World Sickle Cell Disease Patient Week – 20% off our books

World Sickle Cell Patient week

Today is the start of World Sickle Cell Disease Patient Week and we are offering 20% off our books till 31st August 2017.

To view the event on Facebook look out for my videos during the week starting today. 

World Sickle Cell Disease Patient Week

Use the following code at the checkout WSCDPW2017 to get you 20% discount  plus an added bonus a special free gift of a pack of kanad (Konotey-Ahulu Norm Ache Dice) when you purchase a book from our store during the World Sickle Cell Disease Patient Week.

My books are available here

kanad (Konotey-Ahulu Norm Ache Dice)
Free gift when purchasing our books till 22nd July 2017

World Sickle Cell Disease Patient Week

World Sickle Cell Disease Patient Week

WSCDPW [World Sickle Cell Disease Patient Week]

There is such an event called “World Sickle Cell Day” which falls in mid-June every year.

For me who had two brothers and one sister (Victor Agbetey, Jerry Tei and Sussie Konotey-Ahulu) with hereditary cold-season rheumatism or hemikom as this has always been known in my Krobo Tribe in Ghana as the name for Sickle Cell Disease – one day in a year is not enough attention given to a very important problem.

Therefore, I am from July 12 2017, God willing, devoting a whole week to what I am calling WSCDPW ie World Sickle Cell Disease Person or Patient Week – the P is for Person or Patient for, as I hope to show you, some-one with sickle cell disease does not have to be going in and out of hospital regularly and frequently.

So there will be something for 12, 13 14, 16 17, 18 of July, with 15th July as a rest day. During the week matters concerning the Person with sickle cell disease will be discussed. My greatest credential is that from the day I was born several decades ago I was within my immediate family and the extended family surrounded by sickle cell disease relatives – this credential of mine is more important than the fact that as a doctor, I ran the largest Sickle Cell Disease Clinic in the world at the Korle Bu Teaching Hospital. And indeed more important than the fact that with Professor Linus Pauling (discoverer of the molecular pathology of sickle cell haemoglobin for which he got the Nobel Prize) on the platform I was chosen from among 24 Dr Martin Luther Jing Jr Foundation Award Winners for Sickle Cell Research world-wide, to deliver the Award Dinner Lecture in Philadelphia on Wednesday 31st May 1972, the title of my Award Lecture being “The Vital Difference Between Sickle Cell Trait and Sickle Cell Disease”. This does not compare with the fact that I knew about the sickle cell disease patient before I read Medicine.

So, the fact that I was born into a home where my sibllings, and cousins, and aunts, and uncles suffered from sickle cell disease is why I dare to introduce a WSCDPW or World Sickle Cell Disease Patient Week. My aim is not to indulge in controversy. My sole aim, and I mean this, my sole aim is to tell those like my brothers and one sister who inherited an abnormal haemoglobin from both father and mother to give them sickle cell disease – to tell them that they have inherited other genes from the same parents that can produce great achievement in their lives. I shall be greatly privileged to introduce some of these ACHIEVERS to the world, and to help those struggling at the moment with pain and other problems how to succeed. My 643-page book describes no less than 130 real patients and their problems and how they have succeeded or not succeeded in tackling them. Watch this space! My website also has much information.

Professor Felix I D Konotey-Ahulu [whose parents were Traits for Abnormal Haemoglobin genes and whose 3 siblings had sickle cell disease].
Kwegyir Aggrey Distinguished Professor of Human Genetics, University of Cape Coast Ghana, and Former Consultant Physician Genetic Counsellor Korle Bu Teaching Hospital Ghana and 9 Harley Street, London W1G 9AL.

Should we abandon routine blood tests?

Re: Should we abandon routine blood tests? No, not when hereditary erythrocytopathy poses a real problem in a so-called multi-racial population!

Should we abandon routine blood tests_fb

Should we abandon routine blood tests? No, not when hereditary erythrocytopathy poses a real problem in a so-called multi-racial population!


The name “K Siau” [1] sounds Ghanaian from Kwahu Tribe, while the initial K could stand for any of the 7 Ghanaian Male Day-Names: Kwesi – Sunday to > Kwame – Saturday. [Our Kofi Annan was Friday-born]. If Siau is Ghanaian, then he may well be one of the “1 in 3 West Africans” who have inherited the abnormal haemoglobin gene ‘S’ or ‘C’, from one parent, not to mention the 1 in 4 males with Glucose 6 Phosphate Dehydrogenase (G6PD) Deficiency [2 3]. Millions of healthy West Africans in the UK have one gene for hereditary erythrocytopathy (abnormal haemoglobin S or C, plus or minus G6PD Deficiency) [4 5]. Routine blood tests performed just once can discern these [5]. UK is no longer entirely Caucasian therefore doctors need to unmask erythrocytopathy.


Orthopaedic Trainee Dr Alastair Faulkner [6] may have read my account [7] of a postgraduate ward round many years ago. After being asked to examine a white patient I gave my Clinical Impression: “This jaundiced Greek patient with pale nails, scars on one shoulder and right hypochondrium, and with limited straight leg elevation of right leg has avascular necrosis of the shoulder, avascular necrosis of one femoral head, and has had gall stones removed because of chronic haemolysis from Sickle Cell beta-Thalassaemia” [7] Duly impressed, Professor Milne produced laboratory results confirming my diagnosis proving that NOT ONLY BLACK PEOPLE SUFFER FROM SICKLE CELL DISEASE. In Greece, around Lake Kopais, the 30% incidence of sickle cell trait was higher than anywhere in Ghana [8-10]. Any new patient I ever had – Black, White, Hebrew, Asian or Indian must routinely have Haemoglobin Electrophoresis and G6PD test done and be given a Certificate of the findings.


Dr Sam Lewis [11] mentions the “nit-picking rejection of lab requests” in the NHS. When Clinicians request certain tests, NHS Pathologists often decide which to accept and reject. While Locum Consultant Physician in a London Hospital I requested Haemoglobin Electrophoresis on a woman, only for the Pathologist to send a report: “Sickling Test Negative”. I protested I never asked for a Sickling Test; could I have Haemoglobin Electrophoresis, please? The Haematologist asked why if sickling test was Negative I wanted Haemoglobin Electrophoresis? I then told him I had in Ghana the largest collection of Homozygous Haemoglobin C Disease patients (CC) in the world (all sickling negative) and that it was a common cause of miscarriage in pregnant women [12] The Pathologist still refused to do the test so I sent the lady to a Private Lab where Haemoglobin CC Phenotype was confirmed. As husband was Sickle Cell Trait (AS) I counselled them, advising that they should expect to have Sickle Cell Disease (SC) and Haemoglobin C Trait (AC) offspring. [13]


Do not wait till patients complain of “passing urine like coca cola”. Always testing new patients for Abnormal Haemoglobins and G6PD Deficiency was how we discovered that G6PD Deficiency was not just a haemolysis problem, but could aggravate the course of typhoid fever, renal failure, muscle pain, duration of coma from any cause, and the prognosis of sickle cell disease. [2 3 14 -20]. That was how we discovered the first ever African with zero G6PD enzyme in his red cells – a phenomenon thought only to exist in Mediterranean Caucasians. [21 22]. Ringelhann [23], Beutler [24], James Bowman and Robert Murray [25] confirmed high levels of G6PD Deficiency in Blacks on both sides of The Atlantic. Luzzatto, examining 100 “SS” males found 16% to be G6PD Deficient [26]. As salmonellosis is common in sickle cell disease [27 28], and Chloramphenicol is contraindicated in G6PD Deficiency, this test requires doing for all sickle cell disease patients [19].


Relying only on G6PD Colour Tests would have missed our zero-enzyme patients. Quantification is vital for both Abnormal Haemoglobin and G6PD enzyme. That was how we knew that Sickle Cell Traits have 3 modes of Haemoglobin S (20-25%, 30-35%, 36-39.5%) [29] which fact enables us to distinguish the true Sickle Cell Trait (“AS” with S less than 40%) from Sickle Cell beta-plus Thalassaemia Disease (2 abnormal genes) with Sickle gene product way above 50% [28 29 30].
WHO and International Atomic Energy Agency grants funded our tests. [5 28].


