1. A Review of CF Lung Disease Seth Walker, MDDirector, Emory Adult CF Program 2. Outline Pathophysiology of lung disease (how things gowrong) Chronic therapies Tracking lung disease Chronic infection and sputum cultures 3. Pathophysiology of CF lung disease 4. Airway surface liquid 5. Airway surface liquid 6. Early lung diseaseAsymptomatic 3 month old 7 year old, FEV1 96% 7. Hypertonic saline Works to rehydrate mucus and restore airway surfaceliquid Effect lasts 6-7 hours Studied dose is 7% saline 4 ml nebulized twice daily 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. rhDNAse (Pulmozyme) Breaks up DNA from dead white blood cells Thins mucus and improves clearance 2.5 mg nebulized once daily 10. Azithromycin Impairs neutrophils (white blood cells) ability tocause inflammation 500 mg pill three times a week 11. Inhaled antibiotics Aztreonam (Cayston), tobramycin (TOBI), colistin All seem effective Little head-to-head data 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. Augmented airway clearance Significant evidence that chest physiotherapydecreases exacerbations and improves/maintains lungfunction Small studies show similar efficacy among CPT andvest Exercise does not seem effective as airway clearance byitself 14. Preventing exacerbations Greatest benefit of all chronic therapies? Lung function more preserved with less exacerbations 15. Measuring lung function 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 (