The battle against neuroblastoma, the rare and deadly child-killing cancer

An Israeli woman fights to cure the rare and deadly cancer neuroblastoma, which killed her baby.

 ENDLESS HOSPITAL days: ‘Shir was always happy. That’s what kept us sane.’ (photo credit: Einat Dado Baralia)
ENDLESS HOSPITAL days: ‘Shir was always happy. That’s what kept us sane.’
(photo credit: Einat Dado Baralia)

Shir Dado Baralia was a healthy, beautiful baby boy who made his family so happy. When he was one year old, his parents and two older siblings went about their daily lives. Little did they know that before the age of three, Shir would be dead from cancer.

“This is something no child, no parent and no family should ever have to go through,” says Einat Dado Baralia, Shir’s mother. “I want to change the fate of kids with cancer.”

“This is something no child, no parent and no family should ever have to go through. I want to change the fate of kids with cancer.”

Einat Dado Baralia

Shir (whose name means “song” in Hebrew) was diagnosed with neuroblastoma, a rare kind of cancer that mostly strikes toddlers under the age of five. Around 50% of children who suffer from the disease die. 

“For Shir, we had tried every available option, and they just were not good enough,” says Baralia, who now runs the nonprofit neuroblastoma advocacy organization Shir for Life. “These are kids who have not even started their lives. I literally cannot sleep at night when I think about it.”

The story began in December 2020. “Shirkush,” as her son was affectionately called, started to have constant diarrhea. As the mother of three, this didn’t phase Baralia at first. But she did want to get some answers. So she started running between doctors to understand why the diarrhea would not stop.

 ON THE way to one of many surgical procedures – ‘no kid should have to go through that.’ (credit: Einat Dado Baralia) ON THE way to one of many surgical procedures – ‘no kid should have to go through that.’ (credit: Einat Dado Baralia)

“We eliminated allergies, virus, teeth, etc.,” Baralia recalls. Nothing was coming up on the tests. Still, “I definitely didn’t think the diagnosis would be cancer.”

After about a month, blood work showed that Shir was experiencing high LDH levels. This usually relates to some type of tissue damage from an injury, disease or infection. A well-known professor told the family not to worry and referred Shir for a non-urgent gastroscopy test. Meanwhile, his levels continued to rise.

It took three months and five doctors to connect Shir’s high LDH level with the diarrhea that led to the ultrasound that found Shir’s tumor: 11 x 5 cm. Solid. In his stomach. 

“It took the doctors three months to diagnose the source, and only after the fifth doctor saw Shir, did he connect the LDH level and diarrhea, and we were sent to a simple ultrasound. Revealing the worst thing ever – Shir had cancer.

“There was no need to explain to me how wrong it was and that it shouldn’t be there,” Baralia says. 

The action was swift: a combination of chemotherapy, antibodies and biological treatments, including surgery to remove the tumor. But nothing impacted Shir’s rapidly metastasizing cancer.

“After five months of treatments in Israel… we left with our entire family to New York” for treatment at Memorial Sloan Kettering, a hospital that sees hundreds of patients with neuroblastoma a year – many more than the up to 40 seen in Israel – and offers a number of clinical trials.

Baralia says that at first they thought these new alternative treatments were working, as they managed to reduce the masses by 50% after the first five treatments. But after the sixth treatment, which took place in October of 2021, the cancer was out of control again. The New York doctors told the family to go home. 

“We decided it was not yet the time to go back home,” Baralia tells the Magazine. “So we found ourselves in Philadelphia, where an amazing doctor offered a unique treatment…Once again, there was hope.”

The treatment at Children’s Hospital of Philadelphia was “very hard, unlike any other treatment Shir had before.” He was sedated and intubated for a few days, and recovery took longer; but the family was once again optimistic. Reports were showing improvement. But this hope was short-lived. Three weeks later, the cancer was once again growing and spreading at an uncontrollable rate. 

“I felt like I was holding the window with my hands over my shoulders,” Baralia says, “just trying to keep it from closing.

“I celebrated my 40th birthday in a way I don’t wish anyone to celebrate,” she continues, “forced to make a decision whether we go back to Israel, where Shir will die within weeks, or go to Philadelphia to get a long-shot trial treatment that might work or not, but also taking the risk of a potential side effect of growth of the lung lesions that could lead to death the very same day. We decided to take the chance.”

Although Shir did not suffer from the severe side effect, the treatment also had no impact at all. The family returned to Israel a week later, and Shir died three days after their arrival. He was in his parents’ bed and surrounded by love. 

