Meet the scientists!

The disease I work on:

I work on Cystic fibrosis (CF) , the most common inherited disease in the UK. In the UK as a whole, around 1 in 25 people are carriers of the CF mutation and 1 in 2500 babies are born with the disorder. CF is actually most common in Ireland, where 1 in 19 people are carriers of the mutation (carriers don’t suffer from CF but if two carriers have children together, there is a 1 in 4 chance that the child will have the disease).

You might find the idea of mucus quite disgusting but in healthy people it plays the important role of ejecting lots of harmful germs from our lungs and nose. It acts a bit like a conveyor belt or an escalator that stops bacteria from settling down and making us ill.

The problem with CF patients is that they make thick, sticky mucus that can’t eject the bacteria. Essentially, the ‘escalator’ is  stuck. This means that they get lots of bacterial infections, some of which can last over 20 years (!),  and these are the major cause of death (average life expectancy of CF patients is 35).

My research:

I work on the most common and vicious germ offender, a bacterium called Pseudomonas aeruginosa (pronounced Soo-duh-moe-nass ah-eh-roo-gin-oh-sah). Pseudomonas has recently been shown to produce cyanide in the lung of CF patients and I am trying to understand why this is the case, whether the cyanide levels are high enough to be toxic and, if so, whether we can find ways to stop  Pseudomonas  from making it.

Results so far:

So far, evidence from my research suggests that the cyanide is having a significant negative effect on the nose cells of patients, but it’s early days yet and I need to do more experiments in order to confirm my findings!