Sickle Cell Disease: Global Disparities in Prevalance and Outcomes

Unique challenges exist for people with sickle cell disease depending on where they live.

An estimated 300,000 to 500,000 babies worldwide are born with sickle cell disease (SCD) each year. In Africa and India, where SCD is most prevalent, newborn testing is not performed, and many children with sickle cell disease die before they are even diagnosed.

“Their death certificates will say malaria, anemia or pneumonia, though the real cause is sickle cell disease,” says Patrick McGann, MD, assistant professor in the Department of Pediatrics at University of Cincinnati.

Even in developed countries such as the United States, where the incidence of SCD is approximately 3,000 children each year, SCD is not easily treated. Children benefit from newborn screening and early initiation of treatment, but SCD is still characterized by extremely painful sickle cell crises and deadly comorbidities such as infections, lung disease and asthma.

Only one drug — hydroxyurea — is FDA approved to treat SCD. It works by decreasing the frequency of sickle cell crises. In Africa, it costs about $2/day, if it is available at all. The average family income in many parts of Africa is less than $2/day, leaving treatment out of reach.

“Sickle cell disease is a very painful condition with limited treatment options,” says Anthony Villella, MD, director of the Comprehensive Sickle Cell and Thalassemia Program at Nationwide Children’s Hospital. “Historically, there has been a serious lack of awareness and research funding for the development of novel therapies for sickle cell disease. Now, pharmaceutical companies are starting to commit resources to sickle cell research. I am optimistic that we will have more options in the future.”

References:

1. McGann PT. Strategies to Address the Global Burden of Sickle Cell Anemia. Translational Research in Heme/Onc/BMT Lecture at Nationwide Children’s Hospital. 2015 May 22.
2. Sickle Cell Disease Association of America. www.sicklecelldisease.org
3. American Society of Hematology. www.hematology.org