Collaborative effort involving milestone procedure saves premature baby with complex heart disease

Baby Hope looked into her mother’s eyes and gurgled.

Four days short of turning 9 months old, wearing a white onesie with the words “Best Gift Ever” on the front, she made more baby talk.

“You’re always a big chatterbox – what are you saying?” her mother, Elizabeth “Becca” Wyneken, said as she smiled and stared into Hope’s blue eyes.

Becca and Hope endured a lot to get to where they are now — a happy and very grateful mom and a relatively healthy 9-month-old baby girl whose light-brown hair is just starting to fill in.

The odds were stacked against Hope when she was born prematurely at 31 weeks and five days, weighing just 2 pounds, 3 ounces. Today, Hope is alive thanks to a team of doctors, nurses and others who cared for her throughout a four month stay on CHOC’s neonatal intensive care unit (NICU) and cardiovascular intensive care unit (CVICU).

Born with a complex heart disease, as well as only one kidney and defects on her right leg and foot, Hope’s cardiac neonatologist, Dr. Amir Ashrafi, pegged her chances of survival at between 20 and 30%.

When Becca first set eyes on Hope a couple of days after she was born, she was very concerned about her baby’s health.

“Don’t worry, Mom,” Dr. Ashrafi told Becca that day. “I think we can help her.”

It would take an extensive collaboration between highly regarded cardiovascular interventionalists, some of whom were consulted at hospitals as far away as London, to do so.

And it would involve a high-risk procedure never performed on a baby so small at CHOC.

Dr. Amir Ashrafi, neonatologist at CHOC

Grim news at 20-week scan

At 18 weeks pregnant, Becca, a teacher’s aide, went in for a checkup. Blood tests showed her baby had a high risk for Down syndrome and spina bifida.

Two weeks later, a scan of her baby’s anatomy revealed other potential problems.

Her baby had no kidneys, Becca was told. She appeared to have no bladder, no right leg, no lungs, issues with her bowels, and a heart defect.

“I don’t think I stopped crying for the rest of the day,” Becca recalls. “It was horrible.”

She couldn’t drive home from the clinic. A friend had to pick her up. That night, Becca had dinner with her mother and aunt.

Later, lying beside her mother, Becca cried.

“I can’t believe this is happening,” she said.

She felt a poke in her belly.

“Over and over again, when I got upset, she would poke me,” Becca said.

At that moment, she decided on a name for her baby.

“Hope,” Becca told her mother.

Second opinion reveals true complications

Becca got a second opinion about her unborn baby’s condition.

Her baby was missing a kidney and had a leg defect, she was told. Most seriously, Becca was told, she had a defect on her right ventricle, the chamber within the heart responsible for pumping oxygen-depleted blood to the lungs.

Hope’s aorta and pulmonary artery that carry blood away from the heart hadn’t developed properly. She had a hole in her heart as well as one in her left superior vena cava, a vein that helps circulate deoxygenated blood back to the heart. These holes caused blood to drain incorrectly; Hope would need a team of doctors to correct the blood flow.

“Being very small with complex heart disease, your options are very limited with what you can do and the timing of any procedures,” said Dr. Ahmad Ellini, Hope’s primary pediatric cardiologist.

There were lots of sleepless nights as Hope’s team of doctors and nurses monitored her closely. Becca was beside her nearly every night.

Dr. Ashrafi and Dr. Ellini consulted with two outside experts, San Francisco-based Dr. Mohan Reddy, who specializes in complex heart disease in small newborns, as well as renowned thoracic and cardiac surgeon Dr. Glen Van Arsdell of Ronald Reagan UCLA Medical Center on the best course of action.

The team of physicians determined that a stent needed to be inserted under a pulmonary artery that was becoming too narrow, making it hard for blood to flow through it. Such a procedure is risky, especially on a baby so small.

“In Hope’s case, the idea was if we could open up the area below the valve while not injuring the valve, that would be a home run,” said Dr. Sanjay Sinha, a CHOC pediatric cardiologist who put the stent in Hope’s heart. “Two things made this difficult: she was very small, and we had no stents this size.”

A vendor was able to secure the small stent needed a day before Hope’s surgery.

Assisting Dr. Sinha during the procedure was Dr. Michael Recto, medical director of CHOC’s Cardiac Catheterization Lab.

