NLE Respiratory Nursing — Chronic Obstructive & Restrictive Pulmonary DisordersStudy Notes

COPD is defined as a progressive, largely irreversible airflow limitation characterized by an abnormally slow rate of airflow during expiration. It is primarily caused by cigarette smoking and, critically in the Philippine context, biomass fuel exposure (cooking smoke, wood fires) and occupational inhalation injuries (silica, coal dust). COPD is an umbrella diagnosis encompassing two main pathophysiological patterns: chronic bronchitis and emphysema, which frequently coexist in individual patients. **Definition and Epidemiology**: COPD affects millions globally and is a leading cause of morbidity and mortality. In the Philippines, COPD prevalence is compounded by high smoking rates and widespread indoor air pollution from biomass fuels—a critical public health issue that nurses must address during patient education. **Key Causative Factors**: (1) Cigarette smoking (most common); (2) Biomass/cooking-fuel smoke exposure—particularly relevant in Filipino homes where charcoal stoves and wood fires are used; (3) Occupational exposures (silica, asbestos, coal dust); (4) Air pollution; (5) Genetic factors (alpha-1 antitrypsin deficiency, though rare). **Pathophysiological Mechanism**: In COPD, chronic inflammation of the airways leads to airway remodeling, mucus hypersecretion, loss of elastic recoil in emphysema, and air trapping. The result is progressive narrowing of airways and reduced surface area for gas exchange, culminating in V/Q (ventilation-perfusion) mismatch, hypoxemia, and hypercapnia (CO2 retention).

**Clinical Definition**: Chronic bronchitis is clinically defined as a productive cough lasting at least 3 months in each of 2 consecutive years, in the absence of other causes of cough. This definition, established by the American Thoracic Society, is a cornerstone diagnostic criterion. **Pathophysiology**: Chronic airway inflammation triggers hypertrophy of mucus glands in the bronchial tree, leading to excessive mucus production. The mucus, combined with airway narrowing and loss of mucociliary clearance, results in airway obstruction, air trapping, and ventilation-perfusion mismatch. This leads to chronic alveolar hypoxemia and hypercapnia. The hypoxemia triggers pulmonary vasoconstriction and polycythemia (elevated red blood cell count as a compensatory response to chronic hypoxemia). **Classic Clinical Presentation—'Blue Bloater'**: The term 'blue bloater' refers to the characteristic physical appearance: (1) Cyanosis (blue skin) from chronic hypoxemia; (2) Peripheral edema and weight gain from right-sided heart failure (cor pulmonale); (3) Copious, purulent sputum; (4) Obesity; (5) Distended neck veins and right upper quadrant abdominal pain (signs of cor pulmonale). These patients typically retain CO2 and have a hypoxic drive to ventilation. **Respiratory Assessment Findings**: Increased antero-posterior (AP) chest diameter, barrel chest, pursed-lip breathing (spontaneous breathing technique to keep airways open), decreased breath sounds, wheezing, crackles, and use of accessory muscles. **Gas Exchange Abnormalities**: Chronic respiratory acidosis with metabolic compensation—elevated PaCO2, elevated HCO3-, pH near-normal (7.35–7.45 range), and low PaO2.

**Pathophysiology**: Emphysema involves destruction of alveolar walls and loss of elastic recoil of the lungs. Alveoli coalesce into large air-filled spaces (bullae), dramatically reducing the surface area available for gas exchange. Elastin and collagen are destroyed by neutrophil elastase, which is typically held in check by alpha-1 antitrypsin. In emphysema, either excessive elastase activity or antitrypsin deficiency (genetic) allows progressive alveolar destruction. **Mechanism of Air Trapping**: Without elastic recoil, airways collapse during expiration, trapping air distally. This leads to hyperinflation of the lungs, flattening of the diaphragm, and mechanical disadvantage of respiratory muscles. The patient compensates by breathing more deeply and using accessory muscles, and by spontaneously adopting pursed-lip breathing to maintain positive airway pressure during expiration. **Classic Clinical Presentation—'Pink Puffer'**: The term 'pink puffer' describes the appearance: (1) Pink or flushed skin (from preserved oxygenation due to hyperventilation); (2) Lean, wasted appearance (from high metabolic demands of labored breathing and poor nutrition); (3) Pursed-lip breathing (conscious or unconscious strategy); (4) Use of accessory muscles; (5) Minimal cough (sparse secretions in emphysema); (6) Dyspnoea on exertion. These patients typically maintain relatively preserved oxygenation through hyperventilation and do not retain CO2 as readily as 'blue bloaters.' **Physical Assessment**: Barrel chest (increased AP diameter), hyperresonance on percussion, diminished breath sounds, and prolonged expiration. Late findings include clubbing and cyanosis. **Genetic Form**: Alpha-1 antitrypsin (AAT) deficiency, inherited in an autosomal recessive pattern, causes early-onset emphysema (typically before age 45) in non-smokers or light smokers. Screening and AAT replacement therapy are available and should be considered in young emphysema patients.

**Symptom Progression**: Early COPD is often asymptomatic or subtle. As disease progresses, patients develop: (1) Dyspnoea—initially with exertion, then at rest; (2) Chronic cough—productive in bronchitis, sparse in emphysema; (3) Sputum production; (4) Wheezing and chest tightness; (5) Fatigue and weight loss; (6) Recurrent respiratory infections. **Physical Examination Findings**: - Vital signs: tachypnoea (respiratory rate >20), tachycardia, possible hypoxemia on pulse oximetry - General appearance: use of accessory muscles (sternocleidomastoid, intercostal), pursed-lip breathing, barrel chest - Chest inspection: increased AP diameter, asymmetrical chest movement (if emphysema with bullae) - Palpation: reduced tactile fremitus (from air trapping), flattened diaphragms - Percussion: hyperresonance (air trapping), diminished diaphragmatic movement - Auscultation: diminished or absent breath sounds (due to air trapping and reduced airflow), wheezing, crackles (from secretions) - Extremities: cyanosis of lips/nailbeds, clubbing (late sign), peripheral edema (cor pulmonale) - Neck: distended jugular veins (cor pulmonale) - Abdomen: hepatomegaly, right upper quadrant tenderness (cor pulmonale) **Diagnostic Testing**: *Spirometry (Gold Standard)*: Confirms airflow obstruction and assesses severity. Findings in COPD: (1) FEV1/FVC ratio <70% (key diagnostic criterion); (2) FEV1 <80% predicted (GOLD classification); (3) Limited or no bronchodilator responsiveness (FEV1 improvement <12% and <200 mL after salbutamol, distinguishing from reversible asthma). COPD severity grading: GOLD 1 (mild) FEV1 ≥80% predicted; GOLD 2 (moderate) FEV1 50–79%; GOLD 3 (severe) FEV1 30–49%; GOLD 4 (very severe) FEV1 <30%. *Chest X-Ray*: (1) Hyperinflation—increased lucency, flattened diaphragms, increased retrosternal air space; (2) Bullae in emphysema; (3) Increased AP diameter; (4) Attenuation of peripheral vascular markings. CXR may appear relatively normal in early-to-moderate COPD. *Arterial Blood Gas (ABG)*: Reflects chronic respiratory acidosis with metabolic compensation in advanced COPD: (1) Elevated PaCO2 (>45 mmHg); (2) Elevated HCO3- (>26 mEq/L)—renal compensation; (3) pH 7.35–7.45 (near-normal despite elevated CO2); (4) Low PaO2 (<75 mmHg on room air). In mild-to-moderate COPD, ABG may be normal at rest but abnormal with exercise. *Pulse Oximetry*: SpO2 typically <90% on room air in moderate-to-severe COPD; desaturation with exertion is common. *Additional Diagnostics*: (1) High-resolution CT (HRCT)—assesses emphysema distribution, detects bronchiectasis, and may reveal alternative diagnoses; (2) Diffusing capacity (DLCO)—reduced in emphysema, normal or mildly reduced in chronic bronchitis; (3) Alpha-1 antitrypsin level—screening indicated in young patients (<45 years) or non-smokers; (4) Electrocardiogram (ECG)—may show right axis deviation, right ventricular hypertrophy if cor pulmonale; (5) Echocardiography—assesses right ventricular function and pulmonary hypertension.

