A pragmatic, open-label, randomized controlled trial of Plasma-Lyte-148 versus standard intravenous fluids in children receiving kidney transplants (PLUTO).

Acute electrolyte and acid-base imbalance is experienced by many children following kidney transplant. This is partly because doctors give very large volumes of artificial fluids to keep the new kidney working. When severe, fluid imbalance can lead to seizures, cerebral edema and death. In this pragmatic, open-label, randomized controlled trial, we randomly assigned (1:1) pediatric kidney transplant recipients to Plasma-Lyte-148 or standard of care perioperative intravenous fluids (predominantly 0.45% sodium chloride and 0.9% sodium chloride solutions). We then compared clinically significant electrolyte and acid-base abnormalities in the first 72 hours post-transplant. The primary outcome, acute hyponatremia, was experienced by 53% of 68 participants in the Plasma-Lyte-148 group and 58% of 69 participants in the standard fluids group (odds ratio 0·77 (0·34 - 1·75)). Five of 16 secondary outcomes differed with Plasma-Lyte-148: hypernatremia was significantly more frequent (odds ratio 3·5 (1·1 - 10·8)), significantly fewer changes to fluid prescriptions were made (rate ratio 0·52 (0·40-0·67)), and significantly fewer participants experienced hyperchloremia (odds ratio 0·17 (0·07 - 0·40)), acidosis (odds ratio 0·09 (0·04 - 0·22)) and hypomagnesemia (odds ratio 0·21 (0·08 - 0·50)). No other secondary outcomes differed between groups. Serious adverse events were reported in 9% of participants randomized to Plasma-Lyte-148 and 7% of participants randomized to standard fluids. Thus, perioperative Plasma-Lyte-148 did not change the proportion of children who experienced acute hyponatremia compared to standard fluids. However fewer fluid prescription changes were made with Plasma-Lyte-148, while hyperchloremia and acidosis were less common.

Medication adherence in pediatric kidney transplantation: How to build a bridge over troubled water.

Adhering to the immunosuppressive regimen remains one of the biggest challenges for children and adolescents after kidney transplantation. The first paper on nonadherence, co-authored by Dr. Fine, appeared in Pubmed over 45 years ago. Since then, many clinicians and researchers tried to better understand nonadherence and are looking for effective ways to support young people in implementing the complex medication regimen in their daily lives. As a tribute to Dr. Fine, we conducted a comprehensive review providing an overview of adherence-enhancing interventions in the field of pediatric kidney transplantation, thereby focusing on strategies that not only are effective but can also be embedded in daily clinical practice successfully and sustainably. This overview is preceded by a discussion about how to find out who is in need of supportive interventions. We will also argue that interventions should already start before pediatric kidney transplantation and discuss how to decide whether or not a young patient with nonadherence-induced graft loss should undergo retransplantation. We hope this comprehensive overview will rekindle the hope that we can finally turn the tide and beat one of pediatric kidney transplantation's main enemies.

Allied health and nursing practices in pediatric solid organ transplantation: An international survey.

INTRODUCTION: With improved survival in pediatric solid organ transplantation (SOT) care has focused on optimizing functional, developmental, and psychosocial outcomes, roles often supported by Allied Health and Nursing professionals (AHNP). However, there is a scarcity of research examining frameworks of clinical practice. METHODS: The International Pediatric Transplant Association AHNP Committee developed and disseminated an online survey to transplant centers as a quality improvement project to explore AHNP practice issues. Participant responses were characterized using descriptive statistics, and free-text comments were thematically analyzed. Responses were compared across professional groups; Group 1: Advanced Practice Providers, Group 2: Nursing, Group 3: Allied Health. RESULTS: The survey was completed by 119 AHNP from across the globe, with responses predominantly (78%) from North America. Half of respondents had been working in pediatric transplant for 11+ years. Two-thirds of respondents were formally funded to provide transplant care; however, of these not funded, over half (57%) were allied health, compared to just 6% of advance practice providers. Advanced practice/nursing groups typically provided care to one organ program, with allied health providing care for multiple organ programs. Resource constraints were barriers to practice across all groups and countries. CONCLUSION: In this preliminary survey exploring AHNP roles, professionals provided a range of specialized clinical care. Challenges to practice were funding and breadth of care, highlighting the need for additional resources, alongside the development of clinical practice guidelines for defining, and supporting the role of AHNP within pediatric SOT. Professional organizations, such as IPTA, can offer professional advocacy.