Observing the recently developed procedure, known as valve-sparing RVOT (right ventricular outflow tract) stent placement, were several cardiologists, from CHOC and other pediatric hospitals.

“In some patients, there is very little room for a stent. Hope had just enough room for the stent to be placed,” Dr. Sinha explained, “We knew we had the technical skills and ability to do this, but this had never been done before at CHOC on a baby this size.”

A very scary moment

After the surgery, Hope got seriously ill with a viral infection. At one point, Dr. Ashrafi said, her heart stopped but the team was able to revive her.

In cases like Hope’s, where a newborn’s state of health is fragile, members of her clinical team often must pivot in an instant, making their work schedules long and unpredictable.

Hope was at CHOC for four months before she was able to go home. After that, physicians at another hospital removed the stent, closed the hole in her heart, and corrected her left superior vena cava.

Dr. Ellini, who continues to see Hope at her check-ups, is very pleased with her progress.

“She basically has a normal circulation,” he said. “She needed a pacemaker. Overall, she’s doing great. She’s only on one medication and is gaining weight.”

In fact, she’s up to 13 pounds.

Baby Hope

Dr. Ellini said he’s proud of the extensive collaboration that was involved in Hope’s care at CHOC.

“We try to really foster a collaborative team approach in our interventional lab, and this is a great example of that,” he said. “Having a dedicated neonatal cardiac intensive team of physicians and nurses who are really experts in what they do really was paramount in making sure she did well.”

Becca can’t praise Hope’s team at CHOC enough. “They’re totally lifesavers,” she said. “It was a roller coaster — heartbreaking and exiting. I was pretty much afraid all the time, but they treat you like you are family.”

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.

“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.”

More information about the Best Children’s Hospitals rankings can be found here.

In the Spotlight: Irfan Ahmad, M.D.

In addition to treating newborn babies requiring critical care, neonatologist Dr. Irfan Ahmad strives to involve family members in the care of their infant, which he says is essential for providing the best possible care for babies in the CHOC Children’s neonatal intensive care unit.

“I always include parents as part of the care team when treating a baby in the NICU, especially the mother. A mother and her baby were a single unit up until right before the delivery,” Dr. Ahmad says. “Parents are an essential part of the healing team, and building a strong physician-parent relationship is an important aspect of patient- and family-centered care.”

Surgical NICU

An internationally trained neonatologist, Dr. Ahmad also serves as medical director of the surgical neonatal intensive care program at CHOC.

Irfan Ahmad, M.D.
Irfan Ahmad, M.D.

The program will take up residence in CHOC’s recently-opened NICU, which features 36 private rooms with the latest technology and innovations in neonatal care. The 25,000-square-foot unit is nearly triple the size of CHOC’s prior NICU space, and will allow parents to stay overnight with their babies.

“We strongly believe in mother-baby bonding and the value of breast feeding, and our new private NICU rooms are designed to optimize that,” he says.

The recently-opened NICU also features three rooms with surgical lights, allowing minor procedures to be performed at the bedside.

The only Surgical NICU on the West Coast, CHOC’s program is comprised of a multidisciplinary team including neonatologists, pediatric surgeons and anesthesiologists.

“What inspires me the most about care being delivered at CHOC is the combination of passion for helping babies, multidisciplinary interactions, use of modern technology and an atmosphere of teaching,” Dr. Ahmad says. “From dedicated neonatologists present 24 hours a day in the NICU, nurses constantly advocating for best care, nutritionists and pharmacists rounding with the team, physical therapists, wound care teams, lactation specialists and social workers all working together to help a fragile small baby has no parallel.”

Dr. Ahmad’s Surgical NICU team also offers extracorporeal life support (ECLS), also referred to as extracorporeal membrane oxygenation (ECMO) for patients. CHOC is the only facility in Orange County that offers ECLS, which supports the heart and lungs by taking over the heart’s pumping function and the lung’s oxygen exchange until they can recover from injury, surgery or illness.

In addition to neonatologists, the dedicated ECLS team is composed of cardiothoracic and pediatric surgeons, intensive care physicians, nurses, respiratory therapists and cardiopulmonary perfusionists who are experts in their fields and have received additional education to manage the complex equipment and medical needs of the children needing this life-saving technology.

