First in a two-part series
Who knew the havoc that eventually would be unleashed when Keith Thompson and Wilda Beaves were bribed into their first date. Keith’s mother offered him $5 to take Wilda to a church dance in Logan. They courted for a couple of years and then married. He was 18 and she was 17.
He began a career as a general contractor and she began having babies — six of them — and for the next two decades nothing hinted at the tragedy that awaited them and the next generations.
All these years later, Wilda, now 74, stands under sad gray skies on a cool November day, with the graves of loved ones at her feet. Nine members of her family lie in a corner of the Murray City Cemetery, with extra plots already purchased and reserved for the others who carry a cursed gene deep in their chemistry. She outlived them all. How can this be, she wonders? She listens to talk radio or stares at the TV all day — anything to distract her and forget that her husband, four children and five grandchildren are gone, all of them struck down young. She refuses to listen to music because it invokes memories of her lost family.
“I’ve got more family over there than I’ve got here,” says Wilda. “It’s been a hard life.”
It is remarkable what microscopic items can do to a generation of otherwise healthy human beings — DNA and genomes and proteins and things so small that they fit inside a cell. One gene has gone awry in the Thompson family’s chemistry. The P53 gene is mutated (damaged) and unable to do its job, which is to patrol the body to find and stop cell mutations, which, left unchecked, grow and grow and never stop growing. This is cancer. The presence of a mutant P53 gene is known as Li-Fraumeni syndrome (LFS), named after the doctors who discovered it. Passed from generation to generation, the mutant gene is a death sentence. Lacking that healthy tumor-suppressor gene dramatically increases the risk of cancer to a staggering 80-90 percent.
Keith apparently was carrying the mutated P53 gene but never knew it, nor would his wife and children know it for years, until the grandkids came along and it was much too late.
So the dying began. Keith died of cancer in 1982. He was 42 years old. Life went on. His children — Randy, Ricky, Larry, Sharese, Rebecca and Bryce — started their own families.
In 1993, Larry Thompson Jr., Larry’s son who was known as L.J., died of brain cancer at the age of 2. Larry himself had cancer at the same time — his second outbreak of it. His first bout with the disease was at 15. As a teen, he tried to continue life as normally as possible. Even after surgeons removed one of his hips, leaving him 5-foot-8 on one leg and 6-foot on the other, he competed on Brighton High’s wrestling team. He never won a match, but he never was pinned, which had been his stated goal. The cancer returned years later when he was a father, and at the same time he had to watch his tiny son die of cancer.
In 1994, Keith and Wilda’s oldest daughter, Sharese Thompson Means, died of breast cancer at 29, leaving behind a husband and three children, all of the children carrying the mutant gene.
In April 2001, Alex Thompson, the daughter of Ricky, was diagnosed with cancer. She was 7. In April of 2002, her sister Meagan was diagnosed with a brain tumor the day before she was supposed to enter the Missionary Training Center to begin an LDS Church mission in Bolivia.
The incidence of cancer in the family by now was so uncanny that friends asked if they had ever lived near the infamous Nevada nuclear test site. Someone suggested they undergo tests. After the family made inquiries, doctors recommended a genetic test, immediately suspecting Li-Fraumeni syndrome.
Doctors gathered Wilda and her five surviving children around a large table at the Huntsman Cancer Institute and told them about LFS and its implications. Not only did they face a phenomenally high incidence of cancer, but half of them would contract it before their 30th birthdays, often with multiple cancers after that. They explained that they would test Larry first for the mutant P53 gene because he had already had two bouts of cancer and had lost a child to cancer. Six weeks later they called with the test results — Larry had the mutated gene.
By then, fate had intervened. Larry and his son Trevor were duck hunting on Scofield Reservoir when a storm rose up. Witnesses said the hunters turned the boat toward shore to escape the wind-whipped, 6-foot waves, but the wind caught the nose of the boat and lifted it up and up and up until it dumped all its occupants. Larry and Trevor drowned. They were 39 and 12, respectively.
Because Larry’s test had come back positive for LFS, each of the four surviving siblings underwent the test for the mutated P53 gene. The results indicated that Ricky, Bryce and Rebecca have the gene; Randy, the oldest, does not.
Larry cheated cancer, but the disease struck again with a vengeance. In 2003, Alex died at the age of 9. In 2005, Meagan, now married, died of cancer at the age of 22. In 2010, the girls’ father, Ricky, a contractor, teacher and high school wrestling coach, died of cancer at the age of 50. In 2014, Bryce, a businessman and father of four, died of cancer (leukemia) at the age of 39.