Safety of virtual reality use in children: a systematic review.

The study aimed to systematically review available literature regarding the safety of virtual reality (VR) use via head-mounted display in children under 14 years of age. The study was a systematic review including all study designs. A search was conducted in January 2023 in PubMed and EMBASE using key terms referring to 'virtual reality', 'paediatrics' and 'safety'.  Following title and abstract and full-text screening, data were extracted and a narrative synthesis undertaken. Twenty-six studies met criteria for inclusion in the final review.  Limited data suggest that VR may cause mild cybersickness symptoms (not severe enough to cause participants to discontinue use of VR) and that for children with existing amblyopia using VR may result in double vision, which resolves on cessation of VR exposure.  Two randomised control trials did not report differences in adverse events between the intervention (VR use) and control groups. Reporting of safety data was poor; only two studies used a validated measure, and in the remaining studies, it was often unclear how adverse events were defined (if at all), how they were categorised in terms of severity and how they were recorded.     Conclusion: There is limited evidence regarding any potential harms from short exposure to VR in children under 14 years under supervision. Additional research is required to understand increases in cybersickness during and after VR exposure, and the impact of repeated exposure.  Adverse events need to be accurately and routinely recorded to determine any hitherto unknown safety concerns for children < 14 years using VR. What is Known: • Virtual reality (VR) is increasingly being applied in paediatrics, with benefits in terms of anxiety reduction, improved pain management associated with procedures, as an adjunct to physiotherapy and supporting treatments in autistic spectrum disorder.. • Safety guidance in relation to VR use, particularly in younger children, is limited. What is New: • A systematic review of available literature regarding the safety of VR use via head-mounted display in children under 14 years of age demonstrated limited evidence regarding any potential harms from short exposure to VR.. • Studies rarely report safety data and adverse side effects are poorly defined, measured and/or reported. • The lack of a validated measure for evaluating VR-associated symptoms in children compounds the challenging ethical issues of undertaking research into the effects of VR on younger children.

The Impact of Morbidities Following Pediatric Cardiac Surgery on Family Functioning and Parent Quality of Life.

We previously selected and defined nine important post-operative morbidities linked to paediatric cardiac surgery, and prospectively measured their incidence following 3090 consecutive operations. Our aim was to study the impact of these morbidities on family functioning and parental quality of life over 6 months in a subset of cases. As part of a prospective case matched study in five of the ten children's cardiac centers in the UK, we compared outcomes for parents of children who had a 'single morbidity', 'multiple morbidities', 'extracorporeal life support (ECLS)' or 'no morbidity'. Outcomes were evaluated using the PedsQL Family impact module (FIM) at 6 weeks and 6 months post-surgery. Outcomes were modelled using mixed effects regression, with adjustment for case mix and clustering within centers. We recruited 340 patients with morbidity (60% of eligible patients) and 326 with no morbidity over 21 months. In comparison to the reference group of 'no morbidity', after adjustment for case mix, at 6 weeks parent health-related quality of life (HRQoL) and total FIM sores were lower (worse) only for ECLS (p < 0.005), although a higher proportion of parents in both the ECLS and multi-morbidity groups had low/very low scores (p < .05). At 6 months, parent outcomes had improved for all groups but parent HRQoL and total score for ECLS remained lower than the 'no morbidity' group (p < .05) and a higher proportion of families had low or very low scores in the ECLS (70%) group (p < .01). Post-operative morbidities impact parent HRQoL and aspects of family functioning early after surgery, with this impact lessening by 6 months. Families of children who experience post-operative morbidities should be offered timely psychological support.

