I happen to be in Houston right now attending the Society of Surgical Oncology annual meeting. Sadly, I’m only about 12 miles away from the lair of everybody’s favorite faux clinical researcher and purveyor of a cancer cure that isn’t, Stanislaw Burzynski. Such is life. In any case, this conference is all about cancer and how we treat it surgically. That includes prophylactic surgery designed to prevent cancer in people at very high risk. Prophylactic surgery to prevent cancer is never a decision that should be undertaken lightly and almost never is, rants from quacks notwithstanding that make surgeons sound as though they’re chomping at the bit to remove random body parts in a fruitless attempt to prevent cancer.
Certainly that happened a couple of years ago when actress Angelina Jolie announced in a New York Times opinion piece that she had undergone bilateral prophylactic mastectomies (removal of both breasts) with reconstruction because she carries a mutation in the BRCA1 gene that puts her at a very high lifetime risk of breast cancer. The quacks were not pleased, not at all. For example, Mike Adams, ever the restrained one, declared Angelina Jolie inspires women to maim themselves by celebrating medically perverted double mastectomies. Hilariously, there is now a message there about how this article has been removed because it is no longer aligned with the science-based investigative mission of Natural News” and that Mikey “transitioned from outspoken activist to environmental scientist.” You can still find generous samples of Mike’s rant in my discussion of it. A followup article, How Angelina Jolie was duped by cancer doctors into self mutilation for breast cancer she never had, also disappeared. Again, you can see the crazy for yourself in my discussion of this despicable article, in which Adams claimed Jolie could have reduced her risk of cancer to very low levels with “natural” methods, an utterly ridiculous claim for a woman with a BRCA1 mutation.
Of course, BRCA1 mutations that cause breast cancer also cause ovarian cancer. Two years ago, the question remained what Angelina Jolie would do with her ovaries, and this week she answered that question with an other editorial in the New York Times entitled Diary of a Surgery. Mike Adams has shown unusual restraint (or he’s just behind on his rants) in that there is as yet no article castigating surgeons and Jolie once again for removing healthy body parts for no apparent reason. Maybe Mikey really has become an “environmentalist. (Naaah. Just take a look at his front page if you don’t believe me.) In any case, in her article, Jolie announces:
TWO years ago I wrote about my choice to have a preventive double mastectomy. A simple blood test had revealed that I carried a mutation in the BRCA1 gene. It gave me an estimated 87 percent risk of breast cancer and a 50 percent risk of ovarian cancer. I lost my mother, grandmother and aunt to cancer.
I wanted other women at risk to know about the options. I promised to follow up with any information that could be useful, including about my next preventive surgery, the removal of my ovaries and fallopian tubes.
Ovarian cancer is nasty. The main reason it’s nasty is that it’s rarely caught when it’s confined to just the ovary, because the symptoms of early ovarian cancer tend to be vague and nonspecific abdominal or pelvic complaints. Usually, it has started to spread throughout the abdomen before it is discovered, and by then the surgery needed to control it becomes much more extensive, and the chemotherapy needed to mop up the leftover tumor deposits after surgery becomes more grueling. Corresponding with this, the chances of curing it go down considerably.
Now, as I discussed before, the BRCA1 mutation Jolie had produces a 87% lifetime risk of breast cancer. Even so, some women choose close screening and then surgery only if and when the cancer develops. This is possible for breast cancer, thanks to mammography, although there is always the chance of false negatives. For ovarian cancer, such a strategy is more risky because there is no screening test even as good as mammography, even with all its flaws. In Jolie’s case, even though her CA-125 level was normal, apparently other inflammatory markers were elevated, which might be a sign of early ovarian cancer. This, according to Jolie, is what finally pushed her to make the decision to have her ovaries removed, even though she underwent a battery of imaging tests, including a combined PET and CT scan, that were negative.
Now here’s the part that bothers me. We know from her last surgery that Jolie is into a fair amount of woo. Her surgeon, after all, used a homeopathic concoction claimed to improve wound healing and had her surgery done at the Pink Lotus Breast Center, which, as I noted before, is highly into “holistic” bunkum. In her latest editorial, Jolie pulls the same thing:
I did not do this solely because I carry the BRCA1 gene mutation, and I want other women to hear this. A positive BRCA test does not mean a leap to surgery. I have spoken to many doctors, surgeons and naturopaths. There are other options. Some women take birth control pills or rely on alternative medicines combined with frequent checks. There is more than one way to deal with any health issue. The most important thing is to learn about the options and choose what is right for you personally.
