FOR EDUCATION OF DOCTORS: Painful Hip In Black And White Sickle Cell Disease Women

Painful hip in Black and White Sickle Cell Disease Women

The article in the British Medical Journal of Lamb JN, Holton C, O’Connor P, Giannoudis PV. Avascular necrosis of the hip. BMJ 1 June 2019, Vol 365, p 325 (BMJ 2019; 365:I2178) mentioned a woman who had two pregnancies presenting with painful hip due to avascular necrosis of the hip, but the authors neither associated the pregnancy with the hip problem, nor did they mention the nationality of the patient. They listed sickle cell anaemia among the factors that could cause the particular hip problem of avascular necrosis but there was nothing to show that the lady the authors wrote about had sickle cell anaemia which is known to occur in white as well as black patients.

I wrote to the British Medical Journal responding to the article, and required more information about the nationality of the patient, and whether haemoglobin electrophoresis was done to prove “sickle cell anaemia” (SS) or sickle cell haemoglobin C disease (SC) which latter, I said, was more to be associated with hip necrosis than the former because the blood was thicker in the “SC” than the “SS”. But when the article was published my queries were omitted, and information that would assist GP’s in the UK and elsewhere appeared to have been excised inadvertently even though the references to the information were retained: Konotey-Ahulu FID. Avascular hip necrosis: sickle cell haemoglobinopathy predominates worldwide. June 1 2019 https://www.bmj.com/content/365/bmj.l2178 BMJ Rapid Response to Lamb JN et al 1 June 2019, Vol 365, p 325 (BMJ 2019; 365:l2178.

I took advantage of BMJ publication a fortnight later June 15 asking whether MEDICAL FIRMS should come back – a “Firm” being the term used in the days I was a medical student over six decades ago to comprise a Team of doctors in a Teaching Hospital from Consultants down to House Officers and supporting staff in such a cohesive form as is missing today – I took advantage of this BMJ publication to bring back the sections that were missing in my June 1 article so that GP’s may see the true nature of hip problems in both black and white sickle cell disease women demonstrated admirably by Professor Malcolm Milne’s excellent Firm at Westminster Hospital on a postgraduate ward round in 1962.

DOCTORS PLEASE PAY ATTENTION!

Should we bring back THE FIRM? Yes, please! Firms Vary In Excellence: Pregnancy and Sickle Cell Hip Necrosis Revisited.

BMJ’s Cover Page question 15 June 2019 “Should we bring back THE FIRM?” [1] helps me to draw attention to Professor of Medicine Malcolm Milne’s Firm at Westminster Hospital same period as that of Professor Harold Ellis who says of those days “The firms were wonderful”?.[2]. I totally agree. I knew both firms as a postgraduate chasing MRCP’s. In fact, Harold Ellis removed my appendix in August 1963, and you could hardly see the 1-inch long scar. Their Firms were remarkable; that was why I was peeved when the Grand Round of Professor Malcolm Milne that I described in response to Jonathan Lamb and Colleagues’ article on aseptic necrosis of the hip [3] was inadvertently removed. [4]. I reproduce below those sections edited out by mistake, even when their references were retained. GP’s that Lamb et al had in mind for education need these facts about the hip in sickle cell disease. Doctors must think “multiple pregnancies” when an African woman walks into their consulting room with a limp. They must take a detailed history of how long they have been in labour because Lamb and colleagues mentioned two pregnancies [3], but nothing about length of labour which, with legs in prolonged abduction, compromises blood supply to the femoral heads doing worse damage to the high haematocrit sickle cell haemoglobin C disease (SC) and sickle cell beta-thalassaemia (Sbeta-Thal) patients than in sickle cell anaemia (SS) [5-8].

FIRMS OF PROFESSOR HAROLD ELLIS AND PROFESSOR MALCOLM MILNE WERE MORE EXCELLENT THAN SOME OTHER FIRMS.

Medical and Surgical Firms were not equally efficient. Which Medical Firm in the UK would assemble (like Professor Malcolm Milne’s did) the author of an article 8 years earlier (Joe Humble) [8] diagnosing sickle cell disease in a white lady (Greek), then ask an African postgraduate (myself) on Grand Rounds to examine her using clinical approach only (History, Observation, Palpation, Percussion, Auscultation) to arrive at a Clinical Diagnosis of “Sickle Cell beta-Thalassaemia with cholelithiaisis and avascular necrosis of humeral and femoral heads”, then turn to Dr Joe Humble: “Show us the Electrophoresis Strip”? That whole section of my Rapid Response a fortnight ago [4] was excised. I reproduce it so GP’s and others may learn from it. Read this together with my earlier response which stated: “the sickle cell gene frequency in some white people in Greece and in Eti-Turks is greater than that in my own country Ghana”. [4 7 8]. See the excised portion before “Jonathan Lamb et al list fewer conditions …”[4]:-

