Digging for Answers: Seeking a Cure for Ewing Sarcoma

A St. Jude patient

Hannah loves to take things apart and dig things up—enticed by the 
mystery of what lies beneath the surface and by the sheer joy of discovery. 
Between treatments, Hannah channels her energies into crafts and other 
activities that demand creativity and attention to detail.

 

She may have left her spade in North Carolina, but Hannah Allison has brought a sense of vibrant curiosity along with her to St. Jude, where doctors and researchers are doing their own digging—seeking a cure for Ewing sarcoma.

Hannah Allison is always on the lookout for a treasure—scouring her gravel driveway for interesting rocks, meticulously excavating a hill near her home for a cache of metal that she can sell to make some spending money.

But recently, the 12-year-old put these ventures on hold to travel to St. Jude Children’s Research Hospital. There, doctors and researchers are searching for the ultimate trove for kids like Hannah—a cure for Ewing sarcoma, a cancer that grows in the bones or in the soft tissues.

Hannah is no stranger to hospitals and medical procedures. Four years ago, a horrific fire sent the little girl and two of her siblings to a burn unit for 55 days. Nearly 20 percent of Hannah’s body was burned, requiring excruciating treatments and skin grafts to her arm and back.

Finally, life returned to normal. Then, Hannah discovered a knot in her upper left leg and groin area.

Another challenge

“We thought it was a hernia,” Hannah says, crisply relating the events that brought her to St. Jude. “We went to another doctor, and he said it was a lymph node. I took some antibiotics, and then we went to a specialist. Then we went to another hospital, where I had an ultrasound and a CT scan. They found out it was cancer that day.”

When Hannah’s family learned she had cancer, they assumed it was confined to her pelvic area.

“But we found out there were more than 40 tumors in her lungs, as well,” recalls her mom, Melissa.

“As a mother, my heart seemed to leave my body. My mind went to mush; my world was upside down. Because of the fire, Hannah had already met tragedy head-on. My only thought was, ‘Why is something else happening to her?’”

Although Ewing sarcoma is the second most common type of bone cancer in children, it is still extremely rare. Between 200 and 250 children and young adults are diagnosed with Ewing sarcoma each year in the U.S. Because Hannah’s cancer had already spread to her lungs, there was a high likelihood that standard therapy—chemotherapy, surgery and radiation therapy—might not completely eradicate the tumor, or the cancer might return after treatment.

At their North Carolina hospital, Hannah’s oncologist offered the family some advice.

“We think she would benefit from going to St. Jude,” the physician said. “She meets all the criteria to be treated on a new research protocol they have there.”

“When the doctor said, ‘St. Jude,’ warmth went throughout my body,” Melissa recalls. “I felt that’s where God wanted her to be.”

In good hands

For years, Hannah’s grandmother has hung photos of St. Jude patients on her refrigerator—to remind her of the children who benefit from her monthly donations to the hospital. Last December, Hannah suggested that her grandmother hang those photos on the Christmas tree, where the entire family could see them. Ironically, a few weeks later, Hannah became a St. Jude patient herself.

“When we arrived at the hospital, I was amazed at how many children are fighting cancer,” Melissa says. “It overwhelmed me.”

Almost all of those children are treated on clinical trials, which are studies designed to determine the best way to prevent, diagnose or treat diseases. St. Jude has created more clinical trials for cancer than any other children’s hospital in the nation.

Hannah Allison and JoAnn Harper

Nurse Practitioner JoAnn Harper (at right), is part of an extensive team of experts who specializes in taking care of children with Ewing sarcoma. During Hannah Allison’s participation in the ESFT13 clinical trial, the two have forged a special bond. When Hannah recounts a whoopee-cushion prank she played on Harper during a routine clinic visit, the memory sends the feisty 12-year-old into gales of laughter.

Because cancer had already spread to Hannah’s lungs, she qualified to enroll in the ESFT13 clinical trial as a high-risk patient. Children in the high-risk arm of that study receive two cycles of experimental chemotherapy followed by standard Ewing sarcoma treatment. Afterward, patients receive additional chemotherapy to help prevent relapse.

