Knowledge, compliance and serum phenylalanine concentrations in adolescents and adults with phenylketonuria and the effect of a patient-focused educational resource

Background There is a lack of dedicated resources for adolescent and adult patients with phenylketonuria (PKU) and few studies have examined dietary practices within this group. Methods One hundred and seventy-seven PKU patients were sent questionnaires to assess dietary compliance and the preferred format for an educational resource. Seventy-one patients responded; 32 following diet were recruited to assess the resource's impact on test variables. The results were compared for the intervention group (n = 22) and control group (n = 10) at baseline, and 1 and 6 months after resource intervention. Results Most patients were aware of dietary recommendations, although this did not always result in compliance. The preferred resource format was a filofax-style folder with inserts (P < 0.05). There was a significant difference in the extent of change in knowledge score between baseline and 1 month in favour of the intervention group (P < 0.05). The improvement in knowledge was not accompanied by a significant improvement in measures of compliance. Conclusions These findings add to the knowledge base about this patient group and support the use of patient involvement in resource development. It is likely that the test parameters used were not sensitive enough to pick up subtle and longer-term effects on compliance.