‘Let’s do this’

Medication failed to control an upbeat 6-year-old’s seizures. A rare brain surgery restored her health, allowing her to flourish.

‘Let’s do this’

Leanne Parker won’t soon forget the two most frightening moments of her life—the night she first witnessed an epileptic seizure in her daughter Lily, then age 4, and the day Lily underwent brain surgery, two years later, to make the seizures stop.

“I used to be somebody who had everything in control. And when epilepsy came into our life, I realized we had no control over that—and that was really, really hard,” Parker said.

“It was the scariest thing of my life.”

Throughout the turbulent months between Lily’s 2017 epilepsy diagnosis and her 2019 surgery to remove a portion of her brain, her mother learned the importance of trust and optimism.

And young Lily became her guide.

“With all of our visits and our stays at (Spectrum Health Helen DeVos Children’s Hospital), Lily loved every moment of it. And that brought so much peace to me as a mother,” Parker said.

“She always wanted to go back, so when I booked appointments, it was like, ‘Oh, yay!’”

And when surgery emerged as the best treatment option, Lily led the way.

“She was so positive. She talked about it like it really was no big deal,” Parker said.

“You know—‘I’m in good hands. This is going to be OK, because I trust my mom and I trust my doctors and I trust the team. And they’ve all been there taking care of me, so let’s do this.’”

Drug-resistant epilepsy

In the beginning, Lily’s seizures happened roughly once a month, usually at night.

Over time, they increased in frequency to every two weeks.

Each one left her “in a fog,” said Lily’s pediatric neurologist, Angel Hernandez, MD.

Dr. Hernandez tried a succession of anti-seizure medications with Lily, but each one failed to control her seizures. Her diagnosis changed to intractable, or drug-resistant, epilepsy.

“None of the meds worked and she started building up a tolerance to the rescue med that would stop the seizures,” Parker said. “I felt like I was running out of time.”

As the months passed, the risks to Lily’s long-term health increased. Unchecked seizure activity can spread to other parts of the brain and lead to developmental disabilities, Dr. Hernandez said.

To learn whether surgery could be a viable treatment option for Lily, the pediatric epilepsy team worked to identify the source of her seizures.

This meant repeated admissions to Helen DeVos Children’s Hospital for assessments and testing.

“We have to go through a process that typically requires admission to the hospital for a minimum of five days, during which we remove medications, provoke seizures and do imaging studies to help clarify where seizures are coming from,” Dr. Hernandez said.

The process proved stressful for Parker, who had a second daughter at home in Muskegon, Michigan, 40 miles away from the hospital in Grand Rapids.

But the Child Life Services staff brought smiles and fun—sometimes even therapy dogs—with every visit to Lily’s bedside.

And the epilepsy team brought diligence and clear communication.

“I really appreciated that Dr. Hernandez is a very straightforward person,” Parker said. “You want all the cards on the table, and he definitely gave that to us. I just think that he’s the best.”

MRI studies showed that Lily had an abnormality on her occipital lobe—the smallest of the brain’s four lobes, situated at the back of the head.

Dr. Hernandez identified this congenital malformation as the cause of Lily’s seizures. He believed removing this part of the brain could stop her seizure activity.

But surgery would come with a downside: Because the occipital lobe helps the brain process visual information, removing all or part of the lobe would damage Lily’s peripheral visual field.

With the clock ticking, the family met with Casey Madura, MD, a pediatric neurosurgeon, to learn more about the procedure.

Occipital lobectomy

Relying on the epilepsy team’s extensive testing data, Dr. Madura recommended moving forward with an occipital lobectomy, the surgical removal of the occipital lobe.

This procedure is standard but uncommon, Dr. Madura said, because epilepsies stemming from the occipital lobe are relatively rare.

Dr. Madura offered to perform the lobectomy, despite the visual impairment it would cause, because if it worked—a likelihood he put at 60%—the trade-off would be worth it.

“Typically patients will tolerate a new deficit like that if it means that their seizures are gone,” he said. The effects of repeated seizures over time would be “far worse.”

After their meeting with Dr. Madura, the family decided to go ahead with the lobectomy.

