It’s an unseasonably warm February day, and 4-year-old Nora is enjoying the fresh air, immersed in an intense game of “Mother May I?” She’s in the lead, but her friend Jonette Jean-Louis is catching up.
“Nora, you may take four ‘Single Ladies’ steps,” advises Linda Pengeroth. After asking permission, the little girl gleefully skips forward, waving her raised hand in homage to the iconic Beyoncé video. “I won!” she exclaims as she crosses the finish line, a wide smile spreading across her face.
It’s a cute accomplishment for any kid — but Nora isn’t just any kid. Only three weeks earlier, she was in the operating room, undergoing a 10-hour surgical procedure to treat a rare but serious condition called midaortic syndrome. “Watching her like this today, it’s almost like it never happened,” murmurs her mother, Laura.
A dearth of information
Yet Laura and her husband, Jason, know all too well the gravity of the situation. When a routine physical last summer revealed that Nora’s blood pressure was high, they visited a slew of specialists until they received an overwhelming diagnosis: Their seemingly healthy daughter had midaortic syndrome. In this disorder, the part of the aorta (the heart’s largest blood vessel) that runs through the chest and abdomen becomes narrow. This can lead to lower blood flow in the chest, abdomen and lower limbs. Midaortic syndrome can cause dangerously high blood pressure and can be life-threatening if left untreated.
The couple began researching the condition online, only to find very little information about it. Worse, they learned that few physicians are well versed in its treatment. “One of the scariest moments was the realization that not a lot of doctors have experience dealing with midaortic syndrome,” says Jason.
From Berlin to Boston
Fortunately, they found that experience at Boston Children’s Hospital. A call to Jean-Louis, program coordinator for the hospital’s Midaortic Syndrome and Renovascular Hypertension Center, got the ball rolling. “The week before we came to Boston felt like the longest week of our lives, but Jonette put us in touch with the Center’s co-director, Dr. Deborah Stein, who took over an hour out of her day to patiently and honestly answer every question we had,” says Laura. “She’s a godsend, and her brilliance and intuition regarding Nora’s care has been remarkable.” Likewise, Pengeroth, the Center’s registered nurse, was instrumental in helping prepare them for the visit. “She went to bat for us in way I didn’t expect from anyone who wasn’t family,” Laura says.
When the family — who split their time between Kentucky and Berlin, Germany — met with the team in Boston they learned that Nora would likely need surgery, but that her hypertension could potentially be managed with medication in the meantime. By that fall, however, it was clear that medicine alone couldn’t control Nora’s blood pressure. Laura and Jason put their lives in Berlin on hold and began planning for their daughter’s surgery.
A MAGIC moment
While they were prepared for Nora to undergo TESLA, a procedure developed at Boston Children’s that slowly stretches the aorta and its blood vessels over the course of several weeks to months, there was a sudden change of plans once Nora was in the operating room. Her surgeon, Dr. Khashayar Vakili, determined that the little girl was a good candidate for a technique called MAGIC. This surgical procedure, which Vakili also helped pioneer, uses a patient’s own arteries to bypass the diseased portion of aorta and eliminates the need for a synthetic graft.
“It was nerve-wracking to know that Nora is one of just a handful of kids to undergo MAGIC, but Dr. Vakili made it easy for us to trust his expertise. He explained this massively complicated procedure in such a clear way and guided us through every step of her surgery, as well as her recovery afterward,” says Laura. The couple says that they’re also grateful to families like the Kiblers and others who put their faith in MAGIC, helping pave the way for future patients like Nora: “Those parents and their children were so brave to take a chance on this surgery, and their courage helped lead us to this solution for Nora.”
Today, Nora is still recovering from surgery and will need follow-up care — but you wouldn’t know it from her mischievous grin. Members of her care team say they’re blown away by the way she’s bounced back so quickly, which her parents attribute in part to her personality. “She’s very strong-willed,” says Laura. “People always told us that would serve her well, and now I understand why. She’s a fighter.”
As Jean-Louis and Pengeroth prepare to return to their offices, Nora isn’t quite ready to finish the game. “Let’s play again!” she clamors. “Please?”
“There she is,” Laura says, laughing. “She’s back.”
Learn about the Midaortic Syndrome and Renovascular Hypertension Center.