Sickle Cell Disease
Sickle Cell Disease is a term for a group of hereditary blood disorders. Sickle Cell Disease is caused by abnormal hemoglobin, the protein in red blood cells that carries oxygen from the lungs to the tissues. People with sickle cell disease make hemoglobin S (Hb S) instead of the normal hemoglobin A. The red blood cells of persons with sickle cell disease don't last as long as 'normal' red blood cells. This results in chronic anemia.
Also, these red blood cells become rigid and bend, taking on a 'sickle ' or crescent shape. These odd shaped cells are not flexible enough to squeeze through small blood vessels. This may result in blood vessels being blocked. The areas of the body served by those blood vessels will then be deprived of their blood circulation. This damages tissues and organs and causes pain. Organs that can be damaged include bones, kidneys, lungs and the brain.
In the United States, sickle cell disease is found primarily in people of African ancestry. However, many other people, especially those coming from the Mediterranean, Middle East and some part of India have this disorder. The most common type of sickle cell disease is sickle cell anemia which affects more than 50,000 Americans. One in 375 African Americans is born with sickle cell anemia.
Sickle Cell Disease should not be confused with Sickle Cell Trait.
Sickle cell trait is not a disease. Individuals who have sickle cell trait are not ill and require no special treatment. Sickle cell trait is the carrier state for sickle cell disease. People with sickle cell trait make both normal hemoglobin (Hb A)- and hemoglobin S (Hb S). Their hemoglobin type is AS. One in 10 African Americans has
sickle cell trait. Anyone considering having children should have a blood test to determine their hemoglobin type. If both parents have sickle cell trait, there is one chance in four that their baby will have sickle cell disease.
In Maryland, all newborn babies are screened for sickle cell disease. Once a baby is identified, we try to make sure that he or she gets comprehensive follow-up care. Sickle cell disease can cause severe illness, but there are things you can do to try to stay healthy. A child with sickle cell disease should:
- See their pediatrician for all check ups and be seen right away if they get a fever (temperature greater than 101.5 F).
- Get all of their shots on time, and extra pneumococcal and meningococcal shots starting at 2 years of age.
- Take penicillin twice a day at least until they are 6 years old.
- Get a flu shot every year.
- See a hematologist (blood disorder specialist) when diagnosed and at least once a year.
- Get yearly eye exams and a special test (transcranial doppler or TCD) once a year to to check for stroke risk.
The leading cause of death in children with sickle cell disease in the United States is infection. Giving young children with sickle cell disease penicillin every day and giving them special immunizations can prevent most of the infections and dramatically reduce the death rate. Maryland has the lowest death rate in the United States among children with sickle cell disease.
We do not have complete information on how long people with sickle cell disease can expect to live. The reported average life span is 42 years for men and 48 for women. Treatment for the symptoms of sickle cell disease has improved over the years and many people with sickle cell disease are living beyond middle age, holding jobs, and carrying out family responsibilities. We know some patients in their 70's and 80's. Further information can be found at link
www.scinfo.org.
