UK Adult Cystic Fibrosis Centre Research Articles

P324 Changes to the dietetic landscape in a UK adult cystic fibrosis centre following the introduction of triple CFTR modulator therapy and continuous glucose monitoring to screen for CF diabetes

P324 Changes to the dietetic landscape in a UK adult cystic fibrosis centre following the introduction of triple CFTR modulator therapy and continuous glucose monitoring to screen for CF diabetes

EPS9.05 Vantobra®, a new formulation of nebulised tobramycin: early real-world experiences at our large regional UK adult cystic fibrosis centre

EPS9.05 Vantobra®, a new formulation of nebulised tobramycin: early real-world experiences at our large regional UK adult cystic fibrosis centre

P152 Trends in intravenous antibiotic prescriptions pre- and postintroduction of Kaftrio® in a large UK adult cystic fibrosis centre

P152 Trends in intravenous antibiotic prescriptions pre- and postintroduction of Kaftrio® in a large UK adult cystic fibrosis centre

P229 The introduction of an adherence support clinic in a large UK adult cystic fibrosis centre

P229 The introduction of an adherence support clinic in a large UK adult cystic fibrosis centre

Non-invasive ventilation is associated with long-term improvements in lung function and gas exchange in cystic fibrosis adults with hypercapnic respiratory failure

BackgroundNon-invasive ventilation (NIV) is an established treatment option for cystic fibrosis (CF) patients with type 2 respiratory failure but the benefits of this therapy remain unclear. This study examined the long-term outcomes and response to NIV in a large adult CF cohort. MethodsAll patients attending a UK adult CF Centre receiving NIV as treatment for hypercapnic respiratory failure over a nine-year period were studied prospectively. Detailed clinical data was recorded and longitudinal data measurements were examined for the three years pre and post NIV initiation to assess effect of this intervention. Results94 patients, mean age 29.9 (SD 9.7) years, percent predicted FEV1 21.5 (7.3), received NIV. All patients commenced NIV in a hospital setting. 21 remain alive, 24 received double lung transplant, 49 died without lung transplantation. NIV use was associated with a stabilisation and improvement in both FEV1 and FVC from NIV set up to three years post follow-up, in addition to an increase in body mass index and attenuation of PCO2 (all p<0.001). No single parameter was found to predict long-term NIV response but baseline PCO2 (p=0.005), CRP (p=0.004) and age (p=0.009) were identified as independent predictors of mortality. ConclusionsNIV use in CF adults is associated with improvements in lung function and attenuation of hypercapnia which is maintained for up to three years post NIV initiation. Outcomes for CF patients with severe pulmonary disease commenced on NIV have significantly improved with fifty percent of patients expected to survive for approximately five years.

P220 Experiences of virtual cystic fibrosis clinics during the COVID-19 pandemic at one UK adult cystic fibrosis centre

Background: In response to the COVID-19 pandemic, the Oxford Adult CF Centre offered virtual clinics using the NHS Attend Anywhere platform from June 2020. We surveyed patient and staff experience. Methods: An anonymous online patient survey was added to appear at the end of all CF virtual consultations from July to November 2020. A separate anonymous staff survey was distributed by email to all members of the CF multidisciplinary team. Results: 136 responses were received (patient population of 141) and 12/19 (62%) of staff responded. 71% of patients rated a virtual review a very reasonable alternative to face-to-face and 83% felt all their clinical needs were met. 98% rated how comfortable they were to talk to clinicians as 4 or 5/5. 83% staff felt they could provide an acceptable standard of review to most patients. When asked if they could provide a high standard of review, 17% felt they could to most patients, with 58% feeling they could to some patients only. Time efficiency and patient attendance at virtual clinics were perceived as superior to face-to face. 86% of patients rated their satisfaction with the technology as 4 or 5/5, with 100% staff rating as 4/5. 11 negative patient comments related to technology. Poor sound quality and video lag were the main concerns identified by both patients and staff. 82% of patients would like to continue using virtual reviews in future and 100% staff wanted this to be an option for some appointments. Routine clinic visits were rated most popular appointments to be virtual in future by staff and patients. Conclusion: Virtual clinics have been well received by the majority of our adult CF patients during the COVID-19 pandemic. Patients and staff wanted to use virtual reviews in future, although both recognised this is not always suitable for all appointments.

