Regional Adult Cystic Fibrosis Centre Research Articles

P063 Dermatological reactions to elexacaftor/tezacaftor/ivacaftor: experience within a regional adult cystic fibrosis centre

Introduction: Dermatological reactions are relatively common in people with CF (PwCF) treated with elexacaftor/tezacaftor/ivacaftor (ETI). Clinical trial data suggests rates as high as 10.9% following initiation of therapy ( 1 Middleton PG et al. Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele. N Engl J Med. 2019; 381: 1809-1819 Crossref PubMed Scopus (868) Google Scholar ). In the UK, 290 skin and subcutaneous spontaneous suspected adverse drug reactions have been reported through the Yellow Card Scheme (https://info.mhra.gov.uk/).

EPS4.03 An audit of diagnosing cystic fibrosis-related diabetes using newly developed diagnostic criteria using flash glucose monitoring system (Freestyle Libre®) at a regional adult cystic fibrosis centre

Objectives: In 2022, the West Midlands Adult Cystic Fibrosis Centre began using Freestyle Libre flash glucose monitoring system (FGM) as a first line method for cystic fibrosis related diabetes (CFRD) screening, as an alternative to oral glucose tolerance testing (OGTT). There are no published criteria for the diagnosis of CFRD using FGM. Using the WHO criteria, the Brompton’s paediatric clinical guideline and clinical judgement we devised diagnostic criteria to standardise the diagnosis of CFRD using FGM. Diagnostic criteria - CFRD >2 readings >11.1, impaired glucose tolerance (IGT) >2 readings 7.8–11 or <3.5; over a 2 week period. The aim of this study was to compare the number of patients diagnosed with CFRD and IGT using our new FGM diagnostic criteria compared to using our previous non standardised practice of using clinical judgement. We aimed to assess if there was any difference in the number of patients being diagnosed with CFRD or IGT using our new criteria.

P161 Body mass index change in adult patients with cystic fibrosis following the introduction of triple CFTR therapy elexacaftor/tezacaftor/ivacaftor: a regional adult cystic fibrosis centre experience

P161 Body mass index change in adult patients with cystic fibrosis following the introduction of triple CFTR therapy elexacaftor/tezacaftor/ivacaftor: a regional adult cystic fibrosis centre experience

Isolation of Exophiala dermatitidis is not associated with worse clinical outcomes during acute pulmonary exacerbations in cystic fibrosis.

Introduction. The black yeast Exophiala dermatitidis has been isolated in respiratory samples from people with cystic fibrosis (CF). However, adequate detection may require longer incubation periods than the current UK national standard for CF respiratory samples. Furthermore, it is unclear whether isolation of E. dermatitidis is associated with poorer clinical outcomes in CF.Hypothesis/gap statement. E. dermatitidis does not cause clinically significant lung disease in CF adults.Aim. To evaluate differences in clinical outcomes over a 12 month period and during acute pulmonary exacerbations between CF adults with and without untreated E. dermatitidis.Methodology. Incubation times for respiratory samples on Sabouraud dextrose agar with chloramphenicol (SABC) plates at a large regional adult CF centre were extended from 2 to 7 days over a 1 month period. The number of patients from whom E. dermatitidis was isolated, and the length of incubation time prior to isolation, were recorded. Outcomes of treatment of exacerbations with intravenous antibiotics but in the absence of concomitant antifungal therapy were compared between those with and without E. dermatitidis, as were changes in lung function and body mass index (BMI) over a 12 month period.Results. Extended incubation unmasked the presence of E. dermatitidis in 22 of 132 patients; all isolations occurred after >48 h of incubation. Patients who isolated E. dermatitidis had lower rates of Pseudomonas aeruginosa isolation (P=0.02) and higher rates of non-tuberculous mycobacteria isolation (P=0.03), and were more likely to be prescribed a long-term antifungal medication (P=0.03), but had no differences in age, sex, baseline lung function or body mass index (BMI). There were no differences in response to treatment of acute exacerbations between patients with and without E. dermatitidis, or in change in forced expiratory volume in 1 s (FEV1), BMI and number of exacerbations over 12 months of follow-up.Conclusion. E. dermatitidis is not associated with worse clinical outcomes in CF. Given potential side effects and drug interactions, routine targeting of E. dermatitidis with antifungals during acute exacerbations is not advised.

