Changing landscape: psychological care in the era of cystic fibrosis transmembrane conductance regulator modulators.

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.

Adherence to Aerosol Therapy in Young People With Cystic Fibrosis: Patient and Parent Perspectives Following Electronic Data Capture.

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.

The top 10 research priorities in cystic fibrosis developed by a partnership between people with CF and healthcare providers.

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.

Depression in cystic fibrosis; Implications of The International Depression/Anxiety Epidemiological Study (TIDES) in cystic fibrosis.

Children and adults with chronic diseases, as well as their parents, are at increased risk for depression. Where people with CF do exhibit psychological distress it is linked to poorer adherence and pulmonary function, increased hospitalisations and healthcare costs and decreased quality of life. The International Depression Epidemiological Study (TIDES) evaluated depression and anxiety in CF patients and parent caregivers across eight European countries and the USA. Two national and one international data sets have been published. This paper summarises the findings, offers explanations for differences in results, and outlines the clinical implications with consideration given to if and how recommendations could be integrated into managing CF in the UK.

Presenting life with cystic fibrosis: a Q-methodological approach to developing balanced, experience-based prenatal screening information.

BACKGROUND: Cystic fibrosis (CF) is one of the most common life-threatening genetically inherited conditions and prenatal screening for CF is available in many countries. Genetic counsellors and other health professionals are expected to provide information about the condition in a way that facilitates personal decision making. Knowing what information to deliver about complex genetic conditions to support informed screening decisions can be challenging for health professionals. OBJECTIVE: To solicit views from those with personal experience with CF on which aspects of the condition they consider most important to include in prenatal screening materials. METHODS: Q-methodology; an approach to systematically explore variations in viewpoint that combines factor analytic techniques with qualitative approaches to pattern interpretation. SETTING AND PARTICIPANTS: Twelve adults with CF and 18 parents of affected children were recruited from a regional centre in the UK. RESULTS: Five distinct viewpoints on the items most and least important to include in screening information were identified: Factor 1 the normality of life with CF and increasing life expectancy; Factor 2 the hardships and reduced lifespan. Factor 3 medical interventions and the importance of societal support. Factor 4 longer-term consequences of CF. Factor 5 the ability to adjust to the condition. DISCUSSION: The identification of five different views on what represented the most and least important information to include about CF highlights the challenge of portraying a complex genetic condition in a balanced and accurate manner. Novel ways in which Q-methodology findings can be used to meet this challenge are presented.

Decision-making about pregnancy for women with cystic fibrosis.

OBJECTIVES: This study aims to generate experiential data about the decision-making process encountered by 12 women with cystic fibrosis (CF) considering pregnancy. DESIGN: This study uses a qualitative grounded theory methodology to analyse the data and develop a conceptual hierarchical model of salient issues. METHODS: Twelve women completed a semi-structured interview accompanied by quantitative self-report measures of quality of life and psychological well-being, and indicators of health and treatment status were used. RESULTS: Descriptive data demonstrated that the sample did not differ significantly from other published samples. Four core categories emerged: (1) impact of the decision; (2) preparation for making and living with the decision; (3) owning the decision; and (4) personal dilemmas. Each comprised a number of overlapping conceptual and subcategories. Influencing factors included the impact of pregnancy on participants' health, and upon their ability to care for the child, the pregnancy experiences of other women with CF, the moral implications of having a child and the support and beliefs of family members. DISCUSSION: The findings extend the current knowledge in this area. We recommend development of guidelines for supporting and advising chronically ill young women about the psychosocial and emotional issues.

ECFS best practice guidelines: the 2018 revision.

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.

Newborn screening for CF in a regional paediatric centre: the psychosocial effects of false-positive IRT results on parents.

Neonatal screening for cystic fibrosis (CF) has been established in Leeds since 1975. The current method is measuring IRT and genotyping. Newborn screening for CF results in a small but significant number of false positives. This study explored the psychosocial reactions to such results in a group of parents (N=21) using semi-structured interviews. Responses were analysed using descriptive statistics and well-validated content analysis. Mothers described a range of emotions during the screening process including anxiety, distress and upset. Waiting for the repeat IRT test results was identified as the most emotionally difficult stage. Discussion focuses on good practice and implications for CF services.

When Women with Cystic Fibrosis Become Mothers: Psychosocial Impact and Adjustments.

Advances in the treatment and life expectancy of cystic fibrosis (CF) patients mean that motherhood is now a realistic option for many women with CF. This qualitative study explored the psychosocial impact and adjustments made when women with CF become mothers. Women with CF (n = 11) were recruited via an online forum and participated in semistructured telephone interviews about their experiences of becoming a mother. Transcriptions were analysed using Grounded Theory. Analysis revealed three core categories: (i) "Living with CF": how becoming a mother impacted on health and treatment adherence, requiring a change in support from the CF team, (ii) "Becoming a Mother": balancing issues common to new mothers with their CF, and (iii) "Pooling Personal Resources": coping strategies in managing the dual demands of child and CF care. Participants experienced a variety of complex psychosocial processes. Most participants acknowledged an initial negative impact on CF care; however over time they reported successful adaptation to managing dual commitments and that adherence and motivation to stay well had improved. This study highlights the need for preconceptual psychosocial counselling and postpartum adjustment to CF care.

Introducing the need for lung transplantation in children with cystic fibrosis: parental experiences.

Lung transplantation (LTx) is the only treatment available for adult and pediatric end-stage lung disease secondary to cystic fibrosis (CF). The timing of introducing LTx has significant medical and psychological implications for the child and the family. This study explored the views and recommendations of parents of children with CF, who had been asked to consider LTx and referred to a national transplant centre. Parents participated in a telephone-based, semi-structured interview. Responses were analysed using Content Analysis. Parental recommendations and the emergent protocol are discussed, together with implications for clinical practice.

