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Mischievous Jonah Borngrebe is one of a kind. Unfortunately, the tumor in his brain is also extremely rare. St. Jude researchers are helping the world’s scientific community better classify this tumor.
Kris Borngrebe fought back tears as she sat in a Nebraska hospital recovering from the whirlwind her family of six had just endured and unsure whether her 2-year-old son, Jonah, would survive another day. Her husband, Kurt, sat next to her, gently encouraging her to eat lunch, when he had a sudden realization that formed into a troubling question: “Did they really say brain cancer?”
Those surreal words echo through the young couple’s thoughts nine months later as they watch Jonah frolic on playground equipment at the Memphis Grizzlies House, an on-site housing facility at St. Jude Children’s Research Hospital. Jonah is the Borngrebes’ second youngest child—described by Kris as the agitator in the washing machine, who has a loving nature but makes a calm day exciting. He’s quirky, mischievous and a non-conformist.
Jonah is now 3 years old and a veteran of two operations, multiple rounds of chemotherapy and countless visits to St. Jude. Scientists at St. Jude have an intense interest in the remaining tumor cells, which weave through the left half of Jonah’s brain.
One morning in May of 2008, Kris had just finished putting away the family’s laundry when she discovered a normally healthy Jonah drooling as he sat on the floor next to his older brother, Aidan. Jonah shot Kris a glazed look as his head twitched back and forth.
Unable to locate the phone, Kris hoisted Jonah onto her shoulder and frantically ran to a neighbor’s house to call an ambulance. “It was like a bad after-school special,” Kris says. “I was screaming in my neighbor’s driveway, Jonah was convulsing in my arms, my two other little boys were watching from across the street, and the phone died while I was talking to 911.”
Within minutes, Kris and Jonah were in an ambulance speeding to the hospital, as Kris repeatedly called Kurt, who had turned off his cell phone while attending daily Mass.
At the hospital, results of a CT scan revealed a brain tumor. As Kurt entered the hospital room, Kris broke the news to him.
“Kurt and Jonah have a really special bond. Jonah is the only daddy’s boy we have of the four kids. It was hard telling my husband that Jonah had a tumor,” Kris says.
Jonah was transported to another hospital, where a neurosurgeon informed the Borngrebes that an MRI would be performed and that brain surgery was a possibility.
The couple contacted their priest, who administered the last rites to their son.
The news worsened as the MRI revealed the tumor was larger than first expected—the size of a softball instead of a golf ball. Nevertheless, Kurt and Kris remained optimistic as a strong group of family and church members gathered to offer support during the six-hour operation. Most of the tumor was removed; less than a week later, Jonah walked out of the hospital with his family.
“He was walking and talking and fully functional,” Kris says. “He was even potty trained, which he wasn’t before.”
Pathology results revealed what physicians assumed to be a type of benign tumor. The Borngrebes believed they had dodged the worst, but a month later a postoperative MRI revealed that the tumor was growing back.
Physicians, somewhat baffled by the cancer, offered the Borngrebes a chemotherapy protocol that was not tailored to Jonah’s disease. Troubled, the Borngrebes searched for other options.
Researchers and physicians at St. Jude learned of Jonah’s case when Jonah’s physician referred him to St. Jude oncologist Alberto Broniscer, MD, an expert in high-grade gliomas. Broniscer asked St. Jude Pathology Chair David Ellison, MD, PhD, to review the tumor slides.
Ellison thought the tumor had been misidentified. Under the microscope, it showed the pathological characteristics of the extremely rare malignant glioneuronal brain tumor. Even though physicians in Jonah’s home state refused to operate again, St. Jude neurosurgeon Frederick Boop, MD, believed a second operation was possible.
The World Health Organization (WHO) categorizes brain tumors based on a combination of their microscopic pathological characteristics, knowledge of their biological behavior and occasionally by their genetic abnormalities. Brain tumor classifications have been based around the presumed cell of origin— either nerve cells, which are called neurons; or glial cells, which provide structural and nutritional support for neurons.
When viewed under a microscope, a few tumors show characteristics that combine the features of neuronal and glial cells. Jonah’s tumor was one of these. Because it displayed higher grade features in some areas, Ellison classified it as a malignant glioneuronal tumor, grade IV.
Although the scientific literature acknowledges the existence of tumors like Jonah’s, the WHO has yet to recognize the malignant glioneuronal tumor as a distinct entity. Ellison and Broniscer are working together to assess tumors that fall into this broad category. The colleagues have identified a range of glioneuronal tumor types, which they are currently studying. As the scientists focus on the tumors’ molecular genetic characteristics, they hope to create more detailed classifications.
