HEALTH & FITNESS wellness/on the cover THE WILL TO OVERCOME from page A27 friend who has CP [cerebral palsy]." Subia describes his symptoms as "more of an ache than a pain. My muscles stiffen up, especially overnight. At night, I'll get cramps in my legs or arms," which afflicts Horenstein as well. Cruelly, "there's nothing to be done" to stop the disease's progression, Subia says. Neither is on medication. Both men did take part in an unsuc- cessful 2003-2006 clinical trial of the drug Zavesca, which was adminis- tered at New York University School of Medicine and Cleveland University Hospital. "I got Joe involved in the study," says Subia, who had consulted one of its co-directors, Dr. Barbara Shapiro, M.D., PhD., associate professor of neurology at Case Western Reserve School of Medicine in Cleveland. Trying To Treat Horenstein was encouraged when some of his symptoms appeared to diminish. However, he was taken off the drug when he developed tremors in his hands. "Tremors can be part of the disease itself, but the Zavesca also causes trem- ors in about a third of the patients who take it," says Shapiro. "We did not find any statistically sig- nificant benefit of Zavesca," she says, "although some of the investigators had Mike Subia: "My legs began to hurt and that's how I found out." "Much research is directed at optimizing drug delivery into the nervous system as well as insights into the molecular processes that underlie neurodegeneration in Tay-Sachs." Dr. John Fink the impression there was improvement in some of the patients' speech." Shapiro also studies the best anti- depressant medications to use for the one-third to half of LOTS patients who have psychiatric problems. Mental ill- nesses, such as psychosis and bi-polar disease, "are part of the disease itself, not just a reaction to it," she says. "Some medications patients should avoid, while others are relatively safe and beneficial," Shapiro says. Research to combat LOTS is ongoing, according to Dr. John Fink, M.D., pro- fessor of neurology at the University of is specially fitted so that he can propel "walk" the three blocks to Friday and his wheelchair to a position right behind the steering wheel. Both men use hand controls to drive. Saturday services held by the small Orthodox minyan to which he and his family belong. Although Horenstein, who lives alone, can work on cases out of his house, he must travel to the Oakland County As the disease progresses, "We don't "Much research is directed at optimiz- ing drug delivery into the nervous system as well as insights into the molecular pro- cesses that underlie neurodegeneration in Courthouse complex to defend the indigent criminals he works with. He has two adopted children and is a son do as many things together," says his wife, Alice Subia, who is from the Netherlands. "Air travel is harder." Tay-Sachs," says Fink. "Although it will take some time, these approaches will ultimately lead to real of the late Hy Horenstein, who ran Hy Horenstein's Delicatessen for more than 50 years. "It's made me a little more respon- sible," says son Sam, 14, who attends Eton Academy in Birmingham while treatments for this condition," he predicts. That research could possibly be stepped up as President Barack Obama Subia has an office close to home and regularly drives to see customers. He is also able to use his wheelchair to his sister Julianne, 12, is at Hillel Day School of Metropolitan Detroit in Farmington Hills. "I kind of relate to a promises to lift the limits on stem cell research imposed by the Bush adminis- tration. A28 Febr uary 26.2009 The Adjustment Michigan, who sees patients with late- onset Tay-Sachs disease once or twice a year. ❑ What Is Late-Onset Tay-Sachs Disease? Late-onset Tay-Sachs (LOTS) dis- ease is a rare form of Tay-Sachs disease, a lysosomal storage disorder caused by the absence of the enzyme Hexosaminidase A (Hex-A). LOTS was first recog- nized in the 1970s during a rou- tine Tay-Sachs carrier screening. Investigators have discovered very low levels of Hex-A in older children and adults whose symp- toms include lack of coordina- tion, slurred speech and muscle weakness. Approximately one-third of affected individuals suffer from depression and other mental illnesses. Symptoms typically present in adolescence, but many individu- als are misdiagnosed with other muscular or neurological diseases. Accurate diagnosis of LOTS is made through enzyme assay and DNA analysis. As a recently recognized rare form along the Tay-Sachs con- tinuum, the course of LOTS is not completely known. Life expec- tancy is probably not reduced although it is a progressive disease. The disease is transmitted through heredity. Both parents have to carry the Tay-Sachs gene for there to be a possibility of transmission to their child. The genetic mutations that cause both forms of Tay-Sachs are most common in people of Ashkenazi Jewish heritage. But the mutations also appear in cer- tain French-Canadian communi- ties of Quebec, the Old Order Amish community in Pennsylva- nia and the Cajun population of Louisiana. Since there is no cure, it's important for at-risk couples to undergo genetic testing to de- termine if they are carriers. ❑ Information compiled from Web sites of the Mazor Guide to Jew- ish Genetic Diseases, National Tay-Sachs & Allied Diseases As- sociation, and the Genetics Home Reference Guide of the National Institutes of Health.