Top 10 research priorities for congenital diaphragmatic hernia in Australia: James Lind Alliance Priority Setting Partnership.

OBJECTIVES: The Gaps in the Congenital Diaphragmatic Hernia (CDH) Journey Priority Setting Partnership (PSP) was developed in collaboration with CDH Australia, James Lind Alliance (JLA) and the Murdoch Children's Research Institute to identify research priorities for people with CDH, their families and healthcare workers in Australasia. DESIGN: Research PSP in accordance with the JLA standardised methodology. SETTING: Australian community and institutions caring for patients with CDH and their families. PATIENTS: CDH survivors, families of children born with CDH (including bereaved) and healthcare professionals including critical care physicians and nurses (neonatal and paediatric), obstetric, surgical, allied health professionals (physiotherapists, speech pathologists and speech therapists) and general practitioners. MAIN OUTCOME MEASURE: Top 10 research priorities for CDH. RESULTS: 377 questions, from a community-based online survey, were categorised and collated into 50 research questions. Through a further prioritisation process, 21 questions were then discussed at a prioritisation workshop where they were ranked by 21 participants (CDH survivors, parents of children born with CDH (bereaved and not) and 11 multidisciplinary healthcare professionals) into their top 10 research priorities. CONCLUSION: Stakeholders' involvement identified the top 10 CDH-related research questions, spanning from antenatal care to long-term functional outcomes, that should be prioritised for future research to maximise meaningful outcomes for people with CDH and their families.

Seeking alignment of end-of-life goals within the Australian pediatric context.

In this article, we highlight the Australian pediatric palliative care context. We describe the way in which pediatricians may adopt 'directive' shared decision-making to align care plans with their perceptions of harm avoidance for the child. The degree to which 'directive alignment' is ethically appropriate or proportionate in the context of the clinical and social context of the child must be considered. Shared decision-making within palliative care continues to be a challenging dynamic to navigate for pediatricians.

End-of-life care over four decades in a quaternary neonatal intensive care unit.

AIM: Death in the neonatal intensive care unit (NICU) commonly follows a decision to withdraw or limit life-sustaining treatment. Advances in medicine have changed the nature of life-sustaining interventions available and the potential prognosis for many newborn conditions. We aimed to assess changes in causes of death and end-of-life care over nearly four decades. METHODS: A retrospective review of infants dying in the NICU was performed (2017-2020) and compared with previous audits performed in the same centre (1985-1987 and 1999-2001). Diagnoses at death were recorded for each infant as well as their apparent prognosis and any withdrawal or limitations of medical treatment. RESULTS: In the recent epoch, there were 88 deaths out of 2084 admissions (4.2%), a reduction from the previous epochs (132/1362 (9.7%) and 111/1776 (6.2%), respectively, for epochs 1 and 2). More than 90% of infants died after withdrawal of life-sustaining treatment, an increase from the previous two epochs (75%). There was a reduction in deaths from chromosomal abnormalities, complications related to prematurity and severe birth asphyxia. CONCLUSIONS: There continue to be changes in both the diagnoses leading to death and approaches to withdrawal of treatment in the NICU. These may reflect ongoing changes in both prenatal and post-natal diagnostics as well as changing attitudes towards palliative care within the medical and wider community.

Rapid Genome Sequencing: Consent for New Technologies in the Neonatal Intensive Care Context.

The clinical utility of rapid genome sequencing (rGS) in critically unwell infants has been consistently demonstrated, and there are calls for rGS to be implemented as a first-line test in the NICU. A diagnosis from rGS can enable rapid initiation of precision treatment, making it potentially lifesaving. However, in many patients rGS leads to the diagnosis of severe and life-limiting conditions, prompting discussion with families about withdrawal of life-sustaining treatment. The complexity of information about rGS, together with the heightened emotions of parents in the NICU, poses significant challenges for informed decision making in this context. We present a case where both parents are unable to provide informed consent, and the treating team must decide whether to proceed with rGS. Our discussion highlights the important differences between genome sequencing and other types of genetic testing, and the crucial role played by pre-test counseling in facilitating informed consent and preparing parents for a range of possible outcomes. We then discuss the consent paradigms at play in NICUs; whereas admission generally comes with an understanding that the treating team will perform interventions thought to be in the best interest of the child, rGS is substantially different because of its long-term implications for patients and family members. Finally, we look at the ethical interplay between parental consent and the interests of the child. We conclude by showing how cases like this are resolved at our tertiary center and how they may be resolved differently in future.

