Doctor pioneers surgery to treat kids with rare disease
GREENWICH — Steadying his fingers, Dr. Scott Wolfe threads a suture the width of a strand of hair into a needle too small for the naked eye to see.
If he drops the needle, he will need a magnet to find it.
When Wolfe, an internationally recognized peripheral nerve surgeon, looks through a microscope at the Hospital for Special Surgery in New York City as he joins together nerves, he is transported to a world where every action is magnified and time slips away.
The Greenwich resident is restoring movement to the muscles of a patient paralyzed by acute flaccid myelitis, a rare but serious condition that flares up every two years. It mostly affects children, who develop symptoms of a gastrointestinal or respiratory infection, but then lose movement in their arms or legs days later.
“It’s quite dramatic, quite sudden, and just absolutely lifechanging,” said Wolfe, who has pioneered a surgical treatment for patients whose arms, hands and shoulders are paralyzed by AFM, which many call a poliolike illness.
The virus that is likely responsible, enterovirus 68, is elusive: It disappears from the body after it enters, is exceedingly difficult to isolate and elusive to research. There are no medicines available.
Wolfe, who also has an office in Stamford, first encountered the disease when a doctor referred to him a patient whose brachial plexus — a network
“It’s quite dramatic, quite sudden, and just absolutely lifechanging.” Dr. Scott Wolfe, peripheral nerve surgeon who has pioneered a treatment for patients paralyzed by AFM
of nerves that starts in the spinal cord and extends into the underarm — was paralyzed by AFM.
The surgeon, a specialist in nerve injuries to the brachial plexus, recognized AFM’s frightening symptom of paralysis and knew he could treat that. Wolfe has used nerve transfer surgery to treat many patients who have damaged this network, such as motorcyclists and athletes who damaged this network in serious crashes.
Adapting the surgery for patients with upper extremities paralyzed by AFM had never been done before, but Wolfe saw no reason why it would fail. His patient agreed to the surgery, which was a success, and Wolfe published his findings.
Since the diagnosis was first recognized in 2014, AFM has spiked every two years, according to the federal Centers for Disease Control and Prevention. Before 2014, the disease likely existed but
was misdiagnosed, said Dr. Amy Moore, who treats children with the illness at Washington University in St. Louis and runs a test lab researching it.
Most cases develop during a sixweek period from the end of August to October, she said. For the first six months of 2019, the CDC has confirmed seven cases. There were 230 confirmed cases in 2018, 35 cases in 2017, 149 in 2016 and 22 in 2015. Children make up 90 percent of the cases.
“We don’t know enough about it,” Moore said. “We don’t know why a family is sick with a cold and only one of five children come down with AFM. It crosses races, economics and vaccinations. There’s not a good way to organize patients, which adds incredible complexity to treating them.”
Children with AFM go to Johns Hopkins Hospital, Shriners Hospitals for Children in Philadelphia, Children’s Hospital Los Angeles and Washington University Hospital for treatment, she said.
Going forward, the doctors treating AFM should focus on making care standard and studying outcomes of surgeries, Moore said. Her group aims to understand more about the virus before the next large outbreak is expected to occur in 2020.
“We need to be the ones influencing change,” she said.
Only a handful of doctors, including Wolfe and Moore, treat patients paralyzed by AFM, which is a rare subtype of transverse myelitis. In the condition, inflammation to the spinal cord interrupts the messages these nerves send throughout the body, causing pain, muscle weakness and paralysis.
Wolfe has also treated patients with transverse myelitis, including Kale Hyder, who is now a student at Johns Hopkins University studying medicine.
Hyder was a healthy high school student who played basketball but his future ground to a halt when he inexplicably lost movement in his arms and legs, which turned out to be transverse myelitis.
Leg movement returned over time while Hyder went in and out of physical therapy, but he still could not use his arms. Then he heard about Wolfe, who offered Hyder the treatment for patients with AFM, since the recovery looks the same for both. Hyder took the chance.
“I was just happy I had an option,” he said. “No one presented me with an option before.”
Wolfe exhibited a kind of confidence that made Hyder, who was unsure about the treatment when he first visited Wolfe, trust him with such a lengthy surgery.
“He’s a very good, charismatic person, he makes sure all your questions are answered,” Hyder said.
Wolfe performed two backtoback surgeries on Hyder’s arms, but the teen had to wait until the nerves grew to see if it worked. After a long recovery in the hospital with his limbs suspended, the teenager started going to therapy, and after nine months, he started seeing results.
Hyder still does physical therapy to increase his walking endurance and leg strength. In fact, he is participating in a research trial where he trains while wearing a mask that limits his oxygen to see whether that increases his endurance. He has not passed out — yet.
“It’s pretty cool, but it’s pretty tough,” he said. “But it’s interesting to be a part of research as a patient. Eventually I’ll be a researcher or physician, so I am getting both sides.”
The parent to nowgrown children who went through the Greenwich Public School system, Wolfe feels empathy for the children with these injuries and the parents searching for treatment.
“To see a child paralyzed is heartbreaking,” he said. “But to see the hope in these kids’ eyes and the determination they have to get better is absolutely inspiring and invigorating. And the trust that they put in you just sort of propels you forward.”