Four weeks later, on surgery day, Parker watched her daughter skip through the hospital doors.

“Lily went into that surgery with the best mindset. It’s like, ‘We’re going to do this; it’s going to be fine.’”

The young patient’s prediction came true.

Dr. Madura performed the surgery on a Thursday, and Lily went home that weekend. Two weeks later, she returned to school—no big deal.

Dr. Madura’s thoughtful approach reinforced Lily’s attitude, Parker said. During pre-op, he braided and clipped back her hair, minimizing the area to be shaved.

“You could barely even tell that any of her hair was shaved—which I just think is amazing,” Parker said.

The surgeon also used unobtrusive bandages to protect Lily’s incisions.

“He’s like, ‘If we don’t wrap her head, then things seem a little bit better than (if) we treat her as if there’s something seriously wrong,’” Parker said.

“It was the most amazing experience.”

The best outcome

The best part of Lily’s story, of course, is her successful outcome.

As Dr. Hernandez gradually weaned her off epilepsy medications, the seizures remained at bay.

April 2021 marked two years free of seizures, with no medications. And all signs point to this trend continuing.

“Making it this far out without a seizure is a pretty good indication that she has a very good chance to live a life without seizures,” Dr. Madura said.

“That really is what matters—that Lily has gotten the best outcome that we could have hoped for.”

Lily’s positive results include her eyesight. Though she did lose her right-side visual fields, she adjusted easily. Now, at age 8, she plays basketball and baseball at the local YMCA as if nothing happened.

“I thought that was going to be another mountain that we were going to have to climb, and it wasn’t,” Parker said. Instead, Lily has “flourished and gained by leaps and bounds.”

For Parker, it’s all been a lesson in holding onto hope. She counsels parents facing similar situations “to trust the process, to trust your doctors and to lean into them.”

Though it’s hard, “it can be so amazing on the other side.”

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Hardware for the heart

An investigational device helped a Michigan grandmother regain her health.

Hardware for the heart

Willow Eggleston, 66, had long struggled with pulmonary arterial hypertension.

She knew it stemmed from a congenital heart defect. She also knew doctors determined nothing could be done to remedy the problem.

And she remembers precisely the moment her situation tilted from chronic illness to pure terror.

It happened in March 2019.

One minute she was minding Lillian and Evelyn, her two young granddaughters, as they peacefully watched a movie and munched on pizza. The next, she found herself sprawled back in her recliner, one of the girls leaning over her.

“Lilly kept saying, ‘Gramma, wake up! What happened? Are you OK?'” Eggleston said.

Eggleston roused herself enough to know she faced serious trouble.

“I called 911 and I called my daughter to tell her to come and get the kids,” she said. “The two little girls were still there when the ambulance came, lights flashing.”

The ambulance whisked her to Spectrum Health Butterworth Hospital.

There, doctors delivered a dire prognosis.

“When they warned me that they might have to intubate me and put me on a ventilator, it was scary,” Eggleston said. “I tried my best to stay positive.”

While it wasn’t the first time she’d been hospitalized for this condition, it would be the most frightening.

And she found it increasingly difficult to stay positive.

‘That’s just not me’

By her mid 60s, Eggleston had already weathered her share of ups and downs.

Doctors first diagnosed her heart defect, a large hole in her heart known as an atrial septal defect, when she was in her 40s. It helped explain some of the minor symptoms she’d been having, such as flutters and breathlessness, for much of her life.

But the Bath, Michigan, resident also learned she had an inoperable condition.

Any attempt at corrective surgery might be fatal.

Her symptoms worsened over the years.

“By now, I was wearing a diaper and using a walker,” she said. “I didn’t want to eat. And I had so much fluid I couldn’t lay flat on my back.”

Her care team initially suspected a double lung transplant might help, but they quickly determined she wasn’t a suitable candidate for transplant.

Eggleston found it hard to ward off despair. Her body’s systems began to fail, including her kidneys.

She remembers that terrible feeling of defeat.

“That’s just not me,” she said. “It bothered me so much that I wasn’t that strong person anymore. My daughter finally pleaded with me, saying, ‘Mom, please don’t give up.'”

