Wired for hope

Every morning when she awakes, Sydney Amato begins her daily battle with her body.

If she’s lucky, the 16-year-old will have gotten a handful of hours of uninterrupted sleep – dreaming, perhaps, of doing what most healthy kids her age take for granted:

Hanging out with friends. Going to school. Learning to drive.

Because of a neurological condition called dystonia, Sydney, who is in excellent cognitive health but speaks and walks with some difficulty, suffers from involuntary and near-constant contraction of muscles in her neck, arms, legs and trunk.

Her mind is unable to control the painful jerking that makes most of her body twist and go rigid, her muscles moving out of normal sequence.

Born a right-hander, she can feed herself with some struggle using her left hand. She wants to dress and put on makeup herself, but those normally simple tasks become lengthy ordeals.

“My body fights me all the time,” says Sydney, trying to distract herself in her hospital bed one morning by watching an old episode of “Keeping Up with the Kardashians.” Listening to her favorite music – Ariana Grande, Lauren Daigle, Drake – can only temporarily transport Sydney away from her debilitating condition.

“She knows what she wants to do,” says her father, Louis. “But her body won’t let her.”

Specialists at CHOC Children’s are working hard to change that.

CHOC patient and her father
Sydney, pictured with her father.

A first for CHOC

Recently, a team led by Dr. Terence Sanger, a physician, engineer and computational neuroscientist who joined CHOC in January 2020 as its vice president of research and first chief scientific officer, and Dr. Joffre E. Olaya, CHOC’s functional restorative neurosurgeon, implanted several temporary electrodes into Sydney’s brain.

Dr. Terrence Sanger, chief scientist at CHOC Children's

The surgery marked the first time a patient with a movement disorder at CHOC underwent a procedure called deep brain stimulation (DBS).

Working in perfect harmony as a team, Dr. Sanger and Dr. Olaya oversaw the first portion of a three-stage surgery on Sydney. As the surgeon, Dr. Olaya placed the leads following advice from Dr. Sanger, the neurologist, on where they should go.

 In the procedure, millimeter-thick electrodes were precisely positioned into the basal ganglia region of Sydney’s brain – about 3 inches deep. The surgery involved the use of the ROSA robot, the same tool that has been used during brain surgery on epilepsy patients at CHOC since 2015.

Considered one of the most advanced robotized surgical assistants, ROSA — which stands for robotic operating surgical assistant — includes a computer system and a robotic arm. It’s a minimally invasive surgical tool that improves accuracy and significantly reduces both surgery and anesthesia time.

The ROSA robot helped with implanting and targeting the electrodes and a portable operating-room CT scanner confirmed their position.

Turning down the volume

 DBS is designed to ease Sydney’s condition by sending electrical currents to jam her malfunctioning brain signals.

Think of turning down the volume on your car radio.

“Nobody really understands the cause of dystonia,” Dr. Sanger explains, “but there’s probably too much electrical stimulation going on in the motor areas of the brain. We’re trying to calm down that extra noise.”

Although DBS dates to the 1960s, it wasn’t until the 1980s that the modern era of using it to treat adult patients with tremor and Parkinson’s disease began.

In 2000, Dr. Sanger, working with engineers, data scientists, neurosurgeons and others, began implanting electrodes in pediatric patients.

Instead of the established method of placing the leads at predetermined sites and hoping they worked, Sanger and his team, just as they did in Sydney’s case, placed temporary leads to best assess where they should go permanently based on patient response.

In 2016, Dr. Sanger began honing DBS to treat children with dystonia. Before the surgery on Sydney, Dr. Sanger had overseen DBS on 26 children using the same three-stage technique. He says 80% of those children have seen successful results.

Early signs

Sydney began showing symptoms of dystonia – tremors in her hands – when she was 5 ½ years old.

A year later, she was using a wheelchair. She had her first brain surgery at age 7.

Since then, “she’s been all over the U.S.” seeking the right treatment for her condition after several setbacks, her father says.

But her condition was not improving.

Early this year, a neurologist in Kansas City, Missouri, recommended that Sydney see Dr. Sanger.

“I asked him, ‘If Sydney was your kid, where would you go?’” Louis Amato recalls. “He said, ‘Hands down, Dr. Sanger.’”

The COVID-19 pandemic pushed Sydney’s surgery to mid-August.

