Long-term Respiratory Dysfunction Exams

Long-term Respiratory Dysfunction

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Study Questions

Practice Exercise 1

Question 1 :

Which laboratory result will provide the most important information regarding the respiratory status of a child with an acute asthma exacerbation?

A. CBC. B. ABG. C. BUN. D. PTT.

Explanation

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.

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Question 2 :

What is the most important piece of information that the nurse must ask the parent of a child in status asthmaticus?

A. “What time did your child eat last?” B. “Has your child been exposed to any of the usual asthma triggers?” C. “When was your child last admitted to the hospital for asthma?” D. “When was your child’s last dose of medication?”

Explanation

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.

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Question 3 :

Which statement by the parent of a child using an albuterol inhaler leads the nurse to believe that further education is needed on how to administer the medication?

A. “I should administer two quick puffs of the albuterol inhaler using a spacer.” B. “I should always use a spacer when administering the albuterol inhaler.” C. “I should be sure that my child is in an upright position when administering the inhaler.” D. “I should always shake the inhaler before administering a dose.”

Explanation

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.

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Question 4 :

Which should the nurse administer to provide quick relief to a child with asthma who is coughing, wheezing, and having difficulty catching her breath?

A. Prednisone. B. Montelukast (Singulair). C. Albuterol. D. Fluticasone (Flovent).

Explanation

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.

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Question 5 :

Which child with asthma should the nurse see first?

A. A 12-month-old who has a mild cry, is pale in color, has diminished breath sounds, and has an oxygen saturation of 93%. B. A 5-year-old who is speaking in complete sentences, is pink in color, is wheezing bilaterally, and has an oxygen saturation of 93%. C. A 9-year-old who is quiet, is pale in color, and is wheezing bilaterally with an oxygen saturation of 92%. D. A 16-year-old who is speaking in short sentences, is wheezing, is sitting upright, and has an oxygen saturation of 93%.

Explanation

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.

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Question 6 :

Which breathing exercises should the nurse have an asthmatic 3-year-old child do to increase her expiratory phase?

A. Use an incentive spirometer. B. Breathe into a paper bag. C. Blow a pinwheel. D. Take several deep breaths.

Explanation

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.

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Question 7 :

The parents of a 6-year-old who has a new diagnosis of asthma ask the nurse what to do to make their home a more allergy-free environment. Which is the nurse’s best response?

A. “Use a humidifier in your child’s room.” B. “Have your carpet cleaned chemically once a month.” C. “Wash household pets weekly.” D. “Avoid purchasing upholstered furniture.”

Explanation

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.

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Question 8 :

Which characteristic distinguishes status asthmaticus from asthma?

A. Several attacks per month B. Less than six attacks per year C. Little or no response to bronchodilators D. Constant and unrelieved by broncho-dilators

Explanation

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.

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Question 9 :

An 8-year-old child, who has a history of asthma, is seen in the office of the school nurse with coughing
and wheezing. Which of the following actions should the nurse perform first?

A. Assess the child’s peak expiratory flow. B. Educate the child to avoid triggers. C. Transport the child to the emergency department. D. Notify the child’s parents of his condition.

Explanation

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.

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Question 10 :

A 10-year-old child has been prescribed an MDI administered bronchodilator. Which of the following actions should the nurse teach the child to perform when taking the medication?

A. Take care not to shake the medication container before administering. B. Wait no more than 10 seconds between administrations of the medication. C. Exhale completely before placing the medication mouthpiece in the mouth. D. Compress the container for 30 seconds before inhaling the medication.

Explanation

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.

