She’d hold his hand and talk to him during the sudden stiffness or muscle spasms and blank stare. She made sure he wasn’t choking. She stayed calm.

And whenever Luke had to be hospitalized at Cook Children’s Medical Center – Fort Worth, Lexi visited as much as she could. She got to know the neurology nurses, cuddled her favorite therapy dog, and watched as the staff treated Luke with innovative procedures and kindness.

Details that cater to kids made an impression on Lexi under the Blue Peaks. From the playroom to the holographic fairy on the wall … Cook Children’s was magical in her eyes. 

Lexi can’t remember a time she didn’t want to be a nurse. And she found her perfect fit in February 2025 when she started working as a registered nurse in familiar territory: the Cook Children’s neurology department. No longer a visitor, Lexi made Cook Children’s her workplace home. 

For more than a decade while they lived under the same roof, Lexi assisted her brother when his seizures flared up. That experience in caregiving gave Lexi a soft heart and a skillset that she now puts into practice caring for others. Seizures don’t intimidate her. She also sees the world through the eyes of patients’ siblings; she understands their questions and concerns.

“I love pediatric nursing. Children are resilient. They can go through so much, and their little bodies are so tired, but they bounce back and they handle it like champs,” she said. “I love taking care of the patients and their families, talking them through diagnosis, through treatment plans, everything.”

Lexi’s presence by Luke’s side has comforted him during countless seizures over the years. And if you ask Luke what makes his sister a great nurse, he’ll tell you: “Because she cares.”

Onset of Epilepsy

Seizures are caused by abnormal electrical bursts in the brain. Luke was 5 years old and Lexi was 11 in 2013 when his first seizure happened during a Mario Kart video game at their home in Arlington.

“I tried to hand him the remote, and he just couldn’t pay attention, and he couldn’t hear me,” she said. “I ran to get Mom in the other room, and by the time I came back, he was blue and shaking on the ground.” 

Luke rode by ambulance to Cook Children’s, the first of many hospitalizations. His seizures began to occur multiple times a day, sometimes in frequent clusters that medication couldn’t control. He was diagnosed with a severe form of epilepsy called Lennox-Gastaut Syndrome (LGS).  

As a teen Lexi helped their mom, Ami Waggoner, do research on Luke’s disorder. Lexi lived in the same house with her brother and parents for about 10 years after his epilepsy symptoms started. If she was around when Luke had a seizure, Lexi stepped up. She knew her role: keep Luke safe and keep him company until the seizure passed. She’d ask him to squeeze her hand or give a thumbs up if he couldn’t speak. Sometimes she’d record a video of the seizure to show his doctors. 

Ami, who is also a nurse, noticed maturity and nursing traits in Lexi from an early age. A few examples:

• At home whenever Luke had a seizure, Lexi knew where to find the rescue medications and the steps to follow.

• As a softball player and busy teen, Lexi made time after school to hang out with Luke when he was hospitalized.   

• As a volunteer at monthly neurology support group for Cook Children’s families, she provided crafts and activities for children while their parents attended the meetings. The parents knew Lexi was comfortable around seizures, and they trusted her.

Lexi’s face is Luke’s favorite sight to see when he comes out of a seizure, their mom said. Lexi always responded to her brother’s medical needs by keeping her cool and encouraging him every step of the way.  

Choosing Cook Children’s

Lexi worked as a patient care technician while attending the Tarrant County College nursing program. Her first nursing job was at another Fort Worth hospital. But while attending the 2024 Christmas party for neurology patient families at Cook Children’s, she realized she missed the magic. She wanted to work at Cook Children’s.

Ami remembers what Lexi said after deciding to work with pediatric patients like Luke. I think this is my place. This is what I feel like I'm made for.

When Lexi pivoted in her nursing career to join Cook Children’s Neurology, her mother saw all the pieces fall into place. “So yeah, I’m extremely proud,” Ami said. “It’s grown into this amazing thing where she wants to help so many others.”

Lexi works night shift on the medical center’s fourth floor, which includes the epilepsy monitoring unit. Some of her colleagues have helped care for Luke through the years. If Luke is admitted, Lexi can’t be his nurse.

During her teenage years she absorbed the Cook Children’s culture each time she visited her brother in the hospital. She saw nurses practicing safety, respect, generosity and other values. She felt the impact of family-centered care. Now, in a journey that’s come full circle, she’s back at Cook Children’s and paying it forward.

“I definitely have a passion for epilepsy. I have a passion for finding the cure. I have a passion for helping out the siblings on our unit as well,” she said.

When she encounters a patient’s sibling, she checks to make sure they’re OK. Lexi takes time to explain whatever medical procedure their brother or sister is undergoing, like the electrodes hooked up for electroencephalogram (EEG) monitoring.  

Lisa Mayfield, RN got to know Lexi while taking care of Luke for many years on the epilepsy unit at Cook Children’s. She remembers Lexi cheering him up by bringing his favorite snacks, watching movies with him and walking with him around the unit. His seizures didn’t rattle her.

“I was beyond excited when Lexi joined our team,” Lisa said. “She has a unique prospective that she can share with her patients and their families. She has empathy and an understanding that is unique to families that live with epilepsy every day but don’t let it define them. Lexi is an amazing nurse with critical thinking beyond her years. I am excited to watch her continue to grow in the field that she has been training for her whole life.”

When a new patient comes in, Lexi wants the family to know they’ll get the best neurology care possible from a team that delivers on the Cook Children’s Promise to do everything for the child. 

Celebrate the Struggle

Now age 18, Luke still experiences seizures every day. But his health and quality of life have improved thanks to a 2021 surgery at Cook Children’s that implanted a deep brain stimulation (DBS) tool. DBS -- a network of devices and wires -- sends small electrical impulses to specific areas of Luke’s brain. It’s designed to keep the worst seizure activity under control.

Luke’s parents collaborate with epileptologists Cynthia Keator, M.D. and  Jaehyung Lim, M.D., who oversees the DBS, and the specialists at the Jane and John Justin Neurosciences Center at Cook Children’s. They closely monitor how he tolerates different electrical amplitudes and speeds, and make adjustments as needed.

Because of DBS, Luke was able to cut back on medication. He can think more clearly. He’s more energetic.

The Waggoner family has the confidence to travel on vacations they couldn’t take prior to Luke’s DBS surgery. They appreciate the things Luke enjoys, especially trains and trips to the zoo. Their family motto? Celebrate the Struggle.  

“Living with Luke taught me to make the best of any situation. He always has a positive attitude,” Lexi said. “Even though I’ve moved out, Luke is still such a big part of my life. We’re very, very close. I’m thankful that he is doing as good as he is right now, and I love to spend time with him.”

RELATED STORIES:
Brain Stimulation Curbs Teen's Worst Seizures
Teen Becomes First in North Texas to Undergo Landmark Brain Surgery for Epilepsy

Cook Children’s doctors and researchers are aiming to make an impact on the progression and treatment of Lennox-Gastaut Syndrome (LGS), a rare type of epilepsy that causes seizures that often lead to a cognitive or developmental delay. The seizures typically begin in childhood and LGS leads to increased risk for other health problems and mortality.

The Cook Children’s Lennox-Gastaut Comprehensive Care Center has multiple research projects underway that aim to provide vital information to better understand LGS progression, develop preventative interventions and develop more effective, targeted treatments. Early recognition and active management are essential for improving developmental outcomes.

Each year, about 300 to 400 patients with LGS visit Cook Children’s Neurology and about 50 patients are newly diagnosed with LGS.

Cynthia Keator, M.D., Division Chief, Neurology at Cook Children’s Jane and John Justin Institute for Mind Health and the Cook Children’s Epilepsy Monitoring Unit (EMU), has dedicated her career to helping children with Developmental Epileptic Encephalopathy (DEE), specifically LGS.

Cook Children’s awarded Dr. Keator as an Endowed Chair in 2024, leading the Cook Children’s Lennox-Gastaut Comprehensive Care Center.

The center will follow patients, gather data and conduct a more comprehensive study of treatments, including surgery and medications. It is the first comprehensive care center in the country to work on algorithms, protocols and standardized guidelines for LGS care. The goal is to be the main site for clinical trials for new medication therapies for LGS.

The Challenge

Diagnosing LGS can be difficult for epileptologists because symptoms can change over time and some patients may not show some signs until later in the disease progression. LGS is characterized by multiple seizure types, developmental delay and distinct electrical patterns in the brain.

“There is a great degree of variability in the LGS population in terms of when diagnosis is given, or lack thereof, treatments and overall care,” Dr. Keator said.

Patients typically experience a wide range of comorbidities and require substantial support from caregivers and physicians throughout their entire lives.

Common symptoms include frequent seizures which may be drug-resistant. There are common seizure types which may be experienced, including tonic seizures (the most common type), atypical absence and drop attacks (atonic seizures). Other symptoms include:

• Developmental delay or loss of developmental skills
• Cognitive disability and/or impaired communication
• Behavioral issues
• Sleep issues
• Psychiatric issues
• Autism, depression, anxiety, psychosis

The Program

The three pillars of the program include the LGS Clinic, the LGS Natural History Registry, and the Biomarker Study.

The natural history registry helps see how the disease progresses over time in the LGS patients at Cook Children’s and will identify characteristics that confer high risk for LGS development. This will be done in the first two years of the project.

During the following two years in the Biomarker Study, patients with patterns and risk identified from the natural history registry will be captured in the data to determine whether their condition progresses to LGS. The study aims to alter the course of the disease progression.

This analysis will help clinicians recognize early disease symptoms, their progression, severity and frequency.  

Another vital research aspect that could make a considerable difference is identifying what helps identify at-risk populations before they develop LGS, with the potential to prevent it.

Dr. Keator also works with a dedicated Cook Children’s Neuroscience Research team to help her with data collection and research analysis. Christopher Hagen, Ph.D., is the new Research Scientist working with Dr. Keator.

“The journey for these families is incredible,” Dr. Keator said. “For them to know there is a large community of doctors, nurses, scientists and others working together to improve their conditions gives them hope.”

Care and Advice for Families with LGS

Living with epilepsy, or living with a family member who has epilepsy, can be the most challenging experience of one’s life. There is always more to learn and more that can be done in the medical field to help those impacted. For many, what epileptologists and other epilepsy specialists are doing in the medical field is the only hope they have.

“We are always striving for the next best treatment and so are our colleagues throughout the world,” Dr. Keator said. “The impact of epilepsy on a family and community is overwhelming. There is a large community of us working to improve quality of life, access to care, outcomes, treatments and ultimately a cure.”

November is Epilepsy Awareness Month. Epilepsy will affect one in 26 people in the United States during their lifetime. If you have a child with epilepsy, you’re not alone – 3.4 million Americans have this disorder. However, amazing things are happening in genetics, research, medication, surgery and treatment of pediatric epilepsy, bringing hope to more patients than ever before. If your child has been diagnosed, you probably have lots of questions. We can help. If you would like to schedule an appointment, refer a patient or speak to our staff, please call us at the Jane and John Justin Institute for Mind Health.

Discover how rare genetic epilepsies led him to international recognition, why Cook Children's stood out as his perfect home, and how his dedication to research is transforming the future of neurological care. Plus, we explore how art and neuroscience intersect in a way that heals, inspires, and brings the community together.

Join us on Spotify, Apple Podcasts or YouTube for a powerful conversation about innovation, perseverance, and the human connection behind medicine.

A panel of scientists addressing U.S. Congress members on Wednesday includes Professor Christos Papadelis, Ph.D. representing Cook Children’s research and innovation to treat pediatric epilepsy.

Dr. Papadelis leads the Neurosciences Research Center at Cook Children’s, which undertakes groundbreaking studies and collaborates in the care of patients with seizures.

He’s among the experts invited to speak at the Rayburn House Office Building in Washington, D.C. as part of Brain Mapping Day. The Society for Brain Mapping and Therapeutics (SBMT) sponsors the annual event. Its goal? Educating federal lawmakers about current approaches to treating neurological and psychological disorders.

Brain Mapping Day also aims to shape future policies and funding for health care by promoting new strategies to advance the neurosciences.

“It’s a fascinating field, and so much progress has been made in the last several years,” Dr. Papadelis said. “But so many things should be done to translate the existing technology to the clinical practice. We need more interface between the translational research and the clinical teams to bridge them together.”

Dr. Papadelis wants to share his enthusiasm for the strides his team at Cook Children’s is making to pinpoint the exact location of the brain where seizures originate in patients with epilepsy. He and his research colleagues combined two types of imaging that measure the brain’s electrical and magnetic activity. When a patient needs surgery to control their drug-resistant seizures, the researchers can provide the neurosurgeon with the precise point of seizure activity. That mapping tool helps ensure a successful surgical outcome while minimizing the risk of complications in other areas of the brain. 

Dr. Papadelis earned a spot in the lineup of presenters because of his insights and leadership in neurosciences research. He’ll address House members and congressional staff in a portion of the program titled “Detecting Neurological and Neuropsychiatric Disorders Earlier Using Brain Screening Protocols. What Should Be the Policy?”

His overview covers three main points:

• Effective technology is already available.
• Many patients can’t access the technology because they lack health insurance, or insurance approval is delayed, or other barriers.
• More research funding from the National Institutes of Health (NIH) is needed to continue to make advancements.

He plans to talk specifically about pediatric epilepsy. He wants to make the audience on Capitol Hill aware of the research Cook Children’s is doing to help patients become seizure free – and how policies in health care affect those efforts.

M. Scott Perry, M.D., epileptologist and head of Cook Children’s Jane and John Justin Institute for Mind Heath, said the high-level research by Dr. Papadelis and his team is resulting in better understanding of nervous system disorders. Their contributions are getting noticed and putting Cook Children’s on the map.

Dr. Perry said he hopes Dr. Papadelis’ presentation in Washington, D.C. attracts additional collaborators and brings more exposure to the Cook Children’s research model, which operates outside of a traditional academic setting. 

“By focusing on research that delivers near-term benefits to the patients we serve daily, Cook Children’s is able to grow a strong bond between clinical medicine and research that benefits our patients, our medical providers, and our research team -- all in the course of day-to-day practice of medicine,” Dr. Perry said.

William Stigall, M.D., Chief Research Officer, said the invitation to participate in Brain Mapping Day reflects a growing recognition of the research and clinical excellence at Cook Children’s. He credited Dr. Papadelis with academic depth and rigor that complements the work of Dr. Perry and other physicians on the clinical side.

“Dr. Papadelis and physicians at Cook Children’s are performing cutting-edge research and are extraordinarily well positioned to offer insights to our nation’s leaders,” Dr. Stigall said. “The more the rest of the world knows about us, the better the rest of the world will be.”

More about Dr. Papadelis

Born in Athens, Greece, Dr. Papadelis earned a doctorate in Medical Informatics. After stretches of research in Japan and Italy, he came to the United States in 2011 as an instructor in Neurology at Harvard Medical School. He was founding director of the Clinical Magnetoencephalography (MEG) Program at Boston Children’s Hospital.

He moved in 2019 to the Neurosciences Research Center at Cook Children’s in Fort Worth. He also is a Professor of Pediatrics at the Texas Christian University Burnett School of Medicine and of Research in Bioengineering at the University of Arlington.

He’s excited to tell policymakers about a five-year brain-mapping study he’s undertaking, funded by a $2.3 million grant from the NIH. Dr. Papadelis described the project as a unique technique that uses more than 550 sensors on a patient’s head to simultaneously record electrical and magnetic activity in the brain.

“This setup we have here is unique in the country and helps us to identify non-invasively and with high precision the area in the brain to be ablated or resected in surgery in order for patients with drug-resistant epilepsy to become seizure free,” he said. With additional NIH funding, he said, more progress can be made across neuroscience.

He points out that the Justin Institute at Cook Children’s bridges the gap between research and clinical application. The two areas connect to integrate new technology in ways that directly impact the patients.  

Dr. Papadelis, a naturalized American citizen, said he’s honored by the opportunity to share his perspective at the Capitol. And he’s proud to represent Cook Children’s and his research colleagues.

“We're motivated, and we have passion for what we're doing,” he said. “Although we're not physicians, we work closely with the clinical teams, and that relationship helps us feel a responsibility to these kids and families. Every time I see patients in the elevator, for example, I feel emotional. I'm part of helping these kids.”

RELATED STORIES:

History in the Making: Cook Children's Secures HIH Grant

Major Scientific Discovery from Cook Children's Neuroscience Research Center

Groundbreaking Trial Targets Genetic Cause of Epilepsy

Nearly 500,000 children nationwide have epilepsy. For 70% of them, medication successfully controls their seizures. But for 30% of children with epilepsy, medications fail to control seizure activity, making them candidates for surgical intervention.

“For kids whose seizures can’t be controlled with anti-seizure medication, this is a huge burden for the family and the children,” Dr. Papadelis said. “Sometimes these kids have several seizures per day. Often, the best available treatment for them is brain surgery where the neurosurgeon dissects the area of the brain where the seizures originate. If we successfully disable this area, either with laser ablation or with resective or disconnective surgery, we are able to control the seizures and the child can become seizure-free.”

Critical Precision

For successful brain surgery with minimal complications, precise brain and seizure mapping is crucial to interrupting seizures while also preserving crucial physiological functions of the brain, such as language and movement. In many cases, current techniques in brain mapping for seizure localization can precisely pinpoint where seizure activity originates in the brain, known as the epileptogenic zone. These techniques include electroencephalography (EEG), computed tomography (CT) and magnetic resonance imaging (MRI), as well as other modalities.

But not all seizures have a single point of origin. Some seizures originate from a synchronized network of abnormal electrical activity in the brain. In these cases, one test may point to one location for seizure origin, while another localizes a different area of the brain as the culprit.

“There are several new studies, including ones published by my research teams, that show in these cases it's not a single area of the brain which is involved in the generation of the seizures but, rather, is the whole brain network that is somehow synchronized during seizures,” Dr. Papadelis explained.

Neurosurgeons can use intracranial-EEG (iEEG) to help identify epileptogenic networks within a patient, but this test also has limitations, one of them being its invasiveness.

“Many children with epilepsy require an invasive surgery in which we implant electrodes directly into the brain to help us better understand where their seizure activity is starting, prior to us offering a more definitive surgery intending to stop the seizures,” said Daniel Hansen, M.D., a Cook Children’s pediatric neurosurgeon and medical director of Neuro-Trauma. “Dr. Papadelis’ research may potentially lead to not needing this step for some children.”

In addition to being invasive, an iEEG only records activity within the area of the brain where the electrodes are placed rather than throughout the entire structure. These testing variations and limitations make it difficult for surgeons to pinpoint the entire zone within the brain where surgery should be performed in order to interrupt the whole of an epileptogenic network.

Seizure-free Future

Dr. Papadelis aims to give neurosurgeons and epileptologists a new method to localize seizure onset more completely.

“A more detailed understanding of seizure networks will allow us to better counsel patients on their true chance of being seizure free after surgery, while also allowing us to be more precise and targeted with our surgeries,” Dr. Hansen said.

Dr. Papadelis’ study will explore the effectiveness and accuracy of combining the outputs of two types of non-invasive imaging and brain mapping techniques as they’re performed simultaneously — magnetoencephalography (MEG) and high-density EEG (HD-EEG). The MEG test measures the magnetic fields produced by the brain’s electrical activity, while the HD-EEG records brain activity using more than 500 closely spaced electrodes placed all over a patient’s head. Such a setup is unique in the country.

In addition to improved seizure mapping and surgical precision, this new technique may also open the door for children whose cases were previously considered inoperable. 

“This type of work potentially helps not only the many difficult cases we currently encounter, but could uncover other cases that would be good surgical candidates, whereas previously they would not have been considered,” said M. Scott Perry, M.D., epileptologist and head of the Jane and John Justin Institute for Mind Health at Cook Children’s. “Take cases of apparent generalized epilepsy, which is essentially epilepsy coming from the whole brain, as an example. In some instances, this is just a more diffuse network that may still be eligible for treatment if we locate the primary hubs.”

The study will begin in March 2024 and continue for five years. Dr. Papadelis and his colleagues believe this new seizure mapping technique will give physicians a better read on the scope of their patient’s epilepsy, giving more children the chance for a seizure-free future.

Medical students and resident physicians in Amarillo will get more exposure to pediatric neurology thanks to a new and unique partnership between Cook Children’s and the Texas Tech University Health Sciences Center (TTUHSC).

The TTUHSC School of Medicine and Cook Children’s worked together for four years to design and implement their collaboration. It’s an innovative model of health care teamwork: Two Amarillo-based Cook Children’s neurologists serving as TTUHSC teaching physicians. Their job? To evaluate how students and residents interact with patients. 

“The main philosophy is to provide the best care for children in our community. This partnership provides accessibility to more providers,” said Shannon Herrick, M.D., regional chair for TTUHSC and associate professor of pediatrics. “At the same time, it allows learners who are going to go out to these surrounding communities in the future to learn from these providers. That's the goal.”

A little background helps put the partnership in context. Scott Perry, M.D., an epileptologist and head of Cook Children’s Jane and John Justin Institute for Mind Health, began seeing patients in Amarillo once monthly more than eight years ago, in an effort to bring pediatric neurology care closer to the children of West Texas. As the only pediatric neurologist coming to the area at the time, Dr. Perry formed strong relationships with local referring physicians.

Texas Tech Physicians group hired pediatric neurologist Diana Lebron, M.D. several years later to provide full-time neurology care to the area. Her role at the Headache Clinic and General Pediatric Clinic included giving feedback to TTUHSC medical students and residents during their neurology rotations. However, it was clear the demand for pediatric neurology services required another full-time provider.

Meanwhile, pediatric neurologist Rolla Shbarou, M.D. began seeing patients two years ago at Cook Children’s Pediatric Specialties Amarillo. Her fellow neurology specialists in epilepsy and movement disorders, along with doctors in other specialties, continued to come from Fort Worth periodically. But Dr. Shbarou was the clinic’s only full-time physician.

Now, with the new partnership, Dr. Lebron joined Dr. Shbarou at Cook Children’s Pediatric Specialties Amarillo, located at 17 Care Circle. Cook Children’s welcomes Dr. Lebron’s patients to her new location. It’s also a new site for the medical students and residents as they’re being mentored by both Dr. Shbarou and Dr. Lebron as clinical professors.     

Pediatric neurology involves treatment for babies, children and teens who experience seizures, stroke, cerebral palsy, movement disorders, brain injury, chronic headaches and other conditions. The partnership between TTUHSC and Cook Children’s is designed to make access to neurology broader and more convenient for patients in and around Amarillo. Dr. Lebron and Shbarou provide general neurology care daily, while Dave Shahani, M.D. and Dr. Perry provide specialized epilepsy services and Stephanie Acord, M.D. provides movement disorders/spasticity services during monthly trips to Amarillo.

Dr. Perry said the arrangement helps make neurology care available closer to home for families in the Texas Panhandle and beyond.

“Many people we take care of in neurology have complex needs, maybe developmental delays, mobility issues and equipment,” he said. “The kids might be having seizures, and you don’t want to have to travel long distances if you can avoid it for a 30-minute appointment.”

Here’s a closer look at the advantages for each group impacted by the merged approach.

Medical Students and Residents

TTUHSC uses a strategy called academic medicine. Dr.  Herrick explained that students and resident physicians go through weekly or monthly rotations in various specialties – not just neurology, but also cardiology, sports medicine, hematology/oncology and more. Clinical professors critique how the learners handle encounters with patients, such as taking medical histories or conducting physical exams. In the process, the learners pick up on delicate skills.

“It’s a lot of the nuances of bedside manner, the nuances of how do you handle a difficult case and help the family cope and do it in an understanding way,” Dr. Herrick said. “There’s a complexity with children who have seizures or chronic disease that is hard sometimes for families to handle. That’s the art of medicine, and we’re driven to teach that.”

Groundwork for the partnership dates back to 2019, when Dr. Herrick reached out to see if the neurology team at Cook Children’s might be able to support Dr. Lebron so that she wasn’t always on call. That initial conversation grew to a discussion of possible ways that TTUHSC could partner with Cook Children’s on a larger scale.

One advantage of this new collaboration is that students and residents will see an increased number of general neurology and complex neurology patients. They’ll also experience different teaching styles between the two neurologists. Students and residents will do the initial exams and other procedures, with Dr. Lebron and Dr. Shbarou repeating each step to make sure nothing is missed. Families are very understanding of the teaching process, Dr. Herrick said.

The arrangement provides students and residents with greater exposure to a diversity of disorders. And when neurologists from Fort Worth visit Amarillo, they could give lectures to TTUHSC classes on epilepsy, movement disorders or other subspecialties. The intent is to demonstrate the bigger picture of neurology to more prospective neurologists.

“I hope to inspire medical students and pediatric residents, encouraging them to consider a career in pediatric neurology,” Dr. Lebron said. “Introducing residents and students to Cook Children’s doctors could lead to more research and innovation that benefits our patients and inspires future health care professionals.”

Patient Perspective

Many neurology conditions can be treated at the Cook Children’s clinic in Amarillo. But it won’t eliminate long-distance travel in every case, because patients who need certain care might have to go to Fort Worth. Dr. Lebron specializes in headaches in part because she too suffers from migraines.

“We don’t have biological markers for this, and so it makes it challenging,” she said. “To me, it’s exciting to be able to strategize and treat headaches. We have so many tools that we use to try to improve their quality of life.”  

Dr. Lebron’s patients seek her out from as far as southern Kansas. She notified them in anticipation of her move to Cook Children’s in late 2023. A native New Yorker, Dr. Lebron has Puerto Rican and Cuban roots, and she speaks fluent Spanish.

“Being bilingual is a big plus because it helps me connect with more patients and their families. It’s about breaking down language barriers and making sure everyone gets the care and information they need in a way that makes them feel at ease.”

Physician Perspective

TTUHSC will pay teaching stipends to Dr. Lebron and Dr. Shbarou. They’re able to consult with one another as colleagues, plus enjoy the backing of Cook Children’s neurologists in Fort Worth and Prosper to share on-call nights and weekends. 

“It’s hard to get a specialist in rural areas and hard to keep a specialist in those areas until you have some amount of inertia, which is generally created by a team of people,” Dr. Perry said. “To have another provider in the office to talk to means you’re not off on your little island by yourself.”

He and Dr. Herrick expressed hope that this joint investment will strengthen and stabilize the long-term outlook for neurology care in the Amarillo area. The model could potentially expand to other specialties beyond neurology. They say it’s important to emphasize the cooperation and mutual benefit for both TTUHSC and Cook Children’s.

“We wanted to make sure we did it right and to make sure that everyone knew that this was a partnership. We're not competing,” Dr. Herrick pointed out. “This is to enhance the health care of the community.”

The Justin Institute will connect nine specialties under one roof, including Neurology, Neuropsychology, Neurosurgery, Pain Management, Physical Medicine and Rehabilitation, Psychology, Psychiatry, Developmental Pediatrics and Developmental Psychology. Find more information, including video, here.

More details here: New Institute Connects the Dots Between 9 Medical Specialties Under One Roof

The highly anticipated Jane and John Justin Institute for Mind Health will open its doors at Cook Children’s this month. The Institute brings together nine specialties that treat disorders of the nervous system, allowing kids with neurological conditions to receive the holistic care they need under one roof.  

“The concept behind the institute is that these nine specialties share not only a lot of patients, but the same working system in the care that we provide,” says the head of the Justin Institute, Scott Perry, MD. “We want to improve the patient experience and outcome by making sure we're collaborating in the care of every patient, every day, at all times.” 

The Neuro Art Collection

To prepare for the arrival of patients and their families, Dr. Perry curated a collection of awe-inspiring artwork that combines creativity with elements of neuroscience. 

“I love art very much, and it’s a great way to connect with kids,” he said.

Every artist involved in the neuro art collection has some connection to neuroscience — some are PhD neuroscientists themselves and others have disorders of the nervous system. 

“A lot of people think neuroscience and the brain are super complex,” Dr. Perry said. “We hope this art draws people in to learn a little bit more about it.” Each artwork will have a corresponding QR code that links to information about the artist, how the piece was made, and how it’s connected to neuroscience.

