There are about 30,000 people living with cystic fibrosis (CF) in the United States, and they need to eat high-fat, high-calorie foods to stay healthy. Sometimes, people with CF might not even feel well enough to eat the foods most people crave. And when schedules get really hectic, they can't just skip a meal like their friends do.
CF is a genetic disease that affects the body's epithelial cells, which are found in many places, including the sweat glands, the lungs, and the pancreas. An error in these cells causes problems with the balance of salt and water in the body. The body responds by making thick mucus, which blocks the lungs and sometimes other ducts and passageways, causing infections and breathing problems.
This mucus can also keep the intestines from absorbing important nutrients like fat and vitamins from food, which means that people with CF may be shorter and or weigh less than those without CF, and they may get sick more often because their bodies can't fight infections well. People with CF may need extra calories and nutrients to help them fight infection and keep their lungs strong, particularly if they get sick with colds or the flu.
But with the right balance of nutrition, extra fat and calories, and prescribed supplements, though people with CF can keep themselves healthy.
Nutrient Know-How
Like everyone else, people with CF should eat a balanced diet that includes plenty of fruits and veggies, grains and breads, dairy products, and protein. In addition, people with CF have some specific nutritional needs to help them stay healthy.
Some of the nutrients they need to get more of are:
- Protein. About 15% to 20% of calories should come from foods rich in protein, like meat, eggs, soy foods, fish, nuts, or beans.
- Iron. Iron is important to help fight infection. It also helps to carry oxygen blood from the lungs to every cell in your body. Fortified cereal, meats, dried fruits, and dark green vegetables are good sources of iron.
- Salt. People with CF lose a lot of salt in their sweat, especially during hot weather and when they exercise. A good way to replace this salt is by adding salt to food and eating salty snacks. A CF dietitian (a specially trained food and nutrition expert) may recommend sports drinks for after sports practice or exercise, especially during hot weather.
- Zinc. Zinc is important for growth, healing, and fighting infection. Good sources include meats, liver, eggs, and seafood.
- Calcium. Some people with CF are especially at risk for osteoporosis, a condition where the bones become weakened. Dairy products are good sources of calcium — and full-fat dairy products like whole milk are good sources of fat and calories as well. Many fruit juices now include calcium as well.
Fast Facts on Calories
In general, people with CF may need to eat 30% to 50% more calories a day than others — that's about 2,900 to 4,500 calories daily, depending on the individual.
People with CF has different nutritional needs. They can work with a CF dietitian to figure out exactly how many calories they need each day and to create a nutrition plan.
Some people with CF like to keep close tabs on the number of calories they eat in a day. Others find that counting calories is stressful and find it easier to focus on adding calorie boosters with fat in them to the foods they normally eat.
So how can people with CF add calories to meals? In general, they should avoid all diet foods. Whether eating at home or away, here are some simple tips:
- Drink whole milk and milk shakes.
- Add extra butter or margarine to foods like potatoes or pasta.
- Use regular (not diet) dressings on salads or vegetables.
- Eat burgers with bacon and cheese.
- Eat pizza with extra cheese.
- Add cheese to sandwiches.
- At breakfast, eat omelets with extra cheese and ham or bacon.
- Top salads and sandwiches with avocados or guacamole.
- Eat calorie-rich desserts such as ice cream, pudding, and cheesecake.
- Top hot chocolate, pudding, and other desserts with whipped cream.
Besides eating high-calorie meals, it's a good idea for people with CF to carry some high-energy snacks with them. These might include trail mix, nuts, packets of cheese crackers or peanut butter crackers, and veggies like carrots or celery with small containers of dressing that don't need to be refrigerated.
Meals and Munchies
People with CF may need to eat more regularly than some of their friends do, but that doesn't necessarily mean anything weird. Check out this sample meal plan that provides about 3,750 calories. The links to some of these foods will give you recipes for high-calorie foods that you can make:
- Breakfast — 3 frozen pancakes with 1 tablespoon butter and 3 tablespoons syrup; ½ cup of strawberries; Mighty Milk
- Morning snack — 1 cup whole-milk yogurt; ½ cup granola; ½ banana; water
- Lunch — Sandwich made with 4 ounces of turkey, 1 ounce of cheese, 1 tablespoon mayonnaise, 3 teaspoons mustard, lettuce, tomato, and/or onion; 10 baby carrots with 2 tablespoons ranch dressing; ½ cup apple juice; 14 pretzels; water
- Afternoon snack — ½ cup trail mix and 1 cup Mighty Milk
- Dinner — Creamy chicken fettuccine with broccoli; ½ cup juice; water
More Than Just Food
Some people with CF need to take vitamin supplements, especially for the fat-soluble vitamins (vitamins A, D, E, and K). These vitamins are important for healing and they need fat to be absorbed. Because most people with CF have trouble digesting fat, they often have low levels of these vitamins and may be prescribed supplements.
In some cases, people with CF may have low energy or trouble gaining weight, even with good nutrition and supplements. Doctors may recommend they get extra nutrients through a tube that is inserted into the stomach (called tube feeding). Tube feedings, which most people choose to do overnight, provide about 1,000 to 2,000 calories. These overnight feedings leave people with CF free to enjoy normal meals and activities during the day.
Tube feedings may sound gross, but they can be an excellent way to help people who are having trouble gaining weight, especially when they are sick. Having a tube put in for the feedings is usually an uncomplicated procedure that doesn't require extensive surgery or a long hospital stay. In fact, most people can go home the same day they have feeding tubes inserted.
Enzyme Supplements
About 85% of CF patients have pancreatic insufficiency. This means that the body doesn't pass certain chemicals, called enzymes, from the pancreas into the intestines properly. These enzymes are necessary for a person to digest fat, starch, and protein. People with pancreatic insufficiency might have problems with growth and weight gain, and they might also have frequent and bad-smelling bowel movements.
People with pancreatic insufficiency need to take prescribed enzymes with meals and snacks to help them digest their food properly and get the nutrition they need. A CF doctor will work with a dietitian to prescribe enzymes based on weight and how much the person with CF eats at a time. Enzymes need to be taken with every meal and most snacks. They should not be chewed or crushed up, and the dose should only be adjusted by the dietitian or doctor.
Beating the Frustration
It can be difficult for people with CF to eat enough in a day to meet their needs, especially when they aren't feeling well. Although lots of people think they would like to be able to eat whatever they want, many people with CF find it difficult to do so.
If you have CF, eating well and taking your enzymes and supplements will benefit you now and in the future. And if you have a friend with CF, be supportive. It doesn't help to complain or joke about your own eating and dieting frustrations when someone has a much larger challenge.
In addition to the right diet, exercise can help give people with CF the energy and mental boost they may need. Talk to your doctor about the right kind of exercise for you, and pay careful attention to how you feel while exercising — and rest or stop if you get tired.
Like everyone else, people with CF need to take care of their bodies so they are healthy, strong, and full of energy.
Reviewed by: Allison Brinkley, RD, LD/N, CNSD
Date reviewed: February 2007
