Six months before he died, David Rimoin met with a dozen Iranian Jews to speak to us about our disease. A legend in the field of genetics, Rimoin was one half of the team that, in the 1970s, helped nearly eradicate Tay Sachs among the Ashkenazi Jewish population. Forty years and many a pioneering effort later, he had embarked on a similar effort for the Mizrahi Jews of Iran.
Our Iranian Jewish disease is called HIBM. It's 3,000 years old, but was only diagnosed a decade and a half ago. It's genetic, progressive, and debilitating; it leaves the mind intact but eats away at every muscle in the body until the patient is permanently and completely immobilized. There's no treatment or cure.
At the meeting, Rimoin explained that HIMB is caused by a single recessive gene. Isolated cases exist in the general population but among Iranian Jews, one out of 20 individuals is a carrier for the gene. If two carriers have a child together, he or she has a 25 percent chance of having HIBM. This translates into roughly one in 1000 instances of illness.
I thought then of all the stories I had heard in my childhood, about things that seemed to happen only to Iranian Jews, that had been normal and true on the narrow streets and in the crowded ghettoes of Iran, but seemed bizarre and fantastical in Los Angeles and New York, Tel Aviv and Jerusalem.
That Iranian Jews should have their own "special" diseases is not as strange as it may seem: Ours is the oldest community in the diaspora, offspring of the slaves who, in the sixth century BC, were brought into the Babylonian exile. Since then, as Babylon became Persia and Persia became Iran, Jews have maintained a consistent presence in the region. As with our Ashkenazi cousins, with Mediterranean people and Finns, Haitians and Cajuns and every other physically isolated population, our shared genetic background has engendered many fine attributes, and a few unfortunate mutations.
Nor is it especially odd that, in spite of its obvious physical manifestations, HIBM seemed to have gone completely unnoticed in Iran. So had three other, less severe Iranian Jewish illnesses. Statistically, one out of every thousand Iranian Jews should have been affected by one or more condition. But if the sick had been there, they had remained invisible. It's as if every one of these diseases had remained benign or dormant for 3,000 years, only to initiate hostilities the minute Iranian Jews landed on foreign soil. This, in a population where medicine is one of the oldest and most practiced professions, among people whose idea of a closely-guarded secret is something that's shared with two dozen family members on three continents.
The truth, of course, is that both the patients and their families had hidden or denied the existence of a hereditary disease for fear of social stigma. Already a persecuted and maligned minority in Shiite Iran, Jews were reluctant to give fodder to the age-old "inferior race" argument (the same label was attached to Ashkenazi immigrants in the United States) that had been used to oppress and humiliate them. They were afraid, too, of the black mark of having "bad" genes, being viewed as flawed and defective by members of their own communities.
The strange thing is, not much has changed for us in that regard in America.
In the decade that followed the first diagnosis, advances in the science of genetics made it possible to identify the mutations that cause each Iranian Jewish disease. In 2009, Dr. Rimoin and the Medical Genetics Institute at Cedars Sinai launched the first genetic screening program for non-Ashkenazi Jews in the United States. A pilot project in Los Angeles was highly successful. After that, Rimoin anticipated few obstacles. With Tay Sachs, he and his colleague, Michael Kaback had informed, educated, and screened millions in the United States and around the world. This, without the aide of email, smartphones, or social networking sites.
For the Mizrahi project, Rimoin was dealing with populations of approximately 50,000 or more Iranian Jews in Los Angeles and around 15,000 in New York, all concentrated in specific, easy-to-reach areas. (Various Iranian Jewish organizations in these regions believe the populations to be closer to 70,000 and 20,000, respectively.) In Great Neck, where most Iranian Jews live, about half the population are Mashadi Iranians who historically, have married only other Mashadis. They have their own synagogue and community leadership, and while they remain intensely private and determined to maintain their purely Mashadi identity, they are not unaware of the genetic hazards of persistent inbreeding.
In response to this historic breakthrough, Iranian Jews remained impassive, even resistant.
Three years after the end of the pilot project, barely 100 individuals had been tested in Los Angeles, according to Rimoin and his staff. In New York, the numbers were even more disappointing. The Jewish Genetic Disease Consortium, an alliance of nonprofit organizations dedicated to the fight against some 36 Jewish genetic illnesses, found that rabbis in the New York community were reluctant to broach the subject with young people or their families. Patients, afraid of being ostracized, tried to hide or deny their illness. Parents discouraged their children from being tested for fear of damaging their chances at marriage. Families kept their history of HIBM secret, thereby increasing the odds that two carriers would have children together.
Hence, the meeting in Beverly Hills.
David Rimoin was not insensitive to the anxieties and concerns of the people he had set out to help. He was aware that many Iranian Jews still doubt the veracity of the diagnosis or the possibility that, without screening, it would continue to claim victims. Others insist that any effort against HIBM should be directed at finding a cure. A majority warn, perhaps correctly, that a public conversation about the issue will further damage our already-fragile image among our sometimes intolerant neighbors in America.
In some parts of the world, among people with longer histories of hardship and tragedy, truth has many layers -- some visible to the Western eye and others not. Rimoin understood this. What he wished to convey, with as much humility and understanding as I've seen in another person, was that sometimes, only one truth matters.
I believe he was right. Years ago in Iran, I knew a woman who had lost her son in a road accident. These days, I think of that boy when the subject of genetic screening comes up. He was 12 years old, riding a bicycle on a gray and dusty afternoon in Tehran. A car had lost its brakes and was careening down the street toward him, but the boy didn't know that because there were no mirrors on his bike. The driver of the runaway car stuck his head out the window and was yelling at the boy to get out of the way. He had one hand on the horn and the other on the steering wheel, but he couldn't get the kid's attention or get the wheel to move. Then the front bumper of the car picked up the bicycle and threw the boy up a few feet and down on the windshield where his head split open with a loud cracking sound that he couldn't hear, couldn't have heard even if he had been a witness and not the victim of the accident because he was deaf but no one knew it, his family had kept this secret and let him ride the bike with no mirrors.
It's not easy, having to choose between prudence and shame, watchfulness and derision. Only a persecuted minority forever at risk of being judged and condemned for traits that -- although specific to them -- have a counterpart in the general population can understand the torment of a mother who denies her son's deafness. In the end, however, I believe Dr. Rimoin was right. Now that the Mizrahi project has lost its hero, perhaps the children he saved from Tay Sachs 40 years ago and the parents whose children he was trying to save from HIBM when he died can join hands, look again at the gift he left us and this time, see the only truth that matters.
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