BMB results for now and see how you do for the next month or two. She suggests a second opinion from one of the major HCL researchers, Tallman at Northwestern, or Savens at Scripps.

Meanwhile, Pat says having 5-10% really isn't that bad, as your blood levels are coming up nicely! 200 for platelets... oh man, it's been a long time since i've seen that, heh heh... But concerning side effects of the Rituxan, as long as you take the precautions against "reaction," you will do just fine. Everyone who was a little apprehensive was pleasantly surprised how smooth the Rituxan treatments go.

Carolyn: I was treated with 2-CdA in October 2005 and had a BMB in mid December with a residual 5-10% Hairies (I think I had >90% at diagnosis). My counts have remained pretty stable for over 2 years and I never have had any real symptoms that can't be attributed to age, etc., except maybe the overwhelming tiredness that sometimes comes on me. That may be my age as well.

It's your cancer — do it the way you want to. Nice. Thanks, Carolyn.

And Paula: My last BMB was done the first of December and I am at 8% hairy cell, same as I was four years ago when diagnosed. My levels are all at low normal with the exception of ANC and it is at 1500, almost low normal. Sooo... I am just sitting around living life until something more goes south. She still hasn't had any treatment.

Tim failed 2-CdA twice. Then he started the HA-22 clinical trial, but had to back out of it after building up immunity to the drug. Krietman at NIH recommended 2cda+Rituxan, Army Doctor #1 recommended wait until I get worse. With his primary doctor (a third), Tim decided it was too early for another round of 2-CdA, only a year out from his 2006 treatment, with his bone marrow still recovering. So he and his primary doctor went with ritux. Considering the fact that Rituxan does not work very well in patients with marrow that is 50-100% involved, I asked to start early, rather than late and hit the HCL while it was not as established in my spleen and marrow. Sept-Oct 2007 I received 8 weeks of Rituxan. At the start I had so-so counts and knew my marrow was around 15-20% involved with HCL. I am doing pretty well right now. I tolerate the Rituxan very well, no real side effects, so that is also a contributing factor to deciding to continue to get treated with Rituxan to try and maintain my current condition and try and keep the HCL from regaining a foothold.

Finally, John got his diagnosis 14 years ago. He's done 2-CdA five times. Since 2002, I have been out of remission and have tried Rituxan. Rituxan does not have serious side effects, except while it is being administered. You can develop rigors. You have chills and shake. I did. I stayed home from work the next day and then resumed working.

Basic advice: Ignore the bone marrow. Watch your counts. You can live nicely with low counts. I have. I am never sick. This is a chronic disease. Once your ANC gets down to 1.0, it is time to treat. If your platelets get below 100, it is time to treat.

Don't make yourself crazy with this disease. I know now that I often embraced anxiety and fear in the past. Today I am much more relaxed about living with HCL.

All this experience is worth a million times more to me than dry statistics from the medical literature. Sometimes I think the Internet's an act of God. It's a complete miracle for people like me. With a disease that affects only 600 Americans a year, how else would I ever find the others?

Next post: how to decide.

Fear

2008-02-22T15:55:00.007-05:00

Bad news today. My biopsy results came in, and they don't look very good.

Hairy cells still make up 5-10 percent of the bone marrow sample. That's a remission, but only a partial one, and not a very good one at that.

Next stop, another visit with Dr. B on March 3. We'll discuss whether to do a round of rituxan, or whether to wait and watch for symptoms. Usually rituxan is a series of 4-8 weekly treatments. Each infusion takes a few hours. Side effects can be very serious.

At times like this — as if I'd ever had a time like this before — it helps me to remember the Litany Against Fear, from Dune:

I must not fear.; Fear is the mind-killer.; Fear is the little death that brings total obliteration.; I will face my fear.; I will permit it to pass over me and through me.; And when it has gone past I will turn the inner eye to see its path.; Where the fear has gone there will be nothing.; Only I will remain.

Biopsy

2008-02-19T12:23:00.008-05:00

Hemoglobin 13.2 (normal 14.0-17.3)
Absolute neutrophil count 2.2 (normal 1.4-7.5) — NORMAL
White blood cell count 3.1 (normal 4-10)
Platelet count 200 (normal 150-450) — NORMAL

Yesterday I had my followup bone marrow biopsy, or "BMB," as they call it around the Cancer Center. I'll get the results on Friday, or Monday at the latest. They'll tell me whether I've had a CR (complete remission, with no evidence of disease even at the molecular level), a GPR (good partial remission, with up to 1 percent hairy cells visible on flow cytometry), a PR (partial remission, up to 5 percent hairy cells), or — worst case, very rare — no real remission at all.

Dr. B's assistant, Ms. I, did the biopsy. Not quite as easy as last time, partly because she was teaching a student to do the job.

First Ms. I injected lidocaine deep into the upper part of my hipbone, just above the waistline on the right side of the back. This is the worst part, because you can feel the needle. (I don't really understand how a needle can penetrate bone, but it does.) The lidocaine burns like the fires of hell, but in a few seconds you're numb.

Next came the aspiration (narrow needle, for pulling out a fluid sample). Pressure, probing, pain, but not too awful. I just kept on breathing, trying to stay Zen. At the first biopsy in October, they couldn't aspirate at all, even though they tried three times, because the matrix of hairy cells was too thick. This time, it went well, and Ms. I had no trouble sucking out a cubic centimeter of fluid.

After that came the core sample (thick needle, takes out a 1.5 centimeter, narrow, circular core of bone plus bone marrow). These really f-ing hurt. This time Ms. I showed the student how to do it, guiding her hands. The night before I'd heard a doctor friend talk about learning to do this 25 years ago. Now it was my turn to sacrifice my body for the good of the profession. They have to learn somehow.

The student's unconfident, tentative pushing and wiggling actually hurt much more than Ms. I's firm, expert tapping and pressing — but at least she didn't push right on through to sample my kidney instead. Her lack of expertise showed up when they looked at the sample; she'd broken it in half, making it unusable. So Ms. I had to take another core. That's still better than last time, when they did three tries at an aspiration and two core samples — five stabs in all.

