Sept. 14-- When Mya Simmons was born in October, 2018, she immediately began having seizures.
They would occur regularly over 12-hour periods, followed by exhaustion and sleep.
At the time, it seemed all there was to Mya's existence was a cycle of seizures and sleep, said her mother, Miriam Simmons.
But a transformed Mya will be the star of the show when the family celebrates her first birthday next month. The new Mya babbles, laughs constantly, gets upset from time to time and is full of personality. The seizures that consumed her life have stopped.
"She gets upset! Who would have ever known that could even happen?" Simmons said. "She is actually a person now."
In April, Mya underwent 17 hours of brain surgery-two separate procedures-at Wolfson Children's Hospital in Jacksonville, Fla. The goal was to correct the condition that caused her seizures as well as a skull abnormality that, if left untreated, could lead to a misshapen skull, impaired brain growth and headaches, visual impairment and nausea.
Pediatric neurosurgeons Alexandra Beier and Nathan Ranalli carried out a "revolutionary set of surgeries never before performed simultaneously" at Wolfson, according to the hospital. They could not even determine if the surgeries had been performed simultaneously anywhere.
"This is an example of an extremely rare clinical scenario involving a patient with complex neurosurgical diagnoses that we were fortunately able to successfully manage through a collaborative team-oriented approach," said Ranalli.
When Simmons' pregnancy was 30 weeks along, an ultrasound revealed a mass on Mya's brain that was diagnosed as hemimegalencephaly, a disorder that causes one half of the brain to be larger than the other and leads to seizures. Simmons, husband Alex and daughter Ava, now 3, lived in Tallahassee but planned for Mya's birth to be at UF Health in Gainesville for its pediatric expertise.
But Mya arrived early during Hurricane Matthew. She almost immediately started seizing and, Wolfson was best able to transport her so her destination changed to Jacksonville. She was admitted to Wolfson's Level 4 Neonatal Intensive Care Unit, where a scan revealed an additional problem: craniosynostosis, the premature fusion of one or more of the growth plates in the skull.
She did not weigh enough to survive surgery for either condition, so she was discharged for the time being. "With the length of surgery, blood loss and the general risks of anesthesia, the patient's size is very important," said Beier, who is on the UF College of Medicine-Jacksonville staff and neurosurgical director of Wolfson's Pediatric Epilepsy Program. "With Mya, we delayed surgery until she had gained enough weight to tolerate the surgery."
The seizures continued. When caused by hemimegalencephaly, seizures are unresponsive to medication, Beier said.
By April, Mya was ready for surgery. Her family, who had moved to Oakleaf in Clay County, watched her being wheeled to the operating room.
"Her little body didn't even take up half the bed," Simmons said. "She just looked so little."
Ranalli, who is also on the UF College of Medicine-Jacksonville staff and neurosurgical director of Wolfson's Craniofacial, Spasticity and Brachial Plexus Programs, did his part first. He removed the bone at the top of the skull where the sagittal suture, which runs through the center of the skull from front to back, had prematurely closed.
"Because our brains experience a tremendous increase in volume during the first year of life, doubling in size, and throughout childhood ... openness of these sutures is critical to permit normal growth and development of the brain and skull as we age," he said.
Then Beier performed a peri-insular hemispherotomy, disconnecting the left half of the brain from the right to end the seizures.
"There are several points of connection from one hemisphere to another," she said. "The goal is for the abnormal brain where seizures are arising from to be physically disconnected from the brain that is not having seizures. Therefore the abnormal brain may still have abnormal activity, but it can't affect the healthy brain because it's no longer connected."
Mya recovered well following surgery, remained hospitalized for a week and then returned home. She has been seizure-free since surgery but will be on anti-seizure medication for a year or two and has to wear a customized helmet 23 hours a day, seven days a week for the next year to reshape her skull.
Beier and Ranalli said they are optimistic she will not need additional surgery.
"Mya is doing great," Beier said. "The young brain has an amazing ability to re-route information. After disconnecting the left hemisphere from the right, she may favor her left side compared to her right and have mild right-sided weakness, but she should be able to compensate for this and run, jump and play like any other child. As important would be that she is seizure-free, which opens up so many opportunities for her, including driving."
Ranalli said the helmet is having the desired effect on her skull. The surgery and helmet, he said, are "meant to be the only intervention a patient should need ... We will follow her closely over time to ensure that she achieves the expected outcome."
"By addressing (the condition) ... in infancy, the expectation is that she will have an excellent prognosis long-term," he said.
THANKFUL AND CATCHING UP
Mya's parents are ecstatic about the results. Before the surgery, "she couldn't do anything," Miriam Simmons said. "Now she is able to ... have a life."
The goal now, she said, is to "maximize everything she seems capable of doing. ... Thank God she has capacity in a lot of areas."
Although Mya babbles, she does not yet speak. Her parents are looking forward to the day she talks to them.
"We're playing catch-up," she said. "She does gab. She has a lot to say ... I can't wait to hear what that is going to be."
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