A Dutch clinical practice guideline for paediatric palliative care: a systematic review and recommendations on psychosocial care including preloss and bereavement care

Abstract

Background

Children with life-threatening and life-limiting conditions and their families are confronted with many uncertainties, incremental losses, and difficult decisions. Attentiveness to psychosocial care, including psychological, social, and spiritual concerns, is essential during the child’s life until end-of-life and during bereavement and determines quality of life and wellbeing of child and family. However, use and integration of psychosocial care is found to be challenging. In this paper, we provide recommendations that aim to optimise psychosocial care including preloss and bereavement care in paediatric palliative care.

Methods

A multidisciplinary guideline development panel of 25 experts in paediatric palliative care and nine parents was established to develop recommendations on psychosocial care including preloss and bereavement care. We performed a systematic literature search to identify evidence focussing on effectivity of interventions described in systematic reviews (SRs) of randomised controlled trials (RCTs) and RCTs. From the search, we also derived evidence from SRs of observational, qualitative, or mixed-method studies on preloss and bereavement care. The GRADE methodology was used for appraising evidence. We used existing guidelines to deal with lack of evidence. Recommendations were formulated based on evidence, existing guidelines, clinical expertise, and patient and family values.

Results

We identified a total two SRs of RCTs and six RCTs that reported on the effect of psychological interventions, spiritual support interventions, and bereavement care intervention. The evidence showed that only some of the interventions improve certain psychosocial outcomes in children or parents. We were unable to draw conclusions on which intervention is most effective, due to the high variability in interventions and measured outcomes. The evidence from ten SRs of observational, qualitative, or mixed-method studies was used as a basis for preloss and bereavement care recommendations. In total, we formulated 48 recommendations.

Discussion

With the provided recommendations, we aim to optimise psychosocial care for children with life-threatening and life-limiting conditions and their families. Psychosocial care should be tailored to the child and family. We found a lack of evidence on psychosocial care including preloss and bereavement care. Although very challenging, we propose implementing strategies to improve research and thereby further optimise quality of paediatric palliative care. These strategies include the development of a standard set of outcomes and to conduct well-designed multisite RCTs.

Background

Children with life-threatening and life-limiting conditions often receive high complex care which lasts for months or years [1,2,3,4,5]. During the child’s disease trajectory, children and their families are confronted with many uncertainties, incremental losses, and difficult decisions [1, 4]. These children and their families can experience high levels of suffering and are at risk for poor quality of life [6,7,8]. Paediatric palliative care focuses on relieving physical, psychological, social, and spiritual suffering and ensuring the best quality of life for these children and their families from diagnosis until end-of-life and during bereavement [4, 5, 9].

Multiple studies highlighted a need for improvement of paediatric palliative care such as symptom management to ease distressing symptoms and enhance quality of life in both children and their families [6, 7, 10, 11]. Moreover, it is increasingly recognised that not only symptom alleviation, but also attentiveness to psychological, social, and spiritual concerns is essential in paediatric palliative care [12,13,14,15], as this determines quality of life and experienced distress among children and their family members. Furthermore, children and their families experience incremental losses from the moment of diagnosis, throughout the palliative care trajectory, and even new losses after the child’s death. Attentiveness to loss, grief, and bereavement is crucial to support children and their families in coping with these losses [16,17,18].

Psychosocial care, which includes the attentiveness to psychological, social, and spiritual concerns, and preloss and bereavement care, is an essential component of paediatric palliative care and provides a meaningful opportunity to enhance the wellbeing of the child and the whole family [12, 18, 19]. However, the use and integration of psychosocial care including preloss and bereavement care has been found to be challenging [18, 19]. A recent qualitative study among parents in the Netherlands reported that paediatric palliative care still focuses too much on symptom management [20]. The study highlights the importance to see the child as a person and focus on the child and family as a whole, including psychological, social, and spiritual aspects. Additionally, parents experienced a lack of preloss and bereavement care during the child’s palliative disease trajectory and after the death of their child [20].

