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Background: Regular airway clearance techniques (ACTs) and exercise are recommended for children with bronchiectasis, but current clinical practice and their predictors are unknown., Objective: We aimed to describe current use of ACTs and exercise among Australian children with bronchiectasis and identify associated predictors., Methods: Physiotherapy-specific data of 397 children (median age = 8 were extracted from the Australian Bronchiectasis Registry. A multivariate analysis was undertaken to identify predictors associated with the use of regular ACTs and physical exercise., Results: Regular ACTs were undertaken by 118 (30%) children while 192 (48%) engaged in regular exercise. Physical exercise was the most common ACT modality (n = 83, 20%). The likelihood of regular ACT increased in children whose sputum isolated Pseudomonas aeruginosa (OR = 3.88, 95% CI 1.69-8.89) and was 50% higher for every respiratory exacerbation in the previous 12-months that required hospitalization (OR = 1.50, 95% CI 1.15-1.95). For every year older in age, children had increased odds of engaging in physical exercise (OR = 1.21, 95% CI 1.08-1.34) or using an ACT device (OR = 1.21, 95% CI 1.05-1.34). Regular exercise was twice as likely in the presence of bibasal bronchiectasis (OR = 2.43, 95% CI 1.14-5.16), yet less likely in those with ≥1 hospitalizations in the previous 12-months (OR = 0.76, 0.95% CI 0.57-1.03)., Conclusion: Approximately one-third of children with bronchiectasis undertake regular ACTs while physical exercise was undertaken in approximately one in two children. Age, frequent respiratory exacerbations requiring hospitalization and the extent of disease are predictors of undertaking regular ACTs and exercise. Identification of these factors may assist in tailoring ACT, exercise and ACT modality prescription in clinical practice., (© 2024 Wiley Periodicals LLC.)

Background: Although airway clearance techniques (ACTs) and physical exercise are recommended for adults with bronchiectasis, there is little data on current practice and limited guidance predicting clinical approach., Objective: This study aimed to describe current ACT and exercise practice recorded by patients, and identify predictors of regular ACTs, ACT modalities and exercise., Methods: Physiotherapy-specific interventions, quality of life (Quality-of-Life Bronchiectasis questionnaire, QOL-B), demographics and disease severity were extracted from the Australian Bronchiectasis Registry. Multivariate analyses were undertaken to identify predictors of undertaking ACTs or exercise., Results: We included 461 patients; median age of 72 years (interquartile range 64-78 years). Regular ACT use was recorded by 266 (58 %) patients; the active cycle of breathing technique (n = 175, 74 %) was the most common technique. Regular exercise use was recorded by 213 (46 %) patients, with walking the most common form of exercise. A pulmonary rehabilitation referral was made for 90 (19.5 %) of patients. Regular ACT use was associated with a higher treatment burden on QOL-B (Odds ratio (OR) = 0.97, 95 % confidence interval (CI) 0.96 to 0.99). Regular exercise was more likely amongst patients with severe bronchiectasis compared to those with mild disease (OR = 9.46, 95 % CI 1.94 to 67.83) and in those with greater physical function on the QOL-B (OR = 1.02, 95 % CI 1.01 to 1.04)., Conclusion: Approximately half the adults in the registry report regular ACT or exercise; QOL and disease severity predict this engagement. This knowledge may guide the tailoring of ACTs and exercise prescription to optimise physiotherapy management in adults with bronchiectasis., Competing Interests: Declaration of competing interest P.G.M is principle investigator on Insmed, AbbVie, Sanof bronchiectasis trials. R.M.T is the principal investigator for Insmed, ZAMBON trials; Ad boards Beyond Air, AN2 trials. Nil other conflicts of interest were declared., (Copyright © 2024. Published by Elsevier Ltd.)

Competing Interests: PGM reports having been Chief Investigator in the Trikafta trial published in the New England Journal of Medicine 2019; receiving honoraria for lectures and presentations from Vertex, AstraZeneca, Limbic, and MedEd; and being a member of the Cystic Fibrosis Foundation (CFF) Data Safety Monitoring Board, the European Cystic Fibrosis Society Standards of Care Committee, the Australian Cystic Fibrosis Standards of Care Committee, the Australian Cystic Fibrosis Data Registry, and the Australian Bronchiectasis Registry. JLT-C reports having been site or national principal investigator on studies for 4DMT, Vertex, and Eloxx, as faculty at an institution that is part of the CFF Therapeutics Development Network; grant funding from CFF and the National Institutes of Health; payments to institution for clinical trial advising efforts from Vertex and 4DMT; travel support for participation in Board of Trustees meetings from CFF; payments for role as Chair of a data monitoring committee from AbbVie; serving as the adult patient care representative to the CFF Board of Trustees, on the CFF Clinical Research Executive Committee, Clinical Research Advisory Board, Racial Justice Working Group, as immediate past chair of the CFF Therapeutics Development Network's Sexual Health, Reproduction and Gender Research Working Group, as Co-Chair of the Heath Equity Team Science Awards study section, on the scientific advisory board for Emily's Entourage, on the American Thoracic Society Scientific Grant Review Committee, on the Respiratory Research Awards Committee, as Chair-Elect for the ATS International Conference Committee, and on the NHLBI Clinical Trials Study Section; and being a member of the International Advisory Board for The Lancet Respiratory Medicine and the Editorial Board for the Journal of Cystic Fibrosis.