These and their offspring are scattered around the world including the UK. A white woman was correctly diagnosed by me 57 years ago exactly on Professor Malcolm Milne’s Westminster Hospital Postgraduate Grand Rounds using just History (“I come from Greece”), Examination (icteric eyes, scar in right hypochondrium, loss of left shoulder contour due to angulation, and frequent joint pains) as (in my own words) “Sickle Cell beta-Thalassaemia disease with cholecystectomy for pigment stones, and aseptic necrosis of the left humeral head”. Haemoglobin Electrophoresis was produced on the ward round to confirm my clinical diagnosis by Consultant Haematologist Dr Joe Humble [8, page 240] who 8 years earlier had identified the same lady as having sickle cell Thalassaemia [9].Trained in London University in the 1950’s by the likes of Sir Richard Bayliss (Queen’s Physician) I was trained as it were for the bush in order that I could make diagnoses without X-rays. The luxury of MRI did not exist. Fortunately, there are some GP’s in the UK today who can make this kind of clinical diagnosis on History and Examination alone.

PREGNANCY & PROCREATION LIMITATION & HIP NECROSIS

Jonathan Lamb et al quite rightly mention pregnancy: “She had recently given birth to her second child”. [3] I would like to know how long she was in labour for because prolonged 2nd stage of labour we found in Ghana to be one of the precipitating causes of this condition in the multiparas. I once commented thus:
“The peculiar selection of the hip joint for such crippling pathology might have some eugenic significance [5 7 8]. Some of the Hb SC disease women with bilateral hip involvement complained of inability to separate their thighs for coitus, seriously endangering marital relationships, while many of the male patients with hip involvement find coitus impossible because body movement of any sort produces agonizing pain (Illustrative Case Histories 44, 46 and 48” [Ref 8 below].
I went on: “It seemed to me that in addition to priapism this is a further means that Nature has devised to limit dissemination of the sickle cell gene” [8, page 240].

FURTHER INFORMATION REQUIRED TO EDUCATE US, PLEASE

1. What is the nationality of this lady? Ringelhann and I described in some detail Mediterranean and West African Haemoglobinopathy and G6PD Deficiency. [10]

2. How long was she in labour for in both pregnancies?

3. What does the Haemoglobin Electrophoresis show? I once described two Sickle Cell Haemoglobin D disease girls with an English father (“AD” phenotype) and Ghanaian mother (“AS” phenotype) [Case History 133 in Reference 8]. The “SD” phenotype can be as severe as the “SS” (Sickle Cell Anaemia). Another reason why Hb Electrophoresis is important even when Sickling Test is Negative is that in my 36 consecutive homozygous Haemoglobin C disease patients in Ghana “the 35th presented with hip pain”. [8]

Jonathan Lamb et al list fewer conditions that cause aseptic necrosis of the femoral head than the 25 that J E Nixon [11] listed in 1983 in the Journal of the Royal Society of Medicine, but their article [3] has admirably succeeded in alerting clinicians about this condition which afflicts not just Africans and Caucasians but the entire world. We await answers to the 3 questions listed above, but note that Sickle Cell Anaemia “SS” is not the same as Sickle Cell Haemoglobin C disease “SC”. Sickle Cell Trait is “AS” phenotype.

Competing interests: None declared Twitter @profkonotayahul

F I D Konotey-Ahulu MB BS MD(Lond) FRCP(Lond) FRCP(Glasg) DTMH(L’pool) DSc (Hon UCC) DSc (Hon UH) FGA FGCP FWACP FTWAS, ORDER OF MERIT (OFFICER). Kwegyir Aggrey Distinguished Professor of Human Genetics, Faculty of Science, University of Cape Coast, Ghana, and Former Director Ghana Institute of Clinical Genetics Ghana and Consultant Physician Genetic Counsellor in Sickle Cell and Other Haemoglobinopathies Korle Bu Teaching Hospital Accra, and at Phoenix Hospital Group, 9 Harley Street, London W1G 9AL.

1 BMJ Front Cover. Should we bring back THE FIRM? 15 June 2019. [365 379-420 No 82003 ISSN 1759-2151.

2 Ellis Harold. “The firms were wonderful” quoted with portrait in Abi Rimmer The Firm: could it fix teamworking and morale? BMJ 2019; 365: 14105 June 15 2019.

