Pandora's DNA: Tracing the Breast Cancer Genes Through History, Science, and One Family Tree

by Lizzie Stark

  From then on, she kept her eyes shut so hard “that the Eyelids seemed indented into the Cheeks.” For a moment, she thought they were done, but the cutting resumed. “Dr. Larry rested but his own hand, &—Oh Heaven!—I then felt the Knife [rack]ling against the breast bone—scraping it!—This performed, while I yet remained in utterly speechless torture.” She passed out at least twice from pain during the whole thing and could not speak of the operation for months. It’s no wonder that even a single question about her ordeal “disordered” her. “Even now,” she wrote, “9 months after it is over, I have a head ache from going on with the account! & this miserable account, which I began 3 Months ago, at least, I dare not revise, nor read, the recollection is still so painful.” The operation lasted only twenty minutes, “a time, for sufferings so acute, that was hardly supportable.”

  The procedure was hell on her husband, who must have found out about the operation as it was happening. He added a few lines to her letter: “No language could convey what I felt in the deadly course of those seven hours.” Reading Fanny’s letter affected him. “I must own, to you, that these details which were, till just now, quite unknown to me, have almost killed me, & I am only able to thank God that this more than half Angel has had the sublime courage to deny herself the comfort I might have offered her, to spare me, not the sharing of her excruciating pains, that was impossible, but the witnessing so terrific a scene, & perhaps the remorse to have rendered it more tragic. for I don’t flatter myself I could have got through it—I must confess it.”

  Fanny allowed seven men to do this to her, and she faced the horrific ordeal with unimaginable courage. She’s a martyr and an amazon. The miracle of this surgery, in an era without antibiotics, is that she survived the operation and didn’t die from postsurgical infection. And her cancer—if indeed it was cancer and not a benign lump—did not recur.

  Frances Burney’s story, the story of how we used to treat cancer, is one of surgical radicalism. Until the advent of chemotherapy and radiation therapy in the twentieth century, surgery represented medicine’s main tool for treating breast tumors. The Edwin Smith Papyrus, which proclaimed breast cancer as having no cure, also recorded the cauterization of an identifiable breast tumor with an awful-sounding tool called a fire drill. According to a description written in 1296, Leonidas of Alexandria dealt with breast tumors by using a knife to remove the breast, and then cauterizing the wound. The ancient physician Galen was picky about which tumors he’d carve out—they had to be easily accessible. He also cut widely around the tumors to ensure he removed all of the mass—a practice that ensured clean margins—and he eschewed burning surgical sites because it damaged surrounding tissue. In eighteenth-century Europe, surgeons used horrible devices—bladed rings or pairs of blades—to cut off breasts swiftly, a procedure that often led to hemorrhage and disfigurement. From the late 1700s through the end of the following century, science sped along at a fast clip, although advances were patchy at best—known in certain areas of the world, but not others.

  Japanese surgeon Seishu Hanaoka, for example, developed and experimented with anesthesia on his wife and his mother more than forty years before the West started investigating the field. In 1805 he put a woman under and performed a mastectomy—quite possibly the world’s first painless breast removal—and by the end of his career he’d performed 150 of them. Unfortunately for the rest of the world, Japan lived under its sakoku policy of isolation at the time, which prevented the spread of medical breakthroughs. Meanwhile in France, surgeon Jean-Louis Petit published a work on mastectomy that recommended the removal of breast, lymph nodes, fat, and part of the pectoral muscle—a Halsted before Halsted.

  All of these developments, combined with advances in anesthesia and the discovery that surgery required sterile conditions, set the stage for the radical operations of both William Halsted and William Meyer, published separately in 1894. Both operations removed breasts, lymph nodes, and different sections of pectoral muscles but detached items in a different order. Halsted’s meticulous method took four hours, while Meyer slashed time by using scissors for blunt incisions.

