MSN Exam for Cystic Fibrosis

EXPLANATION

 

✔Correct answer:

All of the choices. Cystic fibrosis (CF) is a genetic disorder that affects the exocrine glands, leading to the production of thick, sticky mucus. This mucus can cause blockages and infections in various organs and systems of the body. Therefore, all the listed options are correct as CF impacts multiple systems, including the pancreas, sweat glands, and lungs.

1. Pancreas: In CF, the thick mucus can block the pancreatic ducts, preventing digestive enzymes from reaching the intestines. This can lead to malabsorption of nutrients, poor growth, and difficulty gaining weight. The blocked ducts can also cause inflammation and damage to the pancreas, potentially leading to diabetes.
2. Sweat Glands: CF affects the sweat glands by causing them to produce sweat that is saltier than normal. This is due to a defect in the CFTR protein, which affects salt transport in and out of cells. Patients with CF can lose large amounts of salt when they sweat, leading to dehydration and electrolyte imbalances.
3. Lungs: The respiratory system is significantly affected by CF. The thick mucus can clog the airways, leading to chronic respiratory infections, inflammation, and lung damage. This results in symptoms such as persistent coughing, frequent lung infections, and difficulty breathing.

Imagine the body's systems as a series of pipes and filters. In cystic fibrosis, the mucus in these systems is too thick, like sludge in the pipes, causing blockages and preventing the proper flow of liquids (enzymes, air, etc.). This can damage the pipes and filters over time, leading to various health issues.

Nurses should provide comprehensive care to address the multifaceted impacts of CF, including:

• Educating the patient and family about the importance of nutrition and enzyme supplementation to aid digestion.
• Monitoring hydration status and educating on the need for increased salt intake during hot weather or vigorous exercise.
• Implementing chest physiotherapy and encouraging regular physical activity to help clear mucus from the lungs.
• Administering medications such as bronchodilators, mucolytics, and antibiotics to manage respiratory symptoms and infections.
• Providing emotional and psychological support to help the patient and family cope with the chronic nature of the disease.

 

✘Incorrect answer options:

The pancreas. While the pancreas is indeed affected by cystic fibrosis, selecting only this option overlooks the comprehensive impact of the disease on other systems, such as the lungs and sweat glands.

The sweat glands. This choice, although correct, is incomplete. It does not account for the significant effects of cystic fibrosis on the lungs and pancreas.

The lungs. The lungs are critically affected by cystic fibrosis, but this option alone is not sufficient to cover the full spectrum of the disease's impact on the body, such as on the pancreas and sweat glands.

 

References

• Ignatavicius, D. D., Workman, M. L., Rebar, C. R., & Heimgartner, N. M. (2018). Medical-Surgical Nursing: Concepts for Interprofessional Collaborative Care. Elsevier.
• Lewis, S. L., Bucher, L., Heitkemper, M. M., & Harding, M. M. (2017). Medical-Surgical Nursing: Assessment and Management of Clinical Problems. Elsevier.

EXPLANATION

 

✔Correct answer:

Digital clubbing of fingers and toes. Digital clubbing is a later finding in cystic fibrosis (CF) that typically develops after prolonged hypoxia and chronic lung disease. It involves the enlargement of the fingertips and toes and is usually seen in older children and adults who have had the disease for a longer period. Since the child in question has been newly diagnosed, Nurse Olivia would not expect to see digital clubbing at this early stage.

Digital clubbing results from chronic low oxygen levels in the blood, which is often a consequence of long-standing respiratory issues in cystic fibrosis patients. The pathophysiology behind digital clubbing is not entirely understood, but it is believed to involve increased blood flow to the distal digits and subsequent changes in connective tissue. Early stages of cystic fibrosis do not typically cause the prolonged hypoxia necessary for clubbing to develop.

Imagine rust forming on a car. If a car has only recently been exposed to wet conditions, it won't have visible rust yet. Similarly, digital clubbing requires prolonged exposure to low oxygen levels and won’t appear in a newly diagnosed CF patient.

Nurses should focus on monitoring and managing early symptoms of cystic fibrosis and educating families about potential future complications.

• Regularly assess the child's respiratory status and manage symptoms to prevent complications.
• Educate the family on the importance of adhering to treatment plans, including airway clearance techniques and medications.
• Monitor the child's growth and nutritional status, providing dietary recommendations and enzyme supplements as needed.
• Keep an eye out for early signs of respiratory infections and treat them promptly to prevent progression.
• Provide emotional and psychological support to the family, helping them understand the disease's progression and management.

 

✘Incorrect answer options:

Chronic cough with mucus production. Chronic cough with mucus production is a common and early symptom of cystic fibrosis. Thick, sticky mucus obstructs the airways, leading to persistent coughing and mucus buildup.

Failure to thrive despite good appetite. Failure to thrive is another early finding in cystic fibrosis due to malabsorption of nutrients, despite a good appetite. This is caused by the blockage of pancreatic ducts and insufficient digestive enzymes.

Recurrent respiratory infections. Recurrent respiratory infections are frequent in children with cystic fibrosis due to the thick mucus that creates an environment conducive to bacterial growth, leading to chronic infections and inflammation.

 

References

• Hockenberry, M. J., & Wilson, D. (2019). Wong's Nursing Care of Infants and Children. Elsevier.
• McKinney, E. S., James, S. R., Murray, S. S., Nelson, K. A., & Ashwill, J. W. (2017). Maternal-Child Nursing. Elsevier.
• Ratjen, F., & Döring, G. (2003). Cystic fibrosis. The Lancet, 361(9358), 681-689.

EXPLANATION

 

✔Correct answer:

Undigested fats. In cystic fibrosis (CF), the thick mucus produced by the body can block the pancreatic ducts, preventing digestive enzymes from reaching the intestines. This leads to malabsorption, particularly of fats, because the enzymes necessary to break down fats (lipases) are not adequately present in the intestines. The undigested fats result in stool that is foul-smelling, frothy, and greasy.

The pancreas normally secretes enzymes like lipase, protease, and amylase into the small intestine to aid in the digestion of fats, proteins, and carbohydrates. In CF, the blockage of these enzymes causes malabsorption of nutrients, especially fats, leading to steatorrhea (fatty stools). These fatty stools are characteristic of CF and are typically described as bulky, greasy, and malodorous.

Imagine trying to wash greasy dishes without dish soap. The grease remains on the dishes, making them slippery and difficult to clean. Similarly, without sufficient pancreatic enzymes, fats remain undigested in the intestines, resulting in greasy stools.

Nurses should monitor stool characteristics in patients with cystic fibrosis to assess the effectiveness of enzyme replacement therapy and overall nutritional status.

• Ensure the patient takes pancreatic enzyme supplements with all meals and snacks to improve fat digestion.
• Monitor the patient’s weight, growth, and nutritional status regularly.
• Educate the family on the importance of enzyme supplementation and adherence to the prescribed regimen.
• Provide dietary recommendations, including high-calorie, high-fat foods to meet the increased nutritional needs of children with CF.
• Evaluate for any signs of malabsorption or nutritional deficiencies and adjust the treatment plan as necessary.

 

✘Incorrect answer options:

Sodium and chloride components. While cystic fibrosis does affect chloride transport and results in high levels of chloride in sweat, this is not what causes the stool to be foul-smelling and frothy. Sodium and chloride do not have this effect on stool characteristics.

Partially digested carbohydrates. Partially digested carbohydrates can cause gas and bloating but do not typically result in foul-smelling, frothy stools. The characteristic stool changes in CF are primarily due to malabsorption of fats.

Digestive enzymes like lipase, trypsin, and amylase. The lack of these digestive enzymes is what leads to malabsorption issues in cystic fibrosis. However, their presence in stool is not what causes the characteristic stool changes. It is the absence of these enzymes in the intestines that leads to undigested fats and the resulting stool appearance.

 

References

• Hockenberry, M. J., & Wilson, D. (2019). Wong's Nursing Care of Infants and Children. Elsevier.
• McKinney, E. S., James, S. R., Murray, S. S., Nelson, K. A., & Ashwill, J. W. (2017). Maternal-Child Nursing. Elsevier.
• Orenstein, D. M., Rosenstein, B. J., & Stern, R. C. (2012). Cystic Fibrosis: A Guide for Patient and Family. Lippincott Williams & Wilkins.

