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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

Pulmonary Function Testing in Cystic Fibrosis

Ramachandra R. Sista, MD

Division of Pulmonary & Critical Care Medicine

Stanford University

cystic fibrosis multisystem disorder

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

Cystic Fibrosis - Airways

respiratory pump

Respiratory Pump

Thorax

Diaphragm

Abdomen

Abdomen

pulmonary function testing pft

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

spirometry1

Monitoring of spirometry – simple and quick

  • Early recognition and quick intervention for
  • pulmonary decline / acute exacerbation
  • Minimize loss of lung function

Spirometry

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

Impact of Airway therapies

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

CFRD, Nutrition and Lung function

nutrition and bmi

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

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

cystic fibrosis cornerstones1

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

acknowledgement

David Weill, MD Pediatric Pulmonologists Gundeep Dhillon, MD

Paul Mohabir, MD

Kathy Gesley

Nicole Eden

Lara Freet

Lesley Seeger

CF Clinical Research Team

Acknowledgement