Introduction

Worldwide there are approximately 21 million children and young people aged 0–19 years (hereafter ‘children’) with life-limiting and life-threatening conditions (hereafter ‘life-limiting’) [1]. Life-limiting conditions are those for which there is no hope of cure and from which children will die [2]. Life-threatening conditions are those for which curative treatment may be feasible but may fail [2].

Due to medical advances, increasing numbers of children are living with life-limiting conditions [3, 4]. However, provision of children’s palliative care varies geographically, and increased prevalence has not been met with an equivalent increase in healthcare resource [3, 5].

Palliative care for adults is effective and cost-effective, reducing unplanned admissions and futile treatments [6,7,8], while improving quality of life, care quality, and survival [9,10,11]. There are almost 400 conditions that affect children for which palliative care could be beneficial [3, 12]. However, evidence for effectiveness of children’s palliative care is limited in part due to a lack of a valid and reliable outcome measure [13, 14]. Development of such a measure has repeatedly been identified as a research priority [15,16,17]. A measure is in development in sub-Saharan Africa and Belgium, but primary data to inform measurement has not been generated outside Africa [18,19,20].

Outcome measure development for children with life-limiting conditions is complex due to differences in age and developmental stage, the range of conditions [12], and the role of family in care provision. To establish face and content validity, it is imperative to understand which symptoms and concerns matter the most. However, most studies focus on children with cancer [21], or rely on proxy reports of parent/carers (hereafter ‘parents’) or health and social care (healthcare) professionals [21]. This exclusion of children from participating in primary research directly contradicts the growing focus on children having agency, with a right to be involved in their own healthcare decisions [22, 23] as active partners in their healthcare, not passive recipients [22, 24]. This study aimed to identify the symptoms, concerns, and care priorities of children with life-limiting conditions and their families.

Methods

Study design

Semi-structured, qualitative interview study reported in accordance with the consolidated criteria for reporting qualitative studies (COREQ) [25].

Setting

Children, parents, and healthcare professionals were recruited from six hospitals and three children’s hospices within three UK countries.

Commissioners were recruited via recommendations from healthcare professionals and the UK’s national children’s palliative care advocacy charity.

Sampling and recruitment

Inclusion criteria

Children (5–17 years) with any life-limiting condition; parents/carers with a child < 18 years old with a life-limiting condition; siblings (5–17 years) of children with a life-limiting condition; healthcare professionals with > 6 months experience of caring for children with life-limiting conditions; commissioners of UK paediatric palliative care services.

Exclusion criteria

Children: unable to communicate via an in-depth interview, using ‘draw and talk’ or play methods or via their parents; speaks a language not supported by NHS translation services; currently enrolled in another study; unable to give consent/assent.

Parents/carers and siblings: unable to give consent/assent, speaks a language not supported by NHS translation services.

Purposive sampling was used to ensure maximum variation in key demographics such as age and condition. Given the heterogeneity of the sample, the concept of pragmatic saturation was used to determine the required sample size in order for the dataset to have the required diversity and depth to meet the aims and objectives of the study [26].

Data collection

Semi-structured interviews were conducted using a topic guide informed by a systematic review of symptoms and concerns in children with life-limiting conditions [21] and the World Health Organisation (WHO) definition of paediatric palliative care [27]. The topic guide was reviewed by the study steering group (healthcare professionals, parents, and researchers). Interviews were conducted by LC (experienced children’s palliative care nurse, new to qualitative research), AR (new to qualitative research), and DB (experienced qualitative researcher). All interviewers received training and supervision on conducting interviews with children, including communication, legal, and ethical issues.

Interviews commenced with demographic questions and children were asked about their hobbies and interests to build rapport. Play and drawing were used to aid interviews where required. The topic guide contained an open question asking participants to describe their/their child’s condition and how it affects their/their child’s life. Interviews with professionals asked about the main symptoms, concerns, and care priorities of children with life-limiting conditions. Probes ensured that all domains from the WHO definition of palliative care were discussed, while allowing participants to discuss what mattered most. Interviews were audio-recorded, transcribed verbatim, and pseudonymised.