Today I feel like I've been kicked in the back with a steel-toed boot. Fortunately it's like a bad bruise, only hurts when I sit down wrong or try to twist too much.

Had more blood tests, too. Results are mixed. Hemoglobin and red cells are still climbing nicely. According to UM standards my hemoglobin's even normal now (at 13.2), since they use 13.0 as the bottom of the range. But the WHO standard for normal hemoglobin in men is 14.0, so I'm hoping it'll climb a bit more (and it should since my red cell count is still slightly low). Around mid-March I expect it'll all flatten out.

On the other hand, my white count is subnormal again. Not terrible, but still low. Could be (said Ms. I) because I've got a cold right now; tends to bring the white counts down. She didn't see a reason to worry about it. So I won't. Or at least I'll try.

Waiting for Friday's test results now... nervous but hopeful.

New normal

2008-01-29T10:34:00.000-05:00

Hemoglobin 12.3 (normal 14.0-17.3)
Absolute neutrophil count 3.1 (normal 1.4-7.5) — NORMAL
White blood cell count 4.1 (normal 4-10) — NORMAL
Platelet count 255 (normal 150-450) — NORMAL

New blood work, first in a month (see updated charts). Progress is happening — only hemoglobin remains subnormal.

I should be happy, but I'm not. Chronic optimism works against me here. I hoped for, expected, then simply assumed faster change; I thought all the levels would be well inside the normal range by now. Instead, hemoglobin rose only 1.3 points — less in the last 4 weeks than in the 2 weeks before that. Still a long way to go to hit 14. Now I'm extending the horizon, thinking in terms of the 120 days it takes your bone marrow to replace your entire blood supply. That'll be around the middle of March.

In one way, though, it's reassuring. I'm still plagued by intense daily headaches. I'm a basket case by mid-afternoon most days. The low hemoglobin might contribute to this. Nobody seems to have much of a better idea.

My doctor hypothesized they might be rebound headaches from the ibuprofen I was taking, a lot of it, for various reasons: hip muscle spasm, headaches, dental inflammation, more headaches. I'd heard about Excedrin rebound headaches, but I always thought they came from withdrawal from the caffeine in Excedrin. Turns out you can get rebound headaches from almost any analgesic — ibuprofen, aspirin, Vicodin, even acetaminophen — if you take it for more than a week or 10 days. My doctor told me about this on Tuesday and I stopped the ibuprofen completely the following day, noting that it can take 4-7 days for rebound headaches to subside. Well, today is day 7 since I took any painkillers. The headaches did diminish at first, but in the last couple of days they've come back, just as strong as before.

Another theory was eyestrain or outdated eyeglass prescription, but I had a full eye exam yesterday and they found no problems. They're raising my reading glasses strength to +1.75 from +1.5, but the doctor said that minor change shouldn't be causing massive headaches.

Chemo is a massive bodily insult, and it just may be that the headaches will go on for a while no matter what I do.

Meanwhile I’ve also learned some jaw stretches and massage trigger points for my nighttime teeth clenching. They do seem to be helping, and my teeth are finally getting less sensitive. For a couple of weeks there I could hardly eat, since everything – cold, hot, sweet, crunchy, spicy — hurt my teeth. I'm also trying self-hypnosis and binaural beats, both very relaxing but not an instant solution.

One piece of really really really good news: the MRI results showed no evidence of avascular necrosis in my hip joints. (They thought they'd seen this on my CT scan just before chemo.) I was looking at major surgery if that had turned out to be true. Hard to understand how they could see something on the CT scan that doesn’t show up at all on the MRI, but according to Nurse M the MRI just makes a much better picture.

I'm working more, survived 2.5 hours of meetings on Friday, but I'm really only up to about 60 percent. In the gym I’m working out at full steam, can drive my heart rate to 175 in sprint workouts with no problems. Not bad for a 50-year-old guy whose maximum heart rate is supposed to be 170. So I do feel better physically, even if my head pounds all afternoon most days and I can't work as much as I want to.

My therapist at the cancer center talks about finding a “new normal” in life after cancer. Much as I want to jump back into my old life, forget this ever happened, it ain’t gonna be like that. I have to go slower, take more time to re-enter, readjust expectations and commitments.

I hate that. I once told somebody that my philosophy of life was to run, flat out, until I had to stop. "Slow" isn't really in my vocabulary. At least until now.

Inverted

2008-01-16T09:34:00.000-05:00

My next blood test won't be until Jan. 28, so no new numbers today. Feeling better and stronger every day in most ways. Still having colossal headaches from nightime teeth clenching, but I bought some self-hypnosis CDs that help calm the jaw muscles down at night. So do yoga inversions: the inversion sling, headstands, shoulder stands. I never realized you could stretch the sides of your face in a shoulder stand. Plus, it reboots your brain.

Think about it: gravity does a lot of damage. Skin, breasts, testicles, muscles, ears — everything eventually sags under its force. That's happening to your internal organs as well. Spending a few minutes upside down every day reverses the direction.

A lot of people think of yoga as some kind of fringe thing, but to me it's essential. It does more to rejuvenate, strengthen, and calm me than anything else. There's ancient, real knowledge here, about how body and mind connect. And unlike a lot of exercise modes, you can do it all your life. BKS Iyengar is 89 years old and still practices 4 hours a day. Extreme, OK — but I wouldn't mind being able to do what he does when I'm 89.

Monday I had an MRI for possible avascular necrosis in my right hip joint. Amazingly fast — I was in and out in 50 minutes. Man is that MRI machine ever loud. They gave me earplugs but my ears still rang all day afterward. Forgot to take off my two silver rings, so the magnet made my ring fingers quiver, like scared little minnows attached to my hands.

No results yet, and I don't really want them, either. Now that I know the problem is there, I can feel something that might be related — pain at the adductor muscle attachments — but only in extreme hip positions such as certain yoga poses. I'm taking it easy on those, having no problems at all walking, riding a bike, etc. I'll wait for the doctors to evaluate this.

Meanwhile I came across another great HCL resource: Rob's User Friendly HCL Site and Chat Room. Lots of current and former HCL patients, very welcoming, some good information here.

