To Sickle Cell Education Day in Maryland USA May 15, 2021

Sorry, your Registration Procedure was not geared to record my UK phone number so I could not join you to contribute something and to answer questions that could have cropped up. The following is rather lengthy.

My www.sicklecell.md shows how patients can overcome their Sickle Cell Disease and achieve great things in life. Introducing myself, listen to   Professor Helen Ranney MD PhD of Albert Einstein University College of Medicine in New York: “There is no single clinical experience in the United States comparable to that of Dr Konotey-Ahulu”.

She observed that I always placed “The Patient” above “The Disease”.

My book “The Sickle Cell Disease Patient” with 643 pages including 133 Illustrative Case Histories plus thousands of References, and the Foreword by Howard University’s famous Professor Roland Scott MD, describes all that patients require to help them stay out of the hospital and to achieve great things in life. Not Gene Therapy. Order the book from my website, discounted to £30. It deals with The Patient.

My Johns Hopkins Visits

I am no stranger to Maryland. Your Professors Lemuel Conley and Samuel Charache of Johns Hopkins University Medical College had invited me to do Grand Rounds more than once with them at Baltimore.

On one occasion I showed a slide to their students and physicians on “Sixteen Causes of Severe Jaundice in Sickle Cell Disease” which so impressed Professor Charache that he asked me to give him a copy of the slide. Some of their students who heard him quiz me on those Grand Rounds may well now be your present Physicians and Haematologists.

You can find the list of Sixteen Causes of Severe Jaundice in Sickle Cell Disease on page 174 (Table 13.7) of my Textbook analysing 1,500 Consecutive patients with Sickle Cell Disease in Ghana. I am prepared to send you a signed Complimentary copy of my book because of the friendship of Professors Conley and Charache in the past. Tell me where to send it by DHL (Address plus phone numbers). We were together on the WHO Expert Advisory Panel on Haemoglobinopathies in Geneva.

I was once gratefully surprised when Professor Samuel Charache gave me $150 on the spot in the Emergency Room in Baltimore for diagnosing Right Middle Lobe Pneumonia in a Sickle Cell Anaemia (SS) lady in sickle cell crisis, using “HIPPA” – the 5 Cardinal things taught me at Westminster Hospital School of Medicine, the University of London, by Sir Richard Bayliss MA MD FRCP, Physician of Her Majesty Queen Elisabeth II..

HIPPA stands for “History, Inspection, Palpation, Percussion, Auscultation”. After I announced the diagnosis aloud for nurses and doctors to hear, the lady was wheeled away to be X-rayed. When the diagnosis was then displayed on the screen, confirmed, and the $150 thrust in my hands I was hugely grateful because that amount of One Hundred and Fifty Dollars was equivalent to my 3 months’ salary as a Physician Specialist in Inflation-burdened Ghana. It was then I realised why several of the doctors we trained at home in exactly the way we were trained in the UK, found their way across The Atlantic to acquire Dollars that they sent back home to help relatives. Brain Drain meant Dollar Influx! Diagnose without X-rays and you get Dollars?

 

In later years I invited Professor Samuel Charache to International Conferences of Sickle Cell Disease Patient Achievers The First at The Royal Society of Medicine in London 1993, and the Second in Accra, Ghana, in 1995 when he saw adult Sickle Cell Disease Patients who had never been regularly blood-transfused, and who had graduated from universities. The Hydroxyurea that Prof. Samuel Charache popularized was, and still is, more Disease-oriented than Patient-oriented in that the drug aimed at producing Foetal Haemoglobin F to displace the Abnormal Haemoglobin S with the view to diminishing transfusion requirements. Our ACHIEVERS’ CONFERENCES were aimed at showing off adult patients who were never on regular transfusions as was commonly practised in the UK and the USA.

We have adult Sickle Cell Disease Patients in their Seventh and Eighth Decades of useful life. One Sickle Cell Haemoglobin C Disease (phenotype SC) grandmother, and Women’s Golf Champion, attained the age of 83 years before finishing her Earthly Pilgrimage. During her first pregnancy when repeated urinary tract infections precipitated sickle cell crises some physicians suggested she abort the pregnancy, as was often the practice abroad. She flatly refused, and I concurred with her decision in 1960 when Dr Fred Sai let me admit her to Ward A at Korle Bu Hospital. Being myself born into a Sickle Cell Disease family I knew relatives who carried pregnancies to term. We carefully nurtured Mrs L. O. till she was successfully delivered of Twins by Dr Kwesi Bentsi-Enchil our experienced Obstetrician Gynaecologist. The Trait Twins are now grandparents and successful professionals in their own thriving businesses.

MORAL: Sickle Cell Disease Patients can become ACHIEVERS without Management Practices involving Regular Blood Transfusions, Ante-natal Diagnosis and Terminations of Pregnancy, Morphine and Diamorphine for Sickle Cell Crisis Pain, Hydroxyurea daily to increase Foetal Haemoglobin Level, and Frequent hospitalisations. They can achieve great things right up to their Seventies and Eighties.

EXAMPLES

One ACHIEVER, D. A-B [SS (NORMACHE “AS” father x NORMACHE “AS” mother produced ACHEACHE)] trained to be a brilliant Nurse SRN in Ghana, went to Cold England to do Midwifery SCM, proceeded to the USA to specialise in Chemotherapy and wrote a Textbook. You can read the description of how she hydrated herself intravenously flying at over 30,000 ft more than once. See www.sicklecell.md BLOG “Lesson of the Week – Dorothy Amanor-Boadu SRN SCM – How I, a sickle cell anaemia woman, hydrate myself intravenously at 36,000 feet

Her advice in bold letters to fellow Sickle Cell Disease Patients: “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. 

 Dorothy Amanor-Boadu attended all 3 SICKLE CELL DISEASE PATIENT ACHEVERS’ CONFERENCES in 1993 (London), 1995 (Accra), and in 2010 (Accra) for the Centenary of Herrick’s first description of Sickle Cell Anaemia in the USA. Great ACHIEVER that she is, she runs a Private Nursing Agency in Accra, and has authored a 186-page book entitled “CANCER CHEMOTHERAPY MANUAL” by DOROTHY S AMANOR-OWUSU. She was offered Bone Marrow Transplantation in the USA but refused. She does not think Gene Therapy is the answer either.

One hopes, GOD willing, Maryland Sickle Cell Disease Association can attend a 4th International Conference with their own ACHIEVERS. I was greatly saddened to learn that my good friend Professor Samuel Charache died on 29th January 2019 aged 89 years.

ANOTHER SICKLE CELL DISEASE PATIENT ACHIEVER:

Listen to me interviewing Mr Ebenezer Tagoe with Sickle Cell Anaemia (SS), a great ACHIEVER now in his 7th Decade of life (born on Ghana’s Independence Day 6th March 1957). In 1993 & 1995 Professor Samuel Charache heard Ebenezer testify at both our SICKLE CELL DISEASE PATIENT ACHIEVERS’ CONFERENCES in London and Accra. At www.sicklecell.md  BLOG you can see him being interviewed live “Meet the Professor talking about Sickle Cell Disease patient live”. Mr Ebenezer Tagoe [SS=ACHEACHE (one S-ACHE gene from father, and another S-ACHE gene from the mother)] was counselled by me and he married an “AA=NORMNORM phenotype” lady. They had 2 “AS” Trait boys who graduated.

Ebenezer is now a qualified Pharmacist who had worked in a London Teaching Hospital, and now a successful businessman in Accra. While in London he was appalled by how many patients with Sickle Cell Disease were given Morphine and Diamorphine for Sickle Cell Crisis when he himself had never been introduced to it. He testifies that as he is a pharmacist, he has declined to take daily Hydroxyurea.

Ebenezer Tagoe is fortunate to have Doctors both in Ghana and England whom he described as “excellent” because they always listen to him. He was once going to be given Morphine after surgery in the UK to remove his gallbladder but he refused and asked to be given Ketorolac instead. At all 3 INTERNATIONAL ACHIEVERS’ CONFERENCES, Mr Tagoe announced that he avoided Sickle Cell Crises by drinking no less than 5 Litres of water a day. I now pass this information on to Maryland Sickle Cell Disease Association (MSCDA) members of which may like to contact him through me felix@sicklecell.md and he will be delighted to answer your queries.

ANOTHER SICKLE CELL ANAEMIA (SS) ACHIEVER:

Miss A. K. who is in her 6th Decade of life with Sickle Cell Anaemia is glad to answer questions through me. The only partial blood transfusion she has ever had was for hip replacement (aseptic necrosis). She keeps very well with haemoglobin level always between 7.5 and 8.5 grams per Deci-Litre which she is very pleased with.

SEVENTYONE YEAR-OLD ADE SAWYERR (SC):

Mr Ade Sawyerr with Sickle Cell Haemoglobin C Disease will answer queries about how to avoid sickle cell crises. He has valuable tips and I have taken him with me to answer questions when I am invited for Grand Rounds. Write to him through me.

Glucose 6 Phosphate Dehydrogenase (G6PD) Deficiency

This is one of the 16 causes of Severe Jaundice in Sickle Cell Disease. All new patients I saw in Ghana or UK or in Howard University Washington DC had G6PD enzyme level checked. Some good drugs harm patients with G6PD Deficiency as shown in – https://bit.ly/2sukoPWG6PD Deficiency in Ghanaians: How to recognise it”.  Have all the patients in MSCDA been checked for G6PD Deficiency?

In a way, I am glad I could not join the webinar across The Atlantic because I could not have contributed a tenth of what I have just written.