P453 An eight-year retrospective audit of clinical psychology referrals within the Northern Ireland Regional Adult Cystic Fibrosis Centre

P453 An eight-year retrospective audit of clinical psychology referrals within the Northern Ireland Regional Adult Cystic Fibrosis Centre

123 Prevalence of transmissible strains of Pseudomonas aeruginosa in a cystic fibrosis tertiary referral centre and lung transplant unit

120 Canine detection of Pseudomonas aeruginosa (PA) volatile organic compounds (VOC’s) N.V. Johnston1, J. Rao2, J.S. Elborn3,4, J.E. Moore5. 1Dogs Nose Ltd, Portadown, United Kingdom; 2University of Ulster, School of Biomedical Sciences, Coleraine, United Kingdom; 3Queen’s University Belfast, Centre for Infection and Immunity, Belfast, United Kingdom; 4Belfast City Hospital, Regional Adult Cystic Fibrosis Centre, Belfast, United Kingdom; 5Belfast City Hospital, Department of Bacteriology, Belfast, United Kingdom

Microbial contamination of non-invasive ventilation devices used by adults with cystic fibrosis

There is currently little evidence regarding potential risks of bacterial contamination of non-invasive ventilation (NIV) devices used by cystic fibrosis (CF) patients. The aim of this study was to determine the extent of bacterial contamination of NIV devices in our regional adult CF centre. Seven NIV devices recently used by CF patients chronically infected with Pseudomonasaeruginosa or Burkholderiacepacia complex (BCC) were swabbed in seven areas, both external and internal. Two devices had undergone ethylene oxide (EtO) sterilization between patient use and swabbing, and five devices had not undergone EtO sterilization. Swabs from five devices had insignificant growth of environmental organisms and two devices had significant growth of environmental organisms. No CF pathogens were isolated from any machine. No evidence was found of pathogenic microbial contamination of NIV devices used by CF patients in this small study. We suggest that further studies examine for evidence of bacterial contamination of NIV devices and that this issue should be included in future CF infection control guidelines.

241 When should lung function test be performed to evaluate the effects of an airway clearance therapy session in children and adults with cystic fibrosis?

240 An investigation into microbial contamination of non-invasive ventilation (NIV) devices used in adults with cystic fibrosis (CF) S. Cameron1, P. Agostini1, A. Mutagi2, G. Abbott3, E. Boxall2, E.F. Nash1, J. Whitehouse1, D. Honeybourne1. 1West Midlands Regional Adult Cystic Fibrosis Centre, Heart of England NHS Foundation Trust, Birmingham, United Kingdom; 2Health Protection Agency Laboratory, Birmingham, United Kingdom; 3Infection Prevention and Control Team, Heart of England NHS Foundation Trust, Birmingham, United Kingdom

240 An investigation into microbial contamination of non-invasive ventilation (NIV) devices used in adults with cystic fibrosis (CF)

240 An investigation into microbial contamination of non-invasive ventilation (NIV) devices used in adults with cystic fibrosis (CF) S. Cameron1, P. Agostini1, A. Mutagi2, G. Abbott3, E. Boxall2, E.F. Nash1, J. Whitehouse1, D. Honeybourne1. 1West Midlands Regional Adult Cystic Fibrosis Centre, Heart of England NHS Foundation Trust, Birmingham, United Kingdom; 2Health Protection Agency Laboratory, Birmingham, United Kingdom; 3Infection Prevention and Control Team, Heart of England NHS Foundation Trust, Birmingham, United Kingdom

The use of NIV within a regional adult Cystic Fibrosis Centre

The use of NIV within a regional adult Cystic Fibrosis Centre

Early detection of Pseudomonas aeruginosa – comparison of conventional versus molecular (PCR) detection directly from adult patients with cystic fibrosis (CF)

