Jolie Has Double Mastectomy to Thwart Cancer Gene Risk

Angelina Jolie, named the highest-paid actress in Hollywood by Forbes in 2011, said she had a double mastectomy after learning she has a gene mutation linked to breast cancer, the disease that killed her mother at age 56.

Writing in an editorial in today’s New York Times, the Academy Award-winning actress said she had an 87 percent risk of breast cancer and a 50 percent chance of ovarian cancer before the surgery because of an inherited gene known as BRCA1.

“Once I knew that this was my reality, I decided to be proactive and to minimize the risk as much I could,” Jolie, 37, wrote. Three months of medical procedures for the mastectomies culminated with breast reconstruction and implant surgery on April 27, she said.

In the U.S., more than 100,000 women undergo mastectomy each year to remove breast tissue that could harbor malignant cells, according to Decision Resources, an industry analysis group based in Burlington, Massachusetts,

Mutations in the BRCA1 and BRCA2 tumor-suppression genes are the most common cause of hereditary breast cancer, with the risk rising as high as 80 percent in some families, according to the American Cancer Society. In normal circumstances, BRCA1 provides a protein that repairs damaged DNA in cells. When BRCA1 is broken, the damage accumulates, allowing cells to grow and divide uncontrollably, forming tumors that can develop in the breast, ovaries, fallopian tube and pancreas, according to the National Institutes of Health.

‘Reasonable Decision’

Jolie made “a totally reasonable decision, though there are many options for women who find they carry these mutations,” said Ellen Matloff, director of cancer genetic counseling at Yale University in New Haven, Connecticut.

Women with the mutations can also undergo aggressive surveillance that may involve use of the drugs tamoxifen or Evista to reduce the risk of getting breast cancer, Matloff said in a telephone interview. Evista, sold by Eli Lilly & Co., is an osteoporosis drug shown to cut breast cancer risk in post-menopausal women. The drug, the second approved to prevent breast cancer after the generic medicine tamoxifen, works by blocking estrogen.

“This is a very personal decision that can be very different depending on many factors, including a woman’s age and life experience,” Matloff said. “Women need to work with a genetic counselor to consider their family history, the type of mutation they have and where they are in their lives. A 21-year-old who hasn’t had a child and is single may make a very different decision than an older woman.”

Mammogram Advice

When the mutations are prominent in a family, doctors will often advise women to start undergoing annual mammograms at age 25, rather than beginning at 40, the accepted standard. About 30 to 40 percent of women who know they have the mutations initially decide to undergo a mastectomy, Matloff said, “though that percentage may rise as their life evolves.”

Matloff said a recent survey done among genetic counselors found that 58 percent of them would undergo mastectomy if faced with the knowledge that they carried the mutations and had a strong family history of disease.

The most important thing for all women is to “know your family history” early on, she said. “Talk with everyone and know what cancer they had, whether they were tested and what the results may have been.”

Myriad Test

Myriad Genetics Inc., the world’s biggest genetic testing company, is the lone U.S. provider of BRCA1 and BRCA2 screens. The Salt Lake City-based company, founded in 1991, helped kick-start the gene-testing revolution by pinpointing the two BRCA genes in the mid-1990s.

The company performs about 250,000 BRCA tests a year, with the most comprehensive screens costing about $4,000, said Ron Rogers, Myriad’s executive vice president for corporate communications. About 95 percent of all women eligible for the test are covered by their insurance, he said.

UnitedHealth Group Inc., the biggest U.S. health insurer, covers BRCA tests based on “clinical evidence” in line with criteria established by groups including the American Cancer Society, according to its website. WellPoint Inc., the second-biggest carrier, covers the tests if an enrollee’s family has a “known, deleterious” BRCA mutation, its website said.

Different types of mastectomies are performed. This includes a skin-saving operation that minimizes scarring and gives a more natural look; a modified type that takes all of the breast tissue while leaving the lymph nodes under the arms; and a radical mastectomy that takes all of the breast, many lymph nodes and the muscles of the chest wall under the breast.

Surgery Costs

A mastectomy can cost $15,000 to $50,000 or more, depending on local rates and how extensive the surgery is. The cost of treating breast cancer after it appears can top $100,000 a year, depending on the type of tumor and the surgery, drug therapy and radiation treatment a woman undergoes, according to a 2009 study in the journal PharmacoEconomics.

“A well-done mastectomy is probably in the range of 90 percent protective,” said Larry Norton, an oncologist at Memorial Sloan-Kettering Cancer Center in New York. The risk involved in doing a double mastectomy is very small, particularly compared with the risk of getting breast cancer, Norton said.

Reconstructive surgery following a mastectomy is “getting better and better,” Norton said in a telephone interview.

Jolie’s Experience

Jolie didn’t specify the type of surgery she underwent. Her mastectomy process started on Feb. 2, she wrote, when she had a procedure called a nipple delay, which can detect disease in the breast ducts and draws extra blood flow to the area to increase the chances of saving the nipple.

Two weeks later, she had the breast tissue removed and temporary fillers put in place and after another nine weeks she had a final surgery to reconstruct the breasts with implants. The surgery only left her with small scars, she said.

Jolie’s decision follows by six months the announcement by Sharon Osbourne, a former judge on the reality television show “America’s Got Talent” and the spouse of rocker Ozzy Osbourne, that she had the procedure.

Jolie decided to write about her decision so other women could benefit from her experience and be encouraged to undergo genetic testing, she said. Her chances of developing breast cancer -- a malignancy that kills about 458,000 women globally each year -- is now less than 5 percent. Her own mother fought the disease for almost a decade, she said.

“I can tell my children that they don’t need to fear they will lose me to breast cancer,” Jolie wrote. “They can see my small scars and that’s it.”

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