sweetener aspartame
Why should PKU patients avoid aspartame?
However, certain people with the genetic disease phenylketonuria (PKU), those with advanced liver disease, and pregnant women with hyperphenylalanine (high levels of phenylalanine in blood) have a problem with aspartame because they do not effectively metabolize the amino acid phenylalanine, one of aspartame's …
How much phenylalanine is in Diet Coke?
Six normal subjects each ingested a single 12-oz can of a diet cola (Diet Coke) providing 184 mg aspartame (APM), of which 104 mg is phenylalanine (Phe), and, on another occasion, a single 12-oz can of regular cola (Coke Classic).
What dietary restrictions are necessary for a person with PKU?
People with PKU must avoid foods that are high in protein like meat, fish, poultry, dairy, soy, legumes (dried beans) or nuts. Some fruits and vegetables are higher in protein than others. PHE is in almost everything except sugar, salt, oil, and water. Thankfully, many fruits and vegetables are naturally low in PHE.
What is the life expectancy of someone with PKU?
PKU does not shorten life expectancy, with or without treatment. Newborn screening for PKU is required in all 50 states. PKU is usually identified by newborn screening. A child's outlook is very good if she strictly follows the diet.
Can you eat potatoes with PKU?
Vegetables: Starchy vegetables like white, sweet, purple potatoes (and fries), winter squash, corn, and peas should be eaten sparingly. Most other fresh or frozen vegetables are low in phenylalanine with the exception of the ones on the non-compliant list.
What happens if someone with PKU eat protein?
A dangerous buildup of phenylalanine can develop when a person with PKU eats protein-rich foods, such as milk, cheese, nuts or meat, and even grains such as bread and pasta, or eats aspartame, an artificial sweetener. This buildup of phenylalanine results in damage to nerve cells in the brain.
Can PKU eat rice?
Foods such as regular pasta, bread, rice, and starchy vegetables (e.g., potatoes, peas, corn) are likely to be limited in your diet. Your dietitian may also provide you with some options for “free foods,” which are foods that contain no Phe and can be consumed as fillers (for example, some sugar candies).
What supplements do PKU patients take?
Supplementing with vitamin K may correct a deficiency. People with PKU may be deficient in several nutrients, due to the restricted diet which is low in protein and animal fat. Deficiencies of long-chain polyunsaturated fatty acids (LC-PUFAs),selenium,vitamin B12, and vitamin K may develop on this diet.
Can you outgrow PKU?
A person with PKU does not outgrow it and must stay on the diet for life.
What is the long term outlook for a child with PKU?
The long-term outlook for people with PKU is very good if they follow a PKU meal plan closely and shortly after birth. When diagnosis and treatment are delayed, brain damage may occur. This can lead to intellectual disabilities by the child's first year of life.
How do PKU patients get protein?
Today, the major source of dietary protein for those with PKU consists of mixtures of synthetic AA devoid of phe and a small amount of dietary protein from fruits and vegetables to provide phe.
Can you eat chocolate if you have PKU?
Children and adults with PKU have to eat small amounts of Phe and they cannot eat meats, dairy, eggs, beans or nuts. Someone with PKU cannot eat pizza, birthday cake, regular bread, yogurt, cheese, ice cream, chocolate, hot dogs, hamburgers, or turkey on Thanksgiving!
What happens if your body can't process protein?
If you have it, your body can't process phenylalanine (Phe). Phe is an amino acid, a building block of proteins. It is in almost all foods. If your Phe level gets too high, it can damage your brain and cause severe intellectual disability.
Why can't you give PKU patients a no protein diet?
A low-protein diet is the main treatment for phenylketonuria (PKU). This is because people who have PKU lack an enzyme to properly process the amino acid phenylalanine, a part of protein. Phenylalanine is present in all protein foods and in some nonprotein foods, such as soda pop and artificial sweeteners.
What foods are rich in phenylalanine?
Phenylalanine is found in most protein-containing foods such as milk, eggs, cheese, nuts, soybeans, chicken, beef, pork, beans and fish.
Is PKU more common in males or females?
Each year 10,000 to 15,000 babies are born with the disease in the United States and Phenylketonuria occurs in both males and females of all ethnic backgrounds (although it is more common in individuals of Northern European and Native American heritage.)
What foods do not contain protein?
The following are low-protein foods:
