Jolie puts spotlight on a stark choice

Angelina Jolie won new fans — some in unlikely places — when she revealed Tuesday that she’d had a double mastectomy to ward off a very high genetic risk of breast cancer.

“I’ve become a huge Angelina Jolie fan today,” enthused Dr. Elizabeth Swisher, director of the Breast and Ovarian Cancer Prevention Program at the Seattle Cancer Care Alliance. “Women need to know there’s a woman like Angelina Jolie who can make this decision and go on with their lives. … It’s totally empowering for women.”

Jolie’s column in The New York Times sparked lively conversations among patients, doctors and researchers about who should take such a drastic step, and whether women who are not movie stars can afford to do so.

In a personal and detailed column, Jolie wrote about the death of her mother from cancer at age 56, and how genetic tests revealed that Jolie had an inherited gene mutation, BRCA1, giving her an 87 percent chance of getting breast cancer.

Given that, she wrote, she decided to be proactive, and her risk of breast cancer is now under 5 percent. “I can tell my children that they don’t need to fear they will lose me to breast cancer.”

In general, that mutation and one in a related gene, BRCA2, are rare, although slightly more common in women of Eastern European Jewish descent and a few other ethnic groups. Even so, researchers believe such inherited mutations are linked to as many as 10 percent of the breast cancers in the United States.

About 12 percent of women — one in every eight — will get breast cancer in their lifetime. But just how much the faulty genes raise the chance for an individual woman depends on a number of factors, including family history.

On average, having the gene makes a woman about five times more likely to contract breast cancer, and significantly increases the risk of ovarian cancer.

Despite Swisher’s enthusiasm for Jolie’s choice, she and other doctors emphasize that preventive mastectomy is only one choice for a woman with BRCA1 or BRCA2.

“If you have the BRCA1 or 2 gene, bilateral mastectomy is an option, but it’s not a strong recommendation or a mandate, by any means,” says Dr. Julie Gralow, a professor of breast cancer at the University of Washington.

“We don’t think it’s crazy for a woman with one of those mutations to choose to keep their breasts.”

Ovaries also at risk

For a woman making that choice, Gralow said, it’s about goals. To reduce the chance of ever getting breast cancer, the bilateral mastectomy does the trick.

But the other choice — frequent testing and early detection — likely would catch a cancer early enough to drastically reduce the chance it could prove fatal.

On the other hand, there is no good early detection or treatment for ovarian cancer. For a woman who tests positive for the mutations, Gralow and colleagues would recommend removing ovaries by age 40.

Gralow said the genetic component is only one factor that women should consider.

“Everybody shouldn’t rush off and have this test,” she said. Other important risk factors include being female (men get breast cancer, too), getting older, reproductive history, age at first menstruation, number of children, whether they were breast fed, whether a woman took hormones, and various lifestyle issues.

“Genetics is really important for five to 10 percent of the women, but it’s not the majority cause,” Gralow said.

Insurance coverage?

With Jolie’s announcement also came questions about whether most women could afford the expensive genetic tests, the surgery and reconstruction, and whether insurance would cover those steps for a woman who doesn’t already have cancer.

Doctors reported sometimes having to go through hoops to get insurance approval, particularly for women who don’t have cancer and may have few relatives, making familial cancer history unknown.

But all of the major insurers in Washington, including Medicaid, said they would approve genetic testing, surgery and reconstruction for women with the appropriate family history and preapproval process. Medicare, however, only covers genetic testing for women who already have cancer.

For some doctors, the whole genetic-test issue is a sore spot.

The BRCA1 gene was discovered after painstaking years of work by Swisher’s colleague Mary-Claire King, now a UW geneticist, more than 20 years ago. King strongly believed that the fruits of such research should be public.

But a private company, Myriad Genetics, patented the gene. Now, doctors and patients who need genetic tests for the genes must use the tests the company developed, which may cost as much as $3,000 to $4,000.

“People are using federal public dollars to then take the work and patent and license it — it’s just not right,” said Swisher, who, with King, has developed a single blood test to look for all known cancer genes. She says she’s used it in her research to find the genetic cause of ovarian cancer in dozens of families.

But outside of research, the test can’t be used on patients, because others hold patents on a third of all human genes, including BRCA1 and BRCA2, she said.

Myriad’s patents — and whether a human gene should be patented — are now the subject of a closely watched case before the U.S. Supreme Court.

For most women with family histories of breast or ovarian cancer, the concerns are more personal.

“A difficult decision”

In her column, Jolie went into detail about her surgery, which UW breast surgeon Dr. Sara Javid said typically takes five to six hours. Reconstruction adds another five or six hours if a woman does the concurrent “tummy tuck” process, using her own belly fat for reconstruction.

If implants are needed, the process stretches out for several months and requires additional procedures.

Although risk-reducing mastectomies are more common among women with inherited genetic mutations in this country than in most others, Javid said it’s always a very big decision. “Some of these patients are conflicted even up to the day of surgery,” she said.

Some doctors said they felt women who chose risk-reducing surgery such as Jolie’s were not well supported by friends and, in some cases, family members. “People are really judgmental about it,” said Swisher. “They don’t stop and think — this is a difficult decision.”

She noted support offered through a national nonprofit called FORCE that focuses on families affected by hereditary breast and ovarian cancer.

Now, some of Jolie’s new fans say, these women have a new support system.

“For a woman whose career is tied to her appearance, her willingness to talk about her decision provides support to women and their partners who may face a similar difficult choice,” wrote bioethicist and columnist Arthur Caplan.

For Swisher, Jolie’s latest drama melds in her mind with some of the actress’ earlier movies. “She’s such a powerful woman figure — sexy, but always strong and powerful,” she says. “You can see her with her guns blazing — against cancer risk.”

Material from The Associated Press was included in this report.