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MSN Exam for Cystic Fibrosis (PM)*
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Question 1
Is there a cure for Cystic Fibrosis?
A
True
B
False
Question 2
A 6-year-old with cystic fibrosis has an order for Creon. The nurse knows that the medication will be given:
A
At bedtime
B
With meals and snacks
C
Twice daily
D
Daily in the morning
Question 2 Explanation:
Pancreatic enzyme replacement is given with each meal and each snack.
Question 3
A child who has recently been diagnosed with cystic fibrosis is in a pediatric clinic where a nurse is performing an assessment. Which later finding of this disease would the nurse not expect to see at this time?
A
Positive sweat test
B
Bulky greasy stools
C
Moist, productive cough
D
Meconium ileus
Question 3 Explanation:
Moist, productive cough Option c is a later sign. Noisy respirations and a dry non-productive cough are commonly the first of the respiratory signs to appear in a newly diagnosed client with cystic fibrosis (CF). The other options are the earliest findings. CF is an inherited (genetic) condition affecting the cells that produce mucus, sweat, saliva and digestive juices. Normally, these secretions are thin and slippery, but in CF, a defective gene causes the secretions to become thick and sticky. Instead of acting as a lubricant, the secretions plug up tubes, ducts and passageways, especially in the pancreas and lungs. Respiratory failure is the most dangerous consequence of CF.
Question 4
The nurse is teaching the mother of a child with cystic fibrosis how to do postural drainage. The nurse should tell the mother to:
A
Use the heel of her hand during percussion
B
Change the child’s position every 20 minutes
C
Do percussion after the child eats and at bedtime
D
Use cupped hands during percussion
Question 4 Explanation:
The nurse or parent should use a cupped hand when performing chest percussion. Answer A is incorrect because the hand should be cupped. Answer B is incorrect because the child’s position should be changed every 5–10 minutes and the whole session should be limited to 20 minutes. Answer D is incorrect because chest percussion should be done before meals.
Question 5
Cystic fibrosis is caused by:
A
A defective gene that causes abnormalities in the brain
B
It is not known what the cause is
C
Someone who eats too much salt
D
A defective gene that leads to the making of an abnormal protein
Question 5 Explanation:
Usually, the gene involved makes a protein called CFTR (Cystic Fibrosis Conductance Transmembrane Regulator). This protein regulates the movement of sodium and chloride in the body's cells. Disruption of this protein leads to thicker secretions due to the imbalance of salt and water in the cells. Too little salt and water are outside the cells, making everything thicker
Question 6
The recommended diet for someone with cystic fibrosis is:
A
High calorie and high fat diet
B
High fat diet
C
Low fat diet
D
High calorie diet
Question 6 Explanation:
The current recommendation is 120-150% of the recommended daily allowance of calories for children, and 40% fat. Adults with cystic fibrosis also need to eat high calorie food, with increased fat. This is because a person with cystic fibrosis doesn't absorb nutrients as well from their food, especially fat. A certain amount of fat is needed in your diet to keep you healthy.
Question 7
A client with cystic fibrosis is taking pancreatic enzymes. The nurse should administer this medication:
A
Once per day in the morning
B
Three times per day with meals
C
Once per day at bedtime
D
Four times per day
Question 7 Explanation:
Pancreatic enzymes should be given with meals for optimal effects. These enzymes assist the body in digesting needed nutrients. Answers A, C, and D are incorrect methods of administering pancreatic enzymes.
Question 8
Which of the following is the approximate prevalence ratio for cystic fibrosis?
A
1: 25,000
B
1: 5,000
C
1: 2,000
D
1: 800
Question 9
Body systems affected by cystic fibrosis include:
A
Pancreas
B
Sweat glands
C
Lungs
D
All of these
Question 9 Explanation:
Cystic fibrosis primarily affects the glands that secrete. An abnormal protein is produced making secretions sticky. These thickened secretions are found in the body's sweat, the lungs' mucus, the pancreas' output, liver output, and reproductive organs.
Question 10
An 8-year-old client with cystic fibrosis is admitted to the hospital and will undergo a chest physiotherapy treatment. The therapy should be properly coordinated by the nurse with the respiratory therapy department so that treatments occur during:
A
After meals
B
Between meals
C
After medication
D
Around the child’s play schedule
Question 10 Explanation:
Chest physiotherapy treatments are scheduled between meals to prevent aspiration of stomach contents, because the child is placed in a variety of positions during the treatment process.
