A survey of mental health professionals in a paediatric hospital during COVID-19.

BACKGROUND: There is little understanding of the mental health impact for young people with long-term physical health conditions and mental health professionals' experiences of supporting them during COVID-19. This service evaluation aimed to conduct a survey of the psychological services provided by mental health professionals in a paediatric hospital in relation to COVID-19. METHOD: Clinical psychologists and assistant psychologists (n = 76) across the hospital were asked to complete a survey, asking about their perceptions of COVID-19's impact on patients and families and experiences of providing support during COVID-19. Open-ended survey questions were analysed qualitatively using framework analysis. RESULTS: Respondents described perceived impacts on patients and families around social isolation, school closure, family relationships, physical health, mental health, treatments and social support. Respondents' experiences of providing mental health support during COVID-19 highlighted themes around providing remote/virtual support, workload and facilitators and barriers to their work. CONCLUSIONS: Mental health professionals surveyed reported a complex mental health landscape in young people with long-term physical health conditions and their families during COVID-19. Service-wide involvement is needed to facilitate changes to support vital adaptations to remote/virtual working. Research on the mental health of young people with long-term physical health conditions and staff experiences of providing support is warranted.

Process evaluation of co-designed interventions to improve communication of positive newborn bloodspot screening results.

OBJECTIVE: To implement and evaluate co-designed interventions to improve communication of positive newborn bloodspot screening results and make recommendations for future research and practice. DESIGN: A process evaluation underpinned by Normalisation Process Theory. SETTING: Three National Health Service provider organisations in England. PARTICIPANTS: Twenty-four healthcare professionals (7 newborn screening laboratory staff and 24 clinicians) and 18 parents were interviewed. INTERVENTIONS: Three co-designed interventions were implemented in practice: standardised laboratory proformas, communication checklists and an email/letter template. PRIMARY OUTCOME MEASURES: Acceptability and feasibility of the co-designed interventions. RESULTS: Auditing the implementation of these interventions revealed between 58%-76% of the items on the laboratory proforma and 43%-80% of items on the communication checklists were completed. Interviews with healthcare professionals who had used the interventions in practice provided positive feedback in relation to the purpose of the interventions and the ease of completion both of which were viewed as enhancing communication of positive newborn bloodspot screening results. Interviews with parents highlighted the perceived benefit of the co-designed interventions in terms of consistency, pacing and tailoring of information as well as providing reliable information to families following communication of the positive newborn bloodspot screening result. The process evaluation illuminated organisational and contextual barriers during implementation of the co-designed interventions in practice. CONCLUSION: Variations in communication practices for positive newborn bloodspot screening results continue to exist. The co-designed interventions could help to standardise communication of positive newborn screening results from laboratories to clinicians and from clinicians to parents which in turn could improve parents' experience of receiving a positive newborn bloodspot screening result. Implementation highlighted some organisational and contextual barriers to effective adoption of the co-designed interventions in practice. TRIAL REGISTRATION NUMBER: ISRCTN15330120.

Processing of positive newborn screening results: a qualitative exploration of current practice in England.

OBJECTIVE: To explore current communication practices for positive newborn screening results from the newborn bloodspot screening (NBS) laboratory to clinicians to highlight differences, understand how the pathways are implemented in practice, identify barriers and facilitators and make recommendations for future practice and research. DESIGN: A qualitative exploratory design was employed using semi-structured interviews. SETTING: Thirteen NBS laboratories in England. PARTICIPANTS: Seventy-one clinicians; 22 NBS laboratory staff across 13 laboratories and 49 members of relevant clinical teams were interviewed. RESULTS: Assurance of quality and consistency was a priority for all NBS laboratories. Findings indicated variation in approaches to communicating positive NBS results from laboratories to clinical teams. This was particularly evident for congenital hypothyroidism and was largely influenced by local arrangements, resources and the fact individual laboratories had detailed standard operating procedures for how they work. Obtaining feedback from clinical teams to the laboratory after the child had been seen could be challenging and time-consuming for those involved. Pathways for communicating carrier results for cystic fibrosis and sickle cell disease could be ambiguous and inconsistent which in turn could hamper the laboratories efforts to obtain timely feedback regarding whether or not the result had been communicated to the family. Communication pathways for positive NBS results between laboratories and clinical teams could therefore be time-consuming and resource-intensive. CONCLUSION: The importance placed on ensuring positive NBS results were communicated effectively and in a timely fashion from the laboratory to the clinical team was evident from all participants. However, variation existed in terms of the processes used to report positive NBS results to clinical teams and the people involved. Variant practice identified may reflect local needs, but more often reflected local resources and a more consistent 'best practice' approach is required, not just in the UK but perhaps globally. TRIAL REGISTRATION NUMBER: ISRCTN15330120.

