Category: Sicklecell

Sickle Cell Trait Misinformation and Disinformation
F I D Konotey-Ahulu MB BS MD(London) FRCP FGCP FWACP FTWAS DTMH

Just google or wikipediate “sickle cell trait” and you get a mixture of truth and error which latter has led to (i) selective abortion (ii) insurance injustice (iii) social humiliation (iv) sports ban (v) employment exclusion (vi) clinical mismanagement and (vii) coroners’ perversion of truth. When misinformation turns “sickle cell trait” into “sickle cell disease” the consequences can be dire. Disinformation is deliberate misinformation. When terms like “congenital-abnormality” and “genetic-defect” mean different things to different people, confusion reigns. Wrong ethics plus wrong definitions of congenital or genetic defect make me revisit “abortion for sickle cells” and the Sickle Cell Trait Controversy.

WHO DEFINES “CONGENITAL ABNORMALITY” THAT NEEDS ABORTION?

“When fetal congenital abnormalities are present abortion is a valid therapeutic medical procedure” [1] was Dr Saripanidis’ response to Helen Watt who found it “heartening that so many medical students show at least some unwillingness to carry out abortion, a procedure which lethally affects the health of the fetal child (congenitally abnormal or otherwise”) [2]. Saripanidis’ “congenital abnormality” may be different from others’. Watt was responding to Zosia Kmietowicz who had reported “a fifth of medical students object to abortion for congenital abnormality” [3].

ETHICS DIVIDES EQUALLY BRILLIANT EXPERTS

I once described my Mendellian Dominant extra digits as being of no consequence, but which for a tribe east of us a child born with them was, to our disgust, promptly drowned [4]. Two Fellows of the Royal Society read my likening British doctors who advised prenatal diagnosis and selective abortion for sickle cell disease to “scientific tribesmen” behaving in a way that we Krobo people found reprehensible. Publishing my case in their Genetics books Sir David Weatherall mentioned the ethical implications in a chapter on ETHICS [5]. The other professor did not bother to mention Ethics in relation to Genetics [6]. My own position is well known. [4, 7-12] Pregnancies have been terminated for cleft palate. Extra digits are liable to be prenatally detected and fetus aborted. Making sickle cell trait a disease exposes it to an abortion programme because the received wisdom in the UK is prenatal diagnosis with advice for selective abortion.

SICKLE CELL TRAIT CONFUSED WITH SICKLE CELL DISEASE

Sickle Cell Trait (1 normal ß-globin gene ‘A’ plus 1 ß-globin variant gene ‘S’) is being confused with Sickle Cell Disease (2 ß-globin variant genes at least 1 of which is the sickle cell gene ‘S’). Look at these statistics:

(a) Among over 170 million Nigerians more than 35 million have Sickle Cell Trait ‘AS’.

(b) Ghana’s population of 25 million has 5 million sickle cell traits ‘AS’

(c) Of 6000 medically qualified West African doctors (Nigerian 4,500 and Ghanaian 1,500) working in the USA 1,500 have Sickle Cell Trait (‘AS’)

(d) One in 5 of Nigerian, Ugandan, and Ghanaian doctors in the UK are sickle cell trait.

(e) Korle Bu Teaching Hospital in Accra sees over 100,000 patients annually of whom 20,000 are Sickle Cell Trait (‘AS’)

(f) One in 5 diabetics, 1 in 5 hypertensives, 1 in 5 liver failures, 1 in 5 kidney failures, 1 in 5 suddenly-dropped-down-deads, and 1 in 5 stammerers have the sickle cell trait “AS”, because 1 in 5 of the southern Ghanaian healthy population is sickle cell trait “AS”.

Against this background we have:

(a) Elliott Vichinsky: “Renal medullary carcinoma is a rare and aggressive tumor that is seen almost exclusively in young patients with sickle cell trait” [13].

(b) Charis Kepron, Gino Somers, Michael Pollanen: “Sickle Cell Trait Mimicking Multiple Inflicted Injuries in a 5-Year-Old Boy” [14]

(c) Nigel Key, Vimal Derebail: “During exercise, Sickle Cell Trait appears to be a risk factor for sudden death and/rhabdomyolysis, particularly when the exercise is intense, and is performed at high altitude …” [15]

(d) Tsaras G, Owusu-Ansah A, Boateng FO, Amoateng-Adjepong, Y: “Complications associated with sickle cell trait: a brief narrative review”. [16]

So 100 million African sickle cell traits, millions in southern Turkey (1 in 5 Eti-Turks are ‘AS’), Mediterranean people with millions of ‘AS’ Trait [17-20], plus millions in India’s Madhya Pradesh and Orissa with 43% ‘AS’ Trait [21] are in some danger? Insurance injustice apart, the next step would be to advise aborting any fetus with Hb ‘S’ in it.

“UP-TO-DATE” AND “NOVEL” INSIGHTS ARE OUT OF DATE

Forty years ago exactly the then BMJ Editor Dr Martin Ware helped to retract a sickle cell trait inaccurate publication that caused furore [22-29]. No author has mentioned the 10 Addae’s Criteria required to be fulfilled before someone attributes symptomatology to sickle cell trait [24]. Witkowska et al published that sickle cell trait and sickle cell disease co-existed in the same person [30]. Elliott Vichinsky [13] failed to pick up the genetic heresy of 4 ß-globin genes in one person. I had warned that Witkowska’s patient did not have sickle cell trait but ‘Sickle Cell Haemoglobin Quebec-Chori’ disease [31] which “made it vital for clinicians to probe further any case of sickle cell trait where the symptoms suggest sickle cell disease” [31]. To provide excuse to include the sickle cell trait phenotype in an abortion programme is frightening.

CLINICAL EXPERIENCE MUST SUPERINTEND HAEMATOLOGICAL EXPERIENCE

My views here are not those of a novice. Professor Helen Ranney once said: “There is no single clinical experience in the United States comparable to that of Dr Konotey-Ahulu” [32]. Our family named sickle cell disease patients from 1670 Anno Domini http://www.konotey-ahulu.com/images/generation.jpg correctly identifying phenotypes Chwechweechwe/Hemkom (Sickle cell Disease) “SC” as “pi-gbagblaa”, and “SS” as “gbagblaa” [33 34 38]. Directing the largest Sickle Cell Disease Clinic in the world [34], I could guess the 4 sickle cell disease phenotypes correctly (‘SS’, ‘SC’, SF’, SßThal’) without haemoglobin electrophoresis [34 35]. That was how Haemoglobin Korle-Bu and Haemoglobin Osu-Christiansborg were discovered [36, 37]. We even knew that the commonest cause of severe sickle cell crisis was malaria [34 39], yet erroneous statements like “Sickle Cell Disease protects against malaria” persist.

HISTORY BEHIND CONFUSION WITH SICKLE CELL TRAIT DEFINITION

When Itano discovered Haemoglobin C in 1951 [40], the riddle was solved of seeing sickle cell disease symptomatology in someone who was wrongly routinely referred to as “Sickle Cell Trait” (1 ß-globin gene variant ‘S’ plus 1 normal ß-globin ‘A’) because the blood of one of the parents of the “SC disease phenotype” (2 ß-globin gene variants) did not sickle. Moreover when the ‘AS’ pattern represents 2 abnormal genes as in Sickle Cell ß-plus Thalassaemia, or as in Sickle Cell Haemoglobin Quebec-Chori disease, clinical experience more than haematological experience is what is required to prevent wrong interpretations. [Read that again, and again, please]
Furthermore, the sickle cell colour test does not distinguish between Sickle Cell Trait ‘AS’ and Sickle Cell Hb C disease ‘SC’ [41], and has thus wrongly attributed death to Sickle Cell Trait. I was once guilty of rushing to publication only to be corrected by Dr E J Watson-Williams who advised Family Studies [34, p. 364]. I then discovered to my shame [42] that the man who had died in London during a minor eye operation was “SC phenotype”, not sickle cell trait “AS” phenotype.

IMPLICATIONS FOR SICKLE CELL TRAIT REDEFINED AS SICKLE CELL DISEASE

ABORTION ADVICE is one consequence. INSURANCE PUNISHMENT is another. I was given 4 body guards in Philadelphia for my Keynote Address at The Martin Luther King Jr Foundation Award Ceremony. The title was “Difference between Sickle Cell Trait and Sickle Cell Disease” [43]. Insurance companies wanted the difference blurred. Approached by Professor Bowman in the USA about the Sickle Cell Trait [44] they admitted to loading the trait’s premium. [44] “It is my understanding” says Bowman “that insurance companies test only Blacks for the sickle cell trait” [45]. Did these authors (13-16) exclude Hb Quebec-Chori masquerading as normal Hb ‘A’? Was Hb ‘S’ quantified in all their publications? How many patients had G6PD quantified as 1 in 5 Ghanaian males and 1 in 16 females have total G6PD Deficiency [46 47 48] which affects the kidney adversely [49 50 51]? How many Family Studies were done?

IN SPORT ARE ALL NATIONALITIES PHENOTYPED “FOR THEIR OWN SAFETY”?

Are white skinned Americans included in sickle cell screening before sports? Nobel Laureate Professor James Watson’s great grand parent was an African. [52 53] As 1 in 3 West Africans had a ß-globin gene variant ‘S’ or ‘C’ [10] could Watson have either? Regarding advice given [22] to screen “Negro travellers” at airports for sickle cells for their own safety Dr Djabanor asks regarding White sickle cell traits: “How do we identify them from their external features to thrust upon them the benefits of this advice?” [23]

HAEMOGLOBIN ‘S’ IN TRUE SICKLE CELL TRAIT IS BETWEEN 20% AND 39.7%

Of 82 consecutive sickle cell traits that emerged out of over 400 consecutive West Africans I saw in London that Professor Hermann Lehmann phenotyped for me the 3 known bands of Hb ‘S’ percentages were clearly demonstrated, with the highest percentage less than 40%, and the lowest haemoglobin ‘S’ value was as little as 20% [54]. Are these the levels of Hb S that we are told millions around the world stand in danger from? Writing to The London Times exactly 40 years ago Lehmann confirmed that sickle cell traits competed in the Olympic Games in Mexico City at 7000 ft above sea level and no sudden deaths occurred. Chicago University’s Professor James Bowman said : “Persons with sickle cell trait will no longer be able to become ill or even die lest they find themselves subject of case report” [55], and a Black man beaten to death by police was claimed by the Coroner to have died from sickle cell trait. Professors Simon Dyson and Gwyneth Bosswell recount similar experiences [56]. Social humiliation resulted with suggestion [22] that “Negro travellers” be tested at airports for sickle cells before flight “for their own safety”. Acute appendicitis was misdiagnosed as abdominal sickle cell crisis because sickle cell test was positive, and employment was refused on the grounds of “sickle cell trait”. I found 15 flaws [57] in the article on the 5-year old boy who died and was found with “multiple inflicted injuries” and aspiration pneumonia, only to be published as “Sickle cell trait mimicking multiple inflicted injuries” [14]. My Sickle Cell Trait chapter [34 pp 349-371] deals comprehensively with similar anomalies.

BALANCED POLYMORPHISM AND AFRICAN ANTHROPOGENETICS

We know that the true ‘AS’ [NORMACHE] can be tougher than phenotype ‘AA’ [NORMNORM] because in the toddler age group we find no cerebral malaria child with sickle cell trait [58], which fact is the basis of the Balanced Polymorphism phenomenon [34 pp. 91-108], about which there is so much ignorance [59]. To assess the Hardy Weinberg Equation of gene frequencies without reference to the genetic index ‘MPSI’ that I once invented, and to polygamy [60 61 62], is to ignore African realities.

TRANSPARENCY AND ABSENCE OF PREJUDICE REQUIRED

Why all of a sudden such concern that “for their own safety” sickle cell traits should not be allowed to run competitively? And has there been an attempt to find out why Black families are increasingly refusing prenatal screening in the UK? [63] Is this reluctance related to the rumour that a Teaching Hospital screened a West African mother who refused abortion when told baby would be ‘SS’ but when born was found to be ‘AA’, and “Laboratory Error” was claimed as reason for the misinformation 7 months previously? [63] Medical Practice is known to have been marred in time past by racial prejudice [64 65 66 67], so one needs to ask what proportion of the medical students “object to abortion for congenital abnormality” [1 2 3] not just for ethical reasons but also because they are from ethnic minority groups that harbour suspicions?

Competing interests: I not only carry a congenital abnormality, Mendellian dominant extra manual digits, but I am also one of 11 children of parents both of whom were Traits for beta-globin variant genes [NORMACHE], resulting in 3 siblings born with Sickle Cell Disease [ACHEACHE], 4 with Trait [NORMACHE] and 4 with “AA” [NORMNORM] – see www.konotey-ahulu.com/images/generation.jpg

Felix I D Konotey-Ahulu MD(Lond) FRCP DTMH Kwegyir Aggrey Distinguished Professor of Human Genetics, University of Cape Coast, Ghana and Consultant Physician Genetic Counsellor in Sickle Cell and Other Haemoglobinopathies, 9 Harley Street, Phoenix Hospital Group, London W1G 9AL [E-mail: felix@konotey-ahulu.com
Website: http://www.sicklecell.md]

1 Saripanidis Stravos. Abortion is a valid therapeutic medical procedure. BMJ Rapid Response 30 October 2011. Re: BMJ 2011; 343.doi: 10.1136/bmj.d4717.1

2 Watt Helen. Doctors should not refer for harmful elective procedures. Rapid Response 26 July 2011. Re: BMJ 2011; 343.doi: 10.1136/bmj.d4717.1

3 Kmietowicz Zosia. A fifth of medical students object to abortion for congenital abnormality. 22 July 2011. BMJ 2011; 343.doi: 10.1136/bmj.d4717.1

4 Konotey-Ahulu FID. Ethical issues in prenatal diagnosis. BMJ Clin Res Ed 1984; 289(6438)185 http://www.bmj.com/cgi/reprint/289/6438/185-a.pdf July 21 doi:10.1136/bmj.289.6438.185-a

5 Weatherall D J. Ethical issues and related problems arising from the application of the new genetics to clinical practice. Chapter 12 In The New Genetics and Clinical Practice. Oxford University Press (Third Edition) Oxford 1991, pages 347-48.

6 Jones Steve. The Language of Genes; Biology, History, and Evolutionary Future. Flamingo 1993, London, page 285.

7 Konotey-Ahulu FID. Ante-natal diagnosis of haemoglobinopathies. Lancet 1977; 1: 597-598.

8 Konotey-Ahulu FID. Ethics of amniocentesis and selective abortion for sickle cell disease. Lancet 1982; 1(8262): 38-39. January 2.

9 Konotey-Ahulu FID. Missing the wood for one genetic tree? The First International Symposium on the Role of Recombinant DNA in Genetics – Proceedings – Chania, Crete, Greece, May 13-16 1985. Eds Loukopoulos D, Teplitz RL; Athens, P. Paschalidis 1986, pages 105-116.

10 Ringelhann B, Konotey-Ahulu FID. Hemoglobinopathies and thalassemias in Mediterranean areas and in West Africa: Historical and other perspectives 1910 to 1997 – A Century Review. Atti dell’Accademia dell Science di Ferrara 1998; 74: 267-307 Milan

11 Konotey-Ahulu FID. Antenatal screening for sickle cell disease and beta-thalassaemia. http://www.bmj.com/content/341/bmj.c5132/reply#bmj_el_242914 BMJ Rapid Response Oct 12 2010

12 Konotey-Ahulu FID. Antenatal sickle cell disease/haemoglobinopathy screening. BMJ Rapid Response http://www.bmj.com/content/341/bmj.c5243/reply#bmj_el_243447 October 25 2010

13 Vichinsky Elliott P. Sickle cell trait. Literature Review UpToDate [Accessed 18 Feb 2011] http://www.uptodate.com/contents/sickle-cell-trait?view=print

14 Kepron Charis, Somers Gino R, Pollamen Michael S. Sickle Cell Trait Mimicking Multiple Inflicted Injuries in a 5-Year-Old Boy. Journal of Forensic Sciences Volume 54, No.5, pp 1141 t0 1145 September 2009.

15 Key Nigel S, Derebail Vimal K. Sickle Cell Trait: Novel Clinical Significance. Hematology 2010: 418-422.

16 Tsaras, G, Owusu-Ansah A, Boateng FO, Amoateng-Adjepong, Y. Complications associated with sickle cell trait: a brief narrative review. American Journal of Medicine 2009; 122(6): 507-512.

