Elizabeth's Story: Diagnosed With Mysterious Polio-Like Illness
A patient's battle with Acute Flaccid Myelitis
"Dr. Diane, something is very wrong with Elizabeth.”
I watched in horror as her dad carried her down the hallway and into one of the exam rooms. She looked stiff. She looked like she was in pain. Her face was grimaced. What in the world? Why was this very healthy 4-year-old being carried in? My stomach sank.
Something was very wrong with Elizabeth.
This was in June 2018. I’d seen her a few days before, during her little brother’s checkup. I couldn’t help but notice how quiet she was being. She sat still, in a corner. This was a child that usually loved to bop all over the room.
“Elizabeth, kiddo, you feeling okay?”
“She’s not feeling well. She’s had a little fever and seems under the weather,” said her mom Heather.
I checked her out, looking in her mouth and ears, and listened to her heart and lungs. She looked fine.
“I think this is something viral. Why don’t you let her rest this weekend and let me know how she’s doing next week?”
Little did I know that “next week” meant three days later, and that her dad would be carrying her in like a limp, broken doll into our office.
Nurse practitioner Tara Haraldson and I ran right behind them into the exam room.
She screamed in pain whenever her head was turned. Her neck was stiff, rigid. She hadn’t been eating much and wouldn’t drink. She was having a hard time talking. I was really worried she had meningitis. She was in so much pain she had to lay on the floor of her parent’s car to get to the office.
“Things got much worse yesterday. We took her to the ER earlier this weekend but they thought maybe it was just pneumonia so they tried antibiotics. Her neck pain started that night and has only gotten worse. She can’t turn her head and she’s hurting so much we can barely move her.”
I sent her to Cook Children’s Emergency Room via ambulance. I called the ER doctor to let them know she was coming. That physician also thought it was likely meningitis. We agreed she needed a brain MRI and a spinal tap right away.
Barely one hour later, the ER doctor called me – “It’s acute flaccid myelitis.”
“WHAT?!” I nearly spit out my drink.
This was almost a myth. A pediatric rarity. "The rare polio-like illness that's affecting kids across the country". A disease that affected less than 1 child in a million. I’d only read about this in medical journals. What in the world was it doing in my office – heck, in my city…. in my state?
A 'Polio-type Illness'
The words rang in my head for hours after I got the phone call. I struggled to remember what I had learned about this rare disease in years past. I had certainly never seen it in person – nor had any doctor I knew.
Elizabeth’s AFM was found via her brain MRI. They wheeled her into the ER and took her straight to the MRI machine. The radiologist watched as the images loaded on the screen. She had color changes on it, spanning from the bottom of her brain (the brainstem) all the way down her spinal cord. The radiologist knew what it was in a matter of minutes, and called the ER doctor immediately.
As I sat in my office staring into space that afternoon, I’d remembered that there was an outbreak of this disease in Colorado in 2014. The headlines were splashed all over the front of my medical journals as a warning. Watch out for this, pediatricians! Keep your eyes peeled for this awful polio-like illness. Thankfully, the cases had slowly vanished and I hadn’t heard about it for awhile. Until now.
Why do we keep calling it a “polio-type illness”? Well… because this disease looks exactly like polio. Except, it’s not polio. It’s a brother of polio – not a cousin, or a distant relative – a brother. And it is a terrifying reminder of what polio used to be (and hopefully will never be again).
Polio, caused by the poliovirus, is in the enterovirus family. There are hundreds of enteroviruses. Stay with me.
Enteroviruses are everywhere. Literally everywhere. In my office daily. In your office. In schools. On the grocery store cart. On that door handle. Enteroviruses make millions of people sick yearly with common colds, tummy bug-type symptoms, hand foot and mouth disease, and bad coughs.
Deep breath. It’s OK. Viruses. Are. Everywhere. And it’s fine if your kiddo has symptoms of a tummy bug or a cold right now – I don’t want to cause a panic, and your child will very likely not get AFM. I just want you to know the facts and be educated on this. Elizabeth’s disease is VERY rare and we’re not even 100 percent sure if it’s related. But we’re suspicious.
We think (well…smarter people than myself at the CDC think) that this disease is caused by one of the enteroviruses in particular, known as Enterovirus D68. Could it be caused by things in our environment? Maybe. Could it be caused by our genetics? Sure. Why did Elizabeth’s body allow this common virus to attack her nervous system? Was it something in her genes? Has she been exposed to something environmentally that the majority of the rest of us haven’t? We don’t know.
But there were some big epidemiology studies that linked a big uptick in D68 at the same time an outbreak of AFM happened in Colorado in 2014. And when kids were quickly tested soon after they had limb weakness, it was found in many of them:
“In the 2014 US outbreak the number of EV-D68 cases increased at the same time as polio-like illness in children. In addition, the outbreak of EV-D68 subsided around the same time as the cases of AFM, with cases of both decreasing in October 2014. As mentioned previously, Sejvar et al. detected EV-D68 in eight of 17 respiratory specimens from patients with AFM collected no more than 7 days from onset of limb weakness.”
Every kid with this disease has not tested positive for it (Elizabeth didn’t). We look for evidence of viruses in the blood, in the cerebrospinal fluid, and in the blood. We make our best guesses. Viruses are very small, and sneaky.
If AFM is not caused by a virus, but something else…it hasn’t made itself known yet.
I do think it’s caused by a virus, because of what I’ve read about the outbreaks. 80 percent of kids with this disease in the U.S. have had a respiratory illness, or a fever a few days before starting to show the scary neurological symptoms.
The heroine of my story
Back to Elizabeth, the heroine of my story. While in the ER, they noticed she couldn't stand without severe dizziness. And that her right arm wasn't working very well. Her face was drooping on one side.
Her mother later told me that in the ER, the doctor said that she may never walk again. She may need a breathing machine for the rest of her life. She may not live. They were absolutely terrified.
Her parents ran frantic Google searches, and called families across the state. I dug up every study I could find on the 2014 outbreak and read until my eyes crossed. What could we do to help her?
She was wheeled up to the Cook Children’s Pediatric Intensive Care Unit for extremely close observation. They were worried her breathing muscles might become weak, or her brain function might continue to decline.
I don’t think I slept more than 20 minutes that night. I know Elizabeth’s parents didn’t.
We were waiting to see what would happen to Elizabeth’s body. And there was nothing the ICU doctors, the infectious disease doctors, or the neurologists could do to stop this. Steroids, antibiotics, antiviral medications, other forms of therapy – all were deemed useless over the past few years. We were helpless.
So we watched.
(This is the end of part 1. Read the conclusion to the story tomorrow.)
Get to know the author of this post: Diane Arnaout, M.D.
Dr. Diane Arnaout joined the Cook Children's Willow Park practice in 2011. You can stay connected with Dr. Arnaout on her Instagram account and the Willow Park practice on Facebook. Dr. Arnaout was born and raised in the Dallas-Fort Worth area. She attended college at Texas A&M University and medical school at the UT Health Science Center in San Antonio. She did her pediatric internship and residency at Children's Memorial Hermann Hospital and M.D. Anderson at the Texas Medical Center in Houston, TX where she served as a leader on the medical education committees. She is a board-certified pediatrician. Click to learn more.