CINCINNATI -- Cincinnati Children's Hospital Medical Center, one of the nation's top 10 pediatric facilities, and the Sickle Cell Awareness Group of Greater Cincinnati, Inc., have been awarded a $191,000 federal grant to improve the follow-up rate of infants who screen positive for certain disorders of hemoglobin, including variants of sickle cell disease.
The US Department of Health and Human Services, Health Resources and Services Administration, awarded the grant to the Comprehensive Sickle Cell Center at Cincinnati Children's and the Sickle Cell Awareness Group. The programs are among 15 community-based programs in the nation to receive funding for this project.
Sickle Cell disease is a genetic disorder of hemoglobin, the oxygen-carrying pigment and predominant protein in red blood cells. The disease causes the cell to form a crescent, or sickle shape. This causes a painful "log jam" in the blood vessels, preventing oxygen from getting to other tissues in the body and compromising the body's ability to fight off infection. It eventually causes organ damage. Sickle Cell disease affects mostly individuals of African heritage, but is also found in individuals from the Mediterranean, India and Asia.
For every infant identified with sickle cell disease there are about 50 infants who have a hemoglobin variant, including sickle cell trait. This is a condition in which a person has one copy of the gene for sickle cell but does not have sickle cell disease. There are more than 700 different hemoglobin variants, and a few interact with the sickle cell gene and cause the red blood cell to sickle.
The identification rate of infants with Sickle Cell disease is nearly 100 percent, and most of these infants receive treatment in the first three to four months of life. The identification rate of infants with variants, however, is less then 40 per cent. Although individuals with sickle cell trait or other hemoglobin variants do not have clinical symptoms of the disease, they are of public health concern. Some infants identified as having a variant may actually have the disease and to have a second, confirmatory test for a definitive diagnosis. In addition, parents who have a child with a variant may be at risk of having another child with the disease, and all individuals with a hemoglobin variant are at risk of having a child with sickle cell disease.
The Comprehensive Sickle Cell Center is a component of the division of Hematology/Oncology at Cincinnati Children's. Founded 30 years ago, the Center is a nationally recognized comprehensive center for the care of individuals with sickle cell disease. The Center has been a leader in treatment, education and research.
The Sickle Cell Awareness Group of Greater Cincinnati, Inc. (SCAG) at the forefront of familiarizing the general public with the need for services and programs to address issues facing individuals and families affected by sickle cell disease. It was instrumental in ensuring that our community provided leadership and expertise in education, medical services and research. The organization is now affiliated with the Urban League of Greater Cincinnati, Inc., which enhances SCAG's ability to fulfill its mission of providing services for individuals and families affected by sickle cell disease.
