From the Research
Sickle cell disease management in Saudi Arabia should prioritize hydroxyurea as the primary disease-modifying medication, given its proven efficacy in reducing crisis frequency and severity, as supported by the most recent and highest quality study 1. The disease results from a genetic mutation affecting hemoglobin, causing red blood cells to become sickle-shaped under certain conditions, leading to vaso-occlusive crises, chronic anemia, and organ damage. Management in Saudi Arabia follows international standards, including hydroxyurea (starting at 15-20 mg/kg/day) as the primary disease-modifying medication to reduce crisis frequency and severity, with folic acid supplementation (1 mg daily) and prophylactic penicillin for children under 5 years. Acute pain crises are managed with hydration and analgesics, while blood transfusions are used for severe anemia or stroke prevention. The Saudi government has implemented premarital screening programs to identify carriers and reduce disease incidence, and specialized sickle cell centers exist in high-prevalence regions. Some key points to consider in the management of sickle cell disease include:
- The importance of hydroxyurea in reducing the frequency of painful crises and blood transfusions, as well as its potential to improve quality of life 1.
- The need for individualized treatment plans, taking into account the patient's specific needs and medical history 2.
- The role of blood transfusions in managing severe anemia and preventing stroke, as well as the potential risks and benefits of this treatment approach 3.
- The impact of sickle cell disease on patients' quality of life and financial well-being, highlighting the need for comprehensive and supportive care 4. Patients should maintain hydration, avoid extreme temperatures, and seek prompt medical attention for fever or severe pain. The high prevalence in certain regions is attributed to the protective effect the sickle cell trait provides against malaria, which was historically endemic in these areas. Overall, the management of sickle cell disease in Saudi Arabia requires a multifaceted approach, incorporating hydroxyurea and other evidence-based treatments, as well as supportive care and patient education.