(formerly IRIN News) Journalism from the heart of crises

Sickle-cell disorder killing 100,000 infants a year

[Nigeria] Mustapha Kabiru is three years old and crippled by polio - he has only partial use of his right leg. His father stopped the child from having a free polio vaccination after Muslim and political leaders in Kano, Northern Nigeria, said the vaccine
Sarah Simpson/IRIN

At least 100,000 infants die from the sickle-cell genetic disorder in Nigeria every year, and the country still has the highest incidence of the illness in Africa.

“From available statistics, 100,000 infants die from sickle-cell disease in Nigeria annually, making it the number one sickle-cell endemic country in Africa,” Sadiq Wali, president of the Nigeria Sickle-cell Foundation, told IRIN.

“Based on World Health Organization [WHO] indices, Nigeria accounts for 75 percent of infant sickle-cell cases in Africa and almost 80 percent of infant deaths from the disease in the continent”, Wali said.

According to the WHO, 200,000 infants are born with sickle-cell in Africa every year, with Nigeria accounting for about three-quarters of these births. Sixty percent of the 200,000 will die as infants.

Sickle-cell disease is an incurable genetic disorder widespread in sub-Saharan Africa and among descendents of Africans worldwide. Sufferers have no visible symptoms, but periodically experience severe pain and are also highly prone to anaemia because the blood cells break down after only 10-20 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 affected child. WHO says that in some parts of sub-Saharan Africa, up to 2 percent of children are born with the condition. For more on this see Africa: Little help for those who suffer from blood disorder.

“This genetic disorder alone accounts for 8 percent of infant mortality in Nigeria which calls for urgent attention”, Wali said.

Around four million Nigerians are estimated to suffer from the disease, while 25 million others carry the genes which they pass to their offspring.

Link with malaria?

According to the WHO, sickle-cell is particularly prevalent in areas of high malarial transmission.

“The mutant sickle-cell gene confers a survival advantage against malaria which explains the prevalence of the disease in Nigeria where malaria is endemic,” explained Ibrahim Musa, a Nigerian medical expert based at Kano general hospital.

Carriers of sickle-cell are less prone to being infected with malaria, which attacks red blood cells. However, those with sickle-cell disease are more vulnerable to malaria because of their weakened health, experts say.

Although sickle-cell in infants is curable through bone marrow transplants, lack of expertise and the high cost of the operation makes preventive measures the best option, medical experts say.

“This is why we advocate genetic counselling by intending couples before marriage to determine the status of their genes”, Nigeria Sickle-cell Foundation’s Wali said.

“People should go for a genetic test in the same way they determine their HIV status before marriage as the most effective way to protect their children and curtail the disease”, he said.

Sickle-cell contributes to 9 percent of deaths in children under five in West Africa, and up to 16 percent in some countries. Sickle-cell has a heavy impact on children: malaria is the leading killer of under-fives in Africa.

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