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JPEN J Parenter Enteral Nutr. 2019 Mar;43(3):434-437. doi: 10.1002/jpen.1461. Epub 2018 Oct 24.

Dietary Management of Propionic Acidemia: Parent Caregiver Perspectives and Practices.

Author information

1
Social and Behavioral Research Branch, Social Network Methods Section, National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland, USA.
2
Medical Genomics and Metabolic Genetics Branch, National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland, USA.

Abstract

BACKGROUND:

Propionic acidemia (PA), an autosomal recessive metabolic disorder, has an estimated incidence of 1:105,000-130,000 in the United States.1,2 Nutrition management is a main intervention for PA. Research in inborn errors of metabolism such as phenylketonuria has identified association of parental perceptions and practices with dietary outcomes. Parental perceptions and practices in the nutrition management of PA have not been investigated.

OBJECTIVE:

To assess the dietary perceptions and practices of parental caregivers of children affected by PA.

METHODS:

PA parents were surveyed about perceptions and practices associated with feeding their affected child(ren). The single-page survey was anonymous, and responses to survey items were not identifiable. Parents provided information on how often they followed the prescribed diet and the rationale for any adjustments.

RESULTS:

Parents "always" or "most of the time" followed the prescribed diet for children 4-20 years of age; yet, open-ended responses indicated that 71.4% made situational adjustments to their child's diet for a variety of reasons, including illness, iatrogenic effects, and social events.

CONCLUSIONS:

PA parents make situational adjustments to their child's highly specialized diet. Uncertainty exists as to the situational adjustments being within the guidelines used by the metabolic healthcare team who rely on parents to inform them about dietary situational adjustments.

KEYWORDS:

Inborn error of metabolism; caregiving; dietary adherence; dietary situational adjustments; propionic acidemia

PMID:
30357861
PMCID:
PMC6395508
[Available on 2020-03-01]
DOI:
10.1002/jpen.1461

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