Stories about: midaortic syndrome

Second opinion for midaortic syndrome gives Cameron a second chance

midaortic syndrome

Cameron Grubb likes to shoot Nerf guns, and even his own doctors aren’t immune from his aim — in fact, they often fire back. It’s a playful act that everyone welcomes, however, particularly since this 6-year-old has defied the odds multiple times in his young life.

Just three years ago, Cameron was struggling to survive after being diagnosed with extremely high blood pressure — so elevated, in fact, that his clinicians in Kansas thought the monitor must be broken. When they eventually confirmed the reading, it was 170/140, a dangerous level that sent him to the local intensive care unit for nine days.

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Catching up with Kenslie: Life after midaortic syndrome surgery

Kenslie who had mid-aortic syndrome

We last saw Kenslie Shealy in the spring of 2015, as she was settling back into life at home in South Carolina after a long stay at Boston Children’s Hospital. Kenslie, now 4, had emergency surgery for midaortic syndrome (MAS), a rare condition that affects the heart and kidneys. Untreated, the condition can lead to damage in the brain, intestines and limbs.

At Boston Children’s, Kenslie’s multi-disciplinary team included specialists in cardiology, interventional cardiology, nephrology, transplant and vascular surgery, interventional radiologygenetics and rheumatology.

She recovered well after her first surgery, performed by kidney and liver surgeon Dr. Heung Bae Kim, but her parents, Lori and Calvin, knew she wasn’t “out of the woods” quite yet. Kenslie would eventually need a follow-up surgery to further expand her aorta. She needed to grow a bit more first, so her doctors didn’t set a date for her second procedure.

“They thought she was going to have to wait longer, until she was about 7 or 10 years old, but she’s already outgrowing the stents they put in last winter,” says Lori. “So they scheduled her for September. It’s a good sign. It means she’s healthy, developing well and was ready for it before they thought she would be.”

This summer, while Kenslie played outside and swam as much as possible, her parents planned for what they hoped will be their last multi-week stay in Boston for some time.

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Kenslie’s story: A new brother and a new chance at life

Kenslie and Hollis sleeping

February was a very busy month for the Shealy family from Lexington, South Carolina. Lori and Calvin were expecting the family’s fourth child. Their daughter Kenslie, 2, was ecstatic at the thought of a new baby brother.

“When I was pregnant with Hollis, Kenslie kept asking when he was coming,” Lori says. “She’d say, ‘Mommy, can you just open up your belly? I want to see him.’”

But Kenslie missed meeting Hollis the day he was born.

Instead, she and Calvin were nearly 1,000 miles from home at Boston Children’s Hospital, where Kenslie, who had been diagnosed with midaortic syndrome, was battling for her life. The topsy-turvy month began with symptoms of the flu.

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Changing the face of Midaortic Syndrome treatment to save Sofia

The American Journal of Transplantation recently published a paper, documenting a first-of-its-kind treatment for midaortic syndrome, as performed by Khashayar Vakili, MD, of Boston Children’s Department of Surgery and Heung Bae Kim, MD, director of Boston Children’s Pediatric Transplant Center.

In the following blog Marcus Groff, father of the patient described in the Journal of Transplantation, writes about his daughter’s revolutionary surgery at Boston Children’s.

Sofia

Two years ago my wife and I were anxiously waiting outside the NICU of our local hospital at four in the morning. Inside, our daughter Sofia was being monitored for several medical concerns, including dangerously high blood pressure. The nurses on staff assured us that Sofia was “a fighter” so when we drifted off to sleep later that morning we were exhausted, but joyful. We never saw what was coming, but then, you rarely do.

It would take a few days before the gravity of the situation became too obvious to ignore. While we fretted over our daughter’s high bilirubin and high blood pressure, something ominous was lurking. I couldn’t put my finger on it, but something wasn’t quite right. Still, we took our queues from the medical staff, which seemed confident our Sofia would pull-through. Only later, through tearful goodbyes, would the nurses confide that privately they feared Sofia wouldn’t make it.

We came to Boston Children’s for a second opinion of what was making Sofia so sick—what we got was a name for the phantom malady that was plaguing our daughter: midaortic syndrome (MAS). MAS occurs when the aorta, the main artery that delivers oxygen-rich blood throughout the body, is narrowed or damaged, which can often cause serious spikes in blood pressure by restricting blood flow to the kidneys. (A condition known as renovascular hypertension.)

We finally had a name for Sofia’s illness, but a larger question still loomed: How do you treat it?

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