CHOC Patient is Succesfully Treated for Feeding Problem

CHOC Children's Multidisciplinary Feeding ProgramBefore coming to CHOC Children’s, Pacer Lybbert had never eaten a Cheerio, a piece of toast or even a spoonful of yogurt. He was almost 4 and had never enjoyed birthday cake, Halloween candy or a Thanksgiving dinner with his family. Since birth, Pacer had received almost all of his nutrition through a feeding tube.

Quinn and Mekell Lybbert may never know why their son, now 7, was born unable to swallow. Everything was fine at first, but within a couple of weeks it was clear their baby was struggling to eat. Finally, there was no choice but to put a feeding tube through their infant’s nose. It was supposed to be a short-term fix, but as Pacer grew older, and efforts to help him failed, he had to have a gastric tube inserted directly into his stomach.

Quinn and Mekell had resigned themselves to the possibility that Pacer would need a feeding tube for life. Then, they met a little boy near their new home in Montana who had been successfully treated for a similar problem at the CHOC Children’s Multidisciplinary Feeding Program. Mekell immediately called CHOC.

Five weeks after coming to CHOC, Pacer’s feeding tube was removed. He left for home eating equal parts solid food and a liquid nutritional supplement. Six months later, Pacer was eating regular food with his family — and asking for seconds.

“The interdisciplinary approach is what made this program so different and why it was so successful,” Mekell said. “We had so many people working together as a team trying to figure this out. Had we not gone to CHOC, Pacer would still have a feeding tube.”

See Pacer’s brave journey in this video.

Learn more about CHOC Children’s Multidisciplinary Feeding Program.

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Kara’s Story: A Journey with Epilepsy

Heidi Sexton knew her young epileptic daughter, Kara, needed more help. Anti-seizure medications didn’t help, hospital visits were frequent, and seizures and tantrums continued.

“It was time,” Heidi recalled. “I went in to the next doctor’s appointment with my questions: What else can we try? This isn’t working.”

Dr. Mary Zupanc, director of CHOC Children’s comprehensive epilepsy program, offered her recommendation: Kara needs brain surgery.

A little girl’s long road

Though no doubt a staggering consideration, brain surgery would be the next landmark on a long medical history that belied Kara’s short three years of life.

At just 13 months old, she underwent emergency brain surgery after her frontal lobe hemorrhaged, caused by a previously undiscovered vascular malformation. The hemorrhage in Kara’s brain led to right-side hemiplegia, leaving her no use of her right hand and limited the use her right leg.

Kara underwent physical therapy and began walking at age 2. Life was relatively normal for the Sexton family until just after Kara’s third birthday, when she began acting strangely.

Kara’s body would stiffen, she’d fall down and stare off into space, Heidi recalled. It wasn’t until a visit to the Julia and George Argyros Emergency Department at CHOC Children’s Hospital that the Sextons would learn Kara was having seizures.

At the CHOC Children’s Neuroscience Institute, Kara underwent an electroencephalogram, or EEG, a test that monitors for electrical activity in the brain. Next, she spent six days undergoing long-term video EEG monitoring. From there, Dr. Zupanc, also CHOC’s neurology division chair, made an official diagnosis of epilepsy and prescribed an anti-epileptic medication.

Exploring other options

Nonetheless, Kara’s seizures continued. Kara’s three older siblings were acutely aware of their sister’s disorder, daily counting Kara’s seizures and agonizing over each hospital visit. The family began to placate Kara in hopes of staving off outbursts and tantrums, Heidi said.

“Kara had between two and four seizures a day,” she said. “It really messed with her behaviorally. She’s a lovely kid, but she suffered serious temper tantrums. It was physically and emotionally exhausting.”

A second six-day monitoring session led Dr. Zupanc to change Kara’s medication and, as a precaution, begin evaluating her as a candidate for epilepsy brain surgery.

Further tests and scans revealed that the left hemisphere of Kara’s brain was significantly injured and atrophied – likely related to her earlier hemorrhage. In addition, Kara’s new anti-epileptic medication wasn’t working.

