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Norway: Pakistani children at risk of brain disorders

Nahraf

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They claim that cousin marriages are causing diseases and syndrome among Pakistani children. My parents, grand-parents, and great grandparents were all cousins and we did not have any problems. Now all my cousins have married out of the family and none of the spouses are related.

Islam in Europe: Norway: Pakistani children at risk of brain disorders

Norway: Pakistani children at risk of brain disorders


The risk for serious brain diseases among Norwegian-Pakistani children is 7 times higher than for Norwegian children, and even higher (11 times as high) if the parents are cousins.

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[Ed: The article (PDF, NO) says the risk is 7 times higher, but the accompanying chart shows the incidence rate is 3.5 times higher. ]

Doctor Øivind Juris Kanavin and professor Petter Strømme of Ullevål University hospital in Oslo, wrote an article on children with brain diseases which was published by the Norwegian Medical Association Thursday. Both are experts in brain disorders in children.

They've seen that such disorders are often hereditary. Most cases are complex and in-marriage is a major risk factor.

Kanavin made contact with social-anthropologist Hilde Berge, who works at the learning and competence center for children, youth and their families (LMS-barn) and social worker Ragnhild Gardsjord of the rehabilitation department at Ullevål, who founded a group for Norwegian-Pakistani mothers this winter.

All the women have children with serious brain disorders, and they are are concerned about having more children.

Gardsjord says that it turned out there was a great need for such a group. Many had nobody they could speak to or share the challenges with. One young mother told them that if she didn't have this group, she herself would need to go to a psychologist.

In many cases the parents are relatives, often cousins.

"One mother told me: there was a celebration in the family when I gave birth to a son, but the celebration quickly died down when it turned out that he had a brain disorder. It was problematic for the family."

This young mother, who was married her cousin through an arranged marriage says that she had told her mother in advance that there might be a danger of sick children, says Berge.

Berge and Gardsjord says that the second generation of Norwegian-Pakistanis know much more about hereditary diseases than does the parents' generation.

Berge says that arranged marriages are still very common and that some women say their concerns about links between in-marriage and disease are dismissed. Gardsjord says that the women tell of ignorance among some in the Pakistani community. There is also a lot of taboo, shame, prejudice and guilt.

Berge says that there are stories about children who get better after they were sent back to Pakistan, mothers get 'good advice and tips' about different forms of treatment which are 'good for the children' or which will make 'the children healthy'.

The mothers in the group had asked for a meeting with their parents and parents-in-law, where a doctor could talk about hereditary diseases and give genetic counseling.


The doctors Kanavin and Strømme wrote about the issue because though those are rare diseases, it's very serious for those concerned.

The diseases have high mortality, a very demanding of resources, and meanwhile there are some treatment options for some of them. In addition, there was a study in Oslo that they wanted to clarify.

Kanavin says that children with progressive brain diseases gradually loss motor skills, lose the ability to orient in time and space, have impaired attention, lose the ability to calculate and write, and have reduced social skills. He says the progress can be slow or very fast.

Kanavin says there are many different causes, most of them hereditary. In about 20% of the cases, there is no clear diagnosis.

A lot of assistance is needed for such diseases. It's not uncommon to have 15-30 professionals involved with one patient. Kanavin says that it's challenging work in all areas with these patients.

Through his work Kanavin is in regular contact with families who have children with these serious disease and he admits it can be challenging to meet with the group which is overrepresented, the Norwegian-Pakistanis. It is difficult for many of them to understand the biological causes of these conditions, some don't have the terms to deal with it. Language is also a big problem, and often information is communicated via interpreter.

He says they're rather direct when they speak to parents about the causes and that there's additional risk when the parents are relatives. The diseases occur when the child inherits a defective gene from both the mother and father. They tell the parents that there's a 25% chance that the next child will also get the same thing.

