For Lisa and Mikel Whiting, CHOC not only saved their son’s life, but solved an eight-year mystery — allowing him to pursue dreams once thought unattainable. From infancy, Brant Whiting suffered severe asthma that caused asthma attacks every single night of his life. Brant describes the experience as trying to breathe through a straw.
One night, at age 8, Brant stopped breathing altogether and was rushed to a local hospital. Doctors said that Brant’s labs “were incompatible with life” and he might not survive the trip to CHOC in the transport ambulance. But he did, and Dr. Nick Anas, CHOC’s pediatrician-in-chief and his team worked for five hours to keep Brant alive. They succeeded. After six days in a medically induced coma on life support, Brant awoke — and survived.
In fact, not only did he survive, CHOC doctors were able to determine the underlying cause of Brant’s asthma — gastric reflux. He underwent a fundoplication procedure at CHOC, eliminating about 85 percent of his asthma symptoms. This correct diagnosis gave Brant the chance to pursue his dreams: playing professional baseball. He played catcher on Stanford University’s baseball team for four years before being drafted by the Los Angeles Dodgers. A precious life saved… and a dream fulfilled.
Though less common, hernias on the body’s right side are more dangerous because the liver, a larger organ, can move into the chest cavity, impairing lung development, impeding blood vessel functionality and ultimately causing pulmonary hypertension, says Dr. Mustafa Kabeer, Alicia’s surgeon at CHOC.
Just 20 percent of CDH cases are right-sided, and about 40-50 percent of babies nationwide survive their treatment; conversely, more common left-sided hernias yield about an 80-90 percent survival rate, Dr. Kabeer says.
Prenatal meetings remain key
After the diagnosis, Alicia’s mother Marlen began meeting with Dr. Kabeer and other specialists to prepare for her baby’s birth and treatment afterward.
“It helped because we had the prenatal meeting,” Dr. Kabeer says. “That way, parents can connect a face to a particular job in the care of their baby. They are educated and know what to expect during treatment.”
Just hours after her birth, Alicia’s condition began dramatically deteriorating. A transfer to CHOC was necessary, and Marlen and her husband were warned that Alicia would not likely survive the 2-mile ambulance ride.
But she made it, and quickly began treatment under a life-saving device called extracorporeal membrane oxygenation (ECMO) to help her compromised lungs. First pioneered at CHOC about 40 years ago, ECMO is a heart and lung bypass machine that can be used to rest a failing heart or lungs, providing complete support until the organs recover.
About a week later, Dr. Kabeer performed the procedure to repair the diaphragmatic hernia, all while Alicia remained on ECMO with substantially high risks of uncontrollable bleeding due to the blood thinners needed while on the treatment.
Alicia sailed through the surgery with little bleeding, but within the next day or so, Dr. Kabeer needed to perform two separate procedures to relieve pressure building inside her abdomen that compromised blood flow to the lower half of her body.
Two weeks old and five surgeries
Shortly after this surgery, Alicia began to bleed. During the next four days, she had ongoing bleeding and during the fourth day, lost about 1,500 mL of blood, or about six times her normal blood volume. Hospital staff kept her stable, and Alicia was taken off of ECMO. Two days later, Dr. Kabeer performed a final surgery to close her abdomen, which had been left open all of this time to decrease pressure.
“All of those surgeries were very high-risk surgeries,” Marlen says. “There was a very high chance she wouldn’t make it, but she did perfectly.”
Throughout the entire process, Dr. Kabeer communicated with Marlen and Omar about the risks of the surgeries. And like every other time Alicia’s parents were cautioned about her survival, the tiny infant fought back.
“Even though it’s a difficult subject, and a complicated, emotional and anxiety-provoking issue, we want parents to understand the problem their child is facing and that we’re trying to help them and their baby overcome it,” Dr. Kabeer says. “That connection and rapport are very important and it all stems from honesty.”
“It involved a very transparent discussion,” Dr. Kabeer said. “I laid out for them all of the issues and all of my concerns, and made them see that we’re going to do our best and face these challenges together. I want to give parents reassurance and security to know that not only are they in a good place, but they’re with staff who are well trained.”
Coordinated care in the Surgical NICU
Between her five surgeries and afterward, Alicia was closely monitored inside 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.
In the unit, the team cares for patients jointly, discussing the cases of children like Alicia as a group and forming a treatment plan that often calls for the expertise of other specialties at CHOC.
After several months in the Surgical NICU, Alicia went home with her family. Today, she is a happy, charismatic four-year-old.
“Alicia’s case reinforces the fact that babies are extremely resilient,” Dr. Kabeer says. “It’s amazing that she tolerated all of this. Every patient is unique and this is a perfect example of why we should give them every chance possible.”
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.
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.
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 Neuroscience Institute and released.
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.
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 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 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.
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.”