With all the exciting news in the PKU community lately and new progress with treatment guidelines , as well as the recent announcement of the low protein food coverage in BC. I have been re evaluating my dreams and hopes for the PKU community and what Id like to see next.

Then it was right there right in front of my eyes. Everyday I log into the computer and surf the online PKU community and I see it.  Conversions.  methods of tracking PHE, exchanges vs PHe or Grams of protein.

I see a need for a universal method. So many people give advice on values for food, not realizing that alot of places count differently.

Here is what I know from my own research.

Here in Canada 1 exchange is 15 mg of PHE, 50 mg of PHE is 1 gram of protein.

So for example, my tolerance is 350 mg of PHE, or 23.3 exchanges or 7 grams of protein.

In some places such as the United Kingdom, they count exchanges differently. From what I understand 1 exchange is 1 gram of protein. However they have a large list of “free” foods. They don’t count most fruits and vegetables.

Here everything has protein , so everything has PHE , even if its like 0.7 mg or 24 mg . We count everything.

Not only are the methods of tracking PHE but the way the  blood PHE levels are counted.

Range here in British Columbia, is 2 to 6 mm/dl

where as in the states and other locations, 120 umol to 360 umol

To convert my range to theirs I times 2 by 60 to get 120 umol’s

or to covert back take 120 and divide by 60 giving you the 2 mm/dl again.

This is why, when giving advice to others regarding phe amounts we must keep in mind that its not the same everywhere. Id really like to see a universal method so no matter where you are we count the same , values are the same and blood phe levels are monitored the same.

All foods are counted the same. Virginia Schuett’s food list binder is like a bible for us PKUers over here. She works so hard to make such a detailed list. We relie heavily on the food list. Each item of food is meticulously detailed with weight amount, measuring cup amount, phe amount, protein amount, exchange amount and calories amount.

All these different methods really get confusing after a while, and when speaking in the large online community.  So what can we do to get a universal conversion method? Id like to hear from you!

The National PKU Alliance and Canadian PKU and Allied Disorders are pleased to announce that the first-ever specific medical and dietary guidelines for the treatment of PKU have been released by the American College of Medical Genetics and Genomics and Genetic Metabolic Dieticians International.  The guidelines represent a tremendous step forward in ensuring that all patients in the U.S. and Canada receive the highest quality of care and treatment in the management of their PKU.

Our two national patient and families associations congratulate ACMG and GMDI for their leadership in developing these thorough guidelines. The recommendations are based upon the NIH 2000 Consensus Conference for PKU, the 2012 NIH Scientific Review Conference and a review by the U.S. Agency for Healthcare Research and Quality.  The work groups who prepared these guidelines included PKU doctors from the USA and Canada. The key recommendations on the new guidelines include:

• ·         The guidelines refer to PKU as phenylalanine hydroxylase (PHA)deficiency.
• ·         The treatment of PKU is lifelong with a goal of maintaining blood PHE levels in the range of 120-360 umol/l (2-6 mg/dl) in patients of all ages for life.
• ·         Patients treated within the early weeks of life with initial good metabolic control, but who lose that control in later childhood or as an adult, may experience both reversible and irreversible neuropsychiatric consequences.
• ·         PAH genotyping (i.e. mutation analysis) is recommended for improved therapy planning.
• ·         Medical foods (formula and foods modified to be low in protein) are medically necessary for people living with PKU and should be regarded as medications.
• ·         Any combination of therapies (medical foods, Kuvan, etc) that improve a patient’s blood PHE levels is appropriate and should be individualized.
• ·         Reduction of blood PHE, increase in PHE tolerance or improvement in clinical symptoms of PKU are all valid indications to continue a particular therapy.
• ·         Genetic counseling should be provided as an ongoing process for individuals with PKU and their families.
• ·         Due to an increased risk for neurocognitive and psychological issues, regular mental health monitoring is warranted.  A number of screening tests are recommended to identify those in need of further assessment.
• ·         Blood PHE should be monitored at a consistent time during the day, preferably 2-3 hours after eating.

The National PKU Alliance and Canadian PKU and Allied Disorders are currently in the process of summarizing all of the key recommendations released.  These guidelines should be discussed with your PKU treatment team to help you get the best possible care in managing your PKU.  The medical guidelines can be found athttps://www.acmg.net/docs/Phenylalanine_Hydrosylase_Deficiency_Practice_Guideline_AOP_Jan_2013.pdf.