What Is Cystic Fibrosis?

Cystic fibrosis (CF) is a genetic disorder that affects the body's epithelial cells. These cells are found in many places, including the sweat glands, lungs, and pancreas.

An error in the cells causes problems with the body's balance of salt and water. The body responds by making thick mucus, which blocks the lungs and sometimes other ducts and passageways, causing infections and breathing problems.

How Does Cystic Fibrosis Affect Nutrition?

The sticky mucus from cystic fibrosis can block normal absorption of key nutrients and fat in the intestines, causing:

• poor digestion
• slow growth
• trouble gaining weight
• greasy bowel movements

Why Do Some Kids Need Enzymes?

CF often causes a condition called pancreatic insufficiency. This means the enzymes made in the pancreas that digest fat, carbohydrates, and protein don't pass into the intestines as they should. So the body can't digest food normally.

To replace these enzymes, kids take pancreatic enzymes in pill form:

• Enzymes should be taken with all foods and drinks that have fat and protein.
• For babies and younger children who can't swallow pills, empty the pill's beads into a small spoonful of applesauce. After giving the enzymes, check for beads left behind in the mouth as they can cause irritation.
• Beads should never be sprinkled on foods. They cannot be chewed or crushed. They must be swallowed whole.
• Enzymes are sensitive to temperature. Store in a cool, dry place. Avoid extremes in temperature, such as leaving enzymes in the car during the summer or winter months.

Signs that a child may need enzymes or a change to their enzyme dose include:

• poor weight gain, in spite of a strong appetite
• frequent, large, greasy, or smelly bowel movements
• bloating or gas

The dietitian or doctor will prescribe enzymes based on weight, growth, and bathroom habits. As a child grows, so does their appetite and their enzyme dose.

Nutritional Needs in Kids With Cystic Fibrosis

Nutrition is important for keeping healthy with CF. Many kids with CF will need extra nutrients compared with their peers.

Registered dietitians are an important part of the health care team. They can work with families to help kids with CF get the nutrition they need.

What Extras Do Kids With CF Need?

Kids with cystic fibrosis have some specific nutritional needs:

Salt. Kids with CF lose more salt in their sweat than their peers, especially during hot weather and exercise. The dietitian may suggest adding salt to an infant's formula, offering salty snacks, and sending sports drinks along when kids play sports or have gym class, and in hot weather.

Fat-soluble vitamins (vitamins A, D, E, and K). These vitamins are important for immune function, growth, and healing. They're absorbed along with fat. Most kids with CF have trouble digesting and absorbing fat, which means they may not absorb fat-soluble vitamins. Your child's fat-soluble vitamin levels will be checked once a year, and a CF-specific multivitamin may be prescribed.

Calcium. Kids with CF, especially those with pancreatic insufficiency, are at risk for osteopenia or osteoporosis (weak, brittle bones). Dairy products are good sources of calcium (and the full-fat varieties also are good sources of fat and calories). If your child or family doesn't drink cow's milk, consider trying a milk alternative that is fortified with calcium.

Calories. Some kids with CF need extra calories to grow healthy and strong. The extra calories a child gets should come from a well-balanced, healthy diet.

Why Do Some Kids Need Nutrition Supplements or Tube Feedings?

Sometimes, enzymes and high-calorie meals aren't enough to help a child with cystic fibrosis thrive. It's not always physically possible for kids to get what they need through eating a high-calorie diet.

If that's the case, a doctor or dietitian might recommend adding nutrition supplements to your child's diet. Nutrition supplements are calorie-rich drinks that also have protein, vitamins, and minerals. For some kids, this added source of calories is enough to improve weight gain.

If a high-calorie diet and nutrition supplements are not enough, a doctor or dietitian might recommend tube feedings. These feedings, done through a tube into the stomach, are often given at night for extra calories. They're an excellent way to help kids who have trouble gaining weight, while easing some of the stress around eating. The decision to place a feeding tube can be hard, but many families express relief afterward.

Tube feedings do not mean that you've failed to feed your child well, or that he or she isn't a good eater. Often they just mean that a child needs so many calories a day that it's impossible to keep up without extra help. Having a tube put in for the feedings is usually an uncomplicated procedure that doesn't need extensive surgery or a long hospital stay.

What Else Should I Know?

Meeting the unique nutrition needs of a child with CF isn't always easy. Work with your team to set nutrition goals that your child can reach. Many emotions are linked to feeding a child. Working with the psychologist on your CF care team can help you manage any stress.