1
37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
www.goteborg.com/Jorma Valkonen
11 – 14 JUNE 2014 GOTHENBURG, SWEDEN
FINAL PROGRAMME
WWW.ECFS.EU/GOTHENBURG2014
HIGHLIGHTS OF THE CONFERENCE
ECFS TOMORROW LOUNGE
INTERACTIVE CASE STUDIES: TEST YOUR SKILLS AND RECEIVE IMMEDIATE FEEDBACK!
The ECFS Tomorrow initiative is specially geared towards assembling those who are interested in building their future career in the Cystic Fibrosis community and the ECFS of tomorrow. The ECFS Tomorrow Lounge will feature: • An exciting series of mini-workshops aimed at career development • A relaxed space for conversation and networking
FRIDAY 08:30 – 10:00
Each interactive case presents an evolving patient history and a series of questions designed to test your diagnostic and/or therapeutic skills. You will receive immediate feedback on your answers and treatments choices, along with the opportunity to compare your final score with those of your peers.
More information page 71
ECFS Tomorrow Lounge
E1-E3
E4
MEET THE EXPERTS SESSIONS ePoster Corner B
THURSDAY & FRIDAY: 07:15 – 08:15 ePOSTER CORNERS
Incontro Bar & Restaurant
MIX Restaurant
Exhibition & Poster Area
ePoster Corner A
te Ho
MAIN ENTRANCE
lR
ec
ep
Re
tio
n
gi
st ra
tio
n
In these small interactive breakfast sessions you will have the opportunity to ask questions and discuss a specific topic with experts from the field. The format encourages a more personal approach to learning. Registration for these sessions is additional to the conference.
LEVEL 0
FOLLOW US ON TWITTER * JOIN US ON LINKEDIN * VISIT US ON FACEBOOK
TABLE OF CONTENTS 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
WELCOME / COMMITTEES / ECFS
Patron & Committees Welcome Message European Cystic Fibrosis Society ECFS Working Groups ECFS Young Investigators Awards Travel Grants Awardees ECFS Awards In Memoriam
4 5 6 7 8 9 10 11
CONFERENCE OVERVIEW
Programme Content Description Schedule at a Glance
12 13 – 15
SCIENTIFIC PROGRAMME Wednesday, June 11 Thursday, June 12 Friday, June 13 Saturday, June 14
16 – 17 18 – 28 30 – 40 42 – 45
Poster Area Guided Poster Tours - ePoster Sessions Poster Viewing Posters
46 48 49 50 – 59
POSTERS
MEETINGS
Meetings Overview Meetings Programme
60 61 – 67
EXHIBITION & SATELLITE SYMPOSIA
Sponsor Acknowledgement ECFS Tomorrow Lounge Satellite Symposia Exhibition Floor Plan Exhibitors Profiles CF Community Area
70 71 72 – 73 75 77 – 80 82
GENERAL INFORMATION & INDICES
Conference Floor Plan General Information A – Z Social Programme Speaker, Moderator, Leader, Oral & Poster Presenter Index
86 – 87 88 – 91 92 94 – 98
3
4
PATRON & COMMITTEES 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
PATRON OF THE 37TH EUROPEAN CYSTIC FIBROSIS CONFERENCE Her Majesty Queen Silvia of Sweden
COMMITTEES STEERING GROUP Stuart Elborn, UK Anders Lindblad, SE Gunnar C. Hansson, SE Miguel Cámara, UK Eitan Kerem, IL Su Madge, UK David Sheppard, UK SCIENTIFIC COMMITTEE Scott Bell, AU Thomas Bjarnsholt, DK Mark Butler, UK Jian-Min Chen, FR Raphael Chiron, FR Gary Connett, UK Jane Davies, UK Kris De Boeck, BE Isabelle de Monestrol, SE Nico Derichs, DE Isabelle Fajac, FR Michael Fayon, FR Juliet Foweraker, UK Luis Galietta, IT Charles Gallagher, IE Marita Gilljam, SE Vincent Gulmans, NL Dominic Hartl, DE Trudy Havermans, BE
Helge Hebestreit, DE Lena Hjelte, SE Ellen Julie Hunstad, NO Ferenc Karpati, SE Batsheva Kerem, IL Karsten Kötz, SE Gary Latchford, UK Irene Maguire, IE Eshwar Mahenthiralingam, UK Luigi Maiuri, IT Marcus Mall, DE Hanne Olesen, DK Helen Parrott, UK Jean-Marc Rolain, FR Margareta Sahlberg, SE Kirsten Schaffer, IE Bob Scholte, NL Ursula Seidler, DE Nick Simmonds, UK Kevin Southern, UK Henkjan Verkade, NL Helen White, UK LOCAL LIAISON COMMITTEE Maria Almbacke Duygu Bayraktar Ersoy Britt Leopold Ulrica Sterky
CONFERENCE ORGANISER European Cystic Fibrosis Society Kastanieparken 7 DK-7470 Karup J. Denmark Tel.: +45 8667 6260 Fax: +45 8667 6290 E-mail:
[email protected]
CONFERENCE SECRETARIAT ECFS 2014 Conference Secretariat c/o K.I.T. Group GmbH Kurfürstendamm 71 D-10709 Berlin E-mail:
[email protected] Fax: +49 30 24 603 200 Tel. Registration: +49 30 24 603 338 Tel. Hotel: +49 30 24 603 336 Tel. Sponsoring/Exhibition: +49 30 24 603 242
WELCOME MESSAGE 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
WELCOME TO THE 37TH ECFS CONFERENCE
WELCOME TO GOTHENBURG
Dear Friends and Colleagues,
The ECFS plays an invaluable role in inspiring and guiding progress of CF care in Sweden, particularly through its conferences, publications and consensus reports. Riksförbundet Cystisk Fibros (RfCF), the Swedish Cystic Fibrosis Association, has translated the European Standards of Care into Swedish, something very valuable when lobbying the health authorities for financial support for the CF centers.
It is a great pleasure to welcome you to Gothenburg for the 37th European Cystic Fibrosis Conference. At the ECFS conference, we provide a forum for the discussion of the best and most recent basic and applied science to facilitate translation of the latest knowledge into daily clinical practice. The programme reflects these priorities and brings together scientific and clinical teams from around the world. A high quality programme is planned with international leaders in the field delivering plenary and symposia lectures. The 37th ECFS conference takes place at the Swedish Exhibition & Congress Centre in Gothenburg, one of the most effective venues for conferences in all Scandinavia. It enjoys a unique location right in the centre of Gothenburg and in walking distance to hotels, restaurants, and entertainment. Gothenburg is situated by the sea on the west coast of Sweden and is Sweden‘s second largest city as well as the fifth largest in the Nordic countries. The city was founded in 1621 by King Gustav Adolf II and has since been on an exciting journey from being a shipping and industrial city to a creative hub for innovation. Gothenburg is home to many students and the atmosphere in the city has a culture of energy and fun while promoting learning and knowledge. One of Gothenburg‘s most popular natural attractions is the Southern Gothenburg Archipelago, which is a set of many picturesque islands that can be easily reached by public transportation. This is an excellent opportunity to discuss the important new developments in CF research and clinical care. We are confident the conference will invigorate all in the CF community to continue their efforts to improve quality of life and survival for people with CF.
It is therefore an honour and a great pleasure for the Swedish Cystic Fibrosis Association to be involved in hosting the 2014 ECFS Conference in Gothenburg, Sweden. The venue, Svenska Mässan, is recognized as one of the best large conference venues in Sweden. It is located centrally in Gothenburg and within walking distance to the city. The Swedish Cystic Fibrosis association is very excited by the development of the European CF Patient Registry including many different countries where comparisons of outcomes can be evaluated. Sweden has a lower incidence of CF than in some other European countries. The total number of people with CF is about 680. We have centralized care at four different CF centres, all located in the southern part of the country. People with CF from remote areas in Sweden have to rely on shared care with involvement of their local hospital and this might result in a diminished quality of care if not carefully evaluated continuously. We are now implementing a Swedish CF Registry and it is with excitement we look forward to the results, especially if there are any regional differences to be found. Sweden is one of the few countries which has still to implement newborn screening for CF. RfCF has this at the top of its agenda and we hope this conference will help us to promote this important issue. Welcome!
We are delighted that you have joined us for this exciting, challenging and good fun event and we extend a very warm welcome to Gothenburg!
Stuart Elborn
Anders Lindblad
Gunnar C. Hansson
Ulrica Sterky
Britt Leopold
ECFS President
Conference President
Conference Vice President
Chairperson Local Liaison Committee
Executive Director Swedish Cystic Fibrosis Association
5
6
TITLE EUROPEAN CYSTIC FIBROSIS SOCIETY 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
The European Cystic Fibrosis Society is an international community of scientific and clinical professionals committed to improving survival and quality of life for people with CF by promoting high quality research, education and care.
ECFS PROJECTS CLINICAL TRIALS NETWORK
CTN Director: Isabelle Fajac, Paris, FR The aim of the European Cystic Fibrosis Clinical Trials Network (ECFS CTN) is to intensify clinical research and to bring new medicines to the patients as quickly as possible. This is done by: • Sharing expertise among dedicated CF researchers • Involving and cooperating with the patient organizations • Centralizing review of clinical trial protocols in cooperation with the pharmaceutical industry • Supporting the study conduct in the 30 network sites • Standardizing research procedures and outcome parameters • Providing training to the site’s staff Apart from study design, motivating patients to take part in research and promoting safety of participants in clinical trials are of great importance. The CTN is also in close contact with the CFF Therapeutics Development Network in the US to work together on the review and conduct of global studies. Number of sites: 30 in 11 countries Number of patients represented: 14,000 www.ecfs.eu/ctn
PATIENT REGISTRY
ECFSPR Director: Ed McKone, Dublin, IE The ECFS Patient Registry collects demographic and clinical data from consenting Cystic Fibrosis (CF) patients in Europe. The outcomes of data analyses and comparisons are used to improve standards of care for CF patients, promote innovation and facilitate public health planning and research. See more about the Patient Registry and what happens to the data on www.ecfs.eu/projects/ecfs-patient-registry/intro. The ECFS Patient Registry contains data of over 30,000 patients from 23 countries.
CONFERENCES
Annual ECFS Conference The ECFS annual conference provides a forum for all with a common interest in CF to meet and discuss their latest findings. Basic Science Conference The “New Frontiers in Basic Science of Cystic Fibrosis” Conference is characterised by active discussion of data and ideas at the forefront of research on CF and CFTR, in an informal, co-operative environment.
PUBLICATIONS: JOURNAL OF CYSTIC FIBROSIS
The journal is devoted to promoting the research and treatment of Cystic Fibrosis. The journal publishes original scientific articles, editorials, case reports, short communications and other information relevant to CF. There are currently 6 issues a year with supplements. The Journal of Cystic Fibrosis is published by Elsevier and the Editor in Chief is Dr. Scott Bell, Brisbane, AU.
ECFS GUIDELINES
ECFS Guidelines on issues associated with Cystic Fibrosis: www.ecfs.eu/ecfs_guidelines
CF CONSENSUS REPORTS
www.ecfs.eu/publications/consensus_reports To learn more about the ECFS, visit us at our booth located in the CF Community Area!
CONTACT US European Cystic Fibrosis Society Kastanieparken 7 DK – 7470 Karup J. Tel: +45 86 67 6260 Fax: +45 86 67 6290 Email:
[email protected] Website: www.ecfs.eu
ECFS WORKING GROUPS TITLE 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
DIAGNOSTIC NETWORK WORKING GROUP www.ecfs.eu/ecfs_dnwg Coordinator: Nico Derichs, Berlin, DE AIMS: • Achievement of international consensus on the definitions of CF and CF related disorder • Development, evaluation and European standardisation of new diagnostic techniques • Exchange of emerging information on difficult CF diagnosis in a multinational network of experts • Cooperation with the ECFS Patient Registry about CF diagnosis documentation in registries • Application of diagnostic techniques for drug development and clinical trials in CF, in cooperation with the ECFS Clinical Trials Network 2014: 70 MEMBERS, 22 COUNTRIES
NEONATAL SCREENING WORKING GROUP www.ecfs.eu/www.ecfs.eu/ecfs-neonatal-screening-wg Coordinator: Kevin Southern, Liverpool, UK AIMS : • To support the implementation of newborn screening (NBS) for CF • To monitor performance and compare protocols to optimise effectiveness, whilst reducing negative impact • To encourage enrolment of all infants identified through NBS in clinical trials • To determine the optimal management of infants with an equivocal diagnosis following newborn screening 2014: 460 MEMBERS
EXERCISE WORKING GROUP www.ecfs.eu/ecfs_exercise_wg Coordinator: Helge Hebestreit, Würzburg, DE AIM: To advance patient care in Europe by improving exercise/physical activity assessments and exercise counselling. Specifically, the group will address the following objectives over the next years: • Evaluate existing knowledge on exercise testing, physical activity assessment and exercise counselling/conditioning programmes in Cystic Fibrosis and standardise procedures by generating consensus statements in collaboration with experts from North America and Australia • Stimulate projects to obtain missing information, and • Foster collaboration between European centres
EUROPEAN CF GENE MODIFIER WORKING GROUP Coordinator: Harriet Corvol, Paris, FR AIM: Cystic Fibrosis (CF) is a single gene disorder but considerable clinical diversity exists among patients with the same CFTR mutations, especially lung disease severity. In France, a national study on CF modifier genes has been underway since 2006 and almost 3,500 French CF patients already participated. Similar independent initiatives have been conducted in several European countries. As such, combining these studies into a large European Consortium is essential. Our aim is to amalgamate French and EU-wide clinical and DNA data to identify genetic variants involved in CF disease severity. As similar approaches are currently underway in North-America, effective collaborations are in progress with the ultimate goal of creating an International Consortium.
7
8
ECFS YOUNG INVESTIGATORS AWARDS 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
Following the recommendations of the Scientific Committee during the abstracts review process, the ECFS wishes to commend the quality of the work presented in the abstracts of some young investigators under the age of 35 having applied for the award. The Young Investigator Award includes a monetary grant of € 750, a free registration to the Conference, and a 2014 ECFS membership subscription. We wish to extend our congratulations to the following Young Investigators:
BRICE HOFFMANN After studying Biology at the university of Evry, France, I gradually shifted to structural bioinformatics by interest in molecular modeling. I then had the opportunity to do a PhD in Computational Biology at the Ecole des Mines de Paris, France in 2007 in the laboratory of Jean-Philippe Vert and under the direction of Veronique Stoven. During my PhD, I developed new methods for predicting protein-ligand interactions using machine-learning algorithms. Since 2012, I am a postdoc at the University Pierre et Marie Curie, Paris, France, under the supervision of Isabelle Callebaut. I study the 3D structure of the CFTR protein, which has been modeled by homology in the laboratory. I am mainly interested in molecular dynamics simulations and virtual screening of small molecules by docking and ligand-based approaches. The aim of this collaborative study, involving the expertise of research teams from different scientific fields (biophysics, chemical biology, physiology), is to amplify the syntheses and experimental characterizations of promising molecules, acting as correctors of the CFTR protein. This work is supported by the French Cystic Fibrosis Association “Vaincre la Mucoviscidose“. About the research presented at the 37th European CF Conference, abstract number WS23.1: We performed molecular dynamics experiments from our model of the open form of CFTR protein made by homology on the Sav1866 template. This model was validated using structural information provided by new experimental 3D structures of ABC exporters, and by taking into account the internal symmetry involving the two halves of the protein. We obtained a relevant model of the full open form of the anion channel, particularly well consistent with the experimental data available today. A lateral access path for ions and molecules from the cytosol was also highlighted.
KATHERINE O‘NEILL Dr Katherine O’Neill is a clinical research fellow and a physiotherapist at the Centre for Infection and Immunity, Queen’s University Belfast. She recently completed her PhD which examined the use of lung clearance index as a measure of lung disease severity in Cystic Fibrosis. Her current research interests focus on assessment of the relationships between lung clearance index and the lung microbiota and the responsiveness of measures of inert gas washout tests during airways clearance in Cystic Fibrosis. About the research presented at the 37th European CF Conference, abstract number WS20.5: In her study of 29 clinically stable, child and adult patients with Cystic Fibrosis, a higher colony count of anaerobic bacteria resulted in a better lung clearance index and lower CRP, indicating that a lower load of anaerobic bacteria may reflect microbiota disruption and disease progression in the Cystic Fibrosis lung.
RASMUS L. MARVIG Rasmus Marvig, PhD is specialized in genomic analysis of clinical isolates of bacteria, and he currently works as a research scientist at Rigshospitalet, Copenhagen University Hospital. Rasmus Marvig did his doctorial research in medical microbiology at the Technical University of Denmark from which he also holds an M.Sc.Eng. with honors in Biotechnology. During his studies, Rasmus Marvig has acquired skills in microbiology and genome sequencing from several international partners, and he has been admitted as a Visiting Researcher to Stanford University, Harvard Medical School, Massachusetts Institute of Technology, and École Polytechnique Fédérale de Lausanne. In 2012 Rasmus Marvig was academically honored with the Danish Ministry of Science’s EliteForsk Travel Scholarship. Moreover, he has attained a Graduate Certificate in Business Administration at Copenhagen Business School. About the research presented at the 37th European CF Conference, abstract number WS21.1: In his research, Rasmus Marvig studies the evolution of microbial populations during infection in order to understand the capacity of bacterial pathogens to adapt to different anatomical niches, evade the host immune system, and overcome therapeutic challenges. Rasmus Marvig will in his talk present how insight into the genomic evolution of clonal lineages of Pseudomonas aeruginosa within Cystic Fibrosis airways leads to improved understanding of pathogenesis and the nature of epidemics, and may help the design of effective therapeutic measures.
TRAVEL GRANTS AWARDEES TITLE 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
This year 20 Travel Grants were awarded to outstanding young European investigators who are under the age of 35 registered for a PhD or post-graduate degree or who have graduated within the last 12 months. The Travel Grant includes a monetary grant of € 300, a free registration to the Conference, and a 2014 ECFS membership subscription. We wish to extend our congratulations to the following Travel Award Winners:
TRAVEL GRANTS AWARDEES
ABSTRACT NUMBER
Juan de Dios Caballero, Madrid, ES
83
Clizia Cazzarolli, Verona, IT
WS9.2
Priscilla Cocchi, Florence, IT
57
Sian Counihan, Limerick, IE
251
Louise Cullen, Dublin, IE
52
Barbara Dhooghe, Brussels, BE
WS1.2
Simon Graeber, Heidelberg, DE
WS23.3
David Hannon, Belfast, UK
129
Julia Hentschel, Jena, DE
WS19.6
Elise Lammertyn, Leuven, BE
WS13.6
Renan Mauch, Campinas, BR
119
Luke McGuigan, Dublin, IE
WS19.5
Stephen McWilliam, Liverpool, UK
66
Patrizia Morelli, Genoa, IT
72
Maria Noni, Athens, GR
152
Simon Pybus, Liverpool, UK
14
Johanna Salomon, Heidelberg, DE
WS17.3
Nicole Scheuing, Ulm, DE
WS6.5
Emem-Fong Ukor, Cambridge, UK
227
Constanze Vitzthum, Giessen, DE
WS17.2
9
10
TITLEAWARDS ECFS 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
ECFS AWARD Prof. Eitan Kerem, MD is a world known leader in Cystic Fibrosis research, principal investigator of many national and international multi-centre clinical trials and author of key publications in the field. His interest in Cystic Fibrosis spans all aspects, i.e., the association between phenotype and genotype, prognostic factors for disease severity and prediction of mortality, the molecular mechanisms for disease variability, and development of new mutation-specific pharmacological therapies to correct the basic defects in Cystic Fibrosis. He was also involved in formulating the guidelines that paved the road to the standardization of CF care in Europe and other parts of the world. Additional aspects of his research include asthma (environmental interaction with genetic diseases and its influence on the disease severity, genetic markers of asthma and allergy in closed populations, and variability of disease in closed populations exposed to different environments) and community acquired pneumonia (nectrotizing pneumonia and risk factors for complicated pneumonia). The promotion of the medical, ethical and rights aspects of children with chronic diseases is at the forefront of his interest. He published many papers in this field and is known for his advocacy for children in need. He also has a record of building bridges between Israelis and Palestinians furthering peace through medicine wherever possible. Prof. Kerem was Chairman of the Medical Advisory Board for the Israeli CF Foundation and served as a Board member of the European CF Society. He was a member of numerous organizing committees for national and international conferences. He is a recipient of the prestigious Ordine Della Stella Della Solidarieta Italiana Award. In 2010 he received the “Shalem” award bestowed by the National Foundation for the development of services for individuals with intellectual disabilities. Prof. Kerem was the president of CIPP, the International Congress on Pediatric Pulmonology. In addition he was a member of the editorial boards of “Pediatric Pulmonology,” “Chest“ and “American Journal of Respiratory and Critical Care Medicine,” the leading journals in the field of Pulmonology. He has authored over 170 papers covering all aspects of Pulmonology.
ECFS LIFETIME CONTRIBUTION AWARD Birgitta Strandvik was born in Stockholm in 1938, graduated as MD at the Karolinska Institutet, Stockholm, Sweden in the middle 60s and was authorized in paediatrics in 1974. Her main interest in clinics and research has been focused on pediatric hepatogastroenterology and nutrition and already from her first clinical position in 1964 she has been involved in the care of patients with Cystic Fibrosis. She obtained her PhD in 1973 about “Bile acid metabolism in cholestasis of infancy” and was the next year appointed associate professor at Karolinska Institutet. In 1990 she was appointed professor and chairman at the Department of Pediatrics, the University of Gothenburg, but is after the official retirement in 2007 back as senior professor at the Karolinska Institutet in Stockholm at the Department of Bioscences and Nutrition. She led the Lipid Research Laboratory from 1975-2011. She was President of ESPGHAN 1990-92, pro-vice chancellor of the University of Gothenburg 1995-97 and President of the Swedish Society of Medicine 1998-2000. She has been a member of several international and national scientific boards and for 10 years a member of the Ethical Group against Misconduct and Fraud in Science associated to the Swedish Research Council. She has organized several international meetings, as the ICFMA/ECFS in Stockholm in 2000. She was in 2000 awarded the CFRI Professional Excellence Award, USA, for “tireless dedication and outstanding contribution to CF research and the Cystic Fibrosis community”. She has published more than 250 original papers in international journals and supervised more than 25 PhD students. About half of the publications and the PhD students have been devoted to Cystic Fibrosis. She has founded the CF centres in Stockholm 1974 and in Gothenburg 1992 and been the director of the respective CF centre for decades. Three of the four Swedish CF centres are now led by her PhD students. Her interest in CF research has been based on her clinical interest in the patients‘ wellbeing and thus had a broad approach. She introduced physical activity in the treatment of the patients 30 years ago, which together with the focus on compensation of the low linoleic acid concentrations characteristic of most patients with CF, might have contributed to the good clinical status and survival of the Swedish CF population. The animal studies related to CF have been complementary to the understanding of the fatty acid abnormality. This interest has led to an extended interest of fatty acid metabolism in relation to programming and diseases in later life.
