Nobody knows — not even doctors — what causes a rare brain cancer that kills children

Parents want to know why their three children have suffered from the same rare and deadly brain tumor in a relatively small geographic area of Ocean Springs.

An Ocean Springs pediatrician reported the third case of diffuse intrinsic pontine glioma, or DIPG, to the Mississippi State Department of Health, asking for confirmation that the number is high. State Epidemiologist Paul Byers said the number of cases, though elevated, is too statistically small to draw a conclusion.

The truth is, nobody knows what causes DIPG.

The latest child diagnosed with the inoperable and aggressive tumor that forms on the base of the brain stem is Sophia Myers, a first-grader at Oak Park Elementary at the time of her February diagnosis.

Two other children in the Ocean Springs community — Sophia Mohler, 8, and Jaxon Schoenberger, 6 — died from DIPG.

All three families lived within a 25-mile radius of one another at the time of their child’s diagnosis, and all three attended Ocean Springs schools.

They played on the same beaches and swam in the same South Mississippi waterways.

Sophia Myers’ parents, Josh and Angel Myers, have told doctors about the cluster of cases in the Ocean Springs area since their daughter’s diagnosis. The couple is divorced, but came together to care for Sophia during her illness.

“Some were intrigued by it,” Angel Myers said. “Some said, ‘Well, if you average it out all over the world, it is not of statistical significance.’ But you know, I feel like it is — as a lay person and a mother and a fairly intelligent person.”

When a cancer cluster is investigated, according to the National Cancer Institute, it would still be hard to determine what, if any, environmental factors contributed to it.

Myers is a prosecuting attorney in Jackson, George and Greene counties. She says she won’t give up the fight for answers until someone can explain to her what needs to be done to keep another child from suffering like her Sophia.

She plans to make her concerns known to politicians in Washington, D.C., hoping someone will investigate the number of DIPG and childhood cancers in the area. She also plans to push for more funding for research on DIPG and other childhood cancers. She has previously lobbied in Washington for the National District Attorney’s Association.

Doctors have spoken frankly to her about what it would mean to find a cure for DIPG.

“If you ask the experts, they believe if you find a cure to this cancer, this deadly cancer, you will have the doors open to curing all other cancers,” Angel Myers said.

‘We have never found a cause’

Mark Kieran, director of pediatric neuro-oncology at Dana Farber Cancer Institute in Boston, says doctors have studied similar DIPG clusters in children in other cities around the world but have found no common link.

“What we know about the cause is, we have never found a cause,” he said.

“So is there a chance that these cases are somehow related?” he asked. “Absolutely. It is still a possibility.”

However, Kieran pointed out that doctors have “seen cases where two or three kids within a relatively small area get DIPG, but when they went back and looked into it, we discovered that, in the past 15 years, there has not been a single (other) case.”

The same was true, he said, when doctors looked into the cases of two children in Canada from the same school who were diagnosed with DIPG within two months.

“Obviously, every parent at the school was terrified that something was going on. And again, when we went back in that part of Toronto, it turned out in that part of the city, they hadn’t had a single case in 20 years. It was just a fluke of two cases occurring together, and couple of years later there had not been a single other case.”

Doctors have also studied the number of DIPG cases in the United States versus countries without strict environmental controls to reduce pollution.

“The incidents of DIPG appears the same in the United States, in South America, in the Middle East, in Asia, in Europe, and it doesn’t matter if you grew up in a town or in a city or on a farm, it all seems the same,” Kieran said. “So, it’s not because you live too close to a power line and it’s not because you drink diet coke, or you use the cellphone.”

Genetic factor

In the last five years, Kieran said doctors have identified a common genetic mutation in those stricken with DIPG.

Doctors are learning about the mutation through biopsies of some patients who are still living, and through further study of the tumors donated by the parents of DIPG patients.

“We don’t know what causes the mutation, but at least we know the mutation and we are now developing therapy for those mutations,” he said.

He said some of the studies are “beginning to suggest the time the original mutation of DIPG gets started may actually happen in embryogenesis long before the baby is even born. In other words, you don’t have the tumor when you are born, but the original mutation got there and eventually that is what is going to cause you to have the (DIPG) tumor later in life.”

“So you go from a single fertilized egg and by the time you are born, there are billions and billions of cells so the cells have been dividing like crazy then every once in a while one of the cells makes a mistake and messes up.

“It’s a little bit like messing up a blueprint that comes back to haunt you because the cell that has that mutation in it, in this case in the pons, suddenly reactivates and comes out of hibernation at age 6 and now tells that cell that never should be dividing to start dividing again.”

Then, he said, comes the DIPG diagnosis.

Trying to find answers

After Sophia’s diagnosis, the Sun Herald reached out to Dr. Betty Herrington, the director of pediatric neuro-oncology at the University of Mississippi Medical Center in Jackson, for insight on the cluster of DIPG cases in the Ocean Springs area.

Herrington diagnosed Sophia Myers, but did not treat her in the months after her initial diagnosis.

Herrington told the Sun Herald she would have her assistant send a form for Sophia’s parents to sign to give her authorization to talk about Sophia’s case and the number of cases in the area of Ocean Springs.

Herrington asked the Sun Herald to also send information on the the other two children who suffered from DIPG in the Ocean Springs area.

On May 10, Josh and Angel Myers signed the authorization form and the Sun Herald sent it back to Herrington, along with information she had requested on the other two DIPG cases in the area.

Herrington later sent an email saying she was working on the answers. By late June, Herrington’s assistant sent word that the doctor was “not interested” in an interview at that time and offered apologies for any inconvenience it may have caused.

The Sun Herald also reached out to UMMC’s public relations department to find a doctor to talk about the DIPG cases in the area.

A doctor called and commended the Sun Herald for trying to take a closer look at the cases, but later said he did not know he was going to be quoted.

The doctor asked the Sun Herald to send anything that would be attributed to him in an email so the information could go through the hospital’s compliance department for approval. The Sun Herald declined.

Margaret Baker: 228-896-0538, @Margar45