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. |