Bid to extend cystic fibrosis patients’ lifespan

Scientists from the North are hoping to develop new ways of helping cystic fibrosis patients live longer.

Researchers from Ulster University have joined a £750,000 (€885,000) international study to find alternative treatment options for those with cystic fibrosis-related diabetes (CFRD).

Dr Catriona Kelly said: “Discovering how the defective cystic fibrosis gene affects the body’s ability to regulate insulin levels is crucial to working out how to prevent diabetes from developing in CF sufferers.

“This new research brings cystic fibrosis and diabetes specialists together for the first time to identify crucial new treatment options that could enable the majority of cystic fibrosis patients to live longer and healthier lives.”

Cystic fibrosis is a life-shortening genetic condition that causes lungs to become clogged with mucus, making it hard to breathe.

It affects 450 people in the North and 1,200 children and adults in Ireland. About 50 new cases of cystic fibrosis in Ireland are diagnosed each year. Around 55% of the CF patient population in Ireland is aged 18 or older.

Across the UK, 2.5m people carry the faulty gene that causes CF and around one in every 2,500 babies born every year has the condition.

On average, patients do not live beyond their mid-30s but for those who also develop diabetes — almost half of the adults and 20% of adolescents — life expectancy is even shorter.

Dr Kelly added: “We hope to get a clearer understanding of the mechanism and reasons why people with cystic fibrosis develop diabetes because it is very different to the reasons for type 1 and type 2 diabetes.

“By doing that, we are hoping to identify better ways of treating them.

“At the moment we treat using insulin but there may be less invasive ways.”

While the cause of CFRD is unknown, the development of diabetes accelerates lung disease, which is the main reason for death among people with CF.

The new three-year study, the first of its kind, has been funded by the Cystic Fibrosis Trust, and will look at how genetic defects responsible for CF increase blood sugars and the body’s inability to regulate insulin.

It is being led by Newcastle University in partnership with Lund University in Sweden, the University of Iowa in the US and Szeged University in Hungary.

Dr Anoushka de Almedia, head of research at the Cystic Fibrosis Trust, said: “Such a high proportion of adults with cystic fibrosis also live with the added pressures of diabetes.

“We are really excited that we have an opportunity to understand how this additional burden could be prevented for people in the future.

“Research is the biggest single area of investment for the Cystic Fibrosis Trust and it’s wonderful to see projects like this aiming to make such positive progress in our fight for a life unlimited.”

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