Featured Artists and Artworks

The very first piece in the neuro art collection was created by embroidery artist, Laura Bundeson. Dr. Perry asked her to make a custom piece out of Epilepsy Awareness T-shirts he had designed over the years, and she stitched them together into the shape of a brain. Dr. Perry also worked with Bundeson to create a custom brain pin for all Justin Institute employees to wear.  

Michele Banks, a Washington D.C.-based artist, painted watercolor brains for the collection. She infused each work with calming, nature-related designs, including rivers, trees, fields, and oceans.   

Glass artist and retired oncologist, Reneé Tegeler, created four vibrant fused glass brains. Each piece represents one of the institute’s main groups—neuroscience, child study, psychology, and psychiatry.  

Developmental neurobiologist and woodworker, Louis-Jan Pilaz, contributed several wooden animals made up of different types of brain cells.  

“We presented him with the idea of making animals out of neurons to relate to the kids we take care of, and he took it and ran with it,” Dr. Perry said.

The Institute will also feature a nine-by-fifteen foot mural by blind painter, John Bramblitt. 

“I can’t wait to see the final product because I asked him to hide things in the picture for kids to find,” Dr. Perry said. The mural will also pay homage to the recently retired founding members of the Cook Children’s neurology and neurosurgery departments. 

A Comfortable Space for Patients and Families

“When we thought about creating this space, we thought about the kinds of children that are going to be seen here,” Dr. Perry said. “Kids with behavioral developmental disorders — things like autism — and we thought, what can we do to make it a comfortable environment for them without too much sensory overload?”

Every detail of the Justin Institute has been carefully chosen to create a stimulating, yet soothing space for kids with neurological conditions and their families. Whether it’s searching for a clue on a mural or trying to guess what kind of cells a wooden animal is made of, the Institute will offer limitless ways for patients to engage with art and science in new and exciting ways.

Related Articles: 

• It's a Colorful Life: Mural at Justin Institute Created by World-Renowned Painter who is Blind
• New Institute Connects the Dots Between 9 Medical Specialties Under One Roof

Join us for “Under the Lights,” an inspiring story about a teen with epilepsy, followed by a Q&A with the film's writer and director Miles Levin and executive producer Greg Grunberg. This event is hosted by Scott Perry, M.D., an epileptologist and head of Cook Children’s Jane and John Justin Institute for Mind Health. 

This event begins at 6 p.m. with light appetizers, followed by the film screening and virtual discussion. Registration is required to attend. Get your complimentary tickets here.

The Story

Under the Lights is the story of Sam, a boy with epilepsy, so desperate to feel like a normal kid, he goes to prom knowing that the lights will make him have a seizure. 

Cinema has historically stigmatized and ignored people with epilepsy. A demographic of 1 in 26 who have almost never been represented authentically on screen, and suffer from brutal stigma every day. Written and Directed by filmmaker with epilepsy, Miles Levin.

Learn more about "Under the Lights" here.

In recent years, robots have emerged as invaluable tools in health care by performing everything from the routine task of delivering a patient meal to assisting in highly complex surgical procedures. Their efficiency and precision can help medical professionals save time and save lives.

But a new type of robot ─ one capable of painting with artistry and expression ─ is helping patients at Cook Children’s Medical Center tap into their creativity as they navigate difficult diagnoses and circumstances. The painting robot known as Spikelangelo, or “Spike,” was first rolled out to patients on the epilepsy monitoring unit at Cook Children’s Jane and John Justin Institute for Mind Health.

Oftentimes, patients in the epilepsy monitoring unit spend up to a week in the hospital being monitored continuously for seizure activity. The time can be isolating and slow to pass.

“Creating art with the painting robot gives our patients something to take their minds off of why they are in the hospital,” said M. Scott Perry, M.D., an avid art lover, epileptologist and head of the Justin Institute. “It gives them an activity and a way to engage with something outside of their rooms.”

Therapeutic art activities are a regular part of treatment for Cook Children’s patients, especially for those struggling to express themselves as they undergo tough medical interventions.

“There’s so much research to support the multitude of benefits art offers for everyone in the health care setting, which can be unpredictable, challenging, stressful and frightening for children,” said Jill Koss, director of Family Support Services at Cook Children’s. “We use art as a way for people to explore what they’re going through, to express their feelings, and even to explore who they are because that could be changing and evolving through this process.”

Connect, Create and Collaborate

To produce a masterpiece with the help of Spike, budding artists begin by using their fingers or a stylus, just like they would a paintbrush, to create a work of art on a computer tablet in the comfort of their hospital rooms. Working remotely from its art studio near the medical center’s Child Life Zone, Spike maneuvers its brushes to mirror the artist’s strokes and paints the masterpiece onto a canvas. The studio’s clear enclosure allows hospital staff and guests to watch the robotic artist at work.

Spike’s capabilities are powered by artificial intelligence (AI) algorithms designed to meet the child artist at their level of ability, creativity and attention span. The robot can paint exactly what the creator imagines, or go a step further and provide assistance with color choice and even complete an unfinished creation based on the style and expression of the artist.

For hospitalized children in isolation, Spike can be as much a connector as it is a painter. Shared pieces designed and offered by the creative arts program will give patients the opportunity to virtually collaborate as they work individually and consecutively on the same project.

“I can see, for instance, several kids on the floor working together on a project by each adding their interpretation on how they would paint it,” Dr. Perry said. “And then Spike blending it all together into one painting.”

Patients will be able to take their masterpieces home as a reminder of their creativity, ability and resilience in the midst of challenging circumstances or use them to decorate their hospital rooms. Some may even be displayed throughout the medical center.

Fatherhood Inspires Innovation

Spike was created by artist and roboticist Pindar Van Arman after the responsibilities of fatherhood limited the time he could spend painting each day.

“When I had my children I started having more fun hanging out with my kids, changing diapers and doing stuff that parents do, and all of a sudden I was out of time,” Van Arman said. “I went from painting every day for several hours to realizing it had been a week since I painted.”

In the beginning, Van Arman programmed the robot to perform the simple yet time-consuming and tedious tasks required to prepare a canvas, mix paints, layout the proportions and paint a background. His digital art assistant worked so well that he continued to expand the machine’s capabilities, programming AI algorithms and providing feedback loops for each new idea or task.

“Every couple of months I would wonder what else I could teach it,” Van Arman said. “Like now that I have it painting could I get it to help me actually choose the pallets? Then I added a little artificial intelligence to help me with composition.”

Over the past 16 years, Van Arman has written more than two dozen algorithms to build the brain of the robot and develop it into a full-fledged digitized AI artist that can paint in the same expression and style as its creator as well as make creative decisions of its own.

“With every stroke and painting I make I’m providing feedback and training the robot, and it uses each painting as input for the future,” Van Arman said. “It’s a collaboration with myself — me being the artist and the robot imitating as much of my artistic ability as I could program into a machine. Basically, it’s a digital version of myself.”

The Future of Spikelangelo

Spikelangelo’s residency at Cook Children’s Medical Center is a collaboration between Dr. Perry and Van Arman. The two became friends after Dr. Perry purchased one of Van Arman’s paintings on eBay.

Bringing Spike to the medical campus has been several years in the making as they worked together to program, test and install a machine that meets the specific needs and attention span limitations of child artists. Van Arman made a number of customizations geared towards children, like increasing the number of colors the robot can use, developing paint formulations that don’t dry out too quickly, the addition of a paint-by-numbers feature and the ability to change the width of the paint stroke.

The robot was generously donated to Cook Children’s by Van Arman’s Creative Technology Foundation and is supported by a number of the health system’s donors. Employees at Van Arman's company, Artobotics, also volunteered their time to the project.

Spike’s pilot residency will help shape the robot’s future and Van Arman’s vision for its potential capabilities.

“Through its use at Cook Children’s, I want to find out what is fun for these kids,” Van Arman said. “I want to learn what they enjoy and tailor the robot to them. If it’s popular, I would love to expand it into other venues for children.”

Dr. Perry’s vision is to grow its therapeutic use throughout the medical center and is particularly excited about the possibility of adding voice command for children with cognitive impairments and neuromuscular disabilities. He hopes Spike will one day connect children in hospitals all over the country.

In the meantime, Spike will create, entertain and brighten the days of young artists who visit him in Fort Worth.

By Heather Duge

Valerie Shorten, BSN, RN, and her son, Marquis Shorten, are no strangers to overcoming obstacles. Each one has led them to where they are today.

Marquis faced a life-changing diagnosis at Cook Children’s in 2022 – the same hospital where his mother Valerie works. She was determined to become a nurse after her brother was killed.

“I always thought about what I could have done to help him had I been there,” Valerie said. “That’s when I knew nursing was my calling.”

Nursing Journey

In 2011, Valerie found out about an open position at Cook Children’s – a place she had visited a few years before when Marquis had a minor injury while playing with his brother.

“I remember how caring everyone was and thinking with three boys it was probably not the last time I would be there,” Valerie said.

After 15 months of working in Food Services, she applied for a secretary position in the Neonatal Intensive Care Unit (NICU). In May 2021, she graduated from nursing school and started as a nurse resident at Cook Children’s -- 10 years to the day of her first day in the cafeteria. She rotated through all the ICUs and the emergency department in one year. Now she is a nurse in the Cook Children’s NICU.

“I know that no one chooses to come into this hospital,” Valerie said. “You never know what a person is going through, so I make sure I always give the kind of treatment I would want for my child.”

Shocking Diagnosis

In April 2022, Valerie was in the middle of her nursing shift when she received a text from Marquis that read “My spine hurts.” She told him to take ibuprofen since she thought it could be related to his running.

“Marquis is a runner and was No. 1 in the district,” Valerie said. “He was slated to run in the regional meet to see if he would qualify for state. I thought the pain could be from a pulled muscle or running injury.”

Valerie told him to rest and let her know if it worsened. Eventually, Marquis drove himself to the ED at Cook Children’s and at that point could not feel his right leg.

“I met him at the ED but didn’t think it was anything too severe,” Valerie said.

An MRI revealed a spinal epidural hematoma, which is like a pooling of blood on the spine, and Marquis underwent emergency surgery. If too much time passed, Marquis could have become paralyzed.

“Everything was a blur and I remember being overly emotional as Marquis continued to lose the feeling in his legs,” Valerie said. “But I knew he was at the right place.”

Marquis was quickly wheeled to the operating room where Medical Director of Neuro-Trauma, and pediatric neurosurgeon Daniel Hansen, M.D., removed the large blood clot that was compressing the spinal cord.

Recovering Physically and Mentally

Valerie became anxious thinking about Marquis's mental state when he woke up and realized all he worked for was not going to happen that year at the regional track meet, if at all. 

“A nurse pulled his father, DeMario, and me aside and told me that when Marquis woke up, he asked when he would leave that day because he had a track meet in a few days,” Valerie said. “The real pain came when all the times were posted from the race he missed.”

Valerie and DeMario helped Marquis work through his emotions but did not let him stay down too long.

“I told him he could decide to lay there and be sad or decide what’s going to happen next,” Valerie said. “My life motto is that you are the writer of your story.”

Nurses realized how hard it was for Marquis to face the reality that he missed the regional meet and was not sure what running would look like in the future. During the 10-day stay in the Pediatric Intensive Care Unit, Marquis says the nurses went above and beyond to bring him comfort. On a particularly hard day, the café was out of Chick-fil-A milkshakes, so the nurses gathered all the supplies and made him one.

“They did everything I needed before I even asked for it,” Marquis said. “They fixed my pillows a certain way, brought me the Gatorade flavor I liked with a straw, broke up pills because I couldn’t move my neck to swallow and positioned my toes for me when I couldn’t move them.”

Back on Track

Valerie says everyone was surprised at how fast Marquis progressed. Throughout his healing journey, he used a brace, walker and wheelchair. After lots of hard work in physical therapy, he was cleared to jog four months after surgery and in January 2023 ran in his first race since surgery at Texas Tech University.

“My parents kept me going,” Marquis said. “My track coaches Jesse Heard and Sa’Donna Thornton also were there for me. Coach Heard gave me a love for track and all he instilled in me is a big reason why I treat everyone with kindness. He has sacrificed countless hours just to help me succeed. Coach Thornton played a huge part in helping me gain my confidence back and keep my positive outlook on the situation. I knew God had a plan and it would turn out OK.”

A Lifelong Dream Come True

Marquis says coming back from a major surgery has only made his passion for running stronger, and he wanted to prove to everyone he was just as good. He has accomplished that and more. When he graduated high school in May 2023, he received the Optimist Award. This fall, Marquis will attend Texas Christian University on a full-track scholarship – a goal he set for himself at only 10 years old.

“Marquis decided in fourth grade that he would one day attend TCU,” Valerie said. “Jokingly, I expressed how his father and I did not budget for TCU. At the time, our fourth grader looked at me on the car ride to school and said, ‘Don't worry, I'm going to get a scholarship.’ His dreams came true.”

“The most gratifying part of my job is seeing kids through their illness to the other side,” Dr. Hansen said. “For Marquis, that means a life that is everything he has always wanted.”

Today, Cook Children’s Medical Center - Prosper launched a new testing and diagnostic service that allows children experiencing seizures to receive care closer to their homes and communities. The test, called continuous electroencephalogram (EEG) monitoring, reads electrical activity in the brain and is an essential tool for detecting and diagnosing a seizure disorder. 

Prosper resident and father of two, Kevin Greene knows all too well the challenges of having to leave your community to seek medical care and how that impacts a family. In February, the vice president and administrator at Cook Children’s – Prosper, Greene and his wife Christy, took their 9-month-old son Matthew to the emergency department at Cook Children’s Medical Center in Fort Worth after he experienced what appeared to be a seizure episode at their home.

The Greenes began to notice symptoms in Matthew a couple of weeks prior to the event and consulted with Damian Campbell, D.O., a Prosper-based pediatric neurologist and member of Cook Children’s Physician Network.

“We took Matthew to see Dr. Campbell, who is amazing, and we were watching his condition closely, but following this episode he encouraged us to go to our medical center in Fort Worth for further evaluation,” Greene said. “Our medical center in Prosper was open, but I knew we did not offer continuous EEG monitoring at the time and would not be able to provide the appropriate services to be able to monitor and capture what was happening. Upon arriving in Fort Worth, Matthew was examined in the emergency department where he was ultimately admitted to our epilepsy monitoring unit.”

The new EEG monitoring service at Cook Children’s – Prosper is made possible through collaboration with the Neurosciences team in Fort Worth, an effort that began months before Greene and his family had their own emergency.

Patients experiencing a potential seizure are admitted to the inpatient unit at Cook Children’s – Prosper where an EEG technician sets up mobile monitoring equipment and attaches monitoring electrodes to the patient’s scalp. The test livestreams to clinicians in the Epilepsy Monitoring Unit at Cook Children’s Medical Center in Fort Worth for observation and reading. The monitoring process typically takes at least 24 hours and requires an overnight stay in the hospital.

“This is a relationship that we've been working on for multiple months with the Jane and John Justin Institute for Mind Health team in Fort Worth led by M. Scott Perry, M.D., head of Neurosciences and Cynthia Keator, M.D., Medical Director of Neurology,” Greene said. “It is another great example of how the children and families we care for at Cook Children’s – Prosper will have the full weight and expertise of the entire health care system behind them.”

Several obstacles were overcome to make this remote monitoring service a reality, including building the technological infrastructure to support high-speed and secure data-sharing channels between the two medical centers for real-time monitoring of the patient’s EEG patterns and events.

“We’ve been fortunate to have the support of our main campus while we grow and bring various systems online,” said neurologist Damian Campbell, D.O. of Cook Children’s – Prosper. “Their support has offered us an opportunity to really plan out our own approach here in Prosper. We’re excited to now be able to provide continuous EEG monitoring to our community; another step forward toward our promise of delivering the highest quality care to every child in our care and communities.”

Communication and collaboration protocols between the monitoring team in Fort Worth and clinical team members in Prosper were established to coordinate care activities, share essential information and maintain seamless operations during the monitoring process. 

“The project's success can be attributed to the dedication, expertise, and commitment of professionals from Cook Children's in Prosper and Fort Worth working together to achieve a common goal of providing the best possible care for patients,” said Rickey Ross, manager of the Neurodiagnostics Lab at Cook Children’s – Fort Worth. “Not only does this enhance access to specialized care and more timely interventions for kids in Prosper and the surrounding communities, but it offers convenience and comfort for patients and families, promotes knowledge sharing and optimizes resource utilization, all of which ultimately improve patient outcomes and well-being.”

Monitoring technicians in Fort Worth and nurses in Prosper underwent comprehensive training and education to prepare to support the service. 

“Our nurses and clinical care team members at Cook Children’s – Prosper are excited to be able to care for patient’s needing this critical service,” said Sheralyn Hartline, RN, assistant vice president of nursing and patient care at Cook Children’s – Prosper. “Through the collaboration with our medical team in Fort Worth, we are forever changing the way families are able to access world-class pediatric neurological services close to home.”

“Our family is truly grateful for the amazing care and kindness that was provided to our son during our time in Fort Worth,” Greene said. “It brings me great joy knowing we are now able to extend the same high-quality care and experience to our families seeking care at Cook Children’s Medical Center – Prosper.”

generation and propagation of epilepsy activity in the context of this disorder as a network disease.  

His findings may help physicians to understand how epilepsy activity initiates in a focal brain area of children with epilepsy propagating later in other areas of the brain. Pinpointing these onset areas, where epilepsy starts, would allow stopping seizures through surgery in children suffering from epilepsy without resecting large brain areas, which may damage physiological brain functions such as the language or movements.

Pediatric epilepsy is a neurological disorder, characterized by recurrent seizures due to abnormal electrical brain activity. It has been estimated that 4-10 out of 100 children suffer from epilepsy. A significant proportion of these children are unable to control their seizures with drugs dealing with recurrent uncontrolled seizures. These seizures may have a severe impact on the quality of child’s life leading to serious cognitive and behavioral problems as well as increased mortality. Therefore, it is critical to develop new strategies for the diagnosis and treatment of uncontrolled seizures in children with epilepsy.

Children with uncontrolled seizures undergoing surgery often require the placement of electrodes inside their brain for identifying the area where seizures are generated. Yet, seizures occur spontaneously and propagate fast from onset areas to areas of spread. Thus, we should have to wait for a seizure to occur and this may take several days. This increases the patient’s time in the hospital and thus the risk of infection from invasive electrodes placed in the patient’s brain. 

In this study, Cook Children’s Neuroscience Research team showed that they can map the onset of this propagating activity without having to wait for a seizure to occur. Such a development can shorten the patient’s stay in the hospital improving the presurgical evaluation procedure.

This study is funded by the National Institute of Neurological Disorders and Stroke and is in collaboration with the University Campus Bio Medico in Rome, Italy, the Boston Children’s Hospital, and Harvard Medical School. Margherita Matarrese, a Ph.D. student of Bioengineering under the supervision of Professor Papadelis serves as first author of this scientific paper.

Coming Soon

The Jane and John Justin Institute for Mind Health at Cook Children’s, opening in October 2023, is bringing together nine specialties under one roof. Pediatric specialists in neurological, developmental and behavioral health are changing the way we deliver health care. Together, we’re healing minds and bodies and sharing smiles that warm the soul and connecting care for kids unlike anyone else.

To many parents and their children, M. Scott Perry, M.D., head of Neurosciences at the Jane and John Justin Institute for Mind Health at Cook Children’s, is best known as an epileptologist. One who isn’t afraid to take on rare and difficult childhood epilepsies. An active clinical researcher, always searching for therapies to treat and cure epilepsy. A relentless advocate for his patients.

But his more than 8,000 Twitter followers know him as @TheNotoriousEEG, and follow him for his take on everything from epilepsy research to food, music and art. Despite his healthy following, Dr. Perry was initially reluctant to join the Twitterverse.

“At some point, people within the hospital asked me if I would consider joining social media, and my immediate answer was, ‘No, thank you,’ because I have a lot of stuff to do, and I don't need to add another activity to my life,” Dr. Perry said. “But they seemed to think I would be good at it. So I decided if I could come up with a handle that was clever enough, then I will do it.”

Dr. Perry elicited naming ideas through a contest with his colleagues in the neuroscience department but none of the suggestions felt right. Then, it came to him: @TheNotoriousEEG, a play on his love for 90’s rap music and his favorite artist, The Notorious B.I.G., coupled with a nod to his work with the acronym of a common neurologic test called an electroencephalogram (EEG).

Dr. Perry’s Twitter feed is as diverse as his interests. Yes, you’ll find a lot of posts about epilepsy awareness and medical research, but you’ll also see pictures of the nightly meals he cooks for his family, his weekend cheer dad persona as he follows his youngest daughter to cheer competitions and his support of Texas Christian University where his oldest daughter is studying to be a nurse. Scroll a little more and you’ll pick up on his love for art, something he gets from his antique-loving mother with an eye for beautiful things.

His initial reluctancy to tweet has given way to cautious appreciation, and he’s proven himself a natural at harnessing the power of social media for good.

“Despite all of Twitter's problems, I think that it's a good platform for scientists, frankly, to share information very quickly,” he said. “It's a great way to connect with patients and other advocates quickly and to bring them information that maybe they don't have available to them or to gain knowledge from others that you didn’t have access to. It's a way to share expertise widely with people that might not have access to some of those things all the time. Beyond that, it's a good way to show people that doctors have lives and personalities and, to some degree, we are regular people, too.”

The Doctor from the Delta

Dr.  Perry’s road to becoming a world-class pediatric epileptologist began in the Mississippi Delta where he was born and raised in the small town of Cleveland, Mississippi — home to the Delta State Fighting Okra! His father owned and operated a used car company and his extended family operated lots throughout Mississippi. The family business is still in operation today with his two older siblings at the helm.

As a kid, Dr. Perry had his sights set on an occupation that would take him far beyond Mississippi into parts unknown. He wanted to conquer space exploration as an astronaut. But, during a stint at Space Camp, Dr. Perry was told his vision was not perfect and he would never be able to command a space shuttle. 

“If you know my personality, my goal is to be the commander,” Dr. Perry said. “So I had to step out of the astronaut business and try to look at something else. Being a doctor sounded challenging.”

During his junior and senior year of high school, Dr. Perry attended the Mississippi School for Mathematics and Science, followed by Emory University in Georgia for his undergraduate degree in physics. That’s also where he met his wife of 22 years, Becky.

Dr. Perry went back to his home state to earn his medical degree at the University of Mississippi School of Medicine. It was there that a neuroanatomy class stoked his desire to specialize in neurology. He said that, for him, the subject matter “just clicked.”

Following medical school, Dr. Perry returned to Emory University for his pediatrics and child neurology residencies. In 2008, he joined Nicklaus Children’s Hospital in Miami, Florida, to pursue a neurophysiology fellowship. 

Westbound to Cowtown

When his training was complete, Dr. Perry longed for a place to practice medicine free from the bureaucracy that came with medical facilities attached to teaching institutions. It just so happened that he came across an advertisement for Cook Children’s. He had never heard of the place but was impressed that the medical center had an epilepsy monitoring unit and was performing a healthy amount of epilepsy surgeries, which is where his interest lay.

“It just sounded like a decent opportunity, so I decided I'd come and give it a shot and see who these people were,” Dr. Perry said. “I came and interviewed here and I really loved the concept of what they were doing, and how these were essentially private practice neurologists. They were doing things that you would typically only see being done in an academic medical institution, but they were doing it here in a private children's hospital. This kind of pioneering spirit they had was really impressive to me.”

Thirteen years later, Dr. Perry has blazed a trail at Cook Children's as an expert in rare genetic epilepsies and epilepsy surgery.

“I find both of those things incredibly rewarding because I love to tackle very difficult cases and break them down, hopefully, to determine either where the seizures are coming from or why the seizures are occurring,” he said.

He’s admittedly the type of person that does not deal well with incremental change. It's either go big or go home. That’s why Dr. Perry loves epilepsy surgery. When a patient comes out of an operation seizure-free or with significantly reduced seizure activity for the first time in their lives, it’s an immediate payoff.

When an operation can’t cure a condition, like in the case of many genetic epilepsies, it is his patients and their families that keep him going. The tenacity of the families that deal with these conditions, and the relentless drive to help their children through advocacy, forming their own non-profits, and funding the research necessary to find cures, is all the inspiration he requires to do his job daily. Dr. Perry says that, thanks in large part to their efforts, we see the advent of new therapies and the potential of disease-modifying treatments to correct the underlying genetic cause of these rare conditions.

Collaborating for Mind Health

In addition to his patient load, Dr. Perry is overseeing the transformation of Cook Children’s divisions of neurosciences with the development of a unique and comprehensive care model for children with diseases of the nervous system. It’s known as The Jane and John Justin Institute for Mind Health at Cook Children’s. Nine specialties that commonly overlap in the treatment of nervous system disorders, and have traditionally been siloed in separate locations, are coming together under one roof to make care easier and more efficient for patients and families. The Justin Institute will also open the door of collaboration between physicians and other providers when it comes to shared patients.

“I'm excited about the potential convenience for families to be able to get everything done in one fail swoop. To park your car once, and to miss one day of work, and to get out of school one day and get everything you need,” Dr. Perry said. “Then on the backside, knowing that your doctors are all down the hallway from each other and can be face-to-face about your care and make sure everybody's on the same page.”

Dr. Perry has personally curated much of the neuro-focused art that will hang in the hallways of the Justin Institute, housed in the newly expanded Dodson Specialty Clinics building at Cook Children’s Medical Center. The pieces range from paintings of Fort Worth’s skyline to images of the brain made from glass. All of the art installations were created by artists with a connection to the neurosciences in an effort to make the topic of brain science more approachable.

“I'm very excited about the building because I have spent a lot of time obsessing about how it’s going to look and how it's going to work,” he said. “It'll be our version of Disney for neuroscience. A place you look forward to coming to for the care of your child.”

Getting the Justin Institute up and running hasn’t been easy, but for someone who wants the best possible care experience for patients, it’s been worth it. He says bringing this many specialties together in order to attain a collaborative network encourages change across all nine divisions. It has required a lot of listening and learning on his part in order to understand how specialties outside of his, like behavioral health or developmental pediatrics, run their clinics so that they can build a care model that is good for patients and providers.

Collaboration within the Justin Institute will also expand neuroscience research opportunities into behavioral health, developmental psychology, autism and other disorders associated with the nervous system.

Even though Cook Children’s is not a traditional academic medical center affiliated with a teaching institution where research is a cornerstone of the programming, the medical center has a robust research arm. It’s one of the things that attracted Dr. Perry to the health care system.

“We're the most academic, non-academic place in the country, in my opinion,” Dr. Perry said. “I think the way we're doing it is unique and offers more opportunity for access to patients for enrollment. I like to think it's a bit healthier research environment because the Dodson Neurosciences Research Endowment is there to cover the salaries of the research employees regardless of grant funding. And then, because we're not tied to any one institution, it allows us to collaborate with numerous institutions and have multiple partners.”

Dr. Perry was instrumental in launching the Dodson Neurosciences Research Endowment at Cook Children’s, which he says is a game changer in taking the medical center, already renowned for its clinical care, to one also recognized for ground-breaking research. It’s one of his proudest accomplishments so far.

Even with all of these big medical career moments, Dr. Perry has not forgotten his Mississippi Delta roots. Look no further than his Twitter feed for proof. Shrimp and grits, po-boys and gumbo — all home-cooked by one of the nation’s leading epileptologists, aka @TheNotoriousEEG.

“I'm just a simple man from Mississippi,” Dr. Perry said. “I just happen to be pretty decent at epilepsy.”

Sunday, March 26, 2023 is Epilepsy Awareness Day to spotlight this neurological condition that affects nearly 50 million people worldwide. People are encouraged to wear purple. 

By Ashley Antle

There was a time when constant seizures plagued 4-year-old Sofia Gutierrez-Lopez’s life. They started when she was 19 months old and gradually the seizures occurred hundreds of times within 24 hours. Day and night, Sofia’s brain misfired, stealing her ability to hit developmental milestones and live a normal life.

