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

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

Adverse Clinical Consequences Following Licensing One Drug and Not Another    

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

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

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

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

NHS ENGLAND and NHS SCOTLAND differ on Pregabalin?

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

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

The Le Fanu Legacy for Thoroughness

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

PAIN and OPIATES in HOSPITAL DEATHS

“Hospital drugs left 456 patients dead” was Daily Telegraph’s front-page head-line Thursday June 21 [25] plus an Editorial description of “fatal doses of diamorphine and other inappropriate drugs” in Gosport War Memorial Hospital. Note that when the NCEPOD Report of 2008 revealed that between January 2005 to December 2006 “Nine out of the 19 patients with sickle cell disease who had pain on admission and who then died had been given excessive doses of Opiods” [9] there were neither front-page newspaper-headlines nor calls for prosecutions as we hear today about Gosport.

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

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

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

felix@konotey-ahulu.com              Twitter@profkonoteyahul

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

www.sicklecell.md

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

2 Konotey-Ahulu FID. Opiates for sickle-cell crisis? Lancet 1998; 351: 1438.
[“The question that puzzles me is: Why do west African and West Indian patients with sickle-cell disease who did without morphine in their countries have to be given morphine pumps during sickle-cell crises when they come to the UK?”]

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

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

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

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

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

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

8 NCEPOD (National Confidential Enquiry into Patient Outcome and Death). Sickle: A Sickle Crisis? (2008) [Sebastian Lucas (Clinical Coordinator), David Mason (Clinical Coordinator), M Mason (Chief Executive), D Weyman (Researcher), Tom Treasurer (Chairman) info@incepod.org

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

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

11 Konotey-Ahulu FID. Poor care for sickle cell disease patients: This wake-up call is overdue BMJ Rapid Response May 28 2008 BMJ 2008; 336: 1152 to Susan Mayor “Enquiry shows poor care for patients with sickle cell disease” on National Confidential Enquiry into Patient Outcome and Death (NCEPOD) REPORT “SICKLE:  A Sickle Crisis? (2008) http://www.bmj.com/cgi/eletters/336/7654/1152a#196224 | http://www.info@ncepod.org

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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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.

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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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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.

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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.

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Sickle Cell Trait Confusion: Is It Deliberate? Or Is This Ignorance?

Sickle Cell Trait Confusion: Is It Deliberate? Or Is This Ignorance?

I speak with authority as one who was born into a Sickle Cell Disease home within a Sickle Cell Trait country. One in every 5 of us in southern Ghana including nurses, doctors, business men and women, judges, liars, thieves, university professors, Parliamentarians, athletes, crooks, footballers, Olympic Medallists, and boxers has the Sickle Cell Trait.

In Northern Nigeria with a population of 90 million there are 30 Million Sickle Cell Traits. One in every three babies born there in Kano, Sokoto, Maedeguru is Sickle Cell Trait. And in Accra where I worked at the Korle Bu Teaching Hospital every 1 in 5 babies of the 13000 consecutive deliveries we tested in 12 months had Sickle Cell Trait.

What is more, 1 in every 3 of the white people in Greece where Lake Kopais used to be is Sickle Cell Trait! And now, lo and behold, “In Fontana August is Sickle Cell Trait Prevention Month”. Are they serious in suggesting Sickle Cell Trait needs preventing? Making 1 in 5 of us Ghanaians feel guilty for being born because we are Sickle Cell Trait? Even Sickle Cell Disease Patients need not feel guilty because they often have brilliant genes that their siblings do not possess.

Seriously, believe me, there are two kinds of readers of this Facebook post:

(1) Those who want to learn from me whom Nobel Laureate Professor Linus Pauling listened to when I delivered the Martin Luther King Award Lecture in Philadelphia on the Topic “The Vital Difference Between Sickle Cell Trait and Sickle Cell Disease”, and

(2) Those who prefer what Fontana teaches on Sickle Cell Trait.

For those who have time for me, please set time aside and study the following articles very, very, very carefully:

SICKLE CELL TRAIT

  1. Blaming sudden death on Sickle Cell Trait? http://bit.ly/1Eutn19 
  2. Sickle Cell Trait Misinformation and Disinformation http://bit.ly/1CqYHib
  3. Further Communication on Sickle Cell Trait Misinformation and Disinformation and Sickle Cell Terminology: Disease  or Disorder?          http://bit.ly/1Gm4gNP 
  4. World Sickle Cell Day 19h June 2014 http://bit.ly/1FuNXPi 
  5. Beware of symptomatic sickle cell traits. Lancet, February 29, 1992, page 555. http://bit.ly/2d18oOL
    http://www.thelancet.com/journals/lancet/article/PII0140-6736(92)90377-F/fulltext
  6. 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/
  7. Sickle Cell Trait: As with statins when leading editors disagree please give principles same weight as details/
    http://www.sicklecell.md/blog/index.php/2016/09/statins-when-leading-editors-disagree-please-give-principles-same-weight-as-details/
    http://bit.ly/2dy5fUJ
  8.  http://bit.ly/2bRQ7B1    Tafracher BMJ 8th June 1975

This Ghanaian word Tafracher allows me to call a spade a spade, as it were. [It allows me to say articles describing Sickle Cell Trait as Sickle Cell Disease are (Tafracher) rubbish for how can a Sickle Cell Trait man run at 7000 ft at Olympic Games and beat the whole world with a disease?] 

If you absorb all this information you can help your colleagues and even your doctors in saying exactly what Sickle Cell Trait is, and what it is not.

Felix Konotey-Ahulu FGA MD(Lond) FRCP(Lond) FRCP(Glasg) DTMH FGCP FWACP FTWAS Kwegyir Aggrey Distinguished Professor of Human Genetics, University Cape Coast Ghana, & Former Consultant Physician Genetic Counsellor Sickle Cell & Other Haemoglobinopathies, Korle Bu Teaching Hospital, Accra Ghana, and 9 Harley Street London W1G 9AL [ www.sicklecell.md ] Twitter Felix@profkonoteyahul

Further BMJ Links especially for doctors, nurses & science graduates.

  1. Overseas Med. Graduates bmj.com/content/356/bmj.j574/rr-0
  2. Routine Tests not to be abandoned bmj.com/content/357/bmj.j2091/rr-15
  3. BMA AGM 2017 On Abortion bmj.com/content/357/bmj.j3116/rr

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. I know the difference.

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World Sicklecell Disease Patient Week – Videos

World Sickle Cell Disease Patient Week

After a successful week of videos in July I have put them all together in one post for you to view.

Introduction
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.

Day 1
Professor Konotey-Ahulu explains the reasons behind the Sicklecell Disease Patient Week and a bit about his history.

Day 2
Professor Konotey-Ahulu interviews an achiever of over 50 years old.

Day 3
Professor Konotey-Ahulu talks about the various African tribes which have various names for the Sicklecell disease. He also explains the difference between trait and the disease.

Day 4
Professor Konotey-Ahulu gives a round up of the videos published and a bit more history on what he found during his career.

Day 5
Professor Konotey-Ahulu continues to talk to an achiever on how he stopped the disease from taking over his life and reduced crises periods.

Day 6
Professor Konotey-Ahulu explains his dice (KANAD) and how it can help explain how people get the disease.

Day 7
An achiever Akosua M Dankwa talks about the Sicklecell Disease and how it has affected her life.

Books
The Sickle Disease Patient book is now on sale at a 50% discount. The book can now be purchased here http://blog.sicklecell.md/shop/ FREE KANAD dice with each purchase whilst stock lasts.

Links
Facebook Event – https://www.facebook.com/events/305588243201034
Books – http://blog.sicklecell.md/shop/

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

World Sickle Cell Patient week

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

To view the event on Facebook look out for my videos during the week starting today. https://www.facebook.com/events/305588243201034 

World Sickle Cell Disease Patient Week

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

My books are available here http://blog.sicklecell.md/shop/

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