Psychological distress is prevalent in people with COPD and relates to a worse course of disease. It often remains unrecognised and untreated, intensifying the burden on patients, carers and healthcare systems. Nonpharmacological management strategies have been suggested as important elements to manage psychological distress in COPD. Therefore, this review presents instruments for detecting psychological distress in COPD and provides an overview of available nonpharmacological management strategies together with available scientific evidence for their presumed benefits in COPD. Several instruments are available for detecting psychological distress in COPD, including simple questions, questionnaires and clinical diagnostic interviews, but their implementation in clinical practice is limited and heterogeneous. Moreover, various nonpharmacological management options are available for COPD, ranging from specific cognitive behavioural therapy (CBT) to multi-component pulmonary rehabilitation (PR) programmes. These interventions vary substantially in their specific content, intensity and duration across studies. Similarly, available evidence regarding their efficacy varies significantly, with the strongest evidence currently for CBT or PR. Further randomised controlled trials are needed with larger, culturally diverse samples and long-term follow-ups. Moreover, effective nonpharmacological interventions should be implemented more in the clinical routine. Respective barriers for patients, caregivers, clinicians, healthcare systems and research need to be overcome.

This is the final of four papers updating standards for the care of people with CF. That this paper "Planning a longer life" was considered necessary, highlights how much CF care has progressed over the past decade. Several factors underpin this progress, notably increased numbers of people with CF with access to CFTR modulator therapy. As the landscape for CF changes, so do the hopes and aspirations of people with CF and their families. This paper reflects the need to consider people with CF not as a "problem" to be solved, but as a success, a potential and a voice to be heard. People with CF and the wider CF community have driven this approach, reflecting many of the topics in this paper. This exercise involved wide stakeholder engagement. People with CF are keen to contribute to research priorities and be involved in all stages of research. People with CF want healthcare professionals to respect them as individuals and consider the impact of our actions on the world around us. Navigating life presents challenges to all, but for people with CF these challenges are heightened and complex. In this paper we highlight the concerns and life moments that impact people with CF, and events that the CF team should aim to support, including the challenges around having a family. People with CF and their care teams must embrace the updated standards outlined in these four papers to enjoy the full potential for a healthier life.

Individuals with cystic fibrosis (CF) are at increased risk for anxiety and depression, with negative consequences for adherence, health, and quality of life. New approaches to prevent and treat anxiety and depression that are tailored to the concerns of this population are needed. A CF-specific internet-based cognitive-behavioral therapy (CBT) intervention was developed to increase access to evidence-based mental health care and decrease cost and burden of care for people with CF. To evaluate the usability and acceptability of “eHealth CF-CBT,” an internet-based program integrating therapist-guided online self-management modules with in-person or virtual sessions. Dutch adults with CF (N = 16) and CF health care providers (N = 16) systematically tested all sessions of the eHealth CF-CBT program, provided qualitative feedback, and completed measures including eHealth Impact Questionnaire (eHIQ), and System Usability Scale (SUS). Patient and provider ratings of their overall impression of the eHealth CF-CBT program were high, with scores (mean; SD) of 8.3; 0.6 and 8.2; 0.8 respectively on a 10-point scale. Mean ratings of usability by patients (77.0/100) and providers (73.4/100) surpassed the SUS cut point for good favorability. Ratings (pooled mean; SD) on the assessed eHIQ domains Motivation and Confidence to Act (71.3; 10.0), Information and Presentation (78.9; 9.6), and Identification (62.0; 15.1) were positive, as were assessments of specific elements of session content and format. eHealth CF-CBT is the first therapist-guided internet-delivered CBT-based intervention for adults with CF. Initial evaluation with key stakeholders demonstrated high levels of acceptability and usability and provided input that was integrated to strengthen the program. Effectiveness testing in the Netherlands will be the next step, as well as future international adaptation and dissemination.

Background: Primary ciliary dyskinesia (PCD) might be a risk factor for the development of anxiety and depression. This study investigated the associations between anxiety, depression and health-related quality of life (HRQoL) in individuals with PCD and their caregivers.Methods: Children, adolescents and adults with PCD and their caregivers were invited to participate in a mental health screening programme. During regular yearly outpatient visits, measures of anxiety (GAD-7), depression (PHQ-9), HRQoL (QOL-PCD), lung function (forced expiratory volume in 1 s (FEV₁)) and body mass index (BMI)) were collected and associations of anxiety, depression and HRQoL were estimated.Results: A total of 103 individuals participated in the mental health screening programme. Elevated levels of anxiety (scores ≥10 on GAD-7) were found in 6% of adults (n=33), 14% of children (n=7), 6% of adolescents (n=17) and 20% of caregivers (n=46, 52% of mothers). Elevated depression levels (scores ≥10 on PHQ-9) were found in 18% of adults, 14% of children, 6% of adolescents and 11% of caregivers. Anxiety and depression were associated with scales on the QOL-PCD. Mothers reported higher anxiety scores than fathers (30%versus9%, p=0.03). A strong negative relationship was found between depression in caregivers and physical functioning (QOL-PCD) of the child. Anxiety and depression were not significantly associated with anxiety/depression in their child.Conclusion: This is the first study investigating anxiety and depression in individuals with PCD and their caregivers. Our results revealed elevated levels of anxiety and depression, which were associated with worse HRQoL. These results suggest the need for psychological support in PCD.