Nutrition and Cystic Fibrosis

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By ACCORDANT HEALTH SERVICES

Nutrients are in the food we eat that we use to live and grow. Most of the nutrients in food get taken into the body through the small intestine. Nutrients can only be taken into the body if enzymes from the pancreas are present. In about 93 out of 100 people with CF, mucus blocks the ducts of the pancreas. This stops the enzymes from getting to the food in the small intestine. Without enzymes, food cannot be broken down for use by the body. Food passes out of the body without being digested. This is called malabsorption.

Malnutrition
Malabsorption of nutrients can lead to malnutrition. Malnutrition means that the body is not getting enough nutrients to work the right way. Because nutrients are not being absorbed, people with CF can be malnourished even if they are hungry and eat a lot of food. They may be shorter and weigh less than a healthy person who is their same age and gender.

Taking enzymes helps delay malnutrition. Most people with CF need to take enzyme pills with all their meals and large snacks. Babies need enzymes with their formula or breast milk. These enzymes replace the natural ones that cannot get through the blocked pancreas ducts.

Nutrition and Lung Health
Enzymes alone do not always stop malnutrition. Because of lung disease, people with CF need many more calories than other people do. With CF, it takes more energy to breathe. At rest, a person with CF is still spending more energy than a person who does not have CF. Even mild lung disease calls for many calories to fight swelling and infection.

After age two, everyone with CF needs 110% to 200% more calories than others do. For some teens and adults, this may mean eating as many as 5,000 calories a day. Compare that to the average adult who should only eat about 2,000 calories each day. Children may need up to 2,800 calories a day. But, when people do not feel well, they may not feel hungry. Many also have CFRD (cystic fibrosis-related diabetes) or other CF problems. These factors also lead to malnutrition.

The better your diet is, the better your lung function is likely to be. When your diet is poor, lung disease is much more likely to develop. The good news is that forceful nutrition can slow down or put off lung problems. Great nutrition may even help people with CF live longer. One reason for this is that good nutrition and a good supply of calories help the body fight lung infections. Less lung infections means stronger lungs, which often allows for a longer, healthier life. Most of the time, a higher body weight means better lung function.

The Importance of Early Care
Eating right early on is one of the best ways to avoid malnutrition. Thanks to newborn testing for CF, many parents can now give their child high quality nutrition right from birth. When a person is diagnosed later, CF care providers pay special attention to nutrition in the first 12 months after the diagnosis.

Research shows that a child's nutrition rank at age three can predict how well their lungs will work at age six. Since children with CF are at high risk for malnutrition, it is best to form good eating habits when children are young.

This is not always easy. One study of the eating habits of CF children under age 12 showed that most of them:

• Skipped at least one meal or snack every few days.
• Got enough calories only one day out of four.
• Ate low fat rather than high fat meals about half the time.
• Forgot to take their enzymes about 15% of the time.

There are many ways to improve nutrition. Research shows that the methods all work well, but the most important thing is to start using them early. When good nutrition is started before problems begin, there is a better chance of success. Good early nutrition can have a good effect on health for the rest of a child's life.

Nutritional Therapies
Because of the important role that nutrition plays in CF, preventing malnutrition is a main goal. Nutritional treatment for CF includes three basic parts:

• Diet. People with CF need a healthy, balanced diet that is very high in calories. It should also be high in fat, protein, and salt. People with CF should try to get about 2-4 servings of protein each day. One serving can be found in two tablespoons of peanut butter or a piece of meat or fish that's the size of a deck of playing cards. Thirty-five to forty percent of calories should come from fats. High fat foods include butter, cheese, and nuts. Extra salt and fluids are needed, especially in hot weather or when exercising. There are no rules on diet for adults or children with CF.
• Enzymes. People with CF must take enzymes to help them break down food. Enzyme pills are taken by mouth. Inside the pill there are small beads of enzymes. The beads have a coating that keeps them from dissolving until they get to the small intestine. For babies, capsules are opened and the beads are sprinkled on an acidic food such as applesauce. The amount of enzymes a person needs is based on their age, the size of their body, and the type of food they are eating.
• Vitamins. Vitamins A, D, E, and K are fat-soluble vitamins. They dissolve in the fat you eat. Since digesting fats is a problem for most people with CF, water-soluble forms of these vitamins are taken.

