Clinical Care
Good nutrition is a vital part of staying healthy with cystic fibrosis
Nutrition
Calories
People with CF need to consume lots of calories. This section gives ideas for ways to add more calories to your diet. Enjoy!
Around the House
You can add calories to your diet without spending a lot of money on commercial products. You can add extra calories to meals with food you have around the house, including butter, cream, peanut butter and cheese.
| Ideas for Adding Calories and/or Protein to Meals and Snacks | |
|---|---|
| Any food | Add “nut dust” – grind up nuts and add to food |
| Bread | Add butter |
| Casseroles | Add powdered milk and eggs |
| Chips | Add high-calorie dips |
| Fruits | Add high-calorie dips or whipped cream |
| Hamburger patties | Add eggs before cooking meat |
| Mashed potatoes | Add powdered milk and butter |
| Meats | Make “super-milk” by adding equal parts milk and half and half |
| Pancakes/Waffles | Add powdered milk and eggs |
| Casseroles | Add peanut butter and syrup |
| Pasta | Add butter or margarine before you add the sauce |
| Pizza | Add extra cheese and meats, and dip in dressings |
| Pretzels | Add high-calorie dips |
| Sandwiches | Add butter or mayonnaise |
| Shakes | Add powdered milk |
| Vegetables | Add butter |
Snack Time
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. Here are some snack ideas:
- Pudding
- Ice cream
- Leftover pizza
- Trail mix or granola
- Cheese or peanut butter and crackers
- Almonds, pistachios, peanuts or other nuts
- Muffins or bagels with cream cheese or jelly
- Homemade milkshakes with ice cream and whole milk
Commercial Products
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. Talk to your CF team to find what works best for you.
Exercise
Exercise is essential for both kids and adults with cystic fibrosis. It is important to take a few extra precautions to ensure that you get the greatest benefit from your activity.
Fluids
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. Sports drinks are a good choice because they provide carbohydrates for energy and small amounts of salt.
Salt
The extra salt that individuals with CF lose during exercise should be replaced with salty foods like potato chips. Do not rely on sports drinks alone to replenish salt lost during exercise because these beverages do not contain enough salt to meet CF the needs of people with CF.
Extra Calories
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. It is very important to plan for this extra food so that you do not lose weight.
Fat and Fiber
What is fat?
Fat is one of the three kinds of nutrients that give you energy. Fat has nine calories per gram—more than twice as much as carbohydrates or proteins. Fat, part of a group of substances called lipids, is made up of carbon, hydrogen and oxygen.
Where do I get fat?
Good sources of fat include oils, butter, eggs, cheese and nuts.
Why do I need fat?
Fat helps the body function properly. 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. Fat also serves as the storage substance for the body’s extra calories.
How much fat do I need?
CF patients should get about 35 to 40 percent of their calories from fat. This is more than the recommendation for the general public. Extra fat in your diet will help you meet your increased calorie needs.
What is fiber?
Fiber is a carbohydrate that your body does not digest. It helps give a feeling of fullness, and moves stool through the digestive tract.
Where do I get fiber?
Fiber is present in all plants. Good sources include fruits, vegetables, whole grains and legumes.
Why do I need fiber?
Fiber is important because it helps form stools and decreases the chance of bowel obstruction.
How much fiber do I need?
Adults should get about 25 to 30 grams of fiber a day. For children over age 2, the recommended daily intake is the child’s age plus 5 grams.
Proteins
What is protein?
Protein is one of the three kinds of nutrients that provide calories in your diet. Proteins has 4 calories per gram and is made up of many small amino acids.
Where do I get protein?
Protein can be found in meat, fish, milk, eggs and beans.
Why do I need protein?
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. The cells of muscles, tendons and ligaments are developed and maintained with proteins.
How much protein do I need?
Protein should make up about 16 to 20 percent of your calories. People with CF should try to get two to four servings of protein-rich food every day.
| Protein Serving Sizes |
|---|
| A1 egg or 2 tbsp. of peanut butter |
| Higher fat or protein options like eggs and meat |
| ½ cup of cooked beans, lentils or legumes |
| 2 to 3 ounces of meat, poultry or fish. This is about the size of a deck of playing cards |
Salt and Fluid
What is salt?
Salt is a mineral (sodium chloride) that can be added to foods to change their taste. It has many important functions in the body.
Where do I get salt?
