It’s a cloudy, mid-December morning, but the long wall of bay windows looking out over Baltimore City lets ample dappled sunlight into the physical and occupational therapy suite at the Kennedy Krieger Institute. And Elijah Yanike, of Taneytown, brings his own light anyway, the 5-year-old energetic as he and his mother Lauren greet Michelle Bebo, Elijah’s occupational therapist of the past month.
“Game Mario?” Elijah asks, hopeful.
“We can do Mario at the very end,” Bebo replies, redirecting. “But you know what we can do instead? The robot arm.”
The robot arm looks like a structured sling that fits over Elijah’s right arm and shoulder, but it functions like a powered exoskeleton, according to Bebo.
“It’s an upper arm orthotic, is what it’s called,” she says. “Sensors pick up electrical signals in the muscles so Eli is able to start the contraction himself, and the device is able to listen for those electrical signals and then further help Eli move his muscles.”
With the assistance of the orthotic, Elijah first uses a cardboard tube to knock stuffed targets suspended from a hanger, before engaging in a short duel with his mother, who chooses a plastic blue bat for combat.
It takes a good eye and careful observation to notice anything different about Elijah than any other boy his age as he plays in the therapy suite. A slight preference for using just his left hand, leading a bit more with his left shoulder.
But just a couple of months prior, it wasn’t clear if Elijah would even be walking.
Starting with a sniffle
Diagnosed with acute flaccid myelitis, a polio-like illness that can bring on sudden paralysis in children, Elijah went from what seemed like a bad cold in late September, to being unable to move is right arm, sit up or lift his head by October.
“Eli had a cold and then he was fine after a couple of days,” Lauren Yanike says. “Then he woke up one morning, and he was complaining of head and neck pain. He was crying, it hurt so bad.”
Two emergency room visits supplied treatment with Tylenol, what would turn out to be a false positive for mononucleosis, according to Yanike, and the hope that Elijah would get better with rest.
“But he didn’t get better. He became more paralyzed, and we [Yanike and her husband Anders] ended up calling the pediatrician a few days later and said, ‘Look, we’re carrying him around the house,’ ” Yanike says. “We were pretty scared. We had never seen him that sick in his entire life.”
It was Oct. 11 when Ashley Davis, Elijah’s pediatric nurse practitioner at Westminster Pediatrics, got the call from the Yanikes. Elijah’s previous symptom — headache, lethargy, lack of appetite — all fit with a viral illness. But the new symptoms of paralysis sounded concerning, as did Yanike’s own concern.
“I always tell my parents, ‘Trust your intuition,’ ” Davis said in an interview. “There are certain things like high fevers, changes in your child’s condition and neurologics — sudden arm or leg weakness, facial drooping, difficulty moving the eyes or eyelids, slurring of speech — that are all things that you should bring your child to the doctor for, because even if it’s not acute flaccid myelitis, it’s something.”
Davis referred Elijah to Sinai Hospital where pediatric experts could examine him and try to get to the bottom of what was going on.
“They did a series of tests, called doctors nationwide with Eli’s symptoms and all the doctors nationwide agreed that symptomatically, and with some of the testing that had come through, that Eli had acute flaccid myelitis,” Yanike says.
Acute flaccid myelitis, or AFM, is a rare but recently increasing phenomenon, according to the Centers for Disease Control and Prevention website. Larger numbers of cases have been appearing every two years, in 2012, 2014, 2016 and, as with Elijah, in 2018, according to the CDC, with the majority featuring disturbing common characteristics: previously healthy children developing paralysis, often following a viral illness.
“It’s what we call a polio-like illness, because it affects the same part of the nervous system that the polio virus does,” said Elijah’s pediatric neurologist at Sinai Hospital, Dr. Peggy Lazerow. “We think it’s caused by specific viruses, but we are not able to identify all of them and it usually leads to varying symptoms that can be partial or near total paralysis.”
