G6PD Deficiency in Ghanaians: How to recognise it

G6PD Deficiency in Ghanaians: How to recognise it  

In their instructive seminar Professors Capellini and Fiorelli put Africa first in the list of areas with the “highest frequencies of G6PD deficiency” (Jan 5, p 64) [1], so what are the Ghanaian associations? (i) “Dorkita, I’m passing coca cola urine”. Mist alba, septrin, fansidar, chloramphenicol, APC, are the greatest offenders [2]. (ii) Typhoid disease [3 4] (iii) lobar pneumonia with jaundice [2 4] (iv) Renal failure [5](v) No enzyme-at-all in 5% G-6-P-D deficient males [6](vi) Greater delay in recovery from coma [7] (vii) Greater representation in Cirrhosis of the liver [7] (viii) Greater proportion of diabetics [7] (ix) Sickle cell disease patients fare worse [8 9 10] (x) Female homozygotes (XX) have more severe disease than hemizygotes (XY), making me wonder “how that can be reconciled with the Lyon hypothesis of inactivation of one X chromosome” [10, page 105] (xi) Periodic intravascular haemolysis from interaction between “alpha thalassaemia type 1 equivalent to African homozygous alpha thalassaemia type 2, with G-6PD Total deficiency” [11]. Exercise acts as a trigger. The combination per se does not appear to account for the hyperbilirubinaemia in babies [All cases of ‘march haemoglobinuria’ must be screened for both alpha thalassaemia and G-6PD deficiency]. (xii) The enzyme is found in liver, brain, kidney, adrenals, skin, pancreas, nerve and muscle, hence the extra-erythrocytic manifestations of G6PD deficiency. Bedu-Addo’s description of chloroquine induced bilateral ptosis [12] could have been in one with no enzyme. (xiii) Hepatomegaly with quick progression to cirrhosis [7]. (xiv) Viral Hepatitis is commoner in G6PD deficient patients, and characterised by intrahepatic cholestasis [13]. (xv) Terminology of A-minus (attributed to Africans) and B-minus (Mediterranean)is meaningless, as in A-minus Ghanaians “absence of enzyme in new red cells” produces cases “similar to the Mediterranean type of total deficiency” [6] (xvi) There is an inexplicable north-south divide of incidence: 11% of males are deficient in the north [14] while 23% have G6PD deficiency in the south [15].  (xvii) Protection against malaria has not been proved in Ghana for hemizygotes and female homozygotes [7]. Indeed, blackwater fever is often related to G6PDdeficiency (xviii) “Sabolaa  Emanbii yi” is a truism in my Krobo tribe: “Onions and M&B disagree” [16]. In the Colonial days some who took sulphonamides (‘M&B’) for infections fell gravely ill on eating onions. Dipropyl disulphide in onions “alters G6PD in the metabolic chain within the erythrocytes, which causes denaturing and precipitation of haemoglobin” [17]. (xix) Genetic Counselling goes beyond haemoglobinopathy to erythrocytopathy. A G6PD deficient sickle cell trait mother (AS) has two healthy daughters with her sickle cell trait (AS) husband, and they seek advice for a third pregnancy hoping to get a boy. She is told that although neither daughter has inherited her deficiency, and both avoided sickle cell anaemia (SS), her next child could be SS with G6PD deficiency (severe if a boy) [18] (xx) Voluntary family size limitation (VFSL) [19] is the advice I give hemizygotes who (in Africa) have a high male procreative superiority index (MPSI) [20] in that males have more children than females, with consequent greater donation of abnormal genes to the next generation.         


I declare that I have no conflict of interest


Felix I D Konotey-Ahulu




10 Harley Street, London W1G 9PF, UK


1     Capellini MD, Fiorelli G. Glucose-6-phosphate dehydrogenase deficiency. Lancet 2008; 371: 64-74.


2     Owusu SK. Glucose-6-phosphate dehydrogenase (G-6PD) deficiency in the causation of disease in

       Ghana. Ghana  Med J 1974; 13: 168-170.


3        Owusu SK, Foli AK, Konotey-Ahulu FID, Janosi M. Frequency of Glucose-6-phosphate dehydrogenase deficiency in typhoid fever in Ghana. Lancet 1972; 1: 320.  


4    Adu D, Anim-Addo Y, Foli AK, Yeboah ED, Quartey JKM. Acute renal failure and typhoid fever. Ghana  

      Medical Journal 1975; 14: 172-174.


5     Owusu SK, Addy JH, Foli AK, Janosi M, Konotey-Ahulu FID, Larbi EB. Acute reversible renal failure

       associated with glucose-6-phosphate dehydrogenase deficiency. Lancet 1972; 1: 1255-1257 


6     Owusu SK. Absence of glucose-6-phosphate dehydrogenase in red cells of an African. BMJ  1972; 4: 



7   Owusu SK. Clinical manifestations of glucose-6-phosphate dehydrogenase (G-6PD) deficiency      

       in Ghana. Ghana Med J 1978; 17: 235-39.  


8   Konotey-Ahulu FID. Glucose-6-phosphate dehydrogenase deficiency and sickle cell anaemia. New Eng J

     Med 1972: 287: 887-888.


9   Acquaye CTA, Gbedemah KA, Konotey-Ahulu FID. Glucose-6-pogosphate dehydrogenase deficiency

      incidence in sickle cell disease patients in Accra. Ghana Med J 1977; 16: 4-7


10      Konotey-Ahulu FID. The sickle cell disease patient: natural history from a clinico-epidemiological study   

        of 1550 patients of Korle Bu Hospital Sickle Cell Clinic. London: Macmillan 1992; Watford: Tetteh 

                A’Domeno Co 1996.   


        11   Konotey-Ahulu FID. Alpha thalassaemia nomenclature and abnormal haemoglobins. Lancet 1984; 1:



        12   Bedu-Addo G. Chloroquine induced bilateral ptosis. Trans Roy Soc Trop Med Hyg 2006;

                100: 696-697. 


13      Morrow RH, Smetana HF, Sai FT, Edgecomb JH. Unusual features of viral hepatitis in Accra, Ghana. 

      Ann Intern Med 1968; 68: 1250-1264.


         14   Ringelhann B, Dodu SRA, Konotey-Ahulu FID, Lehmann H. A survey for haemoglobin variants,

    thalassaemia and Glucose-6-phosphate dehydrogenase deficiency in northern Ghana. Ghana Med J 

              1968; 7: 120-124.


15      Owusu SK, Opare-Mante A. Electrophoretic characterization of glucose-6-phosphate dehydrogenase (G6PD) enzyme in Ghana.  Ghana Medical Journal 1972; 11: 304.


        16   Konotey-Ahulu FID. Probing anecdotes in traditional African therapeutics. African Journal of Health  

               Sciences 1194; 1: 53-56.


17      Fenwick G, Hanley AB. The genus Allium – Part 3 Section X Medicinal Effects. CRC Critical Reviews

                  in Food Science and Nutrition 1985; 23: 1-73.


