Brian S. Carter

Circumstances Surrounding the Deaths of Hospitalized Children: Opportunities for Pediatric Palliative Care

Objectives. Little is known regarding the assessment and treatment of symptoms during end-of-life (EOL) care for children. This study was conducted to describe the circumstances surrounding the deaths of hospitalized terminally ill children, especially pain and symptom management by the multidisciplinary pediatric care team. Design. Patients in the neonatal intensive care unit, pediatric critical care unit, or general pediatric units of Vanderbilt Children’s Hospital who were hospitalized at the time of death, between July 1, 2000, and June 30, 2001, were identified. Children eligible for the survey had received inpatient EOL care at the hospital for at least 24 hours before death. A retrospective medical record review was completed to describe documentation of care for these children and their families during the last 72 hours of life. Results. Records of children who had received inpatient EOL care were identified (n = 105). A majority (87%) of children were in an intensive care setting at the time of death. Most deaths occurred in the pediatric critical care unit (56%), followed by the neonatal intensive care unit (31%). Pain medication was received by 90% of the children in the last 72 hours of life, and 55% received additional comfort care measures. The presence of symptoms other than pain was infrequently documented. Conclusions. The duration of hospitalization for most children dying in this inpatient setting was sufficient for provision of interdisciplinary pediatric palliative care. Management of pain and other symptoms was accomplished for many children. The documentation of pain and symptom assessment and management can be improved but requires new tools.

Pediatric Palliative Care and Hospice Care Commitments, Guidelines, and Recommendations

Pediatric palliative care and pediatric hospice care (PPC-PHC) are often essential aspects of medical care for patients who have life-threatening conditions or need end-of-life care. PPC-PHC aims to relieve suffering, improve quality of life, facilitate informed decision-making, and assist in care coordination between clinicians and across sites of care. Core commitments of PPC-PHC include being patient centered and family engaged; respecting and partnering with patients and families; pursuing care that is high quality, readily accessible, and equitable; providing care across the age spectrum and life span, integrated into the continuum of care; ensuring that all clinicians can provide basic palliative care and consult PPC-PHC specialists in a timely manner; and improving care through research and quality improvement efforts. PPC-PHC guidelines and recommendations include ensuring that all large health care organizations serving children with life-threatening conditions have dedicated interdisciplinary PPC-PHC teams, which should develop collaborative relationships between hospital- and community-based teams; that PPC-PHC be provided as integrated multimodal care and practiced as a cornerstone of patient safety and quality for patients with life-threatening conditions; that PPC-PHC teams should facilitate clear, compassionate, and forthright discussions about medical issues and the goals of care and support families, siblings, and health care staff; that PPC-PHC be part of all pediatric education and training curricula, be an active area of research and quality improvement, and exemplify the highest ethical standards; and that PPC-PHC services be supported by financial and regulatory arrangements to ensure access to high-quality PPC-PHC by all patients with life-threatening and life-shortening diseases.

Parental Perceptions of Care of Children at End of Life

Objective: Little is known regarding the perceptions of parents about end-of-life care for their children. This study describes parental perceptions of the care of hospitalized, terminally ill children in the areas of (1) clinical management, (2) interdisciplinary support, and (3) pain and symptom management. Methods: Through a mixed methods design, researchers interviewed a representative sampling of bereaved parents via telephone. Results: Data provided evidence of areas which lack satisfaction with elements of end-of-life care of hospitalized children. Conclusion: Parental satisfaction with their child’s care during end of life indicates need for improvement in pain management, communication, and parental involvement in decision making. While education cannot guarantee desired changes in attitudes or behaviors, it may provide the essential foundation of knowledge, skills, and ethical understanding needed by professional providers.

Co-occurrence of recurrent duplications of the DiGeorge syndrome region on both chromosome 22 homologues due to inherited and de novo events

Background Genomic rearrangements usually involve one of the two chromosome homologues. Homozygous microdeletion/duplication is very rare. The chromosome 22q11.2 region is prone to recurrent rearrangements due to the presence of low-copy repeats. A common 3 Mb microdeletion causes the well-characterised DiGeorge syndrome (DGS). The reciprocal duplication is associated with an extremely variable phenotype, ranging from apparently normal to learning disabilities and multiple congenital anomalies. Methods and results We describe duplications of the DGS region on both homologues in five patients from three families, detected by array CGH and confirmed by both fluorescence in situ hybridisation and single nucleotide polymorphism arrays. The proband in the first family is homozygous for the common duplication; one maternally inherited and the other a de novo duplication that was generated by nonallelic homologous recombination during spermatogenesis. The 22q11.2 duplications in the four individuals from the other two families are recurrent duplications on both homologues, one inherited from the mother and the other from the father. The phenotype in the patients with a 22q11.2 tetrasomy is similar to the features seen in duplication patients, including cognitive deficits and variable congenital defects. Conclusions Our studies that reveal phenotypic variability in patients with four copies of the 22q11.2 genomic segment, demonstrate that both inherited and de novo events can result in the generation of homozygous duplications, and further document how multiple seemingly rare events can occur in a single individual.

The Ritual: Death Pronouncement

BACKGROUND Death pronouncement comes infrequently for the pediatrician. The place of death for most children is in the hospital. For hospitalists and pediatric intensivists in the neonatal intensive care or pediatric intensive care units, death pronouncement is not uncommon. For hospitalist physicians, the call to attend a code or to pronounce a death may come on any given shift and from almost anywhere in the hospital. CONCLUSION A demonstrative case is provided, followed by key concepts for practice and for educating trainees.