Asthma is a chronic respiratory condition that affects the airways in the lungs. In acute asthma exacerbation, airway obstruction and inflammation impair ventilation, leading to hypoxemia and, if severe, hypercapnia.

Rationale for correct answer:
2.ABGis the most important test to assess severity of asthma exacerbation. Early stages of the disease result in ↓ PaO₂, and ↓ PaCO₂ from hyperventilation. Late/severe stages cause ↓ PaO₂, ↑ PaCO₂ from respiratory failure. This provides guidance for oxygen therapy and need for escalation such as intubation.

Rationale for incorrect answers:

1. Complete blood count (CBC)may detect infection or eosinophilia, but it does not evaluate acute respiratory status.

3. Blood urea nitrogen (BUN)reflects kidney function, unrelated to asthma exacerbation.

4. Partial thromboplastin time (PTT)measures clotting time, not respiratory function.

Take home points

• ABGs are crucial in acute asthma to monitor oxygenation and CO₂ retention.
• Pulse oximetry is a quick bedside tool, but ABG confirms severity.
• Rising PaCO₂ in a child with asthma is a red flag for impending respiratory failure.

Status asthmaticusis a severe, prolonged asthma attack unresponsive to standard treatment such as inhaled β₂-agonists. It is a medical emergency requiring rapid intervention to prevent respiratory failure.

Rationale for correct answer:
4.Knowing when the child’s last dose of medication was administeredprovides essential, time-sensitive information to guide immediate treatment decisions. It helps avoid duplicating doses too soon and identifies whether medications were ineffective or if the attack worsened despite therapy.

Rationale for incorrect answers:

1. Knowing the last time the child ate is relevant if intubation or sedation might be required (aspiration risk), but not the most urgent information in status asthmaticus.

2. Knowing whether the child has been exposed to any of the usual asthma triggers is useful for long-term prevention and management, but not immediately life-saving during the acute crisis.

3. Asking when the child was last admitted to the hospital for asthma provides background history of severity but does not influence urgent management in the current emergency.

Take home points

• In status asthmaticus, always prioritize immediate treatment-related information (last dose of bronchodilators, steroids, or other meds).
• Exposure history and hospitalization history are important for long-term care, but life-saving interventions come first.

Nurses must gather information that directly impacts safe and effective acute management.

Albuterol is a short-acting beta-agonist (SABA)used as a rescue medication during asthma attacks or before exercise to relax airway smooth muscle and relieve bronchospasm. Proper inhaler technique ensures the drug reaches the lower airways effectively.

Rationale for correct answer:

1. “I should administer two quick puffs of the albuterol inhaler using a spacer.”This is an incorrect technique. Parents should wait at least 1 minute between puffsto allow the first dose to dilate the airways, which helps the second puff penetrate deeper into the lungs. Administering “two quick puffs” without waiting decreases medication effectiveness.

Rationale for incorrect answers:
2. “I should always use a spacer when administering the albuterol inhaler.”Spacers help children coordinate inhalation and maximize delivery of medication to the lungs, reducing deposition in the mouth/throat.

3. “I should be sure that my child is in an upright position when administering the inhaler.”Upright positioning optimizes lung expansion and medication delivery to the lower airways.

4. “I should always shake the inhaler before administering a dose.”Shaking ensures the medication is properly mixed for accurate dosing.

Take home points

• Parents should wait at least 1 minute between puffs of albuterol.
• Using a spacer, upright positioning, and shaking the inhaler are all correct practices.
• Nurses must emphasize step-by-step technique to improve asthma control and avoid ineffective medication use.

Asthma is a chronic inflammatory airway diseasecharacterized by bronchoconstriction, airway inflammation, and increased mucus production. During acute symptoms such as wheezing, coughing, or shortness of breath, the immediate priority is to relieve bronchospasmand improve airflow.

Rationale for correct answer:
3.Albuterolis ashort-acting bronchodilatorthat works within minutes to provide rapid relief of acute bronchospasm, wheezing, and dyspnea. It is the first-line treatment for an asthma attack.

Rationale for incorrect answers:

1. Prednisoneis a corticosteroid used for longer-term control of inflammation in moderate to severe exacerbations but does not provide immediate relief. It can take several hours to days to be effective.

2. Montelukast (Singulair)is a leukotriene receptor antagonist used for long-term asthma control and prevention, not for acute symptom relief.

