CYSTIC FIBROSIS

Question 1

Cystic fibrosis (CF) is a genetic disorder that causes the [exocrine? or endocrine?] glands to work incorrectly.

Answer 1

Exocrine glands are glands that produce and transfer its secretions (ex: mucous, tears, sweat, digestive enzymes) via DUCTS to an intended area of the body, rather than directly into the bloodstream. This is different from endocrine glands that don’t have ducts but directly secrete chemicals (like hormones) directly into the blood.

Question 2

systems that are affected by cystic fibrosis:

Answer 2

Reproductive, Respi, GI, integumentary

Question 3

the child has a gene mutation on the ____________. The gene that is specifically mutated is called?

Answer 3

exocrine glands; CFTR gene

Question 4

. Cystic fibrosis is an autosomal recessive genetic disorder. Which option below best describes what most likely happens for a child to develop this condition?
A. One parent, who is a carrier of the mutated gene, has to pass it to the child
B. One of the parents has to have cystic fibrosis in order to pass it to their offspring
C. Both of the parents must have cystic fibrosis in order for the child to develop it
D. Both parents, who are carriers of the mutated gene, each pass one mutated gene to the child

Answer 4

D

Question 5

patient with cystic fibrosis is diagnosed with pancreatic insufficiency. As the nurse you know that the patient will be lacking:

Answer 5

PROTEASE
LIPASE
AMYLASE

Question 6

DX TEST

gold standard in testing for cystic fibrosis.

Answer 6

sweat test

Question 7

chest physiotherapy on your patient with cystic fibrosis. This is best performed:

Answer 7

1-2 hours after a meal

Question 8

n mngt

Answer 8

Chest Physiotherapy
Positive Expiratory Pressure
Exercise
Prevent Constipation

Question 9

affected parts

Answer 9

Lungs
Liver
Integumentary

Question 10

mutated CFTR gene vs. non-mutated

Answer 10

Mutated= Thick sticky mucus
Not mutated= Thin & Slippery mucus

Question 11

bonus

A 2 year-old patient with cystic fibrosis is scheduled to take Pancrelipase. How will you administer this medication?
A. orally with yogurt
B. orally with pudding
C. orally with applesauce
D. orally with ice cream

Answer 11

The answer is C. Pancrelipase is a pancreatic enzyme and should be given in an acidic food like apple sauce.

Question 12

bonus

A patient is scheduled to take Pancreatin. When will you administer this medication to the patient?
A. Right before all meals and snacks
B. Right before meals only
C. Immediately after meals and snacks
D. Immediately after meals only

Answer 12

A. Pancreatin is a pancreatic enzyme and the patient needs to take it right before ALL meals and snacks.