Qualitative exploration of health professionals' experiences of communicating positive newborn bloodspot screening results for nine conditions in England.

OBJECTIVE: To explore health professionals' experiences of communicating positive newborn bloodspot screening (NBS) results, highlight differences, share good practice and make recommendations for future research. DESIGN: Qualitative exploratory design was employed using semi-structured interviews SETTING: Three National Health Service provider organisations in England PARTICIPANTS: Seventeen health professionals involved in communicating positive newborn bloodspot screening results to parents for all nine conditions currently included in the newborn bloodspot screening programme in England. RESULTS: Findings indicated variation in approaches to communicating positive newborn bloodspot screening results to parents, largely influenced by resources available and the lack of clear guidance. Health professionals emphasised the importance of communicating results to families in a way that is sensitive to their needs. However, many challenges hindered communication including logistical considerations; difficulty contacting the family and other health professionals; language barriers; parental reactions; resource considerations; lack of training; and insufficient time. CONCLUSION: Health professionals invest a lot of time and energy trying to ensure communication of positive newborn bloodspot screening results to families is done well. However, there continues to be great variation in the way these results are communicated to parents and this is largely influenced by resources available but also the lack of concrete guidance. How best to support health professionals undertaking this challenging and emotive task requires further exploration. We recommend evaluation of a more cohesive approach that meets the needs of parents and staff while being sensitive to the subtleties of each condition. TRIAL REGISTRATION NUMBER: ISRCTN15330120.

Protocol for Project Fizzyo, an analytic longitudinal observational cohort study of physiotherapy for children and young people with cystic fibrosis, with interrupted time-series design.

INTRODUCTION: Daily physiotherapy is believed to mitigate the progression of cystic fibrosis (CF) lung disease. However, physiotherapy airway clearance techniques (ACTs) are burdensome and the evidence guiding practice remains weak. This paper describes the protocol for Project Fizzyo, which uses innovative technology and analysis methods to remotely capture longitudinal daily data from physiotherapy treatments to measure adherence and prospectively evaluate associations with clinical outcomes. METHODS AND ANALYSIS: A cohort of 145 children and young people with CF aged 6-16 years were recruited. Each participant will record their usual physiotherapy sessions daily for 16 months, using remote monitoring sensors: (1) a bespoke ACT sensor, inserted into their usual ACT device and (2) a Fitbit Alta HR activity tracker. Real-time breath pressure during ACTs, and heart rate and daily step counts (Fitbit) are synced using specific software applications. An interrupted time-series design will facilitate evaluation of ACT interventions (feedback and ACT-driven gaming). Baseline, mid and endpoint assessments of spirometry, exercise capacity and quality of life and longitudinal clinical record data will also be collected.This large dataset will be analysed in R using big data analytics approaches. Distinct ACT and physical activity adherence profiles will be identified, using cluster analysis to define groups of individuals based on measured characteristics and any relationships to clinical profiles assessed. Changes in adherence to physiotherapy over time or in relation to ACT interventions will be quantified and evaluated in relation to clinical outcomes. ETHICS AND DISSEMINATION: Ethical approval for this study (IRAS: 228625) was granted by the London-Brighton and Sussex NREC (18/LO/1038). Findings will be disseminated via peer-reviewed publications, at conferences and via CF clinical networks. The statistical code will be published in the Fizzyo GitHub repository and the dataset stored in the Great Ormond Street Hospital Digital Research Environment. TRIAL REGISTRATION NUMBER: ISRCTN51624752; Pre-results.

Breaking the diagnosis of cystic fibrosis to parents: A process not a one-off event.

