Doctors: Success of toddler's operation will alter way treatment's handled
Editor's note: This is the second of a two-part story about a toddler born with his heart outside his chest.
During his first 11 months, Ryan Marquiss spent just 11 weeks at home.
The rest of the time, he was hospitalized, hooked to machines, heavily sedated and strapped down in his bedding. His tiny, deformed heart somehow continued to beat, even though it lay outside his chest, struggling to pump blood.
Doctors called his survival a miracle. Never before had a baby lived with his combination of heart defects: Not only was his heart growing outside his body, it had only one working ventricle.
But nearly three years after his dramatic birth in February 2009, Ryan behaves like other boys his age. His doctors put no limits on his life expectancy, and they say his survival offers unprecedented hope for other babies with serious defects.
"This definitely can be a game-changer," said Dr. Nina Deutsch, pediatric cardiac anesthesiologist at Children's National Medical Center in Washington and one of Ryan's doctors. "From the literature, there are no babies that survived this. Now, for families on the edge, who have been told it's completely hopeless and nothing can happen, they've been given the prospect of this one case that did go well.
"Before, it was, 'There's no chance for survival.' That's not the case any more."
During the hospital stays, Ryan's mother, Leighann Marquiss, 34, of Franklin Park, bonded with her son by singing to him, caressing his arms, holding his tiny fingers. When he opened his eyes, she leaned in, made eye contact and reassured him that Mommy was there.
His sisters, Natalie, 7, and Ainsley, 5, visited often. They played games near the window when Ryan slept and climbed into his crib when he woke.
When they could not visit, Leighann recorded the girls playing at home and played the tapes for Ryan. On one recording, Natalie grabbed the microphone. Days later, as Leighann stood over Ryan's crib, her daughter's voice filled the room:
"Hi, Ryan. I love you. I miss you. I wish you were home. I love your eyebrows. I love your eyelashes. I love your cute face. I love your nose. One day, you will grow up and hear me and understand me."
Ryan's eyebrows "crinkled and then he opened one eye the tiniest bit, looking to see if she was there," Leighann recalled. "Then he smiled a tired, droopy smile."
When Ryan was 4 months old, doctors inserted a ring of tissue expanders around the hole in his chest. For two months, they slowly pumped saline into the silicone sacs to stretch his skin.
Ryan needed open-heart surgery, and Dr. Mary Donofrio, director of the Fetal Heart Program at Children's National and head of Ryan's medical team, feared that a membrane protecting his heart from infection would not heal if cut. The extra skin would allow surgeons to sew the folds together after surgery, to cover his heart.
The operation — a bilateral bidirectional Glenn procedure — would rewire Ryan's heart valves, allowing them to pump blood through the lung arteries, even with only one working ventricle.
The night before the operation, Donofrio and two other doctors stood before the sedated baby and discussed his odds. Only Donofrio believed he would survive.
"Everyone agreed to the plan of care, but some were more pessimistic than others," she said. "I was hopeful it would work. I like to think that way."
It did work. Ryan's heart eventually learned to pump blood on its own. The tissue expanders closed his chest, and Ryan's medical team made history.
In a published peer review case report, they wrote:
"The use of a tissue expansion protocol similar to ours for defect closure has been reported, to our knowledge, only once. Postoperative death due to sepsis resulted. The technique in our case was successful."
Dr. Michael Boyajian, the plastic surgeon who oversaw the procedure, explained why they were the first to successfully use tissue expanders on a baby whose heart is born on the outside: "Most of them don't live long enough for us to try."
Ryan's triumphs were measured in "Beads of Courage."
A program at Children's National awards patients with colorful beads during hospital stays. They string them into necklaces, creating artful representations of their endurance.
Ryan's strand, strung by his mother, grew rapidly. He earned a few beads at a time for simple procedures such as blood draws and X-rays, and larger sums for surviving tube insertions, heart catheterizations and surgeries.
One day as Ryan slept, Leighann met the Beads of Courage program director in her office. She was sitting at her desk, examining a large, multicolored bead bearing the image of a butterfly. It was beautiful, more so than any Ryan had earned.
"Oh! What's the butterfly bead for?" Leighann asked.
The program director turned and stared at Leighann but did not respond.
Leighann understood. The bead meant death.
Looking to the future
Every day that Ryan survives, he becomes more a heart disease patient and less an oddity defying all odds, Donofrio said.
"We've overcome the barriers that the heart being out of the chest gave us," she said. "He probably will need some sort of chest protection operation at some point, but that really is minor and certainly could be overcome with some sort of protective padding when he plays sports."
She smiled at the thought of Ryan one day playing organized sports.
"He probably won't be a competitive athlete, but most of our kids do run around on the playground and play Little League," she said.
On a warm day this fall, Leighann drove Ryan to Blueberry Hill Park in the North Hills. For an hour there, Ryan climbed the jungle gym and shot down the sliding board under the close supervision of Jean Riddle, his physical therapist. Because Ryan spent most of his first year sedated, Riddle is trying to make up for lost time and improve his coordination and conditioning.
"You getting tired yet, Ryan?" she asked him after 30 minutes. Ryan ignored the question and pointed to a green climbing wall. Leighann sat at the top and reached for him as Riddle nudged him upward.
"It's amazing," Riddle said. "When we started, he couldn't go for more than 10 minutes. Now you can't get him to stop."
'Every day is a gift'
Nobody knows how long Ryan will live.
"So much of what we've told them has been wrong, so I'm not about to make any prediction," said Dr. Joshua Kanter, an interventional cardiologist at Children's National who performed several catheterization procedures on Ryan, including one that saved his life by punching a hole in a membrane inside the heart that was preventing proper blood flow.
"It's about making good memories and giving him the highest quality of life. He's got that," Kanter said.
Henry Marquiss marvels at his son's will to survive.
"He just kept fighting, proving everyone wrong," Henry said. "After a while, you're like, 'You know what• Everyone says he's going to die, but he's not dying.'
"Every day is a gift. Whether it's five years, 15 or 85, he's going to live as long as he's going to live."
Ryan could require a heart transplant in time, Donofrio said.
"But modern medicine is progressing so rapidly, it is very likely that 30 years from now we'll have some new solution to make heart function better," she said. "Our job is to give a kid hope. I'm going to stay positive with this and say that Ryan is going to outlive us all."
Ryan will return to Children's National for surgeries. Doctors at Children's Hospital of Pittsburgh provide preventative care.
When he's old enough to understand, Leighann said, she plans to show Ryan his Beads of Courage necklace. The strand is 25 feet long and will get longer.
But it has no butterfly bead.
Ryan Marquiss was born with several heart defects that had led doctors to believe he would die in the womb. Among his ailments:
Ectopia cordis: Ryan's heart grew outside his chest. His rib cage is deformed, with the sternum extending just below his clavicle.
Hypoplastic right heart syndrome (HRHS): Ryan's right ventricle does not work.
Ventricular septal defect (VSD): Ryan has a hole in the wall separating the right and left ventricles of the heart.
Pulmonary valve stenosis: A heart valve disorder in which arteries are abnormally small, compromising blood flow.
Cor triatriatum: A heart defect in which a membrane forms in the atrium and prevents blood flow. A life-saving catheterization procedure fixed this.
Tricuspid atresia: A congenital heart disease in which the tricuspid valve is missing or abnormally developed.
Omphalocele: A defect in the abdominal cavity in which an internal organ — in Ryan's case, part of his colon — extends outside the body. Doctors fixed this problem after his birth.
Hydrocephalus ex vacuo: For Ryan, a non-life-threatening build-up of fluid in the brain.