Parent knowledge regarding food selection for children with PKU: Results of a survey in the United States

OBJECTIVES: Dietary treatment is the main therapy for most patients with phenylketonuria (PKU). Parental knowledge regarding food selection is crucial to ensure adequate metabolic control and brain development during childhood and to promote lifelong adherence and healthy dietary behavior in the offspring. The aims of this study were to assess whether parental or caregiver knowledge regarding nutritional selection for children with PKU is in accordance with medical recommendations and to evaluate factors that influence their level of knowledge.

METHODS: This was a cross-sectional observational study. An online or paper survey (N = 178) was distributed throughout the United States. The survey included a validated food selection questionnaire to assess whether the respondent adequately identified foods that require certain restrictions versus foods that can be consumed freely by an individual with PKU.

RESULTS: General knowledge of food selection among the caregivers was very high or high in nearly 60% (60-98th score percentile). Participants with the lowest scores in general knowledge of the PKU diet (quartile 1) were more likely to label allowed foods incorrectly. Respondents with the highest scores (quartile 4) were more likely to label limited foods correctly but incorrectly label allowed items.

CONCLUSION: Knowledge of restricted foods is important to avoid poor metabolic control, but knowledge of allowed foods can be just as important to allow for a diet that is diverse, palatable, and nutritionally balanced.