No amount or combination of seizure medication helped, which is common with Sofia’s type of epilepsy. Sofia has a severe malformation of the left side of her brain, and that’s where her seizures originated. Her parents were desperate for something — anything — that would free their daughter from the unrelenting seizures and allow her to have as normal a childhood as possible.

In April 2022, Sofia underwent surgery at Cook Children’s. Now a year later, her seizure activity is dramatically reduced and her quality of life dramatically improved. Since surgery, Sofia had one day where she experienced three seizures, compared to hundreds every day before surgery.

M. Scott Perry, M.D., an epileptologist and head of neurosciences at the Jane and John Justin Institute for Mind Health at Cook Children’s, was one of several doctors treating Sofia. He recommended a functional hemispherectomy — a surgery that removes or disconnects half of the brain to interrupt the seizures and stop their assault on the healthy side of the brain. In Sofia’s case, it would be the left side.

Like any surgery, it had its risks, but so did living with a brain under constant attack. Sofia already had developmental delays, and every seizure increased the potential for more. Eventually, the seizures could rob her of the ability to walk, talk and eat. Children with uncontrolled seizures also are at greater risk for sudden death during a seizure.

“In this case, the risk of surgery is weighed against the risk of her continuing to have seizures,” explained Daniel Hansen, M.D., a pediatric neurosurgeon specializing in epilepsy surgery and medical director of neuro-trauma at Cook Children’s Medical Center. “The reality is epilepsy surgery is really quite safe when done by a trained epilepsy surgeon or a pediatric neurosurgeon with epilepsy experience.

“The risk of catastrophic operative complications or unexpected postoperative complications is very low,” Hansen said. “Even knowing that there will likely be permanent changes to strength and vision on the opposite side of the body that are unavoidable, the trade-off to being seizure free is, for most children, completely worth it.”

Fateful Connection on Trip

Even so, having a portion of their child’s brain disconnected was a scary thought for Sofia’s parents.

“It's crazy to think that they could actually go in her brain, take part of her brain out and that is going to help her,” said Cristina Gutierrez-Lopez, Sofia’s mother. “It sounded like fiction.”

Sofia’s parents weren’t quite ready for that step until a trip to Mexico to visit family brought a turn of events that not only confirmed the surgery was necessary, but that Cook Children’s was the right place with the right doctors to have it done. While in Mexico, Sofia suffered a seizure emergency that sent her to the emergency department of a local hospital. A physician there seconded the diagnosis of Cook Children’s neurologists and explained that surgery was the only option for any relief.

Without knowing the family’s already established ties to Cook Children’s, the physician told them about a neurologist he knew in Texas to be one of the best in epilepsy treatment. He attended a presentation made by this Texas doctor at a medical conference. That Texas physician turned out to be Dr. Perry. The same Dr. Perry that Sofia had seen as a patient before her fateful trip to Mexico.

Sofia’s parents were stunned at the connection. Her mother said it was confirmation that God had orchestrated these events to bring them to a place of certainty and peace and that surgery was the right next step for Sofia.

When they returned to Fort Worth, the family met again with Dr. Perry and discussed the hemispherectomy. He introduced them to Dr. Hansen who would perform the procedure. Gutierrez-Lopez told the medical team they were ready.

“I am clear on all the risks,” she said. “I understand that this is the only thing that can possibly help my daughter.”

“When we went home from the hospital, I took home the same Sofia I brought to the hospital, but improved,” Gutierrez-Lopez said. “She had the same communication skills and the same physical ability. Everything was the same or better, plus no seizures.”

As complicated and risky as epilepsy surgery sounds, outcomes like Sofia’s are actually common.

“Sophia's case and her outcome is nothing short of astounding,” Dr. Hansen said. “I mean, we go from a girl who had literally hundreds of seizures a day to almost seizure-free. But although that outcome is amazing and astounding, it is the expected outcome. This is not a one-off sort of thing for children with epilepsy.”

Breaking Cultural Barriers

A perception that the brain is too complex to fix and, therefore, should not be touched is a common misconception, especially among minority groups, according to both Dr. Perry and Dr. Hansen. Dr. Perry is studying the disparities that exist within epilepsy treatment and, in particular, surgery. Many of those disparities are already well documented but a lot of cases use insurance databases to illustrate the fact that more white people have epilepsy surgery than non-white people, according to Dr. Perry. The problem with this approach, he says, is that it only looks at those who underwent surgery and not at those who were offered but declined.

“We say there's a disparity, which is true, but we don't know why there's a disparity,” Dr. Perry said. “Was it because they weren't offered the opportunity because maybe their insurance is not as good? Or their social situation isn't as good, or they were offered but declined for whatever reason?”

Dr. Perry’s study, which now has a database of more than 2,000 patients, examines the cases of those referred for epilepsy surgery and any differences between the work-up of each case. For example, do people of color have fewer medical tests and therefore are not revealed to be good candidates?

“It turns out that the work-ups are not really different based on race or ethnicity,” Dr. Perry said. “However, based on the data we’ve collected so far, people of color are almost four times more likely to decline the opportunity for surgery when offered.”

The question now becomes, why?  

“Insurance as a primary factor is not the whole story,” Dr. Perry said. “I think that's one limitation, but another limitation is there are cultural barriers to getting epilepsy surgery, and if we don't understand those barriers, then getting everybody the best insurance is not going to fix the problem. There's an aversion to this treatment approach and that's something we need to explore a little further because we need to learn what their concerns are so that we can address those barriers and make sure this opportunity is available to everybody.”

Tracy Vang, director of equity and inclusion at Cook Children’s, agrees. She cited the book “The Spirit Catches You and You Fall Down” by Anne Fadiman as an example of how cultural beliefs intersect with medicine when it comes to how some perceive illness, what causes it, and how it should be treated.

That’s why Gutierrez-Lopez shares her daughter’s story. 

She wants other parents, particularly those who share her Latino heritage, to know that while these are hard decisions to make, parents should consider the possibilities for their child over their own fear or perceptions of surgery.

“Fantasy” is the word she used to describe her initial impression of epilepsy surgery. After much of her own research, putting her faith in action, and trusting the capability of Sofia’s doctors, she pushed past her disbelief and fear. 

“As a mother, you never want to expose yourself to losing your children or anything bad happening to them, but when you have special needs children, the pain of seeing them suffer teaches you to be strong enough to take risks when you know there is hope for a better quality of life for them,” Lopez-Rosas said. “We trust in God Almighty and in the wisdom he has given to the doctors and put our little ones in their hands. They would never suggest surgery if they did not know that there is a great chance of success.”

We’re born with a mind, body and soul. When something goes wrong with one, it often affects another. That’s especially true for individuals with diseases of the nervous system — or the brain, spinal cord and nerves — and it’s the motivation for the development of the new Jane and John Justin Institute for Mind Health at Cook Children’s Medical Center – Fort Worth.

The Justin Institute will connect nine specialties under one roof, including Neurology, Neuropsychology, Neurosurgery, Pain Management, Physical Medicine and Rehabilitation, Psychology, Psychiatry, Developmental Pediatrics and Developmental Psychology. It is slated to open fall 2023 in Cook Children’s newly expanded Dodson Specialty Clinics building.

"All of the departments and specialties involved in creating the Justin Institute have worked really hard to break out of their silos and build a system that gives our patients a connected and well-coordinated experience," said Teresa Baker, assistant vice president, Primary and Specialty Services, for Cook Children's Physician Network. "I'm really thankful for their teamwork and focus on doing what is best for the child, and our patient families will be, too, as they see it first hand during their visits."

Physicians began building the Justin Institute’s framework several years ago after identifying a missing link within the Neurosciences program. There was little coordination among overlapping specialties that treat the mental, developmental and physical diseases and disorders associated with the nervous system.

“A majority of people with diseases of the nervous system have accompanying mental health or behavioral health disorders,” said M. Scott Perry, M.D., an epileptologist and head of Neurosciences at the Justin Institute. “For example, about 50% of people with epilepsy will also have mental health disorders such as depression and anxiety.”

That may be because epilepsy, depression and anxiety share the same abnormal brain connections, or because the stress of living with the unpredictability of the disorder leads to mental health struggles. Either way, it’s important to connect the dots of care for those with overlapping mental, intellectual, neurological and developmental health needs.

“Kids get better when all aspects of their health and wellbeing are addressed, including their medical and mental health needs,” said Kristen Pyrc, M.D., Cook Children’s co-medical director of Psychiatry. “Many of our patients are seen by Neurology and Developmental Pediatrics and Developmental Psychology specialties, so we are hoping that being part of one institute will help increase collaboration amongst the disciplines and get patients to the appropriate resources more quickly.”

Everything for the Child

The Justin Institute is built around a patient-centered experience, where everything for the care of a child’s nervous system is in one place and coordinated through one referral, assessment and intake process. It’s designed to eliminate barriers to care like multiple appointments with multiple providers in multiple locations and to simplify the patient’s access to their medical team. For children and their families, it will make a day of doctor’s appointments much easier. For medical providers, it enhances their ability to coordinate care.

“The Justin Institute will help us more readily identify and align services earlier in the treatment process and steer families in the right direction, or in many directions simultaneously,” said Lena Zettler, MA, LPA, director of the Cook Children’s Department of Psychology. “For our patients who have had traumatic childhood experiences, plus mental health issues and neurodevelopmental disorders such as ADHD, this will provide better collaboration and coordination of care, hopefully in a more timely manner.”

It’s about improving the system, Dr. Perry explained. One that can admittedly be hard for families to navigate at times.

“Dealing with a disorder of the nervous system is stressful enough,” he said. “Getting the appropriate care should not make it worse. You shouldn’t have to pack up your child and go to four different places when you need a neurologist, neurosurgeon, psychologist and autism services when we can bring these services that commonly co-mingle together under one umbrella.”

At the Justin Institute, neurologists, neurosurgeons, psychiatrists, psychologists and experts in developmental and behavioral health will work together in one location where walking across the hall to consult with one another on a patient’s case will be the norm. For the patient, that translates to better, more comprehensive and connected care.

“The opportunities for collaboration between specialty departments when they are under one roof has the potential to really enhance the care that we all provide our families,” said Matt Robison, director of Cook Children’s Child Study Center, where kids with complex developmental and behavioral health disorders receive diagnosis, treatment and education.

Child Study Center’s Developmental Pediatrics department and Psychology program will physically relocate to the Dodson Specialty Clinics building as part of the Justin Institute, as well as Cook Children’s psychologists who currently function in different departments on the main hospital campus.

The move undoubtedly brings mental health care front and center and illustrates Cook Children’s commitment to connected care, according to Zettler.

“Cook Children’s understands there is no health without mental health,” she said. “Psychologists, in particular, have much to offer in assessment and complex diagnostics, and I am looking forward to future collaboration, projects and research that will be mutually beneficial for all.”

Cook Children’s already has a robust Neurosciences Research Program, but the Justin Institute will open the door for more cross-specialty studies, much like one currently underway examining the impact of epilepsy on the psychological health of children. Chrystal Cooper, Ph.D., a principal investigator with the Justin Institute, is leading that study.

“Dr. Cooper’s work is an example of the type of collaborative research that is possible across the divisions of the Justin Institute,” Dr. Perry said. “Our plan is to take the successful research program we have in neurosciences with a focus on epilepsy, cerebral palsy and movement and grow that team to include teams dedicated to autism, pain, stroke and more.”

To learn more about the specialties represented at the Justin Institute for Mind Health, visit Instituteformindhealth.org. 

Cook Children’s Health Care System has successfully achieved six rankings in the U.S. News and World Report’s Best Children’s Hospital list for 2022-2023. This is an impressive and exciting jump from 2021 when Cook Children’s ranked nationally in two specialty categories.

The report, which was released today, uses clinical data to measure patient safety, infection prevention and adequacy of nurse staffing. Out of 284 children’s hospitals in the U.S., only 90 ranked in at least one of the 10 pediatric specialties evaluated. The following six Cook Children’s specialties were named among the top programs:

• Pediatric Neurology and Neurosurgery - #29 in the nation
• Pediatric Diabetes and Endocrinology - #38 in the nation
• Pediatric Orthopedics - #41 in the nation
• Pediatric Pulmonology and Lung Surgery - #43 in the nation
• Pediatric Cardiology and Heart Surgery - #48 in the nation
• Pediatric Cancer - #50 in the nation

“For more than 100 years, Cook Children’s has been committed to providing the best quality medical care for children. The U.S. News and World Report rankings further validate the tireless dedication of our staff to fulfill our Promise,” said Rick W. Merrill, President and CEO of Cook Children’s Health Care System. “As we look toward the future, with the opening of our new hospital in Prosper, Texas, later this year, we are excited to bring our world-class pediatric care to even more children and families.”

Out of the six specialties ranked on the Best Children’s Hospital list, all but one are currently operating at Cook Children’s Pediatric Specialties – Prosper. Neurology, endocrinology, pulmonology, cardiology and pediatric cancer services are all available for new patient appointments. 

March 28, 2022, was coming to a close like any other ordinary day for the Chavez-Llanas family. Salvador and Laura Chavez-Llanas were upstairs bathing their youngest of three sons while their older two were playing video games downstairs. In a moment, their peaceful evening turned to chaos.

A cry for help rang out from downstairs. It came from Salvador and Laura’s middle son. Laura rushed to check on him, only to find her oldest, 8-year-old Santiago, on the floor. He was conscious but drooling, and unable to stand up or move the right side of his body. His speech was slurred, and one side of his face drooped.

Laura yelled for her husband’s help. He called 911, and an ambulance arrived within minutes to carry Santiago to the emergency room at Cook Children’s Medical Center. That’s where Laura met Rachelle Herring, M.D., a pediatric neurologist.

“Santiago was pretty severely impaired at that point,” Dr. Herring explained. “He had a dense hemiplegia, which means he really could not move the right side of his body at all. He had very slurred speech but, fortunately, he was alert and understood everything that was going on.”

Santiago quickly underwent imaging tests of his brain. They confirmed he was having an ischemic stroke. This type of stroke occurs when a blood clot blocks the flow of blood in a vessel in the brain, cutting off oxygen and disrupting the function controlled by that part of the brain.

“We were just playing our Nintendo Switch and that's when my controller fell," Santiago said. "I started reaching for it, but then I felt like I was getting weak in my right side so I tried even harder to get it and then I fell down.”

Santi, as his mother calls him, has always been a healthy, active boy who loves playing basketball, swimming and riding bikes with his family. Never did his parents imagine their son would be one of the 5 in 100,000 kids each year ages 1 to 18 to have an ischemic stroke. It’s more common in newborns, occurring in 1 in 2,500 full-term infants during their first month of life.

“It was pretty shocking,” Laura said. “Your world as you know it just disappears. It’s so hard.”

Time was of the essence. With stroke, recovery is directly related to how quickly treatment can be administered. But stroke in children is so rare that the drug considered the gold standard treatment for adults has not been studied enough in kids to be approved for use in pediatric cases.

The medication, called tissue plasminogen activator, or tPA, breaks up blood clots. It’s administered through an IV with the goal of dissolving the clot within the vessel and restoring as much blood flow as possible. To be most effective in reversing damage, it must be administered within 4 ½ hours of the onset of stroke.

Although it’s considered off-label to use tPA to treat children, some hospitals administer the drug to pediatric patients after carefully weighing the risks and benefits.

“That’s what we did in the case of Santiago,” Dr. Herring said. “He came in very quickly after the stroke and I reached out to Dr. Marcela Torres, our hematologist who specializes in blood clots, about giving tPA. We’re basing our decision on the adult data that when the patient comes in within 4 ½ hours of an ischemic stroke, we think tPA can help restore the blood flow to the affected area of their brain."

Dr. Herring explained the risks of using the powerful drug to Santiago’s mom.

“At that point, I couldn’t say no,” Laura said. “Because I want to have my kid with me.”

It was the right call. Not long after receiving the drug Santiago’s symptoms began to resolve, and he was transferred from the ER to the pediatric ICU for monitoring. Because tPA is a blood thinner, patients are at risk of bleeding and must be watched closely. The stroke team also wanted to keep an eye on his neurological status and run tests to determine the cause of the stroke. 

Images of Santiago’s brain showed inflammation of the blood vessels consistent with a condition called cerebral vasculitis.

“We don't know exactly what caused the vasculitis,” Dr. Herring said. “It could have been triggered by some type of prior infection. The body's immune system goes a little haywire and can cause irritation to the blood vessels in the brain.”

Santiago spent two weeks in the hospital, all the while making a remarkable recovery. By the time he was discharged he had only a mild facial droop and had regained all of his movement. He’s on medications to reduce inflammation in his blood vessels, as well as blood thinner to prevent more strokes, and will undergo more imaging tests in the future to make sure his vessels return to normal.

In the meantime, Santiago is enjoying being back home with his family and back to school part time.

Looking back, Laura recalls a few things her son told her that may have signaled something was wrong. A few days before the stroke, Santiago complained of not feeling well. He said he felt like he was going to pass out at school. When questioned in detail by his mother, the only word he could find to describe how he felt was “weird.” Now, Laura wants parents to know to listen to their kids and know the signs and symptoms of stroke.

“Look at your kids,” she said. “Look at how they're behaving, and if they tell you something hurts, pay attention to every single different thing.”

To spot the signs of stroke, remember the acronym BE FAST:

Balance - Is there a sudden loss of balance or coordination?

Eyes - Is there blurred or lost vision?

Face - Is one side of the face drooping or numb?

Arm - Is there weakness, numbness or loss of movement in the arms, legs or one side of the body?

Speech - Is speech slurred?

Time - If you or someone you know is experiencing any of these symptoms, call 911 immediately.

“The most important thing to know is that stroke happens in children,” Dr. Herring said. “If something is different or off about your kid, do not discount it. It could be something serious. Seek emergency care because even if they're not a candidate for tPA, sometimes they're a candidate for other therapies. The sooner we're able to see that patient and try to figure out what's causing the stroke, the better off their prognosis is going to be.”

A pediatric neurologist from Cook Children’s is now seeing patients in Lubbock thanks to a hospital partnership that brings specialized care closer to home for residents of West Texas and eastern New Mexico.

Jennifer Pitts, M.D., joined the Jane and John Justin Neurosciences Center at Cook Children’s in December and opened her full-time practice at Covenant Children’s facilities shortly thereafter.  She provides care for every type of general neurology need in babies, children and adolescents. Those needs include treatment for seizures, stroke, medical aspects of head injuries and brain tumors, cerebral palsy, nerve-muscle disorders, movement disorders, neurofibromatosis and headaches.

The arrival of Dr. Pitts in Lubbock is the latest expansion of a collaboration between Cook Children’s Health Care System and Covenant Children’s that dates back to 2018. That partnership began with cardiothoracic teams from Cook Children’s who travel from Fort Worth to Lubbock four times a year to perform surgery at Covenant Children’s. The partnership grew when two neurologists from Cook Children’s began scheduling visits to Covenant Children’s for specialty areas of epilepsy and movement disorders.  And now, the collaboration has grown again to include full-time neurology.

What makes this news such a welcome development? Pediatricians in the Lubbock region can refer patients to Dr. Pitts’ local clinic; she is also available to assist with in-patient consultations at Covenant Children’s. The arrangement is designed to be sustainable, convenient and accessible for families on the South Plains who would otherwise have to go long distances to reach the nationally recognized neurosciences expertise that Cook Children’s offers.

“The issue is the demand and need in West Texas in general,” said M. Scott Perry, M.D., head of Neurosciences at Cook Children’s. “There are plenty of people who need pediatric neurology specialty services and pediatric neurology subspecialty services, and many of them end up coming to Fort Worth. I hope it provides the ability for kids who need care in the hospital to get it locally without having to be transferred as often.”

Rolla Shbarou, M.D., a Cook Children’s pediatric neurologist, began seeing patients in Amarillo early last year and will serve as another partner to Dr. Pitts for covering patient care. Opening the Lubbock satellite office for Cook Children’s neurology in late 2021 was a long process of working out logistics. “We’ve been talking about this for years,” Dr. Perry said.

Covenant Children's Chief Medical Officer David Gray, D.O., expressed excitement about the new arrangement.  "Dr. Pitts is the latest example of our collaboration with Cook Children’s in bringing broader specialty care to Lubbock so that more and more patients can receive excellent care close to home," Dr. Gray said.

Dr. Pitts expects to stay busy. She sees a benefit for the West Texas families who can now avoid long drives and missed days of work when their child needs a neurology appointment or in-patient care. 

Ask Dr. Pitts about her background, and you’ll find out her passions: animals, equine therapy and most of all, helping kids. She was born in rural Shattuck, Okla., on the far west side of the state, and raised in small towns in South Carolina and Florida. Surrounded as a child by pet dogs, cats, fish and a horse, she wanted to become a veterinarian or engineer when she grew up. Her love for horses led her to give therapeutic riding lessons to children with autism, cerebral palsy and other health challenges. During those therapeutic riding sessions, she was especially aware of the children who were struggling to get their medical needs diagnosed and find specialty care. 

Dr. Pitts strengthened her interest in medicine in college when she worked on research in pediatrics in the physiologic psychology department studying topics like executive function.

Dr. Pitts went on to earn her medical degree at the University of Oklahoma College of Medicine. She was torn between practicing pediatrics and geriatrics, but children won her heart because of their resilient attitude. She completed her combined pediatric and neurology residency at Mayo Clinic, in Rochester, Minn., in 2013.

Neurologists treat the nervous system -- spinal cord, nerves and muscles -- in addition to the brain, Dr. Pitts pointed out. In her Lubbock office she expects to encounter general neurology issues, “anything from cognitive concerns, developmental delay, seizures and headaches.”

A few other things to know about Dr. Pitts… she’s a good listener, a hiking enthusiast, and goes to the park whenever possible with her Bernese mountain dog, Bailey. Her move from the Houston suburbs to Lubbock brings her closer to her roots. She wants the parents of her patients to know they have a team here to help them take care of their children’s needs.

Dr. Pitts works from 4102 24^th Street, Suite 201, in Lubbock. To make an appointment, call 682-885-2500.

Her head and arms lift. Her eyes open, turned to the side. Her body goes rigid and she loses bladder control. Sometimes she moans; usually the episodes unfold silently. The next morning, Baylie doesn’t remember.

“It’s very tough to watch. It never gets easy,” said her mom, Brandi Williams. “Most of the time she just goes right back to sleep.”

Diagnosed at age 2, Baylie is among the estimated 12% of epilepsy patients who experience nighttime seizures. An electroencephalogram (EEG) monitoring study at the epilepsy monitoring unit at Cook Children’s found that her seizures occur six or seven times a night – even more than her parents initially realized.

Dave Shahani, M.D. an epileptologist at Cook Children’s, explained that nocturnal seizures cause special concern because of the risk that a child in the throes of a seizure might suffocate on fluffy bedding or soft toys.

“It’s not that they are any different from other seizures per se, but we tend to worry about them more,” he said. Seizures while sleeping are less likely to be witnessed by a parent, he pointed out. “Any number of nighttime seizures is a high number. Even one a month is a high number.”

The U.S. Centers for Disease Control and Prevention estimates that epilepsy affects 470,000 children nationwide. Irregular electrical activity in the brain characterizes this chronic disorder, which encompasses a wide variety of seizure types. Symptoms of epilepsy can look very different in different people.

Someone having a seizure might collapse, twitch or spasm, stiffen, blink rapidly, stare into space, or lose consciousness, for instance. Nighttime seizures pose the added challenge of sleep disruption. And studies have indicated a higher risk for Sudden Unexplained Death in Epilepsy (SUDEP) during sleep. SUDEP is poorly understood but suspected to involve cardiac or respiratory complications.

Damian Campbell, D.O., a pediatric neurologist at Cook Children’s in Prosper, said Baylie’s frequent and consistent sleeping seizures stand out as “the most extreme case that I’ve experienced.” But he’s encouraged about the prospect of surgically correcting her epilepsy.

Doctors think Baylie’s seizures originate from the right frontal lobe of her brain due to focal cortical dysplasia, a jumbling of the neuron cells as her brain developed before birth. In a surgical procedure scheduled for December, her medical team expects to pinpoint the abnormality’s exact location – and eventually reduce or even eliminate her seizures.

In November, as we observe Epilepsy Awareness Month, we’re highlighting Baylie’s story to illustrate the complexity of epilepsy, to share hope, and to feature the work of the neuroscience experts at Cook Children’s who treat the seizure disorders of more than 13,000 infants and children per year.

Starting to Find Answers

Baylie loves to sing and dance, can charm someone she’s just met, and in her dad’s words “she’s amazing.” So the discovery of their daughter’s epilepsy several years ago came as a surprise to Jabyrie and Brandi Williams. None of Baylie’s five older siblings has seizures. And judging just by appearance, nothing seemed obviously wrong with the toddler. But the family noticed occasional behavior they thought was unusual.

“Every once in awhile she would start staring off into space and then she would start laughing after she came out of it. We would snap our fingers and say ‘Baylie, Baylie,’ and she wouldn’t say anything,” Brandi remembered.

“We didn’t think it was anything at first,” Jabyrie said. “She would look around and laugh, and we thought it was an imaginary friend.” The Williamses referred to these strange bouts as “blanks.”

Then they picked up on other odd signs… dizziness after her blank spells, dark circles under her eyes, and complaints about feeling tired. She struggled to follow directions at home. At gymnastics, she couldn’t focus enough to complete a step-by-step routine. “Walk the beam and then do a handstand and do a cartwheel,” Brandi said. “Her mind couldn’t process doing those things in a certain order.”

After a referral from a pediatrician, Baylie underwent various tests, including an MRI, spinal tap and electroencephalogram (EEG). In 2020, they sought a second opinion at Cook Children’s, where she came under the care of both Dr. Campbell and Dr. Shahani. Further tests showed that Baylie’s “blanks” and trouble concentrating stemmed from the disorganized layering of neuron cells on one side of her brain.

She currently takes two medications that mostly control her daytime seizures. At full-day pre-kindergarten she hasn’t had a seizure during nap time. But a stressful day, Brandi said, might trigger a breakthrough seizure during waking hours. And the nighttime seizures still happen regularly.

“Focal cortical dysplasia can’t be fixed with medication. You can suppress the seizures, but you can’t fix anything. Surgery is the only thing that could fix it,” Brandi said.

Dr. Campbell characterized Baylie as a good candidate for epilepsy surgery. He cited the higher likelihood for surgical success in patients whose seizures onset lies in just one part of the brain instead of generalized in multiple locations. A lesion in Baylie’s right frontal lobe “as a result of being malformed, doesn’t respect the organization of how the brain produces electricity,” Dr. Campbell said.

Dr. Shahani concurred in the assessment of good surgical potential. The four medications Baylie tried didn’t stop her nighttime seizures. “Without any change in her treatment plan, she will continue to have seizures for the rest of her life,” he said.

Next Step, Surgery

A stereo EEG is scheduled for Dec. 13 to precisely locate the source of her brain’s erratic electrical waves. Daniel Hansen, M.D. a neurosurgeon at Cook Children’s Jane and John Justin Neurosciences Center, will make small incisions in Baylie’s scalp and skull to place electrodes that record brain activity. Those findings could pave the way next spring for either a resection (removal of a small amount of brain tissue), or thermal ablation (a probe that uses heat to destroy the area causing the seizures). Cook Children’s performs more than 30-40 epilepsy surgeries annually.

The odds are stacked in Baylie’s favor. Dr. Shahani explained that the target area doesn’t involve critical parts of her brain that control language or motor skills. ‘She presents as a very good candidate for potential seizure freedom,” he said.

For epilepsy patients whose seizures resist medication, alternatives may include dietary therapy or implanted devices such as vagus nerve stimulators. “Early recognition and early treatment always lead to better outcomes,” Dr. Shahani said. And from Dr. Campbell: “Earlier detection is better because uncontrolled seizures can affect patients cognitively over time.”

