Squeals of girlish laughter ring out from the hallway of Ann and Neil Wolff’s Silicon Valley home. Six years ago, it was a sound that the couple despaired of ever hearing.
Both carriers of Tay-Sachs, the couple suffered one pregnancy heartbreak after another until the arrival of two healthy daughters. Rachel was born 5-1/2 years ago, followed just 14 months later by her younger sister, Sarah.
“I feel so blessed,” said Ann Wolff, a 44-year-old social worker whose daughters share her abundant set of corkscrew curls.
Though tinged by loss, the Wolff’s story, which was detailed by the Jewish Bulletin in 1998 shortly after Rachel’s birth, is testimony to three decades of success in screening Ashkenazi Jews for Tay-Sachs and to the medical options available to carrier couples.
“Twenty five percent looks like a small number until it whacks you against the head,” said Neil Wolff, a 45-year-old attorney.
The couple had two miscarriages unrelated to Tay-Sachs, and terminated two other pregnancies when prenatal tests revealed that both fetuses were Tay-Sachs affected.
“At that point, I was ready to throw in the towel,” Ann Wolff said. “I didn’t want to have the pain of loss again.”
Rachel was born with the help of a technique called pre-implantation genetic diagnosis, in which embryos are developed outside a mother’s body and screened before being implanted in the uterus.
Encouraged by that pregnancy, little sister Sarah was conceived the “old-fashioned” way, and a prenatal test determined she, too, was healthy, said Ann Wolff.
Rachel is not even a carrier of the Tay-Sachs gene. The couple will test Sarah later on to determine her carrier status. Though they are normal, Tay-Sachs carriers can pass on the gene for the disease to their offspring. A Tay-Sachs child, however, can only be produced by two carriers.
Despite better public awareness of Tay-Sachs, Ann Wolff thinks that still more education is needed about the disease and the alternatives available for couples who are carriers.
“We just happened to stumble on the technology” of pre-implantation genetic diagnosis, she said. At the Virginia-based Jones Institute for Reproductive Medicine, the Wolffs are just one of five couples who have tried the procedure because of Tay-Sachs. The cost is about $13,000.
“It just gives you an option,” said Wolff. “I think what’s hardest for people psychologically is when you feel you’re trapped.”