Only numbers

2008-01-07T13:48:00.001-05:00

At my last post, on New Year's Eve, I'd just received upbeat blood test results. There's been more good news this week, especially being able to work out again. Still going at a reduced intensity, but I'm doing full-length workouts now — weights, cycling, swimming — and it feels like only a matter of time until I recover all my strength.

Unfortunately, that wasn't the whole story. Bad insomnia plagued me all week, waking me up for good at 3 or 4 in the morning. Thursday I managed a full work day, about nine hours more or less straight through on a grant proposal I'm writing with half a dozen colleagues. Friday morning, another four hours on that proposal. It was intense work: a lot of concentration, rapid thinking, staring at a computer screen, pressure to meet our deadline.

It felt great to work again, but by Friday afternoon my head was pounding like a taiko drum. Now it's Monday, and the headache's still hanging around, even after going back to bed for a while this morning. My teeth hurt again from nighttime teeth grinding. My bite splint helps a bit but doesn't solve the problem. Did some reading about bruxism and discovered that bite splints probably can't fix this, but nobody really has a better idea, even though about 20 percent of the population suffers from this.

Insomnia + teeth grinding = headache, maybe, but I still don't understand why it's so much more intense than it's ever been before. (These aren't new problems for me.) I ruled out various possible causes — supplements, caffeine, squinting — by cutting them out completely for three days, with no effect. I'm praying it's not a cracked or infected tooth.

So I'm seeing this headache as part of the recovery from chemo. However tempting, it's unrealistic to expect to jump back into my normal work life overnight.

Last week's test results are only numbers. They're good indicators of important variables, but they don't tell the whole story about what a week of intravenous poison did to my body, or how much recovery time it still needs. I have to keep taking it a little easier for while, rest more, not expect too much too soon.

New year

2007-12-31T11:43:00.000-05:00

Hemoglobin 11 (normal 14.0-17.3)
Absolute neutrophil count 2.7 (normal 1.4-7.5) — NORMAL
White blood cell count 3.5 (normal 4-10)
Platelet count 217 (normal 150-450) — NORMAL

New charts of my blood counts.

It's New Year's Eve. And time to celebrate. This treatment is working. Another milestone: normal neutrophil counts. That means my immune system is almost completely functional again. Lymphocytes are still low, 0.4 (normal 0.8-5.0), but that's double what they were 2 weeks ago.

At 11, hemoglobin's about where I thought it would be. Still three points to go before I hit normal, but with this much I'm nearly out of the woods. I still feel a bit less energy than usual, but I can go all day now. This is fantastic.

I have to say that for me — and not just because of the last three months — 2007 basically sucked.

So Happy New Year, everybody. 2008 looks really good.

Comeback

2007-12-27T10:35:00.000-05:00

No blood test this week, so no objective indicators — but I'm feeling better fast.

My extended family all made the trek to Ann Arbor for Christmas, six from North Carolina and four from Connecticut. They arrived late on the 22nd. That day and the next couple of days I got pretty tired and had to take naps in the afternoon. But starting Christmas Day, I abandoned the naps. On the 26th I spent 25 minutes on exercise machines, 25 minutes of yoga, then took an almost 2-hr walk with my brothers. And no nap. That would've been inconceivable a week earlier.

My mental hemoglobin calculation goes like this: I was at 9.1 on Dec. 17, four weeks after starting chemo. The median patient reaches normal (14-17.3) eight weeks from the start of treatment. Should be a linear progression, since it's based on red blood cell genesis replacing 1 percent of cells per day. So I should be going from 9.1 to 14 (or higher) in four weeks. That would be a gain of at least 1.25 each week.

It's been eleven days since Dec. 17, so I should've gained about 2 — which would put me at 11.1 today! Feels like it, too. Next test coming on Monday. By then white counts should be normal, except lymphocytes.

Strange: I've been dreaming a lot recently. No dreams at all during and after chemo, or at least none that I remember. Maybe my brain was too oxygen-starved to have any.

Midway

2007-12-18T08:13:00.000-05:00

Hemoglobin 9.1 (normal 14.0-17.3)
Absolute neutrophil count 1.0 (normal 1.4-7.5)
White blood cell count 1.3 (normal 4-10)
Platelet count 246 (normal 150-450) — NORMAL

New charts of my blood counts.

Yesterday was my 2-week followup with Dr. B. He showed up 2.5 hours after our scheduled appointment, but apart from almost falling asleep in my chair, I didn't mind. Now I know to always try for a morning appointment with him.

The best news: I'm out of the danger zone for opportunistic infections. My neutrophil count has climbed higher (1.0) than before I started treatment (0.7). That's still below normal, but anything 1.0 and above is OK. Lymphocytes, the second most numerous type of white blood cell, are at 0.2 (normal 0.8-5.0). Dr. B said lymphocyte counts might stay depressed for 2 years. Fortunately this has few or no clinical consequences, so the neutrophils (see the picture) are the thing to watch.

Now I can finally go out in public, stop worrying so much, and drop some of the more draconian precautions. No more face masks. I actually went to the YMCA with Gabrielle to pick up Luka from day care — first time I've done that in 5 weeks. But with family coming for Christmas in just four days, in general we'll continue the cordon sanitaire through the holidays.

For one thing, my hemoglobin's unchanged, at 9.1.

Hemoglobin, also spelt haemoglobin and abbreviated Hb, is the iron-containing oxygen-transport metalloprotein in the red blood cells of the blood in vertebrates and other animals. In mammals the protein makes up about 97% of the red cell’s dry content, and around 35% of the total content (including water). Hemoglobin transports oxygen from the lungs or gills to the rest of the body, such as to the muscles, where it releases its load of oxygen.

At 2.82, my red cell count is just a hair higher than last week's 2.79 (normal 4.5-5.9). So I'm oxygen-starved until that number comes up. Red cells live about 120 days. They travel 200-300 miles during their lifespan. The body normally replaces them at the rate of about 1% new red cells each day. We can see this happening now, since reticulocytes — new, immature red cells — make up a higher proportion than normal of my total red count. The figure I'd seen in the literature was 8 weeks from start of chemo to normal hemoglobin. In any case, it'll be at least another week or two before I have real energy again. Disappointing, but that's leukemia for you. Also a good reason not to get sick anytime soon. I feel crummy enough without having a cold.