Sydney already had two electrodes in her brain that were only partially working when she came to CHOC in early August for surgery.

After two extensive run-throughs with their team, Dr. Sanger and Dr. Olaya, in a six-hour procedure that at one point required nearly 20 people in the operating room, implanted more electrodes to give Sydney a total of nine.

Dr. Joffre Olaya, a pediatric neurosurgeon at CHOC Children's
Dr. Joffre Olaya

After surgery, optimism

On Thursday, Aug. 20, six days after Sydney’s surgery, Dr. Sanger stopped by her room at CHOC Children’s Hospital. The room was decorated in purple, Sydney’s favorite color.

Dr. Sanger greeted her as CHOC staff members, joined by members of Sanger Lab, Dr. Sanger’s research laboratory that conducts research in pediatric movement disorders, prepared to have Sydney walk back and forth down a hallway while connected to electrical equipment programmed to record signals in her brain and muscles.

A thick coil of multicolored wires snaked from under a large white bandage covering Sydney’s head. Extending about 6 feet, the wires were plugged into specialized recording equipment controlled by Jennifer MacLean, a CHOC pediatric nurse practitioner whose job was to manipulate the strength of electrical charges affecting the four points of contact on each electrode.

The goal: determine which charges worked best and on which electrodes.

“It could have turned out that the DBS procedure made no difference,” Dr. Sanger says. “But we’ve seen a very good response in Sydney.”

For example, her once mostly useless right hand was working much better.

“It gives you goosebumps,” Louis Amato says.

After taking a bite of a veggie burger and sipping some water, Sydney started to walk.

Following her were seven CHOC and Sanger Lab specialists.

“Go nice and slowly,” Jennifer told Sydney. “You’re going too fast for us!”

Perhaps Sydney was anxious to get back to riding Tigger, a quarter horse, in her hometown of Carthage, Missouri. She has been riding him for six months.

CHOC patient horseback riding
Sydney is eager to get back to riding her favorite horse, Tigger.

“Her balance isn’t bad on the horse,” says Louis Amato.

Sydney also loves to tan by her pool and swim.

What she wants most, however, is to be freed from her body so she can return to school and do what most teens enjoy.

“It’s stressful,” says her mother, Angie. “She has a lot of friends her age, but she can’t do a lot of the things they do. She has her days when she can get really upset.”

Now, however, working with Dr. Sanger, Dr. Olaya and the entire team at CHOC, the Amatos are more optimistic than ever.

“We’re hopeful that this is going to be a big life-changer for her,” Angie Amato says. “That would be the best thing that could ever happen – better than winning the lottery.”

‘The A-Team’

After crunching numbers for a week to assess which of the nine electrodes proved to be the most effective based on how Sydney responded to varying degrees of electrical currents, Dr. Sanger and his team settled on four electrodes that were permanently used to treat her condition – three new ones, and one existing one.

The team performed this second surgery on Sydney in late August.

In the third and final surgery, successfully completed in early September, a rechargeable generator that powers the DBS leads was implanted in Sydney’s chest.

“As we get better and better at this and as the technology progresses, we’ll be able to do this on kids who are less sick than Sydney,” Dr. Sanger says.

Dr. Sanger and Dr. Olaya are poised to dramatically improve the lives of many more patients like Sydney at CHOC.

“I’m really excited that we will be doing more of these procedures to help pediatric patients with movement disorders and significantly improve their quality of life,” says Dr. Olaya. “I look forward to continuing to provide this type of personalized care.”

a CHOC patient poses with her mom
Sydney, pictured with her mother.

Angie and Louis Amato say Sydney has never gotten this much special attention during her 11-year-plus medical journey.

“Here at CHOC,” Louis Amato says, “we feel like we’re with the A-Team.”

Says Sydney: “I’ve never felt this much confidence and this good about treatment before.”

Learn more about deep brain stimulation at CHOC 

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CHOC recognized as one of nation’s best children’s hospitals

best-childrens-hospitals-7specialtiesCHOC Children’s is one of a select number of pediatric facilities nationwide to have been ranked today as a best children’s hospital by U.S. News & World Report.

The following CHOC specialties are honored in the 2020-21 Best Children’s Hospitals rankings: neonatology; cancer; diabetes and endocrinology; neurology and neurosurgery; orthopaedics; pulmonology; and urology. Both orthopaedics and diabetes and endocrinology earned a “Top 20” spot. 

