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Cystic Fibrosis

Cystic Fibrosis (CF)

Affecting over 8,500 people in the UK, CF is one of the UK’s most common life-threatening inherited diseases. It is caused by a faulty gene that controls the movement of salt in the body.

Symptoms

Excessive mucus produced by the body affects the lungs and digestive system. This can cause coughs, chest infections, diarrhoea and difficulty in gaining weight. It also affects the pancreas which makes digesting food difficult. This in turn can lead to malnutrition, weakness and poor growth, as well as delayed puberty. Pancreatic disease can also interfere with insulin production, resulting in diabetes. People who have CF are also more likely to have fertility issues and to develop bone disease and liver problems.

Diagnosis

CF is usually diagnosed by screening. This can be carried out through antenatal testing (offered to mothers who are at a high risk of having a baby with CF), carrier testing (a mouthwash test taken to find out if you are a carrier) or newborn screening (using a heel prick to take a blood sample).

Treatment

Treatment can include:

  • Physiotherapy to clear the mucus from the lungs
  • Enzyme pills to help with digestion. Diet is also important to maintain a good body weight needed to fight infections
  • Exercise, to keep the lungs healthy and improve strength
  • Medicines to improve lung function and improve bone health. Antibiotics are frequently needed to fight infections

Outlook/prognosis

Living with CF can be challenging and it is important to have appropriate support to make sure you have the best quality of life. You will need to have care from a range of healthcare professionals at a recognised CF centre such as the Princess Grace Hospital. Charities, including the Cystic Fibrosis Trust, can also help with information and advice.