CYSTIC FIBROSIS
This is an inherited disease that affects the exocrine glands (glands that excrete to the outside of the body). There is excessive secretion of thick mucus which often blocks the ducts of the liver, pancreas, and lungs. Large amounts of sodium chloride are excreted in the perspiration.
If the pancreatic ducts are blocked, the pancreatic enzymes cannot reach the duodenum, and malabsorption is often severe. The child appears haggard, shows signs of tissue wasting, has a large protruding abdomen, and excretes large, foul stools with much fat, starch, and protein.
Dietary management
In pancreatic dysfunction pancreatic extract is given before each meal to aid digestion. A high-calorie high-protein diet is essential. If fats cannot be tolerated medium-chain triglycerides (MCT) are useful to increase the calorie intake. MCT is an oil available in a pharmacy that can be used for food preparation. Because the oil is made up of short-chain fatty acids, it is more readily absorbed. Supplements of the B-complex vitamins, ascorbic acid, and water-soluble preparations of vitamins A and D are usually required. Additional salt intake is required when skin losses of salt are great. Patients find it difficult to eat if their breathing is affected. They must eat slowly and should be given small meals at frequent intervals.
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GENERAL HEALTH