Psychosocial care including preloss and bereavement care should be ensured for all children with life-threatening and life-limiting conditions and their families. Clinical practice guidelines (CPGs) are found to be powerful tools in which knowledge from scientific literature, clinical expertise and patient experiences are combined to provide recommendations for clinical practice [21,22,23]. Care delivered in line with these recommendations can decrease variability in provided care, enhance quality of care, and contribute to integration of care services [21,22,23].

To achieve more optimal psychosocial paediatric palliative care, we aim to provide new evidence-based recommendations focusing on interventions for psychosocial care in which we distinguish psychological interventions, practical and social support, cultural, spiritual, and religious support, and preloss and bereavement care. These recommendations have been developed as part of the revised Dutch CPG on paediatric palliative care [24]. In this paper, we present an overview of the evidence, clinical expertise, and recommendations on psychosocial care.

Methods

The full methodology of the Dutch CPG for paediatric palliative care is outlined in another publication [24].

Scope

This guideline aims to improve quality of palliative care for all children aged 0 to 18 years with life-threatening and life-limiting conditions and their caregivers and siblings (hereafter referred to as families), with the ultimate goal to improve quality of life of children and their families. The guideline covers the entire palliative disease trajectory (from the beginning of the palliative disease trajectory till after the child’s death continuing in bereavement) [24].

In this paper, we aim to provide recommendations for psychosocial care including psychological interventions, practical and social support, cultural, spiritual, and religious support, and preloss and bereavement care.

Multidisciplinary guideline development panel

The guideline development panel included 25 paediatric palliative care experts and a group of nine parents of children with life-threatening and life-limiting conditions (Appendix A). These experts, of which some had specialised certified training in paediatric palliative care, represented various disciplines such as paediatricians, psychotherapists, psychologists, spiritual counsellors, child life specialists, and paediatric nurses. To maintain consistency throughout the guideline, a core group of 11 experts was formed within the panel. The remaining 14 experts were assigned to two working groups (WGs), one dedicated to psychosocial care, and the other to preloss and bereavement care. The psychosocial care WG tackled three topics for which sub-WGs were established, namely psychological interventions, practical and social support, and cultural, spiritual, and religious support. All topics and clinical questions addressed by the (sub)WGs were prioritised based on input from experts and parents [24]. Appendices B and C provide an overview of the working structure and guideline development process.

Representation of patients and their families

To ensure representation of patients and their families, various methods were utilised [24]. First, we gave two members of the core group the specific responsibility of maintaining patient and family representation throughout the guideline development process. Another method involved forming a group of nine parents of children with life-threatening or life-limiting conditions. This group reviewed the initial drafts of all guideline texts and recommendations as well as the draft of the complete guideline. To capture a wide range of experiences, we included parents of children with diverse palliative conditions, ages, and stages of disease. Some of the children with life-threatening or life-limiting conditions were actively receiving palliative care, while others had passed away.

Formulation of clinical questions

The psychosocial care WG formulated four clinical questions on the effect of psychological interventions, practical and social support, and cultural, spiritual, and religious support for children with life-threatening and life-limiting conditions and their family members. The WG that focused on preloss, and bereavement care formulated four clinical questions on the effect of preloss interventions for children with life-threatening and life-limiting conditions and their family members and the effect of bereavement care interventions for their family members, components of preloss and bereavement care interventions, experiences and needs of parents and health care providers, and communicative strategies (Appendix D).

Identification of evidence

To answer clinical questions on the effect of psychosocial care interventions including psychological interventions, practical and social support, cultural, spiritual, and religious support, and preloss and bereavement care interventions, we updated the systematic literature search conducted for the former CPG (2013) which focused on quantitative studies on paediatric palliative care interventions (last updated January 24, 2020) (Appendix E) [24]. Studies were selected based on specific inclusion criteria related to study population (children aged 0 to 18 with life-threatening or life-limiting conditions as defined by the World Health Organisation [5]); study design (randomised controlled trials (RCTs), controlled clinical trials (CCTs), and systematic reviews (SRs) of RCTs and CCTs; and study subject (paediatric palliative care psychosocial care interventions including psychological interventions, practical and social support, cultural, spiritual, and religious support, and preloss and bereavement care). Only studies published in English or Dutch were included (Appendix F) [24]. Included studies were summarised in evidence tables and categorised by outcome measures in summary of findings tables [24]. We formulated conclusions of evidence for each outcome measure and assessed the quality of the total body of evidence using the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) method [25]. The conclusions of evidence were used as a basis for our recommendations [24].

