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The BRCA Connection
With little prompting, Ruth Berman (not her real name), a teacher from the Philadelphia suburbs, can reel off a dozen names of people she knows -- or knows of -- who have battled pancreatic cancer.
"I have a girlfriend who lost her aunt, a girlfriend who lost her brother, a girlfriend who knows three people in her synagogue ... my daughter's close friend's grandmother had it. And my parents' best friend. I count in my little circle 12 people. And if I know of 12 people, chances are there are more out there."
There are many more, including Berman's own father, dead at age 68, and her husband, dead at the age of 70 some 30 years later.
The common thread? All are or were Jewish.
Swimming in the Jewish genetic gene pool puts us at higher risk of certain diseases than the general population, including breast cancer, Tay-Sachs and familial dysautonomia, all of which have been the focus of well-orchestrated publicity campaigns.
Thanks to recent research, pancreatic cancer needs to be added to the list, according to the Pancreatic Cancer Action Network.
Along with smoking, following a diet rich in red meat and other fats, and blowing out 60 or more candles on your birthday cake, researchers are increasingly looking to heredity -- especially roots within the Eastern European Jewish population -- as a risk factor for developing one of the deadliest of cancers.
"Individuals with a BRCA 2 mutation" -- the gene mutation most commonly associated with breast and ovarian cancers -- "are also at higher risk of developing pancreatic cancer," according to the Chicago Center for Jewish Genetic Disorders.
Additionally, the center reports that "It is known that individuals of Ashkenazi Jewish ancestry are at higher risk than other religious groups to develop pancreatic cancer, although it is unclear why. The lifetime risk to develop pancreatic cancer is 1 percent, but in a Jewish individual with a BRCA2 mutation, that risk may be as high as 7 percent to 10 percent. In one study of European families with at least two first-degree relatives with pancreatic cancer, 19 percent were found to carry a BRCA2 mutation."
Researcher Jonathan Brody, M.D., of the Thomas Jefferson University and the Kimmel Cancer Center put those words into terms a lay person can understand.
"We see a higher prevalence of these types of mutations in the Ashkenazic population," Brody said. "If two of your first-degree relatives have pancreatic cancer, and you have the particular genetic mutation, there is a significant increased risk you would get some sort of cancer, including pancreatic.
"People with multiple family members with cancer -- specifically pancreatic, ovarian and breast -- should be genetically tested," said Brody, who works in the hospital's department of surgery. "This genetic information could translate into an early detection strategy for the individual and therapeutic implications down the road."
Because ovarian and breast cancer are more prevalent, the researcher noted, the larger studies have tended to concentrate on those genetic links. Only in the last decade or so has there been a focus on pancreatic cancer patients who harbor these mutations, said Brody, whose work involves developing novel therapeutic strategies to treat cancer of the pancreas.
His hospital has established the Jefferson Pancreas Tumor Registry to track the frequency of the cancer in families with a history of the disease.
Sarah Charles, a genetic counselor with the Kimmel Cancer Center, said that more than 100 families are enrolled in the registry, which has links with the National Familial Pancreatic Tumor Registry at Johns Hopkins Hospital in Baltimore.
On the national level, about 16 percent of the familial cases documented on the registry "are among folks of Eastern European Jewish descent," Charles said -- a hugely disproportionate number, considering that Jews account for an estimated 2 percent of the population of the United States, according to the most recent figures available.
Those are numbers the Philadelphia affiliate of the Pancreatic Cancer Action Network hopes to drum into the collective Jewish psyche. They want the community, informed, vigilant -- and tested, if they fit the BRCA criteria.
The organization represents pancreatic cancer patients and survivors as well as family members who have lost loved ones. In addition to sponsoring educational events and serving as a clearing house for information, the network provides call-in service to help patients take control of their medical care.
Ruth Berman and fellow members of the advocacy group are reaching out to the Jewish world not only to raise the disease's public profile, but also to marshal the same resources being channeled to more well-known forms of cancer.
Through fliers, synagogue newsletters, newspaper articles and an annual walk, they are shining a spotlight on the scourge they say receives the fewest federal dollars of all the major cancer killers: an estimated $89 million in 2009, representing 2 percent of the National Cancer Institute's cancer research budget last year.
"The problem with pancreatic cancer is that awareness is very limited. Of course, it has one of the highest mortality rates, which is probably one of the reasons why people are not seeking to donate a lot of money -- they feel it would be useless," Berman said. "It's absolutely considered a death sentence. Psychologically, people think you're done for, but that's exactly why research has to be increased."
