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Cystic Fibrosis


Cystic fibrosis (CF) is a hereditary life-shortening disorder with repeated respiratory infections and malnutrition as main clinical manifestations. The disorder affects about 30,000 - 40,000 people in the EU and is recently estimated to be nearly 100,000 people worldwide. Cystic fibrosis is one of the most common life-shortening, childhood-onset inherited diseases and it is the most common life-shortening genetic disease in Caucasian populations. The average life expectancy has increased and is now around 40 years of age, but the number of days spent hospitalized is very high.


The genetic defect in CF makes patients more susceptible to infections with Pseudomonas aeruginosa (PA) and chronic lung infections with PA are major causes of premature death. PA infections are treated with antibiotics, but the infection often reappears intermittently until the patient becomes chronically infected and PA becomes impossible to eradicate.   


Pseudomonas aeruginosais a ubiquitous environmental bacterium. It can be recovered in common food and in drinking water. Upon entering the body of CF-patients, PA infects the lower airways. The fact that PA is found everywhere and the patient’s higher susceptibility to infections, make it almost inevitable that CF patients will get recurring infections with PA.  


Frequent use of antibiotics is strongly associated with side-effects such as disturbance of the microbiologic flora - lung infections with opportunistic bacteria and fungi are very common in CF patients - toxicity and allergenicity. In addition, bacteria will commonly develop resistance to antibiotics.


Resistance to antibiotics presents a serious threat to all of us. Every year, 3 million Europeans are infected by resistant bacteria and about 50,000 of them die. Many of these deaths can be attributed to the over-use of antibiotics and actions to limit the use of antobiotics seem necessary to stop this problem from escalating.


Link to CF charities, patient organisations and support: