Cystic Fibrosis

What is cystic fibrosis?

Cystic fibrosis (CF) is one of the UK's most common life-threatening inherited diseases. Cystic fibrosis is caused by a defective gene. As a result, the internal organs, especially the lungs and digestive system, become clogged with thick sticky mucus resulting in chronic infections and inflammation in the lungs and difficulty digesting food.

How common is cystic fibrosis?

Around 10,500 people in the UK have cystic fibrosis; that's 1 in every 2,500 babies born. Cystic fibrosis affects around 100,000 people in the world.

What causes cystic fibrosis?

Cystic fibrosis is a genetic condition. One person in 25 carries the faulty CF gene, usually without knowing; that's over two million people in the UK. If two carriers have a baby, the child has a one-in-four chance of having cystic fibrosis.

What are the symptoms of cystic fibrosis?

In people with cystic fibrosis the lungs make thicker sputum (mucus) than normal, which can trap bacteria in the small airways and lead to infection. Symptoms that typically develop include persistent cough, wheezing, shortness of breath and breathing difficulties and repeated chest infections.

Thickened mucus secretions block the normal flow of digestive juices from the pancreas, which means food can not be 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, a bloated abdomen and tummy aches, constipation and prolonged diarrhoea. Find out more about how the digestive system is affected.

Other symptoms can include sinus infections and nasal polyps. Some adults with cystic fibrosis may also get cystic fibrosis-related diabetes (CFRD), arthritis, osteoporosis and liver problems.

Symptoms usually first develop within the first year of life, but in a minority of cases may not appear until later in childhood or beyond. The severity of symptoms can vary and not all people with cystic fibrosis will have every symptom.

What is the life expectancy of someone with cystic fibrosis?

According to the most recent report from the UK Cystic Fibrosis Registry, based on people with CF who are recorded as alive 2013-2017, half of people born with cystic fibrosis in 2017 would live to at least 47. However, this figure does not necessarily account for significant advances in CF care, including new treatments that are being developed.

How does cystic fibrosis affect daily life?

Cystic fibrosis affects everyone differently, but for many it involves a rigorous daily treatment regime including physiotherapy, oral, nebulised and occasionally intravenous antibiotics, and taking enzyme tablets with food. Some people with CF will have a feeding tube overnight.

Treatment and transplants

People with cystic fibrosis often have to undergo a rigorous daily regime of treatments to stay healthy. This can include taking inhaled and injected drugs to clear mucus and fight infections, taking dozens of enzyme pills to digest food and having physiotherapy morning and night. People with cystic fibrosis may also need a transplant. Lung transplants are the most common type for people with cystic fibrosis, as usually these are the organs most affected by cystic fibrosis. However, some people will have problems with their liver, kidneys or pancreas, and may need these organs transplanted.