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Text Box: The Case Jeff Kerwin's (a pseudonym), life and death, at age 37, are testimony to the very real service delivery dilemma: when client rights and choices clash with professional responsibilities. Jeff was diagnosed as having Prader-Willi syndrome (PWS) when he was in his 20s. PWS is a complex disorder with multiple physical, cognitive, and behavioral characteristics. Treatment of PWS focuses on addressing its symptoms, chiefly the excessive weight gain. Absent interventions, the prognosis is bleak. Jeff lived at home in his early years. Primary among his problems, according to his mother, was his uncontrollable eating and excessive weight, which fluctuated between 200 to over 400 pounds. He was less than five feet tall. At age 30 he was admitted to a community residence developed to serve individuals with PWS.

Text Box: According to his mother and residence staff, the years he spent in this facility were among the healthiest. When he entered the facility he weighed over 400 lbs but through behavior controls, diet, exercise, and counseling, Jeff lost over 250 lbs and was within 30 lbs of his ideal body weight. He also developed a sense of what were his "individual rights" and an increased determination to exercise his views on the matter. After more than five years in the program, Jeff demanded to be discharged. He was adamant about not being placed in a restrictive PWS residence. His attempts at independent living was fraught with problems, especially weight gain (eventually to approximately 500 lbs) and leg ulcers. A new case manager from a private agency notified her supervisor of her concern over Jeff's health. He had difficulty walking even 10 feet. He rejected agency staff suggestions of placement in a supervised

Text Box: residence, but agreed to admission to a PWS rehabilitation program in Pennsylvania, knowing by this time that his PWS was out of control. But he refused to allow anyone, including visiting nurses, in to assess his physical condition. Anticipating that he would be approved for admission to the Pennsylvania program, staff started to make arrangements for his transportation. However, prior to his transfer, Jeff died in his sleep. His death was attributed to congestive heart failure; PWS was identified as a contributing factor. The Lesson Jeff was released from a protective, yet restrictive, environment of supervised living. Professionals believed they acted on Jeff’s behalf by respecting, even facilitating, his wish to live independently, but in hindsight were uncomfortable with the outcome—his death. Increasingly, providers are facing the dilemma posed when professional responsibility and client

Text Box: Could This Happen in Your Program?” originated as a brochure series, intended as a teaching tool, primarily for direct care staff. The objective of the series is to prevent mishaps in mental hygiene facilities/programs by challenging staff, sparking reflection and discussion about policies and practices, with the need to clarify or revise these to reduce the likelihood of similar incidents. Tom Harmon from the New York State Commission on Quality Care originated the idea for the series, which he proposed for “assisting people in the trenches” improve quality of mental hygiene services. The entire series can be viewed on the internet at http://www.cqc.state.ny.us/

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Text Box: choice clash as the service system evolves from a very paternalistic mode—in which clients’ choices and wishes played a back seat role to "professional wisdom" about what is in their best interest—to one in which service recipients are seen as equal partners in steering the course of service delivery and whose wishes and choices should be as valued as the wisdom and advice of professionals. While there are no easy answers as to what to do when partners in the service compact disagree over which direction their shared venture should go, there are guideposts to aide professionals in difficult decision-making. Jeff’s legacy is a challenge to all service providers to reflect upon his life—his disabilities, his abilities and wishes, the services provided, and the decisions made—in order that they are better prepared to respond to a conflict for which there are no easy answers: when clients’ rights and choices clash with professional responsibilities.

Text Box: What is PWS? It is a complex genetic disorder that typically causes low muscle tone, short stature, incomplete sexual development, cognitive disabilities, problem behaviors, and a chronic feeling of hunger that can lead to excessive eating and life-threatening obesity. Is PWS inherited? Most cases are attributed to a spontaneous genetic error that occurs at or near the time of conception for unknown reasons. In very rare cases, a genetic

Text Box: mutation is passed from parent to child. How common is PWS? 1 in every 12,000 to 15,000 people have PWS. It affects both sexes and all races. When does the overeating begin? Usually between 2 and 5 years old. Newborns with PWS do not overeat and often low muscle tone affects their ability to suck so they are at risk of suffering from “failure to thrive” if feeding difficulties are not monitored and treated.

Text Box: Do diet medications work? No medications have worked consistently to suppress appetite in those with PWS. Following a low calorie diet, exercise, and limited access to food is required for most individuals. What behavior problems are common? People with PWS have a tendency to have obsessive/compulsive behaviors , such as picking at skin irritations, collecting and hoarding possessions, repetitive thoughts and verbalizations, and a strong

Text Box: need for routine and predictability. Frustration and changes to routine can set off tears, temper tantrums, or physical aggression. Information for this article was obtained from The Foundation for Prader-Willi Research www..fpwr.org, and The USA Prader-Willi Syndrome Association www.pwsausa.org. For more information on Prader-Willi please visit the previous listed websites.