EXPLANATION

 

✔Correct answer:

False. Currently, there is no cure for cystic fibrosis (CF). CF is a genetic disorder caused by mutations in the CFTR gene, which leads to the production of thick and sticky mucus that can clog the airways and ducts in various organs. While significant advances in treatment have improved the quality of life and life expectancy for individuals with CF, the disease remains chronic and incurable.

The defective CFTR protein affects the movement of salt and water in and out of cells, resulting in thickened secretions in the lungs, pancreas, and other organs. These secretions can cause respiratory infections, pancreatic enzyme insufficiency, and other complications. Treatments aim to manage these symptoms and complications, rather than cure the underlying genetic defect.

Imagine a house with faulty plumbing (the CFTR gene mutation). While you can manage the problems by regularly cleaning and maintaining the pipes (treatments), you cannot permanently fix the underlying issue with the plumbing system (genetic mutation) without replacing it entirely, which is currently not possible with CF.

Nurses should provide comprehensive education and support to families dealing with cystic fibrosis, emphasizing the importance of treatment adherence and regular medical follow-ups to manage the disease effectively.

• Educate the family about the chronic nature of cystic fibrosis and the importance of ongoing treatment.
• Discuss available treatments, such as airway clearance techniques, medications to reduce mucus and inflammation, and pancreatic enzyme supplements.
• Provide information on the latest advancements in CF research, including potential new therapies and clinical trials.
• Encourage a healthy lifestyle, including good nutrition and regular exercise, to help manage symptoms and improve overall health.
• Offer emotional and psychological support to the patient and family, addressing their concerns and providing resources for coping with the disease.

 

✘Incorrect answer option:

True. This answer is incorrect because it suggests that a cure for cystic fibrosis is available, which is not the case. While there have been significant advancements in treatment that have greatly improved patient outcomes and life expectancy, a cure for the genetic disorder remains elusive. It is crucial for patients and families to understand that current treatments focus on managing symptoms and improving quality of life rather than curing the disease.

 

References

• Ignatavicius, D. D., Workman, M. L., Rebar, C. R., & Heimgartner, N. M. (2018). Medical-Surgical Nursing: Concepts for Interprofessional Collaborative Care. Elsevier.
• Lewis, S. L., Bucher, L., Heitkemper, M. M., & Harding, M. M. (2017). Medical-Surgical Nursing: Assessment and Management of Clinical Problems. Elsevier.

EXPLANATION

 

✔Correct answer:

Use cupped hands during the percussion. When performing postural drainage for a child with cystic fibrosis, using cupped hands during percussion is essential. This technique helps create an air cushion between the hand and the chest wall, producing a gentle clapping sound that helps loosen mucus in the lungs. This method is more effective and less painful than using a flat hand or the heel of the hand, ensuring the child remains comfortable during the procedure.

Postural drainage involves positioning the child in specific ways to use gravity to help drain mucus from different parts of the lungs. Percussion (clapping) with cupped hands helps dislodge mucus from the bronchial walls, making it easier for the child to cough up and clear the mucus from the airways. This is crucial for preventing infections and maintaining lung function in children with cystic fibrosis.

Think of tapping on the back of a ketchup bottle with your palm slightly curved. The tapping helps dislodge the ketchup so it can flow out easily, just like cupped-hand percussion helps dislodge mucus in the lungs.

Nurses should provide detailed instructions and demonstrations to ensure that caregivers perform postural drainage correctly and effectively.

• Show the caregiver how to position the child for drainage of different lung segments.
• Demonstrate the correct cupping technique for percussion to ensure gentle but effective mucus dislodgement.
• Advise performing postural drainage before meals or at least two hours after meals to avoid discomfort or nausea.
• Encourage a regular schedule for postural drainage, typically 2-3 times per day, to maintain clear airways.
• Provide resources such as instructional videos or pamphlets to reinforce the teaching.

 

✘Incorrect answer options:

Use the heel of her hand during the percussion. Using the heel of the hand can be too forceful and uncomfortable for the child. The cupped hand technique is preferred for its effectiveness and gentleness.

Change the child’s position every twenty minutes. While changing positions is part of postural drainage, it should typically be done every 3-5 minutes, not every twenty minutes. This ensures that all lung segments are adequately drained within a reasonable timeframe.

Perform percussion after meals and at bedtime. Performing percussion after meals can cause discomfort and increase the risk of nausea and vomiting. It is best to perform postural drainage before meals or at least two hours after eating.

 

References

• Hockenberry, M. J., & Wilson, D. (2019). Wong's Nursing Care of Infants and Children. Elsevier.
• McKinney, E. S., James, S. R., Murray, S. S., Nelson, K. A., & Ashwill, J. W. (2017). Maternal-Child Nursing. Elsevier.
• Kliegman, R. M., St. Geme, J. W., Blum, N. J., Shah, S. S., Tasker, R. C., & Wilson, K. M. (2020). Nelson Textbook of Pediatrics. Elsevier.

EXPLANATION

 

✔Correct answer:

Between meals. Chest physiotherapy (CPT) is a critical component in the management of cystic fibrosis (CF) to help clear mucus from the lungs and improve respiratory function. Scheduling these sessions between meals is optimal because it reduces the risk of nausea and vomiting that can occur if the therapy is performed immediately after eating. This timing also helps ensure that the child is comfortable and able to participate fully in the therapy sessions, enhancing their effectiveness.

Chest physiotherapy involves techniques such as percussion, vibration, and postural drainage to mobilize and clear mucus from the airways. If performed after meals, the physical manipulation of the chest and abdomen can lead to discomfort and gastrointestinal upset. By scheduling CPT between meals, the therapy can be done when the child’s stomach is not full, minimizing discomfort and the risk of aspiration.

Think of trying to exercise or perform strenuous activity immediately after a large meal; it can cause stomach discomfort and may lead to nausea. Similarly, performing chest physiotherapy right after eating can be uncomfortable and less effective.

Nurses should coordinate with the respiratory therapy department to ensure that CPT sessions are optimally timed for the child’s comfort and effectiveness.

• Schedule CPT sessions at least 1-2 hours after meals to allow for digestion.
• Ensure the child is hydrated before the session, as this can help thin mucus secretions.
• Monitor the child for any signs of discomfort or distress during CPT and adjust the technique or timing as needed.
• Educate the family on the importance of timing and how to incorporate CPT into their daily routine at home.
• Work with the child to make the sessions as comfortable and enjoyable as possible, perhaps by incorporating games or music.

 

✘Incorrect answer options:

After meals. Performing chest physiotherapy immediately after meals can cause discomfort and increase the risk of nausea and vomiting. This can make the therapy session less effective and more distressing for the child.

After medication. While it is important to coordinate medication timing, scheduling CPT specifically after medication does not address the issue of gastrointestinal discomfort associated with full meals. However, ensuring that medications that aid mucus clearance are taken as prescribed is crucial.

Around the child’s play schedule. Although considering the child’s play schedule is important for overall well-being, it should not take precedence over the physiological needs of the CPT timing. Scheduling CPT between meals provides a balance between therapeutic effectiveness and the child’s comfort.

 

References

• Hockenberry, M. J., & Wilson, D. (2019). Wong's Nursing Care of Infants and Children. Elsevier.
• McKinney, E. S., James, S. R., Murray, S. S., Nelson, K. A., & Ashwill, J. W. (2017). Maternal-Child Nursing. Elsevier.

EXPLANATION

 

✔Correct answer:

With meals and snacks. Pancrelipase (Pancrease capsules) is a pancreatic enzyme replacement therapy used to aid digestion in patients with cystic fibrosis. It should be given with meals and snacks to ensure that the enzymes are present in the gastrointestinal tract when food is ingested. This timing helps facilitate the digestion and absorption of nutrients, particularly fats, proteins, and carbohydrates, which patients with cystic fibrosis often struggle to digest due to pancreatic insufficiency.