Any food that tastes salty, like chips, pickles, fast food, frozen dinners and french fries is a good source of salt. Adding salt directly to food will also increase your intake.
Why do I need salt?
Salt is essential for muscle function, digestion, and nerve cell health. It also helps move fluid in and out of cells. Since CF patients have a higher than normal concentration of salt in their sweat, they need to replace the salt that is lost.
How much salt do I need?
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. There is not a specific amount of salt that individuals with CF should have every day, but your health-care provider can determine the amount that is right for you.
Fluid
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. Juice and soda, however, can cause children to feel full and decrease their appetite for healthier drinks and snacks, and should be limited to less than six ounces a day for toddlers and children, and less than 12 ounces a day for adolescents and adults.
Nutrition and School
It is important to discuss CF nutrition with a child’s teachers and school nurse as soon as school begins. Educating everyone involved is the best way to provide good care for a school-age child. The CF Foundation has an excellent nutrition educational materials designed for schools.
Pancreatic Enzymes
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. Your child may need to leave class a few minutes early to allow enough time to eat lunch.
Extra Portions/Snacks
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. Your CF team can write a prescription requesting extra portions or snacks, if needed.
Bathroom Breaks
Children with CF may need additional bathroom breaks, especially if their malabsorption is not well controlled. Alerting the teacher of this possibility at the beginning of the school year may help alleviate any embarrassing moments.
Tube Feeding
In an effort to ingest enough calories and gain weight, many children and adults with CF use tube feedings for additional nutritional support.
What is a G-tube?
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. This way, it’s possible to receive an extra 1,000-1,500 calories at night.
What is an NG Tube?
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. With this method, it is possible to get an extra 1,000-1,500 calories at night.
Can I eat during the day if I have a G-tube or an NG tube?
Yes. You can still eat normally throughout the day and you may actually be able to stop taking high-calorie supplements because you will receive so many calories at night.
Do I need to take enzymes with a tube feeding?
Yes. Your CF team will explain how you should take your enzymes; many patients take a “meal” dose of enzymes at the beginning and end of the feedings. There is a also a cartridge with immobilized lipase available to digest fat in the formula, which can be attached to the tubing .
How long will the feedings run?
Your CF team can help you decide your tube feeding schedule. Feedings are usually completed when you sleep.
Do I have to keep a G-tube forever?
No. You should keep your tube only as long as you need it. Some people are able to gain enough weight and eat enough by mouth to have their tube removed, while others prefer the “insurance” that a tube provides.
Who should I talk to about getting a tube?
If you think a tube might be the right choice for you, discuss it with your CF team. They will refer you to a gastroenterologist for tube placement and education.
Gastrostomy tube (G-tube)
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. Your CF team will check your vitamin levels every year to make sure that your body has the amount it needs to function properly.
Important Nutrients
- Zinc
- Iron
- Calcium
- Vitamin A
- Vitamin D
- Vitamin E
- Vitamin K
Vitamin A
What?
A fat-soluble vitamin that can also be made from beta-carotene.
Where?
Found in egg yolks, milk, liver and fortified cereals. Good sources of beta-carotene include dark green or orange fruits and vegetables like carrots, sweet potatoes, winter squash, spinach, broccoli, apricots, cantaloupe and peaches.
Why do I need Vitamin A?
Vitamin A plays an important part in vision, healthy bones and the immune system. Beta-carotene works as an antioxidant to keep tissues healthy.
How much Vitamin A do I need?
The Cystic Fibrosis Foundation recommends the following guidelines for daily Vitamin A intake:
| Age | Vitamin A (IU) |
|---|---|
| 0-12 months | 1,500 |
| 1-3 years | 5,000 |
| 4-8 years | 5,000-10,000 |
| 8 years and older | 10,000 |
Vitamin D
What?
A vitamin that the body absorbs with fat.
Where?
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. Talk to your CF team about the right amount of sun for you.
Why do I need Vitamin D?
Vitamin D helps to build and maintain strong bones by keeping the right amount of calcium and phosphorus in the blood. Without enough vitamin D, your bones can become thin and brittle.
How much Vitamin D do I need?
The Cystic Fibrosis Foundation recommends the following guidelines for daily Vitamin D intake:
| Age | Vitamin D (IU) |
|---|---|
| 0-12 months | 400-500 |
| 1-10 years | 800-1000 |
| 11 years and older | 600-800 |