Cystic Fibrosis and Dietary Needs According to Age

What is Cystic Fibrosis?

Cystic fibrosis (CF) is a genetic disease that causes production of thick mucus in certain organs throughout the body, most commonly the lungs and pancreas. As mucus build-up increases in the lungs, breathing can become difficult; the mucus can also block ducts (pipes) of the pancreas and stop natural enzymes from reaching the intestines and helping the body break down and absorb the nutrients in food.

You Are What You Eat

 All children and adults need to be aware of what they eat whether they have been diagnosed with a disease that affects the digestive system or not. For people diagnosed with CF, not only do they need to make the right meal choices, but they also need to pay attention to the quality and quantity of food they eat and how frequently they eat.

The Cystic Fibrosis Foundation (CFF) recommends adjusting dietary needs according to age and overall weight and growth. It is important at all ages that parents are sensitive to their child’s eating behavior and food intake, through infancy, toddler years, childhood, and adolescence. During these stages, dietary choices and habits can affect the rest of the child’s life.

How Does CF Affect Nutrition?

Most people who have CF also have a condition known as exocrine pancreatic insufficiency (EPI), which means that the important digestive enzymes in the pancreas cannot reach the intestines to aid in the digestion of important nutrients such as fat, carbohydrates and protein. If these nutrients are not digested, all of the vitamins and minerals that are in them will not be absorbed into the body. In order for people who have EPI due to CF to digest and absorb the nutrients in food, they need to take supplemental pancreatic enzymes.

To get enough calories to promote normal weight gain and growth, individuals with CF may need to consume up to two times as many calories as a person without the disease. Also, to be sure that the correct amounts of vitamins are available, a multivitamin designed for people who have CF is usually prescribed.

Even though people who have CF need extra calories, this does not mean people with CF can eat foods like french fries covered in cheese as their only food at every meal! They need to balance food intake at meals and snacks to stay on track with weight gain, growth and overall healthy nutrition.

Cystic Fibrosis and Dietary Needs According to Age

It is important for parents, caregivers and patients to learn good nutrition habits and to understand the different dietary needs of people with CF at every age. The Cystic Fibrosis Foundation (CFF) recommends adjusting dietary needs according to age and overall weight and growth. During the early stages of life, dietary choices and habits can affect the rest of a child's life. It is also important for people with CF to maintain healthy nutrition habits as they become independent during the teen years and into adulthood.

Many people with CF also have a condition known as exocrine pancreatic insufficiency (EPI), in which they lack pancreatic enzymes necessary for proper food digestion. In particular, people with EPI due to CF have difficulty absorbing fats and nutrients provided by them. Therefore, key "fat-soluble" vitamins, including A, D, E and K, are not fully absorbed. People with CF usually take supplemental vitamins daily. Eating foods high in calcium, iron, sodium chloride and zinc, are also important for healthy nutrition. These essential minerals are also available as supplements.

EPI

(Most People who have CF also have a condition known as exocrine pancreatic insufficiency (EPI),which means that the important digestive enzymes in the pancreas cannot reach the intestines to aid in the digestion of important nutrients such as fat, carbohydrates and proteins. if these nutrients are not digested, all of the vitamins and minerals that are in them will not be absorbed into the body.

In order for people who have EPI due to CF to digest and absorb the nutrients in food, they need to take supplements pancreatic enzymes.)

Important Facts for Your Infant with CF

• A balanced diet which, includes optimal calories, vitamins and minerals is important for good nutritional health for your infant with EPI due to CF
• Your infant with CF needs more calories, vitamins and salt when compared to infants who do not have CF. Infants with EPI due to CF need to be given supplemental pancreatic enzymes every time they are fed
• Breast milk and/or infant formula is the best source of nutrition in your baby’s first year of life
• If a baby’s weight gain is slow, your CF Center dietitian or care provider may suggest ways for you to add extra calories to breast milk or formula
• Vitamin supplements are important for babies who have CF to help meet their nutritional needs. Your CF Center dietitian will prescribe the vitamins and minerals that are just right for your baby

• All people who have CF, including babies, need extra salt. It is very important that you add the amount of salt your CF Center dietitian has recommended to your baby’s meals. Too much or too little salt can be a problem for your baby

For most children and people without CF, "nutritious snacks" are lower in fat and calories than is recommended for children and adults with CF. So, in addition to eating higher quantities of nutritious snacks, people with CF are often also advised to “pack in” more calories and fat to each serving. People with CF and caregivers should work with their dietitian to make sure proper nutritional needs are met.

