After actress and filmmaker Angelina Jolie Pitt went public two years ago with her choice to have a preventive double mastectomy to combat the risk of breast cancer, she hinted that another surgery awaited her.
Jolie, who lost her mother, grandmother and aunt to cancer, has the BRCA1 genetic mutation, which puts her at high risk for breast and ovarian cancer.
Now, in an opinion piece published in The New York Times, she has announced that last week she also had surgery to remove her ovaries and fallopian tubes.
“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,” she wrote. “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. Last week, I had the procedure: a laparoscopic bilateral salpingo-oophorectomy.”
Dr. Elizabeth Swisher, medical director of the Breast and Ovarian Cancer Prevention Program at Fred Hutchinson Cancer Research Center’s treatment arm, Seattle Cancer Care Alliance, said Jolie made a lifesaving decision.
“Women with a BRCA mutation have a 40 to 50 percent chance of getting ovarian cancer in their lifetime compared to somebody with an average risk, who has a 1.8 percent lifetime risk,” she said. “They have a huge risk for ovarian cancer and unlike other cancers, we don’t have adequate early detection. If you just wait and get ovarian cancer, the chance of dying is very high. It’s a very deadly disease.”
Swisher said studies have shown that prophylactic measures, such as the removal of ovaries and fallopian tubes, are effective in avoiding both breast and ovarian cancer.
“Women with the BRCA1 and BRCA2 mutation who get their ovaries out decrease their chances of ovarian cancer and they also decrease their chance of breast cancer because of the hormonal changes,” she said. “They decrease their mortality. There’s very good data that you lengthen your lifespan if you have a BRCA mutation and you take out your ovaries. Not to mention the improved quality of life of not having to live through cancer treatment.”
Amy Byer Shainman, a 45-year-old stay-at-home mom from Jupiter, Florida, discovered she was BRCA1 in December of 2009. Like Jolie, she had lost family members to breast and ovarian cancers at a young age. Like Jolie, she also had a very high risk of being diagnosed with both cancers, a grim prospect for the mother of two.
After being tested, Shainman met with a genetic counselor who referred her to a high-risk oncologist. The oncologist laid out her risk management options, which included extra screenings, estrogen-blocking drugs (for women over 60) and prophylactic surgeries.
“You leave that appointment feeling like you’ve been run over by a truck,” said Shainman. “I spent the next month drinking a lot of wine, eating a lot of chocolate and doing a lot of research. I’m a researcher by nature and I had the fortitude to know you have to research, get a second opinion, get a third opinion and go about this the right way.”
In February of 2010, Shainman made her decision. She first had a full hysterectomy – a prophylactic removal of her ovaries, fallopian tubes and uterus (her family history included uterine as well as breast and ovarian cancer). Then several months later, she had a bilateral prophylactic mastectomy.
“It was not a decision that was made lightly,” she said. “But I feel lucky. I haven’t had cancer. And I’d like to stay that way if possible.”
In her opinion piece, Jolie stressed that knowledge was power. She also emphasized that women should make the choice that’s appropriate for them.
“The most important thing is to learn about the options and choose what is right for you personally,” she wrote.
Most breast cancers are not hereditary. The National Cancer Institute estimates no more than 10 percent of all breast cancers are due to inherited gene mutations such as BRCA1 or 2 (there are others). According to NCI, about 12 percent of all U.S. women will develop breast cancer and about 1.4 percent will develop ovarian cancer sometime during their lives.
For women (and men) with a BRCA gene mutation, however, the risks are much higher. These risks vary depending on family history and other factors, but around 55 to 65 percent of BRCA1 women and around 45 percent of BRCA2 women will be diagnosed with breast cancer by age 70. And nearly 40 percent of BRCA1 women and 11 to 17 percent of BRCA2 women will develop ovarian cancer.
The NCI recommends that genetic testing should only be performed when the person’s family history suggests the “the possible presence of a harmful mutation in BRCA1 or BRCA2,” i.e., a history of breast, ovarian, fallopian tube or peritoneal cancers.
Some of the factors associated with an increased likelihood of a BRCA1 or BRCA2 mutation include breast cancer diagnosis before age 50; both breast and ovarian cancers; multiple breast cancers; cases of male breast cancer and Ashkenazi Jewish ethnicity.
But genetic counseling is key, Shainman stressed (as does the NCI).
“They’re the experts who can decipher the cancer risks,” she said, pointing to accredited organizations such as the National Society for Genetic Counselors. “Plus they know the ins and outs and protocols with regard to insurance and which genetic testing companies are less expensive. They know if any of them have reduced fee plans or even free testing.”
Shainman, who has become an advocate for BRCA individuals since 2009, said her insurance covered most of the costs of her genetic testing and prophylactic surgeries.
Dr. Julie Gralow, a clinical researcher and breast cancer oncologist at Fred Hutch and Seattle Cancer Care Alliance, said that’s not unusual.
“In the U.S., most insurance plans would cover oophorectomy in BRCA-positive individuals,” she said. “It is the recommendation of many societies.”
Gralow, currently at a Breast Cancer in the Arab World conference in Amman, Jordan, stressed that this is not necessarily the case everywhere, however.
“Worldwide, it’s a much bigger issue,” she said. “Being in Jordan right now, with representatives from dozens of Middle East countries, I'd say that the wealthier patient could get testing and surgery but not the masses."
Gralow added that Jolie’s willingness to come forward with her story has definitely helped to educate people around the globe as to the risks posed by BRCA mutations, though. Surveys and studies have also shown Jolie’s willingness to come forward has had a “global and long lasting” effect.
“This is something that women all over the world can relate to,” she said. ”Her willingness to be public about this will create awareness and likely save many lives.”
Shainman heartily agreed.
“Now everyone is a lot more familiar with genetic mutations,” she said. “Even if they don’t exactly know what it is, they know that Angelina Jolie did something for her health and I look at that as progress. It’s saving lives. It’s getting the message out.”
Solid tumors, such as those of breast and ovaries, are the focus of Solid Tumor Translational Research, a network comprised of Fred Hutchinson Cancer Research Center, UW Medicine and Seattle Cancer Care Alliance. STTR is bridging laboratory sciences and patient care to provide the most precise treatment options for patients with solid tumor cancers.
Diane Mapes is a staff writer at Fred Hutchinson Cancer Research Center. She has written extensively about health issues for NBC News, TODAY, CNN, MSN, Seattle Magazine and other publications. A breast cancer survivor, she also writes the breast cancer blog doublewhammied.com. Reach her at firstname.lastname@example.org.