Attachment style and children and young people with chronic dermatological conditions.

BACKGROUND: Dermatological conditions are common in childhood and, in their more severe forms, can cause pain, disability and social marginalisation. Despite attachment being a known factor contributing to psychological and physiological development in childhood and several adult studies showing associations between attachment and dermatology outcomes (Tomas-Aragones, 2018), attachment in young dermatology patients has not been investigated. OBJECTIVES: This study examined if (1) 8-16-year-olds with chronic dermatological conditions were more likely to show attachment insecurity than general population peers; (2) attachment style was linked to psychological functioning; and (3) facial involvement was associated with attachment insecurity. METHOD: One hundred and twenty-two 8-16-year-olds attending a specialist paediatric dermatological service were compared on the Child Attachment Interview (CAI) to general population data. The Strengths and Difficulties Questionnaire (SDQ) was used to measure psychosocial functioning. RESULTS: The dermatology group was significantly more likely to be insecurely attached than their general population peers (χ2 [1] = 4.76, p < .05). The secure group self-reported significantly better psychological functioning on all indices compared with the insecure group (Total Difficulties: F[1,89] = 15.30, p < .001). There were no significant differences between secure and insecure groups on parent-reported psychological measures (Total Difficulties: F[1,94] = 0.67, p = .42). Children with facial involvement were not significantly more likely to be insecurely attached. CONCLUSIONS: Increased risk of attachment insecurity, particularly in the anxious pre-occupied category, as well as an association between attachment and psychosocial functioning resonated with adult studies suggesting that further research about the role of attachment with young dermatology patients is warranted.

What are the experiences of non-invasive ventilation for sleep-disordered breathing in children and young people and what outcomes matter? Perspectives of children and young people, their parents and the staff who care for them.

BACKGROUND: Non-invasive ventilation (NIV) for sleep-disordered breathing (SDB) in children and young people (CYP) can result in multiple health outcomes; however, adherence to NIV can be challenging. Suboptimally treated SDB may increase the risk of adverse consequences. Placing children's and parents' goals at the core of their SDB treatment may support adherence to NIV. To identify these health outcomes, it is necessary to gain a greater understanding of CYP's experiences of using NIV, whether they perceive any benefits from NIV use, as well as the outcomes that their parents and NIV staff identify as important. METHODS: Semi-structured qualitative interviews were conducted with nine CYP (aged 4-16 + years), 13 parents and nine healthcare professionals (HCPs); verbatim transcripts were analysed using Framework Analysis. RESULTS: CYP predominantly reported an improvement in levels of energy, focus and ability to concentrate whereas parents also identified outcomes of mood and behaviour. The majority of children showed understanding of the reasons for being prescribed NIV. A subset of children did not notice their SDB. The health outcomes identified by HCPs and parents that could result from improved overnight gas exchange are subjective measures that rely on parent and child report. Measuring these health outcomes focussed on the impact of improved sleep rather than measuring improved sleep itself. CONCLUSIONS: It is important for HCPs administering NIV to ascertain whether CYP have noticed any of their sleep-disordered breathing symptoms and any improvements from using NIV, including the relationship between benefits and side effects. Focussing on promoting understanding for CYP who are unable to link their wellbeing to their previous night's sleep may be futile and HCP strategies should concentrate on the process of tolerating the device. Parents, CYP and HCPs should collaborate to identify treatment goals specifically tailored for the child and monitor any progress against these goals.

Being a child with intellectual disabilities in hospital: The need for an individualised approach to care.

