From the Research
Sickle cell disease is prevalent in Saudi Arabia, with an estimated carrier frequency ranging from 2% to 27% across different regions of the country, and the most common subtypes include HbSS, HbS-β thalassemia, and HbSC disease. The highest prevalence is found in the Eastern Province, particularly in Al-Qatif and Al-Hasa regions, where carrier rates reach 25%, while the disease prevalence is approximately 2.1% 1. The Western and Southern regions also show significant prevalence.
Sickle Cell Subtypes in Saudi Arabia
- HbSS (homozygous sickle cell anemia) is the predominant form
- HbS-β thalassemia
- HbSC disease The Saudi-Indian haplotype is most common in the Eastern Province, characterized by higher fetal hemoglobin levels (15-30%) which often results in a milder clinical course compared to African haplotypes 2. The Benin haplotype is more prevalent in the Western region and is associated with more severe disease manifestations.
Geographic Distribution and Prevalence
The geographic distribution of different subtypes reflects historical migration patterns and genetic isolation of populations within the country. The high prevalence in certain regions is attributed to the historical selective advantage against malaria that the sickle cell trait provides, along with the high rate of consanguineous marriages in Saudi society 1.
Complications and Management
Common complications of SCD in Saudi Arabia include vaso-occlusive crises, acute chest syndrome, and acute painful crisis, among others 1. Hydroxyurea has been shown to be effective in decreasing the frequency of pain episodes and other acute complications in adults and children with sickle cell anemia of HbSS or HbSβºthal genotypes 3. However, there is still insufficient evidence on the long-term benefits of hydroxyurea, particularly in preventing chronic complications of SCD.
Recommendation
Based on the most recent and highest quality study, hydroxyurea is recommended for the management of SCD in Saudi Arabia, particularly for patients with HbSS or HbSβºthal genotypes, to reduce the frequency of pain episodes and other acute complications 3. However, further research is needed to address the uncertainties surrounding the long-term benefits and risks of hydroxyurea, as well as its effects on individuals with the HbSC genotype.