June 2012

Document Type


Degree Name



Oregon Health & Science University


Limited research exists to determine whether education for children with phenylketonuria (PKU) can help improve children’s knowledge of their disorder and increase variety in the diet within the confines of a severe phenylalanine (phe) restriction. The planning, preparation and administration of foods provided to children with PKU is parent driven from birth through early childhood. As children enter school age, parents have less control over the child’s diet, and once a child reaches adolescence parents may have little to no control due to the adolescent’s desire for independence. Because of social pressures and little understanding of the disease many adolescents may have a difficult time choosing appropriate foods to manage their disorder. Poor management of PKU can cause inhibited growth (1, 2), decreased intelligence (3, 4), and difficulty in school (3, 5, 6). The objective was to determine whether group education for children with PKU could improve knowledge of the disorder and diet recommendations immediately following and three months after the education intervention. Researchers investigated whether group education was effective in increasing the amount of fruit and vegetables consumed by children and/or improving metabolic control as measured by blood phe concentrations. Finally, we used a Food Frequency Questionnaire (FFQ) and 24 – hour recall as a means to document protein intake from foods in patients with PKU. Food frequency questionnaires were used to measure usual intake and blood phe concentrations were used to assess metabolic control. This quasi-experimental design assessed change in knowledge, protein intake, fruit and vegetables consumption, and blood phe concentrations in four girls and four boys with PKU. There was no significant change in knowledge score (p = 0.086), protein intake (p = 0.105), fruit (p = 0.414) and vegetable (p = 0.317) consumption, or phenylalanine concentrations (p = 0.499) for girls between baseline measures and three months following education day. Knowledge score (p = 0.667), protein intake (p = 0.105), fruit (p = 0.317) and vegetable (p = 0.317) consumption, and phe concentrations (p = 0.267) for boys were not different from baseline at three months. When comparing boys and girls, change in knowledge score (p = 0.543), protein intake (p = 0.241), fruit (p = 0.207) and vegetable (p = 0.780) consumption, and phe concentrations (p = 0.147) were not significantly different. The FFQ compared to the 24-hour recalls was not significantly different in estimating protein needs before (p = 0.108) or after (p = 0.506) education day. We conclude that a short one-time nutrition education intervention is not effective at improving knowledge, increasing fruit and vegetable consumption, or improving metabolic control as measured by blood phenylalanine levels. The FFQ may be an appropriate tool for estimating protein intake in the PKU population but due to small sample size (n=4) further research is needed in this area.




Graduate Programs in Human Nutrition


School of Medicine



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