BACKGROUND: There is limited qualitative research focussed specifically on what it is like for children and young people with intellectual disabilities coming into hospital, with much of the evidence-base being about those with Autism Spectrum Condition or adults with intellectual disabilities. AIM: To share rich detail of the emotional and physical impact on children and young people with intellectual disabilities of attending hospital, from their own and their parent's perspective. METHODS: Talking Mats interviews, sticker survey and photography with children and young people with intellectual disabilities, and in-depth interviews, hospital diaries and photography with their parents. RESULTS AND CONCLUSIONS: The multiple and compounding layers of complexity surrounding hospital care of children and young people with intellectual disabilities resulted in challenges associated with loss of familiarity and routine, undergoing procedures, managing sensory overload, managing pain and having a lack of safety awareness. An individualised approach to their care is needed.

Behavioral and Emotional Outcomes in Children with Congenital Heart Disease: Effects of Disease Severity, Family Life Stress, Disease-Related Chronic Stress, and Psychosocial Adaptation.

OBJECTIVE: To evaluate whether effects of congenital heart disease (CHD) severity and family life stress on behavioral and emotional functioning are mediated by disease-related chronic stress and psychosocial adaptation. STUDY DESIGN: A cross-sectional analysis of the Pediatric Cardiac Quality of Life Inventory Testing Study was performed. Relationships between CHD severity (comprising 3 groups: mild heart disease, moderate biventricular disease, and single ventricle) and family life stress, on patient- and parent disease-related chronic stress, psychosocial adaptation, and behavioral-emotional outcomes were assessed using structural equation modeling. Patient and parent models were reported separately. RESULTS: There were 981 patient-parent dyads: 22% had mild heart disease, 63% biventricular, and 15% single ventricle; 19% of families reported moderate to major family life stress. Path models revealed that CHD severity and family life stress were mediated by disease-related chronic stress and psychosocial adaptation factors (R2 = 0.18-0.24 for patient outcomes and R2 = 0.33-0.34 for parent outcomes, P < .001, respectively). CONCLUSIONS: The effects of greater CHD severity and family life stress on behavioral-emotional outcomes were mediated by worse disease-related chronic stress and psychosocial adaptation factors. Both disease-related chronic stress and psychosocial adaptation factors may be targets for interventions to improve behavioral and emotional outcomes.

Differences in Quality of Life in Children Across the Spectrum of Congenital Heart Disease.

OBJECTIVE: To create complexity groups based upon a patient's cardiac medical history and to test for group differences in health-related quality of life (HRQOL). METHODS: Patients 8-18 years with congenital heart disease (CHD) and parent-proxies from the Pediatric Cardiac Quality of Life Inventory (PCQLI) Testing Study were included. Outcome variables included PCQLI Total, Disease Impact, and Psychosocial Impact scores. Using a patient's medical history (cardiac, neurologic, psychological, and cognitive diagnosis), latent class analysis (LCA) was used to create CHD complexity groups. Covariates included demographics and burden of illness (number of: school weeks missed, physician visits in the past year, and daily medications). Generalized estimation equations tested for differences in burden of illness and patient and parent-proxy PCQLI scores. RESULTS: Using 1482 CHD patients (60% male; 84% white; age 12.3 ± 3.0 years), latent class analysis (LCA) estimates showed 4 distinct CHD complexity groups (Mild, Moderate 1, Moderate 2, and Severe). Increasing CHD complexity was associated with increased risk of learning disorders, seizures, mental health problems, and history of stroke. Greater CHD complexity was associated with greater burden of illness (P < .01) and lower patient- and parent-reported PCQLI scores (P < .001). CONCLUSIONS: LCA identified 4 congenital heart disease (CHD) complexity groupings. Increasing CHD complexity was associated with higher burden of illness and worse patient- and parent-reported HRQOL.

Cultural and other beliefs as barriers to pediatric solid organ transplantation.