Source: Aftenposten (Norwegian)
 
Chowk: Education: Phuppi ki Beti, Mamoon ka Beta

Phuppi ki Beti, Mamoon ka Beta
Anita Zaidi March 6, 1998

Tags: cousin-marriage , reproduction , medicine
Cousin marriages are very popular in Pakistan. Several studies from
Punjab estimate the prevalence of consanguineous marriages (defined as
marriages between blood relatives) at about 50%. There are distinct cultural
advantages in marrying a close relative. In
a culture where most marriages
are arranged, and there is little chance to interact with members of the
opposite sex, for both partners, there is the comfort of familiarity. From
the boy's (literal translation of larka) parents, there is the hope that
since the daughter-in-law is a bhanji/bhatijee, there will not be much
resentment or conflict with them. For the girl's family, there is the reassurance
that their child is unlikely to be mistreated - an extremely important
consideration in a country where abuse of women is common. In some instances,
there are economic considerations - property remains within the family.

Cousin marriages, therefore, make sense in many ways. However, from
a population genetic standpoint, they may not be a prudent choice. Sexual
reproduction was designed for a reason - to produce genetic mixing and
diversity - as opposed to asexual reproduction practiced by bacteria and
amebae who simply divide themselves to produce identical daughter clones.
Why is genetic mixing important in higher organisms? A brief and simple
primer on human genetics follows.

For each genetic locus (excluding genes on X and Y chromosomes), we
inherit two copies (called alleles), one from each of our parents. If the
genes are the same, the offspring is homozygous for that allele, if they
are different - he/she is heterozygous. In most cases, as long as one of
the two genes is functional, normal human life is possible, and although
an individual may be a carrier of one defective gene, no overt disease
results, because the normal gene compensates to some extent. However, if
both genes are defective, disease does result. Because inbreeding increases
the frequency of homozygous genes, the offspring of consanguineous marriages
have an increased risk over that of the general population of having many
different types of diseases. The magnitude of risk depends on the frequency
of defective genes in the population and the degree of relationship between
the parents (an example will follow later).

The degree of inbreeding for the offspring of a particular couple can
be quantified by calculating the Coefficient of Inbreeding (F). The coefficient
measures the probability (genetics is a game of statistics) that a child
receives two alleles at a given locus that were both from the same ancestor
and are, thus, identical by descent. Some of these numbers are given below:

In Punjab, the only area from which data is available, the average Coefficient
of Inbreeding is 0.029 (Shami et al. Journal of Biosocial Science, Jan
1994). What does this mean in real terms? Suppose that there is a defective
gene in the population that exists in a frequency of 1/200. If random mating
takes place (assuming Hardy-Weinberg Principles), the offspring's chance
of getting two defective genes are 1/40,000 (1/200X1/200). If however,
the parents are first cousins, the risk increases to 1/2977, a thirteen
fold increase (this risk is calculated from a modification of the Hardy-Weinberg
equation (Bronya Keats, Population Genetics, Principles and Practice of
Medical Genetics, calculation available on request). For the individual,
this may seem like a tolerable risk, given all the benefits of a cousin
marriage. However, for society as a whole, it means thousands of children
with a genetic disorder.

Are these just theoretical risks with no basis in fact? Quite the contrary.
Much data now exists to prove that the risks are real. In a study from
Norway (American J of Epidemiology, Mar '97) the investigators compared
approximately 7500 children born to two Pakistani parents (30% of whom
were consanguineous) to children born to Norwegian parents and found the
risk of birth defects to be increased by 39% in the Pakistani children
overall. They attributed 28% of these birth defects to consanguinity -
no increase in risk in children of non-consanguineous Pakistani parents
relative to the Norwegian population was found.

In another study done as part of the 1990/1991 Pakistan Demographic
and Health Survey, data were collected from approximately 26,000 births,
and the effects of consanguinity on mortality (while controlling for other
factors) measured (Annals of Human Genetics, Mar '97). Inbreeding at the
level of the first cousin exerted a significant adverse effect on survival
into childhood. A study from the Aga Khan University in Karachi, showed
a 40% increase in the incidence of low-birth weight babies in offspring
of related parents compared to babies born to non-related parents. The
Annals of Human Biology concluded that the widely favored practice among
Pakistanis, at home and abroad, of marriage between close relatives may
be a contributor factor in their comparatively unfavorable health profile.


