Dutch professionals' discussion preferences with the parents of extremely premature infants varied, but the trend was towards shared decision-making.

AIM: We explored professionals' views on sharing decision-making with parents before and after an extremely preterm birth and what healthcare professionals considered severe outcomes. METHODS: A nationwide, multi-centre online survey was carried out among a wide range of perinatal healthcare professionals in the Netherlands from 4 November 2020 to 10 January 2021. The medical chairs of all nine Dutch Level III and IV perinatal centres helped to disseminate the survey link. RESULTS: We received 769 survey responses. Most respondents (53%) preferred to place equal emphasis on two treatment options during shared prenatal decision-making: early intensive care or palliative comfort care. The majority (61%) wanted to include a conditional intensive care trial as a third treatment option, but 25% disagreed. Most (78%) felt that healthcare professionals were responsible for initiating postnatal conversations to justify continuing or withdrawing neonatal intensive care if complications were associated with poor outcomes. Finally, 43% were satisfied with the current definitions of severe long-term outcomes, 41% were unsure and there were numerous for a broader definition. CONCLUSION: Although Dutch professionals expressed diverse preferences on how to reach decisions about extremely premature infants, we observed a trend towards shared decision-making with parents. These results could inform future guidelines.

Adults born prematurely prefer a periviability guideline that considers multiple prognostic factors beyond gestational age.

AIM: The aim of the study was to explore the perspectives of adults born prematurely on guidelines for management at extreme premature birth and personalisation at the limit of viability. METHODS: We conducted four 2-h online focus group interviews in the Netherlands. RESULTS: Twenty-three participants born prematurely were included in this study, ranging in age from 19 to 56 years and representing a variety of health outcomes. Participants shared their perspectives on different types of guidelines for managing extremely premature birth. They agreed that a guideline was necessary to prevent arbitrary treatment decisions and to avoid physician bias. All participants favoured a guideline that is based upon multiple prognostic factors beyond gestational age. They emphasised the importance of discretion, regardless of the type of guideline used. Discussions centred mainly on the heterogeneity of value judgements about outcomes after extreme premature birth. Participants defined personalisation as 'not just looking at numbers and statistics'. They associated personalisation mainly with information provision and decision-making. Participants stressed the importance of involving families in decision-making and taking their care needs seriously. CONCLUSION: Adults born prematurely prefer a periviability guideline that considers multiple prognostic factors and allows for discretion.

Should the Dutch Law on Euthanasia Be Expanded to Include Children?

The 2002 Dutch Euthanasia law applies to patients aged 12 years and older. Developments in end-of-life care and decision-making in the last decade have sparked the debate about usefulness and necessity to extend euthanasia to include children under 12 years of age. This paper describes two opposite positions: the affirmative position of a pediatrician and expert in pediatric palliative care and the negative position of a jurist and specialist in health law.

[Active life termination in children aged 1-12 years]. / Actieve levensbeëindiging bij kinderen van 1-12 jaar.

In this article we discuss active termination of life in children aged 1-12 years. It is expected that in 2024 active termination of life will be regulated for children between the ages of 1 and 12 who suffer unbearably, when other options to relieve their suffering are not enough. Using a case, we describe how a request for active termination of life in a child can arise and how physicians can respond to it.

Shared decision-making in pediatric palliative care in the Netherlands.

Shared decision-making (SDM) is a process in which health care professionals (HCPs) involve parents and children - when appropriate- to decide together on future treatment. These decisions are based on values that are important for the family, goals of care and preferences for future care and treatment. Elucidation of these values and preferences is preferably done early in the disease trajectory via so-called Advance Care Planning (ACP) conversations. In the Netherlands, ACP and SDM are being adopted by most health care professionals. This has happened only recently. Ten years ago, ACP and SDM were unknown concepts for the vast majority of Dutch HCPs. Today, interest in these conversational approaches is booming in both daily practice and in research. This rise has been reinforced by two recent major advancements in Dutch pediatric palliative care: the Individual Care Plan (ICP) and the Dutch Evidence-Based Guideline on Pediatric Palliative Care (DGPPC). Despite this positive evolution, a lot of work is still ahead. ACP and SDM demand a change in mindset from the traditional paternalistic approach by which the HCP 'knows what is best for this child' to a more humble and open approach in which (non-medical) factors that are important to the child and family and may influence the final treatment decision. Such changes in mindset don't happen overnight. In this article we describe the situation of pediatric palliative care in the Netherlands, with focus on the recent evolution of ACP and SDM.

The academic impact of paediatric research agendas: a descriptive analysis.