Background: Factors associated with severe COVID-19 infection have been identified; however, the impact of infection on longer-term outcomes is unclear. The objective of this study was to examine the impact of COVID-19 infection on the trajectory of lung function and nutritional status in people with cystic fibrosis (pwCF)., Methods: This is a retrospective global cohort study of pwCF who had confirmed COVID-19 infection diagnosed between January 1, 2020 and December 31, 2021. Forced expiratory volume in one second percent predicted (ppFEV 1 ) and body mass index (BMI) twelve months prior to and following a diagnosis of COVID-19 were recorded. Change in mean ppFEV 1 and BMI were compared using a t-test. A linear mixed-effects model was used to estimate change over time and to compare the rate of change before and after infection., Results: A total of 6,500 cases of COVID-19 in pwCF from 33 countries were included for analysis. The mean difference in ppFEV 1 pre- and post-infection was 1.4 %, (95 % CI 1.1, 1.7). In those not on modulators, the difference in rate of change pre- and post-infection was 1.34 %, (95 % CI -0.88, 3.56) per year (p = 0.24) and -0.74 % (-1.89, 0.41) per year (p = 0.21) for those on elexacaftor/tezacaftor/ivacaftor. No clinically significant change was noted in BMI or BMI percentile before and after COVID-19 infection., Conclusions: No clinically meaningful impact on lung function and BMI trajectory in the year following infection with COVID-19 was identified. This work highlights the ability of the global CF community to unify and address critical issues facing pwCF., Competing Interests: Declaration of competing interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: The authors of this manuscript receive funding in the form of consulting fees or honoraria from Vertex Pharmaceuticals, Chiesi Pharmaceuticals, Enterprise Therapeutics, Gilead Sciences, GSK, Astra-Zeneca, Insmed, MSD, Sanofi, Viartis, Zambon, Limbic, Effrx Pharmaceuticals and Omron. Additionally, there is an author who serves on the Novartis Data and Safety Monitoring Board. There are several authors who are employed by the Cystic Fibrosis Foundation (CFF). The CFF, to advance drug development and search for a cure, have contracts with several companies to help fund the development of potential treatments and/or cures for cystic fibrosis. Pursuant to these contracts, CFF may receive milestone-based payments, equity interests, royalties on the net sales of therapies, and/or other forms of consideration. Resulting revenue received by CFF is used in support of our mission. See “How Drugs Get on the Pipeline” on the CFF website for more information. Additionally, CFF may license CFF Patient Registry data to some companies to monitor drug safety as part of the U.S. Food and Drug Administration's required Phase 4 clinical trials process and to encourage research aimed at improving the care of people with CF, while maintaining our obligation and commitment to protect the privacy of Registry participants. In connection with these licenses, and upon request, CFF may also assist company researchers in interpreting CFF Patient Registry data to aid in designing, analyzing, and interpreting real world studies in CF., (Copyright © 2024. Published by Elsevier B.V.)

Background: Lung inflammation is associated with tissue damage in cystic fibrosis (CF). LAU-7b, a novel oral drug candidate, was shown to control inflammation and stabilize CFTR protein in the epithelial membrane during inflammatory stress in preclinical models of CF., Methods: A double-blind, randomized, placebo-controlled Phase 2 study was conducted to evaluate efficacy and safety of LAU-7b in adults with CF. LAU-7b or placebo was administered over 24 weeks as six 21-day treatment cycles each separated by 7 days. The primary efficacy endpoint was the absolute change from baseline in percent predicted forced expiratory volume in 1 second (ppFEV 1 ) at 24 weeks., Results: A total of 166 subjects received at least one dose of study drug (Intent-To-Treat population, ITT), of which 122 received ≥5 treatment cycles (Per-Protocol population, PP). Both treatment arms showed a mean lung function loss at 24 weeks of 1.18 ppFEV 1 points with LAU-7b and 1.95 ppFEV 1 with placebo, a 0.77 ppFEV 1 (40 s) difference, p=0.345, and a 0.95 ppFEV 1 (49 %) difference in the same direction in PP population, p=0.263. Primary analysis of mean ppFEV 1 through 24 weeks showed differences of 1.01 and 1.23 ppFEV 1 , in the ITT (65 % less loss, p=0.067) and PP populations (78 % less loss, reaching statistical significance p=0.049), respectively. LAU-7b had an acceptable safety profile., Conclusion: Although the study did not meet its primary efficacy endpoint in the ITT population, LAU-7b was generally well tolerated and showed evidence of preservation of lung function to support further development., Competing Interests: Declaration of competing interest MW Konstan, LC Lands, D Potvin and D Radzioch report receiving consulting fees from Laurent Pharmaceuticals; JM Houle, R Pislariu, P Colin, I Kianicka are employees of Laurent Pharmaceuticals; D Polineni, J Chmiel, L Bilodeau, PG Middleton, E Matouk, T Kotsimbos, JB Zuckerman, SZ Nasr, TG Liou report no conflict of interest related to this study., (Copyright © 2024. Published by Elsevier B.V.)

