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Saudi Arabia Awakes to the Perils of Inbreeding

May 1, 2003
Saudi Arabia Awakes to the Perils of Inbreeding
By SARAH KERSHAW


RIYADH, Saudi Arabia, April 24 - When she was 17, marrying age for a Saudi
girl, Salha al-Hefthi was presented with a husband.
She was lucky, her parents told her when they planned the wedding, that
she was to marry such a good man, a man from her own tribe, a man who
would care for their children and make a good living. He was the son of
her father's brother - her first cousin - and everyone, including the
bride, agreed that "a first cousin was a first choice," she said.
The couple had two healthy boys, now 22 and 20, but their third child, a
girl, was born with spinal muscular atrophy, a crippling and usually fatal
disease that was carried in the genes of both parents. Their fourth, sixth
and seventh children were also born with the disorder.
Spinal muscular atrophy and the gene that causes it, along with several
other serious genetic disorders, are common in Saudi Arabia, where women
have an average of six children and where in some regions more than half
of the marriages are between close relatives.
Across the Arab world today an average of 45 percent of married couples
are related, according to Dr. Nadia Sakati, a pediatrician and senior
consultant for the genetics research center at King Faisal Specialist
Hospital in Riyadh.
In some parts of Saudi Arabia, particularly in the south, where Mrs.
Hefthi was raised, the rate of marriage among blood relatives ranges from
55 to 70 percent, among the highest rates in the world, according to the
Saudi government.
Widespread inbreeding in Saudi Arabia has produced several genetic
disorders, Saudi public health officials said, including the blood
diseases of thalassemia, a potentially fatal hemoglobin deficiency, and
sickle cell anemia. Spinal muscular atrophy and diabetes are also common,
especially in the regions with the longest traditions of marriage between
relatives. Dr. Sakati said she had also found links between inbreeding and
deafness and muteness.
Saudi health authorities, well aware of the enormous social and economic
costs of marriage between family members, have quietly debated what to do
for decades, since before Mrs. Hefthi was married 23 years ago. Now, for
the first time, the government, after starting a nationwide educational
campaign to inform related couples who intend to marry of the risk of
genetic disease, is planning to require mandatory blood tests before
marriage and premarital counseling.
Mrs. Hefthi, for one, wishes she had been given the opportunity to test
for genetic risks.
"If I knew, I would have said no to that marriage," Mrs. Hefthi, an
elementary school teacher, said the other night, sitting in her living
room with three of her sons.
"Why? It's very painful. Why? If you know something is wrong, would you do
it?"
Mrs. Hefthi did not know it when her daughter was born, but Ashjan, now
18, would never walk. Her childhood would be filled with terrible colds,
sore throats, assorted other illnesses and an obsessive longing to walk
and run like her older brothers.
"Why can't I walk," she would shout to her mother when she was 6.
"It is God's will," her mother would say. "In paradise you will walk."
"In paradise will I have a magic carpet?" she would ask constantly. "In
paradise will I have a horse with wings?"
Ashjan would never be able to comb her hair or dress or clean herself. Her
body would grow only in tiny spurts, her spine curving into the shape of a
half-moon. Once she reached adolescence, she would shrivel year by year,
and she would most likely die by the time she turned 20.
Health officials and genetic researchers here say there is no way to stop
inbreeding in this deeply conservative Muslim society, where marrying
within the family is a tradition that goes back hundreds of years.
Today, when most unions are still arranged by parents, marrying into
wealth and influence often means marrying a relative. Social lives are so
restricted that it is virtually impossible for men and women to meet one
another outside the umbrella of an extended family. Courtships without
parental supervision are rare.
Among more educated Saudis, marrying relatives has become less common and
younger generations have begun to pull away from the practice. But for the
vast majority, the tradition is still deeply embedded in Saudi culture.
Statistics on the prevalence of genetically based diseases and the extent
to which they are a direct result of marriage between close relatives -
second cousins or closer - are scarce or unreliable because many Saudi
parents raise their disabled children in obscurity, ashamed to seek
services.
That has begun to change as more programs intended to educate disabled
children open in Saudi Arabia, where there were almost none until a decade
ago. Genetic research is emerging here and several projects have recently
begun in an effort to do***ent the connection between inbreeding and
disease and to quantify the prevalence of the diseases.
"Saudi Arabia is a living genetics laboratory," said the executive
director of the Prince Salman Center for Disability Research, Dr. Stephen
R. Schroeder, an American geneticist who has been doing research in Saudi
Arabia for the last year. "Here you can look at 10 families to study
genetic disorders, where you would need 10,000 families to study disorders
in the United States."
One of the oldest and best known educational programs for disabled
children in Saudi Arabia is the Disabled Children's Association in Riyadh,
which opened in 1986. There, 200 children from infancy to age 12 suffering
from a variety of diseases and disorders attend day care programs and
classes. At the school, the director, Sahar F. al-Hashani, pointed out at
least one or two students in each of six classrooms whose parents were
related.
Not all marriages between close relatives produce children with genetic
disorders. In fact, most do not. But testing could identify couples who
test positive for serious diseases. Under a fatwa issued by the World
Islamic League in 1990, Islam permits abortions up to 120 days after
conception if an unborn child tests positive for a serious disorder.
In the case of spinal muscular atrophy, if both parents are carriers of
the gene, the couple has a 25 percent chance of having a child with the
disease - or one in four children. The percentage regrettably turned out
to be much higher for Mrs. Hefthi and her husband, with four out of their
seven children afflicted.
Mrs. Hefthi said she would not allow any of her three healthy boys to
marry a relative. In a society that places such a premium on having
children, she said, many people would choose to find another mate if they
learned that they were at risk of having severely disabled children and if
their parents supported their decision.
"I suffered," she said. "People, sometimes when they see me they say how
tired I am. They tell me I could put my children in an institution. But I
tell them I am a mother."


Copyright 2003 The New York Times Company