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Sickle cell anemia

Definition

Sickle cell anemia is a disease passed down through families. The red blood cells which are normally shaped like a disc take on a sickle or crescent shape. Red blood cells carry oxygen to the body.

Alternative Names

Anemia - sickle cell; Hemoglobin SS disease (Hb SS); Sickle cell disease

Causes, incidence, and risk factors

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

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:

  • Fatigue
  • Paleness
  • Rapid heart rate
  • Shortness of breath
  • Yellowing of the eyes and skin (jaundice)

Younger children with sickle cell anemia have attacks of abdominal pain.

The following symptoms may occur because small blood vessels become blocked by the abnormal cells:

  • Painful and prolonged erection (priapism)
  • Poor eyesight or blindness
  • Problems with thinking or confusion caused by small strokes
  • Ulcers on the lower legs (in adolescents and adults)

Over time, the spleen stops working. As a result, people with sickle cell anemia may have symptoms of infections such as:

  • Bone infection (osteomyelitis)
  • Gallbladder infection (cholecystitis)
  • Lung infection (pneumonia)
  • Urinary tract infection

Other symptoms include:

  • Delayed growth and puberty
  • Painful joints caused by arthritis 

Signs and tests

Tests commonly performed to diagnose and monitor patients with sickle cell anemia include:

Treatment

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)
  • Pain medicines
  • 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:

Bone marrow or stem cell transplants can cure sickle cell anemia, but this treatment is not an option for most people. Sickle cell anemia patients often cannot find well-matched stem cell donors.

People with sickle cell disease should have the following vaccinations to lower the risk of infection:

Support Groups

Joining a support group where members share common issues can relieve the stress of a chronic disease. 

Expectations (prognosis)

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)
  • Pain crises
  • Painful and long-term erection (in men)

References

Saunthararajah Y, Vichinsky EP. Sickle Cell Disease: Clinical Features and Management. In: Hoffman R, Benz EJ Jr, Silberstein LE, Heslop HE, Weitz JI, eds. Hematology: Basic Principles and Practice. 6th ed. Philadelphia, Pa: Saunders Elsevier; 2012:chap 40.

Rees DC, Williams TN, Gladwin MT. Sickle-cell disease. Lancet. 2010;376(9757):2018-2031.

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.


Review Date: 3/3/2013
Reviewed By: 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.
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