Cystic fibrosis (CF) is a genetic disorder characterized by the buildup of thick, sticky mucus in the lungs, pancreas, and other organs. This mucus can lead to various complications, including difficulty breathing, malabsorption of nutrients, and frequent infections. Nutrition plays a vital role in managing CF, and a well-planned diet can help individuals maintain a healthy weight, fight infections, and improve their overall quality of life.
Nutritional Needs in Cystic Fibrosis
Increased Calorie Requirements
People with CF typically need up to twice the amount of daily calories as others of similar age and weight. The energy needs of people with CF are estimated to be 1 ½ to 2 times the needs of those without CF. This is because they expend more energy due to chronic lung infections, frequent coughing, and difficulty absorbing nutrients. Fighting infections and coughing on a regular basis also burns extra calories. Maintaining a healthy weight - and sometimes increasing it - is key to fighting infection and keeping your lungs and body strong.
Importance of Fat
CF patients should aim to get about 35 to 40 percent of their calories from fat, which is higher than the recommendation for the general public. Fat has nine calories per gram-more than twice as much as carbohydrates or proteins. Dietary fats provide “essential fatty acids” obtainable only from food. Linoleic acid is the most important essential fatty acid, especially for the growth and development of infants. Fatty acids provide the basic materials to control blood pressure, blood clotting, inflammation, and other body functions. Healthy skin and hair are maintained by fat, and fat helps in the absorption and transport through the bloodstream of vitamins A, D, E, and K.
Protein Requirements
Protein should make up about 16 to 20 percent of your calories. Protein is one of the three kinds of nutrients that provide calories in your diet. Proteins are the building blocks in your body and are the main components of muscle, organs, and glands. Every living cell and all body fluids, except bile and urine, contain proteins. A single egg or 2-3 ounces of meat, poultry, or fish can provide a significant amount of protein. A person with CF should eat protein-rich foods to aid muscle maintenance and physical ability. Research suggests that protein can prevent muscle loss and improve the outcome for people with CF.
Vitamins and Minerals
Vitamins and minerals are very important in a healthy diet. These nutrients help build strong bones and fight infections. It is very difficult to get the proper amount of the following vitamins from foods, which is why your CF team recommends a special multivitamin that is easily absorbed.
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- Vitamin A: Plays an important part in vision, healthy bones, and the immune system. Found in egg yolks, milk, liver, and fortified cereals.
- Vitamin D: Helps to build and maintain strong bones by keeping the right amount of calcium and phosphorus in the blood. Found in fortified milk, liver, salmon, and fortified cereals. Your body can also make Vitamin D during sun exposure, but it is important not to get too much sun because of the risk of skin cancer.
Fluid Intake
Fluid is important for a balanced diet and for hydration. Losing extra salt and water in your sweat increases the chance of dehydration, especially in the summer. Water is an excellent choice to maintain fluid balance. Milk is also a good choice because it provides calories and protein, in addition to fluid.
Determining Individual Needs
There is no one specific recommended method to determine energy needs. Just how much should you weigh? BMI is calculated by dividing your body weight in kilograms (kg) by your height in meters squared (m2). Calculate your BMI with this online BMI calculator from the Centers for Disease Control and Prevention (CDC). The CF Foundation recommends that women maintain a BMI of at least 22 and men, a BMI of at least 23. Spend some time thinking about what your weight gain goal should be. Discuss what you want to accomplish with the dietitian on your CF care team.
Strategies for Increasing Calorie Intake
Adding Calories to Meals
Your dietitian can offer some simple ways to add calories to the food you already eat. Or you can add 100 calories to each meal and snack, so that it equals 500 calories. Remember, these extra calories should be part of a well-balanced diet.
Snacking
People with CF should have 2 to 3 snacks per day. Adding (or increasing) all of your snacks at one time can be difficult so it’s easier to set smaller goals. Adding an extra 300-500 calorie snack or increasing a snack you already have to 300-500 calories is a great way to increase calories. Once the snack becomes a habit, you can add another or try to increase a meal by 300 calories.
High-Calorie Supplements
Many commercial products, like high-calorie drinks, can increase caloric intake. By choosing one you enjoy, you will be more likely to maintain a routine.
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Easy and Affordable Calorie Boosts
As you can see, nothing on this list costs much or takes much effort to make. In other words, you can get your 500 extra calories eating on the go.