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End of Questions

Exams on Long-term Respiratory Dysfunction

Practice Exercise 1

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Lessons

Table of Contents

Objectives
Introduction
Asthma
Practice Exercise 1
Cystic Fibrosis
Practice Exercise 2
Allergic Rhinitis
Obstructive Sleep Apnea
Bronchopulmonary Dysplasia
Practice Exercise 3
Summary
Comprehensive Questions

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Objectives

Describe the incidence, pathophysiology, etiology, clinical manifestations, diagnostic evaluations, and formulate therapeutic and nursing management plans for children with asthma.
Identify and manage acute exacerbations, such as status asthmaticus.
Understand the incidence, pathophysiology, etiology, clinical manifestations, diagnostic evaluations, and formulate therapeutic and nursing management plans for children with cystic fibrosis (CF)
Describe the incidence, pathophysiology, etiology, clinical manifestations, diagnostic evaluations, and formulate therapeutic and nursing management plans for children with allergic rhinitis.
Describe the incidence, pathophysiology, etiology, clinical manifestations, diagnostic evaluations, and formulate therapeutic and nursing management plans for children with obstructive sleep apnea (OSA).
Describe the incidence, pathophysiology, etiology, clinical manifestations, diagnostic evaluations, and formulate therapeutic and nursing management plans for children with bronchopulmonary dysplasia (BPD).
Educate children and caregivers on disease management, including proper medication administration, trigger avoidance, and promoting an allergy-free environment.
Understand the unique challenges and nursing considerations for infants with BPD, including tracheostomy care, and for adolescents transitioning to adulthood with chronic conditions like CF.

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Introduction

Long-term respiratory dysfunction in children refers to chronic conditions affecting the lungs and airways, often leading to persistent symptoms, impaired quality of life, and potential long-term morbidity.
These conditions range from common allergic responses to life-limiting genetic disorders. Pediatric respiratory systems have unique anatomical differences.
Children have smaller, more compliant airways that are easily obstructed by inflammation, mucus, or foreign objects. Their short, narrow trachea and enlarged tonsil/adenoid tissue increase the risk of airway compromise.
Infants are obligate nose breathers, and young children rely heavily on the diaphragm, making them prone to fatigue during periods of increased respiratory effort. They also have fewer alveoli and less surface area for gas exchange compared to adults.
These vulnerabilities mean that chronic conditions like asthma, cystic fibrosis, and bronchopulmonary dysplasia present unique, complex challenges.
Nursing care is pivotal in managing these chronic diseases, focusing on patient and family education, acute symptom control, minimizing complications, and maximizing developmental and quality-of-life outcomes.

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Asthma

Asthma is a chronic inflammatory disorder of the airways characterized by recurrent episodes of wheezing, breathlessness, chest tightness, and coughing, particularly at night or in the early morning. These episodes are usually associated with widespread, but variable, airflow obstruction that is often reversible spontaneously or with treatment.

Incidence

It is the most common chronic disease of childhood, affecting millions of children globally.
Incidence varies but is higher in lower socioeconomic groups and certain ethnic populations.
It is a leading cause of school absenteeism and pediatric hospital visits.

Pathophysiology

Airway Inflammation: Chronic inflammation, driven by IgE-mediated responses to triggers (allergens or irritants), involves immune cells (mast cells, eosinophils, T-lymphocytes).
Bronchoconstriction (Bronchospasm): Smooth muscles surrounding the airways constrict acutely in response to inflammatory mediators (e.g., histamine, leukotrienes), leading to narrowing.
Increased Mucus Production: Mucosal edema and hypersecretion of thick, tenacious mucus further plug the airways.

The combination of these three factors leads to airway hyper-responsiveness (exaggerated bronchoconstriction in response to various stimuli) and airflow obstruction, primarily affecting the expiratory phase, trapping air in the alveoli (air trapping).

Etiology and Triggers

The exact etiology is multifactorial, involving a complex interplay of genetic predisposition (a family history of atopy or asthma is a major risk factor) and environmental factors.

Etiology/Triggers	Examples
Allergens	Pollen, dust mites, mold, animal dander, cockroach droppings.
Irritants	Tobacco smoke (most significant), air pollution, strong odors, cleaning products.
Infections	Viral upper respiratory infections (most common trigger for exacerbations in children).
Exercise	Especially in cold or dry air. (Exercise-induced bronchoconstriction/asthma).
Other	Cold air, strong emotions/stress (crying, laughing), gastroesophageal reflux (GERD).

Clinical Manifestations

Symptoms often vary in severity and frequency:

Classic Triad: Cough (non-productive, hacking, worse at night), Wheezing (high-pitched musical sound on expiration), and Dyspnea (shortness of breath).
Chest tightness: More common in older children).
Prolonged expiratory phase: The time the child takes to exhale is longer than normal.
Use of accessory muscles: Retractions, nasal flaring in infants/toddlers.
Tachypnea, Tachycardia.

