Cystic Fibrosis Ireland is currently supporting a number of research projects. Click on a title from the list below to jump to an overview of your chosen study:

1. Exploring the Experience of People with Cystic Fibrosis and their Partners on the Journey Towards Parenthood and its Impact on Psychological Well-being
2. Pathways - Pathways to Improving Transition to Adult Services & Enhancing Self-Management in Young People living with Cystic Fibrosis
3. RECOVER, Real World Clinical Outcomes with Novel Modulator Therapy Combinations in People with CF
4. Cystic Fibrosis Liver Disease (CFLD); predicting and prescribing in the era of CFTR modulators
5. Testing for bacterial genes – Can it help find early infection in Cystic Fibrosis?
6. CF Telehealth
7. Exploring diet quality in Cystic Fibrosis
8. An overview of diet-related cardio-metabolic risk factors in adults with Cystic Fibrosis in an era of modulator therapy
9. Irish Comparative Outcome Study of Cystic Fibrosis (ICOS) - Part 2
10. Exploring the experiences of people with Cystic Fibrosis and the healthcare professionals involved in their care during the pre-conception to post-partum period

Exploring the Experience of People with Cystic Fibrosis and their Partners on the Journey Towards Parenthood and its Impact on Psychological Well-being

Pathways

Pathways to Improving Transition to Adult Services & Enhancing Self-Management in Young People living with Cystic Fibrosis

Principle Investigator: Dr Caroline Heary from NUI Galway

This research seeks to support adolescents with Cystic Fibrosis (CF) to successfully manage their health, as they prepare for their transition to adult services. Young people with CF in Ireland face a changed clinical context with the introduction of modulator drugs such as Kaftrio. Yet, there are many unknowns associated with these treatments. A shift in clinical care is anticipated, with less demand for inpatient care and a move towards an outpatient model of care. The impact on overall selfmanagement remains unknown. A mixed-method approach will be used to build an evidence-base to enhance transition readiness & support self-management in youth with CF. In 2023 Angeline Traynor joined the project team as post-doctoral researcher. The project team have established a youth advisory group to support the project and have applied for ethical approval. Work is underway to begin recruitment for the study in 2024. Click here for more information on this project.

RECOVER, Real World Clinical Outcomes with Novel Modulator Therapy Combinations in People with CF

Led by Professor Paul McNally

RECOVER is a large, multi-center study which is taking place in eight paediatric and adult sites across Ireland and the UK. Initially RECOVER was due to take place over a three-year period but given the success of the study, the team have applied for a five-year extension. Funded by CF Ireland, the CF Foundation (US) and the CF Trust (UK), the study is examining in detail the impact of the new triple combination CFTR modulator compound, Elexacaftor/Tezacaftor/Ivacaftor (ETI), on the lives and health of people with CF. RECOVER is not a clinical trial as it is examining the effects of ETI only after it has been prescribed for patients by their medical team. The project gathers both routine health data and less commonly used clinical endpoints such as lung clearance index, Chest CT, gastrointestinal symptoms, inflammation and medication adherence, providing unique insights into the effects of the triple combination drug. The project is run in conjunction with the CF registry of Ireland and the UK CF registry. For more information on RECOVER, click here.

Cystic Fibrosis Liver Disease (CFLD); predicting and prescribing in the era of CFTR modulators

Principle Investigator: Dr Emer Fitzpatrick, Dr Marion Rowland UCD

Life expectancy for people with Cystic Fibrosis (PWCF) has improved dramatically over the last two decades. This improved survival has not been observed to the same extent in those with CF liver disease (CFLD). The team propose to use data collected as part of the ILSCFLD to address two inter-related questions regarding this poorly understood disease. The first is about prediction of risk. Currently no useful validated prediction tool for CFLD exists. They will develop a predictor model using the data collected on > 800 children over the last 15 years as part of the ILSCFLD. This tool would help identify those at risk of CFLD who may benefit from early management and thus potentially avoiding complications. They will then validate this tool in a cohort of PWCF in the UK. The second question addresses the influence of CF Transmembrane Conductance Regulator (CFTR) modulators on (i) the development of liver disease and (ii) on those who have already established CFLD. Again, using the dataset collected over the last 15 years prior to and during the emergence of the CFTR modulators, they will examine changes in prevalence of liver disease, and outcomes of those with CFLD who have been started on these agents.

Testing for bacterial genes – Can it help find early infection in Cystic Fibrosis?

Led by Dr. Zina Alfahl, University of Galway

We are carrying out a study in the Children’s CF unit in Galway University Hospital (GUH) to develop tests for bacterial genes in samples from children with CF. We hope that testing for bacterial genes will help find bacteria causing infection in the lung of people with CF before they can be found with the methods that are generally used at the moment. I (Dr Zina Alfahl (Lecturer in Bacteriology, University of Galway) am leading the study with support from Professor Martin Cormican (Professor of Bacteriology, University of Galway & Consultant Microbiologist, Galway University Hospital) and Dr Mary Herzig (Consultant Paediatrician, Galway University Hospital. We are able to do this study with the support of parents and children who attend the CF clinic in GUH who agreed that we can use leftover material after their usual tests are complete. Thanks also to the staff of the Microbiology Laboratory in Galway University Hospital for storing the leftover material and preparing it for use in the study. To find out more about this  study, click here.