In my case, the Eastern and Western doctors I met agreed that surgery to remove my tubes and ovaries was the best option, because on top of the BRCA gene, three women in my family have died from cancer. My doctors indicated I should have preventive surgery about a decade before the earliest onset of cancer in my female relatives. My mother’s ovarian cancer was diagnosed when she was 49. I’m 39.
Ack! There’s the fallacy of “Eastern” versus “Western” medicine again! How many times do I have to point out that that is a rather racist construct, in which the “East” is implied to be more “holistic” and woo-ey, while the “West” is cold, reductionist and scientific. Medicine is medicine. The only thing that matters is the evidence supporting it. Still, at least Jolie in the end made a medically reasonable and justifiable choice to have her ovaries removed in order to prevent ovarian cancer down the road.
Not surprisingly, quacks aren’t happy about this article, either. Even though Mike Adams’ “voice” (such as it is) is absent—normally, I’d have expected him to have an article out no later than yesterday, given that Jolie’s article appeared in the NYT two days ago—Sayer Ji was ready to jump right into the fray ignorantly. What he lacks in Adams’ penchant for histrionics, he makes up for in verbal prestidigitation that ignores medical knowledge. He also was fast out of the gate, throwing together this article the same day Jolie’s article appeared, entitling it Beware of Organ Removal for “Cancer Prevention”: Jolie’s Precautionary Tale. The misinformation begins early and comes fast and furious:
With Angelina Jolie’s recent announcement that she had her ovaries and fallopian tubes removed because of both a BRCA ‘gene defect’ and a history of breast and ovarian cancer in her family, the idea that genes play a dominant role in determining biological destiny and cancer risk is proliferating in the mainstream media and popular consciousness uncontrollably like a cancer.
This is what I like to refer to as genetics denialism, something very common among quacks like Sayer Ji. The idea is that you can control your health, so much so that genes don’t matter, at least not that much. EExamples abound, with Deepak Chopra attacking genetic “determinism” and Mike Adams claiming that human DNA is incapable of storing the complete blueprint of the human form and referring to genetics as a “big lie.” Others invoke the new science of epigenetics as meaning that, in essence, thinking makes it so and you can change your health just by wanting to badly enough. I hear it again and again and again. Never mind that pesky BRCA1 mutation. Sayer Ji tells you you have nothing to worry about:
The reality is that the average woman’s lifetime risk of ovarian cancer is exceedingly small, with the overall risk of developing ovarian cancer by 65 years of age being 0.8 percent and the lifetime risk 1.8 percent. For those with a first-degree relative developing ovarian cancer, as is the case for Jolie, the risk estimates show increases to 4.4 and 9.4 percent, respectively.
It is also important to realize that lifetime ovarian cancer risk does not exist in a vacuum. Considering that it is not cancer (at any site) but heart disease that is the #1 killer of women, focusing on ovarian cancer risk as the primary threat to health is myopic at best, faulty reasoning with deadly consequences at worse. If Jolie had chose to go without radical surgical intervention, it is statistically more likely she would have died from heart-related death than cancer of any kind. The reality is that the lifetime risk of heart disease related death in women is in top position at 23.5%, according to CDC statistics, versus cancer which takes #2 position at 22.1%. And within cancer related deaths in women, breast, lung, colorectal cancer, uterine, thyroid, non-Hodgkin’s lymphoma and melanomas are top on the list, with ovarian cancer in the 8th in position.
Can anyone spot the fallacies here? These are numbers for women who don’t have a documented BRCA1 mutation!!! They are irrelevant to Jolie’s situation, where she has a mutation that’s been shown to result in a 50% lifetime risk of developing ovarian cancer. The study cited was a case control study that only examined the three known major risk factors by history: a history of ovarian cancer in the mother or sister, years of oral contraceptive (OC) use, and number of term pregnancies. That’s it. It didn’t even look at the known BRCA1 mutation that Jolie had.