IN MEMORIAM 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
PROFESSOR DR. GERD DÖRING The field of Cystic Fibrosis lost one of its most important and influential personalities in summer 2013. Prof. Gerd Döring was Editor-in-Chief of the Journal of Cystic Fibrosis (2006–2013) and most recent past President of the European Cystic Fibrosis Society (ECFS) (1998–2006). Prof. Döring’s undergraduate studies were in pharmaceutical chemistry and microbiology. He worked in the University of Tübingen throughout his professional life mostly in the Institute of Hygiene. In 1983, he was appointed Professor of Experimental Hygiene and Experimental Microbiology at the University of Tübingen, where he had received his degree in chemistry in 1974 and his doctorate in natural chemistry in 1978. His doctoral thesis was in transition metal complexes and his inaugural dissertation in the University of Tübingen on Pseudomonas aeruginosa infection in Cystic Fibrosis. In the 80s and early 90s, he spent time studying with Niels Høiby in Copenhagen and in 1992 was awarded a Postgraduate Scholarship from the French Government to work in Lyon. These collaborations had important outcomes and also became the basis of international collaborations with colleagues from many countries especially from Denmark, France, Italy, UK, The Netherlands, USA and Canada. His most significant scientific articles changed the understanding of the pathogenesis of Cystic Fibrosis lung disease particularly in the area of innate immunity, inflammation and the anaerobic conditions found in the CF airway. His most recent important contributions were in understanding the behaviour of anaerobic bacteria, T-cell immunology and regulation in chronic Pseudomonas infection and the importance of cross-infection and potential for treatment with vaccination. He had also developed an interest in the use of inhaled nitric oxide as a therapeutic intervention in Cystic Fibrosis and in the days before he passed away was still actively working on a programme of research to investigate this new treatment modality. In addition to his comprehensive scientific activities Prof. Döring was a significant leader of the European Cystic Fibrosis community. He became President of the European Cystic Fibrosis Society in 1998 following its successful move from the European Working Group for Cystic Fibrosis. During his Presidency, the society became internationally very successful with membership numbers doubled by the end of his term in 2006. During this time he also initiated a highly successful annual conference on basic science and consensus conferences on the treatment of lung infection, early intervention and prevention of lung disease, drug inhalation and related devices, nutrition, along with recommendations for several clinical trials in Cystic Fibrosis, which have had a significant impact on treatment. At the completion of his Presidency of ECFS, Prof. Döring became the Editor-in-Chief of the Journal of Cystic Fibrosis and worked tirelessly, including the days before his passing, on making JCF the pre-eminent journal in the field of Cystic Fibrosis.
The ECFS wishes to honour the memory of Prof. Döring and will be initiating an annual Gerd Döring Award.
11
12
TITLE PROGRAMME CONTENT DESCRIPTION 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
PLENARY
The Plenary sessions, lasting 90 minutes, comprise keynote lectures of experts in the field.
SYMPOSIA
The sessions, lasting 90 minutes, comprise 3 to 4 lectures by experts in the field covered by the symposium and specifically in the topic presented. Each presentation is followed by a discussion time when all attendees are invited to ask questions about/discuss the presentation.
WORKSHOPS
The sessions, lasting 90 minutes, comprise oral presentations of scientific work as outlined in the abstracts. The moderators of the workshops, experts in the field covered by the workshop, will introduce the different presentations and facilitate the discussion time when all attendees are invited to ask questions about/discuss the presentation.
ROUND TABLE DISCUSSIONS
The Round Table discussions, which have a limited attendance, are led by an expert in the topic covered by the round table. The expert will provide a short introduction and then facilitate the discussion.
GUIDED POSTER TOURS
These one-hour tours are led by an expert in the field commenting on the posters displayed at the Conference.
ePOSTER SESSIONS
These are moderated thematic sessions, during which ePosters will be presented on a 50-inch flat screen. These are scheduled in the ePoster Corners A and B, located within the Exhibition and ePoster Corner C, located in front of session rooms H1 & H2, Level 1 of the Conference Centre.
POSTER EXHIBITION
Only abstracts accepted as posters will be presented in their respective categories.
CASE PRESENTATIONS
Informative cases related to diagnosis or management of CF will be presented and discussed. A panel of experts in the field facilitate the discussion and foster exchange of information or discuss treatment possibilities based on their expertise and knowledge of the latest scientific publications.
INTERACTIVE CASE STUDIES
Each interactive case presents an evolving patient history and a series of questions designed to test your diagnostic and/or therapeutic skills. You will receive immediate feedback on your answers and treatments choices, along with the opportunity to compare your final score with those of your peers.
MEET THE EXPERTS
The Meet the Experts sessions are small interactive sessions led by experts to stimulate discussion, answer questions and offer advice. The format encourages a more personal approach to learning.
ePOSTERS
An ePoster is an electronic version of the traditional paper poster displayed on computers. You will be able to consult the ePosters by using one of the computers in the ePosters Area located within the Exhibition. Abstracts selected for an oral presentation do not have a corresponding paper poster but an ePoster only.
SATELLITE SYMPOSIA
The most recent developments in the CF field will be presented during Satellite Symposia supported by industrial partners.
INDUSTRY EXHIBITION
The exhibition includes companies involved in Cystic Fibrosis and features the latest products and services offered by the industry.
MEETINGS
Each year several special interest CF groups hold their meetings in conjunction with the Conference.
OPENING HOURS 18:00 – 21:30 09:00 – 18:00 09:00 – 18:00 09:00 – 12:30
CLOAKROOM & BAGGAGE CLAIM Wednesday, June 11 Thursday, June 12 Friday, June 13 Saturday, June 14
14:00 – 21:30 06:45 – 21:00 06:45 – 19:00 08:30 – 13:30
SPEAKER PREVIEW ROOM Wednesday, June 11 Thursday, June 12 Friday, June 13 Saturday, June 14
10:00 – 19:00 06:45 – 18:00 06:45 – 18:00 08:00 – 11:00
POSTER AREA Wednesday, June 11 Thursday, June 12 Friday, June 13 Saturday, June 14
18:00 – 21:30 08:00 – 18:00 08:00 – 18:00 08:30 – 12:30
REGISTRATION Tuesday, June 10 Wednesday, June 11 Thursday, June 12 Friday, June 13 Saturday, June 14
07:45 – 17:00 08:00 – 20:00 07:00 – 19:30 07:00 – 19:30 08:30 – 13:30
POSTERS PRESENTERS ACCESS Wednesday, June 11 Thursday, June 12 Friday, June 13 Saturday, June 14
14:00 – 18:00 08:00 – 18:00 08:00 – 18:00 08:00 – 14:00
INDUSTRY EXHIBITION Wednesday, June 11 Thursday, June 12 Friday, June 13 Saturday, June 14
SCHEDULE AT A GLANCE TITLE 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
TUESDAY, JUNE 10 08:15 – 17:00 Meeting
Physiotherapy Short Course - The Use of NIV in Children and Adults with CF - Physiology and Clinical Application**
J2
08:30 – 17:30 Meeting
ECFS Board Meeting*
R4
WEDNESDAY, JUNE 11 08:30 – 11:00 Meeting
ECFS CTN Training & Development*
09:00 – 13:45 Meeting
ECFS Exercise Working Group*
H2
09:00 – 16:15 Meeting
International Nurse Specialist Group/CF Meeting**
H1
09:00 – 12:30 Meeting
Physiotherapy Short Course**
J2
09:30 – 12:30 Meeting
CFE/ECFS Joint Symposium - Access to Personalised Medicine
K1
09:30 – 15:00 Meeting
ECFS Neonatal Screening Working Group Meeting
G1
09:30 – 16:40 Meeting
European Cystic Fibrosis Nutrition Group Meeting
R31
09:30 – 16:00 Meeting
European Psychosocial Special Interest Group (EPSIG) Meeting**
11:30 – 15:30 Meeting
ECFS CTN Training & Development*
J1
12:00 – 16:00 Meeting
Journal of Cystic Fibrosis (JCF) Editorial Board Meeting*
R4
12:30 – 14:00 Meeting
International Physiotherapy Group for Cystic Fibrosis (IPG/CF) Annual General Meeting*
J2
13:15 – 17:00 Meeting
ECFS CTN Steering Group Meeting*
H2
13:30 – 16:30 Meeting
ECFS Latin American Meeting**
G2
14:00 – 17:00 Meeting
ECFS Patient Registry Executive Committee Meeting*
14:30 – 16:30 Meeting
Physiotherapy Case Presentations
J2
15:00 – 17:00 Meeting
ACTIVATE-CF Meeting*
G1
17:00 – 18:00 Meeting
ECFS Annual General Meeting*
K1
17:00 – 18:30 Meeting
ECFS Patient Registry Training Meeting*
R4
18:30 – 20:00
Opening Plenary
20:00 – 21:30
Welcome Reception
R26
R5+6
R26
K2-K3 Exhibition
THURSDAY, JUNE 12 07:15 – 08:15
Round Tables
Congress Hall Foyer
07:15 – 08:15 Meet the Experts
Multiple breath washout
ePoster Corner A
07:15 – 08:15 Meet the Experts
Mucus, mucins, and Cystic Fibrosis
ePoster Corner B
07:15 – 08:15 Meet the Experts
Family therapy approaches with our families
ePoster Corner C
08:30 – 10:00 Symposium 1
How to progress with CFTR modulators?
K2-K3
08:30 – 10:00 Symposium 2
CF respiratory microbiome
E1-E3
08:30 – 10:00 Symposium 3
Managing bleeding and thrombosis in CF
K1
08:30 – 10:00 Symposium 4
New psychological approaches in CF
E4
08:30 – 10:00 Symposium 5
Let‘s move - Exercise related guidelines in CF
H2
08:30 – 10:00 Symposium 6
FEV1 and nutritional status: Chicken or the egg?
H1
10:00 – 10:30 COFFEE BREAK
Exhibition
10:30 – 12:00 Symposium 7
Prevention and control of infection
K2-K3
10:30 – 12:00 Symposium 8
Transplantation: Challenges and hopes
E1-E3
10:30 – 12:00 Symposium 9
Inflammation and tissue damage
K1
10:30 – 12:00 Symposium 10
CF 2014 - New diagnostic challenges
E4
* closed meeting
** extra registration required
13
14
TITLE SCHEDULE AT A GLANCE 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
10:30 – 12:00 Symposium 11
Stem cell based airway modeling and regenerative medicine for Cystic Fibrosis
H2
10:30 – 12:00 Symposium 12
Nutritional challenges in adults
H1
12:00 – 14:00 LUNCH BREAK 12:00 – 15:00 Posters
Poster Viewing
Poster Area
12:30 – 14:00 Satellite Symposium Changing faces, changing solutions
K2-K3
12:30 – 14:00 Satellite Symposium CFTR modulation in Cystic Fibrosis
E1-E3
12:30 – 14:30 Meeting
CF Pharmacists Meeting
H2
12:30 – 13:30 Meeting
ECFS Patient Registry Data Quality Project Group Meeting
H1
13:00 – 14:00 Meeting
UK Newborn Screening Special Interest Group Meeting*
K1
13:30 – 14:30 Meeting
ECFS Patient Registry Contributors Meeting
H1
14:00 – 16:00 Meeting
UK Cystic Fibrosis Trust Meeting*
14:00 – 15:00 Posters
ePoster Sessions
14:00 – 15:00 Posters
Guided Poster Tours
15:00 – 16:30 Workshop 1
Fixing basic defects - New therapies
K2-K3
15:00 – 16:30 Workshop 2
Interactions between microbes and the host
E1-E3
15:00 – 16:30 Workshop 3
Exploring new endpoints in clinical trials
K1
15:00 – 16:30 Workshop 4
Using registries to identify new challenges
E4
15:00 – 16:30 Workshop 5
Improving quality and the cost implications
H2
15:00 – 16:30 Workshop 6
CFRD and other metabolic complications
H1
R5+6 ePoster Corners Poster Area
16:30 – 17:00 COFFEE BREAK
Exhibition
17:00 – 18:30 Workshop 7
New antibacterial therapies
K2-K3
17:00 – 18:30 Workshop 8
Transplantation and extracorporeal life support
E1-E3
17:00 – 18:30 Workshop 9
Integrating exercise
K1
17:00 – 18:30 Workshop 10
Adherence
E4
17:00 – 18:30 Workshop 11
Screening and diagnosis
H2
17:00 – 18:30 Workshop 12
Mucus and mucins in CF
H1
18:00 – 19:30 Meeting
ECFS Patient Registry Training Meeting*
18:30 – 20:30 Meeting
ECFS CTN Standardisation Committee Meeting*
R5+6 E4
19:00 – 20:30 Satellite Symposium Cystic Fibrosis treatment - Past, present and future
E1-E3
FRIDAY, JUNE 13 07:15 – 08:15
Round Tables
Congress Hall Foyer
07:15 – 08:15 Meet the Experts
How to get the most out of the CFTR-2 resource
ePoster Corner A
07:15 – 08:15 Meet the Experts
Diagnosis and management of emerging infections
ePoster Corner B
08:30 – 10:00 Symposium 13
Standards of care
K2-K3
08:30 – 10:00 Symposium 14
Interactive case studies
E1-E3
08:30 – 10:00 Symposium 15
Caring in a changing world of CF
K1
08:30 – 10:00 Symposium 16
Multidisciplinary aspects of CFRD
E4
08:30 – 10:00 Symposium 17
Why physiotherapists should get excited about upper airways
H2
08:30 – 10:00 Symposium 18
CFTR - A multidimensional view
H1
10:00 – 10:30 COFFEE BREAK
Exhibition
10:30 – 12:00 Symposium 19
Airway controversies
K2-K3
10:30 – 12:00 Symposium 20
The therapeutic pipeline 2014
E1-E3
10:30 – 12:00 Symposium 21
Nursing in CF - An evolving role * closed meeting
K1 ** extra registration required
SCHEDULE AT A GLANCE TITLE 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
10:30 – 12:00 Symposium 22
CF infections under low oxygen conditions
E4
10:30 – 12:00 Symposium 23
We have screened the baby, now what?
H2
10:30 – 12:00 Symposium 24
Mucus, mucins, and epithelial responses against bugs
H1
11:00 – 14:00 Meeting
CF Ageing Group Meeting
R4
12:00 – 14:00 LUNCH BREAK 12:00 – 15:00 Posters
Poster Viewing
Poster Area
12:30 – 14:00 Satellite Symposium Changing antibiotic delivery to improve CF patients’ lives: Challenges and opportunities
K2-K3
12:30 – 14:00 Satellite Symposium New approaches to pancreatic enzyme replacement therapy
E1-E3
12:30 – 14:30 Meeting
ECFS Diagnostic Network Working Group Meeting
H1
12:30 – 14:30 Meeting
ECFS Gene Modifier Working Group Meeting
H2
12:30 – 14:30 Meeting
ECFS Patient Registry Steering Group Meeting*
K1
13:00 – 14:30 Meeting
ECFS CTN Microbiology Group Meeting*
E4
14:00 – 15:00 Posters
ePoster Sessions
14:00 – 15:00 Posters
Guided Poster Tours
ePoster Corners Poster Area
15:00 – 16:30 Special Symposium AHP research symposium: Changing clinical practice through research with minimal or no funding
K2-K3
15:00 – 16:30 Workshop 13
New insights in lung disease
E1-E3
15:00 – 16:30 Workshop 14
Measurements in physiotherapy
K1
15:00 – 16:30 Workshop 15
Mucus, vitamins and the pancreas
E4
15:00 – 16:30 Workshop 16
New development of microbial diagnostics
H2
15:00 – 16:30 Workshop 17
Epithelial cell biology
H1
16:30 – 17:00 COFFEE BREAK
Exhibition
17:00 – 18:30 Workshop 18
Late breaking science
K2-K3
17:00 – 18:30 Workshop 19
Host responses to infection
E1-E3
17:00 – 18:30 Workshop 20
Indexing the lung
K1
17:00 – 18:30 Workshop 21
Microbial diversity in the lung
E4
17:00 – 18:30 Workshop 22
Psychosocial/Nursing complex case presentations
H2
17:00 – 18:30 Workshop 23
CFTR genetics and function
H1
18:00 – 19:30 Meeting
ECFS Patient Registry Training Meeting*
R4
SATURDAY, JUNE 14 09:00 – 10:30 Symposium 25
Clinical trials in preschool children
K2-K3
09:00 – 10:30 Symposium 26
Inflammation, infection and macrolides
E1-E3
09:00 – 10:30 Symposium 27
Depression and anxiety in CF: Proposals from the CFF-ECFS Guidelines Committee
K1
09:00 – 10:30 Symposium 28
Functional intestinal outcomes measures in CF in the era of correctors and potentiators
E4
09:00 – 10:30 Symposium 29
Pharmacovigilance/Phase 4 studies using registries
H2
09:00 – 10:30 Symposium 30
The “epithelial“ channelome and options for rescue
H1
10:30 – 11:00 COFFEE BREAK
Exhibition
11:00 – 12:30
Closing Plenary
K2-K3
12:30 – 13:00
Closing Ceremony
K2-K3
13:30 – 18:00 Meeting
ECFS Scientific Committee Meeting - Brussels 2015*
* closed meeting
J2
** extra registration required
15
16
WEDNESDAY, JUNE 11 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
K2-K3
Exhibition & Poster Area
K1
Other
07:30 08:00 08:30
MEETINGS
08:30 – 18:30
09:00 09:30
CFE/ECFS JOINT SYMPOSIUM 09:30 – 12:30 CFE/ECFS Joint Symposium Access to Personalised Medicine
10:00 10:30 11:00 11:30 12:00 12:30 13:00 13:30 14:00 14:30 15:00 15:30 16:00
POSTER HANGING
16:30 17:00 ECFS ANNUAL GENERAL MEETING
17:00 – 18:00
17:30 18:00 18:30
OPENING PLENARY
19:00 18:30 – 20:00 19:30
EXHIBITION & POSTER AREA OPEN
20:00
WELCOME RECEPTION 20:30 21:00 21:30
20:00 – 21:30
37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
17:00 – 18:00 ECFS Annual General Meeting*
Room K1
*closed meeting (ECFS Members only)
18:30 – 20:00 Opening Plenary
Room K2-K3
In the presence of: Stuart Elborn, ECFS President Anders Lindblad, Conference President Gunnar C. Hansson, Conference Vice President Johan Moström, Chairman of the Cystic Fibrosis Association of Sweden The Opening Ceremony will include: ► Welcome Address ► Presentation of the ECFS Award ► Presentation of the ECFS Lifetime Contribution Award ► Presentation of the ECFS Young Investigators Awards The ECFS Award is awarded to honour a person or persons who have made a significant contribution to our understanding of Cystic Fibrosis or to the treatment or care of patients with Cystic Fibrosis. The award, previously named the Rossi Medal, is now called the European Cystic Fibrosis Society Award at the request of Prof. Rossi’s family. This year the award goes to Prof. Eitan Kerem M.D. (IL). In previous years the award has been presented to the following: 2002 2003 2004 2005 2006 2007 2008 2009 2010 2011 2012 2013
Prof. Margaret Hodson (UK) Prof. Gianni Mastella (IT) Dr. James Littlewood (UK) Prof. John Govan (UK) Dr. Christian Koch (DK) Dr. Edith Puchelle (FR) Prof. Véra Vávrová (CZ) Prof. Francis Collins (US), Prof. Batsheva Kerem (IL), Prof. John Riordan (US), Prof. Lap-Chee Tsui (HK) Prof. Margarida D. Amaral (PT), Dr. David N. Sheppard (UK) Prof. Gerd Döring (DE) Prof. Niels Høiby (DK) Prof. Kevin Webb (UK)
The ECFS Lifetime Contribution Award is to recognize individuals who have made significant contributions to the Cystic Fibrosis community, nationally and internationally, over a sustained period of time through scholarly endeavours, teaching excellence or innovation to the benefit of Cystic Fibrosis care or research. This year the ECFS Lifetime Contribution Award celebrates the achievements of Prof. Birgitta Strandvik, M.D., PhD. (SE). The ECFS Young Investigators Awards recognize outstanding and promising work by investigators under the age of 35 who submitted an abstract to the ECFS Conference. 20:00 – 21:30 Welcome Reception
The Welcome Reception is free of charge to all registered participants.
Exhibition Area
17
WEDNESDAY JUNE 11
WEDNESDAY, JUNE 11
18
THURSDAY, JUNE 12 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
K2-K3
E1-E3
K1
E4
07:00 07:30 08:00 08:30
SYMPOSIUM 1
09:00 08:30 – 10:00
SYMPOSIUM 2 08:30 – 10:00
How to progress with CFTR CF respiratory microbiome
09:30 modulators?
SYMPOSIUM 3
SYMPOSIUM 4
Managing bleeding and thrombosis in CF
New psychological approaches in CF
08:30 – 10:00
08:30 – 10:00
10:00
COFFEE BREAK IN EXHIBITION
10:30
SYMPOSIUM 7
SYMPOSIUM 8
SYMPOSIUM 9
SYMPOSIUM 10
Prevention and control of
Transplantation: Challenges and hopes
Inflammation and tissue damage
CF 2014 - New diagnostic challenges
SATELLITE SYMPOSIUM
SATELLITE SYMPOSIUM
11:00 10:30 – 12:00 11:30 infection
10:30 – 12:00
10:30 – 12:00
10:30 – 12:00
12:00 12:30 13:00 12:30 – 14:00
Changing faces, changing 13:30 solutions
12:30 – 14:00
LUNCH BREAK
CFTR modulation in Cystic Fibrosis
14:00 14:30 15:00
WORKSHOP 1
WORKSHOP 2
WORKSHOP 3
Fixing basic defects -
Interactions between microbes and the host
Exploring new endpoints in Using registries to identify clinical trials new challenges
15:30 15:00 – 16:30
16:00 New therapies
15:00 – 16:30
15:00 – 16:30
WORKSHOP 4 15:00 – 16:30
16:30
COFFEE BREAK IN EXHIBITION
17:00
WORKSHOP 7
17:30 17:00 – 18:30 18:00
WORKSHOP 8 17:00 – 18:30
19:00
SATELLITE SYMPOSIUM 19:30
19:00 – 20:30
20:00
Cystic Fibrosis treatment Past, present and future
21:00
17:00 – 18:30
New antibacterial therapies Transplantation and Integrating exercise extracorporeal life support
18:30
20:30
WORKSHOP 9
WORKSHOP 10 17:00 – 18:30
Adherence
THURSDAY, JUNE 12
19
37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
H2
H1
Congress Hall Foyer
Exhibition & Poster Area
07:00
ROUND TABLES 07:15 – 08:15
MEET THE EXPERTS 07:15 – 08:15
08:00 08:30
SYMPOSIUM 5
SYMPOSIUM 6
Let‘s move - Exercise
FEV 1 and nutritional status: Chicken or the egg?
