Midterm ch 20 Flashcards
Which of the following is the primary cause of the thickened secretions seen in cystic fibrosis (CF)?
a) Increased sodium absorption and impaired chloride-ion transport
b) Overproduction of mucous in the lungs
c) Impaired protein synthesis in epithelial cells
d) Abnormal activation of digestive enzymes in the pancreas
a) Increased sodium absorption and impaired chloride-ion transport
Rationale: The defective CFTR gene impairs chloride-ion transport across epithelial cells, and increased sodium absorption leads to thickened secretions in various organs, including the lungs and pancreas.
A 4-year-old child with cystic fibrosis (CF) presents with a persistent cough, wheezing, and difficulty breathing. The nurse knows that chronic bacterial and fungal colonization in the airways can result in which of the following long-term complications?
a) Pneumothorax
b) Hyperglycemia
c) Hypotension
d) Nephrotic syndrome
a) Pneumothorax
Rationale: Chronic bacterial and fungal colonization, along with bronchiectasis (abnormal dilation of the bronchi), can lead to respiratory complications such as pneumothorax and hemothorax in children with CF.
A child with cystic fibrosis is experiencing failure to thrive. Which of the following is the most likely cause of this nutritional deficit?
a) Excessive caloric intake due to malabsorption
b) Impaired digestive enzyme flow from the pancreas
c) Hypermetabolism due to chronic respiratory infections
d) Insulin resistance leading to poor glucose absorption
b) Impaired digestive enzyme flow from the pancreas
Rationale: Obstructions in the pancreatic ducts impair enzyme flow, affecting digestion of fats, fat-soluble vitamins, and proteins, leading to malabsorption and failure to thrive.
A newborn is diagnosed with cystic fibrosis after presenting with meconium ileus. The nurse understands that meconium ileus is a result of which of the following?
a) Inadequate secretion of chloride and fluid into the intestines
b) Dehydration due to excessive sodium loss
c) Thickened secretions in the airway causing airway obstruction
d) Accumulation of undigested food in the gastrointestinal tract
a) Inadequate secretion of chloride and fluid into the intestines
Rationale: Meconium ileus occurs due to the failure to secrete adequate chloride and fluid into the intestines, resulting in a small bowel obstruction, commonly seen in 15% of newborns with CF.
A 10-year-old child with cystic fibrosis is at risk for hyponatremic dehydration due to excessive electrolyte loss through perspiration. The nurse should monitor for which of the following symptoms of hyponatremic dehydration?
a) Increased thirst and dark, concentrated urine
b) Weight gain and edema
c) Hyperactivity and irritability
d) Rapid pulse and dry mouth
d) Rapid pulse and dry mouth
Rationale: Excessive electrolyte loss in CF leads to hyponatremic dehydration, which can manifest as a rapid pulse, dry mouth, and signs of dehydration such as decreased urine output.
A 5-year-old child with cystic fibrosis (CF) is experiencing chronic sinusitis, purulent nasal discharge, and postnasal drip. Which of the following is most likely contributing to these symptoms?
a) Excessive production of ciliary action in the nasal passages
b) Increased airway hyperinflation in the lungs
c) Thickened mucus in the sinuses leading to obstruction
d) Increased insulin production causing fluid retention
c) Thickened mucus in the sinuses leading to obstruction
Rationale: In CF, the thickened mucus due to impaired chloride transport leads to blocked sinuses, resulting in chronic sinusitis and related symptoms such as purulent nasal discharge and postnasal drip.
A child with cystic fibrosis is demonstrating chronic, productive cough, wheezing, and coarse crackles on auscultation. What is the primary cause of these respiratory symptoms?
a) Impaired ciliary clearance and bacterial colonization in the airways
b) Overproduction of surfactant in the alveoli
c) Accumulation of undigested food particles in the lungs
d) Increased mucus secretion in the upper respiratory tract
a) Impaired ciliary clearance and bacterial colonization in the airways
Rationale: The impaired ciliary function and thickened mucus lead to airway obstruction, bacterial colonization, and chronic respiratory symptoms, including a productive cough, wheezing, and coarse crackles.
