The diagnosis was in, but Jessica Kulakowski still didn’t have the answers, so she started scouring the internet to find out why her son’s cold had left his right arm virtually lifeless.
Online she found case studies but nothing definitive about why her then-1-year-old boy, Chase, contracted the rare polio-like condition known as acute flaccid myelitis, or AFM. The condition, in the news as a spate of new cases have made headlines in Illinois and beyond, affects mostly children and causes muscles to atrophy, leaving some bedridden with paralysis and unable to breathe on their own. Medical experts have in some cases linked the syndrome to an enterovirus, which causes the common cold and other respiratory illnesses.
Two years after her son’s diagnosis, questions linger for the northwest Indiana mom, who worries anew each time the toddler complains of an ache or has the sniffles: Could this be another complication related to AFM?
“It’s like living in paranoia because there’s just no answer to anything,” Kulakowski said.
Nationwide, the rare condition has gotten more attention after federal health officials reported an increase in cases in recent weeks. They include at least 10 cases diagnosed in Illinois and at least seven in Minnesota, leaving parents like Kulakowski perplexed: How could such a complicated medical journey begin with their little one’s runny nose?
Now 3 years old, Chase races his toy dump trucks through the family’s Dyer, Ind., home, a stark difference from when the paralysis of his right arm caused him to lose his balance. His parents remember the agony of watching him fall as he tried to walk. But a nerve transfer — a procedure that restored movement to his shoulder and elbow — and continued physical therapy has enabled him to use his right arm again, though he continues to build up its strength. In therapy he’s using a bat and knocking things down to strengthen his arm.
“I’m not sure he’s ever going to fully recover from it,” Kulakowski said. “I don’t see it as a realistic outcome. I think he’s always going to know he’s different.”
Paralyzed after nap
Chase’s medical journey started with a runny nose in October 2016 that doctors initially thought was a respiratory infection. Then seemingly over a two-hour nap, Chase’s right arm became paralyzed. He had been under the watch of a baby sitter, but the caretaker only noticed that the boy was tired that day.
David Kulakowski, Chase’s father, knew something was seriously wrong that night while getting his son ready for a bath. He took off his son’s shirt and saw the small arm “just flop.” The father picked up the arm, let it go and watched the lifeless limb fall down. That prompted the couple to take the boy to a nearby urgent care center, where they were told it appeared the boy had AFM.
Skeptical of the quick diagnosis, the couple took the boy to two hospitals for second opinions. Chase spent three days at Lurie Children’s Hospital in Chicago where he underwent an MRI. He was diagnosed and discharged with a list of options for physical therapy.
What Chase was diagnosed with is so rare that the federal Centers for Disease Control and Prevention reports that it affects less than 1 in a million people. Since 2014 there have been 386 confirmed cases of AFM. While the condition itself isn’t believed to be contagious, the viruses that may lead to AFM are. On its website, the CDC explains that nonpolio enteroviruses — which normally don’t make people sick, and if they do, it’s often the common cold — can cause AFM.
Jessica Kulakowski found support in an online community of parents seeking answers about the condition. Chase had been around other children who probably got the same virus. Yet he was the only one who developed AFM.
“Why it continued to his spinal cord? They don’t know,” she said. “That’s the one thing they can’t answer, and here we are two years later and still nobody can answer it.”
Because one exact cause is not known, health officials have looked into possible clues in everything from viruses to environmental toxins to genetic disorders. Some AFM cases have been connected to a severe respiratory illness caused by enterovirus D68, but Chase tested negative for it, his mother said.
AFM happens when a virus travels to a person’s spinal cord, affecting the specific area that correlates with the person’s muscles. Chase didn’t show the most severe symptoms other children have shown, such as being unable to swallow or breathe. Some children start to show signs of weakness after a prolonged cold. Other warning signs include eyelid or facial dropping and difficulty moving the eyes.
Chase’s ‘batteries’
There is no cure for AFM, but doctors can provide supportive care to patients including physical and occupational therapy. Then the parents started to hear about nerve transfers to restore movement in the paralyzed limbs. A private Facebook group of parents dealing with AFM led the family last December to Shriners Hospitals for Children-Philadelphia for a nerve transfer.
Doctors moved nerves from Chase’s rib area — which were considered redundant because they perform the same function as other nerves — to his shoulder and elbow where the nerves weren’t working. The process is really reconnecting a person’s brain to the muscles, said Dr. Dan Zlotolow, an orthopedic hand surgeon who operated on Chase. The results are more promising the younger the patient is and the sooner it happens after the initial injury, Zlotolow said.
In Philadelphia, doctors have done nerve transfers in 16 children with AFM who had paralysis in their hand, arm or shoulder, Zlotolow said. Other doctors across the country have focused on doing the procedures on AFM patients with paralysis in the legs.
In the 16 cases, the children have regained movement, but building up the strength of the muscles will take time, Zlotolow said.
The nerve transfer was an hourslong procedure that was explained to Chase by telling him that doctors were going to try to make his arm work again, Jessica Kulakowski said.
“And he said, ‘Oh, he’s going to put batteries in there,’” she remembers her son saying. She commemorated the date of the surgery by getting a tattoo of batteries on her back.
Uptick in cases
The family made the drive back to Indiana from Philadelphia with Chase and a Spider-Man doll in identical bright-red casts. As his parents took care of Chase, the toddler took care of Spider-Man, making sure they took their medicine together. Three months after surgery, Chase was able to lift his arm and move his elbow with support from his father. Since then he’s continued therapy. He’s working on picking things up, and he tends to default to his left arm now to do things like toss a ball.
When Chase was first diagnosed, it was part of a wave of cases that were reported nationally from August to October 2016. The CDC is again seeing a rise in cases similar to increases in 2016 and 2014, but officials don’t know why more people are getting AFM every other year. The spikes tend to occur from August to October.
Dr. Nancy Messonnier, of the CDC, said in a recent conference call that there have been 62 confirmed cases — the majority involve minors — so far this year in 22 states. There are an additional 127 suspected cases of AFM that officials are further investigating. The CDC began tracking the cases in 2014 when it first started to see a bubble of cases.
Messonnier said the condition is rare, and doctors have been relying on an assessment of symptoms and MRI results to make an AFM diagnosis. The CDC investigates outbreaks of illnesses across the country by gathering information from state health officials, doing laboratory testing and by consulting medical experts, Messonnier said. Health officials still can’t pinpoint whether anyone in particular is more at risk or even why that person would be more likely to get it.
“There is a lot we don’t know about AFM, and I’m frustrated that despite all our efforts we haven’t been able to identify the cause of this mystery illness,” Messonnier said.
Though the condition has been compared with polio, the CDC reports that polio is not the cause of AFM, Messonnier said.
Jessica Kulakowski said she and other parents have been using the increase in cases to get the word out about the need for more research and resources. Her son was featured in a recently created Instagram account that features children with AFM. Among the hashtags used under a photo collage of Chase was #WENEEDRESEARCH.
The increase also means parents are now coming to her with questions. She still doesn’t have many answers.
“What are we supposed to do for our kids if nobody has any idea?” she said.