09:00 08:30 – 10:00
08:30 – 10:00
09:30 related guidelines in CF 10:00
COFFEE BREAK IN EXHIBITION
10:30
SYMPOSIUM 11
SYMPOSIUM 12
Stem cell based airway
Nutritional challenges in adults
11:00 10:30 – 12:00
10:30 – 12:00
11:30 modeling and regenerative
medicine for Cystic Fibrosis
EXHIBITION & POSTERS
12:00 12:30 13:00
LUNCH BREAK
13:30 14:00 14:30
GUIDED POSTER TOURS & ePOSTER SESSIONS
15:00
WORKSHOP 5
WORKSHOP 6
Improving quality and the
CFRD and other metabolic complications
15:30 15:00 – 16:30
16:00 cost implications
15:00 – 16:30
16:30
EXHIBITION & POSTERS COFFEE BREAK IN EXHIBITION
17:00
WORKSHOP 11
WORKSHOP 12
Screening and diagnosis
Mucus and mucins in CF
17:30 17:00 – 18:30 18:00 18:30 19:00 19:30 20:00 20:30 21:00
17:00 – 18:30
THURSDAY JUNE 12
07:30
20
THURSDAY, JUNE 12 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
1 Immunology/Pulmonology/Inflammation Atypical CF - Investigation and management including labels Kris De Boeck, Leuven, BE
7 Nursing/Psychosocial Issues Late diagnosis Kristine Colpaert, Leuven, BE
2
8 Physiotherapy Managing the intubated CF patient Louise Lannefors, Copenhagen, DK
Gastrointestinal/Nutrition/Liver Disease/Metabolic Complications of CF Enteral tube feeding and impact Susannah King, Melbourne, AU
9 Microbiology/Antibiotics Sinus infection and the CF lung Helle Krogh Johansen, Copenhagen, DK
3 Immunology/Pulmonology/Inflammation Eradication of bacteria in adults with CF Harry Heijerman, The Hague, NL 4 Immunology/Pulmonology/Inflammation Fungal infection in CF Andrew Jones, Manchester, UK
10 Physiotherapy Supplemental oxygen on exertion: The practicalities and recommendations Tracey Daniels, York, UK
5 Genetics/Screening/Diagnosis How to communicate newborn screening results to families Mandy Bryon, London, UK
11 Epidemiology/Registry Survival analyses using registries Virginie Scotet, Brest, FR
6 Microbiology/Antibiotics Infection control - Multiple pathogens and multiple practical issues Kirsten Schaffer, Dublin, IE
Congress Hall Foyer Down to Exhibition and Session Rooms E1-E3, E4
2
1
4
3
6
5
Buffet
s able T nd Rou
Buffet
THURSDAY JUNE 12
07:15 – 08:15 ROUND TABLES - CONGRESS HALL FOYER - LEVEL 1
10 8
11 9
7
THURSDAY, JUNE 12
21
37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
07:15 – 08:15
Meet the Experts - Multiple breath washout
Experts: Per Gustafsson, Skövde, SE / Alexander Horsley, Manchester, UK
07:15 – 08:15
Meet the Experts - Mucus, mucins, and Cystic Fibrosis
Experts: Gunnar C. Hansson, Gothenburg, SE / Richard Boucher, Chapel Hill, US
07:15 – 08:15
Meet the Experts - Family therapy approaches with our families
Expert: Araxie Matossian, Brussels, BE
08:30 – 10:00
ePoster Corner B
ePoster Corner C
Symposium 1 - How to progress with CFTR modulators?
Moderators: Eitan Kerem, Jerusalem, IL / Marcus Mall, Heidelberg, DE
08:30 – 08:52
The way forward in drug testing is mutation specific – Katja Conrath, Mechelen, BE
08:52 – 09:14
The way forward in drug testing is phenotype specific – Isabelle Sermet, Paris, FR
09:14 – 09:36
Design based vs high throughput screening – Luis Galietta, Genoa, IT
09:36 – 10:00
Topical vs systemic modulators – Jane Davies, London, UK
08:30 – 10:00
Symposium 2 - CF respiratory microbiome
Moderators: Thomas Bjarnsholt, Copenhagen, DK / Eshwar Mahenthiralingam, Cardiff, UK
K2-K3
E1-E3
08:30 – 08:52
How should we investigate the microbiota of the CF lung? – Julian Marchesi, Cardiff, UK
08:52 – 09:14
The CF respiratory microbiota - What we have learnt so far? – Michael G. Surette, Hamilton, CA
09:14 – 09:36
What can we learn from microbiome studies in other respiratory diseases? – Kenneth Bruce, London, UK
09:36 – 10:00
What can we learn from microbiome studies in other organs? – Petia Kovatcheva, Gothenburg, SE
08:30 – 10:00
Symposium 3 - Managing bleeding and thrombosis in CF
Moderators: Marita Gilljam, Gothenburg, SE / Nicholas Simmonds, London, UK
08:30 – 08:52
Choice, complications and management of venous access devices – Simon Padley, London, UK
08:52 – 09:14
Coagulation update (hypercoagulable and bleeding states) – Fariba Baghaei, Gothenburg, SE
09:14 – 09:36
Haemoptysis & treatment – Elisabeth Tullis, Toronto, CA
09:36 – 10:00
Haematological & vascular issues - Implications for transplantation – Goran Dellgren, Gothenburg, SE
08:30 – 10:00
Symposium 4 - New psychological approaches in CF
Moderators: Gary Latchford, Leeds, UK / Kristin Riekert, Baltimore, US 08:30 – 09:00 Active ingredients in psychotherapy: Current thinking – Gary Latchford, Leeds, UK 09:00 – 09:30 The BALANCE trial: Motivational interviewing for adolescents with CF – Kristin Riekert, Baltimore, US 09:30 – 10:00
Psychological interventions with children and families: Current evidence – Alistair Duff, Leeds, UK
K1
E4
THURSDAY JUNE 12
ePoster Corner A
22
THURSDAY, JUNE 12 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
08:30 – 10:00
Symposium 5 - Let’s move - Exercise related guidelines in CF
THURSDAY JUNE 12
Moderators: Helge Hebestreit, Würzburg, DE / Ruth Dentice, Sydney, AU
H2
08:30 – 08:52
Position stand on physical activity assessment in CF – Don Urquhart, Edinburgh, UK
08:52 – 09:14
Clinical practice guideline on exercise testing in CF – Helge Hebestreit, Würzburg, DE
09:14 – 09:36
Clinical practice guideline on exercise counselling / prescription in CF – Anne Swisher, Morgantown, US
09:36 – 10:00
The Swedish approach to exercise – Margareta Sahlberg, Gothenburg, SE
08:30 – 10:00
Symposium 6 - FEV1 and nutritional status: Chicken or the egg?
Moderators: Anders Lindblad, Gothenburg, SE / Vincent Gulmans, Baarn, NL 08:30 – 08:52
Longitudinal data on FEV 1 – Laura Viviani, Milan, IT
08:52 – 09:10
Nutritional status: Should we aim for the 50th percentile? PRO – Marci Sontag, Aurora, US
09:10 – 09:28
Nutritional status: Should we aim for the 50th percentile? CON – Willie Woestenenk, Utrecht, NL
H1
09:28 – 09:36 Discussion – Anders Lindblad, Gothenburg, SE
09:36 – 10:00
Influence of early stage CFRD on lung function and nutritional status – Renske van der Meer, The Hague, NL
10:00 – 10:30
COFFEE BREAK
10:30 – 12:00
Symposium 7 - Prevention and control of infection
Moderators: Ferenc Karpati, Stockholm, SE / Stephen Holden, Nottingham, UK
10:30 – 10:52
The design of health care facilities to reduce the spread of infection – Lisa Saiman, New York, US
10:52 – 11:14
Guidance on preventing infection in everyday life – Kirsten Schaffer, Dublin, IE
11:14 – 11:36
Prevention of infection with P. aeruginosa: Lessons from epidemiology and genomics – Burkhard Tümmler, Hannover, DE
11:36 – 12:00
M. abscessus - Preventing cross infection – Dorothy M. Grogono, Cambridge, UK
10:30 – 12:00
Symposium 8 - Transplantation: Challenges and hopes
Moderators: Scott Bell, Brisbane, AU / Lennart Hansson, Lund, SE
K2-K3
E1-E3
10:30 – 10:52
Challenges of paediatric transplantation – Christian Benden, Zurich, CH
10:52 – 11:14
How to get the best transplant outcome: Recipient and donor issues in 2014 – Lieven Dupont, Leuven, BE
11:14 – 11:36
Extra-pulmonary transplantation: Liver, pancreas, kidney – Christiane Knoop, Brussels, BE
11:36 – 12:00
Pregnancy and transplantation - The issues – Peadar Noone, Chapel Hill, US
10:30 – 12:00
Symposium 9 - Inflammation and tissue damage
Moderators: Dominik Hartl, Tübingen, DE / Jay K. Kolls, Pittsburgh, US
K1
10:30 – 10:52
The IL-17 axis in CF lung disease – Jay K. Kolls, Pittsburgh, US
10:52 – 11:14
IDO/Kynurenine: A novel anti-inflammatory Th17 counteracting pathway in CF – Luigina Romani, Perugia, IT
THURSDAY, JUNE 12
23
37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
MicroRNAs in CF lung disease – Catherine Greene, Dublin, IE
11:36 – 12:00
CFTR and inflammation: Partners or enemies? – Jean-Michel Sallenave, Paris, FR
10:30 – 12:00
Symposium 10 - CF 2014 - New diagnostic challenges
Moderators: Nico Derichs, Berlin, DE / Inez Bronsveld, Utrecht, NL
10:30 – 10:52
CFTR2: Defining the most common CF-causing mutations – Patrick Sosnay, Baltimore, US
10:52 – 11:14
CFTR3: Personalised characterization of rare CF genotypes – Nico Derichs, Berlin, DE
11:14 – 11:36
Experience of using the term CRMS in the US – Clement L. Ren, Rochester, US
11:36 – 12:00
CF Diagnostic Guidelines: How to classify intermediate phenotypes? – Anne Munck, Paris, FR
10:30 – 12:00
Symposium 11 - Stem cell based airway modeling and regenerative medicine for Cystic Fibrosis
Moderators: Bob Scholte, Rotterdam, NL / Ulrich Martin, Hannover, DE
E4
H2
10:30 – 10:52
Lung injury and repair in CF, prospects for cell therapy and regenerative medicine – Bob Scholte, Rotterdam, NL
10:52 – 11:14
Generation of lung epithelium from pluripotent stem cells – Amy P. Wong, Toronto, CA
11:14 – 11:36
Growing stem cell based lung organoids – Robert Vries, Utrecht, NL
11:36 – 12:00
Stem cell therapy for Cystic Fibrosis – Ulrich Martin, Hannover, DE
10:30 – 12:00
Symposium 12 - Nutritional challenges in adults
Moderators: Helen White, Leeds, UK / Drucy Borowitz, Buffalo, US
H1
10:30 – 10:52
Prevention and treatment of obesity in Cystic Fibrosis - Is it time to revise our guidelines? – Susannah King, Melbourne, AU
10:52 – 11:14
Pregnancy outcomes for the mother and child - The nutritional challenges faced in Cystic Fibrosis – Christine Etherington, Leeds, UK
11:14 – 11:36
Nutritional challenges pre- and post-transplantation – Francis Hollander, Utrecht, NL
11:36 – 12:00
Post ivacaftor – Drucy Borowitz, Buffalo, US
12:00 – 14:00
LUNCH BREAK
12:30 – 14:00
Satellite Symposium - Changing faces, changing solutions
K2-K3
See detailed programme page 72 12:30 – 14:00
Satellite Symposium - CFTR modulation in Cystic Fibrosis
See detailed programme page 72
E1-E3
THURSDAY JUNE 12
11:14 – 11:36
24
THURSDAY, JUNE 12
THURSDAY JUNE 12
37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
14:00 – 15:00
Posters
14:00 – 15:00
ePoster Sessions: Screening different countries Microbiology - Antibiotics Putting the patient first
ePoster Corner A ePoster Corner B ePoster Corner C
14:00 – 15:00 Guided Poster Tours in the following categories: Microbiology Gastrointestinal/Liver Disease/Metabolic Complications of CF/Nutrition 12:00 – 15:00
Poster Viewing: Poster presenters in the following categories are asked to be available at their posters for discussion: • Genetics • CFTR/Cell Biology/Cell Physiology • New Therapies • Microbiology • Gastrointestinal/Metabolic Complications of CF/Nutrition/Growth • Delivery of Care See detailed programme page 48 and 49
15:00 – 16:30
Workshop 1 - Fixing basic defects - New therapies
Moderators: Isabelle Fajac, Paris, FR / Batsheva Kerem, Jerusalem, IL
K2-K3
15:00 – 15:15
WS1.1
The effect of ivacaftor, a CFTR potentiator, in patients with Cystic Fibrosis and a non-G551DCFTR gating mutation, the KONNECTION study – Kris De Boeck, Leuven, BE
15:15 – 15:30
WS1.2
Vardenafil promotes relocalization of F508del-CFTR in human and mouse airways – Barbara Dhooghe, Brussels, BE
15:30 – 15:45
WS1.3
Enhanced correction of F508del CFTR using drug-like small molecules in combination with correctors and potentiators – Markus Haeberlein, Cambridge, US
15:45 – 16:00
WS1.4
A rAAV2/5 based gene therapy model for Cystic Fibrosis airway disease – Marianne Carlon, Leuven, BE
16:00 – 16:15
WS1.5
Restoration of the CFTR function by antisense oligonucleotide splicing modulation – Batsheva Kerem, Jerusalem, IL
16:15 – 16:30
WS1.6
OligoG normalizes the CF mucus phenotype – Anna Ermund, Gothenburg, SE
15:00 – 16:30
Workshop 2 - Interactions between microbes and the host
Moderators: Michael Fayon, Bordeaux, FR / Rebecca Ingram, Belfast, UK
E1-E3
15:00 – 15:10
WS2.1
Liposomal clarithromycin effect on bacterial adhesion to epithelia of Cystic Fibrosis patients – Abdelwahab Omri, Sudbury, CA
15:10 – 15:20
WS2.2
Identification of two novel immunogenic Burkholderia cepacia complex proteins involved in lung cell attachment – Ruth Dennehy, Dublin, IE
15:20 – 15:30
WS2.3
Multifaceted iron acquisition mechanisms of Burkholderia cenocepacia – Niamh Whelan, Dublin, IE
15:30 – 15:40 WS2.4 Pseudomonas aeruginosa capability to colonize the CF lung may be favored by its remarkable ability to recruit zinc under conditions of limited metal availability – Andrea Battistoni, Rome, IT 15:40 – 15:50
WS2.5
Physiological levels of nitrate support anaerobic growth of Pseudomonas aeruginosa at growth rates reported in Cystic Fibrosis sputum – Laura Line, Copenhagen, DK
15:50 – 16:00
WS2.6
Novel murine model for persistence, migration and adaptation of Pseudomonas aeruginosa in the respiratory tract – Joanne Fothergill, Liverpool, UK
THURSDAY, JUNE 12
25
37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
WS2.7
Use of the Galleria mellonella in vivo model to determine the virulence of bacteria colonising the CF airways – Christopher Spence, Belfast, UK
16:10 – 16:20
WS2.8
Genotypic and transcriptomic characterization of a small colony variant of Pseudomonas aeruginosa and its parent strain isolated in a murine model of chronic bacterial colonization – Sharon Irvine, Glasgow, UK
16:20 – 16:30
WS2.9
Inflammatory potential and antimicrobial susceptibility of bacteria in the cultivatable CF lung microbiome – Michael Tunney, Belfast, UK
15:00 – 16:30
Workshop 3 - Exploring new endpoints in clinical trials
Moderators: Philippe Reix, Lyon, FR / Felix Ratjen, Toronto, CA
K1
15:00 – 15:15
WS3.1
The effect of ivacaftor on the rate of lung function decline in CF patients with a G551D-CFTR mutation – Edward McKone, Dublin, IE
15:15 – 15:30
WS3.2
The effect of ivacaftor treatment on lung ventilation defects, as measured by hyperpolarized helium-3 MRI, on patients with Cystic Fibrosis and a G551D-CFTR mutation – Talissa Altes, Charlottesville, US
15:30 – 15:45
WS3.3
MRI-based pulmonary blood flow and lung function in CF patients - Flow changes with pulmonary decline – John Clancy, Cincinnati, US
15:45 – 16:00
WS3.4
Lack of correlation between sputum P. aeruginosa density and FEV 1 changes among CF patients treated with inhaled antibiotics – Donald Vandevanter, Cleveland, US
16:00 – 16:15
WS3.5
Inhaled 7% hypertonic saline treatment in preschool children with Cystic Fibrosis – Silvia Palacio, Buenos Aires, AR
16:15 – 16:30
WS3.6
The effect of inhaled dry powder mannitol (IDPM) on ventilation inhomogeneity (VI) in adults with Cystic Fibrosis – Krystyna Poplawska, Mainz, DE
15:00 – 16:30
Workshop 4 - Using registries to identify new challenges
Moderators: Hanne Olesen, Aarhus, DK / Vincent Gulmans, Baarn, NL
THURSDAY JUNE 12
16:00 – 16:10
E4
15:00 – 15:15
WS4.1
Treatment burden in patients with CF and at least one class 4 or 5 mutation – Jonas Dewulf, Leuven, BE
15:15 – 15:30
WS4.2
How different is the cohort of young CF children included in national registries of countries with and without newborn screening? – Muriel Thomas, Brussels, BE
15:30 – 15:45
WS4.3
Modeling airway infections in Cystic Fibrosis – Ann Granchelli, Salt Lake City, US
15:45 – 16:00
WS4.4
Multicenter prevalence study of nontuberculous mycobacteria in patients with Cystic Fibrosis in Scandinavia – Tavs Qvist, Copenhagen, DK
16:00 – 16:15
WS4.5
Cystic Fibrosis mutations and survival – Theodore Liou, Salt Lake City, US
16:15 – 16:30
WS4.6
Cancer in patients with Cystic Fibrosis – Carsten Schwarz, Berlin, DE
15:00 – 16:30
Workshop 5 - Improving quality and the cost implications
Moderators: Vibsen Bregnballe, Aarhus, DK / Inge Heeres, Rotterdam, NL 15:00 – 15:15
WS5.1
Improving the annual review process - The rainbow project – Jasneek Chawla, Brisbane, AU
15:15 – 15:30
WS5.2
Travelling abroad with Cystic Fibrosis (CF): Current practice and problems – Sarah Lenaghan, Newcastle Upon Tyne, UK
H2
26
THURSDAY, JUNE 12
THURSDAY JUNE 12
37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
15:30 – 15:45
WS5.3
Improving care for adults with end stage Cystic Fibrosis at Bristol Adult CF Centre (BACFC) – Charlotte Addy, Bristol, UK
15:45 – 16:00
WS5.4
Can registry data be used to examine Cystic Fibrosis (CF) health service utilisation? Perspectives of the Irish CF Registry – Abi Jackson, Dublin, IE
16:00 – 16:15
WS5.5
Economic burden of Cystic Fibrosis (CF): Prevalence based cost of illness analysis related to the lung disease severity in Czech CF patients – Milan Macek, Prague, CZ
16:15 – 16:30
WS5.6
Cost-effectiveness of performing positive expiratory pressure versus high frequency chest wall oscillation – Maggie McIlwaine, Vancouver, CA
15:00 – 16:30
Workshop 6 - CFRD and other metabolic complications
Moderators: Lena Hjelte, Stockholm, SE / Hila Elyashar-Earon, Jerusalem, IL
H1
15:00 – 15:10
WS6.1
Longitudinal associations between FEV 1 and HbA1c in a UK cohort of young people with Cystic Fibrosis – Donna McShane, Cambridge, UK
15:10 – 15:20
WS6.2
Glucose tolerance in pediatric patients with Cystic Fibrosis – Laura Zazzeron, Milan, IT
15:20 – 15:30
WS6.3
Subtle defects in glucose metabolism prior to development of diabetes in patients with Cystic Fibrosis – Limor Marko, Jerusalem, IL
15:30 – 15:40
WS6.4
A placebo-controlled trial of insulin therapy with or without adjuvant metformin in patients with Cystic Fibrosis-Related Diabetes (CFRD) – Johanna de Lind van Wijngaarden-van den Berg, The Hague, NL
15:40 – 15:50
WS6.5
Body mass index, carbohydrate intake and insulin dosage per carbohydrate unit in 131 female and 77 male patients with Cystic Fibrosis-Related Diabetes – Nicole Scheuing, Ulm, DE
