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Special COVID-19 Update: Multi-System Inflammatory Syndrome in Children (MIS-C)- Some Real Progress

By Dr. Arthur Lavin

Glossary

  • Virus– a type of germ that consists solely of a bit of genetic material (DNA or RNA) wrapped in a protein coat.  The coat gets the genes into the target cell where the genes force the cell to make zillions of new viruses, and on it goes.
  • Coronavirus– a species name of a number of different viruses.  Called corona because its protein coat is studded with spike shapes that form a crown, halo, or corona of spikes
  • SARS-CoV-2– the specific name of the new coronavirus
  • COVID-19-the name of the illness that the new coronavirus is causing
  • Endemic– an illness always present in a region.  One could say strep throat is endemic in the US
  • Epidemic– a sudden burst of an illness that comes and goes over a limited time
  • Pandemic– an epidemic that bursts across the world not just one region
  • Spreadability– how contagious is the disease, how many people will end up infected
  • Severity– what harm does the disease cause, in terms of  how sick you get and how many it will kill
  • Mask- a mask is a loose-fitting cloth or textile that covers the mouth and nose loosely.  A surgical mask is a mask used in surgery
  • Respirator-  for the purposes of the COVID-19 pandemic and other respiratory illnesses, a respirator is a mask that fits very snugly or tightly to the user’s face.  An N95 mask is a respirator.
  • Personal Protective Equipment (PPE)- PPE are any item that covers any part of the body with the design and intent of keeping viruses in the environment from infecting the wearer of the PPE. PPE’s include all masks (which includes respirators), face shields, eye shields, gloves, gowns.
  • Ventilator- a ventilator is a machine that can force a person unable to breathe to inhale and exhale and control both effectively.  They are sometimes called respirators, but during this pandemic the word respirator is now reserved for reference to a tightly fit mask.
  • Live Virus Swab– this is the swab which attempts to swipe live virus from one’s nose or throat to see if you are currently infected.
  • Antibody Test- (aka serology test) this is the blood test which looks for antibody to the SARS-CoV-2 virus to see if you have been infected in the past.

 

Multi-System Inflammatory Syndrome in Children (MIS-C): Important Updates- Better detection, better outcomes

On May 12, 2020, Real Answers posted an essay on MIS-C.  Back then it was called Pediatric Multi-system Inflammatory Diseasse or PMIS, now it is officially called MIS-C.

The features of the illness remain essentially unchanged, so we are re-posting this essay with few changes, but there are two important updates first.

Better Detection

The biggest news is that pediatriicans, intensivists, infectious disease specialists, immunologists, literally around the world are getting together on-line to discuss all the children with MIS-C they are seeing, comparing notes on how to diagnose the condition early, and what treatments work.

This is still a work in progress, the first case appeared in the world only a few weeks ago, but much is being learned quickly.

The better detection quest is urgent for many reasons:

  • It would be so helpful to know someone suspected of having MIS-C does not have it.
  • It could really help someone recover if we found they had MIS-C early on, before they got too sick.
  • With therapies being developed- see below- and used to good effect now, early treatment can make a real difference.

The news is that blood tests that measure how much various indicators of inflammation circulating in the blood have gone up just might be reliable to tell us if someone has MIS-C.

The details of these tests’ performance is not yet reported, but the news story is encouraging, https://www.nbcnews.com/health/kids-health/blood-markers-discovered-covid-linked-syndrome-children-n1215546

Again, preliminary data are not a solid rock to build a plan on, but they are a glimmer of hope, and have already helped some children.

Better Outcomes

The discussions across the world sharing information also is giving some early signs that treatments being tried appear to be working well.

Early diagnosis and early treatment are leading to many children recovering so well.

Treatments being tried include use of IV administration of antibodies found in donor plasma, a technique called IVIG, which works very well in Kawasaki’s Syndrome, and appears to help in MIS-C.  Other therapies include cutting-edge medications to stop inflammation.

Now, our May 12, 2020 posting on MIS-C, now updated:

Originally a tiny handful of children in the world were known to have this develop.  Then in the last few weeks, cases began appearing in NY City, then a number of other states, including here in Cleveland/

The trend over the few weeks is clear, MIS-C is not a one-time fluke that will only happen once, in one place, and not again, and nowhere else.  Cases are showing up in more and more places, and the children affected all share similar experiences.