Question 11
When administering pancrelipase (Pancreases capsules) to child with cystic fibrosis, nurse Faith knows they should be given:
A
With meals and snacks
B
Every three hours while awake
C
On awakening, following meals, and at bedtime
D
After each bowel movement and after postural drainage
Question 11 Explanation:
Pancreases capsules must be taken with food and snacks because it acts on the nutrients and readies them for absorption.
Question 12
One of the most important pulmonary treatments in cystic fibrosis is:
A
inhaled beta agonists.
B
inhaled corticosteroids.
C
chest physiotherapy.
D
oral enzymes.
Question 12 Explanation:
The major pulmonary problem with CF is thick tenacious secretions. CPT moves the secretions from the small airways to the large where they can be coughed out. Options a and b are used but are secondary to option c; the oral enzymes that CF patients take are for digestion, not pulmonary reasons.
Question 13
Cystic fibrosis is treated by:
A
Airway clearance techniques that aim to move the thick mucus from the lungs
B
Taking special vitamins
C
Taking pancreatic enzymes to help digest food
D
All of these
Question 13 Explanation:
All of these are treatments for cystic fibrosis. Airway clearance techniques help people with cystic fibrosis move thickened mucus from the lungs. Pancreatic enzymes are taken to help digest the food. Special vitamins are needed to help replace vitamins A, D, and K which may not be absorbed well by the CF patient. There are more treatments, of course, but these are the main ones to help keep the person with cystic fibrosis healthy.
Question 14
A child with cystic fibrosis is being treated with inhalation therapy with Pulmozyme (dornase alfa). A side effect of the medication is:
A
Weight gain
B
Hair loss
C
Sore throat
D
Brittle nails
Question 14 Explanation:
Side effects of Pulmozyme include sore throat, hoarseness, and laryngitis. Answers A, B, and C are not associated with Pulmozyme; therefore, they are incorrect.
Question 15
The mother of a child with cystic fibrosis tells the nurse that her child makes "snoring" sounds when breathing. The nurse is aware that many children with cystic fibrosis have:
A
Choanal atresia
B
Nasal polyps
C
Septal deviations
D
Enlarged adenoids
Question 15 Explanation:
Children with cystic fibrosis are susceptible to chronic sinusitis and nasal polyps, which might require surgical removal. Answer A is incorrect because it is a congenital condition in which there is a bony obstruction between the nares and the pharynx. Answers C and D are not specific to the child with cystic fibrosis; therefore, they are incorrect.
Question 16
A 2-year-old client with cystic fibrosis is confined to bed and is not allowed to go to the playroom. Which of the following is an appropriate toy would the nurse select for the child:
A
Puzzle
B
Musical automobile
C
Arranging stickers in the album
D
Pounding board and hammer
Question 16 Explanation:
The autonomous toddler would be frustrated by being confined to be. The pounding board and hammer is developmentally appropriate and an excellent way for the toddler to release frustration.
Question 17
The nurse is providing a health teaching to the mother of an 8-year-old child with cystic fibrosis. Which of the following statement if made by the mother would indicate to the nurse the need for further teaching about the medication regimen of the child?
A
“My child might need an extra capsule if the meal is high in fat”
B
“I’ll give the enzyme capsule before every snack”
C
“I’ll give the enzyme capsule before every meal”
D
“My child hates to take pills, so I’ll mix the capsule into a cup of hot chocolate
Question 17 Explanation:
The pancreatic capsules contain pancreatic enzyme that should be administered in a cold, not a hot, medium (example: chilled applesauce versus hot chocolate) to maintain the medication’s integrity.
Question 18
The foul-smelling, frothy characteristic of the stool in cystic fibrosis results from the presence of large amounts of which of the following:
A
sodium and chloride
B
undigested fat
C
semi-digested carbohydrates
D
lipase, trypsin and amylase
Question 18 Explanation:
The client with cystic fibrosis absorbs fat poorly because of the think secretions blocking the pancreatic duct. The lack of natural pancreatic enzyme leads to poor absorption of predominantly fats in the duodenum. Foul-smelling, frothy stool is termed steatorrhea.
Question 19
Cystic fibrosis is also called:
A
Bronchiectasis
B
Sticky mucus disease
C
Mucovicidosis
D
Irritating cough disease
Question 19 Explanation:
Cystic fibrosis is often called mucovicidosis, especially in other regions of the world. This refers to the thick, sticky mucus secreted by the exocrine glands of someone affected by cystic fibrosis.
