to call it. MIS-C (multisystem inflammatory syndrome in children,
pronounced “miss see”) seems to be the name that has stuck and
is most common in the literature now. It is often described as a
“mixture of Kawasaki syndrome and toxic shock.”
The symptoms are vague: a child of any age with fever for several
days, sometimes rash, severe abdominal pain, diarrhea, malaise.
And then they may crash, sometimes to the point of needing life
support with ECMO. Severity is over a spectrum and sometimes
continues to worsen despite correct treatment. The actual mortality
rate is still being defined, but we knew in May that it was killing
children. With the high rates of asymptomatic pediatric cases of
COVID-19, there may not have been a telltale preceding illness that
might have prompted testing, and the knowledge that the child was
in the pool of potential victims of this secondary syndrome. MIS-C
is considered to be a rare disease, but it is a zebra hiding in a herd of
galloping horses. In late spring, our regional seasonality also brings
more enterovirus and tick-borne infections, which have strikingly
overlapping features. Even in the middle of a viral pandemic, kids
still get UTIs, salmonella, appendicitis and traditional Kawasaki
disease. The next challenge was to try to learn to hear this strange
beast in the midst of the cacophony of hoofbeats.
So pediatricians did what we always do when confusing febrile
diseases present themselves: we called infectious disease and
rheumatology to the bedside. Indeed, the protocols and guides
coming out of the U.K., New York and other places all called for
multi-specialty teams to evaluate, care for the patient and to make
decisions on resource utilization for treatment. The protocol workup
requires many, many labs, often trended over time as well as ECHO
and ECG. The parents are understandably stunned by the amount
of blood we are taking from their sick children and confused when
infectious disease, rheumatology and cardiology all troop in to see
their previously healthy kids.
However, even with the vast array of test results in hand, the
diagnosis is not simple. Many of the guidelines give decision tree
branch points for “abnormal” labs but do not give a scope of how
elevated or depressed a value should be to prompt real concern.
Additionally, despite 10 years practicing as a pediatric hospitalist,
I simply don’t have the context to know what some markers, such
as troponin or IL-6, typically do in a pediatric patient with a febrile
UTI because we don’t send troponin or IL-6 on these children. So,
in vivo, we rely on communication among all the specialists to
confirm a diagnosis and make a treatment plan.
good bedside care to the patient, coordinate the specialists and
find the alternate diagnosis “off-ramp” if possible. The mainstays of
treatment, besides symptomatic care, were anticoagulation, steroids
and IVIG, depending on the severity of disease.
There were difficult conversations with families to explain why
we were doing all the workup and the potential outcomes. I think
the hardest day was having to tell a mom that whatever precautions
they had taken to isolate, they had not been successful. The child
most certainly had SARS-CoV-2 infection (as shown by antibody
testing). We discussed how she needed to monitor her newborn and
her elderly grandmother at home, while dealing with the diagnosis
of MIS-C in the older child. I could see in her face her worry that
she would lose everyone she loved all at once and there would be
nothing she could do about it now. It was difficult to convey my
compassion while covered head to toe with face shield, mask, gown
and gloves, but I did my best. It was a tiring week.
Thankfully, everyone on my service got better instead of worse.
We had learned a bit more about the disease, created ways to exit
the testing protocols when MIS-C seemed unlikely and worked
together across specialties to communicate as a team. I had never
had to learn about and treat a completely new disease entity in real
time before. MIS-C remains a diagnosis that causes consternation
and concern, but we are climbing the steep learning curve as a team,
across specialties and the world, to continue the mission to keep
kids healthy during this unprecedented pandemic.
References
PEDIATRICS
Article referenced (UK case series with 8 patients) : https://www.thelancet.com/
journals/lancet/article/PIIS0140-6736(20)31094-1/fulltext
Article just released describing disease in New York City, including the hospital
where Dr Kingery worked: https://www.nejm.org/doi/full/10.1056/NE-
JMoa2021756
Most recent article describing MIS-C in the US: https://www.nejm.org/doi/
full/10.1056/NEJMoa2021680?query=recirc_curatedRelated_article
(U of L/Norton Children’s Hospital have contributed to this cohort data)
CDC diagnostic and treatment guidelines for providers as of 7/1/2020: https://
www.cdc.gov/mis-c/hcp/
Dr. Hodge is a practicing pediatric hospitalist at Norton Children’s Hospital. She is
also an Associate Professor in the Department of Pediatrics at the University of Louisville
School of Medicine and Director of the Global Education Office (non-member).
All this came in to play my next week on service. Concern in
the community was so high that it seemed like any child with a
fever for more than 24 hours was getting worked up for MIS-C
in the office or emergency department and sent to us. Frightened
parents were Googling the new disease and becoming filled with
dread. New guidelines were published nearly every other day, and
it was difficult to feel knowledgeable and confident that you were
doing the right things. My main jobs as a hospitalist were to give
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