Current Research Projects
The CFAI is currently joint funding four research projects through the MRCG/HRB Joint Funding Scheme. A synopsis of each of these research projects is outlined below:
1. The Role of Mucus and Mucins in Mediating Pseudomonas aeruginosa Colonisation of the Cystic Fibrosis Lung
Principal Investigator: Dr. Marguerite ClyneInstitution: UCD School of Medicine and Medical Science
Co-Applicants: Dr. Valerie Urbach, National Childrens Research Centre, Dr. Paul McNally, Our Lady’s Children’s Hospital and Dr. Barry Linnane, Mid Western Regional Hospital, Limerick.
Summary
Individuals with CF get bacterial infections in the lung which leads to a decline in lung function and to respiratory failure. Pseudomonas aeruginosa, a bacteria which causes a lot of infection in CF patients, lives in mucus that accumulates in the lungs of people with CF. In this environment it forms biofilms, clusters of bacteria coated in material that holds them together, which makes the bacteria very difficult to treat with antibiotics. We aim to study how the bacteria behave when grown in the laboratory with cells that secrete mucus similar to the mucus in the lung. We will determine the role of mucus in promoting biofilm formation by the bacteria and in developing antibiotic resistance. Binding of bacteria to mucins, large proteins present in mucus, is thought to be a mechanism that helps the bacteria to prevent being removed by the natural defense system of the patient. We aim to test the potential of purified mucins to prevent bacteria from sticking to mucus. By determining the role of mucus and of specific components of mucus in promoting bacterial infection and the formation of biofilms we can look at ways to develop new molecules, which would be alternatives to antibiotics that would prevent infection.
2. National Prevalence of Depression and Anxiety in Patients with Cystic Fibrosis and Parents: Impact on Health and Quality of Life
Principal Investigator: Prof Eileen SavageInstitution: UCC
Summary
Details to follow shortly
3. Gender differences in muscle dysfunction and electrical muscle stimulation in CF
Principal Investigator: Prof Charles GallagherInstitution: Department of Respiratory Medicine, National Referral Centre for Adult Cystic Fibrosis, St. Vincent's University Hospital, Elm Park, Dublin 4
Summary
On average, females with cystic fibrosis have more severe disease than males and their life expectancy is less than males. Our recent research suggests that this may be partly related to greater abnormalities in muscle function in females. The first part of this proposal will study leg and respiratory muscle function in adults and children with CF and will examine the role of muscle function in causing symptoms and disability in CF. The results in females will be compared to results in males.
The second part of the study will examine methods to improve muscle function, exercise capacity and symptoms in adults and children with CF. We will use a method of electrical muscle stimulation that has been shown to be of benefit in patients with other diseases but has not been previously studied in cystic fibrosis.
The results of these studies in Ireland will be compared to results from our collaborators in Montreal, Canada. This is a collaborative research proposal involving adult and paediatric cystic fibrosis units in Ireland, University College Dublin and McGill University in Montreal.
4. Genetic Modifiers of the CF Phenotype
Principal Investigator: Dr Ed McKoneInstitution: Department of Respiratory Medicine, National Referral Centre for Adult Cystic Fibrosis, St. Vincent's University Hospital, Elm Park, Dublin
Summary
This project was initiated in 2007, on the premise that some patients with Cystic Fibrosis have very severe disease while others can have relatively mild disease, even if they have the same CF genotype. This suggested that the disease severity is influenced by genes or environmental factors that influence CF lung function, irrespective of CF genotype. The purpose of this proposal was to consolidate an existing database of clinical and genetic information from over 400 Irish CF patients, from CF clinics in Dublin and Belfast. DNA is being collected from CF patients, as well as detailed clinical information regarding lung function, nutritional status and exposure to infection. In collaboration with the University of Washington in Seattle, and using web-based genomic resources, large scale genetic association is being carried out in over 700 CF patients, to identify novel genes that influence CF function. This research may lead to new information about why certain patients get severe CF lung disease and others do not. This information could then lead to new targets for future CF therapies.
Click here for a full list of research projects previously funded by the CFAI.
