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Table 8 Factors to consider during illness management in PKU

From: PKU dietary handbook to accompany PKU guidelines

Diet Dietary advice
Protein substitute Maintenance of protein substitute intake is essential to help minimise catabolism during illness.
It is better to give this in smaller, more frequent doses throughout the day.
Protein substitute given when there is a high temperature may lead to vomiting. It is always better to administer protein substitute when a high temperature is brought under control post administration of anti-pyretic medication.
High carbohydrate intake Encourage frequent high carbohydrate drinks or glucose polymer solution.
Natural protein intake If on dietary treatment only, there is no need to ‘formally’ omit natural protein. In practice, a reduced appetite leads to a lower natural protein intake.
If on sapropterin (and dietary treatment), advice is the same as for dietary treatment only.
Medications All treatment specific medication should be continued during illness.
Medications should be free of aspartame.
Continue sapropterin if prescribed.
Treat precipitating factors Antibiotics (aspartame-free if possible)a.
Anti-pyretic medication Give as necessary for high temperatures.
  1. aBlood phenylalanine concentrations are likely to rise quickly during illness. For the immediate and short-term treatment of infections, if only aspartame containing medicines are available, it may be better to use these until aspartame-free medication is sourced rather than leave a person with PKU without such treatment