Management of nutrition, growth and gastrointestinal symptoms remain an integral part of care in patients with cystic fibrosis. The main nutritional issues are pancreatic exocrine insufficiency with secondary malabsorption, salt depletion, micronutrient deficiency and elevated metabolic requirements. Abdominal pain is also a common presentation and the cause can be difficult to diagnose.
- Pancreatic insufficiency leads to macronutrient maldigestion and impaired weight gain in children with cystic fibrosis (CF), particularly if enzymes are inadequately replaced.
- Patients with CF have an increased recommended daily caloric intake compared with healthy peers.
- The goal for CF nutritional care is to maintain a normal growth pattern.
- Excessive salt loss can lead to hyponatraemic, hypochloraemic dehydration with young children with CF at higher risk. Daily salt replacement is required.
- Gastrointestinal manifestations are common. There are some particular CF-specific diagnoses of abdominal pain to add to the usual differential diagnoses considered for a child without CF.
- Almost all severe cases of CF liver disease are detected within the first two decades of life.