www.turner-white.com Pulmonary Disease Volume 14, Part 6 5
There are at least 3 classes of independent airway
clearance devices: positive expiratory pressure devices, airway oscillating devices (either handheld or
stationary), and high-frequency chest compression/
mechanical percussion devices. Until recently, there
was insufficient evidence to suggest superiority between any of the airway clearance techniques.70–73
However, a recent prospective multicenter randomized controlled study in Canada showed that positive expiratory pressure may prevent more exacerbations than high-frequency chest wall oscillation
via a vest in CF patients ages 6 years and older.74
Exercise
Exercise has become an important component in
the chronic management of CF patients. Exercise
intolerance is well established in this population. It is
mainly related to impaired lung function (especially
those with severe disease)75–78 as well as poor nutritional status and low muscle mass,75–79 although
other factors such as muscle dysfunction,80–83 CF
genotype,84 and physical activity (which is influenced
by a variety of psychosocial factors, such as gender
and parental involvement)85–90 may also influence
exercise capacity in CF patients. Exercise intolerance is dependent on the progression of the disease
and may have a significant role as a prognostic indicator of lung function decline, quality of life, and survival in CF patients.91,92 Exercise training programs
may be a useful therapeutic strategy to improve not
only physical fitness and exercise capacity in CF
patients,93 but also to improve airway clearance,94–96
quality of life,97,98 and lung function preservation93,97,98
and decrease the rate of hospitalization.98 Exercise
training is thought to have a positive impact on non-
pulmonary complications from CF, such as bone
mineral density99,100 and CF-related diabetes.101
Despite the evidence suggesting that exercise
may be beneficial in CF patients, there are no stud-
ies showing that exercise training prolongs survival
in this population. There is also no consensus about
the best exercise training modality: aerobic versus
anaerobic or a combination thereof. Unfortunately,
there have not been any successful strategies to in-
crease participation of CF patients in exercise train-
ing and maintain their adherence to it (the adher-
ence rate of CF patients to exercise training ranges
between 24% and 57%).91,92 The recommendations
for exercise training in CF patients at this time are
based on extrapolations from small studies as well
as an understanding of the physiological mechanism
of exercise and clinical reasoning.91,92 The incorpo-
ration of exercising training as part of the chronic
management of CF patients should be individual-
ized and take into consideration the patient’s age,
physical fitness, nutritional status, disease severity,
and potential barriers to adherence such as burden
of therapy, lack of information about the benefits of
exercise, and lack of parental involvement.
TREATMENT OF EXACERBATION
The management of CF exacerbations should
include the participation of the CF multidisciplinary
team including physicians, nurses, respiratory ther-
apists, social workers, pharmacists, and dietitians.
In addition to appropriate antibiotic therapy, the
therapeutic strategy should focus on airway clearance, nutrition support, drug monitoring, physical
therapy, and detection and management of other
complications such as CF-related diabetes.
The Cystic Fibrosis Foundation recommends
that CF patients receiving chronic treatment with
dornase alfa and hypertonic saline, macrolide
therapy, and bronchodilators should continue and
intensify these therapies during the course of an
exacerbation. 6, 10 With regard to the chronic inhaled
antibiotic therapy, there is no evidence that continuing inhaled antibiotics in addition to systemic
