A rare polio-like disease that has struck at least three Long Island children since a nationwide outbreak began in 2014 typically starts as a common viral infection.
For Emma D’Antonio, 8, of Bohemia, it was an ear infection in August 2018. A week later, her left leg started hurting and she stumbled a few times while walking. The next day, her left leg became paralyzed, and within 24 hours, most of her body below her neck was immobilized.
“I felt helpless,” said Emma’s mother, Valerie D’Antonio. “We didn’t know why this was happening, we didn’t know how this was happening, and we didn’t know what to do to stop it. One moment my little girl is fine and healthy and doing gymnastics and swimming, and the next minute she’s fighting to be OK.”
After three incorrect tentative diagnoses, Emma was diagnosed with acute flaccid myelitis, a nervous system disorder. The disease damages neurons in the gray matter of the spinal cord that are associated with motor function, said Dr. Carlos Pardo-Villamizar, a professor of neurology and pathology at Johns Hopkins University in Baltimore and co-principal investigator for a study on acute flaccid myelitis patients that scientists hope to begin next year.
There is no cure yet for the disease, doctors said. And it's unclear how much of an effect the disease will have on life expectancy, Pardo-Villamizar said.
Most people with acute flaccid myelitis experience a sudden weakening of the legs or arms and a loss of muscle reflexes, Centers for Disease Control and Prevention officials said in an email. The disease can weaken the muscles necessary for breathing. Nearly one in four people diagnosed with the disease in 2018 needed the help of a ventilator to breathe.
The federal CDC has confirmed nearly 600 cases of the disease — mostly in young children — since it began tracking cases in 2014. Several people have died, and in some cases, the cause of death was related to complications from the disease, CDC officials said. The case number likely is an underestimate, because of misdiagnoses, Pardo-Villamizar said.
Evidence points to a virus as the disease’s cause, but research continues on the root, Pardo-Villamizar said. A study led by researchers at the University of California, San Francisco, and published Oct. 21 in the journal Nature found that the spinal fluid of children with acute flaccid myelitis had antibodies to a group of viruses called enteroviruses. Antibodies are produced by the immune system to defend the body against harmful substances such as enteroviruses.
Acute flaccid myelitis usually strikes in the late summer or fall, which is when enteroviruses usually circulate, Pardo-Villamizar said.
When Emma was taken to an emergency room in a small Pennsylvania town near where the family was vacationing, an emergency room doctor recognized the seriousness of the paralysis and weakening of muscles that was spreading throughout her body and consulted a neurologist at a larger hospital. The neurologist feared Emma could stop breathing and die if not airlifted for specialized treatment, Valerie D'Antonio said.
Some doctors don't realize the need for immediate help, parents of children with the disease said.
When Louis Dallojacono, 6, of Farmingdale was taken to pediatricians and then to a hospital emergency room in 2016, doctors and nurses didn’t know what to make of the growing weakness in his legs, said his mother, Theresa Dallojacono, 48.
After Dallojacono told one pediatrician she had to carry him at times because his legs were so weak, he told her Louis, then 3, probably didn’t feel like walking and said, “ 'I think you’ve just been babying him, so give him ibuprofen and call me if he gets worse.’ ”
More than two weeks after the leg weakness began, Louis was diagnosed with acute flaccid myelitis, Dallojacono said. He since has improved with physical therapy, she added.
There is not a consensus on what treatments are effective for the condition, said Emma’s neurologist, Dr. Anusha Yeshokumar, an assistant professor of neurology and pediatrics at the Icahn School of Medicine at Mount Sinai in Manhattan.
“We think [physical] therapies are very helpful, but we don’t have a sense as to which therapies and how often, and whether there are medications that may help in [nerve] regeneration,” she said. “All of that research is in its infancy.”
Some acute flaccid myelitis patients improve markedly after paralysis, but “almost all of the patients are left with some neurological disability,” such as difficulty grasping or holding objects, Pardo-Villamizar said. Others remain severely disabled. Patients with severe disabilities, such as major spinal deformities, likely will have shorter life spans, he said.
A surgery in which healthy nerves are transferred to a muscle group affected by acute flaccid myelitis has helped some patients, Pardo-Villamizar said. But it only works with muscle groups that have a potential for regeneration, not with “dead” muscles, he said.
D’Antonio, 50, plans to take Emma to Baltimore so Pardo-Villamizar and his team can assess if Emma is a candidate for the surgery.
Emma has improved since she was diagnosed. Her left leg is still paralyzed, but she can now move her right leg and toes, although not fully, D’Antonio said. She has regained her upper body strength.