This is the third paper in the series providing updated information and recommendations for people with cystic fibrosis transmembrane conductance regulator (CFTR)-related disorder (CFTR-RD). This paper covers the individual disorders, including the established conditions - congenital absence of the vas deferens (CAVD), diffuse bronchiectasis and chronic or acute recurrent pancreatitis - and also other conditions which might be considered a CFTR-RD, including allergic bronchopulmonary aspergillosis, chronic rhinosinusitis, primary sclerosing cholangitis and aquagenic wrinkling. The CFTR functional and genetic evidence in support of the condition being a CFTR-RD are discussed and guidance for reaching the diagnosis, including alternative conditions to consider and management recommendations, is provided. Gaps in our knowledge, particularly of the emerging conditions, and future areas of research, including the role of CFTR modulators, are highlighted., Competing Interests: Declaration of competing interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: NJS reports consulting fees and/or honoraria from Vertex, Chiesi, Gilead and Menarini. KDB reports consulting fees and/or honoraria from Vertex, ELoxx, Splisense and Arcturus. JGM reports honoraria from Vertex, Chiesi, Abbvie and Viatris. PGM reports consulting fees and/or honoraria from Vertex, AstraZenica, Limbic and MedEd. CS reports consulting fees and/or honoraria from Vertex, Chiesi, TFF and Pfizer. JC reports consulting fees for Astrazeneca, Boehringer Ingelheim, Grifols, Gilead sciences, Insmed, Genentech, Glaxosmithkline, Antabio, Zambon and Trudell. OCY reports consulting fees and honoraria from Vertex. DGD reports consulting fees and/or honoraria from Chiesi, Insmed and Vertex. DP reports payments for educational talks. DVD reports consulting fees for Arcturus, Arrevus, BiomX, Clarametyx, CFF, Enbiotix, Microbion, Pyros, Respirion and Zambon. ISG reports honoraria from Vertex. KWS, FB, VV, EDW, MW, EB, DD, PE, EG, GH, AK, DN, JAN, CR, IS, MDW report no personal fees/honoraria, (Copyright © 2024. Published by Elsevier B.V.)

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., Competing Interests: Declaration of competing interest The authors had no declarations of interest in relation to the current work. Declarations of interest for each author outside the current work are summarised in Supplementary Table 4., (Copyright © 2024. Published by Elsevier B.V.)

Sputum induction is widely used in clinical settings for collection of biological samples from the lower airways. However, in recent years sputum induction has been associated with serious adverse events and even death. This position statement was commissioned by the Thoracic Society of Australia and New Zealand to address major adverse events of two deaths associated with sputum induction that have occurred in Australia in 2021, and outlines best practice for the safe use of sputum induction. The statement resulted from systematic literature searches by a multi-disciplinary group including respiratory physicians, nurses and physiotherapists (paediatric and adults focused). Consumers had input to an advanced draft of the position statement. The position statement covers indications for sputum induction, informed consent, scope of practice of personnel administering the procedure, infection control considerations, details about the sputum induction procedure, safety considerations and risk assessment in clinical settings., (© 2024 The Authors. Respirology published by John Wiley & Sons Australia, Ltd on behalf of Asian Pacific Society of Respirology.)

Background: Most males with cystic fibrosis (mwCF) are infertile but with CF transmembrane conductance regulator (CFTR) modulator-conferred benefits, more are utilizing assisted reproductive technologies (ART). Administration of normal human doses of modulators in animal reproductive models caused no genotoxicity; no human data exists. Potential health decline following modulator discontinuation makes the decision to withhold therapy during reproduction challenging., Methods: From August-October 2021, international CF clinicians completed an anonymous questionnaire regarding mwCF who used modulators during reproduction., Results: We received 42 surveys for mwCF with partner pregnancies. Forty of 42 mwCF utilized ART; 35 continued modulators during sperm retrieval and 40/42 during partner pregnancy. One of four males who discontinued modulators experienced clinical deterioration. First trimester miscarriages occurred in 11.9 % of partner pregnancies. No congenital anomalies were reported., Conclusions: Use of CFTR modulators during reproduction and partner pregnancy in mwCF did not result in a higher-than-expected miscarriage rate nor congenital anomalies., Competing Interests: Declaration of Competing Interest Jennifer L. Taylor-Cousar, MD, MSCS, ATSF Personal financial relationships with commercial interests relevant to medicine within the past 3 years: As faculty in an institution that is part of the CF TDN, I have been site PI on studies for Vertex, 4DMT, and Eloxx. I have done consulting/provided clinical trial design advice for Vertex and 4DMT. I served as Chair of a Data Monitoring Committee for AbbVie. Personal financial support from a non-commercial source relevant to medicine within the past 3 years: I have received grant funding from the CFF and NIH. Personal relationships with tobacco industry entities within the last 3 years: No relationships to disclose. Professional Memberships CFF Clinical Research Executive Committee CFF TDN Sexual Health, Reproduction, and Gender Research Working Group (SHARING) ATS International Conference Committee Chair and Respiratory Health Awards Committee Member National Institutes of Health/National Heart, Blood, Lung Institute Clinical Trials Review Study Section Rachel Janney No relationships to disclose. Peter G. Middleton, MD, PhD, FRACP, FThorSoc. Personal financial relationships with commercial interests relevant to medicine within the past 3 years: I have been site PI on studies for Vertex including the phase III Trikafta study. I have done medico-legal consulting for Shine Lawyers. I have provided lectures and presentations on CF, asthma and COVID amongst other topics for Vertex Pharma, AstraZeneca, Limbic, and MedEd. Family members have shareholdings in Resmed Inc and Sanofi Pharma. Personal financial support from a non-commercial source relevant to medicine within the past 3 years: I have received grant funding from HCF Australia and the Canadian Institutes of Health. I have received support for attending meetings from NSW Health. Personal relationships with tobacco industry entities within the last 3 years: No relationships to disclose. Professional Memberships I am Chair of the Australian Bronchiectasis Registry. I serve on steering committees for the Australian CF Data Registry, the Australian Standards of Care and the ECFS Standards of Care. Raksha Jain, MD, MSc Personal financial relationships with commercial interests relevant to medicine within the past 3 years: As faculty in an institution that is part of the CF TDN, I have been site PI on studies for Vertex, 4DMT, Sound Pharma, Genentech, Armata. I have done consulting/provided clinical trial design advice for Boerhinger-Ingelheim and Recode. I served on a Data Monitoring Committee for Syneos. Personal financial support from a non-commercial source relevant to medicine within the past 3 years: I have received grant funding from the CFF and NIH. Personal relationships with tobacco industry entities within the last 3 years: No relationships to disclose. Professional Memberships CFF TDN Sexual Health, Reproduction, and Gender Research Working Group (SHARING) American Thoracic Society American College of Chest Physicians Julia A. Nightingale BA, BM BCh, MRCP, PhD Personal financial relationships with commercial interests relevant to medicine within the past 3 years: No relationships to disclose. Personal financial support from a non-commercial source relevant to medicine within the past 3 years: I have received grant funding from Chiesi UK. Personal relationships with tobacco industry entities within the last 3 years: No relationships to disclose. Professional Memberships Member of the Royal College of Physicians Adult representative on the UK Cystic Fibrosis Medical Association executive committee Michal Shteinberg Personal financial relationships with commercial interests relevant to medicine within the past 3 years: I have received grants/research support from: GSK, Insmed, Novartis, Trudell pharma, Tel Aviv League for lung diseases; consultation fees: Astra Zeneca, Boehringer Ingelheim, Dexcel, GSK, Kamada, Syncrony-medical, Trumed, Zambon; speaker's fees from Astra Zeneca, Boeringer Ingelheim, GSK, Kamada, Novartis, PhysioAssist, Sanofi, Teva. I serve as a DSMB member for – Bonus biotherapeutics. Personal financial support from a non-commercial source relevant to medicine within the past 3 years: Member of management board of EMBARC; Professional Memberships Executive committee of Israel Society for TB and Mycobacterial diseases; Associate editor- Am J Resp Crit Care med; Editorial board member- Eur Resp J, Chest. Personal relationships with tobacco industry entities within the last 3 years: No relationships to disclose. Danielle Velez, MD Personal financial relationships with commercial interests relevant to medicine within the past 3 years: No relationships to disclose. Personal financial support from a non-commercial source relevant to medicine within the past 3 years: I have received grant funding from the CFF. Personal relationships with tobacco industry entities within the last 3 years: No relationships to disclose. Professional Memberships I am member of the CFF Men's Health Research Subgroup of the CFF TDN Sexual Health, Reproduction, and Gender Research Working Group (SHARING) Natalie E. West, MD, MHS Personal financial relationships with commercial interests relevant to medicine within the past 3 years: As faculty in an institution that is part of the CF TDN, I have been site PI on studies for Corbus, Armata and co-investigator on studies for Vertex. I have done consulting/provided clinical trial design advice for Vertex and Armata. Personal financial support from a non-commercial source relevant to medicine within the past 3 years: I have received grant funding from the CFF and NIH. Personal relationships with tobacco industry entities within the last 3 years: No relationships to disclose. Professional Memberships CFF TDN Sexual Health, Reproduction, and Gender Research Working Group (SHARING) American Thoracic Society Traci M. Kazmerski, MD, MS Personal financial relationships with commercial interests relevant to medicine within the past 3 years: No relationships to disclose. Personal financial support from a non-commercial source relevant to medicine within the past 3 years: I have received grant funding from the CFF and NIH. Personal relationships with tobacco industry entities within the last 3 years: No relationships to disclose. Professional Memberships CFF TDN Sexual Health, Reproduction, and Gender Research Working Group (SHARING) American Thoracic Society, (Copyright © 2023 The Authors. Published by Elsevier B.V. All rights reserved.)