Dr. Zupanc concluded that the next step was a hemispherectomy, a surgical procedure in which one side of the brain is removed or disconnected.

Kara

“On scans, Kara’s hemisphere was completely white,” Heidi said. “The only thing her left hemisphere was doing was causing seizures. There was no question.”

A change of course

Nearly 11 months after the seizures first surfaced, Kara underwent surgery at CHOC to remove a portion of her brain’s left side, and disconnect the remaining portion from the right hemisphere. Performed by neurosurgeon Dr. Joffre Olaya, the procedure lasted six hours.

After 16 days recovering in the hospital’s pediatric intensive care unit, Kara went home just after her fourth birthday.

Much to her parents’ and siblings’ relief, Kara hasn’t had a seizure since her surgery eight months ago. Kara still relies on an anti-epileptic medication, but she may be able to stop taking it completely or use a lower dose of medication soon if her progress continues.

Though considering a second brain surgery was daunting for the Sextons, Heidi and her husband knew the procedure was the right choice to help their daughter.

“I looked at it in a sense of quality of life for Kara,” Heidi said. “Hopefully now she’ll be able to drive one day. Continued seizures would have impaired her cognitively forever, and I didn’t want that.”

Though she still requires extensive care, Kara is doing well physically and emotionally and is looking forward to preschool.

Kara isn’t fully aware of her journey in the last year, but she understands the meaning of the scar on her head just above her ear.

“She knows now that she doesn’t have seizures and Dr. Olaya and Dr. Zupanc fixed her,” Heidi said.

Read other stories about CHOC patients:

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CHOC’s Small Baby Unit Helps Twins’ Succesful Outcome

On March 17, 2010, CHOC Children’s opened its Small Baby Unit within the neonatal intensive care unit, to care for the unique needs of the smallest and sickest babies. Since then, the unit has delivered specialized, high-quality care to close to 300 neonates. In honor of the unit’s fifth anniversary, read about one of the many remarkable patient stories from this special place.

For twins Damian and Victoria Daboub, timing is everything. They made their debut too early — at 27 weeks. Damian was 2 pounds, 14 inches, and Victoria was 2 pounds, 13 inches. Yet their timing was perfect because they arrived just as CHOC Children’s opened its 12-bed Small Baby Unit in March 2010.

Designed expressly for low birth weight babies born at less than 28 weeks or weighing less than 1,000 grams, the Small Baby Unit has its own dedicated team of specialists and is the only one of its kind in Southern California. Further, the unit is designed to aid in the babies’ development with dim lighting and low noise levels.

Damian stayed in the Small Baby Unit for 85 days, and Victoria was there for 105 days. Mom Miriam credits the Small Baby Unit for the twins’ survival. Today, they are “off the charts” in terms of height and weight, and are gregarious, joyful preschoolers. While their language is a bit delayed, it doesn’t stop them from enjoying all of their activities. Miriam truly believes her children’s successful outcome is the direct result of this “little wing where they can focus on growing.”

Learn more about the CHOC Small Baby Unit.

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Physician Tenacity, Experimental Treatment Help Baby With Rare Disease

A toddler with a devastating rare condition has a chance for health, thanks to an experimental treatment and the tenacity of a CHOC Children’s physician.

Galya Chan, 2, is showing marked improvement after nearly nine months of regular infusions of cyclodextrin, a compound that has helped mice with Niemann Pick C disease, the condition also affecting Galya.

Dr. Raymond Wang, a CHOC metabolic disorders specialist, says Niemann Pick C affects one in every 150,000 people and causes cholesterol to accumulate in the brain, lungs, liver and spleen, leading to deterioration and early death.

Even worse, there is no Food and Drug Administration-approved treatment for the condition.

A sick preemie

After a premature birth, Galya developed an extremely enlarged liver and severe jaundice. A battery of tests led to a full exome sequence, which revealed Niemann Pick C – and no available treatment plan.

“The diagnosis is horrible, but it’s better to know than to not know,” says Brian Chan, Galya’s father.

But Dr. Wang was aware of researchers studying the effects of cyclodextrin in animals with Niemann Pick C. The compound had shown to reduce cholesterol levels in their bodies, help animals survive without symptoms longer and have more overall longevity.