Which of the following is a common clinical manifestation of cystic fibrosis related to pancreatic dysfunction?
a) Insulin resistance leading to hyperglycemia
b) Inadequate secretion of digestive enzymes resulting in malabsorption
c) Overproduction of digestive enzymes resulting in pancreatitis
d) Decreased bile flow leading to constipation
b) Inadequate secretion of digestive enzymes resulting in malabsorption
Rationale: The obstruction of pancreatic ducts in CF prevents the secretion of digestive enzymes, leading to malabsorption, which can result in poorly digested food and nutritional deficiencies.
A nurse is assessing a child with cystic fibrosis who is presenting with greasy, foul-smelling stools that float. Which of the following is the most likely cause of this clinical manifestation?
a) Decreased bile secretion from the liver
b) Increased production of digestive enzymes by the pancreas
c) Impaired pancreatic enzyme secretion leading to steatorrhea
d) Malabsorption of water in the gastrointestinal tract
c) Impaired pancreatic enzyme secretion leading to steatorrhea
Rationale: The impaired pancreatic enzyme secretion in CF leads to malabsorption of fats, resulting in steatorrhea (greasy, foul-smelling stools that float).
Which of the following would be the most appropriate nursing intervention for a child with cystic fibrosis who is experiencing constipation and abdominal distention?
a) Administering a low-sodium diet to prevent dehydration
b) Reducing fat intake to prevent further abdominal discomfort
c) Withholding oral nutrition until symptoms resolve
d) Encouraging increased fluid intake and regular physical activity
d) Encouraging increased fluid intake and regular physical activity
Rationale: Increasing fluid intake and promoting physical activity can help alleviate constipation and improve gastrointestinal motility, addressing symptoms of abdominal distention and constipation.
A 12-year-old child with cystic fibrosis is being evaluated for growth and development. The nurse observes delayed onset of puberty and poor weight gain. Which of the following is most likely contributing to these issues?
a) Chronic respiratory infections leading to decreased appetite
b) Vitamin A, D, E, and K deficiencies from poor fat absorption
c) Inadequate insulin production causing hyperglycemia
d) Increased caloric intake due to accelerated metabolism
b) Vitamin A, D, E, and K deficiencies from poor fat absorption
Rationale: The impaired pancreatic function in CF leads to malabsorption of fat and fat-soluble vitamins (A, D, E, and K), contributing to growth delays, poor weight gain, and delayed puberty.
A newborn with cystic fibrosis is diagnosed with meconium ileus. What is the pathophysiological cause of this condition?
a) Failure to secrete sufficient chloride and fluid into the intestines
b) Excessive production of bile obstructing the intestines
c) Malabsorption of nutrients causing intestinal inflammation
d) Decreased motility of the small intestine due to nerve damage
a) Failure to secrete sufficient chloride and fluid into the intestines
Rationale: Meconium ileus occurs when insufficient chloride and fluid are secreted into the intestines, leading to thickened meconium that causes small bowel obstruction.
A nurse is assessing a male adolescent with cystic fibrosis who is concerned about fertility. What is the most common cause of male infertility in cystic fibrosis?
a) Abnormal sperm morphology
b) Reduced production of sperm due to hormonal imbalances
c) Absence of the vas deferens
d) Decreased semen volume due to inadequate prostate fluid production
c) Absence of the vas deferens
Rationale: In males with cystic fibrosis, the absence of the vas deferens is the most common cause of infertility, resulting in obstructive azoospermia.
A child with cystic fibrosis is noted to have a barrel chest and clubbing of the fingers and toes. These clinical manifestations are primarily indicative of which of the following?
a) Chronic respiratory infections leading to airway damage and oxygenation issues
b) Cardiac failure due to insufficient blood flow to the lungs
c) Nutritional deficiencies causing delayed bone development
d) Electrolyte imbalances leading to abnormal skeletal development
a) Chronic respiratory infections leading to airway damage and oxygenation issues
Rationale: The barrel chest and clubbing of the fingers and toes are long-term signs of chronic respiratory issues, such as chronic infections and airway damage, leading to impaired oxygenation and pulmonary function in cystic fibrosis.
