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Mucociliary system

Healthy lungs

In a healthy person there is a constant flow of mucus over the surfaces of the air passages in the lungs removing debris and bacteria.

Healthy Lungs

Constant flow of mucus over lung surface

The cilia (hair like structures) are bathed in an airway surface liquid and can beat effectively to clear mucus from the lung

Disease-causing pathogens are ‘swept’ away

Cough is a natural defence mechanism to help clear mucus (productive cough is useful)

Mucus changes and lack of surface fluid movement in CF

People with cystic fibrosis carry a gene which disrupts ion transport across the epithelial membrane so their airways’ surface becomes dehydrated.

In the lungs, this leads to a reduction in airway surface layer; the cilia cannot beat effectively and the mucus is difficult to clear.

Affected Lungs

The result is thick, sticky mucus that clogs the lungs, severely restricting the natural airway-clearing process. Bacteria can become trapped. Airways can become inflamed and life-threatening lung infections disrupt the lives of people who just want to get on with living a normal life.

Dehydrated airway surface layer

Cilia can’t beat effectively

Mucus is sticky and difficult to clear

Sticky mucus provides a reservoir for infection and inflammation

Failure of productive cough

Adverse events should be reported to Pharmaxis Ltd by email: adverse.events@pharmaxis.com.au.