Furthermore, to answer clinical questions on components of preloss and bereavement care interventions, experiences and needs of parents and health care providers, and communicative strategies, we derived SRs of observational, qualitative, or mixed-method studies from the systematic literature search on paediatric palliative care interventions and through referencing [24]. We synthesised the findings from these SRs in evidence tables and translated these into conclusions of evidence. Given the variety of study designs included in the SRs, determining both individual study quality and quality of the total body of evidence were not feasible [24]. The conclusions derived from the SRs served as the basis for our recommendations [24].

Identification of additional literature

As we expected that evidence would be limited or of low quality, we conducted a search for existing evidence-based paediatric palliative care guidelines that had performed systematic literature searches on this topic. We found one evidence-based guideline, ‘End of life care for infants, children and young people with life-limiting conditions (2016)’ of the National Institute for Health and Care Excellence (NICE) [26]. We used the conclusions of evidence, considerations, and recommendations of the NICE-guideline to inform and support our own recommendations [24].

Translating evidence into recommendations

Multiple factors were considered in translating the evidence into recommendations. First, the WG members considered the quality of the evidence when formulating recommendations, in which a higher quality of evidence was likely to support stronger recommendations. If the evidence focused on one specific group of children (e.g. children with cancer), the WG carefully assessed if evidence could be extrapolated to children with other life-threatening and life-limiting conditions before formulating recommendations. Other factors that were considered to formulate recommendations were, additional literature including existing evidence-based guidelines, values and needs of patients and their family, clinical expertise, acceptability (legal and ethical aspects), feasibility (available time, knowledge and manpower), and benefits versus harms of the interventions [24]. The WG members outlined the relevant considerations for each clinical question, and decisions were made through group consensus [24].

The strength of each recommendation was graded using established evidence-based methods [25, 27] (Appendix G). Recommendations were classified as strong to do (green), moderate to do (yellow), or strong not to do (red) [24].

Results

Identification of evidence

The systematic search for quantitative studies on paediatric palliative care interventions resulted in 5078 citations of which 168 citations were subjected to full-text screening [24]. Ultimately, eight studies (two SRs of RCTs and six RCTs) on interventions related to psychosocial care including preloss and bereavement care were included [28,29,30,31,32,33,34,35] (Appendix H). Additionally, ten SRs of observational, qualitative, or mixed-method studies on preloss and bereavement care interventions components, and communicative strategies were included [36,37,38,39,40,41,42,43,44,45] (Appendix H).

Appendices J and K present the evidence tables and summary of findings tables. Tables 1, 2, and 3 provide the conclusions of evidence. Figure 1 offers an overview of the identified evidence and recommendations for each WG.

Table 1 Conclusions of evidence on paediatric palliative psychosocial care interventions including preloss and bereavement care interventions

Table 2 Conclusions of evidence on preloss and bereavement care intervention components

Table 3 Conclusions of evidence on communicative strategies during the child’s end-of-life and after death

Fig. 1

Identified evidence and recommendations per working group and clinical question

Evidence on effect of psychological interventions

Three studies, two RCTs and one SR of RCTs, reported on psychological interventions that were targeted at improving a variety of psychosocial outcomes in children and adolescents with life-threatening or life-limiting conditions [28, 29, 31]. Two RCTs reported on the effects of the Promoting Resilience In Stress Management (PRISM) intervention which aimed to strengthen four resources of resilience including stress-management, goal-setting, cognitive reframing, and meaning-making in four 30–50 min sessions amongst children and adolescents with cancer [28, 29]. The studies showed that hope-finding was significantly increased in children and adolescents with cancer who received the PRISM intervention as compared to the children and adolescents who received usual psychosocial care provided by a social worker (very low quality evidence) [28]. However, we found no significant effect of benefit-finding (very low quality evidence) [28]. Additionally, no significant difference in percentage of positive health-related quality of life (HR-QoL) trajectories was found between children and adolescents with cancer who participated in the PRISM intervention and those who received usual care (very low quality evidence) [29]. When looking more closely at cancer-specific subdomains of HR-QoL, the percentage of positive trajectories in HR-QoL was only significantly increased in the intervention group in two out of eight cancer-specific subdomains, namely worry and nausea (very low quality evidence) [29]. One SR of RCTs reported on the effects of an educational problem-solving and social skills intervention for children with cystic fibrosis [31]. This intervention showed no significant difference in psychosocial outcomes including perceived loneliness and social support at three, six, and nine month follow-up in children that received the interventions as compared to usual care (moderate quality evidence) [31].

