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Cystic Fibrosis

What is cystic fibrosis?

Affecting one in every 2,500 babies born in the UK, people with cystic fibrosis typically have problems breathing as their lungs become clogged with thick mucus. The condition can worsen over time, with both the lungs and the digestive system struggling to work properly due to the mucus.

Causes of cystic fibrosis

Cystic fibrosis is caused when a child inherits a faulty gene from both parents. As a result, some cells are not able to move water and salt across the cell wall as they should.

It is only when the child inherits a copy of the faulty gene from both parents that cystic fibrosis may occur – the parents don’t normally have the condition themselves. In these circumstances, there’s a 25% chance of the child being born with cystic fibrosis.

What are the symptoms?

Symptoms begin soon after birth or in early childhood. They include recurring chest infections, as bacteria can infect the mucus in the lungs; continual inflammation of the airways, causing them to become widened – known as bronchiectasis; coughing; shortness of breath and wheezing.

The mucus may block the digestive system, affecting the way in which food travels through the gut – this can lead to bowel obstruction soon after birth that may require an operation. People with cystic fibrosis may struggle to gain weight due to malnutrition, as their body struggles to absorb nutrients. They may have diarrhoea and in late childhood become diabetic if their pancreas becomes damaged.

Other symptoms can include sinusitis; nasal polyps; arthralgia or arthritis in late childhood; difficulty in conceiving children in adulthood; liver problems and stress incontinence.

Cystic fibrosis diagnosis

Diagnosis can take place before any symptoms appear, thanks to screening tests that can be carried out soon after birth. Newborns can have a blood spot test to detect problems early on – a small amount of blood is taken from their heel. Tests can also be carried out on adults to find out if they are a “carrier” of the faulty gene.

If doctors think a baby may have cystic fibrosis, further tests can be carried out, including a sweat test – the sweat of a person with cystic fibrosis will have higher levels of salt – and a genetic test of saliva or blood to check for the faulty gene.

Can you prevent cystic fibrosis?

Cystic fibrosis cannot be prevented, as it is a genetic condition. However, people who have cystic fibrosis can enjoy a good quality of life, thanks to the treatments available.

Treatment of cystic fibrosis

Support is available from a team of healthcare professionals at specialist centres, where a care plan will be prepared to meet individual needs. There are several treatments available, including periods in hospital and regular medical appointments to monitor the condition.

Medication is available to treat lung problems and health professionals will teach airway clearance techniques to help remove mucus from the lungs. If the condition progresses over the years so that it becomes severe, affecting normal lung function, then a lung transplant can be carried out.

Nutritional and dietary advice will be provided and treatments for associated problems, such as diabetes, are in the form of insulin medication and a special diet.

With the help of modern medicine and healthcare, cystic fibrosis can be controlled.

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