P192 The introduction of a quality improvement focus group in a large UK adult cystic fibrosis centre

P192 The introduction of a quality improvement focus group in a large UK adult cystic fibrosis centre

EPS1.06 A retrospective audit of home-based spirometry quality in a large UK adult cystic fibrosis centre

Objectives: Spirometry measurement is the gold standard for assessing disease severity in cystic fibrosis (CF). Poor-quality spirometry tests can result in inaccurate measurement of FEV1 and FVC. Manchester Adult CF Centre has transformed from hospital to home-based spirometry testing during COVID-19. Unlike hospital-based spirometry, home-based spirometry relies entirely on the subject to obtain good quality spirometry. Therefore, we sought to ascertain the quality of home-based spirometry and the test errors in our patients. Methods: A convenience sample of adults with CF attending Manchester Adult CF Centre were provided with a NuvoAir home-based spirometer to perform routine lung function between March–October 2020. NuvoAir respiratory platform consists of a mobile phone application, Bluetooth spirometer and an online results portal. The spirometer also provides feedback to patients’ spirometry quality. Initial patient set-up was performed in-hospital or virtually with a member of the CF clinical team. All patient sessions were included irrespective of quality of spirometry test session. Acceptability and repeatability criteria were applied as per NuvoAir software in line with ATS/ERS guidelines, along with assigning a quality grading A–Faccording to ATS/ERS standardised pulmonary function report criteria at time of testing. Results: 66 CF patients (32 female) mean age 31.3 (18–55) performed 343 spirometry sessions totaling 1,041 individual spirometry tests with a NuvoAir device were graded as follows: Grade A = 30.3%, Grade B = 36.2%, Grade C = 3.5%, Grade D = 2.6%, Grade E = 16.6%, Grade F = 10.8%. Further analysis of all 1,041 tests for common errors indicated BX – Back extrapolation 2%, TP- Time to Peak (slow start) 14.6%, CO – Cough 1.4%, ET – Early termination 0.5%, CE – Cessation or glottic closure 12.9%. Overall, general tests A–C considered usable was 70%. Conclusion: The results show good-quality standards can be achieved through home-based spirometry.

P317 Patient experience and satisfaction with 0.9% saline nasal irrigation (SNI) in a large UK adult cystic fibrosis centre and potential barriers to use

P317 Patient experience and satisfaction with 0.9% saline nasal irrigation (SNI) in a large UK adult cystic fibrosis centre and potential barriers to use

WS10.2 Normal weight obesity in cystic fibrosis is associated with glucose tolerance irregularities in cystic fibrosis at a UK adult cystic fibrosis centre

WS10.2 Normal weight obesity in cystic fibrosis is associated with glucose tolerance irregularities in cystic fibrosis at a UK adult cystic fibrosis centre

P245 Screening and treatment of Cystic Fibrosis-Related Diabetes at a UK adult cystic fibrosis centre

P245 Screening and treatment of Cystic Fibrosis-Related Diabetes at a UK adult cystic fibrosis centre

P312 Patient reported use, effects and tolerance of 0.9% saline nasal irrigation (SNI) in a large UK adult cystic fibrosis centre

P312 Patient reported use, effects and tolerance of 0.9% saline nasal irrigation (SNI) in a large UK adult cystic fibrosis centre

P150 Achromobacter prevalence, species distribution and infection status results from a large UK adult cystic fibrosis centre

P150 Achromobacter prevalence, species distribution and infection status results from a large UK adult cystic fibrosis centre

P250 Pattern of use of NIV: 10-year experience of a large UK adult cystic fibrosis centre

P250 Pattern of use of NIV: 10-year experience of a large UK adult cystic fibrosis centre

WS11-4 Current clinical practice in the management of cystic fibrosis-related bone disease in a regional UK adult cystic fibrosis centre

WS11-4 Current clinical practice in the management of cystic fibrosis-related bone disease in a regional UK adult cystic fibrosis centre

248 Exploring the relationship between exercise capacity and pulmonary exacerbations in a UK Adult Cystic Fibrosis Centre

248 Exploring the relationship between exercise capacity and pulmonary exacerbations in a UK Adult Cystic Fibrosis Centre

Travelling abroad with cystic fibrosis: Assessment of risks and healthcare requirements

There is limited information on the risks and healthcare requirements of patients with cystic fibrosis (CF) undertaking travel abroad. Of 100 patients (mean age 24.7 years, mean FEV1 57.3 %predicted) attending a UK adult CF Centre, 96% had travelled abroad but 14% now limited travel on medical advice. They travelled frequently and widely, often undertaking adventurous activities on holidays, but because of the costs involved, 18% travelled without travel insurance and 23% with insurance which did not cover CF. Of those who had ever had an illness abroad 10% had a CF-related illness (7 chest infection, 2 dehydration, 1 pancreatitis) and 12% a non-CF-related illness (4 sunburn, 3 gastroenteritis, 3 ear infection, 1 fall, 1 gastro-oesophageal reflux). There is a wide range of disease severity and assessment of the medical risks and the travel insurance premium to be charged should be based on the individual's health status rather than generically on the basis of a diagnosis of CF.

