Management
Guidelines
Portal
PKU Nutrition Management Guidelines
Second Edition
March 2022, v.2.4
Updated: March 2022
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Resources
Overview

Educational resources and tools are currently available to support nutrition management of PKU. A toolkit containing additional resources specific to these guidelines has been developed and updated.

Learning Objectives for Patients/Families

Learning objectives for individuals with PKU and their caretakers include:

  • Understanding the basic metabolism of PHE and the role of PAH deficiency
  • Recognizing the need to monitor blood PHE
  • Recognizing the need for frequent contact with appropriate healthcare providers (e.g. metabolic dietitian, nurse, physician)
  • Acquiring skills needed to implement the dietary prescription and/or pharmacotherapy
  • Realizing the importance of strict adherence to the dietary prescription and/or pharmacotherapy
  • Recognizing the role of medical food in the dietary management
  • Recognizing the role of adjunct and alternate treatment therapies
  • Acquiring skills needed to achieve optimal nutritional status when normalization of diet is allowed by pharmacotherapy. 
Resources for patients

A compilation of resources for individuals with PKU and their families developed by National PKU Alliance is available at:

http://npkua.org/Resources.aspx

Resources for providers

A compilation of resources for metabolic dietitians developed by GMDI is available at:

www.gmdi.org (accessible only by GMDI members)

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