Treatment of PKU

Safe Phenylananine Levels

Keeping the phenylananine at just the right level is a balancing act. As long as the level is OK most of the time, your child’s development will not be affected by occasional high levels.

What is the right phenylananine level?

PKU clinics differ on what they consider to be acceptable levels of phenylananine. From time to time phenylananine levels do vary. Higher or lower levels are unlikely to be a problem when overall control is good.

General guidelines include the following:

  • Most clinics agree that for children up to eight years old Phe levels should be 100-350 micromoles per litre
  • For older children, teenagers and adults, most clinics recommend a maximum level of 450 micromoles per litre – however, some clinics accept levels up to 600 or 700 micromoles per litre (while this is not ideal), and other clinics say people with PKU should aim to keep their levels around 100-350 micromoles per litre for life.
  • Women planning a pregnancy or who are pregnant need to have lower levels to protect the baby.
  • Be guided by your clinic.

The important thing to remember is that the dietary treatment of PKU has proved highly successful!! It leads to normal, happy, healthy and fit children who grow up into healthy teenagers and then healthy adults. The purpose of this association is to provide you with support from families who are on the same journey you are on.

Clinics currently use their own protein counting lists, which may differ slightly from each other. Links to information from the Children’s Hospital at Westmead and Womens and Children’s Hospital, South Australia are given below.

There are slight difference in which fruits and vegetables are counted between these information sheets, based on the age range of patients at the clinic. Please discuss with your dietician.

Childrens Hospital at Westmead protein counting for PKU

South Australia, Women’s and Childrens Hospital Low Protein diet for PKU

 

Inborn Errors of Metabolism Grant (IEM Grant)

There have been changes to the way the Inborn Errors of Metabolism Grant is being processed moving forward.

Patients diagnosed with PKU must re-apply for the IEM Grant every 12 months.

It is the responsibility of all patients to schedule review appointments, as required by their treating metabolic specialists and to ensure applications are provided to the Department prior to their annual review date.

Metabolic Specialists will be required to notify the Department of patients who fail to comply with the conditions of the IEM program. Following this advice, and if the situation is not adequately addressed, the IEM grant may be withdrawn.

The Department will not issue reminder notices. Grant payments will cease if the re-application is not received.

Contact details for the IEM Grant
IEM Program Officer
Pharmaceutical Benefits Division
Department of Health & Aging
MDP 953, GPO Box 9848
Canberra ACT 2601

Phone: (02) 6289 8980
Fax: (02) 6289 8537
Email: This email address is being protected from spambots. You need JavaScript enabled to view it.

Further information in relation to the IEM Grant and application forms