Fighting for Kennedy: Coping with moyamoya disease

Girl with moyamoya disease is thriving.

If you happen to be waiting in line at the supermarket with Kennedy Grace Cheshire, you’ll likely leave the store with a whole new group of friends. This outgoing five-year-old can’t resist introducing herself to her fellow shoppers — and then introducing them to each other. “She’s never met a stranger,” says her mother, Amber.

Kennedy, who lives in Texas, brought that playful attitude to the East Coast last year when she and her family arrived at Boston Children’s Hospital for evaluation and treatment.

At age two, she had been diagnosed with neurofibromatosis 1 (NF1), a genetic condition that causes symptoms including benign tumors that form from nerve tissue. The diagnosis wasn’t surprising: Kennedy’s father Robert has NF1, too. But a couple of years later, an MRI scan brought a shock. Along with NF1, Kennedy also has moyamoya disease. In this rare but very serious condition, the walls of the carotid arteries that supply blood to the brain become thickened and narrowed. This can increase the risk of blood clots and, in turn, stroke.

An even greater shock came when Kennedy’s doctor in Texas told her parents that there was nothing he could do to treat her surgically until she had already had a stroke. “She was only four years old. That wasn’t acceptable to us,” says her mother.

On the two-hour drive home from their local hospital, she and Robert made a decision: They would do whatever it took to help their daughter. “I’m the type of person who looks for ways to fix a problem right away,” says Amber, who lost her first daughter, Zoey, at birth due to complications from severe spina bifida. “I knew we had to fight for Kennedy.”

‘She’s our daughter, too.’

Little girl with moyamoya disease enjoys cooking.

Amber’s research on moyamoya disease brought her to Dr. Edward Smith and his colleagues in the Moyamoya Disease Program at Boston Children’s. When they arrived at the hospital, Amber and Robert knew they had made the right decision. The results of Kennedy’s angiogram placed her at stage V of the condition (stage VI is the most severe). “There’s no doubt she would have had a stroke,” says Amber.

When it came time for surgery, the Cheshires felt reassured by the professionalism of everyone on Kennedy’s care team. “Dr. Smith told us, ‘When Kennedy is on the operating table, she’s our daughter, too. We’ll treat her the way we would want our own children to be treated,'” says Amber. “That meant the world to us. All the stress and worry we had been through — he took it away when he said that.”

After the procedure was successfully completed, it was time to exhale a bit. Amber recalls Smith using a table, power cord and other nearby props to explain the details of the finished procedure to her dad, Kennedy’s grandfather. And once Kennedy was resting in the ICU, she and Smith were able to blow off some steam by cracking jokes with each other — especially every kid’s favorite, knock-knock jokes.

Thriving with moyamoya

Princess Kennedy plays in Texas after surgery at Boston Children's Hospital.

Kennedy will return to Boston this summer for a follow-up visit, but all signs point to a healthy, happy future. When she’s not baking cakes and brownies, playing with her dog and cat and running around outdoors, she enjoys dancing — an activity she was thrilled to be able to return to after her surgery.

Those who do encounter this vibrant little girl in line at the store should keep one thing in mind: She prefers to be addressed as Princess Kennedy. “She’s hilarious. Every single night, we have to call her that,” laughs Amber, who Kennedy labels “the queen mother.” (Robert, like any good dad, cheerfully accepts the role of “peasant.”)

Amber and Robert say they’re appreciative of everyone at the Moyamoya Disease Program, who answered their questions and walked them through the evaluation and treatment process step by step. “I say it a lot, but we are so grateful to Boston Children’s because without their help we don’t know where Kennedy would be today,” says Amber. As it is, she’s on top of the world.

Learn the latest on moyamoya disease at Boston Children’s annual Moyamoya Family Day event in May.