Sickle cell! sickle cell !! this is a disease that should be talked about every single day because inasmuch as the disease is not curable it is absolutely preventable.
A doctor said love is no longer enough, you need to know your genotype before deciding to have a baby with anyone. But what is the genotype and what does it have to do with sickle cell.
Sickle cell is a group of inherited blood disorders. The normal red blood cell has a round or oval shape which makes it easy to move through blood vessels but in this case, the red blood cell takes the form of a crescent or a sickle which make it difficult to travel through some vessels, especially smaller vessels. This stops blood from getting to certain parts of the body, causing pain and tissue damage in the process.
Sickle cell is developed when two people who have sickle cell trait have a baby. ‘Sicklers’ or SS as they are popularly called around here in Nigeria are born of both parents who have AS genotype. If one parent has the sickle cell trait, there’s nothing to worry about as the disease requires two copies of the gene to be activated.
June 19th is the official World Sickle Cell Day and it is observed annually to constantly educate people about the disease, increase public knowledge and the challenges faced by caregivers and sickle cell persons.
Nigeria has a high number of people living with sickle cell and about 150,000 children are born annually with SCD. Sickle cell was formerly thought to be a spiritual problem and some African literature refer to them as ‘abiku’, usually used to describe children who are born sickly and die before puberty. Some families were known to have such children and they just term them spirits who come to die early and leave the family in pain. But the truth is that the entirety of SCD begins from genotype and genotype compatibility.
What is Genotype
Genotype is inherited from both parents and it plays an important role in your health and wellbeing. The genotype is a part of the genetic makeup of a cell in an individual which determines one of its characteristics. Your genotype determines if you have the sickle cell trait, or have sickle cell anemia or don’t have it at all.
There are basically four types of genotypes.
The AA, AS, SS and AC .
Your genotype can determine your compatibility with your life partner. People who are AA are neither carriers nor do they have sickle cell disease. This makes them compatible with other genotypes without the fear of having a sickle cell child.
The AS on the other hand are carriers and therefore needs to be careful who they marry. AS have a limited option and are only compatible with AA, if an individual with an AS genotype marries an AS too, there’s a 90 per cent chance that an SS child will be born. Although some of them get lucky and have only AS children, some others may have only 1 SS child out of 5 children and in the worst case have 4 SS children out of 5.
SS is outrightly a sickle cell carrier and also has it manifesting. This is the deadliest kind of genotype to have as most people with it suffer so much and die at a young age. It is believed that if they live up to a certain age about 30 or so, they will be free of sickle cell crisis but that has not been proven because some cases are so terrible and it keeps deteriorating.
The AC is like a variant of AS, they are just carriers as well.
It is very important to know your genotype before you get deep into your relationship. I had two course mates who were dating while we were in the university, They dated for three years in school and two more years after we graduated. They began to plan for their marriage and in the midst of the preparation, they took a genotype test only to find out that they were both AS. As sad as it was, they were advised not to get married and they didn’t.
Some may wonder if it is wise to sacrifice love over genotype? I’ll leave you to answer that. Just know that marriages have been broken and partners gone into depression over having to manage a sickle cell child.
Preventing sickle cell disease seems like a very simple thing, but I will advice, simply don’t marry someone who has an incompatible genotype.
Another good thing is that through science, in developed countries, they are able to remove the gene causing the disease using gene therapy. Gene therapy decreases or eliminates the symptoms of SCD.
Sickle cell complication
I still don’t know how parents with sickle cell children manage it. It hurts to watch the children cry from the pain and the regular doctor visits, the sleepless nights and thoughts like, is the child passes on this night? I mean… It’s a scary thing that no one should willingly and knowingly get into.
Some complications of sickle cell include…..
Vision loss
Acute chest syndrome
Severe body pains
High risk of regular infections
Malnutrition and growth retardation etc.
Takeaway
World Sickle Cell Day encourages everyone to get involved in any way possible and the first step is by knowing your genotype. This will greatly reduce the number of babies born with the sickle cell anemia in Nigeria and Africa as a whole.
Sickle cell comes with a lot of complications and both parents and individuals with SCD must learn how to manage it, know what the triggers are and prevent frequent crises.
It is believed that individuals with SDC can live long and fulfill life goals as long as they manage it, take their medications and never miss checkups with their doctors.
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