She blew kisses to her parents, Angelo and Christina, as day after day they took her from their Middle Village home to Cohen Children's Medical Center for tests and treatment and consultations, and oftentimes for specialized therapy in Philadelphia.
She blew kisses to each of the Cohen Children's nurses, who quickly transformed from practitioners to friends and playmates of the beautiful one-year-old girl with the huge eyes. And kisses to her pediatric oncologist, Dr. Jonathan Fish, who in an interview last week remained awestruck by his tiny patient’s unrelenting spirit.
“She was indomitable,” he said, some two weeks after Anastasia passed away to neuroblastoma, a cancer generally found in young children. “Even early in therapy, she came in and was quite sick and uncomfortable, but one of the first things she would do when she walked into a room was blow you a kiss.
“Later on she developed a limp, but she still just hobbled around, doing the same things she always did,” he continued. “None of her demeanor changed even as her physical status deteriorated. She was the ultimate in taking what was coming and making the best of it.”
During an interview at their home, Angelo’s arm freshly emblazoned with a tattoo of his daughter’s name, the parents discussed the span of the daughter and the illness, which claimed her life on July 20.
She loved anything having to do with Mickey Mouse, they said, especially the show Mickey Mouse Clubhouse. She was glued to her iPad and had an affinity for even the harder games. She doled out kisses and waves and hugs everywhere she went. She bestowed incredible joy to those she met, even in the face of terrible circumstance.
“It wasn’t all bad,” Angelo said of her eight months in treatment. “There were good times there.”
Anastasia was first diagnosed in November 2014, after Angelo and Christina brought her to the hospital when she appeared lethargic and started vomiting. Initially believed to be a stomach virus, after a second trip to the hospital doctors found abnormalities in Anastasia’s blood work, eventually diagnosing her with high-risk neuroblastoma just days short of her first birthday.
Generally occurring in children under five years old, neuroblastoma develops from excess embryonic cells, and presents as tumors throughout the body. About 800 children are diagnosed in the United States annually, half of who are high-risk cases, the most aggressive form with which Anastasia was diagnosed.
“By the time we found it, it had metastasized,” said Christina.
Anastasia soon began chemotherapy at Cohen Children's, undergoing a round of four-day treatments every three weeks, with two weeks between each round to recover, from December until February.
Chemotherapy was trying on her body, and for the first month her left eye remained closed as treatment attacked the cancer behind it. However she remained unrelentingly positive throughout, they said.
“She would throw up from the chemo then a second later keep drinking her bottle,” Christina said.
Anastasia seemed to respond well to treatment, regaining her appetite, appearing less lethargic and her left eye eventually re-opening, and after five rounds of chemotherapy, the Kostaris family traveled to the Children’s Hospital of Philadelphia in March to remove her main tumor. During a pre-surgery MRI, however, they received bad news.
“The spot near her clavicle lit up,” said Christina. “Every other [cancerous] spot was gone, but that one had gotten bigger. It was a shock to us, because she seemed to be getting better.”
Doctors said that the location of the tumor near her clavicle made operating impossible, and suggested instead a course of non-standard treatment called MIBG therapy.
A relatively new form of treatment, MIBG therapy combines the MIBG compound, which is absorbed by certain tissues, including neuroblastoma, with radioactive iodine to deliver targeted radiation treatment.
Because MIBG attaches to neuroblastoma cells, it was utilized for decades as a diagnostic tool, demonstrating on MRI scans where cancer was located on the body. By combining the compound with radioactive iodine, oncologists have been able to deliver high doses of radiation directly to cancerous spots of the body.
“In the last couple of decades, oncologists have thought maybe if we give this radioactive compound in very high doses it will deliver targeted radiation, because it gets preferentially taken up by the neuroblastoma,” said Dr. Fish. “Hopefully it kills the neuroblastoma while not causing that much toxicity to the rest of the body.”
The parents were heartened as they researched the new treatment, and Anastasia soon began undergoing the therapy.
“When it works, it works fantastically,” said Angelo. “It goes right to the cancer and melts it.”
Because MIBG therapy involves high doses of radiation, it can only be administered by trained technicians in a special room with extreme precautions, including lead-lined walls, and is currently only administered at 12 sites nationwide.
The Kostarises stayed in Philadelphia for the treatment, Christina swathed in a mask and special protective gown as she lied in the room with Anastasia for days as the radiation seeped from her small body.
Afterwards, the family returned to Middle Village, and some six weeks later, in mid-May, traveled back to Philadelphia for the results. In the weeks after the treatment Anastasia appeared to be feeling better, keeping down food and walking again, and the Kostarises were hopeful.
However when they arrived in Philadelphia, the scans came back showing that three new cancerous spots had developed.
“Christina asked if that effected her survival rate and they wouldn’t look us in the eye,” said Angelo.
Upon returning to Cohen Children's, the depth of the prognosis was made clear.
“We knew it was bad at that point, but when we got back to LIJ, Dr. Fish kind of confirmed it,” he said. “He basically told us we needed a miracle. That was the biggest shock to us because she was so good, she was walking better than ever at that point. We were not expecting that at all.”
Anastasia was put on another round of low-dose chemotherapy in May, with plans to return to Philadelphia in July.
In June, she saw her extended family for the first time since her diagnosis. Chemotherapy greatly weakens the body’s immune system, so she had been kept away from most of her family and friends since November.
However Christina and Angelo’s cousins and nieces and nephews descended on their home for a last time that summer day, and Anastasia able to play with family she hadn’t seen in months.
“She had a great time,” said Christina. “Angelo’s nieces and nephews got to see her, my cousin came over with her kids and she got to play with them. It was nice.”
She passed away on July 20.
In the weeks since Anastasia’s death, the Kostarises have focused their efforts on other families grappling with neuroblastoma. With Cohen Children's already at work trying to establish a facility on the premises to administer MIBG therapy, the family has set up a fundraising page to help bolster efforts.
“Christina told me a couple months ago, wouldn’t it be great if one day we could help raise money for that program, so families don’t have to go somewhere else, they can stay there,” said Angelo. “And the room they plan on using for the MIBG therapy was actually the room she passed away in.”
Dr. Fish said Cohen Children's is at work developing an MIBG program, which he characterized as an innovative new treatment that will only gain traction if more facilities invest in the infrastructure to provide it.
“For high-risk neuroblastoma we’ve kind of maxed out what we can do in terms of chemotherapy and radiation and surgery,” he said. “We have to look for alternative ways of improving survival, and high-dose MIBG is one of those ways.
“The problem is you need this vast infrastructure of a lead-lined room, and radiation-trained staff, to be able to deliver it,” he added. “There are not that many places across the country that can do that. But if we’re going to make this a standard treatment, obviously there has to be more programs capable of delivering high-dose MIBG.”
He said the Kostarises benevolence has come as no surprise to him.
“The fact that they’ve taken the worst possible thing that can happen to parents and are trying to turn it into a positive speaks volumes about who they are and what they’re about,” he said. “Kids are a reflection of their parents, and Christina and Angelo are model citizens and just incredible people and I think Anastasia reflected that.
“They couldn’t have done more for her than they did,” Dr. Fish said. “They were the model of how to care for a sick child.”
Donations for LIJ’s MIBG program can be made here.