What is Cystic Fibrosis?

CF affects various areas in human's body not just the lungs- find out more below

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.