Reda Girgis, MD, medical director of the Lung Transplantation Program referred Eggleston to Joseph Vettukattil, MD, a congenital cardiologist at Spectrum Health Helen DeVos Children’s Hospital.

Dr. Vettukattil has extensive experience repairing heart defects.

“He told me he could fix the hole in my heart,” Eggleston recalled. “I said, ‘No, you can’t. It’s not fixable.’ He said, ‘Yes, I can.'”

Custom from Europe

In a healthy heart, blood comes in through two large veins and then empties oxygen-poor blood into the right atrium or upper chamber.

Blood then flows into the right ventricle as the atrium contracts, which pumps it into the lungs.

Once the lungs replace the oxygen, it flows into the left atrium and then the left ventricle, which pumps it to the entire body.

Normally, that results in equal amounts of blood in each direction, Dr. Vettukattil said.

But the hole in Eggleston’s heart meant that, all her life, her lungs had been receiving three to four times more blood than the body.

This caused a constant, excessive pressure—and it was the source of her worsening symptoms.

In Eggleston’s case, the heart defect manifested as lung pressure.

“She was really unwell,” Dr.  Vettukattil said. “The fluid was accumulating in her body and she couldn’t walk. She couldn’t breathe.”

She was diagnosed with atrial septal defect-associated pulmonary hypertension, a misunderstood and inappropriately managed cardiac defect which occurs mainly in women. The reason why almost 80% of patients with this condition are women is still unclear, Dr. Vettukattil said.

Eggleston’s heart had a large hole—about 40 millimeters, or an inch and half.

“We couldn’t completely close it,” the doctor said. “Her heart wouldn’t be able to cope with the sudden change in the hemodynamics.”

Instead, in June 2019, he performed what’s called a fenestrated atrial septal defect closure, using a Fenestrated Atrial Septal Defect (FASD) Occluder from a European manufacturer. The investigational device, made by Occlutech, a Swedish company, is not yet approved for use in the United States by the Food and Drug Administration.

The FDA granted compassionate-use authorization for use of the FASD Occluder in Eggleston due to her clinical condition.

Rather than opting for open-heart surgery, Dr. Vettukattil inserted the device by threading a wire through an artery near her groin.

In 45 minutes, he reduced the gaping hole in Eggleston’s heart to 8 millimeters.

The catheterization procedure, done at Helen DeVos Children’s Hospital, goes quickly. But the body’s recovery is slow.

Children typically bounce back faster, often within six months. Adult hearts may never return fully to normal, but they do improve.

“The damage has been done to the heart for a long period,” Dr. Vettukattil said.

Much of his work focuses on pediatric patients, but it’s common for heart defects to show up much later in life. He has performed cardiac catheterizations on patients as old as 82.

Congenital heart defects are the most common birth anomalies, affecting about 1% of children born each year.

The Centers for Disease Control and Prevention estimates roughly 1 million children and 1.4 million adults have such defects.

Getting her life back

While her pulmonary arterial hypertension will never fully disappear, Eggleston has gradually felt the benefits of the procedure.

“Within a month, I wasn’t constantly sleeping anymore,” she said. “I still needed the oxygen at first, but then less and less.”

She still uses a portable tank when she goes out for walks.

“It took a year for my heart to get back to the normal size,” she said. “But I can clean the house. I get on the treadmill.”

She and her husband are traveling again, their eyes set on Orange Beach, Alabama, for some time in the sun.

And where she once took three medications to treat her pulmonary hypertension, she’s down to one.

She often pinches herself as she contemplates just how different her life is today.

“I know I’m 66, but I don’t feel it,” she said. “I feel like Dr. Vettukattil gave me another 30 years to enjoy life.”

She continues to monitor her heart rate and oxygen levels.

She and Dr. Vettukattil hope the device in her heart gains approval from the FDA. More people may then learn about the treatment over time.

“It’s this one little piece of hardware that changes everything,” Eggleston said.

Dr. Vettukattil has since fitted the new devices in five patients, all women.

“We have broken the myth that there is nothing that can be done to help them,” Dr. Vettukattil said. “And we believe this procedure will become more common as a result.”

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