CF Telehealth

Principle Investigator: Dr. Tamara Vagg Postdoctoral Researcher and Prof. Barry Plant, UCC

Telehealth in its simplest terms is a virtual consultation between a patient and their care team. Dr Tamara Vagg, Prof. Barry Plant and team conducted a research study that looked into what PWCF and the CF team at Cork thought of telehealth, and had some very positive results. They also received some great suggestions on where else telehealth could be used to support PWCF and their families. Since then, Dr Vagg has undertaken a number of in-depth literature reviews, and learnt a lot about CF telehealth but there still seems to be a lot that the CF scientific community don’t know about guidelines and how to setup a telehealth service. To learn more about this, the team set up a European Cystic Fibrosis Symposium (ECFS) working group called the Telehealth for CF Working Group (THCF), their webpage can be found here https://www.ecfs.eu/telehealth.

The main aim of this group is to try and create guidelines for using telehealth within CF care by including as many perspectives as possible. The group is made up of many different expertise including medical, healthcare, psychology, digital health, registries, patient associations, and PWCF. The overall objective is to investigate how telehealth can complement CF care in a post-covid era. The first aim will be to identify generalisable implementation strategies that can aid CF centres developing their own hybrid models.The next aim will be to investigate which aspects of telehealth can be harnessed to further compliment CF care. For more information, click here.

Exploring diet quality in Cystic Fibrosis

Prof. Audrey Tierney (Primary Investigator) and Mr Cian Greaney (PhD student) from the University of Limerick

The overall aim of the study is to provide a comprehensive evaluation of habitual dietary intakes, dietary patterns and overall diet quality of persons with CF living in Ireland and to investigate the interrelationships between diet quality and patient reported outcome measures. Through online focus groups the experiences and view on nutrition and diet in this cohort, including drivers of food and dietary choices, and investigating enablers and barriers for dietary changes will be explored. In 2023, the team completed recruitment and data collection for the study. The final results are being analysed and will be available in early 2024. The team published a systematic literature review in 2023: Greaney, C., et al. (2023). “What do people with Cystic Fibrosis eat? Diet quality, macronutrient and micronutrient intakes (compared to recommended guidelines) in adults with cystic fibrosis – A systematic review.” Journal of cystic fibrosis. The provisional findings on dietary intakes and quality were also presented at the European Cystic Fibrosis conference in Vienna in June 2023, for which Cian Greaney won a best poster award. Greaney, C., et al. (2023). “P339 Diet quality assessment of adults with cystic fibrosis – comparison to population dietary guidelines: a cross-sectional study.” Journal of Cystic Fibrosis 22:S168.) Click here for more information on thtis study. 

An overview of diet-related cardio-metabolic risk factors in adults with Cystic Fibrosis in an era of modulator therapy

Researcher: Clodagh Landers, Senior Dietitian, National Referral Centre for Adult Cystic Fibrosis, St. Vincent’s University Cystic Fibrosis

Supervisors: Clinical supervisor: Dr Suzanne Carter (Consultant Respiratory Physician, CF National Referral Centre, SVUH Academic supervisor: Professor Clare Corish, Professor of Human Nutrition and Dietetics, in School of Public Health, Physiotherapy and Sports Science, UCD

Currently, there is limited evidence in PWCF on how dietitians should best approach the changing dietary needs of PWCF, particularly, the requirement for a healthy balanced diet with a focus on nutrient quality over quantity. As life expectancy in CF improves, it is critical that we fully understand the prevalence of nutrition related cardio-metabolic risk factors in adults with CF to ensure that dietetic practice and research is in line with the changing clinical needs and priorities of this cohort. Aim: The proposed research will aim to explore the body composition and cardio-metabolic status of adults with CF who are on CFTR modulator therapy for at least 3 months.

Irish Comparative Outcome Study of Cystic Fibrosis (ICOS) - Part 2

Led by Professor Patricia Fitzpatrick

This study is led by Professor Patricia Fitzpatrick, Full Professor of Epidemiology and Biomedical Statistics at University College Dublin (UCD), and her research team at UCD, in collaboration with Paediatric Cystic Fibrosis Consultants and nurses from six specialist CF hospitals: University Hospital Limerick, University Hospital Galway, Cork University Hospital, Children’s Health Ireland at Crumlin, Children’s Health Ireland at Temple St and Children’s Health Ireland at Tallaght. This study is funded by the HRB. The study requires consenting parents to allow the researchers to access their child’s medical charts and CF Registry data, and to complete cost and quality of life questionnaires over the telephone/by post/email. All data is totally confidential. Click here for further information on this research project or to register your interest in taking part.

Exploring the experiences of people with Cystic Fibrosis and the healthcare professionals involved in their care during the pre-conception to post-partum period

In September 2020, Ms Jen Balfe, PWCF, received a four-year scholarship from the Irish Research Council, under the Enterprise Partnership Scheme which is co-funded by CF Ireland. CFI will provide €24,000 in funding to support this work over three years. Jen is supported by a supervisory team of Dr Aisling Walsh (Royal College of Surgeons Ireland) and Dr Jennifer Donnelly (Rotunda and RCSI). The project aims to explore the experiences of PWCF and the healthcare professionals (HCPs) involved in their care, as they navigate fertility and pregnancy journeys in Ireland. Qualitative and quantitative data will be collected through interviews and questionnaires. A PPI group are working closely and collaboratively with the research team with their input sought and welcomed at all stages of the project. In 2023, Jen completed a systematic review exploring the barriers and enablers experience by people with Cystic Fibrosis and their healthcare professionals in accessing and utilising and delivering maternity and Cystic Fibrosis care during the pre-conception to post-partum period. Click here for more information on this study.