Cystic Fibrosis: What is it and why is it bad? Typically a children’s disease because they seldom lived to adulthood Genetic mutation creates a faulty epithelial cell chloride (Cl-) ion transporter. -As a result Cl- is not transported into the mucus of the lung and water does not follow the ions (sphere of hydration). -The mucus is too thick/viscous for cilia to move it (and bacteria in it) out of lung. -Spirometry: CF is both obstructive (mucous) and restrictive (tissue damage). Cycle:Infection Inflammation TissueDamag e More infection more inflammation, etc Shorter Life Span Cure: None exists except for finding a lung donor. Approach: palliative treatment only. Please be sensitive respectful to our guest speaker in this regard
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