Pulmonary Function Testing - The Cystic Fibrosis Center at Stanford

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