3 Lamb JN, Holton C, O’Connor P, Giannoudis PV. Avascular necrosis of the hip. BMJ 1 June 2019, Vol 365, p 325 (BMJ 2019; 365:I2178)

4 Konotey-Ahulu FID. Avascular hip necrosis: sickle cell haemoglobinopathy predominates worldwide. June 1 2019 https://www.bmj.com/content/365/bmj.l2178 BMJ Rapid Response to Lamb JN et al 1 June 2019, Vol 365, p 325 (BMJ 2019; 365:l2178.

5 Konotey-Ahulu FID. Hip disease in Africans, Lancet 1970; 1: 99.

6 Konotey-Ahulu FID. Hip pain and radiographic signs of osteoarthritis: Sickle Cell and other haemoglobinopathy differential diagnosis. BMJ Rapid response to Nieuwenhhuisje MJ, Nelissen RG. Hip pain and radiographic signs of osteoarthritis. BMJ 2015; 351:6262 (03 December 2015) BMJ 2015;351:h5983

7 Konotey-Ahulu FID. Pattern of Sickle Cell Disease in Accra. A Study of 1550 consecutive patients. Dissertation presented to University of London for the Postgraduate Diploma of Doctor of Medicine 1971. Awarded MD(London) February 1972.

8 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. The Macmillan Press Ltd, London 1991 & 1992 and T-AD Co Watford 1996.

9 Humble JG. et al. A family illustrating the double inheritance of a sickle cell trait and of Mediterranean anaemia. J Clin Path 1954; 7: 201-208.

10 Ringelhann B, Konotey-Ahulu FID. Haemoglobinopathies and Thalassaemia in Mediterranean areas and in West Africa: historical and other perspectives 1910-1997 – A Century Review. Atti del’Accademia dela Science di Ferrara Haemoglobinopathies 1998: 74: 267-307.

11 Nixon JE. Avascular necrosis of bone: a review. J R Soc Med. 1983; 76: 681-692.

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PAIN CONTROL : DON’T PREGABALIN PATENT LITIGATIONS RAISE LICENSING POLICY QUESTIONS?

The painstaking way (pun intended) that “a doctor, a pharmacist, and a patent attorney” [1] have analysed the 2013 to 2018 “on going disputes around pregabalin” (British Medical Journal June 9 2018, page 358) needs commendation. I don’t comment on particular details because “Supreme Court decision is due soon” [1], but pertinent questions need answers. This excellent account implies how powerful Drug Licensing Authorities are. It is only after drugs have been licensed that MHRA, NHS, NICE, or BNF issue instructions.  Can we identify the composition of Licensing Bodies so we may hold persons to account individually when undesirable sequelae follow their decision to license a particular drug?

Adverse Clinical Consequences Following Licensing One Drug and Not Another    

I realised over 20 years ago the power Drug Licensing had in the UK when I began pointing out that Morphine and Diamorphine, through respiratory suppression, killed sickle cell disease patients in painful crises.  “If the patient dies” I said in Lancet “sickle cell crisis and chest syndrome will be recorded on the death certificate” [2 3]. When a UK Professor of Obstetrics & Gynaecology [4] advocated Morphine for sickle cell crisis patients in pregnancy, I said in BMJ: “The question that puzzles me is: Why do west African and West Indian patients with sickle cell disease who did without morphine in their countries have to be given morphine pumps during sickle cell crises when they come to the United Kingdom? In any case, in obstetrics what happens to foetal respiration when morphine is used?” [5]

Professor Elisabeth Goodman [6] found Ketorolac as good as Morphine with no   respiratory depression in vaso-occlusive crisis but Liesner, Vandenberghe and Sally Davies said “Ketorolac has no product licence in the UK for this indication” [7]

How extraordinary that a drug that did not kill patients had no Product Licence in the UK, but Morphine and Diamorphine that killed sickle cell disease patients as NCEPOD later confirmed [8] was, and still is, licensed “for this indication”! Indeed, despite NCEPOD’s damning patients-dying-from-Opiate-Overdose Report NICE issued Guidelines advocating Diamorphine intravenously in sickle cell crisis [9].

Cecilia Shoetan’s heart-rending BMJ report of her breathless 32-year-old sickle cell disease daughter dying within seconds when Diamorphine (licensed for this indication) was given intravenously in a London hospital while she stood watching [10] makes frightening nonsense of the NICE Guidance [11].

NHS ENGLAND and NHS SCOTLAND differ on Pregabalin?

