Children with polio-like illness continue to struggle

It started with a headache.

Bailey Sheehan, 7, told her mother she had pain in her head, neck and back. The aches turned into flu-like symptoms, which became so severe Bailey had trouble breathing. Steroid treatments from her pediatrician seemed to help.

But the infection weakened her.

One night, a few days after her respiratory illness appeared, Bailey couldn't summon the strength to pick up her 4-month-old sister. The next morning, Bailey couldn't lift her neck or arms.

When she tried to stand up, she collapsed. "She said, 'Mommy, my legs aren't working,'" says her mother, Mikell, who rushed her to the emergency room.

An hour and a half later, as nurses examined her, Bailey said, "I can't feel my leg."

Bailey's right leg was numb from above her knee to below her ankle. Her right arm was numb from the shoulder to her elbow.

"Doctors just kept saying, 'We don't know what this is,' " Sheehan says.

Bailey's symptoms would have been familiar to many doctors in the 1950s, when the USA was gripped by annual polio outbreaks.

These days, thanks to the polio vaccine, such a fast-moving, devastating paralysis is rare. A neurologist might see only one or two cases in her entire career, says Jim Sejvar, a neuroepidemiologist at the Centers for Disease Control and Prevention.

Yet last fall, some large hospitals saw two or three children with polio-like symptoms per week, Sejvar says.

Bailey is one of at least 112 children in 34 states who have developed sudden, severe muscle weakness, officially known as acute flaccid myelitis, since September, according to the CDC. Like polio, the paralysis occurred largely on one side of the body.

Only one of the children has completely recovered, according to the CDC. Although two-thirds have improved somewhat, many continue to struggle. Most children afflicted by polio were left with lasting disability, Sejvar says.

"It's my worst nightmare," says Sheehan from Welches, Ore. "The pain you see in your child's face – they just want you to fix it, and you don't know how. They want the doctor to fix it, and the poor doctor doesn't know how."

Bailey has lost more sensation since October, when the paralysis developed. A larger portion of her thigh is numb. She's lost most of the vision in her left eye.

The cause of Bailey's disability – and how to treat it and prevent it – remain unknown.

Several viruses can cause polio-like symptoms, including West Nile, adenoviruses, coxsackie, herpes and enteroviruses, a large family that includes polio, Sejvar says.

One enterovirus, EV-D68, is a leading suspect, Sejvar says. It caused a huge outbreak of severe respiratory illness last fall, beginning in August.

That surprised doctors, because enteroviruses aren't usually so severe. They usually cause mild symptoms akin to the common cold. The viruses typically peak in late summer and early fall, Sejvar says. CDC scientists estimate there were millions of cases last year.

Last fall, EV-D68 hospitalized 1,153 Americans. Most were children. Many had asthma. Fourteen people died, although it's not clear whether EV-D68 was to blame, the CDC says.

Scientists have found some connections between the virus and the paralysis cases. First, there's the timing. The fact that the two outbreaks happened so close in time suggests that, even if EV-D68 didn't directly cause the paralysis, it might have played a role, Sejvar says.

Like Bailey, many children with polio-like symptoms had a fever and respiratory illness about a week before the paralysis developed.

Doctors swabbed Bailey's nose and throat as part of a general test for enteroviruses and rhinoviruses, which cause the common cold. The test came back positive. So have tests in some other children. Doctors didn't perform a more sensitive test to look specifically for EV-D68.

Infectious disease specialist Mary Anne Jackson says any link between the paralysis and EV-D68 could be coincidental. Many children develop colds and flu-like symptoms in the fall, says Jackson, director of infectious diseases at Children's Mercy Hospital and Clinics in Kansas City, Mo., one of the first hospitals to report a surge in severe respiratory infections.

All of the affected children have one thing in common: an inflammation of nerve cells in their spinal cords, seen in MRI scans, Sejvar says.

Tests have not found EV-D68 in spinal fluid. Finding the virus in spinal fluid would be a strong sign that the virus contributed to the paralysis, says Samuel Dominguez, a pediatric infectious disease specialist at Children's Hospital Colorado in Aurora. His hospital was the first to report the polio-like conditions to the CDC in September.

It's possible that doctors looked for the virus too late, after it had disappeared, Dominguez says. According to the CDC, there have been only two published reports in which doctors found EV-D68 in the spinal fluid of children with neurologic illness.

Pinning down the causes of sporadic paralysis cases can be difficult.

Doctors don't usually find the polio virus in spinal fluid of affected children, either, although the virus can be found in the stool, says Jackson, whose hospital was the first to report a sharp spike in respiratory illness last summer. Doctors have not found EV-D68 in stool.

Scientists can say one thing with certainty, Sejvar says: What happened last year was not normal.

"Anecdotally, when you ask pediatricians and neurologists about their perceptions, unanimously they will say, 'We have never seen anything of this magnitude,' " he says.

A SENSE OF HUMOR HELPS

Billy Sticklen celebrated Christmas by getting out of his wheelchair.

Billy used a wheelchair for three months after he mysteriously became paralyzed. His illness began in September with a headache, dizziness and flu-like symptoms. Over the next few days, he developed a fever and excruciating neck pain.

One morning, Billy woke up and couldn't raise his arms. The staff at the hospital near his home in Joplin, Mo., suggested sending him by ambulance to Children's Mercy Hospital in Kansas City, which had treated two patients with similar symptoms. That hospital sent him straight to intensive care. Within a few days, Billy was unable to move his legs or raise his head.

"It got to the point where, if I looked down, someone would have to push my head back up for me," says Billy, 13.

Tests found no sign of EV-D68.

