In her short five years of life, Evelyn Cocos has had three open-heart surgeries, five heart catheterizations, one gastrostomy tube (G-tube), one Nissen fundoplication, two supraglottoplasties, two blood infections, one stroke, and countless needle pokes, echocardiograms and CT scans.
But if you looked at her today, you’d see a 5-year-old, curly-haired girl with bright shining eyes, an infectious smile and a strong will. She is active, energetic, and loves to dance. She has an affinity for Disney princesses, horses, music and loves the movie “Frozen.” And at the core of her being, Evelyn is a champion.
No flow = no grow
Evelyn was born Oct. 7, 2012. Just 24 hours later, she was diagnosed with hypoplastic left heart syndrome (HLHS), a congenital heart defect.
Kim Cocos, Evelyn’s mom, remembers fearing for her daughter’s life after the doctors at SSM Health Cardinal Glennon Children’s Hospital gave the family the news.
“I thought she wasn’t going to make it,” said Kim. “I didn’t know one person with a congenital heart defect. The cardiologist told us they could save her life with three open heart surgeries.”
HLHS is a congenital heart defect (CHD) where the left structures of the heart (mitral valve, left ventricle, aortic valve and aortic arch) are small, or hypoplastic.
“It is thought this occurs embryologically because of a limitation to blood flow through the left heart during embryologic development,” said Dr. Jamie Sutherell, Evelyn’s cardiologist. “For example, if the aortic valve is small as it is developing, there will be limited flow through it, which leads to limited growth in the other left-sided structures.”
Dr. Sutherell, a SLUCare pediatric cardiologist with SSM Health Cardinal Glennon, said although this defect cannot be corrected, it can be palliated to create a stable circulation a child can live with.
“The palliation consists of three surgeries, all named after the surgeons who developed them,” he said. “One performed in infancy (Norwood operation), a second between 4 and 6 months of age (bidirectional Glenn shunt), and the third around 3 years of age (the Fontan operation).”
Together, these three surgeries reconfigure circulation so the single right ventricle pumps blood out to a rebuilt aorta to the body. The venous return coming back from the body is diverted straight to the lungs and doesn’t return to the heart like it does in normal circulation.
Congenital heart defects occur in about 1 in 100 children born in the United States, according to Dr. Sutherell. And while most are anomalies like holes in the heart and valve issues, Evelyn’s defect is even more rare, making up only one to three percent of all CHDs.
Heart defects in children can occur for multiple reasons. Genetics plays a role, as do maternal risk factors like maternal diabetes, drugs taken during pregnancy and maternal infections.
“Preventing congenital heart defects is the future,” said Dr. Sutherell. “ But the challenge is that congenital heart defects aren’t often detected until it’s too late.”
Evelyn has had a rocky road on her CHD journey. Through it all, there exists a beacon of hope for the family – the staff of SSM Health Cardinal Glennon.
“Some people say ‘family’ or ‘team,’ but I call them my ‘squad,’” said Kim. “I feel like every single person is behind me.”
Each of Evelyn’s open-heart surgeries has come with its own set of recovery complications, including an aspiration that led to respiratory and weight gain problems, as well as an oral aversion. But Kim knew that in their time of need, they could turn to the staff at SSM Health Cardinal Glennon.
In November 2015, Evelyn started going to the feeding team at SSM Health Cardinal Glennon and has been going once a month for over a year.
“They are miracle workers,” said Kim. “Oral aversion and eating has been one of Evelyn’s biggest challenges. It was one of the hardest parts of the journey, but we’ve had the best team beside us. We’ve had so much support from them.”
Mended Little Hearts
In addition to SSM Health Cardinal Glennon staff, comfort for the Cocos family has also come from other ‘heart parents’ who knew what was ahead, and what they had left behind.
“At the time, even though I had the nurses there who understood me and my feelings, I felt like I needed another parent to talk to who had been in my situation,” said Kim.
The answer was Mended Little Hearts, a national program for peer-to-peer support for children, patients and families affected by CHD. The program connects families who are able to share experiences and provide support.
Brooke Brothers, a nursing care coordinator in the Pediatric Intensive Care Unit and Transitional Care Unit at SSM Health Cardinal Glennon and the representative for the Mended Little Hearts program at the hospital, says it isn’t just for support within the walls of the hospital.
“It’s a lifetime bond,” said Brooke. “The families will talk and learn what’s going on and meet each other and really get to know one another. It’s ongoing support for a lifetime.”
Kim found the program a vital part of getting through the hard times during Evelyn’s surgeries and recovery.
“You just want to know that someone understands,” said Kim. “Your family is there for you, but no one really knows what you’re going through unless you’re a heart parent. You want to connect with someone who’s been there.”
And now that Evelyn is flourishing, Kim is paying that kindness connection forward. She is an active part of the St. Louis Mended Little Hearts program and is an invaluable resource and support person for heart parents.
“I think it’s important for heart families to have that peer-to-peer support,” said Kim. “Everyone wants to feel like they fit in somewhere. When you come to our meetings you know someone has felt the same as you. Someone knows what it’s like to eat the same things for months while watching and praying that your baby’s little body heals. I hope Evelyn’s story can give just a tiny bit of hope that a parent needs on a bad day.”
Many congenital heart defects are now treatable, if not curable, said Dr. Sutherell, which wasn’t the case just 10-15 years ago. Even the ones that can’t be cured can be treated, allowing the child to live an active life.
Children also recover from big surgeries in ways adults never could.
Since Evelyn was 6 months old, she has seen an array of therapists – occupational, physical, developmental. The result is a child who is thriving and loves life.
“She has worked so hard to get where she is now,” says Kim. “I am beyond proud of her.”
As Evelyn recovers and reaches new heights each day, Kim has advice for those just beginning their heart journeys.
“This is going to be the hardest thing you do in your life,” she said. “It will also be your biggest blessing. You will do things you never thought you could do. It’s okay if you don’t know what to do. You’re going to have hard days, but you’re going to have even bigger celebrations. On those hard days you have to hang on to faith and hope.”
For more information on congenital heart defects, click here.