I have described a white Englishman (Hb D Trait) married to a Ghanaian lady (Hb S Trait) and their 2 sickle cell disease daughters (Ref 28, Illustrative Case History 133). If NHS budget is unable to sustain such a routine test burden shall we not ask enquirers to go Private for their health’s sake? Dr K Siau believes while we needed to “avoid unnecessary repetition of blood tests” [1] some routine tests “should be considered part of holistic Medicine …” [1]. Three out of every 100 West Africans in the UK have inherited TWO ABNORMAL HAEMOGLOBIN genes causing hereditary disease capable of showing up in any Clinical Discipline [See the 133 Illustrated Case Histories in Reference 28] not to mention the non-haematological complications of G6PD Deficiency [2 3 20 28 29]. I once expressed disappointment when a seven-part series on genetic epidemiology by “three white professors began in The Lancet on September 10, 2005” [30] only for the series to end without once mentioning the genetic burden of west Africans in the UK. [29]. Will Dr K Siau now add our West African statistics to those of the “4000 Danish patients” he produced [1]? As I said in 1982, we needed to “unearth the hundreds of cases of SC sickle cell disease, some of whom are bread winners, before they are brought in dying or dead in their first crisis for 10 years” [31]

Conflict of Interest: My Parents Heterozygote for Abnormal Hbns (“AC” & “AS”) had 11 children: 3 Sickle Cell Disease “SC”, 2 Heterozygotes “AC”, 2 Heterozygotes “AS”, 4 Homozygotes “AA” [32]. Twitter@profkonoteyahul

F I D Konotey-Ahulu MD(Lond) FRCP(Lond) FRCP(G) DTMH(L’pool) FGCP FWACP FTWAS
Kwegyir Aggrey Distinguished Professor of Human Genetics, University of Cape Coast, Ghana. Former Consultant Physician/Genetic Counsellor in Sickle & Other Haemoglobinopathies, Korle Bu Teaching Hospital, Accra, Ghana and at 9 Harley Street, London WIG 1AA. [ ]


1 Siau K. Should we abandon routine blood tests? BMJ Rapid Response 8 May 2017.

2 Owusu SK. Glucose 6 Phosphate Dehydrogenase Deficiency in the causation of disease in Ghana. Ghana Med J 1974; 13: 168-170.

3 Konotey-Ahulu FID. Glucose 6 Phosphate Dehydrogenase Deficiency and Disease Causation in Ghana. (Editorial) Ghana Medical Journal 1974; 13: 155-158.

4 Ringelhann B, Dodu SRA, Konotey-Ahulu FID. Lehmann H. A survey for haemoglobin variants, thalassaemia, and Glucose 6 phosphate dehydrogenase deficiency in northern Ghana. Ghana Med J 1968; 17: 120-124.

5 Ringelhann B, Konotey-Ahulu FID. Hemoglobinopathies and thalassaemias 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

6. Faulkner Alastair, Reidy Mike, McGowan James. Should we abandon routine blood tests? BMJ 2017; 357: j2091

7 Konotey-Ahulu FID. Hip pain and radiographic signs of osteoarthritis: Sickle cell and other haemoglobinopathy differential diagnosis. BMJ Rapid Response 8 January 2016

8 Choremis G, Sickle cell anaemia in Greece. Lancet 1951; 1: 1147.

9 Choremis G. Sickle cell trait and blood groups in Greece. Lancet 1953; 2: 901-911.

10 Delyannis GA, Tavlarakis N. Sickling phenomenon in Northern Greece. BMJ 1955; 2: 299-301.

11 Lewis Sam. Should we abandon routine blood tests? BMJ Rapid Response May 4 2017

12 Konotey-Ahulu FID. Homozygous Haemoglobin C Disease. In FID Konotey-Ahulu. The spectrum of phenotypic expression of clinical haemoglobinopathy in West Africa. New Istanbul Contribution to Clinical Science 1978 Dec; 12(3-4): 246-257.

13 Konotey-Ahulu FID. SICKLE CELL DISEASE: The Case for Family Planning. Astab Books Ltd., Accra Ghana 1973.

14 Owusu SK. Clinical manifestations of glucose 6 phosphate dehydrogenase (G6PD) deficiency in Ghana. Ghana Medical J 1978; 17: 235-239.

15 Owusu SK, Foli AK, Konotey-Ahulu FID, Janosi M. Frequency of Glucose 6 Phosphate Deficiency in typhoid fever in Ghana. Lancet 1972; 1: 320.

16 Adu D, Anim-Addo Y, Foli AK, Yeboah ED, Quartey JKM. Acute renal failure and typhoid fever. Ghana Medical Journal 1975; 14: 172-174.

17 Owusu SK, Addy JH, Foli AK, Janosi Marianne, Konotey-Ahulu FID, Larbi EB. Acute reversible renal failure associated with glucose 6 phosphate dehydrogenase deficiency. Lancet 1972; 1: 1255-1257.

18 Konotey-Ahulu FID. Glucose 6 phosphate dehydrogenase deficiency and sickle cell anaemia. New Eng J Med 1972; 287: 887-888.

19 Acquaye CTA, Gbedemah KA, Konotey-Ahulu FID. Glucose 6 phosphate dehydrogenase deficiency incidence in sickle cell disease in Accra. Ghana Med J 1977; 16: 4-7.

20 Konotey-Ahulu FID. G6PD Deficiency in Ghanaians: How to recognize it. 16 2008.

21 Owusu SK. Absence of glucose 6 phosphate dehydrogenase (G6PD) in red cells of an African. BMJ 1972; 4: 25-26

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

23 Ringelhann Bela. A simple laboratory procedure for the recognition of the A (African Type) G6PD Deficiency in acute haemolytic crisis. Clin. Chim. Acta 1972; 36: 272-274.

24 Beutler E. Genetic Disorders of Red Cell Metabolism. Medical Clinics of North America 1969; 53: 813-826.

25 Bowman James E, Murray Robert F. Genetic Variation and Disorders in Peoples of African Origin. The Johns Hopkins University Press Ltd, London 1990, pp 171-190.

26 Luzzatto Lucius. G6PD Deficiency frequency and sickle cell association on the African continent. INSERM 1975; 44: 229.

27 Konotey-Ahulu FID, Kuma Eunice. Skeletal crumbling in sickle cell anaemia complicated by Salmonella Typhi infection. British J Clin Practice1965; 575-578.

28 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. T-A’D Company Watford 1996 [First Published 1991 & 1992 The Macmillan Press Ltd., London and Basingstoke] – 643 pages. [This book has 133 Illustrated Case Histories not found elsewhere]

29 Konotey-Ahulu FID. Alpha-Thalassaemia nomenclature and abnormal haemoglobins. Lancet 1984; 1: 1024-1025.

30 Weatherall DJ, Clegg JB. The Thalassaemia Syndromes 1981. Third Edition, Blackwell Scientific. Oxford.

31 Konotey-Ahulu FID. Survey of sickle cell disease in England and Wales.…
BMJ 1982; 284: 111 (January 9)

32 Konotey-Ahulu FID. Sickle Cell Disease in Successive Ghanaian Generations for Three Centuries (Manya Krobo Tribe) Chapter 2 Reference 28 pp 6-20, and FID Konotey-Ahulu 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]

Competing interests: My Parents Heterozygote for Abnormal Hbns (“AC” & “AS”) had 11 children: 3 Sickle Cell Disease “SC”, 2 Heterozygotes “AC”, 2 Heterozygotes “AS”, 4 Homozygotes “AA” [32].


Rev Dr David Silas Laweh Konotey-Ahulu


February 19 2017 was the last Sunday on earth for my younger brother Laweh – Braa Laweh as I called him. I had arrived the previous Friday night from London and, determined not to go to bed without visiting him at home I let the hotel reception ring Joanna, Laweh’s wife and say “A big parcel has arrived from London for you; call to collect it.” Promptly came the reply – it was late, and not easy to get a taxi in the middle of Teshie. Could she not come on Saturday morning instead? “No, it must be tonight”.

Brochure Cover Picture

Click here to read more – Tribute By PROFESSOR F I D KONOTEY-AHULU

UK Practice versus Ghana Practice: kascdispatma

Re: International medical graduates and quality of care: What if quality of care in developing country is better than UK’s?

Re: International medical graduates and quality of care Aneez Esmail, Julian Simpson. 356:doi10.1136/bmj.j574 International Medical Graduates and quality of care: What if quality of care in developing country is better than UK’s?

