Nancy Wexler Is a Doctor Who Has Studied Huntington's Disease for Many Years. Much of Her

Introduction Nancy Wexler is a doctor who has studied Huntington's disease for many years. Much of her work has been conducted in an area of Venezuela where most of the people over 40 already have Huntington's disease. Wexler estimates that at least half of the other people in the area have the dominant Huntington's gene that will cause them to have the disease later in life. Nancy Wexler and her sister have a vested interest in Huntington's disease, since their mother died from the disease. Nancy's father did not have Huntington's. Nancy has helped to develop a test for Huntington's disease, and it is now possible to test for the gene before symptoms appear. Nancy is not certain whether she and her sister should have the test. She has tested many people in Venezuela but has not told them the results of the test. The reason for not sharing the information is that there is no cure or treatment for Huntington's and knowing the test results will do nothing to stop the development and progression of the disease. The year was 1979, and Congress has just issued you the funding to study Huntington's disease- a disorder afflicting more than 50,000 Americans. Very little was known about the inheritance of Huntington's, but Dr. Nancy Wexler (who's mother died of it) knew about the incredibly high rate of the disease around a place called Lake Maracaibo, Venezuela. Here, you'll be a part of a team sent to Lake Maracaibo to study a large family (of more than 5,000 member!), and report your findings! The family spans five generations. It is your job (as it was hers) to pedigree the family and see what's going on here! Below is a fax that has arrived from Lake Maracaibo... Dear scientists, What follows are the results of our interviews with 38 members of the Gonzales family. see what you can make of it. Sincerely. the team Generation V: Luis, son of Zelda and Ramon. AFFLICTED Generation IV: Ramon, son of Ricardo and Lydia. AFFLICTED Zelda, married to Ramon. HEALTHY Felipe, brother of Ramon. AFFLICTED Juan, son of Miguel and Letty. AFFLICTED Cira, daughter of Miguel and Letty. HEALTHY Roberto, son of Miguel and Letty. HEALTHY Nora, daughter of Jesus and Margarita, AFFLICTED Alejandro, son of Pedro and Beatriz. AFFLICTED Delia, daughter of Dano and Andrea. AFFLICTED Tio, son of Dano and Andrea. AFFLICTED Maria, daughter of David and Guadelupe. AFFLICTED Nariza, daughter of David and Guadelupe. AFFLICTED Generation III: Lydia, daughter of Carlos and Imelda. AFFLICTED Ricardo, married to Lydia. HEALTHY Helga, daughter of Carlos and Imelda. HEALTHY Letty, daughter of Carlos and Imelda. AFFLICTED Miguel, married to Letty. HEALTHY Margarita, daughter of Carlos and Imelda. AFFLICTED Jesus, married to Margarita. HEALTHY Beatriz, daughter of Carlos and Imelda. AFFLICTED Pedro, married to Beatriz. HEALTHY Juanita, daughter Javier and Bonita. AFFLICTED Benito, son of Javier and Bonita. HEALTHY Dano, son of Chito and Chelita. AFFLICTED Andrea, wife of Dano. HEALTHY David, son of Chito and Chelita. AFFLICTED Guadelupe, wife of David. HEALTHY Horatio, son of Chito and Chelita. HEALTHY Lucio, son of Chito and Chelita. HEALTHY Generation II: Imelda, daugher of Rigo and Esmerelda. AFFLICTED Carlos, married to Imelda. HEALTHY Javier, son of Rigo and Esmerelda. AFFLICTED Bonita, married to Javier. HEALTHY Chito, son of Rigo and Esmerelda. AFFLICTED Chelita, married to Chito. HEALTHY Generation I: Rigo. AFFLICTED Esmerelda. HEALTHY

Classroom Scenario written by Jeffrey Weld ([email protected]), Pella High School, Pella, IA. http://ed.fnal.gov/help/97/peretz/inherit/inherit3a.html