Assessment of Bereaved Caregiver Experiences of Advance Care Planning for Children With Medical Complexity.

Importance: Advance care planning (ACP) is the process of discussing values and preferences for care to help inform medical decision-making. Children with medical complexity (CMC) often have a shortened life span with an unpredictable clinical course and timing of death; however, there is a paucity of literature that describes the experience of ACP from the perspective of bereaved family caregivers of CMC. Objective: To explore the experiences of bereaved family caregivers with ACP for CMC. Design, Setting, and Participants: This qualitative study included 12 interviews with 13 bereaved family caregivers of CMC whose deaths had occurred in the 5 years before study commencement (2013-2018). Participants were recruited at a single tertiary care pediatric center; CMC were treated by the Complex Care or Long-term Ventilation clinic in Toronto, Ontario, Canada. Data were collected from July to October 2018. Thematic analysis with an inductive approach was used. Exposures: Qualitative interviews were conducted using purposive sampling of bereaved family caregivers using semistructured interviews that were recorded and transcribed. Interviews were conducted until saturation was reached. Main Outcomes and Measures: Transcripts were analyzed to create themes that characterized caregiver experiences with ACP. Results: A total of 13 family caregivers were interviewed in 12 interviews, all of whom were parents (12 [92%] women, 1 [8%] man) of a deceased child (aged 7 months to 12 years). Themes were divided in the 3 following categories, which align with the Donabedian model for health service quality: (1) structure of care, (2) ACP process, and (3) end-of-life experience. Notable subthemes for this population included the importance of accounting for parental expertise in the child's care, recurrent experiences with life-threatening events, relative shock of the timing of death, and the multiple losses that caregivers experienced. Conclusions and Relevance: In this study, parental experiences revealed that there are key aspects of the structure of the child's care, process around ACP, and end-of-life care experiences that provide important reflections on ACP that warrant future study.

Adolescent decision-making in Canadian medical contexts: Integrating neuroscience and consent frameworks.

The primary objective of this commentary is to integrate current neuroscientific research on brain development during adolescence, with existing consent frameworks that do not designate a minimum age for eligibility to consent to, or refuse medical treatment. To reach this objective, the three consent frameworks used in health care settings are outlined: age-based framework; mature minor framework and capacity-based framework. This commentary draws on the Canadian health care system specifically to consider consent frameworks that grant young people with decision-making capacity. Next, a brief review of adolescent brain development findings is presented, particularly pertaining to the decision-making capacity of young people within medical contexts. Ultimately, the question of whether the stage of a young person's brain development impedes their capacity to consent to, or refuse medical treatment is addressed. This commentary provides reassurance as to the compatibility between capacity-based and mature minor frameworks to consent to treatment with current neuroscientific understanding of adolescent brain development.

Parental Moral Distress and Moral Schism in the Neonatal ICU.

Ethical dilemmas in critical care may cause healthcare practitioners to experience moral distress: incoherence between what one believes to be best and what occurs. Given that paediatric decision-making typically involves parents, we propose that parents can also experience moral distress when faced with making value-laden decisions in the neonatal intensive care unit. We propose a new concept-that parents may experience "moral schism"-a genuine uncertainty regarding a value-based decision that is accompanied by emotional distress. Schism, unlike moral distress, is not caused by barriers to making and executing a decision that is deemed to be best by the decision-makers but rather an encounter of significant internal struggle. We explore factors that appear to contribute to both moral distress and "moral schism" for parents: the degree of available support, a sense of coherence of the situation, and a sense of responsibility. We propose that moral schism is an underappreciated concept that needs to be explicated and may be more prevalent than moral distress when exploring decision-making experiences for parents. We also suggest actions of healthcare providers that may help minimize parental "moral schism" and moral distress.

Examining pediatric care for newly immigrated families: Perspectives of health care providers.

Respect for the ethnicity and cultural background of families are important components of health care delivery. However, the needs of new immigrants in pediatric care systems remain under-addressed in the literature. This study utilized a qualitative approach of interpretive description to elicit the pediatric health care experiences and needs of new immigrants to Canada, from the perspective of health care providers who worked extensively with newcomers. Two focus groups of multidisciplinary health care providers were conducted in a tertiary-level pediatric hospital. Results identify that the needs of new immigrant families are often insufficiently met by existing pediatric care practices. Needs include subsistence requirements such as income, transportation, and family understanding about the complex processes of health care in the new society. Newly immigrated families reportedly experience emotional adjustment and social support struggles and needs. Information and support to navigate the health care system are warranted. These formidable challenges invite proactive approaches, including navigation and resource finding supports as well as culturally responsive family-centered care.

