Cognitive testing of the Children's Palliative Outcome Scale (C-POS) with children, young people and their parents/carers.

BACKGROUND: The Children's Palliative Outcome Scale (C-POS) is being developed using best methodological guidance on outcome measure development, This recommends cognitive testing, an established method of item improvement, prior to psychometric testing. AIM: To cognitively test C-POS within the target population to establish comprehensibility, comprehensiveness, relevance and acceptability. DESIGN: Cross-sectional cognitive interview study following COnsensus-based Standards for the selection of health Measurement INstruments (COSMIN) methodology and Rothrock guidance on outcome measure development. Cognitive interviews were conducted using 'think aloud' and verbal probing techniques. SETTING/PARTICIPANTS: Children 5-⩽17 years old with life-limiting conditions and parents/carers of children with life-limiting conditions were recruited from 14 UK sites. RESULTS: Forty-eight individuals participated (36 parents; 12 children) in cognitively testing the five versions of C-POS over two to seven rounds. Content and length were acceptable, and all questions were considered important. Refinements were made to parent/carer versions to be inclusive of non-verbal children such as changing 'share' to 'express' feelings; and 'being able to ask questions' to 'having the appropriate information'. Changes to improve comprehensibility of items such as 'living life to the fullest' were also made. Parents reported that completing an outcome measure can be distressing but this is anticipated and that being asked is important. CONCLUSION: Cognitive interviewing has facilitated refinement of the C-POS, especially for non-verbal children who represent a large proportion of those with a life-limiting condition. This study has enhanced the face and content validity of the measure and provided preliminary evidence for acceptability for use in routine practice.

'My life is a mess but I cope': An analysis of the language children and young people use to describe their own life-limiting or life-threatening condition.

BACKGROUND: Children and young people with life-limiting and life-threatening conditions have multidimensional needs and heterogenous cognitive and communicative abilities. There is limited evidence to support clinicians to tailor their communication to each individual child. AIM: To explore the language children and young people use to describe their own condition, to inform strategies for discussing needs and priorities. DESIGN: Positioned within a social constructivist paradigm, a secondary discourse analysis of semi-structured interview data was conducted incorporating the discourse dynamics approach for figurative language. SETTING/PARTICIPANTS: A total of 26 children and young people aged 5-17 years with life-limiting or life-threatening conditions (6 cancer; 20 non-cancer) were recruited from nine clinical services (six hospitals and three hospices) across two UK nations. RESULTS: The language children and young people use positions them as 'experts in their condition'. They combine medical terminology with their preferred terms for their body to describe symptoms and treatments, and use comparatives and superlatives to communicate their health status. Their language depicts their condition as a 'series of (functional and social) losses', which single them out from their peers as 'the sick one'. Older children and young people also incorporate figurative language to expand their descriptions. CONCLUSION/DISCUSSION: Children and young people can provide rich descriptions of their condition. Paying attention to their lexical choices, and converging one's language towards theirs, may enable more child-centred discussions. Expanding discussions about 'what matters most' with consideration of the losses and differences they have experienced may facilitate a fuller assessment of their concerns, preferences and priorities.

What are the anticipated benefits, risks, barriers and facilitators to implementing person-centred outcome measures into routine care for children and young people with life-limiting and life-threatening conditions? A qualitative interview study with key stakeholders.

BACKGROUND: There is a growing evidence-base underpinning implementation of person-centred outcome measures into adult palliative care. However evidence on how best to achieve this with children facing life-threatening and life-limiting conditions is limited. AIM: To identify the anticipated benefits, risks, barriers and facilitators to implementing person-centred outcome measures for children with life-limiting and life-threatening conditions. DESIGN: Cross-sectional qualitative semi-structured interview study with key stakeholders analysed using Framework analysis informed by the adapted-Consolidated Framework for Implementation Research. SETTING/PARTICIPANTS: A total of n = 26 children with life-limiting or life-threatening conditions, n = 40 parents/carers, n = 13 siblings and n = 15 health and social care professionals recruited from six hospitals and three children's hospices and n = 12 Commissioners of health services. RESULTS: All participants were supportive of future implementation of person-centred outcome measures into care. Anticipated benefits included: better understanding of patient and family priorities, improved communication and collaborative working between professionals and families and standardisation in data collection and reporting. Anticipated risks included increased workload for staff and measures not being used as intended. Implementation barriers included: acceptability and usability of outcome measures by children; burden and capacity of parents/carers regarding completion; privacy concerns; and language barriers. Implementation facilitators included designing measures using language that is meaningful to children and families, ensuring potential benefits of person-centred outcome measures are communicated to encourage 'buy-in' and administering measures with known and trusted professional. CONCLUSIONS: Implementation of person-centred outcome measures offer potential benefits for children with life-limiting and life-threatening conditions. Eight recommendations are made to maximise benefits and minimise risks in implementation.

