Except for the pokes and scans, Anya Bentham could be on vacation.
The 5-year-old girl from England twirls to show off her pretty pink dress. She does a headstand. She chases her baby brother, Noah, behind an exam table.
We told Anya this whole journey is a vacation. West Michigan for us has been a home from home.
All this between medical appointments in her battle against neuroblastoma, a rare form of childhood cancer.
“She’s just incredible,” says her mother, Naomi Bentham. “She has just had a fighting spirit all along.”
She puts her arm around Anya, and says, “You are our Princess Warrior, aren’t you?”
Anya smiles and leans against her.
The holiday feeling is no accident. In the past two years, as they have made regular visits to Spectrum Health Helen DeVos Children’s Hospital to take part in a research trial, Anya’s parents have embraced West Michigan. They make each trip a vacation, visiting sand dunes, beaches and resort towns―as well as Anya’s medical team.
It’s all part of their plan to help Anya enjoy a full childhood, to focus her sights on life, not cancer.
On a recent visit, Anya celebrated a new milestone: She rang a brass bell signaling the end of her treatment through the clinical trial led by Giselle Sholler, MD. For two years, she took a medication known as DFMO―short for difluoromethylornithine.
Completing the trial marks a new phase for the little girl known as the Princess Warrior to her supporters in the United Kingdom who follow her Facebook page. For the first time since she was a toddler, Anya lives medication-free.
Naomi Bentham says she feels hopeful about the future.
“Fingers crossed, she is going to grow up and be a strong woman,” she says. “Because she is a strong little girl now.”
Anya’s dad, Graeme Bentham, says he feels “cautiously optimistic.”
They have come through so much that he has learned to recognize the end of each phase as the beginning of another.
A cry for help
Anya has battled neuroblastoma, a cancer of the central nervous system, for most of her short life.
She was begging her dolly. She was saying, ‘Help me, baby. Help me, baby.’
The Benthams live in Chester, a town near Liverpool. When Anya was 16 months old, her parents noticed she walked with a limp. Doctors at first thought she had an infection in her hip bone, so she underwent surgery.
For the next two months, she endured fevers and pain, as doctors tried to discover the cause.
Watching their baby suffer tore at the Benthams’ hearts. One night at home, Naomi heard Anya–then just 1½ years old―cry out.
“She was begging her dolly. She was saying, ‘Help me, baby. Help me, baby,’” Naomi says. “She was writhing in agony. You could see she was in so much pain.”
Back at the children’s hospital the next day, Anya went through a series of scans. Ultimately, the oncology team spotted a small mass on her left adrenal gland by the kidney. The mass, later diagnosed as a neuroblastoma tumor, measured only 3 centimeters. But the disease had progressed to stage 4.
“We had specialist scans a few days later. The disease was all through her bone marrow,” Naomi says. “It was all throughout her body.”
Neuroblastoma, a cancer of the central nervous system, strikes about 700 children a year in the U.S. It accounts for about 6 percent of all cancers in children and about 15 percent of all childhood cancer deaths.
The study ran from June 2012 to February 2016 and involved 101 children at 25 hospitals and universities in the U.S. The children took a pill form of DFMO―short for difluoromethylornithine―for two years.
The research tests whether the drug, initially created to treat African sleeping sickness, can turn off a pathway in cancer stem cells and prevent the cancer from growing back.
Dr. Sholler reported the results at the Advances in Neuroblastoma Research conference in Queensland, Australia, in June 2016.
The study found an 88 percent “event-free survival rate” for participants. That is about 20 percent higher than the typical event-free survival rate for children two years into remission.
It also demonstrated a 99 percent overall survival rate at two years. One child of 101 children died. Typically, about 10 deaths would have occurred in that population.
“We’re incredibly encouraged by the results,” Dr. Sholler said.
She received approval from the U.S. Food and Drug Administration to run a confirmatory trial. A new phase 2 study testing DFMO in reducing relapse rates began in February. It will expand to 40 sites across the U.S. and Canada.
Thus began the standard series of treatments to defeat neuroblastoma. Little Anya endured chemotherapy, a stem cell transplant and radiation. She also took part in a research trial in the United Kingdom for immunotherapy.
After a year and a half of treatment, tests showed no evidence of the disease. Still, Anya’s parents worried the cancer could return.
“The relapse numbers haunted us,” Naomi says.
About 50 percent of children with neuroblastoma suffer a relapse. For those who relapse, survival rates are reported at less than 10 percent, Dr. Sholler says.
Searching for anything to improve their daughter’s survival odds, the Benthams read medical journals and connected with other parents through a Facebook group. When they learned about Dr. Sholler’s research with DFMO, Graeme took a flight to Grand Rapids, Michigan, to check it out. He attended the 2014 symposium of the Neuroblastoma and Medulloblastoma Translational Research Consortium, a group led by Dr. Sholler.
They struggled with the decision to bring Anya to the U.S. for treatment. Their biggest concern, says Graeme: “Was it going to cause any more heartache and pain to Anya?”
They appreciated the fact that DFMO has minimal side effects. It caused thinning of Anya’s hair, but did not affect her energy level or appetite.
She also could take the medication in pill form at home, rather than in an intravenous line in the hospital.
They decided to enroll Anya in the trial. Anya arrived at Helen DeVos Children’s Hospital for her first visit in July 2014 at age 3. She made seven trips over the next two years.
In that time, she grew into a busy 5-year-old girl who loves pretty dresses and playing outdoors.
“She’s done her first year in school. She can keep up with her friends. She’s strong,” Naomi says. She adds with a laugh: “She’s a diva at times, but she has a right to be.”
The treatment “allowed her to have a life—a full child’s life. It’s not intruded on her life in any way,” Graeme says.
The last pill
In her recent checkup, Anya greets Dr. Sholler shyly at first, then warms up with smiles and hugs.
It’s been a month since she took her last DFMO pill. Her scans look clear, Dr. Sholler says. She wants to see Anya back in six months for a checkup.
The Benthams talk about their plans. That afternoon, they will pack up their rental car and head north to visit Alanson and Traverse City. They will take a ferry to Wisconsin.
“You’ve seen more of Michigan than I have,” Dr. Sholler says.
“We told Anya this whole journey is a vacation,” Graeme says. “West Michigan for us has been a home from home. We are as close to West Michigan adoption as we possibly can be.
“It is part of our life now. We have built special memories.”