Restlessness/Irritability: These are early signs of hypoxia.

Diagnostic Evaluation

History and Physical Exam: Recurrent symptoms, family history, and characteristic lung sounds (wheezing).
Pulmonary Function Tests (PFTs)/Spirometry (usually for children ≥5−6 years): Measures forced expiratory volume in 1 second (FEV1) and forced vital capacity (FVC). A significant reversibility (e.g., 12% or greater improvement in FEV1 after a bronchodilator) supports the diagnosis.
Peak Expiratory Flow Rate (PEFR): Measured using a Peak Flow Meter. Compares the child's result to their personal best or predicted normal. Used for daily monitoring and in the Asthma Action Plan (Green, Yellow, Red Zones).
Allergy Testing (Skin or Blood): To identify specific triggers.
Arterial Blood Gases (ABGs): Not routine, but critical in severe exacerbations (status asthmaticus).
- Early: Respiratory Alkalosis (↓PaCO2, ↑pH) due to hyperventilation (compensatory mechanism).
- Worsening/Fatigue: Normalizing PaCO2 (This is a danger sign indicating respiratory fatigue and impending failure, as the child is no longer able to hyperventilate.
- Late/Failure: Respiratory Acidosis (↑PaCO2, ↓pH) indicating CO2 trapping and poor gas exchange.

Therapeutic Management

Management is guided by the severity and frequency of symptoms, following a stepwise approach (e.g., National Asthma Education and Prevention Program guidelines).

Pharmacologic:
- Quick-Relief (Rescue) Medications:
  - Short-Acting Beta Agonists (SABAs): Albuterol (Salbutamol). Used for acute symptoms and before exercise. Relaxes bronchial smooth muscle.
- Long-Term Control (Preventer) Medications:
  - Inhaled Corticosteroids (ICS): The most effective long-term control therapy. Reduces inflammation and hyper-responsiveness. (e.g., Fluticasone, Budesonide).
  - Long-Acting Beta Agonists (LABAs): Used only in combination with an ICS for moderate/severe persistent asthma. (e.g., Salmeterol, Formoterol).
  - Leukotriene Modifiers: Oral medications (e.g., Montelukast). Blocks inflammatory leukotrienes.
  - Combination Inhalers: ICS + LABA (e.g., Budesonide/Formoterol).

Step-wise Approach for Asthma Management

Prevention and Nursing Care Management

I. Asthma Action Plan

Crucial for self-management. Color-coded zones based on symptoms and PEFR to guide treatment (medication, when to call the provider, when to go to the Emergency Department).

II. Proper MDI Use

A Metered-Dose Inhaler (MDI) should almost always be used with a spacer/holding chamber for children to ensure proper drug delivery and minimize side effects (like thrush from ICS).

MDI Use with Spacer	MDI Use without Spacer
1. Shake the inhaler well and remove the cap.	1. Shake the inhaler well and remove the cap.
2. Insert the mouthpiece of the MDI into the spacer end.	2. Tilt the head back slightly.
3. Place the mask (for infants/toddlers) or mouthpiece (older children) over the face/in the mouth, ensuring a tight seal.	3. Breathe out gently (empty lungs).
4. Press the canister once to release the medication into the spacer.	4. Place the mouthpiece 1-2 inches in front of the mouth or seal lips around the mouthpiece.
5. Slowly inhale 5-10 deep breaths from the spacer, holding the last breath for 10 seconds if possible. (For mask, hold for 30 seconds or 5-10 breaths).	5. Start to breathe in slowly and deeply through the mouth, and press the canister once.
6. Wait 30-60 seconds between puffs if more than one is ordered.	6. Continue to inhale slowly for 3-5 seconds, then hold breath for 10 seconds if possible.
A spacer improves lung deposition and reduces oropharyngeal deposition.	This is less effective delivery; requires perfect coordination.

III. Ways to Increase Expiratory Phase

The goal is to encourage a prolonged, forced expiration to help clear trapped air and secretions.