From now on, I'll only get blood tests every 2 weeks. I won't see Dr. B again for 2 months. Then it'll be time for another bone marrow biopsy, to see exactly how well the treatment worked. Fingers crossed.

While I wait, I won't be posting so often to this blog. I need to start working again, anyway, though until that hemoglobin comes back, concentrating on anything for more than five minutes feels like swimming in molasses. I'm not too good at keeping a reduced schedule — it's more of an all-or-nothing thing with me — but I guess it's time to learn.

Upcrawl

2007-12-15T19:49:00.000-05:00

Decided to use this instead of "upswing," which seems way too dramatic for the pace of progress here.

Yesterday was actually pretty good for about half the day. Got up at our normal 6:45 AM and helped get Luka ready for school. Went for a walk in late morning, instead of waiting until afternoon when I'm already tired. That energized me and I felt OK until late afternoon, when I flagged and my headache came back. Still, not bad.

Today: not so great. Tired and draggy in the morning, hard to shake the headache. Both knees hurting a lot, maybe from walking too much, but no other exercise works and I'll go completely nuts if I don't use my body. Finally got a walk in around 4 PM — and a good thing too, with what sounds like a monster snowstorm blowing in from Indiana tonight. Tomorrow I'll probably be housebound.

Boredom. A good sign, I think. Until now I've been either too tired or too anxious to be bored.

Money

2007-12-13T18:17:00.000-05:00

Hemoglobin 9.1 (normal 14.0-17.3)
Absolute neutrophil count 0.7 (normal 1.4-7.5)
White blood cell count 1.0 (normal 4-10)
Platelet count 200 (normal 150-450) — NORMAL

Ended up with a pretty good day yesterday (Weds.), despite everything. Surprised myself by starting off on a walk and going for a full hour, slowly of course, but at least moving. After that, a few minutes lifting light weights, 30 minutes of yoga. Then into bed with ice packs for my right hip and knee — now sore from stretches I'm doing for my hip — and a one-hour nap. Then I actually felt OK for a few hours.

Later, as if the HCL just had to show me who's boss around here, I had another horrible night, awake 3-5 AM. This morning the infernal headache blasted back in like a monster truck, an evil tom-tom pounding my head into hamburger. On top of that, my entire ribcage burned: pectorals trashed from lifting 8-lb. dumbbells maybe two dozen times. Sixteen f-ing pounds.

Went for more blood tests late in the morning. All the counts are up a bit, especially compared to Monday, when they'd dropped back as the transfusion effect wore off. The best news: platelets are finally NORMAL, well inside the range. White cells and neutrophils still super-low, very risky for infection. My hemoglobin level's still dismal, which is why I have these tremendous headaches, but today's 9.1 looks a lot better than last week's 7.9. I've got some hope of hitting 10 by this time next week. Once I get there, I should start to feel semi-normal. Here are new blood count charts.

Let's talk money.

Frankly, I haven't paid a lot of attention to the hospital's billing notices. That's because I work for a major university with outstanding health insurance plans, so my bills always say do not pay — insurance reimbursement pending. Yeah, I've forked out a few $15 copays, and not every single thing is covered, for example the emergency visit to my dentist on Tuesday. But in general I don't even have to think about how much this all costs. I don't even have to fill out forms. I just show my card and walk away.

But from the cursory glances I've given the billing statements, I'd guess this episode has already cost at least $20,000. Possibly $30,000. Every blood test is a few hundred bucks, even though it takes them less than two minutes to draw the blood and the results come back in under half an hour, the blood having been examined automatically by a machine. CT scans are $1500 a pop, and they did three of them. X-rays, the bone marrow biopsy, flow cytometry, doctor visits. In the US health care system this adds up to unbelievable sums.

This isn't to say that my insurance company is actually paying out that much money. No, it gets a steep discount, on the order of 60-70 percent. God help you if you don't have insurance: then you pay retail.

When we lived in South Africa in 2003-04, my aging father-in-law flew in from Miami for a visit. The night he arrived he collapsed in his room with a brain seizure. He then spent over a week as an in-patient at the best hospital in Durban. He traveled by ambulance, had every brain test known to medicine, saw neurologists multiple times, had a sleep test, numerous blood tests, meds up the wazoo, basically everything they could throw at him. You may think South Africa's the Third World, and a lot of it is, but the medical system there is second to none; this was high-tech medicine at its finest.

Total bill, including 8 nights in a private hospital bed? Less than $6,000. For the same thing in the USA, you'd be lucky to walk out for $30,000.

Editorial, since it's an election year: the US health insurance system is totally broken.

Insurance is supposed to be a collective bet. We all pool our money. When somebody gets sick or injured, they take what they need from the pot. Since we never know when this might happen to us, or how bad it could be, it always makes sense to pay in your share, even if you never end up using it. If you lose, you win. You can be the healthiest guy on the planet. Then you walk out of a Starbucks, get nailed by a drunk driver while crossing the street with your latte, and end up paraplegic with a half-million-dollar hospital bill. Ask me last summer and I'd have told you the insurance companies were making a bundle off me. Now....?

I'm all for free market solutions when they work, but in medicine, they don't. The problem is that you (or your employer) shop for the cheapest insurance, not the cheapest medical care. You do not in fact want the cheapest medical care. You want the best. So the real market competition is at the level of the insurance, not the medical care.

And how do insurance companies keep prices down? They try to be sure that their pool of customers is healthier than everybody else's. That means excluding people from coverage if they have pre-existing medical conditions — like my self-employed friend Todd, who's been told that no company in California will cover him as an individual after his COBRA extension runs out next year. That's just one way we've ended up with millions of uninsured Americans.

The big irony is that we end up pooling our money to pay for them anyway: through higher prices for medical services, through higher taxes, through all the secondary expenses of supporting sick people who can't work. It's stupid. Medical insurance ought to be a basic right, not a privilege of the wealthy. It ought to be single payer, with everybody contributing their share and everybody able to draw out whatever they need.

