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 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 Pediatric Pulmonologists Gundeep Dhillon, MDPaul Mohabir, MD

Kathy GesleyNicole Eden

Lara Freet

Lesley Seeger

CF Clinical Research Team