Cystic fibrosis was named that in 1938 by Dr. Dorothy Anderson, a pathologist in New York City. She described changes in the pancreas as cystic, or fluid-filled, and fibrotic, or scarred.
Question 20
Cystic fibrosis is diagnosed by:
A
Echocardiogram
B
Complete blood panel
C
Chest X-ray
D
Sweat test
Question 20 Explanation:
A small electrode is placed on the skin, which stimulates the sweat glands. Sweat is collected for a period of time and then analyzed. People with cystic fibrosis have an increased amount of chloride in their sweat, due to the abnormal protein manufactured by the cystic fibrosis gene.
Question 21
If one of your parents have the disease does that mean you have a chance of getting the infection?
A
True
B
False
Question 22
The nurse in charge is reviewing a patient’s prenatal history. Which finding indicates a genetic risk factor?
A
The patient is 25 years old
B
The patient has a child with cystic fibrosis
C
The patient was exposed to rubella at 36 weeks’ gestation
D
The patient has a history of preterm labor at 32 weeks’ gestation
Question 22 Explanation:
Cystic fibrosis is a recessive trait; each offspring has a one in four chance of having the trait or the disorder. Maternal age is not a risk factor until age 35, when the incidence of chromosomal defects increases. Maternal exposure to rubella during the first trimester may cause congenital defects. Although a history or preterm labor may place the patient at risk for preterm labor, it does not correlate with genetic defects.
Question 23
At what age is this disease diagnosed?
A
Early years [0-5]
B
Childhood years [5-12]
C
Teenage years [12-17]
D
Adult years [18-50]
Question 24
A male adult client with cystic fibrosis is admitted to an acute care facility with an acute respiratory infection. Prescribed respiratory treatment includes chest physiotherapy. When should the nurse perform this procedure?
A
Immediately before a meal
B
At least 2 hours after a meal
C
When bronchospasms occur
D
When secretions have mobilized
Question 24 Explanation:
The nurse should perform chest physiotherapy at least 2 hours after a meal to reduce the risk of vomiting and aspiration. Performing it immediately before a meal may tire the client and impair the ability to eat. Percussion and vibration, components of chest physiotherapy, may worsen bronchospasms; therefore, the procedure is contraindicated in clients with bronchospasms. Secretions that have mobilized (especially when suction equipment isn’t available) are a contraindication for postural drainage, another component of chest physiotherapy.
Question 25
Which of the following clients is at high risk for developmental problem?
A
A toddler with acute Glomerulonephritis on antihypertensive and antibiotics
B
A 5-year-old with asthma on cromolyn sodium
C
A preschooler with tonsillitis
D
A 2 1/2 –year old boy with cystic fibrosis
Question 25 Explanation:
It is the developmental task of an 18-month-old toddler to explore and learn about the environment. The respiratory complications associated with cystic fibrosis (which are present in almost all children with cystic fibrosis) could prevent this development task from occurring.
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MSN Exam for Cystic Fibrosis (EM)*
Choose the letter of the correct answer. You got 25 minutes to finish the exam .Good luck!
Start
Congratulations - you have completed MSN Exam for Cystic Fibrosis (EM)*.
You scored %%SCORE%% out of %%TOTAL%%.
Your performance has been rated as %%RATING%%
Your answers are highlighted below.
Question 1
The nurse is providing a health teaching to the mother of an 8-year-old child with cystic fibrosis. Which of the following statement if made by the mother would indicate to the nurse the need for further teaching about the medication regimen of the child?
A
“My child might need an extra capsule if the meal is high in fat”
B
“I’ll give the enzyme capsule before every snack”
C
“I’ll give the enzyme capsule before every meal”
D
“My child hates to take pills, so I’ll mix the capsule into a cup of hot chocolate
Question 1 Explanation:
The pancreatic capsules contain pancreatic enzyme that should be administered in a cold, not a hot, medium (example: chilled applesauce versus hot chocolate) to maintain the medication’s integrity.
Question 2
Cystic fibrosis is treated by:
A
Airway clearance techniques that aim to move the thick mucus from the lungs
B
Taking special vitamins
C
Taking pancreatic enzymes to help digest food
D
All of these
Question 2 Explanation:
All of these are treatments for cystic fibrosis. Airway clearance techniques help people with cystic fibrosis move thickened mucus from the lungs. Pancreatic enzymes are taken to help digest the food. Special vitamins are needed to help replace vitamins A, D, and K which may not be absorbed well by the CF patient. There are more treatments, of course, but these are the main ones to help keep the person with cystic fibrosis healthy.