In cystic fibrosis, the thick mucus blocks the pancreatic ducts, preventing digestive enzymes from reaching the intestines. This leads to malabsorption and nutritional deficiencies. By taking pancrelipase with meals and snacks, the enzymes can mix with the food and aid in its breakdown, improving nutrient absorption and preventing gastrointestinal symptoms such as steatorrhea (fatty stools).

Think of pancreatic enzymes as detergent for washing dishes. If you add the detergent to the water while the dishes are soaking, it helps break down the grease and food particles. Similarly, taking pancrelipase with meals helps break down the food for better digestion.

Nurses should ensure proper administration of pancrelipase to maximize its effectiveness and provide education to patients and families on its importance.

• Administer pancrelipase with every meal and snack to ensure proper digestion of food.
• If the child has difficulty swallowing capsules, the contents can be mixed with a small amount of acidic food like applesauce, but should not be chewed or mixed with hot foods or beverages.
• Monitor the child's growth and nutritional status regularly to adjust enzyme dosage if necessary.
• Educate the family on recognizing signs of malabsorption, such as greasy stools or poor weight gain, which may indicate the need for dosage adjustment.
• Encourage adherence to the prescribed enzyme regimen to optimize nutritional absorption and overall health.

 

✘Incorrect answer options:

Every three hours while awake. Administering pancrelipase every three hours is not practical and does not align with the timing of meals and snacks, which is essential for the enzyme's effectiveness.

Upon awakening, after meals, and at bedtime. While taking pancrelipase after meals is correct, it should be taken with meals and snacks rather than waiting until after eating. Taking it upon awakening and at bedtime is unnecessary if no food is consumed at those times.

After each bowel movement and following postural drainage. There is no indication for administering pancrelipase after bowel movements or postural drainage. The medication needs to be taken with food to aid in digestion.

 

References

• Hockenberry, M. J., & Wilson, D. (2019). Wong's Nursing Care of Infants and Children. Elsevier.
• McKinney, E. S., James, S. R., Murray, S. S., Nelson, K. A., & Ashwill, J. W. (2017). Maternal-Child Nursing. Elsevier.
• Orenstein, D. M., Rosenstein, B. J., & Stern, R. C. (2012). Cystic Fibrosis: A Guide for Patient and Family. Lippincott Williams & Wilkins.

EXPLANATION

 

✔Correct answer:

Genetic mutation. Cystic fibrosis (CF) is caused by a genetic mutation in the CFTR (cystic fibrosis transmembrane conductance regulator) gene. This gene is responsible for producing a protein that regulates the movement of chloride and sodium ions across cell membranes. Mutations in the CFTR gene lead to the production of a defective CFTR protein, resulting in the thick, sticky mucus that characterizes cystic fibrosis. This abnormal mucus affects various organs, particularly the lungs and digestive system.

The CFTR gene mutation disrupts the normal function of the chloride channels, leading to the production of thick and sticky mucus. In the lungs, this mucus obstructs the airways and creates an environment conducive to chronic infections and inflammation. In the digestive system, it blocks the pancreatic ducts, preventing digestive enzymes from reaching the intestines and causing malabsorption of nutrients.

Think of the CFTR protein as a gatekeeper that controls the flow of water in and out of a cell. When the gatekeeper is faulty (mutated), it can't regulate the water properly, leading to the buildup of thick and sticky substances (mucus) that block normal functions.

Nurses should focus on educating families about the genetic nature of cystic fibrosis, emphasizing the importance of genetic counseling, and providing comprehensive care to manage symptoms and improve quality of life.

• Provide genetic counseling and support for family members, as cystic fibrosis is an inherited disorder.
• Educate the family on the importance of adherence to treatment plans, including respiratory therapies and medications.
• Monitor the child for respiratory infections and provide prompt treatment to manage complications.
• Ensure the child receives adequate nutrition, including pancreatic enzyme supplements and a high-calorie diet.
• Offer resources for support groups and organizations dedicated to cystic fibrosis to help families cope with the challenges of the condition.

 

✘Incorrect answer options:

Bacterial infection. Bacterial infections are common complications of cystic fibrosis due to the thick mucus in the lungs, but they are not the cause of the disease. The underlying cause is a genetic mutation.

Viral infection. Viral infections can exacerbate symptoms and complications in patients with cystic fibrosis, but they are not the cause of the condition. The etiology of CF is rooted in genetic mutations.

Autoimmune response. Cystic fibrosis is not caused by an autoimmune response. Autoimmune diseases occur when the immune system mistakenly attacks the body's own tissues, which is not the mechanism behind CF. CF is caused by inherited genetic mutations in the CFTR gene.

 

References

• Hockenberry, M. J., & Wilson, D. (2019). Wong's Nursing Care of Infants and Children. Elsevier.
• McKinney, E. S., James, S. R., Murray, S. S., Nelson, K. A., & Ashwill, J. W. (2017). Maternal-Child Nursing. Elsevier.

EXPLANATION

 

✔Correct answer:

Throat soreness. Pulmozyme (dornase alfa) is a mucolytic agent used in the management of cystic fibrosis. It works by breaking down the DNA in the mucus, reducing its viscosity and making it easier to clear from the airways. A common side effect of inhalation therapy with Pulmozyme is throat soreness. This occurs because the medication can cause irritation of the mucous membranes in the throat and airways.

Pulmozyme is an enzyme that specifically targets and cleaves extracellular DNA, which is a major component of the thick, sticky mucus in the lungs of patients with cystic fibrosis. By breaking down the DNA, Pulmozyme reduces the viscosity of the mucus, facilitating its clearance through coughing or suctioning. However, the enzyme's action can also irritate the lining of the throat and upper airways, leading to soreness and discomfort.

Think of using a strong detergent to clean a tough stain. While the detergent effectively breaks down the stain, it can also cause your hands to become dry and irritated. Similarly, Pulmozyme breaks down mucus but can irritate the throat.

Nurses should monitor patients receiving Pulmozyme for any side effects and provide appropriate interventions to manage them.

• Assess the patient for signs of throat soreness or irritation regularly.
• Encourage the patient to drink plenty of fluids to help soothe the throat.
• Consider recommending a throat lozenge or mild analgesic to relieve soreness if appropriate.
• Educate the patient and family about the potential side effects and how to manage them.
• Report any severe or persistent side effects to the healthcare provider for further evaluation and management.

 

✘Incorrect answer options:

Increased weight. Pulmozyme does not typically cause weight gain. Weight management in cystic fibrosis is more commonly influenced by nutritional intake, pancreatic enzyme supplementation, and overall health status.

Loss of hair. Hair loss is not a known side effect of Pulmozyme. Hair loss in cystic fibrosis patients is more likely related to nutritional deficiencies or other underlying health conditions rather than the medication itself.

Fragile nails. Fragile nails are not associated with the use of Pulmozyme. Nail health in cystic fibrosis patients can be affected by nutritional deficiencies or chronic illness but is not a side effect of this medication.

 

References

• Hockenberry, M. J., & Wilson, D. (2019). Wong's Nursing Care of Infants and Children. Elsevier.
• McKinney, E. S., James, S. R., Murray, S. S., Nelson, K. A., & Ashwill, J. W. (2017). Maternal-Child Nursing. Elsevier.
• Pulmozyme (dornase alfa) [Prescribing Information]. (2023). Genentech, Inc.

EXPLANATION

 

✔Correct answer:

At least two hours after a meal. Performing chest physiotherapy (CPT) at least two hours after a meal is recommended to minimize the risk of nausea and vomiting. Chest physiotherapy involves techniques like percussion, vibration, and postural drainage to help mobilize and clear mucus from the lungs. Doing this procedure when the stomach is less full reduces discomfort and the likelihood of gastrointestinal upset, which can be especially important for patients with cystic fibrosis who may already have digestive issues.

CPT helps in mobilizing and clearing thick mucus from the lungs, which is crucial in managing cystic fibrosis. The timing of CPT is essential to ensure the patient’s comfort and effectiveness of the therapy. Performing CPT on a full stomach can increase intra-abdominal pressure and lead to regurgitation, nausea, or vomiting, potentially aspirating stomach contents into the lungs and complicating respiratory issues.