Developing Positive Eating Habits in Infants with CF

• Taking care of a newborn can be challenging. Newborns who are breast fed may want to eat frequently throughout the day and night. Formula fed babies may want to eat every three hours. Once you and your baby have a feeding routine, try to establish a feeding schedule that will make giving supplemental pancreatic enzymes a bit easier
• For a breast fed baby, keep track of how many times a day your baby is eating and how many wet diapers your baby has daily. This, along with weight gain, will help your CF Center dietitian know if your baby is getting enough breast milk
• For a formula fed baby, keep track of how many ounces of formula your baby is drinking with each feeding, how many wet diapers your baby has daily and how many times a day your baby is eating. Your CF Center dietitian will use the information to develop a nutrition plan
• The American Academy of Pediatrics (AAP) suggests that solid food be added to your baby’s diet at six months of age, but some babies may be ready for solids as early as 4 months. You can talk with your CF Center dietitian about adding solids
• Single-grain infant cereals (such as rice cereal) are a good first choice, with the gradual introduction of high calorie strained foods. Your CF Center dietitian can help you learn which strained foods are high in nutrition and calories

Important Facts for Your Toddler with CF

• A balanced diet, which includes optimal calories, vitamins and minerals, is key to good nutritional health for your toddler with EPI due to CF. Remember that your toddler with CF needs more calories, vitamins and salt than toddlers who do not have CF
• Toddlers with EPI due to CF need to take supplemental pancreatic enzymes every time they eat or drink anything that contains fat or protein
• Enzymes, vitamins and extra calories will help your toddler gain weight, grow and be healthy
• Toddlers who have CF need extra calories. Ways to add extra calories and nutrients include:

·         Whole milk at every meal. For extra calories add cream to the milk

·         Whole milk dairy products - like cottage cheese, yogurt and puddings - cream on cereal

·         Using healthy margarine or butter on hot foods, breads and crackers, and extra cheese in casseroles, on pizza and sandwiches puts more calories in the meal

·         Use whole grains and add fat such as margarine, butter, or cream cheese. Serve vegetables with margarine, butter, or salad dressing for dipping. Try ripe avocado as a treat. Dip fresh fruit in whole milk yogurt

·         Fat is the best source of calories for your toddler with CF. Each teaspoon of fat has 45 calories while protein and carbohydrates have 20 calories. So adding fats to all foods will increase the calories

·         Calcium is needed for healthy bones. According to the Institute of Medicine of the National Academies, a toddler needs about 700 mg of calcium daily, so including milk and other dairy products in your toddler’s diet is very important

• Toddlers who have CF need extra salt. Adding salt to food and providing salty snacks for your toddler will help replace the salt loss. You may need to add even more salt on hot and humid days if your toddler is very active. Talk with your dietitian about how much salt is best for your toddler.

Developing Positive Eating Habits in Toddlers with CF

• Set regular times for meals and snacks, so your toddler knows when to eat and can become familiar with a routine for mealtimes. It is recommended that your toddler eat in the same place for each meal or snack to help develop a feeding routine
• Limit drinks to meals and snacks. Drinking juice or milk from a sippy cup throughout the day can interfere with his or her appetite during snack or meal times. If your toddler is thirsty between snack and meals, offer water
• Toddlers can often become antsy and do not want to sit long enough to eat a full meal. By setting a good example and eating with your toddler, you can help them become a better eater
• When offering your toddler a drink or food, give them only healthy choices. For example, offer plain or chocolate milk or toast with cream cheese or butter. By doing this, your toddler can only make a good choice, yet feels they have some control over the choice
• Create a reward system such as a star chart for eating certain foods or for finishing a meal
• Let your toddler help with meal preparation. He or she can tear lettuce, rinse fruit or help to set the table

Important Facts for Your Child with CF

• A balanced diet, which includes optimal calories, vitamins and minerals, is important to good nutritional health for your child with EPI due to CF. Remember that your child with CF needs more calories, vitamins and salt than children who do not have CF
• Children with EPI due to CF need to take supplemental pancreatic enzymes every time they eat or drink anything that contains fat or protein
• Enzymes, vitamins and extra calories will help your child gain weight, grow and be healthy
• A child who has CF needs extra calories. To add extra calories and nutrition include:

