case 4 part 2 Flashcards
what causes pneumothorax in cystic fibrosis patient
structural impairments and altered airflow dynamics
what is a haemoptysis
coughing up blood from the bronchial tubes
what is HFCWO
high frequency chest wall oscillations
used when a person is no longer able to effectively clear mucus airway clearance would prevent infection and inflammation - inflammation lead to haemoptysis
types of medications for cystic fibrosis
amoxicillin and clavulacin
digestive tract enzymes- help to produce protein and fat from food
vitmins
reflux medications
inhaled or nebulised medications- break down mucus
oral antibiotics for respiratory illness
how many types of cystic fibrosis mutations are there
5
clinical presentation of cystic fibrosis
wheezing
recurring chest infections
difficulty putting on weight
yellowing of the skin
dioreah ,constimation , lack of break down of mucus
bowel obstruction in new born babies - meconium liens
what would you hear for occultation on patient with cystic fibrosis
crackles and wheezing
medication commonly used to treat cystic fibrosis
kaftrio- treat the underlying causes by heling the lungs work effectively
diagnosis of cystic fibrosis
based on symptoms
genetics
screen tests
screening test for cystic fibrosis
prenatal screning
new born screaning
sweat test
what is a sweat test
diagnosis and screen for cystic fibrosis
test for levels of chloride in the sweat
60 or greater- diagnosis of cystic fibrosis- chloride helps to break down mucus
30-59 unclear diagnosis
less than 30- cystic fibrosis unlikely
effect of having inadequate surface airway liquid height
insufficient levels of the fluid can impair cilia function
individuals with cystic fibrosis have an insufficient sol layer- which causes it to have a sufficient surfactant height
cilia are unable to sweep away thick mucus so it forms a thick mucus plaque where bacteria proliferate leading to chronic infections
what causes a low sol layer in cystic fibrosis
decrease secretion of chloride and water by epithelial cells – resulted in dehydrated mucus
what is the result of impaired homeostatic regulations in cystic fibrosis
leads to dehydrated and acidic ASL
results of abnormal CFTR protein
creates an adverse acidic enviornment
- damages the antimicrobial function and changes the mucus properties and mucus clearance