That leaves only two of Wilda’s six children alive — Rebecca and Randy (who doesn’t have the gene). Rebecca was actually the first of the children to get cancer. She was barely a year old when she was diagnosed with an adrenal sarcoma, which was removed by surgeons. She hasn’t had cancer since then, but she has had more than a dozen benign tumors removed.
“You live in fear every day that someone in our family is going to get cancer,” says Randy. “You live in fear that you’re going to get a phone call.”
Now the watch is on for succeeding generations. Rebecca’s daughter, Reagan, a softball player at Olympus High, was diagnosed with brain cancer last year at the age of 15. During a nine-hour surgical procedure, doctors were able to remove only one of two tumors — one was too diffuse and close to the brainstem to be excised. She also has had benign tumors.
Because there is a 50 percent chance that parents with LFS will pass on the mutant gene to their children, doctors recommended that the rest of the family undergo genetic tests. Out of Wilda Thompson’s 21 biological grandchildren (several others were adopted), 10 of them have LFS (another might have had it, but he died before he was tested), five of them have or have had cancer, and three of them have died of cancer.
“We joke about who’s next,” says Rebecca as she cries softly. “It’s kind of a sick joke.”
The Thompsons are a rarity. Dr. Joshua Schiffman, a pediatric oncologist at Primary Children's Hospital and the Huntsman Cancer Institute who works closely with the Thompsons, says he and his colleagues are still trying to determine exact numbers. At this point they estimate 500 families in the U.S. have LFS.
“These families say this is like a curse,” says Schiffman.
Through a Facebook page for LFS families, the Thompsons have been able to compare notes with others who share this curse. So far they haven’t found another family that has suffered as many cancers and deaths as they have.
The tally for the beleaguered Thompson family is staggering. Six of the eight members of Keith and Wilda’s family have had cancer, and five have died. As for Wilda’s 21 biological grandchildren, the aforementioned ratios are worse when you consider that four of the children were born to Randy, who does not have the gene, and three more were conceived via an in-vitro fertilization procedure that culled out embryos with the mutated gene. In other words, there are 14 biological grandchildren born to the five siblings with the gene and conceived naturally; 10 of them have the gene, three do not, and one is unknown. Five of those 14 have had cancer and three have died.
And the risk has been passed to still another generation. Wilda has eight great-grandchildren who have been born to a parent with LFS. Four of them have the mutant gene, two do not, and two are too young to be tested for the gene.
One of them has a brain tumor.
In all, that’s 12 people who have been diagnosed with cancer in four generations, so far. The average age of those who died of cancer: 27.
If all this weren’t enough, one grandchild drowned and an adopted grandchild was one of five little girls who climbed into the trunk of a car on a hot August day in 1998 and died. Wilda’s brother died in a plane crash when she was young, and Keith was an infant when his father died in a train wreck.
Cancer has been so prevalent that sometimes more than one family member has had cancer simultaneously. Sisters Meagan and Alex had brain tumors at the same time, but died two years apart. Larry and his young son LJ had cancer at the same time. Reagan and her Uncle Bryce were in the same hospital at the same time, comrades in their battle against a common enemy.
“We ate lunch together and texted each other and visited each other,” says Reagan. “That was really hard when he died.”
The loss of Bryce in February was in many ways the toughest for the family. He was only 39, the youngest of Wilda’s children and the father of four young children. He was an imposing figure at 6-foot-2, 240 pounds, with model good looks. He battled fearlessly in the face of immense pain. He endured an excruciating bone-marrow transplant procedure, and when the cancer returned in a matter of weeks and he was asked if he wanted to endure a second transplant, he said, “I’m willing to put my body through hell as many times as I have to to raise my kids.”
Says his widow, Jenni: “I can’t tell you how painful that was. He’d be crying in pain and grabbing the nurses’ hands and telling them thanks for all you do for me.”
Bryce’s 12-year-old son Kade slept on the floor by his bed, reaching up to hold his father’s hand. Randy, Jenni and her father, Jim Boud, took turns sleeping at the hospital with Bryce for eight months. They helped him to the bathroom, they held him while he vomited and cleaned him up and packed him in ice when his temperature soared to 106 degrees and once as high as 109.
“Yeah, I felt good that I didn’t have the gene, but bad that I have to watch all my family leave,” Randy says. “It’s tough. When you spend time taking care of them and they die, it’s especially difficult.”
Says Rebecca: “I felt guilty when Bryce died. Why take a healthy man with little kids? Mine are grown. My kids know me; his kids won’t know him. They lose a lot of years with their dad, just like he did. It’s pretty hard. It should’ve been me.”
Tomorrow, part 2: Li-Fraumeni syndrome forces the Thompson family and their loved ones to face huge ethical questions, such as whether to marry into the family or whether to have children and risk passing on the deadly mutant gene, as one grandson chose to do.
Email: drob@deseretnews.com