The genetic disorder sickle cell disease (sickle = segl, halvmåneformet) is still surrounded by confusion, discrimination and lack of treatment a centurry after it was discovered, writes IRINnews.
Especially in Africa where more than 200.000 babies are born every year with the disease. Up to one in four adults in sub-Saharan African countries like Nigeria carry the sickle cell trait, according to the World Health Organization (WHO).
For its impact on lives and livelihoods, SCD has been deemed a “threat to the economic and social development of Africa” by the West Africa-based Federation of Associations Combating Sickle Cell Disorder in Africa (FALDA).
OVERSET SYGDOM
Despite advances in treatment and research over the past century, SCD is still largely undiagnosed in the world’s most affected areas where the problem is too complex for any quick-fix solutions, researchers say.
And without treatment there is a 50 percent chance a sickle cell patient will die before the age of five, most commonly of a blood infection.
MISFORSTÅET SYGDOM
– People still don’t know about this sickness and there’s a lot of judgment, forcing sick people to hide, said Dramane Banao, president of a national initiative to fight SCD and mother of a 19-year-old woman with SCD in the West African country of Burkina Faso.
Sickle cell disease is inherited and present at birth, but can show no symptoms for the first four months of life.
BLODET KLUMPER
Characterized by irregular haemoglobin (iron-rich, oxygen-transporting protein in red blood cells), the disease causes red blood cells to morph into a sickle-shape (crescent) instead of a disc, which leads to clumping and blocked blood vessels.
This clumping can cause pain, infection and, in some cases, organ damage.
When sickle-shaped cells die, sickle cell anaemia, the most common form of SCD, takes hold.
Anti-cancer drugs and bone marrow transplants have extended the life expectancy of sickle cell patients into their 50s.
Though carriers do not necessarily experience symptoms, testing is recommended for genetic counselling.
A man and woman, if both are carriers, have a 25 percent chance of having a child with SCD.