Achieving consensus on priority items for paediatric palliative care outcome measurement: Results from a modified Delphi survey, engagement with a children's research involvement group and expert item generation.

BACKGROUND: There is no validated outcome measure for use in children's palliative care outside sub-Saharan Africa. Stakeholders must be involved in the development of such measures to ensure face and content validity. AIM: To gain expert stakeholder consensus on items for inclusion in a paediatric palliative care outcome measure to establish face and content validity. DESIGN: This study was conducted in two phases following Rothrock and COSMIN guidance on patient-reported outcome measure development. Phase 1: Three-round modified Delphi survey to establish consensus on priority items. Phase 2: Item generation meeting with key stakeholders to develop initial measure versions. A young person's advisory group was also consulted on priority outcomes. SETTING AND PARTICIPANTS: Delphi survey: Parents and professionals with experience of caring for a child with a life-limiting condition. Young person's advisory group: young people age 10-20 years. Item generation meeting: bereaved parents, academics and clinicians. RESULTS: Phase 1: Delphi survey (n = 82). Agreement increased from Kendall's W = 0.17 to W = 0.61, indicating movement towards consensus. Agreement between professional and parent ranking was poor (Cohen's kappa 0.13). Professionals prioritised physical symptoms, whereas parents prioritised psychosocial and practical concerns. Advisory group: Children (n = 22) prioritised items related to living a 'normal life' in addition to items prioritised by adult participants. Phase 2: Five age/developmental stage appropriate child and proxy-reported versions of C-POS, containing 13 items, were drafted. CONCLUSIONS: This study highlights the importance and feasibility of involving key stakeholders in PROM item generation, as important differences were found in the priority outcomes identified by children, parents and professionals.

Design and Administration of Patient-Centred Outcome Measures: The Perspectives of Children and Young People with Life-Limiting or Life-Threatening Conditions and Their Family Members.

BACKGROUND: Self-reported health data from children with life-limiting conditions is rarely collected. To improve acceptability and feasibility of child and family-centred outcome measures for children, they need to be designed in a way that reflects preferences, priorities and abilities. OBJECTIVES: The aim was to identify preferences for patient-reported outcome measure design (recall period, response format, length, administration mode) to improve the feasibility, acceptability, comprehensibility and relevance of a child and family-centred outcome measure, among children with life-limiting conditions and their family members. METHOD: A semi-structured qualitative interview study seeking the perspectives of children with life-limiting conditions, their siblings and parents on measure design was conducted. Participants were purposively sampled and recruited from nine UK sites. Verbatim transcripts were analysed using framework analysis. RESULTS: A total of 79 participants were recruited: 39 children aged 5-17 years (26 living with a life-limiting condition; 13 healthy siblings) and 40 parents (of children aged 0-17 years). Children found a short recall period and a visually appealing measure with ten questions or fewer most acceptable. Children with life-limiting conditions were more familiar with using rating scales such as numeric and Likert than their healthy siblings. Children emphasised the importance of completing the measure alongside interactions with a healthcare professional to enable them to talk about their responses. While parents assumed that electronic completion methods would be most feasible and acceptable, a small number of children preferred paper. CONCLUSIONS: This study demonstrates that children with life-limiting conditions can engage in communicating preferences regarding the design of a patient-centred outcome measure. Where possible, children should be given the opportunity to participate in the measure development process to enhance acceptability and uptake in clinical practice. Results of this study should be considered in future research on outcome measure development in children.

Spiritual, religious, and existential concerns of children and young people with life-limiting and life-threatening conditions: A qualitative interview study.

BACKGROUND: Despite being a core domain of palliative care, primary data on spiritual and existential concerns has rarely been collected among children with life-limiting and life-threatening conditions and their families. Existing evidence has tended to focus on the religious aspects among children with cancer. AIM: To identify the spiritual needs of children with life-limiting and life-threatening conditions. DESIGN: Cross-sectional semi-structured, qualitative interview study with children, families and health and social care professionals. Verbatim transcripts were analysed using Framework analysis. SETTING/PARTICIPANTS: Purposively sampled children with life-limiting and life-threatening conditions, their parents and siblings, health and social care professionals recruited from six hospitals and three children's hospices in the UK, and commissioners of paediatric palliative care services recruited through networks and a national charity. RESULTS: One hundred six participants were interviewed: 26 children (5-17 years), 53 family members (parents/carers of children 0-17 years and siblings (5-17 years)), 27 professionals (health and social care professionals and commissioners of paediatric palliative care). Themes included: living life to the fullest, meaning of life and leaving a legacy, uncertainty about the future, determination to survive, accepting or fighting the future and role of religion. Children as young as 5 years old identified needs or concerns in the spiritual domain of care. CONCLUSIONS: Addressing spiritual concerns is essential to providing child- and family-centred palliative care. Eliciting spiritual concerns may enable health and social care professionals to identify the things that can support and enhance a meaningful life and legacy for children and their families.

