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Cystic Fibrosis (CF) is the most common lethal inherited disease in the UK, affecting around 9,500 people nationally and over 90,000 worldwide. Patient’s lungs become filled with thick sticky mucus and they are vulnerable to recurrent chest infections, which eventually destroy the lungs.  The limited life-expectancy in cystic fibrosis relates to early/recurrent bacterial infections, inflammation and irreversible fibrosis, leading to respiratory failure. Pseudomonas aeruginosa (Pa) is the bacterium linked most closely with this, infecting almost two in three people with cystic fibrosis by young adulthood.

One of only a few  funded by the , the Centre, led by NHLI's , seeks to address three major challenges posed by Pseudomonas aeruginosa:

• better detection
• better understanding
• better treatment

The Centre brings together an international group of academics and clinicians working across a number of hospitals and research institutions.

Projects and aims

Non-invasive detection methods

Aim: To investigate and optimise novel, non-culture based methodologies for the early identification of airway infection

Phenotypic adaptation of Pseudomonas aeruginosa to the CF lung and host defence mechanisms
Aim: To understand phenotypic and genotypic changes favouring survival within the CF airway, and explore variability within and between patients

Identifying and detecting Pseudomonas aeruginosa infection and persistence in cystic fibrosis patients
Aim: To understand bacterial characteristics favouring chronic infection and developing synthetic biosensors to detect these phenotypes

Exploring the therapeutic potential of anti-pseudomonal bacteriophages 
Aim: To take an optimised mix of anti-Pa bacteriophages through to a first in man clinical trial in adults with CF

The development of antibiofilm molecules
Aim: To investigate the potential of NO-donor agents in Pa biofilm dispersal