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June 2, 2004

One in twenty (1 of 2)

From: Ha'aretz, Israel - Jun 2, 2004

The astonishing rate of deafness among the Al-Sayed tribe has its historical reasons, but is the State of Israel doing enough for them?

By Kobi Ben-Simhon

The bus sometimes arrives late. Standing at the side of an unpaved dirt road without a sidewalk, their schoolbags on their backs, the children keep themselves busy with conversations carried out with quick hand movements, touching one another in order to grab attention. Occasionally one of them utters an unclear sound. One girl repeatedly gathers up her hair and ties it with a red rubber band. Next to them pass the ordinary children, who are continuing on to the village school. One group walks, the other waits for the special bus to pick them up.

Nobody here gets upset by the sad sight. Every morning, 60 deaf-mute children gather at several points in the village of the Al-Sayed tribe in the northern Negev. It's an ordinary morning, like all the others. More and more good-looking children, a seemingly endless number, continue to stream to the pick-up site. Everyone in the area is familiar with the special beauty of the tribe's children. Abed al-Aziz al-Sayed, a heavy machinery operator, is proud of his beautiful children. This morning he is sending three deaf youngsters to school: Hussan, Mohammed and little Shuruk. The blue-eyed father, who is also deaf, bids them farewell; at home Abdullah, a hearing-impaired infant, is still asleep. Al-Sayed is always smiling beneath his thick mustache. In the village they say that God took his hearing from him and gave him joy and eyes filled with the sea. The bus cuts across the village. The waiting children board quickly: Some of them are going to the special school in Be'er Sheva, the others to a regular school in Tel Sheva.

Fears before the wedding

In the Al-Sayed Bedouin tribe, which numbers about 3,000 people, there are over 150 people who are congenitally deaf. That rate is 50 times the overall world average (5 percent as compared to 0.1 percent). Most of the members of the tribe live in an unrecognized village south of Hura.

Toward evening, the landscape is soothing to the eyes. On a drive from Be'er Sheva in the direction of Arad, dozens of flocks of sheep wander on the yellow plains accompanied by barefoot shepherds. Opposite the high-tech center in Omer, which is designed with blue mirrors, one flock lingers at the side of the road, as though deliberating whether to cross to the other side. On the radio, an announcer with a deep voice invites the listeners for a medical checkup at a private institute in Ramat Aviv. The wind picks up as sunset approaches.

Salman al-Sayed lives among exhausted olive trees. There are no electricity lines and water pipes here. There isn't a single paved road, but Israeli flags wave above several houses in the village. Salman has a dozen children, three of them deaf: two daughters aged 18 and 20, and a 10-year-old son.

"I had fears before the wedding," he says. "We knew that there's a problem in our tribe, and that there was a chance that we would have deaf children, but the truth is that 20 years ago there was little awareness. I didn't take the possibility seriously, at the time everything was from God." He strokes his thin beard with one hand, and after a short silence continues. "I had to deal with it. I expected everything to be okay, but three deaf children were born. What can you do? Throw them away? I had to take care of them, to give them whatever I can."

It's not easy. Even now the younger son doesn't know sign language. The daily routine has given rise to a rudimentary, private sign language used by members of the family. That's how they manage among themselves; that's how most of the village communicates with the deaf.

"There's no question that it's frustrating," says Salman al-Sayed, who makes a living as a security guard. "I have never managed to have a real conversation with my young son, and apparently won't be able to do so; we have only bits and pieces. It hurts. Once I saw in an Egyptian film how they teach deaf children to speak - they showed how a teacher sits with a deaf child and teaches him to speak. It's possible, the tools exist, especially in an advanced country like ours, but because they didn't help us to take care of our children, in addition to being deaf they're also mute. They blurt out sounds. My son doesn't even know sign language. Instead of setting up a special class in the village, they send them to schools that don't have enough resources. I think if they would just save the cost of the buses every morning, they could organize a class for our children that would help them progress."

His deaf daughter Ismain, 20, thanks God that she studied in a school outside the Bedouin "diaspora." She's satisfied, she doesn't complain about her situation, not even for a moment or implicitly. Her face is serious, concentrated; occasionally she smiles shyly, waits for her father to translate her words, and then continues.