They encouraged parents to call a neurologist or epileptologist if they suspect their child might be having seizures. Videos can be a valuable diagnostic tool by capturing a recording of the twitches, spasms, “blanks” or other concerns. Seizures often go undiagnosed or misdiagnosed because they occur so uniquely in each person, Dr. Shahani said.

“It makes it challenging and extremely rewarding to help identify what is a seizure. Parents are our greatest asset because they know their child the best.”

Back in Rowlett, the Williams family keeps Baylie on an evening routine – dinner, medicine, bath, in bed by 8:30pm. She sleeps in her own bedroom, protected from falls by a guard around the edge of her mattress. A camera keeps watch.

“In the beginning she was sleeping with us because we were nervous,’ her mom said. “But we had to come to the agreement that we have to let her be a kid.”

Brandi and Jabyrie expressed optimism that this treatment path will put an end to Baylie’s nighttime seizures and allow her to come off the meds. They urged other parents to know that the signs of epilepsy can be silent and easy to miss. Their advice? Ask questions, pay attention to seizure triggers, and advocate for your child.

“We want answers, and that’s what Cook Children’s gave us,” Brandi said. “If we never would have switched to Cook Children’s we just would have been chasing seizures for the rest of her life. We want her to be as independent and normal as possible. Being on medication for the rest of her life just wasn’t what we wanted for her, if we could help it.”

Left uncontrolled over time, seizures can cause buildup of brain scarring, Dr. Campbell said. Nighttime occurrence interferes with the restorative sleep that children need. He applauded the Williamses for their willingness to follow the recommendation for surgery to stop Baylie’s seizures.

“I hope that this article helps other families with the expected fear with hearing someone say ‘Maybe we should consider surgery,’” Dr. Campbell said. “It is a scary conversation. But I think over time we’ve become optimistic that her story will make it less anxiety-producing for families.”

Luke Waggoner’s epileptic seizures were getting worse, striking multiple times a day sometimes in back-to-back clusters that sent him to the hospital.

The seizures caused the 13-year-old Arlington boy to jerk or jump uncontrollably. He might fall backward so forcefully that he’d bruise. Other seizures left Luke mute, confused and unresponsive.

Luke has Lennox-Gastaut syndrome, a rare type of difficult-to-control epilepsy. His seizures are medically refractory, having failed many medications and other non-pharmacological treatments. Even with five different medications taken multiple times daily, Luke was still having breakthrough seizures, the erratic misfires between neurons in his brain. These seizures often resulted in trips to the emergency room and frequent admissions to the hospital. The medications and frequent seizures also make it hard for him to think and speak clearly.

In the midst of the setbacks came a new treatment option: deep brain stimulation (DBS), a surgical therapy that utilizes electrodes, wires and a generator to modulate the brain’s abnormal electrical impulses. The movement disorders team at Cook Children’s has been utilizing DBS for dystonia since 2007 in children as young as 7 years of age. DBS was recently approved by the U.S. Food and Drug Administration (FDA) for adult patients with Lennox-Gastaut syndrome.

Luke was facing the prospect of undergoing a corpus callosotomy for his epilepsy. This irreversible neurosurgical procedure permanently severs most of the connections between the two halves of the brain to prevent drop seizures, the most dangerous and disabling seizures often seen in Lennox-Gastaut syndrome. Based on extensive experience with pediatric DBS, the movement disorders and epilepsy teams collaborated with Luke’s family about the potential to offer DBS to Luke as an alternative to callosotomy. If the DBS did not work, callosotomy remained an option.

After much discussion and planning, the decision was made to implant temporary electrodes in two sites on each side of Luke’s brain to assess the impact on his seizures and potential unwanted effects. In April 2021, Luke had the temporary leads implanted. After several days of continuous monitoring on the specialized epilepsy unit trying different stimulation settings, the sites for permanent leads were chosen in consultation with Luke and his family. A detailed proposal including the data from the trial was used to get insurance approval for the placement of DBS.

Then in a two-part landmark surgery at Cook Children’s – on July 8, when electrodes were implanted in his thalamus; and on July 14, when wires were placed through his neck to the generator in his abdomen – Luke became the first pediatric patient in North Texas to undergo DBS for intractable epilepsy since the FDA approved the treatment. He also became the first child in the United States to receive the newly approved sensing lead technology DBS system for epilepsy.

“Deciding to do the DBS and for Luke to be the first pediatric patient at Cook Children’s for epilepsy was a very difficult decision, and we did not take it lightly,” said his mom, Ami Waggoner. “We just knew we had to do something to try to help him.”

Cynthia Keator, M.D., medical director of the Epilepsy Monitoring Unit at Cook Children’s, expressed optimism about the potential to mitigate Luke’s seizures for years ahead. Other desired outcomes from the ongoing brain stimulation? Better cognitive function, fewer meds, greater independence and a more normal lifestyle.

“Our hope is that not only will this immediately start to show improvement in his seizures, but give him a chance to have a better quality of life, to be able to go back to school in person, to be able to go outside and not worry about falling down or having a seizure, and to be able to taper off of some of his medications,” Dr. Keator said.

Since 2007, the Jane and John Justin Neurosciences Center at Cook Children’s has established a record of excellence in deep brain stimulation, providing the surgical therapy to almost 150 patients with a movement disorder called dystonia, currently FDA-approved for pediatrics. Luke is a pioneer in DBS because of his underlying condition … epilepsy rather than dystonia.

Ami and Tim Waggoner said their son already has made big strides since his two DBS surgeries in July. His seizure count is down from the pre-surgery norm of five to 10 per day, she said, and the seizures that still occur aren’t the dangerous variety that requires additional “rescue” medications. Luke is able to read, play with Legos and his beloved trains, and go for short trips in the car. He started weaning off one of the drugs he takes. They are amazed at the change.

“You can just look in his eyes and see he’s more with it,’’ Ami said. “This is all really, really exciting. He knows he’s feeling better.” And from Tim: “I’m seeing more energy, fewer seizures, clearer speech and he is able to do more! It is just amazing the difference in just over a month since turning on the generator.” 

Let’s take a closer look at epilepsy facts, the precision involved in deep brain stimulation, and the route Luke took to becoming the first patient to undergo this new treatment for childhood epilepsy.

Epilepsy explained

The U.S. Centers for Disease Control and Prevention estimates that 3.4 million people nationwide have epilepsy, including 470,000 children. There is no cure and often no identifiable cause. Epilepsy is a chronic disorder that results from sudden intense bursts of electrical activity in the brain, manifesting a range of seizure types.

Someone who’s having a seizure might collapse, twitch or spasm, stiffen, blink rapidly, stare blankly, or lose consciousness depending on the type. Medication successfully controls the seizures in up to 80% of children with epilepsy.

Luke was diagnosed at age 5 with generalized epilepsy, which affects both hemispheres of his brain. Big sister Lexi didn’t know what was happening when she witnessed the first seizure.

“He couldn't hear me. And he started walking in a circle and then he just fell over and turned blue. Seeing that freaked me out,” Lexi remembered. “The first few years were really hard for me to understand and get used to it. But now it's to the point where it's just a part of our everyday lives.”

Medications helped at first, his mom said, but the seizures started getting more dangerous and debilitating about three years ago. Ami, who is a nurse, could administer the rescue medications at home when the seizures got especially bad. But even then, about twice a month Luke required hospitalization and intravenous therapies to stop the back-to-back clusters.

Dr. Keator said electroencephalography on Luke found the two distinct patterns of brain waves indicative of Lennox-Gastaut syndrome, (slow spike-and-wave complex and generalized paroxysmal fast activity). A vagus nerve stimulator, which uses a pacemaker-like device implanted in his chest, worked for a while for Luke, but the seizures and hospital stays kept recurring.

Game-changer potential

Warren Marks, M.D., director of the Movement Disorders Program at Cook Children’s, visited Luke’s hospital room in February 2021. Dr. Marks mentioned the prospect of deep brain stimulation, which had been approved by the FDA since 2018 for adults with epilepsy. Cook Children’s anticipated that the FDA’s green light for DBS in epileptic children was on the horizon, and Luke seemed like an ideal candidate.

“Dr. Marks just really believed he could help Luke. He gave us a spark of hope,” Ami recounted. “We were on board from the beginning because of the trust I have in the physicians and Luke’s neurology team. They're just amazing. They have never, ever given up.”

Dr. Marks explained that DBS sends small electrical impulses to targeted areas of the brain to alter the abnormal movements seen in dystonia as well as tremors and Parkinson’s disease. Results were encouraging in the almost 150 dystonia patients who underwent DBS in the past 14 years at Cook Children’s. Dr. Marks thought the therapy held promise for epilepsy patients too.

Collaboration between the Cook Children’s movement disorders and epilepsy teams had already been underway to adapt technology and share expertise, Dr. Marks said. The next step was a weeklong trial in April to gather data on Luke’s tolerance for different electrical amplitudes. And the doctors needed to know exactly where to implant the DBS devices.

“We recorded and stimulated different places in the brain to decide which seemed to be beneficial, but also which didn’t cause him unwanted side effects,” Dr. Marks said. “When you stimulate the brain in these areas, sometimes you get thing that you don’t want. We were trying to find one target that would give us the best chance of success.”

Not only did the April testing phase produce essential data, but during that practice run Luke spoke more clearly and felt better than he had in years, his mom said. So DBS was scheduled for July. John Honeycutt, M.D., medical director of Neurosurgery at Cook Children’s, is the surgeon who implanted several electrodes bilaterally in the centromedian nucleus of Luke’s thalamus, the relay center for transmitting signals in the brain.

The DBS system consists of three main components:

• Leads (pronounced “leeds”) – tiny electrodes embedded deep in the brain to deliver the electricity directly to the target area. They’re held in place by caps screwed into the skull.
• Generator – a mini-computer under the skin of the chest (the abdomen, in Luke’s case). Wires run through the neck to connect leads to a generator. In some cases, the battery is rechargeable.
• Programmer – a tablet that talks to the generator, regulating the strength and frequency of electrical impulses per second. Settings can be adjusted based on the patient’s response via Bluetooth connection.

Dr. Marks described DBS as flexible, specific and less invasive than other surgical approaches. Primary candidates are the patients like Luke whose seizures originate in both halves of the brain. “This has the potential to be an absolute game-changer,” Dr. Marks said. “It’s essentially like delivering medication without all the medication side effects. That’s one way to think about this. We are directly targeting the area of interest without bathing the rest of the brain with unwanted chemicals.”

The mechanism of action of a DBS in epilepsy is not fully understood. Scientific studies have supported that certain thalamic nuclei of the brain, specifically the centromedian nuclei, are generators of the slow spike-and-wave complex and paroxysmal generalized fast activity seen in patients with Lennox-Gastaut syndrome. Studies have shown favorable seizure reduction over time possibly through modulation of network excitability through stimulation of the centromedian nucleus of the thalamus. 

Doctors will continue to monitor Luke and adjust his settings as needed. Dr. Keator hailed Luke’s patience, good humor and cooperative attitude. He considers the medical team at Cook Children’s his best friends and ploy for pranks. “He’s just a trooper, and he lets us try new things with him, which we appreciate,” Dr. Keator said. “He’s just ready to get his life going, and he’s motivated. And that makes our job a lot easier.”

Luke said having epilepsy “can be a little tough at times.” He’s glad for all the care he received at Cook Children’s and the chance to potentially pave the way for DBS in other children who have seizures. “And I hope I get better so I can go on beach vacations and go places to ride lots of trains,” he said.

His parents look forward to the possibility of Luke’s epilepsy improving to the point that he can go to school, sleep over at a friend’s house, or travel without having a seizure. They are cautiously optimistic that deep brain stimulation will provide long-term relief for Luke and other children with epilepsy. And they hold out hope that this latest twist in Luke’s journey can blaze a trail for wider options in epilepsy care.

“I prayed a lot about it. I believe a lot of things happen for a reason. I think Luke is here to show us a story, to teach us something, to show us how brave he is,” Ami said. “His attitude the whole time has been basically ‘I just want to do this to help other kids.’”

This week, SMA was added to the list of required medical screenings every baby in Texas receives at birth. Without newborn screening, many children escape the diagnosis until after permanent neuromuscular damage has occurred. Early recognition and treatment can mean the difference between a near normal life or suffering many skeletal deformities and being unable to breathe without a ventilator.

“The hope and thought is that it’s going to change the lives of patients and their families,” said Stephanie Acord, M.D., a pediatric neurologist at Cook Children’s Jane and John Justin Neurosciences Center. “Without treatment, these kids and those who care for them are significantly affected. The earlier you treat SMA, the better.”

Dr. Acord directs the multi-disciplinary Muscular Dystrophy Association (MDA) Clinic at Cook Children’s Jane and John Justin Neurosciences Center where she and Warren Marks, M.D., medical director of movement disorders at the center, specialize in the treatment of SMA. Dr. Acord shared with us what every expectant parent needs to know about the disorder and the new screening.

What is SMA?

Spinal muscular atrophy is a genetic disorder in which the nerves within the brainstem and spinal cord, called alpha motor neurons, break down and lose the ability to send signals from the brain to the muscles. Without those signals, the muscles grow weak from lack of use and affect a baby’s ability to hold up and control their head, sit up, crawl, walk, swallow, speak and breathe. If left untreated, a child will eventually require a feeding tube and respiratory support, such as a ventilator. Untreated SMA is among the leading genetic causes of death of infants and children.

Spinal muscular atrophy can be passed to a child if both parents have a defective survival motor neuron (SMN1) gene. Parents do not have the disease or signs of the disease and do not usually know they carry an abnormal gene. The faulty gene inhibits the production of the protein that fuels these motor neurons and, without it, the nerves die. There is a back-up copy, the SMN2 gene, but it doesn’t work as well as the SMN1 gene.

It is estimated that about 100 children born in Texas each year have the disorder. Many newborns will not show signs of SMA for months. By that point, you’ve lost valuable treatment time and the ability to give a child with SMA a better life. This is why newborn screening is so important.

What are the signs and symptoms of SMA?

There are several types of SMA. Symptoms vary depending on the type, but muscle weakness and loss of muscle control that worsens over time are general indicators. It is important to note that SMA is not a cognitive disorder, but a muscular one.

Type 0 is the rarest and most severe form of the disorder. Babies born with Type 0 SMA have breathing problems very early on and typically do not survive.

Type 1 is the most common form of SMA and also considered severe. Infants will begin showing signs within six months of life. They have poor head control, do not sit independently and have difficulty sucking and swallowing. Even though cognitively they are normal, they are unable to speak due to weakness. Without treatment they will need assistance with breathing, feeding, communicating, sitting and walking.

Type 2 is a more moderate form of the disorder. Symptoms will become evident between six months to 18 months of life. Infants with Type 2 will have difficulty controlling their lower limbs. They may be able to sit independently and crawl but struggle with walking.

Type 3 is a milder form of SMA. Symptoms such as mild muscle weakness, difficulty walking or frequent respiratory illness do not appear until after 18 months of life. Some people may not experience symptoms until early adulthood.

Type 4 is adult onset SMA. It is rare, progresses slowly and typically doesn’t appear until the early 30s. Most people maintain mobility and are able to manage their symptoms without much interruption to their lives.

What treatment is available for SMA?

The Federal Drug Administration (FDA) approved the first treatment for SMA in December 2016.

“It used to be a diagnosis in which there was very little treatment and little hope,” Dr. Acord said. “But the treatment now available for these patients is completely different than it was just five years ago.”

Today, there are three FDA-approved treatments. Two—Spinraza® and Evrysdi®—are drug therapies that help stimulate the production of a more functional SMN2 protein. These two treatments require ongoing administration of medication at various time intervals. The other—Zolgensma®—is a gene-replacement therapy administered once through an IV that delivers a normal functioning copy of the SMN1 gene.

The treatments have varying degrees of success, but all work to improve the strength and function of muscles through healthier neurons. They help to prolong the amount of time a child can live without additional support such as equipment to help them breathe more comfortably, as well as increase their ability to speak with their voice and to eat to some extent by mouth. The most critical part of any of the treatments is to start as soon as possible following diagnosis.

How will my newborn be screened for SMA?

As of June 1, 2021, SMA is one of 55 disorders on Texas’ list of required newborn screenings. It does not require an additional test. It’s simply bundled into the routine screenings already performed on newborns via blood test.

What happens if my newborn is positive for SMA?

If your newborn’s screening is positive for SMA, it is recommended that they be referred to a specialist, either a pediatric neurologist or a pediatric neurogeneticist, within 24 hours. A specialist will most likely conduct additional testing to confirm the diagnosis and develop a treatment plan.

“Parents need to advocate getting their child to a specialist as quickly as possible,” Dr. Acord said about receiving a positive SMA screen.

Pediatric neurologists at Cook Children’s Medical Center are able to conduct many SMA assessments virtually via telemedicine, which is helping parents and children in parts of the state without specialists get access to care from the comfort of their home.

Click here to learn more about the Muscular Dystrophy Association Clinic at Cook Children’s and for contact information for clinic staff.

To help raise awareness of this rare genetic cause of neurodevelopmental disease, M. Scott Perry, M.D., medical director of neurology and director of the Genetic Epilepsy Clinic at Cook Children’s, shares basic information about the disorder and exciting advancements towards treatment for this rare disease.

What do SCN2A-related disorders look like? 

Children with genetic variants in SCN2A can develop early onset epilepsy with various levels of severity. SCN2A was first discovered as a cause of benign familial neonatal infantile seizures (BFNIS), a syndrome that often occurs in multiple family members. These children can develop seizures as newborns or infants, but can develop normally with good seizure control.

Later, SCN2A was discovered as a cause of infantile spasms and other early onset severe epilepsies of childhood, the so-called early infantile epileptic encephalopathies. The gene has also been linked to Ohtahara Syndrome, Dravet Syndrome, Migrating Partial Epilepsy of Infancy and West Syndrome amongst others. In addition, SCN2A variants are a major cause of intellectual disability, schizophrenia, and autism which may occur without associated epilepsy. A variety of other medical conditions may be present in people with SCN2A-related disorders, including sleep problems, cerebral palsy, and movement disorders to name a few.

What is the cause of SCN2A-related disorders? 

SCN2A is a gene which makes a sodium channel found primarily in the nerve cells that generate electricity. Two issues can occur with SCN2A. The first is a change in the gene which causes a gain of function – a change that allows too much sodium to enter the nerve cell and thus increases electricity – often presenting with epilepsy as a main symptom.

For others, SCN2A variants cause a loss of function – a change that decreases sodium entering the nerve cell and thus decreases electricity – more often presenting with autism and intellectual disabilities. Many mutations in SCN2A are de novo, meaning they occur spontaneously and were not inherited from the parents. This is often the case in more severe disease presentations. There are instances where SCN2A may be inherited from a parent and this is more commonly seen in benign presentations such as BFNIS.

How are SCN2A mutations diagnosed? 

Often, genetic testing can diagnose SCN2A mutations. The Behind The Seizure program provides free testing for children in the U.S. under the age of 8 years.

Magnetic resonance imaging (MRI) scans are often normal and electroencephalogram (EEG) findings may vary.

Is there a treatment for SCN2A-related disorders? 

While there is not yet a cure for SCN2A-related disorders, a significant amount of research is leading to exciting new therapies. Certain traditional sodium channel seizure drugs (for example, phenytoin, lamotrigine) have demonstrated more favorable responses for seizure control in select patients (often gain of function), while in others, sodium channel drugs may aggravate seizures.

New drugs are being developed that specifically target the abnormal channel produced by SCN2A. These treatments may provide more precise control of the channel without disrupting the function of other sodium channels like many traditional sodium channel seizure drugs. This may result in better seizure control with less side effects.

Potentially most exciting is the development of genetic approaches to therapy, treatments that don’t just treat symptoms, but aim to correct the genetic abnormality. Antisense oligonucleotides (ASO) are small pieces of genetic material that can be given to help increase or decrease production of SCN2A. This approach has been used in other genetic conditions (spinal muscular atrophy and Dravet syndrome) with success and represents a promising therapy for SCN2A disorders as well. This is just one of several genetic approaches to therapy on the horizon.

Where can you find more information about SCN2A disorders?

For more information about SCN2A and SCN2A Awareness Day, visit www.scn2a.org. The SCN2A Foundation serves as an excellent resource for information about SCN2A related disorders and helps connect a community of people living with these rare conditions. The site provides updates on SCN2A research as well.

“The impact of this treatment device can be pretty dramatic for the lives of the people who are candidates for it,” said Daniel Hansen, M.D., a neurosurgeon at Cook Children’s Jane and John Justin Neurosciences Center. “These are children who, five years ago, we would have told them there’s really nothing else we have to offer and you’re going to have to continue with medications for the rest of your life and manage it as best you can.”

The RNS System is a small, titanium neurostimulator about one-third the size of a credit card. During implantation, surgeons remove a portion of the skull in the same shape and size as the device and place the stimulator flush with the skull. It sits virtually undetected under the skin. Tiny wires, or leads, connected to the device are placed into the area of the patient’s brain shown to be the origin of their seizures. The RNS System monitors electrical activity in the brain and, when it detects unusual seizure-inducing electrical patterns, delivers an impulse, or stimulation, to disrupt the oncoming seizure. The technology is similar to that of a pacemaker that monitors and stimulates abnormal heart rhythms.

While approved by the FDA for use in adults, RNS can be used in select pediatric cases where all other treatments have been exhausted and where surgical removal of the area of the brain where seizures originate would cause significant permanent defects. Unlike anti-seizure medication, which can cause dizziness, drowsiness, depression or confusion, the RNS System does not cause chronic side effects. Patients do not feel the impulses it delivers.

“There are lots of kids who have responses to medicine or who are candidates for more aggressive surgeries, but there’s a good percentage of kids who we tell there’s really nothing left we can do,” Dr. Hansen said. “Now we have the ability to offer families another option and data shows, over time, you actually have a good chance of getting impressive seizure control.

A Glimmer of Hope

Wyatt Keele and his family had little hope left for relief from his lifetime of debilitating seizures. They tried everything from a ketogenic diet to multiple medications to thermal ablations of brain tissue to removing the portion of the brain where Wyatt’s seizures first originated. None of the interventions gave him significant, long-term seizure reduction or control.

“We were out of options,” Jennifer Keele, Wyatt’s mother, said. “There's no more medications for us. There's no more surgeries as far as taking out more of his brain. We were just kind of stuck.”

In February, doctors at Cook Children’s Medical Center told the Keele’s about the RNS device and its potential as a new treatment option for Wyatt. During the following months, physicians spent time mapping out the electrical activity in Wyatt’s brain using previous brain scans and electroencephalograms (EEGs) in order to pinpoint seizure location and determine potential placement of the device’s leads. By July, the Keele’s were convinced this was the right next step for Wyatt.

“My husband and I had already said that we weren't going to put Wyatt through anymore surgeries,” Keele said. “But after talking to his physicians we started researching and saw that this is a big deal and it has helped so many adults. The success rate is so high that we just couldn’t say no.”

It was the hope they longed for. The potential for Wyatt to be independent, attend school without worry, take part in activities with other kids and do all of the things his older siblings get to do without suffering from daily or weekly seizures.

“We do hope that the RNS is able to, over time, significantly control Wyatt’s epilepsy,” said Cynthia Keator, M.D, Wyatt’s epileptologist and medical director of the epilepsy monitoring unit at Cook Children’s Jane and John Justin Neurosciences Center. “We’re not sure if he’ll be able to fully come off of seizure medications, but we do hope that it'll decrease his medication burden while also decreasing the seizure burden and restoring some of his quality of life.”

The RNS device will continuously monitor and collect data related to Wyatt’s brain and seizure activity and, as it learns more and more, will adjust and improve its seizure-preventing interventions. In other words, the more data points the device collects about the electrical patterns in Wyatt’s brain, the smarter it will get in knowing when to send an impulse to interrupt a seizure, potentially improving Wyatt’s outcomes year after year.

Clinical studies of the effectiveness of the RNS System demonstrated a continuous improvement in seizure reduction over time. According to NeuroPace, at 9 years post implantation, patients experienced a median of 75 percent reduction in seizures and 28 percent experienced seizure-free periods equal to or greater than six months. Quality of life also improved, with patients reporting a rebound in cognitive function and physical and mental health.

The device will be teaching Wyatt’s doctors along the way, too. Data collected by the RNS System can be downloaded wirelessly, providing physicians with important feedback on the intensity, duration and location of seizures. This type of long-term monitoring can help physicians better pinpoint seizure activity origin and guide them in treatment decisions and options as the child grows.

“I think what will be most interesting is what data these devices provide us,” Dr. Keator said. It's another advancement in how we understand epilepsy networks and allows us to continue to find the best ways to ideally help these patients and hopefully find a cure for them.”

Dr. Hansen believes the lessons learned from RNS data collection has the potential to help more than just the device recipients.

“This really does give us the opportunity to capture that long-term data that we never had before, which I think will also be hugely impactful in the overall research of epilepsy,” Dr. Hansen said. “So all of these children and adults who have the device implanted are really creating a unique research population that we never had before. And they all understand that they're getting to contribute something to our understanding of epilepsy and how that electrical activity propagates throughout the brain and that what we find out may help someone else, too.”

#1in26

One in 26 people in the United States will be diagnosed with epilepsy in their lifetime, according to the Epilepsy Foundation. For Wyatt, that diagnosis came at just 10-months old.

“Neither my husband or I had ever dealt with anybody that's had epilepsy before,” Keele said. “So this was a big learning experience for both of us. In the very beginning, we were scared to death all the time because of what we didn’t know and all the scary things that you hear about epilepsy.”

It’s a disease wrapped in a lot of stigma and misunderstanding, Keele said, and their family—Wyatt especially—has experienced the resulting isolation. But they remain guardedly optimistic about the future.

“As parents we try very hard to make sure Wyatt doesn’t feel like he is different,” Keele said. “We feel that is so important because he always says he just wants to be normal. Unfortunately, this is our normal, but we try to tell him God made him this way because he’s one tough kid and can handle anything thrown at him. We are excited because this could be life-changing for him.”

“I was blown away even then by the hospital and by the people here,” Dr. Donahue said.

Twenty four years later, Dr. Donahue will retire from full-time practice on Dec. 15, leaving behind a legacy of expert surgical skill, innovation, continuous improvement and compassionate care.

“Dr. Donahue has really been a pioneer in bringing pediatric neurosurgical care to the children of Fort Worth, North Texas and beyond,” said James Cunningham, M.D., executive vice president and chief medical officer at Cook Children’s Medical Center. “He came to Fort Worth after an impressive training resume and brought with him a keen intellect, advanced surgical skills, and a sense of innovation in the development of new and improved care for children.

“While his accomplishments during his career have been impressive, I believe that Dr. Donahue will be remembered most for his dedicated and compassionate care for the children and families he served,” Dr. Cunningham said. “His work ethic, his mentorship and his collegiality will be sorely missed on a daily basis at Cook Children’s, but I have no doubt that David will continue to find ways to contribute to the health and well-being of our community.”

Dr. Donahue, neurosurgeon at the Jane and John Justin Neuroscience Center at Cook Children’s Medical Center, turned his eyes toward Texas at the urging of fellow neurosurgeon Jack McCallum, M.D. Ph.D., whose adult neurosurgical practice, with the Southwest Neurosurgical Group, was pulling double duty at the time by taking pediatric cases when needed. As the demand for pediatric neurosurgical interventions grew, it became clear that the addition of a full-time pediatric neurosurgeon was imperative to meet the neurological health needs of children in Fort Worth and the surrounding region.

“I wouldn't be here if Dr. McCallum hadn’t invited me to come,” Dr. Donahue said. “That’s how it all started. The good will of that adult neurosurgical group to take on the care of children.”

During his tenure, Dr. Donahue has led the development of the pediatric neurosurgical program at Cook Children’s, growing it from a single surgeon to a nationally recognized team of four surgeons and a number of medical professionals. This growth has led to the addition of innovative technology such as iMRI-guided surgery and the use of endoscopy and stereotactic EEG. Dr. Donahue is quick to credit these advancements at Cook Children’s to his associates while taking pride in his role in assembling the team.

