Present and future treatment modalities for gastrointestinal diseases in cystic fibrosis

Abstract

In recent years knowledge of the basic defect of CF has increased enormously. Many new drugs and treatment strategies are being introduced in the clinic. Nevertheless, there are a number of unsolved problems in the treatment of malabsorption and malnutrition. In spite of innovative technical and pharmacological improvement of pancreatic enzyme preparations maldigestion is still a problem. Better enzymes and coatings are needed. The integrated action of enzymatic digestion, intestinal motility and absorption is not under control. The role of malnutrition in the development of complications and in the outcome of the disease is still under discussion. The importance of a high energy intake is now generally accepted, but the question is how to achieve this. Tube feeding, endoscopic gastrostomy and the use of diets with a high energy content are becoming more popular. In CF the relationship between gastro-oesophageal reflux and lung complications is not well understood, but GER is frequently recognized. In the absence of CF GER is also associated with a number of pulmonary and upper airway diseases. The incidence of liver fibrosis and cholestatic liver disease is increasing with age. The problem is how to identify the patients at risk. The use of choleretic drugs has shown promising results in preliminary studies. Gene therapy in pancreatic and gastrointestinal pathology will be restricted to the liver. The biliary tract could be an interesting target, if patients at risk can be identified. In conclusion, new drugs and new strategies are necessary for the future implementation of the results of new insights in CF.