They say bad luck comes in threes. It’s a maxim that Nancy Marian can attest to.
Three generations of the Burnsville woman’s family suffer from Multiple Endocrine Neoplasia, a rare syndrome that puts them at high risk of pancreatic cancer. The disorder afflicts just one in 30,000 people.
So far, pancreatic cancer has claimed Marian’s mother and tumors have appeared in two of her three sons. Doctors have told Marian it may be only a matter of time before tumors develop on her own pancreas.
Nevertheless, Marian counts herself among the lucky few. Most patients afflicted with pancreatic cancer are diagnosed in the final stages of the disease, when it is already too late. For this reason, pancreatic cancer takes a heavy toll. According to the American Cancer Society, only 26 percent of those diagnosed with pancreatic cancer survive the following year. The long-term outlook is even worse: 94 percent of pancreatic cancer patients will die within five years of their initial diagnosis.
The Marians have faith that their story will turn out differently.
“We caught it early. I think we’re going to write a different page here,” Marian said. “That’s my hope.”
A SLOW DISCOVERY
It took decades for the true nature of the Marian family’s ailment to emerge.
Marian’s mother, Ruby Bauer, had suffered from severe stomach pains and ulcers since her 40s. Like many patients who have pancreatic tumors, Bauer’s symptoms were ambiguous. Patients suffering from pancreatic cancer pften complain of back or abdominal pain, digestive issues and unexplained weight loss — symptoms that could indicate a wide variety of illnesses.
Doctors didn't identify a growth until 1991. Bauer was given two options: Death or a 2 percent chance of survival after a procedure known as the Whipple, an surgery which takes off parts of the pancreas and other digestive organs connected to it in an effort to root out the cancer. The few who survive the procedure often become severe diabetics.
Bauer declined the surgery, resigned to live out the rest of her days in one piece. Six months later, it appeared the tumor hadn't grown. Bauer’s doctors rescinded their dire diagnosis, attributing her recurring stomach troubles to a case of severe inflammation of the pancreas, and sent her home.
Marian’s mother lived with increasing pain until 2001, when Bauer went back to the hospital. This time doctors put all the pieces together. Bauer’s medical team found tumors on her parathyroid gland—a hint that she was afflicted with Multiple Endocrine Neoplasia, a disorder that leads to tumors in three principal areas, the parathyroid, the pituitary and the pancreas. Her family history left little doubt: Bauer’s sister had also had tumors on her parathyroid, and it is suspected that two of her nephews also had endocrine-related tumors.
The team biopsied the tumor on Bauer’s pancreas and found that it was indeed a tumor typical of MEN Syndrome. The growth was producing excess gastrin, a digestive juice, which explained the persistent ulcers and stomach pain.
The syndrome is passed down genetically. It wasn’t long before Marian herself had her own wakeup call in fall 2003. At the time, Marian’s doctors had discovered tell-tale tumors on her parathyroid gland. A battery of tests revealed Marian had MEN Sydrome as well, though her pancreas was tumor free—for the time being.
“I remember them saying ‘Well, you’re good for now,’” Marian recalls. “That’s quite a gray cloud hanging over your head.”
The good news was that with check-ups every six months, doctors would be able to detect any growths on Marian’s pancreas before they could spread.
A FAMILY AFFAIR
It was too late to save Marian’s mother, however. The tumor on her pancreas was now the size of a lemon and had metastasized into her lymph nodes. Doctors told her there was nothing they could do.
Bauer died in 2008, at the age of 75. Little did Marian know that pancreatic tumors were already taking root in two of her sons, both still teenagers. Neither showed any sign of ill health, though her 14-year-old son, Evan, was a bit on the short side.
The next revelation came after doctors suggested that his lack of height could be tied to a hormonal imbalance, and they referred the family to an endocrinologist. After hearing about the family’s history, the endocrinologist immediately screened both Evan and his mother for tumors associated with Multiple Endocrine Neoplasia.
This time, Marian felt her bout with pancreatic cancer had arrived.
“I was thinking, ‘It’s my time,’" Marian said. "‘You’re good for now’ rang in my head. I really thought that I was going to be the one to have a tumor on my pancreas.”
To her surprise, all her tests were clear. Evan wasn't so fortunate: He showed had tumors in all three key areas, including a small tumor on his pancreas.
“I couldn’t believe that he was so young and it was already manifesting itself. I was absolutely floored,” Marian said. “That just made my heart drop.”
The final blow came six months down the road. Initially, it seemed that Marian’s two older sons had been spared. Mike, 22, was in the clear. A CT scan on her middle son, 16-year-old Jacob, showed no tumors. In fact, he had three, which were obscured from view until the medical team at Mayo used another test, an endoscopic ultrasound. Such oversights are common, Marian said: The pancreas is lodged deep inside the abdomen, which makes it difficult to see.
For Jacob, it was a stunning reversal.
“That was a big shock to me. I never knew anything was wrong,” Jacob said of his diagnosis. “It was hard for me to grasp at first, but now I feel really, really privileged that my mom was eager to find out if any of us have it. It could have been a huge deal if no one checked until I turned 60 and the tumor had already spread everywhere.”
A POSITIVE TURN
For now, life goes on as usual. The Marians feel fortunate to have discovered the growths early, a luxury afforded to just 8 percent of all pancreatic cancer patients due to a complete lack of early detection methods, according to the ACS.
At present, both boys are healthy. Evan, now 16, is an honors student at Lakeville South. Jacob, 19, is a sophomore at the University of North Dakota. He hopes to become a nurse.
The boys’ tumors remain wild cards.
“These tumors could stay as they are for 20 years or they could all of a sudden turn on and, 'fwoomp,' start to grow,” Marian said. “There’s no rhyme nor reason to it.”
They're still small—less than 2 centimeters wide—which means the boys are not yet eligible for the Whipple, a surgical technology that remains the only curative recourse even though it is well over 20 years old. The surgery is so invasive that doctors only attempt it if the tumors have shown clear increases in growth and malignance.
As a result, Marian has thrown her efforts into the Pancreatic Cancer Action Network, a non-profit that provides support to families and advocates for increased federal funding into pancreatic cancer research.
The Marians' struggle is statisically exceptional, but they are not alone. The chance of having pancreatic cancer is about 1 in 71. About 44,030 new cases of pancreatic cancer were diagnosed in 2011, according to an ACS report, and about 37,660 people died of the disease.
Though pancreatic cancer is the fourth-leading cause of cancer death, just 2 percent of all federal funds go towards research into the cause and cure. In part, this is due to a relative lack of survivors who might otherwise become advocates, said Marian and Brenda Coleman, a 10-year survivor and the Twin Cities coordinator for the organization.
"I'm not the only one. There are handfuls of us, though not as many as most other cancers," Coleman said. "We're trying to raise enough visibility to attract the survivors who are out there to rally. The survivors are the voice of progress."
The group’s goal is to increase the five-year survival rate to 12 percent by 2020.
At present, the non-profit is simply trying to increase awareness of this low-profile disease. In this regard, the group has found a friend at Burnsville City Hall. Last week, the city council proclaimed November to be Pancreatic Cancer Awareness month.
The group is also hosting a series of events around the Twin Cities, the first of which will happens today, Oct. 25, at the Town Hall Brewery in Minneapolis. Marian is hoping her neighbors come out and support the cause. The event runs from 5 to 7 p.m.
Marian is confident that they can make a difference.
“I put my energies into doing something that I feel can make a positive impact because I’ve got," Marian said. "Time is on our side."