Nutritional recommendations for children with cystic fibrosis

Cystic fibrosis is a genetic disorder caused by a mutation in the CFTR (Cystic Fibrosis Transmembrane Conductance Regulator) protein located on the 7th autosomal chromosome. Defects in the function or absence of this protein disrupt the transport of chloride ions across cell membranes, leading to excessive absorption of sodium and water ions, resulting in thickening and increased viscosity of secretions in the lungs. [1] These ion transport disturbances affect the cells of the respiratory, digestive, and reproductive systems. The pancreas is another organ affected by the disease. Due to cystic changes where mucus accumulates, there is an impairment of its exocrine function, which involves the production of pancreatic juice containing enzymes essential for digestion. Cystic fibrosis is a systemic condition that reduces the quality of life in children and requires treatment with inhalations, a high-calorie diet, and exercises to strengthen respiratory muscles.

Cystic fibrosis, as an incurable genetic disorder, significantly impacts the lifespan and quality of life of affected individuals, primarily due to the extent of respiratory system damage. In the full clinical picture of cystic fibrosis, the following symptoms are observed: elevated chloride ion levels in sweat, recurrent inflammation of the airways, and exocrine pancreatic dysfunction. Consequently, children with this condition exhibit poor appetite, digestive and nutrient absorption disorders, leading to delays in physical and psychomotor development. Adverse fat digestion processes result from a deficiency in pancreatic enzymes, including lipase, which is responsible for fat emulsification. Undigested fats enter the intestines, causing frequent fatty stools. Depending on the degree of preserved pancreatic function, enzymatic supplementation, including lipase, may be necessary.

The diet for children with cystic fibrosis must be high in calories and protein, as affected children have a higher energy requirement than healthy peers. Chronic disruptions in pancreatic function, which lead to the loss of undigested food, as well as frequent respiratory infections, fever, and physical exertion from coughing further increase energy needs. At the same time, children with cystic fibrosis often have a poor appetite and dislike eating. The diet must include pancreatic enzyme supplementation (depending on the condition of the pancreas) and fat-soluble vitamins such as A, D, E, and K. To provide the child with sufficient energy, high-energy-density foods should be used, i.e., products that deliver many calories in small quantities.