Date of Award

2015

Document Type

Open Access Thesis

Department

Genetic Counseling

First Advisor

Emily Jordon

Abstract

Maternal PKU syndrome is the collection of features and birth defects that arise from uncontrolled phenylalanine levels in a pregnant woman with phenylalanine hydroxylase (PAH) deficiency, previously known as phenylketonuria (PKU). Currently, the literature is lacking in exploring what young women are being told about maternal PKU syndrome. In this study, communication patterns between parents and their daughters about maternal PKU syndrome were investigated through an online survey completed by parents. The survey assessed parents’ level of comfort with this topic, the information they discussed with their daughter and the resources they accessed. Followup interviews were conducted with participants to elaborate on survey responses. The majority of participants were very comfortable discussing maternal PKU syndrome (51%); however, 21% reported being very uncomfortable with the discussion. Parents most often discussed health concerns associated with maternal PKU syndrome (94%) and least frequently discussed unplanned pregnancies (58%). The most frequently used resource was a metabolic doctor (73%); however, parents included mothers with PAH deficiency among the most helpful resources. Themes from open-ended responses and interviews emphasized qualities of parents’ discussion with their daughters. Parents commented on the timing and how they presented the information to their daughters. They described challenges such as the emotional impact of the conversation as well as their daughter’s decision-making and consequences of her actions. Lastly, they discussed advice for treatment compliance, resources, and ways to normalize the topic of maternal PKU syndrome. These results can help genetic counselors better understand the parent daughter conversation so that healthcare professionals can better assess and inform families about maternal PKU syndrome.

Rights

© 2015, Hannah Beth Andrews

Share

COinS