Comparison of quadriceps muscle size and quality in adults with cystic fibrosis with different severities of cystic fibrosis transmembrane conductance regulator protein dysfunction.

Background: Cystic fibrosis (CF) is characterized by CF transmembrane conductance regulator (CFTR) dysfunction. CFTR protein is expressed in human skeletal muscle; however, its impact on skeletal muscle is unknown. The objectives of this study were to compare quadriceps muscle size and quality between adults with various severities of CFTR protein dysfunction.

Impact of COVID-19 on Lung Disease in People with Cystic Fibrosis: A 6-Month Follow-Up Study on Respiratory Outcomes.

Background: The impact of COVID-19 on respiratory outcomes in people with cystic fibrosis (pwCF) has not been clearly characterized. We evaluated changes in respiratory function indicators derived from spirometry and pulmonary exacerbation rates 6 months after SARS-CoV-2 infection.

Methods: This multicentre prospective study was based on pwCF enrolled between October, 2020 and June, 2021 in the DECO COVID-19 project.

Transition from paediatric to adult care in cystic fibrosis.

Unlabelled: In the decades since cystic fibrosis (CF) was first clinically defined in the 1930s, there have been many advancements in the treatment and management of this disease. Initially it was considered a disease of childhood where the majority of those affected died before reaching adolescence. Now, through advancements in management and treatment, the vast majority of those affected will live into adulthood.

Rapid fluoroquinolone resistance detection in using mismatch amplification mutation assay-based real-time PCR.

Antimicrobial resistance (AMR) is an ever-increasing global health concern. One crucial facet in tackling the AMR epidemic is earlier and more accurate AMR diagnosis, particularly in the dangerous and highly multi-drug-resistant ESKAPE pathogen, . We aimed to develop two SYBR Green-based mismatch amplification mutation assays (SYBR-MAMAs) targeting GyrA T83I (248) and GyrA D87N, D87Y and D87H (259).

ECFS standards of care on CFTR-related disorders: Updated diagnostic criteria.

This paper is the first in a series providing updated guidance on the definition, evaluation and management of people with a Cystic Fibrosis Transmembrane conductance Regulator (CFTR)-Related Disorder (CFTR-RD). The need for this update relates to more precise characterisation of CFTR gene variants and improved assessment of CFTR protein dysfunction. The exercise is co-ordinated by the European CF Society Standards of Care Committee and Diagnostic Network Working Group and involves stakeholder engagement.

Portable HEPA filtration successfully augments natural-ventilation-mediated airborne particle clearance in a legacy design hospital ward.

As the severe acute respiratory syndrome coronavirus-2 pandemic has proceeded, ventilation has been recognized increasingly as an important tool in infection control. Many hospitals in Ireland and the UK do not have mechanical ventilation and depend on natural ventilation. The effectiveness of natural ventilation varies with atmospheric conditions and building design.

Role of the Physical Therapist in Cystic Fibrosis Care.

In looking back on 2020 and 2021, this Perspective reflects on the monumental impacts of the rollout of cystic fibrosis (CF) transmembrane conductance regulator highly effective modulator therapies and the COVID-19 pandemic on the management of CF. Advancements in the clinical management of people with CF have been both enormous and rapid, and physical therapists specializing in the care of people with CF have been at the forefront of driving this evolution in care. This year sees the 30th anniversary of the UK Association of Chartered Physiotherapists in Cystic Fibrosis and, as is inevitable in reaching such milestones, thoughts have turned to origins, role, impacts, and the future.

A pilot study investigating the effects of a manuka honey sinus rinse compared to a standard sinus rinse on sino-nasal outcome test scores in cystic fibrosis patients.

Background: People with cystic fibrosis (CF) are prone to bacterial respiratory infections; these are often antibiotic resistant, are difficult to treat, and impact on the quality of life and lung function. The upper respiratory tract can act as a reservoir for these pathogens, and as part of clinical care, sinus rinses are used to alleviate symptoms in the upper airway. We have developed a sinus rinse containing manuka honey, to identify whether it can help improve symptoms or reduce the bacterial load.

