Twelve-year-old becomes first CHOC patient to receive life-saving device

Erin Greaves and her son, Sean.
Sean and his mom, Erin.

Erin Greaves woke to the sound of her son’s gasp and the dogs barking. The pediatric nurse raced to 12-year-old Sean’s room and immediately began performing CPR. When paramedics arrived, Sean was experiencing ventricular fibrillation, a serious heart rhythm problem that is the most common cause of sudden cardiac death. He was defibrillated twice before being taken to a nearby hospital, and subsequently transported to CHOC Children’s.

As a patient in CHOC’s pediatric intensive care unit, Sean underwent a series of tests, including a cardiac MRI, cardiac catheterization, exercise tests and genetic testing. Results revealed no structural damage to his heart and no underlying condition. Sean’s ventricular fibrillation was ruled idiopathic, which means no known cause.

Sean’s cardiologist, Dr. Anthony McCanta, has specialized expertise in the treatment of irregular heart rhythms and presented a unique option to Sean and Erin. Physicians primarily use implantable cardioverter defibrillators (ICDs) to treat the most serious heart rhythm problems. The device constantly tracks the patient’s heart rhythm and delivers an electric shock to the heart when it beats abnormally fast. Traditional defibrillators require electrical conductor wires, or leads, to be inserted into the heart through a vein in the upper chest.

Sean and his mom chose a newer treatment option called the Subcutaneous Implantable Cardioverter Defibrillator (S-ICD). The 12-year-old became the first CHOC patient to receive the device.

“The S-ICD is not as invasive as other ICDs since the sensing and defibrillating electrode is implanted directly under the skin of the chest wall and it is connected to a generator in the axilla, or lower armpit area. No component of the device is inside the veins, heart, or even the inner chest itself. Consequently, there are fewer future complications of leads in the vein and heart, and a much faster recovery time — tangible benefits for young active children,” explains Dr. McCanta.

Just one week after Sean’s frightening ordeal, he was back at home in excellent condition.

“Even though I am a pediatric nurse, I am a mom first. I have tremendous gratitude for Dr. McCanta and the CHOC team who took such excellent care of Sean. He is back at home, playing with the dogs and his friends, and looking forward to starting middle school in the fall,” says Erin.

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Derek’s Story: A Landmark Procedure

Derek Young looked like any other baby when he was born in February 1994. But 3-1/2 months later, his mother Pamela noticed his head was slowly getting larger. Doctors diagnosed hydrocephalus, or fluid on the brain, and placed a shunt to drain the fluid. Fast forward 10 years when Derek needed a shunt revision. He was treated at the CHOC Children’s Neuroscience Institute and released.

CHOC Children's Neuroscience Institute

However, six months later, Derek returned to CHOC with what appeared to be a failure of the original shunt. Neurosurgeon Dr. Michael Muhonen decided to perform a pioneering procedure called a third ventriculostomy in which he made a tiny hole in the wall of the third ventricle of the brain — allowing movement of fluid out of the blocked ventricle.

As a result of this extraordinary surgery, Derek no longer required a shunt nor did he or his mother need to live in constant fear of shunt failure. An avid swimmer, this procedure allowed him to continue to pursue his passion, including completing a Catalina-to-Long-Beach swim to raise money for CHOC.

Derek is now a 6’2” 20-year-old junior at Northern Arizona University studying to be an emergency room or intensive care unit nurse, a career directly inspired from his experience with CHOC. From the compassionate, skilled nurses who made him laugh to the expert, encouraging doctors who described the procedure in terms he could understand, Derek’s experience with CHOC was life-changing.

Learn more about CHOC Children’s Neuroscience Institute.

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Genetic Syndrome Causes Cleft Lip, Palate in Sisters

CHOC Children’s Cleft and Craniofacial Program Just like most sisters, Tristyn and Raigyn Snyder will share toys, clothes and friends throughout their lives. They also share something unique: a genetic condition called Van der Woude syndrome.

In the Genes

When Cindy Snyder was 12 weeks pregnant with Tristyn, a prenatal ultrasound showed that the baby had a bilateral cleft lip and palate. After Tristyn was born, specialists at the CHOC Children’s Cleft and Craniofacial Program noticed she also had small indentations on her bottom lip, or “lip pits,” a key marker of Van der Woude syndrome (VWS).

Van der Woude syndrome is the most common single-cause of cleft lip and palate. Individuals with VWS may have a cleft lip with or without a cleft palate, a submucous cleft palate, lip pits along the lower lip, or any combination of these features. Tristyn’s dad, Matt, also has Van der Woude syndrome and had a unilateral cleft lip as a child.

“If a child’s mother or father has Van der Woude syndrome, each child would have a 50 percent chance of also having the condition,” according to Dr. Neda Zadeh, a CHOC Children’s genetic specialist. “VWS can present differently in each person. For instance, a mom may have had lip pits, but her child could have a cleft palate and no evidence of lip pits.”