The July 2007 Scottish Medicines Consortium (SMC) advice regarding Pregabalin differs from its April 2009 directive [12]. Meanwhile, NHS England had her own rules.  Witness the extraordinary situation in which NHS instructed doctors to depart from their usual prescribing for pain [13 14]. Who best should instruct doctors on prescribing practice? When Dr Margaret McCartney asked “Why do we have to prescribe branded Lyrica for pain?” [15] whom was she addressing? Well may Dr Laurence Leaver ask “With friends like NHS England, GP’s do not need enemies” [16].

Just 9 months ago we read “UK government to reclassify pregabalin and gabapentin after rise in deaths” [17]. Such reclassification is a forensic exercise. Disobey instructions and the Law could be after you. Can Freedom of Information identify individuals using “MHRA” or “NHS” or “The government” or “NICE” as cover to issue “not fit for purpose” guidelines? [18] Please investigate MHRA etc Competing Interest Declarations [19]. Pharmacovigilance in a developing country like Ghana has as many as 6 levels of vigilance and each level has potential for corruption [20]. Developed countries are no better, as Dr James Le Fanu exposes in his remarkable book recommended below.

The Le Fanu Legacy for Thoroughness

The name “Le Fanu” is music to Ghanaian ears. Korle Bu Hospital was planned and designed by Dr C V Le Fanu in the early 1910s in Colonial Gold Coast [21]. The outstanding Governor Sir Gordon Guggisberg [22] in 1919 “gave it top priority in his government’s building programme” [21] and when he opened it on 26 October 1923 he wanted it to “acquire a reputation second to none in the medical world”. One of the remarkable members of staff was another Le Fanu, Dr G E H Le Fanu who in 1909 led his laboratory in carrying out “a successful experiment to manufacture active vaccine lymph, locally, for small pox vaccination” [21]. Gold Coast Hospital, renamed Korle Bu Hospital, went on to do exploits including Dr Hideyo Noguchi’s Yellow Fever work. [21]. Meanwhile Dr G E H Le Fanu and colleagues including Dr Albert Hawe continued to tackle Tropical Diseases in a most thorough way [21 23]. Today, a Third “Thorough Le Fanu,” James, whose masterpiece “TOO MANY PILLS” is a ‘Must Read’ for every doctor mentions eight Big-Pharma companies that were fined a total of $Billion10.813 Dollars “for corrupt and illegal practices 2007-2012”. [24, page 25]

PAIN and OPIATES in HOSPITAL DEATHS

“Hospital drugs left 456 patients dead” was Daily Telegraph’s front-page head-line Thursday June 21 [25] plus an Editorial description of “fatal doses of diamorphine and other inappropriate drugs” in Gosport War Memorial Hospital. Note that when the NCEPOD Report of 2008 revealed that between January 2005 to December 2006 “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” [9] there were neither front-page newspaper-headlines nor calls for prosecutions as we hear today about Gosport.

Why the difference? ANSWER: Not because the victims were black, but because Morphine and Diamorphine are LICENCED “for sickle cell crisis indication” in the UK, raising this question:  Does the whole area of Drug Licensing (Pregabalin included) not need looking into urgently?  And when Dr James Le Fanu publishes in the BMJ that “Mass medicalisation is an iatrogenic calamity” [26] do we not wake up to the fact that the very first rung of the ladder to any medicalisation is DRUG LICENSING?

Finally: “Diamorphine has no accepted medical use in the United States” [27]

Competing Interest: I come from a sickle cell disease home: My Trait Parents had 11 children – 3 of us had sickle cell disease, hence my never-ceasing opposition to patient-management of pain that shortens life in anybody. [2 3 5 7 11 28 – 36].

felix@konotey-ahulu.com              Twitter@profkonoteyahul

Felix I D Konotey-Ahulu FGA MB BS MD(Lond) DSc(UCC) FRCP(Lond) FRCP(Glasg) DTMH(L’pool) FGCP FWACP FTWAS ORDER OF THE VOLTA (OFFICER) Kwegyir Aggrey Distinguished Professor of Human Genetics University of Cape Coast, Ghana; Former Consultant Physician Genetic Counsellor in Sickle Cell and Other Haemoglobinopathies Korle Bu Teaching Hospital & Director Ghana Institute of Clinical Genetics, and 9 Harley Street, Phoenix Hospital Group, London W1G 9AL.

www.sicklecell.md

1 Smyth Darren, Goldacre Ben, Croker Richard. Pregabalin: what the patent litigation means for doctors and big pharma. BMJ 2018;361:k2318 https://www.bmj.com/content/361/bmj.k2318.full?   BMJ June 09 2018

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 their countries have to be given morphine pumps during sickle-cell crises when they come to the UK?”]