Imagine shaking a bottle of soda. If you do it right after filling it to the brim, it’s likely to overflow and cause a mess. Similarly, performing chest physiotherapy right after eating can cause stomach contents to move up, leading to discomfort and risk of aspiration.

Nurses should carefully schedule chest physiotherapy sessions to maximize their effectiveness and minimize patient discomfort.

• Schedule CPT sessions at least two hours after meals to ensure the patient’s stomach is not full.
• Monitor the patient for signs of nausea or discomfort during and after the procedure and adjust the timing if necessary.
• Encourage the patient to stay hydrated, as this can help thin mucus secretions and make them easier to clear.
• Ensure the patient understands the importance of regular CPT and adheres to the prescribed schedule.
• Educate the patient and family on performing CPT at home, emphasizing the best times to do it relative to meals.

 

✘Incorrect answer options:

Right before a meal. Performing CPT right before a meal can be uncomfortable and might interfere with the patient's ability to eat due to increased respiratory effort and potential coughing.

During episodes of bronchospasm. CPT should be avoided during episodes of bronchospasm, as it can exacerbate the condition and cause further respiratory distress. Bronchodilators should be used to relieve bronchospasm before attempting CPT.

Once the secretions have mobilized. The purpose of CPT is to help mobilize and clear secretions. Waiting until the secretions have mobilized defeats the purpose of the therapy. CPT should be done regularly to prevent the accumulation of thick mucus.

 

References

• Hockenberry, M. J., & Wilson, D. (2019). Wong's Nursing Care of Infants and Children. Elsevier.
• McKinney, E. S., James, S. R., Murray, S. S., Nelson, K. A., & Ashwill, J. W. (2017). Maternal-Child Nursing. Elsevier.
• Zorc, J. J., & Hall, M. (2011). "Management of Cystic Fibrosis Exacerbations in the Pediatric Patient". Journal of Pediatric Health Care, Elsevier.

EXPLANATION

 

✔Correct answer:

The patient has a child with cystic fibrosis. Having a child with cystic fibrosis (CF) indicates a genetic risk factor because CF is an inherited genetic disorder caused by mutations in the CFTR gene. If a patient has one child with CF, it suggests that both parents are likely carriers of the mutated CFTR gene. This increases the risk that future children could also inherit the disorder.

Cystic fibrosis is an autosomal recessive disorder, meaning that a child must inherit two copies of the mutated gene (one from each parent) to develop the condition. If both parents are carriers, there is a 25% chance with each pregnancy that the child will have cystic fibrosis, a 50% chance that the child will be a carrier, and a 25% chance that the child will have two normal copies of the gene.

Imagine two parents each carrying a recipe card for a cake. This cake recipe requires two specific faulty ingredients to make a special cake. If both parents each pass down their faulty ingredient, the child ends up with both and makes the special cake, representing cystic fibrosis. However, if only one parent passes down a faulty ingredient, the child carries the recipe but doesn’t make the special cake, indicating they are a carrier without symptoms.

Nurses should provide genetic counseling and education to families with a history of cystic fibrosis to help them understand the risks and implications for future pregnancies.

• Offer genetic counseling to discuss the inheritance pattern of cystic fibrosis and the risks for future children.
• Discuss the availability of genetic testing for carrier status in parents and other family members.
• Provide information on prenatal testing options, such as chorionic villus sampling (CVS) or amniocentesis, to detect CF in the fetus.
• Support the family in making informed decisions regarding family planning and prenatal care.
• Refer to specialized CF care centers for ongoing management and support for the affected child and family.

 

✘Incorrect answer options:

The patient is 25 years old. Age alone is not a genetic risk factor for cystic fibrosis. While maternal age can influence the risk of certain chromosomal abnormalities, it is not directly related to the risk of having a child with a genetic disorder like cystic fibrosis.

The patient was exposed to rubella at 36 weeks' gestation. Exposure to rubella (German measles) during pregnancy can lead to congenital rubella syndrome, which can cause various birth defects, but it is not related to genetic risk factors for cystic fibrosis. This is an environmental risk factor rather than a genetic one.

The patient has a history of preterm labor at 32 weeks' gestation. A history of preterm labor may indicate an increased risk for future preterm deliveries, but it is not a genetic risk factor for cystic fibrosis. Preterm labor can result from various factors, including maternal health conditions, infections, or uterine abnormalities.

 

References

• Hockenberry, M. J., & Wilson, D. (2019). Wong's Nursing Care of Infants and Children. Elsevier.
• McKinney, E. S., James, S. R., Murray, S. S., Nelson, K. A., & Ashwill, J. W. (2017). Maternal-Child Nursing. Elsevier.
• Skirton, H., & Patch, C. (2019). Genetic Counseling: A Practical Guide. Cambridge University Press.

EXPLANATION

 

✔Correct answer:

A 2 1/2-year-old boy with cystic fibrosis. Cystic fibrosis (CF) is a chronic, progressive, and life-threatening genetic disorder that primarily affects the lungs and digestive system. Children with cystic fibrosis are at high risk for developmental problems due to multiple factors associated with the disease. These include frequent hospitalizations, chronic respiratory infections, malabsorption of nutrients, and the extensive daily treatment regimens required to manage their condition.

Cystic fibrosis is caused by mutations in the CFTR gene, leading to defective chloride channels in epithelial cells. This defect results in the production of thick, sticky mucus that obstructs the airways and ducts in various organs. The thick mucus can lead to respiratory infections, decreased lung function, and pancreatic insufficiency, which impacts digestion and nutrient absorption. These issues can significantly affect a child's growth and development.

Imagine trying to water a garden with a hose that is clogged and twisted, making it difficult for water (nutrients and oxygen) to reach the plants (cells). This results in the plants not growing well and becoming more susceptible to pests (infections).

Nurses should provide comprehensive care that includes monitoring the child's growth and development, ensuring proper nutrition, and managing respiratory symptoms. Collaboration with a multidisciplinary team, including pediatricians, nutritionists, and respiratory therapists, is crucial.

• Monitor and manage respiratory infections to prevent complications.
• Ensure adherence to prescribed medications, including pancreatic enzymes and vitamins.
• Encourage high-calorie, nutrient-dense diets to support growth.
• Educate the family about the importance of chest physiotherapy and other airway clearance techniques.
• Provide emotional support to the child and family, addressing concerns about the child's development and quality of life.

 

✘Incorrect answer options:

A toddler with acute glomerulonephritis being treated with antihypertensives and antibiotics. Acute glomerulonephritis, while serious, is typically a transient condition that can be effectively managed with appropriate medical treatment. Although these children may need temporary restrictions and close monitoring, they do not generally face the same long-term developmental risks as those with cystic fibrosis.

A 5-year-old with asthma managed on cromolyn sodium. While asthma can affect a child's quality of life and occasionally lead to missed school days, it is generally a manageable condition with proper treatment. Cromolyn sodium helps to control asthma symptoms and prevent attacks, reducing the overall impact on the child's development compared to chronic and systemic conditions like cystic fibrosis.

A preschooler diagnosed with tonsillitis. Tonsillitis is an acute condition that is typically resolved with appropriate antibiotic therapy or, in recurrent cases, with tonsillectomy. It does not generally pose a significant risk for long-term developmental problems, making this option less concerning compared to chronic conditions like cystic fibrosis.

 

References

• Hockenberry, M. J., & Wilson, D. (2019). Wong's Nursing Care of Infants and Children. Elsevier.
• McKinney, E. S., James, S. R., Murray, S. S., Nelson, K. A., & Ashwill, J. W. (2017). Maternal-Child Nursing. Elsevier.

EXPLANATION

 

✔Correct answer:

All of the choices. The comprehensive approach to managing cystic fibrosis (CF) includes multiple treatment methods that address various aspects of the disease. Nurse Daniels should explain that effective management of CF requires a combination of airway clearance techniques, special vitamins, and pancreatic enzymes, among other interventions.

