Public Service Announcement

The good folks at the Liddy Shriver Sarcoma Initiative have produced a short video (in several languages) offering basic information about sarcoma and seeking participants for this summer's "Team Sarcoma" fundraising and outreach event. I'd recommend watching the video without sound; the stock music is annoying. Also, if you already know more than you want to know about soft-tissue sarcoma, the Shriver initiative site is a rich trove of more advanced information about clinical trials, new research, etc.

The video:

"One Life, Live It"

About eight months ago, a 20-year-old woman, Nicole Morgan, from Derbyshire, England, was diagnosed with metastatic epithelioid sarcoma. She died in late February. Her obituary from her small local paper is here. L. pointed this piece out to me. We were both struck by Nicole's willfulness, defiance and bravery.

Her motto had always been "one life, live it", a philosophy she strongly believed in.

And a few weeks before she died, she defied her parents and doctors by having the words tattooed on to her wrist.

Mum Tracey said: "We told her not to go because the doctors had said her platelet count was low but she told us that if we didn't take her she would just take a taxi and get it done anyway."

About 15 family members and friends also got similar tattoos. She was a favorite fan of the local rugby club. She asked for her coffin to be driven to the church in a white hearse pulled by two white horses. Horses! She was so young! But a ghastly attraction for youth, unfortunately, is one of sarcoma's many horrors.

*

I can't help imagining this: If after death we rise up as spirits and wait to be judged, on arrival would Nicole draw back her cooling tattooed wrist and cuff whatever she found there?

My lungs, on air

I'm feeling like a bad storyteller here, the kind who says,
"And then... and then... and by the way...." So be it: The
idea is to write a narrative of the illness that will
eventually be up to date, so my reactions are fresher and
more detailed then they are when I'm trying to recall the
particulars of byegone tedious and narcotic-soaked days in the
hospital.

So, anyway, after being bounced from chemo and admitted to
the hospital with another collapsed lung, this time on the
previously "good" side, Dr. Lump-not-Split was suspicious of
metastasis, despite repeated CT and MRI studies and testing
of some fluid evacuated from the pleura for malignant cells.
(This suspicion wasn't -- isn't -- wrong; while the studies
are possibly capable of picking up tiny pulmonary nodules of
about 5mm plus-or-minus a couple millimeters, such tiny
nodules are easy to miss.) Enter VATS. A surgeon makes about
1cm incision and inserts a video camera into the chest
cavity. In my case, the doctor completely deflated the
relevant lung, inspected it for abnormalities, took a biopsy
(which was normal!), and blew in some talc for
a pleurodesis. I found the immediate aftermath of the
procedure difficult: I felt as though I had been kicked in
theribs, and I also felt a milder version of the same
feeling of lung constriction I had after having pleurodesis
through a chest tube. I was discharged from the hospital
about two days after the procedure with a sore chest that
became intensely painful when I coughed, sneezed or laughed.
Humor was outlawed for a while. Fortunately, the pain
steadily decreased. I'd say it was more or less gone within
10 days; my reward for healing was getting to restart
chemo.

I should point out here that while VATS is a great
technique for all sorts of lung maintenance (biopsies,
removing nodules, fixing pleural effusions and pneumothorax,
even lobectomies) and generally has similar results and
quicker recovery than old-school open lung surgery, the
video camera's resolution is such that it's hard for doctors
to see small lung nodules (one five-year old paper I saw
said 10mm and down, which surprised me) unless they know
what they are looking for. In the case of
early-stage sarcoma lung mets, in which there might be
dozens of unscannable miniscule nodules, the gold standard
for detection/resection is still opening a large incision,
spreading the ribs and allowing the surgeon to take every
bit of your lung in his or her hands and meticulously feel
for lumps and bumps. I'll pass on that one for now.

And then I had chemo, and it was routine, except for the
usual exhaustion, and the shortness of breath, and then
I... Sorry.

Lumps and splits

I'm just trying to get up to date here before I start pontificating about other topics again. 

After the awful pleurodesis and overenthusiastic pain management, I recovered from the various chest tubes and antibiotics and went home for a while before beginning chemotherapy for the second time. After much deliberation, I chose the relatively toxic gemcitabine-docetaxel (gemzar/taxotere, or gem/tax) regimen I successfully took the previous year rather than the relatively tolerable temodar (temozolomide) recommended by my local doctor. I received very little help on making the decision; an e-mail to the NY doctor, the gem/tax proponent, wasn't returned, and the case he made to my local doctor in favor of reusing the drugs wasn't something that she could or would clearly articulate. 

