It takes a village to raise a child- A multidisciplinary approach to promoting paediatric health literacy in cystic fibrosis.

Background: In Northern Ireland, approximately 550 people with cystic fibrosis (PwCF) attend the regional paediatric and adult centres within the Belfast Health and Social Care Trust. This autosomal recessive chronic condition necessitates regular clinical monitoring and a high treatment burden, as well as time implications for the maintenance of respiratory devices. Development of health literacy skills at an early age and promoting children with CF (CwCF) to take an active role in their healthcare has many advantages relating to their long-term self-care in preparation for transition from paediatric to adult care, decision-making and partnership engagement with the CF-multidisciplinary team (CF-MDT).

totally implantable venous access device infection in a person with cystic fibrosis: Complex management considerations.

Totally implantable venous access devices (TIVADs) are frequently used in people with cystic fibrosis as a means of securing consistent vascular access, particularly in the context of severe disease and microbial colonization. Infection of TIVADs is not uncommon and typically associated with coagulase negative staphylococci, though infection with other organisms does occur too. We report on the first case of a TIVAD infection caused by in person with cystic fibrosis.

Addressing pain in people living with cystic fibrosis: Cystic fibrosis foundation evidence-informed guidelines.

Even as many outcomes for people living with cystic fibrosis (PLwCF) improve, individuals still experience extensive symptom burdens. From birth, many PLwCF experience both pain as a symptom of their CF disease and procedural pain, posing detriments to health, functioning, and quality of life. Despite its prevalence and impact, there is no CF-specific guidance for the assessment and management of pain.

Cardiovascular Benefits and Safety Profile of Macrolide Maintenance Therapy in Patients with Bronchiectasis.

Background: Macrolide maintenance therapy (MMT) has demonstrated notable efficacy in reducing exacerbation in patients with bronchiectasis, which is a major risk factor for cardiovascular events. However, a comprehensive assessment of the cardiovascular benefits and safety profile of MMT in this population is lacking.

Methods: This territory-wide cohort study analyzed patients diagnosed with bronchiectasis in Hong Kong between 2001 and 2018.

"Shifting sands in cystic fibrosis": impacts of CFTR modulators on reproductive health in people with cystic fibrosis and challenges related to exposure.

Introduction: Mutation-specific disease modifying drugs such as the triple combination Elexacaftor/Tezacaftor/Ivacaftor (ETI), are associated with significant improvements in physical health. Reproductive health and a pursuit of parenthood are of increased relevance; a dramatic increase in childbirth rates for females with CF has already been observed.

Areas Covered: Fertility in males and females with CF, and any subsequent impact of CFTR modulator therapy, is reviewed.

Phenotypic Evaluation of Rare Cystic Fibrosis Transmembrane Conductance Regulator Mutation Combinations in People with Cystic Fibrosis in Queensland, Australia.

Cystic fibrosis (CF) is a multisystem disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. We describe the distribution of CFTR mutation profiles in sub-tropical Queensland, Australia, and characterise the phenotypes associated with 'rare' CFTR mutation combinations. We conducted a retrospective observational study to analyse the CFTR mutation profiles of 322 people with CF (pwCF) under the care of a large adult CF centre in Queensland, Australia.

Lack of correlation between surface water area and infection with and the non-tuberculous mycobacteria (NTMs) in patients with cystic fibrosis (CF).

Background: People with cystic fibrosis (CF) may develop clinically significant chronic respiratory infections with (PA) and non-tuberculous mycobacteria (NTM). Open water has been suggested to be an important source for continuous or intermittent exposure to these pathogens. To date, there has been a paucity of studies examining the relationship between chronic PA and NTM infection in CF patients and surfaces waters, including blue spaces.

Regulatory adverse drug reaction analyses support a temporal increase in psychiatric reactions after initiation of cystic fibrosis combination modulator therapies.

Introduction: Despite improved outcomes for many people with cystic fibrosis, there have been reports of adverse neuropsychiatric effects of modulator therapy. The aim of this research is to define temporal associations in adverse drug reaction (ADR) reports for available CFTR modulators.