Addressing Moral Distress: lessons Learnt from a Non-Interventional Longitudinal Study on Moral Distress.

BACKGROUND: Moral distress is prevalent within the neonatal intensive care unit (NICU) and can negatively affect clinicians. Studies have evaluated the causes of moral distress and interventions to mitigate its harmful effects. However, the effects of participating in moral distress studies have not been evaluated. OBJECTIVE: To evaluate the impact of participation in a longitudinal, non-intervention research project on moral distress in the NICU. DESIGN: Clinicians who previously participated in an 18-month longitudinal research study on moral distress at two NICUs were invited to complete a questionnaire on the impact of participation. The original study required regular completion of surveys that sought predictions of death, disability and the intensity/nature of moral distress experienced by clinicians caring for extremely preterm babies. Individual and unit-wide effects were explored. Free-text responses to open-ended questions were analyzed using inductive content analysis. RESULTS: A total of 249/463 (53%) eligible clinicians participated. Participation in the original 18-month study was perceived as having a positive impact by 58% of respondents. Clinicians found articulating their views therapeutic (76%) and useful in clarifying personal opinions about the babies (85%). Free-text responses revealed the research stimulated increased reflection, validated feelings and increased dialogue amongst clinicians. Respondents generally did not find participation distressing (70%). However, a small number of physicians felt the focus of discussion shifted from the baby to the clinicians. Intensity and prevalence of moral distress did not significantly change over the 18-month period. CONCLUSIONS: Participating in moral distress research prompted regular reflection regarding attitudes toward fragile patients, improving ethical awareness. This is useful in clarifying personal views that may influence patient care. Participation also enhanced communication around difficult clinical scenarios and improved provider satisfaction. These factors are insufficient to significantly reduce moral distress in isolation.

Whom are we seeking to protect? Extremely preterm babies and moral distress.

Advances in perinatal care bring with them ethical challenges and difficult questions. When should we provide life-sustaining interventions, and who should decide? Particularly at the edges of viability, some clinicians may feel required to provide a level of care that they believe is not in the patient's interests, resulting in moral distress. This article will discuss the complex nature of moral distress arising during the care of extremely preterm babies. It will describe the challenges and cognitive biases present when contemplating potential harms to the baby and recognize the possible costs to both healthcare provider and baby when moral distress arises. Both clinicians caring for extremely preterm babies and the families themselves can experience moral distress. This article argues that for clinicians, recognizing the range of possible sources of moral distress is vital in order to appropriately address moral distress. Moral distress may arise from a desire to protect the baby, but also from an impulse to protect oneself from the emotional burdens of care. Addressing moral distress requires reflection on the factual beliefs, experiences and personal values which lie behind the distress, both within oneself and in discussion with colleagues. Moral distress indicates that a situation is ethically challenging, but it does not necessarily mean that a wrong decision has been made.

Deferred Consent in Neonatal Clinical Research: Why, When, How?

Deferred consent has gained traction in some countries as a possible adjunct to prospective consent for evaluating emergency therapies in the neonatal population. This form of consent has been shown to increase recruitment of acutely and critically unwell patients, potentially reduce parent decision-making burden, and provide more robust evidence for clinical treatments where equipoise exists. However, deferred consent raises complex ethical concerns and guidelines for its use vary across different jurisdictions. The views of all stakeholders, including neonatal providers and parents, are important in determining the appropriateness of deferred consent in high-risk patients. Deferred consent may be ethically justifiable for assessing various treatments, particularly those used in emergency medical management. We present a framework based on neonatal deferred consent trials that assess both non-drug and drug interventions, our experience conducting deferred consent neonatal studies in Australia, and the views of providers and parents on how to best implement deferred consent in the neonatal research setting.

Moral Distress in Neonatology.

