Paediatrics and discomfort with death and dying.

Death and dying, while uncommon in day-to-day paediatrics practice, are becoming increasingly common occurrences as children with life-limiting illnesses are living longer. We reflect on our experiences with death and dying in our residency training and whether paediatrics, as a specialty, is uncomfortable with death. Paediatric trainees should be included in honest discussions about disease trajectories and participate in providing end-of-life care. Anticipatory guidance helps personalize care and can prevent unnecessary procedures or suffering that patients may experience. While trainees may not be present at the end-of-life for many patients, managing death and dying are important competencies for future paediatricians. Current paediatricians should reflect on their comfort with death and how this may impact their patient care.

Case report: Foreign body aspiration requiring extracorporeal membrane oxygenation.

Introduction: Foreign body aspiration is a common cause of respiratory distress in pediatrics, but the diagnosis can be challenging given aspirated objects are mostly radiolucent on chest radiographs and there is often no witnessed choking event. We present a case of a patient who was initially managed as severe status asthmaticus, requiring veno-arterial extracorporeal membrane oxygenation (VA-ECMO) for refractory hypercarbia and hypoxemia, but was later found to have bilateral bronchial foreign body aspiration. This case is unique in its severity of illness, diagnostic dilemma with findings suggesting a more common diagnosis of asthma, and use of ECMO as a bridge to diagnosis and recovery. Patient case: A previously healthy 2-year-old boy presented during peak viral season with a 3-day history of fever, cough, coryza, and increased work of breathing over the prior 24 h. There was no reported history of choking or aspiration. He was diagnosed with asthma and treated with bronchodilator therapy. Physical examination revealed pulsus paradoxus, severe work of breathing with bilateral wheeze, and at times a silent chest. Chest radiographs showed bilateral lung hyperinflation. Following a brief period of stability on maximum bronchodilator therapies and bilevel positive pressure support, the patient had a rapid deterioration requiring endotracheal intubation, with subsequent cannulation to VA-ECMO. A diagnostic flexible bronchoscopy was performed and demonstrated bilateral foreign bodies, peanuts, in the right bronchus intermedius and the left mainstem bronchus. Removal of the foreign bodies was done by rigid bronchoscopy facilitating rapid wean from VA-ECMO and decannulation within 24 h of foreign body removal. Conclusion: Foreign body aspiration should be suspected in all patients presenting with atypical history and physical examination findings, or in patients with suspected common diagnoses who do not progress as expected or deteriorate after a period of stability. Extracorporeal life support can be used as a bridge to diagnosis and recovery in patients with hemodynamic or respiratory instability.

Heterogenous Disease Course and Long-Term Outcome of Children's Interstitial Lung Disease Related to Filamin A Gene Variants.

Rationale: Variable disease course and outcomes have been reported in children's interstitial lung disease associated with FLNA (Filamin A gene) variants. Objectives: To further delineate long-term respiratory outcomes and identify potential contributing factors to severe disease course. Methods: We retrospectively collected longitudinal data from three centers on nine cases (one male) with FLNA variants and early respiratory disease onset (within the first 24 mo of life). Clinical, radiographic, and histopathologic data were analyzed, focusing on cardiorespiratory disease course. Results: All required early respiratory support (three invasive ventilation, three noninvasive ventilation, three supplemental oxygen), and all experienced frequent severe infective respiratory exacerbations. Three died in infancy from refractory respiratory failure and pulmonary hypertension (PH). The six surviving individuals were 3, 10, 11, 15, 18, and 33 years old at time of reporting. The extent of functional respiratory impairment decreased with age; at last follow-up, there were no individuals on home invasive ventilation, one on nocturnal noninvasive ventilation, four on oxygen, and one on no respiratory support. Spirometry consistently demonstrated moderate to severe obstructive defects (forced expiratory volume in 1 s/forced vital capacity [FVC] z-score, -3.76 to -1.77; percent predicted FVC, 31.5% to 92.1%). Seven required PH treatment in early childhood (7/9), and three of the survivors (3/6) still receive treatment. Radiologic and histopathologic findings were consistent among cases. Conclusions: Early mortality was common, but many survivors stabilized even after severe symptoms in infancy. All survivors had persistent obstructive defects on spirometry, and half have persistent or recurrent PH. These typical findings are suggestive of this rare diagnosis and should prompt consideration of genetic testing.

Characteristics and Timing of Mortality in Children Dying With Infections in North American PICUs.

OBJECTIVES: To investigate the characteristics and timing of death of children with severe infections who die during PICU admission. DESIGN: We analyzed demographics, timing of death, diagnoses, and common procedures in a large cohort obtained from the Virtual Pediatrics Systems database, focusing on early deaths (< 1 d). SETTING: Clinical records were prospectively collected in 130 PICUs across North America. PATIENTS: Children admitted between January 2009 and December 2014 with at least one infection-related diagnosis at time of death. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Analysis included data from 106,464 children admitted to PICUs. The 4,240 children (4%) who died were older than PICU survivors. The median (interquartile range) duration in PICU prior to death was 7.1 days (2.1-21.3 d), with 635 children (15%) dying early (< 1 d of PICU admission). Children who died early were older, more likely to have septic shock, and more likely to have received cardiopulmonary resuscitation than those who died later. Withdrawal of care was less likely in early deaths compared with later deaths. After adjusting for age, sex, sepsis severity, procedures (including cardiopulmonary resuscitation and heart, lung, and renal support), and number of admissions contributed per PICU, it was found that children admitted from the emergency department, inpatient floors, or referring hospitals had significantly greater risk of early death compared with children admitted from the operating room. CONCLUSIONS: A substantial proportion of children admitted to PICU with severe infections die early and differ from those dying later in diagnoses, procedures, and admitting location. The emergency department is a key source of critically ill patients. Understanding characteristics of early deaths may yield recruitment considerations for clinical trials enrolling children at high risk of early death.

Clinician-Driven Design of VitalPAD-An Intelligent Monitoring and Communication Device to Improve Patient Safety in the Intensive Care Unit.

The pediatric intensive care unit (ICU) is a complex environment, in which a multidisciplinary team of clinicians (registered nurses, respiratory therapists, and physicians) continually observe and evaluate patient information. Data are provided by multiple, and often physically separated sources, cognitive workload is high, and team communication can be challenging. Our aim is to combine information from multiple monitoring and therapeutic devices in a mobile application, the VitalPAD, to improve the efficiency of clinical decision-making, communication, and thereby patient safety. We observed individual ICU clinicians, multidisciplinary rounds, and handover procedures for 54 h to identify data needs, workflow, and existing cognitive aid use and limitations. A prototype was developed using an iterative participatory design approach; usability testing, including general and task-specific feedback, was obtained from 15 clinicians. Features included map overviews of the ICU showing clinician assignment, patient status, and respiratory support; patient vital signs; a photo-documentation option for arterial blood gas results; and team communication and reminder functions. Clinicians reported the prototype to be an intuitive display of vital parameters and relevant alerts and reminders, as well as a user-friendly communication tool. Future work includes implementation of a prototype, which will be evaluated under simulation and real-world conditions, with the aim of providing ICU staff with a monitoring device that will improve their daily work, communication, and decision-making capacity. Mobile monitoring of vital signs and therapy parameters might help improve patient safety in wards with single-patient rooms and likely has applications in many acute and critical care settings.