People with CF and their Carers

What is CF and how is it treated?

What is Cystic Fibrosis?

Cystic Fibrosis is a genetic disorder that is inherited at birth. It is caused by a faulty gene, and in most cases is picked up at birth through the newborn screening heel prick test. The faulty gene results in the production of abnormal sticky mucous, which builds up in the lungs and digestive system of people with CF, leading to lung infections and digestive problems.1

How it affects the lungs

There is normally a thin layer of mucous lining the inside of our lungs, to catch any bacteria or dust passing by. However, in Cystic Fibrosis, that mucous layer is thick and sticky, clogging up the airways and trapping bacteria, leading to recurrent chest infections.

How it affects the gut

In the digestive tract, the pancreas is an important organ that produces substances called pancreatic enzymes, which are needed to breakdown the food we eat, such as fats, proteins and carbohydrates. In Cystic Fibrosis, the pancreatic ducts become clogged with mucous, trapping these enzymes and stopping them from travelling to the gut where they are needed to digest food. This can lead to important nutrients not being absorbed by the body, causing nutrient deficiencies and malnutrition if left untreated.

How it affects the pancreas

The pancreas also secretes insulin, which is needed to control blood sugar levels. Over time, inflammation and scarring of the pancreas can affect the normal production and secretion of insulin, which could lead to Cystic Fibrosis-related Diabetes, affecting around 30% of adults with Cystic Fibrosis.2

You can find more information on how Cystic Fibrosis can affect the body on the Cystic Foundation Trust website at How does cystic fibrosis affect the body?

How is Cystic Fibrosis Treated?

If you have Cystic Fibrosis, you will be under the care of specialist Cystic Fibrosis team of doctors, nurses, and other health professionals, from a specialist Cystic Fibrosis centre, and will be reviewed and monitored on a regular basis.

Because Cystic Fibrosis can affect the body in several different ways, you may require a range of medications or treatments to support the relevant bodily functions affected. These could include3:

  • Medications targeted at correcting the faulty protein made by the CF gene (called CFTR modulators)
  • Bronchodilators to support the airways
  • Anti-inflammatory medication
  • Mucous-thinning medication
  • Antibiotics if required to treat infections
  • Pancreatic enzyme replacement therapy taken with food to support digestion
  • Vitamin supplements
  • Physiotherapy

Your Cystic Fibrosis specialist team will determine which medications you need and will review and monitor these on a regular basis. More information on different treatment options, alongside diet, mindfulness and exercise tips, can be found on the Cystic Fibrosis Trust website at Cystic fibrosis treatments and medications

  1. NHS Conditions. Cystic fibrosis – NHS (www.nhs.uk) (accessed December 2021)
  2. Cystic Fibrosis Trust. How does cystic fibrosis affect the body? (accessed December 2021)
  3. Cystic Fibrosis Trust Cystic fibrosis treatments and medications (accessed December 2021)