Music (and Tonal Language) and the art of being human – III

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
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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

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Ethics of mitochondrial gene replacement is also ethics of acquired genetic inheritance

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

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Antenatal sickle cell disease haemoglobinopathy screening

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.

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The politics (and economics) of pain relief in the West and Third World

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

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Third International Conference on the Achievements of Sickle Cell Disease Patients

The Third International Conference on the Achievements of Sickle Cell Disease Patients will take place at the Accra International Conference Centre on Monday 19th July, the day before the First World Congress On Sickle Cell Disease begins at the same venue. Attendance is free. Contact Co-Chairs Ms Dorothy Amanor-Boadu (elitens@yahoo.co.uk), Professor Alex Bruce-Tagoe (alexbt_2000@yahoo.com) and Professor Felix Konotey-Ahulu (felix@konotey-ahulu.com), and also the Local Chair for the World Congress Dr Ivy Ekem (ekem_ivy@hotmail.com)

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Current “hit and miss” care provision for sickle cell disease patients in the UK

Current “hit and miss” care provision for sickle cell disease patients in the UK

Following her article [1] on NCEPOD [2] Susan Mayor has, again, produced a succinct summary [3] of the recent publication of ‘Standards’ on the care of patients with sickle cell disease (scd) in the UK [4], written by a multidisciplinary working group chaired by Consultant Haematologist Dr Ade Olujohungbe. The public launch of the publication was on 9 July in the House of Commons where the Archbishop of York, Dr John Sentamu, said: “These Standards are another step in providing consistent care” [3].

HIT AND MISS CARE PROVISION

Hitherto, consistent care has not been UK’s strong point. The description by the ‘Standards’ Group’s chairman, Dr Olujohungbe who, in my opinion, has proved to be the most experienced Clinical Haematologist in the UK on the scd patient since Hammersmith Hospital’s Professor Lucio Luzzatto with whom Olujohungbe was closely associated, underlines what Graham Serjeant [5] and I have bemoaned for years. Olujohungbe said “The care provision for sickle cell disease is currently hit and miss, depending on the attitudes and experience of health professionals” [3]. This sad diagnosis by the leading UK haematologist on the scd patient was precisely why “NCEPOD found that many patients died of complications caused by excessive doses of opiods” [1, 2].

RESPONSE OF TEN DOWNING STREET

During the week when UK media (radio, television, & newspapers) were full of news that the Prime Minister was inviting personal phone calls and contacts on people’s concerns I took the opportunity to write to The Right Honorable Mr. Gordon Brown drawing his attention not only to the publication of the NCEPOD Report [2], but also to the international responses that were beginning to pour in [6]. The reply I got, dated 25 June 2008, from Ten Downing Street was encouraging: “Dear Dr Konotey-Ahulu – The Prime Minister has asked me to thank you for your recent letter and enclosures. Mr. Brown has asked that your letter be forwarded to the Department of Health so that they may reply to you on his behalf. (Signed) MR R SMITH”.

RESPONSE OF THE DEPARTMENT OF HEALTH

I looked forward eagerly to answers to the 3 questions I posed in my correspondence, namely (i) With respect to scd patients dying with overdose of opiods (heroin & morphine) in their blood “What kind of supervision led to this?” [6a] (ii) Why should West Indians and West Africans who did without morphine in their countries be put on morphine pumps when they were admitted to UK hospitals? [6a] (iii) To those UK haematologists who say “unbearable pain is unbearable pain, which must be treated with the most potent analgesic drugs known” I posed this third question, how many of them would prescribe diamorphine (heroin) monthly for their teenage daughters whose dysmenorrhoea was simply unbearable? [6a]. The Department of Health wrote back to me Ref; TO00000325139 dated 16 July 2008. Signed by “Shelley Wilson, Customer Service Centre”. All the questions were totally ignored. Would the National Institute of Clinical Excellence (NICE) now provide specific answers to these my 3 questions?

