COMMUNITY GENETICS 2005;8:52-55
The Campaign to control genetic blood diseases in Bahrain.
Dr Shaikha Al Arrayed ,MBCh B, DHCG, PhD.
Head of Genetic department, Salmaniya Medical Complex,
Kingdom of Bahrain
Genetic blood diseases are frequent in Bahrain as in all Middle Eastern countries.
Previous neonatal screening study done in 1984-1985 showed that the incidence of sickle cell disease (SCD) was 2.1%, of sickle cell trait (SCT) 11%, alpha-thalassaemia.
Trait 20%, and G6PD 25%, while the carrier rate for beta Thalassaemia was 3%.
This means that 210 babies with SCD ,were expected to be born annually.
During the seventies, patients attending health services were treated symptomatically.
At the beginning of the eighties, the service improved for these patients.
The ministry of health began organizing services provided to them
through health centers, medical clinic and pediatric clinic. In 1984,
the first genetic clinic was established at the Salmanyia medical Complex and
premarital counseling began for families at risk. Information booklets were prepared and
distributed widely and educational campaigns started in school and clubs in
a attempt to increase awareness about these diseases among students and the public. In 1991,
Bahrain Hereditary Anemia Society was formed with the aim of fighting these diseases and
raising the standard of care for these patients. In 1992, the Minister of Health formed
a national committee for prevention of genetic disease in Bahrain, which elaborated
a plan-aimed at preventing and eliminating these diseases.
The plan advised concentrating on screening and education.
Screening all pregnant women, followed by the newborn testing if the mother was found to be a carrier.
In 1993, premarital counseling service was organized by the genetic department and maternal and child health care physicians.
A form was designed to standardize the service through all health centers, and although initial acceptance levels were poor momentum has gradually increased.
In 1998, a student-screening project began.
The aim of screening all students before leaving school was to raise the awareness among youth about hereditary anemia, and to advise them to take the right decision in order to prevent these diseases in their future families.
A card with blood results was given to each student along with an information booklet about the disease he/she carries. This project is now entering its sixth year and has made a tremendous impact on awareness, among students and families. And it resulted in an increase in premarital counseling attendance.
In 2001, the Shura Council agreed to make PMC mandatory before marriage, although the couple has freedom of choice. The Ministers' Cabinet also agreed on a PMC law, which will be implemented within one year.
Recently, a newborn screening study was done to determine the effects of this long Campaign (16-18 years). 2000 Bahraini newborns were tested, 18 newborns were found to have sickle cell disease. The new figure for SCD incidence in Bahrain is 0.9% this indicates a 60% decline in incidence rate.
With the continuation of education, awareness campaigns, screening of carriers and premarital counseling, we expect the number of affected children born to be reduced tremendously over the next few years.
Thus, for the first time, Bahrain has recorded a less than one per cent birth rate of babies suffering from sickle cell disease and, if this trend continues, Bahrain will soon become the first country in the Gulf region to completely eliminate the disease.
The state of Bahrain is made up of an Archipelago of thirty-six islands, with an area of 694 square kilometers, located in the Arabian Gulf. The kingdom of Saudi Arabia is on the west and Qatar on the East.
The estimated 2002 population was 627,123.
The percentage of Bahrainis is 62.4% and non-Bahrainis 37.6%.
Growth birth rate and population growth rates over the last five
years have been stable. In 2002 the crude birth rate was 20.1 per 1000 population.
The infant mortality rate has been reduced to 7/1000 live births in 2002.
There has been a significant increase in life expectancy at birth. Children born today
will on average live to 73.8 years. (1)
Falciparum malaria was endemic in Bahrain until 1970, and so the malaria- associated genetic defects of red cells such as Sickle cell disease (SCD), thalassaemia and glucose- 6- phosphate dehydrogenase deficiency (G6PD) were expected to be common. These genetic diseases are passed from generation to generation through marriages between carriers. There is no real cure, and the management is supportive and symptomatic only. Preventive measures remain the best way of dealing with these diseases.
A new-born screening study conducted during 1984-1985 in Bahrain revealed that 2.1% had sickle cell disease, with 210 babies were born every year in Bahrain suffering from SCD, 11.2% were carriers for sickle cell disease, and 20% with G6PD deficiency (2) . The carrier status for Beta thalassaemia was found to be 2-4% in a premarital counselling study. (3)
These statistics reflect the fact that a large number of children are suffering from these diseases. This affects individuals, families, and the society, both socially and economically.
