Cystic fibrosis is a hereditary disorder that affects multiple organ systems such as the respiratory tract, intestines, pancreas and liver. If you suffer from cystic fibrosis, you are at high risk for malnutrition due to the malabsorption and maldigestion of nutrients. According to Kathleen Mahan and Sylvia Stump, authors of "Krause's Food and Nutrition Therapy," proper medical nutrition therapy is a key component to optimizing growth or maintaining weight and pulmonary function. Prior to making any significant changes to your diet, consult your physician and/or a registered dietitian.
Energy Intake
Energy needs for those suffering from cystic fibrosis vary significantly from one individual to the next. Most often, a high-calorie diet is recommended due to malabsorption. According to Mahan and Stump, protein should consist of 15 to 20 percent of your total calories, and fat should total 35 to 40 percent. Increasing fat intake to meet these levels should help you obtain your higher energy requirements and decrease your risk for fat-soluble vitamin deficiencies. According to Trabulsi et al., authors of a research article published in the "American Journal of Clinical Nutrition" concerning energy intake in preadolescents with cystic fibrosis, children with the disease who obtain a high-energy, moderate-fat diet with pancreatic enzyme supplementation should be able to maintain a healthy age- and gender-specific weight.
Vitamins
Pancreatic enzyme supplementation is recommended in "Krause's Food and Nutrition Therapy" if you have poor absorption or maldigestion due to pancreatic insufficiency. If you experience fatty stools, called steatorrhea, diarrhea or other gastrointestinal symptoms, consult your physician to evaluate your needs and dosage recommendations for such enzymes. For individuals on pancreatic enzyme supplementation who take an age-appropriate multivitamin/mineral supplement, water-soluble vitamins B and C seem to be adequately absorbed. Fat-soluble vitamins A, D, E and K, however, remain low. Intakes of such vitamins for adolescents over the age of 8, as recommended by B.W. Ramsey et al., authors of "Nutrition Assessment and Management in Cystic Fibrosis: a Consensus Report," are 10,000 international units per day of vitamin A, 200 to 400 IU per day of vitamin E, 400-800 IU per day of vitamin D and 0.3 to 0.5 mg per day of vitamin K.
Minerals
According to Mahan and Stump, consuming minerals according to the dietary reference intake that is specific for your age and gender should be sufficient; however, sodium and, in some cases, zinc, magnesium and iron, should be given additional attention. Sufferers of cystic fibrosis lose significant amounts of salt in sweat. Low sodium intake can result in dehydration, fatigue and vomiting. Most individuals who consume the average American diet have adequate salt intake; however, during infancy and when experiencing fever, hot weather and physical activity, sodium intake should be increased. As for zinc, magnesium and iron intake, consuming a multivitamin/mineral supplement along with a healthy diet should help ameliorate these deficiencies.
Feeding Strategies
Consuming adequate calories is of significant importance to maintaining optimal health and pulmonary function. Encouraging positive eating behaviors and promoting regular and enjoyable, tasty meals is extremely helpful. Consuming snacks, large portions and foods with high nutrient density, such as nuts and cheese, will help you meet your energy needs. Liquid supplements may prove beneficial as well. A diet high in calories with sufficient vitamins and minerals may slow the decline of pulmonary function, decrease risk for infection and improve your overall quality of life.
References
- "Krause's Food and Nutrition Therapy"; Kathleen Mahan and Sylvia Stump; 2008
- "American Journal of Clinical Nutrition"; Evaluation of Formulas for Calculating Total Energy Requirements of Children With Cystic Fibrosis; J Trabulsi; 2007
- "American Journal of Clinical Nutrition"; Nutrition Assessment and Management in Cystic Fibrosis: a Consensus Report; B.W. Ramsey, et al.; 1992
