‘In our wildest dreams’
Of the roughly 3 million people in the United States who suffer from epilepsy, about a third don’t respond to anti-seizure medications.
Justin Shults is one of them.
Shults is a young-at-heart 32-year-old who goes by the nickname “Squeak.” He lives with his parents, Maggie and Jeff Underwood, at their West Olive, Michigan, home in the woods. Here, near a blueberry farm where Shults worked as a young teen, the family’s two horses graze among the trees.
Shults has struggled with seizures virtually his whole life. The first ones hit at age 4, maybe younger. But in those early years, the stealthy seizures went undetected.
It wasn’t until he turned 15, the proud owner of a driver’s permit, that he had his first obvious seizure.
“We were all sitting down for a meal one day at a restaurant, and that was the first time he had a (noticeable) seizure,” Jeff said. “He just stopped moving—blank stare—and started drooling down the side of his face. That’s when it just clicked for us: He’s having a seizure.”
Things started making sense then, as the family looked back.
Seizures would explain Shults’ short-term memory problems and the many mishaps he’d had while riding his bicycle. Up until then, his troubles were simply explained as developmental challenges.
Doctors diagnosed Shults with epilepsy at age 15 and he started taking the first of many antiepileptic medications. Often a drug or a drug combination would work for a time—maybe a few months—but then the seizures would return.
The disorder soon forced him to make big changes.
First he had to hang up his car keys. Then he had to give up wrestling for West Ottawa High School.
It gradually became clear his epilepsy is what doctors call medically refractory: unresponsive to drugs.
Over time the seizures grew more severe. In his early 20s he started having “drop attack” seizures, which would land him on the floor without warning—all 250 pounds of him.
When he fell, alarming things would happen. He might break furniture, or dent the side of the refrigerator, or land facedown on the gravel driveway. It was dangerous for him and for those around him.
To his great frustration, he wasn’t allowed to carry young children.
“My nephew and niece, when I used to have seizures … I could never, like, stand up and pick them up,” he said recently. “I always had to sit on the floor, and I just couldn’t understand why.”
Not only that, but emerging from a seizure would trigger a flight reflex—he felt the need to get away. This could happen anywhere, his parents said.
“We were going 70 mph down the highway one day and he (had a seizure and) tried to jump out of the car,” Maggie said.
It’s been a long, long, long road. …We got our life back. All three of us.
Things got so bad that in 2009 his neurologist referred him to specialists nearly 200 miles away from home. A MRI scan detected a hypothalamic hamartoma at the center of his brain—a benign tumor that likely caused his seizures.
Although his parents were initially hopeful, they soon learned that options were limited at the time. The one thing the doctors did was implant a vagus nerve stimulator in Justin’s chest—a device that sends signals to the brain to help control seizures.
“But they basically said that the tumor … was inoperable,” Jeff recalled. “This was as good as it was going to get.”
“We were silent the whole way home. It took the wind out of our sails,” Maggie said. “But then we came home, we prayed about it and we decided this is our life. We’re going to … take care of him the best we can.”
By the time Shults was 30, seizures had made a mess of his life.
His attacks became so frequent—often four or five times a day—they impaired his cognitive abilities. Tests showed his IQ had dropped 10 points in five years. At the same time, his behavior had become so volatile his parents could no longer take him out socially.
“His condition was regressing tremendously,” Jeff said.
Then fits of anger set in.
“He would stand there screaming at us, spitting mad. And then he started getting really violent,” Maggie said. His tumor pressed against the hypothalamus, which controls emotions. And the emotion it triggered was rage.
At that point, the family could no longer care for Shults at home. They placed him in a residential care facility—first one and then another. Neither provided the level of care he needed.
Meanwhile, Shults’ longtime neurologists exhausted their options.
After trying what seemed like every available medication and consulting with experts as far away as Germany, they referred the family to Brien Smith, MD, a neurophysiologist who specializes in epilepsy with Spectrum Health Medical Group.
Dr. Smith, chief of neurology, developed Spectrum Health’s Level 4 epilepsy center, the first top-level epilepsy center in West Michigan and the largest such program in the state.
Here the family found fresh hope.
“Dr. Smith was an absolute godsend,” Jeff said. “Within literally the first 10 minutes of the very first visit, he had diagnosed Justin to a T.”
