Second in a two-part series
Jenni and Angie Thompson, widows before their time, embrace in the Murray City Cemetery, where their star-crossed family has gathered, living and dead. Stepping back, Angie kneels in the grass and brushes away sticks and leaves that have fallen on the tombstones, then stares at them in silence.
RICKY DAVID THOMPSON 1960-2010.
MEAGAN THOMPSON ROBBINS 1982-2005.
ALEX JESSIE THOMPSON 1994-2003.
In a seven-year span, she lost her husband and both of their biological children to cancer (they have two adopted children and one from a previous marriage), all in November, two on the same day of the year, Ricky dying seven years after Alex to the day.
“I think about it all the time,” Angie says. “I think, what happened to my life? It has ravaged this family.”
Jenni stands by her side. At her feet, there is another tombstone.
BRYCE DEE THOMPSON 1974-2014.
Jenni and Angie are connected by pain and loss. The extended Thompson family is missing one functioning P53 gene, whose job is to suppress cancer. This is known as Li-Fraumeni syndrome — and those with LFS have an 80 percent to 90 percent risk of cancer.
When Angie took Meagan to Tijuana, Mexico, for alternative treatment, she asked Jenni to come along for support. When she took Alex to Houston for similar treatment, she stayed with Jenni’s brother. And when Angie and Meagan were invited to appear on "The Sharon Osbourne Show" with singer Jewel to discuss LFS, Angie invited Jenni for backstage support.
“Angie and I have gone through this together,” Jenni says. “We understand each other. This situation has been very challenging. It’s been a long, exhausting journey.”
In 2001, when both of his daughters had cancer, Ricky knew there would be more deaths in the family. He had done his own research online and seen the short life expectancy for people with LFS. He bought a dozen burial plots in a corner of the cemetery for extended family to use when the time came. Now he and his daughters occupy three of them. Randy, the oldest of the siblings, explains that the cemetery allows double stacking, which provides more room for the family.
“There are a lot of kids in our family who have this (LFS) and don’t have enough money to buy (burial plots),” says Randy. “That’s why Ricky did it.”
Says Angie: “He offered it to the family. He told everyone to be prepared. And it was his way of taking care of me, of looking ahead. It keeps the family together.”
The Thompsons have been forced to take such practical, preemptive measures. Doctors have recommended that family members with the gene have annual full-body and brain MRIs, as well as blood tests and bone marrow biopsies. Given the high incidence of cancer, early detection is critical. Doctors also told them to avoid red meat, barbecued and microwaved foods, and X-rays, and encouraged them to cook with stainless steel pots and pans.
“We don’t know if this will help, but …,” they said.
The Thompsons also have had to face difficult ethical questions in their personal lives when it comes to preemptive strikes against the gene. Meagan had brain cancer when boyfriend Brian Robbins proposed to her. During their engagement she was diagnosed with breast cancer. They married anyway. She died about a year and a half later. Sharese’s daughter Lindsay, who, like her two siblings, has the mutant gene, underwent a double mastectomy in her early 20s even though she has never had cancer.
But the most difficult ethical question they face is whether to have more children and risk the 50/50 odds that parents with the mutant gene will pass it on to their children.
Jenni and Bryce had already produced one child, Kade, before they learned that Bryce had LFS. Jenni, who suffers from anxiety anyway, was distraught. Did Kade have the gene, too? She couldn’t force herself to get him tested for LFS because part of her just didn’t want to know. Three years later — after panicking every time her son complained of a headache or an ailment — she relented and had him tested. She was shaking and hyperventilating when she showed up for the test results, but a sympathetic nurse told her in the waiting room, “It’s going to be good news today.” Kade does not have the gene. By then, Jenni had already told Bryce, “We are not having any more kids until we do something.”
They paid $16,000 for an in-vitro procedure. Jenni’s eggs were fertilized in a lab with Bryce’s sperm and then 23 embryos were collected, frozen and shipped to a lab in Michigan to be tested. Of the 23 embryos, six had the mutant gene, four did not, and 13 were indeterminate. Three of the good eggs were implanted and nine months later Jenni gave birth to triplets, two of whom are identical twins.