Cultural issues in cystic fibrosis.

Although current numbers of non-Caucasian CF patients are small, collectively they represent sizeable groups and increasingly more is becoming known about the spectrum of mutations in the CFTR gene in different populations. As such it is important to acknowledge that there will be certain sociocultural challenges in interacting with and managing such patients. This paper explores such developments and considers some of the current and future challenges facing those who treat the disease. Techniques applied in other illness contexts for establishing and maintaining effective patient-professional relationships, reviewing patient education, resolving treatment conflicts and improving adherence are discussed, as are the implications for future research.

Depression and anxiety in adolescents and adults with cystic fibrosis in the UK: a cross-sectional study.

BACKGROUND: The International Depression/anxiety Epidemiological Study (TIDES) in the UK aimed: (i) to establish the prevalence of anxiety and depression amongst people with CF compared to a normative sample; (ii) to establish the association between mood, demographic and clinical variables; and (iii) to provide guidance for specialist-referral decision-making. METHODS: Patients (≥12years) completed the Hospital Anxiety and Depression Scale (HADS). CF-HADS scores, expressed as percentiles, were compared with a normative sample. Multiple-regression analysis explored associations between demographic, clinical and mood variables. RESULTS: Thirty-nine CF centres recruited 2065 patients. Adults with CF were similar in terms of anxiety and depression to the general population. Adolescents with CF were less anxious and depressed. For adult patients, older age, unemployment for health reasons and poor lung function were associated with disordered mood. Gender-specific CF-percentile scores were calculated. CONCLUSION: Surveillance, with attention to gender and risk factors is advocated. This work provides unique benchmark scores to aid referral decision-making.

Screening for depression in a single CF centre.

INTRODUCTION: The International Depression and Anxiety Epidemiological Study (TIDES) in CF attempts to provide prevalence data for mental distress in CF patients. The current study reports the results from the UK pilot in a single major CF centre in which the performances of different instruments were compared, and risk of self harm measured. METHODS: Two mental health assessment screening instruments, the Hospital Anxiety and Depression Scale (HADS) and the Patient Health Questionnaire for depression (PHQ-9) were given to all adults with CF attending the Regional Adult CF Unit in Leeds, UK. RESULTS: 232 patients took part. 13 (5.6%) scored in the clinical range for depression on the HADS and 78 (33.4%) on the PHQ9. The suicide risk item on the PHQ9 was endorsed by 24 patients (10.4%). DISCUSSION: Major discrepancies were found in rates of depression detected by two popular screening tools. A significant minority of respondents indicated suicidal ideation. Work is needed to identify the most appropriate screening tool for depression in this population, and it is important that this includes an assessment of self harm.

Decision Making about Risk of Infection by Young Adults with CF.

Young people with cystic fibrosis (CF) are asked to avoid a number of environments associated with increased infection risk, but in practice they need to balance this with competing priorities such as building and sustaining relationships with friends and family. This study explored the process by which young people make these decisions. Mixed methods were used: a vignette study presenting choices around engaging in activities involving a degree of infection risk and a thematic analysis of participant's accounts of their decision making. The eight participants chose to engage in high risk behaviours in 59% of the choices. All participants chose to engage in at least one risky behavior, though this was less likely when the risk was significant. Thematic analysis revealed large areas of misunderstanding and lack of knowledge, leading to some potentially worrying misconceptions about the nature of infections and risk. Young people with CF are not currently making informed decisions around activities that involve increased risk of infection, and there is an urgent need for CF teams to address this in information provision.

Adherence to nebulised therapies in adolescents with cystic fibrosis is best on week-days during school term-time.

OBJECTIVES: Treatment regimen for families of children with cystic fibrosis (CF) is considerable, particularly when nebulised therapies for chronic Pseudomonas aeruginosa airway infection are prescribed. Adherence to these regimens is variable, particularly in adolescence. Previously, we reported children to be more adherent in evenings compared to mornings, suggesting an association with time-pressure. The aim of this study was to determine whether adherence would be better in adolescent patients at weekends and during school holidays when time-pressures may be less. STUDY DESIGN: 24 patients (14 male, median [range] age 13.9 [11.1-16.8] years) were enrolled from two regional paediatric CF centres in the United Kingdom. Data for a full scholastic year, were downloaded openly from a breath-activated data logging nebuliser (I-neb™). Adherence (% of doses taken÷expected number) was calculated during term-times, holidays, weekends and weekdays, for each patient. RESULTS: Large variations in adherence were seen between patients. However, adherence during term-time was significantly better than holidays (p<0.001). Weekday adherence was better than weekend adherence in term-time but not holidays. Interestingly, patients prescribed three daily treatments took on average 1.4 treatments/day, a similar number to those prescribed two daily treatments. CONCLUSION: Overall adherence to inhaled therapies was reasonable, but significantly reduced during holiday periods. This suggests a need for families to have not only time, but also structure in their daily routine to maintain optimal adherence to long-term therapies. It is important for CF teams to appreciate these factors when supporting families.

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.

Motivational interviewing for adherence problems in cystic fibrosis.

This review focuses on adherence in cystic fibrosis (CF), and the factors known to influence it. In particular, it discusses the importance of effective communication in clinical settings and considers the evidence for the effectiveness of motivational interviewing (MI), to increase adaptation and adherence in physical health and CF. The review also contains an overview of the key concepts of MI, its' practice in medical settings and recommendations on how to adopt MI techniques in the routine care of people with CF.