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Five weeks before his due date, Elliot Cleckler was diagnosed with esophageal atresia and tracheoesophageal fistula (TEF), an abnormal connection between the esophagus and the trachea that can cause food traveling down the esophagus or acid refluxing up from the stomach to pass into the lungs. His parents, Jay and Heather, were upset but hopeful. "We left that meeting thinking, it could be so much worse; at least it's just an esophagus," says Jay.
Esophageal atresia is a birth defect that affects about one in every 3,500 to 4,000 babies in the United States, causing the esophagus to form in two unconnected segments—one stemming from the throat, the other from the stomach. Because of the gap between the segments, any liquid, including a baby's own saliva, can't reach the stomach and collects in the back of the throat. In the most severe cases, liquid can flow into the lungs and put the baby at risk for pneumonia or even drowning. Nearly 25 percent of these cases are long gap esophageal atresia (LGEA), in which the ends are so far apart that they can't easily be connected. After six hours of exploratory surgery immediately after his birth, Elliot's surgeons returned with heartbreaking news: The ends of Elliot's esophagus were so far apart that they hadn't even been able to locate the bottom portion. He had LGEA. "We were running completely on hope up until that point," says Heather. "When we found out it was long gap, that hope stumbled a little."
"Everyone told us not to look up LGEA on the Internet," says Jay. "So, of course we did." What Jay and Heather found were stories of children like Elliot, who endured failed surgery after failed surgery. Many of the "success" stories centered on colonic transposition, in which a piece of excess colon is stitched into place as a replacement esophagus, andgastric pull-up, in which the stomach is pulled up into the chest and connected directly to the top section of esophagus. But both procedures have significant drawbacks.
Elliot's surgeons instead advocated for attempting to stretch the two short segments of his esophagus over time to see if the ends could be made to meet. But the Clecklers had reservations. In the best cases, stretching still constricts the internal flow through the esophagus and thins the external walls, making them less resilient. In the worst cases, the esophagus, like a rubber band, will tear if it reaches its elastic limit.
None of the options gave the Clecklers much hope. "We kept coming across statistics showing increased esophageal cancer rates and low growth percentiles," says Heather. "All of a sudden we realized we had gone from reading scary stories to living one."
In their Internet searches, one name the Clecklers kept coming across was John Foker, MD, a pediatric surgeon from the University of Minnesota. Foker had developed his own repair for LGEA that uses tension-induced growth to save the existing esophagus. "These short segments of esophagus already have everything they need to grow into a normal esophagus," says Foker. "We just need to trigger them."
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To do this, sutures are attached to the unconnected ends of the esophagus, stretched slightly and tethered into place. Just enough tension is applied to stimulate the esophagus to grow on its own. "You just pull on the esophagus a little and leave it there," says Rusty Jennings, MD, director of Children's Hospital Boston's Esophageal Advanced Treatment Center. "When you go back the next day you'll find the tether isn't pulling as hard as it was the day before." With the patient kept on a ventilator, paralyzed and heavily sedated for the duration of the process, tension on the sutures is increased gradually until the ends are close enough to be sewn together. It's a deceptively simple concept applied to the most complex system imaginable: the human body.
In addition to growing in length, Foker's method has resulted in thickening and strengthening of the esophageal walls, the opposite of what happens with simple stretching. "The esophagus is like a python," says Foker. "It's a muscle, and it naturally wants to expand." Most patients see an average of 50-fold growth over the course of 10 days. They usually have two to three "growth" operations over a one-to-three-week period, often followed by an anti-reflux procedure. Over a span of three to six months, the children learn to eat solid foods and can have their feeding tubes removed. All of Foker's patients—about 50 in the past 10 yearsóare now able to eat and swallow like other children.
By the time Jay and Heather found their way to Foker, they were emotionally out of gas. Elliot had already been through three major surgeries for LGEA and spent the first four months of his life in a neonatal intensive care unit near their home in Long Beach, California. Unfortunately, they found Foker just as he was retiring and disbanding the only dedicated LGEA team in the country. It was the equivalent of pulling into a gas station on empty, only to find out they're closed.
"It was the only solution that didn't seem crazy to us, and suddenly it wasn't even an option, so we just called Dr. Foker directly," says Jay. Foker confirmed that there was no chance of repairing Elliot's LGEA in Minnesota, but told the Clecklers that he was working directly with Jennings and Bradley Linden, MD, to recreate his program at Children's, now the only pediatric institution in the world to offer his technique.
At Foker's urging, and despite Elliot's doctors' recommendations, the Clecklers contacted Linden directly. "We knew Dr. Linden had trained with Dr. Foker for years, and his confidence made all the difference," says Jay. "At home, so few people we spoke to had ever actually treated a baby with Elliot's condition." Linden told the Clecklers not to be discouraged by the previous failed surgeries, and that Elliot had "a whole chest full of esophagus...we can get it together." Less than a week later, the Clecklers came to Boston, with Elliot's condition deteriorating rapidly. "Elliot was in a death spiral," says Jennings. "His parents saw what was happening and they traveled all the way across the country to break out of the spiral."
Jennings first saw Foker's technique in person when one of his patients chose to go to Minnesota for the operation. Jennings told the family that he wanted to go with them and see Foker's team in action. "They called me the night before and I dropped everything," says Jennings. "When Dr. Foker started, the esophagus was so small that the nerve next to it looked huge. By the time his team was finished, it looked normal."
This is no small feat. Success is surrounded by a minefield of potential problems, including injury to nerves, blood vessels or even the heart. Tearing of tissue due to improper placement of sutures is a constant threat. The esophagus lies right along the recurrent laryngeal nerves; and one wrong move can permanently damage a child's voice. "This is not just a surgical process, it's a system," says Jennings. "You need surgical technicians, a complete ICU, nurses, staff, gastroenterologists and radiologists. At Children's, we have the advantage of depth on our bench. We have a team that can say 'We will fix that child, whatever it takes.'"
The Children's team has already started receiving referrals from around the world, including babies born with esophageal atresia and children whose esophagus had been damaged during other medical procedures.
Foker himself came to Boston to join Jennings and Linden for the final 12-hour surgery, which connected the nubs of Elliot's esophagus for the first time. His ecstatic parents posted a video on their Facebook page of Elliot's saliva passing through his esophagus.
Elliot remained at Children's while he was weaned off of pain medication and learned to eat for the first time. The only permanent damage may be a paralyzed vocal cord from a previous surgery, which limits the upper register of Elliot's voice, but even that now seems minor to the Clecklers. Heather downplays the importance of being able to hit high notes and Jay talks about Elliot having a deep, tough-guy voice.
"Before we came to Children's, we couldn't even think about our future," says Heather. "Now I feel like we're in junior high again, we laugh so much." For the first part of Elliot's life, there hasn't been much cause for laughter, but the tired couple holds hands and beams at the prospect of bringing their son home in time for his first Halloween. "It's hard to imagine that when this all started, I thought, at least it's just an esophagus," says Jay.
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