Top Cystic Fibrosis Diet Tips for Children That Boost Health and Growth

Cystic fibrosis (CF) is a genetic disorder that primarily affects the lungs and digestive system. It is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, leading to the production of thick and sticky mucus that can clog organs, particularly the lungs and pancreas. In the pancreas, thickened secretions obstruct the intra-pancreatic ducts, reducing the delivery of digestive enzymes to the intestines and impairing the absorption of key nutrients. Due to malabsorption caused by pancreatic insufficiency (PI), patients with cystic fibrosis (CF) are prone to malnutrition and growth failure. Optimal nutritional care and support should be an integral part of the management of the disease, with the aim of ameliorating clinical outcomes and life expectancy. Therefore, children with CF require specific dietary recommendations to ensure adequate growth, development, and overall health. This article provides a comprehensive overview of these dietary recommendations, encompassing various age groups and nutritional needs.

Nutritional Goals and Early Intervention

The nutrition support goal in CF care should begin as early as possible after diagnosis and include the achievement of an optimal nutritional status to support the growth stages and puberty development in children, that will further support the maintenance of an optimal nutritional status in adult life. Newborn screening has had a profound impact on nutrition outcomes. As a matter of fact, infants diagnosed with CF via newborn screening, and children introduced to nutritional therapy at an earlier age, have a greater growth than those diagnosed later in infancy and childhood. Early diagnosis through newborn screening is crucial, as it allows for prompt intervention and improved long-term outcomes. The assessment of anthropometric parameters reflecting the nutritional status are recommended as part of routine CF care, with the aim of achieving a nutritional condition comparable to that of healthy children. Indeed, this practice is supported by evidence that an early diagnosis and then prompt intervention are effective in determining a better quality of life and in improving the lung function and growth parameters.

General Dietary Principles

The cornerstone of nutrition in patients with CF is a high calorie, high-fat diet, in conjunction with a better control of malabsorption due to pancreatic enzyme replacement therapy, and attention to the adequate supplementation of fat-soluble vitamins. Children with CF need a diet high in calories to support their growth and development. With extra calories, most children with CF are able to grow and develop normally. Children with CF may need 2,000-2,800 calories daily. They require more calories than children without CF to maintain weight and slow the decline in lung function. They should eat 3 meals and 2 to 3 snacks a day to help meet their calorie needs. Try for 3 meals plus 2 to 3 snacks a day. Adding extra fat to meals and snacks can help meet these needs. It is important to remember that a balanced diet is vital for the whole family. This includes dairy products; grains and starches; fruits and vegetables; and proteins such as meat, poultry, fish, eggs and peanut butter.

High-Calorie Foods

To maintain weight and slow the decline in lung function, your child will need to have more calories than a child without CF. The amount of calories needed is even higher when your child is ill or has an infection. Here are examples of high-calorie foods to add to your child’s diet:

Butter
Cheese
Nuts
Avocado
Eggs
Whole milk (full-fat)
Peanut butter

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. 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.

Read also: CF Diet for Adults

High-Fat Diet

CF patients should get about 35 to 40 percent of their calories from fat. This is more than the recommendation for the general public. 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 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.

Salt Intake

Toddlers with CF lose more salt when they sweat than toddlers who don't have CF. Patients with CF need to have a high salt diet. Children with CF need extra salt in their diets. This is because they lose more salt in their sweat than other children. Add extra salt by using the salt shaker at meals and including salty foods and snacks. These include chips, pretzels, fast foods, French fries, pickles, nuts, and lunch meats. Extra salt intake is important in the hot weather, when children are playing outside and sweating. 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.

Pancreatic Enzyme Replacement Therapy (PERT)

The obstructions in the pancreatic ducts damage the transport of the digestive enzymes and pancreatic bicarbonate to the intestinal lumen. This leads to exocrine PI, poor absorption of fat-soluble vitamins (A, D, E and K) and acidifies intestinal lumen. For this reason, most children with CF need replacement pancreatic enzymes before every meal and snack. These enzymes help the body digest carbohydrates, protein, and fat, which promotes better absorption of nutrients. Your CF dietitian or care provider also will help decide what kind of, and how many, enzymes your child needs. Enzyme dose is based on weight, amount eaten, bowel movements, growth and weight gain.