Five days a week, Emma travels to St. Mary’s Hospital for Children in Bayside, Queens, for an hour and a half of physical therapy and “locomotor training.”
Traditional physical therapy often aims to teach patients how to adjust to a disability, so they can be as mobile as possible in a brace, walker or wheelchair, said Kelly Dougherty, the lead physical therapist for St. Mary’s locomotor training program.
“We’re trying to get the nervous system to perform the task the exact same way it did before they were injured,” Dougherty said.
On a recent morning, Emma was strapped to a harness to keep her from falling as she walked on a treadmill. Physical therapist Caroline Segota and two restorative technicians held Emma’s pelvis and legs, moving and bending the legs as if she were walking on her own, while touching muscles on her thighs that correspond to movement. That touching of the muscles is to stimulate the nervous system, Dougherty said.
Emma sometimes gets upset when she can’t do things other kids can do. On Halloween, she started crying because she couldn’t keep up in her wheelchair with friends as they ran from house to house trick-or-treating, D’Antonio said.
D’Antonio threw Emma a birthday party last month at a pool because, with the help of weekly swimming lessons and aqua therapy, Emma can swim well.
“It was great because in the pool you wouldn’t know that she’s paralyzed because she swims and gets around,” she said.
Another Long Island child with acute flaccid myelitis, Austin Bird, 7, of Merrick, had been going through physical therapy and locomotor training with Emma, but a few weeks ago his mom, Jamie Bird, 42, had to switch him to physical therapy elsewhere because health insurance paid only a fraction of the cost. The insurance company later agreed to a better reimbursement contract, and Austin soon will return to St. Mary’s, as well as continue the other physical therapy, she said.
Dallojacono and her husband paid tens of thousands of dollars out of their own pockets for Louis' physical and occupational therapies, because of limited insurance coverage, until they joined the state's Child Health Plus insurance program.
Austin was diagnosed with acute flaccid myelitis more than two years after Louis and a few weeks after Emma, Bird said. One day in September 2018, Austin had trouble breathing. He seemed to recover with the help of steroids. A few nights later, he woke up with a high fever and severe headache. By the time an emergency room doctor saw him several hours afterward, Austin felt much better and was sent home, Bird said.
Yet as the day went on, Austin’s legs became weaker and weaker. Bird took him back to the emergency room, and he was hospitalized. As with Emma, doctors at first thought he had another neurological disease before he received a diagnosis of acute flaccid myelitis.
“It was devastating,” Bird said. “I was in denial for a very long time. I kept thinking he was going to wake up and be able to move his legs again. It was like it couldn’t be real.”
Like Emma, Austin has made significant improvements. Even though Austin and Emma haven’t been in physical therapy together for a few weeks, they still hang out together on weekends.
“From the day they met each other they felt safe, that they can play together and know what they’re going through,” Bird said. “They really push each other, and they’re proud of each other’s progress.”
Austin knows he’s different from other kids, but “when he’s with Emma, he’s just himself,” she said.
Austin attends school full time, missing only two classes a week because of physical therapy. Emma, with the long daily travels to and from Queens, receives one-on-one tutoring. She also attends two hours of classes a week at her local school, primarily for socialization, D'Antonio said.
D’Antonio urged parents to take any unexplained weakness in the legs or other body parts — however slight — seriously. She’s heard story after story of children later diagnosed with acute flaccid myelitis sent home because doctors didn’t think the weakness signaled anything severe.
“Push for more testing,” she said. “Don’t feel like you’re being a crazy mom. If you feel like something’s off, it could be. It is very rare, but when it happens to you, it’s not that rare at all.”
ACUTE FLACCID MYELITIS SYMPTOMS
- The most common symptom is a sudden onset of leg or arm weakness and loss of muscle reflexes.
- Nearly 1 in 4 people in 2018 with confirmed acute flaccid myelitis cases needed a mechanical ventilator.
- Since 2014, more than 90% of acute flaccid myelitis patients had a mild respiratory illness or a fever from a viral infection before they began feeling muscle weakness.
BY THE NUMBERS
- 596: Confirmed cases nationwide since 2014
- 15: Cases in New York State since 2014
- 28: Confirmed cases nationwide in 2019 as of Nov. 1
- 236: Confirmed cases in 2018
For reasons still unknown, the number of cases rises and falls in alternating years. The CDC is reviewing MRI scans to estimate the number of cases before 2014, agency officials said in an email.
SOURCE: U.S. Centers for Disease Control and Prevention