Background Newborn bloodspot screening identifies presymptomatic babies who are affected by genetic or congenital conditions. Each year, around 10,000 parents of babies born in England are given a positive newborn bloodspot screening result for one of nine conditions that are currently screened for. Despite national guidance, variation exists regarding the approaches used to communicate these results to families; poor communication practices can lead to various negative sequelae. Objectives Identify and quantify approaches that are currently used to deliver positive newborn bloodspot screening results to parents (phase 1). Develop (phase 2), implement and evaluate (phase 3) co-designed interventions for improving the delivery of positive newborn bloodspot screening results. Quantify the resources required to deliver the co-designed interventions in selected case-study sites and compare these with costs associated with current practice (phase 3). Design This was a mixed-methods study using four phases, with defined outputs underpinned by Family Systems Theory. Setting All newborn bloodspot screening laboratories in England ( n = 13). Participants Laboratory staff and clinicians involved in processing or communicating positive newborn bloodspot screening results, and parents of infants who had received a positive or negative newborn bloodspot screening result. Interventions Three co-designed interventions that were developed during phase 2 and implemented during phase 3 of the study. Main outcome measure Acceptability of the co-designed interventions for the communication of positive newborn bloodspot screening results. Results Staff were acutely aware of the significance of a positive newborn bloodspot screening result and the impact that this could have on families. Challenges existed when communicating results from laboratories to relevant clinicians, particularly in the case of congenital hypothyroidism. Clinicians who were involved in the communication of positive newborn bloodspot screening results were committed to making sure that the message, although distressing for parents, was communicated well. Despite this, variation in communication practices existed. This was influenced by many factors, including the available resources and lack of clear guidance. Although generally well received, implementation of the co-designed interventions in practice served to illuminate barriers to acceptability and feasibility. The interventions would not influence NHS expenditure and could be cost neutral when delivered by teleconsultations. Limitations Participants with a pre-existing interest in this topic may have been more likely to self-select into the study. The researchers are experienced in this field, which may have biased data collection and analysis. COVID-19 hindered implementation and related data collection of the co-designed interventions. Conclusions There was variation in the processes used to report positive newborn bloodspot screening results from newborn bloodspot screening laboratories to clinical teams and then to families. The various practices identified may reflect local needs, but more often reflected local resource. A more consistent ‘best practice’ approach is required, not just in the UK but perhaps globally. The co-designed interventions represent a starting point for achieving this. Future work Future work should include a national evaluation study with predefined outcomes, accompanied by an economic evaluation, to assess the acceptability, feasibility and usability of the co-designed interventions in practice nationally. Trial registration This trial is registered as ISRCTN15330120. Funding This project was funded by the National Institute for Health and Care Research (NIHR) Health and Social Care Delivery Research programme and will be published in full in Health and Social Care Delivery Research ; Vol. 10, No. 19. See the NIHR Journals Library website for further project information.

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.

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.

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.

ObjectiveTo explore health professionals’ experiences of communicating positive newborn bloodspot screening (NBS) results, highlight differences, share good practice and make recommendations for future research.DesignQualitative exploratory design was employed using semi-structured interviewsSettingThree National Health Service provider organisations in EnglandParticipantsSeventeen 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.ResultsFindings 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.ConclusionHealth 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 numberISRCTN15330120

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.

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 .

BACKGROUND:The Cystic Fibrosis Trust recently published a standards of care document which stated that patients should be given a physical activity (PA) programme based on their motivations, fitness, and willingness to be active. However, there remains much debate regarding the roles and responsibilities for PA promotion, as well as "optimal" recommendations and advice. This study aimed to qualitatively explore cystic fibrosis (CF) multidisciplinary teams (MDTs) advice, recommendations and practices relating to PA promotion for adolescents with CF.METHOD:Semi-structured interviews were conducted with fifteen members of CF MDTs (11 physiotherapists, two dieticians and two paediatricians). Thematic analysis was used to analyse the data.RESULTS:Major themes identified were: (1) structure of MDTs, (2) recommendations relating to intensities, durations and types of PA, and (3) use of exercise testing. Participants reported variation between MDTs in terms of who is responsible for promoting and supporting PA, the nature of advice given to patients, and the use of exercise testing. Participants consistently lacked confidence in their own or others' knowledge to provide standardised recommendations to patients and highlighted that PA promotion and support was often overlooked during busy periods.CONCLUSIONS:Despite its importance, PA support and promotion is not always prioritised. MDTs lack confidence in their ability to promote PA. Standardised advice and training relating to optimal intensities, durations and types of PA would provide a baseline from which to individualise advice to each patient and could increase confidence in PA promotion among MDTs.

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.