The Cyprus Thalassaemia Control Program has succeeded in reducing the incidence of B- thalassaemia major in the country through measures such as health education, carrier screening, premarital counselling and prenatal diagnosis. This success has encouraged other countries to adopt the same measures.4-8
In Bahrain, during the 1970s patients attending the health services were treated symptomatically.
Thalassaemia patients were under-treated and under-transfused.
Many of them developed complications and dysmorphic features.
Many families had 3-5 children with thalassaemia or sickle cell disease, and patients
with sickle cell disease were treated as malingerers and subjected to neglect in emergency rooms and hospitals.
They were often viewed as drug users since they often come for analgesics.
At the beginning of the 1980s, the service improved for these patients.
The Ministry of Health started to organize services provided to them, through Health Centers,
Medical Clinics and Pediatric Clinics. In 1984,
the first Genetic Clinic was established, at Salmaniya Medical Complex.
Premarital counselling started at the Genetic Clinic for families at risk. Information booklets
were designed and distributed widely, and educational campaigns started in school and clubs,
to increase awareness among students and the public about these diseases.
In 1991 the Bahrain Hereditary Anaemia Society was formed with the aim of fighting the
haemoglobinopathies, and raising the standard of care for the patients.
In 1992 the Minister of Health formed a National Committee for Prevention of Genetic
Disease in Bahrain. The Committee formulated a plan aimed at preventing and eliminating these diseases. The Committee advised to concentration on screening and education 9-12.
Screening all pregnant women started, and if the mother was found to be carrier the newborn was screened.
In 1993 , Premarital Counseling Services at Health Centers were organized by the Genetic Department and Maternal and Child Health Department.
Training courses were given to all primary health care physicians, and a form was designed to
standardize the service through all health centers.
Premarital Counseling (PMC) become available on wide basis through all the Health Centers.
Initially acceptance was poor,
but it gradually has gathered momentum 3.
In 1998 student screening project started. The aim of screening all students before they leave school
was to raise awareness among the youth about hereditary anaemia,
and advise them to take the right decision to prevent these diseases in their future families.
A card with their blood test result was given to each student with an information
booklet about the disease he carries.
This project is continuing for the sixth year, and it has had a tremendous effect on awareness,
not only among students, but also among their families,
as they ask to be tested if they know that their son is a carrier for a certain disease.
It also positively affects attendance for premarital counselling.
In 2001 the Shura Council agreed to make PMC mandatory before marriage.
Genetic counseling will be provided for the couples at risk.
these couples have the freedom of choice if they decide to marry against the advice.
2002, the Ministers' Cabinet Council agreed on the PMC law, to be implemented within one year.
Subjects and Methods:
1-At this stage we wished to update the national figures for the incidence of the common
genetic blood disorders such as sickle cell disease, thalassaemia, and G6PD among newborns in Bahrain,
and compare it with the previously available figure.2- Establish a data base registry for all affected newborns.
3-Identify the incidence of other abnormal hemoglobin.
4- Study the parental ages among Bahraini parents , and 5- Study the consanguinity rate in Bahrain.
The study was a collaborative project between the Ministry of Health,
World Health Organization and Bahrain Hereditary Anemia Society.
The National hereditary Disease Committee organized and directed the project,
which was conducted between February and April 30 ,2002
A total of 2000 Bahraini newborns were tested, which represented all deliveries in
Ministry of Health Maternity Hospitals during the period of study .
80-90% of all Bahraini newborns during that period were included.
Cord blood samples were collected from the newborns, in tubes containing EDTA,
and transferred to the Hematology Laboratory at Salmaniya Medical Complex for analysis.
High performance liquid chromatography HPLC machine was used to perform electrophoresis on the cord blood samples, with a fluorescent screening technique for G6PD deficiency.
A questionnaire was completed for each new born by nurses in the Maternity Units.
The form included demographic data, parental age group,
and consanguinity, and was coded for computerization.
The requisite training of doctors, nurses and technicians was undertaken
by senior personnel in each field. A confirmatory test was conducted on
all the newborns with sickle cell disease .
Five samples from the newborn was not included because the blood was clotted
Only 18 out of the 1995 Bahraini newborns born in Ministry of Health facilities
had sickle cell disease, while sickle cell trait found in 16.3% (325/1995) of the newborns Table 1
SCD was found in 1.9% (5/260) of the fathers ,and 1.3% (16/1210) of mothers tested
Sickle cell trait was found in 8 % ( 21/280) of the fathers , and 14% ( 170/1210) of the mother tested
G-6-pD Deficiency was present in 18.0% of male newborns, and 10.0% of female newborns.
