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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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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