Cystic fibrosis (CF) is traditionally associated with considerable and progressive multisystem pathology, onerous treatment burden, complex psychosocial challenges, and reduced life-expectancy [1], [2], [3], [4], [5], [6], [7], [8], [9].This decade has seen transformative change in management for many, but not all, people with CF. The most notable change comes from Cystic Fibrosis Transmembrane Receptor (CFTR) modulators, which bring significant benefits for people who are eligible for, and able to access, them [10]. However alongside, or perhaps because of, this exciting progress, the past few years have also brought important novel challenges to the psychosocial wellbeing of people with CF.This article, written as a collaboration between CF psychologists, social workers, physicians and nurses aims to provide an accessible overview of the novel psychosocial challenges now faced by children, their families, and adults with CF, and to invite consideration of their changing psychosocial requirements to inform future holistic care. Themes include geopolitical stressors such as the pandemic and its wake, a growing divide between those able or unable to access CFTR modulators, potential rapid changes in life expectancy secondary to these drugs and the inevitable associated challenges this brings; evolving body image, mental health side effects of CFTR modulators, the challenges of adherence in apparently well children and young adults, as well as the diagnostic conundrum and associated anxiety of the cystic fibrosis screen positive inconclusive diagnosis (CFSPID) label. It also highlights some unmet research and service delivery needs in the area

Objectives A Cystic Fibrosis (CF) youth worker (YW) was employed by the Leeds paediatric and adult CF units in 2017 after successful application for the ‘Clinical Excellence and Innovation Award’ from the CF trust. The key elements with the YW role are to assist young people’s personal and social development whilst supporting maximum participation in education, career development and employment. CF is a chronic condition and although many advances are being made with CFTR modulator therapies, it still has median predicted survival age of 53.3 (CF trust data, 2021). CF also carries a large burden of treatment. We wanted to evaluate the impact of our YW within both adult and paediatric services. Method An evaluation was performed, aiming to explore the impact on the adult and paediatric CF services within LTHT of the new YW role. Staff within both departments were contacted and 7 members of the MDT volunteered to be interviewed using thematic analysis. Results The YW was seen as a beneficial part within transition, specifically for support and pathway development with their unique place within the CF Multi-disciplinary team. Youth work has been pivotal in helping with clinical outcomes, especially around breaking down barriers with compliance and supporting nebulisers and exercise. We have aimed to improve educational and career participation, alongside increased socialisation when needed. Specific examples include assisting a patient to live independently, supporting young people with apprenticeship applications and travel to clinic. Our amazing young people have also been videoed for Leeds Children’s Hospital TV to allow patients to watch their transition journey. This was vital during the pandemic with limited face to face transition visits. Our YW presented a Poster evaluating the support at the ECFS in 2018 and we have also the youtube video; Youth Work in Leeds Clinics by the CF trust. The CF youth worker now sits within the youth work team and offers a youth group, youth forum and hospital Duke of Edinburgh expedition. Conclusion The results suggest the CF youth worker role is highly valued by staff and also reflects the survey evaluation with patients in adult and paediatric CF units at LTHT in 2018. We would be happy to help develop a network of CF youth workers within CF units in the UK. We aim to look at pre and post outcome measures to further support the benefits of this new role. The Cystic Youth WorkerService An Evaluation. Jennifer Kay, Sophie Cammidge. NICE guideline; https://www.nice.org.uk/guidance/ng78 Reference Mehmood A, Cammidge S, Guy E, Peckham D, Duff A. Evaluation of youth work support for teenagers and young adults with cystic fibrosis. Journal of Cystic Fibrosis 2018;17:S128. DoH 2006. Transition: getting it right for young people

Background: Although cystic fibrosis (CF) standards of care have been produced and regularly updated, they are not specifically targeting at the adult population. The ECFS Standards of Care Project established an international task force of experts to identify quality standards for adults with CF and assess their adherence. Methods: This study was composed of two phases. In the first one, a task force of international experts derived from published guidelines and graded ten quality standards for adult CF care using a modified Delphi methodology. In the second phase, an international audit was conducted among adult CF centers to retrospectively validate the quality statements and monitor adherence. Results: The task force identified 10 quality standards specific to the care of adults with CF, mainly based on the 2018 ECFS standards of care. 14 adult CF centers participated in the audit, which showed that most quality standards for the management of CF in adults are met across Europe. Heterogeneity in adherence to standards was found across centers according to geographical setting and centers' characteristics . Conclusions: The identification of quality standards is a valuable resource for the standardization and monitoring of care delivery across centers taking care of adults with CF.