**Airway and Oxygenation Management—The Critical Safety Principle**: The single most important safety consideration in COPD nursing is the principle of CONTROLLED OXYGEN THERAPY. Unlike healthy individuals, many COPD patients (especially 'blue bloaters' with chronic CO2 retention) depend on **hypoxic drive** for ventilation. Their respiratory centers are desensitized to elevated CO2; instead, low oxygen levels drive them to breathe. If high-flow oxygen is administered indiscriminately, PaO2 rises, the hypoxic drive is eliminated, ventilation drops, and PaCO2 rises dangerously—a condition called **CO2 narcosis** or **hypercapnic respiratory failure**. **CRITICAL OXYGEN TITRATION PROTOCOL**: - **TARGET SpO2: 88–92%** (NOT the standard 95–100%) - Use LOW-FLOW oxygen devices: nasal cannula at 1–2 L/min - **PREFERRED: Venturi mask (or Venti-mask)**, which delivers precise, controlled FiO2 (24%, 28%, 31%, 35%), ideal for CO2 retainers - Monitor ABG or venous blood gas (VBG) before and 30–60 minutes after starting oxygen to assess response and ensure PaCO2 does not rise excessively - NEVER withhold oxygen from a hypoxic patient out of fear of suppressing ventilation; titrate carefully and monitor continuously - Educate the patient and family: 'More oxygen is NOT always better; we give you JUST ENOUGH to keep you comfortable and keep your blood oxygen safe.' **Positioning and Breathing Techniques**: - **High-Fowler's position** (bed head 80–90 degrees) or leaning forward on an overbed table (tripod position) eases the work of breathing by allowing gravity to assist diaphragmatic excursion and reducing the mechanical disadvantage from flattened diaphragms. - **Pursed-lip breathing (PLB)**: Teach the patient to inhale slowly through the nose for a count of 2, then exhale slowly through pursed lips (as if whistling) for a count of 4. This creates back-pressure in the airways, preventing early collapse during expiration, reducing air trapping, and slowing the respiratory rate. PLB reduces dyspnoea and is a fundamental self-management tool. - **Diaphragmatic (abdominal) breathing**: Teach the patient to breathe using the diaphragm rather than accessory muscles. Place one hand on the chest and one on the abdomen; as the patient breathes in, the abdominal hand should move outward. This is more efficient and less fatiguing than accessory muscle use. **Airway Clearance**: - **Hydration**: Adequate fluid intake (typically 2–3 L/day, unless contraindicated) thins secretions and facilitates expectoration. - **Controlled coughing technique**: Instruct the patient to take 2–3 deep breaths, then cough forcefully twice—the first cough dislodges mucus, the second expels it. This is more effective than uncontrolled coughing and reduces dyspnoea. - **Chest physiotherapy and postural drainage**: In patients with copious sputum (especially bronchiectasis-overlap), position the patient to allow gravity to drain specific lung lobes, followed by gentle chest percussion or vibration. Coordinate with bronchodilator inhalation or systemic mucolytics (e.g., N-acetylcysteine) for maximum efficacy. - **Nebulised bronchodilators and mucolytics**: Short-acting beta-2 agonists (e.g., salbutamol) before chest therapy enhance airway clearance. **Activity and Energy Conservation**: - **Pacing and activity planning**: Teach the patient to plan activities, take rest breaks, and avoid rushing. Dyspnoea is exacerbated by panic and anxiety; slow, controlled activity reduces oxygen demands. - **Small, frequent, high-calorie meals**: Large meals distend the stomach and push the diaphragm upward, worsening dyspnoea. Multiple small meals (5–6 per day) with calorie-dense foods (nuts, peanut butter, whole milk, avocado) maintain nutrition without exacerbating dyspnoea. Monitor weight; cachexia from increased metabolic demands is common and worsens prognosis. - **Energy-saving techniques**: Sit while performing activities; use lightweight tools; simplify tasks; prioritize essential activities. **Infection Prevention**: - **Vaccination**: Annual influenza vaccine and pneumococcal vaccination (PPSV23, PCV13/PCV15) are ESSENTIAL; respiratory infections are a major trigger of COPD exacerbations. - **Infection recognition and early intervention**: Teach the patient to recognize signs of exacerbation—increased dyspnoea, change in sputum colour (green, brown, bloody), increased sputum volume, fever, chills—and seek medical attention promptly. Early antibiotics can prevent hospital admission. - **Hand hygiene and respiratory etiquette**: Regular handwashing, avoiding crowds during respiratory illness season, and asking visitors with symptoms to wear masks reduce infection risk. - **Smoking cessation**: Emphasize that quitting is the SINGLE MOST EFFECTIVE intervention to slow COPD progression. Offer pharmacotherapy (nicotine replacement, varenicline, bupropion) and behavioral support. Even after diagnosis, quitting provides significant benefit. **Pharmacological Support and Monitoring**: See pharmacology section (below) for detailed drug classes, but nursing responsibilities include: monitoring for side effects (tremor, tachycardia from beta-agonists), ensuring correct inhaler technique, and reinforcing adherence to maintenance therapy. **Psychological and Educational Support**: - COPD is a chronic, progressive condition; many patients experience anxiety and depression. Assess mental health and refer to psychiatric services if needed. - Patient education on disease process, self-management, medication use, and exacerbation prevention is foundational. Enroll patients in pulmonary rehabilitation programs where available (increasingly available through Philippine tertiary hospitals). - Advance care planning: As COPD progresses, discuss goals of care, advance directives, and end-of-life preferences in a sensitive, non-threatening manner. **Nursing Diagnoses (NANDA-I) and Maslow-based Prioritization**: Applying NANDA-I taxonomy and Maslow's hierarchy, key diagnoses in COPD include: 1. **Ineffective airway clearance** (related to excessive mucus, loss of elastic recoil, weakness; evidenced by diminished breath sounds, wheezing, productive cough)—PHYSIOLOGICAL, HIGH PRIORITY 2. **Impaired gas exchange** (related to air trapping, ventilation-perfusion mismatch; evidenced by low PaO2, elevated PaCO2, dyspnoea)—PHYSIOLOGICAL, HIGH PRIORITY 3. **Ineffective breathing pattern** (related to air trapping, accessory muscle fatigue; evidenced by prolonged expiration, use of accessory muscles)—PHYSIOLOGICAL, HIGH PRIORITY 4. **Activity intolerance** (related to dyspnoea, poor gas exchange, muscle deconditioning; evidenced by dyspnoea on exertion, fatigue)—PHYSIOLOGICAL 5. **Imbalanced nutrition: less than body requirements** (related to dyspnoea, early satiety; evidenced by weight loss, reduced intake)—PHYSIOLOGICAL 6. **Anxiety** (related to chronic illness, fear of dyspnoea/death; evidenced by restlessness, worry)—PSYCHOLOGICAL 7. **Deficient knowledge** (related to disease management, medication use, exacerbation prevention)—EDUCATIONAL

**Inhaled Bronchodilators—Relievers (Rescue) and Controllers (Maintenance)**: *Short-Acting Beta-2 Agonists (SABA)*: - **Drug**: Salbutamol (albuterol), terbutaline - **Mechanism**: Beta-2 adrenergic receptor agonists in airway smooth muscle → increased cAMP → bronchodilation - **Use**: Rapid relief of acute bronchospasm; typically MDI or nebuliser, 4–6 hourly as needed - **Side effects**: Tachycardia, tremor, jitteriness, headache, hyperglycemia (in high doses) - **Nursing consideration**: Teach correct MDI or nebuliser technique; use before exertion or chest physiotherapy for maximum benefit *Short-Acting Muscarinic Antagonists (SAMA)*: - **Drug**: Ipratropium (atrovent) - **Mechanism**: Blocks vagal parasympathetic bronchoconstriction → bronchodilation - **Use**: Often combined with SABA (e.g., salbutamol + ipratropium nebuliser) for additive effect; slower onset than SABA but effective - **Side effects**: Dry mouth, urinary retention (in sensitive patients), blurred vision if inadvertently sprayed in eyes - **Nursing consideration**: Educate on proper nebuliser technique; remind not to spray in face *Long-Acting Beta-2 Agonists (LABA)*: - **Drugs**: Salmeterol, formoterol, vilanterol - **Use**: Maintenance therapy (twice daily, e.g., salmeterol q12h), NOT for rescue—slower onset than SABA - **Advantage**: Sustained bronchodilation reduces symptoms and exacerbations - **CRITICAL SAFETY**: LABA should NEVER be used as monotherapy in COPD or asthma; must be combined with inhaled corticosteroid (ICS) due to increased risk of severe asthma/COPD attacks and death if used alone - **Side effects**: Similar to SABA (tachycardia, tremor) *Long-Acting Muscarinic Antagonists (LAMA)*: - **Drugs**: Tiotropium (Spiriva), glycopyrrolate, umeclidinium - **Use**: Once-daily (or twice-daily, depending on formulation) maintenance therapy - **Mechanism**: Long-acting parasympatholytic bronchodilation - **Advantage**: 24-hour bronchodilation, improved FEV1 and exercise capacity, reduced exacerbations - **Side effects**: Dry mouth, urinary retention, constipation - **Nursing consideration**: First dose often requires careful patient education; dry mouth can be managed with sugarless candy/lozenges **Inhaled Corticosteroids (ICS)**: - **Drugs**: Fluticasone, beclomethasone, budesonide, ciclesonide, mometasone - **Mechanism**: Reduce airway inflammation, mucus production, and airway remodeling - **Use**: Maintenance therapy in moderate-to-severe COPD, especially in frequent exacerbators (≥2 exacerbations/year) - **Benefit**: Reduce exacerbation frequency, improve FEV1, improve quality of life - **Side effects**: Oral candidiasis (thrush), hoarseness, cough—PREVENTABLE by rinsing the mouth with water and spitting after each use - **CRITICAL NURSING POINT**: After every ICS inhalation, the patient MUST rinse the mouth thoroughly with water and spit out. This single action prevents oral candidiasis and is a core teaching point. - **Systemic absorption**: Minimal with inhaled forms; long-term ICS use does not significantly increase systemic side effects at standard doses **Combination Inhalers**: - **LABA + ICS combinations**: e.g., fluticasone/salmeterol (Seretide), budesonide/formoterol (Symbicort). Convenient, single inhaler containing both agents. LABA in combination is safe (unlike monotherapy) because ICS provides protection against LABA-related adverse events. - **LAMA + LABA combinations**: e.g., umeclidinium/vilanterol (Anoro Ellipta). Dual long-acting bronchodilation without corticosteroid; useful in patients who cannot tolerate ICS. - **Triple therapy**: LAMA + LABA + ICS, e.g., fluticasone/umeclidinium/vilanterol (Trelegy Ellipta). For frequent exacerbators with severe COPD. **Theophylline**: - **Mechanism**: Non-selective phosphodiesterase inhibitor; weak bronchodilator; also has anti-inflammatory and inotropic properties - **Use**: Rarely used now due to narrow therapeutic window and availability of superior agents - **Therapeutic range**: 10–20 mcg/mL; levels >20 cause toxicity - **Side effects**: Tachycardia, arrhythmias, nausea, vomiting, tremor, seizures (toxicity) - **Nursing consideration**: If used, monitor serum levels; interactions with many drugs (ciprofloxacin, cimetidine increase levels); patient should not self-adjust dose; avoid in patients with uncontrolled arrhythmias or seizure disorders **Systemic Corticosteroids**: - **Use**: SHORT-COURSE therapy (typically 5–7 days) during acute exacerbations to reduce airway inflammation and expedite recovery - **Typical regimen**: Prednisone 40–50 mg daily for 5–7 days (do NOT taper after short courses <2 weeks) - **Benefits**: Improve FEV1, reduce recovery time, prevent hospital readmission - **Side effects** (short-term): Hyperglycemia, insomnia, mood changes, increased appetite, dyspepsia - **Nursing considerations**: (1) Give with food; (2) Monitor blood glucose, especially in diabetics; (3) NEVER abruptly stop systemic steroids after prolonged use (>2 weeks) due to risk of adrenal suppression—taper gradually; (4) Short bursts (<2 weeks) do not require tapering **Antibiotics**: - **Indication**: Acute exacerbations with purulent sputum (green, brown, or bloody) suggesting bacterial infection - **Typical agents**: Amoxicillin-clavulanate, fluoroquinolones (levofloxacin, moxifloxacin), macrolides (azithromycin), cephalosporins - **Duration**: Typically 5–7 days - **Nursing consideration**: Teach recognition of signs warranting antibiotics; emphasize completing full course; monitor for side effects (GI upset, C. difficile risk with broad-spectrum agents) **Phosphodiesterase-4 Inhibitors**: - **Drug**: Roflumilast - **Mechanism**: PDE-4 inhibitor in immune and inflammatory cells → reduces inflammation - **Use**: In patients with chronic bronchitis and frequent exacerbations; adjunct to inhaled therapy - **Side effects**: Diarrhea, nausea, weight loss - **Nursing consideration**: Monitor weight; GI side effects often limit use **Mucolytics and Expectorants**: - **N-acetylcysteine (NAC)**: Thins secretions; some anti-inflammatory benefit; nebulised or oral - **Guaifenesin (expectorant)**: In cough syrups; promotes thinning of secretions; evidence of efficacy is modest - **Nursing consideration**: Adequate hydration is typically more effective than pharmacological mucolytics **Inhaler Technique—Critical Nursing Responsibility**: Many patients use inhalers incorrectly, reducing drug delivery by 50–90%. Nursing steps to teach proper MDI technique: 1. Shake the inhaler vigorously 10–15 times 2. Exhale completely to functional residual capacity 3. Place the MDI mouthpiece between the teeth and lips (NOT in the back of the throat) 4. Press down on the canister while simultaneously starting a slow, deep inhalation over 3–5 seconds 5. Hold the breath for 10 seconds (allows distal airway deposition) 6. Exhale slowly 7. Wait 1 minute before the second puff (allows airway relaxation) 8. After use, rinse the mouth (especially critical with ICS) **Spacer devices** significantly improve drug delivery, especially in elderly patients and children. Always recommend a spacer, particularly for ICS. **Nebuliser Use**: - Slower delivery than MDI but may be easier for frail or dyspnoic patients - Treatment time: 10–15 minutes - Educate to breathe normally and deeply during treatment; avoid talking - Clean mouthpiece after use (soak in warm water, air dry) - Check for visible mist; if absent, nebuliser may be malfunctioning

**Acute Exacerbations**: - **Definition**: Sudden worsening of symptoms beyond day-to-day variation, characterized by increased dyspnoea, cough, sputum volume/purulence, and/or fever - **Triggers**: Respiratory infection (most common), air pollution, non-adherence to medications, allergen exposure - **Clinical manifestations**: Increased dyspnoea, change in sputum colour (yellow, green, brown) or volume, wheezing, crackles, possibly fever and fatigue - **Management**: Increase short-acting bronchodilators; add systemic corticosteroids (prednisone 40–50 mg daily × 5–7 days); antibiotics if purulent sputum; oxygen titration to SpO2 88–92%; IV fluids if unable to drink; hospital admission if severe, unable to self-manage, or significant comorbidities - **Nursing role**: Early recognition and intervention dramatically reduce hospital admissions; educate patients on exacerbation signs; maintain outpatient follow-up; reinforce infection prevention **Cor Pulmonale (Right-Sided Heart Failure)**: - **Pathophysiology**: Chronic hypoxemia causes pulmonary vasoconstriction → increased pulmonary vascular resistance → right ventricular strain and hypertrophy → eventual right-ventricular failure - **Manifestations**: Peripheral edema (legs, sacrum), jugular venous distension, hepatomegaly with right upper quadrant tenderness, ascites, weight gain, dyspnoea worsening despite bronchodilators - **Diagnosis**: ECG (right axis deviation, right ventricular hypertrophy), echocardiography (elevated pulmonary artery pressure, dilated right ventricle, tricuspid regurgitation) - **Management**: Optimize COPD therapy (improve oxygenation, reduce pulmonary hypertension); diuretics for fluid overload (cautious use—excess diuresis reduces preload and can worsen CO2 retention); vasodilators (e.g., inhaled nitric oxide, phosphodiesterase-5 inhibitors) in select cases; oxygen therapy (most important intervention) - **Nursing**: Monitor for signs of fluid overload (daily weight, peripheral edema, lung sounds); educate on salt restriction; teach signs of worsening heart failure (increased edema, dyspnoea at rest, orthopnoea) **Secondary Polycythemia**: - **Pathophysiology**: Chronic hypoxemia triggers erythropoietin (EPO) release from kidneys → increased RBC production to maximize oxygen-carrying capacity - **Clinical significance**: Increased blood viscosity → increased risk of thromboembolism (DVT, PE, stroke); worsens dyspnoea paradoxically by increasing cardiac workload - **Management**: Oxygen therapy to reduce hypoxemia and suppress EPO; phlebotomy in symptomatic patients with Hgb >18–19 g/dL (rare) - **Nursing**: Monitor CBC; educate on hydration (prevents blood sludging); watch for signs of thromboembolism; assess for signs of stroke or MI **Pneumothorax**: - **Mechanism**: Rupture of a subpleural bleb (in emphysema) → air enters pleural space → lung collapse - **Risk**: Higher in emphysema-predominant COPD with large bullae - **Presentation**: Acute dyspnoea, chest pain, decreased breath sounds on affected side - **Management**: Oxygen (speeds reabsorption of pleural air), rest; chest tube if large or symptomatic; see Section 11 (Pneumothorax) for detailed management **Respiratory Failure and Need for Mechanical Ventilation**: - **Type II respiratory failure** (hypercapnic): PaCO2 >45 mmHg, pH <7.35; occurs in advanced COPD - **Trigger**: Acute infection, medication non-adherence, excessive oxygen therapy (suppressing hypoxic drive) - **Management**: Non-invasive ventilation (CPAP, BiPAP, NIPPV—"Non-Invasive Positive Pressure Ventilation") is increasingly preferred over intubation in COPD due to risk of ventilator dependence; facilitate discussion on goals of care and advance directives - **Nursing**: Mask fitting and tolerance for NIPPV; sedation support; monitoring of CO2 levels; psychological support for anxious patients **COPD Mortality and Palliative Care**: - COPD is the 3rd–4th leading cause of death globally; many patients die from respiratory failure or cardiopulmonary complications - As COPD progresses, incorporate palliative care principles: focus shifts from cure to comfort, symptom management, and quality of life - **Advance care planning**: Early, sensitive conversations about resuscitation status, mechanical ventilation, and preferred location of death (home vs. facility) honor patient autonomy and reduce inappropriate intensive care - **Opioids for dyspnoea**: Low-dose opioids (morphine) can relieve refractory dyspnoea in end-stage COPD without hastening death if titrated carefully - **Nursing role**: Facilitate goal-setting conversations; ensure symptom comfort; involve family; respect cultural and spiritual preferences

**Definition and Pathophysiology**: Asthma is a chronic inflammatory disorder of the airways characterized by **reversible bronchospasm, airway hyperresponsiveness, and inflammation**, leading to recurrent episodes of wheezing, breathlessness, chest tightness, and cough. Unlike COPD, asthma is fundamentally **reversible**—airway narrowing improves with bronchodilators or resolves spontaneously. **Key Difference from COPD**: Asthma spirometry shows reversible airway obstruction (FEV1/FVC <70%, but improves ≥12% and ≥200 mL after a bronchodilator or with ICS therapy over weeks). COPD shows fixed, irreversible obstruction. **Pathophysiology**: Exposure to a trigger (allergen, viral infection, cold air, exercise, irritant smoke) causes airway smooth muscle contraction, mucosal inflammation and edema, and mucus plugging—all reversible. The underlying inflammation is mediated by Th2 lymphocytes, mast cells, eosinophils, and release of mediators (histamine, leukotrienes, prostaglandins). **Common Triggers**: - Allergens: dust mites, pet dander, pollen, mold - Infections: upper respiratory viral infections (most common trigger in children) - Exercise: exercise-induced asthma (EIA), especially in cold, dry air - Irritants: smoke (cigarette, biomass), air pollution, strong odors - GERD: acid reflux can trigger asthma (common comorbidity) - Aspirin and NSAIDs: in aspirin-sensitive asthma - Emotions: stress, anxiety, laughter, crying - Hormonal: menstrual cycle-related asthma in some women **Clinical Manifestations**: - **Expiratory wheezing** (air traveling through narrowed airways during expiration) - **Dyspnoea** on exertion or at rest - **Cough**—often worse at night, with exertion, or with cold air - **Chest tightness** or pressure - **Prolonged expiration** and use of accessory muscles during attacks - **Wheezing** (may be absent in mild exacerbations or paradoxically in severe obstruction—see 'silent chest,' below) - **Crackles** or rhonchi (from secretions) - **Peak expiratory flow (PEF) reduction** compared to personal best **CRITICAL ASSESSMENT FINDING—'Silent Chest' in Status Asthmaticus**: A severe asthma attack can progress to a point where airways are SO obstructed that **little air is moving, so little or no wheezing is audible**—the chest becomes 'silent.' This is a sign of **severe obstruction and impending respiratory failure**, NOT improvement. A patient with severe dyspnoea, tachypnoea, use of accessory muscles, inability to speak (only 1–2 words per breath), altered mental status, AND an apparently 'silent' chest is in **status asthmaticus** and is a MEDICAL EMERGENCY. Do NOT be falsely reassured by the absence of wheeze. **Diagnosis**: - **Spirometry**: FEV1/FVC <70%; improves ≥12% and ≥200 mL post-bronchodilator or with regular ICS - **Peak Expiratory Flow (PEF)**: Handheld device; <80% of personal best suggests significant obstruction - **Challenge testing** (methacholine or exercise): If spirometry is normal but clinical suspicion is high - **Chest X-ray**: Often normal; rule out other diagnoses (pneumonia, pneumothorax) - **ABG**: In severe attacks, may show initial respiratory alkalosis (low PaCO2 from hyperventilation), progressing to respiratory acidosis if fatigue sets in **Asthma Severity Classification (GINA/NAEPP)**: - **Intermittent**: Symptoms <2 days/week, nocturnal awakenings <2/month, normal FEV1 - **Mild persistent**: Symptoms 2–6 days/week, nocturnal awakenings 2–4/month, FEV1 >80% predicted - **Moderate persistent**: Symptoms daily, nocturnal awakenings >4/month, FEV1 60–80% predicted - **Severe persistent**: Symptoms throughout the day, nocturnal awakenings ≥7/month, FEV1 <60% predicted This classification guides therapeutic intensity: intermittent asthma may need only a reliever; persistent asthma requires a controller (ICS) ± additional agents. **Reliever vs. Controller Therapy (KEY DISTINCTION)**: *Relievers (Rescue Medications)*: - **Short-acting beta-2 agonists (SABA)**: Salbutamol (albuterol) - **Mechanism**: Rapid bronchodilation via beta-2 agonism - **Use**: For acute bronchospasm; inhaled (MDI or nebuliser), onset 5–15 minutes - **Frequency guideline**: If used >2 days/week, indicates inadequate control and need for a controller - **Side effects**: Tremor, tachycardia, jitteriness *Controllers (Preventers)*: - **Inhaled corticosteroids (ICS)**: First-line controller; fluticasone, budesonide, ciclesonide - **Mechanism**: Reduce airway inflammation and hyperresponsiveness - **Dosing**: Low, medium, or high-dose based on severity; twice daily (or once-daily formulations) - **Benefit**: Reduce exacerbations, improve lung function, reduce mortality - **Side effects**: Oral candidiasis (prevent with mouth rinsing), hoarseness, rare systemic effects at high doses - **MUST RINSE MOUTH after every use** - **Timing**: Patient should NOT expect immediate relief; ICS work over days-to-weeks - **Long-acting beta-2 agonists (LABA)**: Salmeterol, formoterol, vilanterol - **CRITICAL**: NEVER use as monotherapy (increases mortality risk); ALWAYS combine with ICS (LABA + ICS) - **Benefit**: 12–24 hour bronchodilation; improve nighttime symptoms and early-morning dips in PEF - **Formulations**: Separate inhalers (patient must remember to take both) or combination inhalers (LABA + ICS in one device, e.g., fluticasone/salmeterol) - **Leukotriene modifiers**: Montelukast (Singulair), zafirlukast - **Mechanism**: Block cysteinyl leukotriene receptors → reduce inflammation and bronchoconstriction - **Use**: Alternative to ICS (less effective) or adjunct in ICS-treated patients - **Advantage**: Oral; especially useful in aspirin-sensitive asthma or exercise-induced asthma - **Rare side effect**: Neuropsychiatric changes (mood changes, suicidality) in rare cases - **Long-acting muscarinic antagonists (LAMA)**: Tiotropium - **Use**: Adjunct in moderate-to-severe asthma inadequately controlled on LABA + ICS - **Mechanism**: Reduce vagal bronchoconstriction - **Theophylline**: Weak controller; rarely used in asthma - **Biologic agents** (for severe eosinophilic asthma): Omalizumab (anti-IgE), mepolizumab (anti-IL-5 for eosinophils), dupilumab (anti-IL-4/IL-13 for Th2 inflammation) - **Use**: Severe asthma inadequately controlled on high-dose ICS + LABA - **Cost**: High; availability limited in resource-limited settings like the Philippines **Acute Severe Asthma Attack (Status Asthmaticus) Management**: 1. **Oxygen**: High-flow to achieve SpO2 ≥95% (asthma patients do not have CO2-retention risk like COPD) 2. **Bronchodilators**: - Nebulised SABA (salbutamol 5 mg) + SAMA (ipratropium 500 mcg) continuously or every 20–30 min × 1–2 hours in first hour - After improvement, reduce frequency 3. **Systemic corticosteroids**: Prednisone 40–50 mg or IV methylprednisolone 125 mg immediately (not delayed) 4. **IV magnesium sulfate**: 2 g IV over 20 min in severe cases with poor response to initial