Chronic critical illness, a complex syndrome affecting patients who survive acute critical illness but remain chronically dependent on intensive medical care, is a major public health burden that extends to the paediatric population.Reference Kahn, Le and Angus 1 Recent data suggest that the proportion of chronically ill children with a primary cardiac diagnosis is increasing, and is now >40%.Reference Namachivayam, Alexander and Slater 2 Care for these children involves multiple prenatal, neonatal, and paediatric subspecialists; clinician communication with families about evolving prognosis and goals of care often fractures as hospital stays lengthen.Reference Boss, Nelson and Weissman 3
In this article, we present three cases representative of paediatric chronic critical illness related to CHD. This case series highlights patient, family, and clinician/system “red flags” – risk factors for suboptimal family–clinician communication and decision making (Table 1).
Table 1 Three cases: red flags.
Case 1
A twin male was born at 36 weeks of gestation weighing 1700 g at a community hospital following intrauterine growth restriction. In the newborn nursery, he developed tachypnoea and cyanosis, prompting transfer to the special care unit. An echocardiogram revealed truncus arteriosus type IIIA with discontinuous branch pulmonary arteries. He was transferred to our tertiary centre, where multiple setbacks – that is, extracorporeal membrane oxygenation, sepsis, and necrotising enterocolitis – occurred, complicated by family mistrust of clinicians and cultural barriers. After an initial 102 days of hospital stay, he was transferred to a chronic-care facility, and from there to another. On day of life 229, he was re-admitted to our centre and died within 48 hours of presumed septic shock.
Case 2
A term newborn with intrauterine drug exposure and limited prenatal care was born at a community hospital with omphalocoele and cyanosis. He was transferred to our hospital. An echocardiogram confirmed single-ventricle heart disease: unbalanced atrioventricular canal, pulmonary atresia, discontinuous pulmonary arteries, and dextrocardia. His initial hospitalisation was for 262 days; he underwent multiple surgical procedures, including cardiac surgery, tracheostomy, and feeding tube placement. His course was complicated by worsening neurodevelopmental prognosis and uncertainty regarding parental legal authority to make medical decisions. He was transferred to a chronic-care facility, went home for 1 day, and was re-admitted for acute on chronic respiratory failure, due to which he passed away at 21 months of age.
Case 3
A fetus was diagnosed at 28 weeks of gestation with pulmonary atresia, double-outlet right ventricle, intrauterine growth restriction, cerebral ventriculomegaly, and polydactyly. Prenatal providers acknowledged prognostic uncertainty, and told the family that a life-limiting syndrome could preclude cardiac repair. A prenatal provider referred the family to hospice; a few other prenatal and postnatal providers knew about this. Elective caesarean section at 39 weeks of gestation was performed at our centre. The clinical course was complicated by recurrent necrotising enterocolitis and bacteraemia, multi-vessel thrombosis, and cerebral infarcts. Extracorporeal membrane oxygenation was considered more than once; there were no documented postnatal discussions regarding alternatives to aggressive interventions. The initial hospital stay was 184 days, with multiple surgical procedures (two cardiac) recorded. Patient management involved >10 subspecialties in addition to neonatal and paediatric intensive care and cardiology.
Discussion
Paediatric chronic critical illness is a relatively new term that describes prolonged or frequent ICU stays together with multi-organ involvement and dependence on life-sustaining technologies.Reference Marcus, Henderson and Boss 4 Patients with CHD, especially those with single-ventricle physiology, are at high risk of paediatric chronic critical illness.Reference Namachivayam, Alexander and Slater 2 Although quality of life, including neurodevelopmental outcomes, in outpatients with CHD is high overall, it is lower in those with complex anatomy and multiple surgeries.Reference Svensson, Idvall, Nilsson and Liuba 5 Hehir et alReference Hehir, Easley and Byrnes 6 detail the non-cardiac challenges for patients in the cardiac ICU, including analgesia, anticoagulation, and feeding. Most paediatric deaths from CHD occur in the ICU following aggressive interventions and amidst significant suffering.Reference Blume, Balkin and Aiyagari 7 Data suggest that these patients are less likely to be seen by a palliative care team than children with other life-threatening diseases.Reference Keele, Keenan, Sheetz and Bratton 8
Coordination of care, communication, and decision-making support are fundamental to improved outcomes for seriously ill children. In this study, we identified patient, family, and system “red flags” that elevate the risk for communication breakdown in the context of CHD. Absence of prenatal diagnosis, extracardiac anomalies, cultural barriers, and social vulnerabilities were common patient and family red flags. Clinician/system red flags included involvement of multiple subspecialists, transfers between units and institutions, and lack of an identified leader responsible for navigating care goals with the family. Out of the three, two patients died without ever going home; we found little documentation of conversations with parents about end-of-life care.
Table 2 highlights potential strategies for addressing these patient, family, and system red flags for patients with CHD and paediatric chronic critical illnesses. These strategies harness different resources to anticipate and address common challenges. Designated members of the broad healthcare team could prioritise continuity of patient care planning and communication with the family. Continuity providers might be primary cardiologists who follow-up the patient throughout the hospitalisation, social workers who provide services across sites within the hospital, or palliative care teams who follow-up the patient across any site of care, starting from the prenatal period. Protocols to routinise family meetings and ICU-subspecialist team discussions are evidence-based methods to enhance communication and decision making.Reference Marcus, Henderson and Boss 4 Early clarity regarding which family members and which clinicians will play lead roles in decision making can preempt the need for care escalation by default.
Table 2 Strategies for addressing red flags.
As we continue to improve survival and quality of life for children with CHD and their families, it is also important to improve outcomes for those children with life-limiting disease. Although cure may not be possible, minimising burden and maximising benefit remain central goals.
Acknowledgements
The authors are grateful for the guidance and expertise of Dr Nancy Hutton in the completion of this study. As a leader in paediatric palliative care, her involvement has been invaluable.
Financial Support
This work was made possible by funding accorded to Dr Boss via the Cambia Foundation’s Sojourns Scholar Leadership Award.
Conflicts of Interest
None.
Ethical Standards
The study was approved by the Office of Human Subjects Research Institutional Review Board at the Johns Hopkins University (IRB00043247).