4. Fluticasone (Flovent)is an inhaled corticosteroid for daily maintenance therapy to reduce airway inflammation. It does not work quickly enough to treat acute attacks.

Take home points

• Albuterol (SABA) is the go-to rescue medication for acute asthma symptoms.
• Corticosteroids (oral or inhaled) are for long-term inflammation control, not acute relief.
• Montelukast is preventive, not a rescue drug.
• Nurses should teach families the difference between rescue medications used for immediate relief and controller medications used for long-term management.

An asthma exacerbation, also called an asthma attackor flare-up, is a sudden worsening of asthma symptoms due to increased inflammation and narrowing of the airways.

Rationale for correct answer:
3.A 9-year-old who is quiet, pale, wheezing bilaterally with SpO₂ 92%: This child is the most critical. Quietness, hypoxemia, and pallorindicate severe obstruction with reduced air entry, suggesting impending respiratory failure. This requires immediate priority intervention such as oxygen, bronchodilator therapy, and possible escalation to advanced airway support.

Rationale for incorrect answers:

1. A 12-month-old, mild cry, pale, diminished breath sounds, SpO₂ 93%: This is concerning but not the most urgent. Diminished breath sounds are worrisome, but SpO₂ is still slightly higher (93%), and the child still has some cry showing air movement.

2. A 5-year-old, complete sentences, pink, wheezing bilaterally, SpO₂ 93%: This child is stable compared to the others. The ability to speak in complete sentencesand normal color indicate adequate air exchange. Wheezing is expected but not immediately life-threatening.

4. A 16-year-old, short sentences, wheezing, sitting upright, SpO₂ 93%: These symptoms indicate moderate distress, but the child is compensating by sitting upright and still moving air with SpO₂ 93%. Not as critical as the quiet 9-year-old with worsening hypoxemia.

Take home points

• Oxygen saturation ≤ 92% signals significant hypoxemia and the need for urgent treatment.
• Prioritization in asthma focuses on recognizing signs of impending respiratory failure: quiet chest, altered mental status, severe retractions, and hypoxemia.
• Always treat the sickest first, not the noisiest. Wheezing children are often moving more air than those who are quiet.

Asthma is a chronic inflammatory airway disorder with air trappingcaused by bronchoconstriction, mucus production, and swelling. Young children often have difficulty with structured breathing techniques such as incentive spirometry. Instead, play-based breathing exercises which helps to mobilize trapped air and improve ventilation.

Rationale for correct answer:
3.Blow a pinwheelis a playful, age-appropriate exercise that helps the child lengthen the expiratory phase, promoting better airway clearance and reducing air trapping.

Rationale for incorrect answers:

1. Using an incentive spirometerrequires a level of cooperation and understanding not expected in a 3-year-old. It is more appropriate for older children and adults.

2. Breathing into a paper bagis not used in asthma; it is sometimes used for hyperventilation due to anxiety. In asthma, this could worsen hypoxemia by limiting oxygen intake.

4. Taking several deep breathswithout a focus on prolonged exhalation does not specifically target the problem of air trapping in asthma.

Take home points

• Age-appropriate, play-based breathing exercises (e.g., blowing bubbles, blowing a pinwheel) are best for young children with asthma.
• The goal is to prolong exhalation and help mobilize trapped air.
• Structured devices like incentive spirometry are best reserved for older, cooperative children.
• Never use techniques that limit oxygen intake (like paper bag breathing) in asthma.

Asthma is a chronic inflammatory airway disorder with air trappingcaused by bronchoconstriction, mucus production, and swelling. Asthma can be triggered by environmental allergens such as dust mites, pet dander, mold, and pollen. Preventive management focuses on allergen avoidance in the home environment.

Rationale for correct answer:
4.“Avoid purchasing upholstered furniture.”Upholstered furniture collects dust and allergens, which can worsen asthma symptoms. Choosing non-upholstered, easy-to-clean furniture helps maintain a more allergy-free environment.

Rationale for incorrect answers:

1. “Use a humidifier in your child’s room.”Humidifiers can actually promote mold growth and dust mites, which are common asthma triggers. A dehumidifier may be more appropriate in damp environments.

2. “Have your carpet cleaned chemically once a month.”Carpet cleaning chemicals may irritate airways. Instead, minimizing carpet use and vacuuming with a HEPA filter vacuum is better for asthma management.

