About Sickle-Cell in Bahrain – Understanding Heath Issue in Context of State Violence

Features of sickle-cell disease in Bahrain
Volume 1, Issue 1, 1995, Page 112-119
Sheikha Salim Al Arrayed1 and Neva Haites

Genetic disorders of haemoglobin are prevalent in Bahrain. In a study of the hospital population covering 56 198 Bahrainis, we found that 2% of newborns have sickle-cell disease (SCD) and 18% have sickle-cell trait, while 24% are carriers of the (-thalassaemia gene. In a study of the presentation of SCD among Bahrainis it was found that the mild form of the disease predominates, but a wide clinical variability is apparent. It was also found that their haematological values are similar to those of patients from Eastern Province, Saudi Arabia, where the mild form of the disease predominates.

Introduction

The state of Bahrain is an archipelago of 33 islands, with the kingdom of Saudi Arabia to the west and Qatar to the east. The 1991 population was 500 000, one third non-Bahraini. Falciparum malaria was endemic in Bahrain until 1970 and so the malaria-associated genetic defects of red cells (sickle-cell disease [SCD], thalassaemia and glucose 6 phosphate dehydrogenase deficiency) were found to be common [1].

In 1990 it was found that hereditary anaemias were the third most frequent diagnosis at the Salmaniya Medical Centre, which is the main hospital in the country [1].

Sickle-cell disease (SCD) drains a country’s health resources and dramatically affects family and personal life. Accordingly we decided to study sickle-cell disease among Bahrainis.

The aims of these studies were to:

1. ascertain the incidence of genetic disorders of haemoglobin in the hospital population in Bahrain

2. ascertain the natural history of sickle-cell disease among Bahrainis

3. investigate the haematological characteristics of the Bahraini SCD patient

4. identify the haplotype associated with SCD mutation among Bahrainis.

We present here a summary of four studies performed on sickle-cell disease among our population.

1. Prevalence of genetic disorders of haemoglobins in the hospital population of Bahrain

Blood samples of 56 198 Bahraini nationals were analysed over a six-year period (1982-

1987). Of the total, 5 503 were neonatal samples (see Table 1) and the rest non-neonatal. Abnormal haemoglobin was detected in 44.35% of neonatal samples (24.2% were a-thalassaemia cases, 18.1% showed sickle-cell trait [SCT] and 2.1% had SCD). Hb Barts was the most common abnormal haemoglobin seen.

In the non-neonatal cases, the overall frequency of SCD was found to be 10.44%, and the frequency of those with SCD and Hb F present was 8.75%, which means that nearly 84 % of the SCD patients had Hb F present. Table 2 shows the distribution of quantitation of fetal haemoglobin (Hb F) in SCD patients with Hb S/F. Hb F varied between 2% and 40%. The majority of cases (about 76%) had Hb F in the range between 4.1% and 20%. The favourable protective role played by Hb F in sickle-cell disease is well-recognized by several workers [2-8], with the severity of the disease being inversely proportional to the quantity of Hb F.

The high incidence among the non-neonatal cases is due to the fact that a good number of cases were referred for Hb electrophoresis from outpatient clinics and hospital wards, and from health centres after getting positive results from a sickling test. …more