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Sickle Cell Anemia

Sickle cell anemia is a disease in which your body produces abnormally shaped red blood cells. The cells are shaped like a crescent or sickle. They don't last as long as normal, round red blood cells, which leads to anemia. The sickle cells also get stuck in blood vessels, blocking blood flow. This can cause pain and organ damage.

A genetic problem causes sickle cell anemia. People with the disease are born with two sickle cell genes, one from each parent. If you only have one sickle cell gene, it's called sickle cell trait. About 1 in 12 African Americans has sickle cell trait. A blood test can show if you have the trait or anemia. Most states test newborn babies as part of their newborn screening programs.

Sickle Cell Anemia on Wikipedia

'''Sickle cell anemia''' (American English), '''sickle cell anaemia''' (British English) or '''sickle cell disease''' is a Genetic disorder in which red blood cells may change shape under certain circumstances. This causes the cells to become stuck in capillary which deprives the downstream tissues of oxygen and causes ischemia and infarction. The disease usually occurs in periodic painful attacks, eventually leading to damage of internal organs, stroke, or anemia, and usually resulting in decreased lifespan. It is common in people from countries with a high incidence of malaria, and especially in West Africa.

Signs and symptoms

Patients have baseline anemia that varies in severity, with hemoglobin levels of 7-9 mg/dl typical. Often white blood cell counts are elevated simply due to marrow hyperactivity. Reticulocyte counts are elevated, reflecting new red blood cells replacing the rapidly cleared older cells - red blood cell life span is markedly reduced in this disease.

Vasoocclusive crises

Vasoocclusive crises are caused by sickled red blood cells that obstruct capillaries and restrict bloodflow to an organ, resulting in ischemia, pain, and organ damage. Because of its narrow vessels and function in clearing defective red blood cells, the spleen is frequently affected. It is usually infarction before the end of childhood in individuals suffering from sickle cell anaemia. This autosplenectomy increases the risk of infection from encapsulated organisms; preventive antibiotics & vaccinations are recommended for those with such asplenia. Liver failure may also occur with time. Bone is also a common target of vasoocclusive damage, especially when the bone is particularly weight-bearing. Such damage may result in avascular necrosis (especially of the femur) and ...   [ Read More ]


External Resources

Carolina Wren Press - Nonprofit publisher whose Lollipop Power Books specialize in multicultural, bilingual, nonsexist, and nontraditional children's books. Publishes Puzzles, the story of a ten-year-old girl with sickle cell anemia.

My Sickle Cell Homepage - Provides a first hand look at Sickle Cell Anemia, built by a person suffering from the disease. Frequently asked questions and a personal diary of the owner's own battle with this disease.

Mamas Health - A definition of sickle cell anemia, what it is, how one gets it, symptoms, statistics and testing.

Adam.com: Sickle Cell Anemia - Takes a look at this disorder with a definition, causes, incidence and risk factors.

The American Sickle Cell Anemia Association - A non-profit organisation based in Cleveland Ohio whose objectives are to raise awareness of the condition. Features educational material, FAQs, support groups and a message board.

M. D. Anderson Cancer CenterPediatric Bone Marrow Transplantation - Services, which are for childhood cancers of the blood, neuroblastoma, Ewing's sarcoma, Wilm's tumor, and non-cancerous blood disorders, including sickle cell anemia and immunodeficiency syndromes.

Family Practice Notebook: Priapism in Sickle Cell Anemia - Looks at the course, management and complications of this disease.

Henderson, Brian - Personal site of Florida native, with information and links to information regarding Sickle Cell Anemia

Boy receives first cord blood transplant for sickle cell anemia - [CNN]

Nevada Childhood Cancer Foundation - Organization provides social, emotional, psychological and educational support for families of children with critical illnesses including HIV/AIDS, hemophilia, sickle cell anemia, cancer, and immunological and renal diseases.


Related Pages on HealthTales.com:

Sickle cell disease
Anemia
Islet Cell Transplantation
X-linked sideroblastic anemia

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