Luke’s Superman Scar

Klipsun writer Evan Keen looks back on his nephew’s life currently living with a congenital heart defect

STORY AND PHOTOS BY EVAN KEEN

(Above) Now 9 years old, Luke is growing and thriving. He will need a third open heart surgery, but he remains stable while waiting.

One Ventricle, Two Jobs

I will never forget the day that I learned about my nephew’s congenital heart defect. It was 2006. I was in seventh grade sitting on the couch watching the Simpsons when the phone rang. Before my mom picked it up I could see out of the corner of my eye that it was my older sister, Jesse. My mom answered the phone, slowly went pale, collapsed to the ground with a loud thump and started sobbing.

I awoke at two in the morning on Jan. 9, 2007, to my mom shaking me and telling me that I would become an uncle. We drove to the hospital where I waited and waited for what seemed like an eternity to meet my nephew. Around 9:30 a.m. I saw my nephew for the first time in a sterile case for about two seconds before he was whisked away to the neonatal intensive care unit (NICU) where they ran tests on him and kept watch on him for the first week and a half of his life.

Luke was born with a heart defect called Double Inlet Left Ventricle. He only has one ventricle pumping blood to his body and his lungs. Essentially, his one ventricle is doing the job of two ventricles. This heart defect happens between eight to 12 weeks in the womb and occurs in about 1 in 100,000 births. The amount of oxygen in Luke’s blood (oxygen saturation, or sats) rests at around 85 percent. To be considered healthy, sats should be around 98 percent. If someone were to walk into a hospital with the sats that Luke has, they would be in critical condition and rushed to the emergency room. Luke has been at this level or worse since he was born.

Jesse was finally able to hold her firstborn child in her arms after 11 days in the NICU. At five weeks old, Luke had his first open heart surgery after going into severe heart failure. A grueling process, the surgery required Luke’s ribcage to be broken open to stop the flooding of blood into Luke’s lungs. The flooding was the main source of his heart failure and why Luke was unable to gain any weight as a newborn.

When Jesse and her husband saw him next, he was covered in tubes and hooked up to machines. Aside from the large amount of equipment, Luke is now sporting a scar that splits his chest vertically, a permanent reminder of his condition. Luke, now nine years old, calls this scar his Superman scar and wears it with pride.

Following his initial surgery, Luke was able to stay home for six months. After that, Luke returned to Seattle Children’s Hospital for his second open-heart surgery; two out of three surgeries he would need to allow his heart to work more efficiently. Soon after taking Luke home from his second surgery, Jesse and her husband knew something was wrong. He was a light shade of blue and having trouble breathing, the exact opposite of what should have been happening after his procedure. They took him to their cardiologist who found that his left diaphragm was nicked in the surgery, which subsequently resulted in another surgery to repair the tear and increase his oxygen levels. Luke wasn’t even a year old.

Since that first year, Luke hasn’t had any surgeries and has remained as healthy as he can be with his condition. My family and I have watched Luke grow and thrive and be a constant joy in our lives. Still, in the back of our minds we know Luke’s battle isn’t over. In fact, it’s just beginning.

The Fontan Procedure

I came home to Puyallup, Washington, to spend some time with my family before my birthday. After dinner, Luke asked us if we could play basketball. As we played Luke was active, running all over the court and blocking out defenders as best as he could. Despite his heart condition, Luke is still the son of athletic parents.

In less than a minute, the pace of the game had slowed down immensely. Luke was less active on the court and huffed and puffed like we had been playing for a long time. Within five minutes, we cut the game short as Luke turned blue and required a break, coughing and panting heavily. It’s moments like this that remind us that another surgery will be needed.

Jesse has become an expert in congenital heart defects since Luke was born and is gravely aware of the seriousness of Luke’s third surgery. The surgery is called the Fontan Procedure, which attempts to divert blood from the right atrium of his heart to the pulmonary arteries without going through the right ventricle of the heart. “There’s also rare, but very real, complications that can happen after the Fontan,” Jesse stammers as she says this to me. The complications of the procedure range from developmental issues to death. Studies in CHD’s are relatively new with many differing ideas on the best time to have the Fontan Procedure done. This operation usually happens between the age of two and three. Under the supervision of Luke’s cardiologist, Jesse and her husband have decided to put this surgery off until it’s absolutely necessary, saying there’s more harm than good that can come from it if it’s done too early.