Question 3
Body systems affected by cystic fibrosis include:
A
Pancreas
B
Sweat glands
C
Lungs
D
All of these
Question 3 Explanation:
Cystic fibrosis primarily affects the glands that secrete. An abnormal protein is produced making secretions sticky. These thickened secretions are found in the body's sweat, the lungs' mucus, the pancreas' output, liver output, and reproductive organs.
Question 4
A male adult client with cystic fibrosis is admitted to an acute care facility with an acute respiratory infection. Prescribed respiratory treatment includes chest physiotherapy. When should the nurse perform this procedure?
A
Immediately before a meal
B
At least 2 hours after a meal
C
When bronchospasms occur
D
When secretions have mobilized
Question 4 Explanation:
The nurse should perform chest physiotherapy at least 2 hours after a meal to reduce the risk of vomiting and aspiration. Performing it immediately before a meal may tire the client and impair the ability to eat. Percussion and vibration, components of chest physiotherapy, may worsen bronchospasms; therefore, the procedure is contraindicated in clients with bronchospasms. Secretions that have mobilized (especially when suction equipment isn’t available) are a contraindication for postural drainage, another component of chest physiotherapy.
Question 5
Cystic fibrosis is diagnosed by:
A
Echocardiogram
B
Complete blood panel
C
Chest X-ray
D
Sweat test
Question 5 Explanation:
A small electrode is placed on the skin, which stimulates the sweat glands. Sweat is collected for a period of time and then analyzed. People with cystic fibrosis have an increased amount of chloride in their sweat, due to the abnormal protein manufactured by the cystic fibrosis gene.
Question 6
If one of your parents have the disease does that mean you have a chance of getting the infection?
A
True
B
False
Question 7
Which of the following is the approximate prevalence ratio for cystic fibrosis?
A
1: 25,000
B
1: 5,000
C
1: 2,000
D
1: 800
Question 8
The nurse is teaching the mother of a child with cystic fibrosis how to do postural drainage. The nurse should tell the mother to:
A
Use the heel of her hand during percussion
B
Change the child’s position every 20 minutes
C
Do percussion after the child eats and at bedtime
D
Use cupped hands during percussion
Question 8 Explanation:
The nurse or parent should use a cupped hand when performing chest percussion. Answer A is incorrect because the hand should be cupped. Answer B is incorrect because the child’s position should be changed every 5–10 minutes and the whole session should be limited to 20 minutes. Answer D is incorrect because chest percussion should be done before meals.
Question 9
One of the most important pulmonary treatments in cystic fibrosis is:
A
inhaled beta agonists.
B
inhaled corticosteroids.
C
chest physiotherapy.
D
oral enzymes.
Question 9 Explanation:
The major pulmonary problem with CF is thick tenacious secretions. CPT moves the secretions from the small airways to the large where they can be coughed out. Options a and b are used but are secondary to option c; the oral enzymes that CF patients take are for digestion, not pulmonary reasons.
Question 10
A child with cystic fibrosis is being treated with inhalation therapy with Pulmozyme (dornase alfa). A side effect of the medication is:
A
Weight gain
B
Hair loss
C
Sore throat
D
Brittle nails
Question 10 Explanation:
Side effects of Pulmozyme include sore throat, hoarseness, and laryngitis. Answers A, B, and C are not associated with Pulmozyme; therefore, they are incorrect.
Question 11
Cystic fibrosis is caused by:
A
A defective gene that causes abnormalities in the brain
B
It is not known what the cause is
C
Someone who eats too much salt
D
A defective gene that leads to the making of an abnormal protein
Question 11 Explanation:
Usually, the gene involved makes a protein called CFTR (Cystic Fibrosis Conductance Transmembrane Regulator). This protein regulates the movement of sodium and chloride in the body's cells. Disruption of this protein leads to thicker secretions due to the imbalance of salt and water in the cells. Too little salt and water are outside the cells, making everything thicker
Question 12
Which of the following clients is at high risk for developmental problem?