18      Konotey-Ahulu FID. Missing the wood for one genetic tree? In, The First International Symposium on

       the Role of Recombinant DNA in Genetics. Proceedings, Chanai, Crete – Greece, May 13 -16, 1985. Eds

       Loukopoulos D, Teplitz R. Athens, P Paschalidis 1986, pages 105-116.


19      Konotey-Ahulu FID.  The male procreative superiority index (MPSI): its relevance to genetical

                 counselling in Africa. In: Eds, Oliver Mayo, Carolyn Leach. Fifty Years of Human Genetics. A

                 Festschrift and liber amicorum to celebrate the life and work of George Robert Fraser. South Australia,

                 Wakefield Press, August 2007 pages 48-50.


         20    Konotey-Ahulu FID. Need for ethnic experts to tackle genetic public health. Lancet 2007; 370: 1826-27.   

Ethnic minorities and sickle cell disease

Ethnic minorities and sickle cell disease

SIR,-Dr John Black's article (30 March,p 984) is most helpful.
Many family doctors are quite familiar with much of what he has so
lucidly stated. The reason why many children (and adults) with sickle cell disease continue to die “before their time” in the UK and elsewhere is failure to appreciate certain facts relating to presentation, a failure of anticipation, and what I usually call a combination of circumstances.

Firstly, presentation. Epistaxis, priapism, enuresis, numbness of the lower lip, sudden aphasia, hemiplegia, blood in the urine, deep jaundice, large tummy, nails white as a sheet, loud precordial murmurs, bruit de diable at the root of the neck, unexplained fever, cough with tachypnoea, fatigability, swollen hands may each and severally be the first indication that the child has sickle cell disease.'

Alternatively, a child's condition may have nothing to do with sickle cell disease but may later be worsened by it-for example, within seven days lobar pneumonia can become pneumonia plus infarction plus pulmonary abscess.' Presentations may also differ from those described in the textbooks.

(1) Teenagers of 12, 13, 14, or 15 years with SS disease may have spleens which, far from vanishing, are enlarged.
(2) The child with genuine SS disease (parents AS, AS) with haemoglobin of 12-3 g/dl should not be treated as having sickle cell trait, because the potential for devastating in vivo sickling is there.

(3) Extreme fatigability with “nails as white as a sheet” during an attack of measles indicates an aplastic crisis from the measles virus.

Haemoglobin drops to 2-5 g/dl within 48 hours. As little as 100-150 ml of packed cells for a 5 year old child and not more than one unit for a 15 year old given over eight hours is lifesaving, even when the haemoglobin was less than 3 g/dl to start with.

Secondly, clinicians should anticipate disasters in patients with sickle cell disease. For example, if perioperative fluid therapy is not planned for a child about to undergo surgery the child will end up with a stroke. Anaesthetists are now more careful than ever so their success is virtually 100″,,, but fluid management before, during, and after operation often leaves a lot to be desired. The clinician should also be aware that the patient who has been admitted and discharged twice within the past week has come in again to die unless the whole management is overhauled.

Thirdly, there are many dangerous combinations of circumstances that may lead to disaster for the unwary.

(1) Personnel: the houseman or GP is away for the weekend; the locum has little clue about what is happening, and goes by the packed cell volume or haemoglobin concentration rather than the patient's condition; the casualty department may refuse to admit the patient, quibbling over whether the patient should go under a physician or a haematologist.

(2) Clinical: abdominal pain might be due to sickle cell crisis,2 3 or the crisis could also have been precipitated by acute appendicitis.'

(3) Genetic: hardly any west African with sickle cell anaemia is without one or more of the other hereditary erythrocytopathies: 86'U/ have concomitant lac or 2ac thalassaemia, and, although this is supposed to ameliorate the SS phenotype, 2OO, of the men and 16″(, of the women also have glucose-6-phosphate dehydrogenase (G6PD) deficiency.4 Contrary to what is claimed from the USA,G6PD deficiency on top of sickle cell disease is an added liability4 (Luzzato L, paper at International Symposium on Sickle Cell Anaemia, Abidjan, Ivory Coast, 1975).

Workers in the UK should always identify which of their patients have G6PD deficiency, because several antimicrobial agents and analgesics which are given to help may in fact harm the patient.


1 Konotey-Ahulu FID. The sickle cell diseases. Clinical manifestations including the “sickle crisis.” Arch Intern Med 1974;133:611-19.

2 Hendrickse RG. Sickle cell anaemia in Nigerian children. Cent AfrJa Med 1960;6:45-57.

3 Valman HB. ABC of I to 7. London: British Medical journal, 1982:21.

4 Acquaye CTA, Gbedemah KA, Konotey-Ahulu FID. Glucose-6 phosphate dehydrogenase deficiency in sickle cell disease patients in Accra. Ghana Med 7


Source : British Medical Journal 1985 (20 April), Volume 290, page 1214.

Home haemodialysis just the thing for Africa’s wealthy patients

Thanks to Dr Geraint James who introduced me to his wife Professor Sheila Sherlock in the early 1960's, I became one of her research fellows on Stanley Shaldon's famous Kidney Unit at the Royal Free Hospital School of Medicine [1]. Working around the clock sometimes, Stanley Shaldon's unit was the first in Europe to introduce home haemodialysis. If, as Professor Christopher Blagg states (5 Jan) “By 1971 58.8% of patients on dialysis in the UK received dialysis at home” [2] this was mainly due to Stanley Shaldon's extraordinary drive [3].

As Ken Farrington pointed out “self supervised haemodialysis performed in the patient's home was pioneered ..largely to cope with increasing numbers” [4]. In the tropics, especially west Africa, many deaths from renal failure are an every day occurrence. Only the very wealthy can extend their lives with renal failure, for only they can afford to run generators with uninterrupted electricity in their homes. I would have liked to see some costing in Professor Blagg's editorial [2].

When Ghanaians woke to the fact that Kwame Nkrumah's “Free Health Care for everybody” was no longer an option, and the government haemodialysis centres collapsed, it became obvious that only the very wealthy could survive end stage renal failure. Not so in the UK and USA, where the poor appear to be able to benefit from expensive health care delivery programmes.

The wearable haemodialysis device recently described by Andrew Davenport and colleagues [5] would be ideal for the African situation, where ambulant patients can just switch on the system when ominous symptoms after straying from prescribed dietary requirements begin to appear [6]. But how much would the wealthy African be expected to set aside for this?

Ghana has succeeded in introducing a National Health Insurance Policy, but because of the sheer numbers involved, it is incapable of covering haemodialysis whether in hospital, at home, or on foot.

Competing interests: None


1 Konotey-Ahulu FID, Baillod RA, Comty CM, Heron JR, Shaldon S, Thomas PK. Effect of periodic dialysis on the peripheral neuropathy of end -stage renal failure. BMJ 1965; 2: 1212-1215.

2 Blagg CR. Haemodialysis. Wide variations in availability exist, and the UK lags behind some other countries. BMJ 2008; 336: 3-4 (Editorial 5 January)

3 Baillod RA, Comty CM, Ilahi M, Konotey-Ahulu FID, Sevitt L, Shaldon S. Overnight haemodialysis in the home. Proc Eur Dial Transplant Assoc 1966; 2: 99

4 Farrington K. Modality selection and patient outcome. In Akoh JA, Hakim N S (Eds) Dialysis Access – Current Practice, London: Imperial College Press 2001, pp 23-47.

