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Still No Cure for Sickle Cell Disease

September 3, 2013

Originally published Sep 19, 2011

It occurs in about 1,000 babies each year in America and in one out of every 500 African-American births. Over 70,000 people in the United States are estimated to have it. It is an inherited disorder that affects red blood cells that become hard and pointed instead of soft and round. These hard and pointed cells block small blood vessels which mean that less blood can reach that part of the body. Tissue that does not receive a normal blood flow eventually becomes damaged. What disease is this?

The disease is called and described as Sickle Cell which is characterized by red blood cells that assume an abnormal, rigid, sickle shape.

September is Sickle Awareness Month and the Georgia Department of Public Health is reminding families of the importance of newborn screenings. Newborns in Georgia are screened for 28 metabolic and hemoglobin disorders. Last year 148,989 newborn screening specimens (blood spots) tested in the Georgia Public Health Laboratory brought diagnoses of 142 infants with metabolic diseases and 125 infants hemoglobin disorders including Sickle Cell anemia.

In Georgia, one in one thousand infants is born with Sickle Cell disease. Georgia has one of the third largest Sickle Cell disease populations in the nation. This disease has been consistently among the top five specific causes of inpatient hospitalization for all age groups of children age one to 21 years.

The disease affects the hemoglobin which is the main substance of the red blood cell. Hemoglobin helps red blood cells carry oxygen from the air in lungs to all parts of the body. Medical problems caused by Sickle Cell disease include lung tissue damage, pain episodes and stroke. The blockage of blood flow caused by sickled cells also causes damage to most organs including the spleen, kidneys and liver.

Sickle cell trait is not an illness. It means that one has inherited a gene for sickle cell. It does not turn into sickle cell disease. If someone has sickle cell trait and his partner has sickle cell trait, they may produce a child with Sickle Cell disease.

Newborns are screened at birth for the disease which is a preventive public health function to identify at-risk infants in the first weeks of life so that early intervention is implemented to prevent disability or death.

“Georgia is one of seven states participating in the Registry and Surveillance for Hemoglobinopathies (RuSH) Project, which is managed by CDC and funded by the National Heart, Lung, and Blood Institute and the National Institutes of Health, to determine the incidence and prevalence of hemoglobinopathies in the United States and to improve the system of care for patients,” said Sharon Quary, Manager in the Newborn Screening Unit.

There is no universal cure for sickle cell disease. Research in gene therapy, the ultimate universal cure, is currently underway.

For additional information, please call the Georgia Newborn Screening Sickle Cell Follow up Program at (404) 657-4143, the Sickle Cell Foundation of Georgia, Inc. at (404) 755-1641.