Prader-Willi Syndrome
Prader-Willi Syndrome: An Investigation into Paternal and Maternal Origins & Characteristics
Abigail McNeal
Liberty University
COUN 502- Professor Myers
August 15, 2011
Abstract
Prader-Willi syndrome, (PWS) is a genetic disorder that occurs in about one in every eight thousand births in the United States. PWS occurs when there is a defect or deletion in the fifteenth chromosome from either maternal or paternal origins. Individuals diagnosed with PWS need enhanced supervision to address the constant need to over eat. Current research identifies that the paternal chromosome presents with significant maladaptive behaviors. Behavioral issues commonly known to the disorder differ dependent on the origin of the parental chromosome. Obesity, physical aggression, temper tantrums, ritualistic behaviors, stubbornness, and self- injurious behavior (skin picking), along with other maladaptive behaviors are all characteristic of individuals with PWS. Due to an increased awareness, and accurate and reliable genetic testing, diagnosis of PWS occurs earlier than in the past. Recommendations for conducting assessments, applying behavior management strategies, and environmental adaptations are all supported by research in managing behaviors among individuals with PWS regardless of the origins.
Keywords: Prader-Willi syndrome, obesity, self-injurious behaviors, skin picking
Prader-Willi Syndrome
Every year in the United States, one in every sixteen thousand babies are born with PWS. PWS is the first human disease attributed to genomic imprinting (Mahgoub 2007). PWS according to Maas, Sinnema, Didden, Maaskant, Smits, Schrandel-Stumpel & Curfs (2010), is caused by a deletion or defect on chromosome fifteen of the paternal copy of a maternally imprinted gene. Infants with PWS have difficulty latching on to their mother’s nipple for nursing and sucking from a bottle; both resulting in alternate feeding methods to increase weight gain. According to Kundert (2008), “Two
Abigail McNeal
Liberty University
COUN 502- Professor Myers
August 15, 2011
Abstract
Prader-Willi syndrome, (PWS) is a genetic disorder that occurs in about one in every eight thousand births in the United States. PWS occurs when there is a defect or deletion in the fifteenth chromosome from either maternal or paternal origins. Individuals diagnosed with PWS need enhanced supervision to address the constant need to over eat. Current research identifies that the paternal chromosome presents with significant maladaptive behaviors. Behavioral issues commonly known to the disorder differ dependent on the origin of the parental chromosome. Obesity, physical aggression, temper tantrums, ritualistic behaviors, stubbornness, and self- injurious behavior (skin picking), along with other maladaptive behaviors are all characteristic of individuals with PWS. Due to an increased awareness, and accurate and reliable genetic testing, diagnosis of PWS occurs earlier than in the past. Recommendations for conducting assessments, applying behavior management strategies, and environmental adaptations are all supported by research in managing behaviors among individuals with PWS regardless of the origins.
Keywords: Prader-Willi syndrome, obesity, self-injurious behaviors, skin picking
Prader-Willi Syndrome
Every year in the United States, one in every sixteen thousand babies are born with PWS. PWS is the first human disease attributed to genomic imprinting (Mahgoub 2007). PWS according to Maas, Sinnema, Didden, Maaskant, Smits, Schrandel-Stumpel & Curfs (2010), is caused by a deletion or defect on chromosome fifteen of the paternal copy of a maternally imprinted gene. Infants with PWS have difficulty latching on to their mother’s nipple for nursing and sucking from a bottle; both resulting in alternate feeding methods to increase weight gain. According to Kundert (2008), “Two
References: Kundert, D. K. (2008).Prader-Willi syndrome. School of Psychology Quarterly, 23(2), 246-257. doi:10.1037/1045-3830.23.2.246 Maas, A Mahgoub, N. A. (2007). Prader-Willi syndrome. Journal of Neuropsychiatry & Clinical Neuroscience, 19(2), 203.