This is a very common mis-conception. Sickle cell anaemia (sca) is not “only found in Black people”. White people in Greece, Sicily, Turkey, and their offspring around the world suffer from sickle cell anaemia (sca). In fact, the highest incidences of the sickle cell gene (S, for short) are not found in Africa at all; they are in India and Saudi Arabia [References 1 to 10].

A common mis-conception. A dangerous misconception. People with sickle cell anaemia do suffer from malaria, and very badly too. Doctors who have been wrongly taught have been known to advise their sickle cell anaemia patients travelling from Europe to the Tropics not to bother taking anti-malarial tablets because the sickle cells make them immune to the parasite. Dangerous advice, as malaria is the commonest cause of sickle cell crisis in Africa [ See also Question 8. References 9, 11 to 14, 21 & 22].

Inadequate knowledge, or plain ignorance is the simple answer. I repeat: malaria affects sickle cell anaemia patients more seriously than it does others. A sickle cell anaemia child is one who has inherited sickle cell genes from both parents [S from father, and S from mother] producing the phenotype SS, which I have come to call ACHEACHE, that is one ACHE gene from father, and the other ACHE gene from mother. No one aches in the rainy season with just one ACHE gene. To suffer from cold season rheumatism, there must be contributions of ACHE from both parents. A child inheriting a sickle cell gene [S] from just one parent, and a normal haemoglobin gene [A] from the other parent is called Sickle Cell Trait [AS phenotype], which for simplicity sake I have come to call NORMACHE. This AS child does not have sickle cell anaemia SS, and reacts to malaria differently.

Even those Science teachers who know this difference between sickle cell anaemia [2 haemoglobin S genes that is ACHEACHE] and sickle cell trait [1 haemoglobin S gene, that is NORMACHE] teach, wrongly, that the AS phenotype (the NORMACHE) is immune to malaria. It certainly is not! Professor Konotey-Ahulu’s dear mother was for ever suffering from malaria until her death aged 89 from something else, and she was Sickle Cell Trait (NORMACHE). But (and this is where the confusion arises) the sickle cell trait (NORMACHE) child before the age of 4 years, withstands better the lethal effects of malignant malaria (ie Plasmodium falciparum ) than either the sickle cell anaemia ACHEACHE child [SS] or the child with normal haemoglobin genes from both parents [AA, that is NORMNORM]. None of the 3 phenotypes is immune to malaria; they all are infected by the parasite, but whereas the NORMNORM AA and ACHEACHE SS child can succumb to severe Falciparum malaria before the age of 4 years of life, the NORMACHE AS child is not prostrated by the disease although some malaria parasites are found in the blood. [References 9, 10 to 18, 20 & 21]. After the age of 4 both NORMACHE and NORMNORM living in the malarious region acquire some immunity in adulthood, but they still suffer from malaria which seldom kills native adults living in the malarious region, unless of course the adult has a double ACHE SS.  The ACHEACHE SS people are known to shrink their useful spleen in adulthood, and because the spleen is needed to remove malarial parasites from the blood, these ACHEACHE (SS) people continue to be prone to severe malarial attacks. All visitors to this website are advised to read this answer to Question 3 over and over again until they are capable of explaining it even to their own doctors. One of Konotey-Ahulu’s   publications in the British Medical Journal described a UK General Practitioner who advised a west African ACHEACHE SS lady going on holiday in the tropics not to bother taking anti-malarial protection, because (as the GP put it) “one ‘S’ protects against malaria, so the ‘SS’ that you possess doubly protects you”. When the lady returned from West Africa with fever and yellow eyes doctors refused to think of malaria, let alone test for it, but blamed everything on her sickle cell anaemia (SS). She died!  

Let the matter be summarized here again: As far as Adult Haemoglobin Types are concerned when dealing with malaria, there are 3 broad phenotypes in adults – (i) ‘AA’ (NORMNORM), (ii) ‘SS’ (ACHEACHE – sufferers of the cold season rheumatism called Sickle Cell Anaemia, and known over the centuries by  their African names, for example Chwechweechwe in the Ga language of Ghana), and (iii) ‘AS’ (NORMACHE known as Sickle Cell Trait). Malaria does kill both the AA NORMNORM child and the SS ACHEACHE child before the age of 4 years when general immunity strengthens, unless they are given prophylactic drugs. The ‘AS’, one ACHE gene plus one NORM gene, manages to survive childhood better than either the ‘AA’ NORMNORM phenotype or the ‘SS’ ACHEACHE phenotype. Because the one ACHE one NORM (Sickle Cell Trait) children possess this survival-in-childhood advantage doctors who should have known the difference between Sickle Cell Trait (One S gene one ‘A’ gene) and Sickle Cell Anaemia (2 ‘S’ genes) but who do not concentrate, take a cerebral short cut and say things like “Anyone with sickle cells is protected against malaria”. One hears this on the radio all the time. People who are reading this material need to help not only their doctors to understand this properly, but also lay people. How to help your doctor is to tell her/him before they ask you any question: “I have Sickle Cell Anaemia, ‘SS’”, or “I have been found to have the Sickle Cell Trait, ‘AS’ which does not cause cold season rheumatism.”.  As far as many doctors are concerned when a person tests ‘POSITIVE’ for sickle cells then that person must be sick. Nothing can be further from the truth. To test ‘POSITIVE’ for sickle cells does not distinguish the one ACHE Sickle Cell Trait (‘AS’)from the 2 ACHE Sickle Anaemia (‘SS’). Another test called Haemoglobin Electrophoresis needs to be done to distinguish the one ACHE one NORM Sickle Cell Trait from the double ACHE ‘SS’ Sickle Cell Anaemia. Please read that again, because Sickle Cell Traits (One sickle gene ‘S’ plus one normal adult Haemoglobin ‘A’ gene ie ‘AS’ combination or NORMACHE) have run in the Olympic Games at Mexico City where the oxygen level is thin at more than 7000 feet, and beaten the whole world to get GOLD MEDALS.  How can such ‘AS’ people be described as sick? Indeed, 1 in 3 of healthy northern Nigerians are NORMACHE (‘AS’), and 1 in 5 healthy southern Ghanaians are also ‘AS’ NORMACHE.
Finally, Haemoglobin ‘A’ (Normal Adult Haemoglobin) has nothing to do with Blood Group A. Always refer to the adult NORM Haemoglobin ‘A’, as ‘Haemoglobin Type A’, not Blood Group A. Please read that again! In Sickle Cell Trait (‘AS’ NORMACHE) the adult ‘A’ Haemoglobin is always a greater percentage fraction than the ‘S’ haemoglobin. If the proportions are reversed so that ‘S’ is say 62% and ‘A’ 35% then that is NOT Sickle Cell Trait, but Sickle Cell Disease [See reference Konotey-Ahulu FID and Ringelhann B (British Medical Journal 1969 in the Publications section].