How to treat phenylketonuria and how to avoid complications

The care and treatment of phenylketonuria in the baby should be guided by the pediatrician, but the main care is to avoid foods rich in phenylalanine, which are mainly foods rich in proteins, such as meat, fish, milk, cheese and eggs. Thus, parents of babies with phenylketonuria should be attentive to their child’s diet, both at home and at school.

In addition, exclusive breastfeeding must also be well oriented by the pediatrician, since breast milk contains phenylalanine, although it is much less than that present in most pharmacy formulas. Ideally, the amount of phenylalanine for a baby up to 6 months of age should be kept between 20 to 70 mg of phenylalanine per kg of body weight.

It is important that treatment for phenylketonuria is followed according to the guidelines of the pediatrician and nutritionist to prevent complications from arising, which are mainly related to the development of the nervous system.

1. Nutritional treatment

Nutritional treatment is the main way to avoid complications of the disease, since it is through food that it is possible to control the levels of phenylalanine in the blood, thus avoiding complications of the disease. It is important that the diet is guided by the nutritionist according to the results of the baby’s tests that must be performed regularly to assess the levels of phenylalanine in the blood.

Phenylalanine can be found in several foods, both animal and vegetable. Thus, to control the disease and avoid complications it is important to avoid some foods, such as:

  • Animal foods:  meat, milk and meat products, eggs, fish, seafood, and meat products, such as sausage, sausage, bacon, ham.
  • Foods of vegetable origin:  wheat, soy and derivatives, chickpeas, beans, peas, lentils, chestnuts, peanuts, nuts, almonds, hazelnuts, pistachios, pine nuts;
  • Sweeteners with aspartame ;
  • Products that contain prohibited foods , such as cakes, cookies, ice cream and bread.

Fruits and vegetables can be consumed by phenylketonurics, as well as sugars and fats. It is also possible to find on the market several special products made for this audience, such as rice, pasta and crackers, and there are several recipes that can be used to produce foods low in phenylalanine.

Check out a list of foods rich in phenylalanine.

How to give breast milk safely

Although the recommendation is to exclude breast milk from the baby’s diet, using only pharmacy milk without phenylalanine, it is still possible to breastfeed the baby phenylketonuric, however for this it is necessary:

  • Do a blood test on the baby every week to check the levels of phenylalanine in the blood;
  • Calculate the amount of breast milk to give to the baby, according to the phenylalanine values ​​in the baby’s blood and according to the pediatrician’s guidance;
  • Calculate the amount of pharmacy milk without phenylalanine, to complete the baby’s feeding;
  • Pump the right amount of breast milk that the mother can give the baby;
  • Use the bottle or relactation technique to feed the baby. 

It is essential to exclude the amino acid phenylalanine from food, so that the baby does not have problems in physical and mental development, such as mental retardation. See what food should be like in phenylketonuria.

2. Use of nutritional supplements

As the diet of the person with phenylketonuria is very restricted, it is possible that he does not have the amount of vitamins and minerals necessary for the proper functioning of the organism and for the correct development of the child. Thus, the nutritionist can recommend the use of supplements and nutritional formulas to ensure the proper growth of the baby and promote its health.

The supplement to be used is indicated by the nutritionist according to the age, weight of the person and the baby’s digestion capacity, and must be maintained throughout life.

Possible complications of phenylketonuria

Complications of phenylketonuria arise when the diagnosis is not made early or when treatment is not followed according to the pediatrician’s guidelines, with an accumulation of phenylalanine in the blood, which can reach specific areas of the brain and lead to the development of permanent changes, such as : 

  • Delay in psychomotor development;
  • Little brain development;
  • microcephaly;
  • Hyperactivity;
  • Behavioral disorders;
  • Decreased IQ;
  • Severe mental disability;
  • Convulsions;
  • Tremors.

Over time, if the child is not properly treated, there may be difficulty in sitting and walking, behavioral disorders and delayed speech and intellectual development, in addition to depression, epilepsy and ataxia, which is loss of control. of voluntary movements.

How to avoid

To avoid complications, it is important that the diagnosis of the disease is made in the first few days after the child’s birth through the heel prick test. If the result is positive, it is important that the treatment is done according to the pediatrician’s guidance.

In addition, in these cases it is important that regular examinations are carried out to check the general health of the child and, thus, indicate changes in the diet and food supplements.

Follow-up exams are usually performed weekly until the baby is 1 year old. Children between 2 and 6 years old repeat the exam every 15 days and, from 7 years old, the exam is done once a month.

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