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 1 in 10,000 people in the EU and nearly 70,000 people worldwide. Cystic fibrosis is one of the most common life-shortening, childhood-onset inherited diseases and in Caucasian populations, it is the most common life-shortening genetic disease. 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 aeruginosa is a ubiquitous environmental bacterium. It can be recovered in common food and in drinking water. Upon entering the body, 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 (infections with opportunistic bacteria and fungi are very common in CF patients), toxicity and allergenicity. In addition to the side-effects, 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 and about 50,000 people die. Many of these deaths can be attributed to the over-use of antibiotics and limiting actions seem necessary to stop this problem from escalating.

Link to CF charities, patient organisations and support:

www.cellscience.com/CFCharities.html