Sickle Cell, the black African blood heritage

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As 31-year-old Magueye Ndiaye describes it, when the pains caused by sickle cell disease come it is like being hit by a tornado.

“The pain is so difficult to describe. It’s right in your bones and you can’t move, you can’t eat,” he said. “You feel it in your back, your joints and in your kidneys.”

Sickle cell disease is an incurable genetic disorder that is widespread in sub-Saharan Africa and among descendents of Africans worldwide. The World Health Organisation (WHO) says that in some parts of sub-Saharan Africa, up to two percent of children are born with the condition.

West Africa, one of the most underdeveloped regions in the world, is particularly hard hit. The prevalence rate in Nigeria is among the highest with about 150,000 children born each year with the disorder.

Inherited illness

Sickle cell disease is passed on genetically from parent to child but not all carriers will have symptoms. In sickle cell, normal haemoglobin, referred to as A, is replaced by abnormal haemoglobin, referred to as S. Haemoglobin is the iron-containing protein in blood that carries oxygen from the lungs to body tissues.

When red blood cells are half A and half S, the person is considered a carrier, but if the red blood cells are formed entirely of SS, then the disorder will manifest itself as sickle cell disease. Red blood cells turn crescent shaped, making them stiff and fragile, clogging blood vessels and depriving the body of oxygen.

Sufferers have no visible symptoms, but periodically they suffer severe pain and are also highly prone to anaemia because the blood cells break down after only 10 to 20 days, rather than the usual four months.

A person can only inherit sickle cell disorder if both parents are carriers of the genetic trait and then there is a one in four chance of giving birth to an SS child.

Role of malaria

The prevalence of malaria in Africa, particularly West Africa, correlates with the high incidence of sickle cell disorder in the region.

Carriers of sickle cell are less prone to being infected with malaria, which attacks red blood cells, according to Dr. Frederic Galacteros, practitioner at the Sickle Cell and Thalassemis Centre at the Henri Mondor Hospital in Creteil, France.

Since carriers have a higher likelihood of surviving than those who might get malaria the genetic trait has a better chance of being passed on.

However, unlike carriers, those with sickle cell disease are actually more vulnerable to malaria because of their weakened health.

Sickle cell has a heavy impact on children, as malaria is the leading killer of those under five in Africa. Children with sickle cell are also highly vulnerable to infections and often suffer pain in their hands and feet. The deformed red blood cells can also clog the spleen, which can lead to death.

Sickle cell contributes to nine percent of deaths in children under five in West Africa, and up to 16 percent in some countries. Half of people born with sickle cell disease will die before the age of five, according to WHO.

Poverty prevents treatment

Africa is the most affected continent, yet the least equipped to deal with the disorder.

Screening at birth is rarely done. Few health centres specialise in the treatment of sickle cell, which can include prescribing painkillers and providing blood transfusions. Simple treatments such as the use of penicillin in children to prevent infections and taking folic acid to fight anaemia are financially out of reach for most West Africans.

A sickle cell association in Benin reported that even when it offered free screenings, few people came because they could not afford the cost to reach the centre. Ndiaye, who is unemployed, said that his recent stay in hospital cost him US$300.

“I studied catering but I have never had a fixed contract. Companies do not want to hire me because they don’t want to have to take care of me if I fall sick,” he said. “You get stigmatised in your professional life and in your private life.”

Sickle cell is still relatively unknown and misunderstood in Africa and those with the disorder often suffer discrimination. In some communities, children are ostracised if they are seen suffering pain from an unknown origin. They can be accused of witchcraft and abandoned by their parents.

Raising awareness

Corinne Mbebi-Liegeois, coordinator of the International Organisation for the Fight Against Sickle Cell, says education is key.

“We need to put in place national programmes for sensitisation to occur in schools and at the university level,” she said. “We also need to educate parents and doctors so they understand the disorder.”

At a recent conference on sickle cell in Senegal’s capital, Dakar, emphasis was placed on the management and treatment of the disease in Africa, considered a priority by WHO. Screening was considered crucial for prevention of sickle cell, and for patients to have knowledge of their disorder and receive proper treatment.

“It’s not easy, we did not ask to have this, and yet we cannot work or live normally,” said Ndiaye. “Society makes it hard for us.”

Health file  The lack of education and political will, poverty, out-moded traditional beliefs, to mention but a few, have been widely blamed for causing severe and sometimes unwarranted health catastrophies of genocidal proportions on the African continent. Child killer diseases, malaria, tuberculosis, water borne diseases, HIV/AIDS, among other preventable ailments have killed millions in their wake. As rightly said by the former UN Secretary General, Kofi Annan, on May 13, 2000 "More people (...) died of Aids in the past year (1999-2000, ndlr) in Africa than in all the wars on the continent".
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