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PKU-Nutritional Management

Feeding babies with PKU

Feeding babies with PKUWhere phenylketonuria is diagnosed as a result of new born screening, it is important to begin dietary treatment immediately. Mother’s milk, as well as providing amino acids and essential fatty acids (mainly Omega 3 with neuroprotective effects), also contains small amounts of phenylalanine. Immediately following diagnosis the daily levels of Phe must first be reduced to within the safe range. Breast-feeding for babies affected by PKU is therefore encouraged and helps create a strong tie between mother and child. Breast feeding must subsequently be supplemented with appropriate amino acid mixtures. Finally, the levels of phenylalanine in the baby’s blood must be regularly monitored.

Feeding children with PKU

Feeding children with PKUChildren are growing and have high calorie and protein requirements. There must therefore be a balance between the minimum possible amount of phenylalanine while meeting their protein and energy requirements. The first step is to prevent the accumulation of phenylalanine. This does not mean a total absence of phenylalanine. It is unnecessary to exclude all foods containing protein; it is advisable to provide foods with a moderate protein content that are however low in phenylalanine. This dietary approach depends on the child’s tolerance (i.e. the form of phenylketonuria present). A diet totally lacking in protein would result in inadequate growth. There must be adequate provision of the right amino acids to keep the child healthy and ensure growth. This can be achieved through supplementary mixtures of amino acids that do not contain phenylalanine and are age appropriate. ItiIs also important to pay attention to a balanced intake of vitamins (above all vitamin D, vitamin A, vitamin B12), mineral salts (calcium), essential fatty acids (Omega 3) and micronutrients (selenium, zinc, iron). Depending on the phenylalanine content tolerated by the child, the protein requirements can be met by means of mixtures of amino acids and natural proteins (foods). For classical phenylketonuria, for example, 350 mg of phenylalanine a day corresponds to approximately 6 g of protein from foods. The caloric requirements must be age appropriate. However, because of the dietary restrictions, may be insufficient. This could compromise growth while prompting the catabolism of proteins from muscle and cause the release of phenylalanine. The diet adherence and effectiveness should be monitored by taking a blood sample each week to check the levels of phenylalanine.

PKU in young people and adults

PKU in young people and adultsThe right diet permits the regular development of adolescents, both mentally and physically, allowing them to lead normal lives.
Adult requirements for amino acids are not significantly different from those of children, but there are some other, equally important, nutrients.

These include:
• calcium and vitamin D to prevent osteoporosis (loss of bone mass)
• folic acid to prevent arteriosclerosis(hardening of arterial walls)
• fiber, for normal digestive health
• Omega 3 essential fatty acids which strengthen immunity and have neuroprotective effects

The amino acid mixtures for young people and adults are available in practical packaging that allows for an active lifestyle.
Testing of phenylalanine in the blood is less frequent than for infants, but regular monthly monitoring is still necessary.
 

PKU and pregnancy

PKU and pregnancyThe desire to have children is the most natural impulse in the world. For women with PKU, constant and rigorous attention is required from the initial “decision” to have a child. A strict diet is necessary even before conception in order to keep the concentration of phenylalanine within the desired limits. Ideally, in the six months before conception, a level of phenylalanine in the blood of 120 µmol/l should be achieved and maintained. Foods that should be cut out completely are all products of animal origin (meat, fish, eggs, milk and dairy products in general) and beans. Grain-based foods (bread, pasta and bakery products) as well as eggs and milk can be substituted with protein- free dietary products. Women with PKU whose children are born without the disease can breastfeed their babies, even though their milk is richer in phenylalanine than milk from a healthy mother. The diet must be maintained for life and routine blood testing will be required to establish that nutritional intake is balanced. The greater the motivation to follow a strict, rigorous diet, the greater its success in ensuring that a child will not face any consequences (e.g. mental retardation) of the mother’s phenylketonuria. The levels of phenylalanine in the blood must be reduced before pregnancy and held stable at 120 µmol/l of blood. If prior to conception, the blood level of phenylalanine in the mother-to-be is fully controlled, i.e. less than 240 µmol per liter of blood, the child has optimum conditions for normal development and, in most cases (as the probability is minimal), the unborn baby will not be affected phenylketonuria even if it is a carrier of the altered gene.
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