3 Konotey-Ahulu FID. Opiates for sickle-cell crisis. Lancet 1998; 352: 651-652. [To David Bevan’s criticism (Lancet 1998; 351: p 1965) of white physicians who agree with Dr Konotey-Ahulu that opiates created addicts in hospital – “When I say routine opiates for sickle crisis are not the way to bring out these patients’ best potential in the long term I am glad to hear white physicians say the same…White physicians who, at the risk of being misunderstood by Bevan, voice their displeasure at what they see happening on their wards deserve commendation, not condemnation.”]

4 Chamberlain G. Medical problems in pregnancy: II. BMJ 1991; 302: 1327-30. (1 June)

5 Konotey-Ahulu, FID. Morphine for painful crises in sickle cell disease. BMJ 1991, 302(6792): 1604. (June 29 1991) (Comment on Professor Chamberlain’s recommendation of morphine in pregnancy in sickle cell disease – BMJ 1991; 302: 1327-30.) doi:10.1136/bmj.302.6792.1604-c

http://www.bmj.com/cgi/reprint/302/6792/1604-c.pdf

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

7 Liesner RJ, Vandenberghe EA, Davies Sally C. Analgeisics in sickle cell disease disease. Lancet 1993; 3411: 188.

8 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

9 NICE. Management of an acute painful sickle cell episode in hospital: summary of NICE guidance. BMJ 2012; 344 doi: https://doi.org/10.1136/bmj.e4063 (Published 27 June 2012) BMJ 2012;344:e4063

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

11 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 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) http://www.bmj.com/cgi/eletters/336/7654/1152a#196224 | http://www.info@ncepod.org

12 National Formulary. Pregabalin. BNF 70. September 2015 – March 2016: p 400.

13 Byrne Paul AC. Doctors are warned not to prescribe generic pregabalin for pain control www.bmj.com/content/350/bmj.h1734 March 30 2015 (BMJ April 7 2015)

14 Barbour James Re: Margaret McCartney – Second use of patents – why do we have to prescribe branded Lyrica for pain? (July 8 2015) “recent direction from a member of NHS health authorities that pregabalin prescriptions for chronic pain www.bmj.com/content/350/bmj,h1724/rapid-responses

15 McCartney Margaret. Second use of patents: Why do we have to prescribe branded Lyrica for pain? BMJ2015;350:h2734. July 8 2015.doi:10.1136/bmj.h2734 pmid:25995105

16 Leaver Laurence B. With friends like NHS England, GP’s do not need enemies “pregabalin to change some to Lyrica, so that Pfizer can maximise their profits at the expense of NHS” www.bmj.com/content/350/bmj.h1724 (02 May 2015)

17 Hopkins H. UK Government to reclassify pregabalin and gabapentin after rise in deaths. (03 October 2017) www.bmj.com/content/358/bmj.j4441 Rapid Response to Gareth Laccobucci. UK government to reclassify pregabalin and gabapentin after rise in deaths BMJ 2017; 358: 4441 https://doi.org/10.1136/bmj.j4441 (Published 25 September 2017) BMJ 2017;358:j4441

18 Rost Felicitas, Wessely Simon. Depression in adults: campaigners and doctors demand revision of NICE guidance. BMJ 2018;361:k2681 [BMJ 23 June 2018, p 426]  “The latest NICE draft guidelines on adult depression is misleading, invalid, not fit for purpose, and potentially harmful to patients”. BMJ 2018; 361: k2681.

19 Hurley Richard. Former MHRA chair takes job at cannabis investment company. Rapid Response Re: Cannabis, cannabis everywhere: UK to review medical cannabis policy as Canada plans imminent legislation for all uses. BMJ 20 June 2018 361:doi10.1136/bmj.k2695 “Sir Breckenridge will help Sativa Investments liaise with the Home Office and the Medicines and Healthcare products Regulatory Agency (“MHRA”), which he has chaired, for the legislation of medicinal cannabis in the UK”.

20 Konotey-Ahulu FID. Who should best pharmacovigilate in developing countries? https://www.bmj.com/rapid-response/2011/11/01/who-should-best-pharmacovigilate-developing-countries 14 September 2007 [ http://oW.ly/Dq8g30dSHe0 ]

21 Addae Stephen. Evolution Of Modern Medicine In A Developing Country: Ghana 1880 – 1960 Durham Academic Press, Ltd., 1 Hutton Close, Bishop Auckland, Durham 1996 [On Governor G Guggisberg and Le Fanu C V & Le Fanu G E H]

22 Goodall HB. Beloved Imperialist – Sir Gordon Guggisberg – Governor of The Gold Coast. The Penland Press Ltd., 1 Hutton Close, South Church, Bishop Auckland, Durham 1998.

