'Every kind of cancer in our family': Sisters face risk and fight disease head on
GRAND FORKS -- When Barb Jensen of Grand Forks found out her mother carried a gene that greatly increases one's risk for developing cancer, she put off taking the test that would determine if she had inherited it.
Her mother's oncologist recommended she and her three sisters all be genetically tested for evidence of the BRCA2 gene. As each of Jensen's sisters went through the test and found they did not have the gene, Jensen held back.
"I waited. ... I had a feeling I had it," she said.
If she did, she didn't want her mother to feel guilty, she said. "My mom was the type who would feel guilty" for passing on the gene, even though it is irrational.
Jensen also delayed genetic testing until passage of a federal law that prohibits insurance companies from denying coverage to those who carry the BRCA2 gene, she said.
Actress Angelina Jolie, 37, drew widespread media attention recently when she announced that because she carries the BRCA1 gene, she had undergone a double-mastectomy to increase her chances for a longer, healthier life.
When Jensen heard that news, she told her husband, Harlan, "She's probably going to save a lot of lives. Women will get that test."
Jolie's mother, Marcheline Bertrand, died from ovarian cancer at age 56. The actress's aunt died from breast cancer last month.
Jensen has a history of fibrocystic breasts, she said. They were dense and heavy, making it difficult for her to tell, in self-examination, if a lump was a sign of cancer.
"I'd always felt that if I was going to get cancer, it would be breast cancer."
In August 2009, nearly a year after her mother died, Jensen chose to go through genetic counseling, in preparation for the BRCA test. She remembers the day she received the results.
"When I found out I had BRCA, I wasn't terribly surprised," she said.
With genetic counseling, she learned that her risk for breast cancer, based on family history, was 57 percent but, because she had the BRCA2 gene, it rose to 94 percent, she said. After mastectomy surgery to remove both breasts, her risk for breast cancer fell to 4 to 6 percent.
Her risk for ovarian cancer had stood at 6 percent, but was elevated to 65 percent with the presence of the BRCA2 gene. After surgery to remove her ovaries, her risk for ovarian cancer decreased to 2 to 4 percent.
"Even though they remove the breast tissue, cancer can develop in other tissue," which is similar to breast tissue, in other parts of the body, she said. "The risk (for cancer) is still there, but it's greatly reduced by having the surgeries."
"For me, the decision to have surgery was a 'no-brainer,' " Jensen said, but dealing with such serious health challenges have been difficult and stressful.
The surgery to remove her ovaries in December 2009 was followed six weeks later by her double mastectomy.
Although her mastectomy and reconstructive surgery were done in the same eight-hour operation, she developed an infection and the reconstruction had to be removed, she said. In all, she's had nine breast- and ovary-related surgeries.
Removing the ovaries "thrusts you into menopause; it was a roller-coaster ride."
After a few months, she went through reconstructive surgery again, and fought off another infection.
"A process that should have taken nine months took me over two years," she said.
"I believe I went in with my headlights on, but I was not prepared for the body image struggle and losing a job because of the surgeries," and not being able to return to work on a set date.
She grieved the loss of her breasts, she said. The surgeries took a toll on her self-image.
"It's a process, a difficult process," but it has given her "a whole different perspective on everything.
"I used to be a person who gets excited about everything. Now, I choose what I get excited about. I like myself a whole lot better."
She plans to have more corrective surgery, probably next year, she said.
She encourages women who may be facing what she has to visit www.facingourrisk.org (Facing Our Risk of Cancer Empowered), where women share information, encouragement and support.
"It's been such a valuable place for me to go."
Finding a sister
One of the unexpected gifts of their experience with the BRCA gene was finding a sister they never knew they had.
In 2007, a year before their mother, Delores ("Susie") Streiffel, died, she told her daughters about a baby she had given up for adoption when she was 16. She had had no further contact.
At their mother's request, the sisters began to search for their sibling to alert her that she may carry the BRCA gene. They eventually found her in Burke, Va.
