Angelina Jolie discusses her tough choices

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Angelia Jolie , who has the BRCA1 mutation, wrote in the New York Times about having her ovaries and fallopian tubes removed. Two years ago, she had a double mastectomy.

In her latest op-ed confessional, Angelina Jolie succinctly captured the complex dilemmas faced by women who carry a genetic defect that predisposes them to breast and ovarian cancer.

Two years ago, the actress, who has a BRCA1 mutation, had both breasts removed. That largely eliminated her 87 percent chance of developing breast cancer, but did nothing to reduce her 50 percent chance of ovarian cancer.

So last week, at age 39, she had her ovaries and fallopian tubes removed. While that cut her ovarian cancer risk by 85 percent, it also ended her fertility, plunged her into menopause, and left her with an estrogen deficiency that raises her risks of problems such as osteoporosis and heart disease.

The estrogen patch she now wears won't completely offset that deficit.

"It is a less complex surgery than the mastectomy," Jolie wrote Tuesday in the New York Times, "but its effects are more severe."

About 15 percent of ovarian cancer patients, and 5 percent of those with breast cancer, have mutations that knock out the normal cancer-suppressing function of BRCA1 or BRCA2.

So far, the only strategy that unequivocally works to reduce the ovarian cancer threat is to be preemptive.

"Nothing has been shown to be as effective as removal of the ovaries and fallopian tubes," said University of Pennsylvania oncologist Susan Domchek, director of the Basser Research Center for BRCA. "We're talking about decreasing the risk of death, so there is a very strong recommendation that women [with the mutation] have their ovaries out around age 40" or as soon as they finish having children.

Jolie, a mother of six and a United Nations special envoy, suggested that less severe options may work.

"Some women take birth control pills or rely on alternative medicines combined with frequent checks," she wrote. "The most important thing is to learn about the options and choose what is right for you personally."

While experts applauded her do-your-homework message - and her sensitivity to individual needs - they stressed the realities: There is no early-detection test for ovarian cancer. (As Jolie noted, her doctors checked her blood level of CA-125, a marker for potential cancer, but didn't rely on that test.) And studies show risk-reducing measures such as birth-control pills are, at best, modestly helpful.

"The other options don't confer the protection," said Jennifer Simmons, chief of breast surgery at Einstein Medical Center Montgomery.

After surgery, hormone-replacement therapy is another balancing act because estrogen's yin-yang effects are complex and vary with age. The scientific consensus is that after natural menopause around age 50, long-term hormone therapy does more harm than good.

But for women like Jolie who are prematurely deficient in estrogen, doctors typically prescribe years of supplements to protect the bones and cardiovascular system - as well as relieve the hot flashes and other miseries of "surgical menopause."

Jolie wrote that she takes "bioidentical" estrogen, but Domchek said there is no evidence that one type of estrogen is better than another.

Estrogen is not a magic bullet, either, as Sandy Cohen of Lafayette Hill can attest. Like Jolie, Cohen has a BRCA1 mutation, underwent a double mastectomy, had her ovaries removed at age 39, and wears an estrogen patch.

"The patch made the hot flashes more manageable, and helped with the mood swings," said Cohen, now 46. "But I still have hot flashes. And my bone density dropped. I'm on the verge of osteoporosis. I exercise with weights and I run" to build bone strength.

Cohen's mutation diagnosis also led to her current role as vice president of volunteer programs with FORCE (Facing Our Risk of Cancer Empowered), an organization for people with BRCA mutations (see www.facingourrisk.org).

Cohen prefers to focus on the benefits of surgery.

"I feel really at peace with my decision," said Cohen, a mother of two. "I don't worry about cancer anymore. That's huge. When I lost my mom to breast cancer, they didn't have these options. My kids don't have to worry like I did."

Jolie expressed a similar sentiment, writing: "I feel at ease with whatever will come."


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