Posts

Jake and Dr. Oluigbo

Doctors at Children’s National give Jake his life back

Jake and Dr. Oluigbo

At the age of 17, Jake underwent surgery led by neurosurgeon Chima Oluigbo, M.D., where he conducted a temporal lobe resection, also called temporal lobectomy, that works to lower the number of seizures, make them less severe or stop them completely. The surgery ended up being successful and it worked to greatly improve his overall quality of life.

Since 1969, November has been considered Epilepsy Awareness Month to highlight the importance of recognizing a seizure and promoting seizure first aid. At Children’s National Hospital, doctors in the division of neurology are committed to finding treatments for epilepsy and have done just that by helping Jacob Yates, an 18-year-old patient, get his life back.

For many families the holidays are meant for spending time with loved ones and enjoying the seasonal festivities. However, the holidays were not always a joyous occasion for Jake and his family. Since he was a baby, many of his holidays were spent in a bed due to a brain disorder that caused him to have developmental delays and, at times, up to 17 seizures a day.

“The holidays were always a tough time for the family because Jake would get so excited around Christmas that it would overwhelm his system and induce seizures that took him days to recover from,” says his mom, Jennifer.

Jake was born a preemie and hours after he was born, doctors at his local hospital had identified that he was having trouble breathing. By coincidence, the Children’s National transport team was on-site to take another patient to Children’s National, but once they looked at Jake they immediately took him instead by SkyBear Air Transport, the hospital’s rapid helicopter transport service.

During his stay at Children’s National, Jake was in the neonatal intensive care unit (NICU) for 11 days and was supported by breathing machines to help with respiratory distress and other issues stemming from him being born prematurely.

“If it wasn’t for the Children’s National transport team coincidentally being at our local hospital, Jake wouldn’t have survived staying at that location,” said Jennifer.

After he was taken care of at Children’s National, he was discharged 11 days later, but at the age of three months Jake was still experiencing respiratory issues and was taken back to his local hospital in Charles County.

“When he first arrived back at the University of Maryland Charles Regional Medical Center, the doctors thought he may have had cystic fibrosis, but it came back that perhaps he was suffering from reflux and they put him on medication,” Jennifer recalls. Unfortunately, this was not the cause and it would not be the family’s last visit to the hospital.

By the age of six months, Jake had his first seizure and he was flown back to Children’s National. Over the next year he was repeatedly admitted to the hospital as his seizures had caused him to stop breathing.

Between the ages of 4 to 6 years old, Jake became a patient of William D. Gaillard, M.D., division chief of epilepsy and neurophysiology and Roger Packer, M.D., senior vice president at the Center of Neuroscience and Behavioral Health at Children’s National. After his visit, both doctors recommended surgery, but Dr. Packer recommended that Jake receive an electroencephalogram (EEG), magnetic resonance imaging (MRI) and go through a sleep study first to identify the specific causes of his seizures.

Now on a new medication, his seizures were maintained for the most part, but doctors were still recommending that it was time for surgery. When Jake was 15, his parents re-evaluated the surgery and learned that their son had a 76% chance of being seizure and medication free.

At the age of 17, Jake underwent surgery led by Chima Oluigbo, M.D., neurosurgeon at Children’s National, where he conducted a temporal lobe resection, also called temporal lobectomy, that works to lower the number of seizures, make them less severe or stop them completely. The surgery ended up being successful and it worked to greatly improve his overall quality of life.

Before the surgery, Jake didn’t speak much, experienced anxiety and had difficulty expressing his emotions. He had never told his mother that he loved her. After the surgery, Jake looked at his mother and said, “I love you babe.”

According to Jennifer, since the surgery her son is a completely different person and states that he has been seizure free for over a year. Equally, Jake and the family can now all look forward to the holidays.

“We’re so excited to have him share the holidays,” Jennifer says. “He feels better and it shows through his attitude and the way he responds to things. Words can’t express the gratitude we have for the doctors at Children’s National Hospital. They gave my son his life back.”