Organ shortage for transplantation is a global problem for both adult and pediatric recipients and this is particularly apparent in certain areas of the world and within specific communities. Cultural and religious beliefs can influence both a decision by a potential donor and/or their family to donate as well as a potential recipient's or their family's decision to accept the need for transplantation, agree to be listed and accept an organ. Globally, there are cultural and religious diversities that can present significant challenges for the transplant professional, particularly those whose practice is based in multicultural communities. In the pediatric population, in particular, barriers to organ transplantation related to culture and religion have not been well-described, with resulting implications for how they should or could be effectively addressed. Therefore, this review was undertaken to elucidate cultural and religious barriers to pediatric organ transplantation and, where feasible, to identify facilitators and strategies for overcoming these barriers.

Quality of life and psychosocial outcomes of adults who were pediatric solid organ transplant recipients: A systematic review.

BACKGROUND: The number of pediatric SOT recipients surviving into adulthood is increasing. Thus, understanding their psychosocial and QoL outcomes is important. We conducted a systematic review to collate existing literature examining QoL outcomes (physical functioning, psychological functioning, social functioning), as well as risk and protective factors associated with QoL, among adults who underwent SOT during childhood. METHODS: A systematic search of five databases, from inception to January 6, 2021, was conducted to identify articles that reported on QoL outcomes for adults (≥18-year of age) who received a SOT during childhood (<19-year of age). RESULTS: Twenty-five articles met inclusion criteria. Studies examined QoL across a range of SOT populations (liver, kidney, heart). QoL and psychosocial outcomes were variable; however, the majority of studies indicated QoL in this population to be similar to the general population, or at least similar to other chronic illness groups, with the exception of physical and social functioning. Factors related to a more optimal medical course, younger age at transplant and follow-up, and positive psychosocial functioning, were found to be predictive of better QoL outcomes. CONCLUSIONS: While several studies indicated QoL to be similar to the general population, the literature is limited in both quantity and quality. No study employed prospective, longitudinal methodologies to systematically evaluate QoL over time and few studies utilized normative-based measures of QoL. Furthermore, several SOT groups were under-represented in the literature (e.g., lung, intestine, multi-visceral). Nonetheless, findings have implications for intervention and clinical decision-making.

An international survey of allied health & nursing professionals during the COVID-19 pandemic: Perspectives on facilitators of & barriers to care.

BACKGROUND: Allied health and nursing professionals (AHNP) are integral members of transplant teams. During the COVID-19 pandemic, they were required to adapt to changes in their clinical practices. The goal of the present study was to describe AHNP perceptions concerning the impact of the pandemic on their roles, practice, and resource allocation. METHODS: An online survey was distributed globally via email by the International Pediatric Transplant Association to AHNP at transplant centers from September to December 2020. Responses to open-ended questions were collected using an electronic database. Using a thematic analysis approach, coding was conducted by three independent coders who identified patterns in responses, and discrepancies were resolved through discussion. RESULTS: The majority of respondents (n = 119) were from North America (78%), with many other countries represented (e.g., the United Kingdom, Europe, Australia, New Zealand, South Africa, and Central and South America). Four main categories of impacts were identified: (1) workflow changes, (2) the quality of the work environment, (3) patient care, and (4) resources. CONCLUSIONS: Participants indicated that the pandemic heightened existing barriers and resource challenges frequently experienced by AHNP; however, the value of team connections and opportunities afforded by technology were also highlighted. Virtual care was seen as increasing healthcare access but concerns about the quality and consistency of care were also expressed. A notable gap in participant responses was identified; the vast majority did not identify any personal challenges connected with the pandemic (e.g., caring for children while working remotely, personal stress) which likely further impacted their experiences.

Emergency Inter-Hospital Transfer of Children to PICUs in the United Kingdom: Qualitative Exploration of Parents' Experiences of Retrieval Teams.

