Abstract
A central characteristic of people with Prader-Willi Syndrome (PWS) is an apparent insatiable appetite leading to severe overeating and the potential for marked obesity and associated serious health problems and premature death. This behaviour may be due to the effects of the genetic defect resulting from the chromosome 15 abnormalities associated with the syndrome. We examine the ethical and legal dilemmas that can arise in the care of people with PWS. A tension exists between a genetic deterministic perspective and that of individual choice. We conclude that the determination of the capacity of a person with PWS to make decisions about his/her eating behaviour and to control that behaviour is of particular importance in resolving this dilemma. If the person is found to lack capacity, the common law principles of acting in a person's "best interests" using the "least restrictive alternative" may be helpful. Allowing serious weight gain in the absence of careful consideration of these issues is an abdication of responsibility.
Full text
PDF
Selected References
These references are in PubMed. This may not be the complete list of references from this article.
- Dykens E. M., Goff B. J., Hodapp R. M., Davis L., Devanzo P., Moss F., Halliday J., Shah B., State M., King B. Eating themselves to death: have "personal rights" gone too far in treating people with Prader-Willi syndrome? Ment Retard. 1997 Aug;35(4):312–314. doi: 10.1352/0047-6765(1997)035<0312:ETTDHP>2.0.CO;2. [DOI] [PubMed] [Google Scholar]
- Greenswag L. R. Adults with Prader-Willi syndrome: a survey of 232 cases. Dev Med Child Neurol. 1987 Apr;29(2):145–152. doi: 10.1111/j.1469-8749.1987.tb02129.x. [DOI] [PubMed] [Google Scholar]
- Holland A. J., Treasure J., Coskeran P., Dallow J. Characteristics of the eating disorder in Prader-Willi syndrome: implications for treatment. J Intellect Disabil Res. 1995 Oct;39(Pt 5):373–381. doi: 10.1111/j.1365-2788.1995.tb00541.x. [DOI] [PubMed] [Google Scholar]
- Ledbetter D. H., Riccardi V. M., Airhart S. D., Strobel R. J., Keenan B. S., Crawford J. D. Deletions of chromosome 15 as a cause of the Prader-Willi syndrome. N Engl J Med. 1981 Feb 5;304(6):325–329. doi: 10.1056/NEJM198102053040604. [DOI] [PubMed] [Google Scholar]
- Swaab D. F., Purba J. S., Hofman M. A. Alterations in the hypothalamic paraventricular nucleus and its oxytocin neurons (putative satiety cells) in Prader-Willi syndrome: a study of five cases. J Clin Endocrinol Metab. 1995 Feb;80(2):573–579. doi: 10.1210/jcem.80.2.7852523. [DOI] [PubMed] [Google Scholar]