Information for people living with CF

CF affects various areas in human's body not just the lungs

CF BODY- pic

LUNGS

  • In healthy lungs, there is a thin layer of mucus that helps your body move dirt and bacteria out of the lungs. In people with CF, this mucus is much thicker and clogs the lungs, creating the perfect environment for harmful bacteria.

  • This means that people with the condition can experience persistent coughing or wheezing, and produce thicker, stickier mucus (sputum). There is also a lack of airway surface liquid, so clearance of these sticky secretions is less effective.

  • People with CF may also have restricted lung function due to the thickening of the walls of the airways, narrowing of the space inside the airways, damage to the airways and obstruction of the airways with mucus.

THE DIGESTIVE SYSTEM

There are many different digestive issues caused by CF

  • Pancreatic insufficiency

  • Poor absorption of fat soluble vitamins DEKA

  • Liver fibrosis

  • Increased risk of bowel cancer

  • Distal intestinal obstruction syndrome (DIOS)

  • Bowel obstructions

  • Constipation

  • Gastro oesophageal reflux disease (GORD)

  • Low BMI’s

  • Potential need for supplementation either orally or via a PEG

THE PANCREAS

The pancreas creates enzymes. Enzymes are chemicals which break down fats, proteins, and carbohydrates in your food. In CF the small tubes which transport these enzymes become blocked with mucus. The enzymes build up in the pancreas instead of reaching the digestive system. This causes the pancreas to become inflamed. CF patients often taken supplementary enzymes (CREON) this helps them digest their food. Those who's pancreas is effected in this way are known as ‘pancreas insufficient’.

CYSTIC FIBROSIS RELATED DIABETES

  • Secondary to inflammation and scarring of the pancreas insulin production can be affected results in Cystic fibrosis related diabetes (CFRD).

  • CFRD is distinct and different from type 1 and type 2 diabetes, but has features of both.

  • Data from the UK CF Registry 2018 indicates that more than one-third of people with CF aged 16 and over are being treated for CFRD.

  • In March 2019, NHS England announced that the Freestyle Libre Flash Glucose Monitoring Sensor will be available to people with cystic fibrosis on insulin treatment. The wearable sensor relays glucose levels to a smart phone or e-reader reducing the need for inconvenient and sometimes painful finger prick blood tests.

THE LIVER

  • Liver disease in people with CF can be related to problems with the drainage of bile out of the liver via the bile ducts and gallbladder, or to scarring in the liver, which is known as fibrosis and cirrhosis.

  • Around 40% of people with CF will have some liver abnormalities, although only around 5–10% of the CF population will experience problems with their health as a result.

Useful websites

About CF

The CF trust The Cystic Fibrosis Trust is the UK's only national charity dealing with all aspects of Cystic Fibrosis www.cftrust.org.uk

The CF foundation The CF Foundation is the leading organisation in the United States devoted to cystic fibrosis www.cff.org

CF worldwide Cystic Fibrosis Worldwide (CFW) is dedicated to improving quality of life and life expectancy for persons living with cystic fibrosis globally www.cfww.org

Ecorn CF Ecorn-cf allows patients, doctors and other CF team members easy access to expert knowledge and advice for cystic fibrosis. www.ecorn-cf.eu

Genetic Alliance UK is the national charity of patient organisations with a membership of over 130 charities supporting all those affected by genetic disorders.www.geneticalliance.org.uk

NHS choices (CF section) NHS information about CF www.nhs.uk/conditions/cystic-fibrosis/Pages/Introduction.aspx

Right now across the UK, there are around 6,500 people in need of an organ transplant, including around 150 children and teenagers. On average three people die every day in need of an organ transplant because there just aren’t enough organ donors.

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Fundraising

We are always amazed and extremely grateful when people go out of their way to raise money for cystic fibrosis services. Some people are motivated by a family member or friend living with CF, and some people have heard about the disease in the press and offer to help.

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