“In this trial, we’re testing a novel set of drugs before standard treatment begins,” explains Fariba Navid, MD, of St. Jude Oncology, principal investigator for ESFT13. That drug combo incorporates three medications with cumbersome names: irinotecan, temozolomide and temsirolimus.

Several years ago, a St. Jude researcher found that adding temozolomide to irinotecan was highly effective against relapsed Ewing sarcoma. In ESFT13, Navid and her colleagues are testing to see how well newly diagnosed patients respond to that combination.

Another St. Jude faculty member conducted early studies with the drug temsirolimus.

“In the lab, research showed that if you add temsirolimus to chemotherapy it becomes more effective,” Navid says. “It seemed like a good fit to combine all three of those drugs.”

The final portion of the clinical trial also builds on a previous St. Jude study, which combines drugs that target the blood vessels to prevent them from feeding any tumor cells that may remain. The entire treatment lasts nearly a year.

“When I explained to Hannah that she would be going into the hospital to get her next treatment,” Navid says, “she wanted me to tell her exactly what the drugs were, how they were going to be given, how many days she was going to be there and exactly what was going to happen. It’s impressive that she’s so involved and interested in her treatment. She also keeps us laughing. She recently used a whoopee cushion to play a joke on her nurse practitioner. I love the fact that she’s taking this in stride and not feeling sorry for herself.”

Ewing expertise

St. Jude has a team of experts who specialize in taking care of children with Ewing sarcoma. A skilled surgical team is available to perform limb-salvage operations or other surgeries, when necessary. Clinicians have access to the latest imaging techniques—such as contrast-enhanced ultrasound and diffuse weighted imaging MRI—as well as radiation treatments that include intensity-modulated radiation therapy and proton beam therapy.

Soon, patients with Ewing sarcoma tumors will be able to receive proton therapy on the St. Jude campus. This treatment can be focused precisely and intensely on the tumor, sparing healthy tissues from radiation exposure. The facility, slated to open next year, will be the world’s only proton therapy center dedicated solely to the treatment of children.

Working with European researchers, St. Jude scientists have also sequenced and are analyzing the genetic features of more than 100 children with Ewing sarcoma to find possible new targets for treating the disease.

The best place

In Hannah’s North Carolina hometown, the sixth-grader is known for her curiosity, high energy and sense of adventure. She loves to take things apart and dig things up—enticed by the mystery of what lies beneath the surface.

“Sometimes I take a hammer and break rocks apart to see what’s inside,” she says.

Years ago, someone buried a junk car in a hill near Hannah’s home. Intrigued, she began to dig for the vehicle. The hole became deeper and deeper and deeper. Hannah was convinced that treasure was within her reach.

“My husband and I discussed filling in that hole, but we knew that if we did, she’d just take the dirt back out again,” her mom says.

When Hannah uncovered metal items, she took them to a junkyard and sold them. Friends and family members began bringing her lawn mowers, weed eaters, metal chairs. She carefully disassembled each item and took those pieces of metal to the scrap yard—thrilled with her newfound income.

At St. Jude, the little girl who used to search for rocks and dig for scrap metal now turns her curiosity toward the medical environment, pushing buttons, checking out equipment, asking questions. “Just like the investigating and the digging she did at home, she’s got to see how everything works at the hospital,” her mom says.

During a recent hospitalization, Hannah entertained herself by checking out every piece of equipment in the vicinity.

“Dr. Navid, I’ve got to show you how high this bed will go!” she said, when her oncologist stopped by for a visit.

“The nurse is going to come in here, and we’re going to get in trouble,” Navid answered with a laugh, as Hannah manipulated the bed’s controls.

Whether she is exploring the hospital’s nooks and crannies or pulling hijinks in the clinic, Hannah enlivens St. Jude with her humor, curiosity and honesty. Her mom is confident they chose the best place to bring Hannah for treatment.

“Everybody here has been wonderful, giving us all the information we need, answering all the questions we have,” she says. “Without the research and the care at St. Jude, where would our children be? What hope would there be?

“I don’t want my child to have cancer, but I’m thankful that she’s here,” Melissa adds. “We’re in awe of what St. Jude has done for us. I want to say, ‘Really? You’re doing all this for our child and our family?’ But they do it not just for Hannah, but for all of these kids.”

Learn more about the ESFT13 clinical trial.

Abridged from Promise, Summer 2014

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