A
A toddler with acute Glomerulonephritis on antihypertensive and antibiotics
B
A 5-year-old with asthma on cromolyn sodium
C
A preschooler with tonsillitis
D
A 2 1/2 –year old boy with cystic fibrosis
Question 12 Explanation:
It is the developmental task of an 18-month-old toddler to explore and learn about the environment. The respiratory complications associated with cystic fibrosis (which are present in almost all children with cystic fibrosis) could prevent this development task from occurring.
Question 13
The nurse in charge is reviewing a patient’s prenatal history. Which finding indicates a genetic risk factor?
A
The patient is 25 years old
B
The patient has a child with cystic fibrosis
C
The patient was exposed to rubella at 36 weeks’ gestation
D
The patient has a history of preterm labor at 32 weeks’ gestation
Question 13 Explanation:
Cystic fibrosis is a recessive trait; each offspring has a one in four chance of having the trait or the disorder. Maternal age is not a risk factor until age 35, when the incidence of chromosomal defects increases. Maternal exposure to rubella during the first trimester may cause congenital defects. Although a history or preterm labor may place the patient at risk for preterm labor, it does not correlate with genetic defects.
Question 14
An 8-year-old client with cystic fibrosis is admitted to the hospital and will undergo a chest physiotherapy treatment. The therapy should be properly coordinated by the nurse with the respiratory therapy department so that treatments occur during:
A
After meals
B
Between meals
C
After medication
D
Around the child’s play schedule
Question 14 Explanation:
Chest physiotherapy treatments are scheduled between meals to prevent aspiration of stomach contents, because the child is placed in a variety of positions during the treatment process.
Question 15
At what age is this disease diagnosed?
A
Early years [0-5]
B
Childhood years [5-12]
C
Teenage years [12-17]
D
Adult years [18-50]
Question 16
A 2-year-old client with cystic fibrosis is confined to bed and is not allowed to go to the playroom. Which of the following is an appropriate toy would the nurse select for the child:
A
Puzzle
B
Musical automobile
C
Arranging stickers in the album
D
Pounding board and hammer
Question 16 Explanation:
The autonomous toddler would be frustrated by being confined to be. The pounding board and hammer is developmentally appropriate and an excellent way for the toddler to release frustration.
Question 17
Cystic fibrosis is also called:
A
Bronchiectasis
B
Sticky mucus disease
C
Mucovicidosis
D
Irritating cough disease
Question 17 Explanation:
Cystic fibrosis is often called mucovicidosis, especially in other regions of the world. This refers to the thick, sticky mucus secreted by the exocrine glands of someone affected by cystic fibrosis.
Cystic fibrosis was named that in 1938 by Dr. Dorothy Anderson, a pathologist in New York City. She described changes in the pancreas as cystic, or fluid-filled, and fibrotic, or scarred.
Question 18
A child who has recently been diagnosed with cystic fibrosis is in a pediatric clinic where a nurse is performing an assessment. Which later finding of this disease would the nurse not expect to see at this time?
A
Positive sweat test
B
Bulky greasy stools
C
Moist, productive cough
D
Meconium ileus
Question 18 Explanation:
Moist, productive cough Option c is a later sign. Noisy respirations and a dry non-productive cough are commonly the first of the respiratory signs to appear in a newly diagnosed client with cystic fibrosis (CF). The other options are the earliest findings. CF is an inherited (genetic) condition affecting the cells that produce mucus, sweat, saliva and digestive juices. Normally, these secretions are thin and slippery, but in CF, a defective gene causes the secretions to become thick and sticky. Instead of acting as a lubricant, the secretions plug up tubes, ducts and passageways, especially in the pancreas and lungs. Respiratory failure is the most dangerous consequence of CF.
Question 19
The recommended diet for someone with cystic fibrosis is:
A
High calorie and high fat diet
B
High fat diet
C
Low fat diet
D
High calorie diet
Question 19 Explanation:
The current recommendation is 120-150% of the recommended daily allowance of calories for children, and 40% fat. Adults with cystic fibrosis also need to eat high calorie food, with increased fat. This is because a person with cystic fibrosis doesn't absorb nutrients as well from their food, especially fat. A certain amount of fat is needed in your diet to keep you healthy.
Question 20
A 6-year-old with cystic fibrosis has an order for Creon. The nurse knows that the medication will be given:
A
At bedtime
B
With meals and snacks
C
Twice daily
D
Daily in the morning
Question 20 Explanation:
Pancreatic enzyme replacement is given with each meal and each snack.
