Eric Cochiolo

Eric was born on January 25, 1999. He lives in California with his parents, Nancy and William. He has an older sister named Rebecca and an older brother named Michael. He passed away when he was 3 years 2 and a half months old, on April 10, 2002. You can find out more about this sweet little boy, Eric, at his web site www.hometown.aol.com/cncochiolo/myhomepage/baby.html

 Hannah DeGroot

Hannah was born on November 23, 1999. She lived in Columbus, Indiana, with her parents, Gina and Jason. She has an older sister named Allissa and a younger brother named Joshua. Hannah was diagnosed with Sandhoff when she was nine months. We met Hannah and her parents at the 2001 NTSAD Family Conference down in Tampa, Florida. She passed away on November 14, 2001. Find out more about Hannah and check out her fantastic photos at her web site www.geocities.com/gide9899/Hannah.html.

 Gemma Danielle Lea

Gemma was born on December 5, 1998. She lived with her parents, Nick and Clare, in England (Culcheth, Warrington, Cheshire). Gemma was diagnosed when she was 10 months old. She passed away in her mother's arms on May 8, 2001. Take a peek at the beautiful web site of this precious "Gem" at: http://www.gemmalea.co.uk/

  Holden Reamy

Holden was born in November 5, 1999. He lived in Oklahoma with his mom, Kelli, and his older brother, Hayden. We met Holden, Hayden and Kelli at the 2001 Family Conference down in Tampa, Florida. Holden passed away on February 22, 2003.

 Conner L. Watkins

Conner was born on March 30, 2000. He lives in Georgia with his parents, Gina and Rodey. He has a younger sister named Cailee. He has Late Onset Sandhoff Disease. His favorite things are the bath and loud music. He also likes the wind in his hair when the sunroof is down - that always gets a laugh from him. You can find out more about this special little boy at his new web site: http://scnwd.com/CWatkins/Conner.html

 Joey and Clayton

Joey was born on June 30, 2000.  He lives in Omaha, Nebraska, with his parents, Joseph and Melissa. He has a younger brother named Clayton, who was also diagnosed with Sandhoff.  Clayton was born on July 9, 2002. Joey passed away on July 3, 2003. Joey's favorite things include being held and just being talked to. Joey had the most adorable smile and laugh as a baby, He is such a sweetie! Clayton enjoys being held and played with. He loves to sit up on his own and to babble and giggle.  He is just a happy little guy! Visit their web site at: http://www.mulliganangels.com

Sandhoff disease is closely related to Tay-Sachs disease. It is a lysosomal storage disorder, but is much less common than Tay-Sachs disease.The fatty material (GM2 ganglioside) which accumulates in the child's brain cells is the same in Sandhoff and Tay-Sachs diseases.

What makes Sandhoff distinct from Tay-Sachs?

The enzyme deficiency in Sandhoff Disease arises from mutations in a different gene on a different chromosome (i.e. the b-subunit gene on chromosome 5 instead of the a-subunit gene on chromosome 13 for Tay-Sachs). As a result, children with Sandhoff Disease have almost no hexosaminidase at all, unlike children with Tay-Sachs who still have Hex-B activity but not Hex-A. Children affected by Sandhoff's disease may show more marked involvement of the bones and abdominal organs.

Carrier Population

Unlike Tay-Sachs disease, Sandhoff disease occurs more commonly in the non-Jewish population.

Links for more information:

National Tay-Sachs and Allied Diseases Association (NTSAD) NTSAD has current information available on where to obtain the tests that are critical to the diagnosis and management of Sandhoff Disease within families. 2001 Beacon Street, Suite 204 Boston, Massachusetts 02135 USA Tel. (617) 277-4463 or 1-800-90-NTSAD