At first, there were only small signs that something was amiss. A few days after Lucy Cadenas was born on May 1, 2019, her mom, Janey Sprouse-Cadenas, noticed that Lucy was having what she described as “baby twitches” or “startles.” Lucy would blink rapidly, her eyes would start to roll, and her arms and legs would spasm slightly.
“It happened so fast,” Sprouse-Cadenas said, recalling the episodes. “They were literally under five seconds.”
By the time Lucy was 2 weeks old, however, the startles had turned into full-blown seizures lasting more than 30 seconds. And they were happening with increasing frequency.
Alarmed, her parents brought her to the local emergency room in Colorado Springs, only to be discharged after several hours. Later that night, though, Lucy experienced another seizure that lasted for more than 30 minutes. This time she was admitted to the hospital for what ended up being nearly a two-week stay.
It was the start of a nearly five-year journey that would take Lucy from Colorado Springs to Cincinnati and ultimately lead her to UCLA Health.
A rare-disease diagnosis
After extensive testing, Lucy was diagnosed with hemimegalencephaly, a rare disorder in which one-half of the brain is abnormally large. In Lucy’s case, the left hemisphere of her brain, which affects the right side of the body, was much larger than it should have been and was triggering her seizures. The diagnosis also meant Lucy had epilepsy and cerebral palsy and was likely to be developmentally delayed.
Hemimegalencephaly is so rare that only a few of the local doctors had heard about it, let alone seen it, Sprouse-Cadenas recalled. After consulting with colleagues at other hospitals to determine the best next steps for 2-week-old Lucy, the local medical team decided to try an anti-seizure medication to see if it would help her. Although seizures triggered by hemimegalencephaly are often drug-resistant, Lucy seemed to respond well, and Sprouse-Cadenas was allowed to bring her home again.
Armed with more information about what they were up against, Sprouse-Cadenas focused on learning everything she could about Lucy’s condition and lining up support. By the time Lucy was 6 months old, she was receiving regular in-home and outpatient occupational, physical and feeding therapy.
Sprouse-Cadenas also brought Lucy to Cincinnati to be seen by a medical team with more experience treating hemimegalencephaly. They discussed Lucy’s options, up to and including a hemispherectomy, a major surgery in which the abnormal half of the brain is disconnected from the other half of the brain, or removed. In an effort to delay or perhaps forestall this, Lucy started taking a chemotherapy medication that was considered experimental for treating seizures but which seemed to help her.
“You could tell she was delayed,” Sprouse-Cadenas said, “but she was 100% seizure-free – or so we thought.”
Previously undetected but constant seizures
About a year passed before Lucy had another seizure, at which point a bump in her medications appeared to bring things under control. That brought another year of relative calm.
But by the spring of 2021, Lucy, now 2, was again having frequent seizures, prompting another hospitalization and additional testing, including another electroencephalogram (EEG) and another magnetic resonance imaging (MRI) scan of her brain.
“They found that she was continuously seizing,” Sprouse-Cadenas said. Even though the seizures weren’t outwardly obvious, her brain activity showed that they were happening nearly all the time.
Lucy began taking additional medications, which again seemed to help, and Sprouse-Cadenas continued to hope there was some way to avoid “having to cut out part of her brain.”
There was one alternative procedure that offered the possibility of a reprieve: a vasovagal nerve stimulator (VNS). As Lucy’s seizures continued to worsen in 2022, Sprouse-Cadenas continued to wrestle with which procedure Lucy should have.
Still hoping to avoid a hemispherectomy, Sprouse-Cadenas opted to have the VNS implanted in Lucy’s chest. The device, often described as a pacemaker for the brain, can be a way to help control epilepsy-induced seizures. It provides regular electrical stimulations that run along the vagus nerve into the brain and is used in addition to anti-seizure medications.
The goal was to lessen the severity of the seizures, Sprouse-Cadenas noted. “It was more palliative care – it likely wasn’t a cure, although I hoped it might be for her, given that her seizures were relatively infrequent.”
A near-fatal seizure, and a wrenching decision
Still, Lucy’s condition was worsening. A near-fatal seizure in April 2023 prompted Sprouse-Cadenas to bring Lucy to UCLA Health in September of that year for a consultation with Aria Fallah, MD, a neurosurgeon who specializes in epilepsy surgery and an associate professor of neurosurgery and pediatrics at the David Geffen School of Medicine at UCLA.
“Within our community (other parents of children with hemimegalencephaly Sprouse-Cardenas had connected with), Dr. Fallah is known as being the doctor,” she said.
During their visit to UCLA Health, “he made me feel very comfortable and validated,” she said.
In January 2024, Sprouse-Cadenas and her wife, Caitlyn Colvin, returned with Lucy to UCLA Mattel Children’s Hospital so Dr. Fallah could perform the hemispherectomy.
