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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February is American Heart Month — Cyrus’ story

Just in time for American Heart Month, learn about our courageous patient, Cyrus, who underwent complex open heart surgery. His successful journey was met by much celebration from his family and his team of CHOC experts.

Hypoplastic Left Heart Syndrome (HLHS) is a rare congenital heart defect, which is 100 percent fatal if left untreated. Sunny and Jereme Brixey knew about their baby’s risks even before Cyrus was born in 2008, having been prenatally diagnosed.

Prior to Cyrus’ birth, the cardiac team at CHOC Children’s already had a plan of action: an approach involving three different open heart surgeries. Three days after he was born, Cyrus underwent complex open heart surgery. He had a second heart surgery at 4 months and a third at 4 years.

After the third procedure in 2013, Cyrus took a long time to recover in the hospital. After being in the hospital for nearly two months due to persistent drainage from a chest tube, Cardiovascular Intensive Care Unit (CVICU) physician Dr. Michele Domico suggested that Cyrus could be discharged home with a portable chest drain. He would be the first patient in the hospital’s history to be sent home with this device. The cardiologists and surgeons agreed and ordered the new portable chest drain for Cyrus, allowing him to go home.

Cyrus, with Dr. Michele Domico, medical director of CHOC Children's CVICU and Choco, at a CHOC Night at Angels Stadium event.
Cyrus, with Dr. Michele Domico, medical director of CHOC Children’s CVICU and Choco, at a CHOC Night at Angels Stadium event.

At home, Cyrus’ parents took care of him and his chest drain so that he could heal, and the drain was able to be removed after a few weeks. This successful procedure was met by a large cheer from Cyrus’ entire CHOC team. For Sunny and Jereme, this expression of joy reflected the care Cyrus received every step of his journey. In addition to the clinical excellence of the medical team, the Brixeys believe the team’s positive, encouraging words, as well as the support extended to the entire family, were a huge factor in his recovery. Since then, the CVICU team has used the portable chest drain in several other patients with great success.

Learn more about the CHOC Children’s Heart Institute.


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Patient Returns to School Symptom-Free

Jaden picWhen Jaden Rascon started fourth grade earlier this week, she didn’t have to worry any longer about her heartbeat suddenly racing – thanks to an innovative procedure at CHOC Children’s.

“I feel good,” the 9-year-old says. “Before, when my heart would speed up, it was very hard to breathe, and it would give me headaches. But now it’s all gone because I got the procedure.”

Jaden recently underwent an outpatient electrophysiology procedure at CHOC to cure her arrhythmia. The experience was short, non-invasive and – even better – required no radiation.

Her procedure is a landmark achievement that signals a new direction for electrophysiology, a field that has already dramatically changed cardiology.  And now, an electrophysiology procedure with no radiation marks an even safer and less invasive cure for a common ailment.

A three-dimensional image of a heart created by cardio-mapping equipment at CHOC Children's.
A three-dimensional image of a heart created by cardio-mapping equipment at CHOC Children’s.

The successful radiation-free electrophysiology procedure is a credit to the skill and expertise of Dr. Anjan Batra, medical director of electrophysiology at the CHOC Children’s Heart Institute, as well as the state-of-the-art cardio mapping equipment inside the hospital’s new Bill Holmes Tower.

“This has really changed our field,” Dr. Batra said. “We can do so much more, and do it better and safer. It’s great to be in a field where we not only treat, but also cure. It’s great to help a patient so that they don’t have to see a doctor for the condition again.”

Dr. Batra performed the procedure using a three-dimensional cardio mapping system. The device uses catheters with locator sensors that transmit signals from inside the heart. This allowed Dr. Batra to visualize the beating heart by using these magnetic sources as reference points, rather than relying on fluoroscopy – an imaging technique that uses X-rays to obtain images of internal organs while they’re in motion – to reveal the catheters’ positions.

Just weeks after her procedure, Jaden’s quality of life has already improved.

Last November, Jaden began complaining of a rapid heartbeat, says her mother, Vera.  A normal resting heart rate for children ages 7 to 9 is between 70 and 110 beats per minute, but Jaden experienced a heart rate of 225 beats per minute during one emergency department visit. She was then referred to CHOC, and was subsequently determined to be a good candidate for an electrophysiology procedure.

“I was nervous at first,” Jaden says, “but then I knew that they were studying the heart for a long time so nothing would go wrong.”

Typically, up to three patients are diagnosed with arrhythmias each day at CHOC, Dr. Batra says.  About 25 years ago, the only cure for these conditions was open heart surgery, and many patients simply tolerated the condition or relied on medication.

Now, the hospital has used electrophysiology procedures to cure more than 100 children with arrhythmias each year, and that figure is expected to increase as awareness grows among parents and the medical community, says Dr. Batra, one of about 200 pediatric electrophysiologists worldwide.

Learn more about CHOC’s electrophysiology services.

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CHOC Patient Experiences Miracle Thanks to Four Amazing Docs

The Bill Holmes Tower at CHOC Children’s is now open, and as we celebrate our new hospital and its state-of-the-art programs and services, including the Julia and George Argyros Emergency Department at CHOC Children’s – Orange County’s first fully dedicated pediatric ED, we’d also like to take an opportunity to celebrate and recognize just a few of the many doctors making miracles happen every day within the walls at CHOC.  Here, we hear from Heather and Curtis Short about the CHOC doctors who saved their son’s life.

Cameron and his Dad, Curtis.
Cameron and his Dad, Curtis.

Cameron was born with cranial stenosis, or improper fusion of growth plates in his skull. When he was 8 months old, we scheduled corrective surgery at CHOC Children’s.

After his surgery, he was stable in the pediatric intensive care unit (PICU), so we went home to sleep.

But a ringing phone jerked us awake at 6 a.m. Cameron had suffered two heart attacks and a stroke. We raced back to the hospital to find the chaplain waiting to console us. Cameron had coded.

We went into the PICU to say goodbye. In a fog, we watched Dr. Adam Schwarz administer chest compressions. He brought Cameron back to life.

We experienced a miracle in the form of Dr. Adam Schwarz, Dr. Anjan Batra and a superb cardiac emergency team.

Twenty-one days later we left the hospital – the three of us, together. Now we have the friendliest, most enthusiastic 5-year-old in Orange County, if not the world.

But CHOC docs still play a very important role in our lives. Every six months, Cameron visits Dr. Michael Rebolledo for an echocardiogram. Since Cameron’s initial hospital stay, CHOC docs have seen him through a heart catheterization and an aortic valve repair.

In fact, just after Cameron’s fifth birthday, we learned that he would need open-heart surgery for a faulty mitral valve. We were terrified. After all, we came too close to losing Cameron the first time.

Again, we were saved by a CHOC doctor – this time, Dr. Richard Gates. He assured us that cardiac surgery would be less risky than Cameron’s original cranial surgery. As he calmed our fears, Dr. Gates handed Cameron his stethoscope, making everyone in the exam room happier.

We left Dr. Gates saying to ourselves, “We can do this. Cameron needs the surgery. We need to go into it with confidence.”

Today we have confidence. And we have Cameron — both thanks to CHOC doctors.

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