Five Key Points About Multisystem Inflammatory Syndrome in Children

COVID-19 has proven an unrelenting foe. Its mortality and morbidity in patients who are elderly with comorbidities is high. A high number of young and healthy individuals have succumbed to this disease, as well as elderly patients with multiple comorbidities who survived, defying expectations.

The variable during this pandemic has been children. Most children who develop COVID-19 present with mild symptoms or are asymptomatic. A small proportion develop an inflammatory syndrome termed multisystem inflammatory syndrome in children, or MIS-C.

Not Quite Kawasaki

Initially identified as Kawasaki disease (KD) when it was first seen in the New York City/Northern New Jersey area, MIS-C appeared with the presentation of large numbers of patients out of the norm, combined with a twist on the age group that made doctors hit the pause button. They began to test these patients and found they all were COVID-positive. Since then MIS-C has been identified throughout the United States wherever we see COVID in pediatric patients.

Kawasaki disease is an acute febrile illness of unknown etiology that primarily affects children younger than 4 years. Clinical signs include fever, rash, swelling of the hands and feet, irritation and redness of the whites of the eyes, swollen lymph glands in the neck, and irritation and inflammation of the mouth, lips, and throat. It is one of the leading causes of acquired heart disease in the U.S., with severe complications that can include coronary artery aneurysms, depressed myocardial contractility and heart failure, myocardial infarction, arrhythmias, coronary artery dilatations, and peripheral arterial occlusion. Children with KD may present in severe shock or cardiac arrest.

MIS-C presents the same way as KD, but MIS-C has been seen in patients up to the teenage years. Some of these children will improve with no treatment, and others will require intensive care.

Signs and Symptoms

The signs and symptoms of MIS-C are slightly different than COVID, and it may not occur to parents that their children are infected with either one. Any child presenting with the following signs/symptoms should be assumed to have COVID:

• Fever
• Abdominal pain
• Vomiting
• Diarrhea
• Neck pain
• Rash
• Bloodshot eyes
• Feeling extra tired

The unpredictability of COVID is what is most distressing for EMS. Healthy people, including children, have ended up in the ICU and died. For the few children who develop severe or life-threatening acute respiratory presentations, assessment and management of symptoms are vital in the prehospital environment. In children severe disease would present with the following signs and symptoms:

• Trouble breathing
• Pain or pressure in the chest that does not go away
• New confusion
• Inability to wake or stay awake
• Pale, gray, or blue-colored skin, lips, or nail beds, depending on skin tone
• Severe abdominal pain

Treatment in the Field

If your patient has mild symptoms, monitor their vital signs and pay particular attention to their pulse oximetry. Provide oxygen as needed and transport in a position of comfort. Remember, children have tremendously efficient compensatory mechanisms, but when they decompensate it is akin to dropping a rock off a tall building.

As with all COVID patients, treat the signs and symptoms: poor ventilations, inadequate oxygenation, and low blood pressure/poor circulation. (You can remember these components using the VIP mnemonic, for ventilations/oxygenation, infusion, and pressors.) 

As with all pediatric ventilation, maintaining an airway and oxygenation are our mainstays. With all things COVID, any number for the SpO₂ below 92% is a harbinger of rapid deoxygenation. If we wait for patients to complain of shortness of breath, this may be too late, and we will be surprised by pulse oximetry readings below 84%. Ventilations and oxygenation with high-flow oxygen are imperative.

Before proceeding to vasopressors, volume infusion using a conservative approach to fluid is crucial. Use crystalloids but don’t bolus a patient with too much too fast. The fluid you infuse, if it is too much or too fast, will intensify the leakage in the alveoli.

If you must transition to vasopressors, epinephrine or norepinephrine are the pressors of choice for pediatric patients. The Society of Critical Care Medicine strongly recommends against the use of dopamine. Push-dose pressors or infusion via an IV pump is best.

Pediatric patients with signs and symptoms of MIS-C should be transported to a children’s hospital for evaluation and treatment. If one is not available, a hospital with a pediatric ED is preferred. The clinical progression can be uneven at best, and pediatric patients may need more advanced resources in rapidly progressing disease, so making sure the patient lands at the right institution is important.