15:50 – 16:00
WS6.6
Continuous glucose monitoring is a useful tool for diagnosis of Cystic Fibrosis-Related Diabetes – Anne-Marie Ebdon, London, UK
16:00 – 16:10
WS6.7
Cystic Fibrosis and bone disease: Defective osteoblast maturation with the F508del mutation in CFTR – Jacky Jacquot, Reims, FR
16:10 – 16:20
WS6.8
Acute kidney injury afflicts a significant proportion of adult patients with Cystic Fibrosis, with an incidence in excess of that of the general population which clusters with specific risk factors – Stephanie English, Cork, IE
16:20 – 16:30
WS6.9
Cystic Fibrosis-Related Diabetes (CFRD): Evidence for a role of miR-155, miR-370 and miR-708 – Maria Street, Parma, IT
16:30 – 17:00
COFFEE BREAK
17:00 – 18:30
Workshop 7 - New antibacterial therapies
Moderators: Patrick Flume, Charleston, US / Tim Lee, Leeds, UK
K2-K3
17:00 – 17:15
WS7.1
Pre-clinical evaluation of novel antibiotic POL7001 against Pseudomonas aeruginosa in lung infection models – Alessandra Bragonzi, Milan, IT
17:15 – 17:30
WS7.2
Antibacterial activity of theta defensin, (RTD-1), against clinical isolates of Pseudomonas aeruginosa – Paul Beringer, Los Angeles, US
17:30 – 17:45
WS7.3
Once-daily liposomal amikacin for inhalation is noninferior to twice-daily tobramycin inhalation solution in improving pulmonary function in Cystic Fibrosis patients with chronic infection due to Pseudomonas aeruginosa – Diana Bilton, London, UK
THURSDAY, JUNE 12
27
37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
WS7.4
Inhaled aztreonam lysine (Cayston) therapy significantly improves lung function, weight, hospitalisations and excerbation rates prospectively - An Irish and UK real world experience – Barry Plant, Cork, IE
18:00 – 18:15
WS7.5
Prolonged improvement in lung function and quality of life in Cystic Fibrosis: A 24-week extension study of levofloxacin nebulization solution (APT-1026) versus tobramycin nebulization solution in stable CF patients with chronic P. aeruginosa infection – Stuart Elborn, Belfast, UK
18:15 – 18:30
WS7.6
Incorporation of a third inhaled antipseudomonal antibiotic class into the management of patients at an adult CF care center – Donald Vandevanter, Cleveland, US
17:00 – 18:30
Workshop 8 - Transplantation and extracorporeal life support
Moderators: Christian Benden, Zurich, CH / Goran Dellgren, Gothenburg, SE
17:00 – 17:15
WS8.1
Lung transplant referrals and outcomes: Experience of a regional adult CF centre 2008-2013 – Christine Etherington, Leeds, UK
17:15 – 17:30
WS8.2
Pulmonary arterial hypertension in adult CF lung transplantation candidates – Libor Fila, Prague, CZ
17:30 – 17:45
WS8.3
Extracorporeal life support as a bridge to lung transplantation: Outcome in Cystic Fibrosis recipients – Urs Bürgi, Zurich, CH
17:45 – 18:00
WS8.4 Extracorporeal life support in Cystic Fibrosis: A single centre experience – Dominic Keating, Melbourne, AU
18:00 – 18:15
WS8.5
Outcome of lung transplantation for Cystic Fibrosis in Sweden – Marita Gilljam, Gothenburg, SE
18:15 – 18:30
WS8.6
Bacterial flora in the airways after lung transplantation in patients with Cystic Fibrosis – Lennart Hansson, Lund, SE
17:00 – 18:30
Workshop 9 - Integrating exercise
Moderators: Don Urquhart, Edinburgh, UK / Helge Hebestreit, Würzburg, DE
E1-E3
K1
17:00 – 17:15
WS9.1
Can lung function predict exercise capacity in patients with CF? – Iben Jensen, Aarhus, DK
17:15 – 17:30
WS9.2
Modified shuttle test performance in CF children and adolescents with different severity of lung disease – Clizia Cazzarolli, Verona, IT
17:30 – 17:45
WS9.3
Reduced physical activity participation is associated with increased need for hospitalisation in adults with Cystic Fibrosis – Narelle Cox, Melbourne, AU
17:45 – 18:00
WS9.4
Exercise in hospital - Let’s work it out – Fiona Shaw, London, UK
18:00 – 18:15
WS9.5
The role of daily physical activity on exercise performance in adults with Cystic Fibrosis – Daniela Savi, Rome, IT
18:15 – 18:30
WS9.6
The All Wales Adult Cystic Fibrosis Centre (AWACFC) Virtual Instruction of Exercise With Technology to Enhance Care - VIEWTEC Programme – Steve Howard, Penarth, UK
17:00 – 18:30
Workshop 10 - Adherence
Moderators: Trudy Havermans, Leuven, BE / Keith Thompson, London, UK
E4
17:00 – 17:15
WS10.1 Can home drug audit prevent oversupply and indicate adherence? – Caroline Whitton, Plymouth, UK
17:15 – 17:30
WS10.2 Adherence to study drugs in clinical trials – Thea Pugatsch, Jerusalem, IL
17:30 – 17:45
WS10.3 Adherence with ivacaftor in Cystic Fibrosis patients with the G551D mutation – Jade Fox, Liverpool, UK
THURSDAY JUNE 12
17:45 – 18:00
28
THURSDAY, JUNE 12
THURSDAY JUNE 12
37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
17:45 – 18:00
WS10.4 Pharmacist intervention to influence beliefs about medication in an adult Cystic Fibrosis clinic – Patrick Wilson, Leicester, UK
18:00 – 18:15
WS10.5 Social support, self-esteem and treatment barriers in adolescents with CF: The LINFA study – Carla Colombo, Milan, IT
18:15 – 18:30
WS10.6 Objective predictors of self-report of adherence in adult with Cystic Fibrosis – Daniel Peckham, Leeds, UK
17:00 – 18:30
Workshop 11 - Screening and diagnosis
Moderators: Anne Munck, Paris, FR / Isabelle de Monestrol, Stockholm, SE 17:00 – 17:15
WS11.1 Development of a comprehensive workflow for the analysis of CFTR gene using next generation sequencing technology – Iveta Valaskova, Brno, CZ
17:15 – 17:30
WS11.2 Age related cut-off levels for immunoreactive trypsin (IRT) in healthy newborns in the first two months of life – Toni Torresani, Zurich, CH
17:30 – 17:45
WS11.3 Change of algorithm in the CF centers influences the amount of equivocal CF diagnoses in the newborn screening program in Switzerland – Jürg Barben, St. Gallen, CH
17:45 – 18:00
WS11.4 Uptake of cascade testing in CF families: Preliminary results from the experience of western Brittany, France – Ingrid Duguépéroux, Brest, FR
18:00 – 18:15
WS11.5 Newborn screening for Cystic Fibrosis in Norway – Emma Lundman, Oslo, NO
18:15 – 18:30
WS11.6 Interpretation of nasal potential difference (nPD) measurements in difficult cases of possible Cystic Fibrosis and the role of published equations – Rishi Pabary, London, UK
17:00 – 18:30
Workshop 12 - Mucus and mucins in CF
Moderators: Richard Boucher, Chapel Hill, US / Dave Thornton, Manchester, UK
H2
H1
17:00 – 17:15
WS12.1 Assembly of MUC2 N-terminal with relevance for mucus formation – Harriet Nilsson, Gothenburg, SE
17:15 – 17:30
WS12.2 Mucins are abnormally concentrated in CF respiratory secretions: Role in disease pathogenesis – Mehmet Kesimer, Chapel Hill, US
17:30 – 17:45
WS12.3 Detachment of mucus requires a specific proteolytic cleavage in the MUC2 mucin explaining why the Cystic Fibrosis mucus is attached to the epithelium – Andre Schütte, Gothenburg, SE
17:45 – 18:00
WS12.4 Planar cell polarity protein network, which controls ciliogenesis and cilia function, is altered in human Cystic Fibrosis bronchial epithelial cells through response to endoplasmic reticulum stress – Sabrina Noël, Brussels, BE
18:00 – 18:15
WS12.5 The mucus and its behavior in rat and pig explant tissues as models of Cystic Fibrosis – Lauren Meiss, Gothenburg, SE
18:15 – 18:30
WS12.6 CFTR is an essential component in cAMP-dependent exocytosis in human and mouse beta-cell insulin secretion – Lena Eliasson, Malmö, SE
19:00 – 20:30
Satellite Symposium - Cystic Fibrosis treatment - Past, present and future
See detailed programme page 72
E1-E3
NOTES
29
37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
THURSDAY JUNE 12
30
FRIDAY, JUNE 13 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
K2-K3
E1-E3
K1
E4
07:00 07:30 08:00 08:30
SYMPOSIUM 13
SYMPOSIUM 14
SYMPOSIUM 15
SYMPOSIUM 16
Standards of care
Interactive case studies
Caring in a changing world of CF
Multidisciplinary aspects of CFRD
09:00 08:30 – 10:00
08:30 – 10:00
09:30
08:30 – 10:00
08:30 – 10:00
10:00
COFFEE BREAK IN EXHIBITION
10:30
SYMPOSIUM 19
SYMPOSIUM 20
SYMPOSIUM 21
SYMPOSIUM 22
Airway controversies
The therapeutic pipeline 2014
Nursing in CF - An evolving role
CF infections under low oxygen conditions
SATELLITE SYMPOSIUM
SATELLITE SYMPOSIUM
11:00 10:30 – 12:00 11:30
10:30 – 12:00
10:30 – 12:00
10:30 – 12:00
12:00 12:30 13:00 12:30 – 14:00
12:30 – 14:00
LUNCH BREAK
Changing antibiotic delivery New approaches to 13:30 to improve CF patients’ lives: pancreatic enzyme Challenges and opportunities replacement therapy 14:00 14:30 15:00
SPECIAL SYMPOSIUM
15:30 15:00 – 16:30
WORKSHOP 13 15:00 – 16:30
AHP Research Symposium: New insights in lung disease through research with 16:30 minimal or no funding
16:00 Changing clinical practice
19:00 19:30 20:00 20:30 21:00
Measurements in physiotherapy
Mucus, vitamins and the pancreas
WORKSHOP 18
WORKSHOP 19
Late breaking science
Host responses to infection Indexing the lung
17:30 17:00 – 18:30
18:30
WORKSHOP 15
15:00 – 16:30
15:00 – 16:30
COFFEE BREAK IN EXHIBITION
17:00
18:00
WORKSHOP 14
17:00 – 18:30
WORKSHOP 20 17:00 – 18:30
WORKSHOP 21 17:00 – 18:30
Microbial diversity in the lung
FRIDAY, JUNE 13
31
37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
H2
H1
Congress Hall Foyer
Exhibition & Poster Area
07:00 07:30
ROUND TABLES 07:15 – 08:15
MEET THE EXPERTS 07:15 – 08:15
08:00 08:30
SYMPOSIUM 17
SYMPOSIUM 18
Why physiotherapists
CFTR A multidimensional view
09:00 08:30 – 10:00
08:30 – 10:00
09:30 should get excited about
FRIDAY JUNE 13
upper airways
10:00
COFFEE BREAK IN EXHIBITION
10:30
SYMPOSIUM 23
SYMPOSIUM 24
11:00 10:30 – 12:00
10:30 – 12:00
We have screened the baby, Mucus, mucins, and epithelial responses against bugs
11:30 now what?
EXHIBITION & POSTERS
12:00 12:30 13:00
LUNCH BREAK
13:30 14:00 14:30
GUIDED POSTER TOURS & ePOSTER SESSIONS
15:00
WORKSHOP 16
WORKSHOP 17
New development of
Epithelial cell biology
15:30 15:00 – 16:30
16:00 microbial diagnostics
15:00 – 16:30
16:30
EXHIBITION & POSTERS COFFEE BREAK IN EXHIBITION
17:00
WORKSHOP 22
17:30 17:00 – 18:30
Psychosocial/Nursing 18:00 complex case presentations 18:30 19:00 19:30 20:00 20:30 21:00
WORKSHOP 23 17:00 – 18:30
CFTR genetics and function
32
FRIDAY, JUNE 13 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
1 Clinical Trials/New Therapies ENaC an underestimated therapeutic target? Marcus Mall, Heidelberg, DE
6 Immunology/Pulmonology/Inflammation Quality improvement-based clinical interventions in CF Michael Boyle, Baltimore, US
2 Physiotherapy Exercise - The Scandinavian approach Margareta Sahlberg, Gothenburg, SE
7 Nursing/Psychosocial Issues Travelling with CF Alan Peres, London, UK
3 Physiotherapy GOR and physiotherapy interventions Brenda Button, Melbourne, AU
8 Microbiology/Antibiotics Update on infection with Achromobacter spp. in CF Niels Nørskov-Lauritsen, Aarhus, DK
4 Genetics/Screening/Diagnosis How to establish newborn screening in your country? Kevin Southern, Liverpool, UK
9 Microbiology/Antibiotics Update on Staphylococcus aureus infection in CF Damian Downey, Belfast, UK
5 Clinical Trials/New Therapies ICM measurements and organoids: Complementary or not? Nico Derichs, Berlin, DE
10 Epidemiology/Registry Which patients to enter in the CF registry - How to define? Muriel Thomas, Brussels, BE
Congress Hall Foyer Down to Exhibition and Session Rooms E1-E3, E4
2
1
4
3
6
5
Buffet
s able T nd Rou
Buffet
FRIDAY JUNE 13
07:15 – 08:15 ROUND TABLES - CONGRESS HALL FOYER - LEVEL 1
10 8 9 7
FRIDAY, JUNE 13
33
37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
07:15 – 08:15
Meet the Experts - How to get the most out of the CFTR-2 resource
Experts: Patrick Sosnay, Baltimore, US / Carlo Castellani, Verona, IT
07:15 – 08:15
Meet the Experts - Diagnosis and management of emerging infections
Experts: Miguel Cámara, Nottingham, UK / Andrew Jones, Manchester, UK
08:30 – 10:00
ePoster Corner A
ePoster Corner B
Symposium 13 - Standards of care
Moderators: Carlo Castellani, Verona, IT / Stuart Elborn, Belfast, UK The new ECFS standards of care: Why and how – Carlo Castellani, Verona, IT
08:52 – 09:14
The new ECFS standards of care: Centre framework – Steven Conway, Leeds, UK
09:14 – 09:36
The new ECFS standards of care: Best practice – Alan Smyth, Nottingham, UK
09:36 – 10:00
The new ECFS standards of care: Quality improvement – Martin Stern, Tübingen, DE
08:30 – 10:00
Symposium 14 - Interactive case studies
Moderators: Harry Heijerman, The Hague, NL / Malena Cohen-Cymberknoh, Jerusalem, IL / Damian Downey, Belfast, UK / Isabelle Sermet, Paris, FR
FRIDAY JUNE 13
08:30 – 08:52
K2-K3
E1-E3
08:30 – 08:52
Chronic progressive hypoxemia in a 14-year-old Cystic Fibrosis patient with a mild pulmonary disease: Work up, diagnosis and treatment – Oded Breuer, Jerusalem, IL
08:52 – 09:14
Clinical deterioration during pregnancy, leading to abortion and acute lung transplantation with fatal outcome – Ulrika Lindberg, Lund, SE
09:14 – 09:36 A binge drinking girl with recurrent pancreatitis – Barbara Bosch, Leuven, BE 09:36 – 10:00
Mundane mutations? To treat or not to treat? – Julie Duncan, London, UK
8:30 – 10:00
Symposium 15 - Caring in a changing world of CF
Moderators: Trudy Havermans, Leuven, BE / Jacqui Cowlard, London, UK
08:30 – 08:52
Parenting: Till death do us part – Vibsen Bregnballe, Aarhus, DK
08:52 – 09:14
Education and work across the lifespan – Dominique Hubert, Paris, FR
09:14 – 09:36
A lifetime of adherence – Mark Butler, London, UK
09:36 – 10:00
Patient-doctor communication in a changing world of CF – Yvonne Prins, Amsterdam, NL
08:30 – 10:00
Symposium 16 - Multidisciplinary aspects of CFRD
Moderators: Eitan Kerem, Jerusalem, IL / John Engelhardt, Iowa City, US 08:30 – 08:52
Insulin and the lung – Nicola Bridges, London, UK
08:52 – 09:14
Endocrine pancreas function at birth: Lessons from the ferret – John Engelhardt, Iowa City, US
09:14 – 09:36
Nutritional approach to the different stages of CFRD – Hila Elyashar-Earon, Jerusalem, IL
09:36 – 10:00
Coping with CFRD diagnosis – Maya Kirszenbaum, Paris, FR
K1
E4
34
FRIDAY, JUNE 13 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
08:30 – 10:00
Symposium 17 - Why physiotherapists should get excited about upper airways
Moderators: Irene Maguire, Galway, IE / Fred Lessire, De Haan, BE
FRIDAY JUNE 13
H2
08:30 – 08:52
Assessment and diagnosis of UAW disease in CF – Jochen Mainz, Jena, DE
08:52 – 09:14
Surgical and medical management of UAW disease – Anissa Souayah, Brussels, BE
09:14 – 09:36
Preparing the upper airways for AC? Role in prevention of development of UAW disease – Fred Lessire, De Haan, BE
09:36 – 10:00
Physiotherapy treatment options for UAW disease – Ruth Dentice, Sydney, AU
08:30 – 10:00
Symposium 18 - CFTR - A multidimensional view
Moderators: Luis Galietta, Genoa, IT / Ann Harris, Chicago, US 08:30 – 08:52
CFTR gene expression control – Ann Harris, Chicago, US
08:52 – 09:14
CFTR structure and thermostability – Robert C. Ford, Manchester, UK
09:14 – 09:36
Mechanism of corrector dF508 CFTR refolding – Gergely Lukacs, Montreal, CA
09:36 – 10:00
CFTR interactome – Margarida Amaral, Lisbon, PT
10:00 – 10:30
COFFEE BREAK
10:30 – 12:00
Symposium 19 - Airway controversies
Moderators: Charles Gallagher, Dublin, IE / Daniel Peckham, Leeds, UK
K2-K3
10:30 – 10:48
Upper airway: Surgery is the answer for sinus symptoms in CF - PRO – Kasper Aanaes, Copenhagen, DK
10:48 – 11:06
Upper airway: Surgery is the answer for sinus symptoms in CF - CON – Jochen Mainz, Jena, DE
11:06 – 11:16
Discussion
11:16 – 11:34
Airways should be kept sterile - PRO – Barry Plant, Cork, IE
11:34 – 11:52
Airways should be kept sterile - CON – Scott Bell, Brisbane, AU
11:52 – 12:00
Discussion
10:30 – 12:00
Symposium 20 - The therapeutic pipeline 2014
Moderators: Isabelle Fajac, Paris, FR / Kris De Boeck, Leuven, BE
H1
10:30 – 10:52
ECFS-CTN overview – Isabelle Fajac, Paris, FR
10:52 – 11:14
Where do we stand with correctors – Michael Boyle, Baltimore, US
11:14 – 11:36
Broadening the spectrum of potentiators – Kris De Boeck, Leuven, BE
11:36 – 12:00
Can we further improve symptomatic therapies – Patrick Flume, Charleston, US
10:30 – 12:00
Symposium 21 - Nursing in CF - An evolving role
Moderators: Ellen Julie Hunstad, Oslo, NO / Mark Butler, London, UK 10:30 – 10:52
Establishing a CF nursing service – Hélène Joachim, Toulouse, FR / Marythe Kerbrat, Roscoff, FR
10:52 – 11:14
The importance of partnership – Inge Heeres, Rotterdam, NL
E1-E3
K1
FRIDAY, JUNE 13
35
37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
11:14 – 11:36
The importance of research in nursing – Els Van der Wiel, Rotterdam, NL
11:36 – 12:00
Advanced nursing practice in CF – Su Madge, London, UK
10:30 – 12:00
Symposium 22 - CF infections under low oxygen conditions
Moderators: Burkhard Tümmler, Hannover, DE / Stuart Elborn, Belfast, UK 10:30 – 10:52
Anoxic persistence of Burkholderia cepacia complex bacteria – Eshwar Mahenthiralingam, Cardiff, UK
10:52 – 11:14
What’s new in our understanding of Pseudomonas aeruginosa anaerobic metabolism? – Daniel Hassett, Cincinnati, US
11:14 – 11:36
Burkholderia cenocepacia behaviour under micro-oxia – Leo Eberl, Zurich, CH
11:36 – 12:00
Pathogenesis and interactions of Prevotella spp. as dominant CF anaerobes – Michael Tunney, Belfast, UK
10:30 – 12:00
Symposium 23 - We have screened the baby, now what?
