Manchester Adult Cystic Fibrosis Centre Research Articles

CSP2023: 294 - Development of a Pelvic Health Service for patients at the Manchester Adult Cystic Fibrosis Centre

CSP2023: 294 - Development of a Pelvic Health Service for patients at the Manchester Adult Cystic Fibrosis Centre

P333 A quality improvement project to pilot the Eating Attitudes and Behaviours tool in Manchester Adult Cystic Fibrosis Centre

P333 A quality improvement project to pilot the Eating Attitudes and Behaviours tool in Manchester Adult Cystic Fibrosis Centre

Improving detection of cystic fibrosis related liver disease using liver fibrosis assessment tools

Background & aimsCystic Fibrosis related liver disease (CFLD) is the 3rd largest cause of death in Cystic Fibrosis (CF). As advances in pulmonary therapies have increased life-expectancy, CFLD has become more prevalent. Current guidelines may underdiagnose liver fibrosis, particularly in its early stages. Newer modalities for the assessment of fibrosis may provide a more accurate assessment. FibroScan is validated in assessing fibrosis for several aetiologies including alcohol and fatty liver, the CFLD cohort have an entirely different phenotype so the cut off values are not transferrable. We appraised fibrosis assessment tools to improve diagnosis of CFLD. MethodsA prospective cohort (n = 114) of patients from the Manchester Adult Cystic Fibrosis Centre, UK were identified at annual assessment. Demographic data including co-morbidity, CFTR genotyping, biochemistry and imaging were used alongside current guidelines to group into CFLD and CF without evidence of liver disease. All patients underwent liver stiffness measurement (LSM) and assessment of serum-based fibrosis biomarker panels. A new diagnostic criterion was created and validated in a second, independent cohort. Results12 of 114 patient classified as CFLD according to the European Cystic Fibrosis Society best practice guidelines. No specific risk factors for development of CFLD were identified. Liver enzymes were elevated in patients with CFLD. Serum biomarker panels did not improve diagnostic criteria. LSM accurately predicted CFLD. A new diagnostic criterion was proposed and validated in a separate cohort, accurately predicating CFLD in 10 of 32 patients (31 %). ConclusionWe present a cohort of patients with CF assessed for the presence of liver fibrosis using blood biomarkers and LSM based platforms. We propose a new, simplified diagnostic criteria, capable of accurately predicting liver disease in patients with CF.Clinical trials number: NCT04277819

When is Burkholderia cepacia complex truly eradicated in adults with cystic fibrosis? A 20-year follow up study

BackgroundBurkholderia cepacia complex (BCC) infection in cystic fibrosis (CF) is associated with increased morbidity and mortality. Current UK guidance recommends segregation of people with CF according to infection status. To date there is no universally agreed consensus on the number of negative samples or time interval since last isolation of BCC for eradication to be deemed successful. MethodsAll cases of new BCC isolation at Manchester Adult Cystic Fibrosis Centre were followed-up between May 2002–May 2022. The number of subsequent positive and negative sputum samples for BCC were recorded, as well as eradication treatment received. Eradication was deemed successful if there were ≥3 negative sputum samples and no further positive sputum samples for the same species and strain ≥12 months until the end of follow-up. ResultsOf 46 new BCC isolation, 25 were successfully eradicated and 21 resulted in chronic infection. 5 (16.7%) cases with exclusively negative sputum samples 6–12 months after initial isolation had subsequent samples that were culture-positive for BCC and 3 (10.7%) cases with exclusively negative sputum samples after 12–24 months had subsequent culture-positive samples. Cases where BCC was eradicated had a greater median number of days of eradication treatment (42, IQR 21–63) compared to those in whom BCC isolation resulted in chronic infection (28, IQR 14–42), p = 0.04. ConclusionsA cautious approach to segregation should be maintained after new isolation of BCC in CF, as some individuals with ≥3 negative samples 12–24 months after initial isolation had subsequent sputum samples culture-positive for BCC.

P428 An evaluation of the current Manchester Adult Cystic Fibrosis Centre (MACFC) exercise service

To understand patient views regarding the exercise service at MACFC and identify areas for improvement.

