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background -3
caucasian
-males more than females
-genetics
Pathos
CFTR gene mutation leads to chloride channel dysfunction
-leads to decrease sodium and water in secretions
s/s -6
thick secretions
-mucus plugs
pneumonia
SOB
dyspnea
stools are floaters
diagnostics -4
CFTR gene testing
- chloride sweat test
- PFT
chest x ray
treatment/nursing -7
trikafta/izucafter monitor dehydration, electrolyte balance, decrease insulin
- fluticasone/flovent- watch for thrush so rinse mouth, and uri
- albuterol watch for tachycardia and K+
-prednisone watch for infection, metabolic syndrome
- mucolytics (guafenisn)- watch for increase of water/edema
- chest physiotherapy multiple times a day
-type 1 dm watch for insulin