A 7-year-old child with cystic fibrosis is being taught about airway clearance techniques. Which of the following interventions should the nurse include in the teaching plan to help the child expel secretions?
a) Encourage the child to practice deep breathing exercises while lying flat
b) Use chest physiotherapy twice a day, including percussion or vibration
c) Instruct the child to avoid coughing as it could cause airway injury
d) Recommend using an oscillating chest vest only once a week
b) Use chest physiotherapy twice a day, including percussion or vibration
Rationale: Chest physiotherapy is crucial for promoting the drainage of secretions from the lungs. It should be performed twice daily, using techniques like percussion or vibration to help expel mucus from the lungs and prevent airway obstruction.
A 4-year-old child with cystic fibrosis is receiving immunizations. What is the primary reason for administering vaccinations to children with cystic fibrosis?
a) To prevent gastrointestinal infections and improve digestion
b) To decrease the risk of diabetes-related complications
c) To reduce the risk of viral and bacterial infections that may worsen lung function
d) To ensure adequate caloric intake and nutritional status
c) To reduce the risk of viral and bacterial infections that may worsen lung function
Rationale: Children with cystic fibrosis are at higher risk for respiratory infections, which can lead to further lung damage. Immunizations help prevent viral and bacterial infections that could exacerbate respiratory symptoms and decline in lung function.
A nurse is preparing to administer chest tube drainage for a child with cystic fibrosis who has developed a pneumothorax. What is the primary goal of this therapy?
a) To improve oxygenation and reduce respiratory distress
b) To remove excess mucus from the lungs
c) To correct electrolyte imbalances caused by the disease
d) To prevent bacterial infection in the lungs
a) To improve oxygenation and reduce respiratory distress
Rationale: Chest tube drainage is used to relieve pneumothorax by removing air from the pleural space, improving lung expansion, and reducing respiratory distress.
A child with cystic fibrosis is undergoing a thoracoscopy to repair ruptured alveoli. What is the primary benefit of this procedure for this patient?
a) It improves gastrointestinal function by relieving abdominal distention
b) It repairs areas of recurrent pneumothorax and prevents future episodes in the same location
c) It improves the absorption of pancreatic enzyme supplements
d) It helps in the management of cystic fibrosis-related diabetes
b) It repairs areas of recurrent pneumothorax and prevents future episodes in the same location
Rationale: Thoracoscopy is performed to repair ruptured alveoli, a common cause of recurrent pneumothorax in cystic fibrosis patients. The procedure helps prevent future episodes by sealing the damaged area of the lung.
A nurse is educating the parents of a child with cystic fibrosis about the importance of a high-calorie, high-fat diet. What is the rationale for this dietary recommendation?
a) It helps reduce mucus production in the lungs
b) It decreases the risk of pancreatitis and gastrointestinal complications
c) It improves insulin production and controls blood sugar levels
d) It promotes weight maintenance and growth while supporting lung function
d) It promotes weight maintenance and growth while supporting lung function
Rationale: A high-calorie, high-fat diet is essential for children with cystic fibrosis to support growth, weight maintenance, and increased lung capacity. It helps provide adequate energy to combat the high metabolic demands of the body and maintain nutritional status.
A 3-year-old with cystic fibrosis is diagnosed with meconium ileus. Which of the following treatments is most appropriate to relieve this condition?
a) Hyperosmolar enemas or isotonic fluid lavage
b) Pancreatic enzyme replacement therapy
c) High-calorie, high-fat diet
d) Vitamin A, D, E, and K supplementation
a) Hyperosmolar enemas or isotonic fluid lavage
Rationale: Meconium ileus is a bowel obstruction caused by thick meconium in newborns with cystic fibrosis. Hyperosmolar enemas or isotonic fluid lavage can help relieve the obstruction by flushing out the thickened meconium and improving intestinal motility.
A nurse is preparing to administer pancreatic enzyme supplements to a child with cystic fibrosis. What is the primary purpose of these supplements?
a) To improve lung function and prevent infections
b) To reduce gastric acid production and protect the pancreas
c) To increase insulin production and prevent diabetes
d) To promote digestion of fats and proteins and prevent malnutrition
d) To promote digestion of fats and proteins and prevent malnutrition
Rationale: Pancreatic enzyme supplements are essential for aiding the digestion of fats and proteins in children with cystic fibrosis, as their impaired pancreas cannot produce sufficient digestive enzymes. This prevents malnutrition and promotes adequate growth.