Finally, GENE EDITING (CRISPR) is woefully unrealistic: How can this deal with the 34,000 babies born every quarter of the year with abnormal haemoglobin genes from both parents in West Africa? Genetic Counselling with Family Size Limitation [GCFSL] is more realistic. See my answer to the Sickle Cell Disease mother married to Sickle Cell Disease man in Tema, Ghana, and who had 13 children all receiving an abnormal haemoglobin gene from both parents causing hereditary disease in all of them. They continued having child after child hoping the next one will avoid hereditary disease but because each Parent had nothing but an aching gene to offer, all the 13 children ended up with ACHE from father, ACHE from mother to be ACHEACHE disease phenotypes.  See on my website how I responded to Mrs D’s reasonable question: “But Doctor: How do we prevent this Nwiiwii (Sickle Cell Disease in Fante) coming in further pregnancies? Study the answer I gave the parents from the Video on my website www.sicklecell.md My invented kanad cubes are given free with every copy of the book – “kanad” stands for KONOTEY-AHULU NORM ACHE DICE

Felix I D Konotey-Ahulu FGA MB BS MD(Lond) DSc(UCC) DSc(UH) 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.konotey-ahulu.com and https://youtu.be/41a1FZSpHd4 which is Video with CITATION of Honorary DSc awarded me by the University of Hertfordshire.

DR REGINALD ADDAE: WATCHFUL CONSULTANT PHYSICIAN

DR REGINALD ADDAE: WATCHFUL CONSULTANT PHYSICIAN
TRIBUTE BY DR FELIX I D KONOTEY-AHULU

Another great Ghanaian Physician has left us for good, but not really forever because much of what he did in life will be remembered for decades to come. Those who read international medical journals will recall the role DR REGINALD ADDAE & DR FRANK T DJABANOR played in SAVING BLACK PEOPLE FROM HUMILIATION.

And these two Ghanaian London-University-trained doctors did this by revealing as TOTALLY FALSE an international medical report which advised that

“If on certain African routes, a Negro traveller must take an unpressurized aircraft it would be wise to ascertain the sickling status before departure”.

imagine you and I being pulled out of a queue boarding an aeroplane to be tested at the airport for sickle cells. The FALSE international publication was so significant that within a week, the world’s leading Science Journal NATURE, and the leading British Newspaper THE TIMES elaborated the original false article and made their own recommendations. THE TIMES Science Correspondent even went further to recommend that all Black Air Crews should be grounded immediately.

FLIGHT FROM KUMASI TO ACCRA

The FALSE medical report was based on a Ghanaian nurse flying from Kumasi to Accra and who during the 45 minutes flight developed tummy problems. On arrival in Accra she was operated upon by a non-Ghanaian surgeon at the 37-General Hospital now called The Military Hospital. The report in the Medical Journal by 3 non-Africans who had never been to Africa, said the Ghanaian nurse was Sickle Cell Trait proven by the standard test called Haemoglobin Electrophoresis. This report caught the sharp eyes of the two Ghanaian Physicians at the Military Hospital even though “Military Hospital” was not mentioned in any of the international publications. Reggie Addae was the first to write to the international Medical Journal asking for proof that the nurse
was sickle cell trait (Haemoglobin Electrophoresis “AS”). Next to write for proof of what was said to have been performed at our Military Hospital was Dr Frank Djabanor. Dr Reginald Addae gave at least 8 (eight) criteria later known as “ADDAE CRITERIA” to be honoured before any clinical condition could be attributed to the Sickle Cell Trait (“AS”). Meanwhile, Kennedy Airport grounded their Black Pilots and Air Crews.

“SCIENTIFIC FALSEHOOD” EXPOSED BY WHITE & BLACK EXPERTS

Professor Hermann Lehmann FRS of Cambridge University wrote to THE TIMES after reading its recommendation that all Black Air Crew should be grounded and pointed out that the Sickle Cell Trait “AS” did not constitute a disease phenotype, and that Sickle Cell Traits ran at the OLYMPIC GAMES at Mexico City, 7,000 ft above sea level, and thrashed the entire world. How could they have a disease? He began his letter of protest thus: “SIR – Your Science Report on December 9 1971 has suggested that all flight crews with SICKLE CELL TRAIT should be removed from flying duties and that all prospective Negro travellers should be screened for sickle cells”.

Dr Frank Djabanor whose protest letter followed Dr Reginald Addae’s said “Sickle Cells are not a Black Condition”, and that Europeans also had Sickle Cell Trait, sometimes “with higher percentages than anywhere in Ghana”. Listen to his logic:

“In some parts of Greece the prevalence of the Sickle Cell Trait is 30% (according to Professors Deliyannis and Tavlarakis, British Medical Journal 1955 volume 2 page 299); more than twice the prevalence in Northern Ghana.” Dr Frank Djabanor then continued with this telling point: “The forebears and descendants of these non-Negro possessors of the sickle cell gene are, doubtlessly, scattered over America and Europe today. How can we identify them, from their external features, to thrust upon them the ‘benefits’ of this advice?” If, “for their own safety” all Negro travellers should be screened, “Surely”, Dr Djabanor continued “the authors would not advocate that we
deny some sections of the world community the benefits of their suggestion”.

Dr Frank Djabanor went HOME to GLORY last year, and my TRIBUTE to him was titled: “Lt-Col [Retd] F FT Djabanor (1938 – 2019). Physician whose exposure of scientific falsehood saved Black People from humiliation. TRIBUTE: ACCRA & Odumase-Krobo 31st March 2019.

I made sure that his burial was marked in the British Medical Journal with a detailed account of what he and Dr Reginald Addae who now follows him to GLORY did for all black people. See https://www.bmj.com/content/364/bmj.I735/rr-7 Konotey-Ahulu FID.
“Aviation Safety: Ghanaians recall media disinformation deriving from scientific misinformation. British Medical Journal Rapid Response March 1 2019 to Partha Kar – Applying Aviation Safety to Healthcare – Are we missing the fundamentals? Partha Kar 364: doi 10.1136/bmj.I735

THIS TRIBUTE TOO LONG?

If some Ghanaians are inclined to think this TRIBUTE is too long, please remember what Dr Reginald Addae and his Colleague Dr Frank Djabanor achieved for us. One in every Five (5) of us Ghanaians reading this, or listening to it, is Sickle Cell Trait (“AS”). Scientists are continuing to claim that 20  percent of us have hereditary “DISEASE SICKLE CELL TRAIT. The British call it a “DISORDER”. The WHO IDM Chart calls it a “DISORDER”. White Experts who knew the truth, and who defended us Black people against Tafracher “scientific nonsense” (Hermann Lehmann, Bela Ringelhann, George Edington, etc) have died. Black Experts have also died (Alexander Boyo, Bill Laing, Teddy Christian, Komla Gbedemah, George Bonney, George Ankra-Badu etc). Now Frank Djabanor and Reggie Addae have also gone.
Those of us Ghanaians remaining who know both the scientific truth, and the “scientific lies” (Alexander Bruce-Tagoe, Joseph Kpakpo Acquaye, Samuel Kwadwo Owusu, Albert Amoah, Dwomoa Adu, Jehoram T Anim, Michael O Matekole, Adzei Klufio, E Q Archampong, Lade Wosornu, myself, etc) will continue to tell the world what Professor Hermann Lehmann FRS told the LONDON TIMES: “Sickle Cell Traits ran at the Olympic Games in Mexico City (where the Oxygen was thin) and beat the entire world”. We salute the Inseparable Duo Frankie and Reggie who have defeated their Last Enemy, Death, and arrived HOME in Heaven. Ao, Ayenyekoo!

Heartfelt Condolences to the loved ones – Sisters Regina, Amelia and adult children Gina, Wendy.

HEAVEN

Oh think to step ashore, and that shore Heaven;

To take hold of a hand, and that hand God’s hand;

To breathe a new air, and that air Celestial air;

To feel invigorated, and know it, Immortality;

Oh think, to pass from the storm and tempest

To one unbroken smile,

To wake and find it GLORY!

Centenarian Sickle Cell Trait Genetic Testing

Hundred-Year-Old Sickle Cell Trait Accesses Genetic Services to Predict Risk of Hereditary Disease in Future Generations

CONGRATULATIONS FROM BUCKINGHAM PALACE!

Dr Emmanuel Evans-Anfom FGA MB ChB FRCS(Edinburgh) FWACS DSc (Salford Univ. Hon) FGCPS DTMH(Edinburgh) who was 100 years on October 7 2019 [1] was congratulated from Buckingham Palace. The Duke of Edinburgh Prince Phillip, PATRON of Ghana Academy of Arts and Sciences [GAAS] and its First President 1959 to 1961 when he was succeeded by Dr Kwame Nkrumah 1961 to 1966 as the Academy’s Second President, sent a message of Congratulations to Dr Emmanuel Evans-Anfom who was himself once 8th of the 19 Presidents of GAAS.

“HISTORY SURPASSES SCIENCE” LECTURE FOR CENTENARIAN.

With Professor Henrietta Mensa-Bonsu FGA the present 19th President of GAAS chairing the Proceedings in a most competent manner, I considered myself greatly honoured and privileged to deliver a SPECIAL LECTURE not only preceded by introductory remarks of Professor Samuel Boakye FGA, Executive Secretary of the Ghana Academy of Arts and Sciences, but also by the impressive statement of Dr Emmanuel Evans-Anfom himself who can be seen and heard on Thursday 30th January 2020 here https://youtu.be/hV8NZJh1pg [2].

CENTENARIAN SERENDIPITOUSLY REQUESTS GENETIC MARKERS

Day after the Lecture dedicated to Dr Emmanuel Evans-Anfom I visited the great man at home at Leonora Lodge surrounded by his family [demure wife Elise, elegant daughter Rachel and confident husband Dr Henry Baddoo, impressive sons Charlie and Nii Teiko] when, out of the blue, he said to me “Felix will you confirm my genotype?” – meaning would I find out if he was/is one of the 1 in 3 Ghanaians (like my parents) with an abnormal beta haemoglobin gene “S” or “C”? [References 3 to 32].