Administration Guidelines

The health care provider prescribes these enzymes, which come in capsule form. Here are some general guidelines for giving your child enzymes:

For younger children, open capsules and sprinkle the contents on an acidic food, such as applesauce or pears. These enzymes don’t taste bad. Ask your child not to chew the sprinkles, because this will keep them from working well. To be sure your child takes them all, put the sprinkles on a small amount of food and watch your child eat them. Some toddlers don't want food and enzymes mixed together. If you are having this trouble, try giving your toddler just the beads in a spoon or small medicine cup before meals and snacks. Enzymes should not be chewed or crushed. If your toddler refuses enzymes, try giving him some choices and control. Let him open up the capsules and pour the beads out.
Older children can choose to swallow the capsules whole with liquid.
Have your child take the capsules with every meal and snack.
Talk with your provider to figure out the correct dosage of pancreatic enzymes your child should take.
Make sure your child brings the enzymes to school and takes them with lunch and snacks. You can help with meals away from home by sending the enzymes and the acidic food (such as applesauce or other fruit) to mix them with.
Your child must take enzymes with liquid nutritional supplements, too.

Vitamin and Mineral Supplementation

Vitamin supplements are crucial because of the malabsorption of vitamins in people with CF. Vitamin supplements help meet your child's nutrition needs and prevent vitamin shortages, especially of the fat-soluble vitamins A, D, E and K. When fat is not absorbed, fat-soluble vitamins are not absorbed. Your toddler may need a prescription for special multivitamins that have more easily absorbed forms of vitamins A, D, E, and K. Supplementing with vitamins It's harder for a child with cystic fibrosis to get needed nutrition from foods alone. Your child may need to take vitamins once or twice a day, as directed by the health care provider. These include fat-soluble vitamins, such as vitamins A, D, E, and K. Vitamins are available in drops or as chewable tablets. Nutritional supplements, such as calcium, multivitamins, and sodium may also be advised when healthy eating is not enough. Ask your child’s provider how much to give your child and to suggest brands that are easy to absorb.

Specific Vitamins and Minerals

Vitamin A: Plays an important part in vision, healthy bones and the immune system.
Vitamin D: Helps to build and maintain strong bones by keeping the right amount of calcium and phosphorus in the blood. 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.
Iron and Calcium: Your child will need additional iron and calcium during puberty and when their bones are growing.
Zinc: Zinc is also important to your child's growth and wound healing ability. It also helps the immune system work correctly.

Discuss your child's vitamin and nutritional supplement plans with the healthcare provider and CF dietitian.

Infant Feeding (0-12 Months)

After the first year, most children do not need to rely on infant formula or breast milk to achieve good nutrition. Give whole milk at every meal. At this point, children can have whole milk to drink. Limit the amount of water. Limit juice to 4 ounces per day until your child is 6 years old. It does not have the same fat and calories that are found in whole milk.

Introducing Solids

At this age, your child can eat any food, as long as it is in a form that is safe for him to chew and swallow. This includes soft and ground meats, chopped finger foods, soft-cooked vegetables, and well-cooked pasta. Let your child use a child-sized spoon. He can practice holding it as you feed him. The more practice, the faster he will learn to eat on his own. Be sure to offer small safe pieces of finger foods and supervise your child so he doesn't choke.

Meal and Snack Schedule

Breakfast gets the body ready for the rest of the day. You can offer three different types of foods for lunch and dinner. Offer two to three snacks a day. Always serve snacks that require enzymes so your child can get the high-calorie and high-fat diet he needs to grow.

Toddler and Preschool Years (1-5 Years)

Continue to offer a variety of nutrient-dense foods, focusing on high-calorie and high-fat options.

Read also: The Hoxsey Diet

Managing Eating Behaviors

Try not to let your child “graze” (eating and drinking small amounts throughout the day). Allowing your child to eat outside of regular meal and snack times can lead to many challenges. It makes it harder to give enzymes if he is always eating. Your child will also not be as hungry during meals. If your child with CF eats all day, he or she will not be hungry at mealtime. Give your child some control over food. Don't force-feed or pressure a child to eat new foods. Lead by example and try new foods yourself.

