Good nutrition and a healthy body weight are extra important if you have cystic fibrosis. You then need more calories and nutrients than someone without cystic fibrosis. A decreased appetite can make it difficult to eat enough.

What is cystic fibrosis?

In cystic fibrosis, the glands produce tough, sticky mucus. That is why this disease is also called cystic fibrosis. The tough mucus can cause problems in various places in the body. For example in the lungs and in the gastrointestinal tract.

Cystic fibrosis is a congenital condition caused by a defective gene. All newborns are screened for cystic fibrosis with the heel prick. In the majority of patients, the diagnosis is made before the first year of life. In the Netherlands it occurs in 1 in 6,200 newborns. In people who are diagnosed with cystic fibrosis later in life, the disease course is usually relatively mild and life expectancy is good.

Symptoms of cystic fibrosis

Cystic fibrosis has several effects on your body, but it mainly affects your lungs and digestive system. The buildup of sticky mucus in your lungs can cause breathing problems and increases your risk of lung infections. Over time, your lungs can no longer work properly.

Mucus can also build up in your pancreas, an organ involved in digestion. The small tubes in your pancreas can become blocked and damaged. This is called pancreatic insufficiency. When this happens, digestive enzymes made in your pancreas cannot pass into your intestines properly. Your body cannot digest food normally. This can lead to a nutrient deficiency. Most people with cystic fibrosis will need to take extra enzymes to break down their food.

Cystic fibrosis affects many different organs in the body. This makes people with the disease more likely to develop other health problems, including diabetes, liver disease, arthritis and osteoporosis. The severity of the symptoms and the course of the disease varies per person.

Nutrition in cystic fibrosis

Good nutrition and a healthy body weight are extra important if you have cystic fibrosis. You need more calories and nutrients. Your body has to work extra hard due to the disease and nutrients are absorbed less well. A balanced, calorie- and protein-rich diet with sufficient salt, fat and extra vitamins can help support your health. Your doctor and/or dietitian can help you with what to eat and drink during your illness.

Malnutrition in cystic fibrosis

The risk of malnutrition is greater when you have cystic fibrosis. Nutritional status is of great importance for the prognosis. Therefore, maintaining optimal nutritional status is paramount in the overall treatment.

Malnutrition or growth retardation may occur in children with cystic fibrosis. The pediatrician keeps an eye on this. Breastfeeding is preferred for children under 1 year of age. Sometimes this is not sufficient. In that case, the pediatrician or dietitian can recommend food for medical use. These foods must be used under medical supervision and are specially formulated for the specific needs of the patient. There are also supplementary diet foods for older children and adults in the form of drinking or tube feeding that provide extra energy and nutrients.

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