Data analysis

Transcripts were analysed by LC, DB, AR, DH, and HS using deductive (from the WHO domains of palliative care [27]) and inductive coding [28, 29]. Analysis followed the five steps of framework analysis: familiarisation, constructing a thematic framework, indexing and sorting, charting and mapping/interpretation [28,29,30] using NVivo software (Version 12). Using framework analysis allowed the authors to compare and contrast the findings from each theme overall and by participant group. Regular meetings were held to discuss emerging themes and resolve any differences (20% of transcripts were independently coded by two researchers). RH, KB, and CES were consulted if discrepancies could not be resolved. Analysis was reviewed by the study steering group throughout the study.

Ethical approval

Ethical approval was granted by the Bloomsbury research ethics committee (HRA:19/LO/0033). Participants over 16 years old provided written informed consent. Those with parental responsibility provided written informed consent for participants < 16 years. Those < 16 years provided written assent.

Results

Participant characteristics

A total of 103 interviews were conducted (April 2019–September 2020) with 106 participants: 26 children, 40 parents, 13 siblings, 15 health and social care professionals and 12 commissioners (see Table 1). Two sets of parents and one set of siblings were interviewed together. ICD-10-chapter headings are reporting for pseudonymity as some children reported rare conditions. Most interviews were carried out face-to-face in a location of the participant’s choosing. Due to the COVID-19 pandemic, 13 interviews were conducted remotely (telephone or video call) [31].

Table 1 Demographic details of participants
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Priority healthcare outcomes

The priority healthcare outcomes of children with life-limiting conditions and their families fitted into five themes—physical, spiritual and existential, emotional and psychological, social and practical, and pursuing normality. Table 2 shows these themes and the subthemes that comprise them. Illustrative quotes are presented in Tables 3, 4, and 5 and supplementary Table 1 (S1). Themes and subthemes were often closely inter-related.

Table 2 Inter-related domains and themes—symptoms, concerns, and care priorities (n = 106)
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Table 3 Participant quotes—physical symptoms and concerns, and spiritual and existential concerns
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Table 4 Participant quotes—emotional and psychological concerns, and social concerns
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Table 5 Participant quotes—practical concerns and normality
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Physical symptoms and concerns

All participants spoke of the importance of managing pain and other physical symptoms (such as seizures and infection), and the impact of multiple medical interventions. Symptom management and children being ‘comfortable’ was important to parents and professionals (T3Q1). Pain and other symptoms were often linked to other themes. For example, if physical symptoms were well managed, then children were more likely to be happy, have reduced anxiety, and be able to participate in normal childhood activities. Professionals discussed symptom management in relation to managing expectations of care and setting realistic goals (T3Q2). Seizures were particularly distressing and often described as difficult to manage by parents (T3Q3), sometimes being triggered by noise and over excitement (T3Q4), meaning siblings had to play quietly.

Participants from all groups spoke of the difficulties children had with eating and drinking. Some children described feeling under pressure to maintain weight (T3Q5), and others required artificial feeding. Healthy siblings spoke of feeling guilty about consuming treats in front of a sibling who was unable to eat (T3Q6).

Tiredness and fatigue were a concern for both children and parents. Parents spoke of lack of sleep and exhaustion which impacted on ability to care for their child (T3Q7). Children spoke of overwhelming fatigue causing lack of stamina and the need to take daytime naps (T3Q8).

Siblings and children with life-limiting conditions were very aware of changes in physical appearance which impacted on school attendance, seeing friends, and social activities (T3Q9).

Spiritual and existential

Professionals spoke of lack of confidence in discussing spiritual and existential issues (T3Q10). For some patients and families, faith offered a source of comfort (T3Q11, S1Q1), whereas for others, it was a potential cause of conflict (T3Q12). Some moved more towards faith, for example, by having their child christened ‘just in case’ (T3Q12). Faith was also important in decisions about future care, with one participant describing how hospital policy on death registration and care of the body conflicted with her own culture (T3Q14).

Participants from all groups spoke about the uncertainty surrounding length of life (T3Q15), with children wanting to plan for their future regardless of their prognosis (T3Q16). Children were often determined to overcome and survive (T3Q17, S1Q3). Parents spoke of adjusting their hopes and dreams for a child who would be unlikely to reach typical life-course milestones (T3Q18) and questioned the meaning of illness (‘why me/why my child?’) (T3Q19). They expressed a desire for their child to live life as fully as possible, to their full potential, experience relationships with others, and have things to hope for and look forward to (T3Q20).

