Putting the Spotlight on Pediatric Chronically Critically Ill Patients

A public health emergency can hide in plain sight, even in top academic children’s centers. Consider patients with pediatric chronic critical illness (PCCI). These are kids and their families who are hospitalized for weeks, months or even years. If these children survive — as is increasingly the case — many become “frequent fliers,” repeat emergency room visitors, cycling through as intensive care unit admissions, to step-down and rehab units, and discharged with alarming regularity.

Historically, medicine has viewed these patients as uncommon outliers in a system designed to seek cures and permanent solutions. That gold standard, that model of “get them better and send them home for good;” can work, even serious cases, such as a baby born with congenital heart disease. Between early medical management and surgery, such a baby should eventually lead a normal life.

That model, however, generally does not work for PCCI cases. We’re talking about a long-term inpatient child with a medically complex issue with no cure.

If the child is fortunate (relatively speaking), they have a well-studied condition such as cystic fibrosis, with a dedicated team that coordinates care among many CF-familiar specialists. Often, however, the diagnosis is a mystery, or an orphan genetic syndrome, with little known regarding treatments or long-term prognosis. Coordinated care teams rarely exist for these children; thus, their medical management is often haphazard. These difficult and sometimes tragic cases have traditionally been seen by providers as one-offs among the pediatric population, and certainly are not business-as-usual.

Or are they?

The few studies done in the 2000s generally focused on pediatric intensive care unit patients, leading many providers to assume that the PICU is the only place in the hospital such cases would be found, which turned out to be critically incomplete intel.

A 2019 Johns Hopkins study shed alarming light on the breadth and depth of what has now become the PCCI crisis. Conducted in conjunction with the multi-center PCCI Collaboration, researchers at the University of Rochester and Johns Hopkins Children’s Center took a single-day snapshot of six pediatric tertiary medical centers. It found that 41 percent of children outside the PICU and NICU setting (as in, on the general wards) also met the criteria for pediatric chronic critical illness.

Their needs, just as with their NICU and PICU counterparts, are overwhelming the traditional system of pediatric clinical care. In return, the system — never designed to fully address these patients’ long-term situation — is placing a crushing emotional and financial burden on these children and their caregivers.

Even within the walls of the Johns Hopkins Children’s Center, doctors and nurses managing certain PCCI cases wonder aloud whether they are doing more harm than good. They also freely admit that the entrenched system of conventional staffing, communication and financial commitment and reimbursement for these patients needs to adapt — and fast.

But how?

Premature infants — the children believed to make up the majority of PCCI cases — were once literally seen as a sideshow. Through the 1940s, a surviving premature infant (under 38 weeks) was such a rarity that preemies on display in incubators attracted huge crowds at the 1939 New York City World’s Fair.

Technology and pharmaceuticals have changed all that. By 2015, some 80 percent of babies born at 26 weeks survived. Studies found that some 20 percent of those children went on to lead normal lives. Another 34 percent had “mild” disabilities; minor cognitive and/or visual impairments. But nearly half experienced moderate (24 percent) to severe (22 percent) disabilities. These latter cases often have no formal diagnosis. They can also require life-long care, frequent hospitalizations, cadres of specialists and therapists, dozens of regular outpatient checkups, and families turning their homes into permanent mini-ICUs.

“These families are suctioning their children, setting up tube feedings, managing the ventilator and physical therapy, (and) providing very, very high levels of medical care in the home,” says critical care neonatologist Renee Boss.

In a vacuum, or as words on a page, this can suggest a seamless transition, with numerous medical professionals working hand in glove with parents, families and extended community caregivers toward a common goal: What is best for the child?

Reality is far messier, often a never-ending communications snafu across the entire chain of care. The norm is this: Doctors never trained nor have given proper time to consult across multiple specialties simultaneously, repeatedly and at length — let alone truly involve families in the long-term ramifications of treatment decisions. Then again, most doctors were never required as students, residents, fellows or attending physicians to spend a minute in a patient’s home. So they really have no first-hand awareness of how disruptive it is for parents and caregivers to be asked to turn their domicile into an ersatz hospital.

Consider also the front-line warriors in the process; NICU, PICU and general ward nurses. The emotional toll of caring for PCCI children for 12 hours a day, weeks on end, and often seeing little to no improvement can be devastating. It’s actually fairly common for nurses to request removal from such cases.

“If you feel like our system doesn’t let us do the right thing, it’s very painful; it has a huge impact on everybody, but especially nurses. They suffer a lot of moral distress,” says Johns Hopkins Children’s Center’s Chief Medical Officer Maggie Moon.

While nurses and doctors get mandated time away from these cases, for inpatients and families, there’s no escape from an experience that’s been described by parents as akin to living in a war zone.

If that sounds traumatic, ponder the other severe stressors PCCI parents experience. There’s the horror of seeing one’s child tethered to life-sustaining equipment. There’s the intense guilt many parents feel, believing that their genetics, environment and/or lifestyle choices somehow contributed to their child’s situation. Taken as a whole, providers often wonder whether such emotionally frayed parents can competently give informed consent for medical care, no matter how much information is provided (see sidebar, page 22).