Developing a bioethics curriculum for medical students from divergent geo-political regions.

BACKGROUND: The World Health Organization calls for stronger cross-cultural emphasis in medical training. Bioethics education can build such competencies as it involves the conscious exploration and application of values and principles. The International Pediatric Emergency Medicine Elective (IPEME), a novel global health elective, brings together 12 medical students from Canada and the Middle East for a 4-week, living and studying experience. It is based at a Canadian children's hospital and, since its creation in 2004, ethics has informally been part of its curriculum. Our study sought to determine the content and format of an ideal bioethics curriculum for a culturally diverse group of medical students. METHODS: We conducted semi-structured interviews with students and focus groups with faculty to examine the cultural context and ethical issues of the elective. Three areas were explored: 1) Needs Analysis - students' current understanding of bioethics, prior bioethics education and desire for a formal ethics curriculum, 2) Teaching formats - students' and faculty's preferred teaching formats, and 3) Curriculum Content - students' and faculty's preferred subjects for a curriculum. RESULTS: While only some students had received formal ethics training prior to this program, all understood that it was a necessary and desirable subject for formal training. Interactive teaching formats were the most preferred and truth-telling was considered the most important subject. CONCLUSIONS: This study helps inform good practices for ethics education. Although undertaken with a specific cohort of students engaging in a health-for-peace elective, it may be applicable to many medical education settings since diversity of student bodies is increasing world-wide.

Neonatal deaths: prospective exploration of the causes and process of end-of-life decisions.

OBJECTIVE: To determine the causes and process of death in neonates in Canada. DESIGN: Prospective observational study. SETTING: Nineteen tertiary level neonatal units in Canada. PARTICIPANTS: 942 neonatal deaths (215 full-term and 727 preterm). EXPOSURE AND OUTCOME: Explored the causes and process of death using data on: (1) the rates of withdrawal of life-sustaining treatment (WLST); (2) the reasons for raising the issue of WLST; (3) the extent of consensus with parents; (4) the consensual decision-making process both with parents and the multidisciplinary team; (5) the elements of WLST; and (6) the age at death and time between WLST and actual death. RESULTS: The main reasons for deaths in preterm infants were extreme immaturity, intraventricular haemorrhage and pulmonary causes; in full-term infants asphyxia, chromosomal anomalies and syndromic malformations. In 84% of deaths there was discussion regarding WLST. WLST was agreed to by parents with relative ease in the majority of cases. Physicians mainly offered WLST for the purpose of avoiding pain and suffering in imminent death or survival with a predicted poor quality of life. Consensus with multidisciplinary team members was relatively easily obtained. There was marked variation between centres in offering WLST for severe neurological injury in preterm (10%-86%) and severe hypoxic-ischaemic encephalopathy in full-term infants (5%-100%). CONCLUSIONS AND RELEVANCE: In Canada, the majority of physicians offered WLST to avoid pain and suffering or survival with a poor quality of life. Variation between units in offering WLST for similar diagnoses requires further exploration.

End-of-life care in Toronto neonatal intensive care units: challenges for physician trainees.

BACKGROUND: Physician trainees in neonatology can find it extremely challenging to care for patients from diverse linguistic and multicultural backgrounds. This challenge is particularly highlighted when difficult and ethically challenging end-of-life (EOL) decision-making with parents is required. While these interactions are an opportunity for growth and learning, they also have the potential to lead to misunderstanding and uncertainty and can add to trainees' insecurity, unpreparedness and stress when participating in such interactions. OBJECTIVES: To explore the challenges for trainees when EOL decisions are undertaken and to encourage them to reflect on how they might influence such decision-making. DESIGN AND INTERVIEW: An in-depth, semi-structured interview guide was developed: the interview questions address trainees' beliefs, attitudes, preferences and expectations regarding discussions of EOL neonatal care. Twelve interviews were completed and the audio records transcribed verbatim, after removal of identifying personal information. RESULTS: Participants identified six domains of challenge in EOL care: withdrawal of life-sustaining treatment based on poor outcome, explaining 'no resuscitation options' to parents, clarifying 'do not resuscitate (DNR)' orders, empowering families with knowledge and shared decision-making, dealing with different cultures and managing personal internal conflict. Participants experienced the most difficulty during the initial stages of training and eventually reported good knowledge of the EOL care process. They had a sense of security and confidence working within a multidisciplinary care team, which includes experienced nursing staff as well as bereavement and palliative care coordinator within the neonatal intensive care unit. CONCLUSIONS: The challenges experienced by physician trainees when providing EOL care can serve as focal points for improving EOL educational programmes for neonatal fellowship training.

