World Sickle Cell Day

Sickle-cell disease (SCD) is a group of blood disorder . The most common type is known as sickle-cell anaemia (SCA). It results in an abnormality in the oxygen-carrying protein haemoglobin  (hemoglobin S) found in red blood cells. This leads to a rigid, sickle shape under certain circumstances. 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 hand and feet, bacterial infections and stroke .Long term pain may develop as patient get older.

Sickle-cell disease occurs when a person inherits two abnormal copies of the hemoglobin gene, one from each parent. This gene occurs in chromosome 1. Several subtypes exist, depending on the exact mutation in each haemoglobin gene. An attack can be set off by temperature changes, stress,dehydration, and high altitude.A person with a single abnormal copy does not usually have symptoms and is said to have sickle cell trait. Such people are also referred to as carriers. Diagnosis is by a blood test and some countries test all babies at birth for the disease. Diagnosis is also possible during pregnancy.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 hydroxycarbamide (hydroxyurea)A small proportion of people can be cured by transplant of bone marrow.

Sickle Cell Disease is one of the most frequent genetic disease worldwide. It is present on four continents: in sub-Saharan Africa , in Asia (Middle-East, Arabic peninsula, India,Pakistan), in the Americas, on the North (USA), centre (Guatemala, Caribbean islands), and on the South (Brazil,Surinam, Guiana), in Southern Europe (Southern Italy and Sicily, Greece, Turkey). It is estimated that 500.000 are born every year with this severe and invalidating condition and that 50% of them will die before the age of 5 years..

Abnormal haemoglobin forms can be detected on haemoglobin electrophoresis. Sickle-cell haemoglobin (HgbS) and haemoglobin C with sickling (HgbSC) the two most common forms . The diagnosis can be confirmed with high performance liquid chromatography ,Genetic testing, are highly specific for HbS and HbC .An acute sickle-cell crisis is often precipitated by infection. Therefore, a urinalysis to detect an occult urinary tract infection, and chest X-ray to look for occult pneumonia should be routinely performed.People who are known carriers of the disease often undergo genetic counseling, before they have a child. A test to see if an unborn child has the disease takes either a blood sample from the fetus or a sample of amniotic fluid. Since taking a blood sample from a fetus has greater risks, the latter test is usually used. Neonatal screening provides a better method of early detection for individuals with sickle-cell disease

Sickle-cell crisis

Splenic sequestration crisis

Acute chest syndrome

Aplastic crisis

Hemolytic crisis