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Cystic Fibrosis Airway Disease

Cystic fibrosis (CF) is the most common inherited disease affecting the developed world, with one in every 2,500 children affected. It is a multi-organ disease, affecting the lungs, gut, liver, pancreas and reproductive tissues. At present about half of those with CF will die by their late 30s from lung disease. Lung disease is the cause of death in over 90% of CF patients.

CF is a genetic (inherited) disease. 1 in 25 people with caucasian ancestry can carry a defective CF gene (often without knowing it, as there are no symptoms if only one defective gene is present). For a baby to be born with CF, both parents have to have a defective CF gene. However not all babies born to two parents with a defective CF gene have CF. There is usually a 25% chance that the baby will have CF; a 50% chance of having one defective gene, but not CF; and a 25% chance of not having any defective CF gene present (meaning that the baby will not have CF, nor any risk of passing it on to their own children).

With CF, the major contributor to worsening health and early death is lung disease. CF results in ongoing loss of lung function due to repeated airway infection and lung tissue damage.

No cure is available. Current treatments are very short lived and can only slow the inexorable destruction of the lung. The lung infections in CF patients continue to be extremely difficult to treat, even with the strongest antibiotics and most innovative therapies.

Complications related to CF can lead to the destruction or malfunctioning of other organs in the body, while the drugs used to treat CF can have debilitating side effects.

Find out more about what it is like to live with CF, or discover how a gene therapy approach to curing CF airway disease is being developed.

More information on CF is available at: www.nlm.nih.gov/medlineplus/cysticfibrosis.html (MedlinePlus, National Library of Medicine, US)

Cystic fibrosis is the most common inherited disease in the developed world, with one in 2,500 children affected.

About half of all people affected by cystic fibrosis will die by their late 30s from lung disease.

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