I asked Dr Henry Baddoo, Consultant Anaesthetist son-in-law to draw blood for me to bring back to the UK for examination in the only laboratory I know in Central London that will quantify not only haemoglobin fractions but also the level of Glucose-6- Phosphate Dehydrogenase red cell enzyme whose deficiency in Ghana is high, 1 in 4 hemizygote males XminusY, 1 in 6 to 1 in 8 females (heterozygotes XminusX and homozygotes XminusXminus ) – Partial and Full Deficiency [18 19 23 24 27 to 42]

PERMISSION TO PUBLISH FINDINGS

I considered it serendipitous to be asked to confirm haemoglobin “genotype” just when BMJ was publishing such articles as “Promises and perils of using genetic tests to predict risk of disease” [43] and “Communities that prefer close blood marriages need more help to access genetic services”. [44] In the reliable Central London Lab I requested my “Konotey-Ahulu Profile Number 2” consisting of (a) Sickling Test, (b) Quantitative Haemoglobin Electrophoresis, (c) G6PD Quantitative value, (d) Haematological Values & ESR, and (e) Blood Group. I know of no other laboratory in the UK – Teaching Hospitals included – that can do these 5 tests for less than £100 (One Hundred Pounds Sterling).

RESULTS: (a) Sickling Test POSITIVE (b) Haemoglobin Electrophoresis “AS” with ‘S’ 38.1% (Hb S level being in the highest range of the 3 known Sickle Cell Trait ranges of ‘S’=20-28%, ‘S’=26-33%, ‘S’=34-39% [17 23 24 45 – 50]. Haemoglobin A2 3.0%, Haemoglobin F is 0.5% and the rest Normal Haemoglobin A. Red cell Glucose-6 Phosphate Dehydrogenase Enzyme level was 6.0 U/g [Normal Range 5.6 – 11.2].

VERDICT: Dr Emmanuel Evans-Anfom is therefore SICKLE CELL TRAIT “AS” with NORMAL G6PD. He readily gave me permission to publish the findings not only to help counter Misinformation, if not Disinformation, pervading the Internet based on flawed publications allowed in the best scientific and medical journals, but also to make this finding in a very alert Centenarian a MEDICAL MEMORANDUM.

MISINFORMATION MAKES NONSENSE OF GENETIC COUNSELLING

The very well-thought-out articles of Ian Scott, John Attia, Ray Moynihan [43] and Naz Khan and Sarah Salway [44] do not adequately explain the sheer size and varying details of genetic epidemiology for which people worldwide request guidance to prevent hereditary pathology in their offspring. The former concentrate on genetic disposition to malignant conditions of breast, ovary, and prostate [43] while the latter concern themselves with genetic consequences of marrying relatives [44]. The genetic epidemiological burden discussed by these authors [43 44] amounts to a tiny-fractional-tip of the global genetic iceberg whose huge base is in the Mediterranean countries, Middle East, Asia, Canada, USA, South America, the West Indies and, notably, Africa where genetic red cell defects, namely Haemoglobinopathy (qualitatively ABNORMAL haemoglobin genes), Thalassaemias (abnormal quantities of NORMAL Haemoglobin genes) and genes for G6PD Deficiency abound world-wide. [18 19 24 27 – 42 51 52 53].

UK’s GENETIC BURDEN IS BUT A FRACTION OF GLOBAL BURDEN

Can we also tackle this world-wide problem efficiently? In the UK 1 in every 25 people carries the Cystic Fibrosis gene. In Ghana 1 in every 3 of us carries either Hb S gene or Hb C gene (like my parents – father AC, mother AS just like Dr Evans-Anfom) with the result that 4 of 100 consecutive children born in Ghana suffer from hereditary aching disease ACHEACHE “SS”, “SC”, or “CC”. We proved this at Korle Bu Teaching Hospital genotyping 13000 consecutive births in a single year. [29], confirming the Hardy-Weinberg Equation [23 24(page 15)]. Yes, 1 in 3 of us in the Diaspora is Sickle Cell Trait “AS” or Haemoglobin C Trait “AC” needing tracing. [54]. Both Professor Graham Serjeant [52] in the West Indies and Professor Sir David Weatherall FRS [53] in Oxford acknowledge this global hereditary burden. For further emphasis, Cystic Fibrosis Carrier State is more than 8 times less than Sickle Cell Carrier State yet, as I [54] commented on Pascale Allotey’s excellent book-review [55] on “ethnicity and access to health care”, Lancet’s 7-part-series on Genetic Epidemiology came and went [56] “and not once was it mentioned that one in three west Africans in the UK has a β-globin variant gene whose unsuspecting owner needs to be identified and helped with genetic counselling and family size limitation.[54] I am sure that Ian Scott and colleagues [43] do not intentionally set out to create “the impression in our minds that genetic epidemiology, which has been my chief concern for decades [24 57-61] had little to do with us non-whites” [54], but a passing reference in their article to the African Diaspora situation [62] would not have been out of place.

HINDRANCES TO REALISTIC GENETIC COUNSELLING AFTER TESTING

Hampering realistic counselling is a combination of factors such as Poor Definitions (eg referring to “Trait” as “Disease” or “Disorder” as do the UK Genetics Council [63], Noke et al [64], NIH [65] plus Laboratory Errors like confusing Sickle Cell Trait with Sickle Cell beta-plus Thalassaemia [24, 53].
But chief of all culprits is Misinformation which if deliberate becomes Disinformation. This very important Misinformation Culprit was scrutinised comprehensively by me [66] when responding to the very instructive article of Semsarian and Ingles “A clinical approach to genetic testing for non-specialists” [67] together with Allison Streetly’s helpful comments [68] and needs not delay us further except to mention the remarkable opportunity
Centenarian Sickle cell trait Dr Emmanuel Evans-Anfom, the international hockey player who has flown hundreds of thousands of miles [1], has given us to debunk once and for all the “scientific” publications on Sickle Cell Trait that lack probity.

SCIENTISTS PERSIST WITH SICKLE CELL TRAIT MISINFORMATION

It is not clear how many of the following examples constitute Disinformation, that is Deliberate Misinformation, but students, doctors, and Science Correspondents of major International Newspapers, Radio Outlets, and Television reporting for the Media, and even GOOGLE and WIKIPEDIA need to look up every single published reference in the following examples so as to be aware of what has happened:
1. Sickle Cell Trait blamed for dying suddenly while exercising [69]
2. Broken bones in child from baby bashing put to Sickle Cell Trait [70]
3. Aggressive renal cancer “seen almost exclusively in young patients with sickle cell trait” [71 Elliott Vichinsky et al.]
4. “Complications associated with sickle cell trait” Tsaras et al [72] Flawed article!
5. Fifteen-year-old black girl sterilized because of sickle cell trait [24]
6. Black prisoners beaten to death in jail – Autopsy verdict “Sickle Cell Trait” [73]
7. “Fatal pulmonary infarction in sickle cell trait”. No electrophoresis done. [74].
8. Sickle Cell Disease (2 beta-globin gene variants) described with Sickle Cell Trait mentioned in the title of article [75]
9. Beware of symptomatic Sickle Cell Traits [76]
10. Flight from Kumasi to Accra (45 minutes) produces intestinal infarction in Sickle Cell Trait [77 to 86]
11. Sickle Cell Traits have their insurance loaded 150% [87]]
12. Pilot with Rhabdomyolysis from Sickle Cell Trait – no laboratory test [88]

Well did I object “Dangerously flawed diagnosis of sickle cell trait in compartment syndrome rhabdomyolysis” [89] and “Blaming sudden death on Sickle Cell Trait”? [90] For other references on Sickle Cell Trait Misinformation see References 91 to 94.

IF LAW MAKERS GET IT WRONG, CAN GENETIC COUNSELLING BE DONE?

Who guides American Insurance Companies when a Massachusetts Law has such a statement as “the disease known as sickle cell trait?”.[95]. There really is no excuse for such scientific obfuscation when there are established agreed definitions in Haemoglobinopathy [96 97]. And Sickle Cell Trait continues wrongly to be written “SCT” by Noke [64] and the NIH [65] where National Foundation/March of Dimes of the USA invited me twice to do Grand Rounds in the days of Professor Rudy Jackson.

The only correct phenotype designation for Sickle Cell Trait is “AS”, never “SCT” – a confusion that Insurance Companies use to defraud people because “SC” is the known designation for Sickle Cell Haemoglobin C Disease. The Martin Luther King Jr Foundation protected me with 4 bodyguards in Philadelphia for my Keynote Address “Vital Difference Between Sickle Cell Trait and Sickle Cell Disease”.[98].

THE GREAT RESPONSIBILITY OF INTERNATIONAL MEDICAL AND SCIENTIFIC JOURNALS: A SERIOUS APPEAL TO THE LANCET!

I appeal to the Editors of Lancet to pursue the authors Rhida A, Khan A, Al-Abayechi S, Puthenveetil V who published this: “Acute compartment syndrome secondary to rhabdomyolysis in a sickle cell trait patient” Lancet 2014; 384:2172 [88] without a shred of evidence for Sickle Cell Trait presented, and this resulted in the article going viral on the Internet, frightening people on Facebook when Whiskey Delta Charlie wrote this (QUOTE):

“Here is what’s possible if you are a carrier of Sickle Cell Trait Sept. 224:21pm2016 #sicklecellawarenessmonth #sicklecelltrait #exertion #Rhabdomyolysis http://dhmi.org/sickle-cell-trait-story/ September is Sickle Cell Awareness Month. Awareness to Sickle Cell Trait the gene responsible for creating Warriors who live with Sickle Cell” (UNQUOTE) plus illustrated ghastly pictures of rotting muscles of Rhabdomyolysis [99].

