Cystic Fibrosis - nutritional considerations

Definition:

Cystic fibrosis is the most life threatening disease in children. It affects many body systems by producing abnormally thick secretions which can obstruct the pancreas, intestines, bile ducts and air passages in the lungs.

Functions:

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) are frequent and foul smelling stools, due to undigested fat, protein and starch.

The major treatment breakthrough for treatment of this disease 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 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 50% greater than the Recommended Dietary Allowance. Historically, low fat diets were discouraged to diminish fat malabsorption. With the advent of pancreatic enzymes, fat can be 30-50% of caloric need, depending on tolerance. 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.