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.

img_5056An innovative solution for midaortic syndrome

Kenslie was scheduled to have an aortic bypass with an artificial graft, but as the date drew nearer, her doctors decided on a different plan. Instead of using artificial material, they would create a tissue graft using part of one of Kenslie’s own veins. This way, the graft would grow with her, and her blood wouldn’t be exposed to foreign material.

“The doctors put me in contact with another mother, Wendy, whose son just had the same procedure they wanted to do on Kenslie. He was the first child at Boston Children’s to have the procedure, and he’s doing great now. It was so nice to be able to talk to Wendy and know what to expect.”

Kenslie had her surgery on Oct. 4, and is doing wonderfully. Looking ahead, Kenslie will still have follow-up appointments, but they will be farther and farther apart, and it’s unlikely she will need a repeat intervention.

“It’s so nice that whenever we come here, we always see the same team — Dr. Kim; her cardiologist Dr. Diego Porras; and her renal specialist Dr. Deborah Stein. They really know Kenslie. They’re pretty in tune to her, and they’re very personable with us,” says Lori.

“I communicate through email with Dr. Stein most often. She connects me with others, as needed. She’s my “go-to.” We see a renal doctor here in Columbia, and Dr. Stein also communicates directly with her.”

For now, Kenslie and her brothers and sisters are focused on preparing for the holidays.

“Throughout this whole experience, I feel like I’ve learned a lot,” says Lori. “And I’ve been teaching my family and community about this condition.”

mid-aortic-syndrome-kenslie-family

Learn more about care for children with Midaortic Syndrome at Boston Children’s.