Section 10 - Cystic Fibrosis Clinical Care Pathway

Section Two:
Pulmonary Management
2.1 Physiotherapy
Chest physiotherapy is an integral part of the management of CF. It is
thought to prevent and reduce pulmonary complications such as atelectasis
and hyperinflation by the removal of bronchopulmonary secretions. Removal
of these secretions reduces the overall proteolytic activity in the lungs, which
could reduce the progression of elastase-mediated damage to the airways and
the mucociliary transport system. The role of the physiotherapist is not
limited to airway clearance but also includes encouragement and advice on
exercise, posture, mobility and inhalation therapy.
Most patients with CF do require some form of chest physiotherapy daily,
however this should be tailored to their individual needs, according to age,
clinical status and social circumstances.
2.1.1 Management of infants
and small children
Traditionally it has been recommended that newly diagnosed infants with CF
begin a twice-daily regimen of postural drainage and percussion undertaken
by a parent or guardian. Postural drainage is carried out in alternate side lying
with a head down tip, supine, prone and in the sitting position. The rationale
for this approach has been based on three arguments. Firstly, early
intervention may prevent the onset of complications and secondly that there
is clear evidence from studies of bronchoalveolar lavage, infant lung function
and radiological imaging that manifestations of lung disease occur at a very
early stage in the disease process. Lastly it is argued that introducing a routine
of chest physiotherapy from an early stage so that it becomes part of the
child’s daily routine will improve compliance with treatment.
With earlier diagnosis and particularly the introduction of neonatal screening
many infants now presenting with CF have very little in the way of
respiratory symptoms and are often well nourished. Although daily treatment
continues to be recommended even in this asymptomatic group of patients
there is as yet no evidence to suggest that routine physiotherapy has an
impact on the course of the pulmonary changes. Furthermore poor adherence
to treatment has been associated with the lack of immediate and obvious
benefit from therapies and daily regimens of chest physiotherapy place a
significant burden on parents and carers. In preference to a routine
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