Pulmonary - Cystic Fibrosis Flashcards
What is the cause of cystic fibrosis?
An abnormal proteins that affects the cells of the body that producing mucus and sweat
- it’s a genetic inherited condition which can be life threatening
What systems are affected with cystic fibrosis?
respiratory
digestive
reproductive
The respiratory is the most affected
How does the mucus effect the body?
mucus from the cells become thick and stuck = reduced function or infection
What is the prevelance of cyctic fibrosis?
Both parents must have the CF gene to pass it on to a child
- an autosomal recessive genetically transferred disorder
- for every pregnancy there is a 1 in 4 chance that the child will have CF
How is CF newborn screened?
Identification of an enzyme in the blood
- (+) results triggers additional testing methods to confirm
Genetic testing is needed because there are different mutations
What is the sweat test?
Measures levels of chloride in sweat
- higher levels = (+) test
How does the sweat test indicate CF?
Altered gene prevents the salt on the skin from being reabsorbed back into the sweat glands
- people with CF tend to sweat more
What are the goals guidelines of CF care?
controlling lung infection
promotic mucus clearance
improving nutritional status
pancreatic status and nutritional supplementation
What is the prognosis of CF?
Dramatic increase in age of survival (38 years)
What is the prevention of CF?
genetic counseling
screening for carrier status
What are sx and complications?
A picture because TOO much
What is the clincal presentation of CF?
A picture AGAIN
What system is most affected?
The pulmonary which presents the most severe complications
What are the goals of PT?
Prevent secondary complications and improve QOL by managing mucus secretions
- maintain or improve lung function and any impairment affecting other systems
What is the acute pulmonary exacerbations?
A picture again
How is each system affected?
A picture again