This is the third in a series of four papers updating the European Cystic Fibrosis Society (ECFS) standards for the care of people with CF. This paper focuses on recognising and addressing CF health issues. The guidance was produced with wide stakeholder engagement, including people from the CF community, using an evidence-based framework. Authors contributed sections, and summary statements which were reviewed by a Delphi consultation. Monitoring and treating airway infection, inflammation and pulmonary exacerbations remains important, despite the widespread availability of CFTR modulators and their accompanying health improvements. Extrapulmonary CF-specific health issues persist, such as diabetes, liver disease, bone disease, stones and other renal issues, and intestinal obstruction. These health issues require multidisciplinary care with input from the relevant specialists. Cancer is more common in people with CF compared to the general population, and requires regular screening. The CF life journey requires mental and emotional adaptation to psychosocial and physical challenges, with support from the CF team and the CF psychologist. This is particularly important when life gets challenging, with disease progression requiring increased treatments, breathing support and potentially transplantation. Planning for end of life remains a necessary aspect of care and should be discussed openly, honestly, with sensitivity and compassion for the person with CF and their family. CF teams should proactively recognise and address CF-specific health issues, and support mental and emotional wellbeing while accompanying people with CF and their families on their life journey., Competing Interests: Declaration of competing interest The authors had no declarations of interest in relation to the current work. Declarations of interest for each author outside the current work are summarised in Supplementary Table 4., (Copyright © 2024. Published by Elsevier B.V.)

This is the second in a series of four papers updating the European Cystic Fibrosis Society (ECFS) standards for the care of people with CF. This paper focuses on establishing and maintaining health. The guidance is produced using an evidence-based framework and with wide stakeholder engagement, including people from the CF community. Authors provided a narrative description of their topic and statements, which were more directive. These statements were reviewed by a Delphi exercise, achieving good levels of agreement from a wide group for all statements. This guidance reinforces the importance of a multi-disciplinary CF team, but also describes developing models of care including virtual consultations. The framework for health is reinforced, including the need for a physically active lifestyle and the strict avoidance of all recreational inhalations, including e-cigarettes. Progress with cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy is reviewed, including emerging adverse events and advice for dose reduction and interruption. This paper contains guidance that is pertinent to all people with CF regardless of age and eligibility for and access to modulator therapy., Competing Interests: Declaration of competing interest KWS has no competing interests. See supplementary materials for full CoI statements for all authors., (Copyright © 2023. Published by Elsevier B.V.)