Despite all the precautions, Jenni’s fears were not allayed. She worried that her babies might still have the mutated gene, but again she was so anxious about it that she put off the test — for 10 years. By then, Bryce had been diagnosed with leukemia. When the hospital called with Kade’s test results, Bryce was in the hospital receiving a bone marrow transplant while Jenni was at home, so paralyzed with fear that she didn’t answer the phone. “I was dry heaving into the toilet,” she says. Bryce called for the test results and then called Jenni at home to tell her the news — the triplets were clear. Jenni cried all the way to the hospital to see Bryce.
“We married at 20, and it’s been tragedy after tragedy after tragedy,” says Jenni, standing over Bryce’s grave. “Everything has been so hard since we married. Every year there’s a major tragedy going on — someone dies or someone has cancer. It keeps your priorities straight. You care about things that matter. I’m happy to learn that lesson, but not the way I had to learn it.”
At Bryce’s funeral, Jenni’s father, Jim Boud, urged the family to weed out the mutant gene, recommending in-vitro as a way to circumvent the suffering that otherwise awaited future generations. The grandchildren — Jenni’s nieces and nephews — have chosen to have children naturally anyway. Those with LFS have produced eight children; four of them have the gene, two do not and two are too young to be tested.
One grandson, Tony Means, discussed the in-vitro procedure with Jenni, but opted not to do it despite emotional pleas from Jenni and other aunts and uncles. He knew his late mother had had the mutant gene, but he didn’t know that he carried it himself when his wife, Erica, bore their first child. After a test revealed that Tony had the gene, he and Erica chose not to have the in-vitro procedure and had four children. Three of his five children have the gene and one of them, a 5-year-old girl, has a brain tumor.
On a Facebook page for an LFS support group, someone asked the group at large if anyone had chosen to have babies after a family member had been diagnosed with LFS. Tony responded: “After lots of thought and prayer, we chose to have four more children. Two of those four have LFS, as does my oldest son. It’s not easy, but we wouldn’t change our choice. Some people think we are stupid, crazy or even selfish, but this was our choice to make and we did so fully aware of the consequences and with our trust in God because it’s all in His hands anyway. You do what you feel is best and don’t look back.”
Jenni responded on the same page: “I did (in-vitro). I could have never lived with myself if I passed LFS on to my children knowing I could have prevented so much suffering for them. My husband had LFS and passed away in February. He also has lost several family members, and it’s been devastating. I wanted to end the chain of suffering for my children. … I could have never looked my kids in the face and told them that I knew there was a 50-50 chance they’d inherit this devastating gene and I knew I could have prevented it but didn’t.”
Tony, an earnest, happy, outgoing, 29-year-old LDS seminary teacher at Orem High, lost his mother Sharese to cancer when he was young. All three of her children have the gene. Before marrying Erica, Tony warned her that there was a good chance he had the LFS gene and that she was “marrying into this cancer curse.” She married him anyway and birthed their children, and then the realities of LFS began to show up.
Tony had a cancerous brain tumor five years ago. He underwent surgery to remove the tumor and when he regained consciousness his left side was paralyzed. After months of rehab, he regained most of his movement and learned to walk again, although he continues to have some paralysis in his left hand and is still weak. The cancer returned two years later, and he underwent six weeks of radiation treatment. He’s been cancer-free since then although he had to have a sarcoma removed from his hip recently. Five-year-old daughter Emma was diagnosed with a brain tumor three years ago, but so far the tumor has not grown.
“We realized this was a big decision (to have children),” Tony says. “A lot of people are very negative about the decision we made. But we’re all God’s children; He does what’s best for us. Nothing is guaranteed. Just because you don’t have the gene doesn’t mean you won’t get something. I let this be up to Heavenly Father.”
