What Is Cystic Fibrosis?

At lunch, Lindsay often gets bored with having the same old conversation with her friends:

"You're so lucky!"

"Yeah, I'd give anything to be able to eat as much as you do and be so skinny!"

"Cheeseburgers or mac and cheese every day — that would be great."

Lindsay is one of about 30,000 people living with cystic fibrosis (CF) in the United States, and she has to eat high-fat, high-calorie foods just to stay healthy. A lot of the time she just doesn't feel well enough to eat the foods most teens crave. And sometimes, when her schedule's really hectic, she'd love to just skip a meal like her 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 teens with CF may be short and underweight for their age, and they may get sick a lot because their bodies can't fight infections well.

People with CF 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.

With the right balance of nutrition, extra fat and calories, and prescribed supplements, though, teens with CF can keep themselves healthy.

Nutrient Know-How

Like everyone else, guys and girls who have 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. Here are some of the nutrients they need to get more of:

• Protein. About 15% to 20% of the calories a teen with CF eats 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 in your blood from your lungs to every cell in your body. Fortified cereal, meats, dried fruits, and dark green vegetables are good sources of iron.
• Salt. Teens 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 gym class, especially during hot weather.
• Zinc. Zinc is important for growth, healing, and fighting infection. Good sources include meats, liver, eggs, and seafood.
• Calcium. 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, teens with CF may need more calories a day than others in their age group — that's about 2,900 to 4,500 calories daily, depending on the individual. What are all those calories for? Like every other teenager, girls and guys with CF need calories to fuel their growth during puberty. They just need more of them.

Every person with CF has different nutritional needs. Teens with CF and their parents can work with a CF dietitian to figure out exactly how many calories they need each day. The dietitian looks at growth and weight gain over time and comes up with 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 milkshakes.
• 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 guys and girls with CF to carry some high-energy snacks with them; try 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 approximately 3,750 calories. The links to some of these foods will give you recipes for high-calorie foods that teens with CF 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 teens with CF need to take vitamin supplements, especially for the fat-soluble vitamins (vitamins A, D, E, and K). These vitamins are important for growth and healing and they need fat to be absorbed. Because most teens with CF have trouble digesting fat, they often have low levels of these vitamins and may be prescribed supplements.

In some cases, teens with CF may have low energy or trouble gaining weight, even with good nutrition and supplements. For these teens, 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 teens 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 teens who are having trouble gaining weight, especially when they're 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 teens who have feeding tubes inserted can go home the same day as they get the procedure done.

Enzyme Supplements

About 85% to 90% 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 digesting 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.

Someone with pancreatic insufficiency needs to take prescribed enzymes with meals and snacks to help food digest properly so he or she gets the nutrition needed for growth and development. A CF doctor will work with a dietitian to prescribe enzymes based on weight, growth, 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 teens 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'd 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. Remember Lindsay's friends? She'd probably appreciate it if they didn't bring up how much she eats every day!

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, teens with CF need to take care of their bodies so they grow up healthy, strong, and full of energy.

Reviewed by: Danielle Scotto, RD, LDN, CNSD, CSP
Date reviewed: September 2010
Originally reviewed by: Allison Brinkley, RD, LD/N, CNSD