Diagnosis consists of the onset of paralysis plus the use of imaging, such as MRI, to confirm a lesion, or damage, to the gray matter in the anterior horn of the spinal cord, according to Lazerow. That’s the portion of the spinal cord nerve tissue responsible for conducting movement signals from brain to body.
Where that lesion is can determine the degree of paralysis, Lazerow said, “although it doesn’t always correspond really well with the lesion we see on the MRI, which is why it’s not totally, completely understood.”
Further complicating things is that while a preceding viral illness seems to be involved in AFM, no one virus or other cause has yet been identified, according to Lazerow. It’s something of a medical mystery.
“I don’t think it’s terribly unusual in the profession, but I do think it’s an unusual concept for the public,” she said. “We can’t prevent our children from getting random viruses and there’s no way to predict who will react poorly to the virus. So there is nothing in our power to prevent it, which is what I think is so scary for parents.”
A rare but rising risk
The CDC has confirmed just 537 cases of AFM since 2014. That it’s still rare is good news, but in some ways problematic, according to Dr. Thomas Clark, medical epidemiologist and deputy division director of the division of viral diseases at the CDC.
“Because it’s rare, it makes it difficult to do the science,” he said in an interview. “It also makes it challenging to study any specific treatments that might benefit kids with AFM.”
Although AFM is polio-like, it has, so far, defied association with a clear cause like the polio virus, Clark said, and it may turn out it has no singular cause at all.
“We don’t think there is clear and definitive evidence for a single cause of AFM because most of the time when we test for viruses in patients, we don’t find it. About half the time we don’t find the evidence of any virus,” he said. “But a lot of things point us to viruses being involved.”
In particular, a respiratory illness with a fever a few days before the onset of paralysis is a common theme, according to Clark, and AFM cases do seem to have a strong seasonality, he said, with most cases occurring from August through November in peak years, a time when respiratory illnesses are circulating.
An enterovirus known as EV71 that causes hand, foot and mouth disease has been associated with some cases of AFM-like paralysis in Asia, Clark said, while in the U.S., the 2014 peak of AFM cases was associated a widespread outbreak of severe respiratory illness caused by Enterovirus D68. But testing has not been able to establish a firm link between the virus and AFM.
“We look in the spinal fluid of patients and tests for viruses, and if you found evidence of a virus in the spinal fluid of someone with AFM, we would consider that to be the cause of AFM,” he said. “But in over 500 cases tested, we’ve only found four with evidence of a virus in their spinal fluid.”
Figuring this out is an active project at the CDC, according to Clark, and there are leads, such as the peaks in AFM cases every two years. That’s a trend, which if it continues, would mark 2020 as the next year to be concerned about.
“A lot of folks working on AFM feel like this biannual pattern and seasonality should be a clue,” Clark said. “But it doesn’t quite jibe — we don’t know exactly if there is one cause or what the triggers are for AFM and what’s the cause of this unusual epidemiology.”
That’s why the CDC is asking public health officials, doctors and parents to keep an eye out and report cases of AFM, according to Clark, to help the organization get a better picture of the epidemiology of AFM and hopefully help solve the puzzle.
“We ask that cases be reported to health departments as soon as they are suspected,” he said. “The sooner we are aware of cases, the sooner we can get the right specimens for laboratory testing at the right time.”
Speed can be of the essence in the treatment of individual cases of AFM as well, and Elijah’s doctors agreed that it was a good thing his parents persisted in getting to the bottom of his problems quickly.
“Eli was fortunate in a sense because a lot of the kids that are presenting, especially in this area, with acute flaccid myelitis are actually more severely affected than Eli was,” Lazerow said. “Having respiratory problems and needing to be intubated, needing to be in the intensive care unit.”
Without a clear cause of AFM, there are also no clear guidelines for its treatment, according to Lazerow, but the current treatment of choice is to give intravenous immunoglobulins, or IVIG, proteins produced by the body’s immune cells to help fight disease. Not everyone responds to that treatment, but Elijah did.
