Why can't some people eat PROTEIN?

Question

I have a friend and her children who cannot eat protein because of some disorder in their bodies. I know the name of the problem when I see it but can't remember it off hand. I know they have to have blood test every so often to check their protein levels.

If I remember right it has something to do with PKU? Can someone explain this to me please?

Answer 1

Phenylketonuria (PKU) is a genetic disorder where the body can't use phenylalanine. Phenylalanine is an essential amino acid found in high protein foods. By essential, that means that our body has to have it to function properly and it can only be found in foods.

The problem is that those with PKU have bodies that cannot break down the phenylalanine. Since it can't be broken down and used, it accumulates in the blood and tissues. If the level gets too high, brain problems will result.

PKU is hereditary but on a recessive gene. The incidence is higher in whites and orientals than in other races. Most states screen each infant for PKU after birth so itreatment can be started.

If untreated, infants may have vomiting, irritability, a rash and smelly urine. Some may have increased muscle tone and more active reflexes. Later, severe brain problems occur (mental retardation and seizures). Other common signs in untreated children include a small head, noticeable cheek bones, large upper jaw bones, widely spaced teeth, poor tooth enamel and stunted growth.

The goal of PKU treatment is to maintain a blood level of phenylalanine between 2 and 10 mg/dl. Since phenylalanine is needed for normal growth, the diet includes it but in much lower amounts than normal using cereals, starches, fruits and vegetables, along with a milk substitute. High protein foods must be avoided (meat, fish, poultry, eggs, cheese, milk, dried beans and peas).