People living with cystic fibrosis generally need to eat more food than people like you or me to stay healthy. Cystic fibrosis, an inherited genetic disorder caused by a mutation on chromosome 7, affects the epithelial cells, specifically those in the lining of the lungs, pancreas and sweat glands.
Cystic fibrosis causes an imbalance in salt and water in the body. When this happens, the body produces a thick mucus, which can coat the lungs and prevent the intestines from absorbing essential nutrients. That interferes with weight gain.
Cystic fibrosis causes difficulty digesting and absorbing fat. Children and teens with cystic fibrosis are often underweight and may have trouble growing because of malabsorption. They require extra calories, particularly from fat and protein to help them grow and prevent illness. Caloric intake can be as much as 4,500 calories a day, more than twice the usual recommended daily limit, depending on their needs.
Diet options for people with cystic fibrosis
Like everyone, people with cystic fibrosis should eat a balanced diet that includes whole grains, fruits and vegetables, dairy products, and protein. High-fat, high-calorie foods and snacks; foods with extra salt and low in sugar, can be of great benefit to people with cystic fibrosis.
Good food choices can include cheeseburgers, pizza with extra cheese and vegetables, eggs, sandwiches with all the toppings, drinking milkshakes and eating desserts made with dairy and topping them with whipped cream.
Extra salt is also important because people with cystic fibrosis lose more salt in their sweat than others do. Keep a saltshaker on the table, allow salty foods and snacks like pretzels, french fries, pickles, and deli meats.
Keeping it simple, what can you add to meet the cystic fibrosis dietary requirements:
- Extra butter and oil to bread, vegetables, salads, and any pan you are cooking in
- Mayonnaise and mustard to salads and sandwiches or baking fish or chicken
- Sour cream to dips, vegetables, and cakes
- Whipped cream to top fruit, desserts, and hot chocolate
- Dips and dressings
- Cheese to eggs, sandwiches, mashed potato and pasta
- Nut butters to crackers, sandwiches, and smoothies
- Milk, cream, and yogurt to breakfast, sauces, and baking
- Protein powder or nutrition supplements to drinks and smoothies.
It’s all about the snacks!
Three meals plus snacks can provide enough calories to help children grow and develop more normally. And this habit needs to remain into adulthood. Offering high energy snacks like seeds, nuts, grains, protein bars, cheese sticks, and vegetables can be easily carried to school and work and do not need refrigeration. Smoothies can be enriched with protein powder, yogurt, and even avocado.
Pancreatic enzymes
Pancreatic enzymes assist cystic fibrosis people better digest food, particularly fat, starch, and protein. This will help with pancreatic insufficiency, the lack of enzymes from the pancreas that help the intestines work properly. Pancreatic insufficiency can interfere with growth and weight gain and cause bad-smelling bowel movements. Supplemental enzymes are taken when eating meals to help food digest properly.
Vitamins
Vitamins are important for everyone’s normal growth and development. Cystic fibrosis people may not get enough vitamins A, D, E, and K. These are fat-soluble vitamins which are normally digested as part of fat absorption. There are vitamins specially formulated for cystic fibrosis people. Healthcare teams can best advise vitamin support.
Getting enough iron and calcium is important when a child’s bones are growing. Zinc can help support the immune system and ability to heal wounds.1
Feeding tubes
Children with cystic fibrosis who simply cannot get enough food into their diet to support normal growth and development may require supplemental feedings. These are done through two kinds of feeding tubes. A NG tube, nasogastric, is a tube inserted through the nose into the stomach. A longer-term approach to supplemental feeding involves a G-tube (gastrostomy). This is a tube that is inserted into the stomach during a surgical procedure that can accommodate enhanced formulas which add up to 2,000 calories in an overnight feeding.
