Cystic fibrosis - nutritional considerations

Cystic fibrosis (CF) is a serious, life-threatening disease in children. It affects many body systems by producing abnormally thick secretions that can obstruct the pancreas, intestines, bile ducts, and air passages in the lungs.

With pancreas blockage, the enzymes (molecules that speed up chemical reactions) needed for absorption in the small intestine are not available, leading to malabsorption. Steatorrhea (fatty diarrhea) is frequent as are foul smelling stools, due to undigested fat, protein, and starch.

The major treatment breakthrough for malabsorption caused by CF was the development of enteric coated pancreatic enzymes. The enzymes are taken with meals and snacks. They improve the digestion and absorption of fat, starch, and protein.

The dietary needs are based on the severity of the symptoms. While many children with CF have delayed growth, the goal is to provide adequate nutrients for normal growth and development. From infancy through adulthood nutritious, high-calorie, high-protein foods are essential. Energy needs can be 50 - 100% greater and protein needs 50% greater than the Recommended Daily Allowance.

Historically, low-fat diets were discouraged to lessen fat malabsorption. With the advent of pancreatic enzymes, fat can be 30 - 50% of calories, depending on the person's ability to tolerate it. The body absorbs fat-soluble vitamins (vitamins A, D, E, K) poorly, so these are commonly prescribed. A multivitamin and liberal salt use is also recommended.

A multi-disciplinary team of a doctor, nurse, respiratory therapist, dietitian, and social worker, work with the patient and family to provide for an improved quality of life. Life expectancy continues to improve as new treatments are developed.

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