A rare polio-like disease that has plagued at least three Long Island children since the national epidemic began in 2014 usually begins as a common viral infection. ear infection in august 2018 A week later, her left foot ached and she stumbled several times as she walked. The next day her left leg became paralyzed and within 24 hours most of the body under her neck became immobile.
"I felt helpless," says Emma's mother, Valerie d'Antonio. "We didn't know why it was happening, we didn't know how it was happening, and we didn't know what to do to stop it. At one point, my little girl is well and healthy, doing gymnastics and swimming, and in the next minute she struggles to be well. "
After three incorrect preliminary diagnoses, Emma was diagnosed with acute loose myelitis, a disorder of the nervous system. The disease damages neurons in the gray matter of the spinal cord that are related to motor function, said Dr. Carlos Pardo-Vilamisar, a professor of neurology and pathology at Johns Hopkins University in Baltimore and principal investigator for acute myelitis patients who scientists hope to start next year.
There is still no cure for the disease, doctors said. And it's unclear what effect the disease will have on life expectancy, Pardo-Vilamizar said.
Most people with acute loose myelitis experience sudden weakening of the legs or arms and loss of muscle reflexes, Centers for Disease Control and Prevention officials said in an email. The disease can weaken the muscles needed for breathing. Nearly one in four people diagnosed with the disease in 2018 needed a ventilator to breathe.
The Federal CDC has confirmed nearly 600 cases of the disease – mostly in young children – since follow-up began in 2014. Several people have died and in some cases the cause of death has been linked to complications from the disease, CDC officials said . Probably the case number is underestimated because of misdiagnoses, Pardo-Vilamizar said.
Evidence suggests that the virus is the cause of the disease, but research continues at its core, Pardo-Vilamisar said. A study led by researchers at the University of California-San Francisco and published on October 21 in the journal Nature found that the spinal fluid of children with acute cold myelitis has antibodies to a group of viruses called enteroviruses. Antibodies are produced by the immune system to protect the body from harmful substances such as enteroviruses.
Acute loose myelitis usually strikes in late summer or fall, which is when enteroviruses usually circulate, says Pardo-Vilamizar.
When Emma was taken to an emergency ward in a small town in Pennsylvania near which the family was resting, a doctor in the emergency ward recognized the severity of the paralysis and the weakening of the muscles that spread throughout her body and consulted a neurologist in the a large hospital. The neurologist feared that Emma could stop breathing and die if she wasn't sent for specialized treatment, Valerie D & # 39; Antonio says.
Some doctors do not realize the need for immediate help, say the parents of children with the disease.
When Louis Dallojacono, 6, of Farmingdale was taken to pediatricians and then in an emergency room at a hospital in 2016, doctors and nurses did not know what to do about the growing weakness in his legs, his mother Teresa said Daloyakono, 48.
After Dallojacono told a pediatrician that he sometimes had to wear it because his legs were so weak, he told her Louis, then a 3-year-old, probably didn't feel like walking and said : "I think you just gave birth to him, so give him ibuprofen and give me a call if it gets worse. "
More than two weeks after the onset of leg weakness, Louis was diagnosed with acute myelitis cotton, Daloyakono said. Since then, he has improved with physical therapy, she added.
There is no consensus on what treatments are effective for condition, said Emma's neurologist, Dr. Anusha Yeshokumar, associate professor of neurology and pediatrics at Icahn Medical School on Mount Sinai in Manhattan.
"We believe [physical] therapies are very useful, but we have no idea what therapies are and how often and if there are medicines that can help and regeneration [nerve] "she said. "All these studies are in the early stages."
Some patients with acute loose myelitis improve significantly after paralysis, but "almost all patients remain with some neurological impairment," such as difficulty in grasping or holding objects, Pardo-Willamisar said. . Others remain severely disabled. Patients with severe disabilities, such as major deformities of the spine, are likely to have shorter life spans, he said.
An operation in which healthy nerves are transferred to a muscle group affected by acute cold myelitis has helped some patients, Pardo-Vilamizar said. "But it only works with muscle groups that have the potential to regenerate, not with" dead "muscles, he said.
Antonio, 50, plans to take Emma to Baltimore so Pardo-Vilamisar and his team can determine if Emma is a candidate for the operation.
