The Physiotherapy Management of Paediatric Cystic Fibrosis: Current Concepts

Article type: Research Article

Authors: Prasad, SA

Affiliations: Cystic Fibrosis Unit, Great Ormond Street Hospital for Children NHS Trust, Great Ormond Street, London WC1N 3JH, UK

Note: [] Correspondence to: S Ammani Prasad. Email: [email protected]

Abstract: Cystic fibrosis (CF) is the most common inherited disease of the Northern European caucasian population. The carrier rate within the UK is 1:25 and the incidence 1:2500 live births. The disease is caused by a defect of the CF gene, the cystic fibrosis transmembrane conductance regulator (CFTR), the protein of which encodes for a chloride channel in the apical membrane of exocrine epithelial cells. In addition CFTR also modifies the function of other ion transporters (sodium and potassium channels) and has an effect on water permeability, ATP transport and mucus secretion. The main clinical characteristics of the disorder are pancreatic insufficiency and chronic progressive lung disease. Other problems include male infertility, liver disease and diabetes. Soon after description of the disease by Dorothy Andersen in 1938, chest physiotherapy in the form of postural drainage and percussion became a mainstay of the respiratory management of CF. Airway clearance continues to play an integral role in the care of children and adults with CF but the role of the physiotherapist in caring for CF has changed significantly over the past three decades and has grown to include physical exercise, postural care and the need to address emerging and unique complications which arise as longevity continues to improve.

Keywords: Cystic fibrosis, Physiotherapy, Airway Clearance, Exercise

DOI: 10.3233/PPR-2009-30203

Journal: Physiotherapy Practice and Research, vol. 30, no. 2, pp. 5-12, 2009