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.

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.

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 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.

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 ( = 11) were recruited via an online forum and participated in semistructured telephone interviews about their experiences of becoming a mother.

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.

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.

Motivational interviewing for adherence problems in cystic fibrosis; evaluation of training healthcare professionals.

Background: Motivational interviewing (MI) offers effective strategies for enhancing behaviour change and is particularly useful for patients who exhibit poor adherence. This study evaluated MI training for cystic fibrosis (CF) teams, which comprised of one 4-hour workshop on MI principles, followed 6 months later by another on applying MI during brief consultations.

Methods: Health professionals (N = 60) from 7 teams completed questionnaires on learning outcomes 6 months after the first workshop, but before the second.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

Feeding behavior problems in children with cystic fibrosis in the UK: prevalence and comparison with healthy controls.

Background: Feeding behavior problems contribute to inadequate dietary intake for many patients with cystic fibrosis (CF). However, to establish effective intervention programs, more needs to be known about the occurrence and distribution of these difficulties. The aims of this study were to establish the prevalence and range of disruptive child behaviors (DCB) in patients with CF and the inappropriate parental responses (IPR) during mealtimes and to compare the results with those of healthy children.