Cystic Fibrosis (CF) is a debilitating disease that affects secretory cells that produce mucus, sweat, and digestive juices. CF is genetically inherited as an autosomal-recessive trait that mostly occurs in the Caucasian population, or about 1 in 3,500 births. The treatment and early detection of the disease has aided in a drastic improvement in the survival rate (the average life expectancy is 37 years), even though some individuals are not diagnosed until the late teens. A defect in the CF gene, located on chromosome 7q, causes mucus to become thick and sticky, which plugs up many essential ducts and passageways. CF predominately alters the proper functions of the lungs and pancreas.
Respiratory Symptoms and Complications
- Coughing up sputum and mucus
- Shortness of breath
- Decreased ability to exercise
- Infections with various different strains of bacteria, which causes many hospitalizations and decreases prognosis
Digestive Symptoms and Complications
- Pancreatic insufficiency – This is due to the production of thick mucus, which blocks the secretion of digestive enzymes from the pancreas into the small intestine. This leads to the inability of the digestive system to metabolize and break down fat consumed though the diet. Also, pancreatic insufficiency can often lead to Cystic Fibrosis-Related Diabetes (CFRD).
- Bulky, foul-smelling stools
- Poor weight gain and/or failure to thrive
- Intestinal blockage
- Gallstones (blocked bile ducts)
- Rectal prolapse
Medical Treatments of Cystic Fibrosis
- Many medications – Antibiotics, mucus-thinning drugs, bronchodilators, inhaled medications, and pancreatic enzymes.
- Chest physical therapy – Such as a clapper or an inflatable vest that shakes the chest cavity to loosen the mucus.
- Surgical procedures – Nasal polyp removal, oxygen therapy, endoscopy and lavage, lung transplant, feeding tube.
- Counseling – Nutritional and/or psychological counseling may be recommended for patients and their families.
Nutritional Implication of Cystic Fibrosis
Due to the GI tract’s inability to break down and absorb fats, CF patients are at a high risk for malabsorption and malnutrition. Because each CF patient manifests the disease differently in terms of the age of the patient, severity of the disease, stage of their treatments, lung function, GI function, and other complications, nutrition interventions are very individualized from patient to patient.
Regardless, the nutrition management of CF patients typically include:
- Controlling maldigestion and malabsorption with pancreatic enzymes – Digestive enzymes, or enteric-coated enzyme microspheres, are consumed orally and released in the duodenum. The dosage of these enzymes depends on the degree of pancreatic insufficiency and the amount and type of food consumed. The more fat being consumed in a meal will results in a higher dosage of enzymes in order to metabolize the fat.
- Meeting increased energy needs – Energy needs will vary depending on the patients age, gender, BMR, physical activity, respiratory function, and severity of malabsorption. (Some tips to increase energy and calories are suggested below)
- Promoting appropriate weight gain – Nutritional supplements are often recommended in order to increase energy intake and promote weight gain.
- Vitamin/mineral supplementation – Typically, fat-soluble vitamins (vitamins A, D, E, and K) are poorly absorbed in CF patients and may need to be taken as a supplement or a multi-vitamin. Also, osteoporosis is also common because vitamin D aids in the absorption of calcium. Therefore, CF patients may have increased calcium needs.
Scandishakes are a common nutrition supplement recommended by dietitians for patients with CF who have increased energy needs.
Scandishakes contain ~600 calories when mixed with eight ounces of whole milk. That’s a lot of calories in a small volume of fluid, which is great for CF patients trying to gain weight!
Here are some nutrition tips for individuals with CF:
- Eat as often as possible
- Prepare foods with, or top foods with, high calorie-condiments such as extra butter, margarine, dressings, gravies, creamy sauces, heaving whipping cream, peanut butter, and whole milk to foods and recipes whenever possible
- Add dried skim milk powder to add protein to sauces and beverages
- Add extra cheese to pizza, baked potatoes, soups, pastas, etc
- Add extra deli meats to sandwiches
- Top bacon to burgers
- Milkshakes, milkshakes, milkshakes
- Add instant breakfast mixes to milk-based drinks
- Add nuts to cookies, cakes, pancakes,
- Add nuts, eggs, meats, and creamy dressings to salads
- Breaded meats and fish adds calories
L. Kathleen Mahan & Sylvia Escott-Stump. Krause’s Food & Nutrition Therapy, 12th Edition