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Children with perplexing syndrome linked to covid-19 may be experiencing deadly ‘cytokine storm’

New York physicians propose early theory, detailing four case studies that were remarkably similar.

May 28, 2020 at 9:01 p.m. EDT
A New York City Police Department officer watches as children play in the Elmhurst section of Queens. (REUTERS/Brendan McDermid)

The four children showed up at the Mount Sinai Kravis Children’s Hospital in late April and early May, almost exactly one month after the peak of New York City’s coronavirus surge. All had fevers, rashes and strange blood readings that did not look like any illness doctors had seen before. And yet, the cases looked remarkably similar to one another.

A study about the children, ages 13, 12, 10, and 5, published in the American Journal of Emergency Medicine, provides the first detailed look at the rapid progression of a mysterious syndrome linked to covid-19 that has alarmed public health officials.

The Centers for Disease Control and Prevention earlier this month issued an alert warning doctors to be on the lookout for what they are calling multisystem inflammatory syndrome in children, or MIS-C, which is now believed to have impacted approximately 300 U.S. children. It appears to have some characteristics in common with Kawasaki disease, a rare illness that typically impacts children under the age of 5 and whose cause is unknown.

There have been at least five children in the United States who have died from the coronavirus-related condition: three in New York State, a 15-year-old girl in Maryland, and a child in Louisiana.

At Mount Sinai, the children, who came through the emergency department, all appeared well initially despite having multi-day fevers. Each complained of different symptoms, but blood tests, imaging and heart monitoring showed they all appeared to be having an exaggerated inflammatory reaction in what doctors suspect is post-viral complication of covid-19.

Christopher Strother, the director of emergency medicine at Mount Sinai, described it as the pediatric version of the “cytokine storm” occurring in some adults with severe illness from the novel coronavirus.

“When adults get covid they might get that terrible lung disease from the inflammatory response,” he said. “This seems a version of how that looks in kids.”

All four children were treated with a drug called tocilizumab to inhibit the inflammatory reaction. The paper’s authors wrote that the “mechanism of injury during cytokine storm is poorly understood, but an exaggerated initial response that persists over time is associated with poor outcomes.”

The first child to arrive at Mount Sinai on Apr. 25 was Malcolm Edgar, a previously healthy 13-year-old from Brooklyn. Edgar, an athlete who is the third baseman and pitcher on a competitive baseball team, had had six days of fever that shot up to 104 degrees. When he woke up on the 25th, he had bloodshot eyes and a rash on the trunk of his body. His tongue had what they described as an odd “strawberry texture.”

Juliet Daly is among the first known children in the United States to suffer from a rare inflammatory condition doctors believe is linked to the coronavirus. (Video: The Washington Post)

At the hospital, doctors ran a battery of tests and his initial vitals seemed fine, except for a slightly elevated heart rate. So they gave him fluids and asked him to wait for the results. Strother recalls that the report he got from the doctor overseeing Malcolm’s care was that “he was walking around the ER eating cheese,” and that “he actually looked quite well.”

But as the night wore on and the nurses kept checking his blood pressure, his readings plummeted. Malcolm himself remembers he was very tired, but not in any pain and that he had been begging to go home.

“I was kind of confused about what was going on,” he said in an interview Thursday. “Everything seemed fine in my checkup, and then they said my blood pressure was low and that’s when everything got crazy.”

Over the next few days, his situation became dire. Imaging showed that he appeared to have pneumonia in both lower lungs, and as he progressed to respiratory failure, he was put on a ventilator.

His mother Ayisha Edgar was at the hospital with him. “I just broke down,” she recalled. “It was very hard for me.”

Blood tests showed markers of inflammation and clotting were off-the-charts elevated. Malcolm’s heart was not working properly, as his arteries showed signs of being enlarged. Meanwhile, three tests doctors gave him for active covid-19 infection came back negative, but an antibody test was positive, indicating he had a prior infection.

His father Sean Edgar, recalled that he and his wife had been sick with mild flu-like symptoms the month before. They lost their sense of taste and smell, but otherwise didn’t think much of their illness. Now they wondered if their son had been infected then, too, although he had showed no symptoms. After Malcolm’s hospital stay, they got tested for covid-19 antibodies and it turned out both had them.

The second patient was a 10-year-old boy who had had a positive covid-19 test 17 days prior. He had come in after five days of fever, diarrhea, cough and a rash all over his body, including his neck, chest, abdomen, back, palms and soles. On his back, there were “dusky areas,” according to the paper.

“[H]e was in no acute distress but complained of general discomfort,” the study’s authors wrote. His fast heart rate and low blood pressure were worrisome, but his breathing was normal. In intensive care, he received blood pressure medication, with an immunoglobulin product that is normally given to patients with Kawasaki disease, and the same immunosuppressant given to the other patients in the study.

The third patient, a 5-year-old boy, had five days of fever, one day of stomach pains and vomiting. He, too, had a rapid heart rate. He was stable for 24 hours until an ultrasound showed he had inflammation in his testicles and thickening gallbladder walls. His blood pressure fell to an emergency-level low of 61/37. In intensive care, the boy also received medication typically used for Kawasaki patients and an anti-inflammatory drug.

The last patient’s case was the mildest. The 12-year-old girl had initially been suspected of having appendicitis. She didn’t have a rash, but after running tests, doctors discovered that her heart was beating super fast — a problem because it means the heart can have trouble filling with blood because it is contracting too rapidly — and her temperature climbed to almost 103 degrees.

All four children have recovered, Strother said, and are back home now.

Malcolm lost 20 pounds in during his 11-day stay the hospital, but did so well after he was off the ventilator that he walked out of the hospital to the cheers of the staff. He’s returned to his school’s Zoom classes and is now strong enough to play some basketball in his backyard and go walk around the local high school track with his dad. He expects to be able to return to playing sports by July.

Sean Edgar says that he would advise other parents who see odd symptoms in their children not to wait: “And if you have to take your child to a hospital, go to a children’s hospital. That was the best choice we made.”

Strother urged doctors who see children with persistent fever to err on the side of caution when monitoring them and ordering tests.

“The trouble is the children look pretty good even when their blood pressure starts to go down,” he said. “You have to be vigilant.”

Read more:

The girl who died twice: Juliet Daly was a healthy 12-year-old in Covington, La., until the coronavirus infected her heart

Young adults in their 20s are also affected by Kawasaki-like disease linked to coronavirus, doctors say

‘Frostbite’ toes and other peculiar rashes may be signs of hidden coronavirus infection, especially in the young