Introduction: Throughout the transition to motherhood, changes are experienced across a woman's physical, mental, social, and occupational self. Maternal chronic illness adds the complexity of increased healthcare needs and navigating a high-risk, medicalised pregnancy, birth, and post-natal period. Literature concerning motherhood transitions in chronic illness generally focusses on the mother's medical health and pregnancy outcomes; little is known about the impacts on women's occupations, balance, and quality of life. Understanding these issues may help support women in a more tailored and holistic way., Objective: This scoping review aims to gather, analyse, and synthesise existing empirical research on occupational engagement and occupational balance as they impact on wellbeing and quality of life in women with a lifetime chronic illness before and during pregnancy and in early motherhood., Method: The review follows the nine-stage framework described in the Joanna Briggs Institute Manual for Evidence Synthesis (2020). Five databases were searched: Embase, Medline, PsycINFO, CINAHL, Scopus, and OT Seeker. Data were extracted and examined via content analysis, described in narrative synthesis, summarised into a conceptual framework, and tabulated., Findings: A total of 8,655 papers were discovered on initial search. Following title and abstract screening, 220 full-text studies were assessed for eligibility, and 46 papers were finally included. Analysis generated four major themes: The Disrupted Transition Journey; Adaptation, Compromise and Choice; Outcomes; and Drawing on What's Available. The themes were conceptualised into a framework to explain how women sought to balance motherhood and illness-related occupations. Adequate access to information, social support, expert care, and financial resources improved both quality of life and healthcare compliance., Conclusion: Findings of this scoping review deepen the understanding of occupational balance during the transition to motherhood in the context of lifetime chronic illness. Healthcare providers and supportive family and friends can use this knowledge to adapt their approach to assisting women with chronic illness on the motherhood journey. These findings may also inform further inquiry into the scope of occupational therapy practice with this population., (© 2023 The Authors. Australian Occupational Therapy Journal published by John Wiley & Sons Australia, Ltd on behalf of Occupational Therapy Australia.)

There is considerable activity with respect to diagnosis in the field of cystic fibrosis (CF). This relates primarily to developments in newborn bloodspot screening (NBS), more extensive gene analysis and improved characterisation of CFTR-related disorder (CFTR-RD). This is particularly pertinent with respect to accessibility to variant-specific therapy (VST), a transformational intervention for people with CF with eligible CFTR gene variants. This advance reinforces the need for a timely and accurate diagnosis. In the future, there is potential for trials to assess effectiveness of variant-specific therapy for CFTR-RD. The guidance in this paper reaffirms previous standards, clarifies a number of issues, and integrates emerging evidence. Timely and accurate diagnosis has never been more important for people with CF., Competing Interests: Declaration of Competing Interest None., (Copyright © 2023. Published by Elsevier B.V.)

Background: In people with cystic fibrosis (CF), regular nebulisation of 6% or 7% saline improves lung function; however, these concentrations are not always tolerable. Clinically, some CF patients report using lower concentrations of saline to improve tolerability, yet the effects of lower concentrations are unknown. This study therefore aimed to evaluate the relative effectiveness and tolerability of 0.9% versus 3% versus 6% saline nebulised twice daily with an eFlow rapid nebuliser., Methods: This was a randomised, blinded, placebo-controlled, parallel-group, multicentre study where subjects inhaled 4 mL of 0.9%, 3% or 6% saline twice daily for 16 weeks. The primary outcome was forced expiratory volume in 1 s. The secondary outcomes were: forced vital capacity (FVC) and forced expiratory flow at 25-75% of FVC; quality of life; exercise capacity; acquisition or loss of bacterial organisms in expectorated sputum; tolerability of nebulised saline; pulmonary exacerbations; and adverse events., Results: 140 participants were randomised to 0.9% (n=47), 3% (n=48) or 6% (n=45) saline. 134 participants (96%) contributed to the intention-to-treat analysis. 3% saline significantly improved lung function and increased the time to first pulmonary exacerbation compared with 0.9% saline but did not improve quality of life. 6% saline had similar benefits to 3% saline but also significantly improved quality of life compared with 3% saline. Only 6% saline delayed the time to intravenous antibiotics for pulmonary exacerbation. Tolerability and adherence were similar., Conclusions: Dilution of 6% saline to 3% maintains the benefits for lung function and exacerbation prevention; however, the positive impacts of 6% saline on quality of life and time to i.v. antibiotics for pulmonary exacerbations are lost., Competing Interests: Conflict of interest: None of the authors has a conflict of interest to disclose apart from the funding and sources of support listed., (Copyright ©The authors 2023. For reproduction rights and permissions contact permissions@ersnet.org.)

This chapter aims to provide expert guidance to obstetricians, general practitioners, allied health staff and women with lung disease about the interactions between pregnancy and different lung diseases. This chapter will cover other airway diseases such as bronchiectasis and cystic fibrosis (CF) together with sarcoidosis and interstitial lung disease (ILD), noting that another chapter covers asthma. The physiological changes which occur in pregnancy, such as the changes in airway physiology, resting ventilation and sleep, are summarised in another chapter. This chapter extends the evidence-based approach and clinical expertise of the recent European Respiratory Society/Thoracic Society of Australia and New Zealand (ERS/TSANZ) taskforce. The papers selected were based on the population (pregnant women with lung disease other than asthma) and the effects of these diseases on risks of pregnancy-associated complications and miscarriages, breastfeeding, nutritional considerations, lung function, long-term maternal outcomes and management considerations during pregnancy. As there are very few randomised control trials in the area, the majority of the literature consists of observational studies (prospective or retrospective), cross-sectional surveys and case series. Other guidelines have also recently been published, which may be helpful to the reader., Competing Interests: Declaration of competing interest AJH is currently a PhD student examining occupational engagement issues in pregnant women with disease, PGM was the chair of the ERS/TSANZ task force on the management of reproduction and pregnancy in women with airway diseases and is a PhD supervisor for AJH., (Copyright © 2022. Published by Elsevier Ltd.)

