How does a rare inflammatory condition afflicting children relate to coronavirus?

A rare inflammatory condition in children that could be connected to the novel coronavirus reached North Texas this month, putting many on edge about what the new syndrome means for the outbreak.

The country’s top public health officials maintained for months that children are expected to be spared from serious coronavirus complications. But the condition, called multisystem inflammatory syndrome in children, or MIS-C, started appearing in late April primarily in children in New York, the country’s epicenter of COVID-19. Now, public health officials are warning doctors across the country to be on the lookout for the condition.

Health experts say it’s similar to Kawasaki disease, a rare condition with unknown cause that affects children younger than 5 and causes inflammation in the blood vessels.

Here’s what you need to know about MIS-C, from health experts and the North Texas mother whose son received a diagnosis of a similar condition.

What are the symptoms of multisystem inflammatory syndrome?

MIS-C can present with varying symptoms, but it typically includes a persistent fever for four or five days, stomach pains, rashes, inflammation and, in severe cases, poor organ functioning and low blood pressure, health experts say.

Doctors have referred to MIS-C as a “Kawasaki-like disease” because the symptoms are so similar. The main difference is that Kawasaki disease affects children only up to 5 years old, while MIS-C can affect adolescents.

In a health alert notification sent to health care providers across the country this month, the Centers for Disease Control and Prevention encouraged doctors to report to local and state health departments possible cases of MIS-C — including cases of kids who meet all or some of the criteria for Kawasaki disease.

Possible cases include patients up to age 21 who are experiencing a fever, inflammation and a severe illness affecting at least two organs requiring hospitalization. The patients also must have tested positive for COVID-19 or COVID-19 antibodies or have had exposure to the virus within about a month of symptoms.

Symptoms can include confusion, red or pink eyes, coughing, diarrhea, headaches, swollen lymph nodes or neck swelling, respiratory symptoms, a sore throat, red or swollen hands and feet, fainting, vomiting, swollen lips or tongue and joint pain, according to the Royal College of Paediatrics and Child Health in the United Kingdom, the New York City Department of Health and Boston Children’s Hospital.

Britney Boler said her 3-year-old son, Titus Foots, had many of the symptoms that are consistent with Kawasaki disease and MIS-C before he received a diagnosis of Kawasaki disease. She said she visited Children’s Medical Center Plano three times before doctors diagnosed his condition.

Titus first got sick at the end of April, and Boler thought it was a cold until his fever spiked to 101. She took him to Children’s, where doctors ruled out strep throat, told Boler he likely had a viral infection and sent them both home.

She took him back the next day after Titus’ fever spiked again and he began complaining about stomach pains. Doctors ran more tests and said he had a urinary tract infection and gave him antibiotics. Just as they were getting ready to leave, his fever spiked again — this time over 103 degrees.

Doctors gave Titus fever-reducing medicine until his fever dropped, and Boler took her son home again.

“I knew it wasn’t right the first two times,” she said. “But you know, I’m not a doctor so [I figured] if I need to come back, I can come back.”

On her third visit, four days after she first brought her son in, doctors made a diagnosis of Kawasaki disease.

“By Friday [his temperature] was at 104 consistently,” she said, adding that he had vomiting and diarrhea the day before. “He had red eyes, his lips were swollen and blistered. His tongue was like the typical strawberry tongue.”

Dr. Jeffrey Kahn, chief of infectious disease at Children’s Health and a professor at UT Southwestern Medical Center, said Kawasaki disease can be difficult to diagnose.

While he couldn’t comment specifically on Titus’ case, he said children have to meet a certain set of criteria — none of which are unique to Kawasaki disease — before the disease is diagnosed. He said the first criteria is having a fever for more than five days.

“You can imagine that if a child is going to go on to have Kawasaki and they’re having fevers on day one or day two, that alone would not fulfill the criteria,” he said, adding that the variety of other symptoms of Kawasaki can progress or appear during those five days.

Further complicating the diagnosis is that there is no diagnostic test for the virus — a diagnosis of Kawasaki is based on clinical findings alone, Kahn said.

Children’s Health said in a statement that it was working to investigate possible cases of the virus, and Kahn said doctors at the hospital would be using the CDC’s criteria.

Is it treatable?

Health experts say that though MIS-C can cause serious complications in some children, it usually can be treated with a few days of hospital care, sometimes in an intensive care unit. The condition isn’t contagious, so children can’t spread it to family members or other children.

Children with the condition are usually treated with intravenous immunoglobulin, which gives the body an antibody boost, aspirin and steroids, health experts say.