  The brutal procedure became the preeminent breast cancer treatment for the next half century. It saved women’s lives but left them stoop-shouldered and with limited arm mobility. The Halsted mastectomy is a relic of its era, when surgery was often meant to be palliative, not curative. Doctors saw plenty of late-stage tumors—many likened them to various sizes of bird’s eggs—cases probably so far advanced they’d be incurable even by today’s standards. Rather than let tumors ulcerate and burst through the skin, giving patients miserable, pained demises, doctors whacked off breasts as a humanitarian effort. That Halsted’s mastectomy was able to improve the relapse rate to a mere 52 percent was extraordinary. Given the sorts of cancer he saw, it’s understandable that he believed it to be a local disease with a local cure.

  The upswing of less drastic procedures, such as lumpectomy during the twentieth century, is not merely the story of scientific advancements, such as chemotherapy and radiation. It’s also the story of women rising up and demanding breast-conserving therapy; it’s the story of how women’s lib upended the relationship between mostly male doctors and their female patients.

  In our culture, breasts have significance in a way that, say, the spleen does not. They are visible and eroticized markers of femininity, so breast surgery necessarily engages with our ideas of gender. As cancer culture historian Ellen Leopold points out, historically, the patriarchy has structured the relationship between mostly female patients and mostly male surgeons. She writes:

  At its most reductive, the aura surrounding breast surgery reinforced the worst gender stereotypes, attributing all power to a male hero and all frailty to a damsel in distress. The surgeon was alert, erect, and skilled, and the patient, asleep, supine, and helpless—that is, without animating or humanizing virtues of any kind. Life-saving surgery, in other words, seemed to require the total degradation of a woman’s spirit as well as of her flesh. This abasement, so integral to the surgical ordeal, was to color every aspect of treatment for most of a century.

  Many early-to mid-twentieth-century practices for treating and talking about breast cancer prove Leopold’s point. As she discusses, in the 1960s the authority of mostly male surgeons was unassailable. Surgeons could also decide whether to tell patients the truth about their conditions—doctors too had trouble choking out the word “cancer.” One survey from 1961 found that 90 percent of physicians did not tell patients the truth—that they had been diagnosed with cancer—preferring euphemisms like “mass,” “lesion,” and “tumor.” Doctors themselves viewed cancer with hopelessness and did not like delivering tough news; they also wanted to shield patients from feeling hopeless after a diagnosis. Interestingly, while doctors overwhelmingly preferred not to tell patients about their cancer diagnosis, patients—89 percent of them—overwhelmingly favored knowing their own diagnosis, according to a 1950 study. This inequality in knowledge affected breast cancer treatment. As Leopold points out, “The fact that her surgeon was unable to communicate the results to her [the patient] directly did not deter him from acting on them unilaterally, that is, without her agreement.” In the early 1950s, breast cancer survivor and advocate Fanny Rosenow called the New York Times to post a notice for a breast cancer support group she was starting. Her call ended up routed to the Times’ social editor, who greeted her request with a pregnant silence. “I’m sorry, Ms. Rosenow, but the Times cannot publish the word breast or the word cancer. Perhaps you could say there will be a meeting about diseases of the chest wall.” She hung up, disgusted, but through the persistence of Rosenow and her fellow survivor Teresa Lasser, the organization Reach to Recovery was founded, a program my grandmother later participated in as a cancer survivor, visiting other patients in their hospital rooms to talk about what to expect. Public discussion of breast cancer in obituaries, for example, also garbed breast and ovarian cancer deaths in euphemisms such as “women�
��s cancer,” or “prolonged illness.” The culture seemed embarrassed about cancer in general, but about breast cancer and other women’s cancers in particular—it silenced women, denying their experiences to each other, refusing even to tell them the name of the malady killing them. The heroic Halsteds of the world rode in on their white horses and carved out internal organs. It was viewed for so many years as the only way. Even after chemotherapy and radiation became available, until the mid-1970s it was standard practice to put a woman out for a breast biopsy and then remove her entire breast in one go if the tumor tested positive to avoid the inconvenience of putting her under general anesthesia twice. These breast biopsies mirrored the larger struggle around women’s rights in the 1960s and 1970s. Who should have dominion over women’s bodies—the women themselves or the male doctors who thought they knew best?

