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  Cystic Fibrosis
  What is it


What is Cystic Fibrosis
Cystic fibrosis is an inherited disease caused by a faulty gene. This gene controls the movement of salt and water in and out of your cells, so the lungs and digestive system become clogged with mucus, making it hard to breathe and digest food. More than two million people in the UK carry the faulty gene − around 1 in 25 of the population. For someone to be born with CF, both parents must carry the faulty gene. If both parents have the gene, there is a 25% chance the child will have CF. If both parents carry the gene there is also a 50% chance of the child being a gene carrier and a 25% chance they will not have the faulty CF gene. There is currently no cure for CF but many treatments are available to manage it, including physiotherapy, exercise, medication and nutrition. Each week five babies are born with CF, and two people die. More than half of the CF population in the UK will live past 41, and improved care and treatments mean that a baby born today is expected to live even longer. Symptoms of CF can include a troublesome cough, repeated chest infections, prolonged diarrhea and poor weight gain. These symptoms are not unique to cystic fibrosis. Cystic fibrosis is a complex disease that affects many different organs.

Lungs
It is common for people with CF to experience some problems with lung function, although not everyone is affected. A combination of physiotherapy and medication can help control lung infections and prevent the build up of mucus that damages the lung. To avoid the risk of cross-infection people with CF should not meet or come into close contact with one another.

Digestive system
Cystic fibrosis affects the pancreas because a build up of thick, sticky mucus blocks the ducts, reducing the amount of insulin produced and stopping digestive enzymes from reaching the intestines to aid digestion. This can cause malnutrition, leading to poor growth, physical weakness and delayed puberty. Medication can help compensate for the failure of the pancreas, such as digestive enzymes that most people with CF take with each meal to help digest food. In older people with CF insulin production can become deficient due to increasing pancreatic disease. Some people develop CF-related diabetes mellitus and their blood sugar levels are no longer controlled, although this is rare in children. Common symptoms of diabetes include thirst, hunger, weight loss and an excessive need to urinate, but not everyone will show obvious symptoms.