“My oldest daughter was in second grade when Shir was diagnosed with cancer,” Baralia says. “I spent her first-grade year with Shir [while I was] on maternity leave, so she was really connected to Shir. The first thing she said when she heard Shir had cancer was ‘But people die from cancer.’ It was really hard.”

Shir’s older brother, Gil, now eight, used to wake up in the morning with stomach pains after his brother was brought to the hospital. “They were so connected,” Baralia says. “It’s hard to explain in words.”

Fifty percent of children diagnosed with neuroblastoma die 

Neuroblastoma is a hard-to-diagnose cancer. More than 50% of patients diagnosed with the disease are already at advanced stages, making it one of the most difficult cancers to treat. 

It is also rare. Although 12% of cancer deaths are a result of the disease, less than 10% of children suffer from this solid tumor cancer. More common in children are blood cancers, such as leukemia, which today has a more than 80% chance of survival

Because it is so rare, it often goes undiagnosed. And it is also not well funded. 

“I learned that there is a potential cure, which in some of the cases was even proven effective on animals, but the researchers cannot get financial support to complete their efforts,” Baralia says. 

Neuroblastoma starts when immature sympathetic system cells grow and divide uncontrollably, forming cancerous cells. It usually presents itself in the adrenal gland but can start anywhere – the neck, chest, stomach or pelvis. From there, it spreads to the lymph nodes, bone marrow, bones, liver and skin, ultimately attacking the lungs and brain.

In Israel, there are about 40 children diagnosed with neuroblastoma a year, according to Dr. Shifra Ash, a specialist in the field of hematology oncology at Rambam Health Care Campus. 

There are six departments in Israel that treat these patients – in central Israel, Jerusalem, Haifa and Beersheba. All the doctors work according to best practices agreed on by the SIOPEN Association, an organization that helps “facilitate clinical, translational and basic research for children and adolescents with neuroblastoma in European countries, as well as worldwide, in order to improve the outcome of these patients,” according to its website. Ash sits on its board.

One of the challenges for improving outcomes for patients with cancer is late diagnosis; for neuroblastoma specifically, the challenge is finding funding for research and new treatment options. 

“A general pediatrician will see one or two patients with cancer in his entire career,” Ash tells the Magazine. “So you cannot think that every patient that comes in with abdominal pain has cancer. You just don’t send every patient with abdominal pain to get an ultrasound.”

For patients who can be treated, Israel has top-level medicine – among the best in the Western world. However, when the treatments did not seem to be working for Shir, Baralia decided to seek help abroad at Memorial Sloan Kettering. She hoped that she could get involved in a Phase I or Phase II clinical trial that could change the trajectory. 

Of course, not everyone has that option.

“Not everyone speaks English,” Baralia notes. “Sometimes other kids in the family are at different stages of life and can’t leave school. It is expensive, and not everyone can come up with the money. Others are not even aware that the option exists.

“Moving abroad has a tremendous impact on the entire family,” she continues. “Enhancing availability [for these experimental trials] in Israel is very important.”

Shir for Life

That is part of why Baralia started Shir for Life. She says she wants to improve neuroblastoma practices through international collaborations, among other methods. Right now, Baralia is working on a program to send Israeli doctors to Children’s Hospital of Philadelphia for a few months to consider options for joint research. 

“Everything in life is money,” Baralia says. “But now these dollars have pictures of children attached to them.”

She is working on raising funds to increase awareness about the disease among parents and doctors, which could lead to earlier diagnosis and improved prognosis. She also wants parents to be aware of neuroblastoma so they may ask about it when something is wrong. 

Finally, she hopes to infuse funds into research.

“I want to identify the best and most promising studies that are lacking funding and support them,” she tells the Magazine

She describes neuroblastoma as an “orphan disease.”  

“While scientists make new discoveries and develop new solutions, they cannot raise the funds needed to produce medicine for children with this disease. Professionally, this is known as the ‘Valley of Death.’

“It seems inconceivable, but it is true,” Baralia continues. “Children’s lives don’t matter as much as money does. Therefore, critical funding for groundbreaking research is lacking.”

Ash says that the last major breakthrough in treatment for neuroblastoma was in 2010, when scientists realized that certain antibodies could improve treatment. The use of these antibodies today means that 50% of children with the high-risk disease die compared to two-thirds. 

She adds that first- and second-line treatments are included in the Israeli health basket and at this point are mainstream. But alternative treatments become a question of resources. Investing in research in Israel would make these treatments more available. 

“Death does not have to be the fate of these kids,” Baralia says. “For me, it won’t help anymore. But I want to save the other children.” 