In the lungs large airways there are thousands of tiny hairs called cilia. These cilia beat backwards and forwards aproximately one thousand times per minute. Lying on the cilia is a fine mucous layer which functions to trap dust, particles and germs which enter our lungs via breathing. These beating cilia continuously transport mucous (with trapped dust and germs) up and out of the airway somewhat like a conveyer belt.
However in the lungs of a person with cystic fibrosis, the consistency of the mucous is changed due the cells' inability to regulate chloride and sodium across the membrane. To simplify, the mucous layer is dehydrated causing it to be thick and sticky. The thick and sticky mucous clogs up the cilia's movement thus making them redundant. The lung's reaction is then to pump out more thick and sticky mucous which further exacerbates the problem. The dust, germs and particles are trapped in the mucous within the lungs, creating the perfect condition for infection to take hold. Each round of lung infection progressively impacts on lung function. Proactive physiotherapy, exercise, and medication are used to help keep the lungs clear and hopefully hold off infection.
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