Sickle cell anemia is caused by an abnormal type of hemoglobin called hemoglobin S. Hemoglobin is a protein inside red blood cells that carries oxygen.
Hemoglobin S changes the red blood cells. The red blood cells become fragile and shaped like crescents or sickles.
The abnormal cells deliver less oxygen to the body's tissues.
They can also easily get stuck in small blood vessels and break into pieces. This can interrupt healthy blood flow and cut down even more on the amount of oxygen flowing to body tissues.
Sickle cell anemia is inherited from both parents. If you get the sickle cell gene from only one parent, you will have sickle cell trait. People with sickle cell trait do not have the symptoms of sickle cell anemia.
Sickle cell disease is much more common in people of African and Mediterranean descent. It is also seen in people from South and Central America, the Caribbean, and the Middle East.
Symptoms usually do not occur until after the age of 4 months.
Almost all people with sickle cell anemia have painful episodes called crises. These can last from hours to days. Crises can cause pain in the lower back, leg, joints, and chest.
Some people have one episode every few years. Others have many episodes each year. The crises can be severe enough to require a hospital stay.
When the anemia becomes more severe, symptoms may include:
The goal of treatment is to manage and control symptoms, and to limit the number of crises. People with sickle cell disease need ongoing treatment, even when not having a crisis. It is best to get care from health care that take care of many patients with sickle cell anemia.
People with this condition should take folic acid supplements. Folic acid helps make new red blood cells.
Treatment for a sickle cell crisis includes:
Blood transfusions (may also be given regularly to prevent stroke)
Plenty of fluids
Other treatments for sickle cell anemia may include:
Hydroxyurea (Hydrea). Helps reduce the number of pain episodes (including chest pain and breathing problems) in some people
Antibiotics. Help prevent bacterial infections, which are common in children with sickle cell disease
Treatments that may be needed to manage complications of sickle cell anemia include:
Joining a support group where members share common issues can relieve the stress of a chronic disease.
In the past, people with sickle cell disease often died between ages 20 and 40. Thanks to a better care people now can live to the age of 50 and beyond.
Causes of death include organ failure and infection.
Calling your health care provider
Call your health care provider if you have:
Any symptoms of infection (fever, body aches, headache, fatigue)
Painful and long-term erection (in men)
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Lee MT, Piomelli S, Granger S, et al. Stroke prevention trial in sickle cell anemia (STOP): extended follow-up and final results. Blood. 2006;108:847-852.
Brawley OW, Cornelius LJ, Edwards LR, Gamble VN, Green BL, Inturrisi C, et al. National Institutes of Health consensus development conference statement: hydroxyurea treatment for sickle cell disease. Ann Intern Med. 2008;148:932-938.
Geller AK, O'Connor MK. The sickle cell crisis: a dilemma in pain relief. Mayo Clin Proc. 2008;83:320-323.
Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by A.D.A.M. Health Solutions, Ebix, Inc., Editorial Team: David Zieve, MD, MHA, Bethanne Black, Stephanie Slon, and Nissi Wang.