According to Dr. Smith, epilepsy patients typically have one of two types of seizures: focal, coming from one part of the brain, or generalized, “where the whole brain short-circuits.”
Shults had attributes of both types, and Dr. Smith discovered through MRI and EEG testing that in addition to the hamartoma in the center of his brain he had another lesion on the side of his brain, which also could cause seizures.
So which was the source of the problem?
“Bottom line is, we didn’t know if it was coming from here or here,” Dr. Smith said, pointing to a sketch of the brain. After consulting with colleagues, he recommended a minimally invasive surgery using a new laser technology called Visualase.
Unlike traditional epilepsy surgery, which requires the removal of the skull to access the brain, Visualase requires only the drilling of a small hole. Guided by MRI images, the surgeon slips the Visualase catheter through the hole and feeds it to the site of the tumor. A laser at the tip of the catheter is used to burn, or thermally ablate, the deep-seated tumor.
In October 2014 Shults became the first patient to receive Visualase treatment at Spectrum Health.
“Instead of going right to (traditional surgery), which would have been much more complicated and risky, we knew there was a direct lesion there, and the thought was, let’s get rid of that lesion with Visualase and see what happens,” Dr. Smith said.
Although the Visualase surgery was the best option available, it involved some risk, so the decision to go ahead with it wasn’t automatic.
“Dr. Smith was very candid,” Jeff said. Among other risks, “he said the optic nerve is right in front of this tumor (and) there’s a possibility that we could damage that optic nerve and you could lose your eyesight.”
The family took some time to think about it and ultimately left the decision to Shults.
“We wanted to have it done,” Jeff said, “but it was his body, his life, so we wanted to make perfectly sure that he was OK with it. Initially he said no.”
Then one day he told his mom to call Dr. Smith. He was ready. He wanted the surgery.
Hayden Boyce, MD, a neurosurgeon with Spectrum Health Medical Group, performed the procedure with the help of intraoperative MRI technology.
“The iMRI is great because it gives us real-time visualization of the temperature changes of the lesion during the procedure,” Dr. Boyce said.
He positioned the laser in the middle of the 13-by-14-millimeter lesion and watched as the tissue burned, losing its functional connection to the rest of the brain.
“The procedure itself was hugely successful,” Dr. Boyce said. “I was expecting that it would probably decrease the frequency of his seizures, but I wasn’t expecting it to completely eliminate his seizures.”
But that’s what has happened.
Shults has been free of seizures for 18 months and counting, an outcome the family never imagined possible.
“We’d really lost pretty much all hope,” Jeff said. “We were hoping the surgery would reduce the seizures. … In our wildest dreams we would have never hoped they would go away.”
The chaos of the family’s former lifestyle is now giving way to a more predictable, stable life.
Shults is living at home again, and his emotions and behavior are back to normal. He’s also regaining cognitive abilities.
Last summer he got back on a bike for the first time in 15 years. In February, his parents left him on his own overnight for the first time. In March, he cooked his first meal: baked potatoes and Italian chicken.
This summer, he’ll take his first plane trip: a flight to Atlanta with his mom to visit an older brother and his family.
“It’s really exciting (thinking about) the next few years with Squeak, being able to do stuff that we never got to do before,” Maggie said.
When a brain has multiple seizures each day, it’s like a battery that’s being drained, Dr. Smith said.
“You burn out a battery, it can’t function,” he said. “And then it’s got to recharge. If you keep burning it out, it never recharges to its complete state.”
Now, as Shults’ brain has time to recharge, it’s making a recovery.
He even sees the changes himself.
“When I had seizures, I didn’t know how to actually communicate well with people,” Shults said.
He now engages in conversation easily and enjoys his new opportunities. Looking to the future, his parents are hopeful he can live semi-independently when they’re gone.
“At the very least, he’ll need a lot less care,” Jeff said. “I can’t impress on you how relieved we are and how grateful we are.”
“It’s been a long, long, long, long road,” Maggie said.
For Dr. Smith, cases like this make his work worthwhile.
“It makes you say, what we are doing really does work,” Dr. Smith said. “Even though it’s not going to work every time, it works.”
And although there’s no guarantee that Shults’ seizures are gone for good, his doctors have cause for optimism.
“I’m hoping to continue to slowly take away some of his medications, and just let him live his life,” Dr. Smith said.
“I think the whole conclusion is, we got our life back,” Maggie said. “All three of us.”