ACPCF Infant Guideline - The Knowledge Network

Clinical guidance for the Physiotherapy Management of Screened
Infants with Cystic Fibrosis
Introduction
These guidelines have been compiled to complement the Delphi Consensus
on Physiotherapy Management of Asymptomatic Infants with Cystic Fibrosis
(CF). 1
Babies born with CF have essentially normal lungs but within variable time
scales develop chronic respiratory disease which will ultimately be fatal in the
majority of patients. With the recent introduction of newborn screening
throughout the UK babies are diagnosed with CF soon after birth, often before
they have any symptoms or lung pathology. Additionally, babies with mild
mutations who may not have become symptomatic until adult life, are also
being diagnosed in infancy. This means that there is now a cohort of infants
who present to CF centres at a very young age and often with no signs or
symptoms of disease. Over the last two decades, the importance of early
intervention has been recognised, 2,3 and most CF centres have adopted a
policy of close monitoring and aggressive treatment of early lung disease. A
significant number of children with CF now have normal lung function well into
early adulthood even though they are very likely to have underlying lung
pathology. 4
Traditionally in the UK, chest physiotherapy has been instigated at diagnosis,
consisting of twice daily postural drainage (PD) using a head down tip
combined with chest wall percussion. Many babies and young children
presenting to CF centres now display no overt signs of respiratory disease and
have good nutritional status with body mass index (BMI) within normal limits.
Specialist physiotherapists in the UK caring for these babies have begun to
question the role of traditional, routine airway clearance in these
‘asymptomatic’ babies. While health professionals agree unanimously that
physiotherapy interventions are appropriate once respiratory symptoms are
apparent, 5,6 the place of routine daily airway clearance prior to this is less
clear. It is also recognised that while infants may be asymptomatic at
diagnosis they may over a given time span, swing along a spectrum of being
asymptomatic at times and symptomatic at others. How this should be dealt
with in terms of routine airway clearance is also not established.
A review of patients and families by the CF Trust reported that chest
physiotherapy is considered a large burden of care. Families wish to know if
routine treatment is necessary in those babies with few or no symptoms. 7
Physiotherapists have a duty to provide safe and effective care and daily
treatment regimens need to be tailored to individual needs, lifestyle and
symptoms, particularly as long term routine ACT is seen as a substantial
burden for patients and families. 7
With these apparently asymptomatic babies the dilemma now facing
physiotherapists is whether it is necessary to recommend daily routine airway
2