Ethical considerations for cardiac surgical interventions in children with trisomy 13 and trisomy 18.

Medical and surgical approaches to children with trisomy 13 and 18 are evolving, and an increasing number of patients are being considered for simple and complex cardiac procedures. This review describes how the shifts in medical and social considerations for children with trisomy 13 and 18 mirror the shifts that occurred 50 years ago for children with trisomy 21. Yet the variability in cardiac lesions, and variability in non-cardiac comorbidities, is much greater for patients with trisomy 13 and 18 than for those with trisomy 21. That variability, combined with the severe neurologic impairment in survivors, complicates the current risk: benefit balance of surgical intervention. Consistent approaches to care for these patients should be built on an evidence base, and should include contributions from specialists in medical ethics and palliative care.

Diagnosis to death: family experiences of paediatric heart disease.

CHD remains one of the leading causes of mortality of children in the United States. There is limited research about the experience of parents from the diagnosis of their child with CHD through the death of their child. A prior study has shown that adults with heart failure go through a series of four transitions: 1) learning the diagnosis, 2) reframing the new normal, 3) taking control of the illness, and 4) understanding death is inevitable. In our qualitative study, we performed semi-structured interviews with parents who have a child die of CHD to determine whether the four transitions in adults apply to parents of children with CHD. We found that these four transitions were present in the parents we interviewed and that there were two novel transitions, one that proceeded the first Jones et al transition ("Prenatal diagnosis") and one that occurred after the final Jones et al transition ("Adjustment after death"). It is our hope that identification of these six transitions will help better support families of children with CHD.

Communication and decision-making regarding children with critical cardiac disease: a systematic review of family preferences.

Critical heart disease in the pediatric population is associated with high morbidity and mortality. Research around the most effective communication and decision-making strategies is lacking. This systematic review aims to summarise what is known about parent preference for communication and decision-making in children with critical heart disease. Database searches included key words such as family, pediatric heart disease, communication, and decision-making. A total of 10 studies fit our inclusion criteria: nine were qualitative studies with parent interviews and one study was quantitative with a parent survey. We found three main themes regarding physician-parent communication and decision-making in the context of paediatric heart disease: (1) amount, timing, and content of information provided to parents; (2) helpful physician characteristics and communication styles; and (3) reinforcing the support circle for families.

Intensity of Vasopressor Therapy and In-Hospital Mortality for Infants and Children: An Opportunity for Counseling Families.

CONTEXT: Most pediatric deaths occur in an intensive care unit, and treatment specific predictors of mortality could help clinicians and families make informed decisions. OBJECTIVE: To investigate whether the intensity of vasopressor therapy for pediatric patients, regardless of diagnosis, predicts in-hospital mortality. METHODS: Single-center, retrospective medical chart review of children aged 0-17 who were admitted between 2005 and 2015 at a pediatric tertiary care center in the U.S. and received any vasopressor medication-dopamine, dobutamine, epinephrine, vasopressin, norepinephrine, or hydrocortisone. RESULTS: During the 10-year period, 1654 patients received at least one vasopressor medication during a hospitalization. Median age at the time of hospitalization was three months, and the median duration of hospitalization was 23 days; 8% of patients had two to five hospitalizations in which they received vasopressors. There were 176 total patients who died while receiving vasopressors; most (93%) died during their first hospitalization. The most common diagnosis was sepsis (34%), followed by congenital heart disease (17%). Dopamine was the most commonly prescribed first-line vasopressor (70%), and hydrocortisone was the most commonly prescribed second-line vasopressor (49%) for all pediatric patients. The incidence of mortality rose sequentially with escalating vasopressor support, increasing from under 10% with the first vasopressor to 48% at the maximum number of agents. The odds of death almost doubled with the addition of each new vasopressor. CONCLUSIONS: The intensity of vasopressor therapy for pediatric patients, regardless of diagnosis, is associated with in-hospital mortality; vasopressor escalation should trigger intensive palliative care supports.

Lived Experience of Pediatric Home Health Care Among Families of Children With Medical Complexity.

Background. For children with complex medical conditions, pediatric home health care is a chronic need. It is a clinical service delivered entirely outside of clinical settings, granting families unparalleled expertise regarding service quality. Methods. Telephone interviews with parents whose children have extensive experiences with home health care. Results: Five themes emerged: (1) benefits of home health care include child survival and family stability; (2) family life is inextricable from home health care schedules, staffing, and services; (3) home health care gaps threaten family physical, mental, and financial well-being; (4) Out-of-pocket costs are common; and (5) families must fight for services as their children's medical conditions evolve. Conclusions. Families understand better than prescribers, providers, or policy makers what is working, and what is not, with home health care. Family expertise should be the foundation for training other families, clinicians, and home health care agencies, and should be a central component of policy and advocacy in this area.

The Pediatric Home Health Care Process: Perspectives of Prescribers, Providers, and Recipients.

BACKGROUND AND OBJECTIVES: Children with medical complexity (CMC) often require pediatric home health care (PHHC) to meet their daily intensive care needs. Pediatricians are central to planning, implementing, and maintaining quality PHHC for CMC, yet a comprehensive road map for this process is lacking. With this national study, we aim to fill that gap. METHODS: Semistructured interviews were conducted with parents and professionals from the 10 US Health Resources and Services Administration regions. Parents were recruited via advocacy groups for families of CMC; professionals with experience with PHHC for CMC were identified by using purposive and snowball sampling. Interview transcripts were qualitatively analyzed for themes. RESULTS: A comprehensive process of prescribing, providing, and maintaining PHHC requires 5 steps: identifying needs, investigating options, developing plans of care, initiating services, and navigating evolving needs. The success of the PHHC process is built on knowledge, anticipation, and early identification of needs; communication; care-coordination infrastructure; skilled home health providers; and the parent-provider relationship. CONCLUSIONS: Many CMC require PHHC to live safely outside of the hospital. Although the PHHC process involves multiple steps and participants, pediatricians' understanding of the process is the foundation of PHHC success. Fostering interagency relationships, increasing longitudinal care coordination, and investing in the PHHC infrastructure may reduce the burden placed on families and CMC as they navigate the complex process of PHHC.