·         Whole milk at every meal. For extra calories add cream to the milk

·         Whole milk dairy products - like cottage cheese, yogurt and puddings - cream on cereal

·         Using healthy margarine or butter on hot foods, breads and crackers, and extra cheese in casseroles, on pizza and sandwiches puts more calories in the meal

·         Use whole grains with added fat such as margarine, butter or cream cheese. Serve vegetables with margarine, butter or salad dressing for dipping. Try ripe avocado as a treat. Dip fresh fruit in whole milk yogurt. Spread peanut butter on toast, crackers and fruit

·         Fat is the best source of calories. Each teaspoon of fat has 45 calories while protein and carbohydrates have 20 calories. So adding fats to all foods will increase the calories

·         Calcium is needed for healthy bones. A child needs about 1,000-1,300 mg of calcium daily - about three or four servings of dairy products daily

• Children who have CF need extra salt to replace the salt lost in sweat throughout the day, so adding salt to food and planning salty snacks will help replace the salt loss. You may need to add even more salt on hot and humid days or if your child is very active. Talk with your dietitian about how much salt is best for your child

Developing Positive Eating Habits in Children with CF

• Set regular times for meals and snack, so your child knows when to eat and can become familiar with a routine for mealtimes. It is recommended that your child eat in the same place for each meal or snack to help develop a feeding routine
• Children can often become antsy and do not want to sit long enough to eat a full meal. By setting a good example and eating with your child, you can help him or her become a better eater
• Create a reward system such as a star chart for eating certain foods or for finishing a meal
• Let your child help with meal preparation. He or she can tear lettuce, rinse fruit or help to set the table
• If your child is a picky eater, pay attention to their favorite foods and adapt meals/menus as their food likes change. It is also helpful to keep food choices simple and make them as attractive as possible. Try making the food on your child’s plate colorful
• Be creative with foods. Make faces on pancakes, cut sandwiches in interesting shapes with cookie cutters, or use vegetables to make faces on mashed potatoes
• As children with CF get older, try to make meal planning and preparation part of your family’s weekly routine. Let each child make a dinner suggestion for a family meal. Fill in the foods such as vegetables or milk to make the meals balanced
• When your child with CF starts sleeping over at friends’ homes, speak with the friends’ parents about your child’s dietary needs. Encourage your child to make healthy choices when eating at their friends’ homes, and pack CF friendly snacks
• When going on vacation with a child with CF, call the hotel ahead of time to arrange for a refrigerator in your room, if available. If you are flying or have a long car trip, bring a bag of snacks such as crackers with peanut butter, muffins or sandwiches to help make the trip go smoothly

Healthy Bones in Children with CF

• It can be difficult for your child to absorb many essential nutrients from food, such as calcium and vitamins D and K, which are beneficial to build strong bones
• To optimize early bone mineral growth, your child should:

·         Intake 1,000 - 1,300 mg of calcium daily or about four servings of dairy products every day, such as whole milk, full-fat yogurt and full-fat cheese. For extra calcium, children can consume calcium-fortified cereals, breads and drinks

·         Eat foods high in zinc and protein, such as meats, eggs and nuts, to aid in bone growth

·         Participate in regular exercise and physical activity to help build more bone early in life. Activities that include running and jumping, such as tag, jumping rope, basketball and soccer, are especially good for developing strong, healthy bones. Keep in mind that exercise burns calories, so it is essential for your child to eat high calorie meals or snacks before and after physical activity.

