By Sharon Wisch-Ray
UT Southwestern’s Pancreatic Cancer Prevention Program wants to do for pancreatic cancer what colonoscopies did for colon cancer: Catch it before it’s too late. This multi-disciplinary team of gastroenterologists, radiologists, surgical oncologists and geneticists want to help people who are at high risk for the disease. This is of particular interest to the Jewish community, which has a higher incidence of pancreatic cancer than the general population.
It is widely known that people who carry mutations in the BRCA1 and BRCA2 genes have a higher risk of breast and ovarian cancers. In fact, 1 in 40 Ashkenazi Jews, both men and women, carries a BRCA gene mutation — more than 10 times the rate of the general population, according to the National Cancer Institute (cancer.gov) and Sharsheret (sharsheret.org).
However, what is not common knowledge in the Jewish community are the other cancers influenced by BRCA1 and BRCA2 mutations. Among them is pancreatic cancer. Rare, but particularly deadly, pancreatic cancer represents only 2 percent of all cancers. Yet, it is responsible for 40 percent of all cancer deaths, according to the Pancreatic Cancer Action Network, making it the third leading cause of cancer-related deaths each year.
What makes it so difficult to treat is that people often do not feel symptoms of the disease until it is advanced and aggressive.
According to the National Cancer Institute, “Pancreatic cancer is difficult to detect and diagnose for the following reasons:
• There aren’t noticeable signs or symptoms in the early stages of pancreatic cancer.
• The signs and symptoms of pancreatic cancer, when present, are like the signs and symptoms of many other illnesses.
• The pancreas is hidden behind other organs such as the stomach, small intestine, liver, gallbladder, spleen, and bile ducts.”
While having a BRCA1 mutation raises your risk of pancreatic cancer by only about a couple of percentage points (to 1-2 percent), a BRCA2 mutation can increase that lifetime risk to 5-10 percent for people who have the mutation, explained Dr. Theo Ross, a professor of Internal Medicine and the director of the Cancer Genetics Program in the Harold C. Simmons Comprehensive Care Center at UT Southwestern Medical Center. She is a member of the program’s multidisciplinary team as well.
Ross explained that in addition to the BRCA mutation, family history is key. “If you have a first-degree relative with pancreatic cancer or two others with pancreatic cancer, such as a cousin… you have a familial risk.” These are the folks who could be screened in the Pancreatic Cancer Prevention Clinic for precancerous cysts and followed closely thereafter.
Time to get tested
Ross stresses the importance of genetic testing for all members of the Jewish community. “The number of people that have a BRCA mutation and know they have a mutation is a small percentage,” she said. “Maybe 15 percent of people with the mutation know about their mutation. If they don’t know, they don’t know about the pancreatic cancer risk.” Ross encouraged people to get tested even if it’s with one of the at-home tests to start. One such test is available at Color.com. Color’s BRCA Test sells for $99. The test can be ordered by your private physician or an independent physician belonging to an external network. The company sends you a saliva collection kit and prepaid return label for you to send your sample back in. Ross says the test is solid. However, it’s important to review your results with a genetic counselor. And, she says, just because you test negative for BRCA1 or BRCA2 doesn’t mean you are in the clear. There are many genes that influence hereditary cancer syndromes. If you see patterns in your family, even if you test negative for BRCA1 and BRCA2 mutations, it’s important to discuss your family history with a genetic counselor.
She became interested in genetics when she was in medical school and started putting two and two together: The number of people in her family who had cancer was alarming. Still, it took years for Ross to ultimately discover she is a carrier of the BRCA1 mutation herself. She discovered this only after she survived melanoma, one of the many cancers enabled by the BRCA mutations. She tells her story in her 2016 book A Cancer in the Family: Take Control of Your Genetic Inheritance. The book, which is now available in paperback on Amazon and from other resellers, is a resource guide for everyone and anyone who is concerned about their cancer risk. Despite the technical topic, it is easy to read.
UT Southwestern’s Pancreatic Cancer Prevention Program was launched in 2016. Dr. Nisa Kubiliun, the director of the program, believes it is poised to make a difference in the lives of those who are at high risk for pancreatic cancer. “Back in the day, nobody realized that pancreatic cysts were a significant cause of pancreatic cancer. They were largely ignored,” she explained. Over time, pancreatic cysts can evolve into tumors.
“When we started, we thought there were people who had pancreatic cysts and needed to be monitored,” she said. Kubiliun said that watching the progression of precursors to pancreatic cancer is a relatively new process. She believes that pancreatic cancer probably develops over the course of many years, but people have only been watching cysts recently.
“Our greatest opportunity is to prevent pancreatic cancer in the first place,” she says. By monitoring changes in the pancreas over time, surgeons can remove a cyst or precursor lesions of the pancreas that look troublesome before they actually turn into cancer.
Kubiliun says the program is growing rapidly, much under the auspices of its benefactor Jewish community member Nancy Wiener Marcus.
