The 19th of June 2024 was just like every other day to me. Nothing made me suspect that it was a day I would end up being sober and distraught as a result of some traumatizing memories that would be re-enacted.
These memories did not come because of the love I have for traumatizing myself, rather the World Sickle Cell Awareness Day reminded me of friends I had lost back in the days to this monster called sickle cell anemia disease.
This special day is aimed at increasing awareness, public knowledge and advocacy for the welfare of people living with this disease.
It made me remember my very brilliant friends who had died during our childhood and early days in the university as a result of their battle with sickle cell anemia disease.
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It is important to know that this date was officially adopted by the General Assembly of the United Nations with the aim of raising awareness about this preventable resource draining disease and also advocating for improved quality of care for the patients.
Before I step a bit further with this conversation, please permit me to ask you what comes to your mind when you hear that someone is a sickle cell disease patient?
This sickle cell disease is a genetic disease that is outrightly hereditary. It is not a contagious disease that can be transmitted from one person to another by contact. Unfortunately, it is a disease that is prevalent in Africa with Nigeria having an overwhelming population of patients living with the disease.
To have this disease, partners or couples who are sickle cell trait carriers (AS, AC, SS hemoglobin genotypes) or sicklers must have given birth to such an offspring with the disease.
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According to World Health Organization African region, “sickle cell disease is a disease that makes the round shape of the red blood cell become like crescent moon.
While round shaped red blood cells can move easily through the blood vessels, the crescent or sickle shaped red blood cells interconnect with each other because of their shapes and sticky nature to form blood clots in these patients.”
These blood clots in the blood vessels are the bane of the problem faced by most sickle cell patients.
Moreso, it would be great to have you look at WHO Africa region’s developed guidance Framework for Sickle Cell Disease Management and Harmonized Guide for Sickle Cell Disease Management in Africa.
The document cited above makes up the WHO SICKLE Package of Interventions for Sickle Cell Disease Management in Africa.
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The sickle shape of the red blood cells predisposes these patients to abnormal destruction of these red blood cells and as a result find it impossible to transport oxygen alongside the red blood cells to parts of the body.
The red blood cells live up to 120 days in the blood stream of people without the sickle cell disease before the body destroys them.
For patients with sickle cell disease, their red blood cells struggle to live up to an average of 15 days maximum and this explains the reason for the anemia often experienced in patients with this condition.
Sickle cell patients when triggered have crisis which often manifests as weakness, pain at the joints, distended stomach, paleness of the body due to lack of red blood cells and a lot of other symptoms.
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Except for in some very rare cases where the patient is rich and as such can afford the cost of having bone marrow transplant or gene therapy, which we suspect the Nigerian superstar Adekunle Gold has done, this disease can only be managed conservatively till death.
In some high-income countries away from the African continent, improved modalities like new born screening, hydroxyurea therapy, gene therapy, improved management strategies and expanded immunization programmes that have reduced mortality rates are being employed routinely.
The question here is, where is Africa in this entire equation?
Anyone reading through this article irrespective of your academic background must come to terms with the reality that one of the most important gifts you must give any child in your custody, is the gift of his or her blood group and genotype obtained from a licensed and accredited Medical Laboratory facility.
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Just for a bit of deviation, it is necessary to pay attention to the blood group and genotype of the people in your space.
Being Rhesus positive for a man often does not have many implications unlike what being Rhesus negative in a female entail, especially as one grows up into her reproductive age as a Lady.
Over the years, I have lived with Sickle Cell Disease patients, and I can tell you from a place of information that it is not a place you want your enemy to be, talk more of you relative.
Kindly read up the concluding part of this article by clicking here.
6 responses to “To love and to perish, the sickle cell disease conundrum. (I)”
Whoa, you really hit the nail on the head there. SS is definitely something that can be prevented if people are more informed and proactive. It’s crazy to think that in this day and age, with all the medical advancements we have, people are still making this ‘mistake.’ More education and awareness campaigns could go a long way in helping folks avoid this issue altogether. At the end of the day, it’s about empowering people with the right info to make informed choices.
Thank you very much Engr. Oscar. We are delighted to get this review from you but going forward i am of the opinion that institutions like ISN can also move a few steps forwards on this by conducting trainings for Medical Laboratory professionals even if it is virtually on the very recent, cost effective and reliable equipment that can be deployed in the field of haemoglobin genotype assay.
Wow, I love to read your write-ups because it is so educative and direct to the point. You’re doing a great job here Sir, please keep up with the good work
We cannot appreciate your readership enough, Doc.
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