What is SCA? Children born with SCA have abnormally shaped, hemoglobin, which is a component of red blood cells. This abnormally shaped hemoglobin is termed Hemoglobin S. The RBCs in individuals with SCA are crescent shaped or sickled especially when they are exposed to low oxygen levels.
The abnormally shaped RBCs carry far less oxygen to the various tissues in the body. They are also more prone to causing clots and blockages in blood vessels leading to thrombosis, in some instances.
The life expectancy for a person with SCA used to be as young as 30 years of age. However, with improvements in the quality of treatment and prevention those people diagnosed with SCA can live well past the age of 50.
The current treatment for SCA is mainly supportive and includes blood transfusions, pain management and antibiotics to prevent bacterial infections, which are common in children with SCA.
To cure SCA, the affected individual must receive a bone marrow transfusion. The life of a normal RBC is about 120 days. The old RBCs are replaced by new ones produced by the bone marrow; such is also the case in those people with SCA. However, in persons with SCA, the bone marrow continues to replenish their blood supply with abnormal RBCs. A bone marrow transfusion would give the affected individual normal bone marrow and as a result normal RBCs.
Unfortunately, this treatment is not available to most SCA patients because there are not enough black bone marrow donors. In her interview with CNN Watkins stressed how crucial it is that more African-Americans donate their bone marrow to help save millions of children from a lifetime of suffering. For more information about becoming a donor please visit: www.marrow.org.
Source: CNN.com, www.ncbi.nlm.nih.gov
Photo Credit: rockstu.com, gossiboocrew.com
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