5 Davenport A, Gura V, Ronco C, Beizai M, Ezon C, Rambad E. A wearable haemodialysis device for patients with end-stage renal failure: a pilot study. Lancet 2007; 370: 2005-2010 (Dec 15).

6 Konotey-Ahulu FID, Anderson G. Treatment of hyperkalaemic cardiac arrest by timely haemodialysis. Ghana Medical Journal 1965; 4: 158-163.

Published in the British Medical Journal on line 10 Jan 2008 http://www.bmj.com/chi/eletters/336/7634/3

Competing interests: None declared

Some thirty features of AIDS in Africa

Some thirty features of AIDS in Africa




In 1987 I visited sixteen African countries to acquaint myself with the AIDS situation on the continent. I obtained information from doctors and health workers about many of the countries I could not visit. 1 was refused a visa to go to Zaire.


A synoptic overview of clinical and other features of AIDS in Africa as I learnt on my sub-Saharan tour is here presented, making mention of some of my teachers. Those not referred to are being protected because the authorities forbade them to take any foreigner on a ward round. What I learnt from the prostitutes is to be published elsewhere.


Aids is not uniform over the 50 countries in Africa. In most it is now in the introductory phase. In 5 or 6 countries AIDS is in the propagation phase with the highest incidence in some French speaking (but not necessarily French related) regions and countries bordering them.


Age block gap. No patients were found between infancy and teens except the blood trans-fused, thus excluding insect vectors in transmission (Dr. Miriam Duggan and Dr. Sewankambo of Uganda, Professor McLarty. Tanzania; Dr. Fleming.  Zambia.)


Repatriation AIDS. In my Krobo tribe in Ghana, all patients had been sent home to die from Ivory Coast (1). Most of West Africa is like that.


100 % Female preponderance. In certain tribes in West Africa males have yet to manifest the disease (1-4).


Perineal devastation easily visible from the foot of the bed with undressed patient lying prone (Dr. Mate-Kole, Korle Bu Teaching Hospital, Ghana.)


Virgins and the nulliparous can get AIDS from the first intercourse due to tears (Dr. Mate-Kole, Korle Bu Teaching Hospital, Ghana).


Pervisemos i.e. ‘persistent virus secreting mothers’ who are asymptomatic but continue to bring forth sick children (Dr. Duggan and Dr. Hanny Friesen, Kampala ; Dr. Chintu, Lusaka).


AIDS Precipitators. Caesarian section and minor procedures like salpingohistograms can turn the asymptomatic into full blown AIDS (Dr. Duggan, Kampala.) (5).


CromwellHospital, Cromwell Road, London SW5 OTLI.

Former Director Ghana Institute of Clinical Genetics, and Consultant Physician, Korle Bu Teaching Hospital, Accra.

*Published in: Annales Universitaires des Sciences de la Santé 1987; 4 (4): 541-544.


Symptomatology of Slim : 20-40 % weight loss, persistent diarrhoea, fever, lymphadenopathy, respiratory symptoms, oral candidiasis and amenorrhoea in child bearing women, with frequent previous history of sexual exposure, of blood transfusion, and/or unsupervised injections (Dr. Sezi, Serwadda & colleagues in Kampala, physicians in Dar es Salaam, and in Lusaka and Ndola, Zambia, Dr. Neequaye et al, Ghana) (6, 7, 8).


Intractable Pruritus in adults, and in infants : this could be the commonest cause of

 insomnia (Dr. Chintu and Dr Subhash Hira, Lusaka.)


Generalised hyperpigmentation with crazy-pavement dermatopathy (Professor. Bodo, Nairobi). Papulo-vesicular eruption rather like chicken pox (Dr. Sezi, Kampala).


Dupuytren's Contracture (Professors Badoe, Archampong and Jaja's new book

“Surgery in the Tropics” p.210 shows this physical sign as a complication of plaque Kaposi's sarcoma) (9). Professor Anne Bayley (Lusaka) showed me two cases of aggresive atypical Kaposi’s sarcoma (AAKS) with this sign.


Elephantiasis of limbs (upper and/or lower) and genitals from AAKS (Professors Bugingo, Rwanda and Anne Bayley, Zambia).


Multidermatomal Herpes Zoster heralds full blown AIDS (Dr. Subhash Hira, Zambia and Dr. Sezi, Uganda).


Adult Kwashiorkor. I saw this syndrome in my Krobo tribe where girls with Repatriation AIDS whose diarrhoea must have included creatorhoea with consequent protein calorie malnutrition.


Accelerated orphan Kwashiorkor. 1 saw this at Dodowa, Ghana, in a baby boy whose mother had died a week after repatriation from Ivory Coast.


Tuberculous pericarditis as a common complication (Dr. Mboussa, Brazzaville and Dr Jahazi, Dares Salaam).


Non-AIDS Diseases producing HIVseropositivity. (Dr. Fleming and Rosemary Mwendapole, Ndola, Zambia) (10). Liver pathology can confuse results and Tanzanian physician  Professor Aaron Massawe postulates “immunoligical turbulence” with

Anti-TB treatment to fake seropositivity.


Radiological “bat's wing” lung in AAKS (Professors Bugingo Rwanda, and Anne Bayley, Zambia) (11)


Sworl Facies: a characteristic “Strikingly worried look”, on the faces of the more discerning patients I visited on ward rounds in Uganda, Rwanda, and Zambia.


Relative Paucity of full blown AIDS. It came as a surprise to find a Zairean man and wife, and a Kenyan itinerant salesman as the only AIDS patients in the 2100-bed KenyattaNationalHospital. Even in Uganda, Rwanda, and Burundi, wards were not overflowing with patients. I entirely agree with Professor Gottlieb Monekossoo, Director of the WHO's Regional Office for Africa when he is reported by The New Scientist as saying: “For many countries in Africa AIDS does not represent the same threat that it does in Europe. In the eyes of health managers AIDS probably ranks only tenth or lower on a list of serious tropical diseases. Malaria, measles, diarrhoeal illnesses, tuberculosis, cholera, meningitis, yellow fever and various cancers account for more deaths and illnesses than AIDS does, at the moment” (12).


Patients are not dying “like flies” as world media report (13). When Uganda's Dr. Sewankambo was recently asked in London what proportion of a hundred gravely ill patients for admission would be AIDS and he replied two, or at most three at the worst times”, he was glared at with incredulity.


Seropositive twin baby lives while seronegative twin dies. Born to a pervisemo (ie persistent virus secreting mother) the infected twin lived while the seronegative twin died from AIDS, in Kigali, Rwanda.


AIDS has not changed health priorities in Africa. I cannot speak for Zaire where I was not permitted to visit, but in no country has AIDS moved into the first 6 health priorities, even in Rwanda, Burundi, Zambia, and Tanzania.


Disagreement about seriousness of the problem. Some expatriate workers in Africa

prophesy doom, but most indigenous doctors while not underestimating the gravity in some countries, consider forecasts exaggerated (Uganda, Rwanda, Burundi, Zambia). I myself have judged the gravity of AIDS in Africa at 5 clinically graded levels.