That's not just the only sane solution. It's also the least expensive one. For the best thinking on this, read Paul Krugman's columns in the New York Times. (Try this one for starters.)

Hanging

2007-12-09T14:47:00.000-05:00

Lost momentum on the blog there for a few days, mainly because there's not much to report. Over the weekend I had more energy from the transfusion. I put most of it into playing with Luka and trying to catch up on the hopeless household backlog of minor repairs, finances, and cleanup. My hip muscle spasm continued, limiting my movement a lot. But I've minimized it with ice, rest, and ibuprofen. Today it finally feels like it's nearly over. I'm increasingly convinced it's not related to whatever's going on in my femur heads.

Since Sunday night the transfusion's been slowly wearing off. The telltale fatigue and eternal headache are settling in like a throbbing fog as my hemoglobin level sinks. Went for another blood test yesterday morning, but the nurse never called me with the results. I didn't have the energy to pursue it (partly because those readings won't mean much, since they're skewed by the transfusion). Another blood test coming Thursday.

I thought I had an appointment with my dentist on Saturday afternoon, but when I got to her office at 2:30 no one was there. She has a direct number for emergencies, and though my teeth were feeling better I decided to call her. I've been seeing her for 5 years or so and we've got a really nice relationship, one of those ones where you feel like you can ask for a favor when you need it.

On the phone she liked my theory that Nystatin — the mouth rinse I've been using for thrush, a horribly sweet goo that requires swishing 4 times a day — might be eating away the enamel and causing the tooth sensitivity. She would've made a special trip to see me anytime, but I decided it could wait until today. She didn't see anything wrong, so we talked about ways to deal with the sensitivity, mainly by using fluoride right after the Nystatin, or maybe trying to get a pharmacist to reformulate the Nystatin in some non-sugary base. For the time being, I've stopped the Nystatin and my teeth are more or less back to normal: lousy but liveable.

Tonight (which is Tuesday, despite the date on this post) I'm basically back to where I was last week at this time, the night before the first transfusion. By now my bone marrow should be putting out red cells and hemoglobin, so I should be on the upswing. But it takes 8 weeks to get to normal, and this is only the end of week 2. The nurse warned me I might need more transfusions before my own cells really come up.

We'll see what tomorrow brings. Right now I can barely move. Giant vise grips are closing on my temples. My eyes hurt. Hope I can sleep.

Pan into Fire

2007-12-07T17:46:00.000-05:00

Physically, I felt better today than I have in three weeks.

That's strange, since today's news ain't good.

Yesterday, trying to take advantage of the hemoglobin reprieve from Wednesday's transfusion, I managed two half-hour walks. Then I spent 10 minutes on a recumbent bike — nowhere near as tiring as the last time I tried that a few days ago — and did a few relaxation asanas. Had a slight fever off and on all day from the transfusion.

That morning I'd read up on avascular necrosis of the femoral head and neck. My CT scan showed trouble there, suggested followup X-rays and MRI. Dr. B had sent me for X-rays, but I was so focused on the leukemia that I just didn't think about it much. I realized I'd better face the music.

That might have been a mistake. By bedtime I started having the (to me) telltale signs of an impending muscle spasm. (Psychosomatic?) By 3 AM my right hip was on fire. I had to haul out the Vicodin.

Bone's alive. Avascular necrosis means that part of a bone loses its blood supply and begins to die. It can recover, and in fact bits of bone are always dying and being replaced by new, living tissue, but in necrosis the dying outpaces repair and eventually becomes permanent. The blood supply can be cut off temporarily or permanently, by trauma, arterial blockage (caused mainly by alcoholism), steroid use, half a dozen other things. Or it can be "idiopathic," meaning we have no idea why this happened to you. In my case, aikido, hyperextension of the hip joint in yoga, maybe the 4 cortisone injections I had in the 1990s, might be causes. If I have this. It's not uncommon: 10-20,000 cases a year.

One day in the winter of 2001, Gabrielle and I went cross-country skiing for a couple of hours in 15° weather. When we finished, we jumped immediately into the car, drove somewhere, spent perhaps an hour sitting in a very cold car. It had been a huge workout — and if you ski you know that the back of the hip does most of the work — but I'd never stretched. When I got out of the car I could tell something was wrong. By the end of that day my right hip ached fiercely. This continued for several days while I essentially ignored it and tried to power through my usual regimen of cycling, stretching, yoga. By Day 4 it hurt so much I couldn't walk. Standing in one place hurt even more than walking; the tiny postural movements your hips make to keep you balanced burned like fire. By then, with one muscle after another trying to take over the load from the other, exhausted ones, the spasm had spread to the entire pelvic girdle. The following morning no position at all — not even lying down — was pain-free. I remember I was supposed to deliver a presentation with Tim Killeen — now director of NCAR — at 8:30 AM, but I literally could not stand up. I had to call and cancel 30 minutes before the presentation. Tim could not have been happy about that.

I finally went to see my doctor. He laughed when I told him about it. You kicked your own butt! He prescribed Vicodin, muscle relaxants, and bed rest.

It worked. I had to stay flat on my back in bed for 3 days, loaded on Vicodin, but after that the spasm subsided and I gradually returned to normal over the space of a few weeks. Even 3 months later, though, the muscles in that hip remained stiff.

Since then this same hip spasm syndrome has recurred, four more times. The etiology was always the same: major exertion with failure to stretch afterward, either preceded or followed by prolonged sitting or lying down. Once after a night on an old, rock-hard futon; another time during a marathon drive from South Africa to Namibia. Right now, a mild workout following a week or so of lying down most of the time. I learned to handle it: eat ibuprofen like candy, ice the hip like crazy for a few days, stretch slooooowly and gently, massage.

Anyway, I always thought of this as your basic sports injury, probably a torn piriformis. Now it seems like it could be a symptom of whatever's going on in my femur. Maybe the skiing produced the original trauma; maybe not. Maybe avascular necrosis isn't it. But Nurse M, who saw the recent X-ray results, told me today that they've asked for further evaluation.