“The one thing that gave me the clue that he might be responding well to the IVIG is that he actually didn’t have any reflexes,” Lazerow said. “You know how we use the reflex hammer to bang on the knees? He didn’t have any reflexes before we gave him the IVIG, and after his reflexes came back.”
For patients who don’t respond to that treatment, there are limited options, according to Lazerow. Steroids and plasma-exchange therapies have been tried, she said.
“What I mention to parents is the emerging possibly of doing nerve transfer in these kids, which is a neurosurgical procedure to regain some function. And that’s very new. There’s been some research done on that, but it’s very new and on the horizon,” Lazerow said. “The timeline for that, who would benefit the most from that, how to go about doing that is really not entirely clear, so it’s really an active area of research.”
Until then, the treatments available are what they are, and they have mixed results.
“There are some kids that have a pretty poor prognosis, unfortunately,” Lazerow said. “It is not the type of situation where we treat and expect full recovery in everyone.”
But when it comes to maximizing recovery, there are a lot more options than in the immediate treatment of AFM cases.
“There isn’t agreement on the acute treatments, but there is agreement from everybody that rehabilitation is really the best treatment at this point for children with AFM,” said Janet Dean, a pediatric nurse practitioner who oversees the rehabilitation program used by Elijah at Kennedy Krieger.
“What we do here is called activity based therapy,” she said. “We’re not the exclusive place that does activity based therapy, but I believe we do it a lot more aggressive than other places.”
Kennedy Krieger is currently treating or has treated 21 cases of AFM that emerged in 2018 alone, with patients from 11 states, according to statistics provided by an institute spokeswoman.
That treatment ranges from the electrical stimulation Elijah experienced with the robotic arm, to more sophisticated machinery designed to help children more severely affected utilize their legs and practice walking to build strength.
“We have children that their legs are working fine, but they can’t hold their heads up and they can’t breathe,” Dean said. “So we put them up in a partial body weight support harness and help them walk on a treadmill.”
Elijah, fortunately, hasn’t needed that much support, according to Yanike.
“He was considered a more mild case and he has come a long way,” she says, as Elijah and Bebo prepare another exoskeleton-like tool, this one connected to a video game.
“I wouldn’t say he’s fully recovered — a lot of these kids, they still don’t know if full recovery is even possible — but he has come a really long way. He’s running, he’s walking, he’s jumping. He’s holding his neck up really well,” his mother says.
Carroll County Breaking News
“We have seen a lot of progress, especially with Eli recently, with how much he is using his arm more on his own even without us prompting,” Bebo adds, as Elijah begins using what looks like the arm of a dental light to control a fish net in a video game based around saving fish from trash in the ocean.
“It’s very motivating for him,” Bebo says. “He gets in a lot of movements for a long period of time and sometimes doesn’t even realize how much he’s using his right up, because he gets so into the games.”
Keeping watch and being a good advocate
Thinking about lessons others could take from her family’s experience, Yanike says she doesn’t want to scare anyone — AFM is still a very rare disease. But given that it is increasingly out there, and affected her family, she thinks it’s important people know to be on the lookout.
“If there’s one thing I could tell parents, I think it’s just important to be aware of AFM,” Yanike said. “If you see the signs and symptoms, follow your gut, be a good advocate for your kid.”
That’s a lesson that comes doctor approved.
“Have what we call in medicine a ‘high index of suspicion,’ ” Lazrow said. “If you feel that something is not right with your child, you should take them to the pediatrician and just be very vigilant.”
“A good rule of thumb for parents is to always be a good advocate for your kids and have a good communication,” added Davis, the nurse practitioner at Westminster Pediatrics. “I always say there is no dumb question and there is no dumb office visit. You should bring your kid in, and if you’re concerned, then I’m concerned.”