Author: Dr Felix Konotey-Ahulu

Re: Management of sickle cell disease patient n the community

FELIX ID KONOTEY-AHULU, Kwegyir Aggrey Distinguished Professor of Human Genetics, University of Cape Coast, Ghana

Consultant Physiician Genetic Counsellor in Sickle Cell and Other Haemoglobinopathies, 9 Harley Street Ltd., Phoenix Hospital Group, London W1G 9AL

British Medical Journal  Rapid Response www.bmj.com/content/348/bmj.g1765/rr/694233

13 April 2014

Re: Management of sickle cell disease in the community. Valentine Brousse, Julie Makani, David C Rees. 348:doi:10.1136/bmj.g1765

Valentine Brousse and colleagues’ Clinical Review [1], gives doctors little guidance for patient management. “Serjeant’s and my combined experience of 80 years covering thousands of patients in sickle cell crisis” can help. [2].

DISAGREEMENT WITH NICE ON PATIENT MANAGEMENT

Graham Serjeant’s Jamaican experience [3] and my Ghanaian [4] differ from NICE’s Guidance [5] in UK where patients died from Morphine/Diamorphine overdose. [6 7] We managed sickle cell disease (scd) patients to university without Morphine/Diamorphine, Hydroxyuurea, or regular transfusions. Our experience (References 1965 to 2014} could save UK patients.

PROOF OF EXPERTISE http://bit.ly/1gDdMlN [8]

(1) Two brothers and one sister suffered from scd “Hereditary Rheumatism” traced back to 1670 AD www.konotey-ahulu.com/images/generation.jpg [9-11]. Siblings’ commonest cause of sickle cell crises characterized by convulsions and priapism [9 10] was Malaria. One brother had ‘gnathopathy’, my invented word for maxillary marrow hyperactivity [10 12 13 14].
(2) Directing largest Sickle Cell Clinic I catalogued 133 Illustrative Case Histories [10] including alcohol-induced myocardial infarction [10, p 503] and mental nerve neuropathy [15], kanumblll sign. Investigate any numb lower lip for scd. [15]
(3) Haematologist Helen Ranney published “There is no single clinical experience in the United States comparable to that of Dr Konotey-Ahulu” [16].
(4) Receiving with Linus Pauling and others “Martin Luther King Jr Foundation Award for Outstanding Research in Sickle Cell Anaemia” my Keynote Address “Difference between Sickle Cell Disease and Sickle Cell Trait” that exposed fraud of Insurance Companies led to being given 4 bodyguards in Philadelphia. [17] By saying Sickle Cell Trait was “largely asymptomatic” [1] Brousse et al repeated Insurance Companies’ misinformation/disinformation [18-21] used to levy Traits 150% Premium.
(5) On WHO Expert Genetics Advisory Panel with Haemoglobinopathy Greats who valued Ethics in Human Genetics. [22]

LESSONS FROM GRAHAM SERJEANT’S JAMAICAN EXPERIENCE

“In Jamaican experience morphia or its derivatives are rarely used or necessary” [23] and “the most painful crises may be treated in a day centre, the patient returning home in the evening” [24]. See more of Serjeant’s publications from 1968 [23-38].

TIPS FROM MY FIRST PUBLICATIONS IN 1965 [39-41] TO LATEST 2014 [42 43]

(1) KEEP A DIARY! Circumstances [10 39], some patient-specific, precipitate crises eg Ghanaian scd man had sickle crisis on eating oranges [10]. Would Grousse et al start Hydroxyurea [1] after twice eating oranges?
(2) ALWAYS CARRY UMBRELLA. One rain-soaked scd boy got sickle crisis and stroke [44]. Trans-cranial Doppler [1] better prophylactic than raincoat? Hot weather too causes sickle crisis [39].
(3) NOSE PICKING causes Epistaxis [39 45].
(4) VALSALVA MANOEUVRE: In 18 causes of ocular bleeds [10] “six were related to sneezing, blowing nose, shouting, bending down, lifting heavy objects, and vigorous exercise” [46]. Avoid Valsalva in labour; Caesarian Section advised.
(5) DOCTORS BEWARE! Ectopic pregnancy, gall stones [47], appendicitis, splenic infarct [10] misdiagnosed as “Abdominal crisis”. Squatting (Tourniquet effect) can start crisis [10]. Opiates cause “Chest syndrome” [48]. Pulmonary embolism missed [49]. Note Intra-family scd phenotype differences [50 51]
(6) EXAMINE COMMUNITY RUMOUR. Medicated bed nets [1] no better than usual nets and may harm babies [52]. Malaria kills scd patients quicker [10 53-55]. Patients shun Hydroxyurea [56 57] and prenatal diagnosis [58 59] Drinking up to 4 L of water a day has stopped crises for past 25 years, as has “Aerobic Oxygen” 20 drops tds in water [60] Coconut juice clearing jaundice? [60.]
(7) STATIONERY BICYCLE aborts early morning priapism [10, 43]
(8) PRE-SURGERY PARTIAL EXCHANGE TRANSFUSION (Bedside method) [10].
(9) GENETIC COUNSELLING & VOLUNTARY FAMILY SIZE LIMITATION [61-69]
(10) SICKLE CELL DISEASE PATIENT ACHIEVERS teach doctors 1993-2010 [70]
(11) G6PD Deficiency: 1 in 4 male and 1 in 16 female scd patients have this. [71 72]
(12) WORLD SICKLE CELL DAY BROADCASTS AND RESOURCES [73 74]

WHAT CHOICE FOR UK CLINICIANS?