A nurse is teaching a child with cystic fibrosis about the role of exercise and physical fitness in managing the disease. What is the most important benefit of regular exercise for this child?
a) It reduces the frequency of gastrointestinal disturbances
b) It improves immune function and prevents respiratory infections
c) It helps maintain lung function and prevent further damage
d) It prevents electrolyte imbalances related to sweat gland dysfunction
c) It helps maintain lung function and prevent further damage
Rationale: Regular exercise is essential for maintaining lung function in children with cystic fibrosis. It helps improve airway clearance, reduces the risk of lung infections, and may slow the progression of respiratory decline.
A 6-year-old child with cystic fibrosis has been receiving acid suppression therapy. Which of the following is the most appropriate reason for administering this therapy to the child?
a) To reduce the risk of lung infections by improving immune function
b) To prevent the damage caused by gastroesophageal reflux, which worsens lung function
c) To reduce pancreatic enzyme secretion that could worsen abdominal distention
d) To improve digestion by enhancing the absorption of nutrients from the intestines
b) To prevent the damage caused by gastroesophageal reflux, which worsens lung function
Rationale: Acid suppression therapy is used to manage gastroesophageal reflux in cystic fibrosis patients. Reflux can worsen lung function by promoting aspiration of gastric contents into the lungs, contributing to respiratory infections and further damage.
A nurse is planning care for a 10-year-old child with cystic fibrosis and their family. Which of the following nursing diagnoses is most appropriate for this child?
a) Ineffective Airway Clearance related to thick mucus in lungs
b) Anxiety related to long-term effects of cystic fibrosis
c) Risk for Impaired Skin Integrity related to frequent respiratory treatments
d) Risk for Ineffective Coping related to uncertainty of long-term prognosis
a) Ineffective Airway Clearance related to thick mucus in lungs
Rationale: Ineffective airway clearance is a key nursing diagnosis for children with cystic fibrosis due to the thick mucus production in the lungs, which can lead to respiratory complications. This diagnosis will guide interventions aimed at improving airway clearance.
During a follow-up visit, a nurse notes that a child with cystic fibrosis is not maintaining an appropriate growth pattern. What should the nurse do first?
a) Increase the child’s caloric intake
b) Assess the child’s appetite and dietary intake
c) Initiate a referral to a dietitian for nutritional counseling
d) Ask the parents to begin supplemental feeding immediately
b) Assess the child’s appetite and dietary intake
Rationale: Before making any changes to the diet, it is important to assess the child’s current eating habits and identify any potential barriers to adequate nutrition, such as appetite changes or difficulties with food digestion.
A nurse is caring for a child with cystic fibrosis who is at risk for infection. Which of the following interventions is most important for preventing infections in the hospital setting?
a) Routinely administering prophylactic antibiotics
b) Rooming children with cystic fibrosis together for support
c) Practicing strict hand hygiene and isolation precautions
d) Limiting visitors to only the child’s immediate family members
c) Practicing strict hand hygiene and isolation precautions
Rationale: Strict hand hygiene and isolation precautions are essential to prevent cross-contamination and reduce the risk of hospital-acquired infections, particularly with highly transmissible pathogens like Pseudomonas aeruginosa.
The nurse is teaching the parents of a child with cystic fibrosis about airway clearance techniques. Which of the following is an appropriate instruction?
a) “Airway clearance techniques should only be performed during hospitalizations.”
b) “Coughing exercises should be performed before any medication is administered.”
c) “Airway clearance techniques should be avoided during episodes of fever.”
d) “Chest physiotherapy should be done twice a day to ensure optimal lung function.”
d) “Chest physiotherapy should be done twice a day to ensure optimal lung function.”
Rationale: Chest physiotherapy is an important component of the daily care routine for children with cystic fibrosis. It helps clear mucus from the airways and should be done regularly, typically twice a day, to maintain optimal lung function.
