Sickle cell anemia is a genetic disorder. It alters the shape of the red blood cells (RBCs). This decreases their ability to carry oxygen. It can also cause acute episodes of pain. These are called a sickle cell crisis. The body will also destroy the sickle cells. The loss of RBCs results in anemia.
The change in the ability and number of RBCs can decrease the amount of oxygen for the body.
Red Blood Cells: Normal and Sickle
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Sickle cell disease is a genetic disorder. If you receive 1 defective gene from each of your parents, then you will have sickle cell disease. If you only have 1 defective gene, you are said to have sickle cell trait, but not sickle cell disease. Although you won’t usually have any symptoms, you can pass this gene on to your children.
Sickle cell disease is more common in people who are black or of sub-Saharan origin. It is also more common in people of Greek and Italian descent as well as in people from some parts of India, Central and South America, and the Arabian Peninsula.
This condition produces a group of symptoms known as a sickle cell crisis. These are episodes of pain that occur with varying frequency and severity. It is usually followed by periods of remission. The risk for a sickle cell crisis increases with any activity that boosts the body's requirement for oxygen. This may include illness, physical or emotional stress, or high altitudes.
These painful crises can last hours or days. They affect the bones of the back, the long bones, and the chest. The crises can be severe enough to require hospital admission for pain control and IV fluids.
Symptoms of sickle cell crisis include:
Complications of sickle cell anemia include:
Hemoglobin electrophoresis is a simple blood test. It can be done by most laboratories or a local sickle cell foundation. Most states require testing of newborns for sickle cell.
Amniocentesis is a form of prenatal testing. It can also detect sickle cell disease.
Basic treatment of sickle cell crisis includes:
In addition, treatment may include:
Newborns with sickle cell disease may be given penicillin . It is given twice a day, beginning at age 2 months. It is continued until at least 5 years of age.
It is recommended that children with sickle cell disease who are 2-5 years of age receive pneumococcal vaccine (PCV-7) . Other routine immunizations should also be given, such as an annual flu shot.
Hydroxyurea is the first drug to significantly prevent complications of sickle cell disease. It increases the production of fetal hemoglobin. This decreases the number of deformed RBCs. As a result, it reduces the frequency of sickle cell crisis. Hydrea is not appropriate for everyone with sickle cell disease.
Blood transfusions can treat and prevent some of the complications. Transfusion therapy can help prevent recurring strokes in children.
A bone marrow transplant from a matched donor may be effective. There are medical risks involved. Recipients must also take drugs that suppress the immune system for the rest of their lives.
Sickle cell anemia cannot be prevented. There are some general guidelines that may keep the condition under control. Be sure to discuss these with your doctor:
American Sickle Cell Anemia Association
Sickle Cell Information Center
About Kids Health—The Hospital for Sick Children
Sickle Cell Foundation of Alberta
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1/4/2011 DynaMed's Systematic Literature Surveillance https://dynamed.ebscohost.com/about/about-us : Vichinsky EP, Neumayr LD, Gold JI, et al. Neuropsychological dysfunction and neuroimaging abnormalities in neurologically intact adults with sickle cell anemia. JAMA. 2010;303(18):1823-1831.
Last reviewed June 2016 by Michael J. Fucci, DO
Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
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