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

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. Impact of Respiratory Pathogens

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. Hypertonic saline

16. Anti Inflammatory Agents

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. Nutrition and BMI
FEV1 percent is positively correlated with BMI percentile for patients 20 to 40 years of age (correlation = 0.92, p < )

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

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