In addition to stewarding the Surgical NICU, Dr. Ahmad’s special clinical interests include caring for babies who require surgery, including those born with structural abnormalities such as diaphragmatic hernia, intestinal obstruction and imperforate anus. His clinical interests also include babies who develop the intestinal infection necrotizing enterocolitis or who have intestinal perforation. His most common diagnoses include intestinal obstruction and trachea-esophageal fistula.

Mandibular Distraction Program

Dr. Ahmad is especially passionate about caring for babies with difficulty breathing due to an undersized or recessed lower jaw, which can be caused by a condition called Pierre Robin Sequence.

In 2008, Dr. Ahmad helped launch a mandibular distraction program at CHOC. Dozens of infants have benefited from mandibular distraction osteogenesis, which involves a plastic surgeon placing a special device in the small lower jaw to expand it, prompting new bone growth over a period of two to three weeks.

Traditionally, babies with this condition have been treated by placing a tracheostomy that remains in place for several years until the child outgrows the condition. Mandibular distraction is a more permanent solution that takes a few months to complete, allowing a baby to go on to have a normal, healthy development.

Constant quality improvement

Passionate about quality improvement, Dr. Ahmad serves as director of quality improvement for NICUs affiliated with CHOC Children’s Specialists. He has participated in several quality improvement initiatives with Vermont Oxford Network and California Perinatal Quality Improvement Collaborative. This includes a project to improve the transition of care for surgical cases from one team to another, decreasing delivery room intubations and preventing premature newborn babies from developing hypothermia.

As the director of quality improvement for CHOC’s network of nine NICUs, he partners with quality improvement teams at each unit in carrying out improvement projects based on local needs. The team currently has nine simultaneous quality improvement projects in the hospitals where CHOC neonatologists round.

Passionate about educating the next generation of pediatricians and neonatologists, Dr. Ahmad also serves as NICU education director for UC Irvine’s pediatric residency program and is an associate clinical professor of pediatrics at UC Irvine. He also trains neonatology fellows through CHOC’s partnership with Harbor-UCLA Medical Center’s neonatal-perinatal medicine fellowship program.

His current research efforts include studying the breathing patterns of full-term babies in order to refine inclusion criteria for the mandibular distraction procedure. He is also currently studying the clinical outcomes of CHOC’s surgical NICU program.

Pursuing his calling to care for children

Dr. Ahmad attended medical school at Aga Khan University in Pakistan. He completed a residency in pediatrics at the University of Oklahoma and a fellowship in neonatal-perinatal medicine at UC Irvine. He has been on staff at CHOC for 10 years. He knew from an early age that he wanted to care for children, so pursuing a pediatrics residency after medical school was a natural choice.

“I was exposed to various specialized fields like cardiology and oncology, but I wanted to take care of the whole patient. I also wanted to see when I could have the most impact on the life of a person,” Dr. Ahmad says. “During my residency when I worked in the NICU, I noted that good care in the first few minutes of life was so critical. Effective resuscitation, followed by intensive care in the NICU could make all the difference for the patient, who can then live a long and accomplished life.”

Dr. Ahmad finds inspiration in the strength of his patient’s families, and is continually renewed and humbled by their gratitude.

“I have been impressed by the strength of the families who have a sick little baby in the NICU. It is extremely difficult to have your newborn on a ventilator struggling for life. Yet, we see the moms and dads holding on to hope and being there for their baby,” Dr. Ahmad says. “Neonatology is a very difficult field with long hours taking care of very sick babies. The gratitude you get from parents when the baby is finally well and going home and the amazing photographs and cards that are sent to us makes everything worthwhile.”

In his spare time, Dr. Ahmad enjoys golfing with his children and developing his photography skills.

Learn more about neonatal services at CHOC Children’s.

CHOC Children’s Opens New NICU with All Private Rooms

CHOC Children’s Hospital has opened its new neonatal intensive care unit (NICU) with 36 private rooms, a feature that will allow parents the opportunity to stay close to their newborns receiving intensive care.

A patient room in CHOC’s new NICU

The 25,000-square-foot unit nearly triples the size of the hospital’s previous Level 4 NICU, which included an open layout that grouped patients in pod-style beds.

