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27 Cards in this Set
- Front
- Back
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what are some signs and symptoms of CF
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salty taste
delayed sexual development steatorrhea weight loss chronic bouts of pancreatitis |
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what protein involved in CF regulates the transport of electrolytes and water
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CFTR
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what organs can be effected by CF
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skin
lungs liver pancrease small intestine reproductive organs |
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what is seen when CF effects the pancreas
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loss of pancreatic enzyme activity
malabsorption of fats, proteins, fat soluble vitamins (ADEK) insulin deficiency |
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what is seen when CF effects the GI/Liver
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portal hypertension
gastroesophageal reflux esophageal varicies |
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what is the first sign of CF in humans
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Meconium ileus at birth
-composed of materials ingested during infants time in uterus |
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what is seen when CF effects the reproductive system
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delayed sexual development
decreased fertility |
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what is seen when CF effects the skin
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normally the skin reabsorbs Na and Cl from sweat
BUT with CF patients lose a large amount of chloride and sodium in their sweat |
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sweat chloride test is used to diagnose CF. what values are indicative of CF
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> 60 mmol/L of Cl
> 40 mmol/L of Cl if < 3 months old |
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what is the mainstay of prophylactic pulmonary therapy for CF
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Chest physical therapy
-vibrations break up pulmonary mucous |
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what are the treatment options of prophylactic pulmonary therapy
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chest physical therapy
Inhaled bronchodilators -Beta 2 agonist (albuterol, levalbuterol) -anticholinergic (ipatropium) |
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what do inhaled mucolytics do
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decrease viscosity of secretions
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what agent causes voice alterations
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Dornase alfa
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what are the agnets used as inhaled mucolytics
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dornase alfa
n acetylcysteine hypertonic (7%) saline |
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what must be given before you can administer Hypertonic (7%) saline
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premedicate with bronchodilator (albuterol/levalbuterol) 15 mins before treatment
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what are the prophylactic inhaled antibiotics
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tobramycin
aztreonam |
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what is seen in acute exacerbations of CF
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increased sputum production
increased cough dyspnea on exertion changes in sputum color febrile weight loss |
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if P. aeruginosa is the causative bacteria of CF what can be given
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aminoglycoside
+ 3rd gen cephalosporin (ceftazidine) OR 4th gen cephalosporin (cefepime) OR antipseudomonal PCN -pipercillin/tazobactam -ticarcillin/clavulanate |
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what happens to the Vd and Clearance of Aminoglycosides in a patient with CF
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both are increased
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what aminoglycosides are used to treat CF
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amikacin
gentamicin tobramycin |
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what toxicities may result from treatment with aminoglycosides
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ototoxicity - related to # of doses and total dose received
nephrotoxicity - related to trough levels |
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what are the 2nd line antibiotic agents for treatment of CF
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carbapenem (imipnenem/cilastin or meropenem)
FQ (ciprofloxacin) Aztreonam Colistimethate sodium Bactrim Azithromycin |
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what enzymes, vitamins, etc must be given to CF patients
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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) |
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what is the advantages of pancreatic enzyme supplementation
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prevent malnutrition
promote weight gain (can be used as a marker to se if therapy works) decrease GI symptoms |
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if a patient is maxed out on their pancreatic enzymes what can be give to optimize pt therapy
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H2 blockers
PPI |
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why are H2 blockers or PPI given along with pancreatic enzymes
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pancreatic enzymes don't work well in a acidic environment
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what agent directly targets the G551D gene in CF
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ivacaftor
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