Three studies, one RCT and two SRs of RCTs, reported on psychological interventions that were targeted at improving several psychosocial outcomes in parents of children with life-threatening or life-limiting conditions [30,31,32]. One RCT investigated the effects of an adapted version of the PRISM intervention directed at parents (PRISM-P) comprising of four 30 to 60 min sessions. The PRISM-P intervention increased resilience and benefit-finding at six month follow-up among parents who received one on one sessions as compared to usual psychosocial care provided by a social worker (very low quality evidence) [30]. No significant effect was found on other outcomes including hope-finding, perceived social support, HR-QoL, perceived stress and psychological distress (very low quality evidence) [30]. When the PRISM-P intervention was delivered in group sessions of two to five parents, no significant effect was found in any of the assessed outcomes (very low quality evidence) [30]. Additionally, one SR of RCTs reported on the effect of a community-based support programme for mothers of children with cystic fibrosis in comparison to a control intervention consisting of contact with a telephone number. No significant difference in anxiety at 12 month follow-up was found among mothers who received the intervention and those who were in the control group (very low quality evidence) [31]. Finally, one SR of RCTs reported on the effect of different psychological interventions for parents of children with cancer including cognitive behavioural therapy, family therapy, problem-solving therapy and multi-systemic therapy in comparison to different control interventions including treatment as usual, active control, or wait-list control [32]. The psychological interventions showed significant improvement in parenting behaviour post-treatment, this effect did not sustain at follow-up (low quality evidence) [32]. Additionally, psychological interventions did improve parent mental health at follow-up, this effect was not seen directly after treatment (very low quality evidence) [32].

Evidence on effect of cultural, spiritual, and religious support

Two RCTs described interventions directed at providing spiritual support [33, 34]. Iranian mothers of children with cancer received a spiritual training package comprising of seven 90-min group sessions and reported less stress, anxiety, and depression post-treatment in comparison to the wait-list control group (very low quality evidence) [33]. All outcomes remained decreased at three month follow-up, but the effect was only found to remain significant for anxiety (very low quality evidence) [33]. Additionally, an educational spiritual intervention consisting of six 45-min educational sessions on cancer disease, diagnosis, treatment, side effects, diet, spirituality, and meaning-making, decreased burn-out scores among parents of children with cancer (very low quality evidence) [34].

Evidence on preloss and bereavement care interventions

One RCT reported on a preloss and bereavement care intervention comprising of three components including a support package with information, peer supporter’s contact via telephone, and contact with a health care provider two to six weeks after death of the child [35]. The preloss and bereavement care intervention had no significant effect on grief reactions among mothers whose children died at age three or younger.

Evidence on components of preloss and bereavement care interventions, and communicative strategies

We included ten SRs of observational, qualitative, or mixed-method studies that reported on preloss and bereavement care intervention components for parents during the child’s end-of-life and after the child’s death [36,37,38,39,40,41,42,43,44,45]. We identified eight preloss and bereavement care intervention components including 1) acknowledging the child’s life and identity 2) acknowledging and enabling parenthood, 3) establishing keepsakes, 4) establishing follow-up contact with health care providers, 5) providing peer support, 6) providing education and information, 7) providing remembrance activities, and 8) offering therapies. For each component, we described the reported corresponding actions that could be taken. For example, studies described the following actions to acknowledge the child’s life and identity: providing the child with a certificate of life, providing the child with a blessing ceremony, acknowledging the child’s identity by learning individual needs and routines, and acknowledging birthdays, holidays, and anniversaries. Furthermore, we identified experiences and needs related to these components that were expressed by parents and health care providers. Generally, parents and health care providers experienced the identified preloss and bereavement care intervention components as desirable or helpful during the end-of-life and after the child’s death. For example, with regard to the component ‘acknowledging and enabling parenthood’, parents reported they desired to maintain a relationship with their child during the end-of-life and wished to be actively involved in the child’s care during this time. Moreover, most parents expressed that washing, holding, or dressing the child during the end-of-life and after the child’s death is helpful and valuable, but also emotionally difficult. They expressed that support and reassurance from health care providers to spend time with their child during the end-of-life and after the child’s death is needed. Table 2 summarises the preloss and bereavement care intervention components, specific actions, and corresponding experiences and needs reported by parents and health care providers.