Kidney and combined kidney and pancreas transplantation may be under-utilized in cystic fibrosis.

Cystic fibrosis (CF) is a multisystem disorder and represents the most common inherited condition leading to death in Western countries. Previous reports of chronic kidney disease (CKD) in CF focus on cases post lung, or other solid organ, transplantation but CKD in CF patients pre transplantation is increasingly recognized as a challenging complication of CF. CKD can evolve as a sequel to acute kidney injury for example after prolonged treatment with aminoglycoside antibiotics during episodes of infection.

Non-respiratory health-related quality of life in people with cystic fibrosis receiving elexacaftor/tezacaftor/ivacaftor.

Elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) was shown to be safe and efficacious in people with cystic fibrosis (CF) heterozygous for F508del and a minimal function mutation (F/MF) or homozygous for F508del (F/F) in two pivotal Phase 3 trials, significantly improving percentage predicted forced expiratory volume in 1 second, Cystic Fibrosis Questionnaire-Revised, Respiratory Domain (CFQ-R RD) scores, and sweat chloride concentration. Here, we analyzed the 11 non-respiratory domains (non-RDs) of the CFQ-R, which assess general health-related quality of life (i.e.

The Exeter Activity Unlimited statement on physical activity and exercise for cystic fibrosis: methodology and results of an international, multidisciplinary, evidence-driven expert consensus.

Background: The roles of physical activity (PA) and exercise within the management of cystic fibrosis (CF) are recognised by their inclusion in numerous standards of care and treatment guidelines. However, information is brief, and both PA and exercise as multi-faceted behaviours require extensive stakeholder input when developing and promoting such guidelines.

Method: On 30 June and 1 July 2021, 39 stakeholders from 11 countries, including researchers, healthcare professionals and patients participated in a virtual conference to agree an evidence-based and informed expert consensus about PA and exercise for people with CF.

Antifungal therapies for allergic bronchopulmonary aspergillosis in people with cystic fibrosis.

Background: Allergic bronchopulmonary aspergillosis (ABPA) is an allergic reaction to colonisation of the lungs with the fungus Aspergillus fumigatus, and affects around 10% of people with cystic fibrosis. ABPA is associated with an accelerated decline in lung function. High doses of corticosteroids are the main treatment for ABPA; although the long-term benefits are not clear, and their many side effects are well-documented.

Self-reported dental attendance, oral hygiene habits, and dietary habits of adults with cystic fibrosis.

Aims: To evaluate the dental attendance, oral hygiene habits, and dietary habits of adults with Cystic Fibrosis in the Republic of Ireland.

Methods And Results: A cross-sectional study was carried out using a structured anonymous questionnaire. A total of 71 adults with Cystic Fibrosis responded.

Lung transplantation for interstitial lung disease in idiopathic inflammatory myositis: A cohort study.

In patients with interstitial lung disease (ILD) complicating classical or amyopathic idiopathic inflammatory myopathy (IIM), lung transplantation outcomes might be affected by the disease and treatments. Here, our objective was to assess survival and prognostic factors in lung transplant recipients with IIM-ILD. We retrospectively reviewed data for 64 patients who underwent lung transplantation between 2009 and 2021 at 19 European centers.

Bacterial Culture Underestimates Lung Pathogen Detection and Infection Status in Cystic Fibrosis.

Microbiological surveillance of airway secretions is central to clinical care in cystic fibrosis (CF). However, the efficacy of microbiological culture, the diagnostic gold standard for pathogen detection, has been increasingly questioned. Here we compared culture with targeted quantitative PCR (QPCR) for longitudinal detection of 2 key pathogens, Pseudomonas aeruginosa and Staphylococcus aureus.

Frailty predicts outcomes in cystic fibrosis patients listed for lung transplantation.