Specific Food Recommendations
Fruits
Fruits are particularly rich in antioxidants and fiber. Fruits and other high-fiber foods can reduce intestinal blockages in people with CF, while antioxidants can help fight off infections. Research suggests that taking antioxidant supplements can reduce inflammation and respiratory symptoms in people with CF. Although the study involved people taking supplements, antioxidants from foods may also be helpful. Some fruits contain vitamin C, which is an essential antioxidant. Children and adults with CF can enjoy a variety of fruits, including:
- Apricots
- Bananas
- Blueberries
- Cantaloupes
- Grapefruit
- Mangoes
- Oranges
- Peaches
- Strawberries
Fruit makes a great snack when on the go. It is also a delicious way to add sweetness and nutrients to breakfast cereals, oatmeal, yogurt, and many other dishes and snacks.
Vegetables
Like fruits, vegetables offer many health benefits. They are rich in antioxidants and fiber and contain essential vitamins and minerals, including calcium. However, dark leafy green vegetables are probably the best option because they are also a good source of the following nutrients:
- Iron, which carries oxygen around the body and helps fight infections.
- Vitamin A, which boosts immunity and supports healthy eyes and intestines.
- Vitamin K, which plays a role in blood clotting.
People can get vitamin K from the following dark leafy greens:
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- Arugula
- Bok choy
- Broccoli
- Brussels sprouts
- Collard greens
- Kale
- Mustard greens
- Spinach
A person should also include a variety of other vegetables in their diet. Orange vegetables, such as carrots and sweet potatoes, are good sources of vitamin A, while garlic has antibiotic properties that may protect against bacterial and viral infections.
Eggs
Eggs are a good source of protein, with one large, boiled egg containing more than 6 grams (g) of protein. Like other animal products, eggs contain vitamin B-12, a nutrient that is essential for red blood cell formation and proper nerve function. Eggs are often a favorite food of children, especially scrambled or fried eggs. People can also use them to make omelets.
Fish and Seafood
Fish and seafood are excellent sources of protein, iron, and vitamin D. Consuming fatty fish, such as salmon, herring, and trout, is a good way to increase the intake of calories and healthful fats. People need vitamin D for strong bones and to aid calcium absorption. Individuals with CF must keep their bones healthy because they have an increased risk of developing bone diseases, such as osteoporosis or osteopenia. Shellfish contain high levels of zinc, which is an essential mineral that supports normal growth and development in children and adolescents. Throughout a person’s lifespan, zinc facilitates healing and good immunity. A zinc deficiency can affect taste and smell, which could further reduce the appetite of a person with CF.
Nuts
Nuts are an excellent snack for both children and adults with CF because they are high in calories and healthful fats and contain good amounts of protein and fiber. Nuts also contain several vitamins and minerals. Almonds are a great source of the antioxidant vitamin E, with 1 oz providing 37% of the recommended daily intake. Peanuts are another excellent source of vitamin E, while Brazil nuts are rich in selenium, a nutrient that protects against oxidative damage and infection.
Dairy
Dairy is a good source of calcium. Calcium is an essential mineral for everyone, but it may be especially important for people with CF who are at increased risk of bone disease. The body does not produce calcium naturally, but people can add it to their diet by taking supplements or consuming products that contain it. Full fat dairy products also provide significant amounts of healthful fat and calories.
Olive Oil
An easy way to increase the number of PUFAs in the diet is to introduce olive oil, which is an essential component of an anti-inflammatory and Mediterranean diet. Adding this oil can also help people take in extra calories and fat when their appetite is lacking.
Dark Chocolate
Dark chocolate is a delicious snack food, and it is a good source of calories and fats. According to an article in the International Journal of Preventive Medicine, cocoa is also a significant source of antioxidants and may have anti-inflammatory properties. To reap the benefits of dark chocolate, it is best to opt for a product that contains at least 70% cocoa solids.
Salt
Salt is a mineral (sodium chloride) that can be added to foods to change their taste. Any food that tastes salty, like chips, pickles, fast food, frozen dinners and french fries is a good source of salt. Salt is essential for muscle function, digestion, and nerve cell health. It also helps move fluid in and out of cells. CF patients need to have a high salt diet. Infants need to have salt added to their formula or baby food. Children, adolescents and adults should use the salt shaker liberally and eat foods that are high in salt, like chips, pickles, fast food, frozen dinners and french fries. Extra salt (along with fluid) is especially important during hot weather.
Eating Patterns and Meal Planning
Eating Regularly
Eat whenever you are hungry. This may mean eating several small meals throughout the day. Keep a variety of nutritious snack foods around. Try to snack on something every hour, such as cheese and crackers, muffins, or trail mix. Try to eat regularly, even if it is only a few bites. Or, include a nutrition supplement or milkshake. Be flexible. If you aren't hungry at dinner time, make breakfast, mid-morning snacks, and lunch your main meals.