The excellent Editorial [February 6 2017] of Professor Aneez Esmail and Julian Simpson, hemmed in as it is to comparing and contrasting UK health care [1] with that of the USA vis-à-vis foreign trained doctors, was not expected to address the question: What if certain aspects of the quality of care in a developing country, say Ghana, is better than that in the UK? What is a doctor trained in Ghana by UK-trained doctors to do when he/she sees something taught at home is contradicted by UK practice with disastrous results?

I dare to ask 21st Century Clinicians to compare themselves with those who taught me, and state honestly whether apart from the CT Scans, Ultrasound machines, MRI’s, and microsurgical techniques, they boast better clinical acumen than these grand-teachers?. I was trained at London University’s Westminster Hospital School of Medicine by the world’s greatest – Sir Clement Price-Thomas, Surgeon of King George VI, Sir Richard Bayliss Physician of Queen Elizabeth II, Sir Stanford Cade, World Number One in cancer surgery assisted by Charlie Westbury MS MRCP FRCS, then Professor J Pulvertaft (Pathology), Sir Arthur Bell Obs/Gynae, etc. and at the Post-graduate level Malariologist Professor Brian Maegraith, Liverpool University School of Tropical Medicine, Sir John Dacie, Haematologist at Hammersmith Hospital where Professors John Goodwin (Cardiology) and J McMichael assisted by Dr Celia Oakley taught me Cardiology, and where I first met Dr Geraint James who introduced me to his wife Professor (later Dame) Sheila Sherlock [2 3] of Royal Free Hospital, where I worked with the Artificial Kidney King of Europe Dr Stanley Shaldon. All these were Clinicians with the “History-Inspection-Palpation-Percussion-Auscultation” (HIPPA) approach to patient care which was what I taught my undergraduate and post-graduate students in Ghana. Then at Cambridge University I was very fortunate to be instructed in Haemoglobinopathy and G6PD Enzymopathy by Professor Hermann Lehmann FRS the Doyen of Abnormal Haemoglobins.

Back home in Ghana my Mentors were Colonial Physician Dr Albert Hawe MD FRCP [4] and Ghanaian Dr Silas Dodu MD FRCP DTMH who had collected most of the prizes at the University of Sheffield.

When the National Foundation and the March of Dimes paid for a 6-week tour of 11 leading Medical Institutions in the USA 45 years ago, and I did Grand Rounds at Cornell, George Washington University Hospital, Columbia University, Johns Hopkins, Yale, Howard, Tennessee, Indianapolis etc, and I often made Haemoglobin phenotype diagnoses without X-rays and Lab results but by just examining the patient using HIPPA
the Professors were so astounded they asked how I did it, and I just said “It’s the British Undergraduate Exercise that prepared me for the bush”.

The situation in the UK has drastically changed. Ghanaian doctors we taught would end up in the UK and be introduced to practice that looked at the blood of the patient before looking at the patient – quite opposite to what we taught them, preferring to look at Protocols hung up on a wall without realising that what is good for one patient may be poison for another. Or that what is recommended by NICE Guidelines could be killing patients [5-7]. How many British doctors, I wonder, are aware that I said 42 years ago in The Lancet that blood transfusion precipitated sickle cell crises? [8] Or that Graham Serjeant described a sickle cell anaemia lady he had never transfused but who going to the USA on holiday from Jamaica, checked into a Clinic for routine tests, was found to be anaemic and promptly transfused, only to die within 48 hours? [9] Or that I have described an “SS” man who would go into sickle cell crisis whenever Haemoglobin level rose above 9 grams per deci-Litre? [10]? I once referred one such patient to Professor Lucio Luzzatto at London’s Post-Graduate Medical School Hammersmith Hospital to venesect her every 6 months to bring Hb below 9 grams lest she went into sickle crisis. Sadly, with dogged determination British doctors would follow a Protocol that said Hb should not fall below 11.0 grams per deci Litre [11]. How many British Doctors would persist with Morphine and Diamorphine even when NCEPOD Report damned the Opiate Culture? [12-19]. Yet NICE continues to recommend intravenous Opiates? [5-7 12-19].

Even when a Nigerian Consultant Haematologist in a UK Teaching Hospital, himself “SS,” wrote a BMJ Editorial on Hydroxyurea saying “patients had been voting with their feet due to side effects” [20 21] clinicians continued to prescribe it.

My immediate elder brother, my immediate younger brother, and the 5th of my parents’ 11 children had sickle cell disease [22]. I know the disease first hand. Having grown tired of criticising other clinicians about their mismanagement of patients with sickle cell disease I am offering doctors, nurses, patients and their relatives a real alternative to what the rest of the world (apart from Graham Serjeant [23] in the West Indies) prescribes [24]. My approach brings hospitalisation to a minimum, preferring patient management to disease treatment [25-29]. My kascdispatma (spelt fully above) produces PATIENT ACHIEVERS who use their non-abnormal haemoglobin genes inherited from the same parents to achieve things that their siblings without the disease have not achieved [30 31]. G6PD status of patients, meaningful genetic counselling with voluntary family size limitation [32], avoidance of harmful received wisdom underline the Common Sense Public Health Approach that is the defining feature of kascdispatma When Professor Helen Ranney of Albert Einstein University College of Medicine New York says “There is no single clinical experience in the United States comparable to that of Dr Konotey-Ahulu “[33] should not serious clinicians and parents struggling to look after sickle cell disease patients try my kascdispatma?.

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

Conflict of Interest: None Declared

1 Aneez Esmail, Simpson Julian. International medical graduates and quality of care. BMJ 2017; 356: j574 (6 February 2017) doi:

2 Konotey-Ahulu FID. Konotey-Ahulu FID. Dame Sheila Sherlock’s Third World Dimension. BMJ rapid response 20 Jan 2002

3 Konotey-Ahulu FID. David Geraint James: Great Champion of Overseas Postgraduates. 18 Nov 2010 [Response to BMJ 341:doi.1136/bmj.c6400] See Ref 170 on Dame Sheila Sherlock 20 Jan 2002. |

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

5 Gillis VL, Senthinathan , Dzingina M, Chamberlain K, Banks K, Baker MR, Longson D [Guideline Development Group]. Management of an acute painful sickle cell episode in hospital. Summary of NICE Guidance. BMJ 2012; 344: e4063 [doi:101136/bmj.e4063]

6 Konotey-Ahulu FID. UK drug related deaths are still rising: So where is NICE? Sept. 6 2009 BMJ Rapid Response to Susan Mayor on “UK drug related deaths are still rising 2 reports say” BMJ Sep 6.

7 Konotey-Ahulu FID. Management of an acute painful sickle cell episode in hospital: NICE guidance is frightening1 Sept 7 2012 [42 references]

8 Konotey-Ahulu FID. Sicklaemic human hygrometers. Lancet 1965 May 8; 1(7393): 1003-1004.

9 Serjeant GR. Blood transfusion in sickle cell disease: A cautionary tale. Lancet 2003; 361: 1659

10 Konotey-Ahulu, FID. The Sickle Cell Disease Patient. Macmillan Education Ltd London 1991/1992. Foreword by Roland B Scott, MD (Howard University) – 36 chapters with 4,500 references, 643 pages. ISBN: 0-333-39239-6 & Konotey-Ahulu FID. The Sickle Cell Disease Patient, Watford: Tetteh-A’Domeno Co, 1996 Reprint of 1991 Macmillan book] [Hb > 9.0 grams >crises, p.482 Case History 21]

11 Claster Susan, Vichinsky Elliott P. Managing sickle cell disease. BMJ 1997; 327: 1151-1155. Doi:10.1136/bmj.327.7424.1151.

12…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)

13 …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) |

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

15 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?”]

16 Konotey-Ahulu FID. Opiates for sickle-cell crisis. Lancet 1998; 352: 651-652. [In response to David Bevan’s criticism (in Lancet 1998; 351: page 1965) of white physicians who agree with Dr Konotey-Ahulu that opiates have created addicts in their 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.”]

17 Konotey-Ahulu FID. Current “hit and miss” care provision for sickle cell disease patients in the UK. BMJ Rapid Response 22 July 2008

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

19 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

20 Olujohungbe A, Cinkotai KI, Yardumian A. Hydroxyurea therapy for sickle cell disease in Britain. BMJ 1998; 316: 1689 (6 June)

21 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. Two examples suffice to ilustrate what I mean: … 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”.]

