Marta Perez: A Young Adult with Phenylketonuria

Price (includes one year of access and CE):  US$8 Course Code: 7SENNF-PRV-09-ddn13s12 CE: 1.00 Hours. Post Test Score Required for Successful Completion: 80%

Course Overview:

Phenylketonuria (PKU) is a genetic disease in which phenylalanine builds up in the body causing brain damage and mental retardation. Today, newborns are routinely screened for PKU, which can be controlled by severely limiting the intake of protein. In the past, it was believed safe to discontinue the PKU diet during childhood, but studies show that adults who go off the diet develop problems, including reduced intellectual functioning, abnormal changes in the brain and nerves, poor social adaptation, and an increased incidence of mental illness. This case scenario involves a newly married woman with PKU who grew and developed without brain damage or mental retardation because she restricted her intake of phenylalanine until adolescence, at which time she went off the diet. She now returns to the PKU Clinic because she wants to have a baby, which means she must resume the diet to prevent prenatal damage to the baby from maternal PKU. As she discusses her feelings about this with the clinic nurse, she also reveals that she is having problems concentrating and feels depressed. The nurse helps the woman express her feelings, teaches about foods and medications that are safe to include in the diet, provides referrals to support resources, and helps the woman to develop a plan of self-care that includes goals, methods, and measures of success.

Course Objectives:

After completion of this learning activity, the learner will be able to:

• describe the role of each member in the partnership triad as he or she is influenced by the environmental, personal, and caring contexts present.
• apply transpersonal teaching-learning methods that help the nurse gain understanding of the client's frame of reference in order to help the client eliminate barriers to successful attainment of health goals related to increased participation and quality-of life.
• describe the relationship between high phenylalanine levels and fetal damage that can be caused by maternal PKU.
• describe the relationship between high phenylalanine levels and potential adult-onset complications of PKU that can affect participation in and quality-of-life.
• identify physical, social, and emotional needs related to resuming a low-phenylalanine diet.
• describe an individualized, collaborative approach to planning care for an adult with PKU.
• provide nonjudgmental and caring validation of the feelings of a client who is coping with the lifestyle changes necessitated by a low-phenylalanine diet.
• locate web-based PKU resources appropriate for use by clients.

This courseware was made possible in part by grant number R43 NR04738-03 from the National Institute of Nursing Research (NINR). Its contents are solely the responsibility of the authors.

Copyright© 2007 by HealthSoft, Inc. All Rights Reserved.

HealthSoft, Inc. is an approved provider of continuing nursing education by the New York State Nurses Association, an accredited approver by the American Nurses Credentialing Center's Commission on Accreditation.

It has been assigned code 7SENNF-PRV-09.

This course is updated annually. Expiration date: 5/1/11

The faculty and planners of this course have no vested interests to disclose.

This course has received no commercial support.