Emma has improved after her diagnosis. Her left leg is still paralyzed, but she can now move her right leg and toes, though not completely, D'Antonio said.
Five days a week, Emma travels to St Mary's Children's Hospital in Bayside, Queens, for an hour and a half of physical therapy and musculoskeletal training.
Traditional Physical Therapy often aims to teach patients how to adapt to a disability so that they can be as mobile as possible in a bracelet, walker or wheelchair, said Kelly Dougherty, lead physiotherapist for the Stomach Locomotive Training Program. Mary.
"We are trying to get the nervous system to do the job the same way they were before they were injured," said Dougherty.
Emma was fastened to her belt recently in the morning to keep her from falling as she walked along a treadmill. Physiotherapist Caroline Segota and two recovery technicians kept Emma's pelvis and legs moving and bending her legs as if walking alone while touching the muscles in her thighs that matched the movement.
Emma sometimes gets frustrated when she can't do things that other children can do. On Halloween, she started crying because she couldn't keep herself in a wheelchair with friends while they were running from house to house – tricks or treats, Antonio said.
Antonio organizes Emma's birthday party last month at the pool, because with the help of weekly swimming lessons and aqua therapy, Emma can swim well.
"It was great because you don't know in the pool that she is paralyzed because she swims and goes home," she said.
Another Long Island kid with acute cold myelitis, 7-year-old Austin Bird of Merrick, went to physical therapy and locomotor training with Emma, but a few weeks ago his mother, Jamie Bird, 42, had to move him to physical therapy elsewhere. because health insurance pays only a fraction of the cost. The insurance company later agreed to a better recovery contract and Austin will soon return to St. Mary's and continue other physical therapy, she said.
Dallojacono and her husband pay tens of thousands of dollars out of their own pockets for Louis' physical and occupational therapies because of their limited insurance coverage until they join the state's Child Health Plus insurance program.
Austin was diagnosed with acute loose myelitis more than two years after Louis and a few weeks after Emma, Bird said. One day in September 2018, Austin had trouble breathing. It seems to be recovering with the help of steroids. A few nights later he woke up with a fever and a severe headache. By the time the emergency doctor saw him a few hours later, Austin felt much better and was sent home, Bird said.
However, with the day, Austin's legs became weaker and weaker. Byrd took him back to the emergency room and he was hospitalized. As with Emma, doctors initially thought he had another neurological condition before being diagnosed with acute loose myelitis.
"It was devastating," Bird said. "I refused for a very long time. I kept thinking that he would wake up and be able to move his legs again.
Like Emma, Austin made significant improvements. Although Austin and Emma haven't been in physical therapy together for several weeks, they still hang out on the weekends.
"From the day they met, they felt safe that they could play together and know what they were doing," Bird said. "They really push each other and take pride in each other's progress."
Austin knows he's different from other kids, but "when he's with Emma, he's just himself," she says.
Austin attends school full time, absent only two hours a week for physical therapy. Emma, with long daily commutes to and from Queens, receives one-on-one lessons. She also attends two classes at a local school, mostly for socialization, said Antonio.
Antonio urged parents to take seriously any unexplained weakness in the legs or other parts of the body – however slight – seriously. She has heard story after story of children diagnosed later with acute cold myelitis sent home because doctors did not think the weakness signaled anything serious.
"Insist on more tests," she said. "Don't feel like a crazy mother. If you feel something is off, it might be. This is very rare, but when it happens to you, it is not so rare at all. ”
Acute floccacid myelitis system
- The most common symptom is the sudden onset of weakness of the leg or arm and loss of muscle reflexes.  More than 1 in 4 people in 2018 with confirmed cases of acute cold myelitis need breathing assistance; mechanical ventilation is most needed.
- As of 2014, more than 90% of patients with acute loose myelitis had mild respiratory disease or a fever of viral infection before they began to experience muscle weakness.
OF THE NUMBERS
- 596: Claims Across the Country from 2014
- 15: Cases in the State of New York from 2014
- 28: Claims Across the Country in 2019 as of November 1
- 236: Confirmed cases in 2018
For unknown reasons, the number of cases has increased and decreased in alternating years. The CDC is reviewing the MRI scan to evaluate the number of cases before 2014, the agency's employees said in an email.
SOURCES: Centers for Disease Control and Prevention in the United States