How is such a disease looked
after?
Phenylalanine being constituent proteins, it is thus advisable to limit the animal and vegetable protein contribution of the patients.
It will be to them thus interdict to consume: meats,
dairy produces, fish, eggs, and cereals , which contain too many proteins.
Remain authorized:
- the fruits and the vegetables , which contain also proteins and thus of phenylalanine, but in variable quantities, and will have thus to be weighed and entered.
- the carbohydrates (sugar and starch), oils and the margarines without proteins , can be consumed freely.
Tables make it possible to know how much phenylalanine is in X grams of
product. All the food is thus weighed, and the menus are made up in order not to bring a
toxic amount of phenylalanine during the day.
This strongly limiting the quantity of food for the children, one has recourse to special dietetic products , low in proteins, making it possible to balance
the modes.
In order to ensure a normal growth, it is advisable their to bring the other
essential amino acids , 22, which in the event of severe mode would miss or in too
small quantities to ensure a normal development of the children.
A mixture of amino acids deprived of phenylalanine is used for stage with
this problem. It is it should be noted that the majority of the current mixtures are of an
extremely unpleasant taste involving many difficulties for the parents who must make it
take to their children imperatively.
Currently, the specialists encourage the continuation of the mode during all
the life .
The epidemiologic studies showed that a strict control of the mode was associated with
better performances, but especially that the interruption of the mode, at some age that it
is had noxious effects on the intellectual performances of the patients.
Currently the target blood phenylalanine rates are variable according to the age of the patients.
| Blood rate of phenylalanine recommended | |
|---|---|
| Age of the patient | Phenylalanine rate in �mole/l |
| 0 - 6 years | 120 - 360 |
| 7 - 14 years | 240 - 480 |
| Teenager - Adult | < 700 |
| Pregnant woman | 60 - 250 |
The control blood of the phenylalanine rates must be frequent so as to adapt the mode according to the possible changes, but the recommendations can vary nevertheless according to patient and processing centers' to another.
