Anna Protsiou was five in 2002 when she was diagnosed with neuroblastoma. She remembers pain and the fruit-scented anesthesia masks that led her to stop eating cherries. She remembers hospital arts and crafts projects. What she barely remembers is the pediatric oncologist who saved her life.
She was a young girl then who didn’t speak English, moving with her family from their native Greece to be treated for a year at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center. Now, after moving with her family to Canada in 2014, she’s a 20-year-old dance student at the School of Contemporary Dancers/ University of Winnipeg and a contortionist with a rubber-band body. She’s ready to claim her history as her own, ready to move beyond photographs of the doctor and memories recounted by her parents, ready to take charge of her own health care.
So Anna traveled to Boston to meet her physician, Dr. Lisa Diller, and learn about potential late effects of the high-dose chemotherapy, radiation and two stem cell transplants that eradicated her cancer after surgeons excised her tumor.
“I don’t know if anything is enough for what Dr. Diller did for me,” Anna says.
“Often it’s the parents who reach out. There is a special bond between the parents and the physician who worked closely together to get a young child through treatment,” Diller says. “To have a young adult reach out was very special.”
Sitting in the clinic exam room, waiting for the doctor, Anna is nervous, but once Diller arrives, the jitters vanish. The two laugh and hug. Diller, now chief medical officer of Dana-Farber/Boston Children’s, has brought a photograph of the two of them in 2003. “I’ve had this on my wall for years,” Diller says. Also on her wall is a photograph of Anna in toe shoes dancing, sent years later by Anna’s father.
Thus begins the reunion and survivor visit. Diller tells Anna about neuroblastoma, a cancer of young children that arises in the developing nervous system. “When I started practicing in 1988, Anna’s type of neuroblastoma was basically incurable,” Diller says. Its current overall cure rate is well over 50 percent, propelled by patients like Anna, whose parents enrolled them in clinical trials, but it remains lower than several other pediatric cancers.
Like two-thirds of new neuroblastoma patients, Anna had metastatic disease, which, at that time, was treated with chemotherapy and stem cell transplant. Dana-Farber/Boston Children’s clinicians had been studying adding a second stem cell transplant to their regimen. Patients, however, were suffering serious side effects of the total body radiation used in one of the transplants. Those who survived were growing very poorly, and a significant number developed secondary, radiation-induced cancers. Anna’s family arrived just as Diller and her colleagues opened a pilot clinical trial using chemotherapy instead of radiation for the second transplant.
“Your dad had a really hard time deciding what to do, to have you go on the trial or to have you get the radiation we knew had been successful,” Diller tells Anna. “He was scared not to do what we had done for years and scared about the side effects of radiation.”
Anna’s parents ultimately opted for the pilot trial. “I can’t imagine being a parent in this situation,” Anna says.
In 2005, the Children’s Oncology Group opened a randomized, multi-center trial, led by Diller, which tested the relative effectiveness one transplant compared to the two transplants from Anna’s pilot study. The three-year survival rate of patients who underwent two transplants was significantly better than that of patients who underwent a single transplant, and in 2016 the double transplant, boosted by a newly FDA-approved immunotherapy drug, became the new standard of care.
“You helped break ground,” Diller says. “You really helped a lot of kids who got neuroblastoma.”
So far, Anna has suffered few late effects of treatment, other than having trouble hearing high-pitched sounds and, perhaps, being shorter than she would have been without neuroblastoma. One chemotherapy agent could lead to cardiac problems, Diller tells her, so she should monitor her heart health. Another drug could affect fertility by causing early menopause. “I apologize for that,” Diller says.
Anna leaves Boston relieved to have met the doctor she forgot, glad to learn she helped subsequent patients, and less scared about her own future.
“Now that I’m more aware of what could happen, I’m less worried. Who am I going to trust to take care of me? I’m going to trust myself. Stand on my own two feet,” Anna says. “I needed to know what happened to me. I was really young so it seemed unreal. It’s a mixture of acknowledging what happened, dealing with it in the future and carrying it with me. Not just knowing a story my parents told me.”