The world celebrates sickle cell day on the 19th of June every year.
What do you know about sickle cell disease, have you even bothered to get information on it?
It will interest you to know that about 15,000 babies are diagnosed with sickle cell disease annually in Ghana.
Sickle Cell Disease is an inherited disorder of the red blood cells specifically a component called hemoglobin.
Haemoglobin is a component of the RBC (Red Blood Cells) which aids in carrying oxygen to your cells.
Mutations do occur in the genes and this leads to variants of hemoglobin common ones in our part of the world are S and C. The F type is rarely diagnosed in adults.
You inherit the genes that encode for this protein from your parents. An individual inherits an allele from each parent. A point mutation in the genetic sequence converts a glutamic acid codon (GAG) to a valine codon (GUG).
Which then produces HbS instead of HbA.
You’re are either a carrier i.e have just one HbS allele or you have the Sickle cell anaemia disease (SS).
Combinations such as SS, SC aren’t favourable and could lead to fatal outcomes.
During sickle cell crisis the sickled red blood cells block vessels supplying blood to bones and other tissues.
Hemoglobin levels go very low (anaemia) – fatigue sets in, severe joint pains, lung and heart injuries amongst others.
Most people with sickle disease require frequent blood transfusion since the body breakdown this type of red blood cells faster as compared to the normal red blood cells.
Although, we have BMT or Blood Stem Cell Transplant a procedure which replaces the unhealthy blood-forming cells in SCD patients with healthy ones the procedure is capital intensive.
Regimens are available for managing the disease but It’s best advised that individuals know their sickling status and genotype to make an informed decision especially when it comes to choosing a life partner.
Marriage between individuals with sickled cell disease with the intention of procreating is a big NO.
It’s a game of chance for partners who are both carriers since they have a 25% chance of bringing forth a child with AA genotype, 25% chance of SS genotype and 50% chance of having carriers.
It’s not advisable to gamble with the fate of your unborn child.
If your genotype is not AA, marry an AA partner.
That’s your best bet.
Don’t be ignorant
Get tested today.
MR. EMMANUEL NDEMELE
BSc. Human Biology