Chronic Critical Illness in Infants and Children

A Speculative Synthesis on Adapting ICU Care to Meet the Needs of Long-Stay Patients

Katherine L. Marcus, BA; Carrie M. Henderson, MD; Renee D. Boss, MD, MHS


Pediatr Crit Care Med. 2016;17(8):743-752. 

In This Article

Abstract and Introduction


Objectives: In this review, we examine features of ICU systems and ICU clinician training that can undermine continuity of communication and longitudinal guidance for decision making for chronically critically ill infants and children. Drawing upon a conceptual model of the dynamic interactions between patients, families, clinicians, and ICU systems, we propose strategies to promote longitudinal decision making and improve communication for infants and children with prolonged ICU stays.

Data Sources: We searched MEDLINE and PubMed from inception to September 2015 for English-language articles relevant to chronic critical illness, particularly of pediatric patients. We also reviewed bibliographies of relevant studies to broaden our search.

Study Selection: Two authors (physicians with experience in pediatric neonatology, critical care, and palliative care) made the final selections.

Data Extraction: We critically reviewed the existing data and models of care to identify strategies for improving ICU care of chronically critically ill children.

Data Synthesis: Utilizing the available data and personal experience, we addressed concerns related to family perspectives, ICU processes, and issues with ICU training that shape longitudinal decision making.

Conclusions: As the number of chronically critically ill infants and children increases, specific communication and decision-making models targeted at this population could improve the feedback between acute, daily ICU decisions and the patient's overall goals of care. Adaptations to ICU systems of care and ICU clinician training will be essential components of this progress.


A humbling consequence of critical care advances over the past 30 years is the emergence of "chronic critical illness" (CCI):[1] a complex syndrome affecting patients who survive acute critical illness but remain chronically dependent on intensive medical care.[2–4] CCI in adults is characterized by the hallmark of prolonged ventilator-dependence and a constellation of neuroendocrine and metabolic abnormalities, organ dysfunction, and recurrent infections.[5,6] Most patients die within 6 months or survive with substantial functional impairment.[7] Related healthcare costs exceed $20 billion annually.[5]

The syndrome of pediatric CCI, inclusive of both neonatal and pediatric patients, lacks a clear definition in the literature, but generally includes prolonged ventilator dependence, days in the neonatal or PICU, technology reliance, severe neurodevelopmental impairment, or overlapping chronic conditions.[8] Without a consensus definition, few articles have targeted outcomes for this population. We do know that long-stay PICU patients receive multiple procedures including mechanical ventilation, intracranial pressure monitoring, and dialysis; they use a disproportionate number of bed days and have increased mortality.[9,10] A recent report showed that long-stay patients accounted for 1% of all PICU patients but utilized 18% of PICU bed days; they had a median age of 4 months at admission, and more than 70% of patients had died or had moderate-to-severe disability at follow-up.[11] Similar prevalence and mortalities have been reported spanning various time frames and across many institutions.[9,12,13] Characterizing neonatal CCI poses particular challenges, since extended neonatal ICU (NICU) stays and prolonged technology-dependence are anticipated for many conditions (Table 1). Some NICU patients will not develop chronic medical needs, but a subgroup will transition into repeated and prolonged PICU stays. Irrespective of long-term outcome, patients with CCI endure repeated interventions, setbacks, and uncertainties while their families experience stress and frustration.

CCI is as much a product of medical innovation as of underlying pathology. A few decades ago, many of these children died from "lethal" conditions. Today, survival for months or years is possible owing to a succession of decisions to trial—and continue—life-sustaining interventions. At the core of these daily and "big picture" decisions are repeated clinician-family conversations. Figure 1 illustrates the complex web of communication and decision making for an infant with complex congenital heart disease, a condition that represents a growing proportion of pediatric CCI.[8,10,17]

Figure 1.

Communication and decision making in pediatric chronic critical illness: The case of congenital heart disease. *Adapted from Srivastava et al (17). NICU = neonatal ICU. Adaptations are themselves works protected by copyright. So in order to publish this adaptation, authorization must be obtained both from the owner of the copyright in the original work and from the owner of copyright in the translation or adaptation.

Families of adult ICU patients report that communication progressively deteriorates over a prolonged hospitalization, and they feel less informed and less involved over time.[18] ICU processes promoting consistency and continuity lag behind the need for those services. Here we review important features of longitudinal communication and decision making for CCI patients. We searched for relevant, English-language studies on MEDLINE and PubMed from inception to September 2015 utilizing the following search terms or variations: "CCI," "pediatric CCI," "medical complexity," "long stay," "communication," "decision making," "family preferences," "ICU systems," and "continuity of care." We also hand searched reference lists and existing files of the authors for pertinent references. From this inquiry, we summarized data regarding what families want as decision making progresses throughout prolonged hospitalizations. We also highlighted prevailing ICU systems and models for clinician training that may undermine our capacity to deliver what families need. Targeting this gap, we applied Kelley et al[19] conceptual model of goal-directed care for patients with serious illness to suggest strategies for improving longitudinal communication and decision making.