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What is Cystic Fibrosis?

Cystic Fibrosis (CF) is a recessive genetic condition. The gene involved in CF gives instructions for the cells to make a protein that controls the movement of salt in and out of cells.

This salt transport gene lies on chromosome 7. Everyone has two copies of chromosome 7 and therefore everyone has two copies of the salt transport gene. Scientists have called it the CFTR (Cystic Fibrosis Transmembrane Regulator) gene.

Causes

Cystic Fibrosis (CF) is an inherited recessive genetic condition. Amongst people of Caucasian ancestry 1 in 25 are genetic carriers for CF, usually without knowing it.

If a baby is born with CF, it means that both parents are genetic carriers for CF. However, even if they are genetic carriers, there is no guarantee that the child will be born with CF.

If two people are genetic carriers for CF and they have a child there is (with every pregnancy):

  • A 1 out of 4 (25%) chance that the child will have CF
  • A 2 out of 4 (50%) chance that the child will be a genetic carrier for CF
  • A 1 out of 4 (25%) chance that the child will not have CF and will not be a genetic carrier for CF.

Treatment

The CF gene was identified in 1989 and this has led to the development of a carrier test, improved treatment and better control of the disease.

At the moment every person who has CF must use intensive daily airway clearance techniques to combat the build-up of mucus in the lungs. Many people with CF will also take up to 40 enzyme replacement tablets each day to aid digestion. They must also follow high energy diets with added vitamins and salt.

Exercise is also a very important part of keeping people with CF healthy as it prevents deterioration of the lungs and improves physical bulk and strength.

Regular visits to CF Clinics, hospitalisation and antibiotic treatment are common for people with CF.

 

At Technipro-Pulmomed, we have a range of products to 
comprehensively manage Cystic Fibrosis. To see our
product range, visit the products page.

* *Information from the Cystic Fibrosis Australia Website**