Moderators: Kevin Southern, Liverpool, UK / Isabelle de Monestrol, Stockholm, SE
H2
10:30 – 10:52
Management of equivocal diagnosis, the European consensus project – Sarah Mayell, Liverpool, UK
10:52 – 11:14
How do we prevent chronic airway infection and nutritional deficiency in the well infant following newborn screening? – Claire Wainwright, Herston, AU
11:14 – 11:36
Physiotherapy: Clearing airways in early life – Pamela McCormack, Liverpool, UK
11:36 – 12:00
Addressing the wellbeing of the family (and the CF team) – Mandy Bryon, London, UK
10:30 – 12:00
Symposium 24 - Mucus, mucins, and epithelial responses against bugs
Moderators: Gunnar C. Hansson, Gothenburg, SE / Richard Boucher, Chapel Hill, US
H1
10:30 – 10:52
The surface versus the gland mucus and mucins – Dave Thornton, Manchester, UK
10:52 – 11:14
The importance of bicarbonate and proteases at the birth of mucins and mucus – Gunnar C. Hansson, Gothenburg, SE
11:14 – 11:36
Biophysical basis for failed mucus transport in CF lung disease – Richard Boucher, Chapel Hill, US
11:36 – 12:00
Lessons of mucus defects in the early event of CF as studied in the CF pig – Mike Welsh, Iowa City, US
12:00 – 14:00
LUNCH BREAK
12:30 – 14:00
Satellite Symposium - Changing antibiotic delivery to improve CF patients’ lives: Challenges and opportunities
K2-K3
See detailed programme page 73
12:30 – 14:00
Satellite Symposium - New approaches to pancreatic enzyme replacement therapy
See detailed programme page 73
E1-E3
FRIDAY JUNE 13
E4
36
FRIDAY, JUNE 13 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
14:00 – 15:00
Posters
14:00 – 15:00
ePoster Sessions: Clinical challenges Bad bugs - What’s next? LCI technical
ePoster Corner A ePoster Corner B ePoster Corner C
14:00 – 15:00 Guided Poster Tours in the following categories: Physiotherapy Immunology/Inflammation/Pulmonology 12:00 – 15:00
FRIDAY JUNE 13
Poster Viewing: Poster presenters in the following categories are asked to be available at their posters for discussion: • Screening/Diagnosis • Immunology/Inflammation • Pulmonology • Physiotherapy • Nursing and Psychosocial Issues • Epidemiology/Registry See detailed programme page 48 and 49
15:00 – 16:30
Special Symposium - AHP Research Symposium: Changing clinical practice through research with minimal or no funding
Moderators: Su Madge, London, UK / Eleanor Main, London, UK
15:00 – 15:22
Clinical questions that can be answered with small scale studies – Susannah King, Melbourne, AU
15:22 – 15:44
Simple studies can determine the smallest worthwhile effect – Ruth Dentice, Sydney, AU
15:44 – 16:06
Low cost ways to reduce bias and improve clarity in clinical studies – Eleanor Main, London, UK
16:06 – 16:30
Minimal requirements for any clinical research study – Kate Blakeley, London, UK
15:00 – 16:30
Workshop 13 - New insights in lung disease
Moderators: Harm Tiddens, Rotterdam, NL / Donald Vandevanter, Cleveland, US
K2-K3
E1-E3
15:00 – 15:15
WS13.1 Patients with mutations that permit 3% or more of wild-type CFTR function are associated with higher FEV 1 – Patrick Sosnay, Baltimore, US
15:15 – 15:30
WS13.2 Change in FEV 1% predicted in one year in patients with nonsense mutations and patients homozygous for F508del – Kris De Boeck, Leuven, BE
15:30 – 15:45
WS13.3 Magnetic resonance imaging detects changes in structure and perfusion, and response to therapy in early Cystic Fibrosis lung disease – Mark Wielpuetz, Heidelberg, DE
15:45 – 16:00
WS13.4 Small-airway disease in Cystic Fibrosis studied with multidetector CT and microCT – Barbara Bosch, Leuven, BE
16:00 – 16:15
WS13.5 Visualising ventilation heterogeneity in mild CF using hyperpolarised 3He MRI, and comparison with lung clearance index (LCI) – Alexander Horsley, Manchester, UK
16:15 – 16:30
WS13.6 Structural alterations in the end-stage Cystic Fibrosis lung: Comparing histopathology to microCT – Elise Lammertyn, Leuven, BE
FRIDAY, JUNE 13
37
37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
15:00 – 16:30
Workshop 14 - Measurements in physiotherapy
Moderators: Louise Lannefors, Copenhagen, DK / Irene Maguire, Galway, IE 15:00 – 15:15
WS14.1 Day-time variability and short term effect of chest physiotherapy on multiple breath nitrogen washout in children with Cystic Fibrosis – Kent Green, Copenhagen, DK
15:15 – 15:30
WS14.2 Tolerability of inhaled dry powder Bronchitol (mannitol) – Fiona Shaw, London, UK
15:30 – 15:45
WS14.3 UK use of bronchial challenge for new therapies – Elaine Lloyd, Liverpool, UK
15:45 – 16:00
WS14.4 Analysis of expiratory flow rates used in autogenic drainage. Are they sufficiently high to mobilize secretions? – Maggie McIlwaine, Vancouver, CA
16:00 – 16:15
WS14.5 Influence of breathing pattern on pulmonary aerosol deposition in patients with Cystic Fibrosis (CF): A pharmacokinetic approach – Annelies Van Velzen, The Hague, NL
16:15 – 16:30
WS14.6 Feasibility study to objectively measure airway clearance technique in Cystic Fibrosis – Zhe Hui Hoo, Sheffield, UK
15:00 – 16:30
Workshop 15 - Mucus, vitamins and the pancreas
Moderators: Birgitta Strandvik, Gothenburg, SE / Anna Ermund, Gothenburg, SE
E4
15:00 – 15:15
WS15.1 Hyperosmolar saline causes detachment of Cystic Fibrosis mucus – Anna Ermund, Gothenburg, SE
15:15 – 15:30
WS15.2 The secreted mucus proteome in ileum of a Cystic Fibrosis mouse model – Ana Rodríguez-Piñeiro, Gothenburg, SE
15:30 – 15:50
WS15.3 Pancreatic duct ligated piglets - Used as a model for pancreatic exocrine insufficiency (PEI) in children: Studies on different parameters of growth and digestibility of macro minerals – Anne Mößeler, Hannover, DE
15:50 – 16:05
WS15.4 Cystic Fibrosis bronchial epithelial cells have impaired ability to activate vitamin D – Terezia Pincikova, Stockholm, SE
16:05 – 16:20
WS15.5 A ten year review of serum vitamin D levels in children with Cystic Fibrosis – Gwyneth Davies, London, UK
15:00 – 16:30
Workshop 16 - New development of microbial diagnostics
Moderators: Jean-Marc Rolain, Marseille, FR / Burkhard Tümmler, Hannover, DE 15:00 – 15:11
WS16.1 Development of a competitive ELISA for the detection of the Burkholderia cenocepacia siderophore, ornibactin – Niamh Whelan, Dublin, IE
15:11 – 15:22
WS16.2 Highly multiplexed molecular detection of CF lung infections – Sally Pattison, Belfast, UK
H2
15:22 – 15:33 WS16.3 Rethinking Pseudomonas aeruginosa (PA) lung infection: Using molecular microbiology rather than culture and antibodies – Helle Krogh Johansen, Copenhagen, DK 15:33 – 15:44
WS16.4 Serum-betaglucan as marker to differentiate between fungal airway colonization and infection in patients with Cystic Fibrosis – Karsten Kötz, Gothenburg, SE
15:44 – 15:55
WS16.5 Selective ion-flow mass spectrometry (SIFT-MS) analysis of exhaled breath as a non-invasive determinant of Pseudomonas aeruginosa infection in CF patients – Rishi Pabary, London, UK
15:55 – 16:06
WS16.6 MALDI-TOF as an alternative method for the identification and typing of Prevotella species from CF patients – Deirdre Gilpin, Belfast, UK
16:06 – 16:17
WS16.7 Use of nrdA sequence clustering for species-level identification of Achromobacter spp. from CF patients in multiple UK hospitals – Dervla Kenna, London, UK
16:17 – 16:28
WS16.8 Whole genome sequencing provides evidence of within-patient evolution and adaptation to the lung of Mycobacterium abscessus – Dorothy M. Grogono, Cambridge, UK
FRIDAY JUNE 13
K1
38
FRIDAY, JUNE 13 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
15:00 – 16:30
Workshop 17 - Epithelial cell biology
FRIDAY JUNE 13
Moderators: Paul Quinton, San Diego, US / Marcus Mall, Heidelberg, DE H1
15:00 – 15:15
WS17.1 Recruitment of CFTR to the enterocyte apical membrane is coordinated with internalization of the transmembrane mucin MUC17 and secretion of the MUC2 mucin from the goblet cells – Hannah Schneider, Gothenburg, SE
15:15 – 15:30
WS17.2 Hydrostatic pressure induced stretch activates CFTR in native pulmonary epithelium – Constanze Vitzthum, Giessen, DE
15:30 – 15:45
WS17.3 SLC26A9 channel functionality is characterised in epithelial cells – Johanna Salomon, Heidelberg, DE
15:45 – 16:00
WS17.4 Expression of ENaC subunits in Fischer Rat Thyroid cells as an investigation tool of the interaction between CFTR and ENaC and evaluation of ENaC inhibitors – Stefano Castellani, Foggia, IT
16:00 – 16:15
WS17.5 A new tool for CF diagnosis: Short circuit current measurements in human nasal epithelial cells collected by nasal brushing – Virginie Pruliere-Escabasse, Créteil, FR
16:15 – 16:30
WS17.6 Colonic mucus formation relies on bicarbonate secretion via apical Cl-/HCO3- exchange – Jenny Gustafsson, Gothenburg, SE
16:30 – 17:00
COFFEE BREAK
17:00 – 18:30
Workshop 18 - Late breaking science
Moderators: Stuart Elborn, Belfast, UK / Isabelle Fajac, Paris, FR
K2-K3
17:00 – 17:30
407 compound, a new corrector for ∆F508CFTR: State of art – Aleksander Edelman, Paris, FR
17:30 – 18:00
Clinical pharmacodynamics of CTX-4430, a potential new oral anti-inflammatory treatment for Cystic Fibrosis – Stuart Elborn, Belfast, UK
18:00 – 18:30
Effect of Ciprofloxacin on ivacaftor, a sensitive CYP3A substrate, in healthy volunteers – Sarah M. Robertson, Boston, US
17:00 – 18:30
Workshop 19 - Host responses to infection
Moderators: Kirsten Schaffer, Dublin, IE / Claus Moser, Copenhagen, DK
E1-E3
17:00 – 17:15
WS19.1 Inflammation and oxidation biomarkers in patients with Cystic Fibrosis (CF): Azithromycin influence – Casilda Olveira, Málaga, ES
17:15 – 17:30
WS19.2 Novel immunological tests for detection of Mycobacterium abscessus infection in patients with Cystic Fibrosis – Dirk Schramm, Düsseldorf, DE
17:30 – 17:45
WS19.3 Virulence of serial Pseudomonas aeruginosa isolates grown under aerobic and anaerobic conditions using the Galleria mellonella infection model – Emilie Vallières, Belfast, UK
17:45 – 18:00
WS19.4 Pro-inflammatory response of THP-1 monocytic cells to lipopolysaccharide from Prevotella sp. isolated from CF patients – Tom Matier, Belfast, UK
18:00 – 18:15
WS19.5 An investigation of Burkholderia cenocepacia adaptation during chronic infection and its impact on host response modulation – Luke McGuigan, Dublin, IE
18:15 – 18:30
WS19.6 Inverse protease-antiprotease relation in upper and lower airway secretions of Cystic Fibrosis patients – Julia Hentschel, Jena, DE
FRIDAY, JUNE 13
39
37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
17:00 – 18:30
Workshop 20 - Indexing the lung
Moderators: Per Gustafsson, Skövde, SE / Alexander Horsley, Manchester, UK 17:00 – 17:15
WS20.1 Are pre-school lung clearance index (LCI) measurements a predictor for later structural lung disease? – Krista Gerbrands, Rotterdam, NL
17:15 – 17:30
WS20.2 Difference between multiple breath washout with N2 (MBWN2) vs SF6 (MBWSF6) in 12 months follow up in children with CF – Anders Lindblad, Gothenburg, SE
17:30 – 17:45
WS20.3 Lung clearance index and Aspergillus colonization are clinical markers of chronic lung changes by spirometry controlled CT in children with Cystic Fibrosis – Thomas Kongstad, Copenhagen, DK
17:45 – 18:00
WS20.4 Is lung clearance index (LCI) affected by the severity of lung disease in CF? – Elpida Hatziagorou, Thessaloniki, GR
18:00 – 18:15
WS20.5 A high colony count of anaerobic bacteria is related to lung clearance index in Cystic Fibrosis – Katherine O’Neill, Belfast, UK - Young Investigator Award Winner
18:15 – 18:30
WS20.6 The use of LCI as an effective tool for monitoring clinical response to ivacaftor therapy in CF patients with at least one G551D-allele – Laura Jenkins, Belfast, UK
17:00 – 18:30
Workshop 21 - Microbial diversity in the lung
Moderators: Niels Høiby, Copenhagen, DK / Joanne Fothergill, Liverpool, UK
E4
17:00 – 17:15
WS21.1 The genetic basis of Pseudomonas aeruginosa pathoadaptation to the airways of Cystic Fibrosis patients – Rasmus L. Marvig, Copenhagen, DK - Young Investigator Award Winner
17:15 – 17:30
WS21.2 Sequence-function analysis of clinical LasR variants of Pseudomonas aeruginosa – Ajai Dandekar, Seattle, US
17:30 – 17:45 WS21.3 In vitro non-mucoid to mucoid phenotypic switching in clinical isolates of Pseudomonas aeruginosa is induced by mannitol – John Moore, Belfast, UK
17:45 – 18:00
WS21.4 Use of genome sequencing to study population diversification and transmission of a Pseudomonas aeruginosa epidemic strain – Craig Winstanley, Liverpool, UK
18:00 – 18:15
WS21.5 A comparative study of motility and biofilm production in Cystic Fibrosis (CF) and environmental Pseudomonas aeruginosa (Pa) isolates – Kay Ramsay, Brisbane, AU
18:15 – 18:30
WS21.6 Comparison of the CF airway microbiome obtained by bronchoscopy vs. sputum – Marianne Muhlebach, Chapel Hill, US
17:00 – 18:30
Workshop 22 - Psychosocial/Nursing complex case presentations
Moderators: Ellen Julie Hunstad, Oslo, NO / Mark Butler, London, UK
H2
17:00 – 17:18
Seeing through the Kalydeco: When a potentially lifesaving treatment fails to bring focus – Beverly Govin, Liverpool, UK
17:18 – 17:36
Psychological care of a teenager with CF undergoing a double lung transplantation and ten years later a re-transplantation – Trudy Havermans, Leuven, BE
17:36 – 17:54
Use of insulin to treat hypoglycaemia - An unusual presentation of CF-Related Diabetes in a teenage girl – Katie Dick, London, UK
17:54 – 18:12
Using behaviour change techniques to improve adherence to inhaled therapy and create habits – Rachael Curley, Sheffield, UK
18:12 – 18:30
An attempt to eradicate Mycobacterium abscessus in a patient with Cystic Fibrosis (CF) – Kairen Griffiths, Aberdeen, UK
FRIDAY JUNE 13
K1
40
FRIDAY, JUNE 13 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
17:00 – 18:30
Workshop 23 - CFTR genetics and function
FRIDAY JUNE 13
Moderators: Nico Derichs, Berlin, DE / Patrick Sosnay, Baltimore, US
H1
17:00 – 17:15
WS23.1 Models of the 3D structure of CFTR: From the understanding of the protein functions to the design of correctors – Brice Hoffmann, Paris, FR - Young Investigator Award Winner
17:15 – 17:30
WS23.2 Characterization of the CFTR mutation c.3700 A>G informs strategies for future medical intervention – Steven Molinski, Toronto, CA
17:30 – 17:45
WS23.3 ICM is sensitive to detect potentiation of CFTR-mediated Cl- secretion in patients with Cystic Fibrosis and the G551D mutation treated with ivacaftor – Simon Graeber, Heidelberg, DE
17:45 – 18:00
WS23.4 Rare CF genotype with severe hepatic failure associated with medium chain acid deficiency (MCAD) in a neonate – Anne Mornand, Geneva, CH
18:00 – 18:15
WS23.5 Clinical importance of homozygous R117C (c.349C>T) CFTR mutation – Isabelle de Monestrol, Stockholm, SE
18:15 – 18:30
WS23.6 Ivacaftor treatment in patients with Cystic Fibrosis who have an R117H-CFTR mutation, the KONDUCT study – Richard Moss, Palo Alto, US
NOTES
41
37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
FRIDAY JUNE 13
42
SATURDAY, JUNE 14 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
K2-K3
E1-E3
K1
E4
SYMPOSIUM 27
SYMPOSIUM 28
07:00 07:30 08:00 08:30 09:00
SYMPOSIUM 25
SYMPOSIUM 26
Clinical trials in preschool
Inflammation, infection and Depression and anxiety macrolides in CF: Proposals from the CFF-ECFS Guidelines Committee
09:30 09:00 – 10:30 10:00 children 10:30
CLOSING PLENARY 11:30 11:00 – 12:30 12:00 12:30
CLOSING CEREMONY 13:00 12:30 – 13:00
14:00 14:30 15:00 15:30 16:00 16:30 17:00 17:30 18:00 18:30 19:00 19:30 20:00 20:30 21:00
09:00 – 10:30
COFFEE BREAK IN EXHIBITION
11:00
13:30
09:00 – 10:30
09:00 – 10:30
Functional intestinal outcomes measures in CF in the era of correctors and potentiators
SATURDAY, JUNE 14
43
37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
H2
H1
Exhibition & Poster Area
07:00 07:30 08:00 08:30 09:00
SYMPOSIUM 29
SYMPOSIUM 30
Pharmacovigilance/Phase 4 studies
The “epithelial“ channelome and options for rescue
09:30 09:00 – 10:30
10:00 using registries
09:00 – 10:30
10:30 11:00
COFFEE BREAK IN EXHIBITION
EXHIBITION & POSTERS
11:30
12:30 13:00 13:30 14:00 14:30 15:00 15:30 16:00 16:30 17:00 17:30 18:00 18:30 19:00 19:30 20:00 20:30 21:00
SATURDAY JUNE 14
12:00
44
SATURDAY, JUNE 14 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
09:00 – 10:30
Symposium 25 - Clinical trials in preschool children
Moderators: Jane Davies, London, UK / Felix Ratjen, Toronto, CA
09:00 – 09:22
How to do an interventional trial in infants and toddlers – Adam Jaffe, Randwick, AU
09:22 – 09:40
We must urgently try out novel drugs in early childhood - PRO – Margaret Rosenfeld, Seattle, US
09:40 – 09:58
We must urgently try out novel drugs in early childhood - CON – Michael Fayon, Bordeaux, FR
09:58 – 10:08
Discussion
10:08 – 10:30
Regulatory challenges in trials with preschool children – Irmgard Eichler, London, UK
09:00 – 10:30
Symposium 26 - Inflammation, infection and macrolides
SATURDAY, JUNE 14
Moderators: Keith Grimwood, Brisbane, AU / Kevin Southern, Liverpool, UK
09:00 – 09:22
Autophagy: Is it important in CF? – Luigi Maiuri, Milan, IT
09:22 – 09:44
Macrolides: Promises and risks – Kevin Southern, Liverpool, UK
09:44 – 10:06
Macrolides and their use in infectious disease: Benefits and risks – Keith Grimwood, Brisbane, AU
10:06 – 10:30
Macrolides: Are they of use in other non-CF-lung diseases? – Lennart Hansson, Lund, SE
09:00 – 10:30
Symposium 27 - Depression and anxiety in CF: Proposals from the CFF-ECFS Guidelines Committee
K2-K3
E1-E3
Moderators: Stuart Elborn, Belfast, UK / Alexandra Quittner, Coral Gables, US
K1
09:00 – 09:15
Prevalence of depression and anxiety in CF: Results of the international TIDES study – Alexandra Quittner, Coral Gables, US
09:15 – 09:30
International survey of CF mental health care delivery – Janice Abbott, Preston, UK
09:30 – 09:40 A parent’s perspective – Ulrica Sterky, Gothenburg, SE 09:40 – 09:50 Stepped care model for screening and intervention – Beth A. Smith, Buffalo, US
09:50 – 10:00
Evidence based psychological intervention for depression and anxiety – Lutz Goldbeck, Ulm, DE
10:00 – 10:10
Evidence based pharmacological interventions for depression and anxiety – Anna Georgiopoulos, Boston, US
10:10 – 10:30
Panel discussion
09:00 – 10:30
Symposium 28 - Functional intestinal outcomes measures in CF in the era of correctors and potentiators
Moderators: Michael Wilschanski, Jerusalem, IL / Henkjan Verkade, Groningen, NL
E4
09:00 – 09:22
Intestine-derived organoids: Tools to predict the restoration of intestinal CFTR function? – Jeffrey Beekman, Utrecht, NL
09:22 – 09:44
Gastrointestinal outcome measures for clinical CF trials: Opportunities and challenges – Frank Bodewes, Groningen, NL
09:44 – 10:06
Video endoscopy for the assessment of intestinal CFTR function – Michael Wilschanski, Jerusalem, IL
10:06 – 10:30
Smart pills for intestinal CF: Treatment effects and future possibilities – Drucy Borowitz, Buffalo, US
SATURDAY, JUNE 14
45
37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
09:00 – 10:30
Symposium 29 - Pharmacovigilance/Phase 4 studies using registries
Moderators: Hanne Olesen, Aarhus, DK / Edward McKone, Dublin, IE
09:00 – 09:22
UK example – Diana Bilton, London, UK
09:22 – 09:44
Examples from the USA – Bruce Marshall, Bethesda, US
09:44 – 10:06
Perspectives from pharma – Sini Eskola, Brussels, BE
10:06 – 10:30
Opportunities for the European Registry – Edward McKone, Dublin, IE
09:00 – 10:30
Symposium 30 - The “epithelial“ channelome and options for rescue
Moderators: Ursula Seidler, Hannover, DE / Paul Quinton, San Diego, US 09:00 – 09:22
Bicarbonate and its channels – Paul Quinton, San Diego, US
09:22 – 09:44
SLC26A9-mediated chloride secretion can prevent lung mucus obstruction – Marcus Mall, Heidelberg, DE
09:44 – 10:06
Kcnn4, Tmem16A, alternative channels that can affect CF disease – Carlos Flores, Santiago, CL
10:06 – 10:30
cAMP compartmentation and CFTR regulation – Manuela Zaccolo, Oxford, UK
10:30 – 11:00
COFFEE BREAK
11:00 – 12:30
Closing Plenary
Moderators: Stuart Elborn, Belfast, UK / Anders Lindblad, Gothenburg, SE
H1
11:00 – 11:30
Mucus - The central problem in CF – Gunnar C. Hansson, Gothenburg, SE
11:30 – 12:00
Preventing and treating pulmonary exacerbations – Patrick Flume, Charleston, US
12:00 – 12:30
ECFS - Your society: Review of the year and plans for next – Stuart Elborn, Belfast, UK
12:30 – 13:00
Closing Ceremony
SATURDAY JUNE 14
H2
K2-K3
K2-K3
During the Closing Ceremony, Prof. Stuart Elborn, ECFS President, will present prizes to the first authors of the best posters in the following categories:
Genetics/Screening/Diagnosis CFTR/Cell Biology/Cell Physiology/New Therapies Microbiology Pulmonology Physiotherapy Gastrointestinal/Liver Disease/Metabolic Complications of CF/Nutrition Nursing/Psychosocial Issues Epidemiology/Registry/Delivery of Care
Awarded authors are respectfully requested to attend the Closing Ceremony to receive their prizes on stage.
POSTER AREA 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
POSTER AREA - LEVEL 0
283 - 298: Epidemiology/ Registry
2 28 83 2 7
24 6 1 21 0 21 4
21 6
17 3 17 8
17 9
13 7 13
2 14 3 13
125 - 168: Pulmonology
2
6 10
10 1
99
97
103
100
10
18 5
14 9
24
28 7 8
25 1 2 24 5 20 8
21 2
3 22 8 19 7 18 1 16
16
25 5 8 23 9 20 3 16 6
25 9 4 23
12
11 0
98
4
96
10
95
93
5
0
15 5
13
12
139
136
105
8
11 4
172
0
107
134
14
132
10
89
27
1 29 4 27
29 0 27
26 3
26 0 23 7 22 4 19 1 19 7 15
1
17
1
13
4
175
169 - 183: Physiotherapy
141
4
15
212
209
6
170
17
16
1
8
20
0
12
245
177
18
11 8
248
3
207
21
4
91
87
85
4
24
143
243
9
168
20
2
6
92
241 - 282: 281 Nursing/ Psychosocial Issues
24
179
109 94
5 28 80 2
205
7
12
130
284
214
203
21
8
12
88
216
14
111
241
145
113 90
252
7
11
11
115
239
166
279
250
3 25 40 2
7
128
16
3
124
126
9 28 7 6 2
147
149
2
84
86
164
5
15
151
277
181
18
9
15
160
117 119
184
0
9
18
108 - 124: Immunology/ Inflammation
201
20
6
120
122
162
288
218
186 188
153 155
220
1
19
197
199
22
5
156
158
237
222
190 192
256
14
224
235
3
22
193
195
286
275
254
7 25 36 2
233
226 228
2
2
23
184 - 218: Gastrointestinal/ Liver Disease/ Metabolic Complications of CF/Nutrition/Growth
229
273
258
1
219 - 240: Delivery of Care
231
27
26
260
262 264
290 292
3
8
26
269
271
29
5
294 296
7
265
267
29
6
298
48 - 107: Microbiology 62
66
70
60
61
74 57
69 71
63
56
58
68
75
59
53
73
55
52
54
72
79
76
78
77
51
48
50
80
82
81 83
49
67
64
36 - 47: New Therapies 36
40 32
13
17
9
10
7
8
18
14 11
12
28
21
37
35
33 - 35: Cell Biology/ Cell Physiology/ CFTR
16
6
3
5
33
15
22
19
2
Poster Help Desk
31
20
23
1 - 7: Genetics
41
34
24 1
29
30
26
27
38
45
39
42
46
25
43
44
47
4
46
8 - 32: Screening/Diagnosis
NOTES
47
37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
POSTERS
48
GUIDED POSTER TOURS - ePOSTER SESSIONS 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
THURSDAY, JUNE 12, 14:00 – 15:00 ePOSTER SESSIONS
SCREENING DIFFERENT COUNTRIES Leader: Kevin Southern, Liverpool, UK Meeting point in the ePoster Corner A located in the Exhibition Area. MICROBIOLOGY - ANTIBIOTICS Leader: Helle Krogh Johansen, Copenhagen, DK Meeting point in the ePoster Corner B located in the Exhibition Area. PUTTING THE PATIENT FIRST Leader: Maya Kirszenbaum, Paris, FR Meeting point in the ePoster Corner C located in front of session rooms H1 & H2 (Hall H – Level 1 of the Conference Centre).
