The children started arriving in hospitals with a distinct set of symptoms during the pandemic.
The condition is rare – approx 150 children in New York City seems to have it, for example – and strange. Few patients test positive for the COVID-19 test, and only a few have difficulty breathing, which has been a hallmark of those fighting a particularly serious COVID-19 infection. Instead, these young patients arrive febrile, rashy, nauseated and with a host of other less visible symptoms that often resemble Kawasaki disease.
For the time being, doctors call this new condition Multisystem Inflammatory Syndrome in Children (MIS-C). But the name has changed several times. “I think this highlights the kind of rapidly changing situation we are in,” says Kevin Friedman, a pediatric cardiologist at the Boston Children’s Hospital. “Nobody really knows what to call it.”
At least 200 cases have been reported in the United States, with more cases popping up daily across the country, according to several news reports. So far, preliminary treatments have prevented death in most patients. But communication surrounding new cases, symptoms and remedies remains essential, says Gabe Owens, pediatric cardiologist at C.S. Mott of the University of Michigan. “The first thing to do to prepare potential patients with this condition to enter through our doors is really to convey awareness.”
What’s in a name?
Temporary or permanent, here’s what the name MIS-C tells us: “Multisystem” refers to the way this disease causes problems with different parts of the body simultaneously. Skin rashes, nausea or diarrhea or kidney struggles – which may appear in one of these patients – involve several groups of organs. The term “inflammatory” refers to the changes that doctors see in patients’ blood flows. A committed immune system increases the number of its agents that move through our bodies and higher levels of these proteins appear in children who face this condition.
Even COVID-19 patients can cope attacks on all types of organsand it may be easy to assume that these symptoms are just like the virus makes itself known in younger patients. But the appearance of the syndrome lags behind the peak of COVID-19 adult cases of about three weeks, says Friedman. Here because New York began seeing them in late April and early Maywhile Friedman’s hospital in Boston has seen numbers rise in the past few days. In addition, only a few of these patients test positive for an ongoing coronavirus infection, and many others test positive for antibody tests, which are swabs that theoretically detect whether or not someone has been exposed to coronavirus in the past.
These factors suggest to some doctors that the virus itself may not cause these symptoms. Instead, the condition could be the result of an immune system overreaction to the virus. But these are only possibilities: the case delay and the various results of the coronavirus tests make it difficult to establish how (or if) this condition is related to COVID-19. For now, at least it seems that the two are somehow connected. “It appears to be associated with COVID-19,” says Owens.
A disease of similarity
When doctors first saw patients with these symptoms, many thought about these cases it resembled another inflammation-related disease called Kawasaki disease. Doctors have known about this syndrome for about 50 years, although it remains fairly mysterious. No one has set the exact reason yet a few thousand children they come to hospitals in the United States every year with swollen hands and feet, the rashes and fever characteristic of the disease, Owens says. Many suspect that this adolescent syndrome appears as a reaction to an undiagnosed bacterial, viral or even fungal infection. How a child’s immune system responds to the pathogen may depend on underlying genetic factors.
It has become clear, however, that whatever is sending children to hospitals during the pandemic is distinct. For one, it affects older children, says Friedman. Kawasaki affects children aged 8 and under; children with this new set of symptoms are on average 10 years old. Some of them are young teenagers. In addition, this new syndrome soon causes serious cardiac complications. “Depending on the study you observe, 20 to 50 percent have a heart problem when they show up on some days of illness,” says Friedman. “In Kawasaki, it’s much lower than that and it usually takes longer.” Some of these new patients end up having extremely low blood pressure or a heart muscle that isn’t working properly.
All these cases exist on a spectrum. Some children have mild symptoms. Others have conditions that look just like Kawasaki. And some need immediate intensive care, says Friedman. But recognizing from the outset that this syndrome is unique compared to other possible diagnoses will help ensure that doctors take the right precautions. When a child arrives in the hospital and a series of tests indicates that they have this new set of symptoms, “it seems to be a suggestion for us to monitor these children closely,” says Owens.
Keep an eye on
Monitoring these patients pays off, as very few children have reported having this new syndrome in New York have died. Although the University of Michigan medical system has not yet seen definitive cases, Owens says his hospital is preparing for them. One way staff are doing this is to plan ahead of time exactly how they will assess potential patients and test various organ systems for problems, an essential step for a disease that may require input from experts across the hospital. “Everything we do can be optimized with the best communication.”
This theory also applies to communication with the public. Children get sick constantly with fevers and colds, says Owens. But when parents start seeing abnormal symptoms – such as persistent high fever, abdominal pain, rash and red eyes – they should go to a doctor. Time is of the essence, as the conditions of children with this syndrome can decrease fairly quickly. “We would prefer to be wrong on the side of caution,” says Owens, pointing out that the syndrome is rare.
Currently, researchers are organizing projects to analyze the DNA of patients with these symptoms to find out why they got so sick, says Friedman. The research could also illuminate the elusive trigger behind Kawasaki’s disease.
Meanwhile, those who are preparing to take care of these new patients rely in part on other health professionals who have seen cases. “We can read things in a textbook or read things about what can happen,” says Owens. “But these are suppliers who have seen these patients and we are indebted to them for sharing their experience.”