Epidemiologic data demonstrate a dramatic improvement in survival for
cystic fibrosis (CF) over the last few decades and projections suggest
that trend will continue. Standard therapy works and should be aggres
sively applied to this patient population. Although the specific thera
pies have evolved over the years, the basic tenets of CF care remain u
nchanged and include antibiotics to control infection, airway clearanc
e, and adequate nutrition. This article focuses on treatment of the pu
lmonary disease and includes a discussion of the following specific co
mponents of a standard therapeutic approach to CF: (1) antibiotics, (2
) airway clearance and exercise, (3) mucolytics, (4) bronchodilators,
(5) oxygen, (6) anti-inflammatory therapies, and (7) nutritional suppo
rt. Judicious application of these therapies coupled with careful moni
toring of pulmonary, nutritional, and metabolic parameters results in
most CF patients surviving into adulthood with an acceptable quality o
f life.