Review Cystic Fibrosis

Pathophysiology of the respiratory system > Pathophysiology

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Cystic Fibrosis

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Question 1:

A client is inquiring about the genetic basis of cystic fibrosis (CF). Which statement by the nurse provides an accurate explanation?

A. "CF is caused by a dominant genetic mutation that always manifests in affected individuals." B. "CF results from the presence of two recessive genetic mutations, one inherited from each parent." C. "CF is due to a spontaneous genetic mutation that occurs during a person's lifetime." D. "CF is exclusively caused by environmental factors and is not related to genetics."

Answer and Explanation

Choice A:

Incorrect. CF is an autosomal recessive genetic disorder, meaning that both copies of the CFTR gene must carry a mutation for the condition to manifest.

Choice B:

Correct. CF is caused by the inheritance of two recessive genetic mutations, one from each parent, resulting in defective CFTR protein function.

Choice C:

Incorrect. CF is primarily a genetic disorder present from birth, not one caused by spontaneous mutations during a person's lifetime.

Choice D:

Incorrect. CF is primarily a genetic disorder with well-established genetic mutations associated with its development.

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Question 2:

A nurse is explaining the typical clinical manifestations of cystic fibrosis (CF) to a newly diagnosed adolescent client. Which symptom should the nurse emphasize as a hallmark of CF?

A. Frequent nosebleeds B. Chronic cough with thick mucus production C. Rapid weight gain D. High blood pressure

Answer and Explanation

Choice A:

Incorrect. Frequent nosebleeds are not a hallmark symptom of CF.

Choice B:

Correct. A chronic cough with thick, sticky mucus production is a hallmark feature of CF due to the accumulation of mucus in the respiratory tract.

Choice C:

Incorrect. Rapid weight gain is not a typical symptom of CF; in fact, individuals with CF often struggle with poor weight gain and malnutrition.

Choice D:

Incorrect. High blood pressure is not a characteristic symptom of CF.

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Question 3:

A client with cystic fibrosis (CF) asks the nurse about the impact of CF on the respiratory system. What response by the nurse accurately describes the respiratory complications associated with CF?

A. "CF primarily affects the upper respiratory tract, leading to frequent sinus infections." B. "CF causes inflammation and obstruction of the airways, leading to chronic lung infections and lung damage." C. "CF typically results in enlarged tonsils and adenoids, which can affect breathing." D. "CF mainly affects the bronchial tubes, causing chronic cough but not lung infections."

Answer and Explanation

Choice A:

Incorrect. CF primarily affects the lower respiratory tract, particularly the bronchi and bronchioles, rather than the upper respiratory tract.

Choice B:

Correct. CF leads to thickened mucus in the airways, which can cause inflammation, recurrent infections, and progressive lung damage.

Choice C:

Incorrect. Enlarged tonsils and adenoids are not a typical manifestation of CF-related respiratory complications.

Choice D:

Incorrect. CF affects both the bronchial tubes and the entire respiratory system, often leading to chronic lung infections.

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Question 4:

A nurse is discussing the impact of cystic fibrosis (CF) on the digestive system with a client. Which statement accurately describes a common gastrointestinal complication of CF?

A. "CF primarily affects the stomach, leading to frequent episodes of gastritis." B. "CF causes inflammation of the liver, resulting in jaundice and liver failure." C. "CF can obstruct the pancreatic ducts, leading to pancreatic insufficiency and malabsorption of nutrients." D. "CF mainly affects the large intestine, leading to chronic constipation."

Answer and Explanation

Choice A:

Incorrect. CF primarily affects the pancreas and small intestine, not the stomach.

Choice B:

Incorrect. While liver complications can occur in some individuals with CF, such as liver disease or cirrhosis, it is not a primary manifestation of the disease.

Choice C:

Correct. CF often results in thickened mucus obstructing the pancreatic ducts, causing pancreatic insufficiency and malabsorption of essential nutrients.

