Before I go on let me explain some terminologies. Sickle Cell DISEASE is NOT the same as Sickle Cell Anaemia. The former, is a big, blanket term for all the abnormalities that are possible genetically, so technically, carriers who are “AS” can be described as having Sickle Cell Disease as well as those with “SS”. The latter refers specifically to those with the SS genotype.
This is the reality: in Ghana, 25% of us are CARRIERS for Sickle Cell Disease. Considering our population currently, that means 6million to 7million of us are carrying an abnormal Sickle Cell gene that together with the same or another abnormal Sickle Cell gene from a carrier (or even a partner with the disease) partner can cause the birth of a child with sickle cell anaemia.
It is not so uncommon in my practice for a couple, married or not, to make the assertion expressed in the title of this article. Mostly, they marry without knowing their status and when they find out by some other means, then the question of probability and chances come up: “So is it the first or fourth child that will have it?” “Can we try (that is, become pregnant) again?” “Surely, we cannot have more than two kids all having the problem?”
Consider the above illustration: The scenario on the left is where a carrier and a “normal” person produce children and none of the kids will have Sickle Cell Anaemia. It is the scenario on the right that is the focus of this article; here both parents are carriers and thus there is a 25% (one-in-four) chance of having a child born with Sickle Cell Anaemia.
So does it mean that once two carriers copulate with a resultant pregnancy, the baby will definitely have Sickle Cell Anaemia, that is, SS?
The answer is NO. There is a one-in-four chance that every pregnancy will result in a child with SS genotype, that is, Sickle Cell Anaemia.
Wait a minute; what do I want to say? Does it mean that for the two carrier partners, when say they have 4 kids only one will have Sickle Cell Anaemia (that is, SS genotype)?
NO! Imagine you have 4 tomatoes in your bowl; 3 green (AS or AA) and the 4th red (SS). Each time there is a pregnancy it is as if you are dipping your hand into this bowl of 4 tomatoes to pick one at random (this is the one-in-four or 25%) with your eyes closed. You don’t know which one will come out (this is the chance).
Now, this is the confusion for most people. They assume that if for the first chance (likened to first pregnancy) say the “child is SS” (red tomato) as they say, then that is it. They further assume then that they can go ahead to have 3 more kids as they will all be “normal” (green tomatoes). This is because they think that once they dipped their hand into the bowl the first time and the red tomato came out, the bowl will now have only 3 green tomatoes for the subsequent “lucky dip”. Meaning, it is impossible to pick red tomato again because that particular one has already been taken out.
The fact is, in the event of two carrier parents, for each pregnancy the slate is wiped clean. The button is reset and there will always be 3 green tomatoes plus one red tomato in the bowl; meaning for each pregnancy, you start all over again. So a couple, both carriers can have 5 kids all “normal” or all SS; or one, two or more of either. It’s all a probability or chance and no one including Science can predict. (I should be careful, may be the Mathematicians can work something out!)
ON THIS WORLD SICKLE CELL DAY:
- Let’s screen our kids at birth so we know their status
- Tell your kids their statuses once they begin to comprehend and not later than 10 years of age
- Every adolescent in Senior High School MUST be tested to know his or her status
- Discuss your sickle cell status with the partner with whom you hope to have kids
- No one should decide for you who to marry or have kids with, but if you decide to do so as carriers, educate yourself on the implications of having a child with Sickle Cell Anaemia and where you can get help
- Finally, no one is a SICKLER; there are only persons with Sickle Cell Disease or Sickle Cell Anaemia. DO NOT STIGMATISE AGAINST THEM. LOVE THEM, EMPLOY THEM, HELP THEM.
PEDIATRICIAN and HEALTH ADVOCATE