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Background Patients with cystic fibrosis (CF) who experience acute declines in percent predicted FEV 1 (ppFEV 1 decreased ≥10% relative to baseline) are often not treated with antibiotics for pulmonary exacerbations (PEx), whereas other patients are treated even when they have not experienced a decline in lung function. Methods We analyzed 2 patient cohorts using 3 years of Epidemiologic Study of CF data. Cohort 1 (12,837 patients) experienced a ≥10% acute decline in ppFEV 1 (n = 22,898) and Cohort 2 (10,416 patients) had a clinician-diagnosed PEx (n = 20,731). Results 70.7% of ≥10% decline events were treated with antibiotics; with intravenous antibiotics used 67.1% of the time. 32.0% of clinician-diagnosed PEx declined Conclusions A clinician's decision to diagnose a PEx and treat with antibiotics often is not defined by measured lung function: a ≥10% FEV 1 decline is not considered an absolute indication of a PEx and the lack of a decline does not contraindicate a PEx. Clinicians appear to use the history of prior PEx plus other variables as factors for diagnosing PEx.

Background The Epidemiologic Study of Cystic Fibrosis using 1995–1996 and 2003–2005 data found that CF centres with lowest FEV 1 tended to use fewer intravenous antibiotics. We repeated the analyses using 2013–2014 UK CF registry data to determine if this was still the case. Methods Analysing data for 2013 and 2014 separately, 28 adult CF centres were ranked according to median % age-adjusted FEV 1 . The top 7 centres were placed in the ‘upper quarter' (best FEV 1 ), the bottom 7 centres in ‘lower quarter' (lowest FEV 1 ), and the rest in ‘middle half'. IV use was stratified according to %FEV 1 , then compared between the three groups. Results Centres in the ‘upper quarter' and ‘middle half' used significantly more IV antibiotics compared to centres in the ‘lower quarter' (van Elteren test P -value Conclusions CF centres with lowest FEV 1 are still distinguished by lower use of intravenous antibiotics.

Background: Studies are required that evaluate real-world outcomes of inhaled aztreonam lysine in patients with cystic fibrosis (CF). Methods: Our treatment-evaluator tool assessed the effectiveness of inhaled aztreonam in routine practice in 117 CF patients across four time periods (6-12 (P2) and 0-6months (P1) pre-initiation, and 0-6 (T1) and 6-12months (T2) post-initiation). Outcomes were: changes in %-predicted forced expiratory volume in 1s (FEV1), body-mass index (BMI), hospitalisation days and intravenous antibiotic usage. Results: Median FEV1% predicted for each 6-month period was 38.9%, 34.6%, 37.1% and 36.5%; median change was -2.0% between P2 and P1, increasing to +0.6% (p

Objective: The use of intravenous antibiotics (IVAB) is a central part of the management of pulmonary infections in cystic fibrosis (CF). Adult patients with CF in England were advised to “shield” by the government (23.03.2020–01.08.2020) during the COVID-19 pandemic. Shielding was described as the requirement to not leave home and minimise all face-to-face contact. At our adult CF centre in London, there was a 50% reduction in requirement for IVAB, compared to the same period in 2019. We aimed to identify potential factors contributing to the reduced requirement of IVAB during shielding. Method: An 8-point patient questionnaire was given to 27 patients who had required ≥3 courses of IVAB in the year 2019. These were designed to identify: percentage of patients shielding, percentage of patients starting a modulator, variation in adherence to regular medications, chest physiotherapy, and requirement to IVAB. Results: 70% response rate (n = 19). [Table Presented] Despite a 50% reduction in IVAB compared to the previous year, the majority of patients reported their requirement for IVAB remained the same (68%). There was no self-reported increase in adherence to regular medications or chest physiotherapy. The majority of patients felt their overall health status remained stable or improved (78%). During this period the majority of patients fully shielded (84%) and started Symkevi®/Kalydeco or Kaftrio®/Kalydeco (as part of a trial or compassionate use program) (84%). Conclusion: Increased adherence to regular medication and physiotherapy did not appear to be a factor in reducing the requirement for IVAB observed during the UK COVID-19 pandemic lockdown. Adherence to government shielding may have reduced exposure to community acquired infections and associated requirement for IVAB. Commencement of a modulator therapy was likely to have a positive impact on overall health and associated of reduction in requirement of IVAB.

