Eight years ago, Dian Corneliussen-James had surgeons cut out half of her right lung, a risky procedure she believes saved her life.
Though she thinks the surgery saved her from death from metastatic breast cancer, which had spread to her lung, she said she is "terrified to go off" the drug, Faslodex, that doctors say could be keeping her alive.
Her survival has prompted doctors and others to call her and patients with metastatic breast cancer like her "outliers" because they don't know why some patients with the incurable disease live a long time. Some say the answer to a longer life with metastatic breast cancer lies in targeted treatments, such as Faslodex, a drug designed to treat the cancer in women after menopause.
Metastatic, or stage IV, breast cancer is cancer that has spread to another organ. The median life expectancy of someone with metastatic breast cancer is 26 months, said Dr. Katherine Tkaczuk, professor of medicine and director of the breast evaluation and treatment program at the University of Maryland Cancer Center. About 155,000 Americans are living with metastatic breast cancer, and about 40,000 patients die annually in the United States, according to the Metastatic Breast Cancer Network.
A change of pace from chemotherapy, which targets all dividing cells in the body, such targeted treatments home in on just cancer cells, said Dr. Robert Donegan, a medical oncologist at Greater Baltimore Medical Center.
Targeted treatments were used first in the 1970s and, in some patients today, they are believed to control metastatic disease so it becomes more like a chronic illness than a fast-moving deadly one, Donegan said.
Targeted treatments have fewer side effects than chemotherapy and help patients maintain their quality of life, he said.
Corneliussen-James, who was diagnosed with HER-2 negative breast cancer in 2006, is beating the odds with injections of Faslodex every 28 days. She goes to her Johns Hopkins doctors from her Annapolis home to get the injections, which produce few side effects.
Though her doctors have offered the option, Corneliussen-James isn't willing to forgo the drug. "It doesn't negatively impact my quality of life," she said.
Going off it could have grave results. The drug appears to suppress the cancer and discourage it from reactivating, placing Corneliussen-James in a "remission" of sorts.
"We don't know if the cells are just dormant on their own or because of the drug," she said.
Marlene King, a friend of Corneliussen-James' and fellow member of Metavivor, an advocacy and support group Corneliussen-James founded with other survivors of the stage IV cancer, has had a different experience with targeted treatment.
She was diagnosed with breast cancer in 2008, and was told it had spread, or metastasized, in 2012. King went off treatment for the cancer in May, after trying several treatments that each worked for a few months and then stopped working, she said.
King decided in May that she didn't want any more drugs, at least for the time being. Her health wasn't great and her stomach and body couldn't handle the drugs.
"I decided it would be nice to take a break for a while," she said.
After a scan in July, her doctors told her there didn't seem to be any major growth of her tumors. Pending results of another scan at the end of September, she said, she will decide whether to continue her break.
While targeted treatments haven't worked for King, there is hope that with targeted therapies, the disease will begin to act more like a chronic illness, Donegan said.
"There's no guarantee," he said. "We need to clinically study. ... We really need good evidence and science to know drugs are going to work in less common genetic abnormalities."
Research on HER-2 positive breast cancer, a type 20 percent to 25 percent of patients with metastatic breast cancer have, is producing new and better drugs. Because so many exist, Donegan tells his patients diagnosed with HER-2 positive that it's possible the cancer can be managed to more like a chronic illness with the right drug.
Patients with HER-2 positive "live sometimes years," said Dr. George Raptis, associate chief of oncology and hematology at North Shore-LIJ Health System in New York. "We had never seen that in the past."
Raptis had a patient who lived 18 years with metastatic breast cancer before dying in 2012. Raptis said that that particular patient benefited from multiple therapies and that the biology of her cancer was sensitive.
Though some types of metastatic breast cancer have relatively successful targeted therapies, much work remains to be done, Donegan said.
"We'll ideally develop medicine that can target those less common types of breast cancer," he said.
There's room for the development of more therapies, which may mean looking more closely at the biology of cancer cells. Now, developing targeted therapies means finding ways to deny a fuel source that causes the cancer to grow and spread, Donegan said.
Doctors emphasize that the women who survive longer remain outliers. Even with targeted drugs such as Herceptin for HER-2 positive breast cancer, some patients respond to the treatment and some don't.
Tkaczuk gives her patients, some of whom have lived for years after diagnosis, different targeted treatments.
While many patients are not ready to deal with the diagnosis, she said, "the main misconception is that someone with metastatic disease is going to die right away."
That's not how Tkaczuk's patient Dianne Harrod reacted when she was diagnosed with stage IV cancer that had spread to her lungs in 2009.
"I said, 'What are we going to do about it?'" said Harrod, 65, who credits her positive attitude and her diet for her survival. "It's what you eat and what you do and don't feel sorry for yourself."
Since she was diagnosed, she's done a lot of targeted therapy, which she said "kind of keeps [the cancer] under control."
"I expect to live a long time," she said. "I just can't go out like that, you know, dying from breast cancer."