Withdrawal of artificial nutrition and hydration in the neonatal intensive care unit: parental perspectives.

OBJECTIVE: To review the experience of the practice of withdrawal of artificial nutrition and hydration (WANH) and to describe parental perspectives on the process. DESIGN: A retrospective chart review and parental survey. SETTING: Tertiary level Neonatal Intensive Care Unit (NICU). PARTICIPANTS: Infants who had WANH after withdrawal of other life-sustaining treatment, and their parents. MAIN OUTCOME MEASURE: Parental perspectives on the care and process were obtained through a survey administered 1 to 4 years after the death of their infant. RESULTS: Fifteen cases (5.5% of all mortality and 0.5% of all admissions) of WANH were identified, and 10 parents participated in the survey. The median (range) gestational age was 40 weeks (31-42) and birth weight was 3409 g (2000-4640). The reason for WANH was predicted poor outcome due to severe neurological injury/disease. The median (range) time between WANH and death was 16 days (2-37). All parents reported favourable perceptions of preparation, support, communication and care. Seven parents reported concerns regarding pain experienced by their infant. Parents reported the ability to spend quality time, creating tangible memories and the virtues and professional qualities of the caregivers to be helpful, but identified that consistency and continuity of care could be improved. CONCLUSION: Within the spectrum of palliative care in neonates, WANH can be a tenable, justifiable and humane practice in the NICU.

Cerebral palsy and assisted conception.

Assisted reproductive technologies have been widely used over the past 30 years, and 1% to 4% of births worldwide are products of these technologies. However, adverse health outcomes related to assisted reproductive technologies, including cerebral palsy, have been reported. We extracted and reviewed all relevant studies cited by Medline from 1996 to 2010 evaluating the role of assisted reproductive technologies as a causative factor for cerebral palsy and poor long-term neurologic outcome. The research suggests that multiple pregnancy, preterm delivery, and babies small for gestational age are factors in the development of cerebral palsy. The vanishing embryo syndrome may also play a role. We review the evidence for these potentially causative factors, as well as their implications for embryo transfer policies.

A case of severe eosinophilia in a preterm infant.

Eosinophilia of prematurity is commonly seen in premature infants as a result of many causes, although a sustained or progressive form is uncommon. Different pathophysiological hypotheses include decreased circulating adrenal steroids and foreign antigen exposure. We present here a case of prolonged extreme eosinophilia in a preterm infant complicated by thrombocytopenia. The patient's eosinophil count reached 91.48 × 10(9)/L and remitted with nonspecific treatment. A comprehensive clinical, laboratory, and radiologic assessment is usually required in the context of a hypereosinophilic preterm infant to recognize the triggering etiology. Nonspecific treatment was the modality of choice because of unclear etiology in our case. Preterm infants with blood eosinophilia might not need immunomodulatory treatment despite extremely high absolute eosinophil counts.

Trends in cause-specific mortality at a Canadian outborn NICU.

OBJECTIVE: To retrospectively review changes in the causes of death of infants dying in the NICU at Canada's largest outborn pediatric center. PATIENTS AND METHODS: All inpatient deaths at the Hospital for Sick Children's NICU that occurred in the years 1997, 2002, and 2007 were retrospectively reviewed to identify the primary cause of death. Classification of the cause of death was based on a modified version of the Perinatal Society of Australia and New Zealand's Neonatal Death Classification. RESULTS: The annual mortality rate remained relatively constant (average of 7.6 deaths per 100 admissions between 1988 and 2007). A total of 156 deaths were analyzed: 53 in 1997; 50 in 2002; and 53 in 2007. The chronological age at which premature infants died increased significantly over the 3 time periods (P = .01). The proportion of deaths attributable to extreme prematurity and intraventricular hemorrhage decreased over the study period, whereas the proportion of deaths attributed to gastrointestinal causes (specifically necrotizing enterocolitis and focal intestinal perforation) increased. The proportion of infants for whom there was a decision to limit care before death was stable at between 83% and 92%. CONCLUSIONS: A larger proportion of outborn premature infants admitted to the Hospital for Sick Children's NICU seem to be surviving the early problems of prematurity only to succumb to late complications.