"I, as opposed to my little brother, learned how to read and write Hebrew in school," she explains, with elaborate hand movements. "Unlike my brother," her father translates her gestures, "I know sign language. I meet with the deaf girls in the village, and it's easy for us to talk among ourselves. I talk to other girls in the family by writing; we sit next to one another and pass paper and a pen back and forth."

She continues to "talk," cutting the air with her hands, and her father's voice hurries to keep up: "In terms of family, we fit in very well and find the ways to express our opinion. One of the things that's important to me today is my marriage. I won't have any real problem finding a groom, but there is little awareness of the issue of deafness. I will insist on finding a suitable groom, so that we won't have deaf children."

Rejecting the revolution

A unique project of genetic counseling for the Bedouin community, which began to operate at Ben-Gurion University (BGU) of the Negev 10 years ago, yielded the tragic data about the Al-Sayed tribe. In a survey that was conducted, it turned out that 27 percent of the marriages in the tribe are between cousins, and 65 percent of the married couples are related to some degree. According to estimates, about one-quarter of the members carry the genetic mutation that causes deafness.

Prof. Rivka Carmi, the dean of BGU's faculty of health sciences, identified the unusual phenomenon and began to implement the counseling project; similar endeavors are being carried out in various countries with groups that suffer from genetic problems. The idea was fairly simple, and together with Dr. Aviad Raz of the department of behavioral sciences and Prof. Ilana Shoham-Vardi of the department of epidemiology and health services evaluation, the team succeeded in identifying 16 genes related to deafness among the members of the Al-Sayed tribe. The findings were passed onto members of the tribe, and genetic mapping was done for all of them. By doing this, the researchers in effect enabled the tribe to break out of its cycle of deafness. The data that were gathered make it possible to check the genetic suitability of couples who are about to get married, and to determine the degree of risk of giving birth to deaf children.

"The deafness in the Al-Sayed tribe is a result of a mutation in a certain gene," explains Carmi. "We don't have an answer as to how the mutation was created, but in a certain generation it happened, and then it began to be passed along from one generation to the next, and among the members of the tribe who married one another. We all have the same genes, but in some of them there is a change, a mutation, in a gene called connexin. Deafness can appear only when both parents are carriers of the mutation, and then there is a 25-percent chance that their child will be deaf. Of course, in a population where there are consanguineous marriages, the chances that two parents who are carriers will meet is much greater, and therefore in the Al-Sayed tribe, more deaf children are born than the average in the general population. This is a huge deviation: In other Bedouin tribes with genetic problems, for example those that cause heart disease, there is a limited number of sick people, a maximum of 15, which is a very large number. The dimensions [of this problem] in the Al-Sayed tribe are entirely different - here it's way beyond the norm."

Genetic counseling has enabled the couples in the tribe to check, both before and after marriage, whether they are at risk of having deaf children. However, the tribe rejected the revolution offered by Prof. Carmi.

Dr. Abed al-Sayed, the main intermediary between the researchers and members of the tribe, still sighs when he recalls the apathy of his relatives. Five years ago he returned to the village from Romania, where he specialized in dentistry. Today he is the head of a successful clinic in neighboring Hura, and is studying for a master's degree in health administration. In the cafeteria at BGU, he orders coffee and sits down.

He has big green eyes and when he talks about his tribe they glitter. He can't understand why the tribe is allowing fate to continue to abuse its children. "I was unable to understand them," he says. "Almost all of them came to my guidance sessions and to the examinations, but very few came to see the results of the examinations. There simply was no response, as though it didn't interest them."

Al-Sayed also married a member of the tribe, but it was clear to both of them that before the wedding, they would undergo genetic counseling. "Many of the members of the tribe, mainly the older people, believe that there is no connection between the deafness in the tribe and genetics," he explains. "They say it's from God. According to one belief, every woman who looked at a deaf child while she was pregnant gave birth to a deaf child."

Al-Sayed offers an almost unbelievable explanation for the fact that members of the tribe aren't fighting against the genetic defect from which they suffer. "They simply don't see deafness as an illness," he says. "We've been experiencing deafness for hundreds of years; today in the village they look upon a deaf person as an ordinary person - he simply doesn't hear. A deaf person isn't considered ill. Because there are so many deaf people, they aren't exceptional any more; everyone has one or two deaf children in the family. The hardship becomes easier, nobody is alone. In every third house there is someone deaf. There are deaf elderly people, there are deaf parents and there are deaf children.