Choice D:

Incorrect. CF primarily affects the pancreas and small intestine rather than the large intestine, and chronic constipation is not a typical symptom of CF.

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Question 5:

A nurse is counseling a couple planning to start a family, and both partners have a family history of cystic fibrosis (CF). What should the nurse advise the couple regarding the genetic risk of having a child with CF?

A. "You both have a family history of CF, so it is impossible for your child to inherit the condition." B. "Since you both carry a single CF gene mutation, your child has a 100% chance of developing CF." C. "Your child will inherit CF only if both of you pass on a copy of the CF gene mutation." D. "The risk of your child having CF is solely determined by the mother's genetic history."

Answer and Explanation

Choice A:

Incorrect. Both partners having a family history of CF increases the likelihood of carrying CF gene mutations, making it possible for their child to inherit the condition.

Choice B:

Incorrect. Having a single CF gene mutation (carrier status) does not guarantee that a child will develop CF; it requires inheriting two CF gene mutations, one from each parent.

Choice C:

Correct. CF is an autosomal recessive genetic disorder, which means that a child will have CF only if they inherit two copies of the CF gene mutation, one from each parent.

Choice D:

Incorrect. The risk of a child having CF is determined by both parents' genetic history, not solely the mother's.

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Question 6:

A nurse is explaining the pathophysiology of cystic fibrosis (CF) to a newly diagnosed patient. Which statement accurately describes the genetic basis of CF?

A. "CF results from a dominant genetic mutation inherited from one parent." B. "CF occurs due to the presence of two dominant genetic mutations." C. "CF is caused by a recessive genetic mutation inherited from both parents." D. "CF is a non-genetic condition caused by environmental factors."

Answer and Explanation

Choice A:

Incorrect. CF is an autosomal recessive genetic disorder, meaning that both copies of the CFTR gene must carry a mutation for the condition to manifest.

Choice B:

Incorrect. CF is not caused by dominant genetic mutations.

Choice C:

Correct. CF results from inheriting two recessive genetic mutations, one from each parent, leading to defective CFTR protein function.

Choice D:

Incorrect. CF is primarily a genetic disorder with well-established genetic mutations associated with its development.

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Question 7:

A nurse is discussing the role of the cystic fibrosis transmembrane conductance regulator (CFTR) protein with a patient. What is the primary function of the CFTR protein in normal physiology?

A. "The CFTR protein regulates sodium absorption in the kidneys." B. "The CFTR protein facilitates chloride secretion in the gastrointestinal tract." C. "The CFTR protein controls the production of red blood cells in the bone marrow." D. "The CFTR protein regulates mucus production and the movement of chloride ions in the respiratory and digestive systems."

Answer and Explanation

Choice A:

Incorrect. The CFTR protein primarily functions in the respiratory and digestive systems, not the kidneys.

Choice B:

Incorrect. While CFTR plays a role in chloride secretion, its primary role is related to mucus production and chloride ion movement in the respiratory and digestive systems.

Choice C:

Incorrect. The CFTR protein is not involved in the production of red blood cells.

Choice D:

Correct. The CFTR protein is responsible for regulating mucus consistency and chloride ion transport in the respiratory and digestive systems.

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Question 8:

A nurse is discussing the underlying genetic defect in cystic fibrosis (CF) with a client. Which of the following statements accurately describes the primary genetic abnormality in CF?

A. "CF is caused by an extra chromosome, leading to genetic imbalance." B. "In CF, there is a duplication of the CFTR gene on chromosome 7." C. "CF results from a mutation in the CFTR gene, which codes for a dysfunctional chloride channel." D. "CF is characterized by the absence of the CFTR gene in affected individuals."

Answer and Explanation

Choice A:

Incorrect. CF is not caused by an extra chromosome; it is a genetic disorder resulting from mutations in specific genes.

Choice B:

Incorrect. CF is not characterized by a duplication of the CFTR gene but rather by various mutations in this gene.

