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What are the symptoms of cystic fibrosis?

What are the symptoms of cystic fibrosis?

Symptoms usually first develop within the first year of life, but may not appear until later childhood. The severity of symptoms can vary.

Lung symptom

The lungs make thicker sputum (mucus) than normal. This can trap bacteria in the small airways and lead to infection. So, symptoms which typically develop include:

  • Persistent cough which typically produces a lot of sputum.
  • Wheezing.
  • Shortness of breath and breathing difficulties.
  • Recurring chest infections. These can be severe such as pneumonia. Repeated infections can damage the lung and lead to poor lung function.

Gut symptoms

The pancreas normally makes digestive juices which contain chemicals (enzymes). The digestive juices normally flow out from the pancreatic duct into the duodenum and digest food.

In people with cystic fibrosis, thickened secretions block the normal flow of the digestive juices from the pancreas. This can result in food not being digested or absorbed properly, in particular, fatty foods and fat-soluble vitamins (vitamins A, D, E and K). This can cause:

  • Malnutrition leading to poor growth and poor weight gain (even if you have a good appetite and eat a lot, as the problem is with digesting and absorbing the food).
  • Large, smelly, greasy, fatty faeces (stools or motions) occur in about a third of cases.
  • Bloated abdomen.

In about 3 in 20 cases the pancreas functions well and there are no or minimal gut symptoms, and mainly just lung symptoms.

Symptoms sometimes occur at birth

About 1 in 10 children with cystic fibrosis are diagnosed shortly after birth. This is due to a condition called meconium ileus where in some cases the gut becomes blocked with meconium - a thick, dark, sticky substance which is made by the baby's gut before being born. Urgent surgery may be needed to relieve the blockage.

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