Cystic Fibrosis

Question 1

what kind of a disorder is this

Answer 1

autosomal recessive

Question 2

which parts of the body does this affect

Answer 2

exocrine glands

Question 3

what happens to secretions with this disease

Answer 3

abnormally viscous

Question 4

what is required for a person to get this disease

Answer 4

2 copies of genes

Question 5

which 2 organs are most affected

Answer 5

pancreas

lungs

Question 6

which compound is unusually high in the body

Answer 6

sodium chloride

Question 7

what are the 3 major presentations in infancy

Answer 7

meconium ileus
failure to thrive
chronic respiratory infection

Question 8

what are the 2 major presentations in older children

Answer 8

fecal impaction

intussusception

Question 9

what are 4 late symptoms

Answer 9

pulmonary hypertension
R ventricular failure
respiratory failure
death

Question 10

what is normally seen as symptoms

Answer 10

non-productive cough
patchy atelectasis
clubbing
barrel chest

Question 11

which 2 things are not digested properly

Answer 11

fat

protein

Question 12

what is a major test in diagnosis

Answer 12

sweat analysis for high NaCl

Question 13

which 4 absorption problems are commonly seen

Answer 13

hyponatremia
hypokalemia
hypoalbuminemia
alkalosis

Question 14

what is the normal value for the sweat chloride test

Answer 14

< 40

Question 15

what is the sweat chloride value for CF pts

Answer 15

> 60

Question 16

at which value would you retest for the sweat chloride test

Answer 16

40-50

Question 17

what should the hydration level be

Answer 17

1.5x normal

Question 18

which classification of med should not be given

Answer 18

antihistamine

Question 19

which 3 enzymes can be given

Answer 19

catazyM-S
Pancrease
Ultrace

Question 20

what are 4 ways to maintain optimal fluid status

Answer 20

high calorie
high protein
increased fluids
enzyme supplements

Question 21

what does lactulose prevent

Answer 21

distal intestinal obstruction

Question 22

when should chest PT be done

Answer 22

1 hour before or 2 hours after meals

before bedtime

Question 23

when should pancreatic enzymes be given

Answer 23

within 30 mins of eating

Question 24

what is the worst complication

Answer 24

pseudomonas

Question 25

what is another serious complication

Answer 25

cor pulmonale