Choice C:

Correct. CF is primarily caused by mutations in the CFTR (cystic fibrosis transmembrane conductance regulator) gene, leading to the production of dysfunctional chloride channels.

Choice D:

Incorrect. CF is not characterized by the absence of the CFTR gene; instead, it involves various mutations in this gene.

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Question 9:

A nurse is explaining the role of the CFTR protein in cystic fibrosis to a client. Which function of the CFTR protein should the nurse highlight?

A. "The CFTR protein regulates blood sugar levels in the body." B. "CFTR facilitates the transport of oxygen in the bloodstream." C. "The CFTR protein controls the movement of chloride ions and water across cell membranes." D. "CFTR is responsible for the production of digestive enzymes in the pancreas."

Answer and Explanation

Choice A:

Incorrect. The CFTR protein does not regulate blood sugar levels.

Choice B:

Incorrect. CFTR does not play a role in oxygen transport in the bloodstream.

Choice C:

Correct. The primary function of the CFTR protein is to regulate chloride ion and water movement across cell membranes, which is disrupted in individuals with CF.

Choice D:

Incorrect. The production of digestive enzymes in the pancreas is not a direct function of the CFTR protein.

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Question 10:

A nurse is discussing the pathophysiological consequences of cystic fibrosis (CF) with a client. Which of the following best describes how CF affects the respiratory system?

A. "CF primarily causes airway constriction due to inflammation." B. "In CF, the thickened mucus in the airways leads to chronic inflammation, infections, and lung damage." C. "CF results in the overproduction of surfactant, causing respiratory distress." D. "The CFTR protein in the lungs fails to produce enough oxygen for normal breathing."

Answer and Explanation

Choice A:

Incorrect. While inflammation can be a consequence of CF, it is not the primary cause of airway constriction.

Choice B:

Correct. In CF, thickened mucus accumulates in the airways, leading to chronic inflammation, recurrent infections, and progressive lung damage.

Choice C:

Incorrect. CF does not result in the overproduction of surfactant, which is a substance that helps reduce surface tension in the lungs.

Choice D:

Incorrect. The CFTR protein does not produce oxygen but rather regulates chloride ion and water transport in the lungs

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Question 11:

A nurse is educating a client about the etiology of cystic fibrosis (CF). Which statement by the client accurately describes the genetic basis of CF?

A. "CF is caused by a single gene mutation, which can be inherited from one parent." B. "CF results from a combination of genetic and environmental factors." C. "CF occurs due to an X-linked genetic mutation passed from mother to daughter." D. "CF is primarily caused by exposure to respiratory pathogens during infancy."

Answer and Explanation

Choice A:

Correct. CF is primarily caused by mutations in a single gene, the CFTR gene, and it can be inherited from one parent, resulting in a carrier state, or from both parents for the disease to manifest.

Choice B:

Incorrect. While genetic factors are the primary cause of CF, environmental factors can exacerbate symptoms but are not the root cause.

Choice C:

Incorrect. CF is not an X-linked genetic disorder; it follows an autosomal recessive pattern of inheritance.

Choice D:

Incorrect. CF is not caused by exposure to respiratory pathogens during infancy; it is primarily a genetic disorder.

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Question 12:

A nurse is discussing the risk factors for cystic fibrosis (CF) with a client. Which statement accurately identifies a risk factor associated with CF?

A. "CF is more common in females than males." B. "Being of African descent significantly increases the risk of developing CF." C. "A family history of CF is a risk factor, as it increases the likelihood of inheriting the mutated gene." D. "CF is unrelated to ethnicity, and anyone can be equally affected."

Answer and Explanation

Choice A:

Incorrect. CF occurs at a similar frequency in both males and females.

Choice B:

Incorrect. CF is not significantly associated with any specific ethnicity, and the risk is not influenced by being of African descent.

Choice C:

Correct. A family history of CF increases the risk because CF is an autosomal recessive genetic disorder, and having affected family members increases the likelihood of carrying CF gene mutations.

