Gene Cosens: A Young Adult with Prader-Willi Syndrome

Price (includes one year of access and CE):  US$10 Course Code: 7SENNF-PRV-09-ddn13s18 CE: 1.50 Hours. Post Test Score Required for Successful Completion: 80%

Course Overview:

Prader-Willi syndrome (PWS) is a genetic condition characterized by uncontrolled appetite causing obesity, mental retardation, and other physical characteristics. It can cause significant limitations, which make it difficult for an adolescent to achieve independence and self-determination. For the past two years, a nineteen-year-old man has been a resident of a group home for people with Prader-Willi syndrome. Before moving to the home, it became increasingly difficult for the man's parents to provide the extensive supervision that he required. He aggressively had begun to pressure neighbors for food during the day, and sneak out at night while his parents slept to forage in garbage cans. These behaviors resulted in a 50-pound weight gain over three months. In this case study, nurses and caregivers work to maximize the man's self-determination and quality of life, while maintaining an optimal level of health and safety within the limits imposed by the nature of PWS. The conflicting goals implied in this statement highlight an ethical dilemma regarding the right of people with PWS to decide for themselves what and how much to eat. In this case scenario, this dilemma is resolved when the nurse, caregiver, and young man work together to establish a safe and caring environment.

Course Objectives:

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

• identify the priority health care challenge for a person with Prader-Willi syndrome.
• identify three major challenges facing a person with Prader-Willi syndrome.
• describe two main forces motivating a person with Prader-Willi syndrome.
• identify interventions to assist a person with Prader-Willi syndrome to control food intake, reduce obsessive compulsive behavior, and maintain optimal health.
• discuss ethical rationale for use of environmental restriction and restriction of the right to freely spend money as parts of the plan of care for a person with Prader-Willi syndrome.

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.