European Respiratory Society Monograph, Vol. 35. 2006
When CF was first described the disease was found to be affecting children who died as a result of it within the first few years of their life. However, it became clear that the CF phenotype differs between individuals and this may, in parallel with improved treatment,explain why certain affected individuals appear to have a more beneficial prognosis with prolonged survival. The knowledge regarding CF has increased tremendously during the previous decades. It is now clear that the disease is caused by a mutation in the gene coding for CF transmembrane conductance regulator, an ion channel responsible for chlorine transport in epithelial cells. The treatment has improved substantially and lifetime expectancy has increased from approximately 6 months to 30 yrs. Treatment of airway infections and obstructions, nutritional repletion, anti-inflammatory therapy and lung transplantation have contributed to improve survival outcomes with the possibility of gene therapy soon becoming a probable option.