Happy Thanksgiving! My name is Karen Stapleton, and my son Noah is a patient at CHOC Children’s. As I prepare to celebrate the holidays with my family, I’m grateful we can be together since we have so much to celebrate. I’m also grateful for Noah’s many doctors and nurses at CHOC because without them, my son wouldn’t be alive.
Noah’s birth story
When I was 29 weeks pregnant with Noah, we learned that he had Down syndrome. Another prenatal ultrasound showed an abnormality in his heart, and we were referred to Dr. Pierangelo Renella, a pediatric cardiologist at CHOC, who diagnosed Noah with tetralogy of fallot, a serious heart defect that causes poor oxygenated blood flow from the heart to the rest of the body. I was scared, but having been a CHOC patient myself as a child, I knew my son would be in good hands.
On July 27 of last year our lives changed forever— Noah was born! I chose to deliver at St. Joseph Hospital in Orange so that my son could be as close to CHOC as possible. When he was born, there were so many doctors and nurses around. I saw Noah quickly enough to give him a kiss before he was whisked away to the Neonatal Intensive Care Unit (NICU) at CHOC.
Shortly after birth, Noah’s care team also diagnosed him with Apert syndrome, a genetic disorder that causes certain bones to fuse early. For Noah, that was his skull, fingers and toes.
It was a success, and 31 days after he was born, Noah finally came home! Weekly trips back to CHOC’s clinics included visits to gastroenterology, pulmonary, cardiology and craniofacial specialists. It was another team effort to prepare Noah for a second open heart surgery that he would eventually need.
But a few weeks later, Noah had respiratory complications, which lead to an emergency open heart surgery at just 2 ½ months old. Thanks to Noah’s cardiothoracic surgeon, Dr. Richard Gates, and Noah’s fighting spirit, he was able to come home shortly after surgery.
Celebrating Christmas at CHOC
Just days before Christmas last year, Noah had to be admitted to CHOC for respiratory failure. It was scary to see my baby sedated for 19 days. Dr. Juliette Hunt, a critical care specialist, recommended that Noah undergo a tracheostomy, where a small opening is made in his windpipe and a tube is inserted to help him breathe. Making a decision like that is hard and scary for a mom, but I had complete trust in Noah’s team, and if they knew it would help Noah breathe easier, then I knew it was the right thing to do.
After that, Noah started to thrive. He gained weight and became strong enough for his next open heart surgery with Dr. Gates. After a mere six days in the Cardiovascular Intensive Care Unit following this surgery, Noah got to come home again!
Even when Noah is doing well, sometimes it can be scary to care for him when he’s at home. During one of our hospital stays, I confided this fear in one of Noah’s favorite nurses, Karissa. She gave me specific tips on what to do during his tummy time and baths, and gave me the courage to care for my son. She encouraged me, and reminded me that CHOC wouldn’t advise me to do anything that wasn’t safe.
Noah’s first birthday
All of this is a lot for a little baby to go through before his first birthday, but Noah has always surprised us and pulled through. Celebrating his first birthday meant more than celebrating his first year of life; it meant celebrating every fight Noah had won over the last year, and it meant appreciating a milestone that at times we thought we might never reach. We decided a super hero theme was perfect for his party because we think of Noah as our little super hero.
After his birthday, Noah continued to flourish and grow! He started rolling over and actively playing, and he has not stopped smiling.
This progress allowed us to prepare for his next major surgery, a frontal orbital advancement, to reshape his skull and forehead that has fused too early due to Apert syndrome.
Before surgery could begin, the doctors needed to cut Noah’s hair to make a safe incision in his skull. We marked another one of Noah’s milestones at CHOC— his first haircut!
Noah has more hurdles and additional surgeries ahead of him, but even with how much he’s fought, he continues to smile. He’s not cranky and he doesn’t cry. He’s enjoying every single day he gets to be here – and that’s the life he has taught me to live too.
If Noah’s care team ever needs a reminder of why they do what you do, I tell them: My son would not be here today if it were not for each and every one of them here at CHOC. And for that, my family will be forever grateful.
In honor of American Heart Month, we spoke to Dr. Michele Domico, medical director of CHOC’s cardiovascular intensive care unit (CVICU), on the importance of having a cardiac intensivist on a child’s heart surgery team.
Q: What role does the cardiac intensivist play in a patient’s overall care?
A: Heart patients will be seen by several doctors during their stay in CHOC’s CVICU. In addition to their cardiovascular surgeons making rounds in the unit, patients will also be regularly visited by their cardiologists who will also manage their care after they go home. But in addition to that care, pediatric heart patients also need a critical care specialist on hand at all times who can care for any unexpected complications that may arise. CHOC’s CVICU has an attending physician in the unit at all times, day or night, to care for our heart patients, and not all heart programs have that. We are also the only place in Orange County that performs neonatal and pediatric open-heart surgery, and have outcomes above the national averages for overall survival rate and neonatal survival rate.
Q: Why is it important for parents to make sure there is a cardiac intensivist on their child’s heart surgery team?
A: General intensivists have training in treating infections, traumas, cardio-respiratory disorders, and other severe problems that may arise. A cardiac intensivist has additional training in caring for exclusively for the most critically ill cardiac patients with severe and rare heart disorders. Patients benefit from having cardiac intensivists on their care teams because they’re prepared to care for any complication that may occur.
Patients with certain heart conditions may require a series of surgeries throughout their childhood. Since there is a cardiac intensive care specialist on call in the CVICU at all times, we really get to know the families we care for. There’s not a single patient who comes to the CVICU for their second surgery that we don’t remember from their first operation. That continuity of care means peace of mind for parents, knowing their child’s heart team knows everything about them.
Q: Why is it important for children to see a pediatric subspecialist for their heart condition?
A: Adult cardiac doctors are trained on structurally normal hearts, where all the pieces of the heart puzzle are intact and everything is in the right place. But children with congenital or acquired heart conditions may have missing or misplaced pieces of their hearts and need specialized care. As pediatric heart specialists, we see patients every day who have unique and severe heart conditions, so we are especially able to care for them.
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.
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.
In CHOC’s CVICU, children receive attention from pediatric cardiac-trained intensivists, nurse practitioners, critical-care registered nurses, and an interdisciplinary medical team. All rooms are private and fully equipped with leading-edge technology that meets the demands of monitoring and treating children with heart problems, and those who have undergone complex heart surgery and heart catheterization procedures. CHOC is the only facility in Orange County that performs open heart surgery on newborns and children.
That combination of well-trained experts and topnotch facilities can lead to improved patient outcomes. The Heart Institute has outcomes above the national averages for overall survival rate and neonatal survival rate. CHOC is routinely named to the Leapfrog Group’s annual list of Top Children’s Hospitals, recognizing a commitment to provide the safest and highest quality of health care.
“Cardiovascular patients are unique and delicate. They require complex care and management. CVICU staff members understand these kids’ physiology,” says Allie Quill, RN, a clinical outcomes coordinator at CHOC. “When our patients get into trouble, their treatment is not routine. All of our kids are so different and require such fine tuning.”
Cardiac pediatric subspecialists are especially trained to care for heart patients in need of critical care, and they have advanced knowledge of cardiovascular disorders.
“Pediatric heart specialists have extra training and experience in caring for children with unique and severe cardiac disorders, something a general care team or an adult cardiac doctor may not be as comfortable with,” says Dr. Michele Domico, medical director of the CVICU.
The CVICU’s design also plays a critical role in improving outcomes for patients. The space encourages parent-clinical caregiver interaction, and ensures a peaceful, healing environment. Our staff recognizes the role that loved ones play in patient healing.
Each private CVICU patient room is divided into three areas:
The first area, closest to the doorway, is the nurse’s zone. Here, visitors will notice a wheeled cart that holds a computer and supplies to allow bedside charting and full access to patients.
The second area belongs to the patient, and features gas hook-ups, monitors and other equipment.
The third area is the parent zone, which includes a sleep sofa, reading light, sink, telephone and other amenities to ensure family comfort.
The CHOC CVICU also includes two fully-equipped procedure rooms that allow physicians to perform life-saving surgery on patients who otherwise could not be transported out of the unit.
Certain heart conditions warrant a series of surgeries throughout childhood, necessitating several stays in the CVICU. For that reason, CVICU staff members often develop close and lasting relationships with patients and families.
“Parents often come back and visit us after their child has been discharged, to thank us for taking special care of their child,” says Domico. “Every year our team receives dozens of school photos and holiday cards from families who we were fortunate enough to bond with in the CVICU. It’s a privilege to see the children we’ve treated grow up and have a healthy childhood.”