A rare polio-like disease is sickening Wisconsin patients with a frightening speed

The honeymoon ended on an ominous note. The cold Bridget Spoerri had been fighting during their stay at a bed and breakfast in Minnesota hit her husband, Adam, on the final day. Hit him hard.

For several days afterward, Bridget asked Adam if he wanted to go to the hospital. Finally, she insisted. On Aug. 5, they headed to the emergency room. They had been married all of two weeks.

At Aurora St. Luke’s Medical Center, the 30-year-old home renovator from Milwaukee was so weak he could not swallow the pills he was given — even when they were crushed. His head wobbled. He could not control his neck muscles. He braced himself with his arms just to breathe.

Adam was admitted to the intensive care unit and within a day or two, he lost muscle control in his left arm, right shoulder, right arm and neck. His face drooped.

The tube in Adam's trachea that helped him to breathe also prevented him from speaking. He had enough strength in his wrist to scratch out brief notes to Bridget on a pad. Turn my head to the left. Leg itchy. Can’t breathe. Help! Help!

“It was awful. He was scraping the cardboard because he couldn’t breathe,” recalled Bridget Spoerri, who is in her first year teaching English at Pulaski High School.

“I thought he was dying every day those first two weeks. I had no hope.”

More than three months later, Adam remains hospitalized with the disease known as acute flaccid myelitis, which has afflicted five other patients in Wisconsin. The others are all children.

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This year, there have been another 84 cases in 26 other states, according to the U.S. Centers for Disease Control and Prevention. Oddly, cases seem to peak every other year. The CDC reported 120 cases in 2014; just 22 in 2015; then 149 in 2016; and 33 in 2017. Researchers don't know the reason for this pattern.

The disease, often called AFM, attacks fewer than one person in a million and is not well understood. Although the condition resembles polio, which was eradicated from the Americas in 1994, patients test negative for the polio virus.

AFM patients experience varying degrees of paralysis in limbs and muscles and can have difficulty breathing or swallowing when those muscles are affected. They do not lose feeling, however.

The reason: The disease destroys cells that send messages from the spinal cord to the rest of the body, but does not harm the cells involved in touch and sensation. 

“AFM is not subtle,” said Rodney Willoughby, program director for antibiotic stewardship at Children’s Hospital and the Medical College of Wisconsin. Symptoms include "sudden weakness in an arm or a leg, or the face — it doesn’t hurt, but is paralyzed — trouble with saliva, or swallowing, sometimes trouble breathing normally or deeply.” The disease “is usually paired with a cold," he added.

Although there has been an increase in cases this year, Willoughby stressed, “AFM is rare, so this is nothing to panic about.”

'They can't find a cause'

Researchers at Children’s Hospital and the Medical College of Wisconsin have been collecting specimens of cerebral spinal fluid and urine for about four years hoping to learn more about AFM. 

The illness has been associated with what's called an enterovirus. This, too, is puzzling because the enterovirus is very common in the summer and fall, yet extremely few patients who catch it then go on to suffer AFM, said Susann Ahrabi-Fard, a state epidemiologist in Wisconsin who has been investigating AFM.

With more than half of the people who get AFM, she said, "there's no specific cause. It drives scientists crazy when they can't find a cause." 

The CDC has not listed a specific treatment, saying only that neurologists may recommend physical or occupational therapy to deal with leg or arm weakness. It is unclear how many people who lose muscle strength are later able to recover it.

“With the small amount of patients we’ve seen here,” Willoughby said, “at least half of the patients with AFM go home without long-lasting damage and virtually no one dies.” 

Others have seen less encouraging results.

“Most of the studies I’ve seen show somewhere between 10 percent and 20 percent make a full recovery. The vast majority have some impairment,” said Michelle E. Melicosta, one of the doctors caring for Margot Becker, a 17-month-old from Delavan who is now receiving rehabilitation treatment at Kennedy Krieger Institute in Baltimore. Kennedy Krieger is internationally known for its children's spine rehabilitation program.

Margot showed her first symptom, a high temperature, on Sept. 9. Her parents thought she might be teething, but a week later she had no control of her neck. Her chin slumped on her chest. After a month at Children's Hospital of Wisconsin, followed by rehabilitation at Kennedy Krieger Institute, she can now hold her head up and use the muscles from her elbow up to her fingers, said her father, Mark Becker.

“She’s done nicely,” said Melicosta. “She’s had steady gradual improvement in her neck muscles.” The swallowing muscles necessary to eat and drink have also recovered and Margot is regaining strength in her right shoulder and upper arm.

Two months before diagnosis

Although AFM often begins with a cold, it rapidly progresses to a far more alarming set of symptoms. 

Feeling weak, feverish and dizzy, Nicholas Adams, a 32-year-old father of three from Ogema went to Aspirus Medford Hospital on Oct. 31, 2016. Doctors thought the runner and former cross country coach might be suffering from dehydration. He received fluids, but declined an offer to undergo a spinal tap, which tests the liquid around the spinal cord. Doctors often use a spinal tap to exclude other causes.

