Dietary Recommendations for Cystic Fibrosis

Eating well is vital for people with cystic fibrosis (CF). 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.

Understanding Cystic Fibrosis

Cystic fibrosis (CF) is a life-threatening genetic disease that causes thick, sticky mucus to build up in the lungs and digestive tract. Millions of Americans carry the cystic fibrosis, or CF gene. Fortunately, most of them don't have cystic fibrosis. That's because you need to inherit one faulty gene from each parent to actually get the disease.

Genes are the coded instructions that tell our bodies how to operate. Usually, the code is correct and everything runs smoothly. But sometimes, the code is incorrect because of a defective gene or genes. In the case of cystic fibrosis, a faulty gene causes the body to produce an abnormally thick, sticky fluid called mucus. This mucus clogs the lungs, making it hard to breathe. It also gets stuck in the pancreas, making it harder for the body to break down and digest food. Parents can pass all kinds of different traits to their children, from blue eyes to blonde hair. Sometimes, parents can also pass the genes for certain diseases to their kids. Cystic fibrosis is one very serious inherited disease that makes it hard for children to breathe and digest food.

How CF Affects Digestion

Cystic fibrosis causes a build-up of mucus that blocks the ducts in the pancreas and means digestive juices containing enzymes do not reach the stomach and therefore food is not broken down properly. The pancreas is an organ in the abdomen behind the stomach. An important job of the pancreas is to make enzymes. These enzymes help the body digest and absorb protein and fats. A buildup of sticky mucus in the pancreas from CF can lead to serious problems, including:

• Stools that contain mucus, are foul smelling, or float
• Gas, bloating, or distended belly
• Problems getting enough protein, fat, and calories in the diet
• Problems absorbing enough protein, fat, and calories from the diet

Because of these problems, people with CF may have a hard time staying at a normal weight. Even when weight is normal, a person may not be getting the right nutrition. Children with CF may not grow or develop correctly.

Read also: The Hoxsey Diet

General Dietary Recommendations

People with cystic fibrosis should have a balanced diet although many will require more energy (calories), to maintain a healthy body weight. A healthy, balanced diet for people with cystic fibrosis supplies energy and nutrition. This is important for normal growth and development.

Dietary and nutritional needs will vary according to age, weight, height, symptoms, lung function and activity level. For some people with CF it can be very difficult to maintain a healthy weight. Having CF can increase the body’s energy needs by 150-200% or even higher if lung function drops or if the body is fighting an infection. Some people with CF, especially those on modulators such as Kaftrio, or those who do not have pancreatic insufficiency, may not struggle to keep weight on. Some people may find they put weight on easily or some may find they maintain weight. In recent years nutrition has come to the forefront as a successful health strategy to improve the lives of people with cystic fibrosis.

Calorie and Macronutrient Intake

People with CF need to eat foods that are high in calories and protein throughout the day. 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.

• Calories: People with CF need to consume lots of calories. Good nutrition and maintaining a good body weight have a huge impact on the quality of life and lifespan of people with CF. They need to eat more calories than people without CF and include a range of high-fat, high-salt food in their diet.
• Fat: 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.
• Protein: Protein should make up about 16 to 20 percent of your calories. Protein is one of the three kinds of nutrients that provide calories in your diet. Proteins are the building blocks in your body, and are the main components of muscle, organs and glands. Every living cell and all body fluids, except bile and urine, contain proteins.
• Fiber: Fiber is a carbohydrate that your body does not digest. Fiber is present in all plants. Adults should get about 25 to 30 grams of fiber a day.

Specific Food Recommendations

People with CF should eat plenty of:

• Fatty foods e.g. milk and dairy products
• Starchy foods
• Protein foods
• Vitamin/mineral rich foods

Here's a recommended diet for someone with cystic fibrosis:

Read also: Walnut Keto Guide

• Add grated cheese to your meals.
• Drink whole milk. Use enriched milk in cooking.
• Eat peanut butter. Put it on bread, use it for a dip, and cook with it.
• Put a teaspoon of butter on hot foods.
• Dab sour cream or yogurt on vegetables.
• Eat breaded meat, chicken, and fish.