Some might find it inconsistent that he refused to take precautions via in-vitro and left it in God’s hands, and yet each year he undergoes full-body and brain MRIs, blood work and a colonoscopy to catch the cancer early. He had to “beg” his insurance company to pay for the scans, but the scans seem to have helped. Tony’s relatives all died within three years of their cancer diagnosis; he has made it five years, although he had the cancer recurrence a couple of years ago. His daughter’s tumor also was detected early.
“If you gave me the option to take this all away and the lessons I’ve learned from it, I’d tell you no,” says Tony. “Yes, I am sad I had a tumor and Mom died and my daughter has a tumor. But I’ve grown so much. I’ve grown so much closer to my Savior. How can we go through the refining fire without these things? I experienced the love of my Heavenly Father when my mother died.”
Drake Everett, Tony’s cousin, shares similar sentiments when he talks about the toll of LFS on him and his family. His sister Reagan has a brain tumor and his mother Rebecca has had multiple tumors. Drake, a gaunt and thoughtful 22-year-old with unruly black hair, found out seven years ago he had the gene. He possesses a hard-won wisdom and world-weariness beyond his years.
“It’s hard,” he begins, standing among the family’s graves, his hands shoved into his pockets against the chill. “It’s always in the back of your head. Even when you’re happy, it’s there. Sometimes it’s hard to do the basics — to get up, to clean, to eat. It would be easy to do nothing. Why bother? Or you can think, I might not have much time, so I need to make my time count. I’ve been told all my life not to use this as an excuse to quit and sit around and do nothing.” He pauses for a moment before he continues. “It’s a humbling life. I wouldn’t trade the lessons I’ve learned from this.”
The Thompsons have found different ways to cope with the losses and their precarious place in the world. The grandmother, Wilda, carries obituaries and photos of deceased family members in her purse. It is almost all she can think of or talk about. “People turn the other way when they see me coming,” she says.
Conversation at family gatherings, complains one of the grandchildren, tends to focus on death and cancer. As Jenni puts it, “It’s hard to go to family Christmas parties when half of them are in the graveyard.”
Kade, who won a national age-group wrestling championship in his weight class last year, has not returned to the mat. His late father was his coach, and they trained and traveled the country to meets together. “It’s hard for him to see all his friends with their dads,” says Jenni.
Drake's sister Reagan, a 16-year-old Olympus High softball player, has a brain tumor but that hasn’t stopped her from starting the TitanTough21 Foundation to raise money for LFS research. She is nothing if not ambitious, hoping to raise $5 million. So far she has raised $2,000. Dabbing at tears, she says, “One of the last text messages we got from Bryce was, ‘We need to find a cure for Li-Fraumeni syndrome.’ ”
The rarity of LFS makes it difficult to get funding, but Dr. Joshua Schiffman, a Utah oncologist who works with LFS patients from around the country, believes it merits financial support because unraveling the mysteries of LFS and the mutant P53 gene would help more than those with LFS.
“If we could solve LFS and understand how to prevent cancer in LFS patients,” he says, “it certainly would have ramifications for the general population. Nearly every cancer involves the P53 gene.”
The rarity of LFS means it also gets little respect from insurance companies. Some of the Thompsons have gone years without having a body or brain MRI because their insurance will not pay for the scans (cost: $2,000-$4,000 each), even though it would save money in the long run (Jenni says her husband’s eight-month bout with leukemia cost close to $2 million).
“(MRS scans) significantly increase the survival rate,” says Schiffman. “We’re fighting to make this the accepted standard of care. I spend a lot of my day on the phone explaining this to insurance companies. LFS is so rare, they’ve never even heard of it.”
Acquaintances frequently ask Jenni if she would marry into the Thompson family again now that she knows about the gene mutation. She says she would discourage her children from marrying into such a situation, but, yes, she would do it all over again.
“If I hadn’t, I wouldn’t have had 17 years with Bryce and I wouldn't have the same kids and all the people from his family that I love,” she says. “But this life hasn’t been for the faint of heart.”
EMAIL: drob@deseretnews.com