Now Seizure Free, Gracin Gets Her Words Back

Gracin Hahne was 3.5 months old when she had her first seizure. “I was changing her diaper,” says Heidi Hahne, Gracin’s mom. “I also noticed something else: there were light patches, like abnormal pigmentations, on her skin.”

Gracin’s seizures were caused by benign tumors, called “tubers,” that develop as part of a condition called tuberous sclerosis complex, or TSC. Seizures and the “ash-leaf” spots on Gracin’s skin are just some of many of symptoms TSC patients may have to varying degrees. Gracin’s neurologist, Candida Brown, MD, started her on a course of anti-seizure medication right away, which stopped Gracin’s infantile spasms.

When she was 1 year old, Gracin’s seizures began breaking through the medication. Adding to and changing her medications brought her seizures back under control for another year, but they broke through again when she was 2. When Gracin turned 3, the seizures came back in force and could not be controlled.

Her parents took her to a local hospital for an overnight electroencephalogram (EEG) study, which detected the frequency of Gracin’s seizures through small electrodes attached to her scalp. “They found that not only was she seizing during the day, but she was also having about 30 seizures while she slept at night,” says Heidi. During her daytime seizures, Gracin’s speech would regress so badly that her parents couldn’t understand her at all.

Heidi and her husband, David Hahne, met other local parents of children with TSC through the Tuberous Sclerosis Alliance. Thanks to the alliance, they learned about two doctors at Lucile Packard Children’s Hospital Stanford who might be able to help Gracin: Brenda Porter, MD, an associate professor of neurology at Stanford University School of Medicine and a specialist in TSC, and Gerald Grant, MD, an associate professor of neurosurgery and, by courtesy, neurology at Stanford University Medical Center and division chief of pediatric neurosurgery at Lucile Packard Children’s Hospital. They also learned that Packard Children’s was the only hospital in Northern California using the innovative ROSA technology to help children suffering from prolific seizure disorders.

Guided by the surgeon, ROSA is a computerized surgical assistant that allows the most precise, minimally invasive movement and placement of tiny electrodes in the brain during surgery. ROSA could help doctors detect seizures deep in Gracin’s brain without having to open her skull or even shave her head, as other traditional methods require.

“Historically, when we used to put depth electrodes in, we did it with something called a grid, which is a much more invasive procedure where you have to remove the top of the skull. It causes a lot more swelling and discomfort,” says Dr. Porter, “whereas the ROSA procedure is really well tolerated, especially in children.”

“For tuberous sclerosis, this is the ultimate treatment,” says Dr. Grant. The ROSA device is part of the Pediatric Center for Brain Engineering at Lucile Packard Children’s Hospital Stanford, which brings together researchers from the Stanford School of Engineering, the Stanford Neurosciences Institute and Stanford Medicine Radiology to focus on brain disorders and developing personalized treatments for those disorders.

The first of Gracin’s two procedures was in December 2016. Using the ROSA device, Dr. Grant made 13 tiny, 1-millimeter holes in Gracin’s skull. He then used ROSA to guide 13 0.8-millimeter electrodes through those openings and deep into Gracin’s brain, setting up a stereo EEG. “Each electrode has contacts along its entire length, so you can get a sampling going into the gray matter of the brain, then into the deep white matter, then into the tuber and then out of the tuber,” Dr. Grant explains.

“They secured those leads to her skull,” says Heidi, “and she sat in bed for seven days with a big wrap on her head and an iPad. She just stayed there like a champ.”

“With the surface EEG, we saw some seizures but not a lot,” says Dr. Porter. “But then, when we put the electrodes in, we saw hundreds.” The majority of Gracin’s seizures were coming from her left temporal lobe, where the brain normally stores language. But because Gracin was so young and her language was not well developed, it was difficult to locate language in her brain.

“The question came down to: was the seizure activity in her language center or near it?” says Dr. Porter. “And the stereo EEG let us answer that question without getting too invasive. We were able to determine that it was not in her language center but adjacent to it.”

In January 2017, less than a month after the ROSA procedure helped locate Gracin’s seizures, Dr. Grant and his team performed the surgery to remove the tubers in her brain. For this procedure, they removed a portion of Gracin’s skull and resected a portion of her left temporal lobe. Throughout the five-hour surgery, Dr. Grant paused periodically to have radiologists assess Gracin’s seizure activity using an EEG. As the surgery progressed, they saw continuous improvement.

The Hahnes were advised by Dr. Grant and Dr. Porter that Gracin’s recovery would take time. With a resection in the left hemisphere, she might not be able to move the right side of her body, walk or talk for at least a few days. “So, we managed our expectations,” says Heidi, “and understood the possibility that Gracin might not be able to recognize us or speak for who knows how long.”

But Gracin, who was recovering after surgery, had plans of her own. “After not seeing her for eight hours, we turn the corner, and there she is flailing and moving her arms and legs,” says Heidi. “She sees us and says, ‘Mommy, Daddy, I want juice!’ Not only was she okay, but she was speaking in complete sentences and using all of her body parts. We started crying — and gave her juice!”

Dr. Porter and Dr. Grant celebrated Gracin’s rapid recovery, too. “It’s so wonderful for us to see,” says Dr. Grant. “It’s one of the most gratifying things we do, taking out areas that are clearly causing more trouble than good. Now she’s talking like crazy. It’s amazing!”

“Since the surgery, she’s much better. She’s seizure free and continuing to progress,” says Heidi. Gracin still needs continuous care for other aspects of her TSC, including monitoring and treating tubers in other parts of her body, and her neurologist will watch for any new seizure activity as she grows. But today, 4-year-old Gracin loves singing, reading books and chatting with her 1-year-old brother, Lucas. “We’re celebrating each of these moments,” says Heidi. “That’s our goal.”