Aretha Franklin, the beloved “Queen of Soul,” died Thursday at age 76 from advanced pancreatic cancer of the neuroendocrine type. As the singer is mourned worldwide, her tragic death is also helping shed some light on a disease experts say is particularly deadly and in need of funding, research and overall awareness.
Pancreatic cancer is difficult to detect early and spreads quickly in the body. The majority of patients, 94 percent, will develop the adenocarcinoma type, which has an overall five-year survival rate of 8 percent. By comparison, the survival rate is 90 percent for breast cancer, 65 percent for colon cancer, and 18 percent for lung cancer.
Franklin had a less common type of pancreatic cancer, which is characterized by tumors that form in the pancreas’ hormone-making cells. While this form of the disease has a slightly better prognosis and spreads less quickly than adenocarcinoma, researchers say both kinds are in dire need of awareness.
“Pancreatic cancer is projected to become the second leading cause of cancer death in the United States by 2020,” Dr. Diane Simeone, professor of surgery and director of the pancreatic cancer center at NYU Langone Health, told HuffPost. “What has been traditionally thought of as an uncommon cancer is actually increasing in frequency.”
In the United States, an estimated 55,440 new cases of pancreatic cancer will be diagnosed and about 44,330 people will die from the disease this year, according to the American Cancer Society. For experts, the distressing single-figure survival rate means it’s one of the deadliest major cancers and more must be done to help those at risk.
Pancreatic cancer is projected to become the second leading cause of cancer death in the United States by 2020. Dr. Diane Simeone, director of NYU Langone Health’s pancreatic cancer center
Dr. Christos Fountzilas, an oncologist specializing in pancreatic cancer at Roswell Park Comprehensive Cancer Center, told HuffPost that what makes this type of cancer particularly deadly is that it spreads early, impacts vital functions of life and is resistant to traditional types of treatment.
“When you compare it to other cancers, pancreas cancer tends to be a cancer that is not sensitive to the usual chemotherapy medications,” he said.
And while neuroendocrine type spreads more slowly, he adds, once it grows “outside of the pancreas, they can still be lethal, even though they don’t move as quickly as the adenocarcinoma. ”
While data surrounding pancreatic cancer is far from encouraging, experts told HuffPost there is promising research in the works around early detection and treatment. Still, there is a long road ahead and they hope Franklin’s death will help raise awareness around risk factors and the need for more research.
“I don’t think pancreatic cancer has gotten enough attention. I don’t think it’s as supported as it should be by agencies.” Fountzilas said. “We are better in terms of awareness compared to 10 years ago, but there’s still work to be done.”
For Simeone, that work includes changing people’s pessimistic attitudes toward fighting the disease.
“There’s actually a lot of things that can be done and it’s very important that information gets out to the public about the risk factors and new treatments,” she said, adding that those diagnosed with pancreatic cancer should always consult with an expert and if possible participate in clinical trials.
“Because that’s the only way we’re going to move the needle in the future and not have unfortunate deaths of pancreatic cancer like we’re witnessing [this week] with Aretha Franklin’s passing.”
Know Your Risk
While there have been advances in early detection for other major cancers, currently there are no effective tests that can be routinely performed to detect pancreatic cancer. This is particularly troubling because early stages of the disease usually show no symptoms.
Runners approach the finish line at the Pancreatic Cancer Action Network’s PurpleStride 5K Run/Walk on June 16, 2012.
“There are imaging tests that can be done of the pancreas, but they’re not quite as sensitive or specific as we need,” Simeone said. “There is a lot of work being done in trying to develop an early detection blood test, and some of that work is quite promising. But it’s still several years away from being validated and approved by the FDA for routine use.”
Doctors know the disease is slightly more common among men and black Americans, and individuals diagnosed are often in their 60s and 70s. But a lack of accurate screening methods means it’s crucial that people know certain risk factors associated with pancreatic cancer.
For example, there is a well-studied correlation between pancreatic cancer and smoking and smokeless tobacco use. But there are other risks doctors say should be kept in mind.
The pancreas, an organ hidden in the upper-back part of the abdomen, creates insulin and digestive enzymes, and is critical in controlling blood-sugar levels. Thus, both diabetes and obesity are also thought to be linked to the disease.
“The increase in pancreatic cancer that’s been happening over the last decade is actually most tightly related to the increase in incidence of obesity and diabetes in our country,” Simeone said.
But, Fountzilas adds, “it is unclear whether people develop cancer because they have diabetes or diabetes is a very early sign of development of pancreatic cancer.”
Genetics also play a role. According to Simeone, somewhere between 10 and 12 percent of pancreatic cancer patients actually have a heritable form of the disease. And people who have gene mutations that increase their risk for breast cancer, colon cancer and melanoma are also at higher risk of pancreatic cancer.
The hereditary impact of the disease can be seen in Jimmy Carter’s family. The former president lost his father, two sisters and brother to pancreatic cancer and has joined efforts to raise awareness around the disease.
“We recommend genetic testing if people have two or more family members that have had pancreatic cancer,” she said. “Or if there is a known germline mutation in a single family member with pancreatic cancer.”
Know The Signs
Signs of pancreatic cancer often don’t manifest until the cancer is in more advanced stages, but doctors still recommend individuals at risk know what to look for. Symptoms of the disease include unexplained new diabetes, unexplained weight loss, upper abdominal pain radiating to the back, and yellowing of the skin and eyes, or jaundice.
“Unfortunately, many patients have vague symptoms and these can go on for several months,” Simeone said, adding that many people don’t realize that the symptoms points to cancer as opposed to gastritis or reflux.
She recommends that people with symptoms seek professional expertise right away because there are treatment options available for pancreatic cancer. Fountzilas agrees.
There are a lot of patients with pancreatic cancer that never make it to an oncologist because people believe there is no hope. Dr. Christos Fountzilas, a pancreatic cancer specialist at Roswell Park Comprehensive Cancer Center
“There are a lot of patients with pancreatic cancer that never make it to an oncologist because people believe there is no hope,” he said. “But no matter, I think every patient who has pancreatic cancer deserves an evaluation by a medical oncologist, a surgical oncologist because treatment options do exist. Even if not curative, at least treatments that can help people maintain their quality of life and live longer.”
Without a known cure, surgically removing a tumor paired with chemotherapy to lower the risk of recurrence is a patient’s best chance at survival. But as of now, less than 20 percent of patients are generally candidates for surgery because by the time the cancer is detected, it has spread beyond the pancreas.
That’s why Fountzilas and Simeone feel strongly that more must be done both to improve early detection and to develop more effective therapies to treat the disease. With that in mind, Simeone will be leading a national initiative, called Precision Promise, at the beginning of 2019 that seeks to rapidly test different combinations of drugs to find a better way to treat pancreatic cancer.
“Just like any tough medical problem we have, when resources are put in to solve a problem and everybody works together, then that’s when advances can be made,” she said. “Pancreatic cancer in general has been underfunded from a research perspective, and I think that needs to change. And the time for it to change is now.”
Clarification: Additional information has been added to this story to clarify which type of pancreatic cancer Franklin had, as well as its characteristics and how it differs from the most common type.
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