Shortly after Tristyn’s birth in 2011, she had a nasoalveolar molding (NAM) device fitted inside her mouth to partially close her cleft. Plastic surgeon Dr. Michael Sundine performed surgery at CHOC to repair Tristyn’s lip when she was seven months old, and he did a second surgery when she was 10 months old to finish her palate repair and remove the lip pits.

Now 3 years old, Tristyn barely shows any physical evidence of her condition. Other than a need for speech therapy, “No one even knows anything happened to her,” Cindy says.

Baby Raigyn after her surgery.
Baby Raigyn after her surgery.

The Decision to Expand Their Family

“We wanted to have another child, regardless,” Cindy says. “Some people are like, how could you have another one? Tristyn is so happy and healthy. How could we not have another one?”

In 2014, Cindy and Matt were thrilled to learn they were expecting another girl, Raigyn. An ultrasound showed that Raigyn’s lip was not forming properly, a sign that she likely had Van der Woude syndrome. A genetic test could have confirmed it, but the Snyders opted out.

“We didn’t really need to know either way, and we felt like the cleft was enough to know,” Cindy says.

The Snyders were prepared when Raigyn was born with a bilateral cleft lip and palate. She wore a NAM device and recently had surgery to repair her lip. In a few months, she’ll have another procedure to repair her palate. Cindy is confident that Raigyn will continue to develop normally, just like her big sister.

“They are perfectly healthy children,” Cindy says. “Everyone’s born with different things, and this is just what they were born with.”

Learn more about cleft and craniofacial services at CHOC.
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Matthew’s Story: Healing in the Surgical NICU

babies surgeryGrace Wu beams as she watches her son Matthew smiling and happy after enjoying his bottle.

“It’s good to see him so happy and active,” she says. “That he could do that makes me very, very happy.”

It’s a marked change from the days following Matthew’s birth almost four months ago.

As a newborn, he was diagnosed with volvulus, a condition wherein the intestine is twisted and can ultimately cut off blood circulation. Symptoms of volvulus include a distended stomach and intolerance to feeding, which Matthew exhibited.

The baby was quickly transferred to CHOC Children’s for emergency surgery at just three days old. CHOC surgeon Dr. Saeed-Ur-Rehman Awan repaired the malformation by performing an ileostomy, wherein the intestine is brought outside the body.

Next, Matthew needed time to heal his organs. He spent the next three months recovering in CHOC’s Surgical Neonatal Intensive Care Unit, a special part of the hospital’s main NICU dedicated to the care of babies who need surgery.

There, Matthew was under the care of a multidisciplinary team that included Dr. Irfan Ahmad, a CHOC neonatologist and co-director of the surgical NICU, and many other clinicians.

In the unit, the team cares for patients jointly, discussing the cases of children like Matthew as a group and forming a treatment plan that often calls for the expertise of other specialties at CHOC.

Another key component of the surgical NICU care team is parents and families. In Matthew’s case, his parents and grandfather, Jerry, partnered with clinicians on every stage of the baby’s care.

“Jerry was there every single day holding Matthew – even when he was crying,” Dr. Ahmad says. “He was a great member of the team, and he provided a lot of support.”

As Matthew began eating orally in small volumes as well as through intravenous methods, he geared up for a second surgery that would reattach his intestines. That procedure was performed just three months after the first.

After several more weeks of recovery, Matthew was able to eat fully from a bottle and was on his way home, much to the relief of his family.

“I was very worried because for the first time, I thought I might lose him,” Grace says. “I am very thankful for the care he’s received.”

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CHOC’s Specialized Metabolics Team Treats Rare Genetic Disorder

CHOC Children’s highly specialized metabolics team identifies rare and dangerous genetic disorders before it’s too late. And early, non-invasive screening is a big reason why Henry Louderback celebrated his fourth birthday last year.CHOC metabolicsHenry was one of 100,000 kids diagnosed each year with tyrosinemia type I, a deficiency of an enzyme that, if untreated, causes liver failure and death. As one of the largest designated newborn screening centers in California, CHOC was able to save Henry, whose medication and diet will prevent the serious and life-threatening complications of the disease.

“He was losing some weight but no huge alarms went off,” Nicole Louderback, Henry’s mother, says of his condition shortly after he was born on Sept. 20, 2010. Newborn screening tests detected the disorder before he was a week old, allowing the metabolics team to start life-saving treatment before Henry’s liver went into failure.

Henry’s now a happy, normally developing child — thanks to what Nicole calls the “incredible” work of Dr. Raymond Wang, a CHOC metabolic disorders specialist, and other members on the metabolics team.

Learn more about metabolic disorders services at CHOC.

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