Meteorological Factors Influence the Presence of Fungi in the Air; A 14-Month Surveillance Study at an Adult Cystic Fibrosis Center.

BackgroundCystic fibrosis is an inherited disease that predisposes to progressive lung damage. Cystic fibrosis patients are particularly prone to developing pulmonary infections. Fungal species are commonly isolated in lower airway samples from patients with cystic fibrosis. Fungal spores are prevalent in the air.MethodsWe performed environmental air sampling surveillance at the Manchester Adult Cystic Fibrosis Centre, UK (MACFC) over a 14-month period to assess fungal growth inside and outside the CF center.ResultsAirborne counts of fungal spores peaked from May to October, both in outdoor and indoor samples. Collection of meteorological data allowed us to correlate fungal presence in the air with elevated temperatures and low wind speeds. Additionally, we demonstrated patient rooms containing windows had elevated fungal counts compared to rooms not directly connected to the outdoors.ConclusionsThis study suggests that airborne Aspergillus fumigatus spores were more abundant during the summer months of the survey period, which appeared to be driven by increased temperatures and lower wind speeds. Indoor counts directly correlated to outdoor A. fumigatus levels and were elevated in patient rooms that were directly connected to the outdoor environment via an openable window designed for ventilation purposes. Further studies are required to determine the clinical implications of these findings for cystic fibrosis patients who are predisposed to Aspergillus related diseases, and in particular whether there is seasonal influence on incidence of Aspergillus related conditions and if screening for such complications such be increased during summer months and precautions intensified for those with a known history of Aspergillus related disease.

P194 Implementation of a cystic fibrosis-specific virtual pulmonary rehab program

Objectives: Pulmonary rehabilitation (PR) is a key management strategy for people with chronic respiratory disease (BTS Guidelines, 2013). However, in cystic fibrosis (CF), despite the benefits of regular exercise being well documented (Radtke et al, 2017), there is limited uptake. Concerns regarding infection control, lack of CF education, and participating with an older age group may be contributing factors. A pilot study demonstrated good clinical benefits to participants in a CF-specific PR program (Bowen et al, 2019) and current CF Foundation guidelines advise that patients with advanced lung disease should be offered such programs (Kapnadak et al, 2020). We aimed to establish a CF-specific PR program at Manchester Adult CF Centre, as part of the CF Trust Physiotherapy Fellowship service development project. Method: Patients completed a bespoke 8-week program, comprised of biweekly 1:1 virtual exercise sessions. Each session included aerobic and strength training linked to patient goals and was progressed according to reported Borg and muscle fatigue scores. A 1-minute sit to stand test (STS) was performed pre-program and repeated at the end. Enrolled patients had access to an online educational website, which covered a range of CF-specific topics. Patient feedback was collected at the end of the program. Results: [Table Preseteed] Patient feedback indicated that 100% of participants felt their goals had been met and that they would recommend the program to other people with CF (PWCF). Conclusion: Some of the program design was in accordance with what was achievable during the COVID-19 pandemic. Alteration of outcome measures may be required as clinical services normalise. Although a small sample, initial feedback has been extremely positive, indicating that a virtual PR approach may be suitable in engaging PWCF in physical activity. Further data is needed to demonstrate if the program is of clinical benefit and to identify the optimum time-frame of the intervention.

Editorial introductions

Editorial introductions

WS02.4 Developing an enhanced transition pathway for young people with additional needs at the Manchester Adult Cystic Fibrosis Centre (MACFC)

WS02.4 Developing an enhanced transition pathway for young people with additional needs at the Manchester Adult Cystic Fibrosis Centre (MACFC)

P411 An audit of patients with cystic fibrosis and additional needs, and of reasonable adjustments to care provision at the Manchester Adult Cystic Fibrosis Centre (MACFC)

P411 An audit of patients with cystic fibrosis and additional needs, and of reasonable adjustments to care provision at the Manchester Adult Cystic Fibrosis Centre (MACFC)

Comparison of Percutaneous Endoscopic Gastrostomy, Megestrol Acetate and Nasogastric feeding in adult patients with Cystic Fibrosis