If malnutrition, slow growth, or slow weight gain are problems, more forceful steps can be taken to help nutrition. Some choices are:

• Behavior Treatment. When children ages 1-12 have slow growth, behavioral treatment may help. It may also help if a child has mealtime behaviors that are causing problems. Behavior treatment is used along with nutrition advice and other treatments to help children gain weight.
• Tube Feeding. If a child is not growing well, or if an adult is having trouble gaining weight, tube feeding may be the answer. Tube feedings are an easy way to give extra calories. Feedings are often done at night while a person is sleeping. Tube feedings can be done in four ways:
• OG (orogastric): by mouth
• NG (nasogastric): through the nose
• GT (gastrostomy): through the stomach
• JT (jejunostomy): through the intestine
• Hunger Stimulants. Two medicines, megestrol acetate and cyproheptadine, can be used to try to increase hunger. But experts do not agree if these help much.
• Growth Hormone. Some studies have shown that recombinant human growth hormone (rhGH) improves both height and weight for children. In one study, the improvements lasted even after the rhGH treatments were stopped. A different study found that rhGH improved growth, but not weight. The people who did this study think that younger people with good lung function will benefit the most from growth hormone treatments.

Checking Nutrition Level
Both children and adults need to have their nutrition level reviewed often. Everyone with CF should have a checkup at least four times a year.

Checking BMI
The best way to tell if a person's nutrition is good is to see if their weight is right for their height. For children, growth in height is also important. Both of these are measured by body mass index (BMI). BMI is a number that stands for a person's height and weight. A higher BMI most often means better lung function.

Three different BMI scales are used: one for babies and children under age two, one for children and teens ages 2-20, and one for adults 20 and older.

• For adults, the goal is a BMI of 23 for men and 22 for women. An adult with a BMI of less than 19 has malnutrition. As age increases, so does malnutrition. It is thought that about 38 out of 100 adults with CF are in the malnutrition grouping. These adults need forceful nutrition treatment.
• For children and teens (ages 2-20), the BMI scale is based on percentiles. The goal for this age group is to keep up a BMI at or above the 50^th percentile. Children and teens with a percentile of less than 25 are "at risk." When the score is at or under the 10^th percentile, the child or teen has malnutrition and needs forceful nutritional treatment.
• For children younger than two, the goal is to reach the 50^th percentile on the "weight for length" scale by their second birthday. Young children are considered "at risk" when their value falls below the 25^th percentile. Values in the 10^th percentile or below means the child is not getting enough nutrients. Steps must be taken to correct the problem.

More than half of adults with CF have a BMI that is lower than the recommended level. This is also true for children. A low BMI is a worry for both adults and children because of the link between good nutrition and living longer. A dietician is a healthcare expert who specializes in helping people get the right amount of nutrients. You or your child should see a dietician who has experience in managing people with CF.

There are easy-to-use BMI calculators for children and adults on the Center for Disease Control and Prevention Web site. Go to www.cdc.gov and type "BMI calculator" in the search box.

Checking Malabsorption

• To make sure of the best possible nutrition, it is important to check both children and adults for any signs that food is not being digested. The signs are:
• Poor weight gain even with a very good appetite.
• Many loose and/or large bowel movements.
• Foul smelling stools.
• Mucus or oil in stools.
• A lot of gas or stomach pain.
• Bloating.

If these signs are present, the type of enzymes or how much you take may need to be changed. Never raise or lower the dose of enzymes without talking to your doctor or dietitian. Taking too many enzymes can harm the large bowel. Taking too few enzymes may cause DIOS (distal obstructive syndrome), a block in the bowel.

Other Signs of Poor Nutrition
There are two other signs that suggest nutrition is poor. These are:

• Delayed puberty. For girls this means no breast growth by age 13 or not starting periods by age 16. For boys this means no genital changes or enlargement of the testicles by age 14. Delayed puberty is also a worry for social and emotional reasons.
• Weight plateau. This means there is no increase in weight for a period of time. For children under age five, a weight plateau has been reached if there is no weight gain for three months or more. For children over age five, a weight plateau is defined as no increase in weight for six months or more.

Ten Tips for Adding Extra Calories

• Drink whole milk at every meal. Better yet, make "super milk" by mixing the same amounts of whole milk and half and half.
• Use cream on your cereal.
• Make smoothies by mixing ice cream and fruit with supplement drinks like Ensure^®, Boost^®, or Carnation^® Instant Breakfast.
• Make "nut dust" by grinding up nuts and sprinkling it on any food you like.
• Butter your veggies, hot soups, potatoes, and pastas.
• Add eggs to hamburger patties before you cook them.
• Add extra cheese to your pizza.
• Add powdered milk to casseroles, meatloaf, and puddings.
• Put peanut butter and syrup on pancakes and waffles.
• Add butter or mayonnaise to sandwiches.

Remember: fats – found in foods such as butter, oil, nuts, cheese, and cream – have 9 calories per gram. That's more than twice as many calories as you get from a gram of protein or carbohydrates!

References

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Last Modified Date: March 19, 2009 © Accordant Health Services, a CVS/Caremark company. �All rights reserved.
This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team.
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