Pulmonary Function Testing

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Pulmonary Function Testing

  1. 1. Pulmonary Function Testing in Cystic Fibrosis Ramachandra R. Sista, MD Division of Pulmonary & Critical Care Medicine Stanford University
  2. 2. Cystic Fibrosis – Multisystem disorder CFTR [Ion Channel] ‘Mutations’ • Defective epithelial salt and fluid transport • 1 in 2-3K live births in US
  3. 3. Cystic Fibrosis - Airways Respiratory Tract/Sinus disease • Chronic airway Inflammation • Colonization by bacteria • Rhinosinusitis • Nasal polyps • Bronchitis/bronchiectasis • Hemoptysis • Productive cough • Copious sputum production/secretions • Lung hyperinflation • PFT evidence for obstructive ventilatory limitation
  4. 4. CF – Airways
  5. 5. Cystic Fibrosis – Lung Function
  6. 6. Respiratory Pump Thorax Diaphragm Abdomen Abdomen
  7. 7. Airways [Trachobronchial tree]
  8. 8. Gas Exchange [Functional units]
  9. 9. Pulmonary Function Testing [PFT] • Spirometry (Flow Volume Loop) ± BDR • Lung volumes • Diffusion capacity (DLCO)
  10. 10. Spirometry Simple, reproducible and office-based • How much of air can you move? - in & out - [vital capacity] • How fast can you move? - in & out - [flow / time] • Based on these two - FVC and FEV1
  11. 11. Spirometry • Monitoring of spirometry – simple and quick • Early recognition and quick intervention for pulmonary decline / acute exacerbation • Minimize loss of lung function
  12. 12. Impact of Respiratory Pathogens • Staph (MRSA, MSSA) • Pseudomonas (mucoid, non-mucoid, MDR) • Haemophilus influenza • Stenotrophamonas maltophilia • Burkholderia cepacia • Achromobacter xylocans • Non-tuberculous mycobacteria [NTM – eg. MAC] • Aspergillus species [ABPA]
  13. 13. Impact of Respiratory Pathogens
  14. 14. Impact of Airway therapies Pulmonary therapies • Antibiotics (PO, IV, IH) • Macrolide therapy (anti-inflammatory/neutrophil) • Bronchodilators • Treatment of ABPA • DNAase (Dornase, Pulmozyme) • Hypertonic saline • N-acetylcysteine • Chest physiotherapy • Exercise
  15. 15. Hypertonic saline
  16. 16. Anti Inflammatory Agents
  17. 17. CFRD, Nutrition and Lung function • Very important part of successful CF management • Aggressive management of CFRD • Neutrophil dysfunction • Associated with accelerated loss of lung function • Increased mortality risk
  18. 18. Nutrition and BMI FEV1 percent is positively correlated with BMI percentile for patients 20 to 40 years of age (correlation = 0.92, p < 0.0001)
  19. 19. CFRD – Glucose Control – FEV1 Semin Respir Crit Care Med 2007
  20. 20. Cystic Fibrosis - Cornerstones • Airway clearance • Dornase alfa (2.5 mg nebulized daily) • Nebulized antibiotics • Oral azithromycin • Inhaled hypertonic saline [7% 2-4 times per day] • Aggressive antibiotic therapy for exacerbations • Nutritional support (high-caloric, high-salt diet) • Replacement of fat-soluble vitamins [A, D, E, K] • Exercise
  21. 21. Cystic Fibrosis - Cornerstones • Airway clearance • Dornase alfa (2.5 mg nebulized daily) • Nebulized antibiotics • Oral azithromycin • Inhaled hypertonic saline [7% 2-4 times per day] • Aggressive antibiotic therapy for exacerbations • Nutritional support (high-caloric, high-salt diet) • Replacement of fat-soluble vitamins [A, D, E, K] • Exercise
  22. 22. Acknowledgement David Weill, MD Pediatric Pulmonologists Gundeep Dhillon, MD Paul Mohabir, MD Kathy Gesley Nicole Eden Lara Freet Lesley Seeger CF Clinical Research Team

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