Cystic Fibrosis

Question 1

Cystic Fibrosis is characterized as an ????? gland dysfunction?

Endocrine or exocrine

Answer 1

Exocrine

Question 2

Cystic Fibrosis is a chronic multisystem disorder and ???? ???? trait disorder.

Answer 2

Autosomal recessive - both parents carry the gene (carriers but don’t have CF) and BOTH pass the recessive gene to the child.

Question 3

With Cystic Fibrosis the mucous produced by the exocrine glands is…

A. Smooth and slippery
B. Thick and copious
C. Thick with granular particles present
D. Discoloured

Answer 3

Thick and copious - causes obstructions in the respiratory, gastrointestinal and reproductive system and affects the integumentary system.

Question 4

Emphysema and atelectasis can occur from Cystic Fibrosis?

True or False

Answer 4

True - airway is obstructed

Question 5

What test, involving the integumentary system, can be done to test for Cystic Fibrosis?

Answer 5

Sweat chloride test

39 or lower - negative
40-59 - further testing needed
60+ positive

Question 6

Abnormal mucous secretion and obstruction during cystic fibrosis. Specifically, what areas in the body are affected?

A. Bronchi
B. Bile ducts
C. Pancreatic ducts
D. Small intestine
E. Parathyroid 
F. Epiglottitis

Answer 6

A,B,C,D

Bronchi - pneumonia, emphysema
Bile ducts - Billary cirrhosis causing hypertension
Pancreatic ducts - malabsorption of nutrients
Small intestine - blockage of meconium in intestine of newborn

Question 7

The term used to describe an intestinal obstruction where the infant cannot pass meconium is referred to as

A. Hirschsprung’s disease
B. Intussusception
C. Meconium ileus
D. Pneumothorax

Answer 7

C. Meconium ileus

Question 8

The GI system cannot absorb fat soluble vitamins in a patient with Cystic Fibrosis and the stool will appear greasy. What vitamins will a CF patient be deficient in?

Answer 8

D
E
K
A

Question 9

Cystic fibrosis (CF) is a genetic disorder that causes the endocrine glands to work incorrectly.

True or False

Answer 9

Cystic fibrosis (CF) is a genetic disorder that causes the EXOCRINE (not endocrine) glands to work incorrectly. 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 10

Select the systems below that are affected by cystic fibrosis:
A. Reproductive 
B. Lymphatic
C. Respiratory 
D. Gastrointestinal 
E. Neuro
F. Integumentary

Answer 10

The answers are A, C, D and F. Cystic fibrosis affects the respiratory system (this causes thick mucus to build up in the lungs and it can affect both the upper and lower respiratory system like the sinuses), gastrointestinal (pancreas, intestines, and liver are all affected due to thick mucus), and integumentary (skin’s sweat glands will make extremely salty sweat).

Question 11

You're educating the parents of an 8-month-old, who was recently diagnosed with cystic fibrosis, about the disease. You explain to the parents that the child has a gene mutation on the \_\_\_\_\_\_\_\_\_\_\_\_. The gene that is specifically mutated is called?
A. endocrine glands; Hbg S gene
B. exocrine glands; CFTR gene 
C. endocrine glands; Chromosome 21
D. exocrine glands; HTT gene

Answer 11

The answer is B. Cystic fibrosis affects the EXOCRINE glands, specifically the Cystic Fibrosis Transmembrane Regulator gene (CFTR). This is a protein that controls the channels of sodium and chloride…hence the sodium and water transport in and out of the cell. These channels are within the membrane of these cells that makes our sweat, mucous, tears, digestive enzymes. Since it is mutated the fluids that are normally thin now become very thick and sticky, which causes many problems in the organs like the lungs, pancreas, intestine etc.

Question 12

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 12

The answer is D. CF is an autosomal recessive genetic disorder. This means that both of the parents are carriers of the disease (they won’t have signs and symptoms of CF). They each have one healthy gene and one mutated gene. For CF to occur in their offspring, the parents will have to each pass ONE of the mutated genes to the child. In this case, it will be the CFTR gene….so the child receives one mutated gene from each parent and this leads to the child to develop CF.

Question 13

You're providing care to an 18-year-old male who has cystic fibrosis. Select all the possible complications this patient can experience due to cystic fibrosis:
A. Blood glucose 255 mg/dL 
B. Hearing disturbances
C. Hemoptysis 
D. Greasy, foul smelling stools 
E. Weight gain
F. Meconium ileus
G. Excessive mucus production 
H. Dyspnea 
I. Coughing 
J. Hyperoxemia
K. Infertility

Answer 13

The answers are: A, C, D, G, H, I, and K. All these are complications of cystic fibrosis. Option B: hearing disturbances not common, Option E: weight LOSS rather than gain due to the inability to digest food due to lacking pancreatic enzymes, Option F: this only occurs in infants…remember meconium is the first “bowel movement” an infant experiences after birth…this patient here is 18-years-old, and Option J: high oxygen in the blood….no but rather low because of the thick mucus blocking air flow in the lungs, which will lead to hypoxia.