Background: Survival predictors are not established for cystic fibrosis (CF) patients listed for lung transplantation (LT). Using the deficit accumulation approach, we developed a CF-specific frailty index (FI) to allow risk stratification for adverse waitlist and post-LT outcomes.

Methods: We studied adult CF patients listed for LT in the Toronto LT Program (development cohort 2005-2015) and the Swiss LT centres (validation cohort 2008-2017).

The Microbiology of Non- Isolated From Adults With Cystic Fibrosis: Criteria to Help Determine the Clinical Significance of Non- in CF Lung Pathology.

There is a paucity of reports on non- (NAPs) in cystic fibrosis, hence this study wished 1). to examine the diversity/frequency of NAPs in an adult CF population, 2) to compare/contrast the microbiology and genomics of NAPs to and 3) to propose clinical and laboratory criteria to help determine their clinical significance in CF lung pathology. Microbiological data was examined from 100 adult patients with cystic fibrosis from birth to present (31/12/2021), equating to 2455 patient years.

Modelling Successful Self-Management in Adults With Cystic Fibrosis: Vicarious Self-Efficacy From Videos of 'People Like Me'.

Background Self-efficacy is an important determinant of treatment adherence, and peer modelling of success can provide vicarious self-efficacy. A series of patient stories ('talking heads' videos) were developed with people with cystic fibrosis (CF) as part of the CFHealthHub multi-component adherence intervention, aiming to demonstrate success with daily therapy in 'people like me'. Methodology One-to-one semi-structured interviews exploring patients' experiences, barriers and facilitators of nebuliser adherence were audio and video-recorded between October 2015 and August 2016.

Nebulizer Cleaning Practices and Adherence to Nebulized Therapies in People With Cystic Fibrosis When Traveling.

Background: Nebulized therapies form an important component of treatment in people with cystic fibrosis (CF). It is important for people with CF to continue to take their nebulized medications when traveling.

Methods: A self-completing anonymous questionnaire was developed, as part of a quality improvement project, to support people with CF educational needs when traveling.

Nurses' experience of an Extracorporeal Membrane Oxygentation (ECMO) clinical support team during the COVID-19 pandemic: A service evaluation.

Background: The COVID-19 pandemic placed unprecedented stress on the National Health Service and critical care units including those with Extracorporeal Membrane Oxygenation (ECMO) facility as this intervention had proved successful with H1N1 patients in 2009. To successfully care for the influx of ECMO patients, an ECMO clinical support team (ECST) formed by redeployed staff was created to assist critical care nurses.

Aim: This service evaluation aims to review the experience of critical care nursing staff working with an ECST during the period of increased provision of ECMO care.

Treatment Preference Among People With Cystic Fibrosis: The Importance of Reducing Treatment Burden.

Background: There is a growing consensus that the perspective of the patient should be considered in the evaluation of novel interventions.

Research Question: What treatment outcomes matter to people with cystic fibrosis (CF), and what trade-offs would they make to realize these outcomes?

Study Design And Methods: Adults attending a specialist CF center were invited to complete an online discrete choice experiment (DCE). The DCE required participants to evaluate hypothetical CF treatment profiles, defined by impact on lung function, pulmonary exacerbations, abdominal symptoms, life expectancy, quality of life, inhaled medicine usage, and physiotherapy requirement.

A case of Elexacaftor-Tezacaftor-Ivacaftor induced rash resolving without interruption of treatment.

The triple combination of Elexacaftor-Tezacaftor-Ivacaftor (ELX-TEZ-IVA) has been shown to markedly improve lung function in persons with cystic fibrosis (pwCF). An important adverse effect of the drug is rash, which was reported in clinical trials and highlighted in case reports. Our report demonstrates a similar adverse event with the drug in one of our patients with spontaneous resolution of the rash not necessitating cessation of treatment or desensitization to the drug as were done in other cases.