Recently, a very special gathering was held here: The Tay-Sachs Foundation marked its 60th anniversary with a national conference at the Marriott Quorum.
So: what is Tay-Sachs? It’s a disease caused by the absence of Hexosaminidase-A (Hex-A), a vital bodily enzyme. If you have it, you’re fine. But without it, cells — particularly nerve cells of the brain and spinal cord — suffer from the pileup of a fatty substance called GM2. Most often, sooner rather than later, this will be fatal.
The condition is rare, and inherited; it occurs when a child receives a mutated gene from both parents. Death most often comes within the early months or years of life. But sometimes, those with it live to adulthood. When onset comes later, the disease’s progress is slower. But there’s a sad trade-off: This extended life is filled with physical problems including tremors, muscle cramps and slurred speech.
Tay-Sachs has been on the list of “Jewish diseases” for a long time, and potential parents have been advised to seek testing; they are then advised that if both carry the gene, they should carefully consider the odds of occurrence as they anticipate pregnancy. But I was surprised to learn that, at the recent conference, many attendees — actually most — were not Jewish. It’s now known that the disease is by no means “exclusive.” The more learning there is about genetics, the better the decisions that can be made. But for a couple with the genes, whether to have or not to have children will be a choice with potential heartbreak.
These days, you can read about Tay-Sachs in an easily accessible variety of online sources; after you do, you may want to join the Foundation in its fight against this genetic destroyer of normal life. But I think your heart will be better touched if you first read a part of a little book written by Sherri Manning, a Tay-Sachs mother who is currently president of the Foundation. She tells her story in the imagined, rhyming words of Dylan James, the son that she and husband Brian lost at age 4. She calls it If Only I Could Talk.
Manning “hears” Dylan saying this at birth: “…an angel whispered to me, ‘It’ll be OK. That’s what mommies and daddies are for…They will care for you like no other, and love you forever more. But you see, angel Dylan, you are one of us. You are very unique … God has special plans for you. Your mommy and daddy will receive the news before you turn two…that you’ll never be able to run or play hide and seek…’ ”
Here’s what might once have been called the exception that proves the rule: Neither Sherri Manning nor her husband is Jewish. However, among the Jewish attendees were two local women, good friends who are both very active in the organization’s efforts, because one lost a son when he was only 19 months old. Now — some 50 years later — she rose to speak extemporaneously before the entire audience. Many thanked her personally afterward, because they have the very personal, very sad understanding of all she went through so long ago, and had now shared so freely with them.
For those of you who want to learn more about this tragic killer and join in the fight against it, the best source is this website: www.NTSAD.org (yes — that means National Tay-Sachs and Allied Diseases! The name alone points out how very much there is yet to learn…)
Sherri Manning concludes Dylan’s story with a rhymed statement about what his too-short life has taught others: “…love, laughter, innocence, hope and compassion…” Its final words are “And so my mission, Dear Lord, is accomplished…without ever having to speak a single word.” This book is not yet available in bound form, but Dylan’s mother has secured her copyright for publication.
I hope all of us will be able to read all of it soon.