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Breast Cancer In Disguise
by Karen Reed-Matthee

Marilyn Johnson woke one morning to find one of her breasts tender and swollen. She immediately scheduled an appointment with her doctor. Although she’d had a normal mammogram six months before, he ordered another – and found nothing. Her symptoms, he said, were likely to be perimenopausal. Marilyn was 49.

Four months went by and a silver-dollar-sized discoloration with the texture of orange peel appeared on her breast. Changing bras and detergents, applying various creams – nothing seemed to help. Her doctor saw her again and prescribed an antibiotic for mastitis. Two weeks later, not satisfied with Marilyn’s progress, he suggested she see a specialist for further diagnosis. Eventually, a biopsy showed Marilyn had inflammatory breast cancer, the most aggressive form of the disease.

Her condition, as is always the case with IBC, was already well advanced. Despite chemotherapy and surgery, Marilyn died, 15 months after she was diagnosed, in the summer of 1998.

The Johnson family lived in Anchorage, Alaska, at the time. Marilyn’s husband, Owen, now lives on Bainbridge Island and has become a leading advocate for raising awareness about inflammatory breast cancer. He funneled his grief at losing his wife, his anger that she wasn’t diagnosed sooner, and his frustration at the scarcity of accessible information about the disease into action. Carrying a card that read “Owen Johnson, Cancer Research Advocate,” he traveled the country, talking to researchers and connecting with other IBC patients and their families.

“I think things happen for a reason,” he says. “I had gone through this with Marilyn … I was at a time in my life when I could arrange things to have discretionary income and discretionary time. And I’m an engineer, a math guy. I’m science-oriented by nature.”

Johnson’s travels and contacts via e-mail with IBC patients, friends and families resulted in the formation of the Inflammatory Breast Cancer Research Foundation, which incorporated as a nonprofit organization in August 1999. He is the president, a fancy title for a full-time volunteer.

“We have two goals,” Johnson says. “The first is to facilitate research. We don’t have a big checkbook, but we know where the patients are. They’re spread out geographically, but they find us via our Web site and we can introduce them to research opportunities.” The second goal is to raise awareness about IBC and advocate on behalf of patients and their families. The foundation’s slogan is “You Don’t Have to Have a Lump to Have Breast Cancer.”

When There’s No Lump
While IBC comprises less than 10 percent of all breast cancers, more than 50 percent of IBC patients die within 24 to 36 months following diagnosis. Only five percent of IBC patients live more than 10 years. And roughly a quarter of all breast cancer-related deaths are from IBC.

“The thing about IBC is that its presentation is different than other breast cancers,” says Dr. Nora Disis, a University of Washington researcher working to find a vaccine for breast and ovarian cancers. “It starts as if it were a skin infection on your breast.”

Cancer cells clog the lymphatic system just below the skin. The symptoms are similar to mastitis, a breast infection, and some doctors, not recognizing IBC, treat it with antibiotics. “During that two-week [or longer] course of antibiotics, it will continue to grow rapidly,” Disis says. In more common forms of breast cancer, she adds, the discovery of a lump spurs doctors to move quickly toward a more definitive diagnosis.
There’s no known method for detecting IBC early: It doesn’t usually show up on mammograms or ultrasounds. “It’s not known if there even is an early stage,” says Johnson.

Similarly, the risk factors for other breast cancers, such as a family history of the disease, don’t seem to apply to inflammatory breast cancer. This contributes to making IBC even more of – in the words of Disis – a “real diagnostic conundrum.”

What makes IBC even more insidious, says Disis, is that it can strike young women, even women who are nursing a new baby and have good reason to suspect they have mastitis, leading to a delayed diagnosis. Other breast cancers usually do not occur in young women. The median age at diagnosis for IBC is 52, as compared to 62 for other breast cancers.

“We just lost a young athlete from California, a 17-year-old, to IBC,” says Nancy Key, a volunteer with the IBC Research Foundation who has been free of the disease for five years. “She was a soccer player. She thought she’d been hit in the breast and was embarrassed to tell her mom … it’s obscene for a 17-year-old to die from breast cancer.”

Time is of the Essence
Key, who lives on Camano Island, makes a plea for rapid and accurate diagnosis of IBC. She believes she’s still alive because her nurse practitioner decided to rule out the worst scenario first.

When an itchy, inflamed spot appeared on one of her breasts, Key, then 47, believed it was a spider bite. But the nurse practitioner suspected IBC and ordered an ultrasound, which showed some type of lymphatic block. Immediately, Key was sent for a biopsy. “I was told ‘You have breast cancer, and it’s the very worst kind.’” A week later, she began treatment.
Now Key is happy to be a member of Club NED (“no expiration date”), which is how the recovering IBC patients she corresponds with online poke fun at the medical jargon (“no evidence of the disease”) assigned to their conditions. “For IBC, doctors never call you cured or in remission,” she says.

That’s because recurrence of the disease is likely. “IBC can be quite amenable to chemotherapy,” says Disis. “It can make it melt away. The problem is it always comes back.” She hopes her research will one day lead to a vaccine that will keep IBC from returning.

In the meantime, Key isn’t whiling away the hours worrying about a recurrence. As a member of the IBC Foundation’s worldwide response team, she answers questions about the disease that come via the organization’s Web site. Team members, she says, avoid offering medical advice but make referrals to other sources of information on IBC and provide support to IBC patients and their families and friends. Key has also volunteered on U.S. Department of Defense (the second largest funding source for breast cancer research after the National Cancer Institute) panels to help prioritize proposals for cancer research dollars.