Emotional and psychological

All participants described many psychological and emotional impacts of living with a life-limiting condition. Where children had been diagnosed during childhood, rather than at birth, they spoke of an awareness of being different and having different life experiences (T4Q21). For some siblings, their experience led to desires to pursue caring careers (T4Q22), while children with life-limiting conditions sought out others with similar experiences (T4Q23).

All participants spoke of the life-altering impact of living with a life-limiting condition (T4Q24). They described anger, worry, sadness (T4Q25, S1Q4), and an overwhelming desire for children to be happy (T4Q26, S1Q5). Older children spoke of loss of privacy, control, and independence (T4Q27–28, S1Q6–7). Parents also faced a loss of privacy due to having professionals in their home, and the wish to maintain some control over their child’s care and condition (T4Q29, S1Q8–9).

There was a sense of children and parents wanting to protect each other from how they were feeling, specifically around discussion of prognosis (T4Q30). Parents found accessing psychological support for themselves and siblings challenging, as this is often hospital-based and does not fit around work and school hours (T4Q31). Individuals also spoke of the importance of memory making (T4Q432).

Social concerns

Children were focused on being able to undertake usual childhood activities such as seeing friends, pursuing hobbies, and playing. School was important to parents and children for maintaining friendships, retaining a sense of normality and planning for a future by preparing for exams (T4Q33, S1Q10). Parents spoke of difficulty in accessing suitable education for their child due to complex medical needs (T4Q34). Many parents and children experienced loneliness and isolation due to absence from school and not being able to find suitable activities for their child to take part in (T4Q35–36, S1Q11). Unclear communication about symptom management goals and service availability often led to unrealistic expectations, causing discord between professionals and families. This impacted on decision-making, trust and respect, and continuity and co-ordination of care (T4Q37–38, S1Q12).

Parents and professionals spoke of the financial impact of having a child with a life-limiting condition in terms of having to give up work, the expense of hospital stays, and self-funding equipment due to lack of availability (T4Q39–40, S1Q13).

Practical concerns

Parents and professionals were concerned with many practical aspects of care. These included care quality, advance care planning, service availability and facilities (T5Q41–42, S1Q14), the huge familial burden of care, and the logistics of managing this (T5Q43, S1Q15). The physical burden of care increased as children grew older (T5Q44). Access to respite care was essential to many parents of children without a cancer diagnosis, allowing them to have uninterrupted sleep and spend time with other children (T5Q45). Children did not share these concerns and were more interested in being at home (not hospital), being able to see their friends and carry on with their usual activities (T5Q46–47, S1Q16–17).

Parents and children felt well informed about the condition, treatment, and available services, which was considered important (T5Q48, S1Q18). Siblings often felt less well informed and not included in care (T5Q49).

Normality

The theme of normality was cross-cutting across all other themes. Children wanted to live life as normally as possible, focusing on being a child first, with their condition secondary to this (T5Q50). They described the importance of seeing friends, attending school, and making plans for the future. To achieve this, physical symptoms need to be well managed. Children with varying diagnoses described normality in different ways, with all wanting to pursue normal childhood activities. When a condition had been present since birth or soon after, children spoke of feeling normal and not knowing any different (T5Q51). Those that had been diagnosed later in childhood spoke of having to adjust to a new normal such as having carers in the home (T5Q52). Those with an uncertain prognosis, such as cancer, wanted life to return to pre-diagnosis normality and desired to be like their healthy peers (T5Q53–54). Parents who had been caring for a child with a life-limiting condition for many years had often adjusted to their child’s care needs and had to remind themselves of their unique situation (T5Q55, S1Q19). Siblings spoke of seeing their unwell sibling as normal but with different needs (T5Q56, S1Q20).

Discussion

This study provides novel evidence of inter-related symptoms, concerns, and care priorities for children with a wide range of life-limiting conditions and their families, from the perspectives of multiple stakeholders (including children). This is an area of knowledge not previously well described [21]. Symptoms and concerns were broadly the same across the spectrum of life-limiting conditions, which is a finding previously reported [21, 32]. Most were evident across participant groups, except practical aspects of care, which were not a priority for children.