3. “Wash household pets weekly.”While washing pets may temporarily reduce dander, it does not eliminate exposure. The best approach is to keep pets out of the child’s bedroom and limit close contact if pet dander is a known trigger.

Take home points

• Best environmental control for asthma: limit dust-collecting items (upholstered furniture, heavy drapes, stuffed animals).
• Humidifiers and strong chemicals should be avoided as they can worsen symptoms.
• HEPA filters, regular cleaning, and minimizing carpet use are better strategies.
• Nurses should educate parents that asthma control involves both medication adherence and environmental allergen reduction.

Status asthmaticusis a severe, life-threatening asthma exacerbation that does not respond to standard bronchodilator therapy. Unlike typical asthma attacks, it is continuous, progressive, and can lead to respiratory failure if not treated promptly in an emergency setting.

Rationale for correct answer:
4.Status asthmaticus is characterized by persistent bronchospasm and airway obstructiondespite treatment with short-acting bronchodilators, requiring emergency interventions such as IV medications, oxygen therapy, or mechanical ventilation.

Rationale for incorrect answers:

1. Several attacks per monthindicate moderate persistent asthma, not status asthmaticus.

2. Less than six attacks per yearindicate mild intermittent asthma, not status asthmaticus.

3. While partial nonresponse may occur in severe asthma, status asthmaticus is defined by complete or nearly complete lack of responseand constant symptoms, making option 4 more precise.

Take home points

• Status asthmaticus is a medical emergency requiring immediate intervention.
• Key distinguishing feature: persistent symptoms unrelieved by usual therapy.
• Early recognition and treatment prevent respiratory failure and hypoxia.
• Nurses should monitor oxygen saturation, respiratory effort, and response to therapy continuously.

Cystic fibrosis (CF) is a genetic disorderthat primarily affects the lungs and digestive system, but can impact other organs as well. CF is caused by mutations in the CFTR gene, which regulates the movement of salt and water in and out of cells. These mutations lead to the production of thick, sticky mucus that clogs airways and ducts in various organs.

Rationale for correct answer:

1. Assess the child’s peak expiratory flow: Measuring PEF is the first action because it gives critical information about the child’s current respiratory status and helps prioritize care. Immediate assessment guides whether quick-relief medications or emergency interventions are needed.

Rationale for incorrect answers:
2.Educate the child to avoid triggers: Education is important for long-term asthma management, but it does not address the acute respiratory distress the child is experiencing.

3. Transport the child to the emergency department: Transport may be necessary if severe symptoms persist or worsen, but initial assessment is required to determine the severity and urgency.

4. Notify the child’s parents of his condition: Parental notification is important, but the child’s respiratory status takes priority. Immediate assessment ensures timely intervention.

Take home points

• In children with asthma, airway assessment is the priority during acute symptoms.
• Peak expiratory flow is a simple, objective measure of airway obstruction.
• Nurses must act rapidly to assess, intervene with quick-relief medications, and escalate care if needed.
• Education on triggers and long-term management follows stabilization.

A metered-dose inhaler (MDI)delivers a precise dose of bronchodilator directly to the lungs. It is a handheld device that delivers a precise amount of medication directly to the lungs in the form of a mist or aerosol. Proper technique is critical to ensure maximum drug delivery and therapeutic effect.

Rationale for correct answer:
3.Exhaling fullyempties the lungs, allowing for maximum inhalation of the medication when the MDI is actuated. Proper timing between exhalation and inhalation ensures optimal delivery to the lower airways.

Rationale for incorrect answers:

1. The MDI must be shakento mix the medication and propellant before each dose.

2. The recommended wait time between puffsof the same bronchodilator is usually about 1 minute, not 10 seconds, to allow the first dose to take effect.

4. The canister is pressed once per puff, not continuously for 30 seconds. Holding it down for an extended time is not necessary and can waste medication.

Take home points

• Proper MDI technique involves shaking the inhaler, exhaling fully, actuating while inhaling slowly, holding breath 5–10 seconds, and waiting 1–2 minutes between puffs if a second dose is prescribed.
• Teaching children the correct technique improves drug delivery, asthma control, and reduces side effects.
• Use of a spacer is recommended for children to ensure better deposition of medication in the lungs.