LOSS OF JOBS FROM SICKLE CELL TRAIT MISINFORMATION

How many pilots might have had their job threatened Corona Virus or no Corona Virus by this flawed Lancet article is not known, but what is known is that previous Editors of Lancet pursued authors of an article supposedly proving that homosexuals and some central Africans shared a common gene Gc1f [100] the flawed Science of which I was the first to criticize as “leaving a lot to be desired” [101] until the authors withdrew it from publication with the confession “Erroneous data”. [102].

So why can’t the present Editors of Lancet pursue the authors of the “Sickle Cell Trait Rhabdomyolysis Pilot” article [88], ask them to produce the alleged “Sickle Cell Trait” pilot so we know exactly what happened? Are we likely to expect another “Erroneous Data” confession in the Lancet? [102]

Researchers that need to read about Sickle Cell Trait Misinformation and Disinformation are referred to the huge number of references on the subject [91-95 103].

LORD SOLLY ZUCKERMAN AND PRIDE AND PREJUDICE IN SCIENCE

It was not Black People who dared to mention Prejudice in relation to Science. To imagine that Genome Sequencing, for example, has the answer to everything including Genetic Counselling fails to take into consideration the human element in Science. During my SPECIAL LECTURE dedicated to Centenarian Dr Emmanuel Evans-Anfom [2] I drew attention to the remarkable article of Professor Sir (later Lord) Solly Zuckerman where he proved conclusively that there was much Prejudice and Pride in Science.[104], and I went on to mention the widespread declaration of Nobel Laureate Professor James Watson that the current Global Sequencing exercise would soon reveal that Africans had inferior intelligence. [105] He subjected himself to Genome Sequencing only to find that he himself was African [105], and I could hardly resist the observation on 4th December 2009 that “Watson, still alive today in USA was brutally reminded not only that there is but one human race, but also that African genes contributed to the Nobel Prize.in ‘Medicine or Physiology’ which he jointly won with Francis Crick and Maurice Wilkins on the DNA Double Helix in 1962”.[106] His ancestor was in Africa same time as my great-grand father Konotey-Adade? [107] There is, indeed, but one human race. [106]

GENETIC COUNSELLING IN THE AFRICAN MILIEU

In the African milieu Genetic Counselling has been done more from laboratory tests, and we must not rely on Genome Sequencing results from experts like Professor James Watson who know what they will find before they do the examination. Simple Haemoglobin Electrophoresis methods (Alkaline and Acid media) plus clinical acumen have helped us quantify our 1 in 3 genetic incidence and to describe new haemoglobins like Haemoglobin Korle Bu [108], Haemoglobin Osu-Christiansborg [109], and discover an adult African, a Nungua fisherman, with only foetal haemoglobin in his body [110], and also tell the world of a No-Enzyme-at-all G6PD Deficient man in Ghana. [33 34]. The colour test is unable to distinguish Sickle Cell Disease “SC” Phenotype from Sickle Cell Trait “AS”. [111]. Terms like “Up-to-date” [71 (Vichinsky)] and “Novel Clinical Significance” [69 (Key and Vimal)] regarding Sickle Cell Trait are more reflection of imperfect knowledge than, as we are urged to think, discovery of new knowledge. Like “SCT” written for Sickle Cell Trait, they are fraudulent Insurance Companies’ delight.

CORONA VIRUS: SIGNIFICANCE FOR WHITES WITH SICKLE CELL TRAIT AND G6PD DEFICIENCY

1. One often heard “Sickle Cell Disease – the hereditary disease of Blacks”. The past week found 1872 Turks POSITVE for Coronavirus. We must expect about 300 of them to be Sickle Cell Trait phenotype because Aksoy found 15% Turks to be Sickling POSITIVE. Indeed, 18% of Eti-Turks in southern Turkey have Sickle Cell Trait. [112], Choremis et al reported a high 30% Sickle Cell Trait incidence in Greece where Lake Kopais once was [113]. With 650 reported cases of POSITIVE COVID-19 just as one island was flooded with Greeks returning home, would it surprise anyone if about 150 of these white-skinned Greeks were Sickle Cell Trait “AS”. Thousands of relatives worldwide are Sickle Cell Trait “AS”. Like Ghana [114], there is also a high incidence of G6PD Deficiency in Turks, Greeks, Italians in the Mediterranean Region as a whole [ 30 42].

2. Reports from China claim that Vitamin C in large doses intravenously has had a remarkably beneficial effect on patients. Linus Pauling who won Nobel Prize for discovering the molecular structure of Sickle Haemoglobin S [115] popularised taking large doses of Vitamin C [116] for the common cold, and for cancer 117], a recommendation that was criticized because he was “just a PhD, not an MD doctor”.
A medically qualified doctor, Physician Dr Abram Hoiffer MD PhD FRCP (C) has given Linus Pauling’s Vitamin C anti-cancer claim respectability. [117] Professor Linus Pauling himself was present and listened approvingly when I gave the Keynote Address “THE VITAL DIFFERENCE BETWEEN SICKLE CELL TRAIT AND SICKLE CELL DISEASE” at the Dr Martin Luther King Jr Foundation Award Dinner in Philadelphia [98] I shook hands with the only person who got two (un-shared-with-anybody-else) Nobel Prizes. He did not seem to be a person who would have talked about Vitamin C through his hat. And, indeed, Vitamin C has been proven to kill tumour cells with hard-to-treat mutation [117].

But what has all this got to do with SICKLE CELL TRAIT in White people? ANSWER: Doctors in Europe who may follow Chinese intravenous Vitamin C therapy for Corona Virus must first find out which of their patients has G6PD Deficiency because such large doses of Vitamin C given intravenously can cause catastrophic intravenous haemolysis [41]. Ghanaian doctors who may be adopting Vitamin C intravenous therapy are advised to check Glucose 6 phosphate Dehydrogenase enzyme level before proceeding. The New York Hospital that has started using i.v Vitamin C [118] will do well to take my advice because one undetected G6PD Deficient patient who collapses on Vitamin C drip will have the Food and Drug Administration come down on them heavily to stop what in my not-so-humble opinion promises to prove once again that Linus Pauling was far from being naïve. If the Chinese wondered why some who received intravenous Vitamin C recovered, while others on the same regime bled from every orifice, I suggest they henceforth do Quantitative G6PD estimation on every patient before treatment is commenced. Greek and Turkish Sickle Cell Traits with G6PD Deficiency, if given large doses of Vitamin C would be misdiagnosed as suffering from Sickle Cell Anaemia “SS” in haemolytic crisis when, in fact, they were Sickle Traits “AS” with anaemia. [24] The G6PD enzyme level of Dr Emmanuel Evans-Anfom is NORMAL.

I very much trust our British-trained Ghanaian Physicians to screen all admissions (as we used to do half century ago on Medical Floor Two Korle Bu Teaching Hospital) for G6PD Deficiency and administer to those found POSITIVE for COVID-19, immediately, 1,500 mg of intravenous Vitamin C. This is the advice of someone (myself) who shook hands with the Genius “Sickle Cell Linus Pauling” on Wednesday 31st May 1972 in Philadelphia. Take meticulous notes, and publish weekly progress promptly.

3. Africans receiving news from China that Blood Groups reacted differently regarding proneness/non-proneness to the present COVID-19 infection, namely that those with Blood Group A fared worse than O patients in their response to treatment, may confuse (as they have often done) “Blood Group A” with “Haemoglobin Type A” and draw wrong conclusions about how Haemoglobin variants (Hb “S” included) can dictate prognosis in COVID-19 illness We must be very careful about News Headlines when it comes to epidemiology.

SCIENCE NEWS HEADLINES CAN CAMOUFLAGE PREJUDICE

For example, after the “science” that homosexuals and some central Africans shared a common gene Gc1f had in Lancet on Saturday 2nd May 1987 [100] the BBC broadcast the information in its “SCIENCE IN ACTION” [119] the very next day 3rd May GMT 09.15. My objections to Lancet followed just days later Lancet [101]. But when the authors confessed their “Erroneous data” [102] some of us were waiting also for BBC to confess “ERRONEOUS SCIENCE IN ACTION.” We wondered why BBC WORLD SERVICE or the BBC HOME SERVICE Programmes did not carry Lancet’s withdrawal of the erroneous findings? Was this an example of BBC reflecting Lord Zuckerman’s diagnosis of “Pride and Prejudice” [104] in ‘Science In Action’? [119]

TWO FINAL CAUTIONARY PIECES OF ADVICE TO REMOVE CONFUSION

1. NEVER use SCT to stand for Sickle Cell Trait. It can be confused with “AS”; the “A” standing for normal Haemoglobin gene and “S” for abnormal sickle cell Haemoglobin, with “S” always less than 40% in the best laboratories. Facebook showed a man calling himself “Sickle Cell Trait” groaning from sickle cell crisis pain. When I commented “Ask your Doctor to do Haemoglobin Electrophoresis on you” he returned the next week to say he was “SC”, which is never Trait because “SC” is two Abnormal genes. Traits always have one normal beta-globin gene “A” plus one abnormal beta-globin gene. That is why I use the term “NORMACHE” for Traits, specifying what abnormal Haemoglobin gene the “ACHE” stands for. [See my “Competing Interest” below].

2. AVOID the term “Heterozygote” which just means 2 different genes. Sickle Cell Trait “AS” is Heterozygote, but so also is “SC” which, to avoid calling a disease “Trait”, is correctly designated as “Double Heterozygote”. [23 24]

THANK YOU VERY MUCH, CENTENARIAN DR EMMANUEL EVANS-ANFOM!