Breaking the news to parents that their child has cystic fibrosis [CF] is most frequently given in the first few weeks of the baby's life as a result of newborn screening. This is optimal to reduce morbidity but can have a significant impact on the parents' mental wellbeing and the parent-child relationship. Parent feedback indicates that assimilating the diagnosis is not a one-off event but a process that takes time. CF professionals therefore need to be aware not only of how they communicate the diagnosis initially but also the ways in which families make sense of this throughout at least the following year. The parent-patient-team relationship is essential to good health outcomes. Key objectives of this paper are to enable: (1) understanding parental responses to the diagnosis which can indicate how well they are managing CF for their child, (2) improving the way in which the diagnosis is communicated and, (3) changing team management of CF in the early years to include parental collaboration to support better mental and physical outcomes.

What factors influence healthcare professionals to refer children and families to paediatric psychology?

OBJECTIVES: This study aimed to investigate factors influencing referral of children with physical illness to paediatric psychology. Due to high rates of mental health problems within this population, studies have shown that referral to paediatric psychology should be increased. However, few studies have examined factors shaping healthcare professionals' referral behaviour. METHODS: This study used the theory of planned behaviour to develop a questionnaire which explores factors influencing the referral of children and families to paediatric psychology. Psychometric properties of the questionnaire were examined. RESULTS: The questionnaire was found to have good reliability and validity. The main constructs of the theory of planned behaviour were useful in predicting intention to refer to paediatric psychology. Specific beliefs about referral were shown to influence intention to refer. CONCLUSION: Findings suggest that individual attitudes and beliefs can impact healthcare professionals' referral behaviour, indicating that multidisciplinary interventions and inter-professional education relating to the psychological aspects of illness are required.

Rethinking Strategies for Positive Newborn Screening Result (NBS+) Delivery (ReSPoND): a process evaluation of co-designing interventions to minimise impact on parental emotional well-being and stress.

BACKGROUND: Newborn blood spot (NBS) screening seeks to prevent ill health, disability and death through early diagnosis and effective intervention. Each year, around 10,000 parents of babies born in England are given a positive NBS result indicating their child may be affected or carriers of one of the nine conditions currently screened for. Despite guidance, these results are inconsistently delivered to parents across geographical regions. There is evidence that many parents are dissatisfied with how NBS results are communicated to them and that poor communication practices can lead to various negative sequelae. The purpose of this study is to co-design, implement and undertake a process evaluation of new, co-designed interventions to improve delivery of initial positive NBS results to parents. METHODS: This mixed-methods study will use four phases with defined outputs. Family Systems Theory will form the theoretical basis for the study. The principles and methods of experience-based co-design will underpin intervention development. Normalisation Process Theory will underpin the process evaluation of the interventions co-designed to improve the delivery of positive NBS results to parents. An economic analysis will determine resource use and costs of current practice and of implementing the new co-designed interventions. The nominal group technique will be used to inform the selection of suitable outcome measures for a future evaluation study. DISCUSSION: The main output of the proposed study will be co-designed interventions for initial communication of positive NBS results to parents ready to be evaluated in a definitive evaluation study.The interventions, co-designed with parents, will help to minimise potential negative sequelae associated with poor communication practices by considering parental and staff experiences as well as healthcare challenges such as finite resources. In addition, information about indicative costs associated with different communication strategies will be determined.It is anticipated it may also be possible to extrapolate principles of good communication practices from the present study for the delivery of bad news to parents for children newly diagnosed with other conditions including cancer and other chronic conditions such as diabetes or epilepsy. TRIAL REGISTRATION: ISRCTN 15330120 date of registration 17/01/2018.

The impact on quality of life on families of children on an elimination diet for Non-immunoglobulin E mediated gastrointestinal food allergies.