Pregnancy outcomes in cystic fibrosis: a 10-year experience from a UK centre

Cystic fibrosis manifests as a multisystem disease, despite this female fertility is relatively preserved with levels approaching that of the non-cystic fibrosis population. We reviewed pregnancies in cystic fibrosis patients over a 10-year period from a UK adult cystic fibrosis centre by considering maternal and fetal outcomes. We conducted a retrospective case-note review of pregnancies during 2003-2013 using respiratory and obstetric records. We observed moderate falls in lung function immediately after delivery, which persisted at 12 months postpartum. We found that a decline in lung function at delivery was a marker for further decline in function during the subsequent postpartum period. We found baseline lung function was predictive of gestational age at delivery. We observed a high incidence of haemoptysis. Consistent with current guidance we found pregnancy is feasible and well tolerated in the majority of patients with cystic fibrosis. There was a high incidence of haemoptysis, which warrants further study.

P201 Pneumocystis Jirovecii Prevalence In A Large Uk Adult Cystic Fibrosis Centre

Introduction and objectives Pneumocystis jirovecii ( PJ ) is an atypical fungus that causes pneumonia in immunocompromised patients. Its role in patients with cystic fibrosis (CF) is unclear. Its reported prevalence in CF ranges from 1–22% but has never been determined in the UK. Here we present preliminary cross-sectional data from an ongoing study at a UK adult CF centre serving >400 patients, to establish prevalence and potential risk factors for infection. Methods Sputum samples were obtained from 100 randomly selected CF outpatients and sent for routine microbiology and PJ DNA PCR assay at enrolment, subsequent visits, and pulmonary exacerbations requiring intravenous antibiotics within 4 months. Data were recorded for demographics, co-morbidities symptom score, spirometry, inflammatory markers, and prophylactic or recent therapeutic antibiotic therapy. Univariate comparisons were made between sputum PJ positive and negative patients. Chi square tests were used for categorical comparisons and independent sample t-tests for continuous independent variables. Results Of the 100 patients, 4 of 100 had a positive sputum PJ PCR at baseline. 50 patients had a routine follow up sample between 1 and 4 months: 2 were positive for PJ. 22 patients had a sputum sample analysed at the onset of a pulmonary exacerbation of which 1 was positive for PJ. Hence, a total of 7 of 100 patients had a single positive sample by PCR for PJ. No patient has had >1 positive sample. None of the baseline parameters were significantly different between PJ positive and PJ negative patients at the level p Conclusion These results suggest that PJ is not an important infecting pathogen in this UK cohort of CF patients. This may be due to frequent use co-trimoxazole for pulmonary exacerbations and high prevalence of prophylactic macrolide antibiotic therapy at our centre compared to other published studies. These preliminary data were underpowered to accurately compare baseline characteristics between PJ positive and PJ negative patients.

P199 Molecular Analysis Demonstrates Shared Strains Of Mycobacterium Abscessus Isolates In Cystic Fibrosis Patients Attending A Single Centre

Introduction and objectives The Mycobacterium abscessus complex is an emerging group of pathogens in cystic fibrosis (CF), which may cause cross infection. The aim of this study was to determine whether CF patients infected with M.abscessus attending a single UK adult CF centre harboured unique or shared strains of M. abscessus. Methods Isolates were from 12 patients attending a single adult CF centre, who yielded one or more positive sputum cultures for M. abscessus complex during the period January 2010 to August 2013. Isolates were identified to subspecies level using hsp65-rpoB concatenated sequence cluster analysis. Variable Number Tandem Repeat (VNTR) analysis was used to compare these isolates and determine whether two or more patients were infected with the same strain. Results 11 isolates were identified as M. abscessus susbp. abscessus . VNTR analysis demonstrated 2 clusters of 6 and 2 patients carrying the same strains of M. abscessus susbp. abscessus , both of which have also been isolated from CF patients from other UK hospitals. Isolates from the remaining 3 patients were unique. One additional isolate was identified as M. abscessus subsp.bolletii. No clear epidemiological connexions between patients within each cluster at our centre have been identified to date. Conclusion These results provide further evidence that some strains of M. abscessus complex may be isolated from multiple CF patients. However, there were no clear epidemiological connexions between patients within clusters at our centre. The same strains have been isolated from patients at different UK CF centres. Further studies are required to determine the mode of acquisition of infection with these strains and whether there is a common environmental source of infection or cross infection between patients.