Let us thank Dr Emmanuel Evans-Anfom for enabling us to dismiss once and for all the fears of Professor James Bowman MD PhD, University of Illinois Professor of Medicine, Genetics, and Haematology when he lamented that “Persons with sickle cell trait will no longer be able to become ill or even die lest they find themselves subject of a case report” [120]

Competing Interest: I am the offspring of NORMACHE x NORMACHE Abnormal Haemoglobin parents (AS x AC) who had 11 children 3 of whom had sickle cell disease ACHEACHE (SC), 4 had Trait NORMACHE (2 AC & 2 AS) and 4 of us took no aching genes from our parents to be NORMNORM “AA” phenotype. Impossible it is to tell NORMACHE Traits from those of us who had no Hb gene variant except through blood test. Sickle cell disease phenotypes suffer hereditary cold-season Rheumatism – Tribal names Hemkom or Chwechweechwe – and are therefore physically identifiable. [24]

felix@konotey-ahulu.com Twitter @profkonoteyahul

Felix I D Konotey-Ahulu FGA MB BS MD(Lond) DSc(UCC) DSc (UH) 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

Website www.sicklecell.md or www.konotey-ahulu.com

1. Evans-Anfom E. To The Thirsty Land – Autobiography of A Patriot. Africa Christian Press Accra, Ghana 2003 ISBN: 9964-87-717-X

2. Konotey-Ahulu FID. HISTORY SURPASSES SCIENCE. Ghana Academy of Arts and Sciences 30th January 2020 SPECIAL LECTURE dedicated to Dr Emmanuel Evans-Anfom, Centenarian https://youtu.be/hV8NZJh1pg Video.

3. Edington GM. Sickle cell anaemia in the Accra district of the Gold Coast. Br Med J 1953;2:957 https://doi,org/10.1136/bmj.2.4843.957 October 31 1953

4. Lehmann H. The distribution of the sickle cell trait. J Clin Path 1953; 6: 329.

5. Edington GM. Sickle cell trait and sickle cell anaemia. BMJ 1954.1: 871

6. Edington GM, Lehmann H. A case of sickle cell haemoglobin C disease and survey of haemoglobin C incidence in West Africa. Trans Roy Soc Trop Med Hyg 1954; 48: 332-336.

7. Edington GM, Lehmann H. Expression of the sickle cell gene in Africa. BMJ 1955; 1: 1308-1311.

8. Edington GM, Lehmann H. Expression of the sickle cell gene in Africa. BMJ 1955; 2: 1328.

9. Edington GM and Lehmann H. The distribution of Haemoglobin C in West Africa. Man 1956; 34-36.

10. Edington GM, Laing WN. Relationship between Haemoglobin C and S and malaria in Ghana. BMJ 1957; 2: 143-1456.

11. Lehmann H, Nwokolo C. The River Niger as a barrier in the spread eastwards of Haemoglobin C. A survey of haemoglobins in the Ibo. Nature 1959; 183: 1587-1588.

12. Thompson GR. of Haemoglobins S, & C in Ghana BMJ 1962; 1: 682-685.

13. Thompson GR, Lehmann H. Combinations of high levels of Haemoglobin F with A, S and C in Ghana. BMJ 1962; 1:1521-1523.

14. Thompson GR. Malaria and stress in relation to Haemoglobin S and C. BMJ 1963; 2: 976-978.

15. Edington GM. Abnormal Haemoglobins in West Africa. Ghana Medical J 1963; 2: 83-87.

16. Edington GM, Watson-Williams EJ. Sickling, Haemoglobin C, Glucose-6 Phosphate Dehydrogenase Deficiency and malaria in Western Nigeria. In Abnormal Haemoglobins. Editors Jonxis JHP and Delafresnaye JF. Blackwell Scientific Publications, Oxford 1965, pages 393-401.

17. Lehmann H. Hemoglobinopathies. Abnormal Hemoglobins and Thalassaemias. In Health Problems in Developing States. Editors Prywe SM, Davies A. Grune and Stratton Inc., 1968, pp158-167.

18. Ringelhann B, Dodu SRA, Konotey-Ahulu FID, Lehmann H. A Survey for Haemoglobin Variants, Thalassaemia and Glucose-6-Phosphate Dehydrogenase Deficiency in Northern Ghana. Ringelhann B, Dodu SRA, Konotey-Ahulu FID, Lehmann H. Ghana Med J 1968; 7: 120-124. Sept. 1968.

19. Konotey-Ahulu FID. EDITORIAL Hereditary Qualitative and Quantitative Erythrocyte Defects in Ghana: An Historical and Geographical Survey. Ghana Med J 7: 118-119. Sept. 1968

20 Konotey-Ahulu FID and Ringelhann B. Sickle-cell anaemia, sickle-cell thalassaemia, sickle-cell haemoglobin C disease and asymptomatic haemoglobin C thalassaemia in one Ghanaian family. BMJ 1969 Mar 8; 1(5644): 607-612. doi:10.1 http://www.bmj.com/cgi/reprint/1/5644/607.pdf doi:10.1136/bmj-1.5644/607 March 8 1969.

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

22. Ringelhann B, Konotey-Ahulu FID, Yawson G, Bruce-Tagoe AA, Miller A and Huisman THJ. Alpha Thalassaemia in West Africa. Symposium on Medical Genetics 1969, Hungary, page 81.

23. Konotey-Ahulu FID. Pattern of Sickle Cell Disease in Accra – A Study of 1,550 Consecutive Patients: A Thesis Presented 1971 for The Postgraduate Degree Of Medicine (MD) In The University Of London. [Awarded in 1972]

24. Konotey-Ahulu FID. The sickle cell disease patient: natural history from a clinico-epidemiological study of 1550 patients of Korle Bu Hospital Sickle Cell Clinic. Tetteh- A’Domeno [TA’D] Co 1996; Macmillan Education 1992.

25. Konotey-Ahulu FID. History of Sickle Cell Disease in Africa. Geographical Distribution and Population Dynamics of Haemoglobins S and C with special reference to West Africa. Ghana Med J 1972; 11: 397-412.

26. Lehmann H, Huntsman RG. Man’s Haemoglobins. North-Holland Publishing Company 1974. Amsterdam

27. Gbedemah KA, Acquaye CTA, Konotey-Ahulu FID and Reindorf CA. Haemoglobin phenotype patterns in more than 1,000 consecutive new-born babies in Ghana. Ghana Med J 1976; 15: 253-256

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

29. 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.

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

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

32. Konotey-Ahulu FID. Sickle Cell Disease as an International Problem. Annals Of The Research Institute Of Health Sciences (Annales De L’Institut De Researche En Sciences De La Sante) 1997; Volume 1 No. 1, pp 1 to 10

33. Owusu SK. Glucose-6-phosphate dehydrogenase (G-6PD) deficiency in the causation of disease in Ghana. Ghana Med J 1974; 13: 168-170.

34. Owusu SK, Opare-Mante A. Electrophoretic characterization of glucose-6-phosphate dehydrogenase in Ghana. Lancet 1972; 11: 44.

35. Owusu SK, Foli AK, Konotey-Ahulu FID, Janosi M. Frequency of Glucose-6-phosphate dehydrogenase deficiency in typhoid fever in Ghana. Lancet 1972; 1: 320.

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

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

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

39. Owusu SK. Clinical manifestations of glucose-6-phosphate dehydrogenase (G-6PD) deficiency in Ghana. Ghana Med J 1978; 17: 235-239.

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

41 Konotey-Ahulu FID. G6PD Deficiency in Ghanaians. How to recognise it. Or Twenty different ways G6PD Deficiency presents in Ghanaians Jan 2008 http://bit.ly/2sukoPW

42.Luzzatto Lucio. G6PD Deficiency frequency and sickle cell anaemia association on the African continent. INSERN 1975; 44: 229.

43. Scott Ian A, Attia John, Moynihan Ray. Promises and perils of using genetic tests to predict risk of disease. BMJ 2020; 368:m14 Feb. 22, p 285.

44.. Khan Naz, Communities that prefer close blood marriages need more help to access genetic services. BMJ 2020; 368 BMJ OPINION Feb. 15 page 243.Easton DF

45. Nance Walter E. Genetic control of haemoglobin synthesis. Science 1963; 141: 123-130.

46. Nance Walter E, Grove J. Genetic determination of phenotypic variation in sickle cell trait. Science 1972; 177: 116-118.

47. Brittenham G, Lozoff B, Harris JW, Barker J, Nayudu MV. Alpha globin gene number: population and restriction endonuclease studies. Blood 1980; 55: 706.

48. Lehmann H, Carrell RW. Nomenclature of the Alpha-Thalassaemias. Lancet 1984; 11: 552.

49. Konotey-Ahulu FID. Missing the wood for one genetic tree? In The First International Symposium on the Role of Recombinant DNA in Genetics. Chania, Greece, May 13-16, 1985, pages 105 to 116.

50.. Konotey-Ahulu FID. Alpha-Thalassaemia nomenclature and abnormal Haemoglobins. Lancet 1984; 1: 1024-25. May 5 [“Of 82 consecutive Sickle Cell Traits seen in London in 24 months 36 (44%) had just one quarter of the total Haemoglobin as Sickle Haemoglobin (Mean 25%, Range 20-28”). The three known peaks od Haemoglobin S proportion in the West African Sickle Cell Trait are around 25%, around 30.

51.Lehmann H, Kynoch Pamela A M. Human Haemoglobin variants and their characteristics. North Holland Publication Company. Amsterdam – New York – Oxford 1976; [Elsevier North-Holland Biomedical Press] ISBN 07204 0585 8

52. Serjeant GR. Sickle Cell Disease. Oxford University Press, Oxford 1992. 53. Weatherall DJ, Clegg JB. The Thalassaemia Syndromes. Blackwell Scientific, Oxford 2008.

