The document reviews cystic fibrosis (CF) lung disease, detailing its pathophysiology, chronic therapies, and the importance of tracking lung function through FEV1 measurements. Key treatments discussed include hypertonic saline, inhaled mannitol, DNase, and antibiotics, alongside airway clearance methods like chest physiotherapy and exercise. Emphasis is placed on regular monitoring and maintaining a healthy weight to manage the progressive nature of CF lung disease.

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