Billy spent two months in the hospital, where he underwent intensive physical and occupational therapy, including sessions in the pool. His family rented an apartment in Kansas City for two months, so he could go to therapy full-time at a specialized rehab center.

Today, Billy can walk with a cane. His left arm remains weak, but his right arm is strong enough to do bicep curls with 2½-pound weights.

He returned home Feb. 1 and still receives intensive full-time therapy. He hopes to return to school within a couple of months.

His sense of humor has helped him cope. Expressing frustration that the plight of kids with polio-like paralysis hasn't gotten enough media attention, Billy quips, "Ebola totally stole our thunder."

MORE QUESTIONS THAN ANSWERS

The stories of children such as Billy and Bailey have left scientists with more questions than answers.

Why did the virus, which has been around since the 1960s, suddenly cause so much suffering? Has it mutated to become more infectious or dangerous? Why did some children develop mild illness while others were hospitalized? Will the virus come back next summer?

To help answer some of those questions, Sheehan has enrolled Bailey, 8, and her brother in a study at Johns Hopkins University in Baltimore.

Researchers Aaron Milstone and Priya Duggal compare saliva samples of children from families in which more than one child had respiratory illness but only one was paralyzed. The researchers hope to find genetic clues that could help them predict which children are more vulnerable than others.

Studies don't indicate EV-D68 has mutated or changed in other ways that would have caused it to suddenly cause so much illness, Jackson says.

Three strains of the virus circulated in the USA last year, and none of them was new, according to the CDC. The predominant strain had been in the USA since 2012 and is related to strains detected in previous years in Europe and Asia.

One reason doctors don't know much about EV-D68 is that they rarely test for it, Jackson says. Unlike measles, enteroviruses aren't among the viruses doctors routinely report to the CDC.

CDC officials are likely to add the polio-like syndrome to that list, Sejvar says. That would encourage doctors to report any new cases of unexplained paralysis to the CDC.

Jackson is working with the CDC to learn whether EV-D68 made a sudden splash in the USA last year or if it has actually been common for years. She's testing 500 blood samples from the past two years to see whether they contain antibodies to EV-D68, which would indicate the person who gave the blood had been exposed to the virus.

"If we do find antibodies, that would mean that this has been out there under the radar," Jackson says.

Jackson is working with the CDC to examine old MRI scans to see whether they show the sort of inflammation visible in children such as Bailey.

By learning more about EV-D68, Jackson says, doctors hope to develop better ways to diagnose, treat and prevent the illness.

OPERATING WITHOUT A ROAD MAP

New treatments and ways to prevent the illness can't come too soon, says Sheehan, who worries the country will see outbreaks of EV-D68 and polio-like symptoms in children again next year.

"I hope these research efforts will produce results quickly so that the illness isn't so devastating," says Billy's mother, Dawn. "So they get them back into the life they were living before they got sick."

Mikell Sheehan has written weekly letters to President Obama and every other politician she can think of, pleading for help in solving the mystery of her daughter's illness.

Even if it's too late to help Bailey, Sheehan says, she hopes scientists can come up with ways to protect other children.

Doctors treating kids such as Billy and Bailey have been operating without a road map, with no textbooks or medical studies to guide them.

Both children have received the same sorts of physical therapies used in children with polio.

Bailey attends school half-time, so she can go to physical therapy six afternoons a week, Sheehan says. Each session lasts one to two hours, in addition to special sessions for swimming and other exercise.

Thanks to therapy, Bailey can walk with the help of a walker and a boot-like brace for her leg. Last week, she was able to climb the stairs without support for the first time, Sheehan says.

Bailey's most recent goal was to relearn to skip. Although she had a hard time skipping in physical therapy, her friends helped bring about a breakthrough, her mother says.

"Ten minutes with her girlfriends, and she was skipping," Sheehan says.

Bailey's physical therapist, Melissa Murray, says she will continue to make progress.

"'The bottom line is she's a kid, and we are just trying to get her back to doing kid things, tapping into the fun of being a kid, tapping into the muscle memory of what she was doing before," Murray says. "Every week, she's doing something she didn't do the week before."

At first, Bailey was unable to tolerate putting any weight on her right leg. Even a brief attempt to stand sent pain shooting up her spine and neck. "She would have spasms and curl up into a ball," says Sheehan, who consulted Children's Hospital Colorado for advice. Doctors suggested giving her a drug called gabapentin, used to treat nerve pain.

"She still has bad days where she just cries," Sheehan says.

Gabapentin has reduced Bailey's pain, although even high doses haven't eliminated it. Those high doses have caused a battery of side effects, including irritability and fatigue so overpowering that Bailey has fallen asleep at school, Sheehan says.

Bailey manages to stay in good spirits much of the time, Sheehan says. She enjoys getting milkshakes after her doctor's appointments. "Well," she says, "at least my taste buds aren't paralyzed."

Bailey, whose story was told on local TV, has received letters of support from around the world, which the family hangs on a "Happy Wall." Her 5-year-old brother loves to play the role of mailman and give his sister new cards, Sheehan says.

Billy and Bailey have gotten support from polio survivors. One, a family friend of Billy's who is nearly 70 years old, visited him in the hospital.

As bad as it's been, Sheehan says, "we're learning that Bailey is one of the lucky ones."

Some children with the polio-like condition have been paralyzed from the neck down. Some have suffered damage to the nerves that control their breathing, forcing them to use respirators, Dominguez says. In the days of polio, those children were put in iron lungs.

Billy's mom says she feels fortunate that her son has done so well, although progress has felt painfully slow.

"I expect to return to my prior state," Billy says. "No one has told me that I won't."