Cystic fibrosis (CF) is a genetic disease. It affects about 30,000 children and young adults in the United States. CF prevents the body from absorbing enough nutrients, making it difficult for the individual to meet his or her nutritional needs. As a result, people with CF may need to eat an enriched diet with more calories and take extra vitamins and enzymes.
Most people with CF are diagnosed by the age of 3. However, tests are available to diagnose this condition much earlier. Some hospitals in North America screen newborns for CF routinely.
CF affects the mucus-producing glands in the pancreas, lungs and intestines, causing thick mucus to buildup and congest the lungs. This can lead to life-threatening infections. The mucus can also cause congestion in the pancreas. This is the gland that makes many of the hormones and enzymes needed for digestion of food.
The mucus buildup can cause malabsorption of nutrients. This is when nutrients from foods are not absorbed, but are instead passed out in the stool. Because of this, people with CF must eat a lot more food to receive enough calories and nutrients to maintain normal weight. Children with CF, whose bodies are using calories and nutrients to grow, must sometimes consume up to six times as many calories as a healthy child in order to grow properly.
Enzymes are proteins made in our bodies. They spark various reactions, including those involved in the breakdown of food. Many times, a person with CF does not produce enough of a fat-digestive enzyme called lipase. He or she may need to take specially formulated enzyme supplements, with each meal, to aid in digestion.
Since people with CF cannot absorb salt from sweat, they need salt, which is sodium chloride, in larger amounts. This is especially true during hot weather when there is increased sweating. Drinking plenty of fluids is important to avoid dehydration, or low body fluid levels.
Higher amounts of vitamins and minerals may also be needed because of the body's difficulty absorbing them. A person with CF needs about twice the recommended daily allowance (RDA) for fat-soluble vitamins, which are vitamins A, D,E and K. Many times supplements are needed because the individual cannot reasonably consume enough of the vitamins in his or her diet.
For all these reasons, an individual with CF needs to be closely followed by a registered dietitian as well as by other healthcare professionals. The registered dietitian handles the complex nutritional issues that come up in the care of these individuals.
Mahan, K, MS, RD, CDE&Escott-Stump, S., MA, RD, LDN. (2000).
Krause's Food, Nutrition,&Diet Therapy (10th ed.). Pennsylvania: W.B. Saunders Company.