That means an MRI. And from what I've read, they only do an MRI if they think you might have this. It's pretty bad news. Avascular necrosis never goes away on its own. It can take a few years to progress, but it virtually always ends in total degeneration of the hip joint. By the time you get symptoms like mine, the treatments are basically all surgical. Core decompression (carving out the core of the bone in hopes that it will revascularize and repair); this doesn't work very well (15-20% success). Metal-on-metal joint socket repair. Total hip replacement: those artificial joints only last 10-15 years.

Nurse M requisitioned the MRI on an urgent basis. I'm trying, trying to hope that this'll turn out to be something less serious. Meanwhile I'm doing what's worked before to make the spasm subside. But once again I feel like I've entered a dark tunnel. Nothing to do but grope on through and hang on.

Transfusion

2007-12-06T08:35:00.000-05:00

Hemoglobin 7.9 (normal 14.0-17.3)
Absolute neutrophil count 0.7 (normal 1.4-7.5)
White blood cell count 0.9 (normal 4-10)
Platelet count 134 (normal 150-450)

Yesterday and the day before were the worst yet. Horrendous headache, fatigue, shortness of breath. Just getting up from a chair made my heart pound. I could barely move, yet sleep eluded me. My pulse pounded in my ears, a sickening squishy sound. Even lying in bed it ran about 75. (My normal: about 55.) Yesterday I woke up at 4:30 AM and couldn't get back down, even though I felt like I'd been run over by a truck.

So they gave me a blood transfusion. I had no idea how long it would take. The answer: a long time. Dragged myself to the hospital at 1 PM for blood tests. Hemoglobin down to 7.9, so no wonder I felt like roadkill. They scheduled the transfusion for 2 PM, but cross-matching took longer than expected and I didn't get into my chair until 3. The runner didn't show up with the first unit of blood until 3:30. By then I was practically moaning.

Consent form: common risk of fever, chills, vomiting, allergic reaction. Uncommon risks of kidney and liver failure (mostly in old people). 1 in 100,000 risk of infection with HIV, hepatitis, or some other horrible virus. I signed off. The nurse put in my IV. I swallowed "premedication": Tylenol and Benadryl, to fend off fever and histamine reactions. Then a complicated, extremely careful cross-checking procedure, with one nurse reading off my information to another, who double-checked her.

Finally the transfusion began. Each unit of blood took about 1.5 hours to pump in; they gave me two. My headache disappeared, but I stayed very groggy, due no doubt to Benadryl on top of no sleep. My temperature rose to about 99.5° (my normal's 97.8°). After the second unit, they kept me there for a half-hour observation period. Went home, still groggy, and put Luka to bed, then crashed myself, still running a slight fever. Woke up at one point in a puddle of sweat, but this didn't recur and I felt more or less normal this morning.

Feeling like a million bucks would not be a good way to describe my current state. Maybe ten bucks. Definitely better than yesterday morning, when I felt like the national debt. Still tired, sore all over.

Dr. B's excellent nurse said the transfusion should boost my hemoglobin to somewhere in the 9-10 range — still below where I'd feel semi-normal, but a lot better than the 7s and 8s. The effect might last a week, but it'll decline during that period. This should take me past the bottom of the trough in my own production of red cells and hemoglobin, which should start to finally rise next week. But I might need another transfusion if it doesn't pick up quickly. Median time to normal hemoglobin is 8 weeks after chemo.

It's strange to feel somebody else's blood in my veins: actually, two people's blood, since I got two units. I thank them, profoundly, whoever they are. This makes me glad I gave blood a few times, though two more transfusions would probably exhaust my karma. I don't know whether they'll let me donate blood in the future, given the hairy cell, but if they will I'll be doing that.

My statistics show a definite upward trend in immune counts. Platelets recover before anything else. Mine should reach the low end of normal next week! Neutrophil counts have risen back to where they were in October. So the bone marrow's working; it just takes longer to start putting out new red cells and hemoglobin. This part nobody warned me about. I'd focused on the immune counts, but the exhaustion from the low red counts will persist at least a couple more weeks.

Other problems might be coming. Several teeth are getting really sensitive, probably from anxiety-related nighttime grinding, but conceivably from infection. All the lying down is taking a toll on my muscles; I'm prone to hip muscle spasms in this kind of condition. I'd hoped to take a walk this morning, but it's 12° here. Maybe the temperature will come up in the afternoon. Somehow I've got to take advantage of this brief reprieve from total exhaustion to move my body a bit.

Statistics

2007-12-04T12:54:00.009-05:00

Here are charts of my immune cell blood counts (top) and red cell/hemoglobin/platelets (bottom). I'll keep updating this as new stats come in. There's a permanent link to this post in About this Blog (top right corner of the blog page.)

About the charts:

Values listed as "normal" are the midpoint of the normal range, so I'll be OK before I reach the levels on the left-hand side of the charts. (If I were better at Excel, I'd have shown the full range.)
There's no baseline data for my own normals from before HCL. One day I'll track that down.
The X-axis doesn't represent time proportionally. Times between tests were longer (3 months) from April 2008 to July 2010. In 2007 and since July 2010, tests are only a week or two apart. Dates at the bottom.
Click on a chart to see a larger version.

Virtuoso

2007-12-03T21:12:00.000-05:00

Hemoglobin 8.3 (normal 14.0-17.3)
Absolute neutrophil count 0.4 (normal 1.4-7.5)
White blood cell count 0.6 (normal 4-10)
Platelet count 120 (normal 150-450)

You should have a normal life expectancy. This sentence rang in our ears as we left Dr. B's office this morning.

First Dr. B's PA — let's call her Ms. X — entered. No smiles here, but on the other hand, a daunting air of capability. Straight to business: any chest pain, shortness of breath, dizziness? Nausea or vomiting? Then a quick physical exam, then she left to discuss with Dr. B.

One thing I learned from House — an easy character to hate, but one who's usually right — is that I don't really care if my doctors are nice people. I want them to be stunningly, overwhelmingly competent (at least so long as that doesn't stop them from really listening). If they also happen to be nice people, yahoo, but that's gravy. If they need to be arrogant or abrasive to get that edge, that's OK with me.