Researchers in Europe and America have realized what a gold mine the
Pakistani population is - for identifying the basis of many genetic diseases.
Thalessemia, a disorder of hemoglobin production and anemia is the most
common one (approximately 1 in 25 individuals in Pakistan carries this
gene), but just a cursory search of recent literature identified a laundry
list of exotic sounding diseases (Combined Factor V-Factor VIII deficiency,
familial aplasia/hypoplasia of pelvis, non-syndromic deafness, retinitis
pigmentosa, muscular dystrophies and so on).

Since 50% or more of our genetic material is expressed in the brain,
it is logical to presume that consanguinity of parents may have subtle
neuro-developmental effects in their children. However, this has never
been studied in practice. Some neurobiologists have speculated that extensive
consanguinity among Muslim populations has led to the current decline of
the Muslim Ummah because our genetic pool is no longer healthy - even if
this has not resulted in obvious disease, it has affected us in subtle
ways. It would be interesting to use sophisticated tests that try to measure
if cognitive differences exist in the offspring of related versus unrelated
parents, in large populations, while controlling for confounding variables.

To summarize, the many advantages of cousin marriages are offset by
the potential biologic risk to the offspring. Cousin marriages should be
discouraged, especially in families where a lot of inbreeding has already
taken place in previous generations. Here again, the bottom line is - diversity
is good, homogeneity is bad!
 
BioNews - Leading bioethicist to reignite debate on cousin marriages

Leading bioethicist to reignite debate on cousin marriages

22 March 2010

By Dr Vivienne Raper

Appeared in BioNews 550

A leading bioethicist is to reignite the debate on the genetic risks of marriage between first cousins. Baroness Ruth Deech, a reproductive law professor and crossbench peer, is to call for a 'vigorous' public campaign to deter the practice, according to a report in The Times. Baroness Deech will be giving a free public lecture titled 'Cousin Marriage' at the Museum of London on Tuesday 22 March during which, The Times reports, she will say the children of first cousins have a raised risk of inherited disabilities and disease.

'The local estimate was that 75 per cent of Bradford disabled children had cousin parents and the rate of cousin marriage in the UK Pakistani community is increasing', Baroness Deech will say. The Times reports that fifty-five per cent of British Pakistanis are married to first cousins and, in Bradford, the figure rises to 75 per cent. One-third of children with recessive disorders are British Pakistanis, but they only account for three per cent of UK births.

Baroness Deech is due to call for married first cousins to use IVF so their embryos can be tested for genetic defects, according to The Times. Other recommendations will include a campaign to deter cousin marriage. 'There is no reason, one could argue, why there should not be a campaign to highlight the risks and the preventive measures, every bit as vigorous as those centering on smoking, obesity and Aids', she will say.

This could include genetics education, such as what it means to carry a mutant gene. According to The Times, Baroness Deech will also suggest a genetic screening programme similar to that used in the Orthodox Jewish community to tackle the recessive genetic disorder Tay-Sachs disease. 'Where marriages are arranged, it is possible to test for carrier status and record the results, without stigmatising individuals', she will say. The Times explains that, when a match is proposed in the Orthodox Jewish community, a registry is checked to ensure it is not between two carriers of Tay-Sachs. 'Some variant of this could be possible in cities such as Bradford, with a high density of immigrant population', Baroness Deech will say.

In an associated Times Analysis piece, legal editor Frances Gibb discusses famous people who have married their first cousins, including Charles Darwin, Albert Einstein and Queen Victoria. She quotes Baroness Deech as saying first cousin marriages will lead to: 'twice as many sick children (four per cent) as in others who are not related'.
 
Better safe than sorry.

Stop marrying cousins.

Do it for the children!

One-third of children with recessive disorders are British Pakistanis, but they only account for three per cent of UK births.

Deny it.
 

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