WHAT’S ALL THE FUSS ABOUT?
According to The World Health Organisation (WHO), each year about 300 000 infants are born with major haemoglobin disorders – including more than 200 000 cases of sickle-cell anaemia in Africa. Globally, there are more carriers (i.e. healthy people who have inherited only one mutant gene from one parent) of thalassaemia than of sickle-cell anaemia, but the high frequency of the sickle-cell gene in certain areas leads to a high rate of affected newborns. Sickle-cell anaemia is particularly common among people whose ancestors come from sub-Saharan Africa, India, Saudi Arabia and Mediterranean countries. Migration raised the frequency of the gene in the American continent. In some areas of sub-Saharan Africa, up to 2% of all children are born with the condition. (WHO, 2006)
In most countries where sickle-cell anaemia is a major public health concern, its management has remained inadequate, national control programmes do not exist, the basic facilities to manage the patients are usually absent, systematic screening is not a common practice and the diagnosis is usually made when a patient presents with a severe complication. Simple, cheap and very cost-effective procedures such as the use of penicillin to prevent infections are not widely available in many countries. The most important challenge is, thus, to improve the prospects for the patients with sickle-cell anaemia in developing countries like Sierra Leone by providing support to their carers. About 70% of all sickle cell anaemia (SCA) subjects reside in Africa, estimated at over 12 million. The prevalence of SCA is estimated at over 2% while infant mortality is about 8% and survival rate of SCA babies in rural areas by five years of age is about 20%. These statistics indicate that SCA is probably the most neglected (and sometimes forgotten by health authorities) serious public health disorder with serious mortality and morbidity rates in Africa (United Nations Economic Commission for Africa, March, 2008). Despite this, it has not been given the attention it requires in Sierra Leone.