Methods: Methods include an analysis of the UK Yellow Card Scheme data for ADRs through accessing interactive Drug Analysis Profiles (iDAPs) to define temporal trends in absolute and proportional counts.

Exploring the complexity of cystic fibrosis (CF) and psychosocial wellbeing in the 2020s: Current and future challenges.

Cystic fibrosis (CF) is traditionally associated with considerable and progressive multisystem pathology, onerous treatment burden, complex psychosocial challenges, and reduced life-expectancy [1-9].This decade has seen transformative change in management for many, but not all, people with CF. The most notable change comes from Cystic Fibrosis Transmembrane Receptor (CFTR) modulators, which bring significant benefits for people who are eligible for, and able to access, them [10].

Evaluating the correspondence between the EQ-5D-5L and disease severity and quality of life in adults and adolescents with cystic fibrosis.

Background: The EQ-5D is the recommended measure to capture health-related quality of life (HRQoL), recognised for use in health technology appraisal bodies. In order to assess whether it is appropriate to use the EQ-5D for making decisions about the cost-utility of treatments in cystic fibrosis (CF), this study assesses the performance of the EQ-5D-5L in adults and adolescents with CF.

Method: This was a cross-sectional observational survey study of patients with CF attending a single large CF centre.

Cloaking antibodies are prevalent in complex infection and their removal restores serum killing.

Introduction: The complex encompasses a group of gram-negative opportunistic pathogens that cause chronic lung infections in people with cystic fibrosis. Distinct from other respiratory pathogens, causes a unique clinical disease in a subset of patients known as 'cepacia syndrome', fulminant pneumonia accompanied by bacteraemia and sepsis with a mortality rate of up to 75%. Due to the bacteraemia associated with this disease, the mechanisms that allow to resist the bactericidal effects of serum complement-depending killing are vital.

Role for DPP4 inhibitor therapy in cystic fibrosis related diabetes: A single centre experience.

Introduction: Insulin remains the only recommended medical treatment for cystic fibrosis related diabetes (CFRD) Whilst there is an established role for orally bioavailable incretin mimetic agents such as the dipeptidyl peptidase-4 inhibitors (DPP4-I) in Type 2 diabetes mellitus, there exists little data on their utility in CFRD.

Aim: To examine the use of DPP4-I therapy in patients with CFRD at a single large adult cystic fibrosis center.

Method: People with CFRD prescribed a DPP4-I were identified from our specialist CFRD clinic and records were retrospectively examined for indication for therapy, tolerability and effectiveness.

Case Report: The Conundrum of What to Pick? Antibiotic Susceptibility Variability in in Cystic Fibrosis: Implications for Antibiotic Susceptibility Testing and Treatment.

Within cystic fibrosis microbiology, there is often mismatch between the antibiotic susceptibility result of an isolated bacterial pathogen and the clinical outcome, when the patient is treated with the same antibiotic. The reasoning for this remains largely elusive. Antibiotic susceptibility to four antibiotics (ceftazidime, meropenem, minocycline and trimethoprim-sulfamethoxazole) was determined in consecutive isolates ( = 11) from an adult cystic fibrosis patient, over a 63 month period.

Carriage of Neisseria meningitidis (meningococci) in the sputum of people with cystic fibrosis (CF): occupational health risks and mitigating interventions for physiotherapists.

Objectives: To estimate the carriage of Neisseira meningitidis (meningococci) in expectorated sputum from people with cystic fibrosis (CF) and to evaluate potential ramifications of such carriage for the health and (NM) wellbeing of physiotherapists performing airway clearance techniques.

Design: Descriptive observational study.

Main Outcome Measures: Meningococcal carriage rate, CFTR mutation type and time to first meningococcal culture were determined.

Keeping up with the pathogens: improved antimicrobial resistance detection and prediction from Pseudomonas aeruginosa genomes.

Background: Antimicrobial resistance (AMR) is an intensifying threat that requires urgent mitigation to avoid a post-antibiotic era. Pseudomonas aeruginosa represents one of the greatest AMR concerns due to increasing multi- and pan-drug resistance rates. Shotgun sequencing is gaining traction for in silico AMR profiling due to its unambiguity and transferability; however, accurate and comprehensive AMR prediction from P.