Living with Phenylketonuria: Perspectives of Patients and Their Families

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 in 1997 (response rate: 64%). It included separate components for caretakers and patients aged 10 to 18. While 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. Phe test results were regarded as the principal signal for the need for dietary adjustment. A home monitor, it was widely agreed, would facilitate better management through more regular and timely feedback. 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 that a home monitor to be an important contributor to the successful management of PKU.