The new unit, located on the fourth floor of CHOC’s Bill Holmes Tower, creates a homey atmosphere with sleeping quarters and storage space outfitted in warm colors and wooden accents to help parents feel more comfortable while their infants receive highly specialized care for extended periods of time.

“CHOC is proud to offer private rooms to our smallest patients and their parents,” said Dr. Vijay Dhar, medical director of CHOC’s NICU. “No one’s vision of parenthood includes a NICU stay, but our new unit will provide parents with the space and privacy to get to know their new baby, and reassurance that they’ll be nearby while their newborn receives the highest level of care.”

Private NICU rooms are a new standard for improved patient outcomes. Benefits for babies cared for in single-family rooms include higher weight at discharge and more rapid weight gain. Also, they require fewer medical procedures and experience less stress, lethargy and pain. Researchers have attributed these findings to increased maternal involvement.

A nurses station in CHOC’s new NICU

A private-room setting provides space and privacy sought by parents to breastfeed, practice skin-to-skin bonding, and be more intimately involved in their baby’s care. Further, individual rooms allow parents to stay overnight with their newborn, and give staff more access and interaction with the family and patient.

In addition to private rooms, the new space includes other features that will enhance patient care. Should an infant need a sudden surgical procedure, three rooms within the unit can quickly be converted into space for surgeries. The unit will also include a life-saving extracorporeal membrane oxygenation (ECMO) unit. Rooms that adjoin can be used to accommodate triplets.

Safety features include same-handed rooms, wherein equipment is positioned in the same location among all rooms to reduce human error; room-adjacent nursing alcoves; and an in-unit nutrition lab for the preparation of breast milk and formula.

CHOC’s new unit also features a family dining space, a room dedicated for siblings, a lactation room and other amenities to ensure the comfort of the entire family.

The CHOC Children’s Foundation has raised $4,381,984 toward the new NICU, including lead gifts from the Argyros Family Foundation, Credit Unions for Kids and philanthropist Margaret Sprague.

For several decades, CHOC has served infants requiring the highest level of care. With the unit’s opening, CHOC’s neonatal services now include 72 beds at CHOC Orange and the CHOC Children’s NICU at St. Joseph Hospital, and 22 beds at CHOC Children’s at Mission Hospital. In addition, a team of premier CHOC neonatologists care for babies at hospitals throughout Southern California.

A room dedicated for NICU patients’ siblings

A suite of specialized services comprises the CHOC NICU: the Surgical NICU, which provides dedicated care to babies needing or recovering from surgery; the Small Baby Unit, where infants with extremely low birth weights receive coordinated care; the Neurocritical NICU, where babies with neurological problems are cohorted; and the Cardiac NICU, which provides comprehensive care for neonates with congenital heart defects.

Visit www.choc.org/nicu to learn more about CHOC’s neonatal services.

 

 

5 Things NICU Families Need to Hear From a Clinician

Having a baby in the Neonatal Intensive Care Unit (NICU) can be an emotional and fraught experience for even the most seasoned parents.

And though they can take comfort knowing that their infants are receiving exceptional care in the CHOC Children’s NICU, parents also look to physicians, nurses and other clinicians for reassurance and support.

Here, parents explain what clinicians can say to help them feel more confident and at ease while their child receives care in the NICU.

“Your baby is important and special.”    

I know you treat many babies like mine, or even those sicker, but this is my baby. It’s important to me that you know her name. I’ve waited a long time for her, and everything I envisioned has been      turned upside down.

“Let me explain that again.” 

I realize I’ve asked the same question several times. Please be patient with me: I am learning an  entirely new language and navigating a new world, and I might need to hear something a few times.

“You are not a burden.”     

I’m sorry that I’ve called the nurses station every night and that I’ve asked for you to be paged so many times. I don’t mean to be a nuisance, but I am worried about my baby constantly.

“May I sit with you?”     

You are so busy, but when you take the time to sit with me during rounds, I feel like my baby is your only patient and you really hear my concerns.

“You are doing a great job.”     

I’m trying my hardest to advocate and care for my baby under circumstances I never would have imagined. I’ve been racking my brain to determine what I did wrong or how I could have prevented this situation, and my confidence as a parent is low. I need reassurance.