The fact that MIS-C is more possible still has not changed the fact that it is still very, very, rare.

What is MIS-C?

As readers of Real Answers will have read several times, MIS-C is a type of vasculitis.  The title says much of what it is about.  It is a problem seen in Children.  As far as we know MIS-C is the only problem that happens to people with COVID-19 that happens mainly in children.  It is surely a Multi-system problem, affecting many parts of the body, as we will describe.   The heart of MIS-C is Inflammation, and it is not well understood beyond the collection of symptoms it involves, and a collection of symptoms not yet fully explained is a Syndrome.  Hence MIS-C.

Now let’s take a closer look at these features.  First, Inflammation.  As described in earlier posts, the immune system has one job, to destroy germs and cells that might destroy us.  It is an army of cells that makes antibodies, and a host of chemicals, and cells too, that can destroy almost any germ or enemy cell.   But it is also an army our body is so careful to keep from firing its weapons unless the enemy is found, and keeping our fire trained on that enemy, not our own cells.   Consider what should happen when our immune system comes across say the strep germ in our throat- it destroys the strep germ and ideally leaves all the normal cells, that is us, next to the strep germ, alone.   Inflammation is a great word for this process, because the chemicals our immune system use actually attach oxygen to the offending germ, that is, it burns the germ, or inflames it.

Typically our immune system overshoots when activated, and cells around the enemy get blasted, or inflamed, and the result is inflammation.  The key features of inflammation are swelling, redness, pain, heat, and even loss of function.  We see the first four in almost all examples of inflammation, including the little red bump of a mosquito bite.

What we are seeing in MIS-C is a whole body storm of serious and severe inflammation.   When our blood vessels get inflamed, that particular form of inflammation is called a vasculitis, or inflammation of our vasculature.  When blood vessels get inflamed, that is, swollen, and when seen red, that means they have trouble circulating blood well, and blood pressure can drop.  If that gets bad enough, a person can go into shock.  This is why when you read about MIS-C you will see that it has features of an old vasculitis from exposure to severe staph infections called Toxic Shock Syndrome.

Further, when blood vessels get inflamed, getting red and swollen, that can be visible where the blood vessels come close to our surface, that is, in our skin.  That redness and swelling is seen as a very disturbing rash, not the typical mild red dots of a diaper rash or common viral illness.  Rather the rashes look like the skin itself is discolored, an angry red or even purple color.

The inflammation of MIS-C affects not only a child’s blood vessels, but their heart, and increasingly now, their gut.   The heart inflammation can be of course very worrisome, interfering with heart function.  Another state of inflammation seen only in childhood that causes striking rashes and a very specific type of heart inflammation is Kawasaki’s syndrome, which is why when you read about MIS-C reports often state it reminds doctors in some ways of Kawasaki’s syndrome.

When it comes to our GI tract, MIS-C appears to cause significant inflammation there as well, leading to serious abdominal pain, vomiting, and/or diarrhea.

As to cause, the only explanation we have is that infection with the SARS-CoV-2 virus seems to be the cause.  No such infection, not MIS-C.  How this virus actually creates such widespread inflammation and why so far mostly in children, remains at this time unknown.

And so, a full-blown case of MIS-C involves a very severe rash, terrible stomach ache, sustained fever, vomiting and diarrhea.

But the problem for all parents is that all children have mild instances of such symptoms.  Almost everyone has a fever at some point, or a rash, or a stomach ache, or even diarrhea.  How do you know if your child is fine, or has one of these universal experiences if it is an early sign of MIS-C, how can a parent know if their child is developing such early symptoms, or is having the far more likely experience of a common, non-MIS-C- completely harmless rash, fever, stomach ache?

What to Look For?

In this section we will try to provide guidance to specifically address this question, particularly now that we know COVID-19 related MIS-C is here in Cleveland.

The key point to be made is that as will any illness, the better your child looks, the less you have to worry.

In many ways the situation is now no different than any time your child, or you, have a cold.  Even before this pandemic, a person with a cold could possibly develop a more serious illness, such as a serious pneumonia.  How could we know if our child had a cold if they were going to, or actually were developing, a dangerous pneumonia?