Background: Self and family management of children and young people's (CYP) asthma is complex and multi-faceted. However, research into the impact of COVID-19 on asthma self-management from family perspectives is scant. Aim: Provide an explanation of self and family-management beliefs and behaviours in CYP with severe or sub-optimally controlled asthma, in the context of the COVID-19 pandemic. Methods: In-depth interviews with parents and CYP, aged 11-15 years, with asthma were analysed using grounded theory methodology. Findings: 21 individuals (11 mothers and 10 CYP) participated. Findings explained that families learnt how to self-manage iteratively through their experiences with asthma, alongside clinical support. COVID-19 had multiple impacts on this learning and asthma self/family management including, highlighting CYP's vulnerability and family understanding of asthma severity; COVID-19 fear and anxiety driving medication adherence; observation of COVID-19 infection as an asthma trigger; the apparent paradox of shielding and not being offered early vaccination; vaccination decision-making; observing improved asthma control during shielding. Conclusions: The study provided a unique opportunity to discuss CYP's asthma self and family-management in the context of the changing COVID-19 restrictions. Families described responding iteratively to changes in knowledge, anxiety, and observations of asthma control under changeable contexts. Such contextual insight informed development of a broader explanation of family management behaviours. These dynamic responses suggest there may be opportunities for interventions to promote self-management improvements.

Objectives/methods: Elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) CFTR modulator was approved in the UK for 6–11 year old children with CF and at least 1 phe508del mutation in January 2022. In our large regional network of 86 eligible children 83 commenced ELX/TEZ/IVA, and we monitored closely for safety as part of routine clinical care.

Cystic fibrosis (CF) has entered the era of variant-specific therapy, tailored to the genetic variants in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene. CFTR modulators, the first variant-specific therapy available, have transformed the management of CF. The latest standards of care from the European CF Society (2018) did not include guidance on variant-specific therapy, as CFTR modulators were becoming established as a novel therapy. We have produced interim standards to guide healthcare professionals in the provision of variant-specific therapy for people with CF. Here we provide evidence-based guidance covering the spectrum of care, established using evidence from systematic reviews and expert opinion. Statements were reviewed by key stakeholders using Delphi methodology, with agreement (≥80%) achieved for all statements after one round of consultation. Issues around accessibility are discussed and there is clear consensus that all eligible people with CF should have access to variant-specific therapy.

Reducing treatment burden in cystic fibrosis (CF) is the top research priority for patients and clinicians. Difficulty accessing medication is one aspect of treatment burden. We investigated this with an online survey available globally for patients with CF and healthcare professionals. Almost three quarters of patients with CF in our survey report difficulty getting repeat prescriptions on time, and most community pharmacists experience interrupted supplies of CF-specific medications. These barriers affect emotional and physical health of people with CF. Two-thirds of people with CF would like to get all their CF medication from one place, their CF centre.