“He brought a lot of things here as a surgeon,” said M. Scott Perry, M.D., epileptologist and medical director of Neurology and the Genetic Epilepsy Clinic at Cook Children’s. “When you talk about the more minimally invasive approaches of laser ablation and stereotactic EEG, he’s come along the whole way, which is why it’s great that he’s a continual learner. He was always willing to learn the next thing and implement it.”

Humble At Heart

Austin Roberts was the recipient of Dr. Donahue’s exceptional care, but it was his humility that made way for a collaborative effort that saved her life.

When Roberts suffered multiple life-threatening complications, including meningitis, following the surgical removal of a brain tumor when she was 6 years old, Dr. Donahue stepped in with a treatment plan that helped her recover fully. Two years later, a seizure revealed the tumor had returned. This time it was imperative to remove the entire tumor to prevent its regrowth. The surgery was extremely risky and could only be safely accomplished with the use of iMRI technology, which Cook Children’s did not have at the time.

Together with the Roberts family, Dr. Donahue reached out to a surgeon using iMRI technology at UCLA Health and arranged for Roberts to have a second surgery to remove the tumor there.

“We are deeply appreciative of Dave’s willingness to partner with the team at UCLA Medical Center as our story could have been very different without what they had to offer with the iMRI,” Carol Roberts, Austin’s mother, said. “His humility saved my daughter’s life.”

But Dr. Donahue wasn’t done taking care of Austin.

Once again, Austin developed meningitis after returning home to Fort Worth. For a second time in her life, Dr. Donahue’s treatment plan and care helped her overcome the life-threatening complication and, at the age of 26, she remains cancer free.

“Next to Jesus, he’s the man that saved my life,” Austin said. “I owe that to him and, yes, to his humility, but also to his resiliency and his honesty. His kindness was so comforting to me and my parents through that whole journey.”

The Roberts became life-long friends with Dr. Donahue and his family, and joined with him and others in a campaign dubbed the Austin Roberts Refuse to Lose Fund to raise money to bring the iMRI technology to Cook Children’s.

“David Donahue was so very instrumental in the Refuse to Lose campaign because he knew exactly what he needed as a neurosurgeon,” said Jennifer Johnson, assistant vice president, Jewel Charity. “The technology was changing so rapidly that, by the time we had raised all of the money, technological advances were better and we were able to get the best imaging technology available.”

Country music legend Garth Brooks lent his voice to the effort by performing a private benefit concert at Bass Performance Hall at the close of the two-year campaign. Playing alongside Brooks that night was Roberts’s father, Dan, also a country music singer and songwriter. In the end, the Austin Roberts Refuse to Lose Fund raised $4.5 million of the $10 million needed for the technology. Because of their work and Dr. Donahue’s focus on his patients’ needs rather than his own acclaim, kids receiving care at Cook Children’s now have access to the same technology that saved Roberts’ life.

“The thing that's unique about pediatric neurosurgery is that you're trying to help people and you're also challenged intellectually,” Dr. Donahue said. “There’s so much happening in the world of science and neuroscience research. This hospital is involved in that, which is really one of the reasons I'm glad I'm here. All those things make it a really good thing to be doing with one's time.”

Patients Sing Praises

While Dr. Donahue’s list of professional accomplishments are many, none carry more weight than the personal praise of his colleagues and patients.

“To the kids he’s cared for, he’s meant a cure from epilepsy for many of them,” Dr. Perry said.

That’s true for Cole Pettit, 22, who battled epilepsy from the time he was a young child into his teens. The condition severely impacted his quality of life and ability to function in school. Medications left him in a fog much of the time. Most doctors said surgery wasn’t an option, until Dr. Donahue and the neurosciences team at Cook Children’s gave them hope.

“It wasn't a hundred percent guarantee,” said Kelley Pettit, Cole’s mother. “There was still some reservation. They couldn't really see super clear where the problem area was, but in talking with the team of doctors, including Dr. Donahue, I felt like there was a chance. And that's the first time that we had heard that in a long time. It was really kind of the first glimmer of hope.”

At the age of 15, Cole underwent surgery performed by Dr. Donahue’s steady hands. He has been seizure free since that day.

“It completely changed the entire trajectory of his life,” Cole’s mom said.

In addition to graduating from high school in the top half of his class—a feat once thought impossible for him—Cole graduated in August from Texas Tech University with a degree in kinesiology.

“Dr. Donahue is an incredible person, his career aside,” Cole said. What he's done for me is incredible. The opportunities I've been given and made available to me by the blessings he's given me are just unreal. I wouldn't have asked for anyone different to have been my surgeon.”

Even when his patients faced difficult outcomes, Dr. Donahue stayed by their side offering as much comfort and support as he did medical expertise.

Charlie Bourland was born prematurely with a chromosome abnormality and suffered from infantile seizures. At the age of 4, he became a patient of Dr. Donahue’s when he underwent surgery to remove a brain tumor. During one particularly difficult hospital stay, Dr. Donahue made a point to check on Charlie after learning he had been admitted. Together with his wife, Dr. Donahue made a special visit to Charlie’s hospital room to meet with the Bourland family and discuss his thoughts on Charlie’s prognosis.

“This was the week before Christmas and I am sure they had somewhere they were supposed to be together,” Karen Bourland, Charlie’s mom, said. “He made us feel important and loved. He made us feel we could get through the terrible times ahead because of all we had already been through together. He made us feel safe.”

Bourland remembers how Dr. Donahue cried with their family that night and comforted Charlie’s siblings. Charlie passed away a little over a year later in 2015 at the age of 15.

“Terrible things can happen to children, despite everyone doing their best for the child,” Dr. Donahue said. “It's just a real tough thing that you sometimes can never do well enough. You always want to do better. That's one of the challenges of this job and a very great stressor.”

Dr. Donahue credits his wife for helping him manage the intensity.

“I'm married to a wonderful pediatric nurse,” he said. “I don't know anybody who could put up with me, number one, but also who could manage these sorts of stresses as well as she can. She has been a real stabilizer for me."

In December, Dr. and Mrs. Donahue will celebrate 41 years of marriage. They have three sons and five grandsons.

Lifelong Learner

Most consider retirement the end of their working life but, always the learner, Dr. Donahue sees it as a new beginning. His post retirement plans include completing a master’s degree in public health and channeling that into advocating for the underserved.

“I think it'll take me a year or two to get that degree because I'm certainly not going to go full time,” he said. “I'm going to have time for my family and my grandchildren and to do some other things that I’ve wanted to do and haven’t had time to do.”

Some of those other things include travel, photography, learning German and honing his newest craft—bread baking.

“He’s baking bread every day,” Mrs. Donahue said. “I now have a pantry full of different kinds of flour. He loves to cook anyway so this has been kind of his thing to try different types of bread and make it for friends and neighbors.”

Even with all of these new endeavors, Dr. Donahue says he’ll still miss the relationships he’s made while doing a job he loves.

“I’ll miss the people, but I really won’t miss staying up all night and worrying about things the way I do now,” Dr. Donahue said. “I don’t think I’ll miss the visions of pain and suffering, but I will miss the children. I will miss the excitement of being in the operating room.”

In like manner, Dr. Donahue is sure to be missed by his colleagues, the staff at Cook Children’s and his patients and their families, but his imprint on the hospital and pediatric neurosurgery in Northeast Texas will remain indelible. His legacy of surgical excellence built on a foundation of compassion, attentiveness, humility and continuous improvement will continue to drive and shape the neurosurgical team at Cook Children’s for years to come.

The Seizure Recognition and First Aid Certification program helps individuals understand and recognize types of seizures, identify signs and symptoms of each, learn how to administer first aid when witnessing a seizure and outlines when to call for help.

“We sought to develop the program in-house knowing that Cook Children’s was an ideal venue to demonstrate how a company could institute wide-scale implementation of the Epilepsy Foundation's training to get large numbers of people educated,” said M. Scott Perry, M.D., epileptologist and medical director of Neurology and the Genetic Epilepsy Clinic at Cook Children’s. “Several of our nurses completed the necessary instructor education and, subsequently, took the training and made it an online course available to our staff. We’re encouraging all staff to complete the training in an effort to make Cook Children’s seizure safe.”

Dr. Perry sits on the professional advisory board for the Epilepsy Foundation of America and chairs the Public Health and Education Committee that reviews the course’s content.

About one in 10 people may have a seizure in their lifetime, according to the Centers for Disease Control. Although common, seizures are as varied as the individuals they impact. While some may result in a visible loss of consciousness or uncontrolled movement, others may be invisible to those who lack experience with or are unaware of the nuances of seizure disorders.

“This program breaks through stereotypes about seizures, even among health care professionals,” said Aubrey Esparza, MSN, RN, CPN, neurosciences clinical nurse leader at Cook Children’s. “Seizures can present in many different ways. Some can be subtle, so it is important to know the signs to look for.”

Oftentimes, unrecognized seizures are incorrectly labeled as behavioral problems, attention deficits or even substance abuse, resulting in a lapse of timely medical treatment, according to Esparza. Knowing what to look for and being able to describe the seizure activity in detail is key in getting individuals the help they need. The certification program emphasizes the importance that seizure description plays in diagnosis and treatment and the types of information health care providers need from a witness for better diagnosis and treatment.

“When a seizure is presumed to be something else, a child is unable to receive timely medical treatment," Esparza said. "Knowing what to look for and being able to describe that seizure activity in detail gives providers so much information as to how to diagnose and treat an individual. The more we know about what an individual’s seizure looks like, the better.”

Certification was first offered in-house to Cook Children’s inpatient neurosciences staff members in September. Since then, 56 members of the neurosciences team have completed certification—now a mandatory part of their continuing education employee training. But understanding how to recognize a seizure is also very valuable for those on general medical floors and in high-traffic areas like patient registration or the cafeteria, and the training is suitable for both medical professionals and non-medical employees. All Cook Children’s staff are encouraged to complete the optional one-hour course through the hospital's employee education portal.

The certification program is also offered to families of patients at risk for seizures or newly diagnosed with epilepsy. So far, nine families have completed the training and have reported increased confidence in managing seizure activity as well as the ability to better recognize and describe seizures. Even families who have children with epilepsy for multiple years have reported that the course is extremely valuable. Training for patient families is currently offered once a week via Zoom and will be provided in-person once it is safe to do so.

The Epilepsy Foundation’s course is open to any adult interested in increasing their confidence in recognizing seizures and providing seizure first aid. It is designed for anyone who works, lives or plays in a setting where seizures could occur or who interacts with someone who has seizures, according to the Foundation.

For more information on how you or members of your organization can become Seizure Recognition and First Aid Certified, visit www.epilepsy.com/firstaid or contact your local Epilepsy Foundation.

Miller Queen suffers from a severe and debilitating form of epilepsy known as Dravet syndrome, but you’d never know it by looking at him. At 7 years old, Miller is doing things that never seemed possible like playing soccer and going to school.

“Daily life when you don’t have seizure control is just a rollercoaster,” his mother Chelsea Queen told NBC 5. “You’re kind of always on edge waiting on the next seizure to happen.”

Starting at 6 months old, Miller was having as many as 10 seizures a day. Every treatment he tried failed and his parents were afraid to take their eyes off of him for even a moment.

Then, they found Cook Children’s and M. Scott Perry, M.D.,  an epileptologist and medical director of Neurology and the Genetic Epilepsy Clinic at Cook Children's.

“When you deal with epilepsies that are so difficult to control, it certainly becomes difficult not to feel that there’s not an answer,” Dr. Perry said.

Miller was enrolled in a clinical trial for the drug fenfluramine, which was once used as a popular appetite suppressant. Only a handful of hospitals nationwide were involved in the trial and Cook Children’s happened to be the only one in Texas. The drug completely stopped Miller’s seizures.

“The treatment completely changed his life,” Queen said in the interview with NBC 5. “He’s always been such a happy kid but now he’s able to experience life to the fullest.”

Overall, the clinical trial showed fenfluramine reduced seizures on average by about 70%. The research helped lead to approval from the Federal Drug Administration, opening up fenfluramine as a treatment for everyone with Dravet syndrome.

“The next medication to try and make you seizure-free is probably 2%, but you know what, you might be that 2% so that’s why we keep looking for it,” Perry said.

For Miller’s parents, they know this to be true.

Matt Queen, Miller’s father told NBC 5 “If something doesn’t work, don’t lose hope.”

For most of her 20 years, Shanley Stuteville has been a patient at Cook Children’s.

She came to the medical center at the age of 3 after she began having seizures. Even as an adult, she continues to be seen by the Neurosciences team. After receiving care for so long, Shanley knows better than most how overwhelming a hospital stay can be, especially for younger kids.

“I was in the hospital last summer for my second phase [of testing], and my nurses were mentioning they were really glad I was older because a lot of the younger kids get scared,” Shanley said. “After that, my mom suggested I should write a book to help them.”

After years of testing and needles, Shanley has a wealth of empathy for younger patients. She recalls her initial feelings of fear of the unknown, but they were quickly lost when she realized her hospital was unlike any other.

Shanley began to write in July 2018, while scheduled to undergo testing at Cook Children's Neurology Epilepsy Monitoring Unit (EMU), where she would be watched 24/7 for four days to see what the source of her seizures was. The book became a family interest when her aunt began to illustrate Shanley’s medical team and created an animated world where leads, IVs and MRI machines weren’t so scary after all.

“Soon an IV was applied to my hand. Their magical spray made it something I could stand,” Shanley wrote. “It took away the pain and for that I was glad. It did not hurt, not even a tad.”

Shanley donated 50 copies of her children’s book to the Epilepsy Unit, but despite her appointments and hospital stays she’s found other ways to give back to her medical team. As a student leader at her university, Shanley completes a lot of service hours, but her service project last year was a hospital-sized treat.

As a “thank you” to her medical team, Shanley baked over 900 cookies for the Dodson Specialty Clinic staff. It took her a period of several school semesters to complete, but she delivered homemade cookies to each floor.

Although she spends an ample amount of time at Cook Children’s trying to figure out why she has seizures, it’s not uncommon for patients with epilepsy to have periods of time without seizures. Shanley would occasionally go 100 days without seizures, and even celebrated with a cake with her nearly lifelong doctor, Howard Kelfer, M.D., a Cook Children’s neurologist. However a life without seizures was never permanent.

“She’d go long periods of time without having a seizure and then one would come back around so it was really discouraging for them because they thought she was going to be over it,” Dr. Kelfer said. “It eventually became very clear that her seizures weren’t responding just to medications. It was always, ‘well she went this long without a seizure, maybe she’ll go longer this next time.”

Shanley was eventually determined as a surgical candidate after her time in the EMU last summer. Her surgery in May 2019 removed a portion of the front lobe in her brain. While she has not had a seizure since, it will take a year of no seizure activity before it can be deemed a success.

Shanley is now able to sleep through the night, a small comfort she didn’t have before her surgery. Her surgeons were also careful in the placement of her scar, which will be hidden by her hair when it begins to grow back.

“I have to say that I wouldn’t mind if it did [show],” Shanley said. “It will always be a reminder of the wonderful men and women at Cook, as well as hopefully provide an example to other children that they can walk through this and recover too.”

Shanley remains an advocate for younger patients, and is recovering quickly after her brain surgery. Her passion for patients inside the medical center has shaped her life for the last 17 years, and she is hopeful to make it a lifelong expression of gratitude.

“Shanley and her family come to all the family support groups and they’re willing to volunteer to talk to other families,” Dr. Kelfer said. “Shanley is extremely motivated to not allow her seizures define who she is.”

Following her surgery last month, Shanley will return to school this fall where she is studying to become a pediatric occupational therapist.

Ryan, 10 years old, wondered if he would ever get the chance to play the game he loves so much after a rare neurological movement disorder call dystonia changed his young life. He went from being a rough and tumble multi-sport athlete to using a wheelchair to get from class to class in his elementary school.

Ryan became the 100^th patient at Cook Children’s to receive deep brain stimulation(DBS) surgery on Monday, Nov. 30, 2015. The surgery was performed by John Honeycutt, M.D., Cook Children’s medical director of Neurosurgery.

Nearly two year later, it’s hard to imagine this little boy once struggled to walk or hold a pencil in his right hand. The successful DBS surgery has brought him back to the normal the Conder family knew before DBS robbed him of his childhood for more than a year.

“I’m the happiest mom in the whole wide world,” Kayla, Ryan’s mom, said. “When he first got diagnosed we were shocked and it was really hard because we had to have help with almost everything. But now he doesn’t need help or want help. I’m just so excited and really amazed. I’m very thankful because he’s like he was before.”

Last year as Ryan his family wait out in the lobby for their appointment, Dr. Honeycutt happens to walk by on his way into the Jane and John Justin Neurosciences Center. After a couple of steps, he realizes who he has passed and stops in his tracks. He comes back to say hello and marvels at the success of Ryan’s surgery.

“It’s a modern medical miracle,” he tells Kayla.

Then it’s time for a visit with Warren Marks, M.D., a neurologist and medical director of the Movement Disorders and Rehabilitations Programs at Cook Children’s. He sees Ryan every three months.

Ryan spent significant time with Dr. Marks before and after surgery at the Cook Children’s Motion Lab, which is equipped with technology that enables a specialized team the ability to analyze the unique movement of each individual patient and plan a treatment plan for them.

Ryan goes through a routine checkup with Dr. Marks. Then they go out to toss a rubber ball to each other. Dr. Marks leaves the game for a bit and gets a reflex hammer that he uses as a make-shift bat. They are having fun and both are in a great mood.

“This is so rewarding. This is why you have a DBS program because you get kids like this," Dr. Marks said. They come back to being completely normal kids. They come back to doing everything they were doing before the surgery. Everything they want to do. It’s perfect.”

Ryan has been cleared to play baseball, basketball and at recess. He’s not allowed to play contact sports like football or soccer. But he does take his football to school to play catch.

His friends call him the robot because of the surgery that includes two battery-operated pulse generators, much like pacemakers, implanted near the collarbone. Ryan doesn’t mind the nickname at all. He kind of enjoys it.

He shows his friends a video Cook Children’s made as it followed Ryan toward his surgery last year. And what do they say?

“They are like, ‘Wow. It’s amazing what modern technology can do these days,” Ryan said.

Did we mention he’s a really funny kid? Even during his worst days, he maintained his sharp sense of humor. But now the smiles come much easier to everyone in the household and even the tears aren’t so bad lately.

“When he was looking at the video the other day, every time I watch it I cry because I get to see where he was and where he is now,” Kayla said. “It’s not crying because I’m sad. It’s crying because I’m happy. I tell him, ‘Ryan I love watching it but it makes me cry.’

Kayla noticed something was wrong with her little boy around September, 2014. She noticed Ryan running differently than normal during one of his baseball games.

When asked what was going on, Ryan said he couldn’t help it. Then after noticing that his toes on his right foot were curling in, Kayla took her son to the family doctor.

Kayla remembered she had cousins who had dystonia and called her aunt to talk to her about it. After the conversation, Kayla arranged a referral to see Dr. Marks.

Dr. Marks commented that Kayla reminded him of someone and then as they talked, he found out that one of her cousins was not only a dystonia patient, but the first one that Dr. Marks treated at Cook Children’s who had deep brain stimulation surgery performed on her. The surgery was done 15 years ago before Cook Children’s began its own DBS program.

After an initial diagnosis, Dr. Marks verified that Ryan had a genetic version of dystonia.

Dystonia is a disabling disease and sometimes painful condition that limits children in many ways, impacting motor, cognitive and social development. Because medications have a limited effect on most forms of dystonia, Cook Children’s began a Deep Brain Stimulation Program of its own in 2007.

Kayla admits to being scared and nervous at the unknown of surgery. But after talking to Dr. Marks and his team, she hoped this would be a fresh chance for Ryan to return to the little boy he was before dystonia began to take over his body.

Ryan walked with his right foot on his toes and his right arm is now curled in, making it difficult to use. She hoped for Ryan to be able to walk and run like before, but also to use his right hand to write. He had to dictate his work at his elementary school.

Kayla said the hardest part was watching the things Ryan could do and how active he was, playing sports and being a typical little boy, to where he needed help walking, taking a shower or cutting up his food.

But that was then. Now Ryan is back to being the fun-loving, sports playing, ornery little boy he was before the surgery.

“The best part of all this … he’s right there,” Kayla said pointing to Ryan. “He’s walking, running and jumping. He’s able to take care of himself. No parent ever wants to see their child go through what Ryan went through. But hands down, we got more than we ever imagined.”

Learn more:

• What Is Deep Brain Stimulation?
• What Is Dystonia?
• The architect: Warren Marks, M.D.
• The surgeon: Helping kids like his own
• Cook Children's Motion Lab
• Cook Children's Movement Disorders Program

The phone from Clinical Genetics came at 5:12 p.m. on April 10, 2018. Grace Wilson-Rabel remembers the exact time and date because it’s the moment that changed her life.

Candace Gamble, M.D., is a clinical geneticist with Cook Children’s, and the call was to let Grace know her daughter’s diagnosis.

Wilson was a secretary for Cook Children’s NICU and she motioned for her charge nurse to say she needed to take the call.

“OK, we received Austyn’s test results back. Her test is positive.”

Austyn had been diagnosed with neurofibromatosis type 1 (NF1), a genetic disorder that affects 1 in 3,000 people throughout the world. NF1 impacts children in many different ways, including light brown skin spots, neurofibromas (small benign growths) on or under the skin and/or freckling in the armpits or groin.

About 50 percent of people with the condition have learning challenges. Health concerns also include the softening and curving of bones, and curvature of the spine (scoliosis), and occasionally tumors may develop in the brain, on cranial nerves or the spinal cord.

While these tumors are usually not cancerous, they can cause health problems by pressing on nearby body tissues.

Following the diagnoses, Grace immediately felt cold fear run through her entire body and she broke down in tears.

After all, everything had changed.

Austyn was born Jan. 23, 2017.

Grace’s pregnancy was normal until her thirty-fifth week when she began to experience high blood pressure. Because it continued to be elevated, she was induced at 37 weeks. Austyn was born weighing 6 pounds, 12 ounces.

“There were absolutely no signs that she had a genetic condition hiding inside of her,” Grace said. “At 2 days old, we had her newborn check-up and discovered that her bilirubin was high.”

At 2 weeks old, Grace noticed her daughter was working hard to breathe and made a “horrendous noise” every time she inhaled, so back to the pediatrician’s office she went with her daughter.

Following three more visits to the Emergency Department at Cook Children’s, Grace made an appointment with Pulmonology where Austyn was diagnosed with laryngomalacia, a congenital softening of the tissues of the voice box, or larynx, above the vocal cords. This new diagnosis caused a whole other set of issues. Austyn’s suck/swallow/breathe coordination was off, which caused failure to thrive and a hospitalization at 7 months.

Due to her low weight and delayed development, Austyn was tested for cystic fibrosis.

“Thankfully that test came back negative, but we were placed in physical therapy because despite working with her every day, she did not crawl until 11 months and did not walk until 19 months,” Grace said. “We would find out later, with her diagnosis, that this is all very common for kids with NF.”

At 3 months, Grace noticed Austyn had three brown spots on her abdomen.

“I didn’t really think too much of it other than I thought they were cute birth marks,” Grace said.

At her 4-month checkup, Grace pointed out the spots to her pediatrician, Joyce Rafati, M.D. Dr. Rafati looked at them and said she thought it could be neurofibromatosis, but it was too early to tell.

By the age of 10 months, Austyn had 13 spots. Dr. Rafati referred Austyn to be seen by Dr. Gamble in Genetics to test for NF.

“The earliest we could be seen by the geneticist was January 29, 2018 – which was four months out,” Grace said. “The hardest part of this whole journey is patience. The wait time to see a specialist always feels so far away and then after the specialist visit we are left in limbo because there are so many ‘wait and see’ moments with NF.”

Austyn received a simple blood draw to test for the genetic condition, but it would take six or more weeks for the results. The test is expensive and has to be approved through insurance, which took another four weeks.

Once she received the approval, she took Austyn to the lab at the Dodson and had Austyn’s blood drawn on March 1, 2018.

Six weeks after the test, Grace received the phone call with the results.

“Processing that was one of the hardest things I’ve ever had to do,” Grace said. “I’m still processing it to this day. You have to go through a grieving process that sometimes takes months to years. You may grieve it your whole life but you have to be strong and you have to push through. NF1 brings lifelong doctor appointments and ‘what if’ questions. And, every day that there isn’t something going on and you’re not in the doctor’s office is a win.”

Grace said what helped her get through the most difficult time of her life was her husband, George. “He’s been my rock through this,” Grace said.

A few months later, Grace and George took Austyn to meet her new neurologist, Jeff McGlothlin, M.D.

“He was amazing, so gentle and thoughtful with Austyn and her condition,” Grace said. “And with talking to us. He answered all of our questions and gave us an idea of what the game plan would be.”

Austyn was supposed to have surgery to fix her airway a couple of weeks after her neurologist appointment so Dr. McGlothlin decided to piggy back on the surgery and do her first MRI to test for optic nerve gliomas the same day so she would only have to be sedated once. Gliomas are non-cancerous brain tumors that can affect a child’s vision.

“The day of the surgery came and we anxiously handed our sweet 18- month-old baby over to the arms of the doctors and nurses,” Grace said. “We knew she was in great hands but it didn’t take away that fear and sadness of what she is having to go through.”

About four hours later Austyn was out of surgery and recovering in the PICU at Cook Children’s. All went well. She had a great night and the next morning she was in good spirits and eating. The MRI was the last thing on Grace’s minds.

Around 10:30 in the morning, while still at Cook Children’s, Grace received a call from the neurologist’s office.

“I didn’t even think that they would be calling me to tell me bad news, I just thought they might be calling to tell me everything looked great. I was wrong,” Grace said. “The woman on the other end said, ‘Dr. McGlothin wanted me to let you know that there are two optic gliomas.’ I couldn’t even breathe. ‘What?’ I asked, choking back tears. She repeated the news and all I could say was, ‘This has been the worst year,’ and I sobbed and sobbed.”

Grace watched Austyn sleeping peacefully. She was so small and innocent and unaware that she had something so concerning inside her “precious little head.”

“Austyn has had a few more eye appointments and another MRI and I am happy to report that as of today her vision is unaffected and her tumors are stable,” Grace said. “We hope and pray that they stay this way. Her neuro-oncologist is pretty confident that she will never need treatment. Since these tumors are inoperable the only treatment option is chemotherapy.”

Grace said Austyn has been through more needle sticks, tests, hospitalizations, etc. in her short two years of life than most adults have. But throughout the process, Grace said she couldn’t be more grateful for Cook Children’s and the team that has taken care of her daughter since that day when her life changed: her pediatrician Dr. Joyce Rafati, gastroenterologist Danny Rafati, M.D., endocrinologist Teena Thomas, M.D., neurologist Dr. McGlothin, neuro-oncologist Jeffrey Murray, M.D., her ENT Michelle Marcincuk, M.D., and pulmonologist Nancy Dambro, M.D.

“Austyn has rocked everything like a champ,” Grace said. “She knows when they want to listen to her lungs, heart and belly, where the stethoscope goes. She knows where the blood pressure cuff goes and holds her arm or leg out for it. She never complains. And all of her doctors and nurses are her friends.”

Dr. Keator, who is a pediatric neurologist and epileptologist at Cook Children’s, began volunteering at Cook Children's at the age of 14 as a title holder with the Miss Texas Organization. Along with many of the other titleholders, she would perform her talent for patients in the atrium. Back then she performed ventriloquist routines.

“My fondest memory is being in the Atrium performing and you look up and see all the children and their parents looking down and watching you,” she said. “It was so daunting then and now I walk past it all the time.”

Then in 1998, through a senior internship program during her senior year at Trinity Valley School, she spent her spring semester with Hematology and Oncology at Cook Children’s.  

The department held a special place in her heart because her cousin battled cancer there. That stint with the doctors made such an impact on her that after completing her internship, she continued to volunteer at the medical center.