Choice D:

Incorrect. While CF can affect individuals from any ethnicity, it is primarily a genetic disorder, and family history plays a crucial role in the risk of inheritance.

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Question 13:

A nurse is educating a client about the inheritance pattern of cystic fibrosis (CF). What information should the nurse provide regarding the likelihood of a child developing CF if both parents are carriers?

A. "If both parents are carriers, there is a 50% chance that their child will have CF." B. "If both parents are carriers, their child is guaranteed to have CF." C. "If both parents are carriers, their child will be a carrier but will not have CF." D. "If both parents are carriers, their child will have a 25% chance of having CF."

Answer and Explanation

Choice A:

Incorrect. If both parents are carriers, their child's risk of having CF is 25%, not 50%.

Choice B:

Incorrect. While the risk is increased, having two carrier parents does not guarantee that a child will have CF.

Choice C:

Incorrect. If both parents are carriers, their child can inherit two normal genes (no CF), one normal and one CF gene (carrier status), or two CF genes (CF).

Choice D:

Correct. When both parents are carriers, there is a 25% chance that their child will inherit two CF genes and have CF, a 50% chance of being a carrier, and a 25% chance of inheriting two normal genes (no CF).

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Question 14:

A nurse is discussing the role of genetic testing in cystic fibrosis (CF) diagnosis with a client. What should the nurse emphasize regarding the importance of genetic testing for CF?

A. "Genetic testing is primarily used to diagnose CF in infants but is not necessary for older individuals." B. "Genetic testing is essential for diagnosing CF at any age, as it can confirm the presence of CF gene mutations." C. "Genetic testing is mainly used to determine the severity of CF symptoms but does not aid in diagnosis." D. "Genetic testing is reserved for family members of individuals already diagnosed with CF."

Answer and Explanation

Choice A:

Incorrect. Genetic testing for CF is important for individuals of all ages, not limited to infants.

Choice B:

Correct. Genetic testing is a crucial diagnostic tool for confirming the presence of CF gene mutations, regardless of the individual's age.

Choice C:

Incorrect. Genetic testing is primarily used for diagnosis, not for determining the severity of CF symptoms.

Choice D:

Incorrect. Genetic testing is not reserved solely for family members of individuals already diagnosed with CF; it is used for diagnostic purposes in suspected cases of CF.

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Question 15:

A nurse is discussing the clinical manifestations of cystic fibrosis (CF) with a newly diagnosed adolescent client. What common respiratory symptom should the nurse emphasize as an early sign of CF?

A. Wheezing and chest pain B. Chronic cough with thick mucus production C. Blood in the urine D. Digestive discomfort and diarrhea

Answer and Explanation

Choice A:

Incorrect. Wheezing and chest pain can occur in CF, but a chronic cough with thick mucus production is often one of the earliest respiratory symptoms.

Choice B:

Correct. A chronic cough with thick, sticky mucus is a hallmark symptom of CF due to the accumulation of mucus in the respiratory tract.

Choice C:

Incorrect. Blood in the urine is not a common early sign of CF; it may indicate other issues.

Choice D:

Incorrect. Digestive discomfort and diarrhea are more related to CF's impact on the gastrointestinal system, rather than being early respiratory symptoms.

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Question 16:

A nurse is caring for a child with cystic fibrosis (CF). What gastrointestinal symptom commonly observed in CF should the nurse be vigilant for?

A. Frequent nosebleeds B. Abdominal pain and bloating C. Rapid weight gain D. Muscle weakness

Answer and Explanation

Choice A:

Incorrect. Frequent nosebleeds are not common gastrointestinal symptoms in CF.

Choice B:

Correct. Abdominal pain and bloating are common gastrointestinal symptoms in CF, often due to the impact of thickened mucus on the pancreas and intestines.

Choice C:

Incorrect. Rapid weight gain is not a typical gastrointestinal symptom in CF; individuals with CF often struggle with poor weight gain and malnutrition.