I decided on gem/tax because I wanted to pursue what I believed was the more aggressive treatment, and because new scans and showed the disease swimming through my retroperitoneal lymph nodes. The plan was to split the dose of taxotere, giving one half the first week of treatmentand the second the following week. The third week of each cycle would be for rest. I tolerated the first infusion of the drugs well, returned the following week for another infusion, and discovered that my lung had collapsed AGAIN. 

I was admitted to the hospital, and met a new pulmonologist who, appropos of my sarcoma and repeated collapsed lungs, said, "I'm a lumper, not a splitter." That is, he was far more inclined to attribute the lung issues to the cancer than to split them off as something unrelated.

In the next exciting chapter, we'll explore VATS -- visually aided thoracic surgery (which requires some "splitting" alas) -- and get provisionally good news from lung biopsies. Perhaps we'll even start chemo again....

Take the meds

What I really wanted to say below was: take the meds. Ask for them if you have to — though it's always nice if a nurse comes to you and asks you. There's no shame in it, and the risk that someone who has never had trouble with substances before will develop an addiction to pain-killers in the hospital is very slight. Stoicism — here defined narrowly as not taking drugs that can make you more comfortable when you need them — is bad for body and mind and, ultimately your recovery. You'll cut them out when you feel better.

Now I'll reinforce this point with an excerpt from the New York Times and then undercut everything with a short story about my own recent hospitalization.

First let's take a whack at the addiction argument with an excerpt from the Times, from an interesting article about a pain doctor:

Virtually everyone who takes opioids will become physically dependent on them, which means that withdrawal symptoms like nausea and sweats can occur if usage ends abruptly. But tapering off gradually allows most people to avoid those symptoms, and physical dependence is not the same thing as addiction. Addiction — which is defined by cravings, loss of control and a psychological compulsion to take a drug even when it is harmful — occurs in patients with a predisposition (biological or otherwise) to become addicted. At the very least, these include just below 10 percent of Americans, the number estimated by the United States Department of Health and Human Services to have active substance-abuse problems. Even a predisposition to addiction, however, doesn’t mean a patient will become addicted to opioids. Vast numbers do not. Pain patients without prior abuse problems most likely run little risk.

With this is in mind, and lungs agonizingly encased in concrete, my initial attitude to pain medicine after the pleurodesis was similar to James Brown's approach to funk: bring it to me.

Patient-controlled analgesia? Check. IV narcotics, check. Oral stuff for breakthroughs, check. I took what they gave me, and they gave me a lot because I was in really difficult pain and the hospital staff from attending physicians down believe strongly in keeping patients, especially cancer patients, comfortable.

This regimen, indeed, made me hurt a whole lot less. 

But it also melted reality. 

I talked little. I had few coherent thoughts. When I lifted a glass to my lips for a quick sip of water, I'd fall asleep mid-rise and wake when the cold water splashed all over. Instead of being "behind the pain" with the medicine, I was ahead of it – and it took a disconcertingly long time to back the drugs off and catch up. At one point, I was possessed with an overwhelming desire to sleep and a sense, possibly accurate, that if I let my head drift down the six inches or so to my pillow and stopped straining to keep my eyes open, I would awaken surrounded by a resuscitation team. It was a creepy sensation, feeling like I had to stay awake to stay alive.

So, yeah, we'll call this episode a pain management failure. I ended up alive, straight, and with a budding pneumonia in one of my lungs; courtesy, probably, of aspirating a little food or water into the lung during one of my junkie nods. Annoyingly, a few of the staff were a little punitive about the whole thing, a little finger-waggy.  Our reaction was, "Hey, we weren't begging for this stuff. You urged us to manage the pain." But despite the shocking news that taking a lot of narcotics in a short period of time has a downside, my experience and reading tell me that almost everything about medical culture in the US is still geared to undertreat pain rather than take it too seriously, and that making the hurting stop (or, more realistically, slow down) has few practical downsides. So take the meds, tough guy or gal, just silence your inner James Brown.

Rate your pain on a scale of....

