NSG2400 Cystic Fibrosis

A 20-year-old woman is known to be heterozygous for the cystic fibrosis (CF) gene. Her husband's genotype is unknown at present, and the couple is expecting their first child. What should the nurse tell the couple about the probability of their baby having CF? 1 25% or less 2 50% or more 3 Extremely common 4 Unknown at this time

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A nurse is admitting a 2-year-old toddler with a tentative diagnosis of cystic fibrosis to the pediatric unit. Pilocarpine is used as part of the diagnostic process. The nurse knows that the pilocarpine will stimulate which process? 1 Secretion of mucus 2 Activity of sweat glands 3 Excretion of pancreatic enzymes 4 Release of bile from the gallbladder

2

A nurse is discussing the need for genetic counseling with a male teenager who has a sibling with cystic fibrosis (CF). The identification of which test by the teenager indicates that he understands the genetic counseling? 1 Chest x-ray 2 Carrier testing 3 Sweat chloride 4 Chromosomal assay

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Which medications does the nurse expect to be prescribed for a preschooler with newly diagnosed cystic fibrosis? Select all that apply. 1 Steroids 2 Antibiotics 3 Antihistamines 4 Pancreatic enzymes 5 Fat-soluble vitamins

2 4 5

A nurse is reinforcing previous learning about cystic fibrosis and its treatment with a 9-year-old child. What is the most suitable information for a child of this age? 1 "The postural drainage will help you feel better." 2 "The dietitian says this meal schedule is best for you." 3 "Your mucus is thick because of the way your mucous glands work." 4 "You have to take your medicine with meals because that's how it was prescribed."

3

A nurse who is caring for a 2-day-old neonate suspects that the infant has cystic fibrosis. What early sign of this disorder did the nurse identify? 1 Meconium ileus 2 Imperforate anus 3 Hemoglobin level of 20 g/dL (200 mmol/L) 4 Total bilirubin level of 8 mg/dL (136 mcmol/L)

1

A nurse is reviewing previous education with the parents and a 12-year-old child with cystic fibrosis (CF). The disease was diagnosed when the child was 3 years old and has resulted in one hospitalization for a respiratory infection. For which potential complications of CF should the family be alert? Select all that apply. 1 Diabetes 2 Hematuria 3 Nasal polyps 4 Prolapsed rectum 5 Pulmonary infections 6 Urinary tract infections

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A nurse is caring for a school-aged child with cystic fibrosis who has been admitted to the pediatric unit with pneumonia. What potential consequence of repeated infections should the nurse consider when caring for this child? 1 Increased irritability 2 Development of diabetes 3 Impaired academic ability 4 Depression of bone marrow

2 Repeated infections in children with cystic fibrosis, over time, contribute to the development of insulin resistance and the development of type 1 diabetes. Increased irritability, changes in academic ability, and bone marrow depression are not related to chronic infections.

A 7-year-old child with cystic fibrosis is receiving an intravenous antibiotic. The medication is supplied in a 125-mL bag of 0.45% sodium chloride. It is to be infused over 30 minutes. At what rate should the infusion pump be set to deliver the medication in the prescribed time? Express your answer as a whole number. ___ mL/hr

250

A 12-year-old child with cystic fibrosis is to receive four pancrelipase capsules five times a day. The nurse explains that the medication should be taken with meals and snacks to accomplish which goal? 1 Enhance oxygenation 2 Limit excretion of fats 3 Facilitate nutrient utilization 4 Prevent iron-deficiency anemia

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What will a nurse teach the parents of a toddler with newly diagnosed cystic fibrosis about the administration of vitamins A, D, E, and K? 1 Offer them in a water-miscible form. 2 Give them during meals and snack times. 3 The dosage is based on the child's height and weight. 4 Present them to the child with fruit juice rather than milk.

1 Because children with cystic fibrosis do not absorb fat-soluble vitamins effectively, they should be given in a water-miscible form. These vitamins may be given with other vitamins once a day; pancreatic enzymes are administered with meals and snacks. The nurse does not have to base the dosage of these vitamins on the child's height and weight. There is no reason to select juice over milk when administering these vitamins.

A nurse is caring for several school-aged children with cystic fibrosis. Why does the nurse anticipate that these children will probably be small and underdeveloped for their age? 1 There is muscle atrophy from lack of motor activity. 2 There is decreased secretion of pituitary growth hormone. 3 These children digest little food because pancreatic enzymes are blocked. 4 These children have anorexia with minimal amounts of nutritional intake.

3 The production of tenacious mucus in the pancreatic ducts prevents the flow of digestive enzymes into the intestines. Therefore fats, proteins, and, to a lesser extent, carbohydrates cannot be digested and absorbed. Muscle atrophy from a lack of motor activity is not a problem associated with cystic fibrosis. Cystic fibrosis does not influence secretion of the growth hormone. Anorexia is not a usual problem in children with cystic fibrosis; usually they are ravenously hungry.

A nurse is discussing sexuality with a teenage female who has cystic fibrosis. Which statement best reflects the teenager's understanding of healthy sexuality? 1 "I can never get pregnant." 2 "Having sex is not possible for me." 3 "My best protection is a diaphragm." 4 "I won't have sex unless I use a condom."

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The parents of a school-aged child with cystic fibrosis tell the nurse that they have changed to natural pancreatic enzymes because of money issues. What is an appropriate response by the nurse? 1 "You don't need to give the enzymes now that your child is in school." 2 "Natural enzymes don't have any side effects and can be taken without regard to meals." 3 "If you are using generic enzymes, you will need to give twice as many to achieve the required effect." 4 "Natural enzymes are not as effective as the brand-name product. This is something you need to discuss with your healthcare provider."

