Cystic fibrosis (CF) is a genetic disorder impacting the epithelial cells, notably in the sweat glands, lungs, and pancreas. This cellular malfunction disrupts the body's salt and water equilibrium, leading to the production of thick mucus. This mucus obstructs the lungs and other passages, triggering infections and respiratory issues. Nutrition plays a vital role in managing CF, given its association with heightened energy expenditure, specific nutritional deficiencies, and malabsorption, primarily stemming from pancreatic insufficiency.
Understanding Cystic Fibrosis and Its Impact on Nutrition
Cystic fibrosis affects the body's epithelial cells, disrupting the balance of salt and water. This leads to the production of thick mucus, which can block the lungs and intestines. This mucus can also prevent the intestines from absorbing important nutrients like fat and vitamins from food, which means that teens with CF may be short and underweight for their age, and they may get sick a lot because their bodies can't fight infections well. Individuals with CF often require more calories and nutrients compared to their peers to combat infections and sustain robust lung function.
Pancreatic Insufficiency and Enzyme Replacement
CF frequently results in pancreatic insufficiency, hindering the passage of fat, carbohydrate, and protein-digesting enzymes from the pancreas to the intestines. This impairs normal food digestion, potentially causing growth and weight gain problems, alongside frequent, foul-smelling bowel movements. Prescribed enzymes taken with meals and snacks aid in food digestion and maintaining a healthy weight. The CF doctor collaborates with a dietitian to tailor enzyme prescriptions based on individual needs. 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, so they should be stored in a cool, dry place.
Nutritional Needs for Individuals with Cystic Fibrosis
While past dietary recommendations emphasized high-calorie, high-fat, and high-salt intake, contemporary approaches prioritize a balanced diet that supports long-term health and well-being. Healthy diet choices can help prevent other conditions, such as heart disease or cancer, while improving your quality of life.
Macronutrient Recommendations
- Protein: Include protein with meals and snacks. Aim for 15-20% of daily calories from sources like meat, fish, eggs, cheese, soy, and nuts to build muscles.
- Fats: Fatty foods are calorie-dense. Aim for 35-40% of daily calories from fat, emphasizing unsaturated sources for essential fatty acids and fat-soluble vitamins.
- Carbohydrates: Choose healthy carbohydrates such as cereals, whole-grain breads and pasta, fruits and vegetables for energy. Avoid carbs with added sugars.
Micronutrient Considerations
- Salt: Individuals with CF lose more salt in their sweat. 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.
- Fat-soluble vitamins (A, D, E, and K): As CF can impair fat absorption, supplementation with a CF-specific multivitamin may be necessary to support immune function, growth, and healing.
- Calcium: Due to the risk of osteopenia or osteoporosis, adequate calcium intake is crucial. Dairy products are good sources of calcium (and the full-fat varieties also are good sources of fat and calories). If you don't drink cow's milk, consider trying a milk alternative that is fortified with calcium.
- Iron: Iron is important to help fight infection. It also helps to carry oxygen in your blood from your lungs to every cell in your body.
- Zinc: Zinc is important for growth, healing, and fighting infection.
Hydration
Maintaining adequate hydration is essential to help keep mucus thinner and aid food passage through the gut. Drink water throughout your day. Keep a full water bottle on hand. Drink about 2 liters of liquid a day. Younger children may need about 1 to 1.7 liters a day. Talk to your doctor about how much fluid you should drink. Drink water, milk and sports drinks. Avoid caffeine and alcohol.
Read also: CF Diet for Adults
Strategies for Meeting Nutritional Needs
For some teens with CF, it may be difficult to eat enough in a day to meet their needs, especially when they aren't feeling well. Although lots of people think they'd like to be able to eat whatever they want, many people with CF find it difficult to do so.
Increasing Calorie Intake
- Add healthy fats: 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: 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.
- Maximize mealtime: 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.
- Focus on high-calorie snacks: Try trail mix, peanut butter and banana sandwiches, full-fat yogurt with granola, or cheese crackers.
- Eat frequently: Eat whenever you are hungry. This may mean eating several small meals throughout the day.
Addressing Specific Challenges
- Picky eaters: Try eating meals as a family. Make meals fun and social.
- Malabsorption: Children with CF may need additional bathroom breaks, especially if their malabsorption is not well controlled.
- 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.
Nutritional Support
- Nutrition supplements: Nutrition supplements are calorie-rich drinks that also have protein, vitamins, and minerals.
- 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.
The Importance of Essential Fatty Acids
For more than 60 years it has been known and confirmed that the patients have a deficiency of linoleic acid, an n-6 essential fatty acid of importance for membrane structure and function. The ratio between arachidonic acid and docosahexaenoic acid, conditionally essential fatty acids of the n-6 and n-3 series, respectively, is often increased.
Linoleic Acid (LA)
The old report about LA deficiency in CF has been confirmed in different blood compartments and in various tissues. Many studies of supplementation with LA have been performed, but were often of relatively short duration and, lacking in stable study conditions, not resulting in clear outcomes and motivating general recommendations. Some studies have shown an important influence on growth and some observation studies have shown associations between lung function and serum phospholipid concentrations of LA.
Docosahexaenoic Acid (DHA)
The most important EFA of the n-3 fatty acid series is docosahexaenoic acid (22:6n-3, DHA). DHA is often decreased in CF, and low concentrations are related to the liver disease. It is of interest that in CF mice receiving long-term treatment with high doses of DHA, liver disease could be attenuated.
The Role of CFTR Modulators
Despite extensive research during more than 30 years since CFTR was detected, no cure of the disease has been developed. In the search for therapy, the high-throughput technique had identified correctors in the synthesis of some mutations and especially one potentiator, ivacaftor, for the action of some mutated proteins making it possible to improve the status of several patients. Combinations of the potentiator and correctors have further increased the potential target of mutations and shown to influence the lung function, sweat test and weight gain but not less the chronic inflammation. Of certain interest is that the modulators are recommended to be ingested together with lipids.
Read also: Children's CF Diet Tips
Read also: CF Dietary Guidelines
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