Understanding Sickle Cell and Knowing Your Genotype

sickle cell tinz

Today (19/06) is World Sickle Cell Day. 19th June of every year is designated to raise awareness of Sickle Cell Disease (SCD), which affects an estimated 500,000 newborns every year. To follow the movement search #WorldSickleCellDay on social media.

What’s Sickle Cell Anaemia:
Sickle cell anaemia is a genetic (inherited) blood disorder in which red blood cells, which carry oxygen around the body, develop abnormally and prevent the stream of blood and oxygen from reaching organs. Instead of being round-shaped, the blood cells are shaped as sickles, or crescents.
These abnormal red blood cells can then clog sections of blood vessels leading to episodes of pain which can be severe. These episodes are called a sickle cell crisis (also known as a vaso-occlusive crisis). They can last from a few minutes to several months, though on average most last five to seven days. Abnormal cells have a shorter life (less than 20 days) compared to normal cells, which live for 120 days. This results in a shortage of red blood cells in the body, which causes anaemia.

Symptoms of SCD:
1. Delayed growth: As oxygen is required for physical development, children with SCD might experience delayed growth and late puberty (around 13-14 years of age). Adults with SCD are most likely light weight and have a slim physique.
2. Yellowing of skin and eyes: Jaundice (yellowing of the skin and the whites of the eyes) occurs as the shortage of red blood cells leads to a build-up of waste in the body.
3. Physical weakness: This includes tiredness and breathlessness; especially after exercise.

Can SCD be managed?
SCD can be managed by taking antibiotics on a daily basis (especially children). Treatment helps reduce the severity and frequency of the symptoms of sickle cell anaemia and prevent complications. In some cases a person with sickle cell anaemia may require regular blood transfusions to help reduce the risks of complications. Treatments can reduce pain, swelling and risk of infections.

Can SCD be cured?
SCD can be cured through bone marrow or cell transplant which is very expensive and risky.


Why You Should Know Your Genotype:
Having the sickle cell trait (ie AS or AC) itself will not cause a person to develop sickle cell anaemia. But if two people with the trait (ie AS/AC and AS/AC) conceive a child then there is a one in four chance that child will be born with sickle cell anaemia (SS).
If your genotype is AS, AC or SS, it’s advisable you marry someone whose genotype is AA. If someone with any of the above genotypes marries another with genotype aside AA, there is a risk of giving birth to a child with sickle cell disease(SCD) i.e. SS or SC. For instance, if a woman who is AS marries a man who is also AS, there is 1/4 probability of having a child with genotype SS (or SC in d case of union between AS & AC) in every conception and if unfortunate, this can occur repeatedly. If you have ever seen someone especially a child with SCD during crisis with severe pain(or any other complication), then you will try as much as possible to prevent its occurrence.
According to Mrs. Biola who is an AS, she indicated that she and her husband had blood tests done before they got married. In her own words “I love my husband so much but not to the detriment of my children. I am an AS and if my fiance (now husband) was an AS, we wouldn’t have gotten married. My parents are both AS and they had an SS child who died at 3 years old. So I learnt my lessons from them. I don’t want to bring any child to this World to suffer and as a matter of fact seeing your child in crisis will affect any marriage”.
When asked, Mrs. Salaudeen indicated that she is an AA while her husband is an AS. She said that if she was an AS, she wouldn’t have married her husband. In her own words “If I were to be an AS, I can’t even imagine dating an AS talk less of marrying one. Its dangerous and not advisable. The life of the kids we are to have is at stake.”

How to know your Genotype:
It can be determined in d medical laboratory by a test called Hb electrophoresis. Knowing your genotype is of great importance because of public health significance of Sickle Cell Disease(SCD), which may result from medically incompatible marriage. Examples of genotypes are AA, AS, AC, SS, SC & CC.

Please know your genotype now before you even start dating someone so you don’t make love an excuse to enter a medically incompatible marriage and give birth to a sickle cell child.
Love (he’s AS and I am AS but we love eachother too much not to marry) and spirituality (she’s AS and I am AS but we believe God will heal us or not give us an SS child) are okay but as the Bible says in Ecclesiates 10:10 “…wisdom is profitable to direct”. Please be wise, know your genotype today and don’t put your future children’s health at risk.

Watch this emotional video of a family’s battle with Sickle Cell titled A Family’s Challenge


World Sickle Cell Day 2014 by International Business Times.
Do You Know Your Genotype? by Sex and Relationship Matters in Africa.
Sickle Cell Anaemia by NHS Choices.

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