BACKGROUND: Increasingly, researchers are involving children and young people in designing paediatric research agendas, but as far as we were able to determine, only one report exists on the academic impact of such an agenda. In our opinion, the importance of insight into the impact of research agendas designed together with children and young people cannot be overstated. The first aim of our study was therefore to develop a method to describe the academic impact of paediatric research agendas. Our second aim was to describe the academic impact of research agendas developed by involving children and young people. METHODS: We based our method on aspects of the Research Impact Framework developed by Kuruvilla and colleagues and the Payback Framework developed by Donovan and Hanney. We named it Descriptive Academic Impact Analysis of Paediatric Research Agendas, consisting of five steps: [1] Identification of paediatric research agendas, [2] Citation analysis, [3] Impact analysis, [4] Author assessment, and [5] Classification of the ease of determining traceability. RESULTS: We included 31 paediatric research agendas that were designed by involving children and young people. These agendas were cited 517 times, ranging from 0 to 71 citations. A total of 131 new studies (25%) were published, ranging from 0 to 23 per paediatric research agenda, based on at least one of the research priorities from the agenda. Sixty studies (46%) were developed by at least one of the first, second, or last authors of the paediatric research agenda on which the studies were based. Based on their accessibility and the ease with which we could identify the studies as being agenda-based, we categorised 44 studies (34%) as easy, 62 studies (47%) as medium, and 25 studies (19%) as difficult to identify. CONCLUSION: This study reports on the development of a method to describe the academic impact of paediatric research agendas and it offers insight into the impact of 31 such agendas. We recommend that our results be used as a guide for designing future paediatric research agendas, especially by including ways of tracing the academic impact of new studies concerning the agendas' research priorities.

Increasingly, researchers are involving children and young people in designing paediatric research agendas. However, few researchers have described the impact of these agendas on the research undertaken. We strongly believe that it is important to know how such agendas affect research, what their impact is. One of the reasons paediatric research agendas are being designed is to create a clear overview of what the research questions are that need to be investigated - if this question is left unanswered, why bother designing the agendas at all? Therefore, we developed a 5-step tool to identify these agendas and to describe their impact. We tested our tool on 31 paediatric research agendas that were designed together with children and young people. These agendas were mentioned 517 times, 131 new studies were based on these agendas, and 60 studies were performed by the same authors who had designed the agendas. Of the new studies, we found 44 that were easy to identify, 62 that were fairly easy, and 25 that were difficult to identify as being based on paediatric research agendas. We hope that our results will serve as a useful guide for future researchers who aim to involve children and young people in designing research agendas. Especially, if ways are included to trace the impact of new studies in relation to the most important questions stated in the original research agendas.

Medical practice and legal background of decisions for severely ill newborn infants: viewpoints from seven European countries.

AIM: To comparing attitudes towards end-of-life (EOL) decisions in newborn infants between seven European countries. METHODS: One paediatrician and one lawyer from seven European countries were invited to attend a conference to discuss the practice of EOL decisions in newborn infants and the legal aspects involved. RESULTS: All paediatricians/neonatologists indicated that the best interest of the child should be the leading principle in all decisions. However, especially when discussing cases, important differences in attitude became apparent, although there are no significant differences between the involved countries with regard to national legal frameworks. CONCLUSION: Important differences in attitude towards neonatal EOL decisions between European countries exist, but they cannot be explained solely by medical or legal reasons.

Dutch guidelines on care for extremely premature infants: Navigating between personalisation and standardization.

OBJECTIVE: There is no international consensus on what type of guideline is preferred for care at the limit of viability. We aimed to conceptualize what type of guideline is preferred by Dutch healthcare professionals: 1) none; 2) gestational-age-based; 3) gestational-age-based-plus; or 4) prognosis-based via a survey instrument. Additional questions were asked to explore the grey zone and attitudes towards treatment variation. FINDING: 769 surveys were received. Most of the respondents (72.8%) preferred a gestational-age-based-plus guideline. Around 50% preferred 24+0/7 weeks gestational age as the lower limit of the grey zone, whereas 26+0/7 weeks was the most preferred upper limit. Professionals considered treatment variation acceptable when it is based upon parental values, but unacceptable when it is based upon the hospital's policy or the physician's opinion. CONCLUSION: In contrast to the current Dutch guideline, our results suggest that there is a preference to take into account individual factors besides gestational age.

The Edge of Perinatal Viability: Understanding the Dutch Position.

The current Dutch guideline on care at the edge of perinatal viability advises to consider initiation of active care to infants born from 24 weeks of gestational age on. This, only after extensive counseling of and shared decision-making with the parents of the yet unborn infant. Compared to most other European guidelines on this matter, the Dutch guideline may be thought to stand out for its relatively high age threshold of initiating active care, its gray zone spanning weeks 24 and 25 in which active management is determined by parental discretion, and a slight reluctance to provide active care in case of extreme prematurity. In this article, we explore the Dutch position more thoroughly. First, we briefly look at the previous and current Dutch guidelines. Second, we position them within the Dutch socio-cultural context. We focus on the Dutch prioritization of individual freedom, the abortion law and the perinatal threshold of viability, and a culturally embedded aversion of suffering. Lastly, we explore two possible adaptations of the Dutch guideline; i.e., to only lower the age threshold to consider the initiation of active care, or to change the type of guideline.

