Pancreatic insufficiency occurs in about 80 percent of patients. Consequent to these deficiencies, inadequate digestion of food results in malabsorption and malnutrition with growth retardation and weight loss. Protein-energy malnutrition impairs immune responses and increases the risk of pulmonary infections. Declining respiratory muscle strength may adversely affect survival. Six small meals per day generally are better tolerated than fewer, larger meals by patients with high caloric requirements.
Extra salt is needed to replace the large amounts of sodium lost in perspiration.
Although uncommon even in CF patients, salt depletion leads to lethargy, weakness, dehydration, and metabolic alkalosis 2. Patients with CF are at risk of developing multiple nutrient deficiencies with their associated clinical manifestations. Even with appropriate pancreatic enzyme therapy, fat malabsorption and associated fat-soluble vitamin deficiencies may still persist in CF patients. Typically CF patients are recommended to consume two times the RDA for vitamins and minerals to prevent the associated deficiencies. Vitamin D deficiency causes rickets in young children and osteomalacia in older children and adults.