School-Aged Children and Adolescents

If your child is in daycare, plan high-calorie meals and snacks with the daycare center staff. Tell them why your child needs extra calories. You may have to send extra high-calorie foods, if the daycare menu can't be changed. 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. 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. Children with CF may need additional bathroom breaks, especially if their malabsorption is not well controlled.

Addressing Specific Nutritional Challenges

Poor Weight Gain

Children with cystic fibrosis (CF) often have poor weight gain. This can happen even when they get enough calories. Infections, breathing problems, and the body's inability to take in certain nutrients (malabsorption) can all lead to the need for extra calories. If your child doesn’t gain weight or is behind on height, special liquid supplements may help add calories. If weight is still a problem, a nighttime tube feeding may be needed. Tell the healthcare provider if feeding your child butter, sour cream, cream cheese, or other fatty foods leads to symptoms of malabsorption (see below).

Malabsorption

Sometimes children with CF don't grow or gain weight even with a high-calorie diet. This can happen if your child's appetite is poor, they have frequent infections, or your child has malabsorption. If your child can't eat enough food for normal growth and development, then they might need supplemental oral or tube feedings, as directed by your child's healthcare provider.

Tube Feeding

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. 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. 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. Your CF team can help you decide your tube feeding schedule. You should keep your tube only as long as you need it. If you think a tube might be the right choice for you, discuss it with your CF team.

Exercise and Fluid Intake

Exercise is essential for both kids and adults with cystic fibrosis. 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. The extra salt that individuals with CF lose during exercise should be replaced with salty foods like potato chips. 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.

Strategies for Picky Eaters

Putting too much emphasis on food can be overwhelming for your child. To avoid turning eating into a power struggle, try these tips:

Make food as appealing as possible.
In an age-appropriate manner, educate your child about cystic fibrosis. Encourage them to ask questions during health care visits.
Talk with teachers and school staff about cystic fibrosis and your child's unique needs. Seek reasonable accommodations that meet your child's needs.
Encourage your child to eat what they can comfortably. Don't pressure your child to eat more than they are able.
Don’t force your child to eat. Big portions can be hard to eat at one sitting. Try for smaller meals more often.
Keep to a schedule, but don’t make your child stay at the table for more than about 20 minutes at a time.
Remove distractions. Don't allow electronic devices, television viewing, toys, books, or games while eating.
Enjoy being together. Relax and sit at the table with your child.
Give younger children a few simple food choices.

Positive Mealtime Strategies

Nutrition is important with CF, because good nutrition is linked to healthier lungs. But the focus on nutrition can sometimes cause stress and arguments at the dinner table.

Keep mealtimes to less than 30 minutes.
Eat in a comfortable environment without distractions (TV, tablets, phones).
Praise your child for positive behaviors, like taking bites of a non-preferred food or finishing a portion. Praise siblings too.
Ignore negative behaviors like complaining, stalling, or outbursts.
Consider a simple reward system, like a sticker chart, to reinforce positive mealtime behaviors. Your child can use the chart to earn a reward or privilege for following mealtime rules (for example, skipping a chore, getting extra screen time, or doing a fun activity with mom or dad).

Introducing New Foods

Often, kids need to taste and try new foods over and over again before they like them. It may take your child a few tries to accept a new food - that's normal!

Choose one food you want your child to try.
Offer the new food at one meal, and put just one bite of it on your child's plate.
Explain to your child what you expect them to do: You want them to eat their regular meal, plus this one bite.
Praise your child for eating, especially for trying the new food.
At the next few meals, as your child begins to get used to it, slowly increase the amount of the new food.
Repeat this process with each new food you serve.

Increasing Calorie Intake

Some kids with cystic fibrosis need more calories than their peers.

