Ten months ago, sitting in our doctor’s cramped office, I reassured my wife: “Think of all the things that modern medicine can repair … this is just an esophagus.” Our son Elliot, due in five weeks or less, had just been diagnosed with likely esophageal atresia/tracheoesophageal fistula (EA/TEF), a defect in which there is a missing section of the esophagus and often an abnormal connection of the esophagus to the trachea. Our doctor assured us that surgeons would fix Elliot’s esophagus in the first few days of his life and that this would become a blip in our family history. We left holding hands, bummed out but oddly comforted by the fact that it was “just an esophagus.”
Against advice, we read information about EA/TEF on the Internet. Our simple understanding of it was shaken by stories of children who had endured surgery after failed surgery, only to find pyrrhic success with a piece of colon or the stomach in place of the esophagus. All EA/TEF kids have a bumpy start, but the common feature of these stories was an initially “long gap” of missing esophagus. These weren’t the only stories out there, and we were aware of the Internet’s bias toward extreme cases. We also read frequently the name ‘John Foker,’ a professor of surgery at the University of Minnesota who had he developed a system for repairing long gap esophageal atresia (LGEA). Of the well-ending stories we found, many were of parents who had moved their family across the country to Minneapolis. Unfortunately for new kids with LGEA, after a long career, Dr. Foker was no longer practicing. We reminded ourselves that most EA/TEFs involve a short gap and are repaired right away. The misfortune of these families seemed remote.
Elliot was delivered at our home hospital in California through an emergency C-section. The diagnosis of esophageal atresia was immediately confirmed, but no one could say if the gap of missing esophagus was short or long. They put a suction catheter in Elliot’s mouth to keep him from drowning on his saliva. Otherwise he looked remarkably normal.
Half-asleep from the night’s events we briefly talked to the attending surgeons. They hurried him away that morning, and after six hours they returned dejected – it wasn’t what they thought. It took open chest surgery (thoracotomy) to diagnose that Elliot had a long gap, which they could not repair. We were becoming a story like the ones that I had discounted online.
Elliot spent the ensuing four months in the neonatal intensive care unit (NICU). He had three major surgeries and uncounted smaller procedures. The recoveries from thoracotomies were especially trying. Every day they stretched his esophagus by pushing flexible rods down his throat. Remarkably, in between these events, Elliot grew into a happy kid. His broad smile stole everyone’s hearts and renewed our endurance.
Over this time I started to read the surgery journals and understood that LGEA repair was among the most challenging surgeries. Well-meaning nurses would ask ebulliently if we were looking forward to Elliot’s next surgery. We would smile and say yes…and we did hope, but mostly we feared surgery. Outside of our attending surgeons, few at our hospital had ever heard of LGEA. If they did, they confused key aspects with more common forms of EA/TEF. It felt like we were half-present and half stuck on a lonely and indescribable planet.
The final repair surgery at our home hospital failed to connect the esophagus over a gap still longer than thought. Recovery was protracted and especially terrifying. The next plan, they said, was to pull Elliot’s stomach into his chest to bridge the gap in his esophagus. We had read the journal articles on stomach pull-ups and the stories of kids who had them, and it was hard to accept. A trend was forming. It seemed the repairs and their long-term consequences would surpass the severity of the original defect. My early mantra, “it’s just an esophagus” became mockingly ironic. We needed to do something.
I had started a dialogue with Dr. Foker. He was surprisingly accessible and his directness was steadying: “Your chances are best at Children’s Hospital Boston…get him there as soon as possible.” Our home surgeons did not agree and felt Foker’s approach was controversial. I contacted Dr. Bradley Linden at Children’s, who had trained with Foker for years. Brad’s calming assurances along with Children’s reputation and affiliation with Harvard doused our worries.
We made our decision, and within four sleepless days, Elliot was in Boston. When we arrived at 3 a.m., Melissa DeFusco, one of our primary nurses, greeted us with her Providence accent: “So we’ve been told Elliot has long gap EA, I’ve taken care of a few other LGEA kids this year…” It was the first time anyone but a surgeon had acknowledged Elliot’s condition. My wife and I had to fight back tears. Our planet no longer seemed indescribable.
The next day we met our surgeons, doctors Brad Linden and Russell Jennings. Brad had been instrumental in arranging Elliot’s transfer. After studying Elliot’s records and ordering fluoroscopy studies, they confirmed they would get Elliot’s esophagus together. This level of confidence was new to us. Everyone we met, from the nurses to the neonatologists to the radiologists, had experience with LGEA. Everyone was good at what they did; there wasn’t a weak link. We were fatigued but filled with hope− it was as if someone flipped a switch and circumstances that seemed dire now seemed easily workable. Elliot was still critical and unstable, but Children’s NICU had him stabilized in a week. In two weeks, Elliot was stealing hearts again with that exuberant smile.
Elliot was also not alone. We befriended another LGEA family who, after a series of failed surgeries on their son, had transferred from South Carolina. But for setting, their LGEA stories seemed the same as ours. We often wondered how many families like us were out there, alone.
Elliot spent two months in the NICU awaiting surgery. The hospital found my wife an I a long term room in the Kent Street House, a mansion in nearby Brookline that has been converted into housing for Children’s families. We were used to sleeping on lobby couches. The Kent house was more like a bed and breakfast, and we slept well for the first night in months.
After a month in Boston, Brad informed us that Dr. Foker himsel, famous for his esophagus repair technique and our guide into Boston, would be part of our surgery team. This “dream team” repaired Elliot’s esophagus in one surgery. We struggled to believe it; we silently worried something would fail like it always did, but Elliot only got better.
A few weeks later, we saw Elliot’s indomitable smile again. For the first time in his life he can swallow food and his own saliva. His face is clear of tape and tubes. With the help of Children’s eating experts, he’s learning to use his new esophagus. Elliot has challenges ahead of him, but he is over this mountain and his spirit can’t be stopped.