Sickle cell anemia is a disease passed down through families. The red blood cells that are normally shaped like a disc take on a sickle or crescent shape. Red blood cells carry oxygen throughout the body.
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:
Treatment for overuse or abuse of narcotic pain medicines
Wound care for leg ulcers
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:
Haemophilus influenzae vaccine (Hib)
Pneumococcal conjugate vaccine (PCV)
Pneumococcal polysaccharide vaccine (PPV)
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.
When to Contact a Medical Professional
Call your health care provider if you have:
Any symptoms of infection (fever, body aches, headache, fatigue)
Painful and long-term erection (in men)
DeBaun MR, Frei-Jones M, Vichinsky E. Hemoglobinopathies. In: Kliegman RM, Stanton BF, St. Geme JW III, et al., eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, Pa: Elsevier Saunders; 2011:chap 456.
Saunthararajah Y, Vichinsky EP. Sickle cell disease: clinical features and management. In: Hoffman R, Benz EJ Jr, Silberstein LE, et al., eds. Hematology: Basic Principles and Practice. 6th ed. Philadelphia, Pa: Elsevier Saunders; 2012:chap 40.
Rees DC, Williams TN, Gladwin MT. Sickle-cell disease. Lancet. 2010;376(9757):2018-2031.
Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.