Achieving child-centred care for children and young people with life-limiting and life-threatening conditions-a qualitative interview study.

This study aims to identify the symptoms, concerns, and care priorities of children with life-limiting conditions and their families. A semi-structured qualitative interview study was conducted, seeking perspectives from multiple stakeholders on symptoms, other concerns, and care priorities of children and young people with life limiting and life-threatening conditions and their families. Participants were recruited from six hospitals and three children's hospices in the UK. Verbatim transcripts were analysed using framework analysis. A total of 106 participants were recruited: 26 children (5-17 years), 40 parents (of children 0-17 years), 13 siblings (5-17 years), 15 health and social care professionals, 12 commissioners. Participants described many inter-related symptoms, concerns, and care priorities impacting on all aspects of life. Burdensome symptoms included pain and seizures. Participants spoke of the emotional and social impacts of living with life-limiting conditions, such as being able to see friends, and accessing education and psychological support. Spiritual/existential concerns included the meaning of illness and planning for an uncertain future. Data revealed an overarching theme of pursuing 'normality', described as children's desire to undertake usual childhood activities. Parents need support with practical aspects of care to help realise this desire for normality. CONCLUSION: Children with life-limiting conditions and their families experience a wide range of inter-related symptoms, concerns, and care priorities. A holistic, child-centred approach to care is needed, allowing focus on pursuit of normal childhood activities. Improvements in accessibility, co-ordination, and availability of health services are required to achieve this. WHAT IS KNOWN: • Existing evidence regarding symptoms, concerns, and care priorities for children with life-limiting conditions is largely limited to proxy-reported data and those with a cancer diagnosis. • Child-centred care provision must be directed by children's perspectives on their priorities for care. WHAT IS NEW: • Social and educational activities are more important to children with life-limiting conditions than their medical concerns. • A holistic approach to care is required that extends beyond addressing medical needs, in order to support children with life-limiting conditions to focus on pursuit of normal childhood activities.

How do person-centered outcome measures enable shared decision-making for people with dementia and family carers?-A systematic review.

Objectives: To identify published evidence on person-centered outcome measures (PCOMs) used in dementia care and to explore how PCOMs facilitate shared decision-making and improve outcomes of care. To build a logic model based on the findings, depicting linkages with PCOM impact mechanisms and care outcomes. Design: Mixed-methods systematic review. We searched PsycINFO, MEDLINE, CINAHL, and ASSIA from databases and included studies reporting experiences and/or impact of PCOM use among people with dementia, family carers, and/or practitioners. Groen Van de Ven's model of collaborative deliberation informed the elements of shared decision-making in dementia care in the abstraction, analysis, and interpretation of data. Data were narratively synthesized to develop the logic model. Setting: Studies were conducted in long-term care, mixed settings, emergency department, general primary care, and geriatric clinics. Participants: A total of 1064 participants were included in the review. Results: Ten studies were included. PCOMs can facilitate shared decision-making through "knowing the person," "identifying problems, priorities for care and treatment and goal setting," "evaluating decisions", and "implementation considerations for PCOM use." Weak evidence on the impact of PCOMs to improve communication between individuals and practitioners, physical function, and activities of daily living. Conclusions: PCOMs can enable shared decision-making and impact outcomes through facilitating collaborative working between the person's network of family and practitioners to identify and manage symptoms and concerns. The constructed logic model demonstrates the key mechanisms to discuss priorities for care and treatment, and to evaluate decisions and outcomes. A future area of research is training for family carers to use PCOMs with practitioners.

COVID-19: Impact on Pediatric Palliative Care.