Chima Oluigbo examines a patient

Eradicating epilepsy with Visualase

Chima Oluigbo examines a patient

Chima Oluigbo, M.D., and his team are using Visualase to identify and eliminate seizure foci and provide patients with a minimally invasive procedure for treating epilepsy.

About one in 26 people will be diagnosed with epilepsy in their lifetime. That adds up to about 3.4 million people in the U.S., or about 1 percent of the population nationwide. This condition can have huge consequences on quality of life, affecting whether children will learn well in school, eventually drive a car, hold down a job or even survive into adulthood.

For most of those that develop epilepsy, medications can keep seizures in check. However, for about a third of patients, this strategy doesn’t work, says Chima Oluigbo, M.D., an attending neurosurgeon at Children’s National Health System. That’s when he and his team offer a surgical fix.

Epilepsy surgery has come a long way, Dr. Oluigbo explains. When he first began practicing in the early 2000s, most surgeries were open, he says – they involved making a long incision in the scalp that can span half a foot or more. After drilling out a window of skull that can be as long as five inches, surgeons had to dig through healthy brain to find the abnormal tissue and remove it.

Each part of this “maximally invasive” procedure can be traumatic on a patient, Dr. Oluigbo says. That leads to significant pain after the procedure, extended hospital stays of at least a week followed by a long recovery. There are also significant risks for neurological complications including stroke, weakness, paralysis, speech problems and more.

However, open surgery isn’t the only option for epilepsy surgery anymore. Several new minimally invasive alternatives are now available to patients and the most promising, Dr. Oluigbo says, is called Visualase. He and his team are the only surgeons in the region who perform this procedure.

In Visualase surgeries, Dr. Oluigbo and his colleagues start by making a tiny incision, about 5 millimeters, on the scalp. Through this opening, they bore an even tinier hole into the skull and thread a needle inside that’s about 1.6 millimeters wide. “The brain barely notices that it’s there,” he says.

The tip of this wire holds a laser. Once this tip is placed directly at the seizure foci – the cluster of nerve cells responsible for generating a seizure – the patient is placed in an intraoperative magnetic resonance imaging (MRI) device. There, after checking the tip’s precise placement, the surgeons turn the laser on. Heat from the laser eradicates the foci, which the surgeons can see in real time using MRI thermography technology. The margins of the destroyed tissue are well-defined, largely sparing healthy tissue.

After the wire is removed, the incision is closed with a single stitch, and patients go home the next day. The majority of patients are seizure free, with rates as high as 90 percent for some types of epilepsy, Dr. Oluigbo says. Although seizure-free rates are also high for open procedures, he adds, Visualase spares them many of open surgeries’ painful and difficult consequences.

“Having done both open surgeries and Visualase,” Dr. Oluigbo says, “I can tell you the difference is night and day.”

Although open procedures will still be necessary for some patients with particularly large foci that are close to the surface, Dr. Oluigbo says that Visualase is ideal for treating medication-resistant cases in which the foci are buried deep within the brain. A typical example is a condition called hypothalamic hamartoma, in which tumors on the hypothalamus lead to gelastic seizures, an unusual seizure type characterized by uncontrollable laughing. He also uses Visualase for another condition called tuberous sclerosis, in which waxy growths called tubers develop in the brain, and for cancerous and benign brain tumors.

It’s gratifying to be able to help these children become seizure-free for the rest of their lives, says Dr. Oluigbo – even more so with the numerous updates he receives from families telling him how much this procedure has improved their children’s lifestyle.

“Visualase has completely changed the way that we approach these patients,” Dr. Oluigbo says. “It’s extraordinary to see the effects that this one procedure can have on the quality of life for patients here at Children’s National.”

Expanding awareness of SUDEP

Madison Berl

Madison M. Berl, Ph.D., is helping to expand awareness of SUDEP among patients, families and caregivers.

When 4-year-old Henry Lapham died in his sleep just weeks after being diagnosed with epilepsy in 2009, it was a shock to everyone — even his pediatrician and neurologist. Henry’s cause of death was sudden unexpected (or unexplained) death in epilepsy persons (SUDEP), a condition that causes sudden death in about 1 of every 1,000 otherwise healthy patients with epilepsy. Neither health care professional had mentioned this as a possibility, as remote as it was.