23 Konotey-Ahulu FID. Some personal encounters with a remarkable physician (Tribute  to Dr Albert Joseph Hawe. OBE CBE MD FRCP DTMH). Ghana Med Journal 1979; 18: 88-90.

24 Le Fanu James. Too Many Pills – How too much medicine is endangering our health and what we can do about it. Little, Brown Book Group, London EC4Y 0DZ.

25 Daily Telegraph. Hospital drugs left 456 patients dead”. Thursday June 21 2018 Front Page. Editorial “Fatal doses of diamorphine and other inappropriate drugs”.

26 Le Fanu James. Mass medicalisation is an iatrogenic calamity. Profligate prescribing has brought a hidden a hidden epidemic of side effects and no benefit to most individuals. [PROVOCATIONS] BMJ 2018; 361: k2794. June 30, page 494.

27 [NOTE WELL]: Ballas S K. Sickle Cell Pain. IASP Press. Seattle, USA, page 168: “Diamorphine has no accepted medical use in the United States”

28 Konotey-Ahulu FID. Management of patients with sickle cell disease. African Journal of Health Sciences 1998; 5: 47[ Commenting on article of Sally Davies and Lola Oni (BMJ 315: 656 -60) “what I feel is more important in the day to day management of patients with a view to keeping them out of hospital, is clinical epidemiology which includes the circumstances of crises. … I fear Davies and Oni’s statement that ‘The Central Middlesex management protocol uses morphine infusions’ will make morphine the accepted drug for sickle crisis management. The consequences of such an approach are dire, especially when some UK hospitals are already making diamorphine their first choice”.]

29 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

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

31 Konotey-Ahulu FID. Management of sickle cell disease versus management of the sickle cell disease patient. BMJ Rapid Response 17 September 2008

http://www.bmj.com/cgi/elettrs/337/sep08_1/a1397#202088

32 Konotey-Ahulu FID. Inquest into diamorphine deaths: Does NCEPOD sickle patients report warrant a similar inquest? BMJ Rapid Response March 7 2009

http://www.bmj.com/cgi/eletters/338/mar03_3/b903#210208

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

34 Konotey-Ahulu FID. Opiods in the UK: What’s the problem? Answer – Good Clinical Practice needs to cover all population groups including sickle cell disease patients. BMJ Rapid Response 18 August 2013 www.bmj.com/content/347/bmj.15108/rr/658208

35 Konotey-Ahulu FID. Management of sickle cell disease patient in the community BMJ Rapid Response 13 April 2014 [90 References] to Brousse V, Makali J, Rees DC: Management of sickle cell disease in the community. BMJ 2014; 348: g1765 doi:10.1136/bmj.g1765 http://www.bmj.com/content/348/bmj.g1765/rr/694233

36 Konotey-Ahulu FID. Management of an acute painful sickle cell episode in hospital: NICE guidance is frightening1 Sept 7 2012 [42 references]] www.bmj.com/content/344/bmj.e4063/rr/599158

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Folic acid in Pregnancy: Birth Defects: Speech Embarrassment: Why abortion?

Tiny Folic Acid in pregnancy prevents huge African Linguistic Embarrassment too, but is abortion for prevention warranted?

In addition to the “birth defects such as spinal bifida and anencephaly” (BMJ 20 0ctober 2018) that “flour fortified with folic acid” [1 2] is expected to prevent when given in early pregnancy, I did state ten years ago that “a mere 500 micrograms daily is known to prevent cleft palate” [3] – a seemingly minor defect but which in the tribal setting constitutes a formidable social pathology [3 4].

SOCIAL PATHOLOGY OF CLEFT PALATE IN THE AFRICAN

How would any BMJ reader feel if when saying “I have scrubbed”, what is heard is “I have passed wind?” The extraordinary thing about this state of affairs is that the speaker is quite unaware that what was just heard was tantamount to blasphemy in Krobo/Dãngme-Gã Mother Tongue where every vowel not only has at least 3 reproducible pitches but also 2 distinct qualities – non-nasal and nasal [5]. Cleft palate phonates nasal and, coupled with any of the 3 distinct pitches, imparts 6 different meanings to the same consonant. I say that again: a vowel in my Tribal Tonal Language imparts 6 different meanings to the same consonant depending on its pitch and quality. [3-6]

“Osa” in Krobo/Dãngme (“Osha” in Gã) [Low High vowel-pitch sequence] means “you scrubbed”. Nasalise the second vowel, same pitch sequence, and you have said “You passed wind” [Osã and Oshã respectively]. Even using the tribal devulgarising prefix “Tafracher!” [7] can never excuse the utterance of such a thing in public. The embarrassment is complete. “It is, therefore” I once remarked “a social disaster of enormous proportions if when an African wants to say she has scrubbed, she is rather heard to say unmentionable things because of cleft palate. The need for early correction of this defect cannot be overemphasized. Any African baby with cleft palate stands in great danger of being ostracised in later life, and should be helped immediately” [4, page 10].