“The problem is there’s no company that’s willing to get behind the treatment for humans,” Dr. Wang said. “There aren’t enough patients with this condition for companies to see the potential and profit for this. It comes down to individual physicians who want to make a difference.”

Taking another route

So, with Galya’s parents in agreement, Dr. Wang began the long process to seek special permission to treat the baby with cyclodextrin.

Writing a treatment protocol especially tailored for Galya, Dr. Wang filed an investigational new drug application with the FDA. CHOC’s Institutional Review Board, a body that examines proposed research, also reviewed the proposed use.

Four months later, Galya became the youngest of just 11 patients nationwide to undergo this treatment.

Currently, Galya undergoes weekly intravenous cyclodextrin infusions. She also receives monthly intrathecal infusions, which are administered through a lumbar puncture to reach her central nervous system.

Each time, Galya is admitted to CHOC’s pediatric intensive care unit. Infusions last six hours, and she stays 15 more hours for observation.

Encouraging outcomes

But the time and effort is paying off: markers of Galya’s cholesterol storage levels have dramatically decreased since she began treatment nearly nine months ago, data shows.

Before receiving the cyclodextrin, those markers were tremendously elevated, more than six times the normal level. Today, her storage levels have dropped more than 80 percent and now hover just above normal, with progress expected to continue. Also, Galya’s liver softened and its volume decreased by about 10 mL.

Galya’s results are so good that Dr. Wang is working to adjust her treatment protocol to receive intrathecal infusions twice monthly.

Moving forward, Dr. Wang and his colleagues will continue to study Galya to determine if the treatments are also healing her lungs, and it’s likely the cyclodextrin treatment will continue indefinitely – or until a different treatment is developed.

“Once we found out the diagnosis, it was sad,” says Brian, Galya’s father. “But now we can put our energy into helping Galya and working to find a cure.”

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Former CHOC Patient is Cancer Free Thanks to Da Vinci Robotic Surgical System

Julia Shenkman is a healthy and accomplished 17-year-old. She has a second-degree black belt in Taekwondo, is a member of her high school’s science team, and is fourth in her senior class in academics. Julia has another distinction: She’s a former CHOC Children’s patient who underwent a surgical procedure that involved the use of the then-landmark Da Vinci robotic surgical system.

In late 2003, CHOC became the first pediatric hospital in California to begin offering robotic surgery for children. The Da Vinci robot was an improvement over minimally invasive laparoscopic surgery because it gave surgeons a better view inside the surgery site and allowed them to more precisely manipulate instruments, which translated to less pain, fewer complications and shorter recovery time for patients.

In January 2006, Julia, then 8, was whisked to the emergency department at St. Joseph Hospital for severe abdominal pain and frequent vomiting. Blood work and X-rays for appendicitis turned up negative. It wasn’t until Julia’s doctor at CHOC, Mustafa Kabeer, pediatric surgeon, ordered an ultrasound that the cause of her distress was found: a large mass in her right ovary. Using the Da Vinci system, Dr. Kabeer operated on Julia and successfully removed the tumor, which was malignant. After three months of chemotherapy, Julia recovered and remains cancer free.

Julia with Dr. Mustafa Kabeer, CHOC Children's pediatric surgeon.
Julia with Dr. Mustafa Kabeer, CHOC Children’s pediatric surgeon.

Pediatric surgeons at CHOC have performed about 100 robot-assisted surgeries, says Dr. Kabeer, who before coming to CHOC helped implement the world’s first pediatric surgery robotics program at Children’s Hospital of Michigan in 2001. Recently, Dr. Kabeer started using the Da Vinci system to perform complex operations through one incision in the belly button, such as for removal of the gallbladder. The Da Vinci system at CHOC was made possible by a $1.5 million sponsorship from Costco Wholesale Corp.

“This type of technology helps us perform a wide variety of surgeries — some of them very complex,” Dr. Kabeer says. “It takes time, commitment and vision to start and maintain such a program, but this is one of the unique things about CHOC: We want to do this, and we are committed to doing it.

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