INTRODUCTION:Children and young people (CYP) with asthma can benefit from reduced exposure to indoor environmental allergens and triggers but may not consistently have avoidance strategies implemented. To inform future interventions to increase trigger and allergen avoidance and enhance asthma control, a greater understanding of the influences on avoidance behaviours is necessary. METHODS:A systematic scoping review was selected to summarize evidence on what influences family uptake of indoor environmental asthma trigger avoidance strategies for CYP with asthma and identify research gaps. Primary studies of any design, including CYP (≤18 years) with asthma, and/or parent-carers, available in English and conducted since 1993, were eligible. Searches included nine databases, hand-searching reference lists and citation searching. FINDINGS:Thirty-three articles were included and are summarized narratively due to heterogeneity. Influences appear complex and multifactorial and include barriers to strategy uptake, health beliefs and personal motivation. Research specifically related to family understanding of allergic sensitisation status and exposure risks, and how these may inform avoidance implementation is required. Patient and public involvement (PPI) was not reported in included articles, although two studies used participatory methods. CONCLUSION:There is limited research on family asthma trigger management, particularly what influences current management behaviours. Variation in families' ability to identify important triggers, understand exposure risk and consistently reduce exposures warrants further exploratory research to explain how families reach avoidance decisions, and what future interventions should aim to address. Further PPI-informed research to address such gaps, could enable theory-based, person-centred interventions to improve the uptake of asthma trigger remediation. PATIENT OR PUBLIC CONTRIBUTION:An asthma-specific PPI group contributed to the decision-making for the funding for the wider project this review sits within. The findings of this scoping review have informed the subsequent phases of the project, and this was discussed with PPI groups (both adult and CYP groups) when proposing the next phases of the project.

Purpose of review The current review provides an overview of key psychological issues and challenges for the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) modulator era of care. It discusses research from diagnosis and beyond, to patient-team communication with a particular focus on medical trials, adherence and living with CFTR modulators. Recent findings The impact of the diagnosis on parents is immense and the complexity of treatment now and in the future, are a challenge for both parents and teams. Communicating digitally is starting to become daily practice for many in CF care, with coronavirus disease 2019 accelerating this process. Participating in trials has a psychological impact, but most of all the (delayed) access and timing of accessing CFTR modulators is an important theme. Adherence remains of significance, both to ‘old’ and ‘new’ treatments. Living with CF in the era of CFTR modulators is beginning to impact on patients’ quality of life, including new possibilities, opportunities and challenges. Summary Psychological care needs to engage and keep pace with the rapid medical changes. Some care priorities remain the same, including psychological screening and assessment, as well as psychoeducation, communication training and psychotherapy. The presence of CF psychologist in the CF clinic remains as important as ever.

In a recent James Lind Alliance Priority Setting Partnership in cystic fibrosis (CF) the top priority clinical research question was: “What are effective ways of simplifying the treatment burden of people with CF?” We aimed to summarise the lived experience of treatment burden and suggest research themes aimed at reducing it. An online questionnaire was co-produced and responses subjected to quantitative and thematic analysis. 941 survey responses were received (641 from lay community). People with CF reported a median of 10 (interquartile range: 6–15) current treatments. Seven main themes relating to simplifying treatment burden were identified. Treatment burden is high, extending beyond time taken to perform routine daily treatments, with impact varying according to person-specific factors. Approaches to communication, support, evaluation of current treatments, service set-up, and treatment logistics (obtaining/administration) contribute to burden, offering scope for evaluation in clinical trials or service improvement.