Question 21
A client with cystic fibrosis is taking pancreatic enzymes. The nurse should administer this medication:
A
Once per day in the morning
B
Three times per day with meals
C
Once per day at bedtime
D
Four times per day
Question 21 Explanation:
Pancreatic enzymes should be given with meals for optimal effects. These enzymes assist the body in digesting needed nutrients. Answers A, C, and D are incorrect methods of administering pancreatic enzymes.
Question 22
There a cure for Cystic Fibrosis
A
True
B
False
Question 23
The mother of a child with cystic fibrosis tells the nurse that her child makes "snoring" sounds when breathing. The nurse is aware that many children with cystic fibrosis have:
A
Choanal atresia
B
Nasal polyps
C
Septal deviations
D
Enlarged adenoids
Question 23 Explanation:
Children with cystic fibrosis are susceptible to chronic sinusitis and nasal polyps, which might require surgical removal. Answer A is incorrect because it is a congenital condition in which there is a bony obstruction between the nares and the pharynx. Answers C and D are not specific to the child with cystic fibrosis; therefore, they are incorrect.
Question 24
The foul-smelling, frothy characteristic of the stool in cystic fibrosis results from the presence of large amounts of which of the following:
A
sodium and chloride
B
undigested fat
C
semi-digested carbohydrates
D
lipase, trypsin and amylase
Question 24 Explanation:
The client with cystic fibrosis absorbs fat poorly because of the think secretions blocking the pancreatic duct. The lack of natural pancreatic enzyme leads to poor absorption of predominantly fats in the duodenum. Foul-smelling, frothy stool is termed steatorrhea.
Question 25
When administering pancrelipase (Pancreases capsules) to child with cystic fibrosis, nurse Faith knows they should be given:
A
With meals and snacks
B
Every three hours while awake
C
On awakening, following meals, and at bedtime
D
After each bowel movement and after postural drainage
Question 25 Explanation:
Pancreases capsules must be taken with food and snacks because it acts on the nutrients and readies them for absorption.
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Question Text
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1) Body systems affected by cystic fibrosis include:
Pancreas
Sweat glands
Lungs
All of these
2) Which of the following clients is at high risk for developmental problem?
A toddler with acute Glomerulonephritis on antihypertensive and antibiotics
A 5-year-old with asthma on cromolyn sodium
A preschooler with tonsillitis
A 2 1/2 –year old boy with cystic fibrosis
3) The recommended diet for someone with cystic fibrosis is:
High calorie and high fat diet
High fat diet
Low fat diet
High calorie diet
4) If one of your parents have the disease does that mean you have a chance of getting the infection?
True
False
5) An 8-year-old client with cystic fibrosis is admitted to the hospital and will undergo a chest physiotherapy treatment. The therapy should be properly coordinated by the nurse with the respiratory therapy department so that treatments occur during:
After meals
Between meals
After medication
Around the child’s play schedule
6) Which of the following is the approximate prevalence ratio for cystic fibrosis?
1: 25,000
1: 5,000
1: 2,000
1: 800
7) Cystic fibrosis is treated by:
Airway clearance techniques that aim to move the thick mucus from the lungs
Taking special vitamins
Taking pancreatic enzymes to help digest food
All of these
8) The mother of a child with cystic fibrosis tells the nurse that her child makes “snoring” sounds when breathing. The nurse is aware that many children with cystic fibrosis have:
Choanal atresia
Nasal polyps
Septal deviations
Enlarged adenoids
9) Cystic fibrosis is caused by:
A defective gene that causes abnormalities in the brain
It is not known what the cause is
Someone who eats too much salt
A defective gene that leads to the making of an abnormal protein
10) The nurse is providing a health teaching to the mother of an 8-year-old child with cystic fibrosis. Which of the following statement if made by the mother would indicate to the nurse the need for further teaching about the medication regimen of the child?
“My child might need an extra capsule if the meal is high in fat”
“I’ll give the enzyme capsule before every snack”
“I’ll give the enzyme capsule before every meal”
“My child hates to take pills, so I’ll mix the capsule into a cup of hot chocolate
11) At what age is this disease diagnosed?
Early years [0-5]
Childhood years [5-12]
Teenage years [12-17]
Adult years [18-50]
50+
12) A child with cystic fibrosis is being treated with inhalation therapy with Pulmozyme (dornase alfa). A side effect of the medication is:
Weight gain
Hair loss
Sore throat
Brittle nails
13) A 6-year-old with cystic fibrosis has an order for Creon. The nurse knows that the medication will be given:
At bedtime
With meals and snacks
Twice daily
Daily in the morning
14) The nurse in charge is reviewing a patient’s prenatal history. Which finding indicates a genetic risk factor?