“At CHOC, we are committed to the highest standards of care, safety and service – and this honor reflects that unwavering dedication,” said Dr. James Cappon, CHOC’s vice president, chief quality and patient safety officer and interim chief medical officer. “Not only does this recognition of our excellence in these subspecialties, including two on the top 20 lists, validate our efforts, but it also offers our patients and families additional assurance of our commitment to their health and safety.”

The Best Children’s Hospitals rankings were introduced by U.S. News in 2007 to help families of children with rare or life-threatening diseases find the best medical care available. Only the nation’s top 50 pediatric facilities are distinguished in 10 pediatric specialties, based on survival rates, nurse staffing, procedure and patient volumes, reputation and additional outcomes data. The availability of clinical resources, infection rates and compliance with best practices are also factored into the rankings.

The U.S. News Best Children’s Hospitals rankings rely on clinical data and on an annual survey of pediatric specialists. The rankings methodology factors in patient outcomes, such as mortality and infection rates, as well as available clinical resources and compliance with best practices.

Learn more about Best Children’s Hospitals rankings.

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Beating brain cancer and giving back: Brodie’s story

Brodie’s cancer survivor party was a chance to celebrate the completion of his cancer treatment by gathering his family and friends for an epic Nerf battle. Wanting to give back to other CHOC Children’s patients, the twelve-year-old boy turned his celebration into a fundraiser. Brodie used the proceeds to buy Legos, journals, games—all his favorite things while in treatment—to brighten the days of other children battling cancer.

brodie-shopping-for-donations
Brodie and his brother shopping for gifts to donate to CHOC patients.

“He wanted to buy more of the things that helped break up his time while he was at CHOC,” says mom Megan. “If you ask Brodie what he wants to be when he grows up, he says ‘I just want to make people happy.’”

Brodie’s journey to a diagnosis

Brodie’s path from initial symptoms to a diagnosis was long. While practicing karate at home, his dad Marcus noticed that his left side didn’t have any “oomph.” Over the next few days, he and Megan realized Brodie was losing coordination in his left hand and left foot.

The first few doctors who saw Brodie ruled out a tumor but couldn’t identify what was causing his loss of coordination. An MRI revealed something on Brodie’s basal ganglia—a collection of nerve cells deep within the brain that help control movement. They were eventually told that Brodie had likely suffered a stroke. Later, another specialist thought it might be iron accumulation on the brain.

“Something looked wrong, but nobody could tell us exactly what it was,” said Marcus.

The family spent 18 months crisscrossing the country, seeking out various pediatric specialists on their quest for answers.

During this time, Brodie had another issue—every five months or so, he would go blind in his left eye for a few days. There was no discernible reason for this loss of vision.

Their quest eventually led them to Dr. Raymond Wang, a pediatric metabolic disorder specialist at CHOC Children’s. He was able to rule out a genetic reason for Brodie’s symptoms, and encouraged the family to seek out the care of the CHOC neurology team. The next time Brodie lost vision in his eye, the family headed for the Julia and George Argyros Emergency Department at CHOC Children’s Hospital. Brodie underwent another MRI, but this time from a different angle due to his eye condition.

They found a tumor.

Another starting line

“The news that Brodie had a brain tumor was not the finish line of the 18-month journey we had been on,” Marcus says. “That was actually the beginning of yet another journey, this one at CHOC.”  

The family was introduced to Dr. Ashley Plant, a pediatric oncologist at CHOC Children’s.

dr-ashley-plant-choc-childrens-oncologist
Dr. Ashley Plant, a pediatric oncologist at CHOC Children’s

“Not only was she professional and a top-quality doctor, but she was also very warm and empathetic in that first meeting,” Marcus says. “She even gave my wife a hug, which made a lasting positive impression.”

Dr. Plant explained that Brodie needed to undergo a biopsy to determine whether the tumor was cancerous or not. Dr. Joffrey Olaya, a pediatric neurosurgeon at CHOC, performed the biopsy.

joffre-olaya-md
Dr. Joffre Olaya, pediatric neurosurgeon at CHOC Children’s

“I was scared to death when he went in for the biopsy. But I knew we were in very good hands with Dr. Plant and Dr. Olaya,” Marcus says.