Furthermore, the studies reported on a number of communicative strategies that provided support to parents during the child’s end-of-life and after the child’s death. We identified following strategies including provision of 1) communication, 2) emotional support, 3) hope, 4) knowledge, 5) consistency and continuity of care, and 6) sense of control. For each strategy we summarised positively and negatively labelled aspects that were reported in the SRs. For example, with regards to provision of communication, honest and straightforward communication was positively labelled, while the communication of inadequate and incomplete information about the child was labelled as negative. In Table 3, we provide an overview of these strategies and the reported positive and negative aspects.

Translating evidence into recommendations

The guideline development panel formulated 27 recommendations on psychosocial care including psychological interventions (n = 8), practical and social support (n = 8), and cultural, religious, and spiritual support (n = 11). Furthermore, a total of 21 recommendations were formulated on preloss and bereavement care. Recommendations were based upon evidence, existing guidelines, clinical expertise, and patient and family values. Table 4 provides an overview of all formulated recommendations. We indicated for each recommendation whether it was supported by evidence.

Table 4 Recommendations on paediatric palliative psychosocial care interventions including preloss and bereavement care interventions

Recommendations on psychological interventions

The evidence showed that some psychological interventions can improve certain psychosocial outcomes in both children with life-threatening and life-limiting conditions and their parents [28,29,30,31,32]. In the identified evidence, we found a large variability in intervention characteristics and measured outcomes [28,29,30,31,32]. Additionally, the quality of evidence was often rated as very low. Therefore, the guideline development panel was unable to recommend specific psychological interventions. Nevertheless, the panel recognised that psychological support could facilitate children with life-threatening and life-limiting conditions and their families in processing their experiences, concerns, and thoughts and formulated recommendations based on clinical expertise and patient values, considering the principles of good clinical practice. To facilitate appropriate psychological support, the panel stated that the health care provider who has the ultimate responsibility for the patients care (primary health care provider), should be responsible for timely initiation of psychosocial care in consultation with the family (recommendation 1, 9, 28). Moreover, the panel emphasised that psychological support should be tailored to the individual needs and preferences of child and family. The panel recognised that in order to tailor psychological support appropriately, attentiveness to the child’s age, developmental stage, and changes in the child’s condition is crucial. Furthermore, the panel highlighted the importance of providing clear information on emotional and psychological consequences of the child’s disease trajectory and monitoring the wellbeing of siblings and other family members actively (recommendation 2–7, 11, 12). The panel realised that the considerations used are not only essential in provision of psychological support but are the basis for provision of practical and social support, cultural, spiritual, and religious support, and preloss and bereavement care as well.

Recommendations on practical and social support

We found no evidence on the effects of practical and social support for children with life-threatening and life-limiting conditions and their family. Therefore, the guideline development panel formulated recommendations based on the NICE-guideline, clinical expertise, and patient and family values. With regards to practical and social support, low to moderate quality evidence from the NICE guideline showed that support for accessing care, and support from family members and local communities is helpful, as reported by parents. The panel acknowledged this and recommended that tailored practical and social support should be offered to the child and family. Additionally, the panel strongly recommended that when having conversations with child and family, health care providers should establish carefully what child and family already know, allow space, and time for emotions, and prepare parents on difficult questions children might ask (recommendation 11, 13). Furthermore, very low to moderate quality evidence from the NICE-guideline showed that parents and other family members greatly benefited from respite care but had difficulties to access it. Both parents and health care providers indicated that awareness and understanding of the benefits of respite care is important. The panel recognised that child and family do not always know what forms of practical support exist. They recommended that the child and family should be informed about existing and available practical support, such as respite care, financial support, and support at work (recommendation 14). Also, moderate quality evidence from the NICE-guideline showed that parents appreciated support that was provided before and after their child’s death. Moreover, they appreciated continuity in care and staff during the end-of-life and after their child’s death. Therefore, the panel recommended to discuss arrangements that can be made prior to and after the child’s death (recommendation 16). Moreover, they highlighted the importance of providing continuity in health care providers as much as possible to create trust and peace of mind (recommendation 10). Finally, the panel emphasised that not only child and family but also health care providers might need practical and social support (recommendation 15).

