At age 4, Harper Robinson doesn’t really remember most of the clinicians who helped change her life — but that doesn’t stop her from greeting Dr. Russell Jennings with a big smile at a recent appointment. “Whenever Harper had surgery, he would cut her dressings into the shape of a heart,” says her mom, Jessica, of Jennings, surgical director of the Esophageal and Airway Treatment (EAT) Center at Boston Children’s Hospital.
Today, the little girl is a ball of energy who loves singing in choir and showing off the dance moves she’s mastered at ballet class. Yet just a few years ago, she was struggling to perform the most basic functions, including breathing and eating. Harper was born with two rare conditions called esophageal atresia (EA, in which the esophagus grows in two separate segments that do not connect) and tracheoesophageal fistula (TEF, an abnormal connection between the esophagus and trachea).
An uncertain future
Although her parents knew before she was born that she would have these conditions — known collectively as EA/TEF — they weren’t fully prepared for the extent of care she would require. The couple, who travel extensively for husband Shane’s job, initially sought treatment at Harper’s birth hospital in St. Louis, Missouri.
When she was just a week old, physicians there attempted to connect the segments of her esophagus, but were unsuccessful. That’s likely because Harper has long-gap EA, in which so much of the esophagus is missing that the ends can’t be easily connected with surgery. Worse, she developed a serious bacterial infection that left her close to death. “We weren’t sure if she would make it through the night,” Jessica remembers.
‘Things finally started to make sense’
Harper did recover, but she still wasn’t enjoying an optimal quality of life. Her breathing remained noisy and labored, and she couldn’t eat by mouth. Five months later, the family was living in Atlanta when they got a call from Dori Gallagher, the clinical nurse coordinator for the EAT Center. “She asked how things were going and how the team could help,” says Jessica.
While Jessica was still pregnant, she and Shane had interviewed Dr. Jennings as a potential surgeon for Harper, but had decided to wait until their daughter was born to decide on a clinical team. Now, it was clear that they were ready to take that step. In Boston, “they immediately knew what we were going through,” explains Jessica. “They were so invested in Harper — and things finally started to make sense.”
Challenges at every turn
In addition to confirming the diagnosis of EA/TEF, the EAT clinicians found that Harper also had a laryngeal cleft and tracheomalacia, the latter of which caused her airway to collapse by almost 90 percent when she breathed. Such answers shed light on Harper’s symptoms, but her path was still a difficult one. For the next year, she faced a series of frustrating ups and downs as Dr. Jennings, Dr. Michael Manfredi, and their team tried to address her esophageal strictures (narrowing caused by scarring from previous surgeries), leading to more than 30 procedures called dilations to stretch the tissue. “There were challenges at every turn, but that only seemed to drive Harper’s clinicians to succeed,” says Jessica. “They gave us the confidence as parents that they could make things right.”
Still, the process was taxing. The Robinsons chose to take more than a year off from surgeries to give Harper a break and allow her to grow and spend time with her family. The little girl relied on a feeding tube to get adequate nutrition. Yet she learned that she loved tasting new exotic foods — everything from pickles to sushi — through a spit fistula, surgically created opening in her neck. “All the food and drink that she swallowed came out onto special bibs,” explains Jessica. “It was messy, but for us it was a vital part of Harper’s progress because it helped her learn to eat, swallow and coordinate breathing.”
Harper was nearly 3 years old when her parents received another call from Dori, who Jessica describes as “my medical mom — she always validates me and answers my questions.” This time, Dori had a question of her own: Was Harper ready to try one more operation? Known as jejeunal interposition, the procedure replaces the missing section the esophagus with a section of the jejunum (the middle part of the small intestine) and is an option for kids with long-gap EA.
Putting their trust in Dr. Jennings, the Robinsons decided to take this step, and Harper underwent jejeunal interposition. Following a remarkably fast recovery, she was able finally enjoy a slice of cake on her birthday. This time, she didn’t just taste it, but could swallow it, too. It was the culmination of three years’ worth of highs, lows, despair and hope.
“Boston Children’s has been there for us through all of it,” says Jessica. “After having our faith tested for so long, this feels like redemption.”
Learn about the Esophageal and Airway Treatment Center.