The patient is 25 years old
The patient has a child with cystic fibrosis
The patient was exposed to rubella at 36 weeks’ gestation
The patient has a history of preterm labor at 32 weeks’ gestation
15) A child who has recently been diagnosed with cystic fibrosis is in a pediatric clinic where a nurse is performing an assessment. Which later finding of this disease would the nurse not expect to see at this time?
Positive sweat test
Bulky greasy stools
Moist, productive cough
Meconium ileus
16) A client with cystic fibrosis is taking pancreatic enzymes. The nurse should administer this medication:
Once per day in the morning
Three times per day with meals
Once per day at bedtime
Four times per day
17) Is there a cure for Cystic Fibrosis?
True
False
18) The foul-smelling, frothy characteristic of the stool in cystic fibrosis results from the presence of large amounts of which of the following:
sodium and chloride
undigested fat
semi-digested carbohydrates
lipase, trypsin and amylase
19) A male adult client with cystic fibrosis is admitted to an acute care facility with an acute respiratory infection. Prescribed respiratory treatment includes chest physiotherapy. When should the nurse perform this procedure?
Immediately before a meal
At least 2 hours after a meal
When bronchospasms occur
When secretions have mobilized
20) A 2-year-old client with cystic fibrosis is confined to bed and is not allowed to go to the playroom. Which of the following is an appropriate toy would the nurse select for the child:
Puzzle
Musical automobile
Arranging stickers in the album
Pounding board and hammer
21) Cystic fibrosis is diagnosed by:
Echocardiogram
Complete blood panel
Chest X-ray
Sweat test
22) Cystic fibrosis is also called:
Bronchiectasis
Sticky mucus disease
Mucovicidosis
Irritating cough disease
23) One of the most important pulmonary treatments in cystic fibrosis is:
inhaled beta agonists.
inhaled corticosteroids.
chest physiotherapy.
oral enzymes.
24) When administering pancrelipase (Pancreases capsules) to child with cystic fibrosis, nurse Faith knows they should be given:
With meals and snacks
Every three hours while awake
On awakening, following meals, and at bedtime
After each bowel movement and after postural drainage
25) The nurse is teaching the mother of a child with cystic fibrosis how to do postural drainage. The nurse should tell the mother to:
Use the heel of her hand during percussion
Change the child’s position every 20 minutes
Do percussion after the child eats and at bedtime
Use cupped hands during percussion
Answers and Rationales
D. All of these . Cystic fibrosis primarily affects the glands that secrete. An abnormal protein is produced making secretions sticky. These thickened secretions are found in the body’s sweat, the lungs’ mucus, the pancreas’ output, liver output, and reproductive organs.
D. A 2 1/2 –year old boy with cystic fibrosis . It is the developmental task of an 18-month-old toddler to explore and learn about the environment. The respiratory complications associated with cystic fibrosis (which are present in almost all children with cystic fibrosis) could prevent this development task from occurring.
A. High calorie and high fat diet . The current recommendation is 120-150% of the recommended daily allowance of calories for children, and 40% fat. Adults with cystic fibrosis also need to eat high calorie food, with increased fat. This is because a person with cystic fibrosis doesn’t absorb nutrients as well from their food, especially fat. A certain amount of fat is needed in your diet to keep you healthy.
B. False . Both your parents must have the gene in order for you to get Cystic Fibrosis
B. Between meals . Chest physiotherapy treatments are scheduled between meals to prevent aspiration of stomach contents, because the child is placed in a variety of positions during the treatment process.
C. 1: 2,000
D. All of these . All of these are treatments for cystic fibrosis. Airway clearance techniques help people with cystic fibrosis move thickened mucus from the lungs. Pancreatic enzymes are taken to help digest the food. Special vitamins are needed to help replace vitamins A, D, and K which may not be absorbed well by the CF patient. There are more treatments, of course, but these are the main ones to help keep the person with cystic fibrosis healthy.
B. Nasal polyps. Children with cystic fibrosis are susceptible to chronic sinusitis and nasal polyps, which might require surgical removal. Answer A is incorrect because it is a congenital condition in which there is a bony obstruction between the nares and the pharynx. Answers C and D are not specific to the child with cystic fibrosis; therefore, they are incorrect.
