Caring for Maternal PKU: Part II

Maternal PKU, Phenylketonuria, Maternal PKU Knowledge Test

For the second installment in the Caring for Maternal PKU series, let’s take a closer look at the knowledge factors incorporated into the participant interviewee questions for Kimberly R. Regis’ Doctor of Nursing Practice final project, Childbearing age women with PKU: Assessment of care needs and preferences. In Part I of the series, questions four and five asked:

4. What is the knowledge level of the participants regarding PKU and maternal PKU?

5. How does knowledge level regarding PKU impact decisions regarding birth-control and family planning?

Why would Regis focus on the knowledge level of the adult women with PKU? For the last two decades, PKU research has focused in large part on emphasizing the need to educate adult women with PKU so they could better self-manage their condition and prevent the occurrence of maternal PKU syndrome. Regis wanted to “gain a better understanding of the ways in which a children’s hospital-based metabolic specialty clinic may best serve them [adult PKU women of childbearing age].” Was the answer that clinics should continue to serve maternal PKU patients by following conventional research, which emphasized the importance of ongoing education? Or was there a need for something more?

To explore these options, Regis started with the knowledge variable and had the six participants take the Maternal PKU Knowledge Test, a 10-question quiz that was developed in 1990 by Shiloh, St. James and Waisbren. The three authors recommended the test as a way for clinics to screen and identify adult women with PKU who might benefit from more education.

The study’s participants answered a majority of the questions correctly, with scores ranging from seven to 10 correct. The question that elicited the most trouble when trying to answer was number seven (you can see a complete list of questions and take the test for yourself by visiting the Maternal PKU Knowledge Test page on PKU Parlor). A common theme that emerged from the open-ended questions was that participants felt they received vague and oversimplified information, which lacked details for explaining the science behind why a developing fetus could be harmed by poor dietary control. One participant stated she had never been taught about maternal PKU saying, “I don’t even remember having the conversation.”

With regard to how the participants’ knowledge level may have impacted their family planning decisions, all of the participants reported that PKU strongly influenced their decision about whether or not to have children. Regis noted that one participant commented that she was afraid to have children because of the possible outcomes, while another participant who originally wanted a large family said that concerns over the PKU diet changed the number of children they would ultimately plan for.

“I never thought growing up that I could never had kids. I always had the big family in my head—five kids, four kids.”

Observations within the study also revealed that even though there were 12 pregnancies among the six participants, only four resulted in live births. So one begs to ask the question: If these six women were relatively well-informed about the risks associated with maternal PKU…AND if knowledge of those risks, as well as ways to minimize those risks, impacted the decisions these women made with regard to family planning, then what else could be the reason behind such a low pregnancy success rate?

Stay tuned for the concluding post in this three-part series on Caring for Maternal PKU. Assuming that education alone may not be enough to improve pregnancy success rates among adult PKU women, Part III will take a look at the support systems available to the participants in Regis’ study.

–NM

Caring for Maternal PKU: Part I

Caring for Maternal PKU: Part III

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5 Comments

Filed under Lo-pro Diet Management, Maternal PKU, Research

5 responses to “Caring for Maternal PKU: Part II

  1. Pingback: Caring for Maternal PKU: Part I | PKU Parlor

  2. Pingback: Caring for Maternal PKU: Part III | PKU Parlor

  3. Betty Yoo

    Do both parents have to have PKU genes for the offspring to be diagnosed with PKU? Or will the gene from even one parent cause the child to be diagnosed with PKU?

    • That’s a great question, Betty. Thank you for thinking to ask it! PKU is inherited through a recessive gene, which means in order for someone to have PKU, they would need to inherit one recessive gene from each parent. You can be considered a “carrier of PKU” if you inherited a recessive gene from one parent but also the non-PKU dominant gene from the other parent. In this case, the offspring would not have symptoms of PKU, but he/she could pass along the gene to their future offspring. Hope this explanation is helpful!
      –NM

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