Cystic Fibrosis Flashcards
1
Q
What is CF?
A
CF is an autosomal recessive disorder affecting the exocrine glands. It is the Impairment of water and ion transport across the epithelial cells of the body causing dehydration and obstructions of various lumens in the body.
- in the lungs na+ and cl- ions cannot escape from the epithelial cells into the airways in order to maintain hydration of mucus. So it is thick and sticky!
- in the gut this impairment causes malabsorption and pancreatic insufficiency
2
Q
Symptoms and signs?
A
Breathlessness Weariness Incessant cough Small stature Delayed puberty Bloated abdomen Persistent chest infections Poor lung function SoB
3
Q
Causes?
A
It is a genetic disorder where the CFTR gene on chromosome 7 does not work properly. A mutation of this gene means that cells are unable to handle sodium and chloride properly.
4
Q
Treatment?
A
Postural drainage Manual techniques to help clear secretions Flutter ACBT Auto genie drainage technique