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A school-aged child with cystic fibrosis has been admitted with a respiratory infection. The child has been very disruptive and angry with staff and parents. What does the nurse suspect is the reason for the child's uncooperative behavior? 1 Spoiled and needs to be adequately disciplined 2 Resentful of the restriction of the hospitalizations 3 Having a reaction to the new respiratory medications 4 Angry about dietary restrictions related to the disease

2

When will a nurse plan to administer pancrelipase to a child with cystic fibrosis? 1 With meals and snacks 2 In the morning and at bedtime 3 On awakening and every 3 hours while the child is awake 4 After each bowel movement and after postural drainage is performed

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A nurse teaches a 5-year-old child with cystic fibrosis how to use an inhaler. What is the most appropriate way to evaluate the child's understanding of the technique? 1 Asking questions about using the inhaler 2 Having the child demonstrate inhaler use 3 Explaining how the inhaler will be used at home 4 Having the child tell the nurse about the technique that was learned

2

The nurse is providing care to an infant who is diagnosed with cystic fibrosis (CF). Which parental statement indicates the need for further education related to the potential for poor growth? 1 "My child's diagnosis causes delayed bone growth." 2 "My child will have a poor appetite, which will lead to poor growth." 3 "My child will have increased oxygen demands, which will lead to poor growth." 4 "My child will have a decreased ability to absorb nutrients, which will cause poor growth."

2

A nurse provides clapping, percussion, and postural drainage every 4 hours for a 3-month-old infant with cystic fibrosis. When is the best time for the nurse to schedule chest physiotherapy? 1 During every feeding 2 Two hours after feedings 3 Right after every feeding 4 Right before every feeding

2

The parents of a toddler with newly diagnosed cystic fibrosis ask a nurse what causes the problems related to this disorder. What should the nurse consider about the primary pathologic process before responding? 1 Hyperactivity of the eccrine (sweat) glands 2 Hypoactivity of the autonomic nervous system 3 Mechanical obstruction of mucus-secreting glands 4 Atrophic changes in the mucosal lining of the intestines

3

A nurse is caring for a 4-year-old child with just-diagnosed cystic fibrosis. The child has been passing loose, bulky, foul-smelling stools and is in the third percentile for weight. What is the best explanation of the growth failure? 1 Impaired digestion and absorption because of the lack of pancreatic enzymes 2 Dyspnea and shortness of breath, which cause anorexia and disinterest in food 3 Increased bowel motility and diarrhea, which lead to inadequate absorption of nutrients 4 Pulmonary obstruction, which causes an oxygen deficit and inadequate tissue nourishment

1

A nurse is reviewing discharge instructions with the parent of an infant with cystic fibrosis. What statement indicates that the parent knows how to administer the pancreatic enzyme replacement? 1 "We should give the medication with feedings." 2 "We should put crushed enteric-coated pills in the formula." 3 "We need to give the medication every 6 hours, even during the night." 4 "We should feed the granules from the capsule in applesauce every morning."

1

The nurse is caring for a school-aged child with cystic fibrosis. Which pathophysiologic factor has the greatest impact on the child's health status and is of priority in the care plan? 1 Extremely thick mucus causes obstructed airways. 2 There is acute inflammation of the lung parenchyma. 3 Endocrine glands secrete increased levels of hormones. 4 Increased irritability of the airways results in obstruction.

1

The parent of a 2-year-old child with just-diagnosed cystic fibrosis expresses concern about the child's frailty and low weight. What is the most appropriate reply by the nurse? 1 "Digestive enzymes will be given to help your child digest food." 2 "Your child's appetite will improve once respiratory therapy is started." 3 "Your child's coughing and shortness of breath prevent adequate chewing of food." 4 "I suggest that you offer baby foods to your child because they are more easily digested."

1

A toddler with cystic fibrosis has been hospitalized with bacterial pneumonia. The nurse determines that the child has no known allergies. What does the nurse conclude is the reason that the healthcare provider selected a specific antibiotic? 1 Tolerance of the child 2 Sensitivity of the bacteria 3 Selectivity of the bacteria 4 Preference of the healthcare provider

2

A child has cystic fibrosis. Which verbalization by the parents about their plan for the child's dietary regimen provides evidence that they understand the nurse's instructions? 1 Restrict fluids during mealtimes. 2 Discontinue the use of salt when cooking. 3 Provide high-calorie foods between meals. 4 Add whole-milk products from the diet.

3

The parents of a 12-year-old boy with cystic fibrosis (CF) ask the nurse why he needs a glucose tolerance test. What information should the nurse consider before replying? 1 Pancreatic scarring predisposes the child to diabetes. 2 The thickened mucus blocks the insulin-secreting glands. 3 The test reveals the degree to which the child adheres to the diet.

1 Pancreatic scarring affects the ability of the islets of Langerhans to produce insulin; about half of all children with CF have altered glucose tolerance. The endocrine glands, which produce insulin, are ductless and are not affected by the thickened mucus in the ducts. However, the general scarring throughout the pancreas does affect the insulin-producing glands. The glucose tolerance test is a measure of the body's ability to produce and metabolize carbohydrates, not a measure of the child's adherence to the diet. The dosage of pancreatic enzymes is based on food consumption, not the blood glucose level.

A nurse manager is providing a class on cystic fibrosis for the pediatric staff nurses. Physiologic adaptations to cystic fibrosis are a result of which problem? 1 Dysfunction of sweat glands 2 Inactivity of respiratory tract cilia 3 Pathology of mucus-secreting glands 4 Overproduction of endocrine gland activity

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