Recommendations on cultural, spiritual, and religious support

We found very low quality evidence that (educational) spiritual trainings can improve psychosocial outcomes [33, 34]. The effectivity of these interventions was studied in populations with cultural, religious, and spiritual backgrounds that were different from the Dutch population [33, 34]. Therefore, the guideline development panel was unable to state whether these same interventions would be effective for child and family in the Netherlands and therefore could not recommend these specific spiritual interventions. Additionally, very low to moderate quality evidence in the NICE guideline showed that parents reported a wide variety in their attitudes and needs regarding cultural, spiritual, and religious support, and experienced benefits and difficulties with regard to one’s beliefs. Moreover, parents reported the use of different aphorisms, rituals, and practices. As a result, the panel could not give recommendations on specific needs and preferences related to cultural, spiritual, and religious support. Instead, the panel emphasised that health care providers should consider and explore child and families’ cultural, spiritual, and religious beliefs, needs, values and wishes and that child and family should have access to cultural, spiritual, and religious support (recommendations 19, 21). The panel emphasises this is especially essential during decision-making, communication of bad news and meaning-making (recommendation 17, 18, 22–26). Spiritual counsellors can support child and family, regardless of their background, when existential questions may arise. Therefore, the panel recommended to engage a spiritual counsellor if necessary (recommendation 27).

Recommendations on preloss and bereavement care

We found very low quality evidence from one RCT, that a preloss and bereavement care intervention, comprising of a support package and health care provider or peer contact, had no significant effect on grief reactions among mothers [35]. From the evidence derived from ten SRs, we identified multiple preloss and bereavement care intervention components and communicative strategies that were experienced as helpful or desirable by both parents and health care providers (Tables 2 and 3) [36,37,38,39,40,41,42,43,44,45]. Based on these results, the guideline development panel emphasised the importance of offering preloss and bereavement care to children with life-threatening and life-limiting conditions and their family. Furthermore, the panel highlighted the importance of understanding the extent of losses that parents encounter. As parental coping with loss and grief entails both containing feelings of grief and experiencing them, losses may be more intertwined with daily experiences then health care providers may be aware of. Therefore, the panel recommended health care providers to be alert to the losses that parents experience, which vary per person (recommendation 29, 30). Moreover, the panel recognised that health care providers can have a great impact on how parents experience the child’s end-of life and can support parents by engaging in conversation with time and attention and seeing the child as a person and family as a whole. It is recommended for health care providers to inform parents on grief reactions, attune their care to the parents’ coping, to be sensitive to signals from parents, to discuss child and family wishes at arranged times, and to be attentive to the impact of culture, spirituality and religion on loss and grief (recommendation 31, 32). Furthermore, health care providers should offer time and space to talk about feelings of loss and grief. However, they should be careful not to disrupt the parents’ coping by, for instance, postponing the exploration of grief when parents are in need of containing their grief. Therefore, the panel recommended to appoint a few health care providers that explore the topic of loss and grief with parents so that others may offer them some respite on the topic (recommendation 33). Based on the identified preloss and bereavement care components and communicative strategies that parents and health care providers perceived as desirable, the panel recommended to make parents feel safe and secure by seeing and acknowledging the child, to actively offer information about possible support, and to provide multiple preloss and bereavement care conversations over time (recommendation 34-38). Health care providers play an important role in the continuity of care and support after the child’s death. During follow-up conversations, they can support parents to build a narrative around their child’s (end-of-) life, by acknowledging the child’s identity, discussing the disease trajectory, exploring future needs regarding bereavement care, and facilitating the parents to provide feedback (recommendation 39, 40).

