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Through generations, family falls victim to a killer gene

It was 1967, and Jane Southerland was in a doctor's office in Terre Haute, Ind., waiting for him to give a blood transfusion to her 4-year-old son, Jeff, who had leukemia.

The boy, pale and weak, sat quietly next to her.

Two other sons, 6-year-old Steve and 2-year-old Michael, were running around in the waiting room playing cowboys and Indians. Michael, pretending he was shot, dutifully collapsed, but comfortably across his mother's lap.

"I was caressing his back, going up and down with my fingers as he lay there," she recalled. "I stopped. I felt a lump just above his waist and just along the spine. I froze. I was scared silly."

Jeff died two days after the transfusion. Two weeks later, a tumor was discovered on Michael's spine during exploratory surgery. He was given radiation treatments and seemed cured until his teens, when cancer showed up again. Then, in 1981, 14 years after his brother Jeff died, Michael lost his battle.

His mother recalls that she was holding Michael's hand tightly and that the last thing he said was, "I love you, Mommy."

The odds of losing two sons to cancer _ what could they be? A million to one? Ten-million to one? A hundred million? More?

But the ordeal was not over. Jane Southerland went on to lose her husband, Ray, to cancer seven weeks after Michael died. And her last surviving son, Steve, got bone cancer and had a leg amputated at 14. Now 36, after going to Oxford and becoming a lawyer, he has a fast-growing, inoperable brain tumor.

Ray's mother, sister and brother all had cancer. The brother died in 1942 at age 2. Everyone thought the cause was spinal meningitis, until a genealogist checked the death certificate. Like a curse, cancer had devastated members of the family for at least seven generations, back to 1840, when the paper trail of death certificates gives out.

Scientists have found that the Southerlands have a mutated gene that makes them highly vulnerable to cancer.

When studies on the gene began in the late 1960s, only a couple of dozen families in the United States were known to carry it.

"Now it is known that there are hundreds of families with the defective gene," said researcher Fred Liof the Dana-Farber Cancer Institute in Boston. Li's work led to discovery of the gene in the 1980s, and families with it are said to suffer the Li-Fraumeni syndrome.

The gene, known as P53, is one of several cancer-suppressing genes in the body. Everyone has two pairs of P53: one pair inherited from the father and one pair from the mother.

If the pair from only one parent is mutated, the children still shouldn't get cancer, Li said. Jane Southerland's P53 genes were okay, so theoretically her children's cancer-suppressing abilities should have been normal.

But something happens that makes the defective pair of P53 genes alter the good pair, said researcher William Blattner, who for many years directed studies of cancer genes at the National Cancer Institute in Bethesda, Md.

What makes the defective P53 alter the healthy P53? Coffee? Chemicals in food? That's what researchers are trying to find out.

Not all the Southerland line got cancer since 1840. There is a 50-50 chance that the defective P53 gene will be passed along to children. The Southerlands didn't even beat those odds.

Steve and his wife, Genny, who married two years ago, have no children.

There is nothing on the horizon that will save Steve, who has had four types of cancer. He gave up chemotherapy on the brain tumor in late February when doctors found it had grown 50 percent in just four weeks. He had to abandon radiation last year when his body had reached the safe limits of dosages. Surgery would almost certainly take away his conscious thought.

Steve has given up his law practice, and he and Genny have moved in with his mother and her husband, Bert McMillen, a former pilot with Eastern Airlines.

Memories washed over Jane as she watched her son smile and laugh, sitting there in his wheelchair in the sunlight that streamed into the living room through the patio doors of her Miami home.

There was the time in 1967 when doctors had told Ray and Jane that there was no hope for Jeff. The couple bundled up their three children in their old, gold Chevrolet Corvair _ "the one that leaked so much oil we had to carry a case of oil in the car" _ and headed for a Florida vacation at the Sea Shell motel in Clearwater.

A few months later, Jeff died. It was tough losing Jeff, who loved to wear a big cowboy hat and tagged along behind his dad everywhere.

Then things seemed to turn around.

"Mike was feeling better after the cobalt treatments in Cincinnati _ that's what they called radiation treatments back then," Jane said. "Ray had been attending college part time all those years and finally got his degree in criminology."

That was 1972. The Southerlands, sure the future would be better, bought their first new car, a black Pontiac Grand Prix hardtop, and headed for Florida.

Ray got a job with Dade County's Police Department at $18,000 a year, more than three times what he had made in Indiana. The Southerlands bought a house with a pool.

Happiness was short-lived.

In 1973, a year after moving to Miami, Steve felt some swelling in his left leg. It was bone cancer. The leg was amputated in March 1974.

Michael died of cancer June 10, 1981. Ray, who had multiple brain tumors, died six weeks later. He was so sick in the final weeks that he never knew cancer had claimed Michael.

In 1986, doctors found Steve had a tumor on the left side of his brain. It was benign. It returned in 1990, again benign. During the 1990 surgery, doctors found another tumor, this one on the right side of the brain.

The tumor had remained about the size of a pea but began growing in May last year. It is next to an area that controls thinking. Any attempt to remove it would probably leave Steve unable to speak or move, he said.

"You know, this hasn't been a bad life. We've met a lot of wonderful people, and we've always gone forward. Always forward," Steve said. "I'm dictating a journal about my life. I'll keep doing it as long as I'm lucid."