Cystic Fibrosis Study Guide

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Sample questions from Cystic Fibrosis Study Guide

This material has 12 questions with answers and rationales. Here are the first 5.

1. 1.The nurse receives reports on four clients with cystic fibrosis. Which client should the nurse assess first?

   • A.D. A client whose weight decreased by 0.5 kg over the past month
   • B.B. A client with sudden right-sided chest pain and markedly diminished right breath sounds
   • C.A. A client reporting greasy stools after frequently missing snack-time enzymes
   • D.C. A client requesting instruction about nebulizer cleaning before discharge

   Rationale: B is correct because sudden unilateral pain and diminished breath sounds suggest pneumothorax, an immediate breathing threat. A requires evaluation of malabsorption and enzyme use but is not the most urgent finding. C is important discharge teaching without acute instability. D requires nutritional assessment, but the reported gradual change is less urgent than possible pneumothorax.

2. 2.A parent asks how to administer prescribed pancrelipase to a child who cannot swallow capsules. Which response is most appropriate?

   • A.A. Crush the beads and mix them into warm milk.
   • B.B. Give the entire daily dose before the child's first meal.
   • C.D. Mix the beads into a full bottle of formula and save unfinished formula for the next feeding.
   • D.C. If the product permits, sprinkle the opened capsule's beads on a small amount of acidic soft food and have the child swallow without chewing.

   Rationale: C is correct because some products permit opening the capsule and immediately administering intact beads with a small amount of acidic soft food. Product instructions must be followed. A destroys the protective coating and uses an inappropriate administration method. B fails to provide enzymes with later meals and snacks. D risks incomplete dosing and prolonged contact with moisture and does not follow safe product-specific administration.

3. 3.An infant has a positive newborn CF screen. A subsequent sweat chloride result is 42 mmol/L. Which explanation should the nurse provide?

   • A.A. The result is intermediate and additional evaluation is needed.
   • B.D. The result proves that the infant is a carrier but cannot have CF.
   • C.C. The result excludes CF because it is below 60 mmol/L.
   • D.B. The result confirms that the infant has severe CF.

   Rationale: A is correct because 30-59 mmol/L is an intermediate sweat chloride range requiring further evaluation. B is incorrect because this result alone does not establish CF or disease severity. C is incorrect because an intermediate result does not exclude CF. D is incorrect because sweat chloride alone does not establish carrier status.

4. 4.A client is prescribed albuterol, inhaled hypertonic saline, airway-clearance therapy, and an inhaled antibiotic. Which schedule best reflects a commonly used sequence when consistent with the client's orders?

   • A.A. Inhaled antibiotic, airway clearance, hypertonic saline, albuterol
   • B.C. Hypertonic saline, inhaled antibiotic, albuterol, airway clearance
   • C.D. Albuterol, hypertonic saline, airway clearance, inhaled antibiotic
   • D.B. Airway clearance, inhaled antibiotic, albuterol, hypertonic saline

   Answer and rationale are in the full material.

5. 5.An 11-year-old with CF has no symptoms of diabetes and a normal hemoglobin A1c. The caregiver asks why an oral glucose tolerance test is ordered. Which response is best?

   • A.A. All children with CF develop diabetes by age 12.
   • B.C. The test determines whether pancreatic enzymes can be discontinued.
   • C.B. A normal A1c can miss CF-related diabetes, and annual oral glucose tolerance screening generally begins by age 10.
   • D.D. The test is needed only because the child must be eating excessive sugar.

   Answer and rationale are in the full material.