Choice D:

Incorrect. Muscle weakness is not a common gastrointestinal symptom in CF but may be associated with other complications.

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Question 17:

A client with cystic fibrosis (CF) presents with clubbed fingers during a routine examination. What should the nurse explain to the client regarding the significance of clubbing in CF?

A. "Clubbed fingers are a common cosmetic concern but do not indicate any medical issues in CF." B. "Clubbing of the fingers can be a sign of advanced lung disease or decreased oxygen levels in CF." C. "Clubbing occurs as a result of excessive sodium intake, which is often seen in CF patients." D. "Clubbing is primarily associated with digestive problems in CF and does not affect the respiratory system."

Answer and Explanation

Choice A:

Incorrect. Clubbed fingers in CF can be more than a cosmetic concern and may indicate underlying medical issues.

Choice B:

Correct. Clubbing of the fingers can be associated with advanced lung disease or reduced oxygen levels in CF.

Choice C:

Incorrect. Clubbing is not related to excessive sodium intake in CF patients.

Choice D:

Incorrect. While CF can affect the digestive system, clubbing of the fingers is primarily associated with respiratory issues.

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Question 18:

A nurse is assessing a client with cystic fibrosis (CF). What respiratory symptom should the nurse expect to find in a client with advanced CF-related lung disease?

A. Dry, nonproductive cough B. Decreased breath sounds C. Hematuria D. Rapid weight gain

Answer and Explanation

Choice A:

Incorrect. In advanced CF-related lung disease, a chronic, productive cough with thick mucus production is more common than a dry, nonproductive cough.

Choice B:

Correct. Decreased breath sounds may occur in clients with advanced CF-related lung disease due to airway obstruction and lung damage.

Choice C:

Incorrect. Hematuria is not a typical respiratory symptom of CF.

Choice D:

Incorrect. Rapid weight gain is not associated with advanced CF-related lung disease; individuals with advanced CF often experience weight loss and malnutrition.

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Question 19:

A nurse is educating a client with cystic fibrosis (CF) about potential complications of the disease. Which of the following should the nurse mention as a common complication of CF in adults?

A. Meconium ileus in newborns B. Failure to thrive in infants C. Osteoporosis in adults D. Rapid growth and development in adolescents

Answer and Explanation

Choice A:

Incorrect. Meconium ileus is a complication typically seen in newborns with CF, not in adults.

Choice B:

Incorrect. Failure to thrive is a concern in infants and young children with CF but not typically in adults.

Choice C:

Correct. Osteoporosis is a common complication in adults with CF due to malabsorption of calcium and vitamin D, chronic inflammation, and reduced physical activity.

Choice D:

Incorrect. Rapid growth and development are typically observed in adolescents, not as a complication but as a part of normal growth.

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Question 20:

A nurse is educating a client about the diagnostic evaluation of cystic fibrosis (CF). What test is commonly used to screen for CF in newborns shortly after birth?

A. Sweat chloride test B. Chest X-ray C. Pulmonary function test D. Complete blood count (CBC)

Answer and Explanation

Choice A:

Correct. The sweat chloride test is commonly used for newborn screening for CF. Elevated sweat chloride levels are indicative of CF.

Choice B:

Incorrect. Chest X-rays may be used to assess lung changes in individuals with CF but are not a primary diagnostic test, especially in newborns.

Choice C:

Incorrect. Pulmonary function tests are used to evaluate lung function but are not typically performed as part of newborn screening for CF.

Choice D:

Incorrect. A complete blood count (CBC) is a general blood test and is not specific for CF diagnosis.

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Question 21:

A nurse is explaining the diagnostic process for cystic fibrosis (CF) to a client and their family. What initial diagnostic test is often used to assess CF in infants who exhibit symptoms suggestive of the condition?

A. Genetic testing B. Sweat chloride test C. Chest X-ray D. Pulmonary function test

Answer and Explanation

Choice A:

Incorrect. Genetic testing can confirm CF but is not typically the initial diagnostic test used in infants with symptoms suggestive of CF.