therapy (smooth muscle relaxant, reduces bronchospasm) 5. **Adjuncts**: Consider IV aminophylline or ketamine (sedation, preserves airway tone) in ICU-level attacks 6. **Monitoring**: Continuous pulse oximetry, ECG, ABG (assess for CO2 retention, exhaustion), peak flow 7. **Disposition**: Admit if any sign of severe attack or inadequate response to treatment **Nursing Management**: - **Positioning**: High-Fowler's or leaning forward to ease breathing - **Breathing techniques**: Teach pursed-lip breathing and slow, deep breathing; reassure patient (anxiety worsens bronchospasm) - **Trigger identification and avoidance**: Assess for allergies, exercise-induced patterns, GERD; educate on trigger avoidance - **Inhaler technique**: Critical; teach and demonstrate correct MDI ± spacer use (shake, exhale, place, inhale-press, hold 10 sec, exhale) - **PEF monitoring**: Teach home PEFR measurement; establish personal best; create green-yellow-red zone action plan (green >80% best = all well; yellow 50–80% = caution, increase relievers; red <50% = danger, seek emergency care) - **Action plan**: Written plan for each zone; when to increase controllers, when to use reliever, when to call doctor or go to ER - **Medication adherence**: Many asthmatic patients under-use ICS because they don't feel sick; emphasize that ICS prevents attacks and should be used daily even when well - **GERD management**: If applicable, treat reflux (PPI, H2-blocker) to reduce asthma symptoms - **Exercise program**: Asthma should NOT prevent exercise; teach use of SABA 15 min before exercise; consider adding LTRA or LABA + ICS if EIA persists **Nursing Diagnoses in Asthma**: - **Ineffective airway clearance** (related to bronchospasm, mucus, inflammation) - **Impaired gas exchange** (related to airway obstruction) - **Anxiety** (related to dyspnoea, fear of attacks) - **Deficient knowledge** (about disease management, trigger avoidance, medication use) - **Activity intolerance** (if significant limitation from symptoms or fear of triggering attacks)

**Definition and Pathophysiology**: Bronchiectasis is **permanent, abnormal dilation of the bronchi** resulting from chronic inflammation and infection that destroys the airway wall structure (elastic tissue, cartilage, smooth muscle). Unlike the reversible obstruction in asthma or the air trapping in emphysema, bronchiectasis involves structural bronchial damage. **Causes and Risk Factors**: - **Chronic/recurrent infections**: Most common; untreated or severe lower respiratory infections (including TB, which is highly relevant in the Philippine context) can cause bronchiectasis - **Cystic fibrosis (CF)**: A genetic disorder causing thick, viscous secretions; bronchiectasis is a cardinal feature - **Primary ciliary dyskinesia**: Genetic disorder affecting mucociliary clearance - **Immunodeficiency**: Common variable immunodeficiency, IgA deficiency, HIV/AIDS—impaired clearance of infection - **Allergic bronchopulmonary aspergillosis (ABPA)**: Hypersensitivity to Aspergillus species colonization - **Obstruction**: Tumor, foreign body, or stricture causing distal infection and bronchial damage - **Gastric reflux and aspiration**: GERD, swallowing disorders - **Post-TB**: Sequela of pulmonary tuberculosis, particularly relevant in the Philippines where TB incidence is high **Pathophysiology Cycle**: Initial infection or obstruction → impaired drainage → recurrent infections → chronic inflammation → airway wall destruction and permanent dilation → further stasis and infection → self-perpetuating cycle. Bacteria colonize the dilated airways: Pseudomonas aeruginosa (most common), Haemophilus influenzae, Staphylococcus aureus, others. **Clinical Manifestations**: - **Chronic cough** (hallmark): Productive, often worse in morning or when lying down (due to gravitational drainage of secretions from overnight) - **Copious, purulent sputum** (distinctive finding): Often described as 'three-layer sputum'—upper layer frothy, middle layer serous/purulent, bottom layer sediment (bacteria, cell debris). Volume can be 50–200 mL/day (far more than COPD). - **Foul-smelling sputum**: Due to anaerobic bacteria colonization - **Recurrent respiratory infections and exacerbations** - **Dyspnoea**, especially with exertion - **Hemoptysis**: Coughing blood or blood-tinged sputum from inflamed mucosa or bronchial necrosis - **Chest pain**: Pleuritic pain from pleural inflammation - **Crackles and wheezes** on auscultation - **Clubbing**: Late sign from chronic infection - **Cyanosis and dyspnoea at rest**: In advanced disease - **Symptoms of cor pulmonale**: In severe, long-standing disease **Diagnosis**: - **High-resolution CT (HRCT) chest**: GOLD STANDARD and definitive diagnostic test. Shows: - Bronchial dilation: bronchoarterial ratio >1.0 (bronchus larger than accompanying artery—normally bronchus is smaller) - Lack of bronchial tapering: normal bronchi taper as they become more distal; dilated bronchi do not - Bronchus to pulmonary artery (B/A) ratio measurement - "Tram-track sign": parallel walls of dilated bronchi resembling railroad tracks - "Ring sign": dilated bronchus in cross-section appears as a ring larger than the accompanying artery - Distribution pattern: cylindrical (uniform dilation), varicose (irregular, nodular dilation), or cystic (large, cyst-like) bronchiectasis - **Spirometry**: Often shows airflow obstruction (FEV1/FVC <70%); may have bronchodilator response - **Chest X-ray**: May show hyperinflation, chronic infiltrates, but less sensitive than HRCT - **Sputum microbiology**: Culture and sensitivity guide antibiotic therapy; identifies colonizing organisms - **Immunological workup**: If indicated by clinical context (recurrent infections, young age), assess immunoglobulin levels, complement, T-cell function **Management**: *Airway Clearance (Priority Intervention)*: The cornerstone of bronchiectasis management is **aggressive, daily airway clearance** to reduce sputum burden, prevent infection, and slow disease progression. - **Postural drainage**: Position patient to drain specific lung lobes by gravity, typically 5–15 minutes per position. For example, to drain the lower lobes, patient sits upright leaning forward 30–45 degrees over a pillow. To drain posterior upper lobes, patient lies supine with pillow under hips (Trendelenburg position). Different positions target different lobes. - **Chest physiotherapy**: While patient is in postural drainage position, apply gentle percussion (tapping) over the affected areas using a cupped hand or mechanical percussor, or vibration (oscillatory vibrations during expiration). These techniques mobilize secretions. - **Autogenic drainage**: Patient-directed breathing technique emphasizing expiratory flow without external force; particularly effective in children and for self-management. - **Oscillatory positive expiratory pressure (OPEP) devices**: E.g., Flutter valve, Acapella—create oscillations in airways during expiration, mobilizing secretions. Portable and patient-friendly. - **Intrapulmonary percussive ventilation (IPV)**: Device delivering rapid bursts of air; mobilizes secretions effectively in hospitalized patients or severe cases. - **Hydration**: Adequate fluid intake (2–3 L/day) thins secretions and facilitates expectoration. - **Mucolytics**: Nebulised N-acetylcysteine (NAC, e.g., 3 mL of 20% solution twice daily) reduces sputum viscosity; often combined with bronchodilators immediately before chest physiotherapy for maximum benefit. - **Frequency**: Daily chest physiotherapy (morning and evening) is standard; adjust based on sputum volume and exacerbation frequency *Pharmacological Therapy*: - **Bronchodilators**: SABA ± SAMA (before chest physiotherapy to open airways and facilitate clearance) - **Inhaled corticosteroids**: May reduce inflammation; evidence is mixed, but often used in frequent exacerbators - **Antibiotics**: - **Maintenance antibiotic inhalation therapy**: Nebulised tobramycin, colistin, or azithromycin to suppress Pseudomonas and other pathogens; reduces exacerbations - **Oral antibiotics for exacerbations**: Based on sputum culture and sensitivity; typical agents include fluoroquinolones, cephalosporins, or aminoglycosides for Pseudomonas aeruginosa - **Duration**: Typically 2–3 weeks for exacerbations - **Antiinflammatory agents**: Azithromycin (non-antibiotic, anti-inflammatory dose, e.g., 250 mg three times weekly) reduces exacerbations in some patients - **Antifungals**: If ABPA is suspected *Complications Prevention*: - **Vaccination**: Annual influenza and pneumococcal vaccination - **Pulmonary rehabilitation**: Exercise, breathing education, and psychosocial support - **Nutritional