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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