Choice B:

Correct. The sweat chloride test is often used as the initial diagnostic test to assess for CF in infants who exhibit symptoms.

Choice C:

Incorrect. Chest X-rays may be used to assess lung changes in individuals with CF but are not typically the initial diagnostic test in infants.

Choice D:

Incorrect. Pulmonary function tests are used to evaluate lung function but are not typically the initial diagnostic test for CF in infants.

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Question 22:

A nurse is discussing the diagnostic evaluation of cystic fibrosis (CF) with a client. What test is commonly performed to assess lung function and monitor disease progression in individuals with CF?

A. Genetic testing B. Sweat chloride test C. Chest X-ray D. Pulmonary function test

Answer and Explanation

Choice A:

Incorrect. Genetic testing is used to confirm the presence of CF gene mutations but is not a measure of lung function.

Choice B:

Incorrect. The sweat chloride test is used for CF diagnosis and may not be performed regularly to monitor lung function.

Choice C:

Incorrect. Chest X-rays may be used to assess lung changes in individuals with CF, but they do not directly measure lung function.

Choice D:

Correct. Pulmonary function tests are commonly performed to assess lung function, monitor disease progression, and guide treatment in individuals with CF.

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Question 23:

A client with suspected cystic fibrosis (CF) is undergoing diagnostic testing. What diagnostic procedure is often used to confirm CF by analyzing the genetic mutations present?

A. Sweat chloride test B. Chest X-ray C. Pulmonary function test D. Genetic testing

Answer and Explanation

Choice A:

Incorrect. The sweat chloride test is used for CF diagnosis, but it does not analyze genetic mutations.

Choice B:

Incorrect. Chest X-rays are used to assess lung changes but do not directly confirm CF by analyzing genetic mutations.

Choice C:

Incorrect. Pulmonary function tests assess lung function but do not confirm CF by analyzing genetic mutations.

Choice D:

Correct. Genetic testing is a diagnostic procedure commonly used to confirm CF by analyzing the specific CF gene mutations present in an individual.

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Question 24:

A nurse is educating a client about the diagnostic process for cystic fibrosis (CF). What diagnostic test measures the concentration of chloride ions in sweat and is often used to confirm CF?

A. Genetic testing B. Chest X-ray C. Pulmonary function test D. Sweat chloride test

Answer and Explanation

Choice A:

Incorrect. Genetic testing is used to confirm CF but does not directly measure chloride ion concentration in sweat.

Choice B:

Incorrect. Chest X-rays may be used to assess lung changes in individuals with CF but are not used to measure chloride ion concentration in sweat.

Choice C:

Incorrect. Pulmonary function tests assess lung function but do not directly measure chloride ion concentration in sweat.

Choice D:

Correct. The sweat chloride test measures the concentration of chloride ions in sweat and is commonly used to confirm CF. Elevated sweat chloride levels are indicative of CF.

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Question 25:

A nurse is educating a client with cystic fibrosis (CF) about airway clearance techniques. Which of the following airway clearance methods involves the use of a mechanical device to deliver high-frequency chest wall oscillations?

A. Postural drainage B. Autogenic drainage C. Positive expiratory pressure (PEP) therapy D. High-frequency chest wall oscillation (HFCWO)

Answer and Explanation

Choice A:

Incorrect. Postural drainage involves positioning the body to help drain mucus but does not use a mechanical device.

Choice B:

Incorrect. Autogenic drainage is a technique that involves controlled breathing and airflow, not a mechanical device.

Choice C:

Incorrect. PEP therapy uses a device to provide positive pressure during exhalation but is different from HFCWO.

Choice D:

Correct. High-frequency chest wall oscillation (HFCWO) involves using a mechanical device to deliver oscillations to the chest wall, helping to mobilize and clear mucus from the airways.

