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27 Cards in this Set

  • Front
  • Back
what are some signs and symptoms of CF
salty taste
delayed sexual development
steatorrhea
weight loss
chronic bouts of pancreatitis
what protein involved in CF regulates the transport of electrolytes and water
CFTR
what organs can be effected by CF
skin
lungs
liver
pancrease
small intestine
reproductive organs
what is seen when CF effects the pancreas
loss of pancreatic enzyme activity
malabsorption of fats, proteins, fat soluble vitamins (ADEK)
insulin deficiency
what is seen when CF effects the GI/Liver
portal hypertension
gastroesophageal reflux
esophageal varicies
what is the first sign of CF in humans
Meconium ileus at birth
-composed of materials ingested during infants time in uterus
what is seen when CF effects the reproductive system
delayed sexual development
decreased fertility
what is seen when CF effects the skin
normally the skin reabsorbs Na and Cl from sweat
BUT
with CF patients lose a large amount of chloride and sodium in their sweat
sweat chloride test is used to diagnose CF. what values are indicative of CF
> 60 mmol/L of Cl
> 40 mmol/L of Cl if < 3 months old
what is the mainstay of prophylactic pulmonary therapy for CF
Chest physical therapy
-vibrations break up pulmonary mucous
what are the treatment options of prophylactic pulmonary therapy
chest physical therapy

Inhaled bronchodilators
-Beta 2 agonist (albuterol, levalbuterol)
-anticholinergic (ipatropium)
what do inhaled mucolytics do
decrease viscosity of secretions
what agent causes voice alterations
Dornase alfa
what are the agnets used as inhaled mucolytics
dornase alfa
n acetylcysteine
hypertonic (7%) saline
what must be given before you can administer Hypertonic (7%) saline
premedicate with bronchodilator (albuterol/levalbuterol) 15 mins before treatment
what are the prophylactic inhaled antibiotics
tobramycin
aztreonam
what is seen in acute exacerbations of CF
increased sputum production
increased cough
dyspnea on exertion
changes in sputum color
febrile
weight loss
if P. aeruginosa is the causative bacteria of CF what can be given
aminoglycoside
+
3rd gen cephalosporin (ceftazidine)
OR
4th gen cephalosporin (cefepime)
OR
antipseudomonal PCN
-pipercillin/tazobactam
-ticarcillin/clavulanate
what happens to the Vd and Clearance of Aminoglycosides in a patient with CF
both are increased
what aminoglycosides are used to treat CF
amikacin
gentamicin
tobramycin
what toxicities may result from treatment with aminoglycosides
ototoxicity - related to # of doses and total dose received
nephrotoxicity - related to trough levels
what are the 2nd line antibiotic agents for treatment of CF
carbapenem (imipnenem/cilastin or meropenem)
FQ (ciprofloxacin)
Aztreonam
Colistimethate sodium
Bactrim
Azithromycin
what enzymes, vitamins, etc must be given to CF patients
vitamins ADEK must be given since they are unable to absorb them
<1 2.5 mg twice a week Vit K
>1 5 mg twice a week Vit K

pancreatic enzymes may be given (protease, lipase, amylase)
what is the advantages of pancreatic enzyme supplementation
prevent malnutrition
promote weight gain (can be used as a marker to se if therapy works)
decrease GI symptoms
if a patient is maxed out on their pancreatic enzymes what can be give to optimize pt therapy
H2 blockers
PPI
why are H2 blockers or PPI given along with pancreatic enzymes
pancreatic enzymes don't work well in a acidic environment
what agent directly targets the G551D gene in CF
ivacaftor