I/O

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!

• 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!

• 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

• 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

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

• 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

• 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

• 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

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!

• 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

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

• 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

• 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

• 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

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

• 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?

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

• 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

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

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

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

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

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

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

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

None of this persisted after treatment in 2007.

Not all symptoms are bad. Two more:

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

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

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

I've certainly had worse problems.

Re-run

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

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

Now, here it comes again.

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

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

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

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

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

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

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

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


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

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

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

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