This review provides a summary for obstetricians, midwives, other health professionals and women contemplating pregnancy about the interactions between pregnancy and breathing during sleep. This review will first examine the normal physiological changes of pregnancy and their relationship to sleep-disordered breathing (SDB), and it will then summarise the current knowledge of SDB in pregnancy. Many changes in the respiratory system during pregnancy, particularly during the third trimester, can alter respiratory function during sleep, increasing the incidence and severity of SDB. These changes include increased ventilatory drive and metabolic rate, reduced functional residual capacity and residual volume, increased alveolar-arterial oxygen gradients and changes in the upper airway. The clinical importance of these changes during pregnancy is demonstrated by the increased incidence of snoring and obstructive sleep apnoea hypopnoea syndrome. As SDB is associated with obesity, the increasing incidence of obesity pre-pregnancy will likely increase SDB during pregnancy over the next decade. If a physician is asked to review a pregnant patient, they should always consider the possibility of SDB., Competing Interests: Declaration of competing interest PGM was the Chair of the ERS/TSANZ task force on the Management of Reproduction and Pregnancy in Women with Airways Diseases., (Copyright © 2022. Published by Elsevier Ltd.)

Survival statistics, estimated using data from national cystic fibrosis (CF) registries, inform the CF community and monitor disease progression. This study aimed to estimate survival among people with CF in Australia and to identify factors associated with survival. This population-based cohort study used prospectively collected data from 23 Australian CF centres participating in the Australian CF Data Registry (ACFDR) from 2005-2020. Period survival analysis was used to calculate median age of survival estimates for each 5-year window from 2005-2009 until 2016-2020. The overall median survival was estimated using the Kaplan-Meier method. Between 2005-2020 the ACFDR followed 4,601 people with CF, noting 516 (11.2%) deaths including 195 following lung transplantation. Out of the total sample, more than half (52.5%) were male and 395 (8.6%) had undergone lung transplantation. Two thirds of people with CF (66.1%) were diagnosed before six weeks of age or by newborn/prenatal screening. The overall median age of survival was estimated as 54.0 years (95% CI: 51.0-57.04). Estimated median survival increased from 48.9 years (95% CI: 44.7-53.5) for people with CF born in 2005-2009, to 56.3 years (95% CI: 51.2-60.4) for those born in 2016-2020. Factors independently associated with reduced survival include receiving a lung transplant, having low FEV 1 pp and BMI. Median survival estimates are increasing in CF in Australia. This likely reflects multiple factors, including newborn screening, improvement in diagnosis, refinements in CF management and centre-based multidisciplinary care., (© 2022. The Author(s).)

Background: This international study aimed to characterise the impact of acute SARS-CoV-2 infection in people with cystic fibrosis and investigate factors associated with severe outcomes. Methods Data from 22 countries prior to 13 th December 2020 and the introduction of vaccines were included. It was de-identified and included patient demographics, clinical characteristics, treatments, outcomes and sequalae following SARS-CoV-2 infection. Multivariable logistic regression was used to investigate factors associated with clinical progression to severe COVID-19, using the primary outcome of hospitalisation with supplemental oxygen., Results: SARS-CoV-2 was reported in 1555 people with CF, 1452 were included in the analysis. One third were aged <18 years, and 9.4% were solid-organ transplant recipients. 74.5% were symptomatic and 22% were admitted to hospital. In the non-transplanted cohort, 39.5% of patients with ppFEV1<40% were hospitalised with oxygen verses 3.2% with ppFEV >70%: a 17-fold increase in odds. Worse outcomes were independently associated with older age, non-white race, underweight body mass index, and CF-related diabetes. Prescription of highly effective CFTR modulator therapies was associated with a significantly reduced odds of being hospitalised with oxygen (AOR 0.43 95%CI 0.31-0.60 p<0.001). Transplanted patients were hospitalised with supplemental oxygen therapy (21.9%) more often than non-transplanted (8.8%) and was independently associated with the primary outcome (Adjusted OR 2.45 95%CI 1.27-4.71 p=0.007)., Conclusions: This is the first study to show that there is a protective effect from the use of CFTR modulator therapy and that people with CF from an ethnic minority are at more risk of severe infection with SARS-CoV-2., Competing Interests: COI Statement All authors declare no conflicts of interest in relationship to this work. Outside of this work the following authors declare payments or honoraria to them or their institution for a combination of lectures, presentations, educational events, advisory boards, steering groups, grants or consultancy fees: SC-Vertex, Chiesi, Profile, Zambon. RC- Vertex, P-RB – Astra-Zeneca, Boehringer Ingelheim, GSK, Insmed, Chiesi, Pfizer, Vertex, Zambon. I deM – Vertex,LN – Vertex, Boehringer,LVS-F – Vertex, AS -Vertex., (Copyright © 2022. Published by Elsevier B.V.)

In developed countries, it is projected that there will be a 70% increase in the number of adults living with Cystic Fibrosis (CF) between 2010 and 2025. This shift in demographics highlights the importance of high-quality transition programmes with developmentally appropriate integrated health care services as the individual moves through adolescence to adulthood. Adolescents living with CF face additional and unique challenges that may have long-term impacts on their health, quality of life and life-expectancy. CF specific issues around socially challenging symptoms, body image, reproductive health and treatment burden differentiate people with CF from their peers and require clinicians to identify and address these issues during the transition process. This review provides an overview of the health, developmental and psychosocial challenges faced by individuals with CF, their guardians and health care teams considering the fundamental components and tools that are required to build a transition programme that can be tailored to suit individual CF clinics., (Crown Copyright © 2020. Published by Elsevier Ltd. All rights reserved.)

