Physiotherapy in infants and young children with cystic fibrosis: current practice and future developments.

Abstract

The manifestation and onset of cystic fibrosis (CF) lung disease is highly variable and the collective wisdom of experienced physiotherapists based in clinical practice is that no standard physiotherapy treatment will suit everyone. Despite this, many physiotherapists keep looking for a standard physiotherapy treatment that will suit all. The number of (apparently) asymptomatic CF neonates diagnosed with newborn screening is increasing worldwide. In the past, physiotherapy in CF was synonymous with airway clearance therapy in order to decrease pulmonary symptoms. The definition of the term ‘modern physiotherapy in CF’ is much wider. It involves the whole individual in a long-term perspective. The traditional physiotherapy treatment approach in infants has been extrapolated from studies in older patients with chronic sputum production. This approach may be flawed as it fails to consider the uniqueness of the immature infant and small child. During the past two decades a number of different airway clearance techniques have been developed. The choice of an airway clearance technique seems currently to be led by culture, tradition and geographical influences. Development of an optimal individualized physiotherapy regimen requires knowledge about respiratory physiology and pathophysiology, the aims of modern CF care and the rationale for the alternative physiotherapy techniques available. Optimal treatment is not synonymous with maximal treatment. Side-effects of treatment in this vulnerable immature population need to be carefully considered to make sure we do no harm. The aims of modern, individually tailored physiotherapy, as one part of the CF care package, are primarily to retard lung disease and preserve physical function in order to improve quality of life and long-term outcomes. ‘Good physiotherapy is the mainstay to clinical well being in CF’.1