17 Aksoy M. Sickle cell trait in Southern Turkey. Lancet 1955; 1: 589-90.

18 Atlay C, et al. Haemoglobin S and some other hemoglobinopathies in Eti-Turks. Human Heredity 1978; 28: 56-61.

19 Choremis C et al. Sickle cell anaemia in Greece. Lancet 1951; 1: 1147.

20 Choremis et al. Blood groups of a Greek community with a high sickling frequency. Lancet 1957; 2: 1333-34.

21 Roy DN, Chaudhuri RSK. Sickle cell trait in the tribal population in Madhya Pradesh and Orissa (India). Journal of Indian Medical Association, 1967; 49: 107-112.

22 Green RL, Huntsman RG, Serjeant GR. Sickle cell trait and altitude. Br Med J 1971; 4: 593-595.

23 Djabanor F F T. Sickle cell trait and altitude. Brit Med J 1972; 1: 113

24 Addae R O. Sickle cell trait and altitude. BMJ 1972; 1: 53. [10 criteria required to satisfy clinicians in regions where 1 in 5 people have the sickle cell trait that symptoms are due to the trait]

25 Konotey-Ahulu FID. Sickle cell trait and altitude. Brit Med J 1972; 1: 177-178.

26 Lehmann Hermann. Sickle cell and flying. The Times (London), 4th January 1972.

27 Konotey-Ahulu FID. An international sickle cell crisis. [Editorial] Ghana Medical J; 1972; 11: 4-8

28 Konotey-Ahulu FID. Sickle cell trait and altitude. BMJ 1972; 2: 231-32 April 22

29 Green RL, Huntsman RG, Serjeant GR. Sickle and altitude. Brit Med J. 1972; 2: 294

30 Witkowska HE, Lubin BH, Beuzard Y et al. Sickle cell disease in a patient with sickle cell trait and compound heterozygosity for haemoglobin S and haemoglobin Quebec-Chori. New England Journal of Medicine 1991; 325: 1150-1154. [Note that the title of this article is incorrect: No human being can be said to have both Sickle cell trait and Sickle Cell Disease. The ‘AS’ pattern is sickle cell trait pattern, but this ‘A’ is not a true ‘A’ but the new haemoglobin called Quebec-Chori, producing a disease phenotype, not trait.]

31 Konotey-Ahulu FID. Beware of symptomatic sickle cell traits. Lancet 1992; Feb. 29, p 565. [Re: The enigma of Haemoglobin Quebec-Chori]

32 Ranney Helen M. Summary of Symposium. In Sickle Cell Disease, Editors H Abramson, J F Bertles, Doris Wethers [C Mosby Co., St Louis] 1973, p 320.

33 Konotey-Ahulu FID. Sickle cell disease in successive Ghanaian generations for three centuries. Chapter 2 pp 6-20 in The Sickle Cell Disease Patient: Natural History from a Clinico-epidemiological study of the first 1550 patients of Korle Bu Hospital Sickle Cell Clinic. Macmillan Press Ltd, London 1991 & 1992 and T-A’D Co Ltd Watford 1996.

34 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. Macmillan Press Ltd, London 1991 & 1992 and T-A’D Co Ltd Watford 1996

35 Konotey-Ahulu FID. Patterns of clinical haemoglobinopathy. E Afri Med J 1969 Mar; 46(3): 149-156. PMID: 5800410 [PubMed – indexed for MEDLINE]

36 . Konotey-Ahulu FID, Gallo E, Lehmann H, Ringelhann B. Haemoglobin Korle Bu (alpha2 beta2 73 Aspartic Acid –> Asparagine), showing one of the two amino acid substitutions of Haemoglobin C Harlem. J Med Genet 1968 June; 5(2): 107-111. & http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=1468514 An example of intra-genic cross-over [Also see G-Accra http://lib.bioinfo.pl/pmid:5722880 ]

37 Konotey-Ahulu FID, Kinderlerer, JL Lehmann H and Ringelhann B. Haemoglobin Osu-Christiansborg. A new chain variant of Haemoglobin A (beta 52 D3 Aspartic Acid –> Asparagine) in combination with Haemoglobin S. Journal of Med Genet 1971 Sep; 8(3): 302-305. http://pubmedcentral.nih.gov/picrender.fcgi?artid=146917&blobtype=pdf or http://lib.bioinfo.pl/pmid:5097135

38 Konotey-Ahulu FID. The Sickle-cell Diseases: Clinical manifestations including the Sickle Crisis http://archinte.ama.assn.org/cgi/reprint/133/4/611-pdf . Arch Intern Med 1974; 133(4): 611-619.

39 Konotey-Ahulu FID. Malaria and sickle-cell disease BMJ 1971 June; 2(5763): 710-711 doi:10.1136/bmj.2/5763.710-d http://www.bmj.com/cgi/reprint/2/5763/710-d.pdf

40 Itano HA. A third abnormal haemoglobin associated with hereditary hemolytic anemia. Proc Nat Acad Sci (Washington) 1951; 37: 775-84

41 Konotey-Ahulu FID. Detecting sickle cell haemoglobin. Brit Med J 1972; 4: 239

42 Konotey-Ahulu FID. Blushing in the black skin. (Invited Editorial) Journal of Cosmetic Dermatology April 2003; 2 (2), 59-60 [PMID: 17156057 PubMed]

43 Konotey-Ahulu FID. Four bodyguards and the perils of unmasking scientific truths. BMJ 2007; 335: 210-211. http://www.bmj.com/cgi/content/full/335/7612/210 (July 28)
doi:10.1136/bmj.39268.553021.47 Print http://www.bmj.com/cgi/reprint/335/7612/210.pdf
[Day & Date: Wednesday 31st May 1972 – Philadelphia, Dr Martin Luther King Jr Foundation Award Ceremony for Outstanding Contributions in Sickle Cell Disease: Banquet – My Keynote Address was on ‘Difference between Sickle Cell Trait and Sickle Cell Disease’. Those also honoured present on the platform with me included Nobel Prize Winners Linus Pauling and Max Perutz, then Hermann Lehmann, Roland Scott, J V Neel, Bella Ringelhann, A C Allison, James Bowman, Helen Ranney, Charles Whitten, Samuel Charache, L Diggs, L Conley, Sam Charache & Graham Serjeant].

44 Konotey-Ahulu FID. Insurance and genetic testing. Lancet 1993, 341: 833. March 27

45 Bowman James. Ethical, legal, and humanistic implications of sickle cell programs. INSERM 1975; 44: 353-378.

46 Ringelhann B, Dodu SRA, Konotey-Ahulu FID and Lehmann H. A survey for haemoglobin variants, thalassaemia and Glucose-6-Phosphate Dehydrogenase Deficiency in Northern Ghana. Ghana Med J 1968; 7: 120-124.

47 Konotey-Ahulu FID. Hereditary qualitative and quantitative erythrocyte defects in Ghana: An historical and geographical survey. Ghana Med J 1968; 7: 118-119 (Editorial 35 references). [First time Chwechweechwe and other tribal names were identified as Sickle Cell Disease]

48 Owusu SK. Glucose 6 Phosphate dehydrogenase (G6PD) deficiency in the causation of disease in Ghana. Ghana Med J. 1974; 13: 168-70

49 Owusu SK. Absence of glucose 6 phosphate dehydrogenase in red cells of an African. Brit Med J 1972; 4: 25-26

50 Owusu SK, Addy JH, Foli AK, Janosi M, Konotey-Ahulu FID and Larbi EB. Acute reversible renal failure associated with glucose-6-phosphate dehydrogenase deficiency. Lancet 1972 June 10; 1(7763): 1255-1257.

51 Adu D et al. Acute renal failure and typhoid fever in Ghana. Ghana Med J 1975; 14: 172-74.

52 Verkaik Robert. Scientist who sparked racism has black genes. The Independent, London. 10 December 2007. [Re: DNA Nobel Laureate Professor James Watson]

53 Konotey-Ahulu FID. There is but one human race. New African, London. Dec 2009, No. 490, page 4.[Re: James Watson who with Francis Crick won Nobel Prize on DNA]

54 Konotey-Ahulu FID. Alpha-Thalassaemia nomenclature and abnormal haemoglobins Lancet 1984; 1: 1024-25 May 5 [“Of 82 consecutive sickle cell traits seen in London in 24 months 36 (44%) had just one quarter of the total haemoglobin as sickle haemoglobin (mean 25%, range 20-28%) ..The three known peaks of haemoglobin S proportion in the West African sickle cell sickle cell trait are around 25%, around 30& and around 38%”)

55 Bowman JE, Bernstein S. Caution about preliminary reports. Pediatrics 1977; 59: 639-40.

56 Dyson Simon, Bosswell Gwyneth. Sickle Cell and Deaths in Custody. Whiting and Birch, London: June 2009; 230 pages “The misuse of Sickle Cell Trait to explain away sudden deaths”.

57 Konotey-Ahulu FID. Blaming sudden death on Sickle Cell Trait? Flaws in article of Charis Kepron, Gino Somers and Michael Pollanen Exposed. September 4 2011. . www.sicklecell.md/blog/?p=105 or www.konotey-ahulu.com/blog/?p=105

58 Commey JOO. Absence of sickle cell trait in 30 consecutive cases of cerebral malaria in Ghana when 6 expected. [Observations in 1986 see Reference 33, page 95] See also Konotey-Ahulu FID. Balanced polymorphism and other factors relating to hereditary qualitative and quantitative erythrocyte defects. Chapter 8, pp 91-108 in Reference 34, and also Chapter 30 Percentage values of haemoglobins S, F, A2 C, A, in various sickle cell phenotypes, and a consideration of the sickle cell trait pp 349-370]..

59 Konotey-Ahulu FID. Malaria and sickle cell: “Protection?” Or “No Protection?” – Confusion reigns. http://www.bmj.com/cgi/eletters/337/oct01_3/a1875#203067 BMJ Rapid response 13 October 2008

60 Konotey-Ahulu FID. Male procreative superiority index (MPSI): The missing co-efficient in African anthropogenetics. BMJ 1980; 281(6256): 1700-1702 http://www.pubmedcentral.nih.gov/picrender.fcgi?artid=1715685&blobtype=pdf doi:10.1136/bmj.281.6256.1700 December 20 – 27 1980 http://www.bmj.com/cgi/reprint/281/6256/1700.pdf

61 Konotey-Ahulu FID. The Male Procreative Superiority Index (MPSI): its relevance to genetical counseling. In FIFTY YEARS OF HUMAN GENETICS A Festschrift and liber amicorum to celebrate the life and work of GEORGE ROBERT FRASER Edited by Oliver Mayo and Carolyn Leach. Wakefield Press 2007 (www.wakefieldpress.com.au) 1 The Parade West, Kent Town, South Australia 5067)

62 Bonney GE and Konotey Ahulu FID. Polygamy and genetic equilibrium. Nature 1977; 265: 46-47 (January 6 1977). doi:10.1038/265046a0
http://www.nature.com/nature/journal/v265/n5589/abs/265046a0.html

63 Konotey-Ahulu FID. Konotey-Ahulu FID. Refusing to provide a prenatal test for refusing later termination of pregnancy can it ever be ethical? BMJ Rapid Response November 20 2006 http://www.bmj.com/cgi/eletters/333/7577/1066#149662

64 Muller-Hill Benno. Murderous Science. Elimination by Scientific Selection of Jews, Gypsies, and Others – Germany 1933-1945. [Translated from German by G R Fraser] Oxford, Oxford University Press, 1988.

65 Clinton President WJ. Apology on behalf of the American Government to 8 survivors of the Tuskegee Syphillis experiment victims. World-wide radio and television PBS News Hour Newsreel Announcement (Jim Lehrer and Charlayne Hunter-Gault) May 16 1997
http://www.pbs.org/newshour/bb/health/may97/Tuskegee_5-16.html

66 Tanne Janice Hopkins. President Obama apologizes to Guatemala over 1940s syphilis study. BMJ 341: doi:10.1136/bmj.c5494 October 4 2010

67 . Konotey-Ahulu FID. President Obama apologizes over Guatemala syphilis study: International co-operative research and practice in jeopardy. Rapid response BMJ 17 October 2010 http://www.bmj.com/content/341/bmj.c5494.full/reply#bmj_el_243183

Blaming sudden death on Sickle Cell Trait?
Flaws In Article Of Charis Kepron, Gino Somers and Michael Pollanen Exposed.

Felix I D Konotey-Ahulu MD(Lond) FRCP(Lond) DTMH(L’pool) FGCP FWACP FTWAS

Dr Kwegyir Aggrey Distinguished Professor of Human Genetics University of Cape Coast, Ghana and Consultant Physician Genetic Counsellor in Sickle Cell and Other Haemoglobinopathies, Ten Harley Street, London W1G 9PF. [Founder & Co-Director of KÁGÈ SICKLE CELL FOUNDATION www.sicklecell.md & felix@konotey-ahulu.com]

“Sickle Cell Trait Mimicking Multiple Inflicted Injuries in a 5-Year-Old Boy” is the title of an article published in the Journal of Forensic Science ¹. I do not know which expert reviewed and passed this article for publication but the 20% of healthy Ghanaians with sickle cell trait have good reason to protest at such a flawed article masquerading (as the authors Charis Kepron and others put it) “the first to describe sickle cell trait pathology as a mimic of a non-accidental injury”¹.

The defects of this article are very many:

(i) Mentioning sickle cell disease (scd) and sickle cell trait (sct) in the same breath betrays clinical ignorance. References 1 and 2 on “sickle cell disease” quoted by Kepron and colleagues in support of a “sickle cell trait” article are therefore totally irrelevant.

(ii) Ponder the following statement of the authors: “Sudden unexpected death in a 5-year old child due to pulmonary complications of sickle cell trait in whom the pattern of bone lesions seen at autopsy mimicked multiple inflicted injuries”.¹ Now, 1 in 5 of all Ghanaian children at home and abroad who may be found to be traumatised and left with broken bones will be found to be sickle cell trait because 20% of the rest of the healthy Ghanaian population are also sickle cell trait ‘AS’. So does that mean the bone defects seen in this Ghanaian child in Canada can be attributed to sickle cell trait?

(iii)   The child had complained of a painful leg, and was said to have had “mild tissue swelling” and yet the X-ray films were not “retrospectively reviewed”.¹ Why?

(iv)   There was no family study of sickling in a case report of such importance. What were the levels of Haemoglobin ‘S’ in the mother and/or the father?

(v)   The haemoglobin electrophoretic report is flawed. Add up the various fractions quoted in the publication and you get 100% [Hb A = 74.9%, Hb S = 23.2%, Hb F = 1.9%], but where is Haemoglobin A₂? How could reputable haematologists, paediatricians, and pathologists forget Hb A₂ in such an electrophoretic report?

(vi)   Autopsy showed the Right lower lobe of the lung to be (in their own words) “pale, congested, consolidated, and focally haemorrhagic”.  The question is this: Are these pneumonic changes never seen in children who do not have sickle cell trait? In other words, does sickle cell trait have to be invoked to explain such pathological changes?

(vii)   “A layered dissection of the posterior para-spinal muscles of the neck” say the authors “showed focal areas of soft tissue haemorrhage”¹ and yet they blame these changes on sickle cell trait, ruling out child abuse?

(viii)   The authors Charis Kepron, Gino Somers and Michael Pollanen report that “Both lungs contained foreign material in keeping with aspiration of gastric contents”, the classical residue for an aspiration pneumonia that could kill a child instantly, yet the sudden death was attributed to sickle cell trait by these authors?

(ix)   Staphylococcus aureus was cultured from both lungs, and yet complicated staphylococcal pneumonia was not suggested as the cause of sudden death, but the sickle cell trait with just 23.2% of sickle cell haemoglobin ‘S’ ?

(x)   The authors state that the sickle cell trait “can be a cause of acute complications normally associated with sickle cell disease including the acute chest syndrome”.¹ This ex cathedra statement is simply not true. The sickle cell trait ‘AS’ had run at Olympic Games at Mexico City over 7000 ft where the air is thin and oxygen concentration is lower than at sea level, and these sickle cell traits ‘AS’ (or NORMACHE as I call them) had competed with and beaten the whole world. And the authors couple this ‘AS’ phenotype with Sickle Cell Disease phenotype (ACHEACHE)? Moreover, people of all nationalities without sickle cell trait die suddenly from aspiration pneumonia, so why should a sickle cell trait child behave differently?

