PETAL, Miss. (WJTV) – Jacoby Bergeron’s condition was very rare. According to the Batten Disease Support and Research Association, he was the only reported child in Mississippi since 1998 to have Batten Disease.
Dixie Bergeron, Jacoby’s mother, said he was always perfect. But by the time he was four, she noticed strange behavior.
“By the time he got to first grade, he was forgetting everything that he learned,” she explained. “And I knew something was wrong.”
After years of tests and questioning doctors, Bergeron finally got her answers. On February 2, 2015, Jacoby was diagnosed with Batten. In March of that same year, he had his first seizure, which lasted 45 minutes. From then, he would go on to lose his ability to walk and talk. But fortunately, he still had his vision.
“I found myself tiptoeing down the hall to make sure he was still breathing in the mornings,” said Bergeron. “I would check on him throughout the night. I haven’t had a solid good night sleep in many years.”
Batten Disease attacks the nervous system. In Jacoby’s case, doctors found in both copies of the genes from his mother and father that there was a spelling error. The error effects proteins from functioning properly and breaking down waste.
“You could think of you’re building up this trash material in cells, and there’s no garbage collection to come by and get this. Well if you get enough build up of this waste product in the cell, the cell stops functioning and will die,” explained Dr. Marcus Lee, pediatric neurologist at UMMC.
There is no cure for Batten Disease. But in 2017, the Food and Drug Administration approved an enzyme replacement therapy. This would put the correct spelling of the gene into the patient, potentially slowing down the symptoms of Batten. But, this therapy costs millions of dollars and multiple treatments are needed.
“It’s very difficult to get drug companies on board to do this because the disease is so rare,and it’s going to cost so much money to get through the research and production of these,” said Dr. Lee. “There’s very little incentive for drug companies.”
In December 2019 and January 2020, the disease started to beat down on Jacoby. During his final weeks, Bergeron said her son could barely drink and started to run a high fever.
His little sister, Myracle, always told his mother when it’s time for him to pass, she didn’t want it to be at home. On January 17, 2020, Jacoby took his last visit to the hospital.
“The whole way there he wasn’t really talking. He kept saying home. And even with him, I didn’t want to lie. And so all I kept telling him is, soon you’ll be home,” said Bergeron.
The life expectancy for a child with Batten is 12-years-old. About two to four children out of every 100,000 will be diagnosed. Jacoby would have turned 18 in April.