Symptoms and Suffering at End of Life for Children With Complex Chronic Conditions.

CONTEXT: Children with cancer and cardiac disease suffer with high symptom burden at end of life (EOL). Little is known about the EOL experience for children with other complex chronic conditions (CCCs). OBJECTIVES: To evaluate symptoms and suffering at EOL for children with noncancer, noncardiac CCCs as well as parental distress related to child suffering. METHODS: This study is a secondary data analysis of a cross-sectional, single-center survey of bereaved parents of children with CCCs who died between 2006 to 2015. The primary outcome was parent-reported child suffering in the final two days of life. RESULTS: Among 211 eligible parents contacted for participation, 114 completed the survey, and 99 had complete primary outcome data (participation rate 47%). Most children had congenital/chromosomal (42%) or progressive central nervous system (22%) conditions. Twenty-eight percent of parents reported high child suffering in the final two days of life. Parents reported that pain and difficulty breathing caused the greatest suffering for children and distress among themselves. Some parents also reported distress related to uncertainty about child suffering. Parents were less likely to report high child suffering if they were confident in knowing what to expect when their child was dying (AOR 0.20; 95% CI 0.07-0.60) or felt prepared for medical problems at EOL (AOR 0.12; 95% CI 0.04-0.42). CONCLUSION: Nearly one-third of parents of children with CCCs report high suffering in their child's final days of life. Parent preparedness was associated with lower perceived child suffering. Future research should target symptoms contributing to parent and child distress and assess whether enhancing parent preparedness reduces perceived child suffering.

Impact of Specialized Pediatric Palliative Care: A Systematic Review.

CONTEXT: Specialized pediatric palliative care (SPPC) is increasingly involved in the care of seriously ill children, yet the evidence on its impact has not been comprehensively reviewed. OBJECTIVE: The objective of this study was to assess the effects of providing SPPC to seriously ill children on patient-, caregiver-, and systems-level outcomes. METHODS: We performed a Systematic Review following Cochrane methods. DATA SOURCES: Medline, Embase, PsycINFO, Global Health, The Cochrane Central Register of Controlled Trials, LILACS, and Web of Science were searched from January 1996 to June 2018. STUDY SELECTION/DATA EXTRACTION: We included randomized controlled, cohort, case-control, and before-after studies in which exposure to SPPC services was the intervention of interest. All outcomes reported in these studies were included. Two investigators independently selected articles, extracted data, and assessed risk of bias of included studies using standardized criteria. RESULTS: Twenty-four studies were included in qualitative synthesis: one nonrandomized controlled trial, 16 cohort studies, and seven before-after studies. Evidence certainty was low. Twenty-one studies had one or more area with high risk of bias, most commonly selection bias, low group comparability, risk for confounding, and inadequate statistical reporting. Studies analyzed 46 domains, operationalized as 136 distinct outcomes. SPPC was associated with better child quality of life scores in all four studies that assessed this outcome. No other outcome showed this consistency. CONCLUSION: Receiving SPPC was associated with better child quality of life. However, the paucity and low certainty of the evidence precluded any firm recommendations about SPPC practice. Larger collaborative networks and greater consensus regarding SPPC research standards are needed.

Patterns and Outcomes of Care in Children With Advanced Heart Disease Receiving Palliative Care Consultation.

CONTEXT: Although access to subspecialty pediatric palliative care (PPC) is increasing, little is known about the role of PPC for children with advanced heart disease (AHD). OBJECTIVES: The objective of this study was to examine features of subspecialty PPC involvement for children with AHD. METHODS: This is a retrospective single-institution medical record review of patients with a primary diagnosis of AHD for whom the PPC team was initially consulted between 2011 and 2016. RESULTS: Among 201 patients, 87% had congenital/structural heart disease, the remainder having acquired/nonstructural heart disease. Median age at initial PPC consultation was 7.7 months (range 1 day-28.8 years). Of the 92 patients who were alive at data collection, 73% had received initial consultation over one year before. Most common indications for consultation were goals of care (80%) and psychosocial support (54%). At initial consultation, most families (67%) expressed that their primary goal was for their child to live as long and as comfortably as possible. Among deceased patients (n = 109), median time from initial consultation to death was 33 days (range 1 day-3.6 years), and children whose families expressed that their primary goal was for their child to live as comfortably as possible were less likely to die in the intensive care unit (P = 0.03) and more likely to die in the setting of comfort care or withdrawal of life-sustaining interventions (P = 0.008). CONCLUSION: PPC involvement for children with AHD focuses on goals of care and psychosocial support. Findings suggest that PPC involvement at end of life supports goal-concordant care. Further research is needed to clarify the impact of PPC on patient outcomes.

Chronic Critical Illness in Infants and Children: A Speculative Synthesis on Adapting ICU Care to Meet the Needs of Long-Stay Patients.

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.

Long-term outcomes in borderline psychopathology: old assumptions, current findings, and new directions.

Borderline personality disorder (BPD) and historical variants of the diagnosis were long held to represent an intractable syndrome of psychopathology consisting of interpersonal, intrapsychic, and affective disturbances. For years, patients labeled "borderline" were regarded pejoratively due at least in part to the lack of effective treatments. Prospective data from recent naturalistic follow-along studies along with the development of treatments with empirically demonstrated efficacy have changed how BPD is viewed. It is now less common to hide the diagnosis from the patient, and BPD has become a useful label to guide the treatment process and help the patient make sense of his or her suffering. Although it is now accepted that BPD is a treatment-responsive disorder and that remission is the norm, more work is needed to help patients achieve a higher level of functioning, and targeting persistent trait-like features suggests new directions for future efforts in treatment development.