BACKGROUND: The impact on health related quality of life (HRQL) has been well studied in children with Immunoglobulin E (IgE)-mediated food allergy. However limited data exists on related quality of life (QOL) of families who have a child suffering from food protein induced non-IgE mediated gastrointestinal allergies. We aimed to establish the QOL of families with children at the beginning of following an elimination diet for non-IgE mediated gastrointestinal food allergies. METHODS: A prospective, observational study was performed. Parents of children aged 4 weeks-16 years who improved after 4-8 weeks of following an elimination diet for suspected non-IgE mediated allergies were included. The Family Impact Module (FIM) of the Pediatric Quality of Life (PedsQL™) was used and we compared our data to two historical cohorts: one with sickle cell disease and another with intestinal failure. RESULTS: One hundred and twenty three children with a median age of 20 months were included (84 boys). The total FIM Score was 57.43 (SD 22.27) and particularly low for daily activities and worry. Factors that impacted significantly included age (p < 0.0001), number of foods excluded (p = 0.008), symptom severity (p = 0.041) and chronic nasal congestion (p = 0.012). Children with non-IgE mediated food allergies had worse scores in all domains (p < 0.0001) compared to sickle cell disease and worse physical (p = 0.04), emotional (p = 0.04) and worry (p = 0.01) domains compared to intestinal failure. CONCLUSIONS: This study found that parent QOL and family functioning was worse in those families who had a child on an elimination diet for non-IgE mediated allergies compared to those with sickle cell disease and intestinal failure, highlighting the impact this disease has on families.

European Cystic Fibrosis Society Standards of Care: Best Practice guidelines.

Specialised CF care has led to a dramatic improvement in survival in CF: in the last four decades, well above what was seen in the general population over the same period. With the implementation of newborn screening in many European countries, centres are increasingly caring for a cohort of patients who have minimal lung disease at diagnosis and therefore have the potential to enjoy an excellent quality of life and an even greater life expectancy than was seen previously. To allow high quality care to be delivered throughout Europe, a landmark document was published in 2005 that sets standards of care. Our current document builds on this work, setting standards for best practice in key aspects of CF care. The objective of our document is to give a broad overview of the standards expected for screening, diagnosis, pre-emptive treatment of lung disease, nutrition, complications, transplant/end of life care and psychological support. For comprehensive details of clinical care of CF, references to the most up to date European Consensus Statements, Guidelines or Position Papers are provided in Table 1. We hope that this best practice document will be useful to clinical teams both in countries where CF care is developing and those with established CF centres.

Developing a measure of eating attitudes and behaviours in cystic fibrosis.

BACKGROUND: Eating disorders or disturbed eating attitudes and behaviours (EABs) may contribute to poor nutritional status in Cystic Fibrosis (CF). Existing measures of disturbed EABs can have different meanings in this population and do not assess CF-related EABs. A self-report measure of EABs in CF was developed to highlight areas of eating disturbance. METHODS: The content validity of a draft measure was evaluated via expert evaluation and literature review and an amended measure piloted with 8 CF patients using cognitive interviewing. A further amended measure was administered to 155 CF patients (11-62 years) attending CF clinics. RESULTS: Principal components analyses revealed a three-factor structure ('Desire for thinness and weight loss', 'Disturbed EABs', and 'Appetite') with good internal consistencies for subscales and the 21-item whole measure. CONCLUSIONS: The measure looks promising as a tool to highlight EAB disturbance in CF. Further work will establish its construct validity and clarify interpretation of subscales.

Guiding principles on how to manage relevant psychological aspects within a CF team: interdisciplinary approaches.

Managing CF can be emotionally and physically challenging for patients and their relatives. The disease and its treatment influence the ability to tackle normal tasks of daily living and unexpected life events. The context within which psychologists work varies according to different cultural backgrounds and their professional and theoretical memberships. The benchmarks presented here focus on four crucial issues: (i) identifying a common base of tools and theoretical reflections through suggested readings, (ii) interdisciplinary work within a CF team and its importance for both persons with CF and other healthcare professionals, (iii) the benefits of an eclectic approach utilising cognitive-behavioural theories for specific psychological problems and, (iv) effective and evaluated transition programmes from paediatric to adult healthcare services.

A model for transition from pediatric to adult care in cystic fibrosis.

With improved medical technology, many chronic medical conditions of childhood are now recognized as conditions of childhood onset. Appropriate treatment of these conditions in the adult patient is required. The pediatric setting is not appropriate in terms of philosophy, service delivery, and relationship with the patient. To design a suitable transition protocol, a survey of teenage patients with cystic fibrosis was carried out and the process was discussed with the relevant clinicians. The results of the survey indicated that young people identified transition to an adult service as necessary and accepted, provided that good preparation is given from the pediatric setting. This article provides a detailed example of a model of transition for cystic fibrosis services to encourage practice and evaluation of provision.