New year

• 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

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

• 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

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

• 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

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

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

• 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

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

• 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

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

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

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

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

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

Singing bowl

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

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

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

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

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

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

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

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

Everyone looked at me.

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

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

Low

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

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

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

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

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

GIGO

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

Over

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

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

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

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

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

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

Standoff

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

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

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

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

Stay happy. Give. Love.

Thanks-giving

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

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

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

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

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

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

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

And many, many more. Thank you all.

Why

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

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

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

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

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

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

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

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

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

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

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

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

Wait

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

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

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

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

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

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

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

Sledgehammer

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

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

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

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

Apology

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

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

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

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

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

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

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

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

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

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

Chemo

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

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

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

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

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

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

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

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

911

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

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

Words fail me.

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

SNAFU

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

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

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

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

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

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

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

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

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

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

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

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

Commitment

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

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

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

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

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

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

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

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

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

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

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

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

Nurses

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

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

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

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

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

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

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

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