Home > Health A – Z > Sickle Cell Anemia

Sickle Cell Anemia

An inherited disease in which the red blood cells have an abnormal crescent shape, block small blood vessels, and do not last as long as normal red blood cells. Sickle cell anemia is caused by a mutation (change) in a gene.

PubMed Health Glossary
(Source: NIH - National Cancer Institute)

Sickle Cell Anemia

Sickle cell anemia (uh-NEE-me-uh) is the most common form of sickle cell disease (SCD). SCD is a serious disorder in which the body makes sickle-shaped red blood cells. "Sickle-shaped" means that the red blood cells are shaped like a crescent.

Normal red blood cells are disc-shaped and look like doughnuts without holes in the center. They move easily through your blood vessels. Red blood cells contain an iron-rich protein called hemoglobin (HEE-muh-glow-bin). This protein carries oxygen from the lungs to the rest of the body.

Sickle cells contain abnormal hemoglobin called sickle hemoglobin or hemoglobin S. Sickle hemoglobin causes the cells to develop a sickle, or crescent, shape.

Sickle cells are stiff and sticky. They tend to block blood flow in the blood vessels of the limbs and organs. Blocked blood flow can cause pain and organ damage. It can also raise the risk for infection... Read more about Sickle Cell Anemia

What works? Research summarized

Evidence reviews

Hormone contraceptives for women with sickle cell anemia

Whether women with sickle cell anemia should use hormonal birth control is unknown. Sickle cell anemia is a blood disease. This type of anemia also causes bone pain known as sickle pain crises. A concern is that women with this disease using hormonal birth control may have blood vessels blocked by blood clots or have more bone pain. Clinicians often do not prescribe these types of birth control due to these concerns. However, many women with sickle cell anemia are sexually active, are able to get pregnant and are interested in contraception.

Inhaled drugs for opening up the airways in cases of acute chest syndrome in people with sickle cell disease

Sickle cell disease is an inherited blood disorder. People with sickle cell disease often suffer from acute chest syndrome, although it is not known why. Acute chest syndrome can cause fever, coughing, chest pain and shortness of breath and can be life‐threatening. Often, people with sickle cell disease and acute chest syndrome also wheeze. This suggests that airways are narrowed, as with asthma. Bronchodilators are drugs which relax the muscles in the airways, thus opening them up to make breathing easier. They are used in this way for asthma, so may be of similar use in acute chest syndrome. However, to March 2014, we found no trials to show the effects of these drugs for this condition. Research needs to assess the benefits and risks of using inhaled bronchodilators for acute chest syndrome in people with sickle cell disease.

Vaccines for preventing severe salmonella infections in people with sickle cell disease

Salmonella organisms are probably second only to pneumococcus among bacterial causes of infection in people with sickle cell disease. Infection with these bacteria can lead to complications and reduce the quality of life of people with the disease and sometimes result in death. Immunization with salmonella vaccines is one of the interventions available to reduce infection by these bacteria. There are different types of vaccines available: the inactivated vaccines and the oral vaccines. We did not find any randomized controlled trials assessing these vaccines in people with sickle cell diseases. We therefore conclude that there is a need for a randomized controlled trial to assess the benefits and risks of the different types of vaccines to evaluate the potential for improving survival and decreasing mortality from salmonella infections in people with sickle cell disease.

See all (101)

Summaries for consumers

Hormone contraceptives for women with sickle cell anemia

Whether women with sickle cell anemia should use hormonal birth control is unknown. Sickle cell anemia is a blood disease. This type of anemia also causes bone pain known as sickle pain crises. A concern is that women with this disease using hormonal birth control may have blood vessels blocked by blood clots or have more bone pain. Clinicians often do not prescribe these types of birth control due to these concerns. However, many women with sickle cell anemia are sexually active, are able to get pregnant and are interested in contraception.

Inhaled drugs for opening up the airways in cases of acute chest syndrome in people with sickle cell disease

Sickle cell disease is an inherited blood disorder. People with sickle cell disease often suffer from acute chest syndrome, although it is not known why. Acute chest syndrome can cause fever, coughing, chest pain and shortness of breath and can be life‐threatening. Often, people with sickle cell disease and acute chest syndrome also wheeze. This suggests that airways are narrowed, as with asthma. Bronchodilators are drugs which relax the muscles in the airways, thus opening them up to make breathing easier. They are used in this way for asthma, so may be of similar use in acute chest syndrome. However, to March 2014, we found no trials to show the effects of these drugs for this condition. Research needs to assess the benefits and risks of using inhaled bronchodilators for acute chest syndrome in people with sickle cell disease.

Vaccines for preventing severe salmonella infections in people with sickle cell disease

Salmonella organisms are probably second only to pneumococcus among bacterial causes of infection in people with sickle cell disease. Infection with these bacteria can lead to complications and reduce the quality of life of people with the disease and sometimes result in death. Immunization with salmonella vaccines is one of the interventions available to reduce infection by these bacteria. There are different types of vaccines available: the inactivated vaccines and the oral vaccines. We did not find any randomized controlled trials assessing these vaccines in people with sickle cell diseases. We therefore conclude that there is a need for a randomized controlled trial to assess the benefits and risks of the different types of vaccines to evaluate the potential for improving survival and decreasing mortality from salmonella infections in people with sickle cell disease.

See all (36)

Terms to know

Anemia
A condition caused when the body does not have enough red blood cells or hemoglobin. Hemoglobin is a protein in the blood that carries oxygen.
Blood
A tissue with red blood cells, white blood cells, platelets, and other substances suspended in fluid called plasma. Blood takes oxygen and nutrients to the tissues, and carries away wastes.
Genetic
Inherited; having to do with information that is passed from parents to offspring through genes in sperm and egg cells.
Hemoglobin
A protein inside red blood cells that carries oxygen from the lungs to tissues and organs in the body and carries carbon dioxide back to the lungs.
Red Blood Cells (Erythrocytes)
A cell that carries oxygen to all parts of the body.
Sickle Cells
Red blood cells that assume an abnormal, rigid, sickle shape.

More about Sickle Cell Anemia

Photo of a young adult

Also called: Sickle cell anaemia, Sickle cell disease, Hemoglobin S disease, Drepanocytosis, SCA, SCD, HbS

Other terms to know: See all 6
Anemia, Blood, Genetic

Related articles:
What Does Blood Do?

Keep up with systematic reviews on Sickle Cell Anemia:

RSS

PubMed Health Blog...

read all...