Cystic fibrosis therapy shows promise

Research presented at the North American Cystic Fibrosis Conference has suggested that a drug combination which enabled the production of thinner mucus could slow down the progression of the disease.

Data shows one in every 2,500 in the UK has cystic fibrosis.

People who suffer from cystic fibrosis, a genetic condition, produce a thick layer of mucus that clogs and inexorably damages the lungs. However, early studies have suggested that the drugs lumacaftor and ivacaftor could alter the lung cells so they make runnier mucus.

The research followed patients for two years and found that on average patients left untreated would expect their lung function to decline by 2.3 per cent, while those who received the therapy experienced a smaller 1.3 per cent loss of lung function.

Dr Michael Konstan, who led the trial from the Case Western Reserve University School of Medicine, said: "These data suggest that the benefits of lumacaftor/ivacaftor are sustained and indicate that the medicine may modify the progression of cystic fibrosis lung disease by treating its underlying cause."

Currently the therapy is not available on the NHS, after it was previously rejected by funding bodies in England and Scotland.