BACKGROUND AND OBJECTIVES: To longitudinally examine the nature of moral distress (MoD) experienced by clinicians caring for extremely low gestational age neonates. METHODS: Neonatologists, medical trainees, and nurses were surveyed at regular intervals on their experience of MoD and their preferred level of care in relation to 99 neonates born <28 weeks' gestational age managed from birth until discharge or death in 2 tertiary NICUs. Clinicians reporting significant distress (≥6 of 10 on Wocial's Moral Distress Thermometer) were asked to provide open-ended responses on why they experienced MoD. Descriptive statistics were used to analyze frequency and intensity of MoD across different clinician characteristics. Open-ended responses were analyzed by using mixed methods. RESULTS: Over 18 months, 4593 of 5332 surveys (86% response rate) were collected. MoD was reported on 687 (15%) survey occasions; 91% of neonates elicited MoD during their hospitalization. In their open-ended answers, clinicians invoked 5 main themes to explain their distress: (1) infant-centered reasons (83%), including illness severity, predicted outcomes, and disproportionate care; (2) management plans (26%); (3) family-centered reasons (19%); (4) parental decision-making (16%); and (5) provider-centered reasons (15%). MoD was strongly associated with the perception of "parents wanting too much." Neonatologists experienced less distress and were more likely than nurses and trainees to align preferred levels of care with family wishes. CONCLUSIONS: The majority of preterm infants will generate some MoD; however, it is rarely shared and of a sustained nature. The main constraint reported by clinicians was "parents wanting too much," leading to disproportionate care.

Interventions to reduce moral distress in clinicians working in intensive care: A systematic review.

OBJECTIVE: To evaluate the effectiveness of interventions to mitigate the harmful effects of moral distress experienced by nursing and medical clinicians working in the intensive care setting. DESIGN: Eligible studies were identified from searches of PubMed, EBSCO (Academic Search Complete, CINAHL and Medline) and Scopus. Included studies were published prior to 20 August 2020. RESULTS: Twelve studies were included in this review comprising three randomised controlled trials, seven quasi-randomised trials and two observational studies. Nine studies reported interventions targeting only nurses while three included both nurses and doctors. The types of interventions identified included: moral empowerment programs, end-of-life educational programs, reflective exercises through individual narrative writing or group reflective debriefing, multidisciplinary case debriefing meetings integrated into clinical practice and moral resiliency training. Due to the overall low methodological quality and high risk of bias, no single intervention may be considered efficacious in managing moral distress. CONCLUSIONS: There is weak evidence that some currently available interventions reduce the moral distress experienced by intensive care health care providers. Larger randomised trials involving all intensive healthcare clinicians are required to evaluate multifaceted interventions.

Does It Matter if This Baby Is 22 or 23 Weeks?

A 530-g girl born at 22 weeks and 6 days' gestation (determined by an ultrasound at 11 weeks) was admitted to the NICU. Her mother had received prenatal steroids. At 12 hours of age, she was stable on low ventilator settings. Her blood pressure was fine. Her urine output was good. After counseling, her parents voiced understanding of the risks and wanted all available life-supporting measures. Many nurses were distressed that doctors were trying to save a "22-weeker." In the past, 4 infants born at 22 weeks' gestation had been admitted to that NICU, and all had died. The attending physician on call had to deal with many sick infants and the nurses' moral distress.

Outcome of vein of Galen malformation presenting in the neonatal period.

