Living with Phenylketonuria: Perspectives of Patients and Their Families

Details

• Personal Author:
  Bilginsoy, Cihan ; Ernst SL ; Leonard CO ; Waitzman N
• Description:
  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. [Description provided by NIOSH]
• Subjects:
  Communicable Disease Control Diet Energy Metabolism Health Care Facilities Workforce And Services Health Personnel Occupational Health Safety Surveys And Questionnaires
• Keywords:
  Children Dietary-effects Disease-control Health-care Health-care-personnel Health-surveys Medical-monitoring Medical-treatment Metabolic-disorders Phenyl-compounds Questionnaires

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• ISSN:
  0141-8955
• Document Type:
  Text
• Funding:
  Grant
• Genre:
  Journal Article
• Place as Subject:
  OSHA Region 8 ; Utah
• CIO:
  National Institute for Occupational Safety and Health (NIOSH)
• Topic:
  Workplace Safety & Health
• Location:
  North America
• Volume:
  28
• Issue:
  5
• NIOSHTIC Number:
  nn:20037447
• Citation:
  J Inherit Metab Dis 2005 Sep; 28(5):639-649
• Contact Point Address:
  C. Bilginsoy, University of Utah, Department of Economics, 1645 Central Campus Dr., Room 308, Salt Lake City, UT 84112
• Email:
  bilginsoy@economics.utah.edu
• Federal Fiscal Year:
  2005
• Performing Organization:
  University of Utah
• Peer Reviewed:
  True
• Start Date:
  20050701
• Source Full Name:
  Journal of Inherited Metabolic Diseases
• End Date:
  20280630
• Collection(s):
  National Institute for Occupational Safety and Health
• Main Document Checksum:
  urn:sha-512:b4ce9ba3d714f7b43a9b2eaf747d0c825baf435d551fc7c81a3dd5328438dd87ee0b0adcc3f6084dd25c163d54333d6ff2e20b65feef5251bca73ef5406995be
• Download URL:
  https://stacks.cdc.gov/view/cdc/188542/cdc_188542_DS1.pdf
• File Type:
  [PDF - 116.62 KB ]