Pulmonary haemorrhage associated with neonatal neurological disease.

Pulmonary haemorrhage (PH) following a neurological insult is referred to as neurogenic pulmonary haemorrhage. This entity has not been described in neonates. We conducted a retrospective study of 50 neonates with PH, with and without a preceding neurological insult. Among 10/22 neonates with a preceding neurological insult, no other predisposing factor or factor associated with PH was identified. Although we speculate that in some neonates, PH may have a neurogenic origin, similar to that described in older patients, a more detailed prospective study is required. Such a prospective study requires sufficient patient numbers and clinical measures to permit a statistically powered analysis of the risk of developing a PH in those with and without neurological injury before we can confidently assert that a neurogenic injury has a causative role in PH.

Human parechovirus causes encephalitis with white matter injury in neonates.

OBJECTIVE: To assess the role of human parechoviruses (HPeVs) as a cause of neonatal cerebral infection and to report neuroimaging findings of newborn infants with encephalitis caused by HPeVs. METHODS: Clinical presentation, cranial ultrasonography, magnetic resonance imaging (MRI) findings, and neurodevelopmental outcome of 10 infants admitted to a neonatal intensive care unit and diagnosed with encephalitis caused by HPeVs are reported. RESULTS: Nine of 10 infants, with a gestational age of 29 to 41 weeks, presented at 36 to 41 weeks postmenstrual age with clinical seizures. Seven had a fever and six had a rash. Clinical presentation was similar to that of infants with enterovirus infection. Cranial ultrasonography showed increased echogenicity in the periventricular white matter in all infants. Neonatal MRI confirmed white matter changes in nine infants, which changed to gliosis on later MRI. Outcome was variable with cerebral palsy in one, a suspect outcome at 18 months in one, learning disabilities at 7 years of age in one, epilepsy in one, and normal neurodevelopmental outcome in five children. Follow-up of one infant was only 9 months. INTERPRETATION: HPeVs should be added to the list of neurotropic viruses that may cause severe central nervous system infection in the neonatal period. White matter injury can be visualized with cranial ultrasonography, but more detailed information is obtained with MRI and especially diffusion-weighted imaging. Because clinical presentation of HPeV encephalitis is similar to that of enterovirus, real-time polymerase chain reaction for both viruses should be performed in atypical presentation of neonatal seizures.

Measuring educational workload: a pilot study of paper-based and PDA tools.

BACKGROUND: Teaching is an important professional role for most faculty members in academic health sciences centres. Careful delineation of educational workload is needed to foster and reward teaching efforts, and to facilitate equitable allocation of resources. AIMS: To promote recognition in teaching and facilitate equitable resource allocation, we developed, piloted, and qualitatively assessed a tool for delineating the educational workload of pediatric faculty in an academic health sciences centre. METHODS: A prototype educational workload measurement tool was developed. Between 2002 and 2004, three successive phases of pilot implementation were conducted to (1) assess the face validity of the tool, (2) assess its feasibility, and (3) develop and assess the feasibility of a PDA (Personal Digital Assistant) version. Participants were interviewed regarding strengths, weaknesses, and barriers to completion. Data were analyzed for recurrent themes. RESULTS: Faculty found that the tool was usable and represented a broad range of educational activities. The PDA format was easier to use and better received. Technical support would be imperative for long-term implementation. The greatest barriers to implementation were skepticism about the purpose of the tool and concerns that it would promote quantity over quality of teaching. CONCLUSION: We developed a usable tool to capture data on the diverse educational workload of pediatric faculty. PDA technology can be used to facilitate collection of workload data. Faculty skepticism is an important barrier that should be addressed in future work.

Portal vein thrombosis in the neonate: risk factors, course, and outcome.

