What are the signs and symptoms, etiology, chronicity, prognosis, medical interventions, lab tests, diagnostics, nursing interventions, and evidence-based practice for Bronchopulmonary Dysplasia (BPD) with chronic lung disease?

Bronchopulmonary Dysplasia (BPD) with Chronic Lung Disease: Comprehensive Management

Pulmonary rehabilitation is the cornerstone of management for patients with BPD-associated chronic lung disease, as it improves exercise performance, reduces breathlessness, and should be implemented for all symptomatic patients. 1

Signs and Symptoms

Respiratory Manifestations

• Chronic and progressive dyspnea (most characteristic symptom)
• Chronic cough (often first symptom)
• Sputum production (may indicate underlying bronchiectasis if large volumes)
• Wheezing and chest tightness (variable throughout the day)
• Recurrent respiratory infections
• Reduced exercise tolerance

Advanced Disease Features

• Fatigue
• Weight loss and anorexia
• Signs of pulmonary hypertension (in approximately one-third of infants with moderate to severe BPD) 2
• Physical signs of airflow limitation/hyperinflation (usually only identifiable with significantly impaired lung function) 1

Etiology

BPD develops primarily in:

• Premature infants requiring mechanical ventilation
• Two distinct forms:
  1. "Old" BPD: Associated with oxidative damage and volutrauma from perinatal mechanical ventilation
  2. "New" BPD: Results from interrupted growth of the lung at earlier stages of fetal development 3

Risk Factors

• Prematurity (especially extremely low gestational age newborns)
• Respiratory distress syndrome requiring treatment
• Mechanical ventilation
• Oxygen therapy
• Genetic predisposition
• Prenatal and postnatal infections 4

Chronicity and Prognosis

BPD is a chronic inflammatory lung disease with:

• Progressive nature with varying severity (mild, moderate, severe)
• Long-term respiratory sequelae extending into adulthood
• Three major patterns in adult survivors:
  1. Asthma-like disease
  2. Obstructive lung disease
  3. Pulmonary hypertension 3

Prognosis Factors

• Pulmonary hypertension significantly increases morbidity and mortality 2
• Long-term oxygen therapy prolongs life in hypoxemic patients 5
• Severity of BPD correlates with long-term outcomes
• Multidisciplinary approach for children with established severe BPD after the neonatal period into adulthood improves outcomes 6

Medical Interventions

Pharmacological Management

• Short-acting bronchodilators (β2-agonists or anticholinergics) as needed for symptoms
• Long-acting bronchodilators:
  • LAMA/LABA combination for persistent symptoms
  • LAMA monotherapy for preventing exacerbations 5
• Diuretics: Natural weaning by relative decrease in dose with increasing weight gain 6
• Oxygen therapy: Supplemental oxygen with saturation target range of 90-95% 6
• Avoid inhaled or systemic corticosteroids for long-term management 6

Surgical Interventions

• Consider for recurrent pneumothoraces or isolated bullous disease
• Lung volume reduction in selected patients
• Lung transplantation for end-stage disease in patients <65 years with:
  • Very poor exercise tolerance
  • Poor lung function (FEV1 <25% predicted)
  • PaO₂ <7.5 kPa and PaCO₂ >6.5 kPa 5

Labs, Diagnostics and Studies

Essential Diagnostics

• Spirometry: Required to establish diagnosis and monitor disease progression
  • Post-bronchodilator FEV1/FVC <0.70 confirms persistent airflow limitation 1
  • Regular lung function monitoring recommended for all children with BPD 6

Classification Based on Spirometry

• Mild: FEV1 >80% predicted
• Moderate: FEV1 50-80% predicted
• Severe: FEV1 30-50% predicted
• Very Severe: FEV1 <30% predicted 5

Additional Studies

• Lung imaging using ionizing radiation only in subgroups (severe BPD or recurrent hospitalizations) 6
• Echocardiography to assess for pulmonary hypertension
• Blood gas analysis to monitor oxygenation and ventilation
• Biomarkers in biological fluids (research ongoing for early diagnosis) 7

Nursing Interventions

• Monitor respiratory status: Rate, effort, oxygen saturation
• Administer prescribed medications and oxygen therapy
• Provide chest physiotherapy and airway clearance techniques
• Educate on proper inhaler technique using "teach-back" approach 5
• Monitor growth and nutrition status
• Prevent respiratory infections:
  • Hand hygiene
  • Vaccination status
  • Individual advice regarding daycare attendance 6
• Support families in home care management
• Coordinate multidisciplinary care

Evidence-Based Practice

• Annual influenza vaccination for all patients 5
• Pneumococcal vaccination recommended 5
• Smoking cessation crucial at all disease stages 5
• Pulmonary rehabilitation improves exercise performance and reduces breathlessness 1
• Multidisciplinary approach for severe BPD from neonatal period into adulthood 6
• Careful timing of pulmonary rehabilitation initiation (not immediately before hospital discharge) 5
• Advance care planning to reduce anxiety and avoid unwanted invasive therapies 5

Emerging Therapies

• Recombinant human superoxide dismutase shows promise in reducing respiratory injury and long-term effects 4
• Bronchoscopic lung volume reduction (BLVR) for select patients with severe disease 5

Key Pitfalls and Caveats

• Poor inhaler technique correlates with poor symptom control; risk factors include older age, multiple devices, and lack of education 5
• Pulmonary hypertension responds suboptimally to pulmonary vasodilators and requires specialized management 2
• Transition from pediatric to adult pulmonary care requires careful planning and coordination 3
• BPD may be difficult to diagnose due to overlapping symptoms with other diseases like pulmonary hypertension 7
• Initiating pulmonary rehabilitation before hospital discharge may compromise survival 5