OUR GENETIC FUTURE

Africans, African-Caribbeans, and African Americans must wake up and realize that their genetic future depends on themselves, and not on any Department of Health. We must reduce the genetic disease burden, starting now, otherwise their children will continue to be subjected to “hit and miss” health care provision, ending up on heroin and morphine pumps. “Unless we Africans are involved in genetic counseling” and voluntary family size limitation (GCVFSL), I said not long ago “the genetic burden on the National Health Service will go up and up” [7]. What is more to the point, unless we take this very seriously our children and grand children will suffer greatly from scd [ACHEACHE syndrome], for “one in three West Africans in the UK has a beta-globin variant gene (ie NORMACHE) whose unsuspecting owner needs to be identified and helped with genetic counseling and family size limitation” [7]. Those who do not quite believe the enormity of the NCEPOD Report [2] should, please, start by taking a good look at how the rest of the world regards the “hit and miss” approach to the present care of sickle cell disease patients in the United Kingdom [6a-6j].

Felix I D Konotey-Ahulu MD(Lond) FRCP(Lond) DTMH(L’pool) – Kwegyir
Aggrey Distinguished Professor of Human Genetics, University of Cape Coast, Ghana and Consultant Physician Genetic Counsellor in Haemoglobinopathies, London W1G 9PF.

felix@konotey-ahulu.com

1 Mayor Susan. Enquiry shows poor care for patients with sickle cell disease. BMJ 2008; 336: 1152

2 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 (Researcher), Tom Treasurer (Chairman)] info@ncepod.org

3 Mayor Susan. Group publishes standards for adult sickle cell disease to reduce number of unexplained deaths. BMJ 2008;337:a771

4 Sickle Cell Society (London) The Standards for the Clinical Care of Adults with Sickle Cell Disease in the UK. http://www.sicklecellsociety.org

5 Serjeant GR. The case for dedicated sickle cell centres. BMJ 2007; 334: 477 (3 March)

6a http://www.bmj.com/cgi/eletters/336/7654/1152-a#196224 [Felix I D Konotey-Ahulu 29 May 2008] Poor care for sickle cell disease patients: This wake up call is overdue

6b http://www.bmj.com/cgi/eletters/336/7654/1152-a#196359 [Kwabena Frimpong-Boateng 30 May 2008] Re: Poor care for sickle cell disease patients: This wake up call is overdue

6c http://www.bmj.com/cgi/eletters/336/7654/1152-a#196514 [Marianne Janosi 3 June 2008] “Poor care for patients with sickle cell disease” BMJ 24 May 2008 Volume 336.

6d http://www.bmj.com/cgi/eletters/336/7654/1152-a#196520 [Cecilia Shoetan 3 June 2008] I lost my Sickle Cell disease adult daughter minutes after being given Diamorphine intravenously when she could not breathe.

6e http://www.bmj.com/cgi/eletters/336/7654/1152-a#196631 [Frank Edwin 5 June 2008] Re: Poor care for sickle cell disease patients: This wake up call is overdue

6f http://www.bmj.com/cgi/eletters/336/7654/1152-a#196848 [Ivy Ekem 9 June 2008] Care for sickle cell patients

6g http://www.bmj.com/cgi/eletters/336/7654/1152-a#197301 [Mawunu Chapman Nyaho 17 June 2008] Poor care for the sickle cell disease patient: “Pain won't kill him, but Morphine could”.

6h http://www.bmj.com/cgi/eletters/336/7654/1152-a#197350 [Emmanuel Jeurry Blankson 18 June 2008] Sickle Cell Disease is managed, NOT treated.

6i http://www.bmj.com/cgi/eletters/336/7654/1152-a#197377 [Yolande M Agble 18 June 2008] Re: Poor care for sickle cell disease patients: This wake up call is overdue.

6j http://www.bmj.com/cgi/eletters/336/7654/1152-a#198669 [Akosua M Dankwa 11 July 2008] Sickle Cell patients deserve to live.

7 Konotey-Ahulu FID. Need for ethnic experts to tackle genetic public health. Lancet 2007; 370: 1826 (December 1)

Competing interests: I come from a sickle cell disease (scd) family, my parents were traits (NORMACHE), and I am actively involved in genetic counselling to reduce the burden of sickle cell disease (ACHEACHE) in future generations.

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