Lung diseases can complicate pregnancy, but little is known about the experiences of pregnancy among women living with such diseases. This survey aimed to understand the experiences of women with a lung condition before and during pregnancy, in childbirth and post-partum. The survey was translated into nine languages and hosted online between March and May 2018. This paper reports on 327 women who had asthma, cystic fibrosis (CF), lymphangioleiomyomatosis (LAM) and sarcoidosis as a sole or primary lung condition. Women with CF and LAM were most likely to report that their condition influenced their decision to have children. Those with CF and LAM who did become pregnant reported greater satisfaction with their healthcare during pregnancy and gave more consideration to factors such as location and type of birth; they were also more concerned about the impact of the pregnancy on their health than women with other diseases. Women with sarcoidosis reported receiving conflicting advice as to both the impact of their condition on pregnancy and how becoming pregnant might impact their health. Women with asthma reported not always being able to access the information they needed from healthcare professionals. The results suggest that healthcare providers should be having dialogues with affected women early on, from before conception, throughout the pregnancy and after giving birth, and training should be provided to healthcare staff to equip them with the information they need to do this., Competing Interests: Conflict of interest: C. Williams is an employee of the European Lung Foundation. Conflict of interest: B. Johnson was an employee of the European Lung Foundation at the time of the study. Conflict of interest: P.G. Gibson reports support for the present manuscript from the National Health and Medical Research Council; and grants of contracts from AstraZeneca and GlaxoSmithKline, payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events for AstraZeneca, GlaxoSmithKline and Novartis, and participation on data safety monitoring or advisory boards for Chiesi and Sanofi, outside the submitted work. Conflict of interest: V. Backer has nothing to disclose. Conflict of interest: G. Hollis has nothing to disclose. Conflict of interest: C. Coleman is an employee of the European Lung Foundation., (Copyright ©The authors 2021.)

Introduction : Cystic fibrosis (CF), the most common life-shortening inherited disorder in people of European descent, also occurs in other ethnicities. The identification of the disease, the isolation of the causative gene, termed the cystic fibrosis transmembrane conductance regulator ( CFTR ) and the improved survival from comprehensive multidisciplinary treatment is one of the success stories of modern medicine. Survival has increased dramatically over the last 50 years, from 10 years in the 1960s to 30 years in the 1990s and approximately 50 years currently. Areas covered : This review will examine the development of highly effective modulators for CF which will revolutionize therapy for more than 90% of the people with CF. This review summarizes the development of triple combination CFTR modulator elexacaftor-tezacaftor-ivacaftor. Expert opinion : The development of this highly effective CFTR modulator for the majority of people with CF will likely change the landscape of CF care. The challenge is to now find highly effective therapy for the remaining 10% of the people with CF who may need other therapeutic agents to correct their primary defect.

Clinical registries that monitor and review outcomes for patients with cystic fibrosis have existed internationally for many decades. However, their purpose continues to evolve and now includes the capability to support clinical effectiveness research, clinical trials and Phase IV studies, and international data comparisons and projects. To achieve this, registries must regularly update the information that they collect and ensure design that is adaptable and flexible to changing needs. The Australian Cystic Fibrosis Data Registry commenced in 1998, and in 2018-19 undertook a transformation to enable it to meet the needs of multiple stakeholders into the future. This included a comprehensive, multidisciplinary review of the registry's data elements, and a redesign and rebuild of the registry's database. The data element review comprised the processes of alignment, comparison, selection, consolidation, revision and definition of finalised data elements. The database redesign included attention to each of the registry functions of data collection, storage and management, and reporting. The revision of a national data collection system is a time-intensive process, and requires significant clinical and other expert engagement. The resulting database, while being continually refined, is now fit for purpose to support Australian clinicians and patients with CF to receive best practice care., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

The Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene is expressed in the heart, but this is not known to cause myocardial dysfunction or abnormalities in the ECG in people with CF. CFTR modulators such as tezacaftor/ivacaftor improve lung function and overall health in people with CF. Minor adverse events have been reported in the early clinical trials, but no consistent changes in the ECGs have been reported. This case highlights an unusual side effect of first degree heart block that occurred after more than 8 months of azithromycin and tezacaftor/ivacaftor in combination. Drug withdrawal and reintroduction confirmed that neither drug alone, but only the combination, caused this change. As tezacaftor/ivacaftor is also present in elexacaftor/tezacaftor/ivacaftor, care may be needed to exclude this delayed interaction with azithromycin., Competing Interests: Declaration of Competing Interest ACP has been involved in the clinical trials of tezacaftor/ivacaftor at Westmead; PGM reports advisory board work for Vertex Pharmaceuticals and was lead author of the pivotal phase III trial of elexacaftor/tezacaftor/ivacaftor; PGM has also been involved in clinical trials of tezacaftor/ivacaftor., (Copyright © 2020. Published by Elsevier B.V.)