Background/Aims: Malnutrition remains an important and common problem in cystic fibrosis (CF) patients. In adult CF patients, weight loss is associated with poor lung function, and nutritional status has been found to be an independent predictor of mortality. We compared changes in weight and forced expiratory volume in 1 s (FEV1) in patients with CF receiving one of three interventions to encourage weight gain: i) oral megestrol acetate (MA); ii) nasogastric (NG) tube feeding; iii) percutaneous endoscopic gastrostomy (PEG), Our aim was to determine and compare the effectiveness of these interventions in i) stabilising weight and ii) stabilising FEV1 in CF adults. Methods: We retrospectively collected data from hospital records of patients attending the Manchester Adult Cystic Fibrosis Centre (MACFC) between June 1998 and June 2012. We included adult patients with CF on any of the three nutritional interventions. Decisions regarding requirement for, and type of feeding intervention were made on a case by case basis by a multidisciplinary team with the choice of feeding intervention depending on MDT opinion and willingness of the patient for each intervention. Results: 53 patients fulfilled inclusion criteria with 12 month follow-up data (17 MA, 14 NG and 22 PEG). Patients showed significant weight gain from baseline for two of the interventions: MA (mean change 2.7 kg, 95% CI 0.5, 5.0) and PEG (mean 2.5 kg, 95% CI 0.7, 4.3). For NG mean weight gain was 2.0 kg (95% CI -0.2, 4.3) which did not reach statistical significance (p=0.073) Analysing change in weight between the interventions no statistically significant differences were identified. Lung function remained stable with small non-significant FEV1 changes over the 12 months: MA (mean change 0.09, 95% CI -0.08, 0.26), NG (mean 0.02, 95% CI -0.21, 0.26) and PEG (mean 0.04, 95% CI -0.12, 0.21, p=0.58). No statistically significant differences in FEV1 changes were found between the interventions. Conclusion: This is the first study to compare 3 different interventions in CF adults. All three interventions appear to be equally effective means of improving nutritional status in this 12 month study. Lung function remained stable but did not improve.

WS01.3 A ‘surprising’ pair: the synergistic antimicrobial activity of polymyxin B in combination with the cystic fibrosis drug ivacaftor against polymyxin-resistant P. aeruginosa

WS01.1 Discontinuation of routine anti-staphylococcal prophylaxis has no adverse effect on a paediatric CF patient population A. Shawcross, J. Holman, A. Maitra, A. Horsley, L.B. Patel, J. Panickar. Royal Manchester Childrens Hospital, Department of Paediatric Respiratory Medicine, Manchester, United Kingdom; University of Manchester, Institute of Inflammation and Repair, University Hospital of South Manchester, Wythenshawe, Manchester, United Kingdom; Manchester Adult Cystic Fibrosis Centre, University Hospital of South Manchester, Wythenshawe, Manchester, United Kingdom; SKMC Hospital, Department of Paediatric Respiratory Medicine, Abu Dhabi, United Arab Emirates

EPS06.9 Real world introduction of Colobreathe™ in children and adults with cystic fibrosis

Objectives: To evaluate the introduction of ColobreatheTM (Colo) from June to November 2015 in French patients attending 2 paediatric and 2 adult CF centres. Methods: All patients were similarly educated on how to inhale Colo (explanations, video) and received a test dose at the CF centre. Telephone follow-up with a CF caregiver was set up at days 1, 3 and 14 in order to assess tolerance and advise patients. Reasons for treatment interruption during the first month were collected. Results: 123 patients (adults 71%, median age 24 years, mean FEV1 % pred. 64±25) received a test dose of Colo. 67% were previously treated by inhaled colistine and 20% had no inhaled antibiotics. Out of the 112 patients who completed the trial, 27.7% interrupted treatment within one month. The reasons were cough (n =22, 71%), bad taste (n = 3), technical problems (n =4), treatment burden (n =2), urticaria (n = 1), medical decision (n =1) or hemoptysis (n = 1). No difference could be seen between those who had and had not interrupted treatment at one month as to age or FEV1, but patients already treated by inhaled colistine were less likely to stop treatment. In the 81 patients who completed one month treatment, cough was also frequently reported but its incidence progressively decreased (50% at D1, 36% at D3, and 14% at D30). 13 out of 112 patients (12%) had technical problems, mainly blocking of the device or broken capsule. At one month, treatment burden was scored at 2 (on a 1–10 scale). Conclusion: Education at introduction of Colo resulted in continuation of treatment in 72% of the patients at one month, cough being the major reason for discontinuation of treatment. Long term assessment is needed. ePS06.10 Antibiotic desensitisation: a single centre review of 2015 M.K. Dooney, K.J. Cox, P.J. Barry, A.K. Webb, A.M. Jones, R.J. Bright-Thomas. Manchester Adult Cystic Fibrosis Centre, Manchester, United Kingdom

