Two years ago, Christa Bottomley’s healthy 5-year-old son caught a viral cold like the rest of the family. Everyone else recovered, but in his case the virus triggered a neurological condition.
The condition is called acute flaccid myelitis, or AFM. Though rare, it has jumped onto the national radar. As doctors struggle to explain its third nationwide peak since 2014, families like the Bottomleys are trying to provide hope to others hit with the sudden, polio-like disease and to push for more awareness in the medical community.
AFM causes inflammation of the nervous system, particularly the gray matter of the spinal cord, which results in weakening muscles in usually one or more arms and legs. It can also affect the face and lead to difficulty swallowing or even breathing. Almost all AFM patients are hospitalized for several days or even months.
It affects mainly children, who sometimes recover over time, often with therapy. Others remain paralyzed, needing long-term care and rehabilitation.
Sebastian was in a hospital in Hershey, Pa., for three weeks and lost movement in both arms and legs. He couldn’t sit up or turn his head in one direction. He spent another 60 days at Kennedy Krieger pediatric hospital in Baltimore, but still couldn’t move his right arm or either leg.
He has since had some mobility restored through surgeries and rehabilitation. He can walk with a walker and leg braces, though he still predominantly uses a wheelchair outside of the house. He must travel from his home in York, Pa., to Kennedy Krieger for weekly therapy.
“I wish people had listened in 2016 when we said this is coming,” Ms. Bottomley says. “Nobody wanted to listen. I think it will be the same thing in 2020, and hopefully we will have their attention then.”
Every two years since 2014 the Centers for Disease Control and Prevention has documented a spike in AFM, almost always in children. The CDC is investigating 219 reports of patients with AFM so far this year, with 80 cases in 25 states confirmed, up from 33 the year before. In 2016 the CDC confirmed 149 cases of AFM and in 2014 there were 120 cases.
Doctors and the CDC differ on the possible cause of the condition and how to treat it. Many doctors speculate AFM is largely fueled by a common virus called the enterovirus, which for most people causes nothing more than a routine upper-respiratory infection. But the CDC believes there could be other culprits.
Some experts believe the condition likely existed before 2014 and think there could be more cases, since there is no national requirement to report as there is for many other conditions, including influenza and measles.
“If there was mandatory reporting, it would help with surveillance,” says Keith Van Haren, an assistant professor of neurology at the Stanford University School of Medicine. “This doesn’t appear to be going away. We need to be preparing and anticipating the possibility of a more severe outbreak.”
More than 50 researchers formed a working group in October to identify the mechanism of the disease and come up with better diagnosis and treatment standards for medical professionals.
In 2014, experts first noticed a link between myelitis—inflammation of the spinal cord—and the enterovirus D68 strain. This year, doctors discovered another possible link, to enterovirus strain A71.
So far this year, Children’s Hospital Colorado has treated about a dozen children with AMF. The majority of them have tested positive for enterovirus A71, with only one testing positive for the D68 strain. The good news, says Samuel Dominguez, a pediatric infectious disease doctor at the hospital, is that most of the children with enterovirus A71 have already recovered. The one with enterovirus D68 hasn’t.
The CDC isn’t focusing just on enterovirus as the main trigger. When samples are taken from the respiratory tract, a sizable portion of patients have tested positive for enterovirus—but not the majority, Kathleen Dooling, medical officer at the CDC and incident manager for their AFM response, says.
Priya Duggal, director of the genetic epidemiology program at Johns Hopkins Bloomberg School of Public Health, is conducting a study to see if the AFM victims share any relevant genes.
Her research group has collected saliva samples from 60 AFM patients and their family members and extracted their DNA so they could sequence their genome.
Suzanne Rybczynski, medical director of the pediatric rehabilitation inpatient hospital at Kennedy Krieger, says they have had six AFM inpatients this year from all over the U.S., with more seeking to be admitted. Some children will go on to receive nerve-transfer surgery, in which nerves that are still working are diverted to help power another muscle group.
The patients come to Kennedy Krieger when they are medically stable—though some are still on ventilators—and ready to start rehabilitation. That includes using robotics and electrical stimulation to help the body’s muscles remember how to move, as well as aquatic and animal-assisted therapy, Dr. Rybczynski says.
On visits there in recent months, Ms. Bottomley has met other families with young children going through the same experience.
“I tell them the recovery will be long and slow, but if they do a little bit of physical therapy every day, that helps the most,” she says.
Among those Ms. Bottomley has met is Chris Carr, from Roanoke, Va., whose 4-year-old son, Camdyn, has been on a ventilator for more than a month.
Camdyn is improving and recently started talking again, but 85% of his body remains paralyzed, including the left side of his face. “He can move his right leg, can wiggle his left toes and can squeeze with the left hand,” says Mr. Carr, who had to quit his job as a welder to stay with his son. “He can’t sit up on his own, he can’t use the bathroom, he can’t do anything.”
Mr. Carr says doctors told him the AFM was caused by the enterovirus, but he isn’t sure which strain. He is already thinking about surgery, but will have to wait at least six months for that. Meanwhile, they will do physical therapy and hope for more improvements.
“It is life-changing,” Mr. Carr says. “My child could need assistance to live for the rest of his life, well past when I’m dead and gone.”
Some Hope for Recovery
Nerve-transfer surgeries are giving some AFM patients movement that their families thought they would never have again.
At age 6, Ariel Serrano was among the first cluster of AFM patients in the fall of 2014. She regained mobility naturally, except in her right arm, and was accepted for surgery, in which doctors take nerves that are still working and divert or relocate them to help power a different muscle group.
Only a few doctors in the country perform such surgeries for AFM patients. Among them is Mitchel Seruya, director of the Brachial Plexus and Peripheral Nerve Center at Children’s Hospital Los Angeles, who removed a muscle from Ariel’s inner thigh and transferred it to her right arm. He then connected rib nerves to the new muscle in her arm.
Now, Ariel can bend her elbow to pick things up and feed herself, though she is still working on her strength, says her mother, Ingrid Herrera. She is back to swimming and took first place in a recent judo competition.
When the whole arm is paralyzed, nerves must be diverted from the ribs and children must learn how to activate their arm by coughing or whistling. But eventually the brain learns to do it automatically.
Some doctors say because nerve transfers in AFM patients haven’t been systematically studied, they shouldn’t be considered a cure. “It may help gain function in one muscle group, but typically there’s not a dramatic benefit,” says Keith Van Haren, an assistant professor of neurology at the Stanford University School of Medicine.
How successful the surgeries are can depend on which part of the body is targeted. Dan Zlotolow, a pediatric upper-extremity surgeon at Shriners Hospital for Children in Philadelphia who has performed 17 surgeries on AFM patients, says he has had a lot of success on elbow surgeries, but less with the shoulder.
The only surgeon who has attempted to do nerve-transfer surgeries in the legs of AFM patients is Amy Moore, an associate professor in plastic surgery at Washington University in St. Louis.
She usually transfers nerves that move the toes to nerves that stabilize the hip or move the knee. Because nerves grow an inch a month, it can take up to a year to see effects.
“It’s not making them normal, but it is providing them with the hope and the potential that one day they may be able to stand or even walk,” she says.
Write to Sumathi Reddy at firstname.lastname@example.org