When asked if she always wanted to be a doctor, Dr. Keator said, “I always knew I wanted to do something in the medical field. We all have that childhood dream of what we want to be when we grow up, and even then I always wanted to help people, especially children.”

As a volunteer, she tried to ask good questions and absorb as much knowledge as possible to prepare her for medical school. She learned the importance of listening to the patients and their families. She also saw science come to life. It wasn’t just about studying words in a book; it was seeing how medicine and medical care actually worked.

“Volunteering here I also realized how little I knew!” Dr. Keator said. “Just regular biology or chemistry class in high school is nothing compared to what people do in real life. It was a process, where I learned that being a doctor was about more than just being smart, it really takes a lot of compassion and motivation. I was able to see the interaction of the doctors and nurses with the patients. I realized I had so much more to learn than what was just in a textbook. ”

It was through her volunteering at Cook Children’s that she developed her interest in working with children, especially those needing chronic or long-term care. “I learned my true passion of wanting to help others was the relationships you develop with your patients and their families,” says Dr. Keator. “In neurology, we care for many children with neurological conditions that require close follow up and care, as many of our patients are cared for from birth through adulthood. Of course, I enjoy the pathophysiology of neurological conditions, especially epilepsy, but, what I also love about my field is that you become part of the team and you truly get to know the family.”

Now at Cook Children’s, Dr. Keator sees new people learning those same life lessons. “I see volunteers today at Cook Children’s and they are all age groups who choose to spend time with our patients. I’m so thankful so many people want to come here and volunteer and help with the children. It’s pretty incredible. “Everyone should volunteer. It’s good for the soul.”

Dr. Keator said that working at Cook Children’s is “totally surreal” because it was everything she aspired to do as a child. “It’s really cool to look back and say, ‘Wow, I did it.’”

Cook Children’s has been utilizing intranasal ketamine as a rescue treatment to break unrelenting migraine attacks in the emergency room and inpatient setting since December 2016. Originally, the drug was utilized out of necessity due to a shortage of dihydroergotamine – the medication previously used most frequently for persistent migraine attacks which failed to respond to outpatient therapy. The team presented their findings at the International Headache Conference in 2017.

“Until that time, little to no experience with nasal ketamine had been reported in pediatric patients,” said Adrian Turner, a clinical pharmacist at Cook Children’s. “However, our institution’s experience is broadening the horizon of potential treatment options.”

Now the U.S. Food and Drug Administration has approved esketamine, a chemical compound similar to the anesthetic ketamine, to relieve depression in hours instead of weeks. The drug, the first to be approved to fight depression since 1988, is believed to help patients who have been treatment resistant to other depression therapies.

Esketamine, developed by Johnson & Johnson, will be marketed under the name Spravato® and is a nasal spray that allows the drug to be absorbed through the lining of the nose, directly to the blood stream.

Patients using Spravato® will also be required to enroll in a Risk Evaluation and Mitigation Strategy (REMS) program to monitor for serious side effects related to sedation, dissociation, abuse/misuse and to support its safe use. Patients will be required to self-administer the drug under direct observation of a healthcare provider.

“This is potentially a game changer for millions of people,” Dr. Dennis Charney, dean of the Icahn School of Medicine at Mount Sinai in New York, told NPR. “It offers a lot of hope.”

At this time, no plans have been made at Cook Children’s to use ketamine for depression, but intranasal ketamine has been vital for helping patients with severe migraines.

Intranasal ketamine for migraines not only worked for a majority of Cook Children’s patients (almost 70 percent of patients between December 2016 and October 2017), but was safe and well tolerated. Any side effects seen, some dizziness and very mild dissociative effects (i.e. feeling funny, out of body experience) were mild and didn’t last longer than one hour after administration.

No patient had to stop due to side effects.

Turner said using ketamine for migraine offers many benefits:

1. Patients don’t need an IV or injection to receive treatment.
2. The number of medications needed to treat the pain may be reduced.
3. The time needed to treat the pain may be reduced.
4. It provides patient with a wider variety of options to treat their migraine.

Ketamine was first created in the 1960s and was largely used as a battlefield anesthetic and veterinary medicine. By the 1980s, hospitals were using the drug for sedation and severe pain.

Until recently, the FDA approved the drug in patients 16 and older for general anesthesia (dental procedures, outpatient surgery, intubation). Compared to other medications used for sedation/anesthesia, ketamine is relatively safe due to less effects on breathing and blood pressure.

In the past few decades, ketamine became the focus of studies for the treatment of depression. The Department of Health and Human Services reported an estimated 16.2 million adults and 3.1 million adolescents aged 12 to 17 in the United States had at least one major depressive episode. This is especially important when considering the rates of suicide. In 2016, the Centers for Disease Control and Prevention (CDC) reported suicide as the number 10 overall cause of death with more than 44,000 deaths attributed to suicide reported. Even more troubling, suicide was reported to be the number 2 overall cause of death for people aged 10-34, number 4 for people aged 35-54, and number 8 for people aged 55-64.

“Expanding treatment options is not only beneficial to improve the quality of lives of patients with depression, but it may also help save many lives in the future. Depression can be very difficult to treat,” Turner said. “Compared to other diseases and diagnoses, depression is not a one-size fits all treatment. Expanding the treatment options is a huge benefit to patients who have exhausted several treatment options with little to no relief.”

Aside from the new indication for depression, ketamine and esketamine both have potential use in post-traumatic stress disorder (PTSD), migraines, refractory/chronic pain conditions, and seizures. There are active studies underway in the U.S. and internationally examining the use of ketamine to treat PTSD, status epilepticus, and oral ketamine to treat chronic pain in adults and children. Cook Children's Pain team is currently treating patients with chronic/refractory pain with a ketamine IV and oral ketamine.

Some may know ketamine as a party drug. Ketamine (also known as Special K, Super K, and Vitamin K among other street names) is known as a potential drug of abuse and a drug used to facilitate sexual assault crimes. When taken illegally, users often inject, snort, or smoke the drug to feelings of calmness and out of body experiences.

Unfortunately, because these are often taken in very large doses, users also experience complications such as hallucinations, immobility, amnesia, and unconsciousness. It’s especially dangerous when given in very high doses or mixed with other drugs and/or alcohol because this greatly increases the chances of serious health problems and/or death. The World Health Organization notes that ketamine is a potential drug for abuse, but the actual incidence of dependence or overdose is rare. Chronic abuse has been linked to urinary tract problems.

But under the guidance of health care providers, ketamine has been highly beneficial.

“Our experience with ketamine has been positive,” Turner said. “The success we have seen at Cook Children’s combined with the approval of esketamine for refractory depression is very exciting. As a health care professional, it is invigorating to see years of research produce positive outcomes for our patients. I look forward to seeing Cook Children’s on the forefront of contributing to this new frontier. ”

She’s trying to be calm, but you can see her fidget as she waits for M. Scott Perry, M.D., an epileptologist and medical director of Neurology at Cook Children’s, to walk in the room.

As soon as Dr. Perry walks in the door, the anxiety that Stephanie’s been trying to hide melts away.

For a moment, this isn’t a doctor and patient family greeting each other, it’s old friends catching up. After all, Dr. Perry began caring for Aaden when he was only 4 days old and he has been with the family throughout his treatment for seizures as a result of tuberous sclerosis complex.

But then it’s time to get down to business.

“I find it easiest just to talk with you about what we went through in conference so you know what I told everybody when we came up with our decisions,” Dr. Perry tells Stephanie.

He is referring to a previous conference where a team of neurologists, a neurosurgeon, a neuroradiologist and more gathered to discuss a treatment plan for Aaden. Now it’s time to discuss the plan with Stephanie.

First, Dr. Perry covers familiar ground. He talks about Aaden’s history. In the conference with the Cook Children’s staff, and now in the room with Stephanie, Dr. Perry marvels at her ability to detect Aaden’s seizures. “It’s almost psychic the way she knows her child is about to have a seizure. She detects a slight difference in her little boy’s mood or a quick far-away look,” Dr. Perry said. “Stephanie is never wrong in detecting the seizures.”

“I’ve always been able to notice it, as soon as he started having seizures,” Stephanie said. “To be honest, I don’t know how I knew. I just know when he’s having one. It’s almost as if he looks at me like. ‘Hey mom something’s wrong.’ I can tell by the look on his face. He’s looking for someone to comfort him or grab him.”

After they go over his history, it’s time to look ahead at what’s next. David Donahue, M.D., a neurosurgeon at Cook Children’s, will perform a surgical procedure called stereo-EEG to pinpoint the location in Aaden’s brain that is causing his seizures.

“Aaden will undergo Stereo EEG placement,” Dr. Perry said. “The initial preoperative workup has localized his seizure onset to a region of the brain on the right side-most likely towards the back part, but he has a number of tubers and potentially epileptogenic areas within the region we’ve localized. We will use Stereo EEG to further hone the localization of seizure onset in hopes of limiting any potential resection to the smallest area possible. We will place a number of depth electrodes into the brain to map out the area of onset – the epileptogenic network. From that information, we will then propose a final epilepsy surgery option to the family.”

Dr. Perry carefully explains the procedure. They talk about the possible risks. For this procedure and where the placement is taking place, a loss of vision is the biggest concern if they have to take out brain tissue in the back, but they hope to avoid this with more precise mapping through Stereo-EEG.

Stephanie admits to being scared, but she also lets Dr. Perry know she believes in him and trusts him completely. They finish talking about the procedure. Dr. Perry asks Stephanie if she has any more questions. Then he examines Aaden briefly before telling the family goodbye.

Next up, Dr. Donahue walks into the room. The seasoned neurosurgeon has performed countless surgeries on children’s brains, but he knows this is never a moment that the patients’ families take for granted.

He tells Stephanie he understands she’s nervous. He goes over the details of the surgery. He explains the look of the electrodes that will be placed in Aaden’s brain (think piano wires). He lets her know that this is what’s best for Aaden as they look ahead to a resective surgery that will hopefully help control or even end the little boy’s seizures.

By the end of the meeting, you can see a different Stephanie. She’s still scared, but there is new found assurance and confidence of the people taking care of her little boy.

Dr. Perry sympathizes with Stephanie, but he is confident this is the best opportunity to help Aaden.

“Epilepsy surgery is an art – and the ‘value’ of the surgery, like art, is sometimes in the eyes of the beholder,” Dr. Perry said. “For example, seizure freedom is the goal of every surgery, but it can sometimes come with adverse functional consequences, like weakness or vision loss. Some consequences obviously outweigh the benefit of seizure reduction/freedom and make surgery a no-go, in others the functional consequences may be acceptable.

“The art is a discussion and decision making process between the epilepsy team, the family, and the patient to decide where the value lies. The surgery itself is an art – as there are multiple potential ways to do it – not just the technologies used (laser ablations, resection, neuromodulation, etc) but the way the technique is used, for example, approaching from different angles to avoid damage to normal brain. These different options all come with a variety of benefits/risks, each which must be weighed with the potential to achieve the end goal of seizure freedom with minimal adverse effects.”

Struggling to make it in the music industry, he gains notoriety when he has a seizure on stage and the video of the event goes viral. The portrayal of the seizure itself is inaccurate, but more troubling is the way the film chooses to make the diagnosis of epilepsy comedic.

Fans of the rapper begin to refer to him as “seizure boy” and even create a ridiculous dance to mock the seizure he had on stage. The official website for the film states “the young rapper with big dreams has one night to bounce back from major embarrassment.”

The fact they label the event of having a seizure in public as an embarrassment only feeds further bias and ridicule for those that suffer from epilepsy. Given 1 in 26 people will suffer from epilepsy in their lifetime, it is a diagnosis every person should be familiar with. It is unfortunate in this day and age, there is still so much misunderstanding, misrepresentation, and stigma for those with epilepsy. Media has the power to educate and raise awareness on a scale unlike any other, yet media has a long history of feeding bias, fear, and confusion about epilepsy.

What is the history of epilepsy in media?

Epilepsy and people with seizures have been represented in film for many years. An analysis of characters with epilepsy since the 1950s show that most characters are portrayed as sad, damaged individuals that are feared and often linked to psychiatric disease.⁽¹⁾ 

The seizures shown on film are almost always convulsive type and violent, though that represents only a small subtype of seizures. This inaccurate portrayal has not improved with time. An analysis of epilepsy in film since 2004 showed almost all seizures depicted remained convulsive type and most were uncontrolled, yet in reality 70 percent of patients gain control of their seizures with medication.⁽²⁾

When simple seizure first aid was delivered in film, 96 percent was performed by a healthcare professional, yet inappropriately provided in 57 percent of the cases. In 97 percent of the portrayals, the cause of the epilepsy was known, though in reality only about 30 percent of epilepsy has a defined cause. These inaccurate depictions set unrealistic expectations for patients, but more importantly teach inappropriate behaviors for those that may be called on to help a person having a seizure in public.

Print media doesn’t fare much better. Newspapers covering neurological conditions were found to utilize stigmatizing language in 21 percent of their stories, and epilepsy represented the most commonly represented diagnosis (30 percent). ⁽³⁾

The advent of social media has taken the ability to marginalize those with epilepsy to another level. Consider this, the internet has become to “go-to” source for medical knowledge with  more than 60 percent of adults reporting they search for medical information on the web – many people using social media sites like Twitter, YouTube, and Facebook to find information.

One would hope user generated information on these platforms would be more accurate and sympathetic, but is it? A seven day analysis of “tweets” related to seizure(s) found over 10,000 tweets.⁽⁴⁾ Of these, nearly 10 percent were written specifically to ridicule and over a third were metaphorical – that is comparing abnormal movements to “seizures” much like the way the “seizure dance” is portrayed in “The After Party.” Overall, 41 percent of tweets were considered derogatory. YouTube videos suffer from inaccuracy as well, with up to a third of videos labeled to depict seizures clearly depicting non-epileptic events – again spreading misinformation. ⁽⁵⁾ In addition, up to 10 percent of videos related to epilepsy/seizures can been labeled as inaccurate or derogatory. ⁽⁶⁾

So much work remains to raise epilepsy awareness throughout the world. Media presents a unique and powerful resource to educate the public on this common condition, its varied presentations, the availability of treatments, and the appropriate response to a person having a seizure. Increased awareness will hopefully erase fear, inaccuracies, and bias towards those with epilepsy.

When films like “The After Party” are created in the name of “entertainment”, they do nothing more than increase the stigma for those with epilepsy, erasing all we in the epilepsy community work to alleviate each day.

References

1. Kerson JF, Kerson TS, Kerson LA. The Depiction of Seizures in Film. Epilepsia 1999 40(8):1163-1167.
2. Moeller AD, Moeller JJ, Rahey SR, Sadler RM. Depiction of Seizure First Aid Management in Medical Television Dramas. Can J Neurol Sci 2011 38(5):723-7.
3. Caspermeyer JJ, Sylvester EF, Drazkowski JF, Watson GL, Sirven JI. Evaluation of Stigmatizing Language and Medical Errors in Neurology Coverage by US Newspapers. Mayo Clin Proc 2006 81(3):300-6.
4. McNeil K, Brna PM, Gordon KE. Epilepsy in the Twitter Era: A Need to Re-Tweet the Way We Think About Seizures. Epilepsy Behav 2012 23(2):127-30.
5. Brna PM, Dooley JM, Esser MJ, Perry MS, Gordon KE. Are YouTube Seizure Videos Misleading? Neurologists Do Not Always Agree. Epilepsy Behav 2013 29(2):305-7.
6. Wong VS, Stevenson M, Selwa L. The Presentation of Seizures and Epilepsy in YouTube Videos. Epilepsy Behav 2013 27(1):247-50.

But when they realized she wasn't using her left arm, they became alarmed.

Autumn admits it took a little bit to be concerned because the last thing on her mind was that her young daughter could have a stroke.

"I didn't even know kids could have one," Autumn said.

Kynlee was born on March 5, 2015, in Oklahoma. Autumn and Darrell hoped for a healthy child, after watching their oldest daughter, Addison, now 9 years old, battle cancer throughout her young life.

But from the beginning, Kynlee faced health problems. She was born with bladder dysfunction and was cared for by Blake Palmer, M.D., a pediatric urology specialist and surgeon. 

"Our older child faced so many medical problems and we kind of became used to having something wrong with our child," Autumn said. "But to have another child with medical issues was very emotional."

Even with everything they had gone through at this point, nothing prepared Autumn and Darrell for what occurred on May 2017 in their little town of Coyle, Okla.

When things didn't change with Kynlee's left arm, they went to their local ER where Kynlee was initially diagnosed with a stroke. She was transferred to a larger hospital in Oklahoma, and after a lengthy hospitalization, the family returned home with limited information about Kynlee's condition.

Once again Kynlee started having similar stroke symptoms in July 2017, but these were thought to be seizures. At first doctors placed Kynlee on seizure medication, but that didn't work because that wasn't effective against the little girl's mini strokes.

At the same time this was going on, doctors found Kynlee needed heart surgery to repair a hole they found in her heart. Kynlee had been scheduled for cardiac surgical repair at another hospital in July 2017, but it was canceled due to high risk and complexity of her medical condition.

Autumn and Darrell were at a loss by the end of the year and struggling to find some hope for their daughter's condition. They found it in an old friend. Dr. Palmer had moved to Cook Children's and was still seeing Kynlee for her urological condition. He suggested Kynlee be seen by Cook Children's Comprehensive Stroke and Thrombosis Program.

Lori Buechler, a nurse in Urology who works with Dr. Palmer, coordinated the family's care with Jo Tilley, the stroke and thrombosis PNP, to transfer records, imaging studies and complications involved with dealing with multiple specialties.

"Dr. Palmer and his nurse Lori have always been there for us," Autumn said. "I'm not sure we could survive without their help."

The Thomas family arrived at Cook Children's Stroke Comprehensive Clinic in January 2018. "We saw the stroke team," she said. "And I mean it was a team. We saw everyone you could think of. We stayed three days at Cook Children's and we found everything in three days that we had waited months to find out. We had a diagnosis. They performed four or five tests that no one had ever performed on her in Oklahoma and she was seen by every specialist you can imagine in that time

"When she got here, Kynlee's condition was severe and significant, which placed her at imminent risk for further harm due to stroke or cardiac arrest," said Fernando Acosta, M.D., a neurologist and associate medical director of Stroke and Thrombosis Program at Cook Children's. "We felt it was in Kynlee's best interest to be admitted as quickly as possible for further investigation." Marcela Torres, M.D., a hematologist and director of the Stroke and Thrombosis Program at Cook Children's, agreed with Dr. Acosta. Kynlee was in a precarious state because, despite her preventive antiplatelet therapy, her congenital heart disease would likely also affect her brain perfusion. So, after her admission, several sub-specialists were quickly consulted, including Cardiac ICU, Cardiology, CV surgery team and Sami Hadeed, M.D., a pediatric pulmonologist.

During her initial admission, Kynlee was placed on the Epilepsy Monitoring Unit without any antiepileptic medication. The EEG showed no signs of epilepsy and she was taken off of her medication. Imaging indicated Kynlee did have a new stroke to her right front lobe. This explained Kynlee's earlier episodes where she lost movement on her left side.

Prior to genetic testing, Kynlee was initially diagnosed with a cerebral vasculopathy, similar to Moya Moya syndrome, a rare disease that affects arteries in the brain.

"Kynlee's treatment plan is an example of the complexity of a pediatric stroke patient," Dr. Torres said. "Because of our stroke program and our involvement with the International Pediatric Stroke Society and pediatric research studies, we are able to provide patients with the highest level of pediatric stroke care."

Then there was that matter with Kynlee's heart. Cardiology completed an echocardiogram and formal evaluation. Vinod Sebastian, M.D., a pediatric heart surgeon at Cook Children's, performed the surgery on Kynlee in late January to complete closure of her aortopulmonary window.

"It was definitely a relief and comfort just because we had people on our side who knew what was going on and wanted to help us," Autumn said. "But also because it was all happening so fast, it was just kind of ... 'WHOA!'"

Along with everything else going on with their two daughters, major changes were occurring in the Thomas household. Autumn delivered her third child, a boy named Parker, in January 2018. Parker was staying at the Ronald McDonald House near the medical center with Darrell, who had recently been laid off from work.

The family was discharged in early February and Kynlee and her parents returned to Oklahoma. Everything was fine Friday and Saturday, but on Sunday Kynlee lost the feeling in her left side again. She was taken to an emergency room in Oklahoma where scans verified another stroke. She was transported by helicopter on that Monday back to Cook Children's. Once at the medical center, she lost movement on her right side as well.

Kynlee remained in the pediatric intensive care unit for a week. Doctors found she wasn't getting enough blood flow to her brain due to her progressive cerebral vascular disease. Pediatric specialists from Neurology, Hematology and Cardiology monitored Kynlee as they balanced her medications to treat her blood pressure and decrease her chance of another stroke.

Over the next week, doctors eventually found the right mix of medications to increase her blood pressure enough to improve the perfusion of her brain. She was monitored closely.

Kynlee also showed improvement in functionality. At the time of discharge she was able to crawl and was recommended for an orthotics consult, as well as outpatient physical and occupational therapy.

Since her discharge, Kynlee's genetic testing that had been ordered at Cook Children's, came back. She was diagnosed with an ACTA2 gene mutation, which causes people to be predisposed to vascular disease including strokes, coronary artery disease and aneurysm.  Autumn says the entire family plans to go through genetic testing for the gene as well.

Things are looking up for the Thomas family. Darrell has a new job. Their older daughter is doing well and Parker shows no sign of any health problems. Kynlee is back home in Oklahoma and doing well thanks to the medications doctors at Cook Children's diagnosed specifically for her condition.

"Kynlee is starting to walk again," Autumn said. "She hasn't been able to walk since her stroke in February. She's doing great. I'm watching her trying to feed her baby brother a Barbie doll bottle."

Autumn laughs. She welcomes this very typical, very silly problem.

They have come such a long way just to feel their own sense of normal.

Stroke and cerebrovascular diseases are within the top 10 causes of death among children and up to 70 percent of stroke survivors have residual neurological impairment.

Because the causes and symptoms are so different, treating stroke in children requires specialized training. Cook Children's Comprehensive Stroke and Thrombosis Program is one of 16 such centers in the U.S. As members of the International Pediatric Stroke Society, the Cook Children's team works with a network of other pediatric hospitals to improve stroke care worldwide.

The program is led by Medical Director Marcela Torres, M.D., a hematologist/oncologist and Co-Director, Fernando Acosta, M.D., a neurologist.

The team is committed to the early recognition, treatment and prevention of pediatric stroke through research, innovation and education.

The program allows patients to be seen by multiple specialists at once, instead of scheduling numerous visits. Patients can see pediatric sub-specialists from:

• Hematology
• Neurology
• Neurosurgery
• Neurodiagnostics
• Neuropsychology
• Interventional Radiology
• Social Worker and Child Life

What are some of the presentations of SCN2A-related disorders? Children with SCN2A can often present with epilepsy which may manifest in several levels of severity. SCN2A was first discovered as a cause of benign familial neonatal infantile seizures (BFNIS), a syndrome presenting in multiple family members with seizure onset as neonates and infants with normal developmental outcome and good seizure control. Later, SCN2A was discovered as a cause of infantile spasms and other early onset severe epilepsies of childhood, the so-called early infantile epileptic encephalopathies. SCN2A has been implicated as one of the causes of Ohtahara Syndrome, Dravet Syndrome, Migrating Partial Epilepsy of Infancy and West Syndrome amongst others. Finally, SCN2A has been found to be a major cause of intellectual disability, schizophrenia, and autism which may occur in the absence of epilepsy.

What is the cause of SCN2A-related disorders? SCN2A is a gene which encodes a sodium channel found within the initial segments of neurons. This location is important to determining whether a neuron will generate a signal or not, thus a reason mutations can present with neurological symptoms. Most mutations in SCN2A are de novo, meaning they occur spontaneously and were not inherited from the parents. This is often the case in more severe disease presentations. SCN2A may be inherited in an autosomal dominant manner in more benign presentations such as BFNIS.

How are SCN2A mutations diagnosed? Diagnosis is made using genetic testing in patients with appropriate clinical features. MRI is often normal and EEG findings may vary.

Is there a treatment for SCN2A-related disorders? Unfortunately, there is not yet a cure for SCN2A-related disorders. Certain sodium channel drugs (for example, phenytoin, lamotrigine) have demonstrated more favorable responses for seizure control in some patients, while in others sodium channel drugs may aggravate seizures. This may be due to how the mutation impacts the function of the sodium channel (gain of function versus loss of function). Aggressive control of seizures with a clear rescue plan for prolonged seizures is important. Other manifestations such as movement disorders, dysautonomia, and neurobehavioral manifestations can be managed to some degree with medications and therapy.

What other problems might be found in patients with SCN2A disorders? In addition to epilepsy and developmental delays, other manifestations of SCN2A can include movement disorders such as dystonia, abnormal gait, ADHD, autism, dysautonomia (i.e. problems with heart rate, blood pressure, and temperature regulation), and GI problems such as feeding difficulties or reflux.

For more information about SCN2A and SCN2A Awareness Day, visit www.scn2a.org.

What are some of the symptoms of SCN8A-related epilepsy? Children with SCN8A epilepsy often present early in life with developmental delays which may occur from birth or may arise shortly after seizure onset. Seizures often begin in the first 18 months of life (average 4 months) with a variety of seizures types including infantile spasms, generalized tonic-clonic, myoclonic, focal-onset, and absence seizures amongst others. Seizures are often difficult to control in 70 percent of patients. Movement disorders such as myoclonus (quick jerk-like movements), dystonia (fixed abnormal posturing of the limbs), ataxia (unsteady, incoordination), and choreoathetosis (constant, irregular, writhing movements) are also common manifestations. Mild to severe intellectual disability is common and many patients demonstrate autistic symptoms.

What is the cause of SCN8A-related epilepsy? Dr. Michael Hammer, Ph.D., first discovered the SCN8A gene as a cause of epilepsy when he was searching for the cause of his daughter Shay’s epilepsy. Shay unfortunately passed away from Sudden Unexplained Death in Epilepsy (SUDEP), but we continue to celebrate her life and raise awareness of this syndrome on her birthday (2/9) each year. To learn more about Dr. Hammer and Shay’s story, click here. 

SCN8A is a gene which encodes a sodium channel found throughout the nervous system and highly expressed in the brain. Sodium channels are in part responsible for generating the electricity of the brain. In children with mutations in SCN8A, sodium channels can open too easily or stay open too long which produced increased electricity and tendency for seizures. Mutations in SCN8A are almost always de novo, meaning they occur spontaneously and were not inherited from the parents. SCN8A mutations cause an autosomal dominant disorder, meaning only one gene mutation is required for symptoms of the disorder to arise.

How is SCN8A-related epilepsy diagnosed? Diagnosis is made using genetic testing in patients with appropriate clinical features. MRI is often normal or may show some global atrophy. EEGs often show slowing of the background rhythm with multifocal spikes.

Is there a treatment for SCN8A-related epilepsy? Unfortunately, there is not yet a cure for SCN8A-related epilepsy, however, certain sodium channel drugs (for example, carbamazepine, phenytoin, oxcarbazepine) have demonstrated more favorable responses for seizure control. Many families have reported levetiracetam to worsen seizures. Aggressive control of seizures with a clear rescue plan for prolonged seizures is important. Other manifestations such as spasticity and movement disorders can be managed to some degree with medications and therapy.

What other problems might be found in patients with SCN8A-related epilepsy? Anecdotally, patients with SCN8A mutations are often reported to have exaggerated startle response or excessive jitteriness in infancy. Some patients may have small head size (microcephaly), visual impairment, hearing impairment, and autonomic dysfunction (for example trouble maintaining temperature or steady heart rate). As more patients with SCN8A are discovered, the characteristics of the syndrome continue to evolve.

For more information about SCN8A and SCN8A Awareness Day, visit http://www.scn8aawarenessday.net/.

