Over the past years significant progress has been made in the field of cystic fibrosis medications, with pancreatic enzymes, H2-histamine inhibitors in order to reduce gastric acidity and enhance fat absorption, and aggressive antimicrobial and anti-inflammatory treatment, along with CFTR potentiators starting to appear as potential drugs leading to a great improvement in life expectancy of CF patients. This evidence concerns the gene CFTR and cystic fibrosis.