19th June has been chosen to celebrate the Sickle Cell Day
Sickle Cell Disease (SCD) is the most frequent genetic disease worldwide. It is present on four continents: in sub-Saharan Africa and in the Maghrib, in Asia (Middle-East, Arabic peninsula, India), 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. Trans-continental, SCD is also trans-ethnic and affects black populations from African origin and Arabic, Indian and Caucasian populations from Southern Europe.
Through advocacy of the Sickle Cell Disease International Organisation (SCDIO), the support of the Republic of Congo and the Republic of Senegal, and the commitment in the scientific world, the African Union (in 2005), the UNESCO (2005), WHO (2006), and the United Nations (2008) recognized sickle cell disease as a public health priority. The 19 of June, has been chosen to celebrate every year the World Sickle Cell Day in order to raise awareness of the disease in the world.
Sickle-cell disease (SCD), also known as sickle-cell anaemia (SCA) and drepanocytosis, is a hereditary blood disorder, characterized by an abnormality in the oxygen-carrying haemoglobin molecule in red blood cells. This leads to a propensity for the cells to assume an abnormal, rigid, sickle-like shape under certain circumstances. Sickle-cell disease is associated with a number of acute and chronic health problems, such as severe infections, attacks of severe pain ("sickle-cell crisis"), and stroke, and there is an increased risk of death.
Sickle-cell disease occurs when a person inherits two abnormal copies of the haemoglobin gene, one from each parent. Several subtypes exist, depending on the exact mutation in each haemoglobin gene. A person with a single abnormal copy does not experience symptoms and is said to have sickle-cell trait. Such people are also referred to as carriers.
The complications of sickle-cell disease can be prevented to a large extent with vaccination, preventive antibiotics, blood transfusion, and the drug hydroxyurea/hydroxycarbamide. A small proportion requires a transplant of bone marrow cells