The biopsy confirmed the mass in Brodie’s brain was a cancerous tumor known as a germinoma. Germ cells are the reproductive cells in an unborn baby. Germ cells that grow in an unusual way can become a tumor. Often, those tumors form in the ovaries or testes. Sometimes during an embryo’s development, these cells can migrate to the brain and result in intracranial (within the skull) germ cell tumors.

A doctor unlike the rest

Throughout their lengthy journey to find a diagnosis, Megan and Marcus had met many different specialists. They consider Dr. Plant a guide on their son’s treatment journey.

“We were, and continue to be, super impressed with Dr. Plant because not all doctors are like her, and we’ve seen a lot of doctors,” Marcus says. “That doesn’t mean they’re not good, but they don’t all have the same bedside manner as she does.”

Throughout the course of Brodie’s treatment, Megan and Marcus had a lot of decisions to make. In those difficult moments, Dr. Plant was right beside them.

“She provided us with all the information we needed to make responsible decisions, but didn’t make decisions for us,” Marcus recalls. “She helped us make the ultimate decisions as his parents.”

Brodie’s treatment plan included six months of chemotherapy at CHOC, followed by seven weeks of proton radiation in San Diego.

Post-treatment, Brodie has returned to CHOC every few months for an MRI of his brain and a check-up with Dr. Olaya.

“We were so grateful to have Dr. Olaya in our corner because he’s always on the ball. He’s very sharp and conscientious, but also very caring,” Megan says. “It might tell you something that when Dr. Olaya comes in the room, Brodie jumps up and gives him a bear hug.”

Finding a familiar face at CHOC

While Brodie was admitted to CHOC Children’s Hospital for chemotherapy, he was visited by a child life specialist from The Cherese Mari Laulhere Child Life Department, a group who strives to normalize the hospital environment for children and their families.

Child life specialists can engage patients in medical play to help them understand procedures and make tests less scary, bring their favorite toys and movies to their rooms, and show them amenities around the hospital like Turtle Talk and Seacrest Studios.

Shayli, the first child life specialist to visit Brodie’s room, turned out to be an old family friend.

“She looked familiar, but as soon as she said her name, a lightbulb went off—we knew her!” Marcus said. “Megan and I have been friends with Shayli’s parents for years, and they’re great people. But we hadn’t seen Shayli since she was a baby—and now here she is, taking care of our son.”

Shayli knew that Brodie loved Star Wars, so whenever characters would make special visits to the hospital, she made sure they didn’t leave before making a special stop in Brodie’s room.

Although Brodie spent a lot of time in his room resting during chemotherapy treatments, he made many visits to the child life playroom as well.

“For a while, it felt like we lived at CHOC, and it was great to have the ability to take Brodie to the play room for air hockey, or watch a movie or play a video game,” Marcus recalls. “There’s nothing you wouldn’t do to take your child’s mind off chemotherapy treatment.”

Brodie today

Despite countless doctor’s appointments and treatments over the last few years, Brodie has no fear of hospitals.

“He’s never met a stranger. Everyone he meets becomes an instant friend,” Megan says. “Brodie gets so excited to see Dr. Plant and the rest of his team. If I tell him that he has an MRI coming up, he’ll say, ‘Sweet!’”

With cancer behind him, Brodie is feeling more like himself. He loves Lego sets, playing X-box with his younger brother Finn, and swimming.

Learn more about the Hyundai Cancer Institute at CHOC Children's

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CHOC recognized as one of nation’s best children’s hospitals

CHOC Children’s is one of only 50 pediatric facilities in the nation to earn recognition as a best children’s hospital by U.S. News & World Report. The following CHOC specialties are honored in the 2019-20 Best Children’s Hospitals rankings: diabetes/endocrinology, cancer, neonatology, neurology/neurosurgery, pulmonology and urology. Cancer ranked in the “top 20.”

“The national recognition for CHOC’s cancer program is well-deserved. There’s nowhere else I’d rather have gone through treatment than CHOC,” says 17-year-old Sydney Sigafus, CHOC patient and cancer survivor. “Everyone who works at CHOC cares about you as a person, not just a patient. I was included in every decision and conversation about my care.”

