Sickle Cell Anemia and Research Funding

sickle cellsSickle cell anemia is a disease passed down through families in which red blood cells form an abnormal crescent shape (red blood cells are normally shaped like a disc).

Causes, Incidence, and Risk Factors

Sickle cell anemia is caused by an abnormal type of hemoglobin called hemoglobin S. Hemoglobin is a protein inside red blood cells that carries oxygen. Hemoglobin S changes the shape of red blood cells, especially when the cells are exposed to low oxygen levels. The red blood cells become shaped like crescents or sickles.

The fragile, sickle-shaped cells deliver less oxygen to the body's tissues. They can also get stuck more easily in small blood vessels, and break into pieces that interrupt healthy blood flow.

Sickle cell anemia is inherited from both parents. If you inherit the hemoglobin S gene from one parent and normal hemoglobin (A) from your other parent, you will have sickle cell trait. People with sickle cell trait do not have the symptoms of sickle cell anemia.

Sickle cell disease is much more common in people of African and Mediterranean descent. It is also seen in people from South and Central America, the Caribbean, and the Middle East.

Symptoms

Symptoms usually don't occur until after age 4 months. Almost all patients with sickle cell anemia have painful episodes (called crises), which can last from hours to days.

These crises can affect the bones of the back, the long bones, and the chest. Some patients have one episode every few years. Others have many episodes per year. The crises can be severe enough to require a hospital stay.

Links and Resources

  • Local funding needed to boost promising sickle cell research here (Cincinnati Herald) June 5, 2010.
    Cincinnati Children’s Hospital Medical Center has been at the forefront in advocacy, treatment and research involving sickle cell disease since its Comprehensive Sickle Cell Center was founded in 1971. Currently, experts at the center are leading many innovative studies that aim to improve treatments for sickle cell disease, including a breakthrough gene transfer therapy that will, if successful, end the suffering caused by the disease.

  • The Sickle Cell Cure Foundation Receives $100,000 Grand Challenges Explorations Grant from the Bill & Melinda Gates Foundation for Innovative Global Health Research (Sickle Cell Cure Foundation) May 15, 2010.
    The Sickle Cell Cure Foundation, Inc. (SCCF), announced today that it has received a US$100,000 Grand Challenges Explorations grant from the Bill & Melinda Gates Foundation. The grant will support an innovative global health research project conducted by Robert H. Broyles, Ph.D., SCCF President, titled “Malaria Stopped by a Human Protein Therapeutic.”

  • Armstead-Barnhill Foundation: Funding Sickle Cell Research at Johns Hopkins (Johns Hopkins Children's Center) May 11, 2009.
    When her young daughter, Leanne, was diagnosed with sickle cell disease in 1991, Andrea Armstead was devastated. An inherited blood disorder without a cure (other than bone marrow transplantation, which carries its own risk of death and serious complications), sickle cell (also known as sickle cell anemia) can lead to excruciating pain, organ damage and strokes. The disease’s mutation in the hemoglobin gene causes abnormal crescent-shaped blood cells to get stuck in blood vessels. Relief from the ensuing painful “crises” can require hospitalization and blood transfusions.

  • Sickle Cell Disease: A Question of Equity and Quality (Pediatrics) May 1, 2006.
    Thirty years ago, the first major federal legislation concerning sickle cell disease treatment was passed, resulting in the development of comprehensive sickle cell centers. We are now at another watershed moment in the treatment of this illness with the passage in October 2004 of the Sickle Cell Treatment Act, designed to substantially expand specialized sickle cell treatment programs.

  • Sickle Cell Research (The Children's Hospital of Philadelphia)
    The Children's Hospital of Philadelphia has a longstanding commitment to sickle cell disease research. More than 25 years ago, the Division of Hematology, to which the Sickle Cell Center belongs, initiated a chronic transfusion program for the prevention of stroke recurrence in children with sickle cell disease. Chronic transfusion for stroke prevention is now the standard of care for sickle cell patients at high risk of stroke.