The surgery is not a cure-all, as 20–30 percent of all Fontan recipients will need a heart transplant later in life, according to the Nelson Textbook of Pediatrics. Due to the Fontan circulation, the liver is at great risk. This problem was discussed at a seminar held by The Children’s Hospital of Philadelphia. The Fontan circulation triggers inflammation, fibrosis and cirrhosis of the liver. This means Luke will not only face a heart transplant, but possibly a liver transplant also.

While Luke’s cardiologist has his thinking, others disagree. “There’s some doctors who say, ‘We would not recommend the path you’re on.’ Then there’s some who strongly recommend the path we’re on, and some say, ‘I guess I’m open to it since I’m looking at Luke,” Jesse says, exacerbated. If the Fontan is successful it will make Luke’s oxygen sats go from 85% to around 90% allowing him to be more active for longer, that being said he will still get tired quickly.

I asked Jesse what her fears are with Luke. “When you don’t really have any consensus or anyone telling you we’re sure this is the right direction, it feels like the decision is on us and it’s scary,” Jesse says. My sister and her husband hope that they won’t look back and see that they made a mistake doing what they believed was right for their son.

Jesse texted me after I talked with her. “Luke was near me so I didn’t say it, but my biggest fear is that there is a complication from the Fontan and he doesn’t survive.”

Nine Years Later

Luke and I are taking a breather after the basketball game. We walk to my basement, Luke’s favorite room in my parents’ house; this is where the videogames are. As Luke works to control his breathing and color starts returning to his face, we begin to play Madden 2015.

Luke is an avid sports fan; his favorite sport is football and his favorite team is the Seattle Seahawks. Luke dreams of one day being a quarterback, strapping on a helmet, playing under the lights and leading his team to victory. But Luke knows that he will never be able to play organized sports with his heart condition.

Luke accepted this long ago but it’s still something that he has to come to terms with. I asked Luke when he notices his heart defect the most, “Mostly when I’m running and my friends are running the track at school it just pops in my head like, ‘Oh I have a heart defect I can’t do that,’ so I’ll just sit on the curb until they come back.” Luke says with a hint of despair.

It only takes Luke around 20 seconds of being active until he feels pain. “It’s kind of like a bruise that constantly hurts,” Luke says about the feeling of his heart after brief exercise. He lets me know that some days are worse than others. It usually only hurts after moving quickly but recently he has been waking up in the middle of the night in deep chest pain. This is a grave reminder that the Fontan procedure will have to be done sooner rather than later.

Luke hits a field goal, now leading 3–0. At this point, he jumps off the couch and dances proclaiming he is the king. This is the Luke that my family and I know; a funny, smart and confident kid whose personality draws people close to him.

While Luke is fully aware of his heart condition, he doesn’t yet know the gravity of how serious it is. Luke is very small for his age, both in weight and height. This is something that won’t change; his heart will always be working so hard that it will affect his weight and growth.

Luke is also unaware of how difficult his first two surgeries were on him. With the Fontan being the longest and most serious of all his surgeries, the recovery will take multiple weeks with intense pain. If Luke were aware of this he wouldn’t be so eager for the surgery. He wants it done sooner rather than later because he knows that a big Lego set comes afterward as a reward. What he can’t grasp is how the rapidly approaching surgery may change major aspects of his life forever.

Perspective

My mom’s voice rings over the intercom as Luke and I run up for some birthday cake. As my family’s voices sing Happy Birthday to me, Luke’s high-pitched voice is the loudest of them all, echoing off the dining rooms walls. When the song comes to an end, I let Luke blow out my candles. He takes as deep of a breath as he can and blows. Not a single flame remains as I’m pulled downstairs again for another gaming session with Luke.

It seems like just yesterday after my mom got up from the floor wiping away tears, and I learned of Luke’s heart condition. I’m sitting on the same couch in the same home except now I’m playing a Lego Pirates of the Caribbean video game with Luke by my side. As he laughs at my impending doom I’m not thinking about his heart; I’m thinking about how lucky I am to have Luke as my nephew.