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.

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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]

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World Sickle Cell Disease Patient Week

World Sickle Cell Disease Patient Week

WSCDPW [World Sickle Cell Disease Patient Week]

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

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

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

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

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

Professor Felix I D Konotey-Ahulu [whose parents were Traits for Abnormal Haemoglobin genes and whose 3 siblings had sickle cell disease].
MB BS MD(Lond) FRCP(Lond) FRCP(Glasg) DTMH (L’pool) DSc(Hon UCC) FGCP FWACP FTWAS ORDER OF THE VOLTA (OFFICER)
Kwegyir Aggrey Distinguished Professor of Human Genetics, University of Cape Coast Ghana, and Former Consultant Physician Genetic Counsellor Korle Bu Teaching Hospital Ghana and 9 Harley Street, London W1G 9AL.

https://www.facebook.com/events/305588243201034

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

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

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

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

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

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

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

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

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

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

  1. Godlee Fiona. Statins: We need an independent review. BMJ 2016; 354: i4992 http://www.bmj.com/content/354/bmj.i4992
  2. Knapton Sarah. “End statins controversy with government review. Daily Telegraph. Friday 16 September 2016, page 1.
  3. Krumholz Harlan M. Statins evidence: when answers also raise questions. Sharing the data is more likely to settle the debate than another review. BMJ 2016; 354: i1463 (doi:10.1136/bmj.i4963)
  4. Dyer Clare. Journal agrees to retract paper after university found study was never done. BMJ 2013; 347: 155 http://dx.doi.org/10.1136/bmj.f5500 Sept 5 2013.
  5. Blakemore Sarah. Statins: We need an independent review. BMJ Rapid response www.bmj.com/content/354/bmj.i4992/rapid-responses.
  6. Rhida A, Khan A, Al-Abayechi S, Puthenveetil V. Acute compartment syndrome secondary to rhabdomyolysis in a sickle cell trait patient. Lancet 2014; 384:2172
  7. Konotey-Ahulu FID. Dangerously flawed diagnosis of sickle cell trait in compartment syndrome rhabdomyolysis http://bit.ly/2d4t9Zd
  8. Konotey-Ahulu FID. Group specific component and HIV infection. Lancet 1987; i: 1267.
  9. Eales L-J, Nye KE, Pinching AJ. Group specific component and AIDS: Erroneous Data. Lancet 1988; i: 936.
  10. Lehmann Hermann. Sickle cell and flying. The Times, London January 4, 1972.
  11. Konotey-Ahulu FID. Four bodyguards and the perils of unmasking scientific truths. BMJ 2007; 335: 210-211 www.bmj.com/cgi/reprint/335/7612/210.pdf
  12. Whiskey Delta Charlie [Facebook September 5 at 5:35 pm] Sickle Cell Trait mixed with Rhabdomyolysis could equal sudden death during exercise or if low oxygen levels are present. Secondary complications can be present as well #sicklecelltrait #exercise #rhabdomyolysis #trackandfield #marathontraining #2milerun #crossfit #physicaltraining #preseason #military my story in the bio# sicklecellawarenessmonth #itsnotblackgene #global #bloodline #fighgtsmalaria #hispanic #mediterranean #europe #asian bloodtests
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