CONTEXT: Children and young people with life-limiting or life-threatening conditions and their families are potentially vulnerable during COVID-19 lockdowns due to pre-existing high clinical support needs and social participation limitations. OBJECTIVES: To explore the impact of the COVID-19 pandemic and lockdowns on this population. METHODS: Sub-analysis of an emergent COVID-19 related theme from a larger semi-structured interview study investigating priority pediatric palliative care outcomes. One hundred and six United Kingdom-wide purposively-sampled Children and young people with life-limiting or life-threatening conditions, parent/carers, siblings, health professionals, and commissioners. RESULTS: COVID-19 was raised by participants in 12/44 interviews conducted after the United Kingdom's first confirmed COVID-19 case. Key themes included loss of vital social support, disruption to services important to families, and additional psychological distress. CONCLUSION: Continued delivery of child- and family-centered palliative care requires innovative assessment and delivery of psycho-social support. Disruptions within treatment and care providers may compound support needs, requiring cordination for families facing multiagency delays.

Inter-Rater Reliability of the Phase of Illness Tool in Pediatric Palliative Care.

BACKGROUND: Phase of Illness is used to describe the stages of a patient's illness in the palliative care setting. Categorization is based on individual needs, family circumstances, and the adequacy of a care plan. Substantial (κ = .67) and moderate (κ = .52) inter-rater reliability is demonstrated when categorizing adults; however, there is a lack of similar studies in pediatrics. OBJECTIVE: To test the inter-rater reliability of health-care professionals when assigning pediatric palliative care patients to a Phase of Illness. Furthermore, to obtain user views on phase definitions, ease of assignment, feasibility and acceptability of use. METHOD: A prospective cohort study in which up to 9 health-care professionals' independently allocated 80 pediatric patients to a Phase of Illness and reported on their experiences. This study took place between June and November 2017. RESULTS: Professionals achieved a moderate level of agreement (κ = 0.50). Kappa values per phase were as follows: stable = 0.63 (substantial), unstable = 0.26 (fair), deteriorating = 0.45 (moderate), and dying = 0.43 (moderate). For the majority of allocations, professionals report that the phase definitions described patients very well (76.1%), and they found it easy to assign patients (73.5%). However, the unstable phase caused the most uncertainty. CONCLUSION: The results of this study suggest Phase of Illness is a moderately reliable, acceptable, and feasible tool for use in pediatric palliative care. Current results are similar to those found in some adult studies. However, in a quarter of cases, users report some uncertainty in the application of the tool, and further study is warranted to explore whether suggested refinements improve its psychometric properties.

The 'surprise' question in paediatric palliative care: A prospective cohort study.

BACKGROUND: The question 'would you be surprised if this patient died in the next 12-months' is widely used for identifying adult patients in the last year of life. However, this has not yet been studied in children. AIM: To assess the prognostic accuracy of the surprise question when used by a multidisciplinary team to predict survival outcomes of children with life-limiting conditions over a 3 and 12 month period. DESIGN: A prospective cohort study. SETTING/PARTICIPANTS: Six multidisciplinary team members working in a children's hospice answered a 3 and 12 month surprise question about 327 children who were either newly referred or receiving care at the hospice between 2011 and 2013. RESULTS: The prognostic accuracy of the multidisciplinary team for the 3 (and 12)month surprise question were: sensitivity 83.3% (83.3%), specificity 93.2% (70.7%), positive predictive value 41.7% (23.6%), negative predictive value 99% (97.5%) and accuracy 92.6% (71.9%). Patients with a 'no' response had an increased risk of death at 3 (hazard ratio, 22.94, p ⩽ 0.001) and 12 months (hazard ratio, 6.53, p ⩽ 0.001). CONCLUSION: The surprise question is a highly sensitive prognostic tool for identifying children receiving palliative care who are in the last 3 and 12 months of life. The tool is accurate at recognising children during stable periods demonstrated through a high negative predictive value. In practice, this tool could help identify children who would benefit from specialist end of life care, act as a marker to facilitate communications on advance care planning and assist in resource allocation.

The use of rapid onset fentanyl in children and young people for breakthrough cancer pain.

