Cystic Fibrosis

What is Cystic Fibrosis?

Cystic fibrosis is an autosomal recessively‐inherited disorder that affects the respiratory and gastrointestinal systems. The faulty gene impairs the body’s ability to regulate movement of salt and water in and out of cells, which results in the production of a thick, sticky mucus within the internal organs. The lungs and pancreas, in particular, can become clogged with this mucus. This can lead to difficulties digesting food, leading to weight loss and chronic lung infections, which can be fatal¹

Who it affects:
Five babies are born with cystic fibrosis in the UK every week¹. 1 in every 2,500 babies born in the UK have cystic fibrosis and it is estimated to affect 70,000‐100,000 people worldwide²).

Symptoms: 
Poor energy intake is often experienced as a consequence of poor appetite that is worsened by chest infections, abdominal pain and gastro‐oesophageal reflux. Energy requirements are increased in relation to the extent of the disease and the frequency and severity of respiratory infections. A lack of pancreatic enzymes can lead to fat maldigestion and malabsorption and deficiencies in the fat soluble vitamins A, D, K and E³. This can have a detrimental effect on nutritional status. Due to the high energy requirements in cystic fibrosis, fat restriction as a means of controlling these symptoms is not recommended ⁴.

Diet and Nutrition: 
Most people with cystic fibrosis will need to take additional enzymes to help break down their food and provide their body with energy; a treatment known as Pancreatic Enzyme Replacement Therapy or PERT. These enzymes minimise symptoms and nutritional consequences of malabsorption and enable a high fat, high energy diet to be eaten. This may help to improve nutritional status, which has been positively associated with lung function (5).