Overview of cystic fibrosis

Cystic fibrosis (CF) is an inherited autosomal recessive condition, and is the most common cause of bronchiectasis and chronic suppurative lung disease in Caucasian children.

CF is a complex multisystem disease, which affects not only the lungs, gastrointestinal tract and pancreas, but also the liver, sinuses, sweat glands, kidneys, bones and reproductive system.

The CF gene codes for a protein known as the cystic fibrosis transmembrane conductance regulator (CFTR). CF gene mutations cause a variety of defects in CFTR production, location or function. Approximately 2000 mutations in the CF gene have been identified and these can be divided into several classes.

This topic is not a comprehensive CF management guide. All patients with CF in Australia are managed by, or in consultation with, a multidisciplinary specialist CF centre. This has been shown to improve clinical outcomes. Treatment protocols vary across different specialist CF centres. Changes in management should only be made after consultation with the specialist CF centre.

The Cystic Fibrosis Australia website has information brochures on CF for health professionals, patients, parents and carers.