NCEPOD discovered “9 out of 19 patients with sickle cell disease who had pain on admission and who then died had been given excessive doses of opiods” [6 7]. Yet NICE still recommends “a strong opiod intravenously” [5]. But Why? [2 4 10 48 75-87]. Patients complain to BMJ [88-90].

1 Brousse V, Makali J, Rees DC. Management of sickle cell disease in the community. BMJ 2014; 348:g1765 doi:10.1136/bmj.g1765

2 Konotey-Ahulu FID Opiates for sickle cell crisis? Lancet 1998: 351: 1438 “The question that puzzles me is why do west African and West Indian patients with sickle cell disease who did without morphine in our countries have to be given morphine pumps during sickle cell crisis when they come to the UK?”

3 Serjeant G. The case for dedicated sickle cell centres. BMJ 2007; 334: 477

4 Konotey-Ahulu FID. Dedicated sickle cell centres. BMJ Rapid responses March 20 2007 http://www.bmj.com/cgi/eletters/335/7618/462#167455

5 NICE GUIDELINES. Management of acute painful sickle cell episode in hospital; summary of NICE Guidance. BMJ 2012; 344: doi:http://dx.doiorg/10 1136/bmj’e’4063

6 NCEPOD – National Confidential Enquiry into Patient Outcome and Death. SICKLE: A Sickle Crisis? 2008 [Sebastian Lucas (Clinical Coordinator), David Mason (Clinical Coordinator), M Mason (Chief Executive), D Weyman (Researcher)/ Tom Treasurer (Chairman) info@incepod.org

7 Mason S. Enquiry shows poor care for patients with sickle cell disease. BMJ 2008; 336: 1152 “In 2 years 9 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 Konotey-Ahulu FID. http://bit.ly/1gDdMlN International request to manage patients, for example, sicklecell.md/blog/index.php/2007/06/request-from-geneva-for-patient-in-hospital-in-colorado-usa June 2007

9 Konotey-Ahulu FID. Pattern of Sickle Cell Disease in Ghana (A Study Of 1,550 Consecutive Patients) – A Thesis Presented For The Degree of Doctor of Medicine (M.D.) In The University Of London 1971 Awarded Feb 1972.

10 Konotey-Ahulu FID. The Sickle Cell Disease Patient. Clinico-epidemiological study of 1550 consecutive patients at Korle Bu Hospital, Accra. T-A’D Co, Watford 1996 http://www.sicklecell.mde/aboutscd.asp
(Reprint of 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. London & Basingstoke, Macmillan Press Ltd 1991/1992. Foreword by Roland B Scott MD, Howard Univ, Washington DC)

11 Konotey-Ahulu FID. Sickle Cell Disease In Successive Ghanaian Generations For Three Centuries (Manya Krobo Tribe) 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.

12 Konotey-Ahulu FID. Effect of environment on sickle cell disease in West Africa; epidemiologic and clinical considerations. Chapter 3 in SICKLE CELL DISEASE – diagnosis, management, education and research. Eds Ahramson, Bertles JF, Wethers Doris L; St Louis – CV Mosby Co 1973, pp 20-38.

13 Konotey-Ahulu FID. The Sickle Cell Diseases: Clinical manifestations including the Sickle Crisis. Archives Intern Med 1974; 133: 611-619. http://archinte.ama.assn.org/cgi/reprint/133/4/611.pdf

14 Konotey-Ahulu FID. The liver in sickle cell disease. Clinical aspects. Ghana Med J 1969; 8: 104-118. (First ever use of word “Gnathopathy”)

15 Konotey-Ahulu FID. Mental nerve neuropathy: a complication of sickle cell crisis. Lancet 1972; 2: 388 [Constitutes discovery of a new physical sign in Clinical Medicine – The kanumblll sign spells out who discovered it, what it is, and where published: “konotey-ahulu numb lower lip Lancet” sign 1972]

16 Ranney Helen. Summary of Symposium 1972 on Sickle Cell Disease – Diagnosis, Management, Education and Research – In SICKLE CELL DISEASE, Eds H Abramson, John F Bertles, Doris Wethers (C Mosby Co), 1973, page 320: “There is no single clinical experience in the United States comparable to that of Dr Konotey-Ahulu”

17 Konotey-Ahulu FID. Four bodyguards and the perils of unmasking scientific truths www.bmj.com/cgi/reprint/335/7612/210.pdf BMJ 2007; 335: 210-11 BMJ July28 2007.