“With advances in understanding cancer cell biology, we can begin to find out how best to classify these unusual tumors, not just in terms of how they look down the microscope, but also in terms of the genetic abnormalities that are responsible for turning a normal cell into a cancer cell,” Ellison says.
Ellison would like to see new editions of the WHO classification incorporate more information on the molecular abnormalities of tumors to help pathologists diagnose unusual entities like malignant glioneuronal tumors, predict how they will behave and better understand how they will respond to therapy.
“This is the sort of situation where St. Jude really comes to the forefront,” Ellison says. “We’ve got the expertise here to do research and clinical studies on these unusual tumors. We are well positioned to solve the problems they present.”
Jonah’s physician in Nebraska helped the Borngrebes make the decision to travel to Memphis.
“Our oncologist in Omaha told us if we stayed there, Jonah would get mediocre chemo that might or might not save his life or do any good, but that at St. Jude, he’d get the full court press,” Kris says.
During this time of turmoil, Kris also faced two other life-altering situations. A week before leaving for St. Jude, she underwent a biopsy to determine whether a lump in her breast was cancerous. And on the day she left for Memphis, Kris discovered she was pregnant with the couple’s fifth child.
“We always wanted a big family, and No. 5 was in the plans, just not right then,” Kris says. “Jonah loves babies more than anything, and I thought, ‘I bet this is the reason we are having this baby right now—this baby is going to give Jonah the will to survive.’”
Kris learned the lump in her breast was benign on the day Jonah underwent his second brain surgery. St. Jude neurosurgeon Frederick Boop removed a sizable portion of the tumor during the operation, but due to the nature of the tumor, a section of inoperable tumor still remained.
The entire Borngrebe family traveled to Memphis for the first six weeks of Jonah’s treatment. Kris’ parents drove their motor home and stayed nearby so that they could help care for the children. Kurt visited for two days twice a month. The upheaval in her family began to wear on Kris. But other St. Jude parents and Kris’ extended family of faith helped her realize that Jonah was in the best place for treatment. Kurt’s mother also provided support by staying with Kris when possible.
“From that point on, I just saw everything through different eyes,” Kris says.
Because Jonah was younger than 3 when he arrived at St. Jude, physicians approached his treatment differently than they would have if he had been older. Older children typically receive both chemotherapy and radiation therapy, but radiation is not administered to younger patients because of the associated side effects.
“The protocol Jonah is being treated with uses a combination of conventional chemotherapy and newer agents of chemotherapy, which target his tumor specifically,” explains Lionel Chow, MD. He and Broniscer oversee Jonah’s therapy, which includes three phases: induction chemotherapy; consolidation chemotherapy; and anti-angiogenic therapy, which attacks the blood vessels that nourish the tumor.
Jonah’s induction chemotherapy consisted of four monthlong rounds of drugs designed to wipe out as much of the remaining tumor as possible. An MRI assessment after the first phase revealed that significant parts of the tumor had dissipated.
The second block of chemo-therapy was not as intense but was still administered in high doses. During this phase, Jonah returned home, visiting St. Jude once a month for treatment. A second MRI revealed the tumor had not grown back, which allowed Jonah to begin the third and current round of chemotherapy—a pill that targets the blood vessels that sustain the tumor.
“We want to prevent any of the single tumor cells or clumps of tumor cells that are left from being able to grow back into a solid mass,” Chow says.
Jonah’s current round of chemotherapy lasts six months and his check-ups can be performed in Nebraska. He will return to St. Jude every three months for MRI scans.
Kris admits that Jonah is not the same child he was before his experience with brain cancer. Once anti-social, he now concentrates on TV programs and asks questions about the programming. Before his treatment, Jonah would either fall asleep or jaunt off into another room to wreak havoc. He now loves spending time with his siblings and requests that Aidan hold his hand when Kris administers shots to boost his white blood cell count.
Jonah is also fascinated and excited about the arrival of the latest Borngrebe—asking his mother often if the baby is still in her stomach and if it will cry when it is delivered. Kris has agreed to let Jonah pray over the baby when it arrives because he was concerned it would be scared.
Kurt and Kris reflect on that first, crazy day of diagnosis and remember how hard the journey has been. But they take comfort in what they have—the small rituals of love that only a family can give.
“Hey, Jonah! Guess what?” they often ask their son.
“You love me,” he invariably responds.
“Yep, we do,” they say.
Reprinted from Promise Spring 2009
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