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Chest Physical Therapy in Cystic Fibrosis: A Review

Cystic fibrosis (CF) is the most common inherited disease in Caucasians, with an incidence of approximately 1 in 2500. It is invariably fatal. Due to variations in the clinical disease, and multiple organ involvement, treatment of cystic fibrosis has generally been concentrated in large regional centers where skilled multidisciplinary care is available. This approach has resulted in earlier diagnosis, improved treatment, and median life expectancy into the late 20s. Although CF was first described in 1932, the CF gene was just found in 1989 and localized to chromosome #7, although the basic defect in CF is still unknown. Recent research indicates the CF gene product may alter a protein responsible for the transport of sodium chloride. The end result is the production of hyperviscos secretions from the exocrine secreting glands. CF is expressed in varying severity in those afflicted: pulmonary, gastrointestinal, hepatobiliary, endocrine, and reproductive systems may all be involved. It is beyond the scope of this paper to discuss each system individually; we will therefore concentrate on pulmonary physical therapy intervention in CF. Failure to thrive, steatorrhea, chronic productive cough, pseudomonas bronchitis and pneumonia, bronchiectasis, and reactive airway disease are routinely seen. Pneumothorax, hemoptysis, cor pulmonale, gallstones, cirrhosis, diabetes, sinusitis, and arthritis are less frequent complications of the disease. Pulmonary manifestations are the major determinant of both quality and duration of life. In 1938, when Dorothy Andersen' described the condition of cystic fibrosis, postural drainage (PD) and percussion became a recognized part of the treatment for this disease. Traditionally, all patients with CF were taught daily programs of passive PD with percussion followed by controlled coughing performed by a PT or relative. This passive approach continues in some parts of the world, but more active methods (eg, forced expiratory technique, positive expiratory pressure, autogenic drainage, and exercise) are now used in several countries. A review of clinical studies of the past 15 years specifically examining the effectiveness of the traditional techniques in CF reveals both positive and inconclusive outcomes. The purpose of this paper is to present current literature evaluating the usefulness of chest physical therapy (CPT) on CF patients.