Cystic fibrosis - nutritional considerations

Definition

Cystic fibrosis (CF) is one of the most life-threatening diseases 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.

Function

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 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.

Side Effects

The most serious complication of CF is infection in the lungs. Also, the inability of the lungs to clear out infection and mucus may lead to the inability to breathe. Nutrition plays an important role in strengthening the patient's immune system to fight disease.

Recommendations

The following are methods for adding protein and calories to the diet. In addition to these tips, make sure that you are taking a multivitamin containing Vitamins A, D, E and K:

• Eat whenever you are hungry. This may mean eating several small meals throughout the day.
  
• Keep a variety of nutritious snack foods around. Try to snack on something every hour. Try cheese and crackers, muffins, or trail mix.
  
• Make an effort to eat regularly, even if it's only a few bites; or include a nutritional supplement or milkshake.