Back at home the next day, Adams discovered he could not move his left arm. On Nov. 2, he went to Aspirus Wausau Hospital where doctors thought the cause might be meningitis. That day, he lost movement in both legs.

After a few days at the Wausau hospital, “the doctors told my wife that whatever was attacking my body would attack my respiratory system and they would not be able to support me,” Adams recalled.

He was rushed by ambulance to University Hospital in Madison on Nov. 4 and placed in an induced coma. Eventually Adams lost movement in both legs and his left arm. His weight plummeted from 186 to 139 pounds.

It would be almost two months before his illness was diagnosed as AFM and eight months before he was well enough to return to Ogema.

Two years after the first symptoms, Adams said some movement has returned to his legs, though he must walk with a crutch. He has little movement in his left arm and has not been well enough to return to work.

Fountains of blood

For Adam and Bridget Spoerri, the disease has proven especially cruel.

After the honeymoon, they spent one night in their new home in Milwaukee's Burnham Park neighborhood. Adam spent the next four weeks in the intensive care unit at Aurora St. Luke’s, Bridget by his side every day of it, getting what little sleep she could in a reclining chair beside his bed. His condition was complicated by the fact that he also has asthma and Crohn's disease.

His three months in the hospital have been marked by jolts — from peril to small moments of joy — all recorded in the meticulous notebooks Bridget has kept. She has marveled at her husband's ability to remain cheerful even in the worst moments.

On Aug. 22, Bridget took a brief break to go to lunch with her father. But the suction used to clear Adam’s secretions had irritated his mouth, causing bleeding. In  a Facebook post she described what happened that evening:

"Out of nowhere, cue fountains of blood. Blood bubbling out of his nose! Blood pouring out of his mouth. Big blobs of clotting blood the size of your pinkie sitting inside the (trachea) tube, which should obviously be clear for air to come and go.

"The blood doesn't stop. I go from 'It's just a little bleed!' to 'Oh my god, this has to be a pulmonary hemorrhage.' Cue panic. Again I am glad that we have to wear masks in the room or he would have had to watch me cry.

"Adam's amazing nursing and respiratory therapy team, jumped into gear...They are flying into action, and I am holding his hand while suctioning blood from his mouth and watching him struggle to breathe, going mental.

"Here comes the 384,204,405th reason why I love Adam Spoerri more than anyone will ever know. While blood is pouring out of all of his orifices like a horror movie, my tears are a fountain and I can feel snot clogging up my mask. He grabs my hand and starts writing letters on my palm to spell a sentence. H...o...w...

"How was your lunch?"

"I thought he was dying...I thought this was the end. And once again, he was trying to calm ME down (and) take care of me."   

A little more than a week after those harrowing moments, the newlyweds found cause for celebration. On Aug. 30, Adam went outside for the first time in weeks. He spent 10 whole minutes in the fresh air.

The following day he was transferred to the Post Acute Medical Specialty Hospital in Greenfield, a long-term care hospital for patients requiring significant recovery time. The move did not signal the end of his crisis. 

On Sept. 3, he wrote: Help! Help! Breathing treatment ASAP. Help. Help.

The struggle to breathe and the inability to even cry out trigger an almost primal fear. 

“It feels like you are about to die," Adam said, mouthing the words. "It’s like death is imminent.”

On Oct. 2, saliva and mucus interfered with his airway and Adam flailed his arms struggling to breathe while his lips and face turned blue. Later, Bridget and Adam tried to find humor in his alarming shade of blue; the incident became, in their telling, “Smurf Day.”

Gradually, Adam progressed.

On Sept. 13, doctors began weaning him off the breathing ventilator. The first day he lasted just 17 minutes and 27 seconds. The following day 29 minutes, then 32, then 40. On Sept. 30, he managed nine and a half hours off the ventilator. 

His record is now more than 34 hours. 

Taylor Finseth, a neurologist at Aurora St. Luke’s, said that Adam is his first ever AFM patient. When the doctor examined an MRI scan of Adam, he noticed an unusual pattern. An area of the spinal cord containing motor nerves should have appeared hazy and gray.

“It looked bright white,” Finseth said. “It really jumped out at you.”

He and the other doctors fought the disease by targeting enterovirus. They treated Adam with intravenous immunoglobulin, which is part of the blood’s plasma. The theory is that the immunoglobulin, which is pooled from a large portion of the population, likely contains antibodies to fight the enterovirus.

Doctors also gave Adam fluoxetine, an antidepressant, which appears to prevent the enterovirus from making copies of itself and therefore growing.

"Adam and his wife have shown such strength through this entire illness. They really amaze me," Finseth said. "The love that his wife shows for him is incredible."  

Bridget comes to visit Adam each afternoon after school lets out. Sometimes she reads to him from his favorite books: "The Hobbit" and "The Lord of The Rings."

She grows excited when he lifts his left arm on his own. "That's completely new!"

She reassures him too that he will be able to return to his passion: renovating their new American Craftsman home, the one they have spent just a day in together.

Friends offered to begin the renovations for Adam. He is accepting some help, but he is determined to do all of the actual design and rebuilding himself.

"I'll do one tile a day if I have to," he said.