22 Konotey-Ahulu FID. The Human Genome Diversity Project: Cogitations of An African Native. Politics and the Life Sciences (PLS) 1999, Vol 18: No 2, pp 317-322. [Invited Commentary on Professor David Resnik’s article: The Human Genome Diversity Project: Ethical Problems and Solutions. [Chart “Sickle Cell Disease in Successive Ghanaian Generations for Three Centuries”]

23 Serjeant GR. Sickle Cell Disease. Oxford. Oxford Univ. Press (2nd Edition) (632 pages)

24 Davies SC, Oni L. Management of patients with sickle cell disease. BMJ 1997; 315: 656-660.

25 Konotey-Ahulu FID. The inheritance of Sickle Cell Disease. New African January 2000, pp 40-43

26 Konotey-Ahulu FID. The Person with Sickle Cell Disease. New African March 2001, pp 38-39.

27 Konotey-Ahulu FID. Teenager with Sickle Cell Disease. New African. June 2001, 41-42

28 Konotey-Ahulu FID. Adult with Sickle Cell Disease. New African Sep. 2001, pp 40-43. |

29 Konotey-Ahulu FID. Sickle-cell disease and the patient. Lancet 2005; 365(9457): 382-83 January 29-February 4. [Commenting on Marie Stuart & Ronald Nagel’s “Sickle-cell disease” Seminar in Lancet 364: 1343-60, and Michaela Buckner’s “Sickle-cell disease: from Sierra Leone to southeast London” Lancet 364: 1361, it is pointed out that “Stuart and Nagel missed something out of their Seminar, which Michaela emphasizes – circumstances.”

30 Konotey-Ahulu FID. Sickle cell achievers’ conference. SICKLE CELL News Review 1995; Summer Edition: 5.

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

32 Konotey-Ahulu FID. Sickle Cell and Allied Haemoglobinopathy: The Genetics That Touches You and Me – University of Cape Coast, Ghana, GOLDEN JUBILEE MESSAGE. (Sept 19 2014)

33 Ranney Helen. “There is no single clinical experience in the United States comparable to that of Dr Konotey-Ahulu”. In Summary of Symposium on Sickle Cell Disease – Diagnosis, Management, Education, and Research – In Sickle Cell Disease, Editors Abramson, JF Bertles, Doris Wethers (C. Mosby & Co) 1972, page 320.

Competing interests: No competing interests

Evidence in a post-truth world: Scientists’ Misinformation & Disinformation

Dr Margaret McCartney highlights something most doctors ignore [1] Her “seams of multiple misinformation” become disinformation when deliberate, as when “Journal agrees to retract paper after university found study was never done” [2].


“There is no real evidence that e-cigarettes do any harm”, is interpreted as “e-fags are harmless”. No evidence is increasingly used as evidence [3]. Prefix “scientific” to “truth”, and Einstein expects integrity. [4] When three doctors confessed that tests they published had been done were in fact not done I concluded letter to BMJ Editor Dr Martin Ware:

“Your readers, Sir, are interested in scientific truth. I am one of them, and the very least I am entitled to expect is that if evidence obtained from my own country cannot be scientific it should at least be true. – I am etc., F I D KONOTEY-AHULU” [5]


Lord Solly Zuckerman FRS said in his excellent article “Pride and Prejudice in Science” that disagreements about truth were often coloured by prejudice, and sometimes fraud. [6] I’ve discussed elsewhere experts’ disagreements. [7]. That Ethics is deprecated and Science re-defined by some scientists is sad. Two examples: (a) Design in nature is now not science [8]. (b) Programmes to genome-sequence whole populations anonymously [9 10] against which I have protested, indicating how dangerous this is in an “Anti-truth world” [11]. Had James Watson been genome-sequenced anonymously would the truth have come out?


James Watson announced that the current global genome-sequencing exercise would show that Africans were less intelligent than other “races” [12], using the term “race” just as Charles Darwin had done: “The Origin of Species By Natural Selection – Or The Preservation Of Favoured Races In The Struggle For Life” [13]. Watson’s three to four trillion DNA bits in his genome were sequenced only to discover that his great-grandparent was African [14]. Convinced he belonged to the “favoured white race” he in fact had a great-grand parent from the African “un-favoured race”. I commented: “Watson, still alive today in the USA, was thus brutally reminded not only that there is but one human race, but also that African genes contributed to the Nobel Prize in ‘Physiology or Medicine’ which he jointly won with Francis Crick and Maurice Wilkins in 1962 on the DNA Double Helix” [15]. James Watson’s opinion was taken as scientific truth because Nobel Laureates are not supposed to be economical with the truth. The Human Genome Project became suspect [11 16 17], and this exposes the
real problem with research in Human Sciences including IQ, Linguistics and even History. Researchers wax eloquent about “Evolutionary Ethics”, making ex cathedra statements like “The capacity to develop ethics is a product of biological evolution” [18]. Such Evolutionary Fundamentalism is considered “scientific truth” while “GOD commanded, and universe materialized” [19] is ridiculed?


Three events in a Lecture Series on Evolution by J Z Young FRS at University College London [20] convinced me that Darwin’s Evolution by Natural Selection never took place. It was more than 3 decades later that another Fellow of the Royal Society Professor Fred Hoyle would confirm my judgement: “How has the Darwinian theory of evolution by natural selection managed, for upwards of a century, to fasten itself like a superstition on so-called enlightened opinion? Why is the story still defended so vigorously?” [21] First event (a): J Z Young was comparing the brain of a new-born human baby with that of an adult chimpanzee [20]. His words “I do not know …” stunned me and Shirley Knight sitting together. (b) One student’s question “Why …?” brought the answer: “’Why?’ questions are not permitted here” (c) J Z Young kept saying “Theory of Evolution” over and over again. This world-authority on the human brain [22] who taught me not only “what to think” to pass exams, but also “how to think” to enable me criticise my teachers, led me to the conclusion I found Professor Fred Hoyle arriving at 3 decades later, namely that Darwin’s Evolution Emperor has no clothes! But then comes Paul Ehrlich [18] sprinkling his 531-page Evolution book with “Why?” questions that he blithely proceeds to answer with ex cathedra pronouncements!


This Welsh Genius [24] proved some “Truths” to be supra-scientific. [23]. I once wrote: “There is a vast area of man’s experience called the spiritual realm which neither Freudian psychoanalysis nor the scientific method can fathom” [25] Dr Lloyd-Jones’ books “Spiritual Depression” [26] and “Conversions Psychological and Spiritual” [27] support this. Because “approaches to scientific truth and spiritual truth are different” [25] one scientist will read Spiritual Depression “with much profit while another, with the same qualifications, will find it unintelligible” [25]. Dr Lloyd-Jones totally dismisses Darwin’s “races”. He recognises only intra-racial marriages: Google “Felix and Rosemary” [28].


Misinformation that Margaret McCartney mentions [1] can be counterbalanced using the same Internet when editors refuse to publish corrections [29]. Are Margaret’s two prescriptive suggestions [1] capable of identifying scientific liars [30-32] and lying journalists who suppress truth about Statins or whatever? [29]


“Did that happen?” is my best question to unravel truth. Was I born on a Saturday? Was I struck by lightning on a Friday 2 days before Christmas? Science can never be used to substantiate the truth behind these events [3 33-36], so why do we defer to Science to verify infinitely more important events defining Christmas? History beats Science [37]. Dismiss me as a creationist sermonizing, and I respond: a creationist is ‘a c’; I prefer to reverse the letters and be known as ‘C a’ – “CREATOR acknowledger” whose MILLENNIUM Hymn spells out supra-scientific Truth [38] about The PERSON who said “Before Abraham was I AM” [39].
Conflict of Interest: Believer in The Lord Jesus Christ [Nuntsɔ Yesu Kristo]
Felix I D Konotey-Ahulu FGA MD(Lond) FRCP(Lond) FRCP(Glasg) DTMH(L’pool) FGCP FWACP FTWAS Kwegyir Aggrey Distinguished Professor of Human Genetics University of Cape Coast Ghana & Former Consultant Physician Genetic Counsellor in Sickle Cell and Other Haemoglobinopathies, 9 Harley Street, London W1G 9AL. Twitter@profkonoteyahul

