In football, linebackers play in the middle of the defense between the defensive line and the secondary, where they do a bit of everything from stopping the run to covering pass receivers to rushing the quarterback. Critical care specialist Meghan Bernier sees herself and colleagues in the pediatric intensive care unit (PICU) playing a similar role, especially when it comes to caring for seriously ill or injured children.
“I call us the linebackers — we’re there, very much in the middle of the action all the time even if you don’t really see us,” says Bernier.
That has been especially true this past year since the coronavirus found its way to the Maryland region in early 2020. Following its arrival, a new mysterious and serious — and in some cases, deadly — related disease called multisystem inflammatory syndrome in children (MIS-C) appeared. Early on, its symptoms confounded diagnosis and treatment by intensivists like Bernier — but not so much today.
“Back in May and June 2020 we were struggling with how to treat these patients and what protocol to use,” says Bernier. “Now, we’re 12 months into MIS-C and have developed a lot of experience. Today I can hear a story from an outside hospital or our transport team about a patient with certain signs and symptoms and can say with pretty significant acumen this child has MIS-C until proven otherwise.”
Many children with MIS-C, Bernier explains, typically present with abdominal pain, difficulty breathing, fevers, gastrointestinal issues, inflammation, neurologic manifestations such as seizures, and skin rashes — all signs and symptoms that can also mimic many other diseases.
“What makes MIS-C such a challenging diagnosis is that it has so much in common with other clinical syndromes that we see, like sepsis or even a GI illness,” says critical care specialist Katherine Hoops.
On the other hand, Hoops adds, the signs can be really subtle — a challenge for families and clinicians to see the forest through the trees.
“It looks like a lot of things but the effects can be devastating,” says Hoops.
Amanda Levin, who leads one of two PICU teams that admit MIS-C patients, agrees: “In the beginning, so many patients came in with vague symptoms, really sick and we weren’t sure why, or seemed to manifest MIS-C with a predilection for one or two organs involved as the primary problem. Early on, and even now, people are not always recognizing that it might be MIS-C, even though we’re getting more and more savvy. We see the whole gamut.”
Further complicating diagnosis and treatment, however, is how quickly these intensivists have seen patients decline due to a tsunami of inflammation attacking multiple organ systems.
“Rapid diagnosis and rapid initiation of treatment is really important because kids can get very ill very fast,” says Hoops. “They may have progressive multisystem failure with heart failure, respiratory failure needing mechanical ventilation, and kidney failure needing dialysis.”
Adds Bernier, “They can go from walking into the ED to needing life support within hours.”
Initially, Hoops says, there was a lot of fear among health professionals about how to respond to MIS-C. At the same time, she adds, intensivists do not like to be put on their heels, which prompted an aggressive fast-paced learning process in the PICU to work collaboratively to understand how MIS-C presented and what treatments could best tame it. They have seen enough cases to always have a high index of suspicion for MIS-C if a critically ill child comes in with symptoms consistent with sepsis.
In addition, garnering 12 months of experience encountering the signs and symptoms of MIS-C, they have built — seemingly brick by brick with each patient they’ve seen or case they’ve reviewed — a diagnostic and treatment algorithm with their pediatric subspecialist colleagues in cardiology, hematology, infectious disease and rheumatology. As cardiac and respiratory functions are the highest priority concerns in their protocol, the intensivists adhere to the ABC formula — airway, breathing, circulation — they’ve been trained to follow for life-threatening conditions.
“Our main role is to help stabilize the critical functions of the body, to make sure the child’s blood pressure is staying stable, the heart rates and function are working appropriately, and the patient is breathing and exchanging air acceptably with whatever medicines and interventions are needed,” says Bernier.
This stabilizing step relies on a team of faculty physicians, fellows, residents and nurse practitioners — a tailored MIS-C group of specialists within the PICU — working 24/7 to constantly evaluate and initiate therapies to prevent worsening of illness. Meanwhile, at times because time is of the essence, Bernier adds, “you have to go with your gut instinct and your best idea of what is going on with the child.”
To help fill in any holes in care — like an unrelenting fast heart rate that despite fluid or antipyretics cannot be brought down — they reach out to their subspecialist colleagues for speedy remedies and input on workup, possible causes and treatment. Collaboration and communication, stresses Bernier, are essential.
“We’re still generalists in the ICU — we can do 80% to 90% of the work, but we need the help of our specialists to refine the last 10% to think of presentations and diseases and workup we hadn’t thought of,” says Bernier.
Hoops agrees: “Through this process we’ve been grateful for a lot of collaborative work from a multidisciplinary team to better understand the disease process and also to develop diagnostic and treatment protocols so we can rapidly identify new cases and quickly intervene to give our patients the best evidence-based therapies.”
One such proven targeted therapy is intravenous immune globulin (IVIG), which Bernier describes as applying white noise to the immune system: “It’s amazing to watch this listless child lying in bed febrile and tachycardic, then get the infusion of IVIG to quiet the immune system, and six to 10 hours later that child has perked up and is playing in the parent’s lap or walking around the room. The parents are like, ‘I have my baby back.’”
That, however, has not and will not always be the case, says Hoops, noting that each child’s recovery is different: “When you see a child in the PICU, it’s hard to predict if they’re going to return to their baseline level of functioning in a week, a month, a year. But we are encouraged that kids are leaving the ICU faster and responding to those therapies.”
So, the learning curve continues?
“Oh sure, we’re still refining these algorithms as we learn about new therapies shown to be effective by our colleagues here at Johns Hopkins and around the world,” says Hoops. “Our practice is constantly evolving with the evidence — that is how we in critical care manage anything.”
The intensivists are also encouraged by the response of organizations like the American Academy of Pediatrics, which they describe as doing a “spectacular job” in educating general pediatricians across the country about the disease process of MIS-C and how to appropriately evaluate and refer patients. The intensivists also point to national collaboratives including rheumatologic societies and the Centers for Disease Control and Prevention in pushing out educational briefs on management of MIS-C. The result is greater recognition of the signs and symptoms of the disease and the need for urgent referral by the front-line general pediatricians.
“Our colleagues in the community are more aware and bringing this up in their differential and thinking of it much faster,” says Bernier.
Managing MIS-C, the intensivists conclude, is not by any means easy work.
The alarm-bell, all-hands-on-deck moments when everybody swoops in to quickly reverse the inflammation and potentially save a life is both exciting and rewarding. But not all patients survive, which takes an intense toll on the team members as well, as they see firsthand the struggles and distress families face — which they also face.
The rewards they cite are seeing a child turnaround following treatment, in some cases dramatically, and getting to know a patient and the family at the bedside or on twice-daily, family-centered rounds. They also cite an intrinsic reward — doing what they love to do well.
“I was drawn to the PICU because I like caring for and thinking about the whole child and the interaction of all of the body’s systems,” says Hoops. “I love our work caring for critically ill and injured children — they challenge us all to be our best.”