TERMINATION OF PREGNANCY FOR CLEFT PALATE?

Nicholas Wald of the Wolfson Institute of Preventive Medicine at Queen Mary University of London is quoted by the BMJ as saying if it is officially confirmed that within weeks flour will be fortified with folic acid “the UK is taking an important step in preventive medicine and helping to avoid disability or termination of pregnancy” [1]. In a Parliamentary Written Question 114833 “Abortion: Cleft Palate” Jim Shannon (Strangford) asked the Secretary of State for Health on 21 November 2017 “how many abortions there were in each of the last 15 years in which the fetus had a cleft palate (a) palate or (b) lip, …” by residents in England and Wales [8]. Answering, Jackie Doyle-Price said that for all gestations there were 9 in 2013, 10 in 2014, 11 in 2015, and 9 in 2016.

IF GHANAIANS COULD DIAGNOSE PRENATALLY WOULD THEY ALSO ABORT?

Certainly Not! At least for one interesting reason: Dr Albert Joseph Hawe MD FRCP OBE CBE was the most well-known white Colonial Doctor the Gold Coast (now Ghana) had ever known for 50 years [9]. He was the doctor stationed at Akuse 10 miles from where I was born in Odumase Krobo, and it was he who so expertly looked after me in the early 1930’s when I was often sickly that my first words in English were “When I grow up I shall go to England. When I grow up I shall be a Dorkita like Dorkita Hor”. His contemporary in our British colony was Dr Cicely Williams who first described Kwashiorkor in my tribe [10 11]. I was amazed when I discovered that the white “dorkita” who had looked after me as a toddler was one of the two Consultants (the other was Ghanaian Dr Silas Dodu) whom I was assigned to work with as Medical Officer on my return from the UK after registration.

My second discovery was that the heavily nasal diction of Dr Albert Hawe was due to Cleft Palate/Lip the scar of repair of which was a prominent feature of his physiognomy! If this remarkable Englishman had been aborted he would not have been in Colonial Gold Coast to work in my tribe and help save my life. But in order that it might not be construed that cleft palate abortion practice in England and Wales had no equivalence in Africa thus proving our superior ethical stance in these things I, born with extra-digits deformity (Mendelian Dominant 1% incidence in Ghana) [12], need to mention what I once said in the BMJ [13] which Professor Sir David Weatherall FRS drew attention to in his excellent textbook “The New Genetics and Clinical Practice”[14]: My Krobo Tribe in south east Ghana cared little about extra digits which they just tied firmly at birth and watched them atrophy and fall off. My stubs are there for all to see today. The tribe east of us beyond the Volta River would rejoice at the sight of a new-born baby with extra digits – destined to be rich. The tribe west of us beyond the hills would drown a baby so deformed until the practice was stopped by missionaries and the Colonial government. The UK “scientific tribesmen” as I described them in the BMJ [13] might have suggested I be aborted alongside pregnancies with cleft palate and sickle cell disease’

DISCUSS WITH AFRICANS WHAT UK LAW ALLOWS BUT ETHICS DISALLOWS

Cleft-palated Dr Albert Hawe for over half a Century treated Ghanaians who then came to England and were told that it would soon be legal to terminate an up-to-term pregnancy because cleft palate had been unmasked through ultrasonography. A procedure that was legal by UK Law would be interpreted in the African mind as perfectly alright. Indeed, it is taught that haemoglobinopathy may be diagnosed prenatally and termination suggested to parents when some of my patients were far brighter than their siblings. [15]. World Class Clinical Geneticists Professor George Fraser [16] and Professor Sir David Weatherall [14] have always emphasized ETHICS in these considerations.