BackgroundPseudomonas aeruginosa (PA) is the most important bacterial pathogen in patients with cystic fibrosis (CF) patients. Currently, routine bacteriological culture on selective/non- selective culture media is the cornerstone of microbiological detection. The aim of this study was to compare isolation rates of PA by conventional culture and molecular (PCR) detection directly from sputum.MethodsAdult patients (n = 57) attending the regional adult CF centre in Northern Ireland, provided fresh sputum following airways clearance exercise. Following processing of the specimen with sputasol (1:1 vol), the specimen was examined for the presence of PA by plating onto a combination of culture media (Pseudomonas isolation agar, Blood agar & McConkey agar). In addition, from the same specimen, genomic bacterial DNA was extracted (1 ml) and was amplified employing two sequence-specific targets, namely (i) the outer membrane protein (oprL) gene locus and (ii) the exotoxin A (ETA) gene locus.ResultsBy sputum culture, there were 30 patients positive for PA, whereas by molecular techniques, there were 35 positive patients. In 39 patients (22 PA +ve & 17 PA -ve), there was complete agreement between molecular and conventional detection and with both PCR gene loci. The oprL locus was more sensitive than the ETA locus, as the former was positive in 10 more patients and there were no patients where the ETA was positive and the oprL target negative. Where a PCR +ve/culture -ve result was recorded (10 patients), we followed these patients and recorded that 5 of these patients converted to being culture-positive at times ranging from 4–17 months later, with a mean lag time of 4.5 months.ConclusionsThis study indicates that molecular detection of PA in sputum employing the oprL gene target, is a useful technique in the early detection of PA, gaining on average 4.5 months over conventional culture. It now remains to be established whether aggressive antibiotic intervention at this earlier stage, based on PCR detection, has any significant benefits on clinical outcome.

INCONTINENCE IN ADULT FEMALES WITH CYSTIC FIBROSIS: A NORTHERN IRELAND SURVEY

SUMMARYThe objectives of this survey were to establish the prevalence, onset, severity and impact of incontinence and attitudes towards the availability of advice and treatment in female adults with cystic fibrosis (CF) in Northern Ireland. All female patients (n=59) at the Northern Ireland Regional Adult CF Centre were posted a questionnaire on incontinence. Leakage of urine occurred in 14/46 respondents. Leakage of urine occurred when chest was bad in 8/14 patients and when chest was good or bad in 3/14 patients. Patients reported that their bladder problem affected their ability to perform airway clearance or cough (13/14), and exercise (4/14). The main reason given for the patients who did not seek help for their incontinence (10/14) was that they were too embarrassed. This study highlights that patients with CF are reluctant to seek treatment for incontinence despite the impact this condition can have.

Perceived benefits of inhaled therapies in cystic fibrosis

A questionnaire on inhalers was sent to all patients at the Northern Ireland Regional Adult Cystic Fibrosis Centre. The results showed widespread use of inhalers. Most patients perceived symptomatic benefit from inhalers, but 50% did not use them according to an agreed protocol. Education on the correct use of their inhalers is important to optimize patient benefit.

A cost description of an adult cystic fibrosis unit and cost analyses of different categories of patients.

There is little information on the costs of running an adult cystic fibrosis centre. The aim of this study was to provide detailed costs to assist funding and planning for these patients. The cost of a regional adult cystic fibrosis centre serving 119 cystic fibrosis patients, categorised according to four treatment regimens, was determined. District health authority, family health service authority, and voluntary resources used from April 1989 to March 1990 were determined, with appropriate bases for allocation of costs and patient based costs from local information. The total annual cost of treating the 119 patients was 980,646 pounds, with an average cost 8241 pounds per patient. An outpatient reviewed at three monthly intervals cost 2792 pounds a year; an outpatient receiving intravenous antibiotics cost 8606 pounds; an inpatient receiving intravenous antibiotics cost 13,501 pounds; and a patient needing a high level of care cost 19,955 pounds. Medication accounted for 57% (561,395 pounds) of the total cost. This analysis has helped us to secure funding for patients with cystic fibrosis and it facilitates the prediction of future requirements. The study also indicates the limitations of using average patient costs and difficulties as a result of the poorly structured British National Health Service accounting and information systems.