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This study surveyed PKU patients and their primary caretakers to assess their current management practices, the barriers to effective management, and the potential utility of a home monitor in managing PKU. A survey instrument was mailed to caretakers of all 50 patients with PKU in Utah between the ages of 2 and 18 years in 1997 (response rate 64%). It included separate components for caretakers and patients aged 10 to 18 years. Although there was uneven compliance with recommended practices, caretakers universally recognized the negative consequences of not adhering to the low-protein diet. There was, however, disagreement regarding such consequences among the older children surveyed. The primary obstacles cited to better adherence were time constraints and stress associated with food preparation and record-keeping, and the restrictions imposed on social life. Phenylalanine test results were regarded as the principal signal for the need for dietary adjustment. Despite the facts that obstacles to dietary adherence are multifaceted and that no single intervention would therefore serve as a panacea, a large majority of respondents believed a home monitor would facilitate better management of PKU through more regular and timely feedback.

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The mineral status in phenylketonuria (PKU) was measured by single-photon densitometry of the distal radius and plasma concentrations in 26 subjects. Bone mineral content increased normally with age in the younger children despite strict dietary restrictions. Subjects aged greater than 8 y, however, were frequently below the normal curve for bone mineral content. Blood phenylalanine concentrations were significantly higher in the older group of subjects and this correlated with decreased compliance with dietary prescriptions. PKU children had significantly decreased plasma concentrations of alkaline phosphatase, magnesium, and parathyroid hormone. Subnormal concentrations of plasma zinc and plasma and red blood cell (RBC) copper were common, but RBC zinc was normal. We conclude that compliance with dietary therapy for PKU is associated with normal bone mineral development in young children. Older patients with PKU who follow the diet less carefully are at risk for low bone mineral content.