Important Facts for Your Teen with CF

• Since the teen years are a time of rapid growth, a balanced diet, which includes optimal calories, vitamins and minerals, is important for good nutritional health for any teen with EPI due to CF. Remember that teens with CF need more calories, vitamins and salt than teens who do not have CF. Even if teens eat a diet that is well-balanced, they may also need to take additional vitamin supplements in order to maintain normal vitamin levels
• In addition, teens with EPI due to CF need to take supplemental pancreatic enzymes every time they eat or drink anything that contains fat or protein. It may be helpful to keep a supply of enzymes wherever you go
• The teen years can be a very busy time with many school and social activities, so getting the necessary daily nutrients can be a challenge. Keeping a supply of healthy snacks in a backpack or locker is a good idea. Examples of healthy snacks include: nuts, trail mix, packaged peanut butter or cheese crackers, canned shakes, or granola bars
• Examples of protein sources include:  meat and meat products, milk and milk products, fish, seafood, soy products such as tofu, beans, eggs and nuts

·         Foods high in protein are often rich in vitamins, minerals and fat

• Additional iron is needed during puberty when muscles and blood volume increases. Meats are the best source of iron
• It can be a challenge to include fruits and vegetables in a high calorie diet. Think about topping ice cream, yogurt, or pudding with fresh fruit. Add vegetables to pizza, lettuce and tomato to sandwiches, and shredded carrots to spaghetti sauce
• Calcium is needed for healthy bones and teens may need 1,300 – 1,500 mg of calcium daily - about four servings of dairy products every day

·         Whole milk and whole milk products, cottage cheese and yogurt have a lot of calories and calcium

• Blend whole milk into milkshakes, melt butter over cooked vegetables, or eat snacks like fresh celery and carrots with a creamy dip
• Fat is the best source of calories. Each teaspoon of fat has 45 calories while protein and carbohydrates have 20 calories. So adding fats to all foods will increase the calories
• Teens who have CF need extra salt to replace the salt lost in sweat throughout the day, so adding salt to food and planning salty snacks will help replace the salt loss. Some teens enjoy eating Chinese noodle soups, pickles, pretzels or chips as a way to get extra salt. You may need to add even more salt on hot and humid days or if you are very active. Talk with your dietitian about how much salt is best for your lifestyle

Developing Positive Eating Habits in Teens with CF

• Encourage your teen to make healthy choices when eating at their friends’ homes, and pack CF friendly snacks
• When going on vacation with a teen with CF, call the hotel ahead of time to arrange for a refrigerator in your room, if available. If you are flying or have a long car trip, bring a bag of snacks such as crackers with peanut butter, muffins or sandwiches to help make the trip go smoothly
• As your teen starts becoming more independent, make an effort to involve him or her in meal preparation including: planning meals, buying foods and cooking. Take your teen to the supermarket and have them participate in shopping for groceries. They may enjoy watching the cooking shows on TV and find recipes online to make for the family

Healthy Bones in Teens with CF

• Having strong, healthy bones is essential for overall good health. Strong bones require enough minerals such as calcium, phosphorus, magnesium and fluoride. When a person does not get enough of these minerals it may lead to either osteoporosis or osteopenia, which means the bones are not as dense as they should be
• Maintaining a well-balanced diet and developing eating habits that build strong bones is especially important for teens entering puberty, a crucial stage in the body’s bone development
• To prevent bone density loss, teens should:

·         Intake 1,300 – 1500 mg of calcium daily or about four servings of dairy products every day, such as whole milk, full-fat yogurt and full-fat cheese. For extra calcium, teens can consume calcium-fortified cereals, breads and drinks, as well as add powdered dry milk to recipes

·         Eat food rich in fat-soluble vitamins D, K and A, which help the body absorb calcium and therefore increase bone density. Egg yolks and fatty fishes, such as salmon and tuna are a good source of these key vitamins

·         Take vitamin supplements designed for people with CF to help build healthy bones

·         Choose foods high in zinc and protein, such as meats, eggs and nuts, to aid in bone growth and maintenance of healthy bone tissue

·         Avoid caffeinated and carbonated drinks like soda and coffee, as they may contribute to lower bone density and do not contain the calcium and vitamin D necessary for bone production

·         Engage in regular exercise and physical activity to help build more bone early in life. A combination of weight-bearing and resistance exercises, like running, walking and weight lifting, are important to good bone health. However, exercise burns calories, so it is essential to eat high calorie meals or snacks before and after physical activity. If your teen is not accustomed to regular physical activity, talk to your CF center care team about building an exercise program to address their health needs

