From the Guidelines
The annual mortality rate from Sickle Cell Disease (SCD) is not explicitly stated in the provided studies, but it is estimated to range from 1-5% per year in developed countries, with significant variations by region and age. According to the study by 1, SCD affects millions of people worldwide, with a great impact on their morbidity and mortality, but it does not provide a specific annual mortality rate. However, the study by 1 mentions that 94% of children with SCD now survive until the age of 18 years, thanks to better surveillance, pneumococcus vaccination, penicillin prophylaxis, and treatment with hydroxyurea, implying a significant reduction in mortality rates.
Some key points to consider:
- Mortality rates have improved significantly in developed countries due to advancements in healthcare and treatment options.
- Children with SCD face the highest mortality risk in the first 5 years of life, particularly in developing countries.
- Common causes of death include acute chest syndrome, stroke, infections, and organ failure.
- Early diagnosis and consistent preventive care significantly improve survival rates for individuals with SCD.
It is essential to note that the provided studies do not offer a clear estimate of the annual mortality rate from SCD. However, based on the information available, it is clear that mortality rates from SCD can be significantly reduced with proper healthcare access, screening programs, and available treatments. The study by 1 highlights the importance of hematopoietic stem cell transplantation (HSCT) as a curative approach for SCD, particularly for young adults with severe complications.
From the Research
Annual Mortality Rate from Sickle Cell Disease (SCD)
- The exact annual mortality rate from SCD is not explicitly stated in the provided studies.
- However, it is mentioned that SCD is characterized by a significant reduction in life expectancy, with an average life expectancy 20-30 years less than the general population 2, 3.
- Approximately 300,000 infants are born annually worldwide with SCD, and most individuals with SCD live in sub-Saharan Africa, India, the Mediterranean, and Middle East 2.
- In the US, nearly all children with SCD survive to adulthood, but the life expectancy remains 20 years less than the general population 2.
- The studies highlight the importance of early detection and treatment, as well as the need for effective programs to manage SCD, particularly in resource-limited settings 4, 5.
- While there are various treatments available, including hydroxyurea, L-glutamine, crizanlizumab, and voxelotor, hematopoietic stem cell transplant is the only curative therapy, but it is limited by donor availability 2, 6.