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The Campaign to control genetic blood diseases in Bahrain.

 
 

Date   :  09-03-2011

Visits  : 58  
 

Attachments : لا يوجد

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COMMUNITY  GENETICS 2005;8:52-55

DOI: 10.1159/000083340

 

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

 

Abstract:

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.

 

Introduction:

 

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 .

 

Result:

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.

 

Discussion:

 

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

 

REFERENCES

 

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)

2002

 

 

 

Mean Age

Total

Sickle cell Disease

 

Carrier for Sickle cell

 

Normal

 

No.

No.

No.

%

No.

%

No

%

New born

0

1995

18

0.9%

325

16.3%

1652

82.8%

Mother

27

1210

16

1.3%

170

14.1%

1024

84.6%

Father

42

260

5

1.9%

21

8.1%

234

90.0%

 

Table /1

Comparison of SC Frequency between  New borns , Mothers and Fathers,2002

 

 

 

 

 

 

 

New Born Screening For G-6-PD deficiency (n=1.535) 

2002

 

 

•1-      Male

Female

Total

Status

No.

%

No.

%

No.

%

Normal

644

81.2%

661

88.9%

1305

85%

Reduced

148

18.7%

81

10.9%

229

14.9%

Trait

-

-

1

0.13%

1

0.1%

Table 2

The Frequency of Bahrain newborns with G6PD deficiency

 

 

 

 

 
     
 

Archive

Title Date Visits

 Levels of pain control

28-09-2011 210

 Public Awareness of Glucose-6- Phosphate Dehydrogenase ( G6PD) Dificiency

11-08-2011 214

 fact about alpha thalassemia

10-08-2011 231

 Prevalence of Abnormal Hemoglobins among Students in Bahrain: A Ten-Year Study

09-03-2011 60

 Beta Thalassemia Frequency in Bahrain: A Ten Year Study

27-09-2010 58

 Public awareness of sickle cell disease in Bahrain

23-09-2010 50

 Premarital genetic counselling A new law in the kingdom of bahrain

23-09-2010 49

 links of several reserch published on Bahrain Medical Bulletin Journal

31-08-2010 52

 The risks and benefits of long-term use of hydroxyurea in sickle cell anemia: A 17.5 year follow-up.

22-06-2010 50

 Frequency of G6PD Deficiency among Bahraini students: A Ten Years Study

01-03-2010 48

الصفحات :1-2-3

الارشيف

العنوان التاريخ الزيارات
 

Public Awareness of Beta Thalassemia in Bahrain

05-04-2012 52
 

THE CHANGING PROFILE OF CONSANGUINITY

01-02-2012 52
 

Levels of pain control

28-09-2011 50
 

The Campaign to control genetic blood diseases in Bahrain.

09-03-2011 58
 

Prevalence of Abnormal Hemoglobins among Students in Bahrain: A Ten-Year Study

09-03-2011 50
 

Beta Thalassemia Frequency in Bahrain: A Ten Year Study

27-09-2010 50
 

Premarital genetic counselling A new law in the kingdom of bahrain

09-03-2011 54
 

Public awareness of sickle cell disease in bahrain

09-03-2011 81
 

روابط لأبحاث مختلفة من مجلة البحرين الطبية

07-07-2010 53
 

Frequency of G6PD Deficiency among Bahraini students: A Ten Years Study

07-07-2010 50

الصفحات :1-2-3-4