By: Zoe Chong
Lianne Degen was 23 years old when she received a life-saving letter from her father’s cousin informing her that the BRCA2 gene mutation existed in her family. After getting tested, Degen found out that she, too, had inherited a bad copy of the BRCA gene.
This meant Degen was estimated to have up to an 85 per cent chance of developing breast cancer and up to a 20 per cent chance of developing ovarian cancer in her lifetime, according to the Canadian Cancer Society.
An average woman has up to an 11 per cent risk of developing breast cancer in her lifetime, according to the Canadian Cancer Society. Inheriting a BRCA mutation, which can occur in either the BRCA1 or BRCA2 genes, only accounts for about five to 10 per cent of breast cancers.
Degen said she looked at these statistics and initially chose to undergo screening, which included annual mammograms and MRIs. Six years later, she said she received a false-positive MRI result that prompted her to make the decision to get preventive surgery.
Degen underwent a prophylactic double mastectomy, the removal of both her breasts, at 29 years old. This surgery reduced her risk of breast cancer to fewer than five per cent.
The decision to undergo this surgery is a growing trend among women who have a BRCA mutation. The increasing prominence of BRCA mutations has launched preventive surgery into the spotlight, especially following Angelina Jolie’s decision to undergo the same surgery after testing positive for a mutation in the BRCA1 gene.
While Jolie has faced overwhelmingly positive reactions for her brave decision to undergo preventive surgery, Degen said there are people who believe her decision was an overreaction to a diagnosis that doesn’t guarantee cancer.
Degen said she was very open about her decision to undergo preventive surgery, telling everyone she came into contact with and blogging about every step of her surgery. This openness left Degen exposed to harsh comments from people questioning her decision. One comment that Degen said still sticks in her head is, “Why would you butcher yourself?”
Forty-two-year-old Sharon Cox, a BRCA1 mutation carrier, said these comments are due to a lack of knowledge on the issue. Cox said she underwent a hysterectomy, removal of the uterus, and a mastectomy when her mother tested positive for the gene mutation after surviving four bouts of cancer.
If her mother had known about being a carrier earlier, she might have been able to have the surgeries that could have prolonged her life, said Cox. Her mother lost her battle against cancer this year.
Cox said she had to continuously justify her decisions to her friends and family, who didn’t understand why she would make such a drastic choice. For her, the decision to undergo preventive surgery was about empowerment, not victimization.
“My mother had cancer five times and I probably would’ve had the same thing happen to me,” she said. “Now I’m probably never going to get breast or ovarian cancer.”
Degen said she thinks people find it hard to digest the idea of preventive surgery because when women actually have a cancer diagnosis, “surgery is a no-brainer,” whereas a mutation carrier may never suffer from cancer.
The decision to undergo preventive surgery, however, is still a life-changing one.
Cara Scharf, 28, said the decision to undergo preventive surgery is a uniquely personal one – one she decided against when she tested positive for the BRCA1 mutation after graduating from university at 22.
Scharf lost her mother to breast cancer when she was only three years old and her maternal grandmother to ovarian cancer before she was born, both of which were BRCA-related.
“To me, at first, it was like facing my own mortality,” Scharf said. “This is how I’m going to die and that’s a really terrible thing to think about when you’re only 22.”
Scharf said she was faced with questions about what she needed to do to jumpstart her life – “I’ve got to figure out what my career’s going to be, who I’m going to marry, when I’m going to have kids,” – all of which she didn’t think she’d have to ask herself at such a young age.
The protocol at the time was to start screening at 25 years old, so Scharf said she put this diagnosis at the back of her mind and lived the next three years without much change.
When Scharf went for her first baseline screening, she said her MRI picked up a tumour. “I went into it thinking it was just going to be nothing and when they actually told me I had breast cancer, it was really hard to believe,” she said.
When Scharf first found out she held the mutated gene, she said she thought removing her breasts was too drastic an option for her age. “I thought that I had time.”
This false idea that there is time, Degen said, emphasizes the need to be proactive.
“It doesn’t have to be a terminal thing,” Degen said, when it comes to breast cancer. “When it’s terminal, it’s usually not caught in time. And that’s what’s really sad and that’s why I’m such an advocate for screening.”
According to researchers, wait times for preventive surgery can reach up to three years in B.C. The research showed that the wait times for preventive surgery were so long that women end up getting breast cancer before the surgery date.
The medical system in Canada understandably prioritizes women who have an active cancer, and women looking to get preventive surgery are forced to wait significantly longer for their surgeries, said Dr. Kirsty Boyd, a plastic surgeon who specializes in breast reconstruction.
Boyd is part of a same-day mastectomy and reconstruction program at the Ottawa Hospital’s Riverside site once a month, geared towards providing better service to women who decide to get preventive surgery.
Dr. James Watters, the medical director of the Women’s Breast Health Centre at the Ottawa Hospital, said this program has reduced wait times for preventive surgeries, which were typically up to a year or more, to within three or four months.
The long wait times are “very tough for women who’ve made this difficult and important decision to undergo surgery, and then we have to tell them you have to wait,” said Watters.
Boyd said Cancer Care Ontario has mandated surgery wait times for women who have cancer to within four weeks, but there is no such mandate for women with a BRCA mutation.
And Scharf’s experience is living proof that a lot can happen in a few years.
“It’s important for women to have access to digestible information that isn’t just full of medical jargon,” said a counsellor at Willow Breast Cancer Support Canada, a non-profit organization that provides free support and information to individuals affected by breast cancer and BRCA mutations.
The Willow counsellor, who’s also a mutation carrier, said women are faced with a mix of emotions when they are diagnosed with the mutation and need support from women who’ve experienced the same thing.
“Grief is a large part of getting your diagnosis. You grieve for yourself, and you grieve for your children if you’re a mother. And if you get the surgeries, you grieve for your body and what you’re losing with that,” she said.
It’s important for women to have support when they’re making these life-changing decisions, she said.
While she said she’s not ready for them yet, the subject of babies has been on Scharf’s mind. On one hand, Scharf said she’s against passing on her faulty gene to her children, but the idea of not having children of her own devastates her.
Scharf decided to freeze her embryos when she was first diagnosed with breast cancer, after being prompted by her father, who’s a doctor.
“I love my family and I don’t want to perpetuate this terrible fortune we have so why wouldn’t I take every step that I could to make sure that I’m the last person in my family line who has to deal with this.”
Scharf said this is her way of taking control over the gene that gave her cancer and took the lives of her mother and grandmother.
The support from services like Willow provides women with the necessary information that will help them make an informed decision when it comes to preventive surgery, said the Willow counsellor.
Unlike Scharf, who had her father, not all women have people in their life to provide them with the right information.
Although Scharf said she still believes preventive surgery is a drastic option, she emphasized how “everybody has a different threshold for how much risk they’re willing to accept.”
“I didn’t get the surgeries and then I got breast cancer, so who’s to say that just because a woman doesn’t already have breast cancer that this isn’t a good idea,” Scharf said.
Cox said it was a big lesson for her to let people be who they are and make their own decisions regarding preventive surgery. She said she learnt that it wasn’t her job to preach or push her own decisions on others.
In the end, Degen, now 34, said her decision to remove her breasts, and soon her ovaries, is a uniquely personal decision and there are many alternatives to surgery available for those with a BRCA mutation.
“What it really comes down to are risks I’m willing to live with and risks I’m not willing to live with,” she said. “I’m not willing to live with my breasts and I’m not willing to live with my ovaries past a point.”