Facial clefts and associated limb anomalies: description of three cases and a review of the literature.

Facial clefts are rare congenital malformations. In the literature these are sometimes reported in combination with limb malformations, especially ring constrictions. This article describes three children with facial clefts and limb ring constrictions with various expressions. The first case has a lateral cleft with associated limb malformations. This combination has, to our knowledge, not yet been reported. The literature about facial clefting and the amniotic band syndrome and the possible etiology of clefting and constrictions in these cases are discussed.

Estimated incidence of sickle-cell disease in Aruba and St. Maarten suggests cost-effectiveness of a universal screening programme for St. Maarten.

OBJECTIVE: To estimate the incidence of Sickle-Cell Disease (SCD) in Aruba and St. Maarten and to determine whether universal screening would be cost-effective according to United Kingdom criteria. METHODS: Consecutive cord blood samples were collected in Aruba and the Dutch part of St. Maarten during 3 and 4 months, respectively. Samples were subjected to High Performance Liquid Chromatography (HPLC) screening of haemoglobin variants. RESULTS: Of the 368 samples (87.6% of all registered births) collected in Aruba, 10 (2.72%; CI 1.3, 4.9%) tested heterozygous for the Sickle-cell gene (HbAS) and 7 (1.90%; CI 0.8, 3.9%) for the haemoglobin C gene (HbAC). Of the 193 samples (83.5%) collected in St. Maarten, 14 (7.25%; CI 4.0, 11.9%) contained HbAS and 10 (5.18%; CI 2.5, 9.3%) HbAC. Hardy-Weinberg equilibrium predicted an incidence of 2.65% for HbAS and 1.86% for HbAC in Aruba and 6.80% for HbAS and 4.86% for HbAC in St. Maarten. These figures imply a newborn rate of about 2 SCD patients per 3 years in Aruba and 2 SCD patients per year in St. Maarten. CONCLUSIONS: Universal screening of newborns for SCD seems cost-effective for St. Maarten.

[Termination of pregnancy after the 20-week ultrasonographic examination: haste and caution]. / Zwangerschapsafbreking na de 20-wekenecho: haast en zorgvuldigheid.

The recently introduced ultrasonographic screening programme for the detection of fetal structural anomalies at 20 weeks' gestation is leading to a growing number of cases with an unclear prognosis. This article presents the decision-making process which followed the screening of two women: one aged 36 years, where a post-screening work-up was conducted and swiftly led to well-balanced decision making to abort a fetus with trisomy 21, and one woman aged 30 years, in whom repeated non-decisive results of further diagnostic tests ultimately led to a hasty decision to abort the pregnancy. Up to 24 weeks, current Dutch law allows the couple to decide to have a termination of pregnancy; thereafter the legal possibility of having a termination is very limited. This may lead to rushed decision-making. It is argued that careful decisions in these matters are more important than staying within the 24-week limit. The national central committee ofexperts which is responsible for the evaluation of all abortions after 24 weeks gestation in the so-called category 2 cases (conditions which will lead to serious and irreparable functional disorders, such as severe spina bifida and hydrocephalus, but which are compatible with life) should take account of this dilemma ofhaste and caution.

[Assessment of late pregnancy terminations, 2004-2007]. / Toetsing van late zwangerschapsafbreking, 2004-2007.

OBJECTIVE: To provide an inventory of the reported late terminations of pregnancy because ofa severe anomaly of the unborn child, i.e. termination after 24 weeks of pregnancy, in The Netherlands for the period 2004-2007. DESIGN: Inventory and descriptive. METHOD: A description is given of the various assessment procedures for the termination of pregnancy after 24 weeks. A distinction is made between abortion for lethal foetal abnormalities (category 1) and severe functional impairments with a limited chance for survival of the unborn (category 2). The level of caution exercised in decision making and performing category 1 terminations is assessed by the professional group, namely by the assessment committee for Late Pregnancy Termination of the Dutch Association for Gynaecology and Obstetrics. Since 15 March 2007, late pregnancy terminations that fall under category 2 have by law been assessed by a national central committee of experts. An overview of the reported cases of late terminations of pregnancy in the Netherlands for the period 2004-2007 is given. RESULTS AND CONCLUSION: The number of reported terminations of pregnancy after 24 weeks (n = 72) has declined considerably since the early 1990s. A possible explanation is that due to increasing technological improvements and the implementation of prenatal screening in early preg-nancy, an abortion can be performed before the 24th week of pregnancy if any severe abnormalities are observed.