Question 14

Hemoptysis refers to the coughing up of ???? stained mucous.

Answer 14

Blood

Question 15

Cystic Fibrosis leads to the pancreas not being able to produce normal amounts of insulin. As a result, the patient becomes insulin deficient and develops what condition?

Answer 15

Diabetes

Question 16

About one hour after eating a meal, your patient, who has cystic fibrosis, starts to experience abdominal pain and bloating. Then two hours later the patient has a bowel movement. The patient's stool appears to be greasy and have a foul odor. Which medication below that is being taken by the patient is not providing a desirable outcome for this patient and needs to be re-addressed by the physician?
A. Guaifenesin
B. Triamcinolone
C. Pancrelipase 
D. Polyethylene Glycol

Answer 16

The answer is C. Patients with CF need pancreatic enzymes because these enzymes that would normally leave the pancreas can’t because of thick mucus. Therefore, they must take a supplementation form of them. The pancreatic enzymes (protease, amylase, and lipase) help digest protein, fats etc. If a patient does not have enough present in the body to aid in digestion, they will start to have abdominal bloating/pain, greasy/foul smelling stools along with weight loss, and vitamin deficiency. The only pancreatic enzyme listed is Pancrelipase. Therefore, the MD needs to re-address the pancreatic enzyme supplementation because the patient is still having issues with digesting these important substances.

Question 17

A patient with cystic fibrosis is diagnosed with pancreatic insufficiency. As the nurse you know that the patient will be lacking:
A. Amylase 
B. Pepsin
C. Protease 
D. Maltase
E. Lipase

Answer 17

The answers are A, C, and E. The patient with CF, who has pancreatic insufficiency, will lack the ability to use pancreatic digestive enzymes such as Amylase, Protease, and Lipase. Remember PAL (they are lacking a PAL). Remember thick mucus is blocking the duct that allows these enzymes to enter the duodenum to aid in digestion.

Question 18

A patient completed a sweat test yesterday. The results are back and are 45 mmol/L. As the nurse you know this means:
A. The patient tested positive for cystic fibrosis.
B. The patient tested negative for cystic fibrosis.
C. The patient needs further testing because results are not conclusive.

Answer 18

The answer is C. A sweat test is gold standard in testing for cystic fibrosis. The result interpretations are the following: 39 mmol/L or less in patients 6 months or older are NEGATIVE for cystic fibrosis 40 to 59 mmol/L needs further testing, not conclusive 60 or more mmol/L POSITIVE for cystic fibrosis

Question 19

The physician orders chest physiotherapy on your patient with cystic fibrosis. This is best performed:
A. immediately after a meal
B. right before a meal
C. 1-2 hours after a meal 
D. only at bedtime

Answer 19

The answer is C. It is best to perform CPT 1-2 hours after a meal (in between meals). You wouldn’t want to do it immediately after a meal due to aspiration or vomiting risk OR right before because this can alter a patient’s appetite due to the mucous that will be expelled (the mucous can have a foul taste or odor to it), and option D is wrong because CPT is done up to 2-4 times a day NOT only at bedtime.

Question 20

You're assisting a patient with performing chest physiotherapy. It is very important you have the patient \_\_\_\_\_\_\_\_\_\_\_ during the therapy sessions.
A. bear down
B. use the incentive spirometer
C. huff cough 
D. use a peak flow meter

Answer 20

The answer is C. This is a special type of coughing that will help the patient cough up the mucous during the CPT. It is very important the patient performs this during their CPT sessions.

Question 21

The physician gives an order for a patient with cystic fibrosis to use a positive expiratory pressure (PEP) device to help with airway clearance. As the nurse you will order which device from supply:
A. Incentive spirometer
B. Bipap
C. Peak flow meter
D. Flutter valve

Answer 21

The answer is D. This device assists with moving mucous from the lungs to the airway so it can be expelled. Another device that does this is called an Acapella.

Question 22

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 22

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

Question 23

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 23

The answer is C. Pancrelipase is a pancreatic enzyme and should be given in an acidic food like apple sauce. A two-year-old can NOT swallow pills, so the medication capsule must be opened and sprinkled in food. Never crush or chew the medication. Also, never mix it in an alkaline food or milk-based food like yogurt, pudding, or ice cream…..this will inactivate the enzymes.