Cystic fibrosis affects multiple systems in the body, primarily the respiratory and digestive systems. The defective CFTR protein leads to thick and sticky mucus production, which can block airways and ducts, causing respiratory and digestive complications.

Managing cystic fibrosis is like maintaining a complex machine that needs various tools for different parts. Just as you need different tools for different maintenance tasks, managing CF requires multiple treatments to address the various aspects of the disease.

Nurses should provide a detailed and holistic overview of the treatment plan to ensure patients and families understand the importance of each component.

• Airway Clearance Techniques: These techniques help to move the thick mucus from the lungs, improving breathing and reducing the risk of infections. Methods include chest physiotherapy (CPT), positive expiratory pressure (PEP) devices, and high-frequency chest wall oscillation (vest therapy). These techniques are crucial for maintaining lung function and preventing complications.
• Taking Special Vitamins: Due to malabsorption issues, patients with cystic fibrosis often require supplements of fat-soluble vitamins (A, D, E, and K). These vitamins are essential for growth, immune function, and overall health.
• Taking Pancreatic Enzymes: Pancreatic enzyme replacement therapy helps digest food by providing the enzymes that the pancreas cannot secrete due to blockage by thick mucus. This therapy is essential for preventing malnutrition and ensuring adequate nutrient absorption.

 

✘Incorrect answer options:

Choosing any one of the other options alone would not provide a comprehensive approach to managing cystic fibrosis. Each option addresses a different aspect of the disease, and all are necessary for effective management.

Airway clearance techniques that aim to move the thick mucus from the lungs. While crucial, this option alone does not address the digestive and nutritional aspects of CF.

Taking special vitamins. Vitamin supplementation is important but does not address the respiratory and digestive enzyme needs of CF patients.

Taking pancreatic enzymes to help digest food. Enzyme therapy is essential for digestion but does not address respiratory issues or vitamin deficiencies.

 

References

• Hockenberry, M. J., & Wilson, D. (2019). Wong's Nursing Care of Infants and Children. Elsevier.
• McKinney, E. S., James, S. R., Murray, S. S., Nelson, K. A., & Ashwill, J. W. (2017). Maternal-Child Nursing. Elsevier.
• Orenstein, D. M., Rosenstein, B. J., & Stern, R. C. (2012). Cystic Fibrosis: A Guide for Patient and Family. Lippincott Williams & Wilkins.

EXPLANATION

 

✔Correct answer:

Three times a day with meals. Pancreatic enzymes should be administered with meals to ensure they are present in the digestive tract when food is ingested. This timing helps the enzymes mix with the food in the stomach and intestines, facilitating the digestion and absorption of nutrients. In cystic fibrosis, thick mucus blocks the pancreatic ducts, preventing the natural enzymes from reaching the small intestine, which is why supplementation with pancreatic enzymes is necessary.

Pancreatic enzymes include lipases, proteases, and amylases, which help break down fats, proteins, and carbohydrates, respectively. When taken with meals, these enzymes help ensure that food is properly digested and that the patient receives adequate nutrition. Without these enzymes, patients with cystic fibrosis would suffer from malabsorption, leading to poor growth, weight loss, and nutrient deficiencies.

Think of pancreatic enzymes as keys that unlock the nutrients in food. If the keys are present when the food is eaten, the body can access and use the nutrients. If the keys are missing, the nutrients stay locked in the food and pass through the digestive system unused.

Medical-Surgical Nursing Intervention: Nurses should ensure that pancreatic enzymes are administered properly and educate the patient's family on the correct timing and method of administration.

• Administer pancreatic enzymes with every meal and snack to ensure they are effective.
• If the patient has difficulty swallowing capsules, the contents can be mixed with a small amount of acidic food like applesauce, but should not be chewed or mixed with hot foods or beverages.
• Monitor the patient's growth, weight, and nutritional status regularly to adjust enzyme dosage if necessary.
• Educate the family on recognizing signs of malabsorption, such as greasy stools or poor weight gain, which may indicate the need for dosage adjustment.
• Encourage adherence to the prescribed enzyme regimen to optimize nutritional absorption and overall health.

 

✘Incorrect answer options:

Once daily in the morning. Administering pancreatic enzymes only once daily is insufficient because the enzymes need to be present with every meal and snack throughout the day to aid in digestion.

Once daily at bedtime. Administering pancreatic enzymes at bedtime is ineffective because there is no food intake at that time that requires digestion. Enzymes need to be taken with meals and snacks.

Four times daily. While this might seem like it covers multiple meals, it does not specify that the enzymes should be taken specifically with meals and snacks. The most effective approach is to take them with each eating occasion to ensure proper digestion and nutrient absorption.

 

References

• Hockenberry, M. J., & Wilson, D. (2019). Wong's Nursing Care of Infants and Children. Elsevier.
• McKinney, E. S., James, S. R., Murray, S. S., Nelson, K. A., & Ashwill, J. W. (2017). Maternal-Child Nursing. Elsevier.
• Orenstein, D. M., Rosenstein, B. J., & Stern, R. C. (2012). Cystic Fibrosis: A Guide for Patient and Family. Lippincott Williams & Wilkins.

EXPLANATION

 

✔Correct answer:

Sweat test. The sweat test, also known as the sweat chloride test, is the primary diagnostic test used to diagnose cystic fibrosis (CF). This test measures the concentration of chloride in the sweat. People with cystic fibrosis have elevated levels of chloride in their sweat due to a malfunctioning CFTR protein, which affects the movement of chloride ions across cell membranes. The sweat test is considered the gold standard for diagnosing cystic fibrosis.

In cystic fibrosis, mutations in the CFTR gene lead to the production of a defective CFTR protein, which disrupts the normal transport of chloride and sodium across epithelial cells. This disruption causes the accumulation of thick, sticky mucus in various organs, particularly the lungs and digestive system. The sweat glands are also affected, resulting in higher-than-normal concentrations of chloride in the sweat.

Imagine a sponge that leaks water (chloride) excessively when squeezed. In cystic fibrosis, the "sponge" (sweat glands) leaks more chloride into the sweat than normal, which can be measured to diagnose the condition.

Nurses should be aware of the procedures involved in the sweat test and provide appropriate pre-test and post-test care to ensure accurate results and patient comfort.

• Explain the procedure to the patient and family, including the importance of the test in diagnosing cystic fibrosis.
• Ensure the patient is well-hydrated before the test, as dehydration can affect the accuracy of the results.
• Monitor the patient during the test for any signs of discomfort or adverse reactions.
• Provide support and reassurance to the patient and family while awaiting the results.
• Discuss the next steps and potential treatment options if the test results indicate cystic fibrosis.

 

✘Incorrect answer options:

Echocardiogram. An echocardiogram is an ultrasound of the heart used to assess cardiac structure and function. While it is a valuable tool for diagnosing heart conditions, it is not used to diagnose cystic fibrosis.

Complete blood panel. A complete blood panel provides information about the overall health and function of various organ systems but does not specifically diagnose cystic fibrosis. It can help identify secondary complications or infections related to CF.

Chest X-ray. A chest X-ray can show changes in the lungs associated with cystic fibrosis, such as thickened airway walls and mucus plugging, but it cannot definitively diagnose the disease. It is typically used to monitor the progression of lung disease in diagnosed CF patients.

 

References

• Hockenberry, M. J., & Wilson, D. (2019). Wong's Nursing Care of Infants and Children. Elsevier.
• McKinney, E. S., James, S. R., Murray, S. S., Nelson, K. A., & Ashwill, J. W. (2017). Maternal-Child Nursing. Elsevier.
• O'Sullivan, B. P., & Freedman, S. D. (2009). "Cystic fibrosis." The Lancet, 373(9678), 1891-1904.

EXPLANATION

 

✔Correct answer:

Crayons and coloring book. Crayons and a coloring book are an excellent choice for a 3-year-old child with cystic fibrosis who is confined to bed. This type of activity is not only age-appropriate but also helps to keep the child engaged and entertained without posing any health risks. Coloring can provide a calm and creative outlet for the child while they are unable to participate in more physical activities.