Cystic fibrosis (CF)is an autosomal recessive disorder affecting the exocrine glands. Thick secretions obstruct the pancreatic ducts, preventing digestive enzymes from reaching the intestines. This results in malabsorption of fats and proteins, leading to steatorrheaand azotorrhea, that is, foul-smelling stools with undigested protein.

Rationale for correct answer:

2. Excessive steatorrhea and azotorrhea indicate that pancreatic enzymes are not being adequately delivered to the intestines. This strongly suggests that the child is either missing doses, taking them incorrectly, or underdosed.Compliance with enzyme replacement is critical to prevent growth failure and nutritional deficiencies in CF.

Rationale for incorrect answers:

1. Vitamin noncomplianceleads to deficiencies (A, D, E, K) but does not directly cause steatorrhea and azotorrhea.

3. A high-fat dietalone does not cause these findings if enzymes are taken as prescribed. Enzyme therapy allows CF patients to tolerate high-calorie, high-fat diets.

4. High fiber intakemay cause bloating or gas, but it does not cause fat and protein malabsorption.

Take home points

• Steatorrhea and azotorrhea results from inadequate pancreatic enzyme therapy in CF.
• Pancreatic enzyme replacement must be taken with all meals and snacks.
• Fat-soluble vitamin supplementation is important but does not directly prevent malabsorption symptoms.
• Nutritional management in CF includes a high-calorie, high-protein, high-fat diet with enzyme supplementation.

Cystic fibrosis (CF) is caused by mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR gene). This gene controls the movement of salt and water in and out of cells. This leads to the production of thick, sticky mucus instead of thin, slippery secretions.

Rationale for correct answer:

4.If one parent has CF, that is, two defective genes and the other is a carrier, that is one defective gene there is a 50% chancethe child will have CF since the child inherits one defective gene from each parent. There is also a 50% chancethe child will be a carrier by inheriting one defective gene from the CF parent and one normal gene from the carrier parent.

Rationale for incorrect answers:

1. CF is an autosomal-dominant trait passed on from the child’s motheris incorrect because CF is autosomal recessive, not dominant.

2. CF is an autosomal-dominant trait passed on from the child’s fatheris inaccurate since inheritance is not dominant.

3. The child of parents who are both carriers having a 50% chance of acquiring CFis incorrect. If both parents are carriers 25% chance the child will have CF, 50% chance the child will be a carrier, and 25% chance the child will be unaffected.

Take home points

• CF is autosomal recessive. A child must inherit two defective CF genes to have the disease.
• Two carrier parents: 25% affected, 50% carrier, 25% unaffected.
• One parent with CF and one carrier parent: 50% affected, 50% carrier.
• Genetic counseling is important for families with a history of CF.

Cystic fibrosis (CF) causes thick, sticky mucus that clogs the airways and predisposes the child to respiratory infections. Chest physiotherapy (CPT)is used to mobilize secretions and improve airway clearance. Timing of CPT is important to optimize effectiveness and reduce complications such as vomiting or aspiration.

Rationale for correct answer:

1.Performing CPT 30 minutes before feedingis correct because airway clearance prior to eating reduces the risk of vomiting or aspirationduring therapy. It also helps open the airways, which improves oxygenation and overall tolerance of feeds.

Rationale for incorrect answers:
2.CPT is performed before suctioning, not after. CPT helps loosen secretions, and suctioning may then be used afterward if needed to clear mobilized mucus.
3.Performing CPT 30 minutes after feedingis incorrect because it increases the risk of regurgitation, vomiting, and aspiration, especially in infants.
4.CPT should not be limited to times of congestion or coughing. It is a routine, daily therapyto maintain airway clearance and prevent mucus plugging.

Take home points

• CPT should be scheduled before meals to minimize aspiration risk.
• Airway clearance therapy is a routine part of CF management, not just during illness.
• CPT helps loosen secretions, and suctioning may follow if secretions are not effectively expelled.
• Timing airway clearance correctly supports nutrition and lung function in infants with CF.

Infants with cystic fibrosis (CF) have increased energy requirementsbecause of the extra work of breathing and chronic lung infections. They also experience malabsorption of fats and proteins due to pancreatic enzyme insufficiency.

