— The choice was stark: Either allow a once-active young boy’s body to continue to deteriorate painfully, or let a surgeon try a risky brain procedure that could cure him, cause further damage — or kill him.
Sam Gladen was a normal boy who loved running around with his friends, riding his bike and playing sports, especially football. But in 2007, he started to limp. Soon his left foot turned inward, at almost a 90-degree angle to his leg.
As doctors tried to determine what was wrong, Sam had to resort to crutches to get around. The boy who took such joy in sports was reduced to being called “the cripple” by other kids in school, some of whom accused him of faking his condition.
Eventually, a name was put on Sam’s condition — dystonia — and medications and painful Botox injections were prescribed in an attempt to make things better. Finally, the young Texan was given hope for permanent relief — brain surgery. The catch was that the procedure could also kill him.
Sam went for the surgery.
‘It was scary’
“I knew I had to do it, but I didn’t want to,” 13-year-old Sam Gladen told TODAY’s Meredith Vieira Monday in New York. It had been five months since he underwent the surgery at Cook Children’s Medical Center in Fort Worth, and Sam looked as normal as a kid can be.
“I’m feeling good,” he told Vieira as his parents, Kent and Debbie Gladen, nodded in agreement. Sam still limps a little, but he’s getting better all the time, riding his bike again, playing touch football with his friends, being a normal kid.
When he first started to experience the symptoms of dystonia and no one knew what was wrong, Sam said, “It was scary. You didn’t know what was going on. You didn’t know what was going to happen to you. It was just scary.”
Because doctors at first couldn’t put a name on what was wrong, other kids decided he was faking his symptoms. “They thought I was just full of it,” he said.
The reason it took so long to diagnose him, Sam’s parents explained, is because dystonia is so rare. No cause has been identified for the neurological condition, in which the brain sends false signals to the muscles, causing them to contort and go into spasms.
The condition can affect any part of the body and can be progressive. Sam’s case started in his foot and was progressing to his knee when doctors finally identified it.
But knowing what it was didn’t cure it. Painful Botox injections paralyzed misfiring muscles, but only temporarily. Medications didn’t help.
Finally, Dr. Fernando Acosta Jr. at Cook Children’s Medical Center told Sam and his parents that there was a new surgical procedure that promised a cure. At the time, Acosta was the only doctor in the country who performed the procedure.
That was the good news. The scary news was that the surgery involved opening Sam’s skull while he was fully conscious, inserting wires deep into his brain, and connecting them to a battery-powered device implanted in his chest. The wires would fire signals that would correct the false signals his brain was sending out.
“The margin for error is nil,” Acosta told NBC News in a report taped at the time of the surgery last May.
Brain surgery while awake
That report shows Sam, tight-lipped and staring straight ahead, being wheeled into the operating room, where a metal ring was screwed into his skull before the surgery. A plastic housing was attached to the ring, and then a hole drilled into Sam’s head — all while he was fully awake.
As Acosta found the area of the brain that was misbehaving, Sam told the doctor when his symptoms felt better and the pain went away.
Vieira asked Sam how he got the courage to go through such a dangerous procedure.
“I sort of glazed over,” he replied. “I didn’t really pay attention to the doctors or anything. I just shut down. My eyes closed over and I stopped listening.”
Looking at his son, now able to run and play like other boys, Sam’s father said that approving the dangerous surgery on his son was not a hard call.