Does it seem to you that you often hear about someone being diagnosed with a “rare” cancer? I’m a survivor of a rare form of breast cancer— inflammatory breast cancer (IBC). But I’ve met hundreds of IBC patients either in person or online. How rare is rare?
All those rare cancers you hear about add up to a surprisingly large percentage of all cancers. The American Cancer Society puts the figure at 13 percent of all cancers, and a study by Carol E. DeSantis, M.P.H., and colleagues, in May 2017 estimates the number to be as high as 20 percent. A rare cancer is one with an incidence of less than six cases per 100,000 people in a given population. So while each of these cancer may be rare, taken all together, it’s not surprising that you hear about rare cancer so often.
On February 28, 2018, Rare Disease Day, medical professionals, researchers, and lay people gathered in Durham, North Carolina, for the first Meeting of the Duke Consortium for Inflammatory Breast Cancer. We were there to learn about some of the unique features of the disease and discuss ways to remove the barriers that all too often prevent prompt diagnosis and treatment of IBC. About 25 percent of those attending were patients and advocates. The theme — Connect NC for IBC — emphasized the demographic mix and top educational and medical institutions that make North Carolina (NC) an ideal place to model outreach for IBC.
Rare Disease Day was chosen for the meeting because many of the issues that make IBC mortality so high relate to its comparative rarity — about 1 to 5 percent of all breast cancers. Experts from all over the United States discussed their work with IBC and how its rarity makes its diagnosis and treatment more difficult. They shared highlights of their research and emphasized how collaboration will be necessary for advances in reducing deaths. Here are some of those:
Diagnosis. Because IBC does not typically present with a lump, patients don’t know that the pain, itching, swelling, and redness they experience might be signs of cancer. Therefore, they may be slow to seek a physician’s help. Even more alarming, when they do see their doctor, their symptoms are often minimized by the physician who may waste valuable time with a “watchful waiting” approach or multiple rounds of antibiotics. So one important focus of the meeting was to brainstorm ways to improve patient and doctor education about IBC.
Treatment. With a rare disease, experience matters. However, few local oncology clinics see enough IBC patients to gain sufficient experience in treating it. Noted IBC experts presented the latest information about the disease and best practices in its treatment. Speakers also highlighted specialized resources available at Duke and other institutions. I was excited to see that the doctor I consulted almost 20 years ago for a second opinion is now leading an IBC clinic at Duke. This specialized clinic will allow patients to get coordinated treatment for their IBC in one location.
Research. Other speakers gave highlights of their research into IBC. Some are studying what makes IBC different from other forms of breast cancer. Because IBC has such a high rate of metastasis, the factors that allow for the spread of cancer are another huge area of research. Another aspect speakers discussed was how to turn basic science discoveries into drugs in the clinic. Despite the varied nature of their approaches, each emphasized a huge problem for studying IBC and other less common cancers. It’s hard to get enough tissue samples to study. Also, a single institution may not be able to accrue enough patients into a clinical trial to get valid results.
Collaboration. All of these issues are connected by the need for collaboration. The experts need to work together to spread their findings to patients and local doctors to reduce misdiagnosis. Oncologists and hospitals need to be quick to refer patients to specialists, especially when cancer has metastasized. No one can be on top of the research for all the types of rare cancers, but there are people who specialize in each of them who do know the latest treatments and opportunities for clinical trials. Researchers need to share their tissue samples and research outcomes. In a world where professional advancement and institutional prestige are based on a competitive, rather than a collaborative, model, this last goal may be the hardest to achieve. But the women and men who gathered in Durham for this conference are making a start.