support**: High-calorie diet to maintain nutrition; cachexia is common from chronic infection *Surgical Intervention*: - **Lung resection**: In localized bronchiectasis (e.g., confined to one lobe) with frequent exacerbations despite optimal medical therapy, surgical resection of the affected segment may be considered. Improves quality of life in selected patients **Nursing Management of Bronchiectasis**: - **Teach and supervise airway clearance techniques**: The patient must master postural drainage and percussions; this is often done at home daily. Observe for correct technique; correct errors. Frequency and timing may need adjustment based on sputum volume. - **Coordinate therapy timing**: Administer nebulised bronchodilators and mucolytics 20–30 minutes before chest physiotherapy; arrange staff/family time to assist - **Monitor for signs of infection**: Assess sputum color, volume, odor, fever, and systemic symptoms; ensure prompt antibiotic therapy - **Nutritional counseling**: Recommend calorie-dense foods; monitor weight - **Psychosocial support**: Chronic sputum production is distressing and socially limiting; counseling and support groups help - **Educate on smoking cessation and avoidance of irritants**: Smoke exacerbates symptoms **Nursing Diagnoses in Bronchiectasis**: - **Ineffective airway clearance** (related to airway dilation, excessive sputum, impaired mucociliary clearance)—HIGH PRIORITY - **Chronic respiratory acidosis** (in advanced disease) - **Activity intolerance** (due to dyspnoea and frequent exacerbations) - **Imbalanced nutrition: less than body requirements** (from chronic infection, cachexia) - **Social isolation** (due to cough and sputum production) - **Risk for infection** (colonized airways, impaired defenses)

**Definition and Pathophysiology**: Restrictive pulmonary disease (also called restrictive lung disease or RLD) is characterized by a **reduction in lung volumes and capacities**, with a limitation in lung expansion rather than airway obstruction. The key pathophysiological concept is that the lungs (or chest wall) cannot fully inflate. **Distinction from Obstructive Disease**: - **Obstructive disease** (asthma, COPD, bronchiectasis): Airflow limitation prevents air from exiting the lungs; FEV1/FVC ratio is LOW (<70%) - **Restrictive disease**: Reduced total lung capacity (TLC); lungs cannot fully expand; FEV1/FVC ratio is NORMAL or HIGH (>80%) because both FEV1 and FVC are reduced proportionally. This is the key spirometric pattern distinguishing restriction from obstruction. **Categories and Causes**: *Pulmonary (Intrinsic Lung) Restriction*: - **Idiopathic pulmonary fibrosis (IPF)**: Progressive scarring of lung parenchyma; cause unknown; typically affects older adults (>60 years); median survival ~3–5 years without treatment - **Occupational/environmental lung diseases**: - **Silicosis**: Inhalation of crystalline silica dust (mining, sandblasting, stone cutting); causes nodular pulmonary fibrosis - **Asbestosis**: Asbestos fiber inhalation (construction, shipyards, insulation); causes pleural thickening and fibrosis; increased lung cancer and mesothelioma risk - **Coal worker's pneumoconiosis (CWP)**: Coal dust exposure (mining); causes progressive massive fibrosis - **Talcosis**: Talc dust exposure - **Sarcoidosis**: Systemic granulomatous disease affecting lungs, skin, eyes, lymph nodes; cause unknown; more common in younger adults and African/Asian populations - **Hypersensitivity pneumonitis (HP)**: Immune reaction to inhaled organic antigens (e.g., bird proteins in bird fanciers, moldy hay in farmers); presents as acute (fever, dyspnoea, cough) or chronic (progressive dyspnoea) - **Drug-induced pulmonary fibrosis**: Bleomycin, amiodarone, methotrexate, nitrofurantoin - **Radiation-induced pulmonary fibrosis**: Post-thoracic radiation (e.g., breast cancer treatment) - **Pneumoconiosis from biomass exposure**: Relevant in Philippines—chronic exposure to wood smoke, agricultural dust *Chest Wall/Pleural Restriction*: - **Kyphoscoliosis**: Spinal curvature limiting chest wall expansion; idiopathic or from neuromuscular disease - **Pleural thickening or effusion**: Reduces lung expansion - **Obesity**: Severe obesity restricts chest wall movement *Neuromuscular Restriction*: - **Diaphragmatic paralysis or weakness**: Phrenic nerve injury, muscular dystrophy - **Guillain-Barré syndrome**: Acute paralysis; if severe, can cause acute respiratory failure - **Myasthenia gravis**: Muscle weakness, including respiratory muscles - **Amyotrophic lateral sclerosis (ALS)**: Progressive motor neuron disease; respiratory failure is a major cause of death - **Spinal cord injury**: Paralysis below injury level; high cervical injuries affect diaphragm **Clinical Manifestations**: - **Progressive exertional dyspnoea**: Often the earliest symptom; worsens over months-to-years - **Non-productive (dry) cough**: Irritant cough without sputum (contrast with productive cough in bronchiectasis or bronchitis) - **Dyspnoea at rest**: In advanced disease - **Fatigue and exercise limitation** - **Fine, high-pitched crackles** ('Velcro crackles'): Characteristic of IPF and pulmonary fibrosis; heard at lung bases, end-inspiration. Named for their similarity to opening Velcro—indicates opening of collapsed alveoli - **Clubbing**: Often present in IPF and asbestosis; sign of chronic lung disease - **Cyanosis**: Late finding - **Signs of cor pulmonale**: Dyspnoea, edema, if pulmonary hypertension develops (common in IPF) **Diagnostic Findings**: *Spirometry*: - **Reduced FVC** (forced vital capacity): Total volume exhaled is low - **Reduced FEV1**: Absolute value low, but proportionally reduced equally with FVC - **FEV1/FVC ratio NORMAL or HIGH** (typically >80%; contrast with <70% in obstruction): This is the KEY distinguishing spirometric finding - **Reduced TLC** (total lung capacity) on body plethysmography confirms restrictive pattern *Diffusing Capacity (DLCO)*: - **Reduced in parenchymal disease** (IPF, sarcoidosis): Reflects loss of alveolar-capillary surface area - **Normal in chest wall restriction**: Lungs are structurally normal; volume is just reduced - Important in differential diagnosis *Chest X-Ray and HRCT*: - **IPF**: HRCT shows "usual interstitial pneumonia" (UIP) pattern—irregular, patchy ground-glass opacities and reticular densities, predominantly in lower lobes and periphery - **Silicosis**: Small rounded opacities (nodules) predominantly in upper lobes; progressive massive fibrosis in advanced cases - **Asbestosis**: Bilateral pleural thickening, pleural plaques, restricted lung bases - **Sarcoidosis**: Bilateral hilar lymphadenopathy (classic), nodular infiltrates - **CXR may appear normal in early disease; HRCT is more sensitive** *ABG*: - **Hypoxemia on exertion**: PaO2 drops significantly with exercise (contrast with resting normal in mild disease) - **Mild hypocapnia**: PaCO2 may be low due to hyperventilation attempting to maintain oxygenation - **Normal or increased A-a gradient** (alveolar-arterial oxygen gradient), especially with exercise *Exercise Testing*: - **6-minute walk test (6MWT)**: Objective measure of exercise capacity; desaturation during exercise (<88% SpO2) or significant drop in PaO2 is notable - **Cardiopulmonary exercise testing**: Assesses gas exchange and cardiac response **Management**: *Pulmonary Fibrosis (IPF)*: - **Antifibrotic agents**: - **Pirfenidone**: Reduces FVC decline; mechanism unclear (anti-inflammatory, antifibrotic) - **Nintedanib**: Tyrosine kinase inhibitor targeting TGF-β pathways; reduces disease progression - **Both agents slow decline** (do not reverse fibrosis) and improve progression-free survival and overall survival - **Side effects**: GI upset (nausea, diarrhea), elevated liver enzymes—monitor LFTs - **Cost**: Expensive; availability may be limited in the Philippines - **Corticosteroids and immunosuppressives**: Evidence is weak; no longer routinely used as monotherapy - **Anticoagulation**: Some trials suggest benefit in IPF; anticoagulation practices vary - **Lung transplantation**: For selected, younger patients with advanced disease; limited availability *Occupational/Hypersensitivity*: - **Primary intervention: Remove antigen exposure** (stop mining, stop bird exposure, leave moldy environment) - **Corticosteroids**: Acute hypersensitivity pneumonitis responds well; chronic cases may benefit - **Supportive care**: Oxygen for hypoxemia, pulmonary rehabilitation *Sarcoidosis*: - **Corticosteroids**: First-line; prednisone at varying doses based on organ involvement and severity - **Immunosuppressives**: Azathioprine, methotrexate if steroid-sparing needed - **Monitoring**: Assess for systemic involvement (eyes, heart, kidneys) *Supportive Care (All RLD)*: - **Oxygen therapy**: If PaO2 <60 mmHg at rest or <55 mmHg on exertion; target SpO2 ≥88% at rest, ≥90% on exertion - **Pulmonary rehabilitation**: Exercise, breathing education, psychosocial support improves symptoms and quality of life - **Nutrition**: Maintain adequate nutrition; some fibrotic diseases increase metabolic demands - **Advance care planning**: As disease progresses (especially IPF), discussions about goals of care, mechanical ventilation, and end-of-life preferences are essential **Prognosis**: - **IPF**: Highly variable; median survival ~3–5 years from diagnosis without treatment; antifibrotic agents extend survival by ~9–12 months (significant but limited) - **Occupational lung disease**: Depends on severity of exposure and reversibility; silicosis and asbestosis typically slowly progressive - **Sarcoidosis**: More variable; many patients stable or spontaneous remission; some progress to advanced fibrosis - **Post-TB bronchiectasis with restrictive features**: Variable based on extent of lung destruction **Nursing Management**: - **Oxygen therapy**: Teach home oxygen use; assess need for oxygen during exertion and sleep (nocturnal desaturation is common) - **Energy conservation**: Similar to COPD; plan activities, take rest breaks - **Pulmonary rehabilitation**: Refer for exercise, breathing techniques, and psychosocial support - **Advance care planning**: Facilitate conversations about prognosis, goals of care, and resuscitation preferences; many patients with IPF may prefer comfort-focused care - **Psychological support**: Chronic progressive disease with poor prognosis causes anxiety and depression; assess mental health; provide counseling - **Medication education**: If on antifibrotic agents, teach about monitoring (LFTs), side effects (GI), adherence importance **Nursing Diagnoses in RLD**: - **Impaired gas exchange** (related to reduced lung volume, loss of alveolar-capillary surface area) - **Activity intolerance** (related to hypoxemia, dyspnoea with exertion) - **Ineffective breathing pattern** (related to restricted lung expansion) - **Anxiety** (related to progressive disease, poor prognosis) - **Deficient knowledge** (about disease, oxygen use, lifestyle modifications)

**Definition**: A **pneumothorax** is the presence of **air in the pleural space** (the potential space between the visceral pleura covering the lung and the parietal pleura lining the chest wall). Air entry into this normally closed space disrupts the negative intrapleural pressure, causing the lung to collapse, and impairing gas exchange. **Physiology of Lung Collapse**: Normally, the pleural space is a potential space with a pressure of approximately -5 cm H2O (negative) relative to atmospheric pressure. This negative pressure keeps the lung expanded against the chest wall. When air enters the pleural space (pneumothorax), the pressure increases toward atmospheric (0) or higher. The elastic recoil of the lung, no longer held open by negative pressure, causes the lung to collapse. The degree of collapse is proportional to the volume of air in the pleural space. **Classification and Types**: *Spontaneous Pneumothorax*: - **Primary spontaneous pneumothorax (PSP)**: Occurs in individuals **without underlying lung disease**; caused by rupture of a **subpleural bleb** (small cyst at the lung apex). Most common in tall, thin young men (average age 20–30 years). Pathophysiology: blebs form due to differential ventilation of apical alveoli; rupture occurs, typically during normal activity or with coughing/straining. - **Secondary spontaneous pneumothorax (SSP)**: Occurs in patients **with underlying lung disease**; common causes: - **COPD**: Rupture of bullae (large, emphysematous air spaces) - **Cystic fibrosis**: Blebs and bullae common - **Pulmonary fibrosis**: Subpleural cyst rupture - **TB**: Cavitary disease rupturing into pleura - **Sarcoidosis**: Cyst rupture - **Histiocytosis X (Langerhans cell histiocytosis)**: Cyst rupture - **Lymphangioleiomyomatosis (LAM)**: Cyst rupture - In the Philippine context, **post-TB pneumothorax** is notably common given high TB prevalence *Traumatic Pneumothorax*: - **Closed traumatic**: Blunt chest trauma (motor vehicle accident, fall, sports injury) causes lung laceration without skin breach - **Open traumatic (penetrating)**: Penetrating wound to the chest wall (stab, gunshot, impaled object) allows air to enter through the wound and pleural space. If the wound is sufficiently large and remains open, atmospheric air can flow freely in and out, creating a **"sucking" chest wound** or **open pneumothorax**. *Iatrogenic Pneumothorax*: - Complication of medical procedures: central line placement (subclavian), lung biopsy, mechanical ventilation (barotrauma), or aspiration of cysts/bullae *Tension Pneumothorax*: - A **one-way valve mechanism** allows air to enter the pleural space during inspiration but prevents exit during expiration. Pressure progressively increases, compressing the lung, shifting the mediastinum (heart and great vessels) toward the unaffected side, and compromising cardiac output. This is a **LIFE-THREATENING EMERGENCY**. **Clinical Manifestations and Assessment**: *Mild-to-Moderate Pneumothorax*: - **Sudden-onset, sharp, pleuritic chest pain**: Worsens with breathing or coughing; often localized to the affected side - **Dyspnoea**: Mild-to-moderate, worse with exertion - **Tachypnoea and tachycardia** - **Decreased/absent breath sounds on the affected side**: Key finding - **Hyperresonance to percussion on the affected side**: Due to air in pleural space - **Reduced chest expansion on affected side** and possible subcutaneous emphysema (air in subcutaneous tissues, palpable as crepitus) - **Hypoxemia**: Mild (SpO2 usually >90%) if small pneumothorax; more significant with larger collapse *Tension Pneumothorax—CRITICAL FINDINGS*: - **Severe, acute dyspnoea and chest pain** - **Profound distress, anxiety, inability to speak** - **Tachycardia and hypotension**: Cardiovascular compromise from reduced venous return - **Cyanosis** - **Tracheal deviation TOWARD the UNAFFECTED (opposite) side**: This is a cardinal sign; the mediastinal shift is visible on CXR and sometimes palpable - **Distended neck veins (JVD)**: From impeded venous return - **Decreased/absent breath sounds on the affected side**; may have muffled heart sounds - **Subcutaneous emphysema**: Often present - **Altered mental status**: From severe hypoxemia and shock - **Risk of cardiac arrest**: Without intervention, cardiovascular collapse is imminent **Diagnostic Findings**: *Chest X-Ray (CXR)*: - **Pneumothorax**: Lucent (black) area in the pleural space without lung markings; a **lung margin** (visceral pleura edge) is visible, separating the collapsed lung from the air-filled pleural space - **Pneumothorax size assessment**: - **Small (<2 cm at the hilum or <20% volume)**: Often monitored conservatively - **Large (≥2 cm or ≥20% volume)**: Typically requires chest tube drainage - **Tension pneumothorax CXR**: **Tracheal deviation away from pneumothorax**, mediastinal shift, compressed heart, flattened diaphragm on affected side, pushed heart and mediastinum toward opposite side - **CXR should be done in full inspiration; expiration CXR may exaggerate small pneumothorax size** *CT Chest*: - **More sensitive than CXR** for detecting small pneumothorax and detecting multiple blebs/bullae - **Not needed acutely** for diagnosis; useful for evaluating underlying lung disease and planning intervention *ABG*: - **Mild hypoxemia**: PaO2 typically 75–85 mmHg on room air for moderate pneumothorax - **Respiratory alkalosis**: PaCO2 may be low from hyperventilation/anxiety - **Improves rapidly with oxygen and lung re-expansion** **Differential Diagnosis**: Always consider and exclude: - **Acute coronary syndrome**: Can mimic pneumothorax; perform ECG - **Pulmonary embolism**: Also presents with pleuritic pain and dyspnoea - **Pneumomediastinum**: Air in mediastinal tissues (from ruptured esophagus, severe asthma); different distribution on imaging - **Rib fracture/flail chest**: From trauma; pain with palpation and movement **Management**: *Small, Stable Primary Spontaneous Pneumothorax (<2 cm; <20% volume; minimal symptoms)*: - **Observation and supportive care** (with close follow-up): - **Rest**: Activity restriction; avoid strenuous exertion - **High-flow oxygen** (if not contraindicated): 6–8 L/min via face mask or nasal cannula at 100% FiO2 accelerates pleural air reabsorption (air is replaced by oxygen, which is rapidly reabsorbed). Cuts reabsorption time from weeks to days. - **Pain