X Marks the Spot

In The Emperor of All Maladies, Dr. Siddhartha Mukherjee wrote, “[T]he statistical confluence of a rare profession and a rare tumor [can] swiftly identif[y] the causal agent of [a] cancer.” He was writing about scrotal cancer in child chimney sweeps in England in the 18th and 19th centuries, and about mesothelioma in asbestos installers in America in the 20th century–but the scientific method behind this statement holds true for any form of cancer in populations of all kinds. If you find multiple cases of a unique cancer in a unique population, with enough data to crunch, you have a chance at identifying a cause. The cancer and the population make a crossroads of sorts–a big “X marks the spot” on the map of cancer. Dig deeply enough at that location, and you just might find treasure.

Cigarette smoking comes to mind. Smokers have a profoundly higher risk of lung cancer than any other unique population (with the possible exceptions of firefighters or miners). But, as Dr. Mukherjee also points out, the problem with identifying a widespread carcinogen is the fact that it’s widespread. Its prevalence becomes noise rather than signal. So many people smoked for so many generations in America that it took horrific numbers of lung cancer deaths to overcome our cultural blindness toward the habit. Once we did figure it out, though, we started educating people about this (perfectly avoidable) cancer risk, and it’s been working: the number of lung cancer deaths due to smoking are already beginning to fall. But we had to have that confluence, that big fat X, shoved right into our faces before we could make the change.

Well, as the news stories began to trickle in on Wednesday May 2, 2018, I was thinking heavily about OM and its statistical confluences in the larger American population. Fortune.com blared this story:

A Rare Eye Cancer Has Started to Appear in 2 States

CNN took up a sizeable chunk of front-page real estate with a full-size thumbnail and a video report, shouting:

38 Auburn Alums Mysteriously Diagnosed with Rare Cancer

Now, anybody in the OM community was probably rolling their (tired and damaged) eyes at the tone of these headlines. “Started to appear”… “Mysteriously”… Seriously? Clickbait much?

Then I read and listened to the contents of these news stories, and I got even angrier. I wasted a fair amount of time weathering a wave of unhelpful cynicism. It took me a day, but I managed to boil down all my (very personal) objections to a single, critically important word:


OM hasn’t “started to appear” anywhere, and certainly not in just two states. This is not an e coli outbreak. This is cancer. OM exists in every single one of the 50 states, and has existed in those 50 states (and all countries of the world) long before yesterday. Just because very few Americans have ever heard of our cancer (including me, up to two years ago) doesn’t mean it hasn’t existed as long as humans have had eyes. OM’s been known as OM, an identifiably unique cancer, for two centuries.

That’s a bigger deal than a lot of people realize.

It’s been only in the last few generations that any form of cancer has been reliably, precisely, identified as cancer. Many of cancer’s effects on the body have been misidentified as various other illnesses instead, leading to no end of misdiagnosis, mistreatment, and misery. If you have grandparents (which we all do, or at least did at one time), you have a very good chance of having seen this problem firsthand: if you asked said grandparents what their parents died of, the answer probably came back impossibly, uselessly, hazy. “Oh, he had gut trouble.” Um, okay, what did Great-Grandpa have? Crohn’s? Pancreatic cancer? A ruptured appendix? Leaking ulcers? Colon cancer? A gall bladder infection? “Oh, we don’t know.”

It can be wildly frustrating to live in an era that has learned to compile multiple lines of intense, precise information to name a single case of breast cancer, while every generation before us has been unable to say whether cancer was even the correct diagnosis. I feel for adopted people, who worry themselves sick over having no medical history from their biological families. That being said, so many folks who grew up with their biological families still have no idea what their medical histories actually are. (This is how close we remain to the Dark Ages, folks: just a few short generations of correctly-handed-down, precise information.)

Need an example? Take breast cancer. Every case of breast cancer can now be described by multiple lines of information, each line representing a progressively deeper level of study and experimentation that required years of research and funding and misery to gain:

  • Invasive or non-invasive?
  • Sarcoma or carcinoma?
  • Ducts or lobules?
  • Estrogen positive?
  • HER2 positive?
  • Triple-negative?

