Breathe Better 2012


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The recent presentation by Dr Seth Walker, of the adult CF clinic given at the last Family Advisory Council respiratory event.

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Breathe Better 2012

  1. 1. A Review of CF Lung Disease Seth Walker, MDDirector, Emory Adult CF Program
  2. 2. Outline Pathophysiology of lung disease (how things go wrong) Chronic therapies Tracking lung disease Chronic infection and sputum cultures
  3. 3. Pathophysiology of CF lung disease
  4. 4. Airway surface liquid
  5. 5. Airway surface liquid
  6. 6. Early lung diseaseAsymptomatic 3 month old 7 year old, FEV1 96%
  7. 7. Hypertonic saline Works to rehydrate mucus and restore airway surface liquid Effect lasts 6-7 hours Studied dose is 7% saline 4 ml nebulized twice daily
  8. 8. Inhaled mannitol (Bronchitol) Works similarly to hypertonic saline Dry powder inhaler 400 mg twice daily (but comes in 40-50 mg capsules) Can cause wheezing and bronchoconstriction Not yet available in the US
  9. 9. rhDNAse (Pulmozyme) Breaks up DNA from dead white blood cells Thins mucus and improves clearance 2.5 mg nebulized once daily
  10. 10. Azithromycin Impairs neutrophils’ (white blood cells) ability to cause inflammation 500 mg pill three times a week
  11. 11. Inhaled antibiotics Aztreonam (Cayston), tobramycin (TOBI), colistin All seem effective Little head-to-head data
  12. 12. Augmented airway clearance Chest physiotherapy with postural drainage (CPT) Percussive vest Positive expiratory pressure (PEP) with oscillation  Acapella, flutter device Autogenic drainage Exercise
  13. 13. Augmented airway clearance Significant evidence that chest physiotherapy decreases exacerbations and improves/maintains lung function Small studies show similar efficacy among CPT and vest Exercise does not seem effective as airway clearance by itself
  14. 14. Preventing exacerbations Greatest benefit of all chronic therapies? Lung function more preserved with less exacerbations
  15. 15. Measuring lung function
  16. 16. FEV1 Reliable Varies up to 10% in people without lung disease Peaks at age 25 years Differs based on age, gender, ethnicity, and height Classifies CF lung disease severity- mild (>70% predicted), moderate (40-70%), and severe (<40%)
  17. 17. What should FEV1 be? The highest physiologically possible? (One patient has had FEV1 148% predicted- should this be the standard for everyone?) Greater than 80% predicted? Greater than 70% predicted? The highest possible value for the longest possible time
  18. 18. 120 100 80FEV1 60 Patient 1 Patient 2 40 20 0 Time
  19. 19. Patient 1 120 100 80FEV1 60 Patient 1 40 20 0 Time
  20. 20. Risk factors for FEV1 decline Young adulthood Higher FEV1 Higher FEV1 variability Chronic infection (inhaled antibiotics) Lower BMI/faster rate of BMI decline Male sex
  21. 21. Rogers et al, J Clin Microbiol 2004
  22. 22. So why still check a culture? Information overload Surveillance Pseudomonal eradication Culture and sensitivities can increase our chance of resolving exacerbations
  23. 23. Take Home Points CF has progressive lung disease Staying healthy is good Doing aerosols and airway clearance regularly and maintaining healthy weight keep you healthy FEV1 trend is more important than number itself The airways are teeming with bacteria Sputum culture gives only a hint of this, but can be useful