Hereditary blood disease is common in Bahrain due to consanguinity marriage. Fighting it is both costly and takes hard efforts. The presentation talks about Thalassemia and Bahrain's experience in combating it.
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•The percentage of related marriages is
high
• in Saudi Arabia, Egypt, and Bahrain it is
50%, 37%, 23%
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•Proportion of genetic abnormalities is 2.5
times more when the father and mother
are related
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Genetic diseases are chronic diseases
with multiple effects; psychological and
immune systems, as well as the economic
aspects of the family.
The economic and social cost of the
disease is high .
Perera, et al 2000
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Occurrence of congenital malformations:
• use of medications during pregnancy.
• Exposure to radiation or chemical substances
during pregnancy.
• Complications of chronic diseases in women,
such as diabetes, hypertension.
• Affection by viral diseases such as Toxo-
Plasmosis
• Various environmental factors.
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The main causes of genetic diseases or
congenital malformations lies in three
factors:
1. Distortions in the genes because of exposure
to maternal diseases and other environmental
factors .
2. congenital deformities transmitted to the fetus
from carrier parents.
3. Reasons related to the process of childbirth
and immediately post-natal period leading to
distortions in the baby.
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Haemoglobinopathies are:
1.a series of hereditary genetic diseases which, if
left untreated, usually prove fatal.
2.the most widespread inherited disorders in the
world, and are also preventable with control
programs
Acemoglu, et al 2008
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The genetic blood diseases, which include
Thalassemia and sickle-cell anemia and
G6PD are the most common genetic disease
in the Mediterranean region
Alalwan World Health Organization
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250 million people (4.5% of the world population)
are with haemoglobinopathy.
Each year about 300,000 infants are born with
major haemoglobinopathies
Angastiniotis,et al 1995
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In Saudi Arabia the following percentages were
reported:
--Thalassaemia (a) between 26-28%
--Sickle-cell anemia ranging between 20-28%
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Beliefs that influence the occurrence of
genetic disease
81% of the households reject abortion
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Premarital Screening
Many nations such as Cyprus, Iran, Palestine,
Saudi Arabia, have adopted the policy of
examination before marriage, which led to a
decline in the proportion of genetic disease.
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Screening programs in the Mediterranean
countries proven to reduce thalassemia
patients.
WHO, 2002
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Iran provides premarital screening for
Thalassemia and permits abortion for
Thalassemia within the first 16 weeks of
pregnancy, in accordance with the 1990
Islamic Fatwa.
Christianson & Modell, 2004
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In Sardinia, thalassemia major was in 1 in
250 births, and declined to 1 in 4000 births.
WHO, 2002
Before 20 years 20% of the population in
Cyprus and Greece was Thalassemia and
sickle-cell anemia carriers. But reached now
to approximately 0.0% in Cyprus.
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Such decrease in the prevalence of
Thalassaemia was attributed to;
- about 5% was due to the separation of the
couple
- 80% aborted the affected fetus during
pregnancy,
- 20% of the couples preferred fewer children,
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In Bahrain
The incidence of hereditary blood diseases in
the neonatal 8 years ago were:
•Sickle-cell anemia 2%
•Carriers of sickle-cell anemia 11%
•Thalassaemia (a) 24%
•Thalassaemia (b) carriers 2%
•G6PD 20%
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In Bahrain
The Ministry of Health introduced PMC service in
December 1992 in an attempt to reduce the
incidence of hereditary blood disorders in Bahrain.
In 1993, national committee for genetic diseases was
established
In 2004, parliament adopted a resolution and a Royal
decree announced making it Compulsory for all
nationals.
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The turnout of examination before marriage
reached to 34% during the 10 years since the
project began in January 1993 and then to 100%
at the present time.
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The premarital check up procedures;
1 - Both spouses have to attend the health
centre.
2 - Both have to sign the application form
examination before of premarital check up.
3 - The Department of Maternal and Child
Welfare will give the couple an appointment
for doctor and nurse.
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First visit:
The nurse takes the health and medical
history and records various body
measurements then prepares various forms
for blood tests> Later the doctor will review
the health and medical history and perform
clinical examination on both couples.
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Next visit:
Doctor discusses the results of the
laboratory tests with couples and
provides advice to them. In case of a
person having a problem or showing sing
and symptoms of genetic disease he/she
will be referred to genetic clinic.
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Once the premarital check up and
counseling is over, the couples are given
a Certificate and it is up to them to
decide whether or not to marry.
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Other advantages of pre-marital check up:
1 - To allow for early detection and treatment of chronic
diseases.
2 - It helps in reducing the dosage or changing the drugs
used in chronic diseases to avoid complications to the
fetus.
3 - Offering general advices and health education on
nutrition to prevent fetal complications due to reasons
related to in proper maternal nutrition.
4 - Economical returns to the state by preventing
congenital anomalies.
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Other advantages of pre-marital check up:
5 - Public education on marriage, pregnancy,
childbearing, and care for body and child.
6 – Provision of medical and psycho-social support for
couples.
7 - Administering required vaccinations.
8 – Early detection of any illness and trial to cure it.
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After implementation of premarital counseling the
percentage has decreased to 0.9% for sickle-cell
anemia which shows a decrease of 60%.
Among school children the percentage also went
down to:
Sickle-cell anemia 2.1%
Carrier of sickle-cell anemia 8.13%
Thalassaemia 9.2%
G6PD 2.23%
G6pd carrier 9.1%
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Appropriate and extensive screening, accurate
detection and counseling of at risk couples,
along with antenatal diagnosis is a promising
strategy for the reduction of mortality and
morbidity from Thalassaemia in countries where
it is prevalent.
Tasleem, et al 2007
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