management**: Analgesics as needed - **Outpatient follow-up**: Repeat CXR in 2–4 weeks; monitor for enlargement or symptoms - **Home oxygen** may be prescribed for first week to accelerate absorption - **Cough suppression**: Avoid coughing/straining - **Patient education**: Seek care if dyspnoea worsens, chest pain increases, or new symptoms develop - **Success rate**: ~50% reabsorb spontaneously; ~20% fail observation alone in PSP *Simple (Non-Tension) Pneumothorax, Larger Size or Symptoms*: - **Chest tube (tube thoracostomy)** to closed-drainage system (water-seal drainage): - **Tube placement**: 4th–5th intercostal space, mid-axillary line (lateral, not anterior), with the tip in the apex of pleural space to evacuate air - **Closed drainage system**: Tubes connected to a drainage bottle with a water seal. The water column maintains seal; as the patient breathes in (negative pressure), the tube allows air to exit the pleural space and bubble through the water; as the patient breathes out (positive pressure), the water column prevents backflow of air into the pleural space. - **Suction**: May be applied to the drainage system to accelerate lung re-expansion (typically -10 to -20 cm H2O). - **Monitoring**: Watch for **continuous bubbling in the water-seal chamber**, which indicates an air leak from the lung (expected initially; should resolve as lung seals; if persistent after 3–5 days, may indicate inability of lung to seal—consider surgery) - **Duration**: Chest tube typically remains for 2–3 days after air leak ceases and lung is fully re-expanded; then removed, dressing applied - **Complications of chest tube**: Tube malposition, subcutaneous migration, infection, re-expansion pulmonary edema (if large volume drained too rapidly; see below) *Tension Pneumothorax—EMERGENCY DECOMPRESSION*: - **This is a MEDICAL/SURGICAL EMERGENCY** requiring immediate intervention without waiting for imaging - **Needle decompression (emergency procedure)**: - **Location**: 2nd intercostal space, **midclavicular line** on the affected side (NOT where the chest tube goes; this is anterior for immediate decompression) - **Needle**: Large-bore needle (14–16 gauge); some sources recommend over-the-needle catheter, leaving the catheter in place and removing the needle - **Technique**: After skin prep and local anesthesia if time permits, insert needle perpendicular to chest, aiming just above the 3rd rib (to avoid vessel/nerve running below each rib). Push through parietal pleura; a sudden gush of air confirms tension pneumothorax. - **Immediate effect**: Air exits, pressure decreases, patient dyspnoea often improves dramatically, heart rate decreases - **Definitive treatment**: After needle decompression, **immediate chest tube insertion** (5–6 weeks later in ICU) is required; needle decompression is temporary bridge to definitive care - **Never delay needle decompression waiting for imaging** in suspected tension pneumothorax *Traumatic Pneumothorax*: - **Closed traumatic**: Usually managed like primary spontaneous (observation for small, or chest tube for large); watch for hemopneumothorax (air + blood). - **Open pneumothorax (sucking chest wound)**: - **IMMEDIATE management**: 1. **Occlusive dressing**: Apply an impermeable (vaseline-gauze, plastic wrap) dressing **taped on three sides only**. Taping three sides creates a **flutter valve**: the dressing allows air trapped in the pleural space to escape during expiration (when intrapleural pressure is positive), but the dressing seals and prevents air from entering during inspiration (when intrapleural pressure is negative). This prevents tension pneumothorax while allowing air evacuation. 2. **CRITICAL**: Do NOT tape all four sides (completely sealed). A fully sealed dressing traps air and can convert an open pneumothorax to a tension pneumothorax. 3. **Position**: High-Fowler's 4. **Oxygen**: High-flow 5. **Chest tube**: Insert to definitive drainage (distinct from the open wound) 6. **Wound care**: After stabilization, inspect for foreign bodies, evaluate for need of surgical exploration/closure 7. **Tetanus prophylaxis**: If indicated **Complications**: *Re-expansion Pulmonary Edema*: - **Mechanism**: When a large collapsed lung is rapidly re-expanded (especially if large volume of air is drained quickly from the pleural space), pulmonary capillaries dilate dramatically and become permeable → fluid leaks into alveoli → pulmonary edema - **Prevention**: Limit suction pressure; drain no more than 1 L in the first hour, then slow drainage - **Manifestations**: Dyspnoea, cough, pink frothy sputum, hypoxemia, crackles - **Management**: Stop chest tube drainage, position upright, oxygen, diuretics, consider steroids; usually self-limited over 24–48 hours *Recurrence*: - **Primary spontaneous**: Recurrence rate ~20–50% (varies by series); risk increases with each recurrence - **Secondary spontaneous**: Higher recurrence rate (~40–50%) - **Tertiary recurrence or bilateral pneumothorax**: Indications for surgical pleurodesis or pleurectomy to prevent future recurrence *Hemopneumothorax*: - Air + blood from traumatic injury; chest tube drains both; monitor for hemorrhage *Subcutaneous/Mediastinal Emphysema*: - Air dissects into soft tissues or mediastinum; usually self-limited; watch for signs of mediastinal compression (cardiac tamponade physiology) *Respiratory Failure and Cardiac Compromise*: - Especially with tension physiology; can result in cardiac arrest **Nursing Management of Pneumothorax**: *Assessment and Monitoring*: - Assess for signs of tension pneumothorax urgently; alert physician immediately if tracheal deviation, JVD, hypotension, or severe distress - Monitor vital signs, respiratory status (rate, depth, SpO2), oxygen saturation continuously - Assess pain and anxiety; provide analgesia and reassurance - Check chest tube drainage system (water-seal, suction level, bubbling, dressing integrity) every hour or per protocol *Chest Tube Care*: - **Secure the tube** with a dressing to prevent dislodgement - **Monitor drainage**: Note color, amount, and consistency (serous, serosanguinous, bloody) - **Assess for air leak**: Bubbling in the water-seal chamber during expiration is expected initially; persistent bubbling suggests ongoing air leak (monitor daily; if persistent after several days, notify physician) - **Ensure patency**: Gently palpate the tube to detect kinks; never milk/strip the tube (this can create excessive negative pressure and harm the lung) - **Positioning**: Keep drainage system **below the level of the patient** (gravity-dependent drainage); secure tubing to prevent pulling or kinking - **Clamping**: Never clamp a chest tube without a specific clinical reason (e.g., brief removal for dressing change). Clamping traps air and can cause tension pneumothorax. - **Ambulation**: Patient can walk with chest tube in place once stable; ensure tubing is secured and drainage system is portable *Oxygen Therapy*: - High-flow oxygen (6–8 L/min) accelerates air reabsorption - Target SpO2 ≥94% initially; then titrate as needed *Pain and Comfort*: - Provide analgesia; reassure patient that breathing deeply (which may worsen initial pain) helps re-expand lung - Position in high-Fowler's to ease breathing - Teach splinting (holding a pillow to chest) during coughing to reduce pain *Education*: - Explain pneumothorax and treatment plan in simple terms - Teach signs of complication (increased dyspnoea, severe chest pain, fever, chills) and when to seek care - For patients with primary spontaneous pneumothorax, discuss activity restrictions and when safe to resume normal activity - For recurrent pneumothorax, discuss surgical options (pleurodesis, pleurectomy) to prevent future episodes - Smoking cessation counseling (smoking may increase recurrence risk) *Chest Tube Removal*: - After imaging confirms lung re-expansion and air leak has ceased (typically 2–3 days), tube is ready for removal - Physician removes tube; patient takes a deep breath as tube is pulled to prevent air entry - Airtight dressing applied immediately - Post-removal: Monitor for signs of recurrence (dyspnoea, chest pain); recurrence can occur shortly after removal or weeks later - Follow-up CXR in 24 hours to confirm no recurrence **Nursing Diagnoses**: - **Ineffective breathing pattern** (related to lung collapse, air in pleural space) - **Impaired gas exchange** (related to collapsed lung) - **Acute pain** (related to pleurisy, underlying trauma if present) - **Anxiety** (related to acute dyspnoea, severity of condition) - **Risk for further lung collapse** (if pneumothorax recurs) - **Deficient knowledge** (about condition, treatment, prevention of recurrence)