Cystic Fibrosis is the most common, inherited, life threatening condition affecting young Australians.
Cystic Fibrosis (CF) primarily affects the lungs and digestive system because of a malfunction in the exocrine system, responsible for producing saliva, sweat, tears and mucus.
There is currently no cure. People with CF develop an abnormal amount of excessively thick and sticky mucus within the lungs, airways and the digestive system. The mucus causes impairment of the digestive functions of the pancreas and traps bacteria in the lungs resulting in recurrent infections which lead to irreversible damage. Lung failure is the major cause of death for someone with CF.
From birth, a person with CF undergoes constant medical treatments and physiotherapy.
CF is ‘autosomal recessive’ meaning that it occurs equally in males and females, the CF gene must be inherited from both parents and it can ‘skip’ generations.
In Australia, one in 2,500 babies are born with CF, that’s one every four days. On average one in 25 people carry the CF gene - most of whom are unaware that they are carriers. Because carriers of CF are unaffected (and therefore show no symptoms) it is hard for them to appreciate that CF may be a real risk. Any of us could be a carrier and we wouldn’t know - think about it, that’s about 1 million unaware carriers - it could be you!
In Tasmania, 1 in 20 people carry the CF gene. This is the second highest rate in the world behind Ireland.
Since 1986, all newborns have been subject to a heel prick test. This test is used to screen for a number of conditions, one of which is an indicator for CF. Diagnosis may also result after a baby is born with obvious CF symptoms such a bowel blockage or failure to "thrive". The heel prick test does not detect everyone; and the definitive test for CF continues to be The Sweat Test, as high levels of salt in perspiration is extremely common amongst those with CF.
Management and treatment of CF is lifelong, ongoing and relentless. A person with CF may consume up to 40 capsules daily to help digest food and may need to do up to three hours of airway clearance each day. It is important for people with CF to visit a CF treatment centre several times each year so that their progress can be monitored.
• Treatment generally involves:
• Intensive daily physiotherapy to clear the lungs
• Enzyme replacement capsules with food to aid digestion
• Antibiotic therapy to treat lung infections
• Aerosol mist inhalations via a nebuliser to help open the airways
• Salt and vitamin supplements
• A nutritious, high calorie, high salt, high fat diet
• Exercise - important to help clear the airways and build core strength.
All this before they even start their normal daily activities like going to school or work.
People with CF are not encouraged to socialise with each other. The risk of cross-infection and exacerbation of lung conditions is too great. CF can be a lonely existence.
About Cystic Fibrosis Community Care (CFCC)
CFCC is the peak community not-for-profit organisation working with and for people living with CF in Victoria and New South Wales.
CFCC provides information, support and guidance to those living with CF and their families. It advocates for services and systems that support the CF community. CFCC seeks to raise awareness, understanding and funds for services and research.
Find out more about CFCC at http://www.cfcc.org.au/