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Question 26:

A nurse is discussing nutritional management with a client who has cystic fibrosis (CF). What dietary recommendation should the nurse emphasize to optimize nutritional intake in individuals with CF?

A. Limit carbohydrate intake to control blood sugar levels. B. Increase sodium intake to maintain electrolyte balance. C. Consume a high-calorie diet rich in fats and proteins. D. Restrict fluid intake to prevent excessive mucus production.

Answer and Explanation

Choice A:

Incorrect. While monitoring carbohydrate intake is important in CF, limiting carbohydrates is not the primary focus of nutrition management.

Choice B:

Incorrect. Increasing sodium intake is not a general recommendation for individuals with CF; it depends on the individual's needs.

Choice C:

Correct. Individuals with CF often require a high-calorie diet rich in fats and proteins to address malabsorption and maintain adequate nutrition.

Choice D:

Incorrect. Restricting fluid intake is not recommended for individuals with CF, as staying hydrated is important for preventing thickening of mucus.

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Question 27:

A client with cystic fibrosis (CF) is experiencing a pulmonary exacerbation. What treatment approach should the nurse anticipate to help manage this acute worsening of symptoms?

A. Initiation of long-term oxygen therapy B. Administration of bronchodilators and corticosteroids C. Surgical lung transplantation D. Recommending bed rest and minimal physical activity

Answer and Explanation

Choice A:

Incorrect. Long-term oxygen therapy is typically reserved for individuals with chronic hypoxemia and is not the primary treatment for a pulmonary exacerbation.

Choice B:

Correct. Bronchodilators and corticosteroids are commonly used to manage pulmonary exacerbations in CF by reducing airway inflammation and improving airflow.

Choice C:

Incorrect. Surgical lung transplantation is considered in severe cases of CF with irreversible lung damage but is not typically the first-line treatment for a pulmonary exacerbation.

Choice D:

Incorrect. Bed rest and minimal physical activity are not recommended as the primary treatment for pulmonary exacerbations; maintaining mobility and airway clearance techniques are important.

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Question 28:

A nurse is educating a client with cystic fibrosis (CF) about the importance of airway clearance techniques. What should the nurse explain as the primary goal of these techniques?

A. To improve lung capacity B. To prevent respiratory infections C. To maintain electrolyte balance D. To clear mucus and secretions from the airways

Answer and Explanation

Choice A:

Incorrect. While airway clearance techniques can help maintain lung function, their primary goal is not to improve lung capacity.

Choice B:

Incorrect. Preventing respiratory infections is an important aspect of CF care, but it is not the primary goal of airway clearance techniques.

Choice C:

Incorrect. Maintaining electrolyte balance is important in CF, but it is not the primary goal of airway clearance techniques.

Choice D:

Correct. The primary goal of airway clearance techniques in CF is to mobilize and clear mucus and secretions from the airways, improving breathing and reducing the risk of infection.

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Question 29:

A nurse is discussing pancreatic enzyme replacement therapy (PERT) with a client who has cystic fibrosis (CF). What should the nurse emphasize as the purpose of PERT in CF management?

A. To control blood sugar levels B. To improve lung function C. To aid in the digestion of fats and nutrients D. To increase mucus production in the airways

Answer and Explanation

Choice A:

Incorrect. PERT is not primarily used to control blood sugar levels; it is used to improve nutrient absorption.

Choice B:

Incorrect. While CF management aims to improve lung function, PERT is specifically used to address pancreatic insufficiency and digestive issues.

Choice C:

Correct. The primary purpose of PERT in CF management is to replace the missing digestive enzymes and aid in the digestion of fats and nutrients.

Choice D:

Incorrect. PERT does not increase mucus production in the airways; it is focused on the digestive system.

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Question 30:

A nurse is providing education to a client with cystic fibrosis (CF). Which dietary recommendation is most appropriate for the client?