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. 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. 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. ISRCTN 15330120 date of registration 17/01/2018

Physical activity (PA) is recommended for all children and young people with CF (CYPwCF), however levels of participation vary between and within individuals. Studies collecting short-term PA data from research trackers are often limited by insufficient wear-time, and fail to reflect variations related to weather/season, weekday/weekend, school holiday, or hospital admission. CYP have reported that comfort, design and feedback features are important, thus commercially available activity trackers with these features may enable better measurement of individual PA patterns over time. Wear-time data were collected from CYPwCF (6-16yrs) asked to wear a Fitbit AltaHR as much as possible during the first 30days of participation in a longitudinal study. From literature, ≥8 recorded daytime hours were considered valid for data analysis with 3-9days providing reliable measurement of PA levels. A total of 1,950days were analysed from 65CYPwCF (median:9.6yrs, 36male). In 30days, CYPwCF contributed mean±SD 16±8days valid for data analysis (range:0-28days). 49/65 (75%) provided >9days (median:21, range:9-28days), 7/65 (11%) provided 3-8days (med:5, range:3-8), and 9/65 (14%) yielded 0-2days, 4/9 due to problems with data synchronising. Days of valid data contributed by participants varied, but 86% easily exceeded traditional thresholds for PA data analysis within 30days. Results suggest that commercially available activity trackers may prove useful in assessing longitudinal patterns of PA in CYPwCF. Further research will continue to evaluate minimal wear-time for accurately and reliably characterising patterns of PA taking account of seasonal and circumstantial changes.

Psychologists are increasingly present in multidisciplinary teams for physical health conditions. This service evaluation explored how MDT members of a cystic fibrosis service experience the psychology service embedded in their team. The results indicated that all aspects of psychology were valued by MDT members.

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.

Dear editors In response to the correspondence by Quittner et al , we have the following comments. It must be stated that we think your The International Depression Epidemiological Study (TIDES) paper1 has merit, and we commend any endeavours to highlight the mental health issues of people with cystic fibrosis (CF) and improvements in psychosocial service provision. The editorial has no intention of minimising the extent of the problem, quite the opposite. Your publication (and further correspondence) reports on two different issues: first, the TIDES study, and second, the recommendations of the International Guidelines Committee on Mental Health (IGCMH) in CF. The second is dependent on the findings of the first. So, the TIDES paper has methodological weaknesses and inconsistencies between the various publications that have devolved from the study.2 ,3 The TIDES authors must be conscientious in the conclusions they draw and the level of clinical significance that is …

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.

The remarkable improvement in survival for patients with cystic fibrosis (CF) is one of the great success stories of modern clinical medicine. Over a period of three decades, a disease associated with childhood dying (as parents of CF children were informed) has become one of adulthood survival into the fifth and sixth decades of life. CF is the most complex of diseases. It remains (despite recent advances) a chronic multisystem incurable disease in a survivor population who are beginning to lead normal working and domestic lives. This improved survival is a direct result of holistic care delivered by multidisciplinary teams (MDT) based in paediatric and adult CF centres.1 The establishment of CF centres around the world has enabled large evidence-based clinical trials, patient registries, best practice guidelines and thereby patient-centred care. Although CF patients appear to cope remarkably well with a disease which places a huge burden of care on an often busy working and social life, it is not surprising as detailed in several small series that CF patients suffer to varying degrees from heightened anxiety and depression.2–4 There is a non-negotiable requirement of CF adults and children to provide lifelong self-care to maintain disease stability and to make the most of a normal quality of life. Perceived poor quality of life can result in a despondency with treatment and poor adherence to the daily regimen leads quickly to deterioration of health.5 ,6 Lifelong medical conditions are not managed by medicine alone, there is a psychological cost and this paper is commended for having elevated the issue of potential psychological impairment in CF. The provision of expert psychosocial care is an essential …

BackgroundOur aim was to develop a measure of eating attitudes and behaviours (EAB) in cystic fibrosis (CF), examine its psychometric properties and conduct initial validation analyses. There are no current measures of EAB suitable for use in the CF population. The intention is to make the measure available for use clinically as a routine measure of EAB in CF. This will be particularly useful given the serious health consequences of poor nutritional status (likely to be related to EAB) in CF and may help to guide treatment planning (i.e., specialist eating disorder service or in-house management by CF team)MethodsAn initial item generation and piloting stage involved literature searches of EAB in CF and eating disorders and how they are measured, consultation with professionals and cognitive interviewing with 8 individuals with CF. The final measure was then administered to 150 participants with CF (11–65 years) recruited from tertiary CF clinics, who are representative of the wider CF population. Participants also completed a series of questions about adherence to other treatments and an existing measure of EAB used in the general population. Other clinical and demographic information was gathered from participants’ notes.ResultsA final measure was developed using standard statistical analyses for assessing the reliability of a measure (exploratory factor analysis and Cronbach’s alpha analyses). Preliminary validation of the measure was carried out by examining its relationships with hypothetically related variables such as clinical status (lung function), body mass index (BMI), adherence and the existing EAB measure.ConclusionThe measure is published and available free of charge (CFEAB).