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
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
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
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