Increased prevalence of Pneumocystis jirovecii colonisation in acute pulmonary exacerbations of cystic fibrosis

This study examined the prevalence of Pneumocystis jirovecii in the sputum of adults with cystic fibrosis during clinical stability and acute pulmonary exacerbation. This was a prospective, longitudinal observational study of patients attending the Manchester Adult Cystic Fibrosis Centre. Sputum samples were analysed for P. jirovecii DNA using PCR at enrolment and up to 5 follow-up visits. Patients were classified as stable or exacerbating using a modified Fuch's pulmonary exacerbation score. 226 samples were tested from 111 patients. P. jirovecii was more likely to be detected in samples at acute pulmonary exacerbation (7/76 (9.2%)) compared with stable visits (3/150 (2%)), p=0.03. P. jirovecii was detected less frequently if patients had received co-trimoxazole within 3 months of sample collection (0% versus 29.7%, p=0.03). Prevalence of P. jirovecii in stable patients is low, but P. jirovecii is detected in approximately 1 in 10 patients experiencing an acute pulmonary exacerbation.

Poetry, Music, Writing and Painting; Developing the artistic talents of Adults with Cystic Fibrosis

Art is an expressive outlet for the physical limitations and emotional frustrations of living with a life limiting condition such cystic fibrosis. In the Manchester Adult Cystic Fibrosis Centre we have facilitated the sharing of the inherent artistic talent of our patients with the support of painters, musicians, potters, creative writers, photographers and textile specialists and our own ward staff in our dedicated 22 bed CF inpatient unit. The programme has provided some splendid works that enliven our ward and, more importantly, continue to inspire our patients as they attempt to overcome the socially limiting consequences of hospital admission.

165 “Real world” introduction of tobramycin inhalation powder TIP™ in adults with cystic fibrosis

Objectives To evaluate the introduction of TIP in adult patients attending Manchester Adult Cystic Fibrosis Centre. Methods In December 2012 TIP was introduced in line with NICE recommendations. Tolerance prior to prescription was assessed at test dose followed by 1 month trial of treatment. Outcome measures included spirometry, 7 day symptom score (Bennett et al, 2010 JCF) and perceived treatment burden (1–10 scale, 10 = overwhelming). IV antibiotic days were calculated for patients who completed 12 months of treatment. 12 month follow up is ongoing. Results Values are median (range) unless stated otherwise. 92 patients received a test dose of TIP, mean age 28 years (17–50), baseline FEV1 1.55 (0.58–4.3), baseline FEV1% predicted 43 (21–112). 68 patients completed 1 month trial. 60/68 patients who completed trial continued treatment. 36/60 (60%) continued for >12 months. 23/36 have received 12 month clinical follow up. Table 1 . Comparison of baseline and 1 month data Baseline (n = 60) 1 month follow up (n = 60) FEV1 (litres) 1.625 (0.575–3.69) 1.875 (0.62–4.15) FEV1 % predicted 46 (21–112) 48.5 (21–116) Symptom score 5 (0–16) 4 (0–8) Treatment burden 5 (0–10) 2 (0–8) Values are median (range). Table 2 . 12 month follow up data (n = 23) pre post FEV1 1.7 (0.575–3.65) 1.71 (0.55–3.73) FEV1% predicted 48 (21–80) 46 (20–85) IV days 26 (0–71) 15 (0–56) Conclusion In “real world” use TIP appears to be associated with reduction in perceived symptom scores and treatment burden; improvement in spirometry and reduction in IV days. Only 60% patients continued TIP for >12 months. Reasons for this need further evaluation.

WS08.3 “Real world” experience of introduction of Colobreathe™ (Colo) in adults with cystic fibrosis

Objectives To evaluate the introduction of Colo in adult patients attending Manchester Adult Cystic Fibrosis Centre. Methods Colo was introduced in August 2013 in line with NICE recommendations. Tolerance was assessed at test dose followed by a 1 month treatment trial. Outcome measures at both timepoints included spirometry, 7 day symptom score (Bennett et al., 2010, JCF) and perceived treatment burden (1–10 scale, 10 = overwhelming). IV antibiotic days were calculated for patients who completed 12 months of treatment. 12 month follow up is ongoing. Results 110 patients received a test dose, mean age 28.5 years (16–56), baseline FEV1 2.01 (0.5–5.78), FEV1% predicted 55 (17–112). [Values are median (range) unless stated otherwise]. 82/110 patients completed trial. 77/82 patients continued treatment following 1 month review. 42/77 patients continued for >12 months, 14/42 have 12 month follow up data for FEV1 and IV days. Table 1 . Comparison of baseline and 1 month data Baseline (n = 77) 1 month follow up (n = 77) FEV1 (litres) 2.01 (0.5–5.78) 2.1 (0.62–5.33) FEV1 % predicted 55 (17–124) 58 (20–117) Symptom score 5 (0–15) 5 (0–21) n = 75 Treatment burden 5.5 (0–10) 2 (0–8) n = 64 Table 2 . 12 month follow up data (n = 14) pre post FEV1 1.78 (0.5–4.7) 1.74 (0.55–4.55) FEV1 % predicted 51 (17–101) 46 (22–95) IV days 14 (0–118) 4.5 (0–162) Conclusion Colo is well tolerated at test dose and 1 month with reduced treatment burden and no change in spirometry nor symptom score. Of concern is that almost half of patients stopped long term treatment within 12 months. Ongoing analysis of 12 month outcomes and the reasons behind treatment cessation will guide future practice.

PTH-145 Comparison Of Percutaneous Endoscopic Gastrostomy, Megestrol Acetate And Nasogastric Feeding In Patients With Cystic Fibrosis

IntroductionMalnutrition remains an important and common problem in cystic fibrosis (CF) patients and weight loss associated with poor lung function is considered a predictor of mortality, although weight loss has...

WS13.6 Structural alterations in the end-stage cystic fibrosis lung: comparing histopathology to microCT

WS13.5 Visualising ventilation heterogeneity in mild CF using hyperpolarised 3He MRI, and comparison with lung clearance index (LCI) A. Horsley1,2, H. Marshall1, L. Smith3, D. Hughes4, F. Horn1, L. Armstrong1, J. Parra-Nobles1, S. Cunningham5, I. Aldag3, J. Wild1, C. Taylor3. 1University of Sheffield, Department of Academic Radiology, Sheffield, United Kingdom; 2University of Manchester, Manchester Adult Cystic Fibrosis Centre, Manchester, United Kingdom; 3Sheffield Children’s Hospital NHS Foundation Trust, Department of Respiratory Medicine, Sheffield, United Kingdom; 4Sheffield Children’s Hospital NHS Foundation Trust, Department of Radiology, Sheffield, United Kingdom; 5Royal Hospital for Sick Children, Edinburgh, Department of Respiratory Medicine, Edinburgh, United Kingdom

37 Clinical outcomes of real world Kalydeco (CORK) study

36 The effect of ivacaftor therapy on clinical and PCR-identified microbial diversity of cystic fibrosis lung infection H. Green1,2, C. Paisey3, A. Smith3, P. Barry1, W. Flight1, A. Jones1,2, J. Marchesi3, A. Horsley1,2, E. Mahenthiralingam3. 1University Hospital of South Manchester NHS Foundation Trust, Manchester Adult Cystic Fibrosis Centre, Manchester, United Kingdom; 2University of Manchester, Institute of Inflammation and Repair, Manchester, United Kingdom; 3Cardiff University, Cardiff School of Biosciences, Cardiff, United Kingdom