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Sickle Cell Disease

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)

About Sickle Cell Disease

The term sickle cell disease (SCD) describes a group of inherited red blood cell disorders. People with SCD have abnormal hemoglobin, called hemoglobin S or sickle hemoglobin, in their red blood cells.

Hemoglobin is a protein in red blood cells that carries oxygen throughout the body.

"Inherited" means that the disease is passed by genes from parents to their children. SCD is not contagious. A person cannot catch it, like a cold or infection, from someone else.

People who have SCD inherit two abnormal hemoglobin genes, one from each parent. In all forms of SCD, at least one of the two abnormal genes causes a person's body to make hemoglobin S. When a person has two hemoglobin S genes, Hemoglobin SS, the disease is called sickle cell anemia. This is the most common and often most severe kind of SCD....Read more about Sickle Cell Disease
NIH - National Heart, Lung, and Blood Institute

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.

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.

Antibiotics for treating osteomyelitis in people with sickle cell disease

We reviewed the evidence to determine whether antibiotics (alone or in combination) given to people with sickle cell disease who have osteomyelitis (a bone infection) before the specific bacterium causing an infection is known is effective and safe as compared to bacterium‐directed antibiotic treatment and whether this effectiveness and safety is dependent on different treatment regimens, age or setting. This is an update of a previously published Cochrane Review.

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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.

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.

Antibiotics for treating osteomyelitis in people with sickle cell disease

We reviewed the evidence to determine whether antibiotics (alone or in combination) given to people with sickle cell disease who have osteomyelitis (a bone infection) before the specific bacterium causing an infection is known is effective and safe as compared to bacterium‐directed antibiotic treatment and whether this effectiveness and safety is dependent on different treatment regimens, age or setting. This is an update of a previously published Cochrane Review.

See all (51)

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.
Erythrocytes (Red Blood Cells)
A cell that carries oxygen to all parts of the body.
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.
Sickle Cells
Red blood cells that assume an abnormal, rigid, sickle shape.

More about Sickle Cell Disease

Photo of a young adult

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

Other terms to know: See all 6
Anemia, Blood, Erythrocytes (Red Blood Cells)

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