Food insecurity in females with phenylketonuria.

Abstract

Phenylketonuria (PKU) is a genetic disorder characterized by insufficient metabolism of phenylalanine. Depending on severity, patients follow a low-phenylalanine diet and may consume medical food (MF) and low-protein modified foods; dietary and medical treatment can be expensive. This study assessed prevalence of food insecurity (FI), the lack of resources to access enough nutritious food to have an active, healthy life, in females with PKU and examined associations with diet and metabolic control. Participants were recruited from a research-based camp in 2018. Adult and adolescent modules of the USDA Household Food Security survey were utilized to categorize participants as food secure [high food security (FS) or marginal FS] or food insecure (low FS or very low FS); results were compared to the general U.S. population. Dietary intake via three-day food records and plasma amino acids were also assessed. Thirty females 11-58 years of age (mean = 21.4 years) participated. Twelve (40%), including seven adolescents (44%) and five adults (36%), were FI compared to the U.S. prevalence of 11.1%. MF protein intake was significantly lower in those with very low FS compared to high FS and low FS (P = .04). Age and intact protein intake were significantly higher in those with very low FS compared to high FS (P < .05). Our study suggests adolescent and adult females with PKU have a higher prevalence of FI than the general U.S. population. Those with very low FS were older, consumed more dietary phenylalanine and intact protein, and less MF protein. Clinicians should consider screening for FI in patients with PKU.

• Authors: Kathryn E Coakley, Suzanne Porter-Bolton, Mary L Salvatore, Rosalynn B Blair, Rani H Singh
• Journal: JIMD reports (JIMD Rep) Vol. 53 Issue 1 Pg. 103-110 (May 2020) ISSN: 2192-8304 [Print] United States
• PMID: 32395415 (Publication Type: Journal Article)
• Copyright: © 2020 The Authors. Journal of Inherited Metabolic Disease published by John Wiley & Sons Ltd on behalf of SSIEM.