GUIDED POSTER TOURS
MICROBIOLOGY Leader: Miguel Cámara, Nottingham, UK Meeting point and start of the tour at Poster 48 GASTROINTESTINAL/LIVER DISEASE/METABOLIC COMPLICATIONS OF CF/NUTRITION Leader: Gary Connett, Southampton, UK Meeting point and start of the tour at Poster 184
POSTERS
FRIDAY, JUNE 13, 14:00 – 15:00 ePOSTER SESSIONS
CLINICAL CHALLENGES Leader: Malena Cohen-Cymberknoh, Jerusalem, IL Meeting point in the ePoster Corner A located in the Exhibition Area. BAD BUGS - WHAT‘S NEXT? Leader: John Moore, Belfast, UK Meeting point in the ePoster Corner B located in the Exhibition Area. LCI TECHNICAL Leader: Florian Singer, Bern, CH Meeting point in the ePoster Corner C located in front of session rooms H1 & H2 (Hall H – Level 1 of the Conference Centre).
GUIDED POSTER TOURS
PHYSIOTHERAPY Leader: Brenda Button, Melbourne, AU Meeting point and start of the tour at Poster 169 IMMUNOLOGY/INFLAMMATION/PULMONOLOGY Leader: Madeleine Ennis, Belfast, UK Meeting point and start of the tour at Poster 108
POSTER VIEWING
49
37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
Poster authors are asked to be present next to their posters according to the following timetable:
THURSDAY, JUNE 12, 14:00 – 15:00 Genetics: Cell Biology/Cell Physiology/CFTR: New Therapies: Microbiology: Gastrointestinal/Liver Disease/Metabolic Complications of CF/ Nutrition/Growth: Delivery of Care:
Posters 1 to 7 Posters 33 to 35 Posters 36 to 47 Posters 48 to 107 Posters 184 to 218 Posters 219 to 240
FRIDAY, JUNE 13, 14:00 – 15:00 Screening/Diagnosis: Immunology/Inflammation: Pulmonology: Physiotherapy: Nursing/Psychosocial Issues: Epidemiology/Registry:
Posters 8 to 32 Posters 108 to 124 Posters 125 to 168 Posters 169 to 183 Posters 241 to 282 Posters 283 to 298
POSTERS LOCATION OF THE POSTERS
All posters will be displayed in the Poster Area within the Exhibition (Level 0 of the Swedish Exhibition and Congress Centre).
Wednesday, June 11 Thursday, June 12 Friday, June 13 Saturday, June 14
INFORMATION FOR POSTER PRESENTERS
18:00 – 21:30 08:00 – 18:00 08:00 – 18:00 08:30 – 12:30
SET-UP AND DISMANTLING Posters may be set-up beginning Wednesday, June 11 at 14:00. Poster presenters will have access to the Poster Area at 08:00 on June 12 and 13. Posters should be removed on Saturday, June 14 between 12:30 and 14:00. Any Posters not removed by 14:00 will be discarded.
POSTERS
THE POSTER AREA WILL BE OPEN AT THE FOLLOWING TIMES:
50
POSTERS 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
NR ABSTRACT TITLE
PRESENTER
GENETICS 1
Alleles polymorphism of microsomal epoxide hydrolase gene (mEPHX) as modifier factor in Cystic Fibrosis
2
Genome sequence of the Danish epidemic strain of Achromobacter ruhlandii
3
High throughput genotyping methods dealing with a wide spectrum of CFTR mutations and low incidence of CF disease in Galicia (NW Spain)
4
Prevalence of ΔF508 mutation in patients with Cystic Fibrosis in a miscegenated population of the Brazilian Northeast
5
Phenotypic characteristics of p.E92K mutation in adults with Cystic Fibrosis (CF) in Russia
6
CFTR dele2,3 (21kb) mutation in Cystic Fibrosis patients in Latvia
7
Genetic prevention of Cystic Fibrosis as a part of the national strategy for rare diseases in University Hospital Brno, Czech Republic (genetic counselling - DNA analysis - newborn screening - education)
N. Rohovyk K. J. Handberg C. Colon E. L. Souza M. Usacheva G. Taurina R. Gaillyová
POSTERS
SCREENING/DIAGNOSIS 8
Phenotyping the 711+1 G>T mutation in Cystic Fibrosis patients
P. Daigneault
9
CFTR rearrangements in Serbian patients with classical Cystic Fibrosis
10
Complex alleles in CFTR gene in Venezuelan patients with Cystic Fibrosis
K. Sanchez
11
Disease related CFTR variants found in the Norwegian screening program
H. Gaup
12
Associated conditions in Cystic Fibrosis: Findings at diagnosis and changes in time, impact on clinical outcomes and quality of life
13
Cystic Fibrosis (CF) newborn screening in the Russian Federation
14
Updated survey of newborn screening for Cystic Fibrosis
15
National newborn screening for Cystic Fibrosis in Switzerland - A parents‘ perspective
16
Influence of newborn screening on the age of diagnosis in Cystic Fibrosis patients
17
Cystic Fibrosis newborn screening (CF NBS): 4 year experience of the Prague Centre with the IRT/DNA/IRT protocol
18
The first date with patients detected by newborn screening - What do we see?
19
Newborn screening for Cystic Fibrosis - Polish experience with CFTR sequencing strategy
20
Inconclusive diagnosis following newborn screening for Cystic Fibrosis (CF): Clinical outcomes in 56 infants from three Spanish CF centres
21
Outcomes of children referred after positive Cystic Fibrosis (CF) newborn screening, who have a normal sweat test
22
IRT/IRT vs. IRT/PAP. Which is the best strategy? A pilot study
23
Implementation of a diagnostic work up after newborn screening for CF in Oslo, Norway
24
Qualitative study on parental views on the most acceptable way to be told their child has a probable diagnosis for Cystic Fibrosis following neonatal screening
A. Crutchley
25
Cystic Fibrosis (CF) prevalence derived from CF newborn screening (CFNBS) in the Czech Republic: Comparison of previous epidemiological and current CFNBS-based disease prevalence data
V. Krulišová
26
Risk factors for poor outcomes in Cystic Fibrosis newborns diagnosed by neonatal screening in Italy: Years 2009 - 2011
27
Infants with an equivocal diagnosis at the Oslo CF centre following newborn screening for CF in Norway
28
Developing a specialist nurse run adult sweat test clinic
A. Divac Rankov
L.-L. Dracea E. Kondrateva S. Pybus C. Rueegg D. Sands A. Holubová J. Hammermann A. SobczynskaTomaszewska S. Gartner C. L. Edmondson A. Teper E. J. Hunstad
T. Repetto E. Bakkeheim A. Peres
POSTERS
51
37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
29
Reducing the rate of inadequate sweat testing for the NBS in the state of Michigan
30
Lung infections in patients with Cystic Fibrosis - Leading microbial pathogens
31
Feasibility of 1H-NMR spectroscopy to detect occurrence of Pseudomonas aeruginosa in saliva of Cystic Fibrosis patients
32
Correlation between structural and functional lung injury in children and adolescents with Cystic Fibrosis
S. Nasr V. Nedkova R. Rivas P. M. N. Oliveira
CELL BIOLOGY/PHYSIOLOGY/CFTR 33
Linking protein N-glycosylation and CF: Malfunctioning CFTR and pulmonary infection yield aberrant N-glycosylation of sputum derived proteins of CF individuals
34
Alterations in Cystic Fibrosis peripheral blood mononuclear cells are induced by an increase in intracellular calcium concentration
35
Cystic Fibrosis transmembrane regulator mutations in Turkish patients with Cystic Fibrosis
M. ThaysenAndersen M. Averna A. Bingol
NEW THERAPIES 36
The effect of ivacaftor therapy on clinical and PCR-identified microbial diversity of Cystic Fibrosis lung infection
H. Green
37
Clinical outcomes of real world Kalydeco (CORK) study
N. Ronan
38
Cayston® in clinical practice - Experiences from a UK adult CF unit
E. Lloyd
39
“Real world“ tolerability, ease of use, patient satisfaction and reported adherence in CF adults commencing Colobreathe®
E. Nash
40
Experience with Colobreathe® in a large adult Cystic Fibrosis unit
41
Pharmacokinetics of nasally administered tobramycin, colistin sulphomethate sodium and a combination of tobramycin and colistin sulphomethate sodium
42
Tobramycin powder for inhalation is effective in advanced stage CF lung disease: The EAGER trial
R. Chiron
43
Experience with fosfomycin: Clinical outcomes at an adult UK CF centre
P. Wilson
44
An alginate oligomer (OligoG) developed as a dry powder for inhalation in Cystic Fibrosis demonstrates an excellent safety profile, similar to a nebulised solution used in a phase 2a trial
A. H. Myrset
45
A first-in-patient clinical trial demonstrates that inhaled alginate oligosaccharide (OligoG) is well tolerated in Cystic Fibrosis (CF) patients
M. Walshaw
46
Ex vivo efficacy of Lynovex®, a next generation tri-functional candidate Cystic Fibrosis therapy
47
A novel point of care lipase (ALCT-460) increases fat hydrolysis and omega-3 fat absorption in pigs with Exocrine Pancreatic Insufficiency (EPI)
R. Sapina-Vivo M. Berkhout
C. Martin
MICROBIOLOGY 48
Interactions of microbes during chronic lung infection in Cystic Fibrosis patients
J. Fothergill
49
Subgingival plaque in patients with Cystic Fibrosis: Potential source for airway colonisation
50
Microbiological characterization of patients in Lisbon Cystic Fibrosis centre
51
The impact of coliforms isolated from cough swabs from Infants under the age of one year with Cystic Fibrosis
52
Burkholderia cenocepacia shows increased attachment to Cystic Fibrosis lung epithelial cells over time of colonisation
53
Genome changes leading to persistence of Pseudomonas aeruginosa in the airways of CF patients
L. Madsen
54
Intraclonal genome diversity of the major Pseudomonas aeruginosa clones C and PA14
N. Cramer
D. McLean R. Espírito Santo E. Robson L. Cullen
POSTERS
D. Fraser-Pitt
52
POSTERS
POSTERS
37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
55
Investigation of the RetS-GacAS regulatory network in clinical P. aeruginosa isolates reveals evolutionary adaptation
M. Lindegaard
56
Pseudomonas aeruginosa end-stage isolates: Analysis of virulence traits and interaction with macrophages
N. Cifani
57
Emergence of a Panton-Valentine leukocidin (PVL) positive MRSA strain in Cystic Fibrosis (CF) patients
P. Cocchi
58
Incidence of non-tuberculous mycobacteria in an adult CF centre
59
Population structure and antimicrobial susceptibility of Pseudomonas aeruginosa from a national survey involving 24 Cystic Fibrosis units in Spain
60
Evaluation of twice-a-year antibiotic susceptibility testing for Pseudomonas aeruginosa in adult patients with Cystic Fibrosis
61
Baseline data from a prospective 5-year registry study to monitor susceptibility to aztreonam of Pseudomonas aeruginosa (PA) isolates from patients with Cystic Fibrosis (CF) in the United States
62
A rapid biofilm antimicrobial susceptibility assay under 24 hours for Cystic Fibrosis patients with P. aeruginosa lung infection
63
Distribution of tobramycin and the impact on bacterial killing in a biofilm model
64
The prevalence of cochlear and vestibular dysfunction in patients with Cystic Fibrosis
66
Association of urinary kidney injury molecule-1 with aminoglycoside exposure in children with Cystic Fibrosis
67
Relative Resistance Index - A scoring system for antibiotic resistance in P. aeruginosa
68
Fast and convenient metalloprotease activity assay based on flow cytometry and its application in Cystic Fibrosis
69
Rapid induction of high level azithromycin resistance in clinical CF isolates
70
Expression of RND-type efflux pumps as a mechanism of antibiotic resistance in clinical Prevotella isolates
L. Sherrard
71
Extended-spectrum β-lactamase (ESβL) production: A mechanism of resistance to ceftazidime in Prevotella species isolated from patients with CF
L. Sherrard
72
Inhalatory antibiotic therapy in Cystic Fibrosis and emergence of colistin resistant Gram-negative non-fermenting bacteria: A new problem in pulmonary infection treatment?
73
Real world evidence on inhaled tobramycin use in CF patients: Analysis of the RAMQ data (Canada)
74
An evaluation of treatment burden following initiation of TOBI® Podhaler® in patients with CF
75
Efficacy of inhaled antibiotics in CF patients with P. aeruginosa (Pa) infection: A network meta-analysis (NMA)
G. Angyalosi
76
Aerosolized antibiotic therapy in Cystic Fibrosis with Pseudomonas aeruginosa airway infection in children
R. Eremciuc
77
Long-term safety of tobramycin powder for inhalation in patients with Cystic Fibrosis: ETOILES study
78
Comparison of pierced versus broken capsules on delivered fine particle dose for the colistimethate dry powder inhaler
79
Intravenous colistin and neurotoxicity: Recommendations for optimal use in Cystic Fibrosis (CF) patients
80
Therapeutical drug monitoring of piperacillin/tazobactam, given as continuous infusion to patients with Cystic Fibrosis
81
Susceptibility of Exophiala dermatitidis to antifungal agents
82
Effect of ivacaftor on CF clinical isolates of Pseudomonas aeruginosa, Staphylococcus aureus and Streptococcus sp. in comparison to antimicrobial peptides
83
Bronchopulmonary infection/colonization in Cystic Fibrosis: Results from a Spanish multicenter study J. D. D. Caballero
84
Respiratory microbiota in children with Cystic Fibrosis and healthy controls
85
What is bacterial colonisation of Cystic Fibrosis children toothbrushes?
86
Comparative study for the evaluation of a new technology for Cystic Fibrosis screening
M. Rongioletti
87
Metagenomic approach to bacterial identification in induced sputum from Cystic Fibrosis subjects
R. del Campo
E. Lloyd J. D. D. Caballero D. Hubert E. DiMango W. Hengzhuang B. Cao V. Harris S. McWilliam J. Ewing G. Bergamini C. McGettigan
P. Morelli G. Angyalosi D. Bilton
U. Sommerwerck M. Ng P. Schelstraete K. Öbrink-Hansen N. Kondori J. Payne
E. Gaillard A. Genevois
POSTERS
53
37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
88
Use of MALDI-TOF-MS for identification of anaerobic bacteria in CF patients
89
Hypermutation in Burkholderia cepacia complex is highly prevalent in chronic respiratory infections of Cystic Fibrosis patients
A. Bosch
90
Burkholderia contaminans in Cystic Fibrosis: Genetic and phenotypic diversity among isolates from long-term infections
A. Bosch
91
Epidemiological study of CF patients with chronic P. aeruginosa colonization in a reference center in Brazil
92
Molecular epidemiological surveillance of Pseudomonas aeruginosa at a Swedish CF centre over a >10-year period (1999-2012)
93
Infection control in Cystic Fibrosis: Assessment of Pseudomonas aeruginosa genotypes isolated from Italian children patients
P. Morelli
94
The effect of P. aeruginosa infection on pulmonary function outcome in a cohort of patients with nonsense mutation Cystic Fibrosis
E. Kerem
95
Susceptibility testing of isolates of Pseudomonas aeruginosa in patients with Cystic Fibrosis Service evaluation
96
Staphylococus aureus in Czech Cystic Fibrosis patients - Prospective study
97
Prevalence and spa genotype characterization of Staphylococcus aureus isolated from patients attending Gothenburg CF centre
98
NTM: Lessons from the UK CF registry
99
Low prevalence of non tuberculosis mycobacteria (MNT) in a referral CF center in Brazil: Methodological limitation or reality?
100 Common clone of Mycobacterium abscessus identified among adults and children in a large UK CF Centre 101 Non-tuberculous mycobacterial infection in a large paediatric Cystic Fibrosis centre: Epidemiology and possible transmission event
N. Ravenni
N. Caçador E. A. Johansson
I. Baker J. Tkadlec E. A. Johansson M. Rosenthal C. E. Levy M. Denton L. Nair
103 “A mushrooming problem or not“ - Prevalence of fungal isolation at the All Wales Adult CF Centre (AWACFC)
L. Speight
104 High prevalence of Aspergillus spp. in a reference CF center in Brazil: An agent that should not be neglected?
C. E. Levy
105 Evaluation of fungal diagnostic stains & prolonged sputum culture in Cystic Fibrosis fungal disease 106 Epidemiological study of A. xylosoxidans in a reference center for CF treatment in Brazil 107 Time trends in clinical indicators for Cystic Fibrosis (CF) patients at transition to adult care
I. Felton C. Capizzani H. Sandhu
IMMUNOLOGY/INFLAMMATION 108 Reduced effect of intravenous antibiotic treatment on sinonasal - Compared to pulmonary inflammatory markers 109 Increased IL-8 production in human CFTR-deficient macrophages 110 Connectivity mapping: An advanced bioinformatics approach to predict A20-inducing small molecules to reduce inflammation 111 Defective A20 signalling in Cystic Fibrosis: Anti-inflammatory action of gibberellin 112 Anti-inflammatory action of gibberellin in Cystic Fibrosis epithelial cells is associated with reduction of kinase expression 113 Increased susceptibility to enterovirus infections in a model for Cystic Fibrosis is associated with an impaired innate and adaptive antiviral immune response 114 Prevotella modulate the Pseudomonas aeruginosa mediated host response in Cystic Fibrosis bronchial epithelial cells
J. Mainz B. Pompili R. Barsden M. Ennis A. Todd E. Svedin A. Bertelsen
POSTERS
102 Fungal colonization in the airways of Spanish Cystic Fibrosis patients: Results from a multicenter study J. D. D. Caballero
54
POSTERS 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
115 Comparison of serum resistance in Prevotella isolates cultured from CF patients when stable and during pulmonary exacerbation
D. Mooney
116 P. histicola drives transcription of an elevated host response compared to P. nigrescens in Cystic Fibrosis bronchial epithelial cells
A. Bertelsen
117 Significance of oxygen depletion in chronic lung infections in Cystic Fibrosis patients 118 BPI-ANCA and Pseudomonas serology in Swedish CF patients - A comparison 119 Diagnostic value of serum antibodies to Pseudomonas aeruginosa in patients with CF in a Brazilian referral center 120 Anti-Pseudomonas aeruginosa IgY antibodies promote bacterial clearance in a murine pneumonia model 121 Anti-P. aeruginosa antibodies and microbiological outcome in not chronically infected patients 122 Modulation of Cystic Fibrosis gut inflammation - A pivotal role of the lung gut axis? 123 How frequent is Clostridium in our CF patients? 124 The prevalence and financial impact of beta-lactam allergy in CF
M. Kolpen U. Lindberg R. Mauch K. Thomsen D. Dolce G. O‘Callaghan L. Pop P. Whitaker
PULMONOLOGY 125 Feasibility of carrying out spirometry in 3 and 4 year old children in a standard clinic setting
POSTERS
126 What factors determine lung function decline in Polish CF children
P. Cole I. Stelmach
127 Improving the multiple breath washout test: Closed circuit washin with a novel bolus gas delivery system substantially reduces washin time and does not alter washout
A. Horsley
128 Multiple breath nitrogen washout in adults with Cystic Fibrosis: A comparison of two commercially available devices
W. Poncin
129 Shortened SF6 MBW is a repeatable and sensitive test in adults and children with CF
D. Hannon
130 Enhanced photoacoustic gas analyser (Innocor) for multiple breath washout. Improvements to analyser response time maintains accuracy at fast ventilation rates, and produces a system that meets all washout technology performance targets
A. Horsley
131 Multiple breath nitrogen washout in healthy children and adults: A comparison of two commercially available devices
W. Poncin
132 Improving the feasibility of multiple-breath nitrogen washout in Cystic Fibrosis adults
W. Poncin
133 Slow and fast lung compartments in Cystic Fibrosis measured by nitrogen multiple-breath washout (N2MBW)
B. Houltz
134 Nutritional status, lung function and LCI among children with CF 135 MEF25 as parameter to evaluate effects of mannitol inhalation 136 Nocturnal oximetry in children with Cystic Fibrosis (CF) 137 Sleep disturbances in CF children: Looking for severity predictors 138 Depiction of anatomic structures of relevance for scoring of Cystic Fibrosis changes by chest tomosynthesis and computed tomography 139 Respiratory motion suppression for three-dimensional, high isotropic spatial resolution, magnetic resonance imaging of the lung without contrast agent injection 140 Is bronchoalveolar lavage necessary for monitoring and treating respiratory infections in children with Cystic Fibrosis? 141 Platelet aggregation capability in children with Cystic Fibrosis 142 Immunisation coverage in children with Cystic Fibrosis: A French multicenter survey 143 Seasonality of Pseudomona aeruginosa isolation in patients with Cystic Fibrosis
E. Hatziagorou M. Leichsenring E. Stockley A. M. Silva H. Asgeirsdottir J. Delacoste B. Alden O. Simonova A. Masson M. Vilella
POSTERS
55
37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
144 The impact of Pseudomonas aeruginosa colonization in CF
F. Freitas
145 The efficacy of the inhalatory treatment with tobramycin in controlling Pseudomonas aeruginosa lung infection in patients with Cystic Fibrosis
S. Sciuca
146 Pseudomonas serology can guide aggressive Pseudomonas eradication programmes 147 Causes of failure to eradicate Pseudomonas aeruginosa in patients with CF
C. Pao M. Cohen-Cymberknoh
148 Use of whole-genome sequencing to identify transmission of Pseudomonas aeruginosa between Cystic Fibrosis patients 149 Changes in antimicrobial susceptibility in adult CF patients with chronic Pseudomonas aeruginosa infection treated with daily inhalation of tobramycin or colistin 150 The impact of viral symptoms on rhinovirus positive pulmonary exacerbations in patients with CF 151 Rare and resistant germs in the examination of sputum and throat swab in patients with Cystic Fibrosis. Results of the German working group “Rare germs“ of the benchmarking project 152 Inhaled antibiotics and Candida albicans chronic colonization in Cystic Fibrosis
E. Nash J. Brouwer L. McWeeney C. Schwarz M. Noni
153 Fungal colonisation of the airway in children with Cystic Fibrosis
S. Wilkinson
154 Scedosporium apiospermum isolation in Cystic Fibrosis patients
M. Noni
155 Aspergillus pulmonary infections in children with Cystic Fibrosis (CF): Prevalences in three Scandinavian CF centres
M. Skov
156 ABPA syndrome (ABPAs) in CF: FEV1 decline, infectious exacerbations and BMI before and after the year of diagnosis (index year), a case control study 157 Long-term impact of fungi on pediatric Cystic Fibrosis lung disease 158 Prevalence of Pneumocystis jirovecii in a cross section of patients attending a large UK adult Cystic Fibrosis centre
A. Hector H. Green S. Van Daele
160 Molecular profiling demonstrates clustering of Mycobacterium abscessus isolates in CF patients from a single centre
H. Green
161 The first results of antimicrobial susceptibility of isolates from adult patients with Cystic Fibrosis from the Province of Vojvodina, Serbia
D. Skrbic
162 Bronchial Artery Embolization (BAE) in adults with Cystic Fibrosis: A single-centre case series 163 TIVAD infection and TIVAD related cardiac problems in transplanted CF patients 164 Incidence, management and outcomes following spontaneous pneumothorax in adult patients with CF attending a Regional Centre 165 Management of pneumothoraces in Cystic Fibrosis (CF): A novel approach using an ambulatory suction device (Thopaz™ system, Medela Ltd) 166 The prevalence and aetiology of pleural effusions and empyema in patients attending a regional adult CF centre
M. Marquette K. Colpaert C. Etherington C. Addy D. Peckham
167 Epidemiology and risk factors for CF arthropathy
J. Röhmel
168 Cystic Fibrosis: An ageing condition
M. Phitidis
PHYSIOTHERAPY 169 A new tool to improve inhalation 170 The King‘s experience of Bronchitol®. One UK centre‘s results of Bronchitol® initiation 171 The patients‘ view of nebulised drugs: Knowledge, adherence, and difficulties 172 Implementation of non-medical prescribing (NMP) within the clinical CF setting 173 Cystic Fibrosis patients‘ performance on Modified Shuttle Walk Test
J. Wikström E. Scott S. Lenaghan A. Henry S. Gambazza
POSTERS
159 Genotyping of Achromobacter xylosoxidans in a Cystic Fibrosis (CF) centre
F. De Baets
56
POSTERS 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
174 Are changes in Modified Shuttle Walk Test performance related to changes in clinical status in CF children and adolescents?