Children with cystic fibrosis can be more susceptible to respiratory infections and may have weakened immune systems. Stuffed animals can harbor dust and allergens, which could pose a risk to the child's respiratory health. Building blocks and puzzles with small pieces may present a choking hazard or may be difficult to manage if the child is confined to bed and has limited mobility.

Think of a coloring book and crayons as a safe and enjoyable indoor activity that keeps the child engaged without any physical risk, similar to how reading a book can be both entertaining and safe for someone who needs to stay in bed.

Nurses should select toys that are safe, appropriate for the child’s developmental level, and do not pose any additional health risks.

• Choose toys that can be easily sanitized to prevent infections.
• Ensure the activities are age-appropriate and promote fine motor skills and creativity.
• Avoid toys that have small parts, are difficult to clean, or can collect dust and allergens.
• Monitor the child’s engagement with the toys to ensure they are enjoying and benefiting from the activities.
• Provide a variety of activities to keep the child stimulated and entertained during their confinement.

 

✘Incorrect answer options:

Stuffed animal. While a stuffed animal might be comforting, it can collect dust and allergens, potentially exacerbating respiratory issues in a child with cystic fibrosis.

Building blocks. Building blocks are generally safe and beneficial for motor skills development, but they might be less suitable for a child confined to bed because of potential choking hazards and the need for physical movement.

Puzzle with small pieces. Puzzles with small pieces can be a choking hazard for young children and may be difficult for a bedridden child to manage, making them a less ideal choice for this scenario.

 

References

• Hockenberry, M. J., & Wilson, D. (2019). Wong's Nursing Care of Infants and Children. Elsevier.
• McKinney, E. S., James, S. R., Murray, S. S., Nelson, K. A., & Ashwill, J. W. (2017). Maternal-Child Nursing. Elsevier.
• Jarvis, C. (2019). Physical Examination and Health Assessment. Elsevier.

EXPLANATION

 

✔Correct answer:

"My child dislikes taking pills, so I'll mix the capsule into a cup of hot chocolate." Mixing enzyme capsules into hot chocolate indicates a misunderstanding of how to administer pancreatic enzymes. Pancreatic enzyme supplements are essential for children with cystic fibrosis to aid in the digestion and absorption of nutrients. These enzymes are sensitive to heat, which can destroy their effectiveness. Therefore, mixing them into hot beverages can render them ineffective.

Pancreatic enzymes help break down fats, proteins, and carbohydrates in the small intestine. For children with cystic fibrosis, whose pancreatic ducts are often blocked by thick mucus, these enzymes must be taken with meals and snacks to facilitate proper digestion. Enzyme capsules should be taken with a cold or room temperature food or liquid to preserve their activity.

Imagine you have special soap that dissolves grease. If you mix it into boiling water, the soap loses its ability to clean. Similarly, enzymes lose their ability to break down food when mixed with hot liquids.

Nurses should provide clear instructions on the proper administration of pancreatic enzymes, emphasizing the importance of preserving enzyme activity.

• Enzyme capsules should be taken with cold or room temperature food or drink.
• If the child cannot swallow capsules, the contents can be sprinkled on a small amount of soft, acidic food like applesauce, which should be consumed immediately.
• Avoid mixing enzyme contents with hot foods or beverages.
• Monitor the child's growth and nutritional status to ensure the enzyme dosage is effective.
• Reinforce the importance of taking enzymes before every meal and snack.

 

✘Incorrect answer options:

"If the meal has a lot of fat, my child might need an extra enzyme capsule." This statement is correct. Meals high in fat require additional enzymes for proper digestion, so administering an extra enzyme capsule in such cases is appropriate.

"I'll give an enzyme capsule before every snack." This statement is correct. Pancreatic enzymes should be taken before meals and snacks to ensure proper digestion and nutrient absorption.

"I'll give an enzyme capsule before every meal." This statement is correct. Enzyme capsules should be administered before meals to facilitate the digestion of food.

 

References

• Hockenberry, M. J., & Wilson, D. (2019). Wong's Nursing Care of Infants and Children. Elsevier.
• McKinney, E. S., James, S. R., Murray, S. S., Nelson, K. A., & Ashwill, J. W. (2017). Maternal-Child Nursing. Elsevier.

EXPLANATION

 

✔Correct answer:

1 in 2,500. Cystic fibrosis (CF) is a genetic disorder that is most commonly found in individuals of Northern European descent. The prevalence of cystic fibrosis is approximately 1 in 2,500 live births in this population. This prevalence ratio indicates that cystic fibrosis is relatively common among genetic disorders, necessitating widespread awareness and early diagnosis for effective management.

Cystic fibrosis is caused by mutations in the CFTR gene, which is responsible for the production of a protein that regulates the movement of salt and water in and out of cells. When this gene is mutated, it leads to the production of thick and sticky mucus that can clog the airways and ducts in various organs, causing severe respiratory and digestive problems.

Think of cystic fibrosis as a rare but significant flaw in a car model, where every 2,500 cars produced have this defect, leading to major performance issues. Early detection and specialized care are crucial to managing the defect and maintaining the car's functionality.

Nurses play a critical role in educating families and communities about cystic fibrosis, emphasizing the importance of genetic screening, early diagnosis, and ongoing management.

• Promote newborn screening programs to identify CF early.
• Provide resources and support for families affected by CF, including information on treatment options and lifestyle modifications.
• Collaborate with multidisciplinary teams to ensure comprehensive care for CF patients, addressing respiratory, digestive, and psychosocial needs.
• Educate the community about the genetic nature of CF and the importance of genetic counseling for at-risk couples.

 

✘Incorrect answer options:

1 in 7,500. While CF is a significant genetic disorder, this ratio underestimates the prevalence of the condition, particularly in populations of Northern European descent where the incidence is higher.

1 in 25,000. This ratio significantly underestimates the prevalence of cystic fibrosis and does not reflect the actual frequency observed in certain populations.

1 in 50,000. This ratio greatly underestimates the prevalence of cystic fibrosis, making it far less common than it actually is, and could lead to misconceptions about the frequency and importance of screening for this disorder.

 

References

• Hockenberry, M. J., & Wilson, D. (2019). Wong's Nursing Care of Infants and Children. Elsevier.
• McKinney, E. S., James, S. R., Murray, S. S., Nelson, K. A., & Ashwill, J. W. (2017). Maternal-Child Nursing. Elsevier.

EXPLANATION

 

✔Correct answer:

Performing chest physiotherapy. Chest physiotherapy (CPT) is one of the most crucial pulmonary treatments for patients with cystic fibrosis (CF). CPT helps clear mucus from the lungs, improving breathing and reducing the risk of respiratory infections. This therapy involves techniques such as percussion (clapping on the chest), vibration, and postural drainage to loosen and mobilize the thick, sticky mucus characteristic of CF, allowing it to be coughed up or suctioned out more easily.

In cystic fibrosis, the defective CFTR protein causes mucus to become thick and sticky, which can block airways and lead to chronic infections and inflammation. Regular chest physiotherapy helps maintain lung function by preventing mucus buildup and facilitating its clearance. This is vital because accumulated mucus provides a breeding ground for bacteria, increasing the risk of recurrent respiratory infections and lung damage.

Think of chest physiotherapy as shaking a tree to dislodge all the leaves. Just as shaking helps the leaves fall to the ground, CPT helps dislodge mucus so it can be cleared from the lungs.

Nurses should incorporate chest physiotherapy into the daily care routine for patients with cystic fibrosis and educate patients and their families on its importance and proper techniques.

• Perform CPT multiple times a day, especially before meals and at bedtime, to maximize mucus clearance.
• Educate the patient and family members on how to perform CPT at home, ensuring they understand the techniques and schedule.
• Use CPT in combination with other treatments like inhaled medications to enhance mucus clearance and improve lung function.
• Monitor the patient for signs of respiratory distress or infections and adjust the therapy regimen as needed.
• Encourage adherence to CPT as a crucial part of the overall treatment plan for cystic fibrosis.

 

✘Incorrect answer options:

Inhaled beta agonist medications. While inhaled beta agonists, such as albuterol, can help open the airways and make breathing easier, they are not the primary treatment for managing the thick mucus characteristic of cystic fibrosis.

