Nutrition

Good nutrition and maintaining good body weight have been shown to have a very important role in management of CF. Approximately 85% of people with CF have some degree of pancreatic insufficiency. This is caused by the thickened mucus blocking ducts in the pancreas and the digestive enzymes are not able to exit into the gut to aid digestion.

Treatment for pancreatic insufficiency may involve enzyme replacement, supplementary feeding and vitamin supplements.

Due to low absorption of nutrition from food, people with CF require up to 150% of normal calorie intake to maintain body weight. Unlike the majority of the population there is no restriction on the amount of high calorie foods containing lots of fat and/or sugar in a CF diet.

People with CF should eat plenty of

• Fatty foods (butter, icecream, chocolate)
• Sugary foods (jam, puddings, sweets)
• Milk and dairy products (cheese, yoghurt)
• Starchy foods (pasta, rice, bread)
• Protein foods (fish, eggs, meat)
• Vitamin/mineral rich foods (fruit, vegetables)

Because of the rich sugary foods common in the CF diet, extra care should be taken with dental care. Brushing teeth twice a day, drinking water after sugary snacks or drinks and visiting a dentist regularly will help maintain teeth in good condition.

The Dietitian will give direction regarding which foods and supplements are best for you and instruct you about how and when you should use them. You should consult your dietitian before making any changes to your regimen.

Enzyme Replacement Therapy

Pancreatic enzyme supplements are used to help digest fats, proteins and carbohydrates (the three components of food that give us energy).

Taking enzyme supplements aids in maintaining good body weight and assists with the absorption of essential nutrients. The dose of enzymes will vary with patient age, diet and how much CF has affected the pancreas.

As directed by your dietitian, enzymes should be taken with meals and snacks. For infants and small children the capsules may be opened and sprinkled on a product such as apple sauce and eaten with a spoon prior to or during the meal. Your CF team will teach you how to do this. Older children will swallow their capsules whole.

Supplementation

In cases where maintaining body weight is a problem, extra food supplementation is required. Usually this will start with extra high calorie foods but can progress to tube- feeding if necessary.

Tube-feeding can take a number of forms depending on the person and their lifestyle. It may be that only a short-term nasogastric tube is required, or if the supplementation is likely to be a longer-term prospect then a gastronomic tube ‘button’ is fitted directly through the stomach wall.

Vitamins & Minerals

Some vitamins are fat-soluble and therefore are susceptible to poor uptake in patients with CF, particularly vitamins A, D and E. Supplements that include these and other vitamins are usual for people with Cystic Fibrosis.

Bone strength can be affected by the poor uptake of minerals such as calcium and phosphorus, which are directly related to vitamin D levels. A diet rich in calcium such as milk, yoghurt, cheese and canned bony fish such as canned salmon can assist with maintaining a good intake of calcium.

There may be other causes of declining bone strength, such as medications like steroids, which are known to adversely affect bone density.

Salt Replacement

In hot weather, or situations where a person with CF sweats a lot, then salt replacement may be required.

The easiest and best way for people with CF to get extra salt is by adding salt to foods or eating foods that are naturally high in salt.

Foods such as:

• potato crisps
• tomato, soy or other sauces and pickles
• salted biscuits and crackers
• salted nuts
• processed meats, salami, ham and bacon
• canned meat in brine (fish, chicken)
• Supplements like electrolyte drinks and/or salt tablets can help prevent dehydration in people with CF.  These supplements also increase thirst that encourages the eater to drink more water or fluid.