Avoid sugary drinks, which can decrease appetite. Instead, have your child drink three servings of whole milk per day and stay hydrated between meals and snacks with water.
Provide structure around eating. Create a meal/snack schedule at home that the whole family follows. Kids who eat all day long tend to take in fewer calories rather than more because they never build up a strong appetite.
Make sure snacks are just as healthy and high-calorie as meals. Offer high-calorie snacks like trail mix, full-fat yogurt with granola, fruit with peanut butter, or whole-grain pita chips with hummus. Avoid low-calorie, low-nutrition snacks like fruit snacks, pretzels, or snack cakes.
If your child isn't hungry for breakfast, consider a high-calorie beverage instead, such as chocolate whole milk, Carnation Breakfast Essentials, or a high-calorie smoothie. Sometimes drinking calories is easier than eating them.

Adding Extra Fat

Sometimes the amount of food a child eats isn't enough to meet calorie goals. The easiest way to add extra calories is by adding extra fat to the food your child already eats.

Add extra butter or oil to pasta, rice, potatoes, or cooked vegetables.
Pair raw vegetables with salad dressing or hummus.
Top salads and sandwiches with avocados or guacamole.
Grill sandwiches in butter or margarine.
Add a splash of heavy cream to milk, oatmeal, smoothies, or cream-based soups.

Examples of Healthy Calorie and Fat Additions

There are many easy ways you can help your child with cystic fibrosis add healthy calories and healthy fats to their diets.

Peanut butter: There are so many ways to eat peanut butter: in a sandwich, on toast, on crackers and - our favorite! - in oatmeal. Oatmeal is a fiber-rich food, and peanut butter will add flavor and creamy texture once melted.
Olive oil: Have you ever gone to a restaurant that serves delicious breads like focaccia accompanied by olive oil that has been seasoned with parmesan cheese? It’s a delicious and easy way to get calories and enjoy a great combination of foods and bold flavors. Olive oil can also be used in cooking to add extra calories.
Avocadoes: Avocadoes can be eaten on their own with seasonings (like salt!) or with many foods, like quesadillas and nachos. They can also be made into guacamole that you can serve with tortilla chips or put in sandwiches. Avocadoes provide unsaturated fat as well as important vitamins and minerals.

When to Contact Your Child’s Doctor

Contact your child's health care provider right away if your child has any of these:

Constipation, belly (abdominal) pain, or other signs of an intestinal blockage
Weight loss or decrease in appetite
2 to 3 loose stools a day, or a change in the size or consistency of stools
New symptoms or symptoms that get worse
Fever of 100.4°F (38.0°C) or higher, or as directed by your child's provider
Increase in coughing, chest congestion, or change in the color of the sputum
Wheezing
Sudden shortness of breath
Chest pain or chest tightness
Coughing up blood from the lungs

Monitoring and Assessment

Once CF is diagnosed, the assessment of anthropometric parameters reflecting the nutritional status are recommended as part of routine CF care, with the aim of achieving a nutritional condition comparable to that of healthy children. For infants and children younger than two years of age, weight, height and weight-for-length percentiles should be used to evaluate growth. The longitudinal growth trajectory should also be used as a benchmark. In children from two to 18 years of age, the nutritional status may be assessed by the BMI. However, it is essential to focus special attention to the nutritional status during the first 12 months after the diagnosis of CF, throughout both the first year of life and the prepuberty period. ESPEN-ESPGHAN guidelines also recommend the annual monitoring of the biochemical markers of malnutrition, including blood count, iron status, plasma fat-soluble vitamin levels, serum liver function and electrolyte measurements. In addition to the BMI, the assessment of the body composition should be considered, with the aim of reaching the target BMI, by increasing muscle mass instead of fat mass. All CF patients should undergo oral glucose tolerance testing yearly, beginning from 10 years of age. Part of the nutrition status evaluation should be a dietary review, at least every 3 months for children and adolescents, using questions about the adherence to dietary advice. Growth charts help your child’s care team track your child’s growth. At 24 months, your child’s care team will measure her or his growth using a different growth chart than they did when your child was younger. Because each chart is different, your child’s percentiles may change.

The Role of CFTR Modulators

In the last decade, the therapeutic possibilities towards CF have grown in a consistent way. The positive effects of CFTR modulators on nutritional status mainly consist in the improvement in weight gain and BMI, both in children and adults, and in an amelioration in terms of the pulmonary function and reduction of exacerbations. Several challenges need to be overcome with the development of new drugs, to transform CF from a fatal disease to a treatable chronic disease with specialized multidisciplinary care.

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