Competing Interest: Born with extra digits – a defect for which termination has been done in the UK [17] Twitter: @profkonoteyahul

Felix I D Konotey-Ahulu FGA MB BS MD(Lond) DSc(UCC) FRCP(Lond) FRCP(Glasg) DTMH(L’pool) FGCP FWACP FTWAS ORDER OF THE VOLTA (OFFICER) Kwegyir Aggrey Distinguished Professor of Human Genetics University of Cape Coast, Ghana; Former Consultant Physician Genetic Counsellor in Sickle Cell and Other Haemoglobinopathies Korle Bu Teaching Hospital & Director Ghana Institute of Clinical Genetics, and 9 Harley Street, Phoenix Hospital Group, London W1G 9AL. [www.sicklecell.md]

 

1 Limb Matthew BMJ Flour to be fortified with folic acid within weeks, say reports. BMJ 2018; 363:k4348.(20 Oct.) BMJ 2018; 363 doi  https://doi.org/10.1136/bmj.k4348     (16 Oct 2018)

2 Campbell D. Folic acid to be added to UK flour in effort to reduce birth defects.Guardian 14

Oct 2018 https://www.theguardian.com/society/2018/oct/14/folic-acid-to-be-added-to-flour-in-effort-to-reduce-serious-birth-defects.

3 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).

http://www.ghanamedassociation.org/ | http://www.pubmedcentral.nih.gov.articlerender.fcgi?artid=2631266 PMID: 19180210 [PubMed – indexed for MEDLINE] (vowel coloration omitted on this NIH site).

4 Konotey-Ahulu FID. Tonic SolFa Is The Foundation Of Tonal Linguistics. DRUMSPEAK (Supplement) International Journal of Research in the Humanities. New Series Vol 3. No. 1, May 2010. Faculty of Arts, Univ. of Cape Coast, Ghana. ISSN (0855-9945).http://bit.ly/bEdcc4

5 Konotey-Ahulu FID. Black people’s red faces and AIDS prevention. Lancet 2000; 355(9214):1559. PMID: 10801206 [African vowel has at least 6 meanings with consonant]

6 Konotey-Ahulu FID. The Remarkable African Ear: Phenomenon of Mid Pitch Arrest Phenomenon in Krobo-Dangme-Gã Tonal Linguistics of South East Ghana. African American Museum of Philadelphia Award Lecture May 5 2007

http://blog.konotey-ahulu.com/blog_archives/2007/5/5/301434.html

7 Konotey-Ahulu FID. Tafracher – Personal View. BMJ 1975; 1(5953): 329. (February 8)  http://www.bmj.com/cgi/reprint/1/5953/329.pdf         http://bit.ly/2bRQ7B1

8 UK Parliament. Abortion: Cleft Palate: Written question – 114833    (21 Nov. 2017)

https://www.parliament.uk/business/publications/…questions…question/…11…/114833/

9 Konotey-Ahulu FID. Some personal encounters with a remarkable physician. (Tribute to Dr Albert Joseph Hawe, OBE, CBE, MD, FRCP, DTMH). Ghana Med J 1979; 18: 88-90.

10 Williams Cicely. D. Kwashiorkor – a nutritional disease of children associated with a maize diet. Lancet 1935; ii: 1151-1152.

11 Konotey-Ahulu FID. Issues in Kwashiorkor. Lancet 1994, February 26, page 548.

12 Bonney GE, Walker M, Gbedemah K and Konotey-Ahulu FID. Multiple births and visible birth defects in 13000 consecutive deliveries in one Ghanaian hospital. In Proceedings of the Second International Congress on Twin Studies Part C Ed Nance W. Progress in Clinical and Biological Research 1978; 24 Pt B: 105-108.

13 Konotey-Ahulu FID. Ethical issues in prenatal diagnosis. BMJ Clin Res Ed 1984; 289(6438): 185. July 21. doi:10.1136/bmj.289.6438.185-a 6143955

http://www.bmj.com/cgi/reprint/289/6438/185-a.pdf

14 Weatherall DJ. Ethical Issues and related problems arising from the application of the new genetics to clinical practice. In: The New Genetics and Clinical Practice. [Third Edition] Oxford University Press. Oxford 1991.

15 Konotey-Ahulu FID. Antenatal sickle cell disease haemoglobinopathy screening. BMJ

http://www.bmj.com/content/341/bmj.c5243/reply#bmj_el_243447  Rapid Resp. Oct 25 2010

16 Fraser GR. FIFTY YEARS OF HUMAN GENETICS – A Festschrift and liber amicorum to celebrate the life and work of George Robert Fraser. Wakefield Press, 1 Parade West, Kent Town, South Australia 5067 www.wakefieldpress.com.au

17 Yeoman Fran. Babies are aborted over an extra finger or club foot. The Times. May 29 2006.

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