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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.

Treatments for long‐lasting and painful erection of the penis in boys and men with sickle cell disease

Priapism (the prolonged painful erection of the penis) is common in males with sickle cell disease (SCD). The length of time priapism lasts differs for different types and so does the medical treatment for it. Self‐management approaches may be helpful. We looked for randomised controlled trials of different treatments to find the best option. We found one study set in Jamaica in 1985 with 11 people. In the study, stilboestrol was compared to placebo. The study only reports one of the outcomes from the review, how often stuttering priapism occurs. There is no difference between treatment groups for this outcome. Due to lack of evidence, we are not able to conclude the best treatment of priapism in SCD. Alpha agonists have become the treatment of choice for acute fulminant priapism and to prevent stuttering priapism. However, this is not supported by a randomised controlled trial. More research is needed. One trial is under way in the UK at this moment in time.

Removing spleens from people with sickle cell disease after a splenic sequestration compared to blood transfusions to prevent further attacks

We reviewed the evidence to see whether removing all, of part of, the spleen to prevent acute splenic sequestration improved survival and decreased illness in people with sickle cell disease, as compared with regular blood transfusion. This is a 2015 update of a Cochrane review first published in 2002, and previously updated in 2013.

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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.

Treatments for long‐lasting and painful erection of the penis in boys and men with sickle cell disease

Priapism (the prolonged painful erection of the penis) is common in males with sickle cell disease (SCD). The length of time priapism lasts differs for different types and so does the medical treatment for it. Self‐management approaches may be helpful. We looked for randomised controlled trials of different treatments to find the best option. We found one study set in Jamaica in 1985 with 11 people. In the study, stilboestrol was compared to placebo. The study only reports one of the outcomes from the review, how often stuttering priapism occurs. There is no difference between treatment groups for this outcome. Due to lack of evidence, we are not able to conclude the best treatment of priapism in SCD. Alpha agonists have become the treatment of choice for acute fulminant priapism and to prevent stuttering priapism. However, this is not supported by a randomised controlled trial. More research is needed. One trial is under way in the UK at this moment in time.

Removing spleens from people with sickle cell disease after a splenic sequestration compared to blood transfusions to prevent further attacks

We reviewed the evidence to see whether removing all, of part of, the spleen to prevent acute splenic sequestration improved survival and decreased illness in people with sickle cell disease, as compared with regular blood transfusion. This is a 2015 update of a Cochrane review first published in 2002, and previously updated in 2013.

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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