Sickle cell

Sickle cell disease (SCD) is a group of blood disorders typically inherited from a person's parents. The most common type is known as sickle cell anaemia (SCA). It results in an abnormality in the oxygen-carrying protein haemoglobin found in red blood cells. This leads to a rigid, sickle-like shape under certain circumstances. Problems in sickle cell disease typically begin around 5 to 6 months of age. A number of health problems may develop, such as attacks of pain ("sickle cell crisis"), anemia, swelling in the hands and feet, bacterial infections, and stroke. Long-term pain may develop as people get older. The average life expectancy in the developed world is 40 to 60 years.

Causes

SCD is inherited from both parents who either have sickle cell disease or are carriers of the sickle cell trait. The gene is more common in certain ethnic groups, including people of African, Middle Eastern, Indian, Mediterranean, and Hispanic descent.

Diagnosis

Diagnosis is by a blood test, and some countries test all babies at birth for the disease. Diagnosis is also possible during pregnancy.

Treatment

The care of people with sickle cell disease may include infection prevention with vaccination and antibiotics, high fluid intake, folic acid supplementation, and pain medication. Other measures may include blood transfusion and the medication hydroxyurea (hydroxycarbamide). A small percentage of people can be cured by a bone marrow transplant.

Epidemiology

As of 2015, about 4.4 million people have sickle cell disease while an additional 43 million have sickle cell trait. About 80% of sickle cell disease cases are believed to occur in Sub-Saharan Africa. It also occurs among people of Hispanic background living in the United States and Spain. In 2015, it resulted in about 114,800 deaths.

See also

• Sickle cell trait
• Thalassemia

References

Sickle cell Resources
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