A PKU diet is the key treatment strategy if you have the genetic disorder phenylketonuria PKU, in which you are missing an enzyme that allows for the conversion of phenylalanine to tyrosine both amino acids. This will also help keep you more full and satisfied. Doing this increases your risk of cognitive and behavioral problems, which can impact your school, work, or relationships. Stay on diet!! What are your concerns? Ann Nestle Eng. Your individualized low-Phe diet will include fruits, vegetables, fats, sugars, and low-protein medical foods, such as low-protein pasta and breads. It’s commonly sold as NutraSweet or Equal, but it’s listed as aspartame in diet, lite, or sugar-free foods like soft drinks, desserts, gelatins, puddings, yogurts, etc. Rather than feeling badly about a high Phe level, use the information to be more determined about how you will regain control.
Phenylketonuria, or PKU, is a genetically inherited birth defect that causes an unwanted buildup of the amino acid phenylalanine in the blood. This buildup occurs because the enzyme that routinely converts one amino acid, phenylalanine, to another amino acid, tyrosine, is absent or deficient. Phenylalanine then accumulates in the blood and is toxic to brain tissue. A treatment team typically consists of a metabolic dietitian, physician, geneticist and psychologist as an infant is monitored during different phases of growth and development in childhood to an adult. A strict diet limited in phenylalanine is the main treatment for PKU. Phenylalanine is found in most protein-containing foods such as milk, eggs, cheese, nuts, soybeans, chicken, beef, pork, beans and fish. As the infant grows and becomes developmentally ready for solid foods, a registered dietitian nutritionist with expertise in PKU will create a unique menu for the child. Special phenylalanine-free foods are available for children and teens with PKU. The metabolic dietitian will create individual eating plans and modify them as needed to promote healthy growth and development. The diet needs to be followed for life. Get help with menu planning from the metabolic dietitian and plan ahead for eating out, school lunches, potlucks, trips and vacations, and other special events. Try to center special occasions around activities rather than food, and encourage your child to be part of the management of his or her diet as early as possible.
The diet for PKU consists of a phenylalanine-free medical formula and carefully measured amounts of fruits, vegetables, bread, pasta, and cereals. Many people who follow a low phenylalanine phe food pattern eat special low protein breads and pastas. They are nearly free of phe, allow greater freedom in food choices, and provide energy and variety in the food pattern. Foods that contain large amounts of phe must be eliminated from a low phe diet. These foods are high protein foods, such as milk, dairy products, meat, fish, chicken, eggs, beans, and nuts. These foods cause high blood phe levels for people with PKU. This target is an easy way to visualize the foods allowed on the diet for PKU. As the foods get further away from the bull’s-eye, they are higher in phenylalanine. The foods outside the target are not included in the low-phenylalanine meal plan. It is not unusual for someone who follows a low phe diet to have 2 kinds of vegetables and a baked potato for dinner. However, if these foods were the only foods a person consumed, his or her diet would be lacking protein, vitamins, and minerals.