Coronavirus: What parents need to know about rare inflammatory disease that affects children, according to experts

Paediatric multisystem inflammatory syndrome (PMIS) reported in at least 16 states across the US

Health officials across the globe have turned their focus on a rare inflammatory syndrome that has impacted children in recent months, with them looking for potential links to Covid-19.

Paediatric multisystem inflammatory syndrome (PMIS) has been reported in at least 25 states across the US and in more than a dozen countries as of 21 May.

Concerns over the syndrome, which exhibits symptoms similar to that of the rare Kawasaki disease, heightened in recent weeks after New York reported at least 100 cases in children, three of whom died, governor Andrew Cuomo said.

European countries – such as the UK, Italy and Spain – also reported cases in recent weeks after the coronavirus reached its peak in their areas.

Doctors at the Papa Giovanni XXIII hospital in Italy released the first scientific study into PMIS that could link it to Covid-19, which was published in The Lancet medical journal on Wednesday. They compared 10 cases of the illness in children initially diagnosed with Kawasaki disease with cases pre-coronavirus. Researchers found a 30-fold increase in cases of a Kawasaki-like disease between February and April.

Of those 10 cases in Italy, eight patients tested positive for Covid-19 antibodies, indicating they were previously infected with the virus. The US has also found some of its presumed PMIS cases testing positive for antibodies.

The World Health Organisation (WHO) and Centres for Disease Control and Prevention (CDC) have now raised alert about the rare inflammatory syndrome developing in children during the coronavirus pandemic, leading parents to wonder what they should be worried about as information surrounding the syndrome develops.

How PMIS differs from Kawasaki disease

Questions evolved in recent weeks about how health officials know the new inflammatory syndrome is not just a variation of Kawasaki disease, which tends to develop in children between the ages of six months to five years old.

Dr Juan Salazar, the physician in chief and infectious disease and immunology specialist at Connecticut Children’s hospital, told The Independent that while Kawasaki disease was not caused by a specific bacteria or virus that health officials are aware of, PMIS developed during the coronavirus.

“Now we have Covid and we have kids that are coming in that look like Kawasaki disease,” he said. “You could still probably call it Kawasaki disease, but it is associated with coronavirus.”

Both are believed to develop post-infection from a virus or bacteria, which explains why PMIS cases lag behind the initial coronavirus infections reported across the US. But while Kawasaki disease is rarely found in children over the age of five, PMIS has yet to distinguish if any age group is at a greater risk of developing symptoms.

States have reported children as old as 18 years old presenting symptoms of PMIS, which would be “extremely rare” for Kawasaki disease, according to Dr Salazar.

His hospital has two assumed cases of PMIS based on the evolving definition of the syndrome by the CDC. Another presumed case could be confirmed from the state in the coming days.

Demographics among the children diagnosed with a Kawasaki-like disease was a key reason that encouraged healthcare professionals to look at the potential for a new inflammatory syndrome, Dr. Nipunie Rajapakse, a pediatrics infectious disease expert with the Mayo Clinic, told The Independent.

Dr Rajapakse cited the recent scientific study out of Italy, which showed the difference in age groups.

“In kids with Kawasaki disease, the average age was around three years of age, whereas kids with this possible Covid-associated inflammatory syndrome had an average age closer to seven years at least,” she said. “This suggests that this is potentially something that is unique to Covid-19, because it’s not following the usual distribution that we see with Kawasaki disease.”

Another difference between the two inflammatory syndromes was how they presented themselves in patients. A higher proportion of those with PMIS, thus far, have shown “low blood pressure or decreased heart function” compared to Kawasaki disease, Dr Rajapakse said.

Children with PMIS also developed a higher likelihood of severe heart or kidney disease, according to Dr Salazar.

Signs and symptoms of PMIS

An important indicator parents should look out for is a high fever lasting over a few days, a similar symptom to that of Kawasaki disease.

Dr James Schneider, the chief of the paediatric intensive care unit at Cohen Children’s Medical Centre, found that 100 per cent of the hospital’s patients being treated had a high fever.

“100 per cent of them have had persistent fever for three or four days,” he told The Independent. “Gastrointestinal symptoms of abdominal pain, vomiting, or diarrhoea are also common, followed by rash.”

The rash could take on almost any form, Dr Schneider added. He recommended for parents evaluating their children to seek consultation from a pediatrician if they were displaying any of the above symptoms.

Another symptom found in patients across the US included inflammation of the eyes, specifically if no discharge presented itself that would mirror conjunctivitis, commonly known as pink eye.

“The really worrisome things that we always ask parents to look out for and the reason to seek emergency medical care are obviously if the child is very sleepy, confused, or is difficult to wake,” Dr Rajapakse said, adding difficulty breathing could be another indicator.

“Most of the symptoms are things that can overlap with a lot of childhood illnesses,” she added, “so we need to make sure that people are able to recognise when it happens to treat it.”

One phenomenon of PMIS was the lack of patients previously showing any signs or symptoms of being infected with the coronavirus before the inflammatory syndrome developed. At Connecticut Children’s, none of the hospital’s three patients showed signs of Covid-19.

“That is another defining feature,” Dr Salazar said. “If it wasn’t for this we would’ve never known they had a prior infection with coronavirus.”

New York state has the most presumed cases for PMIS, and the CDC has since asked the state’s health department to develop criteria that would assist other states in diagnosing and treating children with similar symptoms.

Doctors have found that although PMIS presents differently than Kawasaki disease, similar treatments reacted well in the children impacted.

“The kids have been responding fairly well to the standard therapies that we’re using for Kawasaki disease,” Dr Schneider said about his New York patients, “as well as our standard approach to good old critical care, which include blood pressure medicines to support the heart and blood pressure.”

Intravenous gamma globulin (IVIG), an FDA-approved therapy that alleviates acute inflammation in the patient’s body, is one common treatment now being used for children with PMIS.

At Dr Salazar’s hospital, doctors have combined IVIG with steroids to help reduce inflammation. “That combination seems to be the right treatment for this, and so far it has been very effective for the kids we’ve treated,” he said.

Should parents be worried about PMIS?

Research is still underway about how the inflammatory syndrome could impact children and what risk factors might increase someone’s susceptibility. But the condition is still defined as a rare syndrome.

“Proportionately, I would have to say we have to pause, take a deep breath,” Dr Salazar said. “It is something that we worry about if a kid has it, but for an overwhelming majority of parents this is something they shouldn’t be thinking about too much.”

Health officials are raising the alarm to hospitals across the nation to help them distinguish these children from other patients.

In New York, the state’s department of health has required hospital staff to immediately report any cases that could be PMIS.

“The criteria we’re using ... you need to have evidence of a fever, you need to have evidence of inflammation, and you need to have evidence of multiple-organ involvement,” Dr Schneider said.

But research has yet to determine if the syndrome will impact a larger proportion of children.

At the start of the coronavirus pandemic, health officials indicated the elderly population and those with comorbidities were more at-risk to develop severe symptoms from the Covid-19, reassuring parents about their children.

Although doctors are reminding parents of the rarity of the inflammatory syndrome, it serves as a reminder that Covid-19 still impacts children.

“It’s very important for children to continue to follow the preventative measures we have been recommending all along,” Dr Rajapakse said, “because not getting infected in the first place will be one of the things that decreases the incidences of this inflammatory syndrome among kids.”