Diego and Tatiana Alvarez did all those things to help their son Mirko in his battle against epilepsy in a wild adventure that began a year ago in Bolivia and brought them to Cook Children's Medical Center in Fort Worth, Texas.

Mirko, now 4 years old, has since returned with his family to Bolivia. He's walking and showing amazing progress. It's hard to believe that this little boy went through so much over the course of a year, taking his family with him through a remarkable adventure.

Down a dead-end street

Life in Bolivia is judged on a different scale than how we measure success in the United States. Minimum wage is around $280 a month. You make a decent living at about $800 and anything over $1,000 is considered great.

Diego and Tatiana lived a good life in Santa Cruz, Bolivia, raising their children - Andrey, 10, Mia, 8 and Mirko. Diego helped students learn English and competed in mixed martial arts. Tatiana drew amazing sketches and her art hung in the couple's home.

For three years, Mirko kept up with other children his own age when it came to speaking, running and playing.

Then on Feb. 12, 2016, Mirko ran a very low temperature, but nothing to get too concerned over ... at least not right away. After the fever continued for a couple of days, the family made a doctor's appointment.

The evening prior to the appointment, Diego was in the gym training for his next fight. He put his phone away, but as he was warming up he noticed it was blinking and he felt that something was going on before he picked up the phone. When he answered, he heard his mom hysterically screaming and shouting.

"Something has happened to Mirko," she said. "He's convulsing."

Diego grabbed his stuff and ran to his car. His wife called shortly after. "Mirko's dying," Tatiana cried.

Tatiana held Mirko and stepped outside their home screaming for help, Andrey ran to a neighbor's house to a neighbor, who rushed Tatiana, Mirko and his siblings to the hospital. Diego drove from training to the hospital to meet his family. The convulsions continued all over the little boy's body and his eyes rolled back in his head. The doctor on duty asked Diego to step outside.

"We are a small hospital. We can't take care of him," the doctor said. "You need to take him to a big hospital."

Then the doctor surprised Diego with a question. "Do you have a car?" The doctor explained the ambulance at the hospital wasn't dependable and a newer car would get Mirko to the hospital faster.

Diego, his sister, Mirko, with an IV in his arm, and the doctor piled into the sports car and took off like they were being chased in an action movie.

"Thank God I drove a fast car," Diego said.

The family raced through Bolivia, pounding the horn, screaming at people to get out of the way while running red lights. At one point, a traffic jam stopped the car and the doctor told Diego he had to find a way to get Mirko to the hospital because the little boy only had a few minutes left to live.

"It was terrifying," Diego said. "I was moving on instinct and desperation."

Diego remembered that another hospital was only three blocks away from where they were stopped. He hopped his car on the sidewalk, yelling at people to move. They made it to the bigger hospital's ER. The convulsions lasted for more than 40 minutes. But doctors were able get Mirko stabilized.

Treated Like a Refrigerator

Mirko always had been a daddy's boy. They share a special bond that began at birth. Diego stayed next to his sleeping son, scared of what would happen if he closed his eyes.

"I didn't even know what a seizure was until this happened to Mirko," Diego said.

Mirko woke up at the hospital and smiled at Diego like it was any other day and even asked, "Why are we here?" But any sign of relief vanished as Mirko's eyelids began to twitch again.

Mirko received thorough testing - an MRI and an EEG. The next day, Diego and Tatiana met with a neurologist at the hospital in Bolivia.

"She treated our son like a refrigerator. She was very cold," Diego said. "She said, 'Your son has epilepsy. Give him this medication. I'll see you in three months.' We had so many questions. 'What can he eat?' 'What can he drink?' 'What happens the next time he has a seizure?' 'Will he have a normal life?' 'But we never got the chance to ask anything."

White Spots on the Brain

The MRI scan of Mirko came back, showing "white spots on his brain." A neurologist told Diego that his son could have leukodystrophy, which shows up in the white matter of the brain on scans. Diego looked up the disorder online and his heart plummeted into his stomach. If he had leukodystrophy, Mirko possibly faced loss of motor function, muscle rigidity, the loss of sight and hearing and eventually death."

"I cried a lot," Diego remembers. "It's the worst you could hear about your son. He's going to die. I was never an alcoholic. I never did drugs. I didn't smoke. Neither did my wife. We lived such healthy lives. But we couldn't help but wonder if it was somehow our fault."

Fortunately, Mirko's parents wanted a second opinion. They found a neurologist who told them their son didn't have the fatal disorder. The white spots were likely a result of the MRI machine being so old at the previous hospital.

While that news was good, it only proved what Diego and Tatiana already knew. They weren't getting the best care possible. They would have to go elsewhere to find any hope for Mirko.

A Game of Chance

Their new neurologist told Diego and Tatiana their son wasn't going to die, but he needed surgery to control his epilepsy. Mirko needed surgery to remove the portion of the brain causing the seizures and he needed it fast.

Mirko's seizures came often - 20, 40, 60 seizures or more a day. He lost his quality of life and their happy little boy was losing himself to epilepsy.

"I was tortured," Tatiana said. "I would count the seizures every day. The amazing thing was after every seizure, Mirko would still smile. I read a Facebook post by a dad who said to count the smiles and not the seizures. That changed everything for me. I now saw more smiles than seizures."

Diego and Tatiana were desperate to find help for their son.

Diego's father, Javier, told his son they would find the best place in the world for their son. Somehow, they would find the money to make this miracle happen.

Mirko's parents searched online and researched to find the right hospital for their little boy. They found hospitals in Chile, Brazil, Miami, New York and Houston. They narrowed their focus to a children's hospital in Miami. The soonest they could see him was three months.

But as Diego frequently says, "God has his ways."

During the turmoil of trying to find a place to help his grandson, Javier went to play billiards with friends to distract him from the real world for a little while.

A friend could see something was bothering him and asked what was wrong. When Javier told the man Mirko's story, the friend told him about his niece's child who had a serious neurological disorder. He described to Javier about a place in Fort Worth, Texas called Cook Children's. After being seen at Cook Children's, the family had actually moved to Fort Worth to be near the doctors. That's how much the place had meant to them.

"He told my dad that lady had been all over the world. All the same places we'd been looking at too. But the woman said she couldn't find any treatment for her son until they came to Cook Children's," Diego said.

That evening, Javier talked to his friend's niece about Cook Children's. The following day Javier connected Diego with the woman and they spoke for more than two hours.

"She really convinced me," Diego said. "I felt it. I felt this was the place. We had to get to Fort Worth."

Diego called Cook Children's and was connected to Yadira Nunez, International Business Development liaison. He told her his story and that a neurologist at Cook Children's, had taken care of a family friend's son. Nunez was in Mexico for a conference with a neurologist and put Diego on the phone with the doctor.

Within a matter of hours, Diego and Tatiana booked an appointment that would have them arriving in Fort Worth in less than two weeks.

Sell Everything

With an appointment made, the Alvarez family now had to pay for their trip. Javier bought the plane tickets for Mirko and his parents. The need for money called for drastic steps. On Dec. 5, 2016, Diego spent his birthday selling nearly everything he owned to provide care for his son; and the rest of the family also sacrificed their belongings.

"Thank God Diego's father was there to help and made everything possible," Tatiana said. "Diego always says, 'My dad is our guardian angel.'"

Diego, Tatiana and Mirko arrived in Fort Worth on Dec. 6, 2016. They spent Tatiana's birthday, Mirko's birthday, Christmas and New Year's Day at Cook Children's - all away from their two other children.

Their older son and daughter were heavily impacted by Mirko's condition. Not only were they away from their parents for more than six months, but they had to withdraw from private school.

"Private schools are so important in Bolivia," Diego said. "Public schools there aren't good. You can't get the same education, plus they are insecure. There are kidnappings at the other schools."

Diego's mom went with her grandchildren to the school and stayed there until the end of the day so the kids wouldn't be left alone.

Dump Truck

At Cook Children's, the Comprehensive Epilepsy Team took over the day-to-day care of Mirko as he was admitted to the Epilepsy Monitoring Unit once he arrived. Within 11 hours, he experienced 40 seizures.

"We evaluated Mirko and struggled a bit with the actual reason for his epilepsy, but ultimately decided a large resection of his frontal lobe would be the best answer to help him without hurting him," said Scott Perry, M.D. medical director of Neurology. "We also knew this initial resection may not be adequate, but wanted to try to preserve as much of his brain as we could."

On Feb. 2, 2017, David Donahue, M.D., a neurosurgeon at Cook Children's, performed the surgery on Mirko to remove his left frontal lobe. While the Diego and Tatiana found previous doctors cold, they found themselves being listened to and informed at Cook Children's. Both Diego and Tatiana refer to Dr. Donahue as the "sweetest."

Following surgery, doctors prepared Diego and Tatiana for the possibility that their son may not speak because speech was in the area removed. But when he came out of anesthesia, Mirko looked at his parents and said two simple words typical of many little boys.

"Dump truck."

It's believed that the brain, being the amazing organ that it is, already was using the healthier parts on the opposite side to shift his speech. The other fear was that Mirko would be paralyzed on his right side. But soon after surgery, they noticed while sleeping Mirko moved his right arm and leg.

Diego and Tatiana hoped that the surgery would end Mirko's seizures. While they weren't every day, Mirko still had seizures, although not the severe ones that had been so devastating. The neurological team held out hope that the surgery would eventually end the seizures altogether.

And Diego and Tatiana waited for things to return to how they used to be.

You Can't Go Home ... Yet

Following the initial surgery, Diego, Tatiana and Mirko planned to go home to Bolivia. They had received help and Mirko's seizures weren't as severe and easier to control.

But that wasn't good enough for the Epilepsy Team.

The team knew that the same quality of health care wouldn't be waiting for Mirko in Bolivia and he wasn't responding well enough to medications to control his seizures. They felt it was it was in Mirko's best interest to have one more surgery.

"It was difficult to tell Mirko's family that another major surgery was needed. But we felt under the circumstances, a second surgery was required to establish a better quality of life for Mirko once he returned home," Dr. Perry said. "It was really his only hope at that point."

While the first surgery removed Mirko's left frontal lobe, a few weeks later the second surgery disconnected the entire left hemisphere from the right side of his brain.

After the surgery was performed Mirko developed a blood clot in his brain and the family stayed in Texas for two more months to treat it. Mirko required six blood thinner shots a day for those two months and took them "like a warrior he is." After the blood clot was gone, it was finally time to return home. Mirko and his parents arrived in Santa Cruz, Bolivia on May 26, 2017, just one day before Mother's Day. They celebrated the holiday and Mirko's health six months after their adventure began in Texas.

The surgery and treatment was a success. Mirko is now seizure-free. His mental faculties are intact and he is able to speak. Physical therapy helped Mirko regain the right side of his body and he is now able to walk.

"We used to have a normal life," Tatiana said. "We would go to the cinema. To the mall. We had a normal family. A year later, everything changed because of epilepsy. We have a normal life again now. We are just so thankful to God for Cook Children's and everything they have done for us. We really are."

After all, what's a great adventure without a happy ending.

Celebrating Cook Children's Centennial

Cook Children's is turning 100 and will be celebrating all year! Don't miss out on the fun throughout 2018. Enjoy unique stories, parades, special guests, and community events across Fort Worth. We hope that you can share in some our celebrations because you're the 1 in our 100!

Celebrate with us

For most of the first 20 years of his life, Michael Jankowiak never played ball or even owned a toy. His debilitating cerebral palsy wadded his fingers into a tight fist. He barely moved his hands, except to drive his electric wheel chair.

Then, during a visit to his neurologist, every changed. During a BOTOX® session at Cook Children’s, Fernando Acosta Jr., M.D., a neurologist at the Jane and John Justin Neurosciences Center, told Michael's mother, Lynn, that a surgeon on staff could possibly make a big difference in her son’s life.

Lynn was told Pamela Sherman, M.D., performed surgeries on children with disabilities. 

The original intent of the surgery was to help children clean the palms of their hand and aimed at improving hygiene for patient with significant contractures (the permanent tightening of muscles, tendons ligament or skin that results in a loss of motion in the affected joints).

But the sides effects were, as Lynn puts it, “pretty remarkable.”

Patients who need this surgery often demonstrate limited function with the contracted limb preoperatively. Things such as the ability to trim finger nails, avoid skin breakdown in the palm or elbow and the ease of nursing care with dressing, bathing and transferring to the wheel chair are the focus of surgical intervention. 

"Placing the upper extremity in a more functional position and releasing contractures often has a wonderful added benefit of improving use,” Dr. Sherman said. “Suddenly, the patient with previously limited spontaneous use of their limb has a hand that they are able to use to push a wheelchair control, use a communication board or hold an object.  A little goes a very long way for them.”

For the first time since he was a baby, Michael, who is now 24, opened up his left hand and played with a toy. Michael even held his own glass and brought it to his mouth to take a drink.

“This surgery has given him something new with his life,” Lynn said. “He has never been able to find a toy that he could play with. We took a golf-sized rubber ball. It’s elastic and put a rubber band on it. It looks like it came out of a gum ball machine. But when he’s playing with it, he grins from ear to ear. He can now even hold the ball and drops it for the dogs to play with him.”

After receiving a second surgery on his right hand that summer, Michael could now play on his iPad. He can swipe and select different videos to watch on YouTube.

What may have seemed so routine to most families has been nothing short of a miracle to Lynn because of how far her son has come.

Lynn described her first few months after she learned Michael had cerebral palsy as “fuzzy.” She lived in a terrified blur of emotions and cried for the first year after learning of his diagnosis.

But through her tears Lynn kept her resolve, beginning with one decision – Michael would be transferred from the family home in Abilene to Fort Worth to be treated by Cook Children’s. They then moved to Fort Worth to stay closer to Cook Children’s.

“To see your baby crawling, trying to learn to walk and then all of a sudden he’s not moving, was horrible,” Lynn said. “We insisted he be transferred. If he had not gotten transferred Michael would not be alive. I believe that with all my heart. I would not go anywhere else.”

The first month he stayed in the Pediatric ICU. Since then Michael has been seen by a plethora of specialties at Cook Children’s including Infectious Disease, Neurosciences, the Heart Center, Radiology, Surgery and Rehabilitation Services for issues ranging from pneumonia to cerebral palsy.

Michael stopped moving his extremities at 18 months and was diagnosed at that time.

Mark Shelton, M.D., was the physician on-call the day Michael first arrived at Cook Children’s. Dr. Shelton, a member of the Cook Children’s Physician Network, continues to be Michael’s primary care physician.

“Dr. Shelton, even though he is a specialist, I want him involved in everything,” Lynn said. “I trust him completely. I honestly think Dr. Shelton saved Michael’s life. He’s wonderful and so is his entire staff. He has such wonderful nurses. But all of Cook Children’s has such great nurses.”

Medical Director of Orthopedic Services David Gray, M.D., has also been there for Michael through multiple operative procedures associated with cerebral palsy.

“Dr. Gray is amazing. I remember him when he joined the Cook Children’s staff,” Lynn said. “A few years ago Michael broke his femur. When the ambulance came I told them I wasn’t going anywhere but Cook Children’s. Dr. Gray wasn’t on call that day, but somehow they got in touch with him and Dr. Gray managed to be there when we needed him.”

And now even today, after all this time, Lynn believes Cook Children’s works miracles for her son.

“It’s really the entire system,” Lynn said. “Everybody works so well together. It’s one of those places. I remember how I felt from the first time I walked in at 2 in the morning. It’s just comforting. You knew you were going to be treated well and your child was going to be taken care of by everyone.”

The Epilepsy Foundation describes SUDEP as “the sudden unexpected death of someone with epilepsy, who was otherwise healthy.” No other cause of death is found when an autopsy is performed following SUDEP.

Each year, about 1 in 1,000 adults and 1 in 4,500 children with epilepsy die from SUDEP. While those numbers show how rare SUDEP occurs, the Epilepsy Foundation calls SUDEP the leading cause of death in people with uncontrolled seizures.

Currently physicians don’t have a way to predict or prevent SUDEP. The criteria to classify a death from SUDEP include:

• Patient suffers from epilepsy
• Death occurs suddenly
• Death is unexpected, while the patient is in reasonably good state of health
• Death occurs during normal activities
• No cause of death determined by autopsy
• Death is not a direct result of a known seizure or status epilepticus (but may occur following a seizure)

Some in the medical community fear that discussing SUDEP will raise anxiety in patients and families without reason, said Cynthia Keator, M.D., medical director of the Epilepsy Monitoring Unit at Cook Children’s.

“But I think it’s our responsibility as physicians within the medical community to make people aware of this rare condition,” Dr. Keator said. “We have seen studies that show patients and families want to know the risks they face.”

While the causes of SUDEP aren’t known for sure, SUDEP happens most often at night. Experts believe it may happen when there are problems with breathing, heartbeat and brain function after a seizure.

“Right now, most experts believe the best way to prevent SUDEP is through good seizure-management control. For people with poorly controlled seizures, the risk of SUDEP is much higher. It’s 1out of 150 each year,” Dr. Keator said.

Seizure management control means:

• Taking medication on time, every day – exactly as prescribed.
• Keeping a healthy diary of seizures, test results and asking questions of your doctor.
• Knowing your seizure triggers such as not getting enough sleep or feeling stressed.
• Creating and sharing your seizure response plan with others.

“It’s important for family, friends and caregivers to be informed of what to do during and following a seizure,” Dr. Keator said. “This includes knowledge of the recovery position and cardiopulmonary resuscitation techniques. Also, people need to know they should call an ambulance if the seizure lasts for more than 5 minutes or repeats without full recovery. It’s also important that people do not leave someone who has had a seizure for at least 15 to 20 minutes after the seizure to ensure that recovery continues.”

To learn more about SUDEP, click here.

While many people may have just recently heard of KD, it’s long been a treatment for some children with uncontrollable seizures. These are children who have found little or no relief after failing multiple seizure drugs and treatment methods. With the KD, there are many children who have achieved dramatic reduction in their seizures.

So what is KD exactly?

When used for our epilepsy patients, it is based on a specific ratio of fat: protein + carbohydrate. For example, 3:1 and 4:1 are frequently used KD ratios which means that 75 to 80 percent of the patient’s total calories are coming from fat! This is what is known as the classic ketogenic diet.

This means that for every 3 or 4 grams of fat in the diet, there is 1 gram that is a combination of protein and carbohydrate. Families whose children are placed on the KD are given a specific meal plan for their children. Each meal and snack consists of a combination of food items in specific gram amounts that will yield the prescribed diet ratio.

The KD diet is recommended for specific neurology and epilepsy patients. Good candidates for the diet include patients with seizures that are intractable to drug therapy and patients with metabolic disorders of carbohydrate metabolism, specifically Glucose-1 transporter deficiency syndrome and Pyruvate dehydrogenase deficiency syndrome. There are some medical conditions that make the KD dangerous, such as defects in beta-oxidation, carnitine deficiency and porphyria.

“The ketogenic diet is predominantly used in our patients with intractable epilepsy, specifically those with infantile spasms and Lennox-Gastaut Syndrome,” said Cynthia Keator, M.D., a neurologist and the director of the Ketogenic Diet Clinic at Cook Children's. “ The children are hospitalized to initiate the diet due to possible severe adverse effects (described below). The method on how the diet works is by the by the brain being forced to use ketone bodies (the by-products of fat/lipids) as a fuel source instead of carbohydrates (which is usually the main and quickest primary source of energy for the human body). By using ketones bodies as fuel, this may decrease some patient’s seizures. To date, the mechanism underlying beneficial effects of the Ketogenic diet remain a mystery.”

Unfortunately, about a third of patients do not respond to the ketogenic diet. When the diet does work, however, patients can experience 50 to 90 percent seizure reduction. If seizures improve from being on the diet, some patients can have their medications decreased, however, total elimination of all seizure drugs in rare.

Children usually spend no more than two years on the diet, either because it can be successfully tapered off, it fails to work, or due to the risk of long-term side effects including osteoporosis, elevated triglycerides, kidney stones, constipation, nausea, vomiting, pancreatitis, anemia, and rarely prolonged Q-T syndrome.

At Cook Children’s, the patients are monitored by an experienced team of epileptologists, led by Dr. Keator. The children placed on the diet are offered:

• Counseling and initial monitoring at a tertiary care center.
• A full staff of nutritionists, counselors and other support staff.

So should this diet that’s taken so seriously at Cook Children’s be a part of a summer weight-loss program for parents or kids who are just looking to shed a few pounds?

“The ketogenic diet can be helpful under certain circumstances, but it is not a diet for everyone,” said Jessica Holy, a clinical dietitian who works with the neurosciences at Cook Children’s. It should also be noted that the diet used for our neurology patients is the classic ketogenic diet described by a 3:1 or 4:1 ratio of fat: protein + carbohydrate. The KD making news today that is being used in the general population is not as strict and is closer to a 1:1 ratio. “The classic ketogenic diet at the 3:1 or 4:1 ratio is not medically recommended for the general population due to its adverse effects and possible nutritional deficiency which is dangerous,” states Dr, Cynthia Keator, “however a modified version, closer to a 1:1 ratio, with the aid of a nutritionist and your primary doctor can be accomplished for weight loss and health concerns.”

For anyone just starting the diet, low blood sugar is common. But for children not being monitored by the neurologists at Cook Children’s, the ketogenic diet comes with other concerns.

“We order a glucometer and testing supplies for our KD families so they can test their children’s blood sugar at home and the amount of ketones in the urine,” Holy said. “For both small children and teens, a concern would be inadequate intake of a number of vitamins and minerals that are found in regular portions of fruits, vegetables and grains, all of which are very limited on a KD. Another concern for both groups would be kidney stone formation. When the body maintains a state of ketosis for weeks or months, the balance of acid and base in the blood is disrupted. This can place a person at risk for kidney stone formation, especially if fluid intake is inadequate.”

Holy said the growth of younger children could be slowed though use of the KD due to vitamin and mineral deficiencies, insufficient calorie and/or protein intake and acidosis that is left untreated. The KD does not provide adequate amounts of calcium, so younger children are at risk for poor bone development and teens are at risk for bone loss.

“If families are not trained on what to look for and monitor, some of these side effects can have serious medical consequences,” Holy said. So how do you go about trying to make dietary changes and be “keto friendly” without causing dietary concerns?

Instead of focusing on eating, Holy suggests parents help their child find a way to be more active before tackling a diet.

“When you start to move more, your endorphin production goes up and you start to feel better. This can lead to a natural desire to make better food choices,” Holy said. “I think a reasonable way to approach a low-carbohydrate lifestyle is to, first, try to eliminate all sources of concentrated sugar. This includes obvious things like candy, cakes, cookies, pies, soft drinks and juice drinks, but also check food labels of processed foods.”

Holy recommends parents monitor how much sugar is in their child’s favorite cereal or breakfast bar. Many flavored yogurts are high in sugar.

Next, try to prepare more foods at home and limit visits to fast food restaurants. Think about meals that have a vegetable as the main dish with a side of protein and a small portion of a carbohydrate. Incorporating sources of healthy fats into the diet can add great flavor and keep you feeling fuller longer. For example, use olive oil as a salad dressing or drizzle it over cooked vegetables; have half of an avocado with a meal or for a snack; add nuts and seeds to salads or have a handful as a snack.

These smart choices can make for healthy lifestyle choices that last a lifetime and not begin a life-long trend of following the latest diet fads.

About the Jane and John Justin Neurosciences Center

When a medical condition interrupts your child's life, it can be scary, especially when it's related to the brain and nervous system. If your child is diagnosed with a neurological disorder or disease, it may ease your mind to know that our neurosciences department is one of the largest and most respected in the southwest. Click to learn more.

What Is Dravet Syndrome?

Dravet Syndrome is a rare genetic epilepsy (1 in 25000) that begins in the first year of life with seizures in the setting of fever. Unlike typical febrile seizures, children with Dravet syndrome often seize every single time they have fevers.

They may also seize when they have mild elevations of temperature, for instance, after taking a hot bath or after being outside on a hot day. They are normal developing and healthy in that first year of life, despite the fact they have frequent febrile seizures. These seizures can be very long lasting, 30 minutes or longer.

Their initial seizures are often characterized as hemiclonic seizures, so they’ll be seizing on the right side of the body at one time and the next time they come in, it’s predominately on the left side. After the age of 1, they begin to have seizures in the absence of fever. They have multiple seizure types at that point including absence, generalized tonic clonic seizures, atonic seizures and tonic seizures. The seizure frequency often increases, their EEGs become progressively abnormal and the children themselves can have a stagnation in their development or even a decline in development as the epilepsy progresses.

In the first 10 years of life or so, seizures can be quite frequent and very difficult to control. Generally after that time the seizures slow down, but will still occur throughout life.

What are the current treatment options of Dravet?

The mainstays of treatment of Dravet syndrome are medications. The first being valproic acid (Depakote), another clobazam (Onfi), and another one called stiripentol. Beyond those three primary medicines, there are other medications that can be used with variable success in addition to dietary therapy and recently, medical cannabis or cannabidiol, has shown some promise.

Importantly, there are medications that are used to treat epilepsy that should not be used to treat children with Dravet syndrome. Those are medications that work on the sodium channels. Because the syndrome is a disorder of sodium channels, using medications that work on sodium channels actually makes these kids much much worse. Carbamazepine, Oxcarbazepine, and Lamotrigine are a few of the medicines that work on sodium channels that should be avoided in children with Dravet syndrome.

Does that happen often? That people put these kids on those medications?

Unfortunately, yes. Because those medications are typically used for focal onset seizures and when these kids first present they are having focal seizures people treating them logically say, "Well let’s put them on oxcarbazepine." When these kids get worse the next logical reaction to that is that they say, “Oh they are worse. Let’s give them some more. They must not have enough.” Then they get even worse. That’s actually one way that some of these kids get diagnosed. People recognize that these sodium channel drugs made them much worse, so maybe they have a sodium channel disorder.

I have been guilty of making this mistake. So even in my immense knowledge of this disorder, I have made this mistake.

What impact does Dravet have on the entire family?

It’s interesting that you bring that up because we actually did a project with the University of Washington in Seattle about caregiver burden of caring for Dravet Syndrome. We developed a scale that they are in the process of standardizing for clinical use. These families have difficulties caring for their other children because they can’t spend as much time on the activities of the other kids because they spend so much time focusing on the health care and safety of their kid with Dravet syndrome. Jobs, it’s difficult for both families members to hold down a job because somebody usually has to stay with the child. I imagine it’s difficult for the parents themselves to get much time with each other because one is probably up most of the night worrying about their child, while the other one is up most of the day. The amount of time lost from work and other obligations, community obligations, etc. caring for the child is pretty significant.

And it impacts the siblings too, right?

Exactly. They may not get to do all the things that they want to do. Or if they do get to do those things, if they do want to play baseball, maybe the family can’t take them to baseball all the time. So it’s your neighbors taking them. The parents can’t be there because they are at the doctor or the hospital.

What are some of the advancements that have happened in the care of patients with Dravet and what do you see for them in the future?

Dravet represents one of the best understood epilepsies since we know the genetic mutation that is the cause of the epilepsy. So that’s a really important thing. Understanding that has allowed us now to understand more about why it happens and hopefully get to a point where we understand how to treat it. People with other epilepsies or other genetic epilepsies should appreciate and support research that goes into Dravet syndrome because if you can figure out the genetic cause of one and figure out how to fix it, there’s a decent chance we might apply the same thoughts to other things and figure out how to cure other epilepsies down the road.

As far as big things that are going to come in the Dravet world … One thing is that pharmaceutical companies have increasingly recognized the importance of this syndrome, which is why you have multiple new pharmaceuticals being evaluated. Cannabidiol one. Fenfluramine, the other. Both trials we’re doing here. Some drugs being used in other syndromes are also being considered. The drug being used in muscular dystrophy that skips over the abnormality in the gene that causes the disorder to help make a more normal gene, they are looking at applying the same kinds of ideas in Dravet syndrome. If you could somehow skip over the abnormality in the SCN1A gene and make a more functional protein, might we be able to improve the disorder? Maybe? So those are some of the exciting things.