used alongside standard clinical outcomes this can help us assess areas of need and focus interventions to support patients by delivering patient centred, fully integrated mind-body care.Reference [1] Linkson et al. (2017) Pediatric Pulmonology, 52, S47, p421.P458 Psychological characteristics of cystic fibrosis at all phases of the disease.Results from all 8 Federal Districts of the Russian Federation O. Poletaeva 1 . 1 "Ostrova" Charitable Foundation, Saint-Peterburg, Russian Federation Objectives: Description of the psychological symptoms and their influence on compliance and life expectancy Methods: We have observed a total of 384 people: 100 patients and 284 parents.68 adult, aged 18-60, 30 men and 38 women.32 children, aged 4-18, 17 boys and 14 girls.20 patients waiting or after lung transplantation, including two children, aged 12 and 14. 8 people, aged 18-27, died.284 parents, aged 22-64.Results: The most important observing psychological phenomena are: symptom of psychological rejection of the lungs after transplantation, symptom of loss of the signal system from the transplanted lungs, uncompleted gender identification by the age of 15, lack of vision of one's personal future, level of socialization, depression in parents.Patients after a lung transplantation: psychological rejection of transplanted lungs 50%, loss of the signal system from the transplanted lungs 50%, anxiety 50%, panic attacks 50%; Positive influences included: sufficient gender identification 75%, stable vision of the future 75%, socialization 75%, psychological right to have new lungs 75%.Patients who died at the age of 18-27: lack of gender identification 100%, depression and anxiety 100%, no vision of the future 88%, lack of socialization 88%.Adult: a low level socialization 51%, no vision of the future 46%, anxiety, loneliness 36%, lack of gender identification 30%.Children: considering of the disease as a norm 47%, low level of socialization 41%, anxiety 34%, no vision of the future 31%.Parents: fear for the child's life 59%, considering himself or herself as a "different" kind of person due to gene mutation 49%, depression 49%, despair due to the child's disease 46%, no vision of the future 44%, feeling guilt about the child for transmitting the faulty gene and fear that the child would blame the parents for that 32%.Conclusion: Further research is necessary to study the influence of these psychological phenomena on compliance and life expectancy of patients.

Objectives Parents of children with cystic fibrosis (CF) are at risk of depression and anxiety symptoms, yet, they are an under-researched group. This national cross-sectional study investigated the prevalence of anxiety and depression in parents of children with CF, and examined the associations between these symptoms and their child's physical health and quality of life. Methods A total of 203 parents of children attending nine paediatric CF clinics across Ireland filled out a questionnaire pack containing: a background information questionnaire; the Hospital Anxiety and Depression Scale (HADS); the Centre for Epidemiological Studies Depression Scale (CES-D); and, the Cystic Fibrosis Quality of Life Scale-Revised Edition (CFQ-R). Results According to the HADS, 38% of parents had elevated anxiety and 12% had elevated depression symptoms. Just over a fifth (20.7%) had elevated depression symptoms on the CES-D. Mothers had significantly higher levels of anxiety than fathers. There were statistically significant negative linear correlations between parents' HADS anxiety and depression scores and their child's pulmonary function, and between parents' HADS anxiety and depression scores and their child's quality of life. Conclusions These results indicate that parents are at risk of depression and anxiety symptoms, and that these are associated with the physical health and quality of life of their child with CF. The findings support the need for parents to have mental health screening annually in CF services, as recommended in international guidelines. There is also a need to integrate a family-centred approach into CF services with appropriate supports and mental health referral pathways for parents.

The benefits of improved treatments for cystic fibrosis (CF) depend on optimal adherence, which remains problematic, particularly to aerosol therapy. In this study, we explored the process of adhering to aerosol therapy from the perspective of both adolescents with CF and their parents. Interviews were conducted individually with six adolescents and six parents, informed by accurate adherence data from an electronically chipped, aerosol device. Interview transcripts from audio-recordings were analyzed using grounded theory method (GTM). Major themes revealed differences in perspective between parent and adolescent, with this relationship mediating the cognitive and emotional processes that play a significant role in adherence behavior. These processes are further influenced by interactions with the aerosol therapy treatment regimen, device characteristics, and the context in which adherence is taking place. Parents and adolescents have different views of treatment and how to manage it. Both need to be addressed if optimal adherence is to be achieved.

symptoms and time-frames.Nurses minimised the emotional impact of end of life care by focusing on the practicalities of the nursing role.Debrief meetings had minimal influence on self-reflective practice since attendance was spurned in favour of informal chats with colleagues and family.Conclusion: End of life care in the field of cystic fibrosis holds unique challenges.The impact of a person's death is felt by nursing staff.There is limited recognition within the nursing profession of the cumulative effect of loss.Existent strategies for managing emotional wellbeing fail to fully meet the needs of nursing staff and engagement is not prioritised.Opportunity for service improvement is evident.