But while OM has been recognized and known for a lot of generations, we’re still stuck with the main problem of precisely classifying it in order to precisely treat it. OM barely has one line of description behind its name, and that one line took decades of incredibly difficult and delicate work to arrive at. And we OM patients literally live and die by that very first level of understanding every day:

  • What Type is it?

In all honesty, “Type” can be described as, “Is the tumor very likely to metastasize or not?” And you don’t have to be a cancer researcher to realize just how basic a criterion that is. It’s “learning the alphabet” basic. It’s “let’s see if cutting out the entire eye will help” basic. Imagine if breast cancer were still being treated solely at the level of “is it invasive”! We’d still be doing radical mastectomies for a single lump, and chemo, radiation, targeted therapies, and immunotherapy wouldn’t have been invented yet. The only treatment would be “cut it off and hope for the best.” Up until plaque brachytherapy was applied to OM just a few decades ago, that’s all OM patients had available, too. Things like lasers, gamma knife, and even Type testing were nothing more than theories just a decade or so ago. The clinical trial I’m part of is attempting to identify a single adjuvant treatment that might work…because treatment of metastasis once it happens has an abysmal failure rate. Keytruda (the immunotherapy developed for metastatic cutaneous melanoma) might offer a Hail Mary chance for survival of metastatic OM, but honestly, if you look at the knowledge derived strictly from and for OM, all we’ve accomplished in the last two centuries is saving some sight in the affected eye, and getting better at identifying who’s at the greatest risk of dying from OM. At its most reductive, the treatment and survival arc for OM1A and OM1B is, “Treat the eye and go about your life a bit more mindfully, because you dodged a bullet.” At its most reductive, the treatment and survival arc for OM2 can be summed up like this: “Live until some unknown power decides it’s time for you to die, because the existing science won’t do much to change the outcome.” That’s how basic and important Type is.

Let me be clear here: I’m not at all complaining about the speed or quality of the work being done. The people working on OM are very literally world-class physicians and researchers. People on the other side of the planet pay my oncologist hundreds of dollars an hour just to do Skype consultations on their cases. (As one OM patient put it, “Dr. Sato is a rock star.” And he wasn’t joking in the slightest.) The fact that we’re just now arriving reliably at Type, and barely scratching the deeper levels of understanding, is a testament to the complexity of the problem. And I’m grateful beyond words that OM patients all over the world can benefit from treatments and knowledge gained for other cancers. Without the incredible work being done both for my kind of cancer and others, I wouldn’t have had a clinical trial to be part of. I wouldn’t have had plaque brachytherapy to save my eye. I’d have no chance at all in the face of a possible metastasis. I’d have lost my eye, then died in just a few short years with no hope of survival. (I still might die anyway, but like I said, it’s a testament to the complexity of the problem, not an implied lack of quality in the research or the people doing it. Cancer is just that much of a bastard.)

What I am complaining about, however…and at volume…is the lack of precision in the reporting. Because precision. Fucking. Matters.

Look at a quote from that first article:

“Three friends who attended Auburn University in Alabama have all developed the same rare eye cancer — ocular melanoma, an eye cancer that typically only affects six in every 1 million people.

If that wasn’t strange enough: One of the girls created a Facebook page, where she found 36 more people who have been diagnosed with the eye cancer — 18 in Huntersville, North Carolina, CBS News reports.”

Compare that to the CNN title: “38 Auburn Alums Mysteriously Diagnosed with Rare Cancer.”

There’s enough wrong there to make a logical mind break into a blithering, spit-flying hissy fit.

  • Are there 36, 37, 38, or 39 cases?
  • Are these patients all Auburn alumni, or is that (utterly fucked up) reported total number of patients actually spread out over both Alabama and North Carolina?
  • Are all these patients OM2, or is it a mix of Types?
  • Did they all develop OM while concurrently at Auburn/living in Huntersville? Or did they develop it later, and have researchers just now caught on to a similarity in their pasts? These “girls” weren’t college kids, but middle-aged women. So when, precisely, did their OM develop?