Finally, the panel emphasised the importance of supporting the sick child and sibling during experiences of loss, grief, and bereavement. It is recommended to explore with parents whether or not and how to talk to their children about approaching end-of-life, to explain to children what will happen in a developmentally appropriate way, to give the child the opportunity to express emotions and feelings, to involve siblings at the time of death by giving them a meaningful task, and to help children and siblings regulate emotions by assigning words to expressions or emotions (recommendation 41-46). Furthermore, the panel highlighted that children often grief intermittently and conceptualisation of death may change as children mature and recommend health care providers to consider this when providing support, which may also include prolonging support over a longer period of time as children grief new losses as they reach new developmental stages (recommendation 47, 48).

Discussion

It is widely recognised that a child’s diagnosis with a life-threatening or life-limiting condition impacts the psychosocial functioning of the child and the entire family [8]. This is not only relevant during the child’s life, but also after the death of the child [19, 46]. Provision of psychosocial care including preloss and bereavement care is essential for child and family, enhances quality of life and is often mentioned as being a crucial part of paediatric palliative care [7, 8, 15, 16]. However, studies frequently report a lack of attention to psychosocial care including preloss and bereavement care [8, 20]. In this paper, we present the identified evidence and provide recommendations to optimise psychosocial care including psychological interventions, practical and social support, cultural, spiritual, and religious support, and preloss and bereavement care in paediatric palliative care. Below, we discuss the methods used, the identified evidence, and the provided recommendations.

We formulated recommendations through an evidence-based approach using different methods. First, we systematically searched for quantitative evidence on effectivity of paediatric palliative care interventions in SRs of RCTs and CCTs, RCTs, and CCTs. We identified eight studies reporting on effectivity of psychological interventions, spiritual training interventions, and a bereavement care intervention [28,29,30,31,32,33,34,35]. The quality of the total body of evidence was rated as very low to moderate. We also included evidence from ten SRs of observational, qualitative, or mixed-method studies on preloss and bereavement care [36,37,38,39,40,41,42,43,44,45]. Unfortunately, our clinical questions could be only partly answered with the identified evidence.

Second, since we identified limited evidence, we used the conclusions of evidence, considerations, and recommendations on paediatric palliative care from the NICE-guideline to refine considerations and inform our recommendations [26]. Finally, we formulated all recommendations in close collaboration with a national multidisciplinary guideline development panel consisting of professionals from multiple disciplines and parents. If no or limited evidence or no existing guidelines were available, the recommendations were based upon clinical expertise and patient and family values only. By using different sources of knowledge, i.e. the identified evidence, existing guidelines, clinical expertise, and patient and family values, we were able to compile a comprehensive set of strong recommendations that provide guidance on psychosocial care including preloss and bereavement care.

The identified studies described the effects of a variety of interventions related to psychosocial care, of which only some interventions were found to significantly improve certain psychosocial outcomes in children with life-threatening and life-limiting conditions or their parents. As a result, we identified multiple gaps of knowledge. First, the majority of the studies reported on effects of psychological, spiritual, and preloss and bereavement care interventions among parents. Only three studies focused on the effectivity of psychological interventions among children. The majority of conclusions of evidence were rated as very low quality evidence. Additionally, we found no evidence on practical and social support among both children with life-threatening and life-limiting conditions and their family. Finally, we have found no evidence on the effectivity of any psychosocial care interventions among siblings. Both social and practical support including respite care and psychosocial care interventions for siblings have been well documented knowledge gaps but are yet to be addressed [12, 16, 18, 46, 47]. We acknowledge that due to the small numbers of children who need paediatric palliative care, diversity of diseases, vulnerability of the population and ethical issues, conducting studies in this vulnerable patient population is very challenging [48]. Therefore, we urge for international collaboration to conduct well-designed multisite parallel RCTs to address these knowledge gaps.