1 McCartney Margaret. Evidence in a post-truth world. BMJ 2016; 355: 16363 November 28, 2016
2 Dyer Clare. Journal agrees to retract paper after university found study was never done. BMJ 2013; 347: f55
3 Konotey-Ahulu FID “Private Thoughts: There is no evidence that I was born on a Saturday. Postgraduate Medical Journal of Ghana 2012; 1: 32-33 33” which often proved superiority of history over science in arriving at truth.
4 Einstein Albert “Science can be created only by those who are thoroughly imbued with the aspiration toward truth and understanding” In EINSTEIN: Life and Universe, by Walter Isaacson, New York 2007, Simon & Schuster, page 390 of 675 pages.
5 Konotey-Ahulu FID. Sickle cell and altitude. BMJ 1972; 2: 231-232.
6 Zuckerman Solly. Pride and Prejudice in Science. Aerospace Medicine 1974. 45: 638-647. (Also republished in Ghana Medical Journal 1975; 14: 52-60)
7 Konotey-Ahulu FID. Clinicians facing conflicting recommendations: Use Common sense? BMJ Rapid response Dec 5 2008
8 Sarfati Jonathan. By Design – Evidence for Nature’s Intelligent Designer – The God of the Bible. Creation Book Publishers 2008 Atlanta Georgia, USA [260 pages]
9 Wise Jacqui. Consortium hopes to sequence genome of 1000 volunteers. BMJ 2008; 336: 237 (2 February)
10 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)
11 Konotey-Ahulu FID. 24 Konotey-Ahulu FID. Sequencing genome of 1000 volunteers: Why do this anonymously? African Journal of Health Sciences 2011; 18: 37-52
12 Watson James in Sunday Times: Racial differences in intelligence: James Watson, Nobel Prize winning geneticist persecuted for scientific truth. He claimed that black people were less intelligent than white people and the idea that “equal powers of reason” were shared across racial groups was a delusion. Human-Stupidity (Admin) March 14 2010.
13 Darwin Charles R. Origin of Species by Means of Natural Selection, or The Preservation of the Favoured Races in the Struggle for Life. John Murray, London 1859.
14 Verkalk Robert. Revealed: Scientist who sparked racism row has black genes/The Independent > News> Science 9 December 2007
15 Konotey-Ahulu FID. There is but one human race. New African (London) December 2009, Number 490, page 4.
16 Konotey-Ahulu FID. The Human Genome Diversity Project: Cogitations of an African Native. In POLITICS AND THE LIFE SCIENCES, September 1999, Vol. 18(2), pp 317-322.
17 Konotey-Ahulu FID. Why should Africans fear any human genome project? Africawatch. November 2010, pages 34-36.
18 Ehrlich Paul R. Human Natures – Genes, Cultures, and the Human Prospect. Island Press/Shearwater Books, Washington DC 2000, page 308.
19 Holy Bible. (a) Psalm 30 verses 8 & 9: Let all the earth fear JEHOWAH. Let all the inhabitants of the world stand in awe of HIM. For HE spoke and it was done. He commanded, and it stood fast & (b) Hebrews chapter 1 verses 1 &2: God …has in these last days spoken to us by His SON, Whom HE has appointed heir of all things, through Whom also HE made the worlds & (c) Colossians chapter 1 verses 16 & 17 For by Christ all things were created that are in heaven and that are on earth, visible and invisible …all things were created through HIM and for HIM & (d) St John chapter 1 verses 1 to 3: In the beginning was the Word, and the Word was with God, and the Word was God. All things were made through Him, and without Him nothing was made that was made.
20 The God Delusion title devalues sensible discourse BMJ Rapid Response Oct 19 2016
21.. Hoyle Fred. The Intelligent Universe. Michael Joseph, London, 1985, page 25
22 Young JZ. Philosophy and the brain. New Yok, NY, US Oxford University Press 1987, viii 233 pp.
23 Lloyd-Jones DM. Truth Unchanged Unchanging. Crossway Books March 31 1993. ISBN-10 0-89107-706-5 Foreword>fore>Alistair Begg ..
24 Murray Iain (2013). Life of Martyn Lloyd-Jones 1899-1980 Banner of Truth Trust Edinburgh. [This doctor qualified from St Bartholomew’s Hospital London University with First Class Honours and Distinction in Medicine & Therapeutics. Proceeded Doctorate in Medicine with his MD Thesis on Subacute Bacterial Endocarditis. Assisted Lord Horder, Physician to King George V, then suddenly announced retirement from Clinical Medicine and became a preacher. Those not allergic to Christian Worship may wish to listen to Dr Martyn Lloyd-Jones MD MRCP. Listen to one of his messages at Westminster Chapel on 24th November 1963, Sunday after assassination of President Kennedy: Go to and click on JFK. I was there that never to be forgotten Sunday evening. Christians will cherish Dr Martyn Lloyd-Jones’ 15-minute-long extempore prayer after the second hymn, plus his never to be forgotten long sermon after the 3rd hymn “In the cross of Christ I glory”. When he stepped down from the pulpit drenched in sweat, having expounded Galatians 6 verse 14, and after the last hymn “Abide with me” and Benediction, we just sat there, stunned!] Dr Martyn Lloyd-Jones preached more than 1600 sermons. Jonathan Catherwood has all the details –
25 Konotey-Ahulu FID. The spiritual and the psychological in Clinical Medicine. (Personal View) BMJ 1977; 1: 1595 doi:10.1136/6076/1595 June 15.
26 Lloyd-Jones DM. Spiritual Depression: Its Causes and Cure. (ISBN 9780802813879) Amazon’s Book Store>Spiritual-De [One of top 100 Millennium books]
27 Lloyd-Jones DM. Conversions Psychological & Spiritual>Co 9780851100098 December 1 1959. Critique of Dr William Sargant’s Battle for the Mind
28 Konotey-Ahulu Felix & Rosemary – 50 – YouTube 50th Wedding Anniversary [Dr Martyn Lloyd-Jones marries them Easter Monday April 23 1962]
29 Konotey-Ahulu FID. Sickle Cell Trait: As with Statins When leading editors disagree please give principles same weight as details – September 2016
30 Laine C. Scientific misconduct hurts. Ann Intern Med 2016. doi:10.7326/M16-2550. pmid:27951591.
31 Dyer O. Peer reviewer stole article and published it as his own. BMJ 2016; 355:i6768. doi:10.1136/bmj.i6768. 3
32 Huang Wel. Peer reviewer stole article and published it as its own BMJ 2016; 355:i6768 doi:10.1136/bmj.i6768 :
33 Konotey-Ahulu FID. Born on a Saturday? Science vs History On The Origin and End of the World. Creation 38(4):2016
34 Konotey-Ahulu FID. Case of lightning burns. BMJ 1963; 1: 1547. June 8. doi:10.1136/bmj. 1.5344.1547
35 Konotey-Ahulu FID. Superstition and phenomena in Africa Personal View. BMJ 1969; 2:48 doi:10.1136/bmj.2.5648.48 April 15
36 Konotey-Ahulu FID. The supra-scientific in clinical medicine: a challenge to Professor Know-All. doi:10.1136/bmj.323.7327.1452 Brit Med J 2001; 323(7327): 1452-1453 (22-29 Dec)
37 Konotey-Ahulu FID. History versus Limits of Science: Is Solomonic Genius a Y Chromosome Phenomenon? J Genet Disorders Genetic Reports 2014; 3: 2 [Quoted Konotey-Ahulu FID (2012) “Private Thoughts: There is no evidence that I was born on a Saturday. Postgraduate Medical Journal of Ghana 1: 32-33 33” which often proved superiority of history over science in arriving at truth].
38 Konotey-Ahulu FID. MILLENNIUM Hymn – TIME WAS CREATED. 2000 .(Seven verses with Music)
39 Holy Bible: St John chapter 8 verse 58 – JESUS said to them “Most assuredly, I say to you: before Abraham was, I AM”.

Sickle Cell Trait: As with Statins, When Leading Editors Disagree Please Give Principles Same Weight As Details

Sickle Cell Trait: As with Statins, When Leading Editors Disagree Please Give Principles Same Weight As Details

Dr Fiona Godlee’s Editorial [1] is the basis of a Front Page Headline in the Daily Telegraph “‘End statins controversy’ with government review” [2]. Its Science Editor states “The Lancet argued that thousands of people had been misled into stopping their medication after two articles appeared in the BMJ questioning their use and warning of side effects”. [September 16]. Our editorial giants (BMJ & Lancet) clearly disagree on matters of detail relating to Statins so I am not going to wade into the “who found what?” questions when principles that have not been explored stare us in the face.