Blowing a Pinwheel or Bubbles: Fun, simple activities that require a sustained expiratory effort.
Playing a wind instrument (e.g., harmonica, recorder).
Blowing up balloons (for older, stable children).
"Huffy" or "Foggy" breathing exercises: Exhaling as if trying to fog up a mirror.

IV. Home Allergy-Free Environment

Education focuses on eliminating common triggers:

Dust Mites: Use allergen-proof covers on mattresses, box springs, and pillows. Wash bedding weekly in hot water (≥130∘F). Remove carpets, or vacuum with a HEPA filter vacuum.
Pets: Keep pets out of the child's bedroom and off upholstered furniture. Regular bathing of pets may help but is less effective than removal.
Mold: Fix leaks, use a dehumidifier to keep relative humidity below 50%.
Smoke: No smoking in the home or car. Avoid wood-burning stoves/fireplaces.
Pollen: Keep windows and doors closed, especially during high pollen seasons. Use air conditioning.

Status Asthmaticus

Definition: A severe, life-threatening asthma episode that is refractory to initial treatment such as initial SABA treatments.
Manifestations: Severe respiratory distress, poor air movement ("silent chest"), impending respiratory failure indicated by cyanosis, decreased level of consciousness, and normalizing or rising PaCO2.
Nursing Care:
1. Continuous Monitoring: SpO2, HR, RR, LOC, and ABGs.
2. Oxygen: Administer to maintain SpO2≥92%.
3. Medications: Continuous or frequent nebulized SABA (Albuterol/Salbutamol), IV or oral Systemic Corticosteroids (e.g., Methylprednisolone or Prednisone), and consider IV Magnesium Sulfate (a potent smooth muscle relaxant).
4. Airway Management: Prepare for intubation and mechanical ventilation if respiratory failure is imminent.

Nursing Insight: A "silent chest" following wheezing is a dire emergency! It means air movement has stopped due to severe airway obstruction and requires immediate, aggressive intervention.

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Cystic Fibrosis

Cystic Fibrosis is an autosomal recessive genetic disease that affects the exocrine glands, leading to the production of abnormally thick and sticky mucus that obstructs ducts and passages in multiple body systems, most notably the respiratory, digestive, and reproductive systems.

Incidence

It is the most common fatal inherited disease in Caucasians in the United States.
Improvements in treatment have significantly extended the median life expectancy to ∼40−50 years.

Pathophysiology / Inheritance Pattern

Inheritance Pattern: Autosomal Recessive.
- The child must inherit two copies of the mutated gene (one from each carrier parent) to have CF.
If both parents are carriers (Cc), there is a 25% (1 in 4) chance with each pregnancy that the child will have CF (cc).

Etiology

A mutation in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene on chromosome 7.
- The CFTR protein acts as a chloride ion channel in epithelial cells.
- In CF, the defective CFTR protein fails to transport chloride out of the cells.

This lack of chloride secretion and increased sodium and water reabsorption into the cells leads to dehydrated, viscous secretions in the sweat ducts, lungs, pancreas, liver, and intestine.

Clinical Manifestations per Body System

Respiratory:
- Thick mucus obstructs bronchioles, leading to air trapping, atelectasis, and recurrent infections (chronic cough, wheezing, barrel chest, clubbing).
- Infections often involve specific bacteria, like Staphylococcus aureus and later Pseudomonas aeruginosa.
- Chronic colonization leads to bronchiectasis (irreversible dilation and destruction of the bronchial walls).
Gastrointestinal (Pancreas):
- Thick secretions block the pancreatic ducts, preventing the release of digestive enzymes (lipase, amylase, protease) into the small intestine.
- Results in malabsorption of fats, proteins, and fat-soluble vitamins (A, D, E, K).
- Clinical Signs: Steatorrhea (foul-smelling, fatty, bulky stools), failure to thrive, malnutrition.
- Can lead to CF-related diabetes (CFRD) due to pancreatic damage.
- Gastrointestinal (Intestine/Liver):
  - Meconium Ileus: The initial presentation in ∼10−20% of newborns; thick meconium causes intestinal obstruction.
  - Biliary obstruction leading to focal biliary cirrhosis.
- Integumentary/Sweat Glands:
  - Defective CFTR in the sweat ducts prevents reabsorption of chloride and sodium.
  - Results in abnormally high concentrations of salt in the sweat.
  - Clinical Sign: Parents often report their child "tastes salty" when kissed.
- Reproductive:
  - Males are typically infertile due to congenital bilateral absence of the vas deferens.
  - Females have reduced fertility due to thickened cervical mucus.