The Best Children’s Hospitals rankings were introduced by U.S. News in 2007 to help families of children with rare or life-threatening diseases find the best medical care available. Only the nation’s top 50 pediatric facilities are distinguished in 10 pediatric specialties, based on survival rates, nurse staffing, procedure and patient volumes, reputation and additional outcomes data. The availability of clinical resources, infection rates and compliance with best practices are also factored into the rankings.

us-news-best-childrens-hospitals-6specialties

“We understand how scary it can be for parents whose children are dealing with life-threatening illnesses or injuries. That’s why we are committed to the highest standards of care, safety and service,” says Dr. James Cappon, CHOC’s chief quality officer. “While we are proud of our accolades, including being named a best children’s hospital, we remain focused on preserving the magic of childhood for all kids, whether they are seriously ill or healthy, or somewhere in between.”

Learn more about the Best Children’s Hospitals rankings.

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Six-year-old Beats the Odds Thanks to Life-Saving Brain Surgery at CHOC

At just 6 years old, Madison Morrison has earned the nickname “Miracle Maddy” after recovering from life-saving surgery at CHOC Children’s. The spunky girl, who loves listening to music and drawing, defied the odds of survival when the flu caused encephalitis (viral meningitis with life-threatening brain swelling). Within 48 hours of being taken to the Julia and George Argyros Emergency Department at CHOC Children’s Hospital, Maddy underwent emergency brain surgery and was placed in a medically induced coma. For the next two months, her parents remained by her bedside in the pediatric intensive care unit (PICU).

Mike, Maddy’s dad, says February 4 marked the beginning of their journey. He and his wife Angel never imagined a trip to the emergency department for vomiting would end with their youngest child fighting for her life. On the evening of her admission, Maddy’s nurse immediately noticed when her patient became unresponsive and her pupils became fixed and dilated – grave findings suggestive of dangerous brain swelling. Maddy was intubated, placed in a coma and underwent surgery to have a device placed in her brain to measure and help reduce the pressure inside her skull. Given the fixed volume of the skull, there is little room to accommodate for brain swelling. As it does, the pressure in the skull increases. Seizures, strokes and even death can occur if the pressure rises significantly.

The pressure inside Maddy’s skull remained very high. Her physicians feared she would not survive. After all medical interventions failed to control the brain swelling and lower the pressure in her skull, and after a scan revealed Maddy was at imminent risk of death, CHOC neurosurgeon Dr. William Loudon presented Mike and Angel with one final measure:  a  decompressive craniectomy, a surgery in which part of the skull is removed to allow a swelling brain to expand beyond the normal confines of the closed skull.

miracle-maddy-and-dr-william-loudon
Miracle Maddy and her pediatric neurosurgeon, Dr. William Loudon

“Dr. Loudon patiently explained everything, including the risks, and assured us he’d care for Maddy as though she was his own daughter,” recalls Mike. “We were naturally frightened, but we had confidence in him and trusted he would do everything in his control to save her.”

Mike adds, “He kept his word. We will forever be grateful to him.”

Maddy remained in a coma as she continued to heal. Her PICU care team became, in her parents’ words, “the protectors.”

“Without ever hearing her voice or experiencing her outgoing personality, the team stood by Maddy’s side to not only protect her and save her life, but to love her. The people in CHOC’s PICU are special. There’s no way to understand the emotion and bonds created in that unit unless you’ve been there and experienced the passion within the entire team and the love they have for their jobs, their patients and their parents,” shares Mike.

The PICU team rallied with Mike and Angel when Maddy came out of the coma. She still had a tough recovery ahead, but with the help of CHOC’s speech, physical and occupational therapists, she relearned to walk, talk and eat.

miracle-maddy-recovers-after-brain-surgery-at-choc
Miracle Maddy recovering after brain surgery at CHOC.

The day Maddy was discharged from the PICU was bittersweet. Her parents were thrilled to be taking her home but sad to say goodbye to the staff who had become part of their family.

Angel explains, “Not only did the people in the PICU save Madison’s life, they were smiling faces to us day in and day out. They were a big part of our encouragement, while sharing in our hope and our worry. I was grateful to leave the PICU but sad to leave so many amazing people.”

Shortly after arriving home, Maddy was living up to her nickname. Walking laps around her home, dancing to her favorite songs and enjoying some Snapchat fun, she was putting her hospital stay behind her and focusing on the joys of childhood. And her CHOC family wouldn’t want it any other way for their “Miracle Maddy.”

Learn more about neurosurgery at CHOC

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    Every morning when she awakes, Sydney Amato begins her daily battle with her body. If she’s lucky, the 16-year-old will have gotten a handful of hours of uninterrupted sleep – dreaming, ...
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