“I met Nancy shortly after we launched the program. From the minute I met her she’s been a force for really catapulting the program into the next stratosphere. She’s gone to incredible lengths to get the word out to the community. Her energy, her passion and her desire are inspiring.”
Currently the program is seeing about 20 new patients per week. Kubiliun explains what a good candidate for the program is: anybody with a strong family history of cancer; anybody with a history of pancreatic cancer or cysts of the pancreas; and anyone with a known genetic mutation.
A referral from a physician is not necessary. “They can simply go to our website (https://utswmed.org/conditions-treatments/pancreatic-cancer-prevention/) or call (214-645-8300) and say, “I need to be seen, can I be evaluated?” Kubiliun said they have never turned an individual away. “There is no downside for reaching out and asking us to take a look at your medical history,” she says.
One such person who joined the program is glad she did. Suzanne Calibretti, who is BRCA positive, was being screened with MRIs when the team noticed a change in her pancreas over time. “She was at the step right before it becoming cancer,” said Dr. Kubiliun. “Had she not had that operation, had she not had that pretumor removed, it would have been a completely different ball game. I can’t emphasize enough the greatest opportunity is to prevent pancreatic cancer in the first place.”
If you’ve ever met Nancy Wiener Marcus, then you know she has a heart of gold. About five years ago, Marcus wanted to do something important for her 70th birthday. “I wanted to give some money toward something to help and I wanted it to go toward pancreatic cancer.” Initially, Marcus gave an endowment in honor of her own UTSW gastroenterologist Dr. Mack Mitchell for a fellowship. “That way it could go toward learning about the pancreas and other GI problems,” she explained.
However, Marcus felt the urge to do more and later was introduced to Dr. Kubiliun over lunch one day.
“What do you need?” Marcus said she asked Kubiliun. “It was pulling at me. I needed to do something else. You have to be doing something to make this world a better place.”
Initially, Marcus was going to provide $5,000 for a freezer for storing cells. “By the time I got home, I’d decided I’d start a fund of about $100,000 to get this thing (the Pancreatic Cancer Prevention Program) going.
Marcus is passionate about getting the word out about the program. “My goal is to bring awareness and knowledge to our community. Most doctors don’t know this program even exists. And the Jewish community members don’t know that if they have a mother or aunt who had pancreatic cancer, they can go get tested and see where they are on the spectrum, so they can be followed and detect and do something before it gets into full-stage pancreatic cancer.”
Marcus hopes to bring an education program to the Jewish community in November during Pancreatic Cancer Awareness Month.
“I keep asking myself, what else can I, Nancy, in my own little way be doing to bring about awareness and knowledge to our community so we can arrest the rapid growth of this disease?”
Marcus emphasized that being able to give the money has been a blessing, but if people don’t know about the program, “What’s the point? We need people to get people to take care of themselves and their family.”
A case in point
Many in the Dallas Jewish community know about Jamie Lambert, now 48. The TJP covered her story in November 2016, 16 months after her diagnosis of pancreatic cancer and her Team Jamie Facebook page has about 750 followers.
“The cancer diagnosis in July 2015 was not the first health scare. In July 2014, her gallbladder was taken out and she was discovered to have pancreatitis. She never really recovered.
“She went to the doctor a year later, worried when her body started turning yellow. An ERCP (endoscopic retrograde cholangiopancreatography) found something, and led to a biopsy.
“‘(The doctor) comes in the room, and he says, ‘You have pancreatic cancer,’ Lambert said. It was adenocarcinoma. ‘And he walked out of the room. We knew you don’t live when you have pancreatic cancer.’
“She found an oncologist she liked, Dr. Michael Savin at Medical City, who later closed his office in March and moved to Portland. Savin didn’t have good news.
“‘I was given six months, 12 months to live,’ Lambert said.
“Her stage 3 cancer was too far along for a Whipple surgery, a common method for dealing with pancreatic cancers. But it’s hard to find the disease in time. It was a devastating blow.”
The UTSW Pancreatic Cancer Prevention Program didn’t exist when Jamie was diagnosed. And in fact, a virtual expert on pancreatic cancer today — as many people become when they are afflicted with a dangerous disease — she didn’t know about the program until the TJP shared it with one of her sisters.
Lambert would have met the criteria to be followed, having had pancreatitis and a mother and grandmother that had breast cancer. Interestingly, neither she nor her sisters tested positive for BRCA mutations; however, as Dr. Ross stated earlier, those are not the only mutant genes responsible and testing genes like PALB2 for mutations is key.
Dr. Kubiliun explains that when someone develops pancreatic cancer so young, at age 45, and has a positive family history of cancer, it’s even more important that her first degree blood relatives are followed by a program like the one at UT Southwestern. Now that they know about it, those wheels are set in motion.
In the meantime, Lambert, who has been living with pancreatic cancer for about three years, is going about living her life with her husband Kevin and their three children. “I take care of my kids, pick them up from school, exercise and try and connect with other people who are going through what I am going through.” Jamie, who undergoes chemotherapy twice a month, lives every day to its fullest. “Life’s too short not to,” she says.