Cystic Fibrosis (CF) is the most common, life-shortening, genetic condition affecting young people in Australia.
CF is an inherited condition and one in 25 people are carriers of the CF gene. Carriers of the gene do not have any symptoms of the condition.
Cystic Fibrosis primarily affects the lungs, digestive system and the sweat glands. People with CF produce abnormally thick sticky mucus which blocks the passages in the lungs, greatly reducing oxygen intake and increasing the risk of infection and lung damage over time.
The thick mucus also affects the digestive system by blocking the passage of enzymes (which break down food) from the pancreas to the digestive system. As a result, the food eaten cannot be fully digested and a reduced rate of nutrients is absorbed into the body. Children and adults with CF therefore have difficulty gaining weight and can have bowel problems.
People with Cystic Fibrosis also lose more salt and potassium in their sweat, which is why a sweat test measuring the level of salt in the sweat continues to be the main diagnostic test for CF.
Treatment for CF is an ongoing daily regime. It cannot cure CF, but can greatly improve quality and length of life. Treatment is complicated, interrupts normal daily routines and therefore requires dedicated family support. Treatment generally involves:
- > Daily chest physiotherapy (up to three times a day)
- > Mist inhalations using a high pressure nebuliser
- > Enzyme replacement capsules with all meals and snacks (around 40 a day)
- > Additional vitamins and minerals
- > Antibiotics
- > Intravenous drug treatment
- > Regular hospitalisation
Due to advances in treatment, people with CF are living longer and many people have successfully undergone life saving lung transplants.
At present, there is no cure for Cystic Fibrosis; however with the dedication and commitment to the cause by medical staff and the generous support from both the CF and wider community, we look forward to that day.
For further information about CF, please visit: http://www.cfsa.org.au