A nurse is conducting a psychosocial assessment of a child with cystic fibrosis and their family. Which statement by the parent indicates the need for further counseling?
a) “We have learned to manage the day-to-day care routine for our child’s condition.”
b) “I feel guilty for passing on the gene for cystic fibrosis to my child.”
c) “I try to shield my other children from learning too much about the disease.”
d) “The care routine is exhausting, but it has brought our family closer together.”
c) “I try to shield my other children from learning too much about the disease.”
Rationale: It’s important to address how the disease is communicated to siblings. Shielding them from the reality of cystic fibrosis may hinder their emotional adjustment and coping. Siblings need age-appropriate information about the disease.
When caring for a child with cystic fibrosis, which of the following is a priority nursing intervention during an exacerbation requiring hospitalization?
a) Initiating isolation precautions to prevent cross-infection with other cystic fibrosis patients
b) Administering a high-calorie, high-fat diet
c) Educating the child on performing airway clearance techniques independently
d) Monitoring the child’s emotional status and providing emotional support to the parents
a) Initiating isolation precautions to prevent cross-infection with other cystic fibrosis patients
Rationale: To prevent cross-infection with pathogens like Pseudomonas aeruginosa and Burkholderia cepacia, children with cystic fibrosis are placed in private rooms with standard precautions.
A child with cystic fibrosis is prescribed fat-soluble vitamin supplements (A, D, E, and K). What should the nurse teach the parents regarding these supplements?
a) “Ensure that the vitamins are given twice a day to promote absorption.”
b) “Administer the vitamins with a low-fat meal to ensure maximum absorption.”
c) “Fat-soluble vitamins are typically given in a liquid form only for children.”
d) “Give the vitamins only when the child is experiencing respiratory symptoms.”
a) “Ensure that the vitamins are given twice a day to promote absorption.”
Rationale: Fat-soluble vitamins should be given twice daily to ensure adequate absorption, as they may not be well absorbed in children with cystic fibrosis due to pancreatic insufficiency and poor fat digestion.
A nurse is administering an aerosolized bronchodilator to a child with cystic fibrosis before an airway clearance procedure. What is the most important nursing action?
a) Encourage the child to breathe rapidly to open the airways
b) Ensure the child holds their breath for 10 seconds after inhalation
c) Instruct the child to cough forcefully after the inhalation
d) Administer the medication after performing chest physiotherapy
b) Ensure the child holds their breath for 10 seconds after inhalation
Rationale: The child should hold their breath for 10 seconds after inhalation to allow the bronchodilator to work effectively, opening the airways before airway clearance techniques are performed.
A nurse is teaching the family of a child with cystic fibrosis about pancreatic enzyme supplements. Which of the following is the most important instruction regarding the administration of these supplements?
a) “Administer the enzymes with a high-fat meal only.”
b) “Ensure enzymes are given with meals and snacks to aid digestion.”
c) “Give the enzymes on an empty stomach for better absorption.”
d) “The enzymes should be taken after meals to prevent stomach upset.”
b) “Ensure enzymes are given with meals and snacks to aid digestion.”
Rationale: Pancreatic enzyme supplements should be taken with meals and snacks to aid in the digestion of fats, proteins, and carbohydrates, which are often poorly digested in cystic fibrosis patients due to pancreatic insufficiency.
A child with cystic fibrosis is prescribed ibuprofen to slow the progression of lung function decline. What should the nurse emphasize to the parents regarding this medication?
a) “Make sure your child avoids taking any vitamin supplements with this medication.”
b) “Monitor for signs of gastrointestinal bleeding, such as black, tarry stools.”
c) “This medication should be taken on an empty stomach for optimal absorption.”
d) “It is safe to take aspirin along with ibuprofen for enhanced lung protection.”
b) “Monitor for signs of gastrointestinal bleeding, such as black, tarry stools.”
Rationale: Ibuprofen can cause gastrointestinal irritation and bleeding. Parents should monitor for signs of bleeding and should not give their child aspirin or other NSAIDs unless approved by the healthcare provider.