C. Cazzarolli
175 Exercise intensity during interactive video game
S. Gambazza
176 Assessment of exercise capacity in patients with Cystic Fibrosis 177 Physical activity, lung function and lung clearance index in children with Cystic Fibrosis 178 Quantifying weight bearing activity in children and adolescents with Cystic Fibrosis
A. Sovtic M. Sahlberg S. Gambazza
179 Assessing for ambulatory hypoxia in adult CF patients
A. Morris
180 Development of a musculoskeletal screening tool for adults with CF identifies common causes of pain and problems that potentially decrease physical activity: A tool for use in annual reviews
B. Button
181 Spinal deformities (SD) in young CF patients: Two years experience of screening and follow up 183 Mycobacterium abscessus: Stand alone hospital admission for agressive physiotherapy and nutritional review can extend periods between toxic IV antibiotic treatments
G. Mamprin J. Forton
GASTROENTEROLOGY/LIVER DISEASE/METABOLIC COMPLICATIONS OF CF/NUTRITION/GROWTH 184 Evaluation of the impact of SERPINA gene mutation on the occurrence of liver damage and cholestasis in patients diagnosed with Cystic Fibrosis 185 Transient elastography measurements in adults with Cystic Fibrosis liver disease
G. Jones
186 Transient elastography can be used to guide monitoring of Cystic Fibrosis related liver disease in adult patients
G. Jones
187 Evaluation of Cystic Fibrosis liver disease and the relation with risk factors in a Romanian centre
I. Ciuca
188 Elongation of the small intestine (without histological changes) in young pigs with experimentally induced exocrine pancreatic insufficiency (EPI) - Used as a model for children with EPI POSTERS
S. Więcek
189 The variability of carbohydrate antigen 19-9 (CA 19-9) levels in Cystic Fibrosis patients 190 Pancreatitis in patients with CF
A. Mößeler M. Leichsenring A. Sojo Aguirre
191 Prospective multinational DIOS survey: Concomitant risk factors and treatment alternatives
A. Munck
192 Clinical practice in Oslo University Hospital on diagnosis and management of distal intestinal obstructive syndrome
J. Dypvik
193 Concentration of calprotectin in the stool of children with Cystic Fibrosis
S. Więcek
194 A French survey on gastro-oesophageal reflux in CF: Need for guidance 195 Use of CGMS (Continuous Glucose Monitoring System) in CF children with impaired or indeterminant glucose tolerance 196 Prevalence of undiagnosed pre-diabetes and diabetes in a UK cohort of young people with Cystic Fibrosis 197 Comparison of FRAX/NOGG with UK Cystic Fibrosis Trust guidance for low bone mineral density in Cystic Fibrosis
J. Languepin E. Kavaliunaite L. Selby K. Greenhalgh
198 The prevalence of ΔF508 in Cystic Fibrosis patients with low bone mineral density in Republic of Macedonia
T. JakovskaMaretti
199 Arterial stiffness in an ageing Cystic Fibrosis (CF) population attending the All Wales Adult Cystic Fibrosis Centre (AWACFC)
M. Choudhury
200 Prevalence of dyslipidaemia in an Irish adult Cystic Fibrosis cohort
M. Harrison
201 Vitamin A deficiency: Prevalence and clinical implications for adult Cystic Fibrosis
M. Harrison
202 Vitamin A intake and serum retinol levels in children and adolescents with Cystic Fibrosis
J. Woestenenk
203 Vitamin D3 deficiency as a predisposing factor for nasal polyposis in patients with Cystic Fibrosis
M. Fotoulaki
204 Decreasing fat-soluble vitamin deficiencies by increased awareness and change of vitamin preparation
A. M. Olesen
POSTERS
57
37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
205 Folate deficiency in adult patients at a regional Cystic Fibrosis (CF) centre 206 Serum zinc concentration in Cystic Fibrosis patients with CFTR I1234V mutation associated with pancreatic sufficiency 207 Cow‘s milk allergy in Cystic Fibrosis children 208 Nutritional intervention among children with CF 209 Changes in nutritional status associated with chest exacerbations in children with Cystic Fibrosis 210 Changes in the nutritional status and dietary intake of adults with Cystic Fibrosis since 1997 211 Overweight and obesity in the Cystic Fibrosis Israeli national center 212 Impact of enteral tube feeding on body fat and lean body mass 213 Dietary management of pregnant women with Cystic Fibrosis 214 Cystic Fibrosis and pregnancy, a single centre case-control study
L. Cassidy A. A. Wahab I. Ciuca E. Hatziagorou C. Smith S. King B. Kochavi H. White D. Grunwald D. Hubert
215 Hand grip strength is associated with disease severity in adults with CF
L. Mead
216 Hand strength, is it time to get a grip?
D. Proud
217 A novel use of BrEaD (breathlessness, eating and drinking) 218 Analysis of an online Cystic Fibrosis forum: Common nutritional concerns and quality of nutritional information shared
M. Rezaie L. Pearson
DELIVERY OF CARE 219 A review of hospital and hospital in the home (HiTH) services at a paediatric Cystic Fibrosis (CF) centre 220 Safety, tolerability and patient preference of intravenous colistimethate sodium delivered by bolus compared to infusion in adults with Cystic Fibrosis
222 Successful administration of continuous intravenous ß-lactam therapy for allergic CF patients failing standard antibiotic desensitization 223 Analysis of “cost drivers“ for health care services use in Cystic Fibrosis patients: The experience of the Milan Cystic Fibrosis Center
E. Nash M. G. Pedersen O. Breuer C. Colombo
224 Improving CF inpatient admissions using a value stream mapping approach
C. Pitfield
225 French CF Quality Improvement Program (QIP): The experience of Rennes adult CF Centre
I. Berhault
226 A work load study to determine the proportion of time spent on delivering rescue vs. prevention clinical care in adults with Cystic Fibrosis
M. Lowther
227 Detecting early markers of CF-related pulmonary exacerbations using home telemetry and sputum biomarkers
E.-F. Ukor
228 Telemedicine videoconferencing - Breaking down the barriers for Cystic Fibrosis (CF) patients living in rural and remote areas
J. Wilson
229 Review of personal hand held record for Cystic Fibrosis children 230 The development of a new quick/easy CF wellness score (Alfred Wellness Score for CF, “AweScore-CF“) to improve delivery of clinical care in the outpatient and inpatient settings suggests patients acclimatise to low lung function 231 A study of treatment activities and perceived treatment burden in adults with Cystic Fibrosis 232 The paediatric Cystic Fibrosis service at the Leeds Children´s Hospital - A patient and parent perspective 233 3 year experience of a new clinic dedicated to the care of screened infants with equivocal CF related diagnoses 234 Cystic Fibrosis: New trends in ophthalmological evaluation
O. Narayan B. Button
M. Mahmood H. Hunjan A. Fall A. M. Plateroti
POSTERS
221 Outpatient Parenteral Antimicrobial Therapy (OPAT) in patients with Cystic Fibrosis
F. Moran
58
POSTERS 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
235 The CF couch potato! Habitual physical activity in an adult CF population
S. Howard
236 The once-daily tacrolimus extended-release formulation provides similar drug exposure in non-CF and CF lung transplant recipients when compared to the conventional twice-daily formulation 237 Thrush - A lesser spotted resident in the CF service?
C. Knoop J. Lee
238 Medication reconciliation in Wales - A problem shared
B. Richards
239 Using collaborative design and 3D printing to co-produce devices for CF care
M. Dexter
240 “Like” our Facebook page? - The use of social media to improve communication
N. Reid
NURSING/PSYCHOSOCIAL ISSUES 241 Totally implantable venous access devices (TIVAD), a survey at the Gothenburg CF-centre
G. Haugen
242 In-house provision of a PICC/Midline service by the CF nursing team
D. Williams
243 Home visits of CF coordinating nurses - Experience of Necker hospital
M. Clairicia
244 Awareness of infection control within Cystic Fibrosis health care - A Scandinavian study
E. J. Hunstad
245 Side effects of oral antibiotics in small children with Cystic Fibrosis
K. R. Jakobsen
246 What do parents know? The results of a questionnaire asking about the process of a CF admission 247 How often do we communicate with parents in the first year following diagnosis?
J. Cowlard
248 Taking sputum samples from small children wiht Cystic Fibrosis: A matter of cooperation
M. Pehn
249 Living with Cystic Fibrosis - A lonely, lifelong work
POSTERS
A. Marote
J. Homme
250 The role of understanding in living with Cystic Fibrosis
S. Counihan
251 Living with Cystic Fibrosis today: Beyond the “Burden of Care“
S. Counihan
252 A comparative international study of health related quality of life in Eastern Europe
U. Borawska-Kowalczyk
253 Looking beyond the here and now - An association of lung function, unemployment and deprivation scores in an adult CF population in Wales
V. Edwards
254 Evaluating the psychological impact of newly implemented infection control measures for nontuberculous mycobacterium
V. Shearing
255 Cystic Fibrosis patients acting as carers for their older relatives - The “cared for“ becoming the carer 256 Mothers with CF: Psychosocial data about an almost unknown group 257 Development of parenthood website and pathway to support people with CF considering pregnancy and parenthood 258 A qualitative study exploring the needs of mothers and fathers with CF 259 Parenting stress in mothers with Cystic Fibrosis 260 A prospective cohort study examining the prevalence of emotional and behavioural difficulties in children of patients with Cystic Fibrosis (CF) 261 Planning for pre-implantation diagnosis: A qualitative study 262 Fear of Progression Questionnaire for caregivers of youth with Cystic Fibrosis (FoP-Q/C) 263 Parents‘ understanding of genetic mutation and the implications for their child‘s CF 264 Grandparental knowledge of Cystic Fibrosis treatment 265 Exploring the pre-hospitalisation needs of parents of children with Cystic Fibrosis 266 “Arresting“ psychological issues for better health outcomes in parents of infants and young children with Cystic Fibrosis 267 Bringing bad news: The diagnosis of Cystic Fibrosis in childhood
E. Nash G. Ullrich K. Bateman C. O‘Leary G. Ullrich S. Lynch S. Järvholm A. Fidika G. Latchford A. Vertommen S. Phillips C. Branch-Smith T. Havermans
POSTERS
59
37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
268 The experiences of young people and their parents in the move from paediatric to adult Cystic Fibrosis services in Bristol: Recommendations for an improved transition pathway 269 When I am grown up - Evaluating the effectiveness of a transition program for adolescents with CF transferring to the adult hospital system 270 Evaluation of parents‘ compliance in the treatment of Cystic Fibrosis children 271 Adherence to medications in Cystic Fibrosis patients: A five-year retrospective analysis
H. Wood A. Morgan N. Rohovyk S. Nasr
272 The impact of disease severity and clinical variation on self-reported adherence
N. Shaw
273 Relationship between self-reported adherence, medication collections and clinical variation in Cystic Fibrosis
H. White
274 Do poor adherers enter behaviour change interventions of inhaled therapy? Exploration of the “healthy complier“ effect
R. Curley
275 Understanding barriers to weight gain, nebuliser use and exercise in CF
A. Milne
276 Adherence scale in an adult Cystic Fibrosis center
E. Baran
277 Can imprisonment be good for patients with CF? 278 Developing patient resources for lung transplantation 279 Proposal of a questionnaire for Cystic Fibrosis patients who have just been transplanted to ensure their safety before the first return home after transplantation 280 VEMSE-CF: Psychosocial characteristics of patients participating in a prospective controlled care research study
F. Frost S. Phillips V. David L. Goldbeck
281 Patient-centered outcomes in CF research
N. Cirilli
282 Patient priorities for research in Cystic Fibrosis: The IPaCOR experience
N. Cirilli
283 Can CF patient registries identify pulmonary exacerbations?
G. Sims
284 Prevalence of Staphylococcus aureus (SA) infection in patients with Cystic Fibrosis in a reference unit
M. Chilet
285 Some Cystic Fibrosis patients do not tolerate tobramycin dry powder inhalation
F. Borella
286 Adult patients with Cystic Fibrosis exhibit an increasing prevalence of chronic kidney disease with increasing age, particularly those with Cystic Fibrosis-associated Diabetes mellitus and/or an organ transplant
S. English
287 Prevalence and importance of urinary incontinence in females with Cystic Fibrosis 288 Airway clearance techniques used by people with Cystic Fibrosis in the UK 289 Ageing in Cystic Fibrosis: A comparison of adult patients attending a tertiary referral centre 290 Clinical characteristics of adolescents with Cystic Fibrosis in the Moscow region (Russia)
M. Strandberg T. Daniels M. Harrison E. Kondrateva
291 Cystic Fibrosis mortality trend in Italy between 1970 and 2011
C. Colombo
292 Specific causes of mortality in CF patients in France 2007-2010
P.-R. Burgel
293 Spectrum of mutations and survival data for adult CF patients from Sofia CF center 294 The experience of adult Cystic Fibrosis centre in Lithuania in 2009-2013
I. Galeva K. Malakauskas
295 CF patients diagnosed in adulthood: Spectrum of referral source
N. Hunt
296 Adult CF diagnosis - Missed childhood opportunities or forme fruste disease?
N. Hunt
297 Arab Cystic Fibrosis patients in Israel
M. Lavie
298 Establishment of a Russian Cystic Fibrosis patient registry
N. Kashirskaya
POSTERS
EPIDEMIOLOGY/REGISTRY
60
MEETINGS OVERVIEW 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
TUESDAY, JUNE 10 08:15 – 17:00 Physiotherapy Short Course - The Use of NIV in Children and Adults with CF - Physiology and Clinical Application**
J2
08:30 – 17:30 ECFS Board Meeting*
R4
WEDNESDAY, JUNE 11 08:30 – 11:00 ECFS CTN Training & Development*
H2
09:00 – 13:45 ECFS Exercise Working Group*
R26
09:00 – 16:15 International Nurse Specialist Group/CF Meeting**
H1
09:00 – 12:30 Physiotherapy Short Course**
J2
09:30 – 12:30 CFE/ECFS Joint Symposium - Access to Personalised Medicine
K1
09:30 – 15:00 ECFS Neonatal Screening Working Group Meeting
G1
09:30 – 16:40 European Cystic Fibrosis Nutrition Group Meeting
R31
09:30 – 16:00 European Psychosocial Special Interest Group (EPSIG) Meeting**
R5+6
11:30 – 15:30 ECFS CTN Training & Development*
J1
12:00 – 16:00 Journal of Cystic Fibrosis (JCF) Editorial Board Meeting*
R4
12:30 – 14:00 International Physiotherapy Group for Cystic Fibrosis (IPG/CF) - Annual General Meeting*
J2
13:15 – 17:00 ECFS CTN Steering Group Meeting*
H2
13:30 – 16:30 ECFS Latin American Meeting**
G2
14:00 – 17:00 ECFS Patient Registry Executive Committee Meeting*
R26
14:30 – 16:30 Physiotherapy Case Presentations
J2
15:00 – 17:00 ACTIVATE-CF Meeting*
G1
17:00 – 18:00 ECFS Annual General Meeting*
K1
17:00 – 18:30 ECFS Patient Registry Training Meeting*
R4
THURSDAY, JUNE 12 12:30 – 14:30 CF Pharmacists Meeting
H2
12:30 – 13:30 ECFS Patient Registry Data Quality Project Group Meeting
H1
13:00 – 14:00 UK Newborn Screening Special Interest Group Meeting*
K1
13:30 – 14:30 ECFS Patient Registry Contributors Meeting
H1
14:00 – 16:00 UK Cystic Fibrosis Trust Meeting*
R5+6
18:00 – 19:30 ECFS Patient Registry Training Meeting*
R5+6
18:30 – 20:30 ECFS CTN Standardisation Committee Meeting*
E4
FRIDAY, JUNE 13 11:00 – 14:00 CF Ageing Group Meeting
R4
12:30 – 14:30 ECFS Diagnostic Network Working Group Meeting
H1
12:30 – 14:30 ECFS Gene Modifier Working Group Meeting
H2
12:30 – 14:30 ECFS Patient Registry Steering Group Meeting*
K1
13:00 – 14:30 ECFS CTN Microbiology Group Meeting*
E4
18:00 – 19:30 ECFS Patient Registry Training Meeting*
R4
SATURDAY, JUNE 14 13:30 – 18:00 ECFS Scientific Committee Meeting - Brussels 2015*
* closed meeting
J2
** extra registration required
MEETINGS PROGRAMME
61
37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
TUESDAY, JUNE 10 08:15 – 17:00
Physiotherapy Short Course – The Use of NIV in Children and Adults with CF – Physiology and Clinical Application**
**extra registration required
J2
08:15 – 08:30 Introduction – Irene Maguire, Galway, IE 08:30 – 09:15 The rationale for NIV in CF: How do patients develop respiratory failure – Brigitte Fauroux, Paris, FR 09:15 – 10:00 Which ventilation modes for CF patients – Brigitte Fauroux, Paris, FR 10:00 – 10:30 COFFEE BREAK 10:30 – 11:15
NIV in sleep and acute respiratory failure – Brigitte Fauroux, Paris, FR
11:15 – 12:00
When to commence NIV: Indications for use, pressures, humidification – Louise Lannefors, Copenhagen, DK
12:00 – 12:45 NIV for airway clearance – Louise Lannefors, Copenhagen, DK 12:45 – 13:15
LUNCH BREAK
13:15 – 14:00 NIV and exercise – Maria Cecilia Rodriguez Hortal, Stockholm, SE 14:00 – 14:45 NIV and inhalation – Louise Lannefors, Copenhagen, DK 14:45 – 15:30 Interfaces – Maria Cecilia Rodriguez Hortal, Stockholm, SE 15:30 – 16:00 COFFEE BREAK 16:00 – 17:00 Case presentations – Louise Lannefors, Copenhagen, DK / Maria Cecilia Rodriguez Hortal, Stockholm, SE / Helen Parrott, London, UK
WEDNESDAY, JUNE 11 09:00 – 12:30 Physiotherapy Short Course – The Use of NIV in Children and Adults with CF – Physiology and Clinical Application**
**extra registration required
09:00 – 09:45 Application of NIV in the paediatric population – Maria Cecilia Rodriguez Hortal, Stockholm, SE 09:45 – 10:30 NIV in complex cases – Helen Parrott, London, UK 10:30 – 11:00 COFFEE BREAK 11:00 – 11:45
NIV end of life and bridging to transplant – Helen Parrott, London, UK
11:45 – 12:20
Panel discussion –
12:20 – 12:30 Close of course – Irene Maguire, Galway, IE
J2
MEETINGS
62
MEETINGS PROGRAMME 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
09:00 – 16:15
International Nurse Specialist Group/CF Meeting**
**extra registration required
Moderators: Vibsen Bregnballe, Aarhus, DK / Kristine Colpaert, Leuven, BE / Ellen Julie Hunstad, Oslo, NO
H1
09:00 – 09:30 Registration, coffee & tea – 09:30 – 09:45 Welcome – Ellen Julie Hunstad, Oslo, NO
Plenary session 1 – Vibsen Bregnballe, Aarhus, DK
09:45 – 10:00 A presentation of the networking activity within SNSG-CF – Käthe Strandner, Gothenburg, SE / Gunilla Haugen, Gothenburg, SE 10:00 – 10:30
Home treatment with intravenous antibiotic – SNSG-CF study – Inger Ervander Grandinsson, Lund, SE / Ellen Julie Hunstad, Oslo, NO
10:30 – 11:00
Awareness of infection control within Cystic Fibrosis health care – A Scandinavian study – Ellen Julie Hunstad, Oslo, NO
Plenary Session 2 – Kristine Colpaert, Leuven, BE
11:00 – 12:30 New knowledge of brain development during adolescence with consequences for young people‘s behaviour – Kristina Berg-Kelly, Gothenburg, SE 12:30 – 13:30 LUNCH BREAK
Plenary session 3 – Ellen Julie Hunstad, Oslo, NO
13:30 – 14:15
The pharmacological strain on a long life living with CF – Espen Solem, Copenhagen, DK
14:15 – 14:45 Adherence to medication after lung transplantation – Ulrika Skogeland, Stockholm, SE 14:45 – 15:00 CF poetry from patient – Käthe Strandner, Gothenborg, SE / Gunilla Haugen, Gothenburg, SE
MEETINGS
15:00 – 15:15 COFFEE BREAK
15:15 – 16:15
INSG-CF Annual General Meeting –
Information from committee/national presentations – Vibsen Bregnballe, Aarhus, DK / Doris Thomsen, Copenhagen, DK
09:00 – 13:45
ECFS Exercise Working Group*
*closed meeting
Moderator: Helge Hebestreit, Würzburg, DE 09:00 – 09:15 Introduction – Helge Hebestreit, Würzburg, DE 09:15 – 09:45 Update on clinical practice guideline on exercise testing – Helge Hebestreit, Würzburg, DE 09:45 – 10:15 Clinical practice guideline on assessment of physical activity – Erik Hulzebos, Utrecht, NL 10:15 – 10:45 COFFEE BREAK 10:45 – 12:15
Clinical practice guideline on activity/exercise counselling – Anne Swisher, Morgantown, US
12:15 – 12:45
LUNCH BREAK
12:45 – 13:15
Proposal of new project: Retrospective study on the predictive value of exercise testing in addition to established predictors – Helge Hebestreit, Würzburg, DE
13:15 – 13:45
Update on ACTIVATE-CF – Helge Hebestreit, Würzburg, DE
R26
MEETINGS PROGRAMME
63
37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
09:30 – 16:00
European Psychosocial Special Interest Group (EPSIG) Meeting**
**extra registration required
R5+6
09:30 – 09:35 Welcome – 09:35 – 10:45 Topic 1: Children of parents with CF Mothers with CF: Psychosocial data about an almost unknown group – Gerald Ullrich, Schwerin, DE
Discussion
A prospective cohort study examining the prevalence of emotional and behavioral difficulties in children of patients with Cystic Fibrosis (CF) – Samual Lynch, Birmingham, UK
Discussion
A discussion about service provision for children growing up with parents who have CF – Samantha Phillips, Bristol, UK
10:45 – 11:00 COFFEE BREAK 11:00 – 12:00 Topic 2: Multidisciplinary roles –
The role of the psychologist – Alicia Vandenoetelaer, Brussels, BE
Discussion –
The rehabilitation centre (case presentation) – Thijs Verbruggen, De Haan, BE
Discussion – 12:00 – 13:30 LUNCH BREAK 13:30 – 14:45 Topic 3: CF today –
The experience of young people of transition – Helen Wood, Bath, UK
Discussion
Living with Cystic Fibrosis today: Beyond the “burden of care“ – Sian Counihan, Limerick, IE
Discussion
15:00 – 15:40 Topic 4: Parents of children with CF “Arresting“ psychological issues for better health outcomes in parents of infants and young children with Cystic Fibrosis – Cindy Branch-Smith, Perth, AU
An internet-based psychological intervention for parents of children with CF – Astrid Fidika, Ulm, DE
Discussion – 15:40-15:50
Topic 5: European standards of care –
Overview of the psychological parts – Mandy Bryon, London, UK
15:50 – 16:00 Closing remarks –
MEETINGS
14:45 – 15:00 COFFEE BREAK
64
MEETINGS PROGRAMME 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
09:30 – 12:30
CFE/ECFS Joint Symposium – Access to Personalised Medicine
Moderators: Jacquelien Noordhoek, Baarn, NL 09:30 – 10:00
Personalised medicine – Stuart Elborn, Belfast, UK
10:00 – 10:30
Comparing Western Europe and Eastern European countries – Milan Macek, Prague, CZ
10:30 – 11:00
COFFEE BREAK
11:00 – 11:30
Giving information on patients’ participation in clinical trials – Ulrike Pypops, Brussels, BE
11:30 – 12:00
Challenges of funding CF care in the next decade – Diana Bilton, London, UK
12:00 – 12:30
Panel discussion –
09:30 – 15:00
ECFS Neonatal Screening Working Group Meeting
Moderator: Kevin Southern, Liverpool, UK
K1
G1
09:30 – 10:00 Tea and coffee – 10:00 – 10:10 Welcome to Sweden – Isabelle de Monestrol, Stockholm, SE 10:10 – 10:25 Update on the progress in Europe – Kevin Southern, Liverpool, UK 10:25 – 10:55 The challenges faced in the US and how the QI programme has helped – Susanna McColley, Chicago, US
MEETINGS
10:55 – 11:25 The history of screening in Australia, how the regional programmes have developed and the impact on CF care – John Massie, Melbourne, AU 11:25 – 11:55
Providing genetic counselling after newborn screening and the impact of CFTR2 – Karen Siklosi Raraigh, Baltimore, US
11:55 – 12:25
The working group guidelines on the management of an inconclusive diagnosis following newborn screening for CF – Anne Munck, Paris, FR
12:25 – 13:15
LUNCH BREAK
13:15 – 13:45
Preparatory studies for the implementation of NBS in CF for Sweden – Isabelle de Monestrol, Stockholm, SE
13:45 – 14:15
Assessing cost-effectiveness of newborn screening for CF – Elske van den Akker-van Marle, Leiden, NL
14:15 – 14:45 CF NBS in Norway, the initial process, the challenges and early results – Olav Trond Storrøsten, Oslo, NO 14:45 – 15:00 Discussion –
09:30 – 16:40
This Meeting has been supported by Perkin Elmer and Dynabio.
