BBC News

Cystic fibrosis: Drug breakthrough for NI sufferers


A new drug that will benefit cystic fibrosis (CF) sufferers with the 'Celtic gene' has been approved for use in Northern Ireland.

The drug Ivacaftor, also known as Kalydeco, treats the root cause of CF for people with the G551D gene mutation, which is common in Ireland.

In tests it was found to improve lung function and reduce disease flare-up.

CF is an inherited condition that critically affects the lungs and other organs.

There are about 500 people living with CF in Northern Ireland and about 7% of them carry the mutation, which is sometimes referred to as the Celtic gene.

The disease affects the cells that secrete mucus in the lungs and those that secrete digestive juices in the gut and pancreas, causing these secretions to become thick and cause long-term problems.

Professor Stuart Elborn, director of Queen's University Belfast's centre for infection and immunity, led the original trial for the drug.

"Not only will this breakthrough help patients in Ireland and the UK but it has the potential to change the lives for those with cystic fibrosis around the world," he said.

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