D. A defective gene that leads to the making of an abnormal protein . Usually, the gene involved makes a protein called CFTR (Cystic Fibrosis Conductance Transmembrane Regulator). This protein regulates the movement of sodium and chloride in the body’s cells. Disruption of this protein leads to thicker secretions due to the imbalance of salt and water in the cells. Too little salt and water are outside the cells, making everything thicker
D. “My child hates to take pills, so I’ll mix the capsule into a cup of hot chocolate. The pancreatic capsules contain pancreatic enzyme that should be administered in a cold, not a hot, medium (example: chilled applesauce versus hot chocolate) to maintain the medication’s integrity.
A. Early years [0-5]
C. Sore throat. Side effects of Pulmozyme include sore throat, hoarseness, and laryngitis. Answers A, B, and C are not associated with Pulmozyme; therefore, they are incorrect.
B. With meals and snacks. Pancreatic enzyme replacement is given with each meal and each snack.
B. The patient has a child with cystic fibrosis . Cystic fibrosis is a recessive trait; each offspring has a one in four chance of having the trait or the disorder. Maternal age is not a risk factor until age 35, when the incidence of chromosomal defects increases. Maternal exposure to rubella during the first trimester may cause congenital defects. Although a history or preterm labor may place the patient at risk for preterm labor, it does not correlate with genetic defects.
C. Moist, productive cough . Moist, productive cough Option c is a later sign. Noisy respirations and a dry non-productive cough are commonly the first of the respiratory signs to appear in a newly diagnosed client with cystic fibrosis (CF). The other options are the earliest findings. CF is an inherited (genetic) condition affecting the cells that produce mucus, sweat, saliva and digestive juices. Normally, these secretions are thin and slippery, but in CF, a defective gene causes the secretions to become thick and sticky. Instead of acting as a lubricant, the secretions plug up tubes, ducts and passageways, especially in the pancreas and lungs. Respiratory failure is the most dangerous consequence of CF.
B. Three times per day with meals. Pancreatic enzymes should be given with meals for optimal effects. These enzymes assist the body in digesting needed nutrients. Answers A, C, and D are incorrect methods of administering pancreatic enzymes.
B. False
B. undigested fat . The client with cystic fibrosis absorbs fat poorly because of the think secretions blocking the pancreatic duct. The lack of natural pancreatic enzyme leads to poor absorption of predominantly fats in the duodenum. Foul-smelling, frothy stool is termed steatorrhea.
B. At least 2 hours after a meal. The nurse should perform chest physiotherapy at least 2 hours after a meal to reduce the risk of vomiting and aspiration. Performing it immediately before a meal may tire the client and impair the ability to eat. Percussion and vibration, components of chest physiotherapy, may worsen bronchospasms; therefore, the procedure is contraindicated in clients with bronchospasms. Secretions that have mobilized (especially when suction equipment isn’t available) are a contraindication for postural drainage, another component of chest physiotherapy.
D. Pounding board and hammer . The autonomous toddler would be frustrated by being confined to be. The pounding board and hammer is developmentally appropriate and an excellent way for the toddler to release frustration.
D. Sweat test . A small electrode is placed on the skin, which stimulates the sweat glands. Sweat is collected for a period of time and then analyzed. People with cystic fibrosis have an increased amount of chloride in their sweat, due to the abnormal protein manufactured by the cystic fibrosis gene.
C. Mucovicidosis . Cystic fibrosis is often called mucovicidosis, especially in other regions of the world. This refers to the thick, sticky mucus secreted by the exocrine glands of someone affected by cystic fibrosis. Cystic fibrosis was named that in 1938 by Dr. Dorothy Anderson, a pathologist in New York City. She described changes in the pancreas as cystic, or fluid-filled, and fibrotic, or scarred.
C. chest physiotherapy. The major pulmonary problem with CF is thick tenacious secretions. CPT moves the secretions from the small airways to the large where they can be coughed out. Options a and b are used but are secondary to option c; the oral enzymes that CF patients take are for digestion, not pulmonary reasons.
A. With meals and snacks. Pancreases capsules must be taken with food and snacks because it acts on the nutrients and readies them for absorption.
D. Use cupped hands during percussion . The nurse or parent should use a cupped hand when performing chest percussion. Answer A is incorrect because the hand should be cupped. Answer B is incorrect because the child’s position should be changed every 5–10 minutes and the whole session should be limited to 20 minutes. Answer D is incorrect because chest percussion should be done before meals.