OBJECTIVES: Each year in the United Kingdom there are around 5,000 inter-hospital transfers of critically ill children into PICUs. There are few published descriptions of what this experience is like for parents. The objective was to describe parents' experiences of the inter-hospital transfer of their critically ill child to a PICU. DESIGN: Qualitative in-depth interviews. SETTING: Twenty-four PICUs in England and Wales. PARTICIPANTS: Parent interview participants ( n = 30) were purposively sampled from a larger pool of parent questionnaire respondents to create a sample diverse in child's age, presenting medical illness, retrieval team and whether a parent traveled in the ambulance. MEASUREMENT AND MAIN RESULTS: Open-ended semi-structured interviews using topic guides to encourage parents to describe their experiences of transfer. Interviews were audio recorded, transcribed verbatim and thematically analyzed using Framework Analysis. Parents' perceptions of transport staff as confident and competent through observation of clinical care, and positive communication experiences during the transfer process, were related to feelings of trust and being supported, as well as relief from distress. Parents varied in their needs for conversation and support. Parents who did not travel in the ambulance had fewer opportunities to interact with the transport team and experienced different challenges in the period prior to their child's admission to the PICU. CONCLUSIONS: Retrieval teams can influence how parents experience their child's emergency transfer to the PICU, offering parents proximity to knowledgeable staff. Satisfaction may be related to matching parents' needs. Understanding parents' needs and optimizing opportunities for effective communication between parents and staff are beneficial to parents.

The Relationship Between Postoperative Complications and Children's Quality of Life: A Mediation Analysis to Explore the Role of Family Factors.

OBJECTIVES: To explore whether postoperative morbidities after pediatric cardiac surgery affected children's health-related quality of life (HRQOL) at 6 months, through potentially modifiable parental psychological factors. DESIGN: We undertook a mediation analysis, to explore the causal pathway, based on data from a prospective, case-matched cohort study. PATIENTS: Six hundred sixty-six children undergoing cardiac surgery. SETTING: Five centers in the United Kingdom. INTERVENTIONS: No intervention. MEASUREMENTS AND MAIN RESULTS: Cases of morbidity were identified early after pediatric cardiac surgery, and matched controls with no morbidities were identified at discharge. Four mediators were assessed at 6 weeks after surgery, using the PedsQL Family Impact Module (Parent HRQOL and Family Function) and the PHQ-4 (Anxiety and Depression). The study outcome of child HRQOL was assessed at 6 months with the PedsQL. Of 666 children, 408 (65% of those surviving) contributed to the primary outcome. Children who had extracorporeal life support (ECLS) ( n = 11) ( p < 0.05) and multiple morbidities ( n = 62) ( p < 0.01) had worse 6-month HRQOL than those with a single morbidity ( n = 125) or no morbidity ( n = 209). After adjustment for case mix complexity and sociodemographic variables, there were significant indirect effects of parent HRQOL at 6 weeks, on the PedsQL Total Score (ECLS, -5.1 [-8.4 to -1.8]; p = 0.003; multiple morbidities, -2.1 [-3.7 to -0.5]; p = 0.01), PedsQL Physical Score (ECLS, -5.1 [-8.7 to -1.4]; p = 0.007; multiple morbidities, -2.1 [-3.8 to -0.4]; p = 0.016), and PedsQL Psychosocial Score (ECLS: -5.3 [-8.7 to -1.8); p = 0.003; multiple morbidities, -2.2 [-3.9 to -0.5]; p = 0.01). The proportion of the total effect of ECLS and multiple morbidity on the study outcomes mediated through parent HRQOL ranges between 18% and 61%. There was no evidence that the other three mediators had indirect effects on the study outcome. CONCLUSIONS: Parental HRQOL at 6 weeks after surgery contributes to child HRQOL at 6 months, among those with the severest types of morbidity, and as such should be a target for future interventions.

Long-Term Quality of Life in Congenital Heart Disease Surgical Survivors: Multicenter Retrospective Study of Surgical and ICU Explanatory Factors.