The 0.9% SCD incidence in 2002 compares with the previous estimate of 2.1% reported in 1984084 ,
i.e., a decline of almost 60%.
Now we expect the birth of 90 babies annually with SCD, instead of 210, which means
that 120 newborns have been protected from having the disease annually.
The sickle cell trait prevalence of 16.3 in 2002 compares with previous
estimates of 11% in 1986, 13% in student screening in the year 1998, and 14% in 2001.
As shown from Table 1 ,the incidence of SCD was higher in th parents than their children
The trait rate showed the opposite trend, as it rises gradually, which had been expected,
as more carriers ( HbA/ HbS) marry ( HbA/HbA) persons.
With the continuation of education, awareness campaigns, screening carriers,
and premarital counselling we expect the number of children born with
SCD to be reduced tremendously in the next few years.
The G-6-PD deficiency of 18% in the male newborns,
and 10% in the female newborns, compares with reported values of almost
25% in many previous studies. Ref
Although sickle cell disease is common in Bahrain ,
a previous study showed that we have the mild form of sickle cell disease in approximately
90% of patients. DNA analysis has proven the presence of the Asian haplotype as the common haplotype .
This mutation is associated with high level of HbF.
A situation similar to that in the Eastern province of Saudi Arabia.13-14
For the first time, Bahrain has recorded less than 1% of newborns with sickle cell disease.
If this trend continues, Bahrain could soon become the first country in
the Gulf region to completely eradicate the disease. We hope to be able to
protect all our children from this chronic and debilitating disease in the near future.
This is a success story for both the Ministry of Health in Bahrain, the Bahrain Hereditary
Anemia Society and all those involved in this campaign
1-Health Statistics 2002, Bahrain Health Information Directorate, Ministry of Health.2002
2-Mohammed A, Al-Hilli F, Nadkarni K; Bhagwat G; Bapat J, Hemoglobinopathies and Glucose-6-Phosphate Dehydrogenase Deficiency in Hospital Birth in Bahrain. Annals of Saudi Medicine Vol. 12. 1992: 536-539.
3- Al Arrayed, Premarital Counseling, Eastern Mediterranean Health, Journal,Vol 3,No3, 1997, 415-419.
4-Community Control of Hereditary Anemia: Memorandum from a WHO meeting. Bulletin of the World Health Organization, 1983, 61(1): 63-80.
5- Angastiniotis M., Kyriakidou S., and Hadjiminas M. The Cyprus Thalassemia Control Program. Birth Defects : Original Articles Series, Vol. 23, No.5, 1988 : 417-432.
6-Angsstinoiotis M, Kyriakidou S, and Hadjiminas M, How Thalassemia was controlled in Cyprus . World health Forum Vol. 7 1986 :293-297.
7- Community Control of Genetic and Congenital disorders, EMRO Technical Publication Series 24, World Health organization 1997, 194-199.
8 Hereditary aAemia ( genetic basis, clinical features, diagnosis and treatment). Bulletin of the World Health Organization, 1982, 60(5): 643-60.
9-Al-Arrayed S. The Nature of Sickle-Cell Disease in Bahrain. Journal of the Bahrain Medical Society, 1994, 6(3):125-30.
10-Al Arrayed S , Hamza A, A Survey of Patients with Sickle Cell presentation to Accident and Emergency Department of SMC .Bahrain , Journal of Bahrain Medical Society ,Vol 7,No 2,August 1995,105-112.
11-Al-Arrayed SS, Neva Haites. Features of Sickle-Cell Disease in Bahrain. East Mediterranean health journal, 1995, 1(1):112-9.
12-Al-Arrayed S. Hematological Characteristics in Bahraini Sickle-Cell Disease Patients. Journal of the Bahrain Medical Society, 1991, 2(1):32-5.
13- Al Arrayed SS , Beta Globin Gene Haplotype in Bahraini Sickle Cell Anemia Patients, BMB, Vol 1 March 1995,15-20
14- Nadkarni KV, Al-Arrayed SS, Bapat JP. Incidence of Genetic Disorders of Haemoglobin in the Hospital Population of Bahrain.
Bahrain Medical Bulletin 1991;131:19-24.
New Born Screening For Genetic Blood Diseases (n=1.995)
Sickle cell Disease
Carrier for Sickle cell
Comparison of SC Frequency between New borns , Mothers and Fathers,2002
New Born Screening For G-6-PD deficiency (n=1.535)
The Frequency of Bahrain newborns with G6PD deficiency