The following are ways for adding protein and calories to the diet. Be sure to follow other specific instructions from your health care provider.

• Add grated cheese to soups, sauces, casseroles, vegetables, mashed potatoes, rice, noodles, or meat loaf.
• Use whole milk, half and half, cream, or enriched milk in cooking or beverages. Enriched milk has nonfat dry milk powder added to it.
• Spread peanut butter on bread products or use it as a dip for raw vegetables and fruit. Add peanut butter to sauces or use on waffles.
• Skim milk powder adds protein. Try adding 2 tablespoons (15.6 grams) of dry skim milk powder in addition to the amount of regular milk in recipes.
• Add marshmallows to fruit or hot chocolate.
• Add raisins, dates, or chopped nuts and brown sugar to hot or cold cereals, or have them for snacks.
• A teaspoon (5 g) of butter or margarine adds 45 calories to foods. Mix it into hot foods such as soups, vegetables, mashed potatoes, cooked cereal, and rice. Serve it on hot foods. Hot breads, pancakes, or waffles absorb more butter.
• Use sour cream or yogurt on vegetables such as potatoes, beans, carrots, or squash. It can also be used as a dressing for fruit.
• Breaded meat, chicken, and fish have more calories than broiled or plain roasted.
• Add extra cheese on top of frozen prepared pizza.
• Add coarsely chopped hard cooked egg and cheese cubes to a tossed salad.
• Serve cottage cheese with canned or fresh fruit.
• Add grated cheeses, tuna, shrimp, crabmeat, ground beef, diced ham or sliced boiled eggs to sauces, rice, casseroles, and noodles.

Salt Intake

Teens with CF lose more salt in their sweat than their peers. The dietitian may suggest adding extra salt to foods, eating salty snacks, and drinking sports drinks when teens exercise or spend time outdoors in hot weather. Salt is a mineral (sodium chloride) that can be added to foods to change their taste. Any food that tastes salty, like chips, pickles, fast food, frozen dinners and french fries is a good source of salt. Salt is essential for muscle function, digestion, and nerve cell health. It also helps move fluid in and out of cells. CF patients need to have a high salt diet. 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.

Fluid Intake

Every cell in the body needs water to work properly. You can get most of the water you need from what you drink, but you also can get water from many foods. The amount of water you need depends on your age, weight, activity level, general health and the climate. Do not wait until you feel thirsty to drink water. Some symptoms of dehydration are headache, nausea, loss of appetite, and fatigue. You also can tell if you are dehydrated by looking at the color of your urine. Urine should be clear or a pale yellow. If your urine is dark yellow or brownish, you are not getting enough fluids. Fluid is important for a balanced diet and for hydration. Losing extra salt and water in your sweat increases the chance of dehydration, especially in the summer. Water is an excellent choice to maintain fluid balance. Milk is also a good choice because it provides calories and protein, in addition to fluid.

Enzymes and Vitamin Supplementation

Most people with CF need to take enzymes before they eat to help their body digest food.

Pancreatic 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. Teens with pancreatic insufficiency might have problems with growth and weight gain. They also might have frequent and bad-smelling bowel movements. They'll need to take prescribed enzymes with meals and snacks to help them digest food and keep a healthy weight. A CF doctor will work with a dietitian to prescribe enzymes based on weight, growth, and bathroom habits. Enzymes should be taken with all foods and drinks that have fat and protein. Enzymes should never 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. Consider taking an extra enzyme cap for large meals or fast food meals that are extra fatty.

Read also: Weight Loss with Low-FODMAP

Most people with CF must take pancreatic enzymes. These enzymes help your body absorb fat and protein. Taking them all the time will decrease or get rid of foul-smelling stools, gas, and bloating. Take enzymes with all meals and snacks. Talk to your provider about increasing or decreasing your enzymes, depending on your symptoms.

