|Cause of high blood phenylalanine levels||Action|
|Fever/infection/trauma||See section on illness management|
|Excess natural protein intake||
• Check understanding/calculation of phenylalanine allowance/ exchanges, misinterpretation/misunderstandings of protein amounts in foods. Review portion sizes.|
• Check any intentional dietary non-adherence (e.g. patient chose to eat extra protein).
• Check any special low protein products are low protein and not gluten-free by accident.
• Re-educate patient or family if necessary.
|Inadequate intake of protein substitute||
• Check adherence (at home, nursery, school). Explore any reasons for poor adherence and either re-educate or change type or flavour of protein substitute if appropriate.|
• Check timing of protein substitute (should be spread throughout the day).
• Check patient has adequate supply of protein substitute.
• Re-calculate dose of protein substitute and increase the dosage if necessary.
|Incorrect prescription of protein substitute||• Occasionally the wrong protein substitute may accidentally be prescribed or given by the pharmacist or a home delivery company.|
|Low energy intake/weight loss/catabolism||• Increase energy intake/give extra carbohydrate. Encourage extra low protein foods or high calorie drinks.|
|No obvious reason||• If blood phenylalanine levels are consistently high, consider a reduction in natural protein/phenylalanine by approx. 0.5 to 1 g/day protein or 25 to 50 mg phenylalanine/day.|