A nurse is teaching the parents of a child with cystic fibrosis about the use of aerosolized hypertonic saline (7%). What is the primary action of this medication?
a) To hydrate the airway mucus and stimulate coughing
b) To prevent gastrointestinal reflux
c) To increase mucus production and promote airway clearance
d) To reduce airway hyperresponsiveness and prevent bronchospasm
a) To hydrate the airway mucus and stimulate coughing
Rationale: Hypertonic saline (7%) is used to hydrate the mucus in the airways, stimulating coughing and improving lung function by promoting mucus clearance.
A nurse is administering aerosolized dornase alpha (Pulmozyme) to a child with cystic fibrosis. What is the primary purpose of this medication?
a) To promote bronchial dilation and prevent bronchospasm
b) To hydrate airway mucus and improve coughing
c) To loosen, liquefy, and thin pulmonary secretions
d) To treat bacterial infections in the lungs
c) To loosen, liquefy, and thin pulmonary secretions
Rationale: Dornase alpha (Pulmozyme) is used to thin and break down pulmonary secretions in cystic fibrosis patients, making it easier to clear mucus from the airways and reduce exacerbations.
A nurse is caring for an infant diagnosed with RSV bronchiolitis. Which of the following symptoms is most concerning and indicates the need for immediate intervention?
a) Rhinitis and mild cough
b) Tachypnea greater than 70 breaths per minute
c) Nasal flaring and poor feeding
d) Low-grade fever and irritability
b) Tachypnea greater than 70 breaths per minute
Rationale: Tachypnea greater than 70 breaths per minute is a sign of severe respiratory distress and indicates that the infant may be at risk for respiratory failure, requiring immediate intervention.
Which of the following infants is at highest risk for developing severe RSV infection?
a) An infant born at 35 weeks’ gestation
b) An infant with a history of asthma
c) A 10-month-old infant with a mild upper respiratory infection
d) An infant born at 28 weeks’ gestation who required supplemental oxygen for the first few weeks of life
d) An infant born at 28 weeks’ gestation who required supplemental oxygen for the first few weeks of life
Rationale: Infants born prematurely (less than 32 weeks’ gestation) and those requiring supplemental oxygen are at higher risk for severe RSV infection due to their immature lungs and respiratory system.
A nurse is caring for a child with RSV. The child has increasing irritability, nasal flaring, and wheezing. The nurse notes diminishing breath sounds. What is the nurse’s priority action?
a) Administer nebulized hypertonic saline
b) Reassess the child’s respiratory status frequently
c) Initiate chest physiotherapy to clear the airways
d) Provide a dose of corticosteroids
b) Reassess the child’s respiratory status frequently
Rationale: Diminishing breath sounds indicate that airflow is decreasing, which may signal worsening respiratory status. Frequent reassessment is essential to determine if further interventions, such as oxygen or mechanical ventilation, are needed.
Which of the following is the most appropriate initial treatment for an infant with severe RSV bronchiolitis and hypoxemia?
a) Nebulized albuterol
b) Humidified oxygen therapy
c) Intravenous antibiotics
d) Oral corticosteroids
b) Humidified oxygen therapy
Rationale: Humidified oxygen therapy is the standard treatment for infants with severe RSV bronchiolitis and hypoxemia to maintain adequate oxygen saturation and relieve respiratory distress.
A nurse is preparing to administer palivizumab (Synagis) to a high-risk infant. Which of the following is the correct administration schedule for this medication?
a) Every month for 5 months, starting in October
b) Once during the RSV season, before the first symptoms appear
c) Every 2 months for 3 months, starting in January
d) Monthly for 12 months, starting in January
a) Every month for 5 months, starting in October
Rationale: Palivizumab is administered once a month for 5 months, starting in October or November, at the onset of the RSV season, to help prevent severe RSV infection in high-risk infants.
Which of the following interventions should be avoided in the management of an infant with RSV bronchiolitis?
a) Nasal suctioning before feeding
b) Continuous positive airway pressure (CPAP)
c) Chest physiotherapy
d) Intravenous hydration
c) Chest physiotherapy
Rationale: Chest physiotherapy is not recommended for infants with RSV bronchiolitis as it can be ineffective and potentially harmful in clearing the mucus, and may cause further respiratory distress.