Just from that one quote and one headline, you’re talking about the difference between a statistically expected number of OM patients–because I’m pretty sure there are more than six million people in the states of Alabama and North Carolina combined–and an “X marks the spot” moment for a unique, previously untreatable form of cancer…and that moment is about to result in a serious body count.

Precision. Fucking. Matters.

As it happens, I have personally talked with two of the research assistants who work with Dr. Orloff, the interviewed researcher, at TJU. And they patiently explained to me that the possibility of a cluster in North Carolina or in Alabama is just that…a possibility. Is there something nefarious at work in Huntersville, NC and at Auburn in AL that is cranking out a disproportionate number of OM cases? Absolutely, it’s a possibility. And while I wish OM on absolutely no one, ever, all us OM patients could pray for that “X marks the spot” moment. Because God Almighty, we could use a break in the case…preferably in time to save those affected by that cosmic X.

But it’s also a possibility that the reported cases form lumps in a single area because, 1) they’re occurring over time, not concurrently, or 2) because there’s only one doctor in the entire state who handles that kind of cancer. Researchers in OM see this happen all the time. If you go strictly by cases reported in a state, it’ll look like there are clusters of OM in practically every state in the US, because most states have. Only. One. Doctor who specializes in OM. Some states have none. So the CDC will never see an OM case from those states. Does that mean those states have no OM? Nope.

Precision. Fucking. Matters.

Right now, we have a huge amount of noise being generated by anecdotal evidence from Facebook and mainstream media. But what we need is signal. We need precision.

Much of the science done on the possible causes and metastasis of OM was completed before Type was discovered. Specifically, a trial involving blue light as a possible cause of OM ended with mixed results. Unfortunately, because we have no idea what Type the tumors were that were seeded into the eyes of various rabbits, we have no idea if those rabbits’ tumors metastasized because they were exposed to blue light, or because they were seeded with a Type 2 tumor. This wasn’t a mistake due to sloppy work; we simply lacked the information needed to make a better experiment at the time. But until that research is redone as precisely as possible, blue light can’t be ruled out (or in!) as a cause…and the patients of Auburn and Huntersville can’t be either identified as harbingers of a potential environmental cause endemic to those areas…or revealed as statistical blips in a vastly larger problem. Can you imagine the fallout if blue light from every backlit screen in America is identified as a cause of ocular melanoma? Like smoking, the only way we could identify such a widespread carcinogen is from a pileup of bodies. I’d much rather that not happen. I’d much rather Auburn become an “X” that can lead directly, precisely, to a different environmental cause that’s far more eradicable. But we can’t know that while Facebook and the media are muddying the waters.

Precision matters.

Even today, doctors who know better occasionally throw themselves (and their patients) under the bus by using sloppy terminology. The word “lesion” is used as the traditional name of both a cancerous tumor and a demyelination of the spine caused by an autoimmune response. A doctor asked me if I had lesions anywhere in my body other than my eye, and I answered yes, two in my spine–and his eyes about fell out of his head. I saw the words “death sentence” fly across his face. I had to correct him: demyelinating lesions, Doc, demyelinating lesions. Scars, buddy…not death sentences. Even OM itself is a sloppy term. “Ocular” is more recognizable to the general public than “uveal,” which is why I use it. And even doctors get confused by the “uveal” part of “uveal melanoma”–I’ve had to specify, more than once, to a doctor that my “uveal melanoma” was not a uveal melanoma, but specifically a choroidal melanoma. I can’t help it that even doctors sometimes don’t get their stuff straight.

Precision matters.

Now is it possible that I’ve gotten some facts wrong, or misunderstood some of the science? Absolutely. I’m just a writer who’s had to research something I never wanted to know about, and I’m doing it while under emotional duress. But while under duress I could point out big, big gaps in supposedly credible reporting. And I trust the news generally. What am I supposed to believe now?

Precision matters.

I pray to God that these news stories get cleaned up and clarified, and soon. We need reporting that isn’t going for the shock value and clickbait, and instead goes straight to the heart of the matter. Because some of us…potentially a lot of us…have an awful lot riding on it.


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