Sad, cruel results of Usher’s syndrome

Shakespeare calls music “the food of love.” Of course, he qualifies his statement with “if it be.”
But if he’d said something about reading, I’m sure the comment would be fully inclusive, with no “if” about it.
To read is to pan for gold, forever bringing up bright nuggets to hold (in your head, if not in your hand), to turn around and to admire. The gems found in reading are treasures beyond measure, often surfacing in the most unusual places when you least expect them.
Recently, after reading much about it, I shared here some of the golden nuggets I’d unearthed when reading about Tay-Sachs, a sad disease often connected genetically to Jews of Ashkenazic descent. And now I’m reading about another such disease: Usher syndrome.
The book that was my starting source isn’t scientific or medical; it’s a simple memoir called Not Fade Away by Rebecca Alexander, a young Jewish woman who has Usher’s — a genetic cruelty that steals from its sufferers both eyesight and hearing.
This is by no means a great book, but it certainly deserves reading because of its message. One of its reviewers wrote this about Rebecca, the “messenger”: “You never know how strong you are until being strong is the only choice you have.” We Jews already know such strength from the stories of some very different others — Holocaust survivors come quickly to mind. Now, here, we meet a fraternal twin whose brother is not afflicted as she is, learning how she has thus far successfully navigated her life path of ever-increasing loss, and continues to do so because she has no other choice.
Usher’s reveals itself in several ways. In the first, a child’s difficulties may be present and identifiable at birth. But in the third — as in Rebecca’s case — the onset is slow; because of this, and because the syndrome is rare, it is very difficult to diagnose. Her vision problems came first; the hearing loss came later — an additional, unwelcome surprise.
Rebecca’s theory on Usher’s source is straightforward: Jews with origins in areas such as Kiev — where both her father’s and mother’s families had their genesis — routinely experienced their communities’ decimation by forced military service, pogroms and similar random attacks. These resulted in a diminishing of marriageable choices, which in turn led to cousins marrying cousins. This smaller pool of suitable suitors upped the chances of those carrying “bad” genes ultimately having children together. But no one knew about genes — good or bad — in those days …
There is one member of our local Jewish community routinely encouraging us all to support the Foundation Fighting Blindness, the main organization supporting advancement of treatments for this devastating disease, because her own grandson has it, in its worst form. But thanks to modern medical science, he is on his way to an adulthood of education and accomplishment, which is what the author of Not Fade Away has already achieved.
Here’s what Rebecca Alexander tells us about her own life’s purpose: “As early as I can remember, my father instilled in us the importance of giving back to the community and to the world. There is a Hebrew word, tzedakah, that translates as ‘righteousness’ or ‘justice.’ It is generally used synonymously with ‘charity,’ but what it really means is a balancing of the scales — that charity is not an act of pity, or mercy or even necessarily goodness, but of justice. You give back to make the world a better, fairer place — when you have an abundance, you share with those who have less. This was always an idea that resonated very strongly with me …”
… as it should with us. The Foundation Fighting Blindness welcomes our interest in its ongoing efforts. Visit to read all about Usher’s syndrome; then read Not Fade Away. Alexander has subtitled her story, a true “giving back” of her personal triumph over adversity, A Memoir of Senses Lost and Found.

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