OBJECTIVE: Vein of Galenaneurysmal malformation (VGAM) is a rare but important congenital malformation presenting to neonatal intensive care units (NICUs), and with a change from surgical to endovascular management, survival for this condition has improved. However, there is little reported about the medical management decisions of infants with this condition and the associated long-term neurodevelopmental outcomes. We aim to report a single centre experience of both acute treatment and long-term outcomes of VGAM for those infants admitted to our NICU soon after birth. DESIGN: Retrospective cohort study over a 15-year period from 2001 to 2015 inclusive. SETTING: A quaternary NICU at The Royal Children's Hospital, Melbourne, Australia. PARTICIPANTS: 24 newborn infants referred for management of VGAM. There were no eligibility criteria set for this study; all presenting infants were included. INTERVENTIONS: None. MAIN OUTCOMES MEASURES: Clinical neuroimaging data were gathered. Surviving children were formally assessed with a battery of tests administered by a neuropsychologist and occupational therapist/physiotherapist at various ages across early to middle childhood. RESULTS: Fifteen neonates with VGAM did not survive beyond their NICU admission. 10 of these were not offered endovascular intervention. Of the nine surviving infants, only one had a normal neurodevelopmental outcome. CONCLUSIONS: The mortality of VGAM presenting in the neonatal period was high, and rates of normal neurodevelopmental outcome for survivors were low. These findings contribute to our understanding of which neonates should be treated and highlights the importance of providing clinical neurodevelopmental follow-up to survivors beyond their infant years.

Providing clarity around ethical discussion: development of a neonatal intervention score.

AIM: To develop a Neonatal Intervention Score (NIS) to describe the clinical trajectory of a neonate throughout their neonatal intensive care unit (NICU) admission. METHODS: The NIS was developed by modifying the Neonatal Therapeutic Intervention Scoring System (NTISS) to reflect illness severity, dependency on life-sustaining interventions and overall life trajectory on a longitudinal basis, rather than illness burden. Validity for longitudinal use within the NICU was tested by calculating the score for 99 preterm babies born less than 28 weeks at predetermined time points throughout their admission to tertiary level care at two institutions. RESULTS: A total of 1333 NISs were analysed, ranging from 0 to 32.5 (mean 9.77, SD 5.4). Internal consistency (Cronbach alpha) reached 0.8. NIS moderately correlated to both SNAPPE-II and SNAP-II (Spearman's rho = 0.47, p =< 0.001) within the first 24 hours. CONCLUSION: The NIS is a useful and reliable descriptive tool of relative illness severity and degree of medical interventions throughout a baby's admission. Integrating a longitudinal description of medical dependency of a patient may assist both clinical and ethical decision-making and empirical research by providing an objective account of a baby's clinical trajectory. Establishment of validity within individual institutions is required.

Can the Ethical Best Practice of Shared Decision-Making lead to Moral Distress?

When healthcare professionals feel constrained from acting in a patient's best interests, moral distress ensues. The resulting negative sequelae of burnout, poor retention rates, and ultimately poor patient care are well recognized across healthcare providers. Yet an appreciation of how particular disciplines, including physicians, come to be "constrained" in their actions is still lacking. This paper will examine how the application of shared decision-making may contribute to the experience of moral distress for physicians and why such distress may go under-recognized. Appreciation of these dynamics may assist in cross-discipline sensitivity, enabling more constructive dialogue and collaboration.

Always a burden? Healthcare providers' perspectives on moral distress.

BACKGROUND: Current conceptualisations of moral distress largely portray a negative phenomenon that leads to burnout, reduced job satisfaction and poor patient care. OBJECTIVE: To explore clinical experiences, perspectives and perceptions of moral distress in neonatology. DESIGN: An anonymous questionnaire was distributed to medical and nursing providers within two tertiary level neonatal intensive care units (NICUs)-one surgical and one perinatal-seeking their understanding of the term and their experience of it. Open-ended questions were analysed using qualitative methodology. RESULTS: A total of 345 healthcare providers from two NICUs participated (80% response rate): 286 nurses and 59 medical providers. Moral distress was correctly identified as constrained moral judgement resulting in distress by 93% of participants. However, in practice the term moral distress was also used as an umbrella term to articulate different forms of distress. Moral distress was experienced by 72% of providers at least once a month. Yet despite the negative sequelae of moral distress, few (8% medical, 21% nursing providers) thought that moral distress should be eliminated from the NICU. Open-ended responses revealed that while interventions were desired to decrease the negative impacts of moral distress, moral distress was also viewed as an essential component of the caring profession that prompts robust discussion and acts as an impetus for medical decision-making. CONCLUSIONS: Moral distress remains prevalent within NICUs. While the harmful aspects of moral distress need to be mitigated, moral distress may have a positive role in advocating for and promoting the interests of the neonatal population.