54. Konotey-Ahulu FID. Need for ethnic experts to tackle genetic public health. Lancet 2007; 370: 1826 [doi:10.1016/50140-6736(07)61771-1] www.thelancet.com/journals/lancet/article/PIIS0140-6736(07)61771-1/fulltext

55. Allotey P. Ethnicity and access to health care. Lancet 2007; 370: 475-476

56. Hooper JL, Bishop DT. Population based-based family studies in genetic epidemiology. Lancet 2005; 366: 1397-1406.

57.Konotey-Ahulu FID. Haenoglobinopathy: The Genetics that touches you and me. http://blog.sicklecell.md/sicklecell/sickle-cell-and-allied-haemoglobinopathy-the-genetics-that-touches-you-and-me/ University of Cape Coast Golden Jubilee Message 2012] Konotey-Ahulu FID. Maintenance of high sickling rate in Africa: Role of polygamy. J Trop Med Hyg 1970 Jan; 73(1): 19-21 (38 References). [Traits who voluntarily restrict number of children they produce do diminish burden of abnormal haemoglobin disease (SS SC CC Sbeta-Thalassaemia Cbeta-Thalassaemia Thalassaemia Major SF CF Fbeta-Thalassaemia SKorle Bu SOsu-Christiansborg in the Ghanaian population. My MPSI shows that Males need more to heed this message for Voluntary Family Size Limitation].

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

59. Konotey-Ahulu FID. Maintenance of high sickling rate in Africa: Role of polygamy. J Trop Med Hyg 1970 Jan; 73(1): 19-21 (38 References). [Traits who voluntarily restrict number of children they produce do diminish burden of abnormal haemoglobin disease (SS SC CC Sbeta-Thalassaemia Cbeta-Thalassaemia Thalassaemia Major SF CF Fbeta-Thalassaemia SKorle Bu SOsu-Christiansborg in the Ghanaian population. My MPSI shows that Males need more to heed this message for Voluntary Family Size Limitation].

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

61. Konotey-Ahulu FID. Male procreative superiority index (MPSI): The missing co-efficient in African anthropogenetics. BMJ 1980; 291: 170

62. Konotey-Ahulu FID. Survey of sickle-cell disease in England and Wales. BMJ 1982; 284(6309): 112. doi:10.1136/bmj.284/6309/112-a (Jan. 9 1982) http://www.bmj.com/cgi/reprint/284/6309/112-a.pdf7.

63. Human Genetics Commission (HGC-UK) and direct consumer Genetic Tests, leading to Genetic Counselling. BMJ Rapid Response May 27 2009. http://www.bmj.com/cgi/eletters/338/may15_2/b1995#214r56

64. Noke Melissa, Peters Sarah, Uiph Fiona. A qualitative study to explore how professionals in the United Kingdom make decisions to test children for a sickle cell carrier state. Europ Journal of Human Gentics 2016, 24: 164-170. 74. Noke Melissa, Peters Sarah, Ulph Fiona. A qualitative study to explore how professionals in the United Kingdom make decisions to test children for a sickle cell carrier state. Europ. Journal of Human Genetics 2016; 24:164-170. doi:10.1038/ejhg.2015.104 [Terms like “SC prevalence” and “not so benign nature of ‘SCT’” reveal ignorance of approved Terminology.

65. NIH (Bethesda) “ Sickle Cell Disease is the most common inherited blood disorder in the USA …” http://www.ghr.nlm.gov/condition/sickle-cell-disease [But as Sickle Cell Trait is written SCT when SC is known to be disease phenotype what interpretation do people in the USA put on the recently widely advertised NIH home-use-kit for checking for the gene? Especially when the commercial Haemoglobin S Test-Tube Colour-Test does not differentiate between Sickle Cell Trait “AS” (1 Normal gene + 1 Abnormal gene) and Sickle Cell Disease “SC” phenotype (2 Abnormal genes)?

66. Konotey-Ahulu FID. Genetic Testing and Counselling Towards Genetic Public Health http://www.bmj.com/contrent/358/bmj.j4101/rapid-responses BMJ Rapid Response 21 December 2017 to Semsarian C and Ingles J. PRACTICE [See below]

67. Semsarian C., Ingles J. A clinical approach to genetic testing for non-specialists. BMJ 2017; 358 doi: 10.1136/bmj.j4101. 28 Sept. 2017.

68. Streetly A. A common definition of genetic testing, can we agree on one? 18 October 2017 http://www.bmj.com/content/358/bmj.j4101/rapid-responses .

69. Key Nigel S, Derebail Vimal K. Sickle Cell Trait: Novel Clinical Significance. Hematology 2010: 418-422. “During exercise, Sickle Cell Trait appears to be a risk factor for sudden death and/rhabdomyolysis, particularly when the exercise is intense, and is performed at high altitude”

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

71.. Vichinsky Elliott P. Sickle cell trait. Literature Review UpToDate [Accessed 18 Feb 2011] http://www.uptodate.com/contents/sickle-cell-trait?view=print asserts falsely that “Renal medullary carcinoma is a rare and aggressive tumor that is seen almost exclusively in young patients with sickle cell trait.”

72. Tsaras, G, Owusu-Ansah A, Boateng FO, Amoateng-Adjepong, Y. Complications associated with sickle cell trait: a brief narrative review. American Journal of Medicine 2009; 122(6): 507-512. [The Ghanaian co-authors appear ignorant of what work had been done in Ghana (6 million Sickle Cell Traits) since 1953; See References 3 – 32]

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

74. Malhotra Vinod, Ravi Prakash, Yeun Sook Choi, Bernard Chomet, Clifford G Pilz. Fatal Pulmonary Infarction in a Patient with Sickle Cell Trait. CHEST 1973; 64: 524-26. October 1973. “It is suggested that sickle cell trait should be considered in all Negro patients who present with suspected vaso-occlusive episode”. [Flaws in article: (a) How about the millions of white Greeks and Turks with Sickle Cell Trait? (b) Have the authors excluded Sickle Cell beta-Thalassaemia after quantitative electrophoresis? (c)
Is sickle cell haemoglobin C being referred to as Sickle Cell Trait. (d) Fatal pulmonary infarction occurs in Caucasian women on oral contraceptives. If a “Negro patient” was Sickle Cell Trait “AS”, and had been on contraceptives, why would she be prevented from having pulmonary infarction when white British women were also afflicted?]

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

76. Konotey-Ahulu FID. World Sickle Cell Day 19h June 2014 http://bit.ly/1FuNXPi Beware of symptomatic sickle cell traits. Lancet, Feb 29, 1992, page 555. http://www.thelancet.com/journals/lancet/article/PII0140-6736(92)90377-F/fulltext

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

78. Addae R O. Sickle cell trait and altitude. BMJ 1972; 1: 53. [10 criteria (Addae’s Criteria) required to satisfy clinicians in regions where 1 in 5 people have the sickle cell trait that symptoms are due to the trait and nothing else.]

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

80. Konotey-Ahulu FID. Sickle cell trait and altitude. BMJ 1972; 1: 177-178.

81. Konotey-Ahulu FID. An international sickle cell crisis. [Editorial] Ghana Medical J; 1972; 11: 4-8 [A detailed account of how BMJ withdrew report]

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

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

84. Lehmann Hermann. Sickle cell and flying. The Times (London) 4 Jan 1972 That was when the “Science Editor” used false report on “Sickle Cell Trait and Flying” in BMJ to recommend grounding of all Black Air Crew.

85. Konotey-Ahulu FID. Aviation Safety, Ghanaians recall media disinformation deriving from scientific misinformation. BMJ Rapid Response March 1 2019; https://www.bmj.com/content/364/bmj.l735/rr-7 to Partha Kar: Applying aviation safety to healthcare – are we missing the fundamentals? Partha Kar 364:/doi10.1136/bmj.l735 :

86. Konotey-Ahulu FID. Lt-Col [Retd] Dr Frank F T Djabanor (1938 – 2019) Physician whose exposure of scientific falsehood saved Black People from humiliation. TRIBUTE. Accra & Odumase-Krobo 31st March 2019.

87. Konotey-Ahulu FID. Insurance and genetic testing. Lancet 1993, 341: 833. March 27 [See Reference 98 for when Dr Konotey-Ahulu was given 4 Body Guards in Philadelphia for stressing “Sickle Cell Disease is NOT Sickle Cell Trait and vice versa!” and thus upsetting Insurance Companies]

88. 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 [No evidence for Sickle Cell Trait was presented in this Lancet article].

89. Konotey-Ahulu FID. Dangerously flawed diagnosis of sickle cell trait in compartment syndrome rhabdomyolysis http://bit.ly/2d4t9Zd http://www.sicklecell.md/blog/index.php/2016/09/dangerously-flawed-diagnosis-of-sickle-cell-trait-in-compartment-syndrome-rhabdomyolysis-article/ [No evidence for Sickle Cell Trait was presented in the Lancet article (Reference 88)].

90.. Konotey-Ahulu FID. Blaming sudden death on Sickle Cell Trait? Flaws in article of Charis Kepron, Gino Somers and Michael Pollanen [Reference 37 above Exposed]. September 4 2011 www.sicklecell.md/blog/?p=105 or www.konotey-ahulu.com/blog/?p=105

91.Konotey-Ahulu FID. Sickle Cell Trait Misinformation and Disinformation. https://blog/sicklecell.md/sicklecell/sickle-cell-trait-misinformation-and-disinformation/ November 30 2011

92 Konotey-Ahulu FID. Further Communication on “Sickle Cell Trait Misinformation and Disinformation” and Sickle Cell Terminology: Disease or Disorder? April 6 2012 https://blog/sicklecell.md/sicklecell/further-communication-on-sickle-cell-trait-misinformation-and-disinformation-and-sickle-cell-terminology-disease-or-disorder/

93. Konotey-Ahulu FID. Sickle Cell Trait Confusion: Is it Deliberate? Or is this Ignorance? August 11 2017 https://blog.sicklecell.md/sicklecell/sickle-cell-trait-confusion-is-it-deliberate-or-is-this-ignorance/

94 Konotey-Ahulu FID. Sickle Cell Trait: As with statins when leading editors disagree please give principles same weight as details 20 September 2016 .http://bit.ly/2dy5fUJ/http://www.sicklecell.md/blog/index.php/2016/09/statins-when-leading-editors-disagree-please-give-principles-same-weight-as-details/

95. Beutler E, Boggs DR, Heller P, Maurer A, Motulsky AG, Sheehy TW. Hazards of indiscriminate screening for sickling. N Engl J Med. 1971 Dec 23;285(26):1485–1486 www.europepmc.org/articles/PMC178610 [Authors have commented on a Massachusetts Law which stated in part that “Every child, which the Commissioner of Public Health, by rule Law which stated in part that “Every child, which the Commissioner of Public Health, by rule or regulation, may determine is susceptible to the disease known as sickle cell trait or sickle cell anemia, shall be required to have a blood test”. [How on earth does one explain to the Americans that there is no such thing as “the disease known as sickle cell trait”? Making my African NORMACHE equal to ACHEACHE?]

