Simplified Diet Approach in Phenylketonuria

Use of Dietary Protein and Free Fruits and Vegetables to Improve Metabolic Control Among Teens and Adults With Phenylketonuria: A Mixed Methods Approach

This is a study to determine if a simplified protein counting system, including the free usage of fruits and vegetables low in phenylalanine, will improve the metabolic control in teens and adults with phenylketonuria (PKU).

Anybody with a positive diagnosis of PKU, between the ages of 13-65 years will be invited to participate in this study.

Subjects will be educated on the simplified system upon enrollment, and historical phenylalanine (Phe) levels will be used for comparison. Subjects will also be asked about their attitudes towards their current Phe counting system.

Study Overview

• Status: Completed
• Conditions: Phenylketonuria
• Intervention / Treatment: Behavioral: Dietary Protein Counting and Free Fruits and Vegetables

Detailed Description

This is a study to determine if a simplified protein counting system, including the free usage of fruits and vegetables low in phenylalanine, will improve the metabolic control in teens and adults with phenylketonuria (PKU).

Anybody with a positive diagnosis of PKU, between the ages of 13-65 years will be invited to participate in this study.

Subjects will be educated on the simplified system upon enrollment, and historical phenylalanine (Phe) levels will be used for comparison. Subjects will also be asked about their attitudes towards their current Phe counting system.

Upon enrollment, a 3-day food record and Phe levels over the past 12 months will be collected. Demographic information will be asked, as well as questions regarding their current Phe counting system. Patients will be educated on the new counting system, stressing 3 key principles: 1) free use of fruits and vegetables, low in protein, 2) protein counting as a simpler version of Phe counting, including reinforcement of label reading, and 3) daily use of PKU formula for enhanced nutrient and protein intake. Patients will be given written educational tools to reinforce this new method.

At the end of the 12 months, subjects will be asked to provide a 3-day food record, and questions will be asked about their attitudes toward the use of the new simplified system.

Phenylalanine levels from the 12-month study period will be used to compare with historical data.

• Study Type: Interventional
• Enrollment (Actual): 33
• Phase: Not Applicable

Contacts and Locations

Study Locations

• United States
  • Nebraska
    • Omaha, Nebraska, United States, 68114
      • Children's Hospital & Medical Center
    • Omaha, Nebraska, United States, 68198
      • Unversity of Nebraska Medical Center
    • Omaha, Nebraska, United States, 68131
      • Nebraska Medicine, Midtown Health Center
    • Omaha, Nebraska, United States, 68106
      • Monroe-Meyer Insititute

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 13 years to 65 years (Child, Adult, Older Adult)
• Accepts Healthy Volunteers: No

Description

Inclusion Criteria:

1. Positive diagnosis of PKU
2. Males and females >13 year of age
3. Currently on phenylalanine-restricted diet

Exclusion Criteria:

1. Any woman planning, or becoming pregnant during study duration
2. Any patient who starts Kuvan during the study, as Kuvan lowers phenylalanine levels in some patients.
3. Any patient currently enrolled in other research studies (Prism 301/302 Protocol; Institutional Review Board Protocol # 376-13-FB)

Study Plan

How is the study designed?

Design Details

• Primary Purpose: Treatment
• Allocation: N/A
• Interventional Model: Single Group Assignment
• Masking: None (Open Label)

Number of Arms

1

Arms and Interventions

Participant Group / Arm	Intervention / Treatment
Experimental: Dietary Protein Counting & Free Fruits & Vegetables; Subjects will be educated on new simplified diet approach and phenylalanine levels will be monitored for 1 year, and will be compared to 12 months prior to study enrollment	Behavioral: Dietary Protein Counting and Free Fruits and Vegetables; Subjects will be educated on new simplified diet approach and phenylalanine levels will be monitored for 1 year, and will be compared to 12 months prior to study enrollment

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Metabolic Control in Teens and Adults with PKU	phenylalanine levels	12 months

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
attitudes towards simplified diet approach	qualitative data collection	12 months

Collaborators and Investigators

• Sponsor: University of Nebraska
• Investigators: Principal Investigator: Jill C Skrabal, PhD, University of Nebraska

Study record dates

Study Major Dates

• Study Start (Actual): October 26, 2015
• Primary Completion (Actual): September 20, 2017
• Study Completion (Actual): September 20, 2017

Study Registration Dates

• First Submitted: September 17, 2015
• First Submitted That Met QC Criteria: September 18, 2015
• First Posted (Estimated): September 21, 2015

Study Record Updates

• Last Update Posted (Actual): September 7, 2023
• Last Update Submitted That Met QC Criteria: September 2, 2023
• Last Verified: September 1, 2023

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Metabolic Diseases; Brain Diseases; Central Nervous System Diseases; Nervous System Diseases; Genetic Diseases, Inborn; Metabolism, Inborn Errors; Brain Diseases, Metabolic; Brain Diseases, Metabolic, Inborn; Amino Acid Metabolism, Inborn Errors; Phenylketonurias
• Other Study ID Numbers: 0451-15-EP

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No
• product manufactured in and exported from the U.S.: No