While sitting on her adaptive potty chair one day in mid-January, 6-year-old Astraea Pierce suddenly started wailing—and her parents couldn’t understand why.
“Astraea never, ever cries, but she was crying as if her bones were broken,” recalls her mom, Harlei Pierce. Lately, Harlei had also been noticing changes in Astraea’s social behavior. “She seemed different. She wasn’t as smiley and cutesy anymore.”
Unable to calm their daughter, Harlei and her husband, Melvin, rushed her to the hospital near their home in Vermont, but the physicians could not find anything wrong with Astraea. The hospital didn’t have the resources to provide specialty children’s care, so the physicians recommended the Pierces take their daughter to the emergency department at Dartmouth Health Children’s, about an hour away in Lebanon, New Hampshire. Astraea was quickly admitted to the hospital as an inpatient.
The family was no stranger to Dartmouth Health Children’s. Four years earlier, pediatric neurologists had diagnosed 2-year-old Astraea with a rare mutation of the STXBP1 gene. Because of the mutation, Astraea cannot speak and has several physical disabilities, making it even more difficult for her parents and doctors to determine the source of the sudden severe pain she was now experiencing, as well as some strange bouts of muscular tension that had also begun.
An overnight EEG (electroencephalogram) revealed that the right side of Astraea’s brain was reacting more slowly than the left side, so her doctors scheduled an MRI the next evening. The imaging test was expected to take about an hour, but at about 9:00 pm, after two hours of waiting, Harlei had become extremely worried—especially when she saw Astraea’s neurologist, Deborah Rukin Gold, MD, section chief of pediatric neurology, walking toward her. Dr. Rukin Gold sat down beside her and told her the terrible news: Her daughter had a large brain tumor.
“My whole world started crashing down around me,” Harlei says. “Dr. Rukin Gold gave me a hug and let me cry on her shoulder. Then Dr. [Nadine] SantaCruz, the pediatric oncologist, came and wrapped me up in a hug. I was an utter mess, and they treated me like they would treat their own daughter.”
Everything became a whirlwind after Harlei heard the diagnosis. Astraea needed immediate surgery, so the very next morning, barely two weeks since that initial trip to the Pierces’ local hospital in Vermont, Dartmouth Health Children’s surgeons removed a baseball-size tumor from her brain. It turned out to be an extremely rare and aggressive childhood brain cancer called central nervous system neuroblastoma FOXR2-activated. Since then, Astraea has endured six weeks of radiation and is currently undergoing chemotherapy treatments.
“I have been blown away by the kindness of the PICU (pediatric intensive care unit) nurses on the inpatient unit; they are incredible,” Harlei says. “They are making connections with Astraea even though she’s nonverbal. Sometimes we’re at the hospital for weeks at a time, and it’s frustrating to be away from my family. But the nurses and oncologists make it feel like a home when we are there.”
Now 7 years old, Astraea is a Dartmouth Health Children’s Ambassador, a testimony to the high-quality—and lifesaving—specialty care that children like her receive at the hospital. Harlei also praised the compassionate support of the hospital’s chaplain, one of the medical social workers, the Child Life program for wraparound support services, and the physical and occupational therapists who have worked with Astraea.
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