"Money is an issue, without a doubt," agreed Jefferson's Jonathan Brody, who traveled to Washington, D.C. this year to urge legislators to plow more funding into pancreatic cancer research.
According to the advocacy group, pancreatic cancer is the fourth-leading cause of cancer death in the United States. This year, more than 43,000 people will be diagnosed, and 38,800 people will die from the disease.
People such as Berman's husband, who fought the good fight for years after nagging back pain drove him from internist to neurosurgeon to oncologist before an astute specialist in gastrointestinal cancers put a label on his condition.
Sadly, the teacher said, that experience is all too common.
Backaches like the ones her spouse suffered afflict some 80 percent of Americans, the vast majority of whom do not have pancreatic cancer. Other vague symptoms that accompany the disease -- weight and appetite loss, nausea, jaundice, changes in the stool -- can also be misdiagnosed or overlooked.
There are no reliable detection tools to catch the disease in its earliest stages, when a tumor is small enough to be surgically removed, the American Cancer Society reports. Seconding this, the US Preventive Services Task Force recommends against routine screening for pancreatic cancer.
"That's why we stress the need for genetic testing," Brody said. "If you come from a family that has multiple cancers -- an aunt with ovarian, mom with breast, and people showing up at family reunions with cancer -- that family should be tested" for genetic mutations like BRCA.
There was no such warning for Pia Miller. Weird feelings, yes -- a nerve pain that ran through her at night, a stomach that "felt strange." But beside a cousin of her husband's and the late actor Michael Landon, the Yardley art teacher had zero knowledge of pancreatic cancer that Presidents' Day of 2006 when a doctor uttered words that would change her life.
You have a mass on your pancreas.
After a six-hour operation and a six-day stay in Massachusetts General Hospital, Miller came home with 60 percent of that organ removed. Also gone were her spleen and her gall bladder. What she lacked in body parts, however, she more than made up for in newly acquired medical knowledge.
A biopsy showed that two out of seven nodes had tested positive for cancer, and that the disease had encroached through the margins of the pancreas. The official name of her disease: adenocarcinoma. Prognosis: pretty lousy.
"I knew my chances of living were not good," Miller said bluntly.
She was 58, with a husband, two grown children, three grandchildren and a job she loved.
"Nobody was talking about pancreatic cancer when I was diagnosed," Miller said. "People gave me this puppy-dog look when I told them about it. Even at a support group with other cancer survivors I got that look."
Months of chemotherapy, radiation and clinical trials sent her into remission for two years and allowed her to resume teaching. But a bout of hepatitis in November 2008, possibly drug-induced, was a setback, and although an ensuing CAT scan initially seemed clean, further testing showed the cancer had, indeed, returned.
Another eight-month round of chemotherapy -- with its accompanying fatigue, nausea, vomiting and hair loss -- led to another remission. But the honeymoon was short, and in April of this year the cancer made its unwelcome comeback.
"My understanding is that the body becomes resistant to anti-cancer agents after a while," said Miller. Her hope now lies in proton therapy, a relatively new approach involving a machine called a cyclotron. The Roberts Proton Therapy Center at the Hospital of the University of Pennsylvania, where she is undergoing the treatments, is one of only seven hospitals in the country equipped to offer the protocol.
Throughout the ordeal, she has remained upbeat, Miller said. She and her husband Steve continue to travel -- Greece, France, the Baltics, two cruises -- and Miller regularly wields her paintbrush to produce the color-suffused landscapes she loves.
"I've had more than 40 procedures, but every chance we get we go out to dinner, walk, go bike riding, plan trips. My life is by no means horrible. I lead a glorious life."
She also uses her experience as a soapbox.
Since stumbling across the website of the Pancreatic Cancer Action Network in 2007, the educator attends health fairs, reaches out to doctors and buttonholes everyone who will listen to talk about the disease. She also organized the first fundraising walk for the network in 2007, with 600 participants netting $150,000.
In addition to single-handedly raising about $35,000 for education and research, Miller offers herself up as an example of hope for newly diagnosed patients.
"I'm asked by many of my Jewish friends how to make sure pancreatic cancer doesn't happen to them," Miller said. "I tell them just to be aware. Don't live in fear, but if your doctor tells you backaches are normal, or stomachaches are normal, you have to pursue it further."
Fredda Sacharow is a frequent contributor to Special Sections.