Background: Patient-oriented research approaches that reflect the needs and priorities of those most affected by health research outcomes improves translation of research findings into practice. Targeted therapies for cystic fibrosis (CF) are now a viable treatment option for some eligible individuals despite the heterogeneous patient-specific therapeutic response. This has necessitated development of a clinical tool that predicts treatment response for individual patients. Patient-derived mini-organs (organoids) have been at the forefront of this development. However, little is known about their acceptability in CF patients and members of the public., Methods: We used a cross-sectional observational design to conduct an online survey in people with CF, their carers and community comparisons. Acceptability was examined in five domains: 1) willingness to use organoids, 2) perceived advantages and disadvantages of organoids, 3) acceptable out-of-pocket costs, 4) turnaround time and 5) source of tissue., Results: In total, 188 participants completed the questionnaire, including adults with CF and parents of children with CF (90 (48%)), and adults without CF and parents of children without CF (98 (52%)). Use of organoids to guide treatment decisions in CF was acceptable to 86 (95%) CF participants and 98 (100%) community participants. The most important advantage was that organoids may improve treatment selection, improving the patient's quality of life and life expectancy. The most important disadvantage was that the organoid recommended treatment might be unavailable or too expensive., Conclusions: These findings indicate acceptance of patient-derived organoids as a tool to predict treatment response by the majority of people surveyed. This may indicate successful future implementation into healthcare systems., Competing Interests: Conflict of interest: L.K. Fawcett has nothing to disclose. Conflict of interest: C.E. Wakefield has nothing to disclose. Conflict of interest: S. Sivam has nothing to disclose. Conflict of interest: P.G. Middleton has nothing to disclose. Conflict of interest: P. Wark has nothing to disclose. Conflict of interest: J. Widger has nothing to disclose. Conflict of interest: A. Jaffe has nothing to disclose. Conflict of interest: S.A. Waters has nothing to disclose., (Copyright ©ERS 2021.)

With the growing SARS-CoV-2 pandemic, we need to better understand its impact in specific patient groups like those with Cystic Fibrosis (CF). We report on 181 people with CF (32 post-transplant) from 19 countries diagnosed with SARS-CoV-2 prior to 13 June 2020. Infection with SARS-CoV-2 appears to exhibit a similar spectrum of outcomes to that seen in the general population, with 11 people admitted to intensive care (7 post-transplant), and 7 deaths (3 post-transplant). A more severe clinical course may be associated with older age, CF-related diabetes, lower lung function in the year prior to infection, and having received an organ transplant. Whilst outcomes in this large cohort are better than initially feared overall, possibly due to a protective effect of the relatively younger age of the CF population compared to other chronic conditions, SARS-CoV-2 is not a benign disease for all people in this patient group., Competing Interests: Declaration of Competing Interest The authors have no conflicts of interest to declare relating to this work., (Copyright © 2020 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.)

Background: As their long-term prognosis improves, women with CF are increasingly choosing to have children, but the safety of CFTR modulators in pregnancy and breastfeeding is currently unknown., Methods: A survey was sent to lead clinicians of adult CF centres in Europe, the United Kingdom (UK), United States of America (USA), Australia and Israel requesting anonymised data on pregnancy outcomes in women using CFTR modulators before and during pregnancy and lactation., Results: We identified 64 pregnancies in 61 women taking IVA (n = 31), LUM/IVA (n = 26) or TEZ/IVA (n = 7), resulting in 60 live births. In 44 pregnancies, CFTR modulators were either continued throughout pregnancy or temporarily stopped and then restarted. Two maternal complications were deemed related to CFTR modulator therapy; cessation of modulator therapy resulted in clinical decline in 9 women prompting resumption of therapy during pregnancy. No modulator-related complications were reported in infants exposed in utero and/or during breastfeeding., Conclusions: CFTR modulators were reported to be generally well tolerated in pregnancy and breastfeeding, with only 2 maternal complications that were deemed related to CFTR modulator therapy. Women stopping CFTR modulators in pregnancy may experience a decline in clinical status and in the cases identified in this survey, restarting therapy led to a clinical improvement. Current experience remains limited and longer-term prospective follow-up is required to exclude delayed adverse effects., (Copyright © 2020. Published by Elsevier B.V.)

Information is lacking on the clinical impact of the novel coronavirus, SARS-CoV-2, on people with cystic fibrosis (CF). Our aim was to characterise SARS-CoV-2 infection in people with cystic fibrosis., Methods: Anonymised data submitted by each participating country to their National CF Registry was reported using a standardised template, then collated and summarised., Results: 40 cases have been reported across 8 countries. Of the 40 cases, 31 (78%) were symptomatic for SARS-CoV-2 at presentation, with 24 (60%) having a fever. 70% have recovered, 30% remain unresolved at time of reporting, and no deaths have been submitted., Conclusions: This early report shows good recovery from SARS-CoV-2 in this heterogeneous CF cohort. The disease course does not seem to differ from the general population, but the current numbers are too small to draw firm conclusions and people with CF should continue to strictly follow public health advice to protect themselves from infection., Competing Interests: Declaration of Competing Interest The authors have no conflicts of interest to declare relating to this work., (Copyright © 2020. Published by Elsevier B.V.)

As life expectancy for those with cystic fibrosis (CF) now exceeds 40 years of age, adult hospitals away from specialized CF services are being exposed more frequently to people with acute complications of CF. Well-known manifestations of CF include pulmonary disease and pancreatic insufficiency with malabsorption. However, a less well-known entity is distal intestinal obstruction syndrome (DIOS), which is an important cause of obstructive symptoms in people with CF that must be differentiated from other causes of bowel obstruction. However, one confounding factor is that adults with CF may have elements of both DIOS and mechanical bowel obstruction due to adhesions from previous operations. A recent tragic outcome in a young adult with CF highlights the need for all doctors, both junior and senior, especially those who are not directly involved in day-to-day CF care, to understand the features of DIOS and the appropriate management, which differs from that of a mechanical bowel obstruction. This review aims to highlight the clinical and pathophysiological features of DIOS, differentiate it from other causes of bowel obstruction and contrast management strategies. Improved knowledge of DIOS will help to facilitate appropriate recognition and permit optimal, multidisciplinary management of this CF complication., (© 2019 Royal Australasian College of Surgeons.)