New-school doctors and nurses sometimes call pain "the fifth vital sign" but the only way to measure it is by asking. And the question they usually ask, at least around here, is something like, "Please rate your pain on a scale of 1 to 10, with one being no pain at all and 10 being the worst pain you can imagine."

L. finds the question ridiculous. It launches her on a series of digressions that, paradoxically, might serve to distract a little bit from the pain. You can see how it goes: Hmmmm…. The worst pain you can imagine, not the worst you've experienced… What is a drill slowly turning through the top of your foot? Having your fingernails pulled out one by one? Childbirth? A limb being hacked off with a dull spade?

Somehow that 10 end of the scale is an invitation to weird speculation, and the little faces that sometimes accompany the scale aren't much help for clarifying matters. The face is smiling at stage 1 — but also stage 2. If he's hurting, why is he smiling?

But the scale, even the little cartoons, has meaning – there are big norms around that little scale and, more relevant to the hospital patient, your self-rating plays a role whether you will get pain medicine and what kind you will get. (To some extent. A nurse isn't going to take a nine seriously if you're lolling back on your pillows riveted to Top Chef.) So it makes sense to try and understand the norms underlying the faces so that you can rate your pain in terms that don't over- or understate reality ("For me a 10 is being doused with gasoline and lit on fire, and I feel much better than that even though I can't move from the fetal position, so my pain is a 4." Nice logic, but for the less hardy souls the nurse sees every day, this is more like a 6 or 7, so you haven't helped yourself out.)

This mashed up, multilingual scale from UCLA can help calibrate your internal pain benchmarks, even as it robs one of the perverse sport of speculating on the worst pain you can imagine. In this scheme, pain of five or above is pain you can't get off your mind; the clinical goal would probably be to medicate it down to 3-4 territory, where the pain is annoying and might stop you from, say, getting up but isn't on your mind constantly. The scale also offers a less apocalyptic view of 10: there's crying or moaning, but no crackle of gasoline flames or thunk of a dull spade. This is preferable, I think

More is how you live...

Long silences on cancer blogs are bad. Not as bad as the e-mail titled "Down for the Count" I once sent a close friend, but bad anyway. The day after I'd exposed my little nicks and cuts from chest tube misery -- before, even, I'd managed to take a shower -- I went to work and started feeling a little funny. A chest x-ray seemed smart. So I left work, got the X-ray, and checked into the hospital. Another pneumothorax to add to my collection of pneumothoraces.

*

It's been a while, and I can't really get into the details of the hospital stay now because other stuff has happened, but the whole experience was tough. The standard treatment for recurrent pneumos is a procedure called pleurodesis. 

Backing up, your lungs are essentially double bagged. In between the two "bags," or pleura, is an open area called the pleural space. When a lung "collapses," this generally means that it springs a leak and starts shooting air into the pleural space, raising the pressure in such a way that the lung can't reexpand. But close the space by sticking the inner "bag" around the lungs to the outer "bag" attached to the chest wall, and your problems with pneumothoraces are, hopefully, over.

Problem is, this process sucks. The simple way to do it is to get a bit of morphine and then have a doctor inject a small amount (50ccs or so) of talc slurry through your chest tube into the plural space. You swish the talc around by lying on your bed for a while, then, a few hours later, an inflammatory process starts that closes the pleural space. This hurts a little, but you manage it with analgesia. In my case, the process went down like this: Morphine, OK; talc, oh my god! I felt as if a concrete slurry was being pumped into my lungs, hardening around them instantly and choking with within. The pulmonologist tried to back off a few ccs as I started thrashing and weezing and hurling imprecations in a classic respiratory-impaired-bad-guy voice. Then, somewhat stunningly, the dude leaves! The concrete continues to harden and block my breath, and LA asks, "Do you need help?" And I sort of choke out an affirmative.

And this is where the doctors and nurses begin "managing my pain." This is a key principle in the onc ward in the cold state, one that I am greatly appreciative of. The staff is good at it, and I never really thought there was a downside to, you know, not being pained. But reality is more complicated than that, as I'll explain in a bit.