 Important Facts for Adults with CF

• A balanced diet which includes optimal calories, vitamins and minerals is important for good nutritional health for any adult with EPI due to CF. Remember that you need more calories, vitamins and salt than adults who do not have CF. Even if adults eat a diet that is well-balanced, they may also need to take additional vitamin supplements in order to maintain normal vitamin levels
• Adults with EPI due to CF need to take supplemental pancreatic enzymes every time they eat or drink anything that contains fat or protein. It may be helpful to keep a supply of enzymes with you wherever you go
• Examples of protein sources include: meat and meat products, milk and milk products, fish, seafood, soy products such as tofu, beans, eggs and nuts

·         Food high in protein is often rich in vitamins, minerals and fat

• Additionally, adults should plan their meals ahead for work, parenting and other responsibilities

·         Cook once and eat twice. Make extra servings of meals (such as lasagna) to eat the next day or freeze for later in the week

·         Buy foods in bulk and freeze it in serving sizes that can be defrosted or reheated for a quick snack or meal

·         Keep foods such as nuts, trail mix, pretzels, or canned shakes in your car, desk drawer, or backpack so you always have an available quick snack

• It can be a challenge to include fruits and vegetables in a high calorie diet. Think about topping ice cream, yogurt, or pudding with fresh fruit. Add vegetables to pizza, lettuce and tomato to sandwiches, and shredded carrots to spaghetti sauce
• Calcium is needed for healthy bones and adults may need 1,000 mg of calcium daily - about three or four servings of dairy products every day

·         Whole milk and whole milk products, cottage cheese and yogurt have a lot of calories and calcium

• Fat is the best source of calories. Each teaspoon of fat has 45 calories while protein and carbohydrates have 20. So adding fats to all foods will increase the calories
• Adults who have CF need extra salt to replace the salt lost in sweat throughout the day, so adding salt to food and planning salty snacks will help replace the salt loss. Some adults enjoy eating Chinese noodle soups, pickles, pretzels or chips as a way to get extra salt. You may need to add more salt on hot and humid days or if you are very active. Talk with your dietitian about how much salt is best for your lifestyle

Developing Positive Eating Habits in Adults with CF

• Plan your meals the night before or in the morning and pack a lunch and snacks you can eat while on the go
• Prepare in advance for times when you are very busy at work or college; keep healthy and nutritious snacks in a refrigerator or freezer in your office or dorm
• When going on vacation, call the hotel ahead of time to arrange for a refrigerator in your room, if available. If you are flying or have a long car trip, bring a bag of snacks such as crackers with peanut butter, muffins or sandwiches to help make the trip go smoothly
• If you are planning to eat out for meals, see if the restaurant has a menu online prior to going. You can plan what you are going to eat before you get to the restaurant

Healthy Bones in Adults with CF

• Having strong, healthy bones is essential for overall good health. Strong bones require enough minerals such as calcium, phosphorus, magnesium and fluoride. When a person does not get enough of these minerals it may lead to either osteoporosis or osteopenia, which means the bones are not as dense as they should be
• By following a well-balanced diet and maintaining an ideal body weight, you may be able to  prevent bone density loss and maintain healthy bone tissue
• To optimize bone health through nutritional habits, adults should:

·         Intake 1,000 – 1300 mg of calcium daily or about three or four servings of dairy products every day, such as whole milk, full-fat yogurt and full-fat cheese. For extra calcium adults can consume calcium-fortified cereals, breads and drinks, as well as add powdered dry milk to recipes

·         Eat food rich in fat-soluble vitamins D, K and A, which help the body absorb calcium and therefore increase bone density. Egg yolks and fatty fishes, such as salmon and tuna, are a good sources of these key vitamins

·         Take vitamin supplements designed for people with CF to help maintain healthy bones

·         Choose foods high in zinc and protein, such as meats, eggs and nuts, to aid in bone growth and the maintenance of healthy bone tissue

·         Avoid caffeinated, alcoholic and carbonated drinks like soda and coffee, as they may contribute to lower bone density and do not contain the calcium and vitamin D necessary for bone production

·         Engage in regular exercise and physical activity to help build and maintain strong bones.  A combination of weight-bearing and resistance exercises, like running, walking and weight lifting, are important for good bone health. However, exercise burns calories, so it is essential for you to eat high calorie meals or snacks before and after physical activity. If you are not accustomed to regular physical activity, talk to your CF center care team about building an exercise program to address your health needs

For Recipes Please visit the next Blog (Recipes for Cystic Fibrosis)