The concept of child-centred care encourages healthcare professionals to place the child and their interests at the centre of thinking and, where able, include them as active participants [22]. The focus of care is on the child in the context of the family, while acknowledging the child’s wider environment and relationships [22, 33]. Previous studies have found that children with cancer and their families try to adjust to a ‘new normal’, and those with severe neurological impairment were able to regain some normality with input from a paediatric palliative care team [34,35,36]. Our study adds to the concept of pursuing normality within the context of children living with life-limiting conditions, demonstrating that a child-centred approach to care needs to take an individual and holistic view of the child, ensuring that physical, emotional, social, practical, and spiritual concerns are addressed. This enables children to pursue normal childhood activities such as attending school and seeing friends. Children in our study wanted to be seen as children first, with their condition coming second to this, reinforcing that children do not want to be defined by their condition [37].

We found children wanted the opportunity to make plans for a longer-term future, even if these would not be realised, adding to the concept of pursuing normality. In contrast, a previous study found that children with neuro-disability only want to plan for the present or near future [38]. This difference may be due to the older age of the sample of participants with neurodisability meaning they had a better understanding of their condition. The heterogeneity of conditions in our study may also have contributed to our finding, as curative treatment for some life-limiting conditions is feasible, but may fail [39].

Taking a child-centred approach to care for children with life-limiting conditions needs to incorporate support for the family, while ensuring that the child remains the focus of care [40, 41]. This is important for families of children with life-limiting conditions, as this study demonstrates that they often have to provide complex, burdensome care. Many life-limited children are unable to communicate their needs due to their condition, and parents will need to advocate for their best interests. Parents require access to adequate holistic services, particularly respite care and practical support to enable them to provide care. Parents and siblings need time and space to undertake their own normal activities such as self-care, spending time as a family, and seeing friends. In our study, this was not always achieved, with insufficient or inaccessible practical, psychological, educational, and respite support often highlighted, along with lack of co-ordination and communication between services. To attempt to address this pursuit of normality and accomplish child-centred care, services need to be co-ordinated around child and family needs [40, 42], and this should be considered in the design of future health services for those with life-limiting conditions.

In our study, we found that children as young as five wanted to be informed about their condition, supporting a child-centred approach to care where the child is, where able, encouraged, and supported to be an active participant. Other studies have found that the desire to be informed about a condition is associated with adolescence, rather than younger children [21]. Siblings wanted to be informed, which is a finding previously reported in children whose parents have a life-limiting illness [43].

Strengths and limitations

As far as the authors are aware, this is one of the largest studies conducted exploring symptoms and concerns of children with a range of life-limiting conditions from multiple stakeholder perspectives. We have demonstrated that verbal children from the age of five years old are willing and able to participate in research and share their perspectives on their condition. This study’s strengths include our large sample, wide range of stakeholder participants, and the range of life-limiting conditions. Fathers, who are often underrepresented in palliative care research, represented 25% of our parent sample [44].

Our study has several limitations. Recruitment took place in a small number of UK sites and data on ethnicity was not collected. One site recruited only children with gastrointestinal diagnoses, and this is reflected in the higher number of participants from this group. There are almost 400 different life-limiting conditions known to affect children, so not all could be included [12]. Many children with life-limiting conditions are non-verbal and cannot meaningfully share their perspectives and parent/proxy-reporting has to be used. The findings presented here reflect those of children who were able to participate. As a child-centred approach to care should include support for the family, care must enable them to use their knowledge and experience of their child in order to advocate for them. The child’s needs and interests should always be at the centre of care and decisions [42].

Clinical and research implications

This study provides a comprehensive insight into what symptoms, concerns, and care priorities are important to children with life-limiting conditions and their families, to enable healthcare professionals to support them to be viewed as children, rather than their condition, within a child-centred model of care. We have demonstrated that children can be meaningfully involved in such studies [45]. Findings will be used to develop the construct for a valid child-centred outcome measure for use in this population.

Conclusions

Children want to focus on pursuing normal childhood activities, but need a holistic approach in addressing their care needs to achieve this. Improvements in accessibility, availability, and co-ordination of relevant health services are required.