From the Guidelines
The life expectancy for individuals with sickle cell disease is approximately 40 to 60 years, with significant improvements in survival rates due to modern treatments and comprehensive medical care. This improvement in life expectancy is largely attributed to better surveillance, pneumococcus vaccination, penicillin prophylaxis, and treatment with hydroxyurea, as noted in a study published in Haematologica 1. Key factors influencing life expectancy include the specific type of sickle cell disease, access to medical care, treatment adherence, and the presence of complications such as priapism, avascular necrosis, chronic pulmonary hypertension, stroke, and recurrent veno-occlusive crises. Some of the treatments that have contributed to improved survival rates include:
- Hydroxyurea, which increases fetal hemoglobin production and reduces painful crises
- Regular blood transfusions for certain complications
- Penicillin prophylaxis to prevent infections in young children
- Newer therapies like crizanlizumab, voxelotor, and stem cell transplantation, which have further improved outcomes, as indicated by the international expert panel in Haematologica 1. Early detection through newborn screening and comprehensive care at specialized centers are crucial for maximizing life expectancy, allowing for prompt management of acute complications and prevention of chronic organ damage that typically contributes to premature mortality. According to the study in Haematologica 1, 94% of children with sickle cell disease now survive until the age of 18 years, thanks to these advancements in treatment and care.
From the Research
Life Expectancy with Sickle Cell Disease
- The life expectancy of individuals with sickle cell disease (SCD) is reduced compared to the general population 2.
- Studies have shown that hydroxyurea therapy can improve life expectancy in individuals with SCD by reducing the frequency of painful episodes and other acute complications 3.
- Emerging evidence from observational studies indicates that hydroxyurea prolongs survival in children and adults with sickle cell anemia 3.
- However, the exact life expectancy with SCD is not specified in the available studies, and more research is needed to determine the long-term benefits of hydroxyurea therapy 2, 4.
Hydroxyurea Therapy and Life Expectancy
- Hydroxyurea therapy has been shown to decrease the frequency of painful episodes, acute chest syndrome, and blood transfusions in individuals with SCD 2, 5.
- The therapy also increases the level of fetal hemoglobin (HbF) and reduces the rate of neutrophil counts 2.
- While hydroxyurea therapy has been shown to improve life expectancy, the long-term risks and benefits of the therapy are still being studied 2, 4.
- The addition of hydroxyurea to chronic transfusion therapy has been shown to reduce transfusion burden in patients with sickle cell anemia 6.
Limitations of Current Research
- The current research on life expectancy with SCD is limited, and more studies are needed to determine the exact life expectancy and the long-term benefits of hydroxyurea therapy 2, 4.
- The available studies have methodological limitations, including small sample sizes and limited follow-up periods 2, 3.
- Further research is needed to address the uncertainties surrounding the use of hydroxyurea therapy in individuals with SCD 2, 4.