The baby, Carrie Smith, had been adopted by a Minneapolis couple and raised as an only child. Although she never met her birth mother in person, she was able to talk to her by phone the night before she died in November 2008.
Smith had been diagnosed with breast cancer in 2000 and had undergone a mastectomy. After testing positive for the BRCA gene in 2009, she had surgeries to remove the other breast and her ovaries.
Cancer "gives you more appreciation for life," she said.
Getting tested for the BRCA gene was a "no-brainer," Smith said. "My concern was, did I pass it on to my son?" her only child. (She later learned she had not.)
After her second mastectomy, her plastic and general surgeons recommended she have her ovaries removed, too.
"They harped on it."
Cancer is the reason she found her family, she said. She's not sure she would have, if not for the BRCA2 gene.
Because they both have the gene, Jensen has turned to Smith "when I've doubted something. I'd call her, and she'd say, 'You're doing the right thing.' "
"It's been a short relationship, but a very good one," Jensen said.
Their sister Idona Holbrook of Greenwood, Minn., tested negative for BRCA but is battling breast cancer for the second time. The three genetic tests she has taken were all negative, she said.
"They tell you it (the results of the BRCA test) could be wrong," said their sister Susan Kraft of Grand Forks.
Holbrook is also concerned about her two daughters; she plans to take an advanced test, called BART, to gain certainty.
"Even though you test positive, it doesn't mean you're going to get cancer," said another sister, Jola Streiffel of Grand Forks.
Kraft said she works with a woman who may have the BRCA but is afraid to find out.
"They say they don't want to know, but it eats at them," Smith said.
Also affects men
At age 25, Jensen's daughter, Jill, decided to go through genetic counseling and testing. Her results came back negative for the BRCA gene.
Jensen worries her only other child, a 29-year-old son, could also carry the gene. Jesse, who will be married this fall, has decided to delay testing until after he and his wife have children.
"I support that decision," she said. "I won't really rest until we know if he has it. I pray every day that he'll be negative."
If Jesse doesn't have the gene, "that part dies with him."
On his wedding day, Jesse will wear a teal handkerchief in his pocket in honor and memory of his grandmother, Susie Streiffel, Jensen said. Teal is symbolic for ovarian cancer, which took her life.
Men who carry the BRCA2 gene are at higher risk for secondary cancer, such as prostate and stomach, she said.
The sisters have two brothers who have decided not to be tested.
Relay for Life
Since their initial reunion, the sisters have built an easy camaraderie -- they joke and tease as though they've known each other all their lives. They've taken trips together, and are planning the next one.
At a recent interview, they spoke often of their mother's courageous fight against cancer.
For 11 of her last 14 years, she had chemotherapy, Holbrook said.
"She was a tough lady," Kraft said. "At (5-foot-1) she was tougher than nails."
Since 1996, they've made it a tradition to participate as a team in the annual Grand Forks Relay for Life in honor of family members stricken with cancer and to support cancer research.
"Mom lived for that event," Streiffel said.
"It was our mom's favorite thing," Jensen said. Their father, Hank, looked forward to it, too.
"We've had every kind of cancer in our family," Streiffel said. She and her sisters mention skin, brain, lung, stomach, colon, breast and ovaries.
"But we're in first place for Relay for Life for fundraising."
The family has raised tens of thousands of dollars for the cause.
Kraft said, "We're tired of cancer. We're tired of it taking people from us." She recently lost her husband, Tom, to lung cancer.
Her mother battled ovarian cancer for nearly 15 years before she died at age 78. When she was diagnosed, her doctor in Fargo gave her six months to live.
Most women who have ovarian cancer survive three to five years after diagnosis, Jensen said.
"The doctors at Mayo did some amazing things (for Mom). One of them said, 'We don't know why you're still here.' "
Advice for others
Jensen keeps her experience in perspective.
