By Maura Lerner - McClatchy Newspapers

NORTH MANKATO, Minn. - When Trisha Anderson developed lung cancer at age 2, her doctors were mystified. Children, after all, almost never get lung cancer.

They had no idea then, in 1982, that Trisha's family harbored a genetic disease so rare that it didn't even have a name. And that was only the beginning.

Trisha's parents, Julie and Chuck Schoettler were told that her case was only the third of its kind in the world. So when she got through treatment and recovered, they breathed a sigh of relief.

But a few years later, her little brother Adam developed lung cancer, too. It was just like Trisha's, only worse. In the fall of 1987, he died in his parents' arms at age 4.

Ever since, the Schoettlers and their five surviving children have learned to live in the shadow of this illness, known today as PPB, or pleuropulmonary blastoma.

Now, they are using their blood, sweat and tears to try to help scientists unravel the medical mystery before it attacks another generation.

In this case, "they are the Rosetta stone," said Dr. Louis (Pepper) Dehner, a former University of Minnesota pathologist who studied the family and named the disease.

"The Schoettler story opened up the chapter on this disease, that there was a family predisposition," said Dehner, now a professor at Washington University in St. Louis.

And they might be the key, he said, to finding the cause.

Four of the Schoettlers' six children had some form of the disease - including two sons who have had benign lung tumors removed.

The Schoettlers have joined in an extraordinary genetic research project, which will be presented Sunday in Geneva, Switzerland, at the first international medical conference on PPB.

Nearly 100 members of the extended family have donated blood samples for DNA testing at the urging of Julie Schoettler, who happens to be a nurse. About a quarter of all PPB cases are believed to be inherited.

In January, she lured nearly 70 of her husband's cousins, aunts and uncles to a church basement in Mankato, Minn., for a combination pizza-and-DNA party. Then she rounded up more relatives at a family picnic in August. "This is kind of a passion of mine," she said.

The scientists set up a table and drew blood. "When cancers run in families, you have really a unique window to go in and use genetics to find out what's causing it," said Dr. Ashley Hill, a Washington University scientist who went to Mankato to collect the samples.

If a genetic mutation can be found in this family, Hill said, it could point the way to a treatment, and even shed light on other types of cancer.

Trisha, now 26 and married, was there too, ready to apply pressure in case anyone got cold feet. "I wanted to be there to let them know that this is for the future of our kids," she said.

When a child gets cancer, one of the most common questions is whether other children in the family are at risk. The answer almost always is no, said Dr. Jack Priest, a cancer specialist who treated Trisha and Adam Schoettler at Children's Hospital in St. Paul, Minn.

The Schoettlers, unfortunately, were the exception.

At first, everyone assumed that Trisha had pneumonia. But when her lung collapsed, her mom rushed her to Children's Hospital. And that's where Julie Schoettler saw the pained look in the doctor's eyes and heard the words: "highly malignant."

A few years before, her husband, Chuck, had survived thyroid cancer. Julie, then the mother of three young children, could hardly believe she was hearing the words again.

The doctors gave Trisha a 50-50 chance of survival, based on the limited experience with such a rare tumor.

Trisha had surgery and chemotherapy, with all the side effects imaginable, her mother recalled. After two years, her doctors suspected the treatment itself was killing her, and ended it. "She just blossomed," said Julie. "It was a joy to watch."

While Trisha was in treatment, Julie had given birth to her fourth child, Adam. Three years later, his symptoms began appearing. Doctors assured her that it wouldn't happen twice, but it did.

When doctors found Adam's tumor, it had already spread. And the disease still didn't have a name. Julie spent three months living at the Ronald McDonald House in Minneapolis while Adam was in treatment. Once, she brought his chemotherapy home and Adam fed it to the cat - which shed for weeks afterward, Julie recalled.

At one point, Julie took her children to meet Dehner, who was then at the University of Minnesota. As a pathologist, he had helped diagnose both kids' cancers. But he had never met them.

"I said, 'You've done nothing but look at slides from these guys, and I want you to put a face to it,'" she said. Then she asked him to come up with a name for the illness. He did, and later wrote a scientific paper on PPB based in part on the Schoettlers.

By the summer of 1987, Adam's cancer had spread to his brain. One morning, his father took a picture of him smiling under a tree, wearing a Mickey Mouse sweatshirt. Later that day, a seizure left half his body paralyzed.

Adam died in October, after helping his mother write a poem for his funeral.

When Trisha and her sister Katie were teenagers, they developed thyroid tumors; fortunately, they were benign. Then brother David developed the same thing. All three had surgery and went on with their lives.

Then, this past January, their 16-year-old brother Peter got what he called "a double dose of the stuff." Doctors found tumors in his thyroid and his lung. Both were benign. They found the same in David's lung.

The doctors think the two boys were able to fight off the PPB before it could turn cancerous.

"It would be neat to find out why that happened," said Hill, of Washington University.

Peter, too, was intrigued. At Hill's invitation, he spent this summer at Washington University studying the disease, even examining slides of his own tumor cells. "That was really cool," said Peter, a senior at Mankato West High School.

Today, the Schoettlers say they worry about what might come next, but try to keep a healthy perspective. "You can drive yourself nuts worrying," said Chuck Schoettler. He and Julie have a 4-year-old grandson who is already having chest X-rays to check for signs of trouble.

Chuck Schoettler said he's been told the errant gene probably came from his mother's side of the family. But, he adds, "That's one thing you don't (control), what your ancestors had."

They hope the next generation will be luckier. But Trisha, who doesn't have children yet, is philosophical. "What we have is rare and it's sometimes scary," she said. "But at the same time this is who we are. And this is what has made us who we are."

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WHAT IS PPB?

PPB (pleuropulmonary blastoma) is the name of a rare kind of lung tumor. Only about 175 cases in the world have been reported, most of them in children younger than 5. Dr. Jack Priest keeps track of all reported cases at the International PPB registry at Children's Hospital in St. Paul.

About a quarter of all cases are believed to be inherited. For more information, go to www.ppbregistry.org.

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©2006 Star Tribune (Minneapolis)

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