“I couldn’t imagine doing such an invasive surgery without trying every other option first,” Sprouse-Cadenas said. “I feel like I made the right choice going with UCLA because of how complex the surgery was.”
Still, she recalled the lead-up to the surgery as one of the hardest times of her life. “I didn’t know if she was going to wake up as Lucy,” she said. “I didn’t know if she was ever going to wake up again. But Dr. Fallah promised he’d take care of her.”
A marathon surgery
A previous approach to a hemispherectomy entailed removal of the entire affected half of the brain, but the procedure now generally involves removing a smaller portion and disconnecting the rest of the affected hemisphere from the other side of the brain.
Called a functional hemispherectomy, the procedure is considered less risky than an anatomic hemispherectomy (full removal of the diseased half): Because less of the brain is being removed, there’s a decreased risk for hydrocephalus, a dangerous condition resulting from the buildup of excess cerebrospinal fluid, and there’s also less blood loss. This was a critical consideration for Lucy, who has Von Wellebrand disease, a blood clotting disorder.
Lucy’s surgery took about 14 hours, but was a success. “I remember him saying that it was probably one of the more complicated ones he’s had to do just because of her anatomy being so different,” Sprouse-Cadenas said.
“A lot of neurosurgeons may go their entire careers without seeing a single child with hemimegalencephaly,” said Dr. Fallah. “At UCLA, we treat about four to five kids with hemimegalencephaly a year, which allows us to have expertise in a pretty rare, complex condition, and also a treatment that’s pretty complex.”
Another key strength at UCLA Health is the multidisciplinary approach, Dr. Fallah said. In addition to the neurosurgeon and the neurologist, “there’s an entire team in the background providing critical input,” he said. “This allows us to make the best recommendation for each individual patient.”
During Lucy’s procedure, Dr. Fallah removed the entire temporal lobe on the left side of her brain, then painstakingly disconnected the remaining left hemisphere from the healthy right side of her brain. “It’s a very complex operation,” he noted. “We’re working many hours under a highly magnified operating microscope. And when the brain anatomy is distorted, that makes the surgery even more complex.”
Now seizure-free
Lucy remained heavily sedated for the first few days afterward as the anesthesia slowly wore off. When she awoke, her entire right side was paralyzed and she had lost all sense of balance and couldn’t even sit up unassisted. It was initially pretty traumatic for Lucy, Colvin recalled.
Six days later, she was able to take her first steps. And two weeks after her hemispherectomy, Lucy was cleared to go home.
Since then, Lucy has continued to improve dramatically. “Being seizure-free is at the top in terms of positive results since the surgery,” Colvin said. “But we’ve been able to see a lot of neat aspects of Lucy’s development that she wasn’t able to do before because her brain was too busy trying to survive.”
Given the brain’s neuroplasticity, Lucy’s brain has actually been able to rewire to make up for the missing connections, Dr. Fallah said.
As he explained, “90% of the fibers from one half of the brain go to the other side of the body, but 10% of those fibers actually go to the same side. So, she still has connections from the right brain to the right body. Eventually, what happens is that the normal side of the brain will take over the vast majority of the function and will be able to control both sides of the body.”
Lucy continues to receive intensive outpatient therapy and rehabilitation through a network of providers Sprouse-Cadenas has assembled near their home in Colorado, but is well enough to attend kindergarten. She wears braces on her legs and right hand that provide stability and help her body mechanics, given that her muscle tone has been affected by her cerebral palsy. She also has nighttime splints for her right foot and leg and braces for both hands. (Her right hand, the one affected by her condition, is splinted to keep it from curling, while her left hand is sometimes splinted so that she’ll be forced to use her right hand.)
Her auditory and speech skills have also taken off, Sprouse-Cadenas said. Immediately after the surgery, Lucy was able to speak in sentences – something she hadn’t previously been able to do. Although Lucy’s speech is still delayed, Sprouse-Cadenas noted, “she’s now telling us knock-knock jokes.”
Lucy has also begun weaning off her medications, starting with the chemotherapy drug. After her next set of EEGs, the hope is to wean her off of a second medication, with a third EEG and hopefully a third medication removal taking place six months afterward.
Although hormonal changes may affect Lucy’s seizures once she hits puberty, “we’re hopeful that this procedure will keep that from becoming an issue,” Sprouse-Cadenas said.
Dr. Fallah describes Lucy’s overall progress as “fantastic.”
Sprouse-Cadenas, who calls Lucy “my little warrior,” agreed, although she noted that Lucy “still has such a long road ahead of her. She can be OK one day and the next day can be a hard day,” she said.
Even so, though Lucy “still has some deficits,” Sprouse-Cadenas said, “it’s still night and day from where I thought she’d be at this point in time in her life.”