A. "Limit your protein intake to reduce mucus production." B. "Consume a high-fat diet to promote weight gain and calorie absorption." C. "Avoid all dairy products to prevent lung congestion." D. "Increase your intake of sugary snacks for quick energy."

Answer and Explanation

Choice A:

Incorrect. Protein intake is important for growth and repair but should not be limited in CF. High-calorie intake is more critical.

Choice B:

Correct. CF can lead to malabsorption of fat-soluble vitamins and calories, so a high-fat diet is recommended to support weight gain and nutrient absorption.

Choice C:

Incorrect. Dairy products are a good source of calcium and calories, and they are generally well-tolerated by CF patients. Avoiding them is not necessary.

Choice D:

Incorrect. Increasing sugary snacks is not advisable for CF patients. They need a balanced diet to meet their nutritional needs.

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Question 31:

A nurse is caring for a pediatric client with cystic fibrosis. Which respiratory therapy technique should the nurse prioritize to help manage the client's condition?

A. Postural drainage and chest percussion B. Administering bronchodilators as needed C. Providing oxygen therapy around the clock D. Encouraging deep breathing exercises only during illness

Answer and Explanation

Choice A:

Correct. Postural drainage and chest percussion help mobilize and remove thick, sticky mucus from the airways, which is crucial in managing CF.

Choice B:

Incorrect. While bronchodilators may be used, they are not the primary therapy for managing CF. Airway clearance techniques like postural drainage are more important.

Choice C:

Incorrect. Oxygen therapy should be administered as needed, but it is not required around the clock for all CF patients.

Choice D:

Incorrect. Deep breathing exercises should be encouraged regularly, not just during illness, to maintain airway clearance and lung function.

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Question 32:

A nurse is assessing a client with cystic fibrosis. Which clinical manifestation is commonly associated with CF and should be monitored closely?

A. Hypertension B. Hypoglycemia C. Chronic diarrhea D. Chronic respiratory infections

Answer and Explanation

Choice A:

Incorrect. Hypertension is not typically associated with CF.

Choice B:

Incorrect. Hypoglycemia is not a common manifestation of CF.

Choice C:

Incorrect. Chronic diarrhea is not a common symptom of CF.

Choice D:

Correct. CF is characterized by thick, sticky mucus that can lead to chronic respiratory infections, making this a key clinical manifestation to monitor.

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Question 33:

A nurse is teaching a client with cystic fibrosis about airway clearance techniques. Which technique should the nurse recommend that the client perform daily?

A. Coughing forcefully B. Controlled breathing exercises C. Huff coughing D. Exercising vigorously

Answer and Explanation

Choice A:

Incorrect. While coughing is important, huff coughing is a more effective and less strenuous airway clearance technique for CF patients.

Choice B:

Incorrect. Controlled breathing exercises may help with relaxation but are not the primary airway clearance technique.

Choice C:

Correct. Huff coughing is a technique where the client takes a deep breath and forcefully exhales in short bursts, helping to move mucus from the airways.

Choice D:

Incorrect. Vigorous exercise is beneficial for CF patients but should not replace specific airway clearance techniques.

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Question 34:

A nurse is providing discharge instructions to the parents of a child with cystic fibrosis. Which statement by the parents indicates a need for further education?

A. "We should encourage our child to drink plenty of water throughout the day." B. "We need to administer all prescribed medications, even if our child seems fine." C. "Our child should avoid close contact with other children to prevent infection." D. "Regular follow-up appointments with the CF care team are essential for our child's health."

Answer and Explanation

Choice A:

Correct. Staying well-hydrated is important for CF patients to help keep mucus thin and easier to clear.

Choice B:

Correct. It's important to administer all prescribed medications as directed to manage the condition effectively.

Choice C:

Incorrect. While infection control is important, it is not necessary for the child to avoid close contact with other children. Common-sense hygiene measures should be followed.

Choice D:

Correct. Regular follow-up appointments with the CF care team are crucial to monitor the child's health and adjust the treatment plan as needed.

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Cystic Fibrosis

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