Not "One-Size Fits All"-Jolie Pitt's Story Spotlights Hereditary Cancer Risks

Not "One-Size Fits All"-Jolie Pitt's Story Spotlights Hereditary Cancer Risks

Angelina Jolie Pitt recently added another chapter to her BRCA mutation story, making public her choice to undergo a preventive salpingo-oophorectomy, removal of her ovaries and fallopian tubes to reduce her risk of cancer.

Two years ago, Jolie Pitt announced that she had undergone testing for the BRCA 1/2 mutations due to a strong family history of cancer on her mother’s side (“My Medical Choice,” New York Times, May 14, 2013). She tested positive for the BRCA 1 mutation, which increased her risk of ovarian cancer and breast cancer by 50% and 87%, respectively, and underwent a prophylactic mastectomy, surgically removing both breasts to reduce her risk of breast cancer.

In a recent column for the New York Times, Jolie Pitt described in her “Diary of a Surgery” how a recent cancer scare prompted her to move forward in removing her ovaries, writing “I did not do this solely because I carry the BRCA 1 gene mutation, and I want other women to hear this. A positive BRCA test does not mean a leap to surgery.”

A Conversation Starter

Amy Byer Shainman, a hereditary cancer health advocate who also carries the BRCA 1 mutation, said Jolie Pitt’s public sharing of her experience has definitely made an impact when it comes to hereditary cancer awareness. “As an advocate, I view all of this as a huge step forward and progress—a very positive societal change,” she says.

“Prior to Jolie coming forward, families affected by cancer may not have even known to have these conversations about hereditary cancer risk, or that such a thing as hereditary cancer even existed,” Byer Shainman continued.

“Angelina sharing her story may now give families with family members who currently have cancer, or died of cancer, the thought that maybe the cancer in their family has a hereditary component, and perhaps they need to find out more.”

Joy Larsen Haidle, MS, CGC, president of the National Society of Genetic Counselors (NSGC), said there was a noticeable uptick in interest in genetic testing, primarily for the BRCA mutations, after the actress—director’s first piece.

“When I think back to when she did her first op-ed, it created a huge amount of awareness and discussion amongst the general public,” she says.

To gauge the level of awareness of the “Angeline Jolie effect,” the NSGC conducted a study that found 90% of the genetic counselors surveyed had a significant increase in the number of calls to their offices immediately following Jolie Pitt’s first op-ed. The number of calls continued to increase for 6 months after publication and was still significantly higher one year later.

Understanding Risk

Although awareness of hereditary cancer is only one part of a complicated issue, Larsen Haidle is heartened by the increased dialogue.

“So much of the time when we’re talking about genetics with families, sometimes people who’ve already had cancer feel they don’t need to think about testing,” she says. “It’s still very useful to learn if the cancer happened by chance or if there is a genetic component,” pointing not only to a risk for other family members but also risk of a secondary cancer for the patient.

Families should consider genetic counseling if a close relative developed cancer before age 50, if someone in the group has a history of more than one cancer, if three or more relatives on one side of the family have developed cancer, or if there is a family history of certain cancers or aggressive cancers.

Larsen Haidle suggests that individuals who are concerned with hereditary cancer risk talk with a genetic counselor about testing, discussing the decision with family members and helping to develop a personalized strategy of risk reduction based on the results of the test.

She also stresses that even a negative test result may not be conclusive. “A negative result doesn’t mean a person doesn’t have an inherited component, it just means the test didn’t answer the question.” A patient with a strong family history who tests negative could still have an unknown gene mutation, or there could be limitations to the test.

“We still need to tailor surveillance to what we see in the family tree, and not assume there isn’t a risk factor,” Larsen Haidle says.

It’s also important for patients and survivors to examine both sides of their family trees. “Half of our hereditary risk comes from our father’s side of the family,” Larsen Haidle says. “Women can get caught off guard at young ages if the carrier happens to be a male.”

Other Hereditary Cancers

While Jolie Pitt has raised the level of public knowledge on the breast cancer genes, it remains to be seen if she’s increased awareness of BRCA-related cancers in men and other hereditary cancers, such as von Hippel-Lindau syndrome, which can cause a predisposition to the development of kidney cancer.

“One of the hereditary colon cancer syndromes called Lynch syndrome is as common as the BRCA 1 and 2 gene mutation, but it hasn’t garnered as much attention,” Larsen Haidle says. “Five to 10 percent of all cancers will have a strong hereditary component.”

If an individual does have a hereditary cancer risk, either confirmed by a genetic test or through a family history examination, experts encourage individuals to seek genetic counseling, weigh options, and not make quick decisions regarding risk reduction.

Jolie Pitt outlined how she waited 2 years before undergoing her latest surgery, taking time to prepare herself for premature menopause. It was only after routine blood work pointed to an increase in potential cancer markers that she moved up her timeline for surgery.

“Not everyone may choose surgery,” Larsen Haidle says. “They might choose to do heightened surveillance, particularly women who are young, or if the risk may be lower in that age range—for example, if a person has a family history of cancer, but the family members survived.”

Risk reduction strategies are individualized decisions that incorporate a family’s and the individual’s own medical history, beliefs, and life goals—something Larsen Haidle says Jolie Pitt emphasized in her second op-ed. “There are lots of personal experiences that impact what a person chooses to do,” Haidle says. It’s not a one-size-fits-all plan.”

Byer Shainman, who underwent a prophylactic salpingo-oophorectomy after her sister was diagnosed with ovarian cancer, and later a prophylactic mastectomy, has an additional request of Jolie Pitt: “That she write an op-ed number three about 3 months from now after her hormones have plummeted. I would love for her to discuss her menopausal experience, symptoms, and if she has had to adjust any hormones, medications.”

“This would be very helpful to so many women who have had their ovaries removed and are struggling with these postsurgery issues on a daily basis.”