Because inflammatory breast cancer often is misdiagnosed as an infection and spreads quickly, a nurses observations may be critical in saving time and getting patients started immediately on the right treatment path.
IBC is a rare and highly aggressive cancer appearing in only 1% to 5% of all breast cancers, according to the National Cancer Institute. It often is misdiagnosed as mastitis, particularly among women who are breast-feeding or are fairly recently postpartum.
In most cases, there is no lump. Symptoms include redness, warmth or swelling of the breast, thickening of the skin, sometimes with a peau dorange or (skin of the orange) dimpling, persistent itching or an inverted nipple. These symptoms develop quickly over weeks or months and may not all be present at the same time. It is a particularly tough diagnosis in dark-skinned women because the clinical discoveries rely on redness or discoloration.
If it goes away after antibiotics, its not IBC, says Jacquelyn Lauria, RN, MS, APN-C, AOCNP, an advanced practice nurse at The Cancer Institute of New Jersey. And if its not responding, its getting rapidly worse.
At the time of diagnosis, the redness typically takes over about a third of the breast, she says. Over a few weeks, the skin may begin to harden.
As this is happening, the woman is becoming more and more frightened. The patient may feel isolated because even if she knows other women who have breast cancer, this is not the kind they have, Lauria says. And they likely have read the literature that says the survival rate is low. The five-year survival rate for patients with IBC is between 25% and 50%, which is significantly lower than the survival rate for patients with non-IBC breast cancer.
Psychologically, I think this is the most difficult breast cancer to deal with, Lauria says. Its right there. You wake up every morning and youre looking at it and youre saying Its getting bigger and bigger.
The diagnosis comes after clinical observation, not a pathology report. As soon as the diagnosis is made, a treatment plan has to happen quickly.
The first thing the patient needs is neoadjuvant chemotherapy, Lauria says. Normally a woman with breast cancer would have surgery, then chemotherapy, then probably radiation. Here the chemo comes up front because it is so rapidly growing and because of the risk of distant metastases.
The hope is to shrink the tumor to increase the chance of getting clean margins in surgery.
Michele Frank-Bazer, RN, MS, AOCNS, a clinical nurse specialist at the Department of Breast Medical Oncology at Memorial Sloan-Kettering Cancer Center, says a nurses role likely will include compassionate attention to psychological issues. Because mastectomy is necessary after an IBC diagnosis, nurses should be sensitive to patients sexuality and body image concerns, Frank-Bazer says.
Women with IBC cant reconstruct right away. They are told to wait at least two to three years to make sure everythings OK, and even then its discouraged, Frank-Bazer says. Thats different from other forms of breast cancer. If you had a small breast cancer and had a mastectomy, you could reconstruct at the same time.
Oncology nurses are the most consistent source of support for patients. They are with patients from diagnosis through radiation and throughout their care. They can help patients find a comfortable sleeping position to avoid pressing the tender breast and otherwise manage their pain. Patients often need narcotics, sleep aids and anti-anxiety medication.
They also help coordinate a medical care team consisting of a medical oncologist, surgeon and radiation oncologist, and palliative care for symptom management.
Victoria Miller, RN, BSN, OCN, faculty practice nurse at Monter Cancer Center in Lake Success, N.Y., says, You have to be on top of the pain. Cord compression could be a definite complication.
She says nurses should be alert if a patient calls with back pain or numbness or urinary incontinence. Those may signal cord compression and should be checked with an MRI, she said.
Patients also need an extensive disease workup because it needs to be determined whether there is disease outside of the breast. Women are sent for PET scans or CT scans to look at organs and soft tissues. If the cancer has spread outside the breast, treatments and discussions change.
If the patients not responding to treatment, the doctor will start discussing the risk benefits of additional treatment or possible palliative care, Miller says.
Initiating the End-of-Life Talk
Timing on discussing end-of-life care is tricky. Discussing too early might take away a patients hope. Some patients will have a long-term survival rate. But if the cancer has metastasized and the patient is not responding to treatment, she may need to get her affairs in order quickly.
Its not one discussion, Frank-Bazer says. Its a series of discussions. … Sometimes the patient initiates that conversation, and thats a big relief.
Much is known about the disease. It tends to be diagnosed at an earlier age than other breast cancers and can kill quickly often within 18 to 24 months of diagnosis. There is a high incidence of relapse and progression, and the incidence is much higher in women of African descent. The disease sometimes responds well to chemotherapy, and there are long-term survivors, but thats not always the case.
Hope on the HorizonRobert Schneider, PhD
Despite significant information on IBC, much is unknown. There hasnt been progress in 20 years, says Robert Schneider, PhD, associate director for Translational Research, NYU Cancer Institute and Co-director, Breast Cancer Research Program. The initial clinical trials combining chemo and radiation have saved quite a few lives and have had a big impact, but we havent extended survival past those original studies not by much.
Schneider is working to change that and his discovery, with researchers at the Cancer Institute at NYU Langone Medical Center last year, may have opened a key door.
The study, Essential Role for eIF4G1 Overexpression in Inflammatory Breast Cancer Pathogenesis on the Nature Cell Biology, found that a key gene was overexpressed in the majority of IBC cases. That overexpression was allowing highly mobile cells to form clusters and spread rapidly.
There are no approved drugs targeted specifically to the disease, but now that the gene has been identified, targeted therapies may be on the horizon, Schneider says.
We need a national consortium in which to run clinical trials and do research for IBC, Schneider says. Nobody sees enough cases that they can go it alone. We need to be able to work together, much the way that rare pediatric oncology is handled. We need large multi-institutional trials. Toward that end, Schneider says, researchers are building a large cohort of patients in Cairo, New York and Michigan.
One thing his research does indicate, Schneider says, is that a new experimental drug that seems to function much better than the emptor inhibitor rapamycin may ultimately be a new drug of choice used concurrently with other therapies to treat IBC.
Were seeing some good results in animal models, and we hope to bring it into the clinic in the next year, he says.