Diagnostic Evaluation

Newborn Screening: All U.S. states screen for CF. Initial test measures Pancreatic Enzyme Immunoreactive Trypsinogen (IRT) in a blood spot; high IRT indicates further testing.
Sweat Chloride Test (Gold Standard): Measures the chloride concentration in the sweat.
- Procedure: Pilocarpine iontophoresis is used to stimulate localized sweating, and the sweat is collected and analyzed.
- Diagnostic Levels:
  - Positive/Diagnostic: ≥60 mEq/L
  - Intermediate: 30−59 mEq/L (requires further genetic testing)
  - Normal: ≤29 mEq/L
  - Stool Analysis: Measures fecal fat content to confirm fat malabsorption.

Therapeutic Management

I. Chest Physiotherapy (CPT) and Airway Clearance

The cornerstone of management. Helps to mobilize and clear thick mucus from the airways.
Techniques: Percussion, vibration, postural drainage, High-Frequency Chest Wall Oscillation (HFCWO) vest, positive expiratory pressure (PEP) mask, and active cycle of breathing techniques.
Appropriate Timings: 1-3 times per day (and sometimes more during exacerbations), usually before meals or at least one hour after meals to avoid regurgitation/vomiting.
Medications: Dornase Alfa (Pulmozyme) decreases mucus viscosity and hypertonic saline mobilizes secretions.

II. Nutrition
High-Calorie, High-Protein Diet: To counteract malabsorption and meet increased metabolic demands due to chronic infection/work of breathing.
Fat-Soluble Vitamin Supplementation: Daily supplements of A, D, E, K.

III. Pancreatic Enzyme Replacement Therapy (PERT)

Enzyme capsules containing lipase, amylase, protease must be taken with all meals and snacks to digest food and absorb nutrients.
How and When to Take Them: Swallow capsules whole, or open and sprinkle on small amount of acidic food (e.g., applesauce) immediately before eating. Do not chew or mix with alkaline foods.
- Dosing: Individually titrated based on weight, fat intake, and stool consistency (goal is 1-2 formed stools/day).

IV. Treatment of Constipation/Distal Intestinal Obstruction Syndrome (DIOS)

DIOS is a partial or complete obstruction of the small intestine or ileocecal valve by thick stool and mucus.
Treatment: Oral laxatives like Polyethylene Glycol (Miralax), osmotic solutions, and sometimes enemas are used to relieve the obstruction.

V. Lung Transplant

Considered for patients with advanced lung disease, typically when FEV1<30% of predicted.

Complications

Respiratory complications: Chronic lung infections, bronchiectasis, respiratory failure, pneumothorax, hemoptysis, and allergic bronchopulmonary aspergillosis (ABPA).
Nutritional complications: Malnutrition, growth failure, vitamin deficiencies, and osteoporosis.
Reproductive complications: Infertility in males due to absence of the vas deferens.
Psychosocial complications: Depression, anxiety, social isolation, and decreased quality of life.

Nursing Care Management

Infection Control: Strict hand hygiene; patients with CF should be separated from one another ("Six-foot rule" or distance ≥6 feet) to prevent cross-contamination of resistant organisms.
Monitor Nutritional Status: Daily weights, growth charts, BMI, and serum albumin.
Hydration: Encourage fluids to thin secretions.
Psychosocial Support: CF is a major chronic illness; support groups and mental health resources are vital.

Transitioning to Adulthood

Focus: Shifting responsibility for care from parent/guardian to the adolescent/young adult.
Goals: Medication self-administration, scheduling CPT, managing appointments, understanding insurance, discussing fertility and reproductive health.

Nursing Insight: Due to the risk of Pseudomonas transmission, patients with CF should never share equipment like nebulizers and should maintain a physical distance from others with CF in healthcare settings.

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