18 Konotey-Ahulu FID. Beware of symptomatic sickle cell traits. Lancet 1992; 339: 555. Doi:10.1016/0140-6736(92)90377-F (Pointing out that the Sickle Cell Trait ‘AS’ may not be true Sickle Trait, and that the Haemoglobin C Trait ‘AC’, may be something else, as the “A” has been shown could stand for Hb Quebec-Chori, by Witkowska et al – New Eng J Med 1991; 325: 1150-54 http://www.thelancet.com/journals/lancet/article/PII0140-6736(92)90377-F/fulltext

19 Konotey-Ahulu FID. Blaming sudden death on Sickle Cell Trait? Flaws in article of Charis Kepron, Gino Sobers, Michael Pollanen Exposed Sept 14 2011 www.sicklecell.md/blog/?p=105 or www.konotey-ahulu.com/blog/?p=105

20 Konotey-Ahulu FID. Sickle cell Trait Misinformation and Disinformation Nov 30 2011 www.sicklecell.md/blog/?=108 [Comprehensive Review]

21 Konotey-Ahulu FID. Further Communication on “Sickle Cell Trait Misinformation and Disinformation” and Sickle Cell Terminology: Disease or Disorder? www.sicklecell.md/blog/?p=127 April 16 2012

22 Boyo Alex E, Cabannes R, Conley CR, Lehmann H, Luzzatto L, Milner PF, Ringelhann B, Weatherall DJ, Barrai I, Konotey-Ahulu FID, Motulsky AG. WHO (Geneva) Scientific Group on Treatment of Haemoglobinopathies and Allied Disorders. (Technical Report) 1972; 509: 83 pages.

23 Serjeant GR. Sickle Cell Disease. Oxford, Oxford University Press, 1985.

24 Serjeant GR. Sickle cell disease. Lancet 1997; 35: 725-730.

25 Serjeant GR, Richards R., Barbor PRH, Milner PF. Relatively benign sickle cell anaemia in 60 patients aged over 30 in the West Indies. BMJ 1968; 3: 86

26 Serjeant GR, Serjeant BE, Milner PF. The irreversibly sickle cell: a determinant for haemolysis in sickle cell anaemia. Br J Haematol 1969; 17: 527-533.

27 Serjeant GR, Galloway RE, Gueri MC. Oral zinc sulphate in sickle cell ulcers. Lancet 1970; 2: 891

28 Serjeant GR. The clinical picture of sickle cell anaemia in Jamaica. MD Thesis, University of Cambridge, 1971.

29 Serjeant GR, Ashcroft. Shortening of the digits in sickle cell anaemia. A sequel of the hand-foot syndrome. Trop Gegraph Med 1971; 23: 341-346.

30 Serjeant GR, et al 1972. The conjunctival sign in sickle cell anaemia. JAMA 1972; 219: 1428-31

31 Serjeant GR, et al. The clinical features of sickle cell beta-thalassaemia in Jamaica. Brit J Haematolol 1973; 24: 19-30

32 Serjeant GR, et al. The clinical features of haemoglobin SC disease in Jamaica. Brit J Haematolol 1973; 24: 491-500.

33 Serjeant GR et al. Screening of cord blood for the detection of sickle cell disease in Jamaica. Clim Chem 1974; 20: 666-69.

34 Serjeant GR et al. The internal auditory canal and sensori-neural hearing loss in homozygous sickle cell disease. J Laryngol Otol 1975: 89: 453-455

35 Serjeant GR. Sickle Cell Disease, 2nd Ed. Oxford, Oxf Univ Press, 1992.

36 Serjeant GR, De Ceulaer C, Lethbridge R, et al. The painful crisis of homozygous sickle cell disease. Br J Haematol 1994; 87: 586-591.

37 Serjeant GR, Serjeant B. Sickle Cell Disease, 3rd Ed, Oxford, OUP, 2001.

38 Serjeant GR. Blood transfusion in sickle cell disease. A cautionary tale. Lancet 2003; 361: 1659-60 [Graham Serjeant’s adult not-transfused patient in Jamaica went on holiday in USA, was transfused and died!].

39 Konotey-Ahulu FID Konotey-Ahulu FID. Sicklaemic human hygrometers. Lancet 1965; 1:1003 1004 [Listing precipitating causes of crises eg hot weather etc] [http://www.pubmedcentral.nih.gov/picender.fcgi?artid=1846286&blobtype=pdf

40 Konotey-Ahulu FID. Torrential epistaxis associated with symmetrical facial skin ulceration in sickle cell anaemia. BMJ 1965; 2:859-860 doi:10.1136/bmj2.5466.859
http://www.bmj.com/cgi/reprint/2/5466/859.pdf

41 Konotey-Ahulu FID, Kuma Eunice. Skeletal crumbling in sickle cell anaemia complicated by Salmonella typhi infection. Brit J Clin Practice 1965; 19: 575-578.
http://www.pubmedcentral.nih.gov/picender.fcgi?artid=1846577&blobtype=pdf