(xi)   Invoking acute chest syndrome as cause of death in a sickle cell trait child stems more from ignorance of what has been termed “acute chest syndrome”. The term was coined only in recent decades to explain breathing problems in sickle cell disease patients. This diagnosis assumed huge proportions only when Morphine and Diamorphine constituted the recommended prescription to treat pain of sickle cell crisis in the UK and the USA². The NCEPOD Report of the United Kingdom revealed that  “Nine out of the 19 patients with sickle cell disease who had pain on admission and who then died had been given excessive doses of opiods” (meaning morphine and diamorphine)“ ^{3, 4, 5}. Now, for Kepron and colleagues to invoke the acute chest syndrome as contributory to the Ghanaian child’s sudden death in Canada was just scraping the barrel to link sickle cell trait to the sudden death. Quoting Vichinsky and colleagues ⁶ in support of their acute chest syndrome proposition was a mistake because Vichinsky’s paper failed to diagnose the devastating role of opiates (Morphine and Diamorphine) in the causation of the chest syndrome due to the opiods’ respiratory depressive effect on the very patients who needed oxygen to survive. ⁵

(xii)   The authors’ references 11 and 12 in the article that they cite to blame acute chest syndrome on sickle cell trait failed to take cognisance of the ten Addae’s Critreria ⁷ which must be met before serious symptomatology is blamed solely on sickle cell trait. For instance, the sickle cell trait must not be blamed for serious symptomatology without quantification of Hb A₂ and yet Kepron and colleagues did not even mention Haemoglobin A₂ let alone quantify it.

(xiii)   It is quite surprising how facts that should have made Kepron and colleagues look elsewhere, rather made them fixate on sickle cell trait because they were determined to do just that. Take for example their statement “Although the clinical information required for a diagnosis of acute chest syndrome was missing …” histologic findings were used by them to “prove” acute chest syndrome, and they stated that “acute chest syndrome was felt to be the major contributing factor to the cause of death”.¹ Does the phrase “was felt” have any place in scientific discussion? Should we be concerned with how we “feel” when debating scientific topics, or should we rely on facts alone?

(xiv) The final statement of their inauspicious article says it all; it betrays a deliberate desire to link the un-linkable: “Although Sickle Cell Disease/Sickle Cell Trait is not one of the classic mimickers of child abuse, unusual orthopaedic pathologies can and do occur, and may appear as  inflicted injury on skeletal survey”¹.

(xv)   The authors Kepron, Somers, and Pollanen fail to do their home work in the country that discovered Haemoglobin Quebec-Chori. This haemoglobin masquerades as normal adult Haemoglobin ‘A’ but which when tagged on to sickle cell haemoglobin does not produce sickle cell trait ‘AS’ (ie NORMACHE by my terminology), but rather sickle cell disease (ACHEACHE). This was how I put it in The Lancet in a communication entitled ‘Beware of symptomatic sickle-cell traits’: ⁸

“With the sickle cell population increasing yearly in the UK, the finding by Witkowska and colleagues⁹ of sickle cell Haemoglobin Quebec-Chori genotype producing a sickle cell disease phenotype masquerading electrophoretically as a sickle cell trait makes it vital for clinicians to probe further any case of sickle cell trait where the symptoms suggest sickle cell disease.⁹ Many individuals with true sickle cell trait ‘AS’ (betaA3[⁶Glu; betaA3[⁶Glu à Val]) with ‘A’ greater than ‘S’ have been victimised in respect to employment and life insurance because of substandard medical reports in journals”. ^{10, 11}

 

The authors of this present article do not appear to have read about Haemoglobin Quebec-Chori which was first discovered in their own country Canada.⁹

 

LESSONS DRAWN FROM THIS ‘JOURNAL OF FORENSIC SCIENCE‘ ARTICLE

 

1. Battles that have been fought and won in the Sickle Cell Trait controversy can suddenly be resurrected, so there is need to be vigilant. Forty years ago, based on a false report by authors who had never been to Ghana, on a Ghanaian sickle cell trait during the 45-minute flight from Kumasi to Accra it was suggested that for their own safety “Negro passengers” should be tested at airports for the sickling phenomenon “for their own safety”¹² Ghanaian experts exposed not only the falsehood of the report¹³, but also were instrumental in reversing draconian measures that were being taken world wide based on that false report. Black pilots and air crew had been grounded at Kennedy Airport in the USA because of the false report. It was Ghanaian expertise that restored them to flying duties, and forced the case report publication in the British Medical Journal to be withdrawn.¹⁴ [See the detailed account in FAQs – Frequently Asked Questions on my website www.konotey-ahulu.com or www.sicklecell.md] ¹⁵

2. University of Illinois Professor of Medicine and Pathology Dr James Boweman MD and Dr S. Bernstein observing the spate of journal articles linking all kinds of symptoms with the sickle cell trait were forced to exclaim when a Black man beaten to death in police custody was found to be sickle cell trait, and the death was then attributed to the sickle trait: “Persons with sickle cell trait will no longer be able to become ill or even die lest they find themselves subject of a case report”.¹⁰ This prediction of Boweman and Bernstein in 1977 regarding bogus articles has sadly been fulfilled through this flawed case report of Charis Kepron and colleagues.¹

3.   New attempts are being made presently (in this year of 2011) to ban sickle cell traits from competing in athletics because sudden death has been linked (spuriously) to the sickle cell trait phenotype as recounted in my book.¹¹ My website FAQs have dealt with the flawed articles cited in this respect. If someone who does not sickle dies from exercise, that is considered natural, but when sickle cell trait is found in the person, then it is not considered “natural” – it must be due to the sickle cell trait. Banning 20 per cent of all Ghanaian international athletes (and Black competitors in general) from global athletics “for their own safety” is seriously being considered by some scientists of World Bodies. Some of them talk about “Black Sickle Cell Traits” forgetting that 1 in 6 of the White people in southern Turkey ^{16, 17} and up to 30% of the white people in Greece around where Lake Kopais was had been shown to have the sickle cell trait (‘AS’) ^{18, 19} leading Ghana’s Dr Frank Djabanor once to ask in the British Medical Journal: “How can we identify them by their external features to thrust upon them the benefits of this advice”? ²⁰, namely the advice that Negro passengers should be tested at airports for sickle cell trait “for their own safety” ¹² The term “Black Sickle Cell Traits” must be banned, because there are millions and millions of “White Sickle Cell Traits”. Insurance Companies, and International Sports Federations need to take note of that. If they do not, they must be held to account. Indeed I once pointed out ²¹ that when contacted in the USA by Professor James Boweman “about the harmless sickle cell trait”, 41% of 39 insurance companies admitted to loading the trait premium. “It is my understanding” added Boweman “that insurance companies generally test only Blacks for the sickle cell trait”.²²

4.   What I have said above is no joking matter. African doctors should be aware of global trends that are inimical to their welfare. Articles like that of Kepron et al encourage Insurance Companies to load the premium of Sickle Cell Traits because of the unjustified and unscientific published statements like “Complications of Sickle Cell Disease/Sickle Cell Trait are not usually on the differential diagnosis of traumatic injury”.¹

5   Articles such as we find here in the Journal of Forensic Science are likely to be quoted by inexperienced clinicians and pathologists in support of their equally flawed findings. This must not be allowed to happen. Fortunately, wise editors such as are found for The British Medical Journal and The Lancet in England always go back to correct errors in previous publications whenever these were later pointed out to them. “We therefore wish to withdraw this case”¹⁴ was how Green, Huntsman and Serjeant removed the unsubstantiated “sickle cell trait intestinal infarction” case from publication. Family studies have shown that a case I once thought was sickle cell trait (NORMACHE), was in fact the Ghanaian sickle cell haemoglobin C disease patient (ACHEACHE) who was a banker and who died under anaesthesia in a London hospital during eye surgery. I challenge the editors of Journal of Forensic Science, in the light of what has been said above, to state that the case for child abuse in their case report of the second autism child that they described could not be dismissed, and I urge that these authors’ conclusion that their findings could be attributed to sickle cell trait be dismissed forthwith.

6   There are huge financial interests involved if the true sickle cell trait ‘AS’ (NORMACHE) who beat the whole world at athletics is equated with sickle  cell disease pathology, allowing insurance companies to benefit at the expense of healthy people. As Cambridge University’s Professor Hermann Lehmann, the doyen of Abnormal Haemoglobin research in the UK, wrote to the London Times when the false sickle cell trait story was published on December 9 1971 advocating the removal of sickle cell traits from flying duties: “The sickle cell trait is, in some Africans much more rare than in the population of, say Crete or Coimbatore” and he went on to say that “sickle cell carriers competed without ill effect at the Olympic Games at Mexico at an altitude of 7000 ft”²³ Fancy then a Black athlete coming down from Mexico City to New York at sea level and be told that his Health Insurance premium would go up to 150% because some researcher had published that exercising at 4000 ft had caused death in a sickle cell trait! For the world to be told at the Martin Luther King Jr Foundation Award Ceremony in 1972 in Philadelphia in the presence of Abnormal Haemoglobin Nobel Prize winners Linus Pauling and Max Perutz, that Insurance Companies in the USA were benefiting unfairly from the “Sickle Cell Trait sudden death at 4000 ft” story – a story that lacked scientific veracity – for the world to be told this –  was too much of a risk to take so the organisers of the Award Ceremony provided me whom they had invited to give the Keynote Address on “The difference between Sickle Cell Trait and Sickle Cell Disease” with four body guards for all the time I was in Philadelphia. Read the full story in the British Medical Journal.²⁴

7   Finally, please wake up to the fact that modern researchers fail to recognise and quote thorough work that had been done decades ago. Any modern author that relies on a MEDSEARCH that contents itself with going back only 25 years is deceiving not only themselves but also the rest of us. Professors George M Edington and Hermann Lehmann did such meticulous Abnormal Haemoglobin research in the Gold Coast (Ghana) nearly 60 years ago as has hardly been equalled in thoroughness. ^25-29 What these giants of Abnormal Haemoglobin Research said about Sickle Cell Trait and Sickle Cell Disease all those decades ago has not been bettered by any subsequent work that I know of. Yet modern authors like Kepron and colleagues not only do not refer to them, but rather claim to have discovered new insights into how sickle cell traits present. Professor Bela Ringelhann and I have summarized (with no less than 225 references) much of this and subsequent work.³⁰ Ignoring this material because much of it was published decades ago is doing a great deal of disservice not only to Medical Science but also to us West Africans 1 in 3 of whom is carrying a beta-globin gene variant (NORMACHE).

References

1   Kepron Charis, Somers Gino R, Pollamen Michael S.             Sickle Cell Trait Mimicking Multiple Inflicted Injuries in a 5-Year-Old Boy. Journal of Forensic Science Volume 54, No.5, pp 1141 t0 1145 September 2009.

2   Konotey-Ahulu FID. Morphine for painful crisis in sickle cell disease. Brit Med J 1991; 302: 1604

3   Mayor Susan. Enquiry shows poor care of patients with sickle cell disease. Brit Med J 2008; 336: 1152

4  NCEPOD (National Confidential Enquiry into Patient Outcome and Death) .. produced an 84-page report entitled ‘SICKLE: A Sickle Crisis? (2008)’ The Report (www.ncepod.org) ‘reviews the circumstances around deaths of in-patients with Haemoglobinopathies – sickle and beta-thalassaemia in the 21^st Century in England, Wales, Northern Ireland, and the off-shore islands’…’Nine out of the 19 patients with sickle cell disease who had pain on admission and who then died had been given excessive doses of opiods’’.  Death that was put down to “Acute Chest Syndrome” clearly was due to respiratory depression from the drugs which further produced in vivo sickling.

5   Konotey-Ahulu FID. Poor care for sickle cell disease patients: This wake up call is overdue BMJ Rapid Response May 28 2008 BMJ 2008; 336: 1152 http://www.bmj.com/cgi/eletters/336/7654/1152a#196224 to Susan Mayor “Enquiry shows poor care for patients with sickle cell disease” on National Confidential Enquiry into Patient Outcome and Death (NCEPOD) REPORT “SICKLE: A Sickle Crisis? (2008) info@ncepod.org

6   Vichinsky EP, Neumayr LD, Earles AN, Williams R, Lennette ET, Dean D, et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group. New England J Medicine 2000; 342(25): 1855-66.

7   Addae RO. Sickle cell trait and altitude. Br. Med J 1972; 1: 53.

8   Konotey-Ahulu FID. Beware of symptomatic sickle cell traits. Lancet 1992; February 29, p 555.

9   Witkowska HE, Lubin BH, Beuzard Y et al. Sickle cell disease in a patient with sickle cell trait and compound heterozygosity for haemoglobin S and haemoglobin Quebec-Chori. New England Journal of Medicine 1991; 325: 1150-1154. [Note that the title of this article is incorrect: Sickle cell trait cannot also be referred to as sickle cell haemoglobin Quebec Chori disease. The ‘AS’ pattern is sickle cell trait pattern, but the ‘A’ here is not a true ‘A’ but the new haemoglobin called Quebec-Chori, producing a disease phenotype, not a trait phenotype].

10   Boweman JE, Bernstein S. Caution about preliminary reports. Pediatrics 1977; 59: 639-640.

11   Konotey-Ahulu FID. “Percentage values of haemoglobins S, F, A₂, C, A in various sickle cell phenotypes, and consideration of the Sickle Cell Trait”, In The Sickle Cell Disease Patient: Natural History from a Clinico-epidemiological study of the First 1550 patients of Korle Bu Hospital Sickle Cell Clinic. Macmillan London 1992 & T-A’D Co Watford 1996, Chapter 30, pages 349 to 371.

12   Green RL, Huntsman RG, Serjeant GR. Sickle cell and altitude. Br Med J 1971; 4: 593-595.

13   Konotey-Ahulu FID. An International Sickle Cell Crisis. Ghana Medical Journal 1972; 11: 4-8.

14   Green RL, Huntsman RG, Serjeant GR. Brit Med J 1972; 2: 294

15  Konotey-Ahulu FID. Frequently Asked Questions (FAQs) in www.sicklecell.md or www.konotey-ahulu.com 2001 – 2011.

16   Aksoy M. Sickle cell trait in Southern Turkey. Lancet 1955; 1: 589-590.

17   Altay C, et al. Haemoglobin S and some other hemoglobinopathies in Eti-Turks. Human Heredity 1978; 28: 56-61.

18   Choremis  C et al. Sickle cell anemia in Greece. Lancet 1951; 1: 1147

19   Choremis C et al Blood groups of a Greek community with a high sickling frequency. Lancet 1957; 2: 1333-34

20   Djabanor F F T. The Sickle Cell Trait and Altitude. Brit Med J 1972. 1: 113.

21   Konotey-Ahulu FID. Insurance and genetic testing. Lancet March 3 1993, page 833.

22   Boweman J E. Ethical, legal and humanistic implications of sickle cell programs. INSERM 1975, 44: 353-378.

23   Lehmann Hermann. Sickle cell and flying. The Times (London), 4^th January 1972, editorial page.

24   Konotey-Ahulu FID. Four body guards and the perils of unmasking scientific truths. Brit Med J 2007; 335: 210-211, July 28.

25   Edington GM. Sickle cell anaemia in the Accra district of the Gold Coast. A review of 20 cases. Brit Med J 1953; 2: 957-961

26   Edington GM, Lehmann H. Expression of the sickle cell gene in Africa.  Brit Med J 1955a; 1: 1308-11

27   Edington GM, Lehmann H. Expression of the sickle cell gene in Africa. Brit Med J 1955b; 2: 1328

28   Edington GM, Lehmann H. The sickle cell gene. Am J Clin Path 1956a; 26: 553-56

29   Edington GM, Lehmann H. Sickle cell trait in Africa. Bull WHO1956b; 15: 837-852

30   Ringelhann B, Konotey-Ahulu FID. Hemoglobinopathies and thalassemias in Mediterranean areas and in West Africa: Historical and other perspectives 1910 to 1997 – A Century Review. Atti dell’Accademia dell Science di Ferrara (Milan) 1998; 74: 267-307.

Acknowledgements: I thank Professor Simon Dyson for drawing my attention to the article of Charis Kepron and colleagues. Professor Dyson has done much to alert people to the harm that unfair publications on sickle cell states can do. I recommend his websites for study.

Simon Dyson is Professor of Applied Sociology

Room 1.27 Hawthorn Building

De Montfort University

Leicester LE1 9BH

+44 (0)116 257 7751

sdyson@dmu.ac.uk

http://www.tascunit.com

http://www.sicklecelleducation.com

http://www.sicklecellanaemia.org

 

See for instance his book: Sickle Cell and Deaths in Custody

http://www.amazon.co.uk/Sickle-Deaths-Custody-Simon-Dyson/dp/1861771150

 

—————————————————————————————–

Re:Re: Luc Montagnier … and Andrew Wakefield: living parallel lives
Felix ID Konotey-Ahulu, Kwegyir Aggrey Distinguished Professor of Human Genetics University of Cape Coast Ghana
Consultant Physician Genetic Counsellor in Sickle Cell & Other Hemoglobinopathies 10 Harley St London W1G 9PF
Only fruitcakes believe in God? Correction of inverted inheritance of solomonic genius
British Medical Journal Rapid Response 12 May 2011 http://www.bmj.com/content/342/bmj.d2642/reply#bmj_el_260496

In my rushed rapid response to Mark Struthers’ remark (9 May) that believers in God were/are fruticakes [1] I made King David of Jerusalem the recipient of solomonic genius, when he was in fact the father of the legendary King Solomon. Sorry about that. David’s Psalms are still worth reading as they are full of extraordinary wisdom, including his published diagnostic observations in Psalm 14 verse 1, and Psalm 53 verse 1. Some will say King Solomon inherited the brilliance of his father, but Scripture implies something more profound, indeed something suprascientific. [3]
F I D Konotey-Ahulu MD FRCP DTMH
Kwegyir Aggrey Distinguished Professor of Human Genetics University of Cape Coast, Ghana
and Consultant Physician Genetic Counsellor in Sickle Cell and Other Haemoglobinopathies, 10 Harley Street, London W1G 9PF
felix@konotey-ahulu.com
Conflict of interest: Nothing to declare
1. Struthers Mark. Re: Luc Montagnier … and Andrew Wakefield: living parallel lives BMJ Rapid Response 9 May 2011 http://www.bmj.com/content/342/bmj.d2642/reply#bmj_el_260294
2. Konotey-Ahulu FID. Only fruitcakes believe in GOD? BMJ Rapid Response 11 May http://bmj.com/content/342/bmj.d2642/reply#bmj_el_260425
3. Second Book of Chronicles, Chapter 1 verses 7 to 12.
Competing interests: None declared
Submit rapid response
Published 12 May 2011

Rapid Response British Medical Journal 11 May 2001: Only fruitcakes believe in GOD?
Re:Re: Luc Montagnier … and Andrew Wakefield: living parallel lives
Felix ID Konotey-Ahulu, Dr Kwegyir Aggrey Distinguished Professor of Human Genetics University of Cape Coast, Ghana
Consultant Physician Genetic Counsellor in Sickle Cell & Other Haemoglobinopathies 10 Harley St London W1G 9PF

Only fruitcakes believe in GOD? http://www.bmj.com/content/342/bmj.d2642/reply#bmj_el_260425

Dr Mark Struthers (9 May) has a huge, huge problem. He said this: “I understand there are medical scientists – admittedly at the fruitcake end of the spectrum – who believe in God despite there being not a shred of scientific evidence, medical or otherwise of His existence” [1] Can Dr Struthers provide us with “a shred of evidence” that his brain is sharper than Blaise Pascal’s ever was?
THE GREATEST ACHIEVEMENT OF REASON
Pascal said “There are two excesses: to exclude reason, to admit nothing but reason. The supreme achievement of reason is to realize that there is a limit to reason. Reason’s last step is the recognition that there are an infinite number of things which are beyond it. It is merely feeble if it does not go as far as to realize that” [2]. And Blaise Pascal believed in God. He was no fruitcake.
DAVID MARTYN LLOYD-JONES
Or take Dr David Martyn Lloyd-Jones MB BS(Honours) MD MRCP who died the year Mark Struthers qualified from the University of Sheffield. Lloyd- Jones whom I knew personally, had a great brain, certainly not a fruitcake. His MD Thesis was on Sub-acute Bacterial Endocarditis after qualifying with Honours and Distinction from St Bartholomew’s Teaching Hospital. Lord Horder, Physician of King George and the Royal Household, and Consultant Physician at Bart’s picked Lloyd-Jones to be his Assistant, and they attended Royalty together. Dr Lloyd-Jones believed in God, and has more than 30 books in print – all of them on God. He was an amazing logician, orator, debater, with an unusual, analytical mind. He was no fruitcake.
KING DAVID
But I rather also admire that remarkably brilliant Hebrew King who reigned in Jerusalem. King David was brilliant, having clearly inherited what I have come to call “solomonic genius” from his father, the legendary King Solomon whose wisdom was proverbial, attracting people from the far corners of the earth to see and sample. King David has left us a treasure trove of wisdom in his Psalms, one of which (Psalm 119) contains 176 (one hundred and seventy six) verses. But the reason I name King David among those I am convinced are not fruitcakes, and yet believe in God, is this: King David is an amazing diagnostician. I sincerely advise Dr Mark Struthers to visit his local library and ask them to show him King David’s Psalms. The diagnosis the King makes in Chapter 14 verse 1 is spot on! (Even better than comparable diagnoses Sir Stanford Cade FRCS, Sir Richard Bayliss FRCP, Sir Arthur Bell FRCOG, and Sir Clement Price-Thomas FRCS, taught me to make when I was a medical student at Westminster Hospital School of Medicine in Horseferry Road, London SW1). Now, if after reading Psalm 14 verse 1, Dr Struthers wants a second opinion from King David, I suggest Psalm 53 verse 1.
SCIENTIFIC EVIDENCE
Dr Mark Struthers mentions “scientific evidence” [1] as if it was the pinnacle of all truth. My favourite Nobel Prize Winner in Medicine/Physiology is Professor Sir Peter Medawar. His book, “The Limits of Science” [3] is one that I suggest should be compulsory reading for those like Dr Mark Struthers approaching retirement from active Medical Practice and who think they “know it all” [4]
PRESIDENT BARACK OBAMA
By the way, does Dr Mark Struthers think President Barack Obama is a fruitcake? Cerebrally the man is head and shoulders above most people, quite apart from being a Nobel Laureate. And he believes in God.
Felix ID Konotey-Ahulu MD FRCP DTMH Kwegyir Aggrey Distinguished Professor of Human Genetics, University of Cape Coast, Ghana and Consultant Physician Genetic Counsellor in Sickle Cell and Other Haemoglobinopathies, 10 Harley Street, London W1G 9PF
Conflict of interest: Nothing to declare
1. Struthers Mark. Re: Luc Montagnier … and Andrew Wakefield: living parallel lives BMJ Rapid Response 9 May 2011 http://www.bmj.com/content/342/bmj.d2642/reply#bmj_el_260294
2. Pascal B. Pensees (1657). London: Penguin Books, 1966 (Translated by A Krailsheimer)
3. Medawar P. The Limits of Science. Oxford: Oxford University Press, 1985.
4. Konotey-Ahulu FID. The supra-scientific in clinical medicine: a challenge for Professor Know-All. BMJ 2001; 323: 1452-1453 22-29 December. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1121901
Competing interests: None declared [See next article with Correction]
Submit rapid response
Published 11 May 2011

Observations: Medicine and the Media: The other Twitter revolution: how social media are helping to monitor the NHS reforms
• Martin McKee,
• Katie Cole,
• Louise Hurst,
• Robert W Aldridge,
• Richard Horton
BMJ 342:doi:10.1136/bmj.d948 (Published 16 February 2011)

Facebook and Twitter in bid to create African Scientific Revolution “mizraimically”.
o Felix ID Konotey-Ahulu, Kwegyir Aggrey Distinguished Professor of Human Genetics University of Cape Coast Ghana Consultant Physician Genetic Counsellor in Sickle Cell & Other Haemoglobinopathies 10 Harley Street

The Twitter Revolution that Professor Martin McKee and colleagues (February 12) showed was “helping to monitor NHS reforms” [1] is but one example of the enormous potential of social networking. Since the Obama phenomenon which made use of this to spread critical information to a huge number of people in no time at all, social networking has been used to achieve the hitherto unachievable. In short, social networking has precipitated revolutions the latest of which is the Egyptian Friday 11th February 2011 Cairo’s Tahrir Square Revolution which achieved the hitherto unachievable through the organizational grass roots use of Facebook and Twitter.
AFRICAN SCIENTIFIC REVOLUTION ACHIEVABLE “MIZRAIMICALLY”.
The title of The 5th TWAS-ROSSA Young Scientists Conference (26th – 27th February 2011) which took place at the Hilton Hotel, Nairobi last week [2] was “Exchanging Knowledge on Climate Change Impacts and Vulnerability in Africa: The Role of Networking”. Summing up that remarkable Conference which covered subjects from climate change effects on agriculture, flood risk management, institutional networks for exchange of knowledge, cryobiology, local community knowledge of and adaptation to climate change, etc Dr Eng Shem Arungu-Olende, Executive Director of the African Academy of Sciences hit the nail on the head when he said “Promoting net working does not have to be formal … very exciting research can be communicated from individual to individual, from individual to institutions, and from institution to institution”. The exciting process, said Arungu-Olende, “can generate knowledge and disseminate that knowledge for quite awhile” [2]. This was also emphasized by Professor Mohamed H A Hassan, Executive Director Emeritus of the Trieste based TWAS (Academy of the Developing World) and President of the African Academy of Sciences. Networking that produces an unexpected and impressive result where despair existed before, is what I now describe as a revolution achieved “mizraimically”. Mizraim is what my Krobo tribes people call Egypt, a name originating from biblical times. [3] No where is such a revolution more urgently needed than in the most pressing health issue on the African Continent at the moment.
TACKLING THE SUB-SAHARAN HIV-AIDS MENACE “MIZRAIMICALLY”
(1) “Nearly 1000 babies are born every day in sub-Saharan Africa” is the British Medical Journal’s headline a mere 3 months ago. [4]
(2) “Children from Zevenfontein (South Africa) where 85 per cent of the community are HIV Positive” [5] – Financial Times, London.
To many outside Africa (and even within Africa) this situation produces reactions such as “Normal, of course”; “Not surprising at all”; “What else do you expect?” ; “If we in the Congo don’t change our sexual habits the Congo could be wiped off the map” [6], and “Africa left to the lions” [7] and so on and so forth. Therefore, I ask, does sex alone account for 1000 HIV Positive babies a day born to 1000 mothers a day who must be HIV Positive, and the implied 1000 a day HIV Positive men linked to these women? But to me, and to many fellow Africans the situation calls for a Revolutionary Approach, and hence our great fortune to have Facebook, Twitter, and LinkedIn to help begin this process “mizraimically”! I said as much during my Award Lecture last Saturday (26 February) in Nairobi. When TWAS-ROSSA (Academy of Sciences for the Developing World’s Regional Office for South Saharan Africa) announced last August in Hyderabad, India, that I had been awarded the “2010 TWAS REGIONAL PRIZE ON PUBLIC UNDERSTANDING AND POPULARIZATION OF SCIENCE” [8] I was told that when collecting the Award in Nairobi in February 2011 I would be expected to give a Lecture. Well, I thanked God and took courage, and gratefully announced that I would give the following Double Lecture, one after the other immediately on receipt of the Award:
Public Understanding and Popularization of Science as illustrated by (i) The most prevalent hereditary affliction in sub-Saharan Africa – Sickle Cell Disease and Other Haemoglobinopathies (ii) The most serious acquired affliction in sub-Saharan Africa – HIV/AIDS
Which was what I did last week when I used the first lecture to introduce my recent invention of the ‘kanad’ as a novel tool for genetic counselling and voluntary family size limitation (GCVFSL) in Sickle Cell Disease and Other Haemoglobinopathies [9]. The second lecture concentrated on Africa’s AIDS Catastrophe, and how Facebook, Twitter, LinkedIn could begin to spread the word round, alarm our kith and kin in the Diaspora, and begin a revolution.
SUMMARY OF AIDS LECTURE AT TWAS-ROSSA CONFERENCE NAIROBI
1. One thousand HIV Positive babies born every day in sub-Saharan Africa.
2. 85% of the Zevenfonteirn Community in South Africa are HIV- Positive
3. And this in spite of Global Funds into Africa?
4. In spite of our Ministries of Health and Colleges of Physicians and Surgeons working flat out?
5. Despite WHO’s thundering the “Wear Condoms” and “Stick to one woman” advice?
6. Have top to bottom (ie Globalisation) Health Schemes failed?
7. Was it not time to start a bottom-up (ie from grass roots up) health management approach using our Traditional Chiefs?
8. What happened to Professor Kihumbu Thairu’s grass roots upwards approach emanating from the impressive Symposium organised jointly by The Commonwealth Secretariat and the Kenya Medical Research Institute (KEMRI)? [10] Was Thairu’s approach tried and found wanting?
9. Meaningful Research: Is it not time for every one of us, especially the Young Scientists gathered here, to use Epidemiology
(a) to find out what is happening on our dear Continent to give rise to 1000 HIV Positive babies every single day and to determine how it is possible for 85% of the Zevenfontein Community in South Africa to be HIV Positive when Professor Metz had written to me from Pretoria less than 25 years ago that AIDS (or VIGS as he called it in Afrikaans) among the Blacks was virtually nil? [11] And
(b) To communicate such findings expeditiously by Facebook, Twitter, LinkedIn not only to our African Parliamentarians and Tribal Chiefs but to Africans abroad?
I defined ‘Epidemiology’ in the Nairobi Lecture exactly as I had done in the British Medical Journal after fact finding tours of Africa. Epidemiology, to me, is finding answers to the six questions What? When? Where? Which? How? Why? I told the Young African Scientists that armed with little more than a note book and pen, finding answers to these questions at the grass roots level will provide more information about our plight [12] than dissecting genes of baboons and humans in the laboratory. (I was referring to the misinformation/disinformation that two eminent Harvard University Professors came out with when they concentrated rather on splitting genes in the lab, namely that Senegalese prostitutes harboured antibodies to monkey virus in their blood [13], when in fact there was no truth whatever in their publication! It took another respectable Harvard Professor Carol Mulder to write an editorial to redeem the reputation of that great Institution: “A case of mistaken non- identity” [14]). Though Kenyan scientists have been known to be able sequence the genome of organisms very rapidly, our efforts should be directed rather towards using Epidemiology as I have defined above to arrive at truth very quickly.
10. The African Academy of Science [15] is without doubt a force to reckon with (being robustly supported by the Trieste based Academy of Sciences for the Developing World, TWAS [16], and it benefits from the Network of African Scientific Organisations (NASO). The Academy stands ready to use its “Quarterly Journal of Discovery and Innovation” to disseminate new information. It is ready to resume on-line publishing soon, so I urge that in keeping with the title of the Quarterly Journal the young scientists should aim at going on line immediately and, as I concluded my lecture, “aiming to publish new Ideas, new Findings, new Treatments, new Discoveries, and new Approaches which will command the attention of the rest of the world”.
MONITOR HEALTH PROGRAMMES AND DISSEMIATE INFORMATION
Professor Martin McKee and team [1] are using Twitter and other social media “to monitor NHS reforms”. We in Africa should similarly monitor everything from vaccinations, recommended drugs, official declarations from above, not to mention “Global Programmes”. I have queried why the Maasai in Kinyawa, Kenya, the Luhya in Wabuye, Kenya, and the Yoruba in Nigeria have been genome sequenced anonymously in the Global Sequencing Programme, and yet the international gene sequencers claim they got “informed consent” to do the work [17-20]. African scientists need to find out what exactly is happening. Have some African genes been patented anonymously yet “with informed consent” as the researchers claimed?
All foreign scientists do not have the same ethical calibre. [21] Some feel Africa’s pain; others do not, and we need to distinguish between the two, and work with those who identify with us. I once pointed out after Didier Fassin and Helen Schneider’s amazing article in the British Medical Journal on AIDS in South Africa [22] that scientists with Nazi proclivities did not disappear when Hitler did. [23]. Professor George Fraser and Dr Berno Muller-Hill said as much [24]. Matters that had been dismissed as Conspiracy Theories have now been proven to be Conspiracy Facts leading to open apologies by two living American Presidents, Bill Clinton and Barack Obama [25, 26, 27]. It is a most heartening fact that an increasing number of African Tribal Chiefs are computer literate and will be happy to be kept in the social networking loop. Their subjects prefer to listen to them to being fed top to bottom pronouncements – see how Ghanaian Health Officials were forced to come out to answer questions about serious side effects of Artesunate-Amodiaquine Combination Therapy [28]. Ghanaian market women and roadside vendors are all social networking, while Kenyans are doing their banking business through mobile phones, bypassing the conventional High Street Banks. Scientists in Africa need also to make use of these social networking media to create a health revolution on the Continent.
Felix Konotey-Ahulu MD(Lond) FRCP DTMH FGA FTWAS FWACP FGCP Kwegyir Aggrey Distinguished Professor of Human Genetics University of Cape Coast, Ghana and Consultant Physician Genetic Counsellor in Sickle Cell and Other Haemoglobinopathies, 10 Harley Street, London W1G 9PF.
1 McKee Martin, Cole Katie, Hurst Louise, Aldridge Robert W, Horton Richard. The Other Twitter revolution: how social media are helping to monitor NHS reforms. BMJ 2011; 342: d948 doi: 10.1136/bmj.d948 18 February 2011. http://www.bmj.com/content/342/bmj.d948.full
2 TWAS-African Academy of Sciences The Fifth Scientists’ Conference on “Exchanging Knowledge on Climate Change Impacts and Vulnerability in Africa: The Role of Networking” 26th – 27th February 2011 – Preceded by the NASAC-KNAW Conference on Impact of and Adaptation to Climate Change in Relation to Food Security in Africa 23rd – 25th February 2011. Hilton Hotel Nairobi, Kenya.
3 The Holy Bible. “And the sons of Ham: Cush, and Mizraim (Egypt), and Phut, and Caanan.” Genesis Chapter 10 verse 6. The King James Authourized Version, 1908 Edition – 60th Printing. B B Kirkbride Bible Co, Inc. Indianapolis, Indian, USA 1964.
4 Zarocostas John. Nearly 1000 babies a day in sub-Saharan Africa are infected with HIV. BMJ 2010; 341: c6937 doi: 10.1136/bmj.c6937. Dec. 1 http://www.bmj.com/content/341/bmj.c6937.full
5 Financial Times, London. AIDS in South Africa. Zevenfontein where 85% of the Community are HIV Positive. Friday 20th September 2002, page 14.
6 Finch Scott. The Ravage of AIDS in Africa. BBC World Service: Science In Action, Sunday 18 October 1987, GMT 09.15 to 09.45.
7 Veitch Andrew. How to avoid catching AIDS. The Guardian, London, November 21 1986, page 21.
8 TWAS (Academy of Sciences for the Developing World) Regional Office for sub-Sahran Africa – Announcement in Hyderabad 20 October 2010 http://twas.ictp.it/news-in-home-page/news/twas-regional-prizes-for-public -understanding-and-popularization-of-science
9 The ‘kanad’ in www.sicklecell.md/bio or www.konotey-ahulu.com/bio 2010. The kanad in genetic counselling and voluntary family size limitation (GCVFSL) in sickle cell disease and other haemoglobinppathies.
10 Thairu Kihumbu. Editor Symposium/Workshop Appropriate Technologies for AIDS Management in Africa 3-7 September 1990, Kenya Medical Research Institute, Nairobi with Commonwealth Secretariat SW1 London.
11 Konotey-Ahulu, FID. What is AIDS? Tetteh-A’Domeno Company, Watford, England, 1989, 227 pages ISBN: 0 9515442 0 9
12 Konotey-Ahulu FID. Clinical epidemiology, not seroepidemiology, is the answer to Africa’s AIDS problem. BMJ (Clin Res Ed) 1987; 294(6587): 1593-1594. http://www.bmj.com/cgi/reprint/294/6587/1593.pdf [PubMed – indexed- for MEDLINE (June 20 1987) doi:10.1136/bmj.294.6587.1593]
13 Essex Max, Kanki Phyllis. Comparison of simian immunodeficiency virus isolates. Nature 1988; 331: 621-22
14 Mulder Carol. A case of mistaken non-identity. Nature 1988; 331: 562-63.
15 African Academy of Sciences. http://www.aasciences.org P O Box 24916, Nairobi, Kenya. [Retriring President Emeritus is Professor Mohamed H A Hassan; Incoming President is Professor Ahmahdou Lamine Ndiaye]
16 TWAS. The Academy of Sciences for the Developing World is an autonomous international organization based in Trieste, Italy, that promotes scientific excellence for sustainable development in the South. http://www.twas.org [Retiring Executive Director Emeritus is Professor Mohamed H A Hassan to be succeeded on 28 February 2011 by Professor Romaine Murenzi]
17 Announcement: http://www.1000genomes.org/files/1000Genomes- NewsRelease.pdf International Consortium Announces the 1000 Genomes Project. Major Sequencing Effort Will Produce Most Detailed Map of Human Genetic Variation to Support Disease Studies. (Tuesday January 22 2008).
18 Wise Jaqui. Consortium hopes to sequence genome of 1000 volunteers. British Medical Journal 2008; 336; 237 doi: 10.1136/bmj.39472.676481.DB http://www.bmj.com/content/336/7638/237.1/full January 31 2008
19 Konotey-Ahulu FID. The Human Genome Diversity Project: Cogitations of An African Native. Politics and the Life Sciences (PLS) 1999, Vol 18: No 2, pp 317-322. [Invited Commentary on Professor David Resnik’s article: The Human Genome Diversity Project: Ethical Problems and Solutions.] PMID: 12561789 [PubMed – indexed for MEDLINE]
20 Global Genome Sequencing: Some Ethical Considerations. In Howard University National Human Genome Center Post-Inaugural Symposium on “1000 Genomes Project: On the Frontier of Personalized Medicine” at Ralph J Bunch International Affairs Center, Howard University, 2218 Sixth Street, NW Washington, District of Columbia, USA January 23, 2009. http://www.howard.edu/calendar/main.php?calendarid=medicine&view=event&eventid=1232140247442&timebegin=2009 -01-23
21 Konotey-Ahulu FID. Clinical Genetics: Ghanaian gratitude for British and Hungarian contributors – A personalised historical perspective Ghana Med J 2009; 43: 175-178. [Special Article December No. 4] http://www.ghanamedj.org/articles/December2009/Fina%20Special%Article%20Clinical%20Genetics.pdf
22 Fassin Didier, Schneider Helen. The politics of AIDS in South Africa: beyond the controversies. BMJ 2003; 326: 495-97 (1 March) http://www.bmj.com/content/326/7387/495.full doi;10.1136/bmj.326.7387.495
23 Konotey-Ahulu FID. Wake up call and need for paradigm shift. Brit Med Journal 2003 ‘Rapid Response’ to Didier Fassin and Helen Schneider’s article opened up for Education and Debate: – The politics of AIDS in South Africa: beyond the controversies. Brit Med J 2003; 326; 495- 497 (1 March 2003). http://bmj.bmjjournals.com/cgi/content/full/326/7387/495 or/& http://www.rethinking.org/bmj/response_30917.html
24 Muller-Hill Berno. Murderous Science: Elimination by Scientific Selection of Jews, Gypsies, and Other – Germany 1933- 1945 [Translated from German by George R Fraser] Oxford University Press, Oxford 1988.
25 Clinton President WJ. Apology on behalf of the American government to survivors of the Tuskegee Syphylis Experiment victims. Worldwide radio & Television. May 16 1997. Also Ken Getz; Tuskegee, a Cloud Over Research. The Washington Post. Tuesday, Sept 30, 2008. President Clinton publicly apologized to the eight surviving participants of the shocking and unethical study, saying “What the United States Government did was shameful”.
26 Tanne Janice Hopkins. President Obama apologizes to Guatemala over 1940’s syphilis study. BMJ 2010; 341.c5494 doi:10.1136/bmj.c5494 http://www.bmj.com/content/341/bmj.c5494.full October 9, page 750.
27 Konotey-Ahulu FID. President Obama apologises over Guatemala syphilis study: International cooperative research in jeopardy. http://www.bmj.com/content/341/bmj.c5494.full/reply#bmj_el_243183 BMJ Rapid Response Oct 17 2010
28 Amofah G. Furore over Artesunate-Amodiaquine Combiantion (ACT) Drug. Daily Graphic, Accra. Monday May 5, 2006, page 23.
Conflict of Interest: None declared felix@konotey-ahulu.com
Competing interests: None declared

 Rapid ResponsesNews: New appointment to drug advisory body sparks controversy

• Clare Dyer

BMJ 2011;342:doi:10.1136/bmj.d624 (Published 31 January 2011)

1. http://www.bmj.com/content/342/bmj.d624/reply#bmj_el_249481

“New appointment of evangelical Christian to drug advisory body    sparks controversy” Please spare us emotive headlines!

• Felix ID Konotey-Ahulu, Kwegyir Aggrey Distinguished Professor of Human Genetics University of Cape Coast, Ghana

Consultant Physician Genetic Counsellor in Sickle & Other Haemoglobinopathies 10 Harley St London W1

“New appointment of evangelical Christian to drug advisory body sparks controversy” Please spare us emotive headlines!

The term “sparks controversy” [1] in Clare Dyer’s headline (5 February, page 300) made me think (perhaps naively) that the article would describe something like a miniature Cairo’s Tahrir Liberation Square problem that demanded some extraordinary intervention to prevent catastrophe. Nothing of the sort, as it turned out.

ONE MAN PLUS ONE OTHER PERSON

It was just one man, Evan Harris, plus an “unnamed member of the council” who were not at all happy about Theresa May, the Home Secretary, approving the appointment of an “evangelical Christian” to the “UK Advisory Council Drug’s Council on the Misuse of Drugs” [1]. Perhaps I read too much into the headline, but I fail to see how the appointment of one, yes I mean one, “evangelical Christian”, to the Drugs Advisory Council with its 9 new members must be required to meet with the approval of Mr Harris. Cannot the other 8 new members over rule the “evangelical Christian” whenever they think the latter is talking (tafracher) nonsense? [2]

THE MERIT QUALIFICATION AND EVIDENCE-BASED VIEWS

Clare Dyer states that these appointments “were made under a code of practice that requires all appointments to be made on merit” [1].Can it be shown that Dr Hans-Christian Raabe is devoid of the merit that membership of such an important Drugs Council will benefit from? The Council “prides itself on basing all views on evidence” [1] Would Evan Harris cooperate with me in designing an epidemiological research project among teenagers in the UK and elsewhere to see what proportion of drug abusers were evangelical Christians, and what proportion were not, compared with the rest of the teenage population? Would the findings, significant to a ‘p’ value of 0.0001, prove anything to him and his like-minded colleagues? If a known chain smoker, as I once was, had been appointed to the Council by Theresa May would Harris have displayed similar misgivings?

PROFESSOR DAVID NUTT’s SACKING AND THE RESIGNATIONS

The British Medical Journal [3] conducted some research among its readers asking who agreed or disagreed with the sacking of Professor David Nutt by Mr Alan Johnson. As of 5th November 2009 443 respondents castigated Mr Alan Johnson for dismissing his Chief Scientific Advisor, while 83 (15.8%) agreed with his decision to sack him. Did Evan Harris think all these 83 were “evangelical Christians”? In my response to the discussion on that occasion I described two Fellows of the Royal Society (both of them alive today) who wrote best sellers on Human Genetics [4]. They both used the description in the BMJ of a genetic defect of mine in their text books, but while Professor Sir David Weatherall FRS emphasized the ethical point I was making about my Mendellian Dominant defect [5], the other Fellow of the Royal Society did not even mention the word Ethics once in his 347-page book, nor did he acknowledge the BMJ (and myself) as the source of his information, which proves that some brilliant scientists forget that Science is not the only criterion required in dealing with human situations. The Drugs Council may be packed with brilliant evidence-based scientists some of whom may be Fellows of the Royal Society, but I would not quarrel with Theresa May for including at least one new person who is an evangelical Christian. If some members want to resign because of this, let them. I trained in the UK, and I happen to know that Great Britain is not short of geniuses who can happily step into the shoes of “the departed”. Theresa May probably thinks there is an ethical dimension required in the advice given regarding addictive drugs and teenagers. In my opinion Theresa May deserves commendation, not condemnation.

Conflict of interest: I am a staunch believer in The LORD JESUS CHRIST.

Felix I D Konotey-Ahulu MD(Lond) FRCP DTMH
Kwegyir Aggrey Distinguished Professor of Human Genetics, University of Cape Coast, Ghana and Consultant Physician Genetic Counsellor in Sickle & Other Haemoglobinopathies, 10 Harley Street, London W1G 9PF

felix@konotey-ahulu.com

1 Dyer Clare. New appointment of evangelical Christian to drug advisory body sparks controversy. BMJ 2011; 342: d624 (5 February, page 300)

2 Konotey-Ahulu FID. Tafracher – Personal View. The invaluable Ghanaian word for devulgarizing succeeding words or phrases. BMJ 1975; 1(5953): 329. (February 8) doi:10.1136/bmj.1.5953.329 http://www.bmj.com/cgi/reprint/1/5953/329.pdf & http://www.ucc.edu.gh/node/258

3 Dyer Clare. Scientists want more protection after government adviser is sacked. BMJ 2009; 339.doi: 10.1136/bmj.b4563 (November 4)

4 Konotey-Ahulu FID. Does rejecting a particular scientific opinion mean a rejection of Science? BMJ Rapid Response 10 November 2009. http://www.bmj.com/cgi/eletters/339/nov04_1/b4563#224533

5 Weatherall DJ. Ethical issues and related problems arising from the application of the new genetics to clinical practice. Chapter 12 in D J Weatherall. The New Genetics and Clinical Medicine in Practice. Third Edition. Oxford, Oxford University Press 1991, pages 346-368.

Competing interests: None declared

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Published 6 February 2011

1. http://www.bmj.com/content/342/bmj.d624/reply#bmj_el_249435

Controversy or just plain prejudice?

• Trevor G Stammers, Programme Director in Medical Ethics

St Mary’s University College, London

As director of a centre for evidence based policy, Evan Harris should be well aware of the evidence that men who have sex with men do indeed have a higher incidence of drug misuse (1)(2)(3). Indeed if they do not, then surely Harris’ criticism of Dr Raabe’s alleged comments on homosexuality is entirely irrelevant to Raabe’s position on the Misuse of Drugs Advisory Council?

As for Mark Easton’s reported comments from an unnamed member of the Council threatening to resign over Dr Raabe’s appointment, these say so much about that member’s cowardice and lack of transparency that it may be best if they do resign. At least Dr Raabe has the courage to express his views openly.

1. Stall R, Wiley J. A comparison of alcohol and drug use patterns of homosexual and heterosexual men: the San Francisco men’s health study . Drug Alcohol Dependency. 1988;22:63-73.

2. Stall R, Paul JP, Greenwood G, et al. Alcohol use, drug use and alcohol-related problems among men who have sex with men: the Urban Men’s Health Study. Addiction. 2001;96:1589-1601.

3. McCabe S et al Sexual identity and substance misuse among undergraduate students Substance Abuse 2003 24 77-91

Competing interests: I am also a Christian and former GP

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Published 6 February 2011

« Parent article

Music (and Tonal Language) and the art of being human – III – Published 6 January 2011
o Felix ID Konotey-Ahulu, Dr Kwegyir Aggrey Distinguished Professor of Human Genetics University of Cape Coast Ghana

Consultant Physician Genetic Counsellor in Sickle Cell & Other Haemoglobinopathies 10 Harley St Lond
Dr John Matthews (Dec 11, p 1274) has shown that, contrary to popular perception, a doctor can have interests far beyond the consulting room. His most excellent review [1] of Philip Ball’s book covering music and the brain [2] deserves a 3-part response: Part I dealt with Music, the brain, and language. Part II tackled Origins and what it means to be human. Part III now suggests some meaningful research approaches.

MEANINGFUL RESEARCH APPROACHES
If Science is not equipped to determine how language originated, or how music inspires and gives balm to troubled minds, does that mean there cannot be meaningful research here? Not at all! What this means is that I should not spend my time investigating “how language evolved”, but rather relating what we already know in Music and Language, especially Tonal Linguistics, to what Dr Matthews indicates could be various brain responses through “functional magnetic resource imaging and positron emission tomography” [1], not to mention novel tools for probing the science of Glossogenetics.
Last year I discussed with Professor George Ebow Bonney and Professor Gloria Dunston of the Howard University National Human Genome Centre in Washington DC, USA, how an interdisciplinary project involving Tonal Linguistics, Music, Neurophysiology, History, Geography, Psychology, Anthropology, Glossogenetics, Genome Sequencing, Informatics, and even Egyptology [18] could be initiated. Professor Ayi Kwei Armah, the brilliant Ghanaian author and expert in Hieroglyphics has discovered that the ancient Egyptian name for parrot is “akoo” which is identical to what we call the bird in no less than 8 tribes in West Africa, from Yoruba land in Nigeria , right across to Ghana (Ewe, Krobo-Dangme-Ga, Twi, Fante) and up to Senegal [19]. We must research to find what other ancient Egyptian words are found in black African tribes. [18] Here is another interesting fact: Just the two letters ‘f’ and ‘a’ of the Ga alphabet can be used (with my sound colouring technique) to express reproducibly no less than 75 words, phrases, and sentences in the language, capable of being read by anyone who can sing Tonic Solfa [20]. The phenomenon of mid pitch arrest in Krobo-Dangme [3 4 6 18 20] and of lower mid pitch arrest in Ga [21] is not found in English, but I have discerned the former in Yoruba and in Kikuyu [20]. Research will reveal which other African tribes, or indeed ethnic groups world-wide display this phenomenon. How are they related genetically?
Is the Japanese genome related in any way to the Krobo genome because Nihongono phonates exactly like the Krobo-Dangme “e ji lolo” which means “she/he has not yet left”? When both are hummed, or sung, there is no acoustic difference whatever, and the Tonic Solfa of both is identical, s (low pitch) s (octave high) m m showing no difference between the Japanese and Krobo/Dangme intonations. [3 20]. Please try singing s (low) s (high) m (mid pitch) m (mid pitch) and you would have hummed Japanese and my native Krobo language. Any BMJ reader should be able to do this. It is that easy. These research projects in Linguistics/Tonal Linguistics could be as fruitful in shedding light on brain function, as those that Dr Matthews would like done in Music. If I phonated in my head (ie inaudibly) an arrested Krobo vowel in mid pitch (m) and then dropped 2 semitones to a Ga lower mid pitch vowel (r) would I be able to capture the vowels on a brain scan? Would it be possible to devise a “lie test” for interrogating a suspect who refused to answer questions, using a scan to detect silent tonal language sounds in the head? I can hum Tonic Solfa in my head, and arrest pitches at certain levels as I wish. In Krobo-Dangme the word ‘bo’ pronounced in mid pitch means cloth, but pronounced with low pitch means “to rub”. Will it be possible to record these pitches accurately even without phonating them aloud?
If, with Julie Andrews in “Sound of Music” [22], you can begin to sing “doe, a deer, a female deer…” you will, with the first note, have struck the invaluable 3rd mid pitch in Krobo/Dangme-Ga which, substituting a ‘y’ for Julie’s ‘d’, phonates exactly like the affirmative word “yes” in the language [20]. My colour for this 3rd mid pitch (d) which is also 2 semitones below lower mid pitch (r) is brilliant green [20]. When this particular pitch is arrested to sing Julie Andrews’ ‘doe’ (but with a ‘y’) the resulting word ‘yoe’ means ‘yes’ [20]. Which other tribes have a fixed, reproducible-in-tonic-solfa pitch for “yes”? Would the tribes be related genetically? The avenues for profitable research are protean [23]. Braille can be developed to discern pitch colour for the blind, and the coloured vowels for pitch are ideal for the deaf and tone-deaf. It can be truly said that my entire tribe has perfect pitch in more senses than one: anyone can go straight to a particular pitch in Tonic Solfa without reference to any preceding or succeeding note. Is this the reason why Aficans and African Americans are so musical?

CURRENT GENOME SEQUENCING CONSORTIUM AND LANGUAGE RESEARCH
The present on-going Human Genome Sequencing exercise which Professor George Bonney, Professor Gloria Dunston and I discussed last year [18] could be used to probe some of these aspects of language (and music), remembering at least 3 caveats:
(i) Researchers need to be careful not to miss the genomic wood for the DNA trees
(ii) Nature and nurture must be kept in perspective. There is what I call a quadrilateral interpretation of findings: (a) what is observed is due to nature (genes) alone (b) findings are the result of environment (nurture) alone (c) what you observe is due to both nature and nurture interaction (d) nature and nurture have little to do with the facts.
(iii) Avoid the mistake of deciding exactly what genome sequencing is going to reveal before the work is done [24]. To decide ahead that one tribe’s appreciation of music is more “civilised” than another is to make a serious mistake. This is why in African Anthropogenetics, with all the linguistic ramifications, to do genome sequencing anonymously as is happening now is not at all the right way forward. [25 26 27]

WONDER OF THE INTERNET
If you find all this difficult, take heart because youcan read, and see, and hear it all on line – free of charge. Fortunately, because of the internet anybody reading this can verify the truth of what I have been saying. One can read the colours of the sound pitches, and even phonate the Tonic Solfa of all 75 Ga words, phrases, and sentences formed with the letters ‘f’ and ‘a’ by just clicking http://bit.ly/bEdcc4. [20] One can also verify that the non-tonal English word “agriculture” which, though capable of being understood when pronounced entirely in monotones, nevertheless has 4 quantifiable pitches in Queen’s English; one is invited to down load the 42-page information with colour, free of charge. [20] Indeed, the internet allows me to demonstrate (vocally) to any reader of the BMJ what the Tonic Solfa of my Millennium Hymn [28] sounds like, and (visually) to see me in the flesh singing the hymn while the Tonic Solfa flashes on the screen. Readers may even see me piano-playing the Millennium Hymn all 7 verses of which appear on the screen by typing http://bit.ly/cRrZ0s [28]. As each of the verses ends with the word ” Bethlehem ” I take this opportunity to wish BMJ readers A HAPPY CHRISTMAS AND A HEALTHY NEW YEAR!
F I D KONOTEY-AHULU FGA MD(Lond) FRCP(Lond) FGCP DTMH(L’pool) FTWAS FAAS FWACP ORDER OF THE VOLTA GHANA felix@konotey-ahulu.com
18 Konotey-Ahulu FID. Global Genome Sequencing: Some Ethical Considerations. In Howard University National Human Genome Center Post- Inaugural Symposium on “1000 Genomes Project: On the Frontier of Personalized Medicine”. Washington , DC January 23 2009 http://bit.ly/gs65RD .
19 Armah Ayi Kwei. The Eloquence of the Scribes – a memoir on the sources and resources of African literature. PER ANKH 2006. Popenguine. Senegal (page 190)
20 Konotey-Ahulu FID. Tonic Solfa Is The Foundation Of Tonal Linguistics. DRUMSPEAK (Supplement) International Journal of Research in the Humanities. New Series Volume 3. No. 1, May 2010. Faculty of Arts, University of Cape Coast , Ghana . ISSN (0855-9945). [Lecture given on Monday 12 October 2009, University of Cape Coast in the Occasional Lecture Series during the 40th Congregation. [Publications Unit, University of Cape Coast, Ghana] http://www.bit.ly/bEdcc4 (Down load Free).
21 Konotey-Ahulu FID. Discoveries and New Insights into Tonal Linguistics Facilitate Reading of Mother Tongue: Introducing The Ephraim Amu Principle. The 10th Dr Ephraim Amu Memorial Lecture – Ghana Academy of Arts & Sciences at The British Council, Accra , 13th May 2010. [Report by Dr Doris Yaa Dartey: Ghanaian Times Friday 21 May 2010 “New insight into ‘Tonal Linguistics’ at the 10th Ephraim Amu Memorial Lecture in Accra by Dr [Konotey-Ahulu – ‘Colour the sounds to differentiate the pitches’ – Pregnant women need folic acid to prevent babies with cleft palate’ ]. Video is available.
22 Andrews Julie. The Sound of Music. Broadway Musical 1959. Film Musical 1965.
23 Konotey-Ahulu FID. The Human Genome Diversity Project: Cogitations of An African Native. Politics and the Life Sciences (PLS) USA 1999, Vol 18: No 2, pp 317-322. Symposium in PMID: 12561789 PubMed indexed for MEDLINE
24 Verkaik Robert. Revaled: Scientist who sparked racism row has black genes. The Independent, London , December 10, 2007.
25 Announcement: International Consortium Announces the 1000 Genomes Project. Major Sequences Effort Will Produce Most Detailed Map of Human Genetic Variation to Support Disease Studies. (Tuesday January 22, 2008). http://www.1000genomes.org/files/1000Genomes-NewsRelease.pdf
26 Wise Jaqui. Consortium hopes to sequence genome of 1000 volunteers. BMJ 2008; 336: 237, Feb. 2.
27 Konotey-Ahulu FID. There is but one human race. New African, London . Dec. 2009, page 4.
28 Konotey-Ahulu Felix ID. Millennium Hymn: Time Was Created. Words (7 verses) http://bit.ly/cRrZ0s
Competing interests: None declared
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Published 20 December 2010
3.
Music (and Tonal Language) and the art of being human – II
o Felix ID Konotey-Ahulu, Dr Kwegyir Aggrey Distinguished Professor of Human Genetics University of Cape Coast Ghana
Consultant Physician Genetic Counsellor in Sickle Cell & Other Haemoglobinopathies 10 Harley St Lond
Dr John Matthews (Dec 11, p 1274) has shown that, contrary to popular perception, a doctor can have interests far beyond the consulting room. His most excellent review [1] of Philip Ball’s book covering music and the brain [2] deserves a 3-part response: Part I dealt with Music, the brain, and language. Part II tackles Origins and what it means to be human. Part III suggests meaningful research approaches.

THE ART OF BEING HUMAN
A fundamental part of being human, as John Matthews and Philip Ball emphasize, is to have a brain that is capable of producing music and language. But as the nightingale also sings and the musical chatter of the black bird (Tardus merula) between April and September in the northern hemisphere is capable of being written down in Tonic Solfa as I have done annually in recent years, I can boldly say that producing language, even more than producing music, is the one most characteristic feature of being human. Animals, including birds, can make music, and communicate intelligently, but only human beings have language with all its five levels for communicating information, namely Statistics, Syntax, Semantics, Pragmatics, and Apobetics (thanks to the insight of such experts as Claude Shannon, Noam Chomsky, and Werner Gitt) [9-11]. This is why I have added in brackets “Tonal Language” to the title of Dr Matthews’ article which includes the term “being human”.

THE ORIGIN OF LANGUAGE
Dr Matthews states that “music could hardly be there by chance”. A greater truism is hard to find. He mentions “the perplexing problem of music’s origin” and goes on: “We do not know how or why music started, what the predisposing factors were, or even the form in which the earliest manifestations evolved.” [1] After looking for “a selection advantage” related to “community bonding” Dr Matthews concludes: “We are left surmising”. Well, I turn to 2 famous Nobel Laureates in Physiology/Medicine who, thinking scientifically, tackled the problem of “origins”. First, I turn to Sir Francis Crick (who better?). In a brilliant 13-page scientific exposition to crack “The origin of the genetic code”, Crick was out of his depth, and he admitted as much. [12] Like Dr Matthews, he was “left surmising” [1], so much so that 13 years later the great Francis Crick was constrained to write: “An honest man, armed with all the knowledge available to us now, could only state that in some sense, the origin of life appears at the moment to be almost a miracle. …”. [13]. Wow! Francis Crick has used the word “miracle”?
The other Nobel Prize winner in Medicine/Physiology who discussed origins is my favourite thinker. Writing in his book “The Limits of Science” Professor Sir Peter Medawar makes a comment that goes a long way to shedding light on Dr Matthews’ “perplexing problem of music’s origin”. Professor Medawar states that questions of ultimate origins are “beyond the explanatory competence of science” [14, page xiii], and this: “That there is indeed a limit upon science is made very likely by the existence of questions that science cannot answer and that no conceivable advance of science would empower it to answer” [14, page 68]. So here is one genius, the DNA genius, describing the origin of life (and hence of language, and music) as a “miracle”, while the other Nobel genius says Science need not even bother tackling ultimate origins because Science does not only not have the equipment to probe ultimate origins, but also that it will never have that equipment. In the BMJ I once described as supra-scientific 4 clinical facts in my experience that Science could not explain. [15]. I place the origin of Language and Music in the same supra-scientific category. As I also said in the Journal of the Royal Society of Medicine earlier this year “Origin of the genetic code is supra-scientific” [16], it becomes clear that the origin of music and language must also be supra- scientific.

THE ART OF BEING HUMAN IS ITSELF SUPRA-SCIENTIFIC
There must be aspects of Dr Matthews’ fruitful work with patients and music that do not admit of scientific dissection. It is possible for me to imagine some of his grateful patients with severe arthritis saying “the pain is gone suddenly”, after listening to some moving music, such as the Bulgarian National Choir singing that amazingly magnificent Chorus of the Hebrew Slaves. I would find that just as scientifically inexplicable as the case of the middle aged accountant I once described in the BMJ who suddenly lost his right upper quadrant abdominal pain and the Murphy’s sign of cholelithiasis when he was told “the stone has disappeared”, while in fact the gall stone was later shown by ultrasound to be well and truly still in situ. [15] Music be it enchanting or cacophonous, is indeed the art of being human, which itself is also beyond Science. Furthermore, although “music and the brain” equates to “music and the psychological”, that is not the same as “music and the spiritual” because the spiritual connotes a greater dimension for what it means to be human – a dimension I once showed also in the BMJ [17] to be so different from the psychological that Science has no tools to fathom it.
F I D KONOTEY-AHULU FGA MD(Lond) FRCP(Lond) FGCP DTMH(L’pool) FTWAS FAAS FWACP ORDER OF THE VOLTA GHANA felix@konotey-ahulu.com
9 Shannon Claude E. The Mathematical Theory of Communication. University of Illinois Press 1949 ( Urbana II).
10 Chomsky Noam. Linguistic Contributions to the Study of Mind (Future). Harcourt Brace Jovanovich 1968 http://bit.ly/egkZi1 “By pursuing the kind of research that now seems feasible we can acquire the highly specific way of interpreting phenomena that are in large measure beyond our consciousness and control and that may be unique to man”.
11 Gitt Werner. In the beginning was Information. Christliche Literatur-Verbretung e.V. Postfach 110135.33661 Bielefeld, Germany, 2001. [Professor Werner Gitt coined the word ‘apobetics’ to illustrate the highest of the 5 levels of communication in humans]
12 Crick FHC. The origin of the genetic code. J Mol. Biology 1968: 38: 367-379.
13 Crick FHC. Life itself: its nature and origin. Simon and Schuster, New York 1981, p 88.
14 Medawar Peter. The Limits of Science. Oxford . Oxford University Press, 1985.
15 Konotey-Ahulu FID. The supra-scientific in clinical medicine: a challenge for Professor Know-All. BMJ 2001; 323(7327): 1452-1453 (22-29 Dec). http://www.bmj.com/cgi/reprint/323/7327/1452.pdf doi:10.1136/bmj.323.7327.1452
16 Konotey-Ahulu FID. Origin of the genetic code is suprascientific. JRSM March 15 2010 http://www.sicklecell.md/blog/?p=46 Response to James Le Fanu “The disappointments of the Double Helix” Feb 2010.
17 Konotey-Ahulu FID. Personal View: The spiritual and the psychological in Clinical Medicine. BMJ 1977; 1: 1595. (June 15) doi:10.1136/bmj.1.6076.1595 http://www.bmj.com/content//1/6076/1595.full.pdf
Competing interests: None declared
Submit rapid response
Published 20 December 2010
4.

Music (and Tonal Language) and the art of being human – I
o Felix ID Konotey-Ahulu, Dr Kwegyir Aggrey Distinguished Professor of Human Genetics University of Cape Coast , Ghana
Consultant Physician Genetic Counsellor in Sickle Cell & Other Haemoglobinopathies 10 Harley St Lond
Dr John Matthews (Dec 11, p 1274) has shown that, contrary to popular perception, a doctor can have interests far beyond the consulting room. His most excellent review [1] of Philip Ball’s book covering music and the brain [2] deserves a 3-part response: Part I deals with Music, the brain, and language. Part II tackles Origins and what it means to be human. Part III suggests meaningful research approaches.

MUSIC AND THE BRAIN
Dr John Matthews is right in stating “Music stimulates more parts of the brain than any other intellectual activity.” [1], and that these parts “are shared with other activities, such as language..”, especially, in my opinion, Tonal Language. I have proven that my Ghanaian language Krobo/Dangme-Ga can be read like music [3-5], and that the best way to evaluate Tonal Language is not through the piano, flute, violin, or organ but with Tonic Solfa.

TONIC SOLFA, TONAL LANGUAGE, AND THE BRAIN
Tonic Solfa is an extraordinary musical phenomenon. It was invented by Guido Arezzo, a musically talented monk who was born in 991 AD. Tonic Solfa was taught to my ancestors by the Swiss-German Basel missionaries who went to our tribe in 1828. When the British Colonial Government of the Gold Coast ( Ghana ) deported them during the First World War, Scottish Presbyterians took over the work, and continued teaching every pupil how to sing in Tonic Solfa. That was how I came to learn Tonic Solfa the marvel of which is that whereas any piece of music can be played recognisably in all the 12 keys of the piano there is just one way a particular piece of music can be sung in Tonic Solfa. [3-5] For example, the British national anthem “God save the Queen” can be played in 12 different keys (though traditionally Key G major ie F sharp is used), but the brain is so programmed that there is just one way it can be sung in Tonic Solfa: doe doe re ti doe re mi mi fa mi re doe re doe ti doe (d d r t: d r m m f m: r d r d t d). Similarly, my Mother Tongue can be phonated in Tonic Solfa one way only. Every vowel (without prolonging it) has at least 4 different ways of pronouncing it, giving 4 different meanings to the same consonant [3-5]. As nasalisation (quality) of the vowel at each pitch gives the word a different meaning, one can find that the word written ta (without prolonging the ‘a’) can mean, as I pointed out in the Lancet, chew, palm tree, war, giant ant, narrate, and fish out [6]. The brain of a Krobo child is capable of distinguishing the 6 different meanings of that word ta [6], a feat that defeats many a European expert in Linguistics. .

HUMAN SPEAKING VOICE SPANS AN OCTAVE
I have proven before that the normal speaking human voice spans an octave. One can say everything one wants to say (at least in Tonal Linguistics) using just an octave. Starting from d and going through the d r m scale to the next d is traversing an octave. Filling in the pitches between the 12 keys on the piano one can discern d de r ma me f fe s se la ta t d [13 keys], the space between each key is a semi-tone.[3 4 7] Using one particular phrase in The Hallelujah Chorus of Handel’s Messiah “For The Lord God Omnipotent reigneth” I have shown that the brilliant octaves that Handel introduced in that short phrase reflect exactly the octaves not only in tribal linguistics, but also in spoken English! [3 4 7] Just pronounce the English word “agriculture” (slowly) in Queen;s English. My African ear automatically discerns 4 pitches, a high pitch (‘a’), a low pitch (‘ture’), a mid pitch just below high pitch (‘gri’), and a lower mid pitch (‘cul’). These are fixed pitches that can be characterised as follows: high pitch is an octave above low pitch (just as Handel’s ‘God Om..’ is an octave), mid pitch is exactly 3 semitones below high pitch, and lower mid pitch is exactly 2 semitones below mid pitch.[3 4 7] This information is not in Professor David Crystal’s impressive 484-page book ‘THE CAMBRIDGE ENCYCLOPOEDIA OF LANGUAFGE’ [8]. The brain that is capable of recognising these pitch differences in order to discern one accent from another in the same language, or to distinguish one language from another, or to appreciate one piece of music more than another is remarkable to say the least. To facilitate appreciation of these pitch differences so that written text is correctly readable in Tonal Linguistics I decided to colour them to help even illiterates read their tribal language more easily than when the missionaries wrote them. [4 7]

COLOURING SOUND
I colour high pitch red. Using Key C major for convenience, high pitch corresponds to s or G above middle C, and mid pitch which is 3 semitones below high pitch, I make green, and becomes m or E above middle C. Lower mid pitch then becomes r ie 2 semitones below mid pitch (lime green), before descending to the low pitch s (G below middle C, ie an octave below the high pitch s), which I make blue. [4 7]. Anybody can be taught to pronounce “Agriculture” in Queen’s English by just associating the colours of the 4 vowels with the pitches. I have done the same with Japanese. [4 7] The pronunciation of “Japanese” in Japanese is Nihongono (‘Ni’ low pitch blue, ‘ho’ high pitch red, ‘ngo’ mid pitch green, ‘no’ mid pitch green) – see references 4 and 7 on line for a visual appreciation of these novel approaches to Tonal Linguistics.
F I D KONOTEY-AHULU FGA MD(Lond) FRCP(Lond) FGCP DTMH(L’pool) FTWAS FAAS FWACP ORDER OF THE VOLTA GHANA felix@konotey-ahulu.com
1 Matthews John A. Music and the art of being human. BMJ 2010; 341:c6965 http://www.b,j.com/content/341/bmj.c6965
2 Ball Phillip. The Music Instinct: How Music Works and Why We Can’t Do Without It. Bodley Head, pp 464. ISBN: 978-1847920881
3 Konotey-Ahulu FID. Mother Tongue: The Tadka Phonation Technique for speaking an African Tonal Language – Krobo/Dangme/G? of South-East Ghana . Watford 2001, UK . ISBN 0-9515442-4-1. [To facilitate tribal health education in the Mother Tongue]
4 Konotey-Ahulu FID. The Remarkable African Ear: Phenomenon of Mid Pitch Arrest in Krobo-Dangme-G? Tonal Linguistics of South East Ghana . African American Museum of Philadelphia Award Lecture May 5 2007 http://bit.ly/i5APah
5 Konotey-Ahulu FID. How To Avoid Losing Our Mother Tongue. Substance of Lecture given at the British Council Accra November 2007 for Ghana @50. Ghana Academy of Arts & Sciences – President Dr Letitia Obeng President chairing. Guest of Honour Nene Sakite II, Konor of Manya Krobo, – honours Professor Felix I Domeno Konotey-Ahulu with Citation Plaque of Klo Hingmer (Eye of Krobo). http://www.modernghana.com/GhanaHome/NewsArchive/news_details.asp
6 . Konotey-Ahulu FID. Black people’s red faces and AIDS prevention. Lancet 2000; 355(9214):1559. PMID: 10801206 [PubMed-indexed for MEDLINE]
7 Konotey-Ahulu FID. Social pathology of Cleft Palate in The African: Mathematical Precision of Pitch Gaps in Tribal Tonal Linguistics. Ghana Medical Journal 2008; 42: 89-91. (June 2008). PMID: 19180210 http://bit.ly/f8dVG3
8 Crystal David. THE CAMBRIDGE ENCYCLOPEDIA OF LANGUAGE. Cambridge University Press. Second Edition, 1997.
Competing interests: None declared

www.bmj.com Posted in British Medical Journal on November 19, 2010

Felix ID Konotey-Ahulu, Dr Kwegyir Aggrey Distinguished Professor in Human Genetics University of Cape Coast Ghana
Consultant Physician Genetic Counsellor in Sickle Cell & Other Haemoglobinopathies 10 Harley St London W1G 9PF

Annelien Bredenoord and Peter Braude (8 November) have placed readers of the BMJ in their debt not only by meticulously dissecting the ethical and other dilemmas associated with this particular frontier of genetic research and impending practice, but also by calling for “a transparent public debate” [1]. I join the debate by briefly commenting on (a) Consequences of mitochondrial gene replacement (b) Legality (c) Ethics (d) Informed Consent.
CONSEQUENCES
I coin the term “acquired
More…
Annelien Bredenoord and Peter Braude (8 November) have placed readers of the BMJ in their debt not only by meticulously dissecting the ethical and other dilemmas associated with this particular frontier of genetic research and impending practice, but also by calling for “a transparent public debate” [1]. I join the debate by briefly commenting on (a) Consequences of mitochondrial gene replacement (b) Legality (c) Ethics (d) Informed Consent.
CONSEQUENCES
I coin the term “acquired genetic inheritance” which normally does not make sense, except that what we are talking about is “irreversibility of germ line modification” [1] which is passed on to offspring after offspring, not initiated genetically but instantly inherited through an acquired process. An enlightened offspring is one day entitled to ask “Why was such and such acquired for me and my future children?”, where “such and such” could be any unanticipated genetic defect resulting from the process.
LEGALITY
The legal process that will allow mitochondrial gene replacement depends entirely on where this is done in the world. If the British Parliament votes for it then it will be legal; if not, it will be illegal to practise it in the UK . Laws are not static – one parliament may say “No”, while the next may approve. Society gets the law it deserves. “The deliberate creation of human embryos for research . . .is legally prohibited in many countries” say Annelien Bredenoord and Peter Braude [1], so one wonders what happens when the country is not democratic, and dictators can say what must happen and what must not happen, and to whom? Who enacted Germany ‘s laws during the Nazi regime? [2]
ETHICS
“Ethics” I once said at a Human Genome Diversity Project Symposium “is sometimes not the first thing considered in the excitement of advances in scientific endeavour” [3] but as the article of Bredenoord and Braude makes abundantly clear, what is considered unethical by one group of scientists makes others take a different view: “the deliberate creation of embryos for research should be allowed under strict conditions” [1]. But what are these “strict conditions” that Annelien Bredenoord and Peter Braude have in mind?
I was for many years on the Ethics Committee of a leading UK Private Hospital that gave In Vitro Fertilisation consultations. The Chair-person was a Judge who presided over 10 of us that included another judge, an Obstetrician Gynaecologist, 2 Family Practitioners, a Consultant Physician/Neurologist, a Rabbi, a Consultant Physician/Nephrologist, the Matron of the Hospital, and 1 African, myself (Consultant Physician/Genetic Counsellor). Many were the requests from clients who begged for things they wanted done, but which we turned down unanimously – requests that other Ethics committees in the UK might well have approved simply because they were not illegal. Twice a year we made trips to Cambridge to visit (now) Nobel Laureate Robert Edwards whom I mentioned recently [4] had convened a panel of 30 of us under the auspices of the World Council of Churches on 25 June 1973 in Zurich to thrash out the ethics of what he was then embarking on [5]. I wonder what Bob Edwards now makes of the galloping pace of what is happening at the frontiers of Human Genetics.
INFORMED CONSENT
“A third question” asks Bredenoord and Braude “is how to guarantee adequate informed consent given the complexity of technical information, high uncertainty, and competing interests”? [1] Quite so! Leaving the UK aside and moving to the Third World scenario, we are told by the International Consortium sequencing 1000 genomes globally that “A thousand volunteers have already been recruited from Africa, Asia, America, and Europe” [6] and that the African volunteers who had given informed consent were the “Maasai in Kinyawa, Kenya”, the “Yoruba in Ibadan, Nigeria” and “Luhya in Wabuye, Kenya” [7]. I would dearly want to know how “the complexity of technical information” surrounding the genome sequencing exercise was explained to my fellow African natives. Of course we cannot check any details of what was communicated to them because the whole exercise was officially done “anonymously” [6 7]. Bredenoord and Braude have been impressively transparent in marshalling their theses but could we say that about some researchers who, denied the opportunity in their own countries to do forbidden research, would not hesitate to go to Guatemala with plenty of money in foreign exchange to move mitochondrial gene replacement from bench to the bedside? [8 9].
LOOKING FOR THE PERFECT BABY?
I myself am a bundle of funny genes, one of which is the Mendelian dominant extra digits [10], which (before the Swiss Basel missionaries went to the Gold Coast in 1828) some tribes east of us the Krobo people had considered so reprehensible that affected babies were drowned [10]. So appalled I was when I came to England to study Medicine to find that babies that would be born with the same sickle cell disease phenotype as 3 of my siblings were also recommended routinely to be aborted here legally that I later wrote to the BMJ:
“I was born in the Krobo tribe with extra digits – a Mendelian dominant condition with a 1% incidence at birth in Ghana . Had I been born a few miles south east across the Volta River , there would have been great rejoicing because local tribesmen had it that I was destined to be rich. If my mother had given birth to me a few miles north-west beyond the hills, I would not be here to write to you – I would have been drowned soon after birth. Fortunately the Krobo’s were neutral to extra digits but until the government forbade the practice some tribal elders took it on themselves to decide which genes ought to be allowed to survive!” [10]. I went on “Now some scientific tribesmen in the UK ” were similarly deciding which genes should be allowed to remain and which not [10]. Even those with sickle cell disease have been found in Ghana to have other genes which enabled them to achieve great things in life [11]. Some parents do not lightly consider having a baby with a known pathology, of course they don’t, but if the modern attitude is to go to all lengths, including irreversibly changing the germ line of future generations through replacing mitochondrial genes then many reasonable people will consider that a step too far. As regards the great importance of public debate on this vital matter I conclude with the words of a wise man, Sir David Weatherall FRS:
“Whether by opening up public debate about the long-term possibilities of genetic engineering we can prevent its gross misuse, either politically or in other ways, is for future generations to determine. But there is no reason for not trying. One of the major concerns about human genetic manipulation is that our track record in the ethical aspects of human genetics is not entirely reassuring” [12]. In any case, Africans are scared stiff of genetic and genomic studies practised on them however carefully researchers explain exactly what they are doing and obtain “informed consent”. [3]
Conflict of interest: Nothing to declare
Felix I D Konotey-Ahulu FGA MD(Lond) FRCP DTMH ORDER OF THE VOLTA ( GHANA )
Kwegyir Aggrey Distinguished Professor of Human Genetics, University of Cape Coast and Consultant Physician Genetic Counsellor in Sickle Cell and Other Haemoglobinopathies, 10 Harley Street, London W1G 9PF felix@konotey-ahulu.com
1 Bredenoord Annelien L, Braude Peter. Ethics of mitochondrial gene replacement: from bench to bedside. BMJ 2010; 341:c6021 doi: 10.1136/bmj.c6021 http://www.bmj.com/content/341/bmj.c6021?papertoc= Nov 8
2 Muller-Hill Berno. Murderous Science: Elimination by Scientific Selection of Jews, Gipsies, and Others – Germany 1933-1945. [Translated from German by G R Fraser] Oxford , Oxford University Press, 1988.
. 3 Konotey-Ahulu FID. The Human Genome Diversity Project: Cogitations of an African Native. Politics and The Life Sciences (PLS) Volume 18 Number 2, September 1999, pages 317-322 [Symposium PMID: 12561789 PubMed indexed for MEDLINE]
4 Konotey-Ahulu FID. Genius of Nobel Laureate Robert Edwards goes beyond IV F. http://www.bmj.com/content/341/bmj.c5533/reply#bmj_el_243005 BMJ Rapid Response, 14 October 2010.
5 World Council of Churches. Genetics and the Quality of Life: Study Encounter Vol X, No 1 1974. Report of a Consultation: Church and Society, Christian Medical Commission, World Council of Churches, Zurich , June 1973, Switzerland (Chairman Dr Robert Edwards)..
6 Wise Jaqui. Consortium hopes to sequence genome of 1000 volunteers. BMJ 2008; 336: 237 (February 2).
7 Announcement: International Consortium Announces the 1000 Genomes Project. Major Sequencing Effort Will Produce Most Detailed Map of Human Genetic Variation to Support Disease Studies. (Tuesday January 22 2008). http://www.1000genomes.org/files/1000Genomes-NewsRelease.pdf
8 Tanne Janice Hopkins. President Obama apologises to Guatemala over 1940’s syphilis study. BMJ 2010: 341. c5494 October 9, page 750. http://www.bmj.com/content/341/bmj.c5494.full
9 Konotey-Ahulu FID. President Obama apologises over Guatemala syphilis study: International co-operative research in jeopardy. BMJ Rapid Response http://http://www.bmj.com/content/341/bmj.c5494.full/reply#bmj_el_243183 Oct 17 2010.
10 Konotey-Ahulu FID. Ethical issues in pre-natal diagnosis. BMJ 1984; 289: 185 http://www.bmj.com/cgi/reprint/289/6438/185-a.pdf
11 Amanor-Boadu Dorothy, Bruce-Tagoe Alexander, Konotey-Ahulu FID. The Third International Conference On The Achievements of Sickle Cell Disease (ACHEACHE) Patients, Accra – 19th July 2010. Accra, Adeko Limited. ISBN: 978-1-3927-8. [International Conference Centre – 22 pages]
12 Weatherall DJ. Ethical issues and related problems arising from the application of the new genetics to clinical practice Chapter 12 in The New Genetics and Clinical Practice D J Weatherall, Oxford , Oxford University Press, Third Edition 1991, page 362.
Competing interests: None declared

Responding to

Antenatal haemoglobinopathy screening
by Judy Shakespeare
BMJ 341:doi:10.1136/bmj.c5243 (Published 18 October 2010) www.bbj.com

Rapid Response http://www.bmj.com/content/341/bmj.c5243/reply#bmj_el_243447

October 25 2010 by

Felix ID Konotey-Ahulu, Kwegyir Aggrey Distinguished Professor of Human Genetics University of Cape Coast , Ghana  and Consultant Physician Genetic Counsellor in Sickle&Other Haemoglobinopathies 10 Harley St London W1G
The commissioned editorial [Reference 1] of Judy Shakespeare (18 October) is as comprehensive as the article [2] of Elizabeth Dormandy et al whose concerns the editorial underlined. My comments with references [3] on the Elizabeth Dormandy paper are therefore equally applicable to both articles, and will not largely be repeated here. But there are a couple of clarifications which readers would like to see made.  