OBJECTIVES: Greater congenital heart disease (CHD) complexity is associated with lower health-related quality of life (HRQOL). There are no data on the association between surgical and ICU factors and HRQOL in CHD survivors. This study assess the association between surgical and ICU factors and HRQOL in child and adolescent CHD survivors. DESIGN: This was a corollary study of the Pediatric Cardiac Quality of Life Inventory (PCQLI) Testing Study. SETTING: Eight pediatric hospitals participating in the PCQLI Study. PATIENTS: Patients in the study had the Fontan procedure, surgery for tetralogy of Fallot (TOF), and transposition of the great arteries (TGAs). MEASUREMENTS AND MAIN RESULTS: Surgical/ICU explanatory variables were collected by reviewing the medical records. Primary outcome variables (PCQLI Total patient and parent scores) and covariates were obtained from the Data Registry. General linear modeling was used to create the multivariable models. There were 572 patients included: mean ± sd of age 11.7 ± 2.9 years; CHD Fontan 45%, TOF/TGA 55%; number of cardiac surgeries 2 (1-9); and number of ICU admissions 3 (1-9). In multivariable models, lowest body temperature on cardiopulmonary bypass (CPB) was negatively associated with patient total score (p < 0.05). The total number of CPB runs was negatively associated with parent-reported PCQLI Total score (p < 0.02). Cumulative days on an inotropic/vasoactive drug in the ICU was negatively associated with all patient-/parent-reported PCQLI scores (p < 0.04). Neurological deficit at discharge was negatively associated with parent-reported PCQLI total score (p < 0.02). The variance explained by these factors ranged from 24% to 29%. CONCLUSIONS: Surgical/ICU factors, demographic, and medical care utilization variables explain a low-to-moderate amount of variation in HRQOL. Research is needed to determine whether modification of these surgical and ICU factors improves HRQOL, and to identify other factors that contribute to unexplained variability.

Translation, cultural adaptation and validation of a patient-reported experience measure for children.

BACKGROUND: There is no national, validated, generic patient-reported experience measure (PREM) for children under 15 years of age in Sweden. A recent cross-sectional study found no consensus in how children's voices are heard in paediatric health care, as well as a lack of validated questionnaires. AIM: The aim of this study is to translate, adapt and validate the six versions of the Children's and Young People's PREM for use in a Swedish health care context. DESIGN: An exploratory sequential mixed-method design including cognitive interviews and content validity index (CVI) was used. The interviews focused on evaluating children's understanding of the questionnaire, and the CVI was used to further adjust the relevance of the questionnaire. PARTICIPANTS: A convenience sample of 62 children participated in the cognitive interviews and an additional convenience sample of 42 children was included in the CVI testing. The children, aged 8-16 years, were attending routine visits at paediatric departments in a county hospital and a children's hospital in the mid-Sweden region between October 2020 and June 2022. RESULTS: The translation, adaptation and validation process identified several issues regarding the understanding of the questionnaire in a Swedish context. Adaptations were made based on issues related to context, wording and the structure of the questions. CVI testing resulted in the removal of 3-10 questions in each of the different versions of the questionnaire. CONCLUSION: The study has resulted in six face- and content-validated Swedish versions of the questionnaire ready for pilot testing. Although the versions of the original questionnaire were developed in collaboration with children in the United Kingdom, this did not mean that they could automatically be used in a Swedish health care context. This study confirms the importance of a rigorous process of adaptation and validation to ensure quality and applicability to children accessing health care in different countries. PATIENT OR PUBLIC CONTRIBUTION: Children's views have guided the development of the original instrument and its adaptation to the Swedish health care context. Due to the strong patient involvement in the process of developing the Swedish versions of the questionnaire, the research group made a pragmatic decision to have no other patient contribution in the study.

An improved congenital heart assessment tool: a quality improvement outcome.