Dr. B entered a few minutes later. Up on the table please, then we'll chat. He asked me all the same questions Ms. X had just gone over. Then he did the exact same physical exam, hunting for my spleen, which none of the doctors have been able to palpate even though it's enlarged. Dr. B couldn't feel it either, but he knew how to try. I was thinking why is he repeating everything his PA just did? Then it dawned on me: error-checking. Obsession with detail. Need to know from own experience, not just report.

I sat down beside Gabrielle. Dr. B perched on the exam table, began twiddling a paper towel. And launched into a mini-lecture on hairy cell leukemia that could have come from a textbook, except it couldn't, because he knows the insider details no textbook tells. For example: the Scripps studies of minimal residual disease in HCL always find it (MRD), because they do the bone marrow biopsies just one month after chemo ends. But indolent cancers like HCL take longer than that to clear. Dr. B doesn't do biopsies for MRD in HCL until 3 months. As for MD Anderson, things always work better in Houston. The Northwestern team takes the same approach as Dr. B, who knows this because he's talked it over with the head guy. This little talk went on for at least 25 minutes: precise, detailed, tightly organized, perfectly clear. Completely convincing. He answered every question I'd written down before I could ask.

He knew every study I'd read, cited them. Knew my case history cold, including previous conversations with Dr. A about my diagnosis. Knew my blood counts, not just today's but over the last couple of weeks. After hours of poring over papers on HCL diagnostic pathology, I'd decided my HCL diagnosis was correct. Dr. B repeated the chain of reasoning I'd followed. Being fully convinced of this is an enormous relief, because if you have to get cancer, HCL's the one you want. I just read an entire special issue on HCL in Hematology/Oncology Clinics of North America (Oct. 2006), which says that this disease deserves the disproportionate attention it gets because they are so close to a complete understanding — and a cure. That last word's not one you hear often in cancer research.

As for long-term CD4 T-cell suppression following 2-CdA, Dr. B explained why this doesn't translate into clinical immunosuppression. Many immune cells hide in the lymph nodes, the spleen, elsewhere. So low numbers of circulating cells, the kind detectable in blood tests, don't necessarily correlate with an insufficient supply.

I brought up rituxin and BL22. Dr. B knew all about them. BL22's not ready for prime time; it can cause kidney failure. Dr. B has actually treated at least one HCL patient with rituxin after 2-CdA, and would do the same for me if my remission isn't good enough. But he thinks the risk/benefit balance argues for awaiting a relapse before adding rituxin to the regimen. If 40-50% of all patients never relapse, why not see if I'm in that group first before drenching my blood with another toxic chemical. That's what I'd concluded too, but I needed to hear it from an expert.

I have never witnessed a more confidence-inspiring performance in my life. Dr. B's a virtuoso, a medical master at the top of his game. When he wrapped it up with you should have a normal life expectancy, Gabrielle's voice broke. Mine too.

White cells, up slightly, might be starting their rebound. Platelets are recovering fast! The count's up to 120 — it was around 100 at the first blood tests in October, so this is significant. Means I don't have to worry about cuts so much. Also, the spot on my leg turned out to be nothing worse than eczema, not to worry.

But the bad news isn't quite over. My hemoglobin's dropped even further, and may go a bit lower yet. I'm at the blood transfusion borderline (hemoglobin below 8; mine's 8.3). I could get one now if I asked for it. Might buy me a week of higher functioning, take my hemoglobin up to around 10. That would be incredibly nice, since I've been in bed most of the time for nearly 2 weeks. The infection risk from a transfusion is just 1 in 100,000, but there are other risks, such as fever and other complications from an imperfect blood protein match. Dr. B seemed to want to wait a few more days, at least until the next blood test on Thursday. It's easier to track what my body's doing if they don't interfere with it.

And there's a new issue. My CT scan revealed some kind of problem in the bone marrow of my right femur head and neck, possibly the left one as well. Dr. B thinks it's not the HCL; otherwise we'd have seen the same thing in all my bones. He thinks it's probably avascular necrosis, i.e. bone death from insufficient blood supply. Dr. B sent me downstairs for more X-rays right after our appointment.

Not clear what this means or whether it's serious (yet), though Dr. B did mention hip replacement (but more in a long-term-speculating way). Over the last 6-7 years I've had several episodes of intense muscle spasms in both hips, especially the right hip; this could be related. Maybe connected to 13 years of aikido, which does tend to give your skeleton a pounding.

Still, I haven't had a spasm like that in 2 years, and this problem seems minor compared to cancer. We'll see what the X-rays show.

Came home from the hospital, got into bed and sank into a groggy, horrible sleep until 5:30 PM. And I'm ready to do it again, right now.

Switch

2007-12-02T23:36:00.000-05:00

Last week we debated whether to take Dr. A up on his offer to change to another physician, after the dosing error episode. Eventually we decided to do it. Dr. A proved extremely graceful about helping us make the switch.

Tomorrow we'll see Dr. B for the first time. It's my first hematology followup since starting chemo two weeks ago. Dr. B is a leukemia expert with an outstanding reputation, so I'm hopeful.

Here's what I want to know:

What does he think about whether eradicating minimal residual disease might improve my long-term chances? Minimal residual disease (MRD) basically means some tiny number of remaining HCL cells, detectable by flow cytometry or PCR, the most sensitive tests we now have. "Eradicating" doesn't really mean it; they're really talking about reducing the levels below the threshold of measurement. In a Scripps study, 50% of HCL patients with MRD relapsed, but only 6% without MRD relapsed. However, other studies failed to find a link between presence of MRD and relapse rates.
My CT scan shows some problems in the marrow of the femur head and neck, probably on both sides. Might be leukemia, or something else, or both. The radiologist recommended following up with MRI and dedicated X-rays of that area. When do we do this — now, or after the treatment has had a few more weeks to normalize? What could these results mean?
2-CdA can produce very long term suppression (several years) of CD4 T-cell counts. What are the clinical implications? Will I be immune suppressed, and if so, how and how much?
What the hell is this dime-size spot on my right shin that's been itching sporadically for 2 weeks?