CLARIFICATIONS NEEDED

What does the following mean? “Antenatal screening for sickle cell disease and thalassaemia is just the tip of a genetic iceberg and primary care needs to be prepared.” [1]. How can screening be the tip of an iceberg?
Nor is the following easily comprehensible: “In the United Kingdom , about 250,000 people are healthy carriers of sickle cell gene variants and 12,500 have the disease ..”

 What are healthy carriers of sickle cell gene variants? Does the author mean beta-globin gene variants? If as Judy Anderson states, her fellow general practitioners need to “raise their awareness and skills” to educate their patients are they likely to understand what “sickle cell gene variant carrier” means?

GHANAIAN BASIC TERMINOLOGY ACCEPTABLE TO EVEN ILLITERATES

Will UK General Practitioners countenance the terminology I use to instruct Ghanaian illiterates and fellow health staff with the message of sickle cell disease inheritance? For decades I have told them that all Ghanaians are divided into 3 broad phenotypes, using my own parents and their children [Reference 4] as examples: (i) ACHEACHE (those who have inherited an ACHE gene from both parents and therefore ache in the cold rainy season with sickle cell disease, like 3 of my parents’ 11 children) (ii) NORMNORM (no cold season rheumatism, like 4 of my parents’ children, having inherited no abnormal haemobglobin, and blood test shows no beta globin variant) (iii) NORMACHE (no cold season rheumatism, like the other healthy 4 of us but blood test shows one beta globin variant trait, like our parents). Every new patient of mine for several decades now has had Haemoglobin Electrophoresis done, confirming that 1 in 3 of West Africans is NORMACHE ie a Trait. In my experience, the assumption that identified NORMACHE x NORMACHE marriages will be queuing up to have prenatal diagnosis and selective abortion done when offered it is too presumptuous for the reasons already given in my earlier comments [Reference 3]. Many prefer genetic gambling, then stopping procreation after 1 or 2 children if they happen to avoid ACHEACHE phenotype. If a sickle cell disease offspring arrives, they do not panic because they know many adult ACHEACHE Achievers. [3]

SICKLE CELL DISEASE HAEMOGLOBINOPATHY IS DIFFERENT

 
Sickle cell anaemia, a qualitative molecular pathology ‘SS phenotype’, and beta thalassaemia major a quantitative molecular pathology are both, as Judy Shakespeare correctly points out [1] homozygous recessive, but their public health handling must be entirely different [Reference 3]. No where in the world is beta-thalassaemia minor, the heterozygote, 33.3% of the healthy population, whereas if one phenotypes West Africans anywhere in the world well above 35% of us will be NORMACHE, ie traits of abnormal haemoglobins (AC or AS), and we do not know it until we are investigated by Hb electrophoresis. Helping Ghanaians and Nigerians to make informed decisions in procreation does not lie in prenatal diagnosis and selective abortion as appears to be the case in Cyprus , but does lie in Genetic Counselling with Voluntary Family Size Limitation. [References 4 & 5]. And, take it from me, that works!

Felix I D Konotey-Ahulu MD(Lond) FRCP DTMH ORDER OF THE VOLTA ( GHANA )
Dr Kwegyir Aggrey Distinguished Professor of Human Genetics University of Cape Coast , Ghana and Consultant Physician Genetic Counsellor in Sickle and Other Haemoglobinopathies. [felix@konotey-ahulu.com]

 
Conflict of interest: My parents who were traits for abnormal haemoglobins, had 11children 3 of whom had sickle cell disease, 4 are traits and 4 have no beta globin gene variant.

1 Shakespeare Judy. Antenatal haemoglobinopathy screening. BMJ 2010; 341: c5243 (Editorial 18 October)

2 Dormandy Elizabeth, Gulliford Martin, Bryan Stirling, Roberts Tracy E, Calnan Michael, Atkin Karl, Karnon Jonathan, Logan Jane, Kavalier Fred, Harris Hilary J, Johnston TraceyA, Anionwu Elizabeth N, Tsianakas Vicki, Jones Patricia, Marteau Theresa M. Effectiveness of earlier antenatal screening for sickle cell disease and thalassaemia in primary care: cluster randomised trial. BMJ 341:doi:10.1136/bmj.c5132 (5 October 2010)

3 Konotey-Ahulu FID. Antenatal screening for sickle cell disease and beta-thalassaemia. http://www.bmj.com/content/341/bmj.c5132/reply#bmj_el_242914 BMJ Rapid response. 12 October 2010

4 Konotey-Ahulu FID. Sickle cell disease in successive Ghanaian generations for 3 Centuries. http://www.konotey-ahulu.com/images/generation.jpg 

In The Human Genome Diversity Project: Cogitations of An African Native. Politics and the Life Sciences (PLS) 1999, Vol 18: No 2, pp 317-322. [Invited Commentary on Professor David Resnik’s article: The Human Genome Diversity Project: Ethical Problems and Solutions] PMID: 12561789 [PubMed – indexed for MEDLINE]

5 Konotey-Ahulu FID. Sickle Cell Disease: The Case for Family Planning. Accra . ASTAB Books, Ltd 1973

Competing interests: My parents who were traits for abnormal haemoglobins, had 11 children 3 of whom had sickle cell disease, 4 are traits and 4 have no beta globin gene variant.

Feature: Pain control : The politics of pain
• Tatum Anderson
BMJ 341:doi:10.1136/bmj.c3800 (Published 11 August 2010) – www.bmj.com

The politics (and economics) of pain relief in the West and Third World
o Felix ID Konotey-Ahulu, Kwegyir Aggrey Distinguished Professor of Human Genetics University of Cape Coast Ghana
Consultant Physician Genetic Counsellor in Sickle/Other Haemoglobinopathies 10 Harley St London W1G
The politics (and economics) of pain relief in the West and Third World
Tatum Anderson (14 Aug, p 328) wished pain relievers elsewhere could imitate the “Western world” [1]. His concern needs to be put into perspective. Referring to Armenia he quotes Dr Karfeptyan as saying “Finances remain a problem” [1]. Yes, Health Ministers decide which imported drugs have priority over others.

WHO RECOMMENDATIONS
Tatum Anderson mentions WHO recommendations on pain relief, but WHO
More…
The politics (and economics) of pain relief in the West and Third World
Tatum Anderson (14 Aug, p 328) wished pain relievers elsewhere could imitate the “Western world” [1]. His concern needs to be put into perspective. Referring to Armenia he quotes Dr Karfeptyan as saying “Finances remain a problem” [1]. Yes, Health Ministers decide which imported drugs have priority over others.

WHO RECOMMENDATIONS
Tatum Anderson mentions WHO recommendations on pain relief, but WHO guidelines are not accompanied by money. Useful on paper, they can nevertheless be ignored. Exactly 38 years ago WHO convened some of us to produce an 83-page Technical Report on how to tackle the problem of Sickle Cell Disease. [2]. It was ignored. .
But WHO makes other recommendations which do not rely on foreign aid. WHO knows Traditional Medicine which costs a fraction of what imported drugs cost is widely used [3 4], and aims to “support and integrate traditional medicine into national health systems” [4]. True, some Africans cannot afford imported opiates but “85% of Nigerians use and consult traditional medicine for health care” [5]. Indeed, the pain of my own circumcision when a little boy was managed by a Krobo traditional healer who also “knew about haemostasis” [6]. Neither the ground haemostatic herbs nor the pain killing tincture came from abroad. Did the oral morphine that the Ugandans in Tatum Anderson’s article were using come from abroad, or did they get it from their traditional healers as WHO would have approved of?

WESTERN NATIONAL INTERESTS FIRST BEFORE ANYTHING ELSE
Tatum Anderson writes as if “The Western world” speaks with one voice. Take his comment: “Also, many patients with conditions, such as sickle cell anaemia, …require relief but do not get it” [1] The UK uses Diamorphine for sickle cell crisis patients [7 8], while “heroin has no accepted use in the United States” [9, page 168]. Do these two countries not belong to “The West” and yet do they not differ in their use of this particular opiate? That national interests have more to do with choice of drugs in their Pharmacopoeia is shown by UK’s reaction to Professor Elisabeth Goodman’s finding in the USA [10] that the use in painful crises of Ketorolac was as efficient as morphine but without the latter’s respiratory depression: “Ketorolac has no product licence in the UK for this indication” [11]. Professor Graham Serjeant has stated: “In Jamaican experience morphia or its derivatives are rarely used or necessary” [12, page 204]. I agree [3].

GHANAIAN SICKLE CELL DISEASE ACHIEVERS
On 19 July 2010 The Third International Conference On The Achievements of Sickle Cell Disease Patients [14] was held in Accra, followed on 20-23 July by The First Global Congress On Sickle Cell Disease to mark the 100th anniversary of the first clinical description of Sickle Cell Disease. The Conference had sickle cell anaemia adults in their 7th decade tell us how they managed to achieve great things [14 15]. One known “SS” man with a PhD, who had never in his 63 years been transfused though Hb level was never above 8.8 g/dL and who had never been prescribed Hydroxyurea, astonished delegates when he announced “I do not know when I last took a pain killer for my sickle cell anaemia. Drink plenty of water, avoid malaria, and have a positive attitude to life” [14, page 15].

NATIONAL ENQUIRY INTO PATIENTS OUTCOME AND DEATH (NCEPOD)
The NCEPOD report [7 8] concludes that during 2 years “Nine out of the 19 patients with sickle cell disease who had pain on admission and who then died had been given excessive doses of opiods” [8], and this continues to happen [16 17] in spite of UK’s National Institute of Clinical Excellence (NICE) [18 19]. Is post narcotic administration death (pnad) the kind of relief that Tatum Anderson’s “West” is seeking for our patients, many of whom have become bread winners when their hereditary condition is managed properly? [20 21]

WHAT THE THIRD WORLD CAN AND MUST LEARN FROM THE WEST
There is plenty though The Third World can learn from the West, not least of which is ‘Discipline’ in personal and national affairs. We must learn that every privilege demands some responsibility. Why should it be implied [1] that Bill and Melinda Gates could supply us with free morphine? Why should we Africans expect help to come from outside when we refused to learn lessons like Singapore had done?
Next, we Africans need discipline against corruption. I described how a Lebanese, an Italian, plus a Ghanaian conspired and supplied the Ministry of Health in Accra with Ampicillin capsules stuffed with chalk and cassava powder as “500 mg Ampicillin” [22]. The then Commissioner of Health to whom I reported this, and who began to probe the racket was promptly removed from his post [22]. True, the Italian crook came from the “West” but, generally speaking, “The West” is ready to show us the proper means and ways to deal with situations like that [22]. Our envy of “The West” knows no bounds when we read of their ability to prosecute a French President, discipline a German Chancellor, investigate an Israeli Prime Minister, dismiss a Japanese Minister, and remove a sitting American President for “simply lying”. Twice in less than 3 years the BMJ has published articles on pain relief for Africans [1 23]. Can we also have articles showing how “The West” could help us prevent a Minister of Health from being removed for probing crooks in the pharmaceutical industry, and how we should not expect Bill and Melinda Gates to give us free antimalarial drugs when we have not covered open drains in our cities like Singapore and Trinidad have done? [24].
Felix I D Konotey-Ahulu MD(Lond) FRCP DTMH Dr Kwegyir Aggrey Distinguished Professor of Human Genetics University of Cape Coast, Ghana and Consultant Physician Genetic Counsellor in Sickle and Other Haemoglobinopathies, 10 Harley Street, London W1G 9PF felix@konotey-ahulu.com

1 Anderson Tatum. The politics of pain. Brit Med J 2010; 341: c3800 http:// www.bmj.com/cgi/content/full/341/aug11_2/c3800
2 Boyo AE, Cabannes R, Conley CL, Lehmann H, Luzzatto L, Milner PF, Ringelhann B, Weatherall DJ, Barrai I, Konotey-Ahulu FID, Motulsky AG. Scientific Group on Treatment of Haemoglobinopathies and Allied Disorders. (Technical Report) 1972; 509 – 83 pages.
3 WHO. Traditional Medicine Strategy 2002-2005. Geneva: WHO 2002
4 WHO. Traditional Medicine. Fact Sheet No; 134. http://www.who.int/mediacentre/factsheets/fs134;en
5 Adelaja Abiose. Nigeria boosts research into traditional medicine. Sci Dev Net Dec 6 2006 http://www.scidev.net/en/news/nigeria- boosts-research-into-traditional-medicine.html
6 Konotey-Ahulu FID. Male circumcision and alleged protection against AIDS. http://www.bmj.com/cgi/eletters/335/7631/1206# BMJ 11 Dec 2007 Rapid Response
7 NCEPOD (National Confidential Enquiry into Patient Outcome and Death). Sickle: A Sickle Crisis? (2008) [Sebastian Lucas (Clinical Co- ordinator), David Mason (Clinical Co-ordinator), M Mason (Chief Executive), D Weyman (Research), Tom Treasurer (Chairman) info@ncepod.org
8 Mayor Susan. Enquiry shows poor care for patients with sickle cell disease. BMJ 2008; 336: 1152.
9 Ballas S K. Sickle Cell Pain. IASP Press. Seattle, USA.
10 Goodman Elisabeth. Use of ketorolac in sickle cell disease and vaso-occlusive crisis. Lancet 1991; 338: 641-42.
11 Liesner RJ, Vandenberghe EA, Davies SC. Analgesics in sickle cell disease. Lancet 1993; 341: 188.
12 Serjeant GR. Sickle Cell Disease. Oxford. Oxford University Press, 1985, page 204.
13 Konotey-Ahulu FID. Morphine for painful crises in sickle cell disease. BMJ 1991; 302: 1604.
14 Amanor-Boadu Dorothy, Bruce-Tagoe Alexander, Konotey-Ahulu Felix. The Third International Conference On The Achievements Of Sickle Cell Disease (ACHEACHE) Patients. Accra – 19th July 2010. Adeko Ltd, Accra, Ghana. ISBN: 978-9988-1-3927-8.
15 The Second International Conference On The Achievements Of Sickle Cell Disease Patients, Accra 19th July 1995.
16 Dyer Clare. Inquest begins into deaths after concerns about diamorphine levels. BMJ 2009; 338: b903 [7 March]
17 Konotey-Ahulu FID. Inquest into diamorphine deaths; Does NCEPOD sickle cell patients report warrant a similar inquest? BMJ Rapid response 7 March 2010. http://www.bmj.com/cgi/eletters/338/mar03_3/b903#210208
18 Konotey-Ahulu FID. Poor care for sickle cell disease patients: This wake up call is overdue. http://www.bmj.com/cgi/elettres/336/7654/1152-a#196224 BMJ Rapid response 29 May 2008.
19 Konotey-Ahulu FID. Current “hit and miss” care provision for sickle cell disease patients in the UK. BMJ Rapid Response 22 July 2008 http://www.bmj.com/cgi/eletters/337/jul11_2/a771#199135
20 Serjeant GR. The case for dedicated sickle cell centres. BMJ 2007; 334: 477 (3 March)
21 Konotey-Ahulu FID. Dedicated sickle cell centres. BMJ Rapid response March 20 http://www.bmj.com/cgi/eletters/334/7591/477#162678
22 Konotey-Ahulu FID. Who should best pharmacovigilate in developing countries? 14 September 2007 BMJ Rapid Response to Editorial http://www.bmj.com/cgi/eletters/335/7618/462#167455
23 Logie Dorothy, Leng Mhoira. Africans die in pain because of fears of opiate addiction. BMJ 2007; 335: 685 http://www.bmj.com/cgi/content/full/335/7622/685 October 6 2007.
24 Konotey-Ahulu FID. Fighting Malaria: Isn’t the best approach through Environmental Hygiene and Public Health? BMJ Rapid Response April 26 2009 http://www.bmj.com/cgi/eletters/338/apr20_2/b1627#212782
Competing interests: None declared