🦠 𝘊𝘺𝘴𝘵𝘪𝘤 𝘍𝘪𝘣𝘳𝘰𝘴𝘪𝘴 (𝘊𝘍)

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🦠 Mostly from other sources & not put into my own words. Just a collection of resources:

Cystic Fibrosis

- damages lungs & digestive system

CF affects the cells that produce mucus, sweat & digestive juices. THis causes these fluids to become thick & sticky, which then plugs up tubes, ducts & passageways

CFF.org
✍︎︎ CF is a progressive, genetic disease that causes persistent lung infections & limits the ability to breathe over time.

In people w/ CF, mutations in the CFTF (cystic fibrosis transmembrane) conductance regulator gene causes the CFTR to become dysfunctional. WHen the protein isn't working correctly, it cannot help more chloride (a compartment of salt) to the cell surface. Without the chloride to attract water to the cell surface however, the mucus in various organs becomes thick & sticky.

✍︎︎ In the lungs, mucus clogs the airways & traps germs, like bacteria, leading to infections, inflammation, respiratory failure & other complications. It is for this reason that minimising contact with germs is a top concern for people living with CF.

✍︎︎ In the pancreas, the buildup of mucus prevents the release of digestive enzymes that help the body absorb food and key nutrients, resulting in malnutrition & poor growth. In the liver, the thick mucus can block the bile duct, causing liver disease. In men, CF can affect their ability to have children.

✍︎︎ The defective CF gene contains a mutation. There are 71'700 known mutations of the disease. Most genetic tests only screen for the most common CF mutations. ∴ the test results may indicate a person who is a carrier of the CF gene is not a carrier.

Helpful Resources:

https://nurseslabs.com/cystic-fibrosis/#nursing_management

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