Dietary Management of Propionic Acidemia: Parent Caregiver Perspectives and Practices
Financial disclosure: U.S. Department of Health and Human Services, National Institutes of Health; National Human Genome Research Institute, Division of Intramural Research (ZIAHG200395); Social and Behavioral Research Branch; Principal Investigator, l.m.k.
Conflicts of interest: None declared.
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.