Anticlimax

So last night it was time to change the dressing covering the incision where they stuck the tube that removed the air from my chest and allowed my lung to reinflate itself. I'd rate myself as middling on the sensitivity-to-gore scale -- a PG-13 guy in an R-rated world. My blood and I just don't get along any more. So it was at the last permissible hour, and with a good deal of trepidation, that I peeled off my shirt and began working on the thick layers of white tape that swathed my thorax like an umpire's chest protector. 

At first, I'm nervous. Then, trying to work the maze off centimeter by centimeter, I start getting pissed off. The stuff has practically bonded to my skin, and there's a lot of it; yanking on it just painfully pulls my gristle into a hump, so I have to get in underneath it with the fingers of my off hand and work it to the side so that the other hand's pulling removes tape instead of flesh. This takes a long time and manages to be simultaneously painful and tedious. When I've finally freed the multiple straps holding down the thick wad of gauze, I yank the whole thing off and look up at the mirror, worried about what I'll see.

I look, then look again. There is a crusty smear of dried blood, a small yellowing bruise, a few crisscrossing straps of lobster-red skin, and... nothing. I'm standing there with a couple of squares of yellow petroleum gauze stuff, trying to figure out where to slap it down, and the only plausible candidate is this spider bite, a sorry looking little red scab. This is it? This is the sum total of two procedures, six milligrams of morphine and, initially anyway, a considerable amount of discomfort. Well, yes. I apply the jelly and gauze and fasten it all down with as little tape as seems prudent.

Sarcoma and Smart Drugs

This article from Harvard Magazine tells the story of a man with a particularly ugly form of sarcoma called GIST and his treatment with so-called "smart drugs," agents that destroy tumors by targeting the specific proteins in malignant cells, rather than just mowing down everything growing a la conventional chemotherapy. The patient happens upon the right man, Dana-Farber's Dr. George Demetri, at the right time, just as trials of the smart drug Gleevec are primed to begin.

A caveat on the piece is that it is written by a former president of Dana-Farber, so it's not objective. It doesn't end well, either. But it melds a moving human story with insight into the science behind smart drugs and some of the medical and ethical issues presented by drug trials. It also briefly discusses dasatinib, a Gleevec-like agent that is currently being assessed for action against epithelioid sarcoma in a new study. I'll have more on that soon.

A note on studies

Anybody can type a few keywords into PubMed and have great fun worrying oneself and annoying one's doctor. On the positive side, even if you can't understand the bulk of what is written, knowing about a potential treatment's possible effects and side-effects can make it easier to make a difficult choice.

I am not an expert at evaluating studies, but here are a few tips:

1) Check the sample. More, obviously, is better, though most soft-tissue sarcoma studies involve small numbers of patients due to the rarity of the disease. Remember that in a small sample, even one additional response can skew the number in the absract dramatically. I tend also to be biased toward reports from major American and European cancer centers, as well as those published in big journals like Cancer.

2) Read -- OK, skim -- the whole thing. Most libraries, especially at universities, can access medical papers via their institutional subscriptions. (You can, of course, also find many on paper.) The biggest reason to do this is that soft-tissue sarcomas, which come in at least 66 very different flavors, are quite a diverse group, and what is applicable to one might not be applicable to yours. Usually there's a chart that breaks down the sample by disease and response where you can look for what happened to patients with epithelioid sarcoma, rhabdomyosarcoma, or whatever.

3) Check the date. Both when the study was published and when the patients were actually enrolled and treated. This is particularly crucial for so-called prognostic studies that tell you how other people with your disease did. To gather a statistically valid series of outcomes, the researchers may have to aggregate records going back decades. The progress of cancer treatments is overrated in many ways, but there's no point in worrying yourself sick about what happened to a small group of people 10 or 20 years ago in another country. Things have gotten better.

4) Bring your doctor in. Print at least the abstract, ideally the whole text, of anything that particularly worries or excites you. Then get a copy to your doc so she can help you evaluate it. It's safe to assume that your doctor is quite familiar with the clinical literature (if you don't feel this way, find another oncologist), so it's not like you're delivering news flashes, but sarcoma is rare enough that she might not have seen everything, especially if she spends most of her time treating other illnesses. I would use this tactic sparingly, out of respect for your doctor's time and expertise, but I think most physicians would rather spend a little time helping out occasionally rather than having you base decisions on information that they don't know you have, information that may not be fully relevant or up-to-date.

I'll add to this as I gather more thoughts that might seem helpful.