Next up, Ji goes on to discuss overdiagnosis of ovarian cancer resulting from ultrasound screening. Yes, as I’ve pointed out, any time you screen for a disease, you will diagnose more cases and some of those cases will be overdiagnosed; i.e., disease that would never progress within the lifetime of the patient to cause serious health problems. He even invokes the case of ductal carcinoma in situ (DCIS), a premalignant condition of the breast whose prevalence has skyrocketed with the advent of mammographic screening. Here’s the thing. The frequency of overdiagnosis is related to chance of disease in the population being screened. The lower the chance of disease, the greater the chance that a positive screening test results in overdiagnosis. That’s why we don’t screen women under 40 for breast cancer; the expected prevalence of disease is too low. Indeed, as I’ve discussed many times, even age 40 might be too young, hence all the controversy over the USPSTF recommendations five years ago.
Can you see where I’m leading? With Jolie’s expected lifetime risk of ovarian cancer being 50%, Ji’s discussion of this, too, is irrelevant to her case.
Ji concludes by cherry picking evidence suggesting that BRCA1-associated ovarian cancers might be less aggressive. Actually, not quite. One study Ji cites found that it’s likely a better response to platinum-based chemotherapy in BRCA1-associated cancers that results in better outcomes. Indeed, a recent meta-analysis suggests that BRCA1 carriers who develop ovarian cancer do indeed have higher OS, but that such women who develop breast cancer have a worse survival (which has been known). My answer? So what? They still develop a cancer that has an unfortunately high probability of killing them! Yes, the ovarian cancers they develop might be less deadly than ovarian cancer that just “pops up,” but it’s still deadly! Holy hell, Ji pisses me off with this line of “reasoning,” if you can call it that. Ditto here:
The concept – the meme – that hereditary determines one’s biological destiny is archaic. After the first draft of the human genome project was completed in 2005, they only found 23,000 genes! That’s not enough protein-coding genes to explain the existence of our body, which contains at least 100,000 different proteins. What this epic failure revealed is that it is not the genes themselves that determine health or disease, rather, what factors in our environment, lifestyle and nutrition that activate the expression of certain genes, and silence the expression of others. In the case of BRCA1 and BRCA2, we now know that silencing these genes from the ‘outside in’ results in the same result as being born with a defective gene from the ‘inside out,’ with the important difference that epigenetic – ‘outside in’ – gene silencing can actually be reversed or mitigated. Our genome results from millions of years of evolution, whereas our epigenome is influenced by day to day decisions, many of which depend on what we decide to eat or avoid eating, right now. Choice, therefore, becomes central to determining disease risk. And given that Jolie’s decision to remove her breasts, and subsequently her ovaries, was predicated on a belief that she is helpless in the face of predetermined risk – her decision does not reflect the evidence and biological science itself.
How do we end up living in a world where people believe that their genes – which they do not have access to without biomedical surveillance — determine their destiny? How we end up thinking that ‘gene defects’ are so powerful that removing healthy organs from our body is the only reasonable way to prevent experiencing cancer within them?
If we take this logic to its fullest expression, why wouldn’t someone determined to have a greatly increased risk of brain cancer remove their head prophylactically?
The stupid, it burns.
Yes, depending on the disease, it is a mixture of environment and genes that determine what happens. However, that ratio is not the same for every disease. There’s also the concept called penetrance, which describes how likely a person harboring a certain allele or mutation is to demonstrate the phenotype associated with that allele. 100% means everyone with that mutation gets the phenotype. 50% means half do, like Angelina Jolie’s BRCA1 mutation and ovarian cancer. It’s not 100% or none; penetrance is a spectrum. In the case of BRCA1 mutations, genetics does rule, by and large. For breast cancer, depending on the specific BRCA1 mutation, the lifetime risk of cancer can be as high as 90%. The same is true for a number of mutations that vastly increase the risk of colorectal cancer to the point where the vast majority of carriers develop colorectal cancer before middle age. Ji’s apparent belief that genetics doesn’t matter and “choice” is all is nothing more than what I’ve called The Secret of epigenetics, in which wishing makes it so and we can completely control our health, genetics be damned. If only that were true. It’s not.
By promoting such flagrant misinformation, Sayer Ji endangers the lives of women with BRCA mutations everywhere.