Rationale for correct answer:
3. A higher-calorie formulais appropriate because infants with CF need more calories than healthy infants to meet growth requirements. This ensures they receive enough energy to compensate for malabsorption and increased metabolic demands. Pancreatic enzymes are also given with feeds to aid digestion.

Rationale for incorrect answers:

1.Increasing fruits and vegetablesdoes not meet the high-calorie and high-protein needs of an infant with CF. While they are healthy foods, they are not calorie-dense enough to support growth in CF.

2.Advancing to whole cow’s milkbefore age 12 months is inappropriate. Infants under 1 year should receive breast milk or formula; cow’s milk lacks adequate iron and essential nutrients and may increase the risk of anemia.

4.Increasing carbohydrate intakealone is not sufficient. Infants with CF need balanced nutrition with adequate protein and fatto support tissue growth and energy needs.

Take home points

• Infants with CF need higher-calorie diets due to malabsorption and increased metabolic demands.
• Pancreatic enzymes must be given with feeds to aid digestion and nutrient absorption.
• Cow’s milk is not recommended before 12 months of age.
• Nutritional care in CF focuses on high-calorie, high-protein, high-fat foods once the child is on solids.

Cystic fibrosis (CF) causes thick, sticky mucus that clogs the airways and predisposes the child to respiratory infections. It is a genetic, multisystem disorderthat primarily affects the lungs and digestive system.

Rationale for correct answer:
2.A double lung transplant can improverespiratory functionand quality of life, allowing the child to live longer. However, it does not correct the underlying genetic defect, so problems with digestion, pancreatic insufficiency, and other CF complications persist.

Rationale for incorrect answers:

1.Lung transplant doesnot cure CF; the genetic defect remains, and other organ complications continue.

3. Transplantation cannotreverse multisystem damagesuch as pancreatic insufficiency, liver disease, and infertility. These issues remain lifelong.

4.While transplantation may prolong life, it should not be described as the only chancefor survival or framed in terms of a specific milestone (like college graduation). This may create unrealistic expectations.

Take home points

• Lung transplantation improves survival in CF patients with end-stage lung disease but is not a cure.
• CF remains a multisystem disease; pancreatic and gastrointestinal issues persist after transplant.
• Families should be counseled about realistic expectations and ongoing care needs after transplant.
• Genetic counseling and continued multidisciplinary support remain essential in CF management.

Cystic fibrosis (CF) is a genetic disorder that causes thick, sticky mucus production in the airways, leading to airway obstruction, inflammation, and infections. It is a genetic, multisystem disorder that primarily affects the lungs and digestive system.

Rationale for correct answer:
4.Wheezing respirationsare an early sign of airway obstruction due to mucus plugging in children with CF. Parents should be taught that recurrent wheezing is one of the first respiratory manifestations.

Rationale for incorrect answers:

1. Abarrel-shaped chestdevelops later in the disease from chronic air trapping and hyperinflation of the lungs. It is not an early sign.

2. Achronic productive coughis more typical in later stages, once recurrent lung infections and persistent mucus buildup have progressed.

3. Bronchiectasis, an irreversible dilation and damage of bronchi, is a long-term complication of repeated lung infections, not an early finding.

Take home points

• Early respiratory signs of CF include wheezing and recurrent respiratory infections.
• Progressive signs include chronic cough, barrel chest, and digital clubbing.
• Parents should report persistent wheezing, frequent cough, or recurrent infections early for prompt intervention.
• Early airway clearance therapy helps reduce progression of lung damage.

Children with cystic fibrosis (CF) often develop gastrointestinal complications due to thick, sticky mucus secretions that block the intestines. Constipation is common and is usually managed with oral stool softeners or osmotic laxatives.

Rationale for correct answer:
3.MiraLAX (polyethylene glycol)is commonly prescribed for constipation in children with CF because it draws water into the stool, making it easier to pass. It is safe for long-term use and effective in relieving constipation caused by thick mucus secretions.

Rationale for incorrect answers:

1. Surgery isnot the first-line treatmentfor constipation in CF. Surgery is only considered for severe complications such as distal intestinal obstructive syndrome (DIOS)that does not respond to medical management.

2.IV fluidsmay be given if the child is dehydrated but are not the primary treatment for constipation. Hydration helps, but medications like MiraLAX are typically needed.

4.A clear liquid dietmay temporarily ease symptoms in severe obstruction but is not standard treatment for routine constipation in CF. Adequate nutrition is essential for growth and health.

