Carolyn Milks turns 8 on August 21. It’s a big celebration. Carolyn and her family aren’t just celebrating her birthday — they’re celebrating Carolyn’s return to normal. For most of the summer, things like swimming, riding her bicycle and horsing around with her sisters and cousins had been out of the question for Carolyn.
“This is what kids really want. They just want to be normal and do their normal activities,” says Carolyn’s mother Kristen.
It had been a topsy-turvy spring for the Milks family.
My heart and mind froze. Her whole left side was weak. … Only one part of the body controls both the arm and the leg. I thought, ‘Carolyn has something wrong with her brain.’
Carolyn, normally a bright, active second grader, started having puzzling symptoms in March.
“She was having a hard time concentrating on her homework and was crying, and my husband and I couldn’t figure out why,” recalls Kristen.
Over the next few days Kristen, an occupational therapist, began observing strange movements in her daughter’s left arm and hand. Carolyn appeared to struggle with everyday activities like holding a pencil, tying her shoes, and she even tried to switch her hand dominance. Kristen set up her phone to video Carolyn.
At the end of the week, while Carolyn, her twin sister Laura and their big sister Emma, were playing at a trampoline park, Kristen watched the videos.
“I was becoming alarmed at what I saw with the functioning in her left arm and hand. Later that day, I watched Carolyn almost fall doing a back bend. Her left arm didn’t hold her weight. And then when I watched her walk, she almost fell a couple of times; she didn’t have full control of her left leg. My heart and mind froze. Her whole left side was weak. … Only one part of the body controls both the arm and the leg. I thought, ‘Carolyn has something wrong with her brain.’” …
Ryan (above left) and Tyler Earle of Winnipeg, Canada had a ticking time bomb inside their heads. Both boys have a rare brain disorder called moyamoya that had caused the arteries feeding their brains to become dangerously narrowed.
At first, they experienced only headaches. But then Ryan suddenly lost his ability to write, began having trouble with word-finding and became weak on one side of his body — signs he had suffered a stroke. He was diagnosed with moyamoya and had partial surgery, but a second stroke took away part of his vision and partially paralyzed him.
Ryan needed a second operation as soon as possible. By this time, Tyler was diagnosed with advancing moyamoya disease and would need neurosurgery too, on both sides of his brain. …
Calvin Steede, who lives in Bermuda, will never forget the day in 2011 when he saw the movie “Winnie the Pooh” with his mother and sister. The film ended, and suddenly the boy who likes to draw and play soccer couldn’t put on his backpack. His arms had stopped working. He couldn’t stand, and soon he couldn’t talk.
Ryan Earle is outgoing and upbeat, goofing around in his neurosurgeon’s office. He’s had two strokes, but he’s recovering steadily, a testament to the resilience of children’s brains. His twin brother Tyler, with him at the visit, has avoided a likely stroke altogether.
Both Ryan and Tyler, 8, have a rare brain disorder called moyamoya disease, in which the internal carotid arteries become narrowed, slowing the flow of blood to their brains. Without surgery, moyamoya poses a five-year stroke risk of 60 to 90 percent.
The boys’ moyamoya symptoms were subtle: “They would have a mild headache, then they’d throw up, but then an hour later they’d go to school and they’d be fine,” says their mother, Kathy.
Their pediatrician in Winnepeg, Canada, therefore, was not initially concerned.
But then Ryan, who used to make perfectly formed letters, suddenly couldn’t write. “I thought, ‘this is strange, maybe his hand is broken,’” Kathy recalls.
He also had weakness on one side of his body and was having trouble with language. “He couldn’t find the words to say ‘truck.’ He’d say, ‘those things,’” says Kathy. “The neurologist said he’d had a stroke.”
Ryan underwent an operation for moyamoya in a hospital in Winnipeg to try to restore blood flow to the left side of his brain. But then came a second stroke, worse than the first. It caused Ryan to lose part of his vision, made speech more difficult and partially paralyzed him, forcing him to use a wheelchair.
Ryan needed a second operation, on the right side of his brain. But the Winnipeg hospital was hesitant to move too quickly, wanting to give him time to recover from his stroke.
In the meantime, Tyler was also diagnosed with moyamoya, and imaging tests showed his disease was advancing. Like Ryan, both sides of his brain showed the classic “puff of smoke” (moyamoya in Japanese)—wispy, tangled blood vessels grown by a brain desperate for blood. He would need neurosurgery too.
The Earles had to act. “My perception was, it was a ticking time bomb waiting to go off,” says David, the boys’ dad. “Time was not on our side.”
Their research led them to confer with neurosurgeon Edward Smith, MD, of Boston Children’s Hospital’s Cerebrovascular Surgery and Interventions Center. He reviewed the boys’ scans and told the Earles that his team could operate on both sides of Tyler’s brain on the same day, rather than space out the operations, and that Ryan would not need to wait. “They had done it so many times, they were completely confident,” says Kathy. “They said ‘we can definitely go in.’”
Growing new blood vessels
Smith planned to use an approach developed by his mentor, Michael Scott, MD, called pial synangiosis. First performed at Boston Children’s in 1985, it takes a healthy artery from the scalp and attaches it to the part of the brain that isn’t getting enough blood. The artery naturally takes root, providing a permanent new source of blood.
In May 2013, Ryan and Tyler had their back-to-back moyamoya operations—one on the right side of Ryan’s brain and one on each side of Tyler’s.
“A major consideration was that both sides could be done at the same time,” says David. “Dr. Smith operated in the morning, Dr. Scott in the afternoon.”
Fast-forward one year.
Today, at their one-year checkup, Smith shares the results of the boys’ follow-up brain angiograms, performed by interventional neuroradiologist Darren Orbach, MD, PhD. Both sets of scans show healthy vessels and blood flow, and both Tyler and Ryan have an excellent prognosis.
“You guys are so good at growing blood vessels,” says Kathy, grinning at her boys.
Ryan is clearly bouncing back from his strokes. He still needs a one-to-one worker at school, but his speech is better, and he no longer needs a wheelchair. His headaches are minimal. He’s fighting to catch up with Tyler—who recently won a provincial chess championship in Canada. Like his brother, Ryan is in a French immersion class at school.
“He’s very aware that Tyler can do some things he can’t,” says Kathy. “But he’s constantly improving.”