My husband, Duncan, and I were living in London, England, when Clara was born. Although my pregnancy had started out like any other, I later developed severe polyhydramnios, an accumulation of amniotic fluid that can sometimes indicate the presence of certain congenital issues.
After I delivered, it became clear that Clara had a congenital condition called esophageal atresia with tracheoesophageal fistula (EA/TEF). This condition meant that her esophagus (the tube that carries food from the mouth to the stomach) hadn’t developed properly and didn’t connect to her stomach, but that her esophagus and windpipe were improperly connected to one another through a small fistula. Because of these problems, any liquid she swallowed could not reach her stomach, but was likely to enter her lungs.
Two steps forward, one step back
Clara’s EA/TEF was successfully repaired when she was one day old, but, when she was not recovering as expected, her doctors uncovered another rare congenital condition: a type III laryngeal cleft. This abnormal opening between the larynx and esophagus allows food and liquid to pass into the lungs, leading to even more eating and breathing issues. Her surgeon planned to repair the cleft using a minimally invasive approach called endoscopic surgery when Clara was about three months old. In the meantime, a series of respiratory infections and her risk of aspirating and kept her in the hospital.
Soon, five months had passed — but her doctors believed she was still too small for endoscopic repair. Instead, they now wanted to perform open surgery, which would require even longer hospitalization and carried a high risk of a tracheotomy and permanent damage to her voice.
A laryngeal cleft and a leap of faith
Since Clara’s birth, I had read everything I could about her diagnoses. One name kept coming up in my research: Dr. Reza Rahbar, director of the Center for Airway Disorders at Boston Children’s Hospital. Although we were an ocean away, I was familiar with the hospital’s excellent reputation and decided to reach out.
Speaking with Dr. Rahbar by phone, I was extremely reassured by his knowledge and his confidence in dealing with laryngeal clefts. While he made no promises, he told us that a child’s size wasn’t the determining factor in being able to do an endoscopic repair. We were so impressed after speaking with Dr. Rahbar that we took the huge leap of faith to start the process of moving Clara to Boston, even though this required us to privately arrange for a transfer by air ambulance.
Upon arriving at Boston Children’s, we immediately felt we had made the right decision. We knew that most children with EA/TEF also have tracheomalacia — a defect that causes the airway to collapse when coughing or exhaling — but in Boston we discovered that Clara’s tracheomalacia was more severe than we had thought. Dr. Russell Jennings, a surgeon on her care team, recommended she undergo a posterior tracheopexy, a pioneering surgery that was developed at Boston Children’s.
Going ahead with this procedure was another leap of faith for us. We weren’t expecting another major surgery — and Clara would be only the 70th child in the world to receive it. But Dr. Jennings explained that the tracheomalacia was responsible for many of her severe respiratory symptoms. So we forged ahead, and Clara eventually underwent both procedures. After 277 days as an inpatient, she was finally able to come home to her family.
Two years later, our very sick baby has grown into a sweet, social toddler, who loves reading, going to the park and playing with her dolls and stuffed animals — she particularly likes giving them medical check-ups and making them “all better.” The path hasn’t always been smooth: In the process of her treatment, we learned that Clara actually had a more extensive type III laryngeal cleft, making us even more certain that Dr. Rahbar is one of the few surgeons in the world with the ability to repair it endoscopically.
She has undergone two revisions to the initial procedure and will need another revision this summer, but she continues to improve significantly and has been able to avoid open surgery and has not needed a tracheotomy. Her cleft is 85 percent healed and she now eats a normal diet and weathers minor bugs without distress. Simply put, coming to Boston was the best decision our family has ever made.
Learn more about the Center for Airway Disorders.