Dr Fiona Godlee was right to highlight the principle about the need to continue asking questions in a scientific debate when she said “who should decide when such questions are too dangerous to ask? Certainly not those who have a vested interest in the debate being shut down.” [1] Which leads me to something else that worries me, namely the general reluctance to call a spade a spade in scientific debate. In his otherwise impressive invited Editorial Professor Harlan M Krumholz of Yale School of Medicine makes the following statement: “Some people fear that data sharing could produce poor science that spreads misinformation. But science should be self-correcting when there is open access to the data” [3]. Now, come on, why do we give the impression that something called “poor science” is responsible for spreading misinformation? And when did “science” become “self-correcting”? Are we afraid to say it is scientists, not science, who need blaming for defects? And scientists, not science that have to do the “self-correcting”? Have we forgotten the BMJ article entitled “Journal agrees to retract paper after university found study was never done”? [4]. Was it Mr Poor Science that caused the problem or a human being? Probity involves human beings, not something called “Science”, which leads me to another principle.

To Lisa Blakemore the “debate on childhood vaccine adverse reactions” that some people want to curtail requires the same principle of “Fiona’s current thinking” [5]. But I go further. Just as Lisa Blakemore felt very strongly about childhood vaccines so do I about Sickle Cell Trait misinformation in a reputable journal like The Lancet which refused to publish my protest letter that demanded retraction of a dangerously flawed paper [6] exactly as I presented it [7]. If Lancet is not happy about BMJ’s viewpoint on Statins and there have been calls “for retraction of two BMJ articles that disputed the use of statins in low risk people” [1] why was my call for a retraction of a Sickle Cell Trait article [6] that had no laboratory results but only the word of the patient as evidence of Sickle Cell Trait not heeded? Was it because I implied that the reviewer of the article knew little about sickle cell trait? [7]

I was previously involved in making both BMJ and Lancet withdraw published reports. The then BMJ Editor Dr Martin Ware withdrew a false report [References available]. I was also first to publish that a Lancet article “left a lot to be desired” [8] whereupon Editors Robin Fox, David Sharp, and Imogen Evans pursued the authors who later confessed to “Erroneous data” [9] and the paper was withdrawn. As Former Editor of the Ghana Medical Journal I am now inclined to think from recent developments (Statins imbroglio not excluded) that any expert in future asked to referee a scientific paper must henceforth declare “Conflict of Interest” in the same way as authors are asked to do. I cannot advise Dame Sally Davies how to approach Dr Fiona Godlee’s request but one novel principle that could not be out of place would be to request a “Lack of Conflict of Interest Declaration” from referees of scientific papers especially from those that recommended for publication “Statins are OK”.

The leading author of the BMJ article on Sickle Cell Trait causing intestinal infarction in a lady after just 45 minutes’ flight from Kumasi to Accra leading to their recommendation that “all negro travellers need testing for sickle cells before flight” happened to be an employer of an international airline. BMJ pursued him to produce records on the patient with haemoglobin electrophoresis results confirming sickle cell trait. There was nothing. So BMJ’s Dr Martin Ware had the Case History withdrawn. And now Lancet publishes “acute rhabdomyolysis in a Sickle Cell Trait patient” after flying [6]. “Who passed this article for publication?” was my first of 4 questions sent to the Lancet [7]. Like the case that BMJ withdrew, the paper had no record of Haemoglobin electrophoresis. Nor was there G6PD quantification, no full blood count, nothing. Not even a sickle cell test result.

Our next move is quite clear. Northern Nigeria has 30 million sickle cell traits of whom quite a few are millionaires with private planes. If flying can make their muscles rot as the article in Lancet suggests [6] then they ought to know urgently. They will pay for an investigator to go to the national medical association of the doctor whose paper was published in Lancet and demand to see the patient’s records. We shall then
present the findings to the UK’s Committee on Publication Ethics (COPE) because this is a very serious matter. One in every three of the 90 million people in Northern Nigeria is Sickle Cell Trait, and Insurance Companies will be gloating as they quote Lancet in support of their high fees for Sickle Cell Traits. There is, sadly, no longer our friend Cambridge University’s Professor Hermann Lehmann, FRS to write as he did to The London Times whose Science Correspondent used the BMJ’s non-existent Case History to suggest grounding “All Black Air Crew”. Lehmann simply said “Look here. Black Sickle Trait athletes ran at the Olympic Games at Mexico City 7000 ft high and beat the entire world with Gold Medals” so why use a discredited Sickle Cell Trait Case Report in the BMJ to say all Black Air Crew should be grounded forthwith? [10] There are great financial implications in calling Sickle Cell Trait Sickle Cell Disease, that was why I was given 4 body-guards in Philadelphia for pointing out in my Keynote Address (“The Vital Difference Between Sickle Cell Trait and Sickle Cell Disease”) that Insurance Companies in New York were defrauding Sickle Cell Traits [11]. Indeed, just as I predicted [7], the flawed Sickle Cell Trait Rhabdomyolysis article [6] is being used frenetically on Facebook in such terms as “Sickle Cell Trait mixed with Rhabdomyolysis could equal sudden death during exercise” [12].

The Statins Debate may well involve reputable scientists who are economical with the truth, and we should not equate them with “poor science” that will “correct itself”.

Dr F I D Konotey-Ahulu MD(Lond FRCP(Lond) DTMH(L’pool) Former Editor of the Ghana Medical Journal. Kwegyir Aggrey Distinguished Professor of Human Genetics
University of Cape Coast, Ghana and Lately Consultant Physician Genetic Counsellor in Sickle Cell and Other Haemoglobinopathies, 9 Harley Street, London W1G 9AL.

  1. Godlee Fiona. Statins: We need an independent review. BMJ 2016; 354: i4992
  2. Knapton Sarah. “End statins controversy with government review. Daily Telegraph. Friday 16 September 2016, page 1.
  3. Krumholz Harlan M. Statins evidence: when answers also raise questions. Sharing the data is more likely to settle the debate than another review. BMJ 2016; 354: i1463 (doi:10.1136/bmj.i4963)
  4. Dyer Clare. Journal agrees to retract paper after university found study was never done. BMJ 2013; 347: 155 Sept 5 2013.
  5. Blakemore Sarah. Statins: We need an independent review. BMJ Rapid response
  6. Rhida A, Khan A, Al-Abayechi S, Puthenveetil V. Acute compartment syndrome secondary to rhabdomyolysis in a sickle cell trait patient. Lancet 2014; 384:2172
  7. Konotey-Ahulu FID. Dangerously flawed diagnosis of sickle cell trait in compartment syndrome rhabdomyolysis
  8. Konotey-Ahulu FID. Group specific component and HIV infection. Lancet 1987; i: 1267.
  9. Eales L-J, Nye KE, Pinching AJ. Group specific component and AIDS: Erroneous Data. Lancet 1988; i: 936.
  10. Lehmann Hermann. Sickle cell and flying. The Times, London January 4, 1972.
  11. Konotey-Ahulu FID. Four bodyguards and the perils of unmasking scientific truths. BMJ 2007; 335: 210-211
  12. Whiskey Delta Charlie [Facebook September 5 at 5:35 pm] Sickle Cell Trait mixed with Rhabdomyolysis could equal sudden death during exercise or if low oxygen levels are present. Secondary complications can be present as well #sicklecelltrait #exercise #rhabdomyolysis #trackandfield #marathontraining #2milerun #crossfit #physicaltraining #preseason #military my story in the bio# sicklecellawarenessmonth #itsnotblackgene #global #bloodline #fighgtsmalaria #hispanic #mediterranean #europe #asian bloodtests

Dangerously flawed diagnosis of sickle cell trait in compartment syndrome rhabdomyolysis article

Dangerously flawed diagnosis of sickle cell trait in compartment syndrome rhabdomyolysis article 

I find it astonishing that Lancet that once published “Beware of symptomatic sickle cell traits” 1 published this article of Ali Rhida et al 2 where sickle cell trait was diagnosed purely on the patient’s say so without as much as a sickle cell test nor haemoglobin electrophoresis to quantify abnormal haemoglobin fractions.