Take home points

• Constipation in CF is usually managed with osmotic laxatives like MiraLAX.
• Surgery is reserved for severe, non-resolving intestinal obstruction (DIOS).
• Adequate hydration and enzyme therapy also help prevent constipation.
• GI symptoms in CF require ongoing monitoring to prevent complications.

Cystic fibrosis (CF) is a chronic, progressive genetic disorder affecting multiple systems, primarily the lungs and gastrointestinal tract. Therapeutic management focuses on maintaining adequate nutrition, promoting airway clearance, preventing infections, encouraging activity, and supporting adherence to prescribed therapies to improve quality of life and extend survival.

Rationale for correct answers:

1. Ahigh-protein, high-calorie dietis necessary because CF patients have increased metabolic demands and malabsorption due to pancreatic enzyme insufficiency.

3. Exerciseimproves lung function, enhances airway clearance, strengthens respiratory muscles, and promotes overall health.

4. Minimizing pulmonary complicationsis a cornerstone of CF management. This includes chest physiotherapy, airway clearance techniques, antibiotics, and vaccinations.

5. Medication complianceis critical for effectiveness of therapies such as pancreatic enzymes, fat-soluble vitamins, bronchodilators, mucolytics, antibiotics, and CFTR modulators.

Rationale for incorrect answers:
2.While CF patients require high-calorie and nutrient-rich diets, recommending a specifically high-fat, high-carbohydrate dietis misleading. Fat and carbohydrate intake should be balanced with protein, with pancreatic enzymes given to aid digestion. The emphasis is on high-calorie, high-protein nutrition, not excessive fat/carbs alone.

Take home points

• CF management requires a multidisciplinary approach (nutrition, respiratory therapy, medications).
• High-protein, high-calorie diets with enzyme supplementation support growth and development.
• Daily airway clearance + exercise improves lung function and reduces complications.
• Long-term survival depends heavily on adherence to prescribed therapies and consistent medical follow-up.

The sweat chloride testis the gold standard for diagnosing cystic fibrosis (CF). CF causes defective chloride transport across epithelial cells, leading to abnormally salty sweat. Elevated chloride levels in sweat confirm the diagnosis.

Rationale for correct answer:
3. Asweat chloride level ≥60 mEq/Lis a positive diagnostic indicator of CF in children and adults. This finding strongly supports CF when clinical features are present.

Rationale for incorrect answers:

1. 30 mEq/Lis below the diagnostic threshold. In infants under 6 months, values ≥30 mEq/L are considered suspicious but not diagnostic.

2. 25 mEq/Lis normal and does not indicate CF.

4. 50 mEq/Lis considered borderline/suspicious but not diagnostic. Additional testing (genetic testing or repeat sweat chloride test) would be required.

Take home points

• Sweat chloride level ≥60 mEq/L is diagnostic for CF.
• The sweat chloride test is most accurate when performed at a certified CF center.

Cystic fibrosis (CF) is a genetic disorderthat affects the lungs, digestive system, and other organs by causing the body to produce thick, sticky mucus. CF is caused by mutations in the CFTR gene, which regulates salt and water movement in and out of cells. These mutations disrupt the function of the CFTR protein, leading to mucus buildup.

Rationale for correct answer:
4.Meconium ileusoccurs in newborns with CF due to viscous intestinal secretions,and it may present as abdominal distention, failure to pass meconium within the first 24–48 hours, and bilious vomiting. Early recognition is crucial for prompt surgical or medical intervention.

Rationale for incorrect answers:

1. While CF can later cause intestinal blockages or constipation, it is not typically an early neonatal sign.

2. Decreased appetitemay occur later due to malabsorption, but it is not an early sign in newborns.

3.CF is not associated with elevated albumin levels. Instead, malabsorption can lead to hypoalbuminemiaover time.

Take home points

• Meconium ileus is a hallmark early sign of CF in newborns.
• Newborns with delayed meconium passage or abdominal distention should be evaluated for CF.
• Early detection allows for timely management of nutrition, pancreatic enzymes, and respiratory care.
• Newborn screening and sweat chloride testing confirm the diagnosis.

Children with cystic fibrosis (CF)often have pancreatic insufficiency, leading to malabsorption of fats and proteins. Pancreatic enzyme supplements are essential to improve digestion and nutrient absorption. Proper timing and dosing of enzymes are critical to maximize their effectiveness.