“I was desperate to make sense out of our tragedy,” writes Henry’s mother, Gardiner Lapham, R.N., M.P.H., in “Increasing awareness of sudden death in pediatric epilepsy together,” an article published in the February 2017 issue of Pediatrics. After her son’s death, by working with a group called Citizens United for Epilepsy Research, Lapham connected with other families affected by the same heartbreak. “I have met many bereaved family members,” she adds, “and the most consistent thing I hear is that they wish they had known about SUDEP.”

Now, a new collaboration with Children’s National Health System, where Henry received care, University of Virginia Medical Center (UVA) and other academic medical centers is helping to expand awareness of SUDEP among patients, families and caregivers alike. Known as Childhood Epilepsy Risks and Impact on Outcomes (CHERIO), the multiyear effort aims to develop approaches to increase knowledge about SUDEP and other conditions that can accompany epilepsy, such as attention deficit hyperactivity disorder, autism, anxiety, depression and sleep issues, according to co-authors of the Pediatrics article.

CHERIO got its start in 2014 at the American Epilepsy Society annual meeting. There, Lapham met Madison M. Berl, Ph.D., director of research, Division of Pediatric Neuropsychology at Children’s National, who studies epilepsy comorbidities. When Lapham asked what she could do to help raise awareness of SUDEP at Children’s National, she and Berl, along with William Davis Gaillard, M.D., Henry’s neurologist, hatched a plan.

Working with multiple disciplines and stakeholders, including neuropsychologists, psychiatrists, neurologists, epidemiologists, basic scientists, nurses and parent advocates at both Children’s National and UVA, CHERIO plans to assess the level of knowledge about SUDEP and other epilepsy comorbidities among medical providers and parents and to implement ways to increase knowledge. The first item on the agenda, Berl explains, was to conduct a survey to see just how much doctors knew about SUDEP.

“Although many neurologists are aware of this condition, ours was the first to survey pediatricians, and the majority was not aware of SUDEP – despite having children with epilepsy in their practice,” Dr. Gaillard says. “We know that many neurologists do not discuss SUDEP with patients and the reasons for not talking about SUDEP are varied. Thus, CHERIO felt that in addition to educating neurologists about the need to discuss the risk of death associated with epilepsy, increasing pediatricians’ awareness of SUDEP is one approach that could open more opportunities for families to have this discussion.”

To help make it easier to talk about this risk, the CHERIO team is developing strategies for doctors to start the conversation with patients and their families by framing SUDEP in the context of more common epilepsy comorbidities.

“Clinicians walk a fine line in giving information at the right time to make people more aware,” Berl adds, “but also being realistic and giving information that fits with what’s going on in a particular child’s case. By discussing SUDEP along with other, more common epilepsy risks, it brings context to a family so that they’re not unduly concerned about death – which also can paralyze a family and create unnecessary alarm.” The risk of death in most children with epilepsy is very low, slightly higher than the risks faced by healthy children. But parents of children with complicated epilepsy who have more risk factors for sudden death should be especially aware , she says.

Another way to help facilitate discussion may be through a simple tweak in the medical record, Berl adds. The team is currently developing a checklist that pops up annually in a patient’s medical record to remind clinicians of important points to discuss with patients and their families, including SUDEP.

Additionally, they are working on ways that can help families become more empowered to start the discussion themselves. Materials for the waiting room or questionnaires to fill out before appointments could trigger conversations with care providers, Berl says.

Last, the team also is collaborating with a medical device company that is working on a nighttime monitoring system that could provide an alert if patients with epilepsy experience nighttime seizures, a risk factor for SUDEP. Such technologies have not been proven to prevent SUDEP. Yet, it may help caregivers get help more quickly than if they did not receive the alert.

For each of these efforts, Berl notes, having Lapham as a partner has been key. “She’s part of our meetings and has input into the direction of each project,” Berl explains. “When you have a partner who is so close to the daily work you’re doing, it just heightens those efforts and brings to the forefront the simple message of why this is important.”