MSN Exam for Cystic Fibrosis

1) Body systems affected by cystic fibrosis include:

  1. Pancreas
  2. Sweat glands
  3. Lungs
  4. All of these

2) Which of the following clients is at high risk for developmental problem?

  1. A toddler with acute Glomerulonephritis on antihypertensive and antibiotics
  2. A 5-year-old with asthma on cromolyn sodium
  3. A preschooler with tonsillitis
  4. A 2 1/2 –year old boy with cystic fibrosis

3) The recommended diet for someone with cystic fibrosis is:

  1. High calorie and high fat diet
  2. High fat diet
  3. Low fat diet
  4. High calorie diet

4) If one of your parents have the disease does that mean you have a chance of getting the infection?

  1. True
  2. 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:

  1. After meals
  2. Between meals
  3. After medication
  4. Around the child’s play schedule

6) Which of the following is the approximate prevalence ratio for cystic fibrosis?

  1. 1: 25,000
  2. 1: 5,000
  3. 1: 2,000
  4. 1: 800

7) Cystic fibrosis is treated by:

  1. Airway clearance techniques that aim to move the thick mucus from the lungs
  2. Taking special vitamins
  3. Taking pancreatic enzymes to help digest food
  4. 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:

  1. Choanal atresia
  2. Nasal polyps
  3. Septal deviations
  4.  Enlarged adenoids

9) Cystic fibrosis is caused by:

  1. A defective gene that causes abnormalities in the brain
  2. It is not known what the cause is
  3. Someone who eats too much salt
  4. 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?

  1. “My child might need an extra capsule if the meal is high in fat”
  2. “I’ll give the enzyme capsule before every snack”
  3. “I’ll give the enzyme capsule before every meal”
  4. “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?

  1. Early years [0-5]
  2. Childhood years [5-12]
  3. Teenage years [12-17]
  4. Adult years [18-50]
  5. 50+

12) A child with cystic fibrosis is being treated with inhalation therapy with Pulmozyme (dornase alfa). A side effect of the medication is:

  1. Weight gain
  2. Hair loss
  3. Sore throat
  4. Brittle nails

13) A 6-year-old with cystic fibrosis has an order for Creon. The nurse knows that the medication will be given:

  1. At bedtime
  2. With meals and snacks
  3. Twice daily
  4. Daily in the morning

14) The nurse in charge is reviewing a patient’s prenatal history. Which finding indicates a genetic risk factor?

  1. The patient is 25 years old
  2. The patient has a child with cystic fibrosis
  3. The patient was exposed to rubella at 36 weeks’ gestation
  4. 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?

  1. Positive sweat test
  2. Bulky greasy stools
  3. Moist, productive cough
  4. Meconium ileus

16) A client with cystic fibrosis is taking pancreatic enzymes. The nurse should administer this medication:

  1. Once per day in the morning
  2. Three times per day with meals
  3. Once per day at bedtime
  4. Four times per day

17) Is there a cure for Cystic Fibrosis?

  1. True
  2. 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:

  1. sodium and chloride
  2. undigested fat
  3. semi-digested carbohydrates
  4. 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?

  1. Immediately before a meal
  2. At least 2 hours after a meal
  3. When bronchospasms occur
  4. 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:

  1. Puzzle
  2. Musical automobile
  3. Arranging stickers in the album
  4. Pounding board and hammer

21) Cystic fibrosis is diagnosed by:

  1. Echocardiogram
  2. Complete blood panel
  3. Chest X-ray
  4. Sweat test

22) Cystic fibrosis is also called:

  1. Bronchiectasis
  2. Sticky mucus disease
  3. Mucovicidosis
  4. Irritating cough disease

23) One of the most important pulmonary treatments in cystic fibrosis is:

  1. inhaled beta agonists.
  2. inhaled corticosteroids.
  3. chest physiotherapy.
  4. oral enzymes.

24) When administering pancrelipase (Pancreases capsules) to child with cystic fibrosis, nurse Faith knows they should be given:

  1. With meals and snacks
  2. Every three hours while awake
  3. On awakening, following meals, and at bedtime
  4. 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:

  1. Use the heel of her hand during percussion
  2. Change the child’s position every 20 minutes
  3. Do percussion after the child eats and at bedtime
  4. Use cupped hands during percussion
Answers and Rationales
  1. 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.
  2. 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.
  3. 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.
  4. B. False . Both your parents must have the gene in order for you to get Cystic Fibrosis
  5. 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.
  6. C. 1: 2,000 
  7. 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.
  8. 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.
  9. 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
  10. 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.
  11. A. Early years [0-5] 
  12. 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.
  13. B. With meals and snacks. Pancreatic enzyme replacement is given with each meal and each snack.
  14. 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.
  15. 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.
  16. 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.
  17. B. False 
  18. 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.
  19. 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.
  20. 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.
  21. 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.
  22. 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.
  23. 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.
  24. 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.
  25. 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.