Rationale for correct answer:
4.Pancreatic enzymes should be taken with every meal and snackto ensure that food is properly digested, reduce steatorrhea, and promote adequate growth and nutrition.

Rationale for incorrect answers:

1. Many CF pancreatic enzyme capsules contain microbeads or microspheresthat can be sprinkled on soft foodsif the client cannot swallow capsules whole.

2. Crushing enzymes can destroy the enteric coating, making them ineffective and potentially irritating to the stomach.

3. While frequent meals and snacks are appropriate for nutrition, this alone does not address enzyme administration, which is the key point.

Take home points

• Administer pancreatic enzymes with all meals and snacks for proper digestion.
• Do not crush or chew enzyme capsules/microbeads unless specifically formulated to be sprinkled on soft food.
• Encourage a high-calorie, high-fat, high-protein diet to meet nutritional needs.
• Monitor stool characteristics and growth to assess enzyme effectiveness.

Cystic fibrosis (CF) is a genetic disorderthat affects the lungs, digestive system, and other organs by causing the body to produce thick, sticky mucus. CF is caused by mutations in the CFTR gene, which regulates salt and water movement in and out of cells. These mutations disrupt the function of the CFTR protein, leading to mucus buildup.

Rationale for correct answer:
3.Chest physiotherapy (CPT)helps mobilize and clear secretions, improving airway clearance, lung function, and oxygenation. CPT is a primary therapy for CF. Regular sessions help prevent mucus plugging, reduce infection risk, and improve breathing.

Rationale for incorrect answers:

1. Exercise isencouragedin children with CF because it helps loosen mucus and maintain cardiopulmonary fitness.

2. Coughing is anatural mechanism for clearing mucus. Suppressants may worsen airway obstruction. Antihistamines are not routinely indicated unless allergies are present.

4. Bronchodilatorsare typically given before CPTto open airways, making airway clearance more effective.

Take home points

• Chest physiotherapy 2–4 times daily is essential in CF care.
• Administer bronchodilators before CPT to enhance mucus clearance.
• Encourage regular physical activity to support lung health.
• Avoid suppressing cough; coughing is an important mechanism for airway clearance.

Cystic fibrosis (CF) is a genetic disorderthat affects the lungs, digestive system, and other organs by causing the body to produce thick, sticky mucus. CF is caused by mutations in the CFTR gene, which regulates salt and water movement in and out of cells. These mutations disrupt the function of the CFTR protein, leading to mucus buildup.

Rationale for correct answer:

1. Cystic fibrosis (CF) is an autosomal recessive genetic disorder.Both parents must be carriers of the CF gene for a child to be affected. Genetic counselingprovides information on inheritance patterns, carrier status, and reproductive risks, helping families make informed decisions about future pregnancies.

Rationale for incorrect answers:
2.If both parents are carriers, the chance for a completely unaffected childis 25%, not 50%.

3. The chance of having an affected childwhen both parents are carriers is 25%, not 50%.

4. The chance of a child being a carrieris 50%, not 25%.

Take home points

• CF is autosomal recessive, meaning both parents must carry the gene for a child to be affected.
• Carrier status and inheritance risks should be discussed with a genetic counselor before future pregnancies.
• Understanding genetic risk helps parents plan and access prenatal testing or assisted reproductive options.
• Counseling provides psychosocial support in addition to medical information.

Children with cystic fibrosis (CF) often have pancreatic insufficiency, which leads to malabsorption of fat-soluble vitamins. Supplementation is necessary to prevent deficiencies, maintain growth, support immune function, and promote bone health.

Rationale for correct answers:

1. Vitamin Ais essential for vision, skin integrity, and immune function. Fat malabsorption in CF can lead to deficiency.

3. Vitamin Dsupports calcium absorption and bone health. Children with CF are at risk for rickets and poor bone mineralization.

4. Vitamin Eis an antioxidantimportant for protecting cell membranes and preventing hemolytic anemia.

5. Vitamin Kis necessary for blood clotting.Deficiency can lead to bleeding disorders in children with CF.

Rationale for incorrect answers:
2.Vitamin Cis water-solubleand generally not deficient in CF if the child consumes a normal diet. Supplementation is not routinely required.

