Cystic fibrosis

Cystic fibrosis, also known as mucoviscidosis, CF, and 65 roses, is a condition a person may get from their parents. It makes the body produce thick, sticky mucus, which builds up in the lungs, the digestive system, pancreas, and other parts of the body. It also affects the bones.

People with cystic fibrosis need yearly checks for CF-related diabetes in addition to all of the problems it can cause.

If both parents have the cystic fibrosis gene, and pass it into their child, the child will have cystic fibrosis. A cystic fibrosis gene is needed from each parent. The parent might not have cystic fibrosis but still might have the gene. A person with cystic fibrosis is not contagious (they cannot give it to anyone else).

There is no cure for cystic fibrosis, but there are a lot of medications that help keep people healthy. 1 out of every 3,000 northern Europeans are affected by CF.

What CF does to the body

Cystic fibrosis affects the entire body. Overall, the body does not secret enough Cl- and has trouble moving salt to the parts of the body that need it. Since the body has trouble moving salt, it piles up in places it is not supposed to like the lungs, stomach and intestines. This is called salt retention, and it means the body doesn't or can't remove salt from the body via the urine (pee).

Lungs

When salt gets stuck in the lungs it causes there to be less water, which makes mucus become very thick. It becomes very hard to breathe.

Treatment

There is no cure for cystic fibrosis. Even though people with the disease must do things to stay healthy, because they are more vunerable to germs and allergies. Healthy habits keep the person from getting more sick.

Treatment includes breathing medicine (an inhaler) to help make the lungs stronger and add water to the lungs to keep the mucus thinner so it is easier to cough up. When there is thinner and less mucus it is easier to breathe.

People can stay clean. People can stay away from germs. They can drink water to help the mucus go away. Taking enzymes help digest food if there is mucus in the stomach.

Exercise clears mucus. It builds strong muscles and bones and strengthens the lungs. Taking vitamins (such as vitamin C and D) helps the body fight off infection. It also helps the body grow and function well.

• Inhaled antibiotics are used to keep bacteria from growing in the thick mucus
• Inhaled salt-water helps keep the lungs moisturized
• Inhaled corticosteroids helps make the lungs work better

A person with CF might need daily therapy from a parent or carer to loosen the mucus in their lungs by laying them on their stomach and massaging the back and breathing masks.

Testing for cystic fibrosis

• Genetic test - this is used if the sweat test is positive to see if they have both genes. In the UK all babies are screened at birth for the defect (CFTR gene, chromosome 7)
• Sweat chloride test - this tests the salt level of a persons sweat.

65 roses

"65 roses" is how some children refer to their condition since cystic fibrosis is difficult for a young child to say. '65 Roses' is also a trademarked phrase by the Cystic Fibrosis Foundation to help control its use. It is a very helpful way for young children to understand. When spoken aloud, it sounds similar to cystic fibrosis.

Support for patients and carers

Asthma + Lung UK

Cystic Fibrosis Trust

CF Kids – a charity that provides practical help and support