When one attempts to unravel all the issues involved in these booming PCCI cases — clinical, ethical, financial, psychological, legal and administrative — it’s clear that ad hoc solutions no longer suffice. What’s needed, hospital administrators say, are well-funded, system-wide initiatives supported from top to bottom. Those take time, effort, and, perhaps most importantly for administrators, quality data showing improved outcomes and positive bottom-line impact. But maybe, just maybe, the vanguard for such change within the Children’s Center is finally in place.

Implementing a Team Approach

Imagine running the military without, say, the Joint Chiefs of Staff issuing expert guidance. At the least, the situation among the rank-and-file would be chaotic, redundant and wasteful; at worst, downright dangerous.

And yet, that’s the way complex PCCI cases are often handled — and not just at Johns Hopkins. Re: that aforementioned 2019 report showing the exploding number of PCCI cases? It found that just one of the six hospitals surveyed had a dedicated PCCI complex care team — physicians, nurses and other providers extensively experienced with such cases, tasked with supervising and coordinating the extensive care and support needs for patients and family.

Two of the study’s authors are the Hopkins Children’s hospitalists Eric Biondi and Chris Grybauskas. Both men know that, absent a complex care team, PCCI patients suffer discontinuity of care: Whenever these children come into an ER and are admitted, they are assigned to the specialty team dealing with their specific acute issue (e.g., pulmonology for respiratory distress) and not a team familiar with their overall case.

Such random assignments virtually assure that advice is rarely sought (or is outright ignored) from parents intimately familiar with how their chronically ill kids best respond to certain treatments. Whether it’s setting ventilator pressure, suctioning a tracheostomy tube, prescribing a certain medicine or drawing blood, providers understandably default to “the book.” However, adhering strictly to ICU traditions or standard-of-care guidelines sometimes yields substandard results.

“Some parents know [from home experience] that if you set the suctioning machine higher, to #8 instead of #7 [removing secretions more aggressively from the airway], the child will decompensate. But the ICU staff always uses #8. And what do you think happens?” asks Boss. “Maybe one way to reduce length of stay in the ICU is to follow the parent’s guidance.”

To ensure that PCCI parents are involved and outcomes are optimized, Biondi and Grybauskas formally introduced a Complex Care Consult Service last year. It’s hoped that eventually the service will be upgraded to a Complex Care team that completely oversees PCCI cases. Biondi, director of Pediatric Hospital Medicine, came to Johns Hopkins in 2017 after implementing the University of Rochester’s (NY) Complex Care Team.

“If you look at my career, it’s basically me getting angry about something on a Wednesday afternoon and wanting to fix that thing quickly,” says Biondi, who chairs the American Academy of Pediatrics VIP — the Value in Inpatient Pediatrics network. (The VIP is a national quality improvement initiative involving hundreds of pediatric sites.)

Biondi found a kindred spirit in Grybauskas. A Maryland native and medical director of 9 North, a general care unit, Grybauskas is kind, compassionate … and blunt. “For me, treating these patients right is all about fairness and justice,” says the hospitalist. “Sometimes it’s not a popular thing that, in many of these cases, I’m trying to convince people that the old-fashioned way of delivering care is not up to par.”

Upping the ante is the medical limbo common to many PCCI cases. “You can be a very sick and complex child with cancer and have a fantastic cancer team devoted to you — resources, doctors, and health care professionals who are part of a well-oiled team,” notes Grybauskas. “But say you have a poorly studied rare chromosomal deletion that affects seven bodily systems? Then you’re seeing seven different Hopkins specialists. There is no well-oiled team, no one captaining the ship, no one working together in any formal sense. It’s just the luck of the draw that one family gets a cohesive experience and one doesn’t.”

Working with vital funding from the Children’s Center Innovation Grant Program, Biondi carved out 20 percent of Grybauskas’ clinical time to implement the consulting service. Grybauskas, in turn, recruited veteran nurse practitioner Kathleen Naughton, whose peers consider her exceptional at mining voluminous Epic data, intake charts, and lengthy parent interviews to highlight crucial information. The result is a succinct synopsis that helps providers assessing a new patient.

“What’s created is kind of like a portable medical summary,” says pediatric hospitalist and internist Sara Mixter, who works part time with the service. “When I first meet a patient, I don’t necessarily need a blow-by-blow of their entire medical history. I need to know almost intangible things — like when this child has an episode of hiccups (normally a mild and temporary issue). That’s always a sign that something is going wrong. These are things that, unless they’re clearly pointed out, a physician wouldn’t otherwise know.”

Naughton and Grybauskas strive to uncover big-picture issues that can go overlooked in crisis situations. On this day of daily rounds, they’re sitting at a PICU nursing station with longtime nurse practitioner Beth Wieczorek. Her patient is in the PICU with severe malnutrition for the third time in a year.

