PORTLAND – After years of watching family members battle cancer, Denali Wright of Portland faced an emotional decision: whether to undergo testing to learn whether she carried a genetic mutation that put her at extremely high risk for breast and ovarian cancer.

In the end, her decision was easy.

“I can’t deal with the unknown,” Wright said, “so it wasn’t really a question in my mind.”

She found out that she, like her mother, is among the small number of people nationwide who have the BRCA1 or BRCA2 mutation, which has been linked to hereditary breast and ovarian cancer. With the sobering knowledge that she had an 85 percent risk of developing breast cancer, she chose to have a double mastectomy. She was 31.

On Tuesday, actress Angelina Jolie wrote in a New York Times opinion piece that she is BRCA-positive and had a preventive double mastectomy. Jolie, whose mother died of ovarian cancer, said she had an 87 percent chance of getting breast cancer, but that risk is now just 5 percent because of the surgery.

“Cancer is still a word that strikes fear into people’s hearts, producing a deep sense of powerlessness,” Jolie wrote. “But today it is possible to find out through a blood test whether you are highly susceptible to breast and ovarian cancer, and then take action.”

Jolie and Wright are among a growing number of women who are choosing preventive mastectomies to reduce their risk of cancer by as much as 90 percent. The number of women opting for the surgery increased tenfold from 1998 to 2007, according to a study published in 2010 in the Annals of Surgical Oncology.

Doctors in Maine applauded Jolie on Tuesday for her willingness to share her personal decision and for bringing more attention to preventive testing and treatment.

“A lot of women are not aware of their own risk,” said Dr. Suzanne Hoekstra, a surgeon with Mercy Hospital’s Breast Care Specialists of Maine. “This may help more people take a closer look at their own family history.”

Genetic testing can help prevent cancer, but it is not appropriate for every woman, according to doctors.

Only 5 to 10 percent of cancers are hereditary, and only a small number of women get genetic testing to find out whether they carry the gene mutation. The vast majority of cancer cases are sporadic and unconnected to family history, said Dr. Roger Inhorn, medical director of the Oncology/Hematology Center at Mercy Hospital.

The U.S. Preventive Services Task Force recommends that only women with a strong family history of cancer — just 2 percent of U.S. women — even think about getting a BRCA genetic test.

Not everyone is a candidate for the testing, said Amanda Lamb, a genetic counselor at the Maine Medical Center Cancer Institute’s Cancer Risk and Prevention Clinic. Those who are candidates have increased risk factors, such as:

n Being of Ashkenazi Jewish descent.

n Having multiple close relatives diagnosed with cancer before the age of 50.

n Having male relatives diagnosed with breast cancer.

n Having relatives diagnosed with both ovarian and breast cancer.

The testing — which costs about $3,000 — isn’t always covered by insurance, Hoekstra said, and that price may prevent some women from getting testing and treatment that would significantly reduce their chances of having cancer later in life.

Doug Dunbar, a spokesman for Maine’s Department of Professional and Financial Regulation, which includes the state Bureau of Insurance, said it’s unlikely that most health care plans would cover genetic testing or a mastectomy as a preventive measure.

He said genetic testing is usually covered by insurance only if it’s used to help plan treatment for a diagnosed condition. And, without breast cancer present, a mastectomy or double mastectomy would likely be considered not medically necessary, and therefore not covered.

The surgery can cost tens of thousands of dollars, depending on the extent of the procedure and reconstruction.

That lack of coverage, including for low-income patients covered by MaineCare, makes it all the more important for people to have conversations about their family history and risk factors, Inhorn said.

Although the genetic testing has been done for at least a decade, there is still “a lot of fear about genetic risk and testing,” Inhorn said.

Sometimes, families are fearful about what the testing will show because of the implications for their own health, he said.

Other patients may find relief in knowing their risk factor and their treatment options. A woman who chooses not to have a double mastectomy or have her ovaries removed may opt for increased screening or treatment with medication.

“It can be very emotional, but for most (patients) it’s almost a relief to know they have this, their level of risk and that they can do something about it,” Hoekstra said.

Denali Wright, who’s a pharmacist with Apothecary by Design, said she has a strong family history of cancer. Her father had cancer when she was a child, and her mother had it a few years later. Both survived. A grandmother, a great-grandmother and a great-great-grandmother also were diagnosed with cancer.

Even though her mother survived, “it was very difficult to watch her go through that,” Wright said.

For years, her mother’s doctor encouraged her to get genetic testing, largely to determine whether Wright was at risk of being BRCA-positive. Men and women who have the mutation have a 50 percent chance of passing it on to their children.

Three years ago her mother, Sherry Cahoon, tested positive for the mutation and had a mastectomy on the breast she hadn’t had removed during cancer treatment. Wright had her genetic testing done in Boston around the same time.

“It was kind of a family affair,” Wright said.

Soon after she learned that she was BRCA-positive, Wright found a lump in her breast. She didn’t hesitate to move forward with the preventive mastectomy, but she hasn’t decided yet whether to have her ovaries removed.

“There was pretty much a guarantee at some point I’d have breast cancer,” she said. “That wasn’t something I was willing to gamble on.”

Though she knew that her cancer risk would be reduced, Wright said the decision to go ahead with the surgery was tough. Afterward, she had to deal with new insecurities about her body.

“To know, at 30 years old, I’m going to have scarring and will no longer have my own breasts was very emotional,” she said.

Through it all, her family members — especially her husband, Harry — were very supportive because “they’re huge champions of doing anything we can” to fight cancer.

On Tuesday morning, Wright got a text message from a friend joking that Jolie had followed Wright’s lead and that “I was a trendsetter,” she said.

“I would hope having Angelina Jolie being in the forefront would help other people,” she said, “so they’re not embarrassed or are more willing to talk about it.”

 

Gillian Graham can be contacted at 791-6315 or at:

ggraham@mainetoday.com

Twitter: grahamgillian