The answer to that question has always been the same.  A child who felt fine, was acting normally, whose cold symptoms were mild was so unlikely to have a serious pneumonia or about to develop one, that it would be unreasonable to subject them to evaluation for one.   By the same token, a child with a cold who started struggling to breathe and looking more and more ill was far more likely to be developing something more than a cold, something serious, and needed attention right away.

And so it is with MIS-C.  A child with a mild fever, with a mild rash, who feels fine, very playful, no real distress, no real pain does not have MIS-C and having that child subjected to evaluation for it would cause more harm than good.

So when does the situation cross the line from nothing to worry about, to something to worry about?  Here are some specific guidelines:

  • First and foremost, your basic sense of how sick your child is, or just how worried you are.  That is a sense all doctors rely on and for good reason.  If you think she or he is fine, they probably are.  And if you  are worried and they are fine, no harm done. So if you are worried, call us.
  • MIS-C is marked by high (103 and up), sustained, prolonged fever.  A temperature of 101 each evening for a couple of days is not high, sustained, and prolonged.  But a fever of 104 most of the day for many days, definitely requires attention.
  • Trouble breathing.  Lots of kids with colds sneeze and cough, but between sneezes and coughs, their breathing is very comfy.  The trouble breathing seen in MIS-C looks like a child is having trouble getting air in and out of their chest when not coughing.  If you see this, call for sure.
  • A peculiar rash.  Again, little red dots of diaper rash of with mild colds are not what MIS-C looks like.  The rash of MIS-C looks like the skin itself is raised with swelling, or changed color, often an angry red or even purple color.  Most innocent rashes blanch when pressed, the rashes of MIS-C often will not blanch when pressed.  These rashes of MIS-C, like in Kawasaki’s, look very disturbing because they look so unusual.
  • Severe weakness, rapid pulse.  Almost all children with colds get tired, and their heart races.  So a rapid pulse is not a helpful distinguishing sign between a plain cold and MIS-C.  But kids with MIS-C get more than tired, they get hard to wake up.  If that happens call.
  • Severe abdominal pain, vomiting, and diarrhea.  All children experience stomach flu, with real stomach ache, vomiting and diarrhea, but in particular, if the abdominal pain goes beyond just achiness, and seems like real and even severe pain we need to know.
  • Also, combinations of the above symptoms, of course, merit immediate medical attention.

What it Means for Your Family

The emergence of MIS-C pierces the wonderful invulnerability that had protected our children, and the world of concern we always have for them.

But the emergence of MIS-C, so far, has only raised the possibility of a very, very rare event.  Clearly life would be far better if the chance of a serious problem like MIS-C had remained zero, if it never had emerged.  But if COVID-19, if the SARS-CoV-2 virus was going to cause trouble for children, I continue to hope it remains a very, very rare event.

So now that this terrible condition, MIS-C, has spread to our home community this is what it means:

  1. We all now need to know what MIS-C is and the key symptoms that indicate it is developing, as noted above.
  2. We all need to continue, as we always have, to put our children’s experience into reasonable perspective, and the most important perspective is that if they are acting well, their rashes and fevers are not bothering them much, if they are breathing fine, and if they are in no discomfort pain or distress, the rarity of PMIS comes to reassure us.
  3. At the same time, should the symptoms noted above emerge, and the troubles seem severe, we now need to know PMIS although rare, can happen here, and we should be called.

BOTTOM LINES

  1. We all now need to know what MIS-C is and the key symptoms that indicate it is developing, as noted above.
  2. We all need to continue, as we always have, to put our children’s experience into reasonable perspective, and the most important perspective is that if they are acting well, their rashes and fevers are not bothering them much, if they are breathing fine, and if they are in no discomfort pain or distress, the rarity of MIS-C comes to reassure us.
  3. At the same time, should the symptoms noted above emerge, and the troubles seem severe, we now need to know MIS-C although rare, can happen here, and we should be called.
  4. Now, some tests may help us determine if a pattern of inflammation that suggests MIS-C is brewing is emerging.
  5. And, so many children recover well from MIS-C, there is good reason to hope for a good recovery even when it, rarely, occurs.

To your health,
Dr Arthur Lavin

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