Grade I, not much of a problem; Grade II, a problem exists; Grade III, a great problem;

Grade IV,  an extremely great problem, and Grade V, a catastrophe (13). I recommend     this approach to health workers and urge them to have their own grading criteria. Clinicoepidemiology rather than seroepidemiology will best bring out the truth about the real state of affairs of each country (1).


The Juliana Phenomenon. AIDS in the lake region of Tanzania, bordering Zaire, is known as “Juliana” because, as one prostitute told me,  “A few years ago when the Navy visited Mombasa with 9, 000 troops, some of our girls who travelled there for business were given T shirts with  Juliana marked on them. Many of those who wore the Juliana shirts have since had Slim and died”.



Non-Africans with AIDS. The 6 patients seen in Mombasa with AIDS (1983-1987) by a specialist, were a Zairean, and 5 non-Africans from Europe and the USA; in South Africa all the AIDS has so far occured in non-blacks (Dec 1987), and in Zaire at least 21 Europeans and Americans were known to have had AIDS (Source : Resident Greek Businessman). HIV-2 in West Africa is not specifically African, having been seen in two homosexual men in France (14) and is now known to have Portuguese connections. (15).


Complete Cure Anecdotes were heard in Uganda, Rwanda, Congo Brazzaville (related to tuberculosis) (1), Tanzania, and herbal preparations are being tried in domiciliary management of the disease in Ghana. (16).




It is important that doctors living and working in Africa adopt their own approach to a new disease like AIDS, and not import wholemeal terminologies, diagnostic criteria, and preventive slogans from abroad. Africa in my opinion should abandon the use of “homosexual”, “heterosexual”, “bisexual” etc., and should call a spade a spade. African prostitutes are said to be “heterosexual” but I met girls of whom anal intercourse was demanded by some expatriates for extra money (4), and in Burundi I recently asked a prostitute, “Y-a-t-il quelqu’un qui vous a demande de faire l`amour dans la bouche ?”

And she replied, “Oui Monsieur, mais je leur demande une grande somme d'argent”

 (17). So, one should now use “peno-vaginal sex” for so called heterosexual sex, and “anal sex”or “sodomy” for what is called “homosexual relationship”.  Anal sex has been demanded sometimes for money  in several countries in Africa. And in the first description of the AIDS problem to come from Africa one AIDS patient had a “high recto-vaginal fistula of recent onset” (6), while the Ugandan traders who were found to be seropositive admitted to, “both heterosexual and homosexual casual contacts”(6). It is far better perhaps to say that these traders admitted to both peno-vaginal and anal intercourse (4). As regards diagnosing AIDS without blood tests the Muhimbili Criteria show that one does not have to use criteria from abroad (18).


Finally the kind of research that will help Africans curtail AIDS does not have to be the vaccine orientated research of the developed countries. Public Health methods and clinical epidemiology are Africa's best tools (1).




I thank the clinicians who took me on ward rounds during my recent Africa tour, and the Health Administrators who readily agreed to see me.




1. KONOTEY-AHULU F I D, (1987) Clinical epidemiology, not seroepidemiology, is the answer to Africa's AIDS problem. British Medical Journal 294: 1593

2. NEEQUAYE A.R., NEEQUAYE  J,., MINGLE J.A., OFORI-ADJEl D. (1986). Propondernace of females with AIDS in Ghana. Lancet ii: 978

3. NEEQUAYE A. R., ANKRA-BADU G.A., AFRAM R.A. (1987). Clinical features of human immunodeficiency virus (HIV) infection in Accra. Ghana Medical Journal 21: 3-6

4. KONOTEY-AHULU FID (1987) AIDS: origin, transmission and moral dilemmas. Journal of the Royal Society of Medicine 80: 720.

5 KONOTEY-AHULU FID (1987). Surgery and risk of AIDS in HIV positive patients. Lancet ii: 1146


CARSWELL J. W, KIRYA G.B., BAYLEY A.C., DOWNING R.G., TEDDER R.S., CLAYDEN S.A., WEISS R.A., DALGLEISH A.G. (1985). Slim disease: a new disease in Uganda and its association with HTLV-111 infection. Lancet ii: 849


 An endoscopic, histological and microbiological study. AIDS 1:9


 DOWNING R.G., LUCAS S.(1987) HIV Infection through normal heterosexual contact in Uganda. AIDS 1 :113

9. BADOE E.A., ARCHAMPONG  E.Q., JAJA M., Eds, Principles and Practice of Surgery in the Tropics. Accra: Ghana Publishing Co; 1986

10. FLEMING A.F., KAZI A.R., SCHEINEDER J., GUILLOT F., MWENDAPOLE R., WENDLER I., HUNTSMANN G. (1986). Comparison of HTLV-111 in some Zambian patients. AIDS Forschung (AIFO) 8: 434.

11. BAYLEY C A. (1983). Aggressive Kaposi’s sarcoma in Zambia. Lancet i: 1318-1320

12. MONEKOSO G. In Second International Conference on AIDS in Africa. Naples October 1987. Interview with Sharon Kingman. New Scientist, 15th October 1987, p 26.

13.KONOTEY-AHULU F I D. (1987). AIDS in Africa: Misinformation and Disinformation. Lancet, ii: 206-207.

14. BRUCKER G, BRUN-VEZINET F, ROSENHEIM M, REY M A, KATLAMA C, GENTILINI M. (1987). HIV-2 in two homosexual men in France. Lancet, i: 223 

15. KINGMAN SHARON. (1987). The Portuguese connection. New Scientist, 15th October, p 27

16. QUARTEY J K M, MATE-KOLE M O, OKAI GLORIA, BENTSI CECILIA, DJABANOR F F T, KONOTEY-AHULU F I D. (1988). Domicilliary management and prognosis of AIDS in the Krobo region of South east Ghana. The First International Conference on the Global Impact of AIDS. Barbicon Centre, London, 8 – 10 March.

17. KONOTEY-AHULU F I D. Extensive palatal echymosis from felllatio – a note of caution with AIDS at large. (1987). British Journal of Sexual Medicine, 14: 286-287

18. PALLANGYO K J, MBAGA I M, MUGUSI F, MBENA E, MHALU F S, BREDBERG U, BIBERFIELD G. (1987). Clinical case definition of AIDS in African adults. Lancet, ii: 972.    

First published in Annales Universitaires des Sciences de la Santé 1987; 4: 541-544


Postscript January 2008: What has happened in Africa in the past 20 years since this article was published defies logic. Countries (like South Africa, Namibia, and Malawi) that have moved from my 1987 classification of Grade I (not much of a problem) to Grade IV, tottering on V (Catastrophe) in just two decades require more high quality epidemiological research like Guisselquist and colleagues’ [Reference i] and of Didier Fassin and Helen Schneider [Ref ii] to work out very clearly the way the epidemic is being propagated. It is impossible for sex alone to account for the Financial Times (London) statement that 85% of the inhabitants of Zevenfontein in South Africa are HIV-Positive [Ref iii]. We need a paradigm shift in our approach to the management of this epidemic in Africa [Ref iv].




(i)   Guisselquist D, Rothenberg R, Potterat J, Drucker E. HIV infections in sub-Saharan Africa not explained by sexual or vertical transmission. Internatrional Journal of HIV & AIDS Oct 2002: Vol 13: pages 657-666.

(ii)  Fassin D, Schneider H. The politics of AIDS in South Africa: beyond the controversies. British Medical Journal 2003; Vol 326: pages 495-497 (1 March)

(iii)  Financial Times. AIDS in South Africa. Friday, 20 September 2002, page 14.

(iv) Konotey-Ahulu FID. AIDS in South Africa: wake up call and need for paradigm shift. http://bmjjournals.com/cgi/eletters/326/7387/495#30917 April 3 2003.    

Stem Cells Cure Sickle Cell Anemia In Mice

Last week’s Scienceonline (December 6 2007) had a most instructive report by American scientists, Timothy Townes, Rudolph Jaenisch, Jacob Hanna, and others from the University of Alabama and Massachusetts Institute of Technology’s Molecular Biology Department. They proved that mice that had been genetically modified in the laboratory to develop sickle cell anaemia can be made to produce embryonic stem cells from cells collected in the skin of their tails. Embryonic stem cells are known to be capable in later life of developing into any of different tissues. These so-called induced pluripotent stem cells (IPS cells) were then made to produce healthy haemoglobin by replacing the defective beta-globin sickle cell gene (S) with normal adult gene producing haemoglobin-A (nothing to with Blood Group A!). These were then injected into similar mice with sickle cell anaemia and, lo and behold, normal adult haemoglobin was produced and the mice improved clinically.

            So far so good news, but the cautionary tale is this: it needs the help of retroviruses to shift the genes around to give the required result, and where did you last hear of retroviral activity? AIDS, of course and as one of the scientists put it: “We need a delivery system that does not integrate itself inside the genome. Retroviruses can disrupt genes that should not be disrupted or activate genes that should not be activated”.

            If skin cells of mice can be made to behave like embryonic cells which obey orders given them to produce this or that, the next question is this: “Can human skin cells from sickle cell anaemia patients be similarly programmed to this end?” We can’t wait, though we know that transplanting induced pluripotent cells carry a high cancer risk, not to mention the effect of the high dose of X-rays required to destroy the faulty blood cells in the body before the new ones are planted.

Two more comments:

(i) How much would a New Yorker pay for the procedure, if it proved successful in humans? Cost itself should not disqualify a process that would abolish pain, but one needs ask the question. Until persons producing inherited ‘ACHEACHE’ haemoglobin begin to be able to produce acquired ‘NORMNORM’ or ‘NORMACHE’ haemoglobin through IPS cell manipulation, we need to pursue the public health measures that I have been stressing for preventing crises, and we must continue urging on our kith and kin genetic counselling with voluntary family size limitation [Konotey-Ahulu FID, The Lancet, 1 December 2007, volume 370, pages 1826-27: “Need for ethnic experts to tackle genetic public health”]       

(ii) A report by one Eric Bender in Cambridge Massachusetts (December 6 2007) began like this: “Mice with a human sickle cell anemia disease trait have been treated successfully…..” Now those familiar with this website know that there is no such thing as “sickle cell anemia disease trait”. There just isn’t! While sickle cell anaemia is also sickle cell disease, sickle cell trait is NEITHER of these. Indeed, as the blog above shows, I was given four bodyguards in Philadelphia for stressing this very point, namely that SICKLE CELL TRAIT IS NOT SICKLE CELL ANAEMIA (SICKLE CELL DISEASE). Please read that blog above again, and turn to my FAQ [Frequently Asked Questions]. Even well educated professionals use the terms interchangeably. Remember this: Only sickle cell anaemia can be referred to as sickle cell disease because both have TWO ‘Ache’ genes. The Sickle Cell Trait has only one ‘Ache’ haemoglobin gene. Insurance companies have been known to blur the distinction and have over charged sickle cell traits (many of whom have won gold medals at Olympic Games, even at a height of 8,000 ft above sea level). How can persons who ran and beat the whole world be charged a greater premium for health insurance, than the people they beat at the games?   

Opiates for pain in dying patients and in those with sickle cell disease

Opiates for pain in dying patients and in those with sickle cell disease

Dorothy Logie and Mhoira Leng’s report of 6 October describes a conference in Nairobi which highlighted opiophobia – the fear of using morphine therapeutically – “as a big obstacle facing palliative care services in the (African) continent” [1]. The conference was concerned with dying patients. In his rapid response, however, Jecko Thachil concentrated on the use of opiates in sickle cell disease patients. He states that while “very high amounts of opiates (often in hundreds of milligrams) are often required for the patients who suffer from recurrent sickle crises” [2], the expected analgesic effect leaves much to be desired, not to mention side-effects. Dealing with terminally ill patients is one thing; opiate administration to sickle cell disease patients is something else entirely.

Dr Thachil feels an “increased need for additional education regarding sickle cell disease…” Has he examined the experiences of two physicians who personally supervised thousands of people with sickle cell disease on both sides of the Atlantic continuously for years? [3, 4] Treating sickle cell disease is not the same as managing the sickle cell disease patient [5]; the difference in the two approaches tending to separate haematologists on the one hand, from physicians and family practitioners on the other. When a white British consultant physician in a London teaching hospital complained that “the haematologists here have created a cohort of addicts” [6] a white consultant haematologist in another London teaching hospital tore strips off him, accusing him of being a racist for depriving suffering black patients of pain relief, an attack which made me respond thus: “White physicians who, at the risk of being misunderstood by (that haematologist), voice their displeasure at what they see happening on their wards deserve commendation, not condemnation” [7].

Not far from where Dr Jacko Thachil works, also on Merseyside, “during a ward round in a provincial teaching hospital with consultant haematologists on March 6, 1997, I was shown a woman who had been on continuous opiate infusion since September, 1996” [7]. I went on to say in my Lancet communication: “Far from the consultants taking umbrage because I pointed out that the patient could not have been in sickle cell crisis for 6 months, they were happy to discuss with me the way forward” [7]. Dr Thachil feels “an increased need for additional education regarding …addiction to pain medication…and treatment of pain” and he concludes “but who and where these should be focussed on is a matter for debate” [2].

Not a matter for debate at all in my opinion: May I suggest to him certain facts he might wish to probe in his quest for education?

(a) “In Jamaican experience ..morphia or its derivatives are rarely used or necessary” [8] How did Graham Serjeant achieve this?

(b) “Most painful crises may be treated in a day-care centre, the patient returning home in the evening” [9] How is this possible if hooked up on morphine or diamorphine pump “as in the recommended UK protocol”? [6]

(c) “We are convinced that the chest syndrome in the UK and the USA is not entirely unrelated to the routine use of opiates in those countries for sickle cell crises”. [10] Some nurses I am in touch with can write an MSc thesis on this.