Vitamins

Some vitamins, such as A, D and E are fat-soluble, making them harder for people with CF to absorb. Besides eating a healthy diet, people with CF may also have to take a CF-specific vitamin or multivitamin supplement. Supplements that include these, and other vitamins, are often useful for people with CF. Bone strength can be affected by the poor uptake of minerals such as calcium and phosphorus, which are directly related to vitamin D levels. Ask your provider about taking extra vitamins A, D, E, K, and extra calcium. There are special formulas for people with CF.

Vitamins and minerals are very important in a healthy diet. These nutrients help build strong bones and fight infections. It is very difficult to get the proper amount of the following vitamins from foods, which is why your CF team recommends a special multivitamin that is easily absorbed.

• Vitamin A: Found in egg yolks, milk, liver and fortified cereals. Vitamin A plays an important part in vision, healthy bones and the immune system.
• Vitamin D: Found in fortified milk, liver, salmon and fortified cereals. 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. Vitamin D helps to build and maintain strong bones by keeping the right amount of calcium and phosphorus in the blood.

Special Considerations

In the past, a healthy diet for someone with cystic fibrosis was high-calorie, high-fat, and high-salt. But new therapies make weight gain easier and life expectancy longer. So the focus of a healthy diet is changing. It's becoming more important to consider how diet can keep you feeling healthy as you grow older. Healthy diet choices can help prevent other conditions, such as heart disease or cancer, while improving your quality of life.

Cystic Fibrosis-Related Diabetes (CFRD)

When people have cystic fibrosis-related diabetes (CFRD), it is important to keep blood sugar levels as normal as possible and get enough calories.

Weight Management

For some people with CF, a healthy diet and enzyme replacement is enough to maintain a healthy weight. For others, maintaining or gaining weight may be difficult. For example, people with advanced lung disease need a high-calorie, high-fat diet. If you need help gaining weight, it’s important to include more high-calorie, high-fat foods in your diet to stay healthy. Sometimes high-calorie food choices are not enough to maintain a healthy weight. Oral nutrition supplements can help meet high calorie needs. Tube feeding may also be used to ensure calorie needs are met and a healthy weight is reached.

As the use of CFTR modulator therapy has improved the health of many people with CF, some people have found that they have gained unwanted weight by following a traditional CF diet that is high in calories, fat, and salt. The goal for good health is to achieve and maintain a healthy body weight.

Tube Feedings

Some teens with CF may have trouble gaining weight from a high-calorie diet alone. A doctor or dietitian may recommend adding nutrition supplements to your diet. Nutrition supplements are calorie-rich drinks that also have protein, vitamins, and minerals. For some teens, 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 may recommend tube feedings. These feedings, done through a tube into the stomach, are often given at night for extra calories. These overnight feedings leave teens with CF free to enjoy normal meals and activities during the day. Making the decision to get a feeding tube can be hard, but many teens and families express relief afterward. Tube feedings can help teens gain weight, while easing the stress and conflict around food and eating. Having a tube put in for the feedings usually is a simple procedure that doesn't need extensive surgery or a long hospital stay.

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.

Additional Tips and Considerations

• Eating Patterns: Eat whenever you are hungry. This may mean eating several small meals throughout the day. Keep a variety of nutritious snack foods around. Try to snack on something every hour, such as cheese and crackers, muffins, or trail mix. Try to eat regularly, even if it is only a few bites. Or, include a nutrition supplement or milkshake. Be flexible. If you aren't hungry at dinner time, make breakfast, mid-morning snacks, and lunch your main meals.
• Exercise: Besides the right diet, exercise can help support better lung health, mood, and quality of life. People with CF need to consume lots of calories. You can add calories to your diet without spending a lot of money on commercial products. 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.
• Snacking: People with CF should have 2 to 3 snacks per day. 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.
• School Considerations: 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.
• Financial Assistance: Many people with CF need help paying for food. There are local and national programs that can help. Talk with your CF care team or call CF Foundation Compass to find resources that can meet your needs.

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