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Desiree Torres

Lesch-Nyhan Syndrome

November 2012

Lesch-Nyhan Syndrome
Lesch-Nyhan syndrome is a condition characterized by the overproduction and accumulation of uric acid, a waste product of normal chemical processes that is found in blood and urine. The overproduction of uric acid can cause gouty arthritis (arthritis caused by an accumulation of uric acid in the joints), kidney stones, and bladder stones. Problems with the nervous system and behavioral disturbances are also characteristic of this disorder.

Characteristics
Irritability Nervous System Impairment o Lack of muscle tone o Spasms of limbs and facial muscles Kidney Stones Blood in the urine Pain and swelling of joints Difficulty swallowing Impaired kidney function Self-injury The severity of effects on intelligence vary

result of lip chewing

Prevalence
The occurrence of Lesch-Nyhan is very rare, about 1 in 380,000 live births. It is not more prevalent in any ethnicity but is much more common in males than females.

Lesch-Nyhan Syndrome is named after Doctors Michael Lesch and William Nyhan who diagnosed the first case in 1964.

Desiree Torres

Lesch-Nyhan Syndrome

November 2012

Lesch-Nyhan Syndrome in the Classroom


Children who have Lesch-Nyhan Syndrome engage in self injurious behavior when under stress. Being part of a traditional classroom, with the pressure to be at the same level as other children, causes stress and for this reason, it is more beneficial for a child with LeschNyhan to be in special education classrooms. If protective devices are in place and the child is not engaging in self injurious behavior then other Lesch-Nyhan behaviors, such as spitting or cursing should be ignored. Children with LeschNyhan engage in such behaviors with a desire to receive negative attention and giving any attention (including grimacing) will only encourage these behaviors. The most effective learning actually takes place outside of the classroom, in real-life experiences such as dealing with money and socializing. These skills are important to build the self-esteem that children with Lesch-Nyhan Syndrome need to decrease stress and self injurious behavior Can a child with Lesch-Nyhan Syndrome be placed in a traditional classroom? Whether or not a traditional classroom setting is appropriate would depend on the child since symptoms can vary in severity. Although not suggested, it is possible for a child with less severe symptoms to function in a traditional classroom.

Teaching/Intervention Tips
Traditional classroom settings are not suggested. Use real-life settings and activities as learning opportunities. Use comfortable protective devices to prevent self injury. Use hand over hand method to guide fine motor skill activities. Selectively ignore negative behavior. Use self-esteem building activities.

Desiree Torres

Lesch-Nyhan Syndrome

November 2012

Professional Organizations
National Organization for Rare Disorders http://www.rarediseases.org/ The National Organization for Rare Disorders (NORD) is a non-profit organization that supports the research of rare disorders such as Lesch-Nyhan Disease. The organization provides families and patients with information and mentors.

Parent Organizations
International Lesch-Nyhan Disease Association http://www.lesch-nyhan.org/ This organization was created to help families with children who have LeschNyhan Disease. It provides them with information in understanding how LeschNyhan disease works, treatment options and care providers. Purine Research Society http://www.purineresearchsociety.org/ The Purine Research Society is an organization created by parents of children who are hyperuricemic.

Lesch-Nyhan Syndrome Support Group http://www.mdjunction.com/lesch-nyhansyndrome The Lesch-Nyhan Syndrome Support Group was created so that parents of children with Lesch-Nyhan Syndrome could reach out to each other and share

Lesch-Nyhan Syndrome
Desiree Torres
Resources Lesch-nyhan syndrome. (2012, November 12). Retrieved from http://ghr.nlm.nih.gov/condition/lesch-nyhan-syndrome http://ghr.nlm.nih.gov/condition/lesch-nyhan-syndrome This website gives information about the related genes and inheritance of Lesch-Nyhan Syndrome. Badash, M. (2012). Lesch-nyhan syndrome. Retrieved from http://www.med.nyu.edu/content?ChunkIID=22829 http://www.med.nyu.edu/content?ChunkIID=22829 This website provides a break down of the basics of Lesch-Nyhan Syndrome such as definition, prevalence and characteristics. Official website of the lesch nyhan disease international study group . (2006). Retrieved from http://www.lesch-nyhan.org/en/ http://www.lesch-nyhan.org/en/ This website provides international research information for people around the world. Ishmael, Holly; Frey, Rebecca. "Lesch-Nyhan Syndrome." Gale Encyclopedia of Medicine, 3rd ed.. 2006. Retrieved November 19, 2012 from Encyclopedia.com: http://www.encyclopedia.com/doc/1G2-3451600961.html http://www.encyclopedia.com/topic/Lesch-Nyhan_syndrome.aspx#1 This website gives detailed explanations of what Lesch-Nyhan disease is and explains key terms for better comprehension. Institute for research in developmental disabilities . (n.d.). Retrieved from http://www.matheny.org/institute_for_research.htm http://www.matheny.org/institute_for_research.htm

November 2012

Place Logo Or Company Name Here This website provides suggestions for appropriate educational settings for children with Lesch-Nyhan Syndrome and appropriate
responses and actions for teachers of children with Lesch-Nyhan Syndrome.
Street Address, Suite 555 City, State 55555