Moreover, we found a large variability in reported psychosocial outcomes and outcome measurements, which made it difficult to compare the effectivity of interventions. As a result, we could not draw a definitive conclusion on which intervention can best be used. This was in line with the findings of a recent SR that reported significant heterogeneity in used outcomes in paediatric palliative care [49]. In this SR, 136 outcomes in 46 outcome domains were used to measure the effect of specialised paediatric palliative care interventions [49]. In order to improve comparability between interventions, we suggest developing a standard set of outcome indicators for paediatric palliative care with harmonised outcome definitions and measurement tools [50]. To ensure that the standard set of outcome indicators represents the values and needs of patient, family, and health care providers, all stakeholders should be involved in selecting the outcome indicators [50]. Adopting this standard set of outcomes for paediatric palliative care across organisations and countries, could facilitate the identification of best practices and further improve quality of paediatric palliative care worldwide.

With regard to the translation of evidence into recommendations, a few points should be addressed. First, it should be noted that evidence published from 1970 to 2020 was included in this guideline. As a result, it is possible that we missed some recently published studies. However, given the limited and generally low-quality evidence we found over this large time frame, it is likely that only a small number of studies were missed. We expect that this will not significantly change the recommendations. Nonetheless, there is a possibility that some recommendations might become outdated when new evidence emerges. Guideline development is a time-consuming process, making it challenging to respond to newly published evidence rapidly. To address this, we propose the development and use of automated tools for identifying and appraising evidence for improving efficiency of future guideline updates, allowing an update of a recommendation as soon as new relevant evidence becomes available [51, 52].

Furthermore, due to the lack of evidence, the majority of the recommendations was based upon clinical expertise and patient and family values only. Often, these recommendations are viewed negatively and are considered less trustworthy [53]. However, the incorporation of clinical expertise and patient and family values in clinical decision-making is one of the core principles of evidence-based medicine [54, 55]. As a result, clinical expertise and patient and family values are essential components for interpreting evidence and formulating recommendations. However, it is important to note that recommendations based upon clinical expertise can be prone to personal bias [56]. Therefore, we ensured that the recommendations were developed through a careful process in which only recommendations on which there was group consensus were approved. Unfortunately, the clinical expertise and patient values were not systematically collected. To further improve transparency of recommendations based on clinical expertise and to limit personal bias, using surveys to systematically and independently identify expert opinions and patient values could be an opportunity [56].

Additionally, when translating evidence into recommendations, it became very clear that psychosocial care including psychological interventions, practical and social support, cultural, spiritual, and religious support, and preloss and bereavement care is considered beneficial for all children with life-threatening and life-limiting conditions and their families. Psychosocial care including preloss and bereavement care should not only be offered to every child and family but should also be tailored to the child and family as a whole. We recognise that provision of tailored psychosocial care, requires adequate staffing, time, funding, and education on the importance of psychological, social, and spiritual needs assessment and support [16, 57,58,59]. We emphasise that addressing these issues is a precondition for application of recommendations in clinical practice [59]. We believe a large proportion of these recommendations will be applicable in other countries and can be used to optimise psychosocial care including preloss and bereavement care. However, we recognise that country and culture specific factors should be carefully considered before applying any recommendations.

Furthermore, it is increasingly recognized that caring for children who are suffering, and their families can lead to distress and burnout among health care providers [9, 16]. Health care providers who provide paediatric palliative care should receive support to prevent distress, compassion fatigue, and burnout. However, there remains a lack of studies on the effectivity of interventions aiming to prevent distress and burnout among health care providers [9, 16]. We strongly urge for more research on this topic to formulate evidence-based recommendations.

In conclusion, with the provided recommendations, we aim to optimise psychosocial care including preloss and bereavement care for children with life-threatening and life-limiting conditions and their families. We identified that psychosocial care requires a personal approach tailored to the child and family. We urge for more research on effectiveness of psychosocial care, including psychological interventions, practical and social support, cultural, spiritual, and religious support, and preloss and bereavement care interventions among children with life-threatening and life-limiting conditions and parents, but also in siblings. Furthermore, although very challenging, we propose implementing strategies to improve research and thereby further optimise the quality of paediatric palliative care. These strategies include the development of a standard set of outcomes and international collaboration to conduct well-designed multisite RCTs.