BACKGROUND: CHD was the most prevalent congenital anomaly (60.9 per 10,000, 95% CI 59.0-62.8) in England in 2018, with 1767 babies born with severe cardiac defects. The 30-day survival rates for complex procedures continue to improve; however despite care advances, the early post-operative period and first year of life remain a critical time for these infants. The Congenital Heart Assessment Tool was developed to support parental decision-making, standardise care provision, improve communication, and the safety and quality of care. AIM: To further evaluate the Congenital Heart Assessment Tool. DESIGN: A four centre collaborative mixed-methods quality improvement project funded by The Health Foundation, involving eight phases conducted during 2016-2018. Phases six to eight (clinical simulation exercise, parent workshop, and updated tool) are reported in this paper. RESULTS: Four themes emerged from the clinical simulation exercise (phase six) including: improving documentation; preparation of parents; preparation of health care professionals; and communication. One main theme emerged from the parent workshop (phase seven): "what parents know versus what professionals know [about CHD]". CONCLUSION: These phases further validated the effectiveness of the CHATm in terms of triggering amber and red indicators and demonstrated parents' ability to identify deterioration in their infant's clinical condition. Recommendations arising from the quality improvement project enabled the project team to create an updated version of the Congenital Heart Assessment Tool, CHAT2.

Talking with children and young people with 22q11DS about their mental health, behaviour, learning and communication.

BACKGROUND: 22q11DS11.2 deletion syndrome (22q11DS) is a complex multisystem syndrome characterized by physical abnormalities, psychiatric comorbidities and cognitive deficits. The views of children and young people (CYP) about the challenges associated with their mental health, behaviour, learning and communication difficulties have not been reported. The aim of this study was to address this gap and to understand whether they had help and support with these and their views of this. METHODS: A three-phase mixed-methods study was undertaken, involving interviews with CYP with 22q11DS, a follow-up survey for those aged 11-25 years and a stakeholder workshop at which CYP presented their views of living with 22q11DS to health professionals and parents. Interview transcripts were thematically analysed, and non-parametric statistics were used to analyse survey data. RESULTS: The interviews (n = 13) and survey (n = 32) indicated a mixed picture, with some CYP not reporting ongoing problems; others who had problems received help but a sizeable proportion had unmet needs and wanted to receive help. Two-thirds reported often experiencing negative feelings, and almost half had difficulties with social interactions. Family members were the main sources of support, with teaching assistants identified as an important support at school. CONCLUSIONS: The emotional impact of 22q11DS on CYP can be significant: They often do not understand the consequences of having 22q11DS and are frequently not given strategies to understand and manage their feelings, behaviour or problems. This leads to a range of emotions that manifest in different ways at home and at school. CYP are able to talk about the impact of different aspects of 22q11DS on them, facilitated by the use of creative methods, but they differ in how the condition affects them and their perceptions about that. It is imperative that CYP themselves are asked about their experiences, feelings and needs to ensure tailoring of interventions to their individual requirements.

'Wanting no regrets': Parental decision making around selective dorsal rhizotomy.

BACKGROUND: Selective dorsal rhizotomy (SDR) is an irreversible neurosurgical procedure used to reduce spasticity while aiming to improve gross motor function for children with cerebral palsy (CP). Little research has explored the complexity of parental decision-making experiences surrounding SDR surgery and required rehabilitation. The aim was to explore parental experiences and expectation in the decision-making process around the SDR operation, physiotherapy treatment and outcomes. METHOD: Qualitative methods using in-depth, semi-structured interviews were used. Eighteen parents (11 mothers and 7 fathers) participated whose child had SDR and completed 2-year post-operative rehabilitation. Data were analysed using thematic analysis. RESULTS: Decision making involved an interacting process between the 'parental information seeking experience', 'influence of professional encounters and relationships' and 'emotional and social aspects'. Despite underlying uncertainties about outcomes, parental drivers of expectations and aspirations for their child preceded and sustained this decision-making process. A foundational narrative of 'wanting no regrets' resolved their decision to undertake SDR. Outcomes did not always match expectations, and parents moved away from a position of idealism, which was driven in part by parental information seeking including overly optimistic media representation. CONCLUSION: Universally, parents expressed they had 'no regrets' on their decision, yet many felt a mismatch between expectation and outcomes of SDR surgery. A greater understanding of the complex nature of SDR decision making is required to help improve family preparedness and provide support from clinicians to enable balanced discussions in parental decision-making.