Networking for Solutions
Volunteers like Key are the backbone of the IBC Research Foundation, and they live all over the country. “We are a true 21st century organization, existing primarily on the Net,” says the organization’s executive director, Ginny Mason, who works from her home in northern Indiana. “People from all over the world contact us by e-mail and on our toll-free phone line. The phone calls are usually handled by me because of my medical background.” Mason is a nurse and an 11-year survivor of IBC.

The foundation is dedicated to moving IBC research forward. “Little research has been done on IBC because it is hard to find a group of IBC patients in a given area for study,” Mason says. “We are trying to remedy that by starting a BioBank and Clinical Database.”

The foundation, in collaboration with six other advocacy groups representing rare diseases, has formed the Genetic Alliance BioBank for the collection and storage of biological specimens such as blood, tissue and DNA samples, for research purposes. The database includes patient histories and medical records, including those of deceased patients.

Researchers wanting to use samples must submit a proposal to the foundation, which is evaluated by its board of directors and a medical advisory board. “We began in May and are already receiving proposals from researchers,” Mason says.

Working with researchers in a more business-like fashion, she adds, also guarantees that records and lab samples from IBC patients will be used for purposes approved by the foundation. “Now patients are driving the research process.”

Given that IBC has such a poor prognosis overall, every step forward is a reason to celebrate. The BioBank is certainly promising if it propels research toward a method for detecting and treating IBC earlier, or even a cure. And a relatively new treatment option may result in another small victory. Although new statistics for IBC have not yet been released, Mason says, “we can expect to see a modest increase in survival rates,” probably due to the use of taxane drugs (a category of chemotherapy drugs) and a change in treatment procedure that puts chemotherapy before surgery, with more chemotherapy afterward. “It’s become the standard of care for IBC,” says Mason. “To reduce the tumor load…to kill as many cancer cells as possible so when you get to surgery, they can see how effective the chemo has been. The second round of chemotherapy is cleanup.”

Still, Mason says, the best way for women to protect themselves against IBC is to know the signs, and act as their own health advocates. To that end the IBC Research Foundation will continue to emphasize education about the disease for everyone, including medical professionals.

In all of its efforts, the foundation – even without a long history and much money – is changing the scope of patient advocacy: IBC patients are helping to define the focus of research and are partnering in the research itself.

“We are passionate people,” says Mason. “We have something very important at stake – our lives.”

Can a Vaccine Prevent Recurrence?
As a resident at an inner-city hospital in Chicago, Mary “Nora” Disis saw a number of patients, including young women, with inflammatory breast cancer.

“To be honest, IBC got me into breast cancer research,” she says. “The disease was so incredibly aggressive.”

Today, Dr. Disis is researching ways to prevent the recurrence of breast and ovarian cancer through vaccines that boost the immune systems of patients who’ve been treated for these diseases. It’s a much-needed focus in women’s health: 30 percent of women diagnosed with invasive breast cancer will have recurrences within five years, and 80 percent of women treated for ovarian cancer relapse after the first treatment.

An associate professor of medicine in the oncology division, Disis heads the University of Washington Tumor Vaccine Group. She is also an associate member of the Fred Hutchinson Cancer Research Center.

“A vast majority of studies up until a few years ago were really done in dying patients – to shrink tumors,” Disis explains. “Overall, few responded to vaccinations. Our ploy with breast cancer was to take patients whose tumors could no longer be detected after surgery and chemotherapy, but whose chances of relapse were high, and treat them with a therapeutic cancer vaccination to stimulate their immune response.”

The vaccine, with which Disis has already experienced some success, was developed by “targeting proteins intimately involved in causing the breast cancer.” Disis focused her team on cancer patients’ immune response to a protein called HER-2/neu, which exists in normal cells at low levels but is over-expressed in 20 to 30 percent of breast cancers and in 10 to 15 percent of ovarian cancers. HER-2/neu is thought to contribute to causing these types of cancers.

Cancer cells, Disis says, are not foreign enough to the immune system to trigger an attack. “There’s a need for a cancer vaccine to show these proteins to the immune system in a way that makes them appear like an infection, that forces the immune system to say, ‘Wow! This is bad!’”

Disis and her group are working on separate vaccines: one for breast cancer and one for ovarian cancer. But because some of the same proteins are involved in causing both cancers, Disis says, “one of the questions we’re asking is ‘could there be only one vaccine?’”

In 1996, Disis’ team administered the already-tested vaccine containing cancer-related proteins stripped of their camouflaging parts to more than 60 women who had completed treatment for breast and ovarian cancers. The women received the vaccine multiple times over six months. Disis says her group is just now in the process of following up with these women.

“If we can increase the survival rate by more than 30 percent we would consider the vaccine potentially effective,” Disis says. “We’ll have data in three years that will tell us if this is likely to go.”

Typical Symptoms of IBC
(can include one or more)

• Swelling, usually sudden, sometimes a cup size in a few days
• Itching
• Pink, red, or dark-colored area, sometimes with a texture similar to orange peel (called peau d’orange)
• Ridges and thickened areas of skin
• A bruise that does not go away
• Nipple retraction
• Nipple discharge (may or may not be bloody)
• Breast is warm to the touch
• Breast pain (from a constant ache to stabbing pains)
• Change in color and texture of the areola

Source: Inflammatory Breast Cancer Research Foundation, http://www.ibcresearch.org/

Karen Reed-Matthee is the editor and co-founder of Seattle Woman.

©2005 Caliope Publishing Company