Several new drugs are probably going to be investigated in the future. They've got animal models that they can test drugs on to see which ones might be favorable and investigate it more. I mean who would have ever thought to look at fenfluramine. It was just part of a diet pill in the past. Somebody was smart enough to think, "Well maybe half that drug might be worth something. Let's go look at it." It appears to be very effective. At least in the open trials they've done.

What made you interested in Dravet Syndrome?

Really it was in training, in my residency ... I've always been fascinated with epilepsy and fascinated by the story of epilepsy. I've always been fascinated by the idea that the longer epilepsy goes on the more likely the story might actually be told. Which is why I always harp with my students that when you have a patient with intractable epilepsy, if you do not understand what's going on, you should always start from the beginning and work your way to the present because you might find a pattern and see the story as it's told over and over and over again.

Dravet is one of those things that tells a story over time. So in the beginning when it's just febrile seizures people might not quite put it together. But you take febrile seizures and then the types and how long they are and then put that you've got these new types of seizures. Now you've got developmental delay and EEG abnormalities, over time the story becomes clear.

I found so many cases of Dravet when I was in training in kids that were diagnosed with other things. There was a kid I remember when I was in training that was about to get epilepsy surgery, and this has actually happened multiple times since then, where I've seen people who were in the process of getting evaluated for epilepsy surgery and doctors are going over their history and trying to figure out if the patient was a surgical candidate. This kid sounds like he has Dravet syndrome. They are 14 or 15 years old and their family is wanting to do a surgery and then we find out, no they've got this underlying genetic epilepsy that is not surgically treatable. It's an important thing to figure out.

So over time I have encountered more and more kids with Dravet syndrome. We probably see somewhere between 40 and 50 patients. The longest distance someone came to see me was from Florida. There are a couple of spots in the US where Dravet syndrome is frequently treated and these typically develop from a doctor interested in the syndrome and word of mouth from the Dravet community. It's a very connected community. A group finds a doctor they like who understands what's going on, they share that with everybody and they end up all trying to go to the same place. It makes for a better clinic and better care when we can have that connection with our patients and they are connected with each other.

For more information:

• Jane and John Justin Neurosciences Center
• Dravet Syndrome treated at Cook Children's
• Cook Children's Comprehensive Epilepsy Program
• Contacts and Locations

What is Deep Brain Stimulation Surgery?

Deep brain stimulation surgery involves two parts: implanting electrodes into the brain and a pacemaker under the skin of the chest. The two devices are connected by the surgeons and electrical impulses are sent from the pacemaker to the brain to correct the abnormal impulses of the movement disorder. The two surgeries take place about a week apart from each other. Following both procedures, the child usually goes home the next day.

The Path to Asleep DBS

When neurosurgeons at Cook Children’s first began performing DBS, patients had to be awake. Now, thanks to enhanced technology, patients can be under anesthesia and asleep. This change in the treatment’s technique came with the addition of an iMRI, which is essentially a giant magnet, at Cook Children’s. The iMRI allows neurosurgeons to have pinpoint accuracy while performing a delicate brain surgery.

“With all the technology we have, I know I am in the exact spot I want to be," said John Honeycutt, M.D., medical director of Neurosurgery at Cook Children’s “My accuracy for DBS is 0.5 millimeters."

Dr. Honeycutt is one of the world’s leaders when it comes to using DBS on patients with dystonia and will perform the hundredth surgery. It will be the fifteenth asleep surgery for Cook Children’s.

Building a DBS Program

Prior to 2003, DBS was done almost exclusively for Parkinson’s disease and tremors. Then, the Food and Drug Administration (FDA) allowed for DBS to be done on patients with dystonia, beginning at age 7. At that point, most hospitals weren’t doing the surgery on children.

Warren Marks, M.D., a neurologist and medical director of the Movement Disorders and Rehabilitations Programs at Cook Children’s, says Cook Children’s DBS program grew as part of an evolution of the movement program at the medical center.

Dr. Marks said it took two years of hard work to develop the DBS program at Cook Children’s. It involved assembling and organizing two entire teams – one to do the evaluations and postoperative management and another to do the surgery.

Cook Children’s performed its first DBS surgery in 2007.

“We have slowly and methodically grown the program so we try and do it in the best way we can and try to provide for the most kids we can,” Dr. Marks said. “The program is somewhat unique in that we are really focused on children. When we started our program there was no adult program to work off of. Virtually all DBS programs where surgeries are performed on children are in conjunction with adult programs. We didn’t have an adult program to work off of. We really did start from ground zero.”

The Road Ahead

Both Dr. Honeycutt and Dr. Marks take the hundredth procedure in stride. The milestone reinforces their success, but they say they never looked at this program as a race. They have been very careful in their approach to the surgeries and the patients they are treating.

What excites them is the fact that the last 15 cases were performed while the children were asleep.

Dr. Honeycutt says it can be a traumatic event for the kids to go through surgery awake, even after the efforts to keep them pain free while awake in previous surgeries. So with the advent of iMRI guided system, he saw the opportunity to perform the surgery on children who were asleep.

There will still be some surgeries where the patient has certain disorders that will require him or her to be awake. But for the most part, the surgeries will be done with the patients asleep.

*Illustrations/Graphics courtesy of Clearpoint.

Today, Dr. Marks looks back at that time as “an interesting diversion,” but it really helps explain who the man really is. After all, he’s built one of the nation’s most highly successful comprehensive clinical centers for pediatric movement disorders.

Dr. Marks, who is one of the first two Endowed Chairs at Cook Children’s, has molded a program where Cook Children’s deep brain stimulation serves as the centerpiece.

Most recently, Dr. Marks has seen the end of years of work with the addition of Cook Children’s Motion Lab, which sees children, teens and young adults who have a variety of complex movement disorders, including cerebral palsy, dystonia and traumatic brain and spine injuries.

He just keeps on adding to a legacy where he has brought together the most advanced technology with a kid-friendly atmosphere.

When he was a kid, Dr. Marks loved science and became fascinated with the brain and how it works. When he entered Texas Christian University, Dr. Marks considered a career in chemistry, earning a Bachelor of Science degree from TCU, and then he took that year to pursue architecture.

But once he entered Texas Tech University School of Medicine, Dr. Marks’ life’s work began to take focus. During his training he returned to studying the brain and found he had no choice but to make his career helping children.

“I always liked it that if you are going to do pediatrics, you know it up front,” Dr. Marks said. “If you look at the personality inventory of pediatricians, they don’t look like the rest of the physicians, they look like social workers. I talk to medical students when I have them over at the office and the ones that are going to go into pediatrics have no doubt that’s what they are doing. The ones who have hesitation about it are usually not destined to go into pediatrics.”

Dr. Marks joined Cook Children’s in 1988 and today serves as the medical director for the Movement Disorder and Neurorehabilitation Program.

Dr. Marks said he loves the multidisciplinary approach he finds at Cook Children’s, often working directly with rehabilitation therapists, orthotists, neurosurgeons, orthopedists and others. He has developed several multidisciplinary rehabilitation teams, including the transitional care unit, and specialized multidisciplinary clinics that have been developed for children with spasticity, movement disorders, and neuromuscular disorders.

“You have different people coming from different backgrounds,” Dr. Marks said. “Everybody’s perspective is different and we are all bouncing ideas off one another. It’s one of the great things about Cook Children’s.”

Dr. Marks said that team approach creates better care for patients. He said the goal of the neurology team at Cook Children’s is not just to treat children or find a quick fix, but to make their overall quality of life better. He calls this an exciting time for the Neuroscience Program at Cook Children’s, exploring new and innovative approaches to complex patient issues such as movement disorders and epilepsy.

“We are doing as much as anybody and more than most in the country when it comes to improving children’s lives,” Dr. Marks said. “We continue to expand our offerings. We continue to push the limits of treatment. In the future we will have the ability to treat more children and more complex neurological diseases and make them even better. I’m really excited about our ability to bring these new and innovative approaches to solving some very complex issues.”

During his tenure, Dr. Marks remembers fondly certain patients and how to see their lives dramatically impacted. He recalls sisters he treated who went from being bed ridden to being in wheel chairs to dancing at their senior prom and eventually getting married, leading normal and productive lives.

“Those are the stories you look back and say, ‘Man was I lucky.’ You found the magic key for those kids.”

Then her diagnosis of epilepsy at 7 years old changed this “big character” into a shy little girl, afraid of what people would think if she had a seizure in front of them. When she attempted to return to the porch and entertain her cousins, she had a seizure. They thought she was joking and they all laughed.

“I'm just really a friendly person and I love people,” Bonita said. “I feel like a piece of that was taken away because of my epilepsy.”

Now this 36-year-old mother of four refuses to let the disease that defined her for so long do the same for her 10-year-old daughter Francesca.

“Francesca is so vibrant and full of life. I don’t want her to ever lose that and I never want her to feel the way I did,” Bonita said.

Bonita and her husband, Charles, were concerned about the risk of epilepsy for their two older children Jace, now 15, and Trace,13. But with vibrant and healthy boys, the threat of epilepsy was “off our radar” by the time Francesca was born.

When she was 4 years old, preschool teachers told Charles and Bonita that Francesca threw up a few times during nap time. They didn’t mention anything about convulsions during sleep. But when one of the teachers said Francesca wasn’t making eye contact with them after the nap, Bonita’s intuition told her it was epilepsy.

“In my heart of hearts I knew and I didn't want that for my daughter,” Bonita said. “My husband was not really accepting of that idea. He tried to reassure me, ‘It doesn't have to be that. She might just have a stomach ache or a virus.’ I just felt it.”

The family was referred to a neurologist for a sleep study in their then home of San Antonio and within minutes, they saw Francesca’s arm twitch. It affirmed to Bonita what the EEG would eventually show – Francesca did have epilepsy.

Bonita gave into a short period of beating herself up. After all, she of all people should have recognized the signs of epilepsy she thought.

But the self-pity didn’t last long. Instead, Bonita developed a new resolve. She wouldn’t let her daughter fall into the same trap that she did as a child following her diagnosis. She would become an advocate for her daughter.

“There was a fire lit inside of me that had been missing for a long time because it's totally different watching your child go through this than when it’s yourself going through it,” Bonita said. “The comfort I took as a child was at least I didn’t remember the seizures after they happened. Even though people would tell me, I could get past it eventually. But this is different, seeing it and then it being your child. The time I’m waiting for her to breathe and take that breath … Just for the seizures to stop … it seems like an eternity.”

Following Charles getting his law degree, the Ocampo family returned back to their hometown of Fort Worth. Francesca began seeing Scott Perry, M.D., an epileptologist and medical director of the John and Jane Justin Neurosciences Center at Cook Children’s.

Dr. Perry upped Francesca’s dosage of medicine and has been her doctor since 2013. And Francesca tells her mom all the time how funny he is.

Laughter comes easier to the Ocampo family now. Francesca is the proud older sister of Beau, 3 years old, and is doing well with her epilepsy.

“Francesca’s outlook on life is exactly what we all want for our patients. She won’t let her epilepsy define her, she takes care of herself and she enjoys just being a kid,” Dr. Perry said. “As neurologists, it is our job to help patients and their families get their lives back.”

“The things I have dealt with in my life and even in my adulthood because of epilepsy have been difficult, including depression and anxiety.” Bonita said. “What’s changed my outlook on my own epilepsy is wanting to be an example of life and not an example of fear for Francesca. But because of the way she lives her life, Francesca has inspired me.”

Francesca describes herself as “just a big fireball” who refuses to let her condition get her down. She’s began the Fort Worth Academy of Fine Arts this year and plans to use November, Epilepsy Awareness Month, as an opportunity to explain to her new classmates about living with epilepsy.

“It’s not that I don’t care that I have epilepsy,” Francesca said. “I just feel I’m a normal person like everybody else. I take meds and I have to go to the doctor sometimes and get checkups. What’s happening in here, in my brain, is not epilepsy. It’s just my normal brain. There are just some tweaks to it that makes it kind of weird. I don’t have anxiety at all. The only time I get a little sad is maybe when I have seizure. I’m usually always fine.”

Someday, Francesca wants to sing and be in musicals. But it’s her bold outlook on life that’s helped her mom change the role she’s played since a child from being “scared of my life to stepping out” in the forefront.

Francesca asked Bonita to join her in the pool last year. Bonita told her she’d never learned to swim because she was afraid she would have a seizure in the pool and people would see her. Francesca told her mom not to be afraid and she would teach her how to swim. A year later, Bonita is a swimmer.

Bonita’s drive now is to raise awareness for epilepsy. She wrote a letter to the city of Fort Worth earlier this year that culminated with a proclamation for the local Epilepsy Foundation. She researched to contact the right person at Sundance Square to get the city to go purple for Epilepsy Awareness Month in November and reached out to Cook Children’s to do the same.

“I was so proud of the Ocampo family for helping raise epilepsy awareness and I’m equally proud of the medical center for supporting their efforts,” Dr. Perry said. “Sure, our primary goal as physicians is always to help patients become seizure free, but even more important than that is making sure their quality of life is the best it can be. Part of that goal is making sure everyone is educated about epilepsy, so that fears and misconceptions about the disorder are erased.”

And Bonita plans for this to be only the beginning as she wants people to know what life is like for people living with epilepsy.

All of this because a daughter’s condition has given her mom a new outlook on life that she thought had been lost on her grandmother’s porch nearly 30 years ago.

“Francesca is everything, the very embodiment, I wished I could have been,” Bonita said. “It’s not a vicarious, living through my daughter type of thing. I just don’t want that wonderful sense of fun to fade away in her. Francesca has helped me so much. That’s the cool thing about us. By me not wanting her to ever get to that point of fear and anxiety while wanting to be more of an example to her, I have been put in a position where I have to step outside what I was. Where I did feel like it defined me. I don’t feel that way any longer. This is now just who we are. This is us.”

Cook Children's Epilepsy Program

If you have a child with epilepsy, you're not alone — 2.5 million Americans have this disorder. The National Association of Epilepsy Centers recognizes Cook Children's Comprehensive Epilepsy Program as a Level 4 Pediatric Epilepsy Center. Level 4 epilepsy centers have the professional expertise and facilities to provide the highest level medical and surgical evaluation and treatment for patients with complex epilepsy.Click here to learn more. If you would like to help, please visit our giving page. 

It can be a hard thing for adults to deal with. Unfortunately, many children suffer the same pain. For some, it’s a genetic condition that’s been passed along to them. But for others, migraines are the result of injury. For 15-year-old Olivia Gillespie, her migraine problems began when she received her third concussion.

“It happened in cheerleading practice, I was kicked in the head,” says Gillespie. “I was fine. I didn’t think it was that big of a deal.”

Then months later, Gillespie was on a ski trip with her youth group when a headache came on and never went away.

“I just kept doing what I was doing and tried to not let it have an effect on me,” she said.

Gillespie tried to ignore the pain for two months before she said anything to anyone, but then the pain got worse.

“Olivia had an ongoing headache that would not fade. On a scale of one to 10, it was a consistent five," said Amelia Gillespie, the teen's mother. "It wasn't until her pain reached level seven that it really began to affect her.”

When Cynthia Keator, M.D., pediatric neurologist at Cook Children’s, first saw Olivia, she wanted to make sure her headaches could be treated without medication. Together, they worked on her sleep habits and diet. Dr. Keator also suggested supplements such as B2, and Omega 3, which has shown to help with headaches. In addition, Gillespie was referred to the Biofeedback Clinic at Cook Children’s.

“Biofeedback teaches patients how to control their pain by controlling their breathing, heart rate and body temperature,” says Dr. Keator.

Biofeedback specialist Nanny Christie, Ph.D., has been teaching the painless technique for decades.

“We place the child in a reclining chair and use pads to measure their body activity,” says Christie. “The equipment plugs into a laptop so the kids can see everything on the computer screen. If they tighten a muscle, a line goes up. If they relax, the line goes back down.”

Christie says teaching children to harness their breathing and muscle movement this way allows for real time learning. She says about 80 children are seen in her clinic each month. A typical visit is 45 minutes to one hour and each child is seen roughly four times.

While it wasn’t the end-all, be-all cure for Gillespie, it did help. She says she uses the technique to curb her head pain. If she feels a headache becoming worse, she can focus and calm her anxiousness which in turn can help the headache.

Also, ask your doctor about preventative medication. Your doctor may prescribe something prescription strength or a natural supplement to ease the pain.

Make sure you DON’T ask your child this question:

“How’s your headache?”

You may want to, but remember that asking about a headache is like scratching a mosquito bite. By asking, you’re reminding your child about their pain.

I am doing great! I continue to heal more every day and have come such a long way since the accident on February 16, 2014 and my discharge from Cook Children’s on May 9, 2014.

It is hard to believe that it has been more than a year and half since that horrific night that has forever changed my life.

After being released from Cook Children’s I immediately began outpatient therapy four days a week in Denton, Texas. I was able to graduate with my high school class at the end of May 2014, I walked across the stage and received my diploma, with a very emotional standing ovation from everyone in attendance. In July, I took a college course at North Central Texas College (NCTC) in Gainesville, which is about 20 minutes from my hometown, all the while still attending outpatient therapy.

My parents, sisters, aunts and grandparents arranged for my transportation to and from everywhere.

In August 2014, I underwent surgery to release the contracture in my left Achilles tendon. Then started the Fall 2014 semester of college at NCTC taking six credit hours and by this time my outpatient therapy was down to three days a week.

In late October 2014, I was cleared to drive again and what an amazing feeling that was to be able to have my driving independence back. I completed the Fall 2014 semester at NCTC and registered with Midwestern State University in Wichita Falls, Texas for the Spring 2015 semester. In January 2015 I moved into the dorm at college taking nine credit hours. I was still in physical therapy a few days a week so we transferred my therapy from Denton to Wichita Falls which I completed in February 2015.

I moved back home over the summer and underwent further reconstructive surgery on my left thigh in June. I traveled to New York mid-August with my family for vacation, then upon return immediately moved into an apartment with two of my friends in Wichita Falls to start the Fall semester of classes. I am currently a sophomore at Midwestern State University taking 12 credit hours. I have some little things, everyday things, I have to work harder to achieve, but have and I'm adapting.

I changed my major the other day, but still plan on being in the medical field. Tackling Anatomy and Physiology this semester is a challenge. No one is lying when they say it is hard, requiring a lot of memorization.

My short term memory is one thing that is still not how it was before the wreck, but continues to get better every day. I just have to study more and use note cards, color coding, and anything else that will help me remember the material. In Spring 2016 I will apply for the Respiratory Care program at the University with plans of obtaining a Bachelor of Science in Respiratory Care.

I am so very Blessed to have been placed in Cook Children’s Medical Center for my inpatient recovery in the Neuro Rehabilitation Center and in the care of Dr. (Fernando) Acosta and the team of doctors, nurses, therapists and aids. Their knowledge, skills and encouragement with me throughout my recovery along with Faith, Prayer’s and so much support from my family and friends are a direct relation to my successful recovery. 

Click here to read Abby's original story.

Who were they kidding? There's no emotional prepping anyone could do at that time. So, off to the OR for delivery we went. Talk about scared! They gave my baby a 10 percent chance of survival and they gave me a death sentence if they didn't deliver right then and there.

Graysen was brought into this world three months early weighing 1.4 pounds and not crying, or breathing. I remember seeing that he was the size of the nurse’s hand when she was working on him. I got to take one look at him before they had to intubate him immediately to get him breathing, since he was turning blue.

They then hurried away with him to the delivering hospital’s NICU. He was supposed to be born on March 15th (spring break baby) and he came into this world right before Christmas. With only a diaper the size of a tiny flip cell phone (which was the phone we had 10 years ago!), and under a heat lamp for warmth, inch by inch, ounce by ounce, he grew.

Weeks into life, the doctors decided to start feeding him by NG tube (Nasogastric tube that runs through the nose and into the stomach for feeds).

He did fine tolerating the feeds until he got an infection in the intestines, called necrotizing enterocolitis, also known as Nec. This made his belly swell up and, if not cured properly, could have resulted in a hole in the intestines, which is fatal. Surviving Nec was thought to be low. The doctors called to inform me that he was not doing so well and needed to be transferred by Teddy Bear Transport to the Medical Center’s NICU.

They loaded him up and took him by ambulance after two months in the NICU where he was born to begin his next few months of growing. Cook Children’s slowly nursed him back to better health, and, luckily, Graysen did not need surgery on his intestines. Miraculously, he pulled through another obstacle.

Weeks went by before they attempted to feed him again. In the meantime, the doctors had a central line surgically put into his chest so he could receive his nutrients properly. After a few weeks of feeds, the doctors started to see major improvement. They took him off the respirator and put him on a high flow nasal cannula to help with Graysen’s oxygenation and breathing. He did great with this new machine. Now breathing well, it was time to introduce the bottle at around 3 months. He took it, but not all of it.

The doctors had to decide what to do about the feeds he was leaving behind. They decided to put a G-button surgically into his stomach so that the left over feeds could be received by tube. This was the turning point. The hardest decision I had to make. Once that G-button was placed, I would have a medically dependent child.

After being in the NICU for five months, this was the only way he would be able to come home, and I was ready for that day. I gave the doctors the approval, and off to his first surgery he went. That was the first of 14 surgeries he would have throughout his first 10 years of life. Surgeries followed including fundoplication (an operation to prevent stomach contents from returning to the esophagus), hernia repairs, tonsillectomy, and a tethered spinal cord repair. Just to name a few. His spinal cord was taut at the end and neurosurgery was scheduled at 12 months. He caught meningitis after the surgery and, once again, beat dangerous odds.

Graysen went through so much that he didn’t eat. For six years, he was completely tube fed. He went through many years of intensive feeding therapy. He still didn’t want to eat orally. I put him in kindergarten and he saw his peers eating by mouth. That sparked an interest and, at 6 years old, he started eating. Now he demolishes whole cheeseburgers and fries! Not only has he beat death numerous times, he's gone through 14 surgeries and countless doctor appointments getting him to where he is now. This kid is here for a reason. This month we celebrate Graysen's 10th year of LIVING! Graysen is my Christmas miracle.

I could not have done this alone. I am so thankful that I had, and still have, a great network of people working together at Cook Children’s. Without their dedication to their job and to children, I don’t know if Graysen would have made it. Dr. Nancy Dambro was one of his main doctors from the time Graysen was born. Dr. Michael Deitchman has been his pediatrician through all the rollercoaster ups and downs. I can’t thank him enough for his support, countless visits and patience with us. We also see Dr. Jose Iglesias, Dr. Jill Radack, Dr. Bankole Osuntokun and Dr. Fernando Acosta.

It takes a village in Graysen’s case, and I’m glad our village is Cook Children’s!

Still, Dr Keator takes precautions.

She states, “I suffer from migraines, as due several of my colleagues. Most migraine sufferers have busy lifestyles, and a main trigger is fatigue. Therefore, what I tell my patients and also practice myself: exercise regularly, stay well hydrated, eat well and get adequate sleep. Another very important recommendation is to limit time on electronic devices. I do my best to minimize migraines and headaches by taking frequent computer breaks.”

Most professional and educational activities are spent on some sort of electronic device, and in today’s world, introduction to electronic devices starts at a very young age. Media is everywhere and it is competing for children’s attention.

Statistically speaking, over 75 percent of children have some degree of hand-held or electronic devices. A great portion of the child’s day is spent on these devices, which by default, take away from other activities. Headaches and/or migraines in children can be exacerbated or increased due to spending excess time using electronic devices: watching TV, video games, texting, playing on tablets, etc.

The concern is that the time spent (and the content) on the devices may interfere with other regular childhood activities and natural sleep/wake cycle, especially when used in excess. Dr Keator states, that when children use electronic devices in excess, this leads to interruption in sleep which will lead to fatigue, and for many children it can lead to headaches/migraines. It can also lead to attention problems, school difficulties, eating disorders and obesity. Prolonged time spent on electronic devices interrupts other activities: homework, dinner time, family time, exercise, and sleep.

The American Academy of Pediatrics recommends limiting or completely eliminating screen time for children under the age of 2 years. Then as children get older, media is slowly introduced. For children age 3-5 years, limit to 30-60 minutes; children age 6-9 years 60-120 minutes a day; and for older children over 120 minutes is allowed.

But let’s be realistic; these time limits no longer fit in our current world of technology.

“Technology is here and electronic devices are a multi-billion dollar industry and it is not going anywhere,” Dr Keator states. “In neurology, our recommendation is time limits. The younger you are, the less time that is allowed. However, everyone needs breaks regardless of the age. Try to limit time on electronic devices to 15-30 minute increments followed by breaks. During the breaks, I recommend doing something outside, spending time with friends or family, or just resting; then resume activity on the particular device.”

Dr Keator does acknowledge that many schools are incorporating computers or touch screens into the academic curriculum. For children who are migraine or headache suffers, she recommends getting up and relaxing the eyes and giving the brain time to rest.

“It’s a balance,” she emphasizes. Parents and teachers have to work on positive media/electronic use with time limits and breaks. Media/electronic devices should not be in the bedroom and this includes smart phones. Make dedicated family time without electronics. When electronics are used, make sure it is monitored and make it educational as much as possible.

More about Dr. Keator

Dr. Cynthia Keator joined Cook Children's Neurosciences in July 2012. She is board-certified by the American Board of Psychiatry and Neurology with special qualifications in Child Neurology and sub-specializes in pediatric epilepsy. A good portion of her clinical practice in spent in the Epilepsy Monitoring Unit at Cook Children's Medical Center where she diagnoses and treats patients with epilepsy. 

Dr. Keator is a native of Fort Worth where she was raised and attended Trinity Valley School for 13 years. Uniquely, she volunteered at Cook Children's when she was in high school. "I can truly say that it was my volunteer experience that attracted me to pediatrics andCook Children's. Now having finished my training, it was an easy decision to choose Cook Children's". Dr. Keator completed her undergraduate training at Texas A&M University and then obtained her medical degree at the University of Texas Health Science Center at Houston.

Dr. Keator's husband is also a native of Texas. They live in Fort Worth with their German Shepherd. Dr. Keator enjoys spending time with her family who still reside in the DFW area.

While I have had previous experience addressing academic issues with my patients, my son is now in elementary school, so I am developing a whole new perspective. I still do not understand all of the intricacies of special education, but I am lucky that I have colleagues who I can contact with questions concerning my own son, as well as my patients. 

The process is incredibly complex and confusing! Despite all of my knowledge, I also admit to sometimes feeling intimidated during school meetings. I frequently help the parents of my patients locate special education advocates because it is very easy to feel that your child's needs are not being addressed they way you would like at school.  Although maintaining a strong cooperative relationship with the whole team of professionals who work with your child is important, it is also sometimes necessary to fight more for your own child. That line, however, is not always clear, and all parents need to trust their instincts.

I considered leaving pediatrics after my son's diagnosis because I was not sure that I would be able cope with developmental disabilities at work and home. However, I soon realized that I would not ever really be able to escape my new role as a "special" mom. Instead, I made the decision to embrace the situation, which eventually led me to Cook Children's and specializing in pediatric stroke in my professional and personal life. 

Although having a child who is a stroke survivor does not make me a better neuropsychologist, it does give me more insight into the day-to-day struggles that my patients and their families face. I know that, in addition to the medical and school issues, my patients and their parents may be dealing with complex family, emotional, and financial issues that go along with having a child with special needs. If you do it right, being a parent to any child is a difficult job, and we all have moments when we look back and wish we had handled a situation differently. 

I have learned that it is extremely important for parents to take care of themselves, especially when their children have complex issues, because a child's needs are best met by a healthy parent.

Related Links

• A stroke at birth - part 1
• Stroke at birth - part 2
• Stroke at birth - part 3
• Cook Children's Stroke and Thrombosis Program

About the author

Carla Hearl Morton, Ph.D., is a pediatric neuropsychologist at Cook Children's. She is a licensed psychologist with expertise in how learning and behavior are connected to the development of a child's brain structures and systems. At Cook Children's, neuropsychologists work closely with a team of neurologists andneurosurgeons to provide the best treatments and interventions that meet the individual needs of each child.