[Hopeless and unbearable suffering and deliberate ending of life of newborn infants]. / Uitzichtloos en ondraaglijk lijden en actieve levensbeëindiging bij pasgeborenen.

Deliberate ending of life of newborns is an extreme measure that is usually based on hopeless and existing unbearable suffering. There are currently developments that may lead to clarification and refinement of the standards and rules surrounding deliberate ending of life of newborns. This pertains to the phase immediately following the decision to refrain from curative treatment. An important aspect here is that parents and doctors will have to reach agreement on the extent to which the suffering of the newborn can be classified as unbearable. Furthermore, in the case of deliberate ending of life of newborns, consideration must be given not only to current suffering but also the severe suffering that will develop in the near future. The points ofspecial importance that the medical profession had developed in relation to the assessment of future unbearable suffering may provide assistance here and should be implemented.

[The gestational age label]. / Het etiket van de zwangerschapsduur.

Recent follow-up data on babies born at > 24 weeks' gestation in the Netherlands has shown encouraging results; these outcomes reflect policy in national guidelines to limit treatment in babies born at < 24 weeks' gestation. The use of gestational age as a cut-off point for active treatment in extremely premature infants is, however, scientifically and ethically questionable. As an alternative we should consider a tailor-made approach by well-informed, ethically trained doctors who feel comfortable with complex decision-making, irrespective of the gestational age label.

International variations in application of the best-interest standard across the age spectrum.

OBJECTIVE: Ethically and legally, assertions that resuscitation is in a patient's best interest should be inversely correlated with willingness to forego intensive care (and accept comfort care) at the surrogate's request. Previous single country studies have demonstrated a relative devaluation of neonates when compared with other critically ill patients. STUDY DESIGN: In this international study, physicians in Argentina, Australia, Canada, Ireland, The Netherlands, Norway and the United States were presented with eight hypothetical vignettes of incompetent critically ill patients of different ages. They were asked to make assessments about best interest, respect for surrogate autonomy and to rank the patients in a triage scenario. RESULTS: In total, 2237 physicians responded (average response rate 61%). In all countries and scenarios, participants did not accept to withhold resuscitation if they estimated it was in the patient's best interest, except for scenarios involving neonates. Young children (other than neonates) were given high priority for resuscitation, regardless of existing disability. For neonates, surrogate autonomy outweighed assessment of best interest. In all countries, a 2-month-old-infant with meningitis and a multiply disabled 7-year old were resuscitated first in the triage scenario, with more variable ranking of the two neonates, which were ranked below patients with considerably worse prognosis. CONCLUSIONS: The value placed on the life of newborns is less than that expected according to predicted clinical outcomes and current legal and ethical theory relative to best interests. Value assessments on the basis of age, disability and prognosis appear to transcend culture, politics and religion in this domain.

[Trisomy 18: how lethal is 'lethal'?] / Trisomie 18: hoe lethaal is 'lethaal'?

Should active treatment be available for children with trisomy 18? In the Netherlands, trisomy 18 is described as a lethal condition leading to death during or immediately after birth. The Dutch course of action for trisomy 18 is termination of pregnancy, almost without exception, or passive treatment without medical interventions. But that approach might be outdated. We present a case that inspired physicians and parents to rethink the perception of trisomy 18.

[Insight into end-of-life decisions in newborns in Flanders, Belgium]. / Zicht op medische beslissingen rond het levenseinde bij pasgeborenen in Vlaanderen.

In the Netherlands, as in many other European countries, the majority of deaths in newborns are preceded by end-of-life decisions. In most cases, these decisions concern the withholding or withdrawing of treatment. Drugs with a potential life-shortening effect are often prescribed in the terminal phase of treatment of newborns to alleviate their suffering. The use of lethal drugs in order to deliberately end the life of newborns with a very poor prognosis and intractable severe suffering has been reported by Dutch paediatricians. Recently published data about end-of-life decisions in newborns in Flanders have shown that paediatricians in Flanders also consider the deliberate ending of life in newborns and young infants to be an acceptable option in exceptional circumstances. Real insight into the existing practice remains limited because the deliberate ending of life legally qualifies as murder in both countries. Few cases are reported because of the physician's fear of prosecution. Physicians in Flanders and in the Netherlands have pleaded for a different system of control of the deliberate ending of life in newborns. The Dutch government has recently announced the instalment of a multidisciplinary committee of experts to whom all cases must be reported. The advice of the committee to the prosecuting authorities will be crucial. It is expected that this change will increase the willingness to report cases.