Questions that require answering are

(a) Who passed this article for publication?
(b) What is the G6PD status of this patient? (Galtrey and Passansali described a Kenyan in whom “a diagnosis of G6PD deficiency and rhabdomyolysis was made”) 3
(c) Why did it not occur to the authors that their mention of “suspected sickle cell crisis” 2
should have alerted them to the fact that they were dealing most probably with sickle cell
haemoglobin C disease, “SC” phenotype and not sickle cell trait, “AS” phenotype (with ‘A’ greater than ‘S’)?
(d) Will Lancet Editor be kind enough to request that Ali Rhida and colleagues produce
details not only of all haemoglobins including “A2” and of “F” fractions but also of quantified G6PD level by recalling the patient and conducting these tests they should have done before?

Why is all this extremely important? ANSWER: Because Insurance Companies in the USA are queueing up to load the Premium of anyone called “Sickle Cell Trait”, using as the reason for doing so articles such as we read here. So serious has been the misinformation and disinformation on Sickle Cell Trait that when the Martin Luther King Jr Foundation Award honoured some 20 of us worldwide (including Linus Pauling, Hermann Lehmann, Max Perutz, Roland Scott, Bela Ringelhann, Helen Ranney, Doris Wethers, James Bowman, Charles Whitten, Graham Serjeant, Samuel Charache, A C Allison, J V Neel and others) for outstanding contribution to Sickle Cell Research I, who then ran the largest Sickle Cell Clinic in the world at Korle Bu Teaching Hospital in Ghana was asked to give the Keynote Address on “The Vital Difference between Sickle Cell Trait and Sickle Cell Disease”, and because I produced facts that Sickle Cell Traits had run and won Olympic Gold Medals at Mexico City 7000 ft above sea level only to come to ground level in New York for Insurance Companies to load their Premium, proving to the hilt that sickle
cell disease was being called sickle cell trait deliberately, or through ignorance I was given 4 bodyguards for all the 4 days I was in Philadelphia.4

I do not have to tell Lancet’s Editor what to do, but this article of Ali Rhida et al 2 is nothing short of dangerous, as the references I have accumulated on this matter demonstrate 5.

I declare no competing interests

Felix I D Konotey-Ahulu

Kwegyir Aggrey Distinguished Professor of Human Genetics, University of Cape Coast, Ghana and Recent Consultant Physician Genetic Counsellor in Sickle Cell and Other Haemoglobinopathies, 9 Harley Street Ltd., Phoenix Hospital Group, London W1G 9AL
1 Konotey-Ahulu F. Beware of symptomatic sickle cell traits. Lancet 1992; 339: 555

2 Rhida A, Khan A, Al-Abayechi S, Puthenveetil V. Acute compartment syndrome secondary to rhabdomyolysis in a sickle cell trait patient. Lancet 2014; 384:2172

3 Galtery Clare M, Pathansal R. New onset diabetes complicated by haemolysis and rhabdomyolysis: a case report and review of the literature. Journal of Medical Case Reports 2008; 2: 159

4 Konotey-Ahulu FID. Four bodyguards and the perils of unmasking scientific truths.BMJ 2007; 335: 210-211

5 Konotey-Ahulu FID. (a) Blaming sudden death on sickle cell trait?
(b) Sickle Cell Trait Misinformation and Disinformation (c)
Further Communication on Sickle Cell Trait Misinformation and Disinformation Respectively (a) Sept 19 2011 (b) Nov 30 2011 (c) June 19 2012.

Lesson of the week – Dorothy S. Amanor-Boadu

How I, a sickle cell anaemia woman, hydrate myself intravenously at 36000 feet

Dorothy Amanor-Boadu SRN SCM
The Elite Nursing Agency & Health Promotion Centre P O Box AN 6519, Accra-North, Ghana. [Email:]

I was born with sickle cell anaemia [‘SS’]. My health, which had been pretty stable until a serious sickle crisis was precipitated by (I am embarrassed to say this) a dietary  indiscretion – overeating. This first severe crisis happened at the age of 21 when I was a nurse at the Nursing Training College, Korle Bu Hospital, Accra, and almost cost me my life. If it had not been for the expertise and dedication of my doctors Dr. F.I.D. Konotey-Ahulu and Dr. Alexander Bruce Tagoe who stayed on duty for 3 days with no rest, I would not be here to write this story.

They did not go home during my critical stage complicated by thrombo-embolism brought on after a ‘cut down’ on my right ankle, performed due to inability to find any veins during the crisis. I was in hypovolaemic shock. Overeating is a known precipitating cause of sickle cell crisis

[1]. I went on to qualify as a State Registered Nurse in 1976, and worked as a Staff Nurse in charge of the Dermatology Department and Institute of Clinical Genetics Sickle Cell Clinic at Korle Bu Hospital for a little over a year. I was specially chosen to work with the sickle cell patients because my doctors thought I could empathise with them, which I did. When they were in pain, I identified with them, and wiped their brow.

I then left for the UK, a cold country, where I underwent Midwifery training at the Jessop Hospital for Women in Sheffield. I chose to do midwifery in addition to my SRN, because my mother was a well-known midwife in Accra. After this, I proceeded to the USA to specialise in Oncology Nursing, and even wrote a book

[2]. In the USA, I began to have about two hospital admissions per year for sickle cell crises with hospitalisations lasting up to 10 days at a time. All other crises occurring approximately every three months or so, lasting up to four days, were treated at home. I am now over 50 years of age, but from 1992 or thereabouts, my health gradually, but noticeably declined progressively resulting in more frequent and more severe episodes of crises. This could be dated from when I (foolishly) indulged in overeating again and ended up with an extremely severe crisis in Georgia. During this crisis, I developed a bedsore on the heel of my left heel in less than 18 hours of admission; in fact, it occurred while still in the emergency room! This crisis, occurring after I had left the restaurant with my husband and daughter where we had all enjoyed a Chinese dinner, was the second most severe in my life. [I had been taught to look for a husband who was ‘AA’, and I found one, and we have a daughter with ‘AS’ Haemoglobin Type, just like my parents]. Within 4 hours after the Chinese dinner, I presented myself at the emergency room in severe pain all over the body. Being a nurse, I often directed the doctors what to do. Quite a few times I asked them to ring Dr Konotey-Ahulu in London who would always ask the doctors to listen to me [Oh the wonder of Mobile Phones! Felix was always contactable].

I had never encountered any problems with flying on the ‘long haul flights’ (e.g.6- 9 hours from Ghana to UK/ USA and vice versa) until I reached the age of 40 years; then ‘all hell broke loose’.

Crises became even more frequent. I found it difficult to avoid the precipitating causes like late nights and (dare I say it?) too much good food.

Blood vessels for intravenous fluid (not blood usually) in crisis became more and more difficult to find, so a central venous access device was implanted into my chest which connects to a major blood vessel (superior vena cava) which, when in use, permits blood to be drawn from, and administration of intravenous drugs and fluids (drip). The device is also known as a ‘portocath/infusaport’ (depending on brand inserted). I have had this device over the past fifteen (15) years or so (each device being surgically implanted and removed/changed should infection, leakage or blockage be noted). Mine has been changed five times so far at an interval of every 2- 5 years. My port was initially inserted following an extremely bad crisis where I went into shock and no peripheral veins could be found anywhere on my body including my feet; any line obtained infiltrated within less than 12 hours. Finally, an intravenous (IV) line was inserted into my neck (central line) as a temporary measure until the port was inserted. (This is generally done in the operating room with a mild general anaesthetic or in the radiology procedures lab/room under fluoroscopic guidance with ‘twilight sleep’ induced (awake, but not fully aware)).

It was not until I went to live in the USA that I decided I had had enough of going to hospital for every crisis not resolved by oral hydration and Paracetamol, or other stronger analgesics. In the USA I found patients could come on a daily basis for intravenous drugs to be administered using the same IV site for up to 3 days or more by attaching a ‘heparin well’ to the butterfly or angiocath.

This gave me the idea of having this venous device inserted for me so I could have my intravenous fluids (drip) administered in the comfort of my own home, rather than on the hard emergency room stretcher and be charged $250.00 per hour for those uncomfortable facilities!