Took half a Valium last night. (Dr. F had OK'd that last week.) Slept the sleep of the dead until 9:30 AM. Felt great for an hour, then collapsed again. I went back to bed and slept deeply until 3 PM, thanks to our great friends Susan & TR, who spirited Luka off for 4 hours to bake Tollhouse cookies and watch videotapes of 1930s Betty Boop cartoons. Around dinnertime I started to perk up, but I'm ready to sleep again as soon as I post this. Some combination of the Valium and yesterday's workout.

Yawn. Night-night everyone.

Precautions

2007-12-01T22:07:00.001-05:00

Hellish night last night. Didn't sleep until after 3 AM, due to pain from a symptom we really don't need to discuss. Nothing life-threatening, just painful as hell. Now I have two new bottles of drugs.

Stayed in bed most of the day until mid-afternoon, when I felt rested enough to try some exercise. 12 minutes at settings 2 and 3 on a recumbent bike. Six weeks ago that would have been an easy warm-up. I'd barely have noticed it. Today it drove my heart rate to 145. After that, an hour of yoga. Managed my first inversions in 2 weeks: headstand, shoulder stand. Wow did that feel good, but it wiped me out. Savasana felt like what it means: corpse pose.

I'm still quarantined upstairs while Gabrielle gets over her cold. Only at night, though. During the day we've dropped the face masks, since she's not coughing or sneezing and Luka's well. My great friend Todd has been visiting from California. He got sick too, two days after arriving, but now seems to have pulled through it. Fingers crossed I don't catch it.

Everybody's using Purell and washing their hands obsessively. We don't let anyone in the house without wiping down face and hands with disinfectant. Mostly nobody's come inside anyway, other than our housecleaner. Except for walks outdoors and trips to the hospital, I haven't visited public spaces at all since chemo started almost two weeks ago. Drove to Walgreen's twice to pick up prescriptions, but there's a drive-through window so I never went inside. If I handle a pen somebody else has just used, I Purell my hands afterward. Luka's poor little hands are beet red and painfully chapped from the constant washing.

There's much more. We use paper towels for everything, except bath towels, which we use only once before washing. Rubber gloves for dishwashing. Lysol wipes for kitchen counters and bathroom surfaces, also doorknobs and light switches. I wear a NIOSH respirator every time I go down to the basement, since we've had occasional mold problems. At the hospital I wear my NIOSH mask, and push elevator buttons by shielding my finger with a shirt sleeve. When Luka comes home from school, Gabrielle makes him change clothes and wash before he can see me.

We backed down on the most puritanical food precautions after hearing, from both a nurse and our doctor, that the restrictions on fresh food weren't necessary. Research data show no difference in infection rates with patients allowed to eat fresh vegetables, peelable fruit, etc. The only things to avoid are uncooked vegetables, unpeeled fruit, raw fish, raw meat, hamburger (because it's heavily handled in processing), eggy stuff like potato salad and egg nog (salmonella risk).

All these precautions seem insane sometimes, but only until I start digging into the medical literature. About 30-35 percent of HCL patients get some kind of infection. I'm no exception; I came down with a mild case of thrush, an oral fungus that makes your tongue look like a dirty white sponge. Nystatin's keeping it down (blecch; tastes of metal and saccharin). Most infections don't get past Stage I or II, as they say in medspeak. But a not inconsiderable percentage reach Stage III or IV. You do not want to reach Stage IV. Stage V, if it existed, would be death. And some HCL patients do get there. Not many, but not none either.

Fungus, pneumonia, herpes zoster (shingles) are the most common. On Monday, after they take another set of labs, I'll start Bactrim as a prophylactic against Pneumocystis jirovecii, a fungal pneumonia common in immune compromised people.

Yesterday I came across the Hairy Cell Leukemia Research Foundation. I'd actually seen the site last week and decided it was dead, but it's not. It's full of survivor stories, as well as accounts of people who went through the Rituximab and BL22 clinical trials. Some of these accounts detail incredibly serious infections: people blown up like balloons, their entire skin peeling off, other horrors you only want to know about if you're me right now. One guy had the bad judgment to blast off on an 800-mile high-intensity road trip four days before starting chemo. He got pneumonia on the second day and barely survived.

Also a lot of hope here: people who've been disease-free for 8, 10, 12 years and more. Healthy and happy. I asked the webmaster to link to this blog, since I'm hoping other patients will find it useful. (Leave me a comment if you do!)

Thanks to the wonders of Google Analytics, I see that at least 150 people have read this blog since I started writing it 2.5 weeks ago. The exact count today is 230, but some are probably duplicates, such as the same person (including me) viewing the blog from two different computers, so I discount the number a bit.

Patent

2007-11-30T09:37:00.000-05:00

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

2007-11-29T16:38:00.000-05:00

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

2007-11-27T15:29:00.000-05:00

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

2007-11-27T12:46:00.000-05:00

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

2007-11-27T12:21:00.001-05:00

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

2007-11-26T10:06:00.000-05:00

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

2007-11-26T08:20:00.001-05:00

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

2007-11-24T20:14:00.000-05:00

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

2007-11-23T18:38:00.000-05:00

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

2007-11-22T16:28:00.000-05:00

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

2007-11-21T14:34:00.000-05:00

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

2007-11-20T11:36:00.001-05:00

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

2007-11-20T11:19:00.000-05:00

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

2007-11-20T10:22:00.000-05:00

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

2007-11-19T16:44:00.000-05:00

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

2007-11-18T10:34:00.000-05:00

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

2007-11-17T20:30:00.000-05:00

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

2007-11-16T21:07:00.000-05:00

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

2007-11-16T16:31:00.001-05:00

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

2007-11-16T09:51:00.000-05:00

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

2007-11-15T08:32:00.000-05:00

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

2007-11-15T08:00:00.000-05:00

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

2007-11-14T22:19:00.001-05:00

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

2007-11-13T08:42:00.001-05:00

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

2007-11-12T13:50:00.000-05:00

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

2007-11-11T11:08:00.000-05:00

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.