Cystic fibrosis

What is cystic fibrosis?

Cystic fibrosis is an inherited genetic disease that severely affects the lungs, the digestive system (pancreas and liver) and sometimes the reproductive system. Cystic fibrosis affects the exocrine glands, which produce body fluids such as saliva, sweat, mucus, tears and enzymes. When a person has cystic fibrosis, their mucus glands secrete very sticky mucus that clogs up tiny air passages in the lungs that can cause infection due to tapped bacteria. Repeated infections and blockages can cause irreversible lung damage. Digestive enzymes needed to breakdown food are not secreted by the pancreas, which results in problems with nutrition, and people who suffer from cystic fibrosis often need to eat foods high in kilojoules, fats, sugars and salt. 

Cystic fibrosis is the most common life threatening genetic disease affecting Australian children. Through newborn screening, most babies are diagnosed within the first few weeks of life. Advances in treatment have seen improved quality of life and life expectancy for those with cystic fibrosis, but there is no cure and complications can increase with age. One in every 2500 babies has cystic fibrosis and one in every 25 people in the NSW population is a carrier of cystic fibrosis.

How do we help children who have cystic fibrosis?

The Sydney Children's Hospitals Network provides multidisciplinary care for children with cystic fibrosis, in both inpatient and outpatient settings. These patients are treated for:

• Nutritional deficiencies, requiring treatment by medical experts in gastroenterology
• Respiratory disease, requiring help from lung experts

See also Gastroenterology and Respiratory (lung) services.