Corticosteroids taken by inhalation. Inhaled corticosteroids can reduce inflammation in the airways but are not as effective as chest physiotherapy in clearing mucus from the lungs in cystic fibrosis patients.

Taking oral enzyme supplements. Oral enzyme supplements are essential for managing the digestive complications of cystic fibrosis by aiding in the digestion and absorption of nutrients. However, they do not directly address the pulmonary issues associated with the disease.

 

References

• Hockenberry, M. J., & Wilson, D. (2019). Wong's Nursing Care of Infants and Children. Elsevier.
• McKinney, E. S., James, S. R., Murray, S. S., Nelson, K. A., & Ashwill, J. W. (2017). Maternal-Child Nursing. Elsevier.
• O'Sullivan, B. P., & Freedman, S. D. (2009). "Cystic fibrosis." The Lancet, 373(9678), 1891-1904.

EXPLANATION

 

✔Correct answer:

False. Cystic fibrosis (CF) is a genetic disorder inherited in an autosomal recessive pattern. This means that in order for a child to develop cystic fibrosis, they must inherit two copies of the defective CFTR gene—one from each parent. If only one parent has cystic fibrosis (and thus two copies of the defective gene), the parent can only pass one defective gene to their child. The other parent would need to carry at least one defective CFTR gene for the child to potentially have the disease. Therefore, if the other parent does not carry a defective CFTR gene, the child cannot have cystic fibrosis.

The CFTR gene provides instructions for making a protein that regulates the movement of salt and water in and out of cells. Mutations in the CFTR gene disrupt the function of this protein, leading to the production of thick and sticky mucus that can clog airways and ducts in the lungs, pancreas, and other organs. In an autosomal recessive inheritance pattern, a child must inherit two defective genes (one from each parent) to manifest the disease.

Imagine each parent has a pair of keys (genes) for a door. For the door (cystic fibrosis) to open, the child needs two faulty keys (defective genes). If only one parent has a faulty key, and the other parent has two good keys, the door will not open, and the child will not have the disease.

Nurses should provide genetic counseling to families to help them understand the inheritance pattern of cystic fibrosis and the implications for their children.

• Educate the family about the autosomal recessive inheritance pattern of cystic fibrosis.
• Discuss the importance of genetic testing for both parents to determine their carrier status.
• Provide support and resources for families with a history of cystic fibrosis.
• Explain the potential for genetic counseling and prenatal testing options if desired.

 

✘Incorrect answer option:

True. This answer is incorrect because it suggests that if one parent has cystic fibrosis, the child automatically has a chance of getting the disease, which is not accurate given the autosomal recessive inheritance pattern. The child must inherit two defective genes (one from each parent) to have cystic fibrosis. If the other parent is not a carrier of the defective gene, the child will not develop the disease but will be a carrier if they inherit the defective gene from the affected parent.

 

References

• Ignatavicius, D. D., Workman, M. L., Rebar, C. R., & Heimgartner, N. M. (2018). Medical-Surgical Nursing: Concepts for Interprofessional Collaborative Care. Elsevier.
• Lewis, S. L., Bucher, L., Heitkemper, M. M., & Harding, M. M. (2017). Medical-Surgical Nursing: Assessment and Management of Clinical Problems. Elsevier.

EXPLANATION

 

✔Correct answer:

Nasal polyps. Children with cystic fibrosis (CF) often develop nasal polyps, which are non-cancerous growths in the nasal passages or sinuses. These polyps can obstruct the airways, leading to difficulty in breathing and causing sounds such as snoring or noisy breathing. The thick and sticky mucus characteristic of CF creates an environment prone to chronic inflammation and infection, which can lead to the formation of these polyps.

Nasal polyps develop due to chronic inflammation of the nasal and sinus mucosa. In cystic fibrosis, the mucus is abnormally thick and sticky, contributing to persistent sinus infections and inflammation. Over time, this inflammation can cause the mucous membrane to swell and form polyps, which can obstruct airflow through the nasal passages.

Imagine a narrow garden hose partially blocked by pebbles (nasal polyps). Water (air) has to flow around these obstructions, causing turbulence and noise, similar to how nasal polyps can cause snoring sounds in breathing.

Nurses should be aware of the common complications associated with cystic fibrosis and provide appropriate care and education to manage these conditions.

• Monitor for signs of nasal obstruction, such as snoring, mouth breathing, or recurrent sinus infections.
• Educate the family on the importance of regular ENT (ear, nose, and throat) evaluations for children with CF.
• Discuss potential treatments for nasal polyps, including nasal corticosteroids, saline irrigations, or surgical removal if necessary.
• Ensure adherence to CF treatment regimens, including airway clearance techniques and medications to reduce mucus viscosity and control infections.
• Provide resources and support for managing chronic symptoms and improving the child's quality of life.

 

✘Incorrect answer options:

Choanal atresia. Choanal atresia is a congenital condition where the back of the nasal passage is blocked, typically by abnormal bony or soft tissue. While this can cause breathing difficulties, it is not specifically associated with cystic fibrosis and is a condition present at birth.

Septal deviations. Septal deviations refer to a displacement of the nasal septum. While this can cause breathing difficulties and snoring, it is not a common condition specifically linked to cystic fibrosis. It typically results from trauma or congenital factors, not the chronic inflammation and mucus issues seen in CF.

Enlarged adenoids. Enlarged adenoids can cause nasal obstruction and snoring, particularly in young children. However, they are not specifically related to cystic fibrosis. Enlarged adenoids are more commonly a result of recurrent infections or allergies rather than the chronic inflammatory processes seen in CF.

 

References

• Hockenberry, M. J., & Wilson, D. (2019). Wong's Nursing Care of Infants and Children. Elsevier.
• McKinney, E. S., James, S. R., Murray, S. S., Nelson, K. A., & Ashwill, J. W. (2017). Maternal-Child Nursing. Elsevier.

EXPLANATION

 

✔Correct answer:

Early years [0-5]. Cystic fibrosis (CF) is typically diagnosed in the early years of life, often before the age of 5. Advances in newborn screening programs have made it possible to diagnose CF very early, sometimes even within the first few weeks of life. Early diagnosis is crucial as it allows for the initiation of treatment and management strategies that can significantly improve the quality of life and prognosis for affected individuals.

Cystic fibrosis is a genetic disorder caused by mutations in the CFTR gene. These mutations result in the production of thick and sticky mucus that can cause blockages in various organs, particularly the lungs and digestive system. Early detection through newborn screening, which includes a blood test to check for elevated levels of immunoreactive trypsinogen (IRT), followed by a sweat chloride test if necessary, helps identify affected infants quickly. This early diagnosis allows for prompt intervention to manage symptoms and prevent complications.

Imagine finding a small leak in a dam early on. If you detect and repair it immediately, you can prevent a major flood later. Similarly, early detection of CF allows for timely treatment, preventing severe complications and improving health outcomes.

Nurses should be knowledgeable about the importance of early detection and the protocols involved in newborn screening for CF.

• Educate parents about the significance of newborn screening and the potential outcomes if CF is detected.
• Ensure timely follow-up testing, such as the sweat chloride test, for infants who test positive on the initial screening.
• Provide information and support to families following a diagnosis, including resources on CF management and care.
• Coordinate with multidisciplinary teams to implement early intervention strategies to manage symptoms and improve quality of life.
• Monitor the growth and development of diagnosed children to ensure they receive appropriate nutritional and respiratory care.

 

✘Incorrect answer options:

Childhood years [5-12] While some cases of CF might be diagnosed in this age range, most cases are identified much earlier, often through newborn screening programs. Diagnosing CF later can mean missed opportunities for early intervention and management.

Teenage years [12-17] Diagnosing CF in the teenage years is uncommon and often indicates a milder form of the disease or a missed diagnosis in earlier years. Most CF cases are identified well before this age through standard screening practices.

Adult years [18-50] It is very rare for cystic fibrosis to be diagnosed in adulthood, though it can happen in cases of very mild disease or atypical presentations. The vast majority of CF diagnoses occur in infancy or early childhood due to the effectiveness of newborn screening programs.

