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
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
CF – Airways
Cystic Fibrosis – Lung Function
Respiratory Pump
Thorax
Diaphragm
Abdomen
Abdomen
Airways [Trachobronchial tree]
Gas Exchange [Functional units]
Pulmonary Function Testing [PFT]
• Spirometry (Flow Volume Loop) ± BDR
• Lung volumes
• Diffusion capacity (DLCO)
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
Spirometry
• Monitoring of spirometry – simple and quick
• Early recognition and quick intervention for
pulmonary decline / acute exacerbation
• Minimize loss of lung function
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]
Impact of Respiratory Pathogens
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
Hypertonic saline
Anti Inflammatory Agents
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
Nutrition and BMI
FEV1 Percent Predicted vs. BMI
in Adults 20 to 40 Years
90
FEV1 Percent Predicted
80
Males
70
60
50
40
30
15
16
17
18
19
20
21
22
BMI
23
24
25
26
27
28
29
30
FEV1 percent is positively correlated with BMI percentile for patients
20 to 40 years of age (correlation = 0.92, p < 0.0001)
CFRD – Glucose Control – FEV1
Semin Respir Crit Care Med 2007
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
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
Acknowledgement
David Weill, MD
Gundeep Dhillon, MD
Paul Mohabir, MD
Kathy Gesley
Nicole Eden
Lara Freet
Lesley Seeger
CF Clinical Research Team
Pediatric Pulmonologists