  So let us sing the praises of journalist, breast cancer activist, and cancer patient Rose Kushner, who went after the one-step biopsy practice in the mid-1970s. In 1974 she developed cancer, and she wanted some time between biopsy and breast removal to decide on a course of action. She had to visit nineteen surgeons before she found one willing to biopsy her tumor but not remove the breast. Eventually, she testified before NIH on the matter, arguing that a two-step procedure separating mastectomy from testing would allow surgeons to better assign stages to cancer and offered women the option of making up their own damn minds. After all, it wasn’t the surgeon’s life at stake but his patient’s. Now, the two-step biopsy procedure is the worldwide standard for treatment. As it turns out, Rose was a friend of my cousin Kathy, who participated in her uprising, attending basement meetings for her new patients’ right groups in the early 1980s. Thanks to the advocacy of Rose and other women in the movement, my mother had a day to figure out what sort of treatment she wanted—a huge psychological improvement over my grandmother, who suffered great trauma from waking up after a biopsy with a brutal Halsted mastectomy.

  The same year that Rose Kushner developed breast cancer, so did Betty Ford, who brought breast cancer out of the closet by candidly discussing her diagnosis and mastectomy with the press. She was not suffering from a “woman’s cancer.” She had breast cancer. B-r-e-a-s-t cancer. She spoke the unspeakable words in public, giving interviews to reporters and letting them photograph her in the hospital, and that changed everything. As a 1987 New York Times piece—written thirteen years after Ford’s diagnosis put it, when Ford got cancer, “many Americans still considered mastectomy a taboo subject, too fearful or even shameful to be discussed openly.” Within a few weeks of Ford’s diagnosis, the New York Times reported a four-to tenfold increase in women requesting screening at the American Cancer Society’s twenty-seven free centers. According to her 2011 Time magazine obituary, the former First Lady’s diagnosis set off a screening tidal wave so large that “the reported incidence of the disease rose; some researchers even called this the Betty Ford blip.”

  Forty years later, in 2013, Angelina Jolie would do the same thing for women with BRCA mutations by writing about her preventive mastectomy in a New York Times editorial. Her piece and the ensuing media coverage did several things for BRCA carriers. For starters, BRCA mutation carriers no longer have to preface conversations about surgery with a lecture on genetics and risk. Now, women can simply say, “That thing Angelina did? I did it too,” and the average dinner-party attendee will understand. Second, having a cancer-causing BRCA mutation is pretty complicated, and the breast-removal option sounds draconian until you start thinking through the bigger issues. A lot of people have called BRCA women who elect to have mastectomies crazy. That a big-name celebrity made this same decision in consultation with, presumably, some of the finest doctors money can buy, is a powerful vote in its favor. And although the long-term effects of her announcement are not yet known, a Washington Post article published a few months after her op-ed found that DC-area genetic counselors were fielding twice as many calls about genetic testing. Joy Larsen Haidle, presidentelect of the National Society of Genetic Counselors and a practicing counselor herself, told me, “There has definitely been a Jolie effect.” She explained that immediately following the announcement, she and most of her colleagues “received a dramatic increase in calls asking about the breast cancer genes … and for many of us that trend stayed elevated for several months afterward.” A Harris Interactive poll performed in August of that year found that 86 percent of women had heard about Angelina’s mastectomy, with 5 percent saying they would seek advice about their breast and ovarian health.

  Unfortunately, the idea that women might not be smart enough to make their own medical decisions is still around. After Jolie’s announcement, the media did publish some hysterical, reactionary articles that insulted women’s intelligence—will more women get unnecessary mastectomies after Angelina Jolie? It’s a major medical procedure, not a manicure. If Idris Elba had his testicles removed for a really good reason, do you think men would jump at the chance to be just like him? How stupid do they think we are? Some of the articles confused different concepts of preventive mastectomy, conflating the removal of the remaining healthy breasts in women who had already had cancer (contralateral prophylactic mastectomy) with the removal of both healthy breasts in high-risk women with BRCA mutations, and with contralateral mastectomy in women with BRCA mutations who had previously suffered breast cancer. What science we have shows that prophylactic mastectomy in BRCA women prevents breast cancer. To compare this group to the general population of cancer survivors is misleading and inaccurate.