European Cystic Fibrosis Nutrition Group Meeting
Moderators: Helen White, Leeds, UK / Dee Shimmin, Belfast, UK R31 09:30 – 10:00 Coffee and registration – 10:00 – 11:00 Annual General Meeting –
Micronutrients and vitamins –
MEETINGS PROGRAMME
65
37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
11:00 – 11:20
Vitamin A intake and serum retinol levels in children and adolescents with Cystic Fibrosis – Willie Woestenenk, Utrecht, NL
11:20 – 11:40
Decreasing fat-soluble vitamin deficiencies by increased awareness and change in vitamin preparation – Anne Mørch Olesen, Aarhus, DK
11:40 – 12:00 Serum zinc concentration in Cystic Fibrosis patients with CFTR I1234V mutation associated with pancreatic sufficiency – Atqah Abdul Wahab, Doha, QA 12:00 – 12:30 Folate deficiency in adult patients at a regional Cystic Fibrosis (CF) centre – Laura Cassidy, Leeds, UK 12:30 – 13:15
LUNCH BREAK
13:15 – 13:45
Pancreatic sufficient patient commenced on PERT undergoing numerous gastro interventions with ongoing abdominal symptoms; Where do we go next? – Veronica Lynch, London, UK
13:45 – 14:15
Dietary management of pregnant women with Cystic Fibrosis – Deborah Grunwald, Paris, FR
14:15 – 14:45 Impact of enteral tube feeding on body fat and lean body mass – Helen White, Leeds, UK 14:45 – 15:00 COFFEE BREAK 15:00 – 15:30 Hand grip strength is associated with disease severity in adults with CF – Lucy Mead, Cambridge, UK 15:30 – 16:00 Hand strength, is it time to get a grip? – David Proud, Cardiff, UK 16:00 – 16:30 Case presentation of a 40-year-old female patient with Cystic Fibrosis and pancreatic sufficiency – Francis Hollander, Utrecht, NL 16:30 – 16:40 Discussion and close –
13:30 – 16:30
ECFS Latin American Meeting**
Moderators: Malena Cohen-Cymberknoh, Jerusalem, IL / Eitan Kerem, Jerusalem, IL /
Stuart Elborn, Belfast, UK
G2
13:30 – 13:40 Introduction and welcome – Malena Cohen-Cymberknoh, Jerusalem, IL / Stuart Elborn, Belfast, UK 13:40 – 14:00 The new ECFS Patient Registry. How can it be used by Latin American centers – Edward McKone, Dublin, IE
14:20 – 14:40 Neonatal screening - Where, when and what to perform and what to do with a positive answer – Milan Macek, Prague, CZ 14:40 – 16:00
Round Tables Group 1: Standards of Care – Malena Cohen-Cymberknoh, Jerusalem, IL / Eitan Kerem, Jerusalem, IL Group 2: Setting up a registry – Edward McKone, Dublin, IE / Francisco Reis, Belo Horizonte, BR Group 3: Neonatal screening – Milan Macek, Prague, CZ / Silvia Gartner, Barcelona, ES
16:00 – 16:20 Rapporteurs (5 min per work group) – 16:20 – 16:30 Meeting wrap-up & close – Malena Cohen-Cymberknoh, Jerusalem, IL / Eitan Kerem, Jerusalem, IL / Stuart Elborn, Belfast, UK
This Meeting has been supported by Novartis.
MEETINGS
14:00 – 14:20 Adaptation of Standards of Care in CF to Latin American countries – Malena Cohen-Cymberknoh, Jerusalem, IL
66
MEETINGS PROGRAMME 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
14:30 – 16:30
Physiotherapy Case Presentations
Moderators: Helen Parrott, London, UK / Ruth Dentice, Sydney, AU
J2
14:30 – 15:00 Physiotherapist versus cough. The role of the physiotherapist in treating multifactorial cough – Nicola Mills, Leicester, UK 15:00 – 15:30 The challenges of managing concurrent autosomal recessive conditions, Morquios and Cystic Fibrosis (CF) – Tamara Lazzarin, South Brisbane, AU 15:30 – 16:00 Can you do too much physio? The challenge of balancing rest with a patient´s overwhelming drive to continue with intensive physio right up to end of life – Beth Underhill, London, UK 16:00 – 16:30 Management of neonate with Cystic Fibrosis (CF) & tracheostomy – Ruth Stewart, Glasgow, UK
THURSDAY, JUNE 12 12:30 – 14:30
CF Pharmacists Meeting
Moderator: Amanda Bevan, Southampton, UK
H2
12:30 – 12:35 Introduction – 12:35 – 13:10
Fosfomycin in practice – Patrick Wilson, Leicester, UK
13:10 – 13:45
Self-reported adherence: What does it tell us? – Nicola Shaw, Leeds, UK
13:45 – 14:15
QT prolongation in CF – Tim Gleeson, Sheffield, UK
14:15 – 14:30 Questions –
13:30 – 14:30
ECFS Patient Registry Contributors Meeting
Moderator: Vincent Gulmans, Baarn, NL
The meeting is open for anyone with an interest in the ECFS Patient Registry. It is an informative meeting for potential contributors, persons with an interest in obtaining data for research and other interested parties.
MEETINGS
13:30 – 13:40 Introduction ECFS Patient Registry – Jacqui van Rens, Leuven, BE 13:40 – 13:50 The annual report and centre report: Useful tools – Anna Zolin, Milan, IT 13:50 – 14:00 Demonstration ECFSTracker, the data collection software – Alice Fox, Verona, IT 14:00 – 14:10 How to set-up a (national) patient registry – Vincent Gulmans, Baarn, NL 14:10 – 14:20 A patient’s view – Ulrike Pypops, Brussels, BE 14:20 – 14:30 Questions & answers –
H1
MEETINGS PROGRAMME
67
37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
FRIDAY, JUNE 13 11:00 – 14:00
CF Ageing Group Meeting
R4
The International Study into Ageing in CF Meeting will be chaired by Dr. Nick Simmonds, Prof. John Dodge and Dr. James Yankaskas. Presentations and discussions will be made on the questionnaires for the ISAC Study, the Cancer Study and also the Gene Modifier Study. This is an open meeting and all are welcome to attend.
12:30 – 14:30
ECFS Diagnostic Network Working Group Meeting
Moderator: Nico Derichs, Berlin, DE
H1
12:30 – 12:45 The STARD criteria for evaluation of diagnostic tests – Isabelle Sermet, Paris, FR 12:45 – 13:15
New sweat tests: Rationale of techniques and first results –
Ratiometric single gland – Paola Melotti, Verona, IT
Evaporimetry – Ahn Thao Nguyen, Paris, FR 13:15 – 13:30
How to contribute to CFTR3? – Sheila Scheinert, Berlin, DE
13:30 – 13:50 Ongoing DNWG projects – 13:50 – 14:10 Short presentation of best diagnostic posters – 14:10 – 14:30 Challenge the experts: CF diagnostic cases from Sweden & participants –
MEETINGS
68
TITLE 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
TITLE
69
37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
EXHIBITION & SATELLITE SYMPOSIA SPONSOR ACKNOWLEDGEMENT ECFS TOMORROW LOUNGE SATELLITE SYMPOSIA EXHIBITION FLOOR PLAN EXHIBITORS PROFILES CF COMMUNITY AREA
EXHIBITION & SATELLITE SYMPOSIA
70
TITLE SPONSOR ACKNOWLEDGEMENT 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
THE ECFS WOULD LIKE TO THANK THE FOLLOWING SPONSORS
PLATINUM
GOLD
EXHIBITION & SATELLITE SYMPOSIA
SILVER
TITLE
71
37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
COME VISIT US AT THE ECFS TOMORROW LOUNGE! New ways of connecting Implementing new innovations Bridging the generations of knowledge Leading experts advising a new generation of specialists A common platform for a multi-disciplinary community Active participation in an international society that is setting European standards The ECFS Tomorrow initiative is specially geared towards assembling those who are interested in building their future career in the Cystic Fibrosis community and the ECFS of tomorrow. We see the ECFS Tomorrow as a means to improve one of the fundamental objectives of the society – To support and engage young scientists working in the field of Cystic Fibrosis. It is essential to provide professionals who are new to the community with opportunities to connect in a meaningful way with current leaders and experts. In turn, the new generation of specialists bring up-to-date approaches and practices to the wealth of experience that already exists within the society’s ranks.
WEDNESDAY, JUNE 11, 20:00 – 21:00
FRIDAY, JUNE 13, 12:45 – 13:45
Meet & Greet
How to establish a research group Dominik Hartl, Tübingen, DE
THURSDAY, JUNE 12, 12:45 – 13:45
SATURDAY, JUNE 14, 10:30 – 11:00
Writing successful grants Alexandra Quittner, Coral Gables, US
Farewell Coffee The ECFS Tomorrow Lounge is supported by:
EXHIBITION & SATELLITE SYMPOSIA
Located near the Session Rooms E on Level 0 of the conference venue, the ECFS Tomorrow Lounge will feature two mini workshops aimed at career development.
72
SATELLITE SYMPOSIA 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
THURSDAY, JUNE 12 12:30 – 14:00 Changing faces, changing solutions Moderators: Barry Plant, Cork, IE / Stuart Elborn, Belfast, UK
12:30 – 12:35
K2 - K3
Welcome and introduction – Stuart Elborn, Belfast, UK
12:35 – 12:45 Overview – Barry Plant, Cork, IE 12:45 – 13:05 The role of international/European registries – Edward McKone, Dublin, IE 13:05 – 13:25
Preventing exacerbations – Elisabeth Tullis, Toronto, CA
13:25 – 13:45
Understanding future challenges of clinical care – Scott Bell, Brisbane, AU
13:45 – 13:55 Panel discussion –
13:55 – 14:00
Conclusions and meeting close – Barry Plant, Cork, IE
12:30 – 14:00
CFTR Modulation in Cystic Fibrosis
Moderator: Jilly Carter, Oxford, UK
E1-E3
12:30 – 12:55
The story of CFTR in Cystic Fibrosis – Claire Wainwright, Herston, AU
12:55 – 13:15
Long-term safety and efficacy of CFTR modulation – Ernst Rietschel, Cologne, DE
13:15 – 13:50
How should we evaluate CFTR modulation? Roundtable panel discussion Gordon MacGregor, Glasgow, UK / Dominique Hubert, Paris, FR / Peter Barry, Manchester, UK / Jaqueline Rendall, Belfast, UK / Claire Wainwright, Herston, AU / Ernst Rietschel, Cologne, DE
13:50 – 14:00 Q&A session –
Final remarks and meeting close –
19:00 – 20:30 Cystic Fibrosis treatment – Past, present and future
EXHIBITION & SATELLITE SYMPOSIA
Moderator: Harm Tiddens, Rotterdam, NL
E1-E3
19:00 – 19:05 Welcome and introduction – Harm Tiddens, Rotterdam, NL 19:05 – 19:25 Cystic Fibrosis treatment in paediatric patients – Harm Tiddens, Rotterdam, NL 19:25 – 19:45 Cystic Fibrosis treatment in adult patients – Christopher Goss, Seattle, US 19:45 – 20:05 Rates of adherence, treatment burden and avenues for intervention – Alexandra Quittner, Coral Gables, US 20:05 – 20:20 Q&A session – 20:20 – 20:30 Closing remarks – Harm Tiddens, Rotterdam, NL
SATELLITE SYMPOSIA
73
37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
FRIDAY, JUNE 13 12:30 – 14:00
Changing antibiotic delivery to improve CF patients’ lives: Challenges and opportunities
Moderator: Anders Lindblad, Gothenburg, SE
12:30 – 12:35
Welcome and introduction – Anders Lindblad, Gothenburg, SE
12:35 – 12:50
New modes of antibiotic delivery: Challenges and opportunities – Harm Tiddens, Rotterdam, NL
K2-K3
12:50 – 13:05 Novel methods of antibiotic administration: What does real-world evidence tell us? – Barry Plant, Cork, IE 13:05 – 13:45
Evolving approaches to antibiotic therapy: Cycled versus continuous administration (Point/Counterpoint discussion)
13:05 – 13:10 Debate introduction and historical overview – Anders Lindblad, Gothenburg, SE 13:10 – 13:25
Microbiologist’s perspective – Juliet Foweraker, Cambridge, UK
13:25 – 13:40 Clinician’s perspective – Diana Bilton, London, UK 13:40 – 13:45 Patient cases discussion – Juliet Foweraker, Cambridge, UK / Diana Bilton, London, UK 13:45 – 13:50 Debate key take-home messages and symposium conclusion remarks – Anders Lindblad, Gothenburg, SE 13:50 – 14:00 Panel questions and answers – 12:30 – 14:00
New approaches to pancreatic enzyme replacement therapy
Moderator: Gary Connett, Southampton, UK
E1-E3
12:30 – 12:40 Welcome – Gary Connett, Southampton, UK 12:40 – 13:00 What we do and don’t know about PERT in infants – Drucy Borowitz, Buffalo, US 13:00 – 13:20 PERT in infants and young children: The choice of product matters – Nataliya Kashirskaya, Moscow, RU 13:20 – 13:45 How can we optimise PERT and improve outcomes? – Gary Connett, Southampton, UK 13:45 – 14:00 Discussion –
EXHIBITION & SATELLITE SYMPOSIA
74
TITLE 37th EUROPEAN CYSTIC FIBROSIS CONFERENCE
Cayston is indicated for the suppressive therapy of chronic pulmonary infection due to Pseudomonas aeruginosa in patients with cystic fibrosis aged 6 years or older.
See the difference
EXHIBITION & SATELLITE SYMPOSIA
Feel the difference
CAYSTON ® PRESCRIBING INFORMATION Presentation: A sterile lyophilised powder and sterile solvent for nebuliser solution. Each powder vial contains 75mg aztreonam (as lysine). Each 1ml solvent ampoule contains 0.17% w/v sodium chloride. Indication: The suppressive therapy of chronic pulmonary infections due to Pseudomonas aeruginosa (PA) in patients with cystic fi brosis (CF) aged ≥6 years. Dosage & administration: Cayston should only be used with the Altera® Nebuliser Handset and Altera Aerosol Head, connected to an eBase Controller or an eFlow® rapid Control Unit. Other medicinal products should not be used in the Altera Nebuliser Handset. Patients should use a bronchodilator before each dose; short and long acting bronchodilators can be taken 15 minutes to 4 hours and 30 minutes to 12 hours prior to each dose of Cayston, respectively. For patients taking multiple inhaled therapies, the recommended order is: bronchodilator, mucolytics and lastly, Cayston. Adults & Elderly: 75mg three times daily for 28 days. Doses should be taken at least 4 hours apart. Cayston may be taken in repeated cycles of 28 days on therapy followed by 28 days off Cayston therapy. Clinical studies of Cayston did not include Cayston treated patients aged 65 years and older to determine whether they respond differently from younger patients. Children & Adolescents: Indicated in children aged 6 years and older with the posology the same as for adults. Dosage is not based on weight or adjusted for age. Not recommended in children younger than 6 years. Contraindications: Hypersensitivity to aztreonam or any of the excipients (L-lysine, sodium chloride, water for injections). Warnings & precautions: Allergic reaction: If an allergic reaction to Cayston occurs (of which rash may be indicative), stop administration. Caution is advised when administering Cayston to patients with a history of allergy to β-lactam antibiotics as cross-reactivity may occur. Aztreonam, a monobactam, is only weakly immunogenic. Although not observed with Cayston, the following rare and severe adverse reactions have been reported after parenteral use of other aztreonam-containing products: toxic epidermal necrolysis, anaphylaxis, purpura, erythema multiforme, exfoliative dermatitis, urticaria, petechiae, pruritus, diaphoresis. Bronchospasm: Bronchospasm is a complication associated with nebulised therapies. Despite pre-treatment with a bronchodilator, an acute reduction of ≥15% in forced expiratory volume in 1 second (FEV1) following administration of study therapy was observed in 3% of patients treated with Cayston and 4% of patients receiving placebo. Haemoptysis: Use of Cayston in paediatric CF patients has been associated with haemoptysis during treatment cycles and could have aggravated underlying conditions. Administration of Cayston in CF patients with active haemoptysis should be undertaken only if the benefi ts of treatment outweigh the risks of inducing further haemorrhage. Other: Effi cacy has not been established in patients with FEV1 >75% predicted, and patients with Burkholderia cepacia isolated from sputum in the previous 2 years were excluded from clinical studies. Aztreonam for injection contains arginine, a substance known to cause pulmonary infl ammation, and must not be used for inhalation. The development of antibiotic-resistant PA and super-infection with other pathogens represents a potential risk with antibiotic therapy, which could limit treatment options on Cayston therapy during acute exacerbations. In clinical studies, an increased prevalence of MRSA, MSSA, Aspergillus and Candida species was observed over time in patients treated with several courses of Cayston. During clinical studies of Cayston, isolation of MRSA did not result in worsening of lung function. Interactions: No interaction studies have been performed. No evidence of any drug interactions with Cayston were identifi ed from clinical studies in which Cayston was taken concomitantly with bronchodiltors, dornase alfa, pancreatic enzymes, azithromycin, tobramycin, oral steroids (
