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.

The impact of the COVID-19 pandemic on the emotional well-being and home treatment of Belgian patients with cystic fibrosis, including transplanted patients and paediatric patients.

Background: Little is known about the impact of COVID-19 on patients with cystic fibrosis (CF), despite being considered a high-risk group. This study explored the early impact of COVID-19 on the emotional well-being of patients and self-reported changes in their home therapy since the start of the pandemic.

Methods: Adult patients with CF, lung-transplanted (LTX) CF patients and parents of children with CF completed an online questionnaire, securely linked to their medical files.

Assessing gastro-intestinal related quality of life in cystic fibrosis: Validation of PedsQL GI in children and their parents.

Background: Most patients with cystic fibrosis (CF) suffer from pancreatic insufficiency, leading to fat malabsorption, malnutrition and abdominal discomfort. Until recently, no specific tool was available for assessing gastro-intestinal related quality of life (GI QOL) in patients with CF. As the Horizon2020 project MyCyFAPP aims to improve GI QOL by using a newly designed mobile application, a sensitive and reliable outcome measure was needed.

The future of cystic fibrosis care: a global perspective.

The past six decades have seen remarkable improvements in health outcomes for people with cystic fibrosis, which was once a fatal disease of infants and young children. However, although life expectancy for people with cystic fibrosis has increased substantially, the disease continues to limit survival and quality of life, and results in a large burden of care for people with cystic fibrosis and their families. Furthermore, epidemiological studies in the past two decades have shown that cystic fibrosis occurs and is more frequent than was previously thought in populations of non-European descent, and the disease is now recognised in many regions of the world.

Prevention of anxiety and depression in cystic fibrosis.

Purpose Of Review: Anxiety and depression (A&D) are common in cystic fibrosis (CF). This review defines A&D in the context of CF and examines our current knowledge about A&D, the impact on CF, treatment and illness trajectory and explores potential areas for prevention.

Recent Findings: CF and A&D are interrelated.

Breaking bad news, the diagnosis of cystic fibrosis in childhood.

Background: The day parents are told their child has cystic fibrosis (CF) is imprinted in their memory. Parents often show strong emotions (e.g.

European Cystic Fibrosis Society Standards of Care: Framework for the Cystic Fibrosis Centre.

A significant increase in life expectancy in successive birth cohorts of people with cystic fibrosis (CF) is a result of more effective treatment for the disease. It is also now widely recognized that outcomes for patients cared for in specialist CF Centres are better than for those who are not. Key to the effectiveness of the specialist CF Centre is the multidisciplinary team (MDT), which should include consultants, clinical nurse specialist, microbiologist, physiotherapist, dietitian, pharmacist, clinical psychologist, social worker, clinical geneticist and allied healthcare professionals, all of whom should be experienced in CF care.

Belgian siblings of children with a chronic illness: Is their quality of life different from their peers?

To assess Belgian siblings' self-reported quality of life (QoL) and the impact of illness on four different paediatric illnesses. Healthy siblings (n = 131) of children with type 1 diabetes, cancer, congenital heart disease (CHD) and cystic fibrosis (CF) completed the Child Health Questionnaire and the Sibling Perception Questionnaire. Results were compared to those of a matched group of siblings of healthy children.

Lung clearance index predicts pulmonary exacerbations in young patients with cystic fibrosis.

Rationale: The lung clearance index (LCI) is a promising endpoint for use in cystic fibrosis (CF) clinical trials, but correlations with validated clinical endpoints have not yet been established.

Objective: This study aimed to demonstrate that, in young patients with CF, baseline LCI predicts subsequent pulmonary exacerbation (PE) and correlates with the respiratory domain of the CF Questionnaire-Revised (CFQ-Rresp).

Methods: Baseline LCI, forced expiratory volume in 1 s (FEV1), CFQ-Rresp and PEs over the subsequent year were prospectively recorded in 63 patients aged 5-19 years.

Pain in CF: review of the literature.

Background: This review evaluated pain research in cystic fibrosis (CF).

Methods: OVID MEDLINE, CINAHL, AMED, Web of Science, Pubmed, PsychINFO and PsychARTICLES were searched from January 1995-December 2012 to locate papers assessing pain in CF. A proforma was used to record the rationale for the study, characteristics of the sample, pain assessment tools, pain location, frequency and severity, treatment/self-management, coping and the impact on daily activities and quality of life.

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.

Communication of information about reproductive and sexual health in cystic fibrosis. Patients, parents and caregivers' experience.

Background: This review evaluated research concerning communication and information about reproductive and sexual health (RSH) in cystic fibrosis (CF).

Methods: Papers in the English language reporting RSH issues in CF, published between January 2000 and December 2010, were included. The review focused on (a) the content of information given to parents and patients, (b) the timing of information, (c) the sources of information, (d) attitudes and emotional reactions, (e) chronic illness and sexual behavior, (f) methodological and cultural considerations and (g) ethical considerations.

Adherence to the medical regimen: clinical implications of new findings.

Purpose Of Review: Treatment nonadherence is a common problem, yet adherence to treatments is important for the successful management of cystic fibrosis (CF). Previous work has concentrated on rates of adherence in children and adults using self-report questionnaires. Recent studies have employed new measurement methods and evaluated various treatment components.

Health related quality of life in cystic fibrosis: To work or not to work?

Background: The present study investigated whether patients with CF who are studying or working report a better HRQoL in comparison to non-working/studying patients.

Methods: 57 adult CF patients completed the Cystic Fibrosis Questionnaire-Revised, a CF-specific measure of HRQoL. Medical condition was quantified in terms of FEV1 % predicted, BMI, Pseudomonas aer.

Real life evaluation of intravenous antibiotic treatment in a paediatric cystic fibrosis centre: outcome of home therapy is not inferior.

Background: Limited data exist on the efficacy and safety of home intravenous antibiotic (IV-AB) therapy for pulmonary infection specifically in children with cystic fibrosis (CF).

Methods: We report on the outcome of IV-AB in the home vs hospital setting based on retrospective single centre patient data from 1999 to 2004 (age >5 and <18 years). Treatment location was chosen based on estimation of competence, adherence, social background and patient preference.

Quality of life in patients with Cystic Fibrosis: association with anxiety and depression.

Background: Few studies of patients with CF have looked at the association between patient reported Health-Related Quality of Life (HRQoL) and anxiety and depression. This study investigated whether CF patients with symptoms of anxiety or depression reported lower Health-Related Quality of Life (HRQoL) scores.

Methods: 57 adult CF patients completed the Hospital Anxiety and Depression Scale (HADS) and the Cystic Fibrosis Questionnaire, a CF-specific measure of HRQoL.

Cystic fibrosis: a balancing act?

Cystic fibrosis has evolved from an illness where children died in their teens to one with a longer life expectancy into adult life. Patients now usually keep themselves in a good physical condition, which gives them an opportunity to live a relatively 'normal' life. There is a catch: a patient can only achieve this by adhering to a strict, complex and time-consuming therapeutic schedule, thereby introducing an abnormal element into a 'normal' life.