COLUMBIA — Luke Schawo likes going to preschool, playing with Legos and swinging, as he puts it, "super duper high" on the backyard swing set.
But beneath his shirt, a thick scar runs down the center of his chest.
Here is background information on hypoplastic left heart syndrome from David Draper, director of the Division of Pediatric Cardiology at Women's and Children's Hospital in Columbia.
- Roughly translated from its roots, hypoplastic left heart syndrome means "left heart that is incompletely molded." The left side of the heart is severely underdeveloped
- Anatomically speaking, kids with HLHS have a whole heart, but because the left side is too small to work, parents describe it as being "half a heart."
- In a normal heart, the left side receives the oxygenated, or "red" blood, from the lungs. It then pumps it out to the rest of the body.
- In a hypoplastic left heart, the left side of the heart is too small to adequately supply the needed flow of blood to the body without a complex series of surgeries.
- In its less severe form, HLHS affects only the aorta, the blood vessel that exits the left side of the heart and carries the oxygenated blood to the body.
- More often, HLHS affects the entire left side of the heart, including the mitral valve, which connects the two left-sided chambers of the heart.
- In the first two weeks of embryonic development, there is no heart or vascular system. Cardiovascular maturation continues well after birth, but the majority of the cardiac structures develop between the 18th and the 45th days of gestation. Heart pulsations begin about day 22.
- When the mitral valve is too small during prenatal development, not enough blood can get to the rest of the heart — the left ventricle, the aortic valve and the aorta. Without forward flow during prenatal life, it is thought that there is inadequate growth of these "down stream" cardiac structures.
- Using current equipment, the fetal heart may be visualized using ultrasound, or echocardiogram, technology at about 18 to 20 weeks of gestation. This is when structural cardiac defects are often detected. This is also about the same gestational age as when fetal sex can be assessed accurately.
- Although pediatric cardiologists understand the physiology of HLHS, the fundamental embryological problem — what causes the heart to form the way it does — is not generally understood.
"Did you know I had my heart fixed?" he said.
Luke, 4, has been through three major surgeries to compensate for the size of his heart and correct the flow of blood through his body. The left side of his heart is severely underdeveloped, and without surgery, it wouldn't function. Because only one side of the heart can do the work, his parents describe it as being "half a heart."
Its technical name is hypoplastic left heart syndrome, or HLHS. And though Luke is doing really well these days, the Make-A-Wish Foundation is granting his wish: In May, his family will spend a week at Disney World. As part of its philanthropic partnership with the Make-A-Wish Foundation, MU's chapter of the Chi Omega sorority celebrated the trip with a send-off for Luke and his family.
The American Heart Association estimates that about 1 percent of children born each year have a congenital cardiovascular defect, or a heart defect present from birth. Of those children, about 4 percent to 8 percent have HLHS.
For Luke, life with a heart like his is normal. When his sister was born, Luke, then 3 1/2, asked his parents when Kate would have to have her heart fixed.
For Megan and Keith Schawo bringing Kate, a healthy baby, home from the hospital right away was an adjustment. Luke didn't come home until he was 5 weeks old.
Luke was their first child, so to them, being parents meant staying at the Ronald McDonald House for weeks. It meant spending hours on the commute back and forth between Columbia and Cardinal Glennon Children's Medical Center in St. Louis. It meant submitting at least $400,000 in medical bills to insurance — and that was only after the second surgery.
“As parents, right away we learned not to take a minute of his life for granted,” his mother said.
A life altered
When Megan Schawo was 24 weeks pregnant, she and her husband went in for an ultrasound expecting to hear good news. As Keith Schawo put it, it was "the one where mothers go in expecting to hear, 'Your baby has 10 fingers and 10 toes.'"
But they heard something else altogether that day. "There's a serious problem with your baby's heart," Mark Grant, their obstetrician-gynecologist at the Center for Maternal-Fetal care, told them. He couldn't see all four chambers of the heart in the ultrasound.
The information struck them like news of a death.
"I didn't know if he even had a chance to live. I didn't know how long he had to live. I didn't know what his life quality would be like," Megan Schawo said.
Life had to go on.
“We had to go back to work that same day,” Keith Schawo said. “We had to go to sleep that night and get up the next day.”
Still, the shock of learning of a cardiac defect during pregnancy is much better than learning of it after delivery. Finding out early increases the survival rate of babies born with the problem. Grant said he sees four to six cases of structural heart defects each year.
David Draper, the director of the Division of Pediatric Cardiology at Women's and Children's Hospital in Columbia, said structural heart defects such as HLHS are often detected around the time Luke's was found.
But the defects form much earlier in development.
"The heart is formed at about five or six weeks gestation, so before most people know they're pregnant, the heart is already what it is," Draper said.
Two days after the ultrasound, Megan and Keith Schawo made the first of many visits to see Saadeh Al-Jureidini, a pediatric cardiologist at Cardinal Glennon. He made an official diagnosis and explained the condition. He assured them their baby would be fine.
A life saved
Thirteen days after Luke was born, he had his first surgery, named the Norwood after the surgeon who invented it. It was long and difficult. His surgeon, Andrew Fiore, director of cardiothoracic surgery at Cardinal Glennon, stayed at the hospital overnight in case of complications.
Around 4 a.m., the hospital chaplain told Megan and Keith Schawo, who had been trying to get some sleep on the tile floor of a hospital conference room down the hall, that the doctors were working on Luke again.
His heart had stopped beating. His chest cavity was full of blood, and there was a tear in the back of his heart. But after cleaning out his chest cavity, massaging his heart into motion by hand and putting one stitch in the tear, his heart started beating on its own again.
The worst was over. When Fiore came out of the operating room, he told the Schawos he thought their son would be OK, and he was right. Luke's next two surgeries — the Glenn and the Fontane — went much better. He's done with surgery for now, but there might be more as he grows and medical technology develops.
A wish granted
Although Luke's life is normal now, he still has a life-threatening condition. And that's why the Make-A-Wish Foundation is part of his story.
In summer 2010, Megan Schawo was talking to some of Luke's cardiac nurses about how the family wanted to take a trip to Disney World. The nurses suggested the Schawos let the hospital recommend them for a wish. After a recommendation, an application and a letter from his doctor, a couple of wish-granters came to meet Luke.
"His first wish was to hang out with grandma," said Steve Houser, one of the wish-granters.
After encouraging Luke to come up with other wishes, he decided on two: To go to Disney World or meet Michael Jackson.
By Christmas, the Schawos had a tentative yes. In February, they had an itinerary and a confirmation. Their trip is set for late May — a couple of weeks after Luke's fifth birthday.
Now, Luke lives in anticipation of Disney World.
"We can't bring it up too often," Megan Schawo said. Luke repeatedly asks, "Are we going tomorrow?"
Although it wasn't Disney World, the atmosphere at the Chi Omega send-off was nothing short of magical. Chi Omega sorority sisters and their neighbors lined the sidewalk, cheering as a white limo pulled up. Luke peeked over the halfway rolled down window at the back, waving his arm at the crowd.
He and his parents stepped onto a red carpet, where Truman the Tiger was waiting to greet them. Cameras snapped and recorded as Luke made his way into the house, then back outside for a game of beanbag toss.
The game was interrupted by the arrival of a near celebrity in Luke's eyes: MU linebacker Darvin Ruise came as a representative of the football team, and he, Truman and Luke tossed a football the MU football team had signed.
"It's something he's sure never going to forget," his grandpa, David Schawo, said.
Luke never stopped smiling.