  Still, like Betty Ford and breast cancer patients, Jolie has brought BRCA patients out of the closet. And back in the 1970s, as it became more acceptable to talk about breast cancer, the women’s lib movement also made strides in allowing women to assert sovereignty over their own bodies—for example, the right to abortion guaranteed by Roe v. Wade in 1973. As women demanded control over themselves, the relationship between doctor and patient changed; doctors no longer held a position of unassailable authority over patients. Rose Kushner and fellow journalist Betty Rollin published pieces questioning the necessity of radical mastectomies and heckled surgeons at medical conferences about how radical surgery had never been properly tested in a controlled environment.

  In 1971, the Halsted mastectomy had its eightieth birthday, the anniversary of Halsted’s first description of the procedure. And that year represented the beginning of the end for that operation. Spurred on by patient activism, surgeon Bernard Fisher (known for the wonderful saying “In God we trust. All others [must] have data.”) began studying lumpectomy and radiation. He spent ten years gathering data and found that lumpectomy—a breast-conserving operation in which the tumor and some surrounding tissue are excised—plus radiation worked just as well as radical mastectomy. His research represented the formal fruition of the studies that Geoffrey Keynes had first embarked upon after World War I.

  However unwelcome they are, tumors come with a degree of certainty. If you have a tumor in your breast, doctors will probably try to cut it out, and if the cancer has spread to your lymph nodes, those might go too. The problem has a location, a physical manifestation that may be pinpointed. A tumor presents a clear problem; your risk for cancer is no longer vague. It’s actualized, solidified, and therefore terrible, as terrible as the methods of treatment—surgery, radiation, hormone therapy, and chemotherapy—treatments a blitz of scientific studies has evaluated.

  Take away the tumor—take away the cancer—and only the vagaries of risk remain.

  For much of the twentieth century, medicine put a whole host of women into the “at high risk for breast cancer” category, prescribing mastectomies despite the fact that their effectiveness in preventing cancer had not been scientifically studied. That’s not too surprising, considering Western medicine’s troubling history of removing lady parts with only slight justification.

  Since surgery became tenable after the introduction of antisepsis and anesthesia in the second half of the 1800s, the med
ical version of the track coach’s “Walk it off” has been “Surgically remove your uterus and/or ovaries.” Doctors prescribed oophorectomy and/or hysterectomy for a wide variety of physical and psychological conditions. Irregular period? Epileptic? Nymphomaniac? Precancerous lesions detected on your cervix? Let a historically male surgeon just nip in there and remove those pesky lady parts. After all, if you’re not going to get pregnant, they’re useless, right?

  Of course, these organs aren’t useless. As we now know, the removal of ovaries has been linked to osteoporosis, hip fractures, dementia, short-term memory loss, loss of libido, cardiovascular disease, and more, to say nothing of how the loss of fertility or removal of internal organs might impact a woman’s self-image. Removing the uterus through hysterectomy—the second most common operation (after cesarean section) undertaken by a staggering one in three women in the United States—carries fewer risks, other than the complications like blood clots associated with any surgery and loss of fertility. Removing healthy organs has serious consequences.

  Prophylactic mastectomy has more than a century-long history that traces the reverse path of breast cancer surgery. Even as enthusiasm for mastectomies on cancer patients waned, the idea of removing breasts to prevent cancer in healthy women gained traction. The history of preventive mastectomy wrangles with technological advances that permitted better cosmetic outcomes and made women more willing to undergo such surgery. It also raises questions of risk and risk tolerance: How high must a woman’s risk of breast cancer be before doctors recommend such surgery? Is desperate fear of breast cancer sufficient? Or must she have premalignant changes in the breast as well? And what does precancer even look like?