Gaming with Sickle Cells

Imagine. Image. Branding Ghana. A diamond is just a piece of rock, in the rough, until it is identified as something precious and cleaned to reveal its glory. Then, viola! diamonds are then considered forever! These are the thoughts that have been consuming my thinking and talking moments during the past two weeks.

Then, an opportunity came unto my plate to interview Dr. Konotey-Ahulu. Yes, you guess right! It’s the same Dr. Konotey-Ahulu of sickle cell fame. We must not forget such priceless gems of national assets, our high-achievers here at home and in the Diaspora, in any effort to coherently brand Ghana.

I had several hours of recorded interview with him and came out with a wealth of information that will be published elsewhere. Today, I release to you in this column, a bit of the knowledge I gleaned from him.

He is 79 years old and is still at his game. Like some other medical doctors including Professors Badoe and Archampong of the Department of Surgery of the Korle Bu Teaching Hospital, and other national jewels elsewhere, Dr Konotey-Ahulu has retired from public service but not retired from deepening and sharing knowledge. Lesson? It should not be over until it’s over! We must live our lives fully.

While hanging around the extraordinary Konotey-Ahulu, I also learned his perspectives on the importance of learning HOW to think instead of WHAT to think. The two are very different. Learning by memorizing information ‘by heart’ cannot take us far. Rather, critical thinking that probes to find answers is the path to solving critical and extraordinary problems. But all that information will be reserved for another article.

Today, let’s play a sickle cell game with two sets of three different dices (he gave me as a Christmas gift – far ahead of time). These dice are the KANAD – an acronym for the Konotey-Ahulu Norm-Ache Dice. It is a cube, very much like a six-sided dice used in a ludu game. This is a game for two people to play – a male and a female.

The basis for these dices is that every individual carries two haemoglobin types, one from the father and the other from the mother, which are inherited at conception – either a pair of normal-normal, normal-abnormal or abnormal-abnormal. The dices in the KANAD are therefore the NORMNORM, NORMACHE and ACHEACHE.

Stay with me; don’t get confused. Remember, we’re only setting up to play a game of dice. To increase the reliability of the results of the game, the male and female could play each round of game several times to get a feel for the probability of the combination of haemoglobin types that an offspring could inherit from parents. This game is therefore strongly recommended for people who are of child-bearing age and are going through the motions of selecting a partner with the intention or likelihood of procreating – to populate the earth.

Just imagine if through a simple game of dice, you could eliminate, or at the least, reduce the chances of ending up with a sick offspring! Granted, that life is a game of chance; but we would be better off if we could play life by taking away some of the unknown factors in the chance game. Dr. Konotey-Ahulu uses the dice in genetic counselling.

Here are the results of (taflatse) four rounds I played with a friend during the week – at my dining table. The results made my eyes pop widely open. They are presented below. NORMNORM played against NORMACHE four times resulted in two NORMACHE and two NORMNORM. When we played the NORMACHE dice against the ACHEACHE dice, it resulted in two NORMACHE and two ACHEACHE.

Of course, by now, the results should be obvious for the other pairs of dice. When ACHEACHE is played against ACHEACHE, it can only result in ACHEACHE; while NORMNORM against NORMNORM will only result in NORMNORM. Similarly, when NORMNORM is played against ACHEACHE, it can only result in a NORMACHE.

NORM is the same as haemoglobin Type A; (this is different from Blood Group A) and ACHE is haemoglobin Type S. NORMNORM is the same as AA – non-sickler and therefore no ACHE! NORMACHE is the same as AS – sickle cell trait but with no ACHE! However, since NORMACHE is part A and part S and therefore a carrier of ACHE (the S cell), such individuals could potentially pass on the sickle-shaped red cell membrane to their offspring.

The plot thickens further. Some NORMACHEs are not regular AS but AC because they have a non-A haemoglobin type that is a C.

So therefore if I had been actually mating instead of just playing a game of dice at my dining table, out of the episodes of child birth, I might have given birth to eight SS or ACHEACHEs (that is sick children); eight with the sickle cell trait who will grow up to also potentially pass it on to their offspring; and eight ‘normal’ children.

Of course, this is a probability game and it very much depends on which sperm meets which egg at the august moment of pregnancy. Pregnancy is a probability game; you can never know what you gonna get! For pregnancy to occur, millions of sperms (taflatse) charge towards a woman’s ovary but only one can succeed in the mighty race to fertilize the woman’s egg. It is fascinating that even between what appears to be normal healthy people, genetic gambling can still occur.

So if a NORMACHE and a NORMACHE mate and they are fortunate that their first child is not a sickler, they should stop tempting nature because the probability of having an ACHEACHE (SS) child remains high. Besides, they also stand the chance of passing on S haemoglobin types to the next generation.

Here are some disturbing facts. Twenty percent of the population in Southern Ghana are NORMACHE (AS), that is – they have inherited a sickle cell trait from one parent and a normal haemoglobin type from the other parent. Another ten percent of Southern Ghanaians who are NORMACHE are AC. These figures are the reverse for Northern Ghana with 20 percent AC and ten percent AS.

A whopping one-third (33 percent) of Ghanaians are therefore walking around with one abnormal haemoglobin trait (either an AS or AC). All of such people are perfectly well and do not ache; neither do they know that they carry sickle cells.

Not knowing what they carry in their haemoglobin, they also do not pay attention to what haemoglobin type the partner or target partner is carrying. Then, as if in a grand game of genetic gambling, (taflatse) the mating begins, followed by grand out-dooring parties to celebrate the birth of a child who, may be, just maybe, might be an SS, a sickler who will ache and ache and ache. That is also known as chwechweechwe! No wonder ACHEACHE and chwechweechwe sound alike.

For more information as you and I learn more about sickle cells, visit these websites. www.sicklecell.md; http://blog.konotey-ahulu.com/. You could also check with the Sickle Cell Clinic at Korle Bu Teaching Hospital. Knowledge is power.