MIS-C in My Community

You absolutely will see this. This is one of the reasons for the start of clinical trials involving pediatric patients and COVID vaccines. Cases of pediatric COVID and MIS-C have increased as schools have reopened and restrictions have been lifted. The CDC has reported that the group of patients seeing the most cases during this current surge are those 10–20 years of age.

Long-Term Complications

We are still learning about MIS-C and how it affects children, so we don’t know why some children have gotten sick with it and others have not. We also do not know if children with certain health conditions are more likely to contract MIS-C.

When we talk about “long haulers,” that is a misnomer. Recovery is poorly understood. What does recovery from COVID-19 mean? We have seen 36 million people infected, many of whom have developed cardiomyopathies, lung fibrosis, ground-glass opacities in the lung, end-organ damage from coagulopathies, and neurological abnormalities—strokes, for example. Some patients who have recovered now need heart or lung transplants. The question we struggle with is, are these cases the outliers or part of the long-term trajectory of COVID?  

Currently we do not know the long-term implications of COVID-19 as far as children who develop multisystem inflammatory syndrome. We have seen children develop severely damaged hearts due to COVID. We may not know or understand the full impact of COVID-19 on pediatric patients for years to come.

Summary

We will see MIS-C in our communities. It presents akin to Kawasaki disease, the only difference being that we see it in children into their teenage years. Identify and manage the life-threatening warning signs of cyanosis, chest and abdominal pain, confusion, and decreased level of consciousness. Prioritize airway, breathing, and circulation using the VIP approach. Transport children with MIS-C to a children’s hospital or a facility with a pediatric ED when possible.

Resources

Abrams JY, Godfred-Cato SE, Oster ME, et al. Multisystem Inflammatory Syndrome in Children Associated with Severe Acute Respiratory Syndrome Coronavirus 2: A Systematic Review. J Pediatr, 2020; 226: 45–54 [epub ahead of print].

Association of American Medical Colleges. Coronavirus (COVID-19) Resource Hub, www.aamc.org/coronavirus-covid-19-resource-hub.

Ebina-Shibuya R, Namkoong H, Shibuya Y, Horita N. Multisystem Inflammatory Syndrome in Children (MIS-C) with COVID-19: Insights from simultaneous familial Kawasaki Disease cases. Int J Infect Dis, 2020 Aug; 97: 371–3.

Esposito S, Principi N. Multisystem Inflammatory Syndrome in Children Related to SARS-CoV-2. Paediatr Drugs, 2021 Mar; 23(2): 119–29.

Feldstein LR, Tenforde MW, Friedman KG, et al.; Overcoming COVID-19 Investigators. Characteristics and Outcomes of US Children and Adolescents With Multisystem Inflammatory Syndrome in Children (MIS-C) Compared With Severe Acute COVID-19. JAMA, 2021 Mar 16; 325(11): 1,074–87.

National Emerging Special Pathogens Training and Education Center. Novel Coronavirus (COVID-19), https://repository.netecweb.org/.

National Institutes of Health. Care of Critically Ill Adult Patients With COVID-19, www.covid19treatmentguidelines.nih.gov/critical-care/.

Office of the Assistant Secretary for Preparedness and Response, Technical Resources, Assistance Center, and Information Exchange. COVID-19 Resources, https://asprtracie.hhs.gov/COVID-19.

Pan American Health Organization. COVID-19 Recommendations: Prehospital Emergency Medical Services (EMS), www.paho.org/en/documents/covid-19-recommendations-prehospital-emergency-medical-services-ems.

World Health Organization. COVID-19 Clinical Management: Living Guidance, www.who.int/publications/i/item/WHO-2019-nCoV-clinical-2021-1.

Daniel R. Gerard, MS, RN, NRP, is EMS coordinator for Alameda, Calif. He is a recognized expert in EMS system delivery and design, EMS/health-service integration, and service delivery models for out-of-hospital care.