Meal Planning Tips
- Plan ahead: Give yourself time to plan. Before you go to sleep, think about the busy day ahead. Where will you be spending your time? Where can you easily stash food? Is there a refrigerator nearby? Microwave? How about a place to eat?
- Think about “packability.” Plan meals or snacks that you can carry in your backpack, purse, or briefcase and store in your desk drawer, locker, or a cooler in your car.
- Cook once to eat three times. Use a slow cooker. If you are cooking for only one or two people, look for magazines and recipe books designed for smaller portions so food doesn't go to waste.
- Organize your kitchen so that everything you need for “grab 'n' go” snacks and meals are within arm's length. Keep plastic containers on hand to store meal-sized portions in the refrigerator or freezer.
- Avoid wandering aimlessly around the grocery store by planning ahead.
- Read food labels. Food labels will help you learn to choose foods that meet your goals.
Sample Meal and Snack Ideas
- Breakfast: Overnight oatmeal made with heavy cream and dried fruit and nuts.
- Snacks: Cheese and crackers, muffins, trail mix, peanut butter on bread products, yogurt with fruit and nuts.
- Lunch/Dinner: Add grated cheese to soups, sauces, casseroles, vegetables, mashed potatoes, rice, noodles, or meat loaf. Use whole milk, half and half, cream, or enriched milk in cooking or beverages. Enriched milk has nonfat dry milk powder added to it. Serve cottage cheese with canned or fresh fruit. Add grated cheeses, tuna, shrimp, crabmeat, ground beef, diced ham or sliced boiled eggs to sauces, rice, casseroles, and noodles.
Considerations for Children with CF
School
Each school has its own policy for administering medication, so it’s important to learn the rules at yours. A special form signed by a doctor is usually required to allow your child to take his or her enzymes before lunch. Children who buy lunch at school may not receive adequate portion sizes and may need an extra snack to meet their calorie goals. It is often possible to get extra portions and snacks for your child by talking to the school administration. Children with CF may need additional bathroom breaks, especially if their malabsorption is not well controlled.
Gastrostomy and Nasogastric Tubes
Gastrostomy tubes, or G-tubes, help provide extra calories. A physician implants a small tube through the skin into the stomach, which is connected to a longer tube and pump at night. The pump delivers a high-calorie formula while the patient sleeps. Nasogastric (NG) tubes are inserted through the nose into the stomach. Nasogastric tubes can be inserted and removed every night or left in for up to a month at a time. The NG tube is connected to a longer tube and a pump at night, delivering a high-calorie formula while the patient sleeps.
The Role of Enzymes
Most people with CF must take pancreatic enzymes. These enzymes help your body absorb fat and protein. Taking them all the time will decrease or get rid of foul-smelling stools, gas, and bloating. Take enzymes with all meals and snacks. Talk to your provider about increasing or decreasing your enzymes, depending on your symptoms.
Special Considerations
Vegetarian Diets
For a variety of reasons, many people have switched to a vegetarian diet. If you have CF, it is possible to create nutritious plant-based meals and snacks that pack the protein, fats and carbohydrates you need.
Dehydration
Every cell in the body needs water to work properly. The amount of fluid you need depends on your age, weight, activity level, general health and the climate. Do not wait until you feel thirsty to drink water. Some symptoms of dehydration are headache, nausea, loss of appetite and fatigue. You also can tell if you are dehydrated by looking at the color of your urine. Urine should be clear or a pale yellow. If your urine is dark yellow or brownish, you are not getting enough fluids. Vitamins and medications may temporarily affect color.
Exercise
Exercise is essential for both kids and adults with cystic fibrosis. Drinking enough fluid is very important, especially during the summer. As a rule, people with CF should have 6 to 12 ounces of fluid 15 minutes before exercise and every 15 to 20 minutes during exercise. Fluids should always be available, and planned fluid breaks are very important. Fluids should not contain caffeine because it can cause dehydration. The extra salt that individuals with CF lose during exercise should be replaced with salty foods like potato chips. When you exercise, you burn additional calories. You may need to add an extra snack (or two) to make up for the calories lost during exercise.
Foods to Avoid
Most foods are suitable for people with CF. However, because these individuals have increased calorie and fat needs, they should try to avoid foods that manufacturers label as diet, low fat, fat free, or reduced calorie.