Cystic Fibrosis Flashcards
what kind of a disorder is this
autosomal recessive
which parts of the body does this affect
exocrine glands
what happens to secretions with this disease
abnormally viscous
what is required for a person to get this disease
2 copies of genes
which 2 organs are most affected
pancreas
lungs
which compound is unusually high in the body
sodium chloride
what are the 3 major presentations in infancy
meconium ileus
failure to thrive
chronic respiratory infection
what are the 2 major presentations in older children
fecal impaction
intussusception
what are 4 late symptoms
pulmonary hypertension
R ventricular failure
respiratory failure
death
what is normally seen as symptoms
non-productive cough
patchy atelectasis
clubbing
barrel chest
which 2 things are not digested properly
fat
protein
what is a major test in diagnosis
sweat analysis for high NaCl
which 4 absorption problems are commonly seen
hyponatremia
hypokalemia
hypoalbuminemia
alkalosis
what is the normal value for the sweat chloride test
< 40
what is the sweat chloride value for CF pts
> 60
at which value would you retest for the sweat chloride test
40-50
what should the hydration level be
1.5x normal
which classification of med should not be given
antihistamine
which 3 enzymes can be given
catazyM-S
Pancrease
Ultrace
what are 4 ways to maintain optimal fluid status
high calorie
high protein
increased fluids
enzyme supplements
what does lactulose prevent
distal intestinal obstruction
when should chest PT be done
1 hour before or 2 hours after meals
before bedtime
when should pancreatic enzymes be given
within 30 mins of eating
what is the worst complication
pseudomonas
what is another serious complication
cor pulmonale
