The Basic Problem

Cystic fibrosis (CF) is an inherited disease that results in many of the body's secretions becoming very sticky. The abnormal transport of salt across the body's surfaces (epithelial membranes and the skin) is a major factor causing these abnormal secretions.

Cystic fibrosis is associated with an increased amount of sodium and chloride in the sweat - usually more than 60mmol/l. This is the basis of the Sweat Test that is used to diagnose CF.

THE SWEAT TEST IS THE MOST IMPORTANT AND FREQUENTLY USED CLINICAL TEST FOR THE DIAGNOSIS OF THE CLASSICAL FORM OF CF

The most important practical problem in CF results from sticky secretions in the respiratory passages. There is an increased tendency to blockage of small airways and infections in the lungs. These chest infections, if not treated, become more severe and persistent, eventually leading to progressive lung damage and respiratory failure.

In the pancreas, the sticky secretions lead to blockage of the ducts with secondary damage to the secretory gland tissue, usually before birth. This results in deficiency of the pancreatic digestive juices, both enzymes and bicarbonate, causing severe intestinal malabsorption, threatening nutrition and growth. Fortunately, with modern pancreatic enzyme treatment, the majority of infants and children can grow normally and most have few gastrointestinal symptoms.

CF therefore is a life-threatening multi-organ disease, requiring continuous use of multiple therapies to prevent organ damage, and meticulous care by the patient, the family and the health team over a lifetime. A team approach within the framework of specialised CF centres is essential for optimal care. Every CF patient should have regular input from this team. Local health care professionals such as general practitioners and paediatricians should be part of a 'shared care' arrangement with these teams.