Shelton teenager wages battle against rare illness
In Japanese, moyamoya is a beautiful sounding word, meaning “puff of smoke.”
But for Anna May, 18, a senior ready to graduate from Shelton High School, the word stands for a brain disease that manifested itself just this year and has made her life anything but beautiful.
“I was 17 when it hit me,” Anna said.
It was in January — the morning of her AP psychology exam — and she woke up with poor vision. As she took the exam, things got worse.
“I saw blackness around the words on the paper,” Anna said. “It was very scary.”
Her parents, Julia and Rob May, picked her up from school, and within a few hours she couldn’t control the movements in her limbs.
“She was twitchy,” Julia May said. “Her arms would go up over her head.”
Her parents rushed her to the emergency room at Bridgeport Hospital, where she had a CAT scan that showed “patches” of gray shadows in her brain.
She went immediately to Yale-New Haven Hospital, but it took four days for specialists to determine that she was suffering from moyamoya disease, a rare, hereditary, progressive disorder caused by narrowing and blocked arteries at the base of the brain.
An angiogram showed that her brain had masses of tiny vessels that look like puffs of smoke, Julia May said, and the vessels are formed to compensate for the blockage.
In contrast to Anna’s neural network, a normal brain has several large arteries that carry blood to all areas.
Anna’s mother Julia describes the difference in terms of roadways. “We have I-95; she has suburbia,” she said.
Her brain had been working for years to put together the makeshift network.
Wanted to operate immediately
Yale-New Haven doctors wanted to operate on Anna immediately, but instead, her family sent angiogram images overnight to moyamoya specialists in Boston, Cincinnati and Palo Alto, Calif.
Because the disease occurs predominantly in Japanese and other Asian people, Dr. Gary Steinberg at Stanford Hospital in Palo Alto treats many patients who cross the Pacific Ocean for help, Julia May said.
Two surgeries in California
The Mays flew to California in a snowstorm, and Anna underwent her first surgery a few days later, on Feb. 5, with follow-up surgery on Feb. 20. The revascularization of her brain, using vessels from the back of her head, aimed to create blood flow that had been lost.
She also underwent 12 MRIs that were excruciating for her, because the pounding noise of the machine intensified her headaches and it was difficult for her to remain still during the procedure, because she couldn’t control her muscles.
And then there were the side effects of the surgeries, including vomiting and nerve pain that Anna compared to a “bug zapper.”
“She is so brave,” her mother said.
For Anna, courage alone isn’t enough. She believes it’s her mission to educate people about moyamoya.
“I plan to be an advocate,” she said. “I want to bring awareness about the disease.”
Little research on the disease
According to statistics, one in every two million people are born annually with the disease worldwide, Anna said. But because there has been so little research and funding, there may be many people who are undiagnosed.
There could be as many as one in 200,000 people born with moyamoya, she said, and currently, only 200 people in the United States are diagnosed with the ailment each year.
In addition, many doctors don’t know enough about the disease to look for telltale signs such as the gray shadows on a CAT scan, Anna said.
Seek help if have symptoms
She urges people with symptoms to seek help. “I had symptoms years ago,” Anna said. “My hand would cramp up and my handwriting suffered and became chicken scratch. I couldn’t grip the pen, and I would stress out before AP exams.”
From time to time, her speech would become slurred, she said, and the slurring was noticeable when she ate with friends at a diner this past year. “I thought it was from lack of sleep,” Anna said.
Persistent headaches were treated with migraine medication.
She now knows that the lack of blood flow caused by the disease affects speaking and movement, and the trouble she had with the right side of her body reflects impairment in the left side of her brain.
The disease, for which there is no cure, usually manifests itself in children between the ages of 7 and 8.
Because of Anna’s late diagnosis, the disease gained more of a foothold, and her recovery may be slower, Julia May said.
“We believe [the surgeries were] a success,” she said. “The prognosis is good. If you don’t have surgery, you’re at risk for a catastrophic stroke. She was at the end. We outran the stroke.”
Anna “still has issues,” her mother said, including headaches, extreme fatigue and nerve pain.
These should resolve themselves, she said. Anna’s new neural network “is like rooting grass,” her mother said. “It’s becoming stronger.”
'Going to be a part of me'
Anna has to drink 16 ounces of fluid every two hours to hydrate her brain, and she has to keep an eye out for stroke-like symptoms for the next several months.
“Moyamoya is always going to be a part of me,” she said. “I want to make sure it doesn’t become me. Now I have a direction. I want to help people. I want to be hands-on.”
She’s considering a career in nonprofit organizations or public relations in the human services field, or a position such as a child life specialist within a hospital setting.
Appreciation for support of others
The Mays stayed at the Ronald McDonald House in Palo Alto at no cost for six weeks during the time of Anna’s surgeries.
Staying there with others going through serious illnesses gave Anna a boost. “I felt like wasn’t alone,” she said.
Julia May’s mother from Georgia stayed with Anna’s younger sisters, Lily, 8, and Maggie, 16, while Anna and her mother were in California, and Anna’s father flew from coast to coast for the surgeries in between business trips.
The letters Anna received daily from her friends at Shelton High were “encouraging,” her mother said. “It helped her spirits so far from home.”
Beth Smith, Shelton High headmaster, was “very accommodating,” Julia May said, and was “very involved in Anna’s progress. She rallied the students.”
Casey Degennaro, Anna’s high school guidance counselor, also took a personal interest in her recovery and progress, Julia said.
Tie-dyed wristband fund-raiser
Tie-dyed wristbands are being sold to raise money to help the May family with extensive medical expenses. Each surgery cost $300,000, and health insurance doesn’t cover the entire expense.
Fund-raisers have been conducted during Anna Day at Booth Hill Elementary School and Pajama Day at Shelton Intermediate School.
And Anna, described as “a giver” by her classmates, was helping others even while she was suffering, by making baby blankets for children in Stanford Hospital.
“She wanted to give back,” her mother said.
World Moyamoya Day was May 6
The second annual World Moyamoya Day on May 6 aimed to raise awareness about the disease and collect 10,000 signatures to push for more funding, Anna said.
As the school year moves to its conclusion, Anna is trying to participate in as many activities as possible.
A varsity tennis player, she played in two matches this spring, participated in a 5K race with her boyfriend, attended some classes in person and resumed a part-time job.
She’ll have a follow-up medical examination in August and wants to attend the University of New Hampshire this fall.
“We still have hurdles to overcome,” said Julia May, but her daughter’s attitude helps her carry on. “She’s a very ambitious kid,” Julia May said.