"I see it as a sad story. A deaf child is a handicapped child. The members of the tribe continue to marry among themselves out of a belief that it won't happen to them. I can't understand it; if I have a problem, I try to run away from it, or to solve it. They have all kinds of internal considerations: A genetic test before marriage creates suspicion, and not every couple can begin its relationship with a genetic examination. What happens in the end is that they do a `test' of their own: If they see a family with no deaf people, they conclude that there's no problem, but they're mistaken. The gene can definitely exist, but not be apparent."

Second-class fellahin

Perhaps the greatest problem of the Al-Sayed tribe is their social isolation, which was forced upon them. According to oral histories, the head of the tribe came from Egypt with his wife 150 years ago. The family settled among the Bedouin tribes in the Be'er Sheva region, and subsisted on agriculture and raising animals. When their children grew up, they encountered an unexpected problem: The other Bedouin tribes, who had formed excellent commercial and social ties with them, refused to marry off their daughters to members of the Al-Sayed tribe, "the foreign fellahin" (peasant farmers). Only through tremendous efforts did the head of the Al-Sayed tribe succeed in marrying off his sons to women from the area and from Gaza, but their social status remained very low, because they aren't considered "original" Bedouin. The second generation, therefore, began to marry cousins.

The attempts to forge ties of marriage with other tribes failed repeatedly, and for lack of choice, the Al-Sayeds continued to marry among themselves, for five generations. Since there were more women than men, and according to Bedouin tradition a woman cannot remain single, many men were required to marry more than one woman. One complication led to another, the mutations were passed on and became more widespread from generation to generation, and genetically speaking, the Al-Sayed tribe got itself into a terrible situation. Too many encounters between two carriers of the defective gene led to an unprecedented number of deaf children.

Salah al-Sayed, the father of two deaf daughters, blames the Bedouin tribes for the situation. "I'm angry at them," he hisses with disdain. "They, who thought they were better than us 100 years ago, continue to do so today, and they are considered educated people, ostensibly open-minded. But ethnicity is the name of the game, just as among you, Ashkenazi [Jews of European origin] parents once didn't want their daughter to bring home a Mizrahi [Jews of North African or Middle Eastern origin] groom. It's the same thing, but among you this phenomenon disappeared within a generation or two, and among us it has been going on for 150 years."

Someone has turned on the generator outside, and light floods the house. "The tribes surrounding us are considered original Bedouin, from Saudi Arabia," explains Salah al-Sayed. "We are considered fellahin, second class, from Egypt. When someone from the tribe goes to them to ask for the hand of a groom or a bride, as soon as he leaves that house, he knows that it's a dream that won't come true. They don't want us."

His son places a silver tray with small glasses and a sprig of mint on the table. Al-Sayed pours the tea slowly into the glasses and asks his son to go and play outside. Only after the child leaves does the father begin to talk about his experience.

"I married my first wife because they forced me. I didn't want to," he says. "With her I had the two deaf girls, Rueda and Kokhav." He wanted normal children and began to think about marriage to a second wife, although he had never wanted two wives. He began to build a house for the additional wife even before he knew who she would be.

While he was building, he sketched the family tree of the entire tribe in his head, made calculations and found two women who definitely were not carriers of the mutation that causes deafness. He married one of them, and they had six hearing children. "Today," he says, "we are looking for people of our kind, from the same ethnic class, fellahin from the Negev and from the territories. But from what I can see, the number of such weddings, outside the tribe, is still very small, a drop in the sea of weddings in the tribe."

As a result, he predicts that the problem won't diminish, but will even get worse. "This phenomenon doubles itself - the chances of marriage between two carriers of the gene that causes deafness are only increasing. The people in the tribe continue to avoid the only solution, and continue to marry within the tribe, without making sure that two carriers won't marry. My daughter is about to get married in another year or two, and I will definitely make sure than her husband doesn't carry the gene; there won't be a wedding if she is going to have a bad life."

Bedouin society in the Negev has undergone significant changes, but they haven't affected the nature of intertribal marriages. Other Bedouin tribes continue to veto marriage ties with the Al-Sayed tribe, as they did 100 years ago. And within the tribe, it's enough if the designated couple and their parents undergo genetic testing before agreeing to marriage, and in that way remove the curse of deafness from themselves - but the bonds of tradition are still stronger than they are. Fate, they say in the tribe, determines who will hear and who will not.

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