“The good news is that, like Kawasaki disease, almost all the kids are treatable,’’ Dr. Charles Schleien, chairman of pediatrics at Northwell Health in New York, told USA Today. “It is highly likely that, with treatment, they’re going to be fine. It’s not like the fear of COVID-19 where we know there are no treatments and it’s a matter of luck.”

Boler said her son was admitted to the hospital’s intensive care unit within five minutes of her third visit. Aside from his other symptoms, she said his blood pressure was low enough to require hospitalization.

She said on her third visit to the emergency room, Titus was tested twice for COVID-19. Both tests came back negative.

His treatment included blood pressure medication, antibiotics and the administration of fluids, Boler said. He was in an intensive care unit for about three days.

“He had a lot of lines going through him at that time,” she said.

How common is it?

The condition was first recognized by doctors in the United Kingdom on April 26, according to the CDC. Since then, MIS-C has appeared in several European countries and in New York, which is investigating more than 100 possible cases and has had three deaths from the syndrome.

The condition reached Texas after Cook Children’s Medical Center in Fort Worth announced last week that since May 9 it has treated four children for the syndrome who ranged in age from 6 to 14.

The hospital said three children tested negative for COVID-19 and one tested positive, but that all four had had exposure to the disease before coming to the hospital. As of last week, three kids had gone home and one was being treated in a pediatric intensive care unit.

“We believe all of these cases are related to COVID-19,” Dr. Nicholas Rister, an infectious diseases physician at the hospital, said in a written statement. “The three negative results are evidence of how far the infection had progressed, resulting in the inflammatory syndrome.”

Some health experts have said that places that have been particularly hard hit by the virus should expect to see increased cases of the syndrome.

But other health experts say that overall the condition is rare.

“We don’t want people to panic,” Dr. Purvi Parikh, a pediatric allergist and immunologist at NYU Langone Health, told MarketWatch. “The majority of kids don’t get it. And even if they do, they do very, very well [with treatment.] So don’t be afraid of the ER.”

How does it relate to COVID-19?

Many of the children who have had MIS-C have tested positive for COVID-19. Because inflammatory conditions are usually rare in children — Kawasaki disease, for example, only presents in 1 in 10,000 children under 5 in the U.S. each year — the presence of these clusters indicates a reason to believe there is a link between the virus and the condition, health experts say.

New York Gov. Andrew Cuomo said that of the more than 100 cases the state is looking into, 60% tested positive for COVID-19, 40% tested positive for COVID-19 antibodies, and 14% tested positive for both, MarketWatch reported.

Health experts aren’t completely sure yet why children who have been exposed to COVID-19 are getting MIS-C, but there are a few theories.

During serious cases of the novel coronavirus in adults, severe complications are a result of the immune system’s overactive response to a new virus, and not necessarily the virus itself.

Some health experts say the same thing may be happening in children, but the response may be delayed.

“Often long after viruses are gone, they can cause inflammation or immune changes in your body,” Parikh told MarketWatch. “This could be a delayed consequence of the virus: were kids exposed or infected earlier, and now we’re seeing it? There are a lot of different possibilities.”

Boler said doctors and nurses gave her varying information about whether Titus’ condition was related to COVID-19, and that the reality is “there are so many different case scenarios that could have happened” because of how little is known about the new syndrome.

“The first ICU doctor said, ‘We can sit here and go back and forth and wonder if it was related to COVID or not, but let’s just be happy … and be thankful that it’s not more than what it could have been,’” she said. “But another doctor and other nurses that I’ve talked to have said it possibly could be, he could have possibly had [COVID-19] … two to three weeks prior and now the Kawasaki symptoms were triggered.”

She said doctors at Children’s are planning to do more tests on Titus to see whether his condition was related to COVID-19, and Boler said she plans to push for antibody testing for her son.

Kahn said Kawasaki disease presents in a few children each year, and that it’s possible Titus’ case could have been one of the cases that would normally occur outside the pandemic.

“Any children’s hospital will encounter these children throughout the year,” he said. “The fact that now we’re in the midst of an epidemic with this other disease that seems to be emerging makes it a little bit more challenging for health care professionals, but I can’t imagine a situation where the baseline Kawasaki that we’ve seen for decades is going to change all that much.”

When should parents seek medical treatment for their kids?

Health experts recommend taking children to the emergency room if they are experiencing any symptoms of MIS-C. But because there are so many symptoms, one of the first things to look for is a fever that is persistent for four or five days.

Boler said that if she could give one piece of advice to parents after her experience, it would be to take their children to get help when they feel they’re sick, “no matter what.”

“If it’s one symptom or two symptoms [of Kawasaki], take them in,” she said. “At the end of the day we’re their best advocates and we know our kids better than anyone else. If your son or daughter is so used to playing 24/7 and the next thing you know they’re sleeping 24/7, just take them in. When in doubt, just take them in.”