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Pulmonary Function Testing in Cystic Fibrosis

Pulmonary Function Testing in Cystic Fibrosis. Ramachandra R. Sista, MD Division of Pulmonary & Critical Care Medicine Stanford University. Cystic Fibrosis – Multisystem disorder. CFTR [Ion Channel] ‘Mutations’ Defective epithelial salt and fluid transport 1 in 2-3K live births in US.

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Pulmonary Function Testing in Cystic Fibrosis

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  1. Pulmonary Function Testing in Cystic Fibrosis Ramachandra R. Sista, MD Division of Pulmonary & Critical Care Medicine Stanford University

  2. Cystic Fibrosis – Multisystem disorder • CFTR [Ion Channel] • ‘Mutations’ • Defective epithelial salt and fluid transport • 1 in 2-3K live births in US

  3. 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 Cystic Fibrosis - Airways

  4. CF – Airways

  5. Cystic Fibrosis – Lung Function

  6. Respiratory Pump Thorax Diaphragm Abdomen Abdomen

  7. Airways [Trachobronchial tree]

  8. Gas Exchange [Functional units]

  9. Pulmonary Function Testing [PFT] • Spirometry (Flow Volume Loop) ± BDR • Lung volumes • Diffusion capacity (DLCO)

  10. 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 Spirometry

  11. Monitoring of spirometry – simple and quick • Early recognition and quick intervention for • pulmonary decline / acute exacerbation • Minimize loss of lung function Spirometry

  12. 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] Impact of Respiratory Pathogens

  13. Impact of Respiratory Pathogens

  14. 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 Impact of Airway therapies

  15. Hypertonic saline

  16. Anti Inflammatory Agents

  17. Very important part of successful CF management • Aggressive management of CFRD • Neutrophil dysfunction • Associated with accelerated loss of lung function • Increased mortality risk CFRD, Nutrition and Lung function

  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. CFRD – Glucose Control – FEV1 Semin Respir Crit Care Med 2007

  20. 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 Cystic Fibrosis - Cornerstones

  21. 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 Cystic Fibrosis - Cornerstones

  22. David Weill, MD Pediatric Pulmonologists Gundeep Dhillon, MD Paul Mohabir, MD Kathy Gesley Nicole Eden Lara Freet Lesley Seeger CF Clinical Research Team Acknowledgement

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