(d) Goodman from the USA (where diamorphine is banned for patients) found the use of ketorolac in painful sickle cell crises as efficient as morphine but without the latter’s respiratory depression [11]. So why do British haematologists prefer to use morphine and diamorphine? Answer: “Ketorolac has no product licence in the UK for this indication” [12]

(e) Two questions that I have asked British Haematologists several times but which have never been answered, and which Dr Thachil may now ask the National Institute of Clinical Excellence (NICE) for help in answering:

(i) “Why do West African and West Indian patients with sickle cell disease who did without morphine in their countries have to be given morphine pumps during sickle cell crises when they come to the United Kingdom?” [6, 13]

(ii) If pain from whatever cause deserved the most potent analgesic, and dysmenorrhoea has been known to be intolerably painful, would a British haematologist “not consider it unwise for a hospital to administer diamorphine as routine management of young women?” [7]

Four encouraging signs have emerged in the UK since I have been voicing my displeasure at the use of diamorphine and morphine pumps for patients with sickle cell crises: (1) Some haematologists in the UK and the European continent have abandoned the practice in spite of what the ‘approved protocol’ displays in the emergency rooms. (2) Some sickle cell disease patients have become more vocal in their displeasure of the practice. To them ‘opiophobia’ is not to be condemned [1], but commended. (3) Some family practitioners are looking after their patients at home, using intravenous fluids and other than powerfully addictive opiates to help these patients instead of submitting them to hospital care. (4) A clearer grasp of the causes of crisis has put more emphasis on public health measures (fluids, warmth, treatment of infections, dressing properly, anticipating hazards, immunisations, avoiding tobacco and alcohol), enabling patients prevent crises and helping them use the excellent non-sickling genes they have inherited from their parents to achieve as much of their full potential intellectually as possible [10].

It is therefore not surprising to find that the sickle cell disease patients who have become lawyers, teachers, businessmen and women, nurses & midwives, pharmacists, and even doctors are those whose haematologists have abandoned the opiate culture. Occasionally, however, one found even professors of haematology who would defend the prescribing of diamorphine for a sickle cell disease patient with severe difficulty in breathing. “Chest syndrome” was always there to blame, if the patient died [6].

When in my genetic counselling and family size limitation (GCFSL) drive in Ghana and in the Ghanaian community here in the UK I sense that the urgency of my message is being glossed over I tell my fellow countrymen and women in plain language that if they continue to procreate at the rate they are doing, and more sickle cell disease (ACHE/ACHE) patients are born, the chances are that in the UK they may end up on a heroin drip. This concentrates the mind, and they listen to me. “One in three of you is a NORM/ACHE. Do you want to end up with ACHE/ACHE children who will be given heroin for pain?” [http://www.konotey- ahulu.com/diagram.asp]

Kwegyir Aggrey Distinguished Professor of Human Genetics, University of Cape Coast, Ghana and Consultant Physician Genetic Counsellor, Ten Harley Street, London W1N 1AA, England.

Conflict of interest: None declared

1 Logie D, Leng M. Africans die in pain because of fears of opiate addiction. BMJ 2007; 335: 685

2 Thachil J. The fear of opiate addiction – not unique to Africa. Rapid response BMJ 2007, 8 October.

3 Serjeant GR. Sickle cell disease. Oxford: Oxford university Press, 1992 (Second Edition)

4 Konotey-Ahulu FID. The sickle cell disease patient. London: Macmillan 1991; Waftord: Tetteh-A’Domeno Co, 1996.

5 Konotey-Ahulu FID. Sickle cell disease and the patient. Lancet 2005; 365: 382-383.

6 Konotey-Ahulu FID. Opiates for sickle cell crisis? Lancet 1998; 351: 1438.

7 Konotey-Ahulu FID. Opiates for sickle cell crisis. Lancet 1998; 352: 651-652.

8 Serjeant GR. Sickle cell disease. Oxford. Oxford University Press, 1985, page 204.

9 Serjeant GR. Sickle cell disease. Lancet 1997; 350: 725-730.

10 Ringelhann B, Konotey-Ahulu FID. Hemoglobinopathies and thalassaemias in Mediterranean areas and West Africa: historical and other perspectives 1910 to 1997. Atti dell’Accademia delle Scienze di Ferrara 1998; 74: 267-307.

11 Goodman E. Use of ketorolac in sickle cell disease and vaso- occlusive crisis. Lancet 1991; 338: 641-642.

12 Liesner RJ, Vandenberghe EA, Davies SC. Analgesics in sickle cell disease. Lancet 1993; 341: 188.

13 Konotey-Ahulu FID. Morphine for painful crises in sickle cell disease. BMJ 1991; 302: 1604.

Competing interests: None declared

Link : http://www.bmj.com/cgi/eletters/335/7622/685#177986

There is nothing mysterious about kwashiorkor

There is no mystery [1, 2] about “kwashiorkor”, which is a word from a Ghanaian tribe, my very own Krobo/Dangme/Ga megatribe [3, 4]. Indeed, it was when I was a toddler that the remarkable white woman based at the Princess Marie Louise Hospital in Accra (before the Gold Coast became Ghana) first described the condition which was brought to her attention by my fellow tribes folk [5,6].

Imagine my utter astonishment when, coming over to England to read Medicine in London University, I heard Lecturer after Lecturer state with unwarranted confidence that “kwashiorkor means red hair in an African language”. The word means no such thing in my tribe. Kwashiorkor, as I once pointed out to a British Professor of Paediatrics in this very medical journal “is, primarily, a sibling positional word, which requires careful explanation to the non-native” [3]. It is a reflection of the 'birth position' of the sufferer, before it is a pathology [4], a fact emphasized by Dr Cicely Willams whose descriptions of the condition have not been bettered by anyone [5, 6, 7, 8].

Apart from being a Krobo tribesman, I have a further reason for claiming that I know more about Kwashiorkor than all the non-Ghanaian experts that have written volumes about the syndrome (Cicely Williams excepted). And I say that for this reason: In my tribe, it was said of me (the second child) the day my younger brother was born while I was not yet completely weaned – “afor ese kwasiorkor” [3] which, literally means “it has been born after him kwasiorkor”, stressing my positional risk of being sandwiched between my elder brother, Agbetey, who was just 13 months older than me, and the just arrived sibling who was also just 17 months my junior. See http://www.konotey-ahulu.com/images/generation.jpg for the first 3 children of my parents Rev & Mrs Konotey-Ahulu in Generation VII, and note how closely spaced we were.

The whole tribe expected me to develop kwashiorkor because of the birth of my brother. “The reason I escaped the syndrome first described by Dr Cicely Williams [3, 4] was simply because my educated parents took the same steps that Professor Marsden described from Brazil [9]: I was fed with beans, eggs, milk, minced meat, and Ovaltine” [3]. Less fortunate relatives of my age in my tribe, whose parents also had 3 children in less than 3 years, “and of whom it was also said 'afor ese kwasiorkor' went on to develop kwashiorkor because they could only afford to be fed with maize products such as akasa and k enky” [3].

I went on to make the point that: “The sibling positional word became almost invariably associated with the syndrome Cicely Williams was investigating, and although the term kwashiorkor was occasionally attached to a child who never went on to get the disease ” (just as in my own case), whenever the the syndrome kwashiorkor was seen in a child there was, more likely than not, very close proximity to a younger sibling” [3]. Exceptions, of course, occurred, “when children were orphaned” (as would be the case in Malawi ravaged with AIDS), regardless of sibling positioning, or multiple births. Indeed, I described such children (with photographs) on my AIDS tour of African countries [Ref 10 – See Figure 6.14, page 124]. I also described in a man who was mistaken for suffering from HIV-AIDS [Ref 10, photographs on pages 80-82] what has been called the Adult Kwashiorkor syndrome “in which diarrhoea, pitting oedema, hepatomegaly, dermatopathy, mental apathy, and hair changes result from protein energy malnutrition as, for example, in the creatorrhoea and steatorrhoea of severe alcoholic chronic pancreatitis”. This syndrome was completely reversible in the man described [10] when his alcoholism [he was on one litre and half of gin a day] was treated, diabetes reversed, pancreatin supplied, and protein supplements given [See remarkable change in just 6 months – Ref 10]. Should we have spent research funds finding out how much antioxidants this man's diet contained?

I had the enormous privilege of meeting up with 93-year old Dame Cicely Williams in Oxford in 1986 when we were photographed together[See The Lancet, Ref 4 for what she looked like at 93!]. I thanked her profusely for all that she did in my tribe in those colonial days wihout electron microscopes and sophisticated analysers. We both were greatly baffled why many of today's experts (especially those who have little experience of a tropical sojourn)find it difficult to accept that “Kwashiorkor is the result of a social pathology before it is the outcome of a biochemical pathology” [4]. Clinical epidemiology ie answering the questions Who? Which? Where? When? Why? What? and How? is far and away the best tool to investigate a tropical phenomenon such as kwashiorkor, and Cicely Williams gets my Full Marks for employing that tool with hardly any funds for medical research.

I repeat what I said a decade or so ago in The Lancet: “Those of us who grew up in the kwashiorkor belt and who have also had the benefit of an excellent medical education cannot but caution our ministries of health and social welfare about the danger of missing the social pathology wood for the trees of free radicals and leukotrienes”.

Nothing to declare, except that Kwashiorkor is my tribal language.


1 Fuchs GJ. Antioxidants for children with kwashiorkor. Brit Med J 2005; 330: 1095-1096.

2 Ciliberto H, Ciliberto M, Briend A, Ashorn P, Bier D, Manary M. Antioxidant supplementation for the prevention of kwashiorkor in Malawian children: randomised, double blind, placebo controlled trial. Brit Med J 2005; 330: 1109-1111.

3 Konotey-Ahulu FID. Kwashiorkor. Brit Med J 1991; 302: 180-181.

4 Konotey-Ahulu FID. Issues in kwashiorkor. Lancet 1994; 343: 548.

5 Williams CD. A nutritional disease of childhood associated with a maize diet. Arch Dis Child 1933; 8: 423-8.

6 Williams CD. Kwashiorkor – a nutritional disease of children associated with a maize diet. Lancet 1935; 2: 1151-52.

7 Williams CD. Kwashiorkor. JAMA 1953; 1280-85.

8 Williams CD. Social Medicine in Developing Countries (Millroy Lecture, Royal College of Physicians). Lancet 1958; 863-66.

9 Marsden PD. Kwashiorkor. Brit Med J 1990; 301: 1036-37.

10 Konotey-Ahulu FID. What Is AIDS? T-A'D Co. Watford 1996.

Competing interests: None declared

Source Link as http://www.bmj.com/cgi/eletters/330/7500/1095#106854 14 May 2005

The perils of unmasking scientific truths

BMJ  2007;335:210-211 (28 July), doi:10.1136/bmj.39268.553021.47

Views & reviews

Personal views

The perils of unmasking scientific truths

Felix I D Konotey-Ahulu, consultant physician

London W1N 1AA


To be chosen to deliver the keynote address at the Martin LutherKing Jr Foundation's award banquet took me completely by surprise—andto find that four bodyguards had been assigned me shook me rigid.Nobel laureates Linus Pauling and Max Perutz, along with HermannLehmann, Roland Scott, A C Allison, Graham Serjeant, and I,were among a select few invited to Philadelphia to receive anaward “for outstanding research in sickle cell anaemia.” Butwhy was I asked to deliver the keynote address, with Paulingand other abnormal haemoglobin heavyweights on the platform?

Was it, perhaps, because a foundation commemorating a blackperson wanted to “show off” the only black African among thosereceiving the award? Was it, perhaps, because I was then directorof the largest sickle cell disease clinic in the world? Or wasit because I was the only person to have traced hereditary diseasein his forebears, with named patients, generation by generationback more than three centuries? Or was it the statement madea few weeks back in New York by Professor Helen Ranney of theAlbert Einstein University College of Medicine: “There is nosingle clinical experience in the United States comparable tothat of Dr Konotey-Ahulu”?

Such “perhaps hypotheses” competed in my brain when I arrivedin Philadelphia, that day in 1972. I walked out of the hotelto post a letter to my wife in Ghana. Just as I was about tocross a road, I heard a voice behind me: “Doctor! Doctor!! DoNOT cross that road. Where are you going?” The hugely builtAmerican (black) took the letter from me before dropping thebombshell: “I am one of your four bodyguards.”

The award organisers, who came within minutes of my call, explainedthat the text of my lecture alerted them to several problems.I had distinguished between sickle cell trait and sickle celldisease (sickle cell anaemia) because the terms were being usedinterchangeably, with disastrous consequences, by people whoshould know better. People with the trait (one abnormal gene)cope better than people with two normal genes with falciparummalaria, which kills sickle cell disease patients (two abnormalgenes) quicker than people with two normal genes. I had questionedpublished work which claimed that black Army recruits exercisingat an altitude of 4000 ft collapsed and died because of sicklecell trait. I had asked: “How could black sickle cell traitsrun and beat the whole world at the Olympic Games at MexicoCity, at an altitude of 8000 ft (double the altitude at whichpeople with sickle traits had been said to perish)?”

Why did I need four bodyguards? The award organisers thoughtI needed protection because I used data from an article by JamesBowman, who had named seven insurance companies in the UnitedStates which loaded the premium of black people with sicklecell trait, thus making lots of money on healthy people, whoneeded to pay 150% for health insurance. I explained that insouthern Ghana, where one person in five has the sickle celltrait, one in five sudden deaths in adults from whatever causewould be in people with sickle trait. Moreover, to make insurancerecommendations for only “black” sickle cell trait, withoutmention of “white” sickle trait in people from Greece, Cyprus,Turkey, India, and Saudi Arabia—many of whom lived inthe United States—was not medical science.

The award organisers advised me to leave names of the insurancecompanies out of my lecture. Even so, they could not run therisk that I would be bumped off before the lecture. I cannotremember being able to eat anything at the banquet, but I wasglad to be able to shake hands later with the great and good.I left Philadelphia immediately afterwards—no sight seeingfor me.

How dangerous is it these days to stand firm for scientifictruth—or rather, how risky is it to criticise scientificuntruths? The Ghanaian herbalist Nana Drobo was found with bulletholes in his head, not long after successfully treating a dyingFrench man with AIDS who was sent to him from the Ivory Coast.And how safe is it to point out that some of the prescribedprotocols for global malaria control are not the best answerto the problem? How many bodyguards would be adequate protection,when huge amounts of money are at stake in global health protocols?Money in itself is not sinful—rather, as it says in theBible: “The love of money is the root of all evil.”

Published : BMJ [www.bmj.com]

Heart Attack in sickle-cell disease and possible role of alcohol and over-transfusion?

Sickle-cell anaemia, “SS”, is just one of the 4 common phenotypes comprising sickle-cell disease, defined as possession of 2 globin gene variants at least one of which is the sickle gene.1 The others are sickle-cell Haemoglobin C disease, “SC”, sickle-cell beta-thalassaemia, “Sβ-Thal”, and sickle-cell hereditary persistence of Fetal Haemoglobin, “SFhereditary”. Ghana abounds in beta globin gene defects (qualitative and quantitative), which allowed me to study the clinical epidemiology of these 4 common phenotypes contemporaneously.2 For non-technical readers I use the term AcheAche for Sickle Cell Disease in all its forms, ie the possession of an ‘Ache’ gene from both father and mother.

Dr Pavlü and colleagues wrote an article about a 50 year old lady (Jan 20 2007, p 246)3 which is inadvertently misleading because although sickle cell anaemia ‘SS’’ (which their patient has) is sickle-cell disease, not all sickle cell disease is sickle cell anaemia. In over one thousand consecutive sickle cell disease patients that I have personally followed up, although ECG changes occurred frequently during sickle crises in all 4 phenotypes it is my “impression that signs of acute coronary occlusion and myocardial infarction, reversible after crisis, are more a feature of Hb SC disease where blood viscosity is greater than Hb SS disease (Figs 25.4 and 25.5; Case Histories 84 and 85)”.2   

     One reason their 50-year-old woman developed myocardial infarction could be because of increased red cell mass from “intermittently needed exchange transfusion”.3 What was the low-level haematocrit that determined need for transfusion, and what was the upper level attained afterwards? In my opinion the 110 g/L Hb level that many haematologists in the UK and USA feel “SS” patients required for their steady state does more harm than good1 2 4. I have both a sickle-cell anaemia (“SS”) patient who required to be bled to keep Hb level below 90 g/L otherwise he had thrombotic episodes2 (Case history 21, page 482) and a “Sβ-Thal” adult whom I once sent to Professor Lucio Luzzatto for a second opinion, and who demanded periodic venesections (removal of blood) to get Hb level below 850 g/L to abort incipient sickle crisis. Therefore, when was this 50-year-old last transfused before her myocardial infarction? What was her serum Ferritin level?

     While I totally agree with the authors “myocardial infarction should be included in the differential diagnosis of chest pain in sickle-cell disease despite normal coronary angiography”3 I want information that can help prevent myocardial infarction in my patients. Circumstances are crucial in the welfare of sickle-cell disease patients.4 What, then, were the circumstances of this particular episode? What was this lady’s alcohol intake like? A 44-year-old Hb SC man I once described (Case history 85, page 503)2 “drank Campari and brandy continuously for about 8 hours having had little to eat before going to bed. At 4 am the next morning (12.6.67) he was woken up with severe retrosternal pain ..”2. Cardiologist Professor Silas Dodu not only confirmed myocardial infarction (ECG’s on pages 504 to 508 of my book)2 but also made the correct phenotype diagnosis of Hb SC disease even before electrophoresis report arrived.2 Warned off alcohol, he abstained for 12 months but “the patient died at home after a history of drinking spirits”.2 Alcohol effect on the heart and lungs has been described in sickle-cell states5 so we need to know more about this lady.

Incidentally, insisting on raising transfusion levels of sickle cell disease patients to 110 g/dL (ie 11 grams per deciitre) as sometimes happens in the UK, continental Europe, and the USA can cause what I have come to call “hyperviscosity encephalopathy” which manifests itself with severe headaches, disorietaition, fits, and sometimes such mental symptoms as make doctors call in a psychiatrist. Not necessary. Just quickly remove a substantial amount of the transfused blood, to get the haemoglobin down to between 8 grams and 9 grams per decilitre. Watch the patient, more than you watch the patient’s blood.               

I declare that I have no conflict of interest except that I have a book ‘The Sickle Cell Disease Patient’ (Home Page) and I had 2 brothers and 1 sister with Sickle Cell Disease. My conflict of interest lies in the fact that any time I read about patients given Heroin or Morphine, or over-transfused, and causing complications, I assume them to be my own brothers and sisters. 

Felix I D Konotey-Ahulu


Kwegyir Aggrey Distinguished Professor of Human Genetics, Faculty of Science, University of Cape Coast, Ghana, and Consultant Physician & Haemoglobinopathy Genetic Counsellor, 10 Harley Street, London W1N 1AA, England

1    Ringelhann B, Konotey-Ahulu FID. Haemoglobinopathies and thalassaemias in  

     Mediterranean areas and in West Africa: historical and other perspectives 1910–1997 A

     Century Review. Atti  del’Accademia  della Science di Ferrara 1996; 74: 267-307.

2   Konotey-Ahulu FID. The sickle cell disease patient: natural history from a clinico-

     epidemiological study of the first 1550 patients of Korle Bu Hospital Sickle Cell Clinic. Watford:  

     Tetteh-A’Domeno, 1996.

3    Pavlü J, Ahmed RE, O’Regan DP, Patridge J, LefroyDC, Layton DM. Myocardial infarction in  

       sickle-cell disease. Lancet 2007; 369: 246.

4    Konotey-Ahulu FID. Sickle-cell disease and the patient. Lancet 2005; 365: 382-83.

5    Rubler S, Fleischer RA. Sudden death due to pulmonary thrombosis and infarction. Am J

      Cardiol  1967; 19: 867-73.           

Request from Geneva for patient in hospital in Colorado USA

In early June (2007) I got a frantic phone call from a lady in Geneva saying her brother “SC” was in hospital in Colorado with Sickle Cell Crisis, and had been on Morphine for 3 days, not getting better. Could I ring and advise? The patient, a professional pianist, was glad to talk to me. Within minutes, I discovered 5 precipitating causes of his crisis:

(1) Alcohol prior to illness
(2) Criss-crossing America flying
(3) Lack of sleep for 2 days
(4) Working too hard without a hoiliday
(5) Dehydration from not drinking enough water [He needed at least 2 Litres water daily]

I asked to speak to the Doctor and I told him (politely):
(1) Take him off the Morphine AT ONCE
(2) Hydrate him with 3 Litres of Normal Saline over 24 hours
(3) Injection Ketorolac subcutaneously 30 milligrams six hourly for just 3 times
(4) Get him out of bed as soon a possible
(5) Treat respiratory infection (if any).
(6) Oxygen to counteract the chest syndrome produced by the Morphine.

“And he will be out of hospital in 48 hours”. He was out of hospital in 48 hours. Alive.

Prof F Konotey-Ahulu.