96. Woodruff AW et al. Terminology of the Hereditary Haemoglobinopathies with haemoglobin variants. BMJ 1957; 1: 1235. .

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

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

99. Whiskey Delta Charlie on Facebook 24 September 2016 If you are a Carrier of Sickle Cell Trait Here is what’s possible: #sicklecellawarenessmonth#sicklecelltrait #exertion #Rhabdomyolysis http://dhmi.org/sickle-cell-trait-story/ September is Sickle Cell Awareness Month. Awareness to Sickle Cell Trait the gene responsible for creating Warriors who live with Sickle Cell” (UNQUOTE) plus illustrated ghastly pictures of rotting muscles of Rhabdomyolysis.

100. Eales L-J, Nye KE, Parkin JM, Weber JN, Forster SM, Harris JRW, Pinching AJ. Association of different allelic forms of group specific component with susceptibility to and clinical manifestation of human immunodeficiency virus infection. Lancet 1987; 1: 999-1002.

101. Konotey-Ahulu FID. Group specific component and HIV infection. Lancet; 1: 1267.

102. Eales NJ, Nye KE, Pinching AJ. Group specific component and AIDS: Erroneous data. Lancet 1988; 1: 936.

103. Konotey-Ahulu FID. The Sickle Cell Disease Patient Website www.sicklecell.md

104. Zuckerman Sir Solly. Pride and Prejudice in Science. Aerospace Medicine 1974; 45: 638-347 (Also re-published with permission in Ghana Medical Journal 1975; 14 (No.1).52-60.105.

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

106.. Konotey-Ahulu FID. There is but one human race. New African, London. December 2009, page 4. [Great-grandfather of Nobel Laureate Professor James Watson would have been on the African Continent exactly the same time as my own great-grand father Konotey-Adade born in 1820 (Generation V going back to 1670) [107]. Could the ancestor of James Watson have suffered enforced migration across The Atlantic? Born in 1928, if he is still alive at 92 years of age, would he kindly oblige to have his Haemoglobin Electrophoresis done to see whether he has a 1 in 3 chance of being (like my father and mother) phenotype NORMACHE “AS” or “AC”?

107. Konotey-Ahulu FID. Sickle Cell Disease in successive Ghanaian Generations for Three Centuries (Manya Krobo Tribe). References [23 and 24, pages 6 to 20]

108. Konotey-Ahulu FID., Gallo E, Lehmann H, Ringelhann B. Haemoglobin Korle Bu (alpha2 beta2 73 Aspartic Acid –> Asparagine), showing one of the two amino acid substitutions of Haemoglobin C Harlem. Konotey-Ahulu FID., Gallo E, Lehmann H, Ringelhann B. Haemoglobin Korle Bu (alpha2 beta2 73 Aspartic Acid –> Asparagine), showing one of the two amino acid substitutions of Haemoglobin C Harlem. http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=1468514 http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=1468514

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

110. Kamazura H, Ringelhann B, Konotey-Ahulu FID, Lehmann H, Lorkin PA. The gamma chain in a Ghanaian adult homozygous for hereditary persistence of Fetal Haemoglobin. Acta Hematologica; 51: 197-184. .

111. Konotey-Ahulu FID. Detecting Sickle Haemoglobin. BMJ 1972; 2: 239 Nov 11; 4(5836) 376 See a Massachusetts law quoted below by Dr Beutler et al. Ref. [95]

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

113. Choremis et al.. Blood Groups of a Greek community with a high sickling frequency. Lancet 1957; 2: 1333-1334.

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

115. Pauling Linus et al. Sickle Cell Anemia, a molecular disease. Science1949; 110: 543-8.

116.. Pauling Linus. Vitamin C and the Common Cold. Linus Pauling Institute Oregon State University. 1970.

117. Hoffer Abram, Pauling Linus. Vitamin C and Cancer: Discovery, Recovery, Controversy. 1979. [ISBN-10 1550820788 & ISBN-13 978-1550820782]

118. Mongelli Lorenn. New York Hospitals are treating coronavirus patients with Vitamin C. New York Post March 24 2020 https://nypost.com/2020/03/24/new-york-hospitalsreatingcoronaviruspatientswithvitaminc/?fbclid=IwAR0rO7trfM0j4GE53xuAnclKreZOtCp72X9qBpDT4fBpXb036t0qorg..

119. BBC SCIENCE IN ACTION Sunday 3rd May 1987 Dr Anthony Pinching interviewed about article in Lancet on Saturday 2nd May explaining why AIDS virus was spreading faster Central Africa than in the USA and how a gene Gc1f was common to homosexuals and Central Africans. The findings were found to be false, and withdrawn from publication, but BBC kept quiet about the “Erroneous Data”.

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

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.

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

Male Procreative Superiority Index (MPSI)

Male Procreative Superiority Index (MPSI): 500 children born to just 17 sperm donors

The mathematical Genetic Index that I invented more than 30 years ago “Male Procreative Superiority Index (MPSI)” [British Medical Journal 1980 Volume 281 (6256) pages 1700 to 1702] was meant to explain not only the very high Sickle Cell Trait frequency in certain populations through Polygamy, but also to indicate male superior contribution to what the Daily Telegraph Science Editor (May 7 Front page & page 7) called other “defective genes” in her article “500 children born to just 17 sperm donors”.

But in another article in Journal of Genetic Disorders & Genetic Reports May 13 2014 “History Versus Limits of Science: Is Solomonic Genius a Y Chromosome Phenomenon?” I went further to show that since 1901 Nobel Prizes have been awarded to more than 800 individuals of whom at least 180 (22%) have been recipients of genes from King Solomon – wisest man on earth – who “had seven hundred wives, princesses, and three hundred concubines” [1Kings chapter 11 verse 3]. His MPSI was enormous. The fact that Jewish women’s Nobel Prize winners is a whopping 38% among the world’s females is, I said, “not that solomonic genius resides in the Y chromosome, but that it allows many more offspring than the female”.

Talk these days of Gender Equality is misplaced. Nine seconds and the male’s contribution to baby formation is complete. Females require 9 months! Any reader of this post on Facebook or Linked-In can, by using known relatives like male-female adult twins, prove conclusively that the male twin has in many cases and in any society more children than his sister, thanks to the menopause that halts further female procreation.

Take in Europe even, the divorced male twin who may marry again, and again, and again, does he stop having children with a younger wife simply because his twin sister had her menopause a decade ago? Or take my own Africa, how many women have the same number of children as the man who made them pregnant? Gender Equality makes no procreative sense either in Europe or in Africa. The mathematically minded among us may Google my article “MPSI” and study how the Index can be derived. Also read “History versus Limits of Science: Is Solomonic Genius a Y Chromosome Phenomenon?” to appreciate that the particular gene in question does not have to be in the man’s Y chromosome, but (as I said in the article) “it allows many more offspring than the female”.

Two further points (a) and (b) arise from my MPSI which did not escape one world-class geneticist. In his 2007 book “FIFTY YEARS OF HUMAN GENETICS – A Festschrift and liber amicorum to celebrate the life and work of GEORGE ROBERT FRASER” Oxford University’s world-class Geneticist Professor George Fraser (Remember Fraser Syndrome?) thought my MPSI important enough to include it as one chapter in this 568-page book under the title: “The Male Procreative
Superiority Index (MPSI): It’s relevance to genetical counselling ion Africa”. What was the point I made that has eluded many scientists?

(a) In that chapter I pointed out that something like Prostate Cancer that textbooks mention as having a genetic preponderance among Africans at home and in the Diaspora can be explained with the African’s generally high MPSI. I said (page 49): “As Africans are living longer, the husband with common diseases compatible with lifespan of appropriate length, such as essential hypertension, diabetes mellitus, gout, and even prostate cancer, might in the same way account for more genetic pathology in future generations than would be passed on by any of his wives”. Could Chinese low incidence of prostate cancer be due to the men once forced by Law to have just one child?

(b) Has present severe pressure on African countries for same sex marriage not got more to do with Population Control than Human Rights? MPSI would be meaningless!

Felix I D Konotey-Ahulu, MD FRCP DTMH, Kwegyir Aggrey Distinguished Professor of Human Genetics, University of Cape Coast, Ghana.

Facebook Enquirer November 2017

Facebook enquiries

Look at www.sicklecell.md for correct terms.

What do you mean by sicklecell?
Sickle Cell Trait (Normal gene + Abnormal gene)? Or do you mean sickle cell disease (Abnormal gene + Abnormal gene)?
To simplify things, I call Normal gene NORM and Abnormal gene ACHE because it takes 2 Abnormal genes (ACHEACHE) to make someone ache with the pain of sickle cell crisis. So, sickle cell trait is NORMACHE.

On my www.sicklecell.md Home Page you will see the kanad I invented to explain what happened when my Trait father NORMACHE married my Trait mother NORMACHE. They had 11 children of whom 3 had ACHEACHE, suffering sickle cell disease. Four of us were NORMACHE like our parents (no problems) and 4 also had no problems with NORMNORM.