"This journey is not something you start and stop. It doesn't end. It's important to have a real positive outlook to get through this."
If not for the support of her family, her husband and children, "I don't know if I could have gone through it," she said.
"Ninety percent of (fighting) cancer is attitude," Streiffel said. "She (motioning to Holbrook) has a great attitude. She's my inspiration.
"At Barb's wedding last year, we thought it may be the last time we'd all see her, she was so sick."
Although Holbrook is battling cancer for the second time, she said, "I don't feel sorry for myself. ... You have to celebrate the victories."
She said, "I tell people, you hear the words, 'you have cancer,' that's not a death sentence ... It's just a disease; you learn to live with it."
Smith emphasized that "doctors don't know everything. I had 27 positive lymph nodes. I was told chemo would buy me time. That was 13 years ago. I had Stage 3 breast cancer."
Jensen tells "anyone who will listen" to seek out genetic counseling if they think they may be at risk, she said. "Learn as much as you possibly can, and to talk to as many people as you can.
"There's no such thing as 'information overload.' It's about saving your life."
Genetic counselors provide information in simple terms that everyone can understand, she said.
"Whether you're a man or woman, if more than one person in your family has cancer, it's a good time to ask questions."
She stressed the need to pursue genetic counseling and testing "so your decisions are based on knowledge, not emotion."
For those who are financially stressed, there are resources that can help, she said. "Don't let money be the thing that blocks you from taking action.
"No matter what amount of money you have to pay, it's well worth it -- to take the worry away or prepare you to deal with it.
"Become empowered, so you can make the best decisions for your future."
Understanding risk factors for hereditary cancer
Family and personal medical history hold the key to understanding your risk for breast and ovarian cancer. If your history does suggest an increased risk, consider talking to a genetic counselor to determine whether you are a candidate for genetic testing.
Find out which relatives -- on your mother's and father's sides -- have had cancer, which types and how old they were when diagnosed. Sometimes, these conversations are tough, but it's important to gather as much information as you can.
Any of the following events in your family history may be a sign of hereditary cancer:
A relative with:
-- Bilateral breast cancer (cancer in both breasts)
-- Breast and ovarian cancer
-- Male breast cancer
-- A BRCA mutation
Two relatives with:
-- Breast cancer (one before age 50)
-- Ovarian cancer at any age
-- One with breast cancer and one with ovarian cancer
Three relatives with:
-- Breast cancer at any age
Personal cancer history
Your personal cancer history also affects your risk. Any of these events in your personal history could be a sign of hereditary breast or ovarian cancer:
You were diagnosed with breast cancer before age 50.
You have been diagnosed with ovarian cancer, fallopian tube cancer or primary peritoneal cancer at any age.
You have been diagnosed with:
-- Breast cancer more than once
-- Both breast and ovarian cancer
-- Breast cancer or ovarian cancer at any age, and you are of Ashkenazi Jewish ancestry (Central or Eastern European)
-- Breast cancer and you have a family member with breast or ovarian cancer
What is BRCA?
BRCA1 and BRCA2, named for breast cancer genes 1 and 2, can play an important role in breast and ovarian cancer.
Normally, these genes stop breast and ovarian cells from growing and dividing uncontrolled. When an error occurs, or there is a mutation found, it can increase the chances of cancer developing.
Every person has two copies of each gene. As long as at least one BRCA1 and one BRCA2 gene works normally, your risk for cancer won't be raised. The two copies of each gene act as backups for each other.
However, if both copies of either BRCA1 or BRCA2 are damaged, your body loses a tool for stopping cancer cells from growing.
Some people are not born with normal BRCA genes -- they inherit a mutation (or genetic damage) in one of these genes. Since they don't have backup protection, any damage to the normal BRCA gene can lead to cancer.
Women with a BRCA mutation face a 60 to 87 percent lifetime risk for breast cancer and a 20 to 54 percent lifetime risk for ovarian cancer -- much higher than the general population.