Having convinced my physician (who in America were haematologists) to agree to this, I treated most of my crises at home, administering the IV fluids and drugs knowing I could go on admission with a call to my doctor, if I thought things were not progressing well or I could no longer cope. With the use of the ‘hep well’, I would go to the emergency room to have it inserted (and labs drawn as ordered) and return a few days later when better to have it removed. After the hepwell other venous access devices were developed over
the years, including the ‘Hickman Catheter’ (catheter surgically inserted into a major vessel protruding through the skin with catheter hanging out on the surface), and the ‘Port O Cath’ which is a central venous access device surgically implanted under the skin in the areas of the chest, arm, or groin. I acquired my first one in 1990. When not in use, it cannot always be seen, but since I do not have too much adipose tissue to hide it, mine protrudes, and looks like a little round lump under the skin – I call it my 3rd breast!

This device allowed me to avoid emergency room visits even more. I could now access (stick) myself with the ‘Huber needle’ and hook up my drip without assistance. All I had to do was call my doctor to let him know I was in trouble (i.e. in crisis) and he would call in my prescriptions to the hospital pharmacy. My husband would then pick them up and bring them to me at home, thus bypassing the Emergency Room altogether. Being a nurse, and working in the Haematology/Oncology areas, had really become useful to me in my personal life.

To think that if it had not been for Dr. FID Konotey-Ahulu and Dr. K.A Gbedemah my parents would not have allowed me to become a nurse as the latter thought the profession could prove physically taxing! I am glad I did nursing.

At this stage, I would like to acknowledge the following specialists: Dr. Jeffrey Giguere, in Greenville South Carolina, Dr. Laura de Castro, Durham North Carolina and Dr.Melanie Jacobs, Atlanta Georgia with gratitude for the trust and confidence they had in me to permit me to treat myself with minimal supervision, and without fear that I might become a drug addict and abuse the use of the port.

Well, back to our topic. After having flown on numerous occasions back and forth from the UK to USA and Ghana without incident (the planes by then were all using pressurised cabins and later prohibited smoking also which was helpful), I noticed after the age of 40 that on flights I was now tending to start ‘sickling’ approximately 3-4 hours into the flights. I was advised by Prof. F.I.D. Konotey-Ahulu to hydrate more before and after flights, to eat only a light meal prior to and during the flight, to move about more, and to start using Aerobic Oxygen 15-20 drops to a glass of water three times a day [3], which of course I drink on flights also. Following this advice and to make sure I got through without problems and inconvenience, I requested clearance from the medical department of the carrier I used most frequently (British Airways) for oxygen to be made available to me on the flights and permission for me to administer intravenous (IV) medications and fluids should it become necessary.

This procedure meant my physician and I had to complete a medical form which basically identified my condition and my requirements for the flight (oxygen for which an additional non-refundable charge of $150.00/£200.00 is required) and the physician’s opinion as to my fitness for the flight with confirmation that I did not need to be accompanied. With the assurance that I was a nurse and could take care of myself without assistance, the airline has to date always agreed to my requests.

After travelling yearly over a period of about 4 years, my physicians no longer have to fill forms for my medical clearance – a simple telephone call to the airlines medical division in London, U.K with confirmation of flight itinerary results in a printout attached to my ticket informing airline staff of my diagnosis and requirements always sufficed [Oh thank you British Airways! (and I hope I am not accused of advertising)]. I also request seating at the bulkhead/window (least inconveniencing to other passengers, should drip be required, also more legroom and space to manoeuvre in economy class).

During packing for my intended flight, included in my hand luggage is at least 1-2 litres of IV Fluids, Infusion sets, Syringes of different sizes 10/5/3ml with and without needles, Luer lock caps, adhesive tape, tegaderm dressing, normal saline and heparin flushes, alcohol swabs. Medications as prescribed including tablets and injection Phenergan, Pethidine, Paracetamol, Airtal or Percocet. One or two bottles of IV Ciprofloxacin and a small ‘sharps/biohazard’ container for disposal of needles and glass ampoules are also packed.

Finally, a letter from my physician giving reasons and the need for my possession of needles, lest some busybody security person suspect me of being a terrorist! Just a word here about my experience with pain killers on 3 continents. In Africa, neither Morphine nor Diamorphine are used for sickle crisis. In the USA Diamorphine is banned for clinical use. In the UK, both Morphine and Diamorphine are used for sickle cell crisis. In Ghana, we find Diclofenac, Airtal, and Ketorolac, with plenty of hydration, and occasionally Pethidine very briefly, more than adequate for the severest of crises.

Should I be flying with a carrier whose arrival and departure times are not always reliable a call to the airport to verify that flight is likely to depart on time is made. My goal here is to administer my intravenous fluids no more than an hour before checking in time to ensure adequate hydration for the flight. I have on occasion had to have my flight rebooked due to constant changes in flight departure time. Original departure of one airline that shall remain nameless was for 8pm, changed to 10pm, then 3am. Finally, the plane left without me at 5am. I took the flight 2 days later to get my hydration time right! The moral for other patients reading this article is this: ANTICIPATE PROBLEMS, AND PLAN HOW TO COPE WITH THEM! If necessary, cancel agreed plans (like a flight) in order to save your life.

My normal procedure now before any flight is to pack my bags about two weeks prior to the departure date (generally reduce stress – physical/emotional as much as possible several days/weeks before flight, and increase oral hydration three days before departure). On the date of departure I check in early where possible, return home to rest, take a bath prior to inserting my Huber needle into the centre of the port after cleaning it and an area of approximately 2” circumference with Betadine and alcohol swabs, using sterile technique secure/cover with appropriate dressing – Tegaderm/biocclusive (usually 3”x5”-this is a water repellent dressing applied to secure and protect port from invasion of pathogenic organisms etc) [Picture]. It is possible to shower/bathe without the insertion site becoming wet. Dressing change is required every third day and as necessary. Huber needle (90-degree angled needle) should be changed every 7 days, but should be removed as soon as no longer required for use to avoid risk of infection.

Once ‘hooked up’ I administer 500-1000ml of Dextrose/Saline or Normal Saline over 2-4 hours depending on total amount to be given and how I feel at the time. On completion, the Port is flushed with 10ml saline and 3ml U100 heparin flush, then capped and extension tubing tucked into my ‘bra’. IV infusion/tubing capped off and stored in a new clean self-sealing plastic bag, which is then added to my hand luggage.

Once on board the flight, with any signs of sickling (pain/discomfort) I first request for oxygen which, is either piped directly to my individual seat (from the cockpit) or via small portable tanks supplied by stewardesses. This is administered at 2-4 Litres/min depending on how acute the distress is. After 20 minutes or so, if no relief of symptoms are noted I inform the stewardess of my intent to administer IV Fluids and drugs, and request for a clothes hanger. (Usually at this point there is panic amongst the Air Crew because, despite the clearance printout from the medical department and the Ground Crew having all the details and specifics, the aircrew generally are left with only minimum information such as “they have a medical on board”).

At this point I usually get a visit from the Captain and Chief stewardess. Once everyone gets the fact that there was some miscommunication, I proceed to hang and prime the infusion tubing. The hanger is used to hang the infusion onto an appropriate fixture in front of or above me. Occasionally, depending on location of seat adhesive tape may be required to secure drip at a height to promote gravity drainage. Since the port is already accessed and ready for use all that is required is to flush the extension tubing with saline prior to administering IV medications and hooking up the infusion. Once the rate for infusion is regulated, a little nap with continuous oxygen usually averts any major crisis occurring even if not totally resolved. Most of the time, oxygen is not required for more than 2 hours and IV for 4 hours.

My IV line can be disconnected, flushed and capped off when I feel I no longer need it or at least thirty minutes before landing. To date there have been no complaints or concerns from other passengers. Everyone, crew and passengers alike have been considerate and helpful – willing to change seats where needed.

So now you know how Intravenous fluids can be safely and conveniently self- administered at 36000 feet!

Competing interests: None declared.

Acknowledgement: I thank Professor F I D Konotey-Ahulu for urging me to tell this story to encourage sickle cell disease patients never to give up.

1 Konotey-Ahulu FID. The sickle cell disease patient. Natural history from a
clinico-epidemiological study of the first 1550 patients of Korle Bu Hospital Sickle Cell Clinic. Watford, Tetteh-A’Domeno, 1996, page 122.

2 Amanor-Owusu D. Cancer Chemotherapy Manual. Legon, Accra. Ghana Universities Press, 2002

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

Legend: Accessed Port covered with Tegaderm dressing. Note pallor of my nail: I have sickle cell anaemia with a cruising Haemoglobin level of 8 grams/dL.

Submitted by:Dorothy S. Amanor-Boadu