“This should’ve been caught by the doctors the second time,” grumbles Grybauskas, pointing to a monitor showing the child’s vital stats during all three hospitalizations. The pattern is clear: significant, lifesaving weight gain while an inpatient; dramatic weight loss while at home.

Not content to let the cycle continue, the duo and Wieczorek drill down into the case. Who are the home care providers? Parents? Nurses who accompany the child on the bus and at school? Previous experience suggests that offering additional feeding education could be the answer: Many home and school caregivers struggle with the nuances of using a permanent feeding tube, which this child has.

The consult service is all about asking the right questions and then, when possible, finding workable solutions. Since they opened for business, Grybauskas estimates that specialists have asked for 80 consults. Their service is gaining awareness across the Children’s Center, augmented by a proactive approach: The pair reviews every inpatient admission to see which kids fall under the sometimes hard-to-recognize PCCI banner (as in, what constitutes a Complex Case? Should two specialty teams constantly be involved? Three? Five? No national guidelines exist.)

They’re also constantly touting their service with specialists and parents, including a talk Grybauskas gave recently to the Pediatric Family Advisory Council (PFAC). “This is a game changer for the families that have been able to take advantage of it,” says PFAC member Michelle Morrison. Her 9-year-old son Timmy is a stable medically complex patient who goes to school, loves Legos and video games and has generally experienced a marked decrease in hospitalizations. While Morrison and her husband Mark personally cobbled together Timmy’s team of a dozen specialists in Cincinnati, Baltimore, and Philadelphia, she sees the new Complex Care Consult Service as filling important gaps that constantly confront parents.

That includes the often-byzantine demands of insurance companies denying coverage. Charly Walker was at such an impasse. Her 8-year-old daughter Zoey, born with Aicardi syndrome, a rare genetic condition affecting the brain and other body systems, was being denied a $100,000 bone-saving drug for a seemingly bizarre reason: Zoey’s insurance would cover only an outpatient infusion. Yet no clinic or hospital near her rural Pennsylvania home would administer the drug, fearing complications.

For two years, the little girl went without the medication. Her bones became brittle. Some broke. Enter Grybauskas. It took three days of work and numerous machinations, but in the end, he got the drug covered and administered in the Children’s Center. “That was a victory, but the bottom line was: this kid suffered fractures that the kid never should have suffered,” says Grybauskas. “To me, that’s infuriating.”

For her part, Walker was amazed. “I was like ‘Wow! He was able to accomplish (getting Zoey’s drug) within just a couple of days,’” says Walker, cautiously. “I just hope it isn’t an issue when she has to get the drug again in six months.”

Though the Complex Care Consult Service is new, Grybauskas says he’s using other established Children’s Center programs as a model for growth. He points to Child Life and the Palliative Care Team as success stories: They started small, proved their worth across the Children’s Center, and steadily increased their budgets and staff.

One story is particularly instructive. It involves the Palliative Care Team’s Director Nancy Hutton and the type of dedication that inspires Grybauskas’ work. Several years ago, Hutton met a complexly ill child constantly cycling in and out of the Children’s Center. Hutton was convinced that the child’s family, with more physician involvement and reassurance, could handle most of the child’s emergent issues at home. Hutton visited the child’s home quarterly and talked to the family via phone and video chat both weekly and whenever a mini-crisis arose. The result?

“He’s been out of the hospital for nearly three years,” says Grybauskas. “Now, Nancy has also done a lot. She’s prescribed him medications and functioned as a long-distance primary care doctor for a very complex kid. I had seen this kid when he had been hospitalized (prior to the consult service’s creation). I worked really hard on his case and never thought he would have responded so beautifully to Nancy’s efforts, to her care. She’s really good. And this is also a testament to how simple solutions can be in a disjointed medical system, if you have that one line of communication to somebody who is really invested in you, that can smooth over a lot of other problems.”

In addition to the support of his palliative care colleagues, other Johns Hopkins providers are buying in. Maggie Moon is proud of the consult service’s initial efforts, observing that, “They really acknowledge and embrace those patients’ complexities and help families with their experience of care so they’re not starting at zero again every time they come in.”

Still, there are long-term concerns. The consult service is available only during weekday business hours. That’s not likely to change anytime soon, according to several providers not directly involved with the service who spoke on background. They noted that full-fledged funding for similar concepts haven’t previously come through, and the institutional expectation is still that providers should volunteer their time to such teams and efforts.

Yet, despite this and other potential funding roadblocks — notably Maryland’s unique hospital reimbursement system and Medicare’s loophole that gives higher reimbursements to freestanding hospital specialists than to their Children’s Center counterparts — Grybauskas has faith that the bottom-line overseers get the big picture.

“I’ve had administrators say to me, straight up, ‘Chris, we don’t know how to fully fund a Complex Care Team.’ But they understand, even if we don’t have the data yet, that it makes sense that if we spend some money up front — even if it doesn’t generate revenue — these families should use less ICU days and better avoid the highest levels of hospital care. That’s where it’s most expensive to care for them.

“These administrators have told me…’This might not make money, but this is good medicine.’”