There is production of unusually thick and sticky mucous with the failure of the various organs associated with these ducts and tubes to function normally. It is also associated with very high concentrations of salt in sweat.

In Australia the frequency of cystic fibrosis is 1 in 1800 births. The disease affects many organs in the body and also varies in its severity.

At birth the major problem is bowel obstruction from thick, sticky secretions. Although the lungs are normal at birth the thick mucous causes obstruction of the air passage which leads to infection. Repeated respiratory infections destroy the lung tissue and lung failure is the primary cause of death.

The thick secretions also affect the liver, pancreas and the gut leading to malabsorption of food and failure to thrive.

Until the last 10 years or so most children with cystic fibrosis died in their teens. Now, with better treatment many are living into their 20’s and 30’s. Infertility in both males and females is another complication of the disease but a number of CF mothers have given birth. Their babies have a much higher risk of the disease, about 1 in 40

Treatment deals with the symptoms rather than the underlying disease. Malabsoprtion can be corrected and respiratory complications minimised with physiotherapy, postural drainage of the lungs and antibiotics.

The social, emotional and financial aspects of the disease are the best met by a team approach to the whole family. Support can also be obtained through the Cystic Fibrosis Association in each state.