It is important that readers of this Facebook each find out what Haemoglobin genes have been inherited from their parents. If, like my 3 siblings, any has inherited abnormal (ACHE) haemoglobin gene from each parent then there is no NORM gene to protect from body ache under certain circumstances. I never advise a person with ACHE Haemoglobin gene not to marry someone else, remembering that my parents would have been advised not to marry as some American States are keen to legislate.

Study the kanad video, and come to your own decision. People with sickle cell disease (ACHEACHE) have inherited some brilliant genes from their parents, like beauty, elegance, brains, and become ACHIEVERS in life as we have seen in Ghana. Visit my website, and take time with my Genetic Counselling and Voluntary Family Size Limitation (GCVFSL) http://bit.ly/1w3BuvM
Please get back to me if you can’t access it.

Finally, Sickle (S) is not the only aching gene we can be born with. The second commonest abnormal Haemoglobin aching gene is “C”. Test for “S” alone (Sickle Cell Test) is not enough. I always test for other genes, not just for Sickle Cell Trait. You can be Sickle Test Negative (that is No “S”) and yet be “C” Positive, enabling you and your Sickle-Positive-“S” spouse to have a child who has two aching genes “S” + “C” to produce Hereditary Rheumatism (Sickle Cell Disease), never ever to be called “SC Trait”, but only to be known as “SC Disease”. Sickle Cell Trait is “AS”, never “SC”.

I was born surrounded by both so I know the difference. Note that Sickle Cell Disease ‘SS’ is the only phenotype known as Sickle Cell Anaemia. These terms which are not “Konotey-Ahulu terms”; but from WHO which does not recognise the term “Sickle Cell Anaemia Disease”. If you have ‘S’ from both parents you have “Sickle Cell Anaemia” (SS). If you prefer to say you have “Sickle Cell Disease” then you need to add the phenotype and say “I have Sickle Cell Disease (SS)”. If a lady has Sickle Cell Disease (SC) and develops severe anaemia from heavy periods doctors are not entitled to say she has Sickle Cell Anaemia. She is still “SC” and not “SS”. She has Sickle Cell Disease (SC) with Anaemia, but not “Sickle Cell Anaemia Disease”. [Please read this again!].

Be the one to teach your doctors if they are confused about these terms. I once mentioned how I referred a lady to have her gall stones removed by a world class Surgeon to whom I wrote this: “Please help this Sickle Cell Anaemia (SS) lady”. Less than one hour later in the same hospital he said he called and said to me: “Thank you Felix for sending me that delightful Sickle Cell Trait lady”. So even world-class Specialists don’t know WHO definitions of who has Trait (1 Normal Haemoglobin gene) and who has Disease (No Normal Haemoglobin gene).

TERMS EXPLAINED:

Sickle Cell Trait (1 Normal Gene A+1 Abnormal Gene ‘S’) I call NORMACHE which never gives Hereditary Aches. For Sickle Cell Disease (1 Abnormal Gene ‘S’+any Abnormal Gene ‘S’ or ‘Other’) I prefer ACHEACHE as S+S, S+C, S+D, S+K, S+Korle Bu, S+Osu Christiansborg, S+FPersistence, S+O, S+Kwahu, are all aching Sickle Cell Diseases. It takes 2 ACHES to cause ache.

NOTE CAREFULLY: Normal Haemoglobin ‘A’+Abnormal ‘S’ is Sickle Cell Trait (AS). Normal ‘A’+ Abnormal ‘C’ is Sickling Negative Haemoglobin C Trait (AC).

Haemoglobin gene ‘A’ is NOT to be confused with BLOOD GROUP ‘A’. These 2 genes labelled “A” have nothing to do with each other. To check for Abnormal Haemoglobins ask for “Haemoglobin Type”, not Blood Group.

Sickle Cell Trait and Sickle Cell Disease

SICKLE CELL TRAIT and SICKLE CELL DISEASE

On Facebook 15th November 2017 responding to something on a site which described itself as “Sickle Cell Anemia Disease”, I wrote this:

“Please get your correct definitions of sickle cell disease and sickle cell trait from www.sicklecell.md Let no one deceive you re sickle cell trait. Study and learn”

I then got this message: “You know I have heard from people with sickle cell trait get pain once a year or something it’s not serious but I hear they still can have symptoms I mean it is blood line you know”.

Visiting www.sicklecell.md proved to some doctors that sickle cell disease has often been wrongly called sickle cell trait, and vice versa, with serious consequences.

“Pain once a year” is no proof of sickle cell trait. Millions of people around the world who do not have sickle cell trait have pains more than once a week!

Doctors writing SCT for sickle cell trait imply that “SC” is a Trait, which is wrong because “SC” is 2 Abnormal Haemoglobins – a disease phenotype. The Trait must have NORMAL Haemoglobin A plus S, and the “A” fraction must always be greater than the “S”. Sickle Cell Trait is written “AS Trait”, not SCT. If Electrophoresis shows “AS” (1 Normal gene A greater than S) and the person has symptoms like sickle cell disease then the person may well have Sickle Cell Quebec-Chori disease, with Hb Chori behaving like “A”. See [Konotey-Ahulu FID. Lancet February 29, 1992, page 555 http://bit.ly/2d18oOL

Beware of symptomatic sickle cell traits. Lancet, February 29, 1992, page 555.

http://www.thelancet.com/journals/lancet/article/PII0140-6736(92)90377-F/fulltext]

FOUR THINGS YOU MUST READ ON SICKLE CELL DISEASE PATIENT

FOUR THINGS YOU MUST READ ON SICKLE CELL DISEASE PATIENT

This information for all ages has helped many families.

  1. Konotey-Ahulu FID. The inheritance of Sickle Cell Disease. New African January 2000, pp 40-43
    http://www.konotey-ahulu.com/pdfs/sicklecell_jan2001.pdf
  2. Konotey-Ahulu FID. The Person with Sickle Cell Disease. New African March 2001, pp 38-39.
    http://www.konotey-ahulu.com/pdfs/sicklecell_mar2001.pdf
  3. Konotey-Ahulu FID. The Teenager with Sickle Cell Disease. New African. June 2001, pp 40-42
    http://www.konotey-ahulu.com/pdfs/sicklecell_jun2001.pdf
  4. Konotey-Ahulu FID. The Adult with Sickle Cell Disease. New African Sep. 2001, pp 40-43.
    http://www.konotey-ahulu.com/pdfs/sicklecell_sep2001.pdf
    Also http://www.questia.com

Remember that these sickle cell disease children, teenagers, and adults have inherited from their parents other genes to make them brilliant, beautiful, and much else. They must be looked after properly to make them use their brilliant genes to become ACHIEVERS in life.

See www.sicklecell.md and learn.

Good evening Prof: Should I marry this person?

Question: Good Evening Prof, A lady friend of mine is with SC since birth and she loves this guy who is AS. Should she go on with the marriage even though there is a 50% chance of having sickly children?

Kanad
ANSWER:
Dear C.M., It is not my normal habit to advise who should marry whom, but as you can see from the kanad pictured above with male phenotypes on one side, and female on the other your friend is “SC” (abnormal Haemoglobin ACHE ‘S’ gene from one of her parents, and abnormal Haemoglobin ACHE ‘C’ gene from the other parent, making her ache with sickle cell crisis at certain times.

As you observed, when the dice ACHEACHE on one side is thrown against the dice NORMACHE on the other the probability for each throw of the dice is 1 in 2 (50%) for ACHEACHE to show because the man will show NORM or ACHE with each throw. The sequence is unpredictable because the man may show NORM (‘A’) several times or ACHE (‘S’) several times. Moreover, depending on whether the lady’s ACHE is an egg carrying ACHE ‘S’ or egg with ACHE ‘C’ the children of this union may be ‘AC’ NORMACHE, (‘A’ from the man, ‘C’ from the lady, ‘AS’ NORMACHE like your lady friend’s man, ‘SS’ ACHEACHE, or ‘SC’ ACHEACHE like your lady friend. Please read this statement again until you can explain it to your lady friend. Now, my book “The Sickle Cell Disease Patient” describes exactly such a situation where a Staff Nurse “SC” asked me whether she should go ahead and marry her lover “AS”. After explaining to her just as I have done here, she said to me: “Doctor, I am a nurse and I can care for him when he is unwell. Moreover you have told your patients how to keep out of sickle cell crisis so even if we have “SS” or “SC” children we can cope.” Remember that my kanad shown above (Konotey-Ahulu Norm Ache Dice) has two main functions:

They show you (i) What Could Happen ie PROBABILITY, and what is more important (ii) PREDICTABILITY ie What Will Happen.

If someone tells me: “Doc, I have suffered too much with this hereditary ailment. I do not want any child of mine to suffer like I am doing. Show me the phenotype that I can marry so that even though I have ACHEACHE my children will never have ACHEACHE”. Well, simple: Pick the dice marked NORMNORM and it is impossible to have an ACHEACHE child. But remember that some ACHEACHE people are brighter, more beautiful, and more focussed than their siblings who do not ache. The first option is Genetic Gambling. The second option is Predicting Genetic Certainty.

But here is a beautiful true story: One of my brilliant ACHEACHE “SS” ACHIEVERS fell in love with a NORMACHE “AS” (Sickle Cell Trait) lady. They decided to go ahead and get married hoping that the first child will be from the NORM egg of the lady, and his ACHE sperm, then they will stop, and adopt their second child. Well Mr H.S. engaged this lady, married her, and they had a son, lovely son with all the elegance of the father and the combined genius of both of them, NORMACHE “AS” Sickle Cell Trait. The couple went on to adopt a daughter.

So my duty is to show the difference between Genetic Gambling (Probability), and Genetic Prediction with 100 per cent certainty. If ACHEACHE marries ACHEACHE all the children will be ACHEACHE as shown on the cover of my blue book:

See my website www.sicklecell.md Those who choose Genetic Gambling because they are madly in love should know what could happen. They will limit their family size as Mr H. S. and his wife have done.