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

1. A Review of CF Lung Disease
Seth Walker, MD
Director, Emory Adult CF Program

3. Outline
 Pathophysiology of lung disease (how things go
wrong)
 Chronic therapies
 Tracking lung disease
 Chronic infection and sputum cultures

4. Pathophysiology of CF lung disease

5. Airway surface liquid

6. Airway surface liquid

7. Early lung disease
Asymptomatic 3 month old
7 year old, FEV1 96%

8. 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

9. 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

10. rhDNAse (Pulmozyme)
 Breaks up DNA from dead white blood cells
 Thins mucus and improves clearance
 2.5 mg nebulized once daily

11. Azithromycin
 Impairs neutrophils’ (white blood cells) ability to
cause inflammation
 500 mg pill three times a week

12. Inhaled antibiotics
 Aztreonam (Cayston), tobramycin (TOBI), colistin
 All seem effective
 Little head-to-head data

13. Augmented airway clearance
 Chest physiotherapy with postural drainage (CPT)
 Percussive vest
 Positive expiratory pressure (PEP) with oscillation
 Acapella, flutter device
 Autogenic drainage
 Exercise

14. 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

15. Preventing exacerbations
 Greatest benefit of all chronic therapies?
 Lung function more preserved with less exacerbations

16. Measuring lung function

17. 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%)

18. 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

19. 120
100
80
FEV1 60 Patient 1
Patient 2
40
20
0
Time

20. Patient 1
120
100
80
FEV1
60
Patient 1
40
20
0
Time

21. 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

23. Rogers et al, J Clin Microbiol 2004

25. So why still check a culture?
 Information overload
 Surveillance
 Pseudomonal eradication
 Culture and sensitivities can increase our chance of
resolving exacerbations

26. 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