May 15 (Bloomberg) -- Angelina Jolie’s decision to have a double mastectomy is fueling debate among the thousands of women at risk of developing breast cancer who want to know how, if and when to have their breasts removed.
As many as 1 in 500 people in the U.S., including Jolie, carry a genetic mutation that gives them about a 60 percent chance of developing breast cancer, doctors said. Women in that group face the agonizing choice between surgery to remove their breast tissue or close monitoring for early signs of cancer along with drug treatments that can prevent tumor growth.
Those two paths, each with risks and benefits, can leave patients confused and conflicted over how best to protect themselves, said Susan Domchek, a breast cancer researcher and director of the Basser Research Center at the University of Pennsylvania. Jolie said she chose to have a double mastectomy after learning she had the genetic mutation that increased her risk for cancer, which killed her mother. Domchek said the surgery hasn’t been found to dramatically improve a patient’s life expectancy compared with vigilant screening and medicines though it is the best way to prevent cancer from forming.
“When people have a mastectomy that is a totally reasonable decision, and when people say ’I’m not ready to do this,’ that is also totally reasonable,” Domchek said in a telephone interview. “We have to be careful because there are women out there who are greatly struggling with this and they aren’t making a wrong decision by choosing screening.”
Domchek and other doctors said Jolie’s disclosure in an editorial in yesterday’s New York Times has driven a flood of calls from patients asking if they should get surgery or tested for the genetic mutations. The mutations in the BRCA1 and BRCA2 tumor-suppression genes are responsible for 5 to 10 percent of all breast cancers and 10 to 15 percent of all ovarian cancers, according to the National Cancer Institute.
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.
“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.
About 458,000 women worldwide, including 40,000 in the U.S., die annually from breast cancer. 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.
Different types of mastectomies are performed and new technologies have improved how well doctors can reconstruct the breasts, said Deborah Axelrod, a breast surgeon at NYU Langone Medical Center. 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.
“I’ve seen improvement in the aesthetics of doing mastectomies and patient satisfaction has increased,” Axelrod said in a telephone interview.
A mastectomy can cost $15,000 to $50,000 or more, depending on local rates and how extensive the surgery and is typically covered by insurance. 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.
Patients can also opt for monitoring to catch cancer early and take drugs that reduce the risk of tumor cells growing. Because of advances for treating early-stage breast cancer, closely monitoring, catching the tumor early and treating can have a similar mortality benefit as a mastectomy, Domchek said. From 60 percent to 70 percent of women with the mutation will go on to develop breast cancer without any treatment, she said.
“This is a very personal decision that can be very different depending on many factors, including a woman’s age and life experience,” said Ellen Matloff, director of cancer genetic counseling at Yale University in New Haven, Connecticut. “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.”
There are two drugs approved by U.S. regulator to prevent breast cancer, the generic medicine tamoxifen and Eli Lilly & Co.’s osteoporosis drug Evista. Both work by blocking estrogen, the fuel certain tumors need to grow. Tamoxifen was found to reduce the risk of developing invasive breast cancer by 50 percent over five years in post-menopausal women at high risk for the disease, according to the National Cancer Institute. Five years of Evista reduced the risk of developing breast cancer by 38 percent, according to the NCI.
Jolie, named the highest-paid actress in Hollywood by Forbes in 2011, made “a totally reasonable decision, though there are many options for women who find they carry these mutations,” Matloff said.
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 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.
The test is typically covered by private insurance though in some states Medicaid won’t pay for it, said Domchek.
By law, insurers can’t use the results of genetic exams to set premiums and they don’t get the results of breast-cancer tests, said Lee Newcomer, a senior vice-president for oncology, women’s health and genetics at UnitedHealth Group Inc., the largest U.S. health insurer. Starting next year, President Barack Obama’s health-care law will also prevent carriers from denying coverage based on pre-existing medical conditions.
The tests aren’t recommended for people without a family history of cancer and insurers have denied requests in cases where the tests are deemed unnecessary, said John Whitney, a vice-president of medical policy at WellPoint Inc. Still, when used appropriately, the savings from early detection and prevention can outweigh the costs, he said in a phone interview.
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 chance of developing breast cancer is now less than 5 percent. Her 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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