OBJECTIVE: To determine the risk factors, clinical features, and outcome of infants diagnosed with portal vein thrombosis (PVT). STUDY DESIGN: A retrospective chart review was conducted of all consecutive infants admitted to the Hospital for Sick Children, Toronto, between January 1999 and December 2003 diagnosed with PVT. RESULTS: PVT was diagnosed in 133 infants, all but 5 of whom were neonates, with a median age at time of diagnosis of 7 days. An umbilical venous catheter (UVC) was inserted in 73% of the infants and was in an appropriate position in 46% of them. Poor outcome, defined as portal hypertension or lobar atrophy, was diagnosed in 27% of the infants and was significantly more common in those with an initial diagnosis of grade 3 PVT and in those with a low or intrahepatically placed UVC. Anticoagulation treatment did not appear to have a significant effect on outcome. CONCLUSIONS: PVT occurs early in life; major risk factors in addition to the neonatal period are placement of UVC and severe neonatal sickness. Poor outcome is associated with an improperly placed UVC and with grade 3 thrombus.

Necrotizing enterocolitis: assessment of bowel viability with color doppler US.

PURPOSE: To determine whether absence of bowel wall perfusion at color Doppler ultrasonography (US) is indicative of bowel necrosis in neonates with necrotizing enterocolitis (NEC). MATERIALS AND METHODS: This study was approved by the research ethics board, and informed consent was obtained. Sixty-two neonates enrolled in the prospective study underwent US of the bowel wall. Neonates were divided into two groups. Group A included 30 control subjects with gestational ages (GAs) ranging from 24 to 41 weeks. Group B included 32 neonates with GAs ranging from 24 to 40 weeks who were clinically proved to have or suspected of having NEC. All neonates in group B underwent abdominal radiography. Normative values were calculated in group A. In group B, the sensitivities and specificities of color Doppler US and abdominal radiography for detection of bowel necrosis were computed by using the modified Bell staging criteria for NEC as the reference standard. RESULTS: Two neonates were excluded from group B; thus, a total of 60 neonates were included in the study. In group A, bowel wall thickness ranged from 1.1 to 2.6 mm. Bowel wall perfusion was detected with color Doppler US in all 30 neonates. Color Doppler signals ranged from one to nine dots per square centimeter. Twenty-two of 30 neonates in group B received a diagnosis of NEC. Mild to moderate NEC was diagnosed in 12 neonates. Color Doppler US depicted an isolated segment of bowel-absent blood flow in two neonates; this finding was confirmed with laparotomy. In 10 neonates with severe NEC, color Doppler US depicted isolated or multiple segments of bowel with absent perfusion. Pneumoperitoneum was present in only four neonates. The remaining eight neonates at risk for NEC had no evidence of loops without perfusion at color Doppler US. The sensitivity of free air at abdominal radiography as a positive sign for severe NEC with necrotic bowel was 40% compared with the 100% sensitivity of absence of flow at color Doppler US (P = .03). CONCLUSION: Color Doppler US is more accurate than abdominal radiography in depicting bowel necrosis in NEC.

Unusual neonatal presentation of incontinentia pigmenti with persistent pulmonary hypertension of the newborn: a case report.

Incontinentia pigmenti (Bloch-Sulzberger syndrome) is a multisystem disorder with classical changing skin lesions. The other systems that are involved include the central nervous system, eye, hair, teeth, musculoskeletal system and, occasionally, the cardiovascular system. We report a neonate with a diagnosis of incontinentia pigmenti who presented at birth with pulmonary hypertension. This presentation has not been described in the literature.

Nosocomial transmission of congenital tuberculosis in a neonatal intensive care unit.

Congenital tuberculosis is uncommon, and nosocomial transmission from a congenitally infected infant to another infant has not been reported in the English literature. We report an investigation of 2 infants with tuberculosis who were cared for in the same neonatal intensive care unit. Isolates from both infants were genetically indistinguishable. Transmission between the 2 infants was likely due to contaminated respiratory equipment.

Clinical characteristics and follow up of Down syndrome infants without congenital heart disease who presented with persistent pulmonary hypertension of newborn.

We identified seventeen infants with Down syndrome without structural congenital heart disease who presented with persistent pulmonary hypertension in the newborn period. Respiratory distress with or without hypoxia was the presenting feature in these infants. Pulmonary hypertension resolved in the majority of the survivors. Two infants with refractory pulmonary hypertension benefited from patent ductus arteriosus ligation. Autopsies in two infants demonstrated structural lung immaturity. We suggest that infants with Down syndrome are at risk of developing persistent pulmonary hypertension even in the absence of structural heart disease and these infants should be followed up until resolution of the pulmonary hypertension.