1. the Phe requirement in infancy is high and the natural diet is needed to supplement the insufficient Phe in the low Phe treatment formula. the Phe requirement for patients taking Phe-free treatment formula comes entirely from the natural diet. The best natural diet for infancy is breast milk because it is not only nutritious, but also has low Phe content (only 1/3 of cow’s milk) and high carnitine content.
2, Phe deficiency syndrome: Phe is an essential amino acid, excessive restriction of Phe intake will lead to Phe deficiency, manifested as developmental arrest, loss of appetite, vomiting, diarrhea, rash, hair loss, hypoproteinemia, anemia, hypoglycemia and osteoporosis, etc., and in severe cases, death. The way to prevent this is to regularly test the blood Phe concentration to prevent patients from having a blood Phe concentration below 120 μmol/(2mg/dl)L for a long time. 3. The nutritional value of protein in therapeutic milk powder with amino acid mixture as the main ingredient is poorer than that of natural protein, therefore, under the premise that the blood Phe concentration is controlled in the ideal range, various natural proteins should be consumed as much as possible and attention should be paid to all-round nutritional Maintenance.
4. The osmotic pressure of therapeutic milk powder is higher than that of body fluids even if it is the standard concentration of milk conditioning (15%). The concentration of milk for treatment formula should not be too high during the breastfeeding period, especially for newborns, and water should be fed between feedings to replenish sufficient water.
5. Some patients have difficulty tolerating the unique taste of amino acids in therapeutic milk powder, so consider adding therapeutic milk powder to orange juice, orange juice or apple juice for consumption.
6. Diet therapy should be carried out under the guidance of physicians and dietitians, and it is best to have recipes customized by dietitians according to the prescriptions of physicians. In addition to the therapeutic formula, older children should be fed with low Phe potatoes, starch, and as many fresh vegetables and fruits as possible. Special starch, granulated sugar, and fats that contain almost no Phe can supplement the insufficient calories.