Little Jacob loses Tay-Sachs battle

Thursday, Nov. 11, 2004 | 8:57 a.m.

Even in death, little Jacques Jacob DeFevre was inspiring.

Cradled in the arms of his mother, Summer DeFevre, with his dad, Jimmy, and grandmother Michelle Chapman by their side, the 2-year-old child courageously lost his lifelong battle with Tay-Sachs disease Monday at his North Las Vegas home, they said.

"Jacob weighed only about 10 pounds at the end because he could not eat the last two weeks," Chapman of Henderson said. "He was all bones, and his once-chubby face was sunk in. It was such a horrible way for a baby to die.

"But watching Jacob struggle like that has further inspired me to do what I have to do to prevent another family from ever having to go through this."

Services for Jacob were scheduled for 2 p.m. today at Palm Mortuary, 7600 S. Eastern Ave. Burial was to follow in Palm Valley View Memorial Park.

Earlier this year, Jacob inspired his parents and grandmother to start Jacob's Light Tay-Sachs Disease of Nevada, a nonprofit organization to raise awareness of the extremely rare illness.

Tay-Sachs is a genetic neurological disease that destroys the central nervous system and is always fatal.

One in every 250 people is a carrier of the disease. Carriers cannot get Tay-Sachs. However, when two carriers have children there is a 25 percent chance each child will be born with Tay-Sachs and a 50 percent chance each child will be born a carrier, medical experts say.

The disease is characterized by an inability to swallow, an inability to have a natural bowel movement, seizures, retardation, breathing difficulties, blindness -- all of which afflicted Jacob -- and paralysis.

"I want to scare people into getting blood tests before they consider having children because being scared is nothing compared to later being forced to watch a beautiful child suffer terribly and wither away," Chapman said.

Chapman said there are alternatives for would-be parents who are carriers. They include a "sperm wash" that removes Tay-Sachs. Another option is for parents who have been found to be carriers to get pregnant, test the fetus in the womb to determine if it has Tay-Sachs and to abort the baby if it does, she said.

Chapman said that since being featured in an Aug. 4 Sun story about Tay-Sachs, the going has been slow for raising awareness of the disease.

"We hit a wall at the elections because the doctors I have been working with to raise awareness were busy working on passing Question 3 (the Keep Doctors in Nevada initiative)," Chapman said.

"But, with Jacob's passing I can now channel my energies from care-giver to full-time efforts with Jacob's Light so we can accomplish our goals of working with the medical community to get a testing program going."

Jacob was born Sept. 5, 2002, and he started showing symptoms of Tay-Sachs when he was 6 months old. He progressively deteriorated.

"By doing what I can to get the word out about Tay-Sachs I feel I am accomplishing something," Jimmy DeFevre said in the Aug. 4 Sun story. "My son will die but his spirit will live on through this foundation and the work we will do."

Jacob's other survivors include grandparents Jacques DeFevre of Las Vegas, Don Chapman of Las Vegas, Danny and Dana Rivero of San Diego and Lon Cannon of Florida.

The family said donations can be made in Jacob DeFevre's memory to Jacob's Light Tay-Sachs Disease of Nevada.