42 Konotey-Ahulu FID. Acute osteomyelitis in African children unmasks un-masks sickle cell disease with salmonellosis BMJ Rapid Response February 1 2014 http://www.bmj.com/content/348/bmj.g66?tab=response

43 Konotey-Ahulu FID. Erectile dysfunction: Test, please, for Sickle Cell Disease www.bmj.com/content/348.g129

44 Konotey-Ahulu FID. Sickle cell disease and the patient Lancet 2005; 382-383 Jan 29-Feb 4 [Importance of Circumstances in initiating crises]

45 Konotey-Ahulu FID. Epistaxis from sickle cell disease must not be forgotten www.bmj.com/content/344/bmj.e1097/rr/576087 28 March 2012

46 Konotey-Ahulu FID. Valsalva vitreous haemorrhage and retinopathy in sickle cell haemoglobin C disease. Lancet 1997; 349: 1774

47 Archampong EQ, Konotey-Ahulu FID. Biliary tract disease and sickle cell anaemia in Korle Bu Hospital, Accra. Ghana Med J 1975; 14: 176-180

48 Ringelhann Bela, Konotey-Ahulu FID. Haemoglobinopathies and Thalassaemias in Mediterranean areas and in West Africa: Historical and other perspectives 1910 to 1997. Accademia del Scienze Ferrara Atti, volume 74, Anno Accademico 174 1996-97, pages 297-307 [A Century Review]

49 Shoetan Cecilia. I lost my Sickle Cell Disease adult daughter minutes after being given Diamorphine intravenously when she could not breathe. http://www.bmj.com/cgi/eletters/336/7654/1152-a#196520 June 3 2008 BMJ

50 Konotey-Ahulu FID, Ringelhann B. Sickle cell anaemia, sickle cell thalassaemia, sickle cell haemoglobin C disease, and asymptomatic haemoglobin C thalassaemia in one Ghanaian family. BMJ 1969; 1: 607-612. http://www.bmj.com/cgi/reprint/2/5648/48.pdf [4 Hbnopathy phenotypes]

51 Konotey-Ahulu FID. Patterns of clinical haemoglobinopathy. East African Med J 1969; 46: 149-156 (With tables that distinguish phenotypes clinically)

52 The Globe and Mail, Toronto. Tough flights for mosquito nets. “If they are safe for babies and mothers in Africa, why are they not safe enough in Canada for a week?” 18 August 2006.

53 Konotey-Ahulu FID. Malaria and sickle cell disease. BMJ 1971; 2: 710-11.

54 Djabanor FFT, Reindorf CA, Konotey-Ahulu FID. The effect of sickle cell disease on Ghanaian children. In First International Publication No (HSM) 1974; 73-9141: 70-87

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

56 Olujohungbe A, Cinkotal I, Yardumian A. Hydroxyurea therapy for sickle cell disease in Britain. BMJ Editorial 1998; 316: 1689. “Many patients are unwilling to take the drug.”

57 Olujoungbe Ade. Bi-directional trust is needed in pain management in sickle cell disease. BMJ Rapid Response 2 July 1999 to Maxwell K, Streetly A, Bevan D BMJ 1999; 318: 1585-1590.

58 Konotey-Ahulu FID. Ethical issues in pre-natal diagnosis. BMJ 1984; 289: 185 http://www.bmj.com/cgi/reprint/289/6438/185-a.pdf

59 Konotey-Ahulu FID. Refusing to provide a pre-natal 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

60 Konotey-Ahulu FID. The Sickle Achievers (1). Ghanaian Times, July 23 2005 “I cannot think of a single Ghanaian family that did not have or know of someone with sickle cell disease – known by Tribal names”

61 Konotey-Ahulu FID. The Sickle Achievers (2) Ghanaian Times. August 13 2005 “Some time ago I coined the term for cold season Rheumatism which Europeans call Sickle Cell Disease … The ACHEACHE Syndrome (1 ACHE from each parent”. Makes it easy for Genetic Counselling.

62 Konotey-Ahulu FID. Genetic Counselling in sickle cell disease. BMJ 1969; 3: 235 http://www.bmj.com/cgi/reprint/3/5664/235.pdf (Put ‘ACHE’ for ‘BAD’) PERSONAL VIEW doi:10.1136/bmj.2.5648.48

63 Konotey-Ahulu FID. Sickle cell disease. The Case for Family Planning ASTAB Books Ltd, Accra, 1973

64 Konotey-Ahulu FID. Need for ethnic experts to tackle genetic public health. Lancet 2007; 370: 1836 [doi:10.1016/50140-6736(07)61771-1]
www.thelancet.com/journals/lancet/article/PIIS0140-6736(07)61771-1/fulltext

65 Ringelhann B, Konotey-Ahulu FID, Yawson G, Bruce-Tagoe AA, Miller A, Huisman THJ. Alpha Thalassaemia in West Africa. Symposium in Medical Genetics, Debrecen-Hajduszoboslo, Hungary (April 26-29 1976), pp 614-616 in Szabo G and Papp Z, Eds Medical Genetics, Excerpta Medica 1977

66 Konotey-Ahulu FID. Maintenance of high sickling rate in Africa: Role of polygamy. J Trop Med Hyg 1970 Jan; 73(1): 19-21 (38 References)

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

68 Konotey-Ahulu FID. Male procreative superiority index (MPSI): The missing co-efficient in African anthropogenetics. BMJ 1980; 291: 1700-02.
http://www.bmj.com/cgi/reprint/281/6256/1700.pdf

69 Konotey-Ahulu FID. The Male Procreative Superiority Index (MPSI): It’s relevance to genetical counselling. In FIFTY YEARS OF HUMAN GENETICS – A Festschrift and liber amicorium to celebrate the life and work of GEORGE ROBERT FRASER. Ed: Oliver Mayo & Carolyn Leach. Wakefield Press 2007, 1 The Parade West, Kent Town, Sth Australia www.wakefieldpress.com.au

70 Omaboe Letitia, Konotey-Ahulu FID. The Second International Conference on The Achievements of Sickle Cell Disease Patients. Accra 19th July 1995 Conference Brochure

71 Acquaye CTA, Gbedemah KA, Konotey-Ahulu FID. Glucose-6-phosphate Dehydrogenase Deficiency Incidence in Sickle Cell Disease patients in Accra. Ghana Med J 1977; 16: 4-9

72 Konotey-Ahulu FID. G6PD Deficiency in Ghanaians. How to recognise it.
Click ‘BLOG’ on www.sicklecell.md and click on January 2008 for 20 answers.

73 Konotey-Ahulu FID. WORLD SICKLE CELL DAY 19th June 2012 www.sicklecell.md/blog/?p=132 Featuring (i) The Inheritance of Sickle Cell Disease (ii) The Person with Sickle Cell Disease (iii) The Teenager with Sickle Cell Disease (iv) The Adult with Sickle Cell Disease.

74 WORLD SICKLE CELL DAY 19th June 1013. Broadcast Interview by Tunu Louise Roberts emphasising Public Health Approach to management, and Genetic Counselling with NORMACHE as Trait and ACHEACHE as Disease www.sicklecell.md or www.konotey-ahulu.com (Home Page) Also accessed as http://youtu.be/wEyebVIhr7Q [Suitable for patients and parents

75 Konotey-Ahulu FID. Morphine for painful crises in sickle cell disease. BMJ 1991; 302: 1604 (Commenting on Professor Chamberlain’s recommendation of morphine in pregnancy in sickle cell disease – BMJ 1991; 302: 1327-30). “In obstetrics what happens too foetal respiration when morphine is used?” http://www.bmj.com/cgi/reprint/302/6792/1604-c.pdf

76 Konotey-Ahulu FID. Management of patients with sickle cell disease. African Journal of Health Sciences 1998; 5: 47 [On Sally Davies and Lola Oni in BMJ 315: 656-60 “The Central Middlesex management protocol uses morphine infusions”] Response: “I fear Davies and Oni’s statement will make morphine the accepted drug for sickle crisis management. The consequences for such an approach are dire, especially when some UK hospitals are already making diamorphine their first choice”. And what did NCEPOD find in 2008?

77 Konotey-Ahulu FID. Opiates for sickle cell crisis. Lancet 1998; 352: 651-2

78 Konotey-Ahulu FID. Opiates for pain in dying patients and in those with sickle cell disease www.bmj.com/cgi/eletters/335/7622/685#177986 BMJ Response 11 Oct 2007

79 Konotey-Ahulu FID. Current “hit and miss” care provision for sickle cell disease patients in the UK. BMJ Rapid Response 16 July 2008. http://www.bmj.com/cgi/eletters/337/jul11_2/a/771#199135

80 Konotey-Ahulu FID. Management of sickle cell disease versus management of the sickle cell disease patient. BMJ Rapid Response 17 Sept 2008 http://www.bmj.com/cgi/eletters/337/sep08_1/a1397#202088

81 Konotey-Ahulu FID. Clinicians facing conflicting recommendations. Use common-sense? http://ww.bmj.com/cgi/eletters/337/nov28_2/a2530#205377 December 2008.

82 Konotey-Ahulu FID. Poor care for sickle cell disease patients: This wake-up call is overdue. BMJ Rapid Response (May 28) BMJ 2008; 336: 1152 http://www.bmj.com/cgi/eletters/336/7654/1152-a#196244 to Susan Mayor’s “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)” http://www.info@ncepod.com

83 Konotey-Ahulu FID. Inquest into diamorphine deaths: Does NCEPOD sickle cell patients report warrant a similar inquest? BMJ Rapid Response March 7 2009 http://www.bmj.com/cgi/eletters/338/mar03_3/b903#210208

84 Konotey-Ahulu FID. UK Drug related deaths are still rising: So where is NICE? http://www.bmj.com/cgi/eletters/sep01_1/b3536#219836 BMJ Rapid Response to S Mayor: “UK drug related deaths are still rising.” Sept 6 2009. .

85 Konotey-Ahulu FID. The politics and economics of pain relief in the West and Third World. http://www.bmj.com/cgi/eletters/341/aug11_2/c3800#240684 Response Aug 24 to T Anderson doi:10.1136/bmj.c3800 Aug 11 2010

86 Konotey-Ahulu FID. Management of an acute painful sickle cell episode in hospital. NICE Guidance is frightening! BMJ Rapid Response Sept 7 2012 www.bmj.com/content/344/bmj.e4063/rr/599158 (42 References)

87 Konotey-Ahulu FID. Opiods for chronic non-cancer pain – Chemotherapy – Clinical Guidelines: Where does ultimate responsibility lie? BMJ Rapid Response www.bmj.com/content/346/bmj.f2937/rr/651421 June 25 2013

88 Dankwa Akosua M. Sickle Cell patients deserve to live. BMJ Rapid Response to NCEPOD Report 11 July 2008 [Adult “SS” wrote to the BMJ:
http://www.bmj.com/cgi/eletters/336/7654/1152-a “I know 60 and 70 year olds (yes, sickle cell patients) who have got to that age without ever receiving this as treatment”.

89 Chapman Nyaho Mawunu. Poor care for the sickle cell disease patient: “Pain won’t kill him, but Morphine could”. BMJ Rapid Response to NCEPOD by adult Sickle cell haemoglobin C disease grand-mother 17 June 2008 http://www.bmj.com/cgi/eletters/336/7654/1152a

90 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 ISBN: 978-9988-1-3927-8 “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 remember 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’” [page 15] Quoted in full in link of Reference 85 above. [Dorothy Amanor-Boadu herself is a 59-year old “SS” Nurse Oncologist in Accra].

Competing interests: Coming from a family with 3 Sickle Cell Disease (scd) siblings I find it very difficult to remain detached when the received wisdom in the management of scd patients that was proven by NCEPOD to be harmful is still endorsed by NICE.

FELIX ID KONOTEY-AHULU, Kwegyir Aggrey Distinguished Professor of Human Genetics, University of Cape Coast, Ghana

Consultant Physiician Genetic Counsellor in Sickle Cell and Other Haemoglobinopathies, 9 Harley Street Ltd., Phoenix Hospital Group, London W1G 9AL

Professor George Ebow Bonney, Ph.D.
Research Scientist, Musician, and Christian
Tribute by Professor Felix I D Konotey-Ahulu*

He was a genius! George Ebow Bonney could have read any subject in university and been able to excel at whatever he did. He chose Mathematics with special emphasis on Statistics, making a huge impact on both sides of The Atlantic. It was my great fortune to have had George work with me at the erstwhile Ghana Institute of Clinical Genetics at the Korle Bu Teaching Hospital in Accra.

RESEARCH

Using students on summer holiday from the then 3 main universities (Legon, Cape Coast, and Kumasi) and paying them handsomely I had organized a countrywide survey of male/female differential procreation to decide how this affected the prevalence of the Sickle Cell Gene in the various regions in Ghana. We had just found that males, indeed, had more children than females when George joined us on the advice of geneticist Professor Ebenezer Laing of the University of Ghana. “George”, I said to him “I want a mathematical underpinning of this fact known to all Africans, but which Europeans appear to think is nonsense because they cannot imagine how a man can have more children than a woman”. I presented him with our data, and in no time George produced a marvelous article which we published in the world’s leading Science magazine with him as senior author entitled: “POLYGAMY AND GENETIC EQUILIBRIUM” We travelled to Debrecen in Hungary to present papers at the International Congress in Human Genetics.

We next embarked on Twin Studies from 13,000 consecutive births at the Korle Bu Teaching Hospital. Our unique findings that in Ghana 1 in every 30 consecutive deliveries produced twins compared with 1 in 80 single births in Britain, 1 for 86 in the USA, and 1 in 145 in Japan enthralled geneticists. George Bonney was the one to present the findings of our team (Mary Walker, Koblah Gbedemah, and me) at the International Congress on Twin Studies in the USA, available today as Proceedings of the Second International Congress on Twin Studies Part C, Editor Walter Nance in Progress in Clinical and Biological Research 1978; Volume 24 Pt B, pages 105 to 108.

George Bonney was simply, simply brilliant. When he crossed the Atlantic his worth was soon recognized. His field of research broadened from Genetic Epidemiology, to Statistical Analysis, Mapping of Human Chromosomes, Breast Cancer, Alcoholism, Nicotine Dependence and, recently, Prostate Cancer. Louisiana State University and Howard University held him in very high esteem. Teamed up with Professor Georgia Dunston and brilliant Others at the Howard University Genome Project, George Bonney’s input was well acknowledged. I myself benefited much when he and Professor Dunston invited me to contribute to the First International Inaugural Conference on The Human Genome in Washington DC in 2009. The measure of his global impact can be gauged from the 1988 Tenth Annual George W Sender Award for (wait for it) “The best published work in Biometry in any journal sponsored jointly by the American Statistical Association & the Biometrics Societies of North America” [For researchers seeking to check on this see (i) “Regressive logistic models for familial disease and other binary traits” Biometrics 1986; Volume 42: pages 611 to 625, and also (ii) Biometrics 1987; Volume 43: pages 951 to 973]. He published right up to 2012.

In one National Award Ceremony in the USA, when his name was announced to receive First Prize the audience was expecting to see a Japanese walk to the podium (the name “Bonney” sounded rather Japanese), and were astonished to behold a Ghanaian African walk up accompanied by great applause. I have quite deliberately gone into some detail of what George Ebow Bonney achieved in Science not only to encourage Africa’s younger generation to emulate his hard work, but also (as we shall see in the next section) to debunk the present all-pervading aggressive atheism that says brilliant people do not accept the existence of GOD.

GEORGE BONNEY’S FAITH EXPRESSED IN MUSIC

George’s musical proclivities became evident when, at the University of Ghana, he directed the Male Voice Choir of the University Christian Union. He played the piano and the organ with virtuosity, and his detailed knowledge of Tonic Sol-fa made it easy, and a joy, for him to teach all 4 vocal parts to singers. Remarks Reindorf Baah Perbi, himself an attractive bass voice singer: “I think it was in the Male Voice Choir that some of us learnt the song ‘When we all get to Heaven’” the first verse with chorus of which goes:

Sing the wondrous love of Jesus
Sing His mercy and His grace
In the mansions bright and blessed
He’ll prepare for us a place.
When we all get to heaven
What a day of rejoicing it will be!
When we all see Jesus
We’ll sing and shout the victory!

But Joanna Nerquaye-Tetteh quickly adds that it was not just the male choir that George Bonney taught sacred music to: “He taught the Female Voice Choir too, and he taught us to sing ‘It’s not an easy road, but The LORD is with us!’”

Capt. James Hackman Tachie-Menson’s Hymnal and Book of Anthems
To me Professor George Bonney’s musical legacy is entwined with that of Capt. James H Tachie-Menson whose 4 books are a MUST READ and a MUST SING by all, and I mean all, Ghanaians. I am constrained (if I am to do justice to the sweet memory of George Bonney) to quote the first paragraph of his Foreword to the four extraordinary books:“The four volumes of Songs from Land and Sea: The Captain’s Hymnal in Staff Notation, The Captain’s Hymnal in Tonic Sol-fa Notation, The Captain’s Book of Anthems in Staff Notation, and the Captain’s Book of Anthems in Tonic Sol-fa Notation, bring together for the first

“The four volumes of Songs from Land and Sea: The Captain’s Hymnal in Staff Notation, The Captain’s Hymnal in Tonic Sol-fa Notation, The Captain’s Book of Anthems in Staff Notation, and the Captain’s Book of Anthems in Tonic Sol-fa Notation, bring together for the first time, the sacred compositions of Captain James Hackman Tachie-Menson. I have had indescribable joy collaborating with Captain to produce the First Editions of his work.”

For George Bonney to manage to render each (and every one) of these compositions into Tonic Sol- fa, not only for Treble, but also for Alto, Tenor, and Bass is a mark of rare genius. I do not know of any Professor of Music anywhere in the world who could have done that. Captain James H Tachie-Menson says it all in his Preface to the books:

“I would like to give my very special and sincere thanks to Professor George E. Bonney of Howard University in Washington D.C., the originator and coordinator of the project, who of his own volition and initiative personally transcribed the music from staff notation to tonic sol-fa. He has worked tirelessly to bring this project to fruition”.

GIVEN TO HOSPITALITY

George Ebow Bonney was blessed by the Lord Jesus Christ in the gift of Ewura Efua, that wonderful wife of his. What a privilege for Rosemary, my wife, and myself to be very welcome guests in their beautiful home in Rockville, Maryland in the USA. Many others have testified to their hospitality. We saw and profited from their love for The Lord Jesus who equipped Ewura Efua to live with the punishing schedule of her husband who worked long hours, and appeared to do many things simultaneously. Oh how George will be missed!

My heartfelt Condolences and Rosemary’s go to gallant Ewura Efua, and their children and families Aba Bonney Kwawu and Erwin Kwawu and their children Sela Kwawu and Eli Kwawu, and Kofi Bonney and wife Nicole Bonney, and the larger families in Ghana. May the GOD of all comfort surround them with His amazing love in The Lord Jesus Christ through the power of The Holy Spirit. [2 Corinthians chapter 1 verses 3 & 4]. As for George Ebow Bonney, what a Prospect!

H E A V E N

Oh think to step ashore, and that shore Heaven; To take hold of a hand, and that hand God’s hand; To breathe a new air, and that air Celestial air;
To feel invigorated, and know it, Immortality; Oh think, to pass from the storm and tempest To one unbroken smile,
To wake, and find it GLORY!

*Felix I D Konotey-Ahulu is Dr 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 Ltd, Phoenix Hospital Group, London W1G 9AL [ www.konotey- ahulu.com & www.sicklecell.md ]

Posted on May 15, 2013 by admin.sicklecell.md Rapid Response in British Medical Journal

Felix I D Konotey-Ahulu www.bmj.com/content/345/bmj.f2855/rr/645095 12 May 2013 response to

Most religious followers support assisted suicide for the dying.

British Medical Journal – 11 May 2013 Volume 346, p3 – NEWS

Zosia Kmietowicz www.bmj.com/content/345/bmj.f2855?sso= 

Most religious followers supported assisted suicide for the dying: Survey flawed through inadequate definition of “religious” and “terminally ill”.

Those who “said that they followed a religion” were regarded as “religious followers” [1] and their opinions about “a change in the law to allow assisted suicide for people who are terminally ill” recorded in a survey which I consider deeply flawed because of inadequate definition of “religious” and, when Palliative Care Experts are not themselves unanimous [2] in agreeing on who can be called “terminally ill”, how was the term explained to the “nearly 4500 adults” in the survey? [1]

CHURCH OF ENGLAND QUERIES SURVEY

While it was claimed that there was “strong support for a change in the law” from 72% of 1519 Anglicans in the survey organised by Linda Woodhead, Lancaster University Professor of Sociology of Religion, the Church of England spokesman, quite rightly in my view, said such an important subject “cannot be effectively conducted through the medium of online surveys” – a criticism that Professor Woodhead apparently dismissed on the grounds that she was better placed to define who an Anglican follower was than the Church herself.

ETHICS, NOT SOCIOLOGY, IS CRUX OF THE MATTER

The second flaw in Professor Linda Woodhead’s survey is a lack of distinction between legality and ethics when it comes to questions of assisted suicide. To assume that “the Law” is all that needs consideration in this matter is grossly mistaken. I have previously pointed out in the BMJ how two brilliant British Professors of Genetics, both FRS, treated the ethical principle surrounding a genetic defect I had described in the BMJ, in two entirely different ways in their well known textbooks of Genetics. One FRS used my story to underline the importance of Ethics in Clinical Medicine. The other FRS, also publishing details of my story, did not even mention the word “ethics” in his Genetics book. Assisted suicide is bigger than sociology of religion. Any survey done without highlighting the ethical dimension, regardless of the brilliance and reputation of the organisers of the survey, is deeply flawed.

RELIGION IS BROADER THAN CHURCH, MOSQUE, SYNAGOGUE

Third Flaw: Professor Linda Woodhead needs no reminding that there are atheists who are deeply religious. Some of my brilliant atheistic friends and acquaintances worship Scientific Humanism. If they are also labelled as religious (as they need to be) then does the thrust of the survey with the message it seems to convey not become meaningless?

FACE-TO-FACE SURVEYS PREFERABLE IN CONTROVERSIAL MATTERS

In controversial matters it is always good policy to demand face to face interviews where the interviewer can be asked questions such as: (i) “What is your definition of ‘religion’?” (ii) “Have some terminally ill patients not been known to improve unexpectedly and gone home?” (iii) “Why was the present law on assisted suicide acceptable a decade ago, but not now?” (iv) “If Assisted Suicide is made legal in the UK does that make it ethical?” (v) “Does Hitler’s Nazi Germany have nothing to teach Great Britain?”

Felix I D Konotey-Ahulu MD(Lond) FRCP DTMH [felix@konotey-ahulu.com]

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 Ltd, Phoenix Hosp[ital Group, London W1G 9AL

Competing interests: I am Christian but I do not refer to myself as “religious”.

1 Kmietowicz Zosia. Most religious followers support assisted suicide for the dying. BMJ 201`3; 346: f2855 (11 May, page 3) www.bmj.com/content/346/bmj.f2855?sso

2 Konotey-Ahulu FID. Liverpool care pathway BMJ and Channel Four News: Majority expert choice does not mean best choice.www.bmj.com/content/346/bmj.f1303/rr/634971 March 8 Rapid Response to “Nine out of 10 palliative care experts would choose Liverpool care pathway for themselves” Krishna Chinthapalli in BMJ 2013; 346: 1103 (March 2, pages 2-3) 

Competing interests: None declared

Article appeared in the BMJ – www.bmj.com/content/346/bmj.f2855/rr/645095

by Felix I D Konotey-Ahulu (May 12 2013)

For World Sickle Cell Day 19th June 2012 it is worth reminding ourselves of the 4 consecutive articles I wrote for New African (London) in Jan/March/June/September 2001.

Click to view PDF Document

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

TASC UNIT

http://www.sicklecelleducation.com

http://www.sicklecellanaemia.org

 

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

 

—————————————————————————————–

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

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