BACKGROUND: People with cystic fibrosis face substantial physical, psychological, and social challenges as they move into adolescence and adulthood, which are likely to impact on their health-related quality of life. This study sought to examine the relative importance of physical and mental health variables associated with health-related quality of life in this group. METHODS: Adults and adolescents (N = 174; ≥14 y old) from across 11 adult or pediatric cystic fibrosis clinics in the Republic of Ireland, completed a background questionnaire that contained self-reported physical health variables, pulmonary function (ie, FEV1%) and body mass index. Questionnaire packs also contained the Hospital Anxiety and Depression Scale (HADS) and the Cystic Fibrosis Questionnaire-Revised, which has been specifically designed to assess health-related quality of life in patients with cystic fibrosis. RESULTS: HADS depression and/or anxiety scores were negatively associated with 11 of the 12 Cystic Fibrosis Questionnaire-Revised domain scores. FEV1% was positively associated with 8 domains when controlling for HADS anxiety but only 4 domains when controlling for HADS depression. HADS anxiety and depression scores demonstrated larger effect sizes and explained a greater proportion of the variance than pulmonary function in 8 of the 12 Cystic Fibrosis Questionnaire-Revised domain scores. CONCLUSIONS: Mental health variables, depression and anxiety, were strongly associated with health-related quality of life in subjects with cystic fibrosis and demonstrated greater effect sizes and explained a higher proportion of the variance overall than the physical health indicators, FEV1% and body mass index, which highlighted the importance of screening for, and treating, depression and anxiety symptoms.

Developments in managing CF continue to drive dramatic improvements in survival. As newborn screening rolls-out across Europe, CF centres are increasingly caring for cohorts of patients who have minimal lung disease on diagnosis. With the introduction of mutation-specific therapies and the prospect of truly personalised medicine, patients have the potential to enjoy good quality of life in adulthood with ever-increasing life expectancy. The landmark Standards of Care published in 2005 set out what high quality CF care is and how it can be delivered throughout Europe. This underwent a fundamental re-write in 2014, resulting in three documents; center framework, quality management and best practice guidelines. This document is a revision of the latter, updating standards for best practice in key aspects of CF care, in the context of a fast-moving and dynamic field. In continuing to give a broad overview of the standards expected for newborn screening, diagnosis, preventative treatment of lung disease, nutrition, complications, transplant/end of life care and psychological support, this consensus on best practice is expected to prove useful to clinical teams both in countries where CF care is developing and those with established CF centres. The document is an ECFS product and endorsed by the CF Network in ERN LUNG and CF Europe.

There remain many treatment uncertainties in cystic fibrosis (CF). With limited resources, research should focus on questions which are most important to the CF community. We conducted a James Lind Alliance Priority Setting Partnership in CF. Research questions were elicited and then prioritised in successive surveys. A workshop agreed the final top 10. Online methods avoided cross infection and widened participation. The elicitation survey had 482 respondents (1080 questions) and prioritisation survey 677 respondents. Participants were drawn equally from the patient and clinical communities globally. We have achieved a consensus on 10 research priorities which will be attractive to funders.

Results: 11 females (mean age = 23.4 ± 3.6) and 9 males(mean age = 25.4 ± 4.5) enrolled into the study.12 patients (60%) reported low medication adherence.Patients who had MMAS-8 score less than 6 were ranked as having low adherence.Patients with scores of 6 and 7 were ranked as having medium adherence; 8 and higher as high adherence.Patients with medium and high adherence were grouped together.Patients with anxiety score of 10 and higher were coded as having anxiety, 7 and higher as having depression.Females reported higher mMRC scores( p < 0.005).HADS revealed that 3 females and 3 males (mean age = 25 ± 5) had anxiety(33%).3 of them also had high depression scores.Medicine adherence did not show a significant association between age, duration of disease, occupational status, educational level, hospital admission in the last year, mMRC, Borg's score, presence of anxiety or depression( p values respectively = 0.292, 0.111,0.396,0.426,0.416,0.360,0.584,0.944).Conclusion: Our study demonstrated that adults with CF had low drug adherence.Anxiety or depression were not found to affect adherence to prescribed drugs.Further studies are needed to understand factors that affect medical adherence and how that can be improved.