6. Vitamin B12is also water-soluble, and routine supplementation is usually unnecessary unless a deficiency is diagnosed.

Take home points

• Children with CF require daily supplementation of fat-soluble vitamins (A, D, E, K) due to malabsorption.
• Monitoring growth, bone health, and coagulation status helps ensure supplementation is effective.
• Caregivers should understand that water-soluble vitamins are generally obtained through diet, while fat-soluble vitamins need supplementation.
• Supplements should be given with meals containing fat to enhance absorption.

Cystic fibrosis (CF)is a genetic disorder affecting exocrine glands, resulting in thick, sticky mucus in the lungs, pancreas, and other organs. Children with CF are also prone to recurrent respiratory infections due to mucus buildup and impaired airway clearance.

Rationale for correct answer:

1. Salty skin on the lips or skinis a classic early sign of cystic fibrosis, especially in children with a history of frequent respiratory infections. This warrants evaluation by the primary health-care provider and confirmatory testing such as a sweat chloride test.

Rationale for incorrect answers:
2.Asthmacauses wheezing, coughing, and shortness of breath but does not cause salty-tasting skin.

3. Bronchiolitisis an acute viral infection of the lower respiratory tract in infants, causing cough, wheezing, and congestion. Salty skin is not a feature.

4. Pharyngitisinvolves sore throat, fever, and inflammation of the pharynx, and does not cause salty-tasting skin.

Take home points

• Salty-tasting skin is a hallmark early sign of CF and should prompt further evaluation.
• Recurrent respiratory infections in infancy increase suspicion for CF.
• The sweat chloride test is the standard diagnostic tool.

Early diagnosis allows initiation of airway clearance, nutritional support, and infection prevention strategies.

Bronchopulmonary dysplasia (BPD)is a chronic lung disease of infancy, often seen in premature infants requiring prolonged ventilation or oxygen therapy. These infants frequently need home oxygen, tracheostomy care, and close monitoring. They are highly susceptible to respiratory infections, hypoxemia, and complications with high altitude.

Rationale for correct answer:
3.Camping trip to Yellowstone National Park:Yellowstone is at a high altitude, which has lower oxygen levels in the air. An infant with BPD on home oxygen is at high risk of severe hypoxemia and respiratory distress in such environments. Travel to high-altitude areas is contraindicated until the infant’s respiratory status is stable and cleared by the healthcare team.

Rationale for incorrect answers:

1. Calling the pediatrician if fever or increased secretions: These are early signs of infection or tracheostomy complications, and prompt medical evaluation is necessary.

2. Loosely covering the tracheostomy with a bib during feeding/outside:A loose cover protects the airway from food particles, wind, and debris without blocking airflow.

4. Notifying the power company about home oxygen use:This ensures the family has priority in case of a power outage since home oxygen is life-sustaining equipment.

Take home points

• Infants with BPD on home oxygen should not be exposed to high altitudes without medical clearance.
• Parents should be taught infection prevention, safe tracheostomy care, and oxygen safety.
• Backup power planning and emergency contacts are essential for families using home oxygen.
• Nurses must carefully assess caregiver understanding before discharge to ensure safety at home.

Bronchopulmonary dysplasia (BPD)is a chronic lung disease that primarily affects premature neonates who require prolonged ventilatory support or high concentrations of oxygen. It results from lung injury due to mechanical ventilation, oxygen toxicity, and inflammation, leading to impaired alveolar development, fibrosis, and airway remodeling.

Rationale for correct answer:
2.Exposure to high positive airway pressure and high oxygen concentrations during the first weeks of life can damage immature lungs and interfere with alveolar growth, leading to BPD.

Rationale for incorrect answers:

1. Bronchiolitisis an acute viral infectionof the lower respiratory tract, not caused by ventilatory support or oxygen therapy.

3. Asthmais a chronic inflammatory airway disease, usually diagnosed later in childhood, not directly caused by neonatal ventilatory support.

4. Cystic fibrosisis a genetic disorder caused by mutations in the CFTR gene, unrelated to ventilatory support or oxygen exposure.

Take home points

• Neonates exposed to high oxygen and positive pressure ventilation are at increased risk for BPD.
• BPD involves chronic lung inflammation, airway remodeling, and impaired alveolarization.
• Preventive strategies include gentle ventilation, minimizing oxygen exposure, and early surfactant therapy.