BACKGROUND AND AIMS: No published studies have looked at the dosing and use of rapid onset fentanyl preparations in children. The primary aim of this study was to assess whether there is a correlation between effective dose of rapid onset fentanyl and background oral morphine equivalent analgesia in children less than 18 years old. Secondary objectives included establishing whether there is a correlation between effective dose of rapid onset fentanyl and age and weight. Reported side effects were also reviewed. METHODS: This study is a retrospective case note review of all children less than 18 years old who received rapid onset fentanyl products in a tertiary paediatric oncology centre in England between 2010 and 2015. Correlations were analysed using Spearman's correlation coefficient as data was non-parametric. RESULTS: Data on 26 children (5-17 yrs; 13-100kg) was analysed. The most common diagnosis in children being given rapid onset fentanyl products was a solid tumour (84.6%). Eleven children used sublingual tablets, 17 used lozenges and one used a fentanyl nasal spray (three patients used two different preparations). The only significant correlation found was between dose of fentanyl lozenge and weight (rs=0.81, p<0.001). Very few side effects were reported with the most frequent being nausea (8%) and sleepiness (8%). CONCLUSIONS: Fentanyl lozenges seem to be safe and well tolerated in children as young as five years old, weighing as little as 13kg. Results suggest that children should always be started on the lowest available dose of chosen preparation and that this dose should be titrated according to response. This study demonstrates that there is no correlation between background opioid dose and effective dose of rapid onset fentanyl in children. This mirrors findings of similar studies in adults. There was a strong correlation between effective dose of fentanyl lozenge and weight. This may be in part due to clinicians being more inclined to increase fentanyl lozenge doses as the child is in control of when they have had enough medication. In contrast, buccal tablets are absorbed quickly and the child always receives the full dose, making clinicians more reluctant to titrate the dose. IMPLICATIONS: This article presents initial evidence for feasibility and tolerability of fentanyl lozenges in children as young as five years old, who are on relatively low doses of background opioids. This could be of interest to clinicians who are looking for alternatives to oral opioids to manage breakthrough pain in children with cancer.

Health-related quality-of-life outcome measures in paediatric palliative care: A systematic review of psychometric properties and feasibility of use.

BACKGROUND: The number of children worldwide requiring palliative care services is increasing due to advances in medical care and technology. The use of outcome measures is important to improve the quality and effectiveness of care. AIM: To systematically identify health-related quality-of-life outcome measures that could be used in paediatric palliative care and examine their feasibility of use and psychometric properties. DESIGN: A systematic literature review and analysis of psychometric properties. DATA SOURCES: PsychInfo, Medline and EMBASE were searched from 1 January 1990 to 10 December 2014. Hand searches of the reference list of included studies and relevant reviews were also performed. RESULTS: From 3460 articles, 125 papers were selected for full-text assessment. A total of 41 articles met the eligibility criteria and examined the psychometric properties of 22 health-related quality-of-life measures. Evidence was limited as at least half of the information on psychometric properties per instrument was missing. Measurement error was not analysed in any of the included articles and responsiveness was only analysed in one study. The methodological quality of included studies varied greatly. CONCLUSION: There is currently no 'ideal' outcome assessment measure for use in paediatric palliative care. The domains of generic health-related quality-of-life measures are not relevant to all children receiving palliative care and some domains within disease-specific measures are only relevant for that specific population. Potential solutions include adapting an existing measure or developing more individualized patient-centred outcome and experience measures. Either way, it is important to continue work on outcome measurement in this field.

Palliative and end-of-life care for children with diffuse intrinsic pontine glioma: results from a London cohort study and international survey.

BACKGROUND: More than 90% of patients with diffuse intrinsic pontine glioma (DIPG) will die within 2 years of diagnosis. Patients deteriorate rapidly during the disease course, which severely impairs their quality of life. To date, no specific research on this clinically important subject has been conducted. This study aimed to compile an inventory of symptoms experienced, interventions applied, and current service provision in end-of-life care for DIPG. METHODS: We performed a retrospective cohort study of children with DIPG, aged 0-18 years, who received treatment under the care of 2 London hospitals. Symptoms, interventions, and services applied during the 12 weeks before death were analyzed. In addition, we conducted a global questionnaire-study among health care professionals. RESULTS: In more than 78% of DIPG patients, problems concerning mobility, swallowing, communication, consciousness, and breathing arose during end-stage disease. Supportive drugs were widely prescribed. The use of medical aids was only documented in <15% of patients. Palliative and end-of-life care was mostly based on the health care professional's experience; only 21% of the questionnaire respondents reported to have a disease-specific palliative care guideline available. CONCLUSIONS: This research assessed the current state of palliative and end-of-life care for children with DIPG. Our results show the variability and complexity of symptoms at end-stage disease and the current lack of disease-specific guidelines for this vulnerable group of patients. This first descriptive paper is intended to act as a solid basis for developing an international clinical trial and subsequent guideline to support high-quality palliative and end-of-life care.

Supportive care for children and young people during cancer treatment.

Children, young people and their families come into contact with a large number of health and social care professionals within hospital, education and community settings following a cancer diagnosis. The multiprofessional team best supports these families through an open and communicative approach to care. The side effects of cancer treatment include fever, infection, gastrointestinal upset, altered body image and psychological impact on children, young people and their families. Debate continues as to how children, young people, their siblings and parents cope with childhood cancer and its treatment due to its impact on family quality of life. It is considered that the short- and long-term wellbeing of children, young people and their families during treatment is best supported by continuing routine daily activities, such as attending nursery, school or college.