Cystic fibrosis affects the glands that produce mucus, sweat, enzymes, and other secretions. The most serious consequences of the disease occur in the lungs, pancreas, and intestines, all of which become clogged with thick mucus. As the lungs become congested, the person is especially vulnerable to pneumonia and other infections. If the ducts that normally carry pancreatic enzymes to the small intestine become clogged, digestive problems can result, such as difficulty in breaking down fats and proteins. In addition, abnormal amounts of salt are lost in sweat and saliva, which can lead to serious imbalances in body chemistry.
There is no cure for cystic fibrosis, although scientists are testing gene therapy as a means of correcting the underlying genetic defect. In the meantime, a combination of an enriched diet, vitamin supplements, replacement enzymes, antibiotics and other medications, and regular postural drainage to clear mucus from the lungs serves as the most effective treatment and has greatly improved the outlook for people with cystic fibrosis.
Nutrition Connection
Because diet is critical in managing cystic fibrosis, the treatment team usually includes a clinical dietitian, particularly for children who need to consume many more calories than normally recommended to grow properly. There is no special diet, but the following are general guidelines:
-Eat larger portions and lots of snacks. Children are encouraged to eat high-calorie foods and large amounts at meals. Frequent snacks also help. Babies with the disease may be given a formula that contains predigested fats.
-Eat more protein. For older children, high-protein foods, such as meat, poultry, fish, and eggs, are emphasized. You can enrich whole milk by adding a cup of dried milk per quart or liter.
-Eat more fats. Diets with as much fat as the child can tolerate are recommended. Fats provide more calories per unit than other nutrients, so they are a critical source of energy. The body also needs fat in order to absorb vitamins A, D, E, and K.
-1,300 to 1,500 mg of calcium is the recommended amount someone 9 years old or older with cystic fibrosis should consume to help avoid osteoporosis.
-Balance sugary foods. About 35% of adults ages 20 to 29 and 43 of those over 30, with cystic fibrosis have cystic fibrosis–related diabetes. Unless they have diabetes, they may enjoy sugary foods; these simple carbohydrates are more easily absorbed than starches. However, sweets should be taken with protein to provide balance and supply amino acids needed for growth, immune function, and repair and maintenance of body tissue.
-Consume more sodium. Because cystic fibrosis causes sweat and salivary glands to excrete abnormal amounts of sodium and chloride in perspiration and saliva, salt is an essential part of the diet. It’s crucial to watch sodium levels during hot weather or exercise.
-Drink fluids. Because constipation and intestinal obstruction are common in cystic fibrosis, it’s important to consume adequate water and other fluids. Juices and nectars can provide a higher-calorie intake than water. A doctor may prescribe a laxative to prevent constipation.
-Coordinate a medication regime. Combinations of inhaled medication and antibiotics can be used to make it easier to breathe and help fight against infections.
-Talk to your doctor about supplements. Prescription enzymes that improve absorption of fats and protein have made a big difference for those living with cystic fibrosis. If digestive problems develop despite taking enzymes, supplements of predigested fats may be prescribed to help loosen and clear thick mucus from the airways.
-Monitor for diabetes. Some people with cystic fibrosis may also develop diabetes if the pancreas becomes so clogged that it can no longer make adequate insulin.
QUICK TIP:
Mix a perfect meal
An omelet made with herbs and cheese provides an almost perfect mix of nutrients, protein, and calories for those with cystic fibrosis.
Beyond the Diet
The seemingly insurmountable challenges to those dealing with cystic fibrosis may be eased with the following recommendations:
-Learn coughing triggers. A common treatment for cystic fibrosis teaches a person how to trigger strong coughs that help loosen and clear thick mucus from the airways.
-Seek support. Counseling and therapy can help deal with the tremendous emotional stresses of the disease, including better communication between family members and health care providers, as well as preparing a patient for adult care.