 

References

• Hockenberry, M. J., & Wilson, D. (2019). Wong's Nursing Care of Infants and Children. Elsevier.
• McKinney, E. S., James, S. R., Murray, S. S., Nelson, K. A., & Ashwill, J. W. (2017). Maternal-Child Nursing. Elsevier.

EXPLANATION

 

✔Correct answer:

Mucoviscidosis. Another name for cystic fibrosis is mucoviscidosis. This term highlights the hallmark characteristic of the disease, which is the production of abnormally thick and sticky mucus. The term "mucoviscidosis" is derived from "muco," referring to mucus, and "viscous," indicating the thick, sticky nature of the mucus. This mucus affects various organs, particularly the lungs and digestive system, leading to the typical symptoms and complications associated with cystic fibrosis.

Cystic fibrosis is caused by mutations in the CFTR gene, leading to defective chloride ion transport across epithelial cells. This defect results in the production of thick, sticky mucus that can obstruct the airways and ducts in organs such as the lungs and pancreas. The term "mucoviscidosis" effectively captures the essence of this pathophysiological process.

Think of mucoviscidosis as trying to breathe through a straw filled with honey. The honey represents the thick mucus, making it difficult to breathe and causing blockages, similar to how thick mucus obstructs the airways in cystic fibrosis.

Nurses should provide comprehensive education to families about cystic fibrosis, including its alternative names and the underlying mechanisms of the disease.

• Explain the genetic basis of cystic fibrosis and how it leads to the production of thick, sticky mucus.
• Discuss the various systems affected by the disease, including the respiratory and digestive systems.
• Educate the family on the importance of adherence to treatment regimens, including airway clearance techniques and enzyme supplementation.
• Provide resources and support for managing the chronic nature of the disease and improving quality of life.
• Offer information on support groups and organizations for families affected by cystic fibrosis.

 

✘Incorrect answer options:

Bronchiectasis. Bronchiectasis is a condition that involves the permanent enlargement and damage of the airways in the lungs, often due to chronic infections or inflammation. While bronchiectasis can occur in patients with cystic fibrosis, it is not another name for the disease itself.

Sticky mucus disease. While this term descriptively refers to the main characteristic of cystic fibrosis, it is not an official or commonly used alternative name for the condition.

Irritating cough disease. This term is also descriptive of a symptom commonly experienced by cystic fibrosis patients but is not an alternative name for the disease.

 

References

• Hockenberry, M. J., & Wilson, D. (2019). Wong's Nursing Care of Infants and Children. Elsevier.
• McKinney, E. S., James, S. R., Murray, S. S., Nelson, K. A., & Ashwill, J. W. (2017). Maternal-Child Nursing. Elsevier.
• O'Sullivan, B. P., & Freedman, S. D. (2009). "Cystic fibrosis." The Lancet, 373(9678), 1891-1904.

EXPLANATION

 

✔Correct answer:

Alongside meals and snacks. Creon is a pancreatic enzyme replacement therapy (PERT) used in cystic fibrosis (CF) patients to aid in the digestion and absorption of nutrients. It contains a mixture of digestive enzymes, including lipases, proteases, and amylases, which help break down fats, proteins, and carbohydrates in the food. For maximum effectiveness, Creon must be taken with every meal and snack to ensure that the enzymes are present in the small intestine when the food arrives, enabling proper digestion.

In cystic fibrosis, thick mucus blocks the pancreatic ducts, preventing digestive enzymes from reaching the small intestine. This leads to malabsorption and nutritional deficiencies. Creon provides these necessary enzymes to facilitate digestion. Timing is crucial because the enzymes need to be in the intestine at the same time as the food to work effectively.

Imagine you are baking a cake and you need the oven preheated before you put the cake in. If the oven (enzymes) is not ready when the cake (food) goes in, the cake will not bake properly. Similarly, enzymes need to be present in the intestine when food arrives to ensure proper digestion.

Nurses should educate patients and families about the correct timing for taking Creon to maximize its effectiveness.

• Administer Creon with every meal and snack, ensuring the child takes the enzymes at the beginning of eating.
• If the child has difficulty swallowing capsules, the contents can be mixed with a small amount of acidic food like applesauce, but should not be chewed or mixed with hot foods or beverages.
• Monitor the child's growth and nutritional status regularly to adjust enzyme dosage if necessary.
• Educate the family on the signs of malabsorption, such as greasy stools, abdominal pain, or poor weight gain, and the importance of enzyme adherence.
• Encourage a balanced diet with sufficient calories to meet the increased nutritional needs of children with CF.

 

✘Incorrect answer options:

Before bedtime. Taking Creon before bedtime is not appropriate because it needs to be taken with food to be effective. At bedtime, there is usually no meal or snack to digest, rendering the enzymes useless.

Two times per day. Administering Creon only twice a day is insufficient because the child eats multiple times a day. Enzymes must be present with every meal and snack to facilitate proper digestion and nutrient absorption.

Once every morning. Giving Creon only once in the morning is inadequate. The enzymes need to be taken with every meal and snack throughout the day to ensure that all ingested food is properly digested and absorbed.

 

References

• Hockenberry, M. J., & Wilson, D. (2019). Wong's Nursing Care of Infants and Children. Elsevier.
• McKinney, E. S., James, S. R., Murray, S. S., Nelson, K. A., & Ashwill, J. W. (2017). Maternal-Child Nursing. Elsevier.
• Creon [Prescribing Information]. (2023). AbbVie Inc.

EXPLANATION

 

✔Correct answer:

A high-calorie and high-fat diet. Children with cystic fibrosis (CF) have increased energy needs due to their condition, which affects both respiratory and digestive systems. They often have difficulty absorbing nutrients because of pancreatic insufficiency, which leads to malabsorption of fats and fat-soluble vitamins. To support their growth and overall health, a diet that is both high in calories and high in fat is recommended.

Cystic fibrosis affects the CFTR gene, which is crucial for the proper function of chloride channels in various tissues. This malfunction leads to the production of thick and sticky mucus that blocks ducts in organs such as the pancreas and lungs. The blockage in the pancreas prevents digestive enzymes from reaching the intestines, leading to malabsorption of nutrients, particularly fats. Therefore, a high-calorie, high-fat diet helps compensate for these malabsorption issues and meets the increased energy demands of children with CF.

Think of the body as a factory that needs a certain amount of raw materials (calories and nutrients) to produce goods (energy and growth). In cystic fibrosis, the factory has a lot of broken machinery (malabsorption issues) and therefore needs more raw materials to produce the same amount of goods.

Nurses should provide guidance on maintaining an appropriate diet, including:

• Encouraging frequent meals and snacks that are high in calories and fat.
• Ensuring that Mark takes his pancreatic enzyme supplements with meals to aid in the digestion and absorption of nutrients.
• Incorporating a variety of nutrient-dense foods, such as avocados, nuts, cheese, and whole milk.
• Monitoring weight and growth regularly to adjust dietary recommendations as needed.
• Educating the family on the importance of fat-soluble vitamins (A, D, E, K) and possibly recommending supplementation under medical supervision.

 

✘Incorrect answer options:

A high-fat diet. While a high-fat diet is beneficial, it alone does not address the increased calorie needs of children with cystic fibrosis. They need both high-fat and high-calorie intake to meet their energy demands and support proper growth and development.

A low-fat diet. This would be inappropriate for a child with cystic fibrosis because it does not provide the necessary fats that are crucial for calorie intake and nutrient absorption. A low-fat diet would exacerbate the problem of malabsorption and potentially lead to malnutrition and poor growth.

A high-calorie diet. Although a high-calorie diet is essential, it must also be high in fat to ensure that the child gets enough concentrated sources of energy and can compensate for the malabsorption issues specific to fats. Simply increasing calories without considering fat intake would not be as effective in meeting the child’s nutritional needs.

 

References

• Hockenberry, M. J., & Wilson, D. (2019). Wong's Nursing Care of Infants and Children. Elsevier.
• McKinney, E. S., James, S. R., Murray, S. S., Nelson, K. A., & Ashwill, J. W. (2017). Maternal-Child Nursing. Elsevier.