In Real Life You Don’t Quit

At 18, Jordyn Bennett was on the borderline between childhood and adulthood when she was referred to pediatric epileptologist Gretchen Von Allmen, MD, and pediatric neurosurgeon Manish N. Shah, MD. The two physicians work together with other adult and pediatric epilepsy specialists at UTHealth Houston Neurosciences, where they manage patients over the entire lifespan for a seamless transition of care. Jordyn is among the many patients to benefit from a highly specialized multidisciplinary program where complex cases are the norm.

Her medical record begins at age one with a diagnosis of neurofibromatosis type 1, a condition characterized by changes in skin pigmentation and the growth of tumors along nerves in the skin, brain and other parts of the body. NF1 is also associated with scoliosis, short stature and learning disabilities.

A year and a half later, on Mother’s Day, Jordyn had a stroke and was diagnosed with moyamoya disease, a rare, progressive cerebrovascular disorder caused by blocked arteries at the base of the brain. In children, its first symptom is often stroke or recurrent transient ischemic attacks. Shortly after the stroke, she began having seizures and was started on antiepileptic drugs; she failed one after another.

In her early life Jordyn saw physicians in New Orleans, a 90-minute drive from her family’s home in Walker, Louisiana. “I’ve always done whatever I had to do to take good care of her,” says her mother, Wendi Bennett. “I’m so used to dealing with rare diseases that I hardly know what to do for normal childhood illnesses.”

At the age of four, Jordyn underwent surgery for moyamoya disease at Tulane Medical Center, where neurosurgeons split a temporal artery and sutured it to her brain to create blood flow. The revascularization surgery was successful, and like the majority of children who undergo the procedure, she had no further strokes or problems related to moyamoya.

When she was seven, she developed plexiform neurofibroma, an uncommon variant of neurofibroma associated with multiple nerve bundles. The tumors grew on her spine, causing scoliosis, and she underwent realignment surgery. Meanwhile, she continued having seizures weekly and sometimes daily.

In February 2016, she developed a malignant peripheral nerve sheath tumor in her abdomen and was referred to Houston to John Slopis, MD, medical director of the Neurofibromatosis Program at The University of Texas MD Anderson Cancer Center. The tumor was surrounded by a benign neurofibroma, and no additional
treatment was needed.

It was Dr. Slopis who referred the Bennetts to Dr. Gretchen Von Allmen, chief of pediatric epilepsy for the Texas Comprehensive Epilepsy Program. She scheduled Jordyn for a Phase 1 epilepsy study using video electroencephalography to assess her condition and determine the best treatment.

“After failing trials of two or more antiepileptic drugs at maximum tolerated doses, a child is considered to have medically intractable epilepsy,” says Dr. Von Allmen, an associate professor in the department of Pediatrics at McGovern Medical School at UTHealth.

“Patients are admitted to the Epilepsy Monitoring Unit for three days to a week – rarely more than that – to determine where the seizures are originating and whether surgery is an option. In Jordyn’s case, within three days we were able to capture the seizure she was having multiple times a week. If all the seizures look similar on video EEG, it’s often an indication that one area in the brain is the primary focus.”

During their stay in Houston, they also met Dr. Manish N. Shah, who directs the Pediatric Epilepsy Surgery Program
and the Texas Comprehensive Spasticity
Center at UTHealth Houston.

“We hypothesized that the seizures could be originating in one of a few regions around her speech network, which is distributed around the brain,” says Dr. Shah, an associate professor in the department of Pediatric Surgery. “Normally we monitor the language network by implanting subdural grids, but we were concerned that open surgery would be impossible because of her previous surgery for moyamoya. Because of our closely integrated
team, we were able to schedule a cerebral angiogram immediately to determine blood flow from the temporal artery. We could see that little blood vessels had formed to supply the brain. A craniotomy would have interfered with that blood supply and put Jordyn at risk for stroke.”

Bennett confesses to “googling everything” and had prepared her daughter for the open surgery. “When I found out they couldn’t implant the subdural grids, I was upset,” she says. “I thought, ‘How am I going to tell my child she can’t have that surgery when she was so pumped to have it?’ She asked me what would happen now.”

At the multidisciplinary epilepsy surgery conference, her team of physicians and radiologists reviewed the results of her video EEG, MRI and angiogram and decided the only option was laser surgery.

“Jordyn’s condition – mesial temporal sclerosis – has the most common association with intractable temporal lobe epilepsy,” Dr. Shah says. “In adults, this particular pathology responds well to laser ablation, but in children it’s not as effective so we usually recommend open temporal lobectomy. But at 18, she was
in the gray zone between childhood and adulthood, and no longer truly a child. We believed it would work.”

On May 30, 2017, Dr. Shah took Jordyn to the OR and performed a minimally invasive stereotactic laser ablation of the hippocampus in the temporal lobe. With his patient’s head immobilized in a frame, he inserted a laser fiber the size of a coffee stirrer through a burr hole in her skull.

“The frame allows for extraordinary precision in placing the laser fiber, and using the MR scanner and MR thermometry, we very slowly destroyed the tissue causing the seizures,” he says. “The actual treatment takes 10 to 15 minutes. We close with a single suture, and the patient stays overnight. Laser ablation is a useful tool in our armamentarium, and we have extensive experience with it.”

After the laser ablation surgery, Jordyn’s typical seizures stopped. Six months later she was admitted to the Epilepsy Monitoring Unit at Children’s Memorial Hermann Hospital for a 23-hour video EEG to confirm the procedure’s success. “There was no evidence of any new seizure activity, and she has no deficits or complications from the procedure,” Dr. Von Allmen says. “Before the availability of laser surgery, there would have been little we could have done for
her. Instead, with neuroendovascular, neurosurgery and epilepsy specialists all working together, we were able to give her the best possible result. Most patients who remain seizure free for one to two years after surgery can stop their medication.”

A senior in high school, Jordyn plans to graduate this year. “She’s a trooper,” her mother says. “Neurofibromatosis comes with learning disabilities, and she has some. She’s fighting to do her best. When people ask me why I don’t let her drop out of school and go for a GED, I say because in real life you don’t quit. Last year she missed 82 days and still passed, so I tell her, ‘You’ve come this far, and you’ve got to keep fighting.’ And that’s what she does.

“She’s tiny – at 19 she weighs 95 pounds – but if you meet her, you would never guess there’s anything wrong with her,” Bennett says. “I thank God every day for the doctors she’s had in her life. Dr. Shah and Dr. Von Allmen are terrific and know their stuff. I’m ecstatic for my daughter and pray to God that we keep going down the same path. I couldn’t be prouder of her.”

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At UTHealth Houston Neurosciences, we offer patients access to specialized neurological care at clinics across the greater Houston area. To ask us a question, schedule an appointment, or learn more about us, please call (713) 486-8000, or click below to send us a message. In the event of an emergency, call 911 or go to the nearest Emergency Room.