Midaortic syndrome takes Arizona teen to Boston for innovative surgery

Justin Kibler is tall, strong and lean. At 18, he’s already a competitive rodeo star and an active member of Future Farmers of America (FFA). Looking at him, “he’s the picture of health.” But what can’t be seen, just by looking, is that just four years ago, Justin developed an extremely rare and dangerous disease called midaortic syndrome (MAS). And he needed special care that no one in his entire home state of Arizona could provide.

Midaortic syndrome is characterized by a narrowing of the parts of the aorta (the main artery that delivers oxygen-rich blood throughout the body), running through the chest and abdomen. MAS causes severe high blood pressure and can also significantly damage the brain, kidneys, intestines and limbs. Untreated, the disease is debilitating and life-threatening.

Most children with MAS have obvious symptoms of physical decline, but “Justin was asymptomatic — up to and after the diagnosis, he felt fine.” says his mother, Wendy.

It was at a routine, pre-football season check-up in which Justin’s pediatrician, Dr. Teresa Longoria, first noted his blood pressure was quite high. She ordered more tests: an eye test, an echocardiogram (ultrasound of the heart), and eventually a renal ultrasound that revealed a narrowed aorta. While on the outside, Justin seemed perfectly fit to play any sport he wanted that fall, inside his body there were life-threatening problems affecting his liver, heart and end organs.

“Dr. Longoria had actually trained at Boston Children’s Hospital,” Justin’s mom, Wendy, recalls. “She was familiar with their specialized programs and knew who to reach out to.”

In the meantime, Wendy started searching the internet for more information. “Because MAS is so rare, there isn’t a lot out there. Most of the information is in medical journals and full of jargon.”

Justin’s pediatrician arranged a referral to the Midaortic Syndrome and Renal Hypertension Center at Boston Children’s Hospital, and the family (Justin; his parents, Wendy and Mark; and his younger sister Courtney) went to their first appointment that fall.  Justin’s first surgery was scheduled for just a few months later in January.

“The waiting was the worst part. It was really scary, but it all worked out.”

Justin, who has MAS, with his favorite nurse, Ruth
Justin with his favorite nurse, Ruth

Surgery and recovery

Justin didn’t know it yet, but he was about to make history. As he lay on the operating table, Dr. Heung Bae Kim decided to perform a novel, first-of-its-kind surgery: Rather than relying on prosthetic material to create a graft to bypass the diseased aorta, Dr. Kim would use Justin’s own mesenteric arteries (the arteries that supply blood to the intestines). His idea was that the organic material would grow with Justin, and potentially eliminate the need for a repeat intervention.

The groundbreaking procedure — now called Mesenteric Artery Growth to Improve Circulation (MAGIC) and offered to all eligible patients — was a success. Now, four years later, the shunt has indeed grown along with Justin.

“Since we were first referred to Boston Children’s, we’ve been able to talk to a few other families about the condition and what we’re all going through,” says Wendy. “That mutual support has been great. And we are so grateful for Justin’s clinical team. Dr. Kim is so humble, and we love Dr. Stein, too. All of the staff there were great. If you are lost in the hall, someone will come right up to help you.”

Wendy said she was surprised at how fast the recovery was. “Justin just wanted to get back into his favorite activities — sports,” she says. “After just three months, he was cleared to start roping again.”

While Justin was never in serious pain before the diagnosis, looking back, Wendy says she can recognize minor symptoms that the family overlooked. “He was a fast runner, but could never run long distances,” she says, “He’d wear out.  Now, he runs really well and his legs don’t hurt.”

Justin, who has midaortic syndrome, with his father in Boston
Justin with his father in Boston

This time next year, Justin will be in college, where he hopes to continue competitive rodeo (He practices in the family’s backyard roping arena every night.) and stay involved with FFA.

During the hardest times — the waiting, the traveling, the wondering — Wendy says Justin was always brave and patient. “It was probably the hardest year of all of our lives,” she says, “But, we got through it!”

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