Background The Standardized Treatment of Pulmonary Exacerbations (STOP) program has the intent of defining best practices in the treatment of pulmonary exacerbations (PEx) in patients with cystic fibrosis (CF). The objective of this analysis was to describe the clinical presentations of patients admitted for intravenous (IV) antibiotics and enrolled in a prospective observational PEx study as well as to understand physician treatment goals at the start of the intervention. Methods We enrolled adolescents and adults admitted to the hospital for a PEx treated with IV antibiotics. We recorded patient and PEx characteristics at the time of enrollment. We surveyed treating physicians on treatment goals as well as their willingness to enroll patients in various study designs. Additional demographic and clinical data were obtained from the CF Foundation Patient Registry. Results Of 220 patients enrolled, 56% were female, 19% were adolescents, and 71% were infected with P. aeruginosa. The mean (SD) FEV1 at enrollment was 51.1 (21.6)% predicted. Most patients (85%) experienced symptoms for ≥ 7 days before admission, 43% had received IV antibiotics within the previous 6 months, and 48% received oral and/or inhaled antibiotics prior to IV antibiotic initiation. Forty percent had ≥ 10% FEV1 decrease from their best value recorded in the previous 6 months, but for 20% of patients, their enrollment FEV1 was their best FEV1 recorded within the previous 6 months. Physicians reported that their primary treatment objectives were lung function recovery (53%) and improvement of symptoms (47%) of PEx. Most physicians stated they would enroll patients in studies involving 10-day (72%) or 14-day (87%), but not 7-day (29%), treatment regimens. Conclusions Based on the results of this study, prospective studies are feasible and physician willingness for interventional studies of PEx exists. Results of this observational study will help design future PEx trials.

BackgroundAdults with cystic fibrosis (CF) have altered large artery haemodynamics which is associated with a persisting systemic inflammatory state. We hypothesized that a short-term intervention favorably influencing the inflammatory status may modify their haemodynamic state.MethodsAdult patients with CF were studied immediately preceding and following 2weeks of intravenous antibiotics. Large artery haemodynamics, principally heart rate-adjusted augmentation index (AIx) were obtained. Blood pressure (BP), spirometry and CRP were also measured.ResultsComplete data was available for 15 patients; mean (SD) age 28 (6)years. CRP was reduced while FEV1% predicted improved. Following treatment AIx was lower: 10.9 (10.9)% to 8.1 (10.9)% (p

WS13.5 Structural correlation of multiple breath washout indices derived from alveolar slope analysis in adult cystic fibrosis K. Hurt1,2,3, D. Hansell3,4, A. Nair4, S. Sheard4, M. Hodson2,3, H. Kalsi2,3, D. Bilton2,3, O. Usmani2,3. 1Brighton and Sussex University Hospitals NHS Trust, Respiratory Medicine, Brighton, United Kingdom; 2Royal Brompton Hospital, Respiratory Medicine, London, United Kingdom; 3Imperial College, London, United Kingdom; 4Royal Brompton Hospital, Radiology, London, United Kingdom

diagnosis 25.8 days (range 1–63, s.d. 13.2). By end of year 1, 30% grew Pseudomonas (inc aeruginosa), 36% had abnormal chest x-rays, 27% required IV antibiotics. No correlation between number of contacts with the team and abnormal x-ray, need for intravenous antibiotics, psychosocial issues or positive respiratory (lavage & cough swab) samples in year 1. Children with pancreatic insufficiency had more contacts with the nurse in the year following diagnosis (16 contacts more, 95%CI 4.6–27.3, p = 0.007) than children who were pancreatic sufficient. Per additional week of oral antibiotics, children had 0.7 more contacts (95%CI 0.1 to 1.3, p = 0.025). When both variables were taken into account, pancreatic insufficiency and an additional week of oral antibiotics were associated with 14.5 more contacts (95%CI 3.7–25.4, p = 0.01) and 0.6 more contacts (95%CI 0.04–1.1, p = 0.04) respectively. Conclusion: Data shows more communication with parents whose children are pancreatic insufficient and need more oral antibiotics. It is unclear whether this reflects increased clinical need or parental anxiety. Screening has revolutionised CF care and we employ a policy of frequent contact & support responsive to parental needs.

Mycobacterium abscessus (MA) is emerging as a potential major pathogen in CF with potential implications for transplantation. Ongoing research shows that an aggressive attempt at eradication is the treatment choice. Three patients underwent eradication treatment at Aberdeen Royal Infirmary during 2014, following the Papworth eradication protocol. Patients underwent 4 weeks of multiple intravenous antibiotics followed by a long-term regime of nebulised and oral antibiotics. Close monitoring of sputum cultures was ongoing throughout this time. To date, all three patients have had 3 consecutive negative sputum cultures for MA following eradication therapy. The CF Foundation and ECFS draft guidelines state that eradication therapy should continue for one year after the first of 3 negative sputum cultures, thus within the next 3 months, all 3 patients will be able to discontinue their long-term eradication treatment and return to a routine CF clinic within the department. Patients A and B both dropped their FEV1 predicted values during eradication therapy (9.67% and 30.73% respectively) whilst Patient C experienced a slight increase in predicted FEV1 of 6.78%. Patient B has commenced anti-asthma therapy, which has led to an improvement in her most recent FEV1 recording. All 3 patients have gained weight, with Patient C gaining 13.8 kg over the last 8 months since commencing eradication treatment. Patients A and B have both gained just over 1 kg in weight in this time. Eradication treatment for MA is complex and difficult for some patients to tolerate. We believe that further research regarding treatment and cross infection is imperative.

Objectives The UK CF Trust Standards of care recommend that all adults undergo a chest X-ray (CXR) at annual review; to assess clinical progression and complications. However, the value of CXR in assessing disease progression in CF is debated, with some recommending they are replaced by CT scans (Tiddens et al. 2014). We wished to assess the clinical impact of the routine CXR at annual review. Methods We identified 83 (43%) patients of our total CF cohort (n = 196) who underwent CXR solely as part of their annual review. Radiographs were compared with previous studies and any changes in clinical management resulting from them identified. Results The average age was 30±9 yrs, with a mean FEV 1 79±22%. 69% were male and 31% female. 36% had CF related diabetes. 57% were colonized with Pseudomonas, 7% with B. cepacia and 7% with M. abscessus. Mean oral and intravenous antibiotic days in the preceding year were 14±19 and 16±25 respectively. Of the 83 patients, 15 (18%) had new findings, 8 of whom (53%) went on to have a thoracic CT scan. 14 (17%) needed a change in clinical management. The remaining 68 patients had unchanged CXR appearances, were clinically stable, and required no alteration in management. Conclusions As life expectancy increases annual surveillance of stable patients is an increasingly important part of CF care. We found that screening CXRs identified changes in

Objectives The use of intravenous antibiotics has long been established as the treatment for respiratory exacerbations in patients colonised with Pseudomonas aeruginosa. However improvements in spirometry are not always demonstrated following treatment. Patients often place significant importance on these readings and may doubt the benefit of treatment if an increase in lung function is not seen. A small study comparing the results of exercise test results with spirometry at the start and end of a course of intravenous antibiotics to see if they could be used as a supplementary measure to assess the benefit of treatment. Methods 27 patients (63% male; mean age 35 yrs, range 17–70; mean % FEV1 45.7%, range 17–86%) who were admitted for a two week course of intravenous antibiotics were approached to check their lung function and exercise tolerance at the start of their admission. 18 patients agreed and 9 declined. The tests were then repeated at the end of the hospital admission. The incremental shuttle walk test and the 6 minute walk test were used depending on patient preference. Results 10 patients (55%) show improvement in both their spirometry and exercise tolerance, however 8 patients (45%) demonstrated less than a 2% increase in their lung function; 3 recording a lower value than at the start of treatment. Of these 8 patients, 7 showed improvements in their exercise test results, mean 48.6%, range 7.6–85%. Conclusion Exercise testing as a supplementary measure to assess the benefit of intravenous antibiotic therapy may be of value, particularly in patients who do not show an improvement in spirometry.

Objective Procalcitonin (PCT), a precursor of calcitonin is selectively raised in bacterial infections. The aim of this pilot study was to investigate the role of PCT as a surrogate marker of infection in adults with CF and to discriminate between infection and inflammation in patients with elevated C reactive protein (CRP). Methods Adults with CF receiving hospital intravenous antibiotics for a pulmonary exacerbation were recruited. PCT levels were prospectively measured in patients with elevated C reactive protein (CRP). Results To date 11 subjects, median (range) age, 32 (21–51) yrs have been investigated. CRP and Procalcitonin levels were 185 (69–283) mg/L and 0.3 ( Conclusion PCT is a more specific marker of severe infection than conventional markers such as CRP. Preliminary data suggests that PCT may be useful in discriminating between severe pulmonary and endobronchial infection as well as noninfectious inflammation. This study is ongoing.

352 A teamwork on a multiregional network: an experience about harmonizing homecare practices for intravenous antibiotic on an implantable catheter port M. Kerbrat1, R. Fretay2, D. Chailleux3, F. You3, K. Sery4, M. Chiffolleau5, S. Baumard5, M.T. Jousseaume6, I. Berhault6, M.C. L’Abbe6, C. Pingon6, A. Bataillou6, M. Idres7, C. Matras8, S. Paul9, M.L. Madec9, F. Goarent9, C. Le Saint9, Y. Kerneur9. 1CF Center Nantes, Roscoff, France; 2CF Center Vannes Lorient, Vannes, France; 3CF Center, Nantes, France; 4CF Center, St Nazaire, France; 5CF Centre, Angers, France; 6CF Center, Rennes, France; 7CF Center, St Brieuc, France; 8CF Center, Tours, France; 9CF Center, Roscoff, France