Sickle cell disease (SCD) is a group of blood disorders typically inherited from a person's parent

s.[2] The most common type is known as sickle cell anaemia (SCA).[2] It results in an abnormalit
y in the oxygen-carrying protein haemoglobin found in red blood cells.[2] This leads to a rigid,
sickle-like shape under certain circumstances.[2] Problems in sickle cell disease typically begin aro
und 5 to 6 months of age.[1] A number of health problems may develop, such as attacks of pa
in ("sickle cell crisis"), anemia, swelling in the hands and feet, bacterial infections and stroke.[1] L
ong-term pain may develop as people get older.[2] The average life expectancy in the develope
d world is 40 to 60 years.[2]

Sickle cell disease

Other names

Sickle cell disorder

Sickle cell 01.jpg

Figure (A) shows normal red blood cells flowing freely through veins. The inset shows a cross s
ection of a normal red blood cell with normal haemoglobin. Figure (B) shows abnormal, sickled
red blood cells sticking at the branching point in a vein. The inset image shows a cross-section
of a sickle cell with long polymerized sickle haemoglobin (HbS) strands stretching and distorting
the cell shape to look like a crescent.

Specialty

Hematology

Symptoms

Attacks of pain, anemia, swelling in the hands and feet, bacterial infections, stroke[1]

Complications

Chronic pain[2]

Usual onset

5–6 months of age[1]

Causes

Genetic[3]

Diagnostic method
Blood test[4]

Treatment

Vaccination, antibiotics, high fluid intake, folic acid supplementation. pain medication, blood trans
fusions[5][6]

Prognosis

Life expectancy 40–60 years (developed world)[2]

Frequency

4.4 million (2015)[7]

Deaths

114,800 (2015)[8]

Sickle cell disease occurs when a person inherits two abnormal copies of the haemoglobin gene,
one from each parent.[3] This gene occurs in chromosome 11.[9] Several subtypes exist, depend
ing on the exact mutation in each haemoglobin gene.[2] An attack can be set off by temperatu
re changes, stress, dehydration and high altitude.[1] A person with a single abnormal copy does
not usually have symptoms and is said to have sickle cell trait.[3] Such people are also referred
to as carriers.[5] Diagnosis is by a blood test, and some countries test all babies at birth for th
e disease.[4] Diagnosis is also possible during pregnancy.[4]

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.[5][6] Other measur
es may include blood transfusion and the medication hydroxycarbamide (hydroxyurea).[6] A small
percentage of people can be cured by a transplant of bone marrow cells.[2]

As of 2015, about 4.4 million people have sickle cell disease, while an additional 43 million have
sickle cell trait.[7][10] About 80% of sickle cell disease cases are believed to occur in Sub-Sahar
an Africa.[11] It also occurs relatively frequently in parts of India, the Arabian Peninsula and amo
ng people of African origin living in other parts of the world.[12] In 2015, it resulted in about 11
4,